Successful treatment with intravenous high-dose immunoglobulin for cardiomyopathy in dermatomyositis complicated with rapid progressive interstitial pneumonia.

Cardiomyopathy and rapid progressive interstitial pneumonia (IP) are potentially fatal complications in polymyositis/dermatomyositis. We experienced a dermatomyositis patient with multiple adverse prognostic factors, complicating rapid progressive IP, macrophage activation syndrome (MAS), and cardiomyopathy. IP and MAS improved with strong immunosuppressive therapy, despite which cardiomyopathy developed. Therefore, we applied intravenous high-dose immunoglobulin therapy (IVIg), and cardiac function improved dramatically. This is the first report to present the effectiveness of IVIg for cardiomyopathy in dermatomyositis.

Impact of preserved myocardial contractile function in the segments attached to the papillary muscles on reduction in functional mitral regurgitation.

BACKGROUND: Effectiveness of functional mitral regurgitation (FMR) in heart failure patients is of growing importance for patient prognosis. The purpose of this study was to investigate whether regional myocardial contractile function as assessed by tissue Doppler strain rate imaging can predict reduction in FMR caused by dobutamine. METHODS: Fifty-one patients with depressed left ventricular (LV) ejection fraction (32 ± 9%) secondary to dilated cardiomyopathy and FMR underwent evaluation of effective regurgitant orifice (ERO) of FMR, mitral valve deformation, global LV remodeling, and regional myocardial contractile function assessed by longitudinal peak systolic strain rate (Ssr) in 6 mid-LV segments from standard apical views. We also determined the average Ssr of segments attached to the papillary muscles, that is, the inferior, inferolateral, and anterolateral segments (PM segments Ssr). Low-dose (10 µg/kg per minute) dobutamine-induced reduction in ERO was compared with baseline variables. RESULTS: Baseline valve tenting was associated with dobutamine-induced reduction in ERO (r = -0.30, P < 0.05). Receiver operating characteristic curve analysis showed that baseline valve tenting, LV sphericity index, inferior Ssr, inferolateral Ssr, and PM segments Ssr were predictors of dobutamine-induced ≥30% reduction in ERO. Importantly, only PM segments Ssr predicted dobutamine-induced ≥20% reduction in valve tenting with area under the curve of 0.67 (P < 0.05). CONCLUSIONS: Preserved myocardial contractile function in the segments attached to the PMs was associated with dobutamine-induced reduction in mitral valve tenting and FMR, suggesting that our findings are important for improvement in cardiac function and FMR with medical treatment.

Dobutamine-induced improvement in inferior myocardial contractile function predicts reduction in functional mitral regurgitation: a study using tissue Doppler strain rate imaging.

BACKGROUND: left ventricular (LV) remodeling can increase tethering force to mitral valve and functional mitral regurgitation (FMR). Because the relationship between FMR and regional myocardial function has not been quantitatively evaluated, we conducted a quantitative investigation of this association. METHODS AND RESULTS: the effective regurgitant orifice (ERO) of FMR in 51 patients with depressed LV ejection fraction (32±9%) secondary to ischemic or nonischemic cardiomyopathy was compared with mitral deformation (valve and annulus), global LV remodeling (volume indices, function, and sphericity), and regional myocardial contractile function, as assessed by longitudinal peak systolic strain rate (Ssr) in LV anterior, anteroseptal, inferoseptal, inferior, inferolateral, and anterolateral segments at rest. Low-dose dobutamine (10 µg/kg per minute)-induced changes in ERO were compared with changes in the variables. Multivariable analysis identified the predictors of ERO at rest as mitral valvular tenting (ß=0.062; P<0.001), Ssr in the inferior segment (inferior Ssr) (ß=-0.178; P<0.001), and LV sphericity (ß=0.414; P=0.001) and the predictors of valvular tenting at rest as inferior Ssr (ß=-1.680; P<0.001), LV end-systolic volume index (ß=0.022; P=0.001), and LV sphericity (ß=3.886; P=0.012). Furthermore, dobutamine-induced reduction in ERO was predicted by reduction in valvular tenting (ß=0.087; P<0.001) and increase in inferior Ssr (ß=-0.082; P<0.001), and dobutamine-induced reduction in valvular tenting was predicted by increase in inferior Ssr (ß=-0.860; P<0.001). CONCLUSIONS: inferior regional myocardial dysfunction was quantitatively associated with mitral valvular tenting and FMR. Moreover, improvement with dobutamine of inferior myocardial contractile function attenuated valvular tenting and FMR. Inferior myocardial contractile function can affect the configuration of the mitral apparatus and predict FMR severity.

