Limited utility of repeated vital sign monitoring during initiation of oral propranolol for complicated infantile hemangioma.

BACKGROUND: Initial propranolol recommendations for infantile hemangioma published in 2013 were intended as provisional best practices to be updated as evidence-based data emerged. METHODS: A retrospective multicenter study was performed to evaluate utility of prolonged monitoring after first propranolol dose and escalation(s). Inclusion criteria included diagnosis of hemangioma requiring propranolol of greater than or equal to 0.3 mg/kg per dose, younger than 2 years, and heart rate monitoring for greater than or equal to 1 hour. Data collected included demographics, dose, vital signs, and adverse events. RESULTS: A total of 783 subjects met inclusion criteria; median age at initiation was 112 days. None of the 1148 episodes of prolonged monitoring warranted immediate intervention or drug discontinuation. No symptomatic bradycardia or hypotension occurred during monitoring. Mean heart rate change from baseline to 1 hour was -8.19/min (±15.54/min) and baseline to 2 hours was -9.24/min (±15.84/min). Three preterm subjects had dose adjustments because of prescriber concerns about asymptomatic vital sign changes. No significant difference existed in pretreatment heart rate or in heart rate change between individuals with later adverse events during treatment and those without. CONCLUSION: Prolonged monitoring for initiation and escalation of oral propranolol rarely changed management and did not predict future adverse events. Few serious adverse events occurred during therapy; none were cardiovascular.

Patient-Specific Numerical Analysis of Coronary Flow in Children With Intramural Anomalous Aortic Origin of Coronary Arteries.

Unroofing surgery for anomalous aortic origin of a coronary artery (AAOCA) alters coronary anatomy by opening the intramural segment so that the anomalous coronary orifice arises perpendicularly from appropriate aortic sinus. Computational fluid dynamics modeling (CFD) allows for quantification of hemodynamics linked to morbidity such as wall shear stress (WSS), relative to patient-specific features like the angle of origin (AO). We hypothesize that CFD will reveal abnormal WSS indices in unroofed arteries that are related to AO. Six AAOCA patients (3 left, 3 right) status post unroofing (median = 13.5 years, range 9-17) underwent cardiac magnetic resonance imaging. CFD models were created from pre (n = 2) and postunroofing (n = 6) cardiac magnetic resonance imaging data, for the anomalous and contralateral normally-arising arteries. Downstream vasculature was represented by lumped parameter networks. Time-averaged WSS (TAWSS) and oscillatory shear index (OSI) were quantified relative to AO and measured hemodynamics. TAWSS was elevated along the outer wall of the normally-arising left vs right coronary arteries, as well as along unroofed left vs right coronary arteries (n = 6/group). No significant differences were noted when comparing unroofed and same-sided normally-arising coronaries. TAWSS was reduced after unroofing (eg, 276 ± 28 dyne/cm2 vs 91 ± 15 dyne/cm2; n = 2/group). Models with more acute preoperative AO indicated lower TAWSS at the proximity of ostium. Differences in OSI were not significant. Different flow patterns exist natively between right and left coronary arteries. Unroofing may normalize TAWSS but with variance related to the AO. This study suggests CFD may help stratify risk in AAOCA.

Evaluating the Safety of Oral Propranolol Therapy in Patients With PHACE Syndrome.

