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Ambrisentan has shown effectiveness in the treatment of Group 1 pulmonary arterial hypertension (PAH). Although portopulmonary hypertension (PoPH) is a subset of Group 1 PAH, few clinical trials have been testing PAH therapies in patients with PoPH. The objective of this study is to evaluate the efficacy and safety of ambrisentan in PoPH. This study is a prospective, multicenter, open-label trial in which treatment-naive patients with PoPH with Child-Pugh class A/B were administered with ambrisentan for 24 weeks, followed by a long-term extension (24–28 weeks). The primary end-points were change in pulmonary vascular resistance (PVR) and 6-minutes walk distance (6MWD) at 24 weeks, whereas secondary end-points included safety, World Health Organization (WHO) functional class (FC) and echocardiographic assessments. Of the 31 patients, 23 finished 24 weeks of ambrisentan therapy and 19 finished the extension. PVR decreased significantly (mean ± SD) (7.1 ± 5 vs 3.8 ± 1.8 Wood units, p < 0.001), whereas 6MWD remained unchanged (314 ± 94 vs 336 ± 108 m). Other hemodynamic parameters such as right atrial pressure (13 ± 8 vs 9 ± 4 mm Hg, p < 0.05), mean pulmonary arterial pressure (46 ± 13 vs. 38 ± 8 mm Hg, p < 0.01), cardiac index (2.6 ± 0.6 vs. 3.5 ± 0.7 liter/min/m2, p < 0.001) showed improvement, whereas pulmonary capillary wedge pressure remained unchanged. Of the 22 patients with WHO FC assessments at baseline and 24 weeks, WHO FC improved significantly (p = 0.005). Most frequent drug-related adverse events were edema (38.7%) and headache (22.5%). One episode of leg edema resulted into the permanent discontinuation of ambrisentan. Ambrisentan monotherapy in PoPH improves hemodynamics and FC at 24 weeks; however, it did not show any improvement in 6MWD. These preliminary outcomes should be interpreted with caution (Clinicaltrials.Gov:NCT01224210). [ABSTRACT FROM AUTHOR]

The article discusses a research study which characterized first-time hospitalizations in patients diagnosed with pulmonary arterial hypertension (PAH) in the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL Registry). Data provided shows demographics of patients and their disease characteristics. It also highlights the correlation of hospitalization outcome with subsequent hospitalization and survival of patients.

OBJECTIVE: To investigate the correlation between body mass index (BMI) and pulmonary artery systolic pressure in a large population of patients with pulmonary arterial hypertension (PAH). PATIENTS AND METHODS: The BMI of patients with group 1 PAH enrolled In the Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL) was compared with that of age- and sex-matched controls In the National Health and Nutrition Examination Survey (NHANES) to clarify whether obesity is linked with PAH. The diagnosis of PAH was defined in REVEAL by right-sided heart catheterization. Differences in BMI and the percentage of patients considered obese (BMI ≥30) and underweight (BMI <18.5) in various subgroups of patients enrolled in REVEAL from March 30, 2006, through September 11, 2007, were determined. RESULTS: Mean BMI was no different for patients with PAH (n=2141) than for the NHANES normal comparison group; however, the proportion of obese and underweight patients was increased in patients with PAH. Subgroup analysis demonstrated that subgroups with idiopathic PAH and those with PAH associated with drugs and toxins had both higher BMI and percentage of obese patients, whereas 3 other subgroups (those with PAH associated with congenital heart disease, connective tissue disease, and human immunodeficiency virus) had lower mean BMI. CONCLUSION: Mean BMI of the REVEAL patients was the same as that of the NHANES normal comparison group; however, there were higher percentages of obese and underweight patients in REVEAL. This discrepancy can be explained by the balancing effect of more overweight and underweight patients in different PAH subgroups. The reason for the increased frequency of obesity in idiopathic PAH Is unknown, and additional study is needed. [ABSTRACT FROM AUTHOR]

