Referral For Lung Transplantation Research Articles

(159) - The Landscape of Referral for Lung Transplantation in Pulmonary Arterial Hypertension: A Report From the Phar

(159) - The Landscape of Referral for Lung Transplantation in Pulmonary Arterial Hypertension: A Report From the Phar

FEV1Q: a race-neutral approach to assessing lung function.

Forced expiratory volume in 1 s quotient (FEV1Q) is a simple approach to spirometry interpretation that compares measured lung function to a lower boundary. This study evaluated how well FEV1Q predicts survival compared with current interpretation methods and whether race impacts FEV1Q. White and Black adults with complete spirometry and mortality data from the National Health and Nutrition Examination Survey (NHANES) III and the United Network for Organ Sharing (UNOS) database for lung transplant referrals were included. FEV1Q was calculated as FEV1 divided by 0.4 L for females or 0.5 L for males. Cumulative distributions of FEV1 were compared across races. Cox proportional hazards models tested mortality risk from FEV1Q adjusting for age, sex, height, smoking, income and among UNOS individuals, referral diagnosis. Harrell's C-statistics were compared between absolute FEV1, FEV1Q, FEV1/height2, FEV1 z-scores and FEV1 % predicted. Analyses were stratified by race. Among 7182 individuals from NHANES III and 7149 from UNOS, 1907 (27%) and 991 (14%), respectively, were Black. The lower boundary FEV1 values did not differ between Black and White individuals in either population (FEV1 first percentile difference ≤0.01 L; p>0.05). Decreasing FEV1Q was associated with increasing hazard ratio (HR) for mortality (NHANES III HR 1.33 (95% CI 1.28-1.39) and UNOS HR 1.18 (95% CI 1.12-1.23)). The associations were not confounded nor modified by race. Discriminative power was highest for FEV1Q compared with alternative FEV1 approaches in both Black and White individuals. FEV1Q is an intuitive and simple race-neutral approach to interpreting FEV1 that predicts survival better than current alternative methods.

A multidimensional classifier to support lung transplant referral in patients with pulmonary fibrosis

BackgroundLung transplantation remains the sole curative option for patients with idiopathic pulmonary fibrosis (IPF), but donor organs remain scarce, and many eligible patients die prior to transplant. Tools to optimize the timing of transplant referral are urgently needed. MethodsLeast absolute shrinkage and selection operator (LASSO) was applied to clinical and proteomic data generated as part of a prospective cohort study of interstitial lung disease (ILD) to derive clinical, proteomic, and multi-dimensional logit models of near-term death or lung transplant within 18 months of blood draw. Model fitted values were dichotomized at the point of maximal sensitivity and specificity and decision curve analysis used to select the best performing classifier. We then applied this classifier to independent IPF and non-IPF ILD cohorts to determine test performance characteristics. Cohorts were restricted to patients aged ≤72 years with body mass index 18-32 to increase likelihood of transplant eligibility. ResultsIPF derivation, IPF validation and non-IPF ILD validation cohorts consisted of 314, 105 and 295 patients, respectively. A multi-dimensional model comprising two clinical variables and 20 proteins outperformed stand-alone clinical and proteomic models. Following dichotomization, the multi-dimensional classifier predicted near-term outcome with 70% sensitivity and 92% specificity in the IPF validation cohort and 70% sensitivity and 80% specificity in the non-IPF ILD validation cohort. ConclusionsA multi-dimensional classifier of near-term outcome accurately discriminated this endpoint with good test performance across independent IPF and non-IPF ILD cohorts. These findings support refinement and prospective validation of this classifier in transplant eligible individuals.

Use of Race-Specific Equations in Pulmonary Function Tests Impedes Potential Eligibility for Care and Treatment of Pulmonary Fibrosis.

