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Eccrine angiomatous hamartoma (nevus) is a rare form of congenital tumorous malformation with proliferation of eccrine secretory coils and ducts, surrounding capillary angiomatous channels and occasionally other minor elements. To date, there have been only about 24 cases reported in the literature. We report an additional case with more detailed description of the microscopic findings, including immunohistochemcial observations. The patient was a 28-year-old female who presented with painless, flesh- to reddish brown-colored, violacaeous or bluish subcutaneous nodules on the extremities and trunk. The tumors did not show sweating following exertion. The histologic features were comparable to the previous reported cases. The hamartomatous eccrine sweat glands and ducts and a few apocrine glands demonstrated qualitatively diminished antigens commonly found in the eccrine sweat appartuses, such as carcinoembryonic antigen (CAE) AND S-100 protein. The findings of CD34, CD44, human nerve growth factor receptor and Ulex europaeus antigens have not been previously reported. The histologic features suggested a "harmartomatous" growth rather than a true neoplastic process. [ABSTRACT FROM AUTHOR]

Patients, often with underlying rheumatologic disease, may present with pericardial effusions in the setting of pulmonary hypertension (PHTN). Pericardial drainage in PHTN is associated with significant morbidity and mortality. We describe a patient with PHTN who developed cardiac tamponade that was managed safely and effectively with pulmonary artery catheter-guided pericardiocentesis., Competing Interests: The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

Background: Chronic obstructive pulmonary disease-associated pulmonary hypertension (PH-COPD) results in a significant impact on symptoms, quality of life, and survival. There is scant and conflicting evidence about the use of pulmonary hypertension (PH) specific therapy in patients with PH-COPD. Study Design and Methods: PubMed, OVID, CINAHL, Cochrane, Embase, and Web of Science were searched using various MESH terms to identify randomized controlled trials (RCTs) or observational studies investigating PH-specific therapies in patients with severe PH-COPD, defined by mean pulmonary artery pressure (mPAP) of more than 35 mm Hg or pulmonary vascular resistance (PVR) of more than 5 woods units on right heart catheterization. The primary outcome was a change in mPAP and PVR. Secondary outcomes were changes in six-minute walk distance (6MWD), changes in the brain-natriuretic peptide (BNP), New York Heart Association (NYHA) functional class, oxygenation, and survival. Results: Thirteen studies satisfied the inclusion criteria, including a total of 328 patients with severe PH-COPD. Out of these, 308 patients received some type of specific therapy for PH. There was a significant reduction in mPAP (mean difference (MD) -3.68, 95% CI [-2.03, -5.32], p < 0.0001) and PVR (MD -1.40 Wood units, 95% CI [-1.97, -0.82], p < 0.00001). There was a significant increase in the cardiac index as well (MD 0.26 L/min/m 2 , 95% CI [0.14, 0.39], p < 0.0001). There were fewer patients who had NYHA class III/lV symptoms, with an odds ratio of 0.55 (95% CI [0.30, 1.01], p = 0.05). There was no significant difference in the 6MWD (12.62 m, 95% CI [-8.55, 33.79], p = 0.24), PaO 2 (MD -2.20 mm Hg, 95% CI [-4.62, 0.22], p = 0.08), or BNP or NT-proBNP therapy (MD -0.15, 95% CI [-0.46, 0.17], p = 0.36). Conclusion: The use of PH-specific therapies in severe PH-COPD resulted in a significant reduction in mPAP and PVR and increased CI, with fewer patients remaining in NYHA functional class III/IV. However, no significant difference in the 6MWD, biomarkers of right ventricular dysfunction, or oxygenation was identified, demonstrating a lack of hypoxemia worsening with treatment. Further studies are needed to investigate the use of PH medications in patients with severe PH-COPD.

