23 results on '"Lesser, John R."'
Search Results
2. Effect of Left Ventricular Outflow Tract Obstruction on Left Atrial Mechanics in Hypertrophic Cardiomyopathy.
- Author
-
Williams LK, Chan RH, Carasso S, Durand M, Misurka J, Crean AM, Ralph-Edwards A, Gruner C, Woo A, Lesser JR, Maron BJ, Maron MS, and Rakowski H
- Subjects
- Aged, Cardiomyopathy, Hypertrophic diagnostic imaging, Echocardiography, Female, Heart Atria diagnostic imaging, Heart Septum pathology, Humans, Male, Middle Aged, Radiography, Ventricular Dysfunction, Left diagnostic imaging, Cardiomyopathy, Hypertrophic physiopathology, Heart Atria physiopathology, Magnetic Resonance Imaging, Ventricular Dysfunction, Left physiopathology
- Abstract
Left atrial (LA) volumes are known to be increased in hypertrophic cardiomyopathy (HCM) and are a predictor of adverse outcome. In addition, LA function is impaired and is presumed to be due to left ventricular (LV) diastolic dysfunction as a result of hypertrophy and myocardial fibrosis. In the current study, we assess the incremental effect of outflow tract obstruction (and concomitant mitral regurgitation) on LA function as assessed by LA strain. Patients with HCM (50 obstructive, 50 nonobstructive) were compared to 50 normal controls. A subset of obstructive patients who had undergone septal myectomy was also studied. Utilising feature-tracking software applied to cardiovascular magnetic resonance images, LA volumes and functional parameters were calculated. LA volumes were significantly elevated and LA ejection fraction and strain were significantly reduced in patients with HCM compared with controls and were significantly more affected in patients with obstruction. LA volumes and function were significantly improved after septal myectomy. LVOT obstruction and mitral regurgitation appear to further impair LA mechanics. Septal myectomy results in a significant reduction in LA volumes, paralleled by an improvement in function.
- Published
- 2015
- Full Text
- View/download PDF
3. Intermediate-signal-intensity late gadolinium enhancement predicts ventricular tachyarrhythmias in patients with hypertrophic cardiomyopathy.
- Author
-
Appelbaum E, Maron BJ, Adabag S, Hauser TH, Lesser JR, Haas TS, Riley AB, Harrigan CJ, Delling FN, Udelson JE, Gibson CM, Manning WJ, and Maron MS
- Subjects
- Adult, Cardiomyopathy, Hypertrophic complications, Cohort Studies, Electrocardiography, Ambulatory, Female, Humans, Image Processing, Computer-Assisted, Magnetic Resonance Imaging, Cine, Male, Predictive Value of Tests, Tachycardia, Ventricular complications, Cardiomyopathy, Hypertrophic pathology, Contrast Media, Gadolinium DTPA, Image Enhancement methods, Magnetic Resonance Imaging methods, Tachycardia, Ventricular pathology
- Abstract
Background: In hypertrophic cardiomyopathy (HCM), the arrhythmic potential associated with a variety of left ventricular myocardial signal intensities evident on contrast-enhanced cardiovascular magnetic resonance with late gadolinium enhancement (LGE) is unresolved., Methods and Results: In 145 HCM patients (43±15 years old), visually identified areas of LGE in left ventricle were analyzed quantitatively for intermediate (≥4 but <6 SD) and high (≥6 SD above the mean signal intensity of normal myocardium) LGE signal intensity (LGE-SI). Ambulatory Holter ECGs were obtained within 7.8±8.3 weeks of cardiovascular magnetic resonance. HCM patients with nonsustained ventricular tachycardia, ventricular couplets, and premature ventricular contractions showed greater amounts of intermediate LGE-SI (17±7 versus 10±10 g, 16±10 versus 10±11 g, and 13±8 versus 10±13 g, respectively; P=0.003 to <0.001) and greater amounts of high LGE-SI (15±6 versus 10±8 g, 14±9 versus 10±12 g, and 12±7 versus 10±8 g, respectively; P=0.02-0.003) than patients without these arrhythmias. In HCM patients with either nonsustained ventricular tachycardia, couplets, or premature ventricular contractions, the extent of intermediate LGE-SI exceeded that of high LGE-SI (17±7 versus 15±6 g, 16±10 versus 14±9 g, and 13±8 versus 12±7 g, respectively; P=0.01-0.04). In addition, the receiver operating characteristic area under the curve established intermediate LGE-SI as a better discriminator of patients with nonsustained ventricular tachycardia than was high LGE-SI, with 7 additional patients with this arrhythmia identified., Conclusions: In patients with HCM, intermediate LGE-SI is a better predictor of ventricular tachyarrhythmias (including nonsustained ventricular tachycardia, a risk factor for sudden death) than is high LGE-SI. Longitudinal studies in larger HCM cohorts are justified to define the independent prognostic impact of intermediate LGE-SI.
- Published
- 2012
- Full Text
- View/download PDF
4. CMR with late gadolinium enhancement in genotype positive-phenotype negative hypertrophic cardiomyopathy.
- Author
-
Rowin EJ, Maron MS, Lesser JR, and Maron BJ
- Subjects
- Adult, Cardiomyopathy, Hypertrophic, Familial physiopathology, Female, Fibrosis, Genetic Predisposition to Disease, Humans, Male, Phenotype, Predictive Value of Tests, Stroke Volume, Ventricular Function, Left, Young Adult, Cardiomyopathy, Hypertrophic, Familial genetics, Cardiomyopathy, Hypertrophic, Familial pathology, Contrast Media, Gadolinium, Magnetic Resonance Imaging, Myocardium pathology
- Published
- 2012
- Full Text
- View/download PDF
5. Mitral valve abnormalities identified by cardiovascular magnetic resonance represent a primary phenotypic expression of hypertrophic cardiomyopathy.