Importance: Oral propranolol is widely considered to be first-line therapy for complicated infantile hemangioma, but its use in patients with PHACE (posterior fossa malformations, hemangioma, arterial anomalies, cardiac defects, eye anomalies) syndrome has been debated owing to concerns that the cardiovascular effects of the drug may increase the risk for arterial ischemic stroke. Objective: To assess the incidence of adverse events among patients with PHACE syndrome receiving oral propranolol for infantile hemangioma. Design, Setting, and Participants: This multicenter retrospective cohort study assessed the incidence of adverse events among 76 patients with PHACE syndrome receiving oral propranolol for infantile hemangioma at 11 tertiary care, academic pediatric dermatology practices. Medical records from January 1, 2010, through April 25, 2017, were reviewed. Exposures: Patients received oral propranolol, 0.3 mg/kg/dose or more. Main Outcomes and Measures: The main outcome was the rate and severity of adverse events occurring throughout the course of treatment with oral propranolol, as documented in the medical records. Adverse events were graded from 1 to 5 using a scale derived from the Common Terminology Criteria for Adverse Events and were considered to be serious if they were grade 3 or higher. Results: A total of 76 patients (59 girls and 17 boys; median age at propranolol initiation, 56 days [range, 0-396 days]) met the inclusion criteria. There were no reports of serious adverse events (ie, stroke, transient ischemic attack, or cardiovascular events) during treatment with oral propranolol. A total of 46 nonserious adverse events were reported among 29 patients (38.2%); the most commonly reported nonserious adverse events were sleep disturbances and minor gastrointestinal tract and respiratory tract symptoms. In a comparison with 726 infants who received oral propranolol for hemangioma but did not meet criteria for PHACE syndrome, there was no significant difference in the rate of serious adverse events experienced during treatment (0 of 76 patients with PHACE syndrome and 3 of 726 patients without PHACE syndrome [0.4%]). Conclusions and Relevance: This study found that oral propranolol was used to treat infantile hemangioma in 76 patients with PHACE syndrome and that no serious adverse events were experienced. These data provide support for the safety of oral propranolol in this patient population.

Impact of Initial Shunt Type on Echocardiographic Indices in Children After Single Right Ventricle Palliations.

Background Heart size and function in children with single right ventricle (RV) anomalies may be influenced by shunt type at the Norwood procedure. We sought to identify shunt-related differences during early childhood after staged surgical palliations using echocardiography. Methods We compared echocardiographic indices of RV, neoaortic, and tricuspid valve size and function at 14 months, pre-Fontan, and 6 years in 241 subjects randomized to a Norwood procedure using either the modified Blalock-Taussig shunt or RV-to-pulmonary-artery shunt. Results At 6 years, the shunt groups did not differ significantly in any measure except for increased indexed neoaortic area in the modified Blalock-Taussig shunt. RV ejection fraction improved between pre-Fontan and 6 years in the RV-to-pulmonary artery shunt group but was stable in the modified Blalock-Taussig shunt group. For the entire cohort, RV diastolic and systolic size and functional indices were improved at 6 years compared with earlier measurements, and indexed tricuspid and neoaortic annular area decreased from 14 months to 6 years. The prevalence of ≥moderate tricuspid and neoaortic regurgitation was uncommon and did not vary by group or time period. Diminished RV ejection fraction at the 14-month study was predictive of late death/transplant; the hazard of late death/transplant when RV ejection fraction was <40% was tripled (hazard ratio, 3.18; 95% CI, 1.41-7.17). Conclusions By 6 years after staged palliation, shunt type has not impacted RV size and function, and RV and valvar size and function show beneficial remodeling. Poor RV systolic function at 14 months predicts worse late survival independent of the initial shunt type. Clinical Trial Registration URL: https://www.clinicaltrials.gov . Unique identifier: NCT00115934.

Hybrid Palliation as a Bridge to Biventricular Repair in Critical Aortic Stenosis With Coarctation.

Optimal management-balloon dilation versus surgical valvotomy-of neonatal critical aortic stenosis remains controversial. We describe a term neonate with critical aortic stenosis and coarctation, and severe left ventricular dysfunction with endocardial fibroelastosis who underwent palliation with surgical valvotomy and hybrid as a bridge to early biventricular repair. Initial repair consisted of commissurotomy to create bicuspid morphology with placement of bilateral pulmonary artery bands and maintenance of ductal patency with prostaglandin. Left ventricular function improved over the course of 2 weeks, allowing for repeat surgical valvotomy and coarctation repair. The patient was discharged 2 weeks postoperatively with low normal left ventricular function and mild aortic stenosis and regurgitation.

Clinical Practice Guideline for the Management of Infantile Hemangiomas.

