A variety of cardiovascular lesions has been described in association with Marfan's syndrome, and these account for the sudden and premature death that occurs not infrequently in patients suffering from this disease. The following case is recorded as an example of arachnodactyly complicated by medial necrosis of the coronary vessels, leading to a dissecting aneurysm, with death from acute myocardial infarction.
Case ReportThe patient was a young man, aged 21. Before his present illness his health had been good, except for a minor abdominal injury sustained 9 years previously. Three days before admission to hospital, he developed a pain in the left side of his chest which radiated down his left arm. The pain was sharp and stabbing and persisted for two days, after which it eased off for some hours. He then became more acutely ill, and when admitted complained of severe abdominal pain that recufred intermittently 'and was accompanied by vomiting.On examination he was very pale and shocked, and lips, hands, and feet were cyanosed. Arachnodactyly was noted, but there were no other stigmata of Marfan's syndrome. The radial pulse was not palpable and the blood pressure was unrecordable. The venous pressure was raised and the cervical veins pulsating. The apex beat was visible in the fifth left interspace three inches from the sternum, and the heart rate was 120 a minute. A harsh systolic murmur was heard over the prncordium. An electrocardiogram showed the pattern of a large posterior myocardial infarct. The patient's condition deteriorated rapidly and he died four hours after admission.
NecropsyThe main pathological findings were confined to the heart, which weighed 300 g. Externally there was subepicardial hlmorrhage along the posterior atrioventricular groove. The right atrium was moderately dilated and showed the presence of a transverse tear in the endocardium, 2 cm. in length, situated just above the medial cusp of the tricuspid valve and below the opening of the coronary sinus. Necrotic muscle and blood clot extruded from the tear (Fig. 1). The right ventricle, pulmonary arteries, left atrium, and mitral valve appeared normal. The left ventricle was dilated and the wall was rather atrophic. A ragged tear was found in the posterior wall of the left ventricle close to the mitral ring. The surrounding muscle was soft and friable, and though little altered in colour appeared to be the site of a recent infarct (Fig. 2). The rupture did not extend through the wall of the ventricle to the external surface but communicated with a track that traversed the upper part of the interventricular septum posteriorly, to the wall of the right atrium and terminated in the endocardial tear already noted in this cavity. Macroscopic examination of the coronary arteries revealed no atheroma. The right vessel was somewhat dilated 1 cm. from its origin, and appeared to be occluded by a thrombus at this level and for 2 cm. along its course.Microscopic Examination. A seption of the right coronary artery at the level of occlusion revealed...
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