Familial bilateral blepharoptosis and subvalvular aortic stenosis

PURPOSE: To select ideal radiologic projections for mapping and ablation of tachycardias of right ventricular outflow tract (RVOT). METHODS: Ten hearts from human corpses were studied utilizing radiopaque material to identify the pulmonary valve and three distinct sites on this valve: septal anterior (A), septal posterior (P) and free-wall (L). Next, the hearts were filmed in the frontal plane and in oblique projections with 15 degrees increments to the right and to the left. The projections in which the sites were lateralized on the valve, eased radiologic interpretation and were considered ideal for mapping and ablation. Depending on the proximity of the sites to the lateral extremes of the pulmonary valve, the projections were considered ideal ( ), intermediary (++) and inadequate (+). RESULTS: Projections [table: see text] CONCLUSION: The A site of RVOT was best indicated in the 60 and 45 degrees left anterior oblique projections; the 0 degree postero anterior projection was best for mapping the P site; the L region was best explored in the 60 degrees right anterior oblique projection.     

Stents in aortic coarctation. Immediate results

From September 1996 to March 1997 we implanted stents Palmaz (P-308 Johnson & Johnson) in six patients with aortic coarctation. Age ranged from 13 to 30 years (mean = 20), 3 female and 3 male. We implanted the stent without predilation. Balloon diameter was 12 mm in two cases and 15 mm in four cases for complete expansion we used 14 to 20 mm balloon diameter in five instances and in one case we used a dual balloon 15 + 15 mm. The gradient pre-stent ranged from 30 to 65 mmHg (mean = 44) and decreased to 0 mmHg in five cases and in one patient the residual gradient was 4 mmHg. Systolic aortic pressure pre-stent was 135 mmHg and decreased to 117 mmHg. There were no significant complications. In conclusion, we have demonstrated the feasibility of balloon-expandable stent implantation as a method of alleviation the obstruction in coarctation of the aorta in this group of patients. It is an excellent alternative to surgical treatment and it is better than balloon angioplasty that results in marked improvement in the angiographic appearance of the thoracic aorta. It eliminates the gradient, has minimum morbidity and no mortality, no aneurysm formation. Late restenosis and possible aneurysm formation remains an unlikely complication in view of the ample luminal diameter and the high velocity of flow.     

Surgical treatment of aneurysms of the left ventricle. Immediate and long-term results. Apropos of a consecutive series of 121 cases

From 1978 to 1992, 121 cases of postinfarction left ventricular aneurysm (99 males, 22 females, mean age 60 years) were operated on. The authors insist on a high rate of clinical arhythmogenicity (31.4%) and associated mechanical complications (21%). 76% of patients were in functional NYHA class III or IV. Resection was performed in 90% of patients, plication in 10%. 58% underwent coronary artery bypass grafting (1.7 graft/patient), 16% encircling ventriculotomy, 8% mitral valve replacement and 13% closure of ventricular septal defect. Operative mortality was 14.9% (10% when other mechanical complications where excluded). 5-year survival is 67.9%. Late cardiac deaths are as follow: left ventricular failure (1.8% A/P), Sudden death (1.4% A/P), Myocardial infarction (0.6% A/P). 82% of survivals are in functional NYHA class I or II. Only functional class NYHA III or IV is predictive of late death. We conclude that postinfarction left ventricular aneurysm remains a high risk complication especially when associated with other mechanical complications. When arhythmogenicity is present we suggest rhythmologic surgery and in all cases, complete revascularization.     

Surgical repair for supravalvular aortic stenosis: intermediate to long-term follow-up

The prevalence of different malocclusion features was investigated in 396 6-year-old Icelandic children, using the epidemiologic registration method described by Bj?rk et al. in 1964. Girls were ahead of boys with regard to dental stage (P < 0.01). One or more permanent teeth were congenitally missing in 5% of the children. Postnormal occlusion was found in 27% of the boys and in 31% of the girls, and prenormal occlusion was found in 6% and 5%, respectively. Straight terminal plane at the second deciduous molars was found in individuals with either normal or postnormal occlusion. Thus, it can be misleading to use the relation of the terminal planes as a measurement of the sagittal relation between the jaws. The prevalence of hypodontia was much lower than has been reported previously for Icelandic children.     

