GebFra Science | Review

Cardiomyopathies and Congenital Heart Disease in Pregnancy

Kardiomyopathien und kongenitale Vitia in der Schwangerschaft

Authors
Mechthild Westhoff-Bleck 1, Denise Hilfiker-Kleiner 1, Sabine Pankuweit 2, Bernhard Schieffer 2

Affiliations of the underlying mechanisms. This review article concen-

1 Medizinische Hochschule Hannover, Molekulare Kardio- trates therefore on PPCM, which occurs either in the last
logie, Abteilung für Kardiologie und Angiologie, Hannover, month of pregnancy or in the first 6 months following delivery
Germany in women with previously healthy hearts. The global incidence
2 Klinik für Innere Medizin, Kardiologie, Angiologie is estimated today at roughly 1 : 1000 pregnancies. The condi-
und internistische Intensivmedizin, Klinikum der Philipps- tion is heterogeneous, ranging from mild disease to severe
Universität, Marburg, Germany acute heart failure with cardiogenic shock and sudden cardiac
death of the mother. Important risk factors are pregnancy-as-
Key words sociated hypertensive complications, multiple pregnancy and
peripartum cardiomyopathy, PPCM, pregnancy, congenital greater maternal age. The pathogenesis comprises cleavage,
heart defects induced by increased oxidative stress, of the lactation hor-
mone prolactin into a toxic hormone fragment that damages
Schlüsselwörter blood vessels, known as the 16-kDalton protein fragment. The
peripartale Kardiomyopathie, PPCM, Schwangerschaft, lactation-blocking drug bromocriptine prevents prolactin re-
angeborene Herzfehler lease and promotes healing of PPCM in combination with
pharmacological heart failure therapy; it appears to prevent
received 24. 7. 2018 recurrence in subsequent pregnancies. Uncomplicated preg-
revised 26. 10. 2018 nancy is possible in most patients with congenital heart dis-
accepted 27. 10. 2018 ease. The foetal complications include an increased abortion
rate, prematurity and smallness for gestational age, as well
Bibliography as an increased risk of cardiac malformations. The maternal
DOI [Link] risk comprises mainly arrhythmias, progressive heart failure
Geburtsh Frauenheilk 2018; 78: 1256–1261 © Georg Thieme and thrombembolic complications, with the risk of vessel dis-
Verlag KG Stuttgart · New York | ISSN 0016‑5751 section with a low mortality risk of < 1 % in the case of aorto-
pathies. Individual risk assessment and corresponding close
Correspondence monitoring of the pregnancy are required.
Prof. Dr. Bernhard Schieffer
Klinik für Innere Medizin, Kardiologie, Angiologie ZU SAM ME N FA SS UN G
und internistische Intensivmedizin,
Schwangerschaftsassoziierte Erkrankungen des kardiovasku-
Klinikum der Philipps-Universität, Marburg
lären Systems treten bei bis zu 10 % aller Schwangerschaften
Baldingerstraße, 35043 Marburg, Germany
mit zunehmender Inzidenz auf. Aufgrund der klinischen Dra-
[Link]@[Link]
matik des Erkrankungsverlaufes und der Identifizierung zu-
grunde liegender Mechanismen ist neben angeborenen Herz-
Deutsche Version unter:
fehlern oder vorbestehenden Kardiomyopathien der Mutter
[Link]
insbesondere die peripartale Kardiomyopathie (PPCM) in den
klinischen Fokus gerückt. Diese Übersichtsarbeit konzentriert
AB STR AC T
sich deshalb auf das Krankheitsbild der PPCM, die entweder
Pregnancy-associated diseases of the cardiovascular system im letzten Monat der Schwangerschaft oder in den ersten
occur in up to 10 % of all pregnancies and the incidence is in- 6 Monaten nach einer Entbindung bei zuvor herzgesunden
creasing. Besides congenital heart disease or pre-existing car- Frauen auftritt. Die weltweite Inzidenz wird heute auf ca.
diomyopathy in the mother, the clinical focus has moved es- 1 : 1000 Schwangerschaften geschätzt. Das Krankheitsbild ist
pecially to peripartum cardiomyopathy (PPCM) because of heterogen mit milden Verläufen bis hin zu schwerer akuter
the conditionʼs dramatic clinical course and the identification Herzinsuffizienz mit kardiogenem Schock und plötzlichem

