Imperial College London

DrSonyaBabu-Narayan

Faculty of MedicineNational Heart & Lung Institute

Reader in Adult Congenital Heart Disease
 
 
 
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Contact

 

+44 (0)20 7351 8803s.babu-narayan

 
 
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Location

 

NIHR Cardiovascular Biomedical RChelsea WingRoyal Brompton Campus

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Summary

 

Publications

Publication Type
Year
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176 results found

Karonis T, Scognamiglio G, Babu-Narayan SV, Montanaro C, Uebing A, Diller GP, Alonso-Gonzalez R, Swan L, Dimopoulos K, Gatzoulis MA, Li Wet 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.

Journal article

Cauldwell M, Von Klemperer K, Uebing A, Swan L, Steer PJ, Babu-Narayan SV, Gatzoulis MA, Johnson MRet al., 2016, A cohort study of women with a Fontan circulation undergoing preconception counselling, Heart, Vol: 102, Pages: 534-540, ISSN: 1468-201X

Journal article

Jensen AS, Broberg CS, Rydman R, Diller GP, Li W, Dimopoulos K, Wort SJ, Pennell DJ, Gatzoulis MA, Babu-Narayan SVet 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.

Journal article

Pennell DJ, Baksi AJ, Prasad SK, Raphael CE, Kilner PJ, Mohiaddin RH, Alpendurada F, Babu-Narayan SV, Schneider J, Firmin DNet 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.

Journal article

Ernst S, Underwood R, Babu-Narayan S, Ben-Haim Set 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

Conference paper

Cordina R, von Klemperer K, Kempny A, Senior R, Celermajer DS, Babu-Narayan S, Gatzoulis M, Li Wet al., 2015, Echocardiographic Predictors of Mortality in Adults With a Fontan Circulation, Publisher: LIPPINCOTT WILLIAMS & WILKINS, ISSN: 0009-7322

Conference paper

Wald RM, Valente AM, Gauvreau K, Babu-Narayan SV, Assenza GE, Schreier J, Gatzoulis MA, Kilner PJ, Koyak Z, Mulder B, Powell AJ, Geva Tet 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

Journal article

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

Journal article

Kempny A, Diller G-P, Dimopoulos K, Alonso-Gonzalez R, Uebing A, Li W, Babu-Narayan S, Swan L, Wort SJ, Gatzoulis MAet 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.

Journal article

Babu-Narayan SV, Giannakoulas G, Valente AM, Li W, Gatzoulis MAet 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.

Journal article

Diller G-P, Kempny A, Alonso-Gonzalez R, Swan L, Uebing A, Li W, Babu-Narayan S, Wort SJ, Dimopoulos K, Gatzoulis MAet 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.

Journal article

Diller G-P, Braeutigam A, Kempny A, Uebing A, Alonso-Gonzalez R, Swan L, Babu-Narayan SV, Baumgartner H, Dimopoulos K, Gatzoulis AAet 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.

Journal article

Ernst S, Babu-Narayan S, Bomanji J, Ben-Haim Set 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

Conference paper

Heng EL, Gatzoulis MA, Smith GC, Shore DF, Sethia B, Uemura H, Diller GP, Ho SY, Pennell DJ, Babu-Narayan SVet 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

Conference paper

Roy K, Ueda A, Gomez F, Suman-Horduna I, Babu-Narayan S, Ernst Set 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

Conference paper

Rydman R, Gatzoulis MA, Ho SY, Ernst S, Swan L, Li W, Wong T, Sheppard M, McCarthy KP, Roughton M, Kilner PJ, Pennell DJ, Babu-Narayan SVet 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

Journal article

Heng EL, Bolger AP, Kempny A, Davlouros PA, Davidson S, Swan L, Uebing A, Pennell DJ, Gatzoulis MA, Babu-Narayan SVet 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

Journal article

Ghez O, Dimopoulos K, Babu-Narayan SV, Gatzoulis Met 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

Journal article

Bedair R, Babu-Narayan SV, Dimopoulos K, Quyam S, Doyle A-M, Swan L, Gatzoulis MA, Wong Tet 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

Journal article

Vassiliou V, Heng EL, Donovan J, Greiser A, Babu-Narayan SV, Gatzoulis MA, Firmin D, Pennell DJ, Gatehouse P, Prasad SKet 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

