Primary Corrective Surgery Research Articles

The Craniofacial Collaboration UK: Developmental Outcomes in 7- and 10-Year-Old Children With Sagittal Synostosis

The Craniofacial Collaboration UK (CC-UK) protocol is a shared agreement across the 4 UK Highly Specialist Craniofacial Centres (HSCCs) to conduct robust neurodevelopmental and psychosocial clinical screening for children with craniosynostosis. This agreement allows for the analysis of outcomes of a homogenous sample of children with single suture craniosynostosis (SSC), a frequent limitation of the existing research. The current study is the latest analysis of CC-UK data on behavioral, cognitive, and psychosocial outcomes. The focus of this analysis is 7- and 10-year-olds with nonsyndromic sagittal synostosis (SS) who have undergone primary corrective surgery and completed routine clinical screening at 1 of the 4 HSCCs since the introduction of the CC-UK protocol. Due to changes in clinical pathways, only data from 3 HSCCs is included to preserve homogeneity. Results show that the majority of children with SS fall within the average range across behavioral and neurodevelopmental domains. A notable exception was a task involving perceptual reasoning and visuomotor skills (Block Design). Although this difference was small and the mean score remained within the average range, it suggests some increased risk of subtle difficulty with such skills for children with SS. Across other measures, there was no consistent evidence of any significantly increased risk of poorer outcomes, in line with findings of previous CC-UK papers. Understanding the psychological phenotype of SS is a key research priority for parents and clinicians, and the current study is another step toward achieving this goal.

Anorectal malformation and Hirschsprung’s disease: a cross-sectional multicentre comparison of quality of life and bowel function to a healthy population

PurposeChildren with anorectal malformation (ARM) and Hirschsprung’s disease (HD) often experience bowel symptoms into adulthood, despite definitive surgery. This study evaluates the quality of life (QOL) and bowel functional outcome...

The Craniofacial Collaboration UK: Developmental Outcomes in 7- and 10-Year-Old Children With Metopic Synostosis.

The Craniofacial Collaboration (CC-UK) is a shared initiative across the Psychology teams attached to 4 highly specialized craniofacial centers in the United Kingdom. The CC-UK aims to address key limitations in the existing craniofacial literature by analyzing data for homogenous samples of children with craniosynostosis. This article presents the fifth wave of CC-UK data collection, focused on 7- and 10-year olds who have undergone primary corrective surgery for metopic synostosis (MS). Data for children with sagittal synostosis and MS have previously been presented at 3 and 5 years. This paper continues to build on this with consideration to older age groups, presenting the first CC-UK analysis of cognitive assessment data using the Wechsler Abbreviated Scale of Intelligence-Second Edition. Results show that the majority of children with MS fall within the average ranges across behavioral and neurodevelopmental domains. However, several domains indicated a trend of heightened concern when compared with normative data, particularly for parent-reported outcomes, suggesting that there may be some subtle difficulties for children with MS. Consideration of how these findings compare with that of previous CC-UK analyses is explored. Further, implications for clinical practice and future research are considered, with the need for longitudinal analyses, as well as data from multiple perspectives (eg, school, parents, and self) at older age points to establish patterns over time. Through collaboration across the highly specialized craniofacial centers, the CC-UK hopes to work toward this goal moving forward.

Evaluation of Ain Shams University Hospitals Clinical Pathway for Pulmonary Artery Banding

Abstract Background Pulmonary artery banding (PAB) remains a useful surgical palliation for small neonates and early infants with excessive pulmonary blood flow who are unable or difficult to withstand an open heart surgery using cardiopulmonary bypass or in developing countries where the facilities not suitable for primary corrective surgery in those infants. Objectives In our current study we aimed to study clinical pathway of cases underwent PAB at ASU hospital pediatric cardiac surgery department with discussion of different causes of mortality and morbidity. Patients and Methods Study done by data collection retrospectively of 50 patients underwent pulmonary artery banding in the period from January 2018 to December 2019 who fulfilling the inclusion criteria with evaluation of different clinical pathways for the outcome mainly morbidities and mortality. Results We found that the major risk factors of mortality in cases underwent PAB in ASU hospitals includes patients diagnosed with TGA, low preoperative so2 ≤85, ventilation is high risk factor for mortality so it is recommended to extubate those patients on table or shortly after discharge to ICU as soon as possible, duration of intubation: the longer the duration of intubation, the higher the mortality, postoperative PG across band ≤45.that indicator for a loose band with still persistent pulmonary overflow, re-intubation, post operative morbidities are highly associated with mortality, 11 out of 12 mortality cases suffered from postoperative complications (91.7 % of mortality cases) that includes poor chest condition, stroke, postoperative convulsions and pulmonary hemorrhage. These morbidities result in prolonged intubation and prolonged ICU stay. Conclusion Pulmonary artery banding is palliative procedure done in infants with pulmonary artery overflow with unprotected pulmonary circulation in variable congenital cardiac diseases (VSD, single ventricle, TGA) as a primary stage for total repair when the infant gain weight and get older, the suitable age for banding is < 6 months.