Purpose of Review: Hospitalization in pulmonary arterial hypertension (PAH) patients is an important clinical worsening event significantly associated with subsequent mortality. Furthermore, irrespective of the cause of hospitalization, the overall outcome is closely related to the severity of the right ventricular (RV) dysfunction. Therefore, understanding the pathophysiology of pulmonary hypertension and RV failure is paramount in successfully managing PAH patients requiring hospitalization. This review highlights diagnostic and therapeutic approaches in various clinical scenarios that might be encountered during hospitalization of the World Health Organization group I PAH patient., Recent Findings: This article covers recent literature describing risk factors, predictors of outcome and state-of the art management approach to a hospitalized PAH patients with a special focus on management of RV failure and common complications in PAH requiring hospitalization., Summary: The review highlights the importance of multidisciplinary approach to a hospitalized PAH patient and highlight important implications in clinical practice and knowledge gaps for potential future research., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Sequential triple combination therapy is recommended for pulmonary arterial hypertension (PAH) patients who are not at therapeutic goal on dual therapy, but long-term data on efficacy and safety is scarce., Objective: To assess the long-term impact of sequential triple combination therapy in patients with PAH who are not at goal on dual combination therapy., Study Design and Methods: We performed a retrospective observational study in a racially/ethnically diverse cohort of consecutive PAH patients on a stable dual therapy regimen who remained in intermediate- or high-risk category and were subsequently initiated on sequential triple combination therapy. We studied interval change in functional, echocardiographic, and hemodynamic parameters, REVEAL 2.0 risk category and ERS/ESC 2022 simplified four-strata risk category. Multivariate logistic regression analysis was performed to identify independent predictors of successful risk reduction (achievement or maintenance of REVEAL 2.0 low-risk category). Kaplan-Meier survival curves were created to assess the effect of risk reduction on survival., Results: Out of 414 PAH patients seen in our program, 55 patients received add-on sequential triple combination regimen and had follow-up hemodynamic data. The mean age was 57 years, with 85% women. The most common etiology of PAH was idiopathic/heritable (41.8%). Most patients were WHO functional class III (76.4%), and 34.5% of patients were in high-risk category (REVEAL 2.0). On a median follow-up of 68 weeks, there was a significant improvement in WHO Functional Class ( p  < 0.001), six-minute walk distance (35 m) with 61.8% of patients achieving low-risk status by REVEAL 2.0, and a 28% of patients' improvement in pulmonary vascular resistance. Female gender was identified as a strong predictor of successful risk reduction, whereas Hispanic ethnicity estimated right atrial pressure on echocardiogram and pericardial effusion predicted lower probability of risk reduction. Patients who achieved or maintained low-risk status had significantly improved survival., Conclusion: Add-on sequential triple combination therapy significantly increased functional, echocardiographic, and hemodynamic parameters with improvement in risk category and survival.

Dual combination therapy with a phosphodiesterase-5 inhibitor (PDE5i) and endothelin receptor antagonist is recommended for most patients with 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., Competing Interests: Franck F. Rahaghi reports consultation, research, and speakership honoraria from Bayer and Janssen, consultation and speakership from United Therapeutics, and consultation fees from Acceleron. Vijay P. Balasubramanian reports a research grant from United Therapeutics and serves on a speakers bureau for Bayer. Robert C. Bourge reports research grant support to my institution from United Therapeutics and Bayer, and service on a Scientific Advisory Board at United Therapeutics. Murali M. Chakinala reports grants or contracts from Actelion/Janssen, Bayer, Medtronic, NIH, Reata, Liquidia, Phase Bio, Complexa, United Therapeutics, Altavant, Trio Health Analytics, Reata, Acceleron, Arena, and Gossamer; consulting fees from Altavant, Vaderis Therapeutics, Aerovate, Reata, VWave, and Arena; honoraria from Bayer, Gilead, Simply Speaking, WebMD, and United Therapeutics; support for attending meetings and/or travel from Actelion/Janssen, United Therapeutics, Bayer, Acceleron, Reata, and Gilead; participation on a Data Safety Monitoring Board or Advisory Board for Actelion/Janssen, Express Scripts, Phase Bio, Altavant, Gossamer, United Therapeutics, Bayer, Acceleron, and Liquidia; and leadership or a fiduciary role in the Pulmonary Hypertension Association and the Cure HHT Global Research and Medical Advisory Board. Michael S. Eggert reports research contracts with United Therapeutics (BREEZE and ADVANCE Outcomes), Acceleron, and Actelion. Jean M. Elwing reports grants from Actelion, Acceleron, Reata, United Therapeutics, Liquidia, Phase Bio, Complexa, Gossamer Bio, Bayer, Arena, Eiger, Akros, Bellerophon, and Lung LLC and consulting fees from United Therapeutics, Acceleron, Liquidia, Altavant, Bayer, Gossamer Bio, Actelion, Bayer. Jeremy Feldman reports consulting and giving talks for Bayer, United Therapeutics, and Jansen. Christopher King reports personal fees from Actelion, personal fees from Genentech, United Therapeutics, and Boehringer Ingelheim; has served on advisory boards for and is on the Speakers’ Bureau of Actelion, Boehringer‐Ingelheim Pharmaceuticals, and United Therapeutics. James R. Klinger reports that his institution receives research funding from United Technologies, service on a Steering Committee and a Clinical Outcomes Committee for Bayer, and a leadership role in the Pulmonary Hypertension Association. Stephen C. Mathai reports personal fees from United Therapeutics; participation on a data safety monitoring board or advisory board from United Therapeutics, Actelion, and Bayer, and leadership or a fiduciary role in the PCORI Rare Disease Advisory Panel and the World Symposium on Pulmonary Hypertension. John Wesley McConnell reports consulting fees from Actelion, Bayer, Gossamer, Altavant, and Liquidia; honoraria from Actelion, Bayer, Simply Speaking, Impact PH, and Reata, and participation on a data safety monitoring board or advisory board for Actelion, Liquidia, Gossamer, and Altavant. Harold I. Palevsky reports honoraria for serving on scientific advisory boards for Acceleron, Actelion/Janssen, PhaseBio and United Therapeutics, and serving on a DSMB for United Therapeutics. Ricardo Restrepo‐Jaramillo reports serving on speaker's bureau for United Therapeutics, Bayer, and Actelion. Zeenat Safdar reports serving on a speakers bureau, consultation and advisory boards for Actelion, United Therapeutics, Boehringer Ingelheim, Bayer, and Roche. Jeffrey S. Sager reports personal fees from Bayer pharmaceuticals, grants and personal fees from United Therapeutics, grants and personal fees from Janssen (J and J), and grants from Reata outside the submitted work. Namita Sood reports a speaking fee from Bayer. Roxana Sulica reports research grants from Bayer, United Therapeutics, Complexa, and Reata, and serves on advisory boards for Actelion, Bayer, United Therapeutics, and Reata. R. James White reports research grants from United Therapeutics, Reata, Bayer, Merck, and Janssen and consulting fees from Merck and Bayer. Nicholas S. Hill reports honoraria from Axon Research for this study; grants to his institution from Actelion, Bayer, Gilead, and United Therapeutics; consulting fees from United Therapeutics; and participation in Data Safety Monitoring Boards for United Therapeutics and Pfizer. All authors had access to the Delphi questionnaire analysis and data and participated in the review, revision, and approval of the content of the manuscript for submission. Charles D. Burger reports no disclosures or conflicts of interest., (© 2022 The Authors. Pulmonary Circulation published by John Wiley & Sons Ltd on behalf of Pulmonary Vascular Research Institute.)

