141 results found
Mushemi-Blake S, Surkova E, Hatipoglu S, et al., 2020, Severe regurgitation of a double-orifice left atrioventricular valve in a patient with repaired atrioventricular septal defect: added value of 3D echocardiography, EUROPEAN HEART JOURNAL-CARDIOVASCULAR IMAGING, Vol: 21, Pages: 814-814, ISSN: 2047-2404
Martin-Garcia AC, Dimopoulos K, Boutsikou M, et al., 2020, Tricuspid regurgitation severity after atrial septal defect closure or pulmonic valve replacement, HEART, Vol: 106, Pages: 455-461, ISSN: 1355-6037
Diller G-P, Lammers AE, Babu-Narayan S, et al., 2019, Denoising and artefact removal for transthoracic echocardiographic imaging in congenital heart disease: utility of diagnosis specific deep learning algorithms, INTERNATIONAL JOURNAL OF CARDIOVASCULAR IMAGING, Vol: 35, Pages: 2189-2196, ISSN: 1569-5794
Li W, Yin Y, Dimopoulos K, et al., 2019, Early and late effects of cardiac resynchronization therapy in adult congenital heart disease, Journal of the American Heart Association, Vol: 8, ISSN: 2047-9980
Background: There are limited data regarding cardiac resynchronization therapy (CRT) in adult congenital heart disease (ACHD). We aimed to assess early and late outcomes of CRT amongst patients with ACHD.Methods: We retrospectively studied ACHD patients receiving CRT (2004-2017). Clinical and echocardiographic data were analyzed at baseline, early (1.8±0.8 years) and late (4.7±0.8 years) follow-up after CRT.Results: Fifty-four ACHD patients (median age 46 years, range 18-73 years, 74% male) had CRT (biventricular paced >90%) and were followed for 5.7±3.0 years. Compared to baseline, CRT was associated with significant improvement at early follow-up in NYHA functional class, QRS duration and cardiothoracic ratio (P<0.05 for all); improvement in NYHA class was sustained at late follow-up.Amongst patients with a systemic left ventricle (LV; n=39), there was significant increase in LV ejection fraction (LVEF) and reduction in LV end-systolic volume at early and late follow up (P<0.05 for both). For patients with a systemic right ventricle (RV; n=15), there was a significant early but not late reduction in systemic RV basal and longitudinal diameters.Eleven patients died and 2 had heart transplantation unrelated to systemic ventricular morphology. Thirty-five (65%) patients responded positively to CRT but only baseline QRS duration was predictive of a positive response.Conclusions: CRT results in sustained improvement in functional class, systemic LV size and function. Patients with a systemic LV and prolonged QRS duration, independent of QRS morphology, were most likely to respond to CRT.
McCabe C, Vranesic II, Verdes MC, et al., 2019, Reply to Echocardiographic predictors of outcome in PAH, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 294, Pages: 58-58, ISSN: 0167-5273
McCabe C, Vranesic II, Verdes MC, et al., 2019, Right ventricular systolic to diastolic duration ratio: A novel predictor of outcome in adult idiopathic pulmonary arterial hypertension, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 293, Pages: 218-222, ISSN: 0167-5273
Yin Y, Dimopoulos K, Shimada E, et al., 2019, Early and late effects of cardiac resynchronization therapy in adult congenital heart disease, Congress of the European-Society-of-Cardiology (ESC) / World Congress of Cardiology, Publisher: OXFORD UNIV PRESS, Pages: 1372-1372, ISSN: 0195-668X
Diller G-P, Babu-Narayan S, Li W, et al., 2019, Utility of machine learning algorithms in assessing patients with a systemic right ventricle, EHJ Cardiovascular Imaging / European Heart Journal - Cardiovascular Imaging, Vol: 20, Pages: 925-931, ISSN: 2047-2412
Aims: To investigate the utility of novel deep learning (DL) algorithms in recognizing transposition of the great arteries (TGA) after atrial switch procedure or congenitally corrected TGA (ccTGA) based on routine transthoracic echocardiograms. In addition, the ability of DL algorithms for delineation and segmentation of the systemic ventricle was evaluated. Methods and results: In total, 132 patients (92 TGA and atrial switch and 40 with ccTGA; 60% male, age 38.3 ± 12.1 years) and 67 normal controls (57% male, age 48.5 ± 17.9 years) with routine transthoracic examinations were included. Convolutional neural networks were trained to classify patients by underlying diagnosis and a U-Net design was used to automatically segment the systemic ventricle. Convolutional networks were build based on over 100 000 frames of an apical four-chamber or parasternal short-axis view to detect underlying diagnoses. The DL algorithm had an overall accuracy of 98.0% in detecting the correct diagnosis. The U-Net architecture model correctly identified the systemic ventricle in all individuals and achieved a high performance in segmenting the systemic right or left ventricle (Dice metric between 0.79 and 0.88 depending on diagnosis) when compared with human experts. Conclusion: Our study demonstrates the potential of machine learning algorithms, trained on routine echocardiographic datasets to detect underlying diagnosis in complex congenital heart disease. Automated delineation of the ventricular area was also feasible. These methods may in future allow for the longitudinal, objective, and automated assessment of ventricular function.