1256 Westhoff-Bleck M et al. Cardiomyopathies and Congenital … Geburtsh Frauenheilk 2018; 78: 1256–1261
 Herztod der Mutter. Wichtige Risikofaktoren sind schwanger- angeborenen Herzfehlern sind Schwangerschaften komplika-
schaftsassoziierte hypertensive Komplikationen, Mehrlings- tionslos möglich. Zu den fetalen Komplikationen gehören ne-
schwangerschaften und höheres Alter der Mütter. Die Patho- ben einer erhöhten Abortrate, Früh- und Mangelgeburtlich-
genese beinhaltet eine durch vermehrten oxidativen Stress in- keit ein erhöhtes Risiko kardialer Fehlbildungen. Das mütter-
duzierte Spaltung des Stillhormons Prolaktin in ein blutgefäß- liche Risiko umfasst hauptsächlich Rhythmusstörungen, eine
schädigendes toxisches Hormonfragment (das sog.16-kDa- progrediente Herzinsuffizienz, thrombembolische Komplika-
Protein-Fragment). Bromocriptin, als Abstillmedikament, ver- tionen und bei Aortopathien das Risiko von Gefäßdissektionen
hindert die Freisetzung des Prolaktins und fördert in Kombi- mit einem geringen Mortalitätsrisiko von < 1 %. Erforderlich ist
nation mit einer medikamentösen Herzinsuffizienztherapie eine individuelle Risikoabschätzung und entsprechende eng-
die Heilung der PPCM und scheint Rezidive bei Folgeschwan- maschige Betreuung in der Schwangerschaft.
gerschaften zu verhindern. Bei den meisten Patientinnen mit

