Pediatric frequent relapsing nephrotic syndrome with multiple cerebral infarctions accompanied by patent foramen ovale and cerebral venous sinus thrombosis: a case report.

BACKGROUND: Idiopathic nephrotic syndrome (NS) presents as a hypercoagulable state, of which thromboembolism (TE) is a well-known life-threatening complication. Although TE is more likely to occur in venous vessels than arterial vessels, arterial TE is important because it may cause after-effects, including tissue necrosis and cerebral infarction (CI); therefore, prompt diagnosis and appropriate treatment are required. We report a pediatric NS case with multiple CIs. CASE PRESENTATION: A 14-year-7-month-old Japanese girl was diagnosed with frequent relapsing NS, accompanied by headache and disturbance of consciousness during the second relapse. Brain magnetic resonance imaging (MRI) and four-dimensional computed tomography revealed multiple CIs, vasogenic edema, and cerebral venous sinus thrombosis (CVST). The patient had no underlying thrombophilia other than hypercoagulability due to NS and prednisolone (PSL), and no cardiac arrhythmia; however, a right-to-left shunt through the patent foramen ovale (PFO) was observed with the Valsalva maneuver by echocardiography. Therefore, we assumed that a potential cause of multiple CIs might be an embolic stroke, caused by thrombosis formed from a hypercoagulable state due to NS and PSL treatment and reached through PFO. Antiplatelet and anticoagulant therapies were administered for TE. She was treated with PSL and mycophenolate mofetil (MMF) for NS. Rituximab (RTX) was administered to prevent NS relapse after complete remission (CR). She underwent transcatheter PFO closure at age 14 years and 9 months because we considered that the right-to-left shunt through the PFO would be one of the risks for recurrent cerebral embolism when NS relapses. One year after the onset of CIs, an MRI indicated that the CVST had resolved, leaving no neurological sequelae due to CI; therefore, anticoagulant therapy was discontinued. And then she has been in CR for NS with only MMF therapy. CONCLUSIONS: CI is a serious complication in patients with NS. The pathogenesis of multiple CIs is various, including right-to-left shunt through PFO, in addition to the hypercoagulability due to NS. It is important to investigate and manage underlying risks such as PFO, besides preventing the relapses of NS by aggressive treatments using MMF and RTX, in patients with NS.

The Future of Paediatric Heart Interventions: Where Will We Be in 2030?

PURPOSE OF REVIEW: Cardiac catheterization therapies to treat or palliate infants, children and adults with congenital heart disease have developed rapidly worldwide in both technical innovation and device development in the previous three decades. By reviewing of current status of novel or development of devices and techniques, we will discuss what is likely to happen in paediatric heart intervention in the next decade. RECENT FINDINGS: Recently, biodegradable stents and devices, transcatheter pulmonary valve implantation for the native right ventricle outflow tract and MRI-guided interventions have been progressing rapidly with good immediate to early results. These are expected to be introduced and spread in the next decade although there are still challenges to overcome. The future of paediatric heart intervention is very promising with rapid development of technological progress.

Good response to tolvaptan shortens hospitalization in patients with congestive heart failure.

Tolvaptan has been gradually spread to use as a potent diuretic for congestive heart failure in the limited country. However, the response to this aquaretic drug still is unpredictable. A total of 92 patients urgently hospitalized due to congestive heart failure and treated with tolvaptan in addition to standard treatment was retrospectively analyzed. Responder of tolvaptan treatment was defined as a patient with peak negative fluid balance greater than 500 mL/day, and clinical profiles were compared between 76 responders and 16 non-responders. Responders started to increase daily urine volume (UV) from Day 1 through Day 3. In contrast, non-responders showed no significant increase in daily UV from the baseline up to Day 5. Time between admission and tolvaptan administration was shorter in responders, even without statistical significance (3.3 vs. 4.6 days, p = 0.053). Multivariate analysis revealed that blood urea nitrogen (BUN) [cutoff: 34 mg/dL, odds ratio (OR) 9.0, 95% confidence interval (CI) 1.42-57.3, p < 0.01] and plasma renin activity (PRA) (cutoff: 4.7 ng/mL/h, OR 6.1, 95% CI 1.01-36.4, p < 0.01) at baseline were independent predictors for tolvaptan responsiveness. It suggests that renal perfusion may affect tolvaptan-induced UV. Finally, durations of stay in intensive care unit and total hospitalization were significantly shorter in responders (median: 6.0 vs. 13.0 days, p = 0.022; 15.0 vs. 25.0 days, p = 0.016, respectively). Responders of tolvaptan have lower BUN and renin activity at baseline, and shorten hospitalization period. Trial Registration The study was registered at University Hospital Medical Information Network Clinical Trials Registry (UMIN-CTR) with the identifier UMIN000023594. https://upload.umin.ac.jp/cgi-open-bin/ctr_e/ctr_view.cgi?recptno=R000024988.

Correction to: Good response to tolvaptan shortens hospitalization in patients with congestive heart failure.

In the original publication of the article, the values of blood urea nitrogen (BUN) and plasma renin activity (PRA) have been published incorrectly and the corrected values are as follows.

Minimum-incision trans-subclavian transcatheter aortic valve replacement with regional anesthesia.

