Pulmonary atresia with intact ventricular septum: is neonatal repair advisable?

Abstract

The optimal management of pulmonary atresia with an intact ventricular septum in the neonate remains controversial. The introduction of balloon septostomy and prostaglandin has significantly reduced early mortality but early surgical intervention is necessary to obtain a more adequate pulmonary blood flow. Fourteen neonates with pulmonary atresia and an intact ventricular septum were admitted to the Wessex Cardiothoracic Unit, Southampton from 1979 to 1986. Thirteen patients underwent cardiac catheterization. Cardiac catheterization data and right ventricular angiograms were reviewed retrospectively. Four patients with tripartite ventricles underwent total repair. The others received various palliative operations (valvotomy + modified Blalock-Taussig shunt or modified Blalock-Taussig shunt alone). Retrospective analysis of the angiograms indicated that right ventricular morphology alone is not a satisfactory criterion for surgical management. We have been able to demonstrate that there is a good correlation between the diameter of the tricuspid valve and the diameter of the infundibulum and that successful neonatal repair is possible when the tricuspid valve diameter is above 80% of the normal value for weight and when the tricuspid valve diameter to infundibular diameter ratio (TV/Inf ratio) is 2.2 or less. In patients with a tripartite ventricle but inadequate TV diameter and TV/Inf ratio, a closed pulmonary valvotomy with a modified Blalock-Taussig shunt remains the treatment of choice.

Pulmonary atresia with intact ventricular septum: Predictors of early and medium-term outcome in a population-based study

“…[13][14][15][16] Numerous strategies have been proposed, often based on novel classifications of the RV or coronary arteries. 8,10,12,14,15,[18][19][20][21][22][25][26][27][28] De Leval and Bull and coworkers 17,25 based their strategy on the so-called partite classification of the RV, Hanley and colleagues 10 on the size of the TV, Pawade and associates 15 on the size of the infundibulum, and Giglia and coworkers 19 on the presence of coronary artery stenoses. Because of its rarity, many studies of PAIVS contain relatively few patients but are contemporary; others achieve larger numbers by spanning many years, making them essentially historical.…”

“…guide to initial management. 10,12,14,15,[17][18][19][20][21][22] One of the largest studies proposed a management algorithm dependent on the tricuspid valve (TV) z score on presentation. 10 The aim of the current study is to clarify which presenting clinical and morphologic features play a role in determining early and medium-term outcome and, in particular, which are risk factors for poor outcome.…”

Morphology of Tricuspid Valve in Pulmonary Atresia with Intact Ventricular Septum

“…1): One prototype is a more frequent type, with pulmonary valvular atresia, dysplasia of the tricuspid valve (TV) with a small annulus, underdeveloped RV with a hypoplastic cavity and a hypertrophic wall, atrial septal defect, and patent ductus arteriosus; the other, less frequent type has more severe dysplasia of TV and dilatation of the RV, right atrium (RA), and right atrioventricular junction, with marked thinning of the RV wall. Although the clinical, angiocardiographic, and pathologic profile of the patient with PAIVS is well established [2,19,26,28], the treatment of these infants, who are often critically ill, continues to pose a considerable challenge [1,9,23,30]. Because the majority of these patients have gross underdevelopment of the RV and inadequate pulmonary bloodflow, surgical intervention is directed toward augmenting pulmonary bloodflow and allowing the potential for right ventricular growth by establishing continuity between the RV and the pulmonary artery, and by the construction of a systemic to pulmonary artery anastomosis [1,9,10,19,22,23,25].…”

“…Although the clinical, angiocardiographic, and pathologic profile of the patient with PAIVS is well established [2,19,26,28], the treatment of these infants, who are often critically ill, continues to pose a considerable challenge [1,9,23,30]. Because the majority of these patients have gross underdevelopment of the RV and inadequate pulmonary bloodflow, surgical intervention is directed toward augmenting pulmonary bloodflow and allowing the potential for right ventricular growth by establishing continuity between the RV and the pulmonary artery, and by the construction of a systemic to pulmonary artery anastomosis [1,9,10,19,22,23,25]. However, too frequently, adequate continuity between the RV and pulmonary artery is not accomplished, and adequate growth of the RV is seldom achieved, although such growth has been documented in patients with pulmonary stenosis and a small RV [12,27].…”