Gastrostomy and Tracheostomy After Complete Repair of Tetralogy of Fallot in Children With 22q11.2 Deletion Syndrome.

OBJECTIVES: Caring for a child with gastrostomy and/or tracheostomy can cause measurable parental stress. It is generally known that children with 22q11.2 deletion syndrome are at greater risk of requiring gastrostomy or tracheostomy after heart surgery, although the magnitude of that risk after complete repair of tetralogy of Fallot has not been described. We sought to determine the degree to which 22q11.2 deletion is associated with postoperative gastrostomy and/or tracheostomy after repair of tetralogy of Fallot. DESIGN: Retrospective cohort study. SETTING: Pediatric Health Information System. PATIENTS: Children undergoing complete repair of tetralogy of Fallot (ventricular septal defect closure and relief of right ventricular outflow tract obstruction) from 2003 to 2016. Patients were excluded if they had pulmonary atresia, other congenital heart defects, and/or genetic diagnoses other than 22q11.2 deletion. MEASUREMENTS AND MAIN RESULTS: Two groups were formed on the basis of 22q11.2 deletion status. Outcomes were postoperative tracheostomy and postoperative gastrostomy. Bivariate analysis and Kaplan-Meier analysis at 150 days postoperatively were performed. There were 4,800 patients, of which 317 (7%) had a code for 22q11.2 deletion. There were no significant differences between groups for age at surgery or sex. Patients with 22q11.2 deletion had significantly higher rates of gastrostomy (18% vs 5%; p < 0.001) and higher rates of tracheostomy (7% vs 1%; p < 0.001); there was no difference for mortality. Kaplan-Meier analyses also showed higher rates of gastrostomy (p = 0.024) and tracheostomy (p = 0.037). CONCLUSIONS: The present study establishes rates of postoperative gastrostomy and tracheostomy in children with 22q11.2 deletion after complete repair of tetralogy of Fallot. These data are useful to clinicians for providing families with preoperative counseling.

Ventricular arrhythmia burden after transcatheter versus surgical pulmonary valve replacement.

OBJECTIVE: Comparative ventricular arrhythmia (VA) outcomes following transcatheter (TC-PVR) or surgical pulmonary valve replacement (S-PVR) have not been evaluated. We sought to compare differences in VAs among patients with congenital heart disease (CHD) following TC-PVR or S-PVR. METHODS: Patients with repaired CHD who underwent TC-PVR or S-PVR at the UCLA Medical Center from 2010 to 2016 were analysed retrospectively. Patients who underwent hybrid TC-PVR or had a diagnosis of congenitally corrected transposition of the great arteries were excluded. Patients were screened for a composite of non-intraoperative VA (the primary outcome variable), defined as symptomatic/recurrent non-sustained ventricular tachycardia (VT) requiring therapy, sustained VT or ventricular fibrillation. VA epochs were classified as 0-1 month (short-term), 1-12 months (mid-term) and ≥1 year (late-term). RESULTS: Three hundred and two patients (TC-PVR, n=172 and S-PVR, n=130) were included. TC-PVR relative to S-PVR was associated with fewer clinically significant VAs in the first 30 days after valve implant (adjusted HR 0.20, p=0.002), but similar mid-term and late-term risks (adjusted HR 0.72, p=0.62 and adjusted HR 0.47, p=0.26, respectively). In propensity-adjusted models, S-PVR, patient age at PVR and native right ventricular outflow tract (RVOT) (vs bioprosthetic/conduit outflow tract) were independent predictors of early VA after pulmonary valve implantation (p<0.05 for all). CONCLUSION: Compared with S-PVR, TC-PVR was associated with reduced short-term but comparable mid-term and late-term VA burdens. Risk factors for VA after PVR included a surgical approach, valve implantation into a native RVOT and older age at PVR.

Heparin-Coated Grafts Reduce Mortality in Pediatric Patients Receiving Systemic-to-Pulmonary Shunts.

