Active right atrial emptying fraction predicts reduced survival and increased adverse events in childhood pulmonary arterial hypertension

Kumar, Shine; Vadlamudi, Karunakar; Kaddoura, Tarek; Bobhate, Prashant; Goot, Benjamin H.; Elgendi, Mohamed; Jain, Shreepal; Colen, Timothy; Khoo, Nee Scze; Adatia, Ian

doi:10.1016/j.ijcard.2018.04.125

Cited by 11 publications

(6 citation statements)

References 33 publications

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“…In accordance with this, right-left shunting in the atrium or via the PDA were described as main diagnostic criteria by study participants in the PUsH survey. The majority of the PUsH survey participants considered the dimension of the RV as an additional diagnostic criterion, in line with current recommendations 23 : The expert consensus statement on the diagnosis and treatment of pediatric PH by the European Pediatric Pulmonary Vascular Disease Network (EPPVDN) published in 2016 recommended several parameters in transthoracic echocardiography when diagnosing pediatric PH. 24 PUsH survey participants showed a strong agreement on some of those parameters.…”

Section: Discussionmentioning

confidence: 76%

Current diagnosis and treatment practice for pulmonary hypertension in bronchopulmonary dysplasia—A survey study in Germany (PUsH BPD)

Häfner,

Johansson,

Schwarzkopf

et al. 2023

Pulm. circ.

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Pulmonary hypertension (PH) is the most severe complication in preterm infants with bronchopulmonary dysplasia (BPD) and associated with significant mortality. Diagnostic and treatment strategies, however, still lack standardization. By the use of a survey study (PH in BPD), we assessed clinical practice (diagnosis, treatment, follow‐up) in preterm infants with early postnatal persistent pulmonary hypertension of the newborn (PPHN) as well as at risk for or with established BPD‐associated PH between 06/2018 and 10/2020 in two‐thirds of all German perinatal centers with >70 very low birthweight infants/year including their cardiology departments and outpatient units. Data were analyzed descriptively by measures of locations and distributional shares. In routine postnatal care, clinical presentation and echocardiography were reported as the main diagnostic modalities to screen for PPHN in preterm infants, whereas biomarkers brain natriuretic peptide/N‐terminal pro b‐type natriuretic peptide were infrequently used. For PPHN treatment, inhaled nitric oxide was used in varying frequency. The majority of participants agreed to prescribe diuretics and steroids (systemic/inhaled) for infants at risk for or with established BPD‐associated PH and strongly agreed on recommending respiratory syncytial virus immunization and the use of home monitoring upon discharge. Reported oxygen saturation targets, however, varied in these patients in in‐ and outpatient care. The survey reveals shared practices in diagnostic and therapeutic strategies for preterms with PPHN and BPD‐associated PH in Germany. Future studies are needed to agree on detailed echo parameters and biomarkers to diagnose and monitor disease next to a much‐needed agreement on the use of pulmonary vasodilators, steroids, and diuretics as well as target oxygen saturation levels.

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Section: Discussionmentioning

confidence: 76%

Current diagnosis and treatment practice for pulmonary hypertension in bronchopulmonary dysplasia—A survey study in Germany (PUsH BPD)

Häfner,

Johansson,

Schwarzkopf

et al. 2023

Pulm. circ.

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“…A correlation between RV FAC and RV ejection fraction (RVEF) and TAPSE has been shown in adults with IPAH [94]. In pediatric PAH, an RV FAC < 25% may indicate clinical worsening [95][96][97]. Moreover, a decreased RV FAC was associated with the risk of death in adult and pediatric IPAH patients [93,94].…”

Section: Rv Fractional Area Change (Fac)mentioning

confidence: 99%

Echocardiography for the Assessment of Pulmonary Hypertension and Congenital Heart Disease in the Young

Meinel

Koestenberger

Sallmon³

et al. 2020

Diagnostics

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While invasive assessment of hemodynamics and testing of acute vasoreactivity in the catheterization laboratory is the gold standard for diagnosing pulmonary hypertension (PH) and pulmonary vascular disease (PVD) in children, transthoracic echocardiography (TTE) serves as the initial diagnostic tool. International guidelines suggest several key echocardiographic variables and indices for the screening studies when PH is suspected. However, due to the complex anatomy and special physiological considerations, these may not apply to patients with congenital heart disease (CHD). Misinterpretation of TTE variables can lead to delayed diagnosis and therapy, with fatal consequences, or–on the other hand-unnecessary invasive diagnostic procedures that have relevant risks, especially in the pediatric age group. We herein provide an overview of the echocardiographic workup of children and adolescents with PH with a special focus on children with CHD, such as ventricular/atrial septal defects, tetralogy of Fallot or univentricular physiology. In addition, we address the use of echocardiography as a tool to assess eligibility for exercise and sports, a major determinant of quality of life and outcome in patients with PH associated with CHD.

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“…While we often see atrial dilatation in children quite late in the course of the disease, it reflects a loss of venous pooling capacity and worsening of cardiac integrity. A pediatric study demonstrated that an active emptying fraction of ≥60% and a RV FAC <25% correlated with clinical worsening (14). Progressive worsening of the RA reservoir and conduit function has been reported to describe RA deformation and performance, which have been shown to correlate with functional capacity, invasive hemodynamics and emerged as predictors for adverse outcome in children with PH (15).…”

Section: Assessment Of Right Atrial (Ra) Dimensionsmentioning

confidence: 99%

“…As illustrated above, diastolic dysfunction directly translates into a pathological load of the right atrium. The function of the right atrium, measured with different modalities is compromised and shows impairment with worsening diastolic dysfunction of the RV (14,76,77). While in children dilatation and compromise of the RA appear to occur quite late in the course of longstanding PH compared to adult patients with a similar degree of disease severity.…”

Section: Rv Diastolic Functionmentioning

confidence: 99%

A guide to echocardiographic assessment in children and adolescents with pulmonary hypertension

Lammers¹,

Apitz²,

Michel‐Behnke³

et al. 2021

Cardiovasc Diagn Ther

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Data from the TOPP registry suggested the potential combination of simple diagnostic non-invasive tests (chest X-ray, electrocardiography and echocardiography) to rule out the presence of PH. In fact, none of the 456 children with PH at the time of enrollment had all three tests considered normal (3).Echocardiography not only reveals the underlying cardiac morphology and diagnosis of any associated

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Active right atrial emptying fraction predicts reduced survival and increased adverse events in childhood pulmonary arterial hypertension

Cited by 11 publications

References 33 publications

Current diagnosis and treatment practice for pulmonary hypertension in bronchopulmonary dysplasia—A survey study in Germany (PUsH BPD)

Current diagnosis and treatment practice for pulmonary hypertension in bronchopulmonary dysplasia—A survey study in Germany (PUsH BPD)

Echocardiography for the Assessment of Pulmonary Hypertension and Congenital Heart Disease in the Young

A guide to echocardiographic assessment in children and adolescents with pulmonary hypertension

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