Peak Systolic Pulmonary Artery Pressure Research Articles

MPAP/CO Slope and Oxygen Uptake Add Prognostic Value in Aortic Stenosis.

Recent guidelines redefined exercise pulmonary hypertension as a mean pulmonary artery pressure/cardiac output (mPAP/CO) slope >3 mm Hg·L-1·min-1. A peak systolic pulmonary artery pressure >60 mm Hg during exercise has been associated with an increased risk of cardiovascular death, heart failure rehospitalization, and aortic valve replacement in aortic valve stenosis. The prognostic value of the mPAP/CO slope in aortic valve stenosis remains unknown. In this prospective cohort study, consecutive patients (n=143; age, 73±11 years) with an aortic valve area ≤1.5 cm2 underwent cardiopulmonary exercise testing with echocardiography. They were subsequently evaluated for the occurrence of cardiovascular events (ie, cardiovascular death, heart failure hospitalization, new-onset atrial fibrillation, and aortic valve replacement) during a follow-up period of 1 year. Findings were externally validated (validation cohort, n=141). One cardiovascular death, 32 aortic valve replacements, 9 new-onset atrial fibrillation episodes, and 4 heart failure hospitalizations occurred in the derivation cohort, whereas 5 cardiovascular deaths, 32 aortic valve replacements, 1 new-onset atrial fibrillation episode, and 10 heart failure hospitalizations were observed in the validation cohort. Peak aortic velocity (odds ratio [OR] per SD, 1.48; P=0.036), indexed left atrial volume (OR per SD, 2.15; P=0.001), E/e' at rest (OR per SD, 1.61; P=0.012), mPAP/CO slope (OR per SD, 2.01; P=0.002), and age-, sex-, and height-based predicted peak exercise oxygen uptake (OR per SD, 0.59; P=0.007) were independently associated with cardiovascular events at 1 year, whereas peak systolic pulmonary artery pressure was not (OR per SD, 1.28; P=0.219). Peak Vo2 (percent) and mPAP/CO slope provided incremental prognostic value in addition to indexed left atrial volume and aortic valve area (P<0.001). These results were confirmed in the validation cohort. In moderate and severe aortic valve stenosis, mPAP/CO slope and percent-predicted peak Vo2 were independent predictors of cardiovascular events, whereas peak systolic pulmonary artery pressure was not. In addition to aortic valve area and indexed left atrial volume, percent-predicted peak Vo2 and mPAP/CO slope cumulatively improved risk stratification.

Exercise pulmonary hypertension by the mPAP/CO slope in primary mitral regurgitation.

Exercise-induced pulmonary hypertension (PH), defined by a mean pulmonary arterial pressure over cardiac output (mPAP/CO) slope >3 mmHg/L/min, has important diagnostic and prognostic implications. The aim of this study is to investigate the value of the mPAP/CO slope in patients with more than moderate primary mitral regurgitation (MR) with preserved ejection fraction and no or discordant symptoms. A total of 128 consecutive patients were evaluated with exercise echocardiography and cardiopulmonary testing. Clinical outcome was defined as the composite of mitral valve intervention, new-onset atrial fibrillation, cardiovascular hospitalization, and all-cause mortality. The mean age was 63 years, 61% were male, and the mean LVEF was 66 ± 6%. The mPAP/CO slope correlated with peak VO2 (r = -0.52, P < 0.001), while the peak systolic pulmonary artery pressure (sPAP) did not (r = -0.06, P = 0.584). Forty-six per cent (n = 59) had peak exercise sPAP ≥60 mmHg, and 37% (n = 47) had mPAP/CO slope >3 mmHg/L/min. Event-free survival was 55% at 1 year and 46% at 2 years, with reduced survival in patients with mPAP/CO slope >3 mmHg/L/min (hazard ratio, 4.9; 95% confidence interval, 2.9-8.2; P < 0.001). In 53 cases (41%), mPAP/CO slope and peak sPAP were discordant: patients with slope >3 mmHg/L/mmHg and sPAP <60 mmHg (n = 21) had worse outcome vs. peak sPAP ≥60 mmHg and normal slope (n = 32, log-rank P = 0.003). The mPAP/CO slope improved predictive models for outcome, incremental to resting and exercise sPAP, and peak VO2. Exercise PH defined by the mPAP/CO slope >3 mmHg/L/min is associated with decreased exercise capacity and a higher risk of adverse events in significant primary MR and no or discordant symptoms. The slope provides a greater prognostic value than single sPAP measures and peak VO2.

