The Role of Ablation in Prevention of Recurrent Implantable Cardioverter Defibrillator Shocks in Patients With Tetralogy of Fallot.

BACKGROUND: Implantable cardioverter defibrillators (ICDs) are effective in preventing arrhythmic sudden cardiac death in patients with tetralogy of Fallot (TOF). Although ICD therapies for malignant ventricular arrhythmias can be life-saving, shocks could have deleterious consequences. Substrate-based ablation therapy has become the standard of care to prevent recurrent ICD shocks in patients with ischemic cardiomyopathy. However, the efficacy and safety of this invasive therapy in the prevention of recurrent ICD shocks in patients with TOF has not been well evaluated. METHODS: Records of a total of 47 consecutive TOF patients (mean age: 43.1 ± 13.2 years, male sex: n = 34 [72.3%]) who underwent ICD implantation for secondary prevention between 2000 and 2018 were reviewed. RESULTS: Twenty (42.6%) patients underwent invasive therapy (radiofrequency catheter ablation, n = 8; surgical ablation with pulmonary valve replacement, n = 12) before ICD implantation. Twenty-seven patients (57.4%) were managed noninvasively. During follow-up (median 80.5 [interquartile range, 28.5-131.0] months), 2 (10.0%) patients in the invasive group and 10 (37.0%) patients in the noninvasive group received appropriate ICD shocks (P = 0.036). Logistic regression analysis showed that invasive therapy was associated with a decreased risk of ICD shocks by 81.1% (odds ratio, 0.189; 95% confidence interval, 0.036-0.990; P = 0.049). Furthermore, invasive therapy was associated with decreased risk of the composite outcomes of ICD shock, death, cardiac transplantation, and hospital admission (odds ratio, 0.090; 95% confidence interval, 0.025-0.365; P = 0.013) compared with noninvasive therapy. CONCLUSIONS: Invasive substrate modification therapy was associated with a lower likelihood of ICD shocks and improvement of long-term outcomes in TOF patients.

CONTEXTE: Les défibrillateurs cardioverteurs implantables (DCI) sont efficaces pour prévenir la mort cardiaque subite provoquée par une arythmie chez les patients présentant une tétralogie de Fallot (TF). Bien que le traitement des arythmies ventriculaires malignes par DCI puisse sauver des vies, les chocs administrés peuvent avoir des conséquences délétères. L'ablation du substrat est devenue le traitement de référence pour prévenir l'administration à répétition de chocs par DCI chez les patients atteints d'une cardiomyopathie ischémique. L'efficacité et l'innocuité de ce traitement invasif pour prévenir l'administration de chocs répétés chez les patients présentant une TF n'ont toutefois pas été bien évaluées. MÉTHODOLOGIE: Nous avons examiné les cas consécutifs de 47 patients présentant une TF (âge moyen : 43,1 ± 13,2 ans; hommes : n = 34 [72,3 %]) ayant reçu un DCI en prévention secondaire entre 2000 et 2018. RÉSULTATS: Au total, 20 (42,6 %) patients ont subi un traitement invasif (ablation par cathéter par radiofréquence, n = 8; ablation chirurgicale et remplacement de la valve pulmonaire, n = 12) avant l'implantation d'un DCI. Vingt-sept patients (57,4 %) ont été pris en charge de façon non invasive. Au cours de la période de suivi (durée médiane de 80,5 [intervalle interquartile : 28,5 à 131,0] mois), 2 (10,0 %) patients du groupe ayant subi une intervention invasive et 10 (37,0 %) patients du groupe ayant subi une intervention non invasive ont reçu un choc approprié par DCI (p = 0,036). Les résultats de l'analyse par régression logistique montrent que le traitement invasif est associé à une réduction du risque de choc par DCI de 81,1 % (rapport des cotes : 0,189; intervalle de confiance à 95 % : de 0,036 à 0,990; p = 0,049). En outre, le traitement invasif est associé à une réduction du risque de survenue d'un des événements du paramètre d'évaluation composé, soit un choc administré par DCI, le décès, une transplantation cardiaque ou une hospitalisation (rapport des cotes : 0,090; intervalle de confiance à 95 % : de 0,025 à 0,365; p = 0,013) par rapport au traitement non invasif. CONCLUSIONS: La modification invasive du substrat a été associée à une probabilité plus faible de choc administré par DCI et à une amélioration des résultats à long terme chez les patients présentant une TF.

