Víctor Caicedo

Concomitant mitral valve or atrial septal defect surgery and the modified Cox-maze procedure

Atrial fibrillation (AF) is generally associated with rheumatic valve disease and atrial septal defects (ASD) in young adults. Surgical correction of both disorders fails to convert to sinus rhythm or prevent further episodes of paroxysmal or chronic AF in most patients. The role and efficacy of combining mitral valve surgery or ASD correction with AF surgery in this setting has not been widely addressed and remains to be established. The present study prospectively assessed the recovery of sinus rhythm, functional status, and atrial function in 21 patients (mean age 42 +/- 9.2 years) who underwent a modified Cox-maze procedure concomitant with mitral valve or ASD surgery at our institution between March 1993 and February 1995. Seventeen (81%) had chronic AF, and 4 (19%) had paroxysmal AF, with a mean AF duration of 3.5 +/- 3.6 years (range 0.6 to 15.3). Concomitant surgery was performed in 9 patients (42.9%) with mitral stenosis, 5 (23.8%) with mitral regurgitation, 1 (4.8%) with mitral and aortic regurgitation, and 3 (14.3%) with ASD. Eighteen patients (86%) were in New York Heart Association class II to IV before operation. Doppler echocardiography was performed in all patients before surgery, and 1 week, and 3 and 6 months after surgery in patients maintaining sinus rhythm. One patient with severe mitral stenosis and depressed ventricular function died in the immediate postoperative period. Sinus rhythm was restored in the immediate postoperative period in 7 patients (35%), and in another 10 patients (50%) before discharge (mean 5.8 +/- 2 days). Overall, sinus rhythm was restored before discharge in 17 patients (85%); 3 (15%) patients required antiarrhythmic therapy. Doppler echocardiography performed 3 months after surgery documented atrial contractility (A and E waves) in 12 patients (71%). After a mean follow-up period of 8 months (range 3 to 23), 18 (90%) remained in sinus rhythm. Sinus rhythm was successfully restored and maintained in most patients with drug refractory AF undergoing a concomitant Cox-maze procedure with mitral valve or ASD surgery improving atrial function and New York Heart Association class.

Search of somatic GATA4 and NKX2.5 gene mutations in sporadic septal heart defects

High prevalence of somatic mutations in the cardiac transcription factor genes NKX2.5 and GATA4 have been reported in the affected cardiovascular tissue of patients with isolated cardiac septal defects, suggesting a role of somatic mutations in the pathogenesis of these congenital heart defects (CHDs). However, all somatic mutations have been identified in DNA extracted from an archive of formalin-fixed cardiac tissues. In the present study, to address the hypothesis that somatic mutations are important in isolated CHDs, we analyzed the GATA4 and NKX2.5 genes in the fresh-frozen pathologic cardiac tissue specimen and corresponding non-diseased tissue obtained from a series of 62 CHD patients, including 35 patients with cardiac septal defects and 27 with other cardiac anomalies. We identified one variant and two common polymorphisms in the NKX2.5 gene, and six variants and two common polymorphisms in the GATA4 gene. All identified variants were seen in both the fresh-frozen pathologic cardiac tissue and the corresponding non-diseased tissue, which indicates that they all were constitutional variants. The present study has identified NKX2.5 and GATA4 constitutional variants in our CHD cohort, but was unable to replicate the previously published findings of high prevalence of somatically derived sequence mutations in patients with cardiac septal defects using fresh-frozen cardiac tissues rather than formalin-fixed tissues.

Arco aórtico derecho circunflejo retroesofágico con ligamento arterioso izquierdo: una variedad infrecuente de anillo vascular completo Presentación de un caso

Los anillos vasculares pertenecen a un grupo de anomalias congenitas de los arcos aorticos en los que la traquea y el esofago, o ambos, estan completamente rodeados por estructuras vasculares. Con frecuencia pueden causar obstruccion y, en consecuencia, alteracion de la deglucion y dificultad respiratoria, por lo cual deben incluirse en el diagnostico diferencial de obstruccion de la via aerea superior. El diagnostico temprano y la liberacion quirurgica oportuna de la obstruccion de la via aerea y del esofago, o ambos, pueden mejorar los sintomas en la mayoria de casos. Esta afeccion debe sospecharse y evaluarse en lactantes o ninos pequenos con sintomas respiratorios recurrentes como tos cronica, estridor y sibilancias o, lo que es menos comun, con sintomas relacionados con alteracion de la deglucion. A continuacion se ilustra el caso de un nino de seis anos con problemas de deglucion cronicos y desnutricion a quien se le realizo un diagnostico incidental del arco aortico derecho circunflejo retroesofagico con ligamento arterioso izquierdo corregido mediante cirugia. Vascular rings are a group of congenital anomalies of the aortic arches in which the trachea and esophagus, or both, are completely surrounded by vascular structures. Often, they can cause obstruction and consequently impaired swallowing and respiratory distress, so they must be included in the differential diagnosis of obstruction of the upper airway. Early diagnosis and timely surgical release of obstruction of the airway and esophagus, or both, may improve symptoms in the majority of cases. This condition must be suspected and evaluated in infants or young children with recurrent respiratory symptoms such as chronic cough, stridor, wheezing or, less commonly, with symptoms related to impaired swallowing. We illustrate the case of a six-year-old child with chronic swallowing problems and malnutrition who underwent an incidental diagnosis of circumflex retroesophageal right aortic arch with left ligamentum arteriosum corrected by surgery.

Anomalía del retorno venoso sistémico Drenaje anómalo de la vena cava superior derecha a la aurícula izquierda. Revisión de la literatura y reporte de caso

La anomalia total del retorno venoso sistemico tiene gran variedad de presentaciones; sin embargo, la patologia de mas baja frecuencia es el drenaje de vena cava superior derecha a la auricula izquierda, hecho de peso para que en el mundo se reporten pocos casos. En la Fundacion Clinica Abood Shaio se trato el caso de una paciente de seis anos de edad con drenaje venoso total de cava superior derecha a la auricula izquierda, mediante la tecnica de movilizacion de cava superior y anastomosis cavo-atrial, y se obtuvieron buenos resultados. El caso es mencion corresponde al numero 21 en la literatura mundial.

Aneurisma de la arteria coronaria derecha: Revisión de la literatura y reporte de casos

El aneurisma de la arteria coronaria derecha es una entidad rara, que se presenta principalmente en mujeres. Las causas mas frecuentes de muerte por aneurisma son en su orden: ruptura, trombosis, isquemia. La enfermedad aterosclerotica es la causa mas frecuente de aneurisma espontaneo. La mayoria de los pacientes son asintomaticos, y presentan soplo continuo o moderada cardiomegalia, y pletora en la radiografia de torax. En etapas avanzadas produce cardiopatia isquemica. Para su diagnostico es necesario un juicio clinico adecuado y examenes de rutina para enfermedad coronaria. Se exponen dos casos tratados en la Fundacion Clinica A. Shaio, con base en los cuales se analiza el diagnostico, las complicaciones y el tratamiento realizado.