Evaluation of Index of Cardiac Electrophysiological Balance in Patients With Myotonic Dystrophy Type 1.

BACKGROUND: Myotonic dystrophy type 1(MD1), which is characterized by decreased muscle tone, progressive muscle weakness, and cardiac involvement, is an autosomal dominant and progressive congenital muscle disease. Cardiac involvement more often manifests as conduction abnormalities and arrhythmias (such as supraventricular or ventricular). Approximately one-third of MD1-related deaths occur due to cardiac causes. The index of cardiac-electrophysiological balance (ICEB) is a current parameter calculated as QT interval/QRS duration. The increase in this parameter has been associated with malignant ventricular arrhythmias. In this study, our aim was to compare the ICEB values ​​of MD1 patients and the normal population. MATERIAL AND METHOD: A total of 62 patients were included in our study. They were divided into two groups - 32 MD patients and 30 controls. The demographic, clinical, laboratory, and electrocardiographic parameters of the two groups were compared. RESULTS: The median age of the study population was 24 (20-36 IQR), and 36 (58%) of these patients were female. Body mass index was higher in the control group (p = 0.037). While in the MD1 group creatinine kinase was significantly higher (p <0.001), In the control group creatinine, aspartate aminotransferase, alanine aminotransferase, calcium, and lymphocyte levels were significantly higher (p=0.031, p= 0.003, p=0.001, p=0.002, p=0.031, respectively). ICEB [3.96 (3.65-4.46) vs 3.74 (3.49-3.85) p=0.015] and corrected ICEB (ICEBc) [4.48 (4.08-4.92) vs 4.20 (4.03-4.51) p = 0.048] were significantly higher in the MD1 group. CONCLUSION: In our study, ICEB was found to be higher in MD1 patients than in the control group. Increased ICEB and ICEBc values ​​in MD1 patients may precipitate ventricular arrhythmias in the future. Close monitoring of these parameters can be helpful in predicting possible ventricular arrhythmias and in risk stratification.

Pulmonary hypertension in Takayasu arteritis.

AIM: To determine the frequency and define the causes of pulmonary hypertension (PH) in patients with Takayasu arteritis (TA). METHOD: Sixty-four TA patients were evaluated by transthoracic echocardiography (TTE). Having an estimated systolic pulmonary arterial pressure (sPAP) ≥40 mm Hg by echocardiography or if performed, mean PAP ≥25 mm Hg in right heart catheterization was defined as PH. Clinical, imaging and laboratory results of the TA patients were obtained from hospital files. RESULT: In total, seven (10.9%) patients had PH. Four patients had PH due to left-sided heart disease (group 2 PH), three patients due to pulmonary arterial involvement (PAI; group 4 PH) and one patient due to atrial septal defect (group 1 PH). In one patient, combination of PAI, aortic insufficiency and pulmonary venous return anomaly was present and he was considered to have both group 2 and group 4 PH. PAI was more frequent (42.9% vs 15.7%) in patients with PH but the difference was not statistically significant. The percentage of patients treated with cyclophosphamide and/or biologics was higher in the group with PH as compared to the group without PH (P = 0.015). One patient with group 4 PH had been on pulmonary arterial hypertension (PAH)-specific agents for 8 years. CONCLUSION: Pulmonary hypertension is not infrequent in TA patients and all the potential causes of PH should be carefully evaluated. Patients with severe or treatment-resistant disease are prone to have PH. PAH-specific agents may be effective in patients with group 4 PH.