Chapter 40. Preeclampsia Syndrome. Will Obs.

 Preeclampsia Syndrome

BS. Nguyễn Hồng Anh

Hypertensive disorders include preeclampsia, gestational hypertension, and chronic hypertension and complicate up to 10 percent of pregnancies. As a group, they are one member of the ealy trial—along with hemorrhage and infection—that contributes greatly to maternal morbidity (July, 2019).

Preeclampsia, either alone or superimpose on chronic hypertension, is the most angerous. In the United States from 2011 to 2015, 7 percent o pregnancy-relate maternal eaths were cause by preeclampsia or eclampsia (Petersen, 2019). Most hypertension-relate eaths are eeme preventable (Katsuragi, 2019). In response, Joint Commission (2019) accreite hospitals are now require to track their recognition an timely treatment o hypertension.

In 2018, a workshop to stuy preeclampsia was convene by the National Heart, Lung, an Bloo Institute. Tis buils on the prior work o the American College o Obstetricians an Gynecologists’ ask Force on Hypertension in Pregnancy (2013). Its purpose was to review topics regaring all aspects o preeclampsia an to recommen uture research areas. Many o these topics are iscusse throughout this chapter an Chapter 41.

TERMINOLOGY AND DIAGNOSIS

o coiy the classication o hypertensive isorers o pregnancy, the American College o Obstetricians an Gynecologists (2013, 2020) escribes our types o hypertensive isease:

1. Preeclampsia an eclampsia synrome

2. Chronic hypertension o any etiology

3. Preeclampsia superimpose on chronic hypertension

4. Gestational hypertension, in which enitive evience or the preeclampsia synrome oes not evelop an hypertension resolves by 12 weeks postpartum.

This classification aims to diferentiate preeclampsia syndrome, which is potentially more ominous, from other hypertensive disorders.

Diagnosis of Hypertensive Disorders

Hypertension is diagnosed empirically when systolic and diastolic blood pressures exceed 140 mm Hg and 90 mm Hg, respectively. Korotko phase V is use to ene iastolic pressure.

Previously or pregnant women, increases o 30 mm Hg systolic or 15 mm Hg iastolic above bloo pressure values taken at mipregnancy ha also been use as iagnostic criteria, even when absolute values were <140/90 mm Hg.

These incremental changes are no longer use to ene hypertension. However, bloo pressure surveillance in these gravias is reasonable because eclamptic seizures evelop in some whose bloo pressures have staye below 140/90 mm Hg (Alexaner, 2006).

In other cases, mean arterial pressures that suenly rise but that still lie in normal range—“elta hypertension”— may signiy preeclampsia (Maconal-Wallis, 2012; Zeeman, 2007). We use this term to escribe a relatively acute rise in bloo pressure in iniviual patients, albeit some still with pressures <140/90 mm Hg (Fig. 40-1). Some o these women will go on to have obvious preeclampsia, an some even evelop eclamptic seizures or hemolysis, elevate liver enzyme levels, an low platelet count (HELLP) synrome.

Historically, systolic an iastolic bloo pressure levels o 140/90 mm Hg have been arbitrarily use since the 1950s to ene “hypertension” in nonpregnant iniviuals. However, these levels were selecte by insurance companies to characterize a population o mile-age men. It seems more realistic to ene normal-range bloo pressures or specic populations— such as young, healthy, pregnant women (Lu, 2019; Rey, 2020). o provie such ata, >1000 women were recently stuie longituinally through pregnancy (Green, 2020). Data o this type may shape uture threshols.

Gestational Hypertension

Women with gestational hypertension have bloo pressures that reach 140/90 mm Hg or greater or the rst time ater mipregnancy but lack proteinuria. Almost hal o aecte women subsequently evelop preeclampsia (Jim, 2017). Even so, when bloo pressure rises appreciably, it is angerous to both mother an etus to ignore this elevation only because proteinuria has not yet evelope (Fishel Bartal, 2020). As

Chesley (1985) emphasize, 10 percent o eclamptic seizures evelop beore overt proteinuria can be etecte. Gestational hypertension is reclassie by some as transient hypertension i preeclampsia oes not evelop an bloo pressure returns to normal by 12 weeks postpartum.

■ Preeclampsia Syndrome

Preeclampsia is best described as a pregnancy-specic syndrome that can aect virtually every organ system. Although preeclampsia is more than simply gestational hypertension with proteinuria, the appearance o protein remains a primary iagnostic criterion. It is an objective marker an reects the system-wie enothelial leak that characterizes the preeclampsia synrome.

Last, preeclampsia can be ivie into early onset, <34 weeks; late onset, ≥34 weeks; preterm onset, <37 weeks; an term onset, ≥37 weeks (Burton, 2019; Poon, 2019). In some women with preeclampsia, neither overt proteinuria nor etal-growth restriction are eatures (Sibai, 2009).

Because o this, the ask Force (2013) suggests other iagnostic criteria, some o which are shown in Table 40-1. Multiorgan involvement may be reecte by thrombocytopenia, renal ys- unction, hepatocellular necrosis, central nervous system perturbations, or pulmonary eema.

Te markers liste in able 40-1 help also to classiy preeclampsia synrome severity. Although many use a ichotomous “mil” an “severe” classication, the ask Force (2013) iscourages the use o “mil preeclampsia.” It is problematic that there are criteria or the iagnosis o “severe” preeclampsia, but the binary eault classication is either implie or specically terme “mil,” “less severe,” or “nonsevere” (Alexaner, 2003; Linheimer, 2008). No consensus criteria ene “moerate” preeclampsia, which is an elusive thir category.

We use the criteria recommene by the American College o Obstetricians an Gynecologists (2020), some o which are liste in Table 40-2 an categorize isease as “severe” versus “nonsevere.”

Some symptoms are consiere ominous. Headaches or visual disturbances can precee eclampsia, which is a convulsion in a woman with preeclampsia that is not attributable to another cause. Te seizures are generalize an may appear beore, uring, or ater labor. Te proportion that evelops seizures later—ater 48 hours postpartum—approximates 10 percent (Sibai, 2005; Zwart, 2008). Another symptom, epigastric or right upper quadrant pain, requently accompanies hepatocellular necrosis, ischemia, an hepatic eema. Elevate serum hepatic transaminase levels can be one marker.

Last, thrombocytopenia also signies worsening preeclampsia. It represents platelet activation an aggregation an microangiopathic hemolysis. Other actors inicative o severe preeclampsia inclue renal or cariac involvement. When these signs an symptoms are prooun, they likely cannot be temporize, an elivery will more likely be require.

Importantly, ierentiating nonsevere an severe gestational hypertension or preeclampsia can be misleaing because what might be apparently mil isease may progress rapily to severe isease.

■ Preeclampsia Superimposed on Chronic

Hypertension

Any chronic hypertensive isorer preisposes a woman to evelop superimpose preeclampsia synrome. Chronic unerlying hypertension is iagnose in women with ocumente bloo pressures ≥140/90 mm Hg beore pregnancy or beore 20 weeks’ gestation, or both. Tus, in women who are not rst seen until ater mipregnancy, hypertensive isorers can be icult to classiy. For example, a woman with previously uniagnose chronic vascular isease who is seen beore 20 weeks requently has bloo pressures within normal range. During the thir trimester, however, as bloo pressures return to their originally hypertensive levels, it may be i- cult to etermine whether hypertension is chronic or inuce by pregnancy. Even a careul search or evience o preexisting en-organ amage may be utile, as many o these women have mil isease an no evience o ventricular hypertrophy, retinal vascular changes, or renal involvement.

In 20 to 50 percent o women with chronic hypertension, bloo pressure rises to obviously abnormal levels, typically ater 24 weeks’ gestation. I new-onset or worsening baseline hypertension is accompanie by new-onset proteinuria or other nings liste in able 40-1, superimpose preeclampsia is iagnose (American College o Obstetricians an Gynecologists, 2019a). Compare with “pure” preeclampsia, superimpose preeclampsia commonly evelops earlier in pregnancy. It tens to be more severe an more oten is accompanie by etalgrowth restriction. Te same criteria shown in able 40-2 also urther characterize the severity o superimpose preeclampsia.

INCIDENCE AND RISK FACTORS

Preeclampsia is ientie in 5 to 8 percent o all pregnancies (Jim, 2017; Poon, 2019). Young an nulliparous women are particularly vulnerable, whereas oler women are at greater risk or chronic hypertension with superimpose preeclampsia (Sheen, 2020). In one review o global stuies, the incience o preeclampsia in nulliparas range rom 3 to 10 percent (Sta, 2015). In multiparas, the incience ranges rom 2 to 5 percent (Jim, 2017; Poon, 2019).

Te incience o preeclampsia is also inuence by race, ethnicity, an genetic preisposition. In one stuy by the Maternal–Fetal Meicine Units (MFMU) Network, the incience o preeclampsia was 5 percent in white, 9 percent in Hispanic, an 11 percent in Arican American nulliparas (Myatt, 2012a,b). In aition, black women carry higher risk or associate severe averse outcomes (Gyam-Bannerman, 2020).

For several clinical actors, Bartsch an associates (2016) extracte ata rom more than 25 million pregnancies an calculate relative risks (Table 40-3). Major risks inclue oler age, nulliparity, obesity, iabetes, an chronic hypertension. Another is preeclampsia an especially HELLP synrome in a prior pregnancy (Malström, 2020). Unerlying metabolic syn- rome, hyperhomocysteinemia, or chronic kiney isease are others (Masouian, 2016; Wiles, 2020).

