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Cardiovascular diseases (CVDs), principally ischemic heart disease (IHD) and stroke, are the leading cause of global mortality and a major contributor to disability. This paper reviews the magnitude of total CVD burden, including 13 underlying causes of cardiovascular death and 9 related risk factors, using estimates from the Global Burden of Disease (GBD) Study 2019. GBD, an ongoing multinational collaboration to provide comparable and consistent estimates of population health over time, used all available population-level data sources on incidence, prevalence, case fatality, mortality, and health risks to produce estimates for 204 countries and territories from 1990 to 2019. Prevalent cases of total CVD nearly doubled from 271 million (95% uncertainty interval [UI]: 257 to 285 million) in 1990 to 523 million (95% UI: 497 to 550 million) in 2019, and the number of CVD deaths steadily increased from 12.1 million (95% UI:11.4 to 12.6 million) in 1990, reaching 18.6 million (95% UI: 17.1 to 19.7 million) in 2019. The global trends for disability-adjusted life years (DALYs) and years of life lost also increased significantly, and years lived with disability doubled from 17.7 million (95% UI: 12.9 to 22.5 million) to 34.4 million (95% UI:24.9 to 43.6 million) over that period. The total number of DALYs due to IHD has risen steadily since 1990, reaching 182 million (95% UI: 170 to 194 million) DALYs, 9.14 million (95% UI: 8.40 to 9.74 million) deaths in the year 2019, and 197 million (95% UI: 178 to 220 million) prevalent cases of IHD in 2019. The total number of DALYs due to stroke has risen steadily since 1990, reaching 143 million (95% UI: 133 to 153 million) DALYs, 6.55 million (95% UI: 6.00 to 7.02 million) deaths in the year 2019, and 101 million (95% UI: 93.2 to 111 million) prevalent cases of stroke in 2019. Cardiovascular diseases remain the leading cause of disease burden in the world. CVD burden continues its decades-long rise for almost all countries outside high-income countries, and alarmingly, the age-standardized rate of CVD has begun to rise in some locations where it was previously declining in high-income countries. There is an urgent need to focus on implementing existing cost-effective policies and interventions if the world is to meet the targets for Sustainable Development Goal 3 and achieve a 30% reduction in premature mortality due to noncommunicable diseases., Central Illustration, Highlights • The burden of CVD, in number of DALYs and deaths, continues to increase globally. • CVD burden attributable to modifiable risk factors continues to increase globally. • Countries should invest in existing cost-effective public health programs and clinical interventions to target modifiable risks, promote healthy aging across the lifespan, and reduce disability and premature death due to CVD.

Background: Children with cardiomyopathy (CM) are at risk of sudden cardiac death (SCD), but the incidence and risk factors for this outcome are not clear.Objectives: This study sought to d...

Objectives The study sought to evaluate the late outcomes of a policy of transatrial repair delayed beyond the neonatal period. Background Long-term outcomes of transatrial repair of tetralogy of Fallot are unknown. Methods The records of 675 consecutive patients undergoing a transatrial repair of tetralogy of Fallot between 1980 and 2005 were reviewed, their follow-up updated and survival confirmed from national death registries. One-third (220 of 675) had undergone previous palliation. Median age at repair was 2 years in the first 8 years, and 1 year from 1988 onward. A transannular incision was performed in 75% of cases and autologous pericardium was the material used to patch this incision in 92% of cases. Results There were 7 hospital deaths (1%). Eight patients died during follow-up (2 sudden unexpected and 6 noncardiac deaths). Mean follow-up was 11.7 ± 6.3 years. Twenty-five years' survival was 97% (95% confidence interval [CI]: 95% to 98%). Twenty-five years' freedom from implantation of a valved conduit was 84.6% (95% CI: 77.8% to 89.5%). By multivariable analysis, prior palliation and younger age at repair were predictive of implantation of a valved conduit (hazard ratio: 2.4, 95% CI: 1.3 to 4.6, p = 0.008; hazard ratio: 0.70, 95% CI: 0.50 to 0.96, p = 0.03, respectively). Conclusions During long-term follow-up, transatrial repair of tetralogy of Fallot was associated with a minimal risk of sudden death and low rate of reintervention for right ventricular dilation and residual outflow tract obstruction.

