Imperial College London
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Professor Konstantinos Dimopoulos

Faculty of Medicine, National Heart & Lung Institute

Professor of Practice (Adult Congenital Heart Disease)
 
 
 
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Contact

 

+44 (0)20 7352 8121 ext 82771k.dimopoulos02

 
 
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Location

 

Chelsea WingRoyal Brompton Campus

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Summary

 

Publications

Citation

BibTex format

@article{Constantine:2021:10.1161/JAHA.121.023035,
author = {Constantine, A and Dimopoulos, K and Jenkins, P and Tulloh, RMR and Condliffe, R and Jansen, K and Chung, NAY and Oliver, J and Parry, H and Fitzsimmons, S and Walker, N and Wort, S and Papaioannou, V and von, Klemperer K and Clift, P},
doi = {10.1161/JAHA.121.023035},
journal = {Journal of the American Heart Association},
pages = {1--19},
title = {Use of pulmonary arterial hypertension therapies in Fontan patients: current practice across the United Kingdom},
url = {http://dx.doi.org/10.1161/JAHA.121.023035},
volume = {11},
year = {2021}
}
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RIS format (EndNote, RefMan)

TY  - JOUR
AB  - Background: The Fontan circulation is a successful operative strategy for abolishing cyanosis and chronic volume overload in congenital heart disease (CHD) patients with single ventricle physiology. ‘Fontan failure’ is a major cause of poor quality of life and mortality in these patients. We assessed the number and clinical characteristics of adult Fontan patients receiving pulmonary arterial hypertension (PAH) therapies across specialist centers in the UK.Methods and Results: We identified all adult patients with a Fontan-type circulation under active follow-up in 10 specialist CHD centers in England and Scotland between 2009 and 2019. Patients on PAH therapies were matched to untreated patients. A survey of experts was also performed. Of 1538 Fontan patients followed in specialist centers, only 76 (4.9%) received PAH therapies during follow-up. The vast majority (90.8%) were treated with a phosphodiesterase-5 inhibitor. In 33% of patients, PAH therapies were started after surgery or during hospital admission. In the matched cohort, treated patients were more likely to be significantly limited, have ascites, history of protein losing enteropathy, or receive loop diuretics (p<0.0001 for all), also reflecting survey responses indicating that failing Fontan is an important treatment target. After a median 12[11-15] months, functional class was more likely to improve in the treated group (p=0.01), with no other changes in clinical parameters or safety issues. Conclusions: PAH therapies are used in adult Fontan patients followed in specialist centers, targeting individuals with very advanced disease or complications. Follow-up suggests stabilization of the clinical status after 12 months of therapy.
AU  - Constantine,A
AU  - Dimopoulos,K
AU  - Jenkins,P
AU  - Tulloh,RMR
AU  - Condliffe,R
AU  - Jansen,K
AU  - Chung,NAY
AU  - Oliver,J
AU  - Parry,H
AU  - Fitzsimmons,S
AU  - Walker,N
AU  - Wort,S
AU  - Papaioannou,V
AU  - von,Klemperer K
AU  - Clift,P
DO  - 10.1161/JAHA.121.023035
EP  - 19
PY  - 2021///
SN  - 2047-9980
SP  - 1
TI  - Use of pulmonary arterial hypertension therapies in Fontan patients: current practice across the United Kingdom
T2  - Journal of the American Heart Association
UR  - http://dx.doi.org/10.1161/JAHA.121.023035
UR  - https://www.ahajournals.org/doi/10.1161/JAHA.121.023035
UR  - http://hdl.handle.net/10044/1/93022
VL  - 11
ER  -
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