O lesser actors, human immunoeciency virus (HIV) seropositivity, sleep-isorere breathing, an a male etus pose a slightly higher risk (Facco, 2017; Jaskolka, 2017; Sansone, 2016). Previously aecte amily members are another, an maternal an etal genetics are assuming greater preictive importance (Burton, 2019; Gray, 2018; Phipps, 2019). Last, preeclampsia requently complicates the “mirror synrome” (Chap. 18, p. 364) (ra, 2021). Although smoking uring pregnancy causes various averse pregnancy outcomes, ironically, it lowers the risk or hypertension uring pregnancy.

For eclampsia, seizure incience has ecline in areas where health care is more reaily available. In countries with aequate resources, the incience averages 1 case in 2000 to 3000 eliveries (Jaatinen, 2016; O’Connor, 2013; Schaap, 2019). At Parklan Hospital, the incience has ecline appreciably uring the past ecae an approximates 1 case in 2000 births (Fig. 40-2).

Tis requency may be relate to improve access to prenatal care an our active management approach (Chap. 41, p. 717).

ETIOPATHOGENESIS

Te mechanisms by which pregnancy incites or aggravates hypertension remain unsolve. Any satisactory theory concerning the origins o preeclampsia must account or the observation that gestational hypertensive isorers are more likely to evelop in women with the ollowing characteristics:

• Exposure to chorionic villi for the rst time

• Exposure to a superabundance of chorionic villi, as with twins or hyatiiorm mole

• Preexisting conditions associated with endothelial cell activation or inammation

• Genetic predisposition to hypertension developing during pregnancy.

A etus is not a requisite or preeclampsia to evelop. Although chorionic villi are essential, they nee not be intrauterine. For example, preeclampsia can evelop with an avance abominal pregnancy (Worley, 2008). Regarless o precipitating etiology, the cascae o promoting events leas to systemic vascular enothelial amage, vasospasm, plasma transuation, an ischemic an thrombotic sequelae.

■ Phenotypic Expression

Tis varies wiely or preeclampsia, an phenotype is aecte by the egree o remoeling o uterine spiral arterioles by enovascular trophoblasts. Tis process unerlies the “twostage isorer” theory o preeclampsia pathogenesis. Accor-

ing to Reman an coworkers (2015), stage I—the placental

synrome—is cause by aulty enovascular trophoblastic

remoeling that ownstream causes stage II—the maternal

synrome. Importantly, stage II can be moie by maternal conitions that also maniest enothelial cell activation

or inammation. Tese inclue chronic hypertension, renal

isease, obesity, immunological or connective tissue isorers,

an iabetes.

Such staging is articial, an preeclampsia synrome presents a spectrum o isease (Burton, 2019). Moreover, “iso-

orms” likely exist an are iscusse subsequently. Dierences

inclue maternal an etal characteristics, placental nings,

genetic actors, an early- versus late-onset isease (Gray, 2018;

Phipps, 2019; Poon, 2019).

■ Etiology

O suggeste mechanisms to explain the cause o preeclampsia,

primary ones inclue:

• Placental implantation with abnormal trophoblastic invasion

o uterine vessels

• Dysfunctional immunological tolerance between maternal,

paternal (placental), an etal tissues

 Maternal maladaptation to cardiovascular or inammatory

changes o normal pregnancy

• Genetic factors that include predisposing genes and epigenetic inuences.

■ Stage I—Placental Syndrome

Normal placental implantation, as iscusse in Chapter 5 (p.

90), is characterize by extensive remoeling o the spiral arterioles within the eciua basalis (Fig. 40-3). In this “placental

be,” enovascular trophoblasts replace the vascular enothelial an muscular linings. Tis avantageously enlarges arteriole

iameter (Brosens, 2019). Veins are invae only supercially.

In some preeclampsia cases, but not all, trophoblastic invasion

may be incomplete. With this, eciual vessels, but not myometrial vessels, become line with enovascular trophoblasts.

Te eeper myometrial arterioles thus o not lose their enothelial lining an musculoelastic tissue. As a result, their mean

external iameter is only hal that o corresponing vessels in

normal placentas (Fisher, 2015). Tis mechanism is more prevalent in women with early-onset preeclampsia (Khozhaeva,

2016). Evience suggests a critical role or soluble antiangiogenic growth actors in this aulty enovascular remoeling

(McMahon, 2014).

From placental electron microscopy stuies, early preeclamptic changes inclue enothelial amage, insuation o plasma

constituents into vessel walls, myointimal cell prolieration, an

meial necrosis (De Wol, 1980). Hertig (1945) reerre to lipi

accumulation in myointimal cells an macrophages as atherosis. Tese nings are more common in placentas rom women

iagnose with preeclampsia beore 34 weeks’ gestation (Nelson, 2014). Acute placental vascular atherosis may also ientiy a

group o women at greater risk or atherosclerosis an cariovascular isease later in lie (Sta, 2015) (Chap. 41, p. 726). In pregnancy, abnormally narrow spiral arteriole lumens likely impair

placental bloo ow, reuce perusion, an create a hypoxic

environment (Burton, 2019).

At this point, these changes incite a systemic inammatory

response, which is stage II or the maternal synrome. Deective

placentation is posite to urther preispose aecte women

to gestational hypertension, preeclampsia synrome, preterm

elivery, etal growth-restriction, an placental abruption (Brosens, 2019; Labarrere, 2017; Nelson, 2014).

Immunological Factors

Maternal immune tolerance to paternally erive placental an

etal antigens is iscusse in Chapter 5 (p. 85). Loss o this tolerance is another cite theory or preeclampsia (Erlebacher, 2013).

Certainly, histological changes at the maternal–placental inter-

ace in those with preeclampsia suggest acute grat rejection.

olerance ysregulation might also explain the elevate risk

when the paternal antigenic loa is increase. One example is

complete molar pregnancies, which have iploi complement

o chromosomes solely rom the ather. Tose with later-stage

moles, have a high incience o early-onset preeclampsia.

Women with a trisomy 13 etus also have a 30- to 40-percent

incience o preeclampsia. Te gene or one preeclampsia-linke

actor, soluble ms-like tyrosine kinase 1, is on chromosome 13

(Bolah, 2006). Tese women have elevate serum levels o

antiangiogenic actors, which can aect the placenta (p. 694).

Last, women previously expose to paternal antigens, such as

a prior pregnancy with the same partner, may be “immunize”

against preeclampsia. Conversely, multiparas impregnate by a

new partner have a greater risk o preeclampsia (Mostello, 2002).

Burton an colleagues (2019) reviewe the possible role

o immune malaaptation in preeclampsia pathophysiology.

In women estine to have preeclampsia, extravillous trophoblasts early in pregnancy express reuce amounts o immunosuppressive nonclassic human leukocyte antigen G (HLA-G).

Black women more commonly have the 1597∆C gene allele,

which is associate with incomplete HLA-G expression an

may preispose to preeclampsia (Loisel, 2013). Tis immune

malaaptation may contribute to the eective placental vascularization seen with preeclampsia.

As iscusse in Chapter 4 (p. 61), -helper (T) lymphocytes uring normal pregnancy are prouce so that type 2

activity is increase in relation to type 1. Tis is the so-calle

type 2 bias (Phipps, 2019; Reman, 2015). T2 cells promote

humoral immunity, whereas T1 cells stimulate inammatory

cytokine secretion (Ma, 2019). Beginning in the early secon

trimester in women who evelop preeclampsia, T1 action is

increase.

Genetic Factors

Preeclampsia appears to be a multiactorial, polygenic isorer.

In one stuy o almost 1.2 million Sweish births, a genetic

association was oun or gestational hypertension an or preeclampsia (Nilsson, 2004). War an aylor (2015) cite an

incient risk or preeclampsia o 20 to 40 percent or aughters

o preeclamptic mothers; 11 to 37 percent or sisters o preeclamptic women; an 22 to 47 percent or twins. Ethnoracial

actors are important an evience by the high incience o

preeclampsia in Arican American women. Latina women have

a lower incience because o interactions o American Inian

an white race genes (Shahabi, 2013).

Te hereitary preisposition or preeclampsia likely stems

rom interactions o literally hunres o inherite genes—both

maternal an paternal—that control many enzymatic an metabolic unctions throughout every organ system (Burton, 2019;

riche, 2014). Plasma-erive actors may inuce some o these

genes in preeclampsia (Leseva, 2020; Mackenzie, 2012). Tus, the

clinical maniestation in any given woman with the preeclampsia synrome will reect a spectrum. In this regar, phenotypic

expression will ier among similar genotypes epening on

interactions with environmental components (Yang, 2013).

Hunres o genes have been stuie or their possible association with preeclampsia (Buurma, 2013; Sakowicz, 2016;

War, 2015). However, because o the complex phenotypic

expression o preeclampsia, it is oubtul that any one cani-

ate gene will be oun responsible. Last, a preeclampsia pre-

isposition has also been linke to genes o the etus (Burton,

2019; Gray, 2018; Leseva, 2020).

