Luz Polo

Incidence and Predictors of Sudden Cardiac Arrest in Adults With Congenital Heart Defects Repaired Before Adult Life

Many adult survivors of repaired congenital heart disease (CHD) are at premature risk of death. Sudden cardiac arrest (SCA) is 1 of the leading causes of death but little is known about determinants for SCA in adults with repaired lesions. We sought to determine incidence and risk factors for SCA in a study population of 936 adults with previously repaired CHD who had completed follow-up at a single tertiary center during a mean period of 9 ± 7 years. Mean age at first examination in our institution was 21 ± 7 years. Diagnostic categories included tetralogy of Fallot (216), coarctation of the aorta (157), transposition complexes (99), single ventricle (55), and other CHD (409). During a total follow-up of 8,387 person-years, 22 patients (2.6 per 1,000 person-years) presented with SCA. Incidence of SCA varied widely between specific lesions; the highest incidence was observed in transposition complexes (10 per 1,000 person-years). Independent predictors of SCA were retrospectively identified using multivariate Cox proportional hazard modeling. Age at initial examination and severely impaired subaortic ventricular systolic function were independent risk factors for SCA (severe subaortic ventricular systolic dysfunction, adjusted hazard ratio 29, 95% confidence interval 11 to 72, p <0.001). SCA occurred in 23% of patients with severe subaortic ventricular systolic dysfunction versus 0.7% of patients with nonsevere decreased subaortic ventricular function (p <0.001). In conclusion, severe subaortic ventricular systolic dysfunction is a dominant multivariate predictor of SCA in an unselected population of adult survivors after surgery for CHD. Our data support the consideration of primary prevention strategies in these patients.

Comparison of Outcomes in Adults With Congenitally Corrected Transposition With Situs Inversus Versus Situs Solitus

The long-term outcome of patients with congenitally corrected transposition of the great arteries is mainly determined by progressive morphologically tricuspid valve regurgitation, heart block, atrial arrhythmias, and/or systemic ventricular dysfunction. Situs abnormalities have been reported in ≤34% of cases, but whether clinical differences exist between a situs inversus and situs solitus arrangement has not yet been studied. The clinical records of 38 adults with congenitally corrected transposition of the great arteries (mean age 40 ± 15 years) followed for a mean period of 7.4 years were reviewed. Of these 38 patients, 8 presented with situs inversus and 30 with situs solitus. No significant differences were found between the 2 groups in age, gender, ventricular septal defect, pulmonary tract stenosis, previous surgical repair, or duration of follow-up. However, none of the patients with situs inversus presented with an Ebstein-like anomaly of the morphologically tricuspid valve and none developed nonoperative-related complete atrioventricular block compared to 15 (50%; p = 0.013) and 11 (42%; p = 0.032) of the patients with situs solitus, respectively. At follow-up, 2 patients with situs inversus (25%) presented with sustained atrial arrhythmia, severe tricuspid regurgitation, or severe systemic right ventricular systolic dysfunction compared to 22 (73%) of 30 those with situs solitus (p = 0.034). No patient with situs inversus presented with cardiac death or severe heart failure compared to 12 (40%) of 30 with situs solitus (p = 0.038). In conclusion, Ebstein-like anomaly or spontaneous complete atrioventricular block are rare in patients with congenitally corrected transposition of the great arteries with situs inversus, and late complications are uncommon. The long-term outcome of patients with situs inversus was significantly better than that for patients with situs solitus.

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.

Platypnea-orthodeoxia syndrome after failed percutaneous closure of secundum atrial septal defect.

We report a 51-year-old patient with platypnea-orthodeoxia syndrome after percutaneous closure of a secundum atrial septal defect, an unusual complication of this modality of treatment. Echocardiography, the main diagnostic technique in the present case, showed that one of the percutaneous devices rims was fixed to the anterior wall of the inferior cava vein. Furthermore it showed that the blood flowed from the inferior cava vein, through the defect in the atrial septum, into the left atria.

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.

Right pulmonary branch stent migration

A 24-year-old man present with ostial stenosis in the left pulmonary artery branch, causing a pressure gradient >25mm Hg. He underwent stent insertion, but on balloon liberation, the stent migrated to the pulmonary trunk. Subsequently, it lodged in a lobar branch of the distal right pulmonary artery. When reviewed 1 month later, a posteroanterior chest radiograph showed an anteroposterior position of the stent (Figure 1). Angiography confirmed that the stent lay perpendicular to the right pulmonary artery. Although the patient remained clinically asymptomatic, we considered that surgery was indicated to remove the stent and treat the left pulmonary artery stenosis. An arteriotomy was performed in the right pulmonary branch, and the stent was removed (Figure 2). It was observed that the left pulmonary stenosis was caused by traction produced by the ductal ligament which was resected, and the right pulmonary branch was enlarged with a PTFE patch (Figure 3). Figure 1. Chest radiograph in posteroanterior view, showing the anteroposterior position of the stent.