Rightvertical axillary incision for atrial septal defect: a propensity score matched study.

BACKGROUND: Atrial septal defect is one of the most common types of congenital heart disease. This study aims to explore the surgical and cosmetic effects of open-heart surgery with right vertical axillary incision for simple congenital heart disease in infants. METHODS: From June 2018 to October 2021, children who underwent direct surgery of atrial septal defect in our department were selected for a propensity score matched study. Those with direct surgery through the right vertical axillary incision were included in the right vertical axillary incision group. According to age and weight, propensity score matching method was employed to match children from the right vertical axillary incision group with those undergoing direct surgery through median sternotomy (median sternotomy group) at a 1:2 ratio. Surgery outcomes between two groups were compared to evaluate the effectiveness and safety of right vertical axillary incision group. RESULTS: The median incision length (median, [interquartile range]) in right vertical axillary incision group (4.8 cm, [4.0-5.0]) was shorter than that in median sternotomy group (p < 0.001). The median drainage volume of drainage tube of the right vertical axillary incision group (117.5 ml, [92.8,152.8]) was smaller than that of median sternotomy group (p = 0.021). While no residual bubbles cases in the left and right ventricles and outflow tract were present in the right vertical axillary incision group, 44% of residual air bubble rate in right ventricular outflow tract was detected in median sternotomy group (p = 0.001). Additional sedation and analgesia (p = 0.003), wound infection or poor healing (p = 0.047), thoracic deformity healing (p = 0.029) and appearance satisfaction questionnaire (p = 0.018) in the right vertical axillary incision group were better than those in the median sternotomy group. CONCLUSION: Right axillary vertical incision can effectively reduce surgical trauma, accelerate postoperative rehabilitation. This surgical approach also provides better cosmetic effect, which is easily accepted by children's families and worthy of further clinical application.

A three-dimensional printed model in preoperative consent for ventricular septal defect repair.

BACKGROUND: The 3D printing technology in congenital cardiac surgery has been widely utilized to improve patients' understanding of their disease. However, there has been no randomized controlled study on its usefulness in surgical consent for congenital heart disease repair. METHODS: A randomized controlled study was performed during consent process in which guardians of candidates for ventricular septal defect repair were given detailed explanation of the anatomy, indication for surgery and potential complication and risks using 3D print ventricular septal defect model (n = 20) versus a conventional 2D diagram (n = 20). A questionnaire was finished by each guardian of the patients. Data collected from questionnaires as well as medical records were statistically analyzed. RESULTS: Statistically significant improvements in ratings of understanding of ventricular septal defect anatomy (p = 0.02), and of the surgical procedure and potential complications (p = 0.02) were noted in the group that used the 3D model, though there was no difference in overall ratings of the consent process (p = 0.09). There was no difference in questionnaire score between subjects with different education levels. The clinical outcomes, as represented by the duration of intensive care unit stay, intubation duration was comparable between the two groups. CONCLUSIONS: The results indicated that it was an effective tool which may be used to consent for congenital heart surgery. Different education levels do not affect guardians' understanding in consent. The impact of 3D printing used in this scenario on long term outcomes remains to be defined.

Whole-exome sequencing identified a novel heterozygous mutation of SALL1 and a new homozygous mutation of PTPRQ in a Chinese family with Townes-Brocks syndrome and hearing loss.

BACKGROUND: Previous studies have revealed that mutations of Spalt Like Transcription Factor 1 (SALL1) are responsible for Townes-Brocks syndrome (TBS), a rare genetic disorder that is characterized by an imperforate anus, dysplastic ears, thumb malformations and other abnormalities, such as hearing loss, foot malformations, renal impairment with or without renal malformations, genitourinary malformations, and congenital heart disease. In addition, the protein tyrosine phosphatase receptor type Q (PTPRQ) gene has been identified in nonsyndromic hearing loss patients with autosomal recessive or autosomal dominant inherited patterns. METHODS: A Chinese family with TBS and hearing loss was enrolled in this study. The proband was a two-month-old girl who suffered from congenital anal atresia with rectal perineal fistula, ventricular septal defect, patent ductus arteriosus, pulmonary hypertension (PH), and finger deformities. The proband's father also had external ear deformity with deafness, toe deformities and PH, although his anus was normal. Further investigation found that the proband's mother presented nonsyndromic hearing loss, and the proband's mother's parents were consanguine married. Whole-exome sequencing and Sanger sequencing were applied to detect the genetic lesions of TBS and nonsyndromic hearing loss. RESULTS: Via whole-exome sequencing and Sanger sequencing of the proband and her mother, we identified a novel heterozygous mutation (ENST00000251020: c.1428_1429insT, p. K478QfsX38) of SALL1 in the proband and her father who presented TBS phenotypes, and we also detected a new homozygous mutation [ENST00000266688: c.1057_1057delC, p. L353SfsX8)] of PTPRQ in the proband's mother and uncle, who suffered from nonsyndromic hearing loss. Both mutations were located in the conserved sites of the respective protein and were predicted to be deleterious by informatics analysis. CONCLUSIONS: This study confirmed the diagnosis of TBS at the molecular level and expanded the spectrum of SALL1 mutations and PTPRQ mutations. Our study may contribute to the clinical management and genetic counselling of TBS and hearing loss.

