Baseline metabolic disturbances and the twenty-five years risk of incident cancer in a Mediterranean population.

BACKGROUND AND AIMS: Obesity is predictive of metabolic syndrome (metS), type 2 diabetes, cardiovascular (CV) disease and cancer. The aim of the study is to assess the risk of incident cancer connected to obesity and metS in a Mediterranean population characterized by a high prevalence of obesity. METHODS AND RESULTS: As many as 1133 subjects were enrolled in two phases and followed for 25 years (859 subjects) or 11 years (274 subjects) and incident cancer was registered in the follow-up period. Anthropometric measures and biochemical parameters were filed at baseline and evaluated as predictors of incident cancer by measuring hazards ratios (HR) using multivariate Cox parametric hazards models. Best predictive threshold for metabolic parameters and metS criteria were recalculated by ROC analysis. Fasting Blood Glucose >5.19 mmol/L [HR = 1.58 (1.0-2.4)] and the TG/HDL ratio (log10) (Males > 0.225, Females > 0.272) [HR = 2.44 (1.3-4.4)] resulted independent predictors of survival free of cancer with a clear additive effect together with age classes [45-65 years, HR = 2.47 (1.3-4.4), 65-75 years HR = 3.80 (2.0-7.1)] and male gender [HR = 2.07 (2.3-3.1)]. CONCLUSIONS: Metabolic disturbances are predictive of cancer in a 25 years follow-up of a Mediterranean population following a traditional Mediterranean diet. The high prevalence of obesity and metS and the observed underlying condition of insulin resistance expose this population to an increased risk of cardiovascular disease and cancer despite the healthy nutritional habits.

Fontan conversion to total cavopulmonary connection and arrhythmia ablation: clinical and functional results.

OBJECTIVES: To assess the early results of conversion from atriopulmonary to total cavopulmonary connection in patients with failing Fontan operation. DESIGN: Early clinical and instrumental evaluation of patients undergoing conversion from atriopulmonary to total cavopulmonary connection from April 1999 to November 2000. SETTING: Tertiary referral centre for congenital heart disease. PATIENTS: 11 Fontan patients (mean (SD) age 20.9 (6.7) years) with refractory arrhythmias or ventricular dysfunction. INTERVENTIONS: Total cavopulmonary connection, intraoperative ablation, and AAIR pacemaker implantation. MAIN OUTCOME MEASURES: Holter monitoring, transoesophageal atrial stimulation, ergometric test, and myocardial scintigraphy at a mean (SD) follow up of 16.8 (5.6) months. RESULTS: One early postoperative death occurred. During follow up three patients had relapse of atrial tachycardia, controlled by medical treatment, and two were pacemaker dependent. Transoesophageal stimulation did not induce atrial tachycardia in any patient. Ergometric test showed a diminished exercise tolerance in all but one patient. Mean minute ventilation and maximum oxygen consumption were 62% and 40% of their respective predicted values. Myocardial scintigraphy showed reversal of rest or exercise dysfunction in five patients and improved systemic ventricular function in seven. Mean basal ejection fraction increased from 39.4% (95% confidence interval (CI) 32% to 46%) to 46.5% (95% CI 41.7% to 51.2%) and ejection fraction on effort from 42.3% (95% CI 33.9% to 50.7%) to 50.2% (95% CI 44.5% to 55.9%). CONCLUSIONS: Our data show that total cavopulmonary connection associated with intraoperative ablation and pacemaker implantation allows for better control of arrhythmias and improves ventricular function in the majority of patients with failing Fontan.

Regression of pulmonary fistulas in congenital heart disease after redirection of hepatic venous flow to the lungs.

Although the etiology of pulmonary fistulas in congenital heart disease remains unknown, several hemodynamic factors are thought to play a role. Recently it was postulated that hepatic venous effluent might protect the pulmonary bed from development of pulmonary fistulas. However, there are no experimental data supporting this hypothesis. Here, we present the cases of 2 patients in whom pulmonary fistulas developed after unintentional surgical exclusion of the hepatic venous flow from the pulmonary circulation. In both patients, symptoms resolved after rerouting of the hepatic venous blood to the lungs.

