Cardiac angiosarcoma with pulmonary and adrenal metastases diagnosed by FDG-PET/CT-guided adrenal biopsy.

Primary cardiac angiosarcoma is a high-grade aggressive tumor with a poor prognosis and low incidence. We describe a case of cardiac angiosarcoma, with pulmonary and adrenal metastases, diagnosed via fluorodeoxyglucose-positron emission tomography/computed tomography-guided adrenal biopsy. Learning objective: Cardiac angiosarcoma should be considered in a patient with a cardiac mass with no tumor cells in the pericardial fluid. Fluorodeoxyglucose-positron emission tomography/computed tomography could be useful in determining the biopsy site.

Borg's exertion scale did not coincide with ventilatory anaerobic threshold in atrial fibrillation patients with restored sinus rhythm after ablation.

The determination of precise exercise intensity is essential for effective exercise rehabilitation. The Borg rating of perceived exertion category ratio (CR) scale is utilized to prescribe an appropriate level of exertion intensity. A Borg CR of approximately 13 coincides with the ventilatory aerobic threshold (VAT). Patients with atrial fibrillation (AF) exhibit various symptoms. We hypothesized that the workload at Borg CR13 (Borg CR13-Watt) differs from the workload at the VAT level (VAT-Watt) in AF patients with restored sinus rhythm (SR) following ablation. Accordingly, the relationship between Borg CR13-Watt and VAT-Watt was studied in patients with restored SR. Cardiopulmonary exercise testing (CPET) was performed at 101±88 days after ablation in 150 patients using a bicycle ergometer. No adverse events were observed during CPET. Borg CR13-Watt was significantly higher than VAT-Watt (67.2±27.8 Watt vs. 54.7±17.6 Watt, P<0.0001). Borg CR13-Watt showed significant linear regression with VAT-Watt (regression coefficient, 0.49, P<0.01; correlation coefficient, 0.80, P<0.01). Higher Borg CR13-Watt was associated with greater differences between Borg CR13-Watt and VAT-Watt (ΔWatt). The Bland-Altman plot showed nonconcordance between the two. Male sex, use of antiarrhythmic drugs, and smoking had contributed to the increased ΔWatt. Duration from ablation to time of CPET did not correlate with ΔWatt. Therefore, Borg CR13-Watt did not coincide with VAT-Watt in patients with restored SR. Higher Borg CR13-Watt was associated with greater ΔWatt. Prescribing exertion intensity as determined solely by perceived exertion is inadequate. CPET is required to determine the precise exercise intensity in AF patients with restored SR after ablation.

A Japanese retrospective study of non-tuberculous mycobacterial infection in children, adolescents, and young adult patients with hematologic-oncologic diseases.

Non-tuberculous mycobacterial infection (NTM) is rare in healthy children, with lymphadenitis being the most common presentation. Immunocompromised populations are known to be at high risk, but the clinical picture of NTM infection in pediatric hematology/oncology patients is unclear. In this nationwide retrospective analysis of patients under the age of 40 treated in Japanese pediatric hematology/oncology departments who developed NTM infection between January 2010 and December 2020, 36 patients (21 patients with hematopoietic stem cell transplantation (HSCT) and 15 nontransplant patients) were identified. Post-transplant patients were infected with NTM at 24 sites, including the lungs (n = 12), skin and soft tissues (n = 6), bloodstream (n = 4), and others (n = 2). Nine of twelve patients with pulmonary NTM infection had a history of pulmonary graft-versus-host disease (GVHD), and rapid-growing mycobacteria (RGM) were isolated from five of them. In nontransplant patients, the primary diseases were acute lymphoblastic leukemia (ALL; n = 5), inborn errors of immunity (IEI; n = 6), and others (n = 4). All cases of ALL had bloodstream infections with RGM, whereas all cases of IEI were infected with slow-growing mycobacteria (SGM). In summary, three typical clinical scenarios for pediatric hematology/oncology patients have been established: RGM-induced pulmonary disease in patients with pulmonary GVHD, RGM bloodstream infection in patients with ALL, and SGM infection in patients with IEI. Our findings suggest that NTM must be regarded as a pathogen for infections in these high-risk patients, especially those with pulmonary GVHD, who may require active screening for NTM.

Clinical Outcomes and Predictors of All-Cause Mortality After Complex High-Risk and Indicated Revascularization Using Percutaneous Coronary Intervention.

