Surgical treatment of pulmonary atresia with major aortopulmonary collateral arteries in 83 consecutive patients†

Abstract

Total survival was significantly lower in patients with the 22q11 deletion. Surgical management based on preoperative pulmonary arterial anatomical features improves early surgical results.

A Review of the Management of Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries

“…The Melbourne study achieved repair in 82% of patients with no operative mortality at an age of 1.7 years, which is early considering that this strategy is staged [45]. With a combined staged strategy involving 60 patients, Babliak and colleagues [29] had a repair rate of 60% with no early mortality.…”

“…The two concepts are not mutually exclusive, and most researchers recognize a "combined strategy," as introduced by the Melbourne group (Iyer and Mee [26]), that combines the unifocalization and rehabilitation pathways (Fig 1). Most teams have a preferred strategy, although a few groups adopted a mixed management of their patients [27][28][29][30].…”

“…The Birmingham (UK) team [37] have also promoted the one-stage repair with unifocalization as their preferred strategy, using the midline sternotomy approach where possible-but also advocating combined thoracotomy with sternotomy where necessary to access the MAPCAs. Other groups use unifocalization alongside with another strategy: rehabilitation strategy [27] or combined strategy [28,29]. STAGED UNIFOCALIZATION.…”

“…INSERTION OF MAIN PA IN AORTA. Iyer and Mee [26] and others [28,29,31,47,48] originally described an end-to-side anastomosis of the main pulmonary artery to the ascending aorta, the so-called Melbourne shunt ( Fig 2C). This type of shunt and strategy was used in reports of selected patients with present native and diminutive central pulmonary arteries, in other words, PA/VSD type B [49,50].…”

22q11.2 deletion syndrome and congenital heart disease

“…Anaclerio et al () also reported higher mortality rates in 22q11.2 deleted as compared to non‐deleted patients with tetralogy of Fallot and pulmonary atresia. This trend has persisted over time with higher mortality reported by several groups in the subset with tetralogy of Fallot, pulmonary atresia, and aortopulmonary collaterals (Babliak, Mykychak, Motrechko, & Yemets, ; Bauser‐Heaton et al, ; Carotti et al, ; Chen et al, ).…”