schliessen

Filtern

 

Bibliotheken

Clinical features of paediatric pulmonary hypertension: a registry study

Summary Background Paediatric pulmonary hypertension, is an important cause of morbidity and mortality, and is insufficiently characterised in children. The Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension (TOPP) registry is a global, prospective study designed to provide informati... Full description

Journal Title: The Lancet (British edition) 2012, Vol.379 (9815), p.537-546
Main Author: Berger, Rolf MF, Prof
Other Authors: Beghetti, Maurice, Prof , Humpl, Tilman, MD , Raskob, Gary E, MD , Ivy, D Dunbar, MD , Jing, Zhi-Cheng, MD , Bonnet, Damien, MD , Schulze-Neick, Ingram, MD , Barst, Robyn J, Prof
Format: Electronic Article Electronic Article
Language: English
Subjects:
Quelle: Alma/SFX Local Collection
Publisher: Kidlington: Elsevier Ltd
ID: ISSN: 0140-6736
Zum Text:
SendSend as email Add to Book BagAdd to Book Bag
Staff View
recordid: cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_3426911
title: Clinical features of paediatric pulmonary hypertension: a registry study
format: Article
creator:
  • Berger, Rolf MF, Prof
  • Beghetti, Maurice, Prof
  • Humpl, Tilman, MD
  • Raskob, Gary E, MD
  • Ivy, D Dunbar, MD
  • Jing, Zhi-Cheng, MD
  • Bonnet, Damien, MD
  • Schulze-Neick, Ingram, MD
  • Barst, Robyn J, Prof
subjects:
  • Adolescent
  • ADULTS
  • Age of Onset
  • ARTERIAL-HYPERTENSION
  • Biological and medical sciences
  • BOSENTAN
  • BRONCHOPULMONARY DYSPLASIA
  • Cardiac Catheterization
  • Cardiovascular disease
  • Care and treatment
  • Child
  • Child, Preschool
  • CHILDREN
  • CLASSIFICATION
  • Clinical trials
  • Colleges & universities
  • Demographic aspects
  • demographic statistics
  • Diagnosis
  • Diseases
  • DOWN-SYNDROME
  • drugs
  • Educational materials
  • Female
  • General aspects
  • heart diseases
  • Hospitals
  • Humans
  • Hypertension
  • Hypertension, Pulmonary - diagnosis
  • Hypertension, Pulmonary - etiology
  • Hypertension, Pulmonary - physiopathology
  • Infant
  • Internal Medicine
  • Male
  • medical history
  • Medical sciences
  • morbidity
  • Mortality
  • nationalities and ethnic groups
  • Patient outcomes
  • patients
  • Pediatrics
  • Pharmaceuticals
  • physicians
  • Pneumology
  • polycyclic aromatic hydrocarbons
  • prospective studies
  • pulmonary artery
  • Pulmonary hypertension
  • Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases
  • Registries
  • Statistical methods
  • SURVIVAL
  • syncope
  • trisomics
  • UMCG Approved
  • Vascular Resistance
ispartof: The Lancet (British edition), 2012, Vol.379 (9815), p.537-546
description: Summary Background Paediatric pulmonary hypertension, is an important cause of morbidity and mortality, and is insufficiently characterised in children. The Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension (TOPP) registry is a global, prospective study designed to provide information about demographics, treatment, and outcomes in paediatric pulmonary hypertension. Methods Consecutive patients aged 18 years or younger at diagnosis with pulmonary hypertension and increased pulmonary vascular resistance were enrolled in TOPP at 31 centres in 19 countries from Jan 31, 2008, to Feb 15, 2010. Patient and disease characteristics, including age at diagnosis and at enrolment, sex, ethnicity, presenting symptoms, pulmonary hypertension classification, comorbid disorders, medical and family history, haemodynamic indices, and functional class were recorded. Follow-up was decided by the patients' physicians according to the individual's health-care needs. Findings 362 of 456 consecutive patients had confirmed pulmonary hypertension (defined as mean pulmonary artery pressure ≥25 mm Hg, pulmonary capillary wedge pressure ≤12 mm Hg, and pulmonary vascular resistance index ≥3 WU/m−2 ). 