Fontan-associated liver disease current status and transplantation consideration / 中华肝脏病杂志

Fontan-associated liver disease (FALD) is one of the main complications after the Fontan procedure, manifesting mostly as liver fibrosis and even cirrhosis, with a high incidence rate and a lack of typical clinical symptoms that seriously affect patient prognosis. The specific cause is unknown, although it is considered to be associated with long-term elevated central venous pressure, impaired hepatic artery blood flow, and other relevant factors. The absence of association between laboratory tests, imaging data, and the severity of liver fibrosis makes clinical diagnosis and monitoring difficult. A liver biopsy is the gold standard for diagnosing and staging liver fibrosis. The most important risk factor for FALD is time following the Fontan procedure; therefore, it is recommended to do a liver biopsy 10 years after the Fontan procedure and to be cautious for the presence of hepatocellular carcinoma. Combined heart-liver transplantation is a recommended choice with favorable outcomes for patients with Fontan circulatory failure and severe hepatic fibrosis.

Treatment and prognosis of 826 infants with critical congenital heart disease: a single center retrospective study / 中华心血管病杂志

Objective: To analyze the current status of clinical treatment and factors influencing postoperative mortality in infants with critical congenital heart disease (CCHD) in China, optimize the perioperative management of CCHD, and provide a new scientific basis for clinical decision-making for the optimal management of these patients. Methods: This is a retrospective single-center study. Infants diagnosed with CCHD in Guangdong Provincial People's Hospital from January 2017 to December 2019 (aged 0-1 years at admission) were enrolled. General clinical information, inpatient treatment information, prognosis and complications were collected and analyzed. Multivariate logistic regression analysis was used to explore the independent risk factors of postoperative death in infants with CCHD. Results: A total of 826 infants with CCHD were included, including 556 males (67.3%) and the age at first admission was 51.0 (5.0,178.3) days. 264 (32.0%) cases were tetralogy of Fallot and 137 (16.6%) cases were total anomalous pulmonary venous return. 195 cases (23.6%) were diagnosed prenatally. 196 cases (23.7%) were treated with prostaglandin. The preoperative invasive ventilation time was 0 (0, 0) hour, and the postoperative invasive ventilation time was 95.0 (26.0, 151.8) hours. A total of 668 cases (80.9%) underwent surgical treatment. The age was 100.5 (20.0, 218.0) days during operation and the operation time was 190.0 (155.0, 240.0) hours. Sixty-two cases (7.5%) received medical treatment, and 96 cases (11.6%) gave up treatment. A total of 675 cases (81.7%) were discharged with improvement, 96 cases (11.6%) were discharged after giving up treatment, 55 cases (6.7%) died and 109 cases (13.2%) were readmitted within one year. Complications occurred in 565 (68.6%) cases, including pneumonia in 334 cases (40.4%) and cardiac arrhythmias in 182 cases (22.0%). Multifactorial analysis showed that delayed chest closure (OR=49.775, 95%CI 3.291-752.922, P=0.005), prolonged post-operative invasive ventilator ventilation (OR=1.003, 95%CI 1.000-1.005, P=0.038) and cardiac hypoplasia syndrome (OR=272.658, 95%CI 37.861-1 963.589, P<0.001) were the independent risk factors for mortality in CCHD infants post-operation. Conclusions: Tetralogy of Fallot and total anomalous pulmonary venous return account for the majority of infants with CCHD. The proportion of infants diagnosed prenatally was less than 1/4. The majority CCHD infants received surgical treatment. The main complications are pneumonia and arrhythmia. Delayed chest closure, prolonged postoperative invasive ventilator ventilation and low cardiac output syndrome are the independent risk factors for postoperative death in infants with CCHD.