Immediate and long-term results of the treatment in cancer of the large intestine

Operative treatment of 200 patients with carcinoma of the rectum is analysed. Planned operations were conducted on 101 patients, postoperative mortality was 7.8%. Surgery was undertaken in 93 patients with acute intestinal obstruction, postoperative mortality was 15.05%. Changes of the policy of treatment of patients with acute intestinal obstruction reduced postoperative mortality. Five-year survival was 67.1%.     

Surgical treatment of choledochal cyst in adults: results and long-term follow-up

BACKGROUND/AIMS: Results of the surgical management of 60 adults with choledochal cysts at Chang Gung Memorial Hospital Taipei are presented. PATIENTS AND METHODS: All patients were diagnosed and surgically managed during the period between March 1979 and December 1992. There were 47 females and 13 males, with ages ranging from 16 to 81 years (mean age = 31.9 years). Of the total, there were 41 Type I, one Type II, 14 Type IV, and four Type V choledochal cysts classified according to Todani's classification system. The definite surgical procedures were cyst excision and hepatico-jejunostomy in 43 cases in type I and IV diseases, one cyst excision in type II disease, two hepatectomy for type V disease, cyst enterostomy in seven cases, and T-tube choledochocystostomy in seven cases. RESULTS: The operative mortality rate was 3.3% and the complication rate related to the surgical procedure was 20%. Early postoperative complications include wound infection, leakage of anastomosis, acute pancreatitis, septic shock, left pleural effusion, and intra-abdominal abscessing. Reoperations were needed in the early postoperative days in two cases due to hepatico-jejunostomy leakage and intraabdominal abscess formation. Long-term surgical outcome, concerning recurrence of symptoms, was related to the following factors, excluding the factors of surgery: presence of bile duct lithiasis, common channel less than 22 mm, presence of preoperative pancreatitis and acute angle type pancreaticobiliary unions. Only common channel less than 22 mm is statistically significant. The percentage of recurrence of the symptoms after resectional surgery was 32.5% in the long term follow-up period. Most of the cases responded well to an antibiotics treatment. Three of the cases required a second operation or PTCD as well as dilatation to treat the recurrent cholangitis. CONCLUSION: Although the recurrence of symptoms is not uncommon and satisfactory explanation of the analysed factors is still the treatment of choice for adult patients with choledochal cysts.     

Clinical and hemodynamic follow-up of left ventricular to aortic conduits in patients with aortic stenosis

To assess the long-term results of left ventricular outflow tract reconstruction utilizing an apical left ventricular to aortic valved (porcine) conduit the clinical and hemodynamic data were reviewed from 24 patients who had placement of an apico-aortic conduit. Eighteen of the patients are asymptomatic and taking no cardiac medications. Three patients were reoperated on, one patient 1.5 years after his original operation for subacute bacterial endocarditis and two patients 3 to 4 years after their original operation for severe conduit valve insufficiency. None of the patients is taking anticoagulants and no thromboembolic events have occurred. Postoperative catheterization has been performed 1 to 1.5 years (mean 1.2) after repair in 15 of 21 patients. The rest left ventricular outflow tract gradient has decreased from 102.5 +/- 20 mm Hg preoperatively to 14.8 +/- 9.9 mm Hg postoperatively (probability [p] less than 0.001). Some degree of conduit obstruction was demonstrated by catheter passage in 11 of the 15 patients. In these 11 patients, the obstruction occurred at three distant sites: at the egress of the left ventricle in 9, at the porcine valve in 5 and at the aortic to conduit junction in 1. Isometric exercise in five and supine bicycle exercise in six patients increased the left ventricular outflow tract gradient by 2.5 +/- 1.1 and 20.8 +/- 11.8 mm Hg, respectively, despite an increase in cardiac index of 1 +/- 0.3 and 3.7 +/- 0.4 liters/min per m2, respectively. The data suggest that a left ventricular to aortic conduit is an effective form of therapy for severe left ventricular outflow tract obstruction.     

