Management of impotence. Diagnostic considerations and therapeutic options

Impotence affects an estimated 10 million American men. The cause is usually organic in men over age 50; psychogenic impotence is more common in younger men. Vascular disease is the most common cause of impotence. Evaluation in patients with impotence includes thorough history taking and diagnostic testing. Once the cause of impotence is determined, appropriate management can be chosen. Current therapeutic options include vacuum tumescence devices, self-injection, oral therapy, psychotherapy, and penile prostheses.     

Homozygous deletion of the PTEN tumor suppressor gene in a subset of prostate adenocarcinomas

PURPOSE: Intracavernosal injection therapy is one of the most popular therapies for erectile dysfunction today. Yet, most clinicians consider intracavernosal injection a palliative treatment for erectile dysfunction because of the high patient initiated dropout rate. In contrast, penile prostheses appear to offer a more permanent cure for erectile dysfunction. We compare the long-term outcomes of both therapies in contemporaneously treated patients and determine the reasons for failure of each. MATERIALS AND METHODS: Telephone survey and chart review was conducted on the first 115 patients treated with intracavernosal injection and 65 patients undergoing insertion of a penile prosthesis during the same period at our institution. Mean patient age was 57 and 60 years, respectively, and mean followup of all patients was 5.4 years (range of 3.3 to 16). RESULTS: An equal percentage of patients were lost to followup in both groups, including 19% of the intracavernosal injection group and 18% of the penile prosthesis group. Of the intracavernosal injection patients 6 (6%) died during followup and 10 (19%) of the prosthetic patients died (p < 0.05). At the time of contact only 41% of the patients were still using intracavernosal injection. In contrast, 70% of the patients were still sexually active with the prosthesis (p < 0.01). Mean duration of use of the penile prosthetics was 63 months compared to 37 months for intracavernosal injection (p < 0.001). The most common reasons for discontinuing intracavernosal injection were inadequate erections (16 cases), lack of spontaneity (14), side effects (12), lack of partner (10), loss of sexual interest (6) and spontaneous return of normal erections (4). More than half of the patients (61%) who discontinued intracavernosal injection remain sexually active with other therapies, including penile prosthesis in 11, vacuum devices in 4, vascular surgery in 1 and oral medication in 1, and 14 without any therapy. We could not identify any significant clinical parameters that would accurately predict which patients most benefited by the long-term use of intracavernosal injection therapy. In contrast, only 6 patients discontinued use of the implant because of complications (infection, erosion and malfunction) and 7 for reasons independent of the implant (that is lack of partner, loss of sexual interest and co-morbidity). CONCLUSIONS: Intracavernosal injection serves as only a palliative therapy for the majority of patients with erectile dysfunction but there exists a core group who derives long-term satisfaction with its use. The majority of patients who discontinue intracavernosal injection remain sexually active yet do not progress to more invasive or effective therapies. The reason for discontinuing therapies for erectile dysfunction is often unrelated to the actual therapeutic modality. Our findings suggest that further improvements in intracavernosal injection therapy and the development of alternative methods of delivery of vasoactive agents will have only a limited impact on the overall outcome of therapy for erectile dysfunction and that increased attention to issues separate from the erection is warranted.     

Anal and penile condylomas in HIV-negative and HIV-positive men: clinical, histological and virological characteristics correlated to therapeutic outcome

Clinical, histological and HPV DNA hybridization findings were analyzed for 73 homosexual and 38 heterosexual men attending for anal warts; therapy results were evaluated retrospectively for 76 of these patients. Concurrent anal and penile warts occurred most commonly in the heterosexual men (p < 0.001). While perianal warts were most common in heterosexuals (p < 0.05), intraanal warts were most common in homosexuals (p < 0.001). Altogether 23 homosexual men were HIV-infected; 13 HIV-positive men followed regarding therapeutic outcome were immunologically relatively intact with mean CD4 counts of 524/mm3. Of 136 biopsy specimens 70% revealed benign hyperplasia, 27% AIN I, 2% AIN II and none AIN III. Of ISH positive samples 94% contained HPV 6/11 and 6% HPV 16/18/31/33. Anal warts were cured after an average of 2.5 (mean 1-10) therapy sessions in 64% of heterosexual, in 84% of HIV-negative homosexual and in 62% of HIV-positive homosexual men. The mean number of therapy sessions against anal warts was highest (p < 0.001) and the time for accomplishing cure for anal and penile warts was longest (p < 0.001) in the heterosexual study group.     

