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When circulatory failure occurs after paediatric cardiac surgery despite conventional therapy (inotropic medication, optimising pre- and afterload) mechanical assist devices can be used. Assist devices can be categorised in different ways: according to the type of pump used (centrifugal, axial flow, or pneumatic), according to univentricular or biventricular support and in relation to the presence of an oxygenator. Paediatric devices are non-implantable. Extra-corporeal membrane oxygenation (ECMO) consists of a roller pump and a membrane oxygenator and is a relatively simple means of biventricular cardiac support while respiratory support is provided at the same time. All other devices lack the oxygenator and thus provide only circulatory support. The main disadvantage of ECMO is that it can be used for a maximum of several weeks while modern pneumatic assist devices (MEDOS, Berlin Heart) can be used for several months. Thromboembolic events, bleeding and sepsis are the main complications of all assist devices. The use of circulatory assist devices can increase the chances of survival when severe cardio(pulmonary) failure occurs following surgical correction of congenital heart defects.

The objective of this study was to evaluate the different causes of extubation failure and the consequent mortality rates in a pediatric population after cardiac surgery. We studied 184 consecutive patients with a median age of 9 months (range, 0-165). In 158 patients, extubation was successful (group A). Nine patients were reintubated for upper airway obstruction and finally extubated successfully (group B). Seventeen patients were reintubated for cardiorespiratory failure, finally leading to death in 11 of 17 patients (65%) (group C). Group B patients were younger and had a longer intubation period compared to group A patients. Group C patients had more reoperations (30% vs 4% in group A patients, p < 0.001), a lower PaO2 on admission at the intensive care unit as well as just prior to extubation, a lower base deficit before extubation, and needed more inotropic support during their stay in the intensive care unit. We conclude that extubation failure after pediatric cardiac surgery due to cardiorespiratory failure is a bad prognostic sign. Patients with high inotropic support and a low PaO2 prior to extubation are especially at risk and probably need careful evaluation before final extubation.

Objectives: The aim of this research was to assess right ventricular diastolic and systolic function before and after pulmonary valve replacement (PVR) in adult patients after repair of tetralogy of Fallot., Background: Pulmonary valve replacement (PVR) in adult patients late after repair of tetralogy of Fallot leads to rapid improvement of right ventricular (RV) systolic function., Methods: A total of 16 patients and 8 healthy subjects were included. Median age at initial repair was 4.9 (0.9 to 13.1) years, and mean age at PVR was 28.7 (19.5 to 45.6) years. Cardiac magnetic resonance imaging was performed before and 8 and 22 months after PVR. Right ventricular volumes and function as well as RV in- and outflow patterns were assessed., Results: The volume of the early filling of the RV (Evol) increased from 49.8 +/- 14.7 ml to 53.8 +/- 19.3 ml (not significant) and 62.0 +/- 18.9 ml, respectively (p < 0.05), whereas the volume of the atrial contraction (Avol) remained unchanged. Consequently, the Evol/Avol ratio increased from 1.4 +/- 0.7 before PVR to 1.6 +/- 0.7 at 8 months (not significant) and 2.3 +/- 1.2 at 22 months (p < 0.01). The Evol/Avol ratio was not significantly different from the healthy subjects at 22 months, indicating late recovery of diastolic function. Systolic function improved rapidly after PVR; the indexed RV end-systolic volume decreased from 93.7 +/- 33.0 ml/m2 to 60.9 +/- 18.4 ml/m2 (p < 0.01) and 54.8 +/- 21.0 ml/m2 (p < 0.01)., Conclusions: In adult patients late after total repair of Fallot, PVR leads to late improvement of diastolic function. We speculate that the rapid volume unloading after PVR increases systolic performance, whereas improvement in diastolic function requires long-term remodeling.

Objective: Pediatric cardiac surgery is associated with a temporary rise in cardiac troponin T (cTnT) during the postoperative period. We examined whether dexamethasone given before cardiopulmonary bypass has myocardial protective effects as assessed by the postoperative production of cTnT., Design and Setting: Prospective randomized interventional study in the pediatric intensive care unit in a university hospital., Interventions: Patients were randomly allocated to act as controls or receive a single dose of dexamethasone (1 mg/kg) during induction of anesthesia., Measurements and Results: cTnT was measured four times postoperatively: immediately after admission to the pediatric intensive care unit (PICU) and 8, 15, and 24 h thereafter. The two groups had similar mean cTnT concentrations on PICU admission: those receiving dexamethasone 1.85 ng/ml (1.55-2.15) and those not receiving it 2 ng/ml (95% confidence interval 1.56-2.51). Concentrations of cTnT 8 h after admission to the PICU differed significantly after 8 h: 1.99 ng/ml (1.53-2.45) in those receiving dexamethasone and 3.08 ng/ml (2.46-3.69) in those not receiving it. After subgroup statistical analysis differences between the two groups remained significant only at 8 h, not those after 15 or 24 h., Conclusions: The use of dexamethasone (1 mg/kg) before cardiopulmonary bypass is associated with a brief but significant reduction in postoperative cTnT production. The clinical significance of this effect is unclear.

A 4-year-old previously healthy boy presented with a non-traumatic right parietal hemorrhage. A second life-threatening left cerebral hemorrhage occurred three weeks later and was decompressed with a craniotomy. Transthoracic echocardiography revealed a hypermobile elongated tumor of the mitral valve. The cardiac tumor was successfully resected three weeks after the craniotomy. Histological examination of the cardiac tumor revealed a papillary lesion of spindle cells with smooth muscle cell differentiation. In view of the histological findings and the clinical symptoms, a cellular myofibroblastic tumor was considered the most likely diagnosis in our patient. Although a cardiac tumor is a rare cause of a cerebral hemorrhage, a cardiac evaluation is recommended in pediatric patients with a cerebral hemorrhage of unknown etiology.

Objectives: Little attention has been paid to the effect of the systemic inflammatory response syndrome on intestinal dysfunction in the postoperative period. Several proinflammatory cytokines have been reported to increase the permeability of intestinal mucosa in vitro. We investigated the effect of dexamethasone on gut permeability in pediatric patients undergoing cardiac surgery by using the dual sugar permeability test and absorption of 2 other saccharides., Methods: Thirty-four patients scheduled for cardiac surgery with cardiopulmonary bypass were prospectively randomized to either act as control subjects or to receive dexamethasone (1 mg . kg -1) during induction of anesthesia. Intestinal permeability was measured with 3-O-methyl-D-glucose, D-xylose, L-rhamnose, and lactulose administered orally after induction of anesthesia and 12 and 24 hours later., Results: Lactulose/rhamnose ratios were increased from the outset in both groups (mean 0.57 [95% confidence interval, 0.24-0.91] for the control group and 0.76 [95% confidence interval, 0.35-1.17] for patients receiving dexamethasone). Although the ratios decreased 12 hours (0.29 [95% confidence interval, 0.17-0.42]) and 24 hours later (0.17 [95% confidence interval, 0.08-0.15]) in the dexamethasone group, in the control group there was a rise at 12 hours (0.77 [95% confidence interval, 0-1.64]), with a slight reduction 24 hours later (0.46 [95% confidence interval, 0.06-0.85])., Conclusions: Infants and children undergoing cardiac surgery with cardiopulmonary bypass show a significant reduction in gut permeability when dexamethasone is used during induction of anesthesia. Dexamethasone does not affect the intestinal barrier at the functional level, as assessed on the basis of 3-O-methyl-D-glucose and D-xylose absorption.

