Persistent left superior vena cava identified by transesophageal echocardiography.

A 70-year-old female with severe aortic stenosis presented for aortic valve replacement and underwent routine preoperative Swan-Ganz catheter placement. Transesophageal echocardiography demonstrated a dilated coronary sinus with a catheter present. A high suspicion of venous anomaly prompted an agitated saline study. Flow through the coronary sinus into the right atrium was observed, confirming the presence of a persistent left superior vena cava. Although the persistent left superior vena cava has a low prevalence in the general population, it is one of the most common thoracic venous anomalies. During central venous cannulation, the presence of venous anomalies increases procedural complication rates. Fortunately, our patient remained asymptomatic both before and after catheter insertion. Awareness of this anomaly could help clinicians avoid complications.

Left atrial dissection: A rare entity.

Left atrial wall dissection is uncommon. We present this rarity with transesophageal echocardiography in a 71-year-old female diagnosed with infective endocarditis three months following mitral valve repair, which along with the surgical intervention, may have contributed to the dissection.

Quadricuspid aortic valve: Case series and review of literature.

A quadricuspid aortic valve is an uncommon valve lesion. Its physical manifestations vary, and it may be associated with other cardiac lesions. Echocardiography is the imaging modality of choice, with computerized tomography or cardiac magnetic resonance imaging being utilized as adjunctive imaging modalities in certain cases. Herein, we present a case series of three patients with this valvular lesion treated at our institution, as well as a contemporary review of the literature.

The effects of cardiac resynchronization therapy on left ventricular and mitral valve geometry and secondary mitral regurgitation in patients with left bundle branch block.

BACKGROUND: Secondary mitral regurgitation (MR) is common in patients with left bundle branch block (LBBB) undergoing cardiac resynchronization therapy (CRT). We aimed to define CRT effects on left ventricular (LV) and mitral valve (MV) geometry, and their correlation with MR severity. METHODS: Forty-one patients with LBBB and ≥mild secondary MR underwent CRT between 2009 and 2012, and had baseline and follow-up echocardiograms available. Repeated measure and linear regression analyses were performed to assess for changes in MV and LV geometry and MR severity, and associations with follow-up MR grade. RESULTS: The mean age and baseline QRS duration were 65.5 ± 14.9 years and 160 ± 24 ms. At a mean follow-up of 2.6 ± 1.8 years, there was an increase in LV ejection fraction and reductions in LV end-systolic volume index, MR grade, and end-systolic interpapillary muscle distance (P < .05 for all). Linear correlations were observed between follow-up MR grade and baseline MV tenting height (r = .44), left atrial volume index (r = .41), LV end-systolic volume index (r = .4), MV tenting area (r = .38), LV ejection fraction (r = -.34), and end-systolic interpapillary muscle distance (r = .34) (P < .05 for all). Multiple regression analysis revealed associations between follow-up MR grade and baseline MV tenting height (ß/mm = 0.42, P = .006) and left atrial volume index (ß/mL/m2  = 0.4, P = .008), independent of QRS duration (ß/ms=-0.07; P = 0.6) and nonischemic cardiomyopathy (ß = -0.34, P = .02). CONCLUSIONS: Cardiac resynchronization therapy in patients with LBBB and secondary MR results in LV and MV geometric reverse remodeling and decreases MR severity. Extent of baseline MV tethering is independently associated with persistent MR at follow-up.

Stress Cardiomyopathy in a Patient with Hypertrophic Cardiomyopathy: Case Presentation and Review of the Literature.

Stress cardiomyopathy and hypertrophic cardiomyopathy are two distinct entities with different pathophysiologic causes. In the recent medical literature their concurrency has been described. During the acute phase of a stress cardiomyopathy making the diagnosis of a concomitant hypertrophic cardiomyopathy may be challenging, and has important implications in both the acute and longterm clinical management. Herein, we present a case of a stress cardiomyopathy occurring in a patient with hypertrophic cardiomyopathy, along with a review of the literature.

Effects of cardiac resynchronization therapy after inferior myocardial infarction on secondary mitral regurgitation and mitral valve geometry.

