Biomedical subjects

B A Reitz

Publications and source records attributed to B A Reitz.

At least 55 records · Page 3Linked to original sources

PubMed1996

Pediatric heart-lung transplantation: intermediate-term results.

BACKGROUND: Adult heart-lung transplantation was initiated at Stanford in 1981 and the first pediatric heart-lung transplantation was done in 1986. Intermediate-term results of pediatric heart-lung transplantation at Stanford University are presented. METHODS: A retrospective review of the records of all pediatric heart-lung transplantations done since 1986 was conducted. RESULTS: Nineteen heart-lung transplantations were done in 17 patients. Ages ranged from 2 months to 18 years with a median age of 10 years. At the time of transplantation 5 patients were infants, 7 children, and 7 adolescents. The mean follow-up was 29 +/- 6.2 months (range 1 to 77, median 16) and follow-up was 100% complete. Diagnoses were congenital heart disease in 13, primary pulmonary hypertension in 2, and cystic fibrosis, cystic lymphangiectasia, viral pneumonia, and obliterative bronchiolitis in 1 each. Median wait on the heart-lung transplantation list was 91 days (range 2 to 707). All patients had New York Heart Association class III to IV symptoms, two were receiving ventilator support, and six were receiving oxygen. Fifteen of 19 transplant recipients were discharged from the hospital. The 30-day operative mortality rate was 5.2% (1 of 19). The actuarial survival at 1, 3, and 5 years for all patients was 67%, 51%, and 41%, respectively, and for hospital survivors was 82%, 62%, and 51%. The cause of death was obliterative bronchiolitis in 4, multisystem organ failure in 3, and graft coronary artery disease and chronic airway disease in 1 each. Three patients required retransplantation, 2 because of obliterative bronchiolitis and 1 because of viral pneumonia. Two patients underwent repeat heart-lung transplantation and 1 patient underwent single lung transplantation. Rejection was diagnosed in 73% of recipients, and obliterative bronchiolitis has developed in 32% of recipients. CONCLUSIONS: Survival in pediatric heart-lung transplantation approximates that in the adult procedure at 1, 3, and 5 years. Long-term survival has been achieved but the primary factors limiting further improved survival remain infection and obliterative bronchiolitis.

Adolescent↗

PubMed1996

Risk factors for the development of obliterative bronchiolitis after lung transplantation.

BACKGROUND: Acute rejection has emerged as an important risk factor for obliterative bronchiolitis after lung transplantation. We performed a multivariate analysis to assess the impact of additional variables. METHODS: Seventy-four recipients (48 heart-lung, 18 single-lung, and 8 bilateral-lung recipients) who survived longer than 90 days and underwent transplantation more than 15 months before data analysis were included in this study. Several variables were entered into a Cox regression analysis to determine their association with the development of bronchiolitis obliterans syndrome. RESULTS: Bronchiolitis obliterans syndrome developed in 48 (65%) of 74 patients. Significant correlations were detected for acute rejection score, defined as the sum of pathologic grades of each separate acute rejection episode (p = 0.0004, likelihood ratio test value = 12.4) and for lymphocytic bronchiolitis (p = 0.03). In a bivariate model, episodes of organizing pneumonia and bacterial or fungal pneumonia significantly increased the likelihood ratio test value of the acute rejection score. The addition of the cytomegalovirus infection score, reflecting the frequency and severity of infection, to the combination of the acute rejection score and episodes of bacterial or fungal pneumonia resulted in a further significant increase in the likelihood ratio test value. Significant risk factors for moderate to severe stages of airflow limitation were at least one episode of acute rejection of grade > or = 2, younger recipient age, and any acute rejection episode 180 days or longer after transplantation. CONCLUSIONS: Increasing frequency and severity of acute rejection episodes are strongly associated with the development of bronchiolitis obliterans syndrome. Lymphocytic bronchiolitis appeared to be significant by univariate analysis, but in a two-risk factor model, it did not augment the influence of acute rejection. Organizing pneumonia, bacterial or fungal pneumonia, and increasing severity and frequency of cytomegalovirus infections potentiate the effect of acute rejection. Late episodes of acute rejection and younger recipient age increase the risk for development of advanced disease.

Adolescent↗

PubMed1995

The utility of annual surveillance bronchoscopy in heart-lung transplant recipients.

