Mitral valve operation in patients with the Marfan syndrome.

Optimal surgical treatment of mitral regurgitation in the Marfan syndrome (valve repair versus replacement) is controversial because the underlying connective tissue defect theoretically might compromise repair durability. To examine the results of mitral valve repair in these patients, we did a retrospective review of 160 patients with the Marfan syndrome who had cardiac surgical procedures between January 1983 and January 1993. Thirty-six patients had mitral procedures, 29 of which were repairs. Mitral valve replacement was necessary in seven patients because of extensive annular calcification and/or severe anterior leaflet abnormalities. The 18 men and 11 women undergoing mitral valve repair had a mean age of 26.5 +/- 2.6 years (range 9 months to 54 years); seven patients were less than 18 years of age. Twenty-four of the 29 patients had concomitant aortic root replacement because of aortic dilation or valvular insufficiency. All 29 repairs included annuloplasty, and 11 patients also required leaflet resection. There were no operative deaths. At mean follow-up of 26.6 +/- 4.8 months, there have been three late deaths, two caused by arrhythmia and one by complications of type III aortic dissection. All survivors are in New York Heart Association class I or II. In three patients recurrent mitral regurgitation developed (grade III or IV); 5-year actuarial freedom from significant mitral regurgitation was 88.3%. One patient required repeat mitral annuloplasty after endocarditis of the composite aortic graft spread to the mitral valve. No patient required late mitral valve replacement. These results demonstrate that (1) 22% of patients with the Marfan syndrome who undergo cardiac operation require a mitral valve procedure, (2) most can be treated by mitral repair rather than replacement, and (3) at early follow-up, results of mitral repair in this population are satisfactory.

Low risk of gastroesophageal injury associated with transesophageal echocardiography during cardiac surgery.

Transesophageal echocardiography (TEE) has increasingly been used in cardiology and cardiac surgery with few reported complications. This study was undertaken to determine whether TEE is associated with an increased incidence of gastroesophageal (GE) bleeding or postoperative GE symptoms of anorexia, dysphagia, or sore throat. Forty-one patients who underwent TEE during cardiac surgery and 40 control patients who underwent cardiac surgery without TEE were prospectively followed. In addition, a retrospective chart review of 200 patients who underwent TEE during cardiac surgery was also performed. The following information was derived from the patient interviews and chart reviews: (1) The development of both frank and occult upper gastrointestinal tract (UGI) bleeding; (2) the patient's preoperative anticoagulation status; and (3) the patient's subjective complaints of anorexia, dysphagia, or sore throat. The incidence of postoperative occult or frank UGI bleeding was not increased in the groups who underwent TEE. Additionally, the incidence of postoperative GE symptoms was comparable in the three groups. These findings are discussed in the context of reported complications associated with UGI endoscopy. Based on this analysis, recommendations for the safe performance of TEE have been provided.

Cutaneous pseudo abscesses: an unusual presentation of severe pancreatitis.

In this report, we present a 53-yr-old man with extensive subcutaneous fat necrosis due to acute pancreatitis presenting as fluctuant collections resembling large multiple abscesses. The diagnosis was suggested by examination of the wound aspirate. Findings included absence of organisms on the gram stain, presence of fat globules on wet mount, and an elevated amylase in the wound aspirate. This dramatic presentation preceded any symptoms or signs of overt pancreatitis.

Urinary and gait disturbances as markers for early multi-infarct dementia.

In a retrospective study of 84 outpatients with multiinfarct dementia, urinary and gait disturbances were found in 50% and 27%, respectively, and often preceded dementia and discrete stroke-like episodes by more than 5 years. Compared to patients without urinary disturbance, those with urinary dysfunction were predominantly male and more behaviorally impaired, but were similar in age, cognitive score, depression score, computerized tomography findings, and relative survival. Compared to patients without gait disturbance, those with gait abnormality had a higher Hachinski ischemic score and depression score and were more behaviorally impaired. Urinary and gait abnormalities may be markers for cerebrovascular disease and vascular dementia even in the absence of frank stroke. Damage to bifrontal outflow tracts may be the common pathophysiological mechanism underlying the behavioral and motor symptoms characteristic of vascular dementias.

Arylsulfatase A variants in patients with alcoholism.

Leukocytes from 200 mentally ill patients and 100 normal controls were analyzed for electrophoretic variants of arylsulfatase A. Four different variant forms were found in 15 subjects. There is a relatively high occurrence of the arylsulfatase A variants in patients with alcoholism. Twenty-one per cent (12/56) of patients with alcoholism have a variant enzyme. Only one of the 100 normal controls has a variant enzyme. (This single subject was considered normal by the criteria of the study, namely, a self-report of no current medical problem or psychiatric history. However, upon further testing, it was found that this subject has neurological and neuropsychological deficits). The hypothesis is presented that chronic alcohol intake and abnormal arylsulfatase A act in concert to elevate sulfatide levels which results in abnormalities of brain function. If this hypothesis is correct, persons in whom abnormal arylsulfatase A is expressed may be at risk to the neuropathological effects of alcohol.