NYMC Faculty Publications

A Haemodynamic Conundrum: a Case Report of a Patient with Concurrent Pulmonary Arterial Hypertension and Hypertrophic Obstructive Cardiomyopathy

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European Heart Journal. Case Reports

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Case Report

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BACKGROUND: The co-existence of hypertrophic obstructive cardiomyopathy (HOCM) and pulmonary arterial hypertension (PAH) is extremely rare and poses a management conundrum. This is the first case report in the published literature to describe the diagnosis and management of a patient with both conditions.

CASE SUMMARY: A 49-year-old female with a history of HOCM and recently diagnosed scleroderma presented to the clinic with progressive dyspnoea. Transthoracic echocardiogram demonstrated left ventricular outflow tract (LVOT) obstruction at rest, and elevated pulmonary artery (PA) pressure. Cardiac catheterization (CC) demonstrated an LVOT gradient of 150 mmHg, PA pressure of 88/32 mmHg, pulmonary capillary wedge pressure (PCWP) 12 mmHg, pulmonary vascular resistance 14.8 Wu, and a cardiac index of 1.6 L/min/m

CONCLUSION: In patients with concurrent HOCM and advanced PAH, a multidisciplinary treatment approach is needed to rapidly and safely optimize the background of HOCM in order to permit the use of PAH-specific medications.