viernes, 2 de noviembre de 2012

Más sobre hipertensión pulmonar en la infancia

Hallazgos clínicos en la hipertensión pulmonar infantil: un estudio de registro


Clinical features of paediatric pulmonary hypertension: a registry study.
Berger RM, Beghetti M, Humpl T, Raskob GE, Ivy DD, Jing ZC, Bonnet D, Schulze-Neick I, Barst RJ.
Centre for Congenital Heart Diseases-Paediatric Cardiology, Beatrix Children's Hospital, University Medical Centre Groningen, University of Groningen, Netherlands. r.m.f.berger@umcg.nl
Lancet. 2012 Feb 11;379(9815):537-46. Epub 2012 Jan 11.
Abstract
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.
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3426911/pdf/

nihms382071.pdf


Clasificación funcional de la hipertensión pulmonar en niños: informe de la fuerza de trabajo pediátrica PVRI, Panamá 2012
Functional classification of pulmonary hypertension in children: Report from the PVRI pediatric taskforce, Panama 2011.
Lammers AE, Adatia I, Cerro MJ, Diaz G, Freudenthal AH, Freudenthal F, Harikrishnan S, Ivy D, Lopes AA, Raj JU, Sandoval J, Stenmark K, Haworth SG.
Great Ormond Street Hospital for Children, London, UK.
Pulm Circ. 2011 Aug 2;1(2):280-285.
Abstract
The members of the Pediatric Task Force of the Pulmonary Vascular Research Institute (PVRI) were aware of the need to develop a functional classification of pulmonary hypertension in children. The proposed classification follows the same pattern and uses the same criteria as the Dana Point pulmonary hypertension specific classification for adults. Modifications were necessary for children, since age, physical growth and maturation influences the way in which the functional effects of a disease are expressed. It is essential to encapsulate a child's clinical status, to make it possible to review progress with time as he/she grows up, as consistently and as objectively as possible. Particularly in younger children we sought to include objective indicators such as thriving, need for supplemental feeds and the record of school or nursery attendance. This helps monitor the clinical course of events and response to treatment over the years. It also facilitates the development of treatment algorithms for children. We present a consensus paper on a functional classification system for children with pulmonary hypertension, discussed at the Annual Meeting of the PVRI in Panama City, February 2011.
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3161406/




Características de los pacientes recién nacidos en la hipertensión pulmonar persistente


Patient characteristics in persistent pulmonary hypertension of the newborn.
Roofthooft MT, Elema A, Bergman KA, Berger RM.
Department of Paediatric Cardiology, Beatrix Children's Hospital, University Medical Centre Groningen, University of Groningen, Hanzeplein 1, 9700 RB Groningen, The Netherlands.
Pulm Med. 2011;2011:858154. Epub 2011 May 24.
Abstract
Objective. To assess the impact of PPHN on mortality, morbidity, and behavioural skills. Methods. A retrospective observational study of 143 newborns with PPHN, over an 11-year period, using objective health-status data from medical records and family doctors, and subjective health status data from a standardized Child Behaviour Checklist. Results. The majority of patients were males, treated with inhaled nitric oxide had maladaptation/maldevelopment as pathophysiological mechanism and a gestational age >37 weeks. In term newborns, types of pathophysiological mechanism (P < .001) and Oxygen Index (P = .02) were independent predicting risk factors for PPHN-related mortality. Analysis of preexisting disease and outcome categories in term newborns showed only a significant correlation between the use of iNO and respiratory complaints (P = .03), not confirmed by multivariate analysis and regression analysis. Conclusions. PPHN is a serious, often fatal condition. The incidence of PPHN in preterm newborns is high. In term survivors, PPHN had no additional role in morbidity/outcome.


http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3109632/pdf/PM2011-858154.pdf



Los recientes avances en la comprensión de la hipertensión pulmonar pediátrica.
Recent progress in understanding pediatric pulmonary hypertension.
Abman SH, Ivy DD.
Pediatric Heart Lung Center, Department of Pediatrics, University of Colorado School of Medicine and Children's Hospital, Aurora, Colorado, USA.
Curr Opin Pediatr. 2011 Jun;23(3):298-304. doi: 10.1097/MOP.0b013e3283464a52.
Abstract
PURPOSE OF REVIEW: Pulmonary artery hypertension (PAH) in children contributes significantly to morbidity and mortality in diverse pediatric cardiac, lung, hematologic and other diseases. Advances in pulmonary vascular biology over the past few decades have significantly expanded therapeutic strategies; however, many unique issues persist regarding our understanding of pediatric PAH. RECENT FINDINGS: Recent studies of pediatric PAH include those that highlight gaps in our understanding of pediatric diseases associated with PAH from those of adult onset, emphasizing the strong need for specific studies regarding unique aspects of the pathogenesis and treatment of children with PAH. Registries have begun to provide new data showing differences in physiology, course, and genetics between adult and pediatric forms of PAH. Unfortunately, therapeutic strategies in pediatric pulmonary hypertension are often limited to small observational studies in children and are dependent on results from larger adult studies. In addition, clinical endpoints for studies and care remain poorly defined in infants and children. SUMMARY: Despite many advances, long-term outcomes for children with PAH remain guarded and substantial challenges persist, especially with regard to understanding mechanisms and approach to severe PAH. Future studies are needed to develop novel biomarkers, clinical endpoints and interventions for young children with diverse causes of PAH.
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3128451/pdf/nihms300909.pdf




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Anestesiología y Medicina del Dolor
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