Focused Update on Pulmonary Hypertension in Children—Selected Topics of Interest for the Adult Cardiologist

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.

Double Lung and Heart-Lung Transplantation for Congenital Heart Disease with Eisenmenger Syndrome and Idiopathic Pulmonary Arterial Hypertension

“…The Hannover group reported successful bridges to recovery in patients with PH with the use of awake venoarterial extracorporeal membrane oxygenation [19]. Complex ES remains the main indication for HLTx in PAH [20].…”

“…An important outcome predictor of both procedures is the degree of preoperative right ventricular dysfunction. For successful DLTx, authors propose that patients should have a left ventricular ejection fraction between 32 and 55% and right ventricular ejection fraction between 10 and 25% [20,22,27,28]. In most patients with pulmonary arterial hypertension and preserved left ventricular (LV) function, there is no advantage to HLTx compared with isolated DLTx, even in the presence of limited right ventricular dysfunction.…”