Hypoplastic Circumflex Aorta With Anomalous Left Pulmonary Artery: Uncrossing Operation in a Neonate.

Circumflex aortic arch with coarctation and anomalous origin of the left pulmonary artery from the aorta are rare cardiovascular anomalies. These conditions can lead to early pulmonary hypertension and challenging management. Early diagnosis and surgical intervention are beneficial for optimal outcome. We present a case where both anomalies coexisted and were repaired with aortic uncrossing, arch augmentation, and reimplantation of the left pulmonary artery. To our knowledge, this is the first documented instance of these anomalies coexisting and being repaired in the neonatal period.

Association and Repair of Right Aortic Arch With Aberrant Left Subclavian Artery With Subclavian Stenosis.

Surgical repair of right aortic arch and aberrant left subclavian artery has traditionally involved ligamentum division. Such patients can have stenosis at the origin of the aberrant subclavian artery either at the time of presentation or later. The more recently popularized repair involving resection of Kommerell diverticulum with transfer of the subclavian artery to the left carotid artery allows resection of the stenotic segment and serves as an effective treatment for subclavian stenosis as well. We present three cases of early repair of this arch anomaly with associated subclavian stenosis repaired successfully in that manner.

Bacterial epiglottitis superimposed on oropharyngeal cancer: A case report.

BACKGROUND: Patients undergoing chemotherapy and radiotherapy are placed in an immunocompromised state worth consideration in the event of potential airway compromise, especially when superimposed on an airway-obstructing tumor. We report a case of bacterial epiglottitis in a patient with active oropharyngeal cancer (OPC), who presented in such a way that an infectious etiology was not initially considered in the patient's care. To our knowledge, such a circumstance has not been reported in the literature. CASE: Here, we report a case of a 68-year-old male with advanced-stage OPC who developed respiratory distress and underwent emergent tracheostomy. The patient was diagnosed postoperatively with Haemophilus influenza and Pseudomonas aerugeniosa. Following antibiotic treatment, the patient recovered to the point in which he could then undergo concomitant chemoradiation. The patient later had a recurrence of P. aerugeniosa during their radiotherapy that was also treated with antibiotics. The patient experienced continued symptoms related to their OPC and underwent pharyngectomy. Despite the initial success of this procedure, the patient experienced tumor recurrence and succumbed to his disease. CONCLUSION: This case underscores the importance of considering multiple etiologies concerning airway compromise, as the consequence of delayed cancer treatment may be loss of local cancer control.

Radiation-induced myofibroblastoma within the nasal sinus requiring maxillectomy and ethmoidectomy: A case report and literature review.

We report a case of radiation-induced myofibroblastoma of the right nasal cavity in a patient with a remote history of radiotherapy for pediatric retinoblastoma. The patient required maxillectomy and ethmoidectomy. To our knowledge, a rare number of cases have been reported in this location.

The impact of curated educational videos on pathology health literacy for patients with a pancreatic, colorectal, or prostate cancer diagnosis.

Despite patients having increased access to their own electronic health record (EHR) in recent times, patients are often still not considered a primary audience of pathology reports. An alternative to in-person patient education is the use of multimedia programming to enhance health literacy. Curated video presentations designed to explain diagnosis-specific pathology terms were reviewed by a board-certified pathologist and oncologist team and then shown to patients with a primary diagnosis of either pancreatic, colorectal, or prostate cancer in-clinic; these patients then completed a secure electronic survey immediately afterwards. Seventy patients were surveyed, with 91% agreeing or strongly agreeing that the video they watched increased their understanding of the medical terms used in their pathology reports, with a corresponding average Likert score (ALS) of 4.21 (SD = 0.77, CI = ± 0.18). Furthermore, 95% agreed or strongly agreed that the video they watched both enhanced their understanding of the role of the pathologist in diagnosing cancer (ALS = 4.27; SD = 0.65, CI = ± 0.15) and reported they found the video useful (ALS = 4.27; SD = 0.53, CI = ± 0.13). Curated videos such as those utilized in this study have the potential to increase patient health literacy and inform patients of the multidisciplinary nature of cancer diagnosis.

Blood pressure variability during pediatric cardiac surgery is associated with acute kidney injury.

