Resolution of Severe Ulcerative Colitis Secondary to Nickel Allergy Following Explantation of Amplatzer Septal Occluder Device: A Delayed Presentation.

Nickel is a component of nitinol, an alloy used in several medical devices. Allergy to nickel may place patients at a high risk for severe hypersensitivity reactions. We report a rare case of a patient who developed severe ulcerative colitis ten years following closure of an atrial septal defect with the Amplatzer Septal Occluder device.

Revisiting the Role of Balloon Atrial Septostomy Prior to the Arterial Switch Operation.

OBJECTIVES: Patients with dextro-transposition of the great arteries (d-TGA) frequently undergo balloon atrial septostomy (BAS) prior to the arterial switch operation (ASO) to promote atrial-level mixing. Balloon atrial septostomy has inherent risks as an invasive procedure and may not always be necessary. This study revisits the routine utilization of BAS prior to ASO. METHODS: Single-center, retrospective review of d-TGA patients undergoing the ASO from July 2018 to March 2023. Preoperative patient characteristics, pulse oximetry oxygen saturations (SpO2), cerebral/renal near-infrared spectroscopy (NIRS) readings along with prostaglandin status at the time of the ASO were analyzed with descriptive and univariate statistics. RESULTS: Thirty patients underwent the ASO. Of these, 7 (23%) were female, 25 (83%) were white, and median weight at ASO was 3.2 kg (range 0.8-4.2). Twenty-two (73%) patients underwent BAS. There were no demographic differences between BAS and no-BAS patients. Of those who underwent BAS, there was a significant increase in SpO2 (median 83% [range 54-92] to median 87% [range 72-95], P = .007); however, there was no change in NIRS from pre-to-post BAS. Six (27%) patients in the BAS group were prostaglandin-free at ASO. Balloon atrial septostomy patients underwent the ASO later compared with no-BAS patients (median 8 [range 3-32] vs 4 [range 2-10] days old, P = .016) and had a longer hospital length of stay (median 13 [range 7-43] vs 10 [range 8-131] days, P = .108). CONCLUSIONS: While BAS is an accepted preoperative procedure in d-TGA patients to improve oxygen saturations, it is also an additional invasive procedure, does not guarantee prostaglandin-free status at the time of ASO, and may increase the interval to ASO. Birth to direct early ASO, with prostaglandin support, should be revisited as an alternative, potentially more expeditious strategy.

Pericardial Effusions After the Arterial Switch Operation: A PHIS Database Review.

Background: Pericardial effusion (PCE) is a significant complication after pediatric cardiac surgery. This study investigates PCE development after the arterial switch operation (ASO) and its short-term and longitudinal impacts. Methods: A retrospective review of the Pediatric Health Information System database. Patients with dextro-transposition of the great arteries who underwent ASO from January 1, 2004, to March 31, 2022, were identified. Patients with and without PCE were analyzed with descriptive, univariate, and multivariable regression statistics. Results: There were 4896 patients identified with 300 (6.1%) diagnosed with PCE. Thirty-five (11.7%) with PCE underwent pericardiocentesis. There were no differences in background demographics or concomitant procedures between those who developed PCE and those who did not. Patients who developed PCE more frequently had acute renal failure (N = 56 (18.7%) vs N = 603(13.1%), P = .006), pleural effusions (N = 46 (15.3%) vs N = 441 (9.6%), P = .001), mechanical circulatory support (N = 26 (8.7%) vs N = 199 (4.3%), P < .001), and had longer postoperative length of stay (15 [11-24.5] vs 13 [IQR: 9-20] days). After adjustment for additional factors, pleural effusions (OR = 1.7 [95% CI: 1.2-2.4]), and mechanical circulatory support (OR = 1.81 [95% CI: 1.15-2.85]) conferred higher odds of PCE. There were 2298 total readmissions, of which 46 (2%) had PCE, with no difference in median readmission rate for patients diagnosed with PCE at index hospitalization (median 0 [IQR: 0-1] vs 0 [IQR: 0-0], P = .208). Conclusions: PCE occurred after 6.1% of ASO and was associated with pleural effusions and mechanical circulatory support. PCE is associated with morbidity and prolonged length of stay; however, there was no association with in-hospital mortality or readmissions.

Assessment of Phenobarbital Administration to Reduce Opioid and Benzodiazepine Use in the Immediate Postoperative Period Following Stage 2 Single-Ventricle Palliation.

