The usefulness of speckle tracking echocardiography for the prediction of cardiac involvement in patients with biopsy-proven sarcoidosis.

INTRODUCTION: Cardiac sarcoidosis (CS) is commonly diagnosed based on clinical criteria and abnormalities in noninvasive imaging reported in patients with biopsy-proven extracardiac sarcoidosis. Electrocardiogram and two-dimensional echocardiography have a low sensitivity for CS detection. Cardiovascular magnetic resonance imaging (CMR) and positron emission tomography (PET) have limitations in terms of cost and availability. OBJECTIVES: This study aimed to assess the usefulness of left ventricular longitudinal strain, measured using two-dimensional speckle tracking echocardiography (STE), for the prediction of late gadolinium enhancement (LGE) presence in CMR in patients with biopsy-proven sarcoidosis. PATIENTS AND METHODS: A total of 119 patients with biopsy-proven extracardiac sarcoidosis were divided, according to the clinical criteria proposed by the 2014 Heart Rhythm Society expert consensus statement (HRS 2014), into two groups: 43 individuals with "probable cardiac sarcoidosis", CS(+) and 76 individuals without cardiac sarcoidosis, CS (-). Data from echocardiography, CMR, 12-lead ECG and 24 h Holter monitoring were analyzed. RESULTS: Left ventricular global longitudinal strain (LV-GLS) was slightly reduced in the entire sarcoidosis group (-18.61± 2.96), no difference between the CS (+) and CS (-) subgroups was found (-18.0% ± 3.2% and -18.9% ± 2.8%, respectively; p = .223). No cut-off value for LV-GLS was identified that could predict the presence of LGE. Segmental longitudinal strain impairment partially correlated with the presence of LGE on CMR. CONCLUSIONS: In our cohort of sarcoidosis patients, segmental longitudinal strain proved more helpful in the diagnostic process than LV-GLS. The ultimate role of STE in the diagnosis of CS remains to be established.

Cardiac Magnetic Resonance Relaxometry Parameters, Late Gadolinium Enhancement, and Feature-Tracking Myocardial Longitudinal Strain in Patients Recovered from COVID-19.

COVID-19 infection is associated with myocarditis, and cardiovascular magnetic resonance (CMR) is the reference non-invasive imaging modality for myocardial tissue characterization. Quantitative CMR techniques, such as feature tracking (FT) and left ventricular global longitudinal strain (GLS) analysis, have been introduced as promising diagnostic tools to improve the diagnostic accuracy of suspected myocarditis. The aim of this study was to analyze the left ventricular global longitudinal strain (GLS) and the influence of T1 and T2 relaxation times, ECV, and LGE appearance on GLS parameters in a multiparametric imaging protocol in patients who recovered from COVID-19. The 86 consecutive patients enrolled in the study had all recovered from mild or moderate COVID-19 infections; none required hospitalization. Their persistent symptoms and suspected myocarditis led to cardiac magnetic resonance imaging within 3 months of the diagnosis of the SARS-CoV-2 infection. Results: Patients with GLS less negative than -15% had significantly lower LVEF (53.6% ± 8.9 vs. 61.6% ± 4.8; <0.001) and were significantly more likely to have prolonged T1 (28.6% vs. 7.5%; p = 0.019). Left ventricular GLS correlated significantly with T1 (r = 0.303; p = 0.006) and LVEF (r = -0.732; p < 0.001). Left ventricular GLS less negative than -15% was 7.5 times more likely in patients with prolonged T1 (HR 7.62; 95% CI 1.25-46.64). The reduced basal inferolateral longitudinal strain had a significant impact on the global left ventricular longitudinal strain. ROC results suggested that a GLS of 14.5% predicted prolonged T1 relaxation time with the best sensitivity and specificity. Conclusions: CMR abnormalities, including a myocarditis pattern, are common in patients who have recovered from COVID-19. The CMR feature-tracking left ventricular GLS is related to T1 relaxation time and may serve as a novel parameter to detect global and regional myocardial injury and dysfunction in patients with suspected myocardial involvement after recovery from COVID-19.

