Vascular abnormalities in primary amyloidosis.

AIMS: Primary amyloidosis (AL) is a systemic disease; however, there is limited information regarding the presence and character of vascular abnormalities. METHODS AND RESULTS: Validated ultrasound techniques were used to prospectively determine carotid artery intimal-medial thickness (IMT) and brachial artery flow-mediated dilatation (FMD) in 59 consecutive AL patients and 17 age-similar, healthy, asymptomatic volunteers (CON). Carotid IMT was increased in AL when compared with CON (0.07 +/- 0.02 vs. 0.04 +/- 0.01 mm, P < 0.01). Similarly, brachial artery FMD was significantly lower in AL when compared with CON subjects (3 +/- 7 vs. 12 +/- 8%, P < 0.01). Multivariable analysis revealed that AL was associated with larger IMT and lower FMD after controlling for several confounding variables. However, within AL cases, there was not a significant association of cardiac vs. non-cardiac involvement with IMT or FMD (P = 0.1 and 0.2, respectively). CONCLUSION: AL is associated with abnormal vascular morphology and endothelial dysfunction. Vascular abnormalities do not appear to be related to echocardiographic evidence of cardiac involvement.

Echocardiographically guided pericardiocentesis for treatment of clinically significant pericardial effusion in rheumatoid arthritis.

OBJECTIVE: To assess the safety and efficacy of echocardiographically guided pericardiocentesis for patients with rheumatoid arthritis (RA) and hemodynamically significant pericardial effusion. METHODS: We identified 16 patients with RA who underwent 18 echocardiographically guided pericardiocentesis procedures at our institution over a 20-year period. Clinical and laboratory characteristics of the patients, response to treatment, complications, and need for future pericardial surgery were abstracted from the echocardiography database. RESULTS: Ten patients were men and 6 were women (mean age, 62 yrs; range, 36-75 yrs). On average, patients were diagnosed with RA 11 years before pericardial disease developed. Twelve of 15 patients were seropositive for rheumatoid factor, 10 patients had radiographic evidence of erosions, and 7 patients had rheumatoid nodules. Cardiac tamponade was present in 11 of the 18 cases. Mean volume drained on the first pericardiocentesis was 504 +/- 264 ml (range 120-1000 ml). The fluid was an exudate with a mean protein concentration of 5 g/dl (range 3.3-51.1 g/dl). All cultures and cytologic findings were negative for bacteria and neoplastic cells. No serious complications resulted from echocardiographically guided pericardiocentesis. For 11 patients, a catheter was placed for intermittent drainage over an average of 3 days. Seven patients ultimately required a more definitive surgical procedure. CONCLUSION: Echocardiographically guided pericardiocentesis is a safe and effective treatment for this uncommon but serious complication of RA.

Comparison of usefulness of echocardiographic Doppler variables to left ventricular end-diastolic pressure in predicting future heart failure events.

We sought to determine whether the echocardiographic Doppler parameters of left ventricular diastolic dysfunction predict future heart failure (HF) events and, if so, which parameters best predict HF. We also examined whether the predictive ability of echocardiographic Doppler parameters was related to their prediction of left ventricular end-diastolic pressure (LVEDP). We studied patients who underwent cardiac catheterization and echocardiography performed within a 30-day period. The end point was HF, defined as new-onset or recurrent HF diagnosed by a physician and requiring the initiation or modification of treatment of HF. We identified 289 patients (mean age 63.5 +/- 12.6 years) with a mean follow-up of 10.9 +/- 10.2 months. A total of 24 HF events occurred. LVEDP was a significant predictor of HF univariately and independently in multiple regression models after adjustment for ejection fraction. In Cox models adjusted for age, gender, LVEDP, and ejection fraction, only the left atrial volume index and early mitral inflow to early diastolic tissue velocity (E/e') ratio remained predictive of HF. A multiple regression model, including all echocardiographic variables, showed a persistent, although attenuated, relation of early to late mitral inflow velocity (E/A) ratio and E/e' with LVEDP (p = 0.06 and p = 0.002, respectively). The addition of E/e' or the left atrial volume indexed to body surface area, but not E/A, to the clinical history and left ventricular ejection fraction provided incremental prognostic information. A LVEDP of > or =20 mm Hg, E/e' ratio of > or =15, and left atrial volume index of > or =23 ml/m(2) identified those with a higher risk of HF. In conclusion, invasively determined LVEDP is an independent predictor of future HF events. E/e' and the left atrial volume indexed to body surface area are the best independent predictors of future HF and provide prognostic information incremental to the clinical history and left ventricular ejection fraction.

Left atrial myopathy in cardiac amyloidosis: implications of novel echocardiographic techniques.

AIMS: To assess left atrial (LA) function and determine the prevalence of LA dysfunction in AL amyloidosis (AL) using conventional and strain echocardiography. METHODS AND RESULTS: LA ejection fraction, LA filling fraction, LA ejection force, peak LA systolic strain rate (LAsSR), and LA systolic strain (LA epsilon) were determined in 95 AL patients (70 with and 25 without echocardiographic evidence of cardiac involvement, abbreviated CAL and NCAL, respectively), 30 age-matched controls (CON), and 20 patients with diastolic dysfunction and LA dilatation (DD). Peak LAsSR >2 standard deviations below mean CON value was used as the cut-off for normal LA function. LA ejection fraction was lower in CAL when compared with CON (40.4+/-13.6 vs. 67.0+/-6%, P=0.01). Left atrial septal strain rate and strain were lower in CAL (0.8+/-0.5 s(-1) and 5.5+/-4%, respectively) compared with CON (1.8+/-0.8 s(-1) and 14+/-4%, respectively, P=<0.0001), NCAL (1.6+/-0.8 s(-1) and 13+/-7%, respectively, P<0.0001) and DD (1.3+/-0.4 s(-1) and 10+/-2%, respectively, P<0.0001). Based on peak LA systolic strain rate criteria, the cut-off values for normal LA function were -1.1 s(-1) and -1.05 s(-1) for lateral and septal walls. Using these criteria, LA dysfunction was identified in 32% (lateral LA criteria) and 60% (septal LA criteria) of CAL patients. Lateral and septal LAsSR were lower in CAL patients with vs. those without symptoms of heart failure. Inter- and intra-observer agreement was high for LA strain echocardiography. CONCLUSION: LA function assessment using strain echocardiography is feasible with low intra- and inter-observer variability. LA dysfunction is observed in AL patients without other echocardiographic features of cardiac involvement and may contribute to cardiac symptoms in CAL.

Clinical and echocardiographic characteristics of hemodynamically significant pericardial effusions in patients with systemic lupus erythematosus.

Echocardiographic-guided pericardiocentesis was found to be safe and efficacious in treating 11 patients with systemic lupus erythematosus who had hemodynamically significant pericardial effusions. These patients tended to present early in their disease course, and men were more often affected.