Recommendations for advance care planning in adults with congenital heart disease: a position paper from the ESC Working Group of Adult Congenital Heart Disease, the Association of Cardiovascular Nursing and Allied Professions (ACNAP), the European Association for Palliative Care (EAPC), and the International Society for Adult Congenital Heart Disease (ISACHD).

Survival prospects in adults with congenital heart disease (CHD), although improved in recent decades, still remain below expectations for the general population. Patients and their loved ones benefit from preparation for both unexpected and predictable deaths, sometimes preceded by a prolonged period of declining health. Hence, advance care planning (ACP) is an integral part of comprehensive care for adults with CHD. This position paper summarizes evidence regarding benefits of and patients' preferences for ACP and provides practical advice regarding the implementation of ACP processes within clinical adult CHD practice. We suggest that ACP be delivered as a structured process across different stages, with content dependent upon the anticipated disease progression. We acknowledge potential barriers to initiate ACP discussions and emphasize the importance of a sensitive and situation-specific communication style. Conclusions presented in this article reflect agreed expert opinions and include both patient and provider perspectives.

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

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 ±â€¯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.

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

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.

Heart or heart-lung transplantation for patients with congenital heart disease in England.

BACKGROUND: Increased longevity in patients with congenital heart disease (CHD) is associated with late complications, mainly heart failure, which may not be amenable to redo surgery and become refractory to medical therapy and so, trigger referral for transplantation. We assessed the current role and future prospects of heart and heart-lung transplantation for patients with CHD in England. METHODS: We performed a retrospective analysis of hospital episodes for England for 1997-2015, identifying patients with a CHD code (ICD-10 'Q2xx.x'), who underwent heart or heart-lung transplantation. RESULTS: In total, 469 transplants (82.2% heart and 17.8% heart-lung) were performed in 444 patients. Half of patients transplanted had mild or moderate CHD complexity, this percentage increased with time (p=0.001). While overall, more transplantations were performed over the years, the proportion of heart-lung transplants declined (p<0.0001), whereas the proportion of transplants performed in adults remained static. Mortality was high during the first year, especially after heart-lung transplantation, but remained relatively low thereafter. Older age and heart-lung transplantation were strong predictors of death. While an increase in CHD transplants is anticipated, actual numbers in England seem to lag behind the increase in CHD patients with advanced heart failure. CONCLUSIONS: The current and future predicted increase in the numbers of CHD transplants does not appear to parallel the expansion of the CHD population, especially in adults. Further investment and changes in policy should be made to enhance the number of donors and increase CHD transplant capacity to address the increasing numbers of potential CHD recipients and optimise transplantation outcomes in this growing population.

The outcome of adults born with pulmonary atresia: High morbidity and mortality irrespective of repair.

OBJECTIVES: To describe the characteristics and long-term outcome of a large adult cohort with pulmonary atresia. BACKGROUND: Patients with pulmonary atresia (PA) are a heterogeneous population in terms of anatomy, physiology and surgical history, and their management during adulthood remains challenging. METHODS: 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). RESULTS: Two-hundred twenty-seven patients with PA were identified, 66.1% female, mean age 25.5 ±â€¯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. CONCLUSIONS: 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.

Use of intravenous iron in cyanotic patients with congenital heart disease and/or pulmonary hypertension.

