Clinical introduction A 53-year-old woman with no previous medical history complained of easy fatigue over the last 6 months. She had a positive family history for coronary artery disease but no other risk factors. On physical examination, a 3/6 pansystolic murmur was heard over the apex, and the lung auscultation was unremarkable. Her ECG showed a left anterior fascicular block, with poor R wave progression in the anterior leads (see online supplementary image A). A subsequent echocardiogram revealed a slightly dilated for the patient’s body surface area (BSA) (1.73 m2) left ventricle (55/35 mm), with preserved systolic function and a moderate functional mitral regurgitation. The estimated pulmonary artery pressure was 45 mm Hg. During treadmill radionuclide scintigraphy, her exercise tolerance was normal, with good inotropic response, and 96% oxygen saturation at rest and at peak exercise. A 2 mm ST segment depression was noted at peak effort, which persisted well into recovery (see online supplementary image B). The scintigraphy scan showed extensive reversible anteroapical wall ischaemia (see online supplementary image C). At this point she was referred to us for right and left heart catheterisation. Intracardiac pressures and saturations were: right atrium (RA)RA=3 mm Hg, right ventricle (RV)=26/3 mm Hg, Pulmonary artery (PA)=26/10/mean 16 mm Hg, pulmonary capillary wedge pressure (PCWP)=11 mm Hg, left ventricle (LV)=110/10 mm Hg, Aorta (Ao)=110/60/mean 80 mm Hg, Superior vena cava saturation (SVCsat)=62%, RAsat=62%, PAsat=78%, Aosat=96% and estimated pulmonary to systematic flow ratio (Qp/Qs)=1.8. Her coronary angiography and CT angiography are shown in figure 1A,B.
Question What is the most likely diagnosis?
Right coronary fistula to right ventricle
Kawasaki disease with fistula
Anomalous origin of the left coronary artery from the pulmonary artery
Persistent truncus arteriosus
- Congenital heart disease
- Cardiac catheterization and angiography
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