Anselm Uebing | Imperial College London (original) (raw)

Papers by Anselm Uebing

Research paper thumbnail of Recurrent Wolff-Parkinson-White syndrome in Ebstein anomaly previously treated by transcatheter replacement of tricuspid valve: is catheter ablation of the accessory pathway technically possible?

Archives of Cardiovascular Diseases Supplements, 2017

The mean age was 30 days and an average weight of 3.2kg. One of the essential elements is the Nak... more The mean age was 30 days and an average weight of 3.2kg. One of the essential elements is the Nakata index and the size of the pulmonary ring is much lower (see Table), with a Nakata at 120 and a ring Lung with a z-score at-3. What also makes symptomatic children rapidly with essentially progressive cyanosis, the saturation before treatment was 68% which remains the main surgical indication far behind the Fallot cyanotic crisis. In group 2 (n22) the majority were symptomatic 86% mainly the fallot cyanotic crisis 45%, an average saturation of 86%.Patients in this group were all treated at a complete repair with an age of 80.6 days (39-105) and a weight of 4,6kg (3.5-5.6).They had a favorable Nakata 176 and a favorable pulmonary ring z-score at 1.4. Conclusion In this retrospective monocentric study of this cases of Fallot operated before 3 months, we observed the feasibility of a complete repair after the neonatal period but only in the favorable forms with a superior Nakata index and moderate cyanosis. During neonatal period, in the presence of cyanosed children with an unfavorable Nakata, a palliation stage is necessary with a promising result even if there is much reintervention and postoperative complication.

Research paper thumbnail of Serial right ventricular assessment in patients with hypoplastic left heart syndrome: a multiparametric cardiovascular magnetic resonance study

European Journal of Cardio-Thoracic Surgery, 2021

OBJECTIVES The aim of this study was to assess single right ventricular (RV) function in a larg... more OBJECTIVES The aim of this study was to assess single right ventricular (RV) function in a large cohort of hypoplastic left heart syndrome (HLHS) patients after the completion of total cavopulmonary connection by analysing serial cardiovascular magnetic resonance (CMR) studies. METHODS CMR studies from 95 HLHS patients were analysed. RV end-diastolic and end-systolic volumes (RVEDV, RVESV), ejection fraction (RVEF) and long-axis strain (LAS) were measured from cine images. RESULTS All 95 patients had at least 2 CMR scans and 35 patients had 3 CMR scans. The median age (first quartile–third quartile) at the 3 examinations was 4.2 (3.3–6.1), 9.4 (6.1–11.4) and 14.6 (11.8–16.8) years. RV indexed volumes (RVEDVi and RVESVi) increased from first to the second and from the first and second examination to the third examination in patients with >10 years of age (P < 0.05). There was a slight decrease in RVEF and LAS throughout the examinations, but this was not statistically signifi...

Research paper thumbnail of Predicting Survival in Repaired Tetralogy of Fallot

JACC: Cardiovascular Imaging, 2021

Objectives This study sought to identify patients with repaired tetralogy of Fallot (rTOF) at hig... more Objectives This study sought to identify patients with repaired tetralogy of Fallot (rTOF) at high risk of death and malignant ventricular arrhythmia (VA). Background To date there is no robust risk stratification scheme to predict outcomes in adults with rTOF. Methods Consecutive patients were prospectively recruited for late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) to define right and left ventricular (RV, LV) fibrosis in addition to proven risk markers. Results The primary endpoint was all-cause mortality. Of the 550 patients (median age 32 years, 56% male), 27 died (mean follow-up 6.4 ± 5.8; total 3,512 years). Mortality was independently predicted by RVLGE extent, presence of LVLGE, RV ejection fraction ≤47%, LV ejection fraction ≤55%, B-type natriuretic peptide ≥127 ng/L, peak exercise oxygen uptake (V02) ≤17 mL/kg/min, prior sustained atrial arrhythmia, and age ≥50 years. The weighted scores for each of the preceding independent predictors differentiated a high-risk subgroup of patients with a 4.4%, annual risk of mortality (area under the curve [AUC]: 0.87; P < 0.001). The secondary endpoint (VA), a composite of life-threatening sustained ventricular tachycardia/resuscitated ventricular fibrillation/sudden cardiac death occurred in 29. Weighted scores that included several predictors of mortality and RV outflow tract akinetic length ≥55 mm and RV systolic pressure ≥47 mm Hg identified high-risk patients with a 3.7% annual risk of VA (AUC: 0.79; P < 0.001) RVLGE was heavily weighted in both risk scores caused by its strong relative prognostic value. Conclusions We present a score integrating multiple appropriately weighted risk factors to identify the subgroup of patients with rTOF who are at high annual risk of death who may benefit from targeted therapy.

Research paper thumbnail of Reference Values for Ventricular Volumes and Pulmonary Artery Dimensions in Pediatric Patients with Transposition of the Great Arteries After Arterial Switch Operation

Journal of Magnetic Resonance Imaging, 2021

Background: Pulmonary artery (PA) anatomy in patients with transposition of the great arteries (T... more Background: Pulmonary artery (PA) anatomy in patients with transposition of the great arteries (TGA) after arterial switch operation (ASO) with Lecompte manoeuvre is different compared to healthy subjects, and stenoses of the PA are common. Magnetic resonance imaging (MRI) is an excellent imaging modality to assess PA anatomy in TGA patients. However, disease-specific reference values for PA size are scarce. Purpose: To establish disease-specific reference ranges for PA dimensions and for biventricular volumes and mass. Study Type: Retrospective. Subjects: A total of 69 pediatric patients with TGA after ASO (median age 12.6 years; range 5-17.8 years; 13 females and 56 males). Field Strength/Sequence: 3.0 T, steady-state free precession (SSFP) and gradient echo cine sequences and fourdimensional time-resolved magnetic resonance angiography with keyhole. Assessment: Right and left PA (RPA, LPA) were each measured at three locations during its course around the aorta. Ventricular volumes, mass, and ejection fraction were measured from a stack of short axis cine images. Statistical Tests: The lambda-mu-sigma (LMS) method of Cole and Green, univariate and multivariate linear models, and t-test. Results: Centile graphs and tables for PA dimensions, biventricular volumes, mass, and ejection fraction were created. Univariate linear analysis showed significant associations (P < 0.05) between body surface area (BSA), height, and weight with systolic MPA and RPA diameter. In multivariate linear analysis, only BSA remained a strong predictor for main PA and RPA diameters. For biventricular volumes, the univariate linear model revealed a strong influence of BSA, height, weight, and age (all P < 0.05). On multivariate linear analysis, only body height remained associated. Data Conclusion: Uni-and multivariate linear analyses showed a strong association between BSA and PA diameters, as well as between height and biventricular volumes, and therefore, centile tables and graphs are presented accordingly. Our data may improve MR image interpretation and may serve as a reference in future studies. Level of Evidence: 4 Technical Efficacy Stage: 2

