[Successful chronic treatment with sildenafil in a patient with end-stage heart failure following Fontan procedure]

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

Kardiologia Polska
|March 25, 2011
PubMed

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.

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