Infantile hemangiomas (IHs) occur in as many as 5% of infants, making them the most common benign tumor of infancy. Most IHs are small, innocuous, self-resolving, and require no treatment. However, because of their size or location, a significant minority of IHs are potentially problematic. These include IHs that may cause permanent scarring and disfigurement (eg, facial IHs), hepatic or airway IHs, and IHs with the potential for functional impairment (eg, periorbital IHs), ulceration (that may cause pain or scarring), and associated underlying abnormalities (eg, intracranial and aortic arch vascular abnormalities accompanying a large facial IH). This clinical practice guideline for the management of IHs emphasizes several key concepts. It defines those IHs that are potentially higher risk and should prompt concern, and emphasizes increased vigilance, consideration of active treatment and, when appropriate, specialty consultation. It discusses the specific growth characteristics of IHs, that is, that the most rapid and significant growth occurs between 1 and 3 months of age and that growth is completed by 5 months of age in most cases. Because many IHs leave behind permanent skin changes, there is a window of opportunity to treat higher-risk IHs and optimize outcomes. Early intervention and/or referral (ideally by 1 month of age) is recommended for infants who have potentially problematic IHs. When systemic treatment is indicated, propranolol is the drug of choice at a dose of 2 to 3 mg/kg per day. Treatment typically is continued for at least 6 months and often is maintained until 12 months of age (occasionally longer). Topical timolol may be used to treat select small, thin, superficial IHs. Surgery and/or laser treatment are most useful for the treatment of residual skin changes after involution and, less commonly, may be considered earlier to treat some IHs.

The Impact of the Left Ventricle on Right Ventricular Function and Clinical Outcomes in Infants with Single-Right Ventricle Anomalies up to 14 Months of Age.

BACKGROUND: Children with single-right ventricle anomalies such as hypoplastic left heart syndrome (HLHS) have left ventricles of variable size and function. The impact of the left ventricle on the performance of the right ventricle and on survival remains unclear. The aim of this study was to identify whether left ventricular (LV) size and function influence right ventricular (RV) function and clinical outcome after staged palliation for single-right ventricle anomalies. METHODS: In the Single Ventricle Reconstruction trial, echocardiography-derived measures of LV size and function were compared with measures of RV systolic and diastolic function, tricuspid regurgitation, and outcomes (death and/or heart transplantation) at baseline (preoperatively), early after Norwood palliation, before stage 2 palliation, and at 14 months of age. RESULTS: Of the 522 subjects who met the study inclusion criteria, 381 (73%) had measurable left ventricles. The HLHS subtype of aortic atresia/mitral atresia was significantly less likely to have a measurable left ventricle (41%) compared with the other HLHS subtypes: aortic stenosis/mitral stenosis (100%), aortic atresia/mitral stenosis (96%), and those without HLHS (83%). RV end-diastolic and end-systolic volumes were significantly larger, while diastolic indices suggested better diastolic properties in those subjects with no left ventricles compared with those with measurable left ventricles. However, RV ejection fraction was not different on the basis of LV size and function after staged palliation. Moreover, there was no difference in transplantation-free survival to Norwood discharge, through the interstage period, or at 14 months of age between those subjects who had measurable left ventricles compared with those who did not. CONCLUSIONS: LV size varies by anatomic subtype in infants with single-right ventricle anomalies. Although indices of RV size and diastolic function were influenced by the presence of a left ventricle, there was no difference in RV systolic function or transplantation-free survival on the basis of LV measures.

Transplant-Free Survival and Interventions at 6 Years in the SVR Trial.

BACKGROUND: In the SVR trial (Single Ventricle Reconstruction), 1-year transplant-free survival was better for the Norwood procedure with right ventricle-to-pulmonary artery shunt (RVPAS) compared with a modified Blalock-Taussig shunt in patients with hypoplastic left heart and related syndromes. At 6 years, we compared transplant-free survival and other outcomes between the groups. METHODS: Medical history was collected annually using medical record review, telephone interviews, and the death index. The cohort included 549 patients randomized and treated in the SVR trial. RESULTS: Transplant-free survival for the RVPAS versus modified Blalock-Taussig shunt groups did not differ at 6 years (64% versus 59%, P=0.25) or with all available follow-up of 7.1±1.6 years (log-rank P=0.13). The RVPAS versus modified Blalock-Taussig shunt treatment effect had nonproportional hazards (P=0.009); the hazard ratio (HR) for death or transplant favored the RVPAS before stage II surgery (HR, 0.66; 95% confidence interval, 0.48-0.92). The effect of shunt type on death or transplant was not statistically significant between stage II to Fontan surgery (HR, 1.36; 95% confidence interval, 0.86-2.17; P=0.17) or after the Fontan procedure (HR, 0.76; 95% confidence interval, 0.33-1.74; P=0.52). By 6 years, patients with RVPAS had a higher incidence of catheter interventions (0.38 versus 0.23/patient-year, P<0.001), primarily because of more interventions between the stage II and Fontan procedures (HR, 1.72; 95% confidence interval, 1.00-3.03). Complications did not differ by shunt type; by 6 years, 1 in 5 patients had had a thrombotic event, and 1 in 6 had had seizures. CONCLUSIONS: By 6 years, the hazards of death or transplant and catheter interventions were not different between the RVPAS versus modified Blalock-Taussig shunt groups. Children assigned to the RVPAS group had 5% higher transplant-free survival, but the difference did not reach statistical significance, and they required more catheter interventions. Both treatment groups have accrued important complications. CLINICAL TRIAL REGISTRATION: URL: https://www.clinicaltrials.gov. Unique identifier: NCT00115934.

