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Unusual Survival of Anomalous Left Coronary Artery From the Pulmonary Artery With Severe Rheumatic Mitral Stenosis in Septuagenarian Women: Foes Becoming Friends?

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 ALCA...

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Autores principales: Sinha, Santosh Kumar, Khanra, Dibbendhu, Jha, Mukesh Jitendra, Singh, Karandeep, Razi, Mahamdulla, Goel, Amit, Mishra, Vikas, Asif, Mohammad, Sachan, Mohit, Afdaali, Nasar, Kumar, Ashutosh, Thakur, Ramesh, Krishna, Vinay, Pandey, Umeshwar, Varma, Chandra Mohan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elmer Press 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5012248/
https://www.ncbi.nlm.nih.gov/pubmed/27635184
http://dx.doi.org/10.14740/jocmr2674w
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author Sinha, Santosh Kumar
Khanra, Dibbendhu
Jha, Mukesh Jitendra
Singh, Karandeep
Razi, Mahamdulla
Goel, Amit
Mishra, Vikas
Asif, Mohammad
Sachan, Mohit
Afdaali, Nasar
Kumar, Ashutosh
Thakur, Ramesh
Krishna, Vinay
Pandey, Umeshwar
Varma, Chandra Mohan
author_facet Sinha, Santosh Kumar
Khanra, Dibbendhu
Jha, Mukesh Jitendra
Singh, Karandeep
Razi, Mahamdulla
Goel, Amit
Mishra, Vikas
Asif, Mohammad
Sachan, Mohit
Afdaali, Nasar
Kumar, Ashutosh
Thakur, Ramesh
Krishna, Vinay
Pandey, Umeshwar
Varma, Chandra Mohan
author_sort Sinha, Santosh Kumar
collection PubMed
description 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.
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spelling pubmed-50122482016-09-15 Unusual Survival of Anomalous Left Coronary Artery From the Pulmonary Artery With Severe Rheumatic Mitral Stenosis in Septuagenarian Women: Foes Becoming Friends? Sinha, Santosh Kumar Khanra, Dibbendhu Jha, Mukesh Jitendra Singh, Karandeep Razi, Mahamdulla Goel, Amit Mishra, Vikas Asif, Mohammad Sachan, Mohit Afdaali, Nasar Kumar, Ashutosh Thakur, Ramesh Krishna, Vinay Pandey, Umeshwar Varma, Chandra Mohan J Clin Med Res Case Report 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. Elmer Press 2016-10 2016-08-30 /pmc/articles/PMC5012248/ /pubmed/27635184 http://dx.doi.org/10.14740/jocmr2674w Text en Copyright 2016, Sinha et al. http://creativecommons.org/licenses/by-nc/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Sinha, Santosh Kumar
Khanra, Dibbendhu
Jha, Mukesh Jitendra
Singh, Karandeep
Razi, Mahamdulla
Goel, Amit
Mishra, Vikas
Asif, Mohammad
Sachan, Mohit
Afdaali, Nasar
Kumar, Ashutosh
Thakur, Ramesh
Krishna, Vinay
Pandey, Umeshwar
Varma, Chandra Mohan
Unusual Survival of Anomalous Left Coronary Artery From the Pulmonary Artery With Severe Rheumatic Mitral Stenosis in Septuagenarian Women: Foes Becoming Friends?
title Unusual Survival of Anomalous Left Coronary Artery From the Pulmonary Artery With Severe Rheumatic Mitral Stenosis in Septuagenarian Women: Foes Becoming Friends?
title_full Unusual Survival of Anomalous Left Coronary Artery From the Pulmonary Artery With Severe Rheumatic Mitral Stenosis in Septuagenarian Women: Foes Becoming Friends?
title_fullStr Unusual Survival of Anomalous Left Coronary Artery From the Pulmonary Artery With Severe Rheumatic Mitral Stenosis in Septuagenarian Women: Foes Becoming Friends?
title_full_unstemmed Unusual Survival of Anomalous Left Coronary Artery From the Pulmonary Artery With Severe Rheumatic Mitral Stenosis in Septuagenarian Women: Foes Becoming Friends?
title_short Unusual Survival of Anomalous Left Coronary Artery From the Pulmonary Artery With Severe Rheumatic Mitral Stenosis in Septuagenarian Women: Foes Becoming Friends?
title_sort unusual survival of anomalous left coronary artery from the pulmonary artery with severe rheumatic mitral stenosis in septuagenarian women: foes becoming friends?
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5012248/
https://www.ncbi.nlm.nih.gov/pubmed/27635184
http://dx.doi.org/10.14740/jocmr2674w
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