Standardized Treatment and Diagnostic Approach to Reduce Disease burden in the early postoperative phase in children with congenital heart defects-STANDARD study: a pilot randomized controlled trial.

To explore the effect of a daily goal checklist on pediatric cardiac intensive care unit (PCICU) length of stay (LOS) after congenital heart surgery. This study is a prospective randomized single-center study. Group characteristics were as follows: STANDARD group: n = 30, 36.7% female, median age 0.9 years; control group: n = 33, 36.4% female, median age 1.1 years. Invasive ventilation time, STAT categories, mean vasoactive-inotropic score (VIS)24h, maximal (max.) VIS24h, mean VIS24-48h, max. VIS24-48h, VIS category, number of sedatives, analgesics, diuretics, number of deployed diagnostic modalities, morbidities, and mortality did not differ between both groups. Median PCICU LOS was 96.0 h (STANDARD group) versus 101.5 h (control group) (p = 0.63). In the overall cohort, univariate regression analysis identified age at surgery (b = -0.02), STAT category (b = 18.3), severity of CHD (b = 40.6), mean VIS24h (b = 3.5), max. VIS24h (b = 2.2), mean VIS24-48h (b = 6.5), and VIS category (b = 13.8) as significant parameters for prolonged PCICU LOS. In multivariate regression analysis, age at surgery (b = -0.2), severity of CHD (b = 44.0), and mean VIS24h (b = 6.7) were of significance. Within the STANDARD sub-group, univariate regression analysis determined STAT category (b = 32.3), severity of CHD (b = 70.0), mean VIS24h (b = 5.0), mean VIS24-48h (b = 5.9), number of defined goals (b = 2.6), number of achieved goals (b = 3.3), number of not achieved goals (b = 10.8), and number of unevaluated goals (b = 7.0) as significant parameters for prolonged PCICU LOS. Multivariate regression analysis identified the number of defined goals (b = 2.5) and the number of unevaluated goals (b = -3.0) to be significant parameters.   Conclusion: The structured realization and recording of daily goals is of advantage in patients following pediatric cardiac surgery by reducing PCICU LOS. What is known: • Communication errors are the most frequent reasons for adverse events in intensive care unit patients. • Improved communication can be achieved by discussion and documentation of the patients' goals during daily rounds. What is new: • In the overall cohort age at surgery, severity of congenital heart defect and mean vasoactive inotropic score within the first 24 hours had significant impact on pediatric cardiac intensive care unit (PCICU) length of stay (LOS). • In the intervention group, the number of defined goals and the number of unevaluated goals were significant parameters for prolonged PCICU LOS.

Assessment of left ventricular myocardial work in Turner syndrome patients: insights from the novel non-invasive pressure-strain loop analysis method.

BACKGROUND: Turner syndrome (TS), a relatively rare chromosomal disease, is associated with multiple cardiovascular risk factors that possibly lead to increased left ventricular afterload and functional impairment. The aim of this study was to investigate whether alterations in myocardial work and work efficiency can be found in TS patients through left ventricular pressure-strain loop analysis (PSL). METHODS: Thirty-eight patients with TS and 19 healthy, age-matched controls were recruited for this study. Global peak systolic strain (GLPS) and PSL of the left ventricle was assessed in study participants. TS patients whose history included coarctation of the aorta or prior cardiac surgery were excluded from GLPS and PSL analyses (n=5). RESULTS: Median age was 16.00 years in the TS group and 16.35 years in the control group (P=0.236). GLPS did not show significant differences between both groups (P=0.524). TS patients demonstrated, compared to controls, a significantly higher global myocardial work index (BSA) (mean ± SD: 1,497±505 vs. 1,214±245 mmHg*%/m2; P=0.027). Heart rate was significantly increased in TS patients, compared to controls (mean ± SD: 90.08±14.79 vs. 73.95±15.05 bpm; P<0.001), and correlated significantly with global myocardial work index [body surface area (BSA)] within the TS cohort (r=0.558, P=0.001). CONCLUSIONS: TS patients showed signs of increased myocardial workload that were only detectable through the novel PSL analysis method and not through GLPS. Moreover, elevated resting heart rate was linked with increased myocardial workload in TS patients. Further studies will have to investigate whether TS patients may develop advanced left ventricular systolic dysfunction later in life.

Assessment of inter-atrial, inter-ventricular, and atrio-ventricular interactions in tetralogy of Fallot patients after surgical correction. Insights from two-dimensional speckle tracking and three-dimensional echocardiography.

