Predictors of Valve Failure Following Surgical Atrioventricular Valve Replacement with a Melody Valve in Infants and Children.

Options for atrioventricular (AV) valve replacement in small pediatric patients are very limited. The Melody valve has shown reasonable short-term outcomes. This study was aimed at identifying predictors of valve failure following AV valve replacement with a Melody valve at a single-center. 26 patients underwent 37 AV valve replacements with 31/37 (84%) of valves placed in the systemic AV valve position. Median age at procedure was 17 months (IQR 4-33) and weight was 8.5 kg (IQR 6.25-12.85). Median balloon size for valve implant was 20 mm (IQR 18-22). Repeat intervention occurred in 21 cases (57%) with repeat surgery in all but one. Median freedom from re-intervention was 31 months; 19% were free from re-intervention at 60 months. Age < 12 months weight < 10 kg and BSA < 0.4 m2 were all significant risk factors for early valve failure (p = 0.003, p 0.017, p 0.025, respectively). Valve longevity was greatest with balloon inflation to diameter 1.20-1.35 times the patient's expected annular diameter (Z0), relative to both smaller or larger balloons (p = 0.038). In patients less than 12 months of age, patients with single ventricle physiology had an increased risk of early valve failure (p = 0.004).

Treating a limb-threatening arterial clot in a newborn with catheter-directed thrombolysis.

Non-catheter related arterial thromboembolism in the neonatal population is rare and carries a significant risk of organ damage or limb loss. Thrombolysis, whether systemic or catheter- directed, is reserved either for limb or life-threatening thrombosis due to risk of bleeding especially in premature neonates. In this case, an infant male born at 34 weeks and 4 days gestational age presented with limb-threatening clot in the distal right subclavian artery and proximal right axillary artery with no known cause. After discussion of risks and benefits of various treatment options, he received thrombolysis treatment with low dose recombinant TPA via an umbilical artery catheter. There was complete resolution of the thrombus with this treatment and the patient had no significant bleeding while receiving treatment. Further investigation is needed to identify the patient population that will benefit from catheter-directed thrombolytic therapy and how to best monitor these patients.

Relationship Between Time to Left Atrial Decompression and Outcomes in Patients Receiving Venoarterial Extracorporeal Membrane Oxygenation Support: A Multicenter Pediatric Interventional Cardiology Early-Career Society Study.

OBJECTIVES: To assess the variation in timing of left atrial decompression and its association with clinical outcomes in pediatric patients supported with venoarterial extracorporeal membrane oxygenation across a multicenter cohort. DESIGN: Multicenter retrospective study. SETTING: Eleven pediatric hospitals within the United States. PATIENTS: Patients less than 18 years on venoarterial extracorporeal membrane oxygenation who underwent left atrial decompression from 2004 to 2016. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 137 patients (median age, 4.7 yr) were included. Cardiomyopathy was the most common diagnosis (47%). Cardiac arrest (39%) and low cardiac output (50%) were the most common extracorporeal membrane oxygenation indications. Median time to left atrial decompression was 6.2 hours (interquartile range, 3.8-17.2 hr) with the optimal cut-point of greater than or equal to 18 hours for late decompression determined by receiver operating characteristic curve. In univariate analysis, late decompression was associated with longer extracorporeal membrane oxygenation duration (median 8.5 vs 5 d; p = 0.02). In multivariable analysis taking into account clinical confounder and center effects, late decompression remained significantly associated with prolonged extracorporeal membrane oxygenation duration (adjusted odds ratio, 4.4; p = 0.002). Late decompression was also associated with longer duration of mechanical ventilation (adjusted odds ratio, 4.8; p = 0.002). Timing of decompression was not associated with in-hospital survival (p = 0.36) or overall survival (p = 0.42) with median follow-up of 3.2 years. CONCLUSIONS: In this multicenter study of pediatric patients receiving venoarterial extracorporeal membrane oxygenation, late left atrial decompression (≥ 18 hr) was associated with longer duration of extracorporeal membrane oxygenation support and mechanical ventilation. Although no survival benefit was demonstrated, the known morbidities associated with prolonged extracorporeal membrane oxygenation use may justify a recommendation for early left atrial decompression.

Traditional Long-Term Central Venous Catheters Versus Transhepatic Venous Catheters in Infants and Young Children.

OBJECTIVES: Children with congenital heart disease may require long-term central venous access for intensive care management; however, central venous access must also be preserved for future surgical and catheterization procedures. Transhepatic venous catheters may be an useful alternative. The objective of this study was to compare transhepatic venous catheters with traditional central venous catheters regarding complication rate and duration of catheter service. DESIGN: Retrospective review of 12 congenital heart disease patients from September 2013 to July 2015 who underwent placement of one or more transhepatic venous catheters. SETTING: Single freestanding pediatric hospital located in the central United States. PATIENTS: Pediatric patients with congenital heart disease who underwent placement of transhepatic venous catheter. INTERVENTIONS: Cohort's central venous catheter complication rates and duration of catheter service were compared with transhepatic venous catheter data. MEASUREMENTS AND MAIN RESULTS: Twelve patients had a total of 19 transhepatic venous lines. Transhepatic venous lines had a significantly longer duration of service than central venous lines (p = 0.001). No difference between the two groups was found in the number of documented thrombi, thrombolytic burden, or catheter sites requiring wound care consultation. A higher frequency of infection in transhepatic venous lines versus central venous lines was found, isolated to four transhepatic venous lines that had a total of nine infections. All but one was successfully managed without catheter removal. The difference in the proportion of infections to catheters in transhepatic venous lines versus central venous lines was significant (p = 0.0001), but no difference in the rate of infection-related catheter removal was found. CONCLUSIONS: Without compromising future central venous access sites, transhepatic venous lines had superior duration of service without increased thrombosis, thrombolytic use, or insertion site complications relative to central venous lines. Transhepatic venous catheters had a higher infection rate, and further investigation into the etiology is warranted.

