| Autor: |
Rao, Hussain, Alshami, Noor, Hasan, Mohammed, Sarvestani, Amber Leila, Turk, Elizabeth, Thomas, Amanda S, Kochilas, Lazaros, Raghuveer, Geetha |
| Zdroj: |
Circulation (Ovid); November 2019, Vol. 140 Issue: Supplement 1 pA12610-A12610, 1p |
| Abstrakt: |
Background:Truncus Arteriosus (TA) associated with Interrupted Aortic Arch (IAA) is a rare anomaly and outcomes are not well-defined. The objective of this study is to better define long-term, transplant-free survival in children with TA and IAA who underwent single stage, primary repair.Methods:This retrospective study includes patients <21 years of age enrolled in the Pediatric Cardiac Care Consortium, a multi-institutional, US-based registry of patients with congenital heart defects and their associated interventions. Prospectively acquired long-term outcome data was assessed through 2014 via linkage to the National Death Index and Organ Procurement and Transplant Network.Results:A total of 53 patients with TA and IAA were noted to have undergone single stage repair of these defects. Of these, 51% were male, 58% had TA type I (Collett and Edwards Classification). IAA type B was the most common form of interruption in this cohort (72%). Median age at surgery was 6 days (IQR: 4-13), median weight at surgery was 3.2 kg (IQR: 2.8-3.5). DiGeorge or a related genetic anomaly was documented in less than half of this cohort (45%). There were 25 (47%) in-hospital deaths, 12 of these TA type I (48%) and 13 TA type II (52%). 18 of the 25 deaths had IAA type B (72%) and 7 had IAA type A (28%). Survival after discharge reached 86% over a median follow up period of 17.4 years (IQR: 15.1-20.3) with 4 deaths occurring within a median period of 1.6 years post discharge (IQR: 0.4-2.6). None received a cardiac transplant. In contrast, 6 patients who had staged repair of TA and IAA died following initial IAA repair.Conclusions:Mortality following single stage repair of TA and IAA is primarily early with good long-term survival in those who were alive at hospital discharge. |
| Databáze: |
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