Multimodality Imaging in Hypertrophic Cardiomyopathy for Risk Stratification

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

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756 Left-ventricular outflow tract obstruction increases the risk of sudden cardiac death in patients with hypertrophic cardiomyopathy

European Heart Journal ◽

10.1016/s0195-668x(03)94164-9 ◽

2003 ◽

Vol 24 (5) ◽

pp. 137

Author(s):

L CHOJNOWSKA

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Sudden Cardiac Death ◽

Cardiac Death ◽

Left Ventricular Outflow Tract ◽

Ventricular Outflow Tract ◽

Left Ventricular ◽

Outflow Tract ◽

Ventricular Outflow Tract Obstruction ◽

Left Ventricular Outflow

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Characteristics and Outcomes of Elderly Patients With Hypertrophic Cardiomyopathy

Journal of the American Heart Association ◽

10.1161/jaha.120.018527 ◽

2021 ◽

Vol 10 (3) ◽

Author(s):

Alaa Alashi ◽

Nicholas G. Smedira ◽

Zoran B. Popovic ◽

Agostina Fava ◽

Maran Thamilarasan ◽

...

Keyword(s):

Risk Factors ◽

Hypertrophic Cardiomyopathy ◽

Elderly Patients ◽

Left Ventricular Outflow Tract ◽

Ventricular Outflow Tract ◽

Left Ventricular ◽

Outflow Tract ◽

Alcohol Ablation ◽

Left Ventricular Outflow ◽

Sudden Cardiac Death Risk

Background We report characteristics and outcomes of elderly patients with hypertrophic cardiomyopathy (HCM) with basal septal hypertrophy and dynamic left ventricular outflow tract obstruction. Methods and Results We studied 1110 consecutive elderly patients with HCM (excluding moderate or greater aortic stenosis or subaortic membrane, age 80±5 years [range, 75–92 years], 66% women), evaluated at our center between June 2002 and December 2018. Clinical and echocardiographic data, including maximal left ventricular outflow tract gradient, were recorded. The primary outcome was death and appropriate internal defibrillator discharge. Hypertension was observed in 72%, with a Society of Thoracic Surgeons (STS) score (8.6±6); while 80% had no HCM‐related sudden cardiac death risk factors. Left ventricular mass index, basal septal thickness, and maximal left ventricular outflow tract gradient were 127±43 g/m 2 , 1.7±0.4 cm, and 49±31 mm Hg, respectively. A total of 597 (54%) had a left ventricular outflow tract gradient >30 mm Hg, of which 195 (33%) underwent septal reduction therapy (SRT; 79% myectomy and 21% alcohol ablation). At 5.1±4 years, 556 (50%) had composite events (273 [53%] in nonobstructive, 220 [55%] in obstructive without SRT, and 63 [32%] in obstructive subgroup with SRT). One‐ and 5‐year survival, respectively were 93% and 63% in nonobstructive, 90% and 63% in obstructive subgroup without SRT, and 94% and 84% in the obstructive subgroup with SRT. Following SRT, there were 5 (2.5%) in‐hospital deaths (versus an expected Society of Thoracic Surgeons mortality of 9.2%). Conclusions Elderly patients with HCM have a high prevalence of traditional cardiovascular rather than HCM risk factors. Longer‐term outcomes of the obstructive SRT subgroup were similar to a normal age‐sex matched US population.

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Hypertrophic cardiomyopathy: prevention of sudden cardiac death

10.1093/med/9780198784906.003.0354 ◽

2018 ◽

Author(s):

