Vascular Ring Repair Research Articles

Right-Sided Horner's Syndrome as a Complication of Vascular Ring Repair

Right-Sided Horner's Syndrome as a Complication of Vascular Ring Repair

Upper partial sternal split for pediatric cardiac surgery

ObjectivesWe introduced the use of an upper partial sternal split for pediatric cardiac surgical procedures in our unit in 2016. We report the outcomes of our experience in 51 patients using this approach.MethodsFrom February 2016 to September 2022, 51 patients underwent congenital cardiac surgical procedures using an upper partial sternal split including vascular ring repair (n = 20), subaortic membrane (n = 12), ventricular septal defect closure with aortic valve resuspension (n = 9), aortic arch repair (n = 4), pulmonary artery band (n = 2), pulmonary artery sling (n = 1), supravalvular aortic stenosis (n = 1), aortic valve replacement (n = 1), and pulmonary artery plasty (n = 1). The surgical approach involved a midline skin incision, based on the manubrium, followed by an upper manubriotomy. No special surgical instrumentation was required. Median patient age was 2.9 years (IQR 1.3, 6.0); median body weight was 15 kg (IQR 9.8, 20).ResultsThere was no mortality and no patient required intraoperative conversion to full sternotomy. One patient required re-exploration for bleeding when the incision was converted to a full sternotomy. There were no wound complications in any patient. Twenty-one patients (41%) were extubated on the table and of the remaining 30 patients, 23 patients (76%) were extubated within 24 h of surgery. Eleven patients did not require intensive care unit (ICU) admission. Median ICU and hospital stay was 1 day (IQR 1, 1.25) and 5 days (IQR 4, 8) ,respectively.ConclusionAn upper partial sternal split approach is straightforward and can be performed safely with a preferable cosmetic result in selected pediatric cardiac operations.

Feeding Difficulties Following Vascular Ring Repair: A Contemporary Narrative Review.

Vascular rings are congenital abnormalities of the aortic arch vascular system that compress the trachea and esophagus. A review of long-term outcomes suggests that chronic feeding difficulties can persist following surgical repair of vascular rings. Previous reports of postoperative vascular ring division outcomes indicate that chronic esophageal symptoms may persist following repair, though most available data focuses on persistent respiratory symptoms. It is therefore the aim of this article to summarize and organize recent evidence reporting the frequency, presentation, and management of feeding difficulties following vascular ring repair in pediatric patients. Pathophysiologic mechanisms for postoperative esophageal symptoms may include residual compression from an unresected diverticulum of Kommerell or delayed repair leading to chronic esophageal dysmotility despite correction of esophageal compression. Guidance on the management of feeding difficulties following vascular ring repair is limited. The authors describe success in one case with nasogastric tube feeding and interdisciplinary evaluation. Consensus regarding the management of feeding difficulty following vascular ring repair is needed.

Lymphatic imaging and intervention for chylothorax following thoracic aortic surgery

Reports on lymphatic intervention for chylothorax complicating thoracic aortic surgery are limited. We aimed to evaluate technical and clinical outcomes of lymphangiography and thoracic duct embolization (TDE) for chylothorax complicating thoracic aortic surgery.Nine patients (mean age, 38.9 years) who underwent chylothorax interventions after thoracic aortic surgery (aorta replacement [n = 7] with [n = 2] or without [n = 5] lung resection, and vascular ring repair [n = 2]) were reviewed retrospectively. Magnetic resonance (MR) lymphangiograms were obtained in 5 patients. The median interval between surgery and conventional lymphangiography was 9 days (range, 4–28 days). TDE clinical success was defined as lymphatic leakage resolution with chest tube removal within 2 weeks.MR lymphangiograms revealed contrast leakage from the thoracic duct (n = 4) or no definite leakage (n = 1), which correlated well with conventional lymphangiogram findings. The technical success rate of conventional lymphangiography was 88.9% (8/9); 8 patients showed contrast leakage, while the patient without definite leakage on MR lymphangiography had small inguinal lymph nodes, and thoracic duct visualization by conventional lymphangiography failed. The technical success rates of antegrade and retrograde TDE via pleural access were 75% (6/8) and 100% (3/3), respectively. Clinical outcomes after embolization, as judged by the tube-removal day, were similar between low- (<500 mL/day) and high-output (≥500 mL/day) chylothorax patients. The drainage amount decreased significantly after lymphangiography/TDE, from 710.0 mL/day to 109.7 mL/day (p < .05). The clinical success rate of TDE was 87.8% (7/8).Conventional lymphangiography and TDE yielded high technical success rates and demonstrated encouraging clinical outcomes for chylothorax complicating thoracic aortic surgery.