Diagnosis and treatment of aortic valve stenosis

The present review has attempted to summarize the classic symptoms and signs of aortic valve stenosis, especially in an adult. It is emphasized that all the classic signs rarely are present and their absence may mislead an unwary clinician. The diagnostic help provided by noninvasive tests, including echocardiography and phonocardiography, has been emphasized. A need for cardiac catheterization and angiography in most patients prior to corrective surgery is stressed. The natural history of the disease without operative intervention is dim and a significant risk of sudden death exists. The current surgical approach with immediate and long-term results is summarized. Finally, attention has been drawn to the special clinical circumstances when the aortic valve stenosis provides a strinkingly different clinical picture. We cannot find a better way to end this review than by quoting a warning note given by Thomas Lewis in 1920: "It is the faint cry of an anguished and fast failing muscle, which, when it comes, all should strain to hear, for it is not long repeated. A few months, a few years at most, and the end comes."     

Left ventricular function by echocardiography in children with fixed aortic stenosis

Left ventricular function was evaluated echocardiographically within 24 hours of cardiac catheterization in 45 children with fixed aortic stenosis. Left ventricular systolic time intervals, mean velocity of circumferential fiber shortening, left ventricular end-diastolic volume and shortening fraction were evaluated. Some patients with severe aortic stenosis had a shortened preejection period, prolonged ejection time and increased mean velocity of circumferential fiber shortening in comparison with normal subjects, but these findings were not consistent within the group with severe aortic stenosis or apparent in patients with mild or moderate aortic stenosis. Left ventricular end-diastolic volume was smaller in children with severe obstruction than in normal children. Evaluation of the shortening fraction provided good separation of patients with significant obstruction from those with mild disease. Further, there was a linear correlation between the shortening fraction and the left ventricular-aortic gradient demonstrated at cardiac catheterization. This correlation was also of use in evaluating the response of patients to surgical relief of obstruction. Pre- and postoperative echocardiographic evaluation of the shortening fraction appears to provide a readily available noninvasive method of assessing the course and severity of obstruction in fixed aortic stenosis in children.     

Surgical treatment of thoracoabdominal aortic aneurysm

Graft replacement for thoracoabdomital aortic aneurysm was performed in 8 patients between 1982 and 1989. Five patients in our series underwent reattachment of the branch vessels to openings made in the graft. Partial bypass or external shunt was used in 6 patients during aortic cross-clamping. Selective blood perfusion to the branch vessels was performed in 5 patients intraoperatively. Neither hospital death nor postoperative paraplegia was found in our series. One patient without selective perfusion of abdominal branch vessels during reattachment to the graft developed an acute jaundice postoperatively, but 4 of the 5 patients receiving selective perfusion of branch vessels developed no complication related to abdominal ischemia. These results suggest that intraoperative blood perfusion of aortic branch might be useful for prevention of anoxic complication of the abdominal organs after surgery of thoracoabdominal aortic aneurysm.     

Surgical treatment of chronic pancreatitis. Indications and early results

A report is given on 28 resections on patients with chronic pancreatitis with a high frequency of severe destructive lesions in the head of the pancreas. Pseudocysts, single, multiple or extrapancreatic were present in almost 50%. Strictures of the Ductus Wirsungianus were found in 24 cases and 50% had multiple pancreatic stones. In these cases 12 partial and 9 total duodenopancreatectomies and 7 distal resections were performed. All patients with distal resections survived. Only one patient died in the group with partial and total duodenopancreatectomy, which accounts for a postoperative letality of 4.8%. Postoperative there were 4 reoccurrences of pancreatitis, due to further alcohol abusus, 1 patient died from alcohol intoxication. Patients with total seemed to do better than with partial pancreatectomy at a survey 18 months after operation.     

