Successful transcatheter treatment of large right pulmonary artery to left atrial fistula: a case series and literature review

Abstract Introduction Cyanotic congenital heart diseases are among the most serious anomalies among newborns. A rare type of this condition is direct communication between the right pulmonary artery and left atrium, which presents mostly in adolescence and adulthood. Large shunts, however, should be...

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Main Authors: Hojjat Mortezaeian, Farshad Jafari, Mahmoud Meraji, Avisa Tabib, Hamid Reza Pouraliakbar, Fatemeh Naderi
Format: Article
Language:English
Published: BMC 2024-11-01
Series:International Journal of Emergency Medicine
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Online Access:https://doi.org/10.1186/s12245-024-00770-7
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author Hojjat Mortezaeian
Farshad Jafari
Mahmoud Meraji
Avisa Tabib
Hamid Reza Pouraliakbar
Fatemeh Naderi
author_facet Hojjat Mortezaeian
Farshad Jafari
Mahmoud Meraji
Avisa Tabib
Hamid Reza Pouraliakbar
Fatemeh Naderi
author_sort Hojjat Mortezaeian
collection DOAJ
description Abstract Introduction Cyanotic congenital heart diseases are among the most serious anomalies among newborns. A rare type of this condition is direct communication between the right pulmonary artery and left atrium, which presents mostly in adolescence and adulthood. Large shunts, however, should be corrected as soon as possible, considering their potential to cause congestive heart failure. Case Presentation Two 2- and 13-year-old patients with this pathologic communication complained of exertional dyspnea and central cyanosis. Their physical exams were prominent, with a low oxygen saturation level. The diagnostic modalities used, electrocardiogram, chest X-ray, echocardiogram, CT scan, and selective angiography of the pulmonary arteries, showed right-to-left abnormal blood flow through the right pulmonary artery and left atrium shunt. Finally, both patients were treated successfully by a transcatheter occluder without any complications or follow-up complaints. Discussion The right pulmonary artery and left atrium abnormal congenital connections are rare causes of central cyanosis, mostly present with exertional dyspnea and cyanosis during adolescence or early adulthood. Transthoracic echocardiography, contrast-enhanced CT scans, and angiography of the pulmonary arteries make the diagnosis. The treatment has emerged during the last two decades, shifting from surgical treatment for severe cases to interventional percutaneous strategies, leaving the surgery for cases with no appropriate location for the application of interventional therapy. Conclusion Considering the potentially life-threatening complications of the untreated right pulmonary artery and left atrium fistulas, such as thromboembolic events, early diagnosis is crucial. However, treating these patients is not always straightforward, and strategies should be selected based on the connecting fistula’s anatomical features, location, size, and tortuosity. Although endovascular treatment is the preferred option, some patients need to be treated with surgical procedures due to their structural characteristics.
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spelling doaj-art-64e7db3e3b1341cd919ce48f1baf29752024-12-01T12:08:23ZengBMCInternational Journal of Emergency Medicine1865-13802024-11-011711710.1186/s12245-024-00770-7Successful transcatheter treatment of large right pulmonary artery to left atrial fistula: a case series and literature reviewHojjat Mortezaeian0Farshad Jafari1Mahmoud Meraji2Avisa Tabib3Hamid Reza Pouraliakbar4Fatemeh Naderi5Rajaie Cardiovascular Medical & Research Center, Iran University of Medical SciencesRajaie Cardiovascular Medical & Research Center, Iran University of Medical SciencesRajaie Cardiovascular Medical & Research Center, Iran University of Medical SciencesHeart Valve Disease Research Center, Rajaie Cardiovascular Medical and Research Center, Iran University of Medical SciencesRajaie Cardiovascular Medical & Research Center, Iran University of Medical SciencesRajaei Cardiovascular Medical and Research Center, School of Medicine, Iran University of Medical SciencesAbstract Introduction Cyanotic congenital heart diseases are among the most serious anomalies among newborns. A rare type of this condition is direct communication between the right pulmonary artery and left atrium, which presents mostly in adolescence and adulthood. Large shunts, however, should be corrected as soon as possible, considering their potential to cause congestive heart failure. Case Presentation Two 2- and 13-year-old patients with this pathologic communication complained of exertional dyspnea and central cyanosis. Their physical exams were prominent, with a low oxygen saturation level. The diagnostic modalities used, electrocardiogram, chest X-ray, echocardiogram, CT scan, and selective angiography of the pulmonary arteries, showed right-to-left abnormal blood flow through the right pulmonary artery and left atrium shunt. Finally, both patients were treated successfully by a transcatheter occluder without any complications or follow-up complaints. Discussion The right pulmonary artery and left atrium abnormal congenital connections are rare causes of central cyanosis, mostly present with exertional dyspnea and cyanosis during adolescence or early adulthood. Transthoracic echocardiography, contrast-enhanced CT scans, and angiography of the pulmonary arteries make the diagnosis. The treatment has emerged during the last two decades, shifting from surgical treatment for severe cases to interventional percutaneous strategies, leaving the surgery for cases with no appropriate location for the application of interventional therapy. Conclusion Considering the potentially life-threatening complications of the untreated right pulmonary artery and left atrium fistulas, such as thromboembolic events, early diagnosis is crucial. However, treating these patients is not always straightforward, and strategies should be selected based on the connecting fistula’s anatomical features, location, size, and tortuosity. Although endovascular treatment is the preferred option, some patients need to be treated with surgical procedures due to their structural characteristics.https://doi.org/10.1186/s12245-024-00770-7Interventional CardiologyCongenital heart diseaseVascular shuntCase report
spellingShingle Hojjat Mortezaeian
Farshad Jafari
Mahmoud Meraji
Avisa Tabib
Hamid Reza Pouraliakbar
Fatemeh Naderi
Successful transcatheter treatment of large right pulmonary artery to left atrial fistula: a case series and literature review
International Journal of Emergency Medicine
Interventional Cardiology
Congenital heart disease
Vascular shunt
Case report
title Successful transcatheter treatment of large right pulmonary artery to left atrial fistula: a case series and literature review
title_full Successful transcatheter treatment of large right pulmonary artery to left atrial fistula: a case series and literature review
title_fullStr Successful transcatheter treatment of large right pulmonary artery to left atrial fistula: a case series and literature review
title_full_unstemmed Successful transcatheter treatment of large right pulmonary artery to left atrial fistula: a case series and literature review
title_short Successful transcatheter treatment of large right pulmonary artery to left atrial fistula: a case series and literature review
title_sort successful transcatheter treatment of large right pulmonary artery to left atrial fistula a case series and literature review
topic Interventional Cardiology
Congenital heart disease
Vascular shunt
Case report
url https://doi.org/10.1186/s12245-024-00770-7
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