The efficiency of endothelin receptor antagonist bosentan for pulmonary arterial hypertension associated with congenital heart disease

Kuang, Hong; Wu, Yu; Yi, Qi; Tian, Jie; Wu, Chun; Shou, Weinian; LU, Tie

doi:10.1097/md.0000000000010075

Cited by 18 publications

(17 citation statements)

References 33 publications

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“…20 Many studies have shown that the usage of drugs has certain impact on congenital heart disease, such as anti-infective drugs, sulfonamides have significant impact on the normal development of cardiovascular system, but the relationship between penicillin and congenital heart disease is not certain. 21,22 Antiasthma, antidepressant, antifungal drugs and drugs for treating hypertension and hypothyroidism all increased the incidence of congenital heart disease. 23 Several large-scale studies have shown that pregnant women who took progesterone, amphetamine, anticancer drugs and methionine in the early pregnancy significantly increased the risk of developing CHD in their offspring.…”

Section: Discussionmentioning

confidence: 99%

WITHDRAWN: A multicenter case-control study on non-genetic risk factors in infants with congenital heart disease

W¹,

Deng²,

Huang³

et al. 2019

Preprint

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Background: The incidence of congenital heart disease (CHD) is growing at a rapid speed worldwide, which due to changes of both environmental and lifestyle exposures. This study aimed to explore how the non-genetic risk factors of maternal and perinatal conditions influenced onset of CHD in infants.Methods: Infants with congenital heart disease diagnosed by echocardiography from May 2012 to December 2013 were recruited as positive cases, while healthy individuals without congenital heart disease recruited at the same period were regarded as controls. The general situation of parents and the exposure to environmental factors during perinatal period in the case group and the control group were investigated by questionnaires.Results: A total of424 questionnaires of infants with congenital heart disease and 362 healthy controls were finally collected. We analyzed the risk factors during perinatal period of their mothers showed that their mothers took drugs in early pregnancy (odds ratio [OR]=4.41, 95 % confidence interval [CI]:2.41-6.73), viral infection (OR = 2.97, 95% CI: 1.62-5.73), house decoration (OR = 1.82, 95% CI: 1.43-3.75), hair dyeing and scalding (OR = 2.24, 95% CI:1.46-4.05), parents' exposures to work environment pollution (OR = 2.77, 95% CI: 2.03-5.38) were independent risk factors for cardiovascular malformations. With the increase of exposure factors, the risk of congenital heart disease increased significantly. When pregnant women were exposed to three or more risk factors at the same time, the risk of infants with congenital heart disease in was 17.24 times higher than that of single-factor exposure.Conclusions: Together, the incidence of CHD in infants is correlated with the exposure to perinatal risk factors.

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Section: Discussionmentioning

confidence: 99%

WITHDRAWN: A multicenter case-control study on non-genetic risk factors in infants with congenital heart disease

W¹,

Deng²,

Huang³

et al. 2019

Preprint

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“…No obstante, la [endotelina-1]p (vasoconstrictor endotelial) continúa elevada, llevando a HAP persistente. Ante esto, algunos pacientes se conservan estables por un periodo de 3-5 años; en otros, el deterioro es rápido, lo que conduce a la falla ventricular derecha y muerte 14 .…”

Section: Discussionunclassified

Del soplo inocente a la hipertensión arterial pulmonar

Betancourt-Montoya¹,

Corona-Villalobos²

2019

BMHIM

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Introducción: La detección de cardiopatías congénitas en la etapa neonatal a partir de un soplo cardiaco o cianosis no es efectiva. Las cardiopatías congénitas críticas, como el tronco arterioso común (TAC), causan la mayoría de las muertes neonatales por malformaciones congénitas. El tamizaje por oximetría de pulso en los recién nacidos detecta hasta el 70% de estas cardiopatías. El TAC presenta una alta mortalidad en el primer año de vida. Caso clínico: Se presenta el caso de un paciente de sexo femenino de 4 años de edad con soplo cardiaco, palpitaciones, disnea y cianosis perioral, con diagnóstico al nacimiento de soplo inocente. Se detectó TAC mediante una ecocardiografía. Las resistencias vasculares pulmonares fueron evaluadas por medio de cateterismo cardiaco derecho, con hallazgo de hipertensión arterial pulmonar y vasorreactividad pulmonar. Se realizó corrección quirúrgica. A la fecha, la hipertensión arterial pulmonar continúa presente, por lo que se implementó Bosentan ® (Actelion, USA) como tratamiento a largo plazo. Conclusiones: En recién nacidos, el tamizaje por oximetría de pulso después de las 24 horas de vida es un método efectivo para el diagnóstico oportuno de cardiopatías congénitas críticas antes de los signos de colapso cardiovascular. Por ello, resulta una herramienta diagnóstica fundamental para reducir la morbimortalidad. Aunque la corrección quirúrgica de cardiopatías congénitas con hipertensión arterial pulmonar es factible en algunos pacientes, su manejo subsecuente es complejo e impacta de manera adversa en la calidad de vida.

