Publications by authors named "Aleksander Kempny"

102 Publications

Predicting Survival in Repaired Tetralogy of Fallot: A Lesion-Specific and Personalized Approach.

JACC Cardiovasc Imaging 2021 Oct 7. Epub 2021 Oct 7.

Royal Brompton and Harefield NHS Trust, London, United Kingdom; National Heart Lung Institute, Imperial College London, United Kingdom. Electronic address:

Objectives: We 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 (V0) ≤17 mL/kg per minute, 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.
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http://dx.doi.org/10.1016/j.jcmg.2021.07.026DOI Listing
October 2021

Closing atrial septal defects for prevention of stroke: how small is too small?

Heart 2021 12 11;107(23):1850-1851. Epub 2021 Sep 11.

National Heart and Lung Institute, Imperial College London, London, UK.

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http://dx.doi.org/10.1136/heartjnl-2021-320064DOI Listing
December 2021

The clinical presentation and outcome of aortic coarctation associated with left ventricular inflow and outflow tract lesion in adult patients: Shone syndrome and beyond.

Int J Cardiol 2021 Nov 26;343:45-49. Epub 2021 Aug 26.

Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College London, UK.

Background: Aortic coarctation (AoCoa) is a congenital stenosis of aorta, which often co-exists with other congenital heart defects. Many studies have shown the importance of regular follow-up in these patients however there is scarcity of knowledge relating to the impact of left ventricle inflow lesions (LVIT) and left ventricle outflow track lesions (LVOT). The aim of this study is to evaluate the impact of isolated AoCoa with LVIT and/or LVOT on haemodynamic, morbidity and mortality.

Methods: We have retrospectively analysed clinical data of all adult AoCoa patients who underwent echocardiography between 2010-2018 in our centre. Outcome measures included death, number of hospitalisations for cardiac causes, development of cardiac arrhythmia, new prescription of HF medication.

Results: A total of 406 AoCoa patients were included and were followed for a median 4.2 years. At baseline, 38% patients had AoCoa alone, 54% patients had LVOT, 3% patients had LVIT, and 5% patients had mixed LVIT and LVOT, including patients with Shone syndrome. Patients with mixed LVIT and LVOT had the highest mortality of the four groups and the highest heart failure-related morbidity. Moreover, they were the most prone to have a higher indexed LA volume compared to patients with no LVOT (p=0.0001). During follow-up, 13 patients died, of which 21% patients were from the mixed LVIT and LVOT group.

Conclusions: AoCoa patients with a combination of LVIT and LVOT including Shone complex are associated with a significantly higher morbidity and mortality compared to AoCoa alone.
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http://dx.doi.org/10.1016/j.ijcard.2021.08.017DOI Listing
November 2021

Impact of cyanosis on ventilatory responses during stair climb exercise in Eisenmenger syndrome and idiopathic pulmonary arterial hypertension.

Int J Cardiol 2021 Oct 18;341:84-87. Epub 2021 Aug 18.

Department of Cardiology, Royal Brompton Hospital, London, United Kingdom; National Heart and Lung Institute, Imperial College, London. Electronic address:

Studies assessing exercise ventilatory responses during real-life exercise in pulmonary arterial hypertension (PAH) which include patients with cyanotic congenital heart disease are scarce. We assessed the ventilatory response to stairclimbing in patients with idiopathic PAH (IPAH) and congenital heart disease-associated PAH with Eisenmenger (EIS) physiology compared to healthy controls. Fifteen adults with IPAH, six EIS and 15 age and body mass index (BMI) matched controls were prospectively recruited. Participants completed spirometry and a self-paced stair-climb (48 steps) with portable cardiopulmonary exercise testing (CPET) equipment in-situ. Borg dyspnoea scores were measured at rest and on stair-climb cessation. Both IPAH and EIS groups had amplified ventilatory responses compared to Controls. The rate of increase in minute ventilation (VE) was exaggerated in EIS driven by an early increase in tidal volume (Tv) and more gradual increase in respiratory rate (RR). Peak Tv, RR, Tv: forced vital capacity (FVC) ratio, VE/VCO slope and stairclimb duration were significantly higher in EIS and IPAH compared to controls despite similar baseline spirometry and change in oxygen uptake on exercise. A decline in end-tidal carbon dioxide (CO) and arterial oxygen saturations in early exercise distinguished EIS and IPAH patients. Significant correlations were observed between peak exercise Borg score and stair-climb time (r = 0.73, p = 0.002), peak end-tidal CO (r = -0.73, p = 0.001), peak VE (r = 0.53, p = 0.008), peak RR (r = 0.42, p = 0.011) and VE/VCO slope (r = 0.54, p = 0.001). Patients with IPAH and EIS have exaggerated ventilatory responses to stair-climbing compared to the controls with more severe levels of dyspnoea perception in Eisenmenger syndrome for equivalent oxygen uptake and work.
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http://dx.doi.org/10.1016/j.ijcard.2021.08.020DOI Listing
October 2021

Transcatheter Pulmonary Valve Prosthesis and Pregnancy: Stable Hemodynamics and No Valve-Related Adverse Events.

JACC Case Rep 2020 Jun 17;2(6):852-854. Epub 2020 Jun 17.

Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, United Kingdom.

