Thrombotic and hemorrhagic complications in patients with mechanical heart valve prosthesis attending an anticoagulation clinic.

This study evaluated the advantage of an anticoagulation clinic in terms of the improvement of the clinical quality of oral anticoagulation (i.e. prevention of thromboembolism and low rate of hemorrhagic complications). The incidence of thromboembolic events and major hemorrhagic complications was assessed in a series of 271 patients on oral anticoagulation for mechanical heart valve prosthesis before and after their enrollment in our anticoagulation clinic from January 1987 to December 1990. Risk factors for hemostatic events were also analyzed. The incidence of major hemostatic complications was significantly lower when patients attended the clinic: 1.0 vs 4.9%/pt-yr for hemorrhage and 0.6 vs 6.6%/pt-yr for thrombosis. This depended on three main factors: better dose regulation of warfarin, continuous patient education and early identification of clinical conditions potentially at risk for thrombosis and hemorrhage. Only previous hemorrhagic or thromboembolic events were recognized as major risk factors for hemostatic complications. In conclusion, our study shows that an anticoagulation clinic offers a real advantage to patients with mechanical heart valve prosthesis in terms of prevention of thromboembolic events and hemorrhagic complications.

Growth of the pulmonary anulus and pulmonary arteries after the Waterston anastomosis.

Twenty-six patients with tetralogy of Fallot underwent angiocardiography before a Waterston shunt and again several years later, prior to complete repair (mean interval 40 +/- 15.6 [SD] months). The ratio between the diameter of the pulmonary valve anulus (PVA) and that of the descending thoracic aorta (Ao) increased by 0.32 +/- 0.233, significantly more than is reported to occur after a Blalock-Taussig anastomosis (p = 0.0001). This increase had some relation to the interval between the two angiographic studies (r = 0.34, p = 0.08) and to the mean pressure in the main pulmonary artery (MPA) at the time of the second study (r = 0.35, p = 0.08). The ratio of diameter of the right (RPA) and left pulmonary arteries (LPA) and that of the Ao also increased by 0.24 +/- 0.226 and 0.20 +/- 0.201, respectively. These data suggest that an initial large aortopulmonary shunt may result in less need for transannular patching at complete repair.

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.

Repair of tetralogy of Fallot after Waterston anastomosis.

Sixty-three corrective operations have been performed in patients with Tetralogy of Fallot and a previously constructed Waterston shunt. The patients were from 1.4 to 8 years of age (median 4.3 years), and the mean interval between the Waterston shunt and the repair was 39.3 +/- 16.05 months. Three patients developed pulmonary atresia and six required a second anastomosis prior to intracardiac repair. Kinking and stenosis of the right pulmonary artery at the site of the Waterston anastomosis occurred in 12 (19 percent; 70 percent confidence limits 14 to 26 percent) patients who required patch enlargement of the right pulmonary artery at repair. Seven (11 percent; 70 percent confidence limits 7 to 17 percent) patients died in the hospital and three (5 percent; 70 percent confidence limits 2 to 11 percent) during the period of late follow-up. None of the early or late deaths was specifically related to the presence of the previously performed Waterston anastomosis.

The Senning operation for transposition of the great arteries.

The original Senning technique of interatrial transposition of venous return was selectively employed for physiological correction in 24 patients with transposition of the great arteries (TGA) ranging in age from 74 days to 26 months (median 7 months). Twenty-three had intact ventricular septum and one had a large ventricular septal defect and diaphragmatic subpulmonary stenosis. There were no hospital or late deaths, and at follow-up examination 1.5 to 12 months postoperatively, each patient was asymptomatic, in normal sinus rhythm, and had no clinical evidence of caval or pulmonary venous obstruction. Nine patients underwent cardiac catheterization and angiocardiographic studies between 1.3 and 9 months postoperatively which demonstrated widely patent venous pathways and effective "left" and "right" atrial contraction. This experience lends support to the continued use of the Senning technique for interatrial transposition of venous return.

Cor triatriatum. Clinical presentation and operative results.

