Absence of Pericarditis Recurrence in Rilonacept-Treated Patients With COVID-19 and SARS-CoV-2 Vaccination: Results From the RHAPSODY Long-term Extension.

Background: Rilonacept inhibits the interleukin-1 pathway, and extended treatment in patients with recurrent pericarditis (RP) reduced recurrence risk by 98% in the phase 3 trial, RHAPSODY long-term extension (LTE). Severe acute respiratory syndrome (SARS)-CoV-2 vaccination and/or infection may trigger pericarditis recurrence, and in clinical practice, it is unknown whether to continue rilonacept during SARS-CoV-2 infection. This post-hoc analysis of the RHAPSODY LTE aimed to inform rilonacept management in RP patients vaccinated against SARS-CoV-2 or who contract COVID-19. Methods: Analysis was conducted from May 2020 to June 2022. The LTE portion of RHAPSODY LTE enabled up to 24 months of additional open-label rilonacept treatment beyond the pivotal study. Rilonacept efficacy data in preventing pericarditis recurrence were assessed, and concomitant SARS-CoV-2 vaccination and COVID-19 adverse event data were evaluated. Results: No pericarditis recurrences were temporally associated with vaccination. Sixteen COVID-19 cases were reported; 10 in 30 unvaccinated or partially vaccinated patients (33%) vs 6 of 44 fully vaccinated patients (14%; P = 0.04). Twelve of 16 patients (75%) were receiving rilonacept at the time of infection, and none experienced pericarditis recurrence. One pericarditis recurrence occurred in the peri-COVID-19 period in 1 of 4 patients who had stopped rilonacept treatment > 4.5 months prior. COVID-19 severity was mild in 13 patients, moderate in 2, and severe in 1. Conclusions: Full vaccination effectively reduced COVID-19 events in patients treated with rilonacept. Vaccination or COVID-19 during rilonacept treatment did not increase pericarditis recurrence. Continued rilonacept treatment in patients contracting COVID-19 did not worsen disease severity, whereas rilonacept interruption increased pericarditis recurrence, supporting a recommendation for continued rilonacept treatment for RP during vaccination or COVID-19. ClinicalTrialsgov identifier: NCT03737110.

Contexte: Le rilonacept inhibe la voie de l'interleukine-1 et, d'après les résultats de la période de prolongation à long terme de l'essai de phase III RHAPSODY, la poursuite du traitement par cet agent chez les patients atteints de péricardite récidivante a réduit le risque de récidive de 98 %. La vaccination contre le syndrome respiratoire aigu sévère (SRAS)-CoV-2 ou l'infection à ce virus pourrait toutefois déclencher une récidive de la péricardite, et dans la pratique clinique, on ignore s'il vaut mieux poursuivre le traitement par rilonacept pendant l'infection à SRAS-CoV-2. Cette analyse post-hoc de la période de prolongation à long terme de l'essai RHAPSODY vise à orienter la gestion du rilonacept chez les patients atteints de péricardite récidivante qui sont vaccinés contre le SRAS-CoV-2 ou qui contractent la COVID-19. Méthodologie: L'analyse a été effectuée de mai 2020 à juin 2022. La période de prolongation à long terme de l'essai RHAPSODY a permis d'accumuler des données en mode ouvert pendant une période allant jusqu'à 24 mois au-delà de l'étude pivot. Les données sur l'efficacité du rilonacept en prévention de la récidive de péricardite ont été évaluées, tout comme les données sur la vaccination concomitante contre le SRAS-CoV-2 et les cas de COVID-19. Résultats: Aucune récidive de la péricardite n'a pu être associée sur le plan temporel avec la vaccination. Au total, 16 cas de COVID-19 ont été signalés, dont 10 chez les patients non vaccinés ou partiellement vaccinés sur 30 (33 %) et 6 chez les patients complètement vaccinés sur 44 (14 %; p = 0,04). De ces 16 patients, 12 (75 %) prenaient du rilonacept au moment de l'infection et aucun n'a connu de récidive de la péricardite. Une récidive de la péricardite s'est produite dans la période suivant la COVID-19 chez 1 des 4 patients qui avaient cessé de prendre le rilonacept > 4,5 mois auparavant. La COVID-19 a été légère chez 13 patients, modérée chez 2 patients et sévère chez 1 patient. Conclusions: La vaccination complète a réduit efficacement les cas de COVID-19 chez les patients traités par le rilonacept. La vaccination ou l'infection à SRAS-CoV-2 pendant le traitement par rilonacept n'a pas augmenté le risque de récidive de la péricardite. La poursuite du traitement par rilonacept chez les patients atteints de COVID-19 n'a pas aggravé la sévérité de la maladie, tandis que l'interruption du traitement a augmenté le risque de récidive de la péricardite, ce qui plaide en faveur de la recommandation de poursuivre le traitement de la péricardite récidivante par le rilonacept pendant la vaccination ou la COVID-19. Numéro d'identification ClinicalTrialsgov: NCT03737110.

