Anomalous origin of the left coronary artery from the pulmonary artery. Diagnosis and treatment in 3 pediatric patients

Anomalous origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA) syndrome is a potentially lethal and rare congenital cardiac anomaly (for approximately 1 in 300,000 live births). ALCAPA syndrome mostly presents in the first few months of life. Left untreated, the mortality rate in the first year of life is 90% secondary to myocardial ischemia or infarction and mitral valve insufficiency leading to congestive heart failure. When pulmonary arterial pressure decreases, it results in reversal of flow so that LCA drains from the right coronary artery through collateral vessels into the pulmonary artery. This phenomenon causes ischemia or eventually infarction of the anterolateral left ventricular wall. Sudden death may occur because of inadequate collateral circulation between LCA and the right coronary artery and/or development of arrhythmia. ALCAPA can be found both in infants and in older individuals, with the absence or presence of strong collateral circulation from the right coronary artery, with different clinical presentations. Clinical diagnosis of ALCAPA can be demanding. Findings can be similar to those in dilatative cardiomyopathy and the diagnosis of ALCAPA has to be excluded, principally in the presence of ischemic ECG findings. Currently, the prognosis for patients with ALCAPA syndrome is dramatically improved as a result of both early diagnosis and improvements in surgical techniques, including myocardial preservation. This paper presents 3 patients with ALCAPA syndrome as well as the variations in their age, clinical presentation, modality of treatment (pharmacological treatment, surgical correction and percutaneous coronary stent implantation after graft obstruction) and outcome.