Background: Paediatric cardiac surgery is associated with some degree of myocardial injury. Ischaemic preconditioning (IP) has been investigated widely in the adult population. Volatile agents have been shown to simulate IP providing extra protection to the myocardium during adult cardiopulmonary bypass (CPB) while propofol seems to act through different mechanisms. IP has not been investigated in the paediatric population to the same extent. Cardiac troponin T (cTnT) is a reliable marker of myocardial injury in neonates and children. We have investigated the relationship between three anaesthetic agents, midazolam, propofol, and sevoflurane, and postoperative production of cTnT., Methods: Ninety patients undergoing repair of congenital heart defect with CPB were investigated in a prospective randomized study. cTnT was measured four times during the first 24 h following admission to the paediatric intensive care unit. Other variables measured included arterial blood gases, lactate, fluid balance, use of inotropic drugs, PaO2/FiO2 ratio and ventilator hours., Results: cTnT was elevated in all three groups throughout the study period. The differences between the three groups were not statistically significant. Eight hours after admission to the intensive care unit cTnT concentrations tended to be higher in the midazolam group [mean (95% confidence intervals)]; 2.7 (1.9-3.5) ng ml(-1). Patients receiving a propofol-based anaesthesia had similar concentrations 2.6 (1.7-3.5) ng ml(-1) while those receiving sevoflurane tended to have a lower cTnT production 1.7 (1.3-2.2) ng ml(-1)., Conclusions: Midazolam, propofol, and sevoflurane appear to provide equal myocardial protection in paediatric cardiac surgery when using cTnT as a marker of myocardial damage.

Objective: To analyse the results of the mid-term clinical and echocardiographic follow-up of the pediatric Ross operation., Methods: Echo-Doppler follow-up of 53 consecutive pediatric Ross procedures performed between 1994 and 2003. Median age was 9.7 years at time of operation (2 weeks-17.7 years). Six patients were younger than 3 months. Median age at follow-up was 15.6 years. Aortic valve/left ventricular outflow tract (LVOT) anomalies were congenital in 49 (92%). Seventy percent had previous surgery or balloon valvuloplasty. Root replacement was used in all. Thirteen patients (25%) had LVOT enlargement. Mean cross-clamp time was 113 (69-189) minutes., Results: Early mortality occurred in 3 patients after emergency surgery following balloon failure (n=1) and extended Ross following interrupted arch/VSD repair (n=2). Late mortality was due to LV fibroelastosis in 2 patients and complicated pulmonary artery stenting in another. RVOT reoperations were required because of late homograft obstruction in 2 patients and because of pulmonary artery stenosis in another. Five patients (9.4%) were reoperated for pulmonary autograft dilatation (n=3) and for leaflet fibrosis or perforation (n=2). Autografts were repaired in two patients, while a mechanical valve was inserted in 3 cases. At 9 years the actuarial survival and event free survival were 89 and 74%, respectively. At last follow-up 90% of autograft diameters indexed to body surface area was above the 90th percentile of normal aortic root diameters. LVOT and RVOT gradients were low and autograft insufficiency was trivial to mild in 84% and mild to moderate in 16%. Autograft stenosis was not noticed., Conclusions: The pediatric Ross procedure remains an important tool but autograft dilatation also occurs in the pediatric population. The significance of this finding has yet to be determined.

Aims: Late after total correction, Fallot patients with a long QRS duration are prone to serious arrhythmias and sudden cardiac death. Pulmonary regurgitation is a common cause of right ventricular (RV) failure and QRS lengthening. We studied the effects of pulmonary valve replacement (PVR) on QRS duration and RV volume., Methods and Results: Twenty-six consecutive Fallot patients were evaluated both pre-operatively and 6-12 months post-operatively by cardiac magnetic resonance (CMR). In this study, we present the computer-assisted analysis of the standard 12-lead electrocardiograms closest in time to the CMR studies. For the whole group, QRS duration shortened by 6+/-8 ms, from 151+/-30 to 144+/-29 ms (P=0.002). QRS duration decreased in 18 of 26 patients by 10+/-6 ms, from 152+/-32 to 142+/-31 ms. QRS duration remained constant or increased slightly in eight of 26 patients by 3+/-3 ms, from 148+/-27 to 151+/-25 ms. CMR showed a decrease in RV end-diastolic volume from 305+/-87 to 210+/-62 mL (P=0.000004). QRS duration changes correlated with RV end-diastolic volume changes (r=0.54, P=0.01)., Conclusion: Our study shows that PVR reduces QRS duration. The amount of QRS reduction is related to the success of the operation, as expressed by the reduction in RV end-diastolic volume.

Over the past decades, life expectancy in patients with congenital heart disease has increased dramatically. However, serious complications may develop late after total repair in infancy. These complications are usually the result of longstanding pulmonary regurgitation, which leads to dilatation of the right ventricle and an increased risk for severe arrhythmias. Therefore lifelong follow-up in these patients is required. Cardiac magnetic resonance imaging is the current imaging tool of choice because it offers superior imaging quality and enables accurate quantification of functional parameters such as flow volumes and systolic and diastolic performance.

Objective: Several decellularisation techniques have been developed to produce acellular matrix scaffolds for the purpose of tissue engineering, mostly comprising (non-)ionic detergents or enzymatic extraction methods. However, the effect of chemically induced decellularisation on the major structural and adhesion molecules as well as glycosaminoglycans, and the possible replenishment of lost compounds have escaped attention., Methods: Porcine aortic valves were treated with two different methods: detergent Triton X-100 and enzymatic Trypsine cell extraction. (Immuno-) histochemistry was used to address changes in extracellular matrix constitution (elastin, collagen, glycosaminoglycans, chondroitin sulfate, fibronectin and laminin) and the production of extracellular matrix components by seeded endothelial cells., Results: The Trypsine treated group showed a fragmentation and distortion of elastic fibers. Changes in collagen distribution were observed in both groups. An almost complete washout of glycosaminoglycans and chondroitin sulfate was observed in the Triton and Trypsin treated group, but the latter with a smaller glycosaminoglycans reduction. Both treatments resulted in a considerable washout of the adhesion molecules laminin and fibronectin. Furthermore, seeded endothelial cells were capable of synthesising laminin, fibronectin and chondroitin sulfate., Conclusions: Chemically induced decellularisation by Triton or Trypsine resulted in changes in the extracellular matrix constitution, which could lead to problems in valve functionality and cell growth and migration. Seeded endothelial cells were capable of synthesising extracellular matrix components lost by cell extraction. Further studies on tissue engineering should focus more on the effect of chemically induced cell extraction on the extracellular matrix of the remaining scaffold and the in vitro or in vivo replenishment of lost compounds.

