Assessment of behavioral determinants influencing success of supervised exercise therapy in patients with intermittent claudication: A cross sectional survey.

BACKGROUND: Supervised exercise therapy is the first step in treatment of intermittent claudication. However, adherence to supervised exercise therapy is low. Limited access and reimbursement issues are known reasons, though lack of motivation is often leading. Behavioral determinants influencing motivation and thus adherence to supervised exercise therapy remain to be investigated. In this study we sought to determine which behavioral determinants would be of influence on the long-term adherence of supervised exercise therapy. METHODS: 200 patients, newly diagnosed with peripheral arterial disease Rutherford classification II-III, were sent a questionnaire to assess motivation and behavior with regard to supervised exercise therapy. The questionnaire was constructed using the I-CHANGE model for explaining motivational and behavioral change. Baseline characteristics were acquired from medical records. Alpha Cronbach's was calculated to test reliability of the questionnaire. RESULTS: 108 (54%) patients returned their questionnaire. A total of 79% patients followed supervised exercise therapy. Patients who increased their walking distance after supervised exercise therapy have significantly greater knowledge (p = 0.05), positive attitude (p = 0.03) and lower negative attitude (p = 0.01). Patients with a higher self-efficacy remained significantly more active after participating in supervised exercise therapy (p = 0.05). CONCLUSION: Increasing the determinants knowledge, attitude and self-efficacy will improve adherence to supervised exercise therapy and result in delayed claudication onset time.

Articulating a Patient-Centered Design Space for Cancer Journeys.

Cancer journeys, encompassing patients' cancer experiences through survivorship, are complex and diverse. Individuals must cope with numerous physical and emotional challenges, balancing clinical tasks alongside responsibilities of daily life. Understanding the breadth of factors that contribute to a patient's cancer experience presents a critical challenge in developing holistic patient-centered technology. To further our understanding of the cancer journey, we conducted focus groups and interviews with 31 breast cancer patients. We present a cancer journey framework depicting the responsibilities, challenges, and personal impacts patients face while transitioning from diagnosis through post-treatment survivorship. Through this work, we aim to aid the development of health tools that consider a patient's cancer journey and health needs more broadly, supporting patient's health management alongside the complexities and priorities of daily life.

Morphometric analysis of unbalanced common atrioventricular canal using two-dimensional echocardiography.

OBJECTIVES: This study was designed to define morphometric echocardiographic variables of unbalanced common atrioventricular canal (CAVC) that could aid in appropriate referral for surgical repair. BACKGROUND: Unbalanced CAVC has a high surgical mortality rate. This may be secondary to inappropriate referral of some patients for two-ventricle repair (closure of septal defects) instead of single-ventricle repair (Norwood palliation and Fontan operation). METHODS: The echocardiograms of 103 patients with CAVC were retrospectively reviewed. In the subcostal left anterior oblique view, the area of the atrioventricular (AV) valve aportioned over each ventricle was measured, and an AV valve index (AVVI) was calculated as left/right valve area. The ventricular cavity ratio between the two ventricles was estimated as left ventricular length times width divided by right ventricular length times width. These variables were correlated with surgical referral and outcome. RESULTS: Patients previously categorized as having balanced CAVC all had AVVI > 0.67 (n = 77). Of the patients with unbalanced CAVC (n = 26), 11 had ductal-dependent circulation and underwent Norwood palliation (AVVI 0.21 +/- 0.13, mean +/- SD), and 15 had two-ventricle repair (AVVI 0.51 +/- 0.12, p < 0.0001). Of these 15 patients, 9 have survived, with no difference in mean AVVI between survivors and nonsurvivors (0.52 +/- 0.11 versus 0.49 +/- 0.13, p = 0.72). For all 103 patients, AVVI correlated with ventricular cavity ratio. However, of the unbalanced CAVC group who underwent two-ventricle repair, three nonsurvivors had a discrepancy between AVVI and ventricular cavity ratio (low AVVI but normal ventricular size). A large ventricular septal defect was present in all six nonsurvivors but in only four of nine survivors (p < 0.05). CONCLUSIONS: Echocardiographic morphometry is useful in defining unbalance in CAVC. If AVVI is < 0.67 in the presence of a large ventricular septal defect, a single-ventricle approach to repair should be considered.

