Management of pulmonary hypertension in Down syndrome

Hawkins, A. B.; Hewer, Simon C Langton; Henderson, John; Tulloh, Robert

doi:10.1007/s00431-010-1378-1

Cited by 53 publications

(53 citation statements)

References 20 publications

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“…538 It is well known that infants with Down syndrome often have lung hypoplasia, which increases the risk for PAH, which is accelerated in the setting of cardiac disease or intermittent hypoxia caused by OSA. 181,182 Beneficial responses to ERA or PDE5 inhibitor therapies have been reported in uncontrolled studies in subjects with Down syndrome. 181,539 The actual prevalence, natural history, and response to therapy of PAH in infants and young children with pulmonary growth abnormalities are unknown.…”

Section: Lung Diseasesmentioning

confidence: 99%

“…181,182 Beneficial responses to ERA or PDE5 inhibitor therapies have been reported in uncontrolled studies in subjects with Down syndrome. 181,539 The actual prevalence, natural history, and response to therapy of PAH in infants and young children with pulmonary growth abnormalities are unknown.…”

Section: Lung Diseasesmentioning

confidence: 99%

“…Subjects with Down syndrome have an increased risk for PPHN and are more susceptible to PH with stresses such as CHD, upper airway obstruction, and OSA. 180,181 This increased risk for PH may reflect reduced alveolar surface area and loss of capillary surface area in Down syndrome. …”

Section: Clinical Monitoring Of Individuals At Riskmentioning

confidence: 99%

See 2 more Smart Citations

Pediatric Pulmonary Hypertension

Abman¹,

Hansmann²,

Archer³

et al. 2015

Circulation

919

405

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Abstract-Pulmonary hypertension is associated with diverse cardiac, pulmonary, and systemic diseases in neonates, infants, and older children and contributes to significant morbidity and mortality. However, current approaches to caring for pediatric patients with pulmonary hypertension have been limited by the lack of consensus guidelines from experts in the field. In a joint effort from the American Heart Association and American Thoracic Society, a panel of experienced clinicians and clinician-scientists was assembled to review the current literature and to make recommendations on the diagnosis, evaluation, and treatment of pediatric pulmonary hypertension. This publication presents the results of extensive literature reviews, discussions, and formal scoring of recommendations for the care of children with pulmonary hypertension. Key Words: AHA Scientific Statements ◼ bronchopulmonary dysplasia ◼ congenital diaphragmatic hernia ◼ congenital heart disease ◼ genetics ◼ persistent pulmonary hypertension of the newborn ◼ sickle cell disease © 2015 by the American Heart Association, Inc., and the American Thoracic Society.Circulation is available at http://circ.ahajournals.org DOI: 10.1161/CIR.0000000000000329 †Deceased. The American Heart Association and the American Thoracic Society make every effort to avoid any actual or potential conflicts of interest that may arise as a result of an outside relationship or a personal, professional, or business interest of a member of the writing panel. Specifically, all members of the writing group are required to complete and submit a Disclosure Questionnaire showing all such relationships that might be perceived as real or potential conflicts of interest.This document was approved by the American Heart Association Science Advisory and Coordinating Committee on May 12, 2015, the American Heart Association Executive Committee on July 22, 2015, and the American Thoracic Society on July 24, 2015.The online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIR.0000000000000329/-/DC1. The American Heart Association requests that this document be cited as follows: Abman SH, Hansmann G, Archer SL, Ivy DD, Adatia I, Chung WK, Hanna BD, Rosenzweig EB, Raj JU, Cornfield D, Stenmark KR, Steinhorn R, Thébaud B, Fineman JR, Kuehne T, Feinstein JA, Friedberg MK, Earing M, Barst RJ, Keller RL, Kinsella JP, Mullen M, Deterding R, Kulik T, Mallory G, Humpl T, Wessel DL; on behalf of the American Heart Association Council on Cardiopulmonary, Critical Care, Perioperative and Resuscitation, Council on Clinical Cardiology, Council on Cardiovascular Disease in the Young, Council on Cardiovascular Radiology and Intervention, Council on Cardiovascular Surgery and Anesthesia, and the American Thoracic Society. Pediatric pulmonary hypertension: guidelines from the American Heart Association and American Thoracic Society. Circulation. 2015;132:2037-2099 Copies: This document is available on the World Wide Web site of the American Heart Associat...

