Case presentation
A 30-month-old female weighing 10800 gms, was brought to our hospital
with complaints of dyspnea and recurrent respiratory infections, and was
diagnosed to have visceral situs inversus. She was the 3rd of five
siblings, born to non-consanguineous marriage couple, and she was
delivered with no relevant birth details. Her siblings were normal.
Clinical examination revealed systolic murmur in the base of the heart,
the liver was clear on the left side. Chest x-ray (CXR) showed
dextrocardia with important cardiomegaly along with increased pulmonary
vascular markings (Figure 1). Transthoracic echocardiography (TTE)
confirmed the diagnosis of SIT along with large ASD measuring 8*12 mm
with bidirectional flow, and a non-significant patent ductus arteriosus
(PDA). There was severe right ventricular enlargement with mild to
moderate tricuspid valve regurgitation. The left atrium cavity was
small. Estimated systolic pressure in the right ventricle was 75 mmHg
(calculated from the flow velocity of the tricuspid regurgitation jet
and the assumed central venous pressure), and the mean pressure in the
pulmonary artery was estimated to be 55 mmHg. The right pulmonary veins
were seen draining to the morphologically left atrium, but the left ones
were not visible, therefore computed tomography angiography (CTA) was
requested. CTA revealed that the left pulmonary veins were draining
anomalously to the left-sided right atrium with normal drainage of the
right pulmonary veins to the left atrium (Figure 2). The patient was
operated on through median sternotomy approach. The pericardium was
opened. The morphological right atrium was to the left and the apex of
the heart was right-sided (Figure 3-A). Large PDA was seen to the right
between the right-sided pulmonary artery and the aorta. It was dissected
and closed appropriately. A total cardiopulmonary bypass (CPB) was
prepared. The aorta was clamped and the heart was arrested by antegrade
del Nido cardioplegic solution. The right atrium was opened with an
incision parallel to the atrioventricular groove. The left pulmonary
veins were seen draining directly to the right atrium just above the
large ASD, whereas the right ones were draining to the left atrium. A
large fresh autologous pericardial patch was fashioned to close the ASD
and create a baffle draining the anomalous right pulmonary veins to the
left atrium beneath the patch (Figure 3-B). The right atriotomy was
closed, and the aortic cross clamp was released. The patient was weaned
off the CPB uneventfully. Chest was closed in standard fashion.
Mechanical ventilation was required for 8 hours. The patient’s
convalescence in the intensive care unit was uneventful. Postoperative
TTE showed an excellent result of the operation with estimated systolic
pressure in the right ventricle of about 35 mmHg, along with mild
tricuspid regurgitation. The patient was discharged on the fifth day
without any complications. The patient was followed-up for 12 months,
and she was without any complaints along with important clinical and
radiological improvement.