Characteristics and Surgical Outcomes of Patients With Late Presentation of Anomalous Left Coronary Artery From the Pulmonary Artery: A Multicenter Study

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David M Kwiatkowski ¹, Christopher W Mastropietro ², Katherine Cashen ³, Saurabh Chiwane ⁴, Saul Flores ⁵, Ilias Iliopoulos ⁶, Karan B Karki ⁷, Karl Migally ⁸, Monique R Radman ⁹, Christine M Riley ¹⁰, Peter Sassalos ¹¹, Jennifer Smerling ¹², John M Costello ¹³, Collaborative Research from the Pediatric Cardiac Intensive Care Society Investigators

PMID: 32858217;  DOI: 10.1053/j.semtcvs.2020.08.014

Take Home Points

• 29% of patients with ALCAPA in this large, retrospective, multicenter cohort study presented at greater than one year of age.
• Patients presenting at >1 year of age had excellent intermediate term survival (no mortalities in this series.)
• 11% of patients presenting with ALCAPA at greater than 1 year of age presented with cardiac arrest.
• Although severe LV dysfunction was less common for patients presenting at greater than 1 year than for those presenting at less than 1-year, chronic arrythmia and mitral regurgitation were common.

Commentary from Dr. Barry Deatrick (Baltimore, MD, USA), section editor of Congenital Heart Surgery Journal Watch:  Although most patients with anomalous left coronary artery from the pulmonary artery (ALCAPA) present with heart failure or shock in the first months of life, a population of patients who develop extensive collateralization do not present with acute myocardial ischemia.  Most previous studies of this population are small, and limited to single center studies.  The authors of this study sought to characterize the clinical presentation, outcomes of patients who underwent ALCAPA repair older than 1 year of age with the collective experience from 21 congenital heart centers over 10 years. They hypothesized that this older cohort would present with different clinical symptoms and have different postoperative outcomes than a younger cohort.

A multicenter retrospective cohort study including all patients who underwent initial coronary artery surgery for ALCAPA between January 2009 and March 2018 (21 centers; 248 patients).  Patients who underwent surgical correction at an age > 365 days were identified.  Patients with ARCAPA, single coronary, or other significant cardiac lesions were excluded.  Of the 248 patients identified, 72 underwent operation at greater than 1 year of age.  The median age in this group was 8.3 years, 15 of whom were older than 18.  There was no correlation between the degree of LV dysfunction, and the severity of mitral regurgitation.  11% presented with cardiac arrest, and 4% presented in cardiogenic shock.  35% of patients had been admitted to the hospital for another diagnosis, and 44% of these patients were discharged without the diagnosis of ALCAPA being made.  Compared to the younger patients, patients in the older cohort were more likely to present after cardiac arrest, but less likely to present with respiratory failure or shock, and less likely to require preoperative inotropic or ventilatory support.   No patients received transplantation during the study, although one was listed.  At 1-year follow-up, only 5% had moderate or worse systolic dysfunction, and 19% had moderate or worse mitral regurgitation.  Among the 13 patients who presented with moderate or worse LV systolic dysfunction, recovery was seen in 15% by discharge, 89% by 1-year and 90% by 3-year follow-up.

The authors concluded that patients who presented with ALCAPA older than 1 year of age were more likely to present with cardiac arrest than the younger cohort, the majority of patients who presented older did not have ventricular dysfunction, MR or LV dilation.  Further, they confirmed that outcome of repair after 1 year of age is associated with good intermediate term outcome, and recommend continued follow-up to assess long term outcome.

Table 1.