AV Valve Repair in a 3 kg Infant With Tricuspid Atresia

The authors present a case of AV valve repair in a 3 kg infant with tricuspid atresia. 

The patient was diagnosed prenatally with tricuspid atresia. The patient was born full term with a birth weight of 2.8 kg. Diagnosis was confirmed as tricuspid atresia with transposition of the great arteries. Atrial septal defect and bulboventricular foramen were unrestrictive and the BVF was larger in dimension than the aortic valve. The patient underwent PA banding as a neonate. Distal pulmonary artery pressures were brought down to about one third systemic with saturations around 80%. The band gradient persisted at around 50 mm Hg. The patient did well immediately after surgery but over the next 3-4 weeks developed worsening mitral regurgitation due to annular dilatation. Despite maximal medical therapy, the patient developed low cardiac output, renal failure requiring peritoneal dialysis, and needed mechanical ventilation. The authors recommended surgical mitral valve repair. There was no cleft seen in the anterior leaflet of the mitral valve. They performed an annuloplasty and this significantly improved mitral regurgitation. 

The patient recovered well and was weaned off the ventilator. His renal function recovered and was taken off peritoneal dialysis.

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