317 (88%) patients had pulmonary arterial hypertension (PAH), which was idiopathic [IPAH] or familial [FPAH] in 182 (57%), and associated with other disorders in 135 (43%), of which 115 (85%) cases were associated with congenital heart disease. 42 patients (12%) had pulmonary hypertension associated with respiratory disease or hypoxaemia, with bronchopulmonary dysplasia most frequent. Finally, only three patients had either chronic thromboembolic pulmonary hypertension or miscellaneous causes of pulmonary hypertension. Chromosomal anomalies, mainly trisomy 21, were reported in 47 (13%) of patients with confirmed disease. Median age at diagnosis was 7 years (IQR 3–12); 59% (268 of 456) were female. Although dyspnoea and fatigue were the most frequent symptoms, syncope occurred in 31% (57 of 182) of patients with IPAH or FPAH and in 18% (eight of 45) of those with repaired congenital heart disease; no children with unrepaired congenital systemic-to-pulmonary shunts had syncope. Despite severe pulmonary hypertension, functional class was I or II in 230 of 362 (64%) patients, which is consistent with preserved right-heart function. Interpretation TOPP identifies important clinical features specific to the care of paediatric pulmonary hypertension, which draw attention to the ne
language: eng
source: Alma/SFX Local Collection
identifier: ISSN: 0140-6736
fulltext: fulltext
issn:
  • 0140-6736
  • 1474-547X
url: Link


@attributes
NO1
SEARCH_ENGINEprimo_central_multiple_fe
SEARCH_ENGINE_TYPEPrimo Central Search Engine
RANK2.744374
LOCALfalse
PrimoNMBib
record
control
sourceidgale_pubme
recordidTN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_3426911
sourceformatXML
sourcesystemPC
galeidA343227275
sourcerecordidA343227275
originalsourceidFETCH-LOGICAL-1965t-218b814f663ea1c1af10bf8daa6eae7e41a3002b38375997b5ac37619f6b27c63
addsrcrecordideNqNlP9r1DAYxosobk7_BPVQRAVv5s3XVmEyhjph6A9zIPhDSNPkltlrz6Sd3H_v2_U8b0PcKLS0ed5P3iTP0yx7CGQXCMjXxwQ4mUrF5AuAlxIkhWl-K9sGrvhUcPXtdra9lmxl91I6I4RwScTdbItSygknxXZ2eFCHJlhTT7wzXR9dmrR-sjCuCqaLwU4WfT1vGxOXk9PlwsXONSm0zZuJmUQ3C6nDgdT11fJ-dsebOrkHq-dOdvLh_deDw-nRl4-fDvaPplBI0U0p5GUO3EvJnAELxgMpfV4ZI51xynEwjBBaspwpURSqFMYyJaHwsqTKSraT7Y3cRV_OXWVd00VT60UMc2xStyboyyNNONWz9lwzTmUBgIDPI6BduMaE6C7VVo3rdOWqfqF_eU0I6KJwuFXCc0rBl4oJqcBzbE1KQVlFEPh81VFsf_YudXoeknV1bRrX9kkXFDjljA9TP7miPGv72OB2oYhSIoQccE9H0czUTofGt7gMOyD1PuOMUkWV-K-KqkIVEmiBqt1_qPCq3DzYtnE-4PdL2BsVbM7waqOg7FNoXMJbCrPTLs1Mn9Jl_g3km3Qxym1sU4rOr88KiB5ioC9ioAePawB9EQOdY92jTZOsq_74HgXPVgKT0Pw-msaG9FcnOJ4yDG57e6UBGzrTYQJwX0J9bRvfx2rMzyY-9rOVUdF9w0tTD6atMYcDOmkj0QmQg-YlzzVmQuiCFwrnqrjNPRjFKdIfj3RvWm1mESc4OaYEBLqWFoIxVLwbFQ4DeR5c1MkG11gMd3QWfd6Ga1ewd4VgV_-LH27p0tq-oBPVZIQMDNy7gZCz3wkJbtY
sourcetypeOpen Access Repository
isCDItrue
recordtypearticle
pqid922205560
display
typearticle
titleClinical features of paediatric pulmonary hypertension: a registry study
sourceAlma/SFX Local Collection
creatorBerger, Rolf MF, Prof ; Beghetti, Maurice, Prof ; Humpl, Tilman, MD ; Raskob, Gary E, MD ; Ivy, D Dunbar, MD ; Jing, Zhi-Cheng, MD ; Bonnet, Damien, MD ; Schulze-Neick, Ingram, MD ; Barst, Robyn J, Prof
creatorcontribBerger, Rolf MF, Prof ; Beghetti, Maurice, Prof ; Humpl, Tilman, MD ; Raskob, Gary E, MD ; Ivy, D Dunbar, MD ; Jing, Zhi-Cheng, MD ; Bonnet, Damien, MD ; Schulze-Neick, Ingram, MD ; Barst, Robyn J, Prof