Early changes in ventricular septal defect size and ventricular geometry in the single left ventricle after volume-unloading surgery.

OBJECTIVES: This study investigated the phenomenon of, and the relation between, alterations in ventricular geometry after acute surgical volume unloading of the ventricle and the development of subaortic stenosis in patients with a single ventricle and ventricular septal defect-dependent systemic flow. BACKGROUND: Subaortic outflow obstruction has been observed to occur in patients with a single left ventricle after placement of a pulmonary artery band. The timing and etiology of this phenomenon are not well defined. METHODS: The preoperative and postoperative echocardiograms of 18 patients 14.9 +/- 22.8 months old (mean +/- SD) with a diagnosis of single left ventricle who underwent pulmonary artery banding or cavopulmonary connection were reviewed. Postoperative studies were performed a mean of 7.0 +/- 6.5 days after operation. The ventricular septal defect diameter was measured in two orthogonal views and the area calculated using the formula for an ellipse. Interventricular septal and posterior wall thickness and left ventricular diameter and length were also measured. RESULTS: Mean ventricular septal defect area indexed to body surface area diminished by 36 +/- 23% (3.1 +/- 2.7 to 2.0 +/- 1.8 cm2/m2, p < 0.01). Mean interventricular septal and posterior wall thickness increased significantly, and left ventricular diameter and length decreased significantly. A greater diminution in ventricular septal defect area was noted after cavopulmonary connection (41 +/- 19%, p < 0.01) than after pulmonary artery banding (25 +/- 28%, p = 0.22). CONCLUSIONS: In the single left ventricle, diminution in ventricular septal defect size occurs early and is related to an acute alteration in ventricular geometry that accompanies the decrease in ventricular volume. Ventricular septal defect diminution was greater after volume unloading of the ventricle after cavopulmonary connection than after pulmonary artery banding.

The automatic implantable cardioverter-defibrillator: efficacy, complications and survival in patients with malignant ventricular arrhythmias.

Ninety-four patients underwent surgery for automatic implantable cardioverter-defibrillator implantation. Ninety patients were discharged from the hospital with the device and were followed up for a mean period of 17 +/- 10 months. Forty-six patients experienced at least one discharge of the device under circumstances consistent with a malignant ventricular arrhythmia. One sudden death occurred. Complications included perioperative death (3 patients), post-operative ventricular tachycardia (12 patients) and atrial fibrillation (8 patients), perioperative myocardial infarction (1 patient) and device discharges for sinus tachycardia and supraventricular arrhythmias (17 patients). Six and 12 month survival rates by life table analysis were 98.7 and 95.4%, respectively. Thus, the automatic implantable cardioverter-defibrillator is a highly effective and relatively low risk treatment modality for patients with refractory life-threatening ventricular arrhythmias.

Effect of long-acting somatostatin analog (Somatulin) on renal hyperfiltration in patients with IDDM.

OBJECTIVE: To investigate whether long-acting somatostatin (SMS) can suppress renal hyperfiltration in patients with IDDM. RESEARCH DESIGN AND METHODS: A double-blind, randomized treatment of nine patients with IDDM was used. Selection criteria were renal hyperfiltration (glomerular filtration rate [GFR] > or = 129 ml.min-1.1.73 m2) and absence of hypertension and macroalbuminuria. Treatment was either with a long-acting SMS analog (Somatulin, 30 mg) or with placebo, given by intramuscular injections every 10 days for 9 months. GFR, effective renal plasma flow (ERPF), IGF-I, and 24-h growth hormone (GH) profiles were used as evaluation parameters. RESULTS: Five patients were randomized to Somatulin, four patients to placebo. One of the patients treated with Somatulin stopped after 3 months because of persistent abdominal discomfort after the injections. Somatulin treatment for 3 months lowered GFR and ERPF compared with placebo (P < 0.05). After 9 months, the differences were no longer significant. After 3 months, IGF-I concentrations were decreased in all Somatulin-treated patients. GH secretion tended to increase in the placebo group. CONCLUSIONS: The administration of long-acting Somatulin to patients with IDDM and renal hyperfiltration leads to only a temporary reduction of ERPF/GFR.

