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.