Impact of percutaneous mitral valve clipping on right ventricular functions

Abstract Background Percutaneous mitral valve clipping (PMVC) is being used successfully in the management of severe functional mitral regurgitation (MR) in patients with high surgical risk. However, the impact of (PMVC) on right ventricular volumes and functions is not clear. Aim The aim of the present study was to assess the impact of (PMVC) (on both short and intermediate terms) on right ventricular (RV) volumes and functions using trans-thoracic echocardiography. Methods The study population included 37 patients with severe (MR), and congestive heart failurewho wereat high surgical risk. The mean age was 61±14 years, 9 (24.3%)were males. (PMVC) was successfully done for all patients.All had clinical and echocardiographic follow up at 6 and 12-months post (PMVC). Results MR showed significant improvement after (PMVC). MRwas less than grade II in 29 (78%)of patients at 6 months and persisted at 12 months (P=0.0001). Similarly, tricuspid regurgitation (TR) showed marked improvement. Severe (TR) was seen in 29 patients (78%) before), but decreased to 7 patients (18.9%) after (PMVC) (P=0.004). Left ventricular ejection fraction (LVEF) improved from 32.63±13.35% at base line to 36.31±13.28% at 6 months (P=0.003). This was maintained at 12 months (35.38±91%) Table 1 shows the changes in RV end diastolic volume (RVDV); RV end systolic volume (RVSV) RV ejection fraction (RVEF), Tricuspid annulus plane systolic excursion (TAPSE), RV longitudinal systolic function by tissue Doppler imaging (TDI) as well as peak systolic pulmonary artery pressure (PASP). Conclusion (PMVC) induced a significant reverse remodeling of RV by decreasing both (RVDV) and (RVSV). Moreover, it improved longitudinal RV systolic function as well as (RVEF). This was maintained up to one year follow up. Funding Acknowledgement Type of funding sources: None.

Echocardiographic and Other Preprocedural Predictors of Heart Failure After TIPS Placement in Patients With Cirrhosis: A Single-Center 15-Year Analysis.

BACKGROUND. Heart failure (HF) is an uncommon complication after TIPS placement; its development represents a poor prognostic factor. OBJECTIVE. The purpose of our study was to evaluate the frequency, risk factors, and association with survival of HF developing within 90 days after TIPS placement in patients with cirrhosis. METHODS. This retrospective single-center study included patients with cirrhosis who underwent nonemergent covered-stent TIPS placement from June 2003 to December 2018 and who underwent echocardiography within 2 months before TIPS placement and had at least 90 days of post-TIPS follow-up. Development of HF within 90 days after TIPS was recorded. Frequency of TIPS reduction for post-TIPS HF was determined. Univariable logistic regression analysis and ROC curve analysis were performed to assess potential risk factors for post-TIPS HF. Association of post-TIPS HF and 1-year survival was assessed by the log rank test. RESULTS. The study sample included 107 patients (71 men and 36 women; median age, 58 years). Post-TIPS HF developed in 11 of 107 (10%) patients; median time to development of HF was 16 days (range, 2-62 days). Of these 11 patients, three (27%) required TIPS reduction to achieve resolution of HF symptoms after unsuccessful diuretic therapy. Pre-TIPS right atrium size (odds ratio [OR], 3.26 [95% CI, 1.22-10.16]; p = .03], left ventricle (LV) end-systolic dimension (OR, 5.43 [95% CI, 1.44-24.50], p = .02), LV end-diastolic dimension (OR, 4.12 [95% CI, 1.51-13.47]; p = .009), and estimated peak pulmonary artery systolic pressure (PASP) (OR, 1.27 [95% CI, 1.12-1.50]; p = .001) were associated with post-TIPS HF. AUC of right atrium size, LV end-systolic dimension, LV end-diastolic dimension, and estimated peak PASP for development of post-TIPS HF were 0.71, 0.74, 0.72, and 0.83, respectively. At a cutoff of 31 mm Hg, PASP achieved sensitivity of 70% and specificity of 86% for post-TIPS HF. Patients with post-TIPS HF and those without post-TIPS HF had 1-year survival of 46% versus 73% (p = .06). CONCLUSION. Multiple pre-TIPS echocardiographic variables predict the development of post-TIPS HF in patients with cirrhosis. CLINICAL IMPACT. Preprocedural echocardiography may guide risk stratification in patients with cirrhosis being considered for TIPS placement.

Predicting heart failure transition and progression: a weighted risk score from bio-humoral, cardiopulmonary and echocardiographic stress testing

