Noncontrast free-breathing ECG-gated 3D balanced steady-state free precession in congenital heart disease and aortopathy evaluation.

BACKGROUND: High-fidelity cardiac magnetic resonance (MR) imaging plays a pivotal role in the surveillance of congenital heart disease (CHD) and aortopathy. OBJECTIVE: We aimed to evaluate the quality and accuracy of free breathing, ECG-gated noncontrast three-dimensional (3D) balanced steady-state free precession (bSSFP) in cases of CHDs and aortopathies using contrast-enhanced 3D bSSFP as a reference. We also used one of our routinely used non-ECG-gated 2D-single-shot (SSh) bSSFP sequence as an adjunct to noncontrast 3D bSSFP. MATERIALS AND METHODS: Institutional review board approval was obtained to perform a systematic retrospective analysis of image quality and vascular measurements. Patients with CHD and aortopathy, who were undergoing clinically indicated contrast-enhanced 3D bSSFP, were prospectively identified to also undergo additional noncontrast 3D bSSFP and 2D SSh bSSFP imaging as part of a clinical quality improvement initiative aimed at reducing the use of contrast when feasible. Two readers, blinded to each other's evaluations, graded image quality on a 5-point Likert scale and performed vascular measurements in separate sessions for both 3D bSSFP images. They also reported the visibility of various mediastinal great vessels on 2D SSh bSSFP images. Raw agreement, the weighted kappa statistic, and intra-class correlation coefficients (ICCs) were computed to assess the consistency and agreement between the two readers. Comparative analysis of noncontrast and contrast-enhanced 3D bSSFP imaging was performed in adult and pediatric patients using a two-sided paired t-test and Bland-Altman analysis. A P-value < 0.05 was considered significant for all inference testing. RESULTS: A total of 29 patients (17 males, median age 20.3 years, interquartile range (IQR) 12.5, age range 7-39 years), including 11 pediatric patients under the age of 18 years (6 males, median age 14.5 years, IQR 4.0, age range 7-17 years), underwent retrospective analysis. The overall image quality score for contrast-enhanced 3D bSSFP was significantly higher (P < 0.0001) than that of noncontrast 3D bSSFP for both all subjects (4.4 ± 0.2, range 4.0-4.9 vs 3.7 ± 0.4, range 3.1-4.7) and only pediatric subjects (4.3 ± 0.3, range 4.0-4.9 vs 3.6 ± 0.5, range 3.1-4.4). By combining noncontrast 3D bSSFP and 2D bSSFP, reader 1 and reader 2 rated 423 and 420 vessels diagnostic, respectively, in a total of 435 vessel segments. All landmarks showed similar mean vessel diameters without significant differences between noncontrast and contrast-enhanced 3D bSSFP MR angiography (r = 0.99, bias -0.31 mm, 95% limits of agreement -2.04 mm to 1.43 mm). CONCLUSIONS: Although contrast-enhanced images had better overall image quality, an imaging protocol consisting of noncontrast 2D SSh bSSFP and 3D bSSFP whole-chest images provides diagnostically adequate image quality, and accurate vascular measurements, comparable to free-breathing contrast-enhanced 3D bSSFP in both children and adults with CHD and aortopathies.

(F)utility of preoperative pulmonary function testing in pectus excavatum to assess severity.

PURPOSE: The utility of pulmonary function testing (PFT) in pectus excavatum (PE) has been subject to debate. Although some evidence shows improvement from preoperative to postoperative values, the clinical significance is uncertain. A high failure-to-completion rate for operative PFT (48%) was identified in our large institutional cohort. With such a high non-completion rate, we questioned the overall utility of PFT in the preoperative assessment of PE and sought to evaluate if other measures of PE severity or cardiopulmonary function could explain this finding. METHODS: Demographics, clinical findings, and results from cardiac MRI, PFT (spirometry and plethysmography), and cardiopulmonary exercise tests (CPET) were reviewed in 270 patients with PE evaluated preoperatively between 2015 and 2018. Regression modeling was used to measure associations between PFT completion and cardiopulmonary function. RESULTS: There were no differences in demographics, symptoms, connective tissue disorders, or multiple indices of pectus severity and cardiac deformation in PFT completers versus non-completers. While regression analysis revealed higher RVEF, LVEF, and LVEF-Z scores, lower RV-ESV/BSA, LV-ESV/BSA, and LV-ESV/BSA-Z scores, and abnormal breathing reserve in PFT completers vs. non-completers, these findings were not consistent across continuous and binary analyses. CONCLUSIONS: We found that PFT completers were not significantly different from non-completers in most structural and functional measures of pectus deformity and cardiopulmonary function. Inability to complete PFT is not an indicator of pectus severity.

