Publications
217 results found
Vescovo GM, Kempny A, Alonso-Gonzalez R, et al., 2016, Does the ECG still carry prognostic power in contemporary patients with repaired tetralogy of Fallot?, Congress of the European-Society-of-Cardiology (ESC), Publisher: OXFORD UNIV PRESS, Pages: 763-763, ISSN: 0195-668X
Montanaro C, Alvarez-Alvarez B, Ministeri M, et al., 2016, Pulmonary hypertension in patients with transposition of great arteries or pulmonary atresia, Congress of the European-Society-of-Cardiology (ESC), Publisher: OXFORD UNIV PRESS, Pages: 158-158, ISSN: 0195-668X
Spadotto V, Voges I, Kilner PJ, et al., 2016, Juxtaposition of the atrial appendages: A nidus for thrombus in atriopulmonary Fontan?, Global Cardiology Science & Practice, Vol: 2016, ISSN: 2305-7823
Juxtaposition of atrial appendages is a rare cardiac congenital anomaly, usually associated with other cardiac malformations. Until now, it has not been linked to any significant clinical implications. We report cardiovascular magnetic resonance (CMR) findings of two adult patients who underwent atriopulmonary Fontan operation in the setting of left juxtaposition of the atrial appendages. The patients were in sinus rhythm at the time of the CMR study. Both patients had episodes of sustained atrial tachyarrhythmia requiring electrical cardioversion and were anticoagulated with warfarin with target INR 2-3. CMR images showed a thrombus located in the enlarged and juxtaposed right appendage in both patients. Blood flow frequently appears slow or sluggish in the dilated right atrium following atriopulmonary Fontan surgery. In addition, cine CMR suggested that blood flow reaches very low velocities in the massively dilated juxtaposed right atrial appendage cul-de-sac, thus potentially creating a substrate for clot formation. These findings propose that juxtaposed atrial appendages in atriopulmonary Fontan is an additional risk factor for clot formation, specifically in the dilated right atrial appendage on the left side juxtaposed with the left atrial appendage and that prophylactic anticoagulation is highly justified in these patients.
Babu-Narayan SV, Prati D, Rydman R, et al., 2016, Dyssynchrony and Electromechanical delay are associated with focal fibrosis in the Systemic Right Ventricle - Insights from Echocardiography, International Journal of Cardiology, Vol: 220, Pages: 382-388, ISSN: 1874-1754
BackgroundSystemic right ventricular (RV) dysfunction and sudden cardiac death remain problematic late after Mustard operation for transposition of the great arteries. The exact mechanism for that relationship is likely to be multifactorial including myocardial fibrosis. Doppler echocardiography gives further insights into the role of fibrosis shown by late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) in late morbidity.Methods and resultsTwenty-two consecutive patients, mean age 28 ± 8 years, were studied with 2D echocardiography, and also assessed by LGE CMR. The presence of LGE in 13/22 patients (59%) was related to delayed septal shortening and lengthening (P = 0.002 & P = 0.049), prolonged systemic RV isovolumic contraction time (P = 0.024) and reduced systemic RV free wall and septal excursion (P = 0.027 & P = 0.005). The systemic RV total isovolumic time was prolonged but not related to extent of LGE. LGE extent was related to markers of electromechanical delay and dyssynchrony (delayed onset of RV free wall shortening and lengthening; r = 0.73 & P = 0.004 and r = 0.62 & P = 0.041, respectively, and QRS duration r = 0.68, P < 0.01) and was inversely related to systolic RV free wall shortening velocity (r = − 0.59 & P = 0.042). The presence of LGE was also related to lower exercise capacity, ≥ mild tricuspid regurgitation and more arrhythmia (P = 0.008, P = 0.014 and P = 0.040). RV free wall excursion and systolic tissue Doppler velocity were related to CMR derived RV ejection fraction (r = 0.51, P = 0.015, and r = 0.77, P = < 0.001, respectively).ConclusionPost Mustard repair, myocardial fibrosis is related to dyssynchrony, RV long axis dysfunction and tricuspid regurgitation. Echocardiographic measurements of systemic RV function can be confidently used in serial follow-up following Mustard operation.
