Anatomical reconstruction of proximal coronary artery stenosis in children.

OBJECTIVES: Timing and method of surgical reconstruction for non-sclerotic proximal coronary artery stenosis, occurring de novo or post-coronary artery transfer, are evolving. We have pursued a technique of anatomical reconstruction of ostial and short segment proximal coronary artery stenosis and atresia in children, using patch plasty or interposition vein graft. Here, we discuss the medium- to long-term outcomes. METHODS: Nine consecutive children undergoing 10 left main coronary artery reconstructions using autologous great saphenous vein patch (n = 4), autologous pericardium (n = 4), xenopericardium (n = 1) or great saphenous vein interposition graft (n = 1) were retrospectively analysed. Complementary wedge resection of the stenotic coronary ostium was performed in chronic cases. RESULTS: The aetiology of coronary artery stenosis was post-arterial switch operation (n = 6), Takayasu's arteritis (n = 1), idiopathic left main coronary artery atresia (n = 1) and anomalous origin of the left coronary artery from the pulmonary artery (n = 1). The median age and weight at operation were 0.15 (range 0.01-13.1) years and 4.4 (range 3-13.1) kg, respectively. Survival was 100% at the medi follow-up of 12.6 (range 1-19.2) years. All patients showed normal left ventricular ejection fraction on transthoracic echocardiogram. In 1 patient, kinking of the proximal left circumflex artery resulted in non-significant obstruction. In all other cases, follow-up catheter angiography revealed unobstructed coronary arteries. Cardiac magnetic resonance tomography showed no significant perfusion deficit in any child. CONCLUSIONS: Anatomical reconstruction of the proximal left coronary artery using autologous saphenous vein may allow optimal restoration of physiological coronary blood flow, keeping the option of future coronary bypass operation open.

Coarctation of the aorta.

The surgical treatment of an aortic coarctation requires a resection of the stenotic area and direct suture of the aorta. An extended mobilization allows an enlargement of a hypoplastic distal aortic arch. In ductal dependent circulation, the distal aortic arch can be enlarged with a patch before tackling the coarctation itself. Postsurgical aortic arch stenoses often require a surgical intervention. Our preferred method is an anatomic correction with an enlargement plasty either on the concavity or on the convexity of the arch, depending on the local anatomy. An extra-anatomic bypass is also an option.

Heart transplantation in adolescent and adult patients with congenital heart disease: a case-control study.

BACKGROUND: The number of adolescent and adult patients with congenital heart disease undergoing heart transplantation is increasing. We aimed to better define the characteristics of these patients and their survival after transplantation. METHODS: We describe a group of patients with end-stage heart failure owing to congenital heart disease undergoing heart transplantation at a single tertiary center and compare their short- and long-term survival with a group of matched controls with dilated cardiomyopathy and the entire cohort of transplanted patients at our center. RESULTS: Between 1985 and 2006, a total of 322 orthotopic heart transplantations were performed at our center. Thirteen patients (mean age, 27.5 years) had a diagnosis of congenital heart disease with a wide spectrum of lesions. The survival of these 13 patients was 85% at 30 days, 1, 5, and 10 years and 77% at 20 years, which did not differ significantly to the short- and long-term survival of the entire cohort of patients with heart transplantation and to the survival of age-matched controls with dilated cardiomyopathy. CONCLUSION: In our single-center experience, short- and long-term survival after heart transplantation in a selected, small group of patients with end-stage heart failure owing to congenital heart disease did not differ significantly compared with a group of age-matched controls and the entire cohort.

Early results of balloon dilatation of the stenotic bovine jugular vein graft in the right ventricular outflow tract in children.

UNLABELLED: The aim of this study was to evaluate the early results of interventional balloon dilatation of stenotic bovine jugular vein (BJV) grafts implanted for reconstruction of the right ventricular outflow tract (RVOT) in children. METHODS: From May 2001 to December 2005, 153 BJV grafts were implanted in children in our institution. An average of 16.9 (7.6-41.1) months after implantation, 17 balloon dilatations in a significant stenosis proximal (n = 1), distal anastomosis (n = 8), BJV valve (n = 3), or at multiple sites (n = 5) were performed in 15 children (male:female = 9:6) with a mean age of 3.9 (0.8-13.0) years. Balloon diameter was 75-133.3% (mean 100.3) of the original BJV size. Mean follow-up was 8.8 (2 days to 22.8 months) months. RESULTS: In 10 interventions (58.8%) the instantaneous peak gradient was reduced below 50 mmHg. A balloon diameter > or =100% of the original BJV size correlated significantly with a successful intervention. No major complications, two minor (nonobstructive floating membranes at the dilatation site and one septicemia) occurred afterward. Freedom from reintervention after 6 months was 58.2% for all, 77.8% for dilatations of the proximal anastomosis and mixed stenotic lesions, and 33.3% for the distal anastomosis. CONCLUSION: Balloon dilatation of stenotic BJV grafts is safe and can significantly reduce the pressure gradient in two-thirds of interventions. Balloon diameters above the original graft size should be aimed for. The most frequent stenosis of the distal anastomosis tends to renarrow early after dilatation. Nevertheless, balloon dilatation should be considered in nearly every stenotic graft to gain time until a surgical or interventional graft exchange.