BACKGROUND: Secondary erythrocytosis is common in patients with cyanosis secondary to congenital heart disease (CHD) and/or pulmonary hypertension (PH). This compensatory mechanism aims at increasing oxygen delivery to the tissues, but it requires adequate iron stores. Optimal methods of iron supplementation in this setting remain controversial, with fears of excessive erythropoiesis and hyperviscosity symptoms. We describe our experience using intravenous ferrous carboxymaltose. METHODS AND RESULTS: 142 consecutive cyanotic patients were treated over 5.7 years (201 administrations). Mean age was 51.3 ±â€¯17.6 years and 55 (38.7%) were male. Eisenmenger syndrome (ES) was present in 41 (28.8%), other pulmonary arterial hypertension (PAH) related to CHD (PAH-CHD) in 27 (19.0%), cyanotic CHD without PAH in 16 (11.3%) and PH without CHD in 58(40.8%). Baseline haemoglobin (Hb) concentration was 14.6 ±â€¯3.0 g/dL and haematocrit 0.45 ±â€¯0.09. A 500 mg dose of intravenous (IV) iron carboxymaltose was given in 163 (81.1%) of administrations and a 1000 mg dose in 37 (18.4%). A significant improvement in average Hb, haematocrit, ferritin and transferrin saturation was observed after a median follow-up of 100.0 [70.0-161.0] days (p ≤ 0.0001 for all). There were no cases of excessive erythropoiesis resulting in new hyperviscosity symptoms and/or requiring venesection. A minor transient rash was observed in 2 patients and one patient experienced an air embolus causing a transient ischemic attack. CONCLUSIONS: Intravenous ferrous carboxymaltose appears to be safe in iron deficient patients with cyanosis due to CHD and/or PH, as long as care is taken to avoid air emboli. Further randomised studies are needed to confirm the safety and efficacy of intravenous iron in this setting.

The CRASH report: emergency management dilemmas facing acute physicians in patients with pulmonary arterial hypertension.

Treatment of acute emergencies in patients with pulmonary arterial hypertension (PAH) can be challenging. In the UK and Ireland, management of adult patients with PAH is centred in eight nationally designated pulmonary hypertension (PH) centres. However, many patients live far from these centres and physicians in local hospitals are often required to manage PAH emergencies. A committee of physicians from nationally designated PH centres identified the 'most common' emergency clinical scenarios encountered in patients with PAH. Thereafter, a review of the literature was performed centred on these specified topics and a management approach was developed based on best available evidence and expert consensus. Management protocols were developed on the following PAH emergencies: chest pain (including myocardial ischaemia), right ventricular failure, arrhythmias, sepsis, haemoptysis ('CRASH'), as well as considerations relevant to surgery, anaesthesia and pregnancy. Emergencies are not uncommon in PAH. While expertise in PAH management is essential, all physicians involved in acute care should be aware of the principles of acute management of PAH emergencies. A multidisciplinary approach is necessary, with physicians from tertiary PH centres supporting care locally and planning safe transfer of patients to PH centres when appropriate.

Effect of Pregnancy on Ventricular and Aortic Dimensions in Repaired Tetralogy of Fallot.

BACKGROUND: The aim was to assess whether cardiovascular adaptation to pregnancy in women with repaired tetralogy of Fallot (TOF) adversely affects hemodynamic stability, in particular with respect to right ventricular (RV) dilatation, pulmonary regurgitation, or aortic root dilatation. METHODS AND RESULTS: This was a retrospective cohort study of women with repaired TOF with paired cardiovascular magnetic resonance scans before and after their first pregnancy (baseline RV end systolic volume index 49 mL/m2 and RV end diastolic volume index 118 mL/m2) matched with a comparison group of nulliparous women with TOF. Cases were matched for age at baseline cardiovascular magnetic resonance scan, time between follow-up of cardiovascular magnetic resonance scans, QRS duration, RV ejection fraction, and indexed RV end systolic and diastolic volume at baseline. Effect of pregnancy and time on parameters was assessed using mixed-effects modelling. Nineteen women with repaired TOF who had completed their first pregnancy were identified and matched with 38 nulliparous women. We observed no deleterious effects of pregnancy on RV volumes, aortic dimensions, or exercise data. There was an effect of pregnancy observed in both left ventricular end diastolic volume and left ventricular stroke volume, consistent with a sustained small increase in left ventricular stroke volume attributed to pregnancy (53-55 mL/m2). CONCLUSIONS: Women with repaired TOF and with mild-to-moderate RV dilatation considering pregnancy can be reassured that pregnancy is unlikely to cause deterioration in their cardiovascular status. We recommend that women are routinely assessed and followed up before and after pregnancy and that prepregnancy counseling is tailored to their individual clinical status.

