Identification of patients with autosomal dominant polycystic kidney disease at highest risk for end-stage renal disease

To identify those potential factors that, early in the course of disease, mark a population of patients with autosomal dominant polycystic kidney disease (ADPKD) who have worse renal survival, survival analysis and risk ratio calculation for 1215 ADPKD patients were performed. Survival times were calculated as time to dialysis, transplantation, or death. Risk ratios were calculated using the Cox proportional hazards model. Three hundred eighty-eight patients entered end-stage renal disease and 205 patients died. ADPKD2 subjects had longer renal survival than ADPKD1 subjects (median survival, 68 versus 53 yr; P < 0.0005; risk ratio, 2.5). Women had significantly better renal survival than men (56 versus 52 yr; P < 0.0001; risk ratio, 1.6). Subjects who were diagnosed before age 30 and those who developed hypertension before age 35 had worse renal survival than those subjects who were diagnosed after age 30 or those who remained normotensive after age 35, respectively (age of diagnosis: 49 versus 59 yr; P < 0.0001; risk ratio, 3.2; hypertension: 51 versus 65 yr; P < 0.0001; risk ratio, 4.4). Similarly, those who had an episode of gross hematuria before age 30 had a worse renal outcome than those who did not (49 versus 59 yr; P < 0.0001; risk ratio, 2.6). We have also calculated risk ratios for a combined model. When therapeutic interventions become available for this disease, these populations with high risk ratios should be considered for such interventions.     

Regional differences in age-related decrements of the cutaneous vascular and sweating responses to passive heating

This study was performed to evaluate prognostic factors in ADPKD progression to ERSF. Previously reported negative factors (male gender, age, hypertension, palpable kidneys and UTI) as well as the extra-renal presence of cysts and proteinuria, were analysed in a group of 45 ADPKD patients (Male/Female, 25/20; Age = 40.1 +/- 19.7 yrs, range 21-69). Palpable kidneys were associated with higher serum creatinine values (955 +/- 689 vs 743 +/- 504 umol/l, p < 0.001) but not with a greater prevalence of renal failure. Renal failure (100% vs 60%), higher creatinine values (981 +/- 495 vs 778 +/- 654 umol/l) and hypertension (50% vs 18%) were related to a higher prevalence of extra-renal cysts (p < 0.05). Older patients (> 40 years) had a greater prevalence of renal failure (96% vs 32%, p < 0.001). Also, subjects with palpable kidneys, and those with extra-renal cysts, were significantly older (52.8 +/- 10.3 vs 30.5 +/- 20.6 yrs, p < 0.025; and 42.1 +/- 21.9 vs 38.1 +/- 18.2 yrs, p < 0.025). Patients with renal failure and those with extra-renal cysts had a greater prevalence of proteinuria (65% vs 0%, p < 0.001; and 100% vs 24%, p < 0.001). No correlation was seen for male gender, hypertension or UTI with any known complications of ADPKD. The extrarenal presence of cysts, older age, proteinuria and palpable kidneys were associated with a worse renal outcome, but for this Romanian population we can't confirm previous reports suggesting a role for male gender and early onset of disease.     

Intracranial arterial dolichoectasia in autosomal dominant polycystic kidney disease

Autosomal dominant polycystic kidney disease (ADPKD) is a systemic disorder with a variety of cardiovascular manifestations. This study presents a group of patients with ADPKD who had intracranial arterial dolichoectasia. One hundred seventy-eight ADPKD patients were screened with magnetic resonance angiography, 40 ADPKD patients had conventional angiography, and 98 ADPKD patients underwent a brain autopsy. For comparison, 360 patients without ADPKD who had magnetic resonance angiography and conventional angiography or brain autopsy were also studied. The prevalence of asymptomatic intracranial arterial dolichoectasia was 2.2% (4 of 178), 2.5% (1 of 40), and 2.0% (2 of 98) in the three ADPKD groups, respectively. None of the patients without ADPKD had intracranial arterial dolichoectasia. In addition to the seven patients with asymptomatic disease, two ADPKD patients with vertebrobasilar dolichoectasia had posterior circulation ischemic symptoms. The mean age of the nine patients (five men and four women) was 56.6 yr (range, 41 to 67 yr). The posterior circulation was involved in five patients, the anterior circulation was involved in two patients, and both were involved in two patients. Arterial dissection was believed to have caused middle cerebral artery dolichoectasia in one patient, and intracranial arterial dissections were strongly suspected in two other patients. Six of the nine patients with intracranial arterial dolichoectasia had additional vascular manifestations of ADPKD. Some patients with ADPKD are at an increased risk of developing intracranial arterial dolichoectasia and dissections. Recognizing this association is important because (1) it may be a cause of stroke; (2) it may mimic a saccular aneurysm on radiographic studies; and (3) it suggests that the arteriopathy of ADPKD may be more generalized than previously believed.     

