Coronary Compression Following Percutaneous Pulmonary Valve Implantation Despite a Negative Balloon Sizing Test: Lessons Learnt From This Unusual Complication.

A 16-years-old male with prior diagnosis of situs inversus totalis and pulmonary atresia with interventricular communication underwent percutaneous pulmonary valve implantation 3 months after successful RVOT stenting following a negative balloon sizing test. Once finished the procedure, after consciousness recovery in the intensive care unit, the patient developed oppressive chest pain with very subtle electrocardiographic changes over his basal right bundle branch block. An urgent coronary angiography showed a severe stenosis in the proximal right coronary artery with TIMI 2 distal flow. Intravascular ultrasound imaging confirmed extrinsic compression. A 4 × 21 mm drug-eluting stent was successfully implanted relieving symptoms immediately. TIMI 3 flow was restored and good apposition and expansion were confirmed with intravascular ultrasound. A carefully review of the procedure showed that the pitfall responsible for this complication was the oversizing of the valve with respect to the size of the balloon used for the sizing test.

Spanish registry of percutaneous VSD closure with NitOcclud Lê VSD Coil device: lessons learned after more than a hundred implants.

INTRODUCTION AND OBJECTIVES: The NitOcclud Lê VSD Coil was specifically designed for transcatheter occlusion of ventricular septal defects (VSD) and became available for this purpose in August 2010. Our objective was to describe the Spanish experience of this technique and analyze its reliability and short- to mid-term efficacy. METHODS: National multicenter observational study, which retrospectively recruited all patients (of any age) with VSD (of any location or type) who underwent percutaneous NitOcclud occlusion, using an intention-to-treat analysis, until January 2019. RESULTS: A total of 117 attempts were made to implant at least 1 NitOcclud in 116 patients in 13 institutions. The median [range] age and weight was 8.6 [0.4-69] years and 27 [5.8-97] kg, respectively. In 99 patients (85%), the VSD was an isolated congenital defect. The location was perimembranous in 95 (81%), and 74 (63%) of them were aneurysmatic. The mean fluoroscopy time was 34 [11.4-124] minutes. Of the 117 attempts, 104 were successful (89%) with a follow-up of 31.4 [0.6-59] months. At the last review, final complete occlusion of the defect without residual shunt or with only a minimal shunt was achieved in 92.3% (no shunt, n=73; trivial shunt, n=23). Four patients required a second procedure for residual shunt occlusion. Two devices had to be surgically explanted due to severe hemolysis. There were no deaths or other major complications. CONCLUSIONS: The NitOcclud device can be used successfully for a wide anatomical spectrum of VSD. The main issue is residual shunt, but its incidence decreases over time. The incidence of hemolysis was very low and no permanent changes were detected in atrioventricular conduction.

Hipertensión arterial pulmonar y cirugía de switcharterial neonatal para la corrección de la transposición de grandes arterias / Pulmonary arterial hypertension and neonatal arterial switch surgery for correction of transposition of the great arteries

Introducción y objetivos: Son escasas las publicaciones sobre aparición de hipertensión arterial pulmonar tras la cirugía de switch arterial en periodo neonatal para la corrección de la transposición de grandes arterias. Se evalúa la frecuencia y el comportamiento clínico de esta complicación en una serie de pacientes. Métodos: Se revisó la base de datos y se seleccionó a pacientes con transposición de grandes vasos corregida con switch arterial neonatal en el centro en los que con el tiempo apareció hipertensión pulmonar. Resultados: Se halló a 2 pacientes (1,3%) con transposición de grandes arterias corregida con éxito en la primera semana de vida que luego presentaron hipertensión arterial pulmonar. El primero es una niña de 7 años con diagnóstico de hipertensión pulmonar grave a los 8 meses de edad, sin respuesta a tratamiento médico, que precisó trasplante pulmonar. La anatomía patológica mostró hallazgos compatibles con hipertensión arterial pulmonar grave. El segundo es un niño de 24 meses con diagnóstico de hipertensión pulmonar grave a los 13 meses, sin respuesta al tratamiento médico. Conclusiones: La hipertensión arterial pulmonar es una complicación infrecuente pero muy grave cuya aparición se debe investigar en todo paciente con transposición de grandes vasos sometido a operación de switch arterial neonatal con el fin de instaurar un tratamiento agresivo temprano para los pacientes afectados, dados la escasa respuesta al tratamiento y el mal pronóstico que supone (AU)

Introduction and objectives: There are few reports of the appearance of pulmonary arterial hypertension following arterial switch surgery in the neonatal period to correct transposition of the great arteries. We assessed the frequency and clinical pattern of this complication in our series of patients. Methods: Our database was reviewed to select patients with transposition of the great arteries corrected by neonatal arterial switch at our hospital and who developed pulmonary hypertension over time. Results: We identified 2 (1.3%) patients with transposition of the great arteries successfully repaired in the first week of life who later experienced pulmonary arterial hypertension. The first patient was a 7-year-old girl diagnosed with severe pulmonary hypertension at age 8 months who did not respond to medical treatment and required lung transplantation. The anatomic pathology findings were consistent with severe pulmonary arterial hypertension. The second patient was a 24-month-old boy diagnosed with severe pulmonary hypertension at age 13 months who did not respond to medical therapy. Conclusions: Pulmonary hypertension is a rare but very severe complication that should be investigated in all patients with transposition of the great arteries who have undergone neonatal arterial switch, in order to start early aggressive therapy for affected patients, given the poor therapeutic response and poor prognosis involved (AU)

Pulmonary Arterial Hypertension and Neonatal Arterial Switch Surgery for Correction of Transposition of the Great Arteries.

