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Pulmonary arterial hypertension AND congenital heart disease AND pediatric

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https://www.readbyqxmd.com/read/28702718/single-center-experience-using-selexipag-in-a-pediatric-population
#1
Roberto Gallotti, Diana E Drogalis-Kim, Gary Satou, Juan Alejos
Pulmonary arterial hypertension (PAH) is a rare and progressive disorder. Current treatment in the pediatric population includes phosphodiesterase 5 inhibitors (PDE-5i), endothelin receptor antagonists (ERA), and both inhaled and intravenous prostacyclin pathway agonists. As of December 22, 2015 the first oral prostacyclin pathway agonist, selexipag (Uptravi(®)), was FDA approved in the US. In this case series, we discuss our single-center experience using selexipag in a pediatric population, composed of both patients with idiopathic PAH, and patients with congenital heart disease and PAH...
July 13, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28701597/randomized-comparative-study-of-intravenous-infusion-of-three-different-fixed-doses-of-milrinone-in-pediatric-patients-with-pulmonary-hypertension-undergoing-open-heart-surgery
#2
Neeraj Kumar Barnwal, Sanjeeta Rajendra Umbarkar, Manjula Sudeep Sarkar, Raylene J Dias
BACKGROUND: Pulmonary hypertension secondary to congenital heart disease is a common problem in pediatric patients presenting for open heart surgery. Milrinone has been shown to reduce pulmonary vascular resistance and pulmonary artery pressure in pediatric patients and neonates postcardiac surgery. We aimed to evaluate the postoperative outcome in such patients with three different fixed maintenance doses of milrinone. METHODOLOGY: Patients were randomized into three groups...
July 2017: Annals of Cardiac Anaesthesia
https://www.readbyqxmd.com/read/28506578/-congenital-heart-disease-in-adolescents-and-adults-management-in-a-general-cardiology-department-in-senegal
#3
A Mbaye, M Bodian, A A Ngaïdé, H Abdourafiq, M C B O Leye, S Savodogo, F Aw, M Ndiaye, I Kouamé, K Babaka, M Dioum, N D Gaye, S A Sarr, M B Ndiaye, A D Kane, A Kane
BACKGROUND: Congenital heart diseases in adults include malformations treated in childhood that decompensate secondarily and those asymptomatic at birth, appear later. This study aims to identify congenital heart diseases in adults in general cardiology department of Senegal and to assess clinical presentations, treatment and outcomes. METHODS: We conducted a cross-sectional and descriptive study based on the records of patients aged at least 16 years and followed for congenital heart disease in the cardiology department of the General Hospital of Grand-Yoff in Dakar between May 2003 and March 2015...
May 12, 2017: Annales de Cardiologie et D'angéiologie
https://www.readbyqxmd.com/read/28125022/pediatric-exercise-testing-value-and-implications-of-peak-oxygen-uptake
#4
REVIEW
Paolo T Pianosi, Robert I Liem, Robert G McMurray, Frank J Cerny, Bareket Falk, Han C G Kemper
Peak oxygen uptake (peak V ˙ O 2 ) measured by clinical exercise testing is the benchmark for aerobic fitness. Aerobic fitness, estimated from maximal treadmill exercise, is a predictor of mortality in adults. Peak V ˙ O 2 was shown to predict longevity in patients aged 7-35 years with cystic fibrosis over 25 years ago. A surge of exercise studies in young adults with congenital heart disease over the past decade has revealed significant prognostic information. Three years ago, the first clinical trial in children with pulmonary arterial hypertension used peak V ˙ O 2 as an endpoint that likewise delivered clinically relevant data...
