scholarly journals P957 Prognostic value of pulmonary artery elastic properties in patients with pulmonary hypertension - a comparison of Eisenmenger syndrome to other types of pulmonary hypertension

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
R Enache ◽  
D N Radu ◽  
R Badea ◽  
L Predescu ◽  
P Platon ◽  
...  

Abstract Patients with Eisenmenger’s syndrome (ES) have better survival than other patients with pulmonary arterial hypertension (PAH) probably due to the preservation of right ventricular (RV) function. As in PAH patients RV remodeling and function depend not only on pulmonary artery (PA) pressure but also on the intrinsic properties of PA wall, there is also a possible role of PA stiffness (PAS) as outcome predictor in this setting. Purpose. To study the prognostic role of PAS parameters assessed by 2D transthoracic echocardiography in patients with ES compared to other patients with pulmonary hypertension (PH) receiving specific vasodilator therapy. Methods. Sixty-eight PH patients were enrolled: 27 ES patients and 41 non-ES patients, including patients with other types of PAH (12 idiopathic PAH, 5 operated congenital heart disease, 10 connective tissue disease, 7 other forms of PAH) or chronic thromboembolic PH (7 patients) receiving oral vasodilator therapy. Clinical data, B-type natriuretic peptide (BNP), RV function and PAS parameters were assessed: pulmonary capacitance (PC), PC indexed to body surface area (PC/BSA), pulsatility, elastic modulus (EP), beta-index. PH patients were followed-up for 2.9 years (4 months-6.8 years). Results. Pulmonary vascular resistance (PVR) assessed by right heart catheterization was similar in both groups (11.9 ± 8.0 vs 11.0 ± 6.4 Wood units, p = 0.68). ES patients had lower BNP levels (lnBNP 3.63 ± 1.31 vs 5.31 ± 1.33, p < 0.001) and better RV function than non-ES patients: RV-free wall S wave, RV-S (12.2 ± 2.3 vs 10.2 ± 2.0 cm/s, p < 0.001), RV fractional area change, RV-FAC (40 ± 7 vs 32 ± 9%, p < 0.001), RV global longitudinal strain (RV-GLS) on 3 segments (-20.2 ± 4.4 vs -14.8 ± 6.0%, p = 0.001) or 6 segments (-16.2 ± 4.2 vs -13.1 ± 4.9%, p = 0.011). In ES patients PAS parameters were less impaired than in non-ES group (PC 1.68 ± 0.86 vs 1.18 ± 0.66 ml/mmHg, p = 0.014; PC/BSA 1.05 ± 0.53 vs 0.68 ± 0.37 ml/mmHg m2, p = 0.003; pulsatility 18.8 ± 8.4 vs 13.8 ± 6.4%, p = 0.007, EP 390.7 ± 198.6 vs 578.8 ± 341.6 mmHg, p = 0.007; beta index 6.09 ± 2.85 vs 10.77 ± 6.21, p < 0.001). During follow-up, 12 cardiac deaths occurred: 1 in ES group and 11 in non-ES group (p = 0.021). In non-ES group, predictors of cardiac death were parameters of RV function and PAS: BNP levels (lnBNP 6.20 ± 1.10 in deceased patients vs 4.97 ± 1.27 in survivors, p = 0.007), RV-S (9.1 ± 2.0 vs 10.6 ± 1.9 cm/s, p = 0.038), RV-FAC (25 ± 8 vs 35 ± 7%, p = 0.001), RV-GLS on 3 segments (-11.1 ± 4.4 vs -16.2 ± 6.0%, p = 0.015) or 6 segments (-9.0 ± 3.7 vs -14.6 ± 4.4%, p = 0.001), PC (0.86 ± 0.29 vs 1.32 ± 0.72 ml/mmHg, p = 0.01; PC/BSA (0.51 ± 0.17 vs 0.76 ± 0.41 ml/mmHg m,2 p = 0.013). Conclusion: Patients with ES have better RV function and less impaired PAS compared to patients with other types of PH and similar PVR. Moreover, besides RV function, PAS parameters emerged as predictors of cardiac death in non-ES patients that had worse prognosis than ES patients. The impact of these findings on clinical outcomes in ES patients remains to be further studied.

