Portopulmonary hypertension (PoPH) is a form of pulmonary arterial hypertension (PAH) that can develop as a complication of portal hypertension. Treatment of PoPH includes PAH‐specific therapies, and ...in certain cases, such therapies are necessary to facilitate a successful liver transplantation. A significant number of barriers may limit the adequate treatment of patients with PoPH and explain the poorer survival of these patients when compared to patients with other types of PAH. Until recently, only one randomized controlled trial has included PoPH patients, and the majority of treatment data have been derived from relatively small observational studies. In the present article, we review some of the barriers in the treatment of patients with PoPH and implications for liver transplantation.
Glucocorticoids (GC) are considered first-line therapy for treating sarcoidosis, but there are few data about the adverse consequences of GC. Although there are several steroid-sparing medications ...available for treatment, a large proportion of patients are treated with prolonged courses of GC. The toxicities of GC in sarcoidosis populations have not been carefully evaluated.
We performed a retrospective cohort study of all newly diagnosed sarcoidosis patients who had the entirety of their medical care in a single health system. We analyzed the time to development of a composite toxicity end-point, including diabetes, hypertension, weight gain, hyperlipidemia, low bone density and ocular complications of GC using Cox proportional hazards analysis.
One hundred and five patients were ever treated with GC, whereas 49 were not treated during a median follow-up of 101 months. GC-treated patients developed 1.3 ± 1.1 toxicities during therapy, versus 0.6 ± 1.0 in the non-treated group. After adjustment for age, gender, race and preexisting conditions, the hazard ratio for ever-treated patients was 2.37 (1.34–4.17) for the composite end-point. Age and the presence of preexisting conditions also were associated with reaching the end-point. Similar effects were seen when analyzed for cumulative GC dose and for duration of GC use. For individual end-points, weight gain (HR 2.04) and new hypertension (HR 3.36) were associated with any use of GC.
Our data suggest that GC are associated with clinically important toxicities in sarcoidosis patients, associated with both the cumulative dose and duration of treatment.
•Standard therapy of sarcoidosis includes steroids, often in prolonged courses.•Long-term steroids for sarcoidosis are associated with significant comorbidity.•Steroid therapy in sarcoidosis needs to be re-evaluated and minimized when possible.
Accurate and regular risk assessment is important for evaluation and treatment of pulmonary arterial hypertension (PAH) patients, including those with functional class (FC) II symptoms, a population ...considered at low risk for disease progression. Risk assessment methods include subjective and objective evaluations. Multiparametric assessments include tools based on the European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines (COMPERA and FPHR methods, respectively) and the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL; REVEAL 2.0 tool). To better understand risk status determination in FC II patients, we compared physician-reported risk assessments with objective multiparameter assessment tools.
This retrospective chart analysis included PAH patients with FC II symptoms receiving monotherapy or dual therapy. Physicians were surveyed (via telephone) to obtain an assessment of patient risk using their typical methodology, which might have been informed by objective risk assessment. Patient risk was then calculated independently using COMPERA, FPHR and REVEAL 2.0 tools. Factors associated with incongruent risk assessment were identified.
Of the 153 patients, 41%, 46%, and 13% were classified as low, intermediate, and high risk, respectively, by physicians. Concordance between physician gestalt and objective methods ranged from 43%-54%. Among patients considered as low risk by physician gestalt, 4%-28% were categorized as high risk using objective methods. The most common physician factor associated with incongruent risk assessment was less frequent echocardiography during follow-up (every 7-12 months vs. every 3 months; p = 0.01).
More than half of FC II PAH patients were classified as intermediate/high risk using objective multiparameter assessments. Incorporating objective risk-assessment algorithms into clinical practice may better inform risk assessment and treatment strategies.
The causes and circumstances surrounding death are understudied in patients with pulmonary arterial hypertension (PAH).
We sought to determine the specific reasons and characteristics surrounding the ...death of patients with PAH.
