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Introduction: Diagnosis and Management of Pulmonary Arterial Hypertension: ACCP Evidence-Based Clinical Practice Guidelines
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Cited by (207)
Pulmonary hypertension: Key aspects in preoperative management
2023, Perioperative Care and Operating Room ManagementPulmonary hypertension, characterized by increased pressure in the pulmonary vasculature, can stem from various causes. This condition strains the thin-walled right ventricle of the heart, leading to potential dilation and reduced function over time. Anesthesiologists encounter challenges when managing PH patients during surgery, as the right ventricle poorly responds to inotropic support and undergoes additional stress from surgical conditions. Pulmonary hypertension requires multidisciplinary management in the perioperative setting to ensure appropriate and safe care of patients.
Ensuring safe anesthesia for patients with pulmonary hypertension entails careful planning, including a thorough history and physical examination, preoperative testing, optimization of the disease and comorbidities, and comprehensive risk assessment. It is crucial to identify signs and symptoms of progressing pulmonary hypertension and right heart dysfunction. Symptomatic patients with undiagnosed pulmonary hypertension will benefit from referrals to specialists for further evaluation and testing.
Diagnostic tools such as electrocardiograms, echocardiography, and right heart catheterization offer valuable information for anesthetic planning and risk assessment. They provide insight into the severity of pulmonary hypertension. Right heart catheterization allows direct measurement of hemodynamic parameters, guiding treatment decisions. Hemodynamic classification distinguishes between pre-capillary, post-capillary, and combined pre- and post-capillary pulmonary hypertension. Another categorization method is clinical grouping based on underlying pathological characteristics, which helps tailor management and treatment choices.
This article underscores the significance of perioperative management and the need for a comprehensive evaluation and multidisciplinary approach in patients with pulmonary hypertension. It provides valuable information for anesthesiologists and other healthcare professionals involved in the care of these patients, with a focus on preoperative assessment, risk stratification, and treatment considerations.
Various factors contribute to death in patients with different types of pulmonary hypertension: A retrospective pilot study from a single tertiary center
2022, Respiratory InvestigationA few studies have focused on the cause of death from different types of pulmonary hypertension (PH). This study aimed to systematically analyze the primary and secondary causes of death and compare the profiles between different PH groups.
The contribution of PH to death was assessed in precapillary PH (i.e., group 1 [pulmonary arterial hypertension], group 3 [PH associated with lung disease], and group 4 [chronic thromboembolic PH]) using specific criteria; death was classified into three categories: PH death (death due to PH only), PH-related death, and PH-unrelated death. Disorders other than PH that contributed to death were analyzed, and mortality profiles were compared between groups.
Eighty deceased patients with PH were examined (group 1, n = 28; group 3, n = 39; and group 4, n = 13). The contribution of PH to death was significantly different between the three groups. “PH death” was most common in group 1 (61%), “PH-related death” in group 3 (56%), and “PH-related death” and “PH-unrelated death” in group 4 (38% for both). The highest contributing factor to death other than PH was respiratory failure in group 3 and malignant disease in group 4.
Significant variations in the causes of death were observed in groups 1, 3, and 4 PH patients. In addition to PH, respiratory failure and malignant disease significantly contributed to death in group 3 and group 4 PH, respectively. Understanding the precise death cause may be important in achieving better outcomes in PH patients.
Detection of Pulmonary Hypertension by Combining Echocardiography and Chest Radiography
2022, Academic RadiologyIn pulmonary hypertension (PH) patients, chest radiographs often show an increase in the diameter of the right descending pulmonary artery (RDPA).
The purpose of this study is to evaluate whether a combination of echocardiography and chest radiography for detecting PH is more accurate than echocardiography alone.
Between 2013 and 2019, a total of 1301 patients were included in this study. Among them, 1030 patients with congenital heart disease (CHD) were used to establish a linear regression model by combining echocardiographic and chest radiographic variables, and 136 CHD patients and 135 non-CHD patients were used to compare the accuracy between a new model and the 2015 ESC/ERS guidelines for right heart catheterization recommendation. The chest radiographic diameter of the RDPA, and the echocardiography-measured tricuspid regurgitation pressure gradient and the main pulmonary artery diameter were assessed.
The TG-RDPA composite index correlated more strongly than either the TG or RDPA (r = 0.741 vs 0.709 or 0.544; both p value <0.001). The TG-RDPA composite index was more accurate in detecting PH than the ESC/ERS 2015 guidelines (overall accuracy: 83.8% vs 77.1%; missed diagnoses rate: 12.0% vs 22.5%). The overall accuracy of the main pulmonary artery-RDPA composite index (r = 0.599, p value <0.001) was 84.1% compared to overall accuracy of 77.1% using the ESC/ERS 2015 guidelines.
