Right Ventricular Mass is Associated with Exercise Capacity in Adults with Repaired Tetralogy of Fallot.

The relationship between exercise capacity and right ventricular (RV) structure and function in adult repaired tetralogy of Fallot (TOF) is poorly understood. We therefore aimed to examine the relationships between cardiac MRI and cardiopulmonary exercise test variables in adult repaired TOF patients. In particular, we sought to determine the role of RV mass in determining exercise capacity. Eighty-two adult repaired TOF patients (age at evaluation 26 ± 10 years; mean age at repair 2.5 ± 2.8 years; 23.3 ± 7.9 years since repair; 53 males) (including nine patients with tetralogy-type pulmonary atresia with ventricular septal defect) were prospectively recruited to undergo cardiac MRI and cardiopulmonary exercise testing. As expected, these repaired TOF patients had RV dilatation (indexed RV end-diastolic volume: 153 ± 43.9 mL/m(2)), moderate-severe pulmonary regurgitation (pulmonary regurgitant fraction: 33 ± 14 %) and preserved left (LV ejection fraction: 59 ± 8 %) and RV systolic function (RV ejection fraction: 51 ± 7 %). Exercise capacity was near-normal (peak work: 88 ± 17 % predicted; peak oxygen consumption: 84 ± 17 % predicted). Peak work exhibited a significant positive correlation with RV mass in univariate analysis (r = 0.45, p < 0.001) and (independent of other cardiac MRI variables) in multivariate analyses. For each 10 g higher RV mass, peak work was 8 W higher. Peak work exhibits a significant positive correlation with RV mass, independent of other cardiac MRI variables. RV mass measured on cardiac MRI may provide a novel marker of clinical progress in adult patients with repaired TOF.

Brain volumetric, regional cortical thickness and radiographic findings in adults with cyanotic congenital heart disease.

BACKGROUND: Chronic cyanosis in adults with congenital heart disease (CHD) may cause structural brain changes that could contribute to impaired neurological functioning. The extent of these changes has not been adequately characterized. HYPOTHESIS: We hypothesized that adults with cyanotic CHD would have widespread changes including abnormal brain volumetric measures, decreased cortical thickness and an increased burden of small and large vessel ischemic changes. METHODS: Ten adults with chronic cyanosis from CHD (40 ± 4 years) and mean oxygen saturations of 82 ± 2% were investigated using quantitative MRI. Hematological and biochemical parameters were also assessed. All subjects were free from major physical or intellectual impairment. Brain volumetric results were compared with randomly selected age- and sex-matched controls from our database of normal subjects. RESULTS: Five of 10 cyanotic subjects had cortical lacunar infarcts. The white matter (WM) hyperintensity burden was also abnormally high (Scheltens Scale was 8 ± 2). Quantitative MRI revealed evidence of extensive generalized WM and gray matter (GM) volumetric loss; global GM volume was reduced in cyanosed subjects (630 ± 16 vs. 696 ± 14 mL in controls, p = 0.01) as was global WM volume (471 ± 10 vs. 564 ± 18 mL, p = 0.003). Ventricular cerebrospinal fluid volume was increased (35 ± 10 vs. 26 ± 5 mL, p = 0.002). There were widespread regions of local cortical thickness reduction observed across the brain. These changes included bilateral thickness reductions in the frontal lobe including the dorsolateral prefrontal cortex and precentral gyrus, the posterior parietal lobe and the middle temporal gyrus. Sub-cortical volume changes were observed in the caudate, putamen and in the thalamus (p ≤ 0.005 for all regions). Cortical GM volume negatively correlated with brain natriuretic peptide (R = - 0.89, p = 0.009), high sensitivity C-reactive protein (R = - 0.964, p < 0.0001) and asymmetric dimethylarginine (R = - 0.75, p = 0.026) but not with oxygen saturations, packed cell volume or viscosity. CONCLUSIONS: We present the first comprehensive analysis of brain structure in adults with chronic neurocyanosis due to congenital heart disease. We demonstrate clear evidence for marked macro- and microvascular injury. Cyanotic patients show global evidence for reduced brain volume as well as specific foci of cortical thickness reduction. The GM volume loss correlated with hsCRP, BNP and ADMA suggesting that inflammation, neurohormonal activation and endothelial dysfunction may have important roles in its pathogenesis.

Mechanisms of maintained exercise capacity in adults with repaired tetralogy of Fallot.