The possibility that pulmonary hypertension (PH) may develop in patients with chronic obstructive pulmonary disease (COPD) is well established, but prevalence data vary. The current World Health Organization clinical classification includes COPD in diagnostic group III: PH associated with disorders of the respiratory system or hypoxemia. The National Institute of Health defines PH as a mean pulmonary artery pressure of greater than 25 mmHg. Approximately 10% of the patients seen over the last decade in the PH Clinic at Mayo Clinic in Jacksonville, Florida, have PH due to COPD. The pathophysiology is likely complex and involves hypoxic pulmonary vasoconstriction. Ultimately, chronic hypoxia results in vascular remodeling with narrowing of the vascular lumen. The right heart is forced to generate increased driving pressures to overcome the increased vascular resistance. As the disease progresses, cor pulmonale may develop. The mortality in this setting is increased with five-year survival of 20% to 36% and seems to correlate with worsening PH and age. Fortunately, the PH in most cases is mild and occurs primarily in those with severe hypoxemia. Only 1% to 4% of patients have PH seemingly out of proportion to the severity of the COPD. This disproportionate subgroup may represent an important phenotype that requires a different therapeutic approach. Although supplemental oxygen remains the primary treatment for all PH in association with chronic hypoxia, pulmonary arterial vasodilators may have a therapeutic role in this subgroup. Vasodilators may worsen gas exchange, however, and to date, have no proven benefit. Rigorous future study will be required to determine whether there is a role for using pulmonary arterial vasodilators in this setting. [ABSTRACT FROM AUTHOR]

Advanced Care at Home is a Mayo Clinic hospital-at-home (HaH) program that provides hospital-level care for patients. The study examines patient- and community-level factors that influence health outcomes. The authors performed a retrospective study using patient data from July 2020 to December 2022. The study includes 3 Mayo Clinic centers and community-level data from the Agency for Healthcare Research and Quality. The authors conducted binary logistic regression analyses to examine the relationship among the independent variables (patient- and community-level characteristics) and dependent variables (30-day readmission, mortality, and escalation of care back to the brick-and-mortar hospital). The study examined 1433 patients; 53% were men, 90.58% were White, and 68.2% were married. The mortality rate was 2.8%, 30-day readmission was 11.4%, and escalation back to brick-and-mortar hospitals was 8.7%. At the patient level, older age and male gender were significant predictors of 30-day mortality (P-value <0.05), older age was a significant predictor of 30-day readmission (P-value <0.05), and severity of illness was a significant predictor for readmission, mortality, and escalation back to the brick-and-mortar hospital (P-value <0.01). Patients with COVID-19 were less likely to experience readmission, mortality, or escalations (P-value <0.05). At the community level, the Gini Index and internet access were significant predictors of mortality (P-value <0.05). Race and ethnicity did not significantly predict adverse outcomes (P-value >0.05). This study showed promise in equitable treatment of diverse patient populations. The authors discuss and address health equity issues to approximate the vision of inclusive HaH delivery. [ABSTRACT FROM AUTHOR]

Inhaled treprostinil is an approved therapy for pulmonary arterial hypertension (PAH) and pulmonary hypertension associated with interstitial lung disease in the United States. Studies have confirmed the robust benefits and safety of nebulized inhaled treprostinil, but it requires a time investment for nebulizer preparation, maintenance, and treatment. A small, portable treprostinil dry powder inhaler has been developed for the treatment of PAH. The primary objective of this study was to evaluate the safety and tolerability of treprostinil inhalation powder (TreT) in patients currently treated with treprostinil inhalation solution. Fifty‐one patients on a stable dose of treprostinil inhalation solution enrolled and transitioned to TreT at a corresponding dose. Six‐minute walk distance (6MWD), device preference and satisfaction (Preference Questionnaire for Inhaled Treprostinil Devices [PQITD]), PAH Symptoms and Impact (PAH‐SYMPACT®) questionnaire, and systemic exposure and pharmacokinetics for up to 5 h were assessed at baseline for treprostinil inhalation solution and at Week 3 for TreT. Adverse events (AEs) were consistent with studies of inhaled treprostinil in patients with PAH, and there were no study drug‐related serious AEs. Statistically significant improvements occurred in 6MWD, PQ‐ITD, and PAH‐SYMPACT. Forty‐nine patients completed the 3‐week treatment phase and all elected to participate in an optional extension phase. These results demonstrate that, in patients with PAH, transition from treprostinil inhalation solution to TreT is safe, well‐tolerated, and accompanied by statistically significant improvements in key clinical assessments and patient‐reported outcomes with comparable systemic exposure between the two formulations at evaluated doses (trial registration: clinicaltrials.gov identifier: NCT03950739). [ABSTRACT FROM AUTHOR]

