eComment: Congenital direct communication between the right pulmonary artery and the left atrium: anatomic variations and surgical experience

Abstract

NomenclatureHistorical Pages lumen). We would like to comment on several issues related to congenital RPA-LA communication.The first classification of anatomical variants of this pathology known by this time was based just on the character of pulmonary veins drainage w2x. The publication of new cases of RPA-LA communication led to the broadening of the spectrum of eventual anatomical variants of this defect and to the necessity of a new classification. Our classification w3x is based on the following signs typical for this anomaly: a) the anatomy of pulmonary venous drainage wnormal (type 1), partial (type 2) or total (type 3) anomalous pulmonary venous drainagex; b) the location of anomalous communication origin from the RPA wthe proximal form with the communication originating from the RPA segment between the pulmonary trunk bifurcation and the site of RPA division into the lobar branches (type 1.1) and the distal form with anomalous communication originating from a lobar branch of the RPA (type 1.2); c) the presence (type 1.1A) or the absence (type 1.1.B) of aneurysm on the communication.The priority in surgical treatment of this pathology belongs to A. Blalock, who was the first in the world (14.07.1948) to ligate the anomalous communication in a 15-year-old boy w4x.From November 1999 through November 2009, five patients with RPA-LA communication were operated on in the Bakoulev Center for Cardiovascular Surgery. The age of patients ranged from 1 month 10 days to 10 years 7 months. w(95% CI)s3.5 years (0.11; 10.6)x, there were three boys. Before the operation, SaO 2 was 71.2"11.4% (54-83). All patients had normal pulmonary venous drainage, three of them had concomitant atrial septal defects (ASD) and one had ASD and patent ductus arteriosis (PDA). The correction was performed through median sternotomy, under extracorporeal circulation (ECC). Two patients (with types 1.1A and 1.1B in our classification) underwent ligation or clipping of the anomalous communication in the proximal segment, in one patient (with 1.2B type) the anomalous communication was ligated distally. In the youngest patient (with 1.1A type), the distal orifice of the anomalous communication was sutured from the left atrial cavity with the approach through the right atrium and the ASD. Along with the anomalous communication, ASD was closed in all four cases. The only girl with intact atrial septum (IAS) and 1.2A type of the defect underwent left atriotomy with subsequent suturing of the distal fistula orifice from the left atrium. The result in four patients was good: the shunt across communication was absent, SaO 2 was 96"1.5%. In one case (with IAS) the control echo revealed a moderate residual RPA-LA. Successful endovascular procedure with Sideris button device was made six months after the first operation.

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

“…As some previous reports have noted, many cases do not have any other structural heart disease . As in our study, associated ASDs are common . Other concomitant cardiac malformations are rare, but patent ductus arteriosus, pulmonary or aortic valve regurgitation, and brain abscesses have been reported .…”

“…8 As in our study, associated ASDs are common. 16,24 Other concomitant cardiac malformations are rare, but patent ductus arteriosus, pulmonary or aortic valve regurgitation, and brain abscesses have been reported. 11 RPA-LAF was categorized into 3 types by de Souza e Silva and associates, based on the presence or absence of a fistula aneurysm and on the anatomy of the pulmonary venous drainage.…”

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

“…Cyanosis resulting from a large right-to-left shunting usually is the main feature [1]. For neonates, full and accurate description and delineation of all morphologic and hemodynamic aspects of the RPA-LA communication can be performed at the bedside exclusively using transthoracic echocardiography.…”

Surgical and Transcatheter Closure of Right Pulmonary Artery to Left Atrial Fistula

“…In 2007, Bockeria and associates proposed another classification method, based upon the anatomy of pulmonary venous drainage, the location of anomalous communication origin from the RPA, and the presence or absence of aneurysm on the communication. 8,14 This new classification might be helpful in choosing operative strategies.…”

“…To date, approximately 82 cases have been reported in the literature. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18] Given the rarity of this defect, we describe our experience with interventional and surgical closure of RPA to left atrial fistula in three cases, and make a review of literature focusing on anatomic types and the treatment outcomes.…”