Current prevalence rate of congenital heart disease in 12 month-old and younger infants among four regions of Guangdong province / 中华心血管病杂志

<p><b>OBJECTIVE</b>To summarize prevalence rate and region distribution of congenital heart disease (CHD) in 12-month-old and younger infants among four regions of Guangdong province, China.</p><p><b>METHODS</b>Data from Guangdong CHD monitoring network including 34 monitoring units covering different geographic regions were analyzed. Professional training on screening and diagnosing CHD was provided to each work group member to improve the diagnosis level. CHD infants under or aged 12 months detected in the monitoring unit were included. CHD was diagnosed by fetus and infants echocardiography.</p><p><b>RESULTS</b>From July 2004 to December 2010, 383 281 perinatal were registered and 3263 cases of CHD were detected in the 34 member units of Guangdong CHD monitoring network [total prevalence rate of CHD: 0.851% (3263/383 281), male prevalence rate: 0.868% (1799/207 347), female prevalence rate:0.828% (1456/175 843)].Stillbirth CHD prevalence rate was significantly higher than livebirth CHD prevalence rate [10.627% (676/6361) vs. 0.686% (2587/376 920), P < 0.01]. The total prevalence of CHD was significantly higher in Pearl River Delta region [0.906% (2826/311 823)] than in other regions [0.611% (437/71 458), P < 0.01]. Ventricular septal defect [39.93% (1033/2587) in livebirth] was the most dominant CHD, followed by patent ductus arteriosus [29.84% (772/2587)] and secundum atrial septal defect [13.76% (356/2587)].</p><p><b>CONCLUSIONS</b>The present data indicate that the prevalence of CHD in Guangdong is at the medium-upper level of the country associated with high stillbirth rate. The dominant type of CHD is ventricular septal defect. CHD prevalence is higher in the Pearl River Delta region than in other regions.</p>

Study on the differences of risk factors regarding congenital heart defects between floating population and permanent residents in Guangdong / 中华流行病学杂志

<p><b>OBJECTIVE</b>To analyze the differences of risk factors on congenital heart defect (CHD)between floating population and permanent residents in Guangdong.</p><p><b>METHODS</b>A multicenter case-control study was carried out to investigate the risk factors of CHD in floating population and in permanent residents. Data was from 34 Guangdong CHD Monitoring Network centers during the year of 2004 to 2011. Exposed information related to the parents at pre-pregnancy and early pregnancy periods, was collected, using the same questionnaire survey methodology in the two populations. Possible risk factors were analyzed by univariate analysis and multivariate non-conditional logistic regression(ENTER method)methods. Risk factors were compared between the two populations.</p><p><b>RESULTS</b>Totally, 855 CHD cases and their controls from the floating population, as well as 1673 cases and their controls from the permanent residents were included in this study. Age of the children under study was defined from 28th week of gestation to 1 year old postnatal. In the floating population, specific risk factor for CHD appeared as:maternal passive smoking in early pregnancy, while the specific protective factor as high family income. However, the specific risk factors would include: having diseases as maternal diabetes mellitus or syphilis, living in a newly (within half a year) decorated house or with fetal macrosomia in the permanent residents. High education level showed as a risk factor in floating population, however contrarily, as protective factor to the permanent residents. Except for the factors related to having fever of the mother and infant with low birth weight, factors as having history of deliveries more than two, with maternal virus infection, exposure to chemical agent and negative bearing history etc., have higher OR values in floating population than in the permanent residents.</p><p><b>CONCLUSION</b>Significant differences of risk factors for CHD were noticed between floating population and the permanent residents, which have their individual specific risk factors. Most of the ORs appeared higher in floating population than in the permanent residents.</p>

Protective effect of retrograde venous perfusion of cryogenic liquid via accessory hemiazygos vein and treated with resveratrol on spinal cord injury in swine / 中华外科杂志

