Key result
Extended myectomy reduces gradient to 13 mmHg after failed alcohol septal ablation in obstructive HCM.
Why the study?
HCM with obstruction is a predictor of adverse outcomes requiring surgical treatment, but patients frequently present under the guise of acute coronary syndrome or heart defect.
Case Report (n=1)
Extended myectomy is an effective therapy for obstructive hypertrophic cardiomyopathy, particularly when alcohol septal ablation is ineffective.
Hypertrophic cardiomyopathy (HCM) is a genetically determined disease associated with the presence of mutations in the genes encoding the synthesis of myocardial contractile proteins. The presence of obstruction in the LVL is a powerful predictor of adverse outcomes and serves as an indication for surgical treatment. One of the frequent “masks” that patients with HCMP come with is “acute coronary syndrome” or “heart defect”. Patient O., 43 years old, maintained a good tolerance to physical exertion for a long time. Has 2 children. Pregnancies proceeded without pathology from the cardiovascular system. Mild arterial hypertension was registered after the second birth. In August 2017, the patient was hospitalized with a diagnosis of ACS, diagnostic coronary angiography was performed: the coronary arteries are not changed. During auscultation, systolic noise was heard over the precardial region. Previously, noises were detected. On an echocardiogram, an obstructive variant of HCMP with a gradient of 150 mmHg was diagnosed. Given the high gradient of LVL obstruction, the patient underwent selective alcohol ablation. After alcohol ablation, there was a high gradient in VTLJ – 96 mmHg. Cardialgia and shortness of breath persisted. No cardiac arrhythmias were recorded. In August 2019, the patient underwent an extended myoectomy with good effect. At control examinations a year after myoectomy: the pressure gradient on the AoC is 13 mmHg, VTLJ is 11 mmHg. This example illustrates the need to include HCMP in the circle of differential diagnosis when examining a young patient with chest pain, especially in combination with heart murmurs. Echocardiography with Doppler examination is a simple and affordable diagnostic method. The alcohol septal ablation performed was ineffective, and the extended myoectomy had a good hemodynamic and clinical effect and may be the method of choice of therapy in these patients. The peculiarity of this case is the manifestation of the disease at the age of 43, during two pregnancies without clinical manifestations of pathology of the cardiovascular system.
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Mosina et al. (2023) conducted a case report in Obstructive hypertrophic cardiomyopathy (n=1). Extended myoectomy was evaluated on Pressure gradient. Extended myoectomy successfully reduced the pressure gradient to 13 mmHg in a 43-year-old woman with obstructive hypertrophic cardiomyopathy after an ineffective alcohol septal ablation.
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