Aortic atresia with interrupted aortic arch and bilateral arterial ductus: a successful initial palliation.

A combination of aortic valve atresia and an interrupted aortic arch is a unique disease in which perfusion to the brain and myocardium depends on coexisting lesions or type of interruption. We report a case of aortic valve atresia with type B interrupted arch, bilateral arterial ductus in a neonate who was successfully palliated using a hybrid approach by placing stents in both arterial ductus and banding of branch pulmonary arteries.

Efficacy of nil per oral, total parenteral nutrition, milrinone and non-suction chest tube drainage-based management for chylothorax following pediatric cardiac surgery.

OBJECTIVE: A single centre experience with chylothorax in post cardiac surgical patients. METHODS: Retrospective review. RESULTS: Chylothorax developed in 55 out of 873 operated patients (6.3%). Median age of the chylothorax cohort was 95 days (range 1-995). Neonates constituted 36% and 49% were infants. Group-1(35 patients-treated during the years 2011-2015) included those who were managed with low fat diet initially with other standard measures including steroid, octreotide, pleurodesis, lymphangiogram or thoracic duct ligation whenever required.Group-2 (20 patients, treated between year 2016-2018) were managed with nil per oral, total parenteral nutrition, extended use of milrinone and no use of chest tube suction with other above standard measures when required.Group-1 and group-2 were comparable in terms of their age and weight (p > 0.05).We observed lower volume of chest drainage, shorter intubation time, length of intensive care stay and hospital stay in group-2 compared to group-1 though they were statistically not significant (p > 0.05). Occurrence of massive chylothorax (>20 ml/kg/day) in group-1 was significantly higher [18 patients (51%) in group-1 vs 4 patients in group-2 (20%) (Chi-square 5.25, p = 0.02)]. In hospital mortality in group-1 was higher compared to group-2 (5/35 = 14.5% vs 1/20 = 5%), however, it was statistically not significant [risk ratio 2.86; 95% CI 0.36, 22.77; p = 0.59)]. Acute kidney injury was observed in about 25% of patients who had chylothorax. A higher mortality was observed in patients with chylothorax who had acute kidney injury [5/14 (35%)] compared to those who did not have acute kidney injury [1/41 (2.4%)] (Chi-square 11.89, p = 0.001)]. SUMMARY: In a heterogenous cohort of post-cardiac surgical patients who developed chylothorax, our suggested new regime (nil per oral, parenteral nutrition, extended use of milrinone and no suction applied to the chest drains) contributed to reduce the frequency of massive chylothorax occurrence significantly.

Hemodynamic and Structural Comparison of Human Fetal Heart Development Between Normally Growing and Hypoplastic Left Heart Syndrome-Diagnosed Hearts.

Congenital heart defects (CHDs) affect a wide range of societies with an incidence rate of 1.0-1.2%. These defects initiate at the early developmental stage and result in critical health disorders. Although genetic factors play a role in the formation of CHDs, the occurrence of cases in families with no history of CHDs suggests that mechanobiological forces may also play a role in the initiation and progression of CHDs. Hypoplastic left heart syndrome (HLHS) is a critical CHD, which is responsible for 25-40% of all prenatal cardiac deaths. The comparison of healthy and HLHS hearts helps in understanding the main hemodynamic differences related to HLHS. Echocardiography is the most common imaging modality utilized for fetal cardiac assessment. In this study, we utilized echocardiographic images to compare healthy and HLHS human fetal hearts for determining the differences in terms of heart chamber dimensions, valvular flow rates, and hemodynamics. The cross-sectional areas of chamber dimensions are determined from 2D b-mode ultrasound images. Valvular flow rates are measured via Doppler echocardiography, and hemodynamic quantifications are performed with the use of computational fluid dynamics (CFD) simulations. The obtained results indicate that cross-sectional areas of the left and right sides of the heart are similar for healthy fetuses during gestational development. The left side of HLHS heart is underdeveloped, and as a result, the hemodynamic parameters such as flow velocity, pressure, and wall shear stress (WSS) are significantly altered compared to those of healthy hearts.

