Jayshree Joshi

Cardiovascular Magnetic Resonance in Cardiac Amyloidosis

Background—Cardiac amyloidosis can be diagnostically challenging. Cardiovascular magnetic resonance (CMR) can assess abnormal myocardial interstitium. Methods and Results—Late gadolinium enhancement CMR was performed in 30 patients with cardiac amyloidosis. In 22 of these, myocardial gadolinium kinetics with T1 mapping was compared with that in 16 hypertensive controls. One patient had CMR and autopsy only. Subendocardial T1 in amyloid patients was shorter than in controls (at 4 minutes: 427±73 versus 579±75 ms; P<0.01), was shorter than subepicardium T1 for the first 8 minutes (P≤0.01), and was correlated with markers of increased myocardial amyloid load, as follows: left ventricular (LV) mass (r=−0.51, P=0.013); wall thickness (r=−0.54 to −0.63, P<0.04); interatrial septal thickness (r=−0.52, P=0.001); and diastolic function (r=−0.42, P=0.025). Global subendocardial late gadolinium enhancement was found in 20 amyloid patients (69%); these patients had greater LV mass (126±30 versus 93±25 g/m2; P=0.009) than unenhanced patients. Histological quantification showed substantial interstitial expansion with amyloid (30.5%) but only minor fibrosis (1.3%). Amyloid was dominantly subendocardial (42%) compared with midwall (29%) and subepicardium (18%). There was 97% concordance in diagnosis of cardiac amyloid by combining the presence of late gadolinium enhancement and an optimized T1 threshold (191 ms at 4 minutes) between myocardium and blood. Conclusions—In cardiac amyloidosis, CMR shows a characteristic pattern of global subendocardial late enhancement coupled with abnormal myocardial and blood-pool gadolinium kinetics. The findings agree with the transmural histological distribution of amyloid protein and the cardiac amyloid load and may prove to have value in diagnosis and treatment follow-up.

Cardiac abnormalities in systemic lupus erythematosus. Association with raised anticardiolipin antibodies.

Two-dimensional echocardiographic studies were prospectively performed in 93 patients with systemic lupus erythematosus (SLE) to discover the incidence and spectrum of cardiac abnormalities and to relate these findings to the presence of high levels of anticardiolipin antibodies. Assessment of the intracardiac anatomy was also performed in an additional 12 patients who had increased anticardiolipin antibody levels but did not have SLE. Fifty patients (54%) with SLE had cardiac abnormalities, and 43 patients (46%) had normal hearts. Three categories of cardiac abnormalities were identified--valvular lesions, ranging from vegetations to valvular thickening, were found in 28%, pericardial effusion or thickening was found in 20%, and regional or global left ventricular dysfunction was found in 5%. High levels of anticardiolipin antibodies were detected in 50 patients (54%) with SLE. Of those, only 11 (22%) had an entirely normal heart, whereas the remaining 39 (78%) had at least one cardiac abnormality (valvular lesions in 20, pericardial effusion in 15, and myocardial dysfunction in five patients). In patients with SLE, the presence of abnormal intracardiac anatomy was strongly associated with increased levels of anticardiolipin antibodies (p less than 0.0001). The overall sensitivity and specificity of high levels of anticardiolipin antibodies in the prediction of cardiac abnormalities was 78% and 74%, respectively, with a positive predictive accuracy of 78% and a negative predictive accuracy of 74%. Eight of the 12 patients (67%) who had increased anticardiolipin antibodies but whose disease did not fulfill the American Rheumatism Association classification criteria for SLE had cardiac abnormalities similar to those in patients with SLE compared with only four (33%) who had normal hearts (p less than 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

Detection of atherosclerotic lesions in the aorta by transesophageal echocardiography

Transesophageal echocardiographic studies were prospectively performed in 152 consecutive patients older than age 40 years referred to the echocardiography laboratory to assess the prevalence of atherosclerosis in the thoracic aorta and relate this to a history of systemic embolization. Forty-four patients (29%) had at least 1 atherosclerotic lesion in the thoracic aorta. This was associated with a higher prevalence of coronary artery disease (78% of all patients with coronary artery disease), carotid artery disease (88% of all patients with carotid artery disease) and peripheral vascular disease (all symptomatic patients). Forty-two of all patients (28%) had systemic emboli, 20 (48%) of whom had at least 1 atheromatous lesion in the thoracic aorta. Conversely, only 24 of 110 patients (22%) without previous systemic emboli had atheromatous lesions (p < 0.001). It is concluded that atherosclerotic lesions in the thoracic aorta can readily be identified with transesophageal echocardiography. The detection of atherosclerotic plaques of the aorta represents a marker of diffuse atherosclerotic disease, often associated with carotid, coronary and peripheral vascular disease and with the occurrence of systemic emboli. Transesophageal echocardiography may be used serially to investigate whether dietary or pharmacologic maneuvers, or both, can shrink established atherosclerotic plaques in the thoracic aorta.

Magnitude of myocardial dysfunction is greater in painful than in painless myocardial ischemia: An exercise echocardiographic study

OBJECTIVES This study sought to assess the presence and extent of inducible myocardial dysfunction during painful and painless (silent) myocardial ischemia in a homogeneous patient cohort with coronary artery disease and no previous myocardial infarction. BACKGROUND The functional significance of painless versus painful demand-driven ischemia remains controversial, with conflicting results in published reports regarding the amount of myocardium in jeopardy. METHODS Exercise echocardiography was performed in 89 patients (mean [+/- SD] age 59.3 +/- 8.2 years) with significant coronary artery disease and positive exercise stress test results. Patients were taking no antianginal medications and were classified into painless and painful cohorts after the outcome of a symptom-limited treadmill exercise test. No patients had previous coronary artery bypass surgery. Images were acquired in digital format before and immediately after treadmill exercise testing. RESULTS Fifty-eight patients had painful and 31 painless myocardial ischemia. Clinical and demographic characteristics as well as coronary artery anatomy were similar in both groups. Patients with painless ischemia achieved better exercise performance with greater exercise duration (p < 0.001) and higher maximal rate-blood pressure product (p < 0.001) than those with painful ischemia. New wall motion abnormalities were seen in 54 patients (93%) with painful versus 17 (55%) with painless ischemia (p < 0.001). Total ischemic score was greater in patients with painful than in those with painless ischemia (15.9 +/- 3.7 vs. 12 +/- 1.4, p < 0.001, respectively), with a greater number of ischemic myocardial segments in painful than in painless ischemia (101 [16%] vs. 21 [6%], p < 0.001, respectively). CONCLUSIONS Patients with painless ischemia frequently have regional myocardial dysfunction on exertion detected by echocardiography, but painful episodes are accompanied by a greater magnitude of myocardial dysfunction.

Evaluation of dipyridamole-Doppler echocardiography for detection of myocardial ischemia and coronary artery disease☆

Doppler assessment of left ventricular filling and ejection during dipyridamole stress may supplement wall motion analysis for detection of myocardial ischemia and coronary artery disease (CAD). Thirty-four patients taking no cardioactive therapy were studied using intravenous dipyridamole (0.6 mg/kg) during 2-dimensional and pulsed Doppler echocardiography. Twelve patients had normal coronary arteries (group 1) and the remainder, who had significant CAD, were divided into groups 2 (n = 11) and 3 (n = 11). Only subjects in group 2 developed myocardial ischemia manifest as reversible regional asynergy and ST-segment depression. Heart rate increased (16 +/- 9 beats/min, p less than 0.01) and mean blood pressure decreased (-5 +/- 8 mm Hg, p = not significant) uniformly across groups. Exaggerated hyperkinesia of normally contracting wall segments was the common response to dipyridamole infusion in patients with CAD. The respective mean percent changes in peak early diastolic velocity, peak atrial velocity, their ratio and ejection peak velocity, and mean acceleration for groups 1 (20, 42, -13, 20 and 23%), 2 (22, 32, -2, 10 and 14%) and 3 (23, 33, -6, 16 and 18%) were similar. Comparisons between normal patients and those with CAD and between groups 2 and 3 revealed no significant differences in the effect of dipyridamole on any variable. However, a decrease in both peak velocity and mean acceleration of left ventricular ejection was seen in 3 of 4 group 2 patients who developed severe ischemia. Dipyridamole-Doppler echocardiography is insensitive for detection of CAD and appears unable to identify myocardial ischemia unless this is severe. Hemodynamic changes and compensatory wall motion induced by dipyridamole may explain these findings.

Cardiac responses assessed by echocardiography to changes in preload in hypertrophic cardiomyopathy

Abstract The features of a small left ventricle with diastolic dysfunction and variable left ventricular outflow tract obstruction 1 suggest that cardiac function in hypertrophic cardiomyopathy (HC) may be particularly sensitive to change in preload. However, there is little information concerning clinically relevant fluid manipulation in HC. We have previously shown that stroke volume, at supine rest and during upright exercise, did not change despite significant changes in preload, indicating that patients with HC were functioning on the plateau of the Frank-Starling curve. 2 The current study extends these observations by examining the effect of the same fluid manipulations on 2-dimensional and Doppler echocardiography measurements of cardiac function in patients with HC compared with normal subjects.

Beat-to-beat variability in stroke volume during VVI pacing as predictor of hemodynamic benefit from DDD pacing.

To determine whether the magnitude of Beat‐to‐Beat variability in stroke volume (SVJ during VVI pacing can predici hemodynamic benefit from DDD pacing, we undertook Doppier recordings of systolic and diastolic LV flow during VVI and DDD pacing in 20 patients (age 54 ± 9 years)with DDD pacemakers implanted due to AV block. SV increased by 19%± 10% from VVI to DDD (P < 0.01). This increase was greater (29%± 9%)in patients with a ratio of early (E)/late (A) filling < 1 compared to those with E/A > 1 (10%± 9%) (P < 0.001). Beat‐to‐Beat variability in SV was greater in VVI (13%± 8%)compared to DDD (4%± 1%) (P < 0.001). Patients with E/A < 1 showed greater Beat‐to‐Beat variability in SV during VVI pacing (19 ± 6%)compared to those with E/A > 1 (8%± 4%) (P < 0.001). Beat‐to‐Beat variability in SV during VVI pacing correlated with both percent change in SV from VVI to DDD (r = 0.89, P < 0.001)and E/A (r = ‐0.71, P < 0.001). In conclusion, patients with E/A < 1 derive greater hemodynamic benefit at rest from DDD pacing compared with E/A > 1. In addition, patients with complete AV block who show large variations in SV during VVI pacing may obtain greater hemodynamic benefit at rest from DDD pacing than patients with small variations.

Intermittent Loss of Second Heart Sound

A 48-year-old man with relapsed multiple myeloma developed peripheral neuropathy after a second autologous stem-cell transplantation and was referred to the UK National Amyloidosis Centre for evaluation of possible amyloidosis. There was no history to suggest cardiovascular involvement; specifically, there was no history of breathlessness or syncopal episodes, although the patient had developed atrial fibrillation during the third week after stem-cell transplantation. The patient’s treatment included amiodarone but not digoxin.1 He had developed myeloma-related renal failure and had been receiving dialysis for 6 months. On …

Intraventricular Systolic Flow Mapping in Hypertrophic Cardiomyopathy

Ninety‐two consecutive patients with hypertrophic cardiomyopathy were studied with pulsed and continuous‐wave Doppler and color flow imaging to assess the intraventricular systolic flow profile from apex to base and compare it with that obtained in normals and in patients with aortic stenosis and systemic hypertension. Hypertrophic cardiomyopathy patients had higher intraventricular blood flow velocities (cm/sec) from apex to base compared with normals and aortic stenosis and systemic hypertension patients (apex: 41.5 ± 17.3 vs 24 ± 1.9,26.1 ± 2.9, and 26.4 ± 3.3; papillary muscles: 95.4 ± 66.5 vs 41.9 ± 4.9, 46.2 ± 3.4, and 46.4 ± 5.7; outflow tract: 249.3 ± 176.2 vs 66.9 ± 8.4, 64.1 ± 10.8, and 66 ± 9.5, respectively) (P < 0.001). Eighty‐six (93%) hypertrophic cardiomyopathy patients showed an abnormal intraventricular systolic color flow pattern at one or more sites but none of the patients with aortic stenosis or systemic hypertension or normal controls. Of those, 65 (71%) showed one or more variant (mosaic) flow, all of whom had intraventricular gradients, while 75 showed abnormal aliased flow at a site other than the subaortic area. It is concluded that patients with hypertrophic cardiomyopathy often exhibit an abnormal spatial distribution of the intraventricular systolic flow velocity profile compared with normals and patients with secondary forms of ventricular hypertrophy that can readily be recognized with color flow imaging. This could improve the sometimes difficult separation of hypertrophic cardiomyopathy patients from secondary hypertrophy.

Xamoterol in hypertrophic cardiomyopathy: effects on diastolic function and heart rate

The aims of this study were to determine the effects of the partial beta 1-adrenergic agonist, xamoterol, on diastolic function and ambulatory heart rate in hypertrophic cardiomyopathy. Eleven patients with non-obstructive hypertrophic cardiomyopathy were studied with cross-sectional and Doppler echocardiography and 24-h Holter monitoring before and after a single intravenous dose of xamoterol. Resting heart rate (mean +/- SD) increased from 76 +/- 16 before, to 83 +/- 15 beats/min 15 min after xamoterol, p = 0.03. In the 4-h period after xamoterol, maximum heart rate was reduced (127 +/- 21 to 112 +/- 19, p = 0.01) and minimum heart increased (60 +/- 16 to 67 +/- 17, p = 0.04) compared to the same 4-h period of the previous day. There were no significant changes in cross-sectional or Doppler echocardiographic measurements of left ventricular function following xamoterol. Xamoterol stabilises the heart rate in hypertrophic cardiomyopathy. The absence of a significant effect on Doppler measurements does not preclude a beneficial effect on diastolic function. This initial study suggests that xamoterol should be further investigated as a new medical therapy for hypertrophic cardiomyopathy.