Resultados a corto y mediano plazos de la cirugía de la aorta ascendente en el Síndrome de Marfan

Osvaldo Valdés Dupeyrón; Manuel Naffe Abik-reck; Alejandro Villar Inclán; Nicolás Chao González; Rigoberto Chil Díaz; Lázaro Aldama Pérez

Authors

Osvaldo Valdés Dupeyrón
Manuel Naffe Abik-reck
Alejandro Villar Inclán
Nicolás Chao González
Rigoberto Chil Díaz
Lázaro Aldama Pérez

Abstract

Introduction and Objectives: Marfan syndrome is a genetic disorder that causes destruction of elastic fibers in tissues that contain them. The cardiovascular system is one of the most commonly affected, where a progressive dilatation of the aorta (often asymptomatic) occurs, and if undiagnosed it can lead to the patient's death at an early age in life. The objective was to describe the results of the progress of patients with Marfan syndrome and ascending aortic surgery. Method: A descriptive study was conducted between January 2007 and December 2010, where six consecutive patients with Marfan syndrome underwent surgery in the Service of Cardiovascular Surgery of Hermanos Ameijeiras Hospital. The variables related to the diagnosis and surgical procedures and postoperative progress at one year were analyzed. Results: Aortic disorders in Marfan syndrome occurred at a relatively young age (40.33 ± 8.33). Aortic dissection was the most common disease (66.6%), and was related to the presence of pain and hypertension. Radical techniques predominated (66.6%) over the conservative ones. Surgical mortality was 16.6%, with one death; the remaining patients had a survival at one year of 100%. Conclusions: Male patients with aortic dissection and normal ejection fraction were predominant. The most used surgical technique was that of Bental De Bono. Only one patient died in the hospital, the rest had an excellent evolution at one year.

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