The use of race-specific reference values to evaluate pulmonary function has long been embedded into clinical practice; however, there is a growing consensus that this practice may be inappropriate and that use of race-neutral equations should be adopted to improve access to healthcare. To evaluate if using race-neutral equations to assess percent predicted forced vital capacity (FVC%pred) impacts eligibility for clinical trials, antifibrotic therapy and referral for lung transplantation in Black, Hispanic/Latino, and White patients with interstitial lung disease (ILD) Methods: FVC%pred values for patients from the Pulmonary Fibrosis Foundation Patient Registry were calculated using race-specific (Hankinson 1999), race-agnostic (Global Lung Function Initiative [GLI]-2012), and race-neutral (GLI-2022 or GLI-Global) equations. Eligibility for ILD clinical trials (FVC%pred >45% and <90%), antifibrotic therapy (FVC%pred >55% and <82%), and lung transplantation referral (FVC%pred <70%) using GLI-2022 and GLI-2012 were compared with that of Hankinson 1999. Baseline characteristics were available for 1882 patients (Black, n=104; Hispanic/Latino, n=103; White, n=1675), and outcomes were evaluated in 1531 patients with FVC%pred within ±90 days of Registry enrollment (Black, n=78; Hispanic/Latino, n=72; White, n=1381). Black patients were younger at consent and more likely to be female than Hispanic/Latino or White patients. Compared with GLI-2022, Hankinson's 1999 criteria misclassified 22% of Black patients, 14% of Hispanic/Latino patients, and 12% of White patients for ILD clinical trial eligibility; misclassified 21% of Black patients, 17% of Hispanic/Latino patients, and 19% of White patients for antifibrotic therapy eligibility; and misclassified 6% of Black patients, 14% of Hispanic/Latino patients, and 12% of White patients for lung transplantation referral. Similar trends were observed when comparing GLI-2012 with Hankinson 1999. Misclassification of patients for critical interventions is highly prevalent when using Hankinson's 1999 criteria and highlights the need to consider adopting the race-neutral GLI-2022 for enhanced accuracy and more equitable representation in pulmonary healthcare. Our results make a compelling case for re-evaluating the use of race as a physiological variable and emphasize the pressing need for continuous innovation to ensure equal and optimal care for all patients, regardless of their race or ethnicity. NCT02758808 Primary source of funding: This analysis was sponsored by F. Hoffmann-La Roche, Ltd./Genentech, Inc.

The impact of elexacaftor/tezacaftor/ivacaftor therapy on the pulmonary management of adults with cystic fibrosis: An expert-based Delphi consensus

BackgroundThe advent of elexacaftor/tezacaftor/ivacaftor (ETI) resulted in unprecedented clinical benefits for eligible adults with CF. As a result, the question of whether chronic treatments can be safely stopped or adapted to this new situation has become a matter of great interest. Our objective was to derive a consensus among Italian experts on the impact of ETI on the current clinical management of CF lung disease. MethodsFrom December 2021 to April 2022 a panel of Italian experts endorsed by the national CF scientific society derived and graded a set of statements on the pulmonary management of adults with cystic fibrosis through a modified Delphi methodology. ResultsThe panel produced 13 statements exploring possible modifications in the fields of inhaled antibiotics and mucoactives; airway clearance and physical activity; chronic macrolides and bronchodilators; and lung transplant referral. The areas that the experts considered most urgent to explore were the impact of ETI on the role of inhaled antibiotics and lung transplant. ConclusionsThe list of priorities that emerged from this study could be useful to guide and inform clinical research on the most urgent area of impact of ETI on CF lung disease and its clinical management.

Timing of Lung Transplant Referral in Patients with Severe COVID-19 Lung Injury Supported by ECMO.

Severe respiratory failure caused by COVID-19 often requires mechanical ventilation, including extracorporeal membrane oxygenation (ECMO). In rare cases, lung transplantation (LTx) may be considered as a last resort. However, uncertainties remain about patient selection and optimal timing for referral and listing. This retrospective study analyzed patients with severe COVID-19 who were supported by veno-venous ECMO and listed for LTx between July 2020 and June 2022. Out of the 20 patients in the study population, four who underwent LTx were excluded. The clinical characteristics of the remaining 16 patients were compared, including nine who recovered and seven who died while awaiting LTx. The median duration from hospitalization to listing was 85.5 days, and the median duration on the waitlist was 25.5 days. Younger age was significantly associated with a higher likelihood of recovery without LTx after a median of 59 days on ECMO, compared to those who died at a median of 99 days. In patients with severe COVID-19-induced lung damage supported by ECMO, referral to LTx should be delayed for 8-10 weeks after ECMO initiation, particularly for younger patients who have a higher probability of spontaneous recovery and may not require LTx.

