Tiny hearts, great solutions: a Literature Review about the surgical therapeutic approaches for Hypoplastic Left Heart Syndrome

• Autor(a) principal: Faneca, Beatriz Capela
• Data de Publicação: 2022
• Tipo de documento: Dissertação
• Idioma: eng
• Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
• Texto Completo: http://hdl.handle.net/10400.6/12757
• topic: Cardiopatias Congénitas; Cirurgia Bidirecional de Glenn; Cirurgia de Fontan; Cirurgia de Norwood; Procedimento Hemi-Fontan; Procedimento Híbrido; Síndrome do Coração Esquerdo Hipoplásico; Transplantação Cardíaca; Valvuloplastia Aórtica Fetal; Domínio/Área Científica::Ciências Médicas::Ciências da Saúde::Medicina

Hypoplastic left heart syndrome (HLHS) is a complex congenital malformation characterized by underdevelopment of the left heart with significant left ventricle hypoplasia, including atresia, stenosis, or hypoplasia of the aortic and mitral valves. It is one of the most severe forms of congenital heart disease and, without surgical treatment, the mortality rate for infants with HLHS exceeds 90% in the first year of life. About five decades ago, comfort care was the only therapeutic option available, resulting in premature death of infants with this syndrome. Nowadays, there are two recognized surgical modalities, palliative staged reconstruction, and cardiac transplantation. Conventional surgical therapy involves recruiting the right ventricle to the systemic circulation and separating the pulmonary and systemic circulations, in three stages. It consists of an initial Norwood operation during the neonatal period, followed by a bidirectional Glenn or a Hemi-Fontan procedure performed at 4 to 6 months of age, and a Fontan operation between 18 and 48 months of age. Changes in patient selection, pre- and postoperative management, and improved surgical techniques, have contributed to minimize perioperative mortality to 10%. However, the surviving cohort presents with multiple early and late complications, with some patients evolving to the “failing” Fontan. Cardiac transplantation has been established as a primary treatment option in infants with HLHS. The main advantage is the replacement of a functionally univentricular heart with a normal heart in a single surgery. Although survival is excellent, the multistage palliation has been favored due to limited number of available donors. With prenatal diagnosis advancements in the second decade of the 21st century, in-utero procedures were proposed to change the rules of the game. The rationale for in-utero interventions is restoration of onward flow and reduction of intraventricular pressure, hence promoting left ventricle growth and function. Fetal aortic valvuloplasty requires numerous resources and expertise in fetal diagnosis, obstetric and cardiovascular imaging, catheter techniques, and maternal care. Few centers have the capacity to do so successfully, nevertheless its results are more and more encouraging. This literature review aims to assess the role of different surgical therapeutic options for HLHS, procedures, optimizations, survival, and improvement in quality of life, adverse effects, long-term outcomes, and burden of disease in these patients. It also wants to shed light and explore future perspectives regarding the best approach to these patients.