Preoperative systolic strain rate predicts postoperative left ventricular dysfunction in patients with chronic aortic regurgitation.

BACKGROUND: The best predictor for postoperative left ventricular (LV) systolic dysfunction in patients with chronic aortic regurgitation is still a matter of debate. The aim of this study was to assess the clinical significance of preoperative systolic radial strain rate (Ssr) derived from tissue Doppler echocardiography as a predictor of postoperative LV systolic dysfunction in patients with chronic aortic regurgitation. METHODS AND RESULTS: In 52 patients (mean age, 58 years; 13 women) with isolated chronic aortic regurgitation, we performed standard and tissue Doppler echocardiography before and after operation, obtained echocardiographic parameters such as LV dimensions and LV ejection fraction, and measured Ssr in 4 walls of the LV. Linear regression analysis determined correlations between preoperative parameters and postoperative LV ejection fraction. Receiver-operating characteristic curve analysis assessed the optimal cutoff values of parameters that predicted postoperative LV systolic dysfunction (ejection fraction <50%). The operation caused significant decreases in LV dimensions and volumes and significant increases in Ssr (1.94+/-0.64 to 2.39+/-0.83 per second; P<0.001) and ejection fraction (53.0+/-8.7 to 59.0+/-8.8%; P<0.001). Multiple regression analysis demonstrated that averaged Ssr was the only independent predictor of postoperative LV systolic dysfunction among the covariates examined (P<0.001). Using receiver-operating characteristic curve analysis, averaged Ssr yielded the greatest area under the curve among preoperative parameters (0.80) and was indicated to be a good predictor of postoperative LV dysfunction, with 90.9% sensitivity and 73.2% specificity (cutoff value, 1.82 per second). CONCLUSIONS: Measurement of preoperative averaged Ssr is useful in predicting postoperative LV systolic dysfunction and optimizing surgical timing in patients with isolated chronic aortic regurgitation.

Utility of phase contrast MR imaging for assessment of pulmonary flow and pressure estimation in patients with pulmonary hypertension: comparison with right heart catheterization and echocardiography.

PURPOSE: To compare the utility of phase contrast MR imaging (PC-MRI) for assessment of pulmonary flow and pressure estimation with that of right heart catheterization and echocardiography (cardiac US) in patients with pulmonary arterial hypertension (PAH). MATERIALS AND METHODS: Twenty consecutive patients with suspected PAH underwent PC-MRI, cardiac US, and right heart catheterization. In each patient, PC-MRI was acquired by cine 2D-PC method on a 1.5 Tesla scanner, and stroke volume (SV) and pulmonary arterial systolic pressure (PASP) were assessed by using the modified Bernoulli's equation. To evaluate the agreements of SV and PASP among the three methods, correlations and limits of agreement among the three methods were statistically assessed by using the Bland-Altman's analyses. RESULTS: The correlations and limits of agreement for SV and PASP between PC-MRI and catheterization (r = 0.96, r(2) = 0.94, 1.1 +/- 6.9 mL and r = 0.94, r(2) = 0.88, -3.2 +/- 14.5 mmHg, respectively) were better than between cardiac US and catheterization (r = 0.01, r(2) < 0.01, 8.9 +/- 42.1 mL and r = 0.86, r(2) = 0.72, -5.9 +/- 27.7 mmHg, respectively). CONCLUSION: PC-MRI is more compatible with right heart catheterization than cardiac US in pulmonary flow and pressure estimation.

