Search

Barth syndrome is a rare genetic disease caused by abnormal cardiolipin metabolism, characterized by high mortality within 5 years of diagnosis due to heart failure and/or infectious complications. This article describes a clinical case of an adult patient with Barth syndrome. The peculiarities of the course of the disease are described, including the transformation of the hypertrophic type of cardiomyopathy into the hypokinetic type as the patient grew older. This article demonstrates the difficulty in selecting the optimal treatment of a patient with Barth syndrome in real clinical practice, in the absence of clearly prescribed recommendations and pathogenetic therapy.

The article presents a clinical case of heart failure associated with the anthracycline-containing antitumor therapy in a breast cancer patient with an initially low risk of developing cardiovascular complications.

Aim: To evaluate the effect of Sacubitril/Valsartan (S/V) on the functional status, systolic and diastolic function of the left ventricle (LV), tolerability of therapy and to determine predictors of its effectiveness in patients with cancer therapy-related heart failure (СTRHF)., Materials and Methods: Forty patients 58 [46; 65.5] years of age with HF associated with anthracycline-containing cancer therapy were enrolled. Clinical examination, echocardiography, and assessment of potassium and creatinine levels were performed at baseline and after 6 months of S/V therapy., Results: NYHA functional class (FC) improvement was observed in 22 (64.7%) patients. Radiation therapy (RT) decreased (OR 0.091; 95% CI 0.01-0.83; p =0.03) while baseline low LV EF increased (OR 9.0; 95% CI 1.78-45.33; p =0.008) the odds of FC improvement. LV EF increased from 37.3 [30; 42.5] % to 45 [38; 48] % ( p <0.0001) and exceeded 50% in 7 (20.6%) patients. The odds of LV EF recovery increased when S/V therapy was initiated ≤1 year after anthracycline therapy (OR 10.67; 95% CI 1.57-72.67; p =0.0016) and decreased in patients with the history of RT (OR 0.14; 95% CI 0.02-0.89; p =0.0037) and in patients over 58 years (OR 0.07; 95% CI 0.01-0.68; p =0.022). LV diastolic function improvement included E/e' descent from 13.6 [10; 18.3] to 8.9 [6.9; 13.7] ( p =0.0005), and decrease in diastolic dysfunction grade in 18 (45%) patients ( p =0.0001). No significant change in serum potassium (4.45 [4.2; 4.8] versus 4.5 [4.3; 4.8]; p =0.5) and creatinine (75.4 [67.6; 85.1] versus 75.5 [68.2; 98.3]; p =0.08) levels were observed., Conclusion: S/V therapy is associated with improvement of EF, systolic and diastolic LV function, demonstrates a favorable tolerability profile in patients with СTRHF. Lack of RT and low baseline LV EF increased the odds of LV EF improvement; lack of RT, early (≤1 year) start of treatment after discontinuation of anthracycline therapy, and age <58 years increased the odds of LV EF recovery.

Aim      To evaluate a possibility of using radiofrequency catheter ablation guided by intracardiac echocardiography (ICE), its efficacy and safety for treatment of ventricular tachycardia (VT) of various etiology.Material and methods  Catheter intervention was performed for 20 enrolled patients with symptomatic VT. Ablation procedures were guided by a 3D electroanatomical mapping system and ICE.Results Mean duration of the procedure was 201.2±62.5 min. The procedure was successful (non-inducibility of VT) in 100% of cases. None of the patients had postoperative complications.Conclusion      Ablation of VT arrhythmogenic substrate guided by 3D electroanatomical navigational mapping and ICE without X-ray is feasible and safe.

The article summarizes the current experience of the nuclear medicine department of the Chazov National Medical Research Center of Cardiology in the implementation of myocardium scintigraphy with 99mTc-pyrophosphate with the differential diagnosis of the types of cardiac amyloidosis. Causes of false-positive, equivocal and non-diagnostic results, are analyzed. Possible ways to eliminate mistakes, including by modifying protocols of planar and tomographic research and optimizing the whole diagnostic algorithm for amyloidosis of the heart, are discussed.

Currently the incidence of congestive heart failure after ST-segment elevation myocardial infarction (STEMI) tends to increase. Reperfusion therapy is still the only effective method to reduce an infarct size. Therefore, there is a high unmet need of novel cardioprotective treatments that would improve outcomes in such patients. Recent advances in cardiovascular magnetic resonance (CMR) methods enabled the identification of certain new infarct characteristics associated with the development of heart failure and sudden cardiac death. These characteristics can help identify new groups of high risk patients and used as a targets for novel cardioprotective treatments. This part of the review summarizes novel CMR-based characteristics of myocardial infarction and their role in the prognostic stratification of STEMI patients.