Clinical presentation and echocardiographic characteristics of Uhl's anomaly.

Uhl's anomaly is a rare cardiac malformation that results in partial or complete absence of the right ventricular myocardium. It most commonly presents in prenatal or newborn infants; however, it may also be found in some adults as advanced right-sided heart failure. Differential diagnoses include arrhythmogenic right ventricular dysplasia and Ebstein's anomaly. Herein, we describe the clinical presentation of Uhl's anomaly in a previously undiagnosed middle-aged adult, and review the echocardiographic criteria used to diagnose and differentiate this rare, albeit important, myocardial disorder.

Transaortic Edge-to-Edge Repair for Functional Mitral Regurgitation During Aortic Valve Replacement: A 13-Year Experience.

OBJECTIVE: The study evaluated the feasibility of a transaortic edge-to-edge mitral valve repair (Alfieri stitch) for moderate or greater (≥2+) functional mitral regurgitation (MR) in high-risk patients undergoing aortic valve replacement. METHODS: We retrospectively evaluated 40 consecutive patients who underwent aortic valve replacement combined with a transaortic edge-to-edge mitral valve repair for 2+ or greater functional MR, between February 2002 and April 2015. The MR was graded semiquantitatively as 0 (trace/none), mild (1+), moderate (2+), or moderate to severe (3-4+). RESULTS: Thirty-two patients had aortic stenosis, and eight had aortic regurgitation. The mean ± standard deviation (SD) age was 77.5 ± 5 years, 34 (85%) were male, and the mean ± SD EuroSCORE II was 14.3% ± 12.9. At a median follow-up of 1 month (interquartile range, 0.75-10), there were significant improvements in preoperative versus postoperative median MR grade (3+ vs 1+, P < 0.001), mean left ventricular ejection fraction (34% vs 41%, P = 0.018), left ventricular end-diastolic diameter (54 vs 49 mm, P = 0.005), and pulmonary artery systolic pressure (49 vs 35 mm Hg, P < 0.001). Persistent 3 to 4+ MR occurred in two patients (5%). In 12 patients with at least 6-month follow-up (mean ± SD, 18 ± 11 months), a sustained improvement in all echocardiographic parameters was observed, with persistent 3 to 4+ MR occurring in one patient (8.3%). Actuarial survival at 1, 3, and 4.5 years was 82% ± 6, 71% ± 8, and 65% ± 10, respectively. CONCLUSIONS: A transaortic edge-to-edge repair for 2+ or greater functional MR can be safely performed during aortic valve replacement and is associated with improvements in MR grade, left ventricular remodeling, and pulmonary hemodynamics.

Aortic and/or mitral valve surgery in patients with pulmonary hypertension performed via a minimally invasive approach.

Pulmonary hypertension (PH) in the setting of left-sided valvular heart disease is common, and significantly increases the risk of perioperative morbidity and mortality in patients undergoing aortic and/or mitral valve surgery. Minimally invasive valve surgery is associated with a decreased incidence of perioperative complications, and a faster recovery, when compared with conventional sternotomy. In the present study, the outcomes of 569 patients with PH who underwent minimally invasive aortic and/or mitral valve surgery were analysed. The operative mortality was 3.5%, and postoperative strokes occurred in 1.4%. The mean intensive care unit and hospital length of stays were 50 ± 14 h and 7 ± 1 days, respectively. Patients with severe PH (mean pulmonary artery pressure ≥40 mmHg) had a longer duration of postoperative ventilation and intensive care unit length of stay, when compared with mild/moderate PH, and similar clinical outcomes. In conclusion, a minimally invasive approach to aortic and/or mitral valve surgery in patients with PH is safe and feasible, and may be considered as an alternative to conventional median sternotomy.

Is a minimally invasive approach for mitral valve surgery more cost-effective than median sternotomy?

A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was: is a minimally invasive approach for mitral valve surgery more cost-effective than median sternotomy? Altogether, 51 studies were found using the reported search, of which 7 represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers were tabulated. No randomized controlled trials have assessed the cost-effectiveness of less invasive mitral valve surgery compared with median sternotomy, with the best evidence coming from retrospective and propensity-matched analyses. Five studies compared minithoracotomy versus sternotomy, one compared minimally invasive port-access surgery versus sternotomy and one compared video-assisted minithoracotomy versus sternotomy. The use of a minithoracotomy or a minimally invasive port-access approach for mitral valve surgery resulted in significant reductions in costs of cardiac imaging and laboratory tests, lower use of blood products, fewer perioperative infections, faster recovery, shorter hospital length of stay, fewer requirements for rehabilitation and lower readmission rates in the following postoperative year. We conclude that a minimally invasive approach for mitral valve surgery is safe, effective and significantly more cost-effective than median sternotomy.

