Is LVH a must in an amyloid heart?

Abstract

Abstract Background Transthyretin amyloidosis (ATTR), an infiltrative cardiomyopathy, is characterized by the infiltration of transthyretin-derived amyloid fibrils into the cardiac extracellular space, which causes morphological and functional heart damage. Because of the vast range of symptoms that can accompany ATTR cardiac amyloidosis, the illness remains unknown. However, misdiagnosis or delayed diagnosis due to inconsistencies between clinical findings and the severity of the underlying disease hampers treatment methods. Aim and objectives This article investigates the intricate relationship between ATTR amyloidosis pathogenesis and clinical signs to understand better the challenges and advancements in early discovery, treatment, and prognosis of this concealed sickness. Methods & Results Between November 2022 and April 2024, we conducted a prospective analysis on a cohort of 71 patients diagnosed with ATTR cardiac amyloidosis utilizing PYP imaging at our institution (Al Hada Armed Force Hospital, Taif, Saudi Arabia). The Medical Ethics Review Committee of the hospital accepted this project (2023-720). During our study, we faced two cases without LVH and both cases have grade II-III cardiac uptake. 1st case A 66-year-old male patient who is a known case of ischemic cardiomyopathy with EF 35% since 2018, he underwent PCI in 2018. The patient is following the cardio clinic since 2018 and on every visit he was asymptomatic, The Patient in 2021 experienced new shortness of breath within six minutes’ walk distance less than 400 meters. The patient experienced erectile dysfunction and underwent intervention for his carpal tunnel syndrome. ECG showed old Q in anterior leads. Echo showed the same EF 35% and old wall motion abnormalities. Diagnostic coronary angiography showed patent stents and no residual ischemia. When we revised his old echo study that was done in 2017, we found EF 55%, concentric LVH. PYP bone scintigraphy was decided and showed grade II-III cardiac uptake. 2nd case A 53-year-old hypothyroid female patient and she has metastatic left breast cancer. During her workup bone scan, grade III cardiac uptake was accidentally discovered using an HMDP isotope. The patient was referred to the cardio clinic where the patient denied any cardiac or non-cardiac symptoms. All her labs, ECG and Echo were within normal range. CMR showed diffuse subendocardial LGE suggestive of infiltrative disease. Conclusion Based on unique pathological features of CA that would present as morphological LVH with /without ECG evidence of LVH. We hypothesized that LVH would occur in the later stages of the disease or may be decreased due to other pathology as coronary artery disease. Clinical suspicion remains the cornerstone for early diagnosis and workup.