Surgical unroofing of intramural anomalous aortic origin of a coronary artery in pediatric patients: Single-center perspective.

BACKGROUND: Intramural anomalous aortic origin of a coronary artery (AAOCA) is associated with an increased risk of sudden cardiac death. This is amenable to surgical coronary unroofing, but outcomes studies are lacking. OBJECTIVE: To perform a comprehensive review of our institutional experience with pediatric patients with AAOCA who underwent surgical repair with unroofing of the intramural segment, focusing on preoperative and postoperative course and testing as well as intraoperative findings. METHODS: A retrospective cohort study was conducted to evaluate patients with AAOCA status post-coronary unroofing at Children's Hospital of Wisconsin. Data extraction included symptoms, preoperative and postoperative imaging and testing, surgical findings, and postoperative clinical course. RESULTS: From January 1999 to December 12, 2015, 63 patients underwent unroofing at a median age of 13 years (0.5-18 years). The majority underwent unroofing of an intramural right coronary (79%); 21% had an intramural left AAOCA. Symptoms suggestive of possible ischemia were present in about 50%. Additional structural cardiac anomalies were present in 33%. Transthoracic echocardiography was diagnostic in 60 of 63 (95%) and correlated with surgical findings in all cases. There was no surgical mortality associated with the unroofing, and no additional coronary reinterventions were performed. The median duration of postoperative follow-up was 3.1 years (7 days to 13.6 years). Symptoms either persisted or developed in 46% postoperatively. Postoperative exercise stress testing, stress echocardiography, and cardiac magnetic resonance imaging were performed in 76%, 8%, and 20%, respectively, of the cohort. None identified findings consistent with reversible coronary ischemia. Three patients had sudden cardiac arrest (1 death) after surgery without an identified residual coronary abnormality. CONCLUSIONS: Transthoracic echocardiography, with carefully designed coronary imaging protocols, can be diagnostic in accurately identifying intramural AAOCA in pediatric patients. Unroofing can be performed safely with no early morbidity, but symptoms can persist (including rare life-threatening events) without evidence of ischemia by postoperative provocative testing.

Anatomic Repair of Congenitally Corrected Transposition of the Great Arteries: Single-Center Intermediate-Term Experience.

We present our experience for patients who have undergone an anatomic repair (AR) for congenitally corrected transposition of the great arteries (CCTGA) at the Children's Hospital of Wisconsin. A retrospective chart review of patients who underwent AR for CCTGA from 2001 to 2015 was performed. The cohort consisted of 15 patients (74% male). Median age of anatomic repair was 15 months (range 4.5-45.6 months). Four patients had a bidirectional Glenn (BDG) prior to AR. At the time of AR,-9 (60%) underwent Senning/Rastelli procedure, 4 (26.6%) had double switch operation, and 2 (13.3%) underwent only Senning with VSD closure. Median duration of follow-up was 5.5 years (0.05-14 years). Reoperations prior to discharge included BDG, revision of pulmonary venous baffle, closure of residual VSD, and pacemaker placement. Late reoperations included left ventricular outflow tract obstruction repair, conduit replacement, melody valve placement, and pacemaker implantation. At their most recent follow-up, no patient had heart failure symptoms and only 1 had severely diminished function that improved with cardiac resynchronization therapy. Moderate mitral regurgitation was noted in 15% (2/13), and severe in 7% (1/13). Moderate tricuspid regurgitation was noted in 15% (2/13). One patient, 7% (1/13), developed moderate aortic insufficiency. There was a 100% survival at the time of the most recent follow-up. Patients with CCTGA who have undergone AR have excellent functional status and mid-term survival but reinterventions are common. Longer term studies are needed to determine both the extent and spectrum of reinterventions as well as long term survival.

