Abstract
Some coronary artery anomalies are associated with increased risk of sudden cardiac death and myocardial infarction in young patients. There are few data on the clinical and prognostic relevance of isolated origin of the left circumflex artery from the pulmonary artery, an extraordinarily rare variant of anomalous left coronary artery from the pulmonary artery. (Level of Difficulty: Intermediate.)
Key Words: ALCAPA, anomalous circumflex artery, coronary anomaly, sudden cardiac death
Abbreviations and Acronyms: CHD, congenital heart disease; ECG, electrocardiogram; LCxPA, left circumflex from the pulmonary artery; SCD, sudden cardiac death
Graphical abstract
Some coronary artery anomalies are associated with increased risk of sudden cardiac death and myocardial infarction in young patients. There are few…
History of Presentation
A 39-year-old man was admitted to the emergency department with long-lasting palpitations preceded by abdominal pain, vomiting, and diarrhea. At presentation, the patient was afebrile and hemodynamically stable, with an unremarkable physical examination. The electrocardiogram (ECG) showed sinus rhythm without ST-segment deviation. There was no alteration in blood test results despite a slightly elevated C-reactive protein. In serial determination of markers of myocardial damage, there was an elevation of high-sensitivity troponin I (5.28 ng/ml).
Learning Objectives
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To make a differential diagnosis of myocardial damage in young adults with multimodality imaging.
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To relate coronary anomalies with other CHD.
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To understand the importance of combining clinical presentation, past medical history, and clinical impact of the findings to select the best approach when high-quality published reports are lacking.
Past Medical History
The patient had aortic coarctation partially corrected during childhood with a bypass between the left subclavian artery and the descending thoracic aorta. He underwent reoperation with a Hemashield (Maquet, Rastatt, Germany) aortic graft implantation at 24 years of age. Follow-up cardiac magnetic resonance confirmed a good result of the aortic graft but significant stenoses at the proximal and distal anastomosis of the subclavian-to-aortic bypass (Figures 1A and 1B). He was previously diagnosed with type 1 bicuspid aortic valve with normal function (Video 1). He had no known cardiovascular risk factors.
Figure 1.
Magnetic Resonance Angiography
(A) Volume-rendering technique. (B) 2-dimensional magnetic resonance angiography. Mild dilatation of ascending aorta (white arrows), subclavian-descending aorta bypass (blue arrows) with stenosis at the proximal and distal anastomoses. Permeable Hemashield (Maquet, Rastatt, Germany) graft (asterisks).
Online Video 1.
Short-Axis View Showing Bicuspid Aortic Valve
Differential Diagnosis
In this scenario, different entities must be considered in the presence of elevated troponin, such as myocarditis or acute myocardial infarction type 1 or type 2.
Investigations
First, a transthoracic echocardiogram demonstrated normal left ventricular ejection fraction with no wall motion abnormalities (Videos 2, 3, and 4), bicuspid valve normal function (Video 5), and absence of recoarctation (Video 6, Figure 2). Because of his previous symptoms of viral infection, cardiac magnetic resonance was requested to rule out myocarditis, and the imaging showed not only normal left ventricular systolic function but also no edema (Video 7) or fibrosis (Video 8). To complete the differential diagnosis, cardiac computed tomography angiography was performed. This technique demonstrated the presence of left circumflex artery origin from the pulmonary artery (LCxPA) (Video 9, Figures 3A and 3B) without significant stenosis (Figure 3C). Small aortobronchial fistulous communications (Figure 3D) were noted as remnants of aortic coarctation. Finally, invasive cardiac catheterization confirmed the diagnosis by showing that the left circumflex artery had good retrograde filling through collateral vessels from the left anterior descending and right coronary arteries (Videos 10 and 11).
Online Video 2.
Apical 4-Chamber View
Online Video 3.
Apical 2-Chamber View
Online Video 4.
Apical 3-Chamber View
Online Video 5.
Long Parasternal-Axis View
Online Video 6.
Suprasternal-Axis View
Figure 2.
Doppler Echocardiography of the Descending Aorta
Peak systolic velocity is 161 cm/s, and peak systolic gradient is 10 mm Hg.
Online Video 7.
Cardiac magnetic resonance T2-weighted sequence.
Short-axis view.
Online Video 8.
Cardiac magnetic resonance Late Gadolinium Enhancement Sequence
Short-axis view.
Online Video 9.
Reconstruction 3-Dimensional Coronary Computed Tomography Angiography
Left circumflex artery arising from the pulmonary artery.
Figure 3.
