Muneyoshi Tanimura

Recurrent inflammatory aortic aneurysms in chronic mucocutaneous candidiasis with a gain-of-function STAT1 mutation

a Department of Cardiology and Nephrology, Mie University Graduate School of Medicine, Tsu, Japan b Department of Pediatrics, Mie University Graduate School of Medicine, Tsu, Japan c Department of Thoracic and Cardiovascular Surgery, Mie University Graduate School of Medicine, Tsu, Japan d Department of Hematology and Oncology, Mie University Graduate School of Medicine, Tsu, Japan e Department of Pathology and Matrix Biology, Mie University Graduate School of Medicine, Tsu, Japan f Department of Cell Transplantation, Mie University Graduate School of Medicine, Tsu, Japan g Mie University Research Center for Matrix Biology, Tsu, Japan

Quantifying longitudinal right ventricular dysfunction in patients with old myocardial infarction by using speckle-tracking strain echocardiography

BackgroundWe investigated longitudinal right ventricular (RV) function assessed using speckle-tracking strain echocardiography in patient with myocardial infarction (MI), and identified the contributing factors for RV dysfunction.MethodsWe retrospectively studied 71 patients with old MI (the OMI group) and 45 normal subjects (the Control group) who underwent a transthoracic echocardiography. Global and free wall RV peak systolic strains (PSSs) in the longitudinal direction were measured by using speckle-tracking strain echocardiography. Left ventricular (LV) PSSs were measured in the longitudinal, radial and circumferential directions. Cardiac hemodynamics including peak systolic pulmonary artery pressure was also assessed non-invasively. Plasma brain natriuretic peptide (BNP) levels were measured in all patients.ResultsIn the OMI group, 73% of the patients had a normal estimated peak systolic pulmonary artery pressure of less than 35 mmHg. Global and free wall RV PSS were impaired in the OMI group compared with the Control group, and these RV systolic indices were significantly associated with heart rate, logarithmic transformed plasma BNP, greater than 1 year after onset of MI, Doppler-derived estimated pulmonary vascular resistance, LV systolic indices, LV mass index, infarcted segments within a territory of the left circumflex artery and residual total occlusion in the culprit right coronary artery. Multivariable linear regression analysis indicated that reduced longitudinal LV PSS in the 4-chamber view and BNP levels ≥500 pg/ml were independently associated with reduced global and free wall RV PSS. Moreover, when patients were divided into 3 groups according to plasma BNP levels (BNP <100 pg/ml; n = 31, 100 ≤BNP <500 pg/ml; n = 24, and BNP ≥500 pg/ml; n = 16), only patients with BNP ≥500 pg/ml had a strong correlation between RV PSS and longitudinal LV PSS in the 4-chamber view (r = 0.78 for global RV PSS and r = 0.71 for free wall RV PSS, p <0.05).ConclusionLongitudinal RV systolic strain depends significantly on longitudinal LV systolic strain especially in patients with high plasma BNP levels, but not on estimated peak systolic pulmonary artery pressure. These results indicate that process of RV myocardial dysfunction following MI may be governed by neurohormonal activation which causing ventricular remodeling rather than increased RV afterload.

Combination of Urinary Sodium/Creatinine Ratio and Plasma Brain Natriuretic Peptide Level Predicts Successful Tolvaptan Therapy in Patients With Heart Failure and Volume Overload

To evaluate the short-term clinical and hemodynamic effects of tolvaptan therapy and to identify predictors of the therapeutic outcomes, we retrospectively recruited 60 consecutive hospitalized heart failure (HF) patients (70 ± 11 years) with volume overload. The subjects were divided into two groups on the basis of the changes in HF symptom scores and hemodynamic status assessed by right heart catheterization after tolvaptan therapy (median: 7 days). The majority of patients were successfully treated (group 1). However, 22% of patients (group 2) were unsuccessfully treated, in whom 1) the HF symptom score worsened or 2) there was a stationary HF symptom score ≥ 6 points, and mean PCWP > 18 mmHg and mean RAP > 10 mmHg, after tolvaptan therapy. HF symptom scores, hemodynamic parameters, and plasma brain natriuretic peptide (BNP) level improved in group 1, but all of these parameters remained unchanged in group 2. Lower urine sodium/creatinine ratio (UNa/UCr) and higher BNP level at baseline were independently associated with unsuccessful tolvaptan therapy, and UNa/UCr best predicts unsuccessful tolvaptan therapy with a cut-off value of 46.5 mEq/g·Cr (AUC 0.847, 95% CI: 0.718-0.976, sensitivity 77%, specificity 81%, P < 0.01). Double-positive results of UNa/UCr < 46.5 mEq/g·Cr and plasma BNP level > 778 pg/mL predicted unsuccessful tolvaptan therapy with high diagnostic accuracy (sensitivity 54%, specificity 100%, positive predictive value 100%, negative predictive value 89%, and accuracy 90%). In summary, short-term tolvaptan therapy ameliorated HF symptoms and provided hemodynamic improvement in the majority of patients, and UNa/UCr and BNP level strongly predicted the therapeutic outcomes.

