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Background: Respiratory syncytial virus (RSV) causes significant morbidity and mortality in patients with a history of prematurity and congenital heart disease (CHD). In 2014, the guidelines for Palivizumab became more restrictive for this population. We hypothesized the percentage of RSV+ admissions would increase overall and in this target group (TG) specifically. Methods: We conducted a retrospective review of patients under age 2 years admitted with bronchiolitis two seasons prior to the change (Pre) and two seasons after (Post). Our TG included patients who were eligible prior to the 2014 changes but currently no longer eligible. We used chi-square analysis to answer the two main hypotheses: 1) Percent RSV+/total bronchiolitis Pre vs Post and 2) Percent of TG/RSV+ Pre vs Post. Results: 1283 patients (546 pre, 737 post) were admitted with the diagnosis of RSV between 2012-2016, 866 actually tested positive for RSV (367 Pre, 499 Post). There was no significant difference in the number of total patients admitted with RSV (Pre = 67.2%, Post = 67.7%) or in our TG (Pre 7.1% vs Post 8.2%). TG overall had a more complicated course: longer length of stay, median 5 days, IQR 2 - 12 vs 3 days, IQR 1 - 5, (p , but there was no difference Pre vs Post. Conclusion: The TG had an overall higher acuity, but there was no increase in the number of patients hospitalized with RSV or severity as a result of the Palivizumab guideline changes.

Background: We have previously shown that neonates in profound cardiogenic shock caused by a severe Ebstein anomaly can be successfully salvaged with fenestrated right ventricular exclusion and systemic to pulmonary shunt (modified Starnes procedure). The long-term outcome of single-ventricle management in these patients is not known. Methods: We retrospectively reviewed the records of patients who underwent neonatal Starnes procedure between 1989 and 2015. Patient demographics, clinical variables, and outcome data were collected. Results: Twenty-seven patients (13, 48% boys) underwent the Starnes procedure at 7 (5–9) days of life. All were intubated and on prostaglandin, 24 (89%) were inotrope dependent, and 22 (81%) had no antegrade flow from the right ventricle. Three patients underwent nonfenestrated right ventricular exclusion, 2 (67%) of whom died. Of the remaining 24, 3 (13%) died during the same hospitalization. The 22 neonatal survivors have been followed for 7 (6–8) years: 1 patient is awaiting a Glenn procedure; 1 died after undergoing a Glenn procedure; and the remaining 20 patients have successfully undergone Fontan completion. Their indexed pulmonary vascular resistance was 1.8 (1.2–2.3) W/m 2 , and mean pulmonary pressure was 12 (9–18) mm Hg. At last follow-up, 1 patient had died, and the remaining patients had normal left ventricular function, and all but 1 have New York Heart Association class I symptoms. Two patients have required pacemaker implantation, whereas the rest are in sinus rhythm. Survival for the entire cohort at 1, 5, and 10 years is 81±4%, 81±5%, and 76±3%, respectively, whereas for those with fenestrated right ventricular exclusion, survival at 1, 5, and 10 years is 87±2%, 87±2%, and 81±4%, respectively. Conclusions: Long-term single-ventricle outcomes among neonatal survivors of the modified Starnes procedure are excellent. There is reliable remodeling of the excluded right ventricle and good function of the left ventricle.

OBJECTIVE: Troponin-I (TN-I) levels are elevated following pediatric cardiac surgery with speculation that particular patterns may have prognostic significance. There is lack of procedure-specific data regarding postoperative TN-I levels in infants undergoing cardiac surgery. We hypothesized that TN-I elevation varies with type of surgery and persistent elevation predicts poor prognosis. METHODS: We prospectively measured serial TN-I levels (preoperatively, 4, 8, 12, 24, and 48 hours postoperatively) in 90 infants (age < 1 year) undergoing cardiac surgery: off cardiopulmonary bypass (CPB) (n = 15), on CPB (n = 43), and on CPB with ventricular incision (CPB with ventricular incision) (n = 32). RESULTS: All patients had undetectable baseline TN-I levels. The area under the curve (AUC) of TN-I levels over the 48-hour period was significantly different among the surgical groups (p < 0.002), and highest in patients with CPB with ventricular incision. Generally, TN-I levels peaked by 4 hours after surgery and returned to near-normal levels within 48 hours. A persistent TN-I rise beyond 8 hours after surgery was a strong predictor of postoperative hypoperfusion injury (defined as a composite endpoint of end-organ injury resulting from inadequate perfusion, OR 21.5; p = 0.001) and mortality (30% in those with persistently high TN-I, compared with 3.5% in the remaining patients; p < 0.001), independent of patient age, anatomy/complexity of surgery, and level of postoperative support. CONCLUSIONS: Our data provide benchmark values for TN-I levels following cardiac surgery in infants. Extent of TN-I elevation correlates with type of surgery. Persistent TN-I elevation beyond 8 hours after surgery is strongly associated with postoperative hypoperfusion injury and mortality.