formations. Depending on the underlying disease, the cardiac

Introduction malformation risk is between 2.5 and up to 50 %.
Cardiomyopathies are defined, according to a position paper of The maternal risk comprises mainly arrhythmias, progressive
the European Society of Cardiology, as myocardial diseases char- heart failure and thrombembolic complications, with the risk of
acterised by structural or functional restrictions of the myocardi- vessel dissection in the case of aortopathies. Cohort studies re-
um in the absence of coronary heart disease, hypertension, valvu- port a low mortality risk of < 1 % [9]. However, only a small per-
lar disease or congenital cardiac disease that could explain the centage of high-risk patients were included so the risks of preg-
dysfunction [1]. Based on their specific morphological and func- nancy and delivery may be underestimated particularly in these
tional phenotypes, the cardiomyopathies are divided into hyper- patients.
trophic, dilated, arrhythmogenic and restrictive forms, which are
readily identifiable in clinical practice by diagnostic echocardiog-
raphy. Every form or entity can be due to a familial form because
Peripartum Cardiomyopathy (PPCM)
of a potentially identifiable and often monogenetic change, or can Pregnancy-associated diseases of the cardiovascular system affect
have other non-familial and non-genetic causes. up to 10 % of all pregnancies and the incidence is increasing. The
Dilated cardiomyopathy is characterised by dilation of the left increasing obesity in the population, greater maternal age and in-
ventricle with concomitant impairment of function, and a familial creased multiple pregnancies appear to be promoting this trend.
form, usually due to autosomal dominant mutations in genes of Besides bleeding complications, cardiomyopathies in pregnancy
proteins of the cytoskeleton, the sarcomere, the nuclear mem- and in the peripartum period are today still among the most fre-
brane or the intercalated discs, can be found in approximately quent causes of peripartum morbidity and mortality. Peripartum
25 % of all patients. Non-familial forms can be caused by malnutri- cardiomyopathy (PPCM), heart failure that can occur both acutely
tion or by infectious, autoimmune or toxic causes (alcohol and and gradually shortly before or after delivery, represents a high
chemotherapeutic agents). risk for sustained damage to the young motherʼs health, and may
In 2008, pregnancy or peripartum cardiomyopathy (PPCM) cause death [2, 12, 13].
was classified by Elliot et al. as a separate idiopathic, non-familial
and non-genetic cardiomyopathy. It is therefore a cardiomyopa- Incidence
thy that is characterised by left ventricular systolic dysfunction The incidence of PPCM is estimated globally at approximately
(< 45 %), which occurs at the end of pregnancy or in the months 1 : 1000 pregnancies. There are no reliable figures in Germany
following delivery [2, 3]. Left ventricular dilation can but does not but estimates assume a prevalence of 1 : 1500–2000 births [12].
have to be detectable at the same time, but other causes of car- PPCM is defined through a diagnosis of exclusion; in the position
diac dysfunction must be excluded and there must be a temporal paper of the European Society of Cardiology from this year, which
association with pregnancy. Diagnosis of peripartum cardiomyop- can be accessed at [Link] [2], the definition states:
athy is therefore a diagnosis of exclusion. “PPCM is an idiopathic cardiomyopathy that occurs in the last
Due to advances in the treatment of congenital heart defects, a month of pregnancy or in the months following delivery or termi-
large number of women reach child-bearing age even with com- nation of pregnancy in women with previously healthy hearts”.
plex congenital heart defects. Congenital heart disease now rep- The only diagnostic criterion is the left ventricular ejection frac-
resents the largest number of pregnant women with structural tion (LVEF) measured by echocardiography or MRI, which should
heart disease accounting for over 60–80 % [4]. Congenital heart be ≤ 45 % [2]. Congenital or previously diagnosed heart disease
diseases are a non-homogeneous group that includes simple de- such as chemotherapy-induced myocarditis, genetically caused
fects with a low pregnancy-associated risk and complex lesions cardiomyopathies or known dilated cardiomyopathies exclude
with a significant complication rate [4 – 11]. The foetal complica- PPCM.
tions include an increased abortion rate, prematurity and small-
ness for gestational age, as well as an increased risk of cardiac mal-

Westhoff-Bleck M et al. Cardiomyopathies and Congenital … Geburtsh Frauenheilk 2018; 78: 1256–1261 1257
 GebFra Science | Review

