ABSTRACT We describe a 60-year-old woman with post-myocardial infarction (MI) ventricular septal defect (VSD) and cardiogenic shock who was successfully stabilized with veno-arterial extracorporeal membrane oxygenation (VA-ECMO) as a bridge therapy for the surgical closure of her VSD. This case highlights the role of VA-ECMO in the management of post-MI VSD to improve the results of surgical repair and patient survival.

Background: Thoracoabdominal aortic aneurysm (TAAA) is an infrequent disease and demands a highly specialized and experienced management. Open repair (OR) is the gold standard but it is associated with significant morbidity and mortality. Paraplegia and renal failure are the most important complications. Aim: To report our results with OR treatment of TAAA. Material and Methods: Descriptive study including all patients with TAAA operated electively and consecutively by OR between 1983 and 2019. Main outcomes are operative mortality, renal and neurological morbidity, and long-term survival. Results: We report 45 operated patients aged 33 to 84 years, 74% males. Aneurysm extension according to Crawford classification was I in 18%, II in 18 %, III in 36% and IV in 29%. Operative mortality was 4%. The frequency of paraplegia or paraparesis at discharge was 9%. No patient was discharged on hemodialysis. Survival at 5 and 10 years were 60% and 40% respectively. Conclusions: OR of TAAA is a complex procedure. Our results show perioperative mortality rates comparable to highly experienced centers. Although being a major procedure, OR remains an alternative to treat this serious condition.

Pediatric heart transplantation is an effective therapy to treat advanced heart failure in children. Objectives: To analyze the immediate and mid-term results of pediatric patients listed for heart transplantation. Patients and Methods: Registration of patients admitted to our transplant protocol between October 2001 and July 2016 were reviewed, analyzing demographic data, diagnosis, status at the time of listing, waiting time until transplantation, donor data, use of ventricular assist device, hemodynamic data, complications and global mortality. Results: Thirthy patients where included with a mean age of 9.4 years (1 month to 15 years). The most frequent diagnosis was dilated cardiomyopathy in 24 patients (80%). The status was I (urgency) in 19 cases and II in 11 cases. Ten patients died on the waiting list (33.3%) at an average of 52 days (13-139 days). Fourteen were transplanted (46.7%), with a waiting time of 199.6 days (4-586 days). Nine patients required mechanical support (30%). All patients received triple association of immunosuppression. One patient died 16 days post transplant due to primary graft failure (7.1%). The average follow-up was 43 months (0.5-159 months). Two patients died later on (82 and 55 months), both due to secondary rejection because of voluntary cessation of immunosuppressive therapy. Survival at 1 and 5 years was 93% and 74%, respectively. Conclusions: Our program has successfully transplanted 50% of patients enrolled, with good medium-term survival. A significant proportion of patients were listed as a medical emergency and 34.5% died on the waiting list.

El trasplante cardiaco pediátrico es una terapia efectiva para tratar la insuficiencia cardiaca avanzada. Objetivos: Analizar los resultados inmediatos y a mediano plazo de niños enlistados para trasplante cardiaco. Pacientes y Método: Se analizó el registro de pacientes enlistados para trasplante, entre octubre de 2001 y julio de 2016, analizando datos demográficos, diagnósticos, status de enlistamiento, tiempo de espera, datos de donantes, uso de asistencia ventricular, complicaciones y mortalidad. Resultados: La serie abarca 30 pacientes con edad promedio de 9,4 años (1 mes a 15 años). El diagnóstico principal fue miocardiopatía dilatada en 24 pacientes (80%). El status de ingreso fue I (urgencia) en 19 casos y II (no urgencia) en 11. Fallecieron 10 en la lista de espera (33,3%) en un promedio de 52 días (13 a 139 días). Catorce pacientes fueron trasplantados (46.7%), con un tiempo de espera de 199,6 días (4 a 586 días). Requirieron asistencia ventricular 9 pacientes (30%). Todos recibieron inmunosupresión tri asociada. Un paciente falleció a los 16 días por falla primaria del injerto (7,1%). El seguimiento promedio fue de 43 meses (0,5 a 159 meses). Dos pacientes fallecieron alejadamente (55 y 82 meses) por rechazo secundario al abandono de tratamiento inmunosupresor. La supervivencia a 1 y 5 años fue 93% y 74%, respectivamente. Conclusiones: Nuestro programa ha trasplantado al 50% de los pacientes enlistados con buena supervivencia a mediano plazo. Una proporción significativa de pacientes se enlistó con carácter de urgencia y un 34.5% de los pacientes fallecieron en la lista de espera.

