Right Pulmonary Artery to Left Atrium Communication

Chowdhury, Ujjwal K.; NB, Diplomate; Kothari, Shyam Sunder; Airan, Balram; Subramaniam, Kathirvel; Venugopal, P

doi:10.1016/j.athoracsur.2004.08.058

Cited by 36 publications

(75 citation statements)

References 30 publications

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“…At histologic examination, the sac showed an inner layer of endothelial cells and elastin tissues and an outer layer of muscular tissue, resembling more a vessel than a typical arteriovenous fistula [2]. In our patient, drainage of the pulmonary veins was normal without aneurysm formation as seen in the most common type 1 disorder.…”

Section: Discussionmentioning

confidence: 72%

“…The spectrum of RPA-LA communication includes a heterogeneous group of cases with potentially different embryonic development [2]. It is hypothesized that the lesion results from a fistula that forms during early embryonic development between the pulmonary artery and one of the main pulmonary veins, which later becomes incorporated into the wall of the LA during atrial enlargement.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Catheter Interventional Closure of a Large Right Pulmonary Artery–to–Left Atrial Communication in a Neonate

Burkamp

Ghisla²,

Knirsch

et al. 2012

Pediatr Cardiol

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Right pulmonary artery-to-left atrial communication is a rare congenital vascular malformation that results in a right-to-left shunting. This report describes the case history of a neonate with a large right pulmonary artery-to-left atrial connection resulting in cyanosis and severe heart failure who underwent successful early catheter interventional therapy. In the neonate, this lesion can be diagnosed accurately using transthoracic echocardiography. Closure of the communication can be achieved even in infants via percutaneous interventional catheterization with a low procedure-related risk and a good midterm follow-up result.Keywords Catheter interventional therapy Á Congenital heart defect Á Pulmonary artery-to-left atrium communication Á Neonate One-third of neonatal cyanosis cases are caused by congenital heart defects and it is important to differentiate them from pulmonary disease or persistent fetal circulation [5]. Right pulmonary artery (RPA)-to-left atrium (LA) communication involves a direct connection between the pulmonary artery and the pulmonary vein or LA [11] and is a very uncommon cause of right-to-left shunting. This pathologic vascular communication results in a large shunting of venous blood to the arterial side, leading to both cyanosis and volume overload. Case ReportA female term neonate with a birth weight of 3 kg and 620 g presented 14 h postnatally with central cyanosis and clinical signs of respiratory distress. An echocardiogram showed a large RPA-LA communication from the proximal RPA to the roof of the LA (Fig. 1). The LA and the left ventricle were dilated, and left ventricular function was impaired (ejection fraction, 33%).The patient's condition improved and stabilized after initiation of continuous epinephrine and milrinone administered intravenously. After 6 days, cardiac catheterization was performed with the patient under general anesthesia via a transfemoral venous approach using a 5-Fr introducer sheath. The size and anatomy of the fistula were identified and measured by a selective pulmonary angiogram (Fig. 2).Due to the shunt volume, the diameter of the proximal RPA was double that of the left pulmonary artery. The fistula itself measured 9-mm at the pulmonary end, narrowed to 2.2-mm, and widened again to 9-mm at the atrial end at the roof of the LA.The communication was entered from the pulmonary arterial side with an 0.018 in. wire. Through the 5-Fr sheath and across the wire, a flexible introducer catheter was advanced into the fistula. Successful closure then was performed with a detachable patent arterial duct (PDA) coil (7 9 6-mm, Nit-Occlud; pfm, Cologne, Germany) from the pulmonary arterial side. Three loops of the coil were placed into the LA, and the remaining loops were placed into the fistula and the RPA.After confirmation that the positioning of the device was stable and the flow of the RPA and LA was unimpaired (via a contrast injection through the introducer catheter and a transthoracic echocardiography), the coil was released. A final angiogram s...

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Section: Discussionmentioning

confidence: 72%

Section: Discussionmentioning

confidence: 99%

Catheter Interventional Closure of a Large Right Pulmonary Artery–to–Left Atrial Communication in a Neonate

Burkamp

Ghisla²,

Knirsch

et al. 2012

Pediatr Cardiol

View full text Add to dashboard Cite

show abstract

“…Although transcatheter embolization would be accepted better treatment for patient with arteriovenous malformation, the significant number of complications outweigh the open heart surgery thus, a majority of PALAF cases have undergone surgical repair [6]. Elective surgery is the best treatment to prevent chronic arterial hypoxemia, systemic thromboembolism and pulmonary hypertension.…”

Section: Discussionmentioning

confidence: 99%

An uncommon variant of cyanotic congenital heart disease in a young adult female: a rare case of right pulmonary artery to left atrial fistula (PALAF)

et al. 2015

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BackgroundCyanotic congenital heart disease is not a rare entity, but fistula between the right pulmonary artery and the left atrium is an uncommon vascular anomaly. Although it is a real challenge to diagnose the case, detailed clinical evaluation and selective investigations are keys for diagnosis, and surgical intervention is still considered the best treatment option.Case presentationA 19 years old girl from the remote village of Nepal presented with the history of exercise intolerance associated with cyanosis and clubbing of the extremities. We diagnosed her as a case of right pulmonary artery to left atrial fistula, a rare variant of pulmonary arteriovenous malformation. She underwent successful surgical correction of the anomaly under cardiopulmonary bypass surgery.ConclusionDirect communication between the right pulmonary artery and the left atrium is a rare cyanotic congenital heart disease, which is diagnosed late and often associated with the atrial septal defect. The best treatment available is surgical correction.

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“…Extracorporeal circulation has been applied in 52.5% of reported patients, and 13.5% of patients do not undergo surgery. 3 If left untreated, cerebral or systemic embolization or cerebral abscess, or other complications such as endarteritis, infective endocarditis, and aneurysm rupture 1,3 might develop. The surgical strategy should be individualized and performed as soon as the condition is diagnosed.…”

Section: Case Reportmentioning

confidence: 99%

“…3 The condition is diffi cult to diagnose because patients present without a heart murmur, 3-5 the reasons for which remain unknown. In our patient, the pressure gradient of the communication was not high, and the communication had no stenotic regions.…”

Section: Case Reportmentioning

confidence: 99%

Communication between the right pulmonary artery and left atrium with aneurysm formation

Chikada

Murakami

Takeuchi

et al. 2008

Gen Thorac Cardiovasc Surg

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Communication between the pulmonary artery and the left atrium is a rare congenital anomaly. A 7-year-old boy was referred to our hospital with a diagnosis of pulmonary arteriovenous fistula. Referral to a pediatric cardiologist had been delayed because he had only slight cyanosis and no heart murmur. Catheterization and computed tomography scans confirmed that the right pulmonary branches and pulmonary venous returns were normal and that an additional aneurysmal communication was located between the right pulmonary artery and the left atrium. Surgery proceeded uneventfully together with a cardiopulmonary bypass. The connection from the right pulmonary artery to the aneurysm was ligated, and the connection from the left atrium to the aneurysm was closed with a polytetrafluoroethylene patch. We describe this rare anomaly with cyanosis and no heart murmur.

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Right Pulmonary Artery to Left Atrium Communication

Cited by 36 publications

References 30 publications

Catheter Interventional Closure of a Large Right Pulmonary Artery–to–Left Atrial Communication in a Neonate

Catheter Interventional Closure of a Large Right Pulmonary Artery–to–Left Atrial Communication in a Neonate

An uncommon variant of cyanotic congenital heart disease in a young adult female: a rare case of right pulmonary artery to left atrial fistula (PALAF)

Communication between the right pulmonary artery and left atrium with aneurysm formation

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