Use of intralesional verapamil to dissolve Peyronie's disease plaque: a long-term single-blind study

OBJECTIVES: Multiple conservative therapies for the treatment of Peyronie's disease have been offered with variable and poor response rates. Calcium channel blockers have been shown in vitro and in vivo to inhibit secretion and synthesis of extracellular matrix, including collagen, glycosaminoglycans, and fibronectin, as well as causing increased collagenase and anti transforming growth factor-beta activity. Calcium antagonists, including verapamil, are effective in stimulating the remodeling and degradation of extracellular matrix in tissue by altering the metabolic pathways of fibroblasts. Recently, a pilot study (1994) showed preliminary promising results in treating plaque caused by Peyronie's disease. This randomized single-blind placebo-based study (1994 to 1996) was undertaken to confirm the hypothesis. METHODS: In this randomized single-blind study, 14 patients completed the study and were divided into two groups: the verapamil treatment group (n = 7) or the control saline group (n = 7). Verapamil or saline was injected directly into the Peyronie's plaque once a week for 6 months. Patients were evaluated before and after treatment with duplex ultrasound to confirm the extent of the lesion and to measure volume of the plaque, and by interview and mailed questionnaire 3 months after treatment. Patients being treated with oral calcium antagonists were excluded from the study. RESULTS: A decreased plaque volume was measured in 57% of the verapamil-treated men versus 28% in the control group (P <0.04). Penile curvature demonstrated an improvement trend of 37.71 +/- 9.3 degrees to 29.57 +/- 7.3 degrees in the verapamil-treated patients, but the difference was not significant (P <0.07). Plaque softening was noted in all patients treated with verapamil. There was significant objective improvement in plaque-associated penile narrowing in all patients in the verapamil group. Subjective plaque-associated erectile dysfunction (quality of erection) showed improvement in 42.87% of the verapamil group versus none in the control group (P <0.02). There was no local or systemic toxicity except for an occasional ecchymosis/bruise at the injection site. After a positive clinical response, plaque size, penile angulation, and symptoms continued to improve. Decrease in plaque size was noted in each of the responders in the first 3 months. CONCLUSIONS: This randomized single-blind study suggests that intralesional injection of calcium channel blocker may be a reasonable approach in some selected patients for the treatment of Peyronie's disease with noncalcified plaque and penile angulation of less than 30 degrees. Patients whose plaque failed to respond to intralesional verapamil therapy within 3 months or whose angulation was greater than 30 degrees at presentation were more likely to benefit from surgery.     

Clinical experience with Dynaflex penile prostheses in 120 patients

OBJECTIVES: To present our experience with the Dynaflex (American Medical Systems) self-contained inflatable penile prosthesis and define specific complication and patient dissatisfaction rates. METHODS: From May 1990 through January 1998, 120 men underwent implantation of a Dynaflex prosthesis. The follow-up period ranged from 2 to 80 months (mean 42). RESULTS: Specific complication rates observed after penile prosthesis implantation were as follows: prosthetic infection, 4.16%; mechanical device failure, 7.5%; and patient dissatisfaction because of inability to work the pump, 16.66%. After intensive teaching, the patient dissatisfaction rate dropped to 0.83%. The overall complication rate was 14.15%. CONCLUSIONS: With several types of penile prostheses available, Dynaflex can be regarded as an appropriate alternative with a relatively low mechanical failure rate. However, careful preoperative assessment and patient education in the use of the pump mechanism are essential to obtain a successful result.     

