Long-term results of catheter-based treatment of pulmonary atresia and intact ventricular septum

Abstract

The results confirm that percutaneous interventional perforation is an effective first-stage procedure in patients with pulmonary atresia with intact ventricular septum. The right heart appeared to be adequate to maintain a long-term biventricular circulation in the large majority of cases.

Long term follow-up post catheter intervention for critical pulmonary valve stenosis and atresia with intact ventricular septum: A 25-year single institutional experience utilizing various techniques

“…RV morphology did not independently correlate to these 2 outcomes. This is contrary to existing studies which have concluded that the TV Z score and bipartite RV morphology was predictive for univentricular palliation and the pulmonary valve Z score was not a dependent variable of ventricular outcome [1,2].…”

“…Typical associated conditions are significant tricuspid insufficiency, variable hypoplasia of the right heart structures, right ventricular hypertrophy, right ventricular dysfunction and obligatory right to left shunting at the atrial level. Prior studies have demonstrated that a bipartite right ventricle (RV) and a tricuspid valve (TV) annulus Z score  -3 are associated with the need for supplemental blood flow to the pulmonary arteries via a surgical Blalock Taussig (BT shunt) and a trend toward subsequent univentricular palliation [1,2] as the RV is unable to support systemic cardiac output. Univentricular palliation includes a second stage bidirectional Glenn anastomosis and finally an intra or extracardiac Fontan palliation procedure.…”

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

“…A number of cutoff points have been suggested in other publications, including a TV annulus of at least 11 mm (19) or a z-score of at least Ϫ3.5 (14,17). In our study, these values appear extremely conservative, but it is important to bear in mind the algorithm with which a z-score is calculated.…”

“…In our study, these values appear extremely conservative, but it is important to bear in mind the algorithm with which a z-score is calculated. Our study has chosen to use the algorithms calculated from the data of Daubeney et al (30), in common with the majority of comparable publications (5,14,16,34,36). However, some studies have used algorithms calculated from autopsy measurements (18,39) or have not made explicit which set of algorithms have been used.…”

“…As an alternative, our institution performed the first catheter pulmonary valvotomy in the world for PAIVS in 1990 (10,11), and the technique is now the mainstay of treatment in many centers for patients with favorable anatomy (12)(13)(14)(15)(16)(17)(18)(19)(20).The technique has evolved, and stenting of the arterial duct has been added as a source of additional pulmonary blood flow in selected patients while RV function is being established (21)(22)(23)(24)(25)(26)(27)(28). However, there remains a lack of consensus regarding the best treatment pathway for those patients with borderline anatomy (15,29).…”