Radial versus femoral access, bleeding and ischemic events in patients with non-ST-segment elevation acute coronary syndrome managed with an invasive strategy.

BACKGROUND: Bleeding is a major limitation of antithrombotic therapy among invasively managed non-ST-segment elevation acute coronary syndromes (NSTE-ACS) patients; therefore, we examined the use of radial access and its association with outcomes among NSTE-ACS patients. METHODS: Clinical characteristics and geographic variation in radial access were examined, as well as its association with bleeding, red blood cell transfusion and ischemic outcomes (96-hour death/myocardial infarction/recurrent ischemic/thrombotic bailout; 30-day death/myocardial infarction; 1-year death) in the EARLY versus delayed, provisional eptifibatide in acute coronary syndromes trial. RESULTS: Of 9126 patients, 13.5% underwent radial-access catheterization. Female sex, age, weight, and prior revascularization were inversely associated with radial access, and its use varied widely by country (2%-97%). There were fewer GUSTO severe/moderate bleeds and red blood cell transfusions in the radial access group; however, it was attenuated after adjustment (odds ratio 0.73, 95% confidence intervals [CI] [0.50-1.06], P = .094 and 1.00 [0.71-1.40] P = .991). Ischemic outcomes did not differ by access site. CONCLUSIONS: In this post hoc analysis of a large clinical trial, there was significant international variation in use of radial access for NSTE-ACS patients undergoing invasive management, and it was preferentially used in those at lower risk for bleeding. Radial approach was not associated with a significant reduction in either bleeding or ischemic outcomes. Further study is needed to determine whether wider application of radial approach to acute coronary syndrome patients at high risk for bleeding improves overall outcomes.

Surgery for ischemic mitral regurgitation: should the valve be repaired?

BACKGROUND AND AIM OF THE STUDY: Patients with ischemic cardiomyopathy undergoing coronary artery bypass grafting (CABG) often have concomitant mitral regurgitation (MR). Repairing the valve at the time of surgery is not universally accepted. The results of CABG with or without mitral valve annuloplasty (MVA) were compared in patients with reduced left ventricular (LV) function and ischemic MR. METHODS: Among a total of 195 patients, 108 underwent isolated CABG, and 87 underwent CABG with MVA. The study end-points included survival, degree of MR, and NYHA functional class. RESULTS: Patients in the MVA group were younger (mean age 63 +/- 10 versus 68 +/- 9 years; p <0.001), but had a more severe cardiac pathology, with severe LV dysfunction in 45% versus 26% (p = 0.006) and severe MR in 82% versus 14% (p < 0.001). The operative mortality was 9%, and similar in both groups. The follow up was complete, with a mean survival period of 87 +/- 50 months. Although, overall, no improvement was seen in LV function, symptomatic improvement was more pronounced in the MVA group (p = 0.006). At follow up, residual MR was present in 2% of the MVA group and in 47% of the CABG-only group (p < 0.0001). For the MVA and CABG-only groups, respectively, survival at five and 10 years was 68% and 46% versus 77% and 52% (p = NS). By multivariate analysis, neither degree of MR nor LV function at follow up had any impact on survival. CONCLUSION: In patients with a reduced LV function undergoing CABG, the addition of a mitral annuloplasty does not increase the operative risk. Although patients in the MVA group were more ill, there was a better symptomatic improvement in this group, and they attained a similar survival. It is recommended that MVA be performed at the time of CABG in patients having moderate or greater MR associated with a reduced LV function.

Tricuspid incompetence following permanent pacemaker implantation.