Abstract Funding Acknowledgements Type of funding sources: None. Aims. We tested the prognostic role of a risk score including bio-humoral evaluation, cardiopulmonary-echocardiographic stress (CPET-ESE) and lung ultrasound, in patients with heart failure (HF) with reduced and preserved ejection fraction (HFrEF and HFpEF), and subjects at risk of developing HF (American College of Cardiology/American Heart Association Stages A and B). Methods and results. We evaluated 318 subjects: 94 in Stages A-B, 194 in Stage C (85 HFpEF and 109 HFrEF), and 30 age and sex-matched controls (Stage 0). During a median follow-up of 18.5 months, we reported 40 urgent HF visits, 31 HF hospitalisations and 10 cardiovascular deaths. Cox proportional-hazards regression for predicting adverse events identified five independent predictors and each was assigned a number of points proportional to its regression coefficient: Δstress-rest B-lines &amp;gt;10 (3 points), peak oxygen consumption &amp;lt;16 mL/kg/min (2 points), minute ventilation/carbon dioxide production slope ≥36 (2 points), peak systolic pulmonary artery pressure ≥50 mmHg (1 point) and resting N-terminal pro-brain natriuretic peptide (NT-proBNP) &amp;gt;900 pg/mL (1 point). We defined three risk categories: low-risk (&amp;lt;3 points), intermediate-risk (3-6 points), and high-risk (&amp;gt;6 points). The event-free survival probability for these three groups were 93%, 52% and 20%, respectively. Hazard Ratio was 4.55 for each risk category upgrade (95% confidence interval [CI], 3.44-5.93). The area-under-curve for the scoring system to predict events was 0.92 (95% CI 0.88-0.96). Conclusion. A multiparametric risk score including indices of exercise-induced pulmonary congestion, markers of cardiopulmonary dysfunction and NT-proBNP identifies patients at increased risk for HF events across the HF spectrum. Table 1 Variable EPYC score EPYC score &amp;lt;3 (low risk) n = 217 EPYC score 3-6 (intermediate risk) n = 70 EPYC score &amp;gt;6 (high risk) n = 31 p-value (between risk categories) Event-free (n = 244) 0 (0 - 2) 210 (97) 32 (46) 2 (6) &amp;lt;0.0001 With events (n = 74) 6 (4 - 9) 7 (3) 38 (54) 29 (94) &amp;lt;0.0001 p-value (event-free vs with events) &amp;lt;0.0001 &amp;lt;0.0001 &amp;lt;0.0001 &amp;lt;0.0001 Stage 0-Controls (n = 30) 0 (0 - 1) 30 0 0 &amp;lt;0.0001 Stages A-B (n = 94) 1 (0 - 2) 85 (45) 6 (9) 3 (10) &amp;lt;0.0001 Stage C-HFpEF (n = 85) 3 (1 - 6)*† 46 (25) 29 (41) 10 (32) &amp;lt;0.0001 Stage C-HFrEF (n = 109) 4 (2 - 7)*† 56 (30) 35 (50) 18 (58) &amp;lt;0.0001 p-value (between HF Stages) &amp;lt;0.0001 &amp;lt;0.0001 &amp;lt;0.0001 &amp;lt;0.0001 Values are mean ± standard deviation, n (%), or median [25th quartile, 75th quartile]. * p &amp;lt; 0.01 vs Stage 0-Controls; † p &amp;lt; 0.01 vs Stages A-B. Abstract Figure 1

Predicting the transition to and progression of heart failure with preserved ejection fraction: a weighted risk score using bio-humoural, cardiopulmonary, and echocardiographic stress testing.

Risk stratification of heart failure (HF) patients with preserved ejection fraction (HFpEF) can promote a more personalized treatment. We tested the prognostic value of a multi-parametric evaluation, including biomarkers, cardiopulmonary exercise testing-exercise stress echocardiography (CPET-ESE), and lung ultrasound, in HFpEF patients and subjects at risk of developing HF (HF Stages A and B). Risk stratification of heart failure (HF) patients with preserved ejection fraction (HFpEF) can promote a more personalized treatment. We tested the prognostic value of a multi-parametric evaluation, including biomarkers, cardiopulmonary exercise testing-exercise stress echocardiography (CPET-ESE), and lung ultrasound, in HFpEF patients and subjects at risk of developing HF (HF Stages A and B). We performed a resting clinical/bio-humoural evaluation and a symptom-limited CPET-ESE in 274 patients (45 Stage A, 68 Stage B, and 161 Stage C-HFpEF) and 30 age- and sex-matched healthy controls. During a median follow-up of 18.5 months, we reported 71 HF hospitalizations and 10 cardiovascular deaths. Cox proportional-hazards regression identified five independent predictors and each was assigned a number of points proportional to its regression coefficient: stress-rest ΔB-lines >10 (3 points), peak oxygen consumption <16 mL/kg/min (2 points), minute ventilation/carbon dioxide production slope ≥36 (2 points), peak systolic pulmonary artery pressure ≥50 mmHg (1 point) and resting N-terminal pro-brain natriuretic peptide (NT-proBNP) >900 pg/mL (1 point). The event-free survival probability for low risk (<3 points), intermediate risk (3-6 points), and high risk (>6 points) were 93%, 52%, and 20%, respectively. The area under the curve (AUC) for the scoring system to predict events was 0.92 (95% CI 0.88-0.96), with an accuracy significantly higher than the individual components of the score (all P < 0.01 vs. individual AUCs). A weighted risk score including NT-proBNP, markers of cardiopulmonary dysfunction and indices of exercise-induced pulmonary congestion identifies HFpEF patients at increased risk for adverse events and Stage A and B subjects more likely to progress towards more advanced HF stages.

