Anomalous left coronary artery from the pulmonary artery in adults: a systematic review of clinical presentation, diagnosis, and outcomes.

Abnormal left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary anomaly, occurring in approximately 1 in 300 000 live births and accounting for roughly 0,5 per cent of all congenital heart defects. Without surgical correction, in more than 90% of cases, the defect leads to death in the first year of life. However, a small subgroup of individuals survive into adulthood. In adults, it can manifest as dyspnoea, angina, palpitations and eventually leads to heart failure, arrhythmia and sudden cardiac death. We summarised 60 years of case reports of ALCAPA syndrome in adults and identified 279 patients, the majority of whom were women. In most cases, the diagnosis was based on coronary angiography. The most common symptom was dyspnoea and the least frequent were palpitations. Surgery was performed in 194 patients with a mean age at surgery of 37,46 years. The aim of this study is to obtain clinically relevant information that would be unattainable even in large multicentre clinical trials, given the rarity of this anomaly. This is the first review juxtaposing all ALCAPA cases in adults published in 60 years.