title: Case report of isolated congenital absence of right pulmonary artery with collaterals from coronary circulation.
abstract: Isolated unilateral absence of a proximal pulmonary main artery is a rare congenital lesion which is often associated with other cardiovascular abnormalities and a diverse clinical presentation. It is usually diagnosed in childhood. Patients who survive into adulthood is uncommon. We report a case of 46 year old hypertensive and obese female who presented with progressive dyspnea. She had features of pulmonary hypertension. The diagnosis was confirmed by CT pulmonary angiography which showed absence of right pulmonary artery and conventional pulmonary angiography which showed ipsilateral lung receiving collaterals from Right coronary artery and its branches. The purpose of this report is to highlight the fact that UAPA, although a rare entity, should be kept in mind in patients with unexplained PAH and prolonged respiratory symptoms unresponsive to routine treatment modalities.
authors.affiliation: Resident, Dayanand Medical College & Hospital Unit Hero DMC Heart Institute, India.
authors.forename: Varun
authors.initials: V
authors.lastname: Mohan
authors.affiliation: Dayanand Medical College & Hospital Unit Hero DMC Heart Institute, India.
authors.forename: Bishav
authors.initials: B
authors.lastname: Mohan
authors.affiliation: Dayanand Medical College & Hospital Unit Hero DMC Heart Institute, India.
authors.forename: Rohit
authors.initials: R
authors.lastname: Tandon
authors.affiliation: Dayanand Medical College & Hospital Unit Hero DMC Heart Institute, India.
authors.forename: S
authors.initials: S
authors.lastname: Kumbkarni
authors.affiliation: Dayanand Medical College & Hospital Unit Hero DMC Heart Institute, India.
authors.forename: Shibba Takkar
authors.initials: ST
authors.lastname: Chhabra
authors.affiliation: Dayanand Medical College & Hospital Unit Hero DMC Heart Institute, India.
authors.forename: Naved
authors.initials: N
authors.lastname: Aslam
authors.affiliation: Dayanand Medical College & Hospital Unit Hero DMC Heart Institute, India.
authors.forename: Gurpreet Singh
authors.initials: GS
authors.lastname: Wander
mesh_terms: D000792:Angiography / Q000379:methods; D000925:Anticoagulants / Q000627:therapeutic use; D003097:Collateral Circulation / Q000187:drug effects; D003326:Coronary Circulation / Q000502:physiology*; D003937:Diagnosis, Differential; D004417:Dyspnea / Q000175:diagnosis / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D008875:Middle Aged; D010879:Piperazines / Q000627:therapeutic use; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000000981:diagnostic imaging; D011687:Purines / Q000627:therapeutic use; D018570:Risk Assessment; D000068677:Sildenafil Citrate; D013449:Sulfonamides / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed / Q000379:methods; D016896:Treatment Outcome; D054079:Vascular Malformations / Q000000981:diagnostic imaging*
chemical_list: D000925:Anticoagulants; D010879:Piperazines; D011687:Purines; D013449:Sulfonamides; D000068677:Sildenafil Citrate
keywords: Progressive Dyspnea; Pulmonary artery hypertension (PAH); Unilateral absence of pulmonary artery (UAPA)