Effects of difructose anhydride III on calcium absorption in small and large intestines of rats

Patients with autosomal dominant polycystic kidney disease (ADPKD) have an increased risk of intracranial aneurysms. Reports on arterial aneurysms in other locations have not been conclusive. The present study was initiated to investigate the prevalence of coronary aneurysms. Thirty ADPKD patients who had undergone coronary angiography on clinical indication were identified, 15 after renal transplantation. For each ADPKD patient, a control patient was identified with end-stage renal disease, investigated by coronary angiography, and matched for age, sex, and time relation to transplantation. All angiograms were retrieved and reevaluated with respect to aneurysms, defined as an increase in artery diameter by 50% or more, as well as to pathologic ectasias not fulfilling this criterion. Aneurysms were detected in four ADPKD patients and two control subjects. Five more ADPKD patients, but none of the control subjects, had minor ectasias. One ADPKD patient had a dissecting aortic aneurysm, and another died of aortic dissection during bypass surgery. This study adds to the evidence of an increased risk of extracranial aneurysms in ADPKD patients.     

The prevalence of seminal vesicle cysts in autosomal dominant polycystic kidney disease

BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is a systemic hereditary disorder characterized by bilateral diffuse renal cysts. Extrarenal involvement is a well known manifestation of ADPKD. Data relating to the association between seminal vesicle cysts and ADPKD are limited. The aims of this study are to evaluate the frequency of seminal vesicle cysts in ADPKD and to assess the relationship between seminal vesicle cysts, with cysts in the liver and prostate, and creatininaemia. METHODS: Forty five male patients (mean age 40 years, range 13-67) were included in the study. Each subject underwent a formal interview, physical examination; and abdominal and transrectal ultrasonography. Three patients were infertile, but one of the patients also had varicocele. RESULTS: Seminal vesicle cysts were present in 27 (60%) patients. Liver and prostate cysts were present in 19 (42%) and five (11%) patients, respectively. There was a positive correlation between seminal vesicle cysts, cysts in the liver, and serum creatinine concentrations. CONCLUSION: Our conclusions are: (i) seminal vesicle cysts are not uncommon in ADPKD; (ii) ADPKD should be looked for in patients with seminal vesicle cysts, and (iii) the clinical significance of seminal vesicle cysts in ADPKD remains to be defined.     

Rupture of a previously documented small asymptomatic intracranial aneurysm in a patient with autosomal dominant polycystic kidney disease. Case report

Intracranial aneurysms are common extrarenal manifestations of autosomal dominant polycystic kidney disease (ADPKD). Although their natural history is not completely understood, small asymptomatic intracranial aneurysms in patients with ADPKD often are not treated but are followed with serial magnetic resonance (MR) angiography. The authors report the unique case of a patient with ADPKD who bled from a previously documented asymptomatic 3-mm intracranial aneurysm. This 42-year-old man with ADPKD suffered a subarachnoid hemorrhage (SAH) from a 7-mm left pericallosal artery aneurysm. This aneurysm was clipped and the patient made an excellent recovery. An irregular asymptomatic 3-mm right middle cerebral artery (MCA) aneurysm had also been demonstrated on angiography. While the patient was considering elective surgery for the MCA aneurysm, he suffered a hemorrhage from this lesion 10 weeks after the initial SAH. The aneurysm was clipped and the patient made a satisfactory recovery (he was moderately disabled). In this report the authors indicate that small asymptomatic intracranial aneurysms are not always innocuous in patients with ADPKD, and they suggest that treatment should be strongly considered for these lesions in this group of patients when there is a history of SAH or the aneurysm is irregular in appearance. Because MR angiography studies may not adequately define the configuration of small aneurysms and irregularity may easily be missed, conventional angiography is recommended for patients with ADPKD who are found to have an intracranial aneurysm on screening with MR angiography.     

