Tenosynovial complications identify TTR cardiac amyloidosis among patients with hypertrophic cardiomyopathy phenotype

Recent evidence suggests that carpal tunnel syndrome (CTS) and brachial biceps tendon rupture (BBTR) represent red flags for ATTR cardiac amyloidosis (ATTR-CA). The prevalence of upper limb tenosynovial complications in conditions entering differential diagnosis with CA, such as HCM or Anderson–Fabry disease (AFD), and hence their predictive accuracy in this setting, still remains unresolved. Objective: To investigate the prevalence of CTS and BBTR in a consecutive cohort of ATTR-CA patients, compared with patients with HCM or AFD and with individuals without cardiac disease history. Participants: Consecutive patients with a diagnosis of ATTR-CA, HCM and AFD were evaluated. A control group of consecutive patients was recruited among subjects hospitalized for noncardiac reasons and no cardiac disease history. The presence of BBTR, CTS or prior surgery related to these conditions was ascertained. Results: 342 patients were prospectively enrolled, including 168 ATTR-CA (141 ATTRwt, 27 ATTRm), 81 with HCM/AFD (N = 72 and 9, respectively) and 93 controls. CTS was present in 75% ATTR-CA patients, compared with 13% and 10% of HCM/AFD and controls (P = 0.0001 for both comparisons). Bilateral CTS was present in 60% of ATTR-CA patients, while it was rare (2%) in the other groups. BBTR was present in 44% of ATTR-CA patients, 8% of controls and 1% in HCM/AFD. Conclusions: CTS and BBTR are fivefold more prevalent in ATTR-CA patients compared with cardiac patients with other hypertrophic phenotypes. Positive predictive accuracy for ATTR-CA is highest when involvement is bilateral. Upper limb assessment of patients with HCM phenotypes is a simple and effective way to raise suspicion of ATTR-CA.     

Congenital nasal pyriform aperture stenosis: diagnosis and management

ABSTRACT: BACKGROUND: Congenital nasal pyriform aperture stenosis is a rare and potentially lethal form of airway obstruction in newborns. Immediate recognition and appropriate therapy are mandatory for this potentially life-threating condition. This anomaly may present as an isolated malformation or may be associated with other cranial-facial anomalies. Clinically, CNPS shows unspecific symptoms of nasal airway obstruction such as apnoic crisis, episodic cyanosis and inability to nurse. The purpose of this report is to present author's experience in the surgical management of this rare pathology in 3 patients. Patients and Methods Three cases of congenital nasal pyriform aperture stenosis were reviewed for presentation of the disorder, management and effectiveness of the surgical treatment. RESULTS: All the patients underwent a surgical correction of the pyriform aperture stenosis using a sublabial approach followed by nasal stenting. During the follow-up no cases of restenosis, respiratory failure or cyanosis were detected. CONCLUSIONS: Congenital pyriform aperture stenosis should be suspected in newborns with clinical signs of severe nasal obstruction associated with a difficulty to pass a small catheter though the anterior nasal valve. Timely recognition is mandatory to prevent a potential deadly outcome. Surgical correction of the stenosis though a sublabial approach followed by a nasal stenting revealed to be most effective treatment for these patients.     

Use of information communication technology and stress,burnout, and mental health in older,middle-aged,and younger workers – results from a systematic review

The aim of this systematic review is to summarise quantitative studies in occupational settings observing the association between Information communication technology (ICT) and stress, and burnout, considering age as an effect modifier. A systematic review using PRISMA guidelines was conducted through the following bibliographic databases: PubMed, Web of Science, Psycinfo, and the Cochrane Library. Inclusion criteria were occupational settings and content relevant to our research question. Risk of bias was assessed using the Newcastle–Ottawa scale. Two interventional, 4 cohorts, and 29 cross-sectional studies were found. ICT use in occupational settings was associated with stress seen in cross-sectional studies, but not in interventional studies. There was a concordant association with ICT and burnout in different study designs. Overall, there were no linear trends between age and technostress. We suggest that the observed associations were mostly present in the middle-aged working population and that these associations need to be supported in further studies.     

