SummaryImmunoglobulin4 (IgG4)-related disease is a systemic inflammatory disease characterized by elevation of serum IgG4. It involves various organs such as the pancreas (autoimmune pancreatitis), lacrimal gland (Mikulicz's disease), retroperitoneum (retroperitoneal fibrosis), aorta (aortic aneurysm and aortitis), heart (constrictive pericarditis), and pseudotumors around the coronary arteries. These disorders often coexist in accordance with progression of the disease. Because IgG4-related cardiovascular disorder affects the patient's prognosis, early detection and treatment is important. Coronary CT imaging and echocardiography accidentally detect IgG4-related disorders and 18 FDG-PET imaging can identify active inflammation in the lesions. Measurement of serum IgG4 levels and tissue biopsy are necessary for diagnosis. Minor salivary gland biopsy is recommended even though 18 FDG uptake is not detected when it is difficult to obtain a biopsy specimen from IgG4-related cardiovascular lesions. The first-line treatment is high-dose corticosteroid therapy, however, relapse is often reported. Corticosteroids suppress the development of active inflammatory diseases such as aortitis, pericarditis, and pseudotumors, but already-developed lesions do not respond. A large developed aneurysm can rupture even during or after corticosteroid therapy, therefore, additional surgical treatment may be needed. Treatment of IgG4-related cardiovascular disorders might require higher doses of corticosteroids than IgG4-related extracardiovascular disorders. The adequate dose of corticosteroid, type and dose of immunosuppressant, and surgical intervention should be carefully considered on a case-by-case basis. (Int Heart J 2014; 55: 287-295)

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Right ventricular (RV) function is a prognostic factor in ischemic heart disease (IHD) patients, although its correlations with exercise capacity and cardiac rehabilitation (CR) efficacy are unknown. We aimed to clarify how RV function was associated with exercise tolerance and efficacy of phase II CR in IHD patients. We retrospectively analyzed 301 consecutive IHD patients who underwent phase II CR. We defined RV dysfunction using a combination of RV fractional area change < 35%, tricuspid annular plane systolic excursion < 1.6 cm, and systolic velocity < 10 cm/s. Exercise capacity was assessed using cardiopulmonary testing. The relation between RV function and exercise capacity was analyzed. The all-cause death and major adverse cardiac events (MACE) were evaluated by survival curve. The RV dysfunction group (n = 121) showed impaired left ventricular (LV) systolic and diastolic function before CR contrary to the normal RV function group (n = 180). The presence of RV dysfunction significantly reduced %AT by 4% and %Peak[Formula: see text] by 9% before CR, but increases the degree of improvement in %Peak[Formula: see text] with CR, independent of LV systolic and diastolic function. Univariate analysis demonstrated that previous coronary artery bypass grafting (CABG) was negatively associated with all-cause deaths and MACE. Adjusted for previous CABG, poor prognosis correlated with coexisting LV and RV dysfunction (hazard ratio [HR] 3.91, 95% confidence interval [CI] 1.13-13.53, P = 0.03) and RV dysfunction alone (HR 3.08, 95% CI 1.01-9.37, P = 0.05). In IHD patients, RV dysfunction is associated with exercise intolerance before CR and increased MACE risk, independent of LV function. The CR was effective in patients with RV dysfunction.