The management of pulmonary arterial hypertension (PAH) has significantly evolved over the last decades in the wake of more sensitive diagnostics and specialized clinical programs that can provide focused medical care. In the current era of PAH care, 1-year survival rates have increased to 86%-90% from 65% in the 1980s, and average long-term survival has increased to 6 years from 2.8 years. The heterogeneity in the etiology and disease course has opened doors to focusing research in phenotyping the disease and understanding the pathophysiology at a cellular and genetic level. This may eventually lead to precision medicine and the development of medications that may prevent or reverse pulmonary vascular remodeling. With more insight, clinical trial designs and primary end-points may change to identify the true survival benefit of pharmacotherapy. Identifying responders from non-responders to therapy may help provide individualized patient-centered care rather than an algorithm-based approach. The purpose of this review is to highlight the latest advances in screening, diagnosis, and management of PAH., Competing Interests: Competing interests: RS has received research support from Bayer, United Therapeutics, Reata and Complexa, and served on advisory boards for Actelion, Altavant, Bayer, Gossamer, and United Therapeutics., (© American Federation for Medical Research 2021. Re-use permitted under CC BY-NC. No commercial re-use. Published by BMJ.)

Information on outcomes of COVID-19 in pulmonary arterial hypertension (PAH) patients is limited to a few case series and surveys. Here, we describe our experience at a large Pulmonary Hypertension Center in New York City at the height of the pandemic. We performed a retrospective chart review of eleven consecutive PAH patients who were diagnosed with SARS-CoV-2 infection. We analyzed demographics, PAH severity, risk factors for COVID-19, and COVID-19 severity and outcomes. We found in our sample that 63.6% of patients required intensive care, and there was a 45.45% overall mortality. Most patients had a known COVID-19 contact and mean duration of symptoms prior to presentation was 12 days. Only 4/11 (36%) patients presented to a center with pulmonary hypertension expertise, all of whom survived. Most patients had at least moderate pulmonary hypertension with an average REVEAL score of 7.81 despite double or triple PAH therapy. Our cases series underscores the gravity of SARS-CoV-2 infection in patients with PAH. It also suggests possible interventions to prevent unfavorable outcomes such as preserving social distancing, PAH management optimization, and early and preferential presentation to a center with specialized expertise in PAH.

