Careful Blood Pressure Control Research Articles

Can progression of autosomal dominant or autosomal recessive polycystic kidney disease be prevented?

Data from animal and human studies suggest that the rate of progression of renal insufficiency can be retarded with careful control of blood pressure, institution of a low-protein diet, and the use of lipid-lowering agents. These therapeutic interventions become important when managing patients with renal insufficiency secondary to autosomal dominant polycystic kidney disease (PKD) and autosomal recessive polycystic kidney disease, in which end-stage renal disease is present in nearly 17,000 individuals per year. Several dietary and pharmacologic intervention strategies including blood pressure control, dietary modification, and the use of antioxidants as well as lipid-lowering agents have been studied in humans and animals with PKD in an effort to slow the rate of renal progression. This article reviews the current understanding of the effectiveness of these conventional therapies, as well as novel therapies that specifically target the mediators of cyst formation in PKD using tyrosine kinase inhibitors and gene therapy in an effort to identify potential strategies for retarding cyst formation and parenchymal injury in PKD. Current pharmacologic and dietary strategies fail to show any consistent benefits in preserving renal function and reducing renal injury in human PKD. The therapeutic potential for exciting new gene therapies and pharmacologic agents designed to target the pathophysiologic pathways involved in cyst formation are promising. Randomized, controlled trials in children and adults with early PKD are necessary to evaluate the effectiveness of these therapeutic interventions.

Commentary: Audit of mortality in type 2 diabetes

The paper by Allan et al underscores (a) the view that diabetes is a cardiovascular disease and (b) the appropriateness of the management of patients with type 2 diabetes in primary care. Diabetes is the ideal candidate for shared care between the primary and secondary care sectors. In a UK survey carried out in 1991, diabetes care schemes accounted for 48% of all shared care schemes in operation. The practical design varied widely, and about one third had been set up only since 1989. Shared care has been defined as “the joint participation of hospital consultants and general practitioners in the planned delivery of care for patients with a chronic condition, informed by an enhanced information exchange over and above routine discharge and referral notices”. In the case of diabetes, shared care involves input from a range of health care professionals and a collaborative team approach is essential. The forthcoming National Service Framework (NSF) for diabetes in the UK will undoubtedly argue that the most important person in the team is the patient, who must undertake a responsible role in the constant monitoring of their condition. Over the past three decades many types of shared care systems for diabetes have been tried and tested in the UK. Greenhalgh has systematically reviewed the literature, examining more than fifty schemes (over half of which were unpublished). The main findings were as follows: 1 Established shared care schemes of two types, centralised hospital based and consultant led or decentralised, community based and multidisciplinary. 2 A closely knit steering group or enthusiastic key individual, often the consultant diabetologist, could be clearly identified in all of the published successful schemes. 3 Structured care by primary care physicians with an interest in diabetes and supported by an enthusiastic specialist liaison team to produce comparable and occasionally superior levels of care to those provided solely by the hospital diabetes department. Unstructured care was ineffective and wasteful of resources. 4 Three randomised controlled trials showed that successful share care schemes had two common features – a centralised prompting system for recall and a structured check list for the primary care physician. 5 Successful district wide schemes shared three common features A Extensive planning phase when objectives were clearly defined and the facilities, expertise and commitment of primary care were assessed. B Locally developed written guidelines for the management of diabetes. C A well documented outreach system from the hospital by highly trained nurse facilitators who could advise on practical problems, maintain enthusiasm and enable fast tracking of patients for specialist review. The Fife programme incorporated all of these features. Diabetes is recorded as a cause of death on a minority of death certificates for patients with diabetes. Life years lost for patients with diabetes is strongly related to age at diagnosis and is a means of expressing mortality without relying on accurate prevalence data. The light at the end of the tunnel for these patients is careful control of blood pressure down to a level of 130 over 80 and treatment of other risk factors, particularly aggressive lipid lowering.