Hypertriglyceridemia enhances monocyte binding to endothelial cells in NIDDM.

OBJECTIVE: The mechanisms by which diabetes leads to rapidly progressive atherosclerosis are not fully understood. Adherence of monocytes to the arterial wall is an early event in the development of atherosclerotic lesions. RESEARCH DESIGN AND METHODS: The binding of freshly isolated monocytes from patients with NIDDM, IDDM, and healthy control subjects to a monolayer of endothelial cells obtained from human umbilical vein was investigated. RESULTS: Endothelial adherence of monocytes from normolipidemic patients with IDDM (15.8 +/- 4.5%) or NIDDM (16.9 +/- 4.6%) was comparable to that of monocytes from a control population (15.3 +/- 3.5%). In patients with NIDDM with a serum triglyceride concentration > 2.5 mmol/l, the percentage of cells that adhere to endothelial cells in vitro was significantly increased (23.3 +/- 3.1%). Glycemic control did not correlate with monocyte adherence. The presence of symptomatic atherosclerotic disease, age, or sex was not associated with a change in monocyte binding in vitro. CONCLUSIONS: The results suggest that in NIDDM hypertriglyceridemia should be treated to reduce the high risk for atherosclerosis.

Free and total insulin-like growth factor I (IGF-I), IGF-binding protein-1 (IGFBP-1), and IGFBP-3 and their relationships to the presence of diabetic retinopathy and glomerular hyperfiltration in insulin-dependent diabetes mellitus.

The existing literature on serum insulin-like growth factor I (IGF-I) levels in insulin-dependent diabetes mellitus (IDDM) is conflicting. Free IGF-I may have greater physiological and clinical relevance than total IGF-I. Recently, a validated method has been developed to measure free IGF-I levels in the circulation. Serum free and total IGF-I, IGF-binding protein-1 (IGFBP-1), and IGFBP-3 levels were measured in 56 insulin-treated IDDM patients and 52 healthy sex- and age-matched controls. Diabetic retinopathy was established by direct fundoscopy. In 54 IDDM patients, the glomerular filtration rate (GFR) and effective renal plasma flow were calculated from the clearance rate of [125I]iothalamate and [131I]iodohippurate sodium. Fasting free IGF-I, total IGF-I, and IGFBP-3 levels were significantly lower in IDDM patients than in age- and sex-matched healthy controls (free IGF-I, P < 0.005; total IGF-I, P < 0.001; IGFBP-3, P = 0.001), whereas IGFBP-1 levels were higher (P < 0.001). In IDDM subjects, decreases in free IGF-I, total IGF-I, and IGFBP-3 levels with age were observed (free IGF-I, r = -0.27 and P = 0.05; total IGF-I, r = -0.52 and P < 0.001; IGFBP-3, r = -0.37 and P = 0.005). Free IGF-I was inversely related to fasting glucose in IDDM subjects (r = -0.35; P = 0.01), whereas the relationship between total IGF-I and fasting glucose did not reach significance (r = -0.27; P = 0.06). Age-adjusted free IGF-I levels were significantly higher (P < 0.05) in IDDM subjects with retinopathy than in subjects without retinopathy after adjustment for age. Total IGF-I and IGFBP-3 levels were positively related to GFR (total IGF-I, r = 0.35 and P < 0.05; IGFBP-3, r = 0.28 and P < 0.05). Both of these differences lost significance after adjustment for age. Free IGF-I, total IGF-I, and IGFBP-3 levels were lower and IGFBP-1 levels were higher in insulin-treated IDDM subjects compared to those in age- and sex-matched controls. Free IGF-I, total IGF-I, and IGFBP-3 levels decreased significantly with age in IDDM subjects. Age-adjusted free IGF-I levels in subjects with diabetic retinopathy were higher than those in subjects without diabetic retinopathy. Total IGF-I and IGFBP-3 levels were positively related to GFR in IDDM subjects, but these relations were lost after adjustment for age. Measurement of serum free IGF-I levels in IDDM subjects did not have clear advantages compared to that of total IGF-I, IGFBP-1, and IGFBP-3 levels. Serum IGF-I and IGFBPs reflect their tissue concentrations to a various degree. Consequently, extrapolations concerning the pathogenetic role of the IGF/IGFBP system in the development of diabetic complications at the tissue level remain speculative.

