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Paediatric Pulmonary Hypertension

Denise C Joffe, Thomas K Jones, Mark Reisman, Elizabeth Perpetua, Yuk Law, Andreas Schuler, G Burkhard Mackensen
We present the primary report of a paediatric patient who had placement of the MitraClip device for severe functional mitral regurgitation. The patient was a 14-year-old boy with symptomatic end-stage non-compaction type cardiomyopathy secondary to a mitochondrial cytopathy. He had severe mitral regurgitation, tricuspid valve regurgitation, a severely dilated LV with apical non-compaction, severe LV dysfunction and severe pulmonary hypertension. Despite optimal medical therapy he developed progressive symptoms of congestive heart failure and he was not a candidate for an assist device or cardiac transplantation...
October 10, 2016: EuroIntervention
Kathryn Browning Carmo, Tracey Lutz, Andrew Berry, Martin Kluckow, Nick Evans
AIM: To determine the role of clinician performed ultrasound (CPU) during the retrieval and transport of critically ill term and near term newborns. METHODS: A neonatologist with portable ultrasound accompanied a sample of newborn retrievals to perform cardiac and cerebral ultrasound before and after transportation. RESULTS: A total of fifty-five babies were studied. Median birthweight: 3350 g (2220-5030 g). CPU led to a change in the planned receiving hospital in ten babies...
December 2016: Acta Paediatrica
Marilyne Levy, Mélanie Eyries, Isabelle Szezepanski, Magalie Ladouceur, Sophie Nadaud, Damien Bonnet, Florent Soubrier
The prevalence of germline mutations in paediatric pulmonary hypertension (PH) is poorly documented. The objective of this study was to determine the mutation frequency in PH genes in a paediatric cohort and describe the clinical characteristics of mutation carriers.The study involved 66 index cases with PH: 35 children with idiopathic pulmonary arterial hypertension (IPAH); five children with familial PAH (FPAH); three children with pulmonary veno-occlusive disease (PVOD); and 23 children with PAH associated with congenital heart disease (APAH-CHD)...
October 2016: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
Sainath Raman, Samiran Ray, Mark J Peters
Purpose. Administration of supplemental oxygen is common in paediatric intensive care. We explored the current practice of oxygen administration using a case vignette in paediatric intensive care units (PICU) in the united kingdom. Methods. We conducted an online survey of Paediatric Intensive Care Society members in the UK. The survey outlined a clinical scenario followed by questions on oxygenation targets for 5 common diagnoses seen in critically ill children. Results. Fifty-three paediatric intensive care unit members from 10 institutions completed the survey...
2016: Critical Care Research and Practice
George T Nicholson, Michael S Kelleman, Caridad M De la Uz, Ricardo H Pignatelli, Nancy A Ayres, Christopher J Petit
OBJECTIVE: Shone's syndrome is a complex consisting of mitral valve stenosis in addition to left ventricle outflow obstruction. There are a few studies evaluating the long-term outcomes in this population. We sought to determine the long-term outcomes in our paediatric population with Shone's syndrome and the factors associated with left heart growth. METHODS: All patients diagnosed with Shone's syndrome with biventricular circulation treated between 1978 and 2010 were reviewed...
July 26, 2016: Cardiology in the Young
Michal Schäfer, D Dunbar Ivy, Alex J Barker, Vitaly Kheyfets, Robin Shandas, Steven H Abman, Kendall S Hunter, Uyen Truong
AIMS: Paediatric pulmonary arterial hypertension (PAH) is manifested as increased arterial pressure and vascular resistive changes followed by progressive arterial stiffening. The aim of this study was to characterize regional flow haemodynamic patterns and markers of vascular stiffness in the proximal pulmonary arteries of paediatric PAH patients, and to explore the association with right ventricular (RV) function. METHODS AND RESULTS: Forty paediatric PAH patients and 26 age- and size-matched controls underwent cardiac magnetic resonance studies in order to compute time-resolved wall shear stress metrics, oscillatory shear index (OSI), and vascular strain as measured by relative area change (RAC), and RV volumetric and functional parameters...
July 21, 2016: European Heart Journal Cardiovascular Imaging
Christelle Joffre, Fabrice Lesage, Olivier Bustarret, Philippe Hubert, Mehdi Oualha
AIM: To investigate clinical course and mortality-associated factors in children with Down syndrome (DS) managed in a medical paediatric intensive care unit. METHODS: A single-centre, retrospective study conducted between 2001 and 2010 in DS children aged 1 month to 16 years. RESULTS: Sixty-six patients with a median age of 24 months (1-192) and a male/female ratio of 1.5 were analysed. Patients presented with history of congenital heart disease (n = 52, 78...