Research paper thumbnail of Cardiovascular magnetic resonance normal values in children for biventricular wall thickness and mass

Journal of Cardiovascular Magnetic Resonance, 2021

Background Pediatric patients are becoming increasingly referred for cardiovascular magnetic reso... more Background Pediatric patients are becoming increasingly referred for cardiovascular magnetic resonance (CMR). Measurement of ventricular wall thickness is typically part of the assessment and can be of diagnostic importance, e.g. in arterial hypertension. However, normal values for left ventricular (LV) and right ventricular (RV) wall thickness in pediatric patients are lacking. The aim of this study was to establish pediatric centile charts for segmental LV and RV myocardial thickness in a retrospective multicenter CMR study. Methods CMR was performed in 161 healthy children and adolescents with an age range between 6 and 18 years from two centers in the UK and Germany as well as from a previously published CMR project of the German Competence Network for Congenital Heart Defects. LV myocardial thickness of 16 segments was measured on the short axis stack using the American Heart Association segmentation model. In addition, the thickness of the RV inferior and anterior free wall as...

Research paper thumbnail of 328Pulmonary valve replacement in England: trends and outcome over the past two decades

European Heart Journal, Aug 1, 2017

Long-term management after Tetralogy of Fallot repair / After pulmonary embolism: optimising trea... more Long-term management after Tetralogy of Fallot repair / After pulmonary embolism: optimising treatment and follow up p=0.07) and in those with documented vegetations on echocardiography (75% vs. 44%; p=0.09). On multivariate regression analysis, non-streptococcal IE was an independent predictor of death/re-intervention (HR, 12.0 [95% CI: 1.9-76.4]; p=0.009). Rate and time-to-onset of IE after TPVI Conclusions: The risk of IE after Melody valve implantation, albeit considerable, is lower than previously reported. Surgical/percutaneous re-intervention is a common consequence of IE, although many patients can be managed medically.

Research paper thumbnail of Coarctation of the Aorta

Updates in Hypertension and Cardiovascular Protection, 2016

Coarctation of the aorta (CoA) is a congenital malformation of the aorta, first described by Morg... more Coarctation of the aorta (CoA) is a congenital malformation of the aorta, first described by Morgagni in 1760 [1]. The overall incidence of CoA is 5–8 % of all congenital heart disease, and the condition is 1.7 times more frequent in white males than white females [2]. Aortic coarctation can present as a localised discrete stenosis of the aortic isthmus, but segmental tubular hypoplasia of the aortic arch is also commonly associated. The usual site of CoA is distal to the left subclavian artery.

Research paper thumbnail of MODERATED POSTER SESSION: Deformation and multimodality imaging in congenital heart: Thursday 4 December 2014, 08:30-18:00 * Location: Moderated Poster area

European Heart Journal - Cardiovascular Imaging, 2014

Aim: There is wealth of data on the echocardiographic evaluation of prosthetic valves in the aort... more Aim: There is wealth of data on the echocardiographic evaluation of prosthetic valves in the aortic or mitral position. Similar data are missing for the Melody percutaneous pulmonary valve system (MPPV). We aimed to correlate the anatomic appearance of the MPPV with the haemodynamic result on invasive and echocardiographic assessment. Patients and Methods: All 45 patients who underwent MPPV implantation at our institution between 2007-2013 were studied (median age 30 [range 11-61] years). All patients had complex congenital heart disease. The narrowest dimensions of the MPPV system were taken from the final biplane orthogonal fluoroscopic images to calculate the effective valve opening area (EOA). The post implantation invasive peak-to-peak gradient (DPp-p) and the maximal and mean Doppler gradient (DPmax and DPmean) on the pre-discharge echocardiogram (within 72 hours of implantation) were also obtained. Results: DPp-p after valve implantation was low (11.5+5.2 [range 2-20] mmHg) and there was no significant residual pulmonary regurgitation. The peak and mean gradient across the valve by Doppler assessment were significantly higher than DPp-p (DPmean: 19.3+6.5, DPmax: 33.3+8.2 mmHg; P,0.0001 for both vs. DPp-p). Both Doppler gradients correlated significantly with the invasive peak gradient (DPp-p vs. DPmean: r=0.37, P=0.03; DPp-p vs. DPmax: r=0.39, P=0.02) The EOA of the valve system indexed to body surface area was 132+30 mm2/m2. There were only weak relationships between the indexed EOA and the invasive and Doppler gradients (correlations with EOA: DPp-p: r=-0.32, P=0.06; DPpeak: r=-0.33, P=0.045; DPmean: r=-0.23; P=0.16). Conclusion: Doppler assessment of the percutaneous Melody valve early after implantation consistently overestimates the invasive peak-to-peak gradient. Simple estimates of valve size do not allow prediction of central haemodynamics. These results reflect the complex and variable anatomy of the right ventricular outflow tract/ main pulmonary artery in this group of patients.

Research paper thumbnail of Heart Rate Response During Exercise Predicts Survival in Adults With Congenital Heart Disease

Journal of the American College of Cardiology, 2006

OBJECTIVES To assess the prognostic value of heart rate response to exercise in adult congenital ... more OBJECTIVES To assess the prognostic value of heart rate response to exercise in adult congenital heart disease (ACHD) patients. BACKGROUND An abnormal heart rate response to exercise is related to autonomic dysfunction and may have prognostic implications in ACHD. METHODS We identified 727 consecutive ACHD patients (mean age [Ϯ SD] 33 Ϯ 13 years) with varying diagnoses and without pacemakers. Peak oxygen consumption (peak VO 2), resting heart rate, and the increase in heart rate from resting level to peak exercise ("heart rate reserve") were measured. We also quantified the decrease in heart rate ("heart rate recovery") after cessation of exercise. RESULTS During a median follow-up of 28 months, 38 patients died. Lower values of heart rate reserve, peak heart rate, heart rate recovery, and peak VO 2 (p Ͻ 0.01 for each) were associated with increased mortality in univariate analysis. Furthermore, heart rate reserve predicted mortality independently of antiarrhythmic therapy, functional class, and peak VO 2. Stratifying patients by diagnostic groups revealed that a lower heart rate reserve was also associated with a greater risk of death in patients with complex anatomy, Fontan circulation, and tetralogy of Fallot (p Ͻ 0.05 for each). CONCLUSIONS An abnormal heart rate response to exercise identifies ACHD patients with a higher risk of mortality in the midterm, even after accounting for antiarrhythmic medication and exercise capacity. Heart rate reserve is a simple and inexpensive way to identify ACHD patients at higher mortality risk.

