Recurrent Pediatric Respiratory Distress From a Challenging Vascular Anomaly: The Uncrossing Operation for Circumflex Aorta.

The authors report a case of circumflex aorta causing persistent respiratory distress in a 9-year-old boy who had previously undergone vascular ring division and multiple aortopexies. The uncrossing operation was performed, with symptomatic relief of both posterior and right-sided tracheal compression from the transverse aorta and right aortic arch, respectively.

Partial upper median sternotomy for anterior aortopexy for innominate artery compression syndrome: a case series.

Background: Innominate artery compression syndrome (IAS) is caused by an abnormally originating innominate artery compressing the trachea anteriorly. One option to relieve such compression is an anterior aortopexy (AA). We describe our technique of an AA via a partial upper median sternotomy. Case Description: Nine consecutive patients underwent AA for IAS via a partial upper median sternotomy from July 2017 to November 2020 at two US teaching hospitals. The median age was 9 months [interquartile range (IQR), 3-16.5 months]. The male to female ratio was 1.25. All patients had >70% compression by flexible bronchoscopy. Two patients had previous surgeries. The median follow-up was 6 months (IQR, 4-8.5 months). The indications for the operation were: acute life-threatening events (ALTEs) (4/9 patients), recurrent intubation (4/9), and severe stridor with >70% luminal reduction (1/9). Technical success (defined as ≤20% residual stenosis) was achieved in 78% (7/9) of the patients. The two patients with unsuccessful AAs required either a tracheal resection or an innominate artery reimplantation. Both achieved full symptom resolution. Overall, 78% (7/9) of patients experienced full symptom resolution. Of the two patients without full symptom resolution, one had mild stridor at 6 months post-operation. The other patient without full resolution is awaiting further vocal cord surgery for an associated glottic pathology. Conclusions: A partial upper sternotomy provides a very versatile approach to an AA for IAS. In addition to facilitating an adequate AA, a partial upper sternotomy provides options for direct tracheal surgery or an innominate artery reimplantation in case an optimal result is not obtained by an AA.

Surgical Repair of a Left Main Coronary Artery to Right Ventricle Fistula in a Neonate.

We describe the case of a newborn male with a large fistula from the left main coronary artery to the right ventricle. This case illustrates a rare congenital coronary artery fistula and its successful surgical management in the neonatal period.

Early Outcomes of Pulmonary Valve Replacement With the Edwards Inspiris Resilia Pericardial Bioprosthesis.

BACKGROUND: Controversy regarding the optimal pulmonary valve substitute remains, with no approved surgical valve for pulmonary valve replacement (PVR). Furthermore, unfavorable anatomy often precludes transcatheter PVR in patients with congenital heart disease. We therefore sought to evaluate the feasibility of the Edwards Inspiris pericardial aortic bioprosthesis in the pulmonary position in pediatric and adult patients requiring PVR. METHODS: Data from consecutive patients who underwent PVR from February 2019 to February 2021 at our institution were retrospectively reviewed. Postoperative adverse events included paravalvular or transvalvular leak, endocarditis, explant, thromboembolism, valve thrombosis, valve-related bleeding, hemolysis, and structural valve degeneration. Progression of valve gradients was assessed from discharge to 30 days and one year. RESULTS: Of 24 patients with median age of 26 years (interquartile range [IQR]: 17-33; range: 4-60 years), 22 (91.7%) patients had previously undergone tetralogy of Fallot repair and 2 (8.3%) patients had undergone double-outlet right ventricle repair in the neonatal period or infancy. All patients had at least mild right ventricular (RV) dilatation (median RV end-diastolic volume index 161.4, IQR: 152.3-183.5 mL/m2) and at least moderate pulmonary insufficiency (95.8%) or stenosis (8.3%). Median cardiopulmonary bypass and cross-clamp times were 71 (IQR: 63-101) min and 66 (IQR: 60-114) min, respectively. At a median postoperative follow-up of 2.5 years (IQR: 1.4-2.6; range: 1.0-3.0 years), there were no mortalities, valve-related reoperations, or adverse events. Postoperative valve gradients and the severity of pulmonary regurgitation did not change significantly over time. CONCLUSIONS: At short-term follow-up, the bioprosthesis in this study demonstrated excellent safety and effectiveness for PVR. Further studies with longer follow-up are warranted.

Bivalirudin anticoagulation for cardiopulmonary bypass during cardiac surgery.

