Short- and long-term survival of children treated with ventricular assist devices in Spain, based on 15 years' experience.

OBJECTIVES: To describe the use of ventricular assist devices (VAD) in children in Spain and to identify variables related to survival. METHODS: This is an observational cohort study of all children younger than 18 years of age who underwent an initial implantation of a VAD at any of the 6 paediatric heart transplant centres from May 2006 to December 2020. Subjects were identified retrospectively from each hospital's database. RESULTS: Paracorporeal VADs were implanted in 118 children [pulsatile (63%), continuous (30.5%) or both types (5.9%)]. Small children (<0.7 m2 of body surface area) comprised the majority of this cohort (63.3%). Overall, 67% survived to VAD explantation, and 64.9% survived to hospital discharge. Non-central nervous system haemorrhage (39%) and stroke (38.1%) were the most common complications. Body weight <5 kg, congenital heart disease, pre-implantation bilirubin >34 µmol/l and bridge to decision strategy were associated with a higher mortality at hospital discharge and in the long-term. Interagency registry for mechanically assisted circulatory support (INTERMACS) status 1 and cardiac arrest prior to VAD implantation were related to long-term mortality, whereas pre-implantation renal replacement therapy and extracorporeal membrane oxygenation were not related to mortality. CONCLUSIONS: In Spain, 67% of the VAD-supported children have been bridged to heart transplantation or to recovery. Body weight lower than 5 kg, congenital heart disease diagnosis, cholestatic liver dysfunction, bridge to decision as VAD strategy, INTERMACS-1 status and cardiac arrest were pre-implantation variables related to mortality, whereas pre-implantation renal replacement therapy and extracorporeal membrane oxygenation were not.

Inodilators in the Management of Low Cardiac Output Syndrome After Pediatric Cardiac Surgery.

Postoperative low cardiac output syndrome has been shown to have both a central and a peripheral vascular involvement. Therefore, inodilators which provide with a combination of positive inotropic and vasodilating therapy, conceptually should be an ideal form of treatment. However, contradictory data on these drugs exist. Phosphodiesterase inhibitors (e.g., milrinone) and more recently calcium sensitizers (e.g., levosimendan) have been most commonly used groups in the clinical setting. This review will summarize the pharmacology of inodilators with a special foccus on current clinical evidence. This article addresses the sixth of eight topics comprising the special issue entitled "Pharmacologic strategies with afterload reduction in low cardiac output syndrome after pediatric cardiac surgery".

[Treatment with sildenafil, bosentan, or both in children and young people with idiopathic pulmonary arterial hypertension and Eisenmenger's syndrome]. / Tratamiento con sildenafilo y/o bosentán en niños y jóvenes con hipertensión arterial pulmonar idiopática y síndrome de Eisenmenger.

INTRODUCTION AND OBJECTIVES: Pulmonary arterial hypertension carries a poor prognosis in both adult and pediatric patients. Current understanding of the mechanisms underlying pulmonary arterial hypertension has enabled the rapid development of appropriate drugs, such as endothelin receptor antagonists and 5-phosphodieste-rase inhibitors, that can be administered orally and which are generally well tolerated. The aims of the present study were to evaluate functional class and exercise capacity following long-term treatment with sildenafil or bosentan in patients with idiopathic pulmonary arterial hypertension and Eisenmenger's syndrome and to compare results in the two groups. METHODS: Seven patients were included in the pulmonary arterial hypertension study, and diagnoses of idiopathic pulmonary arterial hypertension were confirmed. Five patients were treated with sildenafil, while two received bosentan. The five patients with a non-restrictive ventricular septal defect and pulmonary arterial hypertension were treated with sildenafil. In one patient, bosentan was added to the sildenafil. RESULTS: Both sildenafil and bosentan significantly improved exercise capacity in patients with idiopathic pulmonary arterial hypertension. The treatment effect was less in those with Eisenmenger physiology. Although the improvement in World Health Organization functional class was greater in patients with idiopathic pulmonary arterial hypertension, it was significant in both groups. CONCLUSIONS: Long-term treatment with sildenafil and bosentan improved both exercise capacity and functional class in patients with idiopathic pulmonary arterial hypertension and in those with hypertension due to congenital heart disease. The changes were more marked in patients with idiopathic pulmonary arterial hypertension.