Objectives The study sought to identify factors associated with increased risk of thrombosis after Fontan. Background The Fontan procedure is the culmination of staged palliation for patients with univentricular physiology. Thrombosis is an important complication after this procedure. Methods An international multicenter randomized controlled trial of acetylsalicylic acid versus warfarin for thromboprophylaxis after the Fontan procedure was conducted in 111 patients, and did not show a significant difference regarding thrombotic complications. We performed a secondary analysis of this previously published manuscript to identify factors associated with thrombosis in this population. Standardized prospective data collection included independent adjudication of all events. Results At 2.5 years after randomization, time-related freedom from thrombosis was 69% (all venous, no arterial events), with 28% of thrombosis presenting with clinical signs or events. Hazard of thrombosis was highest immediately after Fontan with a gradual increase in risk during late follow-up. In multivariable models, factors associated with higher risk of thrombosis were pulmonary atresia with intact ventricular septum (hazard ratio [HR]: 3.64, 95% confidence interval [CI]: 1.04 to 12.70, p = 0.04), pulmonary artery distortion (HR: 2.35, 95% CI: 0.96 to 5.73, p = 0.06), lower pre-operative unconjugated bilirubin (HR: 0.84 μmol/l, 95% CI: 0.72 to 0.99, p = 0.04), use of central venous lines for >10 days or until hospital discharge (HR: 17.8, 95% CI: 3.97 to 79.30, p Conclusions More favorable thromboprophylaxis strategies are needed in light of the difficulties in controlling warfarin therapy and the high prevalence of thrombosis in this population (International Multi Centre Randomized Clinical Trial of Anticoagulation in Children Following Fontan Procedures; NCT00182104)

Children with cardiomyopathy (CM) are at risk of sudden cardiac death (SCD), but the incidence and risk factors for this outcome are not clear.This study sought to determine the incidence and risk factors for SCD in children with varying CM phenotypes from a long-term population-based study of childhood CM.The NACCS (National Australian Childhood Cardiomyopathy Study) is an ongoing longitudinal cohort study including all children in Australia with primary CM who were diagnosed between January 1, 1987, and December 31, 1996, and were10 years of age. The cumulative incidence and risk factors for SCD within individual CM phenotypes were explored using survival analysis.Of 289 eligible patients, 16 (5.5%) experienced SCD over a median follow-up of 11.9 years (interquartile range: 1.7 to 15.4). The risk of SCD varied according to CM phenotype (p=0.007). The cumulative incidence of SCD at 15 years was 5% for dilated cardiomyopathy (DCM), 6% for hypertrophic cardiomyopathy (HCM), 12% for restrictive cardiomyopathy, and 23% for left ventricular (LV) noncompaction. Older age at diagnosis, positive family history of CM, and severity of LV dysfunction were related to increased risk of SCD in patients with DCM, and a higher posterior wall thickness Z-score was the sole risk factor identified for patients with HCM.Predictors of SCD include CM phenotype, family history of CM (DCM), severity of systolic dysfunction (DCM), and extent of LV hypertrophy (HCM). Continuing follow-up of this cohort into adulthood is likely to reveal an ongoing risk of SCD.

