Echocardiographic Screening for Pulmonary Hypertension in Congenital Heart Disease

Dimopoulos, Konstantinos; Condliffe, Robin; Tulloh, Robert; Clift, Paul; Alonso‐Gonzalez, Rafael; Bedair, Radwa; Chung, Natali; Coghlan, Gerry; Fitzsimmons, Samantha; Frigiola, Alessandra; Howard, Luke; Jenkins, Peter; Kenny, Damien; Li, Wei; MacDonald, Simon T.; McCabe, Colm; Oliver, James; Spence, Mark S.; Szánthó, Gergely; Klemperer, Kate von; Wilson, Dirk G.; Wort, Stephen J.

doi:10.1016/j.jacc.2018.08.2201

Cited by 42 publications

(31 citation statements)

References 41 publications

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“…2 Echocardiography is a well-established and widely available technique that is routinely used during the initial screening, follow-up assessment and evaluation of treatment responses for patients with PAH. [5][6][7] Despite advances in other imaging modality techniques such as MRI or CT, echocardiography remains a convenient and valuable option for assessing cardiac morphology and function.…”

Section: Introductionmentioning

confidence: 99%

Association between right atrial area measured by echocardiography and prognosis among pulmonary arterial hypertension: a systematic review and meta-analysis

et al. 2020

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ObjectiveThe purpose of this meta-analysis was to evaluate the association between enlarged right atrial area (RAA), as measured by echocardiography, and prognosis of patients with pulmonary arterial hypertension (PAH).DesignSystematic review and meta-analysis.Data sourcesTo identify potential publications, a comprehensive literature search through MEDLINE, the Cochrane database and the Embase database was performed up to December 2019.Eligibility criteria for selecting studiesStudies were included if they reported Cox regression based-HRs with 95% CIs for all-cause mortality or composite endpoint consisting of death and PAH-related events for echocardiography measurements of the RAA or the right atrial area index (RAAI) in patients with PAH.Data extraction and synthesisThe unadjusted HR with 95% CI was extracted for the final pooled analysis. A random-effects model was used to determine the value of RAA/RAAI in the prognosis of patients with PAH. The data heterogeneity among the studies was estimated by the I2 statistic and the Cochran Q-statistic.ResultsTwelve studies with a total of 1085 patients with PAH were finally included in the meta-analysis. These studies had a mean follow-up time ranging from 9.2 months to 5.0 years. Their findings showed that patients with PAH with enlarged RAA/RAAI were associated with poor prognosis. The risk of all-cause mortality in patients with PAH was found to statistically increase by 50% for every 5-unit increase in RAA/RAAI (HR 1.50, 95% CI 1.28 to 1.75, p<0.001). Similarly, the risk of the composite endpoint also significantly increased by 53% for every 5-unit increase in RAA/RAAI (HR 1.53, 95% CI 1.23 to 1.89, p<0.001). Subgroup analyses in which the patients were stratified by RAA and RAAI were consistent with the main results.ConclusionThe meta-analysis suggested that enlarged RAA/RAAI were associated with increased risk of poor prognosis in patients with PAH.

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Section: Introductionmentioning

confidence: 99%

Association between right atrial area measured by echocardiography and prognosis among pulmonary arterial hypertension: a systematic review and meta-analysis