We aimed to evaluate the outcomes of systemic-to-pulmonary (SP) shunt procedures utilizing heparin-coated (HC) polytetrafluoroethylene (PTFE) vascular grafts compared to uncoated (non-HC) grafts, in order to observe any benefits in pediatric patients. Our institution switched from using non-HC grafts to HC grafts in March 2011. We conducted a retrospective review of consecutive pediatric patients receiving SP shunts from May 2008 to December 2015. Perioperative variables including baseline characteristics, morbidity, mortality, and blood product utilization were evaluated between the HC and non-HC groups. A total of 142 pediatric patients received SP shunts during the study period: 69 patients received HC shunts and 73 patients received non-HC shunts. The HC group had significantly fewer desaturation or arrest events (P < 0.01), fewer shunt occlusions/thromboses (P < 0.01). There was no statistically significant difference in unplanned reoperations between groups (P = 0.18). The HC group demonstrated significantly lower overall 30-day mortality (P < 0.01), as well as shunt-related mortality (P < 0.01). The HC group had significantly lower postoperative packed red blood cell utilization as compared to the non-HC group (P < 0.01). In this study, pediatric patients receiving HC PTFE grafts in SP shunts demonstrated significantly lower shunt-related mortality. The majority of HC grafts remained patent. These findings suggest that HC grafts used in SP shunt procedures may benefit pediatric patients in terms of efficacy and outcomes.

4D MUSIC CMR: value-based imaging of neonates and infants with congenital heart disease.

BACKGROUND: 4D Multiphase Steady State Imaging with Contrast (MUSIC) acquires high-resolution volumetric images of the beating heart during uninterrupted ventilation. We aim to evaluate the diagnostic performance and clinical impact of 4D MUSIC in a cohort of neonates and infants with congenital heart disease (CHD). METHODS: Forty consecutive neonates and infants with CHD (age range 2 days to 2 years, weight 1 to 13 kg) underwent 3.0 T CMR with ferumoxytol enhancement (FE) at a single institution. Independently, two readers graded the diagnostic image quality of intra-cardiac structures and related vascular segments on FE-MUSIC and breath held FE-CMRA images using a four-point scale. Correlation of the CMR findings with surgery and other imaging modalities was performed in all patients. Clinical impact was evaluated in consensus with referring surgeons and cardiologists. One point was given for each of five key outcome measures: 1) change in overall management, 2) change in surgical approach, 3) reduction in the need for diagnostic catheterization, 4) improved assessment of risk-to-benefit for planned intervention and discussion with parents, 5) accurate pre-procedural roadmap. RESULTS: All FE-CMR studies were completed successfully, safely and without adverse events. On a four-point scale, the average FE-MUSIC image quality scores were >3.5 for intra-cardiac structures and >3.0 for coronary arteries. Intra-cardiac morphology and vascular anatomy were well visualized with good interobserver agreement (r = 0.46). Correspondence between the findings on MUSIC, surgery, correlative imaging and autopsy was excellent. The average clinical impact score was 4.2 ± 0.9. In five patients with discordant findings on echo/MUSIC (n = 5) and catheter angiography/MUSIC (n = 1), findings on FE-MUSIC were shown to be accurate at autopsy (n = 1) and surgery (n = 4). The decision to undertake biventricular vs univentricular repair was amended in 2 patients based on FE-MUSIC findings. Plans for surgical approaches which would have involved circulatory arrest were amended in two of 28 surgical cases. In all 28 cases requiring procedural intervention, FE-MUSIC provided accurate dynamic 3D roadmaps and more confident risk-to-benefit assessments for proposed interventions. CONCLUSIONS: FE-MUSIC CMR has high clinical impact by providing accurate, high quality, simple and safe dynamic 3D imaging of cardiac and vascular anatomy in neonates and infants with CHD. The findings influenced patient management in a positive manner.

Association Between Hematologic and Inflammatory Markers and 31 Thrombotic and Hemorrhagic Events in Berlin Heart Excor Patients.