Cerebral Vasoreactivity and Internal Carotid Artery Flow Help to Identify Patients at Risk for Hyperperfusion After Carotid Endarterectomy

Hyperperfusion syndrome is a rare but potentially devastating complication after carotid endarterectomy (CEA). The aim of this study was to investigate whether preoperative measurement of cerebral vasoreactivity (CVR) and intraoperative measurement of internal carotid artery (ICA) flow could identify patients at risk for hyperperfusion after CEA. For 26 patients with unilateral ICA stenosis >/=70%, cerebral blood flow (CBF) and CVR were investigated before and 1 month after CEA, with resting and acetazolamide-challenge single-photon emission CT. CBF on the first postoperative day was also measured. ICA flow was measured before and after reconstruction by electromagnetic flowmeter during surgery. Ipsilateral CBF on the first postoperative day significantly increased relatively (56.6+/-53.2%) as well as absolutely (37.9+/-8.8 to 57.7+/-18.0 mL/100 g per minute) in the reduced CVR group (CVR <12%) but not in the normal CVR group (CVR >/=12%) (10.3+/-15.5% and 40.6+/-7.9 to 43.9+/-5.7 mL/100 g per minute, respectively). One month later, this difference almost disappeared. Two patients showed ipsilateral CBF increase of >/=100%. A significant association of intracerebral steal with hyperperfusion (CBF increase >/=100%) on the first postoperative day was also observed. ICA flow increase after reconstruction significantly correlated with CBF increase on the first postoperative day in the reduced CVR group but not in the normal CVR group. The threshold of ICA flow increase for hyperperfusion was estimated to be 330 mL/min in the reduced CVR group. Single-photon emission CT with acetazolamide challenge and ICA flow measurement during surgery could identify patients at risk for hyperperfusion after CEA, in whom careful monitoring and control of blood pressure should be initiated even intraoperatively.

Expectant management of severe pre-eclampsia in the mid-trimester

Objective: To determine maternal and perinatal outcomes with expectant management of severe pre-eclampsia in the mid-trimester, using a defined entry point. Design: Prospective case series. Thirty-nine women admitted from 24 to 27 week’s gestation with severe pre-eclampsia, whose pregnancies were otherwise stable, were managed expectantly with careful clinical and biochemical monitoring of maternal and foetal status, together with careful blood pressure control, in a high-care obstetric ward. The aim was to safely prolong the pregnancies and thereby improve perinatal outcome. Results: Gestation was prolonged by a median of 12 (range 3–47) days, with greater periods gained at earlier gestations. The overall perinatal loss was 26% and the neonatal loss 17%. The rates of significant maternal complications were low. Conclusion: Expectant management of selected women with severe pre-eclampsia from 24 to 27 weeks’ gestation in a tertiary care unit is acceptably safe and improves perinatal outcome.

Factors associated with perioperative morbidity and mortality in patients with pheochromocytoma: analysis of 165 operations at a single center.

To identify preoperative factors associated with 30-day morbidity and mortality after pheochromocytoma surgery, we carried out an external review of the records of all patients undergoing pheochromocytoma surgery from 1975 to 1997 at a single center. One hundred and forty-seven patients, including 23 with malignant tumors at the time of the first operation, underwent 165 operations. Death, resection of a neighboring organ, further surgery, secondary transfer to an intensive care unit, and any events associated with a surgical stay exceeding 10 days were defined as complications. Mortality and morbidity were 4 of 165 (2.4%) and 38 of 161 (23.6%), respectively. Morbidity included 13 spleen resections and hematomas. Spleen complications were not related to tumor location, but were probably due to the operative strategy used, a transperitoneal complete abdominal exploration including both adrenal glands. Complications were independently associated with preoperative systolic blood pressure [odds ratio (OR), 1.14/cm Hg], urinary metanephrine excretion (OR, 1.18/10 micromol x day), and with the number of operations (repeat vs. first operation OR, 5.36). In conclusion, pheochromocytoma resection consistently involves a risk of complications. Spleen damage should be prevented by complete preoperative localization studies and an elective or laparoscopic surgical approach. Careful blood pressure control should help prevent complications. Patients with high secretion tumors and those undergoing repeat intervention are at high risk of complications and should be referred to centers familiar with pheochromocytoma management.