■ Stage II—Maternal Syndrome

Endothelial Cell Activation

Inammatory changes are believe to be a continuation o the

placental synrome. In response to ischemia or other inciting causes, placental actors are release an initiate a series

o events (Burton, 2019; Davige, 2015). Tus, antiangiogenic an metabolic actors an other inammatory leukocyte

meiators are thought to provoke the systemic endotheliopathy,

which is use synonymously here with endothelial cell activation or dysunction. Injury to systemic enothelial cells is seen

as a centerpiece o preeclampsia pathogenesis (Burton, 2019;

Phipps, 2019).

Cellular ysunction may result rom an extreme activate

state o leukocytes in the maternal circulation (Gervasi, 2001).

Briey, cytokines such as tumor necrosis actor α (NF-α) an

the interleukins may contribute to the systemic oxiative stress

associate with preeclampsia. Tis is characterize by generation o highly toxic oxygen raicals. Tese injure systemic vascular enothelial cells, lower nitric oxie prouction by these

cells, an interere with prostaglanin balance. Other sequelae

inclue prouction o the lipi-laen macrophage oam cells

seen in placental atherosis; activation o systemic microvascular coagulation, which is manieste by thrombocytopenia; an

greater systemic capillary permeability, which is reecte by

eema an proteinuria.

Intact enothelium has anticoagulant properties. Also, systemic enothelial cells, by releasing nitric oxie, blunt the response

o vascular smooth muscle to agonists. Injure or activate

enothelial cells may prouce less nitric oxie an may secrete

substances that promote coagulation an vasopressor sensitivity.

Further evience o enothelial activation inclues changes in

glomerular capillary enothelial morphology, greater capillary

permeability, an elevate bloo concentrations o substances

associate with enothelial activations. Likely, multiple actors

in the plasma o preeclamptic women combine to exert these

vasoactive eects (Myers, 2007; Walsh, 2009).

Vasospasm and Hypertension

Vasospasm has long been associate with preeclampsia. Systemic enothelial activation causes vasospasm, which elevates

resistance to prouce hypertension. Concurrently, systemic

enothelial cell injury promotes interstitial leakage, an platelets an brinogen are eposite in the subenothelial space.

Enothelial junctional proteins are also isrupte, an the subenothelial region o resistance arteries unergoes ultrastructural change (Suzuki, 2003; Wang, 2002). Te much larger

venous circuit is similarly involve.

With iminishe bloo ow because o malistribution

rom vasospasm an interstitial leakage, ischemia o the surrouning tissues can cause necrosis, hemorrhage, an other

en-organ isturbances. One important clinical correlate to

these changes is the markely attenuate bloo volume seen in

women with severe preeclampsia (Zeeman, 2009).

Increased Pressor Responses

As iscusse in Chapter 4 (p. 53), pregnant women normally evelop reractoriness to inuse vasopressors (AbulKarim, 1961). Women with early preeclampsia, however,

have enhance vascular reactivity to inuse norepinephrine

an angiotensin II (Raab, 1956; alleo, 1968). Moreover,

increase sensitivity to angiotensin II clearly precees the

onset o gestational hypertension (Gant, 1974). Paraoxically,

women who evelop preterm preeclampsia have lower circulating levels o angiotensin II (Chase, 2017).

Numerous prostaglandins are thought to be central to preeclampsia synrome pathophysiology. Specically, the blunte

pressor response seen in normal pregnancy is at least partially

ue to iminishe vascular responsiveness meiate by enothelial prostaglanin synthesis. For example, compare with

normal pregnancy, enothelial prostacyclin (PGI2) prouction

is lower in preeclampsia. Tis action appears to be meiate by

phospholipase A2 (Davige, 2015). At the same time, thromboxane A2 secretion by platelets is increase, an the prostacyclin: thromboxane A2 ratio eclines. Te net result avors

greater sensitivity to inuse angiotensin II an vasoconstriction (Spitz, 1988). Tese changes appear as early as 22 weeks

in gravias who later evelop preeclampsia (Chavarria, 2003).

Nitric oxide is a potent vasoilator an is synthesize rom

1-arginine by enothelial cells. Inhibition o nitric oxie synthesis raises mean arterial pressure, lowers heart rate, an reverses

the pregnancy-inuce reractoriness to vasopressors. Nitric

oxie likely is the compoun that maintains the normal lowpressure vasoilate state characteristic o normal etoplacental

perusion (Myatt, 1992). Nitric oxie meiates the eects o

placental growth actor (PlGF) an vascular enothelial growth

actor (VEGF) in vitro (Zhang, 2017). Te eects o nitric

oxie prouction in preeclampsia are unclear. It appears that

the synrome is associate with ecrease enothelial nitric

oxie synthase expression an thus lower nitric oxie activity

(Davige, 2015).

Endothelins are 21-amino-aci pepties an potent vasoconstrictors. Enothelin l (E-1) is the primary isoorm prouce

by human enothelium (Karumanchi, 2016a). Plasma E-1

levels are elevate in normotensive pregnant women. Women

with preeclampsia have even higher levels, an these pepties

may meiate renal injury (Phipps, 2019). Woman with preeclampsia evelop unctional autoantiboies to the enothelin

an angiotensin II receptors (Buttrup, 2018). Interestingly,

treatment o preeclamptic women with magnesium sulate

lowers E-1 concentrations (Sagsoz, 2003). In animal stuies,

silenal reuces E-1 concentrations (Gillis, 2016).

Angiogenic and Antiangiogenic Factors

Placental vasculogenesis is evient by 21 ays ater conception. Te list o pro- an antiangiogenic substances involve

in placental vascular evelopment is extensive, an the VEGF

an angiopoietin amilies are the most stuie. Angiogenic

imbalance escribes excessive amounts o antiangiogenic actors, which are thought to be stimulate by worsening hypoxia

at the uteroplacental interace. rophoblast o women estine

to evelop preeclampsia overprouce at least two antiangiogenic pepties that enter the

maternal circulation (Karumanchi, 2016b).

First, soluble ms-like tyrosine

kinase 1 (sF1t-1) is a soluble

variant o the membrane-boun

receptor or VEGF. As epicte

in Figure 40-4, elevate maternal sFlt-1 levels inactivate an re-

uce circulating PlGF an VEGF

concentrations, leaing to enot helial ysunction (Phipps, 2019).

As shown in ata rom Myatt

an coworkers (2013), sFlt-1 levels

begin to rise in maternal serum

months beore preeclampsia is

evient (Fig. 40-5). Tese high

levels in the secon trimester are associate with a much higher

risk or preeclampsia evelopment (Haggerty, 2012; March,

2015). Tis elevation rom normal levels appears even sooner

with early-onset isease (Vatten, 2012). Tese actors are also

operative in pregnancies complicate by etal-growth restriction (Herraiz, 2012).

A secon antiangiogenic peptie, soluble endoglin (sEng),

inhibits various transorming growth actor beta (GF-β) iso-

orms rom bining to enothelial receptors (see Fig. 40-4).

Enoglin is one o these receptors. Decrease bining to enoglin iminishes enothelial nitric oxie-epenent vasoilation. Serum levels o sEng also begin to rise months beore

clinical preeclampsia evelops (Haggerty, 2012).

Simultaneously elevate levels o sFlt-1 an sEng are associate with more severe orms o preeclampsia (Phipps, 2019).

In one systematic review, thir-trimester increases in sFlt-1

levels an lower PlGF concentrations were oun to correlate with preeclampsia evelopment ater 25 weeks’ gestation

(Wimer, 2007). From another stuy, oubling o sFlt-1 an

sEng expression increase the preeclampsia risk by 39 an 74

percent, respectively (Haggerty, 2012). Te cause o placental

overprouction o antiangiogenic proteins remains an enigma.

Concentrations o the soluble orms are not higher in etal circulation or amnionic ui o preeclamptic women, an their

levels in maternal bloo issipate ater elivery (Sta, 2007).

Clinical use o antiangiogenic protein levels to preict an

iagnosis preeclampsia is being evaluate (p. 703). Moreover,

one preliminary report escribe therapeutic apheresis to

reuce sFlt-1 levels (Tahani, 2016).

PATHOPHYSIOLOGY

■ Cardiovascular System

Disturbances in the cariovascular system are common with

preeclampsia synrome. Tese are relate to: (1) greater cariac

aterloa impose by hypertension; (2) cariac preloa, which

is reuce by a pathologically iminishe volume expansion

uring pregnancy an which is increase by aministration o

intravenous crystalloi or oncotic solutions; an (3) enothelial

activation leaing to leakage o intravascular ui into the extracellular space.

Hemodynamic Changes and Cardiac Function

Te cariovascular aberrations o pregnancy-relate hypertensive isorers vary. Moiying actors inclue preeclampsia

severity, egree o hypertension, presence o unerlying chronic

isease, an the point in the clinical spectrum in which these are

stuie. In some women, these cariovascular changes may precee hypertension (Easterling, 1990; Khalil, 2012; Melchiorre,

2013). Nevertheless, with the clinical onset o preeclampsia,

cariac output eclines, ue at least in part to greater peripheral

resistance (Ferrazzi, 2018).