Erythropoietin therapy and preoperative autologous blood donation in children undergoing open heart surgery.

We assessed the feasibility and efficacy of subcutaneous erythropoietin alpha (EPO) therapy and preoperative autologous blood donation (ABD) in children undergoing open heart surgery. Thirty-nine children were treated consecutively with EPO (100 U x kg(-1) s.c. three times a week in the 3 weeks preceding the operation and i.v. on the day of surgery) and two ABDs were made (Group 1). As controls to compare transfusion requirements, 39 consecutive age-matched patients who had undergone open heart surgery during the two preceding years were selected (Group 2). In a mean time of 20 (SD 5) days, 96% of scheduled ABDs were performed and only three mild vasovagal reactions were observed. The mean volume of autologous red blood cells (RBC) collected was 6 (1) ml x kg(-1) and the mean volume of autologous RBC produced as a result of EPO therapy before surgery was 7 (3) ml x kg(-1), corresponding to a 28 (11)% increase in circulating RBC volume. The mean volume of autologous RBC collected was not different from that produced [6 (1) vs 7 (3) ml x kg(-1), P=0.4]. Allogenic blood was administered to three out of 39 children in Group 1 (7.7%) and to 24 out of 39 (61.5%) in Group 2. Treatment with subcutaneous EPO increases the amount of autologous blood that can be collected and minimizes allogenic blood exposure in children undergoing open heart surgery.

Arterial switch operation: myocardial ischemia reversed by internal mammary artery graft.

We report a case in which an internal mammary artery graft to the left anterior descending coronary artery was successfully used in a newborn boy to reverse life-threatening myocardial ischemia 24 hours after arterial switch operation. One year later, angiography showed patency of both the graft and the transposed coronary ostium with normal left ventricular function. A brief review of the literature shows encouraging results of internal mammary artery grafts in pediatric patients.

Long-term results after atrial correction of complete transposition of the great arteries.

This study presents the late results for the first 104 consecutive patients surviving and atrial repair for transposition of the great arteries (TGA) between January 1971 and December 1978 (group 1). Mean follow-up was 12 years (range, 0.1 to 17.7 years). The actuarial survival rate at 18 years was 84.2% (70% confidence limits, 79% to 88%) for simple TGA and 93.7% (70% confidence limits, 84% to 97%) for complex TGA. Nine of the 11 deaths were sudden. Two (2.6%) of the 78 late survivors operated on for simple TGA are in New York Heart Association functional class III or IV versus 4 (26.7%) of the 15 survivors with complex TGA; the other patients are doing very well. To better assess long-term results, we report the findings for randomly obtained electrocardiograms, Holter monitor recordings, radionuclide angiographic studies, and cardiac catheterizations performed in 1987 in a larger group of 159 long-term survivors of atrial repair operated on at Ospedale Riuniti di Bergamo from January 1971 to December 1984 (group 2), which includes all of group 1. The findings confirm that the arterial switch repair is the procedure of choice for complex TGA and that there is a major incidence (approximately 10%) of systemic right ventricular dysfunction and rhythm disturbances after the atrial repair. On the other hand, our late survival rate at 18 years of 84% for simple TGA with 97.5% of the patients in functional class I is a result that should be kept in mind, especially in institutions where the arterial switch is a relatively new approach and presumably is a higher risk to cause early death.

Assisted circulation for myocardial recovery after repair of congenital heart disease.

