Case report
Informed written consent was taken from the patient’s guardian for the
publication. A 3 month old infant weighing 2.2 Kg was hospitalized with
respiratory distress. On examination, patient was tachypneic, cyanosed
and dilated veins were visualized all over the chest and abdomen. A
faint ejection systolic murmur was heard at left upper sternal border
and per abdomen revealed mild hepatomegaly. Chest X-ray revealed slight
cardiomegaly with prominent hilar markings. Electrocardiogram (ECG) was
consistent with features of right ventricular hypertrophy (RVH). Mild
hepatomegaly was observed on ultrasound abdomen. Trans-thoracic
echocardiography (TTE) revealed anomalous pulmonary venous confluence
behind left atrium (LA), draining directly into superior vena cava (SVC)
from right lateral aspect at junction of SVC with right atrium (RA).
There was a discrete narrowing at this confluence. SVC was grossly
dilated proximal to the stenotic site with flow velocity of 2.5m/sec
across the narrowed segment. RA and right ventricle (RV) were dilated
and an ostium secondum atrial septal defect (OS-ASD) was also present.
Contrast enhanced computed tomography (CECT) chest, revealed that all 4
pulmonary veins (PV) joined to form a common chamber (CC) behind LA
which was draining into SVC at SVC-RA junction with discrete narrowing
at the junction. There was bulbous dilatation of SVC proximal to
stenotic site (Figure 1).
With provisional diagnosis of obstructed supracardiac total anomalous
pulmonary venous connection (TAPVC) and congenital SVC stenosis, patient
was taken up for surgery. Per-operatively, proximal SVC as well
bilateral innominate veins were hugely dilated. Circumferencial stenosis
at lower end of SVC (approximately 1 cm in length), starting at SVC-CC
junction and extending distally till its termination into RA was
observed (Figure 2). Drainage of the CC was into the lower end of SVC at
its right lateral aspect by an opening that was roughly 3-4 mm in size.
RA, RV were dilated. Cardiopulmonary bypass (CPB) was established with
aortobicaval cannulation, with special emphasis on high SVC cannulation.
After cardioplegic arrest, the heart was rotated anticlockwise around
its axis and dropped into right plural cavity. Anastomosis of LA with CC
was done. SVC was widely incised on its lateral aspect. Circumferential
fibrosis was observed within the distal part of SVC emanating from the
drainage site of CC into SVC (Figure 3). The connecting vein between CC
and SVC was doubly ligated from outside, and SVC was augmented with a
rectangular pericardial patch (Figure 4). Patient was weaned off CPB
with inotropic support and nitric oxide (NO). Trans-esophageal
echocardiography (TEE) intra-operatively, ruled out any significant
gradient across SVC-RA junction, and CC-LA anastomosis. Delayed sternal
closure was done and patient had an uneventful recovery.