The use of contrast echocardiography in the diagnosis of an unusual cause of congestive heart failure: achalasia

Extrinsic compression of the left atrium is a potentially life-threatening but unusual cause of congestive heart failure. Achalasia is a motility disorder characterized by impaired relaxation of the lower esophageal sphincter and dilation of the distal two-thirds of the esophagus. We report only the third known case in the world literature of massive left atrial compression by a dilated esophagus in a patient with achalasia. The use of contrast echocardiography with perflutren protein-type A microspheres allowed for differentiation between a compressive vascular structure and the esophagus. This resulted in prompt treatment leading to hemodynamic stability after nasogastric decompression and Botulinum toxin injection at the gastroesophageal junction.     

Metastatic signet ring adenocarcinoma: an unusual cause of cardiac constriction

Pericardial constriction secondary to metastatic adenocarcinoma is exceedingly rare. We present the first recorded case of pericardial constriction secondary to metastatic signet-ring mucinous adenocarcinoma diagnosed by echocardiography. The cornerstones of echocardiographic diagnosis of constriction are the following: interventricular septal bounce phasic with respiration, M-mode recordings of the inferior vena cava, and the characteristic Doppler velocity patterns recorded from the mitral valve, hepatic veins, and mitral annulus.     

Klippel–Feil syndrome: A very unusual cause of severe aortic regurgitation visualized by multimodality imaging

A 51‐year‐old man with Klippel–Feil syndrome (KFS) and immunodeficiency syndrome, status postintravenous immunoglobulin therapy, presented with shortness of breath. He was found to have severe aortic regurgitation in the setting of a trileaflet aortic valve with thickened leaflets and mild prolapse of the right coronary cusp with left ventricular dilation and borderline left ventricular ejection fraction. Although various cardiac anomalies have been described in KPS, otherwise unexplained severe aortic regurgitation has not been previously reported to the best of our knowledge. The patient underwent an uncomplicated surgical aortic valve replacement with a 25‐mm Medtronic Avalus pericardial tissue valve resulting in symptomatic improvement. Intra‐operative management and transesophageal echocardiography can be particularly challenging in KFS patients. We describe the first reported case of severe aortic regurgitation in KPS, review the cardiac anomalies associated with the syndrome, and highlight the clinical challenges in intra‐operative management of these patients.     

Aortic root thrombus complicated by left main coronary artery occlusion visualized by 3D echocardiography in a patient with continuous‐flow left ventricular assist device

Aortic root thrombus is an uncommon complication of continuous‐flow left ventricular assist devices (LVAD). We present the case of a 71‐year‐old man with ischemic cardiomyopathy who underwent destination therapy HeartMate II LVAD placement. Eighteen months later, he presented with a cerebrovascular accident followed by myocardial infarction. Transesophageal echocardiography revealed an aortic root thrombus spanning the left and noncoronary cusps and obliterating the left main coronary artery. We discuss the incidence, risk factors, and management of aortic root thrombus in LVAD patients. To our knowledge, this is the first report of three‐dimensional echocardiography used to characterize an LVAD‐associated aortic root thrombus.     

Fatal ascending aorta-to-right ventricle fistula formation after Staphylococcus aureus endocarditis of bicuspid aortic valve

We present a case of a 46-year-old man with advanced acquired immunodeficiency syndrome and congenitally bicuspid aortic valve endocarditis caused by methicillin- and gentamicin-resistant Staphylococcus aureus. Endocarditis led to root abscess formation, a complete heart block, and fistulous tract formation between the ascending aorta and the right ventricle. Although perivalvular abscess is not an unusual complication of native valve endocarditis, a fatal fistulous communication between the ascending aorta and the right ventricle is exceedingly rare.     

A novel and practical cardiovascular magnetic resonance method to quantify mitral annular excursion and recoil applied to hypertrophic cardiomyopathy

Background

We have developed a novel and practical cardiovascular magnetic resonance (CMR) technique to evaluate left ventricular (LV) mitral annular motion by tracking the atrioventricular junction (AVJ). To test AVJ motion analysis as a metric for LV function, we compared AVJ motion variables between patients with hypertrophic cardiomyopathy (HCM), a group with recognized systolic and diastolic dysfunction, and healthy volunteers.

Methods

We retrospectively evaluated 24 HCM patients with normal ejection fractions (EF) and 14 healthy volunteers. Using the 4-chamber view cine images, we tracked the longitudinal motion of the lateral and septal AVJ at 25 time points during the cardiac cycle. Based on AVJ displacement versus time, we calculated maximum AVJ displacement (MD) and velocity in early diastole (MVED), velocity in diastasis (VDS) and the composite index VDS/MVED.

Results

Patients with HCM showed significantly slower median lateral and septal AVJ recoil velocities during early diastole, but faster velocities in diastasis. We observed a 16-fold difference in VDS/MVED at the lateral AVJ [median 0.141, interquartile range (IQR) 0.073, 0.166 versus 0.009 IQR -0.006, 0.037, P < 0.001]. Patients with HCM also demonstrated significantly less mitral annular excursion at both the septal and lateral AVJ. Performed offline, AVJ motion analysis took approximately 10 minutes per subject.

Conclusions

Atrioventricular junction motion analysis provides a practical and novel CMR method to assess mitral annular motion. In this proof of concept study we found highly statistically significant differences in mitral annular excursion and recoil between HCM patients and healthy volunteers.