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http://dx.doi.org/10.1016/j.jaccas.2020.05.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8302011PMC
June 2020

Enhanced Assessment of Perioperative Mortality Risk in Adults With Congenital Heart Disease.

J Am Coll Cardiol 2021 07;78(3):234-242

Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom; National Heart and Lung Institute, Imperial College London, London, United Kingdom. Electronic address:

Background: In-hospital mortality is a rare, yet feared complication following cardiac surgery in adult congenital heart disease (ACHD). A risk score, developed and validated in ACHD, can be helpful to optimize risk assessment.

Objectives: The purpose of this study was to assess the performance of EuroSCORE II components and procedure-related Adult Congenital Heart Surgery (ACHS) score, identify additional risk factors, and develop a novel risk score for predicting in-hospital mortality after ACHD surgery.

Methods: We assessed perioperative survival in patients aged >16 years undergoing congenital heart surgery in a large tertiary center between 2003 and 2019. A risk variable-derived PEACH (PErioperative ACHd) score was calculated for each patient. Internal and external validation of the model was undertaken, including testing in a validation cohort of patients operated in a second European ACHD center.

Results: The development cohort comprised 1,782 procedures performed during the study period. Re-sternotomy was undertaken in 897 (50.3%). There were 31 (1.7%) in-hospital deaths. The PEACH score showed excellent discrimination ability (area under the curve [AUC]: 0.88; 95% CI: 0.83-0.94), and performed better than the ACHS score in our population (ACHS AUC: 0.69; 95% CI: 0.6-0.78; P = 0.0003). A simple 3-tiered risk stratification was formed: PEACH score 0 (in-hospital mortality 0.2%), 1-2 (3.6%), and ≥3 (17.2%). In a validation cohort of 975 procedures, the PEACH score retained its discriminative ability (AUC: 0.75; 95% CI: 0.72-0.77) and was well calibrated (Hosmer-Lemeshow chi-square goodness-of-fit P = 0.55). There was agreement in expected and observed perioperative mortality between cohorts.

Conclusions: The PEACH score is a simple, novel perioperative risk score developed and validated specifically for ACHD patients undergoing cardiac surgery.
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http://dx.doi.org/10.1016/j.jacc.2021.04.096DOI Listing
July 2021

Effect of medical treatment on heart failure incidence in patients with a systemic right ventricle.

Heart 2021 09 6;107(17):1384-1389. Epub 2021 May 6.

Adult Congenital Heart Centre, National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK.

Background: To date, clinical trials have been underpowered to demonstrate a benefit from ACE inhibitors (ACEis) or angiotensin II receptor blockers (ARBs) in preventing systemic right ventricle (sRV) failure and disease progression in patients with transposition of the great arteries (TGA). This observational study aimed to estimate the effect of ACEi and ARB on heart failure (HF) incidence and mortality in a large population of patients with an sRV.

Methods: Data on all patients with an sRV under active follow-up at two tertiary centres between January 2007 and September 2018 were studied. The effect of ACEi and ARB on the incidence of HF and mortality was estimated using a propensity score weighting approach to control confounding.

Results: Among the 359 patients with an sRV (32.2 (IQR 26.4-38.3) years, 59.3% male, 66% complete TGA with atrial switch repair and 34% congenitally corrected TGA), 79 (22%) had a moderate to severe sRV dysfunction and 138 (38%) were treated with ACEi or ARB. Fourteen (3.6%) patients died, 8 (2.1%) underwent heart transplantation and 46 (11.8%) had a new HF event over a median follow-up of 7.1 (IQR 4.0-9.4) years. On multivariate Cox analysis with adjustment using propensity score weighting approaches, ACEi or ARBs treatment was not significantly associated with a lower HF incidence or mortality in patients with an sRV.

Conclusions: Despite significant neurohormonal activation described in patients with an sRV, there is still no evidence of a beneficial effect of ACEi or ARB on morbidity and mortality in this population.
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http://dx.doi.org/10.1136/heartjnl-2020-318787DOI Listing
September 2021

Perioperative management of patients with pulmonary hypertension undergoing non-cardiothoracic, non-obstetric surgery: a systematic review and expert consensus statement.

Br J Anaesth 2021 04 19;126(4):774-790. Epub 2021 Feb 19.

Adult Intensive Care Unit, Royal Brompton Hospital, London, UK.

Background: The risk of complications, including death, is substantially increased in patients with pulmonary hypertension (PH) undergoing anaesthesia for surgical procedures, especially in those with pulmonary arterial hypertension (PAH) and chronic thromboembolic PH (CTEPH). Sedation also poses a risk to patients with PH. Physiological changes including tachycardia, hypotension, fluid shifts, and an increase in pulmonary vascular resistance (PH crisis) can precipitate acute right ventricular decompensation and death.

Methods: A systematic literature review was performed of studies in patients with PH undergoing non-cardiac and non-obstetric surgery. The management of patients with PH requiring sedation for endoscopy was also reviewed. Using a framework of relevant clinical questions, we review the available evidence guiding operative risk, risk assessment, preoperative optimisation, and perioperative management, and identifying areas for future research.