Deep Learning for Automated Measurement of Total Cardiac Volume for Heart Transplantation Size Matching.

Total Cardiac Volume (TCV)-based size matching using Computed Tomography (CT) is a novel technique to compare donor and recipient heart size in pediatric heart transplant that may increase overall utilization of available grafts. TCV requires manual segmentation, which limits its widespread use due to time and specialized software and training needed for segmentation. This study aims to determine the accuracy of a Deep Learning (DL) approach using 3-dimensional Convolutional Neural Networks (3D-CNN) to calculate TCV, with the clinical aim of enabling fast and accurate TCV use at all transplant centers. Ground truth TCV was segmented on CT scans of subjects aged 0-30 years, identified retrospectively. Ground truth segmentation masks were used to train and test a custom 3D-CNN model consisting of a DenseNet architecture in combination with residual blocks of ResNet architecture. The model was trained on a cohort of 270 subjects and a validation cohort of 44 subjects (36 normal, 8 heart disease retained for model testing). The average Dice similarity coefficient of the validation cohort was 0.94 ± 0.03 (range 0.84-0.97). The mean absolute percent error of TCV estimation was 5.5%. There is no significant association between model accuracy and subject age, weight, or height. DL-TCV was on average more accurate for normal hearts than those listed for transplant (mean absolute percent error 4.5 ± 3.9 vs. 10.5 ± 8.5, p = 0.08). A deep learning-based 3D-CNN model can provide accurate automatic measurement of TCV from CT images. This initial study is limited as a single-center study, though future multicenter studies may enable generalizable and more accurate TCV measurement by inclusion of more diverse cardiac pathology and increasing the training data.

Novel Techniques in Imaging Congenital Heart Disease: JACC Scientific Statement.

Recent years have witnessed exponential growth in cardiac imaging technologies, allowing better visualization of complex cardiac anatomy and improved assessment of physiology. These advances have become increasingly important as more complex surgical and catheter-based procedures are evolving to address the needs of a growing congenital heart disease population. This state-of-the-art review presents advances in echocardiography, cardiac magnetic resonance, cardiac computed tomography, invasive angiography, 3-dimensional modeling, and digital twin technology. The paper also highlights the integration of artificial intelligence with imaging technology. While some techniques are in their infancy and need further refinement, others have found their way into clinical workflow at well-resourced centers. Studies to evaluate the clinical value and cost-effectiveness of these techniques are needed. For techniques that enhance the value of care for congenital heart disease patients, resources will need to be allocated for education and training to promote widespread implementation.

Isolated mitral valve endocarditis: Patient, disease, and surgical factors that influence outcomes.

OBJECTIVE: The objectives of this study were to investigate patient characteristics, valve pathology, bacteriology, and surgical techniques related to outcome of patients who underwent surgery for isolated native (NVE) or prosthetic (PVE) mitral valve endocarditis. METHODS: From January 2002 to January 2020, 447 isolated mitral endocarditis operations were performed, 326 for NVE and 121 for PVE. Multivariable analysis of time-related outcomes used random forest machine learning. RESULTS: Staphylococcus aureus was the most common causative organism. Of 326 patients with NVE, 88 (27%) underwent standard mitral valve repair, 43 (13%) extended repair, and 195 (60%) valve replacement. Compared with NVE with standard repair, patients who underwent all other operations were older, had more comorbidities, worse cardiac function, and more invasive disease. Hospital mortality was 3.8% (n = 17); 0 (0%) after standard valve repair, 3 (7.0%) after extended repair, 8 (4.1%) after NVE replacement, and 6 (5.0%) after PVE re-replacement. Survival at 1, 5, and 10 years was 91%, 75%, and 62% after any repair and 86%, 62%, and 44% after replacement, respectively. The most important risk factor for mortality was renal failure. Risk-adjusted outcomes, including survival, were similar in all groups. Unadjusted extended repair outcomes, particularly early, were similar or worse than replacement in terms of reinfection, reintervention, regurgitation, gradient, and survival. CONCLUSIONS: A patient- and pathology-tailored approach to surgery for isolated mitral valve endocarditis has low mortality and excellent results. Apparent superiority of standard valve repair is related to patient characteristics and pathology. Renal failure is the most powerful risk factor. In case of extensive destruction, extended repair shows no benefit over replacement.

