Poul Lauridsen

Twenty-to-thirty-seven-year follow-up after repair for Tetralogy of Fallot.

OBJECTIVE To describe the long-term prognosis after repair of Tetralogy of Fallot with pulmonary stenosis beyond 20 years. METHODS One hundred and eighty five patients underwent corrective repair of Tetralogy of Fallot at Rigshospitalet in Copenhagen between January 1960 and July 1977. Ninety seven patients had undergone a palliative operation prior to Tetralogy of Fallot repair. All the 125 patients who were discharged from the hospital were traced through the population register and the patients alive July 1997 were contacted by mail and/or telephone and questioned about use of medicine, professional status, family status and ability to perform sport activities. RESULTS Sixty patients died in hospital and 125 patients, 78 males and 47 females, were discharged alive. Among operative survivors, median age at operation was 12.8 years (range 0.4-41 years). Thirteen patients required a reoperation, the main indication was failed VSD closure. There were 16 late cardiac deaths, out of which seven were sudden and unexpected and three were in immediate relation to reoperations. One hundred and nine patients were alive at follow-up. The mean follow-up time was 25.5 years (range 20-38 years). Sixteen percent used cardiac drugs, 89% were, or had been, working normally (all professions from academics to hard manual labors were represented), 53% (64% of women) had given birth after the repair and 51% performed sport activities regularly. No patients were lost to follow-up. CONCLUSIONS The vast majority of the patients seemed to live normal lives 20-37 years after Tetralogy of Fallot repair. Late deaths were cardiac in origin, including sudden death from arrhythmias. The number of late reoperation has been low. Considering the natural history of the disease, Fallot repair has proven to be a beneficial procedure even including the very early experience short after introduction of open heart surgery.

Long-term follow-up of right ventricular function after mustard operation for transposition of the great arteries

As development of right ventricular (RV) failure is a potential risk after Mustard operation for transposition of the great arteries, 17 patients were reexamined 5-13 years postoperatively. Comparisons were made with healthy controls. There were no clinical signs of heart failure. Echocardiographically determined RV end-diastolic diameter was increased to 2.5 +/- 0.8 cm (controls: 1.5 +/- 0.4 cm, p less than 0.001). Comparison of RV systolic time intervals (STI) in patients with normal left ventricular (LV) STI revealed decreased RV function, with RPEPI 165 +/- 19 msec (controls 126 +/- 12, p less than 0.001) and RPEP/RVET 0.484 +/- 0.096 (controls 0.284 +/- 0.045, p less than 0.001). Nuclear angiography demonstrated decreased RV ejection fraction (EF), viz. 42.8 +/- 6.6% (normal RV 53 +/- 6%, LV 68 +/- 9%, p less than 0.001). Only two patients showed normal (5%) rise in RV-EF during exercise. There was no evidence of deterioration with passage of time. The results do not justify use of anatomic repair at our center, since the perioperative mortality might then be higher than in the Mustard or Senning procedures.

Natural History of Vascular Ring with Clinical Manifestations: A Follow-Up Study of Eleven Unoperated Cases

A clinical follow-up study was performed in 11 unoperated patients with symptoms due to vascular ring. The age at diagnosis was 2-48 months (mean 13 months); 7 of the patients were less than 12 months old. The follow-up time varied between 1 and 22 years (median 7 years). Nine patients were entirely free of symptoms, which they had outgrown in the course of 1-2 years after the diagnosis was made; none of these 9 had symptoms after the age of 4 years. Two patients had symptoms due to either oesophageal compression or to severe associated neuromuscular lesions; the follow-up time was 3 and 1 years, respectively. Our findings lead us to support a conservative attitude regarding surgery, at least if only mild symptoms are present, If the patients can be satisfactorily treated medically, they seem to outgrow their symptoms in early childhood.

Is the arterial switch operation still a challenge in small centers

OBJECTIVE In the last years, major changes as regards timing for operation, surgical technique, and perioperative care determined a great improvement in the arterial switch operation (ASO) allowing excellent mid-term results in a few leading centers. This stimulated the widespread adoption of ASO as procedure of choice for transposition of the great arteries (TGA), even in small institutions. We reviewed our early experience with ASO in an attempt to evaluate its safety in a small center. METHODS Since April 1992, 39 consecutive patients underwent TGA repair by ASO in our department. There were 27 patients with simple TGA, 8 with TGA and VSD and 4 with Taussig-Bing heart and aortic coarctation. Median age and weight at operation were 7 days and 3.5 kg, respectively. Neonatal repair was performed in 34 patients. In accordance with the Planché coronary classification, type I was encountered in 21 patients, type II in 4 and type III in 14. Several modifications of the original technique were used, mainly regarding coronary relocation, pulmonary artery reconstruction and approaches for associated VSD closure and aortic arch repair. RESULTS Early mortality was 2.6% (n = 1), the only operative death being related to unsatisfactory coronary relocation. Since modified ultrafiltration was adopted, mean ICU stay decreased from 5 +/- 4 days (n = 21) to 2 +/- 1 days (n = 17) (P < 0.05). Three patients required reoperation for residual ASD and/or VSD closure. There were no late deaths. After a mean follow-up of 26 +/- 15 months all survivors are thriving and are currently asymptomatic. CONCLUSIONS Although this series is rather small, most of the major coronary anomalies and complex anatomic associations were encountered. This experience suggests that neonatal repair of TGA by ASO can be safely accomplished even in small centers. Modified ultrafiltration appears to improve the early outcome of neonates undergoing ASO.

