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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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Trisomy 21 and Congenital Heart Disease: Impact on Health and Functional Outcomes From Birth Through Adolescence: A Scientific Statement From the American Heart Association

Journal of the American Heart Association

Due to improvements in recognition and management of their multisystem disease, the long‐term survival of infants, children, and adolescents with trisomy 21 and congenital heart disease now matches children with congenital heart disease and no genetic condition in many scenarios.

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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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Clinical risk associated with COVID-19 among 86000 patients with congenital heart disease

Open Heart

Objective To determine the magnitude of any excess risk of mortality and hospitalisation due to COVID-19 infection in patients with congenital heart disease (CHD) in the UK healthcare system. We provide the first primary care-derived estimates for COVID-19 hospitalisation and case-fatality rates in patients with CHD.

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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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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).

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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.