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Case Reports
. 2012;39(2):249-54.

Hypoplastic right-heart syndrome presenting as multiple miscarriages

Chadi Dib  1 , Philip A Araoz, Norman P Davies, Joseph A Dearani, Naser M Ammash
Affiliations
Case Reports

Hypoplastic right-heart syndrome presenting as multiple miscarriages

Chadi Dib et al. Tex Heart Inst J. 2012.

Abstract

Reversible causes of miscarriage are many, but they affect only 1% of women who are trying to conceive. Herein, we describe the case of a 23-year-old woman who presented for evaluation of repeated miscarriages and was found to have hypoxemia and erythrocytosis. Further evaluation revealed hypoplastic right-heart syndrome with an intracardiac shunt. She underwent hybrid repair with pulmonary valve balloon valvuloplasty, followed by surgery to perform atrial septal defect closure and a Glenn anastomosis. The erythrocytosis and hypoxemia resolved, and she was able to conceive and deliver a healthy baby at term 2 years later. This is a unique case of a rare congenital heart defect that went unnoticed until adulthood, when attempts at pregnancy failed because of the associated hypoxemia. Timely and appropriate treatment led to a successful pregnancy after repeated miscarriages. This case exemplifies the need for a comprehensive medical evaluation of every woman with a history of multiple miscarriages to determine whether a reversible cause exists.

Keywords: Abortion, spontaneous/etiology; heart defects, congenital/complications/physiopathology/therapy/surgery; treatment outcome; ventricular function, right/physiology.

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Figures

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Fig. 1 Transthoracic echocardiograms (subcostal views) show A) a hypoplastic right heart with increased wall thickness, and B) an atrial septal defect with left-to-right shunt (arrow). LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle
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Fig. 2 Transthoracic echocardiograms (parasternal views) show A) a thickened pulmonary valve (arrows) with limited systolic excursion, and B) with Doppler interrogation, moderate pulmonary stenosis. LV = left ventricle; PA = pulmonary artery; PV = pulmonary valve; RV = right ventricle
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Fig. 3 Color-flow Doppler echocardiograms show a large atrial septal defect (arrows) with A) left-to-right shunt and B) right-to-left shunt. LA = left atrium; RA = right atrium; SVC = superior vena cava
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Fig. 4 Transthoracic echocardiogram (basal 4-chamber view) shows a small right ventricle and tricuspid valve annulus and an atrial septal defect (asterisks). LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle
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Fig. 5 Transthoracic echocardiograms (longitudinal views) show A) a thickened pulmonary valve (arrow) at the level of the pulmonary artery, with limited systolic excursion; and B) with color-flow Doppler interrogation, turbulence caused by pulmonary stenosis (arrow). LA = left atrium; LV = left ventricle; PA = pulmonary artery; PV = pulmonary valve
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Fig. 6 Cardiovascular magnetic resonance (4-chamber view) shows a small right ventricle (RV) in comparison with the left ventricle (LV). The right atrium (RA) is of normal size; however, the interatrial septum bows toward the left atrium (LA).
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Fig. 7 Diastole: Cardiovascular magnetic resonance (3-chamber view, equivalent to echocardiographic parasternal long-axis view) shows that the right ventricular outflow tract (arrowhead) is mildly enlarged in diastole. The left ventricular outflow tract is also shown (arrow). Ao = aorta
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Fig. 8 Systole: Cardiovascular magnetic resonance (3-chamber view, equivalent to echocardiographic parasternal long-axis view) shows that the right ventricular outflow tract (arrowhead) is markedly dilated in systole. The left ventricular outflow tract is also shown (arrow). Ao = aorta
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Fig. 9 Cardiovascular magnetic resonance (oblique coronal view) of the right ventricular (RV) outflow tract shows a systolic jet (arrow), indicating residual pulmonary stenosis. The main pulmonary artery is dilated.
See this image and copyright information in PMC

References

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