Severe heart failure of amylootic etiology

Abstract

Amyloidosis is a systemic disease caused by the extracellular deposition of insoluble fibrils of low molecular weight proteins in various tissues. It can be classified in the forms: primary, secondary, hereditary and senile systemic. We report the case of a 75-year-old male patient who was admitted to a cardiologic emergency with progressive edema in the lower limbs started four months ago. Thirty days before admission he presented dyspnea from medium to small effort, evolving to restlessness and in decubitus (cardiac failure - functional class IV). On physical examination he presented a regular, conscious, tachidispneic, no fever, hydrated, hypochromic (1 +/ 4 +), icteric (2 +/ 4 +), acyanotic and jugular stasis at 45°. The cardiac rhythm was regular in two times and normal heart sounds, with systolic murmur in the mitral focus (2 +/ 6 +) fixed unfolding of the second sound; blood pressure of 80 x 40 mmHg and heart rate of 84 bpm. The admission electrocardiogram showed regular sinus rhythm, 1st degree atrioventricular block, low voltage in frontal plane leads, electrically inactive zone in anteroseptal region and left bundle branch conduction disorder. Chest X-ray with increased cardiac area, pattern of pulmonary congestion and bilateral pleural effusion, more pronounced on the left. A transthoracic echocardiogram was performed too, showing right enlarged cardiac chambers, mildly depressed right ventricular systolic function, moderate to severe tricuspid insufficiency, moderate pulmonary hypertension, left ventricular hypertrophy, left ventricular dysfunction and bright granular echogenicity finding. Ultrasonography of abdomen showed liver and spleen with heterogeneous texture, suggestive of granulomatous disease. Subsequently, hepatic biopsy was performed, confirming the hypothesis of infiltrative disease determined by cardiovascular amyloidosis. Patient evolved with lowered level of consciousness, and acute renal failure, undergoing hemodialysis and transfer to Coronary Unit, where he presented cardiorespiratory arrest and evolution to death.