ANOMALOUS ORIGIN OF THE LEFT CORONARY ARTERY FROM THE PULMONARY ARTERY (ADULT TYPE)

The case of a 7-year-old white female with an anomalous left coronary artery from the pulmonary artery is presented. The clinical findings of an asymptomatic patient with a continuous murmur at the left lower strenal border, with left ventricular hypertrophy by roentgenography and electrocardiography raised the suspicion of an aortic fistula, which was then investigated by retrograde aortography. The aortogram demonstrated the anomalous origin of the left coronary artery with retrograde flow into the pulmonary artery. The age and circumstances of death in previously reported cases indicate that the so-called adult type is not a benign disease. The diagnosis of an anomalous coronary artery from the pulmonary artery is at any age an indication for consideration of surgical intervention.

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Surgery and critical care for anomalous coronary artery from the pulmonary artery

Cardiology in the Young ◽

10.1017/s1047951110001071 ◽

2010 ◽

Vol 20 (S3) ◽

pp. 35-43 ◽

Cited By ~ 3

Author(s):

Timothy A. Fehrenbacher ◽

Michael E. Mitchell ◽

Nancy S. Ghanayem ◽

James S. Tweddell

Keyword(s):

Coronary Artery ◽

Pulmonary Artery ◽

Left Coronary Artery ◽

Ventricular Dysfunction ◽

Left Ventricular ◽

Arterial System ◽

Anomalous Origin ◽

Anomalous Coronary Artery ◽

Young Infants ◽

Anomalous Coronary

AbstractAnomalous origin of the left coronary artery from the pulmonary artery is a rare congenital cardiac malformation that accounts for 0.25–0.50% of children with congenital cardiac disease and can cause myocardial dysfunction in young infants. In any infant presenting with ventricular dysfunction, the diagnosis of anomalous origin of the left coronary artery from the pulmonary artery must be suspected and the origin of the coronary arteries must be confirmed. The diagnosis of anomalous origin of the left coronary artery from the pulmonary artery is an indication for surgical repair. A two-coronary arterial system is the goal and is almost always achievable. The goal of surgical therapy is the creation of a two-coronary arterial system, which appears to provide better long-term survival and protection from left ventricular dysfunction and mitral valvar regurgitation than does simple ligation of the anomalous coronary artery. Direct reimplantation of the anomalous coronary artery is the procedure of choice. It is straightforward and borrows from well-practised techniques commonly used in other procedures such as the arterial switch operation. For the rare patient in whom direct reimplantation is not possible, strategies to lengthen the anomalous coronary artery, or baffle it within the pulmonary root, are available. Mitral valvar regurgitation is common at presentation, but following the establishment of a two-coronary arterial system and satisfactory myocardial perfusion, regurgitation of the mitral valve resolves in the vast majority. Therefore, mitral valvuloplasty at the time of initial surgery for anomalous origin of the left coronary artery from the pulmonary artery is not indicated. Post-operative care requires careful manipulation of inotropic support and reduction of afterload. Mechanical support, with either extracorporeal membrane oxygenation or left ventricular assist device, should be available for use if necessary.

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Repair of Anomalous Left Coronary Artery from Pulmonary Artery (ALCAPA) beyond Infancy

The Heart Surgery Forum ◽

10.1532/hsf98.20131008 ◽

2013 ◽

Vol 16 (4) ◽

pp. 210 ◽

Cited By ~ 2

Author(s):

Sachin Talwar ◽

Aandrei Jivendra Jha ◽

Shiv Kumar Choudhary ◽

Saurabh Kumar Gupta ◽

Balram Airan

Keyword(s):

Coronary Artery ◽

Pulmonary Artery ◽

Respiratory Tract ◽

Left Coronary Artery ◽

Nyha Class ◽

Left Ventricular ◽

Left Ventricular Ejection ◽

Anomalous Left Coronary Artery ◽

Tract Infections ◽

Anomalous Left Coronary

Between January 2002 and December 2012, five patients (4 female) underwent corrective surgery for anomalous left coronary artery from pulmonary artery (ALCAPA). They were older than 1 year (range, 3-56 years). One of the 2 patients younger than 10 years had presented with congestive heart failure, and the other had experienced repeated episodes of lower respiratory tract infection since childhood. Of the remaining 3 adult patients, 2 had experienced angina with effort, and 1 patient had had repeated respiratory tract infections since childhood, with mild dyspnea on effort of New York Heart Association (NYHA) class II. Three patients had the anomalous left coronary artery implanted directly into the ascending aorta via coronary-button transfer, and 2 patients underwent coronary artery bypass with obliteration of the left main ostium. Two patients underwent concomitant mitral valve repair procedures, and 1 patient underwent direct closure of a perimembranous ventricular septal defect. Four patients survived the surgery, and 1 patient died because of a persistently low cardiac output. Follow-up times ranged from 3 months to 4 years. All survivors are in NYHA class I and have left ventricular ejection fractions of 45% to 60%, with moderate (n = 1), mild (n = 1), or no (n = 2) mitral insufficiency. We conclude that a few naturally selected patients with ALCAPA do survive beyond infancy and can undergo establishment of 2 coronary systems with satisfactory results.

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Outcomes of surgical repair of anomalous origin of the left coronary artery from the pulmonary artery in infants and children

Cardiology in the Young ◽

10.1017/s104795112100161x ◽

2021 ◽

pp. 1-6

Author(s):

Tong Feng ◽

Guo Zhangke ◽

Bai Song ◽

Fan Fan ◽

Zhen Jia ◽

...

Keyword(s):

Coronary Artery ◽

Mitral Regurgitation ◽

Ejection Fraction ◽

Pulmonary Artery ◽

Left Coronary Artery ◽

Surgical Repair ◽

Left Ventricular ◽

Anomalous Origin ◽

Dimension Index

Abstract Objectives: Anomalous origin of the left coronary artery from the pulmonary artery is associated with high mortality if not timely surgery. We reviewed our experience with anomalous origin of the left coronary artery from the pulmonary artery to assess the preoperative variables predictive of outcome and post-operative recovery of left ventricular function. Methods: A retrospective review was conducted and collected data from patients who underwent anomalous origin of the left coronary artery from the pulmonary artery repair at our institute from April 2005 to December 2019. Left ventricular function was assessed by ejection fraction and the left ventricular end-diastolic dimension index. The outcomes of reimplantation repair were analysed. Results: A total of 30 consecutive patients underwent anomalous origin of the left coronary artery from the pulmonary artery repair, with a median age of 14.7 months (range, 1.5–59.6 months), including 14 females (46.67%). Surgery was performed with direct coronary reimplantation in 12 patients (40%) and the coronary lengthening technique in 18 (60%). Twelve patients had concomitant mitral annuloplasty. There were two in-hospital deaths (6.67%), no patients required mechanical support, and no late deaths occurred. Follow-up echocardiograms demonstrated significant improvement between the post-operative time point and the last follow-up in ejection fraction (49.43%±19.92% vs 60.21%±8.27%, p < 0.01) and in moderate or more severe mitral regurgitation (19/30 vs 5/28, p < 0.01). The left ventricular end-diastolic dimension index decreased from 101.91 ± 23.07 to 65.06 ± 12.82 (p < 0.01). Conclusions: Surgical repair of anomalous origin of the left coronary artery from the pulmonary artery has good mid-term results with low mortality and reintervention rates. The coronary lengthening technique has good operability and leads to excellent cardiac recovery. The decision to concomitantly correct mitral regurgitation should be flexible and be based on the pathological changes of the mitral valve and the degree of mitral regurgitation.

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