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Biomedicine & Pharmacotherapy =... Oct 2020Neurogenic pulmonary edema (NPE) following acute stroke is an acute respiratory distress syndrome (ARDS) with clinical characteristics that include acute onset, apparent... (Review)
Review
Neurogenic pulmonary edema (NPE) following acute stroke is an acute respiratory distress syndrome (ARDS) with clinical characteristics that include acute onset, apparent pulmonary interstitial fluid infiltration and rapid resolution. The pathological process of NPE centers on sympathetic stimulation and fulminant release of catecholamines, which cause contraction of resistance vessels. Elevated systemic resistance forces fluid into pulmonary circulation, while pulmonary circulation overload induces pulmonary capillary pressure that elevates, and in turn damages the alveolar capillary barrier. Damage to the alveolar capillary barrier leads to pulmonary ventilation disorder, blood perfusion disorder and oxygenation disorder. Eventually, NPE will cause post-stroke patients' prognosis to further deteriorate. At present, we lack specific biological diagnostic indicators and a meticulously unified diagnostic criterion, and this results in a situation in which many patients are not recognized quickly and/or diagnosed accurately. There are no drugs that are effective against NPE. Therefore, understanding how to diagnose NPE early by identifying the risk factors and how to apply appropriate treatment to avoid a deteriorating prognosis are important scientific goals. We will elaborate the progress of NPE after acute stroke in terms of its pathophysiological mechanisms, etiology, epidemiology, clinical diagnosis and early prediction, comprehensive treatment strategies, and novel drug development. We also propose our own thinking and prospects regarding NPE.
Topics: Animals; Humans; Pulmonary Circulation; Pulmonary Edema; Respiratory Distress Syndrome; Stroke
PubMed: 32739737
DOI: 10.1016/j.biopha.2020.110478 -
Experimental Physiology Apr 2023
Topics: Humans; Oxygen; Vasoconstriction; Iron; Hypoxia; Pulmonary Disease, Chronic Obstructive; Pulmonary Gas Exchange; Pulmonary Circulation
PubMed: 36744659
DOI: 10.1113/EP091078 -
Circulation May 2022
Topics: Heart Atria; Heart Failure; Humans; Pulmonary Circulation; Stroke Volume; Ventricular Dysfunction, Left; Ventricular Function, Left
PubMed: 35605032
DOI: 10.1161/CIRCULATIONAHA.122.059810 -
Journal of the American College of... Dec 2020Pulmonary vascular disease, pulmonary endothelial dysfunction, liver fibrosis, renal disease, and exercise intolerance are common in adults with Fontan physiology....
BACKGROUND
Pulmonary vascular disease, pulmonary endothelial dysfunction, liver fibrosis, renal disease, and exercise intolerance are common in adults with Fontan physiology. Although the pathophysiologic mechanisms linking these phenomena have been studied, certain aspects are not well understood.
OBJECTIVES
This study hypothesized that impaired pulmonary vascular reserve (VR) plays a central role linking these abnormalities, and that patients with abnormal pulmonary VR with exercise, compared with patients with normal VR, would display poorer pulmonary endothelial function, greater liver stiffness, more renal dysfunction, and poorer exercise capacity.
METHODS
Symptomatic adults with the Fontan palliation (n = 29) underwent invasive cardiopulmonary exercise testing, echocardiography, and assessment of microvascular function. Abnormal pulmonary VR was defined by the slope of increase in pulmonary pressure relative to cardiac output with exercise >3 mm Hg/l/min. Pulmonary endothelial function was assessed using reactive hyperemia index. End-organ function was assessed using magnetic resonance elastography-derived liver stiffness, glomerular filtration rate, N-terminal pro-B-type natriuretic peptide, and peak oxygen consumption (Vo).