Long-term followup and selection criteria for penile revascularization in erectile failure

PURPOSE: We report the long-term results of penile revascularization surgery for erectile failure and suggest possible selection criteria for this controversial surgical procedure. MATERIALS AND METHODS: In 7 years 62 impotent men who did not respond to pharmacotherapy underwent microsurgical penile revascularization and completed long-term followup evaluation in 41 months (range 18 to greater than 62) consisting of a detailed questionnaire, duplex sonography and optional pharmacotherapy or angiography. The Virag procedure was chosen for the first 7 patients, the original Hauri technique for the next 13 and the modified Mannheim triple anastomosis for 42. RESULTS: Of all patients 34% achieved spontaneous and another 20% pharmacologically induced erections. Success in diabetics and older patients was lower (43% for diabetics, 39% for those older than 50 years at surgery), while it was high in men with less than 2 risk factors (58%) as well as in younger patients (69% for those up to 50 years old). Shunt patency was 92%. Complications such as glans hyperemia developed in 13% of patients, shunt thrombosis in 8% and inguinal hernias in 6.5%. CONCLUSIONS: Patient selection is vital for the successful outcome of penile revascularization surgery. We adhere to strict selection criteria, such as patient age maximum of 50 years, less than 2 risk factors, no recent diabetes and termination of nicotine abuse. Penile revascularization surgery is highly indicated in this group of patients, especially since it is the only causal therapy for erectile failure.     

Penile prostheses for the management of the neuropathic bladder and sexual dysfunction in spinal cord injury patients: long term follow up

During the last 10 years, 90 penile prostheses were implanted in 82 patients with spinal cord injury. Surgery was done 1 month to 25 years (average 4.8 years) after the injury. The follow up period ranged from 1 to 10 years (average 4 years). A prosthesis was implanted for urinary management in 51 patients (62%), for sexual dysfunction in 10 patients (12%) and for both purposes in 21 patients (26%). Ninety-three per cent of the patients who used the implant for urinary management and 64% of the patients who used it for sexual dysfunction were satisfactory. We experienced three extrusions and nine surgical removals due to pain, difficulty of catheterisation and infection (the complication rate was 13.3%). Generally speaking, a penile prosthesis improves the quality of life of patients with spinal cord injury significantly; however, extrusion and infection are still significant problems.     

Gestational choriocarcinoma metastatic to the brain treated successfully by stereotactic radiosurgery and chemotherapy

This report analyzes the intracorporal lengths of 236 consecutive organically impotent men who underwent insertion of a penile prosthesis. The average right or left intracorporal length in this series was 20.9 cm, with a standard deviation of 2.2 cm. Intracorporal length ranged from 14-27 cm. This data may be used as reference ranges for penile prosthesis manufacturers, and for Urologists who implant prostheses or perform penile lengthening procedures.     

Clinical guidelines panel on erectile dysfunction: summary report on the treatment of organic erectile dysfunction. The American Urological Association

PURPOSE: The American Urological Association convened the Clinical Guidelines Panel on Erectile Dysfunction to analyze the literature regarding available methods for treating organic erectile dysfunction and to make practice recommendations based on the treatment outcomes data. MATERIALS AND METHODS: The panel searched the MEDLINE data base for all articles from 1979 through 1994 on treatment of organic erectile dysfunction and meta-analyzed outcomes data for oral drug therapy (yohimbine), vacuum constriction devices, vasoactive drug injection therapy, penile prosthesis implantation and venous and arterial surgery. RESULTS: Estimated probabilities of desirable outcomes are relatively high for vacuum constriction devices, vasoactive drug injection therapy and penile prosthesis therapy. However, patients must be aware of potential complications. The outcomes data for yohimbine clearly indicate a therapy with marginal efficacy. For venous and arterial surgery, based on reported outcomes, chances of success do not appear high enough to justify routine use of such surgery. CONCLUSIONS: For the standard patient, defined as a man with acquired organic erectile dysfunction and no evidence of hypogonadism or hyperprolactinemia, the panel recommends 3 treatment alternatives: vacuum constriction devices, vasoactive drug injection therapy and penile prosthesis implantation. Based on the data to date, yohimbine does not appear to be effective for organic erectile dysfunction and, thus, it should not be recommended as treatment for the standard patient. Venous surgery and arterial surgery in men with arteriolosclerotic disease are considered investigational and should be performed only in a research setting with long-term followup available.     