Objective: The surgical treatment of pulmonary artery stenoses in small children with complex cardiac lesions can be technically difficult. A hybrid-approach combining corrective surgery and intraoperative stent placement may be complementary in these patients., Methods: Descriptive study in 11 small children (age: one week to 12 years, median of 12 months, weight: 2.5-20 kg) after previous cardiac surgery. Intraoperative stenting of pulmonary arteries was performed involving paediatric cardiologist and cardiac surgeon. Stenting was combined with repair of pulmonary atresia (n=2), right ventricular outflow and pulmonary artery reconstruction (n=3), unifocalisation of pulmonary arteries (n=1), revision of distal anastomosis of RV-PA-conduit after truncus repair (n=1), revision of distal anastomosis of RV-PA-conduit after AVSD/Fallot repair (n=1), aortic arch patch reconstruction after anatomical correction for transposition of the great arteries (n=1), bidirectional cavopulmonary anastomosis after Norwood I operation for hypoplastic left heart syndrome, (n=1) retrieval of a dislodged stent from the left pulmonary artery (n=1). In seven patients stenting was planned electively while in four patients it took place on an emergency base., Results: No complications occurred during stent implantation. One patient died three weeks postoperatively from diffuse bleeding due to a coagulation disorder. Ten patients left hospital after the surgical intervention with concomitant stent implantation. Stent re-dilatation was necessary in 4 patients 2-24 months postoperatively. After a median follow-up of 15 months ranging from 3 weeks to 7.5 years all stents were patent as diagnosed by angiography in 6 patients and by colour-Doppler in all other patients. One year after stent placement one stent was removed and another surgically opened during re-operation for conduit replacement in the smallest patient from this series. There was one late death during operative right ventricular outflow-tract reconstruction after initial stent placement., Conclusions: With intraoperative stent placement surgically difficult patch augmentation of small and fragile pulmonary vessels during repair of complex cardiac lesions can be avoided. Stents recruit pulmonary vessels and keep them open and amenable to future percutaneous or surgical interventions.

Objective: To evaluate the results of cardiosurgical treatment of children with Down's syndrome and a complete atrioventricular septal defect (cAVSD)., Design: Retrospective., Method: Data were collected from the records of all patients with Down's syndrome who had been subjected to primary corrective surgery for cAVSD in the period 1980-2003 in Leiden, The Netherlands. Exclusion criteria were: concomitant tetralogy of Fallot or interruption of the aortic arch and palliative banding of the pulmonary artery. Children with an abnormal shape of the ventricle making it impossible to correct biventricularly were not considered in the study. Data on mortality >30 days after the operation and on repeated surgery were included in the analysis if the duration of follow-up was at least 5 years., Results: The group comprised 148 children: 75 girls and 73 boys. The median age at time of surgery was 20 weeks (range: 6 weeks to 3.7 years) and showed a statistically significant decrease during the period under investigation. Of 4 children lost to clinical follow-up actual data could be obtained. The median duration offollow-up was 6 years and 7 months (range: 38 days to 23 years and 11 months). 28 children (19%) died. The mortality within 30 days after the operation decreased from 0-38% in the period 1980-1989 via 0-30% in the period 1990-1999 to 0% in 2000-2003. The percentage ofreoperations related to the correction ofcAVSD (14%; 14/98) did not seem to increase. Of the correction-related reoperations, 14% (2/14) were followed by a second reoperation. Conclusion. In the period 1980-2003, children with Down's syndrome and a cAVSD were corrected surgically at a younger and younger age. The early mortality decreased to 0% in the years 2000-2003. Echocardiography in the first weeks of life for all children with Down's syndrome makes it possible to diagnose those with a cAVSD early enough for surgical repair.

Background: Intestinal mucosal ischaemia can occur in infants and children during and after cardiac surgery. Severe decreases in mucosal perfusion may cause complications such as necrotizing enterocolitis and postoperative mortality. We investigated gut permeability in paediatric patients undergoing cardiac surgery using the dual sugar permeability test and absorption of two other saccharides., Methods: Thirty-four patients undergoing palliative or corrective surgical procedures with and without cardiopulmonary bypass were investigated. Intestinal permeability was measured using 3-O-methyl-D-glucose, D-xylose, L-rhamnose and lactulose, given orally after induction of anaesthesia and 12 and 24 h later., Results: Lactulose/rhamnose ratios were raised from the outset [median 0.39 (confidence interval 0.07-1.8 for patients undergoing operations without cardiopulmonary bypass and 0.30 (0.02-2.6) with cardiopulmonary bypass]. The highest lactulose/rhamnose ratios were recorded 12 h after surgery 0.32 (0.07-6.9), when cardiopulmonary bypass was used. This is approximately seven times the value expected in healthy children. There was an improvement in patients not undergoing cardiopulmonary bypass: 0.22 (0.03-0.85) 12 h and 0.11 (0-0.48) 24 h after induction of anaesthesia. Patients undergoing repair of aortic coarctation showed the fastest recovery: 0.09 (0.03-0.31) 12 h and 0.07 (0.04-0.35) 24 h after induction of anaesthesia., Conclusions: Patients with congenital heart defects have abnormal gut permeability when compared with healthy children of similar age. Cardiopulmonary bypass seems to affect the intestinal barrier morphologically (lactulose and rhamnose absorption) and functionally (3-O-methyl-D-glucose and D-xylose absorption).

