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Background: Fulminant myocarditis presentation (FMP) is a rare and severe presentation of myocarditis. The natural history of FMP and its clinical features associated with poor outcomes are incompletely understood because there is a lack of generalizable evidence. Methods: This multicenter retrospective cohort study included patients hospitalized with histologically proven myocarditis who underwent catecholamine or mechanical support from 235 cardiovascular training hospitals across Japan between April 2012 and March 2017. Clinical features and the prognostic predictors of death or heart transplantation within 90 days on the basis of clinical and pathologic findings were determined using the Kaplan-Meier method, log-rank test, and Cox regression analysis. Results: This study included 344 patients with histologically proven FMP (median age, 54 years; 40% female). The median follow-up was 600 days (interquartile range, 36 to 1599 days) and the cumulative risk of death or heart transplantation at 90 days was 29% (n=98). Results from multivariable Cox regression analysis showed that older age, nonsinus rhythm, low left ventricular wall motion ( Conclusions: The results from analyses of data from this multicenter registry demonstrated that patients with FMP are at a higher risk of death or heart transplantation in real-world settings. These observations inform which clinical and pathologic findings may be useful for prognostication in FMP. Registration: URL: https://www.umin.ac.jp/ctr ; Unique identifier: UMIN000039763.

Background Pheochromocytoma is a rare tumour producing catecholamines and has been more frequently reported than expected in patients with a Fontan physiology. Case summary The patient was a 39-year-old woman born with a univentricular atrioventricular connection and pulmonary valve stenosis. A Fontan operation was performed when she was 22 years old. At the age of 38, she was diagnosed with pheochromocytoma. The most serious problem was the increased occurrence of supraventricular arrhythmias, which easily caused heart failure. We decided to perform a laparoscopic resection. Postoperative recovery was good. There were no findings of malignancy and all postoperative catecholamine levels normalized. She was discharged home on the 14th day after the surgery, walking unaided. Discussion Diagnosis and treatment by tumour resection in the early phase are crucial in patients who undergo the Fontan procedure. However, patients after Fontan surgery have a very low cardiac reserve. Thus, the decision to perform a surgical treatment is a very difficult one to take. In this case, fenestration had been naturally closed. As fenestration provides a consistent source of systemic ventricular preload, we performed transcatheter fenestration dilatation to improve heart failure management before surgical pheochromocytoma removal. Although laparoscopic surgery is generally considered to be less invasive, pneumoperitoneum may interfere with venous return in Fontan physiology patients. There is also a risk of thrombosis via fenestration. Here, we reported the case of a successful laparoscopic pheochromocytoma resection in an adult Fontan patient.

Papillary muscle rupture is a fatal complication with a high operative mortality. Most patients experience cardiogenic shock and hypoxia due to pulmonary edema caused by severe mitral regurgitation. Although preoperative stabilization using a mechanical assist device potentially improves surgical outcomes, an appropriate strategy has not yet been established. ECPELLA, combining venoarterial extracorporeal membrane oxygenation and Impella, has the potential to stabilize preoperative status and improve outcome in patients with refractory cardiogenic shock due to papillary muscle rupture. Herein, we present 3 cases involving the efficacy of ECPELLA and our tips of surgical and ECPELLA management in patients with papillary muscle rupture.

A 31‐year‐old female with 10 years indwelled Hickman catheter for idiopathic pulmonary hypertension presented infectious findings. We decided to remove it but simple traction did not work. Although it was an off labeled use, we could remove it using a laser sheath and snare technique without any complication.

Abnormal liver function test results are often observed in acute decompensated heart failure (ADHF). However, the prognostic value of bilirubin fractionation has not been elucidated. The prognostic value of direct bilirubin (DB), in comparison with total bilirubin (TB), was examined in 556 consecutive patients with ADHF. Patients with elevated DB showed mostly similar patient characteristics including signs of elevated right-sided pressure (frequent hepatomegaly, jugular venous distention, dilated inferior vena cava, and elevated gamma-glutamyltransferase) and decreased cardiac output (cold extremities, decreased pulse pressure, and lower blood pressure) and other parameters of heart failure (HF) severity (increased plasma renin activity, decreased sodium, total cholesterol, and ejection fraction) to elevated TB; however, only patients with elevated DB showed a significant difference in the frequency of HF history and alkaline phosphatase value. Kaplan-Meier analysis showed that patients with elevated DB had a significantly higher rate of the composite end point of all-cause mortality or HF readmission (p = 0.021) compared with those with normal DB, whereas patients with elevated TB did not show a statistically significant difference compared with those with normal TB (NS). A multivariate Cox hazards model showed that DB was an independent predictor of adverse events (adjusted hazard ratio 1.052, 95% confidence interval 1.001 to 1.099, p = 0.034), whereas TB was not (adjusted hazard ratio 1.017, 95% confidence interval 0.985 to 1.046, p = 0.27). Adding DB to existing prognostic variables resulted in higher C-statistics than adding TB (C-statistics: 0.670 to 0.675, 0.670 to 0.674, respectively). In conclusion, elevated DB in ADHF was an independent prognostic predictor that was superior to TB. DB may be useful for further risk stratification in ADHF.

BACKGROUND: Elevation of serum erythropoietin (EPO) level is frequently observed in heart failure (HF) patients, and higher serum EPO level has been reported to be associated with worse clinical outcome in chronic HF. However, serum EPO level was highly influenced by the presence of anemia and its prognostic significance in acute HF (AHF) patients without anemia remains unknown. METHODS: We examined 320 consecutive AHF patients who admitted to our institution between January 2013 and January 2014 from prospective registry. Patients who had anemia defined as a hemoglobin concentration < 13g/dl (males) and RESULTS: Adverse events were occurred in 10 patients (11 %). Higher EPO group had significantly higher incidence of adverse events compared with lower EPO group (19.6% vs 10.9%, P CONCLUSIONS: Higher serum EPO level might be a predictor for worse short-term clinical outcome in AHF patients without anemia.