Histopathological Changes and Clinical Implications in Patients with Hypertrophic Cardiomyopathy

2010 ◽  
Vol 6 (2) ◽  
pp. 88 ◽  
Author(s):  
Vibeke Marie Almaas ◽  
Jan Peder Amlie ◽  
◽  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Small Vessel Disease ◽  
Ventricular Outflow Tract ◽  
Clinical Findings ◽  
Left Ventricular ◽  
Electrical Instability ◽  
Left Ventricular Outflow ◽  
End Stage Heart Failure ◽  
End Stage ◽  
The Relationship

Hypertrophic cardiomyopathy (HCM) is a familial disease with a wide clinical spectrum. Many patients have normal life expectancy, some die suddenly – often as adolescents or young adults – whereas only a few develop end-stage heart failure. In the total HCM population, only 25% develop significant left ventricular outflow tract obstruction. The histopathological hallmarks of HCM are general myocyte hypertrophy, regions of myocyte disarray, different types of fibrosis and small-vessel disease. To better understand the disease development and the aetiology of the electrical instability leading to serious arrhythmias and sudden cardiac death (SCD) in these patients, it is important to explore the relationship between histopathological features and clinical findings and outcome in patients with HCM. This article provides a brief overview of this theme.

The relationship between left ventricular outflow tract gradient and sudden cardiac death in childhood hypertrophic cardiomyopathy

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
G Norrish ◽  
T Ding ◽  
E Field ◽  
C O'Mahony ◽  
P.M Elliott ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Inverse Relationship ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Funding Source ◽  
Left Ventricular Outflow ◽  
The Relationship

Abstract Background The most common mode of death in childhood hypertrophic cardiomyopathy (HCM) is sudden cardiac death (SCD). Left ventricular outflow tract obstruction (LVOTO) is an established risk factor for SCD in adults with the disease. In contrast, the prognostic implications of LVOTO in childhood disease is unclear, with recent studies suggesting that it may have an inverse relationship with the risk of SCD. Purpose The aim of this study was to explore the role of LVOTO and the risk of SCD in childhood HCM. Methods A multi-centre, retrospective, longitudinal cohort of 871 children (diagnosed with HCM <16 years of age) was used to explore the relationship between SCD and LVOTO (LVOT gradient ≥30mmHg). Results 189 patients (23%) had LVOTO, which was mild (30–50mmHg), moderate (50–100mmHg) or severe (>100mmHg) in 58 (6.7%), 98 (11.3%) and 33 (3.8%), respectively. The risk of SCD showed an inverse relation to LVOT gradient severity compared to those with no obstruction: mild HR 1.75 (95% CI 0.89–3.44), moderate HR 1.04 (95% 0.55–1.98), and severe HR 0.7 (0.36–1.35) [figure].On univariable analysis [table] LVOTO was associated with heart failure symptoms (NYHA>1) [p <0.001], maximal wall thickness (MWT) [p <0.001], left atrial (LA) diameter [p <0.001], and future myectomy occurring during follow up [p <0.001]. The inverse relationship observed was not altered by the presence or absence of other traditional risk factors. Conclusions LVOTO appears to have a complex relationship with the risk of SCD in childhood HCM, with multiple contributing factors. The pathophysiological mechanisms behind this observation need further exploration, which may be limited by low patient numbers Cummulative incidence of SCD by LVOTO Funding Acknowledgement Type of funding source: Other. Main funding source(s): British Heart Foundation

scholarly journals Small Vessel Disease: Another Component of the Hypertrophic Cardiomyopathy Phenotype Not Necessarily Associated with Fibrosis

2021 ◽  
Vol 10 (4) ◽  
pp. 575
Author(s):  
Monica De Gaspari ◽  
Cristina Basso ◽  
Martina Perazzolo Marra ◽  
Stefania Elia ◽  
Maria Bueno Marinas ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Clinical Presentation ◽  
Small Vessel Disease ◽  
Small Vessel ◽  
Chronic Ischemic Heart Disease ◽  
Vessel Disease ◽  
Septal Thickness ◽  
End Stage Heart Failure ◽  
End Stage ◽  
The Relationship