The pharmacokinetics of injectable allopurinol in newborns with the hypoplastic left heart syndrome.

OBJECTIVE: The purpose of this investigation was to determine the pharmacokinetic disposition of intravenous allopurinol and its metabolite oxypurinol in neonates with the hypoplastic left heart syndrome (HLHS) and to evaluate the subsequent degree of xanthine oxidase inhibition using serum uric acid as a marker. METHODS: Pharmacokinetic data were evaluated in 12 stable preoperative neonates with HLHS after a single intravenous allopurinol administration of 5 mg/kg or 10 mg/kg. Pharmacokinetic parameters were determined for elimination half-life, clearance, volume of distribution, and mean residence time. Xanthine oxidase inhibition, measured by serum uric acid reduction, was also measured. RESULTS: Pharmacokinetic parameters revealed no statistically significant differences between a 5-mg/kg and 10-mg/kg dose of intravenous allopurinol on elimination half-life, clearance, volume of distribution, and mean residence time. Mean serum uric acid levels were significantly reduced from baseline by 39.99 and 42.94%, respectively, in the 5- and 10-mg/kg treatment groups. DISCUSSION: The enzyme xanthine oxidase plays a key biochemical role in the generation of toxic oxygen-derived free radicals during ischemia-reperfusion conditions. Allopurinol and its active metabolite oxypurinol inhibit xanthine oxidase, and significantly reduce the conversion of hypoxanthine to xanthine and xanthine to uric acid. Cell injury may be caused by toxic oxygen free radicals produced by ischemia-reperfusion injury such as could occur during the repair of HLHS under hypothermic total circulatory arrest. We hypothesize that allopurinol may provide protection from cellular injury in this clinical context.

Comparison of patterns of pulmonary venous blood flow in the functional single ventricle heart after operative aortopulmonary shunt versus superior cavopulmonary shunt.

In this study we investigated the patterns of pulmonary venous flow in children with functional single ventricles to obtain a better understanding of the determinants of transpulmonary blood flow. Sixty-eight patients with functional single ventricles and aortopulmonary shunt (n = 34, group I), or superior cavopulmonary connection (n = 34, group II) underwent transesophageal Doppler echocardiographic assessment of flow in the left upper pulmonary vein before undergoing the next stage of surgery. Twelve patients from group II also underwent simultaneous evaluation of superior vena caval flow. Biphasic forward pulmonary venous flow was noted in 62 patients in sinus rhythm (S wave in systole, D wave in diastole); in 6 patients with junctional rhythm, significant early systolic reversal of flow was present. Both the S- and D-wave velocity-time integrals (VTI) were greater in group I than in group II (S(VTI) 9.9 +/- 4.2 vs 8.0 +/- 2.6, p = 0.02; D(VTI) 8.0 +/- 3.5 vs 4.2 +/- 2.6, p <0.001). In both groups, pulmonary venous flow was predominantly systolic; however, the proportion of flow during ventricular systole was significantly greater in group II than in group I (S(VTI)/D(VTI) group II: 2.4 +/- 1.5; group I 1.4 +/- 0.5, p = 0.001; percent systolic fraction of pulmonary venous flow group II = 67%, group I = 56%, p <0.001). Analysis of superior vena caval flow in group II revealed a single predominant wave with onset at early systole and peak in late systole at a mean of 150 ms after the pulmonary venous S-wave peak. Our data suggest that ventricular systole (i.e., atrial relaxation, atrioventricular valve descent) asserts great influence on transpulmonary blood flow in the functional single ventricle.

