Safety and efficacy of the Amplatzer™ Trevisio™ intravascular delivery system: Post-approval study results.

BACKGROUND: The Amplatzer™ Trevisio™ Intravascular Delivery System (Trevisio DS; Abbott Laboratories, Chicago, IL, USA) facilitates the delivery of Amplatzer™ Occluders and features an ultraflexible tip, which improves assessment of occluder position before release. AIMS: To assess the safety and efficacy of the Trevisio DS for transcatheter closure of patent foramen ovale and atrial septal defect. METHODS: The Amplatzer™ Trevisio™ Intravascular Delivery System Post-Approval Study was a prospective, postmarket, single-arm, multicentre, observational study of the Trevisio DS. Enrolled patients were indicated for transcatheter closure of patent foramen ovale or atrial septal defect. In all procedures, the Trevisio DS was used to deliver Amplatzer™ Occluders. Technical success was defined as successful deployment and release of at least one occluder. Device- or procedure-related serious adverse events were tracked until discharge or day 7, whichever occurred earlier. RESULTS: The study enrolled 144 patients with patent foramen ovale and 107 patients with atrial septal defect at 22 European sites; 53 patients with atrial septal defect (49.6%) were aged<18years. The rate of technical success was 98.4% (97.2% for atrial septal defect, 99.3% for patent foramen ovale). There was one serious adverse event (0.4%), an acute periprocedural device embolization that occurred after occluder release in a patient with atrial septal defect; the device was retrieved percutaneously. This was determined by the implanter to be unrelated to the performance of the Trevisio DS. CONCLUSIONS: The Trevisio DS exhibited a high rate of technical success and an excellent safety profile during transcatheter closure of patent foramen ovale and atrial septal defect.

Polish recommendations for diagnosis and therapy of paediatric stroke.

Stroke remains one of the greatest health challenges worldwide, due to a high mortality rate and, despite great progress in its treatment, the significant disability that it causes. Studies conducted around the world show that the diagnosis of stroke in children is often significantly delayed. Paediatric ischaemic arterial stroke (PAIS) is not only a problem that varies greatly in frequency compared to the adult population, it is also completely different in terms of its risk factors, clinical course and outcome. The main reason for the lack of a rapid diagnosis of PAIS is a lack of access to neuroimaging under general anaesthesia. The insufficient knowledge regarding PAIS in society as a whole is also of great importance. Parents and carers of children should always bear in mind that paediatric age is not a factor that excludes a diagnosis of stroke. The aim of this article was to develop recommendations for the management of children with acute neurological symptoms suspected of ischaemic stroke and further treatment after confirmation of the ischaemic aetiology of the problem. These recommendations are based on current global recommendations for the management of children with stroke, but our goal was also to match them as closely as possible to the needs and technical diagnostic and therapeutic possibilities encountered in Poland. Due to the multifactorial problem of stroke in children, not only paediatric neurologists but also a neurologist, a paediatric cardiologist, a paediatric haematologist and a radiologist took part in the preparation of these recommendations.

Atrial Flow Regulator for Postcapillary Pulmonary Hypertension: First-in-Human Transcatheter AFR Device Implantations in RCM.

Restrictive cardiomyopathy (RCM) has a poor prognosis and limited treatment options apart from heart transplantation (HTx). We report on the first-in-human interventional atrial flow regulator (AFR) implantations in 3 children with RCM, leading to marked clinical and hemodynamic improvement. We propose the AFR as bridge to HTx or destination therapy in RCM. (Level of Difficulty: Advanced.).

Health-Related Quality of Life of Patients with Marfan Syndrome-Polish Study.