BACKGROUND: The effects of cardiac resynchronization therapy (CRT) on secondary mitral regurgitation (MR), and mitral valve (MV) and left ventricular (LV) geometry, in patients with prior inferior myocardial infarction is not clearly defined. We assessed these outcomes utilizing two-dimensional echocardiography, and analyzed echocardiographic geometric variables that may correlate with follow-up MR severity. METHODS: Between 2009 and 2012, 229 CRT were implanted. Twenty-two had prior inferior myocardial infarction, ≥mild MR at baseline, and serial echocardiography. A left bundle branch block was present in 12 (54.5%) patients. The pre-CRT and follow-up echocardiograms were analyzed for: (1) MR severity; (2) MV and LV geometry; and (3) LV remodeling. RESULTS: The median follow-up time was 2.2 years (interquartile range, 0.7-4). In 16 patients without an inferior myocardial scar, there was a reduction in MR jet area/left atrial area ratio (33.2% vs 25.8%; P = 0.06) and MR grade (2.3 vs 1.8; P = 0.05), and an increased LV ejection fraction (26.1% vs 30.9%; P = 0.04) and end-systolic posterior ventricular sulcus-anterolateral papillary muscle angle (133.9 vs 143.9 degrees; P = 0.01). In six patients with scar, there was no change in LV or MR parameters. Regression analysis revealed linear associations between baseline MV tenting height (r = 0.57; P = 0.006), LV end-diastolic diameter index (r = 0.5; P = 0.02), mitral septolateral annular diameter (r = 0.48; P = 0.03), and MV tenting area (r = 0.46; P = 0.03), with follow-up MR jet area/left atrial area ratio. CONCLUSIONS: In patients with prior inferior myocardial infarction and no scar, CRT is associated with decreased MR severity, and improved papillary muscle alignment and LV systolic function at follow-up.

Echocardiographic and clinical markers of left ventricular ejection fraction and moderate or greater systolic dysfunction in left ventricular noncompaction cardiomyopathy.

BACKGROUND: Left ventricular noncompaction (LVNC) is associated with progressive LV systolic dysfunction and dilated cardiomyopathy. We aimed to investigate the echocardiographic and clinical characteristics associated with LV ejection fraction (LVEF) and moderate or greater systolic dysfunction in patients with LVNC. METHODS: Our institutional echocardiography database was retrospectively reviewed between 2008 and 2014, and 62 patients with LVNC were identified. Forty-three (69%) had moderate or greater LV systolic dysfunction (LVEF ≤ 40%) and were compared with 19 (31%) patients with preserved or mildly reduced LVEF (>40%). Linear regression analyses were utilized to identify markers associated with LVEF. RESULTS: The mean age was 63 ± 17 years and noncompacted-to-compacted ratio was 2.3 ± 0.5, and was larger in patients with LVEF ≤ 40% (2.4 vs 2.1; P = .02). Patients with LVEF ≤ 40% were older, had more congestive heart failure, significant QRS interval prolongation, and greater LV remodeling and worse mean global longitudinal strain (GLS). Multivariate regression analysis revealed increased age (standardized regression coefficient (ß) = -0.17; P = .04) and QRS duration (ß = -0.13; P = .08), congestive heart failure (ß = -0.18; P = .04), and worsened GLS (ß = -0.40; P = .001) were independently associated with decreased LVEF in the cohort (overall model fit R2  = 0.71; P < .0001). Increased age (ß = -0.49; P = .01) and QRS duration (ß = -0.50; P = .002), and worsened GLS (ß = -0.33; P = .04), were also associated with a lower LVEF in patients with LVEF > 40%. CONCLUSIONS: The independent markers associated with LVEF and moderate or greater LV systolic dysfunction in patients with LVNC, in particular GLS and QRS duration, may detect high-risk candidates for more aggressive clinical surveillance and medical therapy.

A Focused Review on the Pathophysiology, Diagnosis, and Management of Cardiac Amyloidosis.

