Ángel Aroca

Risk of Aortic Root or Ascending Aorta Complications in Patients With Bicuspid Aortic Valve With and Without Coarctation of the Aorta

The actual incidence of ascending aorta complications (AACs) in adults with bicuspid aortic valve (BAV) and the role of associated coarctation of the aorta (COA) as an independent risk factor for AACs remain unknown. From the Adult Congenital Heart Disease database at La Paz Hospital, 631 patients in whom a BAV was diagnosed by echocardiography or surgical inspection since December 1989 were identified. These patients were then further subdivided into 2 groups according to the presence of an associated COA. AACs included aortic aneurysms (ascending aorta > or =55 mm) and aortic dissection, rupture, or perforation. Patients with a BAV and COA had a greater prevalence of AACs (8.0%) than those with an isolated BAV (3.7%; p = 0.037). The coexistence of COA was the only significant predictor of AACs (odds ratio 4.7, 95% confidence interval 1.5 to 15; p = 0.01). From the total patient group with a BAV, the clinical and echocardiographic data were reviewed for 341 patients without an AAC at baseline (97 with and 244 without COA) who had undergone serial examinations >1 year apart. The median follow-up was 7 years (interquartile range 3.5 to 10.2; total 2,436 patient-years). A new AAC occurred in 13 patients (0.5/100 patient-years). The incidence of AACs was 1.3/100 patient-years in the COA group versus 0.2/100 patient-years in the non-COA group (hazard ratio 7.5, 95% confidence interval 2.0 to 28, p = 0.002). All acute aortic events (dissection or rupture) at follow-up occurred in patients with a BAV and COA. In conclusion, the long-term incidence of AACs in patients with isolated BAV is low, but patients with BAV and associated COA are at increased risk.

Rapid progression of midventricular obstruction in adults with double-chambered right ventricle.

OBJECTIVE The purpose of this study was to determine the rate of progression of midventricular obstruction in adolescents and adults with double-chambered right ventricle. METHODS Clinical and echocardiographic findings in 45 patients (mean age 26 +/- 6 years, range 15-44) diagnosed with double-chambered right ventricle were retrospectively analyzed. Twenty patients underwent surgical repair before the age of 15 years. The relationship between Doppler midventricular pressure gradient and patient age was analyzed in 25 patients without previous repair. Sequential change in midventricular obstruction was determined for patients with 2 or more Doppler echocardiographic examinations performed within at least a 2-year interval. RESULTS Right midventricular pressure gradient in nonrepaired patients was 70 +/- 38 mm Hg (range 25-150). A significant relationship between midventricular obstruction and patient age (r = 0.64, P <.001) was found. Midventricular pressure gradient at initial evaluation was 32 +/- 27 mm Hg in 16 patients < 25 years and 73 +/- 45 mm Hg in 9 patients >/= 25 years (P <.03). After the initial study, 5 patients underwent surgical repair and 13 patients without repair were followed up for a period of 6.1 +/- 2.7 years (range 2-9), in which midventricular pressure gradient increased from 32 +/- 26 mm Hg to 67 +/- 35 mm Hg (P <.001). The slope of the change in midventricular pressure gradient was 6.2 +/- 3 mm Hg per year of follow-up. Seven more patients underwent surgical repair during follow-up due to progression of the obstruction. There was no mortality nor residual midventricular obstruction in surgically repaired patients. CONCLUSIONS Mild right midventricular obstruction shows a fast rate of progression in adolescents and young adults. Thus, close clinical and echocardiographic follow-up is advised, and surgical repair should be considered if significant progression of obstruction is detected.

Chimney Technique for Mitral Valve Replacement in Children

Severe mitral stenosis is unusual in children, but it represents an important challenge for surgeons because of the scarcity of solutions. Several mitral percutaneous and surgical valvuloplasties are performed repetitively to delay mitral valve replacement. Most of the time these procedures show discouraging results. When mitral valve replacement is performed, the annulus may not be large enough to fit a substitute. We present, to our best knowledge, a new technique to implant a large prosthesis in a small annulus without negatively affecting the opening of the leaflets.

Factores de riesgo asociados a la cirugía del adulto con cardiopatía congénita: 22 años de experiencia. ¿Quién debe intervenirlos?

OBJECTIVE To assess the association between mortality in surgery of congenital heart disease in adults, and factors related to patients and operations. METHOD Descriptive study of operations performed by specialized surgeons in congenital heart surgery (238), adult acquired surgery (117), and specialty residents (108). The association of mortality with surgical risk and complexity, specialization of surgeon, cardiopulmonary by-pass and aortic cross clamping was assessed fitting logistic regression models. RESULTS A total of 463 operations were included (442 with cardiopulmonary by-pass) in the study performed between 1991 and 2012. Median age at surgery: 34; 52.8% were women. First surgery: 295, reoperation: 168. Median score of Aristotle was 6.8, with significantly higher complexity since 2001, after restructuring the Unit. Overall hospital mortality was 3.9%. Mortality was significantly associated to number of previous surgeries (OR: 5.02; 95%CI: 1.44-17.52), operations by acquired heart disease surgeons (OR: 3.53; 95%CI: 1.14-10.98), higher Aristotle (OR: 1,64; 95%CI: 1.18-2.29), and high cardiopulmonary by-pass time (OR: 1.13; 95%CI: 1.07-1.19). CONCLUSIONS Surgery of congenital heart disease in adults has been performed with low mortality. High complexity interventions, prolonged cardiopulmonary by-pass times and multiple reoperations were associated to higher mortality. Participation of cardiac surgeons specialized in congenital heart disease is associated with better outcomes.