Treatment Delays in Nonsyndromic Craniosynostosis: A 30-Year Retrospective Case-Control Analysis of the Impact of Socioeconomic and Family Status on Access to Care.

Patients with nonsyndromic craniosynostosis (NSC) generally undergo corrective surgery before 1 year of age to the mitigate morbidities and risks of delayed repair. The cohort of patients who receive primary corrective surgery after 1 year and factors associated with their gaps to care is poorly characterized in literature. A nested case-control study was conducted for NSC patients who underwent primary corrective surgery at our institution and affiliates between 1992 and 2022. Patients whose surgery occurred after 1 year of age were identified and matched 1:1 by surgical date to standard-care control subjects. Chart review was conducted to gather patient data regarding care timeline and sociodemographic characteristics. Odds of surgery after 1 year of age were increased in Black patients (odds ratio, 3.94; P < 0.001) and those insured by Medicaid (2.57, P = 0.018), with single caregivers (4.96, P = 0.002), and from lower-income areas (+1% per $1000 income decrease, P = 0.001). Delays associated with socioeconomic status primarily impacted timely access to a craniofacial provider, whereas caregiver status was associated with subspecialty level delays. These disparities were exacerbated in patients with sagittal and metopic synostosis, respectively. Patients with multisuture synostosis were susceptible to significant delays related to familial strain (foster status, insurer, and English proficiency). Patients from socioeconomically strained households face systemic barriers to accessing optimal NSC care; disparities may be exacerbated by the diagnostic/treatment complexities of specific types of craniosynostosis. Interventions at primary care and craniofacial specialist levels can decrease health care gaps and optimize outcomes for vulnerable patients.

Comparison between the modified Blalock-Taussig shunt and right ventricular outflow tract stent in the palliative treatment for tetralogy of Fallot.

For patients with tetralogy of Fallot (TOF) who are not suitable candidates for primary corrective surgery or have a high surgical risk, transcatheter right ventricular outflow tract (RVOT) stent implantation is considered a safe and effective palliative intervention. This study aims to investigate the therapeutic outcomes of RVOT stent implantation in neonates and infants with TOF in comparison with the modified Blalock-Taussig shunt (mBTS) and to compare the impact of the 2 palliative interventions on arterial oxygen saturation and pulmonary artery development in pediatric patients. Clinical data of 32 patients with TOF admitted to the Second Xiangya Hospital of Central South University from March 2011 to March 2021 were retrospectively collected. The patients were divided into an mBTS group (undergoing mBTS, n=15) and a stent implantation group (undergoing RVOT stenting, n=17) according to the surgical procedures. The 2 groups were assessed and compared in the surgical-related arterial oxygen saturation, postoperative complication rate, mortality rate, and re-intervention rate. The development of the patients' main pulmonary artery, right pulmonary artery, and left pulmonary artery was assessed by z-scores according to echocardiographic results. The children in the stent implantation group were younger and less weighed compared with the mBTS group (both P<0.05). Compared with the preoperative period, children in the stent implantation group had significantly higher arterial oxygen saturation [(75±17)% vs (96±3)%, P=0.026]; z-scores of pulmonary trunk [(-2.82±1.27) points vs (0.86±0.77) points, P=0.014], right pulmonary artery [(-1.88±0.59) points vs (-0.28±0.71) points, P=0.011], and left pulmonary artery [(-2.34±0.36) points vs (-1.67±0.36) points, P=0.036] were significantly increased. However, there were no significant differences in arterial oxygen saturation and pulmonary artery z-scores between pre- and post-mBTS procedures (all P>0.05). RVOT stent would have good surgical outcomes used in TOF patients with low weight and severe comorbidities. It also leads to an higher postoperative oxygen saturation and better promotion of pulmonary artery growth with RVOT stent compared to mBTS.

