The Nutritional Status of Pediatric Patients with Single Ventricle Undergoing a Bidirectional Glenn Procedure.

Single ventricle (SV) physiology is associated with growth retardation in children. The nutritional status of pediatric patients with SV undergoing a bidirectional Glenn (BDG) procedure vitally affects the feasibility of the next operation stages. To explore the nutritional status and to identify specific anthropometric parameters relevant to short-term surgical outcomes in children with SV after the BDG procedure, this study included 151 patients who underwent the BDG procedure. Anthropometric assessments and Infant and Child Feeding Index (ICFI) scores were used to evaluate nutritional status. There was a significant statistical correlation between ICFI and malnutrition in both the height-for-age Z-score (HAZ) and weight-for-age Z-score (WAZ) groups (P < 0.05). The clinical data, including ventilation time, nosocomial infection presence, pressure injury presence, peritoneal dialysis status, and total intensive care unit days, after BDG surgery were significantly different among the HAZ groups (P < 0.05), while nosocomial infection was different among the WAZ groups (P < 0.05). Children after BDG procedure had a high incidence of malnutrition, in addition to disease factors, the type and frequency of dietary intake were also important factors leading to worse clinical outcomes during hospitalization. Therefore, it is vital to maintain an optimal nutritional status in infants with SV who are undergoing a series of surgical procedures.

Clinical features of plastic bronchitis in children after congenital heart surgery.

BACKGROUND: Plastic bronchitis (PB) can occur in patients who have undergone congenital heart surgery (CHS). This study aimed to investigate the clinical features of PB in children after CHS. METHODS: We conducted a retrospective cohort study using the electronic medical record system. The study population consisted of children diagnosed with PB after bronchoscopy in the cardiac intensive care unit after CHS from May 2016 to October 2021. RESULTS: A total of 68 children after CHS were finally included in the study (32 in the airway abnormalities group and 36 in the right ventricular dysfunction group). All children were examined and treated with fiberoptic bronchoscopy. Pathogens were detected in the bronchoalveolar lavage fluid of 41 children, including 32 cases in the airway abnormalities group and 9 cases in the right ventricular dysfunction group. All patients were treated with antibiotics, corticosteroids (intravenous or oral), and budesonide inhalation suspension. Children with right ventricular dysfunction underwent pharmacological treatment such as reducing pulmonary arterial pressure. Clinical symptoms improved in 64 children, two of whom were treated with veno-arterial extracorporeal membrane oxygenation (ECMO) due to recurrent PB and disease progression. CONCLUSIONS: Children with airway abnormalities or right ventricular dysfunction after CHS should be alerted to the development of PB. Pharmacological treatment such as anti-infection, corticosteroids, or improvement of right ventricular function is the basis of PB treatment, while fiberoptic bronchoscopy is an essential tool for the diagnosis and treatment of PB. ECMO assistance is a vital salvage treatment for recurrent critically ill PB patients.

Perioperative extracorporeal membrane oxygenation in pediatric congenital heart disease: Chinese expert consensus.

BACKGROUND: Congenital heart disease (CHD) is one of the main supportive diseases of extracorporeal membrane oxygenation in children. The management of extracorporeal membrane oxygenation (ECMO) for pediatric CHD faces more severe challenges due to the complex anatomical structure of the heart, special pathophysiology, perioperative complications and various concomitant malformations. The survival rate of ECMO for CHD was significantly lower than other classifications of diseases according to the Extracorporeal Life Support Organization database. This expert consensus aims to improve the survival rate and reduce the morbidity of this patient population by standardizing the clinical strategy. METHODS: The editing group of this consensus gathered 11 well-known experts in pediatric cardiac surgery and ECMO field in China to develop clinical recommendations formulated on the basis of existing evidences and expert opinions. RESULTS: The primary concern of ECMO management in the perioperative period of CHD are patient selection, cannulation strategy, pump flow/ventilator parameters/vasoactive drug dosage setting, anticoagulation management, residual lesion screening, fluid and wound management and weaning or transition strategy. Prevention and treatment of complications of bleeding, thromboembolism and brain injury are emphatically discussed here. Special conditions of ECMO management related to the cardiovascular anatomy, haemodynamics and the surgical procedures of common complex CHD should be considered. CONCLUSIONS: The consensus could provide a reference for patient selection, management and risk identification of perioperative ECMO in children with CHD. Video abstract (MP4 104726 kb).