Research paper thumbnail of Impaired heart rate response to exercise in adult patients with a systemic right ventricle or univentricular circulation: Prevalence, relation to exercise, and potential therapeutic implications

International Journal of Cardiology, 2009

Introduction: Exercise limitation is common in patients with a systemic right ventricle or univen... more Introduction: Exercise limitation is common in patients with a systemic right ventricle or univentricular circulation and may be related to chronotropic incompetence (CI). We aimed to investigate the association of CI with exercise capacity, and evaluate whether CI is causally related to exercise intolerance. Patients and methods: Cardiopulmonary exercise tests were performed in patients with a systemic right ventricle (n = 32) or univentricular circulation (n = 32). CI was defined as the inability to achieve 80% of age predicted maximal heart rate reserve ([peak heart rate-resting heart rate]/[220 − age − resting heart rate]). The linearity of the relation between oxygen consumption (VO 2) and heart rate (oxygen pulse) was assessed visually and separately quantified by calculating the quadratic regression coefficient (describing departure from linearity). Results: The prevalence of CI was 59% and 84% in patients with a systemic right ventricle or univentricular circulation, respectively. Patients with CI had a lower peak VO 2 (19.8 ± 5.5 vs. 24.6 ± 6.8 ml/kg/min, P = 0.005), and shorter exercise duration (587 ± 165 vs. 749 ± 176 s, P = 0.001) than those without CI. Oxygen pulse kinetics suggested exercise limitation due to CI in 8 of 43 patients (19%). In contrast, 13 of 43 patients (30%) had oxygen pulse kinetics suggestive of exercise limitation due to ventricular dysfunction or cyanosis. Discussion: Chronotropic incompetence is common and associated with exercise limitation in patients with a systemic right ventricle or univentricular circulation. Visual or mathematical assessment of oxygen pulse kinetics identifies patients in whom an attenuated heart-rate response is responsible for poor exercise capacity and may have therapeutic implications.

Research paper thumbnail of Abnormal Ventilatory Response to Exercise in Adults With Congenital Heart Disease Relates to Cyanosis and Predicts Survival

Circulation, 2006

Background— Limited data exist with which to stratify risk in adult congenital heart disease (ACH... more Background— Limited data exist with which to stratify risk in adult congenital heart disease (ACHD). An increased ventilatory response to exercise, expressed as ventilation per unit of carbon dioxide production (V̇ e /V̇ co 2 slope), is an established predictor of impaired survival in acquired heart disease. We sought to establish the distribution, relation to cyanosis, and prognostic value of the V̇ e /V̇ co 2 slope across a wide spectrum of ACHD patients. Methods and Results— Five hundred sixty ACHD patients of varying diagnoses and 50 healthy controls underwent cardiopulmonary exercise testing at a single laboratory between 2001 and 2004. Patient age was 33.2±12.9 years (mean±SD). Peak oxygen consumption was 23.5±9.0 mL · kg −1 · min −1 . V̇ e /V̇ co 2 slope for all patients was 36.3±15.3. The slope was raised in all ACHD groups compared with controls and was 73% higher in cyanotic patients. Cyanosis, with or without pulmonary arterial hypertension, was the strongest predictor of...

Research paper thumbnail of P733Echocardiographic predictors of outcome in repaired Tetralogy of Fallot patients over 40 years old

European Heart Journal, 2017

Understanding the pulmonary circulation / Imaging arrhythmias in congenital heart disease 149 was... more Understanding the pulmonary circulation / Imaging arrhythmias in congenital heart disease 149 was significantly correlated with RVsm (r:-0.36, p:0.005). RA, RA/PCWP and PAPi did not reveal any correlation. Subgroup analysis of patients with atrial fibrillation (n=30) did not reveal any of the mentioned correlations of new hemodynamic parameters Conclusions: PAE, PAC, RSWI and PAPP are applicative new hemodynamic parameters to reveal RV dysfunction in patients with PH. However, presence of atrial fibrillation leads to the loss of associations between these parameters and conventional prognostic markers

Research paper thumbnail of Tissue Engineered Transcatheter Pulmonary Valved Stent Implantation: Current State and Future Prospect

International Journal of Molecular Sciences, 2022

Patients with the complex congenital heart disease (CHD) are usually associated with right ventri... more Patients with the complex congenital heart disease (CHD) are usually associated with right ventricular outflow tract dysfunction and typically require multiple surgical interventions during their lives to relieve the right ventricular outflow tract abnormality. Transcatheter pulmonary valve replacement was used as a non-surgical, less invasive alternative treatment for right ventricular outflow tract dysfunction and has been rapidly developing over the past years. Despite the current favorable results of transcatheter pulmonary valve replacement, many patients eligible for pulmonary valve replacement are still not candidates for transcatheter pulmonary valve replacement. Therefore, one of the significant future challenges is to expand transcatheter pulmonary valve replacement to a broader patient population. This review describes the limitations and problems of existing techniques and focuses on decellularized tissue engineering for pulmonary valve stenting.

Research paper thumbnail of Analytical Identification of Ideal Pulmonary-Systemic Flow Balance in Patients With Bidirectional Cavopulmonary Shunt and Univentricular Circulation: Oxygen Delivery or Tissue Oxygenation?

Circulation, 2006

Background— In the present study, we extended previous mathematical modeling work on patients wit... more Background— In the present study, we extended previous mathematical modeling work on patients with bidirectional cavopulmonary (“bidirectional Glenn”) anastomosis to assess the potential utility of several descriptors of oxygen status. We set out to determine which of these descriptors best represents the overall tissue oxygenation. We also introduce a new descriptor, S o 2 min, defined as the lower of the superior and inferior vena cava oxygen saturations. Methods and Results— The application of differential calculus to a model of oxygen physiology of patients with bidirectional Glenn allowed simultaneous assessment of all possible distributions of blood flow and metabolic rate between upper and lower body, across all cardiac outputs, total metabolic rates, and oxygen-carrying capacities. When total cardiac output is fixed, although it may intuitively seem best to distribute flow to maximize oxygen delivery (total, upper body, or lower body), we found that for each variable, there ...

Research paper thumbnail of Interventional creation of an atrial septal defect and its impact on right ventricular function: an animal study with the pressure-volume conductance system

Cardiology journal, 2011

BACKGROUND The aim of our study was to assess the suitability of different interventional techniq... more BACKGROUND The aim of our study was to assess the suitability of different interventional techniques to create an atrial septal defect (ASD) and to evaluate the short-term effects of right ventricular (RV) volume overload on RV contractility in the growing swine. METHODS Thirteen ASD and six control animals were studied. An ASD was created by balloon dilatation (BD) of the fossa ovalis (n = 4) or by implantation of a multi-perforated Amplatzer Septal Occluder (n = 4) or a patch-less nitinol device (n = 5). After 4.8 (3.9-6.0) weeks, the amounts of left-to-right shunting (Qp/Qs) and RV contractility (end systolic elastance - Ees) were assessed. RESULTS In the ASD group, a significant left-to-right shunt could be documented (Qp/Qs 1.5 ± ± 0.4). However, a shunt was absent in the BD subgroup (Qp/Qs 1.1 ± 0.1). In animals with devices implanted, a significant relationship between the post-mortem ASD area and Qp/Qs was found (r = 0.68, p < 0.05). Compared to controls, RV contractility...