OBJECTIVE: The Glenn procedure may lead to the development of elevated cerebral venous pressures, which is believed to result in "Glenn headaches." This manifests as excessive irritability, often requiring significant use of opioids and benzodiazepines. This study was designed to report our experience with the use of phenobarbital in the postoperative phase after the Glenn procedure. METHODS: We performed a retrospective chart review to compare Glenn patients before and after implementation of a sedation protocol using phenobarbital. The 2 groups were compared for demographics, surgical characteristics, and cumulative sedation usage. Correlation coefficients between the preoperative catheterization variables and sedation usage were also calculated. RESULTS: Groups A (pre-phenobarbital; n = 8) and B (post-phenobarbital; n = 11) were comparable in terms of demographics, cardiac anatomy, preoperative catheterization data, and hemodynamics. Patients in Group B received a median dose of 21.8 mg/kg of phenobarbital during their ICU stay. Although there was a decreased administration of morphine equivalents (2.60 mg/kg vs 2.25 mg/kg, p = 0.38), benzodiazepine (0.1 mg/kg vs 0.074 mg/kg, p = 0.43), and dexmedetomidine (47 mcg/kg vs 37.2 mcg/kg, p = 0.53) in Group B, the differences were not statistically significant. There was also no strong correlation between preoperative hemodynamic variables and the postoperative sedation requirement, and there was no statistically significant difference in overall outcomes between the 2 groups. CONCLUSIONS: While phenobarbital may have mitigated the use of opioids, benzodiazepines, and alpha-agonist agents in some postoperative Glenn patients, the overall findings for all patients were not statistically significant. Further prospective studies are needed to ascertain the role of phenobarbital in these patients.

Post-Fontan pulmonary artery growth in patients with a bidirectional cavopulmonary shunt with additional antegrade pulsatile blood flow.

BACKGROUND: Patients with antegrade pulmonary blood flow after a bidirectional cavopulmonary shunt (Glenn) may have better pulmonary artery growth. This study evaluated pulmonary artery growth in patients with and without prior additional pulsatile antegrade flow in a Glenn shunt at midterm follow-up after a Fontan procedure. METHODS: We reviewed 212 patients who had single-ventricle palliation in a 10-year period;103 (33 in pulsatile group 1 and 70 in nonpulsatile group 2) were selected for analysis. Data on demographics, procedures, perioperative course, and midterm follow-up after the Fontan procedure were compared. Echocardiography data were collected. Pulmonary artery sizes measured at cardiac catheterization and follow-up echocardiograms were used to calculate the Nakata index. RESULTS: Perioperative details were comparable in both groups, mean pulmonary artery pressure and systemic oxygen saturations were higher in group 1 compared to group 2. Venovenous collaterals were increased in group 1. There was a significant difference in the pre-Fontan and follow-up Nakata index between groups. There was a significant increase in the Nakata index in group 1 between the pre-Glenn and pre-Fontan assessments as well as the Nakata index between the pre-Fontan and midterm follow-up. There was no significant change in the Nakata index in group 2 between assessments. CONCLUSIONS: A pulsatile Glenn shunt is associated with better pulmonary artery growth which continues long after the additional pulsatile flow is eliminated. It is possible that the effects of anterograde pulmonary blood flow on pulmonary artery growth in early life continue long after the Fontan completion.

Surgical Management of Neonatal Ebstein's Anomaly Associated With Circular Shunt.

Ebstein's anomaly is a rare congenital cardiac malformation that may present in the neonatal period with life-threatening physiologic derangement, especially when it is associated with circular shunt. Urgent surgical intervention is critical for survival because of hemodynamic compromise. We review our experience with surgical management of neonates with Ebstein's anomaly associated with circular shunt and review the literature for similar reported cases. The underlying pathophysiology and different options of treatment are discussed.

Attrition in patients with single ventricle and trisomy 21: outcomes after a total cavopulmonary connection.