Pulmonary hypertension (PH) associated with chronic kidney disease (CKD) (PH‐CKD) affects approximately 20%–40% of CKD patients and is associated with increased morbidity and mortality. PH and CKD are both pathophysiologically associated with nitric oxide (NO) deficiency. The NO pathway, an important therapeutic domain in pulmonary arterial hypertension (PAH), is an intriguing but unexplored target in PH‐CKD. We sought to improve understanding of the clinical significance of the NO pathway in patients with PH‐CKD by assessing the hemodynamic response to inhaled NO (iNO) during right heart catheterization (RHC). In this retrospective cohort study, patients with diagnosis codes of PH and stage IV/V CKD or end‐stage renal disease and estimated glomerular filtration rate < 60 mL/min/body surface area who underwent RHC and hemodynamic drug study between July 2011 and June 2021 were eligible. Patients with mean pulmonary artery pressure (mPAP) > 20 mmHg and pulmonary vascular resistance (PVR) > 3 Wood units were included. The final cohort included 37 patients (45.9% female, mean age 72.5 ± 9.7 years). A total of 56.7% of the cohort (21/37) had precapillary PH, while 43.2% (16/37) had combined precapillary postcapillary PH (Cpc‐PH). Median survival was 3.1 years after RHC. iNO was associated with a significant decrease in both mPAP and PVR. Hemodynamic changes in mPAP and PVR were similar in precapillary and Cpc‐PH groups. Among a small subset (n = 14) who were subsequently treated with PAH‐targeted therapy, treatment response was mixed and did not reveal significant benefit. Further studies are warranted to better define the potential role of PAH therapy in PH‐CKD. [ABSTRACT FROM AUTHOR]

Lymphangioleiomyomatosis (LAM) is a rare disease associated with cystic lung destruction and abdominal tumors, including lymphangioleiomyomas, which frequently occur in the retroperitoneal region. Sirolimus therapy is currently recommended for LAM patients with abnormal or declining lung function with an adjusted dose to maintain a serum trough level of 5–15 ng/mL. We describe a significant reduction of retroperitoneal lymphangioleiomyomas after treatment with low‑dose sirolimus therapy (serum trough level <5 ng/mL) in a patient with sporadic LAM. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to an article about ventilator bundle approach to the prevention of ventilator-associated pneumonia.

Background and Aims To determine the prevalence of metabolic syndrome (MetS) - a potential phenotypical risk for pulmonary hypertension (PH) - in patients with PH and by PH diagnostic group including group 1 pulmonary arterial hypertension (PAH); group 1.1 idiopathic PAH, groups 1.2 heritable PAH, 1.3 drug and toxin induced, 1.4 associated PAH, group 2 PH with left heart disease, group 3 PH associated with lung disease and/or hypoxemia, group 4 PH owing to chronic thrombotic and/or embolic disease, and group 5 PH from multifactorial mechanisms. Methods We conducted a retrospective chart review of patients referred to Mayo Clinic in Florida from January 1990 to October 2013 ( N = 1287). Only patients whom PH was confirmed by right heart catheterization were included ( N = 640). MetS was defined with at least three of the following: systemic hypertension, diabetes mellitus, hyperlipidemia and body mass index > 30. Results MetS was prevalent in 247 of 640 (39%) with PH: 13% in group 1.1 PAH, 12% in groups 1.2-1.4 PAH, 54% in group 2, 55% in group 3, 23% in group 4 and 24% in group 5. After adjusting for age, sex and race, diagnostic subgroup analysis revealed a higher prevalence of MetS in WHO Group 2 PH patients [adjusted odd ratios (OR): 2.58, P < 0.0001] and WHO group 3 PH (adjusted OR: 2.77, P < 0.0001) when compared to group 1 PH. Conclusion The prevalence of MetS was observed in 39% of patients with PH; however, among PH patients, the prevalence was higher in group 2 and 3 PH. [ABSTRACT FROM AUTHOR]

The authors comment on the challenges faced by patients with pulmonary hypertension (PH).

