Ebstein’s anomaly and pregnancy

Ebstein’s anomaly and pregnancy

Ebstein’s anomaly of tricuspid valve was first described by Wilhelm Ebstein in 1866 [1]. It is characterized by distal displacement of the septal and posterior leaflets of tricuspid valve. Anterior leaflet is elongated and sail like. A portion of the right ventricle is ‘atrialized’ due to the distal displacement of the tricuspid valve. Right atrium is often grossly dilated. Right to left shunting occurs across a patent foramen ovale producing cyanosis of variable extent. Ebstein’s anomaly is one of the cyanotic congenital heart diseases in which survival to adulthood is common.

Several studies have evaluated the outcome of pregnancy in Ebstein’s anomaly. Donnelly JE et al reported on 42 pregnancies in 12 women with Ebstein’s anomaly. Pregnancy was well tolerated in those who did not have cyanosis or arrhythmia. Though the neonatal outcome was generally good, there was increased risk of prematurity and dysmaturity in babies born to mothers with cyanosis. The authors concluded that maternal cyanosis or arrhythmia were indications for closer maternal and fetal observation.

Another study by Connolly HM et al from Mayo Clinic reported on 111 pregnancies in 44 women with Ebstein’s anomaly [2]. 89% of the deliveries were vaginal delivery and 11% were cesarean section and 76% were live births. Birth weight of infants born to cyanotic patients were significantly lower. The study had also checked on the offspring of males with Ebstein’s anomaly. It was found that 6% of offspring of women with Ebstein’s anomaly had congenital heart disease while 1% of offspring of men with Ebstein’s anomaly had congenital heart disease. This group of authors also concluded that pregnancy is well tolerated in women with Ebstein’s anomaly. In their study, there were no serious pregnancy related maternal complications like maternal death, stroke, congestive heart failure, arrhythmia or endocarditis.

While the above two studies were in 1990s, a report from Japan in 2018 included 17 women with Ebstein’s anomaly who delivered between 1995 -2015 [4]. Delivery was by cesarean section in 8 women including 9 pregnancies, while 9 women including 14 pregnancies underwent vaginal delivery. Elective cesarean section was done in those with significant heart failure or arrhythmias and when two or more of the following criteria were present: cardiothoracic ratio 60% or more, moderate or severe tricuspid regurgitation, tricuspid regurgitation jet pressure gradient 35 mm Hg or more during pregnancy. They found that cardiothoracic ratio and tricuspid regurgitation pressure gradient increased during pregnancy. NYHA (New York Heart Association) functional class deteriorated from class I to class III or II in five cases during pregnancy. The authors advised monitoring of clinical and hemodynamic changes throughout pregnancy to minimize maternal cardiac risk and for selection of mode of delivery.

Another previous report had 13 patients with Ebstein’s anomaly having 27 pregnancies and 21 live births from 1985 to 2011 [5]. Four of the patients had associated atrial septal defect and 6 had associated Wolff-Parkinson-White syndrome. Two patients had undergone closure of atrial septal defect and one had undergone tricuspid valve replacement prior to pregnancy. In 17 pregnancies, patients remained in NYHA class I and had full term vaginal delivery. Cesarean sections were done in three. 20 of the 21 babies had good neonatal prognosis without congenital heart disease. One died due to prematurity. There were 6 spontaneous abortions in this series. Authors concluded that maternal and fetal outcomes were good in patients with Ebstein’s anomaly and NYHA class I. Tachyarrhythmia and cardiac failure have to be looked for in pregnancy.

A smaller study had 8 pregnancies in 4 women with Ebstein’s anomaly, all resulting in vaginal delivery [6]. Two babies were delivered prematurely. One patient had right heart failure during early pregnancy. Another had arrhythmia during labour, which was managed medically. Another patient had arrhythmia in post partum period, which was also managed medically. Authors mentioned that when a woman with Ebstein’s anomaly reaches child bearing age, fertility is not affected even in cyanotic women. They noted that pregnancy outcome is usually favourable under close supervision of obstetrician and cardiologist.

In addition to these studies, there are several single case reports of successful pregnancies in women with Ebstein’s anomaly in literature, though the potential risks mentioned above have to be borne in mind. Careful monitoring of maternal and fetal status is needed. Pregnancies in those with cyanosis, arrhythmia and heart failure carry a higher maternal and fetal risk.

References

1. Ebstein W. Über einen sehr seltenen Fall von Insufficienz der Valvula tricuspidalis, bedingt durch eine angeborene hochgradige Miβbildung derselben [in German]. Archiv für Anatomie, Physiologie und wissenschaftliche Medicin 1866; 2: 238– 54. Cited in: Mazurak M, Kusa J. The Two Anomalies of Wilhelm Ebstein. Tex Heart Inst J. 2017 Jun 1;44(3):198-201.
2. Donnelly JE, Brown JM, Radford DJ. Pregnancy outcome and Ebstein’s anomaly. Br Heart J. 1991 Nov;66(5):368-71.
3. Connolly HM, Warnes CA. Ebstein’s anomaly: outcome of pregnancy. J Am Coll Cardiol. 1994 Apr;23(5):1194-8.
4. Kanoh M, Inai K, Shinohara T, Shimada E, Shimizu M, Tomimatsu H, Ogawa M, Nakanishi T. Influence of pregnancy on cardiac function and hemodynamics in women with Ebstein’s anomaly. Acta Obstet Gynecol Scand. 2018 Aug;97(8):1025-1031.
5. Katsuragi S, Kamiya C, Yamanaka K, Neki R, Miyoshi T, Iwanaga N, Horiuchi C, Tanaka H, Yoshimatsu J, Niwa K, Ikeda T. Risk factors for maternal and fetal outcome in pregnancy complicated by Ebstein anomaly. Am J Obstet Gynecol. 2013 Nov;209(5):452.e1-6.
6. Chopra S, Suri V, Aggarwal N, Rohilla M, Vijayvergiya R, Keepanasseril A. Ebstein’s anomaly in pregnancy: maternal and neonatal outcomes. J Obstet Gynaecol Res. 2010 Apr;36(2):278-83.