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.