Diagnosis and treatment of pediatric pulmonary arterial hypertension

Farhat, Nesrine; Lador, Frédéric; Beghetti, Maurice

doi:10.1080/14779072.2019.1576523

Cited by 11 publications

(13 citation statements)

References 87 publications

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“…3 Despite significant advances in treatment, PAH has a poor prognosis, with an estimated mortality of between 25% -60% within five years of diagnosis. 4 The mechanism of PAH caused by acyanotic CHD occurs because of the gap between the ventricles, the atria, and the arteries, which causes an increase in blood flow and pressure in the pulmonary veins, which induces vascular remodeling of the pulmonary artery wall layer, and then cause right ventricular failure and death. 5 In PAH, pulmonary vasculature is damaged, forming reactive oxygen species (ROS).…”

Section: Introductionmentioning

confidence: 99%

Effect of Pomegranate Extract On N-Terminal Pro Brain Natriuretic Peptide and Asymmetric Dimetylarginine Levels in Children with Pulmonary Artery Hypertension in Acyanotic Congenital Heart Disease

Noer¹,

Rahman²,

Utomo³

2023

Pharmacogn J.

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Background: Pulmonary arterial hypertension (PAH) is one of the complications due to acyanotic congenital heart disease. New treatment approach using natural ingredients from plants has been demonstrated in vitro to prevent and improve vascular remodeling in PAH. Objective: This study aimed to analyze the effect of pomegranate extract on N-terminal pro brain natriuretic peptide (NT pro BNP) and asymmetric dimethylarginine (ADMA) levels in children with PAH in acyanotic CHD. Method: This study is a double-blind, quasi-experimental text with pre-test, post-test, and control group approach to children aged one month to 10 years old in Outpatient

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Section: Introductionmentioning

confidence: 99%

Effect of Pomegranate Extract On N-Terminal Pro Brain Natriuretic Peptide and Asymmetric Dimetylarginine Levels in Children with Pulmonary Artery Hypertension in Acyanotic Congenital Heart Disease

Noer¹,

Rahman²,

Utomo³

2023

Pharmacogn J.

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“…Depending on the etiology, five different groups of PH are described and listed in Table 1 [ 1 , 2 ]: pulmonary arterial hypertension (PAH) (group 1), PH associated with left heart disease (group 2), PH associated with lung disease and/or hypoxemia (group 3), PH associated with pulmonary artery obstruction (group 4), and PH with unclear and/or multifactorial mechanisms (group 5) [ 1 , 2 , 3 , 4 ].…”

Section: Introductionmentioning

confidence: 99%

“…The diagnosis of PAH is suggested upon rigorous clinical examination and requires confirmation by echocardiography and, ultimately, by cardiac catheterization with hemodynamic investigation, which allows the precise measurement of PVR and to test vasoreactivity through the inhalation of pulmonary vasodilators, such as oxygen or nitric oxide. The results of this test determine the treatment choice and prognosis [ 1 , 3 ].…”

Section: Introductionmentioning

confidence: 99%

Growing up with Idiopathic Pulmonary Arterial Hypertension: An Arduous Journey

et al. 2023

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Idiopathic pulmonary arterial hypertension (IPAH) is an uncommon and severe disease. We report the case of a 7-year-old boy investigated for cardiac murmur and exercise intolerance. Pulmonary hypertension (PH) was suspected at clinical examination and confirmed by echocardiography and cardiac catheterization. This case of pulmonary hypertension was classified as idiopathic given the negative etiological investigation. Vasoreactive testing with oxygen and nitric oxide was negative. Therefore, treatment with sildenafil (1.4 mg/kg/d) and bosentan (3 mg/kg/d) was initiated. This allowed the stabilization of, but not a decrease in, pulmonary artery pressure for the next 5 years, during which the patient’s quality of life was significantly reduced. At a later follow-up, the estimated pulmonary pressure was found to have increased and become supra-systemic, with a consequent deterioration in the child’s condition. This led to the decision to enter him into a clinical trial that is still ongoing. Idiopathic pulmonary arterial hypertension is a severe disease that can present with non-specific symptoms, such as asthenia and exercise limitation, which are important not to trivialize. The disease is associated with significantly decreased quality of life in affected children and carries a high burden in terms of mortality and morbidity. The current knowledge about IPAH in children is reviewed, with a particular focus on the future prospects for its treatment and the related quality of life of patients.