Research paper thumbnail of Catheter ablation for patients with end-stage complex congenital heart disease or cardiomyopathy considered for transplantation: Trials and tribulations

International Journal of Cardiology

INTRODUCTION Arrhythmia contributes significantly to morbidity and mortality of patients with con... more INTRODUCTION Arrhythmia contributes significantly to morbidity and mortality of patients with congenital heart disease (CHD) or cardiomyopathy (CMP). It also has the potential to worsen symptoms and is particularly detrimental to patients with advanced heart failure awaiting cardiac transplantation. We report our experience using catheter ablation to treat recurrent arrhythmia in patients with CHD or CMP considered for transplantation. METHODS Five consecutive patients (3 female, mean age 47.8 ± 12.8 years) with complex CHD or CMP (tricuspid atresia, mitral atresia, double inlet left ventricle, arrhythmogenic right ventricular cardiomyopathy, left ventricular non-compaction) presented with either atrial (n = 3) or ventricular (n = 2) arrhythmias. All ablations were guided by three-dimensional (3D) electro-anatomical mapping, plus remote magnetic navigation in 3 patients. RESULTS Patients underwent a median of 2 ablation procedures for a total number of 26 tachycardias. None of the 5 patients experienced further arrhythmia at a median of 939 days (range 4-1375) from their last ablation. During a median follow up of 31 months (range 1-70), three patients underwent successful transplantation at 1375, 1062 and 321 days following their last ablation. One patient with a Fontan circulation died from hepatic cancer and one from end-stage heart failure despite urgent transplant listing. CONCLUSIONS Catheter ablation is feasible in complex cardiac patients considered for heart transplantation and should be offered for rhythm management and patient optimization until a suitable donor is found.

Research paper thumbnail of Surgical and percutaneous pulmonary valve replacement in England over the past two decades

Heart

ObjectivePulmonary valve replacement (PVR) is often required in patients with congenital heart di... more ObjectivePulmonary valve replacement (PVR) is often required in patients with congenital heart disease. We aimed to describe temporal trends in PVR in a nationwide English cohort between 1997 and 2014, survival and the need for re-PVR.MethodsPatients were identified in the Hospital Episode Statistics Database. Survival data were retrieved from the UK Office for National Statistics.ResultsA total of 2733 patients underwent PVR (2845 procedures) over the study period. Median age at first procedure increased from 20.1 years in 1997–2005 to 24.7 years in 2006–2014. The annual number of PVRs increased from 23 in 1997 to 251 in 2014. Homografts were the most common choice in the early years, but the use of xenografts increased after 2005. During a median follow-up of 5.8 years, 176 patients died and 108 required redo PVR. Early (30 day) survival was 98% for all PVRs and was similar for all types of prostheses but longer-term mortality dropped to 92% at 10 years and 90% at 15 years. Age &g...

Research paper thumbnail of The outcome of adults born with pulmonary atresia: High morbidity and mortality irrespective of repair

International Journal of Cardiology

To describe the characteristics and long-term outcome of a large adult cohort with pulmonary atre... more To describe the characteristics and long-term outcome of a large adult cohort with pulmonary atresia. Patients with pulmonary atresia (PA) are a heterogeneous population in terms of anatomy, physiology and surgical history, and their management during adulthood remains challenging. Data on all patients with PA followed in our center between January 2000 and March 2015 were recorded. Patients were classified into the following groups: PA with ventricular septal defect (PA-VSD, 1), PA with intact ventricular septum (PA-IVS, 2) and other miscellaneous PA (PA-other, 3). Two-hundred twenty-seven patients with PA were identified, 66.1% female, mean age 25.5 ± 8.7 years. Over a median follow-up of 8.8 years, 49 (21.6%) patients had died: heart failure (n = 21, 42.8%) and sudden cardiac death (n = 8, 16.3%) were the main causes. There was no significant difference in mortality between the 3 Groups (p = 0.12) or between repaired and unrepaired patients in Group 1 (p = 0.16). Systemic ventricular dysfunction and resting oxygen saturations were the strongest predictors of mortality. Additionally, 116 (51%) patients were hospitalized, driven mainly by the need for invasive procedures, heart failure and arrhythmias. Adult survivors with pulmonary atresia have a high morbidity and mortality irrespective of underlying cardiac anatomy and previous reparative or palliative surgery. We present herewith predictors of outcome in adult life that may assist with their tertiary adult congenital care.

Research paper thumbnail of New York Heart Association (NYHA) classification in adults with congenital heart disease: relation to objective measures of exercise and outcome

European Heart Journal - Quality of Care and Clinical Outcomes

The New York Heart Association functional classification (NYHA class) is often used to describe t... more The New York Heart Association functional classification (NYHA class) is often used to describe the functional capacity of adults with congenital heart disease (ACHD), albeit with limited evidence on its validity in this heterogeneous population. We aimed to validate the NYHA functional classification in ACHD by examining its relation to objective measures of limitation using cardiopulmonary exercise testing (CPET) and mortality.

Research paper thumbnail of Preconception couselling, predicting risk and outcomes in women with mwho 3 and 4 heart disease

International Journal of Cardiology, 2017

All women with CHD, especially those with more severe disease, should be offered preconception co... more All women with CHD, especially those with more severe disease, should be offered preconception counseling (PCC), to discuss the risk of complications and to plan a future pregnancy. Several scoring system have been devised to estimate the risk of adverse events in pregnancies complicated by maternal heart disease (HD) and while comparisons have been made across the whole population, none have focused on the high-risk population. Retrospective cohort study that included women classed as modified WHO (mWHO) 3 and 4 who had a pregnancy from at least 20weeks gestation between 1994 and 2015 managed within our institution. We assessed how well the quoted risk (at PCC) of an adverse event (maternal or fetal) related to the actual rate of occurrence. We calculated NYHA and CARPREG scores for all patients, and the clinician assessment of percentage risk, to predict the occurrence of an adverse outcome. We identified 76 mWHO 3 and 4 women who had a total of 102 pregnancies. However, only in 63 pregnancies had the woman attended PCC. Both maternal and fetal adverse events were common. NYHA did not significantly predict any adverse events, whilst a CARPREG score of &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;gt;3 score predicted heart failure and mWHO4 score predicted maternal death. However, the best prediction of adverse outcomes was a composite quoted risk (percent) given at PCC. Women must have access to PCC as those with worse CARPREG and mWHO scores encounter greater adverse events.