We describe a case of anomalous left coronary artery from the pulmonary artery in association with total anomalous pulmonary venous return. The infant was diagnosed with total anomalous pulmonary venous return at 6 weeks of age and underwent successful surgical repair. On routine follow-up, he was found to have an anomalous left coronary artery from the pulmonary artery without evidence of mitral regurgitation or left ventricular dysfunction. The presence of the left-to-right shunt and secondary elevation in pulmonary artery pressures likely masked the usual findings associated with this coronary anomaly.

A clinician-driven home monitoring program can improve interstage outcomes in single-ventricle patients. Sociodemographic factors have been independently associated with mortality in interstage patients. We hypothesized that even in a population with high-risk sociodemographic characteristics, a home monitoring program is effective in reducing interstage mortality. We defined interstage period as the time period between discharge following Norwood palliation and second-stage surgery. We reviewed the charts of patients for the three-year period before (group 1) and after (group 2) implementation of the home monitoring program. Clinical variables around Norwood palliation, during the interstage period, and at the time of second-stage surgery were analyzed. There were 74 patients in group 1 and 52 in group 2. 59 % patients were Hispanic, and 84 % lived in neighborhoods where over 5 % families lived below poverty line. There was no significant difference in pre-Norwood variables, Norwood discharge variables, age at second surgery, or outcomes at second surgery. There were more Sano shunts performed at the Norwood procedure as the source of pulmonary blood flow in group 2 (p value

In this study, we present a case of prenatally diagnosed tetralogy of Fallot with a double aortic arch, correlating images from fetal echocardiography, transthoracic echocardiography, and cardiac MRI.

Background: We have previously shown that neonates in profound cardiogenic shock due to severe Ebstein’s anomaly can be successfully salvaged with fenestrated right ventricular (RV) exclusion and systemic to pulmonary shunt (modified Starnes procedure). The long-term outcome of single ventricle management in these patients is not known. Methods: We retrospectively reviewed the records of 26 patients who underwent neonatal Starnes procedure between 1989 and 2011. Patient demographics, clinical variables and outcome data were collected. Data is presented as mean ± standard errors or median (interquartile ranges). Results: 26 patients (12, 46% boys) underwent Starnes procedure at 7 (5-9) days of life. All were intubated and on prostacyclin infusion, 24 (92%) were inotrope-dependent and 23 (88%) had no antegrade flow from the RV. Two patients had had prior intervention (one tricuspid annuloplasty and one shunt alone). Three patients underwent non-fenestrated RV exclusion, two (67%) of whom died. Of the remaining 23, 3 (13%) died during the same hospitalization. The 21 neonatal survivors have been followed for 7 (6-8) years. One patient died after Glenn. The remaining 20 have successfully undergone Fontan completion with an indexed pulmonary resistance of 1.8 (1.2-2.3) W/m2 and mean pulmonary pressure of 12 (9-18) mm Hg. At last follow-up, all patients have normal left ventricular function, and all but one patient are in NYHA Class I symptoms. Two patients have required pacemaker implantation, while the rest are in sinus rhythm. Survival at 1, 5 and 10 years are 81±4%, 77±3% and 77±3%, respectively. Conclusion: Long-term single ventricle outcomes amongst neonatal survivors of modified Starnes procedure are excellent. There is reliable remodeling of the excluded RV and excellent function of the left ventricle.

Objectives Conventional prenatal screening for congenital heart disease (CHD) involves a time-consuming and highly operator-dependent acquisition of the four-chamber view and outflow tracts. By acquiring the entire fetal heart instantaneously as a single volume, real-time three-dimensional echocardiography (RT3DE) may facilitate fetal cardiac screening. Methods Four reviewers, each experienced with fetal cardiac imaging, blindly and independently evaluated a single cardiac volume from each of 18 fetuses (11 normal, seven with CHD). Two-dimensional echocardiography served as the gold standard. Three-dimensional evaluation of each fetus included a series of volume acquisitions lasting 2–6 s each. A ‘sweep volume’ technique was developed to fit larger hearts into a single non-gated volume. Results RT3DE had a high sensitivity for detecting CHD (93%), with only a single case being missed by two observers. Specificity for CHD was low (45%), with a high rate of ‘cannot determine’ responses and false positive artifacts. Conclusions These preliminary results suggest that RT3DE has the potential to function as a screening tool for fetal heart disease. However, artifacts must be recognized and minimized, resolution must improve, and substantial training will be necessary prior to widespread clinical use. Copyright © 2005 ISUOG. Published by John Wiley & Sons, Ltd.

