[Successful chronic treatment with sildenafil in a patient with end-stage heart failure following Fontan procedure]
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Updated: Jun 3, 2026

Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 18, 2014
Jacek Białkowski1, Jarosław Rycaj, Roland Fiszer
1Oddział Kliniczny Wrodzonych Wad Serca i Kardiologii Dziecięcej, Śląski Uniwersytet Medyczny, Śląskie Centrum Chorób Serca, Zabrze. abi_med@poczta.onet.pl
Insights
Sildenafil effectively treated severe heart failure in a Fontan patient with high pulmonary vascular resistance. This long-term medication improved symptoms of heart insufficiency, offering a new therapeutic option.
Area of Science:
- Cardiology
- Pulmonary Hypertension
- Pediatric Cardiac Surgery
Background:
- The Fontan procedure is a palliative surgery for complex congenital heart defects.
- Increased pulmonary vascular resistance (PVR) and pulmonary artery pressure (PAP) are significant long-term complications after Fontan surgery.
- Severe heart insufficiency can develop due to elevated PVR and PAP in Fontan patients.
Observation:
- A 21-year-old male, 17 years post-Fontan correction, presented with severe heart insufficiency (NYHA class III).
- The patient exhibited cachexia, massive peripheral edema, and ascites.
- These symptoms were attributed to significantly increased pulmonary vascular resistance and pulmonary artery pressure.
Findings:
- Long-term sildenafil medication was administered to the patient.
- Sildenafil treatment resulted in successful management of the severe heart insufficiency symptoms.
- The patient showed significant clinical improvement following sustained sildenafil therapy.
Implications:
- Sildenafil may be a viable long-term treatment option for Fontan patients with heart failure secondary to increased PVR and PAP.
- This case highlights the potential of targeted pharmacotherapy to manage late complications of Fontan circulation.
- Further research is warranted to explore the efficacy and safety of sildenafil in similar patient populations.
Abstract:
We present a case of a 21 year-old man who has had Fontan type correction 17 years ago with symptoms of severe heart insufficiency (III NYHA class, cahectic, with massive peripheral oedema and ascites) caused by increased pulmonary vascular resistance and increased pulmonary artery pressure. He was treated successfully with long term sildenafil medication.
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