From November 1973 to January 1988, 15 patients with cor triatriatum underwent surgical correction at the Department of Cardiac Surgery, Ospedali Riuniti, Bergamo, Italy. Their ages ranged from 15 days to 48 years. Eight patients (53%) were younger than age 1 year. Evidence of congestive heart failure was present in five infants, whereas three infants, three children, and two adults initially had signs of pulmonary venous obstruction, and two children had a heart murmur only. Five patients had cor triatriatum alone; in ten cases there also was an atrial septal defect. Associated anomalies in four patients included left superior vena cava, ventricular septal defect and left superior vena cava, partial anomalous pulmonary venous connection, and bilateral partial anomalous pulmonary venous connection. Cor triatriatum was repaired with the aid of cardiopulmonary bypass in all patients. Excision of the membrane was accomplished with a right atrial approach in 13 patients and a left atriotomy in one patient. Both atria were opened in one case. All associated anomalies were simultaneously corrected. Three patients (20%) died early after operation. Among the 12 survivors, no late events have occurred, and all of them are presently in New York Heart Association functional class I. A recent echocardiogram shows absence of residual obstruction or shunt and good development of the left cardiac chambers.

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.

Congenital malformations of the mitral valve in children. Pathology and surgical treatment.

A systematic study of congenital mitral valve malformations was undertaken on a surgical basis in an attempt to develop techniques of valvular reconstruction adapted to the various lesions. Forty-seven children between the ages of 4 months and 12 years (average 6 years, 4 months) have been operated upon between January, 1970, and March, 1976. Valvular lesions were classified into four group: Group I, mitral insufficiency owing to valvular lesions: Group II mitral insufficiency with subvalvular lesions; Group III, mitral insufficiency and stenosis; Group IV, stenosis. Associated lesions (ventricular or atrial septal defects, coarctation, or aortic valve stenosis) were present in 31 patients (65 per cent) and were corrected during the same operation. Valve reconsruction was possible in 38 patients whereas valve replacement was necessary in 9 patients. In the valve repair group there were three operative deaths (8 per cent), no late deaths, one reoperation for residual ventricular septal defect, and one myocardial infarction. In the valve replacement group of 9 patients, there were three operative deaths, three late deaths, and one case of repeated embolization. Thirty-one of 38 patients in the valve repair group were classified into Functional Class I after the operation (86 per cent), 2 were in Class II, and one in Class III. Minimal or moderate regurgitation and cardiomegaly persisted in the majority of the patients. Pulmonary artery pressure significantly decreased, however, as demonstrated by postoperative catheterization in 17 patients.

Pediatric heart transplantation without chronic maintenance steroids.

From 1986 to February 1993, 40 children aged 2 months to 18 years (average age 10.4 +/- 5.8 years) underwent heart transplantation. Indications for transplantation were idiopathic cardiomyopathy (52%), congenital heart disease (35%) with and without prior repair (71% and 29%, respectively), hypertrophic cardiomyopathy (5%), valvular heart disease (3%), and doxorubicin cardiomyopathy (5%). Patients were managed with cyclosporine and azathioprine. No prophylaxis with antilymphocyte globulin was used. Steroids were given to 39% of patients for refractory rejection, but weaning was always attempted and generally successful (64%). Five patients (14%) received maintenance steroids. Four patients died in the perioperative period and one died 4 months later. There have been no deaths related to rejection or infection. Average follow-up was 36 +/- 19 months (range 1 to 65 months). Cumulative survival is 88% at 5 years. In patients less than 7 years of age, rejection was monitored noninvasively. In the first postoperative month, 89% of patients were treated for rejection. Freedom from serious infections was 83% at 1 month and 65% at 1 year. Cytomegalovirus infections were treated successfully with ganciclovir in 11 patients. No impairment of growth was observed in children who underwent transplantation compared with a control population. Twenty-one patients (60%) have undergone annual catheterizations and no sign of graft atherosclerosis has been observed. Seizures occurred in five patients (14%) and hypertension was treated in 10 patients (28%). No patient was disabled and no lymphoproliferative disorder was observed.(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.

Fontan repair for tricuspid atresia: experience with 50 consecutive patients.