2018 ◽  
Vol 8 (2) ◽  
pp. 204589401877305 ◽  
Author(s):  
Batool AbuHalimeh ◽  
Milind Y. Desai ◽  
Adriano R. Tonelli

The diagnosis of pulmonary hypertension (PH) requires a right heart catheterization (RHC) that reveals a mean pulmonary artery pressure ≥ 25 mmHg. The pulmonary artery catheter traverse the right atrium and ventricle on its way to the pulmonary artery. The presence of abnormal right heart structures, i.e. thrombus, vegetation, benign or malignant cardiac lesions, can lead to complications during this procedure. On the other hand, avoidance of RHC delays the diagnosis and treatment of PH, an approach that might be associated with worse outcomes. This paper discusses the impact of right heart lesions on the diagnosis of PH and suggests an approach on how to manage this association.


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Fusako Sera ◽  
Tomohito Ohtani ◽  
kei nakamoto ◽  
Shungo Hikoso ◽  
Daisaku Nakatani ◽  
...  

Introduction: The proposed revision of hemodynamic definition of pulmonary hypertension (PH) adopts a lower threshold of mean pulmonary artery pressure (mPAP) > 20 mmHg. In addition, pulmonary vascular resistance (PVR) ≥ 3 Wood units (WU) is included as the definition of pre-capillary component of PH. Heart failure (HF) with preserved ejection fraction (HFpEF) can develop pre-capillary PH as well as post-capillary PH. We aimed to investigate the impact of the proposed definition of PH on clinical diagnosis of PH associated with HFpEF. Methods: From the PURSUIT-HFpEF (Prospective Multicenter Observational Study of Patients with Heart Failure with Preserved Ejection Fraction) registry, 225 patients who were hospitalized with HF and underwent right heart catheterization were categorized according to the current guidelines and the proposed definition of PH: non-PH, isolated post-capillary PH (Ipc-PH), pre-capillary PH, and combined pre- and post-capillary PH (Cpc-PH). In the proposed definition, patients with mPAP > 20 mmHg, PVR < 3 WU, and pulmonary artery wedge pressure ≤ 15 mmHg do not meet criteria for any of the above categories and are categorized as “unclassified PH”. Results: Prevalence of PH was significantly increased in the proposed definition compared to that in the current definition (51% vs 29%, p<0.0001), with a doubled frequency of pre-capillary PH (Fig A). Furthermore, 24 patients (11%) were diagnosed as unclassified PH and accounted for 22% of those with PH by the proposed definition. Among the PH categories in the proposed definition, Cpc-PH category was significantly relevant for worse prognosis at 1 year after discharge in patients with HFpEF (p=0.03 vs non-PH by log-rank test with Bonferroni's correction) (Fig 2). Conclusions: The new definition of PH resulted in a remarkable increase of prevalence of PH in HFpEF with a quite a few patients with unclassified PH and doubled frequency of pre-capillary PH.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
B Koell ◽  
S Ludwig ◽  
O Bhadra ◽  
A Gossling ◽  
N Schofer ◽  
...  

Abstract Background Pulmonary hypertension (PH) due to left heart disease is the most common form of PH. Published literature suggests increased perisurgical mortality in patients undergoing surgical repair in the setting of preexisting PH. The data on the impact of preexisting PH on clinical outcomes after percutaneous Mitral Valve Edge-to-Edge Repair (pMVR) is limited to observational studies and rely mostly on echocardiographic data. Purpose The aim of the current study is to evaluate the influence of preexisting PH in patients undergoing pMVR analyzing periprocedural invasive right heart catheterization data. Methods Between September 2008 and July 2018, a total of 911 patients with moderate-to-severe or severe mitral regurgitation (MR) underwent pMVR at our center. This analysis includes 331 patients with a complete data set for pre- and postprocedural right heart catheterization and echocardiographic assessment as well as available follow-up information after the implantation. Patients are divided according to the etiology of PH. The combined primary endpoint consists of all-cause mortality and rehospitalization for heart failure. Furthermore, a sub-analysis is performed for all patients with preexisting post-capillary PH. Patients with post-capillary PH are divided into two groups based on a postprocedural decrease of pulmonary artery wedge pressure (mPAWP) below the threshold of 15mmHg. Univariate and multivariate Cox regression analyses are performed to assess the influence on long-term outcome. Results Of all 331 patients (57.7% [n= 191] male) undergoing pMVR, 195 (62.1%) had functional MR. Median ejection fraction was 40.5% (29.3, 54.0). Patients were followed-up for a maximum of 4.41 years and the median follow-up time was 1.98 years. Preexisting PH (mean pulmonary artery pressure ≥25 mmHg) was found in 236 (71.1%) patients: 49 patients had pre-capillary PH (≤15 mmHg), 187 had post-capillary PH (pcPH; n=183; mPAWP &gt;15 mmHg). In Kaplan-Meier analysis, no statistically significant difference could be found in overall mortality in patients without or with PH, irrespective of etiology (p=0.43). However, in patients suffering from post-capillary PH, patients with a postprocedural reduction of mPAWP below the threshold of 15mmHg showed a significantly lower risk for overall long-term mortality compared to patients without a relevant mPAWP reduction (p=0.018). Multivariate analysis revealed acute postprocedural decrease of mPAWP below 15mmHg in patients with post-capillary PH to have a significant influence on mortality (HR 2.81 [1.35, 5.86]; p=0.006; Figure 1). Conclusion In contrast to previously published findings, the present results were not able to show a significant impact of PH, disregarding its etiology, on outcome. Nevertheless, a postprocedural decrease of mPAWP below 15mmHg in patients with post-capillary PH is associated with a favorable outcome. Figure 1 Funding Acknowledgement Type of funding source: None