AIM: Severe tricuspid insufficiency (TI) after permanent pacemaker implantation (PPI) has been described in small series of patients, though its incidence is not known. METHODS: We retrospectively analyzed the data of 545 patients who underwent PPI and had Doppler echocardiograms performed before and after the procedure. We excluded 135 patients who had > or =moderate TI on the 1st Doppler echocardiogram. RESULTS: Group 1 included 75 patients (18.3%) who had a >2 grades worsening of TI, and group 2 included 335 patients (82%) with <2 grade increase in TI after PPI. Patients in group 1 were 77 +/- 7 years of age, versus 72 +/- 10 years in group 2 (P < 0.001). There was no difference in left ventricular size and function. The TI gradient before PPI was higher in group 2 (25 +/- 13 mmHg versus 19 +/- 12 mmHg [P < 0.001]), though within the normal range in both groups. The mitral E/A ratio was 0.98 in group 1 versus 1.42 in group 2 (P < 0.001). The systolic TI gradient after implantation was 42 +/- 12 mmHg in group 1, versus 33 +/- 8 mmHg in group 2 (P < 0.001). CONCLUSION: Worsening of TI after PPI was not rare and was observed more often in older patients, with abnormal LV relaxation and who developed pulmonary hypertension after the procedure.

Delayed prolongation of the QRS interval in patients with left ventricular dysfunction.

AIMS: Patients with left ventricular dysfunction (LVD) and prolonged QRS on surface electrocardiogram are at increased risk for heart failure and death and may benefit from resynchronization therapy. Patients with initial narrow QRS may prolong their QRS during the disease course. The occurrence of delayed QRS prolongation, its predictors and associated risk of heart failure hospitalizations (HFH) or death are currently unknown and the subject of this investigation. METHODS & RESULTS: Patients with LVD, QRS < 120 ms and available follow-up ECGs were retrospectively evaluated for persistent unprovoked QRS prolongation >130 ms. Impact on mortality or HFH was assessed using Cox regression with QRS > 130 ms as a time dependent covariate. Following 178 patients for 30 (10;59) median (IQR) months, 28 (16%) patients prolonged their QRS to >130 ms, reaching a QRS duration of 154 ±â€¯29 ms; LBBB pattern was diagnosed among 14 (50%) patients. Patients with delayed QRS prolongation were older (71.9 ±â€¯11.8 vs 64.4 ±â€¯15.1 years p = 0.014), had larger left ventricle and left atrial diameters (6.3 ±â€¯0.9 vs 5.7 ±â€¯0.9 cm p = 0.010; 4.9 ±â€¯0.6 vs 4.5 ±â€¯0.7 cm p = 0.006, respectively) and wider baseline QRS (104.8 ±â€¯12.6 vs 91.4 ±â€¯14.5 ms p < 0.001) which was linearly associated with late QRS prolongation (p for trend<0.0001). In a multivariable model, age, baseline QRS width and left atrial diameter were significantly associated with delayed QRS prolongation. QRS prolongation at follow-up was independently associated with risk of death or HFH (HR 7.426, 95% CI3.017-18.280, p < 0.0001). CONCLUSION: QRS prolongation occurs in a significant proportion of patients with LVD and portends adverse outcome. Advanced age, prolonged QRS and larger left atria are potential predictors. Routine monitoring is justified and physicians may choose to plan ahead for resynchronization therapy in patients at risk for QRS prolongation.

[Surgery for ischemic mitral regurgitation: results and long-term follow-up].

BACKGROUND: The natural history of patients with ischemic mitral regurgitation (IMR) shows a poor prognosis. The surgical risk in these patients is high, but affords improved survival. We examined long-term results after coronary bypass and mitral repair for patients with IMR. METHODS: There were 122 patients operated on between the years 1993-2004. Patient age was 65 +/- 10 years, and 89 (73%) were male. Fifty-one (42%) were in NYHA class IV (mean NYHA class 3.1 +/- 1); 77 (63%) had pre-operative LV function grade 3-4 (mean LV grade 2.8 +/- 1); 103 (84%) had MR grade 4 (mean MR grade 3.8 +/- 0.4). All patients received mitral valve annuloplasty with a flexible ring (size 26 +/- 1 mm). Number of bypass grafts performed was 2.5 +/- 0.9. RESULTS: Operative mortality was 7% (9 patients). Operative survivors (n = 113) were followed for a mean interval of 34 months (range 2-91). NYHA class was 2.2 +/- 1. In 65 patients (58%) LV function was grade 1-2. Mean MR was 1.6 +/- 1 for the whole group, and 28 (25%) remained with MR grade 3-4. Late mortality was 18% (21 patients) 33 +/- 20 months after surgery (range 2-62). Predictors for late mortality were poor LV function, NYHA class IV, and age > 70 years (p < 0.0001). Overall, residual MR did not emerge as a predictor for late mortality. CONCLUSIONS: Surgery for patients with IMR can be performed with an acceptable risk. In our patient group, LV function is the major determinant for late survival, overshadowing the effect of residual MR.