Myocardial Function

Serial echocariographic stuies ocument iastolic ysunction in up to 45 percent o women with preeclampsia (Guirguis,

2015; Vaught, 2018). With this ysunction, ventricles o not

properly relax an cannot ll appropriately. In some aecte

women, unctional ierences persist up to 4 years ater elivery

(Evans, 2011; Orabona, 2017). Diastolic ysunction stems

rom ventricular remoeling, which is a malaaptive response to

the increase aterloa o preeclampsia an aims to maintain normal contractility. High levels o antiangiogenic proteins may be

contributory (Shahul, 2016). In otherwise healthy gravias, these

changes are usually inconsequential. But in those with unerlying ventricular ysunction—or example, concentric ventricular

hypertrophy rom chronic hypertension—urther iastolic ys-

unction may cause cariogenic pulmonary eema (Warhana,

2018). Tis is iscusse urther in Chapters 50 (p. 883) an 52

(p. 916).

Ventricular Function

Despite the relatively high requency o iastolic ysunction

with preeclampsia, clinical cariac unction in most aecte

women is appropriate (Hibbar, 2015). In some preeclamptic women, high-sensitivity cariac troponin levels are slightly

elevate (Morton, 2018). With severe preeclampsia, aminoterminal pro–brain natriuretic peptie (N–pro-BNP) levels

are increase (Zachary, 2017).

Women with preeclampsia synrome usually have slightly

hyperynamic ventricular unction (Fig. 40-6). Both these an

normotensive pregnant women have a cariac output that is

appropriate or let-sie lling pressures. Tis pressure can be

altere by intravenous ui volumes. Tus, aggressive hyration results in overtly hyperdynamic ventricular unction. Tis is

accompanie by elevate pulmonary capillary wege pressures,

an pulmonary eema may evelop espite normal ventricular

unction. Tis is partly because o an alveolar enothelial-epithelial leak, an it is compoune by ecrease oncotic pressure

rom a low serum albumin concentration. In sum, aggressive

ui aministration to otherwise normal women with severe

preeclampsia substantially elevates normal let-sie lling

pressures an raises a physiologically normal cariac output to

hyperynamic levels.

■ Blood Volume

In those with eclampsia, hemoconcentration is a hallmark eature

(Pritchar, 1984). Data rom Zeeman an associates (2009)

show that normally expecte pregnancy bloo volume expansion is severely curtaile (Fig. 40-7). Women o average size have

a nonpregnant bloo volume o 3000 mL, an uring the last

several weeks o a normal pregnancy, this averages 4500 mL

(Chap. 4, p. 59). With eclampsia, however, much or all o the

anticipate 1500 mL excess is lost. Such hemoconcentration

results rom generalize vasospasm that ollows enothelial activation an then rom leakage o plasma into the interstitial

space. In women with preeclampsia—epening on its severity—hemoconcentration is usually not as marke.

Tese changes have substantial clinical consequences. Women

with severe hemoconcentration are unuly sensitive to bloo loss

at elivery that otherwise may be consiere normal. Vasospasm

an enothelial leakage o plasma persist or a variable time ater

elivery as the enothelium is restore to normal. As this takes

place, vasoconstriction reverses, an as the bloo volume reexpans, the hematocrit usually alls rom ilution. Importantly, a

substantive cause o this all in hematocrit requently is the blood loss

incurred at delivery.

■ Hematological Changes

Thrombocytopenia

Te platelet count is routinely measure in women with any

orm o gestational hypertension. Te requency an intensity

o thrombocytopenia vary an are epenent on the severity an uration o preeclampsia (Hellmann, 2007; Hupuczi,

2007). Overt thrombocytopenia—ene by a platelet count

<100,000/µL—reects severe isease (see able 40-2). In general, the lower the platelet count, the higher the likelihoo o

maternal an etal morbiity an mortality. In most cases, elivery is avisable because thrombocytopenia usually worsens.

Ater elivery, the platelet count may continue to ecline or

the rst ay or so. It then usually rises progressively to reach

a normal level within 3 to 5 ays. As iscusse later, in some

instances with HELLP synrome, the platelet count continues to all ater elivery. I this nair is elaye until 48 to 72

hours, preeclampsia synrome may be incorrectly attribute to

one o the thrombotic microangiopathies (Chap. 59, p. 1060).

Another platelet alteration is platelet activation an increase

α-egranulation. Tis leas to release o β-thromboglobulin

an actor 4 an to enhance platelet clearance (Kenny, 2015).

Platelet volume concomitantly increases as young platelets

are release (Bellos, 2018). Paraoxically, in most stuies, in

vitro platelet aggregation is reuce compare with the normal

increase that is characteristic o pregnancy. Tis likely is ue to

platelet “exhaustion” ollowing in-vivo activation. Although the

cause is unknown, immunological processes or simply platelet

eposition at sites o enothelial amage may be implicate.

Levels o platelet-boun an circulating platelet-binable

immunoglobulins are elevate, which suggests platelet surace

alterations. Abnormally low platelet levels o not evelop in

the etuses o women with preeclampsia espite severe maternal thrombocytopenia (Kenny, 2015; Pritchar, 1987). Tus,

thrombocytopenia in a hypertensive woman is not a etal inication or cesarean elivery.

Hemolysis

Severe preeclampsia is requently accompanie by hemolysis,

which maniests as elevate serum lactate ehyrogenase levels

an reuce haptoglobin levels (Burwick, 2018). Other evi-

ence comes rom schizocytosis, spherocytosis, an reticulocytosis in peripheral bloo (Cunningham, 1985; Pritchar, 1954,

1976). Re cell istribution with (RDW) reects variability

in the size o circulating re bloo cells, an RDW is higher

in preeclamptic women (Aam, 2019). Tese erangements

result in part rom microangiopathic hemolysis cause by enothelial isruption with platelet aherence an brin eposition.

Cunningham an coworkers (1995) postulate that erythrocyte morphological changes were partially cause by serum

lipi alterations. Relate, substantively ecrease long-chain

atty aci content is oun in erythrocytes o women with preeclampsia (Mackay, 2012).

Ater early reports o hemolysis an thrombocytopenia with

severe preeclampsia, escriptions were ae o abnormally

elevate serum liver transaminase levels that inicate hepatocellular necrosis (Chesley, 1978). Weinstein (1982) reerre to

this combination o events as the HELLP syndrome (p. 699).

Coagulation Changes

Subtle changes consistent with intravascular coagulation commonly are oun with preeclampsia an eclampsia (Cunningham,

2015; von Daelszen, 2018). Some inclue elevate actor VIII

consumption, increase levels o brinopepties A an B an o

d-imers, an reuce concentrations o the regulatory proteins—

antithrombin III an proteins C an S. Coagulation aberrations

generally are mil an selom clinically signicant (Kenny,

2015; Pritchar, 1984). Unless placental abruption is comorbi,

plasma brinogen levels o not ier remarkably rom levels

oun in normal pregnancy (Cunningham, 2015). As preeclampsia worsens, so o abnormal nings with thromboelastography,

which is escribe in Chapter 44 (p. 774) (Pisani-Conway,

2013). Despite these changes, routine laboratory assessments o

coagulation, such as prothrombin time (P), activate partial

thromboplastin time (aP), an plasma brinogen level, are

not require in the management o pregnancy-associate hypertensive isorers.

■ Endocrine and Hormonal Alterations

Plasma levels o renin, angiotensin II, aldosterone, deoxycorticosterone, an atrial natriuretic peptide (ANP) are substantively

augmente uring normal pregnancy. ANP is release uring atrial wall stretching rom bloo volume expansion, an it

respons to cariac contractility (Chap. 4, p. 65). Serum ANP

levels rise in pregnancy, an its secretion is urther enhance

in preeclampsia (Gu, 2018). Levels o its precursor—proatrial

natriuretic peptide—also are elevate in preeclampsia. Vasopressin

levels are similar in nonpregnant, in normally pregnant, an

in preeclamptic women, although its metabolic clearance is

elevate in the latter two (Dürr, 1999).

■ Fluid and Electrolyte Alterations

In women with severe preeclampsia, the volume o extracellular

fuid, which maniests as eema, is usually much greater than

that in normal pregnant women. Te mechanism responsible

or pathological ui retention is enothelial injury an subsequent extravasation o intravascular ui. Aecte women also

have reuce plasma oncotic pressure. Tis urther isplaces

intravascular ui into the surrouning interstitium. In women

with preeclampsia, electrolyte concentrations o not ier

appreciably rom those o normal pregnant women.

Following an eclamptic convulsion, the serum pH an

bicarbonate concentration are lowere ue to lactic aciosis an

compensatory respiratory loss o carbon ioxie. Te intensity

o aciosis relates to the amount o lactic aci prouce—

metabolic aciosis—an the rate at which carbon ioxie is

exhale—respiratory aciosis.

■ Kidney

During normal pregnancy, renal bloo ow an glomerular

ltration rate (GFR) rise appreciably (Chap. 4, p. 68). With

preeclampsia, several reversible physiological changes ensue.

O clinical importance, renal perusion an GFR are slightly

reuce. Most o the ecrement in GFR is rom increase renal

aerent arteriolar resistance that may be elevate up to veol

(Conra, 2015; Cornelis, 2011).

Morphological changes are characterize by glomerular endotheliosis, which blocks ltration (Phipps, 2019). Diminishe

ltration causes serum creatinine levels to rise to values seen

in nonpregnant iniviuals, that is, 1 mg/mL, an sometimes

higher. Acute kiney injury is iscusse subsequently.

Plasma uric aci concentration is typically elevate in preeclampsia. Te elevation excees that attributable to the reuce

GFR an likely is also ue to enhance tubular reabsorption

(Chesley, 1945). At the same time, preeclampsia is associate

with iminishe urinary excretion o calcium, perhaps because

o greater tubular reabsorption (auel, 1987).