Pulmonary hypertension (PH) is an end result of a diverse array of complex clinical conditions that invoke hemodynamic and pathophysiological changes in the pulmonary vasculature. Many patients' symptoms begin with dyspnea on exertion for which screening tests such as chest roentgenograms and more definitive noninvasive tests such as CT scans are ordered initially. It is imperative that clinicians are cognizant of subtle clues on these imaging modalities that alert them to the possibility of PH. These clues may serve as a stepping stone towards more advanced noninvasive (echocardiogram) and invasive (right heart catheterization) testing. On the CT scan, the signs are classified into mediastinal and lung parenchymal abnormalities. In addition to suspecting the diagnosis of PH, this paper provides a pictorial essay to guide health care professionals in identifying the etiology of PH. This paper also provides concrete definitions, wherever possible, of what constitutes abnormalities in PH, such as dilated pulmonary arteries, pruning of vessels, and increased thickness of free wall of the right ventricle. The sensitivities and specificities of each sign are enumerated. The common radiographic and clinical features of many different etiologies of PH are tabulated for the convenience of the readers. Some newer imaging modalities such as dual-energy CT of the chest that hold promise for the future are also described., (Copyright © 2019. Published by Elsevier Inc.)

In this open-label study, we evaluated the effect of upfront macitentan and riociguat combination in newly diagnosed pulmonary arterial hypertension (PAH) patients. In 15 consecutive PAH patients, we collected clinical and hemodynamic data at baseline, visit 1 (median 4 months) and visit 2 (median 12 months). Survival and transplantation status were analyzed over 36 months. Statistical analysis included student t-test and 95% confidence interval (CI) ( t-statistic or Clopper-Pearson). Kaplan-Meier was used to estimate survival rate. There were 11/15 women (mean age 56 years), in World Health Organization (WHO) functional class (FC) III ( n = 14) or IV ( n = 1). The 6 min walk distance increased from 281.6 m (baseline) to 315.7 m (visit 1) and visit 2 (313.9 m), representing a 34- and 32-m change ( P < 0.05), respectively, associated with Borg score improvements. Brain natriuretic peptide decreased: 318.2 pg/mL (baseline) to 122.0 pg/mL (visit 1) and 98.6 pg/mL (visit 2) ( P < 0.05). WHO FC improved in eight patients (53%, 95% CI 27%-79%). Pulmonary vascular resistance (9.2 to 5.7 Wood Units) and mean pulmonary artery pressure (47.3 to 38.9 mmHg) decreased; cardiac index increased (2.3 to 3.0 L/min/m 2 ) (baseline to visit 2, all P < 0.05). All patients had intermediate and high risk score (baseline); at 1-year follow-up, dual therapy led to reduction to low risk score in 7/15 (47%) patients. There were no unexpected or serious side effects. Three patients died due to unrelated causes; one patient received a lung transplant. Transplant-free survival rate (36 months) was 85%. Preliminary evidence is provided for effectiveness of initial macitentan and riociguat combination therapy in PAH.

Objectives: The aim of this study was to determine the prevalence of exercise-induced pulmonary hypertension (EIPH) in consecutive subjects referred for stress echocardiography for chest pain or shortness of breath and correlate echocardiographic diagnosis of EIPH with hemodynamics at right heart catheterization (RHC)., Background: Elevated pulmonary pressure can lead to significant morbidity and mortality. EIPH by ehocardiography has been described in patients with connective tissue disease. It's prevalence in the setting of routine clinically indicated stress echocardiography unknown., Methods: In a retrospective analysis of 4068 consecutive stress subjects undergoing stress echocardiography, 479 subjects with EIPH were identified. All 479 subjects with EIPH were compared to 479 age and sex matched subjects with normal pulmonary artery pressures post exercise. EIPH was defined as PASP>50mmHg or peak tricuspid regurgitation velocity>3.2m/s. Of 100 patients with EIPH who underwent RHC we identified variables which predicted abnormal hemodynamic findings on RHC., Results: The prevalence of EIPH in subjects referred for stress echocardiography was 11.7%. A greater proportion of subjects with EIPH were obese or had lung disease or connective tissue disease. Of 100 subjects who underwent RHC, 65 had abnormal results. Age>55years (OR 5.1, p<0.01]) or dilated left atrium (OR 4.4, p=0.02]) were independently associated with abnormal right heart hemodynamics., Conclusions: The results demonstrate that 11.7% of patients undergoing clinically indicated stress echocardiography have EIPH. Of those who underwent RHC abnormal hemodynamics were significantly associated with a dilated left atrium or age older than 55years., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Pulmonary hypertension (PH) due to chronic respiratory disease and/or hypoxia is classified as World Health Organization (WHO) Group III pulmonary hypertension. The patients most commonly encountered in clinical practice with group III PH include those with chronic obstructive lung disease (COPD), diffuse parenchymal lung disease, and sleep-disordered breathing. The purpose of this review is to outline the variable clinical significance of pulmonary hypertension in the most common pulmonary disease states and how a clinician may approach the management of these patients., Competing Interests: The authors declare no conflict of interest.

