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.

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.

Sign in / Sign up

Export Citation Format

Share Document