The Role of Catheter-Based and Surgical Treatments in Patients With Congenital Heart Disease and Pulmonary Hypertension

2013 ◽  
Vol 11 (4) ◽  
pp. 189-195 ◽  
Author(s):  
Jamil A. Aboulhosn
Keyword(s):  
Heart Failure ◽  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Congenital Heart ◽  
Initial Period ◽  
Volume Overload ◽  
Heart Volume ◽  
Pulmonary Arterial ◽  
Transcatheter Interventions

This manuscript is intended to provide a brief overview of the indications for and outcomes of surgical and transcatheter interventions for congenital heart disease and pulmonary hypertension (PH). Pulmonary hypertension is frequently encountered in children and adults with congenital heart disease and is most commonly related to large “central” shunts, ie, those occurring at the ventricular or great arterial level (Figure 1). If uncorrected early in infancy or childhood, large central shunts result in increased pulmonary blood flow, left heart volume overload, PH, and heart failure. If the child survives this initial period of volume overload and heart failure, they will very likely develop effacement of the normal pulmonary arterial architecture and severe elevations in pulmonary arterial resistance, eventually resulting in cyanosis and Eisenmenger syndrome.1

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

Abstract 14610: Renal Dysfunction in Adults With Congenital Heart Disease in Vietnam

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Ngoc Thanh Kim ◽  
Thanh Tung Le ◽  
Doan Loi Do ◽  
Thanh Huong Truong
Keyword(s):  
Risk Factors ◽  
Heart Failure ◽  
Atrial Fibrillation ◽  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Renal Dysfunction ◽  
Congenital Heart ◽  
Pulmonary Arterial

Introduction: In Vietnam, knowledge about renal function in adults with congenital heart disease (CHD) is limited. Hypothesis: This study aims to estimate incidence of renal dysfunction in adults with congenital heart disease and risk factors. Methods: This is a cross-sectional study, including 365 CHD patients more than 16 years old. We collected clinical and para-clinical information, estimated glomerular filtration rate (GFR) and calculated the odds ratio (OR) for reduced GFR. Results: Totally, 52.8% patients had GFR < 90 ml/phút/1.73 m 2 . Logistic regression had confirmed the OR for GFR < 90 ml/phút/1.73 m 2 in the group > 60-years-old, the group with atrial fibrillation, the group with heart failure (based on NT-proBNP > 125 pmol/L), and the group with pulmonary arterial hypertension (based on pulmonary artery systolic pressure > 50 mmHg by echocardiography) were 6.46 (95% CI: 1.37 - 30.41), 7.58 (95% CI: 1.66 - 34.56), 2.98 (95% CI: 1.49 - 5.98) and 1.84 (95% CI: 1.02 - 3.33), respectively. Conclusions: Renal dysfunction is common in adults with CHD. Age > 60 years-old, atrial fibrillation, heart failure, and pulmonary arterial hypertension were risk factors for renal dysfunction in adults with CHD.

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.

scholarly journals Predictor factors of pulmonary hypertension in children with left-to-right shunting in acyanotic congenital heart disease

2021 ◽  
Vol 61 (3) ◽  
pp. 119-24
Author(s):  
Weny Inrianto ◽  
Indah Kartika Murni ◽  
Ida Safitri
Keyword(s):  
Heart Failure ◽  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Iron Deficiency ◽  
Congenital Heart ◽  
Septal Defect ◽  
Deficiency Anemia ◽  
Acyanotic Congenital Heart Disease ◽  
Hypertension In Children

Background Left-to-right shunting in acyanotic congenital heart disease (CHD) is the most common type of defect in childhood heart disease. Limited access to specialist health services causes delays in CHD management. In limited resource settings, identification of factors that influence the occurrence of pulmonary hypertension is important in order to decide which patients should be prioritized for defect closure to prevent further complications. Objective To determine predictive factors of pulmonary hypertension after a left-to-right shunt CHD diagnosis. Methods This retrospective cohort study included children aged 1 month to 17 years with isolated atrial septal defect, or ventricular septal defect, or patent ductus arteriosus. Potential predictors studied were iron deficiency anemia, mitral regurgitation, pneumonia, and heart failure. Bivariate analysis was done with Chi-square test and multivariate analysis was done with Cox regression to determine the hazard ratio. Results Pulmonary hypertension occurred in 68 of 176 subjects. Iron deficiency anemia, mitral regurgitation, and pneumonia were not predictives of pulmonary hypertension. However, heart failure was a significant predictive factor for pulmonary hypertension, with a hazard ratio of 4.1 (95%CI 2.2 to 7.5; P=0.001). Conclusions Heart failure is a predictive factor of pulmonary hypertension in children with left-to-right shunting in acyanotic CHD.

