Anomalous origin of the left coronary artery from the pulmonary artery: Diagnosis and postoperative follow up

Background: Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital malformation that presents a diagnostic challe nge to the pediatrician and pediatric cardiologist. Although surgical repai r is always indicated, the optimal technique has yet to be determined. Objectives: To review our experience with the diagnosis of children with AL CAPA and to assess short to midterm surgical results. Methods: Between 1992 and 1998, 13 infants and children (2 months to 15 yea rs) were treated for ALCAPA at our medical center. Eight were diagnosed dur ing the first,year of life; all were symptomatic and had severe dysfunction of the left ventricle. The five patients diagnosed at an older age had nor mal myocardial function. Diagnosis was established by echocardiography alon e in seven patients; six required catheterization (one infant and all older patients), Surgery was performed in 12 patients to establish dual coronary artery system: 7 underwent the Takeuchi procedure and 5 had re-implantatio n of the anomalous left coronary Results: One infant died shortly after diagnosis before repair was attempte d, and one died postoperatively. Four patients required additional surgery: three for late complications of the Takeuchi procedure and one value repla cement for mitral insufficiency. Recent evaluation revealed good global lef t ventricle function in all patients except for one, who is still within th e recovery phase and shows gradual improvement. However, most patients who presented with severe myocardial dysfunction upon diagnosis still display a bnormal features such as echo-dense papillary muscles or evidence of small akinetic segments. In this group, early repair was associated with faster m yocardial recovery, Conclusions: The diagnosis of ALCAPA remains a clinical challenge to the pe diatrician and cardiologist. Diagnosis can be established echocardiographic ally, and early diagnosis and treatment may lead to faster myocardial recov ery. The preferred surgical method appears to be re-implantation of the ALC A. The chance for good recovery of global ventricular function is high even in the Sickest patients, nonetheless abnormal myocardial features can be i dentified even years after surgery.