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Acquired fontan paradox in isolated right ventricular cardiomyopathy


Department of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India

Correspondence Address:
Mahim Saran
Department of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram - 695 011, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-2069.189117

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Year : 2016  |  Volume : 9  |  Issue : 3  |  Page : 251-253

 

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A 44-year-old woman presented with features of congestive heart failure. Echocardiography revealed severe right ventricular dysfunction along with passive minimally pulsatile pulmonary blood flow suggesting very high systemic venous pressures. This was confirmed with cardiac catheterization in which the pressures of superior vena cava and inferior vena cava (19 mmHg) were higher than the pulmonary artery pressures (17 mmHg). Elevation of systemic venous pressures above the pulmonary venous pressures, Fontan paradox, to drive the forward flow, is a specific feature of artificially created cavopulmonary shunts. Late stage of isolated right ventricular cardiomyopathy resulted in the spontaneous evolution of Fontan circulation with a nonfunctional right ventricle in this patient.






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Department of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India

Correspondence Address:
Mahim Saran
Department of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram - 695 011, Kerala
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-2069.189117

Rights and Permissions

A 44-year-old woman presented with features of congestive heart failure. Echocardiography revealed severe right ventricular dysfunction along with passive minimally pulsatile pulmonary blood flow suggesting very high systemic venous pressures. This was confirmed with cardiac catheterization in which the pressures of superior vena cava and inferior vena cava (19 mmHg) were higher than the pulmonary artery pressures (17 mmHg). Elevation of systemic venous pressures above the pulmonary venous pressures, Fontan paradox, to drive the forward flow, is a specific feature of artificially created cavopulmonary shunts. Late stage of isolated right ventricular cardiomyopathy resulted in the spontaneous evolution of Fontan circulation with a nonfunctional right ventricle in this patient.






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