Should a bicuspid aortic valve be replaced in the presence of subvalvar or supravalvar aortic stenosis?

BACKGROUND: A bicuspid aortic valve is commonly associated with other levels of left ventricular outflow tract obstruction. Providing the bicuspid aortic valve is competent and nonobstructive, repair of subvalvar or supravalvar stenosis usually focuses on the obstructive lesions, leaving the valve in situ. The aim of this report was to examine the impact of a bicuspid aortic valve on the risk of reoperation for patients undergoing operation for subvalvar or supravalvar aortic stenosis. METHODS: Since 1976, 47 patients with supravalvar or subvalvar aortic stenosis have undergone repair. The median follow-up is 5.1 years (range, 2 months to 20.1 years). Sixteen patients (34%) had a bicuspid aortic valve that was competent and nonobstructive, and 31 (66%) had a tricuspid aortic valve. RESULTS: Reoperation was required in 9 patients (56%) with a bicuspid aortic valve, in each involving aortic valve replacement with an autograft (3), homograft (2), or prosthesis (4). Six patients (19%) with a tricuspid aortic valve required reoperation, yet only 1 required aortic valve replacement. The freedom from valve replacement was 43% (70% confidence interval, 31% to 55%) in the bicuspid aortic valve group versus 100% (70% confidence interval, 94% to 99.5%) in the tricuspid group at 5 years (p = 0.0001). The freedom from any reoperation at 5 years was 43% (70% confidence interval, 31% to 55%) in patients with a bicuspid aortic valve versus 86% (70% confidence interval, 80% to 93%) in the tricuspid group (p = 0.02). CONCLUSIONS: The data suggest that patients with subvalvar or supravalvar aortic stenosis and a bicuspid valve may be better palliated with a more definitive operation such as the Ross or Ross-Konno procedure.     

The metabolic and cardiovascular consequences of HRT

In a 39-year-old man an isolated, unruptured extracardiac aneurysm of the left sinus of Valsalva led to almost complete rarefication of one aortic valve leaflet, causing insufficiency of the valve. At operation the aneurysm entrance was closed with a patch and prosthetic replacement of the bicuspid aortic valve was performed. The result was satisfactory.     

Compression of anomalous circumflex coronary artery by a prosthetic valve ring

Anomalous origin of the circumflex coronary artery from the right aortic sinus, with a retroaortic course, is usually without consequence. We report a patient who underwent aortic valve replacement for bicuspid aortic valve. The prosthesis sewing ring distorted the circumflex, producing myocardial infarcts and sudden death during exercise.     

Esophageal patching for an unsuturable tracheoesophageal fistula

We report on the case of a 45-year-old man with recurrent syncope and angina with shortness of breath on exertion. Invasive and noninvasive diagnostic methods revealed severely stenosed bicuspid aortic valve, postductal coarctation of the aorta, and a coronary artery-descending aorta fistula. After surgical correction of the coarctation, ligation of the fistula, and aortic valve replacement, the patient's symptoms resolved.     

Helicobacter infection in man: problems to be solved

BACKGROUND: The pulmonary autograft approaches the ideal aortic valve substitute but numerous technical modifications are debated. The valves involved often differ in diameter and shape of the recipient annulus. We previously reported simplified surgical rules and early results of 52 patients and here we extend the report to 76 patients and analyze the mid-term results with respect to valve mismatch. METHODS: We studied the influence of geometric discrepancies. Valve regurgitation and gradient are compared for size discrepancies up to or more than 3 mm between recipient annulus and pulmonary autograft. The normal tricuspid recipient aortic annulus is compared with the flat circular redo-prosthetic or bicuspid annulus. Patients with plication of the aortic annulus and remodeling of the distal aorta are reviewed, and lastly donor homograft to pulmonary autograft discrepancies up to and more than 3 mm are compared. RESULTS: The results were comparable in all groups and no significant differences were observed. CONCLUSION: The pulmonary autograft operation may be simplified, with good results at the least in the first year despite operative tailoring due to valve discrepancies.     

