Treatment of Infants with Pulmonary Atresia and Intact Ventricular Septum: Long-term Follow-up

Abstract

Cardiology| July 01 2003 Treatment of Infants with Pulmonary Atresia and Intact Ventricular Septum: Long-term Follow-up AAP Grand Rounds (2003) 10 (1): 5–6. https://doi.org/10.1542/gr.10-1-5-a Views Icon Views Article contents Figures & tables Video Audio Supplementary Data Peer Review Share Icon Share Facebook Twitter LinkedIn MailTo Tools Icon Tools Get Permissions Cite Icon Cite Search Site Citation Treatment of Infants with Pulmonary Atresia and Intact Ventricular Septum: Long-term Follow-up. AAP Grand Rounds July 2003; 10 (1): 5–6. https://doi.org/10.1542/gr.10-1-5-a Download citation file: Ris (Zotero) Reference Manager EasyBib Bookends Mendeley Papers EndNote RefWorks BibTex toolbar search toolbar search search input Search input auto suggest filter your search All PublicationsAll JournalsAAP Grand RoundsPediatricsHospital PediatricsPediatrics In ReviewNeoReviewsAAP NewsAll AAP Sites Search Advanced Search Topics: interventricular septum, pulmonary atresia, pulmonary valve Source: Agnoletti G, Pichaud JF, Bonhoeffer P, et al. Perforation Agnoletti G, Pichaud JF, Bonhoeffer P, et al. Perforation of the atretic pulmonary valve. Long-term follow-up. J Am Coll Cardiol. 2003;41:1399–1403. In many centers, catheter perforation of the atretic pulmonary valve followed by balloon pulmonary valvuloplasty has become the initial palliative approach for babies born with pulmonary atresia and intact ventricular septum (PAIVS). This report represents the accumulated experience of a group in Paris, France with 39 selected neonates with PAIVS over 11 years (1991–2001). They excluded cases with complicating features known to affect the short- and long-term course of PAIVS that were independent of the quality of relief of valve obstruction. Thirty-nine neonates in this very favorable subset of infants born with PAIVS underwent attempted catheter perforation of the pulmonary valve, and 33 procedures were deemed acutely successful (85%). There were 2 periprocedural deaths (5%), both attributed to catheter perforation of the right ventricle. Four others required urgent surgical attention after the catheter-mediated approach failed. Periprocedural complications were frequent, and included 3 cases each of atrial flutter and necrotizing enterocolitis. Mean total follow-up was 5.5 years among the 33 acute successes. Seventeen (52%) required surgical revision in the neonatal period. Three others had elective surgery later, yielding an actuarial freedom from operation of 35% at 400 days of postnatal life. Multiple operations were required in 9 patients, and further surgery was planned for several others. Although many patients had hemodynamic abnormalities at long-term follow-up, including tricuspid and pulmonary valve regurgitation, right-to-left atrial shunts, and partial cavopulmonary circulation, all surviving patients except 1 were reported to be in New York Heart Association functional class I. The authors conclude that the mortality and morbidity of this procedure is “non-negligible,” and that it offers hope of a satisfactory outcome for a subset of neonates with PAIVS, provided that the selection criteria for patients undergoing the procedure are sufficiently strict. In the neonate, PAIVS presents 2 basic problems. First, the only blood reaching the pulmonary artery arrives through the ductus arteriosus, which is soon to close. Second, considerable cyanosis results from right-to-left atrial shunting due to ineffective forward flow through the right heart. Treatment of PAIVS must, therefore, have 2 objectives. It must supply enough pulmonary blood flow so that the ductus arteriosus is no longer required, and it must promote sufficient right heart flow so that the right-to-left intracardiac shunting resolves. One is struck by how difficult it has been in the past to meet these objectives without multiple interventions and considerable mortality and morbidity.1,2 In the same way, we might initially be tempted to be too critical of the current report in which rates of acute mortality, acute failure, periprocedural complication, and necessity for further neonatal surgery are dismayingly high. We must, it seems, continually remind ourselves that PAIVS is a spectrum of diffuse, hypoplastic right heart disease that involves, with greater or lesser severity, the tricuspid annulus and valve; the right ventricular inlet,... You do not currently have access to this content.