Can Pulmonary Vasodilator Therapy Expand the Operative Indications for Congenital Heart Disease?

Inai, Kei

doi:10.1536/ihj.14-396

Cited by 4 publications

(3 citation statements)

References 57 publications

(71 reference statements)

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“…[28] The limited exercise tolerance and exertional dyspnea could remain persistent for years, which identify a risk for hospitalization, severe cardiovascular events, or death point. Inai [29] concluded that pulmonary vasodilators are probably available to interfere with the ongoing disease process to improve functional capacity and delay the decision for transplantation with no conclusive evidence. Although retrospective studies suggested ES patients with PAH-SDT benefitted from a longer lifespan, improved hemodynamics lasting period for operation, no recommendations for patients with ES have existed.…”

Section: Discussionmentioning

confidence: 99%

What is the position of pulmonary arterial hypertension-specific drug therapy in patients with Eisenmenger syndrome

Kuang

et al. 2019

Medicine

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Background: It is commonly reported a limitation of therapeutic strategy in Eisenmenger syndrome (ES) historically. This qualitative systematic review is conducted to evaluate the safety and efficacy of pulmonary arterial hypertension-specific drug therapy (PAH-SDT) for ES patients for a clinical therapeutic strategy based on evidence. Methods: PubMed, EMBASE, and the Cochrane Library databases have been systematically reviewed up to January 2019. Two reviewers independently conducted a literature search, quality evaluation, and data extraction. The occurrence of death, deterioration, and adverse events (AEs) has respectively been described as a count or percentage. Meta-analysis was conducted by Stata 15.1, and weighted mean differences (WMD) with 95% confidence intervals (CI) were recorded for continuous data. Randomized-effect model or fixed-effect model was applied according to the heterogeneity test. Results: Fifteen citations recruiting 456 patients associated with ES were eventually pooled, which involved 4 RCTs, 6 prospective studies, and 5 retrospective studies. Within the first year, it indicated PAH-SDT significantly ameliorated exercise capacity in 6-minute walk distance (6MWD) ( I 2 = 60.5%; WMD: 53.86 m, 95% CI [36.59, 71.13], P < .001), functional class (FC) (WMD = –0.71, 95% CI [–0.98, –0.44], P < .001) and Borg dyspnea index (WMD = –1.28, 95% CI [–1.86, –0.70], P < .001), in addition to hemodynamics, especially mean pulmonary arterial pressure by 5.70 mmHg (WMD = –5.70 mmHg, 95% CI [–8.19, –3.22], P < .001) and pulmonary vascular resistance by 4.20 wood U (WMD: –4.20, 95% CI [–7.32, –1.09], P = .008), but unsatisfactory effects in oxygen saturation at exercise ( P = .747). In a prolonged medication, bosentan, a dual ERA, has been proved acting an important role in improving exercise tolerance of patients with ES (6MWD: I 2 = 47.5%; WMD: 88.68 m, 95% CI [54.05, 123.3], P < .001; FC: I 2 = 0.0%; WMD = –0.65, 95% CI [–1.10, –0.19], P = .006). While a nonsignificant change of 6MWD was noted in a long-term therapy of ambrisentan ( P = .385). There existed rare evidence about the efficacy and safety of macitentan, phosphodiesterase-5 inhibitors (PDE5i), and prostanoids in a prolonged medication. Most AEs were recorded as mild to moderate with PAH-SDT, but about 4.3% individuals treated with endothelin receptor antagonists (ERAs) suffered from serious ones, and 3.9% suffered from death. Conclusions: This systematic review and meta-analysis proved PAH-SDT as a safe and effective role in ES in an early stage. However, in...

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Section: Discussionmentioning

confidence: 99%

What is the position of pulmonary arterial hypertension-specific drug therapy in patients with Eisenmenger syndrome

Kuang

et al. 2019

Medicine

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“…2-4) Recently, the survival of newborns with CHDs has increased due to massive breakthroughs in cardiovascular diagnostics and cardiothoracic surgery, 5,6) however, this trend consequently generates a completely novel and steady increasing population with grown-up congenital heart disease (GUCH). Patients with GUCH often need long-term expert medical care with high healthcare-related costs.…”

Section: Discussionmentioning

confidence: 99%

A Meta-Analysis of the Relationship Between Maternal Folic Acid Supplementation and the Risk of Congenital Heart Defects

Cao

et al. 2016

Int. Heart J.

