scholarly journals Heart transplantation in patients with hypertrophic cardiomyopathy

2018 ◽  
Vol 2018 (3) ◽  
Author(s):  
Marta Farrero Torres ◽  
Felix Perez-Villa
Keyword(s):  
Heart Failure ◽  
Hypertrophic Cardiomyopathy ◽  
Life Expectancy ◽  
Sudden Death ◽  
Systolic Dysfunction ◽  
Treatment Options ◽  
Advanced Heart Failure ◽  
Clinical Presentations ◽  
End Stage ◽  
Ventricular Filling

[first paragraph of article]Hypertrophic cardiomyopathy has a broad spectrum of clinical presentations, from asymptomatic to patients with advanced heart failure and sudden death. Treatment options are limited, especially in non-obstructive forms. A minority of patients (around 3.5%) can progress to an end-stage state, characterized by systolic dysfunction and restrictive ventricular filling, related to extensive fibrotic replacement and chamber remodeling. In these cases, life expectancy is significantly reduced: a mean 3-year survival time has been reported.

Abstract 4876: LAMP2 Cardiomyopathy is a Particularly Adverse Phenocopy of Sarcomeric Hypertrophic Cardiomyopathy

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Barry Maron ◽  
William C Roberts ◽  
Michael Arad ◽  
Carolyn Y Ho ◽  
Tammy S Haas ◽  
...  
Keyword(s):  
Heart Failure ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Death ◽  
Heart Transplantation ◽  
Systolic Dysfunction ◽  
Ventricular Tachyarrhythmia ◽  
Young Patients ◽  
Disease Process ◽  
Left Ventricular ◽  
Asymptomatic Patients

Mutations in the X-linked lysosome-associated membrane protein gene (LAMP2; Danon disease) produce a morphologic phenocopy of sarcomeric hypertrophic cardiomyopathy (HCM) in young patients, characterized by extreme left ventricular (LV) hypertrophy and pre-excitation. However, the natural history of this newly recognized cardiomyopathy is incompletely resolved. Seven young asymptomatic patients with LAMP2 cardiomyopathy were identified at ages 8 to 15 years; 6 were male. LV hypertrophy was particularly marked (septal thickness 25– 65 mm; mean 42±17) in the presence of nondilated LV cavity. On each ECG, Wolff-Parkinson-White pre-excitation pattern was associated with markedly increased voltages (74±38mm for R- or S-wave). Over the 7±3 year follow-up from initial cardiac diagnosis, all 7 patients experienced particularly adverse disease consequences associated with progressive LV wall thinning and cavity dilatation and systolic dysfunction (ejection fraction, 29±7%) by the ages of 12 – 24 years (mean 20). Of the 7 patients, 5 either died of progressive heart failure, had heart transplantation or were considered for a donor heart; 2 others had sudden death events, including one fatal ventricular tachyarrhythmia refractory to defibrillator therapy and one appropriate defibrillator shock in an asymptomatic female survivor. Pathologic examination of hearts at autopsy showed histopathologic findings compatible with both HCM due to sarcomere protein mutations (i.e., extensive myocyte disarray, intramural small vessel disease, myocardial replacement scarring), and also evidence of a storage disease process (i.e., clusters of myocytes with vacuolated sarcoplasm within fibrotic areas). Heart weights, 1266 and 1425 grams, are the most substantial recorded for hypertrophic cardiomyopathies. LAMP2 cardiomyopathy is a uniformly profound, and particularly deleterious disease entity, causing refractory heart failure with systolic dysfunction as well as sudden death in young patients < 25 years of age. This novel phenocopy of sarcomeric HCM underscores the power of molecular diagnosis for predicting prognosis, and should also raise consideration for intervention with early heart transplantation.

“I Just Feel Like I Always Did”: Inotropic Dependency at End of Life

2019 ◽  
Vol 37 (7) ◽  
pp. 497-502
Author(s):  
Abigail Latimer ◽  
Natalie D. Pope ◽  
Jessica M. McFarlin
Keyword(s):  
Heart Failure ◽  
End Of Life ◽  
Health Care Professionals ◽  
Treatment Options ◽  
Advanced Heart Failure ◽  
Inotropic Support ◽  
Circulatory Support ◽  
Inotropic Therapy ◽  
Her Family ◽  
End Stage

Background: Patients not considered for mechanical circulatory support or heart transplant may be dependent on inotropic therapy at end of life. End-of-life conversations in advanced heart failure can be challenging for providers, but guidelines recommend frequent goals-of-care conversations when inotropes are used as a palliative treatment. The purpose of this study was to identify aspects of care pertinent for health-care professionals working with patients in end-stage heart failure who are receiving continuous inotropic support. Methods: Qualitative analysis was used to examine 3 audio-recorded semistructured interviews with 1 patient, her family, and her cardiologist. The selected patient was an older adult, diagnosed with advanced heart failure, and dependent on continuous inotropic therapy with no other advanced treatment options available. Results: The analysis revealed that (1) reliance on others, (2) contending with uncertainty, and (3) deciding when to discontinue inotropic support were identified as themes central to the patient’s and provider’s experience. Conclusion: This study offers insight into how to best support and communicate with patients having advanced heart failure who are dependent on continuous inotropic therapy at end of life.

