Eddie W.Y. Cheung

New two-dimensional global longitudinal strain and strain rate imaging for assessment of systemic right ventricular function

Objectives: To determine the usefulness of new two-dimensional strain indices, based on speckle tracking imaging, for assessment of systemic right ventricular (RV) function after an atrial switch operation for transposition of the great arteries. Design: Cross-sectional study. Setting: Tertiary paediatric cardiac centre. Methods: 26 patients, mean (SD) age 21.0 (3.6) years at 19.9 (3.2) years after an atrial switch operation, and 27 age-matched controls were studied. Two-dimensional imaging at the four-chamber view was obtained with tracing of the entire RV endocardial border. The RV global longitudinal strain (GLS) and GLS rate were derived using automated software (EchoPAC, GE Medical) and correlated with tissue Doppler-derived RV isovolumic acceleration (IVA), and, in the patient cohort, with cardiac magnetic resonance-derived RV ejection fraction. Results: Intra- and interobserver variability for measurement of GLS, as determined from the mean (SD) of differences in two consecutive results from 20 studies, were 0.06 (1.39)% and 0.24 (1.77)%, respectively. Compared with controls, patients had lower RV GLS (17.1 (1.9)% vs 26.3 (2.9)%, p<0.001), a reduced GLS rate (0.78 (0.11)/s vs 1.33 (0.23)/s, p<0.001), lower RV IVA (1.10 (0.36) m/s2 vs 1.56 (0.53) m/s2, p<0.001) and increased RV myocardial performance index (0.52 (0.09) vs 0.38 (0.09), p<0.001). Both RV GLS and GLS rate correlated positively with RV IVA (r = 0.43, p = 0.001 and r = 0.46, p<0.001, respectively), and negatively with RV myocardial performance index (r = −0.65, p<0.001 and r = −0.57, p<0.001, respectively). In patients, the GLS rate correlated positively with RV ejection fraction (r = 0.62, p = 0.001). Conclusions: Two-dimensional RV GLS and GLS rate are new, potentially useful indices for assessment of systemic RV function.

Impact of Right Ventricular Dilation on Left Ventricular Myocardial Deformation in Patients After Surgical Repair of Tetralogy of Fallot

Left ventricular (LV) dysfunction is 1 of the major determinants of late adverse clinical outcomes in patients after surgical repair of tetralogy of Fallot (TOF). The aim of this study was to test the hypothesis that LV myocardial deformation is impaired in patients after TOF repair and related to right ventricular (RV) dilation and exercise capacity. Longitudinal, radial, and circumferential LV myocardial deformation was determined using speckle-tracking echocardiography in 23 postoperative patients with TOF and compared to that of 23 age-matched controls. Relations between LV strain and strain rate (SR) and RV volumes and exercise parameters were determined in patients. Compared to controls, patients had reduced global LV longitudinal, radial, and circumferential strain (all p values <0.05). Patients with significantly increased RV end-systolic volume (>2 SDs higher than normal; n = 17) had reduced global LV circumferential strain (p = 0.048) and SR (p = 0.038), but similar longitudinal and radial speckle-tracking echocardiographic parameters, compared to those without (n = 6). RV end-systolic volume was correlated inversely with global LV circumferential strain and SR (r = -0.58, p = 0.004, and r = -0.58, p = 0.005, respectively), while RV end-diastolic volume was correlated only with global LV circumferential strain (r = -0.43, p = 0.047). In patients, the LV ejection fraction was correlated with global LV circumferential strain (r = 0.54, p = 0.01) and SR (r = 0.66, p = 0.001) but not with longitudinal or radial speckle-tracking echocardiographic parameters. Using multivariate analysis, global LV circumferential SR (beta = 0.66, p = 0.001) and male gender (beta = 0.46, p = 0.012) were identified as independent predictors of peak oxygen consumption. In conclusion, the negative impact of RV dilation on LV function relates to its influence on LV circumferential strain and SR in patients after TOF repair.

Oxidative stress in children late after Kawasaki disease: relationship with carotid atherosclerosis and stiffness

BackgroundPersistent arterial dysfunction in patients with a history of Kawasaki disease (KD) and an integral role of oxidative stress in the development of cardiovascular disease are increasingly recognized. We sought to test the hypothesis that oxidative stress is increased in KD patients and related to carotid atherosclerotic changes and stiffness.MethodsWe compared the serum levels of oxidative stress biomarkers, carotid intima-media thickness (IMT), and carotid stiffness index among KD patients with coronary aneurysms (n = 32), those without coronary complications (n = 19), and controls (n = 32).ResultsCompared with controls, patients with coronary aneurysms had significantly higher serum levels of malonaldehyde (2.62 ± 0.12 μM vs 2.22 ± 0.07 μM, p = 0.014) and hydroperoxides (26.50 ± 1.13 μM vs 22.50 ± 0.62 μM, p = 0.008). A linear trend of the magnitude of oxidative stress in relation to inflammatory damage was observed for malonaldehyde (p = 0.018) and hydroperoxides (p = 0.014) levels. Serum malonaldehyde and hydroperoxide levels correlated positively with carotid IMT (p < 0.001 and p = 0.034, respectively) and stiffness index (p = 0.001 and p = 0.021, respectively). Multiple linear regression analysis identified serum malonaldehyde level as a significant determinant of carotid IMT (β = 0.31, p = 0.006) and stiffness (β = 0.27, p = 0.008).ConclusionOur findings suggest oxidative stress is increased in KD patients with coronary aneurysms and is associated with carotid intima-media thickening and stiffening.