The concept of complex and high-risk indicated procedures using percutaneous coronary intervention (CHIP-PCI) has recently been defined. However, few studies have investigated the prognosis of patients after CHIP-PCI. We enrolled 322 consecutive patients who underwent CHIP-PCI. CHIP-PCI was defined as a procedure satisfying at least one criterion each for both patient and procedure characteristics, as follows: patient characteristics [age ≥ 75 years old, low left ventricular ejection fraction (LVEF), diabetes mellitus, acute coronary syndrome, previous coronary artery bypass surgery, peripheral arterial disease, severe chronic kidney disease (CKD), chronic obstructive pulmonary disease (COPD), and severe valvular disease] and procedure characteristics [unprotected left main disease, degenerated saphenous or radial artery grafts, severely calcified lesions, last patent conduit, chronic total occlusions, multivessel disease, and use of mechanical circulatory support]. On Kaplan-Meier analysis, 1-, 2-, and 3-year survival rates following CHIP-PCI was 93.8%, 89.2%, and 85.4%, respectively. Moreover, on Cox multivariate hazard analysis, age (≥ 75 years old) (hazard ratio: 4.01, 95% confidence interval: 1.92-8.38, P < 0.01), COPD (hazard ratio: 2.95, 95% confidence interval: 1.38-6.32, P < 0.01), low LVEF (hazard ratio: 3.35, 95% confidence interval: 1.55-7.22, P < 0.01), severe CKD (hazard ratio: 3.02, 95% confidence interval: 1.44-6.36, P < 0.01), and use of mechanical circulatory support (hazard ratio: 5.97, 95% confidence interval: 2.72-13.10, P < 0.01) remained significant predictors of mortality. In conclusion, we revealed the clinical outcomes after CHIP-PCI. The presence of advanced age, COPD, low LVEF, severe CKD, and mechanical circulatory support use might lead to worse clinical outcomes.

Vedolizumab for children with intestinal graft-versus-host disease: a case report and literature review.

Acute graft-versus-host disease (aGVHD) is a challenging complication of allogeneic hematopoietic stem cell transplantation, and alternative therapies for patients showing inadequate response to steroids are limited. Vedolizumab, an anti-α4ß7 integrin antibody widely used for treating inflammatory bowel diseases, has recently been studied in adult patients with steroid-refractory intestinal aGVHD. However, few studies have examined its safety and effectiveness in pediatric patients with intestinal aGVHD. We report the case of a male patient with intestinal late-onset aGVHD treated with vedolizumab. He underwent allogeneic cord blood transplantation for warts, hypogammaglobulinemia, infections, and myelokathexis (WHIM) syndrome and developed intestinal late-onset aGVHD 31 months after transplantation. The patient was refractory to steroids; however, vedolizumab was initiated 43 months after transplantation (at the age of 7 years) and the symptoms of intestinal aGVHD were alleviated. Additionally, favorable endoscopic findings were observed, such as reduction of erosion and regenerative epithelial growth. We also evaluated the efficacy of vedolizumab in 10 patients with intestinal aGVHD (9 from the literature review and the present case). Six patients (60%) showed an objective response to vedolizumab. No serious adverse events were observed in any patients. Vedolizumab is a potential treatment option for steroid-refractory intestinal aGVHD in pediatric patients.

Cryoballoon ablation for atrial fibrillation without the use of a contrast medium: a combination of the intracardiac echocardiography and pressure wave monitoring guided approach.

In cryoballoon ablation (CBA), a contrast medium is commonly used to confirm balloon occlusion of the pulmonary veins (PVs). However, a contrast medium cannot always be used in patients with renal dysfunction and allergy. The present study aimed to assess the efficacy and safety of CBA without the use of a contrast medium. We retrospectively examined consecutive patients with paroxysmal atrial fibrillation (PAF) who underwent first-time CBA. We compared the procedural results and outcomes in patients for whom a contrast medium was used (contrast group) and those from whom a contrast medium was not used (non-contrast group). In the non-contrast group, we used saline injection on the intracardiac echocardiography and pressure wave monitoring for PV occlusion. Fifty patients (200 PVs) and 22 patients (88 PVs) underwent CBA with and without a contrast medium, respectively. The success rate of PV isolation with CBA alone was 93% and 90% in the non-contrast and contrast groups, respectively (p = 0.40). The fluoroscopy time and nadir temperature were significantly lower in the non-contrast group as compared to that in the contrast group. The recurrence rate 1 year after ablation did not differ between the two groups (18% vs. 18%, p > 0.99). Furthermore, the number of reconnected PVs in patients with recurrence was significantly lower in the non-contrast group than in the contrast group (6% vs. 36%, p = 0.017). In conclusion, CBA using the intracardiac echocardiography and pressure monitoring approach without the use of a contrast medium was safe and efficient.