From April, 1975, to October, 1985, 50 patients (mean age, 5.3 years; range, 2.5 to 14 years) underwent a Fontan repair for tricuspid atresia or severe tricuspid stenosis. Fifty-six palliative operations had been performed previously in 43 patients. Twenty-nine patients received an atriopulmonary connection by direct anastomosis [19], interposition of an aortic homograft [7], or a porcine heterograft [3]. An atrium-subpulmonary chamber connection was performed in 21 patients (Björk modification, 10; Dacron tube, 10; valved conduit, 1). Eleven patients (22%) died early, 6 of whom did not meet the established criteria for a Fontan operation. One patient died 6 months postoperatively of superior vena cava thrombosis. Four patients were reoperated on successfully. At a mean follow-up of 28.8 months, 28 patients are in New York Heart Association Functional Class I and 9 are in Class II; 1 patient was lost to follow-up. Thirteen patients underwent control catheterization fifteen days to four years postoperatively (median right atrial pressure, 14 mm Hg). Exercise testing in 9 asymptomatic patients, performed after a mean interval of 33 months, showed a moderate impairment of cardiac performance. We conclude that in select patients, a Fontan repair appears to be a reliable procedure at medium-term follow-up.

Late left atrioventricular valve insufficiency after repair of partial atrioventricular septal defects: anatomical and surgical determinants.

Risk factors for late left atrioventricular (AV) valve insufficiency, which occurred in 16 (18%) of 90 patients evaluated after repair of partial AV septal defect, were examined. The operative findings in 9 patients undergoing reoperation were also examined. Preoperative left AV valve insufficiency was significantly more common in the group with late left AV valve incompetence, as were associated valvular malformations as a whole and fenestrations of valve leaflets in particular. Conversely, the higher incidence of malformed or malpositioned papillary muscles, accessory clefts, and double-orifice left AV valves in the group with late left AV valve insufficiency did not reach significance. The method of surgical treatment of the septal commissure was not a significant factor. In the group having reoperation, additional valvular malformations were found in association with inappropriate treatment of the septal commissure in 7 patients. The 2 remaining patients had either a directly sutured ostium primum or dilatation of the annulus. Three re-repairs were successful. Five patients required prosthetic valve replacement. Preoperative left AV valve insufficiency and associated valvular malformations are major determinants of late left AV valve insufficiency in partial AV septal defect.

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.

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.

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.

Left-side approach to the mitral valve.

BACKGROUND AND AIM OF THE STUDY: The standard, minimally invasive surgical approach to the mitral valve is usually accomplished from the right side, through a longitudinal incision posterior and parallel to the interatrial groove. The left-side approach should perhaps be reconsidered for its suitable exposure of the mitral valve. METHODS: Eleven consecutive patients underwent mitral valve surgery via a left lateral minithoracotomy, with cardiopulmonary bypass performed through the left femoral artery and vein. The left internal mammary artery was used as a coronary graft in one patient. Cardioplegic solution was delivered through the ascending aorta. After direct aorta cross-clamping, mitral surgeries were performed, including mechanical and bioprosthetic implants, redo operations, annuloplasties and repairs. RESULTS: An excellent view of the mitral valve was obtained in all cases, with optimal vision of the whole annulus, chordae tendineae, papillary muscles and aortic valve possible. Exposure of the mitral valve was superior to that achieved with the standard approach from the right, notably with a wider angle of vision and greater flexibility of movement available. Moreover, mitral valve structures were closer to the operator, making the procedure easier to perform. CONCLUSION: The left-side approach to the mitral valve offers excellent vision and exposure of the valve. Although awaiting additional cases to validate results, in the absence of major complications we recommend this technique.

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)

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.

Open heart surgery in the first six months of life.

Intracardiac repair of a variety of cardiac anomalies was performed in 104 infants aged 10 days to 6 mo, presenting with severe hypoxia and/or intractable heart failure. Thirty-eight patients (33 less than three mo of age) did not survive the operation or died during the first postoperative month. Above 6 mo of age, correction of heart defects is often carried out on an elective basis, and results are more favorable. A more convenient choice between corrective and palliative procedures is suggested to achieve better results in the difficult group of patients who require surgery within the first 6 mo of life.

One stage correction of coarctation of the aorta associated with intracardiac defects in infancy.

A two stage surgical approach is usually prepared for symptomatic babies affected by coarctation of aorta associated with intracardiac defects, the preliminary operation being the resection of the coarctation. One stage correction has been attempted in six patients, ranging from 2 to 24 m. of age and from 3,2 to 9,7 Kg. of weight. The associated lesions were a large V.S.D. (3 patients), a V.S.D. with pulmonary stenosis, a mitral stenosis and a mitral insufficiency. The coarctation was approached through a left thoracotomy and the intracardiac defect through a midline sternotomy incision, after the patient was turned around. All patients survived and did well following the double procedure. The two-stage surgical approach is unnecessary and probably unadvisable for patients with coarctation of the aorta and associated intracardiac lesions.