BACKGROUND: The mechanisms whereby cardiac output is augmented with exercise in adult repaired tetralogy of Fallot (TOF) are poorly characterised. METHODS: 16 repaired TOF patients (25 ± 7 years of age) and 8 age and sex matched controls (25 ± 4 years of age) underwent cardiopulmonary exercise testing and then real-time cardiac MRI (1.5 T) at rest and whilst exercising within the scanner, aiming for 30% heart rate reserve (Level 1) and 60% heart rate reserve (Level 2), using a custom-built MRI compatible foot pedal device. RESULTS: At rest, TOF patients had severely dilated RVs (indexed RV end-diastolic volume: 149 ± 37 mL/m(2)), moderate-severe PR (regurgitant fraction 35 ± 12%), normal RV fractional area change (FAC) (52 ± 7%) and very mildly impaired exercise capacity (83 ± 15% of predicted maximal work rate). Heart rate and RV FAC increased significantly in TOF patients (75 ± 10 vs 123 ± 17 beats per minute, p<0.001; 44 ± 7 vs 51 ± 10%, p=0.025), and similarly in control subjects (70 ± 11 vs 127 ± 12 beats per minute, p<0.001; 49 ± 7 vs 61 ± 9%, p=0.003), when rest was compared to Level 2. PR fraction decreased significantly but only modestly, from rest to Level 2 in TOF patients (37 ± 15 to 31 ± 15%, p=0.002). Pulmonary artery net forward flow was maintained and did not significantly increase from rest to Level 2 in TOF patients (70 ± 19 vs 69 ± 12 mL/beat, p=0.854) or controls (93 ± 9 vs 95 ± 21 mL/beat, p=0.648). CONCLUSIONS: During exercise in repaired TOF subjects with dilated RV and free PR, increased total RV output per minute was facilitated by an increase in heart rate, an increase in RV FAC and a decrease in PR fraction.

Imaging of endolymphatic hydrops in Meniere's disease at 1.5 T using phase-sensitive inversion recovery: (1) demonstration of feasibility and (2) overcoming the limitations of variable gadolinium absorption.

Endolymphatic hydrops is the primary histopathological finding in Meniere's disease. We demonstrate the feasibility of imaging endolymphatic hydrops at 1.5 T using phase-sensitive inversion recovery (PS-IR) MRI following intratympanic injection of gadolinium (Gd). PS-IR data were imaged using real reconstruction to enable visualization of the phase of the signal permitting clear definition between bone, unopacified endolymph and perilymph. Data were obtained 24h following injection in 2 control subjects and in 13 successive patients with Meniere's disease. In 11 out of 13 patients, dilated endolymphatic structures were clearly identified as filling defects within the opacified perilymph allowing identification of endolymphatic hydrops. There was a large range in the degree of perilymphatic signal enhancement due to variability in absorption of Gd from the middle ear into the perilymph. The use of multiple TI values allowed confident identification of endolymphatic hydrops in Meniere's patients even when perilymph opacification was suboptimal at one TI value. This is the first time endolymphatic hydrops has been demonstrated at 1.5 T in humans. The methods presented are of significant practical importance and will permit broader application of endolymphatic imaging and may also act to reduce the frequency of failed exams due to inadequate Gd uptake.

Survey of effective dose levels from typical paediatric CT protocols.

Concern over reported large radiation doses leading to a high cancer risk for paediatric CT patients has prompted considerable investigation in paediatric CT. The recent release of software from Germany has allowed effective doses to be calculated from CT protocol information and radiation measurement for standard paediatric patient sizes for both sexes. An initial study has been undertaken in nine radiology departments, four of which were dedicated paediatric departments, for routine chest and abdominal CT procedures. The dose calculation software is based on Monte Carlo simulation of X-ray conditions during a CT procedure and utilized a 'tomographic' phantom model of a 7-year-old child and an 8-week-old baby to allow calculation of organ dose and hence effective dose. Results of the survey indicate that effective doses were higher for females than males, and higher for abdominal procedures. Slightly higher effective doses were calculated for the child compared to the baby. All centres but one recorded lower effective doses with their current protocols than if they had used recommended CT protocols found in the literature. Analysis of the survey data indicates that scan parameters are the main cause of dose variations, although the type of scanner can affect dose by a factor of 2 (when comparing different units) as well as variation in anatomy scanned in protocols. Dose reduction appears to be most closely linked with reduced mAs and increased pitch as expected. The calculation of effective dose appears to be a key factor in assessing CT protocols, particularly for paediatric patients.