descriptionSummary Background Paediatric pulmonary hypertension, is an important cause of morbidity and mortality, and is insufficiently characterised in children. The Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension (TOPP) registry is a global, prospective study designed to provide information about demographics, treatment, and outcomes in paediatric pulmonary hypertension. Methods Consecutive patients aged 18 years or younger at diagnosis with pulmonary hypertension and increased pulmonary vascular resistance were enrolled in TOPP at 31 centres in 19 countries from Jan 31, 2008, to Feb 15, 2010. Patient and disease characteristics, including age at diagnosis and at enrolment, sex, ethnicity, presenting symptoms, pulmonary hypertension classification, comorbid disorders, medical and family history, haemodynamic indices, and functional class were recorded. Follow-up was decided by the patients' physicians according to the individual's health-care needs. Findings 362 of 456 consecutive patients had confirmed pulmonary hypertension (defined as mean pulmonary artery pressure ≥25 mm Hg, pulmonary capillary wedge pressure ≤12 mm Hg, and pulmonary vascular resistance index ≥3 WU/m−2 ). 317 (88%) patients had pulmonary arterial hypertension (PAH), which was idiopathic [IPAH] or familial [FPAH] in 182 (57%), and associated with other disorders in 135 (43%), of which 115 (85%) cases were associated with congenital heart disease. 42 patients (12%) had pulmonary hypertension associated with respiratory disease or hypoxaemia, with bronchopulmonary dysplasia most frequent. Finally, only three patients had either chronic thromboembolic pulmonary hypertension or miscellaneous causes of pulmonary hypertension. Chromosomal anomalies, mainly trisomy 21, were reported in 47 (13%) of patients with confirmed disease. Median age at diagnosis was 7 years (IQR 3–12); 59% (268 of 456) were female. Although dyspnoea and fatigue were the most frequent symptoms, syncope occurred in 31% (57 of 182) of patients with IPAH or FPAH and in 18% (eight of 45) of those with repaired congenital heart disease; no children with unrepaired congenital systemic-to-pulmonary shunts had syncope. Despite severe pulmonary hypertension, functional class was I or II in 230 of 362 (64%) patients, which is consistent with preserved right-heart function. Interpretation TOPP identifies important clinical features specific to the care of paediatric pulmonary hypertension, which draw attention to the need for paediatric data rather than extrapolation from adult studies. Funding Actelion Pharmaceuticals.
identifier
0ISSN: 0140-6736
1EISSN: 1474-547X
2DOI: 10.1016/S0140-6736(11)61621-8
3PMID: 22240409
4CODEN: LANCAO
languageeng
publisherKidlington: Elsevier Ltd
subjectAdolescent ; ADULTS ; Age of Onset ; ARTERIAL-HYPERTENSION ; Biological and medical sciences ; BOSENTAN ; BRONCHOPULMONARY DYSPLASIA ; Cardiac Catheterization ; Cardiovascular disease ; Care and treatment ; Child ; Child, Preschool ; CHILDREN ; CLASSIFICATION ; Clinical trials ; Colleges & universities ; Demographic aspects ; demographic statistics ; Diagnosis ; Diseases ; DOWN-SYNDROME ; drugs ; Educational materials ; Female ; General aspects ; heart diseases ; Hospitals ; Humans ; Hypertension ; Hypertension, Pulmonary - diagnosis ; Hypertension, Pulmonary - etiology ; Hypertension, Pulmonary - physiopathology ; Infant ; Internal Medicine ; Male ; medical history ; Medical sciences ; morbidity ; Mortality ; nationalities and ethnic groups ; Patient outcomes ; patients ; Pediatrics ; Pharmaceuticals ; physicians ; Pneumology ; polycyclic aromatic hydrocarbons ; prospective studies ; pulmonary artery ; Pulmonary hypertension ; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases ; Registries ; Statistical methods ; SURVIVAL ; syncope ; trisomics ; UMCG Approved ; Vascular Resistance
ispartofThe Lancet (British edition), 2012, Vol.379 (9815), p.537-546
rights
0Elsevier Ltd
12012 Elsevier Ltd
2info:eu-repo/semantics/restrictedAccess
32015 INIST-CNRS
4Copyright © 2012 Elsevier Ltd. All rights reserved.
