Amyloidosis comprises a group of diseases that occurs in five to nine cases per million patients per year worldwide irrespective of its classification. Although the hepatic involvement in primary amyloidosis is frequent, the clinical manifestations of liver amyloidosis are mild or even absent. The authors report the case of an aged man who complained of diffuse abdominal pain and marked weight loss and presented clinical signs of hepatopathy. Clinical workup revealed portal hypertension with ascites, hemorrhoids, and esophageal varices. The laboratory tests showed the cholestatic pattern of liver enzymes, hyperbilirubinemia, renal insufficiency and massive proteinuria accompanied by the presence of serum pike of monoclonal lambda light chain protein. The outcome was unfavorable, and the patient died. The autopsy findings revealed the diagnosis of amyloidosis predominantly involving the liver and kidneys. The bone marrow examination demonstrated the deposition of amyloid material associated with clonal plasma cells infiltration. The authors call attention to portal hypertension as a rare manifestation of primary amyloidosis. Meanwhile, this diagnosis should be taken into account whenever the hepatopathy is accompanied by laboratory abnormalities consistent with hepatic space-occupying lesions concomitantly with other organs involvement. In the case reported herein, kidney involvement was also present with renal failure, massive proteinuria with monoclonal serum gammopathy, what reinforced the diagnostic possibility of primary amyloidosis.
Introduction:
Coronary-subclavian steal syndrome (CSSS) is a rare cause of myocardial ischemia after coronary artery bypass grafting (CABG), due to significant stenosis of the proximal portion of the left subclavian artery. It is characterized by blood flow inversion in the left internal thoracic artery, diverting the blood flow from the coronary artery to the left medio-distal subclavian artery. We report a case of a patient who underwent complex surgery to correct the CSSS.
Case report:
A 70-year-old male patient, with history of both percutaneous coronary intervention (PCI) of the right coronary artery and CABG, was admitted to the emergency room with STEMI and complete atrioventricular block. He was initially managed to the cath lab where a transvenous pacemaker was implanted and coronary angiography showed occlusion of the left main, proximal right coronary and left subclavian arteries. Only saphenous vein - second marginal graft was patent and, despite severe stenosis in the proximal portion of the circumflex artery, this artery was responsible for irrigation of the entire left coronary system, as well as retrograde filling of the left internal thoracic artery - anterior descending artery graft. A conservative approach was proposed. For a better anatomically interpretation, coronary angiotomography was indicated and showed obstruction of the left subclavian artery after its origin, with 2.2cm length and refilling before the origin of the ipsilateral vertebral and internal mammary arteries. In addition, most visceral branches demonstrated severe atherosclerosis, and the right common iliac artery and left external iliac artery were occluded. After discussion with the Heart Team, it was decided to perform a left carotid-subclavian graft with a segment of the saphenous vein. The procedure was successful, with no complications and lasted 5 hours. The patient was discharged from hospital one week later.
Discussion:
CSSS is rare and difficult to treat. The most common treatment is angioplasty of the subclavian artery, which was not possible in this case due to the lack of femoral access and the extension of the occlusion. In this patient, with a high risk of death, the bypass surgical revascularization with saphenous graft became the only feasible treatment.
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