Purpose: To assess the time course of right ventricular (RV) function improvement after pulmonary valve replacement (PVR) in patients 25.2 years +/- 7.0 after repair of tetralogy of Fallot., Materials and Methods: The medical ethics committee approved this study, and informed consent was obtained. Cardiac magnetic resonance (MR) imaging was performed before, 7 months after, and 19 months after PVR in 25 consecutive patients with tetralogy of Fallot with a 1.5-T MR imager. RV function was assessed with gradient-echo sequences in the short-axis plane. Pulmonary flow was assessed with a velocity-encoded phase-contrast sequence. Paired t test was used to evaluate follow-up data. Independent samples t test was used to assess differences based on the presence of recurrent pulmonary regurgitation (PR)., Results: Mean indexed RV end-diastolic volume decreased from 166.9 mL/m(2) +/- 41.3 before PVR to 113.5 mL/m(2)+/- 35.7 (P < .001) at 7-month follow-up and 111.7 mL/m(2)+/- 41.1 (P = .46) at 19-month follow-up. The RV ejection fraction was corrected for PR and improved from 25.0% +/- 7.7 before surgery to 44.1% +/- 11.9 (P < .001) and 45.2% +/- 11.1 (P = .39), at 7- and 19-month follow-up, respectively. Recurrent PR after PVR was found in 11 patients; 14 patients did not have recurrent PR. Total reduction of indexed RV end-diastolic volume at 19 months follow-up was more prominent in patients who did not have recurrent PR than in patients who did have recurrent PR (P < .05). Furthermore, improvement of RV ejection fraction corrected for regurgitation was more marked in patients who did not have recurrent PR than in patients who did have recurrent PR (P < .05)., Conclusion: In patients with tetralogy of Fallot, RV function improves rapidly after PVR and is sustained at 19-month follow-up in most patients; however, recurrence of PR after PVR appears to reduce recovery of RV systolic function., ((c) RSNA, 2004.)

We report the usefulness of a median sternotomy approach for the surgical treatment of residual aortic gradients or recoarctation and aortic aneurysms. This series confirms that excellent surgical results can be obtained in this technically challenging group of patients needing postcoarctation repair.

Objectives: Decellularization of aortic valve allografts in advance of transplantation is a promising approach to overcome immune-induced early graft failure. In this study the effects of in vitro cell extraction on extracellular matrix molecules and in vivo remodeling of decellularized aortic valves were investigated in a heterotopic aortic valve rat implantation model., Methods: Rat aortic valve conduits were decellularized by a 2-step detergent-enzymatic extraction method involving sodium dodecyl sulfate in combination with RNase and DNase. Cellular and acellular allogeneic (2x, n = 4) and syngeneic valve grafts (2x, n = 3) were grafted infrarenally into the descending aorta for 21 days. Immunohistochemical techniques were used to study extracellular matrix constitution (elastin, collagen, fibronectin, and chondroitin sulfate) and cellular infiltration., Results: The decellularization procedure resulted in a complete loss of all cellular structures from the entire valve conduit with minimal damage to the extracellular matrix. All transplanted cellular allografts became deformed, swollen, and acellular with major changes in extracellular matrix structure. The transplanted decellularized allografts, however, retained normal preserved valve leaflets comparable to transplanted cellular and acellular syngeneic grafts. With the exception of cellular syngeneic grafts, all other grafts showed retrovalvular thrombi., Conclusions: Damage to the valves caused by decellularization technique is much less than the damage caused by the recipient's immune response. In vitro removal of viable cells in (cryopreserved) homografts may decrease graft failure. Seeding with autologous or major histocompatibility complex-matched donor endothelial cells will be necessary to diminish damage induced by an absent blood-tissue barrier.

Inflammation and oxidative damage are believed to play an important role in the postoperative complications after cardiopulmonary bypass (CPB) in neonates. During the preparation of the prime, red blood cells (RBCs) release non-protein-bound iron (NPBI) and free haemoglobin/haem (Hb/haem). The presence of these prooxidants in the prime solution may increase oxidative stress in neonates undergoing CPB. The solution used as the basis of the prime solution may influence the degree of this oxidative stress. We investigated the NPBI and the Hb/haem binding capacities of two different prime solutions: a prime based on pasteurized human albumin and a prime based on fresh frozen plasma. The presence of NPBI and free Hb/haem were measured during and after the preparation of the prime solution. Only in the albumin prime was NPBI detectable. However, in both primes, the concentrations of free Hb/haem increased. Thus, to reduce the prooxidative effects of NPBI and free Hb/haem, RBCs should be added to the prime at the last possible moment. Adding fresh frozen plasma should be considered, as this would result in no detectable NPBI in the prime solution.

Objective: Transposition of the great arteries is currently treated by performing the arterial switch operation. Dilatation of the neoaortic root is a late complication with unknown cause. Samples of patients with untreated transposition of the great arteries and patients with normally related great arteries were compared to investigate a possible role for vascular remodeling in the dilatation process., Methods: Aortic and pulmonary artery vessel wall and sinus samples were taken from 20 untreated human heart specimens with transposition of the great arteries and 9 age-matched, normal, postmortem human heart specimens, divided into 2 groups according to age. Routine histology and immunohistochemical staining for smooth muscle cell differentiation markers alpha-smooth muscle actin, SM22, and calponin were performed., Results: This study revealed structural differences between the normal aorta and pulmonary artery in the early group, which became more pronounced in the late group. In the early stage in transposition of the great arteries, no marked differences were seen between the aorta and pulmonary artery. With increasing age, however, there was a pronounced down-regulation of all smooth muscle cell markers in the pulmonary artery., Conclusions: There is a structural difference between the normal neonatal aorta and pulmonary artery. The great arteries in transposition of the great arteries differ from each other and from normal vessels, indicating a structural vascular difference in transposition of the great arteries. In the pulmonary artery and sinus of untreated transposition of the great arteries, there is a dedifferentiation of smooth muscle cells with increasing age that we could not correlate to altered flow. This structural abnormality might provide an explanation for the neoaortic root dilatation that has been reported as a late complication of the arterial switch operation.

This report describes the mid-term beneficial hemodynamic effect of biventricular pacing in an infant with congestive heart failure after congenital heart surgery, due to resynchronization of the left and right ventricle, optimization of the AV delay, and (partial) correction of the LV dyssynchrony.

A 17-year-old girl with Turner's syndrome underwent two cardiac operations due to severe mitral stenosis with pulmonary hypertension, caused by a parachute-like mitral valve. The anomaly was associated with persistence of the left superior caval vein, which drained to the coronary sinus, and non-compaction of the left ventricular myocardium. The association of these lesions is rare in patients with Turner's syndrome.

