Juan J. Rufilanchas

Efficacy of losartan vs. atenolol for the prevention of aortic dilation in Marfan syndrome: a randomized clinical trial

AIMS To determine the efficacy of losartan vs. atenolol in aortic dilation progression in Marfan syndrome (MFS) patients. METHODS AND RESULTS A phase IIIb, randomized, parallel, double-blind study was conducted in 140 MFS patients, age range: 5-60 years, with maximum aortic diameter <45 mm who received losartan (n = 70) or atenolol (n = 70). Doses were raised to a maximum of 1.4 mg/kg/day or 100 mg/day. The primary end-point was the change in aortic root and ascending aorta maximum diameter indexed by body surface area on magnetic resonance imaging after 36 months of treatment. No serious drug-related adverse effects were observed. Five patients presented aortic events during a follow-up (one in the losartan and four in the atenolol groups, P = 0.366). After 3 years of follow-up, aortic root diameter increased significantly in both groups: 1.1 mm (95% CI 0.6-1.6) in the losartan and 1.4 mm (95% CI 0.9-1.9) in the atenolol group, with aortic dilatation progression being similar in both groups: absolute difference between losartan and atenolol -0.3 mm (95% CI -1.1 to 0.4, P = 0.382) and indexed by BSA -0.5 mm/m2 (95% CI -1.2 to 0.1, P = 0.092). Similarly, no significant differences were found in indexed ascending aorta diameter changes between the losartan and atenolol groups: -0.3 mm/m2 (95% CI -0.8 to 0.3, P = 0.326). CONCLUSION Among patients with MFS, the use of losartan compared with atenolol did not result in significant differences in the progression of aortic root and ascending aorta diameters over 3 years of follow-up.

Reoperation for dysfunction of the Björk-Shiley mitral disc prosthesis: report of eight cases.

Of 193 patients with Björk-Shiley mitral valve prostheses, replacement was necessary in 8 (4.1%). The reasons for reoperation were: detachment (4), thrombosis (1), technical error (1), and late disc entrapment (2). Five of these patients died (62.5%), the death being directly related to the need for urgent operation because of low cardiac output. We recommend avoiding the use of the larger sized Björk-Shiley prostheses, since striking of the disc against the ventricle wall, probably consequent to postoperative decrease in heart size, may appear even 1 year after implantation of the prosthesis. An early diagnosis and early reoperation offer these patients a much more favorable prognosis.

Cardiac Echinococcosis: Report of 3 Cases and Review of the Literature

Three patients who underwent successful surgical treatment of cardiac hydatid disease are discussed. The nonspecificity of diagnostic measures and the importance of keeping this diagnosis in mind when faced with a patient coming from an area where hydatidosis is endemic are stressed. We propose the use of cardiopulmonary bypass in the surgical treatment of this problem.

Aortic Valve-Sparing in 37 Patients With Marfan Syndrome: Midterm Results With David Operation

BACKGROUND We reviewed our experience with aortic valve-sparing operations in Marfan syndrome during last 5 years. METHODS Between March 2004 and June 2009, 94 patients with aortic root aneurysms underwent valve-sparing operations. Of these, 37 (68% male) were diagnosed with Marfan syndrome, according to the Ghent diagnostic criteria. Mean age was 30 +/- 10 years (range, 11 to 59 years). Moderate/severe aortic regurgitation was present in 13%, and the mean diameter of the Valsalva sinuses was 50 +/- 4 mm (range, 42 to 62 mm). The David V modification was performed in the last 28 patients. Additional procedures were mitral valve repair in 6, tricuspid valve repair in 3, closure of septal atrial defect in 2, and closure of a patent foramen ovale in 13. Mean follow-up was 27 +/- 16 months (range, 1 to 61 months). RESULTS There were no in-hospital deaths and no major adverse outcomes. One patient required implantation of a mechanical prosthesis during the same procedure because of moderate aortic regurgitation. One late death occurred. No patients required reoperation. In the last follow-up, 23 patients did not have aortic regurgitation, 12 had grade I, and 1 had grade II. No thromboembolic complications have been documented, and 97% of the patients are free from anticoagulation. CONCLUSIONS Short-term and midterm results with the reimplantation technique for aortic root aneurysms in Marfan patients are excellent. If long-term results are similar, this technique could be the treatment of choice for these patients.

Cirugía de preservación valvular en 120 pacientes con aneurismas de la raíz aórtica

INTRODUCTION AND OBJECTIVES Several aortic valve sparing techniques have been described for the treatment of aortic root aneurysms. We report our experience using the reimplantation technique in 120 patients. METHODS Between March 2004 and October 2010, 120 patients with aortic root aneurysms underwent David operations. Of these, 51 were diagnosed with Marfan syndrome. Mean patient age was 31 ± 12 years. The mean diameter of the sinuses of Valsalva was 51 ± 5 mm and moderate/severe aortic regurgitation was present in 16% of these patients. In the other 69 patients mean age was 56 ± 14 years, the mean diameter of the sinuses of Valsalva was 53 ± 7 mm and moderate/severe aortic regurgitation was present in 66%. A bicuspid aortic valve was presented in 14 cases. RESULTS Hospital mortality was 1.7%. Mean follow-up was 37 ± 21 months; 94% of the patients survived and 96% had an aortic regurgitation below grade II during 5 years of follow-up. One patient required re-operation because of severe aortic regurgitation. No endocarditis or thromboembolic complications have been documented, and 96% of the patients did not receive any anticoagulation therapy. CONCLUSIONS Short- and mid-term results with the reimplantation technique for aortic root aneurysms are excellent. This technique prevents the need for chronic anticoagulation treatment as well as the complications arising from mechanical prostheses, and it should be the treatment of choice for young patients.