Yin YY, Dimopoulos K, Shamada E, et al., 2019, Effect of cardiac resynchronization therapy on right ventricle in adult with congenital heart disease, Publisher: WILEY, Pages: 508-508, ISSN: 1388-9842
Surkova E, West C, Flick C, et al., 2019, Added value of three-dimensional transthoracic echocardiography in assessment of an adult patient with atrioventricular septal defect, ECHOCARDIOGRAPHY-A JOURNAL OF CARDIOVASCULAR ULTRASOUND AND ALLIED TECHNIQUES, Vol: 36, Pages: 809-812, ISSN: 0742-2822
Diller G-P, Kempny A, Babu-Narayan SV, et al., 2019, Machine learning algorithms estimating prognosis and guiding therapy in adult congenital heart disease: data from a single tertiary centre including 10 019 patients, European Heart Journal, Vol: 40, Pages: 1069-1077, ISSN: 1522-9645
Aims: To assess the utility of machine learning algorithms on estimating prognosis and guiding therapy in a large cohort of patients with adult congenital heart disease (ACHD) or pulmonary hypertension at a single, tertiary centre. Methods and results: We included 10 019 adult patients (age 36.3 ± 17.3 years) under follow-up at our institution between 2000 and 2018. Clinical and demographic data, ECG parameters, cardiopulmonary exercise testing, and selected laboratory markers where collected and included in deep learning (DL) algorithms. Specific DL-models were built based on raw data to categorize diagnostic group, disease complexity, and New York Heart Association (NYHA) class. In addition, models were developed to estimate need for discussion at multidisciplinary team (MDT) meetings and to gauge prognosis of individual patients. Overall, the DL-algorithms-based on over 44 000 medical records-categorized diagnosis, disease complexity, and NYHA class with an accuracy of 91.1%, 97.0%, and 90.6%, respectively in the test sample. Similarly, patient presentation at MDT-meetings was predicted with a test sample accuracy of 90.2%. During a median follow-up time of 8 years, 785 patients died. The automatically derived disease severity-score derived from clinical information was related to survival on Cox analysis independently of demographic, exercise, laboratory, and ECG parameters. Conclusion: We present herewith the utility of machine learning algorithms trained on large datasets to estimate prognosis and potentially to guide therapy in ACHD. Due to the largely automated process involved, these DL-algorithms can easily be scaled to multi-institutional datasets to further improve accuracy and ultimately serve as online based decision-making tools.