Single injection Erector Spinae Plane block for vascular ring repair: Is a catheter required?

Abstract Erector Spinae Plane (ESP) blocks have been used successfully in adults to manage post-operative pain associated with thoracotomy. In pediatric patients, thoracotomy pain is often managed with multimodal analgesia and neuraxial or regional techniques. The use of a single injection ESP block in pediatric thoracotomy patients can provide sensory nerve blockade, reduce sympathetic discharge, and decrease perioperative opioid requirements. This case report describes two pediatric patients undergoing vascular ring repair via left thoracotomy where post-operative pain was managed with a single injection ESP block and multimodal analgesia.

The final reason paediatric Cardiac ICU patients require care prior to discharge to the floor: a single-centre survey.

To determine the Final ICU Need in the 24 hours prior to ICU discharge for children with cardiac disease by utilising a single-centre survey. A cross-sectional survey was utilised to determine Final ICU Need, which was categorised as "Cardiovascular", "Respiratory", "Feeding", "Sedation", "Systems Issue", or "Other" for each encounter. Survey responses were obtained from attending physicians who discharged children (≤18 years of age with ICU length of stay >24 hours) from the Cardiac ICU between April 2016 and July 2018. Survey response rate was 99% (n = 1073), with 667 encounters eligible for analysis. "Cardiovascular" (61%) and "Respiratory" (26%) were the most frequently chosen Final ICU Needs. From a multivariable mixed effects logistic regression model fitted to "Cardiovascular" and "Respiratory", operations with significantly reduced odds of having "Cardiovascular" Final ICU Need included Glenn palliation (p = 0.003), total anomalous pulmonary venous connection repair (p = 0.024), truncus arteriosus repair (p = 0.044), and vascular ring repair (p < 0.001). Short lengths of stay (<7.9 days) had significantly higher odds of "Cardiovascular" Final ICU Need (p < 0.001). "Cardiovascular" and "Respiratory" Final ICU Needs were also associated with provider and ICU discharge season. Final ICU Need is a novel metric to identify variations in Cardiac ICU utilisation and clinical trajectories. Final ICU Need was significantly influenced by benchmark operation, length of stay, provider, and season. Future applications of Final ICU Need include targeting quality and research initiatives, calibrating provider and family expectations, and identifying provider-level variability in care processes and mental models.

Incidence of Postoperative Chylothorax Following Congenital Heart Surgery in Pediatric Patients: A Single-Center Experience.

Although postoperative chylothorax following congenital heart surgery occurs rarely, it is associated with substantial morbidity and mortality. The incidence of postoperative chylothorax has been reported as 2% to 5%. Therefore, we aimed to evaluate the incidence of postoperative chylothorax at our center and compared our results with those of other studies. Between January 2009 and December 2018, there were 2,515 congenital heart repair surgeries performed at our center. Thirty-six patients with postoperative chylothorax were enrolled in this study, and their medical records retrospectively were reviewed. We calculated the overall and surgery-specific incidences. We compared the data of the medical management group with those of the surgical management group. The overall incidence of postoperative chylothorax was 1.4%. The incidence was highest for patients who underwent vascular ring repair (3/32). Moreover, the incidence was higher for single ventricle-related procedure than bi-ventricle-related procedures (5.6% versus 1.0%, P < .0001). Chylothorax was predominantly found on the left side (20/36). Among these cases, six patients died; three of these six were in the surgical management group. The postoperative chylothorax incidence at our center was comparable to those of other centers. However, a reasonable protocol for postoperative chylothorax management to improve outcomes is necessary.

