Surgical treatment for Scheie's syndrome (mucopolysaccharidosis type I-S): report of two cases

Scheie's syndrome (mucopolysaccharidosis type I-S) is a rare genetic lysosomal storage disease affecting mucopolysaccharide metabolism, and is known to include cardiovascular disease. Surgical treatment was carried out in 2 patients with Scheie's syndrome. Patient 1 was a 56-year-old man with triple-vessel coronary artery disease, who successfully underwent coronary artery bypass grafting. Patient 2 was a 52-year-old man with aortic and mitral valve stenosis, who successfully underwent combined aortic and mitral valve replacement. The literature on Scheie's syndrome associated with valvular and coronary artery disease is also reviewed.     

Combined surgery for coronary artery disease and thoracic aortic disease

Combined surgery in 6 cases who had coronary artery disease and thoracic aortic disease simultaneously was analyzed. Case # 1 had ascending aortic replacement under deep hypothermic circulatory arrest because of iatrogenic aortic dissection caused by aortic clamp during the routine coronary artery bypass grafting (CABG). Case # 2 had DeBakey type II chronic dissection. Case # 3 had type I aortic dissection 4 years after the initial CABG. Both case # 2 and # 3 had ascending aortic replacement under retrograde cerebral perfusion along with CABG. Transverse aortic replacement was performed in case # 4, # 5 and # 6 under selective cerebral perfusion along with CABG. Case # 4 was associated with ascending-transverse aortic aneurysm. Case # 5 had aortitis syndrome complicated with severe coronary ostial stenosis and cervical branch stenosis. Case # 6 also had aortitis syndrome, severe coronary ostial stenosis, heavily calcified ascending-transverse aorta, and mitral and aortic regurgitation. This case had mitral and aortic valve replacement additionally. Case # 2 died of low cardiac output syndrome and multi-organ failure postoperatively. Case # 4 did not recover from profound shock that followed the preoperative acute myocardial infarction. The problems of low cardiac output syndrome caused by long interval of ischemic cardiac arrest, and also the problems of proximal anastomotic site of saphenous vein grafts were discussed.     

Complex cases in heart surgery

Between January 1968 and December 1993, 837 patients underwent cardiac operations with were either complex or performed in the presence of a life threatening disease of other vital organs. Of them 74 (8.8%) patients have died within 30 days after the operation. A substantial number of the operations and associated operative death included left ventricular (LV) aneurysmectomy or plication or LV endoaneurysmectomy with coronary artery bypass (CAB) grafts with or without other cardiac procedures, cardiac reoperations, CAB grafts and mitral or aortic valve replacement, combined mitral and aortic valve replacement (MAVR) with or without tricuspid valve replacement and CAB grafts, CAB grafting for an end-stage coronary artery disease (CAD), emergency CAB grafts for an acute myocardial infarction with cardiogenic shock, complex internal thoracic artery (ITA) grafting, and miscellaneous. The best results were achieved in CAB grafts for an end-stage CAD, complex ITA grafting, CAB grafts with mitral or aortic valve replacement, cardiac reoperations. MAVR and miscellaneous. This is probably, related to an intensive treatment of congestive heart failure (CHF) before the operation, pretreatment with the oxygen free radical inhibitor (allopurinol), selective use of an intraaortic balloon assist device and LV venting, routine use of hemoconcentrator (ultrafiltration) during cardiopulmonary bypass in those with CHF, thorough myocardial protection and a complete coronary revascularization.     

Mortality and morbidity among patients who undergo combined valve and coronary artery bypass surgery: early and late results

AIM: To describe mortality and morbidity early and late after combined valve surgery and coronary artery bypass grafting (CABG) as compared with CABG alone. PATIENTS and METHODS: All patients from western Sweden in whom CABG in combination with valve surgery or CABG alone was carried out in 1988-1991. RESULTS: Among 2116 patients who underwent CABG, 35 (2%) had this combined with mitral valve surgery and 134 (6%) had this combined with aortic valve surgery, whereas the remaining 92% underwent CABG alone. Patients who underwent combined valve surgery and CABG were older, included more women and had a higher prevalence of previous congestive heart failure and renal dysfunction but on the other hand a less severe coronary artery disease. Among patients who underwent mitral valve surgery in combination with CABG the mortality over the subsequent 5 years was 45%). The corresponding figure for patients who underwent aortic valve surgery in combination with CABG was 24%. Both were higher than for CABG alone (14%; P < 0.0001 and P = 0.003, respectively). In a stepwise multiple regression model mitral valve surgery in combination with CABG was found to be an independent significant predictor for death but aortic valve surgery in combination with CABG was not. Among patients who underwent mitral valve surgery in combination with CABG and were discharged alive from hospital 77% were rehospitalized during the 2 years following the operation as compared with 48% among patients who underwent aortic valve surgery in combination with CABG and 43% among patients with CABG alone. Multiple regression identified mitral valve surgery in combination with CABG as a significant independent predictor for rehospitalization but not aortic valve plus CABG. CONCLUSION: Among patients who either underwent CABG in combination with mitral valve surgery or aortic valve surgery or CABG alone, mitral valve surgery in combination with CABG was independently associated with death and rehospitalization, but the combination of aortic valve surgery and CABG was not.     