- Author
-
Maron MS, Olivotto I, Harrigan C, Appelbaum E, Gibson CM, Lesser JR, Haas TS, Udelson JE, Manning WJ, and Maron BJ
- Subjects
- Adolescent, Adult, Aged, Aged, 80 and over, Cardiomyopathy, Hypertrophic physiopathology, Case-Control Studies, Child, Cohort Studies, Female, Heart Ventricles pathology, Heart Ventricles physiopathology, Humans, Hypertrophy, Left Ventricular pathology, Hypertrophy, Left Ventricular physiopathology, Male, Middle Aged, Mitral Valve physiopathology, Ventricular Outflow Obstruction pathology, Ventricular Outflow Obstruction physiopathology, Young Adult, Cardiomyopathy, Hypertrophic pathology, Magnetic Resonance Imaging, Mitral Valve pathology, Phenotype
- Abstract
Background: Whether morphological abnormalities of the mitral valve represent part of the hypertrophic cardiomyopathy (HCM) disease process is unresolved. Therefore, we applied cardiovascular magnetic resonance to characterize mitral valve morphology in a large HCM cohort., Methods and Results: Cine cardiac magnetic resonance images were obtained in 172 HCM patients (age, 42±18 years; 62% men) and 172 control subjects. In addition, 15 HCM gene-positive/phenotype-negative relatives were studied. Anterior mitral leaflet (AML) and posterior mitral leaflet lengths were greater in HCM patients than in control subjects (26±5 versus 19±5 mm, P<0.001; and 14±4 versus 10±3 mm, P<0.001, respectively), including 59 patients (34%) in whom AML length alone, posterior mitral leaflet length alone, or both were particularly substantial (>2 SDs above controls). Leaflet length was increased compared with controls in virtually all HCM age groups, including young patients 15 to 20 years of age (AML, 26±5 versus 21±4 mm; P=0.0002) and those ≥60 years of age (AML, 26±4 versus 19±2 mm; P<0.001). No relation was evident between mitral leaflet length and LV thickness or mass index (P=0.09 and P=0.16, respectively). A ratio of AML length to LV outflow tract diameter of >2.0 was associated with subaortic obstruction (P=0.001). In addition, AML length in 15 genotype-positive relatives without LV hypertrophy exceeded that of matched control subjects (21±3 versus 18±3 mm; P<0.01)., Conclusions: In HCM, mitral valve leaflets are elongated independently of other disease variables, likely constituting a primary phenotypic expression of this heterogeneous disease, and are an important morphological abnormality responsible for LV outflow obstruction in combination with small outflow tract dimension. These findings suggest a novel role for cardiac magnetic resonance in the assessment of HCM.
- Published
- 2011
- Full Text
- View/download PDF
6. Management implications of massive left ventricular hypertrophy in hypertrophic cardiomyopathy significantly underestimated by echocardiography but identified by cardiovascular magnetic resonance.
- Author
-
Maron MS, Lesser JR, and Maron BJ
- Subjects
- Adult, Cardiomyopathy, Hypertrophic diagnosis, Cardiomyopathy, Hypertrophic physiopathology, Diagnosis, Differential, Diagnostic Errors, Follow-Up Studies, Humans, Hypertrophy, Left Ventricular diagnosis, Hypertrophy, Left Ventricular etiology, Male, Middle Aged, Severity of Illness Index, Stroke Volume, Tomography, X-Ray Computed, Ventricular Function, Left, Cardiomyopathy, Hypertrophic complications, Defibrillators, Implantable, Echocardiography methods, Electric Countershock instrumentation, Hypertrophy, Left Ventricular therapy, Magnetic Resonance Imaging methods
- Abstract
Cardiovascular magnetic resonance (CMR) is a high spatial resolution, 3-dimensional tomographic imaging technique which may identify regions of massive left ventricular hypertrophy (particularly when confined to the anterolateral free wall) in which the extent of wall thickness is underestimated with traditional 2-dimensional echocardiography in patients with hypertrophic cardiomyopathy (HC). This observation may have potential implications on management strategies as extreme left ventricular hypertrophy is a primary risk factor for sudden death in HC and therefore supports an expanding role for CMR in the evaluation of HC patients.
- Published
- 2010
- Full Text
- View/download PDF
7. Disparity between unusual left ventricular morphology and clinical presentation and course in hypertrophic cardiomyopathy.
- Author
-
Maron BJ, Lindberg J, Haas TS, Kitner C, and Lesser JR
- Subjects
- Adult, Aged, Aged, 80 and over, Diagnosis, Differential, Echocardiography, Heart Failure diagnosis, Humans, Hypertrophy, Left Ventricular diagnosis, Severity of Illness Index, Cardiomyopathy, Hypertrophic diagnosis, Magnetic Resonance Imaging