The Infant with Aortic Arch Hypoplasia and Small Left Heart Structures: Echocardiographic Indices of Mitral and Aortic Hypoplasia Predicting Successful Biventricular Repair.

In infants with aortic arch hypoplasia and small left-sided cardiac structures, successful biventricular repair is dependent on the adequacy of the left-sided structures. Defining accurate thresholds of echocardiographic indices predictive of successful biventricular repair is paramount to achieving optimal outcomes. We sought to identify pre-operative echocardiographic indices of left heart size that predict intervention-free survival in infants with small left heart structures undergoing primary aortic arch repair to establish biventricular circulation (BVC). Infants ≤2 months undergoing aortic arch repair from 1999 to 2010 with aortic and/or mitral valve hypoplasia, (Z-score ≤-2) were included. Pre-operative and follow-up echocardiograms were reviewed. Primary outcome was successful biventricular circulation (BVC), defined as freedom from death, transplant, or single ventricular conversion at 1 year. Need for catheter based or surgical re-intervention (RI), valve annular growth, and significant late aortic or mitral valve obstruction were additional outcomes. Fifty one of 73 subjects (79%) had successful BVC and were free of RI at 1 year. Seven subjects failed BVC; four of those died. The overall 1 year survival for the cohort was 95%. Fifteen subjects underwent a RI but maintained BVC. In univariate analysis, larger transverse aorta (p = 0.006) and aortic valve (p = 0.02) predicted successful BVC without RI. In CART analysis, the combination of mitral valve (MV) to tricuspid valve (TV) ratio ≤0.66 with an aortic valve (AV) annulus Z-score ≤-3 had the greatest power to predict BVC failure (sensitivity 71%, specificity 94%). In those with successful BVC, the combination of both AV and MV Z-score ≤-2.5 increased the odds of RI (OR 3.8; CI 1.3-11.4). Follow-up of non-RI subjects revealed improvement in AV and MV Z-score (median AV annulus changed over time from -2.34 to 0.04 (p < 0.001) and MV changed from -2.88 to -1.41 (p < 0.001), but residual mitral valve stenosis and aortic arch obstruction were present in one-third of subjects. In this cohort of infants requiring initial aortic arch repair with concomitant small left heart structures, successful BVC can be predicted from combined echocardiographic indices. In this complex population, 1 year survival is high, but the need for RI and the presence of residual lesions are common.

Echocardiographic Diagnosis, Surgical Treatment, and Outcomes of Anomalous Left Coronary Artery from the Pulmonary Artery.

BACKGROUND: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare condition that can be difficult to diagnose by echocardiography alone. The purpose of this study was to describe the clinical and echocardiographic presentation of ALCAPA, create a set of critical echocardiographic diagnostic criteria, and report outcomes. METHODS: A retrospective review was conducted of all patients diagnosed with isolated ALCAPA at two major cardiac centers between 1990 and 2015. RESULTS: Of the 37 patients identified, only 54% presented in infancy. The anomalous coronary artery (CA) origin was clearly imaged in only 54% of echocardiographic examinations. However, other consistently identified echocardiographic markers were found, including left CA flow reversal (91%), collateral CA flow (85%), right CA dilation (81%), abnormal pulmonary artery flow (79%), mitral regurgitation (74%), left ventricular dysfunction (66%) and endocardial fibroelastosis (57%). Presenting echocardiograms had five of seven markers in 85% of patients. Left ventricular dysfunction was the most common marker in infants (89% vs 38%, P = .005); older children were more likely to have collateral formation visualized by color Doppler (100% vs 75%, P = .04). Following surgery, there were no early surgical deaths. The median follow-up duration was 10.3 years. At last follow-up, 92% had normal left ventricular function, 3% had moderate or worse mitral regurgitation, and 17% had required reintervention. CONCLUSIONS: Echocardiographic markers can reliably identify ALCAPA; these markers vary with the age of presentation. Surgical outcomes are excellent, and most patients will recover left ventricular and mitral valve function.