The Craniofacial Collaboration UK: Developmental Outcomes in 5-Year-Old Children With Metopic Synostosis.

The Craniofacial Collaboration (CC-UK) was setup in 2015 as a joint initiative between the Psychology teams attached to the 4 highly specialized craniofacial centers in the United Kingdom. The CC-UK aims to address key limitations in the existing craniofacial literature by applying strict exclusion criteria and collating clinical data on a homogenous sample of children. This article reports the fourth wave of data collection from the CC-UK, with the analysis of developmental and behavioral outcomes for children with metopic synostosis at 5 years old. Previous data for sagittal synostosis at 3 and 5 years, and metopic synostosis at 3 years, have been presented. This paper offers the first analysis of developmental and behavioral parent-report measures at school age for metopic synostosis. All children in the current data set had primary corrective surgery. Findings highlight similar patterns to that of previous research among children with single-suture craniosynostosis, as well as earlier CC-UK analyses, with the majority falling within 1 standard deviation of the normative mean. However, differences across key behavioral and emotional domains, with some areas reporting heightened concerns compared with those detected among sagittal groups, may suggest that subtle differences between individual diagnostic groups are present. This further highlights the importance of utilizing homogenous samples within the field of craniofacial research. To further build upon this work, and to provide a greater understanding of how these difficulties and concerns may develop, or diminish, over time, further consideration to longitudinal outcomes is needed for individual diagnostic groups. Through this collaboration, the authors seek to achieve this goal in their future work.

Fetal cardiac parameters for predicting postnatal operation type of fetuses with tetralogy of Fallot

BackgroundTo assess fetal cardiac parameters predictive of postnatal operation type in fetuses with tetralogy of Fallot (TOF).MethodsEchocardiographic data obtained in the second and third trimesters were retrospectively reviewed for fetuses diagnosed with TOF between 2014 and 2018 at Asan Medical Center. The following fetal cardiac parameters were analyzed: 1) pulmonary valve annulus (PVA) z-score, 2) right pulmonary artery (RPA) z-score, 3) aortic valve annulus (AVA) z-score, 4) pulmonary valve peak systolic velocity (PV-PSV), 5) PVA/AVA ratio, and 6) RPA/descending aorta (DAo) ratio. These cardiac parameters were compared between a primary corrective surgery group and a palliative shunt operation followed by complete repair group.ResultsA total of 100 fetuses with TOF were included. Only one neonatal death occurred. Ninety patients underwent primary corrective surgery and 10 neonates underwent a multistage surgery. The PVA z-score, RPA z-score, and RPA/DAo ratio measured in the second trimester and the PVA z-score, RPA z-score, and PVA/AVA raio measured in the third trimester were significantly lower in the multistage surgery group, while the PV-PSV as measured in both trimesters were significantly higher in the multistage surgery group.ConclusionFetal cardiac parameters are useful for predicting the operation type necessary for neonates with TOF.

Treatment outcomes in patients with laryngotracheal lesions and compromised airway during and prior to the coronovirus disease 2019 pandemic: a tertiary institution's experience.

This study aimed to compare treatment outcomes in patients with laryngeal and tracheal stenosis treated during and prior to the coronavirus disease 2019 pandemic period. Patients treated for laryngotracheal lesions with impending airway compromise during the active pandemic period were matched with those treated for similar lesions in the preceding years in a monocentric tertiary hospital setting. During the pandemic period of 55 days, 31 patients underwent 47 procedures. Seven patients (2 children, 5 adults) had open airway surgery, and one had an operation-specific complication. Twenty-four patients (10 children, 14 adults) underwent 40 endoscopic interventions without any complications. Operation specific results during and prior to the pandemic were comparable. The management strategy in patients with laryngotracheal lesions and impending airway compromise should not be altered during periods of risk from coronavirus disease 2019. Avoiding a tracheostomy by performing primary corrective surgery or proceeding with a definitive decannulation would be beneficial in these patients to reduce the risk of contagion.

The Craniofacial Collaboration UK: Developmental Outcomes in 3-Year-Old Children With Metopic Synostosis.