OBJECTIVES: Data are limited regarding the management of children with trisomy 21 (T21) syndrome and a functional single ventricle (FSV). We evaluated patients with T21 and a FSV who had a total cavopulmonary connection (TCPC). METHODS: From September 1999 to August 2012, 139 patients with a FSV underwent a TCPC. Sixty-five had unbalanced atrioventricular septal defect. Thirteen had T21. Three (of 13) had heterotaxy syndrome. The mean age at the Fontan operation was 27.6 ± 12.1 months. RESULTS: The initial procedure was pulmonary artery banding in 9 patients, systemic-to-pulmonary shunt in 2 and Damus-Kaye-Stansel/Norwood procedure in 2. Median follow-up was 69 months (interquartile range 25-75, 21-99). There was 1 death after a Damus-Kaye-Stansel/Norwood procedure and one interstage death after a bidirectional Glenn procedure. Nine (of 11) survivors underwent a Fontan operation. A fenestrated Fontan procedure was the predominate operation in 78%. One patient was deemed unsuitable for a Fontan operation. There was 1 takedown and 1 late death after the Fontan operation. Heterotaxy syndrome did not affect outcome ( P > 0.05). There was no statistical difference in the pre-Fontan McGoon ratio, hospital length of stay, duration of pleural drainage and Fontan-related adverse events between patients with a dominant right ventricle and those with a left ( P > 0.05). CONCLUSIONS: A TCPC in patients with T21 and an FSV is associated with reproducible, satisfactory outcomes. An assisted-Glenn procedure with pulsatile pulmonary blood flow and a fenestrated Fontan may be associated with attenuated perioperative morbidity and late attrition.

Improved Results of Aortic Arch Reconstruction in the Norwood Procedure.

BACKGROUND: Despite several surgical modifications, the reported incidence of neoaortic coarctation after stage 1 palliation (S1P) of hypoplastic left heart syndrome remains high. A modification of aortic arch reconstruction that may decrease the incidence of neoaortic coarctation after S1P is described. METHODS: Between January 2008 and May 2013, 114 patients with hypoplastic left heart syndrome underwent S1P. The hospital records of the 101 surviving patients (89%) were reviewed. Demographic data and perioperative variables for S1P were collected. A modified technique for aortic arch reconstruction that contains a bovine pericardial patch tailored and sutured in a specific way was used in 51 patients (group MT), whereas the traditional technique was used in 50 patients (group TT). Clinical echocardiographic and cardiac catheterization data were analyzed to determine the incidence and location of neoaortic coarctation. RESULTS: There were no significant differences in the baseline characteristics, the intraoperative course, or hospital mortality between the two groups of subjects. Duration of ventilation, intensive care unit stay, and hospitalization were significantly shorter in group MT. The mean follow-up was 2.02 ± 0.63 years and 3.98 ± 0.66 years in each group, respectively. Coarctation developed in 4 of 51 patients (7.8%) in group MT compared with 10/50 patients (20%) in group TT. Multivariable logistic regression analysis showed that the estimated odds of coarctation for group MT were 0.41 (95% confidence interval: 0.04, 4.32) times the estimated odds of coarctation for group TT. CONCLUSIONS: The described technique may decrease the incidence of recoarctation after S1P by minimizing aortic arch and descending aorta distortion and providing ample enlargement of the aorta at its narrowest diameter.

Relationship of Normal Aortic Valve Cusp Dimensions: A Tool to Optimize Cusp Reconstruction Valvuloplasty.

Outcomes of surgical aortic valvuloplasty hinge primarily on optimal leaflet design. Imprecise valve reconstruction can result in insufficiency or stenosis. Predicting postrepair leaflet parameters from pre- or intraoperative readily measurable valve dimensions could result in improved reconstruction. This study analyzes the relationships between different parameters of the normal aortic valve to establish a method of deriving the optimal dimensions of the reconstructed leaflet following valvuloplasty. Morphologic analysis of the normal aortic valve was performed on 50 autopsy specimens. The following parameters were measured: circumference at ventriculoaortic junction, intercommissural circumferential distance (IC), commissural height, leaflet free-margin length (L1-length of the free margin of each leaflet), cusp attachment length (L2-cusp attachment length at the ventriculoaortic junction), and leaflet height (A-ie, the vertical or cephalocaudal distance along the convex surface of the leaflet from the midpoint of the leaflet free margin to the midpoint of the cusp attachment). Univariate linear regression analysis was used to test the interdependence of leaflet dimensions. The leaflet free-margin length L1 correlated strongly with the corresponding IC: r = 0.74, 0.81, and 0.79 for noncoronary, right coronary, and left coronary leaflets, respectively; P = 0.000. Leaflet height A and cusp attachment length L2 also correlated well with the corresponding IC: A-IC, r = 0.56, 0.74, and 0.66; and L2-IC, r = 0.78, 0.85, and 0.80 for noncoronary, right coronary, and left coronary cusps respectively; P = 0.000. Parameters required to construct an aortic valve leaflet can be reliably derived from the IC, which can be easily measured perioperatively. Using this as a basis for leaflet reconstruction might optimize the leaflet graft design process, compensate for pathologic variations in aortic annulus size and commissural height, and could result in better coaptation of the valve leaflets.