Research paper thumbnail of Recurrent Wolff-Parkinson-White syndrome in Ebstein anomaly previously treated by transcatheter replacement of tricuspid valve: is catheter ablation of the accessory pathway technically possible?

Archives of Cardiovascular Diseases Supplements, 2017

The mean age was 30 days and an average weight of 3.2kg. One of the essential elements is the Nak... more The mean age was 30 days and an average weight of 3.2kg. One of the essential elements is the Nakata index and the size of the pulmonary ring is much lower (see Table), with a Nakata at 120 and a ring Lung with a z-score at-3. What also makes symptomatic children rapidly with essentially progressive cyanosis, the saturation before treatment was 68% which remains the main surgical indication far behind the Fallot cyanotic crisis. In group 2 (n22) the majority were symptomatic 86% mainly the fallot cyanotic crisis 45%, an average saturation of 86%.Patients in this group were all treated at a complete repair with an age of 80.6 days (39-105) and a weight of 4,6kg (3.5-5.6).They had a favorable Nakata 176 and a favorable pulmonary ring z-score at 1.4. Conclusion In this retrospective monocentric study of this cases of Fallot operated before 3 months, we observed the feasibility of a complete repair after the neonatal period but only in the favorable forms with a superior Nakata index and moderate cyanosis. During neonatal period, in the presence of cyanosed children with an unfavorable Nakata, a palliation stage is necessary with a promising result even if there is much reintervention and postoperative complication.

Research paper thumbnail of Serial right ventricular assessment in patients with hypoplastic left heart syndrome: a multiparametric cardiovascular magnetic resonance study

European Journal of Cardio-Thoracic Surgery, 2021

OBJECTIVES The aim of this study was to assess single right ventricular (RV) function in a larg... more OBJECTIVES The aim of this study was to assess single right ventricular (RV) function in a large cohort of hypoplastic left heart syndrome (HLHS) patients after the completion of total cavopulmonary connection by analysing serial cardiovascular magnetic resonance (CMR) studies. METHODS CMR studies from 95 HLHS patients were analysed. RV end-diastolic and end-systolic volumes (RVEDV, RVESV), ejection fraction (RVEF) and long-axis strain (LAS) were measured from cine images. RESULTS All 95 patients had at least 2 CMR scans and 35 patients had 3 CMR scans. The median age (first quartile–third quartile) at the 3 examinations was 4.2 (3.3–6.1), 9.4 (6.1–11.4) and 14.6 (11.8–16.8) years. RV indexed volumes (RVEDVi and RVESVi) increased from first to the second and from the first and second examination to the third examination in patients with >10 years of age (P < 0.05). There was a slight decrease in RVEF and LAS throughout the examinations, but this was not statistically signifi...

Research paper thumbnail of Predicting Survival in Repaired Tetralogy of Fallot

JACC: Cardiovascular Imaging, 2021

Objectives This study sought to identify patients with repaired tetralogy of Fallot (rTOF) at hig... more Objectives This study sought to identify patients with repaired tetralogy of Fallot (rTOF) at high risk of death and malignant ventricular arrhythmia (VA). Background To date there is no robust risk stratification scheme to predict outcomes in adults with rTOF. Methods Consecutive patients were prospectively recruited for late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) to define right and left ventricular (RV, LV) fibrosis in addition to proven risk markers. Results The primary endpoint was all-cause mortality. Of the 550 patients (median age 32 years, 56% male), 27 died (mean follow-up 6.4 ± 5.8; total 3,512 years). Mortality was independently predicted by RVLGE extent, presence of LVLGE, RV ejection fraction ≤47%, LV ejection fraction ≤55%, B-type natriuretic peptide ≥127 ng/L, peak exercise oxygen uptake (V02) ≤17 mL/kg/min, prior sustained atrial arrhythmia, and age ≥50 years. The weighted scores for each of the preceding independent predictors differentiated a high-risk subgroup of patients with a 4.4%, annual risk of mortality (area under the curve [AUC]: 0.87; P < 0.001). The secondary endpoint (VA), a composite of life-threatening sustained ventricular tachycardia/resuscitated ventricular fibrillation/sudden cardiac death occurred in 29. Weighted scores that included several predictors of mortality and RV outflow tract akinetic length ≥55 mm and RV systolic pressure ≥47 mm Hg identified high-risk patients with a 3.7% annual risk of VA (AUC: 0.79; P < 0.001) RVLGE was heavily weighted in both risk scores caused by its strong relative prognostic value. Conclusions We present a score integrating multiple appropriately weighted risk factors to identify the subgroup of patients with rTOF who are at high annual risk of death who may benefit from targeted therapy.

Research paper thumbnail of Reference Values for Ventricular Volumes and Pulmonary Artery Dimensions in Pediatric Patients with Transposition of the Great Arteries After Arterial Switch Operation

Journal of Magnetic Resonance Imaging, 2021

Background: Pulmonary artery (PA) anatomy in patients with transposition of the great arteries (T... more Background: Pulmonary artery (PA) anatomy in patients with transposition of the great arteries (TGA) after arterial switch operation (ASO) with Lecompte manoeuvre is different compared to healthy subjects, and stenoses of the PA are common. Magnetic resonance imaging (MRI) is an excellent imaging modality to assess PA anatomy in TGA patients. However, disease-specific reference values for PA size are scarce. Purpose: To establish disease-specific reference ranges for PA dimensions and for biventricular volumes and mass. Study Type: Retrospective. Subjects: A total of 69 pediatric patients with TGA after ASO (median age 12.6 years; range 5-17.8 years; 13 females and 56 males). Field Strength/Sequence: 3.0 T, steady-state free precession (SSFP) and gradient echo cine sequences and fourdimensional time-resolved magnetic resonance angiography with keyhole. Assessment: Right and left PA (RPA, LPA) were each measured at three locations during its course around the aorta. Ventricular volumes, mass, and ejection fraction were measured from a stack of short axis cine images. Statistical Tests: The lambda-mu-sigma (LMS) method of Cole and Green, univariate and multivariate linear models, and t-test. Results: Centile graphs and tables for PA dimensions, biventricular volumes, mass, and ejection fraction were created. Univariate linear analysis showed significant associations (P < 0.05) between body surface area (BSA), height, and weight with systolic MPA and RPA diameter. In multivariate linear analysis, only BSA remained a strong predictor for main PA and RPA diameters. For biventricular volumes, the univariate linear model revealed a strong influence of BSA, height, weight, and age (all P < 0.05). On multivariate linear analysis, only body height remained associated. Data Conclusion: Uni-and multivariate linear analyses showed a strong association between BSA and PA diameters, as well as between height and biventricular volumes, and therefore, centile tables and graphs are presented accordingly. Our data may improve MR image interpretation and may serve as a reference in future studies. Level of Evidence: 4 Technical Efficacy Stage: 2