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SummaryControversial opinions exist with respect to the relationship between maternal folic acid (FA) supplementation and birth prevalence of congenital heart defects (CHDs).Eligible articles were retrieved by searching databases, including PubMed, Cochrane library, EMBASE, CNKI, and WanFang up to September 2015. A meta-analysis was performed to evaluate the effects of FA on CHDs. Odds ratios (ORs) and 95% confidence interval (CIs) were merged using STATA 12.0. Meta-regression analysis was used to explore the possible sources of heterogeneity. Subgroup analysis according to the selected sources was also performed. Publication bias was assessed by Egger's test.Twenty studies were included in the meta-analysis. The overall analysis showed that FA supplementation was significantly associated with decreased risk of CHDs. The meta-regression analysis showed that geographical area could be an important source of heterogeneity. The subgroup analysis based on the geographical area revealed that FA supplementation during pregnancy was a protective factor against CHDs in Chinese and European patients, but not in American patients. Subgroup analysis according to literature quality also displayed positive associations between FA supplementation and the decreased risk of CHDs of China.FA supplementation during pregnancy significantly decreases the risk of CHDs in newborns in China and Europe. (Int Heart J 2016; 57: 725-728) Key words: Literature search, Meta-regression analysis, Geographical area, Pregnancy C ongenital heart defects (CHDs) are the most common structural abnormalities presenting at birth, and they are also one of the leading causes of perinatal and infant mortality.1) It is reported that the prevalence of CHDs accounts for 6% of all neonatal death factors, and also accounts for 46% of all congenital lethal factors.2-4) Recently, the survival of newborns with CHDs has increased due to massive breakthroughs in cardiovascular diagnostics and cardiothoracic surgery, 5,6) however, this trend consequently generates a completely novel and steady increasing population with grown-up congenital heart disease (GUCH). Patients with GUCH often need long-term expert medical care with high healthcare-related costs. 7) Moreover, recently, an increasing number of women have postponed conception to an older age, which consequently results in a higher birth prevalence of congenital defects. 8,9) These defects lead to great economic pressure and mental burdens on society and the families, therefore, searching for effective prevention measures for CHDs is of great practical significance.Folic acid (FA) is an essential nutrient and plays an important role in the development of the cardiovascular system.10-12) The association of FA or multivitamins containing FA supplementation during the critical periods of organ formation with the risk of the occurrence of CHDs has been recognized in past decades. [13][14][15] Several studies have reported that FA or multivitamins containing FA supplementation taken during pregnancy could signifi...

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“…CHD is the most common type of congenital deformity, classified into three types based on hemodynamics: no shunt, left to right shunt, and right to left shunt. [405][406][407] Patients with cyanotic CHD (CCHD) might have a hypoxic response, which leads to abnormalities in endothelial function, vascular remodeling, and thrombosis after emergency surgery. 408 Prolyl-4-hydroxylase2 (PHDP2)/HIF-1α pathway is the key regulator under hypoxia.…”

Section: Hypoxia In Arrhythmiamentioning

confidence: 99%

Hypoxia signaling in human health and diseases: implications and prospects for therapeutics

Luo

Tian

Yang³

et al. 2022

Sig Transduct Target Ther

159

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Molecular oxygen (O2) is essential for most biological reactions in mammalian cells. When the intracellular oxygen content decreases, it is called hypoxia. The process of hypoxia is linked to several biological processes, including pathogenic microbe infection, metabolic adaptation, cancer, acute and chronic diseases, and other stress responses. The mechanism underlying cells respond to oxygen changes to mediate subsequent signal response is the central question during hypoxia. Hypoxia-inducible factors (HIFs) sense hypoxia to regulate the expressions of a series of downstream genes expression, which participate in multiple processes including cell metabolism, cell growth/death, cell proliferation, glycolysis, immune response, microbe infection, tumorigenesis, and metastasis. Importantly, hypoxia signaling also interacts with other cellular pathways, such as phosphoinositide 3-kinase (PI3K)-mammalian target of rapamycin (mTOR) signaling, nuclear factor kappa-B (NF-κB) pathway, extracellular signal-regulated kinases (ERK) signaling, and endoplasmic reticulum (ER) stress. This paper systematically reviews the mechanisms of hypoxia signaling activation, the control of HIF signaling, and the function of HIF signaling in human health and diseases. In addition, the therapeutic targets involved in HIF signaling to balance health and diseases are summarized and highlighted, which would provide novel strategies for the design and development of therapeutic drugs.

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Can Pulmonary Vasodilator Therapy Expand the Operative Indications for Congenital Heart Disease?

Cited by 4 publications

References 57 publications

What is the position of pulmonary arterial hypertension-specific drug therapy in patients with Eisenmenger syndrome

What is the position of pulmonary arterial hypertension-specific drug therapy in patients with Eisenmenger syndrome

A Meta-Analysis of the Relationship Between Maternal Folic Acid Supplementation and the Risk of Congenital Heart Defects

Hypoxia signaling in human health and diseases: implications and prospects for therapeutics

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