Abstract 14102: Prognostic Impact of Peak Systolic Pulmonary Arterial Pressure in Nonagenarians: An Echocardiographic Study

Introduction: Recently, a low threshold of estimated peak systolic pulmonary arterial pressure (eSPAP) of 30 mm Hg was reported to be associated with mortality in the general population. However, less is known about the prognostic impact of eSPAP in nonagenarians. Methods: We retrospectively reviewed 596 patients ≥90 years of age referred for echocardiography with a recorded eSPAP and left ventricular ejection fraction ≥50%. eSPAPs were characterized according to their quartile distribution: first quartile (reference group) (&lt;30 mm Hg), second quartile (30-38 mm Hg), third quartile (39-48 mm Hg), fourth quartile (&gt;49 mm Hg). The associations between quartiles of eSPAP and all-cause mortality were tested in Cox models (adjusting for age, sex, body mass index, hypertension, coronary arterial disease, atrial fibrillation, diabetes mellitus, chronic kidney disease, chronic obstructive pulmonary disease, left-sided valve disease, left atrial area, NYHA class ≥II, left ventricular mass and right ventricular fractional area change). Results: Of the 596 patients, 61% were female, and the median age was 92 years (IQR 91-95). The median eSPAP was 39.0 mm Hg (IQR 30.3-49.0) (Figure). During the median follow-up of 522 days (IQR 59-1337), a total of 268 deaths (45%) occurred. The adjusted risk of mortality in eSPAP 39-48 mm Hg and eSPAP &gt;49 mm Hg were 1.6 and 1.7 times higher than in eSPAP &lt;30 mm Hg, respectively (Table). Conclusions: In conclusion, eSPAP ≥39 mm Hg was independently associated with mortality in nonagenarians. Estimated peak systolic pulmonary arterial pressure could help in prognostic stratification even in oldest-old patients.

Prognostic relevance of exercise pulmonary hypertension for new-onset atrial fibrillation in primary mitral regurgitation

Abstract Background/Introduction New-onset of atrial fibrillation (AF) portends poor prognosis in patients with primary mitral regurgitation (MR). However less is known about prognostic indicator for new-onset AF. Purpose The purpose of this study was to identify the prognostic relevance of exercise pulmonary hypertension for the new-onset AF in patients with primary MR. Methods Total of 114 consecutive patients with primary MR who underwent symptom-limited exercise echocardiography using supine-cycle ergometer were followed for new-onset AF over mean follow-up time of 3.6±2.6 years. Those with prevalent AF and pulmonary hypertension (estimated systolic pulmonary artery pressure ≥50mmHg) prior to exercise echocardiography were excluded from our analysis. We defined exercise-induced pulmonary hypertension (ExPHT) as those with peak estimated systolic pulmonary artery pressure (SPAP) ≥60mmHg or delta SPAP defined as differences between rest and peak SPAP ≥20mmHg. Results The mean age was 61±15 years old and 70 (61%) were male. Of those, 8 (7.8%) had mild MR, 32 (31.1%) had moderate MR, and 63 (61.2%) had severe MR. 60 (52.2%) patients had ExPHT. A total of 27 cases of new-onset AF were found during follow-up where the ExPHT group had higher prevalence of new-onset AF than the non-ExPHT group (35% vs. 11%, p=0.002). Those with ExPHT had significantly stronger association with shorter event-free survival time of new-onset AF (log-rank p&amp;lt;0.001, Figure). When adjusted for age, sex, body surface area, the American Society of Echocardiography MR grade, left atrial dimension, peak systolic blood pressure and heart rate, the multivariable Cox regression analysis showed that those with ExPHT had a hazard risk of 3.1 ([95% CI 1.1–9.1], p=0.039) for new-onset of AF. Conclusions Exercise-induced pulmonary hypertension predicted incident of new-onset AF in those with primary MR. Exercise echocardiography is expected to play an important role in decision making with regards to the optimal timing for surgical intervention in primary MR. Figure 1 Funding Acknowledgement Type of funding source: None

Characteristics and Prognostic Associations of Echocardiographic Pulmonary Hypertension With Normal Left Ventricular Systolic Function in Patients ≥90 Years of Age