Bronchoscopy with transbronchial biopsy (TBBx) and bronchoalveolar lavage (BAL) has an appreciable yield in detecting asymptomatic abnormalities in heart-lung transplant recipients (HLTR) during the early postoperative period. The utility of annual surveillance procedures has not been critically evaluated. We reviewed all annual bronchoscopies performed on 29 HLTR to determine the frequency of asymptomatic abnormalities. Surveillance bronchoscopies (SB) were performed on asymptomatic subjects with unchanged lung function compared with baseline. Surveillance/clinical bronchoscopies (SCB) were those performed in patients with stable decrements in lung function. Nineteen patients underwent 48 SB and 8 had 18 SCB. Five of 15 (33%) SB performed at one year yielded an abnormal TBBx (1 grade 2 acute rejection [AR], 1 grade 1 AR, 1 grade 1 AR with obliterative bronchiolitis [OB] and 2 Pneumocystis carinii pneumonia). At 2 or more years, TBBx was abnormal in 2 of 33 (6%, p = 0.024 compared with first year TBBx) (1 grade 1 AR, 1 lymphocytic bronchiolitis). Pathogens were identified in BAL in 19 (40%) SB. Fourteen (78%) SCB were abnormal. Nine (50%) revealed an abnormal TBBx (all OB), but only 2 (11%) of these altered patient management. Seven (39%) demonstrated pathogens in BAL. We conclude that in HLTR (1) surveillance TBBx rarely yields positive findings 2 or more years posttransplant, (2) surveillance TBBx seldom alters management in patients with stable decrements in lung function, and (3) BAL is useful to screen for potential pathogens.

Adolescent↗

PubMed1995

Surgical management of aortic dissection during a 30-year period.

BACKGROUND: Certain recent studies have demonstrated improved surgical outcome in patients with aortic dissection. We analyzed the surgical survival rates of patients with acute aortic dissections and the late prognosis of those with aortic dissection during a 30-year period. METHODS AND RESULTS: Between 1963 and 1992, 360 patients (256 men and 104 women; mean +/- 1 SD age, 57 +/- 14 years) underwent surgery for aortic dissection: 174 patients had an acute type A (AcA), 46 an acute type B (AcB), 106 a chronic type A (ChA), and 34 a chronic type B (ChB) aortic dissection. The overall operative mortality rate was 24 +/- 8% (26 +/- 3% for AcA, 39 +/- 8% for AcB, 17 +/- 4% for ChA, and 15 +/- 6% for ChB, [+/- 70% confidence limit]). The operative mortality rates for patients with acute aortic dissection (AcA or AcB) were assessed for five time "windows": 1963 to 1972 (42 +/- 8%), 1973 to 1977 (37 +/- 8%), 1978 to 1982 (15 +/- 6%), 1983 to 1987 (27 +/- 6%), and 1988 to 1992 (26 +/- 6%). Logistic regression analysis suggested that the low operative mortality rate during the 1978-to-1982 interval occurred by chance. Multivariate analysis showed earlier operative year, hypertension, cardiac tamponade, renal dysfunction, and older age were independent determinants of operative death. Actuarial survival rates (including early deaths) after 5, 10, and 15 years for AcA patients were 55%, 37%, and 24%; for AcB, 48%, 29%, and 11%; for ChA, 65%, 45%, and 27%; and for ChB, 59%, 45%, and 27%. Multivariate analysis revealed that older age and previous operation were significant predictors for late death. Freedom from reoperation for all patients was 84%, 67%, and 57% at 5, 10, and 15 years, respectively. CONCLUSIONS: Although the operative mortality rate decreased over time for patients with aortic dissection, the risk for those with acute aortic dissection during the last 10 years (1983 to 1992) is probably more realistic than that observed in the preceding 5-year interval (1978 to 1982). The operative mortality rates for patients with chronic aortic dissection have remained relatively static. Earlier diagnosis of acute aortic dissection before development of cardiac tamponade and renal impairment is critical to improve the operative salvage rate. Long-term outcome still is not optimal, which emphasizes the need for better serial postoperative aortic imaging surveillance and medical follow-up and blood pressure control.

Adult↗

PubMed1995

In vivo studies of the maintenance of peripheral transplant tolerance after cyclosporine. Radiosensitive antigen-specific suppressor cells mediate lasting graft protection against primed effector cells.

