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J Am Coll Cardiol. 2018 Dec 4;72(22):2778-2788. doi: 10.1016/j.jacc.2018.08.2201.

Echocardiographic Screening for Pulmonary Hypertension in Congenital Heart Disease: JACC Review Topic of the Week.

Author information

1
Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital and Imperial College London, London, United Kingdom. Electronic address: k.dimopoulos02@gmail.com.
2
Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, United Kingdom.
3
Bristol Heart Institute, University Hospitals Bristol, Bristol, United Kingdom.
4
Department of Cardiology, Queen Elizabeth Hospital Birmingham, Birmingham, United Kingdom.
5
Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital and Imperial College London, London, United Kingdom.
6
Adult Congenital Heart Disease Service, Guy's and St Thomas' Hospital, London, United Kingdom.
7
National Pulmonary Hypertension Service, Royal Free Hospital, London, United Kingdom.
8
Adult Congenital Heart Disease Unit, Southampton University Hospital, Southampton, United Kingdom.
9
National Pulmonary Hypertension Service, Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, United Kingdom.
10
Adult Congenital Heart Disease Unit, Manchester Royal Infirmary, Manchester, United Kingdom.
11
Our Lady's Children's Hospital and Mater Hospital, Dublin, Ireland.
12
East Midlands Congenital Heart Centre, Leicester Cardiovascular Biomedical Research Unit, Glenfield Hospital, Leicester, United Kingdom.
13
Department of Pulmonary Hypertension, Royal Brompton Hospital, London, United Kingdom.
14
Leeds Congenital Heart Unit, Leeds Teaching Hospitals, Leeds, United Kingdom.
15
Department of Cardiology, Royal Victoria Hospital, Belfast, Northern Ireland.
16
Cardiology Department, University Hospital of Wales, Cardiff, United Kingdom.
17
Grown-up Congenital Heart Disease Service, Barts Heart Centre, St. Bartholomew's Hospital, London, United Kingdom.

Abstract

Echocardiography is the mainstay in screening for pulmonary hypertension (PH). International guidelines suggest echocardiographic parameters for suspecting PH, but these may not apply to many adults with congenital heart disease (ACHD). PH is relatively common in ACHD patients and can significantly affect their exercise capacity, quality of life, and prognosis. Identification of patients who have developed PH and who may benefit from further investigations (including cardiac catheterization) and treatment is thus extremely important. A systematic review and survey of experts from the United Kingdom and Ireland were performed to assess current knowledge and practice on echocardiographic screening for PH in ACHD. This paper presents the findings of the review and expert statements on the optimal approaches when using echocardiography to assess ACHD patients for PH, with particular focus on major subgroups: patients with right ventricular outflow tract obstruction, patients with systemic right ventricles, patients with unrepaired univentricular circulation, and patients with tetralogy of Fallot with pulmonary atresia.

KEYWORDS:

cardiac catheterization; echocardiography; pulmonary atresia; pulmonary stenosis; transposition of great arteries; univentricular heart

PMID:
30497564
DOI:
10.1016/j.jacc.2018.08.2201
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