Intravenous leiomyomatosis: a rare cause of intracardiac mass.

Intravenous leiomyomatosis is an unusual clinical condition characterized by histologically benign smooth muscle lesions extending from the uterus into pelvic and systemic veins and, more rarely, into the right cardiac chambers. We report the case of a 45-year-old woman who presented with a three-week history of dyspnea on exertion, shortness of breath and fatigue. Echocardiography showed a large mobile mass in the right atrium prolapsing into the right ventricle and extending to the inferior vena cava. A computed tomography scan revealed a large mass extending from the right atrium to the inferior vena cava and through the systemic veins as far as the popliteal veins. A presumptive diagnosis of large thrombus was made; the correct diagnosis of intravenous leiomyomatosis with intracardiac involvement was obtained only after surgical resection and histologic examination.

Left pulmonary artery evaluation through transesophageal echocardiography.

INTRODUCTION: Transesophageal echocardiography (TEE) has become increasingly useful in the study of patients with suspected pulmonary thromboembolism. OBJECTIVE: The aim of this study was to prospectively evaluate the usefulness of TEE in the study of the distal part of the left pulmonary artery (LPA) as well as the influence of this procedure on total echocardiographic exam duration. METHODOLOGY: A prospective study in two groups of consecutive patients referred for TEE with a one- year interval between evaluation of Group A: 33 patients, 17 male, mean age 54 +/- 24 years, and Group B: 42 patients, 20 male, mean age 48 +/- 27 years (p = NS). The procedure was considered long when it took more than 3 min to evaluate the distal part of the LPA. RESULTS: In group A we were able to visualize the distal part of the LPA in 24 patients (73%) without significant prolongation of total exam duration in 16 patients (48% of group A). In one of the patients with suspected pulmonary thromboembolism thrombi were only observed in the distal part of the LPA. In group B we were able to visualize the distal part of the LPA in 36 patients (86%) without significant prolongation of total exam duration in 26 patients (61% of group B). CONCLUSIONS: 1. Visualization of the distal part of the LPA was possible in more patients, and with TEE time prolongation in less patients, in group B. These differences can be accounted for by the training of the operator in this technique. 2. The importance of visualization of this part of the LPA in guiding treatment in the subset of patients with pulmonary thromboembolism confirms the usefulness of this specific procedure.

Evaluation of hypertrophic obstructive cardiomyopathy by exercise stress echocardiography. New methodology.

Exercise echocardiography with image acquisition during treadmill exercise is the most common form of stress testing used in our department. The usual evaluation of patients with hypertrophic obstructive cardiomyopathy consists of serial echocardiographic studies at rest. The purpose of this study was to evaluate intraventricular gradients during exercise echocardiography in patients with a diagnosis of hypertrophic obstructive cardiomyopathy. We studied 13 patients, 8 male, mean age 56 +/- 9 years Intraventricular gradients were measured using continuous wave Doppler; two evaluations were performed at rest: one in left lateral decubitus and the other in orthostatic position after one minute in this position. The patients then underwent a treadmill exercise test using the modified Bruce protocol, during which intraventricular gradients were measured at peak exercise. Finally, a further measurement was taken in the first 90 seconds of the recovery period in left lateral decubitus. The intraventricular gradient in left lateral decubitus was 54 +/- 29 mmHg; in orthostatic position 69 +/- 30 mmHg (p < 0.001 versus gradient in left lateral decubitus); at peak exercise 109 +/- 47 mmHg (p < 0.001 versus gradient in orthostatic position); and during recovery in left lateral decubitus 78 +/- 33 mmHg (p < 0.001 versus gradient at peak exercise in orthostatic position). We conclude that intraventricular gradients increase significantly in orthostatic position and increase considerably during treadmill exercise testing. The gradients measured in the recovery period do not reflect what happens during effort or therefore during these patients' daily activities. This type of evaluation can help us to better understand the pathophysiology of patients with a diagnosis of hypertrophic obstructive cardiomyopathy and to optimize treatment.

Stress echocardiography in the evaluation of exercise physiology in patients with severe arterial pulmonary hypertension. New methodology.

INTRODUCTION: Doppler echocardiography is usually performed when assessing a patient with severe pulmonary hypertension (PHT), since it enables accurate determination of the severity of the hypertension through evaluation of several morphologic and hemodynamic variables. Echocardiograms are usually performed in left lateral decubitus (LLD). However, symptoms often arise only in a standing position and particularly during exercise. OBJECTIVE: To evaluate a group of patients with severe PHT using Doppler echocardiography during treadmill exercise testing. METHODS: We studied 8 patients (group A), mean age 43.88 +/- 14 years, 7 women; three had idiopathic pulmonary hypertension, 2 pulmonary thromboembolic disease, 2 Eisenmenger syndrome, and one pulmonary hypertension associated with celiac disease. We also studied an 8-patient control group (group B) with similar demographic characteristics, who had tricuspid regurgitation but no known cardiac disease, including pulmonary hypertension (excluded by echocardiogram). In addition to the ergometric variables of stress test duration using the modified Bruce protocol, resting heart rate, peak heart rate (PHR), resting systolic blood pressure (RSBP) and peak systolic blood pressure (PSBP), we evaluated the following echocardiographic variables: pressure gradient between right ventricle and right atrium (RV/RAg) and systolic volume (SV) in left lateral decubitus, in a standing position (SP) and at peak workload (PW). Stress testing was stopped in cases of fatigue and/or dyspnea. RESULTS: In group A, the RV/RAg in LLD was 100 +/- 20 mmHg, 98 +/- 20 mmHg in SP (p = NS) and 129 +/- 27 mmHg at PW (p = 0.003 vs. SP). In group B, the RV/RAg in LLD was 19.8 +/- 3.5 mmHg, 14.6 +/- 2.1 mmHg in SP (p = 0.0005) and 29.5 +/- 3.3 mmHg at PW (p < 0.0001 vs. SP). In group A, SV was 38 +/- 11 ml in LLD, 35 +/- 10 ml in SP and 32 +/- 9 ml at PW (p = NS); in group B, it was 63 +/- 5 ml in LLD, 55 +/- 5 ml in SP and 64 +/- 7 ml at PW (p < 0.0001). PHR was 114 +/- 10 bpm in group A and 145 +/- 8 (p < 0.0001) in group B. RSBP was 113 +/- 13 mmHg and PSBP 112 +/- 21 mmHg (p = NS) in group A, and 116 +/- 18 mmHg and 161 +/- 25 mmHg respectively (p < 0.0001) in group B. In four patients from group A, symptomatic falls in systolic blood pressure and SV occurred at PW. During a mean follow-up of 27 months (between 6 and 44 months), two of these four patients died and one is awaiting lung transplantation. CONCLUSIONS: 1. RV/RAg did not diminish in the standing position and rose significantly with orthostatic isotonic exercise during exercise testing in patients with severe PHT, with pulmonary artery systolic pressure reaching suprasystemic values. 2. Systolic volume and systolic blood pressure did not rise during exercise in patients with severe PHT, and patients with a decrease in systolic volume had worse clinical evolution. 3. Patients with severe PHT appeared to have chronotropic incompetence during exercise compared to the control group.