Assessment, diagnosis and treatment of concomitant obstructive hypertrophic cardiomyopathy and valvular aortic stenosis

Introduction: T he obstruction in the left ventricle’s outflow may be caused by a supravalvular, valvular or subvalvular stenosis. The supravalvular cause is rare, more common being the valvular cause– aortic stenosis of different etiologies, and the subvalvular one – obstructive hypertrophic cardiomyopathy (OHCM) with left ventricular outflow tract (LVOT) obstruction. OHCM and aortic stenosis may coexist, thus determining the cause of symptoms, the differential diagnosis, the severity of the pathologies, and the optimal treatment strategy may be challenging.

Objective: Our purpose is to report a case of OHCM in a patient with associated aortic stenosis, describing the patient`s assessment, diagnosis, and treatment.

Methods: A 70-year-old male patient was addressed to our clinic for signs and symptoms of heart failure and recurrent syncope with maximal tolerated medical treatment. His medical history shows aortic stenosis, OHCM, paroxysmal atrial fibrillation, type 2 diabetes mellitus, essential hypercholesterolemia, and colorectal cancer operated in 2012. The resting ECG revealed left ventricular hypertrophy (LV) (Sokolov Lyon index of 38mm) and characteristic repolarization changes. The blood tests did not provide any additional information. Transthoracic (TTE) and transesophageal echocardi-ography (TEE), the 24-hour ECG Holter monitoring and angiocoronarography were performed for the di-agnosis and treatment.

Results: TTE revealed an OHCM phenotype (interventricular septum of 2,4cm, LVOT gradient of 58mmHg) and LV ejection fraction of 65%. In addition, an intensely degenerated aortic valve was detected, with an aortic valve area (AVA) of 1.2 cm2. The 24-hour ECG Holter monitoring recorded no arrhythmias. Angio-coronarography revealed normal coronary arteries. As the patient refused surgery, septal reduction therapy by alcohol-ablation was performed and the aortic pathology was set to be further reevaluated and followed-up. After the intervention, a significant improvement of the symptoms and a decrease of the LVOT gradient to 19 mmHg were detected. ETT and ETE reevaluation of the aortic valve pathology revealed a mild aortic stenosis, with a mean gradient of 15 mmHg and an AVA of 1.8 cm2.

Conclusions: This case illustrates the clinical course of a patient with aortic stenosis associated with obstructive hypertrophic cardiomyopathy. Both entities cau-se similar symptoms and increased pressure gradient in the LV outflow. In this patient, the symptoms and obstruction were determined by CMHO, with significant post-procedural improvement. Aortic stenosis has been shown to be mild after removal of LVOT obstruction, confirming that CMHO can significantly decrease transvalvular systolic volume by limiting valve opening, with valvular disease being more benign than appears. In a symptomatic patient with both HOCM and aortic stenosis it is important to distinguish which process is dominant or whether both of them are significant.



ISSN – online: 2734 – 6382
ISSN-L 1220-658X
ISSN – print: 1220-658X
The Romanian Journal of Cardiology is indexed by:
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CODE: 379
CME Credits: 10 (Romanian College of Physicians)
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