The Craniofacial Collaboration UK (CC-UK) has been established across the 4 highly specialized craniofacial centers in the UK since 2015. This joint collective aims to address the current limitations within developmental craniofacial research, using robust clinical data from a homogenous sample of children. This paper presents the third wave of findings from the CC-UK, with consideration to developmental and behavioral parent-report measures. Whilst previous data for sagittal synostosis have been presented, this article summarizes the analysis of these outcomes for children with metopic synostosis (MS) at 3 years who have undergone primary corrective surgery. Results highlight similar patterns to that of earlier CC-UK work, with the majority of children falling within 1 standard deviation of the population normative means across all measures. However, statistically significant difficulties were found between group means for children with MS on various developmental and behavioral domains. Prosocial skills and peer difficulties were reported as the greatest areas of behavioral concern for parents, with prosocial skills found to be below the level expected for their chronological age. In order to further understand the developmental trajectory of children with MS, longitudinal examination of individual diagnostic and specific age groups with single-suture craniosynostosis is crucial. The continuation of the CC-UK provides an opportunity to attain this goal.

First metatarsophalangeal fusion with dorsal plate: clinical outcomes

BackgroundFirst metatarsophalangeal joint (MTPJ) fusion is the most effective technique for the treatment of MTPJ primary arthritis, severe hallux valgus and failure of primary corrective surgery of these conditions. It can be achieved through different techniques. We evaluated the outcomes in a cohort of patients treated with dorsal plate arthrodesis.Materials and methodsWe treated 30 feet for 28 patients; the mean follow-up was 35 months. For each foot, we collected radiological and clinical assessment, with the visual analogue scale (VAS) for pain and the Manchester and Oxford Foot questionnaire (MOFQ). The technique consisted in a cup and cone arthrodesis with the application of a low profile dorsal plate. Patients were allowed for immediate weight bearing.ResultsConsolidation was achieved in all cases; in 29 cases, radiographic union was recorded within 6 months from surgery, in one case after 9 months. Comparison between the preoperative and postoperative of VAS and MOXFQ values showed a statistically significant difference (p < 0.05). Only one case developed wound dehiscence as complication.ConclusionsEven if there is still a debate regarding the best system for MTPJ fusion, we believe cup and cone fusion with dorsal plating is an effective method. Moreover, the stability of the osteosynthesis obtained allows for immediate post-operative weight bearing, making patients able to return soon to their normal life.Trial registrationWe present a retrospective study; all patients enrolled were retrospectively registered.

Factors which predict adverse events following surgery in adults with cervical spinal deformity.

The aim of this study was to identify the risk factors for adverse events following the surgical correction of cervical spinal deformities in adults. We identified adult patients who underwent corrective cervical spinal surgery between 1 January 2007 and 31 December 2015 from the MarketScan database. The baseline comorbidities and characteristics of the operation were recorded. Adverse events were defined as the development of a complication, an unanticipated deleterious postoperative event, or further surgery. Patients aged < 18 years and those with a previous history of tumour or trauma were excluded from the study. A total of 13,549 adults in the database underwent primary corrective surgery for a cervical spinal deformity during the study period. A total of 3,785 (27.9%) had a complication within 90 days of the procedure, and 3,893 (28.7%) required further surgery within two years. In multivariate analysis, male sex (odds ratio (OR) 0.90 (95% confidence interval (CI) 0.8 to 0.9); p = 0.019) and a posterior approach (compared with a combined surgical approach, OR 0.66 (95% CI 0.5 to 0.8); p < 0.001) significantly decreased the risk of complications. Osteoporosis (OR 1.41 (95% CI 1.3 to 1.6); p < 0.001), dyspnoea (OR 1.48 (95% CI 1.3 to 1.6); p < 0.001), cerebrovascular accident (OR 1.81 (95% CI 1.6 to 2.0); p < 0.001), a posterior approach (compared with an anterior approach, OR 1.23 (95% CI 1.1 to 1.4); p < 0.001), and the use of bone morphogenic protein (BMP) (OR 1.22 (95% CI 1.1 to 1.4); p = 0.003) significantly increased the risks of 90-day complications. In multivariate regression analysis, preoperative dyspnoea (OR 1.50 (95% CI 1.3 to 1.7); p < 0.001), a posterior approach (compared with an anterior approach, OR 2.80 (95% CI 2.4 to 3.2; p < 0.001), and postoperative dysphagia (OR 2.50 (95% CI 1.8 to 3.4); p < 0.001) were associated with a significantly increased risk of further surgery two years postoperatively. A posterior approach (compared with a combined approach, OR 0.32 (95% CI 0.3 to 0.4); p < 0.001), the use of BMP (OR 0.48 (95% CI 0.4 to 0.5); p < 0.001) were associated with a significantly decreased risk of further surgery at this time. The surgical approach and intraoperative use of BMP strongly influence the risk of further surgery, whereas the comorbidity burden and the characteristics of the operation influence the rates of early complications in adult patients undergoing corrective cervical spinal surgery. These data may aid surgeons in patient selection and surgical planning. Cite this article: Bone Joint J2021;103-B(4):734-738.

