Results of heart retransplantation: subanalysis of the Spanish Heart Transplant Registry.

INTRODUCTION AND OBJECTIVES: Heart retransplantation (ReHT) is controversial in the current era. The aim of this study was to describe and analyze the results of ReHT in Spain. METHODS: We performed a retrospective cohort analysis from the Spanish Heart Transplant Registry from 1984 to 2018. Data were collected on donors, recipients, surgical procedure characteristics, immunosuppression, and survival. The main outcome was posttransplant all-cause mortality or need for ReHT. We studied differences in survival according to indication for ReHT, the time interval between transplants and era of ReHT. RESULTS: A total of 7592 heart transplants (HT) and 173 (2.3%) ReHT were studied (median age, 52.0 and 55.0 years, respectively). Cardiac allograft vasculopathy was the most frequent indication for ReHT (42.2%) and 59 patients (80.8%) received ReHT >5 years after the initial transplant. Acute rejection and primary graft failure decreased as indications over the study period. Renal dysfunction, hypertension, need for mechanical ventilation or intra-aortic balloon pump and longer cold ischemia time were more frequent in ReHT. Median follow-up for ReHT was 5.8 years. ReHT had worse survival than HT (weighted HR, 1.43; 95%CI, 1.17-1.44; P<.001). The indication of acute rejection (HR, 2.49; 95%CI, 1.45-4.27; P<.001) was related to the worst outcome. ReHT beyond 5 years after initial HT portended similar results as primary HT (weighted HR, 1.14; 95%CI, 0.86-1.50; P<.001). CONCLUSIONS: ReHT was associated with higher mortality than HT, especially when indicated for acute rejection. ReHT beyond 5 years had a similar prognosis to primary HT.

Evolución neurológica y resultados de la cirugía pediátrica del arco aórtico mediante perfusión cerebral selectiva / Neurological changes and outcomes of paediatric surgery of the aortic arch using selective cerebral perfusion

INTRODUCCIÓN: Los objetivos son analizar las alteraciones neuropsicológicas a medio plazo de los niños intervenidos del arco aórtico mediante perfusión cerebral selectiva (PCS) y detectar posibles factores modificables en la técnica quirúrgica que pueden ayudar a minimizar la afectación neurológica posterior. MATERIAL Y MÉTODOS: Se establecieron como criterios de inclusión: patología del arco aórtico intervenida mediante PCS durante el primer año de vida, entre el 10 de agosto del 2004 y el 24 de mayo del 2016, fisiología biventricular y edad gestacional mayor de 31 semanas. En ausencia de cromosomopatía, se clasificaron desde el punto de vista neurológico siguiendo el modified Rankin score. En mayores de 4 años se realizaron estudios de inteligencia, nivel de atención, maduración y aptitudes psicolingüísticas. RESULTADOS: Se incluyeron ochenta y dos pacientes, cuya edad media en el momento de la cirugía fue de 1,8 meses. El flujo medio en PCS fue de 32 ml/kg/min. El tiempo medio de PCS fue de 31 min. La mortalidad global de la serie fue del 14,8%. Con disfunción neurológica se observaron un 35,9% y se detectaron como factores de riesgo: cirugía en menores de 10 días de edad, duración de PCS mayor de 40 min y la duración del enfriamiento o calentamiento. Un 35,2% de los pacientes mayor de 5 años fueron diagnosticados de déficit de atención. CONCLUSIONES: Los pacientes intervenidos en el primer año de vida con PCS precisan un seguimiento neuropsicológico y hay factores modificables quirúrgicos que pueden influir en el desarrollo neurológico