INTRODUCTION AND OBJECTIVES: There are few reports of the appearance of pulmonary arterial hypertension following arterial switch surgery in the neonatal period to correct transposition of the great arteries. We assessed the frequency and clinical pattern of this complication in our series of patients. METHODS: Our database was reviewed to select patients with transposition of the great arteries corrected by neonatal arterial switch at our hospital and who developed pulmonary hypertension over time. RESULTS: We identified 2 (1.3%) patients with transposition of the great arteries successfully repaired in the first week of life who later experienced pulmonary arterial hypertension. The first patient was a 7-year-old girl diagnosed with severe pulmonary hypertension at age 8 months who did not respond to medical treatment and required lung transplantation. The anatomic pathology findings were consistent with severe pulmonary arterial hypertension. The second patient was a 24-month-old boy diagnosed with severe pulmonary hypertension at age 13 months who did not respond to medical therapy. CONCLUSIONS: Pulmonary hypertension is a rare but very severe complication that should be investigated in all patients with transposition of the great arteries who have undergone neonatal arterial switch, in order to start early aggressive therapy for affected patients, given the poor therapeutic response and poor prognosis involved.

Versión española del cuestionario de calidad de vida para niños y adolescentes con cardiopatías (PedsQL TM) / The spanish version of the health-related quality of life questionnaire for children and adolescents with heart disease (PedsQL TM)

Introducción y objetivos. Adaptar al español el cuestionario de calidad de vida Pediatric Quality of Life Inventory (PedsQLTM) (Módulo General y Módulo Cardiaco, versión 3.0). Métodos. Metodología de traducción directa e inversa. La entrevista cognitiva se utilizó para probar la versión previa al test. Se realizó la evaluación de las propiedades psicométricas de la versión española. Resultados. La nueva versión contiene algunos cambios de formato con intención de facilitar la lectura de los enunciados y la comprensión de las instrucciones de respuesta (versión para niños de 5-7 años). Se identificaron algunos problemas de «descontextualización» en relación con el desarrollo evolutivo de los niños en las versiones para los niños más pequeños y sus padres (2-4 y 5-7 años). El porcentaje de ítems sin respuesta osciló entre 0 y el 5,9%. Se encontraron efectos techo elevados, especialmente en el Módulo Cardiaco, donde osciló en un 19-48,6%. La consistencia interna fue > 0,7, excepto en la dimensión de Apariencia Física y Funcionamiento Escolar en los niños. Los grados de acuerdo entre la información dada por los niños y la dada por los padres fueron en general muy altos. Se observó una tendencia de descenso de las puntuaciones en todas las escalas en relación con la gravedad de la cardiopatía, pero sólo en algunas dimensiones las diferencias fueron estadísticamente significativas. Conclusiones. La versión española del PedsQLTM presenta algunas diferencias con la versión original, concretamente con las dimensiones de Funcionamiento Escolar y Apariencia Física, como se observó en las entrevistas cognitivas. Por otro lado, no se ha demostrado que tenga validez predictiva (AU)

Introduction and objectives. To adapt the Pediatric Quality of Life Inventory (PedsQLTM) (General Module and Cardiac Module, 3.0 version) into Spanish. Methods. Forward and back translation methodology. Cognitive interviewing was used to check the pre-test version. Psychometric properties were computed for the PedsQLTM Cardiac Module Scales. Cross-informant variance between children and parents was assessed. Results. The Spanish version has some format changes to make it easier to read and to clarify response choices (version for 5-7 years age group). Some semantically complex terms were replaced with synonyms and others illustrated with examples. Some "out of context" problems were identified with respect to some items (version for children 2-4 and 5-7 years). The percentage of missing item responses ranged from 0% to 5.9%. A high ceiling effect was found, especially in the Cardiac Module, which ranged from 19% to 48.6%. Internal consistency was higher than 0.7, except for Physical Appearance Scale and School Functioning in children. Agreement between information given by children and parents was generally very high. A decreasing trend in scores on all scales was observed in relation to the severity of heart disease, but the differences were only statistically significant in some dimensions. Conclusions. The Spanish version of the PedsQLTM differs somewhat from the original version, particularly on the School Functioning and Physical Appearance Scales, as observed in cognitive interviews. Predictive validity was not demonstrated (AU)

The Spanish version of the health-related quality of life questionnaire for children and adolescents with heart disease (PedsQL(TM)).

INTRODUCTION AND OBJECTIVES: To adapt the Pediatric Quality of Life Inventory (PedsQL(TM)) (General Module and Cardiac Module, 3.0 version) into Spanish. METHODS: Forward and back translation methodology. Cognitive interviewing was used to check the pre-test version. Psychometric properties were computed for the PedsQL(TM) Cardiac Module Scales. Cross-informant variance between children and parents was assessed. RESULTS: The Spanish version has some format changes to make it easier to read and to clarify response choices (version for 5-7 years age group). Some semantically complex terms were replaced with synonyms and others illustrated with examples. Some "out of context" problems were identified with respect to some items (version for children 2-4 and 5-7 years). The percentage of missing item responses ranged from 0% to 5.9%. A high ceiling effect was found, especially in the Cardiac Module, which ranged from 19% to 48.6%. Internal consistency was higher than 0.7, except for Physical Appearance Scale and School Functioning in children. Agreement between information given by children and parents was generally very high. A decreasing trend in scores on all scales was observed in relation to the severity of heart disease, but the differences were only statistically significant in some dimensions. CONCLUSIONS: The Spanish version of the PedsQL(TM) differs somewhat from the original version, particularly on the School Functioning and Physical Appearance Scales, as observed in cognitive interviews. Predictive validity was not demonstrated.