Introduction: Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are rare, life-threatening diseases in which chronically elevated pressure in the pulmonary arteries results in vascular remodeling and right heart failure. Treatment goals are to improve patient functioning, exercise capacity, and symptoms; delay disease progression; normalize the right ventricular function; and, ultimately, improve survival. Therapeutic management centers on the affected physiologic pathways and includes endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and prostacyclins. Recently, riociguat, a novel therapeutic agent that stimulates soluble guanylate cyclase via the nitric oxide pathway, was approved for the treatment of both PAH and CTEPH. Clinical trial data show that riociguat significantly improves exercise capacity as well as hemodynamic parameters in PAH/CTEPH., Methods: We report on the early use of riociguat at our center-a large, metropolitan pulmonary hypertension treatment facility that cares for >250 patients with PAH/CTEPH. Through our initial clinical experience, we offer evidence on the benefits of riociguat in three patients with PAH associated with different etiologies, symptoms, and treatment goals., Results: Overall, patients at our center who have received riociguat have experienced clinical benefits, including improvement in symptomatic and hemodynamic parameters, increase in 6-min walk distance, and improvement or stabilization of World Health Organization functional class. In several cases, initial response to riociguat has been encouraging and has helped patients reach their treatment goals. Riociguat appears to be well tolerated, with only one patient experiencing mild, self-limiting side effects., Conclusion: Novel agents are continuously being introduced into the PAH/CTEPH armamentarium, and clinicians must decide how best to integrate them into their existing treatment algorithms. This case series offers initial evidence from our practice on the benefits of riociguat in optimizing hemodynamic and functional parameters. These benefits have been observed in PAH associated with different etiologies and functional status, and in both first-line and combination use., Funding: Bayer HealthCare Pharmaceuticals.

A retrospective analysis of 23 patients was undertaken to evaluate the outcome of pulmonary rehabilitation (PR) in patients with pulmonary hypertension (PH) over a preceding 6-year time frame. Chart review and data analyses were undertaken evaluating pulmonary arterial hypertension pharmacotherapy versus the same therapy with the addition of PR. Analysis included 23 patients who had a mean pulmonary artery pressure of 36.6 while on therapy and who had initial six-minute walk tests (6MWTs) ranging between 54 and 396 meters. Outcomes included 6MWTs and Saint George's Respiratory Questionnaire. There was no improvement in 6MWT postpharmacotherapy. Patients with low 6MWT <250 meters had substantial gains in 6MWT postrehabilitation (average of 86 meters). As well, those with initial 6MWT >250 meters had a significant improvement of 52.55 meters, documenting the utility of PR in patients with PH. PH patients are increasingly seen in PR clinics as they share many characteristics present in chronic respiratory disease states. There is increasing literature supporting the utility of PR in this population. We have found that patients with 6MWTs as low as <250 meters are also candidates for PR and can demonstrate substantial benefits as measured using the 6MWTs., (© The Author(s) 2014.)

Calcific uremic arteriolopathy (CUA) is a rare and potentially fatal disorder of calcification involving subcutaneous small vessels and fat in patients with renal insufficiency. We describe the successful use of intravenous sodium thiosulfate (STS) for the treatment of CUA in two patients. The first case was complicated by the development of a severe anion gap metabolic acidosis, which was accompanied by a seizure. Both patients had complete wound healing within five months. Although STS should be considered in the treatment of CUA, little is known about pharmacokinetics and additional studies are required to determine dosing strategies to minimize severe potential side effects.