Background: Despite extensive knowledge about the quality of life of people suffering from rare diseases, data on patients with Marfan syndrome (MFS) are scarce and inconsistent. Hence, the problem of assessing the quality of life (QOL) and its relationship with the assessment of which ailments are the most burdensome for these patients is still open. Aim: Comparison of the quality of life of patients with MFS and determination as to which of the reported complaints in patients with MFS are related to the QOL of patients. Methods: The study included 35 patients with MFS and 35 healthy controls, matched for gender and age. In the study, the questionnaire of quality of life assessment SF-36 was used to assess the level of health-related quality of life, as well as an interview of the most severe symptoms reported by patients with MFS. Results: The level of the physical dimension of the QOL (p < 0.001) and limiting of roles due to physical health (p = 0.002), as well as the level of general index of the QOL (p < 0.001), were statistically significantly lower in MFS patients when compared to controls. People from both studied groups do not vary in the scope of pain, vitality, social functioning, limiting the roles due to emotional problems, and state of mind but also in the mental dimension of the health-related quality of life (HRQL). Additionally, there has been a correlation between HRQL and the subjective assessment of the effects of orthopedic, ophthalmic, and cardiological problems in life, as well as lower exercise tolerance in the evaluation of people with MFS and QOL in most areas. Conclusions: Patients with MFS present a reduced QOL in the areas of physical functioning, limiting roles due to physical health, general feeling of general health, the physical dimension of the HRQL, and the general index of the QOL; in these areas, they require careful evaluation, as well as medical and psychosocial assistance.

Case Report: Right Heart Failure Mistaken for Obesity-A Fault of Telemedicine.

As a result of the COVID-19 pandemic, telemedicine has become an important branch of healthcare worldwide. Apart from their undeniable advantages, the virtual visits lack physical examination, which can lead to important diagnostic mistakes. We hereby present a case of a pediatric patient whose weight gain, initially attributed to a sedentary lifestyle was, in fact, due to sub-acute right heart failure in the context of a ruptured sinus of Valsalva aneurysm. The condition was not diagnosed until the patient presented at the emergency. The boy was successfully treated with two percutaneous interventions and returned to his previous stature.

The Role of 3D Printing in Planning Complex Medical Procedures and Training of Medical Professionals-Cross-Sectional Multispecialty Review.

Medicine is a rapidly-evolving discipline, with progress picking up pace with each passing decade. This constant evolution results in the introduction of new tools and methods, which in turn occasionally leads to paradigm shifts across the affected medical fields. The following review attempts to showcase how 3D printing has begun to reshape and improve processes across various medical specialties and where it has the potential to make a significant impact. The current state-of-the-art, as well as real-life clinical applications of 3D printing, are reflected in the perspectives of specialists practicing in the selected disciplines, with a focus on pre-procedural planning, simulation (rehearsal) of non-routine procedures, and on medical education and training. A review of the latest multidisciplinary literature on the subject offers a general summary of the advances enabled by 3D printing. Numerous advantages and applications were found, such as gaining better insight into patient-specific anatomy, better pre-operative planning, mock simulated surgeries, simulation-based training and education, development of surgical guides and other tools, patient-specific implants, bioprinted organs or structures, and counseling of patients. It was evident that pre-procedural planning and rehearsing of unusual or difficult procedures and training of medical professionals in these procedures are extremely useful and transformative.

Anthropometric Determinants of Respiratory Sinus Arrhythmia in Children.

BACKGROUND: The study was conducted to investigate the implications of anthropometry in school-aged children on the degree of respiratory sinus arrhythmia observed in clinical settings. METHODS: In a cohort study, 626 healthy children (52% male) aged 10.8 ± 0.5 years attending primary school in a single town underwent a 12-lead electrocardiogram coupled with measurements of height, weight and blood pressure. Indices of respiratory sinus arrhythmia (pvRSA, RMSSD, RMSSDc) were derived from semi-automatic measurements of RR intervals. Height, weight, BMI, blood pressure as well as waist and hip circumferences were compared between subjects with rhythmic heart rate and respiratory sinus arrhythmia, and correlations between indices of sinus arrhythmia and anthropometry were investigated. RESULTS: Respiratory sinus arrhythmia was recognized in 43% of the participants. Subjects with sinus arrhythmia had lower heart rate (p < 0.001), weight (p = 0.009), BMI (p = 0.005) and systolic (p = 0.018) and diastolic (p = 0.004) blood pressure. There were important inverse correlations of heart rate and indices of sinus arrhythmia (r = -0.52 for pvRSA and r = -0.58 for RMSSD), but not the anthropometry. CONCLUSION: Lower prevalence of respiratory sinus arrhythmia among children with overweight and obesity is a result of higher resting heart rate observed in this population.