Prognostic value of follow-up vasoreactivity test in pulmonary arterial hypertension

BackgroundAcute vasoreactivity test with inhaled nitric oxide (NO) is performed during diagnostic right heart catheterization (RHC) to identify patients with pulmonary arterial hypertension (PAH) who respond to calcium channel blockers. Our purpose was to investigate the prognostic importance of follow-up vasoreactivity test after treatment. MethodsWe retrospectively analyzed 36 PAH patients (mean age, 47 years; 61 % treatment-naïve), who underwent diagnostic and follow-up RHC and vasoreactivity tests at our center. The primary outcome was all-cause mortality. ResultsThe median time between baseline and follow-up RHC was 9.7 months. Absolute change in mean pulmonary arterial pressure (ΔmPAP) during NO challenge was less pronounced after treatment, but there was great variability among patients. Overall cohort was dichotomized into two groups: preserved vasoreactivity (ΔmPAP ≤ −1 mmHg) and less vasoreactivity (ΔmPAP ≥0 mmHg) at follow-up RHC. Less vasoreactivity group had higher usage rate of endothelin receptor antagonists and parenteral prostacyclin analogues. During a median observation period of 6.3 years after follow-up RHC, 7 patients died, of which 6 showed less vasoreactivity at follow-up. Absolute ΔmPAP ≥0 at follow-up RHC was associated with all-cause mortality in univariable Cox regression analysis (hazard ratio, 8.728; 95 % confidence interval, 1.045–72.887; p = 0.045), whereas other hemodynamic parameters were not. Absolute ΔmPAP ≥0 at follow-up RHC was associated with all-cause mortality in multivariable Cox analysis adjusted for age and known PAH prognostic factors (HR, 12.814; 95 % CI, 1.088–150.891; p = 0.043). Kaplan-Meier survival analysis revealed a significantly worse survival of less vasoreactivity group compared to preserved vasoreactivity group (log-rank test, p = 0.016). ConclusionsFollow-up vasoreactivity test after treatment could contribute to the detection of high-risk subgroups who might need careful monitoring and referral for lung transplantation.

COUNTERPOINT: Pulmonary Vascular Resistance 2.0— Shedding Light or Casting Shadows?

COUNTERPOINT: Pulmonary Vascular Resistance 2.0— Shedding Light or Casting Shadows?

Predictors of lung transplant waitlist mortality for sarcoidosis

RationaleUnlike in other chronic lung diseases, criteria for lung transplant referral in sarcoidosis is not well-established. Waitlist mortality may offer clues in identifying clinical factors that warrant early referral. We aim to identify predictors for transplant waitlist mortality to improve referral criteria for patients with sarcoidosis. MethodsWe conducted a retrospective analysis of 1034 sarcoidosis patients listed for lung transplantation from May 2005 to May 2019 in the Scientific Registry of Transplant Recipients (SRTR) database. All patients were listed after the establishment of the Lung Allocation Score (LAS). We compared patients who died on the transplant waitlist to those who survived to transplantation. Potential predictors of waitlist mortality were assessed utilizing univariate and multivariate analysis performed via logistic regression modeling. ResultsOf 1034 candidates listed after LAS implementation, 704 were transplanted and 110 died on the waitlist. Significant predictors of waitlist mortality on multivariate analysis include female gender (OR 2.445; 95% CI 1.513–3.951; p = 0.0003) and severe pulmonary hypertension (OR 1.619; 95% CI 1.067–2.457; p = 0.0236). Taller minimum donor height (OR 0.606; 95% CI 0.379–0.969; p = 0.0365) and blood type B (OR 0.524; 95% CI 0.281–0.975 p = 0.0415) were associated with decreased likelihood of death on the waitlist. ConclusionAmong patients with sarcoidosis on the lung transplant waitlist, taller minimum donor height and blood type B were found to be protective factors against death on the waitlist. Female gender and severe pulmonary hypertension have a higher likelihood of death and earlier referral for transplantation in patients with these characteristics should be considered.

