Tricuspid Atresia with multiple surgeries in a 43 year old man

Echo studies

Patient Presentation

This 43-year old man, born with tricuspid atresia, was palliated in childhood with a Waterston -Cooley shunt (later taken down), a Glenn, a Blalock-Taussig, then a graft from descending aorta to left PA (B-T no longer functional). Hemoptysis required coil embolization of right lung AVMs.

At age 31 the aorta-PA graft was coil embolized in preparation for the Fontan: the PDA was ligated, the VSD closed, the ASD closed, a 6 mm fenestration was created, the RA appendage was connected by graft to the left PA, an opening between the RA and the SVC was slightly enlarged to allow some atrial return to the right lung. Due to cyanosis the ASD was closed with a clamshell occluder 2 months later (see transesophageal echo above).

Atrial arrhythmias began at age 25 and were medically treated. Atrial fibrillation recurred 6 years after the Fontan. Sinus rhythm was restored with amiodarone, digoxin, warfarin. An epicardial VVIR pacemaker was placed. VO2 was 14.5 ml/kg/min at baseline.

Clinical course...

Keys to clinical management...