Relationship between blood pressure and glucose tolerance in acromegaly

Hypertension represents a well-known risk factor for cardiovascular diseases. The pathogenesis of hypertension in acromegaly is commonly viewed as multifactorial, but the possible influence of metabolic disorders on blood pressure (BP) in affected patients is largely unknown. The aim of the present study was to evaluate the impact of glucose metabolism abnormalities on BP values in a series of patients with active acromegaly. An open multicentre prospective study. Sixty-eight patients with active disease, aged 47.5 +/- 11.7 years, have been studied. Thirty-nine had normal glucose tolerance (NGT), 16 impaired glucose tolerance (IGT) and 13 suffered from diabetes mellitus (DM). Mean clinical BP values were calculated as the mean of BP values obtained by sphygmomanometric measurement in three separate occasions and mean 24-h, diurnal and nocturnal systolic (SBP) and diastolic (DBP) values were obtained by 24-h ambulatory blood pressure monitoring (ABPM). Patient's age and the degree of glucose tolerance abnormalities were found to significantly and independently influence BP values. All clinical and ABPM SBP and DBP values significantly increased with age by linear regression (P < 0.02 for all BP values, 0.30 < or = R < or = 0.43), and the independent influence of this parameter on BP values was confirmed by mutivariate analysis. Similarly, the independent influence of glucose tolerance abnormalities on BP values was confirmed when introducing age as a covariable in a multivariate analysis, and patients with DM presented significantly higher clinical SBP and 24-h, diurnal and nocturnal SBP and DBP than patients with NGT (P < 0.02 for clinical SBP, P < 0.015 for all ABPM values, respectively). In addition, patients with DM showed significantly higher 24-h, diurnal and nocturnal DBP than those with IGT (P < 0.05 in all cases). In contrast, no significant difference was found between NGT and IGT patients. No significant influence of disease duration, BMI, GH, IGF-I, or fasting and 2-h post glucose load insulinaemia on BP values was observed. Abnormalities of glucose metabolism significantly contribute to increase systolic blood pressure and especially diastolic blood pressure in acromegalic patients. Careful control of blood pressure and of risk factors for developing systemic hypertension, with special reference to glucose tolerance, is mandatory to decrease cardiovascular morbidity and mortality in such patients.

Mechanisms of cerebral hemorrhage after carotid endarterectomy

115 Background: Intracerebral hemorrhage (IH) following carotid endarterectomy (CEA) has a high morbidity and mortality. Traditionally attention has focussed on the cerebral hyperperfusion (HP) syndrome as the leading cause of IH following CEA. Other mechanisms such as an intraoperative event, reperfusion into an old infarct and anticoagulation may also be important. Methods: We performed a case-control study to identify factors leading to IH following CEA. Over a ten year period (1990–9), all CEAs at the Mayo Clinic were assessed for the occurrence of IH within 30 days of the CEA. The relationship of IH to known cerebrovascular risk factors, perioperative EEG and xenon-133 cerebral blood flow (CBF) studies was compared with a control group of CEA patients. Clinical history and imaging of ischemic events and the IH were carefully reviewed in order to determine the possible underlying mechanism(s). HP was defined as the occurrence of doubling of CBF or the presence of hypertension with confusion, severe headache or seizures. Results: Twelve of 2747 (0.4%) CEAs had a subsequent IH. The most common underlying mechanism was HP. Doubling of xenon-133 CBF values were more likely to occur in patients with HP than controls but only one of three patients with a likely hyperperfusion IH had clinical features of the described syndrome. IH associated with HP occurred into the hemisphere surrounding the infarct except in one case of a recent infarct in which the hemorrhage occurred directly into the infarct. A peri-operative event and anticoagulation contributed to IH alone or in combination with other mechanisms in multiple patients. Seven of the 12 IH patients died or remained severely disabled. Conclusion: IH following CEA is an uncommon complication with a high mortality and morbidity. It usually occurs in the setting of HP, peri-operative event or anticoagulation with HP identifiable in the majority of cases. Identification of doubling of CBF at surgery may assist in identifying patients likely to develop HP following CEA and in these patients careful control of blood pressure and avoidance of anticoagulation is important.

Expectant management of early onset, severe pre-eclampsia: maternal outcome.

To evaluate the safety and outcome of women undergoing expectant management of early onset, severe pre-eclampsia. Prospective case series extending over a five-year period. Tygerberg Hospital, a tertiary referral centre. All women (n = 340) presenting with early onset, severe pre-eclampsia, where both the mother and the fetus were otherwise stable. Frequent clinical and biochemical monitoring of maternal status, together with careful blood pressure control, in a high care obstetric ward. Major maternal complications and prolongation of gestation. Multigravid women constituted 67% of the group. Antenatal biochemistry was reassuring with some expected, but not severe, deteriorations. Twenty-seven percent of women experienced a major complication, but few had poor outcomes. No maternal deaths occurred. Most major complications resolved quickly, necessitating only three admissions (0.8%) to the intensive care unit. One woman required dialysis. Pregnancies were prolonged by a mean (median) number of 11 days (9) before delivery, with more time being gained at earlier gestations. The postpartum inpatient stay (89% < or =7 days, bearing in mind that 82% of women were delivered by caesarean section) was not extended. Careful noninvasive management of early onset, severe pre-eclampsia in a tertiary centre can diminish and limit the impact of serious maternal complications. Valuable time to prolong the pregnancy and improve neonatal outcome is thereby gained.

