Truncus arteriosus versus tetralogy of Fallot with pulmonary atresia.

Truncus arteriosus and tetralogy of Fallot with pulmonary atresia may be difficult to differentiate prenatally. We present a case that, on newborn echocardiography, angiography, and intraoperative inspection, shared features of both diagnoses.

Mitral valve prolapse is associated with altered extracellular matrix gene expression patterns.

Mitral valve prolapse (MVP) is the leading indication for isolated mitral valve surgery in the United States. Disorganization of collagens and glycosaminoglycans in the valvular extracellular matrix (ECM) are histological hallmarks of MVP. We performed a transcriptome analysis to study the alterations in ECM-related gene expression in humans with sporadic MVP. Mitral valve specimens were obtained from individuals undergoing valve repair for MVP (n=7 patients) and from non-beating heart-tissue donors (n=3 controls). Purified RNA was subjected to whole-transcriptome microarray analysis. Microarray results were validated by quantitative reverse transcription polymerase chain reaction (RT-qPCR). Gene ontology enrichment analysis was performed. 2046 unique genes showed significant differential expression (false discovery rate <0.5%). After demonstrating appropriate sample clustering, microarray results were globally validated using a subset of 22 differentially expressed genes by RT-qPCR (Pearson's correlation r=0.65, p=0.001). Gene ontology enrichment analyses performed with ErmineJ and DAVID Bioinformatics Database demonstrated overrepresentation of ECM components (p<0.05). Functional annotation clustering calculated enrichment of ECM-related ontology groups (enrichment score=4.1). ECM-related gene expression is significantly altered in MVP. Our study is consistent with the histologically observed alterations in collagen and mucopolysaccharide profiles of myxomatous mitral valves. Furthermore, whole-transcriptome analyses suggest dysregulation of multiple pathways, including TGF-beta signaling.

Assessment of a mitral valve replacement skills trainer: a simplified, low-cost approach.

OBJECTIVES: Simulated mitral valve replacement may aid in the assessment of technical skills required for adequate performance in the operating room. We sought to design and assess a mitral valve replacement training station that is low-cost, nonperishable, portable, and reproducible as a first step in developing a mitral valve surgical skills curriculum. METHODS: Nineteen physicians (7 general surgery residents, 8 cardiothoracic surgery residents, and 4 attending cardiothoracic surgeons) underwent simulated mitral valve replacement testing. Simulated mitral valve replacement was performed on a training station consisting of a replaceable "mitral annulus" inside a restrictive "left atrium." Eight components of performance were graded on a 5-point scale. A composite score (100 point maximum) was calculated by weighting the grades by procedural time. The effect of training level was evaluated using analysis of variance and post hoc Tukey honestly significant difference. RESULTS: The speed of simulated mitral valve replacement varied among general surgery residents, cardiothoracic surgery residents, and attending cardiothoracic surgeons (52.9 ± 9.0 vs 32.8 ± 4.7 vs 28.0 ± 3.5 minutes, respectively; F = 25.3; P < .001). Level of training significantly affected all 8 evaluation components (P < .001). Composite scores increased with level of training (general surgery residents 32.9 ± 11.4, cardiothoracic surgery residents 65.1 ± 11.5, and attending cardiothoracic surgeons 88.3 ± 7.8 of a possible 100 points; F = 35.7; P < .001). Cardiothoracic surgery residents who reported having performed 10 to 50 mitral valve replacements as the primary surgeon had a composite score of 65.0 ± 2.8 (P < .01 compared with attending cardiothoracic surgeons). CONCLUSIONS: Simulated mitral valve replacement can be performed using this simple, affordable, portable setup. Performance scores correlate with level of training and experience, but residents who performed 10 to 50 mitral valve replacements still failed to reach attending-level proficiency. This training simulator may facilitate skills practice and evaluation of competency in cardiac surgery trainees.

Regional changes in coaptation geometry after reduction annuloplasty for functional mitral regurgitation.

BACKGROUND: While it is known that band annuloplasty for functional mitral regurgitation (FMR) improves leaflet coaptation, the effect on regional coaptation geometry has not previously been well defined. We used three-dimensional transesophageal echocardiography (3D-TEE) to analyze the regional effects of semirigid band annuloplasty on annular geometry and leaflet coaptation zones of patients with FMR. METHODS: Sixteen patients with severe FMR underwent a semirigid band annuloplasty. Intraoperative full volume 3D-TEE datasets were acquired pre valve and post valve repair. Offline analysis assessed annular dimensions and regional coaptation zone geometry. The regions were defined as R1 (A1-P1), R2 (A2-P2), and R3 (A3-P3); coaptation distance, coaptation depth, and coaptation length were measured in each region. Differences were analyzed with repeated measures within a general linear model. RESULTS: Band annuloplasty decreased mitral regurgitation grade from 3.7 to 0.1 (scale 0 to 4). Annular septolateral dimension (p<0.01) and coaptation distance (p<0.01) decreased significantly in all regions. Likewise, anterior and posterior leaflet coaptation lengths increased in all regions (p<0.01 and p=0.05, respectively), with region 2 showing the greatest increase (p=0.01). Changes in coaptation depth were not significant. CONCLUSIONS: Semirigid band annuloplasty for FMR produces significant regional remodeling of leaflet coaptation zones, with region 2 showing the greatest increase in leaflet coaptation length. This regional analysis of annular geometry and leaflet coaptation creates a framework to better understand the mechanisms of surgical success or failure of annuloplasty for FMR.