Pulmonary Vein Doppler Patterns in Infants with Single Right Ventricle Anomalies After Initial Staged Palliations.

The aim of this study was to describe serial changes in echocardiographic Doppler pulmonary vein flow (PVF) patterns in infants with single right ventricle (RV) anomalies enrolled in the Single Ventricle Reconstruction trial. Measurement of PVF peak systolic (S) and diastolic (D) velocities, velocity time integrals (VTI), S/D peak velocity and VTI ratios, and frequency of atrial reversal (Ar) waves were made at three postoperative time points in 261 infants: early post-Norwood, pre-stage II surgery, and 14 months. Indices were compared over time, between initial shunt type [modified Blalock-Taussig shunt (MBTS) and right ventricle-to-pulmonary artery shunt (RVPAS)] and in relation to clinical outcomes. S velocities and VTI increased over time while D wave was stable, resulting in increasing S/D peak velocity and VTI ratios, with a median post-Norwood S/D VTI ratio of 1.14 versus 1.38 at pre-stage II and 1.89 at 14 months (P < 0.0001 between intervals). MBTS subjects had significantly higher S/D peak velocity and VTI ratios compared to RVPAS at the post-Norwood and pre-stage II time points (P < 0.0001) but not by 14 months. PVF patterns did not correlate with survival or hospitalization course at 1 year. PVF patterns after Norwood palliation differ from normal infants by having a dominant systolic pattern throughout infancy. PVF differences based upon shunt type resolve by 14 months and did not correlate with clinical outcomes. This study describes normative values and variations in PVF for infants with a single RV from shunt-dependent pulmonary blood flow to cavopulmonary blood flow.

Surgical Valvotomy Versus Balloon Valvuloplasty for Congenital Aortic Valve Stenosis: A Systematic Review and Meta-Analysis.

BACKGROUND: Optimal initial treatment for congenital aortic valve stenosis in children remains unclear between balloon aortic valvuloplasty (BAV) and surgical aortic valvotomy (SAV). METHODS AND RESULTS: We performed a contemporary systematic review and meta-analysis to compare survival in children with congenital aortic valve stenosis. Secondary outcomes included frequency of at least moderate regurgitation at hospital discharge as well as rates of aortic valve replacement and reintervention. Single- and dual-arm studies were identified by a search of PubMed (Medline), Embase, and the Cochrane database. Overall 2368 patients from 20 studies were included in the analysis, including 1835 (77%) in the BAV group and 533 (23%) in the SAV group. There was no difference between SAV and BAV in hospital mortality (OR=0.98, 95% CI 0.5-2.0, P=0.27, I(2)=22%) or frequency of at least moderate aortic regurgitation at discharge (OR=0.58, 95% CI 0.3-1.3, P=0.09, I(2)=54%). Kaplan-Meier analysis showed no difference in long-term survival or freedom from aortic valve replacement but significantly more reintervention in the BAV group (10-year freedom from reintervention of 46% [95% CI 40-52] for BAV versus 73% [95% CI 68-77] for SAV, P<0.001). Results were unchanged in a sensitivity analysis restricted to infants (<1 year of age). CONCLUSIONS: Although higher rates of reintervention suggest improved outcomes with SAV, indications for reintervention may vary depending on initial intervention. When considering the benefits of a less-invasive approach, and clinical equipoise with respect to more clinically relevant outcomes, these findings support the need for a randomized controlled trial.

Adverse Events in Young and Preterm Infants Receiving Topical Timolol for Infantile Hemangioma.