5COPYRIGHT 2012 Elsevier B.V.
6Copyright Elsevier Limited Feb 11-Feb 17, 2012
lds50peer_reviewed
oafree_for_read
citedbyFETCH-LOGICAL-1965t-218b814f663ea1c1af10bf8daa6eae7e41a3002b38375997b5ac37619f6b27c63
citesFETCH-LOGICAL-1965t-218b814f663ea1c1af10bf8daa6eae7e41a3002b38375997b5ac37619f6b27c63
links
openurl$$Topenurl_article
openurlfulltext$$Topenurlfull_article
thumbnail$$Usyndetics_thumb_exl
backlink
0$$Uhttps://agris.fao.org/agris-search/search.do?recordID=US201500129533$$DView record in FAO AGRIS
1$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=25467116$$DView record in Pascal Francis
2$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/22240409$$D View this record in MEDLINE/PubMed
search
creatorcontrib
0Berger, Rolf MF, Prof
1Beghetti, Maurice, Prof
2Humpl, Tilman, MD
3Raskob, Gary E, MD
4Ivy, D Dunbar, MD
5Jing, Zhi-Cheng, MD
6Bonnet, Damien, MD
7Schulze-Neick, Ingram, MD
8Barst, Robyn J, Prof
title
0Clinical features of paediatric pulmonary hypertension: a registry study
1The Lancet (British edition)
addtitleLancet
descriptionSummary Background Paediatric pulmonary hypertension, is an important cause of morbidity and mortality, and is insufficiently characterised in children. The Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension (TOPP) registry is a global, prospective study designed to provide information about demographics, treatment, and outcomes in paediatric pulmonary hypertension. Methods Consecutive patients aged 18 years or younger at diagnosis with pulmonary hypertension and increased pulmonary vascular resistance were enrolled in TOPP at 31 centres in 19 countries from Jan 31, 2008, to Feb 15, 2010. Patient and disease characteristics, including age at diagnosis and at enrolment, sex, ethnicity, presenting symptoms, pulmonary hypertension classification, comorbid disorders, medical and family history, haemodynamic indices, and functional class were recorded. Follow-up was decided by the patients' physicians according to the individual's health-care needs. Findings 362 of 456 consecutive patients had confirmed pulmonary hypertension (defined as mean pulmonary artery pressure ≥25 mm Hg, pulmonary capillary wedge pressure ≤12 mm Hg, and pulmonary vascular resistance index ≥3 WU/m−2 ). 317 (88%) patients had pulmonary arterial hypertension (PAH), which was idiopathic [IPAH] or familial [FPAH] in 182 (57%), and associated with other disorders in 135 (43%), of which 115 (85%) cases were associated with congenital heart disease. 42 patients (12%) had pulmonary hypertension associated with respiratory disease or hypoxaemia, with bronchopulmonary dysplasia most frequent. Finally, only three patients had either chronic thromboembolic pulmonary hypertension or miscellaneous causes of pulmonary hypertension. Chromosomal anomalies, mainly trisomy 21, were reported in 47 (13%) of patients with confirmed disease. Median age at diagnosis was 7 years (IQR 3–12); 59% (268 of 456) were female. Although dyspnoea and fatigue were the most frequent symptoms, syncope occurred in 31% (57 of 182) of patients with IPAH or FPAH and in 18% (eight of 45) of those with repaired congenital heart disease; no children with unrepaired congenital systemic-to-pulmonary shunts had syncope. Despite severe pulmonary hypertension, functional class was I or II in 230 of 362 (64%) patients, which is consistent with preserved right-heart function. Interpretation TOPP identifies important clinical features specific to the care of paediatric pulmonary hypertension, which draw attention to the need for paediatric data rather than extrapolation from adult studies. Funding Actelion Pharmaceuticals.