Montanaro C, Merola A, Kempny A, et al., 2019, The outcome of adults born with pulmonary atresia: High morbidity and mortality irrespective of repair, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 280, Pages: 61-66, ISSN: 0167-5273
Krupickova S, Li W, Gatzoulis MA, et al., 2019, Ramipril for left ventricular diastolic function in patients with pulmonary regurgitation after repair of tetralogy of Fallot, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 278, Pages: 93-93, ISSN: 0167-5273
Dimopoulos K, Condliffe R, Tulloh RMR, et al., 2018, Echocardiographic Screening for Pulmonary Hypertension in Congenital Heart Disease JACC Review Topic of the Week, JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, Vol: 72, Pages: 2778-2788, ISSN: 0735-1097
Krupickova S, Li W, Cheang MH, et al., 2018, Ramipril and left ventricular diastolic function in stable patients with pulmonary regurgitation after repair of tetralogy of Fallot, Scientific Sessions of the American-Heart-Association / Resuscitation Science Symposium, Publisher: ELSEVIER IRELAND LTD, Pages: 64-69, ISSN: 0167-5273
Li W, West C, McGhie J, et al., 2018, Consensus recommendations for echocardiography in adults with congenital heart defects from the International Society of Adult Congenital Heart Disease (ISACHD), International Journal of Cardiology, Vol: 272, Pages: 77-83, ISSN: 0167-5273
The population of adults with congenital heart disease (ACHD) is increasing constantly due to medical, surgical and interventional successes and the input from advanced cardiovascular imaging. ACHD patients are at continuing risk of residua and sequelae related to their CHD contributing to significant morbidity and mortality. Consequently, lifelong expert surveillance is recommended for most patients. Healthcare providers are still working out how best to achieve this objective, how to train enough experts to provide high quality care, and how to organize the delivery of care. Echocardiography is crucial to clinical surveillance providing a comprehensive assessment of cardiac morphology, physiology, pathophysiology, and function. Thus it contributes significantly to the overall clinical management of ACHD patients. The International Society for Adult Congenital Heart Disease (ISACHD; www.isachd.org) is the leading organization of professionals worldwide dedicated to the pursuit of excellence in the care of ACHD patients. Recognizing the critical role of imaging in the diagnosis and management of ACHD, ISACHD established a task force to provide guidance on echocardiographic studies and reporting. The rationale is that standardization of echocardiographic imaging and reporting carries the potential to improve the overall quality of these exams around the world and facilitate collaborative multicenter research. The standardized ACHD protocols provided by the ISACHD task force (found in the appendices) include specific recommendations for data acquisition and reporting for each of the major adult congenital heart lesions. These protocols give a comprehensive and structured approach in the evaluation of ACHD patients and help to ensure excellent patient care.
Di Salvo G, Miller O, Babu Narayan S, et al., 2018, Imaging the adult with congenital heart disease: a multimodality imaging approach-position paper from the EACVI, EHJ Cardiovascular Imaging / European Heart Journal - Cardiovascular Imaging, Vol: 19, Pages: 1077-1098, ISSN: 2047-2412
Advances in the diagnosis and management of congenital heart disease have led to a marked improvement in the survival of adult with congenital heart disease (ACHD) patients. However, ACHD patients are a heterogeneous population, with a large spectrum of anatomic substrates even within specific lesions. In addition, the nature of previous surgery and other intervention is highly variable rendering each patient unique and residual anatomic and haemodynamic abnormalities are very common. As the ACHD population continues to age, acquired heart disease will also require cardiac imaging assessment. It is increasingly recognized in ACHD community that the diagnostic utility of a multimodality cardiovascular approach is greater than the sum of individual tests. In ACHD patients, diagnostic information can be obtained using a variety of diagnostic tools. The aims of this document are to describe the role of each diagnostic modality in the care of ACHD patients and to provide guidelines for a multimodality approach. The goal should be to provide the most appropriate and cost-effective diagnostic pathway for each individual ACHD patient.
