Visual Estimation of the Severity of Aortic Stenosis and the Calcium Burden by 2-Dimensional Echocardiography: Is It Reliable?

OBJECTIVES: Guidelines have recommended aortic valve surgery in asymptomatic patients with severe aortic stenosis and a large aortic valve calcium burden. The purpose of this study was to determine whether visual assessment of aortic valve calcium and stenosis severity are reliable based on 2-dimensional echocardiography alone. METHODS: We prospectively enrolled 68 patients with aortic stenosis and compared them with 30 control participants without aortic stenosis. All had aortic valve calcium score assessment by computed tomography. In a random order, 2-dimensional images without hemodynamic data were independently reviewed by 2 level 3-trained echocardiographers, who then classified these patients into categories based on aortic valve calcium and stenosis severity. RESULTS: The 68 patients (mean age ± SD, 74 ± 10 years) were classified as having mild (n = 28), moderate (n = 22), and severe (n = 18) aortic stenosis. When the observers were asked to grade the degree of valve calcification, the agreement between them was poor (κ = 0.33-0.39). The visual ability to determine stenosis severity compared with Doppler echocardiography had high specificity (81% and 88% for observers 1 and 2). However, sensitivity was unacceptably low (56%-67%), and the positive predictive value was poor (44%-50%). Agreement was fair (κ= 0.58-0.69) between the observers for determining severe stenosis. CONCLUSIONS: Our results suggest that visual assessment of aortic valve calcium has high interobserver variability; the visual ability to determine severe aortic stenosis has low sensitivity but high specificity. Our results may have important implications for treatment of patients with aortic stenosis and guiding the use of handheld echocardiography. Further research with larger cohorts is needed to validate the variability, sensitivity, and specificity reported in our study.

Papillary fibroelastoma of the pulmonary valve--a systematic review.

The pulmonary valve is the least affected site for valvular papillary fibroelastoma. With increasing use of routine echocardiography and other modalities of imaging, pulmonary valve papillary fibroelastomas (PVPFE) are being recognized more frequently. PVPFE is more often an incidental diagnosis and symptomatic patients usually present with shortness of breath. Embolic phenomena and right ventricular outflow tract obstruction are the most serious complications of PVPFE. Since PVPFE is rare, the purpose of this systematic review is to address demographic characteristics, the clinical presentation, management, and outcome of this benign tumor of the pulmonary valve.

Valvular heart disease in patients with osteogenesis imperfecta.

Osteogenesis imperfecta (OI) or "brittle bone disease" is a rare connective tissue hereditary disorder. The most common clinical presentation of OI is bone fractures. OI also involves extraskeletal structures; however, cardiovascular manifestations are rare. In this report, we describe the cardiovascular anomalies of patients with OI who underwent valve surgery and review the literature on this subject.

Cardiac Masses (from a 15-Year Experience With 389 Surgical Cases).

Cardiac masses are highly heterogeneous and vary widely in their clinical presentation, imaging features, and survival outcomes. Our understanding is limited by their rarity and the fact that few are confirmed based on surgical pathology. We set out to provide a comprehensive analysis of all cardiac masses resected at our institution from 1999 to 2015, including imaging methods and histopathologic findings. We found papillary fibroelastomas (PFEs) to be the most commonly resected benign cardiac masses, followed by myxomas. Patients with PFEs most frequently presented with cerebrovascular accidents and transient ischemic attacks, whereas those with myxomas were more likely to present with arrhythmias and palpitations. In contrast, primary malignant cardiac masses were much rarer; angiosarcoma was the predominant subtype with a poor prognosis. Renal cell carcinomas were the most commonly discovered primary cancer for metastatic cardiac masses, and calcified amorphous tumors were the most prevalent non-neoplastic masses. For the detection of cardiac masses, transthoracic echocardiography was the most frequently used but least sensitive of the imaging methods analyzed. Transesophageal echocardiography (TEE) was the most sensitive imaging method. Fluorodeoxyglucose Positron Emission Tomography had similar sensitivity to TEE but was the least frequently used imaging method. Computed tomography and magnetic resonance imaging performed well in detecting most masses; PFEs, for which TEE was the most sensitive, was the exception. In conclusion, we found that PFEs were the most commonly resected benign cardiac masses, and TEE was the most accurate imaging method for the detection of all surgically removed masses at our institution.

