Double Aortic Arch: A Comparison of Fetal CMR, Postnatal CT and Surgical Findings.

BACKGROUND: In double aortic arch (DAA) one of the arches can demonstrate atretic portions postnatally, leading to diagnostic uncertainty due to overlap with isolated right aortic arch (RAA) variants. The main objective of this study is to demonstrate the morphological evolution of different DAA phenotypes from prenatal to postnatal life using 3D fetal cardiac magnetic resonance imaging (CMR) and postnatal CT/CMR imaging. METHODS: 3D fetal CMR was undertaken in fetuses with suspected DAA over a six-year period (Jan 2016 - Jan 2022). All cases with surgical confirmation of DAA were retrospectively studied and morphology on fetal CMR was compared to postnatal CT/CMR and surgical findings. RESULTS: 32 fetuses with surgically confirmed DAA underwent fetal CMR. All demonstrated a complete DAA with left-sided arterial duct. The RAA was dominant in 30/32 (94%). Postnatal CT/CMR was undertaken at median age of 3.3months (IQR 2.0-3.9) demonstrating DAA with patency of both arches in 9/32 (28%), with 6 showing signs of coarctation of the left aortic arch (LAA). The LAA isthmus was not present on CT/CMR in 22/32(69%), the transverse arch between left carotid and left subclavian artery was not present in 1 case. CONCLUSIONS: Fetal CMR provides novel insights into perinatal evolution of DAA. The smaller LAA can develop coarctation or atresia related to postnatal constriction of the arterial duct, making diagnosis of DAA challenging with contrast-enhanced CT/CMR. This highlights the potentially important role for prenatal 3D vascular imaging and might improve intepretation of postnatal imaging.

Transcatheter versus surgical treatment for isolated superior sinus venosus atrial septal defect.

BACKGROUND: The superior sinus venosus atrial septal defect is a congenital communication between the left and right atria. Open surgical approach by patch closure has historically been the only treatment option. Recently, a transcatheter approach has been developed. This study aims to compare the efficacy and safety of surgical and transcatheter approach in treatment of sinus venosus atrial septal defect. METHODS: Between March 2010 and December 2020, 58 patients (median age: 45.4, range 14.8-73.8) underwent either surgical or transcatheter correction of superior sinus venosus atrial septal defect with partial anomalous pulmonary venous drainage. RESULTS: Twenty-four patients (median age: 35.4, range 14.8-66.8) underwent surgery while 34 patients (median age: 46.8, range 15.5-73.8) had a transcatheter treatment. During the catheterization era, 41 patients was considered suitable for a transcatheter closure. In 5 patients, surgery was the patient's or referring physician's choice. In 2 cases, the procedure was unsuccessful; the remaining 34 were successfully closed (94.4% of cases). Intensive care unit stay (median of 1 day, range 0.5-4, vs. 0, range 0-2, p < 0.0001) and hospital stay (median 7 days, range 2-15 vs. 2 days, range 1-12, p < 0.0001), were significantly longer in the surgery group. Total early complication rate, consisted on procedural and in-hospital complication, were higher in the surgical group (62.5% vs. 23.5%; p = 0.005). However, complications in both groups were clinically mild. At follow-up, a small residual shunt was present in 6 patients (surgery group: 2 pts; catheterization group: 4 pts; p: NS). Imaging studies showed significant improvement of right ventricular size and unobstructed pulmonary venous return in all patients. No late complications occurred at follow-up. CONCLUSIONS: Transcatheter correction of sinus venosus atrial septal defect is effective and safe in selected patients and may be considered as a valid alternative to surgery.

Post-operative Outcomes for Vascular Rings: A Systematic Review and Meta-analysis.

