Comprehensive non-invasive assessment of electrocardiographic abnormalities and cardiac arrhythmias in patients with genetically confirmed mitochondrial diseases.

BACKGROUND: There is limited data on cardiac arrhythmias and ventricular repolarization and dispersion abnormalities in patients with mitochondrial diseases (MitD). METHODS: Consecutive 40 patients with genetically proven MitD and 35 healthy controls were studied. Among other examinations all subjects underwent 24-h Holter recording and 12­lead electrocardiography (ECG) with corrected QT (QTc), QT dispersion (QTd), Tp-e and Tp-e/QT ratio assessment. RESULTS: Patients with MitD were 55.4 ± 15.7 years old, the disease duration was 18.5 ± 10.3 years, presented 6 clinical syndromes while mitochondrial and nuclear DNA type of mutation was present in 40 and 60% of cases, respectively. In MitD more frequently 1st degree atrioventricular block and intraventricular conduction defects were observed and also QRS complex duration was increased. Mean values of QTc (p = 0.001), QTd (p = 0.02), Tp-e (p < 0.00001) and Tp-e/QT (p < 0.00001) were significantly higher in MitD than in controls. Correlations between disease duration and PR interval duration (p = 0.003) and Creatine Kinase MB isoenzyme activity (p = 0.02) were found. No differences in depolarization and dispersion parameters were observed according to type of mutation or dominant clinical syndromes. In addition to supraventricular extrasystoles, nonsustained supraventricular tachycardias occurred more frequently in MitD (in 45.0 vs 14.3%, p = 0.0004). Ventricular arrhythmias were rare and observed almost exclusively in subjects with mitochondrial DNA mutation. CONCLUSIONS: In contrast to healthy controls, in MitD patients intraventricular, repolarization and dispersion disturbances were more frequently observed. In addition to bradyarrhythmias observed in some defined MitD syndromes, supraventricular rather than ventricular arrhythmias are more common.

"The post-pulmonary syndrome - results of echocardiographic driven follow up after acute pulmonary embolism".

INTRODUCTION: The concept of post Pulmonary Embolism syndrome includes various combinations of functional, haemodynamic or imaging abnormalities in patients after pulmonary embolism (PE). Although residual obstruction of pulmonary vascular bed is suggested to be a major cause of post Pulmonary Embolism syndrome (post-PE syndrome) other cardiopulmonary abnormalities can be responsible for functional impairment. Therefore, we analyzed the frequency of post-PE syndrome and its potential causes. MATERIAL AND METHODS: We report data of consecutive 845 PE survivors (468 F, aged 62 ± 18 yrs) who were anticoagulated, and followed for at least 6 months. All symptomatic subjects at follow up underwent diagnostic workup. RESULTS: 35% (290/845) of PE survivors recovered functionally, while 65% patients reported a decreased exercise tolerance compatible with post-PE syndrome. One hundred and five symptomatic cases were lost to follow up. After diagnostic workup, chronic thromboembolic pulmonary hypertension (CTEPH) was diagnosed in 38 of 450 (8.4%) symptomatic subjects and chronic thromboembolic pulmonary disease (CTED) was diagnosed in 15/450 (3.3%) of them. Chronic heart failure with reduced ejection fraction (EF) was found in 6.9% (31/450) of patients and 154 patients (34.2%) had leftsided diastolic dysfunction. Valve heart disease was detected in 6.2% (28/450), atrial fibrillation in 31/450 (6.9%), Other causes of reduced exercise tolerance include coronary artery disease in 31/450 (6.9%), pulmonary disease 42/450 (9.3%), morbid obesity 15/450 (3.3%), neoplasms 15/450 (3.3%), psychiatric disorders 1%, rheumatoid disease 1%, anemia 1%. CONCLUSIONS: Approximately 65% of PE survivors report functional impairment, despite at least 6 months of anticoagulation. Persistent pulmonary artery thromboemboli resulting in CTEPH or CTED were detected in 7.2% of PE survivors and 11.8% of symptomatic patients. Leftsided diastolic dysfunction was the most prevalent echocardiographic abnormality, and remained the most common cause of functional limitation affected 34.2% of symptomatic cases.