Surgical treatment of aortic dissection with ringed intraluminal graft--late results and fate of the false lumen

Between April 1981 and March 1992, sixty-nine patients underwent surgery for aortic dissection in our hospital and affiliated hospitals. Of them, 48 patients who survived the operation were followed up 2 months to 11.8 years (mean: 4.6 years). We compared the fate of the false lumen and late results in the 31 patients who underwent ringed intraluminal graft (RIG group) and the 17 patients who underwent conventional graft replacement (GR group). To clarify the status and severity of the dissected aorta, we devised a scoring system (dissection score: DS) to evaluate the long-term changes in the diseased aorta after operation. Of 12 patients (25%) who died of late dissection-related complications, 10 were in the RIG group and 2 in the GR group. The actuarial survival at 5 years was 66 +/- 9% in the RIG group and 88 +/- 8% in the GR group. Late dissection-related events (dissection-related death, redissection, reoperation) were observed in 17 patients, 14 of whom belonged to the RIG group and 3 to the GR group. The dissection-related event-free survival at 5 years was 51 +/- 10% in the RIG group and 88 +/- 8% in the GR group. In 36 (75%) of the 48 patients, including 20 (65%) of the 31 patients in the RIG group and 16 (94%) of the 17 in the GR group (p < 0.05), it was possible to resect the primary intimal tear. The actuarial survival at 5 years was 76 +/- 8% for those 36 cases in which resection of the primary intimal tear was possible and 64 +/- 15% for those in which resection was not possible. The dissection-related event-free survival at 5 years was 72 +/- 8% in the former group and 33 +/- 15% in the latter. The dissection score (DS) decreased from a pre-operative value of 21.5 +/- 8.4 to 12.0 +/- 9.3 postoperatively (p < 0.001). A significant decrease in DS was observed both in the RIG group and the GR group, in the former from 22.1 +/- 7.2 to 13.1 +/- 9.3, and in the latter from 19.9 +/- 11.5 to 9.1 +/- 9.2 (p < 0.01). The DS for those whose primary intimal tear could be resected was significantly decreased postoperatively (pre-operative 20.6 +/- 8.9, post-operative 9.3 +/- 8.4; p < 0.001); but for those whose primary intimal tear could not be resected, there was no difference between the pre- and post-operative DS (pre-operative 23.4 +/- 7.3, post-operative 18.2 +/- 8.7). The percent reduction in DS (%R) was significantly greater in the former group than in the latter one (61% vs. 29%; p < 0.02). DS significantly decreased for those with no late dissection-related events (from 22.4 +/- 9.2 to 10.9 +/- 8.8; p < 0.001) while for those who presented with such events there was no difference between before and after the operation (pre-operative 19.0 +/- 5.7, post-operative 14.9 +/- 10.6). The %R in those with dissection-related events tended to be lower than that in those without such events (33% vs. 58%). Resection of primary intimal tear seemed to be important not only to improve operative results in patients with aortic dissection but also to improve late results. Depending on the site and size of the intimal tear, the ringed intraluminal graft insertion technique often makes intimal tear resection impossible. Indications for this surgical technique are thus quite limited.     

Surgical treatment of thoracoabdominal aortic aneurysms]

Between February, 1981, and April, 1989, 20 patients underwent surgical treatment of thoracoabdominal aortic aneurysms. Most of the patients were operated under temporary external bypass. For Group I and III aneurysms without reconstruction of renal arteries, a modified Crawford's graft inclusion technique was employed to shorten abdominal visceral ischemic time. This modification consists of (1) using adjuncts to perfuse the distal aorta during aortic clamp, (2) starting the first anasistomosis from the distal end of the graft, and (3) shifting the distal aortic clamp on the graft after completing the anastomosis in order to restore abdominal visceral circulation as soon as possible. For Group III and IV aneurysms with reconstruction of renal arteries as well as celiac and superior mesenteric arteries, a modified DeBakey's procedure was employed. This modification consists of (1) using the spiral opening method, (2) doing end-to-end anastomosis at the proximal aortic site, and (3) maintaining the circulation of abdominal organs and spinal cord by using adjuncts during the anastomosis of the proximal end. There were one operative death and two hospital deaths. Paraplegia developed in two cases, one of which was a ruptured case. Renal dysfunction was not found in any case. The survivors were followed from 5 to 103 months, and there was no late death. The results suggest that our modified procedures for thoracoabdominal aortic aneurysms are useful and reliable ones.     