January 24, 2017: Children
https://www.readbyqxmd.com/read/28072745/safety-and-efficacy-of-chloral-hydrate-for-conscious-sedation-of-infants-in-the-pediatric-cardiovascular-intensive-care-unit
#5
Mei-Lian Chen, Qiang Chen, Fan Xu, Jia-Xin Zhang, Xiao-Ying Su, Xiao-Zhen Tu
This study evaluates the safety and efficacy of chloral hydrate administration for the conscious sedation of infants in the pediatric cardiovascular intensive care unit (PCICU).We conducted a retrospective review of the charts of 165 infants with congenital heart disease who received chloral hydrate in our PCICU between January 2014 and December 2014. Chloral hydrate was administered orally or rectally to infants using doses of 50 mg/kg. We collected and analyzed relevant clinical parameters.The overall length of time to achieve sedation was ranged from 5 to 35 min (10...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27801982/risk-factors-for-pulmonary-arterial-hypertension-in-children-and-young-adults
#6
Estelle Naumburg, Lars Söderström, Daniel Huber, Inge Axelsson
OBJECTIVES: Pulmonary hypertension (PH) has been linked to preterm birth explained by congenital heart defects and pulmonary diseases. WORKING HYPOTHESIS: Other factors may influence the risk of PH among adolescences and children born premature. STUDY DESIGN: This national registry-based study assess risk of PH following premature birth adjusted for known risk factors. PATIENT-SUBJECT SELECTION AND METHODOLOGY: All cases born 1993-2010, identified by diagnostic codes applicable to PH and retrieved from the Swedish Registry of Congenital Heart Disease (N = 67)...
May 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27770446/the-role-of-genetics-in-pulmonary-arterial-hypertension
#7
REVIEW
Lijiang Ma, Wendy K Chung
Group 1 pulmonary hypertension or pulmonary arterial hypertension (PAH) is a rare disease characterized by proliferation and occlusion of small pulmonary arterioles, leading to progressive elevation of pulmonary artery pressure and pulmonary vascular resistance, and right ventricular failure. Historically, it has been associated with a high mortality rate, although, over the last decade, treatment has improved survival. PAH includes idiopathic PAH (IPAH), heritable PAH (HPAH), and PAH associated with certain medical conditions...
January 2017: Journal of Pathology
https://www.readbyqxmd.com/read/27683607/clinical-classification-in-pediatric-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease
#8
Willemijn M H Zijlstra, Johannes M Douwes, Mark-Jan Ploegstra, Usha Krishnan, Marcus T R Roofthooft, Hans L Hillege, D Dunbar Ivy, Erika B Rosenzweig, Rolf M F Berger
Congenital heart disease (CHD) is a frequent cause of pediatric pulmonary arterial hypertension (PAH), with diverse etiology and outcome. We aimed to describe phenotypic heterogeneity in pediatric PAH associated with CHD (PAH-CHD), assess the applicability of the Nice CHD classification, and explore whether this classification accurately reflects patient/disease characteristics and survival. All children with CHD from a contemporary cohort of consecutive pediatric PAH patients followed in three major referral centers (Denver, New York, the Netherlands) were characterized and classified on the basis of the latest proposed clinical classification for PAH-CHD (World Symposium on Pulmonary Hypertension, Nice, 2013)...
September 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/27617070/nt-probnp-in-children-with-left-to-right-shunt-and-dilated-cardiomyopathy
#9
Hala Mahmoud Koura, Neamat M Abdalla, Mona Hamed Ibrahim, Maha M A Abo Hashish, Sherif Mohamed Zaki
BACKGROUND: B-type natriuretic peptide (BNP) levels are elevated in children with congenital heart disease involving a left-to-right shunt (LRS) and are also raised in dilated cardiomyopathy (DCM). As far as we know, there are few reports in the literature comparing the change of the NT-proBNP in LRS and DCM especially in the pediatric age group. OBJECTIVES: The aim of the study was to compare the changes of the NT-proBNP in pediatric patients with LRS and DCM. Correlation between the levels of NT-proBNP and the echocardiographic parameters in both groups was determined...
June 2016: Iranian Journal of Pediatrics
https://www.readbyqxmd.com/read/27583409/new-guidelines-for-managing-pulmonary-hypertension-what-the-pediatrician-needs-to-know
#10
Steven H Abman
PURPOSE OF REVIEW: Pediatric pulmonary vascular disease contributes to morbidities and death in diverse clinical settings, ranging from idiopathic or heritable forms of pediatric arterial hypertension, congenital heart disease, developmental lung disorders, chronic lung disease, left heart disease, sickle cell disease, oncologic disease, and systemic disorders. Despite its impact on the clinical courses in so many diseases, information is limited on how to best approach the diagnosis and evaluation of pediatric pulmonary hypertension...
