Abstract 1931: Results of a Transcatheter Based Management for Patients With Pulmonary Aresia-Intact Ventricular Septum and Non- Right Ventricular Dependant Circulation
Background: Transcatheter pulmonary valve perforation (PVP) of pts with pulmonary atresia-intact ventricular septum and non-right ventricular dependant coronary circulation (PAIVS-NRVDCC) has become more common in the past two decades. However, the effect of this strategy on outcomes has not been well established.
Patients and methods: From 1996 –2009, 52 neonates with PAIVS-NRVDCC were treated at our institution. Pts who had procedures elsewhere or severe Ebstein malformation were excluded.
Results: PVP was attempted in 31 of 52 pts (60%); 26 (84%) of these had a successful procedure. The remaining 21 pts (40%) and the 5 failed PVP attempts had surgery. There were no early deaths and 1 late death (3%). Complications of PVP included 5 (16%) myocardial perforations. The frequency of PVP increased over time, with 3/11 pts (27%) from 1996 –99, 8/21 (38%) from 2000 – 03, and 20/20 (100%) since 2004. Of those with successful PVP, 10 (38%) did not have surgery (group I) and 16 (62%) had surgery (group II) prior to discharge. In group II, 10 pts (63%) had a Blalock-Taussig shunt (BTS), 5 (31%) had right ventricular (RV) outflow tract surgery in addition to BTS, and 1 had a pulmonary valvotomy (6%). TV Z-score was larger in group I than in group II pts, with median TV diameter Z-scores of +0.7 (−0.9, 1.73) and −1.1 (−2.8, 2), respectively (p=0.01). Time from PVP to either discharge (group I) or surgery (group II) was significantly different between groups, 15 days (7, 22) and 8 days (0, 46), respectively (p=0.01). There was no difference in the number of trials or lowest arterial PaO2 off prostraglandins between groups. Freedom from operation after successful PVP was 31% (8/26). All pts with a successful PVP had an intermediate or biventricular circulation at median follow up of 1.9 yrs (0.03, 9.7).
Conclusions: PVP can provide definitive palliation in pts with PAIVS. Smaller TV size is associated with greater likelihood of surgery prior to discharge, and may serve as a surrogate for early RV inadequacy. Identification of suitable anatomic substrates may determine pts best suited for PVP and/or surgery.