Pericardial effusion after open heart surgery for congenital heart disease

Objectives: To determine the prevalence and time course of pericardial effusion after open heart surgery for congenital heart diseases and to identify predisposing risk factors. Design and patients: Prospective assessment of development of pericardial effusion in 336 patients (163 males) undergoing open heart surgery for congenital heart disease by serial echocardiography on days 5, 7, 14, 21, and 28 postoperatively. Setting: Tertiary paediatric cardiac centre. Results: The prevalence of pericardial effusion was 23% (77 of 336). Of the 77 patients who developed effusion, 43 (56%) had moderate to large effusions and 18 (23%) were symptomatic. Patients who had a large amount of effusion were more likely to be symptomatic than those with only a small to moderate amount (47.4% v 15.5%, p = 0.01). The mean (SD) onset of pericardial effusion was 11 (7) days after surgery, with 97% (75 of 77) of cases being diagnosed on or before day 28 after surgery. The prevalence of effusion after Fontan-type procedures (60%, 6 of 10) was significantly higher than that after other types of cardiac surgery: repair of left to right shunts (22.1%, 43 of 195), repair of lesions with right ventricular outflow tract obstruction (22.6%, 19 of 84), arterial switch operation (6.7%, 1 of 15), and miscellaneous procedures (25%, 8 of 32) (p = 0.037). Univariate analyses showed that female patients (p = 0.009) and those receiving warfarin (p = 0.002) had increased risk of postoperative pericardial effusion. A greater pericardial drain output in the first four hours after surgery also tended to be significant (p = 0.056). Multivariate logistic regression similarly identified warfarin treatment (β = 1.73, p = 0.009) and female sex (β for male = −0.63, p = 0.037) as significant determinants. Conclusions: Pericardial effusion occurs commonly after open heart surgery for congenital heart disease. Serial echocardiographic monitoring up to 28 days postoperatively is indicated in selected high risk patients such as those with symptoms of postpericardiotomy syndrome and those given warfarin.

Topiramate-Valproate—Induced Hyperammonemic Encephalopathy Syndrome: Case Report

A 15-year-old boy with inverted duplication of chromosome 15 was admitted for acute onset of irritability, increasing sleepiness, and worsening of seizures. He had been on valproate and other anticonvulsants. However, he was found to have hyperammonemia within 2 weeks after the addition of low-dose topiramate to valproate. He recovered within 7 days after discontinuation of valproate. Topiramate was tailed off. The reintroduction of valproate monotherapy caused hyperammonemia again without clinical features of encephalopathy. He also developed anticonvulsant hypersensitivity syndrome following the use of phenytoin. We propose the term topiramate-valproate—induced hyperammonemic encephalopathy syndrome to include the following features: excessive sleepiness or somnolence, aggravation of seizures, hyperammonemia, and absence of triphasic waves on electroencephalography in any individual on simultaneous topiramate-valproate therapy. The ammonia level ranged from 1.5 to 2 times normal. The serum valproate level might be within the therapeutic range. The possible mechanism is topiramate-induced aggravation of all the known complications of valproate monotherapy. This condition is reversible with cessation of either valproate or topiramate. (J Child Neurol 2004;19:157—160).

Functional implications of the right ventricular myocardial performance index in patients after surgical repair of tetralogy of Fallot.

The myocardial performance index (MPI) has been proposed to be a simple echocardiographic index of right ventricular (RV) function in patients after surgical repair of tetralogy of Fallot (TOF). However, its functional status remains to be clarified. The functional implications of RV MPI were determined by exploring its relationships with parameters of RV function as derived from cardiovascular magnetic resonance (CMR), and exercise capacity of postoperative TOF patients Thirty patients (11 males), aged 15.6 ± 3.1 years, who have undergone surgical repair of TOF at 4.0 ± 1.8 years, were studied. The RV and left ventricular (LV) MPIs determined using pulsed-wave Doppler echocardiography were related to CMR-derived RV and LV ejection fractions, and pulmonary regurgitant fraction and treadmill exercise testing parameters. Log RV MPI correlated positively with log LV MPI (r = 0.38, P = 0.037) and negatively with CMR-derived RV ejection fraction (r = −0.4, P = 0.028) and pulmonary regurgitant fraction (r = −0.4, P = 0.031). No significant correlations were found between LV MPI and any of the CMR parameters. Using receiver operated characteristics analysis, a cutoff value of 0.30 for RV MPI was found to have a sensitivity of 100% and specificity of 74% in predicting a RV ejection fraction <35%. Right ventricular, but not LV, MPI correlated inversely with exercise duration (r = −0.45, P = 0.013) and peak oxygen consumption (VO2 max) (r = −0.56, P = 0.001). Multivariate analysis identified RV MPI (β = −0.6, P < 0.001), male sex (β = 0.44, P = 0.01), and duration from surgery (β = −0.30, P = 0.019) as significant determinants of VO2 max. Increased MPI is a reflection of reduced RV ejection fraction and exercise capacity in patients after TOF repair.