2021 ◽  
Vol 8 ◽  
Author(s):  
Weichun Wu ◽  
Bingyang Liu ◽  
Min Huang ◽  
David H. Hsi ◽  
LiLi Niu ◽  
...  

Background: Four-dimensional automatic right ventricular quantification technology (4D auto-RVQ) is a new method that can simultaneously measure right ventricular (RV) structure and strain. The role of 4D auto-RVQ in determining RV function and hemodynamics is not clear. The role of 4D auto-RVQ in determining RV function and hemodynamics is not clear. We assessed the 4D auto-RVQ to measure right heart structure, function, and hemodynamics in patients with pulmonary hypertension (PHTN) correlated with right heart catheterization (RHC).Methods: We enrolled a prospective cohort of 103 patients with PHTN and 25 healthy controls between September 2017 and December 2018. All patients with PHTN underwent echocardiography and RHC. Patients were included if they underwent two-dimensional (2D) and 4D auto-RVQ echocardiographic sequences on the same day as RHC. We analyzed RV functional indices using 2D and 4D auto-RVQ analyses. We divided patients with PHTN into three groups according to echocardiographic image quality as follows: high (n = 24), average (n = 48), and poor (n = 4). Hemodynamic parameters were measured using RHC, including mean right atrial pressure, mean pulmonary arterial pressure, RV cardiac index (RV-CI), and pulmonary vascular resistance.Results: There were significant differences in most 2D and 4D auto-RVQ parameters between patients with PHTN and healthy controls. Interobserver variability showed significant agreement with 4D auto-RVQ for most measurements except for 4D end-diastolic volume. Indices measured by auto 4D-RVQ in the high-quality image group had a good correlation with RHC but not in the average- and poor-quality image group. Mid-RV diameter showed the best predictive power for the right RV-CI [area under the curve (AUC) 0.935; 95% confidence interval (CI), 0.714–0.997; p &lt; 0.001]. RV end-systolic volume &gt;121.50 mL had a 71.43% sensitivity and a 100% specificity to predict right RV-CI (AUC, 0.890; 95% CI, 0.654–0.986; p &lt; 0.001).Conclusions: 4D auto-RVQ may be used to estimate RV function and some hemodynamic changes compared with RHC in PHTN patients with high image quality. Furthermore, a large sample of the study is needed to evaluate RV function by 4D auto-RVQ in PHTN patients with average image quality.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
C Y Huang ◽  
C K Wu ◽  
L Y Lin