How to Distinguish Marfan Syndrome from Marfanoid Habitus in a Physical Examination-Comparison of External Features in Patients with Marfan Syndrome and Marfanoid Habitus.

Marfan Syndrome (MFS) is a systemic disorder caused by mutations in fibrillin-1. The most common cause of mortality in MFS is dissection and rupture of the aorta. Due to a highly variable and age-dependent clinical spectrum, the diagnosis of MFS still remains sophisticated. The aim of the study was to determine if there exist phenotypic features that can play the role of "red flags" in cases of MFS suspicion. The study population included 306 patients (199 children and 107 adults) who were referred to the Department of Pediatric Cardiology due to suspicion of MFS. All patients underwent complete clinical evaluation in order to confirm the diagnosis of MFS according to the modified Ghent criteria. MFS was diagnosed in 109 patients and marfanoid habitus in 168 patients. The study excluded 29 patients with other hereditary thoracic aneurysm syndromes. Comparative analysis between patients with Marfan syndrome and marfanoid habitus was performed. Symptoms with high prevalence and high positive likelihood ratio were identified (pectus carinatum, reduced elbow extension, hindfoot deformity, gothic palate, downslanting palpebral fissures, lens subluxation, myopia ≥ 3 dioptres remarkably high stature). The differentiation between patients with MFS and marfanoid body habitus is not possible by only assessing external body features; however, "red flags" could be helpful in the screening phase.

What Is the Importance of Electrocardiography in the Routine Screening of Patients with Repaired Tetralogy of Fallot?

BACKGROUND: In patients following complete repair of the tetralogy of Fallot, the duration of the QRS complex is associated with the size and mechanical function of the right ventricle, which are contemporarily assessed by cardiac magnetic resonance (CMR). METHODS: 38 patients aged 18.0-54.9 years (median age 24.9 years) who had undergone complete repair of the tetralogy of Fallot were examined using CMR and concomitant 24 h ambulatory electrocardiography monitoring. We used statistical analysis to investigate the correlations between electrocardiographic parameters (heart rate, HR; PQ interval, PQ; QRS duration, QRS; and corrected QT interval, QTc) and CMR results (right ventricular ejection fraction, RVEF; right ventricular end-diastolic volume index, RVEDVI; and right ventricular end-systolic volume index, RVESVI) for patients after early and late repair. RESULTS: The ECG-based parameters were not correlated with time since repair. There were significant correlations between QRS duration and RVEF (r = -0.61), RVEDVI (r = 0.56), and RVESVI (r = 0.54) for early operated patients but not for late-operated patients. No other substantial correlations were reported. CONCLUSION: Despite its role in screening for arrhythmias, electrocardiography has a limited role as a predictor of morphology and function of the right ventricle in patients after repair of the tetralogy of Fallot.

Case Report: Unexplained Fever and Chest Pain in a 5-Year-Old.

Pericarditis is a rare, but severe cause of chest pain in children that can easily be overlooked during routine diagnostics. Fibrinous pericarditis was recognized in a 5-year old patient who presented with fever and chest pain. Despite thorough diagnostics, no evident etiological factor was found. Furthermore, the disease was unresponsive to broad-spectrum antibiotics and NSAIDs, however oral prednisone was found to cause rapid improvement in the patients' condition. It was presumed that the patient's condition was caused by a blunt trauma to the chest experienced 3 days prior to the onset of symptoms. In an 8-month follow-up the patient remains in good overall condition and no recurrences were observed.

Respiratory Sinus Arrhythmia in Children-Predictable or Random?