CARDIAC VALVE REPLACEMENT WITH THE BJORK-SHILEY PROSTHESIS IN YOUNG PATIENTS

Between 1971 and 1974, 26 valve replacements with the Björk-Shiley tilting disc valve prosthesis were performed in 23 children between 4 and 16 years of age. Mitral valve replacement was carried out in 11 patients, aortic valve replacement in 9 and double replacement in the other 3 patients. Several of the patients presented associated lesions which were also corrected at the same intervention, One patient died during the postoperative period; the remaining 22 all showed significant clinical remission. No incidents of thromboembolism or complications of any other nature were observed during follow-up periods of 6 to 26 months. It is our belief that the Björk-Shiley valve represents an improvement over other prostheses currently used for the surgical correction of valve disease in children.

Experiencia inicial con la preservación de la válvula aórtica en el síndrome de Marfan

Introduccion y objetivos La preservacion de la valvula aortica nativa descrita por David ha demostrado ser igual de eficaz que la tecnica de Bentall-Bono, pero ademas evita la anticoagulacion cronica y las complicaciones de las protesis mecanicas. Presentamos nuestra experiencia inicial con esta tecnica en pacientes con sindrome de Marfan. Metodos Desde abril de 2004 hasta abril de 2006 se ha realizado la tecnica de David en 40 pacientes con aneurisma de raiz de aorta. Dieciocho pacientes tenian sindrome de Marfan, con una mediana de edad de 29 anos (intervalo, 13-55 anos). En el estudio ecocardiografico, la mediana del diametro de los senos de Valsalva fue de 53 mm (intervalo, 46-59 mm). Resultados En 17 pacientes se pudo preservar la valvula aortica. No hubo mortalidad hospitalaria ni ninguna complicacion resenable. En el estudio ecocardiografico previo al alta ningun paciente mostro una insuficiencia aortica mayor de grado II. La mediana de seguimiento es de 8 meses (intervalo, 1-24 meses) y se ha producido una muerte por rotura de un aneurisma abdominal. El resto de los pacientes estan en clase funcional I. Conclusiones La preservacion de la valvula aortica mediante reimplante valvular ha mostrado unos resultados excelentes. Evita las complicaciones tromboembolicas y hemorragicas derivadas de las protesis y de la anticoagulacion cronica. Si la valvula aortica reimplantada mantiene una funcionalidad adecuada a largo plazo, deberia convertirse en la tecnica de eleccion en la cirugia de los aneurismas de aorta ascendente en el sindrome de Marfan.

Long-Term Results of Cardiac Valve Replacement with the Delrin-Disc Model of the Björk-Shiley Valve Prosthesis

One hundred two Björk-Shiley valve prostheses with the Delrin-disc occluder were implanted in 83 patients between January, 1971, and July, 1972. Fifty-eight were in the mitral position, 42 in the aortic, and 2 in the tricuspid. Complete follow-up until 1981 was obtained in 93% of the patients (mean follow-up, 66.8 months). Hospital mortality was 18% and late mortality, 19%. Survival according to actuarial methods was 84.8% at 5 years and 78.1% at 9 years after operation. Thromboembolism was detected in 8.8% of patients but caused no deaths. The incidence was 1.2% and 1.5% per year in those patients treated with Coumadin and antiplatelet agents, respectively. Reoperation was necessary in 13% of the patients. Most survivors (72%) are in New York Heart Association Functional Class I, despite a preoperative status of Class III or IV in 57% of the patients. Hospital mortality may be due to poorer understanding of patient management and less refined techniques of myocardial protection. Long-term survival with this prosthesis is similar to that in more recent studies, and rates of thromboembolism and malfunction compare favorably with other prosthetic valves.

Coarctation of the aorta and severe aortic insufficiency: What to repair first?

A new surgical approach is proposed for patients with coarctation of the aorta associated with severe aortic valvular insufficiency. The valvular lesion should be repaired first and the coarctation corrected during a second operation; both interventions should be done during the same hospital stay. We base our approach on the belief that improved coronary perfusion can be achieved when the aortic insufficiency is corrected first. The disadvantages of the opposite surgical approach, such as anticoagulation problems, renal underperfusion, and hypertensive complications are easily avoided.

Combined Mitral and Aortic Valve Replacement with the Björk-Shiley Prosthesis

Combined mitral and aortic valve replacement with the BJORK-Shiley tilting-disc valve prosthesis was performed in 42 patients. Hospital and late mortality rates were both at the 9.5% level. No correlation was seen between mortality and combination of concomitant valve lesions. Morbidity was elevated. No episodes of embolism were observed after surgery, although one patient died of severe haemorrhage. Only one patient showed valvular dysfunction (grade 2/4 leakage), but did not require reoperation. Postoperatively, 75.7% of the patients were asymptomatic for a mean follow-up period of 21.1 months. The Björk-Shiley prosthesis offers a small gradient associated with a low profile, which constitute important advantages in multiple valve replacement.