Intracranial residual lesions following early intensification in a patient with T-cell acute lymphoblastic leukemia: a case report.

BACKGROUND: T-cell acute lymphoblastic leukemia (T-ALL) tends to involve central nervous system (CNS) infiltration at diagnosis. However, cases of residual CNS lesions detected at the end of induction and post early intensification have not been recorded in patients with T-ALL. Also, the ratio and prognosis of patients with residual intracranial lesions have not been defined. CASE PRESENTATION: A 9-year-old boy with T-ALL had multiple intracranial tumors, which were still detected post early intensification. To investigate residual CNS lesions, we used 11C-methionine (MET)-positron emission tomography. Negative MET uptake in CNS lesions and excellent MRD status in bone marrow allowed continuing therapies without hematopoietic cell transplantation. CONCLUSIONS: In cases with residual lesions on imaging studies, treatment strategies should be considered by the systemic response, direct assessment of spinal fluid, along with further development of noninvasive imaging methods in CNS. Further retrospective or prospective studies are required to determine the prognosis and frequency of cases with residual intracranial lesions after induction therapy.

A case of cough syncope diagnosed by the Valsalva maneuver and cough induction test under invasive arterial pressure measurement.

Following the loss of consciousness during the Valsalva maneuver and cough induction test, real-time arterial pressure measurement could clarify the significant blood pressure decrease in a patient with cough syncope.

Pacemaker implantation for persistent sinus node dysfunction in a patient with COVID-19.

A 46-year-old woman was admitted with coronavirus disease-2019 infection. Symptomatic sinus bradycardia occurred, followed by congestive heart failure. Therapeutics such as isoproterenol, theophylline, and cilostazol could not safely improve her symptoms. She underwent pacemaker implantation 53 days after admission. Atrial pacing remained was at 60% after 6 months.

Comparison experiments for radon and thoron measuring instruments at low-level concentrations in one room of a Japanese concrete building.

A comparison on commercially available radon (222Rn) measuring instruments (three types of continuous monitors and a passive 222Rn-thoron (220Rn) discriminative alpha track detector) was carried out at low-level concentrations in one room of a concrete building. The agreements between the continuous monitors were within 15%, while the agreements between each instrument were within 20%. It was also observed that the indoor 220Rn concentration measured by the continuous monitor was quite different from those by the passive detectors due to the mean concentration less than the limit of detection of both measuring instruments.

Lesion characteristics between cryoballoon ablation and radiofrequency ablation with a contact force-sensing catheter: Late-gadolinium enhancement magnetic resonance imaging assessment.

BACKGROUND: Pulmonary vein isolation (PVI) lesions after cryoballoon ablation (CBA) are characterized as a wider and more continuous than that after conventional radiofrequency catheter ablation (RFCA) without the contact force (CF)-sensing technology. However, the impact on the lesion characteristics of ablation with a CF-sensing catheter has not been well discussed. We sought to assess the lesions using late-gadolinium enhancement magnetic resonance imaging (LGE-MRI) and to compare the differences between the two groups (CB group vs. RF group). METHODS: A total of 30 consecutive patients who underwent PVI were enrolled (CB group, 18; RF group, 12). The RF applications were delivered with a target lesion size index (LSI) of 5. The PVI lesions were assessed by LGE-MRI 3 months after the PVI. The region around the PV was divided into eight segments: roof, anterior-superior, anterior carina, anterior inferior, bottom, posterior inferior, posterior carina, and posterior superior segment. The lesion width and visual gap of each segment were compared between the two groups. The visual gaps were defined as no-enhancement site of >4 mm. RESULTS: The mean LSI was 4.7 ± 0.7. The lesion width was significantly wider but the visual gaps were more frequently documented at the bottom segment of right PV in the CBA group (lesion width: 8.1 ± 2.2 vs. 6.3 ± 2.2 mm; p = .032; visual gap at the bottom segment or right PV: 39% vs. 0%; p = .016). CONCLUSIONS: The PVI lesion was wider after CBA, while the visual gaps were fewer after RFCA with a CF-sensing catheter.

Different tissue thermodynamics between the 40 W and 20 W radiofrequency power settings under the same ablation index/lesion size index.