BACKGROUND: We aimed to assess biatrial size and function, interactions on atrial and ventricular levels, and atrio-ventricular coupling in patients after tetralogy of Fallot repair. METHODS: A total of 34 patients with a mean age of 20.9±9 years, and 35 healthy controls, underwent two-dimensional speckle tracking echocardiography for ventricular and atrial strain measurements and real-time three-dimensional echocardiography to assess ventricular and atrial volumes. RESULTS: When compared with controls, tetralogy of Fallot patients had significantly reduced right atrial peak atrial longitudinal strain (p<0.01), right atrial peak atrial contraction strain (p<0.01), right atrial ejection fraction (p<0.01), left atrial peak atrial longitudinal strain (p<0.01), left atrial peak atrial contraction strain (p<0.05), and left atrial ejection fraction (p<0.01). In the tetralogy of Fallot group, left ventricular ejection fraction was negatively related to the right ventricular end-systolic volume normalised to body surface area (r=-0.62, p<0.01). An association was found in patients between the right atrial peak longitudinal strain and mean right ventricular strain (r=0.64, p<0.01). In patients, the left atrial peak longitudinal strain correlated negatively with right atrial end-diastolic volume normalised to body surface area (r=-0.67, p<0.01), whereas the left atrial ejection fraction correlated weakly with left ventricular ejection fraction (r=0.41, p<0.05). CONCLUSIONS: In asymptomatic tetralogy of Fallot patients, biatrial dysfunction exists and can be quantified via two-dimensional speckle tracking echocardiography as well as real-time three-dimensional echocardiography. Different forms of interactions on atrial and ventricular levels are evident among such cohorts.

Assessment of interventricular and right-intraventricular dyssynchrony in patients with surgically repaired tetralogy of Fallot by two-dimensional speckle tracking.

AIMS: We aimed to assess interventricular and right-intraventricular dyssynchrony in patients after tetralogy of Fallot (TOF) repair by two-dimensional (2D) speckle tracking and to identify factors associated with dyssynchrony. METHODS AND RESULTS: Forty-two patients after TOF repair with a mean age of 19.8 years and 42 age-matched healthy controls were studied. Longitudinal myocardial deformation (strain) and time-to-peak intervals were assessed by 2D speckle tracking and tissue Doppler imaging (TDI) in an apical four-chamber view. Dyssynchrony was defined as delay above 3 standard deviations of mean values in the control group. Magnetic resonance imaging (MRI) was performed for evaluation of ventricular function. Using 2D speckle tracking, 22 patients (52%) showed interventricular dyssynchrony and 16 (38%) had right-intraventricular dyssynchrony. The interventricular delay correlated significantly with right ventricular (RV) strain (r = 0.687, P < 0.001), RV systolic pressure (r = 0.535, P = 0.001), QRS duration (r = 0.466, P = 0.002), RV end-diastolic (r = 0.377, P = 0.018), and RV end-systolic volumes (r = 0.452, P = 0.004) as well as RV ejection fraction (r = -0.378, P = 0.018). Similarly, the right-intraventricular delay correlated significantly with RV strain (r = 0.534, P < 0.001), QRS duration (r = 0.428, P = 0.005), RV end-systolic volume (r = 0.34, P = 0.038), and RV systolic pressure (r = 0.413, P = 0.015). In multivariate regression analysis, reduced RV strain and prolonged QRS duration remained the main determinant factors predicting dyssynchrony. Moreover, 2D speckle tracking and TDI showed a significant correlation in the assessment of the interventricular (r = 0.738, P < 0.001) and right-intraventricular delay (r = 0.747, P < 0.001). CONCLUSION: Interventricular and right-intraventricular dyssynchrony are detectable in patients after TOF repair by 2D speckle tracking. Reduced RV myocardial deformation and QRS prolongation are the main factors associated with the observed dyssynchrony.

Assessment of left ventricular dyssynchrony and function using real-time 3-dimensional echocardiography in patients with congenital right heart disease.