Numerical Investigation of the Fetal Left Heart Hemodynamics During Gestational Stages.

Flow-driven hemodynamic forces on the cardiac tissues have critical importance, and have a significant role in the proper development of the heart. These mechanobiological mechanisms govern the cellular responses for the growth and remodeling of the heart, where the altered hemodynamic environment is believed to be a major factor that is leading to congenital heart defects (CHDs). In order to investigate the mechanobiological development of the normal and diseased hearts, identification of the blood flow patterns and wall shear stresses (WSS) on these tissues are required for an accurate hemodynamic assessment. In this study, we focus on the left heart hemodynamics of the human fetuses throughout the gestational stages. Computational fetal left heart models are created for the healthy fetuses using the ultrasound images at various gestational weeks. Realistic inflow boundary conditions are implemented in the models using the Doppler ultrasound measurements for resolving the specific blood flow waveforms in the mitral valve. Obtained results indicate that WSS and vorticity levels in the fetal left heart decrease with the development of the fetus. The maximum WSS around the mitral valve is determined around 36 Pa at the gestational week of 16. This maximum WSS decreases to 11 Pa at the gestational week of 27, indicating nearly three-times reduction in the peak shear stress. These findings reveal the highly dynamic nature of the left heart hemodynamics throughout the development of the human fetus and shed light into the relevance of hemodynamic environment and development of CHDs.

Cost-effectiveness of Oral Versus Intravenous Ibuprofen Therapy in Preterm Infants With Patent Ductus Arteriosus in the Neonatal Intensive Care Setting: A Cohort-based Study.

PURPOSE: Use of ibuprofen for the patent ductus arteriosus (PDA) has become increasingly common. This study aimed to evaluate the clinical and economic impact of oral ibuprofen versus intravenous ibuprofen for PDA among preterm infants. METHODS: This retrospective, cohort-based pilot study examined the clinical and economic associations of oral versus intravenous ibuprofen for PDA. A decision-analytic model was constructed, from the hospital perspective, to follow the oral versus intravenous administrations of ibuprofen for PDA and their clinical and economic consequences. The course regimen of either formulation was an initial 10 mg/kg followed by 5 mg/kg at 24- and 48-h intervals. Clinical and resource utilization data were extracted from Cerner medical database, from 2014 through 2018, at the tertiary neonatal intensive care unit setting in Qatar. The primary outcome measures were the rate of successful closure based on the ductal diameter measure after the first course of treatment and the overall direct medical cost of PDA management. A population of 118 neonates was required for results with 80% power and 0.05 significance. Sensitivity analyses involving unit costs and a subgroup analysis based on gestational age and birth weight, added to a second-order probabilistic analysis of all model inputs, were performed. FINDINGS: Forty infants were available for inclusion in the oral ibuprofen study group, not achieving the desired sample size, with successful PDA closure reported in 64% of cases compared with a reduced success of 36% with intravenous ibuprofen (n = 59) (risk ratio = 0.56; 95% CI, 0.32-0.97; P = 0.04), which was associated with economic advantage to oral ibuprofen. The probabilistic analysis illustrated that oral ibuprofen costs less than intravenous ibuprofen in 72% of patient cases, with QAR 48,751 (US $13,356) (95% CI, QAR 47,500-50,000, US $13,014-$13,699) in mean savings. Sensitivity analyses confirmed the robustness of study conclusions and found that the rate of closure success versus failure was the most influential on results, followed by the occurrence of adverse drug events with both intravenous and oral ibuprofen. Although both ibuprofen formulations had similar safety profiles (P = 0.16), the intravenous formulation was associated with a larger number of adverse drug effects. IMPLICATIONS: This is the first cost-effectiveness evaluation of oral versus intravenous formulations of ibuprofen among infants with PDA. The oral ibuprofen might be associated with an enhanced ductal closure at a considerably lower cost. The study results support recent trends in neonatal intensive care unit practices in favor of the oral administration of ibuprofen.

