Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension: A Systematic Review and Meta-analysis.

PURPOSE: To perform a systematic review and meta-analysis assessing the safety and efficacy of balloon pulmonary angioplasty (BPA) in the treatment of chronic thromboembolic pulmonary hypertension (CTEPH). MATERIALS AND METHODS: Systematic literature searches were performed from inception to June 2022 to identify studies assessing BPA for CTEPH. Outcomes of interest included the following functional and hemodynamic measures: (a) six-minute walk distance (6MWD), (b) New York Heart Association (NYHA) status, (c) World Health Organization (WHO)-Functional Class status, (d) cardiac index (CI), (e) mean pulmonary artery pressure (mPAP), (f) mean right atrial pressure (mRAP), and (g) pulmonary vascular resistance (PVR). Subgroup analysis was also performed for BPA in post-pulmonary endarterectomy (PEA) patients. All reported BPA-related complications were also recorded. Forty unique studies with a total of 1763 patients were identified for meta-analysis. RESULTS: All functional and hemodynamic parameters improved significantly following BPA; 6MWD increased 70 m (95% CI 58-82; P < 0.001), NYHA class improved by - 0.9 classes (95% CI - 1.0 to - 0.8; P < 0.001), WHO-FC class improved by - 1 classes ((95% CI - 1.2 to - 0.9; P < 0.001), CI increased 0.26 L/min/m2 (95% CI 0.17-0.35; P < 0.001), mPAP decreased - 13.2 mmHg (95% CI - 14.7 to - 11.8; P < 0.001), mRAP decreased - 2.2 mmHg (95% CI - 2.8 to - 1.6; P < 0.001), and PVR decreased - 311 dyne/cm/s-5 (95% CI - 350 to - 271; P < 0.001). Meta-analysis of patients who underwent BPA for persistent pulmonary hypertension post-PEA demonstrated significant improvements in 6MWD, WHO-FC, PVR and mPAP. Most common complications included lung injury (8.16%), hemoptysis (7.07%) and vessel injury (5.05%). CONCLUSION: BPA represents a safe and effective treatment option for select individuals with CTEPH with significant improvements in hemodynamic parameters, improved exercise tolerance and a relatively low risk of major complications.

The role of exercise right heart catheterization to guide pulmonary hypertension therapy in older adults.

The spectrum of patients referred for suspected pulmonary arterial hypertension (PAH) includes a population with clinical features suggestive of pulmonary hypertension due to left heart disease (PH-LHD). Even after right heart catheterization (RHC) performed at rest, it can be a challenge to identify patients who will clearly benefit from PAH drug therapy. Therefore, the objective of this study was to evaluate the role of exercise RHC to influence decisions regarding prescription of PAH drug therapy in this population. A retrospective cohort study was conducted of older adults with risk factors for PH-LHD and suspected PH referred for exercise RHC. One year follow-up was conducted to record clinical outcomes, all changes in PAH drug therapy, and changes in patient-reported quality of life. The final cohort included 61 patients, mean age of 69 ± 10; 44% and 34% had a history of coronary artery disease and atrial fibrillation respectively. Exercise changed the proportional breakdown of hemodynamic diagnoses from 36% No PH, 44% PAH, and 20% PH-LHD at rest to 15% No PH, 36% PAH, and 49% PH-LHD. Although a significant proportion of patients were reclassified as PH-LHD, there was an overall increase in the proportion of patients receiving PAH drug therapy, particularly for those with PAH confirmed by exercise RHC. A total of 11 PAH drug prescriptions were employed before exercise RHC increasing to 24 after (p = 0.002). Patients receiving PAH therapy demonstrated significant improvement in self-reported quality of life. Exercise RHC appeared to influence selection of PAH drug therapy.

Pulmonary endarterectomy in severe chronic thromboembolic pulmonary hypertension: the Toronto experience.