Immediate salvage procedure for infected penile prosthesis

PURPOSE: The most devastating complication after the insertion of a penile prosthesis is the development of infection. The standard approach involves removing the entire device, treating intensively with antibiotics and attempting to reinsert a prosthesis at a later date, often with a suboptimal result. Based on the encouraging results of others, during the last 24 months we have used in 2 separate private urological practices a salvage procedure for treatment of infected inflatable penile prostheses. MATERIALS AND METHODS: The protocol used in 7 men with an infected inflatable penile prosthesis included removal of all device components, a 7-step vigorous intraoperative irrigation with 4 different solutions, including vancomycin, immediate reimplantation of a new inflatable penile prosthesis and postoperative outpatient antibiotics with oral ciprofloxacin or intravenous vancomycin or cefazolin. RESULTS: Of the 7 men 6 have experienced excellent results with no infection, minimal morbidity and preservation of penile length. The only failure occurred in a poorly controlled diabetic who required multiple revisions and may have had latent infection for months before it became apparent. CONCLUSIONS: We believe that an immediate salvage procedure for an infected inflatable penile prosthesis is an effective treatment for this difficult complication.     

Implant-supported overdenture therapy: a retrospective study

All patients (n = 46) treated with implant-supported overdentures at the Department of Prosthetic Dentistry, Dental and Medical Health Centre, Halmstad, Sweden, from 1986 to 1993 were studied. The clinical examination was completed in 1994. The material was divided into two subgroups: Group A had been initially treatment planned for an implant-supported overdenture, and Group B had been planned for fixed prostheses but because of loss of implants before loading, treatment with a fixed prosthesis was not possible. The authors present their experience and patient reactions to overdenture therapy in two defined groups of patients. The implant failure rate before loading for Group A (n = 12) was 15% (six implants out of 39), and the rate before loading for Group B (n = 29) was 43.6% (68 implants out of 156). After prosthodontic treatment in Group A, the implant success rate after loading was 87.9%, and the overdenture stability was 84.6%. In group B, 17 implants placed in the maxillae were lost after overdenture therapy, which resulted in an implant success rate of 79.3%. A total of eight overdentures, all of which had been placed in the maxillae, were lost, resulting in an overdenture stability of 73.3%. In this study "change of retentive clips" was the predominant prosthodontic complication related to the overdentures, especially in Group B. Most of these complications (62%) occurred in patients with clinical signs of bruxism. Patient reactions to treatment with an overdenture were positive regarding esthetics for both groups. More negative views were recorded in Group B than in Group A in response to function and retention of the overdenture.     

Erectile response to transurethral alprostadil, prazosin and alprostadil-prazosin combinations

PURPOSE: Transurethral alprostadil has been shown to be efficacious in many men with erectile dysfunction. We compared transurethral alprostadil and prazosin alone, and in combination to treat this disorder. MATERIALS AND METHODS: In this double-blind, placebo controlled study the erectile responses to transurethral alprostadil, prazosin and alprostadil-prazosin combinations were assessed in 234 men 26.8 to 81.5 years old with complete organic erectile dysfunction. Patients self-administered a random sequence of 7 doses in the clinic in 4 weeks. The erectile response was assessed using categorical and visual analog scales. RESULTS: Full penile enlargement or rigidity was achieved by 165 of the 234 men (70.5%) after at least 1 active dose of medication. The most effective alprostadil dose (500 microg.) resulted in full penile enlargement or rigidity in 51.8% of administrations, whereas the most effective prazosin dose (2,000 microg.) and placebo resulted in a similar response in 12.7 and 2.7%, respectively (p <0.001). The 500/2,000 microg. alprostadil/prazosin combination, which resulted in full enlargement or rigidity in 58.9% of doses, was only slightly better than the most effective dose of alprostadil alone (500 microg.). However, combinations of 125/500 and 250/500 microg. alprostadil/prazosin were more effective (p <0.01) than 125 and 250 microg. alprostadil given alone, respectively. The most common side effect of therapy was penile pain, which rarely led to study discontinuation. Hypotension most commonly developed at the higher alprostadil-prazosin combination. CONCLUSIONS: Transurethral alprostadil and alprostadil-prazosin combinations produced erections in men with complete organic erectile dysfunction. This combination therapy may be an option in patients who do not respond to transurethral alprostadil alone.     

