Anomalous Origin of Left

Coronary Artery From Aortic

Arch Associated With
Hypoplastic Left Heart
Syndrome
Satoshi Asada, MD, Masaaki Yamagishi, MD, PhD,
Koichi Nishida, MD, Shintaro Okazaki, MD, PhD,
Yoshinobu Nakayama, MD, PhD, Osamu Monta, MD,
Yasushi Tsutsumi, MD, and Hirokazu Ohashi, MD, PhD
Department of Cardiovascular Surgery, Fukui Cardiovascular
Center, Fukui; Department of Pediatric Cardiovascular Surgery,
Children’s Medical Center, Kyoto Prefectural University of
Medicine, Kyoto; Department of Pediatrics, Fukui Cardiovascular
Center, Fukui; and Department of Anesthesiology, Kyoto
Prefectural University of Medicine, Kyoto, Japan
Fig 1. Volume-rendered images from preoperative computed tomog-
raphy in (A) anterior and (B) right anterior oblique views. An aber-
Anomalous origin of the coronary artery from the aortic rant left coronary artery (red) arises from the distal aortic arch. This
arch associated with hypoplastic left heart syndrome is an aberrant left coronary artery runs along a diminutive ascending aorta,
extremely rare anomaly. Coronary anomalies can signifi- behind the pulmonary trunk. No evidence of ventriculocoronary
cantly deteriorate the clinical outcomes of hypoplastic left communication is identified. The right coronary artery is not detected
heart syndrome. We describe the case of a newborn with in this examination.
concomitant hypoplastic left heart syndrome and
abnormal origin of the left coronary artery arising from aorta. No evidence of ventriculocoronary communication
the distal aortic arch. was detected. The duct was widely open with continuous
(Ann Thorac Surg 2019;107:e51–3) administration of lipo-prostaglandin E1. The intraatrial
Ó 2019 by The Society of Thoracic Surgeons communication was not restrictive. Electrocardiography
and laboratory data revealed no signs of myocardial
ischemia preoperatively.
C oronary artery anomalies in hypoplastic left heart
syndrome (HLHS) are a rare finding. Anomalous
origins of coronary arteries may occur in very exceptional
On day 7, we performed bilateral pulmonary artery
(PA) banding through a median sternotomy. No
abnormal coronary artery was visualized, because we
cases [1]. Coronary abnormalities complicated with
anticipated a hemodynamic drop during dissection of the
HLHS carry a higher risk of poor prognosis. The clinical
abnormal artery. The right PA was encircled with a
course of and surgical strategies for such patients likewise
banding tape between the superior vena cava and
remain problematic.
ascending aorta. The left PA was encircled at the origin
from the pulmonary trunk. The blood pressure dropped
A 2,662-g full-term newborn was delivered by scheduled
drastically with ST elevation on monitor electrocardiog-
caesarian section with the prenatal diagnosis of HLHS
raphy and ventricular fibrillation was induced after mild
and referred to our institution at 5 days old for surgical
banding, so we were unable to complete adequate
repair. She had been diagnosed with HLHS with aortic
banding. Short-duration cardiopulmonary resuscitation
atresia and mitral stenosis, patent ductus arteriosus, and
led to recovery of her heartbeat.
atrial septal defect. No other malformations were detec-
Although we attempted rebanding of the right and left
ted. Preoperatively, she was in a high pulmonary blood
PAs under the use of low-dose inotropic agents, an
flow condition breathing room air, with oxygen saturation
adequate procedure could not be achieved. We were
between 89% and 92%.
compelled to abandon completion of this procedure.
Computed tomography demonstrated a diminutive
Only slight bandings were performed, and she was
ascending aorta and an anomalous origin of the left cor-
transferred to the intensive care unit with the chest
onary artery from the distal aortic arch (Fig 1). Echocar-
open. However, she entered a hemodynamically unsta-
diography showed a hypoplastic ascending aorta without
ble condition and ventricular fibrillation was induced
retrograde blood flow in a Doppler study but also indi-
again. Despite cardiopulmonary resuscitation with high-
cated the presence of an abnormal coronary artery with
dose inotropic agents and antiarrhythmic drugs, as well
antegrade blood flow arranged beside the ascending
as removal of banding tapes, spontaneous heart beat was
not recovered.
Accepted for publication May 12, 2018.
Address correspondence to Dr Yamagishi, Department of Pediatric
Cardiovascular Surgery, Children’s Medical Center, Kyoto Prefectural Dr Yamagishi discloses a financial relationship with
University of Medicine, 465 Kajii-cho, Kamigyo-ku, Kyoto 602-8566, W. L. Gore & Associates, Inc.
Japan; email: myama@koto.kpu-m.ac.jp.