INTRODUCTION: The aims of this article are to analyse the neuropsychological changes in the medium-term in children subjected to aortic arch surgery using selective cerebral perfusion (SCP), as well as to detect any modifiable factors in the surgical technique that may contribute to minimising the subsequent neurological involvement. MATERIAL AND METHODS: Inclusion criteria were established as: aortic arch disease operated on using SCP during the first year of life, between 10 August 2004 and 24 May 2016, biventricular physiology, and gestational age greater than 31 weeks. In the absence of a chromosomal disease, they were classified, from a neurological point of view, using the Rankin score. Children over 4-years of age were subjected to intelligence studies, including attention level, development, and psycho-lingual skills. RESULTS: The study included a total of 82 patients with a mean age of 1.8 months. The mean SCP flow was 32 ml/kg/min. The mean time of SCP was 31 minutes. The overall mortality of the series was 14.8%. Neurological dysfunction was observed in 35.9% of patients, and the following were detected as risk factors: surgery in patients less than 10-days-old, duration of SCP greater than 40minutes, and the time required for the cooling down and/or warming-up. Attention deficit was diagnosed in 35.2% of patients greater than 5-years-old. CONCLUSIONS: Patients operated on using SCP in in the first year of life required a neuropsychological follow-up, and there are modifiable surgical factors that may have an influence on neurological development

[Neurological changes and outcomes of paediatric surgery of the aortic arch using selective cerebral perfusion]. / Evolución neurológica y resultados de la cirugía pediátrica del arco aórtico mediante perfusión cerebral selectiva.

INTRODUCTION: The aims of this article are to analyse the neuropsychological changes in the medium-term in children subjected to aortic arch surgery using selective cerebral perfusion (SCP), as well as to detect any modifiable factors in the surgical technique that may contribute to minimising the subsequent neurological involvement. MATERIAL AND METHODS: Inclusion criteria were established as: aortic arch disease operated on using SCP during the first year of life, between 10 August 2004 and 24 May 2016, biventricular physiology, and gestational age greater than 31 weeks. In the absence of a chromosomal disease, they were classified, from a neurological point of view, using the Rankin score. Children over 4-years of age were subjected to intelligence studies, including attention level, development, and psycho-lingual skills. RESULTS: The study included a total of 82 patients with a mean age of 1.8 months. The mean SCP flow was 32ml/kg/min. The mean time of SCP was 31minutes. The overall mortality of the series was 14.8%. Neurological dysfunction was observed in 35.9% of patients, and the following were detected as risk factors: surgery in patients less than 10-days-old, duration of SCP greater than 40minutes, and the time required for the cooling down and/or warming-up. Attention deficit was diagnosed in 35.2% of patients greater than 5-years-old. CONCLUSIONS: Patients operated on using SCP in in the first year of life required a neuropsychological follow-up, and there are modifiable surgical factors that may have an influence on neurological development.

Valores de referencia (Z-score) de diámetros de porción de entrada y trabecular del ventrículo derecho: primera descripción en población pediátrica española sana / Reference values (Z-score) of inlet and trabecular portion diameters of the right ventricle: First description in Spanish healthy children

INTRODUCCIÓN Y OBJETIVOS: La cuantificación del tamaño del ventrículo derecho (VD) es crucial en determinadas cardiopatías congénitas. Para su evaluación se recomiendan distintas técnicas de imagen, siendo más accesible la medición de diámetros ventriculares mediante ecocardiografía. En pediatría la normalización de parámetros ecocardiográficos es compleja, escasa y heterogénea. El objetivo de este estudio consistió en establecer Z-score de diámetros de VD fiables y reproducibles capaces de predecir valores de referencia en población pediátrica sana española. MÉTODOS: Estudio multicéntrico prospectivo de 661 pacientes sanos (edades 0-18 años, 43,5% mujeres). Los diámetros ecocardiográficos del VD se relacionaron con variables biométricas mediante distintas ecuaciones de regresión. Para su estandarización se analizaron factores de confusión (sexo, edad y variabilidad inter/intraobservador), heterocedasticidad y residuos (test Saphiro-Wilk y Breusch-Pagan). RESULTADOS: Se obtuvieron curvas de normalidad (Z-score) para cada diámetro del VD que permitieron predecir el valor medio de cada diámetro en función de la edad, peso, talla y distintas superficies corporales. La superficie corporal según fórmula de Haycock ofreció un excelente ajuste para los distintos diámetros basal, medial y longitudinal (R2 0,81; 0,82; 0,9). Los factores de confusión no aportaron cambios significativos, por lo que no fueron incluidos en los modelos finales (variabilidad inter- e intraobservador, CCI > 0,9). CONCLUSIONES: Se brindan valores de referencia de diámetros VD de población pediátrica sana. Las curvas de Z-score ofrecidas cubren una importante carencia en cardiología pediátrica y son aplicables a todos los grupos de edad para evaluar el tamaño del VD, de gran interés clínico en la práctica diaria