Background: Hypertrophic cardiomyopathy (HCM) is characterized by myocardial disarray, small vessel disease (SVD), and fibrosis. The relationship between SVD and replacement-type fibrosis is still unclear. Methods: Histopathologic assessment of replacement-type fibrosis and SVD in HCM patients with either end-stage heart failure (HF) or sudden cardiac death (SCD). Chronic ischemic heart disease (IHD) patients served as controls. Results: Forty HCM hearts, 10 HF and 30 SCD, were studied. Replacement-type fibrosis was detected in all HF and in 57% of SCD cases. In SCD, replacement-type fibrosis was associated with older age, greater septal thickness, SVD prevalence, and score (all p < 0.05). Prevalence of SVD did not show significant differences among SCD, HF, and IHD (73%, 100% and 95%, respectively), while SVD score was higher in HF than IHD and SCD (2.4, 1.95, and 1.18, respectively) and in areas with replacement-type fibrosis vs. those without in HF (3.4 vs. 1.4) and SCD (1.4 vs. 0.8) (all p < 0.05). Conclusions: SVD is a frequent feature in HCM independent of the clinical presentation. A higher SVD score is observed in HCM-HF and in areas with replacement-type fibrosis. Although SVD is part of the HCM phenotype, further remodeling of the microcirculation might occur secondarily to fibrosis.

scholarly journals Anesthetic Challenges in the Management of Intracranial Aneurysm Clipping in a Patient with Hypertrophic Cardiomyopathy

2020 ◽  
Author(s):  
Sakshi Duggal ◽  
Priyanka Khurana ◽  
Pragati Ganjoo ◽  
Nilima Das
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Perioperative Complications ◽  
Artery Aneurysm ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Anterior Communicating Artery ◽  
Drug Selection ◽  
Aneurysm Clipping ◽  
Left Ventricular Outflow ◽  
Life Threatening

AbstractAneurysmal surgeries are high-risk procedures due to potential for occurrence of fatal perioperative complications. This risk is exaggerated in the presence of co-existing hypertrophic cardiomyopathy (HCM). It involves asymmetrical hypertrophy of left ventricle with mitral valve dysfunction, leading to left ventricular outflow tract obstruction. Various perioperative factors may precipitate this obstruction resulting in life-threatening consequences. We report the management of a patient with HCM undergoing anterior communicating artery aneurysm clipping and discuss the anesthetic concerns. Comprehensive approach with careful drug selection, vigilant monitoring, and preparedness for complications enabled patient safety and a good neurological outcome.

Angina Pectoris Due to Severe Muscular Bridge in Hypertrophic Cardiomyopathy

1998 ◽  
Vol 6 (2) ◽  
pp. 132-134
Author(s):  
M Şah Topcuoĝlu ◽  
Ayhan Usal ◽  
Cem Kayhan ◽  
Aladdin Pekedis ◽  
Acar Tokcan ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Calcium Antagonist ◽  
Hypertrophic Cardiomyopathy ◽  
Angina Pectoris ◽  
Left Ventricular Outflow Tract ◽  
Young Patients ◽  
Ventricular Outflow Tract ◽  
Myocardial Scintigraphy ◽  
Left Ventricular ◽  
Left Ventricular Outflow

We report the case of a 39-year-old male with hypertrophic cardiomyopathy who complained of angina pectoris. The patient was treated with a beta blocker and a calcium antagonist without effect. Myocardial scintigraphy revealed anterior ischemia. Cardiac catheterization and ventriculography revealed severe systolic narrowing of the left anterior descending coronary artery and no significant pressure gradient across the left ventricular outflow tract. Myotomy was performed on a muscular bridge over the left anterior descending coronary artery and the patient's angina was relieved. In young patients with hypertrophic cardiomyopathy who develop angina refractory to medical therapy, a coexisting muscular bridge should be sought.

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