Pharmacokinetics of treprostinil in children with functional single-ventricle pulmonary arterial hypertension: a randomized controlled trial.

BACKGROUND: Application of Treprostinil (TRE) in the patients with single ventricle (SV) physiology is very limited, and the optimal dose for children has not been determined. In this study, we aimed to analyze plasma samples to assess the attainment of clinically therapeutic concentrations of TRE and its efficacy and safety in the treatment of pediatric functional SV pulmonary arterial hypertension (FSV-PAH).. METHODS: Pediatric patients with FSV-PAH were recruited in this study. IV TRE at an initial rate of 5 ng/kg/min was administered through the femoral vein with an increase in rate to 10 ng/kg/min every 30 minuntil the aiming dose of 80 ng/kg/min had been reached. The drug was gradually discontinued after 12 h of treatment at a stable dose. The mean postoperative pulmonary artery pressure (mPAP), pulmonary-to-systemic arterial pressure ratio (Pp/Ps), and the ratio between arterial oxygen partial pressure and inhaled oxygen concentration (PaO2/FiO2) were used to evaluate the efficacy of TRE treatment. A multiple linear regression model was used to explore the relevant factors associated with TRE blood concentration. RESULTS: A total of eight patients were enrolled in the investigation, with an age range of 2.5-9.9 years. The median stable dose of TRE was 70 ng/kg/min with a range of 55-75 ng/kg/min. The median subliminal dose was 55 ng/kg/min with a range of 25-75 ng/kg/min. A linear relationship was established between the TRE dose and the plasma concentration. TRE blood concentrations were associated with dose and patient height. After TRE treatment, mPAP, Pp/Ps, and PaO2/FiO2 were significantly improved (P<0.05). CONCLUSIONS: A linear relationship was found between the blood concentration of TRE and its dose. IV TRE was an effective therapy without serious side effects in pediatric patients with FSV-PAH. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT02865733.

Feasibility of using a non-sedation protocol for evaluation of neonatal congenital heart disease by using a 16-cm wide-detector computed tomography with a low radiation dose: preliminary experience from a single pediatric medical center.

The aim of this study is to explore the feasibility of using a non-sedation protocol for the evaluation of neonatal congenital heart disease by using 16-cm wide-detector CT with a low radiation dose. Thirty-four neonates (group 1) were enrolled to undergo cardiac CT without sedation between August 2018 and March 2019. The control group (group 2) comprising 20 inpatient neonates was sedated. Cardiac CT was performed using 16-cm area detector 320-row CT with free breathing and prospective ECG-triggering scan mode. The examination completion time, radiation dose, and image quality were compared between the groups. The results of cardiac CT for patients in group 1 who underwent surgery were compared with surgical findings. Intergroup differences in body weight, age, examination completion time, radiation dose, and image quality evaluation were not significant. There was no significant difference in oxygen saturation before and after the examination in group 1. In all, 98 separate cardiovascular abnormalities in 27 group 1 patients were confirmed using surgical reports. The overall sensitivity, specificity, positive predictive value, and negative predictive value of cardiac CT were 94.90%, 100.0%, 100.0%, and 98.53%. The non-sedation protocol can be applied in neonates with congenital heart disease by using 16-cm wide-detector CT with a low radiation dose. Based on the image quality obtained, non-sedative examination did not extend the examination completion time and helped avoid the possible side effects of sedative drugs.

[Effect of prone or spine position on mechanically ventilated neonates after cardiac surgery with acute lung injury].