Navigator coronary magnetic resonance imaging (MRI) was evaluated in assessing coronary artery origins in a pediatric and adolescent population. Sixty-five consecutive infants, children, or adolescents (age range 11 days to 21 years) were referred for MRI evaluations to assess coronary artery origins. Coronary artery origins were unambiguously delineated in 62 of 65 patients. In 3 patients, irregular arrhythmias precluded cardiac gating of the magnetic resonance acquisition. Two patients had anomalous coronary artery origins detected. Twenty-six patients required sedation for the studies. Free-breathing 3-dimensional MRI with real-time navigator correction is a robust method for delineating the coronary artery origins in pediatric and adolescent patients.

Background Patients undergoing operative repair of aortic obstruction are at a lifelong risk of recurrent obstruction, and there is controversy regarding the optimal surgical technique. We have used an alternative strategy for recurrent aortic obstruction, typically involving anatomic reconstruction by means of a median sternotomy, and describe our techniques and results. Methods Twenty-one patients presented with recurrent aortic arch obstruction. Mean age and weight were 7.8 ± 5.4 years (range, 0.21 to 15.2 years) and 30.6 ± 21.8 kg (range, 3.6 to 90 kg), respectively. Recurrence involved the aortic arch to some degree in each case, as the mean preoperative transverse aortic arch z score was −2.9 ± 1.6 (range, −7.0 to 0.1). Thoracotomy was possible in 2 patients, using re-resection with end-to-end anastomosis (n = 1) and patch aortoplasty (n = 1). The remaining 19 patients required median sternotomy, cardiopulmonary bypass, and deep hypothermic circulatory arrest for complete relief of obstruction by aortic arch advancement (n = 10), patch aortoplasty (n = 8), or interposition grafting (n = 1). Results There was 1 hospital death. Invasive blood pressure monitoring revealed no residual arm-to-leg gradient in 19 patients and a 20-mm Hg gradient in 2 patients. There have been no late deaths. No patients have undergone subsequent aortic intervention, and all are asymptomatic up to 85 months postoperatively. Two patients are currently followed with a 10-mm Hg arm-to-leg blood pressure gradient. Conclusions Anatomic reconstruction for recurrent aortic obstruction can be safely accomplished in the majority of patients. We favor median sternotomy because of the ability of establishing cardiopulmonary bypass, the facility of anatomic reconstruction techniques, and the ability to repair concomitant cardiovascular lesions.

Background It has previously been suggested that significant dilatation of the coronary sinus can contribute to left ventricular inflow obstruction and is amenable to surgical correction. The purpose of this study was to review our experience with this rare condition. Methods Since 1995, 6 patients have undergone coronary sinus reduction for concerns of obstruction with other concomitant intracardiac repairs. Preoperative echocardiography identified a significantly dilated left superior vena cava to the coronary sinus in 5 patients (83%) and an abnormal mitral valve in 4 patients (67%); these resulted in abnormal Doppler inflow patterns. Preoperative cardiac catheterization was performed in 5 patients and revealed increased atrial "a" waves, with a gradient to the left ventricular end-diastolic pressure in each case. At the time of surgery, coronary sinus angioplasty was performed in all patients. Results There were no deaths, and there was no major morbidity. Follow-up imaging revealed no significant left ventricular inflow obstruction in any patient. Conclusions We conclude that dilatation of the coronary sinus can become hemodynamically significant and that coronary sinus angioplasty is a safe and effective technique.