WITHDRAWN: Enlargement of the Right Atrium - Diverticulum or Aneurysm?

The publisher regrets that this was an accidental duplication of an article that has already been published in Eur. J. Echocardiogr., 4 (2003) 223-225, . The duplicate article has therefore been withdrawn.

Administration of steroids in pediatric cardiac surgery: impact on clinical outcome and systemic inflammatory response.

Cardiopulmonary bypass (CPB) is associated with a systemic inflammatory response. Pre-bypass steroid administration may modulate the inflammatory response, resulting in improved postoperative recovery. We performed a prospective study in the departments of cardiovascular surgery and pediatric intensive care medicine of two university hospitals that included 50 infants who underwent heart surgery. Patients received either prednisolone (30 mg/kg) added to the priming solution of the cardiopulmonary bypass circuit (steroid group) or no steroids (nonsteroid group). Clinical outcome parameters include therapy with inotropic drugs, oxygenation, blood lactate, glucose, and creatinine, and laboratory parameters of inflammation include leukocytes, C-reactive protein, and interleukin-8. Postoperative recovery (e.g., the number, dosage, and duration of inotropic drugs as well as oxygenation) was similar in patients treated with or without steroids when corrected for the type of cardiac surgery performed. After CPB, there was an inflammatory reaction, especially in patients with a long CPB time. Postoperative plasma levels of interleukin-8 were correlated with the duration of CPB time (r = 0.62, p < 0.001). Administration of steroids had no significant impact on the laboratory parameters of inflammation. Administration of prednisolone into the priming solution of the CPB circuit had no measurable influence on postoperative recovery and did not suppress the inflammatory response.

Aberrant tendinous chords with tethering of the tricuspid leaflets: a congenital anomaly causing severe tricuspid regurgitation.

OBJECTIVE: To define the entity of tricuspid regurgitation caused by tethering of the tricuspid valve leaflets by aberrant tendinous chords. DESIGN: Retrospective study. SETTING: Tertiary care centre (university teaching hospital). PATIENTS: 10 patients with unexplained severe tricuspid regurgitation. METHODS: The last 13 500 echocardiographic studies from our facility were reviewed to identify patients with severe unexplained tricuspid regurgitation. Tethering was defined by the presence of aberrant tendinous chords to the tricuspid valve leaflets limiting the mobility of the tricuspid leaflet and resulting in incomplete coaptation and apical displacement of the regurgitant jet origin. Aberrant tendinous chords were defined as those inserting at the clear zone of the tricuspid leaflet and not originating from the papillary muscle. Patients fulfilling the diagnostic criteria for Ebstein's anomaly were excluded. RESULTS: 10 patients with aberrant tendinous chords tethering one or more tricuspid valve leaflets were identified. There were short non-aberrant tendinous chords in seven patients, five of whom also had right ventricular or tricuspid annulus dilatation. CONCLUSIONS: Tethering of the tricuspid valve leaflets by aberrant tendinous chords can be the sole mechanism of congenital tricuspid regurgitation. It is often associated with short non-aberrant tendinous chords, which may develop secondary to right ventricular or tricuspid annulus dilatation. Awareness of tethering as a cause of tricuspid regurgitation may be important in planning reconstructive surgery.

Enlargement of the right atrium--diverticulum or aneurysm?

Diverticula and aneurysms are rare congenital anomalies of the right atrium. Here, we report a case of a giant congenital diverticulum of the right atrium in a 27-year-old female and discuss the morphological characteristics distinguishing diverticula and aneurysms.

Results of the arterial switch operation in neonates with transposed great arteries.

BACKGROUND: The arterial switch operation is judged the best palliative operation for neonates with transposed great arteries. We aimed to assess the value of analysing a large series of unselected cases by this technically demanding operation and formulate a realistic prognosis. METHODS: We reviewed all 432 neonates (mean age at operation 7 days, mean weight 3.25 kg) who underwent an arterial switch operation between 1987 and 1999. Follow-up (mean time: 4.9 years) was complete in 412 patients. FINDINGS: Survival probability and freedom from reoperation was 94% and 78% at 10 years, respectively. 26 patients died, 16 because of myocardial ischaemia. Risk factors for death included early experience, low weight, associated cardiovascular malformations (especially hypoplasia of the right ventricle or aortic arch), and difficult patterns of coronary arteries. The risk of the coronary artery pattern was greatly reduced in those who had recent operations. At last follow-up, 90% of patients had normal life without treatment, and 94% a normal heart function on echocardiography. INTERPRETATION: The arterial switch operation in neonates achieves excellent results mid-term. Obstruction of the translocated coronary arteries is responsible for most deaths and a substantial number of reoperations. Although confirmation is needed, these results allow anticipation of a favourable long-term prognosis.

