Left ventricular mechanics in patients with hematological malignancies before initiation of chemo- and radiotherapy.

Objective We sought to investigate left ventricular (LV) structure, function and mechanics in the patients with leukemia and lymphoma before initiation of chemotherapy, as well as the relationship between hematological malignancies and reduced LV longitudinal strain. Methods This retrospective investigation included 71 patients with leukemia and lymphoma before chemotherapy and 36 healthy controls. All participants underwent echocardiographic examination before initiation of chemotherapy and radiotherapy. Results LV global longitudinal strain (- 20.2 ± 1.7% vs. - 17.9 ± 3.0%, p < 0.001) was significantly lower in the patients with hematological malignancies than in controls. There was no difference in LV circumferential and radial strains between two observed groups. Subendocardial and subepicardial longitudinal strains were significantly lower in the patients with hematological malignancies (- 20.5 ± 3.6% vs. - 22.5 ± 3.8%, p = 0.001 for subendocardial strain; - 18.0 ± 1.5% vs. - 15.8 ± 2.6%, p < 0.001 for subepicardial strain). Hematological malignancies were associated with reduced global LV longitudinal strain (OR 21.0; 95%CI 2.04-215.0, p = 0.010) independently of age, gender, heart rate, body mass index, left ventricular ejection fraction, left ventricular mass index, and glucose level. Conclusions LV longitudinal strain was impaired in the patients with leukemia and lymphoma even before initiation of chemotherapy. Endocardial and epicardial LV layers are equally affected in the patients with hematological malignancies. Newly diagnosed hematological malignancies were related with reduced LV global longitudinal strain independently of common clinical and echocardiographic parameters.

Right heart masses in a patient with endometrial stromal sarcoma.

We present the case of a 61-year-old woman with a large tumoral infiltration extending from the pelvis throughout the inferior vena cava inferior to the right atrium, protruding into the right ventricle and right ventricular outflow tract. She had been treated 10 years before for low-grade endometrial stromal sarcoma by hysterectomy and adnexectomy followed by hormone- and radio-therapy. Due to cancer recurrence, she underwent peritonectomy, appendectomy, and resection of terminal ileum.

Phasic Left Atrial Function in Cancer Patients Before Initiation of Anti-Cancer Therapy.

: We aimed to explore left atrial (LA) remodeling in the patients with solid cancer before initiation of chemo- or radiotherapy. This retrospective investigation included 92 chemo- and radiotherapy-naive cancer patients and 40 age- and gender-matched controls with a similar cardiovascular risk profile as the cancer group. All participants underwent comprehensive echocardiographic examination before the start of chemo- or radiotherapy. LA phasic function was evaluated in volumetric and strain method. Indexed minimal and pre-A LA volumes were significantly higher in the cancer patients. Total and passive LA emptying fraction (EF) were significantly lower, whereas active LAEF was significantly higher in the cancer patients. LA total longitudinal strain was significantly lower in the cancer patients. Strain rate analysis of LA phasic function showed that LA function during systole and early diastole was reduced in the cancer group, while it was increased during late diastole. These findings indicated that LA reservoir and conduit functions, assessed with LA volumetric and strain analysis, were deteriorated in the cancer group. On the other hand, LA booster pump function was elevated in the cancer group in comparison with the controls. In the whole population, cancer was associated with reduced LA total longitudinal strain independently of age, gender, BMI, LV hypertrophy, E/e' ratio, diabetes, and hypertension. LA phasic function was impaired in the chemo- and radiotherapy-naive cancer patients in comparison with the control group. Cancer, LV hypertrophy, and hypertension were associated with reduced LA longitudinal strain independently of other important clinical parameters.

Right ventricular function and mechanics in chemotherapy- and radiotherapy-naïve cancer patients.

The present research evaluated right ventricular (RV) structure, function and mechanics in the cancer patients before initiation of chemo- or radiotherapy, and the association between cancer and decreased RV longitudinal strain. This retrospective investigation included 101 chemo- and radiotherapy-naïve patients with solid cancer and 38 age- and gender-matched controls with similar cardiovascular risk profile. Echocardiographic examination and strain evaluation was performed in all participants. RV structure and RV systolic and diastolic function estimated with conventional echocardiographic parameters were similar between the cancer patients and controls. However, RV global longitudinal strain (- 22.7 ± 2.6% vs. - 21.1 ± 2.4%, p < 0.001) was significantly decreased in the cancer patients than in controls. The same was revealed for RV free wall endocardial (- 33.6 ± 4.3% vs. - 31.4 ± 4.0%, p = 0.006) and mid-myocardial (- 25.2 ± 3.6% vs. - 23.7 ± 3.8%, p = 0.035) longitudinal RV strains, whereas difference was not found in RV free wall epicardial longitudinal strain. The presence of cancer was independently of age, gender, body mass index, left ventricular hypertrophy, diabetes, hypertension and pulmonary pressure associated with reduced RV global longitudinal strain (OR 3.79; 95% CI 2.18-10.92, p < 0.001), as well as with decreased free wall RV longitudinal strain (OR 5.73; 95% CI 3.17-9.85, p < 0.001). RV strain is deteriorated in the chemo- and radiotherapy-naïve cancer patients. Endocardial and mid-myocardial layers are more affected than epicardial strain in the cancer patients. The presence of cancer is independently of other clinical parameters associated with reduced RV longitudinal strain.

Beyond the sarcomere: CSRP3 mutations cause hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is a frequent genetic cardiac disease and the most common cause of sudden cardiac death in young individuals. Most of the currently known HCM disease genes encode sarcomeric proteins. Previous studies have shown an association between CSRP3 missense mutations and either dilated cardiomyopathy (DCM) or HCM, but all these studies were unable to provide comprehensive genetic evidence for a causative role of CSRP3 mutations. We used linkage analysis and identified a CSRP3 missense mutation in a large German family affected by HCM. We confirmed CSRP3 as an HCM disease gene. Furthermore, CSRP3 missense mutations segregating with HCM were identified in four other families. We used a newly designed monoclonal antibody to show that muscle LIM protein (MLP), the protein encoded by CSRP3, is mainly a cytosolic component of cardiomyocytes and not tightly anchored to sarcomeric structures. Our functional data from both in vitro and in vivo analyses suggest that at least one of MLP's mutated forms seems to be destabilized in the heart of HCM patients harbouring a CSRP3 missense mutation. We also present evidence for mild skeletal muscle disease in affected persons. Our results support the view that HCM is not exclusively a sarcomeric disease and also suggest that impaired mechano-sensory stress signalling might be involved in the pathogenesis of HCM.