OBJECTIVE: To compare the efficacy and safety ventilated with pressure support ventilation (PSV) or neurally adjusted ventilatory assist (NAVA) in neonates undergoing open-heart surgery with acute lung injury (ALI) in spine and prone positions. METHODS: Fifteen neonates with a mean age of (15 +/- 9) days and a mean weight of (3.5 +/- 0.6) kg underwent open-heart surgery with ALI from July to December in 2009 were enrolled in this study. After hemodynamic stabilization ventilated with pressure regulated volume control (PRVC-base), all cases were ventilated with PSV and NAVA both in spine (SP) and prone (PP) positions for 60 minutes in a randomized crossover trial respectively. The hemodynamics, blood gas analysis, airway pressure, electrical activity of diaphragm (EAdi) and asynchrony index (AI) during every mode were recorded. RESULTS: The heart rate, systolic blood pressure and central venous pressure were stable in every mode. The peak inspiratory pressure and mean airway pressure in every mode had no significant difference but were significantly lower than in PRVC-base either in spine or prone position. The respiratory rate in PSV and NAVA with prone position was more rapid than in spine position and in PRVC-base (P < 0.05). But there was no significant difference in minute ventilation (MV) for each mode. The oxygenation index was higher in NAVA or PSV in both positions versus PRVC-base [(200 +/- 60) mm Hg in PRVC-base, (272 +/- 76) mm Hg in PSV-SP, (308 +/- 90) mm Hg in PSV-PP, (347 +/- 84) mm Hg in NAVA-SP and (365 +/- 87) mm Hg in NAVA-PP respectively, P < 0.01]. The oxygenation index was significantly higher in NAVA-PP than in PSV-SP (P < 0.05) while PaCO(2) was in normal range and had no significant difference for any mode. The minimal EAdi in NAVA-PP was significant lower than that in PSV-SP [(0.2 +/- 0.1) microV vs (0.5 +/- 0.2) microV, P < 0.05]. The AI of NAVA either in spine or in prone position was 0. It was significantly lower than that in PSV-SP [(21.5 +/- 4.8)%, P < 0.01] and PSV-PP [(22.4 +/- 3.4)%, P < 0.01]. CONCLUSION: Especially in a prone position, NAVA demonstrates a better synchrony in ALI neonates after cardiac surgery. It helps to provide a better oxygenation for the patients.

Neonatal surgical outcomes after prenatal diagnosis of complex congenital heart disease: experiences of a perinatal integrated diagnosis and treatment program.

BACKGROUND: This study aimed to evaluate neonatal surgical outcomes of patients diagnosed with complex congenital heart disease (CHD) during pregnancy and treated by the newly initiated "perinatal integrated diagnosis and treatment program (PIDTP)". METHODS: We reviewed clinical data of 207 neonates (surgical age ≤ 28 days) who underwent cardiac surgeries in a single center from January 2017 to December 2018, including 31 patients with referrals from the "PIDTP" (integration group) and 176 patients with routine referral treatment (non-integrated group). RESULTS: In the integration group, median admission age was 0 days and median age at surgery was 4 days. In the non-integrated group, median admission age was 8 days (P = 0.001) and median age at surgery was 13 days (P = 0.001). The emergency surgery rate in patients with duct-dependent defects was 36% in the integration group and 59% (P = 0.042) in the non-integrated group, respectively. The in-hospital mortality was 16% in the integration group and 14% (P = 0.78) in the non-integrated group. The 2-year cumulative survival rate after surgery was 83.9% ± 6.6% in the integration group and 80.3% ± 3.1% (P = 0.744) in the non-integrated group. According to multivariable regression analysis, independent risk factors for early mortality of overall neonatal cardiac surgery were low body weight, high serum lactate level, postoperative extracorporeal membrane oxygenation (ECMO) support and prolonged cardiopulmonary bypass (CPB) time. CONCLUSIONS: PIDTP shortens the postnatal transit interval, reduces the emergency operation rate of neonatal critical CHD, and provides better preoperative status for surgery. Patients treated by the PIDTP tend to have more complicated anatomical deformity and a greater requirement for the operation and postoperative management, but early outcome and follow-up prognosis are satisfactory.

[Outcome of oral bosentan in children with congenital heart disease associated pulmonary arterial hypertension].

