ª 2022 The Author(s). Published by Elsevier Inc. on behalf of The Society of Thoracic Surgeons.

12772-9931

This is an open access article under the CC BY-NC-ND license

https://doi.org/10.1016/j.atssr.2022.06.003(http://creativecommons.org/licenses/by-nc-nd/4.0/).

death.1

Althoughthepathophysiologic

Quantitative Myocardial
Perfusion in Surgically
Repaired Coronary
Artery Anomaly

cardiac

Shiro Miura, MD, MSc,
Atsutaka Okizaki, MD, PhD,
Osamu Manabe, MD, PhD,Shunsuke Ohori, MD, Ryo Suzuki, MD,
Chihoko Miyazaki, MD, PhD, andTakehiro Yamashita, MD, PhD

stress testing. However, accurately documenting theDepartment of Cardiology, Hokkaido Ohno Memorial
Hospital, Sapporo, Japan; Department of Radiology,Asahikawa Medical University, Asahikawa, Hokkaido,Japan; Department of Radiology, Saitama MedicalCenter, Jichi Medical University, Saitama-shi, Japan;
Department of Cardiovascular Surgery, Hokkaido Ohno
Memorial Hospital, Sapporo, Japan; and Department ofDiagnostic Radiology, Hokkaido Ohno Memorial
Hospital, Sapporo, Japan

The patient had no remarkable medical history. Elec-The anomalous aortic origin of the right coronary arterywith an intramural course is rare and can cause
myocardial ischemia, for which surgical repair is indicated. We present the case of a 49-year-old man withthis anomaly whose preoperative myocardial perfusion
positron emission tomography revealed inducible
ischemia. Comparison of the preoperative and followup (6 months) positron emission tomography ﬁndings
showed signiﬁcant improvement in regional myocardial
ﬂow reserve and relative ﬂow reserve, in keeping with
the improvement in symptoms. However, positronemission tomography/myocardial perfusion imaging
ﬁndings were difﬁcult to interpret.

mechanism of sudden cardiac death in patients withAAOCA has not been completely elucidated, it isprincipally the result of insufﬁcient myocardial blood
ﬂow (MBF) and MI. Therefore, surgical intervention is
generally

warranted

inpatientswithsymptoms

suggestive of MI or inducible ischemic changes on
evidence of MI using conventional imaging modalities
may be challenging. The patient provided writtenconsent for the publication of the following information.
A 49-year-old man was referred for a 30-year historyof frequent chest pain at rest and during exercise.
trocardiography, echocardiography, and 24-hour Holter
monitoring

revealednosigniﬁcantabnormalities.

Coronary computed tomography angiography (CCTA)
identiﬁed an anomalous right coronary artery (RCA)
arising from the left coronary sinus with an interarterial
and intramural course through a separate stenotic
ostium slightly below the aortic valve commissure(Figures1A,1C).

Invasive

coronary

angiography

conﬁrmed the anomalous RCA without concomitantobstructive

coronaryarterydisease(Figure

Adenosine triphosphate–induced stress131E).

N-ammoniapositron emission tomography (PET) revealed a perfusion abnormality in the inferoposterior area of theleft ventricle (Figure 2A). Both stress MBF andmyocardial ﬂow reserve (MFR) were lower than in the
nonanomalous vessel territory. Surgical intervention
was considered on the basis of the typical symptoms
and evidence of MI. As the course of the intramuralpart was close to the right and left aortic valve
commissure, we performed fenestration by creating a
neo-ostium

attherightcoronarysinuswithout(Ann Thorac Surg Short Reports 2022;-:1-4)

involving the commissure (Figure 3).4 CCTA performedª 2022 The Author(s). Published by Elsevier Inc. on behalf

10 days postoperatively showed a widely open neo-of The Society of Thoracic Surgeons. This is an openostium (Figures 1B, 1D). PET performed 14 days

access article under the CC BY-NC-ND license

postoperatively revealed partial improvement in stress-(http://creativecommons.org/licenses/by-nc-nd/4.0/).

induced ischemia, but regional stress MBF and MFRT

were lower than preoperative values in the anomalous
RCA-supplied territory (Figure 2B). The patient hoped tohe anomalous aortic origin of a coronary artery

remain in the inpatient rehabilitation program for as(AAOCA) from the inappropriate sinus of Val-long as possible mainly because hospital access wassalva is being increasingly detected with the

difﬁcult and because of the incomplete improvement

help of multimodality imaging. Although most AAOCA

insubtypes are benign, high-risk anatomic features (ie,postoperative exercise electrocardiography testing hadinterarterial course, slitlike ostium, intramural course,

notacute takeoff angle with tangential vessel course, andchange suggestive of ischemia, and the patient was dis-

proximal narrowing of the anomalous vessel) are associ-charged 28 days after operation. At the 6-month follow-

ated with increased risks of myocardial ischemia (MI),up, he was asymptomatic. Repeated PET revealed aventricular arrhythmias, heart failure, and sudden

regional defect at rest, which normalized on the stresshissymptoms.

provokedWe

typicalreassured

symptoms

himandthat

theST-segment

Accepted for publication Jun 13, 2022.
Address correspondence to Dr Miura, Department of Cardiology, Hokkaido Ohno Memorial Hospital, 2-1-16-1 Miyanosawa, Nishi-ku, Sapporo 063-0052,
Japan; email: shirotan1027m@yahoo.co.jp.

