scholarly journals The physiologic basis of pulmonary arterial hypertension

2021 ◽  
pp. 2102334
Author(s):  
Robert Naeije ◽  
Manuel J. Richter ◽  
Lewis J. Rubin
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pulmonary Circulation ◽  
Signs And Symptoms ◽  
Lung Mechanics ◽  
Pulmonary Vascular Disease ◽  
Right Heart ◽  
Pressure Flow ◽  
Physiologic Dead Space ◽  
Pulmonary Arterial

Pulmonary arterial hypertension (PAH) is a rare dyspnea-fatigue syndrome caused by a progressive increase in pulmonary vascular resistance (PVR) and eventual right ventricular (RV) failure. In spite of extensive pulmonary vascular remodeling, lung function in PAH is generally well preserved, with hyperventilation and increased physiologic dead space, but minimal changes in lung mechanics and only mild to moderate hypoxemia and hypocapnia. Hypoxemia is mainly caused by a low mixed venous PO2 from a decreased cardiac output. Hypocapnia is mainly caused by an increased chemosensitivity. Exercise limitation in PAH is cardiovascular rather than ventilatory or muscular. The extent of pulmonary vascular disease in PAH is defined by multipoint pulmonary vascular pressure-flow relationships with a correction for hematocrit. Pulsatile pulmonary vascular pressure-flow relationships in PAH allow for the assessment of RV hydraulic load. This analysis is possible either in the frequency-domain or in the time-domain. The RV in PAH adapts to increased afterload by an increased contractility to preserve its coupling to the pulmonary circulation. When this homeometric mechanism is exhausted, the RV dilates to preserve flow output by an additional heterometric mechanism. Right heart failure is then diagnosed by imaging of increased right heart dimensions and clinical systemic congestion signs and symptoms. The coupling of the RV to the pulmonary circulation is assessed by the ratio of end-systolic to arterial elastances, but these measurements are difficult. Simplified estimates of RV-PA coupling can be obtained by magnetic resonance or echocardiographic imaging of ejection fraction.

Potential role of exercise echocardiography and right heart catheterization in the detection of early pulmonary vascular disease in patients with systemic sclerosis

2019 ◽  
Vol 4 (3) ◽  
pp. 219-224
Author(s):  
Gabor Kovacs ◽  
Horst Olschewski
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Vascular Disease ◽  
Right Heart Catheterization ◽  
Pulmonary Vascular Disease ◽  
Heart Catheterization ◽  
Right Heart ◽  
Pulmonary Hemodynamics ◽  
Pulmonary Arterial

Pulmonary vascular disease represents one of the most frequent complications in systemic sclerosis leading to increased mortality. The recognition and appropriate clinical management of early pulmonary vascular disease could significantly improve the prognosis of affected patients. Early pulmonary vascular disease is characterized by the histological signs of pulmonary vascular remodeling, mildly increased mean pulmonary arterial pressure (21–24 mmHg) at rest, abnormal pulmonary hemodynamics during exercise, decreased exercise capacity, and a high risk for development of pulmonary arterial hypertension. Pulmonary hemodynamics can be investigated during exercise by echocardiography or by right heart catheterization both representing important clinical tools for the screening and confirmation of early pulmonary vascular disease. Further studies are needed to better understand the clinical course of systemic sclerosis patients with early pulmonary vascular disease and to define the characteristics of patients that will or will not profit from pulmonary arterial hypertension treatment.

scholarly journals Clinical application of pulmonary vascular resistance in patients with pulmonary arterial hypertension

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Jianying Deng
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pulmonary Vascular Resistance ◽  
Clinical Application ◽  
Vascular Resistance ◽  
Right Heart Failure ◽  
Pulmonary Vascular Disease ◽  
Right Heart ◽  
Pulmonary Arteriole ◽  
Pulmonary Arterial

AbstractPulmonary arterial hypertension is a type of malignant pulmonary vascular disease, which is mainly caused by the increase of pulmonary vascular resistance due to the pathological changes of the pulmonary arteriole itself, which eventually leads to right heart failure and death. As one of the diagnostic indicators of hemodynamics, pulmonary vascular resistance plays an irreplaceable role in the pathophysiology, diagnosis and treatment of pulmonary arterial hypertension. It provides more references for the evaluation of pulmonary arterial hypertension patients. This article summarizes the clinical application of pulmonary vascular resistance in patients with pulmonary arterial hypertension.

scholarly journals Heart and lung transplantation in pulmonary arterial hypertension related to congenital heart disease: an unusual indication

2020 ◽  
Vol 4 (S1) ◽  
Author(s):  
Rosaria Barracano ◽  
Heba Nashat ◽  
Andrew Constantine ◽  
Konstantinos Dimopoulos
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lung Transplantation ◽  
Atrial Septal Defect ◽  
Septal Defect ◽  
Pulmonary Vascular Disease ◽  
Eisenmenger Syndrome ◽  
Pulmonary Arterial ◽  
Recurrent Haemoptysis