Effects of topical instillation of minocycline hydrochloride on cyst size and renal function in polycystic kidney disease

To reduce renal cyst size in autosomal dominant polycystic kidney disease (ADPKD), minocycline hydrochloride solution was instilled into the enlarged cysts in three ADPKD patients. In one patient with reduced renal function, such sclerotherapy apparently diminished cyst size, but without apparent improving effect on renal function at 7 months of follow-up. The second patient, who needed the replacement therapy on admission, had been free from hemodialysis over 4 months after the therapy. Persistent flank pain disappeared in both patients. In the third patient with normal renal function, sclerotherapy was done to get a better control of hypertension. Initially blood pressure decreased, but it returned up to the pre-therapy level irrespective of definite reduction of the enlarged cysts at 8 months of follow-up. The therapy with minocycline hydrochloride did not appear harmful, and may be helpful in the management of ADPKD.     

Factors relating to urinary protein excretion in children with autosomal dominant polycystic kidney disease

Adults with autosomal dominant polycystic kidney disease (ADPKD) who have overt proteinuria (>300 mg/d) have higher mean arterial pressures, lower creatinine clearances, larger renal volumes, and a more aggressive course of renal disease than ADPKD patients without proteinuria. This study examines the relationship between proteinuria and microalbuminuria and similar factors in ADPKD children. A total of 189 children from 81 ADPKD families was included in the analysis. The ADPKD children (n = 103) had significantly greater urine protein excretion rates than the non-ADPKD children (n = 86) (3.9+/-0.3 versus 2.8+/-0.2 mg/m2 per h, P < 0.001). Children with severe renal cystic disease (> 10 cysts; n = 54) had greater protein excretion than those with moderate disease (< or = 10 cysts; n = 49) (4.4+/-0.5 versus 3.3+/-0.2 mg/m2 per h, P < 0.05). The ADPKD children had significantly greater albumin excretion rates than the non-ADPKD children (32+/-6 versus 10+/-2 mg/m2 per 24 h, P < 0.001), and a higher percentage of ADPKD children had significant microalbuminuria (>15 mg/m2 per 24 h in boys and >23 mg/m2 per 24 h in girls) than their unaffected siblings (30% versus 10%, P < 0.05). Thirty percent of ADPKD children had albuminuria and 23% had overt proteinuria. For all ADPKD children, there was no correlation between proteinuria and hypertension. However, there was a significant correlation between urinary protein excretion and diastolic BP among children diagnosed after the first year of life (r = 0.23, P < 0.05). Therefore, proteinuria and albuminuria occur early in the course of ADPKD and may be markers of more severe renal disease.     

Cardiovascular abnormalities in children with autosomal dominant polycystic kidney disease

It is known that adults with autosomal dominant polycystic kidney disease (ADPKD) have an increased incidence of cardiovascular abnormalities, including mitral valve prolapse. The cardiac manifestations of ADPKD in the pediatric population have not been well established. To determine the cardiac manifestations of children with ADPKD, echocardiography was performed in 154 children of 66 families in which one parent has ADPKD. Eighty-six affected children and 68 unaffected children were evaluated in a prospective, single-blinded manner by echocardiography. Affected children were defined as those with any cysts on a concurrent renal ultrasound or those predicted to be gene carriers by gene linkage analysis. A 12% incidence of mitral valve prolapse was found in the affected children compared with only 3% of the unaffected children (P < 0.05). ADPKD children, but not their unaffected siblings, demonstrate a significant correlation between left ventricular mass index and systolic blood pressure. Moreover, hypertensive ADPKD children have significantly larger left ventricular mass index than do normotensive ADPKD children. A 3.5% incidence of congenital heart disease was found in the affected group, whereas 2.9% of the unaffected children had congenital heart disease. It was concluded that systemic manifestations of ADPKD, particularly cardiovascular abnormalities, are present even in childhood and these warrant the clinician's attention.     

A clinical and pathological study of 46 cases of sudden and unexpected death

A series of 46 autopsied adult cases of sudden and unexpected natural death were investigated. In this study, sudden and unexpected death was defined as any death occurring with 24 hours of onset of symptoms in a person with or without probable cause of death suggested by medical history. The cases included 31 males and 15 females aged 26 to 85 years (mean 66.6 years). Age distribution peaked in seventies. The lesions causing sudden and unexpected death according to the most frequent organ systems were, diseases of the heart (acute myocardial infarction with or without old infarct, 20; old myocardial infarction without acute infarction, 2; dilated cardiomyopathy, 2; sarcoidosis, 1; amyloidosis, 2; and valvular disease, 2), the aorta (ruptured aneurysm, 6; dissecting aneurysm, 2), the respiratory tract (pulmonary embolism, 7; pulmonary hypertension, 1), the alimentary tract (intestinal obstruction, 1), and other diseases (cause unknown, 1). The cardiovascular lesions were found in 78.2% of cases autopsied. The sudden and unexpected death caused by acute myocardial infarction was found in 47.8%, and acute myocardial infarction seemed to play a major role in cardiac sudden death in these series. The respiratory lesions were found in 17.4%. Four of seven cases with pulmonary embolism died in two weeks after surgical operation. The most common underlying disease was post-operative condition.     