subject
0Adolescent
1ADULTS
2Age of Onset
3ARTERIAL-HYPERTENSION
4Biological and medical sciences
5BOSENTAN
6BRONCHOPULMONARY DYSPLASIA
7Cardiac Catheterization
8Cardiovascular disease
9Care and treatment
10Child
11Child, Preschool
12CHILDREN
13CLASSIFICATION
14Clinical trials
15Colleges & universities
16Demographic aspects
17demographic statistics
18Diagnosis
19Diseases
20DOWN-SYNDROME
21drugs
22Educational materials
23Female
24General aspects
25heart diseases
26Hospitals
27Humans
28Hypertension
29Hypertension, Pulmonary - diagnosis
30Hypertension, Pulmonary - etiology
31Hypertension, Pulmonary - physiopathology
32Infant
33Internal Medicine
34Male
35medical history
36Medical sciences
37morbidity
38Mortality
39nationalities and ethnic groups
40Patient outcomes
41patients
42Pediatrics
43Pharmaceuticals
44physicians
45Pneumology
46polycyclic aromatic hydrocarbons
47prospective studies
48pulmonary artery
49Pulmonary hypertension
50Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases
51Registries
52Statistical methods
53SURVIVAL
54syncope
55trisomics
56UMCG Approved
57Vascular Resistance
issn
00140-6736
11474-547X
fulltexttrue
rsrctypearticle
creationdate2012
recordtypearticle
recordideNqNlP9r1DAYxosobk7_BPVQRAVv5s3XVmEyhjph6A9zIPhDSNPkltlrz6Sd3H_v2_U8b0PcKLS0ed5P3iTP0yx7CGQXCMjXxwQ4mUrF5AuAlxIkhWl-K9sGrvhUcPXtdra9lmxl91I6I4RwScTdbItSygknxXZ2eFCHJlhTT7wzXR9dmrR-sjCuCqaLwU4WfT1vGxOXk9PlwsXONSm0zZuJmUQ3C6nDgdT11fJ-dsebOrkHq-dOdvLh_deDw-nRl4-fDvaPplBI0U0p5GUO3EvJnAELxgMpfV4ZI51xynEwjBBaspwpURSqFMYyJaHwsqTKSraT7Y3cRV_OXWVd00VT60UMc2xStyboyyNNONWz9lwzTmUBgIDPI6BduMaE6C7VVo3rdOWqfqF_eU0I6KJwuFXCc0rBl4oJqcBzbE1KQVlFEPh81VFsf_YudXoeknV1bRrX9kkXFDjljA9TP7miPGv72OB2oYhSIoQccE9H0czUTofGt7gMOyD1PuOMUkWV-K-KqkIVEmiBqt1_qPCq3DzYtnE-4PdL2BsVbM7waqOg7FNoXMJbCrPTLs1Mn9Jl_g3km3Qxym1sU4rOr88KiB5ioC9ioAePawB9EQOdY92jTZOsq_74HgXPVgKT0Pw-msaG9FcnOJ4yDG57e6UBGzrTYQJwX0J9bRvfx2rMzyY-9rOVUdF9w0tTD6atMYcDOmkj0QmQg-YlzzVmQuiCFwrnqrjNPRjFKdIfj3RvWm1mESc4OaYEBLqWFoIxVLwbFQ4DeR5c1MkG11gMd3QWfd6Ga1ewd4VgV_-LH27p0tq-oBPVZIQMDNy7gZCz3wkJbtY
startdate2012
enddate2012
creator
0Berger, Rolf MF, Prof
1Beghetti, Maurice, Prof
2Humpl, Tilman, MD
3Raskob, Gary E, MD
4Ivy, D Dunbar, MD
5Jing, Zhi-Cheng, MD
6Bonnet, Damien, MD
7Schulze-Neick, Ingram, MD
8Barst, Robyn J, Prof
general
0Elsevier Ltd
1Elsevier
2Elsevier B.V
3Elsevier Limited
scope
0FBQ
15DI
25DJ
3QVL
4IQODW
5CGR
6CUY
7CVF
8ECM
9EIF
10NPM
11AAYXX
12CITATION
13BSHEE
140TT
150TZ
160U~
173V.
187QL
197QP
207RV
217TK
227U7
237U9
247X7
257XB
2688A
2788C
2888E
2988G
3088I
318AF
328AO
338C1
348C2
358FE
368FH
378FI
388FJ
398FK
408G5
41ABUWG
42AN0
43ASE
44AZQEC
45BBNVY
46BEC
47BENPR
48BHPHI
49C1K
50DWQXO
51FPQ
52FYUFA
53GHDGH
54GNUQQ
55GUQSH
56H94
57HCIFZ
58K6X
59K9-
60K9.