Rheumatoid nodules: MR/pathological correlation

A 62-year-old female who had a complaint of palpitation was diagnosed as bicuspid aortic stenosis and left single coronary artery by the echocardiographic examination, cardiac catheterization study and coronary artery angiography. Aortic valve replacement was performed and the patient was discharged 22 days after surgery with good post-operative course. Preoperative coronary artery angiography was important in the case of aortic valvular disease.     

Congenital aortic valve disease. Improved survival and quality of life

OBJECTIVE: The purpose of the study was to assess the effect of recent trends in surgical management, including use of the Ross Operation, on improved survival and quality of life in patients treated surgically for aortic valve (AV) disease at Oklahoma Children's Hospital. BACKGROUND: Surgical treatment of congenital AV disease has proved to be palliative, but newer procedures may be improving outcomes. METHODS: A retrospective review of 301 patients, age 1 day to 26 years (median, 5 years), having a surgical AV procedure or aortic balloon valvuloplasty at Children's Hospital of Oklahoma between 1960 and February 1996, was conducted. Information was collected on all prior and subsequent operations, and follow-up within 1 year was 96% complete. RESULTS: Survival for all patients was 90% +/- 2% at age 10 years and 73% +/- 8% at age 25. By age 5, 52% +/- 4% had required an AV procedure, 89% +/- 3% by age 15. Patient survival was affected adversely by the diagnosis of valvar aortic stenosis, 79% +/- 6% at age 25 compared to 95% +/- 4% for subvalvar aortic stenosis or aortic insufficiency (p = 0.01). The AV morphology did not affect survival, but patients with a bicuspid or unicuspid valve required operative intervention at an earlier age. Survival after autograft replacement of the AV (Ross Operation) was significantly better than for other types of valve replacement (p = 0.0043). Quality of life as assessed by need for reoperation favors the use of the Ross Operation, with freedom from reoperation at 9 years of 87% +/- 7% compared to 55% +/- 5% in all patients after first AV surgery (p = 0.003). CONCLUSIONS: The Ross Operation appears to have a significant advantage in survival and quality of life in children requiring a valve replacement as a first operation or after a prior AV procedure.     

The defective nature of hepatitis delta virus

Forty-nine out of 66 patients with Turner's syndrome and different karyotypes underwent full cardiological evaluations (physical examination, electrocardiography, chest X-ray and echocardiography). Congenital cardiovascular anomalies were found in 11 patients (22.4%). Among the cardiac anomalies in patients with Turner's syndrome, aortic malformations (aortic coarctation [27%], aortic stenosis [18%] and bicuspid aortic valve [18%]) were the most frequent. We observed that the most severe malformations were found in those with karyotype 45,X or 45,X/46,XX. No anomalies were detected in patients with the X isochromosome or those mosaic with the Y component.     

Clinical factors useful in predicting aortic valve structure in patients > 40 years of age with isolated valvular aortic stenosis

A number of reports have described the frequency of coronary arterial narrowing in patients with valvular aortic stenosis. No published reports have examined the structure of the stenotic aortic valve in adults and related the valve structure to variables, including coronary arterial narrowing, useful in predicting that structure. One hundred eighty-eight patients having aortic valve replacement for isolated valvular aortic stenosis were studied. All patients were > 40 years of age at the time of aortic valve replacement, all had coronary angiograms preoperatively, and of 182 patients (97%) measurements of serum total cholesterol had been obtained and 184 (98%) had body mass index calculated. The structure of the operatively excised valve was classified as unicuspid or bicuspid (congenitally malformed), or tricuspid aortic valve. A logistic regression model was developed that found 4 factors (age, serum total cholesterol, angiographic coronary artery disease and body mass index) to be predictive of aortic valve structure: (1) Patients with at least 3 or all 4 factors high or present (i.e., age > 65 years, serum total cholesterol > 200 mg/dl, body mass index > 29 kg/m2 and coronary artery disease) had a low probability (10 to 29%) of having a congenitally malformed valve; (2) patients with at least 3 or all 4 factors low or absent (i.e., age < or = 65 years, serum total cholesterol < or = 200 mg/dl, body mass index < or = 29 kg/m2, and no coronary artery disease) had a high probability (72 to 90%) of having a congenitally malformed valve.(ABSTRACT TRUNCATED AT 250 WORDS)     