Cellular mechanisms responsible for maintenance of peripheral transplant tolerance in a rodent model were evaluated. Donor-specific tolerance was established in ACI rats given a vascularized heterotopic cardiac allograft followed by a 10-day course of cyclosporine. Tolerance was associated with a reduction in donor-specific cytotoxic T lymphocyte precursors and the presence within the spleen of cells capable of transferring suppression in adoptive transfer assays. Experiments using thymectomized animals revealed that the establishment and maintenance of tolerance occurred peripherally, independently of the thymus. Adoptive transfer experiments demonstrated that ongoing graft tolerance was mediated by suppressor cells that were antigen-restricted, radiosensitive, and capable of preventing allograft rejection by naive as well as sensitized cells in vivo. Studies designed to disrupt tolerance demonstrated a remarkable durability of graft protection once established, and give insight into the identity and mechanism of action of suppressor cells generated in this model.

Animals↗

PubMed1995

Repair of coarctation of the aorta in neonates and infants: a thirty-year experience.

Between January 1962 and December 1991, 179 children less than 1 year of age underwent repair of coarctation of the aorta. Group I (1962 to 1971) consisted of 19 patients, group II (1972 to 1981) of 57 patients, group III (1982 to 1991) of 103 patients. Neonates (< 30 days old) made up 60% of group I, 57% of group II, and 70% of group III. The proportion of infants with associated complex cardiac abnormalities was 7% in group I, 25% in group II, and 39% in group III. Techniques of repair included resection with end-to-end anastomosis (n = 65), subclavian flap repair (n = 85), patch aortoplasty (n = 18), and other procedures (n = 11). The early mortality (< 30 days) was lowest in group III (group I, 21%; group II, 21%; and group III, 7%; p < 0.05), but the late mortality was similar in all groups (group I, 11%; group II, 13%; and group III, 15%). The overall actuarial survival was 57.7% +/- 0.15% at 27.1 years in group I, 65.7% +/- 0.07% at 19.7 years in group II, and 77.5% +/- 0.04% at 9.3 years in group III (p = not significant). Twenty-five restenoses requiring intervention occurred in 23 patients, for an overall restenosis rate of 16.4%. The incidence of restenosis was 23% for the patients who underwent end-to-end anastomosis, 11% for those who underwent subclavian flap repair (p < 0.1), and 27% for those who underwent patch aortoplasty (p < 0.01). Balloon angioplasty was successful in relieving 11 of the 12 restenoses in groups II and III.(ABSTRACT TRUNCATED AT 250 WORDS)

Abnormalities, Multiple↗

PubMed1995

Arterial switch and resection of hepatic hemangioendothelioma.

We report on the management of a neonate undergoing arterial switch for transposition of the great arteries and concomitant resection of a hepatic infantile hemangioendothelioma. A preoperative aortogram demonstrated the arterial supply of the hepatic hemangioendothelioma. Pulmonary artery hypertension and myocardial ischemia were noted after separation from cardiopulmonary bypass. Resection of the hepatic malformation produced an immediate reduction in pulmonary hypertension and resolution of the myocardial ischemia. The patient had an uneventful postoperative recovery.

Female↗

PubMed1995

Durability of the Hancock MO bioprosthesis compared with standard aortic valve bioprostheses.

To compare the durability of the Hancock modified orifice (Hancock MO, model 250 [H-MO]) valve with two other commonly used standard aortic valve bioprostheses, a cohort of 1,602 patients undergoing aortic valve replacement using porcine valves between 1971 and 1990 (excluding simultaneous mitral valve replacement) was analyzed retrospectively using Cox model multivariate techniques. Five hundred sixty-one patients received a composite H-MO valve, 652 received a standard Hancock model 242 (H) valve, and 389 received a Carpentier-Edwards model 2625 (C-E) valve. Mean age was 60 +/- 15 years (+/- 1 standard deviation) (71% male). Follow-up (10,247 patient-years) extended to 15 years and was 97% complete. The main focus of this study was bioprosthetic durability, using The American Association for Thoracic Surgery/The Society of Thoracic Surgeons guidelines to define structural valve deterioration (SVD). Multivariate analysis revealed that (younger) age (p < 10(-5), liver disease (p = 0.02), and 1981 to 1985 operative period (p = 0.012) were the only significant, independent predictors of SVD. In concordance with previous reports, the SVD freedom estimate was greater than 90% at 15 years for patients older than 70 years of age. Hepatic dysfunction had an adverse effect on SVD (estimated freedom from event at 10 years was 34 +/- 17% [standard error of mean] versus 78 +/- 2% for those without liver disease), but this affected only 3% of patients. Interestingly, one operative period (1981 to 1985) was associated with a slightly higher risk of SVD compared to the three other 5-year time windows. Valve type did not emerge as a significant risk factor for SVD.(ABSTRACT TRUNCATED AT 250 WORDS)

Actuarial Analysis↗

PubMed1995

Cardiac transplantation for hypertrophic cardiomyopathy associated with Sengers syndrome.