Objectives: Pulmonary hypertension (PH) is commonly observed in patients with diffuse parenchymal lung disease (DPLD). The purpose of this study was to explore the influence of the 6-minute walk test (6M WT) as a simple, non-invasive tool to assess right ventricular (RV) function in patients with DPLD and to identify the need for an echocardiogram (ECHO) to screen for PH. Methods: We retrospectively reviewed 48 patients with PH secondary to DPLD, who were evaluated in the PH clinic at the Mayo Clinic in Jacksonville, Florida, from January 1999 to December 2014. Results: Fifty-two percent of patients had RV dysfunction. They had a significantly greater right heart pressure by ECHO and mean pulmonary arterial pressure (MPAP) from right heart catheterization (RHC) than those with normal RV function. A reduced 6-minute walk distance (6MWD) did not predict RV dysfunction (OR0.995; 95% CI 0.980-1.001,p = 0.138). In addition, worsening restrictive physiology, heart rate at one-minute recovery and desaturation were not different between patients with and without RV dysfunction. However, there were inverse correlations between 6MWD and MPAP from RHC (r = -0.41, p = 0.010), 6MWD and RV systolic pressure (r = -0.51, p < 0.001), and 6MWD and MPAP measured by ECHO (r = -0.46, p =0.013). We also found no significant correlation between 6MWD and pulmonary function test parameters. Conclusions: Our single-center cohort of patients with PH secondary to DPLD, PH was found to have an impact on 6MWD. In contrast to our expectations, 6MWD was not useful to predict RV dysfunction. Interestingly, a severe reduction in the 6MWD was related to PH and not to pulmonary function; therefore, it may be used to justify an ECHO to identify patients with a worse prognosis. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the article, "Lymphangioleiomyomatosis: a clinical update," by F.X. McCormack, in a 2008 issue.

Introduction Submaximal exercise testing ( SET) assesses functional exercise capacity in pulmonary arterial hypertension ( PAH) patients and provides additional physiologic information compared with the 6-min walk test (6 MWT). The relative correlations of the 6 MWT and SET using SHAPE-HF™ with other markers of PAH severity, particularly echocardiogram ( ECHO)-derived mean pulmonary artery pressure ( MPAP), have not been investigated. Objectives (i) Examine the correlation between SHAPE measures with the 6-min walk distance (6 MWD), and (ii) Compare SHAPE parameters and the 6 MWD to clinical measures of PAH severity. Methods Fifty-five consecutive group 1 PAH outpatients were evaluated in a single pulmonary hypertension referral center from March 2011 to June 2012. Data collected: World Health Organization ( WHO) functional class ( FC), brain natriuretic peptide ( BNP), ECHO, 6 MWD and SHAPE results. Data are reported using mean ± standard deviation and Spearman correlation coefficients (r). Results Nine patients were excluded. Of the 46 remaining patients, 78% were women and the average age was 61 ± 13 years. PAH characteristics: WHO FC III-IV 48%; idiopathic PAH 52%; BNP 198 ± 277 pg/mL; 6 MWD 399 ± 97 m; ECHO right atrial pressure ( RAP) 7 ± 4, pulmonary artery systolic pressure ( PASP) 63 ± 29 and MPAP 45 ± 17 mmHg. SET with SHAPE results: mean partial pressure of end-tidal ( PET) CO2 31 ± 6 mmHg at end-exercise; ventilatory efficiency ( VE/ VCO2) 43 ± 16. Significant correlations were found between the 6 MWD and SHAPE variables: VE/ VCO2 (r = -0.57, P < 0.0001) and end-exercise PETCO2 (r = 0.42, P = 0.004). VE/ VCO2 correlated with WHO FC, BNP, RAP, MPAP and PASP and 6 MWD only with WHO FC, BNP and PASP. Conclusion SHAPE outperformed 6 MWD in comparison with other measures of PAH disease severity. [ABSTRACT FROM AUTHOR]

