Second surgical stage in hypoplastic left heart syndrome (HLHS), a step towards hemodynamic stabilization

pp. 36-41

Abstract

Objetive

Our purpose was to demonstrate the importance of the Glenn procedure to achieve hemodynamic stabilization in patients with HLHS operated with the modified Norwood technique the avoiding sudden death. 

Material and methods

A retrospective analysis of 34 patients (14 female and 20 male) with diagnosis of HLHS was done: 32 of them were operated with the modified Norwood technique. Mean age was 20 days and mean weight was 2.8 kg. Out of the 15 survivors, 11 underwent bidirectional Glenn surgery, 6 females and 5 males, with an average age of 11 months and a average weight of 7.4 kg. In the group of the 32 patients submitted to the Norwood procedure 12 died during the immediate postoperative period (in the month after surgery) and 7 during long-term follow-up. Among the 11 patients who underwent  the Glenn procedure, one died early due to sepsis. During long-term follow-up (between 3 and 41 months) in patients with the Glenn procedure mortality rate was 0%, whereas in the pre-operative period the long-term mortality reached 31%. Several factors lead to the circulatory instability of patients with HLHS in the perioperative period and during long-term post-surgical follow-up. Basically, the physiopathology of both systemic and pulmonary interdependent circulations makes them prone to a fall of pulmonary, coronary and/or systemic circulation. Once the Glenn surgery is performed, this circulatory dependence is definitively interrupted. 

Conclusions

1. The initial surgery should be performed as early as possible, and all efforts should be focused in decreasing initial mortality. 2. The main course of action to avoid late mortality of the survivors in the first stage is to perform an early bidirectional Glenn procedure. 3. This strategy should contribute to increase the survival rate in patients with this malformation. 4. Considering these guidelines it is possible to reach a complete reparation with a total cavo pulmonary shunt.