Interventions on residual lesions in patients with heterotaxy syndrome following orthotropic heart transplantation: A single-center experience.

BACKGROUND: Heterotaxy syndrome (HS) is a defect in lateralization which often results in complex intra and extracardiac abnormalities. Orthotropic heart transplantation (OHT) in HS involves intricate and individualized modifications to surgical technique. Post-OHT outcomes are worse in patients with HS, however, the impact of post-OHT residual lesions has not yet been characterized. METHODS: Patients with HS who underwent OHT at Ann & Robert H. Lurie Children's Hospital of Chicago between January 2012 and June 2023 were identified. Patients were excluded if follow-up data was not available due to follow up at a different institution of early mortality. Pre-OHT clinical data, surgical data, and post-OHT surgical and catheterization data were collected. RESULTS: Two early mortalities were excluded from analysis, leaving 15 patients in the study cohort. Median age at OHT was 3.7 years (range: 0.7-15.4). Nine out of 15 patients were diagnosed with residual lesions requiring intervention at a median of 188 days post transplantation. All interventions on residual lesions occurred via catheterization. Overall mortality rate was 27% (4/15) with all deaths occurring in patients with residual lesions (4/9 patients, 44%). 83% (10/12) of lesions were diagnosed via catheterization, and 83% (10/12) of lesions of occurred in the first year after transplant. CONCLUSIONS: Patients with HS are at high risk for residual lesions after OHT, which may contribute to increased mortality. Comprehensive invasive diagnostics were required to diagnose residual lesions, which were all addressed percutaneously.

The aortic uncrossing procedure.

OBJECTIVE: Cross-sectional imaging allows identification of rare patients with a vascular ring and circumflex aorta. The key diagnostic feature is crossing of the transverse aortic arch from right to left posterior to the trachea and superior to the carina in a patient with a right aortic arch. We evaluated our patients who received an aortic uncrossing procedure. METHODS: We reviewed all patients who underwent aortic uncrossing from 2002 to 2022. All patients received preoperative computed tomography imaging and bronchoscopy. RESULTS: Eleven patients ranging in age from 1.5 to 10 years (median 4 years) underwent aortic uncrossing. Two patients had prior left ligamentum division, and 3 patients had prior left aortic arch division. All had significant clinical symptoms. Eight patients had deep hypothermic circulatory arrest (mean 34 minutes), and 3 patients had antegrade cerebral perfusion (median, 28 minutes). Patch material was not used for aortic augmentation, and no patient underwent a posterior tracheopexy or rotational esophagoplasty. Postoperative length of stay ranged from 4 to 31 days (median, 5 days). One patient required a temporary tracheostomy for bilateral recurrent laryngeal nerve paresis, which recovered. One patient required an aortic extension graft to alleviate esophageal compression from an unusual ectatic esophageal course. All patients had relief of airway symptoms and dysphagia. CONCLUSIONS: In properly selected patients with a right aortic arch and circumflex aorta, aortic uncrossing is a safe and effective therapy to treat airway and esophageal compression. The procedure can be conducted with deep hypothermic circulatory arrest or antegrade cerebral perfusion. Careful attention to the location of the esophagus and recurrent laryngeal nerves is required.

Association Between Prenatal Diagnosis and Age at Surgery for Noncritical and Critical Congenital Heart Defects.