Left Ventricular Function and Iron Loading Status in a Tertiary Center Hemochromatosis Cohort-A Cardiac Magnetic Resonance Study.

Background: Haemochromatosis (HCH), a common genetic disorder with variable penetrance, results in progressive but understudied iron overload. We prospectively evaluated organ iron loading and cardiac function in a tertiary center HCH cohort. Methods: 42 HCH patients (47 ± 14 years) and 36 controls underwent laboratory workup and cardiac magnetic resonance (CMR), including T1 and T2* mapping. Results: Myocardial T2* (myoT2*), myocardial T1 (myoT1) and liver T2* (livT2*) were lower in patients compared to controls (33 ± 4 ms vs. 36 ± 3 ms [p = 0.004], 964 ± 33 ms vs. 979 ± 25 ms [p = 0.028] and 21 ± 10 ms vs. 30 ± 5 ms [p < 0.001], respectively). MyoT2* did not reach the threshold of clinically significant iron overload (<20 ms), in any of the patients. In 22 (52.4%) patients, at least one of the tissue parameters was reduced. Reduced myocardial T2* and/or T1 were found in 10 (23.8%) patients, including 4 pts with normal livT2*. LivT2* was reduced in 18 (42.9%) patients. MyoT1 and livT2* inversely correlated with ferritin (rs = −0.351 [p = 0.028] and rs = −0.602 [p < 0.001], respectively). LivT2* by a dedicated sequence and livT2* by cardiac T2* mapping showed good agreement (ICC = 0.876 p < 0.001). Conclusions: In contemporary hemochromatosis, significant myocardial iron overload is rare. Low myocardial T2* and/or T1 values may warrant closer follow-up for accelerated myocardial iron overload even in patients without overt liver overload. Cardiac T2* mapping sequence allows for liver screening at the time of CMR.

Feasibility of Intravoxel Incoherent Motion (IVIM) and Dynamic Contrast-Enhanced Magnetic Resonance Imaging (DCE-MRI) in Differentiation of Benign Parotid Gland Tumors.

Aim: The aim of this prospective study is to identify quantitative intravoxel incoherent motion and dynamic contrast-enhanced magnetic resonance imaging parameters of the most frequent benign parotid tumors, compare their utility and diagnostic accuracy. Methods: The study group consisted of 52 patients with 64 histopathologically confirmed parotid focal lesions. Parametric maps representing apparent diffusion coefficient (ADC), pure diffusion coefficient (D), pseudo-diffusion coefficient (D*), perfusion fraction (FP) and transfer constant (Ktrans), reflux constant (Kep), extra-vascular extra-cellular volume fraction (Ve), and initial area under curve in 60 s (iAUC) have been obtained from multiparametric MRI. Results: Statistically significant (p < 0.001) inter-group differences were found between pleomorphic adenomas (PA) and Warthin tumors (WT) in all tested parameters but iAUC. Receiver operating characteristic curves were constructed to determine the optimal cut-off levels of the most significant parameters allowing differentiation between WT and PA. The Area Under the Curve (AUC) values and thresholds were for ADC: 0.931 and 1.05, D: 0.896 and 0.9, Kep: 0.964 and 1.1 and Ve: 0.939 and 0.299, respectively. Lesions presenting with a combination of ADC, D, and Ve values superior to the cut-off and Kep values inferior to the cut-off are classified as pleomorphic adenomas. Lesions presenting with combination of ADC, D, and Ve values inferior to the cut-off and Kep values superior to the cut-off are classified as Warthin tumors. Conclusions: DWI, IVIM and quantitative analysis of DCE-MRI derived parameters demonstrated distinctive features of PAs and WT and as such they seem feasible in differentiation of benign parotid gland tumors.