Between January 1987 and May 1990, six children underwent ventricular assisted circulation for recovery of myocardial function after cardiac surgery. Their ages ranged from 9 months to 12 years. Three patients had tetralogy of Fallot and one had atrioventricular discordance with ventriculoarterial concordance, ventricular septal defect, and under-over ventricles. Double outlet left ventricle and a large ventricular septal defect with pulmonary hypertension was present in the remaining two. The duration of circulatory support averaged 126 +/- 57 h. An extracorporeal membrane oxygenator (ECMO) was used in four patients, biventricular assistance with centrifugal pumps in one, and left ventricular assistance followed by ECMO in the last patient. Circulatory support was begun in the operating room in two patients who could not be weaned from cardiopulmonary bypass. Both these patients are long-term survivors. Assisted circulation was implanted in four patients in the intensive care unit because of low cardiac output refractory to any pharmacological treatment. Only one of these patients could be weaned from circulatory support but he died 20 days later because of multiorgan failure due to persistent poor myocardial function. The causes of death in the remaining three patients were intracranial hemorrhage, untreatable bleeding, and failure of myocardial recovery, respectively. We believe that early postoperative use of circulatory support can be a major determinant for recovery of myocardial function in patients who have severe low cardiac output following repair of congenital cardiac lesions. Our initial experience with the use of biventricular assistance in small children is encouraging. Nevertheless, further observations are clearly necessary to establish the role of postoperative circulatory support in children with congenital cardiac lesions.

[Total anomalous pulmonary venous drainage: is a surgical correction possible on the sole basis of echocardiography?]. / Ritorno venoso polmonare anomalo totale; è possibile la correzione chirurgica sulla base della sola diagnosi ecocardiografica?

Between April '83 and August '89, 33 patients with total anomalous pulmonary venous drainage were studied at the department of Cardiology in Bergamo. There were 18 males and 15 females, aged between three days and 8 months (average: two months). In this study only cases of isolated total anomalous pulmonary venous drainage were taken into consideration. The patients underwent echocardiographic examination with ATL MK 600, Vingmed 700, ATL Ultramark 9 with 3.5; 5; 7.5; MHz transducers; in the last three years the echocardiographic examination was integrated by continuous and pulsed wave Doppler and, in the last year, by color Doppler. The morphologic diagnosis was routinely established by means of the sequential approach method. A common feature in all types of total anomalous pulmonary venous drainage was the impossibility of defining the connections of the pulmonary veins with the left atrium. Furthermore, patients had a volume overload of the right heart, and atrial septal defects of various sizes. The site of anomalous drainage of the pulmonary veins was assessed by means of multiple cuts from subcostal, precordial and suprasternal windows. The echocardiographic diagnosis was exact and complete in 29 cases (87.9%), and in four cases it was incomplete but basically correct (12.1%). The anatomical findings were confirmed during cardiac surgery in 32 cases and by autopsy in one case of supracardiac total anomalous pulmonary venous drainage in a critically ill patient, who died before surgery. Of the 32 patients who underwent surgical correction, 20 (62.5%) had only an echocardiographic diagnosis, which resulted correct in all cases. The majority of patients with isolated TAPVD can be confidently diagnosed by means of echocardiography, thus, avoiding preoperative catheterization.

Radionuclide evaluation of ventricular function at rest and during exercise in double inlet ventricle.

We studied ventricular volumes and ejection fraction by radionuclide angiography (equilibrium technique) in 15 patients (aged 3-48 years) with double inlet ventricle not yet submitted to corrective surgery. The end-diastolic volume (measured in nine cases) ranged from 108 to 219 ml/m2 (156 +/- 32), being lower than the normal theoretical value (right plus left ventricle) in six cases. Ejection fraction ranged from 30 to 77% (56.4 +/- 13). The value was significantly higher in the subgroup of 10 patients with a dominant left ventricle as compared to the five cases with dominant right or indeterminate ventricular morphology (63.2 +/- 8.3 versus 42.8 +/- 9, P less than 0.01). In seven of the 15 patients, measurements were obtained both at rest and during dynamic exercise in the semi-upright position. The end-diastolic and end-systolic volumes, stroke volume, ejection fraction underwent a slight non-significant reduction (from 158 +/- 29 to 147 +/- 24 ml/m2, from 58 +/- 16 to 56 +/- 24 ml/m2, from 100 +/- 27 to 90 +/- 24 ml/m2, from 64% +/- 9 to 61% +/- 13). During exercise, ventricular volumes mostly behaved as follows: slight reduction of end-systolic volume, decrease of end-diastolic volume, no increase (no change or decrease) of ejection fraction.(ABSTRACT TRUNCATED AT 250 WORDS)

Aortopulmonary window in the first year of life: report on 11 surgical cases.