INTRODUCTION: Heparin is the primary anticoagulant for cardiopulmonary bypass (CPB) support during cardiac surgery. While widely used, ∼2% of cardiac surgery patients develop heparin-induced thrombocytopenia (HIT) and 4-26% develop heparin resistance. Bivalirudin is an alternative anticoagulant mainly used for percutaneous coronary interventions. Given the challenges associated with heparin anticoagulation, we conducted a review to explore the use of bivalirudin for CPB surgery. METHODS: PubMed and Embase scoping review included 2 randomized controlled trials, a retrospective comparison study, 3 pilot studies, and 30 case reports. To provide a contemporary series, we searched for articles published from 2010 to 2023. Our review included studies from both adult and pediatric populations. RESULTS: While data is limited, bivalirudin seems to supply similar effectiveness and safety as heparin for CPB anticoagulation. Across the three comparative studies, the heparin cohorts had a 0-9% mortality rate and 0-27% rate of major bleeding/reoperation compared to a 0-3% mortality and 0-6% major bleeding/reoperation rate for the bivalirudin cohorts. Bivalirudin was successfully used as an anticoagulant in a wide range of CPB surgeries (e.g., heart transplants, ventricular assisted device placements, and valve repairs). Successful patient outcomes were reported with bivalirudin infusion of ∼2 mg/kg/hour, activated clotting time monitoring (target >400 s or 2.5× baseline), use of cardiotomy suctions, minimization of stagnant blood, and post-bypass modified ultrafiltration. CONCLUSION: Bivalirudin is a safe and effective anticoagulant for CPB, especially for patients with HIT or heparin resistance. Further comparative research is called for to optimize bivalirudin utilization for CPB during cardiac surgery.

Cardiac transplantation in adult congenital heart disease: a narrative review.

Background and Objective: As more children with congenital heart disease survive to adulthood, adult congenital heart disease (ACHD) prevalence will increase (currently ~1 million US patients). Heart failure (HF) accounts for 26-42% of ACHD deaths. The rate of ACHD heart transplantations (ACHD HTx) is also increasing. We describe the ACHD HTx recipient/candidate cohort, analyze ACHD HTx outcomes, identify ACHD HTx specific challenges, and discuss opportunities to better serve more patients with ACHD HF. Methods: PubMed literature search including articles published from 2010-2023. Reviewed 89 studies, 67 included. Our search focused on the challenges of ACHD HTx and potential solutions. Key Content and Findings: ACHD HTx recipients are young [median age 35 years, interquartile range (IQR): 24-46 years]. 87-95% of ACHD HTx recipients had prior cardiac surgery. The most common underlying diagnoses include transposition of the great arteries (31%) and Fontan/Glenn circulation (28%). 63% of listed ACHD HTx candidates received a transplant within one year of listing. Post-transplant 1-year survival is 80%, 5-year survival 74%, and 10-year survival 59%. There are 4 unique ACHD HTx challenges: (I) difficulty in assessing pulmonary hypertension, resulting in some centers selecting oversized donor hearts. However, selecting oversized hearts does not improve post-operative mortality and could prolong waitlist time. (II) Increased immunologic sensitization, increasing rejection risk. Desensitization therapy has enabled sensitized HTx recipients to enjoy outcomes similar to non-sensitized recipients. (III) Procedural complexity with ~30% of cases requiring additional surgical reconstruction. Detailed multidisciplinary planning, extensive imaging, and transferring the patient into the operating room early can help manage the complexities and reduce organ ischemic time. (IV) Increased intraoperative bleeding due to patients' surgical histories and circulatory collaterals. Preoperative collateral coil embolization and select utilization of hypothermic circulatory arrest can help reduce bleeding. Additional Fontan specific challenges include extensive great artery repair, liver failure, plastic bronchitis, and protein loss enteropathy. Finally, given limited donor heart availability, mechanical circulatory support is a promising technology for patients with ACHD HF. Conclusions: The prevalence of ACHD HTx is slowly but steadily increasing. The operational complexity of ACHD HTx can be managed, and the majority of recipients have excellent outcomes (59% 10-year survival).

Complex Repair of Anomalous Left Coronary Artery From the Pulmonary Artery in a 55-Year-Old Patient.