Background: Pulmonary endarterectomy (PEA) in severe chronic thromboembolic pulmonary hypertension (CTEPH) is associated with higher risks. However, recent evidence suggests that these risks may be mitigated with the use of extracorporeal membrane oxygenation (ECMO). Methods: We performed a retrospective analysis of 401 consecutive patients undergoing PEA at the Toronto General Hospital between August 2005 and March 2020. Patients with severe CTEPH defined by pulmonary vascular resistance (PVR) >1,000 dynes.s.cm-5 at the time of diagnosis were compared to those with PVR <1,000 dynes.s.cm-5. Results: The New York Heart Association (NYHA) functional class, brain natriuretic peptide (BNP) and 6-minute walk distance were worse in patients with PVR >1,000 dynes.s.cm-5. A greater proportion of patients with PVR >1,000 dynes.s.cm-5 was treated with targeted pulmonary hypertension (PH) medical therapy (38% vs. 18%, P<0.001) and initiated on inotropic support (7% vs. 0.3%, P<0.001) before PEA. Since 2014, the ECMO utilization rate increased in patients with PVR >1,000 dynes.s.cm-5 compared to those with PVR <1,000 dynes.s.cm-5 (18% vs. 3.1%, P<0.001). The hospital mortality in patients with PVR >1,000 dynes.s.cm-5 decreased from 10.3% in 2005-2013 to 1.6% in 2014-2020 (P=0.05), while the hospital mortality in patients with PVR <1,000 dynes.s.cm-5 remained stable (1.2% in 2005-2013 vs. 2.7% in 2014-2020, P=0.4). The overall survival reached 84% at 10 years in patients with PVR >1,000 dynes.s.cm-5 compared to 78% in patients with PVR <1,000 dynes.s.cm-5 (P=0.7). Conclusions: The early and long-term results of PEA in patients with severe CTEPH are excellent despite greater postoperative risks. ECMO as a bridge to recovery after PEA can be useful in patients with severe CTEPH.

Outcome after pulmonary endarterectomy for segmental chronic thromboembolic pulmonary hypertension.

OBJECTIVE: Determine the long-term outcome and need for additional therapy after pulmonary endarterectomy (PEA) for segmental chronic thromboembolic pulmonary hypertension. METHODS: Retrospective analysis of a prospective cohort of 401 consecutive Canadian patients undergoing PEA between August 2005 and March 2020 in Toronto. The outcome of segmental disease defined as Jamieson type 3 was compared with more proximal disease defined as Jamieson type 1 and 2. The cohort was divided into 3 intervals to analyze the trend over time: 2005-2010, 2011-2015, and 2016-2020. RESULTS: Type 3 disease accounted for 41% of patients undergoing PEA durig 2016-2020 compared with 7% in 2006-2010. Total pulmonary vascular resistance improved by 505 ± 485 dynes/s/cm-5 in type 3 disease and by 593 ± 452 dynes/s/cm-5 in type 1 or 2 disease (P = .07). Mortality after PEA was similar between type 3 and type 1 and 2 disease at 30-days (2.8% vs 2.3%; P = .8) and at 1 year (7.7% vs 5.5%; P = .4). At 5 years, the survival was lower in type 3 disease (80% vs 91% in type 1 or 2 disease; P = .002). Type 3 disease was an independent predictor for the initiation of pulmonary hypertension-targeted medical therapy after PEA with a cumulative incidence of 38% at 10 years compared with 20% in type 1 and 2 disease (P < .0001). Post-PEA balloon pulmonary angioplasty was predominantly performed in type 3 disease (8% vs 1% in more type 1 or 2 disease; P = .0002). CONCLUSIONS: PEA achieved excellent early and long-term results in segmental chronic thromboembolic pulmonary hypertension. However, patients with segmental disease are at increased risk of requiring additional therapy after PEA and should be carefully monitored.

Central venoarterial extracorporeal membrane oxygenation as a bridge to recovery after pulmonary endarterectomy in patients with decompensated right heart failure.