INTRODUCTION AND OBJECTIVES: Right ventricle (RV) measurements are crucial for certain congenital heart diseases and various cardiovascular conditions. Echocardiographic RV diameters are especially useful for its assessment. Paediatric echocardiographic data standardisation in normal subjects is complex, scarce, and heterogeneous. The aim of this study was to establish reliable and reproducible echocardiographic reference values (Z-score) of RV diameters in a healthy Spanish paediatric cohort. METHODS: A multicentre study was conducted on 661 healthy subjects (age range 0-18 years, 43.5% female). Several regression models were tested to examine the relationship between RV diameters and biometric variables. Heteroscedasticity and residual associations (Shapiro-Wilk and Breusch-Pagan tests) and confounding factors (gender, age, inter/intraobserver agreement) were considered for an unbiased standardisation. RESULTS: Structured Z-scores were computed for each RV diameter. Predicted mean value for each diameter was determined according to age, weight, height, and different body surface area. The Haycock formula provided the best fit for basal, midcavity, and longitudinal diameters (R2 0.81; 0.82; 0.9). Confounders were not significant, and therefore not included in final models (inter/intraobserver agreement > 0.9). CONCLUSIONS: This study reports reference values for echocardiographic RV diameters from a Spanish healthy paediatric cohort using a rigorous statistical design. These Z-scores partly cover a gap in current paediatric cardiology and represent a relevant diagnostic tool for clinical practice, as well as a useful guide to decision making at any paediatric stage

[Reference values (Z-score) of inlet and trabecular portion diameters of the right ventricle: First description in Spanish healthy children]. / Valores de referencia (Z-score) de diámetros de porción de entrada y trabecular del ventrículo derecho: primera descripción en población pediátrica española sana.

INTRODUCTION AND OBJECTIVES: Right ventricle (RV) measurements are crucial for certain congenital heart diseases and various cardiovascular conditions. Echocardiographic RV diameters are especially useful for its assessment. Paediatric echocardiographic data standardisation in normal subjects is complex, scarce, and heterogeneous. The aim of this study was to establish reliable and reproducible echocardiographic reference values (Z-score) of RV diameters in a healthy Spanish paediatric cohort. METHODS: A multicentre study was conducted on 661 healthy subjects (age range 0-18 years, 43.5% female). Several regression models were tested to examine the relationship between RV diameters and biometric variables. Heteroscedasticity and residual associations (Shapiro-Wilk and Breusch-Pagan tests) and confounding factors (gender, age, inter/intraobserver agreement) were considered for an unbiased standardisation. RESULTS: Structured Z-scores were computed for each RV diameter. Predicted mean value for each diameter was determined according to age, weight, height, and different body surface area. The Haycock formula provided the best fit for basal, midcavity, and longitudinal diameters (R2 0.81; 0.82; 0.9). Confounders were not significant, and therefore not included in final models (inter/intraobserver agreement > 0.9). CONCLUSIONS: This study reports reference values for echocardiographic RV diameters from a Spanish healthy paediatric cohort using a rigorous statistical design. These Z-scores partly cover a gap in current paediatric cardiology and represent a relevant diagnostic tool for clinical practice, as well as a useful guide to decision making at any paediatric stage.

