Marfan cardiovascular disease without the Marfan syndrome. Fusiform ascending aortic aneurysm with aortic and mitral valve regurgitation.
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Biomedical subjects
Publications and source records attributed to R L Reis.
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The number of children in this report treated with either TBW or exchange transfusions is small. Case mortality rates among children with Reye syndrome in Stage IV coma tends to be exceedingly high, varying from 50 to 100%. Intracranial pressure monitoring with the subarachnoid screw may have been an additional factor in increasing our survival data in three patients in the TBW group, since it provided continuous monitoring of ICP and allowed judicious administration of mannitol intravenously. Survival of five of six patients without neurologic sequelae in the present series has encouraged us to coninue utilization of TBW in children with Stage IV Reye syndrome.
Since 1971 we have employed the Morrow procedure in 30 patients with idiopathic hypertrophic subaortic stenosis (IHSS). All manifested obstruction to left ventricular outflow either at rest or with provocation (Valsalva) and 17 had moderate or severe mitral regurgitation. There were no operative deaths. Obstruction was abolished in all patients except one in whom a trivial 15-mm pressure gradient persisted, and all patients with moderate or severe mitral regurgitation evidenced marked amelioration of the mitral regurgitation. Distinct symptomatic improvement has been experienced by all patients, and 20 are entirely asymptomatic postoperatively. No patient has experienced syncope postoperatively although 15 experienced syncope before operation. In 12 patients the electrocardiogram recorded postoperatively was essentially unchanged from the preoperative record. In 16 patients a left anterior hemiblock was apparent postoperatively. In one patient a complete left bundle branch block appeared postoperatively and one patient demonstrated Wolff-Parkinson-White syndrome. One patient died suddenly and unexpectedly 2 years following operation. This patient continued to have palpitations after operation although all other symptoms as well as the left ventricular outflow obstruction were abolished by operation. It is suggested that propranalol administration be continued postoperatively in those patients experiencing palpitations or manifesting arrhythmias. Asymmetrical septal hypertrophy dislocates the cardiac apex and papillary muscles anteriosuperiorly producing abnormal systolic anterior mitral leaflet mition. The Morrow procedure restores more normal ventricular geometry and thereby eliminates the pathophysiological mechanism of obstruction and mitral regurgitation. The myotomies and myectomy should be extended far inferior through the entire width of the septum onto the free lateral ventricular wall. The procedure can be accomplished easily and safely through a transaortic approach. Since the operative risk at present appears to be negligible, prosthetic material is not required, and the risk of significant injury to the conduction tissue is small, we feel the current status of surgery for IHSS is such that the Morrow procedure should be performed earlier in the course of patients with IHSS manifesting obstruction or mitral regurgitation.
The coexistence of severe coronary artery and peripheral vascular lesions is not uncommon. Although diagnostic and sequential therapeutic techniques are standardized, the priority of surgical treatment is unresolved. Eight cases are reported in which simultaneous surgical correction of coronary atherosclerosis and carotid atherosclerosis, abdominal aortic aneurysm, or aortoiliac atherosclerosis was accomplished with success. Two additional cases demonstrate the complications which can occur when coexistent lesions are not corrected simultaneously. The surgical techniques employed are discussed. Because of these results, a further clinical trial seems warranted.
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Between 1970 and 1975, 234 porcine heterograft valve prostheses were implanted in 193 patients with 12 operative (6%) and 21 late deaths (11%). Detailed hemodynamic studies were made 6 months to 1 year following operation in 72 patients. Mean atrioventricular (A-V) diastolic pressure gradients averaged 5.2 mm Hg for 29 mm heterografts and 2.3, 2.5, and 2.7 mm Hg for 31 mm, 33 mm, and 35 mm heterografts, respectively. In 10 patients with aortic prostheses peak systolic gradients averaged 16 mm Hg at rest (range 0-26) but increased to 70 mm Hg during exercise (range 20-125). Anticoagulants were not used except when intracardiac clot was present at operation. There were 12 systemic arterial emboli, all with mitral prostheses and low cardiac output; nine patients were in atrial fibrillation and three were in sinus rhythm. Three patients evidenced late tissue failure. Hydraulic function in the A-V position is excellent: prosthetic valve size is not limited by tertiary orifice and A-V anulus accepts large prosthesis. The patient's anulus is the determinant of aortic prosthesis size; heterograft internal diameter to outside diameter ratio is suboptimal and hydraulic function of the aortic prosthesis is marginal. In our clinical practice we consider the performance characteristics of currently available valves in relation to the individual patient's priorities to select an appropriate prosthesis.
