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Pulmonary arterial hypertension in congenital heart disease

Heart BMJ

LEARNING OBJECTIVES Review the diagnostic pathway for pulmonary arterial hypertension (PAH) in congenital heart disease (CHD). Understand and interpret right heart catheterisation data in pulmonary hypertension related to CHD. Recognise the typical presentation of PAH in patients with CHD.

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Raising the bar: triple therapy for pulmonary arterial hypertension associated with congenital heart disease

Heart BMJ

Pulmonary arterial hypertension related to congenital heart disease as a paradigm of complexity Pulmonary arterial hypertension related to congenital heart disease (PAH-CHD) really represents a paradigm of complexity.

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Pulmonary arterial hypertension related to congenital heart disease with a left-to-right shunt: phenotypic spectrum and approach to management

Frontiers in Cardiovascular Medicine

Patients with pulmonary hypertension associated with a left-right shunt include a wide spectrum of pathophysiological substrates, ranging from those characterized by pulmonary over-circulation to those with advanced pulmonary vascular disease.

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Triple vasodilator therapy in pulmonary arterial hypertension associated with congenital heart disease

Heart BMJ

Objective This study assessed the long-term effects of triple therapy with prostanoids on patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), as there is limited information on the safety and efficacy of this treatment approach.

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Application of a modified clinical classification for pulmonary arterial hypertension associated with congenital heart disease in children: emphasis on atrial septal defects and transposition of the great arteries. An analysis from the TOPP registry

Frontiers in Cardiovascular Medicine

Aims A proportion of patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) do not fit in the current classification.

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Transpulmonary Expression of Exosomal microRNAs in Idiopathic and Congenital Heart Disease–Related Pulmonary Arterial Hypertension

Journal of the American Heart Association

BackgroundPulmonary artery hypertension (PAH) is a fatal disease characterized by a complex pathogenesis. Transpulmonary exosomal miRs offer valuable insights into pulmonary circulation microenvironments. Journal of the American Heart Association, Volume 12, Issue 23 , December 5, 2023.

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Implantation of atrial flow regulator devices in patients with congenital heart disease and children with severe pulmonary hypertension or cardiomyopathy—an international multicenter case series

Frontiers in Cardiovascular Medicine

Its use has been approved in the adult population with heart failure and described for pulmonary hypertension (PH). Only case reports and small series have been published about its use in the paediatric population and for congenital heart disease (CHD).