BACKGROUND: The relationship between the prenatal diagnosis of congenital heart defects (CHDs) and age at CHD surgery is poorly understood, despite the known relationships between age at surgery and long-term outcomes. The objective of this study was to determine the associations between prenatal diagnosis of CHD and age at surgery, and whether these associations differ for critical and noncritical CHDs. METHODS: This is a cohort analysis of patients aged 0 to 9 years who received their initial cardiac surgery at Ann & Robert H. Lurie Children's Hospital of Chicago between 2015 and 2021 with prenatal diagnosis as the exposure variable. All data were obtained from the locally maintained Society of Thoracic Surgeons Congenital Heart Surgery Database at Lurie Children's Hospital. We used multivariable fixed effects regression models to estimate the strength of the association of prenatal diagnosis with age at surgery among patients with critical (surgery ≤60 days) and noncritical (surgery >60 days) CHDs. RESULTS: Of 1131 individuals who met inclusion criteria, 532 (47%) had a prenatal diagnosis, 428 (38%) had critical CHDs, 533 (47%) were female, and the median age at surgery was 119 days (interquartile range, 11-309 days). After controlling for demographics, comorbidities, and surgical complexity, the mean age at surgery was significantly younger in those with prenatally versus postnatally diagnosed critical CHD (7.1 days sooner, P<0.001) and noncritical CHDs (atrial septal defects [12.4 months sooner, P=0.037], ventricular septal defects [6.0 months sooner, P<0.003], and noncritical coarctation of the aorta [1.8 months sooner, P=0.010]). CONCLUSIONS: Younger age at CHD surgery, which is associated with postsurgical neurodevelopmental and physical outcomes, is significantly associated with prenatal CHD diagnosis. This relationship was identified for both critical and noncritical CHDs.

Expanding use of the HeartMate 3 ventricular assist device in pediatric and adult patients within the Advanced Cardiac Therapies Improving Outcomes Network (ACTION).

BACKGROUND: We report current outcomes in patients supported with the HeartMate 3 (HM3) ventricular assist device in a multicenter learning network. METHODS: The Advanced Cardiac Therapies Improving Outcomes Network database was queried for HM3 implants between 12/2017 and 5/2022. Clinical characteristics, postimplant course, and adverse events were collected. Patients were stratified according to body surface area (BSA) (<1.4 m2, 1.4-1.8 m2, and >1.8 m2) at device implantation. RESULTS: During the study period, 170 patients were implanted with the HM3 at participating network centers, with median age 15.3years; 27.1% were female. Median BSA was 1.68 m2; the smallest patient was 0.73 m2 (17.7 kg). Most (71.8%) had a diagnosis of dilated cardiomyopathy. With a median support time of 102.5days, 61.2% underwent transplantation, 22.9% remained supported on device, 7.6% died, and 2.4% underwent device explantation for recovery; the remainder had transferred to another institution or transitioned to a different device type. The most common adverse events included major bleeding (20.8%) and driveline infection (12.9%); ischemic and hemorrhagic stroke were encountered in 6.5% and 1.2% of patients, respectively. Patients with BSA <1.4 m2 had a higher incidence of infection, renal dysfunction, and ischemic stroke. CONCLUSIONS: In this updated cohort of predominantly pediatric patients supported with the HM3 ventricular assist device, outcomes are excellent with <8% mortality on device. Device-related adverse events including stroke, infection, and renal dysfunction were more commonly seen in smaller patients, highlighting opportunities for improvements in care.

Interventricular Septal Hematoma Complicating Ventricular Assist Device Placement in an Infant: Case Report and Review of the Literature.

Interventricular septal hematoma is a rare and life-threatening complication of pediatric cardiac surgery. Commonly seen following ventricular septal defect repair, it has also been associated with ventricular assist device (VAD) placement. Although conservative management is usually successful, operative drainage of interventricular septal hematoma occurring in pediatric patients undergoing VAD implantation should be considered.

The Position and Orientation of the Pulse Generator Affects MRI RF Heating of Epicardial Leads in Children.