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a congenital malformation that classically presents within the first year of life. Few patients survive into adulthood, and initial presentation after the fourth decade of life is rare. We describe a 55-year-old woman who presented after cardiac arrest. She initially refused surgery and underwent automated implantable cardioverter defibrillator placement, followed later by surgical repair involving reimplantation of the left coronary artery to the aorta and pulmonary artery reconstruction using interposition grafts. We report this late presentation of ALCAPA and successful surgical management.

Norwood Aneurysm: Report of a Rare Phenomenon.

An 18-year-old male with complex single-ventricle physiology status post-3-stage palliation developed a large Norwood aneurysm (77 × 67 mm). The patient underwent a successful surgical reconstruction. Care providers must be aware of this rare complication and provide appropriate surveillance.

The procedure and effectiveness of release maneuvers in tracheobronchial resection and reconstruction.

Background: Surgical resection and reconstruction are effective and radical treatments for tracheal tumors. Tension-free, well-perfused anastomosis plays a crucial role in postoperative prognosis. The use of various release maneuvers may be required to minimize anastomotic tension. However, the detailed procedures and effectiveness of them are seldomly reported. In the current study, we demonstrated the procedures and advantages of various release maneuvers during tracheal resection and reconstruction. Methods: All patients who underwent tracheobronchial resection and reconstruction between January 2019 to December 2021 were included in the study. The patients underwent tracheal release maneuvers, including laryngeal suprahyoid, pericardial, hilar, and inferior pulmonary ligament releasing. The patients' clinical features, surgical procedures, complications and postoperative outcomes were also described. Results: A total of 67 patients received release maneuvers during tracheobronchial surgery. Males accounted for a greater proportion (46/67, 65.7%) of the cohort. The mean age was 44.4 years. Most lesions were located in the thoracic and cervical trachea (21/67 and 17/67, respectively), and 18 cases of carinal (9/67) and bronchial (9/67) lesions were also included. Inferior pulmonary ligament releasing was applied to most noncervical lesion patients (39/67). Two cases of thyroid carcinoma with tracheal invasion received laryngeal suprahyoid release maneuvers. Adenoid cystic carcinoma (26.9%) and squamous cell carcinoma (14.9%) were the most commonly seen malignancies. Postoperative bronchoscopy showed no anastomotic abnormalities, including ischemic change, necrosis, or dehiscence. The median postoperative hospital stay was 7 days, ranging from 4 to 38 days. In the current study, a patient with postoperative aspiration had the longest hospital stay. In addition, 3 cases of anastomotic stenosis and laryngeal edema were observed. No other maneuver-related complications or particular discomforts were reported during the 6-month follow-up. Conclusions: Anastomosis is the key to successful tracheobronchial resection and reconstruction. Release maneuvers are recommended to facilitate tension-free anastomosis. In addition to simple neck flexion and paratracheal dissection, laryngeal, hilar, and pericardial releasing allow longer trachea to be resected and preservation of well-vascularized anastomosis. The release maneuvers showed acceptable effect and reliable safety without significant morbidity or mortality.

Long-term Results Using Glutaraldehyde-treated Homograft Pericardium in Congenital Heart Surgery.

BACKGROUND: This study reports the long-term outcomes using glutaraldehyde-treated cryopreserved homograft pericardium (CPH) in neonates, infants, children, and young adults undergoing congenital cardiac surgery. METHODS: A retrospective review was performed of all patients at a single institution (Rady Children's Hospital, San Diego, CA) who had undergone surgical implantation with CPH between 2006 and 2016. The study identified 134 consecutive patients who underwent implantation of a total of 276 patches. The baseline demographic characteristics, primary cardiac diagnosis, surgical characteristics, operative reports, and postoperative catheterization and reoperation reports were analyzed. The use of CPH was categorized by specific anatomic insertion site. RESULTS: The median age at patch implantation was 1.47 years (range, 1 day to 31.6 years). The numbers and locations of patch use were 124 for pulmonary arterial repair, 57 for repair of the aorta, 49 for septal repair, and 43 at other sites. At a median follow-up of 5.29 years, 9 patients had died (6.7%), but none of those deaths were related to CPH. Twelve patients (8.96%) underwent reoperations, and 18 patients (13.4%) underwent catheter interventions at sites of CPH implantation. The 10-year freedom from patch-induced reoperation and catheter intervention rates were 88.5% and 86.9%, respectively. Overall patch failure-free survival was 85.8% and 79.0% at 5 and 10 years, respectively. CONCLUSIONS: The use of CPH patch in the surgical correction of congenital heart disease is effective and durable, as evidenced by the low reintervention rates. These results are comparable to the early and midterm outcomes of other similarly used surgical patches.