A novel three-dimensional printed vacuum bell for pectus excavatum treatment: a preliminary study.

OBJECTIVE: Conservative treatment with a vacuum bell (VB) for pectus excavatum (PE) has now been gradually popularized as an alternative to surgery. We describe our initial experience with a novel three dimensional (3D) printed VB device. METHODS: Prospectively collected data of all patients who started using a 3D printed VB in 2018 at our institution were analyzed. Linear and logistic regressions were used to identify factors associated with effectiveness of device usage. RESULTS: In total, forty-two patients with a median age of 3.6 years were treated with the device. The median follow-up duration was 11.1 months and the mean initial Depth Ratio (DR) was 0.129. There were no permanent sequelae from side effects. Thirty patients with at least one follow-up body scan data showed varying improvement (z = - 4.569, p = 0.0000). Linear regression suggested that longer usage improved outcomes (R2 = 0.235, p = 0.014). By logistic regression there was a trend of younger ages and less initial DR for better improvement though neither was statistically significant (p = 0.086, 0.078, respectively). CONCLUSION: Our initial experience has shown the 3D printed VB may be as effective as other conventional VBs and could be used as an alternative to surgical treatment for selected patients with PE. More experience and studies with this type of VB are needed to demonstrate its superiority with regard to the 3D printing design and optimal timing and indication for use.

Transcatheter closure of a residual shunt with posteroinferior deficient rim after surgical closure of an ASD: a case report.

BACKGROUND: There are few reports in the literature of device closure of residual shunts following initial surgical closure of an atrial septal defect (ASD). This case study reports one such case. We describe here a case of secundum type ASD that was initially closed surgically, followed by device closure of a residual shunt with a posteroinferior deficient rim. CASE PRESENTATION: A 7-month-old boy was admitted to our hospital for elective surgery to surgically correct a secundum type ASD. Unfortunately, a residual shunt 3.5 mm in diameter appeared before discharge and was enlarged at1-year follow-up. The cause of this residual shunt was dehiscence at the posteroinferior aspect, and the posteroinferior rim was 3.7 mm. After careful discussion and preparation, we proceeded with an interventional procedure. A 16 mm ASD occluder (AGA Medical Corp, Plymouth, Minnesota) was deployed successfully with no residual shunt. In some cases of ASD, interventional therapy is not considered due to the size and position of the defect, but we show here, a successful case of interventional therapy for a residual shunt with a deficient rim. CONCLUSION: We have presented a case in which a postoperative residual shunt with a deficient rim was successfully closed with interventional therapy.

The consistency of an optical body surface scanning method compared with computed tomography: a validation study.

OBJECTIVE: The ideal noninvasive method for evaluation of pectus excavatum remains to be defined. We sought to verify the accuracy of an optical body surface scanning method compared with conventional CT scan. MATERIALS AND METHODS: A PrimeSense 3D sensor was used to obtain data from patients undergoing surgical or noninvasive treatment for pectus excavatum. The Haller index, external Haller index, and depth ratio were then calculated from both body scan and computed tomography scan data for the same patients. Statistical analyses were carried out to find if there is consistency between data from body scanning and computed tomography. RESULTS: Data acquisition was complete. In total, 40 patients (median age: 5.03 years, 11 female) with pectus excavatum undergoing nonoperative (n = 13) or surgical Nuss treatment (n = 27) were included. The Haller index was lower in vacuum bell patients, which also had a higher female proportion. Pearson correlation coefficient between external Haller indices from body scanning and from computed tomography and between the depth ratios from body scanning and from computed tomography were 0.63 and 0.84, respectively. By intraclass correlation coefficient method, the correlation coefficient was 0.56 between external Haller indices from body scanning and from computed tomography and 0.80 between depth ratios from body scanning and from computed tomography. CONCLUSION: The optical body surface scanning is a reliable approach to the measurement of PE severity and could be routinely used in the monitoring of PE development of treatment, especially in the pediatric population. STUDY TYPE: Diagnostic test. LEVEL OF EVIDENCE: Level II.