The high prevalence of pulmonary hypertension (PH) in elderly patients is well known. However, much remains unknown about those population. We sought to find the clinical characteristics of echocardiographic PH and the prognostic factors in patients ≥90 years of age. We retrospectively reviewed 310 patients ≥90 years of age (median age 92 years, 64% women) diagnosed as echocardiographic PH (peak systolic pulmonary arterial pressure ≥40 mm Hg) with normal left ventricular systolic function. We defined left heart disease (LHD) as significant left-sided valve diseases, left ventricular hypertrophy and left ventricular diastolic dysfunction by using echocardiography. The endpoint was all-cause death at 2,000 days after diagnosis. LHD was found in 92% of patients. During the median follow-up of 367 days (interquartile range, 39-1,028 days), 151 all-cause deaths (49%) occurred. Multivariable Cox regression analysis demonstrated that right ventricular fraction area change <35% (adjusted hazard ratio [HR]: 2.31; p <0.001), pericardial effusion (adjusted HR: 2.28; p <0.001), serum albumin <3.5 g/dL (adjusted HR: 1.76; p = 0.001), chronic obstructive pulmonary disease (adjusted HR: 1.93; p = 0.001) and New York Heart Association (NYHA) class ≥II (adjusted HR: 1.73; p = 0.004) were associated with mortality after adjusted for age. In conclusion, LHD was significantly associated with echocardiographic PH in most patients ≥90 years of age. Also, the co-morbid factors at diagnosis (right ventricular systolic dysfunction, pericardial effusion, hypoalbuminemia, chronic obstructive pulmonary disease, and NYHA class ≥II) were independently associated with mortality.

Exercise behavior of degenerative mitral stenosis.

Mitral annular calcification (MAC) is increasingly encountered, particularly among the elderly and those with chronic kidney disease, and is often associated with a transvalvular gradient. In contrast to rheumatic mitral stenosis relatively little is known about mitral stenosis due to MAC. We aimed to clarify whether exercise limitation in this group is primarily due to valvular obstruction or ventricular dysfunction resulting from multiple comorbidities. 20 patients with severe MAC (bulky calcium deposits which restricted leaflet motion) were submitted to supine bicycle exercise, measuring Doppler and echocardiographic parameters at baseline and during exercise. They were compared 1:1 to subjects matched for age, sex, and left ventricular wall thickness. At baseline MAC subjects had higher mean mitral valve gradients (MVG) than comparison subjects (7.5 ± 3.8 vs 1.6 ± 0.8mm Hg, p < 0.0001), along with larger indexed left atrial volumes (54.4 ± 14.9 vs 34.0 ± 11.7mL, p < 0.0001) and reduced left atrial strains (reservoir, conduit, and booster pump). With exercise MAC subjects reached higher levels of MVG (17.3 ± 8.4 vs 5.5 ± 2.5mm Hg, p < 0.0001), and pulmonary artery systolic pressure (estimated from tricuspid regurgitant jet [TR] velocity) and displayed a moderate correlation between ΔMVG and ΔTR velocity (r2 = 0.57). MAC subjects whose exercise MVG was ≥ 15mm Hg all had a peak pulmonary artery systolic pressure > 60mm Hg. MAC subjects also had relative chronotropic incompetence. Patients with severe MAC and a transvalvular gradient experience large increases in MVG and pulmonary pressure with exercise, similar to what has been described in rheumatic mitral stenosis. MAC may be an under-recognized cause of dyspnea and exercise intolerance in older patients.

Estimated peak systolic pulmonary artery pressure in young non-complicated patients with type 1 diabetes.

Right ventricle and pulmonary artery pressure have always received less attention in type 1 diabetes than left ventricle. The aim of this study is to compare the right heart performance and the estimated peak systolic pulmonary artery pressure (EPSPAP) in young type 1 diabetes patients with healthy controls. Subjects affected by type 1 diabetes without cardiovascular and respiratory diseases (n=93) and healthy controls (n=56) were evaluated with a comprehensive transthoracic echocardiography. The pulmonary peak systolic arterial pressure was calculated with an established formula based on pulmonary artery acceleration time. The left ventricle's function was found to be normal in all the subjects under study. The estimated peak systolic pulmonary artery pressure was significantly higher in patients with type 1 diabetes compared to the controls (38.5 ± 8.6 vs. 35.4 ± 6.7, p = 0.019). The highest value of EPSPAP was observed in smoking female patients with type 1 diabetes. Basal and mid cavity diameter of the right ventricle were higher in patients with type 1 diabetes. Factors associated with EPSPAP were sex, body mass index, mid cavity diameter and, with an inverse correlation, HDL-cholesterol. The present study suggests that young, uncomplicated patients with type 1 diabetes have a higher estimated peak systolic pulmonary artery pressure. Further studies are needed to define the mechanisms underlying this alteration and its clinical consequences.

P1737 Echocardiographic estimation of pulmonary artery pressure in young non-complicated patients with type 1 diabetes: results from a single-center observational study

Abstract OBJECTIVE Right ventricle and pulmonary artery pressure have received less attention in type 1 diabetes compared to the left ventricle. The aim of the present study was to compare right heart performance and the estimated peak systolic pulmonary artery pressure in young non complicated patients with type 1 diabetes with healthy controls. RESEARCH DESIGN AND METHODS Ninety-three patients with type 1 diabetes without cardiovascular and respiratory diseases were recruited and compared to 56 healthy controls. All subjects were evaluated with a comprehensive trans-thoracic echocardiography and pulmonary peak systolic arterial pressure was estimated with an established formula based on pulmonary artery acceleration time. RESULTS None of the subjects showed alterations of either systolic or diastolic function of the left ventricle. The estimated peak systolic pulmonary artery pressure was significantly higher in patients with type 1 diabetes compared to controls (38.5 ± 8.6 vs 35.4 ± 6.7, p = 0.019). The highest value of EPSPAP was observed in smoker female patients with type 1 diabetes. Basal and mid cavity diameter of the right ventricle were higher in patients with type 1 diabetes. In the multivariate analyses, factors associated with EPSPAP were female sex, body mass index, mid cavity diameter and, with an inverse correlation, HDL cholesterol. CONCLUSIONS The present study suggests that young, uncomplicated patients with type 1 diabetes have a higher estimated peak systolic pulmonary artery pressure. Future studies are needed to better define the mechanisms underlying this alteration and its clinical consequences.