Nashat H, Montanaro C, Li W, et al., 2018, Atrial septal defects and pulmonary arterial hypertension, JOURNAL OF THORACIC DISEASE, Vol: 10, Pages: S2953-S2965, ISSN: 2072-1439
Kavouras C, Li W, Dimopoulos K, et al., 2018, Non compaction cardiomyopathy. Prevalence and relation to outcome among patients with congenital heart diseases, European-Society-of-Cardiology Congress, Publisher: OXFORD UNIV PRESS, Pages: 242-242, ISSN: 0195-668X
Surkova E, Senior R, Li W, 2018, Making complex pathology simple: added value of 3D transthoracic echocardiography in an adult patient with congenitally corrected transposition of great arteries and severe tricuspid regurgitation, EHJ Cardiovascular Imaging / European Heart Journal - Cardiovascular Imaging, Vol: jey100, ISSN: 2047-2412
Boutsikou M, Shore D, Li W, et al., 2018, Anomalous left coronary artery from the pulmonary artery (ALCAPA) diagnosed in adulthood: varied clinical presentation, therapeutic approach and outcome, International Journal of Cardiology, Vol: 261, Pages: 49-53, ISSN: 0167-5273
IntroductionThe diagnosis of ALCAPA syndrome is sporadic in adulthood, of the limited cases in the literature most are incidental or without symptoms. There is a broad spectrum of clinical manifestations of ALCAPA syndrome however, including sudden cardiac death.CasesWe present herewith a series of 12 consecutive patients with ALCAPA, all diagnosed in adulthood (between 18 and 73 years of age). Five patients developed symptoms (breathlessness) after the fourth decade of life, 3 were undiagnosed despite a history of previous mitral valve repair, one presented with heart failure, one with resuscitated cardiac arrest, whereas two patients were asymptomatic. We review in this paper, the clinical history, diagnostic approach and therapeutic choices of ALCAPA syndrome.ConclusionALCAPA syndrome is not confined to childhood, late diagnosis in adulthood has a varied clinical presentation. ALCAPA syndrome should be particularly considered as a potential, albeit uncommon cause of mitral regurgitation and/or dilated cardiomyopathy.
Cordina R, Ministeri M, Babu-Narayan SV, et al., 2018, Evaluation of the relationship between ventricular end-diastolic pressure and echocardiographic measures of diastolic function in adults with a Fontan circulation, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 259, Pages: 71-75, ISSN: 0167-5273
BackgroundEchocardiographic assessment of diastolic function in the setting of Fontan physiology is not well validated. We recently demonstrated that atrioventricular systolic to diastolic duration ratio (AVV S/D ratio) independently predicts mortality in Fontan-adults and that a value >1.1 was associated with poor prognosis.PurposeTo correlate echocardiographic measures of diastolic function with direct measurement of ventricular end-diastolic pressure (VEDP).MethodsA retrospective analysis was undertaken of Fontan-adults who had transthoracic echocardiography (TTE) within 12 months of direct measurement of VEDP during cardiac catheterisation.ResultsFifteen Fontan adults (3 males, mean age 29 ± 9 years) were evaluated. Thirteen patients had dominant morphologic left ventricle and 2 had morphologic right ventricle. Four had atriopulmonary connection and 11 had total cavopulmonary connection. Twelve patients were NYHA Class I/II and 3 were Class III. Time between TTE and cardiac catheter was 46 ± 113 days; VEDP was 8 ± 5 mmHg. Ten patients had preserved ventricular function, 3 had mild and 2 had moderate systolic impairment by subjective TTE assessment. AVV S/D ratio had the strongest correlation with VEDP (r = 0.8, p = 0.001). AVV S/D ratio ≥ 1.1 had 100% positive predictive value and 92% negative predictive value for detecting VEDP >10 mmHg. The only conventional echocardiographic measure of diastolic function that correlated with VEDP was pulmonary vein A wave - atrioventricular A wave duration difference (r = 0.8, p = 0.02).ConclusionsTTE measures reflect VEDP in adults with a Fontan circulation. AVV S/D ratio is a simple parameter yet to enter standard practice that can be used to identify elevated VEDP.