Symptomatic pericardial cyst: a case series.

Pericardial cysts are most commonly located at the cardiophrenic angle or, rarely, in the posterior or anterior superior mediastinum. The majority of pericardial cysts are asymptomatic and are found incidentally. Symptomatic pericardial cysts present with dyspnoea, chest pain, or persistent cough. We describe four patients with symptomatic pericardial cysts who were treated with either echocardiographically guided percutaneous aspiration or video-assisted thoracoscopic surgery, or both; thoracotomy; or conservative therapy.

Emergent pulmonary thromboembolectomy and atrial septal aneurysmectomy for intracardiac impending paradoxical embolism: An en bloc approach to prevent clot embolism and facilitate repair.

Although a patent foramen ovale (PFO) is relatively common, confirmed reports of thrombus entrapped within a PFO are uncommon. Management of impending paradoxical embolism (IPE), also called a thrombus in transit, lacks consensus but includes systemic anticoagulation (e.g., heparin), systemic thrombolysis, or surgical thrombectomy. We present a case of IPE diagnosed with intraoperative transesophageal echocardiography (TEE) as well as a novel en bloc approach to atrial septal aneurysmectomy to minimize embolism and facilitate repair of the interatrial septum. Timely use of intraoperative TEE may aid in diagnosis and help guide the surgical approach to minimize embolic risk with an IPE.

Unusual Cause of Heart Failure in a Patient with Marfan Syndrome: A Late Complication of Bioprosthetic Valved Graft Replacement.

A high-velocity gradient across the left ventricular outflow tract is most often caused by aortic valve stenosis. We describe the unusual case of a high-velocity gradient caused by a kinked ascending aortic graft in a 69-year-old man who had Marfan syndrome. The patient had a history of ascending aortic aneurysm and had previously undergone replacement of the aortic root and ascending aorta with use of a bioprosthetic valved graft. The kinking was caused by dilation of the native aortic arch. The patient underwent successful hemi-arch replacement and repair of the kinked graft. Late complications and reoperation after proximal aortic surgery in patients with Marfan syndrome are rare, and a high-velocity left ventricular outflow tract gradient caused by the kinking of the aorta is unusual.

Echocardiography in centenarians: characteristics, utility and follow-up.

BACKGROUND: Echocardiography has been shown to be a valuable resource in the diagnosis of many cardiac conditions, and can be used in all age groups, from the fetus to the oldest old. In the context of an increasingly aging population, the impact and utility of echocardiography in centenarians is largely unknown. This study is to determine whether echocardiography in centenarians aids in making clinical patient management decisions. METHODS: A retrospective review of echocardiograms from 1986 to 2014, at two affiliated tertiary centers, in individuals who were 100 years or older at the time of the examination. Patient and echocardiogram characteristics, management decisions based on echocardiography, and mortality were documented. RESULTS: 114 centenarians had echocardiograms, with ages ranging from 100 to 107 years (101 ± 1.4 years). In 82 of the centenarians evaluated (72%), no changes in management occurred as a consequence of the echocardiogram. From all management changes directly related to the echocardiogram, 81% (n = 26) of these corresponded to medication adjustments; interventional or surgical procedures followed the echocardiogram only in 4% (n = 5) of the total number of centenarians. Echocardiogram-based changes in management were only significant in patients that were referred for congestive heart failure (P = 0.02). After the echocardiogram was performed, 1-month and 1-year mortality were 15% and 47%, respectively. The median survival after the echocardiogram was obtained was 13 months (range 0.03 to 145 months), with no difference if there was a change or no change in management (P = 0.21). CONCLUSIONS: Among centenarians undergoing echocardiography, despite additional diagnostic information, echocardiograms in centenarians influence management in a minority of cases, most commonly in the form of medication changes for treatment of heart failure. A significant proportion of centenarians are deceased within a year of undergoing echocardiographic assessment. These findings may question the overall utility of echocardiography in these late survivors.

