Successful transcatheter treatment of large right pulmonary artery to left atrial fistula: a case series and literature review
Dublin Core
Title
Successful transcatheter treatment of large right pulmonary artery to left atrial fistula: a case series and literature review
Subject
Interventional Cardiology, Congenital heart disease, Vascular shunt, Case report
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.
Keywords Interventional Cardiology, Congenital heart disease, Vascular shunt, Case report
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.
Keywords Interventional Cardiology, Congenital heart disease, Vascular shunt, Case report
Creator
Hojjat Mortezaeian1
, Farshad Jafari1
, Mahmoud Meraji1
, Avisa Tabib2
, Hamid Reza Pouraliakbar1
and
Fatemeh Naderi3*
, Farshad Jafari1
, Mahmoud Meraji1
, Avisa Tabib2
, Hamid Reza Pouraliakbar1
and
Fatemeh Naderi3*
Source
https://doi.org/10.1186/s12245-024-00770-7
Date
2024
Contributor
Peri Irawan
Format
PDF
Language
ENGLISH
Type
TEXT
Files
Collection
Citation
Hojjat Mortezaeian1
, Farshad Jafari1
, Mahmoud Meraji1
, Avisa Tabib2
, Hamid Reza Pouraliakbar1
and
Fatemeh Naderi3*, “Successful transcatheter treatment of large right pulmonary artery to left atrial fistula: a case series and literature review,” Repository Horizon University Indonesia, accessed April 11, 2026, https://repository.horizon.ac.id/items/show/12535.