Spiral CT angiography in an infant with severe hypoplasia of a long segment of the descending aorta

Annuloaortic ectasia due to Shprintzen-Goldberg syndrome (SGS) is reported. A 10-year-old boy was admitted to our hospital for evaluation of chest pain. On admission, he was diagnosed as SGS on the basis of his various anomalies. Two-dimensional echocardiography showed a bicuspid aortic valve and marked annular dilatation, Doppler flow studies revealed severe aortic regurgitation, and retrograde aortography showed severe aortic regurgitation with annular dilatation. Successful aortic root replacement was performed; subsequent histologic examination of the ascending aorta demonstrated cystic medial necrosis. In conclusion, SGS is a generalized connective tissue dysplasia, with clinical manifestations of cardiovascular lesions similar to those in Marfan syndrome. Aortic root replacement was successfully performed; however, recurrence of aortic aneurysms outside of the ascending aorta should be carefully observed. Surgical treatment for cardiovascular disorders may be necessary to save the life of patients with SGS.     

Congenital heart disease in patients with Turner's syndrome. Italian Study Group for Turner Syndrome (ISGTS)

OBJECTIVE: There is a high prevalence of congenital heart defects in patients with Turner's syndrome. Few studies have reported echocardiographic data in unselected patients according to the different chromosomal patterns. The aim of our study was to evaluate a large series of patients with Turner's syndrome, comparing these data with those of the general population. METHODS: Five hundred ninety-four patients with Turner's syndrome, aged 1 month to 24 years, in the Italian Study Group for Turner Syndrome underwent full cardiologic evaluation. Karyotype distribution was: 45,X (54%), X-mosaicism (13%), and X-structural abnormalities (33%). RESULTS: The prevalence of cardiac malformations was 23%. Bicuspid aortic valve (12.5%), aortic coarctation (6.9%), and aortic valve disease (3.2%) were the most prevalent malformations. In comparison with the general population, partial anomalous pulmonary venous drainage had the highest relative risk. A correlation was found between type of congenital heart defect and karyotype. The patients with 45,X karyotype had the greatest prevalence of partial anomalous pulmonary venous drainage and aortic coarctation, whereas bicuspid aortic valve and aortic valve disease were more common in the patients with X-structural abnormalities. The patients with severe dysmorphic signs showed a significantly higher relative risk of cardiac malformations. CONCLUSION: X-linked factors may be involved in determining cardiac defects in Turner's syndrome.     

Aortic valve replacement with biological substitute

In the quest for an ideal aortic valve substitute, homografts and autografts are well-established options. We reviewed our results with homografts and autografts for aortic valve replacement during the last 5 years. From March 1992 through July 1997, 189 patients (138 male and 51 female), age 8 months to 68 years (mean 31.0+/-4.2 years), underwent aortic valve replacement with a human biological substitute. Of these, 93 patients received a cryopreserved or antibiotic-preserved aortic/pulmonary homograft, whereas 96 patients underwent a Ross procedure. Etiology was rheumatic in 143 (75.6%) patients, bicuspid aortic valve in 40 (21.2%), Marfan's disease in 5 (2.6%), and myxomatous aortitis in 1 (0.5%). Among the homograft group, a scalloped subcoronary implantation technique was used in 54 patients, whereas 32 patients underwent root replacement. Five patients required aortic root and ascending aortia replacement for annuloaortic ectasia. In all patients undergoing the Ross procedure, a root replacement technique was used. Operative mortality was 7.4% (14 patients). Late mortality was 5.3% (10 patients). Follow-up ranged from 1 to 46 months postoperatively. In patients with homograft aortic valve replacement, 76 patients (91.5%) had trivial to mild aortic regurgitation, while 7 patients (8.4%) had important aortic regurgitation. In patients with the Ross procedure, 78 patients (89.6%) had trivial to mild regurgitation. Moderate to severe aortic regurgitation was present in 9 patients (10.3%), all of whom had rheumatic heart disease and were young (< 30 years at surgery). We conclude that homografts and autografts provide an excellent substitute for the diseased aortic valve. Young age (< 30 years) with rheumatic etiology is a major risk factor for early progressive aortic regurgitation in patients undergoing the Ross procedure.     