Outcome after abnormal exercise echocardiography for patients with good exercise capacity: prognostic importance of the extent and severity of exercise-related left ventricular dysfunction.

OBJECTIVES: We sought to define the prognostic implications of the extent and severity of exercise echocardiographic abnormalities in patients with good exercise capacity. BACKGROUND; The exercise capacity of patients with known or suspected coronary artery disease (CAD) is of prognostic importance, as is the extent of exercise-related left ventricular (LV) hypoperfusion or dysfunction. METHODS: We examined the outcomes of 1,874 patients with known or suspected CAD (mean age 64 +/- 10 years, 64% men) who had good exercise capacity (> or = 5 metabolic equivalents [METs] for women, > or = 7 METs for men) but abnormal exercise echocardiograms and analyzed the potential association between clinical, exercise and echocardiographic variables and subsequent cardiac events. RESULTS: Multivariate predictors of time to cardiac death or nonfatal myocardial infarction (MI) were diabetes mellitus (risk ratio [RR] 1.88; 95% confidence interval [CI] 1.2 to 3.0), history of MI (RR 2.44; 95% CI 1.6 to 3.6) and an increase or no change in LV end-systolic size in response to exercise (RR 1.61; 95% CI 1.1 to 2.5). Using echocardiographic variables that were of incremental prognostic value, we were able to stratify the cardiac risk of the study population; cardiac death or nonfatal MI rate per person-year of follow-up was 1.6% for patients who had a decrease in LV end-systolic size in response to exercise (n = 1,330) and 1.2% for patients who did not have any severely abnormal LV segments immediately after exercise (n = 868). CONCLUSIONS: In patients with good exercise capacity, echocardiographic descriptors of the extent and severity of exercise-related LV dysfunction were of independent and incremental prognostic value. Stratification of patients into low- and higher risk subgroups was possible using these exercise echocardiographic characteristics.

Subepicardial aneurysm of the left ventricle.

Three patients are presented in whom a subepicardial aneurysm developed as a complication of a myocardial infarction. This aneurysm of the left ventricle is unusual and has 3 distinctive traits: an abrupt interruption of the myocardium that comprises the mouth and typically a narrow neck of the aneurysm; aneurysmal wall comprised of epicardium with or without a thin myocardial layer; and a propensity to rupture spontaneously regardless of the wall components or stage of development. All 3 patients were given a diagnosis using echocardiography and underwent immediate surgical aneurysmectomy. One patient died as a result of renal and respiratory failure postoperatively. The long-term outcome of the other 2 patients was favorable without evidence of additional cardiac rupture or instability.

Left anterior descending coronary artery stenosis: in a patient with takotsubo cardiomyopathy.

Takotsubo cardiomyopathy is characterized by chest pain, electrocardiographic abnormalities such as ST-segment elevation or depression, and elevated cardiac enzyme levels. Left ventriculography reveals transient akinesis of the involved segment of the myocardial wall (usually the left ventricular apex) and compensatory hyperkinesis of the noninvolved myocardium, which appears as apical ballooning during systole. Existing criteria for the diagnosis of takotsubo cardiomyopathy include the absence of obstructive coronary artery disease. Indeed, previous investigators have found incidental stenosis in only a minority of patients.Herein, we present the unusual case of an 84-year-old woman who sustained 4 episodes of takotsubo cardiomyopathy in 18 years. At the time of the initial episode, coronary angiography revealed no substantial stenosis. Concomitant with the 2nd episode, stenosis in the 1st obtuse marginal branch was treated with stenting. No new lesions were apparent after the patient's 3rd presentation, and the previously placed stent was patent. During the 4th (current) presentation, we detected and percutaneously treated severe stenoses in the patient's left anterior descending coronary artery and 2nd obtuse marginal branch.Although this report is of a single patient only, it definitively illustrates that severe coronary artery disease can occur in patients who have takotsubo cardiomyopathy. We recommend the thorough evaluation of possible coronary artery disease in high-risk patients, even upon the strong clinical suspicion of takotsubo cardiomyopathy.