Evolution of the surgical approach to congenitally corrected transposition of the great arteries.

The traditional surgical approach (physiologic repair) of congenitally corrected transposition of the great arteries (ccTGA) attempts at restoring normal physiology by repairing the associated lesions. It fails to address the most serious anatomic abnormality, mainly ventriculoarterial discordance, and results in less than optimal long-term outcomes. Anatomic repair was introduced to incorporate the left ventricle into the systemic circulation. The excellent short-term and intermediate results of the double switch operation and its modifications make it the procedure of choice for the treatment of ccTGA.

Systematic approach to aortic valvuloplasty in children and young adults.

Valve repair in children and young adults is an attractive alternative to replacement or Ross procedure. In the past, the various valvuloplasty techniques have addressed isolated valve pathology. Recently, however, a holistic approach involving repair of all aortic root structures has evolved and promised to be an excellent alternative to valve replacement. It provides good long-term results and avoids all the drawback of prosthetic valve insertion in this age group. Development of ideal patch material needed for valve repair and better understanding of the anatomy and function of the aortic root may further improve the short- and long-term outcomes of aortic valvuloplasty.

Is additional pulsatile pulmonary blood flow beneficial to patients with bidirectional Glenn?

OBJECTIVE: To compare the results of bidirectional Glenn when performed with or without pulsatile pulmonary blood flow in a cohort of patients with a single ventricle. METHODS: Records of 212 patients undergoing staged single ventricle palliation during a 10-year period were retrospectively reviewed. Of those, 103 (33 in pulsatile group A and 70 in nonpulsatile group B) were selected. RESULTS: Demographics and pre- and intraoperative variables were comparable for both groups. There was no difference in oxygen saturations immediately after the bidirectional Glenn in the 2 groups. The duration and output of chest tube drainage, incidence of chylothorax, and total length of stay was higher in group A. There was no difference in the number of diuretics or oxygen requirement upon discharge between groups. Pre-Glenn measurements showed a mean McGoon ratio in group A of 1.5 (1.46-1.57) and in group B of 1.59 (1.53-1.7) (P = .11); however, there was a significant difference in the ratio between groups at pre-Fontan measurements: group A, 1.76 (1.73-1.79) and group B, 1.6 (1.53-1.66) (P < .05). At pre-Fontan measurements there was a significant difference in mean pulmonary artery pressure between group A (14 mm [12.8-15.2]) and group B (10 mm [9.7-11]) (P < .05) and a trend toward higher incidence of venovenous collaterals in group A. There was no perioperative or interstage mortality in either group. CONCLUSIONS: Pulsatile bidirectional Glenn is associated with better pulmonary artery growth, which might improve long-term outcomes after Fontan. However, it was associated with a higher postoperative complication rate.

Comparison of wire-reinforced and non-wire-reinforced dual-lumen catheters for venovenous ECMO in neonates and infants.

Improved dual-lumen catheter design has resulted in greater efficiency of gas exchange during extracorporeal life support. However, standard venovenous (S-VV) catheters are prone to structural deformation that reduces flow capacity. Wire-reinforced dual-lumen catheters have recently been introduced to reduce the risk of structural deformation. A retrospective review was performed on 25 neonates and infants who received venovenous extracorporeal life support (ECLS) to evaluate the occurrence and nature of catheter-related complications and events related to interrupted ECLS. Comparisons were made between patients supported with wire-reinforced and non-wire-reinforced dual-lumen catheters. No significant difference in number of catheter-related complications or flow-interruption events was observed. The use of wire-reinforced catheters appeared to increase the risk of cardiac perforation whereas non-wire-reinforced catheters appeared to be more prone to early failure. Once support was established, interruptions of extracorporeal flow occurred with the same frequency during the initial 72 hours, regardless of the type of catheter used. Further improvements in dual-lumen VV catheter design may reduce the risk of failure and injury and improve efficiency of extracorporeal gas exchange.