Infants and children with congenital heart defects often receive a cardiac implantable electronic device (CIED). Because transvenous access to the heart is difficult in patients with small veins, the majority of young children receive epicardial CIEDs. Unfortunately, however, once an epicardial CIED is placed, patients are no longer eligible to receive magnetic resonance imaging (MRI) exams due to the unknown risk of MRI-induced radiofrequency (RF) heating of the device. Although many studies have assessed the role of device configuration in RF heating of endocardial CIEDs in adults, such case for epicardial devices in pediatric patients is relatively unexplored. In this study, we evaluated the variation in RF heating of an epicardial lead due to changes in the lateral position and orientation of the implantable pulse generator (IPG). We found that changing the orientation and position of the IPG resulted in a five-fold variation in the RF heating at the lead's tip. Maximum heating was observed when the IPG was moved to a left lateral abdominal position of patient, and minimum heating was observed when the IPG was positioned directly under the heart. Clinical Relevance- This study examines the role of device configuration on MRI-induced RF heating of an epicardial CIED in a pediatric phantom. Results could help pediatric cardiac surgeons to modify device implantation to reduce future risks of MRI in patients.

Development of a Model for the Pediatric Survival After Veno-Arterial Extracorporeal Membrane Oxygenation Score: The Pedi-SAVE Score.

Pediatric cardiac extracorporeal membrane oxygenation (ECMO) patients have high mortality rates. The purpose of our study was to develop and validate the Pediatric Survival After Veno-arterial ECMO (Pedi-SAVE) score for predicting survival at hospital discharge after pediatric cardiac veno-arterial (VA) ECMO. We used data for pediatric cardiac VA-ECMO patients from the Extracorporeal Life Support Organization registry (1/1/2001-12/31/2015). Development and validation cohorts were created using 2:1 random sampling. Predictors of survival to develop pre- and postcannulation models were selected using multivariable logistic regression and random forest models. ß-coefficients were standardized to create the Pedi-SAVE score. Of 10,091 pediatric cardiac VA-ECMO patients, 4,996 (50%) survived to hospital discharge. Pre- and postcannulation Pedi-SAVE scores predicted that the lowest risk patients have a 65% and 74% chance of survival at hospital discharge, respectively, compared to 33% and 22% in the highest risk patients. In the validation cohort, pre- and postcannulation Pedi-SAVE scores had c-statistics of 0.64 and 0.71, respectively. Precannulation factors associated with survival included: nonsingle ventricle congenital heart disease, older age, white race, lower STAT mortality category, higher pH, not requiring acid-buffer administration, <2 cardiac procedures, and indication for VA-ECMO other than failure to wean from cardiopulmonary bypass. Postcannulation, additional factors associated with survival included: lower ECMO pump flows at 24 hours and lack of complications. The Pedi-SAVE score is a novel validated tool to predict survival at hospital discharge for pediatric cardiac VA-ECMO patients, and is an important advancement in risk adjustment and benchmarking for this population.

Influence of Fetal Diagnosis on Management of Vascular Rings.

BACKGROUND: Fetal diagnoses of vascular rings have been increasing. We compared management strategies and outcomes of infants with fetal diagnosis with those with postnatal diagnosis to inform recommendations regarding optimal management. METHODS: A retrospective review was performed of vascular ring operations from January 2000 to June 2019. Standard demographic data (preoperative clinical status, timing of diagnosis, cross-sectional imaging, operative and perioperative details, and clinical outcomes) were collected. Statistical analysis was performed to compare characteristics and outcomes of fetal versus postnatal diagnosis. RESULTS: Of 190 patients, 15% (n = 29) were diagnosed prenatally. Anatomic variants were double aortic arch (n = 66, 14 fetal diagnoses), right aortic arch, aberrant left subclavian artery (n = 94, 12 fetal diagnoses), circumflex aorta (n = 7, 1 fetal diagnosis), and pulmonary artery sling (n = 19, 2 fetal diagnoses). An increasing frequency of fetal diagnoses has been noted in the past 10 years. In 2012 1 of 9 patients (11%) had a fetal diagnosis, whereas in 2018 8 of 11 (72%) had a fetal diagnosis (P < .001). Patients with a fetal diagnosis were significantly younger at the time of surgery (13.1 months [interquartile range (IQR), 20.6] vs 24.0 months [IQR, 87.0], P = .029). There was no difference in postoperative complications or length of stay (3 days [IQR, 1] for fetal diagnoses vs 4 days [IQR, 3] for postnatal diagnoses, P = .50). CONCLUSIONS: Fetal diagnosis leads to the potential for expectant management of vascular ring patients. This has resulted in earlier time of intervention with no increase in postoperative morbidity. This may lead to improved long-term outcomes and potentially alter the natural history for these children.