Pulmonary Hypertension with Left Heart Disease: Prevalence, Temporal Shifts in Etiologies and Outcome.

INTRODUCTION: Pulmonary hypertension has many causes. While it is conventionally thought that the most prevalent is left heart disease, little information about its proportion, causes, and implications on outcome is available. METHODS: Between 1993 and 2015, 12,115 of 66,949 (18%) first adult transthoracic echocardiograms were found to have tricuspid incompetence gradient ≥40 mm Hg, a pulmonary hypertension surrogate. Left heart disease was identified in 8306 (69%) and included valve malfunction in 4115 (49%), left ventricular systolic dysfunction in 2557 (31%), and diastolic dysfunction in 1776 (21%). Patients with left heart disease, as compared with those without left heart disease, were of similar age, fewer were females (50% vs 63% P <.0001), and they had higher tricuspid incompetence gradient (median 48 mm Hg [interquartile range 43, 55] vs 46 mm Hg [42, 54] P <.0001). In reviewing trends over 20 years, the relative proportions of systolic dysfunction decreased and diastolic dysfunction increased (P for trend <.001), while valve malfunction remained the most prevalent cause of pulmonary hypertension with left heart disease. Independent predictors of mortality were age (hazard ratio [HR] 1.05; 95% CI, 1.04-1.05; P <.0001), tricuspid incompetence gradient (HR 1.02; 95% CI, 1.01-1.02, P <.0001 per mm Hg increase), and female sex (HR 0.87; 95% CI, 0.83-0.91, P <.0001). RESULTS: Overall, left heart disease was not an independent risk factor for mortality (HR 1.04; 95% CI, 0.99-1.09; P = .110), but patients with left ventricular systolic dysfunction and with combined systolic dysfunction and valve malfunction had increased mortality compared with patients with pulmonary hypertension but without left heart disease (HR 1.30; 95% CI, 1.20-1.42 and HR 1.44; 95% CI, 1.33-1.55, respectively; P <.0001 for both). CONCLUSIONS: Pulmonary hypertension was found to be associated with left heart disease in 69% of patients. Among these patients, valve malfunction and diastolic dysfunction emerged as prominent causes. Left ventricular dysfunction carries additional risk to patients with pulmonary hypertension.

Does mitral valve intervention have an impact on late survival in ischemic cardiomyopathy?

BACKGROUND: Ischemic mitral regurgitation is associated with reduced survival after coronary artery bypass surgery. OBJECTIVES: To compare long-term survival among patients undergoing coronary surgery for reduced left ventricular function and severe ischemic MR in whom the valve was repaired, replaced, or no intervention was performed. METHODS: Eighty patients with severe left ventricular dysfunction and severe MR underwent coronary bypass surgery. The mean age of the patients was 65 years (range 42-82), and 63 (79%) were male. Sixty-three (79%) were in preoperative NYHA functional class III-IV (mean NYHA 3.3), and 26 (32%) were operated on an urgent/emergent basis. Coronary artery bypass surgery was performed in all patients. The mitral valve was repaired in 38 and replaced in 14, and in 28 there was no intervention. The clinical profile was similar in the three groups, although patients undergoing repair were slightly younger. RESULTS: Operative mortality was 15% (8%, 14%, and 25% for the repair, replacement and no intervention respectively; not significant). Long-term follow up was 100% complete, for a mean of 38 months (range 2-92). Twenty-nine patients (57%) were in NYHA I-II (mean NYHA 2.3). Among the surgery survivors, late survival was improved in the repair group compared to the other groups (P < 0.05). Predictors for late mortality were non-repair of the mitral valve, residual MR, and stroke (P = 0.005). CONCLUSIONS: Patients with severe ischemic cardiomyopathy and severe MR undergoing coronary bypass surgery should have a mitral procedure at the time of surgery. Mitral valve repair offers a survival advantage as compared to replacement or no intervention on the valve. Patients with residual MR had the worst results.

Anaemia and heart failure: aetiology and treatment.