The study sought to evaluate the late outcomes of a policy of transatrial repair delayed beyond the neonatal period.Long-term outcomes of transatrial repair of tetralogy of Fallot are unknown.The records of 675 consecutive patients undergoing a transatrial repair of tetralogy of Fallot between 1980 and 2005 were reviewed, their follow-up updated and survival confirmed from national death registries. One-third (220 of 675) had undergone previous palliation. Median age at repair was 2 years in the first 8 years, and 1 year from 1988 onward. A transannular incision was performed in 75% of cases and autologous pericardium was the material used to patch this incision in 92% of cases.There were 7 hospital deaths (1%). Eight patients died during follow-up (2 sudden unexpected and 6 noncardiac deaths). Mean follow-up was 11.7 ± 6.3 years. Twenty-five years' survival was 97% (95% confidence interval [CI]: 95% to 98%). Twenty-five years' freedom from implantation of a valved conduit was 84.6% (95% CI: 77.8% to 89.5%). By multivariable analysis, prior palliation and younger age at repair were predictive of implantation of a valved conduit (hazard ratio: 2.4, 95% CI: 1.3 to 4.6, p = 0.008; hazard ratio: 0.70, 95% CI: 0.50 to 0.96, p = 0.03, respectively).During long-term follow-up, transatrial repair of tetralogy of Fallot was associated with a minimal risk of sudden death and low rate of reintervention for right ventricular dilation and residual outflow tract obstruction.

Objetives. The present study was designed to estimate the influence of different-shaped adjacent surfaces on regurgitant jets as assessed by color Doppler imaging and laser-Illuminated dye optical visualisation,Background. Because color Doppler techniques provide real-time two-dimentional imaging of flow, the evaluation of valvular regurgitation by analysis of variance-encoded regurgitant jets by this method has been widely used in clinical studies. However, recent studies have demonstrated that color Doppler jet sizes are affected not only by several hemodynamk factors and instrument settings but also by the interaction between jets and adjacent wall surfaces. In clinical conditions, jets may interact with adjacent walls of variable shapes that might have different effects on the jet size.Methods. An in vitro model was constructed consisting of a rigid, optically clear receiving chamber that had no outlet resistance and had a pulsatile pump ejecting through 1.5, 2.3 and 3.1 mm2inflow orifices into the chamber. The surfaces were flat or smoothly and equally curved, convex and concave aluminum positioned at 0,2 and 4 mm from and to the side of the inflow orifices. The pump was run with stroke volumes from 0.5 to 3.0 ml and with a pulse frequency of 70 beats/min, The echocardiographic and laser beams were aimed at the inflow orifice imaging jets perpendicular to the surfaces (vertical view) through the central plane of the jet flows. Maximal jet areas were measured by both color Doppler techniques and laser-illuminated dye visualization.Results. Color Doppler study showed fair correlation between the jet areas and the stroke volumes (r = 0.83 to 0.99), but the jet sizes under different surface conditions were variable. All the surface jet areas at a jet-surface distance of 0 and 2 mm were smaller than free jet areas at the same stroke volume for both flat and convex surfaces (p < 0.001). Flow constraint by the concave surface resulted in the smallest jet areas (p < 0.001). The color Doppler jet areas on the curved surfaces were significantly smaller than the laser-illuminated dye visualization jet areas (p < 0.01 to 0.0001). However, at intermediate jet-surface distances (4 mm and sometimes 2 mm with higher velocity flows), jet interaction with the flat and especially with the convex surface resulted in larger jets. This effect was most pronounced on dye fluorescence studies because flow around these jets consisted mainly of low velocity vortical events with only partial surface adherence and these low velocity swirling flows were not well imaged by color Doppler technique.Conclusions. Our study suggests that the different-shaped adjacent surfaces with different degrees of flow alterations resulted in variable decreases in jet size and that color Doppler imaging could not encode and image the angled and low velocity swirling events well when jets flowed along the curved surfaces. These effects need to be taken into account when interpreting color Doppler images.