OBJECTIVES: Vascular rings may cause tracheal and/or oesophageal compression. For many patients, symptoms/signs have been present for a long period before diagnosis. However, in the era of prenatal diagnosis, some units advocate universal early surgery. The risks and efficacy of surgery must be known to adequately counsel for the operation. This meta-analysis sought to define the morbidity and mortality associated with surgical correction, and persistent post-operative symptoms. METHODS: PubMed, Cochrane Library and CINAHL databases were searched for studies that described the outcome of patients undergoing surgery for a double or right aortic arch (DAA or RAA). Non-comparative and random effects model-based meta-analyses were conducted to calculate the pooled rates of mortality, surgical complications, reintervention, and persistent follow-up symptoms. RESULTS: Nineteen eligible studies were included comprising 18 studies describing outcomes for DAA surgery and 15 for RAA surgery. For DAA surgery, overall mortality rate was 0% [95% confidence interval (CI) 0.0-1.0], post-surgical complication rate 18% [95% CI: 12.0-23.0], prevalence of reintervention 3% [95% CI: 1.0-5.0] and prevalence of symptoms at last follow-up was 33% [95% CI: 17.0-52.0]. For RAA surgery, overall pooled mortality was 0% [95% CI: 0.0-0.0], prevalence of post-surgical complications was 15% [95% CI: 8.0-23.0], reintervention rate was 2% [95% CI: 0.0-4.0], prevalence of symptoms at last follow-up was 40% [95% CI: 26.0-55.0]. CONCLUSIONS: While surgery to correct a vascular ring is safe, the rate of persistent symptoms is high and further strategies must be sought to reduce this burden.

Contemporary surgical outcome and symptomatic relief following vascular ring surgery in children: effect of prenatal diagnosis.

OBJECTIVES: Our goal was to describe postoperative complications and outcomes in a large contemporary cohort of children with an isolated double aortic arch (DAA) or a right aortic arch (RAA) with left arterial ligament and to assess the impact of foetal diagnosis on outcomes. METHODS: We performed a retrospective analysis of all patients who underwent surgery for DAA or RAA with left arterial ligament between 2005 and 2019. RESULTS: A total of 132 children were operated on for a DAA (n = 77) or a RAA (n = 55). Prenatal diagnosis was made in 100/132 (75.8%). Median age at surgery for DAA was 5.0 (1.7-13.3) months and for RAA was 13.9 (6.4-20.1) months. There was no difference in the age at surgery between the prenatal and postnatal cases (8.6 [4.0-15.6] vs 5.4 months [1.8-17.7]; P = 0.37). No surgical deaths occurred. Vocal cord palsy was the most common complication, occurring in 12/132 (9%): of these, 11 resolved spontaneously and 1 required a temporary tracheostomy. Logistic regression demonstrated that older age at operation was the only predictor for a postoperative complication (P = 0.02). Overall, 21/67 (31%) of prenatally detected, symptomatic cases reported residual symptoms/signs 1 year after surgery compared to 18/28 (64%) of postnatally detected cases. Postnatal diagnosis was associated with persistent postoperative symptoms/signs [P = 0.006, odds ratio = 3.9 (95% confidence interval 1.5-9.4)]. CONCLUSIONS: Surgery to relieve a vascular ring resolves trache-oesophageal compressive symptoms in most cases, but parents/patients should be aware that symptoms/signs may persist in the first postoperative year despite effective release of the vascular ring. Earlier surgery and prenatal diagnosis may improve outcomes.

Multisite Veno-Venous Cannulation for Neonates and Nonambulatory Children.

