Giant Pulmonary Artery Thrombotic Material, Due to Chronic Thromboembolic Pulmonary Hypertension, Mimics Pulmonary Artery Sarcoma.

In this article, we present the case of a 38-year-old female who suffered from serious respiratory distress. After an extensive pulmonary artery imaging diagnostic work-up (CTPA, MRA and PET), we were unable to differentiate between chronic thromboembolic pulmonary hypertension (CTEPH) vs. pulmonary artery sarcoma (PAS) due to extensive filling defects and extraluminal findings. Although surgery was postponed for nine months due to the COVID-19 pandemic, CTEPH diagnosis, due to a high-thrombus burden, was finally confirmed after pulmonary endarterectomy (PEA). Conclusively, imaging findings of rare cases of CTEPH might mimic PAS and the surgical removal of the lesion are both needed for a final diagnosis. What is Already Known about This Topic? Pulmonary artery sarcoma (PAS) is a rare but aggressive malignancy, which originates from the intimal layer of the pulmonary artery (PA); Chronic thromboembolic pulmonary hypertension (CTEPH) is based on chronic, organized flow-limiting thrombi inside PA circulation and subsequent pulmonary hypertension. What Does This Study Contribute? Since radiological findings of CTEPH cases might rarely mimic PAS, pulmonary artery endarterectomy and subsequent histopathologic study are needed for a final diagnosis.

The dermatoscopic spectrum of cutaneous lupus erythematosus: A retrospective analysis by clinical subtype with clinicopathological correlation.

The skin is the most common organ of involvement during the course of lupus erythematosus (LE). The literature data concerning the dermatoscopic patterns of the different clinical variants of cutaneous LE (CLE), namely chronic (CCLE), subacute (SCLE), and acute (ACLE), are scarce. To determine the dermatoscopic spectrum of CLE and to correlate the dermatoscopic features with the histological findings. This was a retrospective, observational, multicenter, cohort study. We evaluated the dermatoscopic features in a cohort of patients diagnosed with CLE. Furthermore, we investigated their frequency per clinical subtype and correlated them with the anatomic alterations. We included 79 patients. The most prevalent dermatoscopic features of CCLE included follicular plugs (86.4%, P < .01), patchy distribution (75%, P = .1) of mostly linear curved vessels (56.8%, P = .8), white scales (68.2%, P < .01), and structureless white color (68.2%, P < .01). The most common criteria of SCLE were patchy distribution (90%, P = .1) of mostly linear curved vessels (53.3%, P = .8) and fine white scales (60%, P < .01), while ACLE was characterized by erythema (100%, P < .05) and patchy distribution (100%, P = .1) of mostly dotted vessels (60%, P = .4). Follicular plugs/rosettes in dermatoscopy strongly correlated with follicular plugs in histology (rho = 0.919). Hyperkeratosis significantly correlated with white (rho = 0.644) and yellow/brown scales (rho = 0.225), telangiectasia with linear curved vessels (rho = 0.321) and white color with dermal fibrosis (rho = 0.623). Depending on CLE subtype, distinct dermatoscopic patterns are recognized. In CLE there is a high correlation between certain dermatoscopic criteria and the underneath anatomic alteration.

NT-proBNP/cardiac troponin T ratio >7.5 on the second day of admission can differentiate Takotsubo from acute coronary syndrome with good accuracy.