Tratamiento con sildenafilo y/o bosentán en niños y jóvenes con hipertensión arterial pulmonar idiopática y síndrome de Eisenmenger / Treatment with sildenafil, bosentan, or both in children and young people with idiopathic pulmonary arterial hypertension and Eisenmenger's Syndrome

Introducción y objetivos. La hipertensión arterial pulmonar tiene mal pronóstico en pacientes adultos y pediátricos. El conocimiento actual de los mecanismos que condicionan la hipertensión arterial pulmonar ha permitido descubrir medicamentos como los antagonistas de los receptores de la endotelina e inhibidores de la 5-fosfodiesterasa, administrables ambos por vía oral y generalmente bien tolerados. En este estudio se valora la capacidad funcional y la tolerancia al ejercicio en el tratamiento a largo plazo con sildenafilo o bosentán en pacientes con hipertensión arterial pulmonar idiopática y síndrome de Eisenmenger, y se comparan los resultados en ambos grupos de pacientes. Métodos. Siete pacientes fueron incluidos en el protocolo de estudio de la hipertensión arterial pulmonar, y en ellos se confirmó el diagnóstico de hipertensión arterial pulmonar idiopática. Se trató con sildenafilo a 5 pacientes y con bosentán a 2. Cinco pacientes con comunicación interventricular no restrictiva e hipertensión arterial pulmonar fueron tratados con sildenafilo. En uno de ellos se añadió bosentán al tratamiento con sildenafilo. Resultados. El sildenafilo y el bosentán mejoraron significativamente la capacidad de ejercicio en pacientes con hipertensión arterial pulmonar idiopática. El efecto fue menor en los pacientes con fisiología de síndrome de Eisenmenger. La mejoría en la clase funcional de la Organización Mundial de la Salud fue mayor en los pacientes con hipertensión arterial pulmonar idiopática, aunque fue significativa en ambos grupos. Conclusiones. El tratamiento a largo plazo con sildenafilo y bosentán mejora la capacidad de ejercicio y la clase funcional en la hipertensión arterial pulmonar idiopática y debida a cardiopatías congénitas. Los cambios son más llamativos en los pacientes con hipertensión arterial pulmonar idiopática (AU)

Introduction and objectives. Pulmonary arterial hypertension carries a poor prognosis in both adult and pediatric patients. Current understanding of the mechanisms underlying pulmonary arterial hypertension has enabled the rapid development of appropriate drugs, such as endothelin receptor antagonists and 5-phosphodieste-rase inhibitors, that can be administered orally and which are generally well tolerated. The aims of the present study were to evaluate functional class and exercise capacity following long-term treatment with sildenafil or bosentan in patients with idiopathic pulmonary arterial hypertension and Eisenmenger's syndrome and to compare results in the two groups. Methods. Seven patients were included in the pulmonary arterial hypertension study, and diagnoses of idiopathic pulmonary arterial hypertension were confirmed. Five patients were treated with sildenafil, while two received bosentan. The five patients with a non-restrictive ventricular septal defect and pulmonary arterial hypertension were treated with sildenafil. In one patient, bosentan was added to the sildenafil. Results. Both sildenafil and bosentan significantly improved exercise capacity in patients with idiopathic pulmonary arterial hypertension. The treatment effect was less in those with Eisenmenger physiology. Although the improvement in World Health Organization functional class was greater in patients with idiopathic pulmonary arterial hypertension, it was significant in both groups. Conclusions. Long-term treatment with sildenafil and bosentan improved both exercise capacity and functional class in patients with idiopathic pulmonary arterial hypertension and in those with hypertension due to congenital heart disease. The changes were more marked in patients with idiopathic pulmonary arterial hypertension (AU)