Giant coronary artery aneurysm in a patient with Behçet's disease.

Behçet's disease is a rare autoimmune vasculitis that may cause coronary artery aneurysms. We discuss the evaluation and management decisions for a 19-year-old female with a giant rapidly expanding aneurysm of the proximal left anterior descending coronary artery and Behçet's disease.

Reoperative valve surgery in the elderly: predictors of risk and long-term survival.

BACKGROUND: Elderly patients requiring reoperative cardiac surgery for valve disease are considered high risk for immediate outcomes, but little is known about their long-term survival. It is often conjectured that medical therapy provides equivalent late survival in this population, which may dissuade both patient and surgeon from considering reoperation. We analyzed a cohort of such patients undergoing reoperative valve surgery to determine their long-term survival. METHODS: From 1992 through 2007, 363 patients aged 75 years or more underwent reoperative isolated valve surgery; 211 (58%) had aortic valve replacement and 152 (42%) had mitral valve surgery. Mean age was 80.5 years. Hospital outcomes were prospectively recorded. Survival from all-cause death was determined from the Social Security Death Index. RESULTS: Hospital mortality was 13.8% (12.8% for aortic and 15.1% for mitral valve operations; p = 0.52). Multivariable predictors of hospital death were New York Heart Association functional class III or IV heart failure (odds ratio = 3.19, p = 0.012), dialysis (odds ratio = 15.63, p = 0.003), and more than one reoperation (odds ratio = 2.59, p = 0.058). At 5 years, overall survival was 62% ± 3% for all patients (66% ± 4% for aortic and 56% ± 4% for mitral valve patients). For aortic valve patients aged 80 years or more, 5-year survival was 60% ± 0.6%. Life expectancy table analysis predicted a 5-year survival of 57% for an age-matched and sex-matched comparison group. CONCLUSIONS: Reoperative surgery for elderly patients with isolated aortic or mitral valve pathology is associated with excellent long-term survival, particularly when treating aortic valve disease. While in-hospital mortality is higher among the elderly than among younger patients, specific predictors of poor outcome can be identified preoperatively to risk stratify these patients.

Analysis of the mitral coaptation zone in normal and functional regurgitant valves.

BACKGROUND: Functional mitral regurgitation (FMR) is associated with leaflet displacement and tethering. Little is known about regional coaptation zones, including variations in coaptation length (CL) and contributions of anterior and posterior leaflets. Regional coaptation zones were analyzed in patients with normal mitral valves and with FMR. METHODS: Cardiac surgery patients underwent a three-dimensional transesophageal echocardiography. Four-dimensional volumetric datasets were acquired with Doppler interrogation. Offline analysis was performed. Orthogonal views were extracted in diastole and systole. Leaflet dimensions and coaptation distance and depth were examined for posterior and apical displacement of the coaptation zones. RESULTS: Twenty patients were analyzed (10 normal and 10 with 2 to 4+ FMR). Anterior leaflet CL was greater than posterior leaflet CL: 2.2+/-0.6 mm versus 0.9+/-0.3 mm in region 1, 3.2+/-0.7 mm versus 1.2+/-0.6 mm in region 2, and 1.8+/-0.4 mm versus 0.6+/-0.3 mm in region 3 (p<0.001). The FMR was associated with shorter leaflet CLs, with a mean anterior CL of 1.7+/-0.4 mm versus 3.1+/-0.4 mm (p=0.04), and a mean posterior CL of 0.7+/-0.3 mm versus 1.1+/-0.3 mm (p=0.03). The biggest difference in CLs was in A2-P2. Coaptation distance and depth were higher in the FMR group: 21.7+/-1.0 mm versus 17.9+/-1.0 mm (p=0.01), and 8.6+/-0.7 mm versus 5.0+/-0.7 mm (p<0.01). CONCLUSIONS: Mitral valve leaflet CL is asymmetric in normal valves, with anterior dominance. Functional mitral regurgitation is associated with a relocated coaptation zone, regional changes, and diminished coaptation. These data suggest an "anterior leaflet reserve." Posterior movement of the coaptation line compensates for annular dilation and presumed left ventricular enlargement in order to maintain competency until inadequate anterior leaflet CL occurs.