Immediate results of surgical treatment of gastric stump cancer

An immediate result of surgical treatment of gastric stump cancer in 68 patients is presented. The resectability rate was 23.5%. Lethality after radical operations was 18.7%, after symptomatic operations and exploratory laparotomy-9.6%. Gastric resection was performed, using the method developed in the clinic with wrapping the anastomosis, or gastric stump by a loop of the afferent intestine. In development of gastric stump cancer, local spreading of a tumor is not a reason to discard performance of radical operation.     

Immediate and follow up results of Inoue balloon mitral valvotomy in juvenile rheumatic mitral stenosis

1. One of the main obstacles to the reliable detection of human papillomavirus in cervical intraepithelial neoplasia is that a minute quantity of infected tissue is often all that is available. 2. In this work, proteinase K-phenol-chloroform-treated sections of frozen cervical biopsies were split in two. Half of the material was precipitated by ethanol in the presence of glycogen, and in the remaining half glycogen was absent. 3. On average a 15-fold increase in total DNA yield was obtained with glycogen. As a result, in 63 cases analysed by PCR for human papillomavirus type 16, we were able to detect nearly 20% more positive samples when glycogen was used. 4. The implications of the improved accuracy of diagnosis are that women requiring a closer follow-up can be identified, and conversely rescreening intervals can be extended for those testing negative for oncogenic human papillomavirus types with more confidence than in the past.     

COPD: results of long-term treatment

During the last decades the life expectation of patients with COPD increased continually. Today the life expectation of these patients reach that of the normal general population. Most important for this effect is the improvement of the therapeutical possibilities. Following the experience over nearly 3 decades the treatment regime is described. Glucocorticosteroids are at first line as much as necessary and as less as possible, starting with the inhalative forms adjusted at the clinical course stabilizes the situation. Bronchodilatation around the clock and as good as possible is a further important step. These effects have to be functionally-analytically controlled. It is not enough to follow only the clinical impression. beta 2-sympathicomimetics are the overall dilators with strongest effects and increase even the clearing mechanisms of the bronchopulmonary system. Short- as long-acting medicaments are available, but the duration of the action at each of the different forms is sometimes clearly shorter as the mean values describe. Anticholinergics and theophyllines have weaker bronchodilatation power, but they show additive effects, which could be used at special situations. Combinations as Berodual (beta 2-sympathicomimetics and anticholinergics) can decrease the dosage of the beta 2-sympathicomimetics and therefore increase the therapeutic range. At the situation of exacerbation antibiotics can be very helpful, but sometimes an increase of the dosage of glucocorticosteroids is necessary and sometimes the increase of this dosage alone can control the situation. It is very important to detect the deterioration which can end with a severe life threatening exacerbation as early as possible and to control the situation already at this stage. It is shown at examples of 56 patients functionally-analytically controlled over 5 years that the function values do not deteriorate during the constant treatment regime any more. Even in spite of the constant treatment with beta 2-sympathicomimetics no signs of any kind of down regulation or of tachyphylaxis of the bronchodilatation power could be detected. A bronchodilatation test after 4.2 years of constant bronchodilatation treatment together with beta 2-sympathicomimetics showed at 25 patients the same effect as normally at acute tests without pre-treatment seen. Because after the start of this treatment regime to-day a further deterioration does not take place, it is important to detect signs of the disease as early as possible. This can be achieved as the basis of lung function values measure over a long time. The best basis for this is the individual norm known from measurement at healthy days.     

