Echocardiographic Screening for Postoperative Pericardial Effusion in Children.

Pericardial effusion (PE) after cardiac surgery can be life threatening without timely detection, and the optimal screening method is unknown. We sought to evaluate the role of a surveillance echocardiogram on postoperative day 10 (± 2), determine the incidence of postoperative PE, and identify risk factors. We conducted a retrospective cohort study including all pediatric patients who underwent open heart surgery at a single institution over a 7-month period. To identify risk factors for PE, medical records of patients with PE detected within 6 weeks after surgery (cases) were compared with patients without PE (controls). Of 203 patients, 52 (26%) had PE within 6 weeks; 42 (81%) were trivial-small and 10 (19%) were moderate-large. Twenty-nine (56%) were first detected within 7 days post-operatively, including all cases developing cardiac tamponade (n = 3). An echocardiogram was done 10 (± 2) days post-operatively in 41/52 cases, of which 12/41 (29%) did not have a PE at this time, 24/41 (59%) had a trivial-small PE, and 5/41(12%) had a moderate-large PE; 2 of the latter had no prior detected PE. Closure of an atrial septal defect had the highest incidence of PE (42%). PE cases were associated with postoperative nasopharyngeal detection of a respiratory virus (OR 3.8, p = 0.03). In conclusion, the majority of PE cases were detected within 7 days post-operatively, including all cases subsequently developing cardiac tamponade. Day 10 echocardiography infrequently detected a moderate or large effusion that had previously gone undiagnosed. A positive perioperative nasopharyngeal aspirate for a respiratory virus was associated with postoperative PE.

Percutaneous obliteration of left ventricular cavity to eliminate aortic regurgitation and presumed coronary steal in an infant with hypoplastic left heart syndrome.

The phenomenon of coronary steal is well known in the setting of HLHS (Hypoplastic Left Heart Syndrome) early after the Classical Norwood Operation. We report a rare case of an infant with HLHS [Severe Aortic Stenosis (AS), Mitral Stenosis (MS) and small Left Ventricle (LV)], who developed aortic regurgitation and presumed coronary steal late after the Sano Modification of the Norwood Procedure. Coronary steal developed secondary to progressive aortic and mitral regurgitation and resulted in poor right ventricular function and severe tricuspid regurgitation. We describe a novel interventional approach for obliteration of the LV cavity by using hydrogel coils. LV obliteration eliminated the presumed steal and resulted in improvement in ventricular function, tricuspid regurgitation and clinical course.

Aortico-left ventricular tunnel: the elusive diagnosis.

We present a case of an infant prenatally diagnosed with bilateral outflow-tract obstruction and severe aortic regurgitation who underwent cardiac transplantation at 45 days of life. Aortico-left ventricular tunnel was subsequently diagnosed on pathologic examination of the explant heart. Aortico-left ventricular tunnel is a rare congenital cardiac malformation and can remain undiagnosed if the clinician has a low level of suspicion. Aortico-left ventricular tunnel should be considered in any fetus or newborn with aortic regurgitation.

Heterotaxy syndrome: is a prophylactic Ladd procedure necessary in asymptomatic patients?

Heterotaxy syndrome (HS) is a complex disorder involving thoracic and abdominal asymmetries. Congenital heart disease is often accompanied by an intestinal rotation abnormality (IRA) that may predispose to bowel ischemia and infarction. There is controversy in the literature whether asymptomatic infants with HS must be screened for IRA and, if present, whether a prophylactic Ladd procedure should be performed. We performed a retrospective chart review of all patients who underwent a Ladd procedure from January 2007 to December 2010 at Stollery Children's Hospital, Edmonton, Canada. Twenty-nine patients underwent a Ladd procedure, 22 without HS but with symptomatic malrotation and 7 with HS and asymptomatic malrotation. Asymptomatic HS patients had a complication rate of 57 % after a prophylactic Ladd procedure compared with a complication rate of 9 % in the symptomatic non-HS population. The management of asymptomatic IRA in patients with HS remains controversial. We suggest that HS patients be screened for IRA and that asymptomatic patients be managed conservatively.

Innominate vein vascular ring provides novel insight into systemic venous embryogenesis.

Anomalies of the innominate vein are uncommon in congenital cardiac disease. We report a case of duplicate innominate veins forming a vascular ring encircling the ascending aorta. We postulate that this vascular ring represents the failure of both a dorsal and ventral precardinal anastomosis to regress.

Adherence of families to a group a streptococcal pharyngitis protocol used in a pediatric emergency department.

OBJECTIVES: In an effort to limit inappropriate antibiotic use for children with pharyngitis, our pediatric emergency department (PED) has implemented a strep throat protocol using preprinted prescriptions given to families pending pharyngeal swab results. We sought to determine the rate of adherence of families managed with this protocol and to identify whether clinical features are associated with adherence. METHODS: We conducted a prospective cohort study of children aged 2 to 17 years presented to the PED with suspected group A streptococcal (GAS) pharyngitis. Prescription-filling activity was tracked using a novel carbon-copy prescription and compared with throat swab result. Health records were reviewed for demographic and clinical information. Adherence was defined as prescriptions being filled after notification of a positive swab result and prescriptions not being filled when throat swab result was negative. RESULTS: Three hundred nine children were screened for GAS pharyngitis. One hundred swabs (32.4%) were positive for GAS pharyngitis, of which 15 prescriptions were filled before swab results. No record of prescription filling was found for 37 of the children with positive swab results. Only 9 families (4.3%) filled the prescription when the swab result was negative. Overall, 247 families (80.2%) were adherent to the protocol. Families of children aged 2 to 5 years were more likely to be adherent than those aged 13 to 17 years (odds ratio, 3.5; 95% confidence interval, 1.15-10.66). CONCLUSIONS: Most families are adherent to our GAS pharyngitis protocol. Very few families filled prescriptions when the swab result was negative. Age was the only factor influencing adherence. Our current GAS pharyngitis protocol is an effective management strategy for children presenting with pharyngitis to the PED.