Heng E, Gatzoulis M, Uebing A, et al., 2017, Immediate and midterm cardiac remodelling following surgical pulmonary valve replacement in adults with repaired tetralogy of fallot: a prospective cardiovascular magnetic resonance and clinical study, Circulation, Vol: 136, Pages: 1703-1713, ISSN: 0009-7322
Background: Pulmonary valve replacement (PVR) in patients with repaired tetralogy of Fallot provides symptomatic benefit and right ventricular (RV) volume reduction. However, data on the rate of ventricular structural and functional adaptation are scarce. We aimed to assess immediate and midterm post-PVR changes and predictors of reverse remoeling.Methods: Fifty-seven patients with repaired tetralogy of Fallot (age ≥16 y; mean age, 35.8±10.1 y; 38 male) undergoing PVR were prospectively recruited for cardiovascular magnetic resonance performed before PVR (pPVR), immediately after PVR (median, 6 d), and midterm after PVR (mPVR; median, 3 y).Results: There were immediate and midterm reductions in indexed RV end-diastolic volumes and RV end-systolic volumes (RVESVi) (indexed RV end-diastolic volume pPVR versus immediately after PVR versus mPVR, 156.1±41.9 versus 104.9±28.4 versus 104.2±34.4 mL/m2; RVESVi pPVR versus immediately after PVR versus mPVR, 74.9±26.2 versus 57.4±22.7 versus 50.5±21.7 mL/m2; P<0.01). Normal postoperative diastolic and systolic RV volumes (the primary end point) achieved in 70% of patients were predicted by a preoperative indexed RV end-diastolic volume ≤158 mL/m2 and RVESVi ≤82 mL/m2. RVESVi showed a progressive decrease from baseline to immediate to midterm follow-up, indicating ongoing intrinsic RV functional improvement after PVR. Left ventricular ejection fraction improved (pPVR versus mPVR, 59.4±7.6% versus 61.9±6.8%; P<0.01), and right atrial reverse remodeling occurred (pPVR versus mPVR, 15.2±3.4 versus 13.8±3.6 cm2/m2; P<0.01). Larger preoperative RV outflow tract scar was associated with a smaller improvement in post-PVR RV/left ventricular ejection fraction. RV ejection fraction and peak oxygen uptake predicted mortality (P=0.03) over a median of 9.5 years of follow-up.Conclusions: Significant right heart structural reverse remodeling takes
Kempny A, Hjortshoj CS, Li W, et al., 2017, Response by Kempny et al to Letter Regarding Article, "Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome: A Multicenter Study", CIRCULATION, Vol: 136, Pages: 1078-1079, ISSN: 0009-7322
Cauldwell M, Quail M, Smith GS, et al., 2017, Effect of pregnancy on ventricular and aortic dimensions in repaired tetralogy of Fallot, Journal of the American Heart Association, Vol: 6, ISSN: 2047-9980
Background: To assess whether the cardiovascular adaptation to pregnancy in women with repaired tetralogy of Fallot (TOF) adversely affects haemodynamic stability; in particular with respect to right ventricular (RV) dilatation, pulmonary regurgitation or aortic root dilatation. Methods and Results: Retrospective cohort study of women with repaired TOF with paired Cardiovascular Magnetic Resonance (CMR) scans before and after their first pregnancy (baseline RV end systolic volume index 49mL/m2 and RV end diastolic volume index 118mL/m2) matched with a comparison group of nulliparous women with TOF. Cases were matched for age at baseline CMR scan, time between follow up of CMR scans, QRS duration, RV ejection fraction and indexed RV end systolic and diastolic volume at baseline. The effect of pregnancy and time on parameters was assessed using mixed effects modelling. Nineteen women with repaired TOF who had completed their first pregnancy were identified and matched with 38 nulliparous women. We observed no deleterious effects of pregnancy on RV volumes, aortic dimensions or exercise data. There was an effect of pregnancy observed in both LV EDV and LV SV, consistent with a sustained small increase in LV SV due to pregnancy (53ml/m2 to 55ml/m2).Conclusion: Women with repaired TOF and with mild to moderate RV dilatation considering pregnancy can be reassured that pregnancy is unlikely to cause deterioration in their cardiovascular status. We recommend that women are routinely assessed and followed up before and after pregnancy and that pre pregnancy counselling is tailored to their individual clinical status.