Echocardiographic hemodynamic and morphometric predictors of survival after two-ventricle repair in infants with critical aortic stenosis

OBJECTIVES: The purpose of this study was to identify echocardiographic hemodynamic and morphometric factors that would predict which infants with critical aortic stenosis could undergo relief of left ventricular outflow obstruction as opposed to the Norwood procedure. BACKGROUND: Echocardiographic predictors of survival in infants with critical aortic stenosis after two-ventricle repair have been mainly limited to morphometric factors, which have limitations. Echocardiographic hemodynamic predictors of survival in these patients have not previously been studied. METHODS: Doppler color flow mapping and pulsed Doppler techniques were used to obtain hemodynamic measurements of flow in the ascending, transverse and descending aorta, the ductus arteriosus, and across the aortic and mitral valves in infants with critical aortic stenosis. Morphometric measurements of the left heart structures were obtained, and comparisons were made between survivors and nonsurvivors for the hemodynamic and morphometric factors. RESULTS: Twenty-eight infants (mean age 1 +/- .6 days, mean weight 3.6 +/- .6 kg) with critical aortic stenosis were evaluated. Nineteen had a two-ventricle repair initially attempted, and nine had a Norwood operation. Among the patients with a two-ventricle repair, the hemodynamic factors associated with survival after two-ventricle repair included predominant or total antegrade flow in the ascending (p < 0.01) and transverse aorta (p < 0.05). Aortic valve gradient, mitral valve inflow and direction of flow in the ductus arteriosus and descending aorta were unrelated to outcome. The morphometric factors associated with survival after two-ventricle repair included the indexed aortic annulus (p < 0.0002), aortic root (p < 0.003), ascending aorta (p < 0.008) and left ventricular long-axis length (p < 0.01). Left ventricular volume, mass, ejection fraction and mitral valve area were not related to outcome after two-ventricle repair. CONCLUSIONS: In infants with critical aortic stenosis, predominant or total antegrade flow in the ascending and transverse aorta was associated with survival after two-ventricle repair. Determination of a one- versus two-ventricle repair remains a complex issue in infants with critical aortic stenosis. In addition to established morphometric predictors, hemodynamic information on the direction of flow in the aorta may help to define candidates for the Norwood operation.     

Surgical treatment for acute aortic arch dissection

The authors describe 2 patients with Takayasu's arteritis in whom lupus anticoagulant was positive and the titer of anticardiolipin antibody was elevated. One patient developed diffusely stenotic and occlusive changes in the multiple larger arteries. Histology of the small-sized arteries in another patient showed occlusive vasculitis without thrombosis, in addition to the findings in large-sized arteries compatible with Takayasu's disease. These findings are uncommon in Takayasu's arteritis. These findings suggest that antiphospholipid antibodies may have contributed to the pathogenesis of the extensive vasculopathy and may have triggered vasculitis in these patients.     

Surgical implication of aortic dissection on long-term outcome in Marfan patients

We herein review our 17-year surgical experience for the treatment of ascending aortic aneurysm in patients with Marfan syndrome to clarify the risks of increased mortality and reoperation. The subjects consisted of 15 patients who had all undergone surgery for the aortic root and ascending aorta at Niigata University Hospital between July 1978 and January 1995. Aortic valve replacement and ascending aortic wrapping were performed in 5 patients, Bentall or Cabrol operation in 6, and combined aortic arch reconstruction and Cabrol operation in 2, as the initial surgery. Patients who had an aortic dissection (Stanford type A) at initial surgery were assigned to group I (n = 7), while those with an aortic root aneurysm were assigned to group II (n = 8). In group I, 3 patients required a second operation for the remaining aortic arch aneurysm, and 1 died due to a late rupture of the distal aneurysm. In group II, no patient needed a reoperation; however, 1 died due to an intracranial hemorrhage and another due to composite valve graft failure and distal dissection. The results thus indicate that aortic dissection seems to affect long-term outcome, and therefore the combined repair of the aortic root and transverse arch is recommended in Marfan patients with aortic dissection involving the transverse aortic arch.