Cardiac sarcoidosis in a carrier of transthyretin gene mutation: a case report.

Background: Sarcoidosis is a rare multiorgan inflammatory disorder of unknown aetiology, characterized by the formation of non-caseating granulomas in the affected organs. Cardiac involvement is underrecognized and observed in up to 25% of cases in autopsy studies, and is associated with a high mortality rate, especially due to sudden cardiac death due to ventricular arrhythmias. Case summary: A 41-year-old man well known to our hospital because of his father's diagnosis of cardiac amyloidosis, and carrier of transthyretin (TTR) gene mutation, was hospitalized following a resuscitated cardiac arrest. The patient was hospitalized a month before for a syncopal episode with demonstration of preserved left ventricular ejection fraction (LVEF) with akinetic basal septum at heart ultrasound and normal coronary. Chest computed tomography, performed in the emergency department, was significant for hilar lymphadenopathies and pulmonary nodules highly suggestive of sarcoidosis. A subsequent 18-fluorodeoxyglucose-positron emission tomography (FDG-PET) showed multiorgan phlogistic involvement, including the myocardium. After the diagnosis of cardiac sarcoidosis, the patient was started on steroids therapy and underwent ICD implantation. A follow-up 18-FDG-PET showed a reduction of organs glucose uptake and a follow-up echo an improvement in LVEF. Despite that, he occurs occasional recording of repetitive ventricular arrhythmias and one appropriate ICD shock during the next 12 months. Discussion: Cardiac sarcoidosis is an insidious disease. Its diagnosis can be challenging, with no specific finding in echocardiography. The best strategy would be multi-modality imaging involving both magnetic resonance imaging with late gadolinium enhancement and 18-FDG-PET, followed by biopsy to confirm the diagnosis. Multi-modality imaging should be further used to evaluate the response to treatment and assess prognosis. Since the patient was a known carrier of the TTR gene mutation, many efforts were made in order to come up with the correct diagnosis considering that both cardiac amyloidosis and cardiac sarcoidosis are non-ischaemic cardiomyopathy with systemic involvement.

Echocardiographic Abnormalities in Adults With Anorexia Nervosa.

Anorexia nervosa (AN) is a psychiatric disorder that may lead to cardiac complications. The objective of this study was to evaluate global and regional longitudinal strain changes in patients affected by AN as an early marker of myocardial damage. We prospectively enrolled 48 consecutive patients with AN and 44 age-matched and gender-matched healthy controls. In all subjects, we performed echocardiography, including global longitudinal strain (GLS) measurement. A subset of 33 patients with AN had further echocardiographic examinations during the follow-up. Compared with healthy controls, patients with AN had a greater prevalence of pericardial effusion (9 of 48 vs 0 of 44, p = 0.003), a smaller left ventricular mass (63 ± 15 vs 99 ± 30 g, p < 0.001), a lower absolute value of GLS (-18.9 ± 2.8 vs -20.2 ± 1.8%, p = 0.010) and of basal LS (-15.4 ± 6.0 vs -19.4 ± 2.6%, p < 0.001). The bull's eye mapping showed a plot pattern with blue basal areas in 18 of 48 patients with AN versus 1 of 44 controls (p < 0.001). During the follow-up, of 13 patients with blue areas in the first bull's eye mapping, 11 recovered completely, and of 20 patients with a red bull's eye at the first examination, none presented blue areas at the second one. In conclusion, GLS is significantly altered in patients with AN, and a basal blue pattern on bull's eye mapping identifies more severe cases. These changes seem to be reversible.

Double-valve infective endocarditis: clinical features and prognostic impact-a retrospective study in a surgical centre.