Objectives The purpose of this study was to compare the safety and efficacy of acetylsalicylic acid (ASA) and warfarin for thromboprophylaxis after the Fontan procedure. Background Fontan surgery is the definitive palliation for children with single-ventricle physiology. Thrombosis is an important complication; the optimal thromboprophylaxis strategy has not been determined. Methods We performed a multicenter international randomized trial of primary prophylactic anticoagulation after Fontan surgery. Patients were randomized to receive for 2 years either ASA (5 mg/kg/day, no heparin phase) or warfarin (started within 24 h of heparin lead-in; target international normalized ratio: 2.0 to 3.0). Primary endpoint (intention to treat) was thrombosis, intracardiac or embolic (all events adjudicated). At 3 months and 2 years after the Fontan procedure, transthoracic and transesophageal echocardiograms were obtained as routine surveillance. Major bleeding and death were primary adverse outcomes. Results A total of 111 eligible patients were randomized (57 to ASA, 54 to heparin/warfarin). Baseline characteristics for each group were similar. There were 2 deaths unrelated to thrombosis or bleeding. There were 13 thromboses in the heparin/warfarin group (3 clinical, 10 routine echo) and 12 thromboses in the ASA group (4 clinical, 8 routine echo). Overall freedom from thrombosis 2 years after Fontan surgery was 19%, despite thrombosis prophylaxis. Cumulative risk of thrombosis was persistent but varying and similar for both groups (p = 0.45). Major bleeding occurred in 1 patient in each group. Conclusions There was no significant difference between ASA and heparin/warfarin as primary thromboprophylaxis in the first 2 years after Fontan surgery. The thrombosis rate was suboptimal for both regimens, suggesting alternative approaches should be considered. (International Multi Centre Randomized Clinical Trial Of Anticoagulation In Children Following Fontan Procedures; NCT00182104)

The pre- and postoperative growth patterns or 52 otherwise normal infants undergoing primary surgical closure of a large ventricular septal defect before 7 months of age were reviewed. Serial measurements of weight, length and head circumference were compiled for all patients preoperatively and in 46 long-term survivors and were expressed as Z scores (in standard deviations from the mean for age and gender).By the time of surgery at a mean age of 0.33 year, the mean weight, length and head circumference Z scores of all 52 infants were −2.9, −0.9 and −0.6, respectively, and were all significantly below normal (p < 0.001). At a mean age of 5.7 years, the mean weight, length and head circumference Z scores of 35 patients of normal birth weight were normal or varied only marginally from those of the reference population (−0.4, −0.1 and +0.5, respectively: p < 0.02, p > 0.05 and p = 0.008, respectively) and did not differ significantly in any variable from those of 44 normal siblings. However, among 11 infants with a low birth weight, all three variables remained abnormal at long-term follow-up when compared with the reference population (−1.7, −1.7 and −0.9, respectively; p < 0.001 for each) and 22 normal siblings (p < 0.008). The difference between pre- and postoperative Z scores was highly significant (p ≤ 0.004) for all three variables in the normal birth weight group, but only a significant difference in weight Z scores emerged in the low birth weight group (p < 0.001). Catch-up growth in most cases was complete within 6 to 12 months after operation.Early surgical repair of a large ventricular septal defect results in near normal long-term growth in the majority of patients. Residual growth disturbances are usually due to extracardiac factors. The present results support a policy of early surgical intervention in infants with a large ventricular septal defect.

An accurate but simple and noninvasive method for quantifying flow across a ventricular septal defect has yet to be implemented for routine clinical use. A region of flow convergence is commonly imaged by Doppler color flow mapping on the left septal surface of the ventricular septal defect, appearing as a narrowed region of laminar flow with aliased flow velocities entering the orifice. If the first aliasing region represents a hemispheric isovelocity boundary of a surface of flow convergence and all flow at this surface crosses the ventricular septal defect, the flow through the defect can be estimated by using the radius (R), measured from the first alias to the orifice, and the Nyquist limit (NL) velocity (the now velocity at the first alias). Doppler color flow imaging was performed in 18 children with a single membranous ventricular septal defect undergoing cardiac catheterization at a mean age of 29.8 months (Group I). Indexes of maximal flow rate across the defect were developed from either the radius or the area, obtained by planimetry, of the first alias, based on Doppler color flow images. All indexes were corrected for body surface area and compared with shunt flow (Qp — Qs) and pulmonary to systemic flow ratio (Qp/Qs) determined at cardiac catheterization. Doppler color flow indexes derived from images of flow convergence in both the long-axis (n = 15) and oblique four-chamber (n = 10) views correlated closely with Qp/Qs (r = 0.71 to 0.92) and Qp — Qs (r = 0.69 to 0.97). In another eight patients (Group II), the same indexes correlated well with results of Qp — Qs determined by planimetry of pulsed Doppler curves and by areas measured with use of two-dimensional echocardiography (r = 0.94). These results suggest that simple and clinically useful quantitative indexes of shunting across a ventricular septal defect can be derived from the flow convergence region proximal to the defect.

