Publications by authors named "Humpl T"

Background/objectives: Parents of premature infants experience depression, anxiety, post-traumatic stress disorder, and increased stress, which can negatively impact parent-infant relationships and infant development. To reduce negative consequences and optimally support families, we developed the Transition to Home model (TtH). In this randomized controlled pilot trial (RCT), the feasibility of performing an experimental study to analyse the effects of TtH on parental mental health over time was evaluated.

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Despite exercise intolerance being predictive of outcomes in pulmonary arterial hypertension (PAH), its underlying cardiac mechanisms are not well described. The aim of the study was to explore the biventricular response to exercise and its associations with cardiorespiratory fitness in children with PAH. Participants underwent incremental cardiopulmonary exercise testing and simultaneous exercise echocardiography on a recumbent cycle ergometer.

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Article Synopsis
  • - The study examines a modified clinical classification for pediatric patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), focusing on conditions like atrial septal defects (ASD) and transposition of the great arteries (TGA) using data from the TOPP-1 registry.
  • - 223 out of 531 patients in the registry had PAH-CHD, categorized into groups based on their conditions; 193 were classified, revealing a distribution across predefined categories without any significant survival differences between the groups compared to idiopathic PAH patients.
  • - The conclusions suggest that the modified classification better suits pediatric PAH-CHD cases, though it indicates that some patients without shunts are still un
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Background And Aims: The Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension (TOPP) registry is a global network established to gain insights into the disease course and long-term outcomes of paediatric pulmonary arterial hypertension (PAH). Previously published cohorts in paediatric PAH are obscured by survival bias due to the inclusion of both prevalent (previously diagnosed) and incident (newly diagnosed) patients. The current study aims to describe long-term outcome and its predictors in paediatric PAH, exclusively of newly diagnosed patients.

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There are few programs available aimed at preventing short- and long-term negative consequences after preterm birth and covering the entire care continuum. The "Transition to Home (TtH)" model is such a program, offering structured, individual support for families with preterm infants before and after hospital discharge. This study gathers and examines the parents' views of receiving support from an interprofessional team under the TtH model of care during hospitalization and after discharge.

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Pulmonary veno-occlusive disease (PVOD) is a rare disease leading to pulmonary hypertension and potentially death related to right heart failure and/or respiratory insufficiency. Clinical symptoms are heterogenous and nonspecific: fatigue, decreased exercise tolerance, shortness of breath on exertion, cough, dizziness, chest pain with exercise, palpitations, syncope, as well as nonspecific symptoms such as headache, poor appetite, pallor or perioral cyanosis. Mutations in the (eukaryotic translation initiation factor 2-alpha kinase 4) have been recently described, other risk factors include exposure to organic solvent and trichloroethylene, tobacco exposure and chemotherapy.

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Background: Diastolic pulmonary arterial pressure (dPAP) is regarded to be less sensitive to flow metrics as compared to mean PAP (mPAP), and was therefore proposed for the assessment of a precapillary component in patients with postcapillary pulmonary hypertension (PH). To analyze the diagnostic and prognostic impact of dPAP in patients with pure precapillary PH, we purposed to compare the correlation between dPAP and mPAP, as well as hemodynamically-derived calculations [ratio of PAP to systemic arterial pressure (PAP/SAP), pulmonary vascular resistance index (PVRI), transpulmonary gradient (TPG)], using both dPAP and mPAP, at rest and during acute vasoreactivity testing (AVT) in children with idiopathic or heritable pulmonary arterial hypertension (IPAH/HPAH). Furthermore, we aimed to assess the association of these metrics (at baseline and changes after AVT) with transplant-free survival.

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Background: Ventricular assist devices (VAD) are increasingly used in patients with end-stage heart failure due to acquired heart disease. Limited data exists on the use and outcome of this technology in children.

Methods: All children (<18 years of age) with VAD support included in the German National Register for Congenital Heart Defects were identified and data on demographics, underlying cardiac defect, previous surgery, associated conditions, type of procedure, complications and outcome were collected.

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Article Synopsis
  • * Out of the children studied, 15% died in-hospital postprocedure, while 20% of those successfully discharged either died or underwent lung transplantation within an average follow-up of 3.1 years.
  • * Significant clinical improvements were observed in children after the procedure, including better functional ability and a majority being able to stop prostacyclin infusion, but those with severe complications prior to surgery showed poor outcomes.
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Background: Families with preterm infants find life after hospital discharge challenging and need tailored support to thrive. The "Transition to Home (TtH)"-model offers structured, individual support for families with preterm infants before and after hospital discharge. TtH improves parental mental health and competence, promotes child development and fosters interprofessional collaboration (IPC).