Pediatric Heart Transplant Recipients Bridged with Biventricular Assist Device Have Worse 1 Year Graft Survival.

There are little data on postheart transplant (HT) outcomes for pediatric patients that were supported to HT with biventricular assist device (BiVAD). The United Network for Organ Sharing database was queried for patients <18 years old at time of HT between January 2005 and March 2018, excluding patients bridged with total artificial hearts and right ventricular assist device (VAD). Of 4,904 pediatric HT recipients, patients were grouped by no VAD support (3,934; 80.2%), left ventricular assist device only (736; 15%), and BiVAD (234; 4.8%). Overall graft survival analysis indicates crossing hazard rates between groups over time with the BiVAD group having a significantly lower graft survival at 1 year post-HT. A Cox model adjusted for age, era, diagnosis, and time by group interaction demonstrated increased 1 year hazard ratio (HR) of 8.5 (95% confidence intervals [CI]: 6.15-11.79) comparing BiVAD to no VAD. Comparable hazard between BiVAD and no VAD groups were found at 5 years (HR 1.01; 95% CI: 0.67-1.51), while lower hazard for the BiVAD group was found at 10 years post-HT (HR 0.07; 95% CI: 0.03-0.18). Although pre-HT BiVAD support leads to worse graft survival 1 year post-HT, long-term survival is acceptable.

Applicability and Durability of Valve-Sparing Tetralogy of Fallot Repair.

BACKGROUND: Although valve-sparing repair remains ideal for patients with tetralogy of Fallot, the durability of valve-sparing repair and which patients may have been better served with a transannular patch remain unclear. METHODS: Retrospective review was performed of tetralogy of Fallot operations at our institution from January 2008 to December 2018. Standard demographic data were collected, including echocardiographic parameters, operative details, and clinical outcomes. Statistical analysis was performed comparing the transannular patch and valve-sparing repair groups. RESULTS: Sixty-seven patients underwent tetralogy of Fallot repair with a median age of 4.5 (3.2-6.0) months and weight of 5.8 (5.2, 6.7) kg. Seventeen (25%) patients underwent transannular patch repair and 50 (75%) patients underwent valve-sparing repair. There was no difference in age or weight between patients who underwent a transannular patch repair and those who underwent a valve-sparing repair. At last follow-up (median 42 months), there was a trend of a higher peak pulmonary valve/right ventricular outflow tract gradient (P = .06) in the valve-sparing group, but no difference in the pulmonary valve annulus z-scores. Additionally, the pulmonary valve z-scores in the valve-sparing group decreased from -2.3 ± 1.0 on predischarge echocardiogram of to -1.2 ± 1.6 on last follow-up, with the peak gradient on predischarge 23 (0-37) mm Hg remaining stable on last follow-up at 18 (0-29) mm Hg. There was one reoperation: pulmonary valve replacement six years after a transannular patch. CONCLUSIONS: Obtaining a postrepair pulmonary valve z-score of -2 yields satisfactory, stable valve-sparing repair with pulmonary valve growth, acceptable gradients, minimal regurgitation, and high freedom from reintervention during follow-up.

Surveillance Cultures and Infection in 230 Pacemaker and Defibrillator Generator Changes in Pediatric and Adult Congenital Patients.