A Survey of Referral Rates for Lung Transplant Assessment in Patients with Interstitial Lung Disease Meeting Pulmonary Function Criteria

<h3>Purpose</h3> Progressive interstitial lung diseases (ILD) unresponsive to medical therapy often require referral for lung transplantation (LTx) assessment, as this can prolong quality-of-life and survival. Ideal timing for referral for LTx remains challenging, with late-referral associated with significant morbidity and mortality. Among other criteria, ILD patients should be considered for LTx if forced vital capacity (FVC) is less than 80% or diffusion capacity for carbon monoxide (DLCO) is less than 40%. However, data on referral rates are lacking. The objective of this study was to evaluate referral rates and identify possible barriers associated with non-referral of these patients. <h3>Methods</h3> A single-center, retrospective cohort study of ILD patients who had pulmonary function test (PFT) performed between January 1, 2014, and January 1, 2020. Patients with FVC<80% or a DLCO<40% were included in the study. Patients with absolute contraindications to LTx were excluded. Referral rates were computed, and patients' characteristics were compared between referred and non-referred subjects. Potential predictors of survival were assessed using Kaplan-Meier and Cox proportional hazards models. <h3>Results</h3> Out of 114 ILD patients who met PFT criteria for referral to LTx, only 35 were referred (30.7%), and seven have undergone LTx. Median time from PFT to referral for assessment was 255 days [IQR 35.5-1077.5], while median time from referral to LTx was 88.5 days [IQR 59.5-142.8]. Referred patients were younger [58.4 vs. 66.8, P=0.003], had lower FVC [47 vs. 62.1, P<0.001] and DLCO [24.2 vs. 36.5, P<0.001], and a higher rate of pulmonary hypertension [67.7% vs. 43.8%, P=0.04]. Lower FVC and higher GAP index (a clinical prediction tool used to estimate prognosis) were associated with mortality [HR=0.96, CI 0.95-0.98 P=0.001 and HR=1.97, CI 1.45-2.67 P<0.001 respectively]. There was no difference in mortality between the two groups. <h3>Conclusion</h3> There appears to be under-referral of patients with ILD who are potential LTx candidates. Late referral is associated with markers of severe disease. These patients have higher mortality rates and often miss the window of opportunity for LTx, and should be referred for assessment as early as possible. Further study is suggested to replicate these findings in other transplant centers and internationally.

Utilization of risk assessment tools in management of PAH: A PAH provider survey.

Pulmonary arterial hypertension (PAH) is a chronically progressive fatal disease. A goal‐oriented approach to achieve low risk status has been associated with improved survival. A variety of risk stratification tools are available, but use is low. We conducted a survey to assess potential reasons for under‐utilization. We conducted a survey‐based study of global PAH disease specialists with a goal of assessing risk assessment utilization and identifying modifiable barriers to use. The survey was designed by the American College of Chest Physicians’ Pulmonary Vascular Diseases (PVD) NetWork. Respondents were global members of the PVD NetWork and Pulmonary Hypertension Association. Survey invitations were sent electronically to all members. Participation was anonymous and no provider or patient level data was collected. Participants from four countries responded with the majority (84%) being from the United States. Our survey found suboptimal use of any risk stratification tool with 71/112 (63%) reporting use. A total of 85% of the respondents had more than 5 years of experience in managing PAH. REVEAL 2.0 and European Society of Cardiology/European Respiratory Society risk tools were the most commonly used. A total of 44 (65%) surveyed felt that use of risk tools led to change in PAH therapies. Only 6 (9%) felt they prompted additional testing or changed the frequency of follow‐up. A total of 5 (7%) reported they prompted goals of care/palliative care discussions and 2 (3%) that they triggered lung transplant referral. The vast majority indicated that incorporation of risk tools into electronic medical records (EMR) would improve utilization. PAH risk assessment tools remain under‐utilized. Most respondents were experienced PAH clinicians. More than one‐third were not routinely using risk tools. Most felt that risk tools led to PAH therapy changes but few reported impacts on other aspects of care. The most commonly identified barriers to use were time constraints and lack of integration with EMR.

