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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...
Autores principales: | , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elmer Press
2016
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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. |
format | Online Article Text |
id | pubmed-5012248 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | Elmer Press |
record_format | MEDLINE/PubMed |
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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