The Changing Landscape of Pulmonary Arterial Hypertension in the Adult with Congenital Heart Disease

Dissel, Alexandra C. van; Mulder, Barbara J.M.; Bouma, Berto J.

doi:10.3390/jcm6040040

Cited by 18 publications

(14 citation statements)

References 116 publications

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“…PAH is a hemodynamic and pathophysiological condition in which pulmonary artery pressure increases beyond a certain threshold. It is characterized by distinctive alterations in the pulmonary arterioles that lead to right-sided heart failure, increasing pulmonary arterial pressures, and a high mortality rate [ 4 ]. According to a study, about 47.5% of CHD patients in China have PAH [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

Serum miR-204 and miR-451 Expression and Diagnostic Value in Patients with Pulmonary Artery Hypertension Triggered by Congenital Heart Disease

Qiao

Guan

et al. 2022

Computational and Mathematical Methods in Medicine

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Objective. To determine the level of expression and clinical importance of serum miR-204 and miR-451 in patients with pulmonary arterial hypertension caused by congenital heart disease (CHD-PAH). Methods. From July 2019 to January 2021, 114 infants with congenital heart disease (CHD) were hospitalized at Qingdao’s Fuwai Cardiovascular Hospital. They were grouped into categories: CHD (53 cases) and CHD-PAH (61 cases) based on whether they had pulmonary hypertension (PAH). In addition, 60 healthy children were selected as the control group. All children underwent routine biochemical examination, echocardiography, and pulmonary arterial pressure examination. By using an enzyme-linked immunosorbent assay (ELISA), the levels of brain natriuretic peptide (BNP) and asymmetric dimethylarginine (ADMA) in the blood were measured. Additionally, reverse transcription-polymerase chain reaction (RT-PCR) was used to determine the expression levels of miR-204 and miR-451 in peripheral blood. The correlation between miR-204, miR-451, BNP, ADMA, and mPAP was investigated using Pearson correlation analysis. Results. Consequently, the TC, BNP, and ADMA serum levels were considerably higher in the CHD and CHD-PAH groups than in the control group ( P < 0.05 ), whereas BNP and ADMA serum levels were significantly higher in the CHD-PAH group than in the CHD group ( P < 0.05 ). According to RT-PCR data, the expression levels of miR-204 and miR-451 in the peripheral blood of children in the CHD and CHD-PAH groups were significantly lower ( P < 0.05 ) when compared to the control group. The expression levels of miR-204 and miR-451 in the peripheral blood of children in the CHD-PAH group were substantially lower ( P < 0.05 ) than in the CHD group. Significantly, the findings of the ROC curve revealed that the area under the curve (AUC) of CHD-PAH diagnosed by miR-204 and miR-451 alone was 0.737 and 0.725, respectively, and the AUC of joint diagnosis was 0.840, which was greater than that of single diagnosis ( P < 0.05 ). Patients with CHD-PAH had lower levels of miR-204 and miR-451 in their blood, and this was found to be associated with BNP, ADMA, and mPAP, analyzed by Pearson correlation. Conclusions. Children with CHD-PAH can be diagnosed based on aberrant expressions of miR-204 and miR-451 in their peripheral blood serum. Moreover, CHD-PAH can be diagnosed if the combination of miR-204 and miR-451 is detected as a biomarker, which has a higher diagnostic value.

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Section: Introductionmentioning

confidence: 99%

Serum miR-204 and miR-451 Expression and Diagnostic Value in Patients with Pulmonary Artery Hypertension Triggered by Congenital Heart Disease

Qiao

Guan

et al. 2022

Computational and Mathematical Methods in Medicine

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“…The clinical guidelines do not recommend routine anticoagulant treatment in Eisenmenger syndrome patients and suggest that this therapy may be given in cases of atrial fibrillation and pulmonary artery thrombosis without major haemorrhage. 24 In this study 44,44% (8) with various etiologies such as idiopathic, familial, systemic sclerosis, CHD, human immunodeficiency virus (HIV), liver disease, anorexigen, and obstructive pulmonary venous disease. A total of 93 subjects who were recruited received epoprostenol therapy (28%), bosentan (49%), calcium channel blockers (47%), sildenafil (3%), digoxin (33%), diuretics (57%) and warfarin (49%).…”

Section: Discussionmentioning

confidence: 98%

The Effect of Oral Sildenafil Therapy on Health-Related Quality of Life in Adults with Pulmonary Arterial Hypertension related to Uncorrected Secundum Atrial Septal Defect: A Quasi Experimental Study

