Hereditary amyloidosis represents 10% of all amyloid deposit diseases. Cardiac involvement clinically presents as heart failure. As advanced imaging methods have become available, subclinical detection of cardiac involvement has become a must in those patients since it changes the prognosis of the disease. The objective of the present paper was to determine if echocardiographic parameters of auricular and ventricular mechanics better predict cardiac involvement compared to the classic echocardiographic parameters suggestive of amyloid deposit in the heart. This was a prospective, observational and descriptive study of a cohort of 30 patients with diagnosis of hereditary TTR+ amyloidosis without known cardiac involvement. We performed 2 and 3D echocardiography with left auricular and ventricular mechanics by strain method. Each patient was evaluated at the beginning of the study and at a 1-year follow-up visit. The grade of diastolic disfunction increased from a normal diastole in 67% of patients to only 53% at 1-year follow-up. The antero-posterior diameter of the left atrium increased (p = 0.04), the E/e’ Index also increased (p = 0.01), both suggestive of increased left ventricular filling pressures. The LVEF, as a classic parameter of left ventricular function remained normal in 97% of patients during follow-up. No differences were found in terms of systolic ventricular mechanics; however, diastolic mechanics were altered. Untwist was increased (p = 0.02) and the reservoir auricular function was decreased from 34 to 30% (p = 0.03). Table. Echocardiographic strain and 3D echocardiography can show changes early in patients with cardiac involvement in patients with hereditary TTR+ amyloidosis. Statistically significant results Variable Basal 1 year follow-up P Left atrial anteroposterior diameter (mm) 32.8 ± 5 35.1 ± 5.6 0.004 Ventricular mass (gr/m2SC) 70.7 (61-90.8) 82.6 (69.2-113.7) <0.0001 Left ventricular telediastolic volume (ml) 37.2 ± 11 41.6 ± 11.6 0.03 Mitral A velocity (ms) 113.7 ± 28.6 130.5 ± 19.5 0.03 Lateral e mitral velocity (cm/s) 13.8 ± 5.5 12.4 ± 4.8 0.04 Mitral E/e’ index 7.9 ± 5.2 9.5 ± 5.5 0.01 Untwist 110.8 ± 49.9 127.5 ± 62.6 0.02 Reservoir auricular strain (%) 34.8 ± 8.6 30.8 ± 9.4 0.03 Telesystolic left atrial volume (ml) 25.9 ± 8.8 27.4 ± 11 0.01 Telediastolic left atrial volume (ml) 42.5 ± 12.9 40.7 ± 13.7 0.03 Left atrial ejection fraccion (%) 0.42 ± 0.20 0.34 ± 0.18 0.03 Table 1. Statistically significant results.
Introduction Intravascular leiomyomatosis (IL) is a rare type of tumor common and was first described by Birch-Hirschfeld in 1896. There are currently few cases reported in the literature involving this type of vena cava tumor and the right atrium. This tumors are characterized by nodular, intravascular, histologically benign masses of smooth muscle with variable extensión. The most frequently affected women are pre-menopausal and multiparous. There are no specific clinical manifestations of vascular leiomyomas. Clinical case This is 51-year-old woman with a history of morbid obesity (body mass index of 47kg / m2SC) in bariatric surgery protocol, mixed dyslipidemia, is presented to the emergency service of our Institute, with uterine myomatosis diagnosed 6 months prior to admission. She was admitted for a 2-week evolution characterized by dyspnea of medium efforts that progressed at rest on the day of his admission with acute heart failure. A transthoracic and transesophageal echocardiograhy three-dimensional (TTE, TEE 3D) was made in which a solid-appearing, regular-border, heterogeneous tumor was reported from the inferior vena cava to the right atrium without being attached to the interatrial septum or other structures, with severe protrusion to the right ventricle, highly mobile, heterogeneous, without vascularity with color doppler, causing intermittent obstruction and tricuspid stenosis with a mean gradient of 8mmHg, pulmonary artery systolic pressure calculated at 46 mmHg. TAC showed invasion from the uterine leiomyoma to the inferior vena cava and right atrium. The patient was taken to emergency surgery, the vascular surgery and cardiothoracic surgery performed a long intervention. Macroscopic description reported uterine tumor of 25x19x18cm. Weight of 3.90Kg, with multiple nodular lesions, the largest being 18x15cm. Intracavitary tumor with fragment of tissue. The pathological diagnosis was uterine leiomyomatosis, intravascular leiomyomatosis in right periadnexal vessels. The patient was taken to intensive therapy, although the postoperative period was prolonged, she was discharged in functional class II NYHA. Discussion This case demonstrates the importance of recognizing this rare disease and suspecting it in patients who present with the a symptoms and the role of the echocardiography is fundamental for the characterization of the tumor and the invasion of neighboring structures. The reported cases of intravascular leiomyomas with intracardiac extension are approximately 10% and to date, less than 300 cases have been reported in the literatura. Vascular leiomyomas arise from uterine leiomyomas with vascular invasión, the etiology still remains unknown, it has been associated with high levels of estrogen. The key in its treatment is the complete resection. The prognosis is good after its resection. The recurrence rate is up to 30%. Anti-estrogenic therapy has been considered but its efficacy is controversial. Abstract P1696 Figure. Intravascular leiomyomatosis
A cohort of patients with breast cancer HER2 positive are exposed to chemotherapy with cardiotoxic drugs like anthracyclines (A) and trastuzumab (T). The decrease in longitudinal strain is a helpful tool to predict clinical cardiotoxicity. The purpose of this paper is to determine if echocardiographic parameters like longitudinal strain with speckle tracking predict subclinical cardiotoxicity compared to conventional echocardiographic parameters. This was a retrospective, observational cohort study that involved 20 patients with breast cancer HER2+ who were treated with a chemotherapy reverse sequence (TH-A). We made a basal echocardiogram, 3, 6, 9 and 12 months after chemotherapy with a cumulative dose of 240mg/m2 of doxorrubicin. Cardiotoxicity was defined as a decrease in left ventricular ejection fraction of 10% below 53%, and subclinical cardiotoxicity as a decline of >15% of longitudinal strain. 5 patients (25%) developed decline in longitudinal strain (subclinical cardiotoxicity) 3 months after initiation of chemotherapy, despite 2 of them had normal LVEF (p = 0.01), with the use of heart failure medication they showed partial reversibility. Lineal regression analysis showed that patients with GLS >-19% at baseline did not present cardiotoxicity during the follow up (p = 0.03). Changes in GLS were independent of left ventricular mass, E/A and peak s’ wave velocity (p = 0.01). The diagnosis of subclinical cardiotoxicity by the decrease of GLS is a useful tool with possible therapeutic implications and possible reversibility of cardiotoxicity in patients with reverse chemotherapy sequence. Significant results Binary analysis of echocardiographic variables Value LVEF p GLS p Basal 62.4 ± 6.7 -19.8 ± 1.8 3 months 59.4 ± 11.1 0.615 -18.1 ± 3.9 0.01 6 months 60.2 ± 8.7 0.354 -18.9 ± 3.3 0.185 9 months 61.6 ± 6.0 0.862 -19.65± 1.9 0.408 12 months 61.1 ± 5.7 0.379 -19.8 ± 1.9 0.859 Binary analysis of echocardiographic variables Abstract P1773 Figure. Sequence of chemotherapy
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