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Background: Vertical right axillary minithoracotomy (VRAMT) represents a minimally invasive and cosmetically attractive alternative for selected congenital heart defects. We report our institutional experience with VRAMT, especially regarding the performance of percutaneous femoral venous access to establish extracorporeal circulation in this pediatric population.

Methods: A retrospective single-center analysis was made of children to 16 years of age who underwent corrective cardiac surgery using VRAMT over a period of 5 years.

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Pulmonary vein stenosis is a rare and poorly understood condition causing obstruction of the large pulmonary veins and of blood flow from the lungs to the left atrium. This results in elevated pulmonary venous pressure and pulmonary edema, pulmonary hypertension, potentially cardiac failure, and death. Clinical signs of the disease include failure to thrive, increasingly severe dyspnea, hemoptysis, respiratory difficulty, recurrent respiratory tract infections/pneumonia, cyanosis, and subcostal retractions.

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Objectives: Cardiac catheterisation is commonly used for diagnosis and therapeutic interventions in paediatric cardiology. The inherent risk of the procedure can result in unanticipated admissions to critical care. Our goals were to provide a qualitative description of characteristics and evaluation of children admitted unexpectedly to the cardiac critical care unit (CCCU).

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Background: Composite clinical worsening (cCW) outcomes might allow measurement of disease progression in paediatric pulmonary arterial hypertension (PAH). This TOPP registry analysis investigated three cCW outcomes and their predictive strength for lung transplantation/death.

Methods: Patients ≤17 years with idiopathic/familial PAH or PAH-associated congenital heart disease diagnosed ≤3 months before enrolment were included.

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Objectives: Publications on the paediatric Berlin Heart EXCOR ventricular assist device have revealed that low body weight <10 kg is a significant risk factor for mortality with children weighing <5 kg being at the highest risk. However, these studies are limited to implantation periods prior to 2011. Since then, progress has been made in the optimization of patient selection and management.

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Acquired von Willebrand syndrome (AVWS) is reported in high-flow high-shear congenital cardiac disorders. We hypothesized that the narrowed pulmonary vasculature in idiopathic pulmonary arterial hypertension (IPAH) may induce AVWS. We conducted a cross-sectional evaluation of children with IPAH.

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Purpose Of Review: Pulmonary vascular disease (PVD) complicates the course of many cardiovascular, pulmonary and other systemic diseases in children. The physiological sequelae (pulmonary hypertension and elevated pulmonary vascular resistance) can overwhelm the right ventricle and lead to circulatory collapse. Despite the common end-point, the preceding pathophysiology is complex and variable and requires a tailored approach to diagnosis and management.

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Unlabelled: Prenatal observed/expected lung-to-head ratio (O/E LHR) by ultrasound correlates with postnatal mortality for congenital diaphragmatic hernia (CDH) patients. The aim of this study is to determine if O/E LHR correlates with pulmonary hypertension (PH) outcomes for CDH patients.

Methods: A single center retrospective chart review was performed for CDH neonates from January 1, 2006, to December 31, 2015, (REB #1000053124) to include prenatal O/E LHR, liver position, first arterial blood gas, repair type, echocardiogram (ECHO), and lung perfusion scan (LPS) results up to 5years of age.

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Bronchopulmonary dysplasia, the most common pulmonary complication of extremely preterm (EPT) birth, has longstanding multiorgan repercussions, with increasing reports of emphysema and cardiac disease in early adulthood. There are currently no clear recommendations pertaining to best practices for optimal multidisciplinary cardiorespiratory follow-up of EPT children. We report the outcomes of a 2-day consensus workshop involving a Canadian panel of 31 multidisciplinary experts with the goal of improvement and standardization of the cardiopulmonary follow-up care of EPT infants (i.

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A chimney femoral artery graft for peripheral extracorporeal membrane oxygenation can potentially cause hyperperfusion and subsequent venous congestion in the ipsilateral leg, especially in the context of septic shock and higher flow requirement. This report describes a novel technique to use an additional leg venous cannula to avoid leg congestion as well as to achieve higher total flow.

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Central sleep apnea (CSA) and periodic breathing are unusual findings described in pediatric patients with congestive heart failure. However, CSA has not been reported in children with pulmonary hypertension. We hereby report on a 10-year-old girl with idiopathic pulmonary arterial hypertension who had frequent central events in a periodic breathing fashion seen in her polysomnography, which was normalized following medical treatment leading to improvement of the pulmonary pressures.

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