Abstract 3097: Significance of Delayed Enhancement with Contrast Enhanced Cardiovascular Magnetic Resonance in Hypertrophic Cardiomyopathy

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Martin S Maron ◽  
Evan Appelbaum ◽  
Caitlin Harrigan ◽  
jacki Buros ◽  
C. Michael Gibson ◽  
...  
Keyword(s):  
Heart Failure ◽  
Cardiovascular Magnetic Resonance ◽  
Hypertrophic Cardiomyopathy ◽  
Magnetic Resonance ◽  
Independent Predictor ◽  
Systolic Dysfunction ◽  
Delayed Enhancement ◽  
Cardiovascular Morbidity And Mortality ◽  
End Stage

Background. Cardiovascular magnetic resonance (CMR) with delayed enhancement (DE) can provide in vivo assessment of myocardial fibrosis. DE is an independent predictor of cardiovascular morbidity and mortality in ischemic and non-ischemic dilated cardiomyopathy but its clinical significance in hypertrophic cardiomyopathy (HCM) remains unresolved. Methods and Results. Cine and CMR-DE were performed in 202 HCM patients (42±17 years; 71% male). DE was identified in 103 (51%) HCM patients, occupying 9±11% (range 0.2 to 51%) of LV myocardial volume, including 12% with DE > 25%. DE was present all 10 patients with ejection fraction (EF) ≤50% (i.e., end-stage phase), 9/10 (90%) with EF 51–59% and 84/182 (45%) with EF ≥ 60% (p<0.001). %DE was inversely related to EF (r= −0.4; p< 0.001), most extensive in patients with EF ≤50% (27% vs.9% for other patients, p<0.001) and an independent predictor of EF (r= −0.6; p<0.001). Of the 182 patients with normal EF ≥60%, 49 (27%) were both asymptomatic and had DE (7±7% of LV; 51% transmural), including 7 patients ≥60 years. %DE was unassociated with heart failure symptoms (p=0.1) and age (r = 0.05; p = 0.6). Conclusions. In a large HCM cohort, DE was common and occupied substantial areas of LV myocardium. Importantly, DE was a strong and independent predictor of evolution to “end-stage” with systolic dysfunction. However, extensive DE was also present in many patients without heart failure symptoms, suggesting that such HCM patients undergo vigilant clinical surveillance (including serial CMR) for prospective detection of changes in clinical course and LV remodeling.

Apical myectomy in patients with apical hypertrophic cardiomyopathy and advanced heart failure

2021 ◽  
Author(s):  
Alberto Forteza ◽  
Juan Esteban de Villareal ◽  
Carlos Esteban Martín ◽  
Susana Mingo ◽  
Jessica García-Suárez ◽  
...  
Keyword(s):  
Heart Failure ◽  
Hypertrophic Cardiomyopathy ◽  
Advanced Heart Failure ◽  
Apical Hypertrophic Cardiomyopathy

Abstract 13943: Hypertrophic Cardiomyopathy with Left Ventricular Apical Aneurysm Represents a High-Risk Subgroup Necessitating Aggressive Preventive Therapy: A Long-term Follow-Up Study

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Ethan J Rowin ◽  
Barry J Maron ◽  
Tammy S Haas ◽  
John R Lesser ◽  
Mark S Link ◽  
...  
Keyword(s):  
Heart Failure ◽  
Hypertrophic Cardiomyopathy ◽  
High Risk ◽  
Sudden Death ◽  
Left Ventricular ◽  
Increased Risk ◽  
Long Term Follow Up ◽  
Apical Aneurysm