intermediate‐risk pulmonary arterial hypertension (PAH). The RESPITE and REPLACE studies suggest that switching from a PDE5i to a soluble guanylate cyclase (sGC) activator may provide clinical improvement in this situation. The optimal approach to escalation or transition of therapy in this or other scenarios is not well defined. We developed an expert consensus statement on the transition to sGC and other treatment escalations and transitions in PAH using a modified Delphi process. The Delphi process used a panel of 20 physicians with expertise in PAH. Panelists answered three questionnaires on the management of treatment escalations and transitions in PAH. The initial questionnaire included open‐ended questions. Later questionnaires consolidated the responses into statements that panelists rated on a Likert scale from −5 (strongly disagree) to +5 (strongly agree) to determine consensus. The Delphi process produced several consensus recommendations. Escalation should be considered for patients who are at high risk or not achieving treatment goals, by adding an agent from a new class, switching from oral to parenteral prostacyclins, or increasing the dose. Switching to a new class or within a class should be considered if tolerability or other considerations unrelated to efficacy are affecting adherence. Switching from a PDE5i to an SGC activator may benefit patients with intermediate risk who are not improving on their present therapy. These consensus‐based recommendations may be helpful to clinicians and beneficial for patients when evidence‐based guidance is unavailable. [ABSTRACT FROM AUTHOR]

The article presents a study on the prevalence of acute vasoresponsiveness on pulmonary hypertension (PH) patients. The study used vasodilator testing on PH patients with epoprostenol or nitric oxide to explore vasoresponsiveness in the patients. Results reveal that 26% have responded to the acute vasodilator therapy. The study concludes that significant number of PH patients are acutely vasoresponsive to vasodilator therapy and that PH patients may benefit from calcium channel blockers.

OBJECTIVES: To determine the provider cost of administering intensive care unit (ICU) services, comparing 3 different staffing models for ICU coverage, and to compare the costs of using house staff vs nonphysician providers (NPPs). METHODS: Data were collected on total staff composition and number of beds in ICUs from January 1, 2004, through December 31, 2004, at the 3 Mayo Clinic sites: Rochester, Minn; Jacksonville, Fla; and Scottsdale, Ariz, institutional or national average staff salaries were used to determine total staffing costs per ICU bed per year at each site. Medicare medical education reimbursements were also taken Into account. RESULTS: Costs per ICU bed for physician staffing were $18,630 in Rochester, $37,515 in Jacksonville, and $38,010 in Scottsdale. When NPPs were substituted for house staff, the costs per bed were $72,466 in Rochester, $61,291 in Jacksonville, and $49,909 in Scottsdale. Incremental costs per ICU bed using NPPs were $53,836 in Rochester, $23,776 in Jacksonville, and $11,899 in Scottsdale. CONCLUSION: Use of residents and fellows in ICU staffing at a major tertiary health center is more cost-efficient than use of NPPs. This finding could have implications for the cost of physician services In nonteaching community hospitals and the methods by which care is provided. [ABSTRACT FROM AUTHOR]

Objectives: Angiotensin-converting enzyme inhibitors (ACEIs) and angiotensin receptor blockers (ARBs) are the most commonly prescribed antihypertensives, with prior studies identifying a possible association between long-term use and increased rates of lung cancer. This study evaluated this potential association in a large population using propensity matching.Methods: This was a population-based cohort study in a large healthcare system in three regions of the United States. Pairwise propensity score matching was performed using demographics and comorbidities. All of the adult patients in the healthcare system from January 1, 2000 to April 30, 2018 with at least 1 year of follow-up were included.Results: In total, 3,253,811 patients with a median age of 59 (range 18-103) years were included. The ACEI group had a higher freedom from lung cancer versus controls at 15 years (98.47%, 95% confidence interval [CI] 98.41-98.54) versus 98.26%, (95% CI 98.20-98.33), whereas ARBs had similar rates versus controls at all time points. For patients diagnosed as having lung cancer, median all-cause survival was significantly higher in the ACEI (34.7 months, 95% CI 32.8-36.6) and ARB (30.9 months, 95% CI 28.1-33.8) groups than the control group (20.6 months, 95% CI 20.1-21.1).Conclusions: This study showed lower rates of lung cancer with ACEI use and no difference in risk with ARBs. In addition, use of these medications was found to be associated with increased survival in those diagnosed as having lung cancer. This study supports the continued use of these medications without concern for increasing the risk of lung cancer. [ABSTRACT FROM AUTHOR]