Symptom persistence after vascular ring repair in children

PurposeVascular rings are often diagnosed after evaluation for swallowing and breathing difficulties. Data regarding symptoms following vascular ring repair is sparse. We sought to determine whether symptoms persist using chart review and a survey. MethodsSixty-three patients underwent open vascular ring repair from July 2007 to May 2018. Data regarding vascular anatomy, demographics, pre- and postoperative symptoms, and chromosomal abnormalities were collected. Freedom from reoperation, 30-day mortality, and complications were assessed. Patient families were contacted for a symptom focused survey. ResultsThe median age of surgical intervention was 14.4 months (IQR 5.8–34.7 months) for single aortic arches with an aberrant subclavian artery (SAA), and 5.3 months (IQR 1.3–10.1 months) for double aortic arches (DAA) (Table). Prior to surgery, all but two SAA were symptomatic. There was no operative mortality. Three patients required re-exploration for chylothorax, and three required late aortopexy. At last follow-up, 45% (18/40) SAA and 65% (15/23) DAA had post-operative symptoms. Fourteen patient families completed the symptom survey (10 SAA, 4 DAA). Five SAA had breathing and swallowing symptoms, and 3 SAA and 3 DAA had breathing difficulties. ConclusionsOpen vascular ring repair remains a safe repair. However, further investigation of the persistent symptoms in these patients is merited. Study Type / Level of EvidenceRetrospective Comparative Study, Level III

Contemporary Midterm Outcomes in Pediatric Patients Undergoing Vascular Ring Repair

This single-institution study assessed themidterm outcomes of patients undergoing complete vascular ring (CVR) repair and the need for reintervention. The study included all patients who underwent surgical repair of an isolated CVR from 1996 to 2018 at our institution. Patients who underwent concomitant intracardiac repair were excluded. Data analysis included demographics, type of anomaly, other congenital heart disease, clinical symptomatology, operative technique, perioperative outcomes, reoperation rates, and mortality. CVR repair through open thoracotomy was performed in 148 patients (80 boys [54%]), median age, 1.04 years (interquartile range, 0.4-5.2 years), and median weight, 12.8 kg (interquartile range, 7.5-26.5 kg). The cohort included 72 patients with double aortic arch (DAA), 69 with right aortic arch (RAA) with aberrant left subclavian artery and left ligamentum arteriosum (LLA), 5 with RAA with left descending aorta and LLA, and 2 with RAA with mirror-image branching and LLA. There was 1 outpatient perioperative death (0.7%) 15 days postoperatively. Perioperative complications occurred in 20 patients (14%): 18 (12%) with chylothorax (3 required reintervention), 1 pneumothorax, and 1 vocal cord paresis. Two of 36 patients (5.5%) without primary diverticulum resection required reoperation and subclavian reimplantation at 3 and 4 years, and 1 patient required aortic translocation 9 years later for persistent symptoms. Freedom from reoperation after CVR repair was 93% at 5 years and 86% at 10 years. A small proportion of patients who do not undergo diverticulum resection and aberrant left subclavian artery reimplantation at the time of CVR repair will require reintervention in the future.

Concomitant repair of vascular ring with Kommerell's diverticulum and ventricular septal defect

None.

Reoperation after vascular ring repair.

The majority of patients having surgical intervention for a vascular ring have resolution of their symptoms. However, 5% to 10% of these patients develop recurrent symptoms related either to airway or esophageal compression and may require reoperation. In our series of 300 patients with vascular rings, we performed a reoperation on 26 patients, not all of whom were originally operated on at our institution. The four primary indications for reoperation were Kommerell diverticulum (n = 18), circumflex aorta (n = 2), residual scarring (n = 2), and tracheobronchomalacia requiring aortopexy (n = 4). All patients undergoing reoperation have had preoperative evaluation with bronchoscopy and computed tomographic scanning (CT) with 3-dimensional reconstruction. Patients with dysphagia have had a barium esophagram and esophagoscopy. Patients with a Kommerell diverticulum have undergone resection of the diverticulum and transfer of the left subclavian artery to the left carotid artery. The aortic uncrossing procedure has been used in patients with a circumflex aorta. Aortopexy has been used to treat anterior compression of the trachea by the aorta. Results of these reinterventions have been successful in nearly all cases. Lessons learned from these reoperations can be applied to prevent the need for reoperation by properly selecting the correct initial operation. A dedicated team caring for these children consisting of medical imaging, otolaryngology, cardiovascular-thoracic surgery, and critical care is imperative.