Background: Aortic insufficiency (AI) is rarely amenable to surgical repair. One of the exceptions to that statement is the bicuspid aortic valve Aim: to analyze our results in the repair of a regur-gitant bicuspid aortic valve Method: A review of the cardiac surgery data base in the period January 1994 to July 2014 allowed the identification of 29 patients with AI and a bicuspid aortic valve submitted to surgical repair. The data from the clinical record and the surgical report was analyzed. Survival was established from the National Identification Service. Results: all patients were males. Mean age was 39.4 years (range 19-61). Five patients had infective endocarditis. Preoperative echocardiography revealed severe AI in 25 patients (86%) and moderate AI in 4. Left ventricular diastolic and systolic diameters were 67,8 ± 6,7 and 44 ± 7,1 mm, respectively. All patients presented fusion of the left and right leaflets with mid rafhe, and 3 patients had an incomplete rafhe with a cleft. AI was caused by prolapsed fu-sioned leaflet in 23 patients, leaflet perforation in 3, cleft in one and perforation plus cleft in 2 patients. Triangular resection was performed in 23 (79%) and complemented by annuloplasty in 16 (55%) patients. Three patients underwent closure of a perforation and 3 a repair of the cleft. An additional surgical procedure was performed in 10 patients. All patients underwent intra-operative TEE. 35% had no residual AI and 65% had minimal or mild AI. There was no surgical mortality. Follow up was completed in all patients. Two patients (7%) died from non cardiac causes. Seven patients (24%) had to be re-operated on, a mean of 7.1 years after the initial surgery. Mean survival rate was 19.3 (95% CI 17.6-21), and mean survival free from re-operation was 15.8 years (95%CI 13-18.7) at 20.6 years of follow up. Echo-cardiogram performed at a mean of 4.9 years after surgery showed a reduction of LV systolic diameter of 6,15 ± 7,2 mm (p<0,05), LV diastolic diameter of 11,26 ± 8,7 mm (p<0,05) and fractional shortening of 1,12 ± 5,57% (p<0,33). In the group of 22 patients who did not have a re-operation, AI was absent in 9, mild in 6 and moderate in 3; 4 patients had mild aortic stenosis. Conclusion: Surgical repair of the regurgitant bicuspid aortic valve has low peri-operative mortality rate and excellent late survival. Reoperation, required in 24% of patients, occurred late after the initial operation in most cases.

Introducción: La Insuficiencia Aórtica (IA) excepcionalmente es susceptible de reparación. Una de estas excepciones es la Válvula Aórtica Bicúspide (VAB). Objetivo: Analizar nuestros resultados de la reparación de la VAB insuficiente. Método: Se revisó la Base de Datos para el período enero 1994 a Julio 2014. Se identificaron 29 pacientes y se revisaron las fichas clínicas y protocolos operatorios. La supervivencia se certificó en el Registro Civil e Identificación de Chile. Resultados: Todos los pacientes fueron hombres. La edad promedio fue 39,4 años (19- 61 años). Cinco pacientes presentaban una endocarditis. El ecocardiograma preoperatorio demostró IA severa en 25 casos (86%) y moderada en 4. El diámetro sis-tólico fue 44 ± 7,1 mm y el diastólico 67,8 ± 6,7 mm. La fracción de acortamiento fue 35,96 ± 5,54%. En todos los casos la VAB presentaba fusión del velo coronariano izquierdo y derecho con rafe medio; en 3, el rafe era incompleto produciéndose un cleft. En 23 casos (79%) la IA era secundaria a prolapso del velo fusionado, en 3 a perforación de velo, en 1 a un cleft y en 2 a perforación y cleft. En 23 casos (79%) se efectuó una resección triangular y en 16 (55%) se complementó con una anuloplastía. En 3 se cerró una perforación y en otros 3 se efectuó un cierre primario de cleft. En 10 casos se realizó un procedimiento asociado. En todos los casos se realizó un ecocardiograma transesofágico intra-operato-rio. En 35% no hubo insuficiencia aórtica residual y en 65% esta fue mínima o leve. No hubo mortalidad operatoria. El seguimiento se completó en el 100%. Dos pacientes (7%) fallecieron por causas no cardiacas. Siete (24%) fueron re-operados, en promedio a los 7,14 años. La media de supervivencia fue 19,3 años (IC95% 17,6-21) y la supervivencia libre de re-operación 15,8 años (IC95% 13-18,7), a 20,6 años de seguimiento. El ecocardiograma efectuado en promedio a los 4,9 años demostró una reducción del diámetro sistólico de 6,15 ± 7,2 mm (p<0,05), del diastólico de 11,26 ± 8,7 mm (p<0,05) y de la fracción de acortamiento de 1,12 ± 5,57% (p<0,33). De los 22 pacientes no reoperados, 9 no tenían IA, en 6 esta era leve (1+) y en 3 leve a moderada (2+); 4 pacientes tenían una estenosis aórtica leve. Conclusión: La reparación quirúrgica de la válvula aortica bicúspide insuficiente tiene baja mortalidad peri-operatoria y excelente supervivencia alejada. Si bien el 24% de los pacientes requirió una re-operación, esta fue tardía en la mayoría de los casos.

Aspergillosis usually compromises the respiratory system, but can also affect others. We report a 46 yo female with acute myeloid leukemia, developed febrile neutropenia and dysphagia. Endoscopy revealed esophageal cytomegalovirus-like ulcers, but biopsies showed Aspergillus spp. It's important to consider aspergillosis in the differential diagnosis of esophageal lesions in high-risk patients.

Crigler-Najjar Syndrome is an uncommon genetic disorder characterized by the elevation of unconjugated plasmatic bilirubin secondary to deficiency of the enzyme uridine diphosphate glucuronyltransferase (UDP-GT). We report a 19-years-old woman with the syndrome diagnosed during the neonatal period, when she developed a severe jaundice in the first 10 days of life, reaching unconjugated bilirubin levels of 29 mg/dl, with normal liver function tests. After transient response to phototherapy, the patient was referred to a tertiary medical center in which an extensive work up ruled out other etiologies and the diagnosis of type I Crigler-Najjar syndrome was established. Currently, the patient has a mild mental retardation. She is receiving homemade phototherapy 18 h per day with acceptable control of bilirubin levels. Many mutations have been associated with UDP-GT dysfunction resulting in a broad spectrum of the disease. When bilirubin rises above physiological limits, it permeates the hematoencephalic barrier, inducing bilirubin impregnation of basal ganglia with secondary neuronal damage and necrosis. The worst outcome, kernicterus, is characterized by mental retardation, central deafness, ophthalmoplegia, ataxia, athetosis, spasticity, seizures and death. First line therapy includes phototherapy, but definitive therapy is liver transplantation before the occurrence of neurological damage.