There are few randomised controlled data to guide management of patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD). In this clinical review, common areas of uncertainty in the management of PAH-CHD are identified, the literature is summarised and discussed and a suggested approach offered for each clinical dilemma.
There are few randomised controlled data to guide management of patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD). In this clinical review, common areas of uncertainty in the management of PAH-CHD are identified, the literature is summarised and discussed and a suggested approach offered for each clinical dilemma.
“…In hemitruncus arteriosus, a lesion in which one PA is normally connected, but the other one derives from the aorta, segmental PH can develop in the latter due to pressure and volume overload. Both lesions are normally corrected early in infancy; however, if there is unequal blood distribution due to postoperative PA stenosis or the hypoplasia of branch arteries, segmental PH may occur in the contralateral PA [ 218 ].…”
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.
“…Symptoms of segmental PH are often proportional to the severity of the ventilation-to-perfusion mismatch. A consensus statement to define and classify segmental PH is published in 2018 [ 1 ]. According to the statement, segmental PH is commonly encountered in patients with congenital heart diseases (CHD) such as tetralogy of Fallot (TOF) with pulmonary atresia and ventricular septal defect (VSD), hemitruncus arteriosus (one pulmonary artery is normally connected, but the other one arises from the aorta), truncus arteriosus with stenosis or hypoplasia of a single pulmonary artery or branches, unilateral absence pulmonary artery or isolated pulmonary artery of ductal origin, any large post-tricuspid shunts with peripheral pulmonary stenosis, and isolated peripheral pulmonary artery stenosis, for example, Alagille syndrome.…”
Section: Introductionmentioning
confidence: 99%
“…On the other hand, when RV is directly connected to the pulmonary circulation, the effect of segmental PH on RV is in addition to the load imposed by obstructive lesions such as branch pulmonary artery stenosis or RV-pulmonary artery conduit stenosis. It is essential to understand the anatomy of pulmonary arteries and major aorto-pulmonary collaterals (MAPCAs), as these influence the development of PH, response to therapy, long-term outcome and is key to decide when a patient can undergo bi-ventricular repair [ 1 ]. Although cardiac catheterization is the standard for assessing segmental PH, the calculation of pulmonary vascular resistance (PVR) is difficult and sometimes not helpful.…”
Segmental pulmonary hypertension is a complex condition in children that encompasses many congenital heart diseases including pulmonary atresia with ventricular septal defect, hemitruncus/truncus arteriosus with branch pulmonary artery stenosis, unilateral absent pulmonary artery, and several post-tricuspid shunt lesions. Multimodality imaging is required to confirm and assess pulmonary vascular disease in subjects with major aorto-pulmonary collaterals. We describe 3 children with complex congenital heart defects who have a variable degree of segmental pulmonary hypertension and discuss management strategies and the role of interventional and/or pulmonary hypertension targeted therapies.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