Results: Reported 30 day mortality after non-cardiac and non-obstetric surgery ranges between 2% and 18% in patients with PH undergoing elective procedures, and increases to 15-50% for emergency surgery, with complications and death usually relating to acute right ventricular failure. Risk factors for mortality include procedure-specific and patient-related factors, especially markers of PH severity (e.g. pulmonary haemodynamics, poor exercise performance, and right ventricular dysfunction). Most studies highlight the importance of individualised preoperative risk assessment and optimisation and advanced perioperative planning.

Conclusions: With an increasing number of patients requiring surgery in specialist and non-specialist PH centres, a systematic, evidence-based, multidisciplinary approach is required to minimise complications. Adequate risk stratification and a tailored-individualised perioperative plan is paramount.
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http://dx.doi.org/10.1016/j.bja.2021.01.005DOI Listing
April 2021

Neurological complications in aortic coarctation: Results of a Nationwide analysis based on 11,907 patients.

Int J Cardiol 2021 01 14;322:114-120. Epub 2020 Aug 14.

Adult Congenital Heart Disease Programme, Royal Brompton Hospital, London, UK; National Heart and Lung Institute, Imperial College London, UK.

Background: Patients with coarctation of the aorta (CoA) are at increased risk of periprocedural and late neurological complications. Based on a nationwide inpatient dataset, we assessed the prevalence and risk factors for neurological complications in this cohort.

Method And Results: We included all CoA patients hospitalized in England between 1997 and 2015. The risk for procedural complications, subarachnoid bleeding and ischemic stroke were quantified, and risk factors investigated. Overall, 11,907 patients (60% male) with CoA were identified. The median age at first presentation was 0.6 years with a median follow-up period of 14.4 years. Throughout the study period, 8456 surgical or interventional procedures were performed in 6796 patients. The neurological complication rate for primary surgical or interventional repair in patients born after 1997 was 0.05% and 0.2%, respectively. During a total follow-up period of 146,295 patient-years, late neurological complications such as subarachnoid bleeding and cerebral infarction occurred in 225 patients (0.15%/patient-year). The median age for subarachnoid bleeding was 29 years and 57 years for ischemic stroke. While, arterial hypertension (p = .0014), current smoking (p = .02), dyslipidaemia (p = .007) and diabetes (p = .037) were significantly related to the risk of ischemic stroke, only arterial hypertension emerged as significant risk factor for subarachnoid bleeding.

Conclusions: Neurological complication during primary CoA repair are extremely rare in the current era. In contrast, many CoA patients are afflicted by late complications such as subarachnoid bleeding and ischemic stroke at a relatively young age, emphasizing the need for meticulous and pro-active blood pressure control and reduction of cardiovascular risk factors.
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http://dx.doi.org/10.1016/j.ijcard.2020.08.041DOI Listing
January 2021

Heart transplantation at a single tertiary adult congenital heart disease centre: Too little, too late?

Int J Cardiol 2021 01 14;322:107-113. Epub 2020 Aug 14.

Royal Brompton & Harefield NHS Trust. London, UK.

Background: Heart failure (HF) is the leading cause of death in adults with congenital heart disease (ACHD). Heart transplantation can be an effective therapy for them, albeit unfavourable anatomy, end-organ damage, pulmonary vascular disease, HLA sensitization and lack of robust selection criteria currently limit its application.

Methods: Consecutive CHD patients considered for heart or combined heart and lung transplantation at our tertiary ACHD centre between 2000 and 2018 constitute our study population. Baseline characteristics and outcome, including transplantation and death, were obtained for all patients from designated databases, medical records and the UK Office for National Statistics.

Results: From a total of more than 9000 active ACHD patients under follow-up, 166 (median age 40.4 years) fulfilled inclusion criteria, with a broad spectrum of underlying diagnosis: univentricular heart 22.3%, systemic right ventricle 22.3%, systemic-to-pulmonary shunts and Eisenmenger syndrome 16.3%, left sided valvular lesions 14.5%, tetralogy of Fallot 12.7%, CHD associated with cardiomyopathy 4.8% and other 7.2%. There was a high overall mortality with 39.2% of patients dying over a median follow-up of 2.7 years. A minority of patients (22.9%) were eventually listed and only 13.3% from the 166 patients were actually transplanted. Survival at 1 year after transplantation was 81.8% and remained high long-term (65.5% at 15 years). We describe herewith in detail characteristics and outcome of our cohort and present the transplantation pathway followed.

Conclusion: Of the small number of patients with ACHD considered for heart transplantation at a large tertiary centre, there was high overall mortality, with only a fraction of patients being actually transplanted. Patients who received transplantation, however, had a good outcome. Better patient selection and timing are clearly warranted so that more ACHD patients are considered and potentially benefit from this effective form of therapy.
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http://dx.doi.org/10.1016/j.ijcard.2020.08.047DOI Listing
January 2021

Initial Experience Using the Radiofrequency Needle Visualization on the Electroanatomical Mapping System for Transseptal Puncture.

Cardiol Res Pract 2020 20;2020:5420909. Epub 2020 Jun 20.

Department of Cardiology, Royal Brompton and Harefield Hospital and Imperial College London, London, UK.

Introduction: Transseptal puncture (TSP) is a routine access route in patients with left-sided ablation substrates and is performed safely on fluoroscopy (+/- echocardiographic guidance). We report on our experience using a radiofrequency (RF) needle in an unselected group of patients to demonstrate safety and usefulness of direct tip visualization on the 3D electroanatomical mapping (EAM) system with specific emphasis on total radiation exposure.