OBJECTIVES: Neonates with respiratory failure are ideally supported with veno-venous rather than veno-arterial extracorporeal membrane oxygenation due to the reduced rate of neurologic complications. However, the proportion of neonates supported with veno-venous extracorporeal membrane oxygenation is declining. We report multisite veno-venous extracorporeal membrane oxygenation, accessing the neck, returning to the inferior vena cava via the common femoral vein in neonates and children less than 10 kg. DESIGN: Retrospective case series with 1 year minimum follow-up. PATIENTS: Patients less than 10 kg supported with veno-venous extracorporeal membrane oxygenation accessing the jugular and returning to the femoral vein. SETTING: A 30-bed pediatric intensive care delivering extracorporeal membrane oxygenation to approximately 20 children annually. INTERVENTIONS: Veno-venous extracorporeal membrane oxygenation accessing the jugular and returning to the femoral vein was delivered using two single lumen cannulae. MEASUREMENTS AND MAIN RESULTS: January 2015 to August 2019, 11 patients underwent veno-venous extracorporeal membrane oxygenation accessing the jugular and returning to the femoral vein with median weight of 3.6 kg (interquartile range 2.8-6.1 kg), and median corrected gestational age of 13 days (interquartile range, 2-175 d). The smallest patient weighed 2.1 kg. Seven patients had comorbidities. Extracorporeal membrane oxygenation was technically successful in all patients with median flows of 126 mL/kg/min (interquartile range, 120-138 mL/kg/min) and median arterial oxygenation saturation of 94% (interquartile range, 91-98%) at 24 hours. Nine survived to home discharge, and two were palliated. Common femoral vein occlusion was observed in all patients on ultrasound post decannulation. There was no clinical or functional deficit in the cannulated limb at follow-up, a minimum of 1 year post extracorporeal membrane oxygenation. CONCLUSIONS: Veno-venous extracorporeal membrane oxygenation accessing the jugular and returning to the femoral vein was performed safely in patients under 10 kg with the smallest patient weighing 2.1 kg. Although occlusion of the common femoral vein was observed in patients post decannulation, subsequent follow-up demonstrated no clinical implications. We challenge current practice that veno-venous extracorporeal membrane oxygenation accessing the jugular and returning to the femoral vein cannot be performed in nonambulatory patients and suggest that this strategy is preferred over veno-arterial extracorporeal membrane oxygenation in infants requiring extracorporeal membrane oxygenation for respiratory failure.

Cardiac strangulation after epicardial pacing: the importance of non-specific symptoms and a low index of suspicion.

Cardiac strangulation is a rare and potentially deadly complication of epicardial pacemaker implantation. A young boy presenting with chest pain and tiredness almost 7 years after pacemaker implantation was found to have cardiac strangulation. Literature review revealed 22 cases reported to date with a worrying rise in the number of reports over the past 3 years. Strangulation is associated with implantation of leads at a young age and appears to be related to somatic growth. Serial assessment with chest X-ray and echocardiogram is recommended, at least until full adult growth is attained with further coronary artery imaging reserved for symptoms or suspicious echocardiographic findings. If cardiac strangulation is diagnosed prompt replacement of the offending system is needed.

Postnatal impact of a prenatally diagnosed double aortic arch.

BACKGROUND: A double aortic arch (DAA) is increasingly identified before birth; however, there are no published data describing the postnatal outcome of a large prenatal cohort. OBJECTIVE: To describe the associations, symptoms and impact of prenatally diagnosed DAA. METHODS: Retrospective review of consecutive cases seen at two fetal cardiology units from 2014 to 2019. Clinical records including symptoms and assessment of tracheobronchial compression using flexible bronchoscopy were reviewed. Moderate-severe tracheal compression was defined as >75% occlusion of the lumen. RESULTS: There were 50 cases identified prenatally and 48 with postnatal follow-up. Array comparative genomic hybridisation (aCGH) was abnormal in 2/50 (4%), aCGH was normal in 33/50 (66%) and of those reviewed after birth, 13 were phenotypically normal. After birth, there was a complete DAA with patency of both arches in 8/48 (17%) and in 40/48 (83%) there was a segment of the left arch which was a non-patent, ligamentous connection.Stridor was present in 6/48 (13%) on the day of birth. Tracheo-oesophageal compressive symptoms/signs were present in 31/48 (65%) patients at median age of 59 days (IQR 9-182 days). Tracheal/carinal compression was present in 40/45 (88%) cases. Seven of 17 (41%) asymptomatic cases demonstrated moderate-severe tracheal compression. All morphologies of DAA caused symptoms and morphology type was not predictive of significant tracheal compression (p=0.3). CONCLUSIONS: Genetic testing should be offered following detection of double aortic arch. Early signs of tracheal compression are common and therefore delivery where onsite neonatal support is available is recommended. Significant tracheal compression may be present even in the absence of symptoms.

