A retrospective review of sildenafil in the Australia and New Zealand Fontan registry: Indications and treatment responses
International Journal of Cardiology Congenital Heart Disease(2024)
摘要
Introduction
Rates of morbidity and mortality are high in the setting of Fontan physiology and effective medical therapies are not well-established. Clinical trials assessing phosophodiesterase-5-inhibitors, such as sildenafil, have not demonstrated major benefit in patients with a Fontan-type circulation but have only included stable, well-functioning people.
Methods
We sought to retrospectively characterize the people followed by the ANZ Fontan Registry prescribed sildenafil >30 days post Fontan--surgery.
Results
Of 1867 individuals, 19 (1 %) were prescribed sildenafil and met inclusion criteria; 12 (63 %) were female. Age at commencement was 9 (IQR 13.5) years. Reasons for commencement were Fontan failure with elevated Fontan pressure (n = 4), failure without elevated pressure (n = 4), elevated pressure without failure (n = 6), failure symptoms without invasive assessment (n = 3) and pulmonary arterio-venous malformations (n = 2). At baseline hemodynamic study (n = 16), median Fontan pressure was 15.5 (IQR 3.1) mmHg and PVRi was 2.3 (IQR 1.6) WU*m2. Improvement in the clinical indication was noted in 11/19 (58 %). In the subset in whom sildenafil was commenced with PVRi >2 WU*m2 6/7 (86 %) improved clinically. PVRi decreased by 1.8 (IQR 1.0, n = 5,p = 0.03) WU*m2 on treatment in this group, compared with the <2 WU*m2 group which increased by 0.3 (IQR 0.2, n = 3, p = 0.01) WU*m2.
Conclusions
Some individuals with a Fontan circulation may benefit from sildenafil. Adequately designed clinical trials are needed.
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关键词
Congenital heart disease,Fontan circulation,Fontan failure,Pulmonary vasodilator,Sildenafil
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