Treatment of recurrent penile condylomata acuminata with external application and intraurethral instillation of bacillus Calmette-Guerin

PURPOSE: Condylomata acuminata are caused by human papillomavirus infection. Despite numerous treatment modalities these patients often demonstrate recurrent disease. We report initial experience with bacillus Calmette-Guerin (BCG) therapy in patients not responding to standard treatment. MATERIALS AND METHODS: Between October 1994 and March 1997, 6 men with rapidly recurrent external and intraurethral condylomata acuminata underwent BCG therapy after initial laser treatment. External application and intraurethral instillation of BCG were performed 6 times in weekly intervals. Followup studies included examination and endoscopic inspection of the urethra and bladder. RESULTS: Of the patients 3 completed 1 course of BCG and had no relapse of condylomata acuminata, 2 underwent a second course of BCG and 1 had relapse, and 1 had relapse after discontinuing therapy due to penile edema. The annual recurrence rate decreased from 3.2 before to 0.75 after BCG therapy (p < 0.05, test of equality of 2 percentages). CONCLUSIONS: Immunotherapy with BCG is accepted treatment for superficial transitional cell carcinoma. The BCG induced immune response appears to reduce the recurrence rate in patients with condylomata acuminata.     

Myxobolus cerebralis in Tubifex tubifex from a whirling disease epizootic in Montana

Prolonged suppressive antibiotic therapy may be an alternative to removal of infected orthopedic prostheses in some patients. However, the efficacy of prolonged suppressive antibiotics is not well established. We retrospectively reviewed 18 patients with infected orthopedic prostheses who had been treated with prolonged antimicrobial suppression during the last 10 years. Eighteen episodes of infection were identified in these 18 patients. There were nine men and nine women, and the mean age was 66 years (range, 31-83 years). All patients had a functional prosthesis and were treated with surgical debridement, retention of the prosthesis, and administration of intravenous antibiotics for 6-8 weeks, followed by prolonged oral antibiotic suppression. Fifteen of the 18 patients appear to have had a good response and have been able to retain a functional prosthesis. Complications related to antibiotic suppression occurred in 22% but did not necessitate discontinuation of the antibiotic therapy. Prolonged antibiotic suppression is a reasonable alternative to surgery in selected patients with infected orthopedic prostheses.     