From June, 1975, through April, 1986, 11 consecutive infants with aortopulmonary window were operated on at the Department of Cardiac Surgery, Ospedali Riuniti, Bergamo, Italy. The classic type of aortopulmonary window was present in 6 patients, whereas 4 patients had a ductus type of aortopulmonary window and 1 patient had a double aortopulmonary window, associated transposition of the great arteries, and ventricular septal defect. Operative technique included patch closure of the defect by means of cardiopulmonary bypass using various approaches: transaortic in 3, transpulmonary in 2, and transwindow in 2. The aortopulmonary window was ligated in 3 patients, in 2 of whom an incorrect diagnosis of patent ductus arteriosus was made at cardiac catheterization. Division and suture of the defect through a median sternotomy was performed in the remaining patient, who was moribund on admission. There was a single operative death. All survivors are in New York Heart Association Functional Class I at a mean interval of 6 1/2 years postoperatively.

The modified Fontan operation for double inlet left ventricle. Surgical results, early haemodynamic and functional assessment.

Twenty-one patients with a double inlet left ventricle underwent a modified Fontan between May 1979 and August 1987. All but the first patient in this series were operated upon after August 1984. Their age at operation ranged from 4 to 23 years with a median age of 8 years. Initial palliation was required in 12 patients and the mean interval to repair was 77.1 months, ranging from 38 to 112. Only one of the criteria of Choussat was exceeded in 12 patients. The pulmonary vascular resistance was always less than 4 Um2. A direct atriopulmonary anastomosis was performed in 19 patients and an aortic homograft interposition was used in 2. There was one hospital death due to acute cardiac failure and no late deaths. The average of the mean right and left atrial pressures measured early postoperatively was 12.9 and 8 mmHg respectively. A prolonged hospitalization with a mean of 23 days, ranging from 9 to 69, was required because of recurrent pleural and pericardial effusions which usually resolved after the first 6 postoperative months. The need for early anticoagulation is recommended to prevent the risk of pulmonary thromboembolism observed in 2 of our patients. Cardiac catheterization performed in 19 patients within 1 year after surgery showed that the average of the mean right atrial pressure had decreased to 11.3 mmHg without a gradient across the anastomosis in any case. Two patients required reoperation: one for enlargement of a restrictive ventricular septal defect and the other for closure of a recurrent dehiscence of the patch used for closure of the right a-v valve. Three patients developed a significant subaortic obstruction and have been scheduled for reoperation. Ventricular function was assessed at a mean interval of 11.9 months (17 patients) and 25.5 months (13 patients) from surgery. Gated equilibrium radionuclide ventriculography showed that the ejection fraction was normal (greater than or equal to 50%) and it had increased with time in 10. Yet, the response to exercise was normal in only 5 of the 13 patients re-evaluated. Treadmill exercise testing showed an increase in exercise tolerance from a mean of 7.4 min to a mean of 8.7 min.(ABSTRACT TRUNCATED AT 400 WORDS)

Cross-sectional echocardiographic study of criss-cross hearts and superoinferior ventricles.

From August 1982 to October 1985, 8 consecutive patients, aged 4 months to 8 years (mean 2.6), with criss-cross or superoinferior ventricles, or both, were studied. Seven patients had usual atrial arrangement and 1 had mirror-image arrangement. In 5 patients there was atrioventricular (AV) concordance with crossed AV streams, the ventricular septum was horizontal in 4 and sagittal in 1, and the ventriculoarterial connections were discordant in all. The 3 patients with AV discordance had superoinferior ventricles, but a criss-cross AV relation was not present; the ventriculoarterial connections were concordant in 2 and double-outlet right ventricle in 1. The sequential connections and spatial relations of the cardiac chambers, as well as the associated anomalies, were accurately assessed by echocardiography. The diagnosis was confirmed by angiography in all patients, by surgery in 3 and by autopsy in 2.

Reduction of infection after cardiac surgery: a clinical trial.

Between January 1, 1975, and June 1, 1984, 3,275 patients underwent cardiac operations with cardiopulmonary bypass. No operations were performed in 1978. In Phase I of the study, general operating rooms were used for cardiac operations, and standard methods of antisepsis and asepsis were used. Phase II began in January, 1979, with the opening of two specially constructed operating rooms with complete separation of incoming and outgoing personnel and supplies, and with a laminar airflow system. All personnel scrubbed 3 minutes and changed into autoclaved clothing before entering the operating suite, and scrubbed again for 5 minutes before putting on gowns. By Phase III, which began in July, 1982, all additional protocols against infection were in place including strict techniques in the intensive care unit and a continuous antiinfection surveillance program. In Phase I, 7.3% (70% confidence limits [CL] 6.4 to 8.2%) of patients had an infectious complication; in Phase II, 2.7% (CL 2.3 to 3.2%), and in Phase III, 0.8% (CL 0.5 to 1.2%). The reductions were similar in the four subtypes of infection (superficial presternal infection, mediastinitis, endocarditis, and septicemia). The study indicates that improving the surgical environment, improving the surgical and operating room protocols, and increasing the awareness of the dangers of infection among the personnel can strikingly reduce the incidence of infections after cardiac operations.

Surgical treatment of subpulmonary obstruction in transposition of the great arteries by means of a left ventricular-pulmonary arterial conduit. Late results and further considerations.

Between January, 1976, and June, 1983, 16 patients with transposition of the great arteries and fixed subpulmonary obstruction underwent repair by means of a combined Mustard procedure and placement of a conduit between the left ventricle and main pulmonary artery. Their mean age and weight were 5.3 years and 19 kg. Ten patients had an intact ventricular septum and six had a ventricular septal defect, which because of its size or location precluded a Rastelli repair. A fibromuscular tunnel was the most common type of subpulmonary obstruction (10/16, 62%). There were three early deaths and one late death. Early cardiac catheterization (mean interval from operation, 45 days), performed in 10 of 13 survivors, showed a significant decrease in the mean left ventricular-pulmonary arterial gradient from 66 to 8.5 mm Hg. Late cardiac catheterization (mean interval from operation, 4 years) showed continuing relief of the subpulmonary obstruction with a mean increase in peak systolic transconduit pressure gradient of 6.4 mm Hg. This procedure is an option to direct relief in the management of severe fixed subpulmonary obstruction in patients with transposition and intact ventricular septum or in the presence of a ventricular septal defect when a Rastelli repair is precluded.

Absent pulmonary valve syndrome with associated anomalies of the pulmonary blood supply.

We report four cases of so-called absent pulmonary valve syndrome associated with absence or anomalous origin of the left pulmonary artery. The fate of the patients with this condition appears to be affected mainly by the occurrence of pulmonary complications (three of our patients died of pulmonary causes, two of them after surgery). The proper timing of corrective surgery is still uncertain. Preoperative investigations should strive to obtain a clear-cut identification of the pulmonary arteries, particularly the left one or, in its absence, of the anomalous vascular supply to the lung. Right ventriculography, as employed by us, is insufficient. Pulmonary angiography, aortography and pulmonary vein "wedge" angiography may be needed.

[Surgical treatment of the tetralogy of Fallot with a single pulmonary artery]. / Trattamento chirurgico della Tetralogia di Fallot con una sola arteria polmonare.