Bleeding and thrombotic events remain a significant cause of morbidity in pediatric patients supported with ventricular assist devices (VADs). The objective of this study is to identify the association between markers of anticoagulation and bleeding and thrombosis events during Berlin Heart ExCor support. A retrospective, single-center analysis of 9 patients supported with the Berlin Heart ExCor was performed. Inflammatory and anticoagulation parameters including C-reactive protein, fibrinogen, partial thromboplastin time (PTT), and platelet count were measured at 48 and 24 h before and after bleeding or thrombosis events. Patients served as their own controls, and the same parameters were measured during a control period where subjects did not experience either event. All patients received the anticoagulation regimen proposed by Berlin Heart. A total of 31 bleeding or thrombotic events were identified and matched to 18 control events. Patient with predominantly thrombotic events tended to weigh less than those with bleeding events (Δ7.7 kg, p < 0.001). PTT levels were higher before and after bleeding (Δ17.36, p = 0.002) and thrombosis (Δ8.75, p < 0.001) events relative to control. Heparin dose decreased after a thrombosis event (Δ-5.67, p = 0.097), and this decrease was significantly different from control (p = 0.032). Non-collinearity between heparin dose and PTT should prompt further inflammatory and hematological investigation. In addition, heavier patients were more prone to bleeding complications. The role of inflammation in the development of thrombus or hemorrhages in the pediatric VAD population needs to be studied further.

MRI with ferumoxytol: A single center experience of safety across the age spectrum.

PURPOSE: To summarize our single-center safety experience with the off-label use of ferumoxytol for magnetic resonance imaging (MRI) and to compare the effects of ferumoxytol on monitored physiologic indices in patients under anesthesia with those of gadofosveset trisodium. MATERIALS AND METHODS: Consecutive patients who underwent ferumoxytol-enhanced (FE) MRI exams were included. Adverse events (AEs) were classified according to the Common Terminology Criteria for Adverse Events v4.0. In a subgroup of patients examined under general anesthesia, recording of blood pressure, heart rate, oxygen saturation, and end-tidal CO2 was performed. A comparable group of 23 patients who underwent gadofosveset-enhanced (GE) MRI under anesthesia with similar monitoring was also analyzed. RESULTS: In all, 217 unique patients, ages 3 days to 94 years, underwent FE-MRI. No ferumoxytol-related severe, life-threatening, or fatal AEs occurred acutely or at follow-up. Two patients developed ferumoxytol-related nausea. Between-group (FE- vs. GE-MRI) comparisons showed no statistical difference in heart rate (P = 0.69, 95% confidence interval [CI] 96-113 bpm), mean arterial blood pressure (MAP) (P = 0.74, 95% CI 44-52 mmHg), oxygen saturation (P = 0.76, 95% CI 94-98%), and end-tidal CO2 (P = 0.73, 95% CI 31-37 mmHg). No significant change in MAP (P = 0.12, 95% CI 50-58 mmHg) or heart rate (P = 0.25, 95% CI 91-105 bpm) was noted between slow infusion of ferumoxytol (n = 113) vs. bolus injection (n = 104). CONCLUSION: In our single-center experience, no serious AEs occurred with the diagnostic use of ferumoxytol across a wide spectrum of age, renal function, and indications. Because of the limited sample size, firm conclusions cannot be drawn about the generalizability of our results. Thus, vigilance and monitoring are recommended to mitigate potential rare adverse reactions. LEVEL OF EVIDENCE: 2 J. Magn. Reson. Imaging 2017;45:804-812.

Unrepaired Tetralogy of Fallot with Absent Pulmonary Valve in a Mildly Symptomatic 16-Year-Old Boy.

Absent pulmonary valve is a rare and severe variant seen in only 3% to 6% of patients with tetralogy of Fallot. Fetuses with this combined condition who survive through birth typically need intervention in infancy or early childhood because of respiratory distress, heart failure, or failure to thrive. We describe the unusual case of a mildly symptomatic 16-year-old boy with these conditions who underwent successful primary repair. Our search of the medical literature yielded fewer than 5 cases of tetralogy of Fallot with absent pulmonary valve (or variants with an absent left pulmonary artery) and survival without repair into later adolescence or adulthood.