Pulmonary hypertension in young adults with repaired coarctation of the aorta: An unrecognised factor associated with premature mortality and heart failure

BACKGROUND Adults with repaired coarctation of the aorta (CoA) are at risk for premature cardiovascular death or heart failure (HF). We sought to evaluate risk factors for death or HF in young adults with repaired CoA in childhood. METHODS The medical records of a cohort of 159 adults diagnosed with CoA repaired at a mean age of 4.1 ± 5.7 years were retrospectively reviewed to identify predictors of a combined endpoint of all cause death or hospitalisation for HF by using Cox proportional hazard models. RESULTS Over a follow-up of 26 ± 8 years (median 27 years) after repair, 5 patients died and 7 developed HF requiring hospitalisation. Pulmonary artery systolic pressure >40 mm Hg (PH) was the main predictor of death or admission for HF (HR 32; 95% CI 4.0-250; p=0.001). Neither systemic hypertension, recoarctation, aortic aneurysm, intracardiac lesions nor treatment with beta-blockers or ACEi/ARBs were statistically related to death or HF. Restrictive left ventricular physiology (RLVP) was the main predictor of PH by logistic regression analysis (OR 31; 95% CI 10-92; p<0.001). Patients with severe PH (pulmonary artery systolic pressure >60 mm Hg) showed RLVP (9/10), severe elevation of end-diastolic LV pressure at cardiac catheterization (8/8) and subendocardial late gadolinium enhancement on MRI (5/5). Pathological examination of an explanted heart demonstrated extensive left ventricular subendocardial fibrosis. CONCLUSIONS Pulmonary hypertension related to restrictive left ventricular physiology was the strongest predictor of death or HF in young adults with CoA repaired in childhood. Persistent subendocardial fibrosis might be the structural substrate in some patients.

Cierre quirúrgico de la comunicación interauricular antes o después de los 25 años de edad. Comparación con la evolución natural en pacientes no operados

Introduccion El cierre quirurgico de la comunicacion interauricular (CIA) antes de los 25 anos de edad disminuye las complicaciones durante la vida adulta, pero las consecuencias de la intervencion en pacientes mayores de 25 anos siguen siendo motivo de controversia. Metodos Para comparar los efectos de la cirugia precoz y/o tardia con la evolucion natural se ha estudiado, de forma retrospectiva, a 280 adultos (edad media 40 ± 18 anos) con CIA no restrictiva. Ciento dos pacientes (grupo 1) habian sido operados antes de los 25 anos, 90 (grupo 2) habian sido operados despues de los 25 anos y 88 (grupo 3) no habian sido operados previamente. Se comparo la presion pulmonar sistolica, el tamano del ventriculo derecho y la auricula izquierda, el grado de insuficiencia mitral y tricuspide, la funcion sistolica del ventriculo izquierdo y la prevalencia de fibrilacion auricular. Resultados No habia diferencia en la funcion sistolica del ventriculo izquierdo o en el grado de insuficiencia mitral entre los tres grupos. Comparado con el grupo 2, el grupo 1 tenia menores presion sistolica pulmonar (p Conclusiones El cierre quirurgico de la CIA despues de los 25 anos no previene el deterioro hemodinamico o el desarrollo de arritmias auriculares, por lo que se deberia concentrar esfuerzos en corregir el defecto antes de la edad adulta.

Usefulness of 64-detector computed tomography in the diagnosis and management of patients with congenital heart disease.

INTRODUCTION AND OBJECTIVES Although congenital heart defects are the most common major congenital abnormalities, the associated mortality has been decreasing due to improvements in their diagnosis and treatment. We assessed the usefulness of 64-multidetector computed tomography in the diagnosis and management of these patients. METHODS This 5-year observational, analytical, retrospective, cohort study included a total of 222 tomographic studies of patients with congenital heart disease. Computed tomography scans were read twice and medical records were reviewed. We assessed the complexity of the disease, patient, and radiological technique, and evaluated the contribution of new data in relation to clinical suspicion and diagnostic change. A confidence interval was set at 95% and a P value of<.05 was used as the cutoff for statistical significance. RESULTS In 35.1% of patients, the treatment procedure was performed after computed tomography without other tests. Additional diagnostic catheterization was performed in 12.5% of patients. There were new findings in 77% of patients (82.9% with complex disease), which prompted a change in patient management in 35.6%. All unexpected reports described new findings. No significant differences were found by age, sex, study period, urgency of the test order, patient complexity, or difficulty of the technique. CONCLUSIONS Use of 64-detector computed tomography yields good diagnostic performance in congenital heart disease, prompts changes in management in more than one-third of patients, and reveals new findings in relation to the presumed diagnosis in 77% of patients.