Background: In patients with the tetralogy of Fallot, QRS prolongation predicts malignant ventricular arrhythmias. QRS prolongation may result from right ventricular dilatation. The relation of ECG markers to biventricular wall mass and volumes has not been assessed., Objective: To investigate the relations of surface ECG markers of depolarisation and repolarisation to right and left ventricular volume and biventricular wall mass., Methods: 37 Fallot patients (mean (SD) age 17 (9) years) were studied 14 (8) years after surgical repair; 34 had important pulmonary regurgitation. Left and right ventricular size was assessed from tomographic magnetic resonance imaging (MRI), and the amount of pulmonary regurgitation by velocity mapping MRI. QT, QRS, and JT duration and interlead dispersion markers were derived from a standard 12 lead ECG., Results: Mean QRS duration was significantly prolonged (133 (31) v 91 (11) ms in controls), as were dispersion of QRS (36 (17) v 20 (6) ms), QT interval (87 (48) v 42 (20) ms), and JT interval (93 (48) v 42 (19) ms). Biventricular volumes were increased (right ventricular end diastolic volume, 129 (41) v 70 (9) ml/m(2); left ventricular end diastolic volume, 83 (16) v 69 (10) ml/m(2)), as was right ventricular wall mass (24 (7) v 17 (2) g/m(2)). QRS duration correlated best with right ventricular mass (r = 0.55, p < 0.01)., Conclusions: In patients operated on for tetralogy of Fallot and with pulmonary regurgitation, ECG predictors of ventricular arrhythmias are influenced by several mechanical factors that may occur simultaneously. These include increased right ventricular volume, but also increases in left ventricular volume and in right and left ventricular wall mass.

Background: Lev's contribution to the understanding of the morphology of hearts with double-outlet right ventricle and the surgical feasibility for correction is important and remains in current use. However, the term noncommitted ventricular septal defect remains enigmatic. The aim of this study was to elucidate the morphologic nature of the noncommitted ventricular septal defect in view of its surgical implications., Methods: We examined 67 specimens with double-outlet right ventricle, focusing on the relationship of the ventricular septal defect to the semilunar orifices., Results: The defect was subaortic, subpulmonary, or doubly committed in 55 specimens. In a further 8 specimens, the defect opened into the outlet portion of the right ventricle, but the distance between the ventricular septal defect and the semilunar orifice was extensive, either because of extreme dextroposition of the aorta or a broad ventriculoinfundibular fold, which, in some cases, was associated with a long-outlet septum. A truly noncommitted ventricular septal defect was found in the inlet in the remaining 4 specimens. An atrioventricular septal defect without extension to the outlet was present in 3 cases, and a ventricular septal defect limited to the inlet was found in another case. The ventriculoinfundibular fold, part of the outlet septum and septal band or septomarginal trabeculation, had fused to form a crestlike structure. The septomarginal trabeculation is a useful landmark in the right ventricle to differentiate the inlet from the outlet in different forms of double-outlet right ventricle., Conclusion: We do not suggest to discard the Lev terminology but rather to differentiate the noncommitted ventricular septal defect into 2 types: the truly noncommitted defect of the inlet type and the not-directly-committed defect, which does open into the outlet portion of the right ventricle. The implication for the surgeon is 2-fold. The tricuspid valve or right part of the atrioventricular valve is interposed between the noncommitted ventricular septal defect and the semilunar orifice. The not-directly-committed defect opens into the outlet portion of the right ventricle but is not directly subaortic or subpulmonary.

Objectives: The limited availability of donor valves and experimental evidence that pulmonary valves can withstand systemic pressure made us use cryopreserved pulmonary homografts as aortic valve substitutes. We observed a high incidence of early reoperation because of severe graft insufficiency due to cuspal tears. The mid-term results are evaluated in this study and histological analysis of explanted homografts is performed to investigate the cause of graft failure., Methods: From December 1991 to April 1994, 16 patients (13 male; mean age 37.3 years, range 21-59 years) underwent aortic valve replacement with a cryopreserved pulmonary homograft. The indication was endocarditis (n = 4), bioprosthesis degeneration (n = 3) or congenital aortic valve disease (n = 9). All homografts were implanted freehand in the subcoronary position. All patients were contacted for follow-up and recent echo-Doppler studies were reviewed. Six explanted homografts were examined microscopically using routine histological techniques to analyze changes in cell population, collagen and elastic fiber structure., Results: Follow-up was complete in all patients. Reoperation was required in ten patients because of severe graft incompetence (mean implantation time 5.9 years, range 2.8-8.0 years). In two patients, recurrent endocarditis was the cause of graft failure. In the other eight patients the leaflets looked pliable and thin with gross tearing in one or more cusps. The histopathologic changes observed were remarkably similar in all examined grafts: the cusp tissue was almost non-cellular and the collagen fiber structure had mostly disappeared. At the site of rupture, the tissue had become thin with strongly degenerated collagen and elastic fiber structure. In the six patients with a homograft remaining in situ, echo-Doppler showed trivial to mild insufficiency in five cases and moderate to severe in one case, whereas no significant gradients were observed., Conclusions: We concluded that structural reduction of cell number and degenerative alterations in the molecular composition of the extracellular matrix in valve tissue is the main cause of early graft failure in this series. The use of cryopreserved pulmonary homografts in the systemic circulation is therefore not advised., (Copyright 2002 Elsevier Science B.V.)

Objective: Tissue degeneration reduces the durability of aortic and pulmonary homograft heart valves. Homograft valves can evoke cellular and humoral immune responses that might be detrimental to the valve tissue. Analyzing explanted homograft valves helps in understanding the different factors that eventually lead to tissue degeneration., Methods: A total of 40 homografts was acquired from patients whose grafts had been explanted because of stenosis (n = 22), insufficiency (n = 8), paravalvular leakage (n = 4), other technical problems (n = 4), noncardiac death (n = 1), and stenosis with endocarditis (n = 1). The period of implantation varied from 14 days to 16 years (median, 4 years). Cryopreserved valves (n = 31) were, in the majority, derived from beating-heart donors, whereas the fresh valves were sterilized with antibiotics and stored at 4 degrees C for an average of 32 days. Four unimplanted cryopreserved valves, 1 native aortic valve, and 1 native pulmonary valve were used as references. Analysis included macroscopy, light microscopy with routine hematoxylin and eosin staining (cellularity and tissue structure), and immunohistochemical studies to allow identification of macrophages (CD68) and T lymphocytes (CD3), endothelial cells, leukocyte adhesion molecules (CD54, CD106, and CD62E), and immunoglobulin (IgG) and complement factor (C3) depositions. In situ hybridization for the Y chromosome was performed in 10 cases, with host-donor sex mismatch, to distinguish between host and donor cells. The outcomes of histology and immunohistochemistry were related to clinical factors, such as implantation time and reason for explantation., Results: In the first year after implantation, a strong reduction in cellularity of the valve tissue was observed, with almost acellular tissues after 1 year. Trilaminar tissue architecture disappeared with the same speed, whereas endothelial cells were almost absent in all explants. Macrophages and T lymphocytes were encountered in 85% and 78% of the leaflets, respectively. Expression of leukocyte adhesion molecules was low in almost all grafts, and IgG and C3 depositions were not increased. Valve tissue cellularity consisted mainly of ingrown host cells when the implantation time exceeded 1 year., Conclusions: During the first year of implantation, homograft valves rapidly lose their cellular components and normal tissue architecture. A low-grade inflammatory response was observed, but no convincing evidence of immune-mediated injury was found.

