A case of adult-type anomalous left coronary artery from pulmonary artery (Alcapa syndrome)

Introduction: Anomalous left coronary artery from pulmonary artery, known as ALCAPA syndrome is a rare congenital anomaly, with two forms of presentation – the infant type (with early death in the first year of life) and the adult type (cause of sudden cardiac death).
Case presentation: We present the case of a 42-year-old woman diagnosed 20 years ago with a left coronary artery (LCA) origin abnormality (from the pulmonary artery), asymptomatic until presentation. She was ad-mitted with left ventricular dysfunction as a result of new onset atrial fibrillation with rapid ventricular res-ponse. Transthoracic echocardiography reveals the ori-gin of right coronary artery (RCA) from the right coro-nary sinus (ectatic artery) with multiple turbulent color flow regions (intercoronary collaterals) in interventri-cular septum, moderate-severe systolic dysfunction, diffuse LV hypokinesia, with hyperechogenic endo-cardium, extensive papillary muscle calcification and moderate-severe mitral regurgitation through multiple mechanisms.
The echocardiographic findings are suggestive of adult-type ALCAPA syndrome, also confirmed by transesophageal echocardiography and coronary angi-ography. The LCA was not visualized at the left sinus valsalva. The RCA was dilated and tortuous, filled the left coronary system through collateral vessels and the LCA was originating from the main pulmonary artery. Right and left cardiac catheterization highlights pre-and postcapillary PAH, without right-to-left shunt. The coronary-CT scan evaluation provides additional data on coronary anatomy. Given the presence of left ventricular dysfunction, it is decided to apply a strategy of rhythm control by means of electrical conversion of atrial fibrillation to sinus rhythm and subsequent antiarrhythmic treatment (with amiodarone) and op-timal heart failure treatment. The patient is referred to a cardiovascular surgical center for surgical correction, in order to prevent further myocardial ischemia, ma-lignant ventricular arrhythmias and sudden cardiac death. Implantable cardiac device (ICD) implantation may also be considered in our patient for primary pre-vention of malignant ventricular arrhythmias, in absence of surgical treatment.
Conclusions: The survival of the patient to adulthood was achieved by developing a rich intercoronary colla-teral circulation, which in turn caused a „coronary ste-al” phenomenon with left-to-right shunt and chronic myocardial hypoperfusion. Chronic ischemic injury caused left ventricular systolic and diastolic dysfunction and mitral regurgitation due to papillary muscle impairment, with risk of sudden cardiac death, in the absence of surgical correction. The patient also may require replacement of the mitral valve, due to intrin-sic changes. ALCAPA syndrome is a rare congenital anomaly in children and especially in adults, which is frequently lethal in the absence of specific treatment.

ISSN – online: 2734 – 6382
ISSN-L 1220-658X
ISSN – print: 1220-658X
The Romanian Journal of Cardiology is indexed by:
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CODE: 379
CME Credits: 10 (Romanian College of Physicians)