INTRODUCTION: The ablation index (AI) and lesion size index (LSI) are novel markers for predicting the ablation lesion quality, however, collateral damage is still a concern. This study aimed to compare the lesion characteristics and tissue temperature profiles between 20 W (20 Ws) and 40 W (40 Ws) ablation settings under the same AI and LSI. METHODS: An ex vivo model consisting of swine myocardium (5-6 mm thickness) in a circulating, warmed saline bath was used. Twenty-one tissue temperature electrodes were used. Radiofrequency applications with different power settings were performed with a 10 to 12 g contact force until the AI and LSI reached 350 and 4.5, respectively. RESULTS: A total of 120 radiofrequency (RF) applications and 2520 tissue temperature profiles were analyzed. The speed of the tissue temperature rise with 40 Ws was significantly faster than that with 20 Ws. However, the maximum tissue temperature did not significantly differ between 20 and 40 Ws with the same AI (44.6°C ± 3.9°C vs 45.1°C ± 6.4°C, P = .73), and was significantly lower for 40 Ws with the same LSI (42.8°C ± 3.4°C vs 40.0°C ± 3.4°C, P = .003). For both the AI and LSI, the number of electrodes exhibiting high temperatures (≥39°C) was significantly larger and the duration of high tissue temperatures was significantly longer with 20 Ws. The thermal latency with 40 Ws was greater. CONCLUSIONS: Although the targeted AI and LSI were the same for both 20 and 40 Ws, the tissue temperature profiles differed greatly depending on the RF power setting. A high power setting based on the AI and LSI may reduce the collateral thermal damage.

Lesion distribution after cryoballoon ablation and hotballoon ablation: Late-gadolinium enhancement magnetic resonance imaging analysis.

INTRODUCTION: Pulmonary vein isolation (PVI) lesions after cryoballoon ablation (CBA) are wide and continuous, however, the distribution can depend on the pulmonary vein (PV) size. We sought to assess the relationship between the lesion distribution and PV size after CBA and hotballoon ablation (HBA). METHODS AND RESULTS: A total of 80 consecutive patients who underwent PVI were enrolled (40 with CBA). The lesions were visualized by late-gadolinium enhancement magnetic resonance imaging. The lesion width, lesion gaps, and distance from the PV ostium (PVos) to distal lesion edge (DLE) were assessed. If the DLE extended inside the PV, the value was expressed as a negative value. Although the lesion width was significantly wider in the CB group (7.8 ± 2.0 vs 4.9 ± 1.0 mm, P < .001), the number of lesion gaps was significantly less in the HB group (2.9 ± 2.4 vs 1.3 ± 1.4 gaps, P = .001). The distance from the PVos to DLE was a negative value in both groups, but the impact was significantly greater (-1.5 ± 1.8 vs -0.2 ± 1.2 mm, P < .001) and negatively correlated with PV size in the CB group, but not in HB group (r = -0.27, P = .007). The AF recurrence 12 months after the procedure did not differ (5 [12.5%] of 40 in the CB group vs 4 [10%] of 40 in the HB group, P = .695). CONCLUSIONS: The PVI lesions after HBA were characterized by (a) narrower, but (b) more continuous, (c) smaller lesion inside the PV, and (d) irrespective of PV size as compared to that after CBA.

Percutaneous Pericardiocentesis With the Anterior Approach: Demonstration of the Precise Course With Computed Tomography.

OBJECTIVES: This study aimed to confirm the precise course of a pericardiocentesis with the anterior approach using post-procedural computed tomography (CT). BACKGROUND: Percutaneous epicardial ventricular tachycardia (VT) ablation has been increasingly performed. Although the inferior approach has been the common method, the feasibility of the anterior approach has subsequently been reported. However, the precise course of the anterior approach has not been presented. METHODS: An epicardial ablation with the anterior approach was performed in 15 patients. At the end of the procedure, the epicardial sheath was exchanged for a drainage tube to monitor bleeding. Of those patients, in 9 procedures in 8 patients a CT scan was performed just after the procedure to confirm the course of the drainage tube and to rule out any complications. Epicardial ablation was indicated for a failed endocardial VT ablation in 7 patients and epicardial substrate modification in 1 patient with Brugada syndrome. RESULTS: Volume-rendered images reconstructed from CT demonstrated each course of the drainage tubes and their relation to the surrounding organs. These images revealed that the tube had a curved trace, and did not penetrate the diaphragm or pass through the abdominal cavity. No injury to the surrounding organs was detected in any of the cases. CONCLUSIONS: The precise course of the drainage tube placed along the trajectory of the anterior approach was able to be confirmed using post-procedural CT images. These images support the safety and feasibility of the anterior approach from the anatomic standpoint with a low incidence of abdominal viscera injury.

Critical exacerbation of idiopathic pulmonary fibrosis after transcatheter aortic valve implantation: Need for multidisciplinary care beyond "heart team".

An 82-year-old man with severe aortic stenosis and idiopathic pulmonary fibrosis (IPF) underwent transcatheter aortic valve implantation (TAVI) under general anesthesia. However, following a successful TAVI procedure, he developed progressive respiratory failure because of the exacerbation of IPF. Despite the use of immunosuppressants, the patient could not be saved and he died of respiratory failure. Although TAVI is a less invasive procedure compared to conventional surgical aortic valve replacement, it is currently selected for management of severely ill, frail, and elderly patients. This case highlights the potential risk of IPF exacerbation following a TAVI procedure performed under general anesthesia. .

