Medium-term outcome after anomalous aortic origin of a coronary artery repair in a pediatric cohort

OBJECTIVES: We sought to describe early and late outcomes in a large surgical series of patients with anomalous aortic origin of coronary arteries. METHODS: We performed a retrospective multicentre study including surgical patients with anomalous aortic origin of coronary arteries since 1991. Patients with isolated high coronary takeoff and associated major congenital heart disease were excluded. RESULTS: We collected 156 surgical patients (median age 39.5 years, interquartile range 15–53) affected by anomalous right (67.9%), anomalous left (22.4%) and other anatomical abnormalities (9.6%). An interarterial course occurred in 86.5%, an intramural course in 62.8% and symptoms in 85.9%. The operations included coronary unroofing (56.4%), reimplantation (19.2%), coronary bypass graft (15.4%) and other (9.0%). Two patients with preoperative cardiac failure died postoperatively (1.3%). All survivors were discharged home in good clinical condition. At a median follow-up of 2 years (interquartile range 1–5, 88.5% complete), there were 3 deaths (2.2%), 9 reinterventions in 8 patients (5 interventional, 3 surgical); 91.2% are in New York Heart Association functional class ≤ II, but symptoms persisted in 14.2%; 48.1% of them returned to sport activity. On Kaplan–Meier analysis, event-free survival at follow-up was 74.6%. Morbidity was not significantly different among age classes, anatomical variants and types of surgical procedures. Furthermore, return to sport activity was significantly higher in younger patients who participated in sports preoperatively. CONCLUSIONS: Surgical repair of anomalous aortic origin of coronary arteries is effective and has few complications. Unroofing and coronary reimplantation are safe and are the most common procedures. The occurrence of late adverse events is not negligible, and long-term surveillance is mandatory. Most young athletes can return to an unrestrained lifestyle.

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“…As reported in the literature and in other surgical series [6][7][8][9][10][11][12], in our study AORCA was the most frequent anomaly (67.3%).…”

Section: Discussionsupporting

confidence: 86%

“…Single-centre studies report on limited series, describing surgical repair as safe and effective [6][7][8][9][10][11][12], although late effects on symptoms and risk of SCD are undefined and concerns about the surgical effectiveness remain [13][14][15].…”

Section: Introductionmentioning

confidence: 99%

Surgery for anomalous aortic origin of coronary arteries: a multicentre study from the European Congenital Heart Surgeons Association†

Padalino

Franchetti

Hazekamp

et al. 2019

European Journal of Cardio-Thoracic Surgery

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“…In our experience, surgical indication was in accordance with the most accepted expert consensus surgical guidelines [24,25], despite some intercenter variability. As in other surgical series [10][11][12][13][14], unroofing of the intramural segment was the commonest procedure (56.4%), whose protective effect from AE (OR 0.31, Table 3) is to be related to excision of the intervening roof of the intramural segment, (causing coronary compression and ischemia during effort), the enlargement of the slit like orifice and its relocation to the appropriate sinus, and the elimination of the interarterial course. Coronary reimplantation was favoured when the intramural segment was too short or absent, while CABG procedure was performed in 24 patients at a significantly higher age.…”

Section: Discussionmentioning

confidence: 53%

“…Surgical treatment has been widely proposed, aiming at removing any risk of myocardial ischemia and SCD. Single-center studies are based on limited series describing surgical repair as safe and effective [7][8][9][10][11][12][13], although late effects on symptoms and risk of SCD are undefined and concerns about the surgical effectiveness remain [14][15][16].…”

Section: Introductionmentioning

confidence: 99%

Anomalous aortic origin of coronary arteries: Early results on clinical management from an international multicenter study

Padalino

Franchetti

Sarris

et al. 2019

International Journal of Cardiology

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Background: Anomalous aortic origin of coronary arteries (AAOCA) is a rare abnormality, whose optimal management is still undefined. We describe early outcomes in patients treated with different management strategies. Methods: This is a retrospective clinical multicenter study including patients with AAOCA, undergoing or not surgical treatment. Patients with isolated high coronary take off and associated major congenital heart disease were excluded. Preoperative, intraoperative, anatomical and postoperative data were retrieved from a common database. Results: Among 217 patients, 156 underwent Surgical repair (median age 39 years, IQR: 15-53), while 61 were Medical (median age 15 years, IQR: 8-52), in whom AAOCA was incidentally diagnosed during screening or clinical evaluations. Surgical patients were more often symptomatic when compared to medical ones (87.2% vs 44.3%, p b 0.001). Coronary unroofing was the most frequent procedure (56.4%). Operative mortality was 1.3% (2 patients with preoperative severe heart failure). At a median follow up of 18 months (range 0.1-23 years), 89.9% of survivors are in NYHA ≤ II, while only 3 elderly surgical patients died late. Return to sport activity was significantly higher in Surgical patients (48.1% vs 18.2%, p b 0.001). Conclusions: Surgery for AAOCA is safe and with low morbidity. When compared to Medical patients, who remain on exercise restriction and medical therapy, surgical patients have a benefit in terms of symptoms and return to normal

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“…17 However, in a small cohort of patients following AAOCA repair, QOL scores have been reported to be similar to the general population in short and mid-term. 18,19 This likely needs to be investigated further as noncardiac specific questionnaires were used in this study.…”

Section: Who Do You Think Should Be Invited To These Meetings Besidesmentioning

confidence: 99%

Family perception of unmet support needs following a diagnosis of congenital coronary anomaly in children: Results of a survey

Agrawal

Wright

Carberry

et al. 2017

Congenital Heart Disease

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Medium-term outcome after anomalous aortic origin of a coronary artery repair in a pediatric cohort

Abstract: In the medium-term after AAOCA repair, cardiac-type symptoms frequently persist and morbidity is common, but these do not impair QOL. The significance of these findings in the long-term is unknown and warrants continued follow-up.

Cited by 49 publications

References 27 publications

Surgery for anomalous aortic origin of coronary arteries: a multicentre study from the European Congenital Heart Surgeons Association†

Surgery for anomalous aortic origin of coronary arteries: a multicentre study from the European Congenital Heart Surgeons Association†

Anomalous aortic origin of coronary arteries: Early results on clinical management from an international multicenter study

Family perception of unmet support needs following a diagnosis of congenital coronary anomaly in children: Results of a survey

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