Dilated phase of hypertrophic cardiomyopathy: high-risk reconstructive surgery as an alternative to heart transplantation

2021 ◽  
pp. 31
Author(s):  
S.L. Dzemeshkevich ◽  
A.P. Motreva ◽  
O.V. Kalmykova ◽  
Yu.B. Martyanova ◽  
D.G. Tarasov ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
High Risk ◽  
Heart Transplantation ◽  
Reconstructive Surgery

scholarly journals Post-transplant absolute lymphocyte count predicts early cytomegalovirus infection after heart transplantation

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Minjae Yoon ◽  
Jaewon Oh ◽  
Kyeong-Hyeon Chun ◽  
Chan Joo Lee ◽  
Seok-Min Kang
Keyword(s):  
High Risk ◽  
Heart Transplantation ◽  
Immunosuppressive Therapy ◽  
Lymphocyte Count ◽  
Cytomegalovirus Infection ◽  
Absolute Lymphocyte Count ◽  
Cmv Infection ◽  
Igg Antibody ◽  
Post Transplant ◽  
The Relationship

AbstractImmunosuppressive therapy can decrease rejection episodes and increase the risk of severe and fatal infections in heart transplantation (HT) recipients. Immunosuppressive therapy can also decrease the absolute lymphocyte count (ALC), but the relationship between early post-transplant ALC and early cytomegalovirus (CMV) infection is largely unknown, especially in HT. We retrospectively analyzed 58 HT recipients who tested positive for CMV IgG antibody and received basiliximab induction therapy. We collected preoperative and 2-month postoperative data on ALC and CMV load. The CMV load > 1200 IU/mL was used as the cutoff value to define early CMV infection. Post-transplant lymphopenia was defined as an ALC of < 500 cells/μL at postoperative day (POD) #7. On POD #7, 29 (50.0%) patients had post-transplant lymphopenia and 29 (50.0%) patients did not. The incidence of CMV infection within 1 or 2 months of HT was higher in the post-transplant lymphopenia group than in the non-lymphopenia group (82.8% vs. 48.3%, P = 0.013; 89.7% vs. 65.5%, P = 0.028, respectively). ALC < 500 cells/μL on POD #7 was an independent risk factor for early CMV infection within 1 month of HT (odds ratio, 4.14; 95% confidence interval, 1.16–14.77; P = 0.029). A low ALC after HT was associated with a high risk of early CMV infection. Post-transplant ALC monitoring is simple and inexpensive and can help identify patients at high risk of early CMV infection.

scholarly journals The Indices of Cardiovascular Magnetic Resonance Derived Atrial Dynamics May Improve the Contemporary Risk Stratification Algorithms in Children with Hypertrophic Cardiomyopathy

2021 ◽  
Vol 10 (4) ◽  
pp. 650
Author(s):  
Lidia Ziółkowska ◽  
Łukasz Mazurkiewicz ◽  
Joanna Petryka ◽  
Monika Kowalczyk-Domagała ◽  
Agnieszka Boruc ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
High Risk ◽  
Risk Stratification ◽  
Heart Association ◽  
The Novel ◽  
Identification Performance ◽  
Strong Trend ◽  
Prognostic Utility ◽  
Conduit Function ◽  
High Risk Children

Introduction: The most efficient risk stratification algorithms are expected to deliver robust and indefectible identification of high-risk children with hypertrophic cardiomyopathy (HCM). Here we compare algorithms for risk stratification in primary prevention in HCM children and investigate whether novel indices of biatrial performance improve these algorithms. Methods and Results: The endpoints were defined as sudden cardiac death, resuscitated cardiac arrest, or appropriate implantable cardioverter-defibrillator discharge. We examined the prognostic utility of classic American College of Cardiology/American Heart Association (ACC/AHA) risk factors, the novel HCM Risk-Kids score and the combination of these with indices of biatrial dynamics. The study consisted of 55 HCM children (mean age 12.5 ± 4.6 years, 69.1% males); seven had endpoints (four deaths, three appropriate ICD discharges). A strong trend (DeLong p = 0.08) was observed towards better endpoint identification performance of the HCM Risk-Kids Model compared to the ACC/AHA strategy. Adding the atrial conduit function component significantly improved the prediction capabilities of the AHA/ACC Model (DeLong p = 0.01) and HCM Risk-Kids algorithm (DeLong p = 0.04). Conclusions: The new HCM Risk-Kids individualised algorithm and score was capable of identifying high-risk children with very good accuracy. The inclusion of one of the atrial dynamic indices improved both risk stratification strategies.