INTRODUCTION: Patients with chronic thromboembolic pulmonary hypertension (CTEPH) and decompensated right heart failure (DRHF) have worse outcomes after pulmonary endarterectomy (PEA). We reviewed the role of central veno-arterial extracorporeal membrane oxygenation (VA-ECMO) as a bridge to recovery after PEA in these patients. METHODS: Of 388 consecutive patients undergoing PEA, 40 (10.3%) were admitted with DRHF before PEA. This group was compared to the remaining 348 patients undergoing PEA (elective group). We also compared 2 periods: 2005-2013 (n = 120) and 2014-2019 (n = 268) after which early central VA-ECMO was introduced as a strategy to manage difficulty weaning from cardiopulmonary bypass (CPB). RESULTS: The proportion of patients with DRHF remained similar between the first and second period (13% vs 9%, p = .2). The number of VA-ECMO bridge to recovery increased from 0.8% in 2005-2013 to 6.3% in 2014-2019 (p = .02). In the second period, 29% of DRHF patients were transitioned intraoperatively from CPB to central VA-ECMO for a median duration of 3 (2-7) days. After the introduction of central VA-ECMO as a bridge to recovery, the hospital mortality in patients with DRHF dropped from 31% in 2005-2013 to 4% in 2014-2019 (p = .03). In the long-term, the functional recovery and survival after discharged from hospital was similar between the DRHF group and the elective group. However, at 5 years, DRHF patients more frequently required PH targeted medical therapy (45% vs 20% in the elective group, p = .002). CONCLUSIONS: Central VA-ECMO as a bridge to recovery is an important treatment strategy that can decrease hospital mortality in patients with DRHF and lead to excellent long-term outcome.

Clinical Implications of Body Composition and Exercise Capacity Following Pulmonary Endarterectomy.

BACKGROUND: Pulmonary endarterectomy (PEA) is a curative procedure for patients with chronic thromboembolic pulmonary hypertension. Body composition and exercise capacity have been associated with adverse outcomes in patients undergoing cardiothoracic operations, but their significance with PEA is unclear. We evaluated the association of body composition and 6-minute walk distance (6MWD) with disease severity, hospital length of stay, discharge disposition, and postoperative functional recovery. METHODS: This was a retrospective, single-center cohort study of patients who underwent PEA (January 2014-December 2017). Body composition (skeletal muscle mass and adiposity cross-sectional area) was quantified using thoracic computed tomography with sliceOmatic (TomoVision, Magog, QC, Canada) software. Body mass index was calculated. Association of body composition measures and 6MWD with clinical outcomes was evaluated using multivariable regression models. RESULTS: The study included 127 patients (42% men), aged 58 ± 14 years; body mass index was 31 ± 7 kg/m2 and 6MWD was 361 ± 165 m). Muscle and 6MWD were associated with disease severity measures. Of those surviving hospitalization (n = 125), a greater 6MWD was associated with a shorter hospital stay (1.9 median days per 100 m; p < .001) and higher likelihood of being discharged directly home from hospital (odds ratio, 2.1 per 100 m; P = .004), independent of age, sex, and body mass index. Those with a lower preoperative 6MWD (per 100 m) had a greater increase in their postoperative 6MWD (52 m; P < .0001), independent of age, sex, and body mass index. Body composition measures were not associated with hospital outcomes or exercise capacity in the first year postoperatively. CONCLUSIONS: Exercise capacity was a more prognostic marker of PEA outcomes compared with body composition. Future research is needed to explore pre-PEA rehabilitation strategies.

Pathologically confirmed diffuse alveolar haemorrhage in lymphangioleiomyomatosis.

A 40-year-old woman was referred to pulmonology after presenting with dyspnoea and self-limiting haemoptysis. Chest CT revealed diffuse ground glass opacities and small thin-walled cysts. Bronchoalveolar lavage cultures were negative and cytology revealed haemosiderin-laden macrophages. Transthoracic echocardiogram was normal. Connective tissue disease and vasculitis work-up were negative. Vascular endothelial growth factor-D level was indeterminate. Lung function was normal. She underwent video-assisted thoracoscopic lung biopsy. In addition to findings consistent with lymphangioleiomyomatosis, histopathological examination identified haemosiderosis without capillaritis, confirming a diagnosis of diffuse alveolar haemorrhage in the context of the associated clinical and radiographic features. Follow-up imaging after 5 months showed resolution of the diffuse ground glass opacities. Pharmacotherapy with sirolimus was not initiated due to absence of deterioration in pulmonary function. Diffuse alveolar haemorrhage in patients with lymphangioleiomyomatosis is a rare but important presentation. The few previously reported cases progressed to respiratory failure requiring mechanical ventilation.

Importance of computed tomography in defining segmental disease in chronic thromboembolic pulmonary hypertension.

