Hypoalbuminaemia predicts outcome in adult patients with congenital heart disease

Background In patients with acquired heart failure, hypoalbuminaemia is associated with increased risk of death. The prevalence of hypoproteinaemia and hypoalbuminaemia and their relation to outcome in adult patients with congenital heart disease (ACHD) remains, however, unknown. Methods Data on patients with ACHD who underwent blood testing in our centre within the last 14 years were collected. The relation between laboratory, clinical or demographic parameters at baseline and mortality was assessed using Cox proportional hazards regression analysis. Results A total of 2886 patients with ACHD were included. Mean age was 33.3 years (23.6–44.7) and 50.1% patients were men. Median plasma albumin concentration was 41.0 g/L (38.0–44.0), whereas hypoalbuminaemia (<35 g/L) was present in 13.9% of patients. The prevalence of hypoalbuminaemia was significantly higher in patients with great complexity ACHD (18.2%) compared with patients with moderate (11.3%) or simple ACHD lesions (12.1%, p<0.001). During a median follow-up of 5.7 years (3.3–9.6), 327 (11.3%) patients died. On univariable Cox regression analysis, hypoalbuminaemia was a strong predictor of outcome (HR 3.37, 95% CI 2.67 to 4.25, p<0.0001). On multivariable Cox regression, after adjusting for age, sodium and creatinine concentration, liver dysfunction, functional class and disease complexity, hypoalbuminaemia remained a significant predictor of death. Conclusions Hypoalbuminaemia is common in patients with ACHD and is associated with a threefold increased risk of risk of death. Hypoalbuminaemia, therefore, should be included in risk-stratification algorithms as it may assist management decisions and timing of interventions in the growing ACHD population

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

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

Right atrial area and right ventricular outflow tract akinetic length predict sustained tachyarrhythmia in repaired tetralogy of Fallot

AIMS: Repaired tetralogy of Fallot (rtoF) patients are at risk of atrial or ventricular tachyarrhythmia and sudden cardiac death. Risk stratification for arrhythmia remains difficult. We investigated whether cardiac anatomy and function predict arrhythmia. METHODS: One-hundred-and-fifty-four adults with rtoF, median age 30.8 (21.9–40.2) years, were studied with a standardised protocol including cardiovascular magnetic resonance (CMR) and prospectively followed up over median 5.6 (4.6–7.0) years for the pre-specified endpoints of new-onset atrial or ventricular tachyarrhythmia (sustained ventricular tachycardia/ventricular fibrillation). RESULTS: Atrial tachyarrhythmia (n = 11) was predicted by maximal right atrial area indexed to body surface area (RAAi) on four-chamber cine-CMR (Hazard ratio 1.17, 95% Confidence Interval 1.07–1.28 per cm(2)/m(2); p = 0.0005, survival receiver operating curve; ROC analysis, area under curve; AUC 0.74 [0.66–0.81]; cut-off value 16 cm(2)/m(2)). Atrial arrhythmia-free survival was reduced in patients with RAAi ≥ 16 cm(2)/m(2) (logrank p = 0.0001). Right ventricular (RV) restrictive physiology on echocardiography (n = 38) related to higher RAAi (p = 0.02) and had similar RV dilatation compared with remaining patients. Ventricular arrhythmia (n = 9) was predicted by CMR RV outflow tract (RVOT) akinetic area length (Hazard ratio 1.05, 95% Confidence Interval 1.01–1.09 per mm; p = 0.003, survival ROC analysis, AUC 0.77 [0.83–0.61]; cut-off value 30 mm) and decreased RV ejection fraction (Hazard ratio 0.93, 95% Confidence Interval 0.87–0.99 per %; p = 0.03). Ventricular arrhythmia-free survival was reduced in patients with RVOT akinetic region length > 30 mm (logrank p = 0.02). CONCLUSION: RAAi predicts atrial arrhythmia and RVOT akinetic region length predicts ventricular arrhythmia in late follow-up of rtoF. These are simple, feasible measurements for inclusion in serial surveillance and risk stratification of rtoF patients

Transition to adult care of young people with congenital heart disease: Impact of a service on knowledge and self-care skills, and correlates of a successful transition

Aims Less than one-third of adolescents with congenital heart disease (CHD) successfully transition to adult care, missing out on education of their cardiac condition, and risking loss to follow-up. We assessed the efficacy of our transition clinic on patient education and empowerment and identified correlates of successful transition. Methods and results Overall, 592 patients were seen at least once in our transition service between 2015 and 2022 (age 15.2 ± 1.8 years, 47.5% female). Most adolescents (53%) had moderate CHD, followed by simple (27.9%) and severe (19.1%) CHD. Learning disability (LD) was present in 18.9% and physical disability (PD) in 4.7%. In patients without LD, knowledge of their cardiac condition improved significantly from the first to the second visit (naming their condition: from 20 to 52.3%, P 0.05). Treatment adherence and management involvement, self-reported anxiety, and dental care awareness did not change over time. Successful transition (attendance of ≥ 2 clinics) was achieved in 49.3%. Younger age at the first visit, simpler CHD, and absence of PD were associated with successful transition. Conclusion A transition service positively impacts on patient education and empowerment in most CHD adolescents transitioning to adult care. Strategies to promote a tailored support for patients with LD should be sought, and earlier engagement should be encouraged to minimize follow-up losses

Quantification of biventricular myocardial function using cardiac magnetic resonance feature tracking, endocardial border delineation and echocardiographic speckle tracking in patients with repaired tetralogy of fallot and healthy controls

Background: Parameters of myocardial deformation have been suggested to be superior to conventional measures of ventricular function in patients with tetralogy of Fallot (ToF), but have required non-routine, tagged cardiovascular magnetic resonance (CMR) techniques. We assessed biventricular myocardial function using CMR cine-based feature racking (FT) and compared it to speckle tracking echocardiography (STE) and to simple endocardial border delineation (EBD). In addition, the relation between parameters of myocardial deformation and clinical parameters was assessed. Methods: Overall, 28 consecutive adult patients with repaired ToF (age 40.4 ± 13.3 years) underwent standard steadystate- free precession sequence CMR, echocardiography, and cardiopulmonary exercise testing. In addition, 25 healthy subjects served as controls. Myocardial deformation was assessed by CMR based FT (TomTec Diogenes software), CMR based EBD (using custom written software) and STE (TomTec Cardiac Performance Analysis software). Results: Feature tracking was feasible in all subjects. A close agreement was found between measures of global left (LV) and right ventricular (RV) global strain. Interobserver agreement for FT and STE was similar for longitudinal LV global strain, but FT showed better inter-observer reproducibility than STE for circumferential or radial LV and longitudinal RV global strain. Reproducibility of regional strain on FT was, however, poor. The relative systolic length change of the endocardial border measured by EBD yielded similar results to FT global strain. Clinically, biventricular longitudinal strain on FT was reduced compared to controls (P<0.0001) and was related to the number of previous cardiac operations. In addition, FT derived RV strain was related to exercise capacity and VE/VCO2-slope. Conclusions: Although neither the inter-study reproducibility nor accuracy of FT software were investigated, and its interobserver reproducibility for regional strain calculation was poor, its calculations of global systolic strain showed similar or better inter-oberver reproducibility than those by STE, and could be applied across RV image regions inaccessible to echo. ‘Global strain’ calculated by EBD gave similar results to FT. Measurements made using FT related to exercise tolerance in ToF patients suggesting that the approach could have clinical relevance and deserves further study