Residual Shunts Following Isolated Surgical Ventricular Septal Defect Closure: Risk Factors and Spontaneous Closure.

Although isolated congenital ventricular septal defects (VSD) can be repaired with a high degree of success, residual shunts (RS) are commonplace postoperatively. Small RS are relatively innocuous and tend to spontaneously close with time, despite the emotional burden it poses for the patient and family. A large RS, however, needs ongoing surveillance and may necessitate reintervention. Factors influencing the incidence of RS as well as the likelihood and expected timing of its spontaneous closure are discussed in this study. The patient records and relevant data of 362 consecutive patients undergoing cardiac operation with isolated congenital VSD closure as primary procedure between January 2017 and December 2017 were included in the study. Postoperative transthoracic echocardiograms were performed at hospital discharge, and during follow-up, at 1 month, 3 months, 6 months and 1 year postoperatively. Residual defects were measured under echocardiogram at every follow-up. Factors expected to be associated with RS occurrence and spontaneous closure were included for logistic and Cox regression statistical analysis. There were 113 cases where RS occurred according to the first postoperative echocardiograms that were performed at discharge, of which 80 were confirmed closed during subsequent follow-up, with a median follow-up of 96 days. A cutoff of 1.25 mm for the initial RS was found to be the best predictor of spontaneous closure at 6-month follow-up. Small shunts had higher closure rate than larger ones by a follow-up duration of 300 days, at which the two groups tended to reach a similar spontaneous closure rate. Longer surgical bypass time distinguished small from larger residual shunts measured upon discharge. Following repair of isolated congenital VSDs, the incidence of a residual shunt is high. The majority spontaneously close within 300 days following surgery. Longer bypass time predicted a larger residual shunt upon discharge. Larger than 1.25 mm shunts had lower short-term closure rate but seemed not to differ from smaller shunts beyond 300 days postoperatively.

Sewing needles in the lungs of children: Two case reports.

RATIONALE: Foreign bodies are common in children. However, it is uncommon to have a "sewing needle" penetrating the body percutaneously rather than via a natural orifice. Such cases have been reported from across the world, with needles being found in regions such as the cranium, abdomen, limbs, neck, scrotum, and buttocks. Few cases with a needle inserted in the lung have been reported. PATIENT CONCERNS: We present 2 cases where the needles were found in the chest wall/lung because of suspected child abuse. In the first case, foreign bodies were present in the chest wall and the lung. In the other case, the pointed end was oriented towards, and was very close to, the great arteries. DIAGNOSES: Both cases were diagnosed incidentally when chest X-ray was being performed for other reasons. INTERVENTIONS: Surgery was indicated for both cases, and the needles were excised uneventfully. OUTCOMES: The postoperative course in both cases was uneventful. LESSONS: It is worth noting that in both cases, neither the guardians nor the victims themselves could tell when and how the needles got lodged in the lungs. Collective information suggested that these 2 cases probably involved child abuse. Child abuse in China has long been underestimated and underrecognized. There is still much left to do to tackle this important issue, especially in rural areas.

Red blood cell transfusion threshold after pediatric cardiac surgery: A systematic review and meta-analysis.

BACKGROUND: Restrictive red blood cell transfusion strategy is implemented to minimize risk following allogeneic blood transfusion in adult cardiac surgery. However, it is still unclear if it can be applied to pediatric cardiac patients. The purpose of this systematic review and meta-analysis was to determine the effect of postoperative restrictive transfusion thresholds on clinical outcomes based on up-to-date results of randomized controlled trials (RCTs) and observational studies in pediatric cardiac surgery. METHOD: We searched for RCTs and observational studies in the following databases: the Cochrane Central Register of Controlled Trials, MEDLINE, EMBASE, and ClinicalTrials.gov from their inception to October 26, 2017. We also searched reference lists of published guidelines, reviews, and relevant articles, as well as conference proceedings. No language restrictions were applied and no observational study met the inclusion criteria. RESULTS: Four RCTs on cardiac surgery involving 454 patients were included. There were no differences in the pooled fixed effects of intensive care unit (ICU) stay between the liberal and restrictive transfusion thresholds (standardized mean difference SMD, 0.007; 95% confidence interval CI, -0.18-0.19; P = .94). There were also no differences in the length of hospital stay (SMD, -0.062; 95% CI, -0.28-0.15; P = .57), ventilation duration (SMD, -0.015; 95% CI, -0.25-0.22; P = .90), mean arterial lactate level (SMD, 0.071; 95% CI, -0.22-0.36; P = .63), and mortality (risk ratio, 0.49; 95% CI, 0.13-1.94; P = .31). There was no inter-trial heterogeneity for any pooled analysis. Publication bias was tested using Egger, Begg, or the trim-and-fill test, and the results indicated no significant publication bias. CONCLUSION: Evidence from RCTs in pediatric cardiac surgery, though limited, showed non-inferiority of restrictive thresholds over liberal thresholds in length of ICU stay and other outcomes following red blood cell transfusion. Further high-quality RCTs are necessary to confirm the findings.