Heart failure (HF) is a common disease associated with poor prognosis. Anaemia is commonly associated with HF due to bone marrow depression, reduced availability of iron and haemodilution, and is sometimes aggravated by too frequent blood testing. Low haemoglobin is very detrimental to the haemodynamic state of the patient with decreased cardiac output as it further diminishes the oxygen supply to the tissues. When anaemia is associated with HF. and renal failure, the patient enters a vicious cycle called cardio renal anaemia syndrome. The prognosis of patients with HF is worse as the haemoglobin is lower and even mild anaemia is associated with <1 year survival. Aggressive correction of the anaemia by subcutaneous injections of erythropoeitin and intravenous iron has been shown to improve the functional capacity and quality of life of patients with cardio renal anaemia syndrome and to reduce the need for hospitalization. However, intravenous iron can be detrimental because of increased formation of free radicals, oxidative stress and risk of infection. The level of haemoglobin needed to be achieved is not clear, but it seems indicated to maintain it above 12 g%.

Rofecoxib, a COX-2 inhibitor, lowers C-reactive protein and interleukin-6 levels in patients with acute coronary syndromes.

BACKGROUND: Patients with acute coronary syndromes (ACS) have high levels of inflammatory mediators such as C-reactive protein (CRP) and interleukin (IL)-6. AIM: To evaluate whether patients with ACS treated with rofecoxib, a COX-2 inhibitor, will have reduced CRP, IL-6, and soluble tumor necrotic factor receptor-1 (sTNF-R1) levels and improved endothelial function. METHODS AND RESULTS: Thirty-four patients hospitalized with ACS were randomized to receive rofecoxib, 25 mg/d plus aspirin 100 mg/d, or placebo plus aspirin, 100 mg/d, for a period of 3 months. Blood samples for CRP, IL-6, and sTNF-R1 levels were drawn prior to randomization, and after 1 month and 3 months. CRP levels in the rofecoxib group (n = 18) were significantly lower both at 1 month and 3 months compared to the baseline levels (p < 0.02). IL-6 levels were significantly lower at 1 month (p < 0.02) in the rofecoxib group, but not at 3 months. There was no change in endothelial function or sTNF-R1 levels. CONCLUSION: Patients recovering from ACS had lower levels of CRP and IL-6 at 1 month and lower CRP levels at 3 months when treated with rofecoxib plus aspirin. Suppression of inflammatory processes may lead to retardation of coronary atherosclerosis and coronary events.

Results of coronary artery stenting in women versus men: a single center experience.

BACKGROUND: Prior studies have suggested that women are at higher risk for morbidity and mortality during coronary angioplasty, although long-term prognosis is similar after successful procedures. OBJECTIVES: To examine the role of gender in coronary stenting, including immediate procedural success as well as early and late outcomes. METHODS: The study group comprised 560 consecutive patients (119 women and 441 men) who had undergone stenting over a 3 year period. RESULTS: The indications for coronary stenting were similar among women and men, and stents were successfully deployed at similar rates without complications (92 vs. 90% respectively). Cardiac death or myocardial infarction within 30 days of the procedure was observed in 5% of women and men, whereas none of the women, compared to 1.4% of men, had early revascularization. Bleeding complications occurred in 4% of women and 2% of men. During 10 +/- 2.8 months of follow-up, 58% of women and men underwent repeat cardiac catheterization, revealing similar rates of restenosis, 36 vs. 32% respectively. During the study period, 3.3% of women as compared to 0.9% of men had a cardiac death (not significant). Cardiac death or myocardial infarction was observed in 7% of women and 8% of men, and the combined endpoint of death, myocardial infarction or revascularization, was noted in 24% and 26% respectively. Multivariate Cox analyses of the clinical, angiographic and procedural characteristics revealed that multiple stent deployment was the only predictor of major adverse cardiac event among men, whereas none of these characteristics predicted outcome in women. CONCLUSION: Coronary stenting is performed with similar success rates among women and men, with similar restenosis rates as well as early and late major adverse cardiac events.

Rapid transcatheter occlusion of a coronary cameral fistula using a three-lobed vascular occlusion plug.

Transcatheter occlusion of coronary cameral fistulae has been well reported. In large fistulae multiple devices with prolonged procedures are often required for complete occlusion. We present the case of a large right coronary artery-to-right atrial fistula that was rapidly and simply occluded with the three-lobed Amplatzer Vascular Plug 2.

Repair of ischemic mitral regurgitation: comparison between flexible and rigid annuloplasty rings.