Risk factors tion recovers in patients with PPCM in most cases, while mortality
To date, the aetiology of PPCM is largely unknown. However, a se- of 5–10 % is described [16]. Infectious diseases, viral myocarditi-
ries of risk factors has been identified in the last few years. These des, autoimmune phenomena or major blood loss at delivery are
include, for example, pregnancy-associated hypertensive diseases suggested as other possible causes [12 – 14].
(pre-eclampsia, HELLP syndrome), which were found in nearly Experimental studies showed that increased peripartum oxida-
50 % of PPCM patients in Germany. Multiple pregnancy, multipar- tive stress leads to cleavage of the lactation hormone prolactin in-
ity and pregnancy at a later age, tocolysis and African and Afro- to an N-terminal 16 kDa prolactin fragment (also called vasoinhi-
American origin also appear to be associated with an increased bin), which then causes primary endogenous damage to the vas-
PPCM risk [12 – 14]. cular system through vasotoxic, proinflammatory and proapo-
ptotic effects [19]. The efficacy of bromocriptine, a blocker of pro-
Diagnosis lactin secretion, was successfully tested as treatment in a mouse
Early diagnosis is essential for a good chance of cure. The diagno- model of PPCM [19]. Since bromocriptine has long been used clin-
sis of PPCM is made more difficult by the fact that symptoms are ically as a drug to halt lactation, several attempts at treatment
often not clear and are sometimes difficult to distinguish from and a pilot study in South African patients followed, all of which
normal pregnancy symptoms such as oedema, shortness of showed very promising results [3]. A randomised multicentre
breath and lethargy [2, 15]. The ECG is often normal also. Nonspe- dose-finding study initiated by us was sponsored by the BMBF
cific chest symptoms, palpitations, nocturia and nausea or pulmo- and published last year [20]. This showed that, alongside basic
nary oedema with orthopnoea and tachypnoea as part of cardio- heart failure treatment (beta-blockers, ACE inhibitors), a week of
genic shock, as well as restlessness and agitation are likewise pos- bromocriptine in a dosage of at least 2.5 mg p. o. and thrombosis
sible. A delay in diagnosis and hence in the start of treatment con- prophylaxis sufficed to cure the majority of the patients and dras-
tributes significantly to long-term morbidity and mortality. More- tically reduce the high morbidity and mortality. This treatment
over, spontaneous recovery is common in milder disease but the concept was adopted in the current guidelines as the BOARD
risk of recurrence with a severe disease course in a further preg- treatment regimen (Bromocriptine, Oral heart failure therapies,
nancy is markedly increased [12, 16, 17]. When making the diag- Anticoagulation, vasoRelaxing agents, and Diuretics) [21]. It is im-
nosis, it is important to measure biomarkers of cardiac damage/ portant that this treatment concept can only be used after deliv-
heart failure. Besides the recommended transthoracic echocardi- ery. In patients who become symptomatic in the last month of
ography, laboratory measurement of natriuretic peptide (BNP or pregnancy, delivery should take place as soon as possible, and an
NT-proBNP) as soon as (pregnancy-associated) heart failure is sus- interdisciplinary team of obstetricians, anaesthetists, cardiolo-
