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\title{ASYMPTOMATIC ANOMALOUS LEFT CORONARY ARTERY FROM THE PULMONARY ARTERY
(ALCAPA) IN AN ADULT.}



\author[1]{Demetrio Sharp Dimitri}%
\author[2]{Andrew  Panakos}%
\author[3]{Carlos Alfonso}%
\author[2]{Vicente Orozco-Sevilla}%
\affil[1]{University of Cincinnati Academic Health Center}%
\affil[2]{University of Miami Hospital and Clinics}%
\affil[3]{University of Miami, Miller School of Medicine}%


\vspace{-1em}



  
  \date{April 29, 2026}


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\begin{abstract}
We describe a patient in whom this anomaly was diagnosed during cardiac
catheterization for evaluation of progressive shortness of breath and
mild to moderate mitral regurgitation founded on a transthoracic
echocardiogram.A 58-year-old hypertensive female was evaluated for
progressive shortness of breath. Her physical examination showed a
regular heart rate and rhythm, with normal heart sounds with a
holosystolic systolic murmur on the 4th intercostal midclavicular line
on auscultation. Electrocardiography showed normal sinus rhythm and
criteria and possible left ventricular hypertrophy. Stress
Echocardiogram showed with no evidence of ischemia preserved left
ventricular function with an ejection fraction of 55-60\%, with mild to
moderate mitral regurgitation. Patient underwent a coronary
catheterization for persistent shortness of breath which indicated no
evidence of obstructive coronary disease, normal left ventricular
function with mild to moderate mitral insufficiency. Angiography of the
right coronary artery showed a very large and tortuous artery that
collateralizes into the left main system and supplies the entire left
coronary artery system. The left main coronary origin was from the
pulmonary artery. CTA confirmed the same findings. The recommendations
between cardiology and cardiothoracic surgery were to maximize medical
therapy and follow up due to lack of myocardial ischemia or severe
valvular disease. Currently there are no guidelines or gold standard
management for patients with adult form of ALCAPA that present without
ischemic burden or for patients with the disease that present
asymptomatic with significant coronary collateralization.%
\end{abstract}%



\sloppy


\emph{Title:}

\textbf{Asymptomatic Anomalous Left Coronary Artery from the Pulmonary
Artery (ALCAPA) in an Adult.}

\emph{Authors:}

Demetrio Sharp Dimitri MD\textsuperscript{1}, Andrew Panakos
MD\textsuperscript{2}, Carlos E Alfonso MD\textsuperscript{2}, Vicente
Orozco-Sevilla MD\textsuperscript{1}.

\emph{Institution:}

\textbf{University of Cincinnati Medical Center, Department of Internal
Medicine.\textsuperscript{1}}

\textbf{University of Miami Health, Department of Cardiovascular
Disease.\textsuperscript{2}}

\emph{Corresponding Author:}

Demetrio Sharp Dimitri, MD

Department of Internal Medicine

University of Cincinnati Medical Center, Department of Internal Medicine

Email: sharpddo@ucmail.uc.edu

\emph{Disclosure:}

Demetrio Sharp Dimitri MD: None.

Andrew Panakos MD: None.

Carlos E Alfonso MD: None.

Vicente Orozco-Sevilla MD: None

\textbf{Word count:} 629

\textbf{Keywords: ALCAPA, Mitral valve regurgitation, congenital cardiac
defect, coronary anomaly}

\textbf{Summary}

Anomalous origin of the left coronary artery from the pulmonary artery
(ALCAPA) is considered a rare and very fatal congenital coronary artery
anomaly. It is estimated that this congenital abnormality affects 1 of
every 300,000 live births and accounts for 0.25\% of all congenital
heart defects worldwide. ALCAPA manifests itself most commonly in infant
population and it has a 1-year mortality rate of approximately 90\%,
therefore it is not often considered a diagnosis in the adult population
(4, 5). When it presents in adult it is considered an important cause of
sudden cardiac death (1, 2). We describe a patient in whom this anomaly
was diagnosed during cardiac catheterization for evaluation of
progressive shortness of breath and mild to moderate mitral
regurgitation founded on a transthoracic echocardiogram (3).

