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Severe pulmonary arterial hypertension in a patient with hereditary hemorrhagic telangiectasia and multiple pulmonary and hepatic arteriovenous malformations


1 Department of Cardiology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India
2 Department of Radiology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

Correspondence Address:
Dr. Sreekanth Yerram
Department of Cardiology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/apc.apc_256_20

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Year : 2022  |  Volume : 15  |  Issue : 1  |  Page : 73-76

 

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Hereditary hemorrhagic telangiectasia (HHT) is a genetic disorder in which there is faulty development of the arteries. There is a high incidence of pulmonary hypertension (PH) in these patients, the pathophysiology of which is not fully known. An increase in cardiac output, causing high-output cardiac failure, and increased pulmonary vascular resistance secondary to genetic mutations are the main reasons. We report a 25-year-old male with HHT who presented with right heart failure secondary to PH in whom both the above mechanisms were operating. The coexistence of giant pulmonary arteriovenous malformations with severe PH is a rare scenario influencing management decisions that are discussed. In addition, this patient highlights the classical visceral vascular malformations in this rare disorder.






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1 Department of Cardiology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India
2 Department of Radiology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

Correspondence Address:
Dr. Sreekanth Yerram
Department of Cardiology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/apc.apc_256_20

Rights and Permissions

Hereditary hemorrhagic telangiectasia (HHT) is a genetic disorder in which there is faulty development of the arteries. There is a high incidence of pulmonary hypertension (PH) in these patients, the pathophysiology of which is not fully known. An increase in cardiac output, causing high-output cardiac failure, and increased pulmonary vascular resistance secondary to genetic mutations are the main reasons. We report a 25-year-old male with HHT who presented with right heart failure secondary to PH in whom both the above mechanisms were operating. The coexistence of giant pulmonary arteriovenous malformations with severe PH is a rare scenario influencing management decisions that are discussed. In addition, this patient highlights the classical visceral vascular malformations in this rare disorder.






[FULL TEXT] [PDF]*


        
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