Effects of Cardiopulmonary Rehabilitation on the Muscle Function of Children with Congenital Heart Disease: A Prospective Cohort Study.

Critical medical and surgical advances have led to a shift in the care and management of children with congenital heart disease (CHD). These patients present with muscle deconditioning, which negatively influences their response to exercise, functional capacities, and quality of life. This study evaluates the influence of a cardiopulmonary rehabilitation program (CPRP) on the function of peripheral musculature of children with CHD. A single-center prospective cohort study was designed. Fifteen CHD subjects, between 12 and 16 years of age, with reduced aerobic capacity on a cardiopulmonary exercise test, were included in a three-month, 24-session CPRP. Measurements of the subjects' handgrip strength, biceps brachii and quadriceps femoris strength, and triceps surae fatigue process were collected at the beginning of the program, after completion, and six months after the end of the intervention. A substantial and statistically significant improvement was observed in the subjects' handgrip strength (kg) (p < 0.001), biceps brachii and quadriceps femoris strength (N) (p < 0.001), as well as triceps surae fatigue process (repetitions) (p = 0.018), with a maintenance of the results six months after the intervention. These results suggest that a CPRP could potentially improve the peripheral muscle function of children with CHD. Additional research is needed to confirm and expand on this hypothesis.

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.

Cardiopulmonary Rehabilitation Improves Respiratory Muscle Function and Functional Capacity in Children with Congenital Heart Disease. A Prospective Cohort Study.

Critical surgical and medical advances have shifted the focus of congenital heart disease (CHD) patients from survival to achievement of a greater health-related quality of life (HRQoL). HRQoL is influenced, amongst other factors, by aerobic capacity and respiratory muscle strength, both of which are reduced in CHD patients. This study evaluates the influence of a cardiopulmonary rehabilitation program (CPRP) on respiratory muscle strength and functional capacity. Fifteen CHD patients, ages 12 to 16, with reduced aerobic capacity in cardiopulmonary exercise testing (CPET) were enrolled in a CPRP involving strength and aerobic training for three months. Measurements for comparison were obtained at the start, end, and six months after the CPRP. A significant improvement of inspiratory muscle strength was evidenced (maximum inspiratory pressure 21 cm H2O, 23%, p < 0.01). The six-minute walking test showed a statistically and clinically significant rise in walked distance (48 m, p < 0.01) and a reduction in muscle fatigue (1.7 out of 10 points, p = 0.017). These results suggest CPRP could potentially improve respiratory muscle function and functional capacity, with lasting results, in children with congenital heart disease, but additional clinical trials must be conducted to confirm this finding.

Aneurisma del ductus arterioso: diagnóstico prenatal y evolución / Ductus arteriosus aneurysm: Prenatal diagnosis and outcome

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Aneurismas coronarios gigantes en lactantes con enfermedad de Kawasaki / Giant coronary aneurysms in infants with Kawasaki disease

Introducción: La enfermedad de Kawasaki (EK) es una vasculitis aguda de causa desconocida, predominante en el sexo masculino. Los efectos de la enfermedad a largo plazo dependen de la existencia de lesiones coronarias -aneurismas-, siendo el pronóstico de los pacientes con aneurismas gigantes sombrío, ya que en su evolución natural pueden presentar trombosis coronaria o lesiones obstructivas severas. Objetivos: Describimos las características de una serie de 8 pacientes lactantes con EK y severa afectación coronaria. Se describen la epidemiología y los métodos diagnósticos, y se revisa el tratamiento de la fase aguda, así como las secuelas cardiovasculares a largo plazo. Métodos: Se realizó un análisis descriptivo de pacientes ingresados en el Servicio de Cardiología Pediátrica del Hospital Universitario La Fe (Valencia) por EK con afectación coronaria. Resultados: En todos los casos se objetivó la afectación de más de una arteria. El diagnóstico se instauró precozmente en solo 2 casos, a pesar de ello, ningún paciente presentó deterioro grave de la función ventricular durante la fase aguda. El tratamiento incluyó gammaglobulina por vía intravenosa y ácido acetilsalicílico en dosis antiinflamatorias durante la fase aguda. En los casos de trombosis coronaria se asociaron doble antiagregación y corticoides. Las lesiones aneurismáticas silentes persisten hasta el momento actual. Conclusiones: La EK es la causa más común de enfermedad cardiaca adquirida en niños. El retraso en el diagnóstico se asocia a mayor frecuencia de lesiones coronarias, que podrían incrementar el riesgo de eventos cardiovasculares en la edad adulta, precisando este subgrupo un seguimiento clínico estrecho con control de los factores de riesgo cardiovascular a largo plazo (AU)