Factors Behind Decline of Venovenous Extracorporeal Membrane Oxygenation to Support Neonatal Respiratory Failure.

OBJECTIVES: Neonates with respiratory failure are ideally supported with venovenous rather than venoarterial extracorporeal membrane oxygenation due to the reduced rate of neurologic complications. However, the proportion of neonates supported with venovenous extracorporeal membrane oxygenation is declining. We aimed to explore the factors behind this decline. DESIGN: We conducted a survey in order to understand current practice across the United Kingdom, exploring barriers to neonatal venovenous extracorporeal membrane oxygenation in addition to collecting U.K.-wide retrospective data from 2000 onwards. PATIENTS: Neonates with respiratory failure supported with extracorporeal membrane oxygenation in the United Kingdom. SETTING: All 11 pediatric U.K. extracorporeal membrane oxygenation centers provided data. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The percentage of neonates with respiratory failure supported with venovenous extracorporeal membrane oxygenation has declined from 58% to 16% over the last decade, reflected internationally with a decrease of 1.3% per year. Nine extracorporeal membrane oxygenation centers are able to offer pediatric venovenous extracorporeal membrane oxygenation, but only four offer neonatal venovenous extracorporeal membrane oxygenation. Of those able to provide neonatal venovenous extracorporeal membrane oxygenation, three centers use dual lumen cannulae and one uses neonatal multisite cannulation. The most frequently cited barriers to neonatal venovenous extracorporeal membrane oxygenation were the lack of availability of a suitable dual lumen cannula with concerns of cardiac perforation and challenges of multisite cannulation due to small caliber femoral veins. CONCLUSIONS: The majority of U.K. centers are unable to provide venovenous extracorporeal membrane oxygenation for neonates due to concerns with availability and suitability of dual lumen cannulae. An alternative to be considered is the use of multisite cannulation, which has been used historically and is currently being used in centers internationally and in the United Kingdom.

Use of an Extracellular Matrix Patch for Sternal Wound Dehiscence after Cardiac Surgery in a Neonate.

Management of sternal wound dehiscence in newborns after cardiac operations can be a slow and lengthy process, during which the risk of progression to deep sternal wound infection and mediastinitis remains a concern. We report the case of a neonate born with single-ventricle physiology who underwent a Damus-Kaye-Stansel procedure as first-stage palliation toward creating Fontan circulation. The postoperative period was characterized by sterile wound dehiscence of the subcutaneous layers. We used a CorMatrix extracellular matrix patch as an adjunct to repair the wound defect. After 7 weeks, the wound had healed with excellent results. To our knowledge, this is the first report of extracellular matrix patch implantation for sternal wound reconstruction in a neonate.

Giant Right Atrial Aneurysm: Antenatal Diagnosis and Surgical Treatment.

Giant congenital right atrial aneurysms are rare, with their natural history complicated by death, heart failure, arrhythmias, and thrombosis. Prenatal diagnosis of the defect is feasible and allows an early preparation of a plan for management. We present details of a patient diagnosed prenatally with a giant right atrial aneurysm, which was successfully treated surgically as soon as the patient became symptomatic.

Intramural Left Coronary Artery in Truncus Arteriosus.

An intramural coronary artery in the setting of truncus arteriosus (common arterial trunk) is an uncommon association. Following an uneventful surgical repair, a neonate developed a low cardiac output state deteriorating into cardiac arrest shortly after arrival into the intensive care unit, requiring extracorporeal membrane oxygenation support. Echocardiography and angiography showed occlusion of the left coronary artery, prompting emergency surgical reexploration. A "slit-like" orifice with an intramural left coronary artery was successfully unroofed, allowing full recovery. Full definition of the proximal coronary anatomy beyond the orifices should be investigated preoperatively in truncus arteriosus, as a missed intramural segment could lead to significant morbidity or mortality.