A combination of echocardiography and chest X-ray may be a more accurate way to detect PH and an alternative method for suspected PH patients without tricuspid regurgitation velocity.
Does Duke Activity Status Index help predicting functional exercise capacity and long-term prognosis in patients with pulmonary hypertension?
2021, Respiratory MedicineTo investigate the association of Duke Activity Status Index (DASI) with 6‐minute walk test (6MWT) and WHO-Functional Class (WHO-FC) in patients with pulmonary hypertension (PH), as well as exploring whether DASI can discriminate between the patients with better and worse long-term prognosis according to 400 m cut-off score in 6MWT.
Eighty-five medically stable PH patients who met eligibility criteria were included. All patients were evaluated using 6MWT and DASI. The prognostic utility of the DASI was assessed using univariate linear regression and receiver operating characteristic (ROC) curve analysis.
The DASI was an independent predictor for both 6MWT and WHO-FC, explaining 50% of variance in 6MWT and 30% of variance in WHO-FC class (p < 0.001). In addition, DASI significantly correlated to 6MWT (r = 0.702) and WHO-FC class (r = 0.547). The ROC curve analysis revealed that the DASI had a discriminative value for identifying the patients with better long-term prognosis (p < 0.001), with an area under ROC curve of 0.867 [95% CI = 0.782–0.952]. The DASI ≥26 was the optimal cut-off value for better long-term prognosis, having sensitivity of 0.74 and a specificity of 0.88.
The DASI is a valid tool reflecting functional exercise capacity in patients with PH. Considering its ability to discriminate between the patients with better or worse long-term prognosis, it may help identifying the patients at higher risk.
Pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary arterial pressures and is managed by vasodilator therapies. Current guidelines encourage PAH management in specialty care centers (SCCs), but evidence is sparse regarding improvement in clinical outcomes and correlation to vasodilator use with referral.
Is PAH management at SCCs associated with improved clinical outcomes?
A single-center, retrospective study was performed at the University of Pittsburgh Medical Center (UPMC; overseeing 40 hospitals). Patients with PAH were identified between 2008 and 2018 and classified into an SCC or non-SCC cohort. Cox proportional hazard modeling was done to compare for all-cause mortality, as was negative binomial regression modeling for hospitalizations. Vasodilator therapy was included to adjust outcomes.
Of 580 patients with PAH at UPMC, 455 (78%) were treated at the SCC, comprising a younger (58.8 vs 64.8 years; P < .001) and more often female (68.4% vs 51.2%; P < .001) population with more comorbidities without differences in race or income. SCC patients demonstrated improved survival (hazard ratio, 0.68; P = .012) and fewer hospitalizations (incidence ratio, 0.54; P < .001), and provided more frequent disease monitoring. Early patient referral to SCC (< 6 months from time of diagnosis) was associated with improved outcomes compared with non-SCC patients. SCC patients were more frequently prescribed vasodilators (P < .001) and carried more diagnostic PAH coding (P < .001). Vasodilators were associated with improved outcomes irrespective of location but without statistical significance when comparing between locations (P > .05).
The UPMC SCC demonstrated improved outcomes in mortality and hospitalizations. The SCC benefit was multifactorial, with more frequent vasodilator therapy and disease monitoring. These findings provide robust evidence for early and regular referral of patients with PAH to SCCs.
Pulmonary hypertension in end-stage renal disease
2020, Respiratory MedicinePulmonary hypertension associated with end-stage renal disease (ESRD) is an important yet under-recognized condition and can lead to life-threatening complications. The pathogenesis of pulmonary hypertension is peculiar in ESRD, and understanding it is important to recognize such patients at the earliest and commence appropriate treatment. Many studies have discovered the prevalence of pulmonary hypertension to be up to 80% in ESRD and have been associated with increased mortality. WHO has classified pulmonary hypertension in renal failure to be in group 5, a group defined by unclear multifactorial etiologies. Moreover, there is an improvement with renal transplant and closure of AV fistula, thus confirming the contribution from these. The pharmacological management of pulmonary hypertension in this unique population is not very different from other etiologies. However, one should understand that pulmonary hypertension as such, could be multifactorial, and other secondary causes of pulmonary hypertension should also be recognized and treated accordingly. In this article, we will discuss the concept of pulmonary hypertension in ESRD in detail and the options of treatment.
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