Congenitally bicuspid aortic valves: a surgical pathology study of 542 cases (1991 through 1996) and a literature review of 2,715 additional cases

OBJECTIVE: To describe a clinicopathologic study of a large group of congenitally bicuspid aortic valves surgically excised at a single institution. MATERIAL AND METHODS: The medical charts and bicuspid valves from patients undergoing aortic valve replacement at Mayo Clinic Rochester between 1991 and 1996 were retrospectively reviewed. RESULTS: The age of the 542 patients ranged from 1 to 86 years (mean, 61), and 372 (69%) were men. Among these, 409 (75%) had pure aortic stenosis (AS), 73 (13%) had pure aortic insufficiency (regurgitation) (AI), 53 (10%) had combined AS and AI, and 7 (1%) had normal function. The mean age was higher for those with AS than AI (65 versus 46 years; P < 0.001), whereas the male-to-female ratio was higher for AI than AS (17.3:1 versus 1.7:1; P < 0.001). The two cusps were not equal in size in 95%, and a raphe was present in 76% (67% typical, 9% atypical). Raphal position was described in 315 and was between the right and left cusps in 270 (86%). Raphal absence occurred more often in valves with equal-sized cusps than unequal (33% versus 14%; P = 0.005). Moderate to severe calcification affected valves with AS more frequently than AI (99% versus 41%; P < 0.001). In contrast, annular dilatation was associated with AI more than AS (48% versus 11%; P < 0.001). Acquired commissural fusion involved valves with combined AS and AI more often than the other functional states (31% versus 14%; P = 0.002). Sixteen patients (age range, 18 to 78 years; 13 men) had infective endocarditis (6 active, 10 healed), including 10 with AI (9 men), 3 with AS plus AI, 2 with AS, and 1 with normal function but embolization. CONCLUSION: Functionally, the most common fate of congenitally bicuspid aortic valves was calcific stenosis with or without regurgitation (85%). Because approximately 4 million US citizens have bicuspid valves and because valve replacement is currently the only treatment of symptomatic AS, this disorder will continue to affect health-care costs.     

Studying electric field effects on embryonic myocytes

OBJECTIVE: Little attention has been paid to the occurrence of aortic regurgitation after complete repair in patients with pulmonary atresia and ventricular septal defect or tetralogy of Fallot. To highlight the development of aortic regurgitation or aortic root dilation severe enough to necessitate aortic valve replacement with or without aortic aneurysmorrhaphy or aortic root replacement, we retrospectively reviewed the records of patients who underwent aortic valve operation at our institution subsequent to repair of pulmonary atresia and ventricular septal defect or tetralogy of Fallot. METHODS: We searched the Mayo Clinic database for patients with pulmonary atresia and ventricular septal defect or tetralogy of Fallot who subsequently had aortic valve or aortic root operations. The degree of aortic regurgitation before operation was noted. Aortic sinus and root dimensions were measured. RESULTS: Sixteen patients underwent complete repair at a median age of 17 years, followed by an aortic operation a median of 13.5 years later. All 16 patients had dilated aortic sinuses at the time of the aortic valve operation. These 16 patients had aortic valve replacement: 11 with mechanical prostheses and 5 with bioprostheses. Five of the 16 also had reduction of aortic dilation by lateral aneurysmorrhaphy, and 1 had graft replacement of the ascending aorta. Five patients had associated conditions (evidence of valvular damage, recurrent ventricular septal defect, or history of endocarditis) discovered at the aortic valve operation that have been reported to be related to the development of aortic regurgitation. The remaining 11 patients had progressive aortic regurgitation despite complete, uncomplicated repair. CONCLUSIONS: Progressive aortic regurgitation and aortic root dilation can occur despite complete repair of pulmonary atresia and ventricular septal defect or tetralogy of Fallot.     