BACKGROUND: Takotsubo syndrome (TTS) is not usually diagnosed until patients with suspected acute coronary syndrome (ACS) and echocardiographically detected apical aneurysm are found to have "normal" coronary angiography (CA). Our aim was to explore whether cardiac biomarkers can contribute to the early diagnosis of TTS. METHODS: Ratios of N-terminal-pro brain natriuretic peptide (NT-proBNP) and high-sensitivity cardiac troponin T (cTnT) both expressed in pg/mL [admission and the 3 following days] were compared in 38 patients with TTS and 114 ACS patients of whom 58 had non-ST-elevation myocardial infarction (NSTEMI). RESULTS: NT-proBNP/cTnT ratio at admission and during the following 3 days was significantly higher in TTS compared to patients with ACS [18.4 (8.7-41.7) vs 2.9 (0.8-6.8), 29.6 (14.3-53.7) vs 1.2 (0.5-2.7), 30.0 (11.6-50.9) vs 1.7 (0.5-3.0), 27.8 (11.3-42.6) vs 1.4 (0.6-2.8), respectively, all <0.001]. Βest discrimination of TTS from ACS was possible with the ratio of NT-proBNP/cTnT on the 2nd day. A cut-off value of NT-proBNP/cTnT ratio >7.5 had a sensitivity of 97.3%, a specificity of 95.4% and an accuracy of ∼96% in detecting TTS as opposed to ACS. Furthermore, the ratio of NT-proBNP/cTnT preserved its discriminatory value in the subgroup of patients with NSTEMI. In particular, an NT-proBNP/cTnT ratio >7.5 on the 2nd day had a sensitivity of 97.3%, a specificity of 91.4%, and an accuracy of 93.7% in differentiating TTS from NSTEMI. CONCLUSIONS: An NT-proBNP/cTnT ratio >7.5 on the 2nd day of admission can be useful for the early identification of TTS among selected patients initially presenting with ACS, a ratio more clinically useful in the setting of NSTEMI.

Is premorbid stress assessed by hair cortisol concentration linked to Takotsubo syndrome? Results from a pilot study.

INTRODUCTION: The pathophysiology of Takotsubo syndrome (TTS) is not completely understood and the role of chronic stress is among the main mechanistic links. The aim of this study was to explore whether accumulating hair cortisol concentration (HCC), a novel biomarker of chronic stress, is associated with the occurrence of TTS. METHODS: A consecutive series of 18 TTS patients and 36 age and sex matched healthy controls were included in our analysis. Hair samples were collected from participants'' vertex. The proximal 2.5 cm of hair was cut in equal parts of 0.5 cm, reflecting mean cortisol levels in time intervals of 0-15, 15-30, 30-45, 45-60 and 60-75 days prior to hair collection. RESULTS: HCC was higher in TTS group compared to controls at any time point and increased over time starting from 75 days prior to the event. The rate of HCC increase was significantly higher in TTS patients versus controls (beta of interaction = 0.48; 95%CI: 0.36-0.60; p < 0.001). CONCLUSIONS: The steadily increasing trend of HCC in TTS patients suggests that the additive effect of multiple stressful events over several weeks prior TTS onset may disrupt cortisol homeostasis and play a role in TTS pathophysiology.

The value of physical signs in identifying patients with familial hypercholesterolemia in the era of genetic testing.

Familial hypercholesterolemia (FH) is a common, inherited disorder of cholesterol metabolism characterized by very high plasma concentrations of low-density lipoprotein cholesterol. It is crucial to diagnose and treat this disorder early since if left untreated it increases the risk for coronary artery disease (CAD) at least by 10-fold. Although genetic testing for FH, when available and affordable, should ideally be offered to most individuals with clinical phenotype suggestive of FH, it is underutilized in most countries. Therefore, FH diagnosis in the majority of cases is made by combining cholesterol levels and clinical characteristics of the patient leaving the need for genetic testing usually in equivocal cases. The presence of some cutaneous and ocular signs can raise the suspicion or even lead to the diagnosis of FH among usually "healthy" individuals. These physical signs comprise cutaneous lesions such as tendon xanthomas or the less specific xanthelasmata and ocular signs, such as corneal arcus in individuals under the age of 45 years. The presence of these signs should prompt the physician to request lipid tests and use clinical scores to diagnose FH. If the diagnosis of FH is likely, aggressive lipid-lowering therapy should be initiated to reduce the risk of CAD and a cascade screening of family members should also be requested.