Pulmonary hypertension is associated with increased nonrelapse mortality after allogeneic hematopoietic cell transplantation for myelofibrosis.

Allogeneic hematopoietic cell transplantation (alloHCT) is the only curative therapy for primary myelofibrosis (MF) as well as myelofibrosis secondary to other myeloproliferative neoplasms (MPN). Pulmonary hypertension (PH) is a known complication of MF and may occur in up to 50% of such patients. PH (defined as a mean pulmonary artery pressure ≥25 mmHg at rest) can eventually lead to right heart failure and may be associated with complications after alloHCT. We examined the association of PH with alloHCT outcome in patients with MF associated with MPN. Pre- and post-HCT echocardiograms were reviewed to estimate the peak pulmonary artery systolic pressure (PASP). Median PASP was 37.0 mmHg (range: 16.0-57.9) prior to HCT with 37 of 65 patients (57%) studied. With median follow-up of 35.0 months (range: 3.3-119.4) PH was significantly associated with inferior OS (58.9% vs. 88.8%, P = 0.025), primarily due to increased NRM (21.6% vs. 7.1%, P = 0.007). The majority of the deaths (8 of 14) in patients with PH occurred within 100 days after HCT. In patients with an available post-HCT echocardiogram (n = 33), the median PASP was 30 mmHg (range: 5.0-56.2); eight patients (24%) had persistent PH. Compared with pre-HCT values, PASP was significantly reduced after HCT (p < 0.001). We conclude that PH is associated with inferior survival due to the increased NRM in patients with MF undergoing alloHCT. PH appears at least partially reversible after successful alloHCT. PH should be considered a risk factor for early mortality after alloHCT and surveillance of pulmonary artery pressure in MF patients being considered for alloHCT may be useful.

Sick Sinus Syndrome After the Maze Procedure Performed Concomitantly With Mitral Valve Surgery.

BackgroundTo characterize the development of sick sinus syndrome (SSS) after the additive maze procedure (MP) during mitral valve surgery.Methods and ResultsFollow‐up data (median, 3.6 years) of 750 patients with a prevalence of rheumatic cause of 57.6% were analyzed. SSS occurred in 35 patients with a time‐dependent increase: the incidence rates at 1, 2, and 4 years after surgery were 2.9%, 3.7%, and 4.3%, respectively. The additive MP showed higher risks of SSS development (hazard ratio, 7.44; 95% confidence interval, 3.45–16.05; P<0.001) and pacemaker implantation (hazard ratio, 3.61; 95% confidence interval, 1.95–6.67; P<0.001). Patients who developed SSS showed higher 4‐year rates of clinical events (death, stroke, and hospital admission) (67.5±8.5% versus 33.0±1.9%; P<0.001). After adjustment for age and preoperative peak systolic pulmonary artery pressure, the lesion extent (biatrial versus left atrial MP), not the underlying cause (rheumatic versus nonrheumatic), was independently associated with SSS development (hazard ratio, 3.58; 95% confidence interval, 1.08–11.86; P=0.037). The adverse effect of the biatrial MP was confirmed in patients with trivial or mild preoperative tricuspid regurgitation showing higher SSS incidence (4.6±1.4% versus 1.0±0.7%; P=0.023), not in those with moderate‐to‐severe tricuspid regurgitation (6.8±1.7% versus 3.8±3.8%; P=0.337). Recurrence of atrial fibrillation was not associated with the lesion extent of the MP.ConclusionsAfter the additive MP, the ongoing risk of SSS development should be acknowledged irrespective of the underlying cause. Considering additive risk of biatrial MP with similar atrial fibrillation recurrence rate, minimizing lesion extent is warranted.

Impact of right ventricular dysfunction and end-diastolic pulmonary artery pressure estimated from analysis of tricuspid regurgitant velocity spectrum in patients with preserved ejection fraction.