Respiratory Motion-Registered Isotropic Whole-Heart T2 Mapping in Patients With Acute Non-ischemic Myocardial Injury.

Background: T2 mapping is a magnetic resonance imaging technique that can be used to detect myocardial edema and inflammation. However, the focal nature of myocardial inflammation may render conventional 2D approaches suboptimal and make whole-heart isotropic 3D mapping desirable. While self-navigated 3D radial T2 mapping has been demonstrated to work well at a magnetic field strength of 3T, it results in too noisy maps at 1.5T. We therefore implemented a novel respiratory motion-resolved compressed-sensing reconstruction in order to improve the 3D T2 mapping precision and accuracy at 1.5T, and tested this in a heterogeneous patient cohort. Materials and Methods: Nine healthy volunteers and 25 consecutive patients with suspected acute non-ischemic myocardial injury (sarcoidosis, n = 19; systemic sclerosis, n = 2; acute graft rejection, n = 2, and myocarditis, n = 2) were included. The free-breathing T2 maps were acquired as three ECG-triggered T2-prepared 3D radial volumes. A respiratory motion-resolved reconstruction was followed by image registration of the respiratory states and pixel-wise T2 mapping. The resulting 3D maps were compared to routine 2D T2 maps. The T2 values of segments with and without late gadolinium enhancement (LGE) were compared in patients. Results: In the healthy volunteers, the myocardial T2 values obtained with the 2D and 3D techniques were similar (45.8 ± 1.8 vs. 46.8 ± 2.9 ms, respectively; P = 0.33). Conversely, in patients, T2 values did differ between 2D (46.7 ± 3.6 ms) and 3D techniques (50.1 ± 4.2 ms, P = 0.004). Moreover, with the 2D technique, T2 values of the LGE-positive segments were similar to those of the LGE-negative segments (T2LGE-= 46.2 ± 3.7 vs. T2LGE+ = 47.6 ± 4.1 ms; P = 0.49), whereas the 3D technique did show a significant difference (T2LGE- = 49.3 ± 6.7 vs. T2LGE+ = 52.6 ± 8.7 ms, P = 0.006). Conclusion: Respiratory motion-registered 3D radial imaging at 1.5T led to accurate isotropic 3D whole-heart T2 maps, both in the healthy volunteers and in a small patient cohort with suspected non-ischemic myocardial injury. Significantly higher T2 values were found in patients as compared to controls in 3D but not in 2D, suggestive of the technique's potential to increase the sensitivity of CMR at earlier stages of disease. Further study will be needed to demonstrate its accuracy.

Aging and Hypertension - Independent or Intertwined White Matter Impairing Factors? Insights From the Quantitative Diffusion Tensor Imaging.

Aging disrupts white matter integrity, and so does continuous elevated blood pressure that accompanies hypertension (HTN). Yet, our understanding of the interrelationship between these factors is still limited. The study aimed at evaluating patterns of changes in diffusion parameters (as assessed by quantitative diffusion fiber tracking - qDTI) following both aging, and hypertension, as well as the nature of their linkage. 146 participants took part in the study: the control group (N = 61) and the patients with hypertension (N = 85), and were divided into three age subgroups (25-47, 48-56, 57-71 years). qDTI was used to calculate the values of fractional anisotropy, mean, radial and axial diffusivity in 20 main tracts of the brain. The effects of factors (aging and hypertension) on diffusion parameters of tracts were tested with a two-way ANOVA. In the right hemisphere there was no clear effect of the HTN, nor an interaction between the factors, though some age-related effects were observed. Contrary, in the left hemisphere both aging and hypertension contributed to the white matter decline, following a functional pattern. In the projection pathways and the fornix, HTN and aging played part independent of each other, whereas in association fibers and the corpus callosum if the hypertension effect was significant, an interaction was observed. HTN patients manifested faster decline of diffusion parameters but also reached a plateau earlier, with highest between-group differences noted in the middle-aged subgroup. Healthy and hypertensive participants have different brain aging patterns. The HTN is associated with acceleration of white matter integrity decline, observed mainly in association fibers of the left hemisphere.