Background: Pulmonary regurgitation (PR) late after total correction for tetralogy of Fallot may lead to progressive right ventricular (RV) dilatation and an increased incidence of severe arrhythmias and sudden death. Timing of pulmonary valve replacement (PVR) is subject to discussion, because the effect of PVR on RV function in adults is unclear. In this study, MRI was used to assess the effect of PVR on RV function and PR. Clinical improvement was established by means of the NYHA classification., Methods and Results: Twenty-six adult patients were included. Cardiac MRI was performed at a median of 5.1+/-3.4 months before and 7.4+/- 2.4 months after PVR. Mean preoperative PR was 46+/-10% (range, 25% to 64%). After PVR, 20 of 26 patients (77%) showed no residual PR, 5 patients showed mild residual PR, and 1 patient showed moderate PR. RV end-diastolic volume (RV-EDV) decreased from 305+/-87 to 210+/-62 mL (P<0.001), and RV end-systolic volume (RV-ESV) decreased from 181+/-67 to 121+/-58 mL (P<0.001). No significant change was found in RV-EF (42% versus 42%). However, RVEF corrected for regurgitations and shunting increased from 25.2+/-8.0% to 43.3+/-13.7% (P<0.001). Mean validity class improved from 2.0 to 1.3 (P<0.001)., Conclusions: In adult patients with PR and RV dilatation, late after total correction of tetralogy of Fallot, MRI measurements show remarkable hemodynamic improvement of RV function after PVR and improvement of validity. We therefore advocate a less restrictive management concerning PVR in these patients.

Objective: Limited information is available on outcome after autograft aortic valve replacement, in particular with respect to the durability of the autograft and of the allograft used to reconstruct the right ventricular outflow tract. A retrospective follow-up study of all patients who underwent a Ross procedure in the Netherlands since 1988 was done to obtain an overview of the Dutch experience with this procedure., Methods: From 1988 to January 2000, 348 Ross procedures were performed in nine centers in the Netherlands. Pre-operative, peri-operative and follow-up data from 343 patients in seven centers (99% of all Dutch autograft patients) were collected and analyzed., Results: Mean patient age was 26 years (SD 14, range 0-58) and male/female ratio was 2.1. Bicuspid valve or other congenital heart valve disease was the most common indication for operation. The root replacement technique was used in 95% of patients and concomitant procedures were done in 12%. Hospital mortality was 2.6% (N=9). Mean follow-up was 4 years (median 3.8, SD 2.8, range 0-12.5). Overall cumulative survival was 96% at 1 year (95% confidence interval (CI) 94-98%) and 94% at 5 and 7 post-operative years, respectively (95% CI 91-97%). At last follow-up, 87% of the surviving patients was in New York Heart Association (NYHA) class I. Independent predictors of overall mortality were pre-operative NYHA class IV/V and longer perfusion time. Autograft reoperation had to be performed in 14 patients and reintervention on the pulmonary allograft in 10 patients. Freedom from any valve-related reintervention was 88% at 7 years (95% CI 81-94%)., Conclusions: The Dutch experience with the Ross procedure is favorable, with low operative mortality and good mid-term results. Although both the autograft in aortic position and the allograft in the right ventricular outflow tract have a limited durability, this has not yet resulted in considerable reoperation rates and associated morbidity and mortality.

A 5-month-old infant with aorto-right ventricular tunnel, pulmonary stenosis and a sole supply of the left coronary artery from the tunnel underwent closure of the ventricular end of the tunnel, subsequent to balloon pulmonary valvuloplasty. Care was taken not to obstruct the aortic end of the tunnel in order to maintain the left coronary artery perfusion.

Objective: Oxidative damage and inflammation are believed to play an important role in postoperative complications after cardiopulmonary bypass. During bypass, a prime solution with a high antioxidant capacity may reduce the oxidative damage and inflammation. We investigated total antioxidant capacity and individual scavengers during the preparation of 2 different prime solutions., Methods: The prime solutions were prepared with either pasteurized human albumin or fresh frozen plasma. The total antioxidant capacity was measured with the total radical antioxidant parameter assay and with the ferric-reducing ability of plasma assay. The individual scavengers vitamin C, sulfhydryl groups, uric acid, and total protein were measured before, during, and after the prime preparation. Malondialdehyde was measured as a parameter for lipid peroxidation., Results: Neither prime solution showed a total radical antioxidant parameter value. The ferric-reducing ability of plasma value of prime solutions was lower than that of undiluted human albumin or fresh frozen plasma. Addition of mannitol did not increase the ferric-reducing ability of plasma value. Vitamin C was only found in the fresh frozen plasma prime. Both prime solutions contained sulfhydryl groups and uric acid in low concentrations. During ultrafiltration, low-molecular-weight antioxidants were lost into the ultrafiltrate., Conclusions: We showed that prime solutions based on either albumin or fresh frozen plasma had very low antioxidant capacity and that ultrafiltration of the prime solution further lowers this capacity. A prime solution with a low antioxidant capacity may increase oxidative stress in neonates undergoing cardiopulmonary bypass.

The aim of the study was to investigate renal function and renal replacement therapy after cardiopulmonary bypass surgery in children. Patient characteristics (sex, age, diagnosis), operation type, and death were listed. The study was performed retrospectively using serum creatinine level before, and peak values after, cardiopulmonary bypass surgery for assessment of renal function. Of the children on renal replacement therapy, indication, efficacy, and complications were recorded. In a 5-year period, 1075 children had cardiopulmonary bypass surgery at the Department of Cardiothoracic Surgery at Leiden University Medical Center and Academic Medical Center of Amsterdam. One-hundred eighty (17%) patients developed acute renal insufficiency. Twenty-five (2.3%) patients required renal replacement therapy. Peritoneal dialysis is a safe and effective treatment for children after cardiopulmonary bypass surgery. However, 15 (60%) of 25 children on renal replacement therapy died of nonrenal causes. In 9 out of 10 surviving children, renal function was normal at time of discharge from hospital. Acute renal insufficiency is a frequent complication after open-heart surgery, although renal replacement therapy was infrequently necessary. Peritoneal dialysis is a safe and effective therapeutic measure for children after cardiac bypass surgery.