Proteinuria

Detection o proteinuria helps to establish the iagnosis o

preeclampsia (see able 40-1). Abnormal protein excretion

is empirically ene by 24-hour urinary excretion exceeing

300 mg; a spot urine protein: creatinine ratio ≥0.3; or persistent protein values o 30 mg/L (1+ ipstick) in ranom urine

samples. Although worsening or nephrotic-range proteinuria

was in the past consiere by most to be a sign o severe isease, this oes not appear to be the case (American College o

Obstetricians an Gynecologists, 2013; Bartal, 2020).

Problematically, the optimal metho o establishing abnormal levels o either urine protein or albumin remains to be

ene. For a 24-hour quantitative specimen, the consensus threshol value is ≥300 mg/24 h (American College o

Obstetricians an Gynecologists, 2013; Bartal, 2020). Using

a urinary protein excretion threshol o 165 mg in a 12-hour

sample shows equivalent ecacy (Stout, 2015).

Determination o urinary protein: creatinine ratio may supplant the cumbersome 24-hour quantication (Morris, 2012).

In one systematic review, ranom urine protein:creatinine

ratios <130 to 150 mg/g, that is, 0.13 to 0.15, inicate a

low likelihoo o proteinuria exceeing 300 mg/ (Papanna,

2008). Ratios <0.08 or >1.19 have negative an positive pre-

ictive values o 86 an 96 percent, respectively (Stout, 2013).

However, mirange ratios, or example, 300 mg/g or 0.3, have

poor sensitivity an specicity. Any mirange ratio shoul be

repeate, an i persistent, a 24-hour urine collection or measurement o protein excretion shoul be consiere.

With urine ipstick assessment, results epen on urine

concentration an are notorious or alse-positive an -negative results. A concentrate urine specimen may show a ipstick

value o 1+ to 2+ in women who actually excrete <300 mg/.

Importantly, proteinuria may evelop late, an some women

may alreay be elivere or have ha an eclamptic convulsion

beore it appears. At presentation, 10 to 15 percent o women

with HELLP synrome o not have proteinuria (Sibai, 2004).

In another report, 17 percent o women with eclampsia i not

have proteinuria by the time o seizures (Zwart, 2008).

Anatomical Changes

Sheehan an Lynch (1973) requently oun microscopic

changes that were ientie at autopsy in the kineys o

eclamptic women. Glomeruli are enlarge by approximately 20

percent, they are “blooless,” an capillary loops variably are

ilate an contracte. Enothelial cells are swollen—terme

glomerular capillary endotheliosis (Spargo, 1959). Such swelling

may be severe enough to block or partially block the capillary

lumens (Fig. 40-8). Last, homogeneous subenothelial eposits

o proteins an brin-like material are seen (Hecht, 2017).

Enothelial swelling may result rom angiogenic protein

“withrawal.” Tis is cause by the complexing o ree angiogenic proteins with a compatible circulating antiangiogenic

protein receptor (see Fig. 40-4). Te angiogenic proteins are

crucial or poocyte health, an their inactivation leas to

poocyte ysunction an enothelial swelling (Conra, 2015;

Phipps, 2019). Eclampsia is characterize by greater excretion

o these epithelial poocytes (White, 2014).

Acute Kidney Injury

In one stuy, preeclampsia synrome cause acute kiney injury

(AKI) in 5 percent o patients an in 14 percent o those with

HELLP synrome (Novotny, 2020). With severe preeclampsia,

Roriguez an colleagues (2021) reporte AKI in 15 percent

o women in a stuy rom Parklan Hospital. In most, AKI

was stage 1. In another stuy o 72 women with preeclampsia an renal ailure, hal ha HELLP synrome, an a thir

ha placental abruption (Drakeley, 2002). In a review o 183

women with HELLP synrome, 5 percent ha AKI (Haa,

2000). O those with renal injury, hal ha placental abruption, an most ha postpartum hemorrhage. Abnormal renal

values usually begin to normalize 10 ays or later ater elivery

(Cornelis, 2011; Spaan, 2012). Although mil egrees o AKI

are encountere, clinically apparent acute tubular necrosis is

almost invariably inuce by comorbi hemorrhage an subsequent hypovolemia an hypotension (Chap. 43, p. 753).

o evaluate AKI etiology clinically, urine electrolytes may

be obtaine. Results with preeclampsia reect an intrarenal

cause. In most with preeclampsia, the urine soium concentration is elevate. Instea, changes that inicate a prerenal mechanism inclue increase urine osmolality, elevate urine: plasma

creatinine ratio, an low ractional excretion o soium.

In response to oliguria, soiumcontaining crystalloi temporarily improves urine output. However, rapi

inusions may cause clinically appar ent pulmonary eema (p. 695).

Intensive intravenous ui therapy

is not inicate as “treatment” or

women with preeclampsia an oliguria, unless urine output is iminishe

rom hemorrhage or ui loss rom

vomiting or ever. In nonpregnant iniviuals, intravenous saline inusions

have emonstrate a negative impact

on renal unction. However, at Parklan Hospital, transition rom Ringer

lactate to normal saline i not signi-

cantly impair renal unction in women

with preeclampsia (Yule, 2020).

■ Liver

Hepatic changes are common in

women with severe preeclampsia

synrome. Several gross an microscopic anatomical erangements lea to elevate serum hepatic transaminase levels. Tis

transaminitis inicates hepatocellular injury an is a marker or

severe preeclampsia. Values are selom more than 500 U/L,

but levels exceeing 2000 U/L have been reporte (Chap.

58, p. 1031). In general, serum concentrations inversely ollow

platelet levels, an they both usually return to normal levels

within 3 ays ater elivery.

Anatomical Changes

Regions o periportal hemorrhage in the liver periphery typiy the hepatic lesions o eclampsia (Hecht, 2017; Sheehan,

1973). Extensive involvement such as shown in Figure 40-9 is

unusual. Sheehan an Lynch (1973) escribe that some egree

o hepatic inarction accompanie hemorrhage in almost hal o

women who ie with eclampsia. Tese nings correspone

with reports uring the 1960s that escribe elevate serum

hepatic transaminase levels. Pritchar an associates (1954)

escribe hemolysis an thrombocytopenia with eclampsia.

Tis constellation o hemolysis, hepatocellular necrosis, an

thrombocytopenia was later terme HELLP synrome. Similarities with hepatic sinusoial obstruction are reviewe by von

Salmuth an coworkers (2020).

Liver involvement with preeclampsia may clinically isplay

several maniestations. First, pain is consiere a sign o severe

isease. It typically maniests as moerate to severe right upper

quarant or miepigastric pain an tenerness. Tese women

usually have elevate serum aspartate transaminase (AS) or

alanine transaminase (AL) levels. In some cases, however,

the amount o hepatic tissue involve with inarction may be

surprisingly extensive yet still clinically insignicant. o stuy

this, we perorme magnetic resonance (MR) imaging in 16

women with HELLP synrome (Nelson, 2018). All but two

ha evience o acute liver injury, an the volume o involvement correlate with serum AS levels. Frank inarction is

unusual, an in our experiences, it may be worsene or precipitate by hypotension rom obstetrical hemorrhage. It occasionally causes hepatic ailure—also calle shock liver (Morgan,

2019; Yoshihara, 2016).

Hepatic Hematoma

In another presentation, periportal hemorrhage an inarction

may exten to evelop a hepatic hematoma. Tis in turn can

exten to orm a subcapsular hematoma that may rupture.

Compute tomography (C) scanning or MR imaging greatly

ais iagnosis (Fig. 40-10). Unrupture hematomas are probably more common than clinically suspecte an are more likely

to be oun with HELLP synrome (Nelson, 2018). Although

FIGURE 40-10 Abdominal CT imaging performed postpartum in

a woman with severe HELLP syndrome and right-upper quadrant

pain. A large subcapsular hematoma (asterisk) is seen confluent

with intrahepatic infarction and hematoma (arrowhead). Numerous

flame-shaped hemorrhages are seen at the hematoma interface

(arrows).

once consiere a surgical conition, current management o

a hepatic hematoma is usually observation unless bleeing is

ongoing. In some cases, however, prompt surgical intervention

or angiographic embolization may be liesaving (Chanrasekaran, 2020). In one review o 180 cases o hepatic hematoma or

rupture, 94 percent o aecte gravias ha HELLP synrome,

an in 90 percent o the total, the capsule ha rupture (VigilDe Gracia, 2012). Te maternal mortality rate was 22 percent,

an the perinatal mortality rate was 31 percent. Another review

o 73 cases oun similar outcomes (Gupta, 2021). In rare

cases, liver transplantation is necessary (Escobar Viarte, 2019).

Acute atty liver o pregnancy is sometimes conuse with

preeclampsia (Byrne, 2020; Nelson, 2013). It too has an onset

in late pregnancy, an oten hypertension, elevate serum

transaminase an creatinine levels, an thrombocytopenia

are comorbi. In istinction, the hallmark o acute atty liver

is marke liver ysunction. Liver unction overall is usually

normal in HELLP synrome. able 58-1 (p. 1031) highlights

these clinical ierences.

No convincing ata link pancreatic involvement with preeclampsia synrome. In 407 women with severe preeclampsia, the incience was 1 percent (Sang, 2019). Tat sai, the

occasional case o concurrent hemorrhagic pancreatitis is likely

unrelate (Lynch, 2015). In our experiences rom Parklan

Hospital, lipase an amylase levels are selom elevate in

women with preeclampsia (Nelson, 2018).