Amyloidosis is a systemic disorder that results from abnormal protein metabolism, producing amyloid fibrils that are subsequently deposited within vital organs. Cardiac involvement is typically associated with the specific subtypes of immunoglobulin lightchain, transthyretin, secondary amyloidosis, and dialysis-related amyloidosis. The hallmark of cardiac amyloidosis is the development of restrictive cardiomyopathy and heart failure, usually with a preserved left ventricular ejection fraction. The diagnosis is based on the integration of clinical signs and symptoms, echocardiography, cardiac magnetic resonance imaging, nuclear scintigraphy, electrocardiography, and cardiac biomarkers. Traditionally, management of heart failure symptoms and prevention of heart failure exacerbations have been the cornerstones of therapy. However, various treatments are currently under investigation that aim to eliminate or neutralize the underlying amyloidogenic substrate. Herein, we provide a focused review and discussion of the cardiovascular manifestations, epidemiologic and clinical characteristics, diagnostic modalities, and treatment strategies of cardiac amyloidosis.

A Staged Approach of Proximal Left Anterior Descending Coronary Artery Percutaneous Intervention Followed by Minimally Invasive Valve Surgery.

BACKGROUND AND AIM OF THE STUDY: A subset of patients requiring coronary revascularization of the proximal left anterior descending coronary artery (LAD) and valve surgery may benefit from a staged approach, rather than combined median sternotomy coronary artery bypass graft (CABG) and valve surgery. METHODS: A retrospective evaluation was made of the outcomes of patients with significant proximal LAD and valvular heart disease undergoing a staged approach of percutaneous coronary intervention (PCI) followed by minimally invasive valve surgery (MIVS) at the authors' institution between February 2009 and April 2014. A Kaplan-Meier analysis was performed to estimate mid-term survival. RESULTS: A total of 68 consecutive patients (mean age 75.2 ± 8.9 years) was identified. PCI was performed for one- or two-vessel disease in 76.5% and 23.5% of the patients, respectively. Within a median of 39 days (IQR 11-62 days), 91.2% of patients underwent primary MIVS, and 8.8% underwent re-operative MIVS, of which 58 (85.3%) were single-valve and 10 (14.7%) were double-valve operations. At the time of surgery, 72.1% of the patients were receiving dual anti-platelet therapy. The 30-day mortality was 2.9%. At a mean follow up of 26 ± 16 months, 7.4% of the patients had a non-target vessel acute coronary syndrome, and the survival rate was 88.2%. CONCLUSIONS: Among a select group of patients with proximal LAD and valvular disease, a staged approach of PCI followed by MIVS can be safely performed for primary or re-operative surgery, with excellent mid-term outcomes.

Apical hypertrophic cardiomyopathy with left ventricular apical aneurysm: Importance of multi-modality imaging.

Apical hypertrophic cardiomyopathy (HCM) is an uncommon variant of HCM characterized by apical hypertrophy without the septal predominance seen in the majority of HCM cases. In 2% of patients, a concomitant left ventricular apical aneurysm is observed, which increases the risk of sudden death and adverse HCM-related events. Multimodality imaging is helpful for appropriate identification of this particular morphologic pattern. Herein, we present a case of apical HCM with a left ventricular apical aneurysm, exemplifying the utility of a multimodality approach from resting electrocardiogram, transthoracic echocardiogram, left ventriculography, and cardiac magnetic resonance imaging, for proper risk stratification and treatment planning.

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.

Impact of cardiac resynchronization therapy on mitral valve apparatus geometry and clinical outcomes in patients with secondary mitral regurgitation.