October 2016: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/27122915/insight-into-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease-pah-chd-classification-and-pharmacological-management-from-a-pediatric-cardiological-point-of-view
#11
REVIEW
I-Chen Chen, Zen-Kong Dai
UNLABELLED: Compared with adult patients with pulmonary hypertension (PH), pulmonary vascular disease is characterized by complex heterogeneity in pediatric patients. The Nice PH classification does not completely characterize or individualize any subgroup of pediatric PH. This is in contrast to the Panama classification, in which prenatal and fetal origins of many pulmonary vascular diseases in neonates and children, perinatal pulmonary vascular maladaptation, prenatal and postnatal pulmonary vascular mal-development, and pulmonary vascular hypoplasia are included...
November 2015: Acta Cardiologica Sinica
https://www.readbyqxmd.com/read/26988953/acute-vasodilator-response-in-pediatric-pulmonary-arterial-hypertension-current-clinical-practice-from-the-topp-registry
#12
MULTICENTER STUDY
Johannes M Douwes, Tilman Humpl, Damien Bonnet, Maurice Beghetti, D Dunbar Ivy, Rolf M F Berger
BACKGROUND: In pulmonary arterial hypertension (PAH), acute vasodilator response testing (AVT) is considered important to identify adult patients with favorable prognosis using calcium-channel blocker (CCB) therapy. However, in pediatric PAH, criteria used to identify acute responders and CCB use are insufficiently studied. OBJECTIVES: This study sought to describe current clinical practice of AVT and subsequent treatment decisions in pediatric PAH. METHODS: From January 2008 to May 2013, 529 consecutive children with confirmed pulmonary hypertension were enrolled in an international registry...
March 22, 2016: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/26560082/do-we-need-more-than-a-transthoracic-echocardiography-when-evaluating-children-with-congenital-heart-disease-before-cardiac-surgery
#13
Mohammed H Alghamdi, Muna I Ismail, Talat Mesud Yelbuz, Fahad Alhabshan
AIM: To determine if a transthoracic echocardiography (TTE) can be used as the sole diagnostic imaging modality to evaluate children with congenital heart disease (CHD) undergoing cardiac surgery. METHODS: A retrospective study was carried out at the King Abdulaziz Cardiac Center. We reviewed all pediatric patients who underwent cardiac surgery during the period January 2011 to December 2011. RESULTS: Three hundred ninety-two pediatric patients with CHD fulfilled the inclusion criteria...
May 2016: Congenital Heart Disease
https://www.readbyqxmd.com/read/26458037/lung-pathology-in-pediatric-pulmonary-vein-stenosis
#14
Jennifer E Pogoriler, Thomas J Kulik, Alicia M Casey, Christopher W Baird, Mary P Mullen, Kathy J Jenkins, Sara O Vargas
Pulmonary vein stenosis is a rare progressive narrowing of the extrapulmonary pulmonary veins, presenting predominantly in infancy and virtually always lethal. It typically arises following repair of congenital heart disease, particularly anomalous pulmonary venous return. Histologic characterization of pediatric pulmonary vein stenosis, not previously well described, may provide insight into the disease pathobiology. We retrieved archival lung specimens (biopsy, explant, or autopsy) from patients with pediatric pulmonary vein stenosis...
May 2016: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/26413109/stepwise-approach-using-combined-management-in-patients-with-congenital-heart-disease-and-borderline-pulmonary-vascular-disease
#15
Sang-Yun Lee, Soo-Jin Kim, Jae Sung Son, Seong-Ho Kim, Chang-Ha Lee
BACKGROUND AND OBJECTIVES: Despite remarkable advances in pediatric cardiology, pulmonary arterial hypertension associated with congenital heart disease remains a major problem. In the past decade new vasodilators have been introduced and appear to be effective in reducing pulmonary vascular resistance (PVR). SUBJECTS AND METHODS: From 2000 to 2011, we retrospectively reviewed the records of 22 patients who had congenital septal defects and borderline pulmonary vascular disease (PVD)...