We aimed to analyse the association between right haemodynamic parameters, right ventricular (RV) dysfunction parameters, and outcomes in patients with preserved ejection fraction (EF). Retrospective analysis of right haemodynamic (systolic pulmonary pressure and end-diastolic pulmonary pressure based on tricuspid regurgitation (TR) velocity at pulmonary valve opening time), and RV parameters including size (end-diastolic and end-systolic area), function (RV fractional area change, Tei index, Tricuspid Annular Plane Systolic Excursion, and speckle tracking derived free wall strain), from 557 consecutive patients with preserved EF [EF ≥ 50%; age 64.9 + 20; 52% female; co-morbidity Charlson index 4.7 (2.9, 6.4)]. All cause and cardiac mortality were retrospectively analysed and correlated to echo haemodynamic and co-morbid parameters. TR velocity at pulmonary valve opening time and calculated end-diastolic pulmonary artery pressure were obtainable in 71% of patients. The best haemodynamic univariate predictor of mortality was calculated end-diastolic pulmonary artery pressure [hazard ratio 1.06 (1.04-1.07); P < 0.0001], superior to TR peak velocity and systolic pulmonary artery pressure. Elevated end-diastolic pulmonary artery pressure was associated with all cause and cardiac mortality even when adjusted for all significant clinical (age, gender, and Charlson index), and echo (stroke volume index, left atrial volume index, systolic pulmonary pressure, E/e', and Tei index) parameters. Tei index was superior to all other RV functional parameters (P < 0.05 for all parameters). TR velocity at pulmonary valve opening time and calculated end-diastolic pulmonary artery pressure are obtainable in most patients, and add prognostic information on top of clinical and routine haemodynamic and diastolic parameters.

PP-456 - An Unusual Lutembacher Syndrome

Lutembacher syndrome is defined as a combination of mitral stenosis (MS) and left-to-right shunts at the atrial level. However, the definition of this syndrome has undergone many changes. Although it was described initially as a combination of congenital atrial septal defect (ASD) and acquired rheumatic MS; the definition of Lutembacher syndrome has undergone many changes. Currently, any combination of ASD, congenital or iatrogenic, and MS, congenital or acquired, is referred as Lutembacher syndrome. We report an unusual case of Lutembacher Syndrome that consisted of secundum-type ASD and acquired MS caused by left atrial myxoma in a 45-old woman who presented with a syncopal attack. Transthoracic two dimensional echocardiography in apical 4-chamber view revealed a large left atrial mass attached to the interatrial septum prolapsing into left ventricle (Figure 1A, Video 1). Color Doppler echocardiography showed simultaneously secundum-type ASD and color flow mapping of the proximal isovelocity surface area (PISA) reflecting MS (Figure 1B, Video 2), which is confirmed by also continuous-wave Doppler measurements with mean transmitral gradient of 6 mm Hg and peak systolic pulmonary artery pressure of 75 mm Hg. Successful excision of the mass, which proved to be a myxoma, and closure of the ASD completely. The patient made an uneventful recovery and was discharged 4 days later.

Does patient-prosthesis mismatch have a negative impact on outcomes following mitral valve replacement?

A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was whether patient-prosthesis mismatch (PPM) has a negative impact on patients undergoing mitral valve replacement in terms of postoperative mortality, incidence of postoperative pulmonary hypertension (PH) and higher transmitral gradients. Altogether 103 papers were found using the reported search, 18 of which represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated. Overall, 8 studies with a total of 4812 patients reported that PPM has a significantly negative impact on long-term mortality, whereas 5 studies with a total of 1558 patients reported no effect on mortality. One study with a total of 2440 patients reported preoperative PH as the risk factor for increased mortality in the presence of moderate or severe PPM. Three studies evaluated the effect of PPM on postoperative PH and reported that PPM was associated with significantly increased postoperative PH. The majority of the studies reported that PPM was associated with higher peak or mean transmitral gradient and systolic pulmonary artery pressure. The results presented in these studies suggest that PPM in patients undergoing mitral valve replacement was associated with increased postoperative mean and peak transmitral gradient and higher postoperative systolic pulmonary artery pressure. PPM may be associated with increased long-term mortality. Severe PPM was directly associated with increased long-term mortality when compared with moderate or no PPM. Evidence suggests that PPM is associated with increased incidence of postoperative PH.

Prognostic Value of Exercise-Stress Echocardiography in Asymptomatic Patients With Aortic Valve Stenosis