<p><b>OBJECTIVES</b>To observe the protective effect of retrograde venous perfusion of cryogenic liquid via accessory hemiazygos vein and treated with resveratrol on spinal cord injury and evaluate the expression changes of microtubule-associated protein 2 (MAP-2) after spinal cord ischemia reperfusion injury (SCII) in swine.</p><p><b>METHODS</b>Eighteen swine were divided into 3 groups: group I/R (n = 6, operation group), group CL (n = 6, retrograde venous perfusion of cryogenic liquid), group CL+Res (n = 6, retrograde venous perfusion of cryogenic liquid and treated with resveratrol after ischemia). In the group I/R, the aorta was clamped for 60 minutes and then removed. In the group CL and CL+Res, 9 g/L cold (4 °C) saline solution (perfusion rate, 16.65 ml/min) was infused into the accessory hemiazygos vein during ischemia.In the group CL+Res, the swine were treated with resveratrol (10 mg/kg) after spinal cord ischemia. Arterial pressure, blood gas analysis and the spinal canal and nasopharyngeal temperature changes were monitored during the surgery. Nervous function were assessed at 6 hours, 1, 2 days, 1, 2, 4 weeks and MAP-2 expression were detected at 4 weeks after reperfusion by using Western blot analysis in spinal cord tissue.</p><p><b>RESULTS</b>After operation 18 swine were all survival. Behavioral scores of all groups decreased until 1 week after reperfusion and increased as time went by. The scores of group CL and CL+Res were higher than group I/R (F = 8.612, 17.276 and 11.985, P = 0.035,0.011 and 0.023) at 6 hours, 1, 2 days, group CL+Res were higher than group CL(P = 0.021) at 1 days after surgery. After descending aortic cross clamping, the spinal canal and nasopharyngeal temperature were obviously decreased in all groups and dropped to the lowest at 60 minutes after ischemia and 20 minutes after reperfusion in group I/R and the other groups respectively(F = 23.187-55.029, P < 0.01).In group CL(0.54 ± 0.26) and CL+Res (0.66 ± 0.31), the MAP-2 expression were higher than group I/R(0.37 ± 0.18) (F = 9.381, P = 0.037) , and the level in group CL+Res was higher than in group CL (P = 0.021) .</p><p><b>CONCLUSION</b>Retrograde venous perfusion of cryogenic liquid via accessory hemiazygos vein and treated with resveratrol can relieve the ischemia-induced spinal cord injury in swine.</p>

Risk factors of congenital heart defects in fetal and infants born from 2004 to 2011 in Guangdong / 中华心血管病杂志

<p><b>OBJECTIVE</b>To analyze the risk factors of congenital heart defects (CHD) in fetal and infants born from 2004 to 2011 in Guangdong province.</p><p><b>METHODS</b>Babies with CHD aged from 28th week of gestation to 1 year old postnatal from July 1 2004 to December 31 2011 were registered in Guangdong CHD monitoring network with 34 participating units. Totally 2568 CHD cases were included, and 1: 1 matched with a normal control cohort by gender, living district and birth date (time span within 3 months).Exposed information of mother and father at pre-pregnancy and early pregnancy was collected. Post collinearity diagnostics analysis, univariate analysis results were included in a multivariate analysis model with forward stepwise conditional logistic regression.</p><p><b>RESULTS</b>Multivariate conditional logistic regression analysis showed that high risk factors for CHD included low birth weight infant (OR = 5.34, P < 0.01), macrosomia (OR = 1.67, P < 0.05), low per capita income (0-1200 yuan, OR = 1.68, P < 0.01), exposure to chemical agent at early pregnancy (OR = 19.72, P < 0.01), floating population (OR = 2.13, P < 0.01), abnormal reproductive history (OR = 3.18, P < 0.01), exposure to passive smoking (OR = 2.59, P < 0.01), suffering from fever (OR = 3.74, P < 0.01), equal to or more than twice parity (OR = 1.45, P < 0.01), living in a newly (within six months)-decorated-apartment (OR = 2.74, P < 0.01), suffering from virus infection (OR = 2.08, P < 0.01), rural residence (OR = 1.33, P < 0.01), living in an apartment within 50 meters of major traffic road (OR = 1.52, P < 0.01), syphilis infection at early pregnancy (OR = 13.06, P < 0.05) and father's drinking habit at pre-pregnancy (OR = 1.57, P < 0.05).</p><p><b>CONCLUSION</b>Numerous risk factors for CHD in fetal and infants of Guangdong province are indicated by our results, comprehensive intervention should be considered in pre-pregnancy and early pregnancy to reduce the risk of CHD.</p>