Cordina R, von Klemperer K, Kempny A, et al., 2016, Echocardiographic predictors of mortality in adults with a fontan circulation, Journal of the American College of Cardiology: Cardiovascular Imaging, Vol: 10, Pages: 212-213, ISSN: 1936-878X
Bonello B, Shore DF, Uebing A, et al., 2016, AORTIC DILATATION IN REPAIRED TETRALOGY OF FALLOT: FEATURES, DETERMINANTS AND PROGRESSION, Annual Meeting of the British-Congenital-Cardiac-Association, Publisher: BMJ PUBLISHING GROUP, Pages: A2-A3, ISSN: 1355-6037
Heng EL, Gatzoulis MA, Uebing A, et al., 2016, EARLY CARDIAC REMODELLING AFTER PULMONARY VALVE REPLACEMENT IN PATIENTS WITH REPAIRED TETRALOGY OF FALLOT, Annual Meeting of the British-Congenital-Cardiac-Association, Publisher: BMJ PUBLISHING GROUP, Pages: A26-A26, ISSN: 1355-6037
Rydman R, Shiina Y, Diller G-P, et al., 2016, MORTALITY AND VT IN EBSTEIN'S ANOMALY OF THE TRICUSPID VALVE: A PROSPECTIVE CARDIOVASCULAR MAGNETIC RESONANCE STUDY, Annual Meeting of the British-Congenital-Cardiac-Association, Publisher: BMJ PUBLISHING GROUP, Pages: A27-A27, ISSN: 1355-6037
Karonis T, Scognamiglio G, Babu-Narayan SV, et al., 2016, Clinical course and potential complications of small ventricular septal defects in adulthood: Late development of left ventricular dysfunction justifies lifelong care, International Journal of Cardiology, Vol: 208, Pages: 102-106, ISSN: 1874-1754
BackgroundPatients with small ventricular septal defects (VSDs) are thought to have excellent long-term survival, although complications may not be uncommon.MethodsWe identified all patients aged ≥ 16 years with native isolated VSD between January 2000 and September 2013. Clinical outcomes were retrospectively reviewed. Transthoracic echocardiograms performed within 2 years of last assessment were reviewed for hemodynamic sequelae.ResultsTwo-hundred-and-thirty-one patients, 100 (43%) male, mean age at last follow-up 34 ± 13 years were studied. During the recorded period there were no deaths. The majority (224/231; 97%) were asymptomatic. Documented arrhythmias occurred in 7 patients (3%), double-chamber right ventricle (DCRV) in 29 (13%), more than mild aortic regurgitation in 6 (3%) and infective endocarditis in 24 (10%) patients.Surgery due to complications associated with VSD was performed in 26 (11%) patients at a median age of 27.6 years (IQR: 16.1–38.7) due to DCRV (n = 17, 65%), infective endocarditis (n = 6, 23%), progression of left–right shunt (n = 2, 8%) and aortic regurgitation (n = 1, 4%).At most recent echocardiography (n = 164), 10 (6%), had reduced LVEF, 34 (21%) had increased LVEDD and 17 (10%) had LVESD > 4.0 cm. Thirty-two patients (25%) with normal LV dimensions had LA enlargement suggesting LV diastolic dysfunction.ConclusionsWe report a non-negligible incidence of major complications or clinical events during late follow-up of adult patients with restrictive VSDs. Furthermore, we show co-existing LV dysfunction, systolic or diastolic in a subset of patients. Indication for VSD closure in childhood may be recognized, whereas lifelong follow-up for adult with restrictive VSDs is clearly warranted.
