Investigations
Chest radiograph showed cardiac enlargement resembles a classic ‘calabash’ configuration (Figure 1A and 1B). A CT scan further presented anomalous pulmonary vein (PV) and to check any remaining PV drainage into the left atrium.
Three-dimensional images further delineated the supracardiac TAPVR in coronal view (Figure 2A) and sagittal view (Figure 2B). It found several pulmonary veins draining separately into the collecting vein. The collecting vein, located above the left atrium, ascended and formed a dilated vertical vein. The vertical vein drained into the superior vena cava (SVC) by a dilated left innominate vein was observed. Other CT findings include no pulmonary venous obstruction, a large atrial septal defect (ASD), cardiomegaly with the dilated right ventricular forming the right lateral heart border.
Cardiac catheterisation revealed common drainage of pulmonary veins into a collecting vein. This vein ascended and formed a left innominate vein and finally drained into the SVC (Figure 2C and 2D). Haemodynamic data obtained as follows: aortic pressure 74/48mm Hg, main pulmonary artery pressure 28/9 (11) mm Hg, right ventricular (RV) pressure 11/4 (7) mm Hg. The elevated RV pressure with a pressure gradient of 24 mm Hg was also suggestive of pulmonary stenosis. Oxygen saturation from arterial blood gas was 96.0% under room air. The calculated pulmonary vascular resistance (PVR) was 0.87 wood unit.
The patient was referred for surgical repair. She underwent a successful surgical correction of TAPVC and ASD close. At procedure, she was diagnosed with supracardiac TAPVC and found excessively dilated SVC, innominate vein, and right heart chamber. The pulmonary artery was larger than the aorta (ratio=2.5:1). The large ASD was measured at 31mm × 22mm diameter and the vertical vein was at 22mm × 26 mm diameter. The LA was connected to the PV confluence through the anastomosis. A patch material has been used to close the large ASD. Patient was discharged well on the sixth postoperative day. No palpitations and exertional dyspnea during the half year fellow-up.