Prevalence of heart disease in school children in rural Kenya using colour-flow echocardiography

In a survey using colour-flow echocardiography and a portable generator, of one thousand one hundred fifteen children, three had clinical and echocardiographic evidence of rheumatic heart disease giving a prevalence rate of 27/1000. Sixty nine (6.2%) of the children examined had trivial mitral regurgitation (TMR). Of these, three had associated trivial aortic regurgitation. Isolated trivial aortic regurgitation was not seen. Four children had isolated mild-moderate regurgitation of the pulmonary valve. Congenital heart disease (CHD) was found in two children-one with secundum atrial septal defect and one with a ventricular septal defect and pulmonary stenosis, giving a prevalence of 1.8/1000. One child had a bicuspid aortic valve and two persistent left superior vena cava. It is feasible to carry out an echocardiographic survey using a portable generator in schools where electricity is not available. The prevalence of rheumatic heart disease (RHD) is higher than previously found in Kenya. The prevalence of TMR is surprisingly high. This was associated with familial clustering reminiscent of RHD. The importance of these findings must await the results of further investigation.     

Sub-trochanteric fractures. A comparative study between gamma nail and angular osteosynthesis with lateral cortical support]

METHODS: Thirty-three children and young adults with congenital aortic valve disease underwent pulmonary autograft replacement of the aortic valve between October 1993 and March 1997. There wer six females and 27 males; at operation, median age was 16 years (range: 3 to 41 years) and median body weight 60 kg (range: 14 to 121 kg). Fifteen patients (46%) had undergone one or more previous cardiac surgical procedures. A bicuspid aortic valve was present in 31 patients (94%); moderate to severe aortic stenosis and regurgitation was present in 10 (30%) and 26 (79%), respectively. RESULTS: All patients underwent the Ross procedure while in NYHA class I (64%) or class II (36%). A preoperative shortening fraction of 41 +/- 1.4% suggested well-preserved systolic function, but the mean left ventricular end-diastolic pressure of 16.6 +/- 1.3 mmHg was consistent with preoperative left ventricular pressure and volume overload. The aortic root was replaced using an interrupted suture technique in two patients and with three separate running sutures in 31. The right ventricular outflow tract was reconstructed in all classes with a cryopreserved pulmonary homograft valved conduit (median diameter 23 mm; range 19 to 30 mm). Intraoperative complications included transient atrioventricular dissociation (one), permanent atrioventricular dissociation (one), and left coronary artery distortion relieved by shortening the distal ascending aorta (one). Postoperatively, postpericardiotomy syndrome developed in six patients (18%), supraventricular tachycardia in three (9%), and ventricular tachycardia in one (3%). At three days after surgery, one patient developed ischemic left ventricular dysfunction requiring repositioning of the distorted left coronary artery higher on the neo-aortic root. Hospital survival rate was 100%. During a median follow-up of 17 months (range: 1 to 41 months) one patient suffered a non-cardiac death due to blunt trauma. there has been a significant postoperative improvement in NYHA class among surviving patients (class I, 94%; class II, 6%; p = 0.004 versus preoperative). Postoperative aortic regurgitation was absent or trivial in 17 (60%) and mild in the remaining 11 (40%) patients for whom follow-up echocardiographic data are available. One patient required reoperation 16.5 months after the Ross procedure to replace a rapidly degenerating pulmonary homograft, and one with moderately severe homograft stenosis and five with mild homograft stenosis are being monitored. Postoperatively, a gradual early expansion in the diameter of the neo-aortic root and reduction in echocardiographic indices of left ventricular hypertrophy and dilatation occurred. CONCLUSIONS: Pulmonary autograft replacement of the aortic valve in young patients with congenital aortic valve disease has produced excellent short-term anatomic/physiologic results and symptomatic relief with no mortality. Indices of left ventricular dilatation and hypertrophy regress after repair when the Ross operation precedes important deterioration in preoperative ventricular function. Important technical considerations include: (i) the native distal ascending aorta should be sufficiently shortened before performing the distal aortic anastomosis; and (ii) the left coronary anastomosis should be positioned relatively high on the neo-aortic root with a slight amount of tension. Both of these maneuvers reduce the likelihood of coronary artery distortion. Rapid degeneration of the pulmonary homograft and the propensity towards progressive dilatation of the neo-aorta are important postoperative considerations. Until more is known about the etiology and natural history of these two potential complications, postoperative anti-inflammatory and/or immunosuppressive therapy and strict control of hypertension should be strongly considered.     