Relation between ionized calcium concentration and ventricular pump performance in the dog under hemodynamically controlled conditions.

The effect of plasma ionized calcium concentration on left ventricular function was studied in the canine heart on right heart bypass. Stroke volume, mean arterial pressure and heart rate were controlled. Plasma ionized calcium was lowered to 0.58 +/- 0.01 mM by citrate infusion and raised to 1.70 +/- 0.01 mM by calcium chloride infusion in random order in each dog. Left ventricular function at each of these ionized calcium levels was compared with that in an immediately preceding normocalcemic period. At a constant stroke work (16.9 +/- 0.2 g-m), sustained hypercalcemia was associated with a small decrease in left ventricular end-diastolic pressure (1.7 +/- 0.7 cm H2O, p less than 0.05) despite a marked increase in peak left ventricular dP/dt (first derivative of ventricular pressure) averaging 34 percent (p less than 0.001). Coronary blood flow, tension-time index and myocardial oxygen consumption were not significantly altered. Stroke work determined at a left ventricular end-diastolic pressure of 14 cm H2O, by interpolation in left ventricular function curves, was 11 +/- 4.4 percent above that at control normocalcemia (p less than 0.05). At a constant stroke work (16.9 +/- 0.2 g-m), sustained hypocalcemia was associated with a marked depression of left ventricular function as demonstrated by a substantial increase (from 4.9 +/- 0.3 to 12.7 +/- 1.1 cm H2O, p less than 0.0001) in left ventricular end-diastolic pressure (p less than 0.0001), decreased mean systolic ejection rate (p less than 0.01) and decreased peak left ventricular dP/dt (p less than 0.0001). Coronary blood flow increased (p less than 0.05) whereas myocardial oxygen consumption did not change significantly. A marked displacement of left ventricular function curves to the right (compared with curves obtained during normocalcemia) was observed, and stroke work determined at a left ventricular end-diastolic pressure of 14 cm H2O was 52 +/- 5.4 percent below control level (p less than 0.001). It appears that hypercalcemia, when initiated from a normal control level, provides only a small enhancement of ventricular pump performance (as indexed by the stroke work-left ventricular end-diastolic pressure relation) despite a marked increase in peak left ventricular dP/dt, whereas marked improvement of left ventricular performance may be expected when calcium infusion is initiated from an ionized calcium level that is below normal.

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.

Cerebral oxygenation during cardiopulmonary bypass in children.

OBJECTIVE: Previous work has found cerebral oxygen extraction to decrease during hypothermic cardiopulmonary bypass in children. To elucidate cardiopulmonary bypass factors controlling cerebral oxygen extraction, we examined the effect of perfusate temperature, pump flow rate, and hematocrit value on cerebral hemoglobin-oxygen saturation as measured by near infrared spectroscopy. METHODS: Forty children less than 7 years of age scheduled for cardiac operations with continuous cardiopulmonary bypass were randomly assigned to warm bypass, hypothermic bypass, hypothermic low-flow bypass, or hypothermic low-hematocrit bypass. For warm bypass, arterial perfusate was 37 degrees C, hematocrit value 23%, and pump flow 150 ml/kg per minute. Hypothermic bypass differed from warm bypass only in initial perfusate temperature (22 degrees C); hypothermic low-flow bypass and low-hematocrit bypass differed from hypothermic bypass only in pump flow (75 ml/kg per minute) and hematocrit value (16%), respectively. Cerebral oxygen saturation was recorded before bypass (baseline), during bypass, and for 15 minutes after bypass had been discontinued. RESULTS: In the warm bypass group, cerebral oxygen saturation remained at baseline levels during and after bypass. In the hypothermic bypass group, cerebral oxygen saturation increased 20% +/- 2% during bypass cooling (p < 0.001), returned to baseline during bypass rewarming, and remained at baseline after bypass. In the hypothermic low-flow and hypothermic low-hematocrit bypass groups, cerebral oxygen saturation remained at baseline levels during bypass but increased 6% +/- 2% (p = 0.05) and 10% +/- 2% (p < 0.03), respectively, after bypass was discontinued. CONCLUSIONS: In children, cortical oxygen extraction is maintained during warm cardiopulmonary bypass at full flow and moderate hemodilution. Bypass cooling can decrease cortical oxygen extraction but requires a certain pump flow and hematocrit value to do so. Low-hematocrit hypothermic bypass and low-flow hypothermic bypass can also alter cortical oxygen extraction after discontinuation of cardiopulmonary bypass.