Ó 2019 by The Society of Thoracic Surgeons 0003-4975/$36.00

Published by Elsevier Inc. https://doi.org/10.1016/j.athoracsur.2018.05.028
e52 CASE REPORT ASADA ET AL
ANOMALOUS CORONARY ARTERY ORIGIN IN HLHS
Fig 2. Posterior view of the heart on autopsy and scheme of the anomalous origin of the left coronary artery from the aortic arch. An aberrant left
coronary artery arises (black arrows) from the distal aortic arch. This coronary artery runs along the small ascending aorta (*, yellow arrow).
Heart autopsy confirmed the anomalous origin of the
left coronary artery from the distal aortic arch (Fig 2). In
addition, the ostium of this aberrant left coronary artery
was mildly stenotic. The right coronary artery arose from
the original aortic root, but was very hypoplastic. No
obvious ventriculocoronary communication was found.
Comment
The combination of an anomalous origin of the coronary
artery and HLHS is extremely rare. Aberrant origin of the
left coronary artery from the pulmonary artery (ALCAPA)
has been described in the literature [1]. To the best of our
knowledge, only two reports have described an anoma-
lous origin of the coronary artery associated with HLHS
other than ALCAPA. One right coronary artery arose
from the descending thoracic aorta [2] and the other from
the brachiocephalic trunk [3].
Although we only performed preoperative computed
tomographic angiography for this patient, involvement of
a minor ventriculocoronary communication cannot be
completely ruled out. Vida and colleagues [4] reported a
higher mortality rate with the mitral stenosis-aortic
atresia variant of HLHS than with other anatomic sub-
types of HLHS, and ventriculocoronary communications
associated with this variant have emerged as showing a
higher risk of death [4]. In addition, occurrence of ven-
triculocoronary communications appeared limited to this
variant in an autopsy study by Nathan and colleagues [5].
They recommended preoperative angiography for all
patients with mitral stenosis-aortic atresia to confirm and
better delineate ventriculocoronary communications.
Performing bilateral PA banding as a first palliation
was assumed to have somehow influenced hemodynamic
Ann Thorac Surg
2019;107:e51–3
balance, including coronary blood flow, in this patient.
Surgical strategies for extremely rare coronary anomalies
associated with HLHS are still under dispute. Predicting
hemodynamic changes of coronary blood flow through a
narrow ascending aorta or a long aberrant coronary ar-
tery is difficult after bilateral PA banding. According to
the computational model simulation, bilateral PA band-
ing for HLHS with a narrow ascending aorta can cause
coronary insufficiency [6]. This problem can also occur
with coronary anomalies. Such hemodynamic changes
after banding might have led to blood flow instability in
this abnormal coronary artery, with or without involve-
ment of the ventriculocoronary communication.
Obviously, coronary artery anomalies should be eval-
uated preoperatively [7]. In the setting of confirmed
diagnosis of such a coronary anomaly, the primary Nor-
wood palliation combined with coronary artery recon-
struction should be taken into account, but aberrant
coronary artery reconstruction represents a herculean
procedure with great risk of coronary insufficiency,
especially in the neonatal period. That was why we chose
bilateral PA banding as an initial palliation, but this
attempt had an unfortunate consequence. Optimal man-
agement strategies for HLHS with such coronary abnor-
malities need to be established despite the extremely low
incidence of these anomalies. Heart transplantation as a
surgical alternative can also be taken into consideration
wherever possible.
References
1. Nathan M, Emani S, Marx G, Pigula F. Anomalous left coro-
nary artery arising of the pulmonary artery in hypoplastic left
hearts: case series and review of literature. J Thorac Car-
diovasc Surg 2011;142:225–7.
Ann Thorac Surg
2019;107:e51–3
2. Cheatham JP, Ruyle NA, McManus BM, et al. Origin of the
right coronary artery from the descending thoracic aorta:
angiographic diagnosis and unique coronary artery anatomy
at autopsy. Cathet Cardiovasc Diagn 1987;13:321–4.
3. Liu F, Huang G, Zhang J. Anomalous origin of a coronary
artery from the right brachiocephalic trunk associated with
complex congenital heart disease. Pediatr Cardiol 2010;31:
163–5.
4. Vida VL, Bacha EA, Larrazabal A, et al. Surgical outcomes for
patients with the mitral stenosis-aortic atresia variant of hy-
poplastic left heart syndrome. J Thorac Cardiovasc Surg
2008;135:339–46.
CASE REPORT ASADA ET AL e53
ANOMALOUS CORONARY ARTERY ORIGIN IN HLHS
5. Nathan M, Williamson AK, Mayer JE, Bacha EA, Juraszek AL.
Mortality in hypoplastic left heart syndrome: review of 216
autopsy cases of aortic atresia with attention to coronary ar-
tery disease. J Thorac Cardiovasc Surg 2012;144:1301–6.
6. Backer CE, Corsini C, Cosentino D, et al. Effects of pulmonary
artery banding and retrograde aortic arch obstruction on the
hybrid palliation of hypoplastic left heart syndrome. J Thorac
Cardiovasc Surg 2013;146:1341–8.
7. Saroli T, Gelehrter S, Gomez-Fifer CA, van der Velde ME,
Bove EL, Ensing GJ. Anomalies of left coronary artery origin
affecting surgical repair of hypoplastic left heart syndrome
and Shone complex. Echocardiography 2008;25:727–31.