Twenty-three children and young persons with a congenital long QT syndrome were identified; the median age at the time of referral was 10 years (range 4 days to 19 years) and 14 patients (61%) had a family history of the syndrome. Among the 19 patients with symptoms, the initial symptom was syncope in 13 (69%), aborted sudden death in 5 (26%) and near drowning in 1 (5%). There were three deaths during a combined follow-up period of 67 patient-years (average annual mortality rate 4.5%). Patients who did not respond to therapy with a beta-adrenergic blocker and those who died were significantly younger than the remaining patients at the time of diagnosis (p ≤ 0.05 for both).Analysis of 44 treadmill exercise tests performed by 16 patients revealed significant prolongation of the median corrected QT (QTc) interval in response to exercise, with maximal prolongation present after 2 min of recovery (median QTc interval 0.52 s versus a baseline value of 0.47 s, p < 0.001). Characteristic changes in T wave configuration were noted in 8 of 15 patients on at least one occasion during ambulatory Holter electrocardiographic monitoring, including T wave alternation in two patients, both of whom died shortly afterward.It is suggested that the congenital long QT syndrome is associated with a significant mortality rate in childhood despite the use of conventional therapy in symptomatic patients. Ambulatory Holter monitoring and treadmill exercise testing may be helpful, both in confirming the diagnosis of a long QT syndrome and in monitoring the adequacy of treatment. Children presenting with sustained ventricular arrhythmias appear to merit early consideration of dual therapy (beta-blockade plus cardiac pacemaker or left cardiac sympathectomy).

Between January 1978 and June 1993, 32 patients presented for management of double outlet RV with non-committed VSD. 29 patients had definitive palliation or repair with 3 early and 1 late deaths (hospital mortality: 10.4%). The VSD type was muscular in 4, inlet in 12 and perimembranous but distant from both great arteries in 16 patients. Complicating anomalies with a major influence on management were present in 23 children (72%). These included aortic arch interruption (3), coarctation of the aorta (4), pulmonary stenosis (11), pulmonary atresia (2). multiple VSOs (3), AV discordance (2), left atrial isomerism (3), unbalanced ventricles (8) and criss-cross AV connection (2) with 11 children having more than one of these lesions. Initial palliation was performed in 20 patients and consisted of pulmonary artery banding in 8 (with repair of an interrupted aortic arch in 2), systemic-pulmonary artery shunt in 9 and repair of coarctation of the aorta in 3. Of these, 17 subsequently had definitive surgery: Fontan procedure in 9 and biventricular repair in 8 (arterial switch in one, intraventricular baffle in 7). 3 patients died following attempted biventricular repair. 12 patients underwent repair as the initial procedure: Fontan procedure in 2 and biventricular repair in 10 (arterial switch in 3 and intraventricular baffle in 7) with no deaths. Thus, of the 29 patients who had definitive surgery, 11 had Fontan procedures and 18 biventricular repair with no death in the Fontan group and 3 early deaths in the biventricular repair group (not statistically significant by Fischer's exact test, p = 0.27). Only 2 patients have required reoperations, both for residual VSDs. 9 of the Fontan procedures were undertaken in patients who had complex anatomy (e.g. AV discordance, unbalanced ventricles, multiple VSDs, atrial isomerism or pulmonary atresia), whereas 14 children of the biventricular repair group had non-complex anatomy (p l 0.005). Surgery for double outlet RV with non-committed VSD needs to be tailored to individual patients, but biventricular repair was achieved in a substantial majority of patients with non-complex anatomy with relatively low risk and a low incidence of reoperations.