Pulmonary Capillary Hemangiomatosis and Refractory Hypoxemia During PH Therapy

<h3>Introduction</h3> Pulmonary capillary hemangiomatosis (PCH), a rare cause of pulmonary hypertension (PH), presents clinically with right heart dysfunction and may be indistinguishable from typical pulmonary arterial hypertension. Vasodilator therapy exacerbates hypoxemia in PCH despite improvement in heart failure symptoms. Lung transplant is the only definitive therapy. We present the case of a patient succumbing to progressive hypoxemia on PH therapy during lung transplant evaluation. Autopsy revealed PCH. <h3>Case Report</h3> A 42-year-old female with PH due to undifferentiated connective tissue disease presented with acute respiratory distress. She was diagnosed 2 years prior and initially treated with macitentan, tadalafil and diuretics with clinical improvement; however, worsening shortness of breath and new finding of hypoxemia developed after 1 year. Imaging revealed faint patchy nodular opacities initially attributed to UCTD-associated pneumonitis. Steroids and mycophenolate failed to achieve radiographic improvement. Repeat right heart catheterization showed continued elevation in pulmonary artery pressure and stably reduced cardiac output. Subcutaneous infusion of Treprostinil was initiated 2 months prior to admission, which worsened hypoxemia. Clinical diagnosis of pulmonary veno-occlusive disease (PVOD) vs PCH was suspected and lung transplant evaluation initiated. On admission, patient presented with syncope and was found to have refractory hypoxemia despite high-flow oxygen supplementation. Cardiogenic shock and hypoxemic respiratory failure ensued, resulting in hepatic and renal failure, leading to cardiac arrest and unsuccessful resuscitation. Autopsy showed PCH. <h3>Summary</h3> Refractory hypoxemia during up-titration of vasodilator therapy for PH should prompt clinical suspicion for atypical causes of PH such as PCH. Early lung transplant referral is essential if PCH/PVOD is suspected given its poor prognosis and potentially harmful responses associated with traditional PH therapy.

CFTR Modulator Therapy and Its Impact on Lung Transplantation in Cystic Fibrosis.

Cystic fibrosis (CF) is the most common autosomal recessive disorder in Caucasian people and is caused by mutations in the gene encoding for the CF transmembrane conductance regulator (CFTR) protein. It is a multisystem disorder; however, CF lung disease causes most of its morbidity and mortality. Although survival for CF has improved over time due to a multifaceted symptomatic management approach, CF remains a life-limiting disease. For individuals with progressive advanced CF lung disease (ACFLD), lung transplantation is considered the ultimate treatment option if compatible with goals of care. Since 2012, newer drugs, called CFTR modulators, have gradually become available, revolutionizing CF care, as these small-molecule drugs target the underlying defect in CF that causes decreased CFTR protein synthesis, function, or stability. Because of their extremely high efficacy and overall respectable tolerability, CFTR modulator drugs have already proven to have a substantial positive impact on the lives of individuals with CF. Individuals with ACFLD have generally been excluded from initial clinical trials. Now, however, these drugs are being used in clinical practice in selected individuals with ACFLD, showing promising results, although randomized controlled trial data for CFTR modulators in this subgroup of patients are lacking. Such data need to be gathered, ideally in randomized controlled trials including patients with ACFLD. Furthermore, the efficacy and tolerability of the newer modulator therapies in individuals with ACFLD need to be monitored, and their impact on lung disease progression and the need for lung transplantation as the ultimate therapy call for an objective evaluation in larger patient cohorts. As of today, guidelines for referral and listing of lung transplant candidates with CF have not incorporated the status of the new CFTR modulator therapies in the referral and listing process. The purpose of this review article, therefore, is threefold: first, to describe the effects of new therapies, with a focus on the subgroup of individuals with ACFLD; second, to provide an update on the recent outcomes after lung transplantation for individuals with CF; and third, to discuss the referral, evaluation, and timing for lung transplantation as the ultimate therapeutic option in view of the new treatments available in CF.

