Resonancia magnética cardíaca en el seguimiento alejado de pacientes con tetralogía de Fallot / Cardiac Magnetic Resonance in long term follow-up of Tetralogy of Fallot

INTRODUCCIÓN: La tetralogía de Fallot (TF) es la cardiopatía congénita cianótica más frecuente. La insuficiencia pulmonar (IP) y dilatación del ventrículo derecho (VD) son las complicaciones más frecuentes a largo plazo. La resonancia magnética cardiaca (RMC) es el "gold standard" para la evaluación del VD. OBJETIVO: Analizar la información obtenida de las RMC en el seguimiento de pacientes con TF. PACIENTES Y MÉTODO: Se incluyeron RMC realizadas entre 2007 y 2012 a pacientes con TF, reparados con parche transanular (PTA) o ampliación infundibular (AInf) y sin recambio valvular pulmonar (RVP). La fracción de regurgitación pulmonar (FRP), el volumen y función ventricular fueron evaluados. RESULTADOS: Se realizaron 122 RMC a 114 pacientes. Edad promedio al examen 15,4 ± 7,4 años. 53,3% presentó IP severa (> 40%). La media del volumen de fin de diástole del VD (VFDVD) fue 157,3 ± 38,6 ml/m2, fin de sístole (VFSVD) de 85,3 ± 27 ml/m2 y fracción de eyección (FEVD) 46,4 ± 7,1%. 48,4% presentaba un VFDVD mayor de 150 ml/m2 y el 32,8% mayor a 170 ml/ m2. El PTA se relacionó con mayores volúmenes de VD que la AInf. VFDVD mayor a 170 ml/m2 mostraron peor FEVD (FEVD 47,9 ± 7% vs 43,2 ± 6,4%, p < 0,01). DISCUSIÓN: Casi la mitad mostró una significativa dilatación del VD demostrando que la indicación de RMC es tardía en el seguimiento. El PTA se asoció con mayores VFDVD y VFSVD pero no a peor FEVD.

INTRODUCCIÓN: Tetralogy of Fallot (TOF) is the most frequent cyanotic congenital heart disease. Pulmonary regurgitation (PR) and right ventricle (RV) enlargement and dysfunction are the most common long-term complications. Cardiac magnetic resonance (CMR) is the gold standard for RV evaluation. OBJECTIVE: To analyze CMR results in the follow-up of TOF patients. PATIENTS AND METHOD: All CMR performed between 2007 and 2012 in TOF patients with transannular patch (TAP) repair or infundibular widening, and without pulmonary valve replacement (PVR) were included. Pulmonary regurgitant fraction (PRF), ventricular end-diastolic (EDV) and end-systolic volume (ESV), and ejection fraction (EF) were examined. RESULTS: 122 CMR were performed in 114 patients. Average age at CMR was 15.4±7.4 years. 53.3% of them presented severe PR (> 40%). RVEDV was 157.3 ± 38.6 ml/m2, RVESV was 85.3 ± 27 ml/m2 and RVEF was 46.4 ± 7.1%. RVEDV was > 150 ml/ m2 in 48.4% and > 170 ml/m2 in 32.8% of patients. Patients with TAP showed larger RV volumes compared with those with infundibular widening. RVEDV > 170 ml/m2 showed worse RVEF that those with lower RVEDV (47.9 ± 7% vs 43.2 ± 6.4%, p < 0.01). CONCLUSION: Almost half of the pa tients showed significant RV enlargement, demonstrating that the indication of CMR is late in their follow-up. TAP was associated with higher RVEDV and RVESV, but no worse RVEF.

Resultados alejados de la cirugía reparadora de la insuficiencia mitral isquémica / Long-term results of mitral valve repair for ischemic mitral insufficiency

Background: The preferred treatment for ischemic mitral insufficiency is mitral valve repair with a prosthetic ring, because it does not deteriorate left ventricular function, allowing better immediate and long-term results. Aim: To assess long-term results of mitral annuloplasty with a prosthetic ring for ischemic mitral insufficiency. Patients and Methods: One hundred patients (68 men), with a mean age of 65.7 ± 8.6 years were included. They underwent a mitral annuloplasty with a prosthetic ring to treat ischemic mitral insufficiency, between February 1992 and May 2009. Fortyfour had a history of prior myocardial infarction and 46 had an evolving acute coronary syndrome. The inferior left ventricular wall was involved, exclusively or associated with an adjacent wall, in 72 cases. Coronary artery bypass grafts were performed in 92 patients and 32 required intra-aortic balloon pumping at some time during the peri-operative period. Results: Operative mortality was 10% (10 patients). During follow-up 30 patients died, at an average of 39 months after surgery (range: 3-142 months). Actuarial long-term survival rates at 1, 3 and 5 years were 79%, 72% and 64.5%, respectively. Trans esophageal echocardiogram performed in the operating room showed none or minimal residual mitral insufficiency in 96% of the cases. Echocardiographic follow-up was completed in 80% of the survivors; 79% of them had no or minimal mitral insufficiency. Only one patient was re-operated on due to severe mitral insufficiency and 4 required a permanent pacemaker. Conclusions: Considering the critical illness of these patients, good long-term results were observed after treatment of ischemic mitral regurgitation performing a mitral annuloplasty with a prosthetic ring.

