The role of standard echocardiographic parameters in endomyocardial biopsy proven cardiac amyloidosis [Uloga standardne ehokardiografije u amiloidozi srca dokazanoj biopsijom miokarda]

Biočić, Stanko and Jakšić Jurinjak, Sandra and Vincelj, Josip and Barić, Davor and Manojlović, Spomenka and Delić Brkljačić, Diana (2015) The role of standard echocardiographic parameters in endomyocardial biopsy proven cardiac amyloidosis [Uloga standardne ehokardiografije u amiloidozi srca dokazanoj biopsijom miokarda]. Collegium Antropologicum, 39 (2). pp. 441-445. ISSN 0350-6134

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Abstract

Primary light-chain (AL) amyloidosis is a plasma cell dyscrasia associated with the deposition of immunoglobulin-derived amyloid in multiple organs. In the heart, this results in an infiltrative cardiomyopathy, with increased left ventricular wall thickness, normal or decreased left ventricular (LV) cavity size and congestive heart failure. Cardiac involvement is a major determinant of prognosis of AL amyloidosis. We report a patient with cardiac amyloidosis proven by cardiac biopsy, and aim to point out at transthoracic echocardiography as the hallmark of diagnostics. Echocardiography revealed increased LV thickness at 20mm, impaired LV ejection fraction (EF) at 35%, enlarged atria, transmitral deceleration time at 156 ms and increased E/A ratio at 4.25. Early diagnosis and intervention can have a significant impact on the patient's response to treatment, especially when the underlying condition involves a malignancy or infiltrative disorder. Standard transthoracic echocardiography as a noninvasive diagnostic tool is valuable and has a significant role in diagnosis and prognosis.

Abstract in Croatian

Primarna amiloidoza je diskrazija plazma stanica sa depozitima amiloida u multiplim organima. U srcu, to rezultira infiltrativnom kardiomiopatijom, zadebljalih stijenki ventrikla, normalnim ili smanjenim šupljinama srca te zatajenjem srca. Zahvaćenost srca je najvažniji prognostički faktor amiloidoze. Prikazujemo pacijenticu u dobi od 52 godine sa biopsijom miokarda dokazanom amiloidozom srca, u cilju prepoznavanja važnosti i naglašavanja ehokardiografskih karakteristika amiloidoze srca te ehokardiografije kao temeljnog dijagnostičkog postupka. Amiloidozu srca karakterizira dijastolička disfunkcija zbog infiltracije miokarda amiloidom. Rano prepoznavanje može poboljšati prognozu bolesnika. Na amiloidozu srca treba posumnjati kod bolesnika sa progresivnom dispnejom, uz nedilatativnu kardiomiopatiju sa zadebljalim stijenkama ventrikla u ehokardiogramu te zabilježenom mikrovoltažom u EKG-u. Pravovremena dijagnoza ima odraza na bolesnikov odgovor na liječenje, osobito ako osnovno stanje uključuju malignitet ili infiltrativni poremećaj, stoga standardna transtorakalna ehokardiografija kao neinvanzivna dijagnostička metoda ima značajnu ulogu u dijagnostičkom postupku i prognozi bolest.

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