Pulmonary arterial hypertension (PAH) is characterized by progressive increases in pulmonary vascular resistance, leading to right heart failure and death. Guidelines recommend customization of treatment, necessitating the development of effective strategies for transitioning patients among treatments. In this study, we characterized our experience with patient transitions from parenteral prostacyclin to inhaled iloprost. We retrospectively assessed records from 11 centers of 37 consecutive patients with PAH aged ≥ 18 years who were treated with intravenous (IV) or subcutaneous (SC) prostacyclin analogues and transitioned to inhaled iloprost. The transition period began on the first day of inhaled iloprost with the intent of discontinuing parenteral prostacyclin and ended on the first day on inhaled iloprost free of parenteral prostacyclin. Persistence was defined as the absence of (1) parenteral prostacyclin while remaining on inhaled iloprost during post-transition Days 1-90 and (2) no reinitiation of parenteral prostacyclin during post-transition Days 90-365. All patients were clinically stable before transitioning to inhaled iloprost. The mean age was 46.5 years, 70.3% were female, 51.4% had idiopathic PAH, and 43.0% were in New York Heart Association Functional Class III. Among patients with an overlapping transition, the mean transition period was 10.5 days. A transition dosing algorithm was used in 10 patients (27.0%). At one year, 78.4% of the patients remained persistent on inhaled iloprost and 81.1% were free of clinical worsening. In selected patients on background oral PAH therapy, transitioning from parenteral prostacyclin to inhaled iloprost appears safe and feasible and is associated with long-term success. Further study is needed to define the optimal patient selection criteria and transition algorithm.

Over the past few years an increasing number of prospective controlled sarcoidosis treatment trials have been completed. Unfortunately, these studies utilize different endpoints making comparisons between studies difficult. At the recent World Association of Sarcoidosis and other Granulomatous disease (WASOG) meeting, a session was dedicated to the evaluation of clinical endpoints for various disease manifestations. These included pulmonary, pulmonary hypertension, fatigue, cutaneous, and a classification of clinical disease phenotypes. Based on the available literature and our current understanding of the disease, recommendations for clinical evaluation were proposed for each disease category. For example, it was recommended that pulmonary studies should include changes in the forced vital capacity. Additionally, it was recommended that all trials should incorporate measurement of quality of life.

Pulmonary hypertension (PH) is a recognized complication of sarcoidosis, with increased morbidity and poor prognosis. Sarcoidosis-associated pulmonary hypertension (SAPH) is typically seen in advanced cases, with pulmonary fibrosis, destruction and obliteration of the pulmonary vasculature, and chronic hypoxemia. PH can, however, occur in the absence of pulmonary fibrosis, suggesting alternative pathophysiological mechanisms. Diverse processes may coexist in the pathogenesis of SAPH, and there is an overlap with mechanisms of pulmonary arterial hypertension (PAH). This has encouraged the study of PAH-specific therapeutic agents in the treatment of SAPH. In small series, prostacyclin analogues, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors have been shown to improve hemodynamics, functional status, and outcomes. This article reviews the most recent data available in the epidemiology, pathophysiology, diagnosis, and treatment of SAPH., (Copyright Thieme Medical Publishers.)

Purpose: To retrospectively identify pulmonary arterial (PA) flow parameters measured with phase-contrast magnetic resonance (MR) imaging that allow noninvasive diagnosis of chronic PA hypertension (PAH)., Materials and Methods: The study was HIPAA compliant and was approved by the institutional review board; a waiver of informed consent was obtained. Fifty-nine patients (49 female patients; mean age, 46 years; range, 16-85 years) known to have or suspected of having PAH underwent breath-hold phase-contrast MR imaging and right-sided heart catheterization (RHC). The presence of PAH (mean pulmonary artery pressure [mPAP], >25 mm Hg) was confirmed in 42 patients. Parameters, including PA areas, PA strain, average velocity, peak velocity, acceleration time, and ejection time, were measured in each patient by investigators blinded to RHC results. These measurements were correlated with mPAP, systolic pulmonary artery pressure (sPAP), and pulmonary vascular resistance index (PVRI). The diagnostic ability of phase-contrast MR imaging to depict PAH was quantified. Statistical tests included Spearman rho coefficients, receiver operating characteristic curve analysis, and Bland-Altman plots., Results: Results showed average velocity to have the best correlation with mPAP, sPAP, and PVRI (r = -0.73, -0.76, and -0.86, respectively; P < .001). Average velocity (cutoff value = 11.7 cm/sec) revealed PAH with a sensitivity of 92.9% (39 of 42) and a specificity of 82.4% (14 of 17). Sensitivity and specificity for the minimum PA area (cutoff value = 6.6 cm(2)) were 92.9% (39 of 42) and 88.2% (15 of 17), respectively., Conclusion: The average blood velocity throughout the cardiac cycle is strongly correlated with pulmonary pressures and resistance.