Cauldwell M, Von Klemperer K, Uebing A, et al., 2016, A cohort study of women with a Fontan circulation undergoing preconception counselling, Heart, Vol: 102, Pages: 534-540, ISSN: 1468-201X
Heng EL, Kellman P, Gatzoulis MA, et al., 2016, Quantifying right ventricular diffuse fibrosis in tetralogy of fallot-a novel customised approach for the challenges of the right ventricle, Journal of Cardiovascular Magnetic Resonance, Vol: 18, Pages: 1-3, ISSN: 1097-6647
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Jensen AS, Broberg CS, Rydman R, et al., 2015, Impaired Right, Left, or Biventricular Function and Resting Oxygen Saturation Are Associated With Mortality in Eisenmenger Syndrome: A Clinical and Cardiovascular Magnetic Resonance Study., Circulation: Cardiovascular Imaging, Vol: 8, ISSN: 1941-9651
BACKGROUND: Patients with Eisenmenger syndrome (ES) have better survival, despite similar pulmonary vascular pathology, compared with other patients with pulmonary arterial hypertension. Cardiovascular magnetic resonance (CMR) is useful for risk stratification in idiopathic pulmonary arterial hypertension, whereas it has not been evaluated in ES. We studied CMR together with other noninvasive measurements in ES to evaluate its potential role as a noninvasive risk stratification test. METHODS AND RESULTS: Between 2003 and 2005, 48 patients with ES, all with a post-tricuspid shunt, were enrolled in a prospective, longitudinal, single-center study. All patients underwent a standardized baseline assessment with CMR, blood test, echocardiography, and 6-minute walk test and were followed up for mortality until the end of December 2013. Twelve patients (25%) died during follow-up, mostly from heart failure (50%). Impaired ventricular function (right or left ventricular ejection fraction) was associated with increased risk of mortality (lowest quartile: right ventricular ejection fraction, <40%; hazard ratio, 4.4 [95% confidence interval, 1.4-13.5]; P=0.01 and left ventricular ejection fraction, <50%; hazard ratio, 6.6 [95% confidence interval, 2.1-20.8]; P=0.001). Biventricular impairment (lowest quartile left ventricular ejection fraction, <50% and right ventricular ejection fraction, <40%) conveyed an even higher risk of mortality (hazard ratio, 8.0 [95% confidence interval, 2.5-25.1]; P=0.0004). No other CMR or noninvasive measurement besides resting oxygen saturation (hazard ratio, 0.90 [0.83-0.97]/%; P=0.007) was associated with mortality. CONCLUSIONS: Impaired right, left, or biventricular systolic function derived from baseline CMR and resting oxygen saturation are associated with mortality in adult patients with ES. CMR is a useful noninvasive tool, which may be incorporated in the risk stratification assessment of ES during lifelong follow-up.
Pennell DJ, Baksi AJ, Prasad SK, et al., 2015, Review of Journal of Cardiovascular Magnetic Resonance 2014, Journal of Cardiovascular Magnetic Resonance, Vol: 17, ISSN: 1532-429X
There were 102 articles published in the Journal of Cardiovascular Magnetic Resonance (JCMR) in 2014, which is a6 % decrease on the 109 articles published in 2013. The quality of the submissions continues to increase. The 2013JCMR Impact Factor (which is published in June 2014) fell to 4.72 from 5.11 for 2012 (as published in June 2013).The 2013 impact factor means that the JCMR papers that were published in 2011 and 2012 were cited on average4.72 times in 2013. The impact factor undergoes natural variation according to citation rates of papers in the 2 yearsfollowing publication, and is significantly influenced by highly cited papers such as official reports. However,the progress of the journal’s impact over the last 5 years has been impressive. Our acceptance rate is <25 %and has been falling because the number of articles being submitted has been increasing. In accordance withOpen-Access publishing, the JCMR articles go on-line as they are accepted with no collating of the articlesinto sections or special thematic issues. For this reason, the Editors have felt that it is useful once per calendar year tosummarize the papers for the readership into broad areas of interest or theme, so that areas of interest canbe reviewed in a single article in relation to each other and other recent JCMR articles. The papers are presented inbroad themes and set in context with related literature and previously published JCMR papers to guide continuity ofthought in the journal. We hope that you find the open-access system increases wider reading and citation of yourpapers, and that you will continue to send your quality papers to JCMR for publication.
Ernst S, Underwood R, Babu-Narayan S, et al., 2015, Modulation of Ganglionated Plexi as an Addition to Pv Isolation in Persistent Atrial Fibrillation Ablation, Scientific Sessions and Resuscitation Science Symposium of the American-Heart-Association (AHA), Publisher: LIPPINCOTT WILLIAMS & WILKINS, ISSN: 0009-7322
Cordina R, von Klemperer K, Kempny A, et al., 2015, Echocardiographic Predictors of Mortality in Adults With a Fontan Circulation, Publisher: LIPPINCOTT WILLIAMS & WILKINS, ISSN: 0009-7322
Wald RM, Valente AM, Gauvreau K, et al., 2015, Cardiac magnetic resonance markers of progressive RV dilation and dysfunction after tetralogy of Fallot repair, HEART, Vol: 101, Pages: 1724-1730, ISSN: 1355-6037
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- Citations: 65