Repair of Vascular Ring with Resection of Kommerell Diverticulum and Transposition of Aberrant Left Subclavian Artery

A 32-year-old female presented with dysphagia. Radiographic studies revealed external compression of esophagus by a vascular ring. The anatomy was a right-sided aortic arch with aberrant retroesophageal left subclavian artery, emanating from a large Kommerell diverticulum (KD). Traditional repair with ligamentum division and adhesiolysis leaves a large KD still adjacent to the esophagus with the potential for persistent or recurrent symptoms. The objective of this study was the modification of operative technique to minimize the potential for persistent or recurrent symptoms. The operative repair included resection of KD with transposition of the left subclavian artery into the left carotid artery, in addition to the division of the ligamentum arteriosum and mobilization of the esophagus. The patient's dysphagia resolved and postoperative barium studies showed no residual compression. There were no significant perioperative complications. Resection of KD is a potential adjunct to traditional repair of vascular rings and might offer better long-term palliation by minimizing residual vascular compression of the esophagus.

Diagnosis and treatment of vascular ring associated with congenital tracheal stenosis in infants

Objective To investigate the operation method for congenital tracheal stenosis(CTS) with vascular ring(VR), in order to minimize surgical risk. Methods The data of 53 VR patients diagnosed by CT imaging, fiber bronchoscope and color doppler echocardiography were analyzed retrospectively.The age ranged from 5.0 days to 2.5 yeas old, median age was 12.5 months, including pulmonary artery sling, double aortic arch, right arch combined with abnormal left subclavian artery and innominate artery oppression, et al, in which 12 patients(8 male, 4 female) associa-ted with CTS symptoms.All patients were treated by operations, 8 patients were dead, others were followed up for 3 to 5 years at 6-month interval. Results Fifty-three patients with VR were categorized into 2 treatment groups.There were 41 patients(25 male, 16 female) without CTS symptoms treated by VR repair(3 cases died), mortality was 7.3%.Of the 12 patients(8 male, 4 female) with VR+ CTS symptoms, 7 cases were treated by selective VR repair with 2 cases deaths, 5 cases underwent combined VR and CTS repairs with 3 cases died, for an overall mortality of 41.7%.Of the 41 patients with VR but no CTS, 7 cases had intracardiac anomalies with 2 cases died.In contrast, 3 of 12 patients with VR+ CTS and intracardiac anomalies with 2 cases died; 5 patients received cardiac and CTS repairs(3 cases died) and 7 cases in selective VR repairs(2 cases died). Conclusions Not all cases of TN associated with VR require combined correction.CTS and the combined cardiac abnormalities increased the risk of VR surgery in children.The individualized treatment program should be developed for such children. Key words: Vascular ring; Congenital tracheal stenosis; Individualized; Infant

DOUBLE AORTIC ARCH IN A 14-YEAR-OLD GIRL

INTRODUCTION: Double aortic arch (DAA) is the most common clinically recognized form of vascular ring, in which the trachea and esophagus are encircled by the aortic arch and its branches. The overall incidence is unknown. Half to two-thirds of patients undergoing vascular ring repair have a DAA; of these 42% have respiratory symptoms, one third have dysphagia and one third are asymptomatic. Surgical division of the arch (usually on the left) is associated with prompt relief of symptoms.

Monitoring cerebral oxygenation in a pediatric patient undergoing surgery for vascular ring

Regional cerebral oxygenation can be monitored using near-infrared spectroscopy (NIRS). Inadequacy of collateral cerebral circulation and regional cerebral ischemia during cardiac and vascular surgery may be detected by the use of NIRS monitoring. We report a 2-year-old child who underwent surgical repair of vascular ring and subclavian reimplantation, where use of NIRS helped in early detection and timely intervention to prevent prolonged cerebral ischemia.

