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Background Cardiosphere‐derived cell (CDC) infusion was associated with better clinical outcomes at 2 years in patients with single ventricle heart disease. The current study investigates time‐to‐event outcomes at 8 years. Methods and Results This cohort enrolled patients with single ventricles who underwent stage 2 or stage 3 palliation from January 2011 to January 2015 at 8 centers in Japan. The primary outcomes were time‐dependent CDC treatment effects on death and late complications during 8 years of follow‐up, assessed by restricted mean survival time. Among 93 patients enrolled (mean age, 2.3±1.3 years; 56% men), 40 received CDC infusion. Overall survival for CDC‐treated versus control patients did not differ at 8 years (hazard ratio [HR], 0.60 [95% CI, 0.21–1.77]; P=0.35). Treatment effect had nonproportional hazards for death favoring CDCs at 4 years (restricted mean survival time difference +0.33 years [95% CI, 0.01–0.66]; P=0.043). In patients with heart failure with reduced ejection fraction, CDC treatment effect on survival was greater over 8 years (restricted mean survival time difference +1.58 years [95% CI, 0.05–3.12]; P=0.043). Compared with control participants, CDC‐treated patients showed lower incidences of late failure (HR, 0.45 [95% CI, 0.21–0.93]; P=0.027) and adverse events (subdistribution HR, 0.50 [95% CI, 0.27–0.94]; P=0.036) at 8 years. Conclusions By 8 years, CDC infusion was associated with lower hazards of late failure and adverse events in single ventricle heart disease. CDC treatment effect on survival was notable by 4 years and showed a durable clinical benefit in patients with heart failure with reduced ejection fraction over 8 years. Registration URL: https://www.clinicaltrials.gov; Unique identifiers: NCT01273857 and NCT01829750.

AimsLimited data exist on risk factors for the long-term outcome of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD-PAH). We focused on the index of pulmonary vascular disease (IPVD), an assessment system for pulmonary artery pathology specimens. The IPVD classifies pulmonary vascular lesions into four categories based on severity: (1) no intimal thickening, (2) cellular thickening of the intima, (3) fibrous thickening of the intima, and (4) destruction of the tunica media, with the overall grade expressed as an additive mean of these scores. This study aimed to investigate the relationship between IPVD and the long-term outcome of CHD-PAH.MethodsThis retrospective study examined lung pathology images of 764 patients with CHD-PAH aged

Richter Transformation (RT) is the phenomenon of progression from Chronic Lymphocytic Leukemia (CLL) into an aggressive Large-Cell Lymphoma and is typically characterized by diffuse lymphadenopathy combined with the classical “B symptoms”. While rare, other causes of sudden onset diffuse lymphadenopathy in patients with CLL can occur; one of the rarest being necrotic herpes simplex lymphadenitis. We report a case that presented similarly to Richter Transformation including PET-CT scan findings consistent with RT but was histologically proven to be necrotic herpes simplex lymphadenitis. We identified less than 20 reported cases of this phenomenon in English language literature. Our patient was successfully treated with appropriate antiviral therapy due to timely recognition of the correct disease process. Our case reinforces the importance of maintaining diagnostic suspicion when approaching sudden onset lymphadenitis in this patient population.