BACKGROUND: Postoperative infections can occur during surgical replacement of pulse generators for pacemakers and implantable cardioverter-defibrillators. The incidence of infection is poorly documented in children and patients with adult congenital heart disease. The utility of surveillance cultures obtained from device pocket swabs is unknown in this group. METHODS: We reviewed surgical replacements of cardiovascular implantable pulse generators from 2010 to 2017. Two cohorts were defined. In a surveillance cohort (123 patients), aerobic and anaerobic culture swabs of the device pocket were obtained at the time of generator change. In a nonsurveillance cohort (107 patients), generator change occurred without obtaining cultures. RESULTS: During 230 generator changes (mean patient age 19 years; 77% with structural congenital heart disease), two clinical infections occurred at the surgical site (0.9% incidence). Neither infection occurred in the surveillance cohort. Cultures were positive in 12 (9.8%) of 123 patients in the surveillance cohort, but 11 of 12 were likely contaminants and none were subsequently associated with clinical disease. There was no association between clinical infection or positive surveillance cultures and the location of pulse generator, the presence of other concurrent surgeries, or a history of prior pocket infection. CONCLUSIONS: Clinical infection was rare after pulse generator change in children and young adults. No cases required reintervention on the pocket. Surveillance cultures did not improve clinical care. These data extend current recommendations that surveillance cultures are not required during generator change to the pediatric and young adult population.

24-Year Results of Nonfenestrated Extracardiac Fontan Including Fontan Conversions.

BACKGROUND: There is active debate regarding the optimal method of Fontan palliation. In light of this, we reviewed our experience with the nonfenestrated extracardiac Fontan including Fontan conversion. METHODS: We performed a retrospective review of all nonfenestrated extracardiac Fontan and Fontan conversion operations at our institution from December 1, 1994 to December 31, 2018. Standard demographic data were collected, including underlying anatomy, preoperative ventricular and valvular function, operative details, perioperative data, and clinical outcomes. Statistical analysis included comparison between initial extracardiac Fontan patients and Fontan conversions, as well as analysis of risk factors for adverse outcomes. RESULTS: There were 341 patients with an overall operative mortality of 4 patients (1.2%). Of these, 193 were extracardiac nonfenestrated Fontan completion operations (57%) and 148 were Fontan conversions (43%). Length of stay was 11 days (SD, 6 days) with ventilator duration of 28 hours (SD, 26 hours). Six of the completion Fontan patients (3%) required Fontan takedown at a median time of 2.5 days. Upon multivariable analysis, risk factors associated with adverse events (mortality, Fontan takedown, and/or transplant) included increased cardiopulmonary bypass time, preoperative decreased dominant ventricular function, and length of stay. Kaplan-Meier curves demonstrated that mild or greater preoperative ventricular dysfunction decreased survival as well as freedom from adverse events for both initial extracardiac Fontan and Fontan conversion patients. CONCLUSIONS: Over the past 24 years, our strategy of nonfenestrated extracardiac Fontan has achieved low operative mortality for both initial Fontan and Fontan conversion. There is a steady attrition of Fontan patients to cardiac transplantation; the key risk factor is preoperative ventricular dysfunction.

Evolution and Current Results of a Unified Strategy for Sinus Venosus Surgery.

BACKGROUND: Given recent reports of percutaneous closure of sinus venosus atrial septal defects, we reviewed our experience with surgical repair. Owing to the high incidence of arrhythmias with the two-patch technique, since 2001 we have used either one-patch repairs or the Warden procedure. METHODS: A retrospective review was performed of pediatric patients undergoing sinus venosus atrial septal defect repair at our institution from January 1, 1990, to July 1, 2018. Standard demographic data such as echocardiographic and cross-sectional imaging along with operative details and clinical echocardiographic outcomes were collected. RESULTS: The cohort included 144 patients with a median age of 4.3 years (interquartile range, 8.5). Inferior SVASD was present in 24 patients (17%). A single autologous untreated pericardial patch was used for 114 patients (79%), a two-patch technique for 20 patients (14%, last performed in 2000), and a Warden procedure in 10 patients (7%). Median length of stay was 4 days (interquartile range, 2). On echocardiogram follow-up, no patient had pulmonary vein stenosis. One patient who had the Warden procedure required a balloon dilation of the superior caval vein 2 years postoperatively and a stent 3 years later. Two-patch patients were substantially less likely to be in normal sinus rhythm (41%) on postoperative electrocardiograms compared with the other two techniques (81% one-patch and 89% Warden, P = .02). CONCLUSIONS: The great majority of patients with sinus venosus atrial septal defects can be successfully repaired with a single patch of autologous pericardium. We transitioned to using either a single pericardial patch or the Warden procedure, resulting in a higher frequency of normal sinus rhythm on postoperative electrocardiograms.

