Clinical introduction

A 42-year-old women with a history of mitral regurgitation was playing tennis when she suddenly collapsed. Bystanders performed cardiopulmonary resuscitation. An automated external defibrillator revealed polymorphic ventricular tachycardia. The patient was shocked once with 200 J with return of spontaneous circulation. She was brought to the emergency department where her vitals revealed a heart rate of 133 beats/min, a respiratory rate of 30 breaths/min, an oxygen saturation of 89% and a blood pressure of 124/98 mm Hg. She was intubated. The chest X-ray revealed cardiomegaly. An ECG revealed sinus tachycardia, left axis deviation, Q waves in V1–V3, lateral T-wave inversions, a non-specific interventricular conduction delay and a corrected QT interval of 500 ms (see online supplementary figure S1). A transthoracic echocardiogram revealed an EF of 30%–35%, normal valvular function, hypokinesis of the mid and distal inferior and anterior walls and mid and distal septum. Lab work revealed a negative troponin and toxicology panel. She was taken for coronary angiography due to concern for acute coronary syndrome (figure 1).

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Figure 1

Images of the patient's coronary anatomy via angiography. (A) Right Anterior Oblique (RAO): View at zero degrees; (B) Left Anterior Oblique (LAO): cranial view (initial injection opacifying the right coronary artery (RCA)) and (C) LAO: cranial view (post-RCA injection).

Question

What would be the next step in management?

1. Attempt Percutaneous Coronary Intervention (PCI) to restore flow through the left anterior descending (LAD)

2. Consult cardiothoracic surgery for mitral valve repair

3. Consult cardiothoracic surgery for revascularisation

4. Consult electrophysiology for implantable cardiac defibrillator

For the answer see page 1664

From the question on page 1626

Answer: C

The correct answer is option C. The patient has the adult type of anomalous left coronary artery arising from the pulmonary artery (ALCAPA) or Bland–White–Garland syndrome. The image reveals that the left main coronary artery does not arise from the left coronary cusp, but rather arises from the pulmonary artery (PA). The right coronary artery (RCA) is large and fills the left coronary system in a retrograde manner. The image shows the RCA filling the LAD, and then emptying into the PA.

Option A is incorrect because there is no evidence of a stenotic lesion in the LAD. Option B is incorrect, because although she had an apparent history of mitral regurgitation at presentation there is no evidence of significant mitral valve dysfunction on exam or on the echocardiogram. Option D is incorrect because surgical correction is the treatment of choice as her arrhythmia was secondary to myocardial ischaemia.

Patients with ALCAPA syndrome are subject to chronic left ventricular subendocardial ischaemia, and therefore are at risk for sudden cardiac death (80%–90%) due to the development of malignant ventricular arrhythmias. The treatment is surgical revascularisation either via coronary artery bypass grafting or reimplantation of the left coronary to the aorta.1 During surgery, the gross examination confirmed our angiographic findings, thus the patient underwent successful reimplantation of the anomalous left coronary to the aortic root.