The Beneficial Effect of Renin-Angiotensin-Aldosterone System Blockade in Marfan Syndrome Patients after Aortic Root Replacement

PURPOSE: In this study, we evaluated the long term beneficial effect of Renin-Angiotensin-Aldosterone System (RAAS) blockade therapy in treatment of Marfan aortopathy. MATERIALS AND METHODS: We reviewed Marfan syndrome (MFS) patients who underwent aortic root replacement (ARR) between January 1996 and January 2011. All patients were prescribed beta-blockers indefinitely. We compared major aortic events including mortality, aortic dissection, and reoperation in patients without RAAS blockade (group 1, n=27) to those with (group 2, n=63). The aortic growth rate was calculated by dividing the diameter change on CT scans taken immediately post-operatively and the latest scan available. RESULTS: There were no differences in clinical parameters except for age which was higher in patients with RAAS blockade. In group 1, 2 (7%) deaths, 5 (19%) aortic dissections, and 7 (26%) reoperations occurred. In group 2, 3 (5%) deaths, 2 (3%) aortic dissections, and 3 (5%) reoperations occurred. A Kaplan-Meier plot demonstrated improved survival free from major aortic events in group 2. On multivariate Cox, RAAS blockade was an independent negative predictor of major aortic events (hazard ratio 0.38, 95% confidence interval 0.30-0.43, p=0.002). Mean diameter change in descending thoracic and supra-renal abdominal aorta was significantly higher in patients without RAAS blockade (p<0.05). CONCLUSION: In MFS patients who underwent ARR, the addition of RAAS blockade to beta-blocker was associated with reduction of aortic dilatation and clinical events.

Resultados da substituiçäo da aorta ascendente e valva aórtica com reimplante de artérias coronárias / Results of replacement of ascending aorta and aortic valve with reimplantation of coronary arteries

Avaliar a técnica de substituiçäo da valva aórtica e aorta ascendente com reimplante de artérias coronárias no tratamento cirúrgico de aneurismas da aorta ascendente. Entre janeiro de 1980 e dezembro de 1989, 38 paciente sforam operados por essa técnica. Vinte e dois pacientes eram portadores de aneurisma da aorta ascendente com insuficiência da valva aórtica; 14 de dissecçäo crônica de aorta e dois foram operados por dissecçäo na fase aguda. Quatro doentes haviam sido operados previamente por outras técnicas. A mortalidade imediata foi de 5,2%; um paciente por baixo débito e outro por complicaçäo neurológica. Cinco doentes (13,1%) morreram tardiamente, dos quais dois subitamente. O seguimento dos 31 sobreviventes variou de 2 a 72 meses com média de 25; (29(93,5%) estavam na classe funcional I e dois em classe II. Dezesseis pacientes foram submetidos a reestudo por um ou mais dos seguintes métodos: angiografia digital, tomografia computadorizada, ecocardiograma ou angiografia convencional, de 6 a 60 meses após a operaçäo, com média de 33 meses. Todos apresentavam boas condiçöes dos óstios coronários e das próteses. Os resultados imediatos e tardios foram semelhantes nos pacientes operados por dissecçäo ou aneurisma verdadeiro. A técnica de substituiçäo de valva aórtica e aorta ascendente com reimplante de artérias coronárias apresenta baixa mortalidade e bons resultados tardios

Purpose - To analyse a 10-year experience with the Bentall and De Bono technique for surgical treatment of aneurysms of ascending aorta. Patients and Methods - From January 1980 to December 1989, the Bentall and De Bono technique was employed in 38 patients. Twenty-two patients had aneurysm of ascending aorta with aortic insufficiency; 14 had chronic aortic dissections. Four patients were operated on previously by other techniques. Results The immediate mortality was 5.2%; one patient due to low-output syndrome and one had neurological complications. Five patients (13.1%) died late postoperatively. The surviving 31 patients were followed up from two to 72 months (mean 25). Of these, 29 (93.5%) were in functional class I and two in class II. Sixteen patients had late evaluation by one or more of the following methods: digital ang iography, ches t computerized tomography, echocardiography, or conventional angiography, 6 to 60 (mean 33) months after operation. All of them had good conditions of composite valve graft and coronary artery reattachment. The immediate and late results were similar in patients with aneurysms and aortic dissections. Conclusion - The aortic valve and aortic ascending portion replacement with reimplantation of coronary arteries is of low mortality and fairly good late outcome