pected has proved to be a useful guide and should be measured gists, cardiac surgeons and neonatologists should be on site. Fol-
early in a suspected case [18]. Measurement of troponin T (or tro- lowing delivery, the BOARD regime should be started, depending
ponin I) as well as determination of inflammatory markers (CRP, on the patientʼs haemodynamic stability.
PCT, leucocyte count) helps to distinguish it from, for instance,
myocarditis. Cardiac MRI should be performed in addition to fol- Treatment
low-up echocardiography for differential diagnosis. A 12-channel With early diagnosis and treatment in accordance with guidelines,
ECG is also recommended for prompt detection of possible over 60 % of patients recover completely within the first 12 months
changes and an increased risk for ventricular arrhythmias. and a further 47 % recover partially (i.e., improvement in left heart
pump function [left ventricular ejection fraction, LV‑EF] by at least
Pathogenesis 10 % and at least one heart failure class [New York Heart Associa-
The pathogenesis of PPCM is incompletely understood. It has tion, NYHA class]), and only about 3 % remain in heart failure [20].
been argued that it might be a vascular disease triggered by the Even if these patients recover clinically and also echocardio-
hormonal milieu and oxidative stress in late pregnancy or the graphically, an increased long-term risk for sudden cardiac death
early post-delivery period. However, it remains unclear why only unfortunately persists so that a defibrillator vest or possibly an
a small number of pregnant women develop any symptoms of implantable defibrillator (ICD) must be considered in high-risk
PPCM at all. Based on the aetiology of dilated cardiomyopathy, in- patients even when cardiac function has recovered completely
flammatory or genetic factors have also been postulated in the [22].
pathogenesis of PPCM, but analysis of cardiac muscle biopsies It is vitally important during intensive treatment that the fre-
has yielded few indicative results. Only one genetic study in a quently used stress hormones (adrenalin, dobutamine) are not
study population of 172 patients with PPCM of mutations in pro- used for intensive therapy as these can harm the patients and
teins that are associated with dilated cardiomyopathy suggests a even cause irreversible terminal cardiac damage [23].
genetic predisposition in 15 % of the patients, the majority of
which involve changes in a structural protein named titin [15]. It Further pregnancies
can be assumed with certainty, however, that other exogenous Further pregnancies are associated with a high risk of recurrence
and/or genetic factors are involved in the development of PPCM and patients should be counselled about this [16]. A subsequent
since patients with PPCM and changes in the titin gene develop pregnancy is not impossible, however, but patients must be moni-
the disease at an earlier time than patients with dilated cardiomy- tored closely and delivery should take place in an experienced
opathy and demonstrate normal left ventricular function again centre with the interdisciplinary collaboration of cardiology,
much more often subsequently. However, left ventricular func- anaesthesia, neonatology and gynaecology/obstetrics [17]. The

