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

Abstract

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...

Interventional closure of RPA-to-LA communication in an oligosymptomatic neonate

“…At our centre, we have now seen two patients with a RPAto-LA communication presenting a wide spectrum of clinical presentation. Our last case [3] presented with central cyanosis and clinical sign of respiratory distress. The patient was administered epinephrine and milrinone to stabilize her condition.…”

Transthoracic Echocardiography for Diagnosis of Right Pulmonary Artery to Left Atrial Fistula

“…17 Transcatheter occlusion has been an effective alternative treatment. [28][29][30] In conclusion, echocardiography was effective in diagnosing RPA-LAF when the appropriate views were used, and was also useful for visualization of the fistula and associated complications, and malformations for determining the type of RPA-LAF.…”

“…Simple ligation or division of the anomalous connection is generally the operative procedure of choice . Transcatheter occlusion has been an effective alternative treatment …”

Right Pulmonary Artery to Left Atrial Fistula in a Neonate

“…Ten cases of transcatheter closure of RPA-LA fistulas have been reported, 4,8,10,26 –32 four of whom have been neonates. 8,10,14,16 Potential complications of a transcatheter approach are device dislocation or migration, device erosion through the thin-walled aneurysmal fistula, incomplete closure, recanalization, and access-related complications. Slack et al 8 emphasized the importance of proper coil length selection to minimize the potential for compromise of RPA flow.…”

“…To our knowledge, there have been 17 reported cases of RPA-LA fistula diagnosed in neonates since 1976 (Table 1). 2,[5][6][7][8][9][10][11][12][13][14][15][16][17] One patient was successfully managed nonoperatively, 11 four underwent transcatheter closure, 8,10,14,16 one was still awaiting percutaneous closure, 13 one died before surgical intervention, 6 and seven (70%) of the ten neonates (including the present case) survived operative repair. 7,9,12,15,17 We report the case of a newborn infant with an RPA-LA fistula who was successfully treated by ligation and division on cardiopulmonary bypass.…”