Expectant management of early onset, severe pre-eclampsia: perinatal outcome.

To evaluate the perinatal outcome of expectant management of early onset, severe pre-eclampsia. Prospective case series extending over a five-year period. Tertiary referral centre. All women (n = 340) presenting with early onset, severe pre-eclampsia, where both mother and the fetus were otherwise stable. Frequent clinical and biochemical monitoring of maternal status with careful blood pressure control. Fetal surveillance included six-hourly heart rate monitoring, weekly Doppler and ultrasound evaluation of the fetus every two weeks. All examinations were carried out in a high care obstetric ward. Prolongation of gestation, perinatal mortality rate, neonatal survival and major complications. A mean of 11 days were gained by expectant management. The perinatal mortality rate was 24/1,000 (> or = 1,000 g/7 days) with a neonatal survival rate of 94%. Multivariate analysis showed only gestational age at delivery to be significantly associated with neonatal outcome. Chief contributors to neonatal mortality and morbidity were pulmonary complications and sepsis. Three pregnancies (0.8%) were terminated prior to viability and only two (0.5%) intrauterine deaths occurred, both due to placental abruption. Most women (81.5%) were delivered by caesarean section with fetal distress the most common reason for delivery. Neonatal intensive care was necessary in 40.7% of cases, with these babies staying a median of six days in intensive care. Expectant management of early onset, severe pre-eclampsia and careful neonatal care led to high perinatal and neonatal survival rates. It also allowed the judicious use of neonatal intensive care facilities. Neonatal sepsis remains a cause for concern.

Sensitivity, specificity, and surgical impact of somatosensory evoked potentials in descending aorta surgery

Background. We evaluate the efficiency of multilevel somatosensory evoked potential (SEP) monitoring for intraoperative re-establishment of blood flow to the spinal cord in 63 patients undergoing descending aorta repair. Methods. The presence of critical vessels in a cross-clamped aortic segment was ascertained by a 15 minute SEP observation period while the segment between the clamps was vented to drain out the collateral flow. Results. SEPs influenced the surgical strategy in 17 cases (27%): use of the Biomedicus in 1 traumatic rupture; critical vessel reimplantation or distal clamp replacement in 13 cases of segmental spinal ischemia; and hastening the procedure or proximal clamp replacement in 3 cases of left carotid ischemia. There were no cases of unexplained multilevel SEP abnormalities. Immediate paraplegia was observed in 2 cases (1 pre-existing; 1 forecast by a 199-minute period of SEP absence due to segmental ischemia); 2 patients presented delayed paraplegias despite unchanged intraoperative SEPs, and 1 case presented a transient paraplegia due to lower motoneuronal involvement. Conclusions. SEPs efficiently identified critical vessels to be reimplanted in order to avoid immediate paraplegia. However, systematic additional vessel reimplantation, if technically feasible, and prolongation of SEP monitoring during the postoperative period with careful blood pressure control are needed to prevent delayed paraplegia.

Surgical Interruption of Patent Ductus Arteriosus in Children

From January 1981 to September 1996, 447 patients with patent ductus arteriosus (122 male and 325 female) aged from 0.75 to 14 years, underwent surgery in our institute. Systolic blood pressure was maintained below 90 mm Hg with sodium nitroprusside and a vertical incision was made under the left axilla through the third intercostal space. Simple ligation was performed when the ductus was less than 10 mm in diameter. In 313 patients with a patent ductus arteriosus of 10 mm diameter or greater, a Dacron cushion was placed under the ligature. There was no death or evidence of residual patency. We recommend careful control of systolic blood pressure during the whole procedure and the use of a cushion under the ligature when closing a large ductus.

How to protect the kidney in diabetic patients: with special reference to IDDM.