June 2016: Journal of Paediatrics and Child Health
Jamie N Colombo, Ricardo A Samson, Santiago O Valdes, Omar Meziab, David Sisk, Scott E Klewer
Sudden cardiac arrest is a rare but devastating cause of death in young adults. Electrocardiograms may detect many causes of sudden cardiac arrest, but are not routinely included in pre-athletic screening in the United States of America partly because of high rates of false-positive interpretation. To improve electrocardiogram specificity for identifying cardiac conditions associated with sudden cardiac arrest, an expert panel developed refined criteria known as the Seattle Criteria. Ours is the first study to compare standard electrocardiogram criteria with Seattle Criteria in 11- to 13-year-olds...
June 20, 2016: Cardiology in the Young
Daynia E Ballot, Victor A Davies, Peter A Cooper, Tobias Chirwa, Andrew Argent, Mervyn Mer
OBJECTIVE: Report on survival to discharge of children in a combined paediatric/neonatal intensive care unit (PNICU). DESIGN AND SETTING: Retrospective cross-sectional record review. PARTICIPANTS: All children (medical and surgical patients) admitted to PNICU between 1 January 2013 and 30 June 2015. OUTCOME MEASURES: Primary outcome-survival to discharge. Secondary outcomes-disease profiles and predictors of mortality in different age categories...
2016: BMJ Open
Farhana Akter, Gerry Coghlan, Achala de Mel
Nitric oxide (NO) has a significant role in modulating the respiratory system and is being exploited therapeutically. Neonatal respiratory failure can affect around 2% of all live births and is responsible for over one third of all neonatal mortality. Current treatment method with inhaled NO (iNO) has demonstrated great benefits to patients with persistent pulmonary hypertension, bronchopulmonary dysplasia and neonatal respiratory distress syndrome. However, it is not without its drawbacks, which include the need for patients to be attached to mechanical ventilators...
August 2016: Therapeutic Advances in Cardiovascular Disease
Amanda Hauck, Ruixin Guo, D Dunbar Ivy, Adel Younoszai
AIMS: Tricuspid annular plane systolic excursion (TAPSE) is a measure of right ventricular (RV) longitudinal function that correlates with functional status and mortality in adults with pulmonary hypertension (PH). The diagnostic and predictive value of TAPSE in children with PH has not been fully examined. We aimed to define TAPSE across aetiologies of paediatric PH and assess the correlation between TAPSE and measures of disease severity. METHODS AND RESULTS: TAPSE measurements were obtained in 84 children and young adults undergoing treatment for PH and 315 healthy children to establish z-scores at moderate altitude for comparison...
April 20, 2016: European Heart Journal Cardiovascular Imaging
Georg Hansmann, Christian Apitz, Hashim Abdul-Khaliq, Tero-Pekka Alastalo, Phillip Beerbaum, Damien Bonnet, Karl-Otto Dubowy, Matthias Gorenflo, Alfred Hager, Anne Hilgendorff, Michael Kaestner, Martin Koestenberger, Juha W Koskenvuo, Rainer Kozlik-Feldmann, Titus Kuehne, Astrid E Lammers, Heiner Latus, Ina Michel-Behnke, Oliver Miera, Shahin Moledina, Vivek Muthurangu, Joseph Pattathu, Dietmar Schranz, Gregor Warnecke, Peter Zartner
UNLABELLED: : The European Paediatric Pulmonary Vascular Disease (PVD) Network is a registered, non-profit organisation that strives to define and develop effective, innovative diagnostic methods and treatment options in all forms of paediatric pulmonary hypertensive vascular disease, including specific forms such as pulmonary arterial hypertension (PAH)-congenital heart disease, pulmonary hypertension (PH) associated with bronchopulmonary dysplasia, persistent PH of the newborn, and related cardiac dysfunction...
May 2016: Heart: Official Journal of the British Cardiac Society
Georg Hansmann, Christian Apitz
Treatment of children and adults with pulmonary hypertension (PH) with or without cardiac dysfunction has improved in the last two decades. The so-called pulmonary arterial hypertension (PAH)-specific medications currently approved for therapy of adults with PAH target three major pathways (endothelin, nitric oxide, prostacyclin). Moreover, some PH centres may use off-label drugs for compassionate use. Pulmonary hypertensive vascular disease (PHVD) in children is complex, and selection of appropriate therapies remains difficult...
May 2016: Heart: Official Journal of the British Cardiac Society
Michael Kaestner, Dietmar Schranz, Gregor Warnecke, Christian Apitz, Georg Hansmann, Oliver Miera
Acute pulmonary hypertension (PH) complicates the course of several cardiovascular, pulmonary and other systemic diseases in children. An acute rise of RV afterload, either as exacerbating chronic PH of different aetiologies (eg, idiopathic pulmonary arterial hypertension (PAH), chronic lung or congenital heart disease), or pulmonary hypertensive crisis after corrective surgery for congenital heart disease, may lead to severe circulatory compromise. Only few clinical studies provide evidence on how to best treat children with acute severe PH and decompensated RV function, that is, acute RV failure...