RESULTS
Compared with individuals with normal VR (n = 8), those with abnormal VR (n = 21) displayed higher central and pulmonary venous pressures, and more severely impaired cardiac output and stroke volume responses to exertion, but similar pulmonary vascular resistance at rest. Patients with abnormal VR displayed more severely impaired reactive hyperemia index, increased liver stiffness, lower glomerular filtration rate, higher N-terminal pro-B-type natriuretic peptide, and lower peak Vo. As compared to pulmonary vascular resistance at rest, slope of increase in pulmonary pressure relative to cardiac output displayed stronger correlations with reactive hyperemia index (r = -0.63 vs. r = -0.31; Meng test p = 0.009), magnetic resonance elastography-derived liver stiffness (r = 0.47 vs. r = 0.29; Meng test p = 0.07), glomerular filtration rate (r = -0.52 vs. r = -0.24; Meng test p = 0.03), N-terminal pro-B-type natriuretic peptide (r = 0.56 vs. r = 0.17; Meng test p = 0.02), and peak Vo (r = -0.63 vs. r = -0.26; Meng test p = 0.02).
CONCLUSIONS
Pulmonary vascular limitations in Fontan physiology are related to pulmonary endothelial and end-organ dysfunction, suggesting a mechanistic link between these commonly observed findings, and these abnormalities are more apparent during exercise testing, with little relationship at rest.
Topics: Adult; Cardiac Output; Endothelium, Vascular; Exercise; Exercise Test; Female; Fontan Procedure; Heart Defects, Congenital; Humans; Male; Peripheral Vascular Diseases; Prospective Studies; Pulmonary Circulation; Rest; Vascular Resistance; Young Adult
PubMed: 33272370
DOI: 10.1016/j.jacc.2020.10.003 -
Hypertension (Dallas, Tex. : 1979) Jun 2020Pulmonary hypertension (PH) due to left heart disease (LHD) is the most common type of PH and is defined as mean pulmonary artery systolic pressure of >20 mm Hg and... (Review)
Review
Pulmonary hypertension (PH) due to left heart disease (LHD) is the most common type of PH and is defined as mean pulmonary artery systolic pressure of >20 mm Hg and pulmonary capillary wedge pressure >15 mm Hg during right heart catheterization. LHD may lead to elevated left atrial pressure alone, which in the absence of intrinsic pulmonary vascular disease will result in PH without changes in pulmonary vascular resistance. Persistent elevation in left atrial pressure may, however, also be associated with subsequent pulmonary vascular remodeling, vasoconstriction, and an increase in pulmonary vascular resistance. Hence, there are 2 subgroups of PH due to LHD, isolated postcapillary PH and combined post- and precapillary PH, with these groups have differing clinical implications. Differentiation of pulmonary arterial hypertension and PH due to LHD is critical to guide management planning; however, this may be challenging. Older patients, patients with metabolic syndrome, and patients with imaging and clinical features consistent with left ventricular dysfunction are suggestive of LHD etiology rather than pulmonary arterial hypertension. Hemodynamic measures such as diastolic pressure gradient, transpulmonary gradient, and pulmonary vascular resistance may assist to differentiate pre- from postcapillary PH and offer prognostic insights. However, these are influenced by fluid status and heart failure treatment. Pulmonary arterial hypertension therapies have been trialed in the treatment with concerning results reflecting disease heterogeneity, variation in inclusion criteria, and mixed end point criteria. The aim of this review is to provide an updated definition, discuss possible pathophysiology, clinical aspects, and the available treatment options for PH due to LHD.
Topics: Disease Management; Heart Failure; Humans; Hypertension, Pulmonary; Pulmonary Circulation; Pulmonary Wedge Pressure
PubMed: 32336230
DOI: 10.1161/HYPERTENSIONAHA.119.14330 -
The Canadian Journal of Cardiology Jul 2022Fontan palliation results in a hemodynamically complex circulation with multisystem consequences, which in the long term adversely affect many body processes. Systemic... (Review)
Review
Fontan palliation results in a hemodynamically complex circulation with multisystem consequences, which in the long term adversely affect many body processes. Systemic venous hypertension, nonpulsatile low-shear pulmonary blood flow, and low cardiac output are the 3 main characteristics of a Fontan circulation, leading to unavoidable slowly progressive failure. An appreciation of how the hemodynamics of a Fontan circulation change with time and relate to the various modes of Fontan circulatory failure is important. Accurate hemodynamic assessment aid this understanding and may permit early identification of potentially treatable drivers of decline. While no evidence-based or guideline-directed pharmacologic management strategy has been established in Fontan patients, understanding the hemodynamics of Fontan circulation failure will assist in the rational selection of potentially helpful drug therapies for individual patients. In this review, we present hemodynamic concepts of the optimal Fontan physiology and Fontan circulatory failure, review practical aspects of invasive hemodynamic assessment, and discuss the role of drug therapies in increasing systemic venous blood flow return and decreasing ventricular filling pressures in Fontan circulation. Often complementary to catheter-based or surgical interventions, pharmacologic management aims at preserving patency of the circuit, adequate systolic and diastolic ventricular function, atrioventricular valve function, an unobstructed ventricular outflow tract, and pulmonary vascular integrity in order to maintain an acceptable cardiac output.