Eleven patients with Tetralogy of Fallot and congenital or acquired absence of one pulmonary artery (PA) have been operated upon at the Department of Cardiac Surgery of Ospedali Riuniti-Bergamo-Italy. Eight children had agenesis of the left PA, one of the right PA and two had complete occlusion of the right PA after a palliative operation. Four patients had only a palliative procedure (one Blalock-Taussig, two Waterston, three PTFE shunts) with one operative death. Two patients underwent a two-staged procedure, that is an initial shunt (one Blalock and one PTFE shunt) followed by subsequent total correction. Five patients underwent primary total correction. No operative death occurred among the patients who had total correction. Right ventricular outflow reconstruction has been accomplished with an infundibular patch in one patient, an external valved conduit in two patients, and a transanular patch plus orthotopic bioprosthesis in 4 patients. One patient with a valved conduit died one year postoperatively in right heart failure caused by pulmonary hypertension. The pulmonary vascular disease was probably due to pulmonary microembolization following the severe hemolysis that lasted for twenty days in the early postoperative period. The nine surgical survivors are in good general condition with maximum follow-up of 7 years. The surgical approach either in case of palliation or in case of corrective surgery is finally discussed. In our experience TOF with unilateral absence of a PA is a congenital anomaly that can be corrected with results comparable to those of uncomplicated TOF.

Waterston anastomosis for initial palliation of tetralogy of Fallot.

Two hundred twenty-seven patients (median age 5.4 months) in whom a Waterston anastomosis was done for initial palliation of tetralogy of Fallot between 1966 and 1979 were studied. Twelve patients died in the hospital (5.3%; 70% confidence limits, 3.8% to 7.3%). Young age, low weight, and poor clinical condition did not appear to be incremental risk factors, whereas a too large or a too small shunt was largely responsible for the hospital mortality and morbidity. Follow-up information was available in all the 215 patients discharged from the hospital. At the last follow-up visit, before any further surgical procedure, 74% of the patients were clinically in good condition. By actuarial methods, 97.7% of hospital survivors were alive and 95.8% were event-free at and beyond 3 years postoperatively. Eighty-six patients have been catheterized in preparation for secondary repair (mean interval between Waterston shunt and catheterization, 2.9 +/- 1.38 years). One patient developed pulmonary vascular disease, four acquired pulmonary atresia, and 14 had a severe kinking of the right pulmonary artery at the site of the anastomosis.

Surgical repair of persistent truncus arteriosus in infancy.

Fourteen patients younger than two years of age with persistent truncus arteriosus underwent primary repair. Twelve of them were less than 1 year and 4 less than 3 months of age. Intractable heart failure was the indication for surgery in all patients but one who had increased pulmonary vascular resistance. There were 5 hospital and 2 late deaths. Six out of the 7 survivors (median follow-up: 29 months) were symptom-free. The remaining infant who preoperatively had significant truncal valve regurgitation was doing fairly well 2 1/2 years after repair. Our experience suggests that, although the mortality remains high, primary repair for infants with persistent truncus arteriosus is feasible and offers better overall results than does pulmonary artery banding followed by later intracardiac repair. We advise primary repair for all infants with intractable heart failure or increasing pulmonary vascular resistance with or without truncal valve regurgitation. Elective repair is recommended before the age of 2 years to minimize the risk of pulmonary vascular disease.

Complete transposition of the great arteries with intact ventricular septum and left ventricular outflow tract obstruction. Surgical management and anatomic considerations.

Surgical management of patients with complete transposition and intact ventricular septum may become difficult in the presence of left ventricular outflow tract obstruction. A Mustard operation and direct resection of the obstruction through the pulmonary artery has been the treatment of choice for this combination. Our study of the structure of the left ventricular outflow tract in four specimens with the anatomic findings of complete transposition, intact ventricular septum, and subpulmonary stenosis suggests that direct resection of the stenosis through the pulmonary artery can seldom be adequate without major risk of damaging either the mitral valve or the conduction tissue. An alternative procedure, namely, a combined Mustard operation and insertion of an external conduit from the left ventricle to the main pulmonary artery, has been employed in the management of six patients with this combination of lesions. One early and one late death occurred. Postoperative cardiac catheterization performed in all of the survivors before discharge from the hospital showed good relief of the stenosis and no significant gradient across the conduit.