Síndrome de Twiddler: una causa infrecuente de disfunción de marcapaso definitivo. Caso clínico / Twiddler syndrome: Report of one case

We report a 26year old patient who had a single chamber pacemaker implantation one year before. During a routine pre-operative evaluation, pacemaker dysfunction was demonstrated due to sensing and pacing failure, associated to left pectoral muscle rhythmic contraction. Chest X-ray confirmed Twiddler syndrome, in which twisting or rotation of the device inside the pocket results in lead dislodgement and device malfunction.

Reemplazo valvular aórtico con prótesis biológica en pacientes mayores de 70 años con estenosis aórtica / Aortic valve replacement with bioprosthesis in patients 70 years old or older with aortic stenosis

Background: Aortic valve stenosis is the most prevalent valvulopathy in the elderly, but it is frequently undertreated. Aim: To assess early and long term results of valve replacement with bioprostheses in patients 70 years old or older with aortic stenosis. Patients and Methods: A review of the database of the Cardiovascular Surgery Service of our hospital, for the period 1990-2007, identified 156 patients with aortic valve stenosis, aged 76.7 ± 4.5 years (67 percent males), that were subjected to an aortic valve replacement with bioprosthesis. Seventy eight (50 percent) were in functional class III-IV. Six patients had emergency surgery. In 67 patients (43 percent) coronary artery bypass graft were performed. Long term mortality was ascertained obtaining death records from Chilean death registry. Results: Operative mortality was 3.2 percent (5 patients). Four patients (2.6 percent) required a reoperation due to bleeding. The mean follow up was 66 ± 41 months. The survival rate at 1, 3 and 5years was 94 percent, 88 percent and 80 percent, respectively. Forty two patients (27 percent) were 80years old or older. In this sub-group, 1 patient (2.3 percent) died during the peri operative period and the estimated survival rate at 1, 3 and 5years was 97 percent, 92 percent, and 80 percent, respectively. Multivariate risk analysis, for the whole group, identified low ejection fraction and concomitant coronary artery bypass graft surgery as predictors of late mortality. Conclusions: Aortic valve replacement for aortic stenosis with bioprostheses in patients 70 years old or older had low perioperative mortality and morbidity and a good long term survival.

Disección aórtica tipo A: Resultados operatorios y seguimiento a mediano plazo / Surgical results among 100 patients with type A aortic dissection: Retrospective review

Background: Tipe A aortic dissection involves the ascending aorta and has high mortality rates without surgical treatment. Aim: To report the results of surgical treatment of type A aortic dissection. Material and Methods: Retrospective review of medical records of 100 patients aged 17 to 78 years (73 percent males) operated between January 2000 and August 2008, for type A aortic dissection. Follow up was performed with telephone interviews and review of national death records. Results: Eighty three percent of patients had an acute dissection. Operative mortality was 27 and 20 percent for patients with acute and chronic dissection, respectively. Mortality was 50 percent among patients aged 70 years or more, compared with 21 percent among their younger counterparts, The most common complication was bleeding that required a new surgical procedure in 18 percent of patients. Actuarial survival was 70 percent at five years. Cardiovascular problems caused the death of two of the nine patients that died during follow up. Conclusions: Surgical mortality among patients with type A aortic dissection was higher among patients with acute episodes and those aged 70 years or more.

Estudio comparativo de vaciamiento y tiempo de tránsito de estómago y colon intratorácico post esofagectomía total con técnica radioisotópica / Comparative study of clearence and time of stomach transit and intrathoraxic total post esophagectomy with radioisotopic technique

Después de efectuarse esofagectomía total, ya sea por cáncer o por patología benigna, las alternativas de reconstrucción del tránsito digestivo son el ascenso de un tubo gástrico o la intreposición de un segmento de colon, fectuándose una anastomosis a nivel cervical. El objetivo del presente estudio fue evaluar la motilidad y el vaciamiento del segmento ascendido o interpuesto, mediante técnica radio-isotópica, 3 meses después de la cirugía. Se estudiaron 13 pacientes a quienes se le efectuó ascenso gástrico y 13 pacientes con interposición de colon. Después de la ingesta de un bolo radiactivo de Tc99 súlfuro coloide, se determina el porcentaje de vaciamiento y las características motoras del segmento ascendido a los 15 y 50 seg, 5 y 10 min. y tiempo de tránsito total, tanto en decúbito supino como en posición de pie. Tanto en estómago ascendido como en colon interpuesto, se observan alteraciones motoras de tipo adinámico, con marcado retardo en el tiempo de tránsito y vaciamiento, observándose una mejoría parcial en posición de pie

Galactosemia / Galactosemia

Se describe el caso de un lactante galactosémico, que manifiesta su enfermedad en el primer mes de vida, caracterizándose por mal progreso ponderal, acidosis, ictericia, hepatoesplenomegalia, daño neurológico y ausencia de cataratas. El diagnóstico se confirmó mediante cromatografía de azúcares en orina, la que demostró galactosuria previa administración de galactosa en la dieta. Su tratamiento consistió en supresión de la ingesta de galactosa; con él se consiguió mejoría parcial de la función hepática, pero no del daño neurológico