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Published on: August 8, 2022
Revelation of an obstructive hypertrophic cardiomyopathy in an elderly patient
Salvatore Patanè1, Filippo Marte1, Alessandro Portaro2
1Cardiologia Nuovo Presidio Ospedaliero Cutroni Zodda-Barcellona P.d.G (Me), AUSL5 Messina, Italy.
Insights
Hypertrophic cardiomyopathy (HCM) is a common genetic heart disease causing left ventricular hypertrophy. This case highlights obstructive HCM in an elderly patient, emphasizing the need for precise diagnosis and tailored treatment strategies.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Hypertrophic cardiomyopathy (HCM) is a primary, autosomal dominant genetic heart disease.
- It is characterized by left ventricular hypertrophy (LVH) without other cardiac or systemic causes.
- HCM is clinically heterogeneous, with phenotypic features developing across all age groups.
Observation:
- Most HCM patients are prone to dynamic left ventricular outflow tract (LVOT) obstruction.
- Obstruction results from systolic anterior motion of the mitral valve and septal contact.
- Hypertrophic obstructive cardiomyopathy (HOCM) can lead to severe symptoms, heart failure, ischemia, and death.
Findings:
- Accurate determination of hypertrophy site and LVOT obstruction is crucial for treatment.
- Treatment options for HOCM include pharmacotherapy, alcohol septal ablation, surgical myectomy, and pacemaker therapy.
- This study presents a case of obstructive HCM in an 84-year-old woman with a significant LVOT gradient (188 mm Hg) and syncope.
Implications:
- Understanding the specific hypertrophy patterns guides optimal therapeutic decisions in HOCM.
- Early diagnosis and management of HOCM can prevent severe complications.
- This case underscores the importance of considering HCM in elderly patients presenting with syncope and outflow tract obstruction.
Abstract:
Hypertrophic cardiomyopathy (HCM) is classified as a primary cardiomyopathy. HCM is a clinically heterogeneous but relatively common autosomal dominant genetic heart disease that probably is the most frequently occurring cardiomyopathy. HCM is characterized morphologically and defined by a hypertrophied, nondilated left ventriculum (LV) in the absence of another systemic or cardiac disease that is capable of producing the magnitude of wall thickening evident (e.g., systemic hypertension, aortic valve stenosis). Most HCM patients have the propensity to develop dynamic obstruction to LV outflow under resting or physiologically provocable conditions, produced by systolic anterior motion of the mitral valve with ventricular septal contact. The phenotypic features of HCM may develop at any age from infancy to adulthood, and are characterized by a great heterogeneity in the extent, magnitude, and distribution of left ventricular hypertrophy. Hypertrophic obstructive cardiomyopathy (HOCM) often leads to heart failure, severe ischemia, severe symptoms and death. Determination of the exact site of the hypertrophy and of the obstruction of the left ventricular outflow tract, in asymmetric septal hypertrophy, establishes which is the best treatment strategy. In the treatment of HOCM, drug therapy with negatively inotropic drugs, percutaneous transluminal septal myocardial ablation by alcohol-induced septal branch occlusion, surgical myectomy and DDD pacemaker therapy are considered the therapeutical options. We present a case of an obstructive hypertrophic cardiomyopathy in an 84-year-old Italian woman with a left ventricular outflow tract (LVOT) peak gradient with the Valsalva maneuver of 188 mm Hg and with a history of first episode of syncope.
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