OBJECTIVE: To investigate the outcome of dual endothelin receptor antagonist bosentan in children with congenital heart disease (CHD) associated pulmonary arterial hypertension (PAH). METHODS: A total of 32 children were recruited into this prospective and observational study. Among them, there were 18 cases with left-to-right shunt and 14 cases with elevated pulmonary vascular resistance (PVR) in functional single ventricle (FSV). All the cases were treated with oral bosentan, initiated from 90 days before or 8 years after operation, and were followed up periodically to analyze the clinical outcome and monitor its side effects. RESULTS: In the left-to-right shunt group, pulmonary arterial pressure (PAP) was measured at (57 +/- 26), (52 +/- 31) and (46 +/- 22) mm Hg after oral bosentan therapy at 1, 2 and 3 months respectively. The measurements significantly decreased as compared with the pre-dosing level of (74 +/- 15) mm Hg (P < 0.05). After a 3-months therapy of bosentan, World Health Organization functional class (WHO FC) improved significantly (P < 0.01). In the elder cases, the 6-minute walking distance after a 3-month bosentan therapy significantly increased as compared with the pre-dosing level, i. e. (497 +/- 56) vs (424 +/- 31) m (P < 0.05). In the FSV group, as compared with the pre-dosing level, the transcutaneous oxygen saturation increased significantly in the last follow-up during bosentan exposure, i. e. (86 +/- 5)% vs (78 +/- 6)% (P < 0.01). WHO FC improved significantly (P < 0.01) and the incidence of facial edema and pleural effusion was significant lower (P < 0.05) in the last follow-up for the treatment group. Patients tolerated bosentan well and no significant rise in hepatic transaminases was observed. CONCLUSIONS: Bosentan is safe in treating CHD associated PAH in children. In left-to-right shunt cases, oral bosentan can reduce PAP and improve both WHO FC and exercise capacity. And it can also improve WHO FC and transcutaneous oxygen saturation in FSV and reduces the occurrence of elevated PVR-related complications.

[Application of neurally adjusted ventilatory assist in infants who underwent cardiac surgery for congenital heart disease].

OBJECTIVE: Neurally adjusted ventilatory assist (NAVA) is a new mode of mechanical ventilation that delivers ventilatory assist in proportion to neural effort. This study aimed to compare the hemodynamic safety, oxygenation and gas exchange effects ventilated with NAVA and with pressure support ventilation (PSV) in infants who underwent open-heart surgery. METHODS: Twenty-one infants who underwent open-heart surgery for congenital heart disease (mean age 2.9+/- 2.1 months and mean weight 4.2+/- 1.4 kg) were enrolled. They were ventilated with PSV and NAVA for 60 minutes respectively in a randomized order. The hemodynamic, oxygenation and gas exchange effects produced by the two ventilation modes were compared. RESULTS: Three cases failed to shift to NAVA because of the bilateral diaphragmatic paralysis after operation. In the other 18 cases, there were no significant differences in the heart rate (HR), systolic blood pressure (BPs) and central venous pressure (CVP) in the two ventilation modes. The PaO2/FiO2 (P/F) ratio in NAVA was slightly higher than in PSV, but there was no statistical difference. PaCO2 did not show significant differences in the two modes. The peak inspiratory pressure (PIP) and electrical activity of the diaphragm (EAdi) in NAVA were significantly lower than in PSV. The EAdi signal after extubation was higher in infants who needed reintubation or intervention of noninvasive mechanical ventilation than in those who were extubated successfully (30.0+/- 8.4 microV vs 11.1+/- 3.6 microV; P<0.01). CONCLUSIONS: As the first study of application of NAVA in infants in China, this study shows that NAVA has the same homodynamic effects as PSV. However the PIP for maintaining the same level of PaCO2 in NAVA is significantly lower than that in the traditional PSV. Monitoring the EAdi signal after extubation may show the risks of reintubation or intervention of noninvasive mechanical ventilation.

Nutritional outcomes in infants with food allergy after cardiac surgery.

BACKGROUND: Food allergy is a rapidly growing public health concern because of its increasing prevalence, as well as life-threatening potential. There is limited knowledge on the nutritional status for the pediatric congenital heart disease (CHD) patients with food allergy. OBJECTIVE: This study investigated both clinical and nutritional outcomes according to the CHD infants with food allergy. METHODS: Forty CHD infants with food allergy and 39 controls were recruited in Shanghai, China. The height and weight for age and weight for height were converted to z-scores to evaluate their effects on nutritional status before and after CHD operation. RESULTS: Cow's milk showed the most frequently sensitized food allergen. The WHZ in the ≥2 allergen group was different before operation and after operation (P = .040). The number of sensitized food allergens significantly correlated with the WHZ (r = -0.431, P = .001), WAZ (r = -0.465, P = .000), and HAZ (r = -0.287, P = .025). Infection and NT-BNP showing negative correlation with WHZ and WAZ of infants with food allergy. CONCLUSIONS: The increased number of sensitized food allergens is associated with negative effects on both short-term and long-term nutritional status in infants with CHD. Therefore, the meticulous and continuous evaluation and management of both growth and nutritional status should be considered in CHD patients with a high number of sensitized food allergens.

