Background: Databases for Congenital Heart Disease (CHD) are effective in delivering accessible datasets ready for statistical inference. Data collection hitherto has, however, been labour and time intensive and has required substantial financial support to ensure sustainability. We propose here creation and piloting of a semi-automated technique for data extraction from clinic letters to populate a clinical database. Methods: PDF formatted clinic letters stored in a local folder, through a series of algorithms, underwent data extraction, preprocessing, and analysis. Specific patient information (diagnoses, diagnostic complexity, interventions, arrhythmia, medications, and demographic data) was processed into text files and structured data tables, used to populate a database. A specific data validation schema was predefined to verify and accommodate the information populating the database. Unsupervised learning in the form of a dimensionality reduction technique was used to project data into 2 dimensions and visualize their intrinsic structure in relation to the diagnosis, medication, intervention, and European Society of Cardiology classification lists of disease complexity. Ninety-three randomly selected letters were reviewed manually for accuracy. Results: There were 1409 consecutive outpatient clinic letters used to populate the Scottish Adult Congenital Cardiac Database. Mean patient age was 35.4 years; 47.6% female; with 698 (49.5%) having moderately complex, 369 (26.1%) greatly complex, and 284 (20.1%) mildly complex lesions. Individual diagnoses were successfully extracted in 96.95%, and demographic data were extracted in 100% of letters. Data extraction, database upload, data analysis and visualization took 571 seconds (9.51 minutes). Manual data extraction in the categories of diagnoses, intervention, and medications yielded accuracy of the computer algorithm in 94%, 93%, and 93%, respectively. Conclusions: Semiautomated data extraction from clinic letters into a database can be achieved successfully with a high degree of accuracy and efficiency.

OBJECTIVE:Myocardial fibrosis has been associated with poorer outcomes in tetralogy of Fallot, however only a handful of studies have assessed its significance in the current era. Our aim was to quantify the amount of late gadolinium enhancement in both the LV and RV in a contemporary cohort of adults with surgically repaired tetralogy of Fallot, and assess the relationship with adverse clinical outcomes.DESIGN:Single centre cohort study SETTING: National tertiary referral center Patients: One hundred fourteen patients with surgically repaired tetralogy of Fallot with median age 29.5 years (range 17.5-64.2). Prospective follow-up for mean 2.4 years (SD 1.29).INTERVENTIONS:Cardiovascular magnetic resonance was performed, and late gadolinium enhancement mass was estimated for the LV using the 5-SD remote myocardium method, and for the RV using a segmental scoring system. Cohort characterization was determined through the use of a computerized database.OUTCOME MEASURES:Survival analysis from time of scan to first adverse event, defined as an episode of atrial arrhythmia, sustained ventricular arrhythmia, hospitalization with heart failure, or implantable cardioverter-defibrillator insertion.RESULTS:Eleven patients experienced an adverse outcome in the follow-up period, although there were no deaths. LV late gadolinium enhancement was associated with adverse outcomes in a univariate model (P = .027). However, when adjusted for age at scan the significant variables included NYHA class (P = .006), peak oxygen uptake (P = .028), number of prior sternotomies (P = .044), and higher indexed RV and LV end diastolic volumes (P = .002 and P < .001), but not RV or LV late gadolinium enhancement.CONCLUSIONS:Formal quantification of late gadolinium enhancement is not currently as helpful in ascertaining prognosis compared to other, more easily assessed parameters in a contemporary cohort of tetralogy of Fallot survivors, however assessment particularly of the LV holds promise for the future.

The population of adults with congenital heart disease (CHD) now exceeds the population of children with CHD. The long-term management of these patients relies on sequential assessment of anatomy and physiology and integration with symptoms, all targeted toward decision making around intervention. The advances in technology have vastly improved our assessment of anatomy and function. However, while the assessment of chronic heart failure in acquired heart disease has been revolutionized by the proven utility of cardiac biomarkers, their use in adult CHD is still being assessed.

