Partial anomalous pulmonary venous connection with intact atrial septum in a child with ventricular septal defect: a case report

Abstract

Partial anomalous pulmonary vein connection (PAPVC) is a rare congenital abnormal cardiac defect involving the pulmonary veins draining into the right atrium (RA) directly or indirectly by venous connection. Ninety percent of PAPVCs are accompanied by atrial septal defect (ASD). To our knowledge, there is no previous report of PAPVC with ventricular septal defect (VSD) without ASD in Korea, and in this paper, we report the first such case. A 2-day-old girl was admitted into the Chonnam National University Hospital for evaluation of a cardiac murmur. An echocardiogram revealed perimembranous VSD without ASD. She underwent patch closure of the VSD at 5 months of age. Although the VSD was completely closed, she had persistent cardiomegaly with right ventricular volume overload, as revealed by echocardiography. Three years later, cardiac catheterization and chest computed tomography revealed a PAPVC, with the right upper pulmonary vein draining into the right SVC. Therefore, correction of the PAPVC was surgically performed at 3 years of age. We conclude that it is important to suspect PAPVC in patients with right ventricular volume overload, but without ASD.

Keywords: Atrial heart septal defect; Partial anomalous pulmonary venous connection; Ventricular heart septal defect.

Fig. 1

(A) The transthoracic echocardiographic parasternal short axis view shows a perimembranous VSD (arrow). (B) The color-Doppler 4 chamber view shows a VSD (arrow) with left to right shunt flow. (C) The color-Doppler subcostal view shows an intact atrial septum. VSD, ventricular septal defect; RA, right atrium; RV, right ventricle; LA, left atrium; LV, left ventricle.

Fig. 2

(A) The post-surgery 2-dimensional echocardiographic parasternal short axis view shows a closed VSD. (B) The 2-dimensional 4 chamber view shows both RA and RV enlargement. (C) The M-mode echocardiographic short axis, mid-ventricular view shows a paradoxical septal movement, i.e., an upward deviation of the ventricular septal wall (arrow) during the systolic phase, due to volume overload of the RV. VSD, ventricular septal defect; RA, right atrium; RV, right ventricle; LA, left atrium; LV, left ventricle.

Fig. 3

(A) Angiography of the RUPV shows a partial anomalous pulmonary venous connection, consisting of the RUPV, which drains into the RA through the SVC. (B) Chest computer tomography shows the RUPV (arrow), which drains into the SVC. RUPV, right upper pulmonary vein; RA, right atrium; SVC, superior vena cava; Ao. Aorta; PA, pulmonary artery.

Fig. 4

After partial anomalous pulmonary venous connection correction, the 2-dimensional echocardiographic 4 chamber view shows the improvement of both RA and RV enlargement. RA, right atrium; RV, right ventricle; LA, left atrium; LV, left ventricle.