Development and User Testing of a Dynamic Tool for Rheumatic Heart Disease Management.

OBJECTIVE: Most rheumatic heart disease (RHD) registries are static and centralized, collecting epidemiological and clinical data without providing tools to improve care. We developed a dynamic cloud-based RHD case management application with the goal of improving care for patients with RHD in Uganda. METHODS: The Active Community Case Management Tool (ACT) was designed to improve community-based case management for chronic disease, with RHD as the first test case. Global and local partner consultation informed selection of critical data fields and prioritization of application functionality. Multiple stages of review and revision culminated in user testing of the application at the Uganda Heart Institute. RESULTS: Global and local partners provided feedback of the application via survey and interview. The application was well received, and top considerations included avenues to import existing patient data, considering a minimum data entry form, and performing a situation assessment to tailor ACT to the health system setup for each new country. Test users completed a postuse survey. Responses were favorable regarding ease of use, desire to use the application in regular practice, and ability of the application to improve RHD care in Uganda. Concerns included appropriate technical skills and supports and potential disruption of workflow. CONCLUSION: Creating the ACT application was a dynamic process, incorporating iterative feedback from local and global partners. Results of the user testing will help refine and optimize the application. The ACT application showed potential for utility and integration into existing care models in Uganda.

Tolerability and Acceptability of Autonomous Immersive Virtual Reality Incorporating Digital Twin Technology for Mass Training in Healthcare.

INTRODUCTION: As part of onboarding and systems testing for a clinical expansion, immersive virtual reality (VR) incorporating digital twin technology was used. While digital twin technology has been leveraged by industry, its use in health care has been limited with no prior application for onboarding or training. The tolerability and acceptability of immersive VR for use by a large population of healthcare staff were unknown. METHODS: A prospective, observational study of an autonomous immersive VR onboarding experience to a new clinical space was conducted from May to September 2021. Participants were healthcare staff from several critical care and acute care units. Primary outcomes were tolerance and acceptability measured by reported adverse effects and degree of immersion. Secondary outcomes were attitudes toward the efficacy of VR compared with standard onboarding experiences. RESULTS: A total of 1522 healthcare staff participated. Rates of adverse effects were low and those with prior VR experience were more likely to report no adverse effects. Odds of reporting immersion were high across all demographic groups, though decreased with increasing age. The preference for VR over low-fidelity methods was high across all demographics; however, preferences were mixed when compared with traditional simulation and real-time clinical care. CONCLUSIONS: Large-scale VR onboarding is feasible, tolerable, and acceptable to a diverse population of healthcare staff when using digital twin technology. This study also represents the largest VR onboarding experience to date and may address preconceived notions that VR-based training in health care is not ready for widespread adoption.

Improved donor lung size matching by estimation of lung volumes based on chest X-ray measurements.

