Table 3

Outcomes of Interarterial ARCA/ALCA Patients (Studies With ≥20 Patients and at Least 1 Year of Follow-Up)

First Author, Year (Ref. #)YearPopulationAge, yrsTotal N (ARCA/ALCA)Follow-Up, yrsSurgery (n) (ARCA/ALCA)PCI (n) (ARCA/ALCA)Comments/Morbidity
Imaging Cohorts
Kaku et al. (48)199617,731 adults referred for ICA56 ± 1244 (44/0)5.6 ± 4.20 (0/)All patients treated conservatively and no deaths attributed to ARCA.
Lytrivi et al. (90)200814,546 children referred for TTE4 ± 527 (23/4)2.5 ± 3.06 (5/1)No deaths attributed to ARCA/ALCA among 22 patients with follow-up.
Krasuski et al. (91)2011210,700 adults referred for ICA58 ± 1454 (36/18)9.2 [4.5–16.1]28 (20/8)Among 301 AAOCA, similar all-cause mortality with surgery (n = 36 of 94, 38%) vs. no surgery (n = 95 of 207, 46%). Among 54 IAC, lower all-cause mortality with surgery (n = 5 of 28, 18%) vs. no surgery (n = 12 of 26, 46%), but underpowered for comparison. No perioperative deaths occurred.
Lee et al. (92)201222,925 adults referred for CTA
53 IAC vs. 34 subpulmonic ARCA
56 ± 1253 (53/)2.5 [0.8–3.9]8 (8/)1 CV death, 3 nonfatal MI, 8 UA in follow-up among 53 with IAC. Angina more common in IAC group vs. subpulmonic course (43% vs. 6%, p = 0.001)
Opolski et al. (67)20138,522 adults referred for CTA56 ± 1322 (20/2)1.3 ± 1.02 (1/1)Among 72 AAOCA, 97% (n = 70 of 72) conservatively managed, with no deaths or MI attributed to AAOCA; 27% had worsened symptoms. Syncope more common in IAC compared with no IAC (33% vs. 9%, p = 0.04).
Ripley et al. (38)201459,844 adults referred for MRA54 [40–64]64 (/)4.3 [2.5–7.8]23 (/)13 single coronaries with unclear course. 3 deaths not attributed to IAC.
Nasis et al. (49)20159,774 adults referred for CTA58 ± 1344 (36/8)2.3 ± 1.39 (6/3)No cardiac deaths or acute coronary syndrome in follow-up.
Cheezum et al. (5)20175,992 adults referred for CTA52 ± 1743 (40/3)5.8 [3.8–7.8]13 (12/1)2 late (≥90-day) CV deaths in ARCA patients, not attributed to ARCA vessel.
Revascularization Cohorts
Osaki et al. (53)200831 ARCA/ALCA patients; subset of 7 ALCA with surgery6 ± 731 (18/13)4.8/1.97 (0/7)1 patient with symptoms after surgery; 1 cardiac death (care withdrawn POD1 for neurological impairment after resuscitation); No surgery/surgery.
Davies et al. (93)200936 ARCA/ALCA surgical repair44 ± 1636 (21/15)1.1 ± 2.836 (21/15)2 LAD included; 1 (3%) recurrent symptoms; 1 death from subdural bleed.
Frommelt et al. (94)201127 ARCA/ALCA surgical repair13 ± 427 (20/7)1.8 []27 (20/7)No ischemic symptoms or deaths in follow-up. 7 patients with trivial AI.
Mumtaz et al. (95)201122 ARCA/ALCA unroofing15 []22 (15/7)1.4 []22 (15/7)1 (5%) with chest pain but normal ICA post-operatively. No deaths in follow-up.
Kaushal et al. (96)201127 ARCA/ALCA surgical repair14 ± 1227 (25/2)1.2 ± 0.127 (25/2)2 (7%) with noncardiac symptoms. No deaths or significant morbidity.
Mainwaring et al. (51)201474 ARCA/ALCA surgical repair15 []74 (47/27)6.0 []74 (47/27)1 heart transplant; remaining patients free of cardiac symptoms. No deaths.
Wittlieb-Weber et al. (97)/Brothers et al. (52)2014/200724 ARCA/ALCA surgical repair12 []24 (16/8)5.3 []24 (16/8)13 (54%) with follow-up symptoms, none with ischemia. Normal quality of life when reported (n = 12). No deaths, 1 emergent reoperation POD1, 2 post-operative infections, 4 mild AI, 11 effusions (1 requiring drainage).
Sharma et al. (98)201475 ARCA/ALCA unroofing40 ± 2075 (69/6)1.6 ± 0.275 (69/6)1 (1%) late noncardiac death. No cardiac symptoms at follow-up.
Angelini et al. (42)201567 ARCA w/IVUS;
42 PCI, 3 surgery, 22 medical therapy
48 ± 1267 (67/)5.0 ± 2.93 (3/)42 (42/)Symptoms improved in PCI subset. No ARCA related deaths. n = 10 had clinically indicated follow-up ICA, including n = 4 with in-stent restenosis (n = 2 BMS, n = 2 DES) and n = 1 with late-stent thrombosis.
Feins et al. (99)2016259 patients with AAOCA;
61 with surgery, 29 ARCA/ALCA
43 ± 329 (24/5)3.8 ± 0.829 (24/5)97% free of symptoms attributed to coronary anomaly; 1 death from endocarditis of a prosthetic valve. No perioperative deaths.
Reviews
Moustafa et al. (100)2008264 ALCA (130 autopsy, 62 with no follow-up, 72 with follow-up)36 ± 2272 (/72)1.757 (/57)4 (/4)1 (2%) with severe AI after ALCA unroofing, otherwise no significant operative morbidity. No deaths in follow-up. Weighted average.
Nguyen et al. (68)2012326 ALCA surgical repair326 (/326)0–14326 (/326)2.2% symptoms, 9.2% morbidity, 0.6% cardiac death (n = 2 of 325) in follow-up

Values are mean ± SD [IQR] unless otherwise indicated.

AAOCA = anomalous aortic origin of the coronary artery; AI = aortic insufficiency; BMS = bare-metal stent; CTA = computed tomography angiography; CV = cardiovascular; DES = drug-eluting stent; F/U = follow-up; IAC = interarterial course; ICA = invasive coronary angiography; IVUS = intravascular ultrasound; LAD = left anterior descending artery; MRA = magnetic resonance angiography; MI = myocardial infarction; PCI = percutaneous coronary intervention; POD = post-operative day; SD = standard deviation; TTE = transthoracic echocardiogram; UA = unstable angina; other abbreviations as in Table 1.

  • Not applicable or not reported.

  • Median value.