Organisation of care for pregnancy in patients with congenital heart disease.

Improvements in surgery have resulted in more women with repaired congenital heart disease (CHD) surviving to adulthood. Women with CHD, who wish to embark on pregnancy require prepregnancy counselling. This consultation should cover several issues such as the long-term prognosis of the mother, fertility and miscarriage rates, recurrence risk of CHD in the baby, drug therapy during pregnancy, estimated maternal risk and outcome, expected fetal outcomes and plans for pregnancy. Prenatal genetic testing is available for those patients with an identified genetic defect using pregestational diagnosis or prenatal diagnosis chorionic villus sampling or amniocentesis. Centralisation of care is needed for high-risk patients. Finally, currently there are no recommendations addressing the issue of the delivery. It is crucial that a dedicated plan for delivery should be available for all cardiac patients. The maternal mortality in low-income to middle-income countries is 14 times higher than in high-income countries and needs additional aspects and dedicated care.

Body mass index in adult congenital heart disease.

OBJECTIVE: Abnormal body mass index (BMI) is associated with higher mortality in various cardiovascular cohorts. The prognostic implications of BMI in adults with congenital heart disease (ACHD) are unknown. We aim to assess the distribution of BMI and its association with symptoms and survival in the ACHD population. METHODS: We included 3069 ACHD patients (median age 32.6 years) under follow-up at our institution between 2001 and 2015. Patients were classified based on BMI as underweight (<18.5), normal weight (18.5-25), overweight (25-30) or obese (>30), and symptoms, exercise capacity and mortality were assessed. RESULTS: Overall, 6.2% of patients were underweight, 51.1% had normal weight, 28.2% were overweight and 14.6% were obese. Higher BMI values were associated with lower all-cause and cardiac mortality on univariable Cox analysis, and this effect persisted after adjustment for age, defect complexity, cyanosis and objective exercise capacity. Higher BMI was especially associated with better prognosis in symptomatic ACHD patients (HR 0.94 (95% CI 0.90 to 0.98), p=0.002) and those with complex underlying cardiac defects (HR 0.96 (95% CI 0.91 to 0.997), p=0.048) In patients with a complex cardiac defect who had repeated weight measurements, weight loss was also associated with a worse survival (HR 1.82 (95% CI 1.02 to 3.24), p=0.04). CONCLUSIONS: ACHD patients with a higher BMI had a lower mortality. The association between BMI and mortality was especially pronounced in symptomatic patients with complex underlying cardiac defects, suggesting that cardiac cachexia may play a role. Indeed, weight loss in complex ACHD patients was linked to an even higher mortality.

Clinical course and potential complications of small ventricular septal defects in adulthood: Late development of left ventricular dysfunction justifies lifelong care.

BACKGROUND: Patients with small ventricular septal defects (VSDs) are thought to have excellent long-term survival, although complications may not be uncommon. METHODS: We identified all patients aged ≥ 16 years with native isolated VSD between January 2000 and September 2013. Clinical outcomes were retrospectively reviewed. Transthoracic echocardiograms performed within 2 years of last assessment were reviewed for hemodynamic sequelae. RESULTS: Two-hundred-and-thirty-one patients, 100 (43%) male, mean age at last follow-up 34 ± 13 years were studied. During the recorded period there were no deaths. The majority (224/231; 97%) were asymptomatic. Documented arrhythmias occurred in 7 patients (3%), double-chamber right ventricle (DCRV) in 29 (13%), more than mild aortic regurgitation in 6 (3%) and infective endocarditis in 24 (10%) patients. Surgery due to complications associated with VSD was performed in 26 (11%) patients at a median age of 27.6 years (IQR: 16.1-38.7) due to DCRV (n=17, 65%), infective endocarditis (n=6, 23%), progression of left-right shunt (n=2, 8%) and aortic regurgitation (n=1, 4%). At most recent echocardiography (n=164), 10 (6%), had reduced LVEF, 34 (21%) had increased LVEDD and 17 (10%) had LVESD >4.0 cm. Thirty-two patients (25%) with normal LV dimensions had LA enlargement suggesting LV diastolic dysfunction. CONCLUSIONS: We report a non-negligible incidence of major complications or clinical events during late follow-up of adult patients with restrictive VSDs. Furthermore, we show co-existing LV dysfunction, systolic or diastolic in a subset of patients. Indication for VSD closure in childhood may be recognized, whereas lifelong follow-up for adult with restrictive VSDs is clearly warranted.

