Download Pregnant Patients with Ebstein`s Anomaly Clinical and

A.Leśniak-Sobelga 1, L.Tomkiewicz-Pająk 1, M.Kostkiewicz 1,
S.Wiśniowska-Śmiałek 1, J.Pająk 2 , P.Podolec 1
(1) Institute of Cardiology, Jagiellonian University,
Dept.of Cardiovascular Diseases, John Paul II Hospital,
Kraków, Poland
(2) Medical University of Silesia, Poland
Ebstein’s anomaly (EA)
 is an uncommon congenital heart defect with
a prevelence of 0.3-0.5%, characterized primarily
by abnormalities of the tricuspid valve and right
ventricle.
 EA does not affect fertility even in cyanotic women.
 An interatrial communication (ASD/PFO) is present
in 80-94% of patients with EA.
 Survival into adulthood is common, there were also
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described survivals up to 85 years.
EA may manifest clinically at any age and has a highly
variable clinical course.
Adults often present with cyanosis, dyspnea, palpitations,
decreasing exercise tolerance, fatigue.
Exercise tolerance is dependent on heart size and oxygen
saturation.
Accessory pathways (Wolff-Parkinson-White s.) are common
associated with EA (6-36%). First degree a-v block is
found in up to 50% of patients, RBBB is a typical finding .
 Right ventricular (RV) dysfunction is often observed,
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left ventricular (LV) dysfunction is rare.
While pregnant patients with EA are usually acyanotic,
those with interatrial shunting can develop shunt
reversal and cyanosis in pregnancy.
Paradoxical embolism can occur even in totally
asymptomatic patients.
The presence of arrhythmia or cyanosis in the mother
is associated with increased maternal and fetal risk.
The risk of congenital heart disease in offspring is
reported in 4 - 6%, and familial EA in 0.6%.
 We report six pregnants (mean age 25.4 ± 6.2 y)
with EA who have been recently under our care.
 Medical history and physical examination, New York
Heart Association (NYHA) class assessment, ECG
and echocardiography, oxygen saturation,
NT-proBNP levels were performed every month
during consecutive trimesters (TR) of pregnancy
and after delivery.
Maternal outcome:
 palpitations and dyspnea (6 pts), syncope -1
pregnant in 6th week.
 supraventricular arrhythmias (5 pts) were common
during the 2nd and 3rd trimester, av block IIº-1
pregnant, av block Iº-1 pregnant,
 there were no cyanotic patients,
 S02 and NT-proBNP levels didn’t change significantly
 the functional status was good, all of them persisted
during pregnancy in NYHA class II.
ASD II
 Caudal displacement of the septal and posterior
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leaflets of the tricuspid valve > 8 mm/m² (6 pts),
RV and RA enlargement,
moderate to severe TR,
atrial septal defect (ASD II) with left-to-right shunt
and mean RV systolic pressure 42±6 mmHg (4 pts),
according to the Celermajer echocardiographic
grading score 3 pts were encountered to class 1,
2 pts to class 2 and 1 - to class 3,
no deterioration of the RV or LV function
during pregnancy occurred
 All 6 patients delivered vaginally at 36-40 week
of pregnancy.
 Fetal and neonatal outcome: mean birth weight
2860g (range 2510-3460g), Apgar score 9-10.
 On physical examination and echo screening there no
congenital heart disease in the offspring was detected.
 Endocarditis prophylaxis in the peripartum period was
not implemented (as it is currently not indicated in
pregnants with EA , ESC Guidelines 2011)
 Data from our Ebstein's anomaly pregnants cohort
confirm that, even in case of severe tricuspid
regurgitation, pregnancy is tolerated relatively well.
 No right ventricle dysfunction or pulmonary
hypertension occurred.