Most cases of infective endocarditis (IE) involve a single valve, and little is known concerning IE that simultaneously affects two valves. The involvement of more than one valve may imply more severe and extensive cardiac lesions. In these patients, surgery may be challenging. We aimed to determine the clinical characteristics, the therapeutic strategy, and the prognostic impact of double-valve IE (DVIE). We retrospectively included in the analysis that 440 consecutive patients with definite active IE in a single surgical centre. DVIE occurred in 75 of the total enrolled 440 patients (17%) and involved mostly the combination of mitral and aortic valves (N = 63, 84%). Most patients had double-native IE (N = 45, 60%). Staphylococci were less frequent in patients with double-valve than single-valve IE (SVIE). The proportion of patients undergoing valve repair among those treated surgically was higher for patients with DVIE than for SVIE (p < 0.03). Valve repair of at least one valve was associated with non-significant better survival than double replacement. DVIE was associated with higher all-cause mortality than SVIE (p < 0.013) and a higher relapse rate (p = 0.023). DVIE was not associated with a higher risk of composite non-fatal adverse events. DVIE represents a considerable proportion of overall cases of IE, mainly involving aortic and mitral valves, with a jet lesion on the mitral valve; Staphylococcus is significantly less frequent than in SVIE; DVIE is independently associated with higher mortality and relapse rate; finally, mitral valve repair is feasible in a considerable proportion of surgical cases.

Infective endocarditis in intravenous drug abusers: clinical challenges emerging from a single-centre experience.

BACKGROUND: Intravenous drug abuse (IDA) is a known risk factor for infective endocarditis (IE) and is associated with frequent relapses, but its prognostic impact is still debated. The potential futility of surgery in this population is a further issue under discussion. We aimed to describe the clinical characteristics, the therapeutic strategy, and the prognosis associated with IDA in IE. METHODS: We retrospectively analysed 440 patients admitted to a single surgical centre for definite active IE from January 2012 to December 2020. RESULTS: Patients reporting IDA (N = 54; 12.2%) were significantly younger (p < 0.001) and presented fewer comorbidities (p < 0.001). IDA was associated with a higher proportion of relapses (27.8 vs. 3.3%, p < 0.001) and, at multivariable analysis, was an independent predictor of long-term mortality (HR 2.3, 95%CI 1.1-4.7, p = 0.015). We did not register multiple relapses in non-IDA patients. Among IDA patients, we observed 1 relapse after discharge in 9 patients, 2 relapses in 5 patients and 3 relapses in 1 patient. In IDA patients, neither clinical and laboratory variables nor the occurrence of even multiple relapses emerged as indicators of an adverse risk-benefit ratio of surgery in patients with surgical indication. CONCLUSIONS: IE secondary to IDA affects younger patients than those with IE not associated with IDA. Probably due to this difference, IE secondary to IDA is not associated with significantly higher mortality, whereas the negative, long-term prognostic impact of IDA emerges in multivariate analysis. Considering the good prognosis of patients with uncomplicated IE treated medically, surgery should be reserved to patients with a strict- guidelines-based indication. However, since there are no clear predictors of an unfavourable risk-benefit ratio of surgery in patients with surgical indication, all patients with a complicated IE should be operated, irrespective of a history of IDA.

A new-onset pulmonary artery stenosis in a young man: case report.

BACKGROUND: Poorly differentiated and undifferentiated sarcomas are the most common primary tumours of the pulmonary arteries. They usually affect large-calibre vessels and present with predominantly intraluminal growth. Dyspnoea, cough, chest pain, and haemoptysis are the most common presenting symptoms. Clinical and imaging manifestations can mimic pulmonary embolisms and correct diagnosis may require multimodal imaging. The overall prognosis is poor; however, early diagnosis and complete surgical resection seem to improve the prognosis. CASE SUMMARY: A 31-year-old male was admitted to our department after a pre-syncopal episode associated with dyspnoea of recent onset. Echocardiography showed a mass with irregular borders attached to the pulmonary artery trunk, almost obliterating its lumen and determining a flow acceleration with a peak velocity and gradient, respectively, of 3.8 m/s and 60 mmHg. At cardiac magnetic resonance imaging and positron emission tomography-computed tomography scan, the mass had inhomogeneous contrast impregnation and an intense 18-fluorodeoxyglucose uptake, both findings are highly suggestive of an angiosarcoma of the pulmonary artery. Biopsy specimens were taken through bronchoscopy but the material was insufficient for diagnosis. The patient decided to continue treatment in another hospital, where he died a few months later. DISCUSSION: The presence of a unique mass involving the main trunk of the pulmonary artery or proximal branches associated with rapidly progressive dyspnoea in a patient at low risk for pulmonary embolism should raise the suspicion of primary sarcoma of the pulmonary artery. There are no guidelines for the treatment. Surgery and neo/adjuvant chemotherapy are reported in literature but burdened by bias and concerning a small number of cases.