Regulation of antigen receptor signal transduction by protein tyrosine kinases

The purpose of this study was to assess the value of electron beam computed tomography in the detection of cardiac calcifications in coronaries and valves of dialysis patients and to determine the rate at which calcification progresses. Forty-nine chronic hemodialysis patients aged 28 to 74 years were compared with 102 non-dialysis patients aged 32 to 73 years with documented or suspected coronary artery disease, all of whom underwent coronary angiography. We used high-resolution electron beam computed tomography scanning to make 30 axial slices with a distance of 3 mm between each slice. The number of calcifications, the surface area, and the average and highest density values were measured. We calculated a quantitative coronary artery calcium score and assessed calcification of mitral and aortic valves. In dialysis patients, the measurements were repeated after 12 months. The coronary artery calcium score was from 2.5-fold to fivefold higher in the dialysis patients than in the non-dialysis patients. Hypertensive dialysis patients had higher calcium scores than non-hypertensive dialysis patients (P < 0.05). A stepwise, multiple regression analysis confirmed the importance of age and hypertension. No correlation between calcium, phosphate, or parathyroid hormone values and the coronary calcium score was identified; however, the calcium score was inversely correlated with bone mass in the dialysis patients (r = 0.47, P < 0.05). The mitral valve was calcified in 59% of dialysis patients, while the aortic valve was calcified in 55%. The coronary artery calcium score was correlated with aortic valvular, but not mitral valvular calcification. A repeat examination of the dialysis patients at an interval of 1 year showed a disturbing tendency for progression. Our data under-score the frequency and severity of coronary and valvular calcifications in dialysis patients, and illustrate the rapid progression of this calcification. Finally, they draw attention to hypertension as an important risk factor in this process.     

The emerging role of MIF in septic shock and infection

A case of successful patch angioplasty for the right coronary artery (RCA) orifice dissection following selective coronary perfusion is reported. A 56-year-old woman who had mitral restenosis, aortic stenosis, and atrial fibrillation with bradycardia-tachycardia syndrome was referred to our hospital for operation. The operation, which contained aortic valve replacement and mitral commissurotomy, was performed with hypothermic cardiopulmonary bypass and crystalloid cardioplegic arrest. The ascending aorta was opened, and selective coronary perfusion was performed. The right coronary cannula was difficult to insert and dislodged several times. At the second infusion of the cardioplegic solution, the right coronary orifice and ascending aortic wall was dissected. The dissection extended to the proximal RCA. The aortotomy was extended into the RCA beyond its orifice. The Xenomedica pericardial patch was used to enlarge the diameter of the RCA with closing the dissected cavity. Then the patch was brought onto the side of the aorta and the aortotomy was closed in the usual manner. Post operative coronary angiography revealed widely patent RCA orifice and good runoff. Two years after operation the patient is free of angina with unlimited physical activity.     

A case report of infective endocarditis with total rupture of the posterior papillary muscle after aortic valve replacement

We reported a case with severe mitral regurgitation caused by total rupture of the posterior papillary muscle two days after aortic valve replacement. A 62-year-old man was transferred to our hospital with high fever and dyspnea with severe aortic regurgitation caused by infective endocarditis. The left heart failure occurred suddenly two days after the initial operation. Echocardiogram revealed massive mitral regurgitation and rupture of the posterior papillary muscle. He underwent emergent mitral valve replacement. Histological examination of the papillary muscle showed typical ischemic necrosis without inflammation. The postoperative course was uneventful. We suggested the papillary muscle rupture in this case may be due to coronary artery emboli occurred in association with infective endocarditis.     