- Published
- 2010
- Full Text
- View/download PDF
8. Hypertrophic cardiomyopathy phenotype revisited after 50 years with cardiovascular magnetic resonance.
- Author
-
Maron MS, Maron BJ, Harrigan C, Buros J, Gibson CM, Olivotto I, Biller L, Lesser JR, Udelson JE, Manning WJ, and Appelbaum E
- Subjects
- Adolescent, Adult, Aged, Aged, 80 and over, Cardiomyopathy, Hypertrophic diagnostic imaging, Cardiomyopathy, Hypertrophic genetics, Child, Echocardiography, Female, Fibrosis, Gadolinium, Humans, Hypertrophy, Left Ventricular diagnostic imaging, Hypertrophy, Left Ventricular genetics, Male, Middle Aged, Phenotype, Prospective Studies, Radionuclide Imaging, Time Factors, Ventricular Outflow Obstruction diagnosis, Ventricular Outflow Obstruction genetics, Young Adult, Cardiomyopathy, Hypertrophic diagnosis, Hypertrophy, Left Ventricular diagnosis, Magnetic Resonance Imaging
- Abstract
Objectives: Our purpose was to characterize the pattern and distribution of left ventricular (LV) hypertrophy by cardiovascular magnetic resonance (CMR) to more precisely define phenotypic expression and its clinical implications in hypertrophic cardiomyopathy (HCM)., Background: Based on prior pathologic and 2-dimensional echocardiographic studies, HCM has been regarded as a disease characterized by substantial LV wall thickening., Methods: Cine and late gadolinium enhancement CMR were performed in 333 consecutive HCM patients (age 43 +/- 17 years)., Results: Basal anterior LV free wall and the contiguous anterior ventricular septum were the most commonly hypertrophied segments (n = 256; 77%). LV hypertrophy was focal (involving < or = 2 segments [< or = 12% of LV]) in 41 patients (12%), intermediate (3 to 7 segments [13% to 49% of LV]) in 112 patients (34%), and diffuse (> or = 8 segments [> or = 50% of LV]) in 180 patients (54%); 42 patients (13%) showed hypertrophied segments separated by regions of normal thickness. The number of hypertrophied segments was greater in patients with LV outflow tract obstruction (> or = 30 mm Hg) than without (10 +/- 4 vs. 8 +/- 4 per patient; p = 0.0001) and was associated with an advanced New York Heart Association functional class (p = 0.007). LV wall thickness was greater in segments with late gadolinium enhancement than without (20 +/- 6 mm vs. 16 +/- 6 mm; p < 0.001). We also identified 40 (12%) of HCM patients with segmental LV hypertrophy largely confined to the anterolateral free wall, posterior septum, or apex, which was underestimated or undetected by echocardiography., Conclusions: Although diverse, patterns of LV hypertrophy are usually not extensive in HCM, involving < or = 50% of the chamber in about one-half the patients, and are particularly limited in extent in an important minority. Contiguous portions of anterior free wall and septum constituted the predominant region of wall thickening, with implications for clinical diagnosis. These observations support an emerging role for CMR in the contemporary evaluation of patients with HCM.
- Published
- 2009
- Full Text
- View/download PDF
9. Clinical profile and significance of delayed enhancement in hypertrophic cardiomyopathy.
- Author
-
Maron MS, Appelbaum E, Harrigan CJ, Buros J, Gibson CM, Hanna C, Lesser JR, Udelson JE, Manning WJ, and Maron BJ
- Subjects
- Adult, Cardiomyopathy, Hypertrophic complications, Cardiomyopathy, Hypertrophic physiopathology, Diagnosis, Differential, Disease Progression, Endomyocardial Fibrosis complications, Endomyocardial Fibrosis physiopathology, Female, Follow-Up Studies, Heart Failure etiology, Heart Failure physiopathology, Humans, Male, Prognosis, Prospective Studies, Risk Factors, Stroke Volume physiology, Ventricular Function, Left physiology, Cardiomyopathy, Hypertrophic diagnosis, Endomyocardial Fibrosis diagnosis, Heart Failure diagnosis, Magnetic Resonance Imaging methods
- Abstract
Background: Contrast-enhanced cardiovascular magnetic resonance with delayed enhancement (DE) can provide in vivo assessment of myocardial fibrosis. However, the clinical significance of DE in hypertrophic cardiomyopathy (HCM) remains unresolved., Methods and Results: Cine and cardiovascular magnetic resonance with DE were performed in 202 HCM patients (mean age, 42+/-17 years; 71% male), DE was compared with clinical and demographic variables, and patients were followed up for 681+/-249 days for adverse disease events. DE was identified in 111 (55%) HCM patients, occupying 9%+/-11% of left ventricular myocardial volume, including >25% DE in 10% of patients. The presence of DE was related to occurrence of heart failure symptoms (P=0.05) and left ventricular systolic dysfunction (P=0.001). DE was present in all patients with ejection fraction < or =50% but also in 53% (102/192) of patients with preserved ejection fraction (P<0.001); %DE was both inversely related to (r=-0.3; P<0.001) and an independent predictor of ejection fraction (r=-0.4; P<0.001). DE (7%+/-7% of left ventricle) was present in 54 patients who were asymptomatic (and with normal ejection fraction). Over the follow-up period, the annualized adverse cardiovascular event rate in patients with DE exceeded that in patients without DE but did not achieve statistical significance (5.5% versus 3.3%; P=0.5)., Conclusions: In a large HCM cohort, DE was an independent predictor of systolic dysfunction but with only a modest relationship to heart failure symptoms. These data suggest an important role for myocardial fibrosis in the clinical course of HCM patients but are not sufficient at this time to consider DE as an independent risk factor for adverse prognosis.
- Published
- 2008
- Full Text
- View/download PDF
10. Assessment and significance of left ventricular mass by cardiovascular magnetic resonance in hypertrophic cardiomyopathy.