Background: Pulmonary arterial hypertension is a progressive, fatal disease. Published treatment guidelines recommend treatment escalation on the basis of regular patient assessment with the goal of achieving or maintaining low-risk status. Various strategies are available to determine risk status. This analysis describes an update of the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) risk calculator (REVEAL 2.0) and compares it with recently published European Society of Cardiology/Respiratory Society guideline-derived risk assessment strategies.Methods: A subpopulation from the US-based registry REVEAL that survived ≥ 1 year postenrollment (baseline for this cohort) was analyzed. For REVEAL 2.0, point values and cutpoints were reassessed, and new variables were evaluated. The Kaplan-Meier method was used to estimate survival at 12 months postbaseline; discrimination was quantified using the c-statistic. Mortality estimates and discrimination were compared between REVEAL 2.0 and Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA) and French Pulmonary Hypertension Registry (FPHR) risk assessment strategies. For this comparison, a three-category REVEAL 2.0 score was computed in which patients were classified as low-, intermediate-, or high-risk.Results: REVEAL 2.0 demonstrated similar discrimination as the original calculator in this subpopulation (c-statistic = 0.76 vs 0.74), provided excellent separation of risk among the risk categories, and predicted clinical worsening as well as mortality in patients who were followed ≥ 1 year. The REVEAL 2.0 three-category score had greater discrimination (c-statistic = 0.73) than COMPERA (c-statistic = 0.62) or FPHR (c-statistic = 0.64). Compared with REVEAL 2.0, COMPERA and FPHR both underestimated and overestimated risk.Conclusions: REVEAL 2.0 demonstrates greater risk discrimination than the COMPERA and FPHR risk assessment strategies in patients enrolled in REVEAL. After external validation, the REVEAL 2.0 calculator can assist clinicians and patients in making informed treatment decisions on the basis of individual risk profiles.Trial Registry: ClinicalTrials.gov; No. NCT00370214; URL: www.clinicaltrials.gov. [ABSTRACT FROM AUTHOR]

Objectives: To describe the effect of implementing a contemporary perioperative pulmonary hypertension (PH)-targeted protocol in patients with pulmonary arterial hypertension (PAH) undergoing noncardiac surgery (NCS).Methods: The data of consecutive patients with PAH diagnosed by right heart catheterization who underwent NCS between January 1, 2006 and February 9, 2016 were reviewed. Patient demographics, etiology of PAH, clinical features, diagnostic data, utilization of PH-specific medications, and trend of perioperative complications rate were recorded during the study period.Results: In the base cohort of 375 patients, 37 had NCS. The mean age at surgery was 62 years. Most patients were women (78%) classified in group 1 PAH. At the time of the surgery, 86% were New York Heart Association functional class III/IV and 97% had American Society of Anesthesiologists classifications 3 and 4. A larger proportion of patients displayed lower PAH risk scores between 2006 and 2011 (P = 0.045). Conversely, a higher percentage of patients exhibited moderately high to very high PAH risk scores between 2012 and 2016 (P = 0.003). Perioperative and anesthetic-related morbidity was 27%, and no difference was observed between either period (P = 0.944). Most of the complications (70%) were related to general anesthesia. Two deaths (5%) occurred in our study group, both during the 2006-2011 period.Conclusions: The combination of a multidisciplinary perioperative approach, utilization of novel pulmonary vascular disease-targeted therapy, adequate perioperative optimization, and thoughtful selection of anesthetic technique seems to be a potential strategy to at least maintain similar perioperative outcomes among higher- and lower-risk patients with PAH undergoing NCS. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate the utility of anti-nuclear antibody (ANA) levels in distinguishing the cause of pulmonary arterial hypertension as idiopathic (IPAH) or connective tissue disease related (CTD-PAH). Methods: We retrospectively identified patients with IPAH or CTDPAH seen between 2010 and 2012 at our institution. Medical records were reviewed for demographic and clinical data and laboratory values. Results: Of 115 patients identified, 65 (56%) had IPAH and 50 (44% had CTD-PAH. The mean age was 59 years and most of the patients (76%) were women. Most patients (64%) were in World Health Organization functional class III or IV Compared with the IPAH group, the CTD-PAH group had significantly increased B-type natfiuretic peptide levels (635 vs 325 pg/mL; P = 0.02) and decreased pulmonary vascular resistance (6 vs 9 WU; P = 0.04). The median ANA level was significantly higher in the CTD-PAH group than the IPAH group (7 vs 0 U; P < 0.001). The area under the receiver operating characteristic curve for a positive ANA to predict CTD-PAH was 0.91 (P < 0.001). A cutoff of 5 U for predicting ANA provided an optimal specificity of 94% and a sensitivity of 70%. The resulting likelihood ratio using the same cutoff was 12 (P < 0.001), or a positive predictive value of 91% with a negative predictive value of 79%. Conclusions: In this selected cohort of patients, a quantitative ANA value >5 U may be useful in distinguishing CTD-PAH from IPAH, but a lower level does not confidently exclude CTD-PAH. [ABSTRACT FROM AUTHOR]