A case of acute heart failure due to myocardial infiltration of mycosis fungoides.

Mycosis fungoides (MF) has been reported to be the most common cutaneous lymphoma with a good prognosis and myocardial infiltration is clinically rare. We hereby report a case of rapidly progressing acute heart failure due to myocardial infiltration by MF. Perfusion cardiac magnetic resonance imaging (MRI) demonstrated a massive perfusion defect in the left ventricle (LV) where multiple nodular enhancement areas by delayed enhancement MRI could be documented in the postero-lateral wall of the LV, which resulted in a deterioration of the LV function and mitral regurgitation. Autopsy confirmed the myocardial infiltration by the MF, which corresponded with the MRI findings. .

Serial observation of electrocardiographic responses to corticosteroid therapy in a patient with right ventricular-predominant cardiac sarcoidosis.

Predominant or isolated right ventricular involvement in cardiac sarcoidosis is uncommon, but should always be considered in a case of right ventricular hypertrophy combined with ventricular arrhythmia and/or conduction disturbance. Although improvement in right ventricular hypertrophy and atrioventricular conduction disturbance following corticosteroid therapy has been reported, the detailed serial electrocardiographic responses during corticosteroid therapy, as well as temporal changes in the electrocardiographic, biochemical, and morphological responses, have not been reported. We describe the clinical course and supportive imaging findings of reversible right ventricular hypertrophy and cardiac conduction disturbance in a case of right ventricular-predominant cardiac sarcoidosis.

The lesion characteristics assessed by LGE-MRI after the cryoballoon ablation and conventional radiofrequency ablation.

Background: Rhythm outcomes after the pulmonary vein isolation (PVI) using the cryoballoon (CB) are reported to be excellent. However, the lesions after CB ablation have not been well discussed. We sought to characterize and compare the lesion formation after CB ablation with that after radiofrequency (RF) ablation. Methods: A total of 42 consecutive patients who underwent PVI were enrolled (29 in the CB group and 13 in the RF group). The PVI lesions were assessed by late gadolinium enhancement magnetic resonance imaging 1-3 months after the PVI. The region around the PVs was divided into eight segments: roof, anterior-superior, anterior-carina, anterior-inferior, bottom, posterior-inferior, posterior-carina, and posterior-superior segment. The lesion width and lesion gap in each segment were compared between the two groups. Lesion gaps were defined as no-enhancement sites of >4 mm. Results: As compared to the RF group, the overall lesion width was significantly wider and lesion gaps significantly fewer at the anterior-superior segment of the left PV (LAS) and anterior-inferior segment of the right PV (RAI) in the CB group (lesion width: 8.2 ± 2.2 mm vs 5.6 ± 2.0 mm, P = .001; lesion gap at LAS: 7% vs 38%, P = .02; lesion gap at RAI: 7% vs 46%, P = .006). Conclusions: The PVI lesions after CB ablation were characterized by extremely wider and more continuous lesions than those after RF ablation.

Left ventricular outflow tract obstruction caused by massive mitral annular calcification in a patient with hypertensive heart disease.

Mitral annular calcification (MAC) is frequently observed, but it rarely causes left ventricular outflow tract (LVOT) obstruction (LVOTO). An 83-year-old woman with hypertension, diabetes, and dyslipidemia was admitted to our hospital because of exertional dyspnea. She was diagnosed with hypertensive heart disease. Her symptoms were exacerbated by exertion, and she had no symptoms at rest. Transthoracic echocardiography showed massive posterior MAC, a sigmoid septum, and LVOTO, with a peak gradient of 15.4 mmHg at rest. Systolic anterior motion of the anterior mitral leaflet was not found. Moreover, the LVOT gradient in the stress condition was evaluated, and an increased LVOT gradient (47.3 mmHg) and chest discomfort was noted after 20 µg/kg/min of dobutamine was administered and the Valsalva maneuver was used. Hence, the patient was diagnosed with latent LVOTO. Interestingly, the distance between the septal wall, which was protruding into the left ventricular cavity, and the mitral valve coaptation, which was pushed up by the posterior MAC, had become closer, causing dynamic LVOTO. Since it is difficult to treat LVOTO with medication, ultimately, septal myectomy and mitral valve replacement were performed, which improved her symptoms. Evaluating the LVOT pressure gradient in stress condition is important in patients with MAC. .

Evaluation of platelet reactivity using P2Y12 reaction units in acute coronary syndrome with essential thrombocythemia: A case report.