Trifecta St. Jude medical® aortic valve in pulmonary position.

Introduction: To evaluate an aortic pericardial valve for pulmonary valve (PV) regurgitation after repair of congenital heart defects. Methods: From July 2012 to June 2016 71 patients, mean age 24 ± 13 years (four to years) underwent PV implantation of aortic pericardial valve, mean interval after previous repair = 21 ± 10 years (two to 47 years). Previous surgery at mean age 3.2 ± 7.2 years (one day to 49 years): tetralogy of Fallot repair in 83% (59/71), pulmonary valvotomy in 11% (8/71), relief of right ventricular outflow tract (RVOT) obstruction in 6% (4/71). Pre-operative echocardiography and MRI showed severe PV regurgitation in 97% (69/71), moderate in 3% (2/71) with associated RVOT obstruction. MRI and knowledge-based reconstruction 3D volumetry (KBR-3D-volumetry) showed mean PV regurgitation = 42 ± 9% (20-58%), mean indexed RV end-diastolic volume = 169 ± 33 (130-265) ml m-2 BSA and mean ejection fraction (EF) = 46 ± 8% (33-61%). Cardio-pulmonary exercise showed mean peak O2/uptake = 24 ± 8 ml kg-1 min-1 (14-45 ml kg-1 min-1), predicted max O2/uptake 66 ± 17% (26-97%). Pre-operative NYHA class was I in 17% (12/71) patients, II in 70% (50/71) and III in 13% (9/71). Results: Mean cardio-pulmonary bypass duration was 95 ± 30' (38-190'), mean aortic cross-clamp in 23% (16/71) 46 ± 31' (8-95'), with 77% (55/71) implantations without aortic cross-clamp. Size of implanted PV: 21 mm in seven patients, 23 mm in 33, 25 mm in 23, and 27 mm in eight. The z-score of the implanted PV was -0.16 ± 0.80 (-1.6 to 2.5), effective orifice area indexed (for BSA) of native PV was 1.5 ± 0.2 (1.2 to -2.1) vs. implanted PV 1.2 ± 0.3 (0.76 to -2.5) (p = ns). In 76% (54/71) patients surgical RV modelling was associated. Mean duration of mechanical ventilation was 6 ± 5 h (0-26 h), mean ICU stay 21 ± 11 h (12-64 h), mean hospital stay 6 ± 3 days (three to 19 days). In mean follow-up = 25 ± 14 months (six to 53 months) there were no early/late deaths, no need for cardiac intervention/re-operation, no valve-related complications, thrombosis or endocarditis. Last echocardiography showed absent PV regurgitation in 87.3% (62/71) patients, trivial/mild degree in 11.3% (8/71), moderate degree in 1.45% (1/71), mean max peak velocity through RVOT 1.6 ± 0.4 (1.0-2.4) m s-1. Mean indexed RV end-diastolic volume at MRI/KBR-3D-volumetry was 96 ± 20 (63-151) ml m-2 BSA, lower than pre-operatively (p < 0.001), and mean EF = 55 ± 4% (49-61%), higher than pre-operatively (p < 0.05). Almost all patients (99% = 70/71) remain in NYHA class I, 1.45% = 1/71 in class II. Conclusion: (a) Aortic pericardial valve is implantable in PV position with an easy and reproducible surgical technique; (b) valve size adequate for patient BSA can be implanted with simultaneous RV remodelling; (c) medium-term outcomes are good with maintained PV function, RV dimensions significantly reduced and EF significantly improved; (d) adequate valve size will allow later percutaneous valve-in-valve implantation.

Virtual 3D Modeling of Airways in Congenital Heart Defects.