Proximal sano anastomosis aneurysm due to degeneration of bovine pericardial patch.

We report a 5-year-old girl with hypoplastic left heart syndrome who originally underwent a Norwood procedure with a Sano shunt. Subsequent procedures included a bidirectional Glenn and extracardiac Fontan with a Gore-Tex graft (W.L Gore and Associates, Flagstaff, AZ). The patient was found to have a heart murmur on a follow-up visit 3 years later. Echocardiography and computed tomography imaging showed an aneurysm of the proximal Sano anastomosis. The patient underwent successful surgical repair.

Novel pH1N1 viral cardiomyopathy requiring veno-venous extracorporeal membrane oxygenation.

OBJECTIVE: To report a case of pH1N1 viral infection presenting as heart failure requiring mechanical extracorporeal life support. DESIGN: Case report. SETTING: Pediatric intensive care unit at a regional children's hospital. PATIENT: Obese 15-yr-old boy who presented with pH1N1-related cardiomyopathy and respiratory failure that required extracorporeal membrane oxygenation. INTERVENTIONS: Extracorporeal membrane oxygenation, echocardiography, high-frequency oscillating ventilation. MEASUREMENTS AND MAIN RESULTS: Discovery of severe dilated cardiomyopathy and respiratory failure. CONCLUSIONS: Patients with pH1N1 may present in profound heart failure in addition to respiratory failure. Extracorporeal membrane oxygenation may play an important role in managing these complex patients.

Repair of mitral valve and left atrioventricular disruption caused by blunt chest trauma.

We describe a case of acute cardiac failure in a patient involved in a motor vehicle accident. A diagnosis of acute mitral regurgitation and left atrioventricular disruption was made with transesophageal echocardiography. Successful repair was performed using the mitral valve leaflets to patch the disruption followed by valve replacement.

Flow impeding fungal thrombus in the ascending aorta.

Mycotic endoaortitis is a rarely occurring infectious entity with an extremely high mortality rate. We report an uncommon case of Phialemonium endoaortits with its management. This large fungal thrombus in the ascending aorta caused significant impediment to the blood flow.

Acute sirolimus pulmonary toxicity in an infant heart transplant recipient: case report and literature review.

Sirolimus-associated pulmonary disease should be considered in the differential diagnosis of acute respiratory distress syndrome in transplant recipients receiving this drug. It represents a rare, potentially lethal, and yet reversible adverse effect. We report the case an infant who presented with acute respiratory distress 57 days after heart transplantation 3 days after starting sirolimus. The acute presentation and prompt resolution after discontinuation of this drug suggest a direct toxic effect to the lungs. To our knowledge, this is the first published pediatric description of this syndrome after heart transplantation.

Advanced age and preinjury warfarin anticoagulation increase the risk of mortality after head trauma.

BACKGROUND: A large population of patients on oral anticoagulants is exposed to the risk of traumatic brain injury (TBI). Effects of age and anticoagulation on TBI outcomes need to be assessed separately. METHODS: Retrospective analysis of consecutive series of TBI patients (age 18 years and older) in a suburban teaching hospital. RESULTS: A total of 1,493 adult blunt head trauma patients between January 2001 and May 2005 were analyzed. Of these, 159 patients were warfarin-anticoagulated at the time of trauma. The mortality in anticoagulated patients was statistically significantly higher than in the control group (38/159, 23.9% vs. 66/1,334, 4.9%; p < 0.001; odds ratio 6.0). Mortality of patients over 70 years of age was significantly higher than in the younger population (p < 0.001). Both mortality and the occurrence of intracranial hemorrhage (ICH) after head trauma were significantly increased with higher INR (Cochran's linear trend p < 0.001), especially with INR over 4.0 (mortality 50%, risk of ICH 75%). Preinjury warfarin anticoagulation and age were found to be predictive of survival in a binary logistic regression model (92.5% correct prediction, p = 0.027). Addition of Injury Severity Score and initial Glasgow Coma Score to this model only modestly improved its predictive performance (95.4% correct prediction, p < 0.001). CONCLUSIONS: Both age and warfarin anticoagulation are independent predictors of mortality after blunt TBI. Warfarin anticoagulation carries a six-fold increase in TBI mortality. Age over 70 years and excessive anticoagulation are associated with higher mortality, as well.