At what cost is collagenase clostridium histolyticum viable for treating Peyronie's disease in a public healthcare system?

Collagenase clostridium histolyticum is a recognized non-surgical management for Peyronie's disease, licenced for use in the UK for patients with a palpable plaque and a curvature deformity of at least 30°. However, it is not currently funded for use within the National Health Service. Collagenase clostridium histolyticum has also recently been withdrawn from the European and other markets worldwide, but there is potential for this to be produced off-patent in the future. To determine whether collagenase clostridium histolyticum is cost-neutral when compared to surgical management within a public health care system, using the National Health Service as an example. Two published protocols for the administration of collagenase in Peyronie's disease were identified-the 'IMPRESS protocol' and the 'London protocol'. Costs were taken from published NHS literature. Surgical intervention rates after collagenase clostridium histolyticum administration and primary penile plication were taken from published literature. The costs of the two published protocols were calculated with costs of any repeat surgical intervention were included within each protocol per patient cost. At the current cost per vial of collagenase to the National Health Service, the IMPRESS protocol per patient costs £3,832.77 (143.7%) more than primary surgery, whilst the London protocol costs £70.77 (2.7%) more than primary surgery. At a cost of £548.41 per vial, collagenase administered under the London protocol would be a management option for Peyronie's disease cost neutral to primary corrective surgery. Central funding of collagenase in a public healthcare system would enable the management of Peyronie's disease to be moved to the outpatient setting. For this to be done at no additional cost to the NHS, it would require a cost reduction per vial of collagenase of £23.59 (4.1%), to a cost of £548.41.

Preliminary Analysis From the Craniofacial Collaboration United Kingdom Developmental Outcomes in Children With Sagittal Synostosis.

The Craniofacial Collaboration United Kingdom (CC-UK) was established across the 4 Highly Specialized Craniofacial Centres (HSCCs) in the UK in 2015. This is the first wave of data to be analyzed, looking at 3-year-old children with sagittal synostosis who have had primary corrective surgery. This is a comprehensive, homogenous dataset, looking at parental measures of development and behavior. The results indicate that the majority of children are rated by their parents as falling within one standard deviation of the mean for both developmental and behavioral measures. However, there was a trend across the age groups within the sample which, although not statistically significant, indicates that more developmental difficulties may become apparent as children get older. Behavior was rated as more problematic, and the areas of greatest parental concern were Conduct (challenging or confrontational behavior) and Hyperactivity domains, where 24% of children were rated as within the clinically significant range. Although the majority of children were rated as falling within the average range, the difference in the mean between the sagittal and the normative group was significant in 5 of the 6 behavioral domains. Further research is required to examine whether these findings are stable over time and to look at the mechanism which might be driving these changes. It is anticipated that future CC-UK analysis will elucidate this more clearly.

S8A-02 SESSION 8A: METOPIC SYNOSTOSIS THE CC-UK – ROUTINE DEVELOPMENTAL SCREENING PROTOCOL IN THE UK RESULTS FOR CHILDREN WITH METOPIC AND SAGITTAL SYNOSTOSIS AT 3 AND 5 YEARS OF AGE.