Heng EL, Gatzoulis MA, Babu-Narayan SV, 2015, Tailoring counselling after pulmonary valve surgery in repaired tetralogy of Fallot, HEART, Vol: 101, Pages: 1695-1696, ISSN: 1355-6037
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- Citations: 1
Kempny A, Diller G-P, Dimopoulos K, et al., 2015, Determinants of outpatient clinic attendance amongst adults with congenital heart disease and outcome, International Journal of Cardiology, Vol: 203, Pages: 245-250, ISSN: 1874-1754
Background:Adult congenital heart disease (ACHD) guidelines advise life-long, regular, follow up in predefined intervals for ACHD patients. However, limited data exist to support this position. We examine, herewith, compliance to scheduled outpatient clinic appointments and its impact on outcome.Methods and results:We examined 4461 ACHD patients (median age at entry 26.4 years, 51% female) and their follow up records at our tertiary centre between 1991 and 2008. Clinic attendance was quantified from electronic hospital records. For survival analysis we employed the last clinic attendance before 2008 as starting of follow-up. Overall 23% of scheduled clinic appointments were not attended. The main predictors of clinic non-attendance (CNA) were younger age, non-Caucasian ethnicity, lower socioeconomic status, number of previous CNAs and the lack of planned additional investigation/s (e.g. echocardiography) scheduled on the same day. During a cumulative follow-up time of 48,828 patient-years, 366 (8.2%) patients died. Both, the number of CNAs (HR = 1.08, 95% CI 1.05–1.12 per CNA, P < 0.001) and the ratio of CNA to follow up period (HR = 1.23, 95% CI 1.04–1.44 per CNA/year, P = 0.013) emerged as predictors of mortality independent of adjustment for patients' age, disease complexity, functional class and socioeconomic status.Conclusions:Patient adherence to scheduled ACHD outpatient-clinics is associated with better survival. Identifying patients at an increased risk of CNA in a single tertiary centre is feasible. Our data provides previously lacking evidence supporting the practice of periodic assessment of ACHD patients at tertiary clinics. Non-attenders should be specifically targeted and receive counselling to modulate their increased risk of death.
Babu-Narayan SV, Giannakoulas G, Valente AM, et al., 2015, Imaging of congenital heart disease in adults., European Heart Journal, Vol: 37, Pages: 1182-1195, ISSN: 1522-9645
Imaging is fundamental to the lifelong care of adult congenital heart disease (ACHD) patients. Echocardiography remains the first line imaging for inpatient, outpatient, or perioperative care. Cross-sectional imaging with cardiovascular magnetic resonance (CMR) or computed tomography (CT) provides complementary and invaluable information on cardiac and vascular anatomy and other intra-thoracic structures. Furthermore, CMR provides quantification of cardiac function and vascular flow. Cardiac catheterization is mostly reserved for assessment of pulmonary vascular resistance, ventricular end-diastolic pressure, and percutaneous interventions. There have been further advances in non-invasive imaging for ACHD including the application of advanced echocardiographic techniques, faster automated CMR imaging, and radiation dose reduction in CT. As a result ACHD, a heterogeneous population, benefit from appropriate application of multiple imaging modalities matched with tertiary ACHD expertise.
Diller G-P, Kempny A, Alonso-Gonzalez R, et al., 2015, Survival prospects and circumstances of death in contemporary adult congenital heart disease patients under follow-up at a large tertiary centre, Circulation, Vol: 132, Pages: 2118-2125, ISSN: 0009-7322
Background—Adult congenital heart disease (ACHD) patients have ongoing morbidity and reduced long-term survival. Recently, the importance of specialized follow-up at tertiary ACHD centers has been highlighted. We aimed to assess survival prospects and clarify causes of death in a large cohort of patients at a single, tertiary center.Methods and Results—We included 6969 adult patients (age 29.9±15.4 years) under follow-up at our institution between 1991 and 2013. Causes of death were ascertained from official death certificates. Survival was compared with the expected survival in the general age- and sex-matched population, and standardized mortality rates were calculated. Over a median follow-up time of 9.1 years (interquartile range, 5.2–14.5), 524 patients died. Leading causes of death were chronic heart failure (42%), pneumonia (10%), sudden-cardiac death (7%), cancer (6%), and hemorrhage (5%), whereas perioperative mortality was comparatively low. Isolated simple defects exhibited mortality rates similar to those in the general population, whereas patients with Eisenmenger syndrome, complex congenital heart disease, and Fontan physiology had much poorer long-term survival (P<0.0001 for all). The probability of cardiac death decreased with increasing patient’s age, whereas the proportion of patients dying from noncardiac causes, such as cancer, increased.Conclusions—ACHD patients continue to be afflicted by increased mortality in comparision with the general population as they grow older. Highest mortality rates were observed among patients with complex ACHD, Fontan physiology, and Eisenmenger syndrome. Our data provide an overview over causes of mortality and especially the spectrum of noncardiac causes of death in contemporary ACHD patients.