Late graft patency and symptom relief after aorta-coronary bypass.

One hundred six consecutive patients underwent elective or emergency coronary artery bypass grafting (CABG) between January, 1974, and November, 1975. There were 90 men of an average age of 54 years and 16 women an average of 64 years. Unstable angina (preinfarction angina, angina decubitus, and crescendo angina) was present in 54 patients of this group and eight were in congestive heart failure. Sixty-two of the 106 had previously had myocardial infarctions and four had evolving infarctions. There were four operative deaths (3.8%) and one early hospital death (less than 30 days' hospitalization). Perioperative infarction occurred in five of the survivors. Of the 197 grafts placed in the 101 survivors, 94% were patent by angiography at 1 to 2 weeks (175 of 187 vein grafts and 10 of 10 left internal mammary grafts). At 1 to 2 years after CABG, 62% of the survivors consented to repeat angiography at which time 94% of the grafts were patent (101 of 108 vein grafts and seven of seven left internal mammary grafts). Clinical follow-up of 81 of the 101 survivors at 1 year found 99% of them to be asymptomatic or improved. Repeat clinical follow-up of all survivors (99 of 101) at 3 to 4 years found 93.9% asymptomatic or improved. Overall survival, including operative deaths, was 92.4% at 4 years.

Results of Norwood's operation for lesions other than hypoplastic left heart syndrome.

Norwood's operation provides satisfactory palliation for neonates with hypoplastic left heart syndrome. The dominant physiologic features of hypoplastic left heart syndrome, ductal dependency of the systemic circulation and parallel pulmonary and systemic circulations, are shared by a multitude of other less common congenital heart malformations. Theoretically, these should be equally amenable to palliation by Norwood's operation. Between January 1990 and June 1994, 60 neonates with malformations other than hypoplastic left heart syndrome underwent initial surgical palliation by Norwood's procedure. Diagnoses included single left ventricle with levo-transposition of the great arteries (12); critical aortic stenosis (8); complex double-outlet right ventricle (8); interrupted aortic arch with ventricular septal defect and subaortic stenosis (7); ventricular septal defect, subaortic stenosis, and coarctation of the aorta (7); aortic atresia with large ventricular septal defect (6); tricuspid atresia with transposition of the great arteries (6); heterotaxy syndrome with subaortic obstruction (3); and other (3). There were 10 hospital deaths and 50 survivors (83% survival). After the introduction of inspired carbon dioxide therapy into the postoperative management protocol (1991), 42 of 47 patients survived (89% survival). Mortality was independent of diagnosis and essentially the same as that for hypoplastic left heart syndrome. With minor technical modifications, Norwood's operation provides satisfactory initial palliation for a wide variety of malformations characterized by ductal dependency of the systemic circulation in anticipation of either a Fontan procedure or a biventricular repair.

Preoperative risk-of-death prediction model in heart surgery with deep hypothermic circulatory arrest in the neonate.

