Natural history of abnormal conduction and its relation to prognosis in patients with dilated cardiomyopathy

Xiao, Han; Roy, Craig; Fujimoto, Shinichi; Gibson, D G

doi:10.1016/0167-5273(95)02502-2

Cited by 237 publications

(158 citation statements)

References 14 publications

Supporting

Mentioning

142

Contrasting

Unclassified

Order By: Relevance

“…We encompassed these disease manifestations in diagnoses drawn from national registries and compared the incidences of myocarditis, heart failure/cardiomyopathy, tachyarrhythmia, cardiac arrest, syncope, and dyspnea/edema in PKP2 c.419C4T carriers and non-carriers without any significant association. In addition, as both ARVC and DCM patients often exhibit ECG abnormalities, 22,23 including arrhythmias, ventricular conduction abnormalities, and abnormal electrocardiographic intervals, we investigated the association of PKP2 c.419C4T genotype with these parameters, and again found no associations. These data were consistent with a lack of echocardiographic signs of left ventricular systolic dysfunction or chamber dilatation -findings that contribute to the definition of DCM.…”

Section: Discussionmentioning

confidence: 99%

Plakophilin-2 c.419C>T and risk of heart failure and arrhythmias in the general population

et al. 2015

View full text Add to dashboard Cite

A rare genetic variant in the desmosomal gene plakophilin-2 (PKP2) c.419C4T(p.(S140F)) has repeatedly been identified in patients with dilated cardiomyopathy (DCM) and arrhythmogenic right ventricular cardiomyopathy (ARVC). Whether this is a disease-causing variant remains highly controversial. We tested this hypothesis using three approaches. Initially, in a prospective study of 10 407 individuals from the general population, including 2688 who developed heart failure or arrhythmias during 414 years of follow-up, PKP2 c.419C4T was identified in 98 individuals (0.94%). PKP2 genotype was not associated with electrocardiographic or echocardiographic changes, or with plasma levels of probrain natriuretic peptide (all P ≥ 0.05). In c.419C4T carriers versus non-carriers, multifactorially adjusted hazard ratios were 1.26 (95% confidence interval: 0.77-2.07) for heart failure, 1.40 (0.90-2.17) for arrhythmias, 1.15 (0.78-1.71) for end points combined, and 1.33 (0.98-1.80) for allcause mortality. The cumulative survival as a function of age and PKP2 genotype was similar among carriers and non-carriers (P = 0.14). Second, comparing 517 patients referred for genetic testing with 1918 matched controls, odds ratios as a function of c.419C4T genotype were 2.11 (0.50-8.99) for ARVC, 0.72 (0.16-3.28) for hypertrophic cardiomyopathy (HCM)/DCM, and 1.28 (0.46-3.54) for end points combined. Third, in in vitro studies cellular localization of plakophilin-2, plakoglobin, connexin-43, or N-cadherin were similar in cells transfected with wild-type or mutant plakophilin-2. In conclusion, combining epidemiological data, with data on patients referred for genetic testing for ARVC or HCM/DCM, and data from in vitro studies, PKP2 c.419C4T did not associate with heart failure, arrhythmias, or premature death, with ARVC or HCM/DCM, or with effects in vitro, suggesting that this is not a disease-causing variant.

show abstract

Section: Discussionmentioning

confidence: 99%

Plakophilin-2 c.419C>T and risk of heart failure and arrhythmias in the general population

et al. 2015

View full text Add to dashboard Cite

show abstract

“…the paradox we have in this case is the progressive deterioration of electric cardiac function which ended with a picture of bilateral bundle branch block. these patients are known to have their ventricle activated solely via the Maheim fibres at the top of the interventricular septum 6 . with such degree of heart block, prolongation of pR interval, and delayed depolarisation, broad QRs duration, they become electrically and mechanically unstable and require permanent cardiac pacing for prognostic purposes, even if not for symptoms 7 .…”

Section: Discussionmentioning

confidence: 99%

Progressive conduction disease late after adriamycin treatment of lymphoma, despite normalised ejection fraction