BACKGROUND: Radiological assessment of patients with chronic thromboembolic pulmonary hypertension (CTEPH) is critical to decide whether patients should be treated with pulmonary endarterectomy (PEA). Although computed tomography pulmonary angiography (CTPA) is increasingly used for decision making in CTEPH, the value of CTPA to predict surgical findings and outcome has never been explored. METHODS: We retrospectively reviewed 100 consecutive patients with high-quality CTPA undergoing PEA for CTEPH between May 2015 and December 2017. The most proximal level of disease in the pulmonary artery on CTPA was classified by two blinded radiologists as level 1 (main pulmonary artery), 2a (lobar pulmonary artery), 2b (origin of basal segmental pulmonary artery), 3 (segmental pulmonary artery) or 4 (predominantly subsegmental pulmonary artery). RESULTS: CTPA demonstrated level 1 in 20%, level 2a in 43%, level 2b in 11%, level 3 in 23% and level 4 in 3%. A majority of males presented with level 1 (55%) and level 2 (57%), and a majority of females (83%) with level 3 (p=0.01). Levels 3 and 4 were associated with longer duration of circulatory arrest (p=0.03) and higher frequency of Jamieson type III disease at surgery (p<0.0001). Requirement for targeted pulmonary hypertension therapy after PEA was 28% at 3 years in level 2b/3/4 compared with 6% in level 2a and 13% in level 1 (p=0.002). Level 2b/3/4 was an independent predictor for targeted pulmonary hypertension therapy after PEA (hazard ratio 4.23, 95% CI 1.24-14.39; p=0.02). CONCLUSIONS: High-quality CTPA provides accurate evaluation of CTEPH patients. The level of disease on CTPA can help guide peri-operative planning and post-operative monitoring.

Incident pulmonary arterial hypertension associated with Bosutinib.

Pulmonary arterial hypertension is associated with tyrosine kinase inhibitors used in the treatment of chronic myeloid leukemia. Dasatinib is a known cause of drug-induced pulmonary arterial hypertension. There have been case reports linking Bosutinib with deterioration of pre-existing pulmonary arterial hypertension. Here, we present a case of a 37-year-old woman with chronic myeloid leukemia treated with Bosutinib who was diagnosed with pulmonary arterial hypertension. Prior to Bosutinib, she had received Dasatinib without documented cardiopulmonary toxicity. Withdrawal of Bosutinib led to partial reversal of pulmonary arterial hypertension, and with the addition of pulmonary arterial hypertension-targeted treatment, there was near normalization of hemodynamics.

Correction of pulmonary hypertension with intensive hemodialysis: A case report.

Pulmonary hypertension (PH) defined as a mean pulmonary arterial pressure ≥25 mmHg is a common complication in hemodialysis occurring in up to 58% of patients. PH is classified according to its etiology. We report in a patient with severe PH of mixed etiology (connective tissue disease and left-sided heart failure) who improved after initiation of intensive home hemodialysis. We postulated that the use of a frequent mode of hemodialysis may ameliorate PH via enhanced volume control. Thereby, an intensive hemodialysis schedule may be preferred renal replacement modality in this patient population.

Symptoms Are More Useful Than Echocardiography in Patient Selection for Pulmonary Endarterectomy.

BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is still largely underdiagnosed in the general population. Although transthoracic echocardiogram (TTE) is recommended to screen for CTEPH, it may not detect patients with chronic thromboembolic disease (CTED) and mild or exercise-induced pulmonary hypertension (PH) who could also benefit from pulmonary endarterectomy (PEA). METHODS: All patients referred to our CTEPH program with persistent mismatched perfusion defects on ventilation-perfusion (VQ) scan between January 2005 and June 2015 were divided into three groups according to TTE and right heart catheterization (RHC) as follows: (1) typical CTEPH group (PH on RHC and TTE), (2) TTE-negative (neg) CTEPH group (PH on RHC, but not TTE), or (3) CTED group (no PH on RHC and TTE). RESULTS: Of 225 patients with abnormal VQ scans, 188 (84%) had typical CTEPH, 15 had TTE-neg CTEPH, and 22 had CTED. PEA was performed in 179 patients (80%). Reasons for exclusion in CTEPH patients included primarily distal disease (n = 11) and comorbidities (n = 10). In contrast, the absence of functional limitation was the main cause of exclusion in CTED patients (75% versus 3% in CTEPH patients, p < 0.0001). The 90-day mortality rate after PEA was 4% in the typical CTEPH group and 0% in the TTE-neg CTEPH and CTED groups. Pulmonary arterial pressures and functional class significantly improved after PEA in all three groups. CONCLUSIONS: Patients with mild CTEPH can benefit from PEA, but may not be detected by TTE. Symptomatic patients with functional limitation and persistent mismatched perfusion defects on VQ scan should undergo further investigations with pulmonary angiogram and RHC.