1258 Westhoff-Bleck M et al. Cardiomyopathies and Congenital … Geburtsh Frauenheilk 2018; 78: 1256–1261
recommendations on medication for further planned pregnancy
are in accordance with the guidelines [24]. It is important that
Congenital Heart Disease in Pregnancy
ACE inhibitors are contraindicated during the first trimester be-
cause of their teratogenic effect; they are not a first-line treat- Antenatal care
ment later in the pregnancy but can be used. The AT antagonists Patients with congenital heart disease require individual counsel-
(angiotensin II type 1 receptor antagonists), mineralocorticoid re- ling prior to pregnancy about the risks for mother and baby. This
ceptor antagonists or ivabradine are contraindicated during preg- enables cardiac defects requiring treatment to be managed be-
nancy and lactation. Tapering and finally discontinuation of this fore pregnancy. The patient should be aware of high risks so that
medication are therefore necessary before the start of a further she can decide against pregnancy if appropriate. On the other
pregnancy. Beta-blockers may and should be continued during hand, she must also be conscious of the medical consequences in-
pregnancy (in Germany, only metoprolol is licensed during preg- cluding the need for close monitoring during pregnancy.
nancy). Diuretics should be used restrictively and only when With uncomplicated congenital heart defects, a single cardiol-
clearly indicated (obvious fluid retention, pulmonary congestion); ogy review to determine cardiac status often suffices, while all
a pro-diabetogenic effect has been described for thiazide diuret- other patients should have one clinical review per trimester. Re-
ics. The dosage should be adjusted because of their influence on view of high-risk patients is necessary at intervals of 1–4 weeks
placental perfusion and possible development of oligohydram- from 18–20 weeks, depending on the risk profile and clinical pic-
nios. If an unplanned pregnancy occurs, the contraindicated ture as the haemodynamic stress increases. Organ screening ul-
drugs should be discontinued immediately as they can harm the trasound of the baby should be performed in all pregnant women
embryo. An initial cardiology review with echocardiography, clini- at 20–24 weeks.
cal examination and measurement of natriuretic peptides is rec- Pregnancies with significant cardiac risks, especially when
ommended when pregnancy is diagnosed, at 4-week intervals high-risk, need referral to a maximum-care hospital with expertise
from 20 weeks of gestation and at 2-week intervals after in the treatment of congenital heart defects. Close interdiscipli-
30 weeks. In addition, close gynaecological and obstetric atten- nary collaboration comprising joint cardiological and gynaecolog-
dance is recommended for prompt detection of potential preg- ical care from the start is required.
nancy complications. Close monitoring (echo, even weekly if nec- Individual risk assessment is based on the underlying congeni-
essary) is necessary in the last two months of pregnancy so that tal condition, the cardiac lesions and clinical symptoms [9, 11, 24,
delivery can be initiated if there is clinical deterioration. The deliv- 25]. When assessing the overall situation, the biomarker NT-
ery should take place in an experienced centre with interdiscipli- proBNP can provide additional valuable information as NT‑pro
nary collaboration between cardiology, gynaecology/obstetrics, BNP < 128 pg/ml in the 20th week of pregnancy was detected as
anaesthesia and neonatology. Depending on the clinical status, an independent predictor of cardiac complications.
LV‑EF (left ventricular ejection fraction) and concomitant dis-
eases, the delivery modality should also be considered (vaginal Prevention of complications
delivery versus section). Vaginal delivery is possible in stable pa- Patients with congenital heart disease have evidence of post-
tients, but elective caesarean section should be discussed with thrombotic syndrome in up to 60 % of cases, necessitating the
the patient if there are feared cardiological complications. Phar- use of compression hose [26 – 28]. Iron deficiency anaemia should
macological inhibition of lactation by means of bromocriptine is also be treated promptly (Hb < 11.5 g/dl).
recommended following delivery, with resumption of the oral Cardiac lesions such as severe aortic or mitral stenosis, native
heart failure medication, regardless of clinical symptoms and aortic isthmus stenosis and significant aneurysms of the aorta
LVEF. Outpatient cardiological review following delivery is strongly due to aortopathies should be treated interventionally or surgi-
recommended [25]. cally before a pregnancy.
Treatment of arterial hypertension is essential to reduce the
Long-term management risk of aortic complications; an average blood pressure of less
For the long-term management of PPCM patients, annual review than 120–130 mmHg is desirable and blood pressure peaks
is important, when medications can be checked and adjusted if should be avoided. Beta-blocker therapy is regarded as basic
necessary. Advice on family planning and contraception should treatment in Marfan syndrome.
be provided in every case. Hormone-free contraceptive methods
(copper IUD, condoms) should be suggested, and a hormonal Cardiac complications
IUD containing levonorgestrel or an oral monopreparation con- The most frequent complications that occur include arrhythmias,
taining desogestrel may be possible in women with heart failure. progressive heart failure symptoms and thrombembolic compli-
On the other hand, patients should be advised explicitly against cations.
oestrogen-containing preparations as there can be potentially Supraventricular tachycardias must be terminated as soon as
negative interactions with the heart failure therapy and there is possible as progressive heart failure symptoms with worsening of
an increased thrombosis risk. ventricular function are possible with sustained tachycardia. De-
pending on the existing status, prophylactic beta-blocker therapy
and intermittent or long-term anticoagulation are required subse-
quently [24]. If there is no spontaneous conversion to sinus
rhythm or haemodynamic instability is present, cardioversion is

