Andrew Constantine, Robert M. R. Tulloh, Konstantinos Dimopoulos, Petra Jenkins, Robin Condliffe, Katrijn Jansen, Natali A. Y. Chung, James Oliver, Helen Parry, Samantha Fitzsimmons, Niki Walker, Stephen John Wort, Vasilios Papaioannou, Kate von Klemperer, Paul Clift
Royal Brompton HospitalRoyal Brompton and Harefield Hospitals. Imperial College London. Liverpool Heart and Chest Hospital. University Hospital Bristol. Royal Hallamshire Hospital and Sheffield Teaching Hospitals. Freeman Hospital and Newcastle Upon Tyne Hospitals. St Thomas’ Hospital. Leeds Teaching Hospitals. Southampton General Hospital and University Hospital. Golden Jubilee National Hospital. St Bartholomew’s Hospital. Queen Elizabeth Hospital.
United Kingdom
Journal of the American Heart Association
J Am Heart Assoc 2022; 11:
DOI: 10.1161/JAHA.121.023035
Abstract
Background: The Fontan circulation is a successful operative strategy for abolishing cyanosis and chronic volume overload in patients with congenital heart disease 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 patients with Fontan physiology receiving pulmonary arterial hypertension (PAH) therapies across specialist centers in the United Kingdom.
Methods and Results: We identified all adult patients with a Fontan-type circulation under active follow-up in 10 specialist congenital heart disease centers in England and Scotland between 2009 and 2019. Patients taking PAH therapies were matched to untreated patients. A survey of experts was also performed. Of 1538 patients with Fontan 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, have a 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 of 12 months (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 patients with Fontan circulation followed in specialist centers, targeting individuals with advanced disease or complications. Follow-up suggests stabilization of the clinical status after 12 months of therapy.
Category
Class I. Pulmonary Hypertension Associated with Congenital Heart Disease
Medical Therapy. Efficacy or Lack of Efficacy
Medical Therapy. Adverse Effects or Lack of Adverse Effects
Age Focus: Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes