Hypertrophic Cardiomyopathy

Wigle, E. Douglas; Rakowski, Harry; Bp, Kimball; Wg, Williams

doi:10.1161/01.cir.92.7.1680

Cited by 689 publications

(218 citation statements)

References 171 publications

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“…HCM was diagnosed by established clinical, hemodynamic, and echocardiographic criteria. 13 No significant intraventricular pressure gradient was detected at rest or during provocative maneuvers (the Valsalva maneuver and isoproterenol infusion) after completion of the study protocol in any patient with HCM. The control group consisted of 7 patients undergoing diagnostic cardiac catheterization to evaluate atypical chest pain.…”

Section: Patientsmentioning

confidence: 80%

“…cDNA was synthesized from 300 ng of total RNA and human heart total RNA (Clontech) at 42°C for 50 minutes with oligo(dT) [12][13][14][15][16][17][18] and Moloney murine leukemia virus RT (SuperScriptII, Gibco BRL). The DNAse I treatment was carried out in the same reaction mixture as the RT before addition of reverse transcriptase to remove contaminating genomic DNA, as described by Dilworth and McCarrey.…”

Section: Taqman Real-time Quantitative Rt-pcr Analysismentioning

confidence: 99%

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Reduced Myocardial Sarcoplasmic Reticulum Ca ²⁺ -ATPase mRNA Expression and Biphasic Force-Frequency Relations in Patients With Hypertrophic Cardiomyopathy

et al. 2001

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Background-The relationship between left ventricular (LV) contractile functional reserve and gene expression ofCa 2ϩ -handling proteins in patients with hypertrophic cardiomyopathy (HCM) remains to be clarified. Methods and Results-We calculated the maximum first derivative of LV pressure (LV dP/dt max ) and the LV pressure half-time (T 1/2 ) during pacing in 14 patients with nonobstructive HCM (LV ejection fraction Ͼ55%) and 7 control subjects. Endomyocardial tissue was obtained, and mRNA levels of sarcoplasmic reticulum Ca 2ϩ -ATPase (SERCA2), ryanodine receptor-2, phospholamban, calsequestrin, and Na ϩ /Ca 2ϩ exchanger were quantified by use of a real-time quantitative reverse transcription-polymerase chain reaction method. Group A consisted of 7 HCM patients who showed a progressive rise in the LV dP/dt max with increased heart rate. Group B consisted of 7 HCM patients in whom the heart rate-LV dP/dt max relation was biphasic at physiological pacing rates. Both the mean maximal wall thickness and the LV hypertrophy score in group B were greater than in group A (20Ϯ5 versus 15Ϯ3 mm and 7Ϯ1 versus 5Ϯ2 points, respectively). SERCA2 mRNA levels were significantly lower in group B (SERCA2/GAPDH ratio 0.34Ϯ0.15) compared with group A (0.72Ϯ0.27) and control subjects (0.85Ϯ0.47), whereas the mRNA expression of ryanodine receptor-2, phospholamban, calsequestrin, and Na ϩ /Ca 2ϩ exchanger were similar in all groups. Conclusions-These results suggest that downregulation of SERCA2 mRNA, resulting in altered Ca 2ϩ handling, may contribute to impaired LV contractile reserve in HCM patients with severe hypertrophy, even in the absence of detectable baseline systolic dysfunction. (Circulation. 2001;104:658-663.)

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Section: Patientsmentioning

confidence: 80%

Section: Taqman Real-time Quantitative Rt-pcr Analysismentioning

confidence: 99%

Reduced Myocardial Sarcoplasmic Reticulum Ca ²⁺ -ATPase mRNA Expression and Biphasic Force-Frequency Relations in Patients With Hypertrophic Cardiomyopathy

et al. 2001

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“…The clinical course of FHC is highly variable, with a spectrum at presentation that ranges from the asymptomatic carrier state to premature sudden death in childhood. The risk factors for sudden death in FHC include young age at presentation, syncope, myocardial ischemia, inducible sustained ventricular tachycardia during EP study, and a malignant family history (21)(22)(23)(24). In FHC patients who have survived cardiac arrest, electrophysiologic testing has demonstrated that sinus node dysfunction, electrogram fractionation, and inducible ventricular arrhythmias are common (16,25,26).…”

Section: Discussionmentioning

confidence: 99%

Electrophysiological abnormalities and arrhythmias in alpha MHC mutant familial hypertrophic cardiomyopathy mice.

Berul

Christe²,

Aronovitz³

et al. 1997

J. Clin. Invest.

101

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A new mouse cardiac electrophysiology method was used to study mice harboring an ␣ -myosin heavy chain Arg403Gln missense mutation ( ␣ -MHC 403/ ϩ ), which results in histological and hemodynamic abnormalities characteristic of familial hypertrophic cardiomyopathy (FHC) and sudden death of uncertain etiology during exercise. Wild-type animals had completely normal cardiac electrophysiology. In contrast, FHC mice demonstrated ( a ) electrocardiographic abnormalities including prolonged repolarization intervals and rightward axis; ( b ) electrophysiological abnormalities including heterogeneous ventricular conduction properties and prolonged sinus node recovery time; and ( c ) inducible ventricular ectopy. These data identify distinct electrophysiologic abnormalities in FHC mice with a specific ␣ -myosin mutation, and also validate a novel method to explore in vivo the relationship between specific genotypes and their electrophysiologic phenotypes. ( J. Clin. Invest. 1997. 99: 570-576.)

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“…Management of HCM with midventricular obstruction is difficult and although ventricular septal myotomy -myectomy or dual-chamber (DDD) pacing is the treatment of choice for patients with severe outflow tract obstruction refractory to drug treatment, 5,22 its role in this subgroup of patients with HCM is not yet established. Without other predictors of outcome, except for an outflow gradient no less than 30 mmHg, 23,24 we began treatment with ablocker, although some HCM centers favor verapamil or class Ia antiarrhythmic agents.…”

Section: Discussionmentioning

confidence: 99%