Clinical features and pregnancy outcomes in women with aortopulmonary window defect: case series

Abstract

Background: Aortopulmonary window is a rare congenital heart defect that results in severe pulmonary arterial hypertension (PAH), Eisenmenger syndrome, and congestive heart failure in the first months of life. Pregnancy is absolutely contraindicated in the patients with this condition.

Case summary: This paper describes two clinical cases of pregnancy in patients (28 and 20 years old) with aortopulmonary window defect, severe PAH, and Eisenmenger syndrome that ended in preterm delivery by caesarean section. One patient died in the postpartum period due to progression of right ventricular heart failure. The younger patient survived childbirth and the postpartum period; later, she continued therapy at the PAH centre.

Discussion: We describe unusual cases of clinical features in pregnant women with aortopulmonary window defect. Due to the rare occurrence and low survival rate of patients with uncorrected aortopulmonary window defect, descriptions of clinical cases of this defect in adults are very rare. It is very important to note the necessity of observation of these patients in specialized centres by a multidisciplinary team of healthcare professionals, due to the high risk of cardiovascular, obstetric complications, and death.

Keywords: Aortopulmonary window; Case report; Eisenmenger syndrome; Pregnancy.

Figure 1

Patient 1. (A) Transoesophageal echocardiography at 29 5/7 weeks: ‘Supravalvular location of aortopulmonary junction up to 30 mm with balanced blood flow (truncus arteriosus type)’. (B) Concentric narrowing of the small-calibre pulmonary artery lumen due to wall fibrosis (arrow), intraalveolar haemorrhage as a demonstration of disseminated intravascular coagulation syndrome (asterisks), ×200 increase.

Figure 2

Patient 2. (A) Non-contrast magnetic resonance imaging (MRI) of the aorta at 32 3/7 weeks: ‘The ascending aorta and pulmonary trunk are communicating for 35 mm after 5–10 mm of divergence’. (B, C) Echocardiography 6 months after delivery: ‘Defect between aorta and pulmonary artery up to 41 mm with predominantly right-to-left shunt. Qp/Qs of 0.8’. (D, E) Electrocardiogram (EСG)-gated computed tomography angiography cinematic rendering (16 day postpartum). RV, right ventricle; LV, left ventricle; Ao, aorta; PV, pulmonary valve; LPA, left pulmonary artery. Star, aortopulmonary fenestration. Electrocardiogram-gated computed tomography angiography was performed and revealed two atriums and two ventricles with concordant connection. The aorta took its origin from the left ventricle and the pulmonary artery from the right ventricle. The location of the aortic and of the pulmonary valves was typical. From the level of the aortic sino-tubular junction, the large aortopulmonary fenestration with length of about 48 mm was found. Above, the fenestration aorta was enlarged to 60 mm. And the pulmonary artery was divided to the right and left branches directly after the fenestration. (F, G) Electrocardiogram-gated computed tomography angiography volume rendering technique (VRT) soft tissue reconstruction (16 day postpartum). LV, left ventricle; LVO, left ventricular outflow tract; RV, right ventricle; RVO, right ventricular outflow tract; AV, aortic valve; PV, pulmonary valve. Star, aortopulmonary fenestration. There was an extensive defect of the aortopulmonary septum of 4.6 × 4.8 cm from the level of the sino-tubular zone and significant dilation of the ascending thoracic aorta (above the defect level, the diameter was 6.4 × 6.1 cm).