Purpose: To evaluate whether functional and metabolic changes recover after aortic valve replacement (AVR)., Materials and Methods: Eighteen men with aortic valve stenosis (mean pressure gradient +/- SD, 79.9 mm Hg +/- 15.1) underwent magnetic resonance (MR) imaging and phosphorus 31 MR spectroscopy. In nine patients who underwent AVR, MR imaging and spectroscopy were repeated 40 weeks +/- 12 after AVR. Ten age-matched healthy men were control subjects., Results: Before AVR, the myocardial phosphocreatine (PCr)-to-adenosine triphosphate (ATP) ratio in the 18 patients was 1.24 +/- 0.17 and 1.43 +/- 0.14 in the control group (P <.01). In nine patients who underwent follow-up MR spectroscopy, the ratio increased from 1.28 +/- 0.17 to 1.47 +/- 0.14 (P <.05) following AVR. Before AVR, early acceleration peak corrected for cardiac output was (0.043 +/- 0.008) x 10(-3) sec(-1) in patients and (0.081 +/- 0.033) x 10(-3) sec(-1) in the control group (P <.05). After 40 weeks +/- 12, the mean early acceleration peak corrected for cardiac output in the nine patients increased significantly to (0.055 +/- 0.006) x 10(-3) sec(-1) (P <.05), although it was still significantly lower than that of the control group (P <.05). Before AVR, a significant correlation was found between the myocardial PCr-ATP ratio and left ventricular diastolic function (n = 18; P <.05)., Conclusion: Severe aortic valve stenosis leads to a decreased myocardial PCr-ATP ratio and impairment of left ventricular diastolic function; following AVR, the ratio normalizes completely, whereas function improves significantly. There is an association between altered myocardial high-energy phosphate metabolism and impaired left ventricular diastolic function.

Objectives: To analyze the results of pulmonary valve insertion late after initial repair of Fallot's tetralogy. Pulmonary insufficiency (PI) after correction of Fallot's tetralogy is usually well tolerated in the short term, but is associated with symptomatic right ventricular dilatation and an increased risk of ventricular arrhythmias over longer periods of time., Methods: From 1993 to July 2000, 51 patients were reoperated for PI at a mean age of 25.7+/-11.9 years. The mean age at initial repair was 6.4+/-7.2 years. Patients with a conduit inserted at initial operation, with absent pulmonary valve syndrome or with a more than moderate ventricular septal defect at reoperation were excluded from the study. A cryopreserved pulmonary (96%) or aortic (4%) homograft was implanted in the orthotopic position with the use of cardiopulmonary bypass 19.3+/-9.1 years (2.7-40.3 years) after initial correction. Preoperative symptoms (New York Heart Association, NYHA class), degree of PI (echo-Doppler, MRI), right ventricular dimensions (MRI) and QRS duration were compared to findings at last follow-up., Results: Follow-up is complete and had a mean duration of 1.7+/-1.4 years. Hospital mortality was 2%. No serious morbidity occurred. Severe PI was present preoperatively in all patients. At last follow-up echo-Doppler studies showed PI to be absent or trivial in 96% and mild in 4% of patients. In 13 patients MRI studies were performed both pre- and postoperatively: in this group PI was reduced from a mean of 48 to 4%. After 6 months NYHA capacity class had improved significantly from 2.3+/-0.6 to 1.4+/-0.5. After 1 year end-diastolic and end-systolic right ventricular volumes were reduced significantly. Right ventricular ejection fraction and QRS duration remained unchanged., Conclusions: PI late after correction of Fallot's tetralogy may lead to serious symptomatic right ventricle dilatation. After pulmonary homograft insertion right ventricular dimensions decrease rapidly and functional improvement is observed in almost all patients.

Placement of Implantable cardioverter devices in young children is complicated because of the relatively large size and heavy weight of these devices. A technique is described where the device is implanted in the left perirenal space while an endovascular lead is used instead of an epicardial patch electrode.

Background: The aim of this study was to determine the morbidity, mortality, and hemodynamics after implantation of the Freestyle stentless bioprosthesis in the aortic position., Methods: A total of 280 patients were operated on from June 1993 to July 1999 as part of a multicenter investigation. Factors influencing hospital mortality and long-term survival were assessed by logistic regression and Cox proportional hazards analysis. Patients were evaluated postoperatively at discharge, at 3 to 6 months, and yearly by clinical examination and color flow Doppler echocardiography., Results: Hospital mortality in this group was relatively high (9.6%). Logistic regression analysis showed that cross-clamp time, age, myocardial infarction, diabetes, left ventricular hypertrophy, coronary artery disease, New York Heart Association class III or IV and female gender were the independent predictive factors. According to the Kaplan-Meier method, the 4-year survival for hospital survivors was 94%. In the multivariate Cox proportional hazard analysis, only coronary artery disease proved to be prognostic. During follow-up, 11 patients developed paravalvular leakage due to prosthetic dehiscence at the side of the noncoronary cusp. Performance of the prosthesis as assessed by echocardiography was excellent. Mean gradient decreased significantly between discharge and follow-up at 3 to 6 months. At 1-year follow-up trivial regurgitation was found in 6 patients (3%) and mild regurgitation in 4 (2%). Regurgitation did not increase with time. The effective orifice area increased significantly from discharge to follow-up at 3 to 6 months., Conclusions: Hospital mortality after implantation of a stentless bioprosthesis was higher compared to conventional prosthesis. A high incidence of prosthesis dehiscence at the proximal suture line was found, which was probably due to technique. Hemodynamic performance up to 3 years showed low transvalvular gradients. There is echocardiographic evidence for reduction of left ventricular hypertrophy and improvement of left ventricular function.

Objective: Dilatation and valve regurgitation are disturbing sequelae of the pulmonary root functioning at systemic pressures. We tried to characterize the histologic mode of adaptation of the neoaortic wall., Methods: We compared routine histologic studies, immunohistochemical staining, and computer-assisted morphometric analysis of aortic, pulmonary autograft, and native pulmonary wall specimens from pigs in which, as a newborn, a valveless pulmonary autograft had been implanted in the aorta., Results: Histologic examination of the pulmonary autograft revealed a viable, normally revascularized wall without degenerative phenomena. Smooth muscle cells were enlarged and rearranged. The characteristic "pulmonary" medial elastin lamellar structure was retained, which was confirmed by morphometry. Immunohistochemistry of the autograft revealed relatively strong staining of type III collagen and alpha smooth muscle actin, exclusive staining of basic fibroblast growth factor, and no staining of proliferation markers proliferating cell nuclear antigen and Ki67., Conclusion: The developing pulmonary autograft in the aortic position becomes normally revascularized, lacks major degenerative phenomena, and retains its own typical pulmonary morphologic features. Remodeling is accomplished by increased extracellular matrix deposition with collagen as an important constituent. The marked expression of growth factors in the autograft suggests the persistence of increased metabolic activity.