Damage to the circumflex coronary artery during mitral valve repair with sliding leaflet technique

We report a case of damage to the circumflex coronary artery during mitral valve repair using sliding leaflet technique in a patient with a posterior mitral leaflet prolapse and coronary artery disease who underwent mitral valve reconstruction using Carpentier's technique and coronary artery bypass grafting. This case underscores the risk of circumflex coronary artery injury during mitral valve reconstruction, especially in patients with left coronary dominance or codominance, and therefore emphasizes the importance of knowing the coronary artery anatomy preoperatively. The use of intraoperative transesophageal echocardiography is mandatory for the evaluation of mitral valvuloplasty.     

Lidocaine cardioplegia for prevention of reperfusion ventricular fibrillation

Lidocaine addition to crystalloid cardioplegic solution for prevention of reperfusion ventricular fibrillation after the release of the aortic cross-clamp was studied in 50 patients undergoing coronary artery bypass grafting and in 30 patients undergoing mitral or aortic valve replacement. Twenty-six of the patients undergoing coronary artery bypass grafting received lidocaine, 100 mg/L of cardioplegia, whereas a control group of 24 patients received cardioplegia without lidocaine. In the group undergoing valve replacement, 14 patients received lidocaine cardioplegia and 16 patients served as control. In the coronary artery bypass grafting group, lidocaine cardioplegia reduced significantly the incidence of reperfusion ventricular fibrillation from 100% to 42%. In the valve group, lidocaine cardioplegia also reduced significantly the incidence of reperfusion ventricular fibrillation from 93% to 42%. In both groups, lidocaine cardioplegia decreased the number of direct-current countershocks required to defibrillate the heart, with no significant increase in the incidence of high-grade atrioventricular block.     

Resolution of an aortic mobile mass with anticoagulation without evidence of arterial embolism

The prognostic significance of aortic mobile debris detected by transesophageal echocardiography (TEE) in patients without history of embolism has not been established. A mobile aortic arch mass was found by TEE in a 59-year-old man with coronary artery disease, and with rheumatic mitral valve disease, and with no embolic symptoms. The patient was anticoagulated for 6 weeks and the mass was no longer seen on repeated TEE. He had no embolic symptoms during 9 months of follow-up. Different therapeutic approaches to mobile aortic debris are discussed and anticoagulant treatment of asymptomatic cases is advocated.     

Prevalence of heart disease in older women in a nursing home

The prevalence of coronary artery disease (CAD) and the incidence of new coronary events are similar in older men and women. Independent risk factors for new coronary events in older women include age, prior CAD, cigarette smoking, hypertension, diabetes mellitus, high serum total cholesterol and triglycerides, and low serum high-density lipoprotein cholesterol. Older women have a higher prevalence of hypertension than older men. In older women with hypertension, echocardiographic left ventricular hypertrophy is a powerful independent predictor of new coronary events, atherothrombotic brain infarction, and congestive heart failure (CHF). Older women have a higher prevalence of rheumatic mitral stenosis and of mitral annular calcium than older men. Older women and men have a similar prevalence of valvular aortic stenosis, aortic regurgitation, mitral regurgitation, hypertrophic cardiomyopathy, and idiopathic dilated cardiomyopathy. The prevalence and incidence of CHF increase with age. The prevalence of normal left ventricular ejection fraction associated with CHF increases with age and is higher in older women than in older men. The prevalence of chronic atrial fibrillation increases with age and is similar in older men and women. Atrial fibrillation is an independent predictor of new coronary events and thromboembolic stroke in older women. Older women with unexplained syncope should have 24-hour ambulatory electrocardiograms to determine whether pauses > 3 seconds are present, requiring permanent pacemaker implantation.     

Coronary artery dissection - a complication of cardiac catheterization without sequelae: case report and review of the literature

This report describes an acute right coronary artery dissection occurring during diagnostic cardiac catheterization. Following catheter manipulation in the vicinity of the aortic valve, the patient complained of mild chest discomfort and had transient electrocardiographic evidence of acute inferior wall myocardial infarction with runs of 2:1 atrioventricular block. Within 5 min, the EKG reverted to precatheterization appearance, and the patient was asymptomatic. Coronary angiography revealed a dissection of the proximal vessel without obstruction. The patient had no clinical sequelae while monitored in the intensive care unit. The patient underwent elective aortic and mitral valve replacement. The area of the dissection was directly visualized, and no abnormality was noted. We review the literature of spontaneous and iatrogenic coronary artery dissections with regard to pathology, diagnosis, and prognosis, and make recommendations for therapy.     