Unusual distal right pulmonary artery origin from right ductus arteriosus with uncommon left-sided aortic arch / Inusual origen de la parte distal de la arteria pulmonar derecha en un ductus arterioso derecho en arco aórtico izquierdo no común

Abstract A bilateral persistent ductus arteriosus with right proximal pulmonary artery agenesis is a very unusual anatomic variant. These malformations are assumed to occur during the transformation of the sixth aortic arch with the pulmonary trunk in the development of the aorta and its branches. A 2-months preterm baby was referred by severe bronchopulmonary dysplasia. Transthoracic echocardiography showed a large pulmonary trunk and left pulmonary artery. No right proximal pulmonary artery was found. Cardiac computed tomography study showed bilateral ductus arteriosus. The right proximal pulmonary artery was originated from an arterial structure coming off the right subclavian artery, a right ductus arteriosus. The aortic arch was left-sided, with a peculiar variant at the exit of the supraaortic vessels. Right subclavian artery emerged as the first supraaortic vessel, followed by a common trunk, giving rise to both carotids and the left subclavian artery as the last vessel. Right lung was hypoplastic with interstitial disease. The calibre of the right proximal pulmonary artery was inadequate to perform a corrective surgery. LDA closure was performed, keeping a prostaglandin infusion to maintain right ductus arteriosus patency until right proximal pulmonary artery size will be big enough to perform a successful surgical reconstruction. Unfortunately, right ductus arteriosus became narrow at its origin and she developed severe pulmonary hypertension and the patient died.

Resumen El ductus arterioso bilateral con agenesia proximal de la arteria pulmonar derecha es una variante anatómica rara producida por alteraciones de la transformación del sexto arco aórtico en las arterias pulmonares, la aorta y sus vasos, durante el desarrollo embrionario. Se presenta el caso de un bebé prematuro de dos meses, que fue derivado a nuestro hospital por displasia broncopulmonar severa. El ecocardiograma transtorácico no identificó el origen de la arteria pulmonar derecha. El estudio por tomografía computarizada cardiaca mostró ductus arterioso bilateral e identificó el origen distal de la arteria pulmonar derecha en una rama de la arteria subclavia derecha, identificada como ductus arterioso derecho. El pulmón derecho era hipoplástico y presentaba alteraciones intersticiales. A su vez, el patrón de los vasos supraaórticos era anómalo. El primer vaso correspondió a la arteria subclavia derecha, seguido por tronco común de ambas carótidas y por último la arteria subclavia izquierda. El calibre de la arteria pulmonar derecha fue inadecuado para realizar una cirugía correctiva, por lo cual se decidió cerrar el ductus arterioso izquierdo y mantener infusión de prostaglandina hasta que la arteria pulmonar derecha tuviera un calibre adecuado para la cirugía. Infortunadamente, el ductus arterioso derecho se cerró en su origen, desarrollándose hipertensión pulmonar severa y la paciente falleció.

Eficacia de anakinra como tratamiento de la pericarditis recurrente / Efficacy of anakinra as a treatment for recurrent pericarditis

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A 12-year-old boy with chest pain and dyspnoea.

CLINICAL INTRODUCTION: A previously healthy 12-year-old boy presented with chest pain and dyspnoea. ECG (figure 1), echocardiogram (figure 2) and myocardial MRI (figure 3) were performed.Electromyography revealed mild proximal weakness. Blood tests showed creatine kinase 997 UI/L, aspartate transaminase 398 UI/L, alanine transaminase 293 UI/L and lactate deshidrogenase 1730 UI/L. Ophthalmology test showed diffuse loss of pigment in the retinal pigment epithelium. Myocardial biopsy was performed (figure 4). A cardiomyopathy was suspected. LAMP2 gene was sequenced, and a stop mutation was identified. QUESTION: What is the most likely diagnosis suggested based on the patient's tests and history? Danon's disease.Fabry's disease.Hypertrophic cardiomyopathy.Noonan's syndrome.Pompe's disease.

Cortical hyperostosis as a side-effect of prolonged use of prostaglandins.

Cortical hyperostosis is a very uncommon side-effect of prolonged prostaglandin therapy with distinctive radiological signs that may be accompanied by painful swelling of the limbs and responds rapidly to withdrawal of therapy.