scholarly journals Role of cardiac magnetic resonance in stratifying the prognosis of patients with pulmonary arterial hypertension

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
A De Lorenzis ◽  
F Dardi ◽  
D Guarino ◽  
M Palazzini ◽  
I Magnani ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Wall Thickness ◽  
Congenital Heart ◽  
Univariate Analysis ◽  
Occlusive Disease ◽  
Systolic Volume ◽  
Disease Aetiology ◽  
End Systolic Volume ◽  
Pulmonary Arterial

Abstract Background Pulmonary arterial hypertension (PAH) is a rare disease characterized by a complex remodeling of heart structures. Cardiac magnetic resonance (CMR) is the gold standard for a non-invasive evaluation of right ventricle (RV) volumes and mass. Purpose To define the relationship between clinical, functional, biochemical, haemodynamic and CMR parameters and survival in patients with PAH. Methods Consecutive patients with PAH referred to our centre underwent clinical, functional, brain natriuretic peptide (BNP) plasma levels, haemodynamic and CMR evaluation. All patients were treated according to current guidelines. Univariate Cox analysis for survival was performed. Parameters with a p-value <0.1 at the univariate analysis were included in the multivariate analysis. Results One hundred forty-seven patients with PAH (mean age 49±17 years, 69% female) were included in the study. Etiology of PAH was: idiopathic/heritable (49%), associated with connective tissue disease (19%), congenital heart disease (12%), portal hypertension/HIV infection (12%) and pulmonary veno-occlusive disease (8%). Thirty-six patients died during follow-up. Parameters significantly associated with mortality at the univariate analysis were age [Hazard Ratio (95% Confidence Interval): 1.043 (1.020–1.067); p<0.001], six-minute walk test (6MWT) [HR: 0.995 (0.993–0.998); p<0.001], WHO-functional class [HR: 2.489 (1.025–6.041); p=0.044], idiopathic-heritable-congenital heart disease aetiology [HR: 0.182 (0.085–0.389); p<0.001], connective tissue disease aetiology [HR: 2.274 (1.099–4.704); p=0.027], pulmonary veno-occlusive disease aetiology [HR: 5.864 (2.328–14.773); p<0.001], right atrial pressure [HR: 1.098 (1.032–1.169); p=0.003], pulmonary artery oxygen saturation [HR: 0.947 (0.921–0.975); p<0.001], BNP levels [HR: 2.214 (1.213–4.039); p=0.010], RV wall thickness [HR: 0.633 (0.399–1.006); p=0.053], RV end diastolic volume [HR: 1.012 (1.003–1.021); p=0.007], RV end systolic volume [HR: 1.014 (1.003–1.024); p=0.011]. Parameters independently associated with mortality at the multivariate analysis were age [HR: 1.035 (1.006–1.064); p=0.018], idiopathic-heritable-congenital heart disease aetiology [HR: 0.355 (0.146–0.860); p=0.022], pulmonary veno-occlusive disease aetiology [HR: 3.129 (1.071–9.143); p=0.037], pulmonary artery oxygen saturation [HR: 0.953 (0.919–0.989); p=0.011], RV wall thickness [HR: 0.527 (0.300–0.927); p=0.026], RV end systolic volume [HR: 1.016 (1.003–1.029); p=0.014]. Conclusion RV wall thickness and RV end-systolic volume are associated with prognosis in patients with PAH independently from clinical and haemodynamic characteristics. These parameters may be used in the overall risk stratification of PAH patients. FUNDunding Acknowledgement Type of funding sources: None.

scholarly journals Pulmonary Arterial Hypertension Associated with Congenital Heart Disease in Adults over the Age of 40 Years

2020 ◽  
Vol 9 (12) ◽  
pp. 4071
Author(s):  
Susanne J. Maurer ◽  
Katharina Stöckemann ◽  
Claudia Pujol ◽  
Jürgen Hörer ◽  
Peter Ewert ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Congenital Heart ◽  
Univariate Analysis ◽  
All Cause Mortality ◽  
Pulmonary Arterial ◽  
Significant Mortality

Background: Pulmonary arterial hypertension associated with adult congenital heart disease (PAH-ACHD) leads to significant mortality at a young age. Risk factors for a negative outcome in older adults are lacking. Methods: PAH-ACHD patients ≥ 40 years of age under active follow-up between January 2005 and December 2018 were included. Demographic data, as well as medical/surgical history, were retrieved from hospital records. The primary end-point was all-cause mortality. Results: In total, 65 patients (67.7% female, mean age 45.19 ± 6.75 years) were included. Out of these, 46 (70.8%) had a shunt lesion, 12 (18.5%) had PAH associated with complex congenital heart defects, and 7 (10.8%) had segmental pulmonary hypertension due to major aorto-pulmonary collaterals. Down syndrome was present in 13 patients (20.0%). During a median follow-up of 4.2 years (IQR 1.2–7.5), 16 patients (24.6%) died. On univariate analysis, NT-proBNP (log), creatinine, and a previous history of ventricular arrhythmias were predictors of all-cause mortality. Upon multivariate analysis, NT-proBNP (log) (HR: 4.1, 95% CI: 1.2–14.4, p = 0.029) and creatinine (HR: 16.3, 95% CI: 2.2–118.7, p = 0.006) remained as independent predictors of all-cause mortality. Conclusions: PAH-ACHD patients over the age of 40 years are burdened with significant mortality, of which NT-proBNP and creatinine are independent predictors.

