Abstract
Pulmonary hypertensive vascular disease (PHVD), and pulmonary hypertension (PH), which is a broader term, are severe conditions associated with high morbidity and mortality at all ages. Treatment guidelines in childhood are widely adopted from adult data and experience, though big differences may exist regarding aetiology, concomitant conditions and presentation. Over the past few years, paediatric aspects have been incorporated into the common guidelines, which currently address both children and adults with pulmonary hypertension (PH). There are multiple facets of PH in the context of cardiac conditions in childhood. Apart from Eisenmenger syndrome (ES), the broad spectrum of congenital heart disease (CHD) comprises PH in failing Fontan physiology, as well as segmental PH. In this review we provide current data and novel aspects on the pathophysiological background and individual management concepts of these conditions. Moreover, we focus on paediatric left heart failure with PH and its challenging issues, including end stage treatment options, such as mechanical support and paediatric transplantation. PH in the context of rare congenital disorders, such as Scimitar Syndrome and sickle cell disease is discussed. Based on current data, we provide an overview on multiple underlying mechanisms of PH involved in these conditions, and different management strategies in children and adulthood. In addition, we summarize the paediatric aspects and the pros and cons of the recently updated definitions of PH. This review provides deeper insights into some challenging conditions of paediatric PH in order to improve current knowledge and care for children and young adults.
Highlights
Pulmonary Hypertension (PH) or Pulmonary Hypertensive Vascular Disease (PHVD) is a severe condition characterized by a progressive increase in pressure or vascular resistance, leading to chronic right heart failure
The aim of this review is to discuss some of the novel changes of the sixth WSPH, and to highlight some challenging issues specific for paediatric PHVD that have not been covered by previous reviews
Many aspects are derived from research achievements originating from adult pulmonary hypertension (PH), which has provided substantial knowledge and evidence, especially in management modalities
Summary
Pulmonary Hypertension (PH) or Pulmonary Hypertensive Vascular Disease (PHVD) is a severe condition characterized by a progressive increase in pressure or vascular resistance, leading to chronic right heart failure. While treatment guidelines in childhood are mainly based on adult data and experience, significant differences exist regarding aetiology, concomitant conditions and presentation. Current registries have provided a growing understanding of specific features, presentation, epidemiology and outcomes of paediatric pulmonary hypertension (PH) [1,2,3,4]. The aim of this review is to discuss some of the novel changes of the sixth WSPH, and to highlight some challenging issues specific for paediatric PHVD that have not been covered by previous reviews. We discuss advanced PH treatments due to left heart disease in children. Some conditions are still not covered in current guidelines and recommendations and require a patient-based approach
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have