BACKGROUND: Blood pressure variability (BPV), defined as the degree of variation between discrete blood pressure readings, is associated with poor outcomes in acute care settings. Acute kidney injury (AKI) is a common and serious postoperative complication of cardiac surgery with cardiopulmonary bypass (CPB) in children. No studies have yet assessed the association between intraoperative BPV during cardiac surgery with CPB and the development of AKI in children. METHODS: A retrospective chart review of children undergoing cardiac surgery with CPB was performed. Intraoperative BPV was calculated using average real variability (ARV) and standard deviation (SD). Multiple regression models were used to examine the association between BPV and outcomes of AKI, hospital and intensive care unit (PICU) length of stay, and length of mechanical ventilation. RESULTS: Among 231 patients (58% males, median age 8.6 months) reviewed, 51.5% developed AKI (47.9% Stage I, 41.2% Stage II, 10.9% Stage III). In adjusted models, systolic and diastolic ARV were associated with development of any stage AKI (OR 1.40, 95% CI 1.08-1.8 and OR 1.4, 95% CI 1.05-1.8, respectively). Greater diastolic SD was associated with longer PICU length of stay (ß 0.94, 95% CI 0.62-1.2). When stratified by age, greater systolic ARV and SD were associated with AKI in infants ≤ 12 months, but there was no relationship in children > 12 months. CONCLUSIONS: Greater BPV during cardiac surgery with CPB was associated with development of postoperative AKI in infants, suggesting that BPV is a potentially modifiable risk factor for AKI in this high-risk population.

Myocardial ischaemia and valve insufficiency caused by a dysplastic aortic valve cusp: a previously unreported unique morphologic anomaly.

Isolated aortic regurgitation and myocardial infarction are a rare congenital defect among neonatal patients. We present a case of a neonate with an unusual aortic valve morphology causing both regurgitation and obstruction of the left coronary artery ostium. Despite both non-invasive and invasive imaging modalities, accurate diagnosis of the valve morphology was only determined by direct visualisation at the time of surgical repair. To the knowledge of authors, this particular aortic valve morphology in neonatal population has not been previously reported in the literature.

Pulmonary artery banding in complete atrioventricular septal defect.

OBJECTIVES: To analyze outcomes after pulmonary artery banding (PAB) in complete atrioventricular septal defect (AVSD), with a focus on surgical pathway outcome and timing, survival, and atrioventricular valve function. METHODS: PAB was performed in 50 of 474 infants (11%) from 28 institutions between 2012 and 2018 at a median age of 1.1 months. The median duration of follow-up was 2.1 years. Atrioventricular valve function was assessed by review of pre-PAB and predischarge echocardiograms (median, 9 days postoperatively). Competing-risks methodology was used to analyze the risks for biventricular repair, univentricular repair, and death. RESULTS: At 2 years, the proportions of patients who underwent biventricular repair, univentricular repair, and death were 68%, 13%, and 12%, respectively, with 8% awaiting definitive repair. After PAB, atrioventricular valve regurgitation decreased in 14 infants and increased in 10, but the distribution of regurgitation severity did not change significantly in the total cohort or subgroups. The intended management plan at PAB was deferred biventricular/univentricular decision (23 infants), 2-stage biventricular repair (24 infants), and univentricular repair (3 infants). Among the 24 infants intended for biventricular repair, 23 achieved biventricular repair and 1 died before repair. Survival at 4 years after biventricular repair among patients with previous PAB (93%) was similar to the 4-year survival of the patients who underwent primary biventricular repair (91%; n = 333). CONCLUSIONS: PAB is a successful strategy in complete AVSD to bridge to biventricular repair and has similar post-biventricular repair survival to primary biventricular repair. Changes in atrioventricular valve regurgitation after PAB were variable.

Correction to: Non-surgical Risk Factors for the Development of Chylothorax in Children after Cardiac Surgery-Does Fluid Matter?

The article Non­surgical Risk Factors for the Development of Chylothorax in Children after Cardiac Surgery­Does Fluid Matter?, written by Tanya Perry, Kelly Bora, Adnan Bakar, David B. Meyer and Todd Sweberg, was originally published electronically on the publisher's internet portal (currently SpringerLink) on November 2019 with open access. With the author(s)' decision to step back from Open Choice, the copyright of the article changed on November 2019 to © Springer Science+Business Media, LLC, part of Springer Nature 2019 and the article is forthwith distributed under the terms of copyright.

A Case of Persistent Left Superior Vena Cava and Left Pulmonary Venous Drainage to the Coronary Sinus.

We report a case of a persistent left superior vena cava draining to the right atrium via the coronary sinus in conjunction with partial anomalous pulmonary venous return of the left pulmonary veins to the coronary sinus. Although a persistent left superior vena cava is typically of little clinical consequence, in this case, it complicated surgical repair of the congenital heart disease. Successful repair of this unusual combination of systemic and pulmonary venous anomalies required a combination of two well-described surgical techniques.

Non-surgical Risk Factors for the Development of Chylothorax in Children after Cardiac Surgery-Does Fluid Matter?