Methods And Results: We retrospectively reviewed 42 consecutive left-sided ablation procedures with TSP performed using an RF needle guided by fluoroscopy and/or EAM visualization by a single operator. The procedures included atrial fibrillation ( = 33), atrial tachycardia ( = 8), and ventricular tachycardia ( = 1) ablations. Fourteen of 41 patients had congenital heart disease, including 9 patients with previous septal closure. Twenty-two patients had at least one previous TSP. All TSPs were performed successfully and without complications. The overall median fluoroscopy time amounted to 3.2 min and median exposure of 199.5 Gy m. In a subgroup of patients ( = 27), the RF needle was visualized on the EAM system: median radiation time was 0.88 (interquartile range: 0-3.4) min and median exposure 33.5 [0-324.8] Gy m.

Conclusions: TSP using an RF needle is an effective technique, also in congenital patients with artificial patch material and in normal patients with multiple previous TSPs. Moreover, the RF needle tip visualization on EAM allows a low (or even zero) fluoroscopy approach.
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http://dx.doi.org/10.1155/2020/5420909DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7322610PMC
June 2020

EmPHasis-10 health-related quality of life score predicts outcomes in patients with idiopathic and connective tissue disease-associated pulmonary arterial hypertension: results from a UK multicentre study.

Eur Respir J 2021 02 25;57(2). Epub 2021 Feb 25.

Pulmonary Vascular Disease Unit, Royal Papworth Hospital, Cambridge, UK.

Health-related quality of life (HRQoL) scores assess symptom burden in pulmonary arterial hypertension (PAH) but data regarding their role in prognostication and risk stratification are limited. We assessed these relationships using the emPHasis-10 HRQoL measure.1745 patients with idiopathic PAH (IPAH), drug-induced PAH (DPAH), heritable PAH (HPAH) (collectively "(I/D/H)PAH"), or connective tissue disease-associated PAH (CTD-PAH), who had completed emPHasis-10 questionnaires at one of six UK referral centres between 2014 and 2017, were identified. Correlations with exercise capacity and World Health Organization (WHO) functional class were assessed, and exploratory risk stratification thresholds were tested.Moderate correlations were seen between emPHasis-10 scores and 6-min walk distance (r=-0.546), incremental shuttle walk distance (r=-0.504) and WHO functional class (r=0.497) (all p<0.0001). Distribution of emPHasis-10 score differed significantly between each WHO functional class (all p<0.0001). On multivariate analysis, emPHasis-10 score, but not WHO functional class, was an independent predictor of mortality. In a risk stratification approach, scores of 0-16, 17-33 and 34-50 identified incident patients with 1-year mortality of 5%, 10% and 23%, respectively. Survival of patients in WHO functional class III could be further stratified using an emPHasis-10 score ≥34 (p<0.01). At follow-up, patients with improved emPHasis-10 scores had improved exercise capacity (p<0.0001) and patients who transitioned between risk groups demonstrated similar survival to patients originally in those risk groups.The emPHasis-10 score is an independent prognostic marker in patients with (I/D/H)PAH or CTD-PAH. It has utility in risk stratification in addition to currently used parameters. Improvement in emPHasis-10 score is associated with improved exercise capacity.
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http://dx.doi.org/10.1183/13993003.00124-2020DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7905834PMC
February 2021

Severe Left Ventricular Outflow Tract Obstruction Immediately After Surgical Repair of Ebstein's Anomaly.

JACC Case Rep 2020 May 20;2(5):725-731. Epub 2020 May 20.

Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, United Kingdom.

A 52-year-old man following surgery for Ebstein's anomaly after repair developed acute hemodynamically significant left ventricular outflow tract obstruction with systolic anterior motion of the mitral valve and severe mitral regurgitation. Fluid resuscitation and weaning of inotropes were unsuccessful. Left ventricular outflow tract obstruction and mitral regurgitation resolved by using esmolol. ().
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http://dx.doi.org/10.1016/j.jaccas.2020.02.026DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8302009PMC
May 2020

Chronic thromboembolic disease following pulmonary embolism: time for a fresh look at old clot.

Eur Respir J 2020 04 3;55(4). Epub 2020 Apr 3.

Dept of Pulmonary Hypertension, Royal Brompton Hospital, London, UK.

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http://dx.doi.org/10.1183/13993003.01934-2019DOI Listing
April 2020

Cardiac catheter intervention complexity and safety outcomes in adult congenital heart disease.

Heart 2020 09 23;106(18):1432-1437. Epub 2020 Mar 23.

Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK.

Objective: To describe the intervention spectrum, complexity, and safety outcomes of catheter-based interventions in a contemporary adult congenital heart disease (ACHD) tertiary cohort.

Methods: All patients over 16 years who underwent a catheter-based intervention for ACHD in our centre between 2000 and 2016 were included. Baseline demographics, clinical characteristics, indications for and complexity of intervention, procedural complications and early and mid-term mortality were analysed.