Constantinos O’Mahony

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Sudden Cardiac Death ◽

Risk Stratification ◽

Cardiac Death ◽

Clinical Care ◽

Psychological Impact ◽

Ventricular Outflow Tract ◽

Left Ventricular ◽

Icd Implantation ◽

European Society Of Cardiology

Sudden cardiac death (SCD) secondary to ventricular arrhythmias is the most common mode of death in hypertrophic cardiomyopathy (HCM) and can be effectively prevented with an implantable cardioverter defibrillator (ICD). The risk of SCD in HCM relates to the severity of the phenotype and regular risk stratification is an integral part of routine clinical care. For the primary prevention of SCD, risk stratification involves the assessment of seven readily available clinical parameters (age, maximal left ventricular wall thickness, left atrial diameter, left ventricular outflow tract gradient, non-sustained ventricular tachycardia, unexplained syncope, and family history of SCD) which are used to estimate the risk of SCD within 5 years of clinical evaluation using a statistical risk prediction model (HCM Risk-SCD). The 2014 European Society of Cardiology Guidelines provide a framework to aid clinical decisions and consider patients with a 5-year risk of SCD of less than 4% as low risk and recommend regular assessment while those with a risk of 6% or higher should be considered for an ICD. In patients with an intermediate risk (4% to <6%) ICD implantation may also be considered after taking into account age, co-morbid conditions, socioeconomic factors, and the psychological impact of therapy. Survivors of ventricular fibrillation arrest should receive an ICD for secondary prevention unless their life expectancy is less than 1 year. Following device implantation, patients should be followed up for device- and disease-related complications, particularly heart failure and cerebrovascular disease.

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Hypertrophic Cardiomyopathy Mimicking Acute Anterior Myocardial Infarction Associated with Sudden Cardiac Death

Case Reports in Medicine ◽

10.1155/2012/236154 ◽

2012 ◽

Vol 2012 ◽

pp. 1-4 ◽

Cited By ~ 1

Author(s):

Y. Daralammouri ◽

M. El Garhy ◽

K. Same ◽

B. Lauer

Keyword(s):

Myocardial Infarction ◽

Hypertrophic Cardiomyopathy ◽

Sudden Cardiac Death ◽

Cardiac Death ◽

Valsalva Maneuver ◽

Ventricular Outflow Tract ◽

Hypertrophic Obstructive Cardiomyopathy ◽

Left Ventricular ◽

Anterior Myocardial Infarction ◽

Left Ventricular Outflow

Hypertrophic cardiomyopathy is the most common genetic disease of the heart. We report a rare case of hypertrophic obstructive cardiomyopathy mimicking an acute anterior myocardial infarction associated with sudden cardiac death. The patient presented with acute ST elevation myocardial infarction and significant elevation of cardiac enzymes. Cardiac catheterization showed some atherosclerotic coronary artery disease, without significant stenosis. Echocardiography showed left ventricular hypertrophy with a left ventricular outflow tract obstruction; the pressure gradient at rest was 20 mmHg and became severe with the Valsalva maneuver (100 mmHg). There was no family history of sudden cardiac death. Six days later, the patient suffered a syncope on his way to magnetic resonance imaging. He was successfully resuscitated by ventricular fibrillation.

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Outcomes following transaortic septal myectomy in hypertrophic cardiomyopathy

International Surgery Journal ◽

10.18203/2349-2902.isj20201060 ◽

2020 ◽

Vol 7 (4) ◽

pp. 1270

Author(s):

