Left Ventricular Septal Hypertrophy in Elderly Patients With Aortic Stenosis.

OBJECTIVES: Left ventricular (LV) septal hypertrophy in aortic stenosis raises diagnostic and therapeutic questions. However, the etiology and clinical consequences of this finding have not been well studied. The aim of this study was to perform a morphologic evaluation of the LV in aortic stenosis and to investigate the contributing factors and consequences of septal hypertrophy. METHODS: Patients with moderate or severe aortic stenosis were prospectively enrolled. Patients with previous myocardial infarction, wall motion abnormalities, at least moderate valvular regurgitation, known cardiomyopathy, an LV ejection fraction of less than 50%, and age younger than 65 years were excluded. RESULTS: Forty-one patients underwent a final analysis. Septal hypertrophy (LV septal wall thickness ≥15 mm) was confirmed in 21 of 41 patients. The septal hypertrophy group had higher peak aortic valve velocity, a higher diabetes mellitus rate, and a higher rate and longer duration of hypertension than those without septal hypertrophy. The peak aortic valve velocity (odds ratio, 7.1; 95% confidence interval, 1.4-37.1) and diabetes mellitus (odds ratio, 7.4; 95% confidence interval, 1.2-46.2) were the significant factors associated with septal hypertrophy by multivariate analysis. Intraventricular conduction disturbance on electrocardiography was more frequent in the septal hypertrophy group (P = .021). CONCLUSIONS: Left ventricular septal hypertrophy was commonly observed in elderly patients with aortic stenosis, and a higher aortic valve velocity, hypertension, and diabetes mellitus were associated factors. Intraventricular conduction disturbance occurred more often in patients with septal hypertrophy than those without, which implies the pathophysiologic consequence. Further studies are needed to determine the impact of septal hypertrophy and intraventricular conduction disturbance on the prognosis of patients after aortic valve interventions.

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.

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.

Incidentally Discovered Right Atrial Mass: A Rare and Unexpected Etiology.

Primary cardiac sarcoma is a rare type of intracardiac mass. This report describes a patient with atrial flutter who had a new right atrial mass incidentally discovered on transesophageal echocardiography. A thrombus was suspected based on radiographic appearance, but there was minimal change with anticoagulation. The mass was resected and found to be an undifferentiated pleomorphic cardiac sarcoma, an uncommon sub-type within the already rare category of primary cardiac neoplasms. This report highlights the importance of considering primary malignancy and thoroughly correlating radiographic and clinical evidence during the diagnostic workup of patients with intracardiac masses.

Accurate guidance of a catheter by ultrasound imaging and identification of a catheter tip by pulsed-wave Doppler.

BACKGROUND: With the advent of numerous minimally invasive medical procedures, accurate catheter guidance has become imperative. We introduce and test an approach for catheter guidance by ultrasound imaging and pulsed-wave (PW) Doppler. METHODS: A steerable catheter is fitted with a small piezoelectric crystal at its tip that actively transmits signals driven by a function generator. We call this an active-tip (AT) catheter. In a water tank, we immersed a "target" crystal and a rectangular matrix of four "reference" crystals. Two-dimensional (2D) ultrasound imaging was used for initial guidance and visualization of the catheter shaft, and then PW Doppler mode was used to identify the AT catheter tip and guide it to the simulated target that was also visible in the 2D ultrasound image. Ten guiding trials were performed from random initial positions of the AT catheter, each starting at approximately 8 cm from the target. RESULTS: After the ten navigational trials, the average final distance of the catheter tip from the target was 2.4 ± 1.2 mm, and the range of distances from the trials was from a minimum of 1.0 mm to a maximum of 4.5 mm. CONCLUSIONS: Although early in the development process, these quantitative in vitro results show promise for catheter guidance with ultrasound imaging and tip identification by PW Doppler.

Predictors for the development of severe tricuspid regurgitation with anatomically normal valve in patients with atrial fibrillation.

