Multi-imaging assessment of successful surgical treatment of pulmonary artery dilatation and dissection in Marfan syndrome

<span class="paragraphSection">A 42-year-old male patient with a 1-month history of dyspnoea of great effort, daily oppressive chest pain with intensity 5/10 irradiated to the shoulder and left scapular region, palpitations and haemoptysis,. He was diagnosed according the new Ghent criteria of Marfan syndrome. He had aortic dilatation and systemic score 13/20. Transthoracic echocardiography showed dilatation of the trunk of the pulmonary artery (70 mm) and right and left branches and dissection of the pulmonary trunk, above the valvular plane with a wide communication site, extending to the left pulmonary brunch (<span style="font-style:italic;">A</span> and <span style="font-style:italic;">B</span>; see Supplementary dataSupplementary data online, <span style="font-style:italic;">Movie S1</span>), and dilatation of the ascending aorta (43 mm). Computed tomography corroborated these findings. (<span style="font-style:italic;">C</span>, <span style="font-style:italic;">D</span> and <span style="font-style:italic;">E</span>). The histopathological study of the pulmonary artery demonstrated extensive replacement of the elastic mid layer by repairing connective tissue fibroblasts, mild cystic necrosis in the middle elastic layer and extensive recent haemorrhage in the adventitia suggesting acute dissection (<span style="font-style:italic;">F</span>). The patient went for surgical treatment with a surgical time of 5 h and 32 min.</span>

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