We report the success of transcatheter coil embolization of congenital coronary artery fistulas in infants and young children, primarily using a retrograde coaxial delivery system. Transcatheter coil embolization of coronary artery fistulas has been used successfully in the adult population with limited reports in the pediatric population, especially in infants. Ten patients underwent transcatheter coil embolization of congenital coronary artery fistulas between January 1993 and August 1999. The median age was 4.5 years (ranging from 5 weeks to 9.8 years). Coils were delivered exclusively using a retrograde arterial approach in 8 of 10 patients. A coaxial system was used in 8 patients. Angiography immediately after placement of coils revealed no residual shunt in any patient. Recent follow-up evaluation is available for 9 patients (median duration of follow-up, 7.9 months). Echocardiographic and clinical evaluations show complete occlusion in 8 of 9 patients. All patients are asymptomatic with normalization of chamber dimensions and coronary artery caliber. Transcatheter coil embolization of congenital coronary artery fistulas can be performed safely and successfully in infants and young children. The use of a retrograde approach and coaxial system is safe and effective and may provide better accuracy, stability and precision for placement of coils.

The term “ventricular septal defect” (VSD) denotes a gap or opening in the ventricular septum. There are several anatomically distinct types of VSDs, differing both in morphology as well as location within the ventricular septum. Transesophageal echocardiography (TEE) plays a very important role in the evaluation of VSDs, primarily in the intraoperative setting, as a method of monitoring surgical VSD closure. In addition, TEE also provides essential information and guidance during device closure of certain types of VSDs, either in the cardiac catheterization laboratory or via the perventricular approach. This chapter discusses the anatomy and morphology of the different types of VSDs, along with their echocardiographic evaluation by TEE.

Takayasu's arteritis is a granulomatous giant-cell arteritis of the aorta and its major branches. It primarily affects East Asian women in their second or third decade of life but is well known to affect all ethnicities across the world. Given its systemic nature, Takayasu's arteritis has multiorgan involvement, with the majority of disease morbidity related to the cardiovascular, central nervous, and renal systems. This report describes an unusual presentation of Takayasu's arteritis in a 2½-year-old boy. This is the first report in the literature describing the presence of both severe aortic regurgitation and diffuse coronary artery involvement in a patient so young.

We document the presentation profiles, treatment strategies, and clinical outcomes in a relatively large cohort of pediatric patients with intracardiac thrombi (ICT). We performed a retrospective review of patients diagnosed with ICT by echocardiography at a tertiary pediatric hospital during a 10-year period. These patients received medical therapy or thrombectomy. We provided echocardiographic descriptions of the ICT—size, chamber location, and mobility/morphology. The outcome measures were ICT (persistence, resolution, or embolization), effectiveness of therapy, and patient morbidity and mortality. There were 40 ICT diagnosed in 31 patients (22 males and 9 females). Mean age at diagnosis was 8.8 years (range, 15 days to 18 years). Overall mortality was 12/31 patients (39%); only one death was attributed to ICT embolization. Embolic events occurred in 4/31 patients (13%). The most common initial therapies included heparin infusion (n = 15), warfarin (n = 7), and aspirin (n = 7). The ICT resolved with medical therapy alone in 19/30 patients (63%). One patient required surgical thrombectomy. The cohort was divided into group 1 (dilated cardiomyopathy), group 2 (status post Fontan operation), and group 3 (other diagnoses). In group 1 (n = 11), there were 8 deaths. Embolization occurred in 2/5 large ICT, resulting in cerebral infarction and death (n = 1) and renal infarction (n = 1). The most common ICT location was the left ventricle (n = 10). Severe ventricular systolic dysfunction was present in 10/11 patients (91%). In group 2 (n = 9), there was 1 death. Embolization occurred in 1/7 large ICT, resulting in seizures and temporary paresis. All ICT were located in the Fontan pathway. Severe ventricular systolic dysfunction was present in 2/9 patients (22%). In group 3 (n = 11), there were 3 deaths. Embolization occurred in 1/9 small ICT, resulting in coronary emboli. ICT are most commonly diagnosed in pediatric patients with dilated cardiomyopathy or patients status post Fontan operation. The majority of ICT resolve with medical therapy. Larger ICT tend to embolize more frequently, and the morbidity secondary to embolizatioh is significant. Rarely is mortality due to ICT embolization. The prognosis is poor for patients with left ventricular ICT or ICT in the presence of ventricular systolic dysfunction.

Little is known about the characteristics and outcomes of fetuses with pericardial effusions (PEs); therefore, this study sought to identify factors associated with fetal PEs and the natural histories and outcomes of fetuses with PEs. Large PEs are associated with a greater likelihood of structural heart disease, impaired cardiac function, and chromosomal abnormalities, and PEs with hydrops or extracardiac malformations are associated with death. Most fetal PEs resolve, and fetuses with isolated PEs have a very good prognosis.