Background: Portopulmonary hypertension (POPH) is defined as pulmonary arterial hypertension (PAH) in the context of portal hypertension. Severe POPH has been considered an absolute contraindication of orthotopic liver transplantation (OLT). Objective: Since there are no definitive guidelines for the immediate preoperative, intraoperative, and postoperative evaluation and treatment of POPH patients, we have used published literature along with our experience to review current knowledge in this area. [ABSTRACT FROM AUTHOR]

The article presents a study which investigates the feasibility of having echocardiographic data that could reflect pulmonary artery pressures (PAP) and right ventricular function during exercise in patients with pulmonary arterial hypertension (PAH). Ten patients undergoing right heart catheterization (RHC) were evaluated using echocardiography. Results reveal the feasibility of exercise echocardiography for the assessment of hemodynamics and right heart function in PAH patients.

The article discusses the study which evaluated the outcomes of screening patients with pulmonary hypertension (PH) for human immunodeficiency virus (HIV) infection. The researchers identified and reviewed the records of PH patients who had serologic testing for HIV antibody from July 1992 to November 2008. The study found that PH presence alone did not represent enough pretest probability to warrant HIV screening.

The article discusses a research study on the accuracy and precision of several echocardiographic methods for calculating mean pulmonary artery pressure (MPAP). The study subjects included patients with simultaneous echocardiography and right-sided heart catheterization (RHC). The three methods that were used for the calculation of MPAP included the Syyed equation, the Chemla equation, and the mean gradient method.

The article presents a retrospective study on the association between pulmonary hypertension (PH) and thyroid dysfunction in Jacksonville Pulmonary Hypertension Center in Jacksonville, Florida from 1992 to 2006. It has found out that out of the 356 patients, 250 were female and median age was 65 years and most of them have suffered from severe pulmonary artery systolic pressure. It has determined that a significant percentage of PH patients have coexisting thyroid dysfunction.

Objective: The objective of this study was to evaluate the prevalence of acute bronchodilator responses in patients who were evaluated for pulmonary lymphangioleiomyomatosis. Methodology: This study was a retrospective review of the medical records of 50 patients, who were assessed for lymphangioleiomyomatosis from 1978 to 2002 at Mayo Clinic in Rochester, Minnesota, and Jacksonville, Florida, USA. Each medical record was reviewed for pertinent clinical and pulmonary function test data, including results for lung volume and flow, diffusing capacity and response to bronchodilator. Results: At initial testing, the mean FEV1/FVC ratio for the whole group was 60%; 35 patients (70%) had airflow obstruction; five patients (10%) had only restriction; and 10 (20%) had neither restriction nor obstruction. Three patients (6%) showed a positive response to bronchodilator at initial testing and only one patient showed a positive response at 1 year. Pulmonary function testing at 1-year follow up was available in a subgroup of this cohort (23 patients; 46%), and only one patient (2%) had a positive response to bronchodilator. Although there was a statistically significant decrease in the FEV1/FVC ratio (from 64% to 62%; P = 0.02), no other differences were found at 1-year follow up. Conclusions: In its advanced state, lymphangioleiomyomatosis produces irreversible airflow obstruction. A positive response to bronchodilator was unusual. Other measures of pulmonary function showed little or no change over a 12-month period. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To examine the frequency and microbial pattern of pneumonia and its effect on survival in the current era of orthotopic liver transplantation (OLT). PATIENTS AND METHODS: At the Mayo Clinic in Jacksonville, Fla, the medical records of consecutive patients who underwent their first OLT between February 1998 and January 2001 were retrospectively reviewed through the end of the first year post-transplantation. RESULTS Of 401 study patients, 20 developed pneumonia; estimates of incidence with corresponding 95% confidence interval (CI) at 1 and 12 months were 3% (1%–5%) and 5% (3%–7%), respectively. Pseudomonas aeruginosa was the predominant microorganism identified (in 8 of 14 patients) during the first month after transplantation. Between the second and sixth months, 2 of the 4 cases of pneumonia were due to fungal infections of Aspergillus fumigatus. Cytomegalovirus was associated with Aspergillus in 1 patient. No other viral or Pneumocystis carinii pneumonia was diagnosed. There were only 2 cases of pneumonia between 7 months and 1 year after transplantation, neither of which was fungal. Approximately 40% (95% CI, 14%–58%) of patients with pneumonia died within 1 month after diagnosis. The relative risk of mortality in the first month after onset of pneumonia was estimated to be 24 (95% CI, 10–54), which is strong evidence of increased risk of mortality with pneumonia (P«.001). CONCLUSIONS: Pneumonia appears to occur less often after OLT than previously reported but still has a substantial negative effect on survival. In the early period after OLT, P aeruginosa continues to be the predominant organism causing pneumonia. [ABSTRACT FROM AUTHOR]