Rydman R, Shiina Y, Diller GP, et al., 2017, Major adverse events and atrial tachycardia in Ebstein’s anomaly by cardiovascular magnetic resonance, Heart, Vol: 104, Pages: 37-44, ISSN: 1468-201X
Objectives Patients with Ebstein’s anomaly of the tricuspid valve (EA) are at risk of tachyarrhythmia, congestive heart failure and sudden cardiac death. We sought to determine the value of cardiovascular magnetic resonance (CMR) for predicting these outcomes. Methods Seventy-nine consecutive adult patients (aged 37±15 years) with unrepaired EA underwent CMR and were followed prospectively for a median 3.4 (range 0.4-10.9) years for clinical outcomes, namely major adverse cardiovascular events (MACE: sustained ventricular tachycardia/heart failure hospital admission/cardiac transplantation/death) and first-onset atrial tachyarrhythmia (AT).Results CMR-derived variables associated with MACE (n=6) were right ventricular (RV) or left ventricular (LV) ejection fraction (EF) (HR 2.06[95%CI 1.168-3.623],p=0.012 and HR 2.35[95%CI 1.348-4.082],p=0.003, respectively), LV stroke volume index (HR 2.82[95%CI 1.212-7.092],p=0.028) and cardiac index (HR 1.71[95%CI 1.002-1.366],p=0.037);all remained significant when tested solely for mortality. Prior history of AT (HR 11.16[95%CI 1.30-95.81],p=0.028) and NYHA-class >2 (HR 7.66[95%CI 1.54-38.20],p=0.013) were also associated with MACE; AT preceded all but one MACE events suggesting its potential role as an early marker of adverse outcome (p=0.011). CMR variables associated with first-onset AT (n=17;21.5%) included RVEF (HR 1.55[95%CI 1.103-2.160],p=0.011)],total R/L volume index (HR 1.18[95%CI 1.06-1.32],p=0.002), RV/LV end diastolic volume ratio (HR 1.55[95%CI 1.14-2.10],p=0.005) and apical septal leaflet displacement/total LV septal length (HR 1.03[95%CI 1.00-1.07],p=0.041); the latter two combined enhanced risk prediction (HR 6.12[95% CI 1.67-22.56],p=0.007). Conclusion CMR-derived indices carry prognostic information regarding MACE and first-onset AT amongst adults with unrepaired EA. CMR may be included in the periodic surveillance of these patients.
Vamvakidou A, Chahal N, Hampson R, et al., 2017, STROKE VOLUME DETERMINED FLOW RESERVE DOES NOT PREDICT THE TRUE SEVERITY OF LOW-FLOW LOW-GRADIENT AORTIC STENOSIS AND IS NOT A ROBUST MARKER OF CONTRACTILE RESERVE IN PATIENTS UNDERGOING LOW-DOSE DOBUTAMINE ECHOCARDIOGRAPHY, Annual Conference of the British-Cardiovascular-Society (BCS), Publisher: BMJ PUBLISHING GROUP, Pages: A94-A95, ISSN: 1355-6037
Beurtheret S, Tutarel O, Diller GP, et al., 2017, Contemporary cardiac surgery for adults with congenital heart disease, Heart, Vol: 103, Pages: 1194-1202, ISSN: 1355-6037
Objective Advances in early management of congenital heart disease (CHD) have led to an exponential growth in adults with CHD (ACHD). Many of these patients require cardiac surgery. This study sought to examine outcome and its predictors for ACHD cardiac surgery.Methods This is an observational cohort study of prospectively collected data on 1090 consecutive adult patients with CHD, undergoing 1130 cardiac operations for CHD at the Royal Brompton Hospital between 2002 and 2011. Early mortality was the primary outcome measure. Midterm to longer-term survival, cumulative incidence of reoperation, other interventions and/or new-onset arrhythmia were secondary outcome measures. Predictors of early/total mortality were identified.Results Age at surgery was 35±15 years, 53% male, 52.3% were in New York Heart Association (NYHA) class I, 37.2% in class II and 10.4% in class III/IV. Early mortality was 1.77% with independent predictors NYHA class ≥ III, tricuspid annular plane systolic excursion (TAPSE) <15 mm and female gender. Over a mean follow-up of 2.8±2.6 years, 46 patients died. Baseline predictors of total mortality were NYHA class ≥ III, TAPSE <15 mm and non-elective surgery. The number of sternotomies was not independently associated with neither early nor total mortality. At 10 years, probability of survival was 94%. NYHA class among survivors was significantly improved, compared with baseline.Conclusions Contemporary cardiac surgery for ACHD performed at a single, tertiary reference centre with a multidisciplinary approach is associated with low mortality and improved functional status. Also, our findings emphasise the point that surgery should not be delayed because of reluctance to reoperate only.
