Platelet Aggregation Before Aspirin Initiation in Pediatric Patients With Congenital Heart Disease at High Risk of Thrombosis.

Background: Aspirin following unfractionated heparin is the most common anticoagulation strategy for pediatric patients who experienced cardiac surgery at high risk of thrombosis. The platelet aggregation test is the golden method to evaluate the aspirin effect on platelet function. However, the platelet aggregation basal status before postoperative aspirin initiation and the related clinical influencing factors hasn't been investigated systemically in this population. Methods: In a prospective cohort of 247 children, arachidonic acid-induced platelet aggregation (PAG-AA) was measured by means of light transmission aggregometry (LTA) before the first dose of aspirin after cardiac surgical procedure and the perioperative variables were also collected. Distribution of this population's PAG-AA basal status was described. Univariate and multivariate logistic regression analysis were performed to identify the main influencing factors of PAG-AA. Results: The median time of aspirin administration was 2 (1-27) days after surgery and the corresponding median value of basal PAG-AA was 20.70% (1.28-86.49%), with 67.6% population under 55% and 47.8% population under 20%. Patients undergoing cardiopulmonary bypass (CPB) had a significantly lower basal PAG-AA than those without (30.63 ± 27.35 vs. 57.91 ± 27.58, p = 0.013). While patients whose test done within 3 days after CPB had a significantly lower PAG-AA than those out of 3 days (25.61 ± 25.59 vs. 48.59 ± 26.45, p = 0.001). Univariate analysis implied that the influencing factors of the basal PAG-AA including CPB use, test time point, cyanosis, and platelet count. Multivariate regression analysis indicated that only CPB use, test time point, and platelet count were the main independent influencing factors for the basal PAG-AA. Conclusion: The majority of children have impaired basal platelet aggregometry responses before postoperative aspirin initiation. The main influencing factors are CPB use, test time point, and platelet count. To establish the platelet aggregometry baseline prior to commencement of aspirin therapy, testing should be performed 3 days later following the procedure when effect of CPB is basically over.

Aortopulmonary Collateral Arteries in Noncyanotic Congenital Heart Disease.

Development of major aortopulmonary collateral arteries are strongly associated with cyanotic congenital heart disease. However, they have rarely been reported in noncyanotic congenital heart disease. We report a rare case of a newborn originally diagnosed with an atrial septal defect, a ventricular septal defect, and pulmonary arterial hypertension who underwent complete repair. Failure to progress postoperatively lead to the delayed diagnosis of aortopulmonary collateral arteries. Percutaneous embolization and surgical ligation of aortopulmonary collateral arteries resulted in rapid recovery.

Vasopressin in Vasodilatory Shock for Both Left and Right Heart Anomalous Pediatric Patients After Cardiac Surgery.

Although the use of vasopressin has become commonplace in pediatric patients with vasodilatory shock after cardiac surgery, its efficacy and hemodynamic effects have not been systematically documented. Furthermore, previous studies were mainly limited patients with left heart anomalies. To date, the use of vasopressin in patients with right heart anomalies has not yet been reported. To clarify the hemodynamic effects of vasopressin on pediatric patients with vasodilatory shock after cardiopulmonary bypass, 70 consecutive patients, most of whom with right heart anomalies, were retrospectively analyzed in Fuwai Hospital from October 2013 to September 2015. Vasopressin was administered continuously at a dose of 0.0002 to 0.002 u/kg/min. Hemodynamics, urine output, and catecholamine vasopressor doses were compared before and after vasopressin initiation. Results showed that besides the significant increase in blood pressure at 2 h after vasopressin administration, the systemic vascular resistance index also prominently elevated from 894.3 ± 190.8 dyn/s to 1138.2 ±â€Š161.4 dyn/s per cm per m, while the heart rate, right atrial pressure, pulmonary artery pressure had a trend of decline. Subsequently, the fluid requirement, the catecholamine vasopressor requirement both decreased and urine output increased. Lactate concentration showed a later remarkable decline at 12 h since vasopressin administration. All the 70 patients survived to hospital discharge. In conclusion, low dose of vasopressin administration was associated with great and timely hemodynamic improvement for pediatric patients with vasodilatory shock after cardiac surgery without any significant adverse effects.