61KB0
62KB~
63LK8
64M0R
65M0S
66M0T
67M1P
68M2M
69M2O
70M2P
71M7N
72M7P
73MBDVC
74NAPCQ
75PQEST
76PQQKQ
77PQUKI
78Q9U
79S0X
807X8
81BOBZL
82CLFQK
835PM
sort
creationdate2012
titleClinical features of paediatric pulmonary hypertension: a registry study
authorBerger, Rolf MF, Prof ; Beghetti, Maurice, Prof ; Humpl, Tilman, MD ; Raskob, Gary E, MD ; Ivy, D Dunbar, MD ; Jing, Zhi-Cheng, MD ; Bonnet, Damien, MD ; Schulze-Neick, Ingram, MD ; Barst, Robyn J, Prof
facets
frbrtype5
frbrgroupidcdi_FETCH-LOGICAL-1965t-218b814f663ea1c1af10bf8daa6eae7e41a3002b38375997b5ac37619f6b27c63
rsrctypearticles
prefilterarticles
languageeng
creationdate2012
topic
0Adolescent
1ADULTS
2Age of Onset
3ARTERIAL-HYPERTENSION
4Biological and medical sciences
5BOSENTAN
6BRONCHOPULMONARY DYSPLASIA
7Cardiac Catheterization
8Cardiovascular disease
9Care and treatment
10Child
11Child, Preschool
12CHILDREN
13CLASSIFICATION
14Clinical trials
15Colleges & universities
16Demographic aspects
17demographic statistics
18Diagnosis
19Diseases
20DOWN-SYNDROME
21drugs
22Educational materials
23Female
24General aspects
25heart diseases
26Hospitals
27Humans
28Hypertension
29Hypertension, Pulmonary - diagnosis
30Hypertension, Pulmonary - etiology
31Hypertension, Pulmonary - physiopathology
32Infant
33Internal Medicine
34Male
35medical history
36Medical sciences
37morbidity
38Mortality
39nationalities and ethnic groups
40Patient outcomes
41patients
42Pediatrics
43Pharmaceuticals
44physicians
45Pneumology
46polycyclic aromatic hydrocarbons
47prospective studies
48pulmonary artery
49Pulmonary hypertension
50Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases
51Registries
52Statistical methods
53SURVIVAL
54syncope
55trisomics
56UMCG Approved
57Vascular Resistance
toplevel
0peer_reviewed
1online_resources
creatorcontrib
0Berger, Rolf MF, Prof
1Beghetti, Maurice, Prof
2Humpl, Tilman, MD
3Raskob, Gary E, MD
4Ivy, D Dunbar, MD
5Jing, Zhi-Cheng, MD
6Bonnet, Damien, MD
7Schulze-Neick, Ingram, MD
8Barst, Robyn J, Prof
collection
0AGRIS
1NARCIS
2NARCIS: Datasets
3NARCIS:Publications
4Pascal-Francis
5Medline
6MEDLINE
7MEDLINE (Ovid)
8MEDLINE
9MEDLINE
10PubMed
11CrossRef
12Academic OneFile (A&I only)
13News PRO
14Pharma and Biotech Premium PRO
15Global News & ABI/Inform Professional
16ProQuest Central (Corporate)
17Bacteriology Abstracts (Microbiology B)
18Calcium & Calcified Tissue Abstracts
19Nursing & Allied Health Database
20Neurosciences Abstracts
21Toxicology Abstracts
22Virology and AIDS Abstracts
23Health & Medical Collection
24ProQuest Central (purchase pre-March 2016)
25Biology Database (Alumni Edition)
26Healthcare Administration Database (Alumni)
27Medical Database (Alumni Edition)
28Psychology Database (Alumni)
29Science Database (Alumni Edition)
30STEM Database
31ProQuest Pharma Collection
32Public Health Database
33Lancet Titles
34ProQuest SciTech Collection
35ProQuest Natural Science Collection
36Hospital Premium Collection
37Hospital Premium Collection (Alumni Edition)
38ProQuest Central (Alumni) (purchase pre-March 2016)
39Research Library (Alumni Edition)
40ProQuest Central (Alumni Edition)
41British Nursing Database
42British Nursing Index
43ProQuest Central Essentials
44Biological Science Collection
45eLibrary
46ProQuest Central
47Natural Science Collection
48Environmental Sciences and Pollution Management
49ProQuest Central Korea
50British Nursing Index (BNI) (1985 to Present)
51Health Research Premium Collection
52Health Research Premium Collection (Alumni)
53ProQuest Central Student
54Research Library Prep
55AIDS and Cancer Research Abstracts
56SciTech Premium Collection
57British Nursing Index
58Consumer Health Database (Alumni Edition)
59ProQuest Health & Medical Complete (Alumni)
60Nursing & Allied Health Database (Alumni Edition)
61ProQuest Newsstand Professional
62ProQuest Biological Science Collection
63Consumer Health Database
