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

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


Sunitha Aramalla1, Srinivas Bhyravavajhala1, Bharathi Vanaparty1, Ramakrishna Narayanan2, Sreekanth Yerram1 
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

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.


How to cite this article:
Aramalla S, Bhyravavajhala S, Vanaparty B, Narayanan R, Yerram S. Severe pulmonary arterial hypertension in a patient with hereditary hemorrhagic telangiectasia and multiple pulmonary and hepatic arteriovenous malformations.Ann Pediatr Card 2022;15:73-76


How to cite this URL:
Aramalla S, Bhyravavajhala S, Vanaparty B, Narayanan R, Yerram S. Severe pulmonary arterial hypertension in a patient with hereditary hemorrhagic telangiectasia and multiple pulmonary and hepatic arteriovenous malformations. Ann Pediatr Card [serial online] 2022 [cited 2022 Aug 20 ];15:73-76
Available from: https://www.annalspc.com/article.asp?issn=0974-2069;year=2022;volume=15;issue=1;spage=73;epage=76;aulast=Aramalla;type=0