Exhaled Nitric Oxide in Systemic Sclerosis Lung Disease.

Background. Exhaled nitric oxide (eNO) is a potential biomarker to distinguish systemic sclerosis (SSc) associated pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD). We evaluated the discriminative validity, feasibility, methods of eNO measurement, and magnitude of differences across lung diseases, disease-subsets (SSc, systemic lupus erythematosus), and healthy-controls. Methods. Consecutive subjects in the UHN Pulmonary Hypertension Programme were recruited. Exhaled nitric oxide was measured at 50 mL/s intervals using chemiluminescent detection. Alveolar and conducting airway NO were partitioned using a two-compartment model of axial diffusion (CMAD) and the trumpet model of axial diffusion (TMAD). Results. Sixty subjects were evaluated. Using the CMAD model, control subjects had lower median (IQR) alveolar NO than all PAH subjects (2.0 (1.5, 2.5) versus 3.14 ppb (2.3, 4.0), p = 0.008). SSc-ILD had significantly lower median conducting airway NO compared to controls (1009.5 versus 1342.1 ml⁎ppb/s, p = 0.04). SSc-PAH had increased median (IQR) alveolar NO compared to controls (3.3 (3.0, 5.7) versus 2.0 ppb (1.5, 2.5), p = 0.01). SSc-PAH conducting airway NO inversely correlated with DLCO (r -0.88 (95% CI -0.99, -0.26)). Conclusion. We have demonstrated feasibility, identified that CMAD modeling is preferred in SSc, and reported the magnitude of differences across cases and controls. Our data supports discriminative validity of eNO in SSc lung disease.

Role of extracorporeal life support after pulmonary endarterectomy: a single-centre experience.

OBJECTIVES: Extracorporeal life support (ECLS) for rescue after pulmonary endarterectomy (PEA) has become a viable option. This study aims to present a single-centre experience looking at the indications and outcome of ECLS after PEA. METHODS: Retrospective analysis of all patients undergoing PEA from January 2008 to January 2015 in our institution. RESULTS: Among 144 consecutive patients undergoing PEA for chronic thromboembolic pulmonary hypertension, 6 (4%) received ECLS postoperatively for right ventricular (RV) failure (n = 3), severe hypoxaemia (n = 2) and haemorrhagic pulmonary oedema (n = 1). ECLS configuration was central veno-arterial (cVA) in 3 patients, peripheral VA (pVA) in 1 and veno-venous (VV) in 2. One patient with cVA was switched to VV after 5 days. Overall ECLS duration ranged between 3 and 39 (median 5) days. ECLS patients had higher preoperative total pulmonary vascular resistance (TPR) compared with non-ECLS patients (1477 ± 671 vs 954 ± 462 Dynes.s.cm(-5), P = 0.009) and more frequently required hospital admission for RV failure before surgery (50 vs 9%, P = 0.02). The overall in-hospital mortality rate for all patients was 2% (3/144), including one ECLS patient on pVA. The remaining 5 ECLS patients (83%) were discharged from the hospital and are alive after a median follow-up of 11 (range 6-27) months. Two ECLS patients (40%) are on therapy for residual PH compared with 13 (10%) in the non-ECLS patients (P = 0.09). CONCLUSIONS: ECLS is a safe and important rescue option after PEA. The use of ECLS may expand eligibility for PEA by allowing sicker patients to undergo surgery.

Intrathoracic Rosai Dorfman Disease with Focal Aggregates of IgG4-bearing Plasma Cells. Case Report and Literature Review.

