Subepicardial lipoma of the posterior atrioventricular sulcus in children: a case report and review of the literature.

Cardiac lipoma is rarely reported in the pediatric population. We reported a case of subepicardial lipoma of the posterior atrioventricular sulcus in a child. The tumor was resected successfully and the patient recovered well after the operation.

Intraatrial conduit Fontan procedure: indications, operative techniques, and clinical outcomes.

BACKGROUND: The intraatrial conduit (IAC) Fontan procedure is one of the Fontan modifications and is usually not considered the first choice. In this large series, we report our experience of the IAC Fontan procedure for the treatment of a functional single ventricle and review its indications, techniques, and clinical outcomes. METHODS: Between 2009 and 2013, 101 patients with a functional single ventricle underwent an IAC Fontan procedure. The median surgical age was 44 months and weight was 15 kg. The cardiac malformations included tricuspid atresia in 10, double-inlet left ventricle in 8, double-inlet right ventricle in 15, double-outlet right ventricle in 18, congenitally corrected transposition of the great arteries in 11, complete atrioventricular septal defect in 8; criss-cross in 1, pulmonary atresia with intact ventricular septum in 3, Ebstein anomaly in 3, and others in 3. Heterotaxy syndrome was found in 21 patients. An intraatrial polytetrafluoroethylene conduit was implanted to construct the Fontan pathway. In 75 patients, a 2.7-mm to 4.5-mm fenestration was made to reduce the intraconduit pressure. Eighty-three patients had previously undergone a Glenn operation. Median follow-up was 29 months (range, 2 to 60 months). Doppler echocardiography, electrocardiography, and Holter monitoring were used to evaluate hemodynamic performance and arrhythmias. RESULTS: There were 2 hospital deaths, 1 Fontan takedown, and 3 midterm deaths. Conduit thrombosis developed in 1 patient. Atrial flutter developed in 2 patients, who underwent electrical cardioversion. Junctional bradycardia developed in 2 patients, but they did not require permanent pacemakers. Overall survival was 97.0% at 1 year and 94.1% at 5 years. CONCLUSIONS: The IAC modification provides excellent operative and midterm outcomes in most patients with a functional single ventricle. However, a longer follow-up time is required to demonstrate its real advantages.

[Visualization of the age-related changes in expressions of DNA methyltransferases in mouse oocytes using two-photon imaging system].

Quantum dot (QD) is widely used as fluorescent labeling dye for its strong anti-fluorescence quenching, high quantum yield, wide absorption spectra, and narrow emission spectra. In the present paper, QD 585-labeled DNA methyltransferases (Dnmts) and Hoechst 33342-labeled chromosomes were imaged simultaneously using two-photon imaging system. The authors' results showed that aging mouse oocytes may be not suitable for in-vitro maturation: both the localizations and expression levels of Dnmts in in-vitro matured oocytes of aging mice were changed, and these changes may be related to the abnormal DNA methylation modification.

Involvement of a novel GATA4 mutation in atrial septal defects.

Atrial septal defect (ASD) is one of the most common types of congenital heart disease and is associated with a significant increase in the morbidity and mortality of affected individuals. Accumulating evidence indicates that genetic defects play important roles in the pathogenesis of congenital ASD. However, ASD is genetically heterogeneous and the genetic determinants for ASD in the majority of the patients remain to be identified. In this study, the entire coding region of GATA4, a gene encoding a zinc-finger transcription factor crucial to embryogenesis, was initially sequenced in 120 unrelated patients with ASD. The available relatives of patients carrying the identified mutation and 200 ethnicity-matched unrelated control individuals were genotyped. The functional characteristics of the GATA4 mutant were compared to its wild-type counterpart using a luciferase reporter assay system. A novel heterozygous missense GATA4 mutation, p.G21V, was identified in 2 unrelated families with ASD, which was not detected in the control population nor reported in the human gene mutation database. Alignment of multiple GATA4 proteins displayed that the affected amino acid residue was highly conserved across species. Functional analysis showed that the p.G21V GATA4 mutation was associated with a decreased transcriptional activity. The findings underscore the pathogenic link between compromised GATA4 function and congenital ASD, providing new insight into the molecular mechanism involved in this common form of congenital cardiovascular anomalies.

