Pulmonary arterial compliance is a useful predictor of pulmonary vascular disease in congenital heart disease

Muneuchi, Jun; Ochiai, Yoshie; Masaki, N; Okada, Seigo; Iida, Chiaki; Sugitani, Yuichiro; Ando, Yumiko; Watanabe, Mamie

doi:10.1007/s00380-018-1263-9

Cited by 8 publications

(6 citation statements)

References 19 publications

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“… 30–32 Increased pulmonary blood flow, as seen in ASD, VSD, and PDA, can lead to tunica media proliferation, leading to increased pulmonary vessel resistance. 32 , 33 Furthermore, acquired conditions that elevate left atrial pressure (e.g. hypertension or valvular heart disease) may increase the left-to-right shunt in simple ASD.…”

Section: Discussionmentioning

confidence: 99%

Lifetime risk of comorbidity in patients with simple congenital heart disease: a Danish nationwide study

El‐Chouli¹,

Meddis

Christensen³

et al. 2022

European Heart Journal

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Aims In a continuously ageing population of patients with congenital heart disease (CHD), understanding the long-term risk of morbidity is crucial. The aim of this study was to compare the lifetime risks of developing comorbidities in patients with simple CHD and matched controls. Methods and results Using the Danish nationwide registers spanning from 1977 to 2018, simple CHD cases were defined as isolated atrial septal defect (ASD), ventricular septal defect (VSD), pulmonary stenosis, or patent ductus arteriosus in patients surviving until at least 5 years of age. There were 10 controls identified per case. Reported were absolute lifetime risks and lifetime risk differences (between patients with simple CHD and controls) of incident comorbidities stratified by groups and specific cardiovascular comorbidities. Of the included 17 157 individuals with simple CHD, the largest subgroups were ASD (37.7%) and VSD (33.9%), and 52% were females. The median follow-up time for patients with CHD was 21.2 years (interquartile range: 9.4–39.0) and for controls, 19.8 years (9.0–37.0). The lifetime risks for the investigated comorbidities were higher and appeared overall at younger ages for simple CHD compared with controls, except for neoplasms and chronic kidney disease. The lifetime risk difference among the comorbidity groups was highest for neurological disease (male: 15.2%, female: 11.3%), pulmonary disease (male: 9.1%, female: 11.7%), and among the specific comorbidities for stroke (male: 18.9%, female: 11.4%). The overall risk of stroke in patients with simple CHD was mainly driven by ASD (male: 28.9%, female: 17.5%), while the risks of myocardial infarction and heart failure were driven by VSD. The associated lifetime risks of stroke, myocardial infarction, and heart failure in both sexes were smaller in invasively treated patients compared with untreated patients with simple CHD. Conclusion Patients with simple CHD had increased lifetime risks of all comorbidities compared with matched controls, except for neoplasms and chronic kidney disease. These findings highlight the need for increased attention towards early management of comorbidity risk factors.

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Section: Discussionmentioning

confidence: 99%

Lifetime risk of comorbidity in patients with simple congenital heart disease: a Danish nationwide study

El‐Chouli¹,

Meddis

Christensen³

et al. 2022

European Heart Journal

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“…Langelben et al reported that an increase in pulmonary blood flow is mainly accommodated by recruitment, with progressive distention according to a further increase in animal models (17). In patients with CHD-PH, Cp rather than Rp can predict the development of pulmonary vascular diseases, such as medial thickness or hypoplastic arterioles, suggesting that a low Cp reflects an early stage of reduced effective pulmonary vascular beds following impaired recruitment and distensibility of the pulmonary capillaries (18). Recently, Cp has gained attention in the management of patients with different conditions of pulmonary arterial hypertension (Table 2) (13, 14, 18-29).…”

Section: Pulmonary Vascular Resistance and Capacitancementioning

confidence: 99%

Comprehensive assessments of pulmonary circulation in children with pulmonary hypertension associated with congenital heart disease

Muneuchi

Ezaki²,

Sugitani³

et al. 2022

Front. Pediatr.