Causes of lethal outcome in hypertension

Comparative analysis of the causes of fatal outcomes in hypertensive disease is presented on the grounds of postmortem findings in 2,091 patients who had died in hospitals in the period between 1953 and 1975. It was established that the mortality of disorders of cerebral circulation reduced significantly in 1973--75 as compared to that in 1963--05 and 1953--55. One fourth of patients with hypertensive disease died of myocardial infarction. It was noted that death of renal failure among patients with hypertensive disease decreased progressively.     

Germinal and somatic mutations in the PKD2 gene of renal cysts in autosomal dominant polycystic kidney disease

Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in one of three genes: PKD1 on chromosome 16 accounts for approximately 85% of cases whereas PKD2 on chromosome 4 accounts for approximately 15%. Mutations in the PKD3 gene are rare. All patients present with similar clinical phenotypes, and the cardinal symptom is the formation of fluid-filled cysts in the kidneys. Previous work has provided data supporting the notion that cysts in ADPKD1 are focal in nature and form after loss of function of polycystin 1. This became evident by demonstrating that the normal PKD1 allele was inactivated somatically by loss of heterozygosity or by mutagenesis in a subset of renal or liver cysts examined. We show in this report, for the first time, multiple novel somatic mutations within the PKD2 gene of epithelial cells, in both kidneys of an ADPKD2 patient. From a total of 21 cysts examined, seven (33%) had the same C insertion within the inherited wild-type allele. In two other cysts, a nonsense mutation and a splice site AG deletion had occurred in a PKD2 allele that could not be identified as the inherited wild-type or mutant. We suggest that the autosomal dominant form of ADPKD2 occurs by a cellular recessive mechanism, supporting a two-hit model for cyst formation.     

Association between pancreatic cystadenocarcinoma, malignant liver cysts, and polycystic disease of the kidney

Polycystic kidney disease is an autosomal dominant disease that may be associated with cystic disease of the liver. In women, the cysts may develop early and be more troublesome than in men. Cystadenocarcinoma of the pancreas is uncommon, comprising 1% of primary pancreatic malignancies. This case report is the first to describe a familial association between polycystic kidney disease and cystadenocarcinoma of the pancreas and liver in the English medical literature. A patient with autosomal dominant polycystic kidney disease (ADPKD) and multiple hepatic cysts developed cystadenocarcinoma of the pancreas with multiple malignant liver cysts. The patient's mother, sister, and niece had ADPKD, and the patient's sister also died of pancreatic cystadenocarcinoma. We believe that the development of these two disease entities in which the primary pathology is cyst formation has a genetic association.     

Systemic manifestations of adult polycystic kidney disease: an analysis of 205 cases

Adult polycystic kidney disease (APKD) is a common genetic disease and one of the important reasons of end stage renal failure. Although renal multiple cysts are clearly an important manifestation of APKD; other systemic manifestations are both common and clinically important. The authors reviewed 205 cases from 180 APKD families (107 male 98 female). Their age ranged from 10 to 71 years. Renal cyst is one of the many renal manifestations. Hypertension, hematuria and flank pain are its major complications. Hepatic cysts, pancreatic cysts, cardiac valvular lesions, intracranial aneurysms and splenic cysts are included in the array of systemic manifestations.     