The involvement of the airway is not uncommon in the presence of complex cardiovascular malformations. In these cases, a careful inspection of the relationship between the airway and the vasculature is paramount to plan the surgical procedure. Three-dimensional printing enhanced the visualization of the cardiovascular structure. Unfortunately, IT does not allow to remove selected anatomy to improve the visualization of the surrounding ones. Computerized modeling has the potential to fill this gap by allowing a dynamic handling of different anatomies, increasing the exposure of vessels or bronchi to show their relationship. We started to use this technique to plan the surgical repair in these complex cases where the airway is affected. This technique is routinely used in our Institution as an additional tool in the presurgical assessment. We report four cases in which the airways were compressed by vascular structures - ascending aorta in one, left pulmonary artery sling in one, patent ductus arteriosus in one, and major aorto-pulmonary collateral artery in one. We believe this technique can enhance the understanding of the causes of airway involvement and facilitate the creation of an appropriate surgical plan.

The influence of stents on microbial colonization of the airway in children after slide tracheoplasty: a 14-year single-center experience.

OBJECTIVES: This study describes the microbial colonization profile of the airway in children after slide tracheoplasty (STP) with and without stents, and compares colonization to children undergoing cardiothoracic surgical procedures without airway related disease. METHODS: A 14-year retrospective single case note review was performed on patients undergoing STP and stent insertion. Nose and throat (NT) and bronchoalveolar lavage (BAL) specimens were analyzed for microbial profile and expressed as cumulative mean microorganisms per patient (MMP). RESULTS: Forty-three patients (median age ± SD 15.02 ± 31.76 months) underwent STP and 141 patients underwent cardiothoracic but no airway surgery (median age ± SD 31.7 ± 47.2 months). Sixteen patients required a stent after STP. One-hundred seventy-two positive microbial specimens were identified. The predominant 6 microorganisms were (1) Staphylococcus aureus; (2) Pseudomonas aeruginosa; (3) Haemophilus influenzae not type B; (4) Coliforms; (5) Streptococcus pneumoniae; and (6) Candida Albicans, and accounted for 128 (74%) of all positive specimens found. Children with stents had more MMP compared to children without stents after STP [4.06 ± 2.38 and 2.04 ± 2.24 MMP (P < 0.001), respectively]. Both groups of children after STP had more MMP compared to the control group (P < 0.001). Children with stents had more microbial colonization of their lower respiratory tract compared to their upper respiratory tract (3.36 ± 2.02 and 1.36 ± 0.93 MMP (P < 0.01) respectively). Staphylococcus aureus colonization of the lower respiratory tract was significantly higher in children with stents compared to children without stents after STP [0.5 and 0.15 MMP (P < 0.05) respectively]. CONCLUSIONS: This study indicates airway surgery and the subsequent use of stents to be a significant risk factor for microbial colonization of the airway in children. More specifically airway stents appear to increase colonization in the distal airway, which appears unrelated to that of the upper respiratory tract.

Outcomes of slide tracheoplasty in 101 children: a 17-year single-center experience.

OBJECTIVE: Our study describes the results from surgical slide tracheoplasty (STP) in children with long segment tracheal stenosis. METHODS: Demographic and preoperative conditions, operative details, and outcome measures, including the need for endoscopic airway intervention and mortality, were collected for children undergoing STP between February 1995 and December 2012. RESULTS: One hundred one patients (median age, 5.8 months; range, 5 days-15 years) underwent STP. Seventy-two patients (71.3%) had associated cardiovascular anomalies. Preoperative ventilation was necessary in 56 patients (55.4%), whereas extracorporeal membrane oxygenation was required in 10 patients (9.9%). Abnormal bronchial arborization was present in 39 children (38.6%), which included 13 patients (12.8%) with an anomalous right upper lobe bronchus and 17 patients (16.8%) with tracheal trifurcation. Airway stenosis extended into 1 or both bronchi in 24 patients (23.7%) and preoperative malacia was present in 24 patients (23.7%). STP was extended into the bronchus in 47 patients (46.5%). Overall survival was 88.2% (mortality in 12 patients). Post-STP balloon dilation was necessary in 45 patients (44.6%) and stenting was required in 22 patients (21.8%). Multivariate analysis revealed preoperative extracorporeal membrane oxygenation (P < .05), preoperative malacia (P < .001), and bronchial stenosis (P < .05) to be adverse predictors of survival. Preoperative malacia was a significant risk factor for stenting (P < .05). CONCLUSIONS: STP is a versatile and reliable technique associated with low morbidity and mortality when compared with previous strategies for children with long segment tracheal stenosis. The presence of preoperative bronchomalacia is a significant risk factor for death and postoperative stenting.

