Dissecting Aneurysm of the Coronary Artery in Arachnodactyly

Abstract

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...

May emergency hypertension be reason of spontaneous coronary artery dissection?

“…However, three types of clinical presentation have been described: those with no discernible systemic cause, those with known arterial vasculopathy such as atherosclerosis, and those associated with the peripartum period [3,4]. Some possible causes of coronary artery dissection have included, surgical complication, blunt chest trauma [5], Marfan's syndrome, arachnodactyly [6], cystic medial necrosis, sarcoidosis, polyarteritis nodosa, Kawasaki disease, fibromuscular dysplasia, hyperplasia, angiomatosis, cocaine use [7], intensive physical exercise [8], previous renal transplant, peripartum hormone influence [4], complications related to catheter procedures and aortic dissection. Unlike aortic dissection, SCAD has not definitely been associated with hypertension.…”

Polyarteritis nodosa presenting as acute myocardial infarction with coronary dissection

“…Dissection of the right or left circumflex coronary arteries is less common. Most SCAD cases are idiopathic, but it has been associated with Marfan's syndrome, angiitis, trauma, sarcoidosis, and cystic medial necrosis [21][22][23][24]. Autopsy series frequently describe an eosinophilic periadventitial infiltrate [25,26].…”

Kardiovaskuläre Probleme bei Kindern und Jugendlichen mit Marfan-Syndrom

“…Methods: Echocardiographic and colour doppler flow studies were performed in 44 patients with a mean age of 9,2 years (0, [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19]2).In addition to the standard Mmode measurements, the aortic diameter was determined at four discrete points (aortic valve anulus, aortic sinus, aorta ascendens, aortic arch) by two-dimensional echocardiography.The data were compared with the literature, and a control group of 80 healthy children with a mean age of 9,4 years (0,1-19,2). Results: 86% of the patients showed at least one pathologic echocardiographic abnormality: Aortic root ectasia was found in 84%, and mitral valve prolaps in 59%.Colour flow doppler detected mitral regurgitation in 25%.7% of the patients showed aortic insufficiency, and 7% had tricuspid valve involvement.In patients with the Marfan syndrome left atrial diameter corrected for body surface area was significantly greater with 2,22±0,72 cm/m 2 than in the control group with 1,15±0,38 cm/m 2 .Left ventricular enddiastolic diameter and fractional shortening showed no difference between the two groups.…”

“…Seltenere kardiale Befunde sind der Trikuspidalklappenprolaps sowie eine Ektasie des Pulmonalarterienhauptstamms [3,38]. In Einzelfällen wurden auch Aneurysmen der Koronararterien beobachtet [16]. Schwere und Progredienz der kardiovaskulären Befunde sind entscheidend für Das Marfan-Syndrom ist eine autosomal-dominant vererbte Bindegewebserkrankung, die mit einer Häufigkeit von ca.…”