Therapeutic Strategy for Patients with Concomitant Pulmonary Artery Hypertension and Hypertrophic Obstructive Cardiomyopathy: A Rare Case Report

Figures

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  Figure 1. Clinical course before index hospitalization. The patient was diagnosed with PAH according to the results of right heart catheterization. Combination therapy for PAH was performed using sildenafil, macitentan, and riociguat. However, plasma BNP level increased gradually. Annual follow-up transthoracic echocardiography found gradually progressive myocardial hypertrophy despite of initiation of irbesartan and spironolactone and well-controlled blood pressure. IVS, interventricular septal; PW, posterior wall; LVDd, left ventricular diastolic diameter; LVDs, left ventricular systolic diameter; LVEF, left ventricular ejection fraction; BNP, B-type natriuretic peptide; mRAP, mean right atrial pressure; mPAP, mean pulmonary arterial pressure; PAWP, pulmonary arterial wedge pressure; CO, cardiac output; PVR, pulmonary vascular resistance.

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  Figure 2. Transthoracic echocardiography images following termination of anti-PH agents (A–D) and those following medication adjustment for HOCM (E–H). (A) Diffuse left ventricular hypertrophy is obvious; (B) An accelerated blood flow in the left ventricular outflow tract with a Vmax of 3.4 m/s and moderate mitral regurgitation probably due to systolic anterior motion of the mitral valve; (C) Left ventricular outflow tract obstruction with aortic valve semi-closure (yellow arrow head); (D) Systolic anterior motion of the mitral valve (white arrow head); (E) Left ventricular hypertrophy got milder; (F) Neither obvious left ventricular outflow tract obstruction nor mitral regurgitation; (G) No obvious aortic valve semi-closure (yellow arrow head); (H) No obvious systolic anterior motion of the mitral valve (white arrow head). (A,B,E,F) were obtained at B-mode and others were obtained at M-mode. PH, pulmonary hypertension; HOCM, hypertensive obstructive cardiomyopathy.

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  Figure 3. Right heart catheterization at rest (A) and at the time of Brockenbrough phenomenon (B). (A) At rest, the left ventricular pressure gradient was 56 mmHg; (B) At the time of the Brockenbrough phenomenon, a significant pressure gradient between LV and Ao, following the PVC, was observed. PVC, premature ventricular contraction; LV, left ventricle; Ao, aorta. (A) Right heart catheterization showed 56 mmHg of the resting left ventricular pressure gradient and (B) the Brockenbrough phenomenon, which is the significant gradient between the left ventricular (LV) and aortic pressure (Ao) on the beat post-PVC, was positive.