Topics: Fontan Procedure; Heart Defects, Congenital; Heart Ventricles; Hemodynamics; Humans; Pulmonary Circulation; Ventricular Function
PubMed: 35469974
DOI: 10.1016/j.cjca.2022.04.014 -
American Journal of Obstetrics &... Jan 2021The Fontan operation was first performed in 1968 and is a palliative procedure for children born with single ventricle forms of congenital heart disease. Today, 70,000... (Review)
Review
The Fontan operation was first performed in 1968 and is a palliative procedure for children born with single ventricle forms of congenital heart disease. Today, 70,000 patients worldwide have Fontan circulation today, half of them women, and with an expected 30-year survival of >80%, this population is expected to double in the next 20 years. The Fontan operation surgically redirects systemic venous blood return directly to the pulmonary circulation, bypassing the single ventricle. This abnormal anatomy results in significant challenges for the cardiovascular system and is marked by a sustained, abnormally elevated systemic venous pressure combined with decreased cardiac output. As more women with Fontan circulation reach childbearing age, understanding the unique risks of pregnancy to the mother and fetus and how to best provide clinical care for these women during pregnancy is imperative. However, there are limited clinical data to guide counseling and management in this population. This expert review offers an analysis of the literature about Fontan circulation during pregnancy and describes our center's current multidisciplinary approach to care for these women in the preconception, antepartum, intrapartum, and postpartum periods.
Topics: Child; Female; Fontan Procedure; Heart Defects, Congenital; Humans; Postpartum Period; Pregnancy; Pulmonary Circulation; Univentricular Heart
PubMed: 33451613
DOI: 10.1016/j.ajogmf.2020.100257 -
Journal of Biomechanical Engineering Feb 2022Pulmonary hypertension (PH) is a progressive disease that is characterized by a gradual increase in both resistive and reactive pulmonary arterial (PA) impedance....
Pulmonary hypertension (PH) is a progressive disease that is characterized by a gradual increase in both resistive and reactive pulmonary arterial (PA) impedance. Previous studies in a rodent model of PH have shown that reducing the hemodynamic load in the left lung (by banding the left PA) reverses this remodeling phenomenon. However, banding a single side of the pulmonary circulation is not a viable clinical option, so-using in silico modeling-we evaluated if the banding effect can be recreated by replacing the proximal vasculature with a compliant synthetic PA. We developed a computational model of the pulmonary circulation by combining a one-dimensional model of the proximal vasculature with a zero-dimensional line transmission model to the 12th generation. Using this model, we performed four simulations: (1) Control; (2) PH; (3) PH with a stenosis in the left PA; and (4) PH with proximal vessel compliance returned to Control levels. Simulations revealed that vascular changes associated with PH result in an increase in pulse pressure (PP), maximum pressure (Pmax), maximum wall shear stress (WSS), and maximum circumferential stress (σθθ) relative to controls, in the distal circulation. Banding the left PA reduced these measurements of hemodynamic stress in the left lung, but increases them in the right lung. Furthermore, left PA banding increased reactive PA impedance. However, returning the proximal PA compliance to Control levels simultaneously decreased all measures of hemodynamic stress in both lungs, and returned reactive PA impedance to normal levels. In conclusion, if future in vivo studies support the idea of hemodynamic unloading as an effective therapy for PH, this can be surgically achieved by replacing the proximal PA with a compliant prosthesis, and it will have the added benefit of reducing reactive right ventricular afterload.