Introduction: The Craniofacial Collaboration UK (the CC-UK) is a Clinical Psychology-led developmental screening pathway for patients from the 4 Highly Specialised Craniofacial Centres in the UK (HSCC). The current paper is the first comparison across suture types, examining the results for 3 and 5-year-old children with sagittal (SS) and metopic (MS) synostosis. This is the first step towards understanding differences in the presentation of these different diagnostic groups, with the aim of facilitating evidence-led targeting of resources to greatest need. Methods: Parents of 3 and 5-year-old children were asked to complete questionnaires regarding both developmental and behavioural aspects: the Ages and Stages Questionnaire (ASQ) and the Strengths and Difficulties Questionnaire (SDQ). Questionnaires are given to families in advance of attendance at one of the HSCC. As both questionnaires are widely used standardised tools, results can be compared with normative data. Children were excluded from the analysis if they had not previously undergone primary corrective surgery, or had a known diagnosis with a genetic or other condition (such as epilepsy) which might be expected to impact their developmental progress. Results: Response rate ranged from 51% for the SDQ in 3-year olds with SS to 81% for the ASQ at 3. ASQ data are reported for 375 children and SDQ data for 275. Comparison with normative data indicated no major deficits or delays for the majority of children with both SS and MS groups at 3 and 5 years. However, there was a consistent difference in results between the two diagnostic groups at both age groups, with lower mean parental ratings across all domains of the ASQ and greater numbers of concerns related to behavioural and social functioning for both age groups for children with MS on the SDQ. Conclusion: Whilst the majority of children in both diagnostic groups fell within the range that would be expected for their age, children with MS were rated by their parents as performing less well on both developmental and behavioural measures across all domains. This is an important indication that there may be differences in performance related to diagnostic group. Despite attempts to exclude children with known conditions, it is important to consider the higher rate of chromosomal abnormalities and other conditions, such as in vitro exposure to medication, that are present in MS and could have an impact on development.

Primary Results of Late Surgical Treatment in Children with Tetralogy of Fallot from a Developing Country

Objectives: The purpose was to show how important is to operate on the Senegalese African children presenting with Tetralogy of Fallot (TOF) regardless of their age. Methods: It is a retrospective, descriptive analysis of data from the Department of Pediatric Cardiology, University Hospital of Tuebingen’s data base which was searched for all cases of TOF in foreign patients younger than 15 years between 05/2004-10/2016. Results: 16 children from Senegal with TOF were referred for treatment. Mean age of patients was 7.0 years (range 0.9 - 14.8 years). Primary corrective surgery was performed in 13/16 patients. A primary shunt procedure was required in 3/16 patients. All patients were discharged in good condition with a median 13 postoperative days. We did not register any deaths. All became asymptomatic with pulse oximetry oxygen saturations greater than 95%. The right ventricular outflow tract (RVOT) gradient was less than 30 mmHg in all patients and 2 patients had moderate pulmonary valvular insufficiency. Conclusions: It was still necessary to operate Senegalese children presenting with TOF irrespective of their age.

Analysis of the impact of race on blood transfusion in pediatric scoliosis surgery.

Surgical correction of pediatric scoliosis is associated with significant blood loss. Minimizing estimated blood loss and blood transfusion is beneficial as transfusions have been associated with increased morbidity, including risk of surgical site infections, longer hospitalizations, and increased cost. Although there is evidence that African-American or Black adults are more likely to require intraoperative blood transfusion compared with Caucasian or White adults, the reasons for this difference are unclear. The electronic records for all patients <18y/o undergoing primary corrective scoliosis surgery by a single pediatric orthopedic surgeon at a single academic medical center between 2013 and 2015 were collected and reviewed. Multivariate models were performed to assess the association between Black race and blood loss/transfusion in primary pediatric scoliosis surgery. In a multivariate model, Black race was independently associated with 1.61 times higher estimated blood loss than White race (P<.01; 95% CI=1.16-2.23). Additionally, compared to a White patient, the odds a Black patient received blood transfusion was 6.25 times higher (P=.03; 95% CI=1.56-25.06) and among the patients who received blood transfusion, Black race was independently associated with 2.61 times greater volume of blood transfusion than White race (P<.01; 95% CI=1.54-4.41). Black race was independently associated with increased estimated blood loss, increased rate of blood transfusion, and increased amount of blood transfused during surgical correction of pediatric scoliosis. Further investigation is needed to better understand the etiology of the disparity and assess opportunities for improving outcomes.

