Key result
Transcatheter closure of RPA-LA fistula normalizes hypoxemia and resolves cyanosis.
Why the study?
Does direct antegrade transcatheter closure using a 16 mm Amplatzer Muscular VSD Occluder safely and effectively resolve right-to-left shunting and cyanosis in a pediatric patient with a right pulmonary artery to left atrium fistula?
Case Report (n=1)
No
Does direct antegrade transcatheter closure using a 16 mm Amplatzer Muscular VSD Occluder safely and effectively resolve right-to-left shunting and cyanosis in a pediatric patient with a right pulmonary artery to left atrium fistula?
This case demonstrates that a right pulmonary artery to left atrium fistula—a rare cause of unexplained cyanosis with a normal standard echocardiogram—can be definitively diagnosed via bubble contrast echocardiography and CTPA, and safely closed via a simplified direct antegrade transcatheter approach without needing a veno-venous loop or transseptal puncture.
Answers are grounded in this paper and cite their sources.
May extend minimally invasive options for rare pediatric RPA-LA fistulas; hypothesis-generating and needs validation in larger series.
Case DesCriptionA 9-year-old boy presented with a history of persistent cyanosis, fatigue, and severe failure to thrive.The cyanosis was initially noted via pulse oximetry monitoring during the COVID-19 pandemic and had been progressively worsening.The patient's daily activities were markedly restricted due to New York Heart Association (NYHA) class II dyspnea over the preceding 2 years.Interestingly, multiple transthoracic echocardiograms performed at outside facilities during this period were reported as normal.Upon physical examination, the child appeared severely underweight, with a weight of 21.1 kg (<3rd percentile) and a height of 127 cm (<25th percentile).His systemic oxygen saturation (SpO₂) on room air was 68%, accompanied by a heart rate of 94 beats/min and a respiratory rate of 42 breaths/ min.The baseline clinical examination, chest radiograph, and electrocardiogram (ECG) were otherwise unremarkable (Figs 1 and 2).A repeat transthoracic echocardiogram revealed no structural intracardiac anomalies or shunts.Pulmonary artery systolic pressure was within normal limits, while the left atrium and left ventricle demonstrated mild dilation.A saline bubble contrast echocardiogram using agitated normal saline revealed dense opacification of the leftsided cardiac chambers after three cardiac cycles, highly suggestive of a large extracardiac right-to-left shunt (Fig. 3).To optimize preprocedural planning, a contrast-enhanced computed tomography pulmonary angiogram (CTPA) with volume rendering technique (VRT) reconstructions was performed (Figs 4 and 5).The multiplanar imaging provided a definitive, confirmatory diagnosis of a right pulmonary artery to left atrium (RPA-LA) fistula.Beyond diagnostic confirmation, the VRT reconstructions allowed for a rigorous, noninvasive assessment of the true dimensions of the fistulous tract, its exact morphology, and its spatial relationship to adjacent cardiac structures.
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Lakshmivenkateshiah et al. (2026) conducted a case report in Right pulmonary artery to left atrium (RPA-LA) fistula causing persistent cyanosis and severe failure to thrive (n=1). Antegrade transcatheter device closure using a 16 mm double-disk Amplatzer Muscular VSD Occluder was evaluated on Complete fistula occlusion, systemic oxygen saturation (SpO2), and clinical catch-up growth at 6 months. Antegrade transcatheter closure with a 16 mm Amplatzer Muscular VSD Occluder achieved complete RPA-LA fistula exclusion, normalizing SpO2 from 68% to 98-100% and resulting in 4.5 kg weight gain at 6 months.
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