Surgical treatment of renovascular hypertension

Two hundred sixty-four patients exhibiting renal artery occlusive disease underwent operation for renovascular hypertension between 1961 and 1977. Included were 27 pediatric patients. Fibrodysplastic disease affected 132 adults. Atherosclerotic lesions affected 51 patients with and 54 patients without clinically overt extrarenal arteriosclerotic cardiovascular disease. Ischemic kidney renin hypersecretion (renal: systemic index greater than 0.48), associated with suppressed contralateral kidney renin secretion (renal: systemic index approaching 0.0) predicted curability most reliably. Three hundred forty-eight operations were performed, of which 297 were primary and 51 were secondary procedures. Nephrectomy was initial therapy in 15 cases. Three operative deaths occurred among 51 patients manifesting overt extrarenal arteriosclerotic disease. No operative mortality was encountered among the other 213 patients. Surgical benefits were afforded 96% of pediatric patients and adults with fibrodysplastic disease, 91% of patients with focal renal arteriosclerosis, and 73% of those exhibiting overt extrarenal arteriosclerosis.     

Ruptured abdominal aortic aneurysm: the internist as diagnostician

PURPOSE: To define the clinical features and assess the frequency and causes of missed diagnoses of ruptured abdominal aortic aneurysm (AAA) in patients initially presenting to internists. PATIENTS: All identified patients with ruptured AAA presenting to internists during a 7 1/2-year period at a large academic medical center. METHOD: Chart review. RESULTS: We identified 23 patients with a ruptured AAA presenting to internists. Most had abdominal pain and tenderness, back or flank pain, and leukocytosis, whereas anemia and profound hypotension (systolic blood pressure below 90 mm Hg) were uncommon at presentation. In 14 cases (61%), the diagnosis of ruptured AAA was initially missed. Nine patients had an interval of 24 hours or more between presentation to the internist and surgery or death. The diagnosis was not made until after shock developed in nine patients who were hemodynamically stable at presentation. Of 17 patients who underwent surgery, 7 of 8 with preoperative shock died, compared with 2 deaths in 9 patients (p < .02) without shock. All six patients who did not have surgery died, yielding an overall mortality of 65% for the series. Ruptured AAAs were most frequently misdiagnosed as urinary tract obstruction or infection, spinal disease, and diverticulitis. Chart review revealed a general lack of physician awareness of the syndromes of contained rupture of AAA and symptomatic unruptured AAA. CONCLUSIONS: In patients with ruptured AAA who present to internists, the diagnosis is often delayed or missed and this appears to adversely effect survival. Internists should familiarize themselves with the presentation and management of ruptured AAA.     

Mortality following elective infrarenal aortic reconstruction: a Joint Vascular Research Group study

BACKGROUND: This study aimed to define the cause of death in patients undergoing elective infrarenal aortic reconstruction. METHODS: Members of the Joint Vascular Research Group who had collected details prospectively of patients undergoing elective aortic reconstruction provided information on those who died. RESULTS: Details of 3786 patients were obtained. Some 171 patients died (133 following abdominal aortic aneurysm (AAA) and 38 after aortofemoral bifurcation graft (AFBG) for occlusive disease). The mortality rate following AAA repair was 4.8 per cent, rising to 16 per cent if repair was combined with either renal or distal reconstruction (P < 0.001). Similar results were obtained with AFBG (3.4 and 11 per cent respectively, P < 0.001). The first major complication encountered was cardiac (39.8 per cent), followed by bleeding (20.5 per cent), respiratory (13.5), and gut (5.3 per cent), or limb ischaemia (6.4 per cent). Bleeding was commoner following reconstruction for aneurysm compared with that for occlusive disease (P < 0.05). Eighty-six patients (50.3 per cent) died from the first major complication. Of the remainder, 45 (53 per cent) developed multisystem organ failure (MSOF). The most commonly involved systems were cardiac, respiratory and renal. CONCLUSION: Cardiac problems were the major cause of death following infrarenal aortic reconstruction. MSOF is the 'final common pathway' in about half of the patients who survive the initial complication.     