Oral anticoagulation and VKORC1 polymorphism in patients with a mechanical heart prosthesis: a 6-year follow-up

Therapy with Vitamin K antagonists (VKA) effectively reduces the thrombosis risk in many clinical conditions. Genetic variants of vitamin K epoxide reductase (VKORC-1) are associated with increased VKA effect and bleeding risk. It is unknown whether these variants could also affect the long-term outcome in patients with high-dosage oral anticoagulation and/or more difficult adherence to the therapeutic INR range. Hundred and twenty-four patients with mechanical heart valve replacement assuming VKA were genotyped for VKORC-1 ?1639G>A (Rs9923231) polymorphism. Hemorrhage, venous thrombosis and atherothrombotic events were retrospectively assessed for a 6-year period. Furthermore, stability of their INR in relationship with the VKORC-1 genotype was investigated day-by-day for 3?months. No differences were observed in hemorrhage and venous thrombosis events according to rs 9923231. GG genotype carriers (n?=?41) had no atherothrombotic events, while 4 strokes, 4 TIA and 3 AMI were diagnosed in A carriers (n?=?83; P?=?0.0008). During the daily observation period, A allele carriers had lower VKA requirements (4.7, 3.7, 2.2?mg/day for GG/GA/AA genotype respectively; P?=?0.00001), higher mean INR (2.7, 2.8, 2.9; P?=?0.05) and a higher number of examinations above the therapeutic range than GG carriers (17 % vs. 0 % in GG genotype, P?=?0.036). Conversely, patients with GG genotype had a more stable dosage of VKA (P?=?0.006) and a higher percentage of examinations under the therapeutic range (51, 43 and 36 % in GG, GA and AA genotype, respectively, P?=?0.040). In patients with high dosage VKA, VKORC-1 polymorphism is associated to a different warfarin dosage, anticoagulation level, time spent outside the therapeutic range and, in the long-term, a different incidence of atherothrombotic events.     

Patient-Specific Modeling of Stented Coronary Arteries Reconstructed from Optical Coherence Tomography: Towards a Widespread Clinical Use of Fluid Dynamics Analyses

The recent widespread application of optical coherence tomography (OCT) in interventional cardiology has improved patient-specific modeling of stented coronary arteries for the investigation of local hemodynamics. In this review, the workflow for the creation of fluid dynamics models of stented coronary arteries from OCT images is presented. The algorithms for lumen contours and stent strut detection from OCT as well as the reconstruction methods of stented geometries are discussed. Furthermore, the state of the art of studies that investigate the hemodynamics of OCT-based stented coronary artery geometries is reported. Although those studies analyzed few patient-specific cases, the application of the current reconstruction methods of stented geometries to large populations is possible. However, the improvement of these methods and the reduction of the time needed for the entire modeling process are crucial for a widespread clinical use of the OCT-based models and future in silico clinical trials.     

(In)accuracy of blood pressure measurement in 14 Italian hospitals

OBJECTIVES:: The diagnosis and control of hypertension depend on accurate measurement of blood pressure (BP). The literature on the accuracy of BP recording by health professionals is, however, limited, and no study directly interviewed patients in the hospital setting. This multicenter cross-sectional study aimed at evaluating the compliance to current recommendations on BP measurement by health professionals directly from patients and to investigate potential predictors of higher quality in BP recording. METHODS:: A trained nurse interviewed a random sample of adult patients hospitalized for an ordinary admission (except in the emergency room) lasting more than one night, without mental disorder, who had their BP routinely measured by the hospital personnel less than 3?h before. The questionnaire contained 15 items on the main procedures that are common to current guidelines. RESULTS:: Fourteen public hospitals from seven regions of Italy participated, and 1334 questionnaires were collected. Nine of the recommended practices were followed in the majority (>70%) of BP recordings, whereas some others were infrequent or rare: in 98.6, 82.2 and 81.1% of the participants, respectively, the arm circumference was never recorded, BP was measured only once, and BP was never recorded in both arms. Overall, 10 or more recommended procedures were followed during 33.4% recordings. At multivariate analysis, physicians were less likely than nurses to provide a more accurate BP measurement. CONCLUSIONS:: The operator's compliance to some recommendations in BP measurement is unacceptably low. This survey provides detailed indications for medical directors on the procedures and settings to prioritize in educational programs, which are definitely needed.