[Judgement of the left ventricle retraining in the rapid two stage-switch operation].

OBJECTIVES: To Summarize the results of left ventricle retraining in rapid two-stage switch operation and to determine the estimating index of left ventricle retraining and the best time of the second stage operation. METHODS: From September 2002 to September 2007, 21 patients underwent rapid two stage switch operation. There were 13 male and 8 female patients, ageing from 29 to 250 d [mean (103 ± 69) d, median 75 d], weighting from 3.5 to 7.0 kg [mean (5.0 ± 1.2) kg, median 5.0 kg]. After pulmonary band, bedside echocardiography was regularly done every other day. Paired t-test was used to analyze the changes of left ventricular end-diastolic dimension (LVDd), left ventricular posterior wall dimensions (LVPWd), diastolic intra-ventricular septal dimensions (IVSd), left ventricular (LV) mass and LV mass indexed for body surface area. RESULTS: The mean interval was (9 ± 5) d. After the left ventricle preparative operation, the left ventricular to right ventricular pressure ratio (pLV/RV) raised from 0.47 ± 0.15 to 0.91 ± 0.20 (P < 0.01). LV mass indexed for body surface area raised from (30 ± 11) g/m(2) to (60 ± 20) g/m(2) (P < 0.01). Extremely significant difference of LV mass existed between pre-arterial switch operation and pre-left ventricle preparative operation, and significant difference existed in LVDd, LVDd(3), LVPWd and IVSd between the two operative timing points. CONCLUSIONS: The left ventricular function of the transposition of the great arteries can be retraining by the left ventricle preparative operation. The interval of left ventricle retraining should be controlled in 7 to 10 d, and the pLV/RV reach 0.65 and the LV mass index over 50 g/m(2) are two important indicators of the second stage operation of arterial switch operation.

Novel NKX2-5 mutations in patients with familial atrial septal defects.

Atrial septal defect (ASD) is a common cardiovascular malformation and an important contributor to substantial morbidity and mortality. Increasing evidence demonstrates that mutated NKX2-5, a gene encoding a homeobox transcription factor crucial to cardiogenesis, is a significant genetic determinant for congenital ASD. Nevertheless, the genetic basis for ASD in a majority of ASD patients remains largely unknown. In the current study, the entire coding region of NKX2-5 was sequenced initially for 58 unrelated probands with familial ASD. The relatives of the probands harboring identified mutations and 200 unrelated control individuals were subsequently genotyped. Three novel heterozygous NKX2-5 mutations (p.P43GfsX59, p.C46 W, and p.S179F) were identified respectively in three families with autosomal dominantly inherited ASD. These mutations, absent in 200 control individuals, cosegregated with ASD in the families that had complete penetrance. The findings expand the spectrum of mutations in NKX2-5 linked to ASD and contribute to genetic counseling, clinical interventions, and prenatal prevention of ASD for individuals with genetic susceptibility.

[Novel GATA4 mutations identified in patients with congenital atrial septal defects].

OBJECTIVE: To identify the genetic defects in patients with congenital atrial septal defects (ASD). METHODS: The clinical data and blood samples from 180 unrelated subjects with congenital ASD were collected and evaluated. Two hundred healthy individuals served as controls. The coding exons and the flanking introns of GATA4 gene were amplified by polymerase chain reaction and sequenced using the di-deoxynucleotide chain termination approach. The acquired sequences were aligned with the sequences publicized in GenBank by the aid of programme BLAST to identify the sequence variations. Clustal W software was applied for analysis of the conservation of altered amino acids. RESULTS: Two novel heterozygous missense GATA4 mutations were identified in 2 out of 180 ASD patients. Namely, the triplet substitutions of GTC for GGC at codon 21 and TCG for CCG at codon 87 were detected, predicting the conversions of glycine into valine at amino acid residue 21 (G21V) and proline into serine at amino acid residue 87 (P87S). None of the two mutations were detected in 200 healthy controls. Across-species alignment of GATA4 encoded protein sequences displayed that the mutated amino acids were highly conserved evolutionarily. Additionally, a single nucleotide polymorphism c.99G>T was observed. However, the polymorphic frequency distribution in ASD cases was similar with that in healthy controls (for genotype GT, χ(2) = 0.7556, P = 0.3847; for allele T, χ(2) = 0.7235, P = 0.3950). CONCLUSIONS: Two novel mutations of GATA4 gene are identified in two unrelated ASD patients. This finding provides new insight into the molecular etiology responsible for ASD.