The surgical approach to a double outlet right ventricle (DORV) is dependent on the spatial relationship of the semilunar valves, outflow tracts and ventricular septal defect (VSD). The purpose of the study was to assess the value of MRI for the evaluation of cardiovascular anatomy in patients before and after surgery for DORV. Spin echo MRI was performed in 12 patients with DORV (eight preoperative and four postoperative patients). Thin-section MRI was performed in three orthogonal planes and selected angulated views were obtained. Conventional imaging by color Doppler echocardiography and cine-angiocardiography and surgical findings, when present, served as the reference standards. The results found that the spatial relationship between semilunar valves and VSD was accurately assessed by MRI in eight out of eight preoperative patients. In the four postoperative cases MRI depicted the morphology of both outflow tracts and provided adequate information on their patency. Of the eight preoperative patients, five have undergone corrective surgery and the MRI findings were confirmed. MRI provided additional information to conventional imaging preoperatively in three cases in which the VSD opened into the outlet portion of the DORV, without there being a direct relation to a semilunar valve. In two preoperative cases in which the VSD was directly committed to the aorta, conventional imaging was conclusive. MRI was unable to depict aberrant chordae tendineae in four out of four cases. We conclude that MRI provides accurate additional anatomic information in patients with DORV, which is helpful in presurgical planning as well as during follow-up. Spin echo MRI does not visualize aberrant chordae tendineae.

This study aimed to identify and characterize patients who developed prosthetic dehiscence after aortic valve replacement with the Freestyle Stentless bioprosthesis. Review of patients' records and preoperative echocardiogram was performed. Prosthetic dehiscence developed in 10 patients after 2.5 to 49 months (mean 19). Most patients were symptomatic at presentation. Nine patients were reoperated. Dehiscence was typically located at the inflow suture line beneath the noncoronary cusp. Four patients died. The cause of dehiscence is assumed to be related primarily to surgical technique.

The objective of this study was to determine normal Doppler and 2-dimensional characteristics of the Freestyle stentless aortic bioprosthesis. The Freestyle aortic bioprosthesis is a new type of aortic xenograft, and experience is limited. We therefore determined the normal range of echocardiographic and Doppler examinations of this valve. Three hundred thirty-nine consecutive patients with a Freestyle aortic bioprosthesis underwent an echocardiographic and Doppler examination according to a common protocol. Investigations were done within 4 weeks after operation, after 3 to 6 months, and after 1, 2, and 3 years. With a valve size from 19 to 27 mm, mean gradients decreased from 7.9 +/- 5.1 mm Hg at discharge to 5.5 +/- 3. 8 mm Hg after 3 to 6 months (P <.001). Thereafter, gradients remained stable. Effective orifice area 1 year after implantation was 1.59 +/- 0.58 cm(2) for the 21-mm valves, 1.92 +/- 0.74 cm(2) for the 23-mm valves, 2.03 +/- 0.64 cm(2) for the 25-mm valves, and 2.52 +/- 0.72 cm(2) for the 27-mm valves (P <.001). The performance index, the ratio of the measured effective orifice area in the patient divided by the effective orifice area measured in vitro, increased from 67% +/- 20% at discharge to 82% +/- 29% after 1, 2, and 3 years. Performance index was especially very high in the smaller-sized valves. After implantation with the subcoronary technique or root-inclusion technique, small cavities could be seen between the native aortic root and the Freestyle valve. Doppler values were evaluated for the Freestyle stentless porcine bioprostheses in the aortic root. Gradients appear to be close to those measured in native valves over a time period of 3 years.

Objective: Case histories of all patients (n = 29) operated on for supravalvular aortic stenosis from 1962 to the present were reviewed to study different techniques and outcomes. The technique of symmetric aortoplasty with 3 patches (1 in each sinus) is described and compared with other methods., Methods: Case reports were reviewed and follow-up was completed by contacting the patient's (pediatric) cardiologist. We aimed for a last follow-up visit, including Doppler echocardiographic studies, in a period no more than 12 months earlier than December 1997. Supravalvular aortic stenosis was discrete in 25 and diffuse with involvement of the aortic arch and arch vessels in 4 patients. Additional anomalies were bicuspid aortic valve (n = 5), coarctation (n = 3), ascending aortic aneurysm (n = 1), mitral valve insufficiency (n = 2), pulmonary valvular stenosis (n = 1), and peripheral pulmonary artery stenosis (n = 2). Eleven patients had Williams syndrome and 1 patient had Noonan syndrome. Symmetric aortoplasty with 3 patches (1 in each sinus) was used in 13 patients, whereas other nonsymmetric methods (1, 2, or Y-shaped patches) were used in 16 patients. Mean follow-up was 10.5 years (range: 4 months-36 years)., Results: All techniques adequately decreased the pressure gradient. Progression of preoperative aortic valve insufficiency or de novo regurgitation was not observed except in 1 patient in whom the patches inserted were too large., Conclusions: No difference could be demonstrated in outcome for any surgical technique; however, reconstruction of the aortic root with autologous pericardial patches in each sinus after transection of the aorta has the advantage of symmetry while restoring the normal aortic root anatomy.

In the early nineties, the stentless porcine aortic bioprosthesis has been reintroduced successfully. Because of the limited experience, knowledge of clinical complications is limited. Therefore, we describe an unusual complication of dynamic obstruction after aortic valve replacement with a stentless porcine valve in a 70 year old man 18 months after implantation. We discuss the complications of stentless aortic prostheses known so far, describe operative techniques used and their characteristic two dimensional echocardiographic images.

Because congenitally corrected transposition is a rare congenital anomaly, little is known about the prognosis of patients with this syndrome. The present study evaluated the functional status and clinical course of 73 patients (42 male and 31 female) aged 10 days to 73 years. Follow-up was 1 week to 37 years (mean 12.7 years). Particular attention was paid to the systemic right ventricle, considering the current controversies about long-term right ventricular function. Survival of patients with this condition in general was significantly below normal. The total mortality rate was 11% (8 patients) after a mean follow-up of 12.7 years (range 10 days to 37 years). Mean age at death was 18.5 years (range 6 days to 63 years). The mean age of the survivors at latest follow-up was 21.4 years (range 4 months to 73 years). In most patients, right ventricular function deteriorated and tricuspid valve regurgitation increased, which began at a very young age. Patients without associated lesions developed complications at a higher age compared with the total group. Right ventricular and tricuspid valvular function deteriorated more frequently in patients following intracardiac operation (28% and 52%, respectively) compared with patients undergoing palliative intervention (16%) or no surgery at all (28%). We conclude that survival of patients with this condition is substantially reduced compared with the natural history of an age- and gender-matched general population. This could be explained by the associated cardiac defects, but also by the anatomic condition itself.