BACKGROUND AND AIMS: Atrial fibrillation (AF) may be a risk factor for severe functional tricuspid valve regurgitation (FTR). We aimed to determine the predictors of severe FTR in patients with AF. METHODS AND RESULTS: From our echocardiographic laboratory database, we searched for and reviewed the medical records of consecutive patients with severe FTR and AF seen at Mayo Clinic in Arizona from 2002 through 2009. Our search identified 42 patients who met all inclusion criteria. These patients (cases) with severe FTR and AF were compared with 38 patients (controls) with AF who had no greater than mild tricuspid regurgitation. Case patients with severe FTR were older than controls (mean, 81 years vs. 76 years; P < 0.001) and more frequently had chronic AF (69% vs 26%; P < 0.001). Mean right atrial volume (86 mL/m(2) vs 46 mL/m(2) ; P < 0.001), right ventricular volume (42 mL ± 33 mL vs 22 mL ±8 mL; P < 0.001) and tricuspid annular diameter (3.6 cm vs 3.0 cm; P < 0.001) were larger in cases than in controls. Patients with severe FTR also had a higher prevalence of right-sided heart failure (69% vs 16%; P < 0.001). After adjusting for age and gender, right atrial and right ventricular volumes were independent predictors for the development of severe FTR in patients with AF (odds ratio, 1.7 [95% CI, 1.3-2.8] for every 10 mL/m(2) increase in right atrial volume; P = 0.0002 and odds ratio, 3.1 [95% CI, 1.5-8.9] for every 10 mL increase in right ventricular volume; P = 0.0002). CONCLUSIONS: Severe FTR occurs in older patients with chronic AF as a result of marked right atrial and right ventricular dilatation; and enlargement of the tricuspid annulus in the absence of pulmonary hypertension. More importantly, severe FTR leads to increased prevalence of right-sided heart failure underscoring the nonbenign nature of chronic AF.

Optogenetic Control of Engrafted Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes in Live Mice: A Proof-of-Concept Study.

Background: Cellular transplantation has emerged as promising approach for treating cardiac diseases. However, a poor engraftment rate limits our understanding on how transplanted cardiomyocytes contribute to cardiac function in the recipient's heart. Methods: The CRISPR/Cas9 technique was employed for stable and constitutive gene expression in human-induced pluripotent stem-cell-derived cardiomyocytes (hiPSC-CMs). Myocardial infarction was induced in adult immunodeficient mice, followed by intramyocardial injection of hiPSC-CMs expressing either CCND2/channelrhodopsin 2 (hiPSC-CCND2OE/ChR2OECMs) or CCND2/luciferase (hiPSC-CCND2OE/LuciOECMs). Six months later, hemodynamics and intramural electrocardiogram were recorded upon blue light illuminations in anesthetized, open-chest mice. Results: Blue light resets automaticity of spontaneously beating hiPSC-CCND2OE/ChR2OECMs in culture, but not that of hiPSC-CCND2OE/LuciOECMs. Response to blue light was also observed in mice carrying large (>106 cells) intracardiac grafts of hiPSC-CCND2OE/ChR2OECM but not in mice carrying hiPSC-CCND2OE/LuciOECMs. The former exhibited single premature ventricular contractions upon light illumination or ventricular quadrigeminy upon second-long illuminations. At the onset of premature ventricular contractions, maximal systolic ventricular pressure decreased while ventricular volume rose concomitantly. Light-induced changes reversed upon resumption of sinus rhythm. Conclusions: We established an in vivo model for optogenetic-based modulation of the excitability of donor cardiomyocytes in a functional, reversible, and localized manner. This approach holds unique value for studying electromechanical coupling and molecular interactions between donor cardiomyocytes and recipient hearts in live animals.

Tricuspid Regurgitation Impact on Outcomes (TRIO): A Simple Clinical Risk Score.

OBJECTIVE: To determine which clinical variables infer the highest risk for mortality in patients with notable tricuspid regurgitation (TR) and to develop a clinical assessment tool (the Tricuspid Regurgitation Impact on Outcomes [TRIO] score). PATIENTS AND METHODS: A single-center retrospective cohort of 13,608 patients with undifferentiated moderate to severe TR at the time of index echocardiography between January 1, 2005, and December 31, 2016, was included. Baseline demographic and clinical data were obtained. Patients were randomly assigned to a training (N=10,205) and a validation (N=3403) cohort. Median follow-up was 6.5 years (interquartile range, 0.8 to 11.0 years). Variables associated with mortality were identified by Cox proportional hazards methods. A geographically distinct cohort of 7138 patients was used for further validation. The primary end point was all-cause mortality over 10 years. RESULTS: The 5-year probability of death was 53% for moderate TR, 63% for moderate-severe TR (hazard ratio [HR], 1.24 [95% CI, 1.17 to 1.31]; P<.001 vs moderate), and 71% for severe TR (HR, 1.55 [95% CI, 1.47 to 1.64]; P<.001 vs moderate). Factors associated with all-cause mortality on multivariate analysis included age 70 years or older, male sex, creatinine level greater than 2 mg/dL, congestive heart failure, chronic lung disease, aspartate aminotransferase level of 40 U/L or greater, heart rate of 90 beats/min or greater, and severe TR. Variables were assigned 1 or 2 points (HR, >1.5) and added to compute the TRIO score. The score was associated with all-cause mortality (C statistic = 0.67) and was able to separate patients into risk categories. Findings were similar in the second, independent and geographically distinct cohort. CONCLUSION: The TRIO score is a simple clinical tool for risk assessment in patients with notable TR. Future prospective studies to validate its use are warranted.