[Staged surgical treatment of pulmonary atresia with ventricular septal defect].

OBJECTIVE: To determine the choice of palliative procedures, timing and techniques of second-stage operations. METHODS: Between April 2004 to July 2008, 50 consecutive patients with pulmonary atresia with ventricular septal defect (PA/VSD) underwent two-stage operation. Palliative procedures included modified Blalock-Taussig shunt (n = 5), central shunt (n = 2), pericardial patch enlargement (n = 10), pericardial tube (n = 4) and Gore-Tex conduit (n = 29). The interval period was 7 - 49 months (20.0 +/- 10.0 months). In the second stage, a surgical shunt was interrupted in 7 cases. Ventricular septal defect was closed in all patients, but fenestrated ventricular septal defect patch was used in 6 cases. Right ventricular outlet tract (RVOT) was widened with pericardial patch in 42 cases and conduit exchange in 8 cases. Aortopulmonary collateral arteries (MAPCASs) unifocalization (n = 1), ligation or transcatheter occlusion with embolization coils (n = 4) and maintaining open or untreated (n = 4). RESULTS: Death occurred in 2 and the mortality rate was 4%. Postoperative complications included residual shunt (n = 3), residual obstruction (n = 3), complete AV block (n = 1), athetosis (n = 1) and acute renal failure (n = 3). Neither death nor complication was reported during a follow-up period of 3 months to 4 years. CONCLUSION: A palliative procedure should be individualized to the patient's morphology of central pulmonary artery and clinical status of a patient. Right ventricular outlet tract reconstruction, pulmonary arterioplasty, fenestration of VSD patch in baby with suprasystemic right ventricular pressure and appropriate interventions with MAPCASs are key to decrease the mortality and morbidity of staged operations for PA/VSD.

[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.

Evaluation of early macrophage activation and NF-kappaB activity in pulmonary injury caused by deep hypothermia circulatory arrest: an experimental study.

This study aimed to analyze changes in nuclear factor-kappa B (NF-kappaB), inflammation factors, and macrophages in pulmonary tissue under deep hypothermia circulatory arrest (DHCA) at different time points, which can be used to infer the role of early macrophage activation and NF-kappaB activity in pulmonary injury. The possible pathogenic mechanisms of DHCA-induced pulmonary injury were investigated in this study to provide an experimental basis for clinical lung protective strategies. Piglets (n = 12) were randomly divided into 2 groups, with 6 piglets in each group. The control group had ambient temperature cardiopulmonary bypass (CPB), and the experimental group had DHCA. Both groups had conventional CPB with 30 min of parallel circulation. Changes in NF-kappaB and inflammatory factors were examined in each group at 6 different time points. At 0.5 h after ischemia-reperfusion, NF-kappaB expression in the nucleus of pulmonary tissue reached its peak, and brown-stained nuclei were mainly polymorphonuclear antibodies. At 1 h after ischemia-reperfusion, plasma tumor-necrosis factor (TNF)-alpha in the experimental group was significantly increased compared with that before reperfusion (P < 0.05). The plasma levels of interleukin (IL)-8 and IL-6 in the experimental group were significantly increased at 1.5 h after ischemia-reperfusion compared with the levels before reperfusion (P < 0.05). Early activation of NF-kappaB under DHCA might play an important role in DHCA-induced pulmonary injury.

Use of the lecompte procedure for ventriculoarterial connection abnormalities in infants and children with congenital heart diseases.