2

CASE REPORTMIURA ET AL

Ann Thorac Surg Short Reports
2022;-:1-4

CORONARY FLOW IN CORONARY ARTERY ANOMALYFIGURE 1 (A) Preoperative and (B) postoperative coronary computed tomography angiography (CCTA; upper, axial image;lower, curved planar reformatted) ﬁndings show anomalous right coronary artery (RCA) between the aortic route (Ao) andpulmonary artery (PA), which features a separate ostium, interarterial course, acute takeoff angle (*), intramural course(red arrowheads in A), and oval proximal vessel narrowing (yellow arrow in A). The neo-ostium (blue arrowheads in B) was
created at the right coronary sinus (RCS). (C) Preoperative and (D) postoperative volume-rendered images reveal thetakeoff level of anomalous RCA below the aortic valve commissure (yellow arrowheads in C). (E) Invasive coronary
angiography reveals the anomalous origin of the RCA from the left coronary sinus (LCS) of Valsalva without concomitantobstructive coronary artery disease. (LCA, left coronary artery.)

image (reverse ﬁll-in; Figure 2C). The 3-dimensionalthat in the left anterior descending artery–supplied terri-

integration of PET/myocardial perfusion imaging (MPI)

tory, signiﬁcantly increased (preoperative, postoperative,with CCTA at the preoperative, postoperative, andand follow-up RFR values: 0.69, 0.74, and 0.87,

follow-up phases clearly illustrated a more accurate

respectively).

assignment of vascular distribution in relation to theserial change of MPI in the anomalous RCA territory

COMMENT(Video). In contrast to the preoperative status, regional
stress MBF and MFR improved postoperatively. Of note,

In this AAOCA case, serial changes (preoperative, post-the relative ﬂow reserve (RFR),3 deﬁned as the ratio ofoperative, and 6-month postoperative status) in quan-stress MBF in the anomalous RCA-supplied territory totitative

PET

assessments,

PET/MPIﬁndings,andCASE REPORTAnn Thorac Surg Short Reports
2022;-:1-4

MIURA ET AL

CORONARY FLOW IN CORONARY ARTERY ANOMALYFIGURE 2 Perfusion polar maps (A, preoperative; B, postoperative; C, 6-month follow-up) of stress and rest using 13N-ammonia
positron emission tomography (PET)/myocardial perfusion imaging with PET-derived ﬂow indices (presented at the bottom) in eachcoronary artery territory. The normal mean myocardial blood ﬂow (MBF) at rest and stress and myocardial ﬂow reserve (MFR) are 0.71
mL/min per gram (normal range, 0.61-1.1), 2.58 mL/min per gram (1.86-4.33), and 3.54 (3.16-4.8), respectively,2 and a relative ﬂowr e s e r v e ( R F R ) o f ‡0 . 7 8 w a s c o n s i d e r e d n o r m a l . 3 ( L A D , l e f t a n t e r i o r d e s c e n d i n g c o r o n a r y a r t e r y ; L C X , l e f t c i r c u m ﬂ e x c o r o n a r y a r t e r y ;RCA, right coronary artery.)symptoms are described. Notably, our patient showedvarious anatomic high-risk features.1 Considering thepresentation of typical symptoms and evidence ofinducible MI, surgical repair was performed per current
guidelines.5

Surgicalrepairwasperformedwithfenestration because the intramural course was below
the sinotubular junction.4 Extensive takedown andresuspension of aortic valve commissures, which cancause aortic regurgitation, were avoided with thismethod.
Evaluation of MI using 13N-ammonia PET before andafter surgical repair is technically challenging with

FIGURE 3 Intraoperative demonstration of the anomalous right coronary ar-limited data. The patient initially showed stress-induced

tery (A) before and (B) after surgical repair by fenestration. The dotted blackischemia in the anomalous RCA-supplied territory, which
partially improved in concordance with his symptoms. Atdouble arrow indicates the intramural segment of the anomalous right coronary artery (RCA).

34

CASE REPORTMIURA ET AL

Ann Thorac Surg Short Reports
2022;-:1-4

CORONARY FLOW IN CORONARY ARTERY ANOMALY

follow-up, PET/MPI demonstrated the reverse ﬁll-in

In this case, the ﬁndings of serial quantitative as-pattern, reportedly challenging to interpret.6 Notably,

sessments using 13N-ammonia PET were concordant

the phenomenon might be a signiﬁcant indicator ofwith symptomatic improvement, but those of qualita-

improved myocardial viability, as reported in a prior

tive assessments with PET/MPI were inconclusive,

study of patients after coronary artery bypass grafting.7

suggesting that performing quantitative assessments in

Residual postsurgical MI, which may be fatal, isaddition to PET/MPI might help determine the indica-

common among children despite successful surgicaltion, timing, and effectiveness of surgical repair for

repair for AAOCA.8 Postoperatively, the regional stressAAOCA.

MBF in the anomalous RCA-supplied territory did notincrease, and the regional MFR dropped slightly from2.96 to 2.56. Both parameters were markedly improved at
the follow-up PET. These ﬁndings imply that recovery or
normalization of myocardial hypoperfusion, ischemia,
and microvascular dysfunction in the anomalous coronary territory might take longer than expected. RFR
could be another useful noninvasive indicator in patientswith AAOCA because it signiﬁcantly improved with theimprovement in symptoms.

The Video can be viewed in the online version of this article [https://doi.org/10.1016/j.atssr.2022.06.003] on http://www.annalsthoracicsurgery.org.

FUNDING SOURCES
The authors have no funding sources to disclose.

DISCLOSURESThe authors have no conﬂicts of interest to disclose.

PATIENT CONSENT
Obtained.

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