Symptomatic mechanical heart valve thrombosis: high morbidity and mortality despite successful treatment options.

BACKGROUND: Recommendations for treatment of mechanical prosthetic heart valve thrombosis (PVT) include systemic thrombolysis and/or reoperation. Data on complications and outcome are limited. METHODS: Clinical and echocardiographic findings of 17 patients with mechanical PVT were reviewed. Complications and outcome of surgery and/or thrombolysis were analysed. Prospective follow-up was obtained. RESULTS: Symptomatic PVT occurred 8.4 +/- 7.2 years after mechanical valve replacement at mean age 55 +/- 15 years. Thrombosis involved the mitral valve in 12 patients (71%), the aortic valve in 4 (24%) and the tricuspid valve in one (6%). The reason for PVT was inadequate anticoagulation in 11 patients (65%), endomyocardial fibrosis in 2 (12%) and unknown in 4 (24%). Prior to diagnosis, systemic emboli occurred in 6 patients (35%). Thirteen patients (76%) presented in functional class NYHA IV. Haemodynamic valve obstruction was documented by echocardiography in 15 patients (88%). Treatment included primary reoperation in 12 patients (71%), thrombolysis with urokinase in 3 (18%) (with reoperation in 1), reinstitution of adequate anticoagulation in one (6%); death occurred before treatment in one (6%). Intraoperatively, both pannus and thrombus were found in 5 of 13 patients (38%). Treatment-related emboli occurred in 5 patients (29%), to the brain in 3, to the legs in one and to a coronary artery in one. Five patients died (mortality 29%) within 30 days due to multiorgan failure/septicaemia (3 patients), congestive heart failure (1), or cerebral emboli (1). Follow-up after 28 +/- 28 months in the 12 surviving patients was unremarkable. CONCLUSIONS: The most common aetiology for obstructive PVT is thrombus formation due to inadequate anticoagulation. PVT remains a serious complication with high morbidity and mortality despite aggressive treatment by thrombolysis and/or surgery. Surgery is often needed due to the frequent presence of pannus and/or large thrombi. However, long-term prognosis after successful treatment of PVT is excellent.

Technical considerations in the repair of combined aortic arch and diaphragm rupture after blunt trauma.

Rupture of the aortic arch and laceration of the diaphragm after blunt trauma are two serious lesions that are difficult to manage. (1,2) We repaired both lesions in a patient with an anterior approach (i.e., a median sternotomy and a superior median laparotomy). The repair, carried out during extracorporeal circulation, turned out to be relatively easy to perform. The potential dangers associated with the use of cardiopulmonary bypass after blunt trauma must be acknowledged. In selected cases, however, when no strong contraindications to the administration of anticoagulation exist, the anterior approach and the use of cardiopulmonary bypass offer an excellent exposure for the repair of both lesions.

Results of the Lecompte procedure in malposition of the great arteries and pulmonary obstruction.

OBJECTIVE: Assessment of the Lecompte procedure, our repair method of choice in malposition of the great arteries with pulmonary stenosis. METHODS: A retrospective analysis of 42 patients (median age at operation, 1.4 years) operated on between 1986 and 1999 for various forms of great artery malposition, ventricular septal defect, and pulmonary stenosis. Relevant associated findings included the insertion of a tricuspid papillary muscle on the conal septum (nine patients), absence of conal septum (six patients), hypoplasia of a side pulmonary artery (four patients), and hypoplasia of the right ventricle (one patient). A preliminary systemico-pulmonary shunt was created in 28 patients and a cavo-pulmonary anastomosis in one patient. At operation, the conal septum (whenever present) was resected (36 patients), the pulmonary bifurcation was usually translocated over the ascending aorta (37 patients), and the main pulmonary artery was enlarged with a patch of pericardium. A monocusp valve was fashioned within the patch in 40 patients. The follow-up information was complete in 32 patients and ranged from 0.4 to 14 years (mean, 5.4+/-3.2 years). RESULTS: The survival rate at 5 years was 92+/-5%. Three patients died post-operatively (mortality, 7%) and none during follow-up. The freedom from reoperation was 86+/-8 and 51+/-22% at 5 and 10 years, respectively. Six patients were reoperated, all for a pulmonary stenosis. Calcification of the monocusp patch was present in all. Pulmonary stenosis developed in three further patients: one underwent percutaneous dilatation and two are awaiting surgery. No procedural factors thought to have a potential correlation with the development of right ventricular outflow tract stenosis could be individualized on univariate analysis. CONCLUSIONS: The Lecompte procedure, which allows early repair of these defects, provides overall good results. The need to reoperate on the right or left ventricular outflow tract seems reduced in comparison with the Rastelli operation, the other alternative.

