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Pediatric Arterial hypertension

Yuri Sonoda, Kenichiro Yamamura, Kanako Ishii, Kazuhiro Ohkubo, Kenji Ihara, Yasunari Sakai, Shouichi Ohga
Prostaglandin I2 (PGI2) causes hyperthyroidism, a critical complication in patients with pulmonary arterial hypertension (PAH). However, it remains unknown whether PGI2 may have unfavorable effects on thyroid functions in children with congenital portosystemic venous shunt syndrome (CPSVS). We present a boy with CPSVS who developed PAH at 7 years of age. During the PGI2 therapy, he experienced thyrotoxicosis at 17 years of age. The literature review showed that the past 12 patients with PAH (median 11 years of age) developed hyperthyroidism during 1 to 11 years of PGI2 treatments...
October 16, 2018: Journal of Clinical Research in Pediatric Endocrinology
T Ueno, S Hiwatashi, R Saka, H Yamanaka, Y Takama, Y Tazuke, K Bessho, S Kogaki, T Yonekura, H Okuyama
Pediatric living donor liver transplantation (LDLT) in patients with advanced portopulmonary hypertension (PoPH) is associated with poor prognoses. Recently, novel oral medications, including endothelin receptor antagonists (ERAs), phosphodiesterase 5 (PDE5) inhibitors, and oral prostacyclin (PGI2) have been used to treat PoPH. Pediatric patients with PoPH who underwent LDLT from 2006 to 2016 were enrolled. Oral pulmonary hypertension (PH) medication was administered to control pulmonary arterial pressure (PAP)...
March 15, 2018: Transplantation Proceedings
Ammar Al-Mashhadi, Michael Häggman, Göran Läckgren, Sam Ladjevardi, Tryggve Nevéus, Arne Stenberg, A Erik G Persson, Mattias Carlström
BACKGROUND: As much as 20% of all cases of hypertension are associated with kidney malfunctions. We have previously demonstrated in animals and in pediatric patients that hydronephrosis causes hypertension, which was attenuated by surgical relief of the ureteropelvic junction (UPJ) obstruction. This retrospective cohort study aimed to investigate: (1) the proposed link between hydronephrosis, due to UPJ obstruction, and elevated arterial pressure in adults; and (2) if elevated blood pressure in patients with hydronephrosis might be another indication for surgery...
October 8, 2018: Upsala Journal of Medical Sciences
Shinichi Takatsuki, Shun Yanai, Satoshi Ikehara, Tomotaka Nakayama, Horoyuki Matsuura
Syncope is more common in children with idiopathic pulmonary arterial hypertension (PAH) than in adults with PAH. Although syncope is associated with a poor prognosis in adult PAH, the clinical effects of syncopal events on disease severity and outcome in children have not been carefully investigated. This study assessed the prevalence of syncope in pediatric PAH and examined its clinical, hemodynamic, and prognostic importance. This retrospective study assessed clinical data, including syncope status, from 78 children (37 girls) with idiopathic and heritable PAH (median age at diagnosis, 11 years)...
October 5, 2018: Pediatric Cardiology
Eve Grillo Carvalho, Henrique Guarino Colli Peluso, Lorena Luana Batista, Cissa Santos Moreira, Juliana Suzano Moraes Protti, Maria Cristina Bento Soares, Aline de Freitas Suassuna Autran, Amanda Rocha Soares Almeida, Denise Cristina Rodrigues, Lívia Verônica Grillo Romano Bernardes, Luciana Pimenta de Paula
INTRODUCTION: The posterior reversible encephalopathy (PRES) syndrome encompasses a set of clinical-radiological findings associated with severe systemic arterial hypertension. This case report proposes to discuss the identification, diagnosis, and management of PRES in the pediatric population. CASE PRESENTATION: Female patient, 10 years old, admitted to the emergency room with complaint of oliguria and generalized edema. At the initial physical exam, the only alteration present was anasarca...
September 21, 2018: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
Hanno H Leuchte, Henrik Ten Freyhaus, Henning Gall, Michael Halank, Marius M Hoeper, Harald Kaemmerer, Christian Kähler, Gabriela Riemekasten, Silvia Ulrich, Martin Schwaiblmair, Ralf Ewert
In the summer of 2016, delegates from the German Respiratory Society, the German Society of Cardiology and the German Society of Pediatric Cardiology met in Cologne, Germany, to define consensus-based practice recommendations for the management of patients with pulmonary arterial hypertension (PAH). These recommendations were built on the 2015 European Pulmonary Hypertension guidelines and included new evidence, where available, and were last updated in the spring of 2018. This article focusses on the proposed risk stratification and assessment of disease progression in patients with pulmonary arterial hypertension (PAH), covering 3 parts: In part 1, methods and markers that are recommended to assess severity and progression of PAH are discussed and commented...
