Discussion:
Endomyocardial fibrosis (EMF) is a dwindling disease worldwide now limited to coastal India and parts of Africa and South America1. It is characterized by fibrotic replacement of ventricular myocardium leading to impaired relaxation thus causing elevated diastolic pressures, and also valvular regurgitation. Management of EMF consists of control of heart failure and the surgical option of endocardial decortication and if required valvar repair/replacement. Further, the role of bidirectional Glenn has been reported in the advanced course of the disease2.
Ours is a unique case where this tropical disease process complicated the long term management of TOF. The index patient was documented to have elevated right-sided filling pressures at the age of 14 years. However, in the absence of ventricular dilatation or effacement, endomyocardial fibrosis was not suspected and elevated right ventricular filling pressures were attributed to the restrictive pattern seen in delayed presentation of TOF3. While a diagnosis of EMF in its inflammatory stages would have helped us initiate anti-inflammatory therapy, it seems unlikely that it would have altered the surgical plan. Since the patient tolerated the postoperative period without low cardiac output syndrome, it is clear that the right ventricular volumes were adequate for biventricular repair. Advanced stages of this disease process have been reported earlier as the development of Fontan physiology in the case of endomyocardial fibrosis4. Since the patient showed symptomatic improvement with medications, he was kept on close medical management temporarily.
The diastolic right-to-left shunt across the residual VSD was attributed to the elevation of RV end-diastolic pressures more than left ventricular diastolic pressures.