Results: Overall, 1644 catheter-based interventions were performed. Intervention complexity ranged from simple (67.5%) to intermediate (26.4%) and to high (6.1%). Commonly performed procedures were atrial septal defect (33.4%) and patent foramen ovale closure (26.1%) followed by coarctation of the aorta (11.1%) and pulmonary artery interventions (7.0%). Age at index intervention was 40±16 years, 758 (46.1%) patients were male, 73.2% in New York Heart Association (NYHA) class I, 20.2% in NYHA class II, whereas 6.6% in NYHA class III/IV. In-hospital mortality was 0.7%. Median postinterventional length of stay was 1 day. Complications occurred in 129 (7.9%) with major adverse events in 21 (1.3%). One-year postintervention survival rates were 98.7% (95% CI 98.2 to 99.2). Over the study period, there was a notable shift in intervention complexity, with a predominance of simple procedures performed in early years and more complex procedures in later years. Furthermore, the case mix during the study broadened (p<0.001) with new catheter-based interventions and a more individualised approach to therapy.

Conclusion: This study shows an increasing complexity and expanding variability of ACHD catheter-based interventions, associated with low major complications, short hospital stays and low early and mid-term mortality. Ongoing investment in ACHD catheter interventions is warranted.
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http://dx.doi.org/10.1136/heartjnl-2019-316148DOI Listing
September 2020

Grown-up Congenital Heart Surgery in 1093 Consecutive Cases: A "Hidden" Burden of Early Outcome.

Ann Thorac Surg 2020 11 5;110(5):1667-1676. Epub 2020 Mar 5.

Department of Congenital Cardiac Surgery, Leeds Teaching Hospitals, Leeds, United Kingdom.

Background: Surgery in grown-ups with congenital heart disease (GUCH) is characterized by complex anatomy, comorbidities, reoperations, and technical challenges. Although 30-day postoperative mortality is low, this measure might be insufficient to reflect adverse outcome monitoring. Our study aimed to establish whether prolonged intensive care unit (ICU) stay (≥7 days) and 6-month mortality were more clinically meaningful measures than 30-day mortality and to identify predictors of adverse outcome.

Methods: All consecutive GUCH patients from 1998 to 2015 were identified. Perioperative characteristics, diagnoses, and postoperative data were collected retrospectively. Predictors of 30-day and 6-month mortality and prolonged ICU stay were determined with logistic regression. Era effect was tested for quality assurances by dividing the cohort into 4 time intervals.

Results: Within 17 years, 1093 consecutive cardiac surgical procedures were identified in 1026 GUCH patients. During the study period, 30-day mortality improved significantly, with an overall 30-day mortality of 1.5%; 6-month mortality and prolonged ICU stay were 2.4% and 6.7%, respectively. Despite a decreased number of preoperative patients in New York Heart Association Functional Classification III or higher, prolonged ICU stay increased over the eras. Predictors of adverse outcome were New York Heart Association class III or higher, preoperative renal failure, disease of great complexity, preoperative ventilator support, cardiopulmonary bypass time, and concomitant procedures.

Conclusions: In the current era of low 30-day mortality, extended 6-month mortality and prolonged ICU stay reporting may be more realistic measures of adverse outcomes for counseling GUCH patients at risk.
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http://dx.doi.org/10.1016/j.athoracsur.2020.01.071DOI Listing
November 2020

Right ventricular outflow tract prestenting with AndraStent XXL before percutaneous pulmonary valve implantation.

Arch Cardiovasc Dis 2020 Feb 18;113(2):113-120. Epub 2020 Feb 18.

Pôle des cardiopathies congénitales de l'enfant et de l'adulte, centre de référence malformations cardiaques congénitales complexes (M3C), hôpital Marie-Lannelongue, université Paris-Sud, université Paris-Saclay, 133, avenue de la Résistance, 92350 Le Plessis-Robinson, France.

Background: The indications for percutaneous pulmonary valve implantation (PPVI) have been extended to include large dysfunctional right ventricular outflow tracts (RVOTs). Prestenting of the RVOT is commonly performed before PPVI in order to ensure a stable landing zone. The AndraStent XXL (AndraMed GmbH, Reutlingen, Germany), a cobalt-chromium stent with semi-open cell design, has unique mechanical properties in this indication but is no longer available in France.

Aims: To assess the efficiency of AndraStent XXL before PPVI.

Methods: In this retrospective multicentre cohort study, 86 AndraStents XXL were implanted in 77 patients in 6 centres.

Results: PPVI was indicated mainly for pulmonary regurgitation (75.3%) in native or patched RVOT (88.3%). The stents were manually mounted on balloon catheters and delivered through sheaths using a conventional femoral approach. PPVI was performed successfully in 97.4% of patients after successful prestenting, generally during the same procedure (77.9%). There were no deaths associated with stent implantation, and four patients experienced five complications, mainly stent embolization, including one requiring surgery. Neither stent fracture nor dysfunction were observed in any patient during a mean follow-up of 19.2±8.7months. Stent analysis showed an excellent maximal stent expansion (97.1%) regardless of balloon size. A 22.3%±3.4 stent shortening with a 30mm balloon was observed.

Conclusions: Implantation of large cobalt-chromium AndraStent XXL stents is efficient for prestenting before PPVI.
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http://dx.doi.org/10.1016/j.acvd.2019.12.004DOI Listing
February 2020

Tricuspid regurgitation severity after atrial septal defect closure or pulmonic valve replacement.