Cirugía de la transposición de las grandes arterias con septo ventricular íntegro

La transposicion simple de las grandes arterias (TGA) con septo interventricular integro es una cardiopatia congenita cianogena frecuente, representando un 8% de las cardiopatias congenitas. Su mortalidad sin tratamiento quirurgico es muy elevada, siendo de aproximadamente el 45% en el primer mes, con una supervivencia al ano del 10%. Tras las correcciones fisiologicas tipo Mustard o Senning practicamente abandonadas en la actualidad, la correccion anatomica ( switch arterial) es la intervencion que, junto con la mejoria en el tratamiento pre y postoperatorio, ha llevado a los excelentes resultados en el tratamiento de los pacientes con TGA. Se revisan la fisiopatologia y la clinica, los criterios diagnosticos, las tecnicas quirurgicas y los resultados en cuanto a mortalidad hospitalaria y seguimiento tardio. La correccion anatomica de la TGA es, ademas, el principal marcador de calidad de un programa de cirugia cardiaca neonatal.

Interventional catheterization after the Norwood procedure

INTRODUCTION AND OBJECTIVES To carry out a retrospective analysis of the indications for, and the results and complications of interventional catheterization after the Norwood procedure. METHODS Between February 1993 and December 2006, 25 interventional catheterizations were performed in 14 patients who had undergone the Norwood procedure, prior to the Glenn or Fontan procedure. RESULTS Nine angioplasties were carried out for recoarctation in seven of the 14 patients (2 patients developed restenosis after their first angioplasty). Detachment of the left pulmonary artery occurred either immediately or during follow-up in 3 patients who underwent the classical Norwood procedure. Overall, 10 pulmonary artery angioplasties were required in 7 patients. Three patients needed embolization: one of venous collaterals (using coils), one of the left superior vena cava (using an Amplatzer duct occluder), and one of a left Blalock-Taussig shunt (using an Amplatzer duct occluder). Two patients required a cavopulmonary (Glenn) anastomosis, and another underwent fibrinolysis for thrombosis of the superior vena cava and pulmonary artery. Other findings, which were not treated percutaneously, included: stenosis of the supra-aortic trunk (n=6), femoral artery stenosis (n=2), femoral vein thrombosis (n=5), and subclavian vein thrombosis (n=1). The following complications were recorded: arterial ischemia (n=2), cardiac arrest or bradycardia (n=4), and transient atrioventricular block (n=1). CONCLUSIONS Following stage I of the Norwood procedure, the angiographic and hemodynamic assessments needed for the diagnosis and treatment of pulmonary artery or aortic arch stenosis must be carried out promptly. Although treating recoarctation by angioplasty can be effective, restenosis frequently occurs. With the Sano procedure, detachment of the left pulmonary artery, but not pulmonary artery stenosis, can be avoided. After stage II, the presence of venovenous collaterals must be ruled out, because they frequently require embolization. In these patients, interventional catheterization is associated with a higher incidence of complications than in other groups.

Reintervenciones quirúrgicas y trasplante de órganos torácicos

Actualmente, mas del 85% de los pacientes que nacen con una cardiopatia congenita alcanzan la vida adulta, muchos de ellos gracias a procedimientos quirurgicos o hemodinamicos realizados durante la infancia. De los pacientes mayores de 18 anos con cardiopatias congenitas, se estima que casi un 20% necesitan en algun momento de su evolucion un procedimiento intervencionista, la mayor parte de las veces quirurgico, debido bien a diagnosticos fuera de la edad infantil en el caso de las cardiopatias mas benignas, bien a la existencia de lesiones residuales, secuelas y/o complicaciones en el caso de las formas mas complejas intervenidas en etapas precoces de la vida. La intervencion o la reintervencion de estos pacientes durante la edad adulta, excluido el grupo mas sencillo de las comunicaciones interauriculares con o sin drenaje venoso pulmonar anomalo asociado, constituyen una nueva subespecialidad de la cirugia cardiovascular, con tendencia general a que las realicen cirujanos cardiacos que habitualmente operan cardiopatias congenitas. Cuando estas afecciones alcanzan un estadio final debido a la disfuncion severa de uno o ambos ventriculos, problemas tecnicos quirurgicos que impliquen alto riesgo para una cirugia convencional o la presencia de hipertension pulmonar secundaria a enfermedad vascular pulmonar, todavia restan como armas terapeuticas los trasplantes de organos intratoracicos: corazon y pulmones, sea en bloque o por separado. El objeto de este articulo es la revision retrospectiva del subgrupo mas complejo de congenitos adultos intervenidos en la Unidad de Cardiopatias Congenitas del Adulto del Hospital La Paz, aquellos que habian sufrido operaciones previas, bien para reparar una cardiopatia solo paliada, bien para tratar lesiones residuales o secuelas de una cardiopatia previamente corregida, asi como reflejar la experiencia del Hospital Puerta de Hierro en el trasplante de organos intratoracicos en adultos con cardiopatias congenitas.