Background: Respiratory sinus arrhythmia (RSA) is associated with better health in children. Aim: The study was conducted to analyze the trajectory of RSA in 10-year-olds. Methods: A follow-up study on 120 healthy children (62 boys) aged 10.7 ± 0.5 years consisted of a standard 12-lead electrocardiogram, measurements of height, weight and blood pressure. The protocol was repeated after 3 years. Assessment of RSA based on semi-automatic measurements of RR intervals included: the difference between the longest and shortest RR interval duration (pvRSA), the root mean square of differences between successive RR intervals (RMSSD), the standard deviation of the RR interval length (SDNN) and their equivalents corrected for heart rate (RMSSDc and SDNNc). Results: A the first visit 61.7% of children presented with RSA; 51.7% 3 years later. 23.3% of them had RSA only on the first examination; 13.3% only on the second one. The pvRSA, RMSSD, and SDNN measured in 2019 did not differ significantly from their 2016 equivalents (p > 0.05). The decline in RSA defined by RMSSD was noted in 52.5% of children and in 54.2% when defined by SDNN. The corrected values decreased in 68.3 and 64.2% of the participants for RMSSDc and SDNNc, respectively. The students with RSA at both visits had lower heart rate (p < 0.001) and systolic blood pressure (p = 0.010) compared to those with rhythmic electrocardiograms. Conclusions: RSA in children is changeable, though its measurable indices should be adjusted to heart rate.

Families of people with Marfan syndrome. The relationship between the functioning of the family system and the life quality of the affected persons. / Rodzina osoby z zespolem Marfana. Zwiazki funkcjonowania systemu rodzinnego z jakoscia zycia osoby chorej.

OBJECTIVES: The purpose of the study was to analyze the functioning of afamily as perceived by a person with Marfan syndrome and to look for relationships between the characteristics of the system and the overall quality of life of the ill. METHODS: Participants included 33 individuals with Marfan syndrome and 33 individuals without chronic illness. We used the Family Evaluation Scale - the Polish adaptation of the FACES-IVby D.H. Olson and the Satisfaction with Life Scale (SWLS) by Diener, Emmons, Larson and Griffin. RESULTS: People with Marfan syndrome perceive their families as significantly less coherent and significantly more disengaged than people without chronic illness. This family system of people with Marfan syndrome can be characterized by low scores on the "Cohesion" and "Flexibility" and high scores on the other four scales showing the level of imbalance of the family as a system, which makes this family profile similar to an ?unbalanced' system. Life satisfaction of people with Marfan syndrome correlated positively with "Cohesion", "Flexibility" and "Family Satisfaction" as features of family system perceived by them. CONCLUSIONS: The obtained results confirm the importance of supporting families of people with Marfan syndrome and specialist help aimed at dealing with emotional burden related to the health of the patient.

Percutaneous closure of atrial septal defect: a consensus document of the joint group of experts from the Association of Cardiovascular Interventions and the Grown-Up Congenital Heart Disease Section of the Polish Cardiac Society.

Atrial septal defect is the most common congenital heart lesion in adults. Although atrial septal defect closure is recommended in those with right heart enlargement or paradoxical embolism, data informing such indications in adults are quite limited. This population has many unique characteristics and needs. In recent years, significant progress has been made with regard to diagnostic modalities that facilitate the diagnostic workup of these patients. However, the decision­making process, especially in selected adult patients population (advanced age, various concomitant diseases, poor LV function [both systolic and diastolic], pulmonary hypertension, concomitant arrhythmias or multiple defects, or deficient rims) is still not easy. Available data are predominantly focused on imaging endpoints and short­term morbidity and mortality rather than long­­term. The evidence base for outcomes with or without defect closure comes from various studies with different observation periods. Moreover, the clinical experience in diagnosing and treating that subgroup of patients is inhomogeneous between individual physicians (cardiologists, imaging specialists, operators) and between small and large experience centers. In the view of the above, the joint group of experts from the Association of Cardiovascular Interventions and the Grown­Up Congenital Heart Disease Section of the Polish Cardiac Society developed the following consensus opinion in order to standardize the principles of diagnosis, indications for treatment, methods of performing procedures, and tenets of postoperative care in Poland.