Rosai Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic disorder that typically presents as painless cervical adenopathy. Occasionally, Rosai Dorfman disease may involve extranodal sites and it can be associated with constitutional symptoms. In this report, we present a patient with progressive dyspnea on exertion, obstruction on spirometry, and paratracheal soft tissue thickening extending to the proximal bronchi and hila. After extensive radiologic and interventional measures, including multiple biopsies, the diagnosis was established on pathology showing characteristic features of Rosai Dorfman disease in a paratracheal lymph node and features of extranodal Rosai Dorfman disease with extensive fibrosis in the paratracheal mass. In addition, the involved paratracheal lymph node demonstrated focally increased IgG4-positive plasma cells, a finding that was not present in the tracheal mass. A review of intrathoracic manifestations of Rosai Dorfman disease including the proposed causes and causative triggers is presented. The histological features and the current understanding of the management and prognosis are also discussed. This unusual presentation highlights the need to consider histiocytic disorders in the differential diagnosis of paratracheal disease.

Sex disparities in systemic sclerosis-associated pulmonary arterial hypertension: a cohort study.

BACKGROUND: The impact of male sex as a determinant of health outcomes in systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) is controversial. The primary objective of this study was to evaluate the effect of sex on survival in patients with SSc-PAH. The secondary objectives were to evaluate the effect of sex on age of PAH diagnosis, time from SSc diagnosis to PAH diagnosis, and SSc disease manifestations. METHODS: Sex-based disparities were evaluated in a cohort of SSc-PAH patients with a primary outcome of time from PAH diagnosis to all-cause mortality. Secondary outcomes were differences in age of diagnosis, disease duration, and SSc manifestations. Survival differences were evaluated using Kaplan-Meier and Cox proportional hazard models. RESULTS: We identified 378 SSc-PAH (58 males, 320 females) patients, with a female:male ratio of 5.5:1. Males had a shorter mean ± standard deviation time from SSc diagnosis to PAH diagnosis (1.7 ± 14 versus 5.5 ± 14.2 years); shorter PAH duration (3.5 ± 3.1 versus 4.7 ± 4.2 years), increased frequency of renal crisis (19 % versus 8 %, relative risk (RR) 2.33, 95 %CI 1.22, 4.46), interstitial lung disease (67 % versus 48 %, RR 1.41, 95 %CI 1.14, 1.74), and diffuse subtype (40 % versus 22 %, RR 1.84, 95 %CI 1.26, 2.69). Males appeared to have decreased 1-, 2-, 3-, and 5-year survival (83.2 %, 68.7 %, 53.2 %, 45.6 %) compared to females (85.7 %, 75.7 %, 66.4 %, 57.4 %). However, there was no difference in mortality between sexes (HR 1.43 (95 %CI 0.97, 2.13). CONCLUSIONS: Sex disparities appear to exist in the frequency of PAH, time to PAH diagnosis, PAH disease duration and SSc disease burden. However, male sex does not independently impact SSc-PAH survival.

A case of pulmonary arterial hypertension associated with adult hemophagocytic lymphohistiocytosis.

Hemophagocytic lymphohistiocytosis (HLH) is an aggressive, life-threatening syndrome of excessive immune activation. Presentation is most common among the pediatric population, and cases in adults are rare. The number of nonhematologic presentations described in relation to HLH has been growing. We present a case involving a woman who developed HLH after autologous stem cell transplantation for mantle cell lymphoma. Months later, she received a diagnosis of pulmonary arterial hypertension (PAH) while undergoing treatment for her HLH. To our knowledge, PAH associated with adult HLH has only been described in the literature once before. PAH may now be a potential differential diagnosis for patients with HLH who present with respiratory symptoms.

Survival in rheumatoid arthritis-associated pulmonary arterial hypertension compared with idiopathic pulmonary arterial hypertension.

