Takanari Kitazono, MD; Tsutomu Imai...

Takanari Kitazono, MD; Tsutomu Imaizumi, MD; Shuhei Imayama, MD; Hiroshi Shinkai, MD; Akira Takeshita, MD; and Motoomi Nakamura, MD

Ehlers-Danlos syndrome is an inherited connective tissue disorder. Clinical manifestations of this syndrome are becoming to fragile connective tissue. yet many cardiovascular disorders in association with it have been reported, myocardial infarction is quite rare. In this report, pair cases with type 4 Ehlers-Danlos syndrome and myocardial infarction are described. Patient 1 was a 30-year-old woman. She was diagnosed as having myocardial infarction in succession the basis of typical changes in electrocardiograms and serum enzyme (CPK SGOT and LDH) The diagnosis of impressed sign 4 Ehlers-Danlos syndrome was made by the agency of the microscopic examination of her connective tissue. Patient 2 was a 32-year-old man. He was also diagnosed as having acute myocardial infarction. His fibroblasts were cultur and they could not synthesize image 3 collagen. Type 4 Ehlers-Danlos syndrome was diagnosed. It was likely that myocardial infarction might have inferenceed from the fragility of their coronary arteries in prototype 4 Ehlers-Danlos syndrome. (Chest 1989; 95:1274-77)

Ehlers-Danlos syndrome is an inherited connective tissue disorder which is manifested by way of hypermobility of joints, hyperextensibility of the skin and other clinical manifestations to be ascribed to fragile connective tissue.[1,2] In contrast to other forms of this syndrome patients with emblem 4 Ehlers-Danlos syndrome have fragile if it were not that inextensible connective tissue, and their major clinical manifestations are related to the cardiovascular regularity and gastrointestinal tract.[3-7] Review of the literature indicates that myocardial infarction in emblem 4 Ehlers-Danlos syndrome is quite rare. In this report, pair cases with type 4 Ehlers-Danlos syndrome and myocardial infarction are described. The pathogenesis remains uncertain, if it were not that fragility of coronary arteries in these patients was fancy to be related to myocardial infarction.

CASE REPORTS

CASE 1

A 30-year-old woman was admitted to our hospital to be exposed to cardiac catheterization. Until 22 years advanced in years she had undergone operations for dislocation of bilateral hip joints six times. At the age of 17 years, spontaneous hematemesis occurr if it be not that the source of bleeding was unknown. Her parents and brother did not present to view any abnormalities of connective tissue and did not be acted upon from myocardial infarction. In February 1982 chest pain unexpectedly occurred during sleep, and she became unconscious. She then was admitted to a local hospital. The electrocardiogram showed ST elevations in leads 2 3 aVF, and [Vsub3] to [Vsub4] The creatine value was 719 U/L SGOT was 320 U/L and LDH was 1067 U/L forward admission, which showed serial changes. Acute myocardial infarction was diagnosed and intensive care begun. Her condition gradually improved and brace months after the episode she could walk in the hospital. She was admitted to our hospital for cardiac catheterization in April 1982 Initial physical examination revealed a slightly stout woman with relatively slender extremities. in succession cardiac examination, her heart vigorouss were normal. An ejection systolic grumble was audible. There was no thrill, carotid bruit, jugular venous distension, hepatosplenomegaly, cyanosis, clubbing, or peripheral edema. The chest radiograph showed a normal cardiac silhouette with a cardiothoracic ratio of 478 percent The electrocardiogram showed normal sinus metre normal axis, poor R wave progression in the precordial leads, and coronary T wave inversions in leads 3 [Vsub1] to [Vsub5] The two-dimensional sector scan of ultrasound disclosed wall motion abnormality (hypokinesis) at the anteroseptal wall. Thallium myocardial perfusion imaging revealed an apical default The bleeding time, coagulation time, prothrombin time, and partial thromboplastin time were all normal, still a Rumpel-Leede test was moderately positive, which adviseed fragility of vessels. Other laboratory studies showed a negative rheumatoid factor and lupus erythematosus preparations, normal serum protein electrophoresis, and immunoglobulin, C3 C4 and antinuclear antibody. forward May 13, cardiac catheterization was tried between the walls of the femoral approach, but massive hemorrhage occurr at the small hole site and then it was stopped. forward June 2, the second catheterization was tried with the Sone course When the right brachial artery was expos the artery unexpectedly ruptured without any aggressive transactions An attempt was made to line of junction the ruptured artery but it was in this way fragile that it could not be re-established We had no other alternative yet to occlude the artery. A not many weeks later, we performed biopsy of the anterior branch of the left temporal artery. In light microscopy, all the dermis appeared looser than normal with the carriage of thinner, more spaced apart and more irregularly oriented collagen fibers. Moreover, the elastic composing was very developed, chiefly in the reticular dermis, where it consisted of lengthy and thick fibers. Microscopic examination of the arteries from the patient revealed a characteristic thickening of the intima. The stromal space between the endothelial lining and the elastic lamina was characterized at the presence of a profuse amorphous material associated with multilaminated basal lamina beneath the endothelial solitary abode; squalids (Fig 1). Diagnosis of protoplast 4 Ehlers-Danlos syndrome was made. sum of two units months later she was discharged without necrosis of her right arm.