Journal article

Rydman R, Gatzoulis MA, Kilner PJ, Pennell DJ, Babu-Narayan SVet 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

Journal article

Heng EL, Kellman P, Gatzoulis MA, Moon J, Gatehouse P, Babu-Narayan SVet al., 2015, Pilot data of right ventricular myocardial T1 quantification by free-breathing fat-water separated dark blood saturation-recovery imaging, Journal of Cardiovascular Magnetic Resonance, Pages: 1-2, ISSN: 1097-6647

Journal article

Heng EL, Kellman P, Mohiaddin R, Alpendurada F, Kilner PJ, Pennell DJ, Gatzoulis MA, Keegan J, Babu-Narayan SVet al., 2015, The utility of free-breathing, motion-corrected late gadolinium enhancement for right ventricular fibrosis imaging in congenital heart disease, Journal of Cardiovascular Magnetic Resonance, Pages: 1-2, ISSN: 1097-6647

Journal article

Rydman R, Shiina Y, Gatzoulis MA, Pennell DJ, Kilner PJ, Babu-Narayan SVet al., 2015, Predicting atrial tachycardia and major cardiovascular events in adults with unrepaired Ebstein's anomaly of the tricuspid valve, Journal of Cardiovascular Magnetic Resonance, Pages: 1-2, ISSN: 1097-6647

Journal article

Moceri P, Kempny A, Liodakis E, Gonzales RA, Germanakis I, Diller G-P, Swana L, Marino PS, Wort SJ, Babu-Narayan SV, Ferrari E, Gatzoulis MA, Li W, Dimopoulos Ket al., 2015, Physiological differences between various types of Eisenmenger syndrome and relation to outcome, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 179, Pages: 455-460, ISSN: 0167-5273

Journal article

Keegan J, Gatehouse PD, Haldar S, Wage R, Babu-Narayan SV, Firmin DNet al., 2015, Dynamic Inversion Time for Improved 3D Late Gadolinium Enhancement Imaging in Patients with Atrial Fibrillation, Magnetic Resonance in Medicine, Vol: 73, Pages: 646-654, ISSN: 1522-2594

Purpose—High resolution 3D late gadolinium enhancement (LGE) imaging is performed withsingle R-wave gating to minimise lengthy acquisition durations. In patients with atrial fibrillation(AF), heart rate variability results in variable magnetisation recovery between sequence repeatsand image quality is often poor. In this study, we implemented and tested a dynamic inversiontime scheme designed to reduce sequence sensitivity to heart rate variations.Methods—An inversion-prepared 3D segmented gradient echo sequence was modified so thatthe inversion time (TI) varied automatically from beat-to-beat (dynamic-TI) based on the timesince the last sequence repeat. 3D LGE acquisitions were performed in 17 patients prior to RFablation of persistent AF both with and without dynamic-TI. Qualitative image quality scores,blood signal-to-ghosting ratios (SGRs) and blood-myocardium contrast-to-ghosting ratios (CGRs)were compared.Results—Image quality scores were higher with dynamic-TI than without (2.2 +/− 0.9 versus 1.8+/− 1.1, p = 0.008) as were blood-myocardium CGRs (13.8 +/− 7.6 versus 8.3 +/− 6.1, p = 0.003)and blood SGRs (19.6 +/− 8.5 versus 13.1 +/− 8.0, p = 0.003).Conclusions—The dynamic-TI algorithm improves image quality of 3D LGE imaging in thisdifficult patient population by reducing the sequence sensitivity to RR interval variations.