Interrupted right-sided aortic arch: performance of umbilical xenograft after primary neonatal corrective surgery

Interrupted aortic arch (IAA) associated with right-sided aorta descendens is a rare congenital malformation. We present a case of a female patient who was diagnosed with type B IAA and received primary corrective surgery as a neonate. A bovine umbilical vascular graft was used to establish aortic arch continuity. Thirty-nine years later the same patient presents with graft-dilatation and a distal aneurysm requiring a surgery. This case shows a late complication of a biological xenograft in term of long-term material performance. Moreover, it highlights the necessity for regular follow-ups in order to detect and treat late onset complications respectively.

DNA Microarray and Quantitative Analysis Reveal Enhanced Myocardial VEGF Expression with Stunted Angiogenesis in Human Tetralogy of Fallot

Tetralogy of Fallot (ToF) is a cyanotic congenital heart disease with prominent right ventricular hypertrophy (RVH) associated with impaired myocardial oxygen and nutrient supply. Consequently, the right ventricle may manifest in altered molecular phenotype with a number of adaptive and inherited gene profiles which are largely unknown. The aim of the present study was to investigate the myocardial differential gene expression profile and to assess myocardial vascularisation in patients with ToF. DNA microarray analysis on right ventricular biopsies from ToF-patients operated for primary corrective surgery (referred as ToF-1; n=12, mean age 0.5year) and age matched controls (n=6) was validated by Northern hybridisation and RT-PCR. Employing immunohistochemistry and video image analysis expression of vascular endothelial growth factor (VEGF), vascular density (by α-SMA and CD31 staining) and myocyte cross sectional area (Gomori's reticuline staining) were assessed in ToF-1 and adult patients (referred as ToF-2, n=12, mean age 30years) who underwent surgery for pulmonary regurgitation and compared the data with respective age matched controls (n=6/12). DNA microarray analysis revealed altered expression pattern for 236 genes including enhanced (1.5-2.2-fold) expression of angiogenic factors and their receptors including; VEGF, flt-1, flk-1 angiopoietin-2, FGF-2, FGF-R1, PDGF-A, whereas, flt-4, Tie, TGF-β, TGF-β3R showed decreased (1.6-3.4-fold) expression in ToF-patients. Northern blot analysis verified the expression patterns of VEGF and flk-1 in both ToF-1 and ToF-2 patients. VEGF staining in cardiomyocytes was increased in ToF-1 (1.5-fold, p<0.05) as compared to ToF-2. Video image analysis revealed enhanced vascular density (p<0.01) with enlarged myocyte cross sectional area (p<0.01), but vascular wall thickness remained unchanged in ToF-1 patients as compared to age matched controls. Our data suggest that RVH is associated with profound changes in gene profile for a number of genes, where VEGF/VEGF-R system contributes to enhance, but stunted myocardial angiogenesis in patients with ToF.

Modified Blalock Taussig shunt: a not-so-simple palliative procedure

Thirty-two consecutive isolated modified Blalock Taussig (BT) shunts performed in infancy since 2004 were reviewed and analysed to identify the risk factors for shunt intervention and mortality. Sternotomy was the only approach used. Median age and weight were 10.5 (range 1-74) days and 2.9 (1.9-4.4) kg, respectively. Shunt palliation was performed for biventricular hearts (Tetralogy of Fallot/double outlet right ventricle/transposition of great arteries_ventricular septal defect_pulmonary stenosis/pulmonary atresia_ventricular septal defect/others) in 21, and univentricular hearts in 11, patients. Hypoplastic left heart syndrome patients were excluded. Two procedures required cardiopulmonary bypass. Median shunt size was 3.5 (3-4) mm and median shunt size/kg body weight was 1.2 (0.9-1.7) mm/kg. Reduction in shunt size was necessary in 5 of 32 (16%) patients. Three of 32 (9%) patients died after 3 (1-15) days due to cardiorespiratory decompensation. Lower body weight (P = 0.04) and bigger shunt size/kg of body weight (P = 0.004) were significant risk factors for mortality. Acute shunt thrombosis was observed in 3 of 32 (9%), none leading to death. Need for cardiac decongestive therapy was associated with univentricular hearts (P < 0.001), bigger shunt size (P = 0.054) and longer hospital stay (P = 0.005). Twenty-eight patients have undergone a successful shunt takedown at a median age of 5.5 (0.5-11.9) months, without late mortality. Palliation with a modified BT shunt continues to be indicated despite increased thrust on primary corrective surgery. Though seemingly simple, it is associated with significant morbidity and mortality. Effective over-shunting and acute shunt thrombosis are the lingering problems of shunt therapy.