64Health & Medical Collection (Alumni Edition)
65Healthcare Administration Database
66Medical Database
67Psychology Database
68Research Library
69Science Database
70Algology Mycology and Protozoology Abstracts (Microbiology C)
71Biological Science Database
72Research Library (Corporate)
73Nursing & Allied Health Premium
74ProQuest One Academic Eastern Edition
75ProQuest One Academic
76ProQuest One Academic UKI Edition
77ProQuest Central Basic
78SIRS Editorial
79MEDLINE - Academic
80OpenAIRE (Open Access)
81OpenAIRE
82PubMed Central (Full Participant titles)
jtitleThe Lancet (British edition)
delivery
delcategoryRemote Search Resource
fulltextfulltext
addata
au
0Berger, Rolf MF, Prof
1Beghetti, Maurice, Prof
2Humpl, Tilman, MD
3Raskob, Gary E, MD
4Ivy, D Dunbar, MD
5Jing, Zhi-Cheng, MD
6Bonnet, Damien, MD
7Schulze-Neick, Ingram, MD
8Barst, Robyn J, Prof
formatjournal
genrearticle
ristypeJOUR
atitleClinical features of paediatric pulmonary hypertension: a registry study
jtitleThe Lancet (British edition)
addtitleLancet
date2012
risdate2012
volume379
issue9815
spage537
epage546
pages537-546
issn0140-6736
eissn1474-547X
codenLANCAO
noteshttp://dx.doi.org/10.1016/S0140-6736(11)61621-8
abstractSummary Background Paediatric pulmonary hypertension, is an important cause of morbidity and mortality, and is insufficiently characterised in children. The Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension (TOPP) registry is a global, prospective study designed to provide information about demographics, treatment, and outcomes in paediatric pulmonary hypertension. Methods Consecutive patients aged 18 years or younger at diagnosis with pulmonary hypertension and increased pulmonary vascular resistance were enrolled in TOPP at 31 centres in 19 countries from Jan 31, 2008, to Feb 15, 2010. Patient and disease characteristics, including age at diagnosis and at enrolment, sex, ethnicity, presenting symptoms, pulmonary hypertension classification, comorbid disorders, medical and family history, haemodynamic indices, and functional class were recorded. Follow-up was decided by the patients' physicians according to the individual's health-care needs. Findings 362 of 456 consecutive patients had confirmed pulmonary hypertension (defined as mean pulmonary artery pressure ≥25 mm Hg, pulmonary capillary wedge pressure ≤12 mm Hg, and pulmonary vascular resistance index ≥3 WU/m−2 ). 317 (88%) patients had pulmonary arterial hypertension (PAH), which was idiopathic [IPAH] or familial [FPAH] in 182 (57%), and associated with other disorders in 135 (43%), of which 115 (85%) cases were associated with congenital heart disease. 42 patients (12%) had pulmonary hypertension associated with respiratory disease or hypoxaemia, with bronchopulmonary dysplasia most frequent. Finally, only three patients had either chronic thromboembolic pulmonary hypertension or miscellaneous causes of pulmonary hypertension. Chromosomal anomalies, mainly trisomy 21, were reported in 47 (13%) of patients with confirmed disease. Median age at diagnosis was 7 years (IQR 3–12); 59% (268 of 456) were female. Although dyspnoea and fatigue were the most frequent symptoms, syncope occurred in 31% (57 of 182) of patients with IPAH or FPAH and in 18% (eight of 45) of those with repaired congenital heart disease; no children with unrepaired congenital systemic-to-pulmonary shunts had syncope. Despite severe pulmonary hypertension, functional class was I or II in 230 of 362 (64%) patients, which is consistent with preserved right-heart function. Interpretation TOPP identifies important clinical features specific to the care of paediatric pulmonary hypertension, which draw attention to the need for paediatric data rather than extrapolation from adult studies. Funding Actelion Pharmaceuticals.
copKidlington
pubElsevier Ltd
pmid22240409
doi10.1016/S0140-6736(11)61621-8
oafree_for_read