et al. 2020

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“…As the most prevalent type of human birth defect, congenital heart defect (CHD) occurs in about 1% of all live neonates, accounting for nearly a third of all forms of developmental abnormalities (Benjamin et al, 2019;Oliveira-Brancati et al, 2020). Although minor CHD may resolve spontaneously (Benjamin et al, 2019), serious CHD may lead to poor health-related quality of life (Amedro et al, 2018(Amedro et al, , 2019Boukovala et al, 2019), reduced exercise capacity (Müller et al, 2018;Abassi et al, 2019;Smith et al, 2019), abnormal nervous develop ment or brain injury (Peyvandi et al, 2018(Peyvandi et al, , 2019Khanna et al, 2019), hemorrhagic or ischemic stroke (Bokma et al, 2018;Giang et al, 2018;Pedersen et al, 2019), pulmonary hypertension (Brida and Gatzoulis, 2018;Dimopoulos et al, 2018;Kaemmerer et al, 2018;Pascall and Tulloh, 2018), acute kidney injury or renal dysfunction (Lui et al, 2017;Gist et al, 2018), infective endocarditis (Jortveit et al, 2018;Tutarel et al, 2018;Cahill et al, 2019), cardiac dysfunction or congestive heart failure (Gilbert et al, 2018;Lal et al, 2018;Sabanayagam et al, 2018;Chan et al, 2019), ventricular or supraventricular dysrhythmia (Labombarda et al, 2017;Barry et al, 2018;Hernández-Madrid et al, 2018;Fuchs et al, 2019), and death (Lynge et al, 2018;Yu C et al, 2018). Although vast advance in cardiac surgery allows over 90% of CHD newborns to survive into adulthood, it results i...…”

Section: Introductionmentioning

confidence: 99%

A novel TBX5 mutation predisposes to familial cardiac septal defects and atrial fibrillation as well as bicuspid aortic valve

Jiang

Zhao

et al. 2020

Genet. Mol. Biol.

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“…Guidance on when and for whom to consider screening of PAH in patients with portal hypertension or congenital heart disease is detailed in a separate review in this supplement, 70 but it is also important to note that screening algorithms/methods should be tailored to the at-risk population concerned 68 . Patients considered for correction of CHD with prevalent systemic-to-pulmonary shunts should be screened for PH by means of RHC; the presence of PAH and the magnitude of pulmonary vascular resistance elevation is associated with significant risk after CHD correction 71 . Patients considered for liver transplantation should be screened for PH by echocardiography followed by RHC if needed, as the presence of PAH is associated with significant mortality risk after transplantation 1 , 2 .…”

Section: Screening Strategies For Pulmonary Arterial Hypertensionmentioning

confidence: 99%

Screening strategies for pulmonary arterial hypertension

Kiely

Lawrie

Humbert

2019

European Heart Journal Supplements

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Pulmonary arterial hypertension (PAH) is rare and, if untreated, has a median survival of 2–3 years. Pulmonary arterial hypertension may be idiopathic (IPAH) but is frequently associated with other conditions. Despite increased awareness, therapeutic advances, and improved outcomes, the time from symptom onset to diagnosis remains unchanged. The commonest symptoms of PAH (breathlessness and fatigue) are non-specific and clinical signs are usually subtle, frequently preventing early diagnosis where therapies may be more effective. The failure to improve the time to diagnosis largely reflects an inability to identify patients at increased risk of PAH using current approaches. To date, strategies to improve the time to diagnosis have focused on screening patients with a high prevalence [systemic sclerosis (10%), patients with portal hypertension assessed for liver transplantation (2–6%), carriers of mutations of the gene encoding bone morphogenetic protein receptor type II, and first-degree relatives of patients with heritable PAH]. In systemic sclerosis, screening algorithms have demonstrated that patients can be identified earlier, however, current approaches are resource intensive. Until, recently, it has not been considered possible to screen populations for rare conditions such as IPAH (prevalence 5–15/million/year). However, there is interest in the use of artificial intelligence approaches in medicine and the application of diagnostic algorithms to large healthcare data sets, to identify patients at risk of rare conditions. In this article, we review current approaches and challenges in screening for PAH and explore novel population-based approaches to improve detection.

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Echocardiographic Screening for Pulmonary Hypertension in Congenital Heart Disease

Cited by 42 publications

References 41 publications

Association between right atrial area measured by echocardiography and prognosis among pulmonary arterial hypertension: a systematic review and meta-analysis

Association between right atrial area measured by echocardiography and prognosis among pulmonary arterial hypertension: a systematic review and meta-analysis

A novel TBX5 mutation predisposes to familial cardiac septal defects and atrial fibrillation as well as bicuspid aortic valve

Screening strategies for pulmonary arterial hypertension

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