Introduction: Kawasaki disease (KD) is an acute vasculitis of unknown origin and predominant in males. The long-term effects of the disease depend on whether there are coronary lesions, particularly aneurysms. The prognosis of patients with giant aneurysms is very poor due to their natural progression to coronary thrombosis or severe obstructive lesions. Objectives: A series of 8 cases is presented where the epidemiology and diagnostic methods are described. The treatment of the acute and long-term cardiovascular sequelae is also reviewed. Methods: A descriptive analysis was conducted on patients admitted to the Paediatric Cardiology Unit of La Fe University Hospital (Valencia) with KD and a coronary lesion. Results: More than one artery was involved in all patients. Although early diagnosis was established in only two cases, none of the patients had severe impairment of ventricular function during the acute phase. Treatment included intravenous gammaglobulin and acetylsalicylic acid at anti-inflammatory doses during the acute phase. A combination of dual antiplatelet therapy and corticosteroids was given in cases of coronary thrombosis. The silent aneurysms continue to persist. Conclusions: KD is the most common cause of acquired heart disease in children. The delay in diagnosis is associated with a greater likelihood of coronary lesions that could increase the risk of cardiovascular events in adulthood. Thus, this subgroup requires close clinical monitoring for a better control of cardiovascular risk factors over time (AU)

[Giant coronary aneurysms in infants with Kawasaki disease]. / Aneurismas coronarios gigantes en lactantes con enfermedad de Kawasaki.

INTRODUCTION: Kawasaki disease (KD) is an acute vasculitis of unknown origin and predominant in males. The long-term effects of the disease depend on whether there are coronary lesions, particularly aneurysms. The prognosis of patients with giant aneurysms is very poor due to their natural progression to coronary thrombosis or severe obstructive lesions. OBJECTIVES: A series of 8 cases is presented where the epidemiology and diagnostic methods are described. The treatment of the acute and long-term cardiovascular sequelae is also reviewed. METHODS: A descriptive analysis was conducted on patients admitted to the Paediatric Cardiology Unit of La Fe University Hospital (Valencia) with KD and a coronary lesion. RESULTS: More than one artery was involved in all patients. Although early diagnosis was established in only two cases, none of the patients had severe impairment of ventricular function during the acute phase. Treatment included intravenous gammaglobulin and acetylsalicylic acid at anti-inflammatory doses during the acute phase. A combination of dual antiplatelet therapy and corticosteroids was given in cases of coronary thrombosis. The silent aneurysms continue to persist. CONCLUSIONS: KD is the most common cause of acquired heart disease in children. The delay in diagnosis is associated with a greater likelihood of coronary lesions that could increase the risk of cardiovascular events in adulthood. Thus, this subgroup requires close clinical monitoring for a better control of cardiovascular risk factors over time.

Miocardiopatía hipertrófica intraútero y síndrome de Noonan neonatal: una asociación a recordar / Prenatal hypertrophic cardiomyopathy and neonatal noonan syndrome: an association to remember

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Endocarditis sobre válvula de Eustaquio redundante: diagnóstico neonatal / Redundant eustachian valve endocarditis: neonatal diagnosis

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