Large response to cardiac resynchronization therapy in a patient with segmental paradoxical systolic expansion identified by strain imaging.

An 84-year-old man with non-ischaemic cardiomyopathy underwent cardiac resynchronization therapy (CRT) based on the presence of drug-refractory heart failure, depressed left ventricular ejection fraction (25%), and wide QRS complex (160 ms). Longitudinal tissue velocity revealed no significant dyssynchrony (23 ms in Yu index and 35 ms in opposing wall delay). However, longitudinal tissue Doppler strain revealed unique appearances in apical four-chamber and long-axis views. The anterior and inferior septum at basal and mid-levels had reversed strain (becoming positive), indicating paradoxical systolic expansion. Ejection fraction dramatically improved from 26 to 50% the day following CRT, and this beneficial effect of CRT was sustained 12 months following CRT. The presence of the segmental reversed strain might have a potential to predict a large response to CRT in the assessment of longitudinal dyssynchrony.

Accessory mitral valve associated with aortic and mitral regurgitation and left ventricular outflow tract obstruction in an elderly patient: a case report.

A 65-year-old man was admitted to our hospital because of exertional dyspnea. Transthoracic and transesophageal echocardiography showed a parachute-like structure measuring 20 x 16 mm, which projected into the left ventricular outflow tract (LVOT) and passed through the aortic valve in systole, and prolapsed back into the left ventricular cavity in diastole. Moderate aortic and mitral regurgitation were also observed, as well as LVOT obstruction with a peak gradient of 30 mmHg. There were no other congenital cardiac abnormalities. In addition, real-time three-dimensional transthoracic echocardiography showed that the parachute-like structure in the LVOT was attached to the anterior mitral leaflet and left ventricular lateral wall by a chorda tendineae-like structure. The diagnosis of accessory mitral valve was based on the echocardiographic characteristics. Surgical treatment was performed because of the presence of accessory mitral valve, moderate aortic and mitral regurgitation, and LVOT obstruction. The postoperative course was uneventful, and the patient has been asymptomatic during a follow-up period of 24 months. Echocardiographic examination proved to be useful for the detection of accessory mitral valve.

Relationship between regional and global left ventricular systolic and diastolic function in patients with coronary artery disease assessed by strain rate imaging.

BACKGROUND: The relationship between regional and global left ventricular function has not been evaluated. The present study aimed to assess whether regional myocardial contraction and relaxation reflect global left ventricular systolic and early diastolic function, respectively. METHODS AND RESULTS: The study involved 45 patients with coronary artery disease (CAD). Conventional echocardiography was performed, and segmental peak strain rate (SR) in the systole (S(SR)) and early diastole (E(SR)) were obtained from tissue Doppler imaging in each of 18 segments of the left ventricular myocardium from the apical views. The E(SR) was significantly correlated with S(SR) in all studied segments (r=0.55, p<0.0001). The average values of SR indices in each patient were defined as S(SR) index and E(SR) index, which were derived by dividing the summed value of the S(SR) or E(SR) by the number of respective assessable segments. A significant positive relationship was observed between the S(SR) index and left ventricular ejection fraction (r=0.85, p<0.0001). Also, there was a positive correlation between the E(SR) index and mitral flow E (r=0.68, p<0.0001). CONCLUSIONS: Regional myocardial contraction and relaxation, as assessed by SR imaging, were closely related in patients with CAD. Furthermore, regional myocardial contraction and relaxation are important factors affecting global left ventricular systolic and early diastolic function.

Improved regional myocardial diastolic function assessed by strain rate imaging in patients with coronary artery disease undergoing percutaneous coronary intervention.