Pseudoaneurysm of Innominate Artery Graft Stump in an Infant.

Innominate artery grafts are often utilized in pediatric cardiac surgery and very rarely lead to complications, including infection. Here, we present a unique case of an infant who underwent repair of coarctation of the aorta and hypoplastic arch using a GORE-TEX graft (W. L. Gore and Associates, Newark, DE) for antegrade cerebral perfusion. The graft subsequently became infected with Pseudomonas and formed a pseudoaneurysm with resultant tracheal compression. The presentation, diagnosis, and management of this mycotic pseudoaneurysm are described.

Pneumonectomy for Idiopathic Fibrosing Mediastinitis Mimicking Neoplasm in a Child.

The unique case of a child with idiopathic fibrosing mediastinitis mimicking neoplasm is presented. A 5-year-old boy presented with pneumonia and was found to have a complex, heterogeneous, and calcified mediastinal mass along the left hilum. Percutaneous and surgical biopsies, while suggesting a potential epithelial malignancy, were nonconclusive. Owing to worsening symptoms of airway obstruction and chest wall invasion, resection was performed for therapeutic and diagnostic purposes. This ultimately required pneumonectomy on cardiopulmonary bypass. Pathology revealed fibrosing mediastinitis with infiltration of lung parenchyma, and subsequent workup for infectious, neoplastic, granulomatous, and autoimmune etiologies was negative.

Staged repair of truncus arteriosus with double aortic arch, tracheoesophageal fistula, and choanal atresia via a multi-disciplinary approach: a case report.

The previously unreported case of a child with an exceedingly rare amalgamation of complex defects, including truncus arteriosus (TA), double aortic arch (DAA), tracheoesophageal fistula, and choanal atresia is presented. First, on day-of-life (DOL) 2, with a joint effort involving Pediatric Cardiac Surgery, General Surgery, and Otolaryngology, division of tracheoesophageal fistula and repair of esophageal atresia, along with choanal atresia repair, was carried out. Via a right thoracotomy, the tracheoesophageal fistula, located medial to the azygous vein, was skeletonized and ligated. The proximal esophagus was then mobilized up to the thoracic inlet as it coursed through the vascular ring. This enabled esophageal anastomosis with preservation of both aortas. Next, on DOL 11, the child underwent TA repair. Following a standard midline sternotomy and cooling to moderate hypothermia, the left aortic arch was divided and oversewn. The aorta was then transected anteriorly, and the main pulmonary artery (MPA) exiting the posterior aorta was harvested as a single button. The aortic defect from the pulmonary artery button was repaired with autologous pericardium. Next, through a right ventriculotomy, the previously seen conoventricular septal defect was identified and closed. Finally, a 10-mm pulmonary homograft was anastomosed to the pulmonary artery bifurcation to complete the repair. The patient was discharged on DOL 78 and was noted to be doing well at 1-year follow-up. This case validates the feasibility of fistula repair complicated by DAA through a right thoracotomy, the durability of staged, complete repair of TA and DAA, and the advantages of a holistic, team-based approach that optimizes timing of all repairs based upon a careful consideration of the exponential, rather than additive, effects of multi-organ disease on post-cardiac surgery outcomes in neonates.

Successful Management of a Patient With Jacobsen Syndrome and Hypoplastic Left Heart Syndrome.

Jacobsen syndrome (JS) is a rare genetic condition characterized by intellectual disability, hematologic abnormalities, and congenital heart defects. A male infant presented at birth with phenotypic findings of JS and echocardiographic findings of hypoplastic left heart syndrome (HLHS). Array comparative genomic hybridization was performed at age three days and revealed an 8.1 Mb terminal deletion on the long arm of chromosome 11, consistent with JS. At five days of age, a hybrid stage 1 procedure was performed. At age 46 days, he underwent a Norwood operation followed by bidirectional Glenn at age six months. He is presently 23 months old and doing well. With careful consideration of the individual patient and comorbidities associated with JS, we propose that at least a subset of patients with JS and HLHS can do well with staged surgical palliation.

Congenital tracheal stenosis & associated cardiac anomalies: operative management & techniques.