Background There is a well documented link between low socioeconomic status and mortality in acquired cardiac disease. The relationship between socioeconomic status and survival in adult congenital heart disease has not been explored. We sought to define this relationship in a national cohort of adult survivors of tetralogy of Fallot, the commonest cyanotic congenital cardiac lesion. Methods Prospective landmark analysis of a national cohort of adult survivors of tetralogy of Fallot followed up by the Scottish adult congenital cardiac service for Scotland (SACCS). Comparison of distribution of Carstairs deprivation scores with national data for Scotland. Kaplan Meier curves to define survival by Carstairs deprivation category within the cohort. Results We identified 382 adult survivors of tetralogy of Fallot throughout Scotland (225 male, 157 female). Transition to the adult service occurs at approximately age 16, andmean follow-up from this baseline was 19.1 +/- 12.6 years). We identified Carstairs deprivation categories (Depcats) from the 2001 census for the cohort as follows:5.6% Depcat 1, 10.6% Depcat 2, 21.0% Depcat 3, 23.9% Depcat 4, 18.0% Depcat 5, 11.4% Depcat 6, 9.5% Depcat 7. This distribution was not significantly different from the overall distribution of Depcat scores for the whole population of Scotland (.98) (Figure 1). Cumulative survival for the cohort was 0.83 at 40 years from baseline, and there were 16 deaths in total. There was no statistically significant difference between survival in each deprivation category by Mantel-Cox log rank test (p = 00.745) (Figure 2). Conclusions In contrast to other cardiovascular disease in Scotlandthere is no statistically significant association between mortality in adult survivors of tetralogy of Fallot and socioeconomic status. Although the reason for this remains unclear it may relate to the provision of a national service which provides regular follow-up and timely intervention for these complex patients.Comparison with less centralised services is warranted.

OBJECTIVE:The study objective was to evaluate long-term trends in morbidity and mortality in a national cohort of adult patients with a systemic right ventricle due to the atrial switch for transposition of the great arteries or congenitally corrected transposition of the great arteries. METHODS:We performed a retrospective cohort study from a baseline of 18 years, including life table and Kaplan-Meier analysis for probability of death/transplant, arrhythmia, surgical or percutaneous intervention, and permanent pacemaker insertion. RESULTS:A total of 97 adults with transposition of the great arteries-atrial switch (Mustard procedure in 80/Senning procedure in 17) and 32 adults with congenitally corrected transposition of the great arteries survived. The median ages at latest follow-up were 29 and 34 years, respectively. At 40 years of follow-up, freedom from death or transplant was 0.90 for those with transposition of the great arteries-atrial switch and 0.84 for those with congenitally corrected transposition of the great arteries (P = .833). Freedom from arrhythmia at 40 years of follow-up was 0.51 for those with transposition of the great arteries-atrial switch and 0.93 for those with congenitally corrected transposition of the great arteries (P = .007). Freedom from intervention at 40 years of follow-up was 0.33 for those with transposition of the great arteries-atrial switch after initial repair and 0.53 for those with congenitally corrected transposition of the great arteries (P = .938). Freedom from pacemaker insertion at 40 years of follow-up was 0.77 for those with transposition of the great arteries-atrial switch and 0.62 for those with congenitally corrected transposition of the great arteries (P = .161). CONCLUSIONS:Those patients who survive to adulthood with a systemic right ventricle experience low mortality and good functional status up to 40 years of age. However, there is a substantial burden of atrial tachyarrhythmia, and this occurs significantly earlier in those with transposition of the great arteries-atrial switch. Management of atrial tachyarrhythmia, along with systemic right ventricular dysfunction and systemic atrioventricular valve regurgitation, is likely to be the major challenge for this group of patients over the next decade.