Westhoff-Bleck M et al. Cardiomyopathies and Congenital … Geburtsh Frauenheilk 2018; 78: 1256–1261 1259
 GebFra Science | Review

indicated. Trans-oesophageal echocardiography is required to ex- Delivery

clude intracardiac thrombus production with more prolonged In most pregnancies, transvaginal delivery under epidural anaes-
supraventricular tachycardia. This necessitates an adequate fast- thesia is possible, with a shortened second expulsive stage if ap-
ing period because of the delayed gastric emptying during preg- propriate. From the cardiological point of view, planning a primary
nancy. section should be limited to high-risk patients. These are patients
Digitalis glycosides and adenosine can be used safely during who are not able to deal with the up to 60 % increase in cardiac
pregnancy. The data are weak for specific antiarrhythmic agents output during labour or who have aortic aneurysms with a high
(quinidine, procainide, flecainide, sotalol) but there are no known dissection risk. The use of ECLS therapy should be considered early
significant teratogenic risks. Use of amiodarone requires monitor- in the case of haemodynamic instability or severely impaired ven-
ing of thyroid function [11]. tricular function.
If heart failure symptoms occur, beta-blocker therapy is recom-
mended initially. Treatment escalation with diuretics (hydrochlo-
rothiazide, furosemide, spironolactone) is required if cardiac oe-
Summary
dema or pulmonary congestion occurs. Even if foetal side effects PPCM is a life-threatening disease that occurs in women with pre-
are possible (bradycardia, reduced foetal growth), this medication viously healthy hearts. Since the symptoms cannot be clearly dis-
should not be withheld from pregnant women when indicated but tinguished from other pregnancy-induced conditions or infec-
the foetal status should be monitored since improvement of the tious diseases, cardiological investigation with echocardiography
heart failure symptoms with optimisation of maternal haemody- should always be performed in suspected cases. NT-proBNP is a
namics improves foetal development and likelihood of survival. suitable diagnostic marker, while classic cardiac enzymes do not
In the case of progressive heart failure, an interdisciplinary deci- allow indicative diagnosis of PPCM [12, 24]. Heart failure therapy
sion on how to proceed is necessary [15]. in accordance with the guidelines should then be started as soon
Thrombosis of a mechanical valve replacement is a life-threat- as possible [21]. Patients requiring intensive care or with a rapidly
ening complication. There is currently no generally accepted con- progressive disease course with cardiogenic shock should be
cept of anticoagulation treatment during pregnancy [6]. Vitamin transferred promptly to experienced centres that can provide ex-
K antagonists (observing a maximum dose) and fractionated hep- tracorporeal life support (ECLS). Central bed allocation pro-
arins are used, and close monitoring of the anti-Xa level is essen- grammes such as IVENA, which is used in the German state of
tial (target 0.6–1.2 IU/ml depending on valve type/risk factors) [6, Hesse, can be utilised. At the University Hospital in Marburg,
24]. In this case, anticoagulation is a balancing act between avoid- these patients receive interdisciplinary intensive care in the ECLS
ance of thrombosis, bleeding complications and foetal malforma- unit of the cardiology intensive care unit. Since PPCM has a very
tion risks. The risk of valve thrombosis is higher on heparin ther- good recovery rate but is also associated with a sustained in-
apy but the foetal malformation risk is lower. Hospital admission creased risk of recurrence, especially in subsequent pregnancies,
is essential if valve thrombosis occurs. Intravenous heparin ther- and an increased risk of sudden cardiac death, PPCM should be
apy can be attempted initially (target PTT 60–80 s). If this is un- followed by close cardiological follow-up and possibly defibrillator
successful or the patient is haemodynamically unstable, lysis or implantation.
surgical valve replacement is required. Uncomplicated pregnancy is possible in most patients with
Peripartum aortic dissection occurs in aortopathy or Marfan congenital heart defects. Individual risk assessment is required,
syndrome. Bicuspid aortic valves (increased risk with an aortic di- with corresponding close monitoring in pregnancy.
ameter > 50 mm) and Marfan syndrome (increased risk with aortic High-risk pregnancies require close interdisciplinary collabora-
diameter > 45 mm) represent the most frequent congenital dis- tion between gynaecologists and cardiologists. Patients should
eases associated with this complication. It should be noted that attend an expert centre even in early pregnancy. Pregnancies can
the risk of dissection is not limited to the delivery period only but be maintained longer by optimising the required cardiac treat-
is still markedly increased for a week post partum. ment through close cardiological monitoring to detect increasing
Fixed severe pulmonary hypertension due to Eisenmenger syn- cardiac problems, or delivery can be planned before cardiac de-
drome continues to be associated with high maternal morbidity compensation. When the mortality risk is high, delivery with ECLS
and mortality. Medication with phosphodiesterase-5 inhibitors/ available on standby can improve the prognosis.
prostacyclines is licensed as specific therapy. Management of
these patients requires close interdisciplinary collaboration from Conflict of Interest
the start. The haemodynamic stress is poorly tolerated even in
the second trimester, which leads to reduced placental perfusion. The authors declare that they have no conflict of interest.
Moreover, saturation of less than 90 % is associated with a high
early abortion rate.
If cardiac deterioration occurs, lung maturation should be initi-
ated promptly and caesarean section should be planned.

1260 Westhoff-Bleck M et al. Cardiomyopathies and Congenital … Geburtsh Frauenheilk 2018; 78: 1256–1261
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