Pump controlled retrograde trial off from VA-ECMO.

We describe our novel technique of Pump Controlled Retrograde Trial Off that relies on the retrograde flow to maintain circuit integrity and allow a longer trial off from venoarterial extracorporeal membrane oxygenation support without circuit clot formation or significant patient hemodynamic compromise. This technique avoids the insertion of an arteriovenous bridge and the need to clamp the circuit. We present data on five neonatal patients who were trialled off using this method.

Pediatric coronary artery revascularization: a European multicenter study.

BACKGROUND: We sought to evaluate the hospital and midterm results of different surgical revascularization techniques in pediatric patients within the European Congenital Heart Surgeons Association. METHODS: From 1973 to 2011, 80 patients from 13 European Congenital Heart Surgeons Association centers underwent 65 pediatric coronary artery bypass grafting (PCABG) and 27 other coronary artery procedures (OCAP; 12 patients had combined PCABG and other coronary artery procedures). Excluded were patients with Kawasaki disease. Median age at the time of coronary procedure was 2.3 years (range, 2 days to 16.9 years); 33 patients (41.2%) were younger then 12 months. An emergency procedure was necessary in 34 patients (42.5%). RESULTS: Twelve patients (15%) died in the hospital; age at surgery (p=0.02) and the need for an emergent procedure (p=0.0004) were related to hospital mortality. Median follow-up time was 7.6 years (range, 0.9 to 23 years). There were 3 late cardiac deaths, all after a median time of 4 years (range, 9 months to 8.8 years) after PCABG. Fourteen patients (20.5%) presented with symptoms, including congestive heart failure (n=10) and angina (n=4), that were significantly associated with a low ejection fraction (p<0.001) and the presence of moderate or severe mitral valve regurgitation (p=0.0003). Six patients underwent a reintervention for impaired myocardial perfusion; all of them had a stenotic or atretic PCABG (p=0.001), and the majority were symptomatic (5 of 6 patients; 83.3%; p=0.001). CONCLUSIONS: Both PCABG and other coronary artery procedures are suitable surgical options in pediatric patients with impaired myocardial perfusion, which increases operative and midterm survival. Such population of patients needs to be followed for life to prevent and treat any possible cause of further myocardial ischemia.

Aortopexy for the treatment of tracheomalacia in children: review of the literature.

Severe tracheomalacia presents a significant challenge for Paediatricians, Intensivists, Respiratory Physicians, Otolaryngologists and Paediatric Surgeons. The treatment of tracheomacia remains controversial, but aortopexy is considered by most to be one of the best options. We conducted a review of the English literature relating to aortopexy.Among 125 papers, 40 have been included in this review. Among 758 patients (62% males) affected with tracheomalacia, 581 underwent aortopexy. Associated co-morbidities were reported in 659 patients. The most frequent association was with oesophageal atresia (44%), vascular ring or large vessel anomalies (18%) and innominate artery compression (16%); in 9% tracheomalacia was idiopathic. The symptoms reported were various, but the most important indication for aortopexy was an acute life-threatening event (ALTE), observed in 43% of patients. The main preoperative investigation was bronchoscopy. Surgical approach was through a left anterior thoracotomy in 72% of patients, while median approach was chosen in 14% and in 1.3% a thoracoscopic aortopexy was performed. At follow-up (median 47 months) more than 80% of the patients improved significantly, but 8% showed no improvement, 4% had a worsening of their symptoms and 6% died. Complications were observed in 15% of patients, in 1% a redo aortopexy was deemed necessary.In our review, we found a lack of general consensus about symptom description and evaluation, indications for surgery, though ALTE and bronchoscopy were considered by all an absolute indication to aortopexy and the gold standard for the diagnosis of tracheomalacia, respectively. Differences were reported also in surgical approaches and technical details, so that the same term "aortopexy" was used to describe different types of procedures. Whatever approach or technique was used, the efficacy of aortopexy was reported as high in the majority of cases (more than 80%). A subgroup of patients particularly delicate is represented by those with associated gastro-esophageal reflux, in whom a fundoplication should be performed. Other treatments of tracheomalacia, particularly tracheal stenting, were associated with a higher rate of failure, severe morbidity and mortality.