Research paper thumbnail of Cardiovascular magnetic resonance normal values in children for biventricular wall thickness and mass

Journal of Cardiovascular Magnetic Resonance, 2021

Background Pediatric patients are becoming increasingly referred for cardiovascular magnetic reso... more Background Pediatric patients are becoming increasingly referred for cardiovascular magnetic resonance (CMR). Measurement of ventricular wall thickness is typically part of the assessment and can be of diagnostic importance, e.g. in arterial hypertension. However, normal values for left ventricular (LV) and right ventricular (RV) wall thickness in pediatric patients are lacking. The aim of this study was to establish pediatric centile charts for segmental LV and RV myocardial thickness in a retrospective multicenter CMR study. Methods CMR was performed in 161 healthy children and adolescents with an age range between 6 and 18 years from two centers in the UK and Germany as well as from a previously published CMR project of the German Competence Network for Congenital Heart Defects. LV myocardial thickness of 16 segments was measured on the short axis stack using the American Heart Association segmentation model. In addition, the thickness of the RV inferior and anterior free wall as...

Research paper thumbnail of 328Pulmonary valve replacement in England: trends and outcome over the past two decades

European Heart Journal, Aug 1, 2017

Long-term management after Tetralogy of Fallot repair / After pulmonary embolism: optimising trea... more Long-term management after Tetralogy of Fallot repair / After pulmonary embolism: optimising treatment and follow up p=0.07) and in those with documented vegetations on echocardiography (75% vs. 44%; p=0.09). On multivariate regression analysis, non-streptococcal IE was an independent predictor of death/re-intervention (HR, 12.0 [95% CI: 1.9-76.4]; p=0.009). Rate and time-to-onset of IE after TPVI Conclusions: The risk of IE after Melody valve implantation, albeit considerable, is lower than previously reported. Surgical/percutaneous re-intervention is a common consequence of IE, although many patients can be managed medically.

Research paper thumbnail of Coarctation of the Aorta

Updates in Hypertension and Cardiovascular Protection, 2016

Coarctation of the aorta (CoA) is a congenital malformation of the aorta, first described by Morg... more Coarctation of the aorta (CoA) is a congenital malformation of the aorta, first described by Morgagni in 1760 [1]. The overall incidence of CoA is 5–8 % of all congenital heart disease, and the condition is 1.7 times more frequent in white males than white females [2]. Aortic coarctation can present as a localised discrete stenosis of the aortic isthmus, but segmental tubular hypoplasia of the aortic arch is also commonly associated. The usual site of CoA is distal to the left subclavian artery.

Research paper thumbnail of MODERATED POSTER SESSION: Deformation and multimodality imaging in congenital heart: Thursday 4 December 2014, 08:30-18:00 * Location: Moderated Poster area

European Heart Journal - Cardiovascular Imaging, 2014

Aim: There is wealth of data on the echocardiographic evaluation of prosthetic valves in the aort... more Aim: There is wealth of data on the echocardiographic evaluation of prosthetic valves in the aortic or mitral position. Similar data are missing for the Melody percutaneous pulmonary valve system (MPPV). We aimed to correlate the anatomic appearance of the MPPV with the haemodynamic result on invasive and echocardiographic assessment. Patients and Methods: All 45 patients who underwent MPPV implantation at our institution between 2007-2013 were studied (median age 30 [range 11-61] years). All patients had complex congenital heart disease. The narrowest dimensions of the MPPV system were taken from the final biplane orthogonal fluoroscopic images to calculate the effective valve opening area (EOA). The post implantation invasive peak-to-peak gradient (DPp-p) and the maximal and mean Doppler gradient (DPmax and DPmean) on the pre-discharge echocardiogram (within 72 hours of implantation) were also obtained. Results: DPp-p after valve implantation was low (11.5+5.2 [range 2-20] mmHg) and there was no significant residual pulmonary regurgitation. The peak and mean gradient across the valve by Doppler assessment were significantly higher than DPp-p (DPmean: 19.3+6.5, DPmax: 33.3+8.2 mmHg; P,0.0001 for both vs. DPp-p). Both Doppler gradients correlated significantly with the invasive peak gradient (DPp-p vs. DPmean: r=0.37, P=0.03; DPp-p vs. DPmax: r=0.39, P=0.02) The EOA of the valve system indexed to body surface area was 132+30 mm2/m2. There were only weak relationships between the indexed EOA and the invasive and Doppler gradients (correlations with EOA: DPp-p: r=-0.32, P=0.06; DPpeak: r=-0.33, P=0.045; DPmean: r=-0.23; P=0.16). Conclusion: Doppler assessment of the percutaneous Melody valve early after implantation consistently overestimates the invasive peak-to-peak gradient. Simple estimates of valve size do not allow prediction of central haemodynamics. These results reflect the complex and variable anatomy of the right ventricular outflow tract/ main pulmonary artery in this group of patients.

Research paper thumbnail of Heart Rate Response During Exercise Predicts Survival in Adults With Congenital Heart Disease

Journal of the American College of Cardiology, 2006

OBJECTIVES To assess the prognostic value of heart rate response to exercise in adult congenital ... more OBJECTIVES To assess the prognostic value of heart rate response to exercise in adult congenital heart disease (ACHD) patients. BACKGROUND An abnormal heart rate response to exercise is related to autonomic dysfunction and may have prognostic implications in ACHD. METHODS We identified 727 consecutive ACHD patients (mean age [Ϯ SD] 33 Ϯ 13 years) with varying diagnoses and without pacemakers. Peak oxygen consumption (peak VO 2), resting heart rate, and the increase in heart rate from resting level to peak exercise ("heart rate reserve") were measured. We also quantified the decrease in heart rate ("heart rate recovery") after cessation of exercise. RESULTS During a median follow-up of 28 months, 38 patients died. Lower values of heart rate reserve, peak heart rate, heart rate recovery, and peak VO 2 (p Ͻ 0.01 for each) were associated with increased mortality in univariate analysis. Furthermore, heart rate reserve predicted mortality independently of antiarrhythmic therapy, functional class, and peak VO 2. Stratifying patients by diagnostic groups revealed that a lower heart rate reserve was also associated with a greater risk of death in patients with complex anatomy, Fontan circulation, and tetralogy of Fallot (p Ͻ 0.05 for each). CONCLUSIONS An abnormal heart rate response to exercise identifies ACHD patients with a higher risk of mortality in the midterm, even after accounting for antiarrhythmic medication and exercise capacity. Heart rate reserve is a simple and inexpensive way to identify ACHD patients at higher mortality risk.

