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\begin{document}

\title{A rare case of Left Main Coronary Artery Atresia presenting as Dilated
Cardiomyopathy and misdiagnosed as ALCAPA}



\author[1]{Bengisu Karakose}%
\author[1]{Serdar Başgöze}%
\author[2]{Ersin Erek}%
\affil[1]{Acibadem Universitesi Tip Fakultesi}%
\affil[2]{Acibadem University}%


\vspace{-1em}



  
  \date{May 27, 2024}


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\selectlanguage{english}
\begin{abstract}
This case report presents an extremely rare case of left main coronary
artery atresia in a 14-week-old female infant presenting with severe
symptoms of dilated cardiomyopathy.%
\end{abstract}%



\sloppy


A rare case of Left Main Coronary Artery Atresia presenting as Dilated
Cardiomyopathy and misdiagnosed as ALCAPA

Bengisu Karakose\textsuperscript{1}, Serdar Basgoze\textsuperscript{2},
Ersin Erek\textsuperscript{2}

\textsuperscript{1} School of medicine, Acibadem Mehmet Ali Aydinlar
University, \selectlanguage{polish}İ\selectlanguage{english}stanbul, Turkey

\textsuperscript{2} Department of Pediatric Heart Surgery, Faculty of
Medicine, Atakent Hospital, Acibadem Mehmet Ali Aydinlar University,
\selectlanguage{polish}İ\selectlanguage{english}stanbul, Turkey

\textbf{Corresponding Author:} Serdar Basgoze, MD

\textbf{Address:} Department of Pediatric Cardiac Surgery, Faculty of
Medicine, Acibadem Mehmet Ali Aydinlar University, 34303 Halkali,
Istanbul, Turkey.

\textbf{Phone number:} +90 533 626 03 50, +90 212 404 41 77

\textbf{Email address:}basgozeserdar@gmail.com

ORC\selectlanguage{polish}İ\selectlanguage{english}D number of the corresponding author: http://orcid.org/
0000-0002-6146-2095

IRB approval, consent statement and clinical trial registration are not
applicable for this study.

Summary

This case report presents an extremely rare case of left main coronary
artery atresia in a 14-week-old female infant presenting with severe
symptoms of dilated cardiomyopathy.

\emph{Introduction}

Coronary artery anomalies are relatively uncommon congenital disorders
of the coronary artery anatomy. The incidence of coronary artery
anomalies has been reported to be 0.6--1.3\%. {[}1{]} Left main coronary
artery atresia (LMCAA) is the rarest form of congenital coronary
malformations, in which coronary ostium and main trunk in the left
coronary artery system is absent {[}2{]}. Thus, blood flows from the
right coronary artery to the left coronary artery through collateral
vessels {[}3{]}. Clinical presentation, management, and prognosis of
this disease depend on the characteristics of the collaterals and native
vessels {[}4{]}. Hereby, we report a case of congenital atresia of the
left main coronary artery, misdiagnosed as Anomalous left coronary
artery from the pulmonary artery (ALCAPA).

Case Presentation

A 14-week-old female infant, who had respiratory distress worsened after
bronchiolitis, was admitted to the intensive care unit. She was
intubated in another hospital before the admission. On the first
evaluation, the patient was cyanotic and had a cutis marmorata
appearance on her skin. Capillary refill time was 4 seconds, and her
pulse was week. She had tachycardia and bilaterally crepitant rales.
According to the echocardiographic examination, the left atrium and left
ventricle were greatly enlarged and left ventricular contractions were
markedly decreased (Shortening Fraction:13\%), thus indicating a dilated
cardiomyopathy. Figure 1 represents the right ventricle (RV) stuck
between the sternum and the left ventricle (LV). Septum movements were
better than the posterior LV wall. A moderate degree of insufficiency
was observed in the mitral and tricuspid valve. Pulmonary arterial
pressure was measured to be approximately 60 mmHg through the tricuspid
valve insufficiency. There was also a mild degree of insufficiency in
the aortic valve. Pulmonary and aortic flows were decreased due to low
cardiac output. Since she had a cardiogenic shock, IVIG, hydrocortisone,
and inotropic support drugs were started with the preliminary diagnosis
of myocarditis. The patient was placed on extracorporeal membrane
oxygenation (ECMO)~due to the low cardiac output leading to multiorgan
failure using neck vessels. After two weeks on ECMO support, the patient
was successfully weaned and separated from ECMO support. Both Coronary
Multi-detector Computed Tomography (MDCT) and heart catheterization were
performed with suspicion of ALCAPA syndrome. Figure 2 shows the coronary
arteries anatomy.

The shadow indicated by the black arrow may appear as the pulmonary
cusp, which the left coronary artery drains, in Figure 3. Although
origin of the left coronary artery from aorta or pulmonary artery, could
not be visualized, the patient underwent operation for left coronary
artery revascularization, with the preoperative possible diagnosis of
ALCAPA. After a midline sternotomy, the thymus was resected, and the
pericardium was opened. After heparinization, aortic and bicaval
cannulation was performed to initiate cardio-pulmonary bypass (CPB). The
pulmonary artery and aorta were dissected from each other. Diastolic
cardiac arrest was provided with 30\selectlanguage{ngerman}°C systemic hypothermia and antegrade
tepid blood cardioplegia after cross-clamping. Tepid blood cardioplegia
was repeated every 20 minutes until releasing cross-clamp. The left side
of the heart was vented. Pulmonary arteriotomy and aortotomy were
performed. There were no left coronary ostia neither in the pulmonary
artery nor in the aorta.