BACKGROUND: Patients after repair of congenital right heart disease (CRHD) may exhibit left ventricular (LV) dyssynchrony (LVD). However, the diagnosis of LVD is difficult and its reliability limited because current methods do not assess LVD of the whole LV simultaneously. The aim of the study was to assess LVD according to a novel global systolic dyssynchrony index (SDI) derived from real-time 3-dimensional echocardiography in patients after repaired CRHD. METHODS: Two-dimensional echocardiography and real-time 3-dimensional echocardiography were performed in 30 patients after CRHD repair and in 30 matched healthy controls. Real-time 3-dimensional echocardiography data sets provided time-volume curves, and 2 global SDIs were derived from the dispersion of time to reach minimal systolic volume according to a 16- or 17-LV segment model. RESULTS: Both SDIs were significantly elevated in the patient as compared with the control group (P < .001). A cutoff value for both SDIs was calculated and LVD defined as one of the SDIs exceeding cutoff. Left ventricular dyssynchrony was present in 5 (100%) of 5 patients with a LV ejection fraction (EF) <50% and 13 (52%) of 25 patients with preserved LVEF, thus being diagnosed in a total of 18 (60%) of 30 patients. Moreover, patients with LVD showed a significantly higher degree of pulmonary regurgitation (P = .01) with elevated right ventricular volumes and altered septal motion. Stepwise multivariate analysis identified LVEF (P = .005) and the degree of pulmonary regurgitation (P = .02) as independent predictors of LVD. CONCLUSIONS: Left ventricular dyssynchrony can be detected in about 60% of patients after CRHD repair and is mainly due to significant pulmonary regurgitation resulting in an altered septal motion and systolic LV function.

Noninvasive assessment of liver fibrosis in patients with Fontan circulation using transient elastography and biochemical fibrosis markers.

OBJECTIVE: Studies have shown that patients with failing Fontan circulation may develop liver cirrhosis with its sequelae. Therefore, early detection of fibrosis is essential to identify patients at risk. Transient elastography has been evaluated for noninvasive staging of liver fibrosis in a multitude of studies. METHODS: A total of 39 patients who underwent the Fontan procedure were included in the study. All patients underwent an abdominal ultrasound, transient elastography, and detailed laboratory testing. Conventional echocardiography assessment of inflow and outflow and ventricular function were performed. RESULTS: Significant signs of liver fibrosis were found in 36 of 39 children according to the elastography method and in 28 of 39 children according to the measured biochemical fibrosis markers. The Spearman correlation coefficient between the liver stiffness measured with transient elastography and the time interval since the Fontan operation was highly significant (0.514, P = 0.001). CONCLUSION: The present study shows that patients who undergo the Fontan procedure are at increased risk of developing liver fibrosis and liver cirrhosis. The risk increases with the age of the patient and the time interval since the Fontan procedure. The noninvasive measurement of liver fibrosis using transient elastography and fibrosis marker scores can be a useful tool to identify patients at risk and for noninvasive surveillance.

Assessment of regional atrial function in patients with hypertrophic cardiomyopathies using tissue Doppler imaging.

BACKGROUND: This study applied tissue Doppler imaging and color tissue Doppler imaging to study atrial function changes in patients with hypertrophic cardiomyopathy (HCM). The profile of the segmental atrial velocities and the strain rate were determined and compared with those of normal matched control subjects. METHODS: This study investigated 20 patients with HCM and 20 age-matched healthy control subjects. In a four-chamber apical view, tissue Doppler imaging was used to measure the lateral left and right atrial (LA and RA) and interatrial septal (IAS) wall systolic, early, and late diastolic velocities. Similarly, the atrial strain rate during ventricular systole (SR(S)) and the early (SR(E)) and late (SR(A)) diastolic phases in patients and control subjects were measured. The interventricular septal tissue Doppler-derived isovolumic relaxation time was calculated. RESULTS: Only the IAS annular and middle segments showed a significant reduction in the early diastolic velocity (mean, 4.01 +/- 2.2 vs 8.7 +/- 1.1, p = 0.001; 3.23 +/- 2 vs 6.01 +/- 1.9, p = 0.001, respectively) for the patients with HCM in comparison with the control subjects. Generally, the atrial strain rate was clearly reduced. The systolic strain rate (SR(S)) was significantly reduced in the LA wall in the annular (p = 0.007) and middle (p = 0.001) segments and in the IAS middle segment (p = 0.007). Similarly, there was a reduction of the early diastolic strain rate (SR(E)) in the LA annular (p = 0.001) and middle (p = 0.01) segments and in the IAS annular (p = 0.05) and middle (p = 0.001) segments, as well as in the RA annular segment (p = 0.02). The RA middle segments showed insignificant changes. CONCLUSION: Atrial function may be affected by HCM due to impairment of myocardial diastolic function. Strain rate imaging is reproducible, yields readily obtained parameters that provide unique data about global and longitudinal segmental atrial contraction, and can quantify the atrial dysfunction in patients with HCM.