The Nuss procedure for pectus excavatum during atrial septal defect closure through a minimal right oblique infra-axillary thoracotomy: A case report.

RATIONALE: Pectus excavatum (PE) is normally an isolated congenital disorder, but it can also occur with congenital heart defect (CHD). The optimal strategy for the management of children with PE and concurrent CHD remains under debate. The surgical strategy has evolved over the last 20 years from staged repair to simultaneous repair of both defects. We present a case of using the Nuss procedure for PE during atrial septal defect (ASD) closure through a minimal right oblique infra-axillary thoracotomy. To our knowledge, this is the first report to describe the correction of PE and CHD by this approach. PATIENT CONCERNS: A 3.6-year-old patient weighing 13 kg was admitted for elective repair of PE and an ASD. DIAGNOSES: Clinically, the patient had typical features of PE with chest computed tomography (CT) revealing a Haller index of 4.4 and a grade 2 systolic murmur being heard the loudest at the 2nd-3rd intercostal space, abutting the left sternal border. Echocardiography confirmed a 2-hole secundum ASD with the upper defect being 8 mm, the lower defect 5 mm, and the 2 holes being 5-mm apart, which was deemed unsuitable for interventional closure. INTERVENTIONS: After discussion with and consent from the family, the child underwent concomitant surgery for both defects. We performed an ASD repair under cardiopulmonary bypass (CPB) on the beating heart through the right oblique infra-axillary thoracotomy, and then, the standard Nuss procedure was performed using a 9-inch bar. OUTCOMES: Satisfactory ASD closure was confirmed by postoperative echocardiography. Satisfactory PE correction was confirmed by physical examination and postoperative chest radiography. The postoperative recovery process was uneventful, and the patient was discharged 6 days postoperatively. LESSONS: This case shows that in carefully selected cases with concomitant PE and ASD, a combination of the Nuss procedure and ASD repair by CPB through infra-axillary thoracotomy can be safely performed, avoiding sternal incision, which leads to bleeding and sternal dehiscence, and results in better aesthetic and surgical outcomes.

[Simultaneous surgical treatment for pectus excavatum combined with congenital cardiothoracic diseases].

OBJECTIVE: To study the methods and principles for simultaneous treatment in the children with pectus excavatum (PE) combined with congenital cardiothoracic diseases.
 Methods: The medical records of all children, who underwent simultaneous repair of PE combined with congenital cardiothoracic diseases, were retrospectively reviewed in Hunan Children's Hospital from January 2007 to September 2018. The patients were divided into a PE combined with congenital heart disease (CHD) group (n=17) and a PE combined with thoracic disease group (n=10). The repair with a custom-made sternal lifting device, a Nuss repair, were performed in the treatment of PE, and the correction of the CHD was performed by heart open surgery using cardiopulmonary bypass (through sternotomy or right infra-axillary thoracotomy) or by transcatheter closure under echocardiography or X-ray-guided percutaneous intervention in the PE combined with CHD group. The children in the PE combined with thoracic disease group underwent thoracic surgery plus Nuss procedure concurrently.
 Results: All 27 pediatric patients underwent simultaneous repair of the PE combined with congenital cardiothoracic diseases. In the PE combined with CHD group, the duration of hospital stay ranged from 8.0 to 25.0 (13.2±4.8) days. Two patients had delayed healing of the surgical wound and 1 patient developed a small left pleural effusion postoperatively. In the PE combined with thoracic disease group, the duration of hospital stay ranged from 10.0 to 34.0 (19.9±7.5) days. One patient was complicated with chylothorax and 2 patients were complicated with pleural effusionin. The treatment for the patients in the 2 groups was satisfactory. No severe complications like surgical death, severe bleeding, chest organ injuries, and implant rejections were observed.
 Conclusion: According to the characteristics of patients, individualized programs should be selected in order to correct children's PE combined with congenital cardiothoracic diseases in the same period, which are safe, effective and can avoid the risk of multiple operations and anesthesia, and can reduce the financial burden of family.