- Author
-
Olivotto I, Maron MS, Autore C, Lesser JR, Rega L, Casolo G, De Santis M, Quarta G, Nistri S, Cecchi F, Salton CJ, Udelson JE, Manning WJ, and Maron BJ
- Subjects
- Adult, Cardiomyopathy, Hypertrophic diagnostic imaging, Cardiomyopathy, Hypertrophic physiopathology, Case-Control Studies, Female, Humans, Hypertrophy, Left Ventricular diagnostic imaging, Hypertrophy, Left Ventricular physiopathology, Male, Phenotype, Risk Assessment, Stroke Volume, Ultrasonography, Cardiomyopathy, Hypertrophic diagnosis, Hypertrophy, Left Ventricular diagnosis, Magnetic Resonance Imaging
- Abstract
Objectives: Our aim was to assess the distribution and clinical significance of left ventricular (LV) mass in patients with hypertrophic cardiomyopathy (HCM)., Background: Hypertrophic cardiomyopathy is defined echocardiographically by unexplained left ventricular wall thickening. Left ventricular mass, quantifiable by modern cardiovascular magnetic resonance techniques, has not been systematically assessed in this disease., Methods: In 264 HCM patients (age 43 +/- 18 years; 75% men), LV mass by cardiovascular magnetic resonance was measured, indexed by body surface area, and compared with that in 606 healthy control subjects., Results: The LV mass index in HCM patients significantly exceeded that of control subjects (104 +/- 40 g/m(2) vs. 61 +/- 10 g/m(2) in men and 89 +/- 33 g/m(2) vs. 47 +/- 7 g/m(2) in women; both p < 0.0001). However, values were within the normal range (< or = mean +2 SDs for control subjects) in 56 patients (21%), and only mildly increased (mean +2 to 3 SDs) in 18 (16%). The LV mass index showed a modest relationship to maximal LV thickness (r(2) = 0.38; p < 0.001), and was greater in men (104 +/- 40 g/m(2) vs. 89 +/- 33 g/m(2) in women; p < 0.001) and in patients with resting outflow obstruction (121 +/- 43 g/m(2) vs. 96 +/- 37 g/m(2) in nonobstructives; p < 0.001). During a 2.6 +/- 0.7-year follow-up, markedly increased LV mass index proved more sensitive in predicting outcome (100%, with 39% specificity), whereas maximal wall thickness >30 mm was more specific (90%, with 41% sensitivity)., Conclusions: In distinction to prior perceptions, LV mass index was normal in about 20% of patients with definite HCM phenotype. Therefore, increased LV mass is not a requirement for establishing the clinical diagnosis of HCM. The LV mass correlated weakly with maximal wall thickness, and proved more sensitive in predicting outcome.
- Published
- 2008
- Full Text
- View/download PDF
11. Occurrence and frequency of arrhythmias in hypertrophic cardiomyopathy in relation to delayed enhancement on cardiovascular magnetic resonance.
- Author
-
Adabag AS, Maron BJ, Appelbaum E, Harrigan CJ, Buros JL, Gibson CM, Lesser JR, Hanna CA, Udelson JE, Manning WJ, and Maron MS
- Subjects
- Adolescent, Adult, Aged, Blood Pressure Monitoring, Ambulatory, Cardiomyopathy, Hypertrophic pathology, Cardiomyopathy, Hypertrophic physiopathology, Female, Fibrosis complications, Fibrosis physiopathology, Health Status Indicators, Humans, Male, Middle Aged, Prevalence, Prospective Studies, Risk Assessment, Tachycardia pathology, Time Factors, Ventricular Premature Complexes, Cardiomyopathy, Hypertrophic complications, Magnetic Resonance Imaging, Tachycardia epidemiology, Tachycardia etiology
- Abstract
Objectives: Our aim was to determine whether myocardial fibrosis, detected by cardiovascular magnetic resonance (CMR), represents an arrhythmogenic substrate in hypertrophic cardiomyopathy (HCM)., Background: Myocardial fibrosis is identified frequently in HCM; however, the clinical significance of this finding is uncertain., Methods: We studied prevalence and frequency of tachyarrhythmias on 24-h ambulatory Holter electrocardiogram (ECG) with regard to delayed enhancement (DE) on contrast-enhanced CMR in 177 HCM patients (age 41 +/- 16 yrs; 95% asymptomatic or mildly symptomatic)., Results: Premature ventricular contractions (PVCs), couplets, and nonsustained ventricular tachycardia (NSVT) were more common in patients with DE than those without DE (PVCs: 89% vs. 72%; couplets: 40% vs. 17%; NSVT: 28% vs. 4%; p < 0.0001 to 0.007). Patients with DE also had greater numbers of PVCs (202 +/- 655 vs. 116 +/- 435), couplets (1.9 +/- 5 vs. 1.2 +/- 10), and NSVT runs (0.4 +/- 0.8 vs. 0.06 +/- 0.4) than non-DE patients (all p < 0.0001); DE was an independent predictor of NSVT (relative risk 7.3, 95% confidence interval 2.6 to 20.4; p < 0.0001). However, extent (%) of DE was similar in patients with and without PVCs (8.2% vs. 9.1%; p = 0.93), couplets (8.5% vs. 8.4%; p = 0.99), or NSVT (8.3% vs. 8.5%; p = 0.35)., Conclusions: In this large HCM cohort with no or only mild symptoms, myocardial fibrosis detected by CMR was associated with greater likelihood and increased frequency of ventricular tachyarrhythmias (including NSVT) on ambulatory Holter ECG. Therefore, contrast-enhanced CMR identifies HCM patients with increased susceptibility to ventricular tachyarrhythmias.
- Published
- 2008
- Full Text
- View/download PDF
12. ACCF/AHA 2007 clinical competence statement on vascular imaging with computed tomography and magnetic resonance. A report of the American College of Cardiology Foundation/American Heart Association/American College of Physicians Task Force on Clinical Competence and Training.