September 13, 2018: International Journal of Cardiology
Rohit S Loomba, Seth B Gray, Saul Flores
INTRODUCTION: Ketamine is a drug often used for procedural sedation or as adjunct agent for general sedation in children with congenital heart disease. In the clinical realm, there is often confusion regarding the effects of ketamine on hemodynamics, particularly pulmonary vascular resistance and systemic vascular resistance. We performed a meta-analysis of studies investigating the effects of ketamine on hemodynamics. METHODS: A systematic review was conducted to identify studies characterizing the hemodynamic effects of ketamine in children with congenital heart disease...
September 27, 2018: Congenital Heart Disease
Piers Blackett, Minu George, Don P Wilson
Pediatric lipid screening and management with the aim of reducing and preventing adult disease is an internationally accepted concept, and guidelines have been published in several countries. However, implementation by the practicing pediatric community in the United States has been less than expected and delays have been attributed to uncertainty among providers. Reduced screening rates have also been reported for conditions contributing to arterial wall pathology such as obesity, hypertension, and prediabetes despite accumulating evidence that detection and intervention can lead to risk reversal...
August 29, 2018: Journal of Clinical Lipidology
Li Jiang, Wei Sun, Kai Zhang, Bin Zhou, Xiangqing Kong
Sildenafil is a pulmonary artery hypertension (PH)-targeted drug that finds an increased indiscriminate use in children with PH secondary to congenital heart disease (CHD).We performed a meta-analysis to evaluate the effects of sildenafil on pediatric patients with PH secondary to CHD during perioperative period.PubMed, EMBASE, the Cochrane Library, and the Google Scholar were searched up to May 2016 for randomized controlled trials (RCTs) assessing the perioperative treatment of sildenafil in pediatric patients with PH secondary to CHD...
September 25, 2018: International Heart Journal
Tammo Delhaas, Yvette Koeken, Heiner Latus, Christian Apitz, Dietmar Schranz
Aims: To quantitatively evaluate the basic pathophysiological process involved in the creation of Eisenmenger syndrome in pediatric pulmonary arterial hypertension (PAH) patients by either atrial septostomy (AS) or Potts shunt (PS) as well as to predict the effects of AS or PS in future PAH patients. Methods: The multi-scale lumped parameter CircAdapt model of the cardiovascular system was used to investigate the effects of AS and PS on cardiovascular hemodynamics and mechanics, as well as on oxygen saturation in moderate to severe PAH...
2018: Frontiers in Physiology
Daisuke Yoshinaga, Shiro Baba, Takuya Hirata, Hiroyuki Fukushima, Masatsugu Hamaji, Akihiro Aoyama, Toyofumi F Chen-Yoshikawa, Hiroyuki Yamagishi, Hiroshi Date, Toshio Heike
Pediatric pulmonary hypertension after surgery for congenital heart disease is a significant complication. We present a case of living-donor lung transplantation for a 12-year-old girl with pulmonary hypertension after surgical repair of transposition of great arteries. Despite repairing the transposition of great arteries, her growth was severely restricted because of progressive pulmonary hypertension; thus, lung transplantation was discussed. Standard bilateral lobar transplantation seemed unfeasible due to oversized grafts, so we performed a single lobar transplantation...
September 6, 2018: General Thoracic and Cardiovascular Surgery
Marius M Hoeper, Christian Apitz, Ekkehard Grünig, Michael Halank, Ralf Ewert, Harald Kaemmerer, Hans-Joachim Kabitz, Christian Kähler, Hans Klose, Hanno Leuchte, Silvia Ulrich, Karen M Olsson, Oliver Distler, Stephan Rosenkranz, H Ardeschir Ghofrani
In the summer of 2016, delegates from the German Respiratory Society, the German Society of Cardiology and the German Society of Pediatric Cardiology met in Cologne, Germany, to define consensus-based practice recommendations for the management of patients with pulmonary arterial hypertension (PAH). These recommendations were built on the 2015 European Pulmonary Hypertension guidelines and included new evidence, where available. The treatment algorithm for PAH was modified based on the observation that there are now many patients diagnosed with IPAH who are at an advanced age and have significant cardiopulmonary comorbidities...
August 25, 2018: International Journal of Cardiology
Ekkehard Grünig, Nicola Benjamin, Ulrich Krüger, Harald Kaemmerer, Satenik Harutyunova, Karen M Olsson, Silvia Ulrich, Felix Gerhardt, Claus Neurohr, Armin Sablotzki, Michael Halank, Alberto M Marra, Hans-Joachim Kabitz, Günther Thimm, Klaus-Günther Fliegel, Hans Klose
In the summer of 2016, delegates from the German Respiratory Society, the German Society of Cardiology and the German Society of Pediatric Cardiology met in Cologne, Germany, to define consensus-based practice recommendations for the management of patients with pulmonary arterial hypertension (PAH). These recommendations were built on the 2015 European Pulmonary Hypertension guidelines aiming at their practical implementation, considering country-specific issues, and including new evidence, where available...