Depression requiring anti-depressant drug therapy in adult congenital heart disease: prevalence, risk factors, and prognostic value.

BACKGROUND: Depression is prevalent in adults with congenital heart disease (ACHD), but limited data on the frequency of anti-depressant drug (ADD) therapy and its impact on outcome are available. METHODS AND RESULTS: We identified all ACHD patients treated with ADDs between 2000 and 2011 at our centre. Of 6162 patients under follow-up, 204 (3.3%) patients were on ADD therapy. The majority of patients were treated with selective serotonin-reuptake inhibitors (67.4%), while only 17.0% of patients received tricyclic anti-depressants. Twice as many female patients used ADDs compared with males (4.4 vs. 2.2%, P < 0.0001). The percentage of patients on ADDs increased with disease complexity (P < 0.0001) and patient age (P < 0.0001). Over a median follow-up of 11.1 years, 507 (8.2%) patients died. After propensity score matching, ADD use was found to be significantly associated with worse outcome in male ACHD patients [hazard ratio 1.44 (95% confidence interval 1.17-1.84)]. There was no evidence that this excess mortality was directly related to ADD therapy, QT-prolongation, or malignant arrhythmias. However, males taking ADDs were also more likely to miss scheduled follow-up appointments compared with untreated counterparts, while no such difference in clinic attendance was seen in females. CONCLUSIONS: The use of ADD therapy in ACHD relates to gender, age, and disease complexity. Although, twice as many female patients were on ADDs, it were their male counterparts, who were at increased mortality risk on therapy. Furthermore, males on ADDs had worse adherence to scheduled appointments suggesting the need for special medical attention and possibly psychosocial intervention for this group of patients.

Survival Prospects and Circumstances of Death in Contemporary Adult Congenital Heart Disease Patients Under Follow-Up at a Large Tertiary Centre.

BACKGROUND: Adult congenital heart disease (ACHD) patients have ongoing morbidity and reduced long-term survival. Recently, the importance of specialized follow-up at tertiary ACHD centers has been highlighted. We aimed to assess survival prospects and clarify causes of death in a large cohort of patients at a single, tertiary center. METHODS AND RESULTS: We included 6969 adult patients (age 29.9 ± 15.4 years) under follow-up at our institution between 1991 and 2013. Causes of death were ascertained from official death certificates. Survival was compared with the expected survival in the general age- and sex-matched population, and standardized mortality rates were calculated. Over a median follow-up time of 9.1 years (interquartile range, 5.2-14.5), 524 patients died. Leading causes of death were chronic heart failure (42%), pneumonia (10%), sudden-cardiac death (7%), cancer (6%), and hemorrhage (5%), whereas perioperative mortality was comparatively low. Isolated simple defects exhibited mortality rates similar to those in the general population, whereas patients with Eisenmenger syndrome, complex congenital heart disease, and Fontan physiology had much poorer long-term survival (P<0.0001 for all). The probability of cardiac death decreased with increasing patient's age, whereas the proportion of patients dying from noncardiac causes, such as cancer, increased. CONCLUSIONS: ACHD patients continue to be afflicted by increased mortality in comparison with the general population as they grow older. Highest mortality rates were observed among patients with complex ACHD, Fontan physiology, and Eisenmenger syndrome. Our data provide an overview over causes of mortality and especially the spectrum of noncardiac causes of death in contemporary ACHD patients.

Systemic right ventricular fibrosis detected by cardiovascular magnetic resonance is associated with clinical outcome, mainly new-onset atrial arrhythmia, in patients after atrial redirection surgery for transposition of the great arteries.