RATIONALE: Organ size matching is an important determinant of successful allocation and outcomes in lung transplantation. While computed tomography (CT) is the gold standard, it is rarely used in an organ-donor context, and chest X-ray (CXR) may offer a practical and accurate solution in estimating lung volumes for donor and recipient size matching. We compared CXR lung measurements to CT-measured lung volumes and traditional estimates of lung volume in the same subjects. METHODS: Our retrospective study analyzed clinically obtained CXR and CT lung images of 250 subjects without evidence of lung disease (mean age 9.9 ± 7.8 years; 129 M/121F). From CT, each lung was semi-automatically segmented and total lung volumes were quantified. From anterior-posterior CXR view, each lung was manually segmented and areas were measured. Lung lengths from the apices to the mid-basal regions of each lung were measured from CXR. Quantified CT lung volumes were compared to the corresponding CXR lung lengths, CXR lung areas, height, weight, and predicted total lung capacity (pTLC). RESULTS: There are strong and significant correlations between CT volumes and CXR lung areas in the right lung (R2 = .89, p < .0001), left lung (R2 = .87, p < .0001), and combined lungs (R2 = .89, p < .0001). Similar correlations were seen between CT volumes and CXR measured lung lengths in the right lung (R2 = .79, p < .0001) and left lung (R2 = .81, p < .0001). This correlation between anatomical lung volume (CT) and CXR was stronger than lung-volume correlation to height (R2 = .66, p < .0001), weight (R2 = .43, p < .0001), or pTLC (R2 = .66, p < .0001). CONCLUSION: CXR measures correlate much more strongly with true lung volumes than height, weight, or pTLC. The ability to obtain efficient and more accurate lung volume via CXR has the potential to change our current listing practices of using height as a surrogate for lung size, with a case example provided.

Hand-Constructed CorMatrix Tubular Valve Used for Tricuspid Valve Replacement in Neonate With Congenital Tricuspid Dysplasia: Five-Year Follow-Up.

Tricuspid valve (TV) dysplasia is a rare congenital defect that, in severe cases, can cause heart failure necessitating surgical correction. TV replacement options are limited in neonates since few commercial valves are available and the rates of failure and postoperative mortality are high. The authors report a neonate with TV dysplasia who underwent replacement with a hand-constructed tube valve using small intestinal submucosal extracellular matrix, which functioned well for >5 years.

Impact of Size Matching on Survival Post Heart Transplant in Infants: Estimated Total Cardiac Volume Ratio Outperforms Donor-Recipient Weight Ratio.

PURPOSE: Cardiac volume-based estimation offers an alternative to donor-recipient weight ratio (DRWR) in pediatric heart transplantation (HT), but has not been correlated to post-transplant outcomes We sought to determine whether estimated Total Cardiac Volume (eTCV) ratio is associated with HT survival in infants. METHODS: The UNOS database was used to identify infants (age:<1year) who received HT in 1987-2020. Donor and recipient eTCV were calculated from weight using previously published data. Patient cohort was divided according to the significant range of eTCV ratio; characteristics and survival were compared. RESULTS: 2845 infants were identified. Hazard ratio with cubic spline showed prognostic relationship of eTCV ratio and DRWR with the overall survival. The cut-point method determined an optimal eTCV ratio range predictive of infant survival was 1.05-1.85 whereas no range for DRWR was predictive. 75.6% patients had an optimal TCV ratio, while 18.1% were in the lower (LR) and 6.3% in the higher (HR) group. Kaplan-Meier analysis showed better survival for patients within the optimal vs LR (p=0.0017), and a similar significantly better survival when compared to HR (p=0.0053). The optimal eTCV ratio group (n=2151) had DRWR ranging from 1.09-5; 34.3% had DRWR 2-3, and 5.0% DRWR>3. CONCLUSION: Currently, an upper DRWR limit has not been established in infants. Therefore, determining the optimal eTCV range is important to identifying an upper limit that significantly predicts survival benefit. This finding suggests a potential increase in donor pool for infant recipients since over 40% of donors in the optimal eTCV range includes DRWR values>2 that are traditionally not considered for candidate listing.

Are Parent Discharge Readiness Scores Effective for Patients With Congenital Heart Disease After Cardiac Surgery?

OBJECTIVE: To assess discharge readiness and clinical engagement post-discharge in families of children undergoing congenital heart surgery. STUDY DESIGN: This prospective cross-sectional study was performed at a major tertiary pediatric cardiac referral center. Eligible parents and caregivers completed a discharge readiness tool, the Readiness for Hospital Discharge Scale for Parents of Hospitalized Children, via online survey on the day of discharge. Clinical engagement data included subsequent phone calls, clinic visits, emergency department visits, and hospital readmissions. Readiness for Hospital Discharge Scale for Parents of Hospitalized Children scores were measured as follows: very high (9-10), high (8-8.9), moderate (7-7.9), and low (<7). Descriptive statistics were used to describe demographic data. RESULTS: In total, 128 families enrolled between April and December 2021. Parent discharge readiness scores ranged from "high" to "very high." Families with lower socioeconomic status and younger patients (especially single-ventricle infants or "interstage") had a greater proportion of clinic visits, emergency department visits, and hospital readmissions within 30-days postdischarge compared with other groups. CONCLUSIONS: Discharge readiness scores were not associated with clinical engagement. We identified vulnerable populations as evidenced by a greater frequency of clinical engagement in the immediate postoperative period, particularly younger patients and first-time surgeries. Although these visits may be appropriate, novel programs could enhance education and emotional support to prevent delay in seeking care or creating excessive stress and anxiety after discharge.

