Violeta Sánchez

Pulmonary vascular remodeling in pulmonary hypertension due to chronic heart failure.

Pulmonary hypertension (PHT) associated with chronic heart failure (CHF) is a risk factor of right ventricular failure after heart transplantation (HT). Our aim was to study pulmonary vascular changes in patients with CHF and to assess any correlation with haemodynamic data.

Impact of mild pulmonary hypertension on mortality and pulmonary artery pressure profile after heart transplantation.

BACKGROUND Pulmonary hypertension is a risk factor for early mortality after transplantation, but the risk threshold is debated. Also, little is known about the evolution of pulmonary circulation after transplantation. The aim of this study was to determine the influence of current risk pulmonary pressure parameters on early post-operative mortality and to assess the time-related changes in pulmonary pressure after surgery. METHODS One hundred twelve consecutive transplanted patients were studied retrospectively to determine the influence of trans-pulmonary gradient of >12 mm Hg and pulmonary vascular resistance of >2.5 Wood units, at baseline or after vasodilator test, on early mortality. A multivariate analysis was used to study the hemodynamic parameters associated with early mortality. The pulmonary pressures of all surviving patients were studied for up to 3 years after surgery. RESULTS Early mortality in the groups with and without pulmonary hypertension were 24.4% and 5.6%, respectively (p =.009). The only variable that was independently associated with early mortality was the pulmonary vascular resistance index (odds ratio = 1.459). Mild pulmonary hypertension disappeared 1 year after heart transplantation. CONCLUSIONS Mild pulmonary hypertension is a risk factor for early postoperative mortality. The hemodynamic parameter most closely associated with early mortality is pulmonary vascular resistance index. The hemodynamic profile of pulmonary circulation after heart transplantation is partially dependent on the level of pulmonary hypertension before transplantation, at least during the first year after surgery.

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.

Guías de práctica clínica de la Sociedad Española de Cardiología en tromboembolismo e hipertensión pulmonar

La hipertension pulmonar primaria es una enfermedad de caracter progresivo, mas frecuente en mujeres jovenes y de mediana edad. Su etiologia se desconoce, aunque existe una predisposicion familiar hasta en un 6% de los casos. Las teorias patogenicas actuales se centran en la existencia de disfuncion endotelial y fallos en los canales ionicos de las fibras musculares lisas del vaso. Las pruebas diagnosticas se dirigen a descartar las causas secundarias y a evaluar la gravedad de la enfermedad. El test vasodilatador agudo es imprescindible para la eleccion del tratamiento mas adecuado. La anticoagulacion oral esta indicada en todos los pacientes. El trasplante de pulmon queda reservado a aquellos casos en los que fracasa el tratamiento medico. La septostomia auricular es un procedimiento paliativo util en casos seleccionados. La hipertension pulmonar tromboembolica cronica es una forma especial de hipertension pulmonar secundaria; aunque indistinguible clinicamente de la hipertension pulmonar primaria, su diagnostico resulta crucial, ya que es posible su curacion mediante la realizacion de tromboendarterectomia pulmonar. El tromboembolismo pulmonar es frecuente en pacientes hospitalizados, presentando elevadas tasas de mortalidad (el 30% en pacientes no tratados). El diagnostico es dificil, ya que puede acompanar o simular otras enfermedades cardiopulmonares. Las pruebas diagnosticas no invasivas tienen una baja sensibilidad y especificidad. Nuevas alternativas como la determinacion de dimero D o la TAC helicoidal incrementan la precision diagnostica. El tratamiento estandar consiste en la administracion de heparina durante 5-10 dias y posteriormente anticoagulantes orales durante 3-6 meses. La prevencion con heparinas o dextranos en los pacientes de alto riesgo ha demostrado claros beneficios.

Aortic biomechanics by magnetic resonance: Early markers of aortic disease in Marfan syndrome regardless of aortic dilatation?

BACKGROUND Previous studies demonstrated the usefulness of MRI in the evaluation of aortic biomechanics in Marfan patients with aortic dilatation. However, these parameters have not been well studied in earlier stages of aortic disease. The present work aimed to study aortic biomechanics: aortic distensibility (AD) and pulse wave velocity (PWV), by MRI in Marfan patients without advanced aortic disease. METHODS Eighty consecutive Marfan patients were compared with 36 age- and sex-matched controls. MRI images at the level of ascending, descending and abdominal aorta were used to determine AD and PWV. RESULTS Marfan patients (27 men; age: 32.0 ± 10.5 years; mean aortic root diameter: 37.2 ± 4.6mm) had lower AD at all levels (ascending 2.6 ± 2.1 vs. 6.2 ± 3.7 mm Hg(-1)·10(-3), p<0.001; descending 3.1 ± 2.0 vs. 8.3 ± 4.2, p<0.001; and abdominal 4.5 ± 2.2 vs. 14.0 ± 5.2, p<0.001), higher aortic arch PWV (8.1 ± 6.5 vs. 4.3 ± 1.8m/s, p<0.01) and ascending-to-abdominal PWV (6.1 ± 3.0 vs. 4.7 ± 1.5m/s, p<0.01) compared with controls. Thirty-five Marfan patients had a non-dilated aortic root (mean aortic root diameter: 34.5 ± 3.8 mm). In multivariable analyses, after adjustment for age, pulse pressure and aortic dimensions, AD remained lower and PWV higher in Marfan patients; even Marfan patients with non-dilated aortic root showed impaired aortic biomechanics compared with controls. Z-score for ascending AD<-3.5 distinguished Marfan patients from controls with 82.5% sensitivity and 86.1% specificity. CONCLUSIONS Aortic biomechanics by MRI were abnormal in the entire aorta in Marfan patients. Moreover, Marfan patients without dilated aortic root showed clear impairment of aortic biomechanics, which suggests that they may be used as early markers of aortic involvement in these patients.