scholarly journals Adult congenital heart disease and the COVID-19 pandemic

Heart ◽  
2020 ◽  
Vol 106 (17) ◽  
pp. 1302-1309 ◽  
Author(s):  
Robert M Radke ◽  
Tim Frenzel ◽  
Helmut Baumgartner ◽  
Gerhard-Paul Diller
Keyword(s):  
Heart Failure ◽  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
High Risk ◽  
Congenital Heart ◽  
Pragmatic Approach ◽  
Right Heart ◽  
High Risk Patients ◽  
Risk Patients

Adults with congenital heart disease (ACHD) may be at high risk in the case of COVID-19. Due to the heterogeneity of ACHD and secondary complications, risk profiles are, however, not uniform. This document aims to give an overview of relevant data and outline our pragmatic approach to disease prevention and management. Based on anatomy and additional physiological factors including symptoms, exercise capacity, heart failure, pulmonary hypertension and cyanosis, we propose a pragmatic approach to categorising patients into low-risk, intermediate-risk and high-risk groups. We regard especially patients with complex cyanotic conditions, those with palliated univentricular hearts, heart failure, severe valvular disease or pulmonary hypertension as high-risk patients. To avoid infection, we recommend self-isolation and exemption from work for these cohorts. Infected ACHD patients with low or moderate risk and without signs of deterioration may be remotely followed and cared for at home while in self isolation. High-risk patients or those with signs of respiratory or cardiovascular impairment require admission ideally at a tertiary ACHD centre. Especially patients with complex, cyanotic disease, heart failure and arrhythmias require particular attention. Treatment in patients with cyanotic heart disease should be guided by the relative degree of desaturation compared with baseline and lactate levels rather than absolute oxygen saturation levels. Patients with right heart dilatation or dysfunction are potentially at increased risk of right heart failure as mechanical ventilation and acute respiratory distress syndrome can lead to increase in pulmonary arterial pressures.

Hospitalisations for heart failure predict mortality in pulmonary hypertension related to congenital heart disease

Heart ◽  
2018 ◽  
Vol 105 (6) ◽  
pp. 465-469 ◽  
Author(s):  
Despoina Ntiloudi ◽  
Sotiria Apostolopoulou ◽  
Konstantinos Vasiliadis ◽  
Alexandra Frogoudaki ◽  
Aphrodite Tzifa ◽  
...  
Keyword(s):  
Heart Failure ◽  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Congenital Heart ◽  
Cox Regression ◽  
Strong Predictor ◽  
Adult Congenital Heart Disease ◽  
Significant Proportion ◽  
Cox Regression Analysis

ObjectiveDespite the progress in the management of patients with adult congenital heart disease (ACHD), a significant proportion of patients still develop pulmonary hypertension (PH). We aimed to highlight the rate of the complications in PH-ACHD and the predicting factors of cumulative mortality risk in this population.MethodsData were obtained from the cohort of the national registry of ACHD in Greece from February 2012 until January 2018.ResultsOverall, 65 patients receiving PH-specific therapy were included (mean age 46.1±14.4 years, 64.6% females). Heavily symptomatic (New York Heart Association (NYHA) class III/IV) were 53.8% of patients. The majority received monotherapy, while combination therapy was administered in 41.5% of patients. Cardiac arrhythmia was reported in 30.8%, endocarditis in 1.5%, stroke in 4.6%, pulmonary arterial thrombosis in 6.2%, haemoptysis in 3.1% and hospitalisation due to heart failure (HF) in 23.1%. Over a median follow-up of 3 years (range 1–6), 12 (18.5%) patients died. On univariate Cox regression analysis history of HF hospitalisation emerged as a strong predictor of mortality (HR 8.91, 95% CI 2.64 to 30.02, p<0.001), which remained significant after adjustment for age and for NYHA functional class.ConclusionsLong-term complications are common among patients with PH-ACHD. Hospitalisations for HF predict mortality and should be considered in the risk stratification of this population.

Pulmonary hypertension

2020 ◽  
pp. 3695-3710
Author(s):  
Nicholas W. Morrell
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lung Disease ◽  
Congenital Heart ◽  
Heart Function ◽  
Gene Encoding ◽  
Pulmonary Arterial

Symptoms of unexplained exertional breathlessness or symptoms out of proportion to coexistent heart or lung disease should alert the clinician to the possibility of pulmonary hypertension, and the condition should be actively sought in patients with known associated conditions, such as scleroderma, hypoxic lung disease, liver disease, or congenital heart disease. Heterozygous germ-line mutations in the gene encoding the bone morphogenetic protein type II receptor (BMPR2) are found in over 70% of families with pulmonary arterial hypertension. Pulmonary hypertension is defined as a mean pulmonary arterial pressure greater than 25 mm Hg at rest, and may be due to increased pulmonary vascular resistance (e.g. pulmonary arterial hypertension), increased transpulmonary blood flow (e.g. congenital heart disease), or increased pulmonary venous pressures (e.g. mitral stenosis). Exercise tolerance and survival in pulmonary hypertension is ultimately related to indices of right heart function, such as cardiac output.

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