HELLP Syndrome

Tis acronym stans or hemolysis, elevate liver enzyme levels, an low platelet count. No strict enition o the syn-

rome is universally accepte, an thus its reporte incience

varies.

In women with preeclampsia, those with HELLP synrome

typically have worse outcomes than those without it (Martin,

2012, 2013). In the previously note stuy o 183 women with

HELLP synrome, 40 percent ha averse outcomes, an two

mothers ie (Haa, 2000). Complications inclue eclampsia

in 6 percent, placental abruption—10 percent, AKI—5 percent,

an pulmonary eema—10 percent. Stroke, hepatic hematoma,

coagulopathy, acute respiratory istress synrome, an sepsis were

other complications. In one review o 693 women with HELLP

synrome, 10 percent ha concurrent eclampsia (Keiser, 2011).

Obstetrical outcomes also may suer. In one stuy comparing

women with HELLP against those with preeclampsia, rates o

eclampsia were greater with HELLP—15 versus 4 percent; preterm birth—93 versus 78 percent; an perinatal mortality—9 versus 4 percent, respectively (Sep, 2009). Because o these marke

clinical ierences, some postulate that HELLP synrome has a

istinct pathogenesis (Reimer, 2013; Vaught, 2016).

■ Central Nervous System

Heaaches an visual symptoms are common with severe preeclampsia, an associate convulsions ene eclampsia. Te

earliest anatomical escriptions o brain involvement came

rom autopsy specimens, but C an MR imaging an Doppler stuies have ae important insights.

Neuroanatomical Lesions

From early anatomical escriptions, brain pathology accounte or only approximately a thir o

atal cases, such as the one shown in Figure 40-11.

In act, most eaths were rom pulmonary eema,

an brain lesions were coinciental. Tus, although

gross intracerebral hemorrhage was seen in up to 60

percent o eclamptic women, it was atal in only hal

o these (Richars, 1988). With ata rom Sheehan

an Lynch (1973) shown in Figure 40-12, cortical an subcortical petechial hemorrhages are other

principal lesions oun at autopsy in women with

eclampsia. Te classic microscopic vascular lesions

consist o brinoi necrosis o the arterial wall an

perivascular microinarcts an hemorrhages. Other

lesions inclue nonhemorrhagic areas o “sotening” throughout the brain, hemorrhages in the

white matter, an subcortical eema (Hecht, 2017;

Willar, 2018). Hemorrhage in the basal ganglia

or pons, oten with rupture into the ventricles, may

evelop.

Cerebrovascular Pathophysiology

Clinical, pathological, an neuroimaging nings have le

to two general theories to explain cerebral abnormalities with

eclampsia. Enothelial cell ysunction likely plays a key role

in both. Te rst theory suggests that in response to acute an

severe hypertension, cerebrovascular overregulation leas to

vasospasm an eventual tissue inarction (rommer, 1988).

Little objective evience supports this mechanism.

Te secon theory is that suen elevations in systemic

bloo pressure excee the normal cerebrovascular autoregulatory

capacity (Schwartz, 2000). Regions o orce vasoilation an

vasoconstriction evelop, especially in arterial bounary zones.

At the capillary level, isruption o en-capillary pressure causes

increase hyrostatic pressure, hyperperusion, an extravasation

o plasma an re cells through enothelial tight-junction openings. Tis leas to vasogenic edema.

Most likely, the true mechanism combines these two. Tus,

a preeclampsia-associate interenothelial cell leak evelops at

bloo pressure levels much lower than those that usually cause

vasogenic eema, an this is couple with a loss o upper-limit

autoregulation (Fugate, 2015; Zeeman, 2009). As shown in

Figure 40-13, these abnormalities maniest as the posterior

reversible encephalopathy syndrome (PRES). Lesions principally

involve the occipital an parietal cortices, but other areas

are oten involve, although less extensively (Elow, 2013;

Zeeman, 2004a).

Cerebral Blood Flow

Autoregulation is the mechanism by which cerebral bloo

ow remains relatively constant espite alterations in cerebral

perusion pressure. In nonpregnant iniviuals, this mechanism protects the brain rom hyperperusion when mean arterial pressures increase up to 160 mm Hg. Tese pressures are ar

greater than those seen in most women with eclampsia. Tus,

to explain eclamptic seizures, it was theorize that autoregulation must be altere by pregnancy. Some investigators have

shown impaire autoregulation in women with preeclampsia

(Bergman, 2021b; Janzarik, 2014). When stuie 2 to 3 years

postpartum, women who ha preeclampsia ha returne to

normal autoregulation (Janzarik, 2018).

Zeeman an associates (2003) showe that cerebral bloo

ow uring the rst two trimesters o normal pregnancy is similar to nonpregnant values. But uring the last trimester, ow

signicantly rops by 20 percent. Tey oun greater cerebral

bloo ow in this trimester in women with severe preeclampsia compare with ow in normotensive pregnant women (Lee,

2019; Zeeman, 2004b). aken together, these nings suggest

that eclampsia occurs when cerebral hyperperusion orces capillary ui interstitially because o enothelial amage. Tis leak

leas to perivascular eema. Some ata suggest that the bloo-

brain barrier is not impaire, but in-vitro stuies may inicate

increase permeability (Bergman, 2021a; Burwick, 2018).

Neurological Manifestations

Several neurological maniestations typiy the preeclampsia synrome. Each signies severe involvement an requires

immeiate attention.

First, headache and scotomata are thought to arise rom

cerebrovascular hyperperusion that has a preilection or the

occipital lobes. In women preceing an eclamptic convulsion,

up to 75 percent have heaaches, an 20 to 30 percent have

visual changes (Sibai, 2005; Zwart, 2008). Te heaaches vary

in severity an persistence. In our experiences, they are unique

in that they o not usually respon to traitional analgesia but

requently improve ater magnesium sulate inusion.

Convulsions are iagnostic or eclampsia. Tese are cause by

abnormal excessive or synchronous neural activity in the brain.

Evience suggests that extene seizures can cause signicant

brain injury an later brain ysunction.

Blindness an generalized cerebral edema are iscusse in subsequent sections. Last, women with eclampsia have been shown

to have some cognitive ecline when stuie 5 to 10 years ollowing the involve pregnancy (Bergman, 2021c). Tis is iscusse urther in Chapter 41 (p. 727).

Neuroimaging Studies

With C imaging, localize hypoense lesions are requently

seen with eclampsia at the gray- an white-matter junction an

primarily in the parietooccipital lobes. Frontal an inerior

temporal lobes, the basal ganglia, an thalamus are other sites

(Brown, 1988). Tese hypoense areas correspon to petechial

hemorrhages an local eema. Eema o the occipital lobes or

iuse cerebral eema may cause blinness, lethargy, an con-

usion (Cunningham, 2000). Wiesprea eema can appear

as marke compression or even obliteration o the cerebral

ventricles. Such women may evelop signs o impening liethreatening transtentorial herniation.

Several MR imaging acquisitions are use to stuy women

with eclampsia (Singh, 2021). Common nings are hyperintense 2 lesions in the subcortical an cortical regions o

the parietal an occipital lobes, which reect PRES (see Fig.

40-13). Te basal ganglia, brainstem, an cerebellum are other

involve sites (Brewer, 2013; Zeeman, 2004a). PRES lesions are

almost universal in women with eclampsia, an their incience

in women with severe preeclampsia approximates 20 percent

(Hosapatna Basavarajappa, 2020; Mayama, 2016). Although

usually reversible, a ourth o these hyperintense lesions with

eclampsia have restricte iusion that signiy cerebral inarctions. Tese have persistent MR imaging nings (Loureiro,

2003; Zeeman, 2004a).

Visual Changes and Blindness

Retinal artery an venular calibers are ecrease in women with

preeclampsia (Soma-Pillay, 2018). Tese changes, along with

visual cortex involvement, can cause scotomata, blurre vision,

or iplopia, which is common with severe preeclampsia an

eclampsia. Tese symptoms usually improve with magnesium

sulate therapy, or lowere bloo pressure, or both.

Blindness is rare with preeclampsia alone, but it complicates

up to 15 percent o women with eclampsia (Cunningham,

1995). It can evelop a week or more ollowing elivery. Blin-

ness is usually reversible an may arise rom three potential

areas. Tese are the occipital lobe’s visual cortex, the lateral

geniculate nuclei, an the retina.

Occipital blinness is also calle amaurosis. With MR imaging,

aecte women usually have evience o extensive occipital lobe

vasogenic eema. O 15 women care or at Parklan Hospital,

occipital blinness laste rom 4 hours to 8 ays, but it resolve

completely in all cases (Cunningham, 1995). Rarely, extensive

cerebral inarctions may result in total or partial visual eects.

In the retina, ischemia, inarction, or serous etachment

may occur (Hanor, 2014). Retinal inarction, terme Purtscher

retinopathy, is rare (Fig. 40-14). Serous retinal detachment is

usually unilateral an selom causes total visual loss. Asymptomatic serous retinal etachment is relatively common with

preeclampsia (Gupta, 2019). In most cases o eclampsia-associate blinness, visual acuity subsequently improves (Manura,

2021). I blinness is cause by retinal artery occlusion, vision

may be permanently impaire (Roos, 2012).

