Concept of an expandable cardiac valve for surgical implantation in infants and children.

BACKGROUND: Options for cardiac valve replacement in children are limited to fixed-diameter prostheses that do not accommodate for somatic growth. An externally stented bovine jugular vein graft has been modified for surgical valve replacement in pediatric patients, with the intention of subsequent valve expansion in the catheterization laboratory as the child grows. METHODS: Pediatric patients at a single institution who underwent surgical implantation of an expandable bovine jugular vein valve between 2010 and 2014 were reviewed retrospectively. Forty-two patients underwent implantation at median age of 10 months (range, 3 weeks to 5.8 years) in aortic, mitral, pulmonary, or tricuspid positions. Numerous techniques for valve modification and implantation were used. RESULTS: The valve was competent with low gradient acutely postoperatively in all patients. Eight patients experienced central or paravalvular deterioration, and 7 required reoperation for valve-related adverse outcomes. Twenty patients underwent at least one previous valve repair or replacement. Twenty patients underwent 32 episodes of catheter-based balloon expansion of the valve, exhibiting a significant decrease in median gradient from 12 mm Hg to 8 mm Hg (P < .001) with no significant increase in grade of regurgitation. At 12 months after implantation, Kaplan-Meier analysis indicated that 88% would be expected to be free from reoperation (95% confidence interval, 78%-98%). A total of 6 deaths occurred, 3 before discharge and 3 late. CONCLUSIONS: A surgically implanted externally reinforced bovine jugular vein demonstrates acceptable short-term function and is amenable to catheter-based enlargement as the child grows. Modification of valve design and implantation techniques are necessary to reduce perivalvular complications.

Inadequate technical performance scores are associated with late mortality and late reintervention.

BACKGROUND: We have shown previously that technical performance score (TPS) is strongly associated with early mortality and major postoperative adverse events in a diverse group of patients. We now report evaluation of the validity of TPS in predicting late outcomes in the same group of patients. METHODS: Patients who underwent surgery between June 1, 2005 and June 30, 2006 were included. The TPS were assigned based on discharge echocardiograms and certain clinical criteria as previously described. Follow-up data for up to 4 years were retrospectively collected. Cox proportional hazards models were used for analysis. RESULTS: A total of 679 patients were included in the analysis. One hundred twenty-three (18%) were neonates, 213 (31%) infants, 291 (435) children, and 52 (8%) adults. Four hundred ninety-one (72%) were in low-risk adjustment in congenital heart surgery (RACHS; 1 to 3), 109 (16%) in high risk (4 to 6), and 27 (4%) were less than 18 years and could not be assigned a RACHS score. Three hundred thirty-one (48%) had an optimal TPS, 283 (42%) adequate, 61 (9%) inadequate, and 4 (1%) could not be scored. There were 34 (5%) late deaths and 149 (22%) late unplanned reinterventions. By univariate analysis, age, RACHS-1 categories, and TPS were all significantly associated with late reintervention (p < 0.001 for all), while TPS and RACHS-1 were significant factors for mortality (p < 0.001). On multivariable modeling, inadequate TPS was strongly associated with both late mortality (p = 0.001; HR [hazard ratio] 3.8, CI [confidence interval] 1.7 to 8.4) and late reintervention (p = 0.002, HR 2.1, CI 1.3 to 3.3) after controlling for RACHS-1 and age. CONCLUSIONS: The TPS has a strong association with late outcomes across a wide range of age and disease complexity and may serve as a tool to identify patients who are at a higher risk for late reintervention or mortality.

Biventricular conversion after single-ventricle palliation in unbalanced atrioventricular canal defects.

BACKGROUND: Management of unbalanced common atrioventricular canal (UCAVC) defect by a single-ventricle (SV) approach frequently results in poor outcomes, especially in trisomy 21 patients. In this report we describe our results with conversion to biventricular circulation in UCAVC patients with SV palliation. METHODS: Retrospective review of patients with UCAVC undergoing biventricular conversion from prior SV palliation between 2003 and 2011 was conducted. Mortality and freedom from reinterventions were analyzed using nonparametric methods. RESULTS: Sixteen children with UCAVC (8 patients [50%] were left dominant) and prior SV palliation underwent conversion to biventricular circulation between 2003 and 2011. Median follow-up was 18 months (range, 3 to 94 months). Surgical indications included worsening cyanosis, severe atrioventricular valve regurgitation, or failing bidirectional Glenn or Fontan physiology. All patients had either unequal distribution of the common atrioventricular valve of greater than 60% or one hypoplastic ventricle. By magnetic resonance imaging or computed tomography, 8 patients with right dominant atrioventricular canal had a median left ventricular end-diastolic volume of 32 mL/m(2) (range, 22 to 35 mL/m(2)). Eight patients with a left dominant atrioventricular canal had a median right ventricular end-diastolic volume of 42 mL/m(2) (range, 26 to 64 mL/m(2)). Eleven patients (69%) had trisomy 21, and 3 patients (19%) had heterotaxy. Stages of palliation included stage I in 2 patients, bidirectional Glenn in 10 patients, hemi-Fontan in 2 patients, and Fontan in 2 patients. There was 1 (6%) operative (right ventricle dominant) and 1 (6%) late death (left ventricle dominant). Eight patients required reinterventions, 3 (19%) surgical and 6 (38%) catheter-based. On follow-up, all had improvement in cyanosis and symptoms. CONCLUSIONS: Biventricular conversion from failing SV palliation in UCAVC can be accomplished with an acceptable early and late morbidity and mortality, although need for reintervention was not uncommon.