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“… 4 , 5 While there is a significant body of literature and clinical experience surrounding the use of parenteral prostacyclins in pediatric patients, less is known about alternative drug delivery methods such as enteral administration. 6 Given that parenteral routes of prostacyclin administration (IV, SC, or inhaled) are invariably accompanied by complicated logistics as well as lifestyle compromises, enteral prostacyclin administration represents an attractive treatment option. For example, site pain, which at times can be intolerable, is a well‐documented side effect of SC treprostinil.…”

Section: Introductionmentioning

confidence: 99%

“…However, parenteral prostacyclins remain the cornerstone in the medical management of PAH and remain the only therapeutic modality to have shown an improvement in long‐term survival with PAH 4,5 . While there is a significant body of literature and clinical experience surrounding the use of parenteral prostacyclins in pediatric patients, less is known about alternative drug delivery methods such as enteral administration 6 . Given that parenteral routes of prostacyclin administration (IV, SC, or inhaled) are invariably accompanied by complicated logistics as well as lifestyle compromises, enteral prostacyclin administration represents an attractive treatment option.…”

Section: Introductionmentioning

confidence: 99%

Hemodynamic assessment of transitioning from parenteral prostacyclin to selexipag in pediatric pulmonary hypertension

et al. 2022

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Despite the increase in therapeutic options, parenteral prostacyclins remain the cornerstone in the medical management of pulmonary arterial hypertension (PAH). While the use of parenteral prostacyclins in pediatric patients is well documented, less is known about alternative drug delivery methods such as enteral administration. Given that parenteral routes of prostacyclin administration (IV or SC) are invariably accompanied by complicated logistics and lifestyle compromises, enteral prostacyclin administration represents an attractive treatment option. Selexipag (Uptravi®) was approved for adults PAH in 2015. There is limited data on the hemodynamic efficacy of transitioning from parenteral prostacyclins to selexipag, particularly in the pediatric population. We report 11 pediatric PAH patients who underwent this transition, in which 10 had complete cardiac catheterization data before and following the transition to selexipag. All patients/families reported an improvement in quality of life, and the transitions occurred without adverse effects. However, 3 of the 11 (27%) did not tolerate the transition; two for worsening hemodynamics, and one for acute right ventricular failure in the setting of an intercurrent illness. In addition, the transition to selexipag was associated with a modest increase in pulmonary vascular resistance index (6/10) and decrease in cardiac index (6/10) in some patients. Selexipag use in pediatric PAH represents a significant addition to our therapeutic arsenal, and its use provides a meaningful improvement in quality of life compared with other prostacyclin formulations. However, when goals of care include aggressive disease management, a decision between improved quality of life and possible adverse outcomes must be considered, and its substitution should include cautious, close, long‐term follow‐up.

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Diagnosis and treatment of pediatric pulmonary arterial hypertension

Cited by 11 publications

References 87 publications

Effect of Pomegranate Extract On N-Terminal Pro Brain Natriuretic Peptide and Asymmetric Dimetylarginine Levels in Children with Pulmonary Artery Hypertension in Acyanotic Congenital Heart Disease

Effect of Pomegranate Extract On N-Terminal Pro Brain Natriuretic Peptide and Asymmetric Dimetylarginine Levels in Children with Pulmonary Artery Hypertension in Acyanotic Congenital Heart Disease

Growing up with Idiopathic Pulmonary Arterial Hypertension: An Arduous Journey

Hemodynamic assessment of transitioning from parenteral prostacyclin to selexipag in pediatric pulmonary hypertension

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