Coronary Computed Tomography Angiography Reconstruction Images
(A and B) Left circumflex (LCX) artery origin from the right pulmonary artery (RPA). (C) Curved multiplanar image of the left circumflex artery. (D) Coronal plane of aortobronchial fistulas.
Online Video 10.
Left Anterior Descending Artery Catheterization With Retrograde Filling of Left Circumflex Artery
Online Video 11.
Right Coronary Artery Catheterization With Retrograde Filling of Left Circumflex Artery
Management
During hospitalization, no arrhythmias were detected in continuous ECG monitoring. In the absence of chest pain or new clinical events, the patient was considered at low risk for sudden cardiac death (SCD). He was conservatively managed and discharged to be closely followed.
Follow-Up
The patient has been followed up for 1 year. A recent treadmill test with single-photon emission computed tomography with sestamibi was informed as clinical and ECG negative but showed mild basal inferolateral ischemia on perfusion imaging (Figures 4A and 4B). Given the absence of symptoms and the limited perfusion defect, expectant management was maintained.
Figure 4.
SPECT-MIBI Stress-Testing Bullseye
(A) Peak of stress. Tracer hypocaptation in basal-inferolateral wall resulting from exercise-induced ischemia (asterisk). (B) At rest, perfusion tracer uptake increases (asterisk). Ant = anterior; Endo = endocardium; Epi = epicardium; Lat = lateral; Sep = septal; SPECT-MIBI = single-photon emission computed tomography sestamibi.
Discussion
The clinical implications of LCxPA are not well established. Garcia et al. (1) first described it in adults in 1992, in a patient with no other congenital heart disease (CHD) who reported dyspnea and chest pain at rest. Recently, few additional cases have been described in adults: 2 presented as SCD, 1 with double-outlet right ventricle (2), and 1 without CHD (3), and another patient presented with mild exertional chest pain, wall motion abnormalities noted on echocardiography, and aortic coarctation (4). In contrast, in the larger experience with LCxPA among pediatric patients (5, 6, 7), the presentation was mostly exertional chest pain and dyspnea, and the association with other CHDs was higher. Two main questions arise from the scarce data described in published reports. First, given the higher life expectancy of patients with CHD nowadays, coronary anomalies, and this anomaly in particular, should be considered in the differential diagnosis of exertional symptoms or SCD. In this regard, noninvasive imaging techniques comprise the first diagnostic approach, requiring also invasive angiography in some cases, which enables physicians to: 1) assess adequate retrograde flow from collateral vessels; and 2) have a basal angiogram for comparison in the middle to long term in case the patient presents with extensive myocardial ischemia. In contrast, there are no consensus and guidelines defining the management of LCxPA. Although the latest guidelines (8) recommend surgery for asymptomatic anomalous left coronary artery from the pulmonary artery, they do not specify guidance on variants such as LCxPA; therefore, the only evidence is based on case reports. In these cases, adult patients presented with resting angina, dyspnea, New York Heart Association functional class III or IV, or aborted cardiac death in shockable rhythm were surgically treated. Only 1 reported patient (9) had angina and no pathological findings on ECG or echocardiography but was surgically treated. However, most of the adults described in published reports have had no previous heart operations (3,5, 6, 7,9,10). Besides, survival depends on collateral development (5,9), and surgery is recommended when symptoms are attributed to ischemia (10). Conversely, our patient reported palpitations with no chest pain on admission, and although findings of single-photon emission computed tomography with sestamibi were slightly positive for ischemia, the treadmill test result was clinically negative at high load, and he remained free from angina during follow-up. This patient had considerable collateralization, 2 previous thoracic surgical procedures, and symptoms that could be attributed to supraventricular tachycardia triggered by relative hypovolemia secondary to gastroenteritis. After careful consideration, we chose conservative management and close follow-up. Certainly, we cannot ensure completely the appropriateness of our management. Nonetheless, we present the case of a patient who, more than a year after the diagnosis, has no symptoms or cardiovascular rehospitalizations. This outcome supports medical treatment as an option in selected scenarios.
Conclusions
LCxPA is a rare coronary anomaly, but it has been described in association with CHD, especially aortic coarctation. Awareness of this entity will enable early diagnosis and provide potential prognostic benefit. Furthermore, there is a gap of evidence regarding the best treatment of this anomaly. Although cardiac surgery has been extensively used, our case shows that conservative management may be an option in some circumstances.
Footnotes
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the JACC: Case Reportsauthor instructions page.
Appendix
For supplemental videos, please see the online version of this paper.
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