BACKGROUND: We hypothesized that fibrosis detected by late gadolinium enhancement (LGE) cardiovascular magnetic resonance predicts outcomes in patients with transposition of the great arteries post atrial redirection surgery. These patients have a systemic right ventricle (RV) and are at risk of arrhythmia, premature RV failure, and sudden death. METHODS AND RESULTS: Fifty-five patients (aged 27±7 years) underwent LGE cardiovascular magnetic resonance and were followed for a median 7.8 (interquartile range, 3.8-9.6) years in a prospective single-center cohort study. RV LGE was present in 31 (56%) patients. The prespecified composite clinical end point comprised new-onset sustained tachyarrhythmia (atrial/ventricular) or decompensated heart failure admission/transplantation/death. Univariate predictors of the composite end point (n=22 patients; 19 atrial/2 ventricular tachyarrhythmia, 1 death) included RV LGE presence and extent, RV volumes/mass/ejection fraction, right atrial area, peak Vo(2), and age at repair. In bivariate analysis, RV LGE presence was independently associated with the composite end point (hazard ratio, 4.95 [95% confidence interval, 1.60-15.28]; P=0.005), and only percent predicted peak Vo(2) remained significantly associated with cardiac events after controlling for RV LGE (hazard ratio, 0.80 [95% confidence interval, 0.68-0.95]; P=0.009/5%). In 8 of 9 patients with >1 event, atrial tachyarrhythmia, itself a known risk factor for mortality, occurred first. There was agreement between location and extent of RV LGE at in vivo cardiovascular magnetic resonance and histologically documented focal RV fibrosis in an explanted heart. There was RV LGE progression in a different case restudied for clinical indications. CONCLUSIONS: Systemic RV LGE is strongly associated with adverse clinical outcome especially arrhythmia in transposition of the great arteries, thus LGE cardiovascular magnetic resonance should be incorporated in risk stratification of these patients.

Long-term natural history and postoperative outcome of double-chambered right ventricle--experience from two tertiary adult congenital heart centres and review of the literature.

BACKGROUND: Double-chambered right ventricle (DCRV) is a rare form of congenital heart disease. Little is known about the outcome during adult life. Here we report the combined experience of two tertiary Adult Congenital Heart Disease Centres and systematically review the published literature. RESULTS: 50 patients (32 female, median age 39 years [IQR: 27;53]) with DCRV under follow-up could be identified. A retrospective review of medical records was performed. Almost all patients (96%) had ventricular septal defects as underlying diagnosis. Eight patients remained completely asymptomatic during follow-up and have been managed conservatively thus far. The remaining patients developed symptoms at a median age of 26 years. Surgical correction was performed in 33 patients (median age at operation 27 years). No residual intraventricular gradient was present at the latest follow-up in 91% of operated patients and functional class improved significantly with only 6 patients remaining in NYHA class 2. There was no early or late operative mortality and no patient required re-operation for DCRV during a median follow-up of 8 years. CONCLUSIONS: Contemporary adult DCRV patients have good survival prospects and low long-term morbidity. Despite occasionally presenting with considerable intraventricular gradients we could not identify any case of sudden death in our unoperated adult sub-population. Although asymptomatic adults may be encountered even with severe obstruction, symptom development is common during adult life. Cardiac surgery in this cohort is inherently low risk and offers good long-term haemodynamic and functional results justifying early intervention in consideration of the progressive nature of this rare congenital lesion.

Histopathology of the great vessels in patients with pulmonary arterial hypertension in association with congenital heart disease: large pulmonary arteries matter too.

BACKGROUND: Pulmonary arterial hypertension (PAH) is considered primarily a disease of the distal pulmonary arteries whereas little is known on the effect of long-standing pulmonary hypertension on the larger proximal pulmonary arteries. This study aims to investigate the structural changes in the great arteries of adults who developed PAH in association with congenital heart disease (CHD), with severe cases termed Eisenmenger syndrome. METHODS: We performed macroscopic and light microscopy analyses on the great arteries of 10 formalin-fixed human hearts from patients with PAH/CHD and compared them to age-matched healthy controls. A detailed histology grading score was used to assess the severity of medial wall abnormalities. RESULTS: Severe atherosclerotic lesions were found macroscopically in the elastic pulmonary arteries of 4 PAH/CHD specimens and organised thrombi in 3; none were present in the controls. Significant medial wall abnormalities were present in the pulmonary trunk (PT), including fibrosis (80%), and atypical elastic pattern (80%). Cyst-like formations were present in less than one third of patients and were severe in a single case leading to wall rupture. The cumulative PT histology grading score was significantly higher in PAH/CHD cases compared to controls (p<0.0001) and correlated positively with larger PT diameters (ρ=0.812, p<0.0001) and the degree of medial wall hypertrophy (ρ=0.749, p<0.0001). CONCLUSIONS: Chronic PAH in association with CHD results in marked macroscopic and histological abnormalities in the large pulmonary arteries. These abnormalities are likely to affect haemodynamics and contribute to morbidity and mortality in this cohort.