Stem-cell-based, tissue engineered tracheal replacement in a child: a 2-year follow-up study.

BACKGROUND: Stem-cell-based, tissue engineered transplants might offer new therapeutic options for patients, including children, with failing organs. The reported replacement of an adult airway using stem cells on a biological scaffold with good results at 6 months supports this view. We describe the case of a child who received a stem-cell-based tracheal replacement and report findings after 2 years of follow-up. METHODS: A 12-year-old boy was born with long-segment congenital tracheal stenosis and pulmonary sling. His airway had been maintained by metal stents, but, after failure, a cadaveric donor tracheal scaffold was decellularised. After a short course of granulocyte colony stimulating factor, bone marrow mesenchymal stem cells were retrieved preoperatively and seeded onto the scaffold, with patches of autologous epithelium. Topical human recombinant erythropoietin was applied to encourage angiogenesis, and transforming growth factor ß to support chondrogenesis. Intravenous human recombinant erythropoietin was continued postoperatively. Outcomes were survival, morbidity, endoscopic appearance, cytology and proteomics of brushings, and peripheral blood counts. FINDINGS: The graft revascularised within 1 week after surgery. A strong neutrophil response was noted locally for the first 8 weeks after surgery, which generated luminal DNA neutrophil extracellular traps. Cytological evidence of restoration of the epithelium was not evident until 1 year. The graft did not have biomechanical strength focally until 18 months, but the patient has not needed any medical intervention since then. 18 months after surgery, he had a normal chest CT scan and ventilation-perfusion scan and had grown 11 cm in height since the operation. At 2 years follow-up, he had a functional airway and had returned to school. INTERPRETATION: Follow-up of the first paediatric, stem-cell-based, tissue-engineered transplant shows potential for this technology but also highlights the need for further research. FUNDING: Great Ormond Street Hospital NHS Trust, The Royal Free Hampstead NHS Trust, University College Hospital NHS Foundation Trust, and Region of Tuscany.

Congenital absence of cartilaginous tracheal rings associated with esophageal atresia and trifurcated carina: a novel anomaly?

Tracheomalacia associated with esophageal atresia (EA) is a well-known condition. However, complete absence of tracheal rings (TRs) is extremely rare. Our aim is to describe a novel triad of conditions and to discuss the best treatment. An expremature male operated for EA presented with severe respiratory distress. The diagnosis of absent cartilage rings, suspected on bronchoscopy, was confirmed by optical coherence tomography. The absence of TRs was localized to a short tracheal segment, and the carina trifurcated into right upper lobe, right intermediate, and left main bronchus. The patient was treated with resection and anastomosis with a completely satisfactory course. Absence of TRs was previously reported by us in 2 other cases, both with associated EA and trifurcation of the carina. One child was treated with tracheostomy and the other with a stent, but the outcome was far from optimal. The patient with tracheostomy eventually underwent resection and anastomosis with tracheostomy closure. Congenital absence of TRs is extremely rare. Although localized, it is responsible for severe symptoms owing to complete tracheal collapse and may be misdiagnosed as tracheomalacia. In our experience, it has been associated with EA and trifurcated carina. Our limited experience suggests resection of the abnormal segment and tracheal anastomosis as the best treatment.