Female donor hearts can improve survival for male pediatric heart transplant recipients.

BACKGROUND: Both gender- and weight-matching between donor and recipient are thought to impact survival in pediatric heart transplantation, with clinical dogma holding that male donor hearts and "ideal" weight-matching yield superior survival. The composite impacts of gender and weight on post-transplant survival (PTS) are understudied. METHODS: All pediatric (age <18) heart recipients between 1989 and 2021 with the complete recipient and donor gender and weight data were identified in the United Network for Organ Sharing database. Patients were grouped by recipient-donor gender (M & F) and donor-to-recipient weight ratio (DRWR; undersized [<0.8], ideal-sized [0.8-1.5], oversized [>1.5]). RESULTS: A total of 10 697 patients were identified. Among male recipients, PTS was greatest with oversized DRWR from either male or female donors (median 22.4 and 20.6 years; p < .001 vs. others) and lowest for undersized DRWR from either male or female donors (median 13.4 and 13.2 years; p < .001 vs. others). The majority (64%) of male recipients received ideal-sized DRWR, among which female donor hearts yielded superior survival to males (median 18.9 vs. 17.4 years, p = .014). No differences in PTS existed for female recipients on the basis of gender-match, DRWR, and gender/DRWR together (all p > .1). CONCLUSIONS: When considered together, gender and DRWR pairings impact PTS in male-but not female-pediatric heart transplant recipients. For males receiving ideal-sized DRWR organs (most common pairing, >60%), male recipients achieve superior survival when female donor hearts are transplanted. These findings suggest that if weight is being used for size-matching, donor gender should also be considered, particularly for male recipients.

Echocardiography Provides a Reliable Estimate of Total Cardiac Volume for Pediatric Heart Transplantation.

BACKGROUND: Donor-to-recipient size matching for heart transplantation typically involves comparing donor and recipient body weight; however, weight is not linearly related to cardiac size. Attention has shifted toward the use of computed tomography- (CT-) derived total cardiac volume (TCV), that is, CT-TCV, to compare donor and recipient heart organ size. At this time, TCV size matching is near impossible for most centers due to logistical limitations. To overcome this impediment, echocardiogram-derived TCV (ECHO-TCV) is an attractive, alternative option to estimate CT-TCV. The goal of this study is to test whether ECHO-TCV is an accurate and reliable surrogate for TCV measurement compared with the gold standard CT-TCV. METHODS: ECHO-TCV and CT-TCV were measured in a cohort spanning the neonatal to young adult age range with the intention to simulate the pediatric heart transplant donor pool. ECHO-TCV was measured using a modified Simpson's summation-of-discs method from the apical 4-chamber (A4C) view. The gold standard of CT-TCV was measured from CT scans using three-dimensional reconstruction software. The relationship between ECHO-TCV and CT-TCV was evaluated and compared with other anthropometric and image-based markers that may predict CT-TCV. Inter-rater reliability of ECHO-TCV was tested among 4 independent observers. Subanalyses were performed to identify imaging views and timing that enable greater accuracy of ECHO-TCV. RESULTS: Banked imaging data of 136 subjects with both echocardiogram and CT were identified. ECHO-TCV demonstrated a linear relationship to CT-TCV with a Pearson correlation coefficient of r = 0.96 (95% CI, 0.95-0.97; P < .0001) and mean absolute percent error of 8.6%. ECHO-TCV correlated most strongly with CT-TCV in the subset of subjects <4 years of age (n = 33; r = 0.98; 95% CI, 0.96-0.99; P < .0001). The single-score intraclass correlation coefficient across all 4 raters is 0.96 (interquartile range, 0.93-0.98). ECHO-TCV measured from a standard A4C view at end diastole with the atria in the plane of view had the strongest correlation to CT-TCV. CONCLUSIONS: ECHO-TCV by the A4C view was found to be both an accurate and reliable alternative measurement of CT-TCV and is derived from readily available donor ECHO images. The ECHO-TCV findings in this study make the ECHO method an attractive means of direct donor-to-recipient TCV size matching in pediatric heart transplantation.

Deep Learning for Automated Measurement of Total Cardiac Volume for Heart Transplantation Size Matching.

Background: Total Cardiac Volume (TCV) based size matching using Computed Tomography (CT) is a novel technique to compare donor and recipient heart size in pediatric heart transplant that may increase overall utilization of available grafts. TCV requires manual segmentation, which limits its widespread use due to time and specialized software and training needed for segmentation. Objective: This study aims to determine the accuracy of a Deep Learning (DL) approach using 3-dimensional Convolutional Neural Networks (3D-CNN) to calculate TCV, with the clinical aim of enabling fast and accurate TCV use at all transplant centers. Materials and Methods: Ground truth TCV was segmented on CT scans of subjects aged 0-30 years, identified retrospectively. Ground truth segmentation masks were used to train and test a custom 3D-CNN model consisting of a Dense-Net architecture in combination with residual blocks of ResNet architecture. Results: The model was trained on a cohort of 270 subjects and a validation cohort of 44 subjects (36 normal, 8 heart disease retained for model testing). The average Dice similarity coefficient of the validation cohort was 0.94 ± 0.03 (range 0.84-0.97). The mean absolute percent error of TCV estimation was 5.5%. There is no significant association between model accuracy and subject age, weight, or height. DL-TCV was on average more accurate for normal hearts than those listed for transplant (mean absolute percent error 4.5 ± 3.9 vs. 10.5 ± 8.5, p = 0.08). Conclusion: A deep learning based 3D-CNN model can provide accurate automatic measurement of TCV from CT images.

Does age matter? Cardiopulmonary profiles of adolescents versus adults with pectus excavatum presenting for surgical evaluation.

PURPOSE: We sought to analyze differences in presentation and cardiopulmonary function between those referred for surgical consultation as adolescents (11-17 years) versus adults (18 + years). METHODS: Presenting symptoms, past medical history, and results from cardiac MRI (CMR), pulmonary function testing (PFT), and cardiopulmonary exercise testing (CPET) were reviewed in 329 patients evaluated preoperatively between 2015 and 2018. Adjusted regression modeling was used to measure associations between pectus indices and clinical endpoints of cardiopulmonary function. RESULTS: Our sample included 276 adolescents and 53 adults. Adults presented more frequently with chest pain (57% vs. 38%, p = 0.01), shortness of breath (76% vs. 59%, p = 0.02), palpitations (21% vs. 11%, p = 0.04), and exercise intolerance (76% vs. 59%, p = 0.02). Their Haller indices (5.2 [4.2, 7.0] vs. 4.7 [4.0, 5.7], p = 0.05) and cardiac asymmetry (1.8 [0.5] vs. 1.6 [0.5], p = 0.02) were also higher. In continuous outcome analysis, adolescents had higher FEV1/FVC on PFT and higher work on CPET (p < 0.01). CONCLUSIONS: Adults with pectus excavatum were more symptomatic than adolescents with deeper, more asymmetric deformities, decreased FEV1/FVC and exercise capacity. These findings may support earlier versus later repair to prevent age-related decline. Further studies are warranted.

Reoperation after early and late failure of mitral valve repair for degenerative disease.

OBJECTIVES: To determine mechanisms of early and late failure after mitral valve repair for degenerative disease, identify factors associated with re-repair, and evaluate durability of re-repair. METHODS: From January 2008 to July 2020, 330 reoperations were performed for recurrent mitral valve dysfunction after initial valve repair for degenerative disease. Mechanisms of repair failure were determined by review of preoperative imaging and operative reports. Multivariable analysis was performed to identify factors associated with likelihood of re-repair or replacement. Durability of re-repair was assessed using longitudinal analysis of postoperative echo data. RESULTS: Eighty-five of 330 (26%) reoperations were performed for early repair failure within 1 year and 245/330 (74%) for late failure thereafter. Suture/annuloplasty dehiscence, systolic anterior motion, hemolysis, and ventricular remodeling were more common mechanisms of early failure and disease progression and fibrosis late failure. Forty percent (34/85) of early failures were re-repaired versus 24% (59/245) of late failures. Re-repair was more common than replacement in recent years and was associated with earlier reoperation (median 1.5 vs 3.9 years; P = .0001). No in-hospital deaths occurred after re-repair; 2 patients (0.8%) died after valve replacement. Freedom from severe mitral regurgitation after re-repair was 93% at 7 years. CONCLUSIONS: Mitral valve re-repair can be performed with low rates of mortality and morbidity for early and late valve dysfunction. Mechanisms of early repair failure differ from those of late failure and are generally more amenable to re-repair. In selected patients who present after failed repair, we prefer mitral re-repair to valve replacement whenever technically feasible.

Comparing donor and recipient total cardiac volume predicts risk of short-term adverse outcomes following heart transplantation.

INTRODUCTION: In pediatric heart transplantation, donor: recipient weight ratio (DRWR) has long been the sole metric for size matching. Total cardiac volume (TCV)-based size matching has emerged as a novel method to precisely identify an upper limit of donor organ size of a heart transplant recipient while minimizing the risk of complications from oversizing. The clinical adoption of donor: recipient volume ratio (DRVR) to prevent short-term adverse outcomes of oversizing is unknown. The purpose of this single-center study is to determine the relationship of DRWR and DRVR to the risk of post-operative complications from allograft oversizing. METHODS: Recipient TCV was measured from imaging studies and donor TCV was calculated from published TCV prediction models. DRVR was defined as donor TCV divided by recipient TCV. The primary outcome was short-term post-transplant complications (SPTC), a composite outcome of delayed chest closure and prolonged intubation > 7 days. A multivariable logistic regression model of DRWR (cubic spline), DRVR (linear) and linear interaction between DRWR and DRVR was used to examine the probability of experiencing a SPTC over follow-up as a function of DRWR and DRVR. RESULTS: A total of 106 transplant patients' records were reviewed. Risk of the SPTC increased as DRVR increased. Both low and high DRWR was associated with the SPTC. A logistic regression model including DRWR and DRVR predicted SPTC with an AUROC curve of 0.74. [95% CI 0.62 0.85]. The predictive model identified a "low-risk zone" of donor-recipient size match between a weight ratio of 0.8 and 2.0 and a TCV ratio less than 1.0. CONCLUSION: DRVR in combination with DRWR predicts short-term post-transplant adverse events. Accepting donors with high DRWR may be safely performed when DRVR is considered.

Quantitative cardiopulmonary magnetic resonance imaging in neonatal congenital diaphragmatic hernia.

BACKGROUND: Pulmonary arterial hypertension, impaired cardiac function and lung hypoplasia are common in infants with congenital diaphragmatic hernia (CDH) and are associated with increased morbidity and mortality. Robust noninvasive methods to quantify these abnormalities in early infancy are lacking. OBJECTIVE: To determine the feasibility of MRI to quantify cardiopulmonary hemodynamics and function in infants with CDH and to investigate left-right blood flow and lung volume discrepancies. MATERIALS AND METHODS: We conducted a prospective MRI study of 23 neonates (isolated left CDH: 4 pre-repair, 7 post-repair, 3 pre- and post-repair; and 9 controls) performed on a small-footprint 1.5-tesla (T) scanner. We calculated MRI-based pulmonary arterial blood flow, left ventricular eccentricity index, cardiac function and lung volume. Using the Wilcoxon rank sum test for continuous data and Fisher exact test for categorical data, we made pairwise group comparisons. RESULTS: The right-to-left ratios for pulmonary artery blood flow and lung volume were elevated in pre-repair and post-repair CDH versus controls (flow: P<0.005; volume: P<0.05 pre-/post-repair). Eccentricity index at end-systole significantly differed between pre-repair and post-repair CDH (P<0.01) and between pre-repair CDH and controls (P<0.001). CONCLUSION: Cardiopulmonary MRI is a viable method to serially evaluate cardiopulmonary hemodynamics and function in critically ill infants and is useful for capturing left-right asymmetries in pulmonary blood flow and lung volume.