Lysell-Bergström¹,

Johansson²,

Widman³

et al. 2015

ICFJ

View full text Add to dashboard Cite

Adriamycin (doxorubicin) chemotherapy is known for its acute and late cardiotoxic complications 1 . In up to 2% of patients they manifest in the form of suppressed ventricular systolic function with its well known consequences, raised filling pressures and mitral regurgitation. Its potential direct effect on conduction disturbances, however, has not been systematically described. we present a case of a patient who had severe heart failure following adriamycin lymphoma treatment who despite full recovery of systolic left ventricular (lV) function developed progressive conduction disease that required life saving cardiac pacing.A 56-year old male with known asthma presented with skin rash and was diagnosed with t-cell lymphoma. He was commenced on chemotherapy with CHoep (including doxorubicin 50 mg/ m 2 and 90 mg at a time) and by the end of the fourth cycle (four months of treatment) he was admitted with cardiogenic shock with raised inflammatory markers and liver enzymes. the patient received full heart failure treatment and antibiotics for secondary pneumonia. three weeks later the cardiopulmonary function was stabilized and he was moved to a rehabilitation unit. during admission, lV ejection fraction was less than 40%, which recovered over time and 3 years later increased to 70%. In contrast, during admission QRs duration was 118 ms with normal axis which progressively broadened, becoming 155 ms associated with first degree heart block (p-R interval >260ms) and right axis deviation, consistent with bilateral Bundle Branch Block (Fig 1). As a result of the progressive conduction disease, the patient developed significant lV asynchrony that compromised his total filling time to 150 ms (normal 400 ms). A pacemaker implantation was recommended for prognostic reasons.

show abstract

“…[17][18][19] In cardiomyopathy, the intrinsic QRS duration progressively increases over time, and both the initial QRS and extent of QRS prolongation are linked to patient mortality. [19][20][21] CRT can decrease the QRS duration by providing biventricular pacing and reducing left ventricular volume.…”

Section: Discussionmentioning

confidence: 99%

Effects of Cardiac Resynchronization Therapy on Ventricular Electrical Remodeling in Patients With Heart Failure

et al. 2015

View full text Add to dashboard Cite

SummaryCardiac resynchronization therapy (CRT) reverses structural remodeling of the left ventricle. We investigated whether CRT reverses left-ventricular electrical remodeling.Eighty patients were enrolled and implanted with CRT-devices. Echocardiography and electrocardiography data were obtained from each patient prior to implantation and two years after implantation. At two years after implantation, the patients were classified into a responder group and a non-responder group based on echocardiography.Over the next 2 years, 75 patients completed follow-up, and 5 patients had died. Echocardiography results showed that 23 patients could be classified as non-responders and 52 as responders. Larger numbers of non-responders were diagnosed with either ischemic cardiomyopathy (ICM) or nonspecific intraventricular conduction delay (NICD). The intrinsic QRS duration was not changed in responders, patients with dilated cardiomyopathy, or in the patient categories of male and female. However, the intrinsic QRS duration was significantly prolonged in non-responders and patients with ischemic cardiomyopathy (P = 0.041). The mean left ventricular end-diastolic diameter in the responder group was significantly decreased by CRT (P < 0.05), while there was no significant change in intrinsic QRS duration.While CRT does not reduce the intrinsic QRS duration, it can delay negative ventricular electrical remodeling. Continuous CRT is necessary. (Int Heart J 2015; 56: 495-499)

show abstract

Natural history of abnormal conduction and its relation to prognosis in patients with dilated cardiomyopathy

Cited by 237 publications

References 14 publications

Plakophilin-2 c.419C>T and risk of heart failure and arrhythmias in the general population

Plakophilin-2 c.419C>T and risk of heart failure and arrhythmias in the general population

Progressive conduction disease late after adriamycin treatment of lymphoma, despite normalised ejection fraction

Effects of Cardiac Resynchronization Therapy on Ventricular Electrical Remodeling in Patients With Heart Failure

Contact Info

Product

Resources

About