Heart 2020 03 23;106(6):455-461. Epub 2019 Aug 23.

Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, United Kingdom.

Objectives: Cardiac surgery or catheter interventions are nowadays commonly performed to reduce volume loading of the right ventricle in adults with congenital heart disease. However, little is known, on the effect of such procedures on pre-existing tricuspid regurgitation (TR). We assessed the potential reduction in the severity of TR after atrial septal defect (ASD) closure and pulmonic valve replacement (PVR).

Methods: Demographics, clinical and echocardiographic characteristics of consecutive patients undergoing ASD closure or PVR between 2005 and 2014 at a single centre who had at least mild preoperative TR were collected and analysed.

Results: Overall, 162 patients (mean age at intervention 41.6±16.1 years, 38.3% male) were included: 101 after ASD closure (61 transcatheter vs 40 surgical) and 61 after PVR (3 transcatheter vs 58 surgical). Only 11.1% received concomitant tricuspid valve surgery (repair). There was significant reduction in the severity of TR in the overall population, from 38 (23.5%) patients having moderate or severe TR preoperatively to only 11 (6.8%) and 20 (12.3%) at 6 months and 12 months of follow-up, respectively (McNemar p<0.0001). There was a significant reduction in tricuspid valve annular diameter (p<0.0001), coaptation distance (p<0.0001) and systolic tenting area (p<0.0001). The reduction in TR was also observed in patients who did not have concomitant tricuspid valve (TV) repair (from 15.3% to 6.9% and 11.8% at 6 and 12 months, respectively, p<0.0001). On multivariable logistic regression including all univariable predictors of residual TR at 12 months, only RA area remained in the model (OR 1.2, 95% CI 1.04 to 1.37, p=0.01).

Conclusions: ASD closure and PVR are associated with a significant reduction in tricuspid regurgitation, even among patients who do not undergo concomitant tricuspid valve surgery.
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http://dx.doi.org/10.1136/heartjnl-2019-315287DOI Listing
March 2020

Transapical Mitral Melody Valve-in-Valve Implantation in a Child: An Interesting Alternative for Failing Melody Valve.

JACC Cardiovasc Interv 2019 08 31;12(16):e137-e138. Epub 2019 Jul 31.

Paediatric Cardiology and Cardiac Surgery Services, Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom; National Heart and Lung Institute, Imperial College, London, United Kingdom. Electronic address:

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http://dx.doi.org/10.1016/j.jcin.2019.06.005DOI Listing
August 2019

A single-centre, placebo-controlled, double-blind randomised cross-over study of nebulised iloprost in patients with Eisenmenger syndrome: A pilot study.

Int J Cardiol 2020 01 4;299:131-135. Epub 2019 Jul 4.

Adult Congenital Heart Disease Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital London, United Kingdom; National Heart and Lung Institute, Imperial College, London, United Kingdom. Electronic address:

Background: Pulmonary arterial hypertension (PAH), is a rare and progressive disease with a high morbidity and mortality. Prostanoid pulmonary vasodilators are the most effective treatment for idiopathic and connective tissue associated PAH. Nonetheless, data examining their safety and efficacy in patients with Eisenmenger syndrome the most severe form of PAH, that is, related to cyanotic congenital heart disease (CHD-PAH) remains limited.

Aim: To evaluate safety and the clinical efficacy of nebulised iloprost in patients with Eisenmenger syndrome who are on maximum background oral PAH therapy.

Methods: This pilot study was a randomised, double-blind, placebo-controlled, cross-over study. Patients were randomised to receive nebulised placebo or iloprost for 12 weeks and were then crossed over, with a 7-14-day washout. The primary endpoint was a change in 6-minute walk distance (6MWD).

Results: Sixteen patients (11 females, aged 47.3 ± 9.8 year) were recruited, twelve completed the study. All were in WHO-FC III, with a resting oxygen saturation of 84 [81-87] % and a median 6MWD of 290 [260-300] m. There was no significant difference in the primary endpoint between nebulised iloprost (0[-4-9]m) and placebo (10 [-15-51]m), p = 0.58. There were no safety concerns with nebulised iloprost.

Conclusions: Our pilot study provides preliminary evidence that the addition of nebulised iloprost to maximum oral PAH therapy did not improve the primary endpoint of 6MWD. Nebulised iloprost was well tolerated with no significant safety concerns in CHD-PAH.
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http://dx.doi.org/10.1016/j.ijcard.2019.07.004DOI Listing
January 2020

Covered Stent as a Bridge to Surgery for Obstructive Melody Pulmonary Valve Endocarditis.

Pediatr Cardiol 2019 Dec 27;40(8):1752-1755. Epub 2019 Jul 27.

Paediatric Cardiology and Cardiac Surgery Services, Royal Brompton Hospital, Sydney Street, London, SW36NP, UK.