The use of ranolazine in non-anginal cardiovascular disorders: A review of current data and ongoing randomized clinical trials.

Ranolazine has characteristic properties of a selective inhibitor of the inward sodium current. It is primarily indicated as an anti-anginal agent in patients with coronary artery disease and chronic stable angina. Recently, ranolazine has been noted to possibly impart beneficial effects in various other cardiac conditions, including new-onset, paroxysmal, and chronic atrial fibrillation, post-operative atrial fibrillation, ventricular arrhythmias, post-revascularization coronary artery disease, chemotherapeutic cardiotoxicity, and diastolic and microvascular dysfunction. Herein, we present a review of the current clinical evidence describing the adjunctive or synergistic effects of ranolazine in non-angina related cardiovascular disorders, and include a discussion of the ongoing randomized trials investigating the therapeutic potential of ranolazine in a variety of cardiovascular diseases.

The Use of Statins in the Treatment and Prevention of Atrial Fibrillation.

The hydroxy-methyl-glutaryl-CoA reductase inhibitors (statins) are used extensively in the treatment of hyperlipidemia and in the primary and secondary prevention of cardiovascular and cerebrovascular diseases. Statins have also been demonstrated to confer secondary pleiotropic benefits in a variety of other disease processes, including a potential advantage in treating and preventing atrial fibrillation. These effects are primarily due to the up-regulation of endothelial nitric oxide synthase activity and a decrease in nicotinamide adenine dinucleotide phosphate oxidase production, which leads to downstream effects that improve the electromechanical function of atrial and myocardial tissue. The following serves as a focused and updated review of the published clinical data regarding the pleiotropic effects of statins in atrial fibrillation.

A Meta-Analysis of Ring Annuloplasty Versus Combined Ring Annuloplasty and Subvalvular Repair for Moderate-to-Severe Functional Mitral Regurgitation.

BACKGROUND: A ring annuloplasty (Ring) for moderate-to-severe functional mitral regurgitation (MR) is associated with suboptimal outcomes, and adjunctive subvalvular repair techniques (Ring + Subvalvular) have been advocated to improve repair durability. However, the outcomes of this strategy are not clearly defined. METHODS: A systematic review was performed to identify studies that compared a Ring versus Ring + Subvalvular repair for the treatment of moderate-to-severe functional MR. A meta-analysis was conducted on clinical outcomes and echocardiographic measures of follow-up MR and left ventricular reverse remodeling. Risk ratio (RR) and mean difference (MD) were calculated by the Mantel-Haenszel and Inverse Variance methods under a fixed or random effects model, as appropriate. RESULTS: Seven non-randomized studies were identified, with a total of 531 patients (Ring = 301, Ring + Subvalvular = 230). At follow-up (range 30-47 months), a Ring + Subvalvular repair was associated with a lower mortality (RR = 0.59, 95%confidence interval [CI] 0.37-0.94, p = 0.03), greater freedom from moderate or greater MR (RR = 0.44, 95%CI 0.27-0.72, p = 0.001), and improved left ventricular end-diastolic diameter (MD = -3.49 mm, 95%CI -5.45 to -1.53, p = 0.0005) and New York Heart Association functional class (MD = -0.25, 95%CI -0.42 to -0.08, p = 0.004). A trend toward a reduced risk of reoperation for recurrent MR was noted with a combined Ring + Subvalvular repair (RR = 0.28, 95%CI 0.06-1.22, p = 0.09). Finally, a Ring + Subvalvular repair did not impact operative mortality or follow-up left ventricular ejection fraction. CONCLUSION: A combined Ring + Subvalvular repair for moderate-to-severe functional MR can be safely performed, and may improve mid-term outcomes, as compared with Ring alone.

Combined Mitral and Tricuspid Valve Surgery Performed via a Right Minithoracotomy Approach.

OBJECTIVE: Combined mitral and tricuspid valve surgery is associated with an increased perioperative morbidity and mortality. We evaluated the outcomes of a less invasive right minithoracotomy approach in patients undergoing primary or reoperative double-valve surgery. METHODS: We retrospectively evaluated 132 consecutive patients with mitral and tricuspid valve disease who underwent double-valve surgery via a right minithoracotomy at our institution between January 2009 and April 2014. RESULTS: The cohort included 81 female (61%) and 51 male (39%) patients, with a mean ± SD age of 67 ± 13 years. The mean ± SD preoperative left ventricular ejection fraction, mitral regurgitation grade, and creatinine were 53% ± 12%, 3.8 ± 0.6, and 1.26 ± 1.17, respectively. The patients underwent primary (88%) or reoperative (12%) mitral and tricuspid valve surgery, which consisted of 88 mitral repairs (67%), 44 mitral replacements (33%), 131 tricuspid repairs (99%), and 1 tricuspid replacement (1%). Postoperatively, there were 6 cases of acute kidney injury (5%), 6 reoperations for bleeding (5%), 4 cerebrovascular accidents (3%), and 12 cases of atrial fibrillation (9%). The median intensive care unit length of stay and total hospital lengths of stay were 61 hours (interquartile range, 43-112 hours) and 8 days (interquartile range, 6-13 days), respectively. The in-hospital mortality was 4%. Actuarial survival at 1 and 5 years was 93% and 88%, respectively. CONCLUSIONS: In patients undergoing primary or reoperative mitral and tricuspid valve surgery, a right minithoracotomy approach is associated with a low perioperative morbidity and good midterm survival.

