Objectives. To define indications and outcome of surgical repair in premature or low birth weight (≤ 2.5 kg) neonates with symptomatic tricuspid valve (TV) disease.
Methods. Between 2004-2008, 6 neonates, with mean birth weight of 2.1±0.3 kg (range 1.7–2.5 kg), were treated for symptomatic TV pathology. Severe TV stenosis was present in 3 (tumor in 2, mycetoma 1) and severe regurgitation in 3 (3 Ebstein/-like dysplasia), with marked RA dilatation in all. Five (83%) had severe pulmonary hypertension and 3 ductal dependent pulmonary blood flow.
Results. Preoperative stabilization required vasoactive drugs in 5 patients, mechanical ventilation with PGE1 infusion in 3 (mean 37 dd), associated with iNO in 2 (mean 21 dd) and atrial septostomy in 1. Surgery, deferred to mean age of 2.8±3.1 mos (20 dd-6 mos) and weight of 2.9±1.5 kg (2.2-4.5 kg), consisted in: mass excision+TV repair in 3, TV repair+RA remodelling in 2, stage 1 palliation followed by RA remodelling+Glenn shunt in 1. There was 1 hospital death: 4 patients required postoperative iNO and 3 were discharged on sildenafil/bosentan therapy. During a 4-year follow-up (mean 2.1±1.4), there was 1 late death with a 67% event-free survival. Follow-up echocardiography showed absent-trivial TV regurgitation in all late survivors, with normalized right heart dimensions.
Conclusions. Symptomatic tricuspid valve pathology in newborns is a highly lethal disease, due to association with low birth weight/prematurity and pulmonary hypertension. Preoperative hemodynamic stabilization and elective surgery in early infancy is feasible in the majority of patients increasing the likelihood of durable repair.