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Section: Lung Diseasesmentioning

confidence: 99%

Section: Lung Diseasesmentioning

confidence: 99%

See 1 more Smart Citation

Pediatric Pulmonary Hypertension

Abman¹,

Hansmann²,

Archer³

et al. 2015

Circulation

919

405

View full text Add to dashboard Cite

show abstract

“…Obstructive sleep apnoea is common in children with SD, affecting 30-50% of this patient group compared with 3% of the general paediatric population. This may result from a number of factors, including hypotonic upper airway, adeno-tonsillar hypertrophy, macroglossia, glossoptosis, flattened mid-face and narrow nasopharynx in these patients (Hawkins et al, 2011). This association of CHD in DS with pulmonary arterial hypertension, has led a neonatal screening and, as medical treatment becomes increasingly unsuccessful, early definitive cardiac surgery is usually undertaken at 3-6 months of age (Vohra et al, 2010).…”

Section: Pulmonary Arterial Hypertensionmentioning

confidence: 99%

Early Diagnosis of Congenital Heart Disease in the Neonatal Period

Ortigado¹

2011

Prenatal Diagnosis and Screening for Down Syndrome

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“…Patients with DS and CHD are thought to develop PAH more rapidly than non-DS patients with CHD. 4 In patients with DS and severe pulmonary hypertension, cardiologists need to determine whether there are surgical indications when they perform the cardiac catheterizations. In those cases, the Heath-Edwards (H-E) classification, and in Japan the index of pulmonary vascular disease (IPVD) 5 proposed by Yamaki and Tezuka, 6 determined from a lung biopsy play an important role in assessing the need for surgery.…”

mentioning

confidence: 99%

What Causes Pulmonary Arterial Hypertension in Down Syndrome With Congenital Heart Disease?

Hosokawa

Vanderpool

Ishii

et al. 2018

Circ J

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H-E classification and IPVD score. Furthermore, earlier progression of histopathological changes in pulmonary arteriopathy could not be proved in patients with DS despite accounting for age in the histological findings. Previous papers have reported no difference in the incidence of PAH between patients with DS and those without DS. Vázquez-Antona et al showed that results of a hemodynamic study of pulmonary hypertension and vasodilation test with oxygen were similar for patients with DS and those without the chromosomal abnormality, 10 though they did not examine histological findings.Cohort selection, other types of respiratory problems and anatomic variability are other reasons that could explain the results in the current study by Masaki et al. Patients who had undergone pulmonary artery banding were excluded from this study, which might cause underestimation of the severity of pulmonary arteriopathy in the group of patients with DS. Other factors could be contributing to the pathogenesis of PAH in patients with DS. Pandit et al suggest there are many respiratory problems in both the upper and lower airways of children with DS. 11 Anatomic characteristics in DS such as large tongue, small hypopharynx, pharyngeal hypotonia, tonsils and adenoid enlargement, laryngomalacia, and tracheomalacia cause upper airway problems. In addition, gastroesophageal reflux, immunologic dysfunction, tracheal bronchus, airway malacia, CHD, and pulmonary hypoplasia cause recurrent lower airway diseases. 11 Cooney and Thurlbeck showed that 6 of 7 patients with DS had hypoplastic lungs, and 5 had CHD. 12 Patients with DS have a decreased number of alveoli in relation to acini, enlarged alveoli and alveolar ducts, and a smaller alveolar surface area, which can lead to exacerbation of pulmonary hypertension in DS. 12 Bush et al investigated the histologic features of impaired development of the lung vasculature and alveoli and the presence of intrapulmonary bronchopulmonary anastomoses (IBA) in patients with DS using specimens obtained from autopsy. 13 These patients had frequent histologic evidence of impaired lung alveolar and vascular development, D own syndrome (DS) is the most common chromosomal abnormality. A recent study showed that its incidence is 1 in 319-1,000 live births. 1 Approximately half of all newborns with DS have congenital heart disease (CHD): 2 atrioventricular septal defect (AVSD: 45%), ventricular septal defect (VSD: 35%), secundum atrial septal defect (8%), and tetralogy of Fallot (4%). 3 DS strongly associates with pulmonary arterial hypertension (PAH). Patients with DS and CHD are thought to develop PAH more rapidly than non-DS patients with CHD. 4 In patients with DS and severe pulmonary hypertension, cardiologists need to determine whether there are surgical indications when they perform the cardiac catheterizations. In those cases, the Heath-Edwards (H-E) classification, and in Japan the index of pulmonary vascular disease (IPVD) 5 proposed by Yamaki and Tezuka, 6 determined from a lung biopsy play an im...

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Management of pulmonary hypertension in Down syndrome

Abstract: In order to manage this complex group of patients, a combined cardiological, respiratory and surgical approach was required. A protocol with cardiac catheterisation, blood tests and respiratory assessment is suggested for the management of pulmonary hypertension in these children.

Cited by 53 publications

References 20 publications

Pediatric Pulmonary Hypertension

Pediatric Pulmonary Hypertension

Early Diagnosis of Congenital Heart Disease in the Neonatal Period

What Causes Pulmonary Arterial Hypertension in Down Syndrome With Congenital Heart Disease?

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