European heart journal. Case reports · 2021
Role of imaging for diagnosis and management of aortic valve papillary fibroelastoma and cardiac amyloid light chain amyloidosis: a case report.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Dimov Ivan, Meuleman Nathalie, de Cannière Didier, Unger Philippe
Аннотация
BACKGROUND: We report the case of a patient who presented with concomitant aortic valve papillary fibroelastoma (PFE) and cardiac amyloidosis. Although histologically benign, PFE confers an increased thromboembolic risk, and surgical excision is often indicated. However, outcomes of cardiac surgery are poor in patients with cardiac amyloidosis.
CASE SUMMARY: A 61-year-old man with complaints of dyspnoea and weight loss of 10 kg developing over the past 5 months was evaluated in the cardiology clinic. Echocardiography revealed sessile aortic valve PFE and was also highly suggestive of cardiac amyloidosis. The diagnosis of amyloid light chain amyloidosis secondary to indolent multiple myeloma was eventually confirmed. Therapy with daratumumab, bortezomib, cyclophosphamide, and dexamethasone allowed full remission over a 6-month period and resulted in marked improvement in symptoms and cardiac function as evaluated by global longitudinal strain. Further workup with cerebral magnetic resonance revealed multiple vascular sequelae. Surgical removal of the aortic fibroelastoma with bioprosthetic aortic valve replacement was performed successfully and the patient had an uneventful recovery.
DISCUSSION: Papillary fibroelastoma and cardiac amyloidosis are rare and most likely unrelated entities. Concomitant presentation of both conditions in the same patient presents a unique therapeutic challenge. By allowing cardiac function to be monitored during chemotherapy, speckle-tracking echocardiography can prove instrumental in determining the optimal timing of surgical intervention.
CASE SUMMARY: A 61-year-old man with complaints of dyspnoea and weight loss of 10 kg developing over the past 5 months was evaluated in the cardiology clinic. Echocardiography revealed sessile aortic valve PFE and was also highly suggestive of cardiac amyloidosis. The diagnosis of amyloid light chain amyloidosis secondary to indolent multiple myeloma was eventually confirmed. Therapy with daratumumab, bortezomib, cyclophosphamide, and dexamethasone allowed full remission over a 6-month period and resulted in marked improvement in symptoms and cardiac function as evaluated by global longitudinal strain. Further workup with cerebral magnetic resonance revealed multiple vascular sequelae. Surgical removal of the aortic fibroelastoma with bioprosthetic aortic valve replacement was performed successfully and the patient had an uneventful recovery.
DISCUSSION: Papillary fibroelastoma and cardiac amyloidosis are rare and most likely unrelated entities. Concomitant presentation of both conditions in the same patient presents a unique therapeutic challenge. By allowing cardiac function to be monitored during chemotherapy, speckle-tracking echocardiography can prove instrumental in determining the optimal timing of surgical intervention.