BACKGROUND: The success of oral propranolol for treatment of infantile hemangiomas (IHs) has led practitioners to use topical ß-blockers. In preterm infants, clinicians frequently turn to topical timolol, with the presumption that topical application will result in less systemic absorption. We used Holter monitoring to assess for drug-induced bradycardia in high-risk infants. METHODS: We retrospectively reviewed the charts of 22 at-risk infants who received a Holter monitor to assess for association between timolol administration and development of significant bradycardia. RESULTS: Four infants had episodic bradycardia detected by Holter monitoring. Two of these infants were full term; weighed more than 3,000 g; and had rare, brief, asymptomatic episodes unrelated to the timing of the timolol application. The other two infants had symptomatic bradycardia while on timolol and were the only two babies that weighed less than 2,500 g at initiation of therapy. Both were young (postmenstrual age [PMA] 34 and 37 wks) at initiation and had a timolol dose above the average exposure for the cohort. CONCLUSION: In this cohort of at-risk infants, topical timolol appeared to provide safe treatment for IHs in full-term infants receiving a dose of less than 0.2 mg/kg/day, but infants with a PMA of less than 44 weeks and weight at treatment initiation of less than 2,500 g may be at risk of adverse events, including bradycardia, hypotension, apnea, and hypothermia. We recommend close monitoring of temperature, blood pressure, and heart rate in premature and low-birthweight infants with IHs at initiation of and during therapy with topical timolol.

Aortic arch repair in children with PHACE syndrome.

BACKGROUND: PHACE syndrome is characterized by infantile hemangioma and developmental abnormalities of the brain, arteries of head and neck, and aortic arch. METHODS: We retrospectively reviewed The PHACE Syndrome International Clinical Registry to identify children with PHACE who had operative repair of aortic arch obstruction at Children's Hospital of Wisconsin. RESULTS: Seven patients (median 11 months, range 1 week-6 years) with PHACE required aortic arch reconstruction from 1996 to 2015. All needed complex surgical approaches (4 conduit grafts, 2 patch aortoplasties, 1 subclavian flap) to relieve the obstruction because of long-segment transverse and proximal descending aortic arch dysplasia that included multiple areas of stricture with adjacent aneurysmal dilatation. Aberrant origin of a subclavian artery was found in 6 of 7. The 3 children who had surgery after age 1 showed significant progression of the arch obstruction and/or adjacent aneurysmal segment dilatation after their initial infant evaluation. No deaths or perioperative complications occurred despite associated cerebrovascular arterial dysplasia in 5 of 7. Recurrent arch obstruction developed in 3 of 7 at an intermediate follow-up interval of 6.2 years (2 had interposition graft replacement at 8 and 11 years due to somatic growth; 1 had repeat patch aortoplasty 11 months after initial repair secondary to recurrent stenosis). CONCLUSIONS: Extensive aortic arch reconstruction is commonly required in children with PHACE syndrome and coarctation due to the bizarre nature of the obstruction. Complete preoperative imaging is needed to fully characterize the aortic and cerebrovascular arterial anomalies. Recurrent obstruction is common given the non-native tissue techniques needed to relieve the arch anomaly.

Technical performance score is associated with outcomes after the Norwood procedure.

OBJECTIVES: The technical performance score (TPS) has been reported in a single center study to predict the outcomes after congenital cardiac surgery. We sought to determine the association of the TPS with outcomes in patients undergoing the Norwood procedure in the Single Ventricle Reconstruction trial. METHODS: We calculated the TPS (class 1, optimal; class 2, adequate; class 3, inadequate) according to the predischarge echocardiograms analyzed in a core laboratory and unplanned reinterventions that occurred before discharge from the Norwood hospitalization. Multivariable regression examined the association of the TPS with interval to first extubation, Norwood length of stay, death or transplantation, unplanned postdischarge reinterventions, and neurodevelopment at 14 months old. RESULTS: Of 549 patients undergoing a Norwood procedure, 356 (65%) had an echocardiogram adequate to assess atrial septal restriction or arch obstruction or an unplanned reintervention, enabling calculation of the TPS. On multivariable regression, adjusting for preoperative variables, a better TPS was an independent predictor of a shorter interval to first extubation (P=.019), better transplant-free survival before Norwood discharge (P<.001; odds ratio, 9.1 for inadequate vs optimal), shorter hospital length of stay (P<.001), fewer unplanned reinterventions between Norwood discharge and stage II (P=.004), and a higher Bayley II psychomotor development index at 14 months (P=.031). The TPS was not associated with transplant-free survival after Norwood discharge, unplanned reinterventions after stage II, or the Bayley II mental development index at 14 months. CONCLUSIONS: TPS is an independent predictor of important outcomes after Norwood and could serve as a tool for quality improvement.