BACKGROUND: Minimum-incision trans-subclavian transcatheter aortic valve replacement (MITS-TAVR) is usually performed in patients who are contraindicated for transfemoral TAVR, under regional anesthesia (RA). This study aimed to evaluate the safety and efficacy of MITS-TAVR under RA compared to MITS-TAVR under general anesthesia (GA). METHODS: This single-center observational study included 44 consecutive patients who underwent MITS-TAVR under RA (RA group, n = 19) and GA (GA group, n = 25). RA was achieved using an ultrasound-guided nerve block. RESULTS: The rates of respiratory disease (RA vs. GA, 36.8 % vs. 4.0 %; p < 0.01) and dialysis (79.0 % vs. 0 %; p < 0.01) were significantly higher in the RA group. STS score was significantly higher in the RA group (RA vs. GA, 10.8 ±â€¯1.06 % vs. 7.87 ±â€¯0.93 %; p < 0.01). Both groups had a 100 % procedural success rate. The two groups showed comparable operation room stay times (RA vs. GA, 160 ±â€¯6.96 min vs. 148 ±â€¯5.90 min; p = 0.058). The mean rate of change in blood pressure, used as an index of hemodynamic stability, was significantly lower in the RA group (RA vs. GA, 19.0 ±â€¯3.4 % vs. 35.5 ±â€¯3.0 %; p < 0.01). No in-hospital deaths occurred in either group. One case of minor dissection occurred in the GA group (RA vs.GA, 0 % vs. 4.0 %, p = 0.378). The intensive care unit stay (RA vs. GA, 0.21 ±â€¯0.11 days vs. 1.24 ±â€¯0.10 days; p < 0.01) and hospital stay (RA vs. GA, 7.00 ±â€¯1.73 days vs. 12.2 ±â€¯1.44 days; p < 0.01) were significantly shorter in the RA group. CONCLUSIONS: MITS-TAVR under RA is safe and effective and might be a promising alternative approach. It could ensure intraoperative hemodynamic stability and shorten intensive care unit and hospital stays.

Melody Valve Fracture Causing Mitral Stenosis: Novel Solution for Transapical Valve-in-Valve.

The use of Melody valves in the mitral position has been introduced in clinical practice. Stent fracture is a recognized complication of Melody valve implantation in the pulmonary position; however, reports in the mitral position are rare. We present the case of an 8-year-old boy in whom complete fracture of the proximal stent struts occurred, causing acute severe mitral stenosis, and in whom urgent hybrid transapical Melody valve implantation in the fractured Melody valve was performed successfully using a novel modified technique. This modification simplified the implantation, led to reduced time of the procedure, and minimized hemodynamic instability.

Clinical characteristics of hospitalized heart failure patients with preserved, mid-range, and reduced ejection fractions in Japan.

AIMS: There are regional differences in the patient characteristics, management, and outcomes of hospitalized patients with heart failure (HF). The aim of this study was to evaluate the clinical characteristics and outcomes of Japanese patients who are hospitalized with HF on the basis of the left ventricular ejection fraction (LVEF) stratum. METHODS AND RESULTS: We retrospectively conducted a multicentre cohort study of 1245 hospitalized patients with decompensated HF between 2013 and 2014. Of these patients, 36% had an LVEF < 40% [HF with reduced ejection fraction (HFrEF), median age 72 years, 71% male], 21% had an LVEF 40-49% [HF with mid-range EF (HFmrEF), 77 years, 56% male], and 43% had an LVEF ≥ 50% [HF with preserved EF (HFpEF), 81 years, 44% male]. The primary outcome was death from any cause, and the secondary outcomes were cardiac death and re-hospitalization due to worsened HF after hospital discharge. There were high proportions of non-ischaemic cardiomyopathy (32%) in HFrEF patients, coronary artery disease (44%) in HFmrEF patients, and valvular disease (39%) in HFpEF patients. The frequencies of intravenous diuretic and natriuretic peptide administration during hospitalization were 66% and 30%, respectively. The median hospital stay for the overall population was 19 days, and the length of stay was >7 days for >90% of patients. In-hospital mortality was 7%, but was not different among the LVEF groups (HFrEF 7%, HFmrEF 6%, and HFpEF 8%). After a median follow-up of 19 months (range, 3-26 months), 192 (17%) of the 1156 patients who were discharged alive died, and 534 (46%) were re-hospitalized after hospital discharge. There were no significant differences in mortality after hospital discharge among the three LVEF groups (HFrEF 18%, HFmrEF 16%, and HFpEF 16%). There were no differences in cardiac death or re-hospitalization due to worsened HF after hospital discharge among the LVEF groups (cardiac death: HFrEF 8%, HFmrEF 7%, and HFpEF 7%; re-hospitalization due to worsened HF: HFrEF 19%, HFmrEF 16%, and HFpEF 17%). Multivariable-adjusted analyses showed that the HFmrEF and HFrEF groups, compared with the HFpEF group, were not associated with an increased risk for in-hospital death or death after hospital discharge. Non-cardiac causes of death and re-hospitalization after hospital discharge accounted for 35% and 38%, respectively. CONCLUSIONS: Our results revealed different clinical characteristics but similar mortality rates in the HFrEF, HFmrEF, and HFpEF groups. The most common cause of death and re-hospitalization after hospital discharge was HF, but non-cardiac causes also contributed to their prognosis. Integrated management approaches will be required for HF patients.

Inadvertent consequences of percutaneous coronary intervention to treat unstable spontaneous coronary artery dissection.

We present two cases of spontaneous coronary artery dissection (SCAD), which were diagnosed and treated with emergent percutaneous coronary intervention (PCI). Patients with ongoing ischemia due to SCAD need emergent coronary revascularization with PCI or coronary artery bypass grafting. We discuss the difficulties of PCI to bail out unstable SCAD regardless of the modern techniques and modalities. Brief reviews of the literature with relevance are included. .