Isolated Left Atrial Amyloidosis: Acute Premitral Stenosis Secondary to Spontaneous Intramural Left Atrial Hemorrhagic Dissection

Abstract

This case presentation describes the very rare condition of isolated atrial amyloidosis and it's associated complications. A 53-year-old male presented to hospital in severe pulmonary edema secondary to spontaneous intramural left atrial hemorrhage and subsequent wall dissection causing acute "pre-mitral valve" obstruction. The patient was subsequently found to have AL subtype amyloid, which has never before been reported in isolated atrial amyloidosis. The prognosis is poor in AL type amyloidosis with cardiac involvement. (J Am Soc Echocardiogr 2006; 19:938.e1-e4.) CASE REPORTA 53-year-old man was transferred to a metropolitan teaching hospital from a small rural hospital with a presumed diagnosis of bilateral pneumonia. He had originally presented with a 2-day history of fever, cough, dyspnea, and pleuritic chest pain. Initial chest radiograph showed bilateral infiltrates thought to be bronchopneumonia; ciprofloxacin and azithromycin were started.On arrival to the teaching hospital emergency department, he was peripherally shut down, tachypneic (45/ min), tachycardic (120/min), and hypotensive (blood pressure 85/50 mm Hg). He was intubated and ventilated after transfer to the intensive care department because of agitation and hypoxia unresponsive to noninvasive ventilation. His electrocardiogram on admission to the intensive care department was normal and his troponin I level was 1.10 g/L, white cell count was 28.9 ϫ 10 9 /L, and serum lactate was 3.6 mmol/L. A repeated chest radiograph showed the classic bat-wing appearance of acute pulmonary edema. A pulmonary artery catheter was inserted and his cardiac index was 1.6 L/min/m 2 with a pulmonary artery occlusion pressure of 30 mm Hg. An

Spontaneous dissecting aneurysm of the left atrium complicated by cerebral embolism: A report of two cases with review of literature

“…It can be caused by any cardiac interventions, including both surgical and catheter-based that involve manipulation of the LA. LA dissection is related to mitral valve surgery as well as coronary artery disease, arrhythmia, trauma, tumor, and spontaneous occurrence 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20. Reported incidence rate following mitral valve surgery is 0.16% 1 .…”

“…In review of literature, 9 cases of spontaneous LA dissection without any intra-cardiac manipulations have been reported 4, 13, 14, 15, 16, 17, 18, 19, 20. Some of these spontaneous cases were attributed to underlying pathology including 3 cases of amyloid light-chain amyloidosis,15, 16, 19 2 cases of severe mitral annular calcification,4, 6 one case of infective endocarditis 20 and three were truly spontaneous without any known etiology. None of our cases had mitral annular calcification or endocarditis.…”

“…Presentation may be early or delayed. Spontaneous dissection of the LA has been rarely reported in the literature 13, 14, 15, 16, 17, 18, 19, 20. We herein describe two patients, who presented with stroke and were found to have LA dissection with pseudo-aneurysm formation.…”

Left atrial dissection

“…The causes of LA dissection were thought to be postoperative shear forces against the tissue between the mitral and aortic prostheses and marked tissue fragility due to systemic sclerosis and steroid treatment [14]. Spontaneous occurrence was seen in 11 patients [60,[62][63][64][66][67][68][69], including 2 patients primarily diagnosed with amyloidosis [61,65] and 2 patients with mitral annular calcification, which could have been the origin of the dissection in the LA wall [59,68]. Detailed information is summarized in Table 1.…”

Case of Left Atrium Intramural Hematoma After Percutaneous Coronary Intervention

“…К факторам риска развития ИГЛП относят коллагеновые сосудистые заболевания, использование стероидов [16], прием антикоагулянтов, многокомпонентной антитромботической терапии [1]. Известны случаи образования спонтанной диссекции ЛП и ИГЛП [1] на фоне амилоидоза сердца, терапии варфарином, когда был превышен уровень МНО выше референсных значений [8,17,18], острого инфаркта миокарда без проведения ЧКВ [2,19], массивного кальциноза кольца МК [20,21], инфекционного эндокардита [1]. При массивном кальцинозе кольца МК к формированию диссекции ЛП ведет инфекция и некроз заднего отдела кольца МК [1].…”