Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery in Adults: A Comprehensive Review of 151 Adult Cases and A New Diagnosis in a 53‐Year‐Old Woman

Abstract

Background: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary abnormality associated with early infant mortality and adult sudden death. As it predominantly presents in the first year of life, diagnosis in living adults is extremely rare. Current management is based on limited case series or extrapolated from pediatric cases. Modern advances in noninvasive cardiac imaging have substantially increased the number of diagnoses, uncovering a large adult population that has not been reviewed. Hypothesis: The availability of newer diagnostic modalities correlates with an increasing incidence in an older cohort, and true association between sudden death and ALCAPA may be lower, especially among older patients. Methods: A comprehensive literature search was performed for all case reports of ALCAPA on MEDLINE and PubMed using the keywords ALCAPA, Bland-White-Garland, and coronary anomaly; and augmented by references from published case reports from 1908 to 2008. All adult cases, defined by age 18 years and older, were reviewed for this article. Results: One hundred fifty-one adult cases of ALCAPA are described, in addition to the case of an asymptomatic 53-year-old woman. The average reported age was 41 years old with the oldest being 83. Sixty-six percent of the patients presented with symptoms of angina, dyspnea, palpitations, or fatigue; 17% presented with ventricular arrhythmia, syncope, or sudden death; and 14% were asymptomatic. Twelve percent were diagnosed at autopsy. The majority had some form of surgical correction during their clinical course. Conclusions: ALCAPA is a rare and life-threatening condition in adults. The availability of newer, less invasive diagnostic modalities has resulted in more frequent identification of this condition in an older cohort.

Anomalous origin of the left coronary artery from the pulmonary artery: case report and review

“…8 However normal echocardiograms may be seen in 10% of patients and the presence of ischaemia in stress ECG or imaging such as in our patient is well documented. 1 Coronary angiography alludes to the diagnosis with the presence of a large tortuous RCA with collaterals filling the LCA system as seen with our patient, figure 1. Non-invasive imaging modalities such as cardiac CT and or MRI allow visualization of the origin of the LCA from the PA, dilated tortuous RCA and collateral arteries with CMR having the advantage of being able to assess myocardial viability and ischaemia.…”

“…1 True incidence of older patients are not know with only case reports of patients older than 50 years of age. 2,3 Krause 4 and Brooks 5 first described anomalous arteries from the pulmonary artery in 1865 and 1885 respectively from post mortem examinations.…”

“…17% presented with ventricular arrhythmia, syncope, or sudden death and 14% of patients were asymptomatic. 1 ECGs often show lateral ischaemia or evidence of previous anterior myocardial infarction with presence of Q waves in as many as 50% of patients and left ventricular hypertrophy being seen in up to 28% with 4% of patients having a normal ECGs. 1 A dilated RCA with retrograde Doppler flow from LCA to PA with visualisation of septal flow due to collaterals have been described as diagnostic echocardiographic criteria for ALCAPA.…”

“…1 ECGs often show lateral ischaemia or evidence of previous anterior myocardial infarction with presence of Q waves in as many as 50% of patients and left ventricular hypertrophy being seen in up to 28% with 4% of patients having a normal ECGs. 1 A dilated RCA with retrograde Doppler flow from LCA to PA with visualisation of septal flow due to collaterals have been described as diagnostic echocardiographic criteria for ALCAPA. 8 However normal echocardiograms may be seen in 10% of patients and the presence of ischaemia in stress ECG or imaging such as in our patient is well documented.…”

Anomalous Coronary Artery Found in the Syncopal Workup of an Elderly Man

“…2 A recent case series of 151 adult patients with anomalous coronary arteries found that the mean reported age was 41 years, with 17% presenting with life-threatening conditions such as ventricular arrhythmia, syncope, or sudden death. 10 Coronary artery with an anomalous origin represents a rare yet important cause of syncope. The left coronary artery or the left main artery typically arises from the left sinus of Valsalva and branches into the left anterior descending and left circumflex arteries.…”