A 20-year follow-up of internal carotid artery endarterectomy with bifurcation advancement.

BACKGROUND: Carotid artery disease is a frequent cause of transient ischemic attack and of cerebral infarction. For two last decades, we have been performing endarterectomy of the internal carotid artery with bifurcation advancement. METHODS: From January 1977 until December 1997, all records of patients who underwent internal carotid artery endarterectomy with bifurcation advancement were reviewed. Data were collected from patients charts and by a questionnaire. 160 patients (80.6% men, 19.4% women, average lifetime 65.1 year) underwent a total of 181 endarterectomies with bifurcation advancement. RESULTS: The 30-day mortality was 1.9% and the postoperative stroke plus death rate 3.1%. The incidence of reoperations was 0.6% with an average follow up of 64 months. In one patient (0.6%), a significant restenosis of the repaired carotid artery was observed. The 1, 5 and 10 years neurological death free survival (including early mortality) was 99.3%, 97.2% and 92.5% and the overall survival (including early mortality) was 96.3%, 78.9% and 59.3% (Kaplan-Meier). CONCLUSIONS: The technique of the internal carotid artery endarterectomy by bifurcation advancement is a safe and reliable method for improvement of cerebral blood supply. Or foreign material or autologous vein can thus be avoided. This method offers excellent long term patency and has a notable lack of late restenosis.

Temporary one-and-a-half ventricular repair to relieve lymphomatous obstruction of the pulmonary artery.

Mediastinal lymphoma may infiltrate the conoarterial junction of the heart and cause life-threatening compression of the pulmonary artery. Partial debulking of the tumor along the left pulmonary artery and creation of a cavopulmonary anastomosis on the right pulmonary artery restored satisfactory perfusion of the lungs and unloading of the right ventricle in a 19-year-old woman. Remission of the tumor after chemotherapy allowed subsequent takedown of the cavopulmonary anastomosis.

Surgical treatment of acquired left ventricular pseudoaneurysms.

BACKGROUND: We present a review of our experience with acquired pseudoaneurysms of the left ventricle in order to establish the risk of surgical repair. METHODS: Ten patients operated upon for a left ventricular pseudoaneurysm in our clinic between 1984 and 1999 were reviewed. The pseudoaneurysm, a complication of myocardial infarction (four acute and three chronic) or previous cardiac surgery (three chronic), was resected in all patients and the ventricular wall defect closed with direct sutures (five cases) or a patch (five cases). Coronary artery bypass graft was performed in 6 patients. RESULTS: Three patients died (postoperative mortality 30%) after repair of an acute postinfarction (2 patients) or a chronic postsurgical (1 patient) pseudoaneurysm. Three patients died during follow-up (median 4 years) of a carcinological (2 patients) or cardiac (1 patient) cause. Two years after repair, 5 patients were in New York Heart Association class I or II, and 1 patient was in class III. CONCLUSIONS: Repair of left ventricular pseudoaneurysms can be performed with acceptable results, although mortality is significant in acute myocardial infarction and redo operations. Propensity for fatal rupture, however, is higher than the surgical risk in acute pseudoaneurysms or in large or expanding chronic ones and warrants surgical repair. The best approach to small asymptomatic chronic pseudoaneurysm is unsettled.

Experience with postinfarction left ventricular free wall rupture.

BACKGROUND: Postinfarction rupture of the left ventricle is a rare event in which approach is not clearly standardised and outcome after repair is unknown. Our experience with this pathology was reviewed to analyze methods of repair and assess outcome beyond the patient's hospitalisation. METHODS: Five patients underwent surgical repair of a postinfarction ventricular rupture between 1990 and 1998. Electromechanical dissociation (3 patients) and sudden hypotension and bradycardia (2 patients) were clinical indicators of rupture. Four patients underwent repair with cardiopulmonary bypass and 1 patient without. Repair consisted of epicardial patching (2 patients), direct suture (1 patient), infarct-exclusion (1 patient), and debridement and patch closure (1 patient) of the rupture. Myocardial revascularization was performed in 3 patients and mitral valve repair in 1 patient. RESULTS: A satisfactory hemodynamic state was restored and bleeding was controlled in all patients. Two patients died postoperatively and another patient died 4 months after hospital discharge as a result of cardiac failure and/or sepsis. The other 2 patients are alive and in excellent condition 6 and 30 months respectively after repair. CONCLUSIONS: Postinfarction rupture of the left ventricle bears a high mortality, but survival with an excellent quality of life is possible after surgical repair.