The Duration of the correction loss after removing cheneau brace in patients with adolescent idiopathic scoliosis.

OBJECTIVE: The aim of the study was to evaluate the loss of truncal rotation over 54 hours after removing Chêneau brace. METHODS: The studied groups consisted of 39 girls aged 10-18 years old, diagnosed with adolescent idiopathic scoliosis (AIS) and treated with Chêneau brace (CAST) and 20 AIS girls aged 10-18 years old, not treated with bracing. Posterior-anterior radiographs were obtained from the clinical assessment of all subjects and were subsequently used to determine Cobb angles. The measurements of the angle of trunk rotation (ATR) were taken with the Scoliometer® and back-contour device during Adams forward bending test by the two evaluators. The changes in ATRs during 54 hours of observation were performed after the brace had been taken off (0, 2, 24, 30, 48 and 54 hours after debracing). This was described using VATR variable, defined as the change in the absolute Scoliometer® readings in the time intervals against the time interval Δt between the measurements. During back-contour assessment the differential factor (kra) has been used for the digital analysis. The changes in kra over 54 hours of observation were expressed as Vkra factor, defined as the difference in the absolute value of the amplitude differential factor (kra) in the time intervals against the time interval Δt between the measurements. RESULTS: The highest changes were observed in the thoracic as well as in lumbar spine in patients with Cobb angle ≥30°, axial rotation of the apical vertebrae within 5-15°, Risser sign 0-2. The biggest change in the trunk rotation after Chêneau brace had been taken off was noted within the first two hours of observation. CONCLUSION: The patients should be advised to take the brace off for a minimum of two hours before the scheduled x-ray, to allow full relaxation of the trunk in order to obtain reliable radiological images of the deformation. LEVEL OF EVIDENCE: Level III Therapeutic study.

Arthrogryposis multiplex congenital - multidisciplinary care - including own experience.

Arthrogryposis multiplex congenital (AMC) is a heterogeneous disorder, characterized by nonprogressive multiple intra-articular contractures, which can be recognized at birth. The prevalence in Europe is estimated at about 1 per 12,000. Etiopathogenesis of arthrogryposis is multifactorial. Symptoms of some forms of arthrogryposis can be found in the clinical presentation of selected genetic disorders, e.g. Pena Shokeir syndrome. Arthrogryposis can also result from environmental factors such as medication, trauma or chronic illness during pregnancy, as well as from oligohydramnios or abnormal structure of the uterus. In this particular disorder prenatal diagnosis is crucial, because it determines the level of hospital reference during the delivery of the affected child. The highest reference degree hospital is preferential, with staff prepared for the multidisciplinary approach to the treatment of the newborn. The key to success is rehabilitation treatment and it should be initiated as soon as possible. In a substantial number of cases, physical therapy can replace invasive corrective surgery, but even when orthopedic treatment is required, it should always be preceded and followed by rehabilitation.

Early diagnosis and treatment of children with skull deformations. The challenge of modern medicine.

Skull deformations affect approximately 45% of newborn babies, the most common ones being: plagiocephaly, brachycephaly and scaphocephaly. The first symptoms can be observed 4 to 8 weeks after birth. The causes of skull deformation in newborns can be divided into congenital ones and those acquired after birth. An increase in the incidence of acquired head deformations can be attributed to the "BACK TO SLEEP" campaign, carried out in 1992 by the American Academy of Pediatrics (AAP), which was aimed to reduce the frequency of sudden infant death syndrome (SIDS) by placing babies to sleep in the supine position. By the year 2000, the number of SIDS incidents had been significantly lowered, however, it seems that this improvement was achieved at the cost of an increased number of head deformations [3, 4, 5, 34]. Skull deformations, if left untreated, may have consequences for the future. Plagiocephalic deformations may be associated with delayed intellectual and motor development [2]. Early recognition of the condition and the appropriate classification of each skull deformation are crucial for the success of the treatment [8]. Treatment choice depends on the etiology of the problem and its severity, as well as on the age of the infant. Available options include training for the parents/caregivers, physical therapy, custom head orthosis and surgical intervention.

