Aortopulmonary window with pumonary atresia with ventricular septal defect with D-transposition of great arteries: extremely rare anomaly

Abstract

Aortopulmonary window (APW) is rare a congenital heart disease accounting for 0.1%-0.2% of all congenital heart defects. The 35% of the APW has been associated with wide variety of other structural heart diseases such as ventricular septal defect, persistent ductus arteriosus, arch anomalies and coronary artery anomalies. To the best of our knowledge, only six cases of APW with pulmonary atresia with ventricular septal defect has been described in the literature. It resembles the type 1 truncus arteriosus, and differentiation from this condition is important prior to surgical correction. We present a case of 14-year-old girl child; she was diagnosed with APW with pulmonary atresia with ventricular septal defect and D transposition of great arteries with the help of echocardiography, cardiac catheterisation and cardiac CT.

Keywords: cardiovascular Medicine; interventional cardiology; radiology (diagnostics).

Figure 1

Chest X-ray showing situs solitus, levocardia, boot-shaped heart with right ventricle type of apex (blue star), atretic end of main pulmonary artery (yellow arrow), dilated aorta (white arrow) and borderline pulmonary vascularity traced up to middle one-third of lung fields.

Figure 2

ECG showing right axis deviation with clockwise rotation (note small q waves in lead 2, lead 3 and lead aVF), right ventricular hypertrophy (prominent R in V1), sudden transition of R in V1 to S in V2 (suggestive of ventricular septal defect) and R in V6 is 10 mm and S is 7 mm (suggestive of preserved left ventricular forces).

Figure 3

Parasternal long axis view showing non-restrictive subaortic ventricular septal defect with atretic pulmonary valve (marked by #) and aortic valve (marked by *). Note aorta arising from right ventricle (RV) and pulmonary artery from left ventricle (LV).

Figure 4

Echocardiogram showing atretic pulmonary valve (blue arrow), aortopulmonary window (marked by yellow *), dextro transposition of great arteries as aorta from anatomical right ventricle (RV) through aortic valve and blind pulmonary end towards left ventricle (LV). Note adequate size of LV and RV.

Figure 5

Echocardiogram showing flow from left ventricle (LV) through large subaortic ventricular septal defect into aorta. No flow from LV to pulmonary artery because of atretic pulmonary valvular end (marked by white arrow).

Figures 6

Right ventricle(RV) angiography (coarsely trabeculated chamber) on lateral view showed (A) filling of left ventricle (LV) through ventricular septal defect marked by arrow; (B) atretic pulmonary end (marked by curved arrow) towards LV and (C) dilated aorta arising from RV.

Figures 7

Right ventricle (RV) angiography on anteroposterior view showed (A) filling of left ventricle (LV) through ventricular septal defect and blind pulmonary end (marked by vertical arrow) towards LV; (B) aorta arising from RV and (C) filling of pulmonary artery from aorta through aortopulmonary window (marked by curved arrow).

Figures 8

Left ventricle (LV) angiography revealed (A) atretic pulmonary end (marked by arrow); (B) filling of right ventricle (RV) through ventricular septal defect and then filling of aorta and (C) filling of main pulmonary artery from aorta.

Figures 9

Aortic root angiography on left anterior oblique cranial view showed (A) origin of main pulmonary artery (MPA) from ascending aorta; (B) MPA selectively entered with pigtail catheter from aortic side through aortopulmonary defect and (C) on giving contrast into MPA in right anterior oblique cranial view, it is dividing into left pulmonary artery (LPA) and right pulmonary artery (RPA).

Figure 10

Cardiac CT (transverse and coronal section) showed origin of aorta from right ventricle and origin of main pulmonary artery from aorta through aortopulmonary window.