Unplanned re-intervention within 30 days after pediatric cardiac surgery / 中国胸心血管外科临床杂志

@#Objective     To recognize the risk factors of unplanned re-interventions within 30 days after pediatric cardiac surgery and evaluate the outcome of re-interventions. Methods     We retrospectively analyzed the clinical data of 202 children in Fuwai Hospital between January 1, 2015 and August 31, 2017. There were 115 males and 87 females at average age of 32.4 months with range of 3 days to 14 years. Results     There were 202 children who underwent unplanned re-intervention during 30 days post-operation, including 54 re-adjustments of pulmonary blood flow, 34 re-corrections for residual cardiac abnormalities, 28 cardiopulmonary resuscitations, 38 for coagulation problems, 19 pericardial drainages, 11 palliative re-operations to deliver heart load and 6 diaphragmatic folds and 12 others. The mortality rate among children who underwent unplanned re-inventions after cardiac surgery was 10.9% (22/202). It was much higher than those free from re-interventions (0.7%). Time of mechanical ventilation was 284.3 (11–2 339) h, and mean ICU stay was 17.7 (1–154) d, significantly longer than those free from re-interventions at the same period. Conclusion     Unplanned re-interventions after pediatric cardiac surgery is associated with higher mortality rate and longer recovery time. Early identifying risk factors and re-intervention can reduce the complications and improve the prognosis.

Application of pulmonary surfactant in the treatment of severe acute respiratory distress syndrome in different age baby with congenital heart diseases / 中国胸心血管外科临床杂志

@#Objective To evaluate the efficacy of pulmonary surfactant (PS) on severe acute respiratory distress syndrome (ARDS) in different age baby with congenital heart disease. Methods We divided 43 baby patients into two separate groups including a little baby group (12 patients with age less than 3 months) and an infants group (31 patients with age of 3 months to one year). Both groups of patients were treated with intratracheal PS at the same time. The clinical data were collected and analyzed. Results The little baby group had lower body weight. There was no statistical difference in the cardiopulmonary bypass (CPB) time, operation blocking time, mechanical ventilation time, ICU stay time between the two groups (P>0.05). Before treatment, arterial partial presurre of oxygen (PaO2), fractional oxygen concentration in inspire gas (FiO2), the ratio of arterial PO2 to the inspired oxygen fraction (P/F) and arterial-alveolar N2 difference or gradient (a/A) had no difference between the two groups (P>0.05). After treatment, PaO2 and P/F of both groups were significantly lower than before (P<0.05), and FiO2 and P/F were significantly higher than before (P<0.05). After 24 h of treatment, PaO2 and P/F of the little baby group was significantly higher than that of the infants group (P<0.05), and FiO2 and P/F were significantly lower than those of the infants group (P<0.05). Conclusion PS treating severe ARDS in little baby with congenital heart disease has better effect than infants.

NEXN inhibits GATA4 and leads to atrial septal defects in mice and humans.

AIMS: Cardiac structural genes have been implicated as causative factors for congenital heart diseases (CHDs). NEXN is an F-actin binding protein and previously identified as a disease gene causing cardiomyopathies. Whether NEXN contributes to CHDs aetiologically remains unknown. Here, we explored the function of NEXN in cardiac development. METHODS AND RESULTS: First, we determine the role of NEXN in cardiac differentiation using mouse P19cl6 in vitro model; we demonstrated that NEXN inhibited cardiac contractile markers, serving as a negative regulator. Interestingly, we found this effect was mediated by GATA4, a crucial transcription factor that controls cardiac development by knockdown, overexpression, and rescue experiment, respectively. We then generated transgenic mouse models and surprisingly, we discovered cardiac-selective expression of the NEXN gene caused atrial septal defects (ASDs). Next, to search for the mutations in NEXN gene in patients suffering from ASDs, we sequenced the exon and exon-intron joint regions of the NEXN gene in 150 probands with isolated ASDs and identified three mutations in the conserved region of NEXN (c.-52-78C>A, K199E, and L227S), which were not found in 500 healthy controls. Finally, we characterize the related mechanisms and found all mutations inhibited GATA4 expression. CONCLUSION: We identify NEXN as a novel gene for ASD and its function to inhibit GATA4 established a critical regulation of an F-actin binding protein on a transcription factor in cardiac development.