Abnormal Origin of the Left Coronary Artery From the Pulmonary
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Case Report
Abnormal Origin of the Left Coronary
Artery From the Pulmonary Artery
Discovered at Age 67: What to Do?
Fadoua Lachhab, MD
1
, Rachida Amri, MD
1,2
,
Loubna Mahfoudi, MD
1
, and Said Moughil, MD
1
Abstract
Anomalous left coronary artery from the pulmonary artery (ALCAPA) was described by Abbott in 1908. In most cases, it is an
isolated lesion, being the most common cause of myocardial ischemia in children. The associated mortality rate without inter-
vention reaches 90% during childhood. We report the case of a 67-year-old woman, who underwent coronary angiography for
investigation of atypical chest pain and was found to have ALCAPA. The patient refused surgery and has remained asymptomatic
on a medical regimen.
Keywords
adult congenital heart disease, anomalous coronary artery, ischemia, quality of life
Submitted November 17, 2017; Accepted March 27, 2018.
Introduction
Anomalous left coronary artery from the pulmonary artery
(ALCAPA) is a congenital malformation first described by
Abbott in 1908 following the autopsy of a 60-year-old patient
who died suddenly. In 1933, Bland, White, and Garland
described the clinical syndrome associated with this congenital
heart disease through the observation and autopsy of a child
who died of a myocardial infarction, leading to the name of
Bland-White-Garland’s syndrome.
1
Most often an isolated lesion, ALCAPA is the most common
cause of myocardial ischemia in children. Its reported fre-
quency of 0.24% to 0.46% of all congenital heart disease may
be a slight underestimate, in view of the fact that many patients
die suddenly without any explainable cause.
2
The death rate in the absence of surgical correction
reaches 90% during childhood. Survival in remaining 10%
of patients is felt to be the result of devel opment of an
extensive intercoronary collateral circulation, frequently in
the setting of a larg e domin ant rig ht coro na ry arte ry. Nev-
ertheless, approximate ly 90% die s uddenly at an average
age of 35.
Few patients reach age 50 years without surgery. A review
of existing literature shows that only 26 have been reported to
this point and only 2 were over 60 years old. These data are
generally interpreted as being supportive of a strategy whereby
surgical correction is recommended and planned as soon as the
diagnosis is made, even in the absence of symptoms.
Clinical Observation
A 67-year-old woman was referred for evaluation of atypical
chest pain. During her childhood, she had experienced episodes
of dyspnea, unexplained and never explored, and had under-
gone three surgeries under general anesthesia without any
record of untoward incidents. Clinical examination revea led
neither heart murmur nor signs of heart failure. The chest radio-
graph showed a V2 cardiomegaly. The resting electrocardio-
gram revealed a sinus rhythm without signs of myocardial
ischemia. The transthoracic echocardiogram showed moderate
left ventricular hypertrophy without dilatation (end-diastolic
diameter 45 mm) and no abnormality of global or segmental
left ventricular function. Left ventricular ejection fraction was
60%. Mild mitral regurgitation was noted, with a hyperecho-
genic appearance of the posterior mitral pillar (Figure 1). An
important intercoronary collateral circulation was visualized by
color flow mapping (Supplemental Video 1). On a short-axis
view, the right coronary artery appeared dilated and the left
1
Department of Cardiovascular Surgery B, Mohammed V University, Rabat,
Morocco
2
Department of Cardiology B, Mohammed V University, Rabat, Morocco
Corresponding Author:
Fadoua Lachhab, App 9, Residence Amal, rue Kaki, Mahaj Riad, Rabat 11100,
Morocco.
Email: fadoulachhab@hotmail.com
World Journal for Pediatric and
Congenital Heart Surgery
1-3
ª The Author(s) 2018
Article reuse guidelines:
sagepub.com/journals-permissions
DOI: 10.1177/2150135118772834
journals.sagepub.com/home/pch
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