Clinical and Surgical Approach in the Hypoplastic Left Heart Syndrome

pp 231-237

Abstract

The term hypoplastic left heart syndrome (HLHS)is used to describe a complex of congenital cardiopathies, which share the variable degree of under-development of left heart structures. The most common presentation of this syndrome includes aortic and/or mitral valve stenosis or atresia and ductus dependence of systemic circulation. This malformation is responsible of 25% of deaths due to cardiopathy in the first week of life in the absence of a medical or surgical treatment. Since July 1997 we have developed a program to treat this malformation in our hospital. In this period 7 patients with HLHS were operated. Their ages ranged from 20 to 60 days and their weight from 2.750 to 3.500kg. The modified Norwood technique was performed in 6 patients. The seventh baby, with tricuspid insufficiency and right ventricular dysfunction underwent cardiac transplantation plus reconstruction of the hypoplastic aortic arch. Among those patients with Norwood operation there were 3 deaths, 2 in the immediate postoperative period due to arrhythmia and the other one because of sudden death two months after the surgery. In this work we analyze the preoperative, intraoperative and postoperative events of the seven patients as well as their nutritional recovery, neurological development, pulmonary flow and hypoxic status with both surgical techniques.

Conclusions

1) It is possible to achieve survival in HLHS in our country with medical and pharmacological management followed by surgical treatment. 2) The modified Norwood procedure is the best option for those patients with HLHS without valve insufficiency or right ventricular dysfunction. 3) The neonatal heart transplantation is a valid option in those cases with tricuspid regurgitation and right ventricular hypokinesis.