Variability in clinical decision-making for ventricular assist device implantation in pediatrics.

BACKGROUND: Minimal data exist on clinical decision-making in VAD implantation in pediatrics. This study aims to identify areas of consensus/variability among pediatric VAD physicians in determining eligibility and factors that guide decision-making. METHODS: An 88-item survey with clinical vignettes was sent to 132 pediatric HT cardiologists and surgeons at 37 centers. Summary statistics are presented for the variables assessed. RESULTS: Total respondents were 65 (72% cardiologists, 28% surgeons) whose centers implanted 1-5 (34%), 6-10 (40%), or >10 (26%) VADs in the past year. Consensus varied by patients' age, diagnosis, and Pedimacs profile. Highest agreement to offer VAD (97%) was a mechanically ventilated teenager with dilated cardiomyopathy. Patients stable on inotropes were less likely offered VAD (11%-25%). SV infant with Pedimacs profile 2 had the most varied responses: 37% offered VAD; estimated survival ranged from 15% to 90%. Variables considered for VAD eligibility included mild developmental delays (100% offered VAD), moderate-severe behavioral concerns (46%), cancer in remission >2 years (100%), active malignancy with good prognosis (68%) or uncertain prognosis (36%), and BMI >35 (74%) or <15 (69%). Most respondents (91%) would consider destination therapy VADs in pediatrics, though not currently feasible at 1/3 of centers. Factors with greatest influence on decision-making included HT candidacy, families' goals of care, and risks of complications. CONCLUSIONS: Significant variation exists among pediatric VAD physicians when determining VAD eligibility and estimating survival, which can lead to differences in access to emerging technologies across institutions. Further work is needed to understand and mitigate these differences.

Early experience with the HeartMate 3 continuous-flow ventricular assist device in pediatric patients and patients with congenital heart disease: A multicenter registry analysis.

BACKGROUND: The HeartMate 3 ventricular assist device (VAD) is a newer centrifugal continuous-flow VAD used for bridge-to-transplant and destination therapy in adults. However, there is limited experience regarding its use in children and adults with complex congenital heart disease (CHD). METHODS: The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) is a multicenter learning network comprised of pediatric hospitals implanting VADs in children and adults with complex CHD. We examined the outcomes of patients undergoing HeartMate 3 implantation at an ACTION center between December 2017 and September 2019. RESULTS: The HeartMate 3 was implanted in 35 patients at 9 ACTION centers, with a median age of 15.7 (8.8-47.3) years, median weight of 65.7 (19.1-114.1) kg, and median body surface area (BSA) of 1.74 (0.78-2.36) m2. Of the cohort, 14 patients (40%) weighed <60 kg. Diagnoses included dilated cardiomyopathy (63%), dilated cardiomyopathy in neuromuscular disease (20%), and CHD (17%). Of those with CHD, most had a Fontan circulation. With a median 78 days of follow-up, there was 1 death on device (97% survival); 20 out of 35 (57%) underwent transplantation with no post-transplantation mortality. There were no episodes of stroke or pump thrombosis. CONCLUSIONS: Use of the HeartMate 3 in ACTION centers was associated with a low incidence of mortality and adverse events. Patients as small as 19 kg (BSA 0.78 m2) were successfully implanted and supported, indicating that this device may be appropriate for older children and small adults.

Surgical Management of Aorto-Ventricular Tunnel. A Multicenter Study.