Introducing enhanced recovery after surgery in a high-volume orthopaedic hospital: a health technology assessment.

BACKGROUND: The number of patients undergoing joint arthroplasty is increasing worldwide. An Enhanced Recovery After Surgery (ERAS) pathway for hip and knee arthroplasty was introduced in an Italian high-volume research hospital in March 2018. METHODS: The aim of this mixed methods observational study is to perform a health technology assessment (HTA) of the ERAS pathway, considering 938 procedures performed after its implementation, by means of a hospital-based approach derived from the EUnetHTA (European Network for Health Technology Assessment) Core Model. The assessment process is based on dimensions of general relevance, safety, efficacy, effectiveness, economic and financial impact, equity, legal aspects, social and ethical impact, and organizational impact. A narrative review of the literature helped to identify general relevance, safety and efficacy factors, and a set of relevant sub-dimensions submitted to the evaluation of the professionals who use the technology through a 7-item Likert Scale. The economic and financial impact of the ERAS pathway on the hospital budget was supported by quantitative data collected from internal or national registries, employing economic modelling strategies to identify the amount of resources required to implement it. RESULTS: The relevance of technology under assessment is recognized worldwide. A number of studies show accelerated pathways to dominate conventional approaches on pain reduction, functional recovery, prevention of complications, improvements in tolerability and quality of life, including fragile or vulnerable patients. Qualitative surveys on clinical and functional outcomes confirm most of these benefits. The ERAS pathway is associated with a reduced length of stay in comparison with the Italian hospitalization average for the same procedures, despite the poor spread of the pathway within the country may generate postcode inequalities. The economic analyses show how the resources invested in training activities are largely depreciated by benefits once the technology is permanently introduced, which may generate hospital cost savings of up to 2054,123.44 € per year. CONCLUSIONS: Galeazzi Hospital's ERAS pathway for hip and knee arthroplasty results preferable to traditional approaches following most of the HTA dimensions, and offers room for further improvement. The more comparable practices are shared, the before this potential improvement can be identified and addressed.

Effects of early virtual reality-based rehabilitation in patients with total knee arthroplasty: A randomized controlled trial.

BACKGROUND: Virtual reality (VR)-based rehabilitation is a promising approach for improving recovery in many conditions to optimize functional results, enhancing the clinical and social benefits of surgery. OBJECTIVE: To assess the efficacy of an early rehabilitation performed by the VR-based rehabilitation versus the traditional rehabilitation provided by physical therapists after primary total knee arthroplasty (TKA). METHODS: In this randomized controlled clinical trial, 85 subjects met the inclusion criteria and were randomized 3 to 4 days after TKA to an inpatient VR-based rehabilitation and a traditional rehabilitation. Participants in both groups received 60 minutes/day sessions until discharge (around 10 days after surgery). The primary outcome was the pain intensity. The secondary outcomes were: the disability knee, the health related quality of life, the global perceived effect, the functional independent measure, the drugs assumption, the isometric strength of quadriceps and hamstrings, the flexion range of motion, and the ability to perform proprioception exercises. Outcomes were assessed at baseline (3-4 days after TKA) and at discharge. RESULTS: VR-based or traditional rehabilitation, with 13% of dropout rate, shown no statistically significant pain reduction between groups (P = .2660) as well as in all other outcomes, whereas a statistically significant improvement was present in the global proprioception (P = .0020), in favor of the VR-based rehabilitation group. CONCLUSIONS: VR-based rehabilitation is not superior to traditional rehabilitation in terms of pain relief, drugs assumptions and other functional outcomes but seems to improve the global proprioception for patients received TKA. LEVEL OF EVIDENCE: Therapy, level 1b. CONSORT-compliant. TRIAL REGISTRATION: http://www.clinicaltrials.gov, ClinicalTrials.gov, NCT02413996.