Purpose: To retrospectively evaluate the accuracy and reproducibility of the cardiac magnetic resonance (MR) imaging-derived left ventricular septal-to-free wall curvature ratio for prediction of the right ventricular systolic pressure (RVSP) in patients clinically known to have or suspected of having pulmonary hypertension (PH), with same-day right-side heart catheterization (RHC) as the reference standard., Materials and Methods: Institutional review board approval was received for this HIPAA-compliant study. Sixty-one patients clinically known or suspected of having PH underwent cardiac MR and RHC on the same day. Interventricular septal curvature (C(IVS)) and left ventricular free wall curvature (C(FW)) measured at end systole were used to derive the curvature ratio (C(IVS)/C(FW)). Effective distending transmural pressure (dP(FW)) and transseptal pressure gradient (dP(IVS)) were assumed to be equivalent, respectively, to the systolic blood pressure (SBP) and the difference between SBP and RVSP. Curvature ratio and SBP were used to noninvasively estimate RVSP. Linear regression analysis was performed to assess the difference between curvature ratio and rate of pressure rise (dP) ratio (dP(IVS)/dP(FW)). The accuracy of the dichotomized curvature ratio in PH detection was analyzed by using receiver operating characteristic (ROC) curves., Results: PH, defined as RVSP higher than 40 mm Hg, was confirmed with RHC in 46 patients. A direct linear correlation between dP ratio and curvature ratio was observed (r = 0.85, P < .001). Bland-Altman analysis revealed moderate agreement between cardiac MR- and RHC-derived RVSPs (mean difference, -1.1 mm Hg +/- 15.9 [standard deviation]). ROC analysis of the accuracy of the curvature ratio for detection of increased RVSP revealed 87% sensitivity and 100% specificity (area under ROC curve, 0.95; P < .001). Intraobserver (r = 0.97) and interobserver (r = 0.95) curvature ratio measurements were closely correlated., Conclusion: In patients clinically known to have or suspected of having PH, cardiac MR-derived curvature ratio, as compared with RHC measurement, was an accurate and reproducible index for estimation of RVSP.

Pulmonary artery sarcomas are rare neoplasms of the pulmonary artery that are often confused with chronic thromboembolic disease, as both diseases have similar presentations. In patients with presumed chronic thromboembolic pulmonary hypertension, certain clinical and imaging characteristics may suggest the alternative diagnosis of pulmonary artery sarcoma. In this article we present a case of a man initially diagnosed with chronic thromboembolic pulmonary hypertension, but who was later found to have pulmonary artery sarcoma. We review the distinguishing characteristics of the two diseases and discuss possible treatment strategies.

Study Objective: To differentiate the clinical, radiographic, and physiologic profile in patients with sarcoidosis with and without pulmonary hypertension., Design: Retrospective survey., Setting: Tertiary care center., Patients: One hundred six patients with sarcoidosis were classified by two-dimensional echocardiography into two groups: group 1, 54 patients with pulmonary hypertension; group 2, 52 patients without pulmonary hypertension., Interventions: Patients underwent two-dimensional and Doppler echocardiography, chest radiography (CXR), pulmonary function testing, and arterial oxygen saturation determination, and the test results were compared between the two groups. Statistical analysis was performed using independent-sample t test and chi2 test, as appropriate; p < 0.05 was considered to be significant., Results: Predicted spirometric values and lung diffusing capacity were significantly lower in patients in group 1 compared to patients in group 2: FVC, 54% vs 64% (p = 0.0065), FEV(1), 47% vs 61% (p = 0.0005), forced expiratory flow, midexpiratory phase, 35% vs 52% (p = 0.0363), and single-breath diffusing capacity of the lung for carbon monoxide (D(LCO)sb), 39% vs 54% (p = 0.0001). Sixty percent of patients in group 1 had radiographic Scadding stage 4 sarcoidosis, while no radiographic stage predominated in group 2. Arterial oxygen saturation, need for oxygen supplementation, and degree of desaturation after exercise did not differ between groups., Conclusions: The presence of pulmonary hypertension in patients with sarcoidosis is associated with higher prevalence of stage 4 sarcoidosis by CXR and lower predicted spirometric and D(LCO)sb measurements.

Nitroglycerin and dipyridamole are two commonly available and well tolerated vasoactive medications. Their acute hemodynamic effects in patients with pulmonary arterial hypertension are not well defined in the current literature. The authors retrospectively analyzed the acute hemodynamic effects of IV nitroglycerin, dipyridamole, and epoprostenol in 59 patients with pulmonary arterial hypertension as determined by changes from baseline in systemic and pulmonary hemodynamic parameters. Statistical analysis was performed using the independent sample t test. A p value <0.05 was considered significant. Nitroglycerin is predominantly a vasodilator of the pulmonary vasculature with moderate systemic vasodilator effect, while dipyridamole is primarily a positive inotropic agent. Epoprostenol is a potent vasodilator of both pulmonary and systemic vessels and a strong positive inotropic agent. Nitroglycerin and dipyridamole may be useful in the acute management of pulmonary arterial hypertension.