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Pulmonary hypertension associated with congenital heart disease (CHD-PH) encompasses different conditions confounded by the left-to-right shunt, left heart obstruction, ventricular dysfunction, hypoxia due to airway obstruction, dysplasia/hypoplasia of the pulmonary vasculature, pulmonary vascular obstructive disease, and genetic variations of vasoactive mediators. Pulmonary input impedance consists of the pulmonary vascular resistance (Rp) and capacitance (Cp). Rp is calculated as the transpulmonary pressure divided by the pulmonary cardiac output, whereas Cp is calculated as the pulmonary stroke volume divided by the pulmonary arterial pulse pressure. The plots of Rp and Cp demonstrate a unique hyperbolic relationship, namely, the resistor–capacitor coupling curve, which represents the pulmonary vascular condition. The product of Rp and Cp is the exponential pressure decay, which refers to the time constant. Alterations in Cp are more considerable in CHD patients at an early stage of developing pulmonary hypertension or with excessive pulmonary blood flow due to a left-to-right shunt. The importance of Cp has gained attention because recent reports have shown that low Cp potentially reflects poor prognosis in patients with CHD-PH and idiopathic pulmonary hypertension. It is also known that Cp levels decrease in specific populations, such as preterm infants and trisomy 21. Therefore, both Rp and Cp should be individually evaluated in the management of children with CHD-PH who have different disease conditions.

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“…It is a metabolic byproduct of hemoglobin breakdown that also acts as an endogenous antioxidant molecule [ 9 ]. Total bilirubin (TBIL) is well established prognostic factor in heart failure [ 10 ] and PAH [ 8 ]. However, most prior PAH studies focused on patients with predominantly idiopathic or connective tissue disease-related cases.…”

Section: Introductionmentioning

confidence: 99%

Noninvasive Monitoring of Severe Pulmonary Artery Hypertension in Atrial Septal Defect Patients: Role of Serum Bilirubin Combined with Uric Acid

Zhang,

Lin,

Jin

et al. 2024

Rev. Cardiovasc. Med.

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Background: Atrial septal defect (ASD) patients commonly experience severe pulmonary arterial hypertension (SPAH), which is frequently associated with a poor prognosis. While serum bilirubin levels, indicative of liver function, are known predictors of right heart failure (RHF), their potential to differentiate SPAH in ASD patients is yet to be ascertained. The purpose of this study was to discover the potential correlations between serum bilirubin levels and ASD patients with SPAH. Methods: In this cross-sectional study, 102 ASD patients admitted from December 2019 to November 2020 were enrolled and divided into two cohorts: those with SPAH and those without. Blood tests were conducted to measure serum direct bilirubin (DBIL), total bilirubin (TBIL), alanine aminotransferase (ALT), aspartate aminotransferase (AST), uric acid (UA) and N-terminal pro B-type natriuretic peptide (NT-proBNP). Additionally, all participants underwent transthoracic echocardiography, and invasive hemodynamic data were gathered through right heart catheterization. Results: ASD patients with SPAH exhibited significantly elevated serum DBIL (5.2 3.0 vs. 2.4 1.5 µmol/L, p 0.001) and TBIL (24.6 20.7 vs. 10.1 4.8 µmol/L, p 0.001) levels in comparison to those without SPAH. However, ALT and AST levels remained comparable between the cohorts. Additionally, the SPAH cohort displayed higher serum UA (403.5 131.6 vs. 317.8 67.9 µmol/L, p 0.001) and NT-proBNP levels. Serum DBIL levels, when analyzed independently of other variables, correlated with an increased risk of mean pulmonary arterial pressure (mPAP) in ASD patients ( = 1.620, p = 0.010). A DBIL concentration of 2.15 mg/dL effectively differentiated ASD patients with SPAH from those without, with a sensitivity of 92.9% and a specificity of 51.4% (area under the curve [AUC]: 0.794, 95% confidence interval [CI]: 0.701–0.886, p 0.001). Notably, the combination of DBIL and UA had a higher sensitivity of 92.9% and specificity of 71.6% (AUC: 0.874, 95% CI: 0.799–0.949, p 0.001). Conclusions: Elevated serum DBIL and TBIL levels in ASD patients with SPAH were correlated with poor cardiac function and heightened pulmonary artery pressure. The combination of DBIL and UA has emerged as a strong noninvasive predictor for SPAH in ASD patients, presenting a potentially novel therapeutic biomarker.

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Pulmonary arterial compliance is a useful predictor of pulmonary vascular disease in congenital heart disease

Cited by 8 publications

References 19 publications

Lifetime risk of comorbidity in patients with simple congenital heart disease: a Danish nationwide study

Lifetime risk of comorbidity in patients with simple congenital heart disease: a Danish nationwide study

Comprehensive assessments of pulmonary circulation in children with pulmonary hypertension associated with congenital heart disease

Noninvasive Monitoring of Severe Pulmonary Artery Hypertension in Atrial Septal Defect Patients: Role of Serum Bilirubin Combined with Uric Acid

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