Madhusudan Kummari ◽

Amaresh Rao Malempati ◽

Surya S. Gopal Palanki ◽

Kaladhar Bomma

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Mitral Valve ◽

Cardiac Death ◽

Left Ventricular Outflow Tract ◽

Ventricular Outflow Tract ◽

Left Ventricular ◽

Septal Myectomy ◽

Left Ventricular Outflow ◽

History Of ◽

The Mean

Background: The objective of the study was to evaluate effect of myectomy and its impact on survival for a period of one year and to identify the co-morbid conditions that would increase the risk of surgery.Methods: The study was conducted on the patients admitted in a single unit of department of cardiothoracic surgery, NIMS, Hyderabad during the period of 2014 to 2018. The study was a retrospective observational study. 21 patients were enrolled in the study after approval from institute ethics committee. All the patients between 7 to 70 years who underwent septal myectomy were included in the study.Results: Out of the 21 patients underwent modified Morrows myectomy 16 (76.2%) were male and 5 (23.8%) were female. The most common presenting symptom was dyspnea (81%), followed chest pain (76%), palpitations (62%) and syncope (38%). 5 (24%) patients had a family history of sudden cardiac death. Preoperative beta blockers were used by 15 (72%) patients. 11 patients had severe mitral regurgitation, out of which 8 patients underwent valve replacement and 3 underwent mitral valve repair. The mean preoperative left ventricular outflow tract obstruction gradient was 86.86 and the mean postoperative gradient was 23.47. 3 patients had implantable cardioverter defibrillator insertions. All patients had symptom relief.Conclusions: Surgical treatment of hypertrophic cardiomyopathy through transaortic septal myectomy is safe and effective method to relieve left ventricular outflow tract obstruction. Mitral valve replacement can be done for cases with structural defect of mitral valve. Early detection and intervention in patients with family history of sudden cardiac death would reduce the risk of death and ensure long term survival.

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Hypertrophic cardiomyopathy: prevention of sudden cardiac death

ESC CardioMed ◽

10.1093/med/9780198784906.003.0354_update_001 ◽

2018 ◽

pp. 1462-1466

Author(s):

Constantinos O’Mahony

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Sudden Cardiac Death ◽

Risk Stratification ◽

Cardiac Death ◽

Clinical Care ◽

Psychological Impact ◽

Ventricular Outflow Tract ◽

Left Ventricular ◽

Icd Implantation ◽

European Society Of Cardiology

Sudden cardiac death (SCD) secondary to ventricular arrhythmias is the most common mode of death in hypertrophic cardiomyopathy (HCM) and can be effectively prevented with an implantable cardioverter defibrillator (ICD). The risk of SCD in HCM relates to the severity of the phenotype and regular risk stratification is an integral part of routine clinical care. For the primary prevention of SCD, risk stratification involves the assessment of seven readily available clinical parameters (age, maximal left ventricular wall thickness, left atrial diameter, left ventricular outflow tract gradient, non-sustained ventricular tachycardia, unexplained syncope, and family history of SCD) which are used to estimate the risk of SCD within 5 years of clinical evaluation using a statistical risk prediction model (HCM Risk-SCD). The 2014 European Society of Cardiology Guidelines provide a framework to aid clinical decisions and consider patients with a 5-year risk of SCD of less than 4% as low risk and recommend regular assessment while those with a risk of 6% or higher should be considered for an ICD. In patients with an intermediate risk (4% to <6%) ICD implantation may also be considered after taking into account age, co-morbid conditions, socioeconomic factors, and the psychological impact of therapy. Survivors of ventricular fibrillation arrest should receive an ICD for secondary prevention unless their life expectancy is less than 1 year. Following device implantation, patients should be followed up for device- and disease-related complications, particularly heart failure and cerebrovascular disease.

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Sudden Cardiac Death: A Modern Pathology Approach to Hypertrophic Cardiomyopathy

Archives of Pathology & Laboratory Medicine ◽

10.5858/arpa.2013-0489-rs ◽

2015 ◽

Vol 139 (3) ◽

pp. 413-416 ◽

Cited By ~ 4

Author(s):

Linda Kocovski ◽

John Fernandes

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Sudden Cardiac Death ◽

Cardiac Death ◽

Cardiac Myocyte ◽

Clinical Symptoms ◽

Ventricular Outflow Tract ◽

Left Ventricular ◽

Exertional Dyspnea ◽

Left Ventricular Outflow ◽

Cardiac Myocyte Hypertrophy

Hypertrophic cardiomyopathy is one of the most common causes of sudden cardiac death among young adults and adolescents. Unfortunately, the first manifestation of the condition may be sudden death during exertion, such as sporting activities. Other clinical symptoms include exertional dyspnea, angina, and syncope. Postmortem examination often reveals asymmetrical septal thickening and mural plaque formation in the left ventricular outflow tract. Histologic analysis shows cardiac myocyte hypertrophy, myofiber disarray, and interstitial and replacement fibrosis. Molecular analysis for known genetic abnormalities is essential to genetic counseling of living relatives of decedents to assess and reduce the risk of sudden cardiac death from hypertrophic cardiomyopathy.

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