Mechanical occlusion of the inferior vena cava: an unusual complication after repair of pectus excavatum using the nuss procedure.

We describe the case of a 13-year-old girl with a pectus excavatum in whom acute occlusion of the inferior vena cava developed after a nuss repair. In this hemodynamically unstable patient, we evaluated the possibility of a penetrating injury to the thoracic and abdominal structures before confirming the diagnosis of inferior vena cava obstruction with a venogram. Removal of the nuss bar relieved the unexpected problem.

Hemidiaphragm plication after repair of congenital heart defects in children: quantitative return of diaphragm function over time.

OBJECTIVE: Phrenic nerve injury resulting in hemidiaphragm paresis leads to morbidity in children undergoing repair of congenital heart defects. Previous studies have documented short-term benefits of diaphragm plication, but little is known about the return of diaphragm function. METHODS: We reviewed 46 consecutive patients undergoing hemidiaphragm plication after repair of congenital heart defects. The function of plicated diaphragms was measured at follow-up fluoroscopy using excursion of the unplicated side as a control. RESULTS: The median age at the procedure resulting in phrenic nerve injury was 6.4 months (0-62 months). Among the 46 patients, 29 (63%) and 17 (37%) had repair for single and 2-ventricle defects, respectively. Hemidiaphragm paresis occurred on the left side in 32 patients (70%). Phrenic nerve injury was documented at a median of 8 days (1-84 days) after operation. The median time from diagnosis to plication was 2 days (0-21 days). Five patients required prolonged ventilation after plication. One patient died 10 weeks later, and 4 patients required tracheostomy. The remaining 41 patients were extubated within 2 days (0-19 days). In 17 patients, fluoroscopy assessing diaphragm motion was performed at a mean interval of 16.4 months after plication. Excursion of the plicated diaphragm was 77% of the contralateral side. There was a trend toward improved function over time. CONCLUSIONS: Hemidiaphragm paresis results in significant morbidity after repair of congenital heart defects. Early diagnosis and plication result in timely extubation. The plicated diaphragm demonstrates return of function that may improve over time. This is the first study to numerically quantitate the degree of diaphragm recovery.

Fate of the right ventricle after fenestrated right ventricular exclusion for severe neonatal Ebstein anomaly.

OBJECTIVES: Neonates with Ebstein anomaly can have severe left ventricular dysfunction caused by right ventricular enlargement and ventricular septal displacement. Fenestrated right ventricular exclusion and Blalock-Taussig shunt (Starnes procedure) have been performed to mitigate this problem; however, the fate of the excluded ventricle and its effect on the systemic ventricle have yet to be documented. METHODS: Intermediate-term data have been retrospectively collected on the 12 survivors of neonatal right ventricular exclusion. Echocardiographic examinations were compared from the time of the original right ventricular exclusion and before second-stage Glenn palliation. Measurement of the Great Ormond Street ratio (area of right atrium plus atrialized right ventricle divided by area of trabeculated right ventricle plus left atrium and left ventricle), ratio of right ventricular to left ventricular area, degree of ventricular septal impingement into the left ventricle, and left ventricular shortening fraction have been documented. RESULTS: In the 12 survivors of right ventricular exclusion, a decrease in the mean Great Ormond Street ratio was observed (before fenestrated right ventricular exclusion: 1.04 +/- 0.49 vs before Glenn palliation: 0.31 +/- 0.10, P = .01). The average pre-Glenn right ventricular/left ventricular ratio was substantially less than 1.0 (mean, 0.63 +/- 0.27), demonstrating right ventricular regression. The degree of left ventricular septal impingement decreased by an average of 38% (P = .008), normalizing left ventricular morphology. At the time of Glenn palliation, the left ventricular shortening fraction was normal in all patients (mean, 42% +/- 7%). CONCLUSIONS: After neonatal right ventricular exclusion (Starnes procedure) for severe Ebstein anomaly, there is a reduction in right ventricular size, as demonstrated by echocardiographic evidence of a significant decrease in Great Ormond Street ratios. This regression correlates with ventricular septal realignment and normalization of left ventricular function.