High incidence of abdominal venovenous collaterals after Kawashima operation.

INTRODUCTION: Systemic venous collaterals have been found at different stages of single-ventricle palliation, specifically after Kawashima operation. In this study, we present the incidence, clinical features, associated risk factors, and management of such venovenous collaterals (VVCs) after Kawashima procedure. MATERIALS AND METHODS: A retrospective review of all the patients who underwent Kawashima procedure prior to December 2011 at Hamad General Hospital, Qatar, was performed. Data were collected and reviewed from medical records. RESULTS: The study group involved a total of six patients with Kawashima procedure. The median age of patients was 112.7 months (range 46-336 months), and median age at the time of Kawashima operation was 31.7 months (range 15-187 months). Mean systemic arterial oxygen saturation (Spo 2) after Kawashima operation was 94.5% ± 1.5%. In five patients, during a median follow-up of 53.4 months (range 16.9-147.9 months), the Spo 2 declined to a mean of 78.8% ± 8.2%. A total of 16 venous collaterals were observed in the study group, and majority (70%) of these collaterals were subdiaphragmatic. CONCLUSION: In our study, abdominal VVCs were observed in 100% of the patients who were followed after Kawashima operation. We therefore recommend that thorough evaluation for such VVCs should be part of the ongoing evaluation of patients after Kawashima operation, especially in those with low Spo 2. We also recommend early surgical rerouting of hepatic veins to pulmonary arteries in all post-Kawashima patients.

Doppler assessment of the ratio of the systolic to diastolic duration in normal children: relation to heart rate, age and body surface area.

BACKGROUND: It has been shown that the ratio of systolic duration to diastolic duration (S/D) is a valuable global index of ventricular dysfunction in pediatric dilated and restrictive cardiomyopathy and is also a valuable index of the ventricular function of single systemic right ventricles in children who have undergone palliation of hypoplastic left-heart syndrome. The purposes of this study were to establish normal values for the S/D ratio in children without heart disease and to investigate its variation with heart rate (HR), age, and body surface area. METHODS: Of 752 children found to have functional murmurs by clinical means, 179 (24% of the total database) had trace holosystolic tricuspid regurgitation. These patients ranged in age from 0.02 months to 19 years. For this study, the diastolic interval was defined as the period of right ventricular filling (ie, the period between 2 tricuspid regurgitant jets) and the systolic interval as the remainder of the cardiac cycle corresponding to the duration of holosystolic tricuspid regurgitation, including the periods of isovolumic contraction and relaxation. The relations between the systolic and diastolic periods and their ratio (S/D ratio) and HR, age, and body surface area were evaluated using univariate and multivariate linear regression analysis. RESULTS: Patient's age ranged from 0.02 months to 19 years (mean, 70.18+/-65.12 months), body surface area from 0.11 to 2.51 m2 (mean, 0.85+/-0.55 m2), and HR from 50 to 156 beats/min (mean, 96.72+/-23.19 beats/min). The systolic period ranged from 208.5 to 467 ms (mean, 314.08+/-52.57 ms) and the diastolic period from 166.5 to 809 ms (mean, 341.34+/-129.61 ms), yielding a S/D ratio ranging from 0.397 to 1.62 (mean, 0.995+/-0.23). Systolic period duration showed a linear negative decrease with increasing HR (y=1.9228x+500.05, r=-0.85). Diastolic duration decreased in an exponential fashion as HR increased (y=130,679x(-1.3232), r=-0.88). The S/D ratio correlated positively with HR (y=-1.656+0.0265 HR-0.000954 HR2, r=76). On multivariate analysis, no significant correlations of S/D ratio with age or body surface area were found. CONCLUSIONS: The results of this study provide useful reference values for the S/D ratio across a wide range of HRs in children, adolescents, and young adults.