Case Report:
A 14-years-old boy born of non-consanguineous parents, hailing from Kanyakumari, along the southern coast of India, was diagnosed with TOF and referred to our institute. He underwent a cardiac catheterization and was found to have elevated right-sided filling pressures and systemic right ventricular (RV) pressures. The right atrial mean pressure was 15 mmHg and right ventricular end-diastolic pressure was 26 mmHg. He underwent trans-atrial intracardiac repair with a transannular patch. The postoperative period was complicated by right-sided pleural effusion which was managed with intercostal drain for 6 days. On follow up he was noted to have a tiny residual perimembranous ventricular septal defect and was kept on conservative management. He was lost to follow up five years after surgery.
He presented again at 40 years of life with functional class II dyspnea on exertion for 6 months and recent-onset pedal edema. He was noted to be in atrial fibrillation with controlled ventricular rate. The mean jugular venous pressure was elevated with prominent v waves and sharp y descent. Echocardiography revealed moderate tricuspid and pulmonary regurgitation and dilated right ventricle. A restrictive perimembranous ventricular septal defect was noted shunting left-to-right during systole and right-to-left during diastole (Figure 1, A-B) . A detailed evaluation showed apical obliteration of the RV apex and reduced trabeculations. Cardiac MRI showed RV apex obliteration(Figure 1, C-D) with late gadolinium enhancement of RV apical endomyocardial. RV volumes were calculated to be 145 ml/m2 in end-diastole and 85 ml/m2in end-systole.
After stabilization, cardiac catheterization was done to study the hemodynamics. Right atrial mean was elevated (v 28mmHg, m16mmHg). There was no gradient from RV inflow to RV outflow, RV inflow (systolic 29mmHg, dip diastolic 12mmHg, end-diastolic 18mmHg), and RV outflow (Systolic 28mmHg, end-diastolic 17mmHg) (figure 2) . Pulmonary artery pressures were systolic 22mmHg, diastolic 12mmHg and mean 16mmHg. There was no right ventricular outflow tract obstruction. There was an 8% step-up in oxygen saturation at the ventricular level with a shunt fraction of 1.25:1. RV angiogram showed RV apical obliteration and moderate tricuspid regurgitation. A left ventricular angiogram showed normal left ventricular contour with good ventricular function and a small sub-aortic ventricular septal defect shunting left-to-right.
He was kept on close medical management with a diuretic. He is planned for a pulmonary valve replacement with bidirectional Glenn shunt in case of recurrent heart failure or symptomatic worsening.