Effects of different arterial oxygen partial pressures on serum protein S100β and neuron specific enolase during cardiopulmonary bypass in infants with cyanotic congenital heart disease / 中华儿科杂志

<p><b>OBJECTIVE</b>A prospective study was conducted to probe into the relationship between arterial oxygen partial pressure (PaO2) and brain injury during cardiopulmonary bypass (CPB) in infants with cyanotic congenital heart disease (CHD).</p><p><b>METHOD</b>Enrolled in the study were 45 cyanotic infants, who were less than three years old and underwent corrective cardiac surgery from August 1(st), 2010 to January 31(st), 2011 at Guangdong General Hospital. All the infants had a pulse oxygen saturation (SpO2) lower than 85% and were randomly allocated into three groups by a specific computer program. In controlled group 1 (G1 group), PaO2 levels were controlled at 80 - 120 mm Hg (1 mm Hg = 0.133 kPa) during CPB; in controlled group 2 (G2 group), PaO2 levels at 120 - 200 mm Hg during CPB; while in uncontrolled group (G3 group), PaO2 levels were at 200 - 400 mm Hg during CPB. Blood samples were collected just before starting CPB, at the end of CPB, and at 3 h, 5 h, and 24 h after CPB (T1, T2, T3, T4, T5) for the determination of serum concentrations of protein S100β, neuron specific enolase (NSE), and adrenomedullin (ADM) by ELISA.</p><p><b>RESULT</b>Protein S100β rose significantly after starting CPB. In group G3, it reached a peak of (699 ± 139) ng/L by the end of CPB, significantly higher than those in groups G1 and G2 [(528 ± 163) ng/L and (585 ± 155) ng/L], and was positively correlated with PaO2 levels (r = 0.526, P < 0.01). NSE levels of group G1 were continuously rising after starting CPB and reached significantly high levels at 3 h or 5 h after CPB [(12.2 ± 3.4) µg/L and (12.3 ± 3.7) µg/L], while those of group G2 rose significantly during CPB [(10.9 ± 4.8) µg/L] and even higher at 3 h or 5 h after CPB [(12.6 ± 5.1) µg/L and (13.2 ± 5.4) µg/L]. NSE levels of group G3 rose significantly during CPB and maintained at a high level [(12.2 ± 5.7) µg/L] afterwards. There was no significant difference in serum ADM concentrations among different time points in each group and among these three groups. All the infants were discharged from the hospital without any obvious nervous symptom and sign.</p><p><b>CONCLUSION</b>High PaO2 during CPB in infants with CHD might cause an increase of serum protein S100β and NSE, indicating that brain injury might become worse with a higher PaO2 and might be positively correlated with PaO2 during CPB.</p>