Endocarditis is life threatening after percutaneous pulmonary valve implantation. We report a patient with acute heart failure caused by obstructive pulmonary vegetation in a Melody valve. Because of severe right ventricular dysfunction, immediate cardiac surgery was contraindicated. The patient underwent trans-catheter implantation of a covered stent to relieve obstruction. Following an initially good outcome, surgery was performed 2 weeks later. This was complicated by left main coronary artery tear and fatal bleeding. Despite covered stent was an effective bridge, surgery remains extremely challenging in such cases with important surrounding inflammation and fibrosis as well as potential proximity of coronary arteries.
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http://dx.doi.org/10.1007/s00246-019-02168-2DOI Listing
December 2019

Right Ventricular to Left Ventricular Ratio at CT Pulmonary Angiogram Predicts Mortality in Interstitial Lung Disease.

Chest 2020 01 24;157(1):89-98. Epub 2019 Jul 24.

National Pulmonary Hypertension Service, Royal Brompton and Harefield NHS Trust, London, England.

Background: Patients with interstitial lung disease (ILD) may develop pulmonary hypertension (PH), often disproportionate to the severity of the ILD. The right ventricular to left ventricular diameter (RV:LV) ratio measured at CT pulmonary angiogram (CTPA) has been shown to provide valuable information in patients with pulmonary arterial hypertension and to predict death or deterioration in acute pulmonary embolism.

Methods: Demographic characteristics, ILD subtype, echocardiography, and detailed CTPA measurements were collected in consecutive patients undergoing both CTPA and right heart catheterization at the Royal Brompton Hospital between 2005 and 2015. Fibrosis severity was formally scored according to CT criteria. The RV:LV ratio at CTPA was evaluated by using three different methods. Cox proportional hazards analysis was used to assess the relation of CTPA-derived parameters to predict death or lung transplantation.

Results: A total of 92 patients were included (64% male; mean age 65 ± 11 years) with an FVC 57 ± 20% predicted, corrected transfer factor of the lung for carbon monoxide 22 ± 8% predicted, and corrected transfer coefficient of the lung for carbon monoxide 51 ± 17% predicted. PH was confirmed at right heart catheterization in 78%. Of all the CTPA-derived measures, an RV:LV ratio ≥ 1.0 strongly predicted mortality or transplantation at univariate analysis (hazard ratio, 3.26; 95% CI, 1.49-7.13; P = .003), whereas invasive hemodynamic data did not. The RV:LV ratio remained an independent predictor at multivariate analysis (hazard ratio, 3.19; 95% CI, 1.44-7.10; P = .004), adjusting for an ILD diagnosis of idiopathic pulmonary fibrosis and CT imaging-derived ILD severity.

Conclusions: An increased RV:LV ratio measured at CTPA provides a simple, noninvasive method of risk stratification in patients with suspected ILD-PH. This should prompt closer follow-up, more aggressive treatment, and consideration of lung transplantation.
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http://dx.doi.org/10.1016/j.chest.2019.06.033DOI Listing
January 2020

Chronic thromboembolic pulmonary hypertension following long-term peripherally inserted central venous catheter use.

Pulm Circ 2019 Apr-Jun;9(2):2045894019859474

1 National Pulmonary Hypertension Service, Royal Brompton Hospital, London, UK.

A 36-year-old woman presented with recurrent pulmonary emboli (PE) despite oral anticoagulation. She was a type I diabetic with severe gastroparesis requiring insertion of multiple long-term peripherally inserted central catheters (PICC) over a 10-year period. Imaging at presentation demonstrated a PICC-associated mobile mass in the right atrium and signs of pulmonary hypertension (PH). She was thrombolyzed and fully anticoagulated, and diabetic management without PICC strongly recommended. PH persisted, however, and she developed chronic thromboembolic pulmonary hypertension (CTEPH), for which successful pulmonary endarterectomy (PEA) surgery led to symptomatic and hemodynamic improvement. This was the first case of CTEPH reported related to long-term PICC use outside the setting of malignant disease, and a novel observation that the PEA specimen contained multiple plastic fragments. Long-term PICC placement increases the risk of CTEPH, a life-threatening, albeit treatable, complication.
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http://dx.doi.org/10.1177/2045894019859474DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6598327PMC
June 2019

Efficacy of catheter ablation for atrial fibrillation in patients with congenital heart disease.

Europace 2019 Sep;21(9):1334-1344

Department of Cardiology, Royal Brompton and Harefield Hospital and Imperial College London, Sydney St, Chelsea, London, UK.

Aims: Advances in surgical techniques allow an increasing number of children with congenital heart disease (CHD) to reach adulthood. As patients grow older, atrial fibrillation (AF) is evolving into a major clinical concern and can be difficult to manage medically. Primary AF catheter ablation may, therefore, have a role in this setting but few reports have evaluated its efficacy in CHD patients.

Methods And Results: We retrospectively reviewed 58 consecutive patients [median age 51, interquartile range (IQR) 44-63 years, 57% male] with AF (45% paroxysmal) who underwent 122 ablation procedures in our tertiary centre in the last decade. The majority had CHD of moderate or severe complexity (57%, Bethesda Class 2 or 3) with a dilated left atrium (LA) (81%) and/or right atrium (86%). At 1-year from the first ablation, 32.8% of patients remained in sinus rhythm. Multiple procedures were required in 35 (60%) patients. Freedom from AF at 1-year after the 2nd and 3rd ablation was 40.9% and 36.5%, respectively. Multivariable predictors of AF recurrence were underlying anatomic complexity [hazard ratio (HR) in Bethesda 3 1.98, P = 0.006], type of AF (HR for persistent 1.87, P = 0.004), and indexed LA dimensions (HR for cm2/m2 1.06, P = 0.03).

