Pulmonary hypertension and myocardial function in infants and children with Down syndrome

Smith, Aisling; Levy, Philip; Franklin, Orla; Molloy, Eleanor J.; El-Khuffash, Afif

doi:10.1136/archdischild-2019-318178

Cited by 7 publications

(3 citation statements)

References 34 publications

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“…Other studies have observed similar findings. Smith et al [ 54 ] reported that DS patients had a higher prevalence of PH with or without an underlying CHD and the difference between the two groups lies in the underlying etiology and the age of presentation. Iwaya et al [ 55 ] reported a lower pulmonary arterial compliance in individuals with CHD in DS when compared to CHD without DS.…”

Section: Discussionmentioning

confidence: 99%

Prevalence of pulmonary hypertension among children with Down syndrome: A systematic review and meta-analysis

Pujari¹,

Jameel²,

Taksande³

et al. 2021

WJCP

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BACKGROUND Pulmonary hypertension (PH) has serious short- and long-term consequences. PH is gaining increasing importance in high risk groups such as Down syndrome (DS) as it influences their overall survival and prognosis. Hence, there is a dire need to collate the prevalence rates of PH in order to undertake definitive measures for early diagnosis and management. AIM To determine the prevalence of PH in children with DS. METHODS The authors individually conducted a search of electronic databases manually (Cochrane library, PubMed, EMBASE, Scopus, Web of Science). Data extraction and quality control were independently performed by two reviewers and a third reviewer resolved any conflicts of opinion. The words used in the literature search were “pulmonary hypertension” and “pulmonary arterial hypertension”; “Down syndrome” and “trisomy 21” and “prevalence”. The data were analyzed by Comprehensive Meta-Analysis Software Version 2. Risk of bias assessment and STROBE checklist were used for quality assessment. RESULTS Of 1578 articles identified, 17 were selected for final analysis. The pooled prevalence of PH in these studies was 25.5%. Subgroup analysis was carried out for age, gender, region, year of publication, risk of bias and etiology of PH. CONCLUSION This review highlights the increasing prevalence of PH in children with DS. It is crucial for pediatricians to be aware of this morbid disease and channel their efforts towards earlier diagnosis and successful management. Community-based studies with a larger sample size of children with DS should be carried out to better characterize the epidemiology and underlying etiology of PH in DS.

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Section: Discussionmentioning

confidence: 99%

Prevalence of pulmonary hypertension among children with Down syndrome: A systematic review and meta-analysis

Pujari¹,

Jameel²,

Taksande³

et al. 2021

WJCP

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“…In addition to the cardiovascular effects of atrioventricular septal defect, infants with trisomy 21 are at an increased independent risk of pulmonary hypertension and myocardial dysfunction in both the neonatal period and beyond. 21 While it is not routine practice at our institution to screen for immune deficiency in this group, increasingly appreciated is the substantial immune dysregulation associated with the condition which includes impairments in both innate and adaptive immune systems. This places these infants at an increased risk of serious infection and sepsis, while dysregulated cytokine release can lead to deleterious outcomes.…”

Section: Geneticsmentioning

confidence: 99%

Outcomes and characteristics in term infants with necrotising enterocolitis and CHD

Kelleher,

Coleman,

McMahon

et al. 2024

Cardiol Young

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Background: CHD is a significant risk factor for the development of necrotising enterocolitis. Existing literature does not differentiate between term and preterm populations. Long-term outcomes of these patients are not well understood. The aim was to investigate the baseline characteristics and outcomes of term normal birth weight infants with CHD who developed necrotising enterocolitis. Methods: A retrospective review was performed of infants from a single tertiary centre with CHD who developed necrotising enterocolitis of Bell’s Stage 1–3, over a ten-year period. Inclusion criteria was those born greater than 36 weeks’ gestation and birth weight over 2500g. Exclusion criteria included congenital gastro-intestinal abnormalities. Sub-group analysis was performed using Fisher’s exact test. Results: Twenty-five patients were identified, with a median gestational age of 38 weeks. Patients with univentricular physiology accounted for 32% (n = 8) and 52% of patients (n = 13) had a duct-dependent lesion. Atrioventricular septal defect was the most common cardiac diagnosis (n = 6, 24%). Patients with trisomy 21 accounted for 20% of cases. Mortality within 30 days of necrotising enterocolitis was 20%. Long-term mortality was 40%, which increased with increasing Bell’s Stage. In total, 36% (n = 9) required surgical management of necrotising enterocolitis, the rate of which was significantly higher in trisomy 21 cases (p < 0.05). Conclusion: Not previously described in term infants is the high rate of trisomy 21 and atrioventricular septal defect. This may reflect higher baseline incidence in our population. Infants with trisomy 21 were more likely to develop surgical necrotising enterocolitis. Mortality at long-term follow-up was high in patients with Bell’s Stage 2–3.

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“…In cases of severe idiopathic PH, RV function is impaired due to the high vascular resistance caused by pulmonary vascular occlusive lesions [6,7]. Similarly, in infants and children with PH, such as that seen in DS, RV contractility and diastolic capacity are impaired [8,9]. We hypothesized that the right ventricle may be under pressure and the RV function may be impaired preoperatively in patients with a large VSD with strong pulmonary vascular occlusive lesions.…”

Section: Introductionmentioning

confidence: 99%

Predicting Postoperative Hypertension Among Patients With Ventricular Septal Defect in Infants

Kawamura

Ueno

Shimozono

et al. 2021

Preprint

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Purpose: Patients with strong pulmonary vascular occlusive lesions are at risk of developing postoperative pulmonary hypertension (PH). We aimed to evaluate preoperative right ventricular (RV) function in patients with ventricular septal defect (VSD) who required cardiac surgery during infancy and consequently developed postoperative PH and to determine whether we could preliminarily evaluate postoperative PH in these patients.Methods: We retrospectively analyzed 55 infants with VSD who underwent cardiac surgery between March 2014 and April 2020. We evaluated the measurements of preoperative atrial/ventricular general function and 2D atrial/ventricular strain between these two groups: a group with postoperative PH (post-PH) and a group without postoperative PH (post-NPH). Results: Post-PH patients had a significantly lower tricuspid annular plane systolic excursion (TAPSE) (11.2 mm) and TAPSERA (the proportion of TAPSE due to RA contraction alone) (6.6 mm) than the post-NPH patients (14.1 mm, 8.5 mm). Furthermore, the post-PH group had a significantly lower peak right atrial (RA) longitudinal strain (PRALS) (31.0%) than the post-NPH group (43.0%). Multivariate logistic regression analysis identified that PRALS and TAPSERA were independent echocardiographic parameters for the presence of post-PH. The sensitivity and specificity of predicting post-PH for ≤38% of the PRALS were 83.0% and 100.0%, respectively, with an area under the curve of 0.94 (p < 0.01).Conclusion: Preoperative RA function and RV diastolic function decreased in the post-PH group. The RA strain and TAPSERA could be useful factors for predicting postoperative PH.

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Pulmonary hypertension and myocardial function in infants and children with Down syndrome

Cited by 7 publications

References 34 publications

Prevalence of pulmonary hypertension among children with Down syndrome: A systematic review and meta-analysis

Prevalence of pulmonary hypertension among children with Down syndrome: A systematic review and meta-analysis

Outcomes and characteristics in term infants with necrotising enterocolitis and CHD

Predicting Postoperative Hypertension Among Patients With Ventricular Septal Defect in Infants

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