- Author
-
Kramer CM, Budoff MJ, Fayad ZA, Ferrari VA, Goldman C, Lesser JR, Martin ET, Rajagopalan S, Reilly JP, Rodgers GP, Wechsler L, Creager MA, Holmes DR Jr, Merli G, Newby LK, Piña I, Rodgers GP, and Weitz HH
- Subjects
- Humans, Cardiovascular Diseases diagnosis, Clinical Competence standards, Education, Medical standards, Magnetic Resonance Imaging, Tomography, X-Ray Computed
- Published
- 2007
- Full Text
- View/download PDF
13. Images in cardiovascular medicine. Diagnostic utility of cardiac magnetic resonance imaging in monozygotic twins with hypertrophic cardiomyopathy and identical pattern of left ventricular hypertrophy.
- Author
-
Maron BJ, Haas TS, and Lesser JR
- Subjects
- Adolescent, Electrocardiography, Humans, Myocardium pathology, Cardiomyopathy, Hypertrophic genetics, Cardiomyopathy, Hypertrophic pathology, Hypertrophy, Left Ventricular genetics, Hypertrophy, Left Ventricular pathology, Magnetic Resonance Imaging, Twins, Monozygotic
- Published
- 2007
- Full Text
- View/download PDF
14. Cardiac magnetic resonance imaging for detection of an abscess associated with prosthetic valve endocarditis: a case report.
- Author
-
Harris KM, Ang E, Lesser JR, and Sonnesyn SW
- Subjects
- Abscess etiology, Endocarditis diagnosis, Gram-Positive Bacterial Infections complications, Humans, Male, Middle Aged, Prosthesis-Related Infections complications, Abscess diagnosis, Endocarditis etiology, Gram-Positive Bacterial Infections diagnosis, Heart Valve Prosthesis adverse effects, Magnetic Resonance Imaging methods, Propionibacterium acnes, Prosthesis-Related Infections diagnosis
- Abstract
Propionibacterium acnes is an organism frequently isolated in cultures and often dismissed as a contaminant. A patient with a febrile illness and prosthetic aortic valve was suspected of having infectious endocarditis. Magnetic resonance imaging was useful in defining a paravalvular abscess associated with prosthetic valve endocarditis due to Propionibacterium acnes that was then successfully surgically repaired.
- Published
- 2007
- Full Text
- View/download PDF
15. ACCF/ACR/SCCT/SCMR/ASNC/NASCI/SCAI/SIR 2006 appropriateness criteria for cardiac computed tomography and cardiac magnetic resonance imaging: a report of the American College of Cardiology Foundation Quality Strategic Directions Committee Appropriateness Criteria Working Group, American College of Radiology, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, American Society of Nuclear Cardiology, North American Society for Cardiac Imaging, Society for Cardiovascular Angiography and Interventions, and Society of Interventional Radiology.
- Author
-
Hendel RC, Patel MR, Kramer CM, Poon M, Hendel RC, Carr JC, Gerstad NA, Gillam LD, Hodgson JM, Kim RJ, Kramer CM, Lesser JR, Martin ET, Messer JV, Redberg RF, Rubin GD, Rumsfeld JS, Taylor AJ, Weigold WG, Woodard PK, Brindis RG, Hendel RC, Douglas PS, Peterson ED, Wolk MJ, Allen JM, and Patel MR
- Subjects
- Decision Making, Delphi Technique, Humans, Risk Assessment, Cardiovascular Diseases diagnostic imaging, Cardiovascular Diseases pathology, Magnetic Resonance Imaging adverse effects, Tomography, X-Ray adverse effects
- Published
- 2006
- Full Text
- View/download PDF
16. Lack of Phenotypic Differences by Cardiovascular Magnetic Resonance Imaging in MYH7 (β-Myosin Heavy Chain)-Versus MYBPC3 (Myosin-Binding Protein C)-Related Hypertrophic Cardiomyopathy.
- Author
-
Weissler-Snir, Adaya, Hindieh, Waseem, Gruner, Christiane, Fourey, Dana, Appelbaum, Evan, Rowin, Ethan, Care, Melanie, Lesser, John R., Haas, Tammy S., Udelson, James E., Manning, Warren J., Olivotto, Iacopo, Tomberli, Benedetta, Maron, Barry J., Maron, Martin S., Crean, Andrew M., Rakowski, Harry, and Chan, Raymond H.
- Abstract
Background--The 2 most commonly affected genes in hypertrophic cardiomyopathy (HCM) are MYH7 (β-myosin heavy chain) and MYBPC3 (β-myosin-binding protein C). Phenotypic differences between patients with mutations in these 2 genes have been inconsistent. Scarce data exist on the genotype-phenotype association as assessed by tomographic imaging using cardiac magnetic resonance imaging. Methods and Results--Cardiac magnetic resonance imaging was performed on 358 consecutive genotyped hypertrophic cardiomyopathy probands at 5 tertiary hypertrophic cardiomyopathy centers. Genetic testing revealed a pathogenic mutation in 159 patients (44.4%). The most common genes identified were MYH7 (n=53) and MYBPC3 (n=75); 33.1% and 47% of genopositive patients, respectively. Phenotypic characteristics by cardiac magnetic resonance imaging of these 2 groups were similar, including left ventricular volumes, mass, maximal wall thickness, morphology, left atrial volume, and mitral valve leaflet lengths (all P=non-significant). The presence of late gadolinium enhancement (65% versus 64%; P=0.99) and the proportion of total left ventricular mass (%late gadolinium enhancement; 10.4±13.2% versus 8.5±8.5%; P=0.44) were also similar. Conclusions--This multicenter multinational study shows lack of phenotypic differences between MYH7- and MYBPC3- associated hypertrophic cardiomyopathy when assessed by cardiac magnetic resonance imaging. Postmutational mechanisms appear more relevant to thick-filament disease expression and outcome than the disease-causing variant per se. [ABSTRACT FROM AUTHOR]
- Published
- 2017
- Full Text
- View/download PDF
17. Significance of Late Gadolinium Enhancement at Right Ventricular Attachment to Ventricular Septum in Patients With Hypertrophic Cardiomyopathy.