Background: Increasing penetration of high spatial resolution cardiovascular magnetic resonance (CMR) imaging into routine cardiovascular practice has resulted in more frequent identification of a subset of hypertrophic cardiomyopathy (HCM) patients with thin-walled, scarred left ventricular (LV) apical aneurysms. Prior experience involved relatively small numbers of patients with short follow-up and therefore the risk associated with this subgroup remains incompletely defined. Therefore, we assembled a large HCM cohort with LV apical aneurysms and long-term follow-up in order to clarify clinical course and prognosis. Methods and Results: Of 2,400 HCM patients, 60 (2.5%) were identified by CMR with LV apical aneurysm, 24 to 86 years of age, including 19 (32%) <45 years old; 70% male, and followed for 5.6 ± 3.5 years. Over the follow-up period, 24 patients experienced 31 adverse disease-related complications including: appropriate implantable cardioverter-defibrillator discharge for VT/VF (n=11), received or listed for heart transplant (n=6), heart failure death (n=5), nonfatal thromboembolic events (n=4), resuscitated out-of-hospital cardiac arrest (n=3), and sudden death (n=2). In addition, an intracavitary thrombus was identified in the apical aneurysm in 9 patients without a thromboembolic history. Combined HCM-related death and aborted life threatening event rate was 8.6% per year, nearly 6-fold greater than the 1.5% annual mortality rate reported in the general HCM population. Conclusions: Patients with LV apical aneurysms represent a high-risk subgroup within the diverse HCM spectrum, associated with substantial increased risk for disease-related morbidity and mortality, including advanced heart failure, thromboembolic stroke and sudden death. Identification of this unique HCM phenotype should prompt consideration for primary prevention ICD, and anticoagulation for stroke prophylaxis.

MIOCARDIOPATIA NÃO COMPACTADA - UMA REVISÃO DA LITERATURA

2018 ◽  
Vol 5 (2) ◽  
pp. 74-78
Author(s):  
Danilo Silva Sousa ◽  
Eduardo Akio Pereira I ◽  
Carlos Roberto de Oliveira Júnior ◽  
Ricardo Mendonça de Paula ◽  
Genildo Ferreira Nunes
Keyword(s):  
Heart Failure ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Death ◽  
Heart Association ◽  
Clinical Findings ◽  
Left Ventricular ◽  
World Health ◽  
Main Diagnosis ◽  
Insuficiencia Cardiaca ◽  
Health Organization

RESUMO Introdução - A miocardiopatia não compactada (MNC) é uma patologia de ocorrência familiar com histórico relevante de morte súbita e insuficiência cardíaca; é considerada como uma miocardiopatia não classificada pela Organização Mundial da Saúde (OMS). Segundo a Associação Americana de Cardiologia, é uma cardiomiopatia primária. Desenvolvimento - A MNC resulta de uma alteração genética que leva a uma parada do processo de compactação miocárdica, caracterizada pela persistência de trabeculações e recessos profundos que se comunicam com a cavidade ventricular. A apresentação clínica inicial varia de assintomático a apresentação de sintomas relacionados à dor torácica, insuficiência cardíaca e arritmias. Os principais métodos de diagnóstico para MNC são estudos ecocardiográficos e ressonância magnética cardíaca, tendo como principais diagnósticos diferenciais a forma apical de cardiomiopatia hipertrófica, a combinação de cardiomiopatia hipertrófica apical e MNC, cardiomiopatia hipertensiva, fibroelastose endocárdica, trombo apical ou tumores entre outros. Considerações finais - A terapêutica disponível inclui tratamento para insuficiência cardíaca, arritmias e eventos tromboembólicos, mas é necessário particularizar a terapêutica relacionada à anticoagulação e prevenção primária de morte súbita cardíaca.   Palavras-chave: Cardiomiopatias; insuficiência cardíaca; arritmias cardíacas. ABSTRACT The left ventricular noncompaction (LVNC) is a patology with a familiar occurrence and relevant historic of sudden death and heart failure; it's considered an unclassified cardiomyopathy by World Health Organization (WHO). According to American Heart Association (AHA), it's a primary cardiomyopathy. Development - The LVNC results in a genetic alteration which leads to stop miocardial compaction process, characterized by persistence of trabeculation and deep intertrabecular recesses communicating with the ventricular cavity. The initial clinical findings may vary from asymptomatic to chest pain, heart failure and arrhythmias. The main diagnosis methods for LVNC are echocardiography and cardiac magnetic resonance imaging, having as main differential diagnosis the apical form of hypertrophic cardiomyopathy, a combination of both apical hypertrophic cardiomyopathy and LVNC, hypertensive cardiomyopathy, endocardial fibroelastosis, apical thrombus, or tumours among others. Final considerations - The menagement available includes the treatment for heart failure, arrhythmias and thromboembolic events, but it's neceassary particularize the therapeutics related to anticoagulation and primary prevention of sudden death. Keywords: Cardiomyopathies; heart failure; cardiac arrhythmias.

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