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Mild to moderate pulmonary hypertension secondary to elevation of left atrial pressure is common in patients with hypertrophic cardiomyopathy. However, severe pulmonary hypertension of systemic levels is unusual in these patients, especially in the absence of severe fixed obstruction and severe mitral regurgitation.
A 74 year old woman presented with severe exertional dyspnoea. On echocardiography, she was found to have hypertrophic obstructive cardiomyopathy with a labile left ventricular outflow tract gradient, mild to moderate mitral regurgitation, and severe pulmonary hypertension. She underwent implantation of a permanent pacemaker but continued to have severe symptoms. At the time of cardiac catheterisation, there was a labile left ventricular outflow tract gradient between 5–50 mm Hg. The mean left atrial pressure was 20 mm Hg. The pulmonary artery pressure was 100/35 mm Hg, at a time when the aortic systolic pressure was 108 mm Hg (left hand panel: LV, left ventricular pressure; Ao, aortic pressure; PA, pulmonary artery pressure; LA, left atrial pressure).
Alcohol septal ablation was performed without complications. Six months after the procedure, the patient was free of exertional symptoms. Repeat cardiac catheterisation demonstrated resolution of the left ventricular outflow tract gradient and a decrease in the mean left atrial pressure. The pulmonary artery systolic pressure had dropped to 62 mm Hg with an aortic systolic pressure of 160 mm Hg (right hand panel).