BACKGROUND: Cardiac resynchronization therapy (CRT) may improve secondary mitral regurgitation (MR) in patients with cardiomyopathy. The effects on mitral valve (MV) and left ventricular (LV) geometry, however, have not been clearly defined. METHODS: Between 2009 and 2012, 229 CRT implants were performed at a single academic center. Seventy-one had ≥mild MR at baseline and serial echocardiography, without subsequent MV intervention. The pre-CRT and follow-up echocardiograms were retrospectively reviewed for (1) MV and LV geometry measurements; (2) MR grade; and (3) LV remodeling indices. RESULTS: The mean age was 67 ± 15 years, and the cardiomyopathy was ischemic in 37 (52%). At a mean follow-up of 4.0 ± 1.9 years, there were significant improvements in LV ejection fraction and size, MR grade, MV tenting area and anterior leaflet tethering angle, and end-systolic interpapillary muscle distance (IPMD), and reductions in moderate-to-severe or severe MR (27% vs 15%; P = .04) and New York Heart Association functional class III/IV symptoms (83% vs 41%; P < .001). Multivariable analysis revealed the pre-CRT MV tenting height (OR 1.25, 95% CI 1.01-1.56; P = .04) and end-systolic IPMD (OR 1.14, 95% CI 0.99-1.32; P = .08) as independently associated with moderate or greater MR at follow-up. Finally, at 5 years post-CRT implantation, the estimated survival and freedom from LV assist device or cardiac transplantation was 61%. CONCLUSIONS: CRT results in favorable effects on MV and LV geometry and decreases the prevalence of moderate-to-severe or severe MR and heart failure symptoms. The pre-CRT MV tenting height and IPMD are independently associated with persistent MR at follow-up.

Isolated Left Ventricular Noncompaction Cardiomyopathy: A Transient Disease?

Isolated left ventricular noncompaction is either a distinct cardiomyopathy or a morphologic trait shared by several different types of cardiomyopathies. Although there is no current gold standard for its diagnosis, cardiac imaging is the most commonly accepted modality. Described is a case of left ventricular noncompaction that resolved 2 years after the initial diagnosis.

Completeness of revascularization and its impact on the outcomes of a staged approach of percutaneous coronary intervention followed by minimally invasive valve surgery for patients with concomitant coronary artery and valvular heart disease.

BACKGROUND: A staged approach of percutaneous coronary intervention (PCI) followed by minimally invasive valve surgery (MIVS) is an alternative to the conventional combined coronary artery bypass and valve surgery for patients with concomitant coronary artery and valve disease. Limited data exist on degree of the completeness of revascularization achieved with this approach and its impact on outcomes. METHODS: A total of 138 patients, who underwent a staged approach between January 2009 and June 2013, were retrospectively evaluated. Coronary angiograms were reviewed by two cardiologists blinded to outcomes and were then categorized into two groups: complete or incomplete revascularization, which was defined as ≥1 major epicardial coronary arteries of at least 2.0 mm diameter with ≥70% untreated obstruction after the index PCI and before MIVS. RESULTS: Complete and incomplete revascularization was achieved in 105 (76%) and 33 (24%) patients, respectively. The patients with incomplete revascularization had a lower ejection fraction, a higher STS score, and more prior myocardial infarctions and multi-vessel coronary artery disease. There were no differences in the post-operative complications, 30-day mortality, or 3-year survival (84 vs. 83%, P = 0.68). After a median follow-up of 29 months, incompletely revascularized patients had a higher incidence of acute coronary syndrome (2.9 vs. 12.9%, P = 0.05). CONCLUSIONS: In patients undergoing a staged approach of PCI followed by MIVS, incomplete revascularization did not significantly impact the short or mid-term survival, but was associated with an increased incidence of acute coronary syndrome at follow-up. © 2015 Wiley Periodicals, Inc.

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.

Outcomes of Minimally Invasive Valve Surgery in Patients with Multiple Previous Cardiac Operations.

