Medical Director UPR School of Medicine San Juan, Puerto Rico, United States
Abstract: Introduction /
Objective: The outcome of patients with Hypoplastic Left Heart Syndrome (HLHS) has dramatically improved because of the refinement of surgical techniques and perioperative care. Norwood procedure (NP), the first surgical step of staged palliation for hypoplastic left heart syndrome (HLHS), aims to provide unobstructed systemic blood flow through a reconstructed aortic arch, to control pulmonary blood flow through a shunt, and create an unrestricted communication between the atria. There has been debate on the timing of the procedure suggesting that early procedures achieve better outcomes. The aim of our study was to analyze the outcomes of patients with HLHS who underwent Norwood procedure in terms of the age of the patient at procedure and factors associated with overall mortality. We hypothesize that early Norwood procedure for HLHS in the first 2 weeks of life does not decrease the mortality rate nor length of stay.
Methods: A retrospective review of the pediatric cardiovascular surgery database at the Centro cardiovascular de PR y del Caribe was performed to identify neonates with HLHS who underwent Norwood procedure (NP) from June 2017 to June 2021. Patients included in the study are those with a diagnosis of HLHS or a related single ventricle anomaly and a planned NP. Demographics, outcome, and clinical data were gathered from electronic medical records. Data was expressed as means ± SD or percentiles as appropriate. To evaluate differences in LOS among groups a Mann-Whitney test was conducted. A Fisher’s exact test was used to evaluate differences in outcomes among groups.
Results: A Total of 18 patients met inclusion criteria. The average age of the study population was 22 ± 9.3 days, 60 % (10) were males and 3.2 Kg was the average weight. Overall study population length of stay (LOS) was 39 ± 22 days, and overall mortality rate was 28%. There was no difference in mortality rate in patients with a NP before 14 days old versus older patients ( 40% vs 30%, p = 0.71). There was no difference in LOS when comparing LOS among earlier versus later procedure in time to discharge from the Cardiac ICU (20.6 versus 28.5 days, p = 0.43).
Conclusion: Our study population suggest that the time of Norwood procedure does not impact the mortality rate as previously reported. We propose that improvement in the pre-operative management and identification of other co morbidities have a higher impact in the survival of these population.
