Postoperative Serum Troponin Trends in Infants Undergoing Cardiac Surgery.

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. 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). All patients had undetectable baseline TN-I levels. The area under the curve 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, odds ratio 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 and/or complexity of surgery, and level of postoperative support. 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.

Anomalous left coronary artery from the pulmonary artery discovered following total anomalous pulmonary venous return repair: a rare entity.

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.

Single-Ventricle Outcomes After Neonatal Palliation of Severe Ebstein Anomaly With Modified Starnes Procedure.

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/m2, 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.

Home Monitoring Program Reduces Mortality in High-Risk Sociodemographic Single-Ventricle Patients.

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 <0.05). There were more unplanned hospital admissions and percutaneous re-interventions in group 2. Patients in group 2 whose admission criteria included desaturation had a 45 % likelihood of having an unplanned re-intervention. Group 2 noted an 80 % relative reduction in interstage mortality (p < 0.01). In a multiple regression analysis, after accounting for ethnicity, socio-economic status, and source of pulmonary blood flow, enrollment in a home monitoring program independently predicted improved interstage survival (p < 0.01). A clinician-driven home monitoring program reduces interstage mortality even when the majority of patients has high-risk sociodemographic characteristics.

Prenatal diagnosis of tetralogy of Fallot with a double aortic arch.

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.

Case Report of a 2-year-old boy with Takayasu's arteritis: an atypical, severe presentation of a rare disease.

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.

Hypoplastic left heart syndrome in patients with Kabuki syndrome.

The association of cardiac defects with Kabuki syndrome has been well described. The majority of these defects are isolated shunt lesions, conotruncal abnormalities, or various forms of arch obstruction. This report describes a series of three patients with hypoplastic left heart syndrome and Kabuki syndrome. The series illustrates the full spectrum of left-sided obstructive lesions and expands the phenotype of cardiac defects associated with Kabuki syndrome.

Characteristics and outcomes of fetuses with pericardial effusions.

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.

Usefulness of real-time navigator magnetic resonance imaging for evaluating coronary artery origins in pediatric patients.

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.

Anatomic reconstruction for recurrent aortic obstruction in infants and children.

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.

Left ventricular inflow obstruction associated with persistent left superior vena cava and dilated coronary sinus.

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.

Retrograde transcatheter coil embolization of congenital coronary artery fistulas in infants and young children.

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.