Effect of Balloon:Annulus Ratio on Incidence of Pulmonary Insufficiency Following Valvuloplasty.

OBJECTIVE: Pulmonary insufficiency following balloon valvuloplasty for pulmonary valve stenosis has been shown to result in right ventricular dilation, which may necessitate pulmonary valve replacement. This study investigates the consequences of employing a more conservative balloon:annulus ratio of ≤1.2 against the currently used ratios of >1.2. The aim of the study was to evaluate whether a more conservative balloon:annulus ratio would maintain procedural success with adequate relief of the pulmonary valve gradient, while minimizing pulmonic insufficiency. DESIGN: Procedural data were collected on cases considered for balloon valvuloplasty for congenital pulmonic stenosis at Rady Children's Hospital, San Diego between March 31, 2003 and April 9, 2014. The study includes 98 patients whose median age at the time of procedure was 3.6 months (range: 1 day to 271 months); median follow-up was 15.2 months (range 24 days to 106 months). The subjects were divided into two groups based on their balloon:annulus ratio: ≤1.2 (n = 67) and >1.2 (n = 31). RESULTS: The difference in pulmonary gradient reduction-the marker of procedural success-was not significant between the two groups (P = .33). Although both groups had an increase in pulmonary valve insufficiency from baseline, the ≤1.2 group had significantly less insufficiency compared to the >1.2 group (P = .008). Reintervention rates were not statistically significant between both groups (P = .89). CONCLUSION: Performing pulmonary balloon valvuloplasty with a balloon:annulus ratio of ≤1.2 produces significantly less pulmonary insufficiency than a ratio of >1.2 without reducing procedure efficacy.

Right ventricular dysfunction in children supported with pulsatile ventricular assist devices.

OBJECTIVES: To describe the incidence and severity of right ventricular dysfunction (RVD) in pediatric ventricular assist device (VAD) recipients and to identify the preoperative characteristics associated with RVD and their effect on outcomes. METHODS: Children bridged to transplantation from 2004 to 2011 were included. RVD was defined as the use of a left VAD (LVAD) with an elevated central venous pressure of >16 mm Hg with inotropic therapy and/or inhaled nitric oxide for >96 hours or biventricular assist (BiVAD). RESULTS: A total of 57 children (median age, 2.97 years; range 35 days to 15.8 years) were supported. Of the 57, 43 (75%) had an LVAD, and of those, 10 developed RVD. The remaining 14 (25%) required BiVAD. Thus, RVD occurred in 24 of 57 patients (42%). Preoperative variables such as younger age (P = .01), use of extracorporeal mechanical support (P = .006), and elevated urea (P = .03), creatinine (P = .02), and bilirubin (P = .001) were associated with RVD. Multiple logistic regression analysis indicated that elevated urea and extracorporeal mechanical support (odds ratio, 26.4; 95% confidence interval, 2.3-307.3; and odds ratio, 27.8; 95% confidence interval, 2.5-312.3, respectively) were risk factors for BiVAD. The patients who developed RVD on LVAD had a complicated postoperative course but excellent survival (100%), comparable to those with preserved right ventricular function (91%). The survival for those requiring BiVAD was reduced (71%). CONCLUSIONS: RVD occurred in approximately 40% of pediatric VAD recipients and affects their peri-implantation morbidity and bridging outcomes. Preoperative extracorporeal membrane oxygenation and elevated urea were risk factors for BiVAD. Additional studies of the management of RVD in children after VAD implantation are warranted.

Heterotaxy syndrome and intestinal rotation abnormalities: a survey of institutional practice.

PURPOSE: Abnormalities of intestinal rotation (IRA) are commonly associated with heterotaxy syndrome (HS). There is controversy whether asymptomatic infants with HS require screening for IRA and if present, whether a prophylactic Ladd procedure is indicated. The objective of this study is to determine institutional practice across North America in the management of asymptomatic infants with HS and IRA. METHODS: We performed an international, multi-institutional web based survey to examine current practice and opinions in the management of IRA in HS patients. RESULTS: Overall response rate was 30%. Of physicians surveyed, 84% believe that HS patients should be screened for IRA in the neonatal period. 61% of general surgeons, 50% of cardiovascular surgeons and 45% of cardiologists feel that all patients with HS and an asymptomatic IRA should have a prophylactic Ladd procedure. 55% of physicians stated they would be comfortable with conservative management for patients with HS and asymptomatic IRA. CONCLUSIONS: The risk of midgut volvulus, morbidity and mortality from elective procedures and cardiovascular prognosis must be considered prior to an elective Ladd procedure on asymptomatic HS patients. There are practice variance among sub-specialists caring for these patients, a lack of expert consensus, and a paucity of evidence-based data for IRA in this population.