Low dose flunarizine protects the fetal brain from ischemic injury in sheep

This retrospective long-term study analyzes the clinical function, failures, and radiographic status of 131 Richards Series 2 prostheses in total hip arthroplasties (THAs) performed during 1981 and 1982. After a minimum follow-up period of 11 years, the results were evaluated. Sixty two (54%) patients with 71 THAs were available for follow-up; 44 (38%) patients with 49 THAs had died. Three patients with three THAs were lost to follow-up, and seven patients with eight THAs were unable to participate in the examination due to causes not related to their present hip status. Ten patients underwent revision THA during the observation period. The median hip functional index (HFI) was 8.6 preoperatively and 15.8 at follow-up. Hip functional index correlated to radiographic signs of loosening. With revision as endpoint, the Kaplan-Meier survivorship for all 131 hips was 93% after 10 years and 91% after 12 years. The radiographic examination at follow-up revealed 5 (8%) definite loose cups and 12 (19%) definite loose stems. Including the number of aseptic loosened cups and stems confirmed at revision, aseptic loosening was found in 6% of all cups and 13% of all stems. This radiographic evaluation confirmed that lack of containment of the cup and varus position of the stem were factors influencing the long-term stability of the prostheses, whereas etiology, type of arthritis, and ectopic ossification did not. A fixation and stability score that was modified for cemented prostheses was developed and was of value in detecting the prostheses at risk. The long-term results of the Richards Series 2 THA are acceptable and comparable with other long-term studies on conventional prosthetic designs. Ten percent to 20% revision THAs due to aseptic loosening can be expected. In addition, patients younger than age 60 at surgery and especially men were found to belong to a high-risk group with regard to revision THA. These patients must be evaluated carefully so that revision THA can be performed before the bone bed is destroyed.     

A surgical algorithm for the treatment of Peyronie's disease

PURPOSE: When conservative treatment of Peyronie's disease fails, the optimal surgical approach is not well defined. Multiple factors, including penile rigidity, degree of curvature, shaft narrowing with hinge effect and erectile response to vasoactive penile injections, indicate that no single approach is likely to solve the problem in all patients. MATERIALS AND METHODS: A surgical algorithm was developed for the treatment of Peyronie's Disease based on our previous surgical experience, which was used prospectively in 103 consecutive men. Penile straightening without prosthesis was offered to patients with adequate rigidity for coitus. Specifically, for mild to moderate curvature less than 60 degrees without hourglass or hinge effect deformity the less complicated tunica albuginea plication procedure was performed. For those men with more severe, complex curvature greater than 60 degrees and/or significant hourglass or hinge effect deformity plaque incision or partial excision with dermal grafting was offered to limit shaft shortening and to reconstruct a shaft with normal caliber to provide optimal axial support during intromission. For men with poor sexually induced erections and/or inadequate response to intracavernosal pharmacotherapy penile prosthesis placement was recommended to provide adequate straightening and rigidity. RESULTS: Of 22 patients who underwent plication procedures 91% remained potent and the penis remained straight postoperatively. Of 52 patients who underwent an incision or partial excision and grafting procedure, 48 had dermal grafts with the penis remaining straight in 94% and 75% remaining potent postoperatively. A total of 29 patients received a prosthesis with the penis remaining straight in 93% who were sexually active postoperatively. During the follow up period (mean 22.3 months) there have been no mechanical device failures. CONCLUSIONS: Surgical outcome was optimized with this algorithm, which correlates surgical complexity to the underlying severity of the penile deformity and erectile capacity.     

Penile vibratory stimulation in spinal cord injured men: optimized vibration parameters and prognostic factors

OBJECTIVE: To study the efficacy of penile vibratory stimulation (PVS) with optimized vibration parameters in spinal cord injured (SCI) men and to examine prognostic factors for success. DESIGN: Case series. SETTING: University hospital outpatient clinic. PATIENTS: Thirty-four consecutive SCI men seeking fertility treatment. INTERVENTION: PVS with optimized vibration parameters to induce reflex ejaculation. MAIN OUTCOME MEASURES: Ejaculatory response; semen analysis. RESULTS: Antegrade ejaculation was seen in 65% of patients. High rates were seen in lesions above T10 (81%) and in presence of hip flexion and bulbocavernosus reflexes (77%). Of men with lesions above T10, those with a penile prosthesis had lower ejaculation rates (40% vs 90%). Average total sperm counts were 968 million, with 26% motility. CONCLUSIONS: High rates of ejaculation are seen with optimized vibration parameters, especially in men with lesions above T10 and intact lower spinal reflexes. A penile prosthesis may impair success with PVS.     