The Circumflex artery (Cx) and left anterior descending artery (LAD)
were visible, but small in diameter. They were merged into a small blind
pouch, 2 cm away from the left coronary sinus of the aorta. There was no
possibility for preparation and anastomosis to the aorta, due to very
small diameter and fragile vessel. Left internal mammary artery (LIMA)
was harvested. LAD, near to the pouch was opened, LIMA-LAD anastomosis
was performed with 8-0 prolene. Pulmonary artery and aortotomy were
closed. Cross clamp was removed after de-airing. The patient was weaned
from CPB with high dose s of inotropic support including, adrenaline,
noradrenaline, dopamine, and milrinone. The sternum was left open due to
hemodynamic instability. Cardiopulmonary bypass time was 141 minutes,
and cross-clamp time was 101 minutes.

The patient needed ECMO support due to the low cardiac output on the
second postoperative day. The patient died of massive cranial hemorrhage
on ECMO, despite some improving ventricular function in the 10th
postoperative day.

Discussion

LMCAA is a quite rare condition with unclear etiology. In LMCAA, the
left coronary system receives blood only by collateral arteries from the
RCA. Thus, the heart may eventually be unable to cope with collateral
circulations and develop myocardial ischemia. Patients mostly present
with nonspecific symptoms depending on their age group and the formation
of collateral vessels from the right coronary artery to the left
coronary artery. Adult patients usually present with angina pectoris at
an advanced age when collateral flow cannot keep pace with myocardial
demands. Children and adolescents often present with chest pain,
dyspnea, syncope and tachyarrhythmia, and sudden cardiac death {[}2,
3{]}. Infants mostly present with growth retardation and myocardial
infarction {[}2{]}. Catastrophic situations like sudden cardiac arrest,
low cardiac output, and cardiomyopathy, as seen in our case, can also be
the first symptoms of infants with LMCAA. {[}5, 6{]} Since these
symptoms are not specific to LMCAA, the clinical diagnosis might be
neglected. Thus, other coronary anomalies should be excluded to reach a
correct diagnosis. As stated in a retrospective study done by Yildiz et
al., LMCA was the most common anomalous vessel {[}1{]}. Separate origins
of LAD and CX from the left coronary sinus of Valsalva were the most
seen anomaly and should be excluded in patients presenting with symptoms
of myocardial infarction. ALCAPA is one situation that LMCAA can be
confused with and should be differentiated from {[}2,7{]}.

Although congenital atresia of the left main coronary artery usually
occurs as an isolated cardiac lesion, concomitant anomalies including
bicuspid aorta, supravalvular aortic stenosis, right coronary ostial
stenosis, pulmonary stenosis, ventricular septal defect, and mitral
valve prolapsus secondary to myocardial ischemia can be seen {[}4, 8,
9{]}.

The diagnosis of LMCAA can be done by coronary angiographic findings,
which usually show no left coronary ostium and left coronary artery
filled in a retrograde manner via the RCA instead of antegrade blood
flow. In recent years, MDCT has also played an essential role in
diagnosing LMCAA in older children and adults and can be used in
patients suspected of congenital coronary artery abnormalities {[}3,
10{]}. According to some researchers, MDCT provides more precise details
in a less invasive way than coronary angiography and is thus recommended
for evaluation of congenital coronary abnormalities. {[}11{]} MDCT not
only defines the anatomic course and the ostium shape, but also has less
complications as coronary spasm than conventional coronary angiography.
However, there is no reliable research reporting the use of MDCT in
infants with coronary anomalies. {[}9{]}

The prognosis of LMCAA is poor {[}2{]}. Due to the symptomatic nature of
LMCAA and the risk of sudden cardiac death, patients in the pediatric
population with LMCAA should undergo surgical intervention to restore
the antegrade flow to the left coronary system. Various surgical
interventions have been described and coronary artery bypass grafting
using the internal mammary artery or saphenous vein has been identified
as the treatment of choice, regardless of the caliber of left sided
vessels {[}4, 8, 12{]}. A LIMA graft for LMCAA appears to be a
reasonable early interventional approach with successful results 1 year
postoperatively. Nevertheless, due to the rarity of this disease,
long-term postoperative outcomes have not been reported yet {[}2, 4, 5,
8, 9, 11{]}. Alternative to bypass grafting, direct surgical
reimplantation or reconstruction of the left main coronary artery using
azygos vein have been described to provide the shortest and most
efficient way for blood to the myocardium. {[}6, 12, 13{]}. The
advantages they offer relative to bypass grafting are prevention of the
occlusion of main trunk, provision of the antegrade flow and avoidance
of competitive flow. After the application of these techniques,
reoperative surgery can be easily performed. However, application of
this technique for LMCAA is not common and the long-term outcomes of the
surviving patients were unknown {[}6, 12{]}.

Conclusion

LMCAA is an extremely rare congenital coronary anomaly in which the left
coronary ostium and left the main trunk in the left coronary artery
system are absent. Considering the severe symptoms such as myocardial
infarction or sudden cardiac death, surgical revascularization should
probably be the treatment of choice.

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