Abstract

Clinical data: In the course of life, the patient developed three types of clinical manifestations. The first one was characterized by dyspnea and tachypnea, from birth to five months of age, increased pulmonary flow and heart failure, with cardiomegaly and hepatomegaly. The second phase was characterized by stability without significant symptoms, which lasted until the age of three, presumptive of balanced aortopulmonary flow. The third phase, with decreased pulmonary flow, had mild cyanosis that worsened two years ago. Exertion fatigue was noted for four years ago. The patient maintained saturation above 85% and up to 75% two years ago, hemoglobin = 17 g/dL and hematocrit = 62%. Physical examination: Eupneic, cyanotic +++, normal pulses, no jugular venous distention. Weight: 54 kg; height: 160 cm; BP: 115/60 mmHg; HR: 78 bpm; oxygen saturation = 78%. Aorta was palpated ++ in the suprasternal notch. In precordium, apical impulse in the 4th right intercostal space and discrete systolic impulses at the right sternal edge. Second hyperphonetic heart sound, +++, at pulmonary area, increases toward the lower left sternal edge. Discrete and mild continuous murmur, +/++, at pulmonary area, irradiating to the back. Liver was not palpated.

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