- Author
-
Chan, Raymond H., Maron, Barry J., Olivotto, Iacopo, Assenza, Gabriele E., Haas, Tammy S., Lesser, John R., Gruner, Christiane, Crean, Andrew M., Rakowski, Harry, Rowin, Ethan, Udelson, James, Lombardi, Massimo, Tomberli, Benedetta, Spirito, Paolo, Formisano, Francesco, Marra, Martina P., Biagini, Elena, Autore, Camillo, Manning, Warren J., and Appelbaum, Evan
- Subjects
- *
HYPERTROPHIC cardiomyopathy , *RIGHT heart ventricle , *HEART septum , *GADOLINIUM , *CARDIOVASCULAR system , *SUDDEN death , *PHYSIOLOGY , *PATIENTS , *MAGNETIC resonance imaging - Abstract
Cardiovascular magnetic resonance (CMR) with extensive late gadolinium enhancement (LGE) is a novel marker for increased risk for sudden death (SD) in patients with hypertrophic cardiomyopathy (HC). Small focal areas of LGE confined to the region of right ventricular (RV) insertion to ventricular septum (VS) have emerged as a frequent and highly visible CMR imaging pattern of uncertain significance. The aim of this study was to evaluate the prognostic significance of LGE confined to the RV insertion area in patients with HC. CMR was performed in 1,293 consecutive patients with HC from 7 HC centers, followed for 3.4 ± 1.7 years. Of 1,293 patients (47 ± 14 years), 134 (10%) had LGE present only in the anterior and/or inferior areas of the RV insertion to VS, occupying 3.7 ± 2.9% of left ventricular myocardium. Neither the presence nor extent of LGE in these isolated areas was a predictor of adverse HC-related risk, including SD (adjusted hazard ratio 0.82, 95% confidence interval 0.45 to 1.50, p = 0.53; adjusted hazard ratio 1.16/10% increase in LGE, 95% confidence interval 0.29 to 4.65, p = 0.83, respectively). Histopathology in 20 HC hearts show the insertion areas of RV attachment to be composed of a greatly expanded extracellular space characterized predominantly by interstitial-type fibrosis and interspersed disorganized myocyte patterns and architecture. In conclusion, LGE confined to the insertion areas of RV to VS was associated with low risk of adverse events (including SD). Gadolinium pooling in this region of the left ventricle does not reflect myocyte death and repair with replacement fibrosis or scarring. [ABSTRACT FROM AUTHOR]
- Published
- 2015
- Full Text
- View/download PDF
18. Prognostic Value of Quantitative Contrast-Enhanced Cardiovascular Magnetic Resonance for the Evaluation of Sudden Death Risk in Patients With Hypertrophic Cardiomyopathy.
- Author
-
Chan, Raymond H., Maron, Barry J., Olivotto, Iacopo, Pencina, Michael J., Assenza, Gabriele Egidy, Haas, Tammy, Lesser, John R., Gruner, Christiane, Crean, Andrew M., Rakowski, Harry, Udelson, James E., Rowin, Ethan, Lombardi, Massimo, Cecchi, Franco, Tomberli, Benedetta, Spirito, Paolo, Formisano, Francesco, Biagini, Elena, Rapezzi, Claudio, and De Cecco, Carlo Nicola
- Subjects
- *
HYPERTROPHIC cardiomyopathy , *HEART disease related mortality , *CONTRAST-enhanced magnetic resonance imaging , *CARDIAC magnetic resonance imaging , *SUDDEN death , *PROGNOSIS ,RISK factors - Abstract
Background--Hypertrophic cardiomyopathy (HCM) is the most common cause of sudden death in the young, although not all patients eligible for sudden death prevention with an implantable cardioverter-defibrillator are identified. Contrast-enhanced cardiovascular magnetic resonance with late gadolinium enhancement (LGE) has emerged as an in vivo marker of myocardial fibrosis, although its role in stratifying sudden death risk in subgroups of HCM patients remains incompletely understood. Methods and Results--We assessed the relation between LGE and cardiovascular outcomes in 1293 HCM patients referred for cardiovascular magnetic resonance and followed up for a median of 3.3 years. Sudden cardiac death (SCD) events (including appropriate defibrillator interventions) occurred in 37 patients (3%). A continuous relationship was evident between LGE by percent left ventricular mass and SCD event risk in HCM patients (P=0.001). Extent of LGE was associated with an increased risk of SCD events (adjusted hazard ratio, 1.46/10% increase in LGE; P=0.002), even after adjustment for other relevant disease variables. LGE of ≥15% of LV mass demonstrated a 2-fold increase in SCD event risk in those patients otherwise considered to be at lower risk, with an estimated likelihood for SCD events of 6% at 5 years. Performance of the SCD event risk model was enhanced by LGE (net reclassification index, 12.9%; 95% confidence interval, 0.3-38.3). Absence of LGE was associated with lower risk for SCD events (adjusted hazard ratio, 0.39; P=0.02). Extent of LGE also predicted the development of end-stage HCM with systolic dysfunction (adjusted hazard ratio, 1.80/10% increase in LGE; P<0.03). Conclusions--Extensive LGE measured by quantitative contrast enhanced CMR provides additional information for assessing SCD event risk among HCM patients, particularly patients otherwise judged to be at low risk. [ABSTRACT FROM AUTHOR]
- Published
- 2014
- Full Text
- View/download PDF
19. Left Atrial Remodeling in Hypertrophic Cardiomyopathy and Susceptibility Markers for Atrial Fibrillation Identified by Cardiovascular Magnetic Resonance.