Accuracy of the ultrasonic cardiac output monitor in healthy term neonates during postnatal circulatory adaptation / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Echocardiography is regarded as a gold standard for measuring hemodynamic values. The ultrasonic cardiac output monitor (USCOM) is a new method for measuring hemodynamics and could provide non-invasive point of care guidance. So far, there are no published USCOM reference values for neonates, nor has USCOM's accuracy been established in this population. We aimed to determine the accuracy and clinical utility of the USCOM in healthy neonates relative to published echocardiographic data, to establish normal hemodynamic parameters that it measures, and to assess the possible role of USCOM as an alternative to echocardiography as a trend monitor.</p><p><b>METHODS</b>Right and left heart hemodynamics of 90 normal neonates were measured during circulatory adaptation over the first three days of life using the USCOM and automated oscillotonometry.</p><p><b>RESULTS</b>Heart rate showed a significant decline from days one to three, from 126 to 120 (P < 0.001). Systolic, diastolic and mean arterial pressures all increased significantly from 66 to 71 mmHg, 33 to 38 mmHg and 44 to 49 mmHg, respectively (P < 0.001 in each case). Right ventricular cardiac index (RV-CI) showed no change with a mean of 5.07 L × min(-1) × m(-2). Left ventricular cardiac index (LV-CI) declined from 3.43 to 3.00 L × min(-1) × m(-2) (P < 0.001). RV-CI exceeded LV-CI on all three days by a mean of 61%. The systemic vascular resistance index (SVRI), based on LV-CI, increased significantly over the three days from 1083 to 1403 dyne × sec × cm(-5) × m(2) (P < 0.001).</p><p><b>CONCLUSIONS</b>Normal neonatal hemodynamic values, as indicated by USCOM, were established. LV-CI measurement showed excellent agreement with published echocardiographic studies. RV-CI was constant and exceeded LV-CI for all three days of this study. It may be falsely high due to flow velocity measurement errors arising from the pulmonary branch arteries, and may represent a limitation of the USCOM method. The progressive rise of arterial pressure and SVRI despite a declining LV-CI may indicate functional closure of the ductus arteriosus, with the greatest change occurring within the first 24 hours. Evidence of closure of the foramen ovale was not observed.</p>

Early and mid-term results after 17 mm St Jude Regent mechanical valve replacement in 44 patients with small aortic root / 南方医科大学学报

<p><b>OBJECTIVE</b>To analyze the changes in the cardiac function after St. Jude Regent mechanical valve replacement and assess the prosthesis-patient matching.</p><p><b>METHODS</b>From October 2007 to March 2009, 44 patients received implantation of 17 mm St. Jude aortic prostheses in our hospital. The patients were followed up for clinical symptoms, signs, electrocardiogram (ECG), echocardiogram and cardiac functions, and the results were compared with those of randomly selected 44 patients receiving 21 mm St. Jude aortic prostheses.</p><p><b>RESULTS</b>In 17 mm St Jude Medica Regent valve group, 8 patients presented with ECG ST segment changes, 3 complained of chest tightness, 3 had occasional chest pain and discomfort, and 8 had grade II and 4 grade III cardiac function. In 21 mm St Jude Medical Regent valve group, 6 patients had ECG ST segment changes, 2 complained of chest tightness, 2 reported occasional chest pain and discomfort, 11 had grade II and 2 grade III cardiac function. No significant differences were found in these indices between the two groups (P=0.32). Compared with those before operation, the two groups showed significant improvements in the left ventricular end-diastolic diameter, left ventricular posterior wall thickness, left ventricular mass index, and aortic pressure gradient (P<0.05). A significant increase in the left ventricular ejection fraction occurred 6-12 months after operation, but without statistical difference between the two groups (P>0.05).</p><p><b>CONCLUSION</b>For underweight patients (<60 kg) and those with small body surface area (<1.6 cm(2)), 17 mm St. Jude Medical Regent valve prosthesis may produce good therapeutic effect, and some indices are even close to those after placement of 21 mm St. Jude Medical Regent valve prosthesis. No obvious prosthesis-patient mismatch occurs after the placement of the 17 mm valve prosthesis and aortic valve ring expansion is not necessary.</p>

