Mitral Valve Surgery in the First Year of Life.

Data are limited on outcomes associated with mitral valve surgery in infants. Prior studies report high mortality and increased risk for late cardiac failure particularly for those with mitral stenosis. We sought to evaluate outcomes in patients with mitral stenosis (MS) or regurgitation (MR) who had mitral valvuloplasty or replacement in the first year of life. A retrospective analysis of all patients in a single institution who underwent mitral valvuloplasty or replacement in their first year of life from 2004 to 2016 (n = 25), excluding patients with single ventricle pathology or those undergoing surgery for atrioventricular canal defect, was carried out. Median age and weight at surgery were 76.5 days (range 2-329) and 4.5 kg (range 3.0-10.1), respectively. The primary mitral pathology was MR in 16 and MS in 9 patients. Median follow-up among living patients was 4 years (range 106 days-12.3 years). Overall survival was 96% at 30 days and 87.8% at 1, 5, and 10 years. There were three early deaths (12%), all within 6 weeks of surgery. There were no late deaths. Three patients required valve replacement, 1 of which had a primary mitral valve replacement and died within 30 days of surgery. Re-intervention-free survival (surgical and catheter based) was 83.8%, 73.3%, and 48.9% at 1, 5, and 10 years per Kaplan-Meier estimates. There was no difference in re-intervention-free survival between patients with MR versus MS. No risk factors for death or re-intervention were identified. Mitral valvuloplasty and replacement can be performed in infants under 1 year of age with acceptable survival and need for re-intervention.

Dual-phase whole-heart imaging using image navigation in congenital heart disease.

BACKGROUND: Dual-phase 3-dimensional whole-heart acquisition allows simultaneous imaging during systole and diastole. Respiratory navigator gating and tracking of the diaphragm is used with limited accuracy. Prolonged scan time is common, and navigation often fails in patients with erratic breathing. Image-navigation (iNAV) tracks movement of the heart itself and is feasible in single phase whole heart imaging. To evaluate its diagnostic ability in congenital heart disease, we sought to apply iNAV to dual-phase sequencing. METHODS: Healthy volunteers and patients with congenital heart disease underwent dual-phase imaging using the conventional diaphragmatic-navigation (dNAV) and iNAV. Acquisition time was recorded and image quality assessed. Sharpness and length of the right coronary (RCA), left anterior descending (LAD), and circumflex (LCx) arteries were measured in both cardiac phases for both approaches. Qualitative and quantitative analyses were performed in a blinded and randomized fashion. RESULTS: In volunteers, there was no significant difference in vessel sharpness between approaches (p > 0.05). In patients, analysis showed equal vessel sharpness for LAD and RCA (p > 0.05). LCx sharpness was greater with dNAV (p < 0.05). Visualized length with iNAV was 0.5 ± 0.4 cm greater than that with dNAV for LCx in diastole (p < 0.05), 1.0 ± 0.3 cm greater than dNAV for LAD in diastole (p < 0.05), and 0.8 ± 0.7 cm greater than dNAV for RCA in systole (p < 0.05). Qualitative scores were similar between modalities (p = 0.71). Mean iNAV scan time was 5:18 ± 2:12 min shorter than mean dNAV scan time in volunteers (p = 0.0001) and 3:16 ± 1:12 min shorter in patients (p = 0.0001). CONCLUSIONS: Image quality of iNAV and dNAV was similar with better distal vessel visualization with iNAV. iNAV acquisition time was significantly shorter. Complete cardiac diagnosis was achieved. Shortened acquisition time will improve clinical applicability and patient comfort.

Use of a semi-automated cardiac segmentation tool improves reproducibility and speed of segmentation of contaminated right heart magnetic resonance angiography.

Three-dimensional printing has an increasing number of clinical applications in pediatric cardiology. Time required for dataset segmentation and conversion to stereolithography (STL) format remains a significant limitation. We investigated the impact of semi-automated cardiovascular-specific segmentation software on time and reproducibility of segmentation. Magnetic resonance angiograms (MRAs) of 19 patients undergoing intervention for right ventricular outflow lesions were segmented to demonstrate the right heart. STLs were created by two independent clinicians using semi-automated cardiovascular segmentation (SAS) and traditional manual segmentation (MS). Time was recorded and geometric STL disagreement was determined (0 % = no disagreement, 100 % = complete disagreement). MRA datasets were categorized as clean when only right heart structures were present in the MRA, or contaminated when left heart structures were also present and required removal. Eighteen (seven clean and 11 contaminated) cases were successfully segmented with both methods. Time to STL for clean datasets was faster with MS than SAS [median 209 s (IQR 192-252) vs. 296 s (272-317), p = 0.018] while contaminated datasets were faster with SAS [455 s (384-561) vs. 866 s (310-1429), p = 0.033]. Interobserver STL geometric disagreement was significantly lower using SAS than MS overall (0.70 ± 1.15 % vs. 1.31 ± 1.52 %, p = 0.030), and for the contaminated subset (0.81 ± 1.08 % vs. 1.75 ± 1.57 %, p = 0.036). Most geometric disagreement occurred at areas where left heart contamination was removed. Semi-automated segmentation was faster and more reproducible for contaminated datasets, while MS was faster but equally reproducible for clean datasets. Semi-automated segmentation methods are preferable for contaminated datasets and continued refinement of these tools should be supported.