September 2015: Korean Circulation Journal
https://www.readbyqxmd.com/read/26311236/aortopulmonary-collateral-artery-in-prenatal-exposure-to-carbamazepine-endovascular-therapy-and-technical-considerations-a-case-report
#16
Gloria Pelizzo, Valeria Calcaterra, Savina Mannarino, Lorenzo Paolo Moramarco, Giovanni Leati, Pietro Quaretti
INTRODUCTION: Aortopulmonary collateral arteries are an uncommon variant of alternative blood supply in cases of complex congenital heart disease. Although surgery may still be the classic approach for this condition, mini-invasive endovascular occlusion has been recently attempted as an alternative less traumatic procedure. Children born to women with epilepsy are at increased risk of congenital malformations. CASE PRESENTATION: A cardiovascular malformation in a 6-year-old white boy with prenatal exposure to carbamazepine is described...
2015: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/25847058/human-lung-microrna-profiling-in-pulmonary-arterial-hypertension-secondary-to-congenital-heart-defect
#17
Kai Ma, Qian Zhao, Weidan Chen, Hao Zhang, Shoujun Li, Xiangbin Pan, Qiuming Chen
OBJECTIVE: Although several microRNAs were reported to play essential roles in pulmonary artery hypertension due to hypoxia or monocrotaline, their potential role in pulmonary arterial hypertension secondary to congenital heart disease is largely unknown. This study aimed to indentify microRNAs implicated in pulmonary arterial hypertension secondary to congenital heart disease in children. METHODS: Using microRNAs microarray, we profiled the microRNAs in the lung specimen from 12 congenital heart disease patients, (6 with pulmonary arterial hypertension and the others without)...
December 2015: Pediatric Pulmonology
https://www.readbyqxmd.com/read/25697042/-pediatric-pulmonary-hypertension
#18
D Dunbar Ivy, Steven H Abman, Robyn J Barst, Rolf M F Berger, Damien Bonnet, Thomas R Fleming, Sheila G Haworth, J Usha Raj, Erika B Rosenzweig, Ingram Schulze Neick, Robin H Steinhorn, Maurice Beghetti
Pulmonary hypertension (PH) is a rare disease in newborns, infants, and children that is associated with significant morbidity and mortality. In the majority of pediatric patients, PH is idiopathic or associated with congenital heart disease and rarely is associated with other conditions such as connective tissue or thromboembolic disease. Incidence data from the Netherlands has revealed an annual incidence and point prevalence of 0.7 and 4.4 for idiopathic pulmonary arterial hypertension and 2.2 and 15.6 for pulmonary arterial hypertension, respectively, associated with congenital heart disease (CHD) cases per million children...
October 2014: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/25665593/treating-pulmonary-hypertension-in-pediatrics
#19
REVIEW
Frédéric Lador, Nicole Sekarski, Maurice Beghetti
INTRODUCTION: Pulmonary hypertension is a hemodynamic condition occurring rarely in pediatrics. Nevertheless, it is associated with significant morbidity and mortality. When characterized by progressive pulmonary vascular structural changes, the disease is called pulmonary arterial hypertension (PAH). It results in increased pulmonary vascular resistance and eventual right ventricular failure. In the vast majority of cases, pediatric PAH is idiopathic or associated with congenital heart disease, and, contrary to adult PAH, is rarely associated with connective tissue, portal hypertension, HIV infection or thromboembolic disease...
April 2015: Expert Opinion on Pharmacotherapy
https://www.readbyqxmd.com/read/25661272/a-retrospective-comparison-of-dexmedetomidine-versus-midazolam-for-pediatric-patients-with-congenital-heart-disease-requiring-postoperative-sedation
#20
Li Jiang, Sheng Ding, Hongtao Yan, Yunming Li, Liping Zhang, Xue Chen, Xiumei Yin, Shunbi Liu, Xiuying Tang, Jinbao Zhang
We hypothesized that postoperative sedation with dexmedetomidine/fentanyl would be effective in infants and neonates with congenital heart disease and pulmonary arterial hypertension (PAH). Children who were <36 months of age, had congenital heart disease with PAH, and had been treated at our hospital between October 2011 and April 2013 (n = 187) were included in this retrospective study. Either dexmedetomidine/fentanyl (Group Dex) or midazolam/fentanyl (Group Mid) was used for postoperative sedation. The main outcome variables included delirium scores, supplemental sedative/analgesic drugs, ventilator use, and sedation time...
June 2015: Pediatric Cardiology
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