A retrospective analysis was undertaken of clinical data and catheterization studies of 151 consecutive unselected patients who underwent aorta-coronary bypass at the University of Kansas Medical Center between 1971 and 1973. The purpose was to determine the effect of preoperative left ventricular function and extent and severity of coronary artery obstruction on operative mortality rate and long-term survival. The postoperative follow-up period ranged from 10 to 49 months and averaged 26 months. Left ventricular function was assessed by qualitative analysis of left ventricular angiograms. Severity of coronary obstruction was quantified by scoring coronary arteriograms according to the system of Friesinger and associates. Patients with normal or near normal ventriculograms were considered to have good left ventricular function. Patients showing moderate or severe impairment of contraction were considered to have poor left ventricular function. Obstruction scores ranging from 2 to 7 points were classified as low scores, and scores from 8 to 15 points were classified as high scores. Four groups of patients were identified based upon preoperative left ventricular function and obstruction severity: Group I, 29 patients with good left ventricular function and low scores; Group II, 22 patients with poor left ventricular function and low scores. Group III, 28 patients with good left ventricular function and high scores. Elective aorta-coronary bypass in these three groups was accompanied by no operative or late deaths. Group IV comprised 72 patients with poor left ventricular function and high scores. In this group there was a 10 per cent operative mortality rate (7 of 72 patients) and a 5 per cent year late mortality rate. Relief of angina occurred equally in all groups. Thus operative risk can be prospectively determined by analysis of left ventricular function and severity of coronary obstruction. Surgical treatment resulted in negligible operative and late mortality rates (0 per cent) in all patients except those in whom poor ventricular function was accompanied by severe and diffuse coronary artery obstruction. Operation should be offered to this latter group (Group IV) despite the higher operative and postoperative risk because of salutary postoperative results.
The long-term durability of saphenous vein bypass grafts and their effect on existing intrinsic coronary artery disease remain ill defined. Therefore, sequential catheterization studies were performed in patients selected for study solely on the basis of documentation of a patent graft at an earlier study performed three to nine months postoperatively; at that time 29 patent grafts were demonstrated in 20 patients. Fifteen to 36 months postoperatively (average 22 months), 27 grafts were unchanged, 1 manifested minimal luminal irregularities and 1 was occluded. In one additional patient, studied 4 months and 4 1/2 years postoperatively, the graft was widely patent and had good distal runoff at the second study. Sequential coronary arteriograms revealed that progression of disease to complete occlusion occurred in 24 percent of vessels with severe lesions proximal to a patent graft, whereas progression of disease distal to a graft anastomosis was uncommon. Of 25 vessels not receiving grafts, disease progressed in 5 (20 percent). Grafts that are patent 3 months after operation appear to remain patent for at least 2 to 3 years, and their presence does not unduly accelerate the disease process involving the native coronary arteries.
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The effects of hydrocortisone on left ventricular force-velocity relations, systolic length-tension curves, and diastolic pressure-volume curves (compliance) were determined before and 30 and 60 minutes after a 1 hour period of normothermic ischemic cardioplegia. Five dogs were given no hydrocortisone, 5 dogs 150 mg. per kilogram of hydrocortisone immediately after aortic unclamping, and 5 dogs 150 mg. per kilogram of hydrocortisone 20 minutes before clamping. Thirty minutes after unclamping, there was a 45 per cent decrease in contractility (p less than 0.005). At 60 minutes, there was a significant improvement (p less than 0.025), although a 32 per cent depression persisted. There were no differences among the three groups (p less than 0.05). Dogs not given hydrocortisone before clamping, compliance decreased 18 per cent (p less than 0.05), and in those given hydrocortisone immediately after unclamping, compliance decreased 28 per cent (p less than 0.025). The decreases in compliance in the hydrocortisone-treated dogs were significantly less (p less than 0.05) than the decreases observed in the dogs not given hydrocortisone.
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