Fibromyalgia Syndrome - a multidisciplinary approach.

According to American College of Rheumatology fibromyalgia syndrome (FMS) is a common health problem characterized by widespread pain and tenderness. The pain and tenderness, although chronic, present a tendency to fluctuate both in intensity and location around the body. Patients with FMS experience fatigue and often have sleep disorders. It is estimated that FMS affects two to four percent of the general population. It is most common in women, though it can also occur in men. FMS most often first occur in the middle adulthood, but it can start as early as in the teen years or in the old age. The causes of FMS are unclear. Various infectious agents have recently been linked with the development of FMS. Some genes are potentially linked with an increased risk of developing FMS and some other health problems, which are common comorbidities to FMS. It is the genes that determine individual sensitivity and reaction to pain, quality of the antinociceptive system and complex biochemistry of pain sensation. Diagnosis and therapy may be complex and require cooperation of many specialists. Rheumatologists often make the diagnosis and differentiate FMS with other disorders from the rheumatoid group. FMS patients may also require help from the Psychiatric Clinic (Out-Patients Clinic) due to accompanying mental problems. As the pharmacological treatment options are limited and only complex therapy gives relatively good results, the treatment plan should include elements of physical therapy.

Pediatric rehabilitation in children with rare diseases--preliminary report.

As consultants in rehabilitation medicine and pediatrics sometimes we realize that most of our patients and their families are left unsupported and alone in terms of rehabilitation therapy during the treatment of their primary disease. Probably we forget that rehabilitation should be an integral part of every treatment process. In the case of rare diseases, their basic treatment should be carried out in speciality facilities (speciality hospitals) where personel and equipment are able to run the most effective treatment. Rehabilitation medicine offers full range of cheap, non-invasive diagnostic tools. Well-chosen equipment can help with physical therapy and allow the independence of the child and full social integration. In an era of rapid change in civilization and progress of science, professionals use new technologies more often. This also refers to the field of rehabilitation medicine. Exoskeletons and lokomats created nowadays allow the use of new technologies in rehabilitation treatment processes. For specific disorders effective treatment may include even virtual reality (VR). Early initiation of the rehabilitation process can improve the therapeutic effects and the overall outcome of the treatment, identifying the needs and realizing the available goals of rehabilitation that could build the sense of safety in the family and enhance their trust in the therapeutic team.

Arthrogryposis in infancy, multidisciplinary approach: case report.

BACKGROUND: Arthrogryposis multiplex congenita is an etiopathogenetically heterogeneous disorder characterised by non-progressive multiple intra-articular contractures, which can be recognised at birth. The frequency is estimated at 1 in 3,000 newborns. Etiopathogenesis of arthrogryposis is multifactorial. CASE PRESENTATION: We report first 26 weeks of life of a boy with severe arthrogryposis. Owing to the integrated rehabilitation approach and orthopaedic treatment a visible improvement in the range of motion as well as the functionality of the child was achieved. This article proposes a cooperation of various specialists: paediatrician, orthopaedist, specialist of medical rehabilitation and physiotherapist. CONCLUSIONS: Rehabilitation of a child with arthrogryposis should be early, comprehensive and multidisciplinary. Corrective treatment of knee and hip joints in infants with arthrogryposis should be preceded by the ultrasound control. There are no reports in the literature on the ultrasound imaging techniques which can be used prior to the planned orthopaedic and rehabilitative treatment in infants with arthrogryposis. The experience of our team indicates that such an approach allows to minimise the diagnostic errors and to maintain an effective treatment without the risk of joint destabilisation.