Earliest pulmonary vein potential-guided cryoballoon ablation is associated with better clinical outcomes than conventional cryoballoon ablation: A result from two randomized clinical studies.

INTRODUCTION: With regard to short-term outcome in atrial fibrillation (AF), the benefit of cryoballoon ablation (CBA) by pressing a balloon against the earliest pulmonary vein (PV) potential site during PV isolation (earliest potential [EP]-guided CBA) has been previously demonstrated. The present study aimed to evaluate the long-term outcome of the EP-guided CBA. METHODS AND RESULTS: This study included 136 patients from two randomized studies, who underwent CBA for paroxysmal AF for the first time. Patients were randomly assigned to the EP-guided and conventional CBA groups in each study. In the EP-guided CBA group, we pressed a balloon against the EP site when the time-to-isolation (TTI) after cryoapplication exceeded 60 and 45 s in the first and second studies, respectively. We compared the clinical outcomes for 1 year after the procedure between the EP-guided CBA group (68 patients) and the conventional CBA group (68 patients). The primary endpoint was the recurrence of atrial arrhythmia after ablation. No significant differences in baseline characteristics were observed between the two groups. Compared with the conventional CBA group, the EP-guided CBA group had a significantly higher success rate at TTI ≤ 90 s (98.5% vs. 90.0%, p < .001); lower touch-up rate and total cryoapplication; and shorter procedure time, and fluoroscopy time. The recurrence at 1 year after ablation was significantly lower in the EP-guided CBA group than in the conventional CBA group (6.0% vs. 19.4%; p = .019). CONCLUSIONS: The EP-guided CBA approach can facilitate the ablation procedure and achieve low recurrence at 1 year after ablation.

Acute pancreatitis following allogeneic hematopoietic stem cell transplantation in children.

Acute pancreatitis (AP) is a potential complication of hematopoietic stem cell transplantation (HSCT), but its incidence and risk factors remain unclear. Thus, we reviewed the cases of 259 consecutive children who received allogeneic HSCT at our institution between January 2000 and December 2017 to determine the incidence and risk factors of AP. Thirteen patients developed AP during a median follow-up period of 4.4 years. The median time from HSCT to AP onset was 80 days (range 29-2426 days), and cumulative incidence (CI) at 4 years was 5.0% [95% confidence interval (95% CI) 2.7-8.3%]. The CI of AP was significantly higher in patients who received bone marrow or peripheral blood stem cells than in those who received cord blood (7.2% versus 0.0% at 4 years, P = 0.02) and was higher in patients who developed grade II-IV acute graft-versus-host disease (GVHD) than in those who did not (31.4% versus 1.4% at 4 years, P < 0.001). Multivariate analysis showed that grade II-IV acute GVHD was an independent risk factor for AP [hazard ratio 15.2 (95% CI 4.1-55.8), P < 0.001] and was strongly associated with post-HSCT AP in children.

Correlation of the peak oxygen consumption and ventilatory aerobic threshold by cardiopulmonary exercise testing with atrial fibrillation recurrences after ablation in patients with paroxysmal atrial fibrillation.

BACKGROUND: The cardiopulmonary function is hypothesized to be associated with atrial fibrillation/atrial tachyarrhythmia (AF/AT) recurrence after AF ablation. PURPOSE: To clarify the relationship between the cardiopulmonary function after successful ablation and AF/AT recurrence. METHODS: We examined 31 patients with paroxysmal AF who underwent AF ablation. Cardiopulmonary exercise testing (CPET) was performed at 1month after the ablation. A continuously increasing loading method on a bicycle ergometer was employed for the CPET. RESULTS: No adverse events, including AF/AT recurrence, occurred during the CPET. Among 31 patients, AT/AF recurrence was observed in seven (23%). The ventilatory anaerobic threshold (VAT) and peak oxygen consumption (VO2) were significantly higher in patients without AF/AT recurrence than in those with AT/AF recurrences (peak VO2 23.6 ± 5.7 vs 17.2 ± 4.1 mL/kg/min; VAT, 16.7 ± 2.8 vs 13.8 ± 2.7 mL/min/kg). The areas under the receiver operating characteristic curve for the peak VO2 and VAT were 0.786 (P < .01) and 0.789(P < .01), respectively. Both indices had a sensitivity of 70%-80% and specificity of 70%-80% for predicting AT/AF recurrence. Similar results were obtained for the percent values of the predicted peak VO2 and VAT. CONCLUSIONS: The present pilot study found that CPET can be performed safely at approximately 1 month after AF ablation. The peak VO2 and VAT were significantly associated with AT/AF recurrence. The peak VO2 and VAT were thought to provide helpful information regarding AT/AF recurrence.