BACKGROUND: The Lecompte (REV) procedure is used to correct abnormal ventriculoarterial connections in patients with congenital heart diseases; it avoids the need for an extracardiac conduit for pulmonary outflow tract reconstruction. The present study aimed to investigate effectiveness and criteria of the REV procedure in children with abnormal ventriculoarterial connections. METHODS: Thirty-eight children (mean age, (2.2 +/- 1.7) years; mean weight, (11.5 +/- 3.8) kg) with abnormal ventriculoarterial connections who had an REV procedure in our hospital from January 1998 to May 2006 were studied. Only 10 patients had the usual anteroposterior relationship of the two great arteries. The infundibular septum between the two semilunar valves was aggressively resected to enlarge it and construct a straighter left ventricular outflow tract and a wide tunnel between the ventricular septal defect (VSD) and the aorta. Eighteen cases had the original REV procedure; 20 had a modified REV procedure. RESULTS: All patients are alive; none developed severe complications. The postoperative right ventricular (RV) to left ventricular (LV) pressure ratio was 0.20-0.45. Five patients had RV dysfunction; 2 patients had a pressure gradient in the RV ventricular outlet of 30.0-34.5 mmHg; 3 cases had a 37.5-47.3 mmHg pressure difference in the RPA. All patients had an RV pressure less than half the systemic pressure. These gradients' magnitudes in all patients were consistent with the post-operative RV to LV pressure ratio (P < 0.05). During the follow-up (mean, (4.2 +/- 0.6) years), 2 patients had an RPA pressure gradient of 24.0-29.3 mmHg which abated to less than 10 mmHg after two years. CONCLUSIONS: The REV procedure provides satisfactory short- to medium-term results. It may be superior to the Rastelli procedure for treating ventriculoarterial connection abnormalities; it allows early, complete anatomic repair and reduces the need for late re-operation, since no extracardiac conduit is needed. Longer follow-up is needed to determine long-term outcomes.

Arterial switch operation with coronary arteries from a single sinus in infants.

BACKGROUND: The implantation of a coronary artery (CA) is critical for the arterial switch operation (ASO) done to treat complete transposition of the great arteries (TGA). Coronary artery abnormalities are risk factors for both early and late mortality after surgery. In this study, the methodology and effects of ASO surgery with coronary arteries from a single sinus were evaluated. METHODS AND RESULTS: From March 1999 to June 2006, 31 patients were treated with ASO with coronary arteries from a single sinus in our hospital. They aged 11 hours 16 months (2.8 +/- 3.9 months) and weighted 2.3 7.8 Kg (3.1 +/- 2.5 Kg). 27 cases had TGA and a ventricular septal defect (VSD), and 4 had TGA and an intact ventricular septum (IVS). During surgery, a CA button was implanted in the new proximal aorta with "trapdoor" technique or by inverting 90 degrees dorsally; pericardium or arterial augmentation was implanted at the base of the new major artery. The mortality rate after surgery was 25.8%. After 2-5 years of follow-up, 2 cases with residual shunting recovered spontaneously, 2 cases had residual pulmonary artery obstruction (30-56 mmHg), and none of the patients had any significant changes in myocardial ischemia. CONCLUSION: The implantation of an abnormal coronary artery is practical and feasible; it can reduce both the occurrence of twisting and deforming in the coronary artery after implantation, as well as myocardial ischemia after surgery. Thus, this could improve the surgical success and cure rates.

Surgical experience of coarctation of the aorta in infants and young children.

We evaluated the effectiveness of surgical treatment for coarctation of the aorta in infants and young children, based on our experience over 7 years. From March 1999 to December 2005, 168 cases of coarctation of the aorta in patients aged 21 days to 3 years were treated by end-to-end, extended end-to-end, or extended end-to-side ascending aorta and aortic arch anastomosis. The mortality rate was 0.6%. In 138 (82%) patients, the pressure in the lower extremity was higher than in the upper extremity. During 6-24 months of follow-up, hoarseness appeared in 6 patients. Echocardiography revealed stenosis of the end-to-end anastomosis in only 2 patients. We suggest that the best option for surgical treatment of coarctation of the aorta with associated cardiac malformations is a one-stage procedure using a median sternotomy approach. It is better to perform extended end-to-end anastomosis or anastomosis between the distal descending aorta and the left wall of the ascending aorta, or to extend this anastomosis to the transverse arch.