Abstract Background Eisenmenger syndrome is a multisystem disorder, characterised by a significant cardiac defect, severe pulmonary hypertension and long-standing cyanosis. Despite the availability of pulmonary hypertension therapies and improved supportive care in specialist centres, Eisenmenger patients are still faced with significant morbidity and mortality. Case presentation We describe the case of a 44-year-old woman with Eisenmenger syndrome secondary to a large secundum atrial septal defect. Her pulmonary vascular disease was treated with pulmonary vasodilators, but she experienced a progressive decline in exercise tolerance, increasing atrial arrhythmias, resulting in referral for transplantation. Her condition was complicated by significant recurrent haemoptysis in the context of extremely dilated pulmonary arteries and in-situ thrombosis, which prompted successful heart and lung transplantation. She made a slow recovery but remains well 3 years post-transplant. Conclusions Patients with Eisenmenger syndrome secondary to a pre-tricuspid lesion, such as an atrial septal defect have a natural history that differs to patients with post-tricuspid shunts; the disease tends to present later in life but is more aggressive, prompting early and aggressive medical intervention with pulmonary arterial hypertension therapies. This case illustrates that severe recurrent haemoptysis can be an indication for expediting transplantation in Eisenmenger syndrome patients.

scholarly journals Critical Care Management of Decompensated Right Heart Failure in Pulmonary Arterial Hypertension Patients – An Ongoing Approach

2021 ◽  
Vol 7 (3) ◽  
pp. 170-183
Author(s):  
Ioan Tilea ◽  
Andreea Varga ◽  
Anca-Meda Georgescu ◽  
Bianca-Liana Grigorescu
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Intensive Care ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Care Management ◽  
Right Heart Failure ◽  
Right Heart ◽  
Continuous Increase ◽  
Pulmonary Arterial

Abstract Despite substantial advancements in diagnosis and specific medical therapy in pulmonary arterial hypertension patients’ management, this condition continues to represent a major cause of mortality worldwide. In pulmonary arterial hypertension, the continuous increase of pulmonary vascular resistance and rapid development of right heart failure determine a poor prognosis. Against targeted therapy, patients inexorable deteriorate over time. Pulmonary arterial hypertension patients with acute right heart failure who need intensive care unit admission present a complexity of the disease pathophysiology. Intensive care management challenges are multifaceted. Awareness of algorithms of right-sided heart failure monitoring in intensive care units, targeted pulmonary hypertension therapies, and recognition of precipitating factors, hemodynamic instability and progressive multisystem organ failure requires a multidisciplinary pulmonary hypertension team. This paper summarizes the management strategies of acute right-sided heart failure in pulmonary arterial hypertension adult cases based on recently available data.

scholarly journals Echocardiographic Prognosis Relevance of Attenuated Right Heart Remodeling in Idiopathic Pulmonary Arterial Hypertension

2021 ◽  
Vol 8 ◽  
Author(s):  
Qin-Hua Zhao ◽  
Su-Gang Gong ◽  
Rong Jiang ◽  
Chao Li ◽  
Ge-Fei Chen ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Survival Rates ◽  
Left Ventricular ◽  
Functional Improvement ◽  
Right Heart ◽  
Long Term Follow Up ◽  
Pulmonary Arterial ◽  
Rv Function

Background: Right ventricular (RV) function is a great determination of the fate in patients with pulmonary arterial hypertension (PAH). Monitoring RV structure back to normal or improvement should be useful for evaluation of RV function. The aims of this study were to assess the prognostic relevance of changed right heart (RH) dimensions by echocardiography and attenuated RH remodeling (ARHR) in idiopathic PAH (IPAH).Methods: We retrospectively analyzed 232 consecutive adult IPAH patients at baseline assessment and included RH catheterization and echocardiography. ARHR at the mean 20 ± 12 months' follow-up was defined by a decreased right atrium area, RV mid-diameter, and left ventricular end-diastolic eccentricity index. The follow-up end point was all-cause mortality.Results: At mean 20 ± 12 months' follow-up, 33 of 232 patients (14.2%) presented with ARHR. The remaining 199 surviving patients were monitored for another 25 ± 20 months. At the end of follow-up, the survival rates at 1, 3, and 5 years were 89, 89, and 68% in patients with ARHR, respectively, and 84, 65 and 41% in patients without ARHR (log-rank p = 0.01). ARHR was an independent prognostic factor for mortality. Besides, ARHR was available to further stratify patients' risk assessment through the French PAH non-invasive-risk criteria.Conclusions: Echocardiographic ARHR is an independent determinant of prognosis in IPAH at long-term follow-up. ARHR might be a useful tool to indicate the RV morphologic and functional improvement associated with better prognostic likelihood.

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