Associazione Nazionale Medici Cardiologi Ospedalieri




Starnazzi Francesco L’Aquila(L’Aquila) – Universit√† degli studi de L’Aquila | Dei Lorenzo-Lupo L’Aquila(L’Aquila) – Universit√† degli studi de L’Aquila | Trambaiolo Paolo Roma(Roma) – Ospedale Sandro Pertini

Background: Light-chain amyloidosis (AL) is caused by misfolded light chains produced by a small B-cell clone in bone marrow, that aggregate in plasma and deposit in various tissue, included the heart. The progressive nature of the disease and the high mortality rate within 6 months of diagnosis makes early diagnosis very important.

Clinical Case: A 64-year-old patient presented to the Emergency Room (ER) with shortness of breath for a month and externally positive exercise test for symptoms without ECG alterations. The ECG performed in the ER diffuse low voltages with no repolarization abnormalities. Laboratory tests showed an increase in Troponin I| from 0.658 to 0.669 ng/ml (normal range <0.012) and elevated NTproBNP at 7080 pg/ml. The electrophoresis profile indicated MGUS, known since 2015.The patient was admitted to the Intensive Cardiac Unit (ICU) with a diagnosis of NSTEMI. The medical history revealed a family history of Chronic Coronary Syndrome (CCS) and hypertension. Home therapy included beta-blockers and Angiotensin Receptor Blockers (ARBs). Upon admission to ICU, the patient was asymptomatic. Coronary angiography revealed dual stenosis of the anterior descending coronary artery and was treated by dual drug eluting stent. The patient 's symptoms remained unchanged. A single subsequent episode of paroxysmal dyspnea occurred. An echocardiogram conducted in the EchoLab raised suspicion of cardiac amyloidosis. Hematological exams to assess a possible AL type showed positive results. The patient underwent a bone marrow aspirate because the hematologists were unable to perform a biopsy due to the concomitant dual antiplatelet therapy. We are currently waiting for the histological results to start chemotherapy. Conclusions: An early suspicion of amyloidosis could have aided in established the correct timing for angioplasty and double anti-aggregation. This clinical case requires a multidisciplinary approach between cardiologists and hematologists, especially given the very high mortality within a few months of diagnosis.