P178 TYPE–A AORTIC DISSECTION IN MARFANOID HABITUS

Abstract

Abstract Aortic dissection (AD) is the tearing of the tunica intima of the aortic wall with creation of a false lumen. Type–A AD, in which the intimal flaps originates in the aortic tract proximal to the left subclavian artery, is a surgical emergency because – if left untreated – it has mortality rates as high as 1–2% every hour, from the onset of symptoms. Certain collagenopathies, such as Marfan Syndrome, are a predisposing condition for AD. We describe the case of a 25–year–old man with Marfanoid habitus who came to the Emergency Room of our facility, with acute pulmonary oedema. He had been complaining about thoraco–abdominal pain, bloody mucus and worsening dyspnoea since the week before. Upon cardiology consultation, a transthoracic echocardiogram showed an aneurysm of the aortic root (70 mm) and the ascending aorta (90 mm), with dissection flap extended beneath the aortic valvular plane, with torrential aortic valve insufficiency and eccentric jet, a left ventricle severely dilated and a severe systolic dysfunction (EF 25%). On urgent CT angiography the dissection flap was observed to extend cranially to the right common carotid artery and caudally to the renal arteries. The patient then underwent cardiac surgery to replace the aortic valve with a mechanical prosthesis and the ascending aorta as per the Bentall procedure and to replace the aortic arch with reimplantation and debranching of the supra–aortic trunks. Four months after discharge, the patient presented again due to sustained ventricular tachycardia (SVT) which was interrupted by electric cardioversion. On this occasion, an ICD implant was indicated as a secondary prevention measure. After one month, recurrence of SVT was observed, which was successfully treated by the implanted ICD, and therefore indication was given for an electrophysiologic study and subsequent transcatheter radiofrequency ablation of the ventricular arrhythmia. The case presented is of particular interest as it highlights the crucial role of a rapid echocardiographic assessment focussed on making a diagnosis and directing treatment, despite a misleading clinical picture due to duration and atypicality of symptoms. It should also be stressed that an overall assessment of the patient, based on habitus and age, plays a major role in establishing a suspected diagnosis and guiding the patient towards the most appropriate treatment.