Gonzalo Urcelay

Cardiovascular magnetic resonance findings in a pediatric population with isolated left ventricular non-compaction

BackgroundIsolated Left Ventricular Non-compaction (LVNC) is an uncommon disorder characterized by the presence of increased trabeculations and deep intertrabecular recesses. In adults, it has been found that Ejection Fraction (EF) decreases significantly as non-compaction severity increases. In children however, there are a few data describing the relation between anatomical characteristics of LVNC and ventricular function. We aimed to find correlations between morphological features and ventricular performance in children and young adolescents with LVNC using Cardiovascular Magnetic Resonance (CMR).Methods15 children with LVNC (10 males, mean age 9.7 y.o., range 0.6 - 17 y.o.), underwent a CMR scan. Different morphological measures such as the Compacted Myocardial Mass (CMM), Non-Compaction (NC) to the Compaction (C) distance ratio, Compacted Myocardial Area (CMA) and Non-Compacted Myocardial Area (NCMA), distribution of NC, and the assessment of ventricular wall motion abnormalities were performed to investigate correlations with ventricular performance. EF was considered normal over 53%.ResultsThe distribution of non-compaction in children was similar to published adult data with a predilection for apical, mid-inferior and mid-lateral segments. Five patients had systolic dysfunction with decreased EF. The number of affected segments was the strongest predictor of systolic dysfunction, all five patients had greater than 9 affected segments. Basal segments were less commonly affected but they were affected only in these five severe cases.ConclusionThe segmental pattern of involvement of non-compaction in children is similar to that seen in adults. Systolic dysfunction in children is closely related to the number of affected segments.

New Surgical Approach to Device Closure of Multiple Apical Ventricular Septal Defects

We present an alternative technique for closing multiple ventricular septal defects difficult to access during surgery. A guidewire is advanced through the right ventricular free wall and through the main apical defect to the left ventricle, and this approach is used to place an Amplatzer device to occlude the ventricular septal defects. The procedure is performed in the beating heart, under intraoperative transesophageal echocardiographic guidance, and without extracorporeal circulation. It appears to be a simple and reproducible procedure with excellent short-term results.

Cierre perventricular de la comunicación interventricular múltiple con dispositivo Amplatzer

Se presenta una tecnica alternativa para el cierre de la comunicacion interventricular de dificil acceso quirurgico. Con el corazon latiendo y sin necesidad de circulacion extracorporea, se punciona el ventriculo derecho y se avanza una guia de alambre hasta el ventriculo izquierdo a traves del defecto apical principal; esto permite colocar un dispositivo Amplatzer que ocluye completamente los defectos. El procedimiento se realiza con vision ecocardiografica transesofagica, es simple y con excelentes resultados a corto plazo.

Mortalidad operatoria y estratificación de riesgo en pacientes pediátricos operados de cardiopatía congénita: experiencia de 10 años

Estudio de cohorte no concurrente (ene-ro 2000-julio 2010). Las cirugias fueron estratificadas segun puntuacion de riesgo. La tendencia de morta-lidad operatoria se evaluo dividiendo el periodo es-tudiado en tres subperiodos. Comparamos nuestros resultados con los publicados por las Sociedades de Cirujanos de Cardiopatias Congenitas y la de Ciruja-nos de Torax.

Resultados de un programa nacional de trasplante cardíaco pediátrico: fortalezas y debilidades

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.Introduction: 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. Material 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 urgently and 34.5% died on the waiting list.

Síndrome de hipoplasia de corazón izquierdo: experiencia de 10 años de un programa de etapificación quirúrgica

UNLABELLED Hypoplastic left heart syndrome (HLHS) is a lethal congenital heart disease in 95% of non-treated patients. Surgical staging is the main form of treatment, consisting of a 3-stage approach, beginning with the Norwood operation. Long term survival of treated patients is unknown in our country. OBJECTIVES 1) To review our experience in the management of all patients seen with HLHS between January 2000 and June 2012. 2) Identify risk factors for mortality. PATIENTS AND METHOD Retrospective analysis of a single institution experience with a cohort of patients with HLHS. Clinical, surgical, and follow-up records were reviewed. RESULTS Of the 76 patients with HLHS, 9 had a restrictive atrial septal defect (ASD), and 8 had an ascending aorta ≤2mm. Of the 65 out of 76 patients that were treated, 77% had a Norwood operation with pulmonary blood flow supplied by a right ventricle to pulmonary artery conduit, 17% had a Norwood with a Blalock-Taussig shunt, and 6% other surgical procedure. Surgical mortality at the first stage was 23%, and for Norwood operation 21.3%. For the period between 2000-2005, surgical mortality at the first stage was 36%, and between 2005-2010, 15% (P=.05). Actuarial survival was 64% at one year, and 57% at 5years. Using a multivariate analysis, a restrictive ASD and a diminutive aorta were high risk factors for mortality. CONCLUSIONS Our immediate and long term outcome for staged surgical management of HLHS is similar to that reported by large centres. There is an improvement in surgical mortality in the second half of our experience. Risk factors for mortality are also identified.

Resultados inmediatos y alejados del switch arterial en pacientes con dextrotransposición de grandes arterias: experiencia de 20 años.

Estudio retrospectivo de pacientes sometidos a switch arterial entre mayo de 1992 y noviembre de 2012. Se comparo periodo 1 (1992 a 2002) con periodo 2 (2003 a 2012). Se de-finio D-TGA simple aquella sin lesiones asociadas y D-TGA compleja aquella con asociacion de comunica-cion interventricular o coartacion aortica.

Randomized Trial of Early Use of Inhaled Nitric Oxide in Newborns with Acute Lung Disease and Pulmonary Hypertension

Randomized Trial of Early Use of Inhaled Nitric Oxide in Newborns with Acute Lung Disease and Pulmonary Hypertension