BACKGROUND AND OBJECTIVE: In this study, we evaluated survival in rheumatoid arthritis-associated pulmonary arterial hypertension (RA-PAH) compared with idiopathic pulmonary arterial hypertension (IPAH) patients, and evaluate differences in disease severity and treatment. METHODS: We conducted a retrospective cohort study of RA-PAH and IPAH at the University Health Network Pulmonary Hypertension Programme, Toronto, Canada. The primary outcome was time to all-cause mortality. We evaluated survival using Kaplan-Meier curves. Using a propensity score-matched cohort, we used Cox proportional hazards models to estimate survival. RESULTS: Screening 1385 patients identified 18 RA-PAH and 155 IPAH patients. RA-PAH patients had an older median age of onset (64.0 vs 53.7 years) and lower baseline mean pulmonary arterial pressure (mPAP) (41 vs 50 mm Hg, P = 0.02). RA-PAH patients tended to have a higher proportion of females (83% vs 70%, relative risk 0.55, 95% confidence interval (CI): 0.19-1.57), lower proportion with baseline World Health Organization functional class III/IV (39% vs 52%), lower median baseline brain natriuretic peptide (58.4 vs 95.0 pg/mL) and longer baseline 6-min walk distance (440 vs 397 m). There were 35 deaths, 2/18 (11%) RA-PAH patients and 33/155 (21%) IPAH patients. The unadjusted 1-year survival was 93% for RA-PAH and 94% for IPAH. In the matched cohort, there were seven deaths: 2/18 (11%) RA-PAH and 5/18 (28%) IPAH patients, hazard ratio 1.53 (95% CI: 0.15-2.84). Separation of survival curves did not achieve statistical significance, log-rank 0.56. CONCLUSIONS: Compared with IPAH patients, RA-PAH patients have an older age of onset and lower baseline mPAP. RA-PAH patients have comparable survival to IPAH patients.

Pulmonary endarterectomy in severe chronic thromboembolic pulmonary hypertension.

BACKGROUND: The outcome of patients with chronic thromboembolic pulmonary hypertension (CTEPH) undergoing pulmonary endarterectomy (PEA) after urgent hospitalization for decompensated right heart failure (DRHF) remains unclear. METHODS: Among 120 consecutive patients undergoing PEA, 16 (13%) presented with a history of urgent hospitalization for DRHF (severe RHF group). This group was compared with the remaining 104 patients presenting with total peripheral vascular resistance (TPR) < 1,200 dynes · sec · cm(-5) (n = 78, control group) and >1,200 dynes · sec · cm(-5) (n = 26, high TPR group). RESULTS: DRHF occurred predominantly in patients with TPR > 1,200 dynes · sec · cm(-5) (prevalence of 32% vs 5% in patients with TPR < 1,200 dynes · sec · cm(-5), p < 0.0001). The overall in-hospital mortality after PEA was 4% (n = 5). All deaths occurred in patients with TPR > 1,200 dynes · sec · cm(-5) and DRHF. The proportion of patients with residual PH immediately after surgery was higher in the high TPR group (50%) and severe RHF group (56%) compared with the control group (29%; p = 0.04). In multivariate analysis, risk factors for residual PH after surgery were TPR > 1,200 dynes · sec · cm(-5), Jamieson disease Type III-IV, and female gender. A history of DRHF by itself was not a risk factor for residual PH after PEA. The overall 5-year survival was 87%. After a median follow-up of 20 months, the proportion of patients receiving medical therapy for residual PH was higher in patients with post-operative mean pulmonary artery pressure ≥35 mm Hg (61% vs 9%, p = 0.0007). CONCLUSIONS: DRHF occurs more frequently in patients with TPR > 1,200 dynes · sec · cm(-5), increasing the operative risk in these patients. The outcome of patients with high TPR in the absence of DRHF is excellent. However, patients with residual mean pulmonary artery pressure ≥35 mm Hg frequently receive pulmonary hypertension therapy after PEA.

A rare cause of hemoptysis.

Angiosarcomas are rare, malignant, endothelial-cell tumors of vascular origin that can arise at any body site. They frequently metastasize to the lung, heralded by dyspnea, hemoptysis, chest pain, pneumothoraces, and diffuse pulmonary hemorrhage. However, in most cases lung metastases are discovered after the diagnosis of a primary angiosarcoma has already been established. Very rarely will an undiagnosed metastatic angiosarcoma present as diffuse pulmonary hemorrhage. We describe the case of a 59-year-old male who presented to hospital with dyspnea and hemoptysis. CT chest revealed rapidly progressing nodular changes and broncho-alveolar lavage returns were progressively bloody. Open lung wedge biopsy ultimately revealed metastatic angiosarcoma and extensive pulmonary hemorrhage. Our case highlights the key clinical, radiological, and pathological features of this rare malignancy that frequently metastasizes to the lung and reminds clinicians to consider it as a cause of hemoptysis and pulmonary hemorrhage.