Lung Transplantation as Acute ‘Rescue’ Therapy

<h3>Purpose</h3> Lung transplantation (LT) is an effective treatment of end-stage lung disease. Despite recommendations for optimal referral for LT, some patients still present in distress seeking emergent rescue LT. Traditionally, these patients have been considered too ill to benefit from LT. Clinical outcomes for this population remain uncertain. Here, we compare outcomes between emergently transplanted patients with standard LT recipients. <h3>Methods</h3> A retrospective review of all LT recipients at our center between January 1, 2015 and December 31, 2019 was performed. 204 patients underwent standard evaluation and ten patients were referred for and underwent rescue LT. The rescue cohort consisted of patients not previously known to the transplant team but were urgently evaluated and listed for LT, after presenting in respiratory distress (oxygen needs ≥ 60% FiO₂ or need for mechanical ventilation +/- ECMO support). <h3>Results</h3> Mean LAS at LT was 81.99 in the rescue group (RG) and 39.19 in the standard group (SG) (student's t test: p<0.0001). One year survival in the RG was 90% and 88.23% in the SG (Kaplan-Meier p=0.89). Median ICU length of stay (LOS) of the RG and SG were 14 and 5 days respectively (Mann-Whitney U test p=0.4). The median index LOS was 31 days in the RG and 19 days in the SG (Mann-Whitney U test p=0.50). The total number of hospital days in the first post-LT year was a median of 46.5 days in the RG and 40 in the SG (Mann-Whitney U test p=0.50). Of surviving recipients, 55.6% of RG and 14.1% of SG patients required dialysis (HD) at hospital discharge (χ ²=10.97; p=0.006). Of surviving recipients, 33.3% of the RG and 7.2% in the SG were receiving HD at one year (χ ²=7.54; p=0.03). 44.4% of the RG were discharged to home vs. 83.3% of the SG (χ ²=6.28; p=0.03). <h3>Conclusion</h3> Compared to patients referred in accordance with published guidelines, the RG experienced no worse 1 year post-LT survival but did have increased index ICU stays, HD needs, and need for post-hospitalization inpatient care. Given these observances, our results support continuing to offer LT as a rescue therapy.

P077 Clinical factors affecting timing of referral for lung transplantation for people with cystic fibrosis: a national comparison of opinions between adult cystic fibrosis and transplant centres

P077 Clinical factors affecting timing of referral for lung transplantation for people with cystic fibrosis: a national comparison of opinions between adult cystic fibrosis and transplant centres

P076 Using the Cystic Fibrosis Foundation consensus guidelines to assess the timing of lung transplant referral for individuals with cystic fibrosis in a single large adult UK centre: do they help?

P076 Using the Cystic Fibrosis Foundation consensus guidelines to assess the timing of lung transplant referral for individuals with cystic fibrosis in a single large adult UK centre: do they help?

Lung transplant referrals for individuals with cystic fibrosis: A pediatric perspective on the cystic fibrosis foundation consensus guidelines.

Lung transplant referral guidelines for individuals with cystic fibrosis (CF) were published recently. Most of the recommendations focus on the specific indications and barriers to transplantation in adults with CF. Although the number of children with CF and end-stage lung disease continues to decrease, the specific issues related to pediatric patients merit further elucidation. We address each recommendation from the recent publication with a pediatric perspective. Furthermore, we note some significant differences between the practice and policy related to lung transplantation between Canada and the United States.