Atypical presentation of takotsubo cardiomyopathy.

Midventricular ballooning syndrome, an atypical variant of takotsubo cardiomyopathy (TCM), is characterized by transient wall motion abnormalities of the midsegment of the left ventricle with apical sparing. In contrast to the typical form of TCM, apical contractility is preserved and may even be hyperkinetic in midventricular TCM. We present a case of atypical TCM in an 86-year-old woman who had chest pain while accompanying her husband in the emergency department as he was evaluated for chest pain.

Anterolateral papillary muscle rupture: an unusual complication of septic coronary embolism.

In most cases, acute mitral valve regurgitation in the setting of infective endocarditis is caused by the destruction of either the mitral valve leaflets or the chordal apparatus. A 54-year-old woman had development of respiratory failure due to pulmonary oedema from severe acute mitral valve regurgitation in the setting of acute bacterial endocarditis. She was found to have a ruptured anterolateral papillary muscle from occlusion of the circumflex artery by embolic vegetations arising from the aortic valve. Although this occurrence is uncommon, an embolic phenomenon resulting in myocardial infarction and subsequent rupture of papillary muscle must be considered as a cause of acute severe mitral valve regurgitation.

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.

Occurrence of left-sided heart valve involvement before right-sided heart valve involvement in carcinoid heart disease.

Carcinoids are rare neuroendocrine tumours that occur primarily in the gastrointestinal tract. Carcinoid heart disease is characterized by fibrous plaque deposition on the endocardial surface of the cardiac valves and chambers. It affects the right heart valves in 85% of cases and the left heart valves in 15%. We present an unusual case of a patient with metastatic carcinoid heart disease in whom typical carcinoid aortic and mitral valve lesions developed 2 years prior to the development of severe right-sided carcinoid valvular heart disease.

Takotsubo cardiomyopathy in a patient with pulmonary embolism.

Takotsubo cardiomyopathy is an acute cardiac syndrome characterized by transient regional wall motion abnormalities of the left ventricular apex or midventricle. Patients often present with chest pain or dyspnoea, ST-segment elevation, and minor elevations of cardiac enzyme levels. Takotsubo cardiomyopathy has been associated with severe emotional or physical stress such as severe burns, spinal cord injury, subarachnoid haemorrhage, and multiple traumas. We report a case of takotsubo cardiomyopathy in a 79-year-old woman with pulmonary embolism. Although pulmonary embolism has been listed as a potential cause of takotsubo cardiomyopathy, this is the first case reported with this association.

Potential role of Reynolds number in resolving Doppler- and catheter-based transvalvular gradient discrepancies in aortic stenosis.

BACKGROUND AND AIM OF THE STUDY: Discrepancies in mean transvalvular gradient have been observed between Doppler echocardiography and catheter-based techniques in the assessment of aortic stenosis (AS). The Reynolds number (RE) has been shown to influence Doppler-derived gradients, and may be useful in resolving Doppler- and catheter-based gradient discrepancies in AS. The study aim was to assess the influence of the RE on such discrepancies. METHODS: A pulsatile in-vitro heart model using a bioprosthetic aortic valve with leaflets sutured together was used to simulate AS. Simultaneous gradients were measured using Doppler echocardiography and high-fidelity catheters while the RE was varied, by testing solutions of different density and viscosity across a range of cardiac outputs. RESULTS: The echocardiographic and catheter-derived mean gradient (MG) values were correlated (r = 0.89; p < 0.0001); however, significant differences in the MG were observed across hemodynamic states. A direct linear relationship was identified between RE and the absolute difference in MG measured using the two techniques (r = 0.94, p < 0.0001). Relative to catheter-based measurements, the MG was underestimated by Doppler (range: 13-16 mmHg) at low RE (median 6,999) and overestimated (7-33 mmHg) at high RE (median 34,268). However, agreement between catheter- and Doppler-derived gradients was within 5 mmHg at intermediate RE (median 17,284) (p < 0.0001). CONCLUSION: The underestimation of Doppler-derived MGs at low RE relative to catheter-based measurements may be due to an exclusion of viscous friction from the simplified Bernoulli equation, while the overestimation of Doppler-derived MGs at high RE may be due to a pressure recovery effect. However, within an intermediate range of RE, where the effects of viscous and inertial forces are balanced, the agreement between catheter- and Doppler-derived gradients was excellent.