Topics: Hemodynamics; Humans; Hypertension, Pulmonary; Pulmonary Artery; Pulmonary Circulation; Vascular Resistance
PubMed: 34251418
DOI: 10.1115/1.4051719 -
Current Opinion in Pulmonary Medicine Sep 2021Treatment options for Group 3 pulmonary hypertension, characterized as secondary to chronic hypoxia or lung disease, remain an elusive holy grail for physicians and... (Review)
Review
PURPOSE OF REVIEW
Treatment options for Group 3 pulmonary hypertension, characterized as secondary to chronic hypoxia or lung disease, remain an elusive holy grail for physicians and patients alike. Despite increasing identification and investigation into this pulmonary vasculopathy group with the second-highest frequency and highest mortality, there are no therapeutic interventions that offer the significant improvements in morbidity and mortality comparable to those benefiting other pulmonary hypertension groups including pulmonary arterial hypertension. This review examines the data on available and emerging Group 3 pulmonary hypertension treatments.
RECENT FINDINGS
Pulmonary vasodilators have yielded equivocal results in this patient population, although recent evidence shows modestly improved outcomes with inhaled treprostinil in interstitial lung disease-associated pulmonary hypertension. With pulmonary vasodilators providing limited benefit, emerging data support the right ventricle as a potential treatment target in Group 3 pulmonary hypertension.
SUMMARY
Group 3 pulmonary hypertension is associated with significant morbidity and mortality. Pulmonary vasodilators offer only limited haemodynamic and exertional benefits, and lung transplantation remains the only cure for this deadly disease. The right ventricle may provide a novel intervention target.
Topics: Humans; Hypertension, Pulmonary; Lung Transplantation; Pulmonary Artery; Pulmonary Circulation; Vasodilator Agents
PubMed: 34175858
DOI: 10.1097/MCP.0000000000000788 -
Cardiology in the Young Oct 2023Pulmonary arterial compliance, the dynamic component of pulmonary vasculature, remains inadequately studied in patients with left to right shunts. We sought to study the... (Review)
Review
INTRODUCTION
Pulmonary arterial compliance, the dynamic component of pulmonary vasculature, remains inadequately studied in patients with left to right shunts. We sought to study the pulmonary arterial compliance in patients with left to right shunt lesions and its utility in clinical decision-making.
MATERIALS AND METHODS
In this single-centre retrospective study, we reviewed cardiac catheterisation data of consecutive patients of left to right shunt lesions catheterised over one year. In addition to the various other parameters, pulmonary arterial compliance was calculated, as indexed pulmonary flow (Qpi) / (Heart rate × pulse pressure in the pulmonary artery). RC time was also calculated, as the product of pulmonary arterial compliance and pulmonary vascular resistance index. Patients were divided into "operable," "borderline," and "inoperable" based on the decision of the treating team, and the pulmonary arterial compliance values were evaluated in these groups to study if it can be utilised to refine the operability decision.
RESULTS
298 patients (Median age 16 years, 56% <18 years) with various acyanotic shunt lesions were included. Overall, the pulmonary arterial compliance varied with Qpi, pulmonary artery mean pressure, and pulmonary vascular resistance index, but did not vary with age, type of lesion, or transpulmonary gradients. The median pulmonary arterial compliance in patients with normal pulmonary artery pressure (Mean pulmonary artery pressure less than 20 mmHg) was 4.1 ml/mmHg/m (IQR 3.2). The median pulmonary arterial compliance for operable patients was 2.67 ml/mmHg/m (IQR 2.2). Median pulmonary arterial compliance was significantly lower in both inoperable (0.52 ml/mmHg/m, IQR 0.34) and borderline (0.80 ml/mmHg/m, IQR 0.36) groups when compared to operable patients (p < 0.001). A pulmonary arterial compliance value lower than 1.18 ml/mmHg/m identified inoperable patients with high sensitivity and specificity (95%, AUC 0.99). However, in borderline cases, assessment by this value did not agree with empirical clinical assessment.The median RC time for the entire study population was 0.47 S (IQR 0.30). RC time in operable patients was significantly lower than that in the inoperable patients (Median 0.40 IQR 0.23 in operable, 0.73 0.25 in inoperable patients (p < 0.001).
CONCLUSIONS
Addition of pulmonary arterial compliance to the routine haemodynamic assessment of patients with shunt lesions may improve our understanding of the pulmonary circulation and may have clinical utility.
Topics: Humans; Adolescent; Pulmonary Artery; Pulmonary Circulation; Hypertension, Pulmonary; Retrospective Studies; Vascular Resistance
PubMed: 36325920
DOI: 10.1017/S1047951122003341