Sengers' syndrome is a rare condition consisting of congenital cataracts, mitochondrial myopathy, and hypertrophic cardiomyopathy. The syndrome is transmitted in an autosomal recessive pattern. Progressive cardiac failure is the cause of death in most patients. This report describes cardiac transplantation for the treatment of the cardiomyopathy associated with Sengers' syndrome.

Cardiomyopathy, Hypertrophic↗

PubMed1995

Obliterative bronchiolitis after lung and heart-lung transplantation.

Obliterative bronchiolitis (OB) has emerged as the main cause of morbidity and mortality in the long-term follow-up after lung and heart-lung transplantation. The pathogenesis of OB is multifactorial, with acute rejection and cytomegalovirus infection being the main risk factors for the development of OB. The final common pathway of all inciting events seems to be an alloimmune injury, with subsequent release of immunologic mediators and production of growth factors leading to luminal obliteration and fibrous scarring of the small airways. Analyzing the 14 years of experience in 163 patients at Stanford University, we found a current incidence of bronchiolitis obliterans syndrome or histologically proven OB within the first 3 years after lung and heart-lung transplantation of 36.3%, with an overall prevalence of 58.1% after heart-lung and 51.4% after lung transplantation. Both pulmonary function indices (forced expiratory flow between 25% and 75% of forced vital capacity and forced expiratory volume in 1 second) and transbronchial biopsies have proven helpful in diagnosing bronchiolitis obliterans syndrome or OB at an early stage. Early diagnosis of OB and improved management have achieved survival rates in patients with OB after 1, 3, 5, and 10 years of 83%, 66%, 46%, and 22%, compared with 86%, 83%, 67%, and 67% in patients without OB. Recently, different experimental models have been developed to investigate the cellular and molecular events leading to OB and to evaluate new treatment strategies for this complication, which currently limits the long-term success of heart-lung and lung transplantation.

Bronchiolitis Obliterans↗

PubMed1995

Endogenous and exogenous pituitary-specific promoters are differentially controlled.

We have engineered GH3 cells with reporter genes under control of the growth hormone and prolactin promoters and measured protein production. The results indicate very low level production of reporter proteins from the cells regardless of the promoter used to drive expression. This was surprising in light of the observation that the cells still produced high levels of endogenous growth hormone and prolactin. Chinese hamster ovary (CHO) cells were engineered to express the Pit-1 transactivator. Transfection of reporter genes under control of the prolactin promoter demonstrated a clear enhancement of expression levels compared to the same promoter in parental CHO cells. Pit-1 expression is not sufficient, however, for high level, stable expression from the growth hormone promoter. These results indicate that the growth hormone and prolactin promoters are not sufficient for high level, stable expression even in normally permissive cells and suggest that Pit-1 alone is not sufficient for strong promoter activity from the integrated plasmids.

Animals↗

PubMed1995

Aortic root replacement. Risk factor analysis of a seventeen-year experience with 270 patients.

Between September 1976 and September 1993, 270 patients underwent aortic root replacement at our institution. Two hundred fifty-two patients underwent a Bentall composite graft repair and 18 patients received a cryopreserved homograft aortic root. One hundred eighty-seven patients had a Marfan aneurysm of the ascending aorta (41 with dissection) and 53 patients had an aneurysm resulting from nonspecific medial degeneration (17 with dissection). These 240 patients were considered to have annuloaortic ectasia. Thirty patients were operated on for miscellaneous lesions of the aortic root. Thirty-day mortality for the overall series of 270 patients was 4.8% (13/270). There was no 30-day mortality among 182 patients undergoing elective root replacement for annuloaortic ectasia without dissection. Thirty-six of the 270 patients having root replacement also had mitral valve operations. There was no hospital mortality for aortic root replacement in these 36 patients, but there were seven late deaths. Twenty-two patients received a cryopreserved homograft aortic root; 18 of these were primary root replacements and four were repeat root replacements for late endocarditis. One early death and two late deaths occurred in this group. Actuarial survival for the overall group of 270 patients was 73% at 10 years. In a multivariate analysis, only poor New Year Heart Association class (III and IV), non-Marfan status, preoperative dissection, and male gender emerged as significant predictors of early or late death. Endocarditis was the most common late complication (14 of 256 hospital survivors) and was optimally treated by root replacement with a cryopreserved aortic homograft. Late problems with the part of the aorta not operated on occur with moderate frequency; careful follow-up of the distal aorta is critical to long-term survival.