Surgical treatment of Peyronie's disease: a new approach

We treated 12 patients with intractable Peyronie's disease with a new approach based on simple incision of the fibrotic plaque(s) and stenting of the corpora with penile implants. Infection in 1 case necessitated removal of the prostheses, while the remaining 11 patients had satisfactory functional and anatomical results. The procedure is indicated for patients with 1) Peyronie's disease and impotency, 2) normal potency and extensive disease and 3) normal potency and localized disease in selective cases. In this series insertion of the penile prostheses did not change sexual prowess in previously potent patients.     

Increased acceptance of the International Normalized Ratio (INR) as a monitoring parameter of oral anticoagulation therapy in Germany. GELIA Study Group

In 1994 the German Cardiac Society recommended the International Normalized Ratio (INR) instead of "Quick's test" for the long-term management of oral anticoagulation therapy. Parallel cardiac surgical centers in Germany and Switzerland started patient recruitment for the GELIA study (German Experience with Low Intensity Anticoagulation). By the end of 1996, 862 consecutive patients with mechanical heart valve replacement had been enrolled. According to the protocol of the GELIA study, intensity of anticoagulation, methods used to control the oral anticoagulation therapy as well as other factors relevant for the anticoagulation management are reported every three months. The so far accumulated data material of the GELIA study seemed, therefore, appropriate to analyze potential changes in the acceptance of INR during the time period 1993-1996. Potential differences in the anticoagulation management were analyzed separately according to the time of enrollment of patients and changes during the follow up period, e. g., a switch from "Quick's test" to INR. Taking the time of enrollment into account, the percentage of patients who had INR-controlled oral anticoagulation therapy increased from 0% (1993), 14.3% (1994), 63.2% (1995) to 74.2% in 1996. During the follow up, there was a change from "Quick's test" to INR controls in 7 patients (1994; 2.1%), 67 patients (1995; 10.5%) and 142 patients (1996; 17.3%) with a consequent increase of INR controlled patients to a total of 34.2% in 1994, 52.0% in 1995, and 73.9% in 1996.     

Intermediate survival in neonates with aortic atresia: a multi-institutional study. The Congenital Heart Surgeons Society

OBJECTIVE: Controversy persists with regard to the treatment of patients with aortic atresia. Staged reconstructive operations and primary transplantation have been advocated as treatment strategies, but in many instances no treatment is undertaken. A multi-institutional study was undertaken for the purpose of characterizing this challenging patient group, comparing the prevalence and outcomes of the various treatment strategies, and identifying potential predictors of success or failure with each. METHODS AND RESULTS: A total of 323 neonates with aortic atresia were entered into a 21-institution prospective, nonrandomized study between January 1, 1994, and January 1, 1997. Three protocols were used, nonexclusively in many institutions: (1) staged reconstructive surgery with initial palliation by a Norwood procedure and eventual Fontan operation, (2) heart transplantation as initial definitive therapy, and (3) nonsurgical management. Analysis was based on initial protocol assignment: staged reconstructive surgery in 253 patients, heart transplantation in 49 patients, and nonsurgical management in 21 patients. For all patients initially entered into the 2 surgical treatment protocols, survival at 1, 3, 12, 24, and 36 months after entry was 67%, 59%, 52%, 51%, and 50%, respectively. A multivariable analysis found incremental risk factors for death at any time after entry to be lower birth weight (P=.04), associated noncardiac anomaly (P=.007), and entry into the nonsurgical protocol (P < .0001) or the staged reconstructive surgery protocol (P=.03). Four institutions had higher survival statistics; 2 used a heart transplantation protocol and 2 used a staged reconstructive surgery protocol. For the 113 patients treated at these 4 institutions, survival at 1, 3, 12, 24, and 36 months after entry was 77%, 70%, 64%, 62%, and 61%, respectively. Survival among the 4 institutions was similar (P=0.1). CONCLUSIONS: Among patients with aortic atresia, other features of cardiac structure including aortic size, degree of left ventricular hypoplasia, and degree of mitral hypoplasia or atresia are not predictive of survival from 2 surgical protocols. The highest survival was achieved with either treatment strategy at institutions strongly committed to the use of one or the other surgical management protocol.