A novel two-dimensional echocardiographic finding in cardiac amyloidosis.

BACKGROUND: A number of echocardiographic findings characteristic of cardiac amyloidosis (CA) have been described, each with limitations. METHODS: A distinctive wall motion pattern of preserved myocardial thickening at left ventricular apex with hypokinesis in basal and midsegments was observed in two patients with biopsy proven CA. Following this observation, endomyocaradial biopsy files beginning in 2007 were reviewed. Seven consecutive patients with documented CA were identified. Two-dimensional (2D) echocardiograms for each were reviewed in consensus by two experienced echocardiographers. Clinical and electrocardiographic data were obtained from chart review. RESULTS: All patients were men with class II-IV heart failure. Six had light chain CA, 1 senile CA. Six patients had coronary angiography. One had a 60% left anterior descending coronary artery stenosis. Five had nonobstructive disease. Echocardiograms for all seven patients demonstrated the distinctive pattern of preserved myocardial thickening at apex with hypokinesis in basal and midsegments. Reduced ejection fraction was present in six and increased wall thickness and myocardial echogenicity in seven. Other echo signs of amyloid were variably present. Three had low voltage on electrocardiogram. CONCLUSION: A distinctive 2D echocardiographic pattern of preserved segmental wall motion at left ventricular apex with hypokinesis in basal to midsegments was consistently identified in seven consecutive patients with endomyocardial biopsy-proven CA.

Pseudoaneurysm with thrombus and left ventricular inflow obstruction after left circumflex stenting.

A 61-year-old woman had stenting of the left circumflex coronary artery. She had a repeat coronary angiogram the day after stenting because of hypotension and orthopnea. The left circumflex stent was patent. A transesophageal echocardiogram showed a 2.5 cm x 3.0-cm mass in the atrioventricular groove compressing the left atrium. A pseudoaneurysm with thrombus and left ventricular inflow obstruction was diagnosed. The patient was observed for 48 hours to allow the pseudoaneurysm to seal and coagulate. She then had surgical evacuation of the thrombus, which had caused her hypotension and orthopnea by compression of the left atrium.

Aortic valve commissural tear mimicking type A aortic dissection.

A 52-year-old man came to the local emergency department with symptoms of heart failure and transient chest pain. Transthoracic echocardiography showed severe aortic regurgitation and a dilated ascending aorta. Aortic dissection was suspected, and he was transferred to our institution. Transesophageal echocardiography appeared to confirm the presence of a type A dissection. A mobile, linear structure was present in the proximal ascending aorta, suggesting the presence of dissection flap. Aortic cusp prolapse and severe aortic regurgitation were seen. At surgery, no aortic dissection was present. Rather, the commissure between right and left aortic valve cusps was separated from the wall of the aorta. Motion of the torn commissure with the cardiac cycle apparently led to the transesophageal echocardiographic appearance described. The ascending aorta was dilated. Histopathologic examination of the aorta confirmed the visual appearance of cystic medial necrosis. Aortic valve commissural tear is a rare event, which may lead to severe aortic regurgitation. This entity may lead to the false-positive transesophageal echocardiographic diagnosis of type A dissection.

The Organization of Projections from Subdivisions of the Auditory Cortex and Thalamus to the Auditory Sector of the Thalamic Reticular Nucleus in Galago.

Anterograde and retrograde transport techniques were used to study the connexions between different subdivisions of the auditory cortex and thalamus with the thalamic reticular nucleus in the prosimian, Galago. In particular, the goal was to determine whether the primary auditory nucleus, GMv, and its cortical target, area I of the auditory cortex (A I), project to a different region of the auditory sector of the reticular nucleus from the secondary auditory nuclei, GMmc and Po and their cortical targets outside A I. The results show that the projections to and from the auditory sector are indeed segregated: injections of wheatgerm agglutinin-conjugated horseradish peroxidase into either GMmc or Po labelled cells and terminals along the medial, lateral and ventral borders of the auditory sector, forming a U-shaped pattern. Projections from area II of the auditory cortex produced almost an identical pattern of the terminal labelling in the auditory sector. In contrast, injections into GMv-labelled cells and terminals in the centre region of the auditory sector, in the 'interior' of the U-shaped region. Projections from A I were distributed to both the U-shaped border region and the central core of the auditory sector probably because A I received projections from GMmc, Po and GMv. The significance of these results depends on a comparison between the auditory and visual sectors of the reticular nucleus. Both sectors are divided into tiers or subsectors-one related to the primary relay nucleus, i.e. GLd or GMv, and the other related to the secondary relay nuclei, i.e. pulvinar nucleus, GMmc, Po, etc.