- Author
-
Maron, Barry J., Haas, Tammy S., Maron, Martin S., Lesser, John R., Browning, Joseph A., Chan, Raymond H., Olivotto, Iacopo, Garberich, Ross F., and Schwartz, Robert S.
- Subjects
- *
HYPERTROPHIC cardiomyopathy , *ATRIAL fibrillation , *HEART failure , *STROKE patients , *MAGNETIC resonance imaging , *MYOCARDIAL infarction complications , *THERAPEUTICS - Abstract
In hypertrophic cardiomyopathy (HC), atrial fibrillation (AF) is an important determinant of clinical deterioration due to heart failure or embolic stroke. This study characterizes left atrial (LA) structural and functional parameters to establish markers predictive of AF risk, using cardiovascular magnetic resonance (CMR) imaging. We studied 427 consecutive patients with HC in sinus rhythm with CMR (age 44 ± 18 years), including 41 who developed clinically overt AF after study entry (2.6 ± 2.1 years), 49 patients with AF before CMR, 337 patients with HC but without AF, and 244 normal controls. LA chamber was assessed for absolute and indexed end-diastolic volume (LAEDV), end-systolic volume, and percent ejection fraction (LAEF). In the 41 prospectively studied patients with HC who developed AF during follow-up, LAEDV was significantly greater than in patients without AF (146 ± 48 vs 107 ± 37 ml) or in normal controls (81 ± 24 ml, p <0.001). Percent LAEF was lower in patients developing AF (36 ± 10%) than without AF (46 ± 12%) or controls (55 ± 9%, p <0.001). Multivariate analysis identified LAEF (<38%), LAEDV (≥118 ml), and age (≥40 years) as independently associated with AF occurrence. In conclusion, CMR measures of LA remodeling and dysfunction reliably identified patients with HC at risk for future development of AF. Decrease in LAEF represents a strong novel marker of susceptibility to AF in this disease. [ABSTRACT FROM AUTHOR]
- Published
- 2014
- Full Text
- View/download PDF
20. Obesity and its Association to Phenotype and Clinical Course in Hypertrophic Cardiomyopathy.
- Author
-
Olivotto, Iacopo, Maron, Barry J., Tomberli, Benedetta, Appelbaum, Evan, Salton, Carol, Haas, Tammy S., Gibson, C. Michael, Nistri, Stefano, Servettini, Eleonora, Chan, Raymond H., Udelson, James E., Lesser, John R., Cecchi, Franco, Manning, Warren J., and Maron, Martin S.
- Subjects
- *
OBESITY , *PHENOTYPES , *HYPERTROPHIC cardiomyopathy , *BODY mass index , *COHORT analysis , *MULTIVARIATE analysis , *MAGNETIC resonance imaging , *PATIENTS - Abstract
Objectives: This study sought to assess the impact of body mass index (BMI) on cardiac phenotypic and clinical course in a multicenter hypertrophic cardiomyopathy (HCM) cohort. Background: It is unresolved whether clinical variables promoting left ventricular (LV) hypertrophy in the general population, such as obesity, may influence cardiac phenotypic and clinical course in patients with HCM. Methods: In 275 adult HCM patients (age 48 ± 14 years; 70% male), we assessed the relation of BMI to LV mass, determined by cardiovascular magnetic resonance (CMR) and heart failure progression. Results: At multivariate analysis, BMI proved independently associated with the magnitude of hypertrophy: pre-obese and obese HCM patients (BMI 25 to 30 kg/m2 and >30 kg/m2, respectively) showed a 65% and 310% increased likelihood of an LV mass in the highest quartile (>120 g/m2), compared with normal weight patients (BMI <25 kg/m2; hazard ratio [HR]: 1.65; 95% confidence interval [CI]: 0.73 to 3.74, p = 0.22 and 3.1; 95% CI: 1.42 to 6.86, p = 0.004, respectively). Other features associated with LV mass >120 g/m2 were LV outflow obstruction (HR: 4.9; 95% CI: 2.4 to 9.8; p < 0.001), systemic hypertension (HR: 2.2; 95% CI: 1.1 to 4.5; p = 0.026), and male sex (HR: 2.1; 95% CI: 0.9 to 4.7; p = 0.083). During a median follow-up of 3.7 years (interquartile range: 2.5 to 5.3), obese patients showed an HR of 3.6 (95% CI: 1.2 to 10.7, p = 0.02) for developing New York Heart Association (NYHA) functional class III to IV symptoms compared to nonobese patients, independent of outflow obstruction. Noticeably, the proportion of patients in NYHA functional class III at the end of follow-up was 13% among obese patients, compared with 6% among those of normal weight (p = 0.03). Conclusions: In HCM patients, extrinsic factors such as obesity are independently associated with increase in LV mass and may dictate progression of heart failure symptoms. [Copyright &y& Elsevier]
- Published
- 2013
- Full Text
- View/download PDF
21. Risk Stratification and Outcome of Patients With Hypertrophic Cardiomyopathy ≥60 Years of Age.
- Author
-
Maron, Barry J., Rowin, Ethan J., Casey, Susan A., Haas, Tammy S., Chan, Raymond H.M., Udelson, James E., Garberich, Ross F., Lesser, John R., Appelbaum, Evan, Manning, Warren J., and Maron, Martin S.
