Surgical strategy for pulmonary atresia with intact ventricular septum: initial management and definitive surgery

Abstract

Pulmonary atresia with intact ventricular septum is difficult to manage, and the surgical procedures are associated with high mortality and morbidity. The lesion is morphologically heterogeneous, with varying degrees of right ventricular and tricuspid valve hypoplasia. Aberrations of the coronary circulation are common. A single approach is impractical because the wide spectrum of right heart morphology makes a uniform surgical approach impossible. Anatomical criteria, such as the degree of tricuspid valve or right ventricular hypoplasia or whether a right ventricular infundibulum is present, have been used to assign the infants to single-ventricular or biventricular repair tracks. If the patient is considered a candidate for biventricular repair, right ventricular decompression, either alone or combined with a systemic-pulmonary artery shunt, is performed, whereas only a systemic-pulmonary artery shunt is performed in a candidate for single-ventricular repair. Several authors reported that the Z-value of the tricuspid valve diameter could be a guide to the initial procedure; however, poor interobserver reproducibility and wide normal ranges are serious limitations. Definitive operations, including biventricular repair, one-and-a-half ventricular repair, or Fontan-type operation, are performed to eliminate cyanosis. The most important problem when planning a strategy for a definitive repair is the fact that right ventricular growth and its contribution to pulmonary flow is difficult to predict before the operation. The management protocol should be determined by the precisely estimated morphology of the right ventricle and tricuspid valve, and the adequacy of the protocol should be analyzed based on surgical outcomes.

Long-Term Outcome Following Catheter Valvotomy for Pulmonary Atresia With Intact Ventricular Septum

“…Following successful perforation of the PV, those with a smaller RV tend to require more interventions in their treatment pathway. This staged approach should not be seen as a failure of the initial procedure, but rather as the tailoring of treatment to fit such a heterogeneous group (26,29,43). It is The Values are median (range), mean ϮSD, or n (%).…”

Aortic perfusion score for pulmonary atresia with intact ventricular septum: An antegrade coronary perfusion scoring system that is predictive of need for transplant and mortality

“…While these management strategies have continued to improve, there are still no established guidelines by which an appropriate strategy may be selected. Patients with PAIVS may undergo a single ventricle repair, one and a half ventricle repair, two ventricle repair or cardiac transplantation, many of which can be achieved through a variety of surgical and interventional techniques …”

“…Patients with PAIVS may undergo a single ventricle repair, one and a half ventricle repair, two ventricle repair or cardiac transplantation, many of which can be achieved through a variety of surgical and interventional techniques. 6,7 Despite advances in the selection of management strategy, reported mortality remains high. Survival of 60% to 85% has been reported at 1 year, 50% to 81%, at 5 years and 60 to 81% at 10 years, although lower survival was noted largely in patients born in earlier decades.…”

Multimodality cardiac imaging of a double chambered right ventricle with intrapulmonary shunting: a case report

“…Pulmonary atresia with an intact ventricular septum (PAIVS) is a spectrum of duct-dependent cyanotic congenital lesions, which include atresia of the pulmonary valve, varying degrees of right ventricular and tricuspid valve (TV) hypoplasia, along with anomalies of the coronary circulation [2]. Surgical treatment for PAIVS commonly occurs in two stages.…”

“…The first stage consists of transpulmonary valvotomy with possible creation of a systemic-pulmonary artery shunt to relieve the duct-dependent circulation, promote forward blood flow and allow for growth of the hypoplastic RV. The second and often definitive repair stage, consists of an anatomic biventricular repair, a one-and-a-half ventricular repair or a Fontan-type procedure depending on the degree of residual hypoplasia of the RV and TV [2]. Our patient underwent repair of his PAIVS and hypoplastic RV with a closed pulmonic valvotomy and creation of central AP shunt, followed by a definitive one-and-a-half ventricular repair consisting of a bidirectional superior vena cava-pulmonary artery (Glenn) shunt, ASD closure and central AP shunt closure.…”