Background: Triglyceride (TG) deposit cardiomyovasculopathy (TGCV) is a novel cardiovascular disorder and was recently encoded as an orphan disease in Europe (ORPHA code: 565612). Defective lipolysis results in TG accumulation in the myocardium and coronary arteries in TGCV. The myocardial washout rate (WR) of iodine-123-β-methyl iodophenyl-pentadecanoic acid (BMIPP) is an essential indicator to evaluate myocardial lipolysis in vivo. TGCV is classified into primary and idiopathic type with and without PNPLA2 mutation, respectively. Here, we present the clinical correlation perspectives of TGCV patients in Chiba, Japan, to increase the awareness of this orphan disease and facilitate its diagnosis. Methods: We enrolled 234 patients who underwent BMIPP scintigraphy between September 2015 and July 2019. The diagnosis of TGCV was made based on the criteria we reported previously. Blood smear tests were performed for TGCV classification. The distributions of TGCV in each comorbidity were investigated. Results: In total, 104 patients were diagnosed with definitive idiopathic TGCV (I-TGCV). They had various comorbid conditions, including heart failure with reduced ejection fraction and multivessel coronary artery disease requiring revascularization. Moreover, the serum TG levels in I-TGCV patients were not high, and there was no correlation between serum TG level and BMIPP WR (n=205, p-value=0. 31), supporting the pathophysiological hypothesis of TGCV. Conclusion: I-TGCV patients showed multiple coexistence of coronary artery disease, heart failure of unknown etiology, or diabetes mellitus. For patients with such clinical characteristics, BMIPP scintigraphy and calculation of WR should be considered proactively for the diagnosis of TGCV.
Background:The arithmetic mean of washout rate (WR) (namely, AMWR) of each segment is a commonly used algorithm for calculating WR from a polar map in single-photon emission computerized tomography (SPECT). However, in this algorithm, uneven radiotracer uptake among segments affects WR calculation. To solve this possible issue, we formulated a modified algorithm for calculating WR based on the total count (namely, TCWR). Methods: The WR of iodine-123-β-methyl-p-iodophenylpentadecanoic acid (BMIPP) was calculated using TCWR and AMWR, and WR values using TCWR and AMWR were compared by disease. Participants included those without cardiovascular diseases (normal), those with CD36 deficiency, triglyceride deposit cardiomyovasculopathy (TGCV), TGCV with old myocardial infarction (OMI), and non-TGCV with OMI. Results: WR values using TCWR and AMWR did not differ significantly in the following groups: normal, 27.4± 8.5 and 27.3±8.5% (p=0.97); CD36 deficiency, -3.2±6.5 and -4.1±7.4% (p=0.81); TGCV, 2.4±6.3 and 2.2± 6.3% (p=0.93); and TGCV with OMI, -0.9±7.6 and -3.7±8.4% (p=0.32). However, AMWR showed a lower WR than TCWR in non-TGCV with OMI (4.8±8.7 and 18.9±6.7%, p=0.0008). Conclusions: TCWR is suitable for calculating WR using SPECT polar maps even in cases with heterogeneous radiotracer uptake, such as OMIs. TCWR may be applied to measuring the WR of radiopharmaceuticals other than BMIPP in investigating the pathophysiology of heart diseases.
Pembrolizumab, a treatment for advanced cancers, can cause immune-related adverse events (irAEs), and among these, esophagitis is rare. We report on an 80-year-old man with squamous cell lung carcinoma who started treatment with pembrolizumab at 200 mg every three weeks. After the first administration, the patient developed odynophagia. Esomeprazole did not improve symptoms, and upper gastrointestinal endoscopy and biopsy revealed esophagitis dissecans superficialis, which was considered as an irAE. Oral prednisolone was started, and odynophagia rapidly improved and the mucosal lesions healed. However, steroid withdrawal was unsuccessful because of repeated relapses. When patients on immune checkpoint inhibitors develop dysphagia, esophagitis should be suspected as an irAE.
Background Triglyceride (TG) deposit cardiomyovasculopathy (TGCV) is a novel cardiovascular disorder and was recently encoded as an orphan disease in Europe (ORPHA code: 565612). Defective intracellular lipolysis results in TG accumulation in the myocardium and coronary arteries in TGCV. The myocardial washout rate (WR) of iodine-123-β-methyl-p-iodophenylpentadecanoic acid (BMIPP) is an essential indicator to evaluate myocardial lipolysis in vivo, and decreased WR (<10%) of BMIPP is one of the essential items of diagnostic criteria for TGCV. Purpose To clarify clinical findings of TGCV including comorbid conditions and laboratory findings. Methods We enrolled 234 patients who underwent BMIPP scintigraphy between September 2015 and July 2019. The distributions of TGCV in each comorbidity, cardiac functions and laboratory findings were investigated. Results In total, 104 patients were diagnosed with definitive TGCV. The BMIPP WR of TGCV patients was −1.37±10.6%. TGCV patients had various comorbid conditions, including coronary artery disease (75%), diabetes mellitus (56%), and heart failure (21%). Left ventricular ejection fraction (LVEF) of TGCV patients was significantly lower than that of non-TGCV patients (38.1±18.0% vs. 43.6±18.9%, p-value=0.026). Moreover, among those who did not take lipid-lowering drugs, there was no difference in the serum TG level between TGCV and non-TGCV patients (TGCV: n=44, 127±84.6 mg/dL, non-TGCV: n=66, 133±70.7 mg/dL, p-value=0.73). Conclusions TGCV patients showed multiple coexistence of coronary artery disease, diabetes mellitus, or heart failure with lower LVEF. Serum TG level was not significantly different between TGCV and non-TGCV patients. Serum TG did not affect the intracellular TG accumulation in TGCV patients directly, and this result was consistent with the pathophysiological hypothesis that the TG accumulation in the myocardial cytoplasm is due to intracellular lipase dysfunction. Funding Acknowledgement Type of funding sources: None.
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