Successful aorto-circumflex coronary bypass grafting through left thoracotomy following aortic and mitral valve replacement

We report successful descending thoracic aorto-circumflex coronary artery bypass grafting using a saphenous vein graft through left thoracotomy in a 44-year-old female. The patient developed severe angina attack after aortic and mitral valve replacement. Coronary angiography showed 99% stenosis of the circumflex coronary artery. Under general anesthesia, left femoral vein to arterial partial cardiopulmonary bypass was performed via left 4 th intercostal space. Body temperature was lowered to 22 degrees centigrade, and spontaneous cardiac fibrillation occurred. After minimal exposure by pericardial dissection of the circumflex coronary artery, distal anastomosis and then proximal anastomosis at the descending thoracic aorta was carried out under cardiac fibrillation. This surgery was done with minimal intra-and postoperative bleeding. Postoperative course was very smooth, and the patient was discharged and leading a normal life for 6 months after surgery.     

Relationships between left ventricular mass, left ventricular work and coronary artery size in aortic regurgitation--possible mechanism of myocardial ischemia

To investigate the relationships between coronary artery size, left ventricular (LV) mass, and LV stroke work in aortic regurgitation (AR), these values were measured in 19 patients with severe AR. Twenty normal subjects and 15 patients with mitral regurgitation (MR) were used as control groups. The coronary area index, i.e., the coronary cross-sectional area divided by body surface area (BSA), was larger in the AR group than in the control groups in all measured sites except for the peripheral left anterior descending coronary artery (LAD) and right coronary artery (RCA). However, the coronary area index divided by the LV mass was significantly smaller in AR and MR patients than in normal subjects. Furthermore, the coronary area index divided by LV stroke work was smaller in AR patients than in MR patients and normal subjects. These results suggest that the coronary blood flow associated with the increased LV mass and stroke work caused by regurgitation was insufficient in patients with severe AR, especially in the area of the LAD. Therefore, the occurrence of myocardial ischemia in patients with severe AR may involve inadequate enlargement of the coronary artery which perfuses the LV, in addition to factors such as decreased coronary perfusion pressure, increased coronary artery resistance and decreased coronary flow reserve.     

Risk of reoperative valve replacement for failed mitral and aortic bioprostheses

BACKGROUND: One factor influencing the choice of mechanical versus bioprosthetic valves is reoperation for bioprosthetic valve failure. To define its operative risk, we reviewed our results with valve reoperation for bioprosthetic valve failure. METHODS: Records of 400 consecutive patients having reoperative mitral, aortic, or mitral and aortic bioprosthetic valve replacement from January 1985 to March 1997 were reviewed. RESULTS: Reoperations were for failed bioprosthetic mitral valves in 219 patients, failed aortic valves in 153 patients, and failed aortic and mitral valves in 28 patients. Including 26 operations (6%) for acute endocarditis, 153 operations (38%) were nonelective. One hundred nine patients (27%) had other valves repaired or replaced, and 72 (18%) had coronary bypass grafting. The incidence of death in the mitral, aortic, and double-valve groups was respectively, 15 (6.8%), 12 (7.8%), and 4 (14.3%); and the incidence of prolonged postoperative hospital stay (>14 days) was, respectively, 57 (26.0%), 41 (26.8%), and 8 (28.6%). Only 7 of 147 patients (4.8%) having elective, isolated, first-time valve reoperation died. Multivariable predictors (p < 0.05) of hospital death were age greater than 65 years, male sex, renal insufficiency, and nonelective operation; and predictors of prolonged stay were acute endocarditis, renal insufficiency, any concurrent cardiac operation, and elevated pulmonary artery systolic pressure. CONCLUSIONS: Reoperative bioprosthetic valve replacement can be performed with acceptable mortality and hospital stay. The best results are achieved with elective valve replacement, without concurrent cardiac procedures.     

Minimally invasive heart surgery--a fad or a clinically recognized therapy method?

Within the last 5 years new less invasive surgical techniques have been developed in the field of cardiac surgery. This new field named "minimally invasive cardiac surgery" can be subdivided into techniques which do not require cardiopulmonary bypass and are used mainly for coronary artery surgery (called minimally invasive direct coronary artery surgery, MIDCAB technique). This MIDCAB procedure can be done through a small left anterior thoracotomy or a sternotomy. In addition there are other methods which allow the performance of complex cardiac surgery through small accesses in combination with the use of an endovascular CPB system and internal aortic clamping to achieve cardioplegic arrest (so-called Port-Access method). Also for valvular surgery, new surgical techniques were developed allowing access to mitral and aortic valves through limited incisions. In addition, new less invasive techniques were developed for congenital heart surgery. This article will describe the various surgical techniques and define the indications for minimally invasive cardiac surgery.     