Actuarial Analysis↗

PubMed1994

Tissue-specific differences in the establishment of tolerance. Tolerogenic effects of lung allografts in rats.

With the increasing frequency of transplantation of two or more organs into a single recipient, it has become evident that different organs are rejected with different kinetics. In this study the kinetics of skin, lung, and heart allograft rejection were compared in a rodent model. To study the influence of different allografts on the recipient's immune system, simultaneous or sequential skin, lung, or heart transplants were performed in various combinations, using DA rats as recipients for PVG allografts. Recipients receiving primary allografts were treated postoperatively with ten doses of cyclosporine (CsA) or preoperatively with 4 doses of rabbit antirat thymocyte globulin (ATG). Subsequent transplants were performed a minimum of 40 days later without additional immunosuppression. All primary skin allografts and 60% of primary lung allografts were rejected, while 100% of the heart allografts were accepted indefinitely. Recipients of primary skin allografts rejected subsequent skin, lung, or heart allografts with accelerated kinetics. Recipients of primary heart allografts accepted subsequent skin, lung, and heart allografts indefinitely without further immunosuppression. Surprisingly, animals that had rejected a primary lung allograft accepted subsequent skin or heart allografts indefinitely. Simultaneously transplanted skin and lung allografts were concordantly rejected. However, these animals accepted a subsequent heart allograft indefinitely, suggesting a strong tolerizing effect of lung allografts. Our results indicate that tissue-specific differences are critical, not only in determining acceptance or rejection of a primary allograft but also in determining the fate of subsequent allografts.

Animals↗

PubMed1994

High-level expression of biologically active, soluble forms of ICAM-1 in a novel mammalian-cell expression system.

LFA-1/ICAM-1 interaction is important in facilitating a number of cellular events including antigen-specific T-cell activation and leukocyte transendothelial migration. We are interested in defining residues and contact sites that mediate ICAM-1 interaction with the integrin receptor, LFA-1. To provide sufficient material to facilitate study of the interaction of this ligand-receptor pair, we have developed a new high-level mammalian-cell expression system based on the use of the herpes simplex virus (HSV) VP16 transactivator and the HSV IE175 promoter to direct expression of foreign genes in BHK cells. In this system, the gene of interest is expressed as a fusion protein with a carboxyl terminal decapeptide tail to aid in identification, quantitation, and affinity purification of recombinant protein. This system allowed rapid generation of cell lines producing high levels of levels of soluble proteins corresponding to the full-length extracellular (sICAM453) and the amino terminal two immunoglobulin domains (sICAM185) of ICAM-1. Both sICAM453 and sICAM185 were biologically active and were purified in a single step from conditioned media by antibody affinity chromatography.

Amino Acid Sequence↗

PubMed1994

Inhibition of neutrophil adhesion during cardiopulmonary bypass.

Blood contact with synthetic surfaces during cardiopulmonary bypass (CPB) causes a diffuse inflammatory reaction that includes neutrophil activation. The purpose of this study was to determine if inhibition of neutrophil adhesion with a new antiinflammatory agent NPC 15669 (N-(9H-(2,7-dimethylfluorenyl-9-methoxy)-carbonyl)-L-leucine) could reduce pulmonary injury in a porcine model of CPB. NPC 15669 blocks adherence of activated neutrophils by inhibiting upregulation of the Mac-1 (CD11b/CD18) adhesion molecule. Sixteen piglets underwent 2 hours of hypothermic CPB followed by 2 hours of observation; 8 received NPC 15669 (10 mg/kg intravenous bolus followed by 6 mg.kg-1.h-1 intravenous infusion) and 8 received equal volumes of vehicle. After 90 minutes of CPB, expression of neutrophil adhesion molecule subunit CD18 increased 118% in control piglets but only 36% in piglets treated with NPC 15669 (p < 0.01). Although neutropenia developed in all animals during CPB, lung tissue myeloperoxidase content was significantly lower in treated than in control animals 2 hours after CPB (94.9 +/- 10.4 versus 46.9 +/- 5.5 mumol.10 mg-1.min-1; p < 0.002). Free radical-mediated lipid peroxidation (quantitated by spectrophotometric assay of plasma conjugated dienes) was significantly reduced by treatment with NPC 15669 during and after CPB. Pulmonary function was better in NPC 15669-treated animals: 2 hours after CPB, pulmonary vascular resistance increased 477% in control piglets but only 140% in piglets receiving NPC 15669 (p < 0.03); arterial oxygen tension was significantly greater in piglets receiving NPC 15669 (428 +/- 33 mm Hg) than in controls (141 +/- 46; p < 0.0001).(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

PubMed1994

Developments in cardiac transplantation.