Conclusion: While ablation may be a valid option for the treatment of AF in CHD patients, multiple procedures are likely to be required. Early referral and careful patient selection are essential to optimize the results of AF ablation, achieving a low rate of recurrence. Further studies are needed to validate our prognostic model and guide clinical practice.
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http://dx.doi.org/10.1093/europace/euz157DOI Listing
September 2019

Sex differences in publication volume and quality in congenital heart disease: are women disadvantaged?

Open Heart 2019;6(1):e000882. Epub 2019 Apr 3.

Department of Cardiology III - Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Muenster, Germany.

Background: Women are underrepresented in leading medical positions and academia. The gender-gap in authorship of congenital heart disease (CHD) publications remains unknown. As determinants of gender equity in this field are poorly characterised, we aimed to quantify and characterise publications in CHD and to assess factors associated with female representation in research.

Methods And Results: We identified 35 118 CHD publications between 2006 and 2015 for which author gender could be ascertained. Overall, 25.0% of all authors were female. Women accounted for 30.2% and 20.8% of all first and senior authorship positions with great geographic heterogeneity. While globally female first and senior authorship increased by 0.8% and 0.6%/year, some geographic regions showed no improvement in gender representation. Significant predictors of female first authorship on logistic regression analysis were country gross domestic product, human development index, gender inequality index and a female senior author (p<0.0001 for all). Publications with a female lead author tended to be published in journals with a higher impact factor (IF) and to attract more citations compared with those with a male author. Mixed gender authorship was associated with higher IF and number of citations. Women were less disadvantaged when the analysis was confined to original research.

Conclusions: While modest improvement in female authorship over time was noted, women remain underrepresented in contemporary academic CHD. Manuscripts with mixed gender authorship had higher IF and more citations. The main predictor of female first authorship was a female senior author. These data should inform policy recommendations regarding gender parity.
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http://dx.doi.org/10.1136/openhrt-2018-000882DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6519433PMC
February 2021

Early histological changes of pulmonary arterial hypertension disclosed by invasive cardiopulmonary exercise testing.

Pulm Circ 2019 Apr-Jun;9(2):2045894019845615

1 Adult Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK.

Early diagnosis of pulmonary artery hypertension (PAH) is diagnostically challenging given the extent of pulmonary vascular remodeling required to bring about clinical signs and symptoms. Exercise testing can be invaluable in this setting, as stressing the cardiopulmonary system may unmask early disease. This report describes a young patient with a positive family history of PAH in whom contemporaneous invasive cardiopulmonary exercise testing and surgical lung biopsy reveal the novel association between exercise pulmonary hypertension (ePH) and early histological changes of PAH. Exercise PH currently carries no pathological correlates which means the hemodynamic effects of early pulmonary vascular remodeling remain unknown. Following the recent proceedings from the World Symposium in Pulmonary Hypertension 2018, which broaden the hemodynamic definition of PAH, this report suggests an important association between ePH and early pulmonary vascular remodeling supporting a role for exercise hemodynamic evaluation in patients at increased familial risk of PAH.
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http://dx.doi.org/10.1177/2045894019845615DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6469282PMC
April 2019

Long-term mortality and cardiovascular burden for adult survivors of coarctation of the aorta.

Heart 2019 08 28;105(15):1190-1196. Epub 2019 Mar 28.

Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK.

Objective: To examine the contemporary long-term outcome after coarctation repair.

Methods: This is a retrospective cohort study of 834 patients aged ≥16 years who underwent coarctation repair under single-centre follow-up. Repair was performed at a median age of 3 years (lower-upper quartile: 1 month to 15 years) by surgery in 83% (690/834) and angioplasty/stenting in 17% (144/834). Survival was compared with an age- and gender matched normal population. Other outcomes included arch reintervention, aortic valve intervention, ascending aortic intervention, and residual/re-coarctation and resting hypertension at latest follow-up.

Results: After a median follow-up of 27 years (lower-upper quartile: 18-36), there were 38 late deaths (5%, 38/834). Overall survival was 99%, 88% and 65% at 30, 50 and 70 years of age, respectively, significantly reduced compared with a matched normal population (standardised mortality ratio: 3.20, log-rank: p<0.001). Thirty per cent (246/834) required ≥1 arch reintervention, 13% (111/834) an aortic valve intervention and 5% (43/834) an ascending aortic intervention. Freedom from aortic valve and ascending aortic intervention was 83% and 92% at 50 years and 53% and 81% at 70 years of age, respectively. Residual/re-coarctation (gradient ≥25 mm Hg or repair site/diaphragm ratio ≤70%) at latest follow-up was present in 60% (282/474) and resting hypertension in 57% (379/661).

Conclusions: Long-term survival in contemporary adult survivors of coarctation repair is significantly lower than a matched normal population with accelerated decline after the third decade. Nearly 60% of patients eventually develop hypertension, whereas approximately 50% require further invasive cardiovascular treatment by 50 years of age. Our risk-stratifying data may enable personalised follow-up strategies for this common congenital heart condition.
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http://dx.doi.org/10.1136/heartjnl-2018-314257DOI Listing
August 2019
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