Research paper thumbnail of Impaired heart rate response to exercise in adult patients with a systemic right ventricle or univentricular circulation: Prevalence, relation to exercise, and potential therapeutic implications

International Journal of Cardiology, 2009

Introduction: Exercise limitation is common in patients with a systemic right ventricle or univen... more Introduction: Exercise limitation is common in patients with a systemic right ventricle or univentricular circulation and may be related to chronotropic incompetence (CI). We aimed to investigate the association of CI with exercise capacity, and evaluate whether CI is causally related to exercise intolerance. Patients and methods: Cardiopulmonary exercise tests were performed in patients with a systemic right ventricle (n = 32) or univentricular circulation (n = 32). CI was defined as the inability to achieve 80% of age predicted maximal heart rate reserve ([peak heart rate-resting heart rate]/[220 − age − resting heart rate]). The linearity of the relation between oxygen consumption (VO 2) and heart rate (oxygen pulse) was assessed visually and separately quantified by calculating the quadratic regression coefficient (describing departure from linearity). Results: The prevalence of CI was 59% and 84% in patients with a systemic right ventricle or univentricular circulation, respectively. Patients with CI had a lower peak VO 2 (19.8 ± 5.5 vs. 24.6 ± 6.8 ml/kg/min, P = 0.005), and shorter exercise duration (587 ± 165 vs. 749 ± 176 s, P = 0.001) than those without CI. Oxygen pulse kinetics suggested exercise limitation due to CI in 8 of 43 patients (19%). In contrast, 13 of 43 patients (30%) had oxygen pulse kinetics suggestive of exercise limitation due to ventricular dysfunction or cyanosis. Discussion: Chronotropic incompetence is common and associated with exercise limitation in patients with a systemic right ventricle or univentricular circulation. Visual or mathematical assessment of oxygen pulse kinetics identifies patients in whom an attenuated heart-rate response is responsible for poor exercise capacity and may have therapeutic implications.

Research paper thumbnail of Abnormal Ventilatory Response to Exercise in Adults With Congenital Heart Disease Relates to Cyanosis and Predicts Survival

Circulation, 2006

Background— Limited data exist with which to stratify risk in adult congenital heart disease (ACH... more Background— Limited data exist with which to stratify risk in adult congenital heart disease (ACHD). An increased ventilatory response to exercise, expressed as ventilation per unit of carbon dioxide production (V̇ e /V̇ co 2 slope), is an established predictor of impaired survival in acquired heart disease. We sought to establish the distribution, relation to cyanosis, and prognostic value of the V̇ e /V̇ co 2 slope across a wide spectrum of ACHD patients. Methods and Results— Five hundred sixty ACHD patients of varying diagnoses and 50 healthy controls underwent cardiopulmonary exercise testing at a single laboratory between 2001 and 2004. Patient age was 33.2±12.9 years (mean±SD). Peak oxygen consumption was 23.5±9.0 mL · kg −1 · min −1 . V̇ e /V̇ co 2 slope for all patients was 36.3±15.3. The slope was raised in all ACHD groups compared with controls and was 73% higher in cyanotic patients. Cyanosis, with or without pulmonary arterial hypertension, was the strongest predictor of...

Research paper thumbnail of P733Echocardiographic predictors of outcome in repaired Tetralogy of Fallot patients over 40 years old

European Heart Journal, 2017

Understanding the pulmonary circulation / Imaging arrhythmias in congenital heart disease 149 was... more Understanding the pulmonary circulation / Imaging arrhythmias in congenital heart disease 149 was significantly correlated with RVsm (r:-0.36, p:0.005). RA, RA/PCWP and PAPi did not reveal any correlation. Subgroup analysis of patients with atrial fibrillation (n=30) did not reveal any of the mentioned correlations of new hemodynamic parameters Conclusions: PAE, PAC, RSWI and PAPP are applicative new hemodynamic parameters to reveal RV dysfunction in patients with PH. However, presence of atrial fibrillation leads to the loss of associations between these parameters and conventional prognostic markers

Research paper thumbnail of Tissue Engineered Transcatheter Pulmonary Valved Stent Implantation: Current State and Future Prospect

International Journal of Molecular Sciences, 2022

Patients with the complex congenital heart disease (CHD) are usually associated with right ventri... more Patients with the complex congenital heart disease (CHD) are usually associated with right ventricular outflow tract dysfunction and typically require multiple surgical interventions during their lives to relieve the right ventricular outflow tract abnormality. Transcatheter pulmonary valve replacement was used as a non-surgical, less invasive alternative treatment for right ventricular outflow tract dysfunction and has been rapidly developing over the past years. Despite the current favorable results of transcatheter pulmonary valve replacement, many patients eligible for pulmonary valve replacement are still not candidates for transcatheter pulmonary valve replacement. Therefore, one of the significant future challenges is to expand transcatheter pulmonary valve replacement to a broader patient population. This review describes the limitations and problems of existing techniques and focuses on decellularized tissue engineering for pulmonary valve stenting.

Research paper thumbnail of Analytical Identification of Ideal Pulmonary-Systemic Flow Balance in Patients With Bidirectional Cavopulmonary Shunt and Univentricular Circulation: Oxygen Delivery or Tissue Oxygenation?

Circulation, 2006

Background— In the present study, we extended previous mathematical modeling work on patients wit... more Background— In the present study, we extended previous mathematical modeling work on patients with bidirectional cavopulmonary (“bidirectional Glenn”) anastomosis to assess the potential utility of several descriptors of oxygen status. We set out to determine which of these descriptors best represents the overall tissue oxygenation. We also introduce a new descriptor, S o 2 min, defined as the lower of the superior and inferior vena cava oxygen saturations. Methods and Results— The application of differential calculus to a model of oxygen physiology of patients with bidirectional Glenn allowed simultaneous assessment of all possible distributions of blood flow and metabolic rate between upper and lower body, across all cardiac outputs, total metabolic rates, and oxygen-carrying capacities. When total cardiac output is fixed, although it may intuitively seem best to distribute flow to maximize oxygen delivery (total, upper body, or lower body), we found that for each variable, there ...

Research paper thumbnail of Interventional creation of an atrial septal defect and its impact on right ventricular function: an animal study with the pressure-volume conductance system

Cardiology journal, 2011

BACKGROUND The aim of our study was to assess the suitability of different interventional techniq... more BACKGROUND The aim of our study was to assess the suitability of different interventional techniques to create an atrial septal defect (ASD) and to evaluate the short-term effects of right ventricular (RV) volume overload on RV contractility in the growing swine. METHODS Thirteen ASD and six control animals were studied. An ASD was created by balloon dilatation (BD) of the fossa ovalis (n = 4) or by implantation of a multi-perforated Amplatzer Septal Occluder (n = 4) or a patch-less nitinol device (n = 5). After 4.8 (3.9-6.0) weeks, the amounts of left-to-right shunting (Qp/Qs) and RV contractility (end systolic elastance - Ees) were assessed. RESULTS In the ASD group, a significant left-to-right shunt could be documented (Qp/Qs 1.5 ± ± 0.4). However, a shunt was absent in the BD subgroup (Qp/Qs 1.1 ± 0.1). In animals with devices implanted, a significant relationship between the post-mortem ASD area and Qp/Qs was found (r = 0.68, p < 0.05). Compared to controls, RV contractility...

