hypoplastic left heart syndrome submitted to the first surgical stage

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Hypoplastic Left Heart Syndrome (HLHS) consists of a group of congenital heart diseases characterized by varying degrees of underdevelopment of the left ventricular cavity, and biventricular surgical correction is not possible. This syndrome is present in 1.4% to 3.8% of all congenital heart diseases, which corresponds to 0.016 to 0.036% of live births. Despite low incidence, the HLHS is responsible for 23% of deaths of cardiac origin during the first week of life and 15% of deaths by the same cause by the end of the first month.Norwood surgery was first described in 1981 and consists on the first of a 3-step phase for a palliative cardiac approach based on the right ventricle as a single cardiac pump.Despite the improvement in the survival of these patients, the first stage is still associated with a high length of hospital stay, a high rate of complications and a high mortality rate.Some comorbidities, such as prematurity, low weight, or associated malformations increase the risk for Norwood's surgery, increasing its morbidity and mortality. In these cases, the hybrid procedure emerged as an alternative in the last 20 years because it is less invasive, offering a lower risk to patients.. Despite the decrease in early mortality with this surgical strategy, the patients still maintained a high mortality level after the 2nd stage, with late survival comparable to Norwood Surgery. In the United States, only 13% of cardiac surgery services have high hybrid procedure rates and 50% opt for Norwood Surgeries in all cases of HLHS

The experience of the service also brings a great variability of results, making the retrospective analysis of cases essential for the creation of protocols on this topic.It is important to improve not only longevity but also the quality of life of patients [8]. This study aims to evaluate the morbidity and mortality of patients diagnosed with HLHS who underwent the Hybrid procedure or Norwood Surgery and its evolution to the second stage (Glenn's surgery) and the third stage (Fontan's surgery).The preoperative variables that were analyzed were gender, twinness, birth weight, prematurity, prenatal diagnosis of congenital heart disease, prenatal diagnosis of other comorbidities the diagnosis of genetic syndromes, the presence of preoperative organ dysfunction, the highest lactate and the lowest pH before the procedure. The variables related to the surgical procedure were the type of surgery, the age in the surgical procedure and the weight in the surgery, while the postoperative variables were the length of stay in the Intensive Care Unit, the length of hospital admission and the age at the time of death.Categorical variables were analyzed through frequencies and percentages while non-categorical variables were analyzed through means (symmetric variables) and medians (asymmetric variables). The chi-square test was performed to assess the chance of discharge from the ICU, as well as logistic regression for multivariate analysis. For the survival analysis, the Kaplan-Meier test was used. In all cases, a p below 0.05 was considered statistically significant.

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American Journal of Phytomedicine and Clinical Therapeutics
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