Aortic valve tumor causing acute myocardial infarction in a child

A 10-year-old child presented with biventricular failure after an acute myocardial infarction. On investigation a large tumor arising from the aortic valve was diagnosed. The patient underwent successful aortic valve replacement for complete excision of the tumor mass, which was reported to be a papillary fibroelastoma. This case report highlights the unusual presentation of an aortic valve tumor in a child with myocardial infarction. The surgical dilemmas of the timing of the operation and the nature of the operation are discussed.     

Redo operation after surgery for aortic aneurysm and dissection

Thirteen patients who underwent redo operation after surgical treatment of aortic aneurysm and dissection were presented. In 8 patients, redo operations were performed for aortic dissection following aortic valve replacement. A-C bypass, the Koster-Collins operation and replacement of thoracic aorta. In the other 5 patients, the reasons for redo operation were aortic root enlargement after replacement of ascending aorta and aortic valve replacement, pseudoaneurysm and aneurysmal dilatation around coronary button for the Bentall operation and recurrent aneurysm after patch aortoplasty and thoracoabdominal replacement using the Crawford's maneuver. To prevent these redo operation, adequate selection of surgical procedures and meticulous operative techniques should be required in primary operation.     

Focus on bovine products

Echocardiographic (ECHO) findings in 211 consecutive examinations performed at Eldoret Medical Services from August 1992 to May 1994 were analysed. Most patients were referred by physicians from Eldoret and from the surrounding hospitals for assessment of heart failure or a heart murmur. Thirty three echocardiograms were judged as being normal and 177 abnormal. Of the abnormal ECHOs, congenital heart disease accounted for 38.4% while rheumatic heart disease accounted for 40.7%: Of the 68 patients with congenital defects, 21 (30.1%) had ventricular septal defects; 19 (27.9%) persistent ductus arteriosus; five (7.5%) mitral valve; four (5.9%) tetralogy of Fallot; and three (4.4%) atrial septal defects. In addition, there were two (3.0%) patients each with complete atrio-ventricular septal defects, tricuspid atresia, complete transposition of the great arteries, truncus arteriosus, single ventricle and bicuspid aortic valve. Of the 67 patients with uncomplicated rheumatic heart disease, mitral valve involvement alone was seen in 39 (58.2%), mitral and aortic in 26 (38.8%) and aortic valve alone in two (3.0%). Rheumatic heart disease was complicated by bacterial endocarditis in five patients. Nine patients had pericardial effusion. The possible role of colour-flow ECHO as a feasible, non-invasive and, in the long term, cost-effective means of allowing for early detection and timely institution of secondary prophylaxis while rheumatic heart disease is still asymptomatic is highlighted.     

Sporicidal effect of Presept and Chloramine B on Bacillus cereus spores

Recently the number of plastic operations of the cardiac valves is increasing. The authors present an account on 10 patients with stenosis of the aortic valve where they performed a reconstruction without the necessity of a prosthesis. Commissurotomy and rasping can be performed in patients when the basic anatomical shape and dimensions of the valve are preserved. None of the patients died, one was successfully reoperated on account of aortic insufficiency. The authors describe and discuss the tactics and technique of the operation. They discuss the possibility of reconstruction of the aortic valve in patients indicated for aortocoronary reconstruction where the aortic defect appears to be of minor impact.