Background: Anomalous aortic origin of the coronary arteries (AAOCA) has an estimated prevalence of 0.1%-0.3%. Data on risks and benefits of surgery vs. observation to guide management decisions are limited. Methods: In a single center, we included patients (pts) with AAOCA with right (R) and/or left (L) intramural (IM), interarterial IA) or intraconal (IC) course from 1996-2014. We excluded pts with benign CA anomalies or major structural heart disease. Results: Among 154 pts, median age at diagnosis (dx) was 8.5 (range 0.1-50) yrs, median f/u was 1.92 (0.1-12.8) yrs, and 65% were male. The AAOCA course was IA in 116 (75%), IM in 52 (34%) and IC in 3 (2%). AAORCA was present in 126 (82%), of whom (51) 40% had repair. AAOLCA was present in 28 (18%), of whom 20 (71%) had repair; of the remaining 8, 2 had intraconal course; 2 were second opinions, 1 had Friederich’s ataxia, and 3 are awaiting surgery. In the surgical group, all had IA/IM CAs, and CA unroofing was performed in 90%, of whom 50% also had resuspension of the intercoronary commissure (table). Major perioperative complications occurred in 4 pts (6%: 1 ECMO, 1 mediastinitis, 2 early reoperation), 1 pt had late AoV repair. In the surgical group, no pts died; in the observed group, 2 pts with AAORCA (2.3%) died of severe non-cardiac comorbidities. Pts with surgery vs. observation were more likely to have AAOLCA (28% vs.10%, p=0.003), and, at time of dx, symptoms of chest pain/syncope (63% vs.13%, p<0.001), age >10 yrs (median 11 vs. 6 yrs, p<0.001), and exercise restriction (47% vs.13%; p<0.001). In multivariable modeling, surgical intervention was associated with chest pain or syncope (p<0.001) and older age (p=0.03). Conclusion: In our center, all pts with AAOLCA with IM or IA course underwent surgery, but management of AAORCA was variable, and rare but serious complications occurred. Studies with long-term f/u are needed to develop evidence-based management guidelines for AAOCA patients.
Assunta Fabozzo, Matthew DiOrio, Jane W Newburger, Andrew J Powell, Hua Liu, Francis Fynn-Thompson, et al. (2014). Anomalous Aortic Origin of Coronary Arteries: A Single Center Experience. Lippincott Williams & Wilkins.
Anomalous Aortic Origin of Coronary Arteries: A Single Center Experience
FABOZZO, ASSUNTA;
2014
Abstract
Background: Anomalous aortic origin of the coronary arteries (AAOCA) has an estimated prevalence of 0.1%-0.3%. Data on risks and benefits of surgery vs. observation to guide management decisions are limited. Methods: In a single center, we included patients (pts) with AAOCA with right (R) and/or left (L) intramural (IM), interarterial IA) or intraconal (IC) course from 1996-2014. We excluded pts with benign CA anomalies or major structural heart disease. Results: Among 154 pts, median age at diagnosis (dx) was 8.5 (range 0.1-50) yrs, median f/u was 1.92 (0.1-12.8) yrs, and 65% were male. The AAOCA course was IA in 116 (75%), IM in 52 (34%) and IC in 3 (2%). AAORCA was present in 126 (82%), of whom (51) 40% had repair. AAOLCA was present in 28 (18%), of whom 20 (71%) had repair; of the remaining 8, 2 had intraconal course; 2 were second opinions, 1 had Friederich’s ataxia, and 3 are awaiting surgery. In the surgical group, all had IA/IM CAs, and CA unroofing was performed in 90%, of whom 50% also had resuspension of the intercoronary commissure (table). Major perioperative complications occurred in 4 pts (6%: 1 ECMO, 1 mediastinitis, 2 early reoperation), 1 pt had late AoV repair. In the surgical group, no pts died; in the observed group, 2 pts with AAORCA (2.3%) died of severe non-cardiac comorbidities. Pts with surgery vs. observation were more likely to have AAOLCA (28% vs.10%, p=0.003), and, at time of dx, symptoms of chest pain/syncope (63% vs.13%, p<0.001), age >10 yrs (median 11 vs. 6 yrs, p<0.001), and exercise restriction (47% vs.13%; p<0.001). In multivariable modeling, surgical intervention was associated with chest pain or syncope (p<0.001) and older age (p=0.03). Conclusion: In our center, all pts with AAOLCA with IM or IA course underwent surgery, but management of AAORCA was variable, and rare but serious complications occurred. Studies with long-term f/u are needed to develop evidence-based management guidelines for AAOCA patients.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