Cerebral Edema

Maniestations that suggest wiesprea cerebral eema are

worrisome. During 13 years at Parklan Hospital, 10 o 175

women with eclampsia were iagnose with symptomatic cerebral eema (Cunningham, 2000). Symptoms range rom lethargy, conusion, an blurre vision to obtunation an coma.

In most cases, symptoms waxe an wane. O these 10, three

became comatose an ha imaging nings o transtentorial

herniation. One woman ie.

Mental status changes generally correlate with the egree

o involvement seen with C an MR imaging stuies. Tese

women are very susceptible to sudden and severe blood pressure

elevations, which can acutely worsen the already widespread vasogenic edema. Tus, careul bloo pressure control is essential.

■ Uteroplacental Perfusion

Compromise uteroplacental perusion is almost certainly a major

contributor to the increase perinatal morbiity an mortality

rates associate with preeclampsia synrome (Harmon, 2015).

Contributing eects in enovascular trophoblastic invasion were

iscusse earlier (p. 692). Tus, measurement o uterine, intervillous, an placental bloo ow woul likely be inormative.

Attempts to assess these in humans have been hampere. Barriers

inclue the placenta’s inaccessibility, the complexity o its venous

efuent, an the nee or invasive techniques or raioisotopes.

As a surrogate, sonographic measurement o uterine artery

bloo ow velocity can estimate resistance to uteroplacental bloo ow. Vascular resistance is estimate by comparing

arterial systolic an iastolic velocity waveorms (Chap. 14, p.

262). By the completion o placentation, impeance to uterine

artery bloo ow is markely ecrease, but with abnormal placentation, abnormally high resistance persists (Everett, 2012;

Napolitano, 2012). In earlier stuies, peak systolic:iastolic

velocity ratios rom uterine an umbilical arteries in preeclamptic pregnancies were measure. In some cases, but not all, resistance was higher (Ferrazzi, 2018; ruinger, 1990).

Another Doppler waveorm, terme uterine artery “notching,” has been linke with increase risks or preeclampsia

or etal-growth restriction (Groom, 2009). However, in one

MFMU Network stuy, notching ha a low preictive value

except or early-onset, severe isease (Myatt, 2012a).

Resistance in uterine spiral arteries also has been measure.

In one stuy, mean resistance values were greater in all women

with preeclampsia compare with those in normotensive controls (Matijevic, 1999). Another stuy use MR imaging an

other techniques to assess placental perusion ex vivo in the

myometrial arteries remove rom women with preeclampsia

or etal-growth restriction (Ong, 2003). In both conitions,

myometrial arteries exhibite similar vascular responses.

Despite these nings, evience or compromise uteroplacental circulation is oun in only a ew women who later

evelop preeclampsia. Inee, when preeclampsia evelops

uring the thir trimester, only a thir o women with severe

isease have abnormal uterine artery velocimetry (Li, 2005). In

a stuy o 50 women with HELLP synrome, only a thir ha

abnormal uterine artery waveorms (Bush, 2001). In general,

the extent o abnormal waveorms correlates with severity o

etal involvement (Ghiini, 2008; Groom, 2009).

Tese sonographic nings have value or preiction o etalgrowth restriction but not preeclampsia (American College o

Obstetricians an Gynecologists, 2019b; Demers, 2019). Several other ow velocity waveorms have been investigate or

preeclampsia preiction. However, none is suitable or clinical

use (De Kat, 2019; ownsen, 2018).

Fetal-growth Restriction

Discusse in etail in Chapter 47, this potential consequence o

preeclampsia can serve as one severity inicator (see able 40-2).

Namely, poor growth is usually conne to etuses o women

estine to evelop severe preeclampsia (Mateus, 2019). Perry

an colleagues (2020) reporte that pregnancies complicate by

etal-growth restriction more likely ha maternal hemoynamic

inices similar to preeclampsia. Measures showe higher mean

arterial pressure, greater systemic vascular resistance, lower car-

iac output, an elevate uterine artery pulsatility inex. Fetuses

born to preeclamptic mothers have cariac remoeling similar

to growth-restricte etuses (Youse, 2020).

PREDICTION

Some biological markers implicate in the genesis o the preeclampsia synrome have been measure to help preict its

evelopment. Although most have been evaluate in the rst hal

o pregnancy, some have been teste as preictors o severity in

the thir trimester. Still others have been use to orecast recurrent preeclampsia. Overall, these eorts have resulte in testing

strategies with poor sensitivity an poor positive preictive values

or preeclampsia (Cone-Aguelo, 2015; De Kat, 2019). Currently, no screening tests or preeclampsia are predictably reliable,

valid, and economical.

Because combinations o tests an risk actors might be superior to single preictors, some have evelope multivariable

screening algorithms (Boutin, 2021; Brunelli, 2020; Copel, 2020;

Serra, 2020; Snell, 2020). One rst-trimester screening protocol

using serum sFlt-1 levels has been propose (Pihl, 2020). Other

examples are mipregnancy algorithms (Black, 2020; Peguero,

2021; Stepan, 2020). None has been aequately verie su-

ciently or wiesprea clinical use (Capriglione, 2020).

■ Vascular Resistance Testing

and Placental Perfusion

Most tests in this category are cumbersome, time consuming,

an inaccurate. o evaluate bloo pressure changes, three tests

assess the bloo pressure rise in response to a stimulus. In one,

women at 28 to 32 weeks’ gestation rest in the let lateral ecubitus position an then roll to lie supine. With this roll-over

test, rising bloo pressure in response to the maneuver signi-

es a positive test. Te isometric exercise test employs the same

principle by squeezing a hanball. Te angiotensin II inusion

test provies incrementally higher oses intravenously, an the

hypertensive response is quantie. Sensitivities o all three

tests range rom 55 to 70 percent, an specicities approximate 85 percent (Cone-Aguelo, 2015).

Uterine artery Doppler velocimetry is posite to reect aulty

trophoblastic invasion o the spiral arteries. Te poor preictive

value o this or preeclampsia was escribe in the Uteroplacental Perusion section (p. 702).

■ FetalPlacental Unit Endocrine Function

Several serum analytes have been propose to help preict preeclampsia. Newer ones are continually ae. In general, none

o these tests is clinically benecial or hypertension preiction.

■ Renal Function Tests

Hyperuricemia results rom reuce uric aci clearance cause

by iminishe glomerular ltration, increase tubular reabsorption, an ecrease secretion. In one stuy, the sensitivity

o serum uric aci levels to etect preeclampsia range rom 0

to 55 percent, an specicity was 77 to 95 percent (Cnossen,

2006). Tese are selom use to iagnose preeclampsia (Chescheir, 2019).

Isolate gestational proteinuria is a risk actor or preeclampsia (Jayaballa, 2015; Morgan, 2016). But, as a preictive test or

preeclampsia, microalbuminuria has sensitivities ranging rom

7 to 90 percent an specicities spanning 29 to 97 percent

(Cone-Aguelo, 2015).

■ Endothelial Dysfunction and Oxidative Stress

Enothelial activation an inammation are major participants

in preeclampsia pathophysiology. Levels o some implicate

compouns are elevate in the bloo o aecte women an

have been assesse as preictors.

Fibronectins are high-molecular-weight glycoproteins release

rom enothelial cells an extracellular matrix ollowing enothelial injury. In one systematic review, however, neither cellular

nor total bronectin levels were clinically useul to preict preeclampsia (Leeang, 2007).

Trombocytopenia an platelet dysunction are integral eatures o preeclampsia. Platelet activation causes their augmente

estruction an lower bloo concentrations. Platelet volume

is increase because o platelet immaturity, an platelet volume has been escribe to be an early preictor o preeclampsia (Bellos, 2019; Mayer-Pickel, 2021). Although markers o

coagulation activation escribe earlier (p. 696) are elevate,

they substantively overlap with levels in normotensive pregnant

women (von Daelszen, 2018).

O oxidative stress markers, higher levels o lipi peroxies

couple with ecrease antioxiant activity can be seen with

preeclampsia. Other markers are iron, transerrin, and erritin; resistin; hyperhomocysteinemia; blood lipids; an antioxi-

ants such as ascorbic acid an vitamin E (Christiansen, 2015;

Cone-Aguelo, 2015; Mackay, 2012; Mignini, 2005). However, none has sucient preictive value.

■ Angiogenic and Antiangiogenic

Factor Imbalance

An imbalance in angiogenic an antiangiogenic actors is convincingly linke to preeclampsia pathogenesis (p. 694). Serum

levels o VEGF an PlGF begin to rop beore clinical preeclampsia evelops. At the same time, levels o some antiangiogenic actors, such as sFlt-1 an sEng, begin to rise. Factor levels

an ratios ier signicantly between women with preeclampsia

an those who are normotensive. Tese show especially goo

preictive perormance with early-onset preeclampsia (Burton,

2019; Cereira, 2019; Phipps, 2019; Stepan, 2020).

Tese tests also can serve as iagnostic ajuncts (Duhig,

2019; Zeisler, 2016). First, they may ai ierentiating

between preeclampsia an mimics that inclue chronic hypertension, chronic kiney isease, systemic lupus erythematosus,

an immunological thrombocytopenia. Tese tests can also

help ierentiate mil an severe isease. Tese plus other

multiple markers will likely have a uture role in rst-trimester

preeclampsia screening (Sovio, 2019).