Abstract Introduction The pathophysiology of HFpEF is complicated and treatments that improve outcomes in HFrEF have no substantial benefits in HFpEF. Impairments in LV global longitudinal strain (GLS) and LV diastolic function parameters have been regarded as novel echocardiographic markers to predict cardiovascular events in HFpEF. Preliminary trials indicate that iloprost, an inhaled prostacyclin analougue, improves exercise hemodynamics in both patients with pulmonary artery hypertension and pulmonary hypertension associated with HFpEF. Purpose We aim to investigate the impact of inhaled iloprost on LV global longitudinal strain, LV diastolic function and RV function during exercise in HFpEF population. Methods 34 patients diagnosed with HFpEF were included with exclusion of severe pulmonary, coronary artery, valvular heart and pericardial diseases. Subjects were randomized 1:1 to inhalation of iloprost or placebo. Transthoracic echocardiography was performed at rest and after 6-minute supine bicycle exercise at 20-Watt workload. We utilized tissue speckle tracking by QLAB software to derive LV GLS and global strain rate during LV isovolumetric relaxation period (SRIVR). E/e', E/SRIVR, tricuspid regurgitation pressure gradient (TRPG), tissue Doppler imaging of RV lateral tricuspid annulus and tricuspid annular plane systolic excursion (TAPSE) were measured to evaluate changes of myocardial performance. Results LV GLS during exercise significantly enhanced after iloprost use. E/SRIVR, a novel predictor of LV filling pressure, was significantly decreased. E/e' was also lower in iloprost group by trend. Improvements in RV function and degree of pulmonary hypertension during exercise with iloprost were observed in this study. Echocardiographic myocardial function endpoints during 20-Watt excercise between iloprost and placebo groups Iloprost (N=17) Placebo (N=17) P value LV global longitudinal strain, % −4.96±1.20 −0.75±3.00 <0.001 E/SRIVR, cm 206.41±113.55 454.13±262.98 0.005 E/e' mean 10.77±2.39 14.41±12.14 0.235 TAPSE, cm 2.62±0.62 2.37±0.56 0.221 TRPG, mmHg 28.37±12.47 44.25±9.29 <0.001 TDI lateral tricuspid annulus, cm/s 16.41±4.61 14.77±2.92 0.222 Effects of iloprost at rest and exercise Conclusions Our study is the first to demonstrate that, in patients with HFpEF, inhaled iloprost favorably enhances LV GLS reserves, decreases LV diastolic filling load, reduces pulmonary hypertension and thereby improves RV function during exercise. Acknowledgement/Funding This work was supported in part by the National Science Council of the Republic of China, Taiwan (NSC107-2314-B-002-265-MY3)


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
R Enache ◽  
D N Radu ◽  
R Badea ◽  
L Predescu ◽  
P Platon ◽  
...  

Abstract In patients with pulmonary hypertension (PH) right ventricular (RV) function is a well-recognized determinant of clinical outcome, including cardiac death. Data regarding the prognostic value of comorbidities in patients with PH are scarce. Purpose. To identify predictors of cardiac death and all-cause mortality on mid-term follow-up in PH patients receiving specific vasodilator therapy. Methods. Sixty-eight patients (40 ± 16 years, 51 women) with PH (12 with idiopathic pulmonary arterial hypertension-PAH, 32 with congenital heart disease, 10 ith connective tissue disease, 7 with chronic thromboembolic PH and 7 with other forms of PAH) treated with pulmonary vasodilators were studied. Clinical parameters (NYHA class, 6-minutes walking distance, 6MWD), biological (hemoglobin, B-type natriuretic peptide-BNP), and echocardiographic parameters of RV function (TAPSE, tissue-Doppler imaging-derived free-RV wall S- and e’-wave, RV fractional area change (FAC), Tei index, I/H, RV isovolumic acceleration-IVA) and cardiac index were assessed. RV global longitudinal strain (RV-GLS) was measured from the apical 4-chamber view as the average of 3 (free-wall) and 6 (free-wall and interventricular septum) segments by 2D speckle-tracking echocardiography. Parameters of pulmonary artery stiffness (PAS) were assessed: pulsatility, capacitance, elastic modulus, dynamic compliance. Two endpoints were defined: cardiac death and all-cause mortality. Results. PH patients were followed-up for 35 months (4-81). Comorbidities were identified in 15 patients (non-cardiac surgery, anemia, chronic kidney disease, neoplasia, respiratory infections). During follow-up, 20 patients died, 14 deaths being cardiac deaths. At univariable analysis, the following parameters correlated with all-cause mortality: age (51 ± 14 in deceased patients vs 36 ± 15 years in surviving patients, p &lt; 0.001), 6MWD (262 ± 171 vs 369 ± 155 m, p = 0.015), FAC (30 ± 9 vs 38 ± 8%, p &lt; 0.001), 6 segments RV-GLS (-12.3 ± 5.1 vs -15.1 ± 4.5%, p = 0.025), S-RV (9.9 ± 2.2 vs 11.4 ±2.3 cm/s, p = 0.016), TAPSE (16.3 ± 3.3 vs 18.6 ± 3.5 mm, p = 0.014), right atrial (RA) area (30.6 ± 13.1 vs 21.2 ± 8.8 cm2, p = 0.001) hemoglobin (12.9 ± 2.6 vs 15.2 ± 3.0 g/dl, p = 0.004) and BNP levels (lnBNP, 5.8 ± 1.3 vs 4.1 ± 1.4, p &lt; 0.001), presence of comorbidities (10 vs 5, p = 0.001). At multivariable analysis, only comorbidities (p = 0.05) and FAC (p = 0.07) seemed to independently predict all-cause mortality. Predictors of cardiac death at univariate analysis were age, RV function parameters, RA area, PA capacitance (1.02 ± 0.56 vs 1.48 ± 0.80 ml/mmHg, p = 0.05) and cardiac index (2.48 ± 0.64 vs 3.31 ± 1.34 l/min/m2, p = 0.03) but only age (p = 0.03) and cardiac index (0.06) emerged as independent predictors of cardiac death. Conclusions: In PH patients, RV function and comorbidities predict all-cause mortality, while age and cardiac index are independent determinants of cardiac death. Identifying and properly treating associated pathologies could impact survival in this setting.