August 27, 2018: International Journal of Cardiology
Britta Höcker, Lutz T Weber, Ulrike John, Jens Drube, Henry Fehrenbach, Günter Klaus, Martin Pohl, Tomáš Seeman, Alexander Fichtner, Elke Wühl, Burkhard Tönshoff
BACKGROUND: Variable effects of steroid minimization strategies on blood pressure in pediatric renal transplant recipients have been reported, but data on the effect of steroid withdrawal on ambulatory blood pressure and circadian blood pressure rhythm have not been published so far. METHODS: In a prospective, randomized, multicenter study on steroid withdrawal in pediatric renal transplant recipients (n = 42) on cyclosporine, mycophenolate mofetil, and methylprednisolone, we performed a substudy in 28 patients, aged 11...
September 4, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Elizabeth J Thompson, Krystle Perez, Christoph P Hornik, P Brian Smith, Reese H Clark, Matthew Laughon
OBJECTIVE:  Pulmonary hypertension causes substantial morbidity and mortality in infants. Although Food and Drug Administration approved to treat pulmonary arterial hypertension in adults, sildenafil is not approved for infants. We sought to describe sildenafil exposure and associated diagnoses and outcomes in infants. STUDY DESIGN:  Retrospective cohort of neonates discharged from more than 300 neonatal intensive care units from 2001 to 2016. RESULTS:  Sildenafil was administered to 1,336/1,161,808 infants (0...
August 6, 2018: American Journal of Perinatology
Jordan D Awerbach, Richard A Krasuski, Michael G W Camitta
There are >1.4 million adult congenital heart disease (CHD; ACHD) patients living in the United States. Coronary artery disease (CAD) is at least as prevalent in ACHD patients as in the general population and has become a leading cause of their mortality. In the majority of cases, CAD in the ACHD population is driven by the presence of traditional cardiovascular disease (CVD) risk factors. 80% of ACHD patients have at least one CVD risk factor. Hypertension (HTN), obesity and physical inactivity are frequently seen in both pediatric and adult patients with CHD...
July 21, 2018: Progress in Cardiovascular Diseases
Na Zhu, Carrie L Welch, Jiayao Wang, Philip M Allen, Claudia Gonzaga-Jauregui, Lijiang Ma, Alejandra K King, Usha Krishnan, Erika B Rosenzweig, D Dunbar Ivy, Eric D Austin, Rizwan Hamid, Michael W Pauciulo, Katie A Lutz, William C Nichols, Jeffrey G Reid, John D Overton, Aris Baras, Frederick E Dewey, Yufeng Shen, Wendy K Chung
BACKGROUND: Pulmonary arterial hypertension (PAH) is a rare disease characterized by distinctive changes in pulmonary arterioles that lead to progressive pulmonary arterial pressures, right-sided heart failure, and a high mortality rate. Up to 30% of adult and 75% of pediatric PAH cases are associated with congenital heart disease (PAH-CHD), and the underlying etiology is largely unknown. There are no known major risk genes for PAH-CHD. METHODS: To identify novel genetic causes of PAH-CHD, we performed whole exome sequencing in 256 PAH-CHD patients...
July 20, 2018: Genome Medicine
Melanie J Dufva, Uyen Truong, Pawan Tiwari, Dunbar D Ivy, Robin Shandas, Vitaly O Kheyfets
The right ventricle and left ventricle are physically coupled through the interventricular septum. Therefore, changes in the geometry and mechanics of one ventricle can directly affect the function of the other. In treatment of pediatric pulmonary arterial hypertension, the left ventricle is often overlooked, with clinical focus primarily on improving right ventricular function. Pediatric pulmonary arterial hypertension represents a disease distinct from adult pulmonary arterial hypertension based on etiology and survival rates...
July 2018: Pulmonary Circulation
Michael Khoury, Philip R Khoury, Lawrence M Dolan, Thomas R Kimball, Elaine M Urbina
BACKGROUND AND OBJECTIVES: New pediatric hypertension definitions were recently published in a clinical practice guideline (CPG). We evaluated the impact of the CPG, compared with the previous guideline ("Fourth Report on the Diagnosis, Evaluation, and Treatment of High Blood Pressure in Children and Adolescents"), on the prevalence of hypertension and associations with target organ damage (TOD) in high-risk youth. METHODS: Participants (10-18 years old) undergoing an evaluation of the cardiovascular effects of obesity and type 2 diabetes mellitus in youth were studied...
August 2018: Pediatrics
Jacopo Burrello, Elvira M Erhardt, Gaelle Saint-Hilary, Franco Veglio, Franco Rabbia, Paolo Mulatero, Silvia Monticone, Fabrizio D'Ascenzo
Pharmacological treatment is indicated in children and adolescents with hypertension unresponsive to lifestyle modifications, but there is not enough evidence to recommend 1 class of antihypertensive drugs over others. We performed a network meta-analysis to compare the results of available randomized clinical trials on pharmacological treatment of pediatric hypertension. From a total of 554 potentially relevant studies, 13 randomized placebo-controlled clinical trials enrolling ≥50 patients and a follow-up ≥4 weeks were included...
August 2018: Hypertension
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