Background and Objectives: Hypertriglyceridemia is perceived to promote atherosclerotic pathology, but its role in stroke has not been well defined. We aimed to assess the contribution of hypertriglyceridemia to residual vascular risk in patients with atherothrombotic stroke. Methods: The Tokyo Women’s Medical University Stroke Registry is an ongoing prospective, observational registry, in which 870 patients with acute ischemic stroke or TIA within 1 week of onset were consecutively enrolled and followed up for 1 year. Hypertriglyceridemia was defined as serum triglycerides levels of ≥150 mg/dL under fasting conditions. Significant stenosis of the cervicocephalic arteries was defined as having 50% or greater stenosis or occlusion. The primary outcome was major adverse cardiovascular events, including nonfatal stroke, nonfatal acute coronary syndrome, and vascular death. Results: Of 870 patients (mean age, 70.1 years; male, 60.9%), 217 (24.9%) had hypertriglyceridemia. High triglycerides levels were significantly associated with an increased prevalence of intracranial artery stenosis, particularly in the anterior circulation, rather than extracranial artery stenosis. Patients with hypertriglyceridemia had a greater risk of major adverse cardiovascular events than those without (annual rate, 20.9% vs. 9.7%; P<0.001), even after adjustment for potential confounders, including baseline low-density lipoprotein cholesterol and statin use (adjusted hazard ratio, 2.46; 95% confidence interval, 1.62-3.74). The higher risk of vascular events in hypertriglyceridemia versus non-hypertriglyceridemia patients was observed among patients with stroke of atherothrombotic origin (n=174; annual rate, 35.1% vs. 14.2%; P=0.001), those with significant intracranial artery stenosis (n=247; annual rate, 29.9% vs. 14.7%; P=0.006), and those with significant extracranial carotid artery stenosis (n=123; annual rate, 23.0% vs. 9.4%; P=0.042). In contrast, hypertriglyceridemia was not predictive of recurrent vascular events in patients with cardioembolic stroke (n=221; annual rate, 19.1% vs. 10.5%; P=0.18). Discussion: Hypertriglyceridemia is an important modifiable risk factor that drives residual vascular risk in patients with stroke of atherothrombotic origin, even while on statin therapy. Trial Registration Information: The Tokyo Women’s Medical University Stroke Registry is registered at UMIN000031913 (https://upload.umin.ac.jp). Classification of Evidence: This study provides Class I evidence that in patients with atherothrombotic stroke, hypertriglyceridemia is associated with an increased risk of major cardiovascular events.

Considerable improvements have been made in care and provision for patients with congenital heart disease in the United Kingdom. However, delayed presentation of adult patients with sequelae of known childhood cardiac defects reflects the current situation that there is no national registry of patients with congenital heart disease, and this "lost cohort" of patients is difficult to trace. Maintaining regular follow-up for selected patients with congenital heart disease can be challenging for a variety of reasons, but remains particularly important as emerging therapies and treatment strategies continue to alter management. Despite recent calls from a variety of organisations to establish a national registry of patients with congenital heart disease, progress has been slow. Faced with competition for resources, the costs of such a venture may be cited as a likely hurdle, but the potential advantages for patients and healthcare providers alike justify calls to integrate a registry as part of the ongoing reorganisation of congenital heart services in the United Kingdom.

Background—Phosphodiesterase type 5 (PDE5) inhibitors (eg, sildenafil) are a novel, orally active approach to the treatment of pulmonary arterial hypertension. The role of natriuretic peptides in the response to sildenafil was examined in mice lacking NPR-A, a guanylyl cyclase-linked natriuretic peptide receptor, in which pulmonary hypertension was induced by hypoxia. Methods and Results—Mice homozygous for NPR-A (NPR-A+/+) and null mutants (NPR-A−/−) were studied. Sildenafil inhibited the pressor response to acute hypoxia in the isolated perfused lungs of both genotypes. This effect was greater in the presence of atrial natriuretic peptide in the perfusate in NPR-A+/+ mice but not NPR-A−/− animals. In vivo, NPR-A mutants had higher basal right ventricular (RV) systolic pressures (RVSPs) than did NPR-A+/+ mice, and this was not affected by 3 weeks of treatment with sildenafil (25 mg · kg−1 · d−1). Both genotypes exhibited a rise in RVSP and RV weight with chronic hypoxia (10% O2 for 21 days); RVSP and RV weight were reduced by continuous sildenafil administration in NPR-A+/+ mice, but only RVSP showed evidence of a response to the drug in NPR-A−/− mice. The effect of sildenafil on hypoxia-induced pulmonary vascular muscularization and cyclic GMP levels was also blunted in NPR-A−/− mice. Conclusions—The natriuretic peptide pathway influences the response to PDE5 inhibition in hypoxia-induced pulmonary hypertension, particularly its effects on RV hypertrophy and vascular remodeling.