2013 ◽  
Vol 135 (2) ◽  
Author(s):  
David Schreier ◽  
Timothy Hacker ◽  
Gouqing Song ◽  
Naomi Chesler

Pulmonary arterial hypertension (PAH) is a rapidly fatal disease in which mortality is typically due to right ventricular (RV) failure. An excellent predictor of mortality in PAH is proximal pulmonary artery stiffening, which is mediated by collagen accumulation in hypoxia-induced pulmonary hypertension (HPH) in mice. We sought to investigate the impact of limiting vascular and ventricular collagen accumulation on RV function and the hemodynamic coupling efficiency between the RV and pulmonary vasculature. Inbred mice were exposed to chronic hypoxia for 10 days with either no treatment (HPH) or with treatment with a proline analog that impairs collagen synthesis (CHOP-PEG; HPH + CP). Both groups were compared to control mice (CTL) exposed only to normoxia (no treatment). An admittance catheter was used to measure pressure-volume loops at baseline and during vena cava occlusion, with mice ventilated with either room air or 8% oxygen, from which pulmonary hemodynamics, RV function, and ventricular-vascular coupling efficiency (ηvvc) were calculated. Proline analog treatment limited increases in RV afterload (neither effective arterial elastance Ea nor total pulmonary vascular resistance significantly increased compared to CTL with CHOP-PEG), limited the development of pulmonary hypertension (CHOP-PEG reduced right ventricular systolic pressure by 10% compared to HPH, p < 0.05), and limited RV hypertrophy (CHOP-PEG reduced RV mass by 18% compared to HPH, p < 0.005). In an acutely hypoxic state, treatment improved RV function (CHOP-PEG increased end-systolic elastance Ees by 43%, p < 0.05) and maintained ηvvc at control, room air levels. CHOP-PEG also decreased lung collagen content by 12% measured biochemically compared to HPH (p < 0.01), with differences evident in large and small pulmonary arteries by histology. Our results demonstrate that preventing new collagen synthesis limits pulmonary hypertension development by reducing collagen accumulation in the pulmonary arteries that affect RV afterload. In particular, the proline analog limited structural and functional changes in distal pulmonary arteries in this model of early and somewhat mild pulmonary hypertension. We conclude that collagen plays an important role in small pulmonary artery remodeling and, thereby, affects RV structure and function changes induced by chronic hypoxia.


2019 ◽  
Vol 116 (10) ◽  
pp. 1700-1709 ◽  
Author(s):  
Mario Boehm ◽  
Xuefei Tian ◽  
Yuqiang Mao ◽  
Kenzo Ichimura ◽  
Melanie J Dufva ◽  
...  

Abstract Aims The temporal sequence of events underlying functional right ventricular (RV) recovery after improvement of pulmonary hypertension-associated pressure overload is unknown. We sought to establish a novel mouse model of gradual RV recovery from pressure overload and use it to delineate RV reverse-remodelling events. Methods and results Surgical pulmonary artery banding (PAB) around a 26-G needle induced RV dysfunction with increased RV pressures, reduced exercise capacity and caused liver congestion, hypertrophic, fibrotic, and vascular myocardial remodelling within 5 weeks of chronic RV pressure overload in mice. Gradual reduction of the afterload burden through PA band absorption (de-PAB)—after RV dysfunction and structural remodelling were established—initiated recovery of RV function (cardiac output and exercise capacity) along with rapid normalization in RV hypertrophy (RV/left ventricular + S and cardiomyocyte area) and RV pressures (right ventricular systolic pressure). RV fibrotic (collagen, elastic fibres, and vimentin+ fibroblasts) and vascular (capillary density) remodelling were equally reversible; however, reversal occurred at a later timepoint after de-PAB, when RV function was already completely restored. Microarray gene expression (ClariomS, Thermo Fisher Scientific, Waltham, MA, USA) along with gene ontology analyses in RV tissues revealed growth factors, immune modulators, and apoptosis mediators as major cellular components underlying functional RV recovery. Conclusion We established a novel gradual de-PAB mouse model and used it to demonstrate that established pulmonary hypertension-associated RV dysfunction is fully reversible. Mechanistically, we link functional RV improvement to hypertrophic normalization that precedes fibrotic and vascular reverse-remodelling events.


Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Maria Drakopoulou ◽  
Konstantinos Stathogiannis ◽  
Konstantinos Toutouzas ◽  
George Latsios ◽  
Andreas Synetos ◽  
...  

Objective: Severe aortic stenosis leads to increased pulmonary arterial systolic pressure. A controversy still remains regarding the impact of persistent pulmonary hypertension (PHT) on prognosis of patients undergoing transcatheter aortic valve implantation (TAVI). We sought to investigate the impact of persistent PHT on 2-year all-cause mortality of patients with severe aortic stenosis following TAVI. Methods: Patients with severe and symptomatic aortic stenosis (effective orifice area [EOA]≤1 cm 2 ) who were scheduled for TAVI with a self-expanding valve at our institution were prospectively enrolled. Prospectively collected echocardiographic data before and after TAVI were retrospectively analyzed in all patients. Pulmonary artery systolic pressure was estimated as the sum of the right ventricular to the right atrial gradient during systole and the right atrial pressure. PHT following TAVI was classified as absent if <35 mmHg and persistent if ≥35 mmHg. Primary clinical end-point was 2-year all-cause mortality defined according to the criteria proposed by the Valve Academic Research Consortium-2. Results: Hundred and forty patients (mean age: 82±9 years) were included in the study. The primary clinical end point occurred in 17 patients (12%) during a median follow-up period of 2 years. Mean pulmonary artery systolic pressure was reduced in all patients following TAVI (45±9 versus 41±6 mmHg, p<0.01). Mortality rate was higher in patients with persistent PHT compared to patients with normal pulmonary artery systolic pressure following TAVI (26% versus 14 %, p<0.01). Patients that reached the primary clinical end point had a higher post procedural mean systolic pulmonary pressure (43±9 versus 39±6 mmHg, p=0.02). In multivariate regression analysis, persistence of PHT (OR: 2.51, 95% CI: 1.109-7.224, p=0.01) was an independent predictor of long-term mortality. Conclusions: The persistence of pulmonary hypertension after TAVI is associated with long term mortality. Identifying the population that will clearly benefit from TAVI is still need to be validated by larger trials.


Author(s):  
Hyun-Jung Lee ◽  
Hyung-Kwan Kim ◽  
Sang Chol Lee ◽  
Jihoon Kim ◽  
Jun-Bean Park ◽  
...  

Abstract Aims We investigated the prognostic role of left ventricular global longitudinal strain (LV-GLS) and its incremental value to established risk models for predicting sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM). Methods and results LV-GLS was measured with vendor-independent software at a core laboratory in a cohort of 835 patients with HCM (aged 56.3 ± 12.2 years) followed-up for a median of 6.4 years. The primary endpoint was SCD events, including appropriate defibrillator therapy, within 5 years after the initial evaluation. The secondary endpoint was a composite of SCD events, heart failure admission, heart transplantation, and all-cause mortality. Twenty (2.4%) and 85 (10.2%) patients experienced the primary and secondary endpoints, respectively. Lower absolute LV-GLS quartiles, especially those worse than the median (−15.0%), were associated with progressively higher SCD event rates (P = 0.004). LV-GLS was associated with an increased risk for the primary endpoint, independent of the LV ejection fraction, apical aneurysm, and 2014 European Society of Cardiology (ESC) risk score [adjusted hazard ratio (aHR) 1.14, 95% confidence interval (CI) 1.02–1.28] or 2011 American College of Cardiology/American Heart Association (ACC/AHA) risk factors (aHR 1.18, 95% CI 1.05–1.32). LV-GLS was also associated with a higher risk for the composite secondary endpoint (aHR 1.06, 95% CI 1.01–1.12). The addition of LV-GLS enhanced the performance of the ESC risk score (C-statistic 0.756 vs. 0.842, P = 0.007) and the 2011 ACC/AHA risk factor strategy (C-statistic 0.743 vs. 0.814, P = 0.007) for predicting SCD. Conclusion LV-GLS is an important prognosticator in patients with HCM and provides additional information to established risk stratification strategies for predicting SCD.


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