Review
Anomalous origin of the left coronary artery from the pulmonary artery: collective review of surgical therapy

https://doi.org/10.1016/S0003-4975(02)03633-0Get rights and content

Abstract

Anomalous origin of the left coronary artery from the pulmonary artery is an extremely rare but potentially fatal congenital coronary anomaly. Prompt surgical reestablishment of a two-coronary system on diagnosis yields excellent results and allows progressive and nearly total myocardial recovery. Follow-up of all patients is required to assess the adequacy of repair and to exclude ongoing or recurrent myocardial insult.

Section snippets

Physiology

The original clinical spectrum of ALCAPA was first reported in 1933 [9]. Also known as the Bland-White- Garland syndrome, myocardial ischemia results in the typical presentation in infants of failure to thrive, profuse sweating, dyspnea, pallor, and atypical chest pain on eating or crying. In adults, malignant arrhythmias leading to sudden death may be the first manifestation of the anomaly [10].

Edwards [11] is credited with the first pathophysiological explanation of coronary artery dynamics

Classification

Few classifications of ALCAPA exist, and those that do are commonly descriptive in regard to the origin of the anomalous left coronary artery. In a general classification of coronary artery anomalies, Ogden [29] defined ALCAPA as a major coronary anomaly, referring to a coronary origin other than from the aortic root. More recently, Smith and associates [18] proposed a nomenclature relating to the sinus of origin of the anomalous coronary artery. Using the standard designation 1 for the sinus

Diagnosis

Although ALCAPA is very rare, a high index of suspicion is required during the workup of any infant or child with global myocardial dysfunction. The most important differential diagnosis is with dilated cardiomyopathy, which also commonly presents with moderate to severe congestive heart failure, massive cardiomegaly on chest roentgenogram, ischemic signs on the electrocardiogram (ECG), and a murmur of mitral insufficiency. Although rather simple noninvasive investigations can lead to rapid

Surgical evolution and current techniques

Surgical correction is the gold standard in the therapy for ALCAPA and has undergone considerable evolution since its inception. One of the earliest operative attempts was by Willis J. Potts from Children’s Memorial Hospital in Chicago, who created an aortopulmonary anastomosis in 2 patients, thereby increasing PA blood flow and hence, left coronary artery oxygen saturation [38]. In 1953, Mustard reported an end-to-end anastomosis between the left common carotid artery and the ALCAPA [39]. Five

Surgical results

The operative mortality rate for all surgical techniques combined was in the range of 75% to 80% in the early 1980s [21], but it has been lowered to the 0% to 23% range in the current era 3, 5, 19, 22, 31, 36, 46, 54. After all techniques that reestablish a two-coronary system, a return to normal size of the previously dilated RCA with regression of the intercoronary collateral network has been observed. To date, no difference in long-term LV function or late mortality has been demonstrated

Operative risk factors

Various risk factors for operative survival have been identified in the treatment of ALCAPA, including decreased preoperative LV function 20, 22, 26 and young age at operation [26]. Although younger patients are seen with more severe LV failure, surgical intervention results in a more rapid and complete recovery of myocardial function, as assessed by echocardiography [23]. Infants seen later (>10 months) have less depressed LV contractility from increased intercoronary collateralization, but

Myocardial scarring and mitral valve procedures

The surgical correction of ALCAPA strives to address not only the coronary problem, but also the other end points of ischemic insult to the left heart. Left ventricular free wall aneurysm and mitral valve insufficiency are the two potential surgical targets, and treatment of the latter is still controversial.

Most myocardial scarring does not result in the formation of an aneurysm, and postinfarction ventricular septal defects or cardiac rupture [63] are extremely rare in patients with ALCAPA.

Mechanical circulatory support

Encouraging results have been achieved with the use of cardiac mechanical support as a bridge to recovery in the postoperative setting of ALCAPA 8, 24, 31, 46, 67, 68, 69, 70, 71, 72. Such support measures include LV assist devices 8, 31, 68, 69, 70 extracorporeal membrane oxygenation 24, 46, 71, and intraaortic balloon pumps [72] in older children. In a report by del Nido and associates, [8], 5 survivors among 7 infants who underwent surgical correction of ALCAPA and intractable LV failure

Ultrastructural residual and follow-up modalities

Although improvement in myocardial function is expected after surgical correction of ALCAPA 22, 23, 56, 73, normalization of LV function may take as long as 22 months 22, 23, 31, 56. This slow recovery results from preoperative ultrastructural abnormalities caused by chronic myocardial hypoperfusion at the cellular level, which need time to normalize 18, 22, 56, 74. More rapid regression of LV enlargement and more complete recovery of LV function constitute the rationale to perform earlier

Conclusions

A high index of suspicion and appropriate diagnostic modalities should allow rapid diagnosis of ALCAPA in any patient seen with dilated congestive cardiac failure. The surgical results of operations that reestablish a two- coronary artery circulation have dramatically improved in recent years in this otherwise fatal disease. Regardless of the degree of preoperative ventricular impairment, an aggressive early surgical approach is warranted, as it represents the only possibility to salvage

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