During the development to overt nephropathy, diabetic patients go through several characteristic stages of renal disease, moving from normo- to micro- to macroalbuminuria. Microalbuminuria is defined as a urinary albumin excretion between 20 and 200 microg/min; values <20 microg/min are designated as normoalbuminuria, and values >200 microg/min are designated as macroalbuminuria. Only with macroalbuminuria does the glomerular filtration rate (GFR) fall consistently. The decisive intermediary endpoints are postponement or prevention of micro/macroalbuminuria and reduction or prevention of the fall in GFR (stronger endpoint), with postponement of end-stage renal disease as a final endpoint. Good metabolic control can prevent or postpone the development of microalbuminuria, the earliest sign of diabetic renal disease. The ideal realistic therapeutic window may be an HbA1c value between 7 and 8.5% (mean reference value 5.5%). Thus, efforts should aimed at implementing the best possible control before the onset of microalbuminuria, with the other important aim of minimizing hypoglycemic side effects. In patients with microalbuminuria, blood pressure gradually increases, and early antihypertensive treatment becomes crucial. Good glycemic control (with the same glycemic goal as above) may be difficult to achieve in some of these patients, but it is still important. With overt nephropathy, defined as clinical proteinuria, a relentless decline in GFR is inflicted, unless patients are carefully treated with antihypertensive agents, often in combination therapy. Good metabolic control is still strongly warranted because patients with high HbA1c progress much more rapidly. The natural history of the rate of fall in GFR may be reduced from 12 to 3 ml x min(-1) x year(-1), but genetic factors may be involved; the ACE-genotype DD seems to progress more rapidly during treatment. Protein restriction is also of some interest. Early screening is recommended in all guidelines, with emphasis on testing for albuminuria, including microalbuminuria, along with careful control of glycemia and blood pressure.

P-1-122 - Surgery of the anomalles of the vertebral artery

Superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis has been used in moyamoya disease (MD) and non-moyamoya ischemic stroke (non-MD). It is important to monitor hemodynamic changes caused by bypass surgery for postoperative management. We evaluated the bypass blood flow during STA-MCA anastomosis by using indocyanine green (ICG) fluorescence angiography.We evaluated the bypass blood flow in 13 MD and 21 non-MD patients during STA-MCA anastomosis by means of ICG angiography with injection of ICG into the anastomosed STA. The ICG perfusion area was calculated when the ICG fluorescence intensity reached maximum. We measured cortical oxygen saturation before anastomosis by means of visual light spectroscopy.ICG angiography demonstrated bypass blood flow from the anastomosed STA to the cortical vessels in all patients. The ICG perfusion area in MD (20.7 ± 6.6 cm2) was significantly larger than that in non-MD (8.4 ± 9.1 cm2, P < 0.05). The cortical oxygen saturation (58.9% ± 8.3%) in MD was significantly lower than that in non-MD (73.4% ± 9.5%, P < 0.05).ICG angiography with injection of ICG into the bypass artery allowed quantitative assessment of bypass blood flow. The bypass supplies blood flow to a greater extent in MD than in non-MD during surgery. This might be caused by a larger pressure gradient between the anastomosed STA and recipient vessels in MD. These observations indicate that MD requires careful control of systemic blood pressure after surgery to avoid cerebral hyperperfusion syndrome. ICG angiography is considered useful for facilitating safe and accurate bypass surgery and providing information for postoperative management.

P-1-123 - Arterial reconstruction for vertebral artery stenosis at the origin

Superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis has been used in moyamoya disease (MD) and non-moyamoya ischemic stroke (non-MD). It is important to monitor hemodynamic changes caused by bypass surgery for postoperative management. We evaluated the bypass blood flow during STA-MCA anastomosis by using indocyanine green (ICG) fluorescence angiography.We evaluated the bypass blood flow in 13 MD and 21 non-MD patients during STA-MCA anastomosis by means of ICG angiography with injection of ICG into the anastomosed STA. The ICG perfusion area was calculated when the ICG fluorescence intensity reached maximum. We measured cortical oxygen saturation before anastomosis by means of visual light spectroscopy.ICG angiography demonstrated bypass blood flow from the anastomosed STA to the cortical vessels in all patients. The ICG perfusion area in MD (20.7 ± 6.6 cm2) was significantly larger than that in non-MD (8.4 ± 9.1 cm2, P < 0.05). The cortical oxygen saturation (58.9% ± 8.3%) in MD was significantly lower than that in non-MD (73.4% ± 9.5%, P < 0.05).ICG angiography with injection of ICG into the bypass artery allowed quantitative assessment of bypass blood flow. The bypass supplies blood flow to a greater extent in MD than in non-MD during surgery. This might be caused by a larger pressure gradient between the anastomosed STA and recipient vessels in MD. These observations indicate that MD requires careful control of systemic blood pressure after surgery to avoid cerebral hyperperfusion syndrome. ICG angiography is considered useful for facilitating safe and accurate bypass surgery and providing information for postoperative management.