Aorto-ventricular tunnel (AoVT), a rare congenital anomaly, is a channel originating in the ascending aorta just above the sinotubular junction and leading to the cavity of the left ventricle (AoLVT), or, rarely, the right (AoRVT). This study reviews our collective 30-year experience with the surgical treatment of AoVT. Data were submitted by 15 participating centers on 42 patients who underwent correction of AoVT between 1987 and 2018. Of these, 36 had AoLVT, and 6 AoRVT. The tunnel originated above the right coronary sinus in 28 (77.8%) patients. For AoLVT, most operations were performed early (median age 25 days, range: 1 day-25 years). In contrast, AoRVT was diagnosed and repaired later (median age 6 years, range: 1 month-12 years). Surgically important coronary ostial displacement was common. Patch closure of the aortic orifice only was the commonest surgical repair for AoLVT (23 patients), while in AoRVT, both orifices or only the ventricular one was closed. Aortic valvar insufficiency, severe or moderate, coexisted in 11 (30.5%) patients with AoLVT, and aortic valvuloplasty was performed in 8, mainly due to aortic valve stenosis. Aortic valvar insufficiency at discharge ranged from trivial to mild in almost all patients. Early mortality was 7.14%, with 3 patients with AoLVT succumbing to cardiac failure. There were 2 early reoperations and 1 late death. AoVT is a rare malformation. AoLVT usually necessitates surgery in early life. AoRVT is rarer, diagnosed and repaired later in life. Surgical repair by patch closure, with concomitant aortic valve repair as needed, is associated with good results.

Pediatric Tracheal Surgery: A 25-Year Review of Slide Tracheoplasty and Tracheal Resection.

BACKGROUND: The purpose of this study was to assess the outcomes of slide tracheoplasty and tracheal resection in pediatric patients and analyze the data for predictors of outcomes. METHODS: A retrospective review of tracheal surgery from January 1, 1993 to May 1, 2018 was performed. Demographic data, operative details, perioperative data, and clinical outcomes were collected. The study investigators' management strategy has evolved over time, with less rigid bronchoscopy, more reliance on postoperative computed tomographic imaging, and the use of inhaled Ciprodex (combination of ciprofloxacin and dexamethasone) since 2007. RESULTS: The study included 41 patients, with a median age of 4.1 months and a median weight of 4.2 kg. There were 6 neonates and 24 infants. Slide tracheoplasty was performed in 27 patients (66%), and resection with end-to-end anastomosis was performed in 14 (34%). Eleven patients (27%) had a pulmonary artery sling. Simultaneous intracardiac repairs requiring cross-clamp and cardioplegia were performed in 9 patients (22%). Lung agenesis (n = 6) or severe hypoplasia (n = 2) was present in 8 patients (20%). Complications included tracheostomy in 5 patients (12%) and in-hospital death in 3 patients (7%). There were no cases of mediastinitis. Inhaled Ciprodex was used to decrease granulation tissue at suture lines. Median intubation time was 7 days, and median length of stay was 25.0 days. There was no difference in outcomes when comparing intracardiac repairs with the remaining patients, lung agenesis or hypoplasia vs the remaining cohort, or neonates vs infants. CONCLUSIONS: Slide tracheoplasty and tracheal resection are effective operative strategies for infants and children with tracheal stenosis, including patients with lung agenesis/hypoplasia. Simultaneous repair of intracardiac anomalies and pulmonary artery sling is recommended.

Unusual Complication of the Nuss Procedure: Fistulization of the Internal Thoracic Artery to the Pulmonary Artery.

Although minimally invasive repair of pectus excavatum has been shown to have a low complication rate in large series, several case reports have documented life-threatening complications, including bleeding and cardiac perforation. We present a rare case of an arteriovenous malformation from the internal thoracic artery to the pulmonary artery caused by occlusion of the internal thoracic artery by the Nuss bar followed by an unidentified angiogenic process. The patient became symptomatic and required transcatheter coil embolization.