Journal article

Pennell DJ, Baksi AJ, Kilner PJ, Mohiaddin RH, Prasad SK, Alpendurada F, Babu-Narayan SV, Neubauer S, Firmin DNet al., 2014, Review of Journal of Cardiovascular Magnetic Resonance 2013, Journal of Cardiovascular Magnetic Resonance, Vol: 16, ISSN: 1532-429X

There were 109 articles published in the Journal of Cardiovascular Magnetic Resonance (JCMR) in 2013, which is a21% increase on the 90 articles published in 2012. The quality of the submissions continues to increase. The editorsare delighted to report that the 2012 JCMR Impact Factor (which is published in June 2013) has risen to 5.11, upfrom 4.44 for 2011 (as published in June 2012), a 15% increase and taking us through the 5 threshold for the firsttime. The 2012 impact factor means that the JCMR papers that were published in 2010 and 2011 were cited onaverage 5.11 times in 2012. The impact factor undergoes natural variation according to citation rates of papers inthe 2 years following 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 accordancewith Open-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 calendaryear to summarize the papers for the readership into broad areas of interest or theme, so that areas of interestcan be reviewed in a single article in relation to each other and other recent JCMR articles. The papers arepresented in broad themes and set in context with related literature and previously published JCMR papers toguide continuity of thought in the journal. We hope that you find the open-access system increases widerreading and citation of your papers, and that you will continue to send your quality manuscripts to JCMR forpublication.

Journal article

Cambronero Cortinas E, Garcia Guereta Silva L, Rivero Jimenez N, Deiros Bronte L, Bret Zurita M, Sanz Pascual E, Labrandero C, Corbi Pascual M, Arreo Del Val V, Gutierrez Laraya Aguado F, Shimada E, West C, Babu-Narayan S, Li W, Castro M, Abecasis J, Santos M, Reis C, Ribeiras R, Marques M, Andrade M, Abecasis M, Mendes M, Karvounaris S, Karampetsos V, Michas G, Argirou A, Mavrommatis P, Pereira VH, Portugues J, Calvo L, Ferreira F, Machado I, Lourenco M, Lourenco A, Bercu G, Tamisier Det al., 2014, Case-based session: new ways to look at old problems in cardiac heart disease: Saturday 6 December 2014, 11:00-12:30Location: Agora., Eur Heart J Cardiovasc Imaging, Vol: 15 Suppl 2, Pages: ii230-ii231

Journal article

Heng EL, Babu-Narayan SV, 2014, Repaired tetralogy of fallot, Cardiac CT and MR for Adult Congenital Heart Disease, Pages: 199-224, ISBN: 9781461488743

© 2014 Springer Science+Business Media New York. All rights reserved. The anatomy of tetralogy of Fallot (TOF), together with the pathophysiological consequences, was first described by Etienne-Louis Fallot in 1888. The tetrad of overriding aorta, right ventricular (RV) outflow tract obstruction, ventricular septal defect (VSD) and consequent RV hypertrophy is all due to antero-cephalad deviation of the outlet septum during fetal development. TOF is the most common cyanotic congenital heart disease (CHD), accounting for 10 % of CHD patients and occurring in 1 in 3,600 births (Shinebourne et al., Heart 92:1353-1359, 2006). It carries a recurrence risk of 3 % in siblings. There is genetic microdeletion in 22q11 in 15-25 % of TOF patients in whom TOF is part of DiGeorge syndrome. There is a spectrum of morphology, despite the four features that comprise 'tetralogy', and the severity of the RV outflow tract (RVOT) obstruction is the major determinant of first clinical presentation. Surgical palliation was achieved using the Blalock-Taussig shunt (subclavian artery to ipsilateral pulmonary artery connection) in 1944, a landmark event as these patients underwent the first cardiac surgery. Subsequently, surgical repair was described in the 1950s with the advent of cardiopulmonary bypass. Primary surgical repair involves patch closure of the VSD and intervention to the RVOT to relieve obstruction. RV muscle bundles are resected. Depending on the size of the outflow tract, pulmonary valve and pulmonary arteries; an RVOT patch, transannular patch and/or pulmonary artery (PA) patch may be required for RVOT reconstruction. In the variants with anomalous coronary arteries or in pulmonary atresia-type Fallot, an RV to PA conduit may be used.

Book chapter

Menichetti F, Spadotto V, Zorzi A, Kempny A, Horduna IS, Mantziari L, Leventopoulos G, Babu-Narayan S, Ernst Set al., 2014, Predictors of supraventricular arrhythmias (SA) and outcome of catheter ablation in patients with ebstein anomaly (EA): a single-center experience, Annual Meeting of the European-Society-of-Cardiology (ESC), Publisher: OXFORD UNIV PRESS, Pages: 584-584, ISSN: 0195-668X

Conference paper

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