Type A dissection in young patients.

OBJECTIVES: Ascending aortic dissection still remains a poorly understood dramatic disease with unpredictable results for the patient in spite of all the cardiac surgeons' efforts. Mean age of disease onset generally is in the sixth decade and one decade earlier in the case of connective tissue abnormalities. Type A dissection among young patients (<40 years) remains rare. An isolated analysis of these patients may help to understand the pathology and identify risk factors and may show postoperative disease progression.

METHODS: From July 1968 to February 2009, 27 patients (22 men and five women) younger than 40 years old at admission were operated for acute or chronic type A dissection. A cross-sectional follow-up was done in December 2009 by phone interview and analysis of the most recent available echocardiography and/or CT-scan.

RESULTS: A connective tissue disease was causative in 46% and a bicuspid aortic valve (BAV) was found in 22% of the patients. Fourteen patients had a Bentall procedure and 13 patients a simple prosthetic ascending aortic replacement. Early in-hospital mortality was, with 11%, lower than the average early mortality rate. Twenty-six percent of patients developed neurological complications. During a mean follow-up of 117 months, 20 patients survived in good cardiac health (late mortality rate 8%). Aortic root dilatation was the main re-operation cause and occurred in almost 40% of patients after a simple prosthetic ascending aortic replacement.

CONCLUSIONS: Predominant etiologies for type A dissection in young patients are connective tissue diseases, BAV, severe hypertension, vascular diseases and cocaine abuse. Early in-hospital mortality rate was low and principally influenced by massive myocardial infarction due to coronary dissection as well as severe neurological disorders. Aortic root dilatation after a prosthetic ascending aortic replacement was the main re-operation cause and occurred earlier during follow-up than arch or more distal aortic dilatation. Surgical ascending aortic repair for type A dissection in young patients is lifesaving with excellent long-term survival.