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“…Recently, the MAESTRO study (macitentan in Eisenmenger syndrome to restore exercise capacity) did not show results that were superior to placebo in regards to the primary end-point of change from baseline at the end of 16 weeks of treatment. 6 Nevertheless, the metaanalysis with 456 pooled PAH-CHD patients treated by bosentan as an adds on therapy 7 showed significant improvement in the 6MWD and with respect to the World Health Organization functional class (FC) and an additional statistically significant difference in mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance index (PVRi). These mixed outcomes led to a hesitation in adding combination therapy, as recommended by the 2015 European Society of Cardiology (ESC)/European Respiratory Society (ERS) pulmonary hypertension (PH) guidelines for a risk assessment strategy.…”

Section: Introductionmentioning

confidence: 99%

Improved low-risk criteria scores for combination therapy of sildenafil and generic bosentan in patients with congenital heart disease with severe pulmonary hypertension: A prospective open label study

Durongpisitkul

Chungsomprasong

Vijarnsorn

et al. 2021

JRSM Cardiovascular Disease

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Objective We evaluated the efficacy and safety of the bosentan as a sequential add-on therapy with sildenafil in pulmonary arterial hypertension with congenital heart disease (PAH-CHD) patients. Material and method Twenty patients who were receiving sildenafil were given generic bosentan for up to a year. Hemodynamic data was collected from cardiac catheterization at pretreatment and at three months. Comparisons were made between the total scores of the four, low-risk criteria adapted from the 2015 ESC/ERS pulmonary hypertension guidelines, which are: 1) WHO functional class of I or II, 2) 6MWD of more than 440 m, 3) right atrial pressure of less than 8 mm Hg, and 4) cardiac index ≥2.5 L/min/m2, performed at the beginning of therapy, 3-months, 6-months, and 1 year. Results Patients’ average age was 27 ± 11 years old (12–53). PVRi decreased from 16.7 ± 9.5 to 12.7 ± 10.3 Wood unit (WU) m2 (p = 0.025) and PVRi/SVRi decreased from 0.69 ± 0.33 to 0.49 ± 0.32 (p = 0.001). During the follow-up, the composite scoring of the low risk scores for 19 patients was increased significantly from 1.8 ± 1.0 at baseline to 2.3 ± 0.9 at 3 months, to 2.9 ± 0.8 at 6 months, and 3 ± 0.7 at 1 year (p = 0.001). Conclusion We demonstrated intermediate term benefits for generic bosentan as an add-on therapy to sildenafil in patients with PAH-CHD by improving PVRi, and PVRi/SVRi at three months. A significant improvement was also seen in the combined scores of the low-risk criteria from below 2 to 3 at one year (p = 0.001). Thai Clinical Trials Registry (TCTR): TCTR identification number is TCTR20200506006.

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The efficiency of endothelin receptor antagonist bosentan for pulmonary arterial hypertension associated with congenital heart disease

Abstract: Supplemental Digital Content is available in the text

Cited by 18 publications

References 33 publications

WITHDRAWN: A multicenter case-control study on non-genetic risk factors in infants with congenital heart disease

WITHDRAWN: A multicenter case-control study on non-genetic risk factors in infants with congenital heart disease

Del soplo inocente a la hipertensión arterial pulmonar

Improved low-risk criteria scores for combination therapy of sildenafil and generic bosentan in patients with congenital heart disease with severe pulmonary hypertension: A prospective open label study

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