[Analysis surgical results of 113 patients with complete transposition of the great arteries].

OBJECTIVE: To review and analysis the surgical results of 113 arteries Switch operations. METHODS: One hundred and thirteen patients had been repaired by arterial Switch operation from January 2001 to December 2005. There were 60 patients with transposition of the great arteries and intact ventricular septum (TGA/IVS), 53 patients with transposition of great arteries and ventricular septal defect (TGA/VSD). The lowest body weight was 2.3 kg, and the youngest operative age was 6 h. The arteries Switch operation was performed underwent deep hypothermic circulation arrest and low-flow perfusion. RESULTS: The total mortality was 9.7%. There were 5 deaths among TGA/IVS (8.3%), 6 deaths among TGA/VSD (11.3%). Following improvement of surgical technique, post-operative management and cardiopulmonary bypass, the operative mortality was decreased from 16.6% to 5.5%. CONCLUSIONS: The main reason for operative mortality was abnormal coronary arteries. The incidence of abnormal coronary arteries was high at TGA/VSD. The surgical results was not infected by the position of great arteries. The low cardiac output was appeared if the ratio of left ventricular pressure and right ventricular pressure less than 0.6.

[Single-stage arterial switch operation for transposition of the great arteries and Taussig-Bing with aortic arch obstruction].

OBJECTIVE: To evaluate one-stage arterial Switch operation for transposition of the great arteries (TGA) and Taussig-Bing with aortic arch obstruction. METHODS: From January 2001 to June 2004, 8 patients had aortic arch obstruction, 3 with TGA and 5 with Taussig-Bing. Except one patient was 8 months old, all of others were 5 days to 3 months old, the mean operation age was (40 +/- 36) d and the mean weight was (4.3 +/- 0.5) kg. All patients were repaired by one-stage operation. The aortic arch obstruction was repaired in deep hypothermia circulatory arrest, and arterial switch procedure was performed in deep hypothermia and low flow perfusion. RESULTS: There had 1 death who was 8 months old and had low cardiac output, complete artrioventricular block (AVB) and severe pulmonary hypertension postoperation. One patient was 3 months old who had asphyxia at 5 days postoperatively. Six patients followed up from 5 months to 2 years. One Taussig-Bing with interrupted aortic arch had residual obstruction at the anastomosis of aorta. Two had trivial aortic valve regurgitation, and one had mild pulmonary valve regurgitation. CONCLUSIONS: One-stage repair for TGA and Taussig-Bing with aortic obstruction achieves excellent results. The reasons for the death were pulmonary hypertension and abnormal coronary artery. The operative procedure should be performed as early as possible for the better result.

Lung perfusion with oxygenated blood during aortic clamping prevents lung injury.

To evaluate the protective effect of continuous pulmonary perfusion with oxygenated blood during aortic crossclamping, 12 mixed-breed piglets (7-12 kg) were placed on cardiopulmonary bypass for 130 minutes. An experiment group of 6 (group E) had continuous pulmonary perfusion with oxygenated blood during cardiopulmonary bypass, while the other 6 served as controls (group C). Pulmonary function was measured at the beginning and end of cardiopulmonary bypass and one hour later. Histology was compared before and after cardiopulmonary bypass. Pulmonary function after cardiopulmonary bypass was significantly better in group E than group C. There was preservation of the normal pulmonary parenchyma in group E, whereas group C had marked intra-alveolar edema and abundant intra-alveolar neutrophils. Anoxia of lung tissue during aortic crossclamping on cardiopulmonary bypass is probably the major factor in lung injury. Continuous pulmonary perfusion was effective in preventing lung injury during aortic crossclamping.