OBJECTIVE: Our goal was to generate a preoperative risk-of-death prediction model in selected neonates with congenital heart disease undergoing surgery with deep hypothermic circulatory arrest. METHODS: We completed a single-center, prospective, randomized, double-blind, placebo- controlled neuroprotection trial in selected neonates with congenital heart disease requiring operations for which deep hypothermic circulatory arrest was used. An extensive database was generated that included preoperative, intraoperative, and postoperative variables. Variables (delivery, maternal, and infant related) were evaluated to produce a preoperative risk-of-death prediction model by means of logistic regression. An operative risk-of-death prediction model including duration of deep hypothermic circulatory arrest was also generated. RESULTS: Between July 1992 and September 1997, 350 (74%) of 473 eligible infants were enrolled with 318 undergoing deep hypothermic circulatory arrest. The mortality was 52 of 318 (16.4%), unaffected by investigational drug. The resulting preoperative risk model contained 4 variables: (1) cardiac anatomy (two-ventricle vs single ventricle surgery, with/without arch obstruction), (2) 1-minute Apgar score (5), (3) presence of genetic syndrome, and (4) age at hospital admission for surgery (5 days). Mortality for two-ventricle repair was 3.2% (4/130). Mortality for single ventricle palliation was 25.5% (48/188) and was significantly influenced by Apgar score, genetic diagnosis, and admission age. The preoperative model had a prediction accuracy of 80%. The operative risk model included duration of deep hypothermic circulatory arrest, which significantly (P =.03) increased risk of death, with a prediction accuracy of 82%. CONCLUSIONS: In this selected population, postoperative mortality risk is significantly affected by preoperative conditions. Identification of infants with varying mortality risks may affect family counseling, therapeutic intervention, and risk stratification for future study designs.

Congenital Heart Surgery Nomenclature and Database Project: truncus arteriosus.

The extant nomenclature for truncus arteriosus (TA) is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. A modified Van Praagh (VP) classification is proposed involving three main categories of TA: TA with confluent or near confluent pulmonary arteries (large aorta type, VP A1, A2), TA with absence of one pulmonary artery (VP A3), and TA with interrupted aortic arch or coarctation (large pulmonary artery type, VP A4). A comprehensive database set is presented that is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented that will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Congenital Heart Surgery Nomenclature and Database Project: tetralogy of Fallot.

The extant nomenclature for tetralogy of Fallot (TOF) is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. The general categories of TOF are: classic TOF with varying degrees of pulmonary stenosis, TOF with common atrioventricular canal defect, and TOF with absent pulmonary valve. Although centers may choose to code a fourth general category, TOF with pulmonary atresia, this lesion will be grouped with pulmonary atresia-ventricular septal defect for multi-institutional analysis. A comprehensive database set is presented that is based on a hierarchical scheme. Data are entered at various levels of complexity and detail that can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented that will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Fontan operation: influence of modifications on morbidity and mortality.

The mortality rate of the Fontan operation for heart malformations with a single or dominant ventricle has been reduced by dividing the procedure into two stages. The hemi-Fontan procedure allows early reduction of the volume work of the single ventricle and remodeling of ventricular geometry before a completion Fontan operation. Despite the improvement of survival with this strategy (8% mortality for completion Fontan versus 16% mortality for primary Fontan operation), morbidity related to serous effusions remains substantial. Further technical modifications have been undertaken in an effort to reduce morbidity and further reduce mortality. From January 1990 through June 1993, 200 patients underwent completion Fontan procedures after previous hemi-Fontan operations. Mean age was 23 months, and 157 patients were less than 24 months of age. Diagnoses were hypoplastic left heart syndrome (127 patients), tricuspid atresia (19 patients), single left ventricle (17 patients), complex double-outlet right ventricle (16 patients), pulmonary atresia with intact ventricular septum (8 patients), and other (13 patients). Overall, early mortality rate was 8% (16 patients). In the last 112 patients, the procedure was modified technically by creating one or more fenestrations in the baffle used to separate systemic venous blood from pulmonary venous blood (36 patients), or by excluding one or more hepatic veins from the systemic venous pathway (76 patients). Early mortality for these 112 patients was reduced to 4.5% (5 patients). Substantial morbidity from serous effusions occurred at a rate of 45% (35 of 78 patients) among survivors who had received neither technical modification.(ABSTRACT TRUNCATED AT 250 WORDS)

Congenital Heart Surgery Nomenclature and Database Project: single ventricle.

The extant nomenclature for single ventricle (SV) hearts is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. Efforts were made to include all relevant nomenclature categories using synonyms where appropriate. Although many issues regarding single ventricle or univentricular hearts remain unresolved among anatomists and pathologists, a classification is proposed that is relevant to surgical therapy. A comprehensive database set is presented, which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail, which can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum data set is also presented that will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.