Peripartum Cardiomyopathy Presenting with Syncope due to Torsades de Pointes: a Case of Long QT Syndrome with a Novel KCNH2 Mutation

Nishimoto, Orie; Matsuda, Morihiro; Nakamoto, Kei; Nishiyama, Hirohiko; Kuraoka, Kazuya; Taniyama, Kiyomi; Tamura, Ritsu; Shimizu, Wataru; Kawamoto, Toshiharu

doi:10.2169/internalmedicine.51.5943

Cited by 15 publications

(9 citation statements)

References 9 publications

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“…KCNH2 c.2682_2685dup p.(Asp896Hisfs*25) is located in the 3′ end of exon 11 and within a few base pairs reach of 3 other frameshift variants previously reported in patients with LQTS 19, 20, 21…”

Section: Resultsmentioning

confidence: 92%

Genetic and Phenotypic Characterization of Community Hospital Patients With QT Prolongation

Gibbs

Thalamus

Tveten

et al. 2018

JAHA

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BackgroundCongenital long‐QT syndrome (LQTS) is a genetic disorder characterized by prolongation of the corrected QT interval (QTc) on an ECG. The aim of the present study was to estimate the prevalence of pathogenic and likely pathogenic sequence variants in patients who had at least 1 ECG with a QTc ≥500 ms.Methods and ResultsTelemark Hospital Trust is a community hospital within the Norwegian national health system, serving ≈173 000 inhabitants. We searched the ECG database at Telemark Hospital Trust, Norway, from January 2004 to December 2014, and identified 1531 patients with at least 1 ECG with a QTc ≥500 ms. At the time of inclusion in this study (2015), 766 patients were alive. A total of 733 patients were invited to participate, and 475 accepted. The 17 genes that have been reported to cause monogenic LQTS were sequenced among the patients. Pro‐QTc score was calculated for each patient. A molecular genetic cause of LQTS was detected in 31 (6.5%) of 475 patients. These patients had a lower pro‐QTc score than those without pathogenic or likely pathogenic variants (1.7±1.0 versus 2.8±1.6; P<0.001).ConclusionsCompared with the general population, hospitalized patients with a QTc ≥500 ms in at least 1 ECG recording had an increased likelihood for pathogenic and likely pathogenic variants in LQTS genes. We recommend increased awareness of the possibility of LQTS in patients with at least 1 ECG with a QTc ≥500 ms.

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Section: Resultsmentioning

confidence: 92%

Genetic and Phenotypic Characterization of Community Hospital Patients With QT Prolongation

Gibbs

Thalamus

Tveten

et al. 2018

JAHA

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“…Hormonal changes are suggested to play a role in the pathophysiological mechanism 54 55. A ventricular arrhythmia in the third trimester or postpartum period in previously healthy woman may also be the first presentation of peripartum cardiomyopathy 56 57. A remarkably reduced risk of events in long QT syndrome has been observed during the course of pregnancy, while the postpartum period seems to be associated with an increased risk 58…”

Section: Arrhythmiasmentioning

confidence: 99%

Managing cardiac emergencies in pregnancy

Hagen

Cornette

Johnson

et al. 2016

Heart

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“…Although a normal ECG does not rule out the diagnosis, most women suffering from PPCM have an abnormal ECG. The most common abnormalities on the ECG are ST- or T-wave abnormalities, p-wave abnormality, bundle branch block, left ventricular hypertrophy, ventricular or supraventricular arrhythmias, and QRS-axis deviation [56-58]. Radiological signs of heart failure such as cardiomegaly, Kerley B lines, prominent pulmonary vasculature, pulmonary edema, and pleural effusion can be found at chest X-ray.…”

Section: Imagingmentioning

confidence: 99%

Peripartum Cardiomyopathy: Current State of Knowledge, New Developments and Future Directions

Biteker¹,

Kayataş²,

Duman³

et al. 2014

CCR

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Peripartum cardiomyopathy (PPCM) is a form of idiopathic dilated cardiomyopathy affecting women in late pregnancy or early puerperium. Although initially described in the late 1800s, it has only recently been recognized as a distinct cardiac condition. The reported incidence and prognosis varies according to geography. The clinical course varies between complete recovery to rapid progression to chronic heart failure, heart transplantation or death. In spite of significant improvements in understanding the pathophysiology and management of the PPCM many features of this unique disease are poorly understood, including incidence, etiology, epidemiology, pathophysiology, predictors of prognosis and optimal therapy. The present article revisits these concepts and recent advances in PPCM.

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Peripartum Cardiomyopathy Presenting with Syncope due to Torsades de Pointes: a Case of Long QT Syndrome with a Novel KCNH2 Mutation

Cited by 15 publications

References 9 publications

Genetic and Phenotypic Characterization of Community Hospital Patients With QT Prolongation

Genetic and Phenotypic Characterization of Community Hospital Patients With QT Prolongation

Managing cardiac emergencies in pregnancy

Peripartum Cardiomyopathy: Current State of Knowledge, New Developments and Future Directions

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