Cleft posterior mitral valve leaflet in an adult with Turner syndrome diagnosed with the use of 3-dimensional transesophageal echocardiography.

Turner syndrome is a monosomy (45,X karyotype) in which the prevalence of cardiovascular anomalies is high. However, this aspect of Turner syndrome has received little attention outside of the pediatric medical literature, and the entire spectrum of cardiovascular conditions in adults remains unknown. We present the case of a 34-year-old woman who had Turner syndrome. When she was a teenager, her native bicuspid aortic valve was replaced with a mechanical prosthesis. Fifteen years later, during preoperative examination for prosthesis-patient mismatch, severe mitral regurgitation was detected, and a congenital cleft in the posterior leaflet of the mitral valve was diagnosed with use of 3-dimensional transesophageal echocardiography. The patient underwent concurrent mitral valve repair and aortic valve replacement. To our knowledge, this is the first report of a cleft in the posterior mitral valve leaflet as a cardiovascular defect observed in Turner syndrome, and the first such instance to have been diagnosed with the use of 3-dimensional echocardiography.

Favorable effects of left ventricular reconstruction in patients excluded from the Surgical Treatments for Ischemic Heart Failure (STICH) trial.

OBJECTIVE: We sought to examine the hemodynamic effects at 1 month and 1 year of left ventricular reconstruction by means of endoventricular patch plasty for patients with acute or chronic, very severe post-myocardial infarction heart failure who would have been systematically excluded from the Surgical Treatments for Ischemic Heart Failure (STICH) trial. METHODS: From 2002 to May 2008, 274 patients underwent left ventricular reconstruction for post-myocardial infarction scarring; 117 of these patients would not have been eligible for the STICH trial. The pertinent criteria for exclusion included 12 patients with no coronary vessel suitable for coronary artery bypass grafting; 17 patients within a month of myocardial infarction, including 11 with acute heart failure (8 septal ruptures and 3 cases of ventricular tachycardia); 48 patients receiving intravenous inotropes, intra-aortic balloon pumping, or both; 15 patients with bifocal or posterior scarring; 4 patients scheduled for heart transplantation; and 21 patients meeting 5 other exclusion criteria. These patients (mean age, 64 years; age range, 34-83 years) preoperatively had a mean 49% (range, 35%-75%) scarred left ventricular circumference, as determined by means of magnetic resonance imaging assessment. In the patients with chronic heart failure, the preoperative ejection fraction was 26% ± 4% (range, 9%-34%), the end-diastolic volume index was 130 ± 43 mL/m(2) (range, 62-343 mL/m(2)), and the end-systolic volume index was 95 ± 37 mL/m(2) (range, 45-289 mL/m(2)). Mitral regurgitation was mild to severe in 56 patients and associated with annular dilatation (≥35 mm) in 51 patients. A strategy of left ventricular reconstruction by means of endoventricular circular suturing and patching excluded the scarred left ventricular wall and was balloon sized to provide a diastolic volume of 50 mL/m(2). Circular patches were used for anteroseptoapical lesions, and triangular patches were used for posterior lesions. The mitral valve was repaired in 51 patients, and coronary revascularization was performed in 105 patients (arterial grafts in 95 and mixed in 12). Seventy-eight patients had endocardectomy, and cryotherapy was used in 39 patients for ventricular tachycardia. RESULTS: Four in-hospital and 2 delayed deaths occurred during the first year. In 101 patients with chronic heart failure, magnetic resonance imaging revealed that ejection fraction improved from 26% ± 4% preoperatively to 40% ± 8% at 1 month and 44% ± 11% at 1 year postoperatively. At these same time points, the end-diastolic volume index was reduced from 130 ± 43 mL/m(2) to 81 ± 27 and 82 ± 25 mL/m(2), respectively, and the end-systolic volume index was reduced from 96 ± 45 mL/m(2) to 50 ± 21 and 47 ± 20 mL/m(2), respectively. CONCLUSIONS: With minimal associated mortality, left ventricular reconstruction produces durable improvement in left ventricular function in patients with a large scarred ventricular wall. Considering that this patient cohort would have been systematically excluded from the STICH trial, care should be taken not to extrapolate that study's results too widely so as to inappropriately deny selected patients an effective treatment for ischemic cardiomyopathies with an injured ventricle.