The usefulness of Doppler echocardiography in the preoperative assessment of valvulopathies. A comparison with the hemodynamic and surgical findings

The Doppler echocardiography and cardiac catheterization studies of all patients who underwent valvular surgery in a three-year period were reviewed to assess the correlation between the estimated severity of valvular disease by both methods. Two-hundred and thirty-five patients (group I: 140 male, age 58 +/- 12; 95 female, age 60 +/- 13) underwent both studies within 6 months. There was agreement on estimation of severity of valve lesions in 140 of 162 patients with aortic valve disease (93% of stenosis, 82% of regurgitations and 79% of mixed lesions), in 58 of 80 patients with mitral valve disease (83% of stenosis, 76% of regurgitations and 33% of mixed lesions) and in 10 of 16 patients with prosthetic valve disfunction. The correlation between both methods was significantly lower in mixed mitral lesions than in the remaining native valve lesions (p < 0.05). Significant disagreement occurred in 4 cases of aortic valve disease, four of mitral valve disease and five of prosthetic disfunction. When disagreement was present, Doppler often underestimated the severity of the disease. Disagreement was more frequent in patients with combined aortic and mitral disease. According to the surgical conclusions cardiac catheterization provided a diagnostic profit in the assessment of the disease severity in 8, 11 and 22% of cases of aortic and mitral valve disease and prosthetic valve disfunction, respectively. Coronary artery disease was present in 19% of patients who underwent coronary arteriography. One-hundred and two patients (group II: 44 m, 48 +/- 15; 58 f, 53 +/- 11) underwent surgery without previous cardiac catheterization.(ABSTRACT TRUNCATED AT 250 WORDS)     

Late survival after valve operation in patients with left ventricular dysfunction

BACKGROUND: Left ventricular dysfunction is a predictor of hospital mortality after cardiac valve operation. We evaluated late survival in a large cohort of these patients. METHODS: From 1980 to 1993, 257 patients with a preoperative ejection fraction of 0.40 or less underwent aortic (n = 177), mitral (n = 72), or combined (n = 8) valve operation, with or without concomitant coronary artery bypass grafting. RESULTS: Hospital mortality was 12.5%. Follow-up was 98% complete. Logistic regression analysis showed that an ejection fraction of less than 0.30, mitral regurgitation, concomitant coronary artery bypass grafting, emergency operation, and reoperation were independent correlates of hospital mortality (all at p < 0.05). Kaplan-Meier survival curves of the 220 hospital survivors showed a 65% 5-year survival. Multivariate analysis revealed preoperative use of diuretics, male sex, reoperation, age exceeding 60 years, and aortic regurgitation to be independent predictors of poor late outcome (all at p < 0.05). CONCLUSIONS: The liability of left ventricular dysfunction with regard to diminished long-term survival is not completely reversed by valve operation. If operation is not performed before left ventricular dysfunction develops, postoperative medical treatment of these dilated, remodeled ventricles should be considered.     

Elective stenting of carotid artery stenosis in patients with severe coronary artery disease

AIMS: To evaluate the feasibility and safety of elective carotid stent implantation in patients with carotid stenoses and concomitant coronary artery disease, as an alternative to combined carotid and coronary surgery. METHODS: We treated 50 patients with >70%, stenoses in 53 carotid arteries with balloon angioplasty followed by elective stent implantation. All patients had severe coronary artery disease, and/or mitral insufficiency, aortic stenosis, rhythm disorders or generalized arteriosclerosis. In three patients the opposite carotid artery was occluded; nine patients had bilateral stenoses of which two received stents bilaterally. RESULTS: Fifty-six successful stent implantations (42 Wallstents, eight BeStents, two AVE-Microstents, one Palmaz Schatz stent, three Sito stents) were performed, reducing the baseline percent stenosis from 78 +/- 18%, to 13 +/- 11%. Complications included three transient ischaemic attacks, one minor and one major stroke. Follow-up was available for 46 patients over a mean of 10 months. Three asymptomatic restenoses and one deformation of a BeStent occurred. CONCLUSION: Our preliminary results indicate that carotid artery stenting in patients with concomitant severe coronary artery disease is feasible, safe, and may be an alternative to combined carotid and coronary surgery.     

Mycotic aneurysm of the left coronary artery

We report a 24-year-old man with mitral valve endocarditis complicated by acute myocardial infarction due to coronary embolism. Percutaneous transluminal coronary angioplasty and subsequent mitral valve replacement were performed. Postoperative coronary angiography revealed formation of a mycotic aneurysm of the left anterior descending coronary artery at the site of balloon inflation. The patient then underwent successful resection of the aneurysm with coronary artery bypass grafting.