Congenital coronary arterial fistulas are rare anomalies that have traditionally been managed by surgical ligation. However, in recent years endovascular therapy has been employed with encouraging results. Between 1993 and 1996, we performed transcatheter coil embolization of coronary arterial fistulas to the right atrium or ventricle in four children ranging in age from 4.5 to 9.8 years. Cardiac and coronary arterial anatomy were diagnosed correctly on the preoperative echocardiogram in all patients, including the origin, course, and termination of the fistulas. The fistula was occluded completely in three of the patients, whereas trivial residual flow remained in the fourth. Transesophageal echocardiography was useful for monitoring the embolization procedure. In one of the patients, the fistula reopened while the child was on overnight heparin, although the magnitude of flow was less than that before the embolization. At follow-up ranging from 10 to 43 months, there was no flow through the fistula in any patient. We present our experience with these patients, with a focus on the importance of echocardiographic evaluation before, during, and after transcatheter therapy of coronary arterial fistulas.

artery (PA) arising from rudimentary pouch (*). Fetal echocardiography was performed at 19 and 29 weeks gestation after an abnormal screening ultrasound suggested left heart hypoplasia. The echocardiogram revealed left-sided hypoplasia with d-transposition of the great arteries and a small, posteriorly positioned pulmonary artery (PA) with both prograde and retrograde flow demonstrated. There appeared to be a moderate ventricular septal defect and, of note, there was unusual aneurysmal tissue of indeterminate origin below the pulmonary annulus. Postnatal echocardiography confirmed d-transposition of the great arteries with the aorta anterior and rightward of the smaller PA. The PA arose directly from a rudimentary, aneurysmal blind pouch (Figure 1) with bidirectional flow between the blind pouch and the main PA (Figure 2). However, no communication between the pouch and the remainder of the heart could be demonstrated by Doppler interrogation. The aneurysmal pouch was centrally located in the heart and appeared to possibly consist of redundant atrioventricular (AV) valve tissue contiguous with a leaflet of the left-sided AV valve. The PA was supplied by a large, tortuous patent ductus arteriosus typical for pulmonary atresia. There were 2 distinct AV valves entering a common single ventricle, with hypoplasia of the left-sided AV valve noted. The patient was initially treated with prostaglandin infusion to maintain ductal patency, followed by placement of a right-sided Blalock-Taussig shunt in the neonatal period. She subsequently underwent successful bidirectional Glenn anastomosis and left pulmonary arterioplasty at 7 months of age. This case represents an extremely unusual form of functional pulmonary atresia in which abnormal intracardiac aneurysmal tissue of indeterminate ori

OBJECTIVES We sought to determine if early primary repair of acyanotic tetralogy of Fallot (ToF) can be performed safely with low requirement for transannular patching (TAP) and thereafter allow normal right ventricular outflow tract (RVOT) growth. BACKGROUND Early primary repair of ToF normalizes intracardiac flow patterns, which may allow subsequent normal RVOT growth. Traditionally repair is deferred until symptoms occur or children are deemed of adequate size for operative risk to be acceptable because of a perceived increased requirement for TAP in small infants. METHODS Between July 1992 and March 1999, 42 acyanotic infants aged 4 to 87 days (median 62) and weight 2.6 to 6.6 kg (median 4.55) underwent complete repair of ToF. Pulmonary annulus measured 4 to 10.5 mm (median 6.5) with “z-value” of −5.6 to +3.0 (median −1.9). RVOT reconstruction was tailored to each patient; pulmonary valvotomy was performed in 26, main pulmonary arterioplasty in 22, and infundibular patching in 2. Only 10 (24%) required TAP. RESULTS Postoperative RVOT gradient was 0 to 30 mm Hg (median 10) and pRV/pLV ratio 0.3 to 0.6 (median 0.44). Pulmonary insufficiency was trivial/mild. There were no deaths. Junctional ectopic tachycardia developed in seven; only one required treatment. ICU stay was 2 to 14 days (median 4) and hospital stay 4 to 22 days (median 7). At follow-up 12 to 64 months later (median 38) there were no deaths. One child required reoperation for recurrent RVOT obstruction and two required balloon pulmonary arterioplasty. Follow-up RVOT gradient was 0 to 36 mm Hg (median 12), unchanged from early postoperative condition, and median z-value was −1.2 (−2.8 to +2.5); pulmonary insufficiency remained trivial/mild. CONCLUSIONS Complete repair of acyanotic ToF can be performed in early infancy with low morbidity and mortality and low requirement for TAP. Though results are not statistically significant, early repair may allow normal RVOT growth thereafter.