Surgical treatment of 128 cases of constrictive pericarditis / 南方医科大学学报

<p><b>OBJECTIVE</b>To summarize the experience with surgical treatment of constrictive pericarditis.</p><p><b>METHODS</b>A retrospective analysis of the post-operative clinical data was conducted in 128 surgical patients with chronic constrictive pericarditis.</p><p><b>RESULTS</b>Two early postoperative death occurred in this group due to severe low cardiac output syndrome, with the mortality rate of 1.57%. The postoperative complications included low cardiac output syndrome (13.2%), arrhythmia (7.02%), acute renal insufficiency (3.9%), respiratory insufficiency (3.1%), wound infection (2.3%), postoperative chest bleeding (1.6%) and cerebral infarction (0.78%). Relapse occurred in one case because of incomplete pericardial resection.</p><p><b>CONCLUSIONS</b>Constrictive pericarditis should be confirmed as soon as possible with actively surgery, and the extent of pericardial resection should be decided according to the individual conditions. Complete untethering of the diseased pericardium should be performed with active prevention of postoperative complications.</p>

The application of bidirectional Glenn procedure on 68 pediatric cases with functional univentricle heart / 中华外科杂志

<p><b>OBJECTIVE</b>To analyze 68 pediatric cases with functional univentricle heart who underwent bidirectional Glenn procedure during from April 1998 to December 2005.</p><p><b>METHODS</b>There were 47 males and 21 females in this group, aged from 5 months to 14 years old and weighed from 6.7 to 30.0 kg. Among them, 39 cases were received bidirectional Glenn procedure on the right side, 13 cases on the left side and 16 cases on both sides. Three cases had the pulmonary artery banded; one case had the pulmonary artery ligated;one case had the original A-P shunt cut off; six cases had the PDA ligated; four cases had the MAPCAs cut off; one case had TAPVC corrected contemporarily; two cases of PAPVC were also corrected; four cases had the atrial-ventricular valve repaired.</p><p><b>RESULTS</b>Three cases died. The mortality was 4.4%. The mean post-operative pressure of super vena cava was (15.9 +/- 2.4) mm Hg (1 mm Hg = 0.133 kPa), higher than the pre-operative one (8.3 +/- 1.8) mm Hg (P < 0.01). The mean post operative SpO(2) was (89.3 +/- 4.2)%, higher than the pre-operative one (78.4 +/- 6.0)% (P < 0.01).</p><p><b>CONCLUSIONS</b>Bidirectional Glenn procedure is of satisfied effect on surgical treatment for functional univentricle heart. The persistent forward flow from pulmonary artery should be reserved in bidirectional Glenn procedure.</p>

Diagnosis and Management of Vascular Ring Combined with Tracheal Compression in Infants and Neonates / 实用儿科临床杂志

Objective To explore the diagnosis and therapy experience of vascular ring combined with tracheal compression in infants and neonates.Methods Sixteen cases(including 7 boys and 9 girls,aged 1 day to 12 months)with vascular ring combined with tracheal compression hospitalized in Guangdong General Hospital from Jun.2004 to Dec.2009 were enrolled.In these 16 children,13 cases had congenital heart malformations.All children underwent X-ray,echocardiography and spiral computed tomography examination.Nine cases received bronchoscopy study.Fifteen cases performed surgical division of vascular ring with cardiopulmonary bypass and 1 case underwent vascular ring division and tracheoplasty.Eleven cases received management of congenital heart defect simultaneously.Results Vascular ring anomalies included pulmonary artery sling in 5 children,right aortic arch-left ligmentum/aberrant left subclavian artery in 8 cases,double aortic arch in 1 case,innominate artery compression in 1 case,and pulmonary sling combined with right aortic arch-aberrant left subclavian artery in 1 case.There were 2 ring-sling complex cases in this study.The diagnosis of vascular ring were correctly made by echocardiography in 7 children and made by spiral computed tomography in all 16 cases.Two cases combined with tracheal ring died.In the follow-up study of 11 cases,5 cases were still vulnerable to wheezing.Conclusions The common presentation of tracheal compression in infants and neonates associated with vascular ring are tachypea,stridor,and dyspnea.Multi-slices spiral computed tomography is an important imaging modality.Surgical divisions of vascular ring are safe procedure in most cases and tracheal compression can be relieved by this operation.In patients with severe tracheal stenosis,tracheoplasty should be recommended.