Implantable cardioverter defibrillator in a 4-month-old infant with cardiac arrest associated with a vascular heart tumor.

A previously healthy male infant was resuscitated after spontaneous ventricular fibrillation at 9 weeks of age. Echocardiography revealed three tumors in the left ventricle not amenable to complete resection. Despite treatment with antiarrhythmic agents the ventricular arrhythmias continued. When the child was 4 months old and weighed 7 kg an ICD system was implanted using epicardial sense-pacing leads and a superior vena caval lead as a subcutaneous defibrillator coil placed posterior on the left thorax. Shocks were delivered between the subcutaneous coil lead and the intraabdominally placed ICD can. This ICD array system has not been reported previously.

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.

Ventriculo-arterial discordance: switching the morphologically left ventricle into the systemic circulation after 3 months of age.

OBJECTIVE: To retrospectively examine a 4 year policy of restoring the morphologically left ventricle to the systemic circuit in patients presenting after 3 months of age with ventriculo-arterial discordance with or without associated atrio-ventricular discordance. This policy was stimulated by the known tendency of the morphologically right ventricle to develop dysfunction sooner or later when left in the systemic circuit. Such a policy dictates a more complex surgical approach and, at this point, it remains controversial whether or not the increased surgical complexity is warranted. METHODS: From July 1, 1993 to March 31, 1997, a total of 29 patients were entered into a protocol for placement of the morphologically left ventricle into the systemic circuit. Three groups of patients were identified. Group I; congenitally corrected transposition in 14 patients -- were treated with either a Senning plus arterial switch operation or Senning plus Rastelli procedure. Group II; failed atrial switch procedure in 12 patients of which nine proceeded to arterial switch operation with Senning or Mustard takedown and atrial reseptation. Group III; D-transposition of the great vessels presenting more than 1 year after birth in three patients who underwent arterial switch operation alone. A deconditioned morphologically left ventricle required reconditioning by means of preparatory pulmonary artery banding in 17 of 29 patients. In the patients requiring pulmonary artery banding, an average of 2.1 pulmonary artery bandings was required to prepare the morphologically left ventricle for a systemic pressure workload. RESULTS: In those patients with a deconditioned morphologically left ventricle requiring preparatory pulmonary artery banding, the mean ratio between the left ventricular and right ventricular systolic pressure increased from 0.48 to 0.95. The left ventricular mass increased from 46.6 to 81.8 g/m2 in five patients subjected to serial MRI measurement. Three patients failed the preparatory pulmonary artery banding and did not proceed to anatomical correction. Two subsequently died at a later time. In the patients proceeding to complete anatomical correction: group I -- there were no early or late deaths. Two patients required pacemaker implantation post-operatively. Group II -- there were two in-hospital deaths, one early due to intrapulmonary hemorrhage and one late, secondary to postoperative left ventricular failure with a stormy post-operative course requiring successful ECMO placement and weaning. These patients were 18 and 25 years old, respectively. One patient proceeded to cardiac transplantation 3 months after surgery due to ongoing morphologically left and right ventricular dysfunction. Group III -- all patients continue to do well. CONCLUSIONS: Late anatomic correction of ventriculo-arterial discordance with or without atrio-ventricular discordance can be performed at a relatively low risk. Reconditioning of the morphologically left ventricle can be achieved by sequential pulmonary banding but is not without risk. Failure to achieve adequate reconditioning of the morphologically left ventricle by pulmonary artery banding in the older patient probably increases the risk of non-survival and may be offset by timely transplantation. Longer follow-up and an assessment of the functional status of these patients is required to assess whether or not this complex surgical approach is indeed warranted.

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.

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.

Direct bronchial artery revascularization and en bloc double lung transplantation--surgical techniques and early outcome.

BACKGROUND: Lung transplantation including direct bronchial artery revascularization (BAR) has produced promising early results in small clinical series. METHODS: In Copenhagen primary en bloc double lung transplantation with BAR, with the left mammary artery used as conduit, has been performed in 47 patients from 1992 to the end of 1995. After introduction of the bloc into the recipient, the mammary-to-bronchial artery anastomosis is performed as the first anastomosis, allowing perfect exposure and early reperfusion. Internal mammary-bronchial artery arteriography has been performed routinely after operation. RESULTS: Bronchoscopic examination performed in all patients documented normal airway healing in 42, disturbed in two, and complicated in three. Arteriography performed in 42 patients demonstrated complete BAR in 25, incomplete in 15, and failed BAR in 2. Failed BAR was associated with complicated airway healing. The 1- and 2-year survival rate (Kaplan-Meyer) is 83%. Eleven patients have died, only one within 30 days. The total incidence of bronchiolitis obliterans syndrome at 3 years (with Kaplan-Meier technique) is 33%. Successful BAR has also been performed with an adjusted technique in a limited number of heart-lung and single lung transplantations. Our total experience of BAR in any type of lung transplantation includes 65 patients with an arteriographic BAR success rate of 94% (50 of 53 examined patients). CONCLUSIONS: Experience has improved the surgical technique and has made BAR reliable and safe, be it double lung, single lung, or heart-lung transplantation. Early results are good, but only follow-up will show if long-term results after lung transplantation will be improved by BAR. Already today, en bloc double lung transplantation with BAR is a viable alternative to sequential bilateral lung transplantation.

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.