Totally Endoscopic Robotic-Assisted Repair of Patent Ductus Arteriosus and Vascular Ring in Children

This study reports on our initial experience with robotically assisted patent ductus arteriosus (PDA) closure and vascular ring division in children. From April 2002 to May 2004, 15 patients underwent PDA closure (n = 9) and vascular ring repair (n = 6) by a totally endoscopic approach, utilizing the Da Vinci robotic system. The mean age of the patients was 8.3 +/- 4.7 years (range, 3 to 18) and the mean weight, 35.5 +/- 19.0 kg (range, 14.1 to 77.0 kg). Three thoracoscopic trocars were used to accommodate the endoscopic camera and two surgical instruments with an additional small incision for lung retraction. After dissection by the surgeon seated at the master console, PDA ligation with clips or division of the atretic arch and ductal ligament was performed. Total operative times were 170 +/- 46 minutes (PDA) and 167 +/- 48 minutes (vascular ring). One patient with vascular ring was converted to thoracotomy because of dense adhesions due to previous surgery. Precise and easy surgical maneuver was possible with the articulated surgical instruments and three-dimensional visualization in 14 patients. Intraoperative transesophageal echocardiography confirmed no persistent shunt in all PDA patients. No laryngeal nerve injury and hemorrhage were noted. All patients were extubated in the operating room. Median length of postoperative hospital stay was 1.5 days. Robotically assisted PDA closure and vascular ring division is a feasible and safe procedure. Future technologic improvement, including smaller instrument size and incorporation of tactile feedback, may permit application of this technique to even younger infants and intracardiac repairs.

Amplatzer Closure of Atrial Septal Defect and da Vinci Robot-Assisted Repair of Vascular Ring

Technology for minimally invasive approaches to congenital heart disease is a rapidly evolving field. This case report reviews a novel approach to combining two of the newer technologies available to treat a pediatric patient with an atrial septal defect (ASD) and a vascular ring. This report is the first to describe the use of the da Vinci surgical system to assist in a thoracoscopic procedure for a pediatric patient. The da Vinci assisted division of the vascular ring, joined with an Amplatzer closure of the ASD, demonstrates how maximum benefit can be obtained for patients by combining emerging technologies.

Video-Assisted Thoracic Surgical Procedures in Children

The general principles and current applications of pediatric video-assisted cardiothoracic surgery (PVACTS) are reviewed. The purpose of PVACTS is to improve surgical quality and precision in selected operations. In the 1990s PVACTS has expanded to include the management of a variety of pulmonary, mediastinal, and cardiac lesions. Currently, PVACTS is carried out using a video camera connected to a low-profile scope and a specialized set of surgical instruments. PVACTS is an accepted modality for the diagnosis (by biopsy) of pleuropulmonary and mediastinal disease, and the treatment of pediatric empyema, spontaneous pneumothorax, and mediastinal cysts. Diaphragmatic plication, repair of chylous leak, and ligation of collateral vessels have all been done using PVACTS. PVACTS patent ductus arteriosus (PDA) ligation and vascular ring repair are being successfully carried out in several institutions. The technique at The Children's Hospital of Philadelphia is described. Indications and techniques for PVACTS lobectomy and pneumonectomy are less well established. Suggested anecdotal methods are described. Cardioscopy carries the hope of improving intracardiac repair, and has been applied to several lesions. The future of PVACTS depends on the surgeon's willingness to master it, industry's willingness to customize instruments for pediatric use, and developments in the fields of virtual imaging and augmented reality. Copyright 1998 by W.B. Saunders Company

Tracheal diverticulum with recurrent apnea and segmental pulmonary atelectasis

A patient presented with recurrent episodes of apnea and segmental pulmonary atelectasis. These episodes persisted following repair of esophageal atresia and tracheoesophageal fistula and repair of vascular ring causing tracheal compression. A tracheal diverticulum was identified and resected. The patient's symptoms disappeared following surgery.