We hypothesize that there are post-operative, non-surgical risk factors that could be modified to prevent the occurrence of chylothorax, and we seek to determine those factors. Retrospective chart review of 285 consecutive patients < 18 years who underwent cardiac surgery from 2015 to 2017 at a single institution pediatric intensive care unit. Data was collected on patient demographics, cardiac lesion, surgical and post-operative characteristics. Primary outcome was development of chylothorax. Of 285 patients, median age was 189 days, median weight was 6.6 kg, 48% were female, and 10% had trisomy 21. 3.5% of patients developed upper extremity DVTs, and 8% developed chylothorax. At 24 h following surgery, a majority were in the 0-10% fluid overload category or had a negative fluid balance (63% and 34%, respectively), and a positive fluid balance was rare at 72 h (16%). In univariate analysis, age, weight, bypass time, DVT, arrhythmia, and trisomy 21 were significantly associated with chylothorax and adjusted for in logistic regression. Presence of an upper extremity DVT (OR 49.8, p < 0.001) and trisomy 21 (OR 5.8, p < 0.001) remained associated with chylothorax on regression modeling. The presence of an upper extremity DVT and trisomy 21 were associated with the development of chylothorax. Fluid overload was rare in our population. The presence of positive fluid balance, fluid overload, elevated central venous pressure, and early initiation of fat containing feeds were not associated with chylothorax.

Modified Distal Dunk Technique for Right Ventricle to Pulmonary Artery Shunt in Stage 1 Palliation.

Although the use of a right ventricle to pulmonary artery shunt as a source of pulmonary blood flow in stage 1 Norwood palliation is common, there is no uniform agreement on the optimal surgical technique. We present a technique for creating the distal connection aimed at minimizing procedural bleeding, promoting distal patency, and facilitating second-stage palliation.

Does Prophylactic Ibuprofen After Surgical Atrial Septal Defect Repair Decrease the Rate of Post-Pericardiotomy Syndrome?

Post-pericardiotomy syndrome (PPS) is an inflammatory process involving the pleura, pericardium, or both and occurs after cardiothoracic surgery. Surgical atrial septal defect (ASD) closure is associated with higher incidence of PPS post-operatively as compared to other operations. Reported incidence of PPS varies from 1 to 40%. NSAIDs are often used to treat PPS and in our center, some practitioners have prescribed ibuprofen prophylactically. This study sought to investigate the impact of prophylactic treatment with ibuprofen on the development and severity of PPS following surgical ASD closure, with particular attention to secundum-type ASDs. We retrospectively reviewed clinical and operative data of all surgical ASD repairs in our center from 1/2007 to 7/2017. ASDs were grouped by subtype. PPS was considered positive if the primary cardiologist diagnosed and documented clinical signs of PPS on post-operative outpatient follow-up. Records were reviewed to confirm documented diagnosis of PPS. A total of 245 cases were reviewed with 207 having sufficient data. Median age was 2 years (range 4 months-27 years), female 57%. Overall incidence of PPS was 10%. There was no difference in incidence of PPS in those prescribed ibuprofen as compared to those who were not. This was true for both the entire cohort and the subgroup analysis (P = 1.0). Four patients overall required pericardiocentesis, none of whom received prophylactic ibuprofen. Prophylactic ibuprofen prescription following surgical ASD repair did not reduce the rate of PPS in our cohort.

Promoting Pulmonary Arterial Growth via Right Ventricle-to-Pulmonary Artery Connection in Children With Pulmonary Atresia, Ventricular Septal Defect, and Hypoplastic Pulmonary Arteries.

BACKGROUND: Complete repair of pulmonary atresia (PA) ventricular septal defect (VSD) with hypoplastic or absent native pulmonary arteries, often with major aortopulmonary collateral arteries (MAPCAs), involves construction of an adequate sized pulmonary arterial tree. We report our results with a previously described staged strategy using initial right ventricle (RV)-to-reconstructed pulmonary arterial tree (RV-PA) connection to promote pulmonary arterial growth and facilitate later ventricular septation. METHODS: We retrospectively reviewed data for all patients (N = 10) with initial echocardiographic diagnosis of PA-VSD and hypoplastic pulmonary arteries operated in our center from October 2008 to August 2016. Pulmonary arterial vessel size measured on preoperative and postoperative angiography was used to calculate Nakata index. RESULTS: Seven patients had PA-VSD, three had virtual PA-VSD, and seven had MAPCAs. All underwent creation of RV-PA connection at a median age of 7.5 days and weight 3.6 kg. Eight patients had RV-PA conduits, two had a transannular patches, and seven had major pulmonary artery reconstruction simultaneously. There were no deaths or serious morbidity; one conduit required revision prior to complete repair. Complete repair with ventricular septation and RV pressure less than half systemic was achieved in all patients at a median age of 239 days. Nakata index in neonatal period was 54 mm2/m2 (range 15-144 mm2/m2) and at time of septation 184 mm2/m2 (range 56-510 mm2/m2; P = .004). Growth rates of right and left branch pulmonary arteries were similar. The 10 patients underwent 28 catheterizations with 13 interventions in 8 patients prior to full repair. CONCLUSION: Early palliative RV-PA connection promotes pulmonary arterial growth and facilitates eventual full repair with VSD closure with low RV pressure and operative risk.

