Morbidity and mortality in adults with a Fontan circulation beyond the fourth decade of life.

AIMS: To evaluate the late outcomes of adults (above 35 years) with a Fontan-type circulation, for whom current data on morbidity and mortality are lacking. METHODS AND RESULTS: Data were collected retrospectively on consecutive patients with Fontan circulation above the age of 35 years followed in three European specialist centres. Overall, 115 Fontan patients were included [median age 35 (range 35-48) years, 47.8% female]. The most common underlying congenital heart disease diagnosis was tricuspid atresia (n = 58, 50.4%), and the age at first Fontan completion was 9.1 (interquartile range 5.0-15.8) years. Almost two-thirds (61.7%) of patients had undergone an atriopulmonary Fontan, and 23.5% had received a total cavopulmonary connection. One-third required repeat surgery or intervention. Most patients (55.9%) were in New York Heart Association functional class II or class I (30.6%), 76 (66.1%) patients had experienced at least one arrhythmia, and eight (7.0%) protein-losing enteropathy. At a median follow-up of 5.0 (2.4-10.3) years, 15 (13.0%) patients were referred for transplantation assessment and 19 (16.5%) patients died, mainly from heart failure (84.2%). Univariable predictors of death or transplantation included lower serum albumin level [hazard ratio (HR) 1.09 per g/L decrease, 95% confidence interval (CI): 1.04-1.15, P = 0.0009], prior heart failure admission (HR 4.28, 95% CI:1.75-10.44, P = 0.001), prior atrial tachycardia or flutter (HR 3.02, 95% CI: 1.23-7.38, P = 0.02), and baseline pulmonary vasodilator therapy (HR 8.59, 95% CI:1.05-70.13, P = 0.04). Lower serum albumin and prior atrial tachycardia or flutter remained significant on bivariable analysis. CONCLUSION: Our study highlights the significant morbidity and mortality in older adults with a Fontan-type circulation, emphasizing the need for lifelong specialist surveillance with frequent risk stratification, close monitoring, and early consideration for transplantation assessment.

This study sheds light on the complex medical journey of adults living with the outcomes of Fontan surgery­a procedure performed in early childhood. These individuals have reached the milestone of their forties and beyond, yet they confront an array of significant health challenges that necessitate lifelong, individualized congenital heart disease care. The key findings are as follows: While adults with Fontan circulation are living longer, they are at high risk of death, mainly due to heart failure. They also face a host of other health issues, including the need for additional surgeries or interventions. Nearly two-thirds have experienced some form of heart rhythm problem, and a substantial number eventually require evaluation for a heart transplant.Heart transplants within this group were rare, which may be linked to the various barriers to transplantation in the Fontan population. Moreover, those with multiple indicators of advanced disease have a heightened risk of life-threatening events, reinforcing the critical need for personalized and continuous specialist care designed to meet their distinct health requirements.

Repair of Aortic Regurgitation in Young Adults: Sooner Rather Than Later.

Background Establishing surgical criteria for aortic valve replacement (AVR) in severe aortic regurgitation in young adults is challenging due to the lack of evidence-based recommendations. We studied indications for AVR in young adults with severe aortic regurgitation and their outcomes, as well as the relationship between presurgical echocardiographic parameters and postoperative left ventricular (LV) size, function, clinical events, and valve-related complications. Methods and Results Data were collected retrospectively on 172 consecutive adult patients who underwent AVR or repair for severe aortic regurgitation between 2005 and 2019 in a tertiary cardiac center (age at surgery 29 [22-41] years, 81% male). One-third underwent surgery before meeting guideline indications. Postsurgery, 65% achieved LV size and function normalization. LV ejection fraction showed no significant change from baseline. A higher presurgical LV end-systolic diameter correlated with a lack of LV normalization (odds ratio per 1-cm increase 2.81, P<0.01). The baseline LV end-systolic diameter cut-off for predicting lack of LV normalization was 43 mm. Pre- and postoperative LV dimensions and postoperative LV ejection fraction predicted clinical events during follow-up. Prosthetic valve-related complications occurred in 20.3% during an average 5.6-year follow-up. Freedom from aortic reintervention was 98%, 96.5%, and 85.4% at 1, 5, and 10 years, respectively. Conclusions Young adult patients with increased baseline LV end-systolic diameter or prior cardiac surgery are less likely to achieve LV normalization after AVR. Clinicians should carefully balance the long-term benefits of AVR against procedural risks and future interventions, especially in younger patients. Evidence-based criteria for AVR in severe aortic regurgitation in young adults are crucial to improve outcomes.

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

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.

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

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.

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

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.

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

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.

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

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.

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

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.

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

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. (Level of Difficulty: Intermediate.).

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.

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

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.

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

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.

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

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.

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

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.

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

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.

Surgical and percutaneous pulmonary valve replacement in England over the past two decades.

OBJECTIVE: Pulmonary 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. METHODS: Patients were identified in the Hospital Episode Statistics Database. Survival data were retrieved from the UK Office for National Statistics. RESULTS: A 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 >16 years and percutaneous PVR were risk factors for death. The cumulative incidence for re-PVR at 10 years was 8% for all PVRs and 11% at 15 years. Risk factors for re-PVR were complex diagnosis, male gender and black ethnicity. CONCLUSION: There was a significant increase in the number of PVRs performed in England over the last two decades and a significant change in the type of prosthesis employed. While early mortality was low across the board, longer-term mortality was not negligible in this young population.

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.