Clinical Presentation of Childhood ALCAPA: a Systematic Review and Meta-analysis

Abstract

Introduction and Objectives. Anomalous left coronary artery from pulmonary artery is a rare congenital heart defect that leads to a mortality in 90% of the affected infants if left untreated. Hence, a diagnosis of this malformation demands urgent surgical correction. However, the diagnosis is a clinical challenge as it presents in a similar way to common pediatric conditions. This systematic review aimed to analyse and estimate the frequency of signs and symptoms to assist physicians in early detection of this condition. Methods. We searched two databases, Pubmed/Medline and Web of Science, without date or language restrictions and, when appropriate, meta-analysed data from the included studies. Results. We screened 853 abstracts for eligibility and included 9 studies (71 children) in the analysis. Overall, 24 presenting signs and symptoms were identified and ranked according to frequency. Three features were present in >50% of children: irritability (80%), tachypnea (59%) and cardiomegaly (51%). An additional eight features were present in a third to a half of children: sweating (47%), heart murmur (44%), dyspnea (42%), hepatomegaly (40%), failure to thrive (40%), respiratory failure (40%), cardiogenic shock (36%) and palpitation (33%). Only 1 of the 71 children was asymptomatic on diagnosis. Conclusion. Children presenting with symptoms of unexplained congestive heart failure, angina-like symptoms (as colic pain and gastroesophageal reflux), mitral insufficiency murmur or cardiomegaly should alert physicians to possible anomalous left coronary artery from pulmonary artery. Furthermore, children with suspected myocarditis should also be evaluated specifically for this malformation.