Comorbidities frequency in Takotsubo syndrome: an international collaborative systematic review including 1109 patients.

BACKGROUND: To identify predisposing factors that can result in the onset of takotsubo syndrome, we performed an international, collaborative systematic review focusing on clinical characteristics and comorbidities of patients with takotsubo syndrome. METHODS: We searched and reviewed cited references up to August 2013 to identify relevant studies. Corresponding authors of selected studies were contacted and asked to provide additional quantitative details. Data from each study were extracted by 2 independent reviewers. The cumulative prevalence of presenting features and comorbidities was assessed. Nineteen studies whose authors sent the requested information were included in the systematic review, with a total of 1109 patients (951 women; mean age, 59-76 years). Evaluation of risk factors showed that obesity was present in 17% of patients (range, 2%-48%), hypertension in 54% (range, 27%-83%), dyslipidemia in 32% (range, 7%-59%), diabetes in 17% (range, 4%-34%), and smoking in 22% (range, 6%-49%). Emotional stressors preceded takotsubo syndrome in 39% of patients and physical stressors in 35%. The most common comorbidities were psychological disorders (24%; range, 0-49%), pulmonary diseases (15%; range, 0-22%), and malignancies (10%; range, 4%-29%). Other common associated disorders were neurologic diseases (7%; range, 0-22%), chronic kidney disease (7%; range, 2%-27%), and thyroid diseases (6%; range, 0-37%). CONCLUSIONS: Patients with takotsubo syndrome have a relevant prevalence of cardiovascular risk factors and associated comorbidities. Such of associations needs to be evaluated in further studies.

Usefulness of the troponin-ejection fraction product to differentiate stress cardiomyopathy from ST-segment elevation myocardial infarction.

The presentation of stress cardiomyopathy (SC) with nonobstructive coronary artery disease mimics that of ST-segment elevation myocardial infarction (STEMI) due to coronary occlusion. No single parameter has been successful in differentiating the 2 entities. We thus sought to develop a noninvasive clinical tool to discriminate between these 2 conditions. We retrospectively reviewed 59 consecutive cases of SC at our institution from July 2005 through June 2011 and compared those with 60 consecutives cases of angiographically confirmed STEMI treated with primary percutaneous coronary intervention in the same period. All patients underwent acute echocardiography, and the peak troponin I level was determined. The troponin-ejection fraction product (TEFP) was derived by multiplying the peak troponin I level and the echocardiographically derived left ventricular ejection fraction. Comparing the SC and STEMI groups, the mean left ventricular ejection fraction at the time of presentation was 30 ± 9% versus 44 ± 11%, respectively (p <0.001), and the peak troponin I was 7.6 ± 18 versus 102.2 ± 110.3 ng/dl, respectively (p <0.001). The mean TEFP was thus 182 ± 380 and 4,088 ± 4,244 for the SC and STEMI groups, respectively (p <0.001). Receiver operating characteristic curve analysis showed that a TEFP value ≥250 had a sensitivity of 95%, a specificity of 87%, a negative predictive value of 94%, a positive predictive value of 88%, and an overall accuracy of 91% to differentiate a true STEMI from SC (C-statistic 0.91 ± 0.02, p <0.001). In conclusion, for patients not undergoing emergent angiography, the TEFP may be used with high accuracy to differentiate SC with nonobstructive coronary artery disease from true STEMI due to coronary occlusion.

Asymptomatic anomalous left anterior descending artery arising from the right coronary artery with a rare anterior course.

The presence of a malignant course on the coronary circulation is the second leading cause of sudden cardiac death in young adults. One type of malignant conformation is an anomalous left anterior descending artery (LAD) arising from the right coronary artery (RCA). In a study examining 70,850 patients undergoing coronary angiography, the prevalence of this anomaly was noted in 0.006% of total patients and in 2.3% of congenital coronary anomaly cases. Of the four patients presenting with this anomaly, only one was noted to have an interarterial course of the LAD between the aorta and pulmonary artery. We describe the case of a patient who was incidentally found to have a malignant anomalous LAD arising from the RCA when he presented with a non-ST elevation myocardial infarction due to a ruptured plaque in an obtuse marginal branch. This patient survived for 57 years with no symptoms.