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.