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Relevant clinical history and physical exam
A 57 year-old female was admitted due to a two-month history of shortness of breath aggravated by exertion and straining. She previously underwent kidney transplantation due to reflux nephropathy.
She was previously on anti-hypertensives, but was discontinued after renal replacement. She had no history of diabetes and was a non-smoker. Her physical examination was unremarkable.
Relevant test results prior to catheterization
Baseline electrocardiogram showed sinus rhythm with left ventricular hypertrophy and negative T waves and ST segment depression on leads V2-V6 (ECG). Coronary artery disease was considered. A dobutamine stress echocardiogram was done, which showed mild hypokinesia in the anterior interventricular septum and apical segments during peak infusion, with runs of supraventricular tachycardia.
Relevant catheterization findings
Coronary angiogram showed no significant stenosis in all visualized coronary segments. A heavy stream of contrast agent entering the left ventricle from the mid to distal left anterior descending artery was noted (Fig 2).
An aortogram was done which showed an anomalous RCA originating superior to the exit of the left main.
Left ventriculogram showed apical obliteration at end-systole with a typical spade-like configuration suggestive of apical hypertrophic cardiomyopathy.
Impression then was an anomalous RCA with coronary cameral fistula and apical hypertrophic cardiomyopathy. A repeat transthoracic echocardiogram was done to further interrogate the drainage of the coronary fistula, which showed
LV hypertrophy with concentration in the apical segments without intracavitary systolic gradient. Color Doppler demonstrated mosaic flow from the epicardial LV layer to the cavity (Fig 5).
A 256-slice CT scan was requested, which confirmed an anomalous RCA originating from the left coronary cusp, with a slit-like ostium, a high-angle take-off, and an interatrial course (Fig 6).
The patient underwent CABG of the proximal RCA using SVG to decrease the risk of sudden cardiac death.
The procedure was tolerated and the patient was discharged stable and well. Follow-up 2D echocardiogram a month after showed similar findings of apical hypertrophy with color flow from the epicardium to the LV chamber, but with improved contractility and no evidence of hypokinesia. The patient is currently well with good functional capacity and had no recurrence of failure symptoms.
The case presented is that of ACAOS, R, with associated coronary-cameral fistula to the LV and apical HCM. A high-index of suspicion is needed due to its atypical presentation. Diagnosis is made with a combination of cardiac imaging procedures. Treatment will depend on the severity, high-risk features, and hemodynamic significance of each anomaly. In this case, both the apical HCM and fistula were clinically not significant and were managed conservatively. The ACAOS R, though more benign compared to ACAOS L, still carries the risk of SCD and was thus managed surgically.