Adult Reconstructive Surgery: A High-Risk Profession for Work-Related Injuries

2016 ◽  
Vol 31 (6) ◽  
pp. 1194-1198 ◽  
Author(s):  
Saad M. Alqahtani ◽  
Mohammad M. Alzahrani ◽  
Michael Tanzer
Keyword(s):  
High Risk ◽  
Reconstructive Surgery ◽  
Work Related

Hypertrophic cardiomyopathy and mtDNA depletion. Successful treatment with heart transplantation

2002 ◽  
Vol 12 (1) ◽  
pp. 56-59 ◽  
Author(s):  
F.M. Santorelli ◽  
M.G. Gagliardi ◽  
C. Dionisi-Vici ◽  
F. Parisi ◽  
A. Tessa ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Heart Transplantation ◽  
Successful Treatment ◽  
Mtdna Depletion

Heart transplantation in high risk patients: Post-operative management using OKT3

1990 ◽  
Vol 4 (6) ◽  
pp. 75
Author(s):  
J. Neidecker ◽  
P. Brule ◽  
M. Gressier ◽  
J. Ninet ◽  
D. Bompard ◽  
...  
Keyword(s):  
High Risk ◽  
Heart Transplantation ◽  
Operative Management ◽  
High Risk Patients ◽  
Risk Patients

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.

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.

Abstract 236: Obstructive Sleep Apnea in Hypertrophic Cardiomyopathy: Comparing Exercise Tolerance, Cardiac Remodeling, Clinical and Genetic Data

2014 ◽  
Vol 7 (suppl_1) ◽  
Author(s):  
Suwen Kumar ◽  
Benjamin Ebner ◽  
Jeniffer Bragg-Gresham ◽  
Peter Farrehi ◽  
Sharlene Day
Keyword(s):  
Obstructive Sleep Apnea ◽  
Sleep Apnea ◽  
Hypertrophic Cardiomyopathy ◽  
High Risk ◽  
Cardiac Remodeling ◽  
Exercise Tolerance ◽  
Functional Class ◽  
Nyha Functional Class ◽  
Obstructive Sleep ◽  
Lv Mass

Background: Prevalence of obstructive sleep apnea (OSA) in hypertrophic cardiomyopathy (HCM) is estimated between 32% and 71%. Individuals with HCM and OSA have increased blood pressure, BMI, , ascending aorta size, left atrial size, left ventricular end diastolic diameter, E/e’ ratio, atrial fibrillation rates and NYHA functional class. It has been suggested that treatment of OSA can decrease the need for septal reduction. However, studies have found no effect of OSA on septal thickness or outflow gradient. It is not known how OSA affects exercise performance or cardiac remodeling assessed by MR. Genetic propensity toward OSA in HCM has not been reported. We propose that OSA predicts decrease exercise tolerance and that cardiac remodeling could be identified using MR. We sought to report on HCM genotype in OSA as well as compare our clinical and echo data with other investigators. Methods: Subjects were identified through our institution’s HCM database. They were surveyed using the STOP-BANG (SB) questionnaire, a validated questionnaire to identify individuals at high risk for OSA. We stratified patients into high risk (HR) and low risk (LR) groups, based on a cut point of greater than or equal to 3 on SB. Demographics and clinical characteristics were extracted from our database. Prevalence and means were compared between the two groups, using Chi-square and t-tests. Differences between the groups were adjusted for age, sex, and BMI using linear mixed models for continuous measures and logistic regression for dichotomous measures. Results: There were 206 respondents, of those 160 (78%) scored high risk for OSA, 60 of which had a history of polysomnogram (PSG) confirming OSA. Having a HR vs. LR SB was associated with a significantly greater likelihood of stroke, CHF hospitalization, NYHA functional class >2, reduced peak VO2, reduced anaerobic threshold and increased LA diameter. Adjusted comparisons for age, gender, and BMI showed that had significantly higher PAWP and LV mass index. Of those with a prior diagnosis of OSA we compared therapy compliant and non-compliant individuals and found they differed on LV mass index (HR=98.7 g/m2 vs. LR=62.0 g/m2, p=0.01). Conclusions: OSA occurs frequently in HCM and is associated with decreased exercise tolerance, worse hemodynamics, poor outcome as well as increased LV mass, which may be attenuated by therapy. OSA is an important and modifiable risk factor in HCM. Prospective evaluation utilizing PSG based diagnosis and positive pressure therapy is warranted.

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