- Subjects
- *
HYPERTROPHIC cardiomyopathy , *ARTERIOGRAPHY , *ANGIOGRAPHY , *KAPLAN-Meier estimator , *DISEASE risk factors , *MORTALITY - Abstract
The article presents a study on the risk stratification and outcome of hypertrophic cardiomyopathy (HCM) patients ages over or equal to 60. The study employs coronary arteriograms, computed tomographic angiography, and Kaplan-Meier method. Results reveal that HMC patients are at low risk for disease-related mortality/morbidity even with conventional risk factors.
- Published
- 2013
- Full Text
- View/download PDF
22. Spectrum and Clinical Significance of Systolic Function and Myocardial Fibrosis Assessed by Cardiovascular Magnetic Resonance in Hypertrophic Cardiomyopathy
- Author
-
Olivotto, Iacopo, Maron, Barry J., Appelbaum, Evan, Harrigan, Caitlin J., Salton, Carol, Gibson, C. Michael, Udelson, James E., O'Donnell, Christopher, Lesser, John R., Manning, Warren J., and Maron, Martin S.
- Subjects
- *
HYPERTROPHIC cardiomyopathy , *REGULATION of heart contraction , *CARDIOVASCULAR diseases , *LEFT heart ventricle , *VENTRICULAR remodeling , *HEART fibrosis , *DIAGNOSIS , *MAGNETIC resonance imaging - Abstract
In hypertrophic cardiomyopathy (HCM), the clinical significance attributable to the broad range of left ventricular (LV) systolic function, assessed as the ejection fraction (EF), is incompletely resolved. We evaluated the EF using cardiovascular magnetic resonance (CMR) imaging in a large cohort of patients with HCM with respect to the clinical status and evidence of left ventricular remodeling with late gadolinium enhancement (LGE). CMR imaging was performed in 310 consecutive patients, aged 42 ± 17 years. The EF in patients with HCM was 71 ± 10% (range 28% to 89%), exceeding that of 606 healthy controls without cardiovascular disease (66 ± 5%, p <0.001). LGE reflecting LV remodeling showed an independent, inverse relation to the EF (B-0.69, 95% confidence interval −0.86 to −0.52; p <0.001) and was greatest in patients with an EF <50%, in whom it constituted a median value of 29% of the LV volume (interquartile range 16% to 40%). However, the substantial subgroup with low-normal EF values of 50% to 65% (n = 45; 15% of the whole cohort), who were mostly asymptomatic or mildly symptomatic (37 or 82% with New York Heart Association functional class I to II), showed substantial LGE (median 5% of LV volume, interquartile range 2% to 10%). This overlapped with the subgroup with systolic dysfunction and significantly exceeded that of patients with an EF of 66% to 75% and >75% (median 2% of the LV volume, interquartile range 1.5% to 4%; p <0.01). In conclusion, in a large cohort of patients with HCM, a subset of patients with low-normal EF values (50% to 65%) was identified by contrast-enhanced CMR imaging as having substantial degrees of LGE, suggesting a transition phase, potentially heralding advanced LV remodeling and systolic dysfunction, with implications for clinical surveillance and management. [Copyright &y& Elsevier]
- Published
- 2010
- Full Text
- View/download PDF
23. Significance of Papillary Muscle Abnormalities Identified by Cardiovascular Magnetic Resonance in Hypertrophic Cardiomyopathy
- Author
-
Harrigan, Caitlin J., Appelbaum, Evan, Maron, Barry J., Buros, Jacqueline L., Gibson, C. Michael, Lesser, John R., Udelson, James E., Manning, Warren J., and Maron, Martin S.
- Subjects
- *
CARDIOMYOPATHIES , *HYPERTROPHIC cardiomyopathy , *MAGNETIC resonance imaging , *PHENOTYPES - Abstract
Increased thickness of the left ventricular (LV) wall is the predominant feature of the hypertrophic cardiomyopathy (HC) phenotype. The structural characteristics of the LV papillary muscles (PMs) have received little attention. In this study, cardiovascular magnetic resonance (CMR) was used to characterize PM morphology in a large HC population. Cine and delayed enhancement (DE) CMR images were obtained in 201 patients with HC and 43 control subjects. PM number and mass index were greater in patients with HC compared with controls (2.5 vs 2.1, p <0.001, and 6 ± 2 vs 3 ± 2 g/m2, p <0.001, respectively), including 109 (54%) with PM mass ≥7 g/m2 (≥2 SDs above the mean for controls). Greater LV wall mass index was associated with more substantial PM mass (r = 0.09, p <0.001). Furthermore, 12 patients with HC (19%) had normal LV mass with localized wall thickness but increased PM mass. In patients with HC with LV outflow obstruction at rest, PMs were positioned closer to the ventricular septum (displaced anteriorly: 58% vs 42% for subjects without obstruction, p = 0.02), with more marked hypertrophy (9 ± 5 vs 6 ± 4 g/m2, p <0.001). Preoperative CMR identified 3 patients with accessory, anteriorly displaced PMs judged to contribute to outflow obstruction, which were resected during septal myectomy. DE of the PMs was identified in 13 patients with HC (6%), including 3 with DE confined to PMs. In conclusion, CMR demonstrates LV PMs to be part of the cardiomyopathic process in HC, with increases in number and mass, and not uncommonly associated with remodeling with DE. The identification of accessory PMs may be useful in planning preoperative strategy. [Copyright &y& Elsevier]
- Published
- 2008
- Full Text
- View/download PDF
Catalog
Discovery Service for Jio Institute Digital Library
For full access to our library's resources, please sign in.