PULMONARY CAPILLARY HEMANGIOMATOSIS: A RARE CASE OF STABLE DISEASE WITHOUT TREATMENT

SESSION TITLE: Medical Student/Resident Pulmonary Vascular Disease Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Pulmonary capillary hemangiomatosis (PCH) is a rare cause of primary pulmonary hypertension caused by extensive proliferation of pulmonary capillaries within parenchymal, vascular, and bronchial tissue. Typically, PCH progresses rapidly with a median time to lung transplantation of two years post diagnosis. To our knowledge, we present the longest case of biopsy proven PCH that has been stable without treatment for seven years. CASE PRESENTATION: A healthy 23-year old non-smoking male presented to the emergency department (ED) with a 2-week history of hemoptysis associated with intermittent left-sided pleuritic chest pain, fatigue, and dyspnea. His physical exam, routine lab work, and chest X-ray were unremarkable. He was discharged home and prescribed clarithromycin for suspected bronchitis. He returned to the ED months later with ongoing symptoms and was referred for urgent outpatient consultation to pulmonology. Bronchoscopy was performed with no blood on bronchoalveolar lavage analysis. Further serum rheumatologic and vasculitis panels were negative. A non-contrast computed tomography (CT) scan of his chest revealed subtle bilateral upper lung predominant centrilobular ground-glass opacities. (Figure 1) With worsening hemoptysis, he returned to the ED six months later. A CT Chest angiogram was performed and showed progressive bilateral airspace opacities. (Figure 2) The patient was referred to Thoracic Surgery for lung biopsy. This demonstrated septal proliferation of capillaries consistent with PCH without evidence of vasculitis, venous occlusion or microorganisms. Immunofluorescence staining was also negative. (Figure 3) The patient remains stable with no signs of progression or pulmonary hypertension on echocardiogram. His enhanced CT and pulmonary function tests remain normal seven years from his initial presentation. DISCUSSION: The typical presentation of PCH includes progressive dyspnea, cough with or without hemoptysis, chest pain, and fatigue. (1) Chest X-ray can be normal or progress to demonstrate bilateral opacities in keeping with diffuse alveolar hemorrhage (DAH). CT chest often shows diffuse centrilobular ground-glass opacities. (1) Bronchoscopy may not reveal erythrocytes if the patient is not actively bleeding. Diagnosis of PCH is essential, as vasodilators used in pulmonary hypertension can cause hemodynamic instability in PCH. (2) Interestingly, an autopsy case series found incidental cases of PCH in patients who were asymptomatic during life. (3) This raises the possibility that subclinical cases of PCH may exist which do not require urgent referral for lung transplantation. CONCLUSIONS: This case represents the longest active case of PCH after confirmation with biopsy without treatment. Re-evaluating the expected prognosis for individuals diagnosed with PCH is important, as median survival may be longer than currently reported in the literature. Reference #1: O'keefe MC, and Post MD. Pulmonary Capillary Hemangiomatosis: A Rare Cause of Pulmonary Hypertension. Archives of Pathology & Laboratory Medicine, 2015;139(2):274–277. Reference #2: Almagro P, Julia J, Sanjuame M, Gonalez G, Casalots J, Heredia JL, et al. Pulmonary Capillary Hemangiomatosis Associated with Primary Pulmonary Hypertension. Medicine. 2002;81(6):417–24. Reference #3: Havlik DM, Massey LW, Williams WL, and Crooks LA. Pulmonary Capillary Hemangiomatosis- like foci: an autopsy study of 8 cases. Anatomic Pathology. 2000;113(5):665-662. DISCLOSURES: No relevant relationships by Meredith Chiasson, source=Web Response No relevant relationships by Jasper Johar, source=Web Response No relevant relationships by Daria Manos, source=Web Response No relevant relationships by Matthew Nunn, source=Web Response

Risk stratification in PAH.

[No abstract. Showing first paragraph of article]Pulmonary arterial hypertension (PAH) is a chronic disease of the pulmonary vasculature characterized by progressive narrowing of the pulmonary arteries leading to increased pulmonary vascular resistance, right heart failure, and ultimately premature death.There has been a significant improvement in the available medical therapeutic options in this field that have impacted the short-term survival and morbidity in these patients. However, the median survival post-diagnosis remains unacceptable at 7 years.Physicians’ ability to predict PAH disease progression and risk allows them to determine the patient’s prognosis, make informed adjustments to therapy, and monitor his or her response to therapy . If widely adopted, risk prediction can enhance the consistency of treatment approaches and improve the timeliness of referral for lung transplantation. This approach should lead optimal, directed care that ultimately reduces morbidity and improves mortality in patients with PAH.

Lung transplant referral practice patterns: a survey of cystic fibrosis physicians and general pulmonologists

BackgroundMany individuals with cystic fibrosis (CF) die from respiratory failure without referral for lung transplant. Physician practices that may expedite, delay, or preclude referral, are poorly understood.MethodsTwo parallel, web-based surveys focusing on lung transplant referral triggers and barriers, as well as pre-referral evaluation, were emailed to pulmonologists practicing in the New England region. One questionnaire was sent to CF providers (n = 61), and the second to general pulmonary providers practicing at the same institutions (n = 61).ResultsThere were 43 (70%) responses to the CF provider survey, and 25 (41%) responses to the general pulmonary (‘non-CF’) provider survey. Primary reasons for CF providers to refer their patients included: rapidly declining lung function (91%) and a forced expiratory volume in 1 s (FEV1) below 30% predicted (74%). The greatest barriers to referral for both CF and non-CF providers included active tobacco use (65 and 96%, respectively, would not refer), and active alcohol or other substance use or dependence (63 and 80%). Furthermore, up to 42% of CF providers would potentially delay their referral if triple-combination therapy or other promising new, disease-specific therapy were anticipated. In general, non-CF providers perform a more robust pre-referral medical work-up, while CF providers complete a psychosocial evaluation in higher numbers. Across both groups, communication with lung transplant programs was reported to be inadequate.ConclusionsPhysician-level barriers to timely lung transplant referral exist and need to be addressed. Enhanced communication between lung transplant programs and pulmonary providers may reduce these barriers.