Essential thrombocythemia (ET) has been reported to cause acute coronary disease. However, the efficacy of anti-platelet therapy for ET is unclear since there are individual differences in the platelet function of ET patients. Here we report a case of a 62-year-old man with ET who was admitted to our hospital because of acute coronary syndrome. He underwent coronary angioplasty. Dual anti-platelet therapy with aspirin (81 mg/day) and clopidogrel (75 mg/day) was subsequently initiated. We evaluated platelet reactivity in P2Y12 reaction units, and subsequently determined anti-platelet drugs and corresponding doses. .

[Thrombopoietin receptor agonists administration for acute exacerbation of chronic idiopathic thrombocytopenic purpura and subsequent anticoagulant therapy for accompanying deep venous thrombosis of the lower limbs].

We report two patients (70- and 49-year-old Japanese men) with acute exacerbation of chronic idiopathic thrombocytopenic purpura (ITP) and deep venous thrombosis of the lower extremities. Both were successfully managed with thrombopoietin receptor agonist (TPO-RA) administration. Both had ITP refractory to steroid treatment. Their immature platelet fraction (absolute-IPF) counts were increased and paralleled the platelet recoveries after TPO-RA (eltrombopag and romiplostim, respectively) without progression of thrombosis. Although ITP has recently been evaluated as a thrombophilic disorder, reports on acute exacerbation of ITP with newly diagnosed thrombosis are limited, and the pathophysiology and association between ITP and thrombosis remain to be elucidated. Moreover, the influences of TPO-RA on thrombosis are still controversial. To our knowledge, this is the first case report describing patients with exacerbation of ITP who developed thrombosis and were treated with TPO-RA. The outcomes of our cases underscore the importance of monitoring thrombosis and not delaying the initiation of anticoagulation treatment during the use of TPO-RA.

Granulomatosis with polyangiitis presenting as an orbital inflammatory pseudotumor: a case report.

INTRODUCTION: Granulomatosis with polyangiitis is a systemic inflammatory disease that often presents with necrosis, granuloma formation and vasculitis of small- to medium-sized vessels. Affected patients usually present with disease of the upper respiratory tract, lungs and kidneys, but this disease has been reported to involve almost any organ. We report the case of a patient with ocular manifestations of granulomatosis with polyangiitis after the remission of renal and auditory manifestations. CASE PRESENTATION: An 81-year-old Japanese woman had a four-year history of biopsy-proven antineutrophil cytoplasmic antibody-related glomerulonephritis that had been treated with oral prednisolone and was in serological remission. She had also recovered from a one-year history of complete hearing loss immediately following the steroid treatment for glomerulonephritis. She gradually experienced right eye visual disturbance and exophthalmos over a two-month period. Radiographic and histopathological findings revealed an orbital inflammatory pseudotumor. The administration of prednisolone completely restored her right eye visual acuity and eye movement after two weeks. Considering this case retrospectively, our patient had an orbital inflammatory pseudotumor caused by granulomatosis with polyangiitis including a medical history of reversible hearing loss, although her glomerulonephritis had remitted with an undetectable level of specific antineutrophil cytoplasmic antibody. CONCLUSIONS: In this patient, hearing loss and visual loss occurred at different times during the course of treatment of granulomatosis with polyangiitis. Clinicians should consider a differential diagnosis of granulomatosis with polyangiitis in patients with treatable hearing and visual loss.

FGD5 mediates proangiogenic action of vascular endothelial growth factor in human vascular endothelial cells.

OBJECTIVE: Vascular endothelial growth factor (VEGF) exerts proangiogenic action and induces activation of a variety of proangiogenic signaling pathways, including the Rho family small G proteins. However, regulators of the Rho family small G proteins in vascular endothelial cells (ECs) are poorly understood. Here we attempted to clarify the expression, subcellular localization, downstream effectors, and proangiogenic role of FGD5, a member of the FGD family of guanine nucleotide exchange factors. METHODS AND RESULTS: FGD5 was shown to be selectively expressed in cultured human vascular ECs. Immunofluorescence microscopy showed that the signal for FGD5 was observed at peripheral membrane ruffles and perinuclear regions in human umbilical vein ECs. Overexpression of FGD5 increased Cdc42 activity, whereas knockdown of FGD5 by small interfering RNAs inhibited the VEGF-induced activation of Cdc42 and extracellular signal-regulated kinase. VEGF-promoted capillary-like network formation, permeability, directional movement, and proliferation of human umbilical vein ECs and the reorientation of the Golgi complex during directional cell movement were attenuated by knockdown of FGD5. CONCLUSIONS: This study provides the first demonstration of expression, subcellular localization, and function of FGD5 in vascular ECs. The results suggest that FGD5 regulates proangiogenic action of VEGF in vascular ECs, including network formation, permeability, directional movement, and proliferation.