This study investigated the effects of percutaneous coronary intervention (PCI) on global and regional left ventricular diastolic function, as assessed by strain rate (SR) imaging. In 27 patients with coronary artery disease, we performed echocardiography before and after PCI to obtain segmental peak systolic SR and peak early diastolic SR (E(SR)). PCI did not significantly change peak systolic SR in the ischemic (1.59 +/- 0.59-1.66 +/- 0.52/s) and nonischemic (1.64 +/- 0.59-1.61 +/- 0.53/s) segments. E(SR) in the ischemic segments was significantly smaller than that in the nonischemic segments at rest (1.82 +/- 0.71 vs 2.03 +/- 0.64/s, P < .01). PCI caused a significant increase in E(SR) from 1.82 +/- 0.71 to 2.29 +/- 0.92/s in the ischemic (P < .001), but not in the nonischemic, segments. The peak early diastolic transmitral flow velocities after PCI were improved in patients with greater extent of improvement of E(SR) in the ischemic segments. These findings suggest that the improvement in left ventricular early diastolic filling after PCI may be associated with the degree of improvement in impaired regional myocardial relaxation.

Surgical treatment for Löffler's endocarditis with left ventricular thrombus and severe mitral regurgitation: a case report.

A 65-year-old female was admitted to our hospital because of dyspnea. Laboratory examinations revealed hypereosinophilia at a local hospital. Transthoracic and transesophageal echocardiography showed normal left ventricular dimension and function. The left ventricular apex was obliterated and the posterior and lateral walls were thickened by an abnormal mass. The posterior mitral leaflet was encapsulated by this abnormal mass. The limited motion of the posterior mitral leaflet caused mitral malcoaptation, resulting in severe mitral regurgitation. Hypereosinophilia was considered to be idiopathic, as no other disorders known to cause secondary eosinophilia were found. No other organ dysfunction was associated with the condition. Thus, the diagnosis was Loffler's endocarditis associated with hypereosinophilic syndrome. The patient was given conservative medical treatment immediately on admission. However, heart failure caused by mitral regurgitation would be difficult to treat with conservative medical treatment, so we chose a surgical strategy. The symptoms obviously improved after valve replacement and removal of the abnormal mass, and the patient was discharged. However, she died of cerebral infarction at a local hospital 3 months later.

Pulmonary artery stenosis due to external compression by a calcified pericardial band.

A 60-year-old male with exertional dyspnea was referred to our hospital. Right pulmonary artery stenosis due to external compression by a calcified band was diagnosed by echocardiography, computed tomography, and magnetic resonance imaging. Percutaneous transluminal angioplasty was conducted in vain due to vascular recoil and failure of stent delivery. Pulmonary bypass grafting was performed successfully. The surgery indicated a probable etiology of chronic pericarditis. This is an extremely rare case of adult pulmonary artery stenosis without a known history of congenital disease, constrictive pericarditis, tuberculosis, or surgery.

[A case of malignant lymphoma associated with diffuse pulmonary involvement successfully treated with rituximab].

A 66-year-old man was admitted to our hospital with a chief complaint of fever. Histopathological examination of the inguinal lymph nodes revealed follicular B-cell non-Hodgkin's lymphoma (NHL). In spite of 9 cycles of chemotherapy (CHOP/COP), progression of the disease was seen. Fever and dyspnea developed. The chest radiograph revealed diffuse pulmonary infiltrates. Transbronchial biopsy revealed pulmonary involvement of diffuse B-cell lymphoma. The tumor cells showed positive staining for CD20. Salvage chemotherapy (ESHAP, EPOCH) was not effective. We decided to perform mono-immunotherapy with the monoclonal CD20 antibody rituximab. One month later, the patient was in almost complete remission without adverse events. It is suggested that rituximab may be effective for refractory NHL with pulmonary involvement.