scholarly journals 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

2021 ◽  
Vol 10 ◽  
pp. 204800402098221
Author(s):  
Kritvikrom Durongpisitkul ◽  
Paweena Chungsomprasong ◽  
Chodchanok Vijarnsorn ◽  
Prakul Chanthong ◽  
Supaluck Kanjanauthai ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Congenital Heart ◽  
Functional Class ◽  
Low Risk ◽  
Right Atrial ◽  
Risk Scores ◽  
Open Label ◽  
Risk Criteria

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.

scholarly journals Prevalence and profile of congenital heart disease and pulmonary hypertension in Down syndrome in a pediatric cardiology service

2014 ◽  
Vol 32 (2) ◽  
pp. 159-163 ◽  
Author(s):  
Felipe Alves Mourato ◽  
Lúcia Roberta R. Villachan ◽  
Sandra da Silva Mattos
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Down Syndrome ◽  
Heart Disease ◽  
Congenital Heart ◽  
Septal Defect ◽  
Heart Diseases ◽  
Heart Defects ◽  
Descriptive Analysis ◽  
Atrioventricular Septal Defect

OBJECTIVE:To determine the frequence and profile of congenital heart defects in Down syndrome patients referred to a pediatric cardiologic center, considering the age of referral, gender, type of heart disease diagnosed by transthoracic echocardiography and its association with pulmonary hypertension at the initial diagnosis.METHODS:Cross-sectional study with retrospective data collection of 138 patients with Down syndrome from a total of 17,873 records. Descriptive analysis of the data was performed, using Epi-Info version 7.RESULTS: Among the 138 patients with Down syndrome, females prevailed (56.1%) and 112 (81.2%) were diagnosed with congenital heart disease. The most common lesion was ostium secundum atrial septal defect, present in 51.8%, followed by atrioventricular septal defect, in 46.4%. Ventricular septal defects were present in 27.7%, while tetralogy of Fallot represented 6.3% of the cases. Other cardiac malformations corresponded to 12.5%. Pulmonary hypertension was associated with 37.5% of the heart diseases. Only 35.5% of the patients were referred before six months of age.CONCLUSIONS: The low percentage of referral until six months of age highlights the need for a better tracking of patients with Down syndrome in the context of congenital heart disease, due to the high frequency and progression of pulmonary hypertension.

scholarly journals Nutritional assessment and associated factors in children with congenital heart disease in Ethiopia.

2021 ◽  
Author(s):  
Temesgen Tsega Desta
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Associated Factors ◽  
Congenital Heart ◽  
Nutritional Assessment ◽  
Failure To Thrive ◽  
Cross Sectional ◽  
Care Givers ◽  
Increased Risk

ABSTRACT Infants and children with congenital heart disease exhibit a range of delays in weight gain and growth. In some instances, the delay can be relatively mild, whereas in other cases, cause the failure to thrive. OBJECTIVES To determine the nutritional status and associated factors of pediatric patients with congenital heart disease. MATERIAL AND METHODS A cross sectional analytical study was done over a period of 6months (Feb to Jul 2020). A total of 228 subjects with congenital heart disease that come to the cardiac center during the study period where included until the calculated sample size was attained. Data was collected from patient card and care givers of the children included in the study after obtaining their informed consent using data inquiry sheet. RESULTS A total of 228 children from age 3month to 17yrs. Most of the subjects had acyanotic heart disease accounting for 87.7%. The overall prevalence of wasting, underweight and stunting were 41.3%, 49.1% and 43% respectively. Among this children with congenital heart disease those with PAH were found more likely have wasting compared to those without PAH with an odds of 1.9 (95% CI: 1.0-3.4) and also greater chance of being stunted with an odds of 1.9 (95% CI: 1.0-3.4). children above 5years of age were 2.3 times more likely to be underweight. CONCLUSION Malnutrition is a major problem of patients with CHD. Pulmonary hypertension and older age are associated with increased risk of undernutrition. KEYWORDS: Acyanotic, cyanotic, Pulmonary hypertension, underweight, wasting and stunting.

Pulmonary hypertension and congenital heart disease: An insight from the REHAP National Registry

2015 ◽  
Vol 184 ◽  
pp. 717-723 ◽  
Author(s):  
Rafael Alonso-Gonzalez ◽  
Carmen Jiménez Lopez-Guarch ◽  
Maria Teresa Subirana-Domenech ◽  
José María Oliver Ruíz ◽  
Isabel Otero González ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Congenital Heart ◽  
National Registry

scholarly journals Is it possible to operate four heart valves in a patient with heart failure, congenital heart disease and pulmonary hypertension?

Cor et Vasa ◽  
2012 ◽  
Vol 54 (9-10) ◽  
pp. e295-e299 ◽  
Author(s):  
Marie Zvěřinová ◽  
Jana Popelová ◽  
Petr Pavel ◽  
Roman Gebauer ◽  
Pavel Jehlička ◽  
...  
Keyword(s):  
Heart Failure ◽  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Congenital Heart ◽  
Heart Valves

Pulmonary hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 781-784
Author(s):  
Shahin Moledina ◽  
Bejal Pandya
Keyword(s):  
Quality Of Life ◽  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Cardiac Surgery ◽  
Heart Disease ◽  
Congenital Heart ◽  
Eisenmenger Syndrome ◽  
Pulmonary Arterial ◽  
Careful Assessment

Congenital heart disease is a major cause of pulmonary arterial hypertension (PAH) and this can largely be prevented by early repair. PAH in the presence of systemic-to-pulmonary communication, leads to shunt reversal and cyanosis, with multiple systemic consequences (Eisenmenger syndrome). Congenital heart disease patients with PAH are vulnerable and are at high risk from non-cardiac surgery, pregnancy, and inappropriate medical treatment (e.g. excessive venesection). Survival is reduced, but is better than in idiopathic PAH. Recommendations for surgery should be based on careful assessment by experts. Modern PAH pharmacotherapy is showing promise in improving quality of life.

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