Improving Outcomes for Infants with Single Ventricle Physiology through Standardized Feeding during the Interstage.

Congenital heart disease is identified as the most common birth defect with single ventricle physiology carrying the highest mortality. Staged surgical palliation is required for treatment, with mortality historically as high as 22% in the four- to six-month period from the first- to second-stage surgical palliation, known as the interstage. A standardized postoperative feeding approach was implemented through an evidence-based protocol, parent engagement, and interprofessional team rounds. Five infants with single ventricle physiology preprotocol were compared with five infants who received the standardized feeding approach. Mann-Whitney U tests were conducted to evaluate the hypotheses that infants in the intervention condition would consume more calories and have a positive change in weight-to-age z-score (WAZ) and shorter length of stay (LOS) following the first and second surgeries compared to infants in the control condition. After the protocol, the change in WAZ during the interstage increased by virtually one standard deviation from 0.05 to 0.91. Median LOS dropped 32% after the first surgery and 43% after the second surgery. Since first- and second-stage palliative surgeries occur within the same year of life, this represents savings of $500,000 to $800,000 per year in a 10-infant model. The standardized feeding approach improved growth in single ventricle infants while concurrently lowering hospital costs.

Total anomalous pulmonary venous connection: factors associated with mortality and recurrent pulmonary venous obstruction.

BACKGROUND: Surgical repair of total anomalous pulmonary venous connection (TAPVC) is associated with high rates of mortality and need for reintervention. The purpose of this study was to identify variables associated with surgical mortality and, in particular, to define predictors of recurrent pulmonary venous obstruction. METHODS: All patients who underwent surgical repair for TAPVC from 2005 to 2010 at a single institution were included in our analysis. Hospital course, operative data, and outpatient records were reviewed. RESULTS: Fifty-one patients were available for review and all were included in the analysis. Anatomic TAPVC subtypes included supracardiac 26 (51%), intracardiac 10 (19.6%), infracardiac 9 (17.6%), and mixed 6 (11.8%). Pulmonary venous obstruction was present at initial operation in 13 (25.5%) patients. Median age at repair was 18 days and median weight was 3.6 kg. Single-ventricle physiology was present in 9 (17.6%), with a diagnosis of heterotaxy syndrome in 7 (13.7%). There were 5 (9.8%) operative and 2 late deaths. Recurrent pulmonary venous obstruction requiring reintervention was found in 8 (15.7%) patients with median time to reintervention of 220 days. Obstructed TAPVC was found to be associated with surgical mortality (p=0.01). Cardiopulmonary bypass (p=0.02) and aortic cross-clamp times (p=0.03) were found to be associated with increased risk for reintervention. Intraoperative transesophageal echocardiography findings of a mean confluence gradient 2 mm Hg or greater was found to be markedly associated with recurrent pulmonary venous obstruction requiring reintervention (p≤0.001). CONCLUSIONS: Mortality after repair of TAPVC is highest in patients presenting with obstruction at time of repair. Longer cardiopulmonary bypass and cross-clamp times are associated with recurrent pulmonary venous obstruction requiring reintervention. The strongest association with need for reintervention was in patients with intraoperative transesophageal echocardiography Doppler evidence of pulmonary venous obstruction.

Cardiac surgery in infants with low birth weight is associated with increased mortality: analysis of the Society of Thoracic Surgeons Congenital Heart Database.

OBJECTIVE: The evaluation of operative mortality risk for cardiac surgery in infants with low weight is limited. To determine whether low weight is a risk factor for increased mortality, we reviewed the experience within the Society of Thoracic Surgeons Congenital Heart Surgery Database of infants who have undergone surgical correction or palliation for congenital heart disease. METHODS: We analyzed mortality in 3022 infants ages 0 to 90 days weighing 1 to 2.5 kg (n = 517) and greater than 2.5 to 4 kg (n = 2505) who underwent cardiac surgery from 2002 through 2004 at 32 participating centers. Patients were grouped according to the primary procedure performed and analyzed according to their weight at the time of surgical intervention. Patients were also analyzed according to Risk Adjustment for Congenital Heart Surgery-1 and Aristotle Basic Complexity scores. RESULTS: Compared with infants weighing 2.5 to 4 kg, infants weighing less than 2.5 kg had a significantly higher mortality for the following operations: repair of coarctation of the aorta, total anomalous pulmonary venous connection repair, arterial switch procedure, systemic to pulmonary artery shunt, and the Norwood procedure. Lower infant weight remained strongly associated with mortality risk after stratifying the population by Risk Adjustment for Congenital Heart Surgery-1 levels 2 through 6 and Aristotle Basic Complexity levels 2 through 4. CONCLUSIONS: Low weight at the time of surgical intervention is associated with increased mortality in patients undergoing several types of cardiovascular procedures. These data do not allow assessment of specific risks or benefits of any particular treatment strategy. However, they do support the need for prospective analysis of specific treatment strategies for these high-risk patients.

An infant with hypoplastic left heart syndrome and spinal muscular atrophy.

We report an infant with hypoplastic left heart syndrome consisting of mitral valvar atresia, aortic valvar atresia, hypoplasia of the aortic arch and coarctation of the aorta, who demonstrated respiratory failure and global hypotonia, and who was eventually diagnosed with spinal muscular atrophy.