Pankaj Jariwala, Venkata Nagarjuna Maturu, Johann Christopher, Kartik Pandurang Jadhav
Yashoda Hospitals. Care Hospitals.
India
Indian Journal of Thoracic and Cardiovascular Surgery
Indian J Thorac Cardiovasc Surg 2021; 37 (Suppl 1): 144-154
DOI: 10.1007/s12055-020-01032-w
Abstract
Congenital, isolated unilateral agenesis of pulmonary arteries (UAPA) has a bimodal clinical presentation. It is either diagnosed in early infancy or during adulthood. Early diagnosis and timely surgical correction at infancy prevent the development of irreversible pulmonary hypertension and enable prolonged survival. Though surgical intervention is a conclusive method of treatment, its outcome depends upon the reversibility of pulmonary hypertension and the adequacy of the pulmonary artery dimension. Adults with UAPA can be asymptomatic (diagnosed incidentally) or symptomatic. They may present clinically with myriads of symptoms like exertional dyspnea, recurrent lung infection, hemoptysis, and atypical chest pain. For cases where definitive surgical treatment is not feasible, interventional and medical management should be carried out as part of palliative therapy. A retrospective analysis of 22 cases of UAPA in adults published in the literature from the years 2017-2020 are presented here, along with our institutional case.
Category
Segmental Pulmonary Arterial Disease
Abnormal Systemic to Pulmonary Arterial Collaterals or Connections
Age Focus: Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes