Syncope from dynamic left ventricular outflow tract obstruction simulating hypertrophic cardiomyopathy in a patient with primary AL-type amyloid heart disease.

Dynamic left ventricular outflow tract (LVOT) obstruction is seen classically in hypertrophic cardiomyopathy. Cardiac amyloidosis can present with asymmetric hypertrophy that resembles hypertrophic cardiomyopathy, and, in some cases, with dynamic LVOT obstruction. The occurrence of syncope in such patients is not uncommon. The syncope is usually thought to be related to mechanisms other than LVOT obstruction, such as arrhythmias, conduction disturbances, orthostatic hypotension, or vasovagal effects associated with neuropathy.Herein, we report the case of a patient who had immunocyte-derived (primary AL-type) cardiac amyloidosis with the echocardiographic appearance of hypertrophic cardiomyopathy and evidence of LVOT obstruction that caused syncope. We were able to provoke and identify dynamic LVOT obstruction that produced presyncopal symptoms similar to those that typically occur in such patients spontaneously. Dynamic LVOT obstruction as a cause of syncope should be considered in patients who have cardiac amyloidosis and echocardiographic evidence of hypertrophic cardiomyopathy.

Stem cells improve left ventricular function in acute myocardial infarction.

BACKGROUND: Animal studies have suggested dramatic improvement in cardiac function after acute myocardial infarction (AMI) through regeneration of the myocardium or neovascularization by transfer of cells derived from bone marrow (BMC) generated clinical studies. Recently published small sized studies have yielded mixed results, leaving the question unanswered. HYPOTHESIS: We analyzed data from these studies in a meta-analysis to investigate if intracoronary stem cell therapy was effective in improving cardiac function. METHODS: A total of 7 randomized controlled trials meeting the inclusion criteria were identified by a systematic literature search. Primary endpoint was change in global left ventricular ejection fraction (LVEF) baseline to follow-up (ranging between 3 to 6 months). The meta-analysis consisted of 516 patients (BMC group, 256; control group, 260). A 2-sided alpha error of less than .05 was considered to be statistically significant (P<.05). RESULTS: There were no significant differences in patient characteristics between the BMC treatment and control groups at baseline. Compared to the control group, patients in the BMC treatment group had significantly greater increase in LVEF from baseline to follow-up (mean difference: 6.108%; SE: 1.753%; 95% confidence interval [CI]: 2.672%- 9.543%; P<.001). CONCLUSIONS: The present meta-analysis suggests that intracoronary bone marrow stem cell infusion may be effective in improving left ventricular systolic function in patients after acute myocardial infarction.

Antiretroviral therapy in the treatment of HIV-associated nephropathy.

BACKGROUND: The effect of antiretroviral therapy (ART) on the clinical course of patients with human immunodeficiency virus (HIV)-associated nephropathy (HIVAN) is not well-established. This study was undertaken to further elucidate the potential benefit of ART in HIV-infected patients with documented HIVAN. METHODS: A cohort of 263 consecutive HIV-infected patients referred to the Johns Hopkins renal clinic from 1995 to 2004 was examined. Patients were included if they had biopsy-proven HIVAN and did not require dialysis within 1 month of their kidney biopsy. The cumulative probability of renal survival was calculated using the Kaplan-Meier method. Multivariate analysis was performed using the Cox regression method. RESULTS: Fifty-three patients among 152 biopsied patients had HIVAN. Among 36 patients who met the inclusion criteria, 26 were treated with ART (group I) and 10 patients were not (group II). Except for age, baseline demographics and clinical characteristics were similar in the two groups. Renal survival was significantly better in the group receiving ART by both univariate (P = 0.025) and multivariate analysis (overall adjusted hazard ratio = 0.30; 95% confidence interval 0.09-0.98; P < 0.05) for ART compared with no treatment. CONCLUSIONS: Patients with biopsy-proven HIVAN treated with ART had better renal survival compared with patients who did not receive ART. HIVAN should be considered as an indication to initiate ART.

Nephrotic range proteinuria and CD4 count as noninvasive indicators of HIV-associated nephropathy.

PURPOSE: Human immunodeficiency virus (HIV)-associated nephropathy is a common and serious cause of progressive renal insufficiency in patients with HIV, frequently presenting with nephrotic range proteinuria. The purpose of this study is to document the histopathologic diagnoses seen in HIV-positive patients with and without nephrotic range proteinuria and to evaluate the predictive value of both nephrotic range proteinuria and CD4 count in diagnosing HIV-associated nephropathy. METHODS: We performed a cross-sectional, single-center study of all 107 HIV-positive patients who had both a renal biopsy and urine protein measurement between 1995 and 2002. Nephrotic range proteinuria was defined as a urine protein-to-creatinine ratio > 3 or a 24-hour urine protein > 3 g. Clinical and laboratory characteristics of those patients with and without HIV-associated nephropathy were compared. Sensitivity, specificity, and positive and negative predictive values of nephrotic range proteinuria in the diagnosis of HIV-associated nephropathy were determined. RESULTS: Fifty-five biopsied patients had nephrotic range proteinuria, among whom 29 (53%) were diagnosed with HIV-associated nephropathy. Among the remaining patients, 12 had non-HIV-associated nephropathy focal segmental glomeruloscerlosis, 3 had membranoproliferative glomerulonephritis, 2 had AA Amyloid, 2 had diabetic nephropathy, and 7 had other diagnoses. Sensitivity, specificity, and positive and negative predictive values of nephrotic proteinuria in the diagnosis of HIV-associated nephropathy were 73%, 61%, 53%, and 79%, respectively. The patients with HIV-associated nephropathy had a significantly higher creatinine (8.2 mg/dL vs 2.5 mg/dL, P < .001) and a lower CD4 count (158 count/mm3 vs 349 count/mm3, P < .01) at the time of biopsy. Although significantly more patients with HIV-associated nephropathy had a CD4 count below 200 (P = .03), among those with a CD4 count below 200, 10 of 30 patients (33%) had diagnoses other than HIV-associated nephropathy. Injection drug use, presence of hepatitis C, and hypertension were not associated with HIV-associated nephropathy. CONCLUSION: Our results suggest that HIV patients with nephrotic range proteinuria warrant a kidney biopsy because the presence of nephrotic range proteinuria, even in the presence a low CD4 count, does not establish the diagnosis of HIV-associated nephropathy.

Sonography as a predictor of human immunodeficiency virus-associated nephropathy.

OBJECTIVE: To determine whether renal sonography can be used to predict the pathologic diagnosis of human immunodeficiency virus-associated nephropathy. METHODS: This cross-sectional study evaluated 87 human immunodeficiency virus-positive patients who underwent both kidney biopsy and renal sonography after referral to the Johns Hopkins Renal Clinic from January 1995 to July 2002. Using a standardized measure of echogenicity, an independent blinded radiologist reviewed the original sonographic images. Sensitivity, specificity, positive and negative predictive values, receiver operating characteristic curves, and likelihood ratios were determined with the use of the biopsy pathologic report as the criterion standard. RESULTS: Thirty-four patients (39%) had biopsy-proved human immunodeficiency virus-associated nephropathy. A higher serum creatinine level, greater proteinuria, and black race were associated with human immunodeficiency virus-associated nephropathy, whereas age, sex, hypertension, and diabetes were not. Sensitivity and specificity for the highest 2 levels of echogenicity were 96% and 51%, respectively Sensitivity and specificity for the highest level of echogenicity were 40% and 95%. The likelihood ratio for the diagnosis of human immunodeficiency virus-associated nephropathy on the basis of the highest echogenicity score was 7.4 (95% confidence interval, 1.3-73.0; P = .006). The likelihood ratio for the lowest 2 echogenicity scores was 0.08 (95% confidence interval, 0.002-0.57; P = 0.003). Kidney size was not associated with human immunodeficiency virus-associated nephropathy status. CONCLUSIONS: This study provides evidence that, among patients with human immunodeficiency virus and kidney disease, the highest and lowest levels of sonographic echogenicity have diagnostic value in respectively establishing or excluding human immunodeficiency virus-associated nephropathy.