Cerebral embolism in elderly patients with atrial fibrillation

Novalvular (nonrheumatic) atrial fibrillation (NVAF) is the most common cardiac condition associated with presumed embolic stroke, accounting for approximately half of the cardiogenic embolic infarctions. Of autopsied stroke patients in the Tokyo Metropolitan Geriatric Hospital, cerebral infarction was found in 75%, intracranial hemorrhage in 19%, and coexisting cerebral hemorrhage and cerebral infarction in 6%. Twenty-eight percent of the cerebral infarctions were embolic infarctions of cardiac origin, 56% of which were caused by NVAF. The incidence of cardiogenic brain embolism ranged from 6 to 23% of the ischemic strokes, and NVAF is the most frequent substrate for brain embolism. Atrial fibrillation increases in its incidence with increasing age. Chronic AF was observed in 10%, and paroxysmal AF in 7% of the autopsied elderly patients. Most of them were nonrheumatic AF. Twenty-two percent of the AF patients had large cerebral infarction, and 15% had medium-sized cortical infarction at the autopsy. NVAF is a very important cause of fatal massive cerebral infarction in the elderly. Of 56 patients with fatal massive cerebral infarction who died within 2 weeks after the strokes, 25 (45%) had embolic stroke associated with NVAF. Anticoagulant therapy prevents recurrent cerebral embolism of cardiac origin. The proper time to initiate anticoagulant therapy following cardiac brain embolism is controversial. Immediate initiation of anticoagulant therapy can reduce the early recurrence, but can result in secondary brain hemorrhage or hemorrhatic transformation. Patients with NVAF may have a lower risk of recurrence during the first 2 to 4 weeks following the initial embolic stroke compared with other cardioembolic sources. Cerebral embolism with NVAF can recur during a long period.(ABSTRACT TRUNCATED AT 250 WORDS)     

Pre-operative echocardiographic abnormalities and adverse outcome following renal transplantation

BACKGROUND: Premature cardiovascular disease is now the leading cause of death in renal transplant recipients. Although patients with progressive renal disease have many of the conventional risk factors for cardiovascular disease these do not have the same predictive power as they do in the general population. Echocardiographic abnormalities, notably left ventricular hypertrophy, have been shown to be associated with adverse outcome in patients on dialysis. METHODS: The echocardiograms were studied from 141 patients who were examined on the eve of renal transplantation between 1988 and 1990 to try to identify factors predicting outcome. Thirty-four patients have since died, 22 of cardiovascular disease. Ninety-three of the survivors and 27 of the dead patients had echocardiographic traces suitable for analysis. RESULTS: Left ventricular mass index was increased in those patients who died (median 167 vs 134 g/m2; P=0.03), as were end-systolic (4.3 vs 3.4 cm; P<0.01) and end-diastolic (5.8 vs 5.2 cm; P<0.01) diameters. Systolic function was also more severely impaired (fractional shortening, 27 vs 33%; P<0.01). Apart from age, only systolic function and end systolic diameter were independent predictors of outcome in multivariate analysis. CONCLUSIONS: This pattern of echocardiographic abnormality is similar to that reported in long-term dialysis populations, despite the adverse effects on survival. Moreover, despite potential benefits of transplantation on cardiac function, left ventricular hypertrophy, ventricular dilatation and systolic dysfunction were all associated with adverse outcome following transplantation. We conclude that echocardiography identifies markers for premature death following transplantation and provides targets for therapeutic intervention.     

Peroxidase activity of liver microsomal vitamin D 25-hydroxylase and cytochrome P450 1A2 catalyzes 25-hydroxylation of vitamin D3 and oxidation of dopamine to aminochrome

OBJECTIVES: This study sought to determine the long-term risk of sudden cardiac death in patients with hemodynamically stable sustained ventricular tachycardia complicating coronary artery disease. BACKGROUND: The prognosis and risk of sudden cardiac death in patients with a history of myocardial infarction and ventricular tachyarrhythmias have not been clearly defined. Prior studies are limited by a short follow-up period and by inclusion of patients with heterogeneous cardiac diseases and presenting arrhythmias. METHODS: A retrospective cohort analysis was performed on data from 124 patients, followed up for a mean of 36 +/- 30 months, who received electrophysiologically guided therapy for hemodynamically stable ventricular tachycardia after remote myocardial infarction. RESULTS: Seventy-eight patients were treated pharmacologically (medical group), and 46 patients underwent map-guided subendocardial resection (surgical group). Nine patients (7.3%) died suddenly, 5 (4.0%) died of noncardiac causes, 9 (7.3%) died of a perioperative complication, and 20 (23.4%) died of other cardiac causes. At 1, 2 and 3 years, sudden death occurred at cumulative rates of 2 +/- 1%, 3 +/- 2% and 7 +/- 3%, whereas total mortality was 20 +/- 4%, 28 +/- 4% and 32 +/- 5% (mean +/- SD). Sudden cardiac death (p = 0.047) and total mortality (p = 0.036) were higher in patients with multivessel disease and were similar for both treatment groups. CONCLUSIONS: Although the overall mortality in postinfarction patients presenting with hemodynamically stable ventricular tachycardia treated with electrophysiologically guided antiarrhythmic therapy is high, the risk of sudden death in these patients appears to be low (average 2.4%/year).