Successful Treatment with Mepolizumab for Coronary Spastic Angina Associated with Eosinophilic Granulomatosis with Polyangiitis.

A 46-year-old man with a history of bronchial asthma and chronic sinusitis presented to our hospital with chest pain. We suspected angina evoked by epicardial coronary spasm and performed an ergonovine provocation test to diagnose coronary spastic angina (CSA). The patient also met the diagnostic criteria for eosinophilic granulomatosis with polyangiitis (EGPA) and was treated with 60 mg prednisolone (PSL) for EGPA-associated CSA. After PSL administration, eosinophils decreased, and angina attacks disappeared. However, when PSL was tapered to 12.5 mg, chest pain recurred. We administered mepolizumab subcutaneously and chest pain disappeared. Additional mepolizumab may be effective for EGPA with CSA.

Low incidence of restenosis after successful balloon pulmonary angioplasty in patients with chronic thromboembolic pulmonary hypertension.

Balloon pulmonary angioplasty (BPA) is now a treatment option for patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). However, the incidence of restenosis and long-term changes in vessel diameters in pulmonary arteries after BPA are unknown. The present study investigated the incidence of restenosis by measuring changes in vessel diameter after BPA. We reviewed 58 patients (168 lesions) with CTEPH who underwent single dilation for the target lesion (type A/B/C lesions) during BPA procedure followed by selective pulmonary angiography more than 6 months after the final BPA procedure. The outcomes of BPA were assessed in terms of pulmonary artery diameters. In a median follow-up of 1.9 (1.2-2.7) years, restenosis occurred in only one case with a type C lesion after BPA (0.6%). In type A/B lesions, the minimal lumen diameter was significantly enlarged at follow-up after BPA [3.48 (2.59-4.34) to 4.22 (3.31-4.90) mm]. In type C lesions, the minimal lumen diameter was unchanged at follow-up after BPA [3.15 (1.96-3.64) to 3.28 (2.38-4.61) mm]. The present results revealed that restenosis after BPA rarely occurs in type A/B/C lesions. Minimal lumen diameters for type A/B lesions continually increased and those for type C lesions did not decrease. Stent implantation in type A/B/C lesions would be unnecessary after BPA.

Oxygen inhalation can selectively dilate pulmonary arteries in patients with chronic thromboembolic pulmonary hypertension before balloon angioplasty.

BACKGROUND: Pulmonary injury is a major complication of balloon pulmonary angioplasty (BPA) for chronic thromboembolic pulmonary hypertension (CTEPH). Lung injury after BPA can be exacerbated by a high mean pulmonary arterial pressure (PAP). Although oxygen inhalation is expected to lower mean PAP in patients with CTEPH, no relevant investigation has been conducted. METHODS: Consecutive patients with CTEPH who underwent BPA were enrolled in this study. We evaluated the hemodynamics using right heart catheterization while breathing ambient air and with administration of 5 L/min oxygen for 10 min. RESULTS: This study included 52 consecutive patients with CTEPH, of whom 23 (44%) were treated with specific pulmonary vasodilators. Exposure to oxygen was well tolerated. Oxygen administration significantly decreased mean PAP by 3.8 ± 3.2 mmHg (p<0.001) and pulmonary vascular resistance by 0.8 ± 1.8 Wood units (p<0.001). Moreover, the ratio of pulmonary vascular resistance to systemic vascular resistance was significantly reduced by 13.5% (p<0.001). Multivariate regression analysis identified baseline mean PAP (ß = -0.427, p = 0.006) as the only significant predictor of decreased mean PAP under oxygen administration. No significant difference in oxygen effect on mean PAP was found between patients with and without vasodilators. CONCLUSIONS: In patients with CTEPH, 5 L/min supplemental oxygen inhalation could decrease mean PAP significantly by selective pulmonary artery dilatation, regardless of the usage of vasodilators, and thus could be helpful to maximize the safety of BPA. CLINICAL TRIAL REGISTRATION: UMIN Clinical Trials Registry (No.: UMIN000026882); URL: https://www.umin.ac.jp/ctr/index.htm.

