278 A CASE OF ACCIDENTAL DIAGNOSIS OF ALCAPA SYNDROME IN ASYMPTOMATIC CHILD
Authors: Pazzi Sara , Rubino Francesca , Pizzini Jessica , Ciuffreda Matteo , Cristofaletti Alessandra , Antonia Prioli Maria , Pighi Michele , Luciano Ribichini Flavio 
Published: December 2022

A 7-year-old (y.o.) boy was referred to our center for a specialist evaluation due to alteration of the ventricular repolarization in the anterior and lateral lead on the electrocardiogram at medical evaluation for physical activity. The child was totally asymptomatic, with normal growth and unremarkable past medical history. The physical examination revealed only S4 and a minimal apical systolic murmur.

Transthoracic echocardiogram demonstrated a mildly dilated left ventricle (LV) and normal left ventricular ejection fraction. Strain pattern of basal-medium segments of lateral wall was reduced. Right coronary artery (RCA) appeared dilatated and the left coronary artery (LCA) showed an inverse flow at Doppler evaluation. Findings were suspicious for ALCAPA diagnosis (Anomalous Left Coronary Artery Arising from the Pulmonary Artery); a rare congenital heart disease that, if not treated, leads to death in 90% of cases during first year of life. Adult type of ALCAPA (adolescence-60 y.o) is even more rare and can cause heart failure due to a dilatative cardiomyopathy, chronic cardiac ischemia and, in 80-90% of cases, sudden cardiac death.

We performed a coronary angiography that confirmed the anomalous origin of LCA from the pulmonary artery. The RCA was dominant, enlarged and supplied an important collateral flow for LCA. Coronary angiography is indicated not only for diagnosis confirmation, but also for coronary tree demonstration, especially in adult type of ALCAPA, because of the presence of not rare anomalies or “adaptation” of peripheral coronary circulation. All these features are important positive prognostic factors that enable survival beyond infancy. LCA ostium stenosis or bronchial artery collateral vessels are also common but weren't seen in this case. In adult type of ALCAPA surgery is usually indicated at the time of diagnosis, so a coronary button transfer and pulmonary Valsalva sinus reconstruction with autologous pericardial patch were performed. This is the preferred surgical technique in adult patients as it is the most anatomic correction. Since before hospitalization heart failure therapy with betablocker was introduced and after surgery aspirin therapy was started too.

Post-surgery transthoracic echocardiogram highlighted a moderate pericardial effusion without haemodynamic impact and a pleural effusion, so an anti-inflammatory therapy with ibuprofen, cortisone and then colchicine was started. After discharge anti-inflammatory therapy was under-titrated until suspension based on gradual reduction of pericardial and pleural effusion. Patient was always free of symptoms and with good performance status.

ALCAPA or Bland–White–Garland syndrome is an uncommon congenital heart disease. The presence of adequate collateral circulation between RCA and LCA allows to survive beyond infancy (adult type). We described a case of early diagnosis of adult type of ALCAPA in asymptomatic child thanks to a careful examination of basal electrocardiogram for evaluation of non-competitive sport practice.