Acute mesenteric ischemia is a life-threatening vascular emergency that requires early diagnosis and intervention to adequately restore mesenteric blood flow and to prevent bowel necrosis and patient death. The underlying cause is varied, and the prognosis depends on the precise pathologic findings. Despite the progress in understanding the pathogenesis of mesenteric ischemia and the development of modern treatment modalities, acute mesenteric ischemia remains a diagnostic challenge for clinicians, and the delay in diagnosis contributes to the continued high mortality rate. Early diagnosis and prompt effective treatment are essential to improve the clinical outcome. [ABSTRACT FROM AUTHOR]

Rationale: Although there have been reports of pulmonary toxicity with low-dose amiodarone, it is generally believed that low-dose therapy is safe. Methods: The clinical data for eight patients identified from a retrospective chart review are presented. Results: All of the patients were receiving amiodarone, 200 mg/d, for an average of 2 years. The average age was 77 years (range, 65 to 89 years). Seven of the eight patients were male. Seven of the eight patients presented with dyspnea on exertion, and three of the eight patients presented with cough. All of the patients had a clinical diagnosis of amiodarone-induced pulmonary toxicity. Open-lung biopsies were obtained on two patients that were consistent with amiodarone-induced pulmonary toxicity. None of the patients were in congestive heart failure. Treatment involved cessation of amiodarone. In addition, three patients received corticosteroids. Five of the patients improved symptomatically with this conservative approach, and four patients improved radiographically. One patient died with progressive respiratory insufficiency (presumably from amiodarone pulmonary toxicity). One patient was unavailable for follow-up. Conclusion: Amiodarone-induced pulmonary toxicity can occur at a daffy dose of 200 mg. Clinicians must remain alert to this possibility even with this low-dose therapy. [ABSTRACT FROM AUTHOR]

Registry data worldwide indicate an overall female predominance for pulmonary arterial hypertension (PAH) of 2–4 over men. Genetic predisposition accounts for only 1–5% of PAH cases, while autoimmune diseases and infections are closely linked to PAH. Idiopathic PAH may include patients with undiagnosed autoimmune diseases based on the relatively high presence of autoantibodies in this group. The two largest PAH registries to date report a sex ratio for autoimmune connective tissue disease-associated PAH of 9:1 female to male, highlighting the need for future studies to analyze subgroup data according to sex. Autoimmune diseases that have been associated with PAH include female-dominant systemic sclerosis, systemic lupus erythematosus, rheumatoid arthritis, Sjögren's syndrome, and thyroiditis as well as male-dominant autoimmune diseases like myocarditis which has been linked to HIV-associated PAH. The sex-specific association of PAH to certain infections and autoimmune diseases suggests that sex hormones and inflammation may play an important role in driving the pathogenesis of disease. However, there is a paucity of data on sex differences in inflammation in PAH, and more research is needed to better understand the pathogenesis underlying PAH in men and women. This review uses data on sex differences in PAH and PAH-associated autoimmune diseases from registries to provide insight into the pathogenesis of disease. [ABSTRACT FROM AUTHOR]