Pulmonary Arterial Hypertension In Adults With Congenital Heart Disease: General Overview of Disease Mechanisms

2007 ◽  
Vol 6 (3) ◽  
pp. 121-125 ◽  
Author(s):  
Ingram Schulze-Neick ◽  
John E. Deanfield
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Congenital Heart ◽  
Vascular System ◽  
Hypertensive Crisis ◽  
Multidisciplinary Care ◽  
Structural Heart Disease ◽  
Pulmonary Arterial

Adults with congenital heart disease (CHD) have become a rapidly expanding group of complex patients requiring multidisciplinary care in specialty centers by those trained in CHD. They represent one of the most challenging subgroups of patients with pulmonary arterial hypertension (PAH) due to the presence of structural heart disease with or without coexisting cyanosis and its complications. The primary focus of attention for these patients is the lungs, whose vascular system is affected by shunt flow, or is also congenitally malformed, or has been altered by surgical procedures. When PAH develops, it affects physical exercise tolerance, travel to high altitudes, pregnancy, operability, and anesthesia (myocardial failure due to pulmonary hypertensive crisis), and thus general morbidity and mortality in this special patient group.

scholarly journals Strategies for the management of pulmonary arterial hypertension in patients with congenital heart disease

2020 ◽  
Vol 4 (S1) ◽  
Author(s):  
Nathalie Liew ◽  
Zoya Rashid ◽  
Robert Tulloh
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Congenital Heart ◽  
Standard Management ◽  
Advanced Therapies ◽  
Pulmonary Arterial ◽  
The Uk

Abstract Background Pulmonary hypertension (PH) is commonly seen in adults who have congenital heart disease (CHD). Therapy is available for pulmonary arterial hypertension (PAH) and has greatly benefitted many patients with PAH related to CHD (PAH-CHD) over the last 15 years, with evidence of improved quality of life and prognosis in those with Eisenmenger syndrome and repaired PAH-CHD. In this review, we describe the standard management and advanced therapies for PAH, which are available in specialist PH centres around the UK and Ireland, and how these are used in PAH-CHD. Decisions around the choice of therapy are governed by commissioning and available evidence. Conclusion We explain the different pathways for action and the variety of medications now at our disposal to help this important group of patients.

scholarly journals Predictive role of perioperative neutrophil to lymphocyte ratio in pediatric congenital heart disease associated with pulmonary arterial hypertension

BMC Surgery ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Xiaoqiang Yin ◽  
Mei Xin ◽  
Sheng Ding ◽  
Feng Gao ◽  
Fan Wu ◽  
...  
Keyword(s):  
Mechanical Ventilation ◽  
Congenital Heart Disease ◽  
Cardiac Surgery ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Congenital Heart ◽  
Prolonged Mechanical Ventilation ◽  
Pulmonary Arterial ◽  
Mechanical Ventilation Time

Abstract Background We aimed to explore the relationship between the neutrophil to lymphocyte ratio (NLR) and the early clinical outcomes in children with congenital heart disease (CHD) associated with pulmonary arterial hypertension (PAH) after cardiac surgery. Methods A retrospective observational study involving 190 children from January 2013 to August 2019 was conducted. Perioperative clinical and biochemical data were collected. Results We found that pre-operative NLR was significantly correlated with AST, STB, CR and UA (P < 0.05), while post-operative NLR was significantly correlated with ALT, AST, BUN (P < 0.05). Increased post-operative neutrophil count and NLR as well as decreased lymphocyte count could be observed after cardiac surgery (P < 0.05). Level of pre-operative NLR was significantly correlated with mechanical ventilation time, ICU stay time and total length of stay (P < 0.05), while level of post-operative NLR was only significantly correlated to the first two (P < 0.05). By using ROC curve analysis, relevant areas under the curve for predicting prolonged mechanical ventilation time beyond 24 h, 48 h and 72 h by NLR were statistically significant (P < 0.05). Conclusion For patients with CHD-PAH, NLR was closely related to early post-operative complications and clinical outcomes, and could act as a novel marker to predict the occurrence of prolonged mechanical ventilation.

scholarly journals Contemporary use of Selexipag in pulmonary arterial hypertension associated with congenital heart disease: a case series

2020 ◽  
Author(s):  
Sarah Blissett ◽  
David Blusztein ◽  
Vaikom S Mahadevan
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Side Effects ◽  
Arterial Hypertension ◽  
Exercise Capacity ◽  
Congenital Heart ◽  
Pulmonary Veins ◽  
Case Series ◽  
Pulmonary Arterial

Abstract Background There are significant risks of parenteral prostacyclin use in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), which may limit their use. Selexipag is an oral, selective prostacyclin analogue that has been shown to reduce disease progression and improve exercise capacity in patients with PAH-CHD. Administering Selexipag in patients with PAH-CHD could potentially overcome some of the risks of parenteral therapy while improving clinical outcomes. Case summary We report five cases highlighting the clinical uses of Selexipag in patients with PAH-CHD. In the first two cases, Selexipag was initiated as part of a Treat-to-close strategy. In the third case, initiation of Selexipag improved symptoms and objective exercise capacity in a patient with Eisenmenger syndrome. In the fourth and fifth cases, rapid cross-titration protocols were used to transition from parenteral prostacyclins to Selexipag. In the fourth case, Selexipag was initiated in the context of significant side effects limiting parenteral prostacyclin use. In the fifth case, Selexipag was used to down-titrate from parenteral prostacyclins following closure of a sinus venosus atrial septal defect and redirection of anomalous pulmonary veins. Discussion Selexipag is a promising oral therapy for patients with at various stages of the spectrum of PAH-CHD to improve symptoms, exercise capacity and, in some cases, haemodynamics. Our cases also highlight practical aspects of Selexipag use including targeting the individualized maximally tolerated dose for each patient, managing side effects and managing dose interruptions.

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