All deaths of patients with pulmonary hypertension (PH) followed in the Cleveland Clinic Pulmonary Vascular Program were prospectively reviewed by the PH team. A total of 84 patients with PAH (age 58 ± 14 yr; 73% females) who died between June 2008 and May 2012 were included.
PH was determined to be the direct cause of death (right heart failure or sudden death) in 37 (44%) patients; PH contributed to but did not directly cause death in 37 (44%) patients; and the death was not related to PH in the remaining cases (n = 7; 8.3%). In three (3.6%) patients the final cause of death could not be adequately assessed. Most patients died in a healthcare environment and most received PH-specific therapies. In our cohort, 50% of all patients with PAH and 75.7% of those who died of right heart failure received parenteral prostanoid therapy. Less than half of patients had advanced healthcare directives.
Most patients with PAH in our cohort died of their disease; however, right ventricular failure or sudden death was the sole cause of death in less than half of patients.
Pulmonary hypertension (PH) is usually associated with a normal or decreased cardiac output (CO). Less commonly, PH can occur in the context of a hyperdynamic circulation, characterized by high CO ...(>8 L/min) and/or cardiac index ≥4 L/min/m2 in the setting of a decreased systemic vascular resistance. PH due to high CO can occur due to multiple conditions and in general remains understudied. In this review article we describe the pathophysiology, etiology, diagnosis, hemodynamic characteristics, and management of PH in the setting of high CO. It is important to recognize this distinct entity as PH tends to improve with treatment of the underlying etiology and PH specific therapies may worsen the hemodynamic state.
•High cardiac output is an uncommon but important cause of PH.•Increased flow through pulmonary circuit increases sheer stress and increases mPAP.•Important causes: high-output heart failure, cirrhosis, fistulas, obesity, anemia.•Treatment of underlying condition may lead to resolution of PH.
Microvascular changes play central roles in the pathophysiology of SSc and SLE, and represent major causes of morbidity and mortality in these patients. Therefore, clinical tools that can assess the ...microvasculature are of great importance both at the time of diagnosis and follow‐up of these cases. These tools include capillaroscopy, laser imaging techniques, infrared thermography, and iontophoresis. In this review, we examined the clinical manifestations and pathobiology of microvascular involvement in SSc and SLE as well as the methodologies used to evaluate the microvasculature.
To summarize knowledge and identify gaps in evidence regarding treatment of right ventricular dysfunction (RVD) in acute respiratory distress syndrome (ARDS).
We conducted a comprehensive search of ...MEDLINE, Embase, CINAHL, Web of Science, and the Cochrane Central Register of Controlled Trials.
Studies were included if they reported effects of treatments on right ventricular function, whether or not the intent was to modify right ventricular function.
Data extraction was performed independently and in duplicate by two authors. Data items included the study design, patient population, type of intervention, comparison group, and RV-specific outcomes.
Of 1,430 studies screened, 51 studies reporting on 1,526 patients were included. By frequency, the included studies examined the following interventions: ventilator settings (29.4%), inhaled medications (33.3%), extracorporeal life support (13.7%), intravenous or oral medications (13.7%), and prone positioning (9.8%). The majority of the studies were non-randomized experimental studies (53%), with the next most common being case reports (16%). Only 5.9% of studies were RCTs. In total, 27% of studies were conducted with the goal of modifying RV function.
Given the prevalence of RVD in ARDS and its association with mortality, the dearth of research on this topic is concerning. This review highlights the need for prospective trials aimed at treating RV dysfunction in ARDS.
Pulmonary arterial hypertension (PAH) is a progressive disease, which can be potentially fatal. The management of a complex disease like PAH requires a multidisciplinary approach from a team ...consisting of physicians, nurses, social workers, and pharmacists. Adherence to PAH-specific therapy is one of the key factors in the management of this disease. Poor adherence to treatment is a common problem in PAH as it is in many chronic diseases. Management of medication interruptions is a challenge in patients with PAH that can lead to negative consequences. However, for most PAH-specific drugs, there are no clear guidelines on how to manage temporary or abrupt medication discontinuations. In this review, we summarized the available literature and provide suggestions on how to manage interruptions of PAH-specific therapies.
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