Research paper thumbnail of Catheter ablation for patients with end-stage complex congenital heart disease or cardiomyopathy considered for transplantation: Trials and tribulations

International Journal of Cardiology

INTRODUCTION Arrhythmia contributes significantly to morbidity and mortality of patients with con... more INTRODUCTION Arrhythmia contributes significantly to morbidity and mortality of patients with congenital heart disease (CHD) or cardiomyopathy (CMP). It also has the potential to worsen symptoms and is particularly detrimental to patients with advanced heart failure awaiting cardiac transplantation. We report our experience using catheter ablation to treat recurrent arrhythmia in patients with CHD or CMP considered for transplantation. METHODS Five consecutive patients (3 female, mean age 47.8 ± 12.8 years) with complex CHD or CMP (tricuspid atresia, mitral atresia, double inlet left ventricle, arrhythmogenic right ventricular cardiomyopathy, left ventricular non-compaction) presented with either atrial (n = 3) or ventricular (n = 2) arrhythmias. All ablations were guided by three-dimensional (3D) electro-anatomical mapping, plus remote magnetic navigation in 3 patients. RESULTS Patients underwent a median of 2 ablation procedures for a total number of 26 tachycardias. None of the 5 patients experienced further arrhythmia at a median of 939 days (range 4-1375) from their last ablation. During a median follow up of 31 months (range 1-70), three patients underwent successful transplantation at 1375, 1062 and 321 days following their last ablation. One patient with a Fontan circulation died from hepatic cancer and one from end-stage heart failure despite urgent transplant listing. CONCLUSIONS Catheter ablation is feasible in complex cardiac patients considered for heart transplantation and should be offered for rhythm management and patient optimization until a suitable donor is found.

Research paper thumbnail of Surgical and percutaneous pulmonary valve replacement in England over the past two decades

Heart

ObjectivePulmonary valve replacement (PVR) is often required in patients with congenital heart di... more ObjectivePulmonary valve replacement (PVR) is often required in patients with congenital heart disease. We aimed to describe temporal trends in PVR in a nationwide English cohort between 1997 and 2014, survival and the need for re-PVR.MethodsPatients were identified in the Hospital Episode Statistics Database. Survival data were retrieved from the UK Office for National Statistics.ResultsA total of 2733 patients underwent PVR (2845 procedures) over the study period. Median age at first procedure increased from 20.1 years in 1997–2005 to 24.7 years in 2006–2014. The annual number of PVRs increased from 23 in 1997 to 251 in 2014. Homografts were the most common choice in the early years, but the use of xenografts increased after 2005. During a median follow-up of 5.8 years, 176 patients died and 108 required redo PVR. Early (30 day) survival was 98% for all PVRs and was similar for all types of prostheses but longer-term mortality dropped to 92% at 10 years and 90% at 15 years. Age &g...

Research paper thumbnail of The outcome of adults born with pulmonary atresia: High morbidity and mortality irrespective of repair

International Journal of Cardiology

To describe the characteristics and long-term outcome of a large adult cohort with pulmonary atre... more To describe the characteristics and long-term outcome of a large adult cohort with pulmonary atresia. Patients with pulmonary atresia (PA) are a heterogeneous population in terms of anatomy, physiology and surgical history, and their management during adulthood remains challenging. Data on all patients with PA followed in our center between January 2000 and March 2015 were recorded. Patients were classified into the following groups: PA with ventricular septal defect (PA-VSD, 1), PA with intact ventricular septum (PA-IVS, 2) and other miscellaneous PA (PA-other, 3). Two-hundred twenty-seven patients with PA were identified, 66.1% female, mean age 25.5 ± 8.7 years. Over a median follow-up of 8.8 years, 49 (21.6%) patients had died: heart failure (n = 21, 42.8%) and sudden cardiac death (n = 8, 16.3%) were the main causes. There was no significant difference in mortality between the 3 Groups (p = 0.12) or between repaired and unrepaired patients in Group 1 (p = 0.16). Systemic ventricular dysfunction and resting oxygen saturations were the strongest predictors of mortality. Additionally, 116 (51%) patients were hospitalized, driven mainly by the need for invasive procedures, heart failure and arrhythmias. Adult survivors with pulmonary atresia have a high morbidity and mortality irrespective of underlying cardiac anatomy and previous reparative or palliative surgery. We present herewith predictors of outcome in adult life that may assist with their tertiary adult congenital care.

Research paper thumbnail of New York Heart Association (NYHA) classification in adults with congenital heart disease: relation to objective measures of exercise and outcome

European Heart Journal - Quality of Care and Clinical Outcomes

The New York Heart Association functional classification (NYHA class) is often used to describe t... more The New York Heart Association functional classification (NYHA class) is often used to describe the functional capacity of adults with congenital heart disease (ACHD), albeit with limited evidence on its validity in this heterogeneous population. We aimed to validate the NYHA functional classification in ACHD by examining its relation to objective measures of limitation using cardiopulmonary exercise testing (CPET) and mortality.

Research paper thumbnail of Preconception couselling, predicting risk and outcomes in women with mwho 3 and 4 heart disease

International Journal of Cardiology, 2017

All women with CHD, especially those with more severe disease, should be offered preconception co... more All women with CHD, especially those with more severe disease, should be offered preconception counseling (PCC), to discuss the risk of complications and to plan a future pregnancy. Several scoring system have been devised to estimate the risk of adverse events in pregnancies complicated by maternal heart disease (HD) and while comparisons have been made across the whole population, none have focused on the high-risk population. Retrospective cohort study that included women classed as modified WHO (mWHO) 3 and 4 who had a pregnancy from at least 20weeks gestation between 1994 and 2015 managed within our institution. We assessed how well the quoted risk (at PCC) of an adverse event (maternal or fetal) related to the actual rate of occurrence. We calculated NYHA and CARPREG scores for all patients, and the clinician assessment of percentage risk, to predict the occurrence of an adverse outcome. We identified 76 mWHO 3 and 4 women who had a total of 102 pregnancies. However, only in 63 pregnancies had the woman attended PCC. Both maternal and fetal adverse events were common. NYHA did not significantly predict any adverse events, whilst a CARPREG score of &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;gt;3 score predicted heart failure and mWHO4 score predicted maternal death. However, the best prediction of adverse outcomes was a composite quoted risk (percent) given at PCC. Women must have access to PCC as those with worse CARPREG and mWHO scores encounter greater adverse events.