\textbf{CASE}

A 58-year-old otherwise hypertensive female that was evaluated because
of her progressive shortness of breath and mild to moderate mitral
regurgitation founded on a transthoracic echocardiogram.

Her physical examination showed a regular heart rate and rhythm, with
normal heart sounds with an II/III holosystolic systolic murmur on the
4\textsuperscript{th} intercostal midclavicular line, no rubs or gallops
were present on auscultation. No lower extremity pitting edema was
noticed.

Electrocardiography showed normal sinus rhythm and criteria and possible
left ventricular hypertrophy.

Stress Echocardiogram with no evidence of ischemia and preserved left
ventricular function with an ejection fraction of 55-60\%, left atrium
dilated with mild to moderate mitral regurgitation.

Patient underwent a coronary catheterization which indicated no evidence
of obstructive coronary disease, normal left ventricular function with
mild to moderate mitral insufficiency. Angiography of the right coronary
artery showed a very large and tortuous artery that collateralizes into
the left main system and supplies the entire left coronary artery
system. The left main coronary origin was from the pulmonary artery. CTA
confirmed the same findings.

The recommendations between cardiology and Cardio Thoracic surgery were
to maximize medical therapy including beta blockers and diuresis and
close follow up due to the lack of myocardial ischemia,severe valvular
disease or heart failure.

\textbf{Discussion.}

ALCAPA is a very rare condition in which the left main coronary artery
originates on the pulmonary artery. The decreased flow into the left
coronary arteries due to coronary artery steal from the low pulmonary
artery resistance leads to ischemia and infarction of the myocardial
tissue an usually is lethal if is not corrected as an infant (3).

The infant type of ALCAPA is considered to be very fatal if untreated
with a 1-year mortality of approximately 90\% (5). Infants commonly
present with failure to thrive, breathing difficulties, irritability,
and distress while crying or eating. The adult type of ALCAPA is related
to sudden cardiac death and symptoms of ischemia, mitral valve
regurgitation, and left ventricular dysfunction (3,4).

The treatment of the ALCAPA infant type is absolutely surgically with
either two-coronary system coronary button transfer, Takeuchi procedure
(trans- pulmonary baffling), coronary artery bypass grafting (CABG)
combined with ligation of the anomalous left coronary artery (4,5).
ALCAPA Adult type treatment is sometimes controversial, although the
preferred approach is surgical including CABG combined with anomalous
left coronary artery ligation and patch of the pulmonary artery (4,5).
This approach should only be considered if the patient adult has or
develops evidence of ischemia, severe mitral regurgitation or heart
failure secondary to a significant shunt and volume overload (4).

Currently there are no guidelines or gold standard management for
patients with adult form of ALCAPA that present with minimal to
non-disease of the myocardium? How can we monitor the disease course and
possibly risk stratify patients for possible complications? Is there a
screening benefit for patients with high probability of ALCAPA? How much
real benefit does the surgical intervention really offers to this
patient's population group? What type of conduit is appropriate if CABG
planned vein or double mammary. This case raises questions regarding the
management of this rare congenital coronary anomaly

Reference:

1.- Alexi-Meskishvili V, Berger F, Weng Y, lange pE, Hetzer R. anomalous
origin of the left coronary artery from the pulmonary artery in adults.
J Card surg 1995; 10: 309--315.

2.- Arciniegas E, Farooki ZQ, Hakimi M, Green EW. Management of
anomalous left coronary artery from the pulmonary artery. Circulation
1980;62(2 pt 2):I180--I189.

3- Fernandes ED, Kadivar H, Hallman GL, Reul GJ, Ott DA, Cooley DA.
Congenital malformations of the coronary arteries: the Texas Heart
Institute ex- perience. Ann Thorac Surg 1992;54:732--740.

4.- H. Wesselhoeft, J.S. Fawcett, A.L. Johnson. Anomalous origin of the
left coronary artery from the pulmonary trunk: Its clinical spectrum,
pathology, and pathophysiology, based on a review of 140 cases with
seven further cases. Circulation, 38 (1968), pp. 403-425.

5.- Ishihata T, Takeda H, Katohno E, et al. An adult case of
Bland-White-Garland syndrome with col- laterals from the bronchial
artery. Heart Vessels 1994;9:218--222.

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