Cardiac transplantation has matured as a therapeutic intervention, allowing definitive treatment of critically ill children and adults with end-stage heart disease. The ongoing critical shortage of donor organs continues to deny hundreds of individuals access to this intervention. Accordingly, many of the most meaningful recent advances made in the field of cardiac transplantation involve means of expanding our donor pool. While current immunosuppressive regimens have been considerably successful in the management of acute cellular rejection, management of the problems of acute and chronic vascular rejection remains disappointing. Advances in this arena remain particularly urgent for physicians and surgeons involved in the care of heart transplant recipients.

Adult↗

PubMed1994

Current diagnosis and prescription for the Marfan syndrome: aortic root and valve replacement.

Since 1976, the procedure of choice for patients with Marfan aneurysm of the ascending aorta at this institution has been the Bentall procedure or its modification. One hundred seventy-four Marfan patients have had ascending aortic aneurysm repair with a prosthetic composite graft. An additional four children and two adults have had aortic root replacement with a cryopreserved homograft. Thirty-seven (21%) patients came to operation with an acute or chronic dissection involving the ascending aorta. Thirty-one (17%) patients having aortic root replacement also had mitral valve repair or replacement. The first 86 patients in the series had a hemostatic Bentall wrap constructed; however, in the last 88 patients, the ascending aorta has been transected completely and the aortic wall tacked loosely over the composite graft. It is our preference to construct a direct end-to-side anastomosis of each coronary ostium to the side of the composite graft. In the past, we used the Cabrol interposed graft for low-lying coronary ostia, but currently favor the "button technique" for this procedure. One hundred fifty-six of 180 patients underwent elective root replacement with no hospital mortality. Twenty-four patients underwent urgent or emergency surgery; 17 of these patients with acute dissection or rupture underwent emergency surgery. Two of these patients with rupture died intraoperatively. Therefore, overall hospital mortality among the 180 patients was 1.1%. There have been 14 late deaths among 178 patients discharged from the hospital (7.9%). Five of these late deaths occurred among our first 11 patients operated on between 1976 and 1979. Since 1980, there have been nine late deaths among 167 patients (5.4%).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMed1994

Surgical management of aortic dissection in patients with the Marfan syndrome.

BACKGROUND: Aortic dissection is one of the most lethal potential complications in patients with the Marfan syndrome. METHODS AND RESULTS: Among 360 patients undergoing operative treatment of aortic dissection between 1963 and 1992, 40 had the Marfan syndrome. There were 24 men and 16 women with a mean age of 35 +/- 9 years (+/- 1 SD; range, 15 to 54 years). These patients included 16 with acute type A, 2 with acute type B, 18 with chronic type A, and 4 with chronic type B aortic dissections. The aortic arch was involved in 29 cases. Preoperative complications included acute aortic valvular insufficiency in 13 patients, rupture into the pericardial space in 3, and loss of peripheral pulses in 9. The site of primary intimal tear was the ascending aorta in 25 patients, the aortic arch in 2, the descending aorta in 7, and not identified in 6. Operations included ascending aortic and aortic valvular replacement (with or without coronary artery reimplantation) in 22 patients, ascending aortic replacement alone in 5, and descending thoracic aortic replacement in 9. Four operative deaths (10 +/- 5% [+/- 70% confidence limits]) occurred in 3 acute patient-years and 1 chronic type A patient-years. Long-term follow-up (216 patient-years; range, 1 month to 22 years; mean, 5.4 years) revealed 15 late deaths, 7 from late aortic sequelae. The overall actuarial survival estimates were 71 +/- 8%, 54 +/- 10%, and 22 +/- 11% at 5, 10, and 15 years, respectively. Twenty late aortic operations were required in 14 patients. CONCLUSIONS: Despite satisfactory early results, the long-term survival of patients with the Marfan syndrome was suboptimal (albeit similar to those without the Marfan syndrome). Future progress will pivot on reducing the incidence of aortic dissection in these patients with medical therapy and/or earlier surgical intervention and enhanced postoperative serial imaging surveillance of the entire aorta.

Actuarial Analysis↗