This 56-year-old woman was discovered to have a heart murmur in her teens. She was told that “she was born with it”. In the past 10 years she had developed increasing dyspnea on exertion, moderate cyanosis and finger clubbing:

• Sinus rhythm
• Biatrial enlargement
• Incomplete right bundle branch block (iRBBB)
• Right ventricular hypertrophy
• Severe diffuse ST depression and T wave inversion.

Comments: The echocardiogram demonstrated severe right ventricular hypertrophy. The LV ventricular angiogram showed a small hypertrophic LV and the presence of a patent ductus arteriosus. At cardiac catheterization the pulmonary artery pressure was 98/53, the aortic pressure 115/52. There was left-to-right and right-to-left shunt, with a significant step-up of oxygen from the RV outflow tract to the PA. The oxygen saturation of the aortic blood was 91.5%.