Fourteen- to nineteen-year follow-up after corrective repair for tetralogy of Fallot.

A long-term follow-up was made of the first 28 patients who survived corrective repair for tetralogy of Fallot at the Department of Thoracic Surgery R, Rigshospitalet. Copenhagen. They were discharged in the years 1960–1964. There were 20 males and 8 females. The age at operation was 6–41 years (median 12 years). Nineteen patients had previous palliative operations. All of them had one or more postoperative clinical examinations at the hospital and 18 had postoperative heart catheterizations. Six patients had died, five within 10 years after operation (21.4%: 95% confidence limits 8.3–41.0%). Four late deaths were clearly related to residual cardiovascular abnormalities, insufficient VSD closure being the main problem. One further death was due to a malignant arrhythmia provoked at the routine postoperative heart catheterization. Two deaths were sudden, one in a patient with a ruptured VSD. The only patient with transient a-v block postoperatively, who later died, did so from pump failure after the second...

Systemic Arterial Embolism After Mitral Valvulotomy

During an average period of follow-up of 6 years, 255 patients subjected to closed mitral valvulotomy had an incidence of late arterial emboli of 1.0% per patient-year. In an unoperated series of 271 patients with mitral stenosis the incidence was 3.7% per patient-year. The significant protective value of mitral valvulotomy against late systemic embolism was particularly evident in patients with pre-operative atrial fibrillation. In subjects with pre-operative arterial emboli the rate of late embolism was 3.0% per patient-year, and it is suggested that long-term anticoagulant therapy is considered in this group of patients.

Double-homograft Repair of Truncus Arteriosus with Severe Truncal Valve Dysfunction

An infant with truncus arteriosus and severe dysfunction of the truncal valve including both stenosis and insufficiency successfully underwent primary repair. This included the insertion of two separate valved homograft conduits. Early outcome has been excellent and the patient is doing well after 6 months with only echocardiographic evidence of mild aortic valve regurgitation. Double-homograft repair is a realistic option in cases of truncus arteriosus with severe malformation of the truncal valve.

Pre- and Postoperative Clinical and Haemodynamic Results after Prosthetic Replacement of Cardiac Valves

A clinical and haemodynamic follow-up study was performed on 27 patients after cardiac operations with insertion of prosthetic mitral or mitral and aortic valves. The functional capacity improved postoperatively in 25 patients. The pulmonary wedge position and pulmonary artery pressures at rest were significantly reduced postoperatively, just as the pulmonary artery pressure during exercise was significantly lower at the follow-up. The postoperative heart size was also smaller, whereas cardiac index and pulmonary vascular resistance did not show any significant changes. Only a few of the patients obtained complete normalization of the haemodynamic variables. Possible reasons for this result are discussed.

Early Experience with the Norwood Procedure

The improved results of the Norwood procedure have recently stimulated its widespread adoption in many centres. Since 1993, 19 infants with hypoplastic left heart syndrome or similar conditions underwent a first-stage Norwood procedure. Circulatory arrest time was significantly reduced by using a modified repair of the aortic arch. The early mortality rate was 31.5% (n = 6). The addition of CO2 to the inspired gas mixture resulted in less early postoperative instability. Nine patients have subsequently undergone bidirectional cavopulmonary shunt and one fenestrated total cavopulmonary connection. Overall there have been five late deaths, two as result of failure of cavopulmonary operations. All the eight survivors are presently in good condition. One is awaiting bidirectional cavopulmonary shunt and the other seven total cavopulmonary connection. This early experience encourages the continued offering of the Norwood procedure to patients with hypoplastic left heart syndrome or its variants. Increasing experience with the perioperative care and a more careful evaluation before cavopulmonary operations may determine further improvement in the outcome.

A STUDY OF POSTOPERATIVE RESIDUAL INTRACARDIAC SHUNTS WITH SERIAL DYE-DILUTION RECORDINGS

Thirty-four patients operated on for ASD were examined per- and postoperatively, and a residual shunt was demonstrated in 13 cases. Likewise 15 patients who had radical correction of Steno-Fallots Tetralogy were examined: 7 were found to have residual shunts. Two of these patients died shortly after operation. The 18 patients surviving with residual intracardiac shunts were re-examined 18 months to 4 years after the operation. The applied dye-dilution technique is described. All veno-arterial shunts, up to 30%, had closed spontaneously or decreased to become haemodynamically insignificant, but 1 patient developed an arterio-venous shunt of 50% due to spontaneous postoperative rupture of the defect. Of arterio-venous residual shunts, 6 out of 9 remained unaltered or enlarged after operation for ASD. In tetralogy of Steno-Fallot three residual left-to-right shunts disappeared, one diminished and one remained unchanged. Where a patch is used for closure of the defect no residual shunt should be tolerated. C...