ObjectivesThis study sought to evaluate the prognostic value of mean pressure gradient (MPG) increase and peak systolic pulmonary artery pressure (SPAP) measured during exercise stress echocardiography in asymptomatic patients with aortic stenosis (AS). BackgroundExercise testing is recommended in asymptomatic AS patients, but the additional value of exercise-stress echocardiography, especially the prognostic value of MPG increase and peak SPAP, is still debated. MethodsWe enrolled all consecutive patients with pure, isolated, asymptomatic AS and preserved ejection fraction ≥50% and normal SPAP (<50 mm Hg) who underwent symptom-limited exercise echocardiography at our institution. Occurrence of AS-related events (symptoms or congestive heart failure) or occurrence of aortic valve replacement was recorded. ResultsWe enrolled 148 patients (66 ± 15 years of age; 74% males; MPG: 47 ± 13 mm Hg; SPAP: 34 ± 6 mm Hg). No complications were observed. Thirty-six patients (24%) had an abnormal exercise test result (occurrence of symptoms, fall in blood pressure, and/or ST-segment depression) and were referred for surgery. Among the 112 patients with a normal exercise test result, 38 patients (34%) had abnormal exercise echocardiography scores (MPG increase >20 mm Hg and/or SPAP at peak exercise >60 mm Hg). These 112 patients were managed conservatively. During a mean follow-up of 14 ± 8 months, an AS-related event occurred in 30 patients, and 25 patients underwent surgery. Neither MPG increase >20 mm Hg nor peak SPAP >60 mm Hg was predictive of occurrence of AS-related events or aortic valve replacement (all p > 0.20). In contrast, baseline AS severity was an important prognostic factor (all p < 0.01). ConclusionsIn this observational study including 148 patients with asymptomatic AS, we confirmed and extended the importance of exercise testing for unveiling functional limitation. More importantly, neither the increase in MPG nor in SPAP at peak exercise was predictive of outcome. Our results do not support the use of these parameters in risk-stratification and clinical management of asymptomatic AS patients.

0055 : Pulmonary hypertension and transcatheter aortic valve implantation: prevalence, prognosis impact and evolution

In patients undergoing transcatheter aortic valve implantation, measurement of pulmonary pressure may help to stratify the clinical risk. However, data may lead to patient misclassification and the role of pulmonary vascular resistance which include cardiac output has never been investigated. One hundred and seventy one consecutive patients with significant symptomatic aortic stenosis (aortic valve area <1 cm2or 0.6 cm2m2 who prospectively were scheduled for transaortic valve implantation, underwent preoperative right-sided heart catheterization and quantitative Doppler echocardiography. Of these, 99 (57.9%) had pulmonary hypertension [invasive mean pulmonary artery pressure (PAP) ≥25mm Hg] and 40 experienced cardiac events during a 1-year follow-up (readmission for heart failure in 16 patients, sudden and non-cardiac death in 24). In univariate analysis, patients who had cardiac events exhibited a higher both peak systolic PAP (46.9±12.1 versus 40.8±12,0mm Hg; P=0.026) and transpulmonary pressure gradient (12.6±4.5 versus 10.1±3,7mm Hg; P=0.011), as well as increased pulmonary vascular resistance (PVR, 2.7±1.0 vs. 2.0±0.8 WU, p = 0.002). Peak systolic PAP>40mm Hg and PVR>2.05 WU were selected by receiver operating curve for predicting cardiac events with the best sensitivity and specificity. By multivariate Cox regression analysis, independent predictors of cardiac events were as follows: body mass index (P=0.005), a mitral regurgitation (P=0.018), and a spectrum of peak systolic PAP>40mm Hg with simultaneous PVR>2.05 WU compared with other patients [2.6 (1.39-4.89), p=0.003]. Right heart catheterization could be useful to identify a highrisk subset of aortic stenosis patients candidate for transcatheter aortic valve implantation and help for clinical decision making.

Echocardiographic features of arrhythmogenic right ventricular cardiomyopathy: analysis of misdiagnosis and miss diagnosis

Objective To evaluate the value of echocardiography on the diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC), and to improve the diagnositic accuracy of ARVC by echocardiography. Methods According to the 2010 European Heart Association guideline, twenty-one patients with ARVC were diagnosed from September 2003 to June 2014. The patients were divided into four groups (confirmed, suspiciously diagnosis, miss diagonisis, misdiagnosis) and the echocardiographic features were retrospectively analyzed including the right ventricular (RV) movement, the diameter of RV outflow tract (RVOTd), fractional area change of RV (RVFAC), the severity of tricuspid regurgitation (TR) and peak pulmonary artery systolic pressure (PASP). Results Of 21 patients, 15 (71.4%) were confirmed by echocardiography, which had the typical ARVC echocardiographic features including the hypokinetic, akinetic or aneurysm of RV, dilation of RVOTd [mean RVOTd (40±3)mm], and RV FAC<33% [mean (21±7)%]. TR were noticed in all the 15 patients but the PASP were normal [mean (27±9)mmHg, 1mmHg=0.133 kPa]. Three (14.3%) were suspiciously diagnosed which had the RV wall hypoakinetic, 1 with pure RVOTd dilation and 2 with RV and RVOTd dilation, all 3 patients had mild TR, 33%<RVFAC≤40% and PASP were in normal range. Two patients had normal echocardiography which was miss diagnosed, one patient was misdiagnosed as dilated cardiomyopathy. Conclusions The different stages of ARVC patients had different echocardiographic features, the patients were easily diagnosed when the ARVC patients in RV failure stage. But for the early and late stage, the diagnosis should combine the clinical manifestation and other imagingfacilities to avoid miss diagnosis and misdiagnosis. Key words: Echocardiography; Arrhythmogenic right ventricular dysplasia; Diagnostic errors