Stage I palliation for hypoplastic left heart syndrome: Norwood versus Sano modification.

PURPOSE OF REVIEW: Advancements in surgical technique and perioperative care have significantly improved the survival of infants born with hypoplastic left heart syndrome. A recent modification to the Norwood procedure is being adopted by many centers to improve postoperative hemodynamic stability and survival to stage II palliation. The late effects of this modification, however, are speculated and have not been investigated. RECENT FINDINGS: Center-specific improved short-term outcomes have been reported in a few small, nonrandomized studies of a new approach to the Norwood procedure, which utilizes a right ventricle to pulmonary artery shunt or Sano modification to provide pulmonary blood flow rather than the standard modified Blalock-Taussig shunt. SUMMARY: The classic Norwood procedure and Sano modification each have specific advantages and disadvantages in both the short and long term. Data comparing the two techniques are nonrandomized, contradictory, and utilize historical controls. The optimal shunt to improve survival to the second-stage palliation is unknown. A multicenter randomized clinical trial comparing the Sano with the modified Blalock-Taussig shunt in hypoplastic left heart syndrome or variants is currently in progress and should hopefully provide future guidelines for shunt selection based on clinical presentation.

Current surgical therapy for Ebstein anomaly in neonates.

OBJECTIVE: Neonates with profound heart failure resulting from Ebstein anomaly have historically had poor outcomes. We report our institutional experience with the surgical management of Ebstein anomaly in severely symptomatic neonates. METHODS: A retrospective review of all patients (n = 16) undergoing neonatal intervention for Ebstein anomaly between 1992 and 2005 has been carried out. The indications for operation were overt heart failure, cyanosis, and acidosis associated with tricuspid regurgitation, depressed right ventricular function, and severe cardiomegaly. The magnitude of cardiac enlargement was assessed by cardiothoracic ratio and Great Ormond Street ratio (area of right atrium + atrialized right ventricle/area of functional left atrium + left ventricle). The operative strategy was first to assess for the possibility of tricuspid valve repair with or without right ventricular outflow tract reconstruction. If this was not feasible, then right ventricular exclusion was performed by oversewing the tricuspid valve with a pericardial patch. A reduction atrioplasty was done and, depending on the extent of the atrialized portion of the right ventricle, plication was performed. A modified Blalock-Taussig shunt provided pulmonary blood flow. This univentricular approach (Starnes procedure) evolved to include a fenestration in the tricuspid valve patch to allow for right ventricular decompression. Analysis included overall and group-specific survival as well as the testing of perioperative clinical, morphologic, and surgical variables for correlation with mortality and morbidity. RESULTS: Mean age and weight at operation were 8 +/-10 days and 3.1 +/- 0.4 kg. Tricuspid valve repair was undertaken in 3 patients with 1 requiring conversion to right ventricular exclusion 3 months after the initial operation. In those with right ventricular exclusion, the tricuspid valve patch was fenestrated in 10 and nonfenestrated in 3. One patient had heart transplant as the initial procedure. There were 5 hospital deaths (31%) and no late deaths among the survivors. Survival in the cohort with a fenestrated tricuspid valve patch was 80% (8/10) versus 33% (1/3) for the nonfenestrated group. This difference did not reach statistical significance, although the trend seems clinically important. There was no difference in the cardiothoracic ratio (0.82 fenestrated vs 0.84 nonfenestrated: P = .802) or the Great Ormond Street ratio (1.2 fenestrated vs 1.02 nonfenestrated: P = .477) between the two groups. Among the 9 survivors of right ventricular exclusion, 3 have had completion of their Fontan, and all 9 have undergone a bidirectional Glenn procedure. All operations including homograft placement in the right ventricular outflow tract, whether during repair or during right ventricular exclusion, ended in death. CONCLUSION: Right ventricular exclusion with a fenestrated tricuspid valve patch combined with right atrioplasty and right ventriculoplasty and a Blalock-Taussig shunt (Starnes procedure) has provided effective palliation for neonates presenting with critical Ebstein anomaly and a tricuspid valve that cannot be repaired.