Introduction: ALCAPA syndrome is a rare congenital heart defect that is present in only 1/300,000 live births and typically presents in infancy as left heart failure. In cases of untreated ALCAPA, mortality reaches up to 85% in the first year of life. Rarely, patients are asymptomatic in childhood and present as adults with signs and symptoms of myocardial ischemia. Case Description: An 18-year-old female with a history of ALCAPA (Anomalous Left Coronary Artery from the Pulmonary Artery) syndrome status-post repair presents with exertional chest pain associated with nausea, light headedness, and shortness of breath that began during marching band practice. Of note, the patient was previously asymptomatic and was diagnosed with ALCAPA incidentally at 13 years of age. Due to patient anatomy and sufficiency of collateralization from the RCA, the LCA ostium at the MPA was patch closed rather than translocating the coronary artery as is traditionally done for ALCAPA repair. The patient was initially evaluated by EMS and in the emergency. Serial EKGs, CXR, and troponins were normal and the patient was discharged home. At follow-up with her cardiologist, the patient continued to describe these symptoms. The patient underwent cardiac MRI stress test, revealing an inducible perfusion defect in the LAD and LCx coronary artery distribution. MRI also showed a small area of apical hypokinesis in the left ventricle and an area of transmural late gadolinium enhancement in the apical anterior segment (Figure 1). Cardiac CT demonstrated small collaterals from the dominant RCA supplying the LCA system (Figure 2). The patient also underwent cardiac catheterization for surgical preparation, which revealed a significant reduction in the number and caliber of RCA collaterals supplying the left coronary system in comparison to the time of initial diagnosis. The patient was ultimately optimized on metoprolol to minimize myocardial demand until successful CABG with LIMA graft. Discussion: In the reported case, the rare asymptomatic ALCAPA patient underwent a sub-optimal repair due to anatomical limitations and later presented with signs of myocardial ischemia, in a fashion similar to what would be seen in an unrepaired patient. Reports of chest pain with negative biomarkers and ECG are often not taken seriously in young adults, and this patient’s coronary anomaly may have gone undetected without a thorough history and physical and specialized imaging. Early detection and repair of coronary anomalies is essential in preventing long term myocardial damage. It is imperative to include congenital heart disease in the differential for chest pain or other cardiac symptoms in young, previously healthy patients. Conclusion: This case highlights the importance of taking a thorough history and having a broad differential in cases of typical chest pain, regardless of age or known risk factors.

Section on Minority Health, Equity, and Inclusion Program

Figure 1

Cardiac MRI stress test revealed an inducible perfusion defect in the LAD and LCx distribution. MRI also showed a small area of apical hypokinesis in the left ventricle and an area of transmural late gadolinium enhancement in the apical anterior segment.

Figure 1

Cardiac MRI stress test revealed an inducible perfusion defect in the LAD and LCx distribution. MRI also showed a small area of apical hypokinesis in the left ventricle and an area of transmural late gadolinium enhancement in the apical anterior segment.

Close modal
Figure 2

Cardiac CT demonstrated origin of the narrow LCA at the MPA and small collaterals from the dominant RCA supplying the LCA system.

Figure 2

Cardiac CT demonstrated origin of the narrow LCA at the MPA and small collaterals from the dominant RCA supplying the LCA system.

Close modal