[Postoperative complications after orthotopic liver transplantation: clinical analysis of 18 cases].

OBJECTIVE: To investigate experience in diagnosis and treatment of postoperative complications in patients undergoing orthotopic liver transplantation (OLT). METHODS: Complications, treatment and management following liver transplantation in 16 cases were analyzed retrospectively. RESULTS: Of 16 patients, 5 patients had advanced liver cirrhosis, 7 primary liver carcinoma, 1 liver failure after hepatectomy for liver cancer, 1 Wilson's disease, 1 chronic renal failure and liver cirrhosis and 1 acute live failure. Twelve patients survived, the longest survival was 4 years. Complications following OLT included: intra-abdominal bleeding in 3 cases, intracerebral vascular lesions in 2, pulmonary infection in 6, adult respiratory distress syndrome in 2, suprahepatic inferior vena caval occlusion in 2, hepatic artery thrombus in 1 case, bile duct stone or sludge in 3, bile leakage in 1 case, acute rejection in 2, chronic rejection in 2, acute renal failure in 2. Six patients died during perioperative period, one patient died of intracerebral bleeding, one from adult respiratory distress syndrome, one of acute renal failure one of hepatic artery thrombus, one of acute rejection and one of liver failure. CONCLUSIONS: Proper prevention and treatment can effectively reduce complications following OLT during perioperative period. The timely diagnosis, treatment and prophylactics are necessary to prevent these complications.

[Clinical experience with 13 cases of orthotopic liver transplantation].

OBJECTIVE: To review our experience with orthotopic liver transplantation. METHODS: Thirteen liver transplantation were performed in 12 patients (including one liver retransplantation), of whom 5 patients received the transplantation for end stage liver cirrhosis, 4 for primary liver carcinoma, 1 for liver failure after hepatectomy for liver cancer, 1 for Wilson's disease, and 1 for chronic renal failure and liver cirrhosis. Retransplantation was done in 1 patient for chronic graft rejection. Of the 13 operations, 10 underwent the classical procedures of orthotopic liver transplantation, while 2 adopted modified piggyback technique, with 1 of the patients receiving retransplantation. RESULTS: Nine patients survived the transplantation with the longest survival over 2 years. Four patients died in the perioperative period, due to intracerebral bleeding, adult respiratory distress syndrome, acute renal failure and hepatic artery thrombus, respectively. CONCLUSIONS: Liver transplantation is an effective treatment for various end-stage liver diseases. Strict patient selection, appropriate timing of the operation and proper perioperative care are all essential for the success of liver transplantation.

[Evaluation on the early hemodynamic changes after cardiac surgery for congenital heart diseases in neonates].