Cardiothoracic ratio from postero-anterior chest radiographs: a simple, reproducible and independent marker of disease severity and outcome in adults with congenital heart disease.

OBJECTIVE: The wide spectrum of intracardiac anatomy and reparative surgery available for adults with congenital heart disease (ACHD) makes uniform measurement of cardiac size and disease severity challenging. The aim of this study was to assess the prognostic potential of cardiothoracic ratio, a simple marker of cardiomegaly, in a large cohort of ACHD. PATIENTS AND SETTING: Chest radiographs from 3033 ACHD patients attending our institution between 1998 and 2007 and 113 normal controls of similar age were analyzed blindly. DESIGN: Cardiothoracic ratio derived from plain postero-anterior chest radiographs, was compared between ACHD patients and controls, different diagnostic subgroups and different functional classes. Relationship between cardiothoracic ratio and survival was assessed using Cox regression. RESULTS: Average cardiothoracic ratio in ACHD was 52.0±7.6% (over 50% in 56.4%), significantly higher in all ACHD diagnostic subgroups compared to controls (42.3±4.0%, p<0.0001) and highest in the "complex" cardiac anatomy, Ebstein's anomaly and Eisenmenger subgroups. Cardiothoracic ratio related to functional class, but was high even in asymptomatic patients. During a median follow-up of 4.2years, 164 patients died. Patients with a cardiothoracic ratio >55% had an 8-fold increased risk of death compared to those in the lowest tertile (<48%). Even patients with mildly increased cardiothoracic ratio (48-55%) had an adjusted 3.6-fold increased mortality compared to the lowest tertile. CONCLUSIONS: Cardiothoracic ratio derived from postero-anterior chest radiographs is a simple, and reproducible marker, which relates to functional class and predicts independently mortality risk in ACHD patients.

Detrimental impact of socioeconomic status on exercise capacity in adults with congenital heart disease.

OBJECTIVES: To evaluate the relationship between socioeconomic status (SES), access to physical activity resources, urban-rural dwelling, levels of pollution and exercise capacity in adult congenital heart disease (ACHD) patients. BACKGROUND: Exercise intolerance is prevalent in ACHD and the contributing factors are poorly understood. METHODS: A total of 1268 ACHD patients living in England who underwent cardiopulmonary exercise testing at our center were included. Neighborhood deprivation (English Indices of Deprivation), urban-rural dwelling, availability of green space, distance to the closest gym/fitness center and levels of pollution were estimated based on administrative data. RESULTS: Urban-rural dwelling, availability of green space and levels of pollution were unrelated to exercise capacity. Lower SES was associated with a significantly lower peak oxygen consumption (P<0.002) and heart rate reserve (P<0.004). This association was non-linear and most pronounced in ACHD patients with cardiac defects of medium complexity living in the most socioeconomically disadvantaged communities. Low SES was associated with higher prevalence of diabetes (P=0.015) and smoking (P=0.01). Coronary artery disease was rare in this young population and low SES was found to be related to exercise capacity independently of the presence of coronary artery disease. CONCLUSIONS: Living in poorer areas is associated with exercise intolerance in contemporary ACHD patients. Although low SES is linked to traditional cardiovascular risk factors, the deleterious effects of SES on exercise capacity seem to be only partially mediated via coronary artery disease. Reducing social inequalities in ACHD patients may have a positive effect on quality of life and long-term prognostic implications.