Hidayati

Gharini

Hartopo

et al. 2020

Preprint

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Background: Assessment of health-related quality of life (HRQoL) are often measured as an important patient-reported outcome (PRO) in clinical studies. Pulmonary arterial hypertension (PAH) is a common complication of atrial septal defect (ASD). This study aimed to compare the HRQoL of PAH related uncorrected secundum ASD at pre and post therapy with oral sildenafil therapy. Methods: We conducted quasi experimental study at Sardjito General Hospital Yogyakarta since April 2016 to August 2017. Adults with PAH related uncorrected secundum ASD, listed on Congenital Heart Disease and Pulmonary Hypertension (COHARD-PH) registry, and met the inclusion and exclusion criteria were recruited as subject. Interview was done at pre and 12 weeks post oral sildenafil therapy 3 x 20 mg using the EQ-5D-3L questionnaire. Statistical analysis was done using paired T-test to determine the differences of EQ5D utility and EQVAS score at pre and post therapy. Results: A total of 18 adult patients with PAH related to uncorrected secundum ASD were enrolled in this study (83.33% female; mean age 38.72 ± 10.81 years old). The most frequent reported problems pre therapy were pain/discomfort (83%) and anxiety/depression (78%). Paired T-test results showed the mean value of EQ5D utility score pre and post therapy were 0.558 and 0.664 post therapy (p:0.014; 95% CI 0.024-0.187). Meanwhile, the mean of EQ-VAS pre and post therapy were 65 and 71.67, respectively (p:0.005; 95% CI 2.32-11.02). Conclusion: The administration of oral sildenafil therapy 3 x 20 mg during 12 weeks in adult patients with PAH related uncorrected secundum ASD gives better HRQoL.

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“…Current evidence of anticoagulant drugs' efficacy and safety in PAH patient populations is limited. The clinical guidelines do not recommend routine anticoagulant treatment in Eisenmenger syndrome patients and suggest to give anticoagulant treatment in atrial fibrillation and pulmonary artery thrombosis without major haemorrhage [32].…”

Section: Discussionmentioning

confidence: 99%

The effect of oral sildenafil therapy on health-related quality of life in adults with pulmonary arterial hypertension related to uncorrected secundum atrial septal defect: a quasi experimental study

Hidayati

Gharini

Hartopo

et al. 2020

Health Qual Life Outcomes

View full text Add to dashboard Cite

Background: Assessment of health-related quality of life (HRQoL) are often measured as an important patientreported outcome (PRO) in clinical studies. Pulmonary arterial hypertension (PAH) is a common complication of atrial septal defect (ASD). This study aimed to compare the HRQoL of PAH related uncorrected secundum ASD at pre and post therapy with oral sildenafil therapy. Methods: We conducted quasi experimental study at Sardjito General Hospital Yogyakarta since April 2016 to August 2017. Adults with PAH related uncorrected secundum ASD, listed on Congenital Heart Disease and Pulmonary Hypertension (COHARD-PH) registry, and met the inclusion and exclusion criteria were recruited as subject. Interview was done at pre and 12 weeks post oral sildenafil therapy 3 × 20 mg using the EQ-5D-3L questionnaire. Statistical analysis was done using Wilcoxon test and paired T-test to determine the differences of EQ-5D utility and EQ-VAS score at pre and post therapy. Results: A total of 18 adult patients with PAH related to uncorrected secundum ASD were enrolled in this study (83.33% female; mean age 38.72 ± 10.81 years old). The most frequent reported problems pre therapy were pain/ discomfort (83%) and anxiety/depression (78%). Wilcoxon test showed the median of EQ-5D utility score were increased after sildenafil therapy (before = 0.604, after = 0.664; Z = − 2703; p:0.007), respectively. Meanwhile, the paired T-test results showed an increase of EQ-VAS mean difference 6.67 ± 8.75 (p:0.005; 95% CI 2.32-11.02) after sildenafil therapy. Conclusion: The administration of oral sildenafil therapy 3 × 20 mg during 12 weeks in adult patients with PAH related uncorrected secundum ASD gives better HRQoL.

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The Changing Landscape of Pulmonary Arterial Hypertension in the Adult with Congenital Heart Disease

Cited by 18 publications

References 116 publications

Serum miR-204 and miR-451 Expression and Diagnostic Value in Patients with Pulmonary Artery Hypertension Triggered by Congenital Heart Disease

Serum miR-204 and miR-451 Expression and Diagnostic Value in Patients with Pulmonary Artery Hypertension Triggered by Congenital Heart Disease

The Effect of Oral Sildenafil Therapy on Health-Related Quality of Life in Adults with Pulmonary Arterial Hypertension related to Uncorrected Secundum Atrial Septal Defect: A Quasi Experimental Study

The effect of oral sildenafil therapy on health-related quality of life in adults with pulmonary arterial hypertension related to uncorrected secundum atrial septal defect: a quasi experimental study

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