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Background Adults with repaired Tetralogy of Fallot (rTOF) comprise one of the largest cohorts among adults with congenital heart disease (ACHD). These patients have a higher burden of atrial arrhythmias (AA), leading to increased adverse events, including stroke and transient ischemic attack (TIA). However, the data on factors associated with stroke/TIA in rTOF are limited, and classic risk factors may not apply. We studied event rates and associated factors for thromboembolism in a rTOF cohort. Methods Retrospective cohort study of all adult patients age >18 years with rTOF followed at a single ACHD tertiary care center. AA of interest were atrial fibrillation (AF) and atrial flutter (AFL). Results Data from 260 patients were identified, mean age 37.6 SD 13.3 years, followed over 5108 patient‐years (mean 16.6 SD 8.2 years). 43 patients had AF and/or AFL, and 30 patients had thromboembolic events, of which 19 patients had stroke/TIA. The event rate for any thromboembolism was 3.39 per 100 patient‐years follow‐up in patients with AA, compared to 1.80 in patients without (P = .07). In univariate analysis, older age and diabetes were associated with thromboembolic events. In multivariate analysis, only older age was associated with thromboembolic events. Conclusions In our relatively young cohort of adults with rTOF, there was a high prevalence of AA, associated with nearly double the rate of thromboembolic events compared to patients without AA. Older age alone is independently associated with thromboembolic events. Further studies into assessment of silent AA are required, and routine assessments should be considered at an earlier age., There is a high prevalence of atrial arrhythmias in our relatively young cohort of adults with repaired Tetralogy of Fallot. Atrial arrhythmias are associated with twofold increase in risk of thromboembolic events. Older age was identified as a key risk factor, and traditional risk factors were not applicable.

Patients with heritable aortic disease (HAD) have an increased risk of ventricular arrhythmias and sudden cardiac death. Although mitral valve prolapse is common in HAD, mitral annulus disjunction (MAD) has only recently been described in these patients. This under-recognized condition may be a contributing factor to otherwise unexplained ventricular arrhythmias and sudden cardiac death in patients with HAD. This case series describes 3 patients in an adult HAD clinic who have concomitant mitral valve prolapse, MAD, and malignant arrhythmias. These cases may represent a unique disease entity or overlap syndrome, and they introduce MAD as a potential arrhythmogenic risk marker in HAD.

Background: Atrial arrhythmias (AA) are common in adults with congenital heart disease (ACHD). Although Intra-atrial Reentrant Tachycardia (IART) is well described in ACHD, Atrial Fibrillation (AF) is uncommon, but increasingly recognized. Patients with Transposition of the Great Arteries (TGA) and congenitally corrected-TGA (CC-TGA) have a high burden of AA at a relatively young age. However, long-term data of AA and associated thromboembolic risk are lacking in these patients. The prevalence, associated clinical factors, and complications of AA in a longitudinal TGA cohort was studied. Methods: A retrospective cohort study of all TGA patients from a single tertiary care centre was conducted. Data regarding documented atrial arrhythmias, thromboembolic events, and factors associated with thromboembolism were extracted and analyzed. Mean values and standard deviations were calculated for normally distributed continuous variables. When frequencies and means were compared, the chi-squared test and student t-test were used, respectively. Results: One-hundred twenty-five patients with TGA (76 TGA, 49 CC-TGA) were followed for a mean of 20.8 ± 13.2 years. AF was confirmed in 20% (n = 25) and there were 5 (20%) thromboembolic complications within the AF population. AF was associated with an annual thromboembolic event rate of 2.7%/year (stroke/transient ischemic attack 1.7%, systemic embolism 1.0%). Conclusion: AF is relatively common in the TGA ACHD population in long-term follow up. Although annual risk of thromboembolism is low in this young group of patients, life-time cumulative risk is potentially high. TGA patients should be screened actively for AF and appropriate anticoagulation therapy initiated. It is unclear if established risk prediction scores in other non-valvular AF populations may be applicable to this cohort.

Background. Hepatic fibrosis is a potential complication following Fontan surgery and heralds long-term risk for cirrhosis. Transient elastography (TE) is a rapid, noninvasive method to assess liver fibrosis by measuring liver stiffness.Objectives. To compare liver stiffness and liver biochemistries in pediatric Fontan patients with age- and sex-matched controls and to determine patients’ acceptance of TE.Methods. Patients were recruited from British Columbia Children’s Hospital. Twenty-two Fontan patients (15 males) were identified. Demographic information and cardiac data were collected. TE was measured using size-appropriate probes.Results. The median age of the Fontan cohort was 13.7 (5.9–16.8) years. Time from Fontan surgery to TE was 9.6 (1.0–12.9) years. The median Fontan circuit pressure was 13 (11–14) mmHg. TE values were higher in Fontan patients versus controls (18.6 versus 4.7 kPa,p<0.001). There was no association between TE values and patient age (r=0.41,p=0.058), time since Fontan surgery (r=0.40,p=0.062), or median Fontan circuit pressure (CVP) (r=0.35,p=0.111). Patients found TE to be nonpainful, convenient, and safe.Conclusions. TE is feasible to assess liver stiffness in children following Fontan surgery. Pediatric Fontan patients have markedly elevated liver stiffness values. TE may have important utility in liver care follow-up of pediatric Fontan patients.

A child with complex congenital heart disease in 2008 is very likely to survive a series of surgical and medical interventions, and confront an array of medical and psychosocial stressors that are presently poorly understood. As approaches to medical problems change, careful assessment of those results is essential, and the initial work of the multicentre Pediatric Heart Network is a huge step in the right direction, setting the stage for proper controlled trials of therapies. Major complications, notably ventricular failure, rhythm problems and thromboembolism, will affect nearly one-quarter of survivors, necessitating further interventions. Appropriate educational and psychosocial support for these children and their families is the next challenge for all of us in the field of paediatrics. How ironic would it be to have invested so much in early survival, only to allow the child to fail in life itself.

In kwashiorkor the heart is clinically and radiologically small. This study utilises echocardiography, a tool not previously used in this disease, to show that this is due to decreased muscle mass.

A trial was conducted using sequential analysis by pairs to compare the efficacy of corticosteroids and salicylates in the treatment of acute rheumatic carditis. The results show a significantly favourable effect of steroid treatment both in clinical response and in reduction of the erythrocyte sedimentation rate. In addition, patients receiving steroids usually had a shorter hospital stay. The use of steroids in acute rheumatic fever with carditis is recommended.