Preoperative and late postoperative mitral regurgitation in ventricular reconstruction: role of local left ventricular deformation.

BACKGROUND: We sought to analyze the characteristics of local left ventricular deformation related to functional mitral regurgitation (MR) in post-anterior myocardial infarction scar, and to evaluate how local remodeling contributes to late development of MR after surgical ventricular reconstruction by endoventricular circular patch plasty repair. METHODS: Two hundred twenty-one consecutive patients (aged 60 +/- 9 years; 193 males) with previous transmural anterior infarction underwent heart catheterization both before and 1 year after endoventricular circular patch plasty repair. Preoperative global left ventricular shape determinants (eccentricity and circularity indexes), regional curvature and wall motion (centerline), and both preoperative and 1-year postoperative hemodynamic parameters (volumes, ejection fraction, capillary wedge and pulmonary artery pressures) were calculated. RESULTS: Forty-eight patients had (MR patients), and 173 did not have (NoMR patients) angiographic MR grade 2 or more preoperatively; at follow-up, 30 NoMR patients had MR (late MR [LMR]). Before surgery, MR patients had larger left ventricular volumes, higher capillary wedge and mean pulmonary artery pressures, and lower ejection fraction and cardiac index. The LMR patients had similarly high capillary wedge and pulmonary artery pressures as MR patients; otherwise, they did not differ from NoMR patients. Mitral regurgitation patients had wider lateral wall akinesia and greater inferior wall asynergy; the inferobasal region was hypokinetic in LMR patients. In MR patients, inferior wall systolic curvature was less negative; the inferobasal region had a more positive curvature in LMR patients. CONCLUSIONS: Local deformation of the inferior wall with loss of systolic inward bending is associated with functional MR, while asynergy and systolic deformation of the inferobasal region and high capillary wedge pressure are prognostic signs of MR development late after endoventricular circular patch plasty repair.

Surgical ventricular restoration improves mechanical intraventricular dyssynchrony in ischemic cardiomyopathy.

BACKGROUND: In ischemic cardiomyopathy, dyssynchrony of left ventricular (LV) mechanical contraction produces adverse hemodynamic consequences. This study tests the capacity of geometric rebuilding by surgical ventricular restoration (SVR) to restore a more synchronous contractile pattern after a mechanical, rather than electrical, intervention. METHODS AND RESULTS: A prospective study of the global and regional components of dyssynchrony was conducted in 30 patients (58+/-8 years of age) undergoing SVR at the Cardiothoracic Center of Monaco. The protocol used simultaneous measurements of ventricular volumes and pressure to construct pressure/volume (P/V) and pressure/length (P/L) loops. Angiograms were done before and after SVR to study a 600-ms cycle during atrial pacing at 100 bpm. Mean QRS duration was similar, at 100+/-17 ms preoperatively and 114+/-28 ms postoperatively (NS). Preoperative LV contraction was highly asynchronous, because P/V loops showed abnormal isometric phases with a right shifting. Endocardial time motion was either early or delayed at the end-systolic phase so that P/L loops were markedly abnormal in size, shape, and orientation. Postoperatively, SVR resulted in leftward shifting of P/V loops and increased area; endocardial time motion and P/L loops almost normalized to allow a better contribution of single regions to global ejection. The hemodynamic consequences of SVR were improved ejection fraction (30+/-13% to 45+/-12%; P=0.001); reduced end-diastolic and end-systolic volume index (202+/-76 to 122+/-48 and 144+/-69 to 69+/-40 mL/m(2); P=0.001); more rapid peak filling rate (1.75+/-0.7 to 2.32+/-0.7 EDV/s; P=0.0001); peak ejection rate (1.7+/-0.7 to 2.6+/-0.9 Sv/s; P=0.0002), and mechanical efficiency (0.56+/-0.15 to 0.65+/-0.18; P=0.04). CONCLUSIONS: SVR produces a mechanical intraventricular resynchronization that improves LV performance.