Simultaneous operative repair of pectus excavatum and concurrent congenital heart defects.

OBJECTIVES: The aim of this study is to describe an effective surgical strategy for the treatment of children with pectus excavatum (PE) and concurrent congenital heart defect (CHD). METHODS: We retrospectively reviewed the medical records of all children (n = 16) who underwent simultaneous repair of PE with concurrent CHD at the Hunan Children's Hospital from July 2007 to December 2017. Among them, 7 patients had a simple atrial septal defect (ASD); 4 patients had a simple ventricular septal defect (VSD); 3 patients had both VSD and ASD; 1 patient had VSD, ASD and patent ductus arteriosus; and 1 patient had VSD complicated by pulmonary hypertension. To repair PE, we performed one of the 3 procedures: an open sternal elevation involving freeing the tissue from the posterior sternum and lifting the sternum by wires, a Nuss repair or a repair with a custom-made sternal lifting device. The CHDs were repaired using one of the 3 methods as well: open heart surgery using cardiopulmonary bypass, transcatheter closure under echocardiography or X-ray-guided percutaneous intervention. RESULTS: In all 16 patients, the operations led to satisfactory repair of both conditions without serious complications. All parents and children were satisfied with the postoperative appearance of the chests. CONCLUSIONS: By choosing the correct operative approach based on patient characteristics, simultaneous elective repair of PE and concurrent CHD is safe and effective, avoids the risk of multiple staged operations and achieves satisfactory outcomes.

Limb ischemic preconditioning reduces heart and lung injury after an open heart operation in infants.

Open heart surgery supported by cardiopulmonary bypass is associated with heart and lung ischemia-reperfusion injury (IRI). Limb remote ischemic preconditioning (RIPC) reduces injury caused by ischemia-reperfusion in multiple distant organs. We conducted a prospective clinical trial (randomized and controlled) to test the feasibility and safety of limb RIPC, as well as its protective effects against myocardial and pulmonary IRI for infants undergoing repair of simple congenital heart defects. Infants undergoing repair of ventricular septal defects were enrolled in our study and randomly assigned to one of two treatment groups: limb RIPC or control. RIPC was induced twice (24 h and 1 h preoperatively) via three 5-min cycles of ischemia and reperfusion on the left upper arm using a blood pressure cuff. Lung compliance, respiratory index (RI), and cardiac inotropic score (IS) were calculated for each patient. Serum concentrations of the following factors were measured perioperatively: interleukin (IL)-6, IL-8, IL-10, and tumor necrosis factor (TNF)-alpha; lactate dehydrogenase (LDH), creatine kinase (CK), and its isoenzyme (CK-MB), and troponin I (TnI); malondialdehyde (MDA) and superoxide dismutase (SOD). The expression of heat shock protein 70 (HSP 70) in cardiomyocytes was analyzed by Western blot. Surgical outcomes, including limb movement and sensory function, were recorded in detail. Sixty infants weighting less than 7 kg were studied, with 30 patients in the RIPC group and 30 in the control group. Within 6 months of discharge from the hospital, no limb disability, sensory disturbance, or other surgical complications were found in any patient. Compared with the control group, patients in the RIPC group had higher Cs and Cd, along with lower RI and IS at various postoperative phases. At the beginning of the operation, serum concentrations of IL-6, IL-8, IL-10, TNF-alpha, LDH, CK, and TnI were higher in the RIPC group than the control group. Postoperatively, release of cytokines and leakage of heart enzymes were attenuated in the RIPC group; serum concentrations of cytokines and heart enzymes were lower in the RIPC group at some, but not all, postoperative time points. Furthermore, the RIPC group had lower coronary sinus venous concentrations of MDA and higher concentrations of SOD. Similarly, the expression of HSP 70 was upregulated in cardiomyocytes from the RIPC group. Limb RIPC can be applied safely and easily in infants, can attenuate systemic inflammatory response syndrome, and can increase systemic tolerance to IRI, imparting a protective effect against myocardial and pulmonary IRI. The expression of HSP 70 has an important role in the mechanism of action for RIPC.