Congenital atresia of the left main coronary artery in an infant

Sohn, Su Ye; Jang, Gi Young; Choi, Byung Min

doi:10.1631/jzus.b0900361

Cited by 14 publications

(11 citation statements)

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“…Both infants and adult patients with left main coronary artery atresia have been reported [ 5 , 34 , 35 ]. Adults may present as patients of ischemic heart disease and only during angiography may be diagnosed to have left main atresia.…”

Section: Discussionmentioning

confidence: 99%

Surgical management of anomalous origin of coronary artery from pulmonary artery

Mishra

2021

Indian J Thorac Cardiovasc Surg

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Background Anomalous origin of coronary artery from pulmonary artery (AOCAPA), as is evident from the name, is defined as abnormal origin of either coronary artery from the pulmonary artery. The consequences vary in most cases and these anomalies lead to severe coronary hypo-perfusion and ventricular dysfunction. The common variants of this cardiac malformation are an anomalous origin of a left coronary artery from a pulmonary artery (ALCAPA) and anomalous origin of the right coronary artery from a pulmonary artery (ARCAPA). Another rare variant is left main coronary artery atresia that resembles ALCAPA in its mode of presentation. This article presents a single surgeon experience of managing this complex subset of the coronary anomaly from April 2006 to July 2019. Material and methods The 105 patients, who underwent surgery for AOCAPA from April 2006 to July 2019, have been included in the study. The patients have been analysed by follow-up echocardiography and electrocardiography (ECG) at our hospital by paediatric cardiologists. Out of 105 patients of AOCAPA, 98 (93.3%) patients underwent ALCAPA repair, of which 59 (60.2%) were males and 39 (39.7%) were females. Four out of five patients, who had an anomalous origin of the left coronary artery from the right pulmonary artery (ALCARPA), had an intramural aortic course. Three patients (3%) had left main coronary artery atresia and four patients (4%) had ARCAPA. It may be mentioned that seven infants (7.14%) and one adult patient (1%) underwent concomitant mitral valve repair. All the patients with ALCAPA, left main coronary artery atresia and ARCAPA, and 1 of the patients with ALCARPA, underwent coronary relocation. In four out of five patients with ALCARPA, unroofing of intraaortic intramural course was performed. Results Out of 105 patients of AOCAPA, 9 (8.5%) patients had in-hospital mortality. Five infants (5.0%) with ALCAPA and one patient (1%) with ALCARPA died in the post-operative period due to severe left ventricular dysfunction, mitral regurgitation (MR) and sepsis. One adult patient (1%) with ALCAPA, who underwent coronary relocation using in situ trap door technique and mitral valve (MV) repair, died due to massive intracranial bleeding. Two patients out of three (66.6%) with left main coronary atresia died in intensive care unit (ICU) after 3rd and 4th postoperative day, due to low cardiac output, severe ventricular dysfunction and severe MR. Patients were followed up for a median 5.9 years. Seven patients were lost to follow-up, including the sole survivor of left main coronary atresia, after a median follow-up of 4 years after surgery. Three patients underwent mitral valve replacement for progressive residual MR. There has been no late mortality. Conclusion AOCAPA is a rare congenital cardiac anomaly, which usually presents in infancy with left ventricular dysfunction and mitral valve regurgitation. Early diagnosis and surgical re-establishment of the dual coronary system has given gratifying results, with improvement in left ventricular function ...

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Section: Discussionmentioning

confidence: 99%

Surgical management of anomalous origin of coronary artery from pulmonary artery

Mishra

2021

Indian J Thorac Cardiovasc Surg

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“… 4 To date, coronary angiography has been essential for the definitive diagnosis of this disease, but in recent years, the usefulness of coronary angiographic CT has been reported. 5 Angiography has limitations in its diagnostic capabilities because of the lack of extraluminal evaluation. Differential diagnosis includes anomalous origin of right/LCA from pulmonary artery, congenital LCA stenosis, and so on, all of which can be differentiated by coronary angiography CT.…”

Section: Discussionmentioning

confidence: 99%

Sudden cardiac arrest of a 16-year-old boy with left main coronary artery atresia: a case report

Tomura¹,

Nakagami²,

Yamaguchi³

et al. 2020

European Heart Journal - Case Reports

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Background In many cases, the cause of exercise-induced cardiopulmonary arrest in young persons is thought to be fatal arrhythmia, and one of the causes is ischaemic heart disease. Left main coronary artery atresia (LMCAA) is an extremely rare disease in which there is a congenital defect of the left main coronary artery, causing heart failure and exercise-induced angina attacks at a young age. Thus, it is disease that should be differentiated when examining young persons with chest pain. Case summary A 16-year-old boy experienced sudden cardiopulmonary arrest during soccer practice, was brought to our hospital for emergency treatment after return of spontaneous circulation. Elective coronary angiography revealed findings indicating an osmium defect in the left coronary artery (LCA) and blood flow via collateral circulation from the right coronary artery. Contrast-enhanced coronary computed tomography (CT) angiography showed a defect in the LCA ostium and LMCAA was diagnosed in the patient. After coronary artery bypass grafting was performed, but the patient was discharged in an ambulatory state with a wearable cardiac defibrillator. Postoperative course has been favourable. Discussion Left main coronary artery atresia is an extremely rare disease in which there is a congenital defect of the left main trunk of the coronary artery and should be differentiated when encountering cases of heart failure or exercise-induced angina/arrhythmia attacks in young persons who are not at risk for atherosclerosis. Exercise electrocardiogram may show a false negative result, and therefore coronary CT is useful for diagnosis.

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“…Clinical diagnosis of LMCAA might be neglected due to the wide range of nonspecific presenting symptoms including chest pain in a young patient, dyspnea, syncope or aborted sudden cardiac death 2, 5, 6, 7. Patients could remain asymptomatic for a long time due to a well development collateral system.…”

Section: Discussionmentioning

confidence: 99%

“…Patients could remain asymptomatic for a long time due to a well development collateral system. LMCAA is often an isolated anomaly but association with other congenital heart defects such as pulmonary stenosis, bicuspid aortic valve, supravalvular aortic stenosis and ventricular septal defects has been described 2, 5. Selective coronary angiography is helpful but multislice CTA would provide more precise details in a less invasive manner and is therefore recommended for evaluation of congenital coronary abnormalities 8 .…”

Section: Discussionmentioning

confidence: 99%

“…The blood flow to the left heart chambers is provided by collaterals from the right coronary artery. The prognosis is poor due to occurrence of chronic myocardial ischemia and reultant myocardial dysfunction 1, 2. Management in adults consists of surgical revascularization with arterial and venous bypass grafts placed on left anterior descending (LAD) and left circumflex (LCX) arteries.…”

Section: Introductionmentioning

confidence: 99%

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