Management of early nephropathy in diabetic patients.

Diabetic patients go through several stages of renal disease, moving from normo- to micro- to macroalbuminuria. Good metabolic control can prevent or postpone the development of microalbuminuria, the earliest sign of diabetic renal disease. Thus, efforts should focus on obtaining the best possible control before the onset of microalbuninuria. In patients with microalbuminuria, blood pressure starts to increase, and early antihypertensive treatment becomes important. Good glycemic control may be difficult to achieve. With overt nephropathy, defined as clinical proteinuria, a relentless decline in glomerular filtration rate (GFR) occurs unless patients are carefully treated with antihypertensive agents. Protein restriction may also be necessary, but a clear beneficial effect of optimized diabetes care is difficult to document. Early screening is recommended, with an emphasis on testing for albuminuria, including microalbuminuria, along with careful control of blood pressure.

New composite graft repair for patients with and without Marfan's syndrome

We developed a new composite graft repair technique to prevent the complications associated with Bentall-type procedures. The technique consists of the creation of a double proximal suture line and direct coronary artery implantation as a Carrel patch without wrapping. We retrospectively compared the outcome in patients with Marfan's syndrome (n = 16) and other disorders (aortitis, 11 patients; degenerative disease, 18 patients) who had undergone this procedure. In patients with Marfan's syndrome, there was one hospital death and six deaths during long-term follow-up. Among patients with Takayasu's arteritis, there was one hospital death and one late death. There was one late death in the patients with degenerative disease. No mortality or complications directly related to the surgical procedure occurred in any group. The high late mortality in patients with Marfan's syndrome was primarily due to distal aortic dissection or rupture. Thus, the major complications associated with Bentall-type procedures can be prevented through the use of our new composite graft technique. However, close serial follow-up, careful blood pressure control, and early detection and treatment of distal recurrence are mandatory in the postoperative management of patients with Marfan's syndrome.

Hypertension-Related Renal Injury: A Major Contributor to End-Stage Renal Disease

The US Renal Data System Annual Report reveals that 57% of new cases and of end-stage renal disease are attributed to hypertensive nephropathy and diabetic nephropathy. Analyses of the data on serum creatinine from the Multiple Risk Factor Intervention Trial confirms that one in 20 of the hypertensive men exhibits a rate of decline in renal function that equals or exceeds 3% per year, the rate of loss being greater in older men, black men, and men with higher baseline blood pressure. Effective blood pressure treatment with maintenance of diastolic blood pressure below 95 mm Hg protected renal function in non-blacks but not in blacks, despite comparable blood pressure reduction in blacks. A longitudinal study of a diabetic cohort (n = 131) revealed that hypertension, plasma angiotensin II, and aldosterone are independent predictors of accelerated loss of renal function in diabetic nephropathy, possibly aggravated by diuretic usage as part of the antihypertensive regimen in diabetic nephropathy. No significant loss of renal function could be documented in those individuals whose blood pressure was adequately controlled (systolic blood pressure, < 140 mm Hg). These findings provide emphasis for the importance of adequate blood pressure control in both essential hypertension and hypertension associated with diabetes mellitus. They also provide support for the proposal that careful blood pressure control offers promise for reducing the incidence of end-stage renal disease in both hypertensive nephropathy and diabetic nephropathy.

Coarctation resection in children with Turner’s syndrome: A note of caution

Eight children were recognized to have Turner's syndrome, among 353 patients over 1 year of age who had undergone surgical treatment for coarctation of the aorta. Of these eight children, three developed a significant perioperative hemorrhage from aortic rupture, resulting in one death and one instance of paraparesis related to a period of prolonged hypotension. In two of the other five patients with Turner's syndrome, a decision was made to perform an angioplasty rather than a resection of the coarctation because of apparent friability of the aortic wall. In contrast, only one of the 345 patients without Turner's syndrome died as a result of surgical treatment, and none developed spontaneous perioperative aortic rupture or neurologic deficit. This experience suggests that the operative risk for coarctation of the aorta in this subgroup of patients is considerably greater than that in patients without Turner's syndrome (p < 0.001). Special precautions should include use of rubber-jaw vascular clamps, choice of technique to avoid tension at the anastomotic suture line, and careful control of systemic blood pressure intraoperatively and postoperatively. Indications for surgical treatment of coarctation as well as the type of operative procedure must be individualized cautiously in patients with Turner's syndrome.