Valoración de la eficacia y la seguridad del losartán frente al atenolol en la prevención de la dilatación de la aorta en el síndrome de Marfan

INTRODUCTION AND OBJECTIVES Marfan syndrome is an inherited disease of the connective tissue. Recent trials have indicated the use of losartan (a transforming growth factor beta inhibitor) in these patients prevents aortic root enlargement. The aim of our clinical trial is to assess the efficacy and safety of losartan versus atenolol in the prevention of progressive dilation of the aorta in patients with Marfan syndrome. METHODS This is a phase III clinical trial conducted in two institutions. A total of 150 subjects diagnosed with Marfan syndrome, aged between 5 and 60 years, of both sexes, and who meet the Ghent diagnostic criteria will be included in the study, with 75 patients per treatment group. It will be a randomized, double blind trial with parallel assignment to atenolol versus losartan (50 mg per day in patients below 50 kg and 100 mg per day in patients over 50 kg). Both growth and distensibility of the aorta will be assessed with echocardiography and magnetic resonance. Follow-up will be 3 years. CONCLUSIONS Efficacy of losartan versus atenolol in the prevention of progressive dilation of the aorta, improved aortic distensibility, and prevention of adverse events (aortic dissection or rupture, cardiovascular surgery, or death) will be assessed in this study. It will also show the possible treatment benefits at different age ranges and with relation to the initial level of aortic root dilation.

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.

Successful heart transplantation in patients with inherited myopathies associated with end-stage cardiomyopathy

UNLABELLED Inherited myopathies in patients with secondary end-stage cardiomyopathies have always been considered a relative contraindication for cardiac transplantation. High operative risk related to muscle impairment and potential graft involvement secondary to the underlying myopathy have been the two main reasons implicated in the poor prognosis. OBJECTIVE The aim of this study was to evaluate the outcome in patients who underwent cardiac transplantation in our hospital due to end-stage cardiomyopathy secondary to inherited myopathies. METHODS Among 311 patients who underwent heart transplantation in our hospital, five (2%) had end-stage cardiomyopathies related to inherited myopathies. Four patients had muscular dystrophy (three Beckers muscular dystrophy and one hips-dystrophy) and the fifth desminopathy. In one patient cardiomyopathy was the initial manifestation of the disease. Mean age at the time of transplantation was 38.6 years (range from 24 to 55). The mean follow-up after transplantation was 57.4 months (range from 13 to 128). The intraoperative and postoperative course of these individuals did not show higher complication rates than other patients. All recipients experienced successful rehabilitation; no evidence of graft dysfunction has been detected during follow-up. All of them are alive with a good performance status. CONCLUSIONS In our experience, patients who underwent heart transplantation due to end-stage cardiomyopathy secondary to inherited myopathy with only a mild degree of muscle impairment did not display higher postoperative nor long-term complications compared to other recipients.

Acute rejection after heart transplantation.

Acute rejection (AR) seems to be less common with current immunosuppressive strategies; however, it remains a major cause of morbidity and mortality in the first year following heart transplantation. Despite great interest in noninvasive methods for detecting rejection, the endomyocardial biopsy remains the standard method for AR identification and, recently, the cardiac biopsy grading system has been reviewed. Moreover, the availability of several immunosuppressive drug combinations has generated confusion in the minds of clinicians. This review will focus on recently published studies that are related to the clinical impact of AR, combination regimens of chronic maintenance immunosuppression and specific therapeutic options for treating AR.

Spanish Acute Aortic Syndrome Study (RESA). Better Diagnosis Is Not Reflected in Reduced Mortality

INTRODUCTION AND OBJECTIVES Because acute aortic syndrome (AAS) is associated with high mortality, early diagnosis and treatment are vital. The aim of the Spanish Acute Aortic Syndrome Study (RESA) was to investigate the effectiveness of current treatment of AAS in a broad range of tertiary care hospitals in Spain. METHODS Between January 2005 and December 2007, 24 tertiary care hospitals reported data on 519 patients with AAS (78% male, mean age 61 +/- 13 years, range 20-92 years): 357 had type-A AAS and 162 had type B. RESULTS The time delay between symptom onset and diagnosis was <24 hours in 67% of cases and >72 hours in 11%. Some 80% of patients with type-A AAS were treated surgically. The interval between diagnosis and surgery was <24 hours in 90% of cases. In patients with type-B AAS, 34% received invasive treatment: 11% had surgery and 23% underwent endovascular procedures. Mortality during hospitalization in patients with type-A disease was 33% in those treated surgically and 71% in those treated medically. Mortality in patients with type-B disease was 17% with medical treatment, 27% with endovascular treatment and 50% with surgical treatment. CONCLUSIONS Despite significant advances in the diagnosis of AAS, in-hospital mortality remains high. The findings of this study are representative of a broad range of unselected patients undergoing treatment for the disease and support the need for continuing improvements in therapeutic approaches to AAS.