BACKGROUND: The study aim was to evaluate the outcomes of minimally invasive valve surgery, performed via a right anterior thoracotomy approach, in patients with a history of multiple (more than two) prior cardiac surgeries. METHODS: A retrospective review was conducted of all minimally invasive valve operations performed in patients with a prior history of two or more cardiac surgeries, including coronary artery bypass grafting (CABG) and/or valve surgery, at the authors' institution between January 2008 and November 2014. RESULTS: A total of 38 consecutive patients (23 males, 15 females; mean age 65.8 ± 14.6 years) were identified. Nine patients (24%) had two prior CABG operations, 18 (47%) had more than two prior valve surgeries, and 11 (29%) had a cardiac operative history that included both CABG and valve surgery. A total of 34 (89.5%) isolated valve procedures was identified; these consisted of 24 (64%) mitral valve operations, nine (23.7%) aortic valve replacements, and one (0.3%) tricuspid valve repair. Four patients (10.5%) underwent combined mitral and tricuspid valve surgery. Postoperatively, two patients (5.3%) had cerebrovascular accidents, three (7.9%) required reoperation for bleeding, and three (7.9%) had acute kidney injury. The median hospital length of stay was 9.5 days (IQR: 7-16 days). The 30-day mortality was 7.9%. The cumulative survival at one year was 82%, and was 72% at five years. CONCLUSIONS: Minimally invasive reoperative valve surgery after multiple prior cardiac operations is safe and feasible, with good perioperative outcomes and mid-term survival.

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.

Effects of Statin Therapy in Patients with Systemic Lupus Erythematosus.

OBJECTIVES: Systemic lupus erythematosus (SLE) is associated with a significant risk of cardiovascular mortality. The use of statins for lipid modulation and the prevention of cardiovascular disease in this population also may impart pleiotropic anti-inflammatory and immunomodulatory effects. Our aim was to identify studies that compared the use of statins and placebo or no statin therapy in patients with SLE. METHODS: A meta-analysis was conducted on the follow-up measures of serum lipid levels, inflammatory markers, and disease activity, which was measured using the SLE Disease Activity Index (SLEDAI) score. The mean difference (MD) was calculated by the inverse variance method under a fixed or random-effects model, as appropriate. RESULTS: A total of eight studies met our inclusion criteria, including five randomized controlled trials. The total number of patients was 814 (statins 446, placebo/no statins 368), and follow-up ranged from 1 month to 7 years. The total cholesterol (MD -23.48 mg/dL, 95% confidence interval [CI] -34.57 to -12.39, P < 0.0001), low-density lipoprotein (MD -20.7 mg/dL, 95% CI -30.51 to -10.89, P < 0.0001), and high-sensitivity C-reactive protein (MD -0.40 mg/dL, 95% CI -0.64 to -0.16, P = 0.001) levels were significantly reduced by statin therapy. There was no change with statin use in serum levels of high-density lipoprotein and conventional C-reactive protein, and there was no difference in the SLEDAI score. CONCLUSIONS: The use of statins in SLE reduced the serum lipid and high-sensitivity C-reactive protein levels, which suggests a role for the primary prevention of cardiovascular disease. Statins did not affect the SLEDAI score, and therefore their use for modifying SLE disease activity levels is not presently supported.

Targeting the Papillary Muscles in Mitral Valve Repair for Ischemic Mitral Regurgitation.

Ischemic mitral regurgitation due to left ventricular remodeling and leaflet tethering is associated with decreased survival, and the optimal management remains unknown. Restrictive mitral annuloplasty is the current treatment of choice, but it is associated with a 15% to 30% incidence of late recurrent mitral regurgitation, which confers a poor prognosis. A pathophysiology-guided approach to surgical repair is preferable, with a goal of alleviating leaflet tethering and restoring proper subvalvular mechanics. In patients with preoperative predictors of annuloplasty failure, combining a papillary muscle repositioning technique with conventional annuloplasty repair allows for complete geometric repair of the ventriculomitral unit.

Pleiotropic effects of the 3-hydroxy-3-methylglutaryl-CoA reductase inhibitors in pulmonary diseases: a comprehensive review.

The 3-hydroxy-3-methylglutaryl-CoA reductase inhibitors (statins) are used extensively in the treatment of hyperlipidemia. They have also demonstrated a secondary benefit in a variety of other disease processes, actions which are known as pleiotropic effects. Review of the current pulmonary literature suggests a potential advantage of statin usage in a variety of pulmonary conditions. Our paper serves as a focused discussion on the pleiotropic effects of statins in the most common pulmonary disorders.