Variation in Perfusion Strategies for Neonatal and Infant Aortic Arch Repair: Contemporary Practice in the STS Congenital Heart Surgery Database.

BACKGROUND: Regional cerebral perfusion (RCP) is used as an adjunct or alternative to deep hypothermic circulatory arrest (DHCA) for neonates and infants undergoing aortic arch repair. Clinical studies have not demonstrated clear superiority of either strategy, and multicenter data regarding current use of these strategies are lacking. We sought to describe the variability in contemporary practice patterns for use of these techniques. METHODS: The Society of Thoracic Surgeons Congenital Heart Surgery Database (2010-2013) was queried to identify neonates and infants whose index operation involved aortic arch repair with cardiopulmonary bypass. Perfusion strategy was classified as isolated DHCA, RCP (with less than or equal to ten minutes of DHCA), or mixed (RCP with more than ten minutes of DHCA). Data were analyzed for the entire cohort and stratified by operation subgroups. RESULTS: Overall, 4,523 patients (105 centers) were identified; median age seven days (interquartile range: 5.0-13.0). The most prevalent perfusion strategy was RCP (43%). Deep hypothermic circulatory arrest and mixed perfusion accounted for 32% and 16% of cases, respectively. In all, 59% of operations involved some period of RCP. Regional cerebral perfusion was the most prevalent perfusion strategy for each operation subgroup. Neither age nor weight was associated with perfusion strategy, but reoperations were less likely to use RCP (31% vs 45%, P < .001). The combined duration of RCP and DHCA in the RCP group was longer than the DHCA time in the DHCA group (45 vs 36 minutes, P < .001). CONCLUSION: There is considerable variability in practice regarding perfusion strategies for arch repair in neonates and infants. In contemporary practice, RCP is the most prevalent perfusion strategy for these procedures. Use of DHCA is also common. Further investigation is warranted to ascertain possible relative merits of the various perfusion techniques.

A primary cardiac sarcoma with features of a desmoplastic small round cell tumor in an adolescent male.

Malignant primary cardiac tumors are very rare.Desmoplastic small round cell tumors are also rare tumors and,when present, are generally found in the abdomen. We report a case of an adolescent male who presented with chest pain,abdominal pain, and difficulty in breathing, who was found to have a primary cardiac sarcoma with several characteristic features of a desmoplastic small round cell tumor.

Outcomes of the Fontan procedure using cardiopulmonary bypass with aortic cross-clamping.

BACKGROUND: Avoidance of cardiopulmonary bypass (CPB) and aortic cross-clamping during the Fontan procedure has been advocated to improve outcomes. We continue to use CPB with aortic cross-clamping for the Fontan procedure. METHODS: We performed a review of patients undergoing the Fontan procedure between January 1, 2000 and December 31, 2004. RESULTS: The Fontan procedure was performed in 160 patients. The median age was 2.2 years (range, 1.0 to 29.1 years). Hypoplastic left heart syndrome or a variant was present in 114 patients (71%), and heterotaxy was present in 19 (12%). CPB and modified ultrafiltration were used in all patients. Aortic cross-clamping was used in 154 (96%) of 160 patients and deep hypothermic circulatory arrest (DHCA) in 132 (83%). A lateral tunnel Fontan was performed in 69 patients (43%) and an extracardiac Fontan in 91 (57%). A fenestration was created in 144 patients (90%). Two patients died. Freedom from death or takedown was 98% (157/160). Median duration of pleural drainage was 2 days (range, 1 to 44 days) and was more than 14 days in 16 patients. Median duration of hospitalization was 6 days (range, 3 to 55 days). The small number of deaths precluded assessment of risk factors for mortality. By multivariable analysis, risk factors for pleural drainage longer than 3 days were extracardiac connection (p < 0.001) and increasing mean pulmonary artery pressure before the Fontan procedure (p = 0.033). By multivariable analysis, risk factors for hospitalization for more than 7 days were extracardiac connection (p = 0.003), increasing duration of total support (CPB and DHCA, p = 0.027), and decreasing systemic oxygen saturation before the Fontan procedure (p = 0.048). CONCLUSIONS: The Fontan procedure can be performed using CPB and aortic cross-clamping with low morbidity and mortality.