Adults undergoing catheterization at pediatric hospitals encountered AE less frequently than children did. The congenital heart disease adjustment for risk method for adults with congenital heart disease is a new tool for assessing procedural risk in adult patients.

BackgroundCongenital heart disease (CHD) is associated with risk factors for ischemic stroke including cardiac arrhythmias and heart failure. However, few long‐term follow‐up data exist on ischemic stroke risk and associated mortality in adults with CHD.Methods and ResultsUsing Danish nationwide registries, we identified individuals aged ≥18 years diagnosed with CHD, at any age, from 1963 to 2017 and a sex and birth year‐matched (1:10) general population comparison cohort. We computed risks, as well as sex and birth year‐adjusted hazard ratios (aHRs) for ischemic stroke and 30‐day post‐stroke mortality in CHD adults compared with the general population. Analyses were stratified according to age <60 years (young) and ≥60 years (older). We identified 16 836 adults with CHD. The risk of ischemic stroke at age 60 years was 7.4% in the CHD cohort and 2.9% in the general population cohort. The adjusted hazard ratios for ischemic stroke compared with the general population was 3.8 (95% CI: 3.3–4.3) in young CHD adults and 1.6 (95% CI: 1.4–1.9) in older CHD adults. The adjusted hazard ratios for post‐stroke mortality compared with the general population was 2.3 (95% CI: 1.2–4.4) in young CHD adults and 1.3 (95% CI: 0.9–1.9) in older CHD adults.ConclusionsBoth younger and older CHD adults have an increased risk of ischemic stroke and by 60 years of age 7.4% of CHD adults will have had an ischemic stroke. Post‐stroke mortality was also increased in CHD adults compared with the general population.

Tissue Doppler imaging (TDI) measurements have been demonstrated to correlate with central venous pressure (CVP) in adults. We hypothesize that TDI measurements also correlate with CVP in children after cardiac surgery. Patients younger than 10 years with invasive CVP monitoring after cardiac surgery were studied. Annular tissue and atrioventricular inflow velocities were measured daily during CVP monitoring. Tissue Doppler imaging measurements were compared with CVP by univariate analyses. Subgroup analyses were performed for univentricular versus biventricular hearts. p values < or = 0.05 were considered significant. Fifty studies were performed on 28 subjects; 20 studies were performed on patients with univentricular physiology; and 30 studies were performed on patients with biventricular physiology. For all subjects, CVP correlated significantly with the right ventricular TDI measurements of e' velocity (r = -0.43), a' velocity (r = 0.35), e'/a' ratio (r = -0.42), and E/e' ratio (r = 0.44). For biventricular patients, e' velocity (r = -0.35), e'/a' ratio (r = -0.38), and E/e' ratio (r = 0.58) correlated significantly. For univentricular patients, E velocity (r = -0.62), e' velocity (r = -0.50), and a' (r = 0.61) velocity correlated significantly. Tissue Doppler imaging measurements of the right heart significantly correlated with invasively measured CVP in pediatric subjects. Different measures may correlate more strongly in those with biventricular versus univentricular physiology. With additional study, TDI may prove a valid tool for noninvasively assessing CVP in children with both bi- and univentricular physiology.

Tetralogy of Fallot (ToF) can be associated with a wide range of extracardiac anomalies, with an underlying etiology identified in approximately 10% of cases. Individuals affected with Myhre syndrome due to recurrent SMAD4 mutations frequently have cardiovascular anomalies, including congenital heart defects. In addition to two patients in the literature with ToF, we describe five additional individuals with Myhre syndrome and classic ToF, ToF with pulmonary atresia and multiple aorto‐pulmonary collaterals, and ToF with absent pulmonary valve. Aorta hypoplasia was documented in one patient and suspected in another two. In half of these individuals, postoperative cardiac dysfunction was thought to be more severe than classic postoperative ToF repair. There may be an increase in right ventricular pressure, and right ventricular dysfunction due to free pulmonic regurgitation. Noncardiac developmental abnormalities in our series and the literature, including corectopia, heterochromia iridis, and congenital miosis suggest an underlying defect of neural crest cell migration in Myhre syndrome. We advise clinicians that Myhre syndrome should be considered in the genetic evaluation of a child with ToF, short stature, unusual facial features, and developmental delay, as these children may be at risk for increased postoperative morbidity. Additional research is needed to investigate the hypothesis that postoperative hemodynamics in these patients may be consistent with restrictive myocardial physiology.

Combining surgical unifocalization procedures with subsequent early and intensive catheter-based pulmonary artery rehabilitation may improve vascular growth, ultimately rendering many patients suitable for fenestrated VSD closure. Risk stratification, including intraoperative exit angiography, is essential to determine the need for early transcatheter interventions.

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