Diller G-P, Braeutigam A, Kempny A, et al., 2015, Depression requiring anti-depressant drug therapy in adult congenital heart disease: prevalence, risk factors, and prognostic value, European Heart Journal, Vol: 37, Pages: 771-782, ISSN: 1522-9645
Background Depression is prevalent in adults with congenital heart disease (ACHD), but limited data on the frequency of anti-depressant drug (ADD) therapy and its impact on outcome are available.Methods and results We identified all ACHD patients treated with ADDs between 2000 and 2011 at our centre. Of 6162 patients under follow-up, 204 (3.3%) patients were on ADD therapy. The majority of patients were treated with selective serotonin-reuptake inhibitors (67.4%), while only 17.0% of patients received tricyclic anti-depressants. Twice as many female patients used ADDs compared with males (4.4 vs. 2.2%, P < 0.0001). The percentage of patients on ADDs increased with disease complexity (P < 0.0001) and patient age (P < 0.0001). Over a median follow-up of 11.1 years, 507 (8.2%) patients died. After propensity score matching, ADD use was found to be significantly associated with worse outcome in male ACHD patients [hazard ratio 1.44 (95% confidence interval 1.17–1.84)]. There was no evidence that this excess mortality was directly related to ADD therapy, QT-prolongation, or malignant arrhythmias. However, males taking ADDs were also more likely to miss scheduled follow-up appointments compared with untreated counterparts, while no such difference in clinic attendance was seen in females.Conclusions The use of ADD therapy in ACHD relates to gender, age, and disease complexity. Although, twice as many female patients were on ADDs, it were their male counterparts, who were at increased mortality risk on therapy. Furthermore, males on ADDs had worse adherence to scheduled appointments suggesting the need for special medical attention and possibly psychosocial intervention for this group of patients.
Ernst S, Babu-Narayan S, Bomanji J, et al., 2015, Which multiplexed images can be used to guide invasive electrophysiology procedures?, Congress of the European-Society-of-Cardiology (ESC), Publisher: OXFORD UNIV PRESS, Pages: 34-35, ISSN: 0195-668X
Heng EL, Gatzoulis MA, Smith GC, et al., 2015, Early cardiac remodelling post-pulmonary valve replacement in patients with repaired tetralogy of Fallot, Congress of the European-Society-of-Cardiology (ESC), Publisher: OXFORD UNIV PRESS, Pages: 357-358, ISSN: 0195-668X
Roy K, Ueda A, Gomez F, et al., 2015, Use of remote magnetic navigation and 3D image integration to avoid transbaffle access in patients after total cavopulmonary connection surgery: a single centre experience, Congress of the European-Society-of-Cardiology (ESC), Publisher: OXFORD UNIV PRESS, Pages: 560-560, ISSN: 0195-668X
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Rydman R, Gatzoulis MA, Ho SY, et al., 2015, Systemic Right Ventricular Fibrosis Detected by Cardiovascular Magnetic Resonance Is Associated With Clinical Outcome, Mainly New-Onset Atrial Arrhythmia, in Patients After Atrial Redirection Surgery for Transposition of the Great Arteries, CIRCULATION-CARDIOVASCULAR IMAGING, Vol: 8, ISSN: 1941-9651
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- Citations: 38
Heng EL, Bolger AP, Kempny A, et al., 2015, Neurohormonal activation and its relation to outcomes late after repair of tetralogy of Fallot, HEART, Vol: 101, Pages: 447-454, ISSN: 1355-6037
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- Citations: 24
Ghez O, Dimopoulos K, Babu-Narayan SV, et al., 2015, Repair of tetralogy of Fallot-how much can we achieve with a single operation?, EUROPEAN JOURNAL OF CARDIO-THORACIC SURGERY, Vol: 47, Pages: 535-536, ISSN: 1010-7940
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- Citations: 1
Bedair R, Babu-Narayan SV, Dimopoulos K, et al., 2015, Acceptance and psychological impact of implantable defibrillators amongst adults with congenital heart disease, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 181, Pages: 218-224, ISSN: 0167-5273
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- Citations: 22
Vassiliou V, Heng EL, Donovan J, et al., 2015, Longitudinal stability of gel T1 MRI Phantoms for quality assurance of T1 mapping, Journal of Cardiovascular Magnetic Resonance, Vol: 17, ISSN: 1532-429X
Rydman R, Gatzoulis MA, Kilner PJ, et al., 2015, Systemic right ventricular fibrosis detected by CMR predicts adverse clinical outcome in patients after atrial redirection surgery for transposition of the great arteries, Journal of Cardiovascular Magnetic Resonance, Pages: 1-2, ISSN: 1097-6647
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