OBJECTIVE: To accurately evaluate the early hemodynamic status of neonates who undergo complex neonatal cardiac surgery, through monitoring the cardiac index (CI), serum lactate (Lac), mixed venous oxygen saturation (SvO(2)). METHODS: From January to November 2007, haemodynamic data of 80 patients who had open heart surgery for congenital heart disease were analyzed within 48 hours after operation. Of the 80 patients, 47 were neonates, their age ranged from 3 days to 29 days [mean (21.98 + or - 8.15) days] and weight ranged from 2.6 kg to 4.2 kg [mean (3.51 + or - 0.39) kg]. As the control group, 33 young infants at the age of 30 days to 180 days [mean (76.36 + or - 24.79) days] with body weight ranged from 3.1 kg to 6.0 kg [mean (4.59 + or - 0.59) kg] were also enrolled. The value of CI derived from pulse contour and was calculated by using the PiCCO system. Meanwhile, measurements of serum lactate level and SvO(2) were recorded. Serial measurements of the cardiac output were performed for the neonates. RESULTS: CI in survivors of neonates (2.01 + or - 0.35) L/(min x m(2)) was lower than that of the infants (2.26 + or - 0.39) L/(min x m(2)) after cardiac surgery (P < 0.05) at 2 h, 6 h postoperatively. However, urine output remained normal. The value of pulse pressure in neonates was less than that in young infants. Serum lactate level in neonates was significantly higher than that of young infants during cardiac surgical procedures (P < 0.01) at 12 h postoperatively; the SvO(2) was more than 60% postoperatively in survived neonates, there was no significant difference (P > 0.05) in SvO(2) between neonates and young infants during preoperative and postoperative periods. There was a positive correlation between CI and SvO(2). Four neonates and 1 young infant died after surgical treatment, surgical mortality was 8.5% and 3.0%, respectively. The deaths of the neonates were related to the cardiocirculatory function decompensation, unrelieved severe acidosis preoperatively, and the transposition of great artery with coronary artery malformation and longer cardiopulmonary bypass. The patients with significantly high arterial blood lactate levels during the first 6 - 12 hours postoperatively had poor outcome. Lactate levels were higher than 10 mmol/L and SvO(2) less than 50% in neonates who developed multiple organ system failure. One young infant died of sudden arrhythmia after surgical treatment, whose death may be related the surgical procedure itself with pulmonary artery banding and blalock-taussig shunt leading to increased preload and afterload of the heart. CONCLUSIONS: Elevated serum lactate level postoperatively may reflect intraoperative tissue hypoperfusion. Serial blood lactate level measurements may be an accurate predictor of clinical outcomes in children after pediatric open heart surgery. Mixed venous oxygen saturation changes more rapidly than other standard hemodynamic variables. The higher mortality of neonates with congenital heart disease is related to the malformation complexity itself and illness severity.

Survey of the use of peripherally inserted central venous catheters in neonates with critical congenital cardiac disease.

Neonates with congenital cardiac disease are a special population. They are often critically ill, and need prolonged intravenous access. To date, no study has evaluated the efficacy and safety of peripherally inserted central venous catheters placed in this unique population. Our goal was to evaluate the use of such catheters in neonates with critical congenital cardiac disease, and to study features such as duration of use, reasons for removal of catheters, and complications. We inserted a total of 124 catheters in 115 neonates with critical congenital cardiac disease who were admitted to the Intensive Care Unit at Texas Children's Hospital from August 2002 to August 2004. The patients had a mean age of 10 days, and a mean weight of 3.1 kilograms. The peripherally inserted catheters were in place for a mean of 22.3 days. Therapy was completed in 76.6% patients at the time of removal of the catheter. The incidence of occlusion, dislodgement, and thrombus was 4.0%, 2.4%, and 1.6%, respectively. The infection rate was 3.6 per 1000 catheter-days, with a median onset on 37 days after placement. We conclude that central venous catheters, when inserted peripherally, provide reliable and safe access for prolonged intravenous therapy in neonates with critical congenital cardiac disease.

[Percutaneous radiofrequency ablation combined with transcatheter arterial chemoembolization and percutaneous ethanol injection for recurrent small hepatocellular carcinoma].

OBJECTIVE: To explore the value of percutaneous radiofrequency ablation (PRFA) combined with transcatheter arterial chemoembolization (TACE) and percutaneous ethanol injection (PEI) in the management of recurrent small hepatocellular carcinoma. METHODS: Between March 2001 and March 2005, 52 patients with recurrent hepatocellular carcinoma (tumor size< or =5 cm) underwent PRFA, and 14 of the patients (tumor size 3-5 cm) also received TACE and PEI, and their clinical data were analyzed retrospectively. RESULTS: MRI or CT after PRFA revealed complete coagulative necrosis of the tumor in 38 cases (tumor size <3 cm). In the 14 patients (tumor size 3-5 cm) with also TACE and PEI, complete necrosis occurred in 11 cases (78.6%). In the patients involved in this study, the 1-, 2-, 3- and 4-year survival rates were 96.2%, 69.4%, 45.5% and 30.0%, respectively. CONCLUSIONS: PRFA is an effective modality for local treatment of recurrent small hepatocellular carcinoma, capable of total elimination of tumors <3 cm. For tumors of 3-5 cm, combination with TACE and PEI may help increase the tumor necrosis rate following the ablation and raise the patients' survival rate.