High-sensitivity cardiac troponin I assay to screen for acute rejection in patients with heart transplant.

BACKGROUND: A noninvasive biomarker that could accurately diagnose acute rejection (AR) in heart transplant recipients could obviate the need for surveillance endomyocardial biopsies. We assessed the performance metrics of a novel high-sensitivity cardiac troponin I (cTnI) assay for this purpose. METHODS AND RESULTS: Stored serum samples were retrospectively matched to endomyocardial biopsies in 98 cardiac transplant recipients, who survived ≥3 months after transplant. AR was defined as International Society for Heart and Lung Transplantation grade 2R or higher cellular rejection, acellular rejection, or allograft dysfunction of uncertain pathogenesis, leading to treatment for presumed rejection. cTnI was measured with a high-sensitivity assay (Abbott Diagnostics, Abbott Park, IL). Cross-sectional analyses determined the association of cTnI concentrations with rejection and International Society for Heart and Lung Transplantation grade and the performance metrics of cTnI for the detection of AR. Among 98 subjects, 37% had ≥1 rejection episode. cTnI was measured in 418 serum samples, including 35 paired to a rejection episode. cTnI concentrations were significantly higher in rejection versus nonrejection samples (median, 57.1 versus 10.2 ng/L; P<0.0001) and increased in a graded manner with higher biopsy scores (P(trend)<0.0001). The c-statistic to discriminate AR was 0.82 (95% confidence interval, 0.76-0.88). Using a cut point of 15 ng/L, sensitivity was 94%, specificity 60%, positive predictive value 18%, and negative predictive value 99%. CONCLUSIONS: A high-sensitivity cTnI assay seems useful to rule out AR in cardiac transplant recipients. If validated in prospective studies, a strategy of serial monitoring with a high-sensitivity cTnI assay may offer a low-cost noninvasive strategy for rejection surveillance.

Use of a highly sensitive assay for cardiac troponin T and N-terminal pro-brain natriuretic peptide to diagnose acute rejection in pediatric cardiac transplant recipients.

BACKGROUND: Biomarkers have been proposed to augment or replace endomyocardial biopsy (EMB) to diagnose acute transplant rejection (AR). A new, highly sensitive assay for troponin T detects levels of cardiac troponin T (cTnT) 10- to 100-fold lower than standard assays but has not been investigated in transplant patients. N-terminal pro-brain natriuretic peptide (NT-proBNP) has not been evaluated in pediatric transplant patients. The purpose of this pilot study was to evaluate the association of cTnT and NT-proBNP with AR in pediatric cardiac transplant patients. METHODS: Plasma was obtained at the time of EMB from pediatric patients ≥ 1 year old. N-terminal pro-brain natriuretic peptide was measured in fresh plasma at the time of biopsy, and cTnT was measured from frozen, stored samples using the highly sensitive assay for troponin T. Biomarker data were correlated with EMB results. Cellular AR was defined as an International Society for Heart and Lung Transplantation biopsy score of grade ≥ 2R. RESULTS: Fifty-three blood samples were obtained from 42 patients (mean age 11 years). Seven episodes of AR occurred in 5 patients. Biopsies with vs without AR were associated with higher cTnT (median [interquartile range {IQR}] 66 [45-139] vs 7 [2-13] pg/mL, P = .001) and NT-proBNP (median [IQR] 11,169 [280-23,317] vs 334 [160-650] pg/mL, P < .01). After successful treatment of AR in 5 patients, cTnT fell markedly (median [IQR] 53.5 [44.8-66.5] to 10.7 [1.5-16.4], P = .05). CONCLUSION: In this pilot study, we found marked elevation of cTnT and NT-proBNP among children with AR. Moreover, reduction in cTnT levels after treatment paralleled improvement in EMB results. If these findings are confirmed in larger prospective studies, monitoring with these biomarkers may obviate surveillance EMB.