■ Other Markers

Cell-ree DNA (cDNA) o placental origin can be etecte in

maternal plasma (Chap. 16, p. 327). It is hypothesize that

cDNA is release in preeclampsia by accelerate apoptosis o

cytotrophoblasts. However, one MFMU Network stuy oun

no correlation between total cDNA levels an preeclampsia

preiction (Silver, 2017).

Other investigate markers inclue glycosylate hemoglobin A1c, serum cystatin-c, an rst-trimester estimate placental volume (Bellos, 2019; Cavero-Reono, 2018; Kim, 2021).

Proteomic, metabolomic, an transcriptomic technologies can

be employe to stuy serum an urinary proteins an cellular

metabolites. Preliminary stuies inicate their potential preictive value (Bahao-Singh, 2013; Ma, 2014).

PREVENTION

Various strategies use to prevent or moiy preeclampsia

severity have been evaluate. Some are liste in Table 40-4.

With the possible exception o aspirin, none is convincingly

an reproucibly eective.

■ Dietary and Lifestyle Modifications

A low-salt iet was one o the earliest researche preventions

but is not supporte by ata (De Snoo, 1937). O stuies, one

ranomize trial showe that a soium-restricte iet was ine-

ective in preventing preeclampsia (Knuist, 1998).

Regular exercise uring pregnancy is linke to a lower risk o

eveloping preeclampsia (Barakat, 2016; Morris, 2017). In one

systematic review, a similar tren towar risk reuction with

exercise was note (Kasawara, 2012). Only a ew stuies have

been ranomize.

Somewhat relate, one retrospective cohort stuy o 677

nonhypertensive women were hospitalize or be rest because

o threatene preterm elivery (Abenhaim, 2008). When outcomes o these women were compare with those o the general

obstetrical population, be rest was associate with a signi-

cantly reuce risk o eveloping preeclampsia (relative risk

0.27). From two small ranomize trials, prophylactic be rest

or 4 to 6 hours aily at home was successul in signicantly

lowering the preeclampsia incience in women with normal

bloo pressures (Meher, 2006). Currently, the Society or

Maternal-Fetal Meicine (2020) oes not recommen reuce

activity or women with hypertensive isorers or or prevention o preeclampsia.

Calcium supplementation has been stuie, but in one trial

o more than 4500 low-risk nulliparas, supplementation aile

to ecrease the risk or preeclampsia or pregnancy-associate

hypertension (Levine, 1997). Similar nings were reporte

rom another trial (Homeyer, 2019). In aggregate, most trials

show that unless women are calcium ecient, supplementation oers no benets (Palacios, 2019; Sanchez-Ramos, 2017).

Subclinical hypothyroidism is associate with increase preeclampsia risk. Tis insuciency has been postulate to stem

rom ioine eciency, but a recent metaanalysis oun no association between ioine suciency an preeclampsia (Businge,

2021). Folic acid was evaluate in a ranomize trial (Wen,

2018). Nearly 2500 high-risk women were given a 4-mg olic

aci ose aily or placebo. Te incience o preeclampsia in

both groups approximate 14 percent.

Cardioprotective atty acids oun in some sh likely prevent

inammation-meiate atherogenesis. Tus, it was posite that

they might also prevent preeclampsia. However, ranomize

trials conucte thus ar show no such benets rom sh oil

supplementation (Zhou, 2012). In one longituinal cohort

stuy, a seaoo iet, compare with a western one, provie a

protective eect against preeclampsia (Ikem, 2019).

■ Antihypertensive Drugs

Because o the prior purporte benets o soium restriction

or preeclampsia prevention, iuretic therapy became popular

with the avent o chlorothiazie in 1957 (Flowers, 1962). In

one metaanalysis o nine ranomize trials with more than

7000 pregnancies, women given iuretics ha a lower incience

o eema an hypertension but not o preeclampsia (Churchill,

2007). Because women with chronic hypertension are at high

risk or preeclampsia, several ranomize trials have evaluate

various antihypertensive rugs to reuce the incience o superimpose preeclampsia (Chap. 41, p. 713). A critical analysis o

these trials by Sta an coworkers (2015) aile to emonstrate

benets or this goal.

■ Antioxidants

Data imply that an imbalance between oxiant an antioxiant

activity plays a role in preeclampsia pathogenesis. Tus, naturally occurring antioxiants—vitamins C, D, an E—might

reuce such oxiation. Several ranomize stuies have assesse

antioxiant vitamin supplementation or women at high risk

or preeclampsia (Burton, 2019; Villar, 2009). Te Combine

Antioxiant an Preeclampsia Preiction Stuies (CAPPS) by

the MFMU Network inclue almost 10,000 low-risk nulliparas (Roberts, 2010). None o these stuies showe reuce

preeclampsia rates in women supplemente with vitamins C

an E compare with those given placebo.

Statins were propose to prevent preeclampsia because they

stimulate heme oxygenase-1 expression, which inhibits sF1t-1

release. Early animal ata suggest that statins may prevent

hypertensive isorers o pregnancy (Lewis, 2017). Te MFMU

Network plans a ranomize trial to test pravastatin or prevention, an a pilot stuy is complete (Costantine, 2016).

Metormin inhibits hypoxic inducible actor 1α by lowering mitochonrial electron transport chain activity. It reuces

sFlt-1 an sEng activity an thus has potential to prevent

preeclampsia (Brownoot, 2016). In a preliminary stuy, pre-

iabetic women were given metormin or placebo throughout

pregnancy, an metormin-treate women ha a lower inci-

ence o severe preeclampsia (Racine, 2021). However, other

clinical stuies are lacking.

■ Antithrombotic Agents

Preeclampsia is characterize by vasospasm, enothelial cell

ysunction, inammation, an activation o platelets an the

coagulation–hemostasis system. Other sequelae inclue placental inarction an spiral artery thrombosis (Nelson, 2014).

Tus, antithrombotic agents have been evaluate to prevent

preeclampsia. Low-molecular-weight heparin has been stuie

in ranomize trials. In a subsequent metaanalysis using iniviual ata rom 963 women, the risk or recurrent preeclampsia, abruption, or etal-growth restriction was similar in women

receiving heparin or placebo (Roger, 2016).

Low-dose Aspirin

In low oses o 50 to 150 mg aily, aspirin eectively inhibits

platelet thromboxane A2 biosynthesis. It has minimal eects

on vascular prostacyclin prouction. Still, several clinical trials

have shown limite benets in preeclampsia prevention. In a

ranomize trial rom the MFMU Network, risks or averse

outcomes were not signicantly reuce with aspirin therapy

(Caritis, 1998). Tis stuy was ollowe by numerous similar

stuies an metaanalyses.

In another ranomize trial o more than 1600 women at

high risk or preterm preeclampsia, oral low-ose aspirin was

given aily rom 11 to 14 weeks’ gestation until 36 weeks to prevent recurrence (Rolnik, 2017). Te rate o preterm preeclampsia recurrence was 1.6 percent in the aspirin group compare

with 4.3 percent in the placebo arm. In a metaanalysis, Roberge

an colleagues (2017) oun that aspirin prophylaxis initiate

beore 16 weeks’ gestation was associate with a signicant risk

reuction—approximately 60 percent—o preeclampsia an

etal-growth restriction. At the same time, however, Meher

an associates (2017) perorme an iniviual participant ata

metaanalysis an reporte a much lower—approximately 10

percent—risk reuction. Eects were signicant whether therapy was initiate beore or ater 16 weeks’ gestation.

In a subsequent metaanalysis, Roberge an coworkers (2018)

oun that aspirin prophylaxis given starting ≤16 weeks’ gestation reuce the risk o preterm, but not term, preeclampsia.

urner an colleagues (2020) reporte that aspirin improve

some perinatal outcomes inepenent o eects on preeclampsia risk. But to the contrary, the review by Chaemsaithong an

coworkers (2020) oun no benets even when low-ose aspirin was given beore 11 weeks’ gestation.

Base on these ata, the U.S. Preventive Services ask Force

(2021) recommens low-ose aspirin prophylaxis or women

at high risk or preeclampsia. Te American College o Obstetricians an Gynecologists (2018, 2020) now recommens

low-ose aspirin be given between 12 an 28 weeks’ gestation

to help prevent preeclampsia in high-risk women. Caniates

inclue those with ≥1 o the ollowing: prior preeclampsia,

chronic hypertension, overt iabetes, renal isease, autoimmune isorers, an multietal gestation. Supplementation

may be considered or those with more than one o these qualities: nulliparous, age oler than 35 years, obese, amily history o preeclampsia, vulnerable socioemographics, an prior

low-birthweight or growth-restricte neonate. Tese results

have also raise the question as to whether all pregnant women

shoul be given aspirin (Ayala, 2019).

Low-dose aspirin coupled with heparin mitigates thrombotic sequelae in women with lupus anticoagulant (Chap.

62, p. 1116). Because o a similarly high prevalence o placental thrombotic lesions oun with severe preeclampsia,

trials have assesse the possible merits o such treatments

or women with prior preeclampsia. In two ranomize trials, women with a history o early-onset preeclampsia were

given aspirin alone or a regimen o enoxaparin plus aspirin

(Groom, 2017; Haa, 2016). Outcomes were similar.

Low-molecular-weight heparin, with or without aspirin, may

ecrease the risk or preeclampsia in high-risk women (e

Vries, 2012; Wang, 2020).

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