Congenital tracheal stenosis can lead to symptomatic airway obstruction in children and often mandates surgical correction. Over the past half-century, numerous tracheal reconstruction techniques have been developed, including tracheal resection with end-to-end anastomosis (for short-segment complete tracheal stenosis), patch tracheoplasty, slide tracheoplasty, and homograft and autograft augmentation repairs. However, operative management of congenital tracheal stenosis is often complicated by the presence of congenital heart disease, the most common of which is pulmonary artery sling. When present concomitantly, combined repair of both defects is feasible and is currently the preferred approach. Questions have been raised about the optimal timing and sequence of surgery, and some have advocated staged repair for patients with complex associated cardiac lesions. However, evidence from the past two decades suggests that concomitant repair can be performed with excellent results. The current standard of care involves the use of cardiopulmonary bypass to simultaneously repair the tracheal defect using slide tracheoplasty and all associated cardiac anomalies. Advances in operative techniques and extracorporeal circulation, progressive understanding of the pathological basis of combined congenital tracheal and cardiac disease, and a multidisciplinary approach to patient care have all contributed to the successful outcomes seen in the modern era. This article describes the combined surgical correction of tracheal stenosis and double-outlet right ventricle-tetralogy of Fallot type in an infant, provides a detailed step-by-step description for performing a slide tracheoplasty along with various other less favored tracheoplasty techniques, and reviews the current literature discussing such combined repairs.

Carinal Resection and Reconstruction for an Obstructing Inflammatory Myofibroblastic Tumor in a Child.

Inflammatory myofibroblastic tumor (IMT) is a rare soft tissue tumor characterized by proliferation of fibroblastic cells associated with an inflammatory infiltrate. Inflammatory myofibroblastic tumors have a predilection for the pediatric population and are usually found in the lung parenchyma but rarely at the carina. They rarely metastasize but can be locally destructive. Surgical resection is the cornerstone of therapy, which results in excellent survival despite risk of local recurrence. We present the case of a nine-year-old girl with an IMT mass at the carina and obstructing the left main stem bronchus, requiring extensive resection and reconstruction.

Arterial Switch Operation in a Patient With Ehlers-Danlos Syndrome Type IV.

Ehlers-Danlos syndrome vascular type IV is characterized by translucent skin, easy bruising, and fragility of arteries. A full-term female infant presented at four weeks of age with a diagnosis of d-transposition of the great arteries with restrictive atrial septal defect. She successfully underwent emergent balloon atrial septostomy and placement of patent ductus arteriosus (PDA) stent. She required restenting of the PDA and pulmonary artery banding prior to arterial switch procedure. At 16 months of age, the patient successfully underwent arterial switch procedure without complication. This report demonstrates the feasibility of an arterial switch operation along with long-term follow-up of this rare condition.

Aortopulmonary Window With Pulmonary Atresia and Interrupted Aortic Arch: A Very Rare Triad.

Aortopulmonary window (APW) is a rare lesion, accounting for 0.2% to 0.6% of all congenital heart diseases. We report a rare case of an infant with APW, interrupted aortic arch, and pulmonary atresia with intact interventricular septum and right ventricle-dependent coronary circulation. This report describes the anatomy of this lesion set, the complex surgical palliation that was required, and the management of postoperative complications.

Aspergillus aortitis & aortic valve endocarditis after coronary surgery.

The authors report a case of Aspergillus aortitis and aortic valve endocarditis that developed after coronary artery bypass surgery resulting in recurrent, embolic, bilateral lower extremity ischemia. This necessitated multiple lower extremity embolectomies followed by redo-sternotomy, extensive annular debridement, root reconstruction with a modified Bentall technique, and hemiarch replacement. This case highlights the challenges in diagnosis and management of this rare disease entity and some of its devastating complications.

Fontan Revision: Presurgical Planning Using Four-Dimensional (4D) Flow and Three-Dimensional (3D) Printing.

Pulmonary arteriovenous malformations (AVMs) can be a complication of certain postoperative Fontan patients whose hepatic venous blood return is not distributed evenly to both lungs. A ten-year-old female, who had previously undergone staged single ventricle palliation for complex congenital heart disease, underwent a Fontan revision due to significant left-sided pulmonary AVMs and increasing arterial oxygen desaturation. The combination of four-dimensional flow cardiac magnetic resonance imaging and three-dimensional printing enabled presurgical planning for a Fontan takedown and diversion of hepatic venous flow to the azygous vein that resulted in significant clinical improvement.