The impairment of endocardial radial strain is related to aortic stenosis severity in patients with aortic stenosis and preserved LV ejection fraction using two-dimensional speckle tracking echocardiography.

BACKGROUND: Myocardial function is heterogeneous in different myocardial layers. Recently, two-dimensional speckle tracking echocardiography has been used to define myocardial deformation parameters of the left ventricular (LV) segment. This study aimed to investigate strain in subendocardial and subepicardial layers in patients with aortic stenosis (AS) and preserved LV ejection fraction (LVEF) using speckle tracking echocardiography. METHODS: Parasternal short-axis and apical long-axis views of the left ventricle were acquired at the mid-papillary level in 35 control subjects and 32 patients with AS and preserved LVEF. Radial, circumferential, and longitudinal strain in subendocardial and subepicardial layers at the posterior and anteroseptal segments were calculated. RESULTS: There was no significant difference in circumferential strain in subendocardial and subepicardial layers between the control subjects and the patients with AS. Similarly, there was no significant difference in epicardial radial strain at the posterior and anteroseptal segments between the control subjects and the patients with AS. Longitudinal strain at both the posterior and anteroseptal segments was significantly decreased in the AS group compared with that in the control group. AS patients had significantly decreased values of endocardial radial strain compared with those in controls (anteroseptal: 18.2 ± 11.2 vs. 34.5 ± 14.8, P < 0.005; posterior: 25.2 ± 14.8 vs. 32.6 ± 12.6, P < 0.05). In the AS group, endocardial radial strain in the posterior and anteroseptal segments was significantly correlated with the aortic valve area (posterior: r = 0.41, P < 0.05; anteroseptal: r = 0.33, P < 0.05). CONCLUSION: Patients with AS and preserved LVEF have impaired longitudinal strain and endocardial radial strain, although circumferential strain and epicardial radial strain are preserved. Despite preserved LVEF, endocardial radial strain was associated with AS severity.

Comparison of two-dimensional and real-time three-dimensional transesophageal echocardiography in the assessment of aortic valve area.

BACKGROUND: The accuracy of two-dimensional transesophageal echocardiography (2D-TEE) for the measurement of aortic valve area (AVA) in patients with aortic stenosis (AS) depends upon the cross-section selected for imaging. Real-time three-dimensional transesophageal echocardiography (3D-TEE) may overcome this limitation of 2D-TEE. The goal of this study was to compare 3D-TEE with 2D-TEE for the measurement of AVA. METHODS AND RESULTS: Twenty-five patients with AS underwent TEE. In 2D-TEE, the aortic valve image was obtained at the orifice level in the short-axis view, and AVA was measured by planimetry of the acquired images (2D-AVA). In 3D-TEE, 3D data containing the entire aortic valve were obtained. Then, a short-axis cross-section containing the smallest orifice in mid-systole was cut from the 3D data during image postprocessing, and the AVA was measured by planimetry (3D-AVA). The 3D-AVA was significantly smaller than the 2D-AVA (0.79±0.35cm(2) vs. 0.93±0.40cm(2), p<0.0001), but there was a strong correlation between 3D-AVA and 2D-AVA (R=0.94). Although the frame rate was lower in 3D-TEE than in 2D-TEE (17±6Hz vs. 58±16Hz), the 3D-AVA determined at each frame during systole showed that the difference between 3D-AVA and 2D-AVA was not explained by the lower frame rate. The time required for image acquisition of the aortic valve was shorter with 3D-TEE than with 2D-TEE (p=0.0005). CONCLUSIONS: The geometric AVA is smaller with 3D-TEE than with 2D-TEE, and the difference is not due to the lower frame rate of 3D-TEE. The improved accuracy of 3D-TEE along with reduced image acquisition time indicates that 3D-TEE is superior to 2D-TEE for the assessment of AVA.