Pulmonary arterial hypertension is a severe disease with poor prognosis, caused by obliteration of the pulmonary vasculature as a result of pulmonary-vascular remodeling, active vasoconstriction and in situ thrombosis. Left untreated, pulmonary arterial hypertension results in right-ventricular failure and death. There has been dramatic progress in the treatment of pulmonary arterial hypertension during recent years. A remarkable number of randomized-controlled trials with agents known to target specific abnormalities present in pulmonary arterial hypertension have been completed. Most commonly, therapeutic efficacy was judged by the ability of the drug under study to improve exercise capacity and to decrease the rate of severe complications. Completed clinical trials have mainly evaluated patients with relatively advanced disease. Despite these advances, responses to therapy in pulmonary arterial hypertension are not uniformly favorable and frequently incomplete. In addition, the methods of delivery and the adverse effect profile of the currently available pulmonary arterial hypertension-specific drugs create further management difficulties. Based on newly identified pathobiologic abnormalities in the pulmonary vasculature, future studies are likely to focus on the discovery of new therapeutic targets. Clinical trial design will continue to evolve in an attempt to enable inclusion of patients with less advanced disease and evaluation of treatment combinations or comparisons of the currently approved drugs.

Porto-pulmonary hypertension and right heart failure are relatively prevalent complications of end-stage liver disease and may increase mortality of patients undergoing cadaveric orthotopic liver transplantation. Even with extensive pre-transplant evaluation, these complications are frequently diagnosed unexpectedly in the operating room and transplant procedure may need to be aborted due to high perioperative mortality from both right and left ventricular failure. Living-related liver transplantation is a new surgical alternative to cadaveric liver transplantation, but presence of porto-pulmonary hypertension increases its postoperative mortality as well. Due to inherent elective nature, however, living-related liver transplantation may allow for preoperative hemodynamic optimization and treatment of right ventricular failure. To the authors' knowledge, this is the first reported case of an adult patient with porto-pulmonary hypertension who underwent successful living-related liver transplantation. Favorable transplantation outcome was obtained in this case through good hemodynamic control with long-term IV epoprostenol therapy, preoperative right heart calcification, and perioperative administration of pulmonary vasodilators and inotropic agents.

Primary pulmonary hypertension is a rare disease of the pulmonary vasculature manifested by dyspnea on exertion, syncope, and signs and symptoms of right heart failure. In the absence of adequate treatment, primary pulmonary hypertension has a grave prognosis, with a median survival of 2.8 years. Pulmonary arterial hypertension develops in association with known risk factors and predisposing clinical conditions, and shares many clinical, pathological and therapeutic characteristics with primary pulmonary hypertension. Therapeutic choices in pulmonary arterial hypertension depend on the etiology of the disease, severity of functional impairment and hemodynamic response following acute vasodilator administration during right heart catheterization. Agents currently approved for the specific treatment of pulmonary arterial hypertension are continuous intravenous epoprostenol, subcutaneous treprostinil and oral bosentan. A small group of patients who demonstrate true acute vasoreactivity at right heart catheterization may be chronically treated with oral calcium channel blockers. In addition, most patients with pulmonary hypertension receive conventional treatment, represented by anticoagulants, diuretics, inotropic medication or oxygen supplementation. Treatment of pulmonary arterial hypertension has significantly altered the natural course of the disease, with pronounced symptomatic, functional and survival benefit. Current clinical research focuses on the discovery of new targets of therapy and the use of a combination treatment approach, which will offer hope and valuable insight into the pathogenetic basis of this devastating illness.

We report the first patient with acquired immunodeficiency syndrome in whom molluscum contagiosum and Cryptococcus neoformans were documented in the same cutaneous lesion. While cases of the co-occurrence of two pathologic entities in patients with acquired immunodeficiency syndrome have been reported, namely cytomegalovirus with herpes simplex virus and Kaposi's sarcoma with Histoplasma capsulatum, the findings in this patient are unique. The clinical presentation of cutaneous lesions caused by molluscum contagiosum and by Cryptococcus neoformans is reviewed. The findings in this case are more remarkable still given the often-noted tendency of cutaneous cryptococcosis to masquerade as molluscum contagiosum. It may be that the lesions of molluscum contagiosum create a favorable environment for cryptococcal dissemination. Examination of a biopsy specimen is crucial to the diagnosis of skin lesions, often atypical or deceptive, in immunosuppressed patients.