Key result
Phlebotomy and supportive care improve symptoms to NYHA class I in adult unoperated TOF-PA.
Why the study?
Tetralogy of Fallot with pulmonary atresia is one of the most severe cyanotic congenital heart diseases, and long-term survival without surgical intervention is exceedingly rare.
Case Report (n=1)
No
This case report demonstrates rare 43-year unoperated survival and successful completion of two pregnancies in a patient with Tetralogy of Fallot and pulmonary atresia, supported by extensive major aortopulmonary collateral arteries (MAPCAs) and managed conservatively for hyperviscosity syndrome.
Answers are grounded in this paper and cite their sources.
Phlebotomy may relieve hyperviscosity symptoms in unoperated TOF-PA; leaves open generalizability and long-term benefit beyond this single case.
Background: Tetralogy of Fallot with pulmonary atresia (TOF-PA) is one of the most severe cyanotic congenital heart diseases.Long-term survival without surgical intervention is exceedingly rare. Case Summary:We report a 43-year-old female from Karnataka, India, who presented with dyspnea on exertion, headache, and blurring of vision for 2 months.She had never undergone any cardiac surgery, surviving 2 pregnancies with no postnatal complications.On examination, she had SpO₂ 78%, marked clubbing, and a continuous murmur over all lung fields, suggestive of major aortopulmonary collateral arteries (MAPCAs).Hyperviscosity symptoms were also present.Echocardiography confirmed TOF-PA physiology, and her long-term survival is attributed to extensive systemicpulmonary collateralization.Conclusion: This case exemplifies survival in unoperated TOF-PA due to well-developed MAPCAs, emphasizing the need for lifelong monitoring.
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A 2026 study conducted a case report in Unoperated Tetralogy of Fallot with pulmonary atresia, major aortopulmonary collateral arteries (MAPCAs), and hyperviscosity syndrome (n=1). Phlebotomy and supportive medical management was evaluated on Symptomatic improvement and NYHA functional class at discharge. Supportive management with phlebotomy improved symptoms from NYHA class II-III to class I in a 43-year-old woman with unoperated TOF-PA, SpO2 78%, and extensive MAPCAs.
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