BACKGROUND: The surgical treatment of ischemic mitral regurgitation (MR) usually involves implantation of an annuloplasty ring. We compared results of mitral valve repair using a flexible or a rigid annuloplasty ring in patients with ischemic MR undergoing coronary artery bypass graft surgery. METHODS: There were 169 patients. A flexible ring was implanted in 117 and a rigid ring in 52. Age and clinical profile, degree of left ventricular dysfunction, and degree of MR (mean 3.2) were similar between groups. RESULTS: Operative mortality was 9% in each group. Follow-up (58 +/- 30 months for flexible group and 14 +/- 7 months for rigid group) was available for 91%. For the flexible and rigid ring groups, respectively, mean New York Heart Association functional class was 1.9 and 1.6, with 33% and 14% in classes III to IV (p = 0.03); mean MR grade was 1.25 and 0.7 (p = 0.006). There was no difference in left ventricle function or dimensions. At follow-up, 29 patients (34%) in the flexible group had residual MR of moderate degree or greater compared with 6 (15%) in the rigid group (p = 0.03). Mean tricuspid incompetence gradient was 39 and 34 mm Hg (p = nonsignificant); however, the degree of reduction was greater in the rigid group (p = 0.001). Late mortality was observed in 32 patients, all in the flexible group. CONCLUSIONS: Clinical and hemodynamic results are better with rigid mitral annuloplasty rings compared with flexible rings. That result may be due to ring design, which dictates not only the annular diameter but also annular configuration. Longer follow-up is needed to determine differences in survival.

Transient left ventricular apical ballooning. Report of 5 Israeli patients.

BACKGROUND: Transient left ventricular (LV) apical ballooning is characterized by acute onset of chest pain with reversible balloon-like LV motion abnormality, hypercontractile basal segments, ST segment elevation or T-wave inversion in anterior chest leads and mild cardiac enzyme rise in the absence of significant coronary disease. METHODS: We describe 5 patients (4 females) with anteroapical ballooning who were hospitalized with acute myocardial infarction and showed ST segment elevation in anterior chest leads. RESULTS: Echocardiogram demonstrated apical ballooning with normal or hypercontractile contraction of the basal segments. Four patients had severe mitral incompetence and one had mild incompetence. All patients had also systolic anterior motion and 4 had a significant LV outflow (LVOT) gradient. All patients underwent cardiac catheterization soon after admission showing non-significant narrowing of the coronary arteries. At discharge 4 patients had normal LV function and 1 was mildly impaired. CONCLUSIONS: LV apical ballooning is relatively rare. It should be suspected in older patients, mainly women, with severe mitral incompetence and LVOT gradient.

C-reactive protein and NT-proBNP as surrogate markers for pulmonary hypertension in Gaucher disease.

BACKGROUND: N-terminal pro-Brain Natriuretic Peptide (NT-proBNP) values correlate with mild-moderate pulmonary hypertension assessed by tricuspid insufficiency (TI) gradient>or=30 mm Hg in Gaucher disease. The purpose of this study is to ascertain improved risk stratification based on correlation with NT-proBNP and C-reactive protein (CRP), a standard marker of inflammation. METHODS: Patients with type I Gaucher disease were selected to reflect differing degrees of echocardiographically determined TI gradient values. NT-proBNP was performed by immunoassay and CRP by standard methods. FINDINGS: There were 45 patients (18 males; 40%); mean age=42.5 (range: 4-80) years. Median NT-proBNP value=153 (range: 46-6703) pg/ml; median CRP value=0.145 (range: 0.02-2.69) mg/dl. There was a statistically significant correlation between these values (r=0.445, P<0.01). Elevations of CRP and NT-proBNP were risk factors for pulmonary hypertension with odds ratios of 8.47 and 4.9, respectively. The area under the Receiving Operator Characteristic (ROC) curve for diagnosis of pulmonary hypertension was 0.93+/-0.04 for CRP, and 0.7+/-0.1 for NT-proBNP. All patients with pulmonary hypertension had elevation of either CRP or NT-proBNP (100% sensitivity). CONCLUSIONS: Elevated CRP was a better predictor of pulmonary hypertension in Gaucher disease than elevated NT-proBNP values. Elevated CRP (>0.5 mg/dl) or elevated NT-proBNP (>150 pg/ml) reduces the need to perform echocardiography by more than half, even in this group with over-representation of pulmonary hypertension. The role of inflammatory features in pulmonary hypertension in Gaucher disease is discussed. Further studies are required to ascertain if this approach is useful for prognosis of pulmonary hypertension.