Successful surgical treatment of a gigantic congenital coronary artery
fistula immediately after birth
Yorihiko Matsumoto, Takaya Hoashi*, Koji Kagisaki and Hajime Ichikawa
Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan
* Corresponding author. 5-7-1 Fujishiro-dai, Suita, Osaka, 565-8565, Japan. Tel: +81-6-68335012; fax: +81-6-68339865;
e-mail: firstname.lastname@example.org (T. Hoashi).
Received 12 March 2012; received in revised form 24 April 2012; accepted 28 April 2012
A foetus was prenatally diagnosed with a gigantic (12 mm) coronary artery fistula (CAF) from the left anterior descending (LAD) coron-
ary artery to right ventricular apex at 38 weeks of gestation. LAD was dilated to 10 mm with partial aneurysmal changes. Because of
concern for sudden ischaemic cardiogenic shock soon after birth, the child was electively delivered by caesarean section, with surgical
fistula closure subsequently performed 1 h after birth. We also highly suspected the presence of a clinically significant accessory diag-
onal branch just around the fistula, thus direct fistula closure from outside the heart without cardiopulmonary bypass was abandoned
and cardiopulmonary bypass was initiated. The terminal end of LAD was carefully opened, and the fistula was directly closed with four
pairs of 6-0 polypropylene mattress sutures under cardioplegic arrest, while the opened terminal end of LAD was also repaired with
plegetted 6-0 polypropylene mattress and over-and-over sutures. After 4 days of post-surgical extracorporeal life support for over-
systemic pulmonary hypertension, the patient recovered without complications. Although postoperative echocardiography 5 months
after the operation showed normal cardiac function without ventricular asynergy, the dilated and aneurysmal LAD remained unchanged.
Keywords: Coronary artery fistula • Newborn • Coronary artery aneurysm
With recent improvements in foetal cardiac Doppler colour flow
mapping, coronary artery fistula (CAF) is often diagnosed during
the prenatal period, and some newborns require surgical or
catheter-based intervention in neonatal period [1–4]. The
optimal timing and the method of treatment depend on the size
and location of communication between the coronary artery
and the cardiac chamber. However, surgical intervention for car-
diopulmonary collapse immediately after birth is required in
some cases. We report a case of a gigantic CAF from left anterior
descending (LAD) coronary artery to right ventricular apex, for
which we successfully performed surgical CAF closure under car-
diopulmonary bypass within 1 h after birth.
Foetal echocardiography at 38 weeks of gestation revealed a
dilated and partial aneurysmal LAD (8–10 mm), communicating
with the right ventricular apex through a 12-mm wide orifice
(Fig. 1A). Sudden cardiopulmonary collapse soon after birth
caused by severe coronary steal condition along with a physio-
logical decrease in pulmonary arterial pressure was highly antici-
pated, thus elective caesarean (C)-section procedure and
subsequent surgical repair were planned.
The child was delivered by elective C-section at 40 weeks of
gestation with a birth weight of 3564 g, and then the surgical
repair was started 50 min after birth. Using a median sternot-
omy, the dilated, aneurysmal and tortuous LAD was identified,
and the distal portion was found to be in communication with
the right ventricle (RV) apex (Fig. 2A and B). We attempted to
compress the terminal end of LAD to assess whether the direct
closure of CAF from the outside was possible. However, ST
waves on the II and V5 leads elevated and blood pressure
decreased, which indicated that a clinically significant accessory
diagonal branch was arising from nearby fistula. Hence, we
initiated cardiopulmonary bypass. Antegrade cardioplegia was
then infused after aortic cross-clamping by pressing the fistula
with a finger.
The aneurysmal terminal end of LAD was incised open under
cardioplegic arrest and a 12-mm wide fistula was identified. We
closed the orifice of fistula with four pairs of 6-0 polypropylene
mattress sutures (Fig. 2C), then repaired the opened LAD using
6-0 polypropylene mattress sutures with fresh autopericardial
strips, which were reinforced with 5-0 polypropylene continuous
After weaning from cardiopulmonary bypass, systolic pulmon-
ary arterial pressure was 48 mmHg and systemic systolic blood
pressure was 37 mmHg. Thus, extracorporeal life support was
initiated to treat the over-systemic pulmonary hypertension and
then successfully removed 4 days later. The postoperative course
was uneventful and the patient was discharged at 2 months of
age without any symptoms. Five months after surgery, echocar-
diography showed that both ventricles were normally sized and
functioning, without any ventricular wall motion asynergy and a
© The Author 2012. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.
Interactive CardioVascular and Thoracic Surgery 15 (2012) 520–522
doi:10.1093/icvts/ivs240 Advance Access publication 29 May 2012
CASE REPORT - CONGENITAL
tiny residual shunt from the LAD artery to the RV. On the other
hand, cardiac computed tomography revealed that the dilated
and aneurysmal LAD had not regressed (Fig. 1B). Presently,
Coumadin administration is being continued with a target pro-
thrombin time–international normalized ratio of 1.5, according
to the protocol for the treatment of coronary artery aneurysm
associated with Kawasaki disease.
A CAF is known to range from asymptomatic with spontaneous
closure to a life-threatening condition. Although rare, it should
not be overlooked that some patients in this anatomical group
develop sudden heart and/or respiratory failure soon after
birth because of a severe form of the lesion. In such cases, only
quick closure of the fistula allows the survival of the affected
A result of the presence of such a fistula is that physiological
pulmonary hypertension is severe and surgical intervention
might be delayed. In our experience, the coronary artery con-
necting CAF can have some stenotic portions that regulate blood
flow shunted from the coronary artery to cardiac chambers.
However, the present patient had a non-obstructed rather than
an excessively dilated LAD coronary artery. In fact, the diameter
of LAD was much larger than that of ascending aorta. No
Figure 1: (A) Foetus echocardiography showing the dilated and partially aneurysmal LAD coronary artery, communicating with the right ventricle at the apex
through a 10-mm wide fistula (white arrows). (B) Three-dimensional computed tomography image obtained at 5 months after the operation showing that the
dilated and aneurysmal LAD coronary artery has not regressed.
Figure 2: (A) Intraoperative macroscopic image and (B) related illustration showing the dilated and aneurysmal LAD coronary artery (white arrows). (C) An incision
was made on the aneurysmal terminal end of LAD to explore the 12-mm wide CAF. The CAF was then closed with four pairs of 6-0 polypropylene mattress
sutures. RV, right ventricle; LV, left ventricle; Ao, aorta; PA, pulmonary artery.
Matsumoto et al. / Interactive CardioVascular and Thoracic Surgery
previously published reports have noted such a CAF, thus we Download full-text
were concerned that an unexpected systemic and/or coronary
steal condition might develop. As a result, we decided to
perform surgical intervention immediately after finding the ECG
change. We believe that undertaking the urgent operation soon
after birth was justified and, along with assistance from the col-
laborating medical team, greatly contributed to the rapid suc-
cessful treatment of the present patient. The fate of the dilated
and aneurysmal LAD remains unclear. Because of the risk of
rupture or thrombus formation, long-term follow-up examina-
tions and continual anticoagulation therapy are mandatory .
In summary, we treated a newborn with a gigantic CAF from
LAD to RV with successful surgical closure within 1 h after birth.
Cardiopulmonary bypass and cardioplegic arrest were required
so as to not injure the branching diagonal coronary artery ori-
ginating from the nearby fistula. Postoperative echocardiography
findings revealed normal cardiac function without ventricular
asynergy, while computed tomography showed that dilation and
aneurysmal change of LAD was not regressed.
Conflict of interest: none declared..
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