May 2016: Heart: Official Journal of the British Cardiac Society
Anne Hilgendorff, Christian Apitz, Damien Bonnet, Georg Hansmann
Persistent pulmonary hypertension of the newborn (PPHN) is the most common neonatal form and mostly reversible after a few days with improvement of the underlying pulmonary condition. When pulmonary hypertension (PH) persists despite adequate treatment, the severity of parenchymal lung disease should be assessed by chest CT. Pulmonary vein stenosis may need to be ruled out by cardiac catheterisation and lung biopsy, and genetic workup is necessary when alveolar capillary dysplasia is suspected. In PPHN, optimisation of the cardiopulmonary situation including surfactant therapy should aim for preductal SpO2between 91% and 95% and severe cases without post-tricuspid-unrestrictive shunt may receive prostaglandin E1 to maintain ductal patency in right heart failure...
May 2016: Heart: Official Journal of the British Cardiac Society
Rainer Kozlik-Feldmann, Georg Hansmann, Damien Bonnet, Dietmar Schranz, Christian Apitz, Ina Michel-Behnke
Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a complex disease that presents with a broad spectrum of morphological and haemodynamic findings of varying severity. Recently, the aspect of paediatric pulmonary hypertensive vascular disease (PPHVD) has been introduced to expand the understanding of the full spectrum of pulmonary hypertension and increased pulmonary vascular resistance. Evaluation and treatment of PAH-CHD/PPHVD-CHD can be divided into in different topics...
May 2016: Heart: Official Journal of the British Cardiac Society
Joseph Pattathu, Matthias Gorenflo, Anne Hilgendorff, Juha W Koskenvuo, Christian Apitz, Georg Hansmann, Tero-Pekka Alastalo
Childhood-onset pulmonary arterial hypertension (PAH) is considered complex and multifactorial, with relatively poor estimates of the natural history of the disease. Strategies allowing earlier detection, establishment of disease aetiology together with more accurate and sensitive biomarkers could enable better estimates of prognosis and individualise therapeutic strategies. Evidence is accumulating that genetic defects play an important role in the pathogenesis of idiopathic and hereditary forms of PAH. Altogether nine genes have been reported so far to be associated with childhood onset PAH suggesting that comprehensive multigene diagnostics can be useful in the assessment...
May 2016: Heart: Official Journal of the British Cardiac Society
Heiner Latus, Titus Kuehne, Philipp Beerbaum, Christian Apitz, Georg Hansmann, Vivek Muthurangu, Shahin Moledina
Childhood pulmonary hypertension (PH) is a heterogenous disease associated with considerable morbidity and mortality. Invasive assessment of haemodynamics is crucial for accurate diagnosis and guidance of medical therapy. However, adequate imaging is increasingly important in children with PH to evaluate the right heart and the pulmonary vasculature. Cardiac MR (CMR) and computed tomography (CT) represent important non-invasive imaging modalities that may enable comprehensive assessment of right ventricular (RV) function and pulmonary haemodynamics...
May 2016: Heart: Official Journal of the British Cardiac Society
Christian Apitz, Georg Hansmann, Dietmar Schranz
Invasive assessment of haemodynamics (ventricular, pulmonary) and testing of acute vasoreactivity in the catheterisation laboratory remain the gold standard for the diagnosis of pulmonary hypertension (PH) and pulmonary hypertensive vascular disease. However, these measurements and the interpretation thereof are challenging due to the heterogeneous aetiology of PH in childhood and potentially confounding factors in the catheterisation laboratory. Patients with pulmonary arterial hypertension (PAH) associated with congenital heart disease who have a cardiovascular shunt need to undergo a completely different catheterisation approach than those with idiopathic PAH lacking an anatomical cardiovascular defect...
May 2016: Heart: Official Journal of the British Cardiac Society
Martin Koestenberger, Christian Apitz, Hashim Abdul-Khaliq, Georg Hansmann
Transthoracic echocardiography (TTE) is a useful method for non-invasive screening of patients at risk of pulmonary hypertension (PH). Since TTE often serves as the initial study before invasive cardiac catheterisation, misinterpretation of TTE variables may lead to missed or delayed diagnosis with devastating consequences for the patients, or unnecessary invasive diagnostics that have inheriting risks. Due to the heterogeneous anatomy in congenital heart disease, particularly the assessment of myocardial function in children with PH is challenging...
May 2016: Heart: Official Journal of the British Cardiac Society
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