title: Unusual Survival of Anomalous Left Coronary Artery From the Pulmonary Artery With Severe Rheumatic Mitral Stenosis in Septuagenarian Women: Foes Becoming Friends?
abstract: ALCAPA syndrome (anomalous origin of the left coronary artery from the pulmonary artery) is a rare disease but lethal with clinical expression from myocardial infarction, congestive heart failure to death during early infancy and unusual survival to adulthood. We report a 73-year-old woman with ALCAPA who presented with exertional dyspnea (NYHA functional class II) over past 2 years. Physical examination revealed soft S, long mid diastolic rumbling murmur and apical pan-systolic murmur. Electrocardiography displayed biatrial enlargement and poor R progression and normal sinus rhythm. Echocardiography established calcified severe mitral stenosis (MS), presence of continuous flow entering the pulmonary trunk, turbulent continuous flow in inter-ventricular septum with left to right shunt in contrast echocardiography and normal systolic function. Coronary angiogram showed absence of left coronary artery (LCA) originating from aorta, dilated and tortuous right coronary artery (RCA) and abundant Rentrop grade 3 intercoronary collateral communicating with LCA originating from pulmonary trunk which was also confirmed on coronary CT angiogram thus establishing diagnosis of ALCAPA. It is exceedingly rare to be associated with severe MS. However, such a long survival in our patient can be explained by the severe pulmonary arterial hypertension which may be contributing to lesser coronary steal.
authors.affiliation: Department of Cardiology, LPS Institute of Cardiology, G.S.V.M. Medical College, Kanpur 208002, Uttar Pradesh, India.
authors.forename: Santosh Kumar
authors.initials: SK
authors.lastname: Sinha
authors.affiliation: Department of Cardiology, LPS Institute of Cardiology, G.S.V.M. Medical College, Kanpur 208002, Uttar Pradesh, India.
authors.forename: Dibbendhu
authors.initials: D
authors.lastname: Khanra
authors.affiliation: Department of Cardiology, LPS Institute of Cardiology, G.S.V.M. Medical College, Kanpur 208002, Uttar Pradesh, India.
authors.forename: Mukesh Jitendra
authors.initials: MJ
authors.lastname: Jha
authors.affiliation: Department of Cardiology, LPS Institute of Cardiology, G.S.V.M. Medical College, Kanpur 208002, Uttar Pradesh, India.
authors.forename: Karandeep
authors.initials: K
authors.lastname: Singh
authors.affiliation: Department of Cardiology, LPS Institute of Cardiology, G.S.V.M. Medical College, Kanpur 208002, Uttar Pradesh, India.
authors.forename: Mahamdulla
authors.initials: M
authors.lastname: Razi
authors.affiliation: Department of Cardiology, LPS Institute of Cardiology, G.S.V.M. Medical College, Kanpur 208002, Uttar Pradesh, India.
authors.forename: Amit
authors.initials: A
authors.lastname: Goel
authors.affiliation: Department of Cardiology, LPS Institute of Cardiology, G.S.V.M. Medical College, Kanpur 208002, Uttar Pradesh, India.
authors.forename: Vikas
authors.initials: V
authors.lastname: Mishra
authors.affiliation: Department of Cardiology, LPS Institute of Cardiology, G.S.V.M. Medical College, Kanpur 208002, Uttar Pradesh, India.
authors.forename: Mohammad
authors.initials: M
authors.lastname: Asif
authors.affiliation: Department of Cardiology, LPS Institute of Cardiology, G.S.V.M. Medical College, Kanpur 208002, Uttar Pradesh, India.
authors.forename: Mohit
authors.initials: M
authors.lastname: Sachan
authors.affiliation: Department of Cardiology, LPS Institute of Cardiology, G.S.V.M. Medical College, Kanpur 208002, Uttar Pradesh, India.
authors.forename: Nasar
authors.initials: N
authors.lastname: Afdaali
authors.affiliation: Department of Cardiology, LPS Institute of Cardiology, G.S.V.M. Medical College, Kanpur 208002, Uttar Pradesh, India.
authors.forename: Ashutosh
authors.initials: A
authors.lastname: Kumar
authors.affiliation: Department of Cardiology, LPS Institute of Cardiology, G.S.V.M. Medical College, Kanpur 208002, Uttar Pradesh, India.
authors.forename: Ramesh
authors.initials: R
authors.lastname: Thakur
authors.affiliation: Department of Cardiology, LPS Institute of Cardiology, G.S.V.M. Medical College, Kanpur 208002, Uttar Pradesh, India.
authors.forename: Vinay
authors.initials: V
authors.lastname: Krishna
authors.affiliation: Department of Cardiology, LPS Institute of Cardiology, G.S.V.M. Medical College, Kanpur 208002, Uttar Pradesh, India.
authors.forename: Umeshwar
authors.initials: U
authors.lastname: Pandey
authors.affiliation: Department of Cardiology, LPS Institute of Cardiology, G.S.V.M. Medical College, Kanpur 208002, Uttar Pradesh, India.
authors.forename: Chandra Mohan
authors.initials: CM
authors.lastname: Varma
keywords: ALCAPA syndrome; Contrast echocardiography; Coronary CT; Mitral stenosis; NYHA functional class; Rentrop collateral