An unusual presentation of hemolytic anemia in a patient with prosthetic mitral valve.

Although rare, periprosthetic valvular regurgitation can cause hemolytic anemia. We present the case of a 63-year-old man who had an unusual presentation of hemolytic anemia due to periprosthetic mitral valve regurgitation (PMVR) in the presence of cold agglutinins. Due to high surgical risk, PMVR was percutaneously closed with three Amplatzer devices under the guidance of three-dimensional transesophageal echocardiography.

Asymptomatic chronic severe organic mitral regurgitation: contemporary management strategies.

Abstract: Chronic organic mitral regurgitation resulting from mitral valve prolapse is increasing in prevalence due to aging of the population. Although "watchful waiting" has been the conventional standard for many years in patients with minimal or no symptoms and chronic severe organic mitral regurgitation, several factors including changing etiology, better understanding of the natural history, improved diagnosis and advances in surgical techniques are now paving the way for earlier mitral valve surgery. The ability to successfully repair the myxomatous mitral valve with low operative risk (< 1%) and advances in imaging in the recent years have led to a major change in the treatment paradigm for organic mitral regurgitation. It is clear from the preponderance of evidence that mitral valve repair in asymptomatic patients with preserved ejection fraction and severe organic mitral regurgitation leads to "cure" or "suppression" of the disease, underscoring the need for early surgery. Whether further advances in imaging, robotic surgery, and percutaneous mitral valve repair lead to even better outcomes remains to be seen.

Aortic tumor or mobile thrombus?

Isolated large mobile mass in the thoracic aorta can be due to thrombus or, rarely, aortic tumor. We report the case of a 61-year-old man with no history of medical problems presenting with neurologic deficits and in whom a large mobile echogenic mass in the distal aortic arch was found with transesophageal echocardiography. Given his few cardiovascular risk factors and absence of other systemic symptoms, he received anticoagulant therapy. Subsequent resolution of the aortic mass suggested a diagnosis of thrombus. This case illustrates an unusual manifestation of aortic arch atherosclerosis and underscores the utility of transesophogeal echocardiography for patients with ischemic stroke.

A Case of Severe Mitral Valve Regurgitation in a Patient with Leadless Pacemaker.

An 85-year-old man with cardiac history notable for atrial fibrillation diagnosed 10 years ago which was being treated with atenolol and warfarin presented to our institution with persistent atrial fibrillation. His echocardiogram showed ejection fraction (EF) of 56%, no regional wall motion abnormalities, mild mitral and pulmonary regurgitation, and trivial tricuspid regurgitation. Despite this treatment, he had recurrent episodes of paroxysmal symptomatic atrial fibrillation with a rapid rate requiring multiple emergency department visits and hospital admissions. Given difficulty to control the rate, he underwent atrioventricular (AV) nodal ablation and leadless pacemaker insertion. Fifteen days after the procedure, he was found to have a severe mitral regurgitation murmur.

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.

Cerebral ischemic events associated with 'bubble study' for identification of right to left shunts.

BACKGROUND AND PURPOSE: Detection of an intracardiac shunt is frequently sought during the evaluation of patients with cryptogenic ischemic stroke and agitated saline intravenous injection, or "bubble study" (BS), is performed in most cases. We present the first attempt to identify the clinical features in patients who had cerebral ischemic events with BS. METHODS: Using a list serve established by the American Academy of Neurology, a member posted a question regarding the safety of BS in patients with patent foramen ovale. A standardized questionnaire was used to gather data about patients with cerebral ischemic events, details of each case were reviewed, and the findings pooled. RESULTS: Five patients with ischemic complications of BS (all female, aged 42 to 90 years) were identified from 4 institutions, 3 ischemic strokes and 2 transient ischemic attacks. Events occurred either during or within 5 minutes of BS. Early brain MRIs confirmed acute infarction in 3, including one who had transient symptoms. MRI infarct volumes were small, and deficits were mild in those who developed stroke. Diagnostic evaluation revealed a patent foramen ovale alone in one case, a pulmonary arteriovenous malformation in one case, and a patent foramen ovale and/or pulmonary shunt in 3 cases. CONCLUSIONS: Ischemic cerebrovascular complications can occur in patients who undergo BS and are associated with the presence of cardiac or pulmonary shunts. The true incidence and degree of disability remains unknown, and further study is indicated to assess the impact of technical differences in BS methodology. Novel methods to promote physician communication such as the use of electronic list serves may reduce barriers to reporting of drug, technique, or device complications and should be explored to identify rare complications that otherwise will likely go unappreciated.