Tocilizumab for uncontrollable systemic inflammatory response syndrome complicating adult-onset Still disease: Case report and review of literature

Rationale: Adult-onset Still disease (AOSD) is a rare systemic inflammatory disease of unknown etiology characterized by evanescent salmon-pink rash, fever spikes, arthralgia, and lymphadenopathy. AOSD usually has a good prognosis, but it can sometimes be fatal, especially when it is complicated by systemic inflammatory response syndrome (SIRS) and multiple organ failure. Patient concerns: A previously healthy 26-year-old woman was referred to our hospital for persistent high fever and mild systemic edema. Five days later, the patient presented with dyspnea, hypotension, and anuria. Anasarca developed with massive pleural effusion, ascites, and systemic edema, resulting in an increase of 47 kg in body weight. Diagnoses: The patient was diagnosed as AOSD after infection, malignancy, hematologic disorders, and other autoimmune diseases were excluded. Interventions: We administered tocilizumab, an IL-6 receptor inhibitor, intravenously in addition to cyclosporine, prednisolone, plasma exchange, and continuous hemodiafiltration. Outcomes: The patients systemic condition improved. After stabilization by all medications, the patient was managed and responded to tocilizumab alone. To the best of our knowledge, this was the first case of severe SIRS complicating AOSD that was successfully treated with an anti- IL-6 receptor antibody. Lessons: SIRS should not be overlooked in a patient with steroid-resistant AOSD and edema. Inhibitors of the IL-6 receptor can be used safely and effectively to control AOSD complicated with severe SIRS.

Effect of Anemia on Cardiovascular Hemodynamics, Therapeutic Strategy and Clinical Outcomes in Patients With Heart Failure and Hemodynamic Congestion

BACKGROUND We investigated the effect of anemia on cardiovascular hemodynamics, therapeutic strategies and clinical outcomes in heart failure (HF) patients.Methods and Results:We divided 198 consecutive HF patients who underwent right heart catheterization before in-hospital HF treatment into 2 groups according to the presence or absence of hemodynamic congestion (HC: mean pulmonary capillary wedge pressure ≥15 mmHg and/or mean right atrial pressure ≥10 mmHg). The hemoglobin level correlated with the cardiac index (CI) and systemic vascular resistance index (SVRI) (r=-0.34 and 0.42, P<0.05, respectively), and was the strongest contributor of SVRI only in the HC group. Anemic patients more frequently required intravenous inotropic support despite having higher CI and lower SVRI than non-anemic patients in the HC group. The novel hemodynamic subsets based on mean right atrial pressure and estimated left ventricular stroke work index but not Forrester subsets appropriately predicted the need for intravenous inotropic support. The probability of hospitalization for worsening HF during 2-year follow-up period was significantly higher in anemic patients than in non-anemic patients in the HC group. CONCLUSIONS Anemia had a direct effect on cardiovascular hemodynamics and thus can confound therapeutic planning in HF patients with HC. The novel hemodynamic subsets can be applied in daily clinical practice regardless of the presence or absence of anemia.

A Survival Case of Fulminant Right-Side Dominant Eosinophilic Myocarditis

A 59-year-old Japanese woman was admitted to a nearby hospital with dyspnea and general malaise. Transthoracic echocardiography revealed right ventricular (RV) dilatation with severely reduced systolic function and leftward shift of the intraventricular septum. She was initially diagnosed with acute right heart failure, and fell into cardiogenic shock requiring an intra-aortic balloon pump and inotropic agents. An endomyocardial biopsy (EMB) demonstrated extensive interstitial edema, infiltration of inflammatory cells including numerous eosinophils, and myocytolysis with eosinophil degranulation. She was histologically diagnosed with eosinophilic myocarditis. Steroid pulse therapy was initiated, and her hemodynamic status improved along with dramatic recovery of the RV function. EMB 6 days after the initiation of steroid pulse therapy showed the disappearance of infiltration and degranulation of eosinophils, although lymphocytic infiltration still remained. Positron emission tomography-computed tomography (PET/CT) 23 days after steroid pulse therapy showed an increased 18F-FDG uptake in the intraventricular septum and left ventricle, suggesting persistent myocardial inflammation. She was then treated with a maintenance dose of prednisolone. She became free of symptoms and follow-up echocardiography showed normal cardiac function 3 months after the initiation of corticosteroid treatment. In addition, EMB and PET/CT showed no inflammation. This is the first case report of fulminant and right-sided dominant eosinophilic myocarditis successfully treated with corticosteroid.

Fulminant Myocarditis With Prolonged Active Lymphocytic Infiltration After Hemodynamic Recovery

Fulminant myocarditis is a highly mortal syndrome. Meanwhile, the clinical course in surviving patients is generally self-limiting. This is a rare case of fulminant myocarditis with prolonged lymphocytic infiltration after hemodynamic recovery. A 64-year-old man was diagnosed with fulminant myocarditis and required intensive care with veno-arterial extracorporeal membrane oxygenation. Left ventricular function gradually improved but complete atrioventricular block (CAVB) persisted. Follow-up endomyocardial biopsies (EMBs) showed prolonged active infiltration of lymphocytes along with 18F-FDG uptake in 18F-FDG PET/CT until about 70 days after the onset. Therefore, he underwent immunosuppressive therapy for 3 months. Follow-up EMB revealed no evidence of infiltration of lymphocytes and no abnormal 18F-FDG uptake despite irreversible CAVB. Although repeated EMB and 18F-FDG PET/CT was not a standard strategy, it played an important role in the treatment decision in the present case.