Cardiomyopathies/physiopathology

ABCIC

Viewpoint

Non-compaction cardiomyopathy: How Can Imaging Contribute?

José de Arimateia Batista Araújo Filho et al. Antonildes Nascimento Assunção-Jr José Rodrigues Parga

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IJCS

Case Report

Isolated Left Ventricular Apical Hypoplasia without Lamin A/C Gene Mutation

Paulo Henrique Manso et al. Veridiana Kiill Suazo Fernando Tadeu Vasconcelos Amaral Mauro Cruz Jurca Henrique Simão Trad

Abstract

Isolated left ventricular apical hypoplasia is a rare cardiomyopathy, with a broad range of clinical presentations. Since this entity was already described in association with osteomuscular diseases, mutation in the Lamin A/C gene has been regarded as a possible cause of this disease. This study describes the case of an asymptomatic teenager with isolated left ventricular apical hypoplasia and arthrogriposis but with no mutations in the entire Lamin A/C gene.

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ABC

Original Article

Prognostic Value of a New Marker of Ventricular Repolarization in Cirrhotic Patients

Angelo Antunes Salgado et al. Paulo Roberto Benchimol Barbosa Alinne Gimenez Ferreira Camila Aparecida de Souza Segrégio Reis Carlos Terra

Abstract

Background:

There is still debate about the relationship between changes in ventricular repolarization on the surface electrocardiogram and cirrhosis severity.

Objective:

To study the relationship between variables related to ventricular repolarization and the clinical severity of the cirrhotic disease.

Methods:

We selected 79 individuals with hepatic cirrhosis, classified according to the Child-Pugh-Turcotte criteria (Child A, B, and C). We measured the QT and corrected QT (QTc) intervals, and the interval between the peak and the end of the T wave (TpTe), and we identified their minimum, maximum, and mean values in the 12-lead electrocardiogram. We also calculated the dispersion of the QT (DQT) and QTc (DQTc) intervals.

Results:

In 12 months of clinical follow-up, nine subjects underwent hepatic transplantation (Child A: 0 [0%]; Child B: 6 [23.1%]; Child C: 3 [18.8%]; p = 0.04) and 12 died (Child A: 3 [12.0%]; Child B: 4 [15.4%]; Child C: 5 [31.3%]; p = 0.002). No significant differences were observed between the cirrhotic groups related to the minimum, maximum, and mean values for the QT, QTc, TpTe, DQT, and DQTc intervals. A minimum TpTe interval ≤ 50 ms was a predictor for the composite endpoints of death or liver transplantation with a sensitivity of 90% and a specificity of 57% (p = 0.005). In the Cox multivariate analysis, the Child groups and a minimum TpTe of ≤ 50 ms were independent predictors of the composite endpoints.

Conclusion:

The intervals QT, QTc, DQT, DQTc, and TpTe have similar distributions between different severity stages in cirrhotic disease. The TpTe interval proved to be a prognostic marker in subjects with cirrhosis, regardless of disease severity (NCT01433848).

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Non-compaction cardiomyopathy: How Can Imaging Contribute?

Isolated Left Ventricular Apical Hypoplasia without Lamin A/C Gene Mutation

Abstract

Prognostic Value of a New Marker of Ventricular Repolarization in Cirrhotic Patients

Abstract

Background:

Objective:

Methods:

Results:

Conclusion:

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