Moceri P, Bouvier P, Baudouy D, et al., 2016, Cardiac remodelling amongst adults with various aetiologies of pulmonary arterial hypertension including Eisenmenger syndrome-implications on survival and the role of right ventricular transverse strain., Eur Heart J Cardiovasc Imaging
AIMS: Survival in pulmonary arterial hypertension (PAH) and Eisenmenger syndrome (ES) relates to right ventricular (RV) function. Little is known about differences of ventricular function between ES patients and those suffering from other PAH aetiologies. In this study, we compared global ventricular function assessed by speckle-tracking in adult patients with ES, other PAH aetiologies, or healthy controls; and assessed the relationship between ventricular function and survival. METHODS AND RESULTS: We performed a prospective cohort study recruiting 83 adult PAH patients (43 ES and 40 other PAH aetiologies patients) and 37 controls between March 2011 and June 2015. Patients with complex congenital heart disease were excluded. Fifty-three patients (63.9%) were in NYHA functional class ≥III at baseline and 60 (72.3%) were on advanced therapies. Mean RV peak longitudinal strain was -16.3 ± 7% in ES, lower compared with healthy controls (P < 0.001) but similar to other PAH aetiologies (P = 0.6). Mean RV peak transverse strain was +26.1 ± 17% in ES, lower than in controls (P < 0.001) but higher than in other PAH aetiologies (P < 0.001). No difference was observed between ES and other PAH in LV circumferential and longitudinal strain. Over a median follow-up of 22.6 months (3.3-32.2), 22 (26.5%) patients died all from cardio-pulmonary causes. ES and RV peak transverse strain were independent predictors of survival. RV peak transverse strain ≤22% identified patients with a 14-fold increased risk of death. CONCLUSION: Right ventricular remodelling differs between adults with ES and other PAH aetiologies. ES and increased RV free wall transverse strain are associated with better survival.
Kempny A, Hjortshøj CS, Gu H, et al., 2016, Predictors of death in contemporary adult patients with Eisenmenger syndrome: a multicentre study, Circulation, Vol: 135, Pages: 1432-1440, ISSN: 0009-7322
BACKGROUND: -Eisenmenger syndrome (ES) is associated with substantial morbidity and mortality. There is no consensus, however, on mortality risk stratification. We aimed to investigate survival and predictors of death in a large, contemporary cohort of ES patients. METHODS: -We identified in a multicentre approach adults with ES under follow-up between 2000 and 2015. We examined survival and its association with clinical, electrocardiographic, echocardiographic and laboratory parameters. RESULTS: -We studied 1098 patients (median age 34.4years, range 16.1-84.4years, 65.1% female, 31.9% with Down syndrome). The majority had a post-tricuspid defect (n=643, 58.6%), followed by patients with a complex (n=315, 28.7%) and pre-tricuspid lesion (n=, 12.7%). Over a median follow-up of 3.1years [IQR 1.4-5.9], allowing for 4361.6 patient-years observation, 278 patients died and six and six underwent transplantation. Twelve parameters emerged as significant predictors of death on univariable analysis. On multivariable Cox regression analysis only age (HR 1.41/10years, 95%CI 1.24-1.59, P<0.001), pre-tricuspid shunt (HR 1.56, 95%CI 1.02-2.39, P=0.041), oxygen saturation at rest (HR 0.53/10%, 95%CI 0.43-0.65, P<0.001), presence of sinus rhythm (HR 0.53, 95%CI 0.32-0.88, P=0.013) and presence of pericardial effusion (HR 2.41, 95%CI 1.59-3.66, P<0.001) remained significant predictors of death. CONCLUSIONS: -There is significant premature mortality amongst contemporary adults with ES. We report, herewith a multivariable mortality risk stratification model based on five simple, non-invasive predictors of death in this population.
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