Large fetal pulmonary arteriovenous malformation detected at midtrimester scan with subsequent high cardiac output syndrome and favorable postnatal outcome.

Prenatal diagnosis of pulmonary arteriovenous malformations (PAVM), caused by abnormal communications between pulmonary arteries and pulmonary veins, is rarely described. We report a case of a PAVM between the right pulmonary artery and the left atrium, referred to our prenatal unit at 22 + 1 weeks of gestation, with severe cardiomegaly, dilation of the right pulmonary artery and a right pulmonary vein and retrograde flow in the ductus arteriosus. The fistula was located in the right lung and showed a broad, disturbed flow at color Doppler with high velocity and low pulsatility. The fetus was monitored weekly and cardiac function remained sufficient until 36 + 1 weeks of gestation, when increasing cardiomegaly prompted delivery by cesarean section. The newborn was transferred to the neonatal intensive care unit, intubated and the fistula was surgically removed. The boy could be discharged at the 43rd day of life and showed an uneventful course until the last follow-up at the age of 4 years, with no residual mental or physical handicaps and a normal cardiac function. Despite adverse outcomes described in previously reported cases of large PAVM complicated by severe cardiomegaly at midtrimester scan, our case had a good outcome.

Coronary interventions in congenital heart diseases: from preterm to young adult patients.

BACKGROUND: The small vessel size of infants and children makes interventional treatment of impaired coronary perfusion, such as stenoses, complete occlusions, and fistulae, demanding. Materials and techniques appropriate for this young age group have to demonstrate their ability to effectively treat these lesions. METHODS AND RESULTS: Between 2004 and 2011, 14 patients with an age of 9 days to 25 years (median 4.6 years) and a bodyweight of 1.7-65 kg (median 14 kg) underwent coronary intervention. In 3 cases, emergency revascularization of the left coronary artery (CA) was performed successfully, followed by stent implantation in 1 patient. Embolization of coronary arterial fistulae with coils and vascular plugs was effective in 10 patients. An antegrade, retrograde or combined approach to achieve the most distal device placement preserved all side branches. One infant with pulmonary atresia and an intact ventricular septum was prepared for biventricular repair by step-by-step closure of the right ventricular to the CA connections. No procedure-related deaths occurred. CONCLUSION: Congenital and post-procedural coronary obstructive lesions can be considered for effective treatment with balloon dilation at any age as a salvage procedure. In coronaries impaired by external compression, stent implantation can restore perfusion, but long-term results are missing. Interventional closure of coronary fistulae has shown improvement of coronary arterial perfusion. The latter techniques can be used to close right ventricular to CA connections in patients with pulmonary atresia to prepare for biventricular repair, but bail-out strategies should be planned in all coronary interventions.

Congenital heart surgery: expected versus observed surgical performance according to the Aristotle complexity score.

BACKGROUND: The Aristotle score quantifies the complexity involved in congenital heart surgery. It defines surgical performance as complexity score times hospital survival. We studied how expected and observed surgical performance evolved over time. METHODS: 2312 main procedures carried out between 2006 and 2010 were analyzed. The Aristotle basic score, corresponding hospital survival and related observed surgical performance were estimated. Expected survival was based on the mortality risks published by O'Brien and coauthors. Observed performance divided by expected performance was called the standardized ratio of performance. This should trend towards a figure above 100%. Survival rates and performance are given with 95% confidence intervals. RESULTS: The mean Aristotle basic score was 7.88 ± 2.68. 51 patients died: observed hospital survival was 97.8 % (97.1 %-98.3%). 115 deaths were anticipated: expected survival was 95.2% (93.5%-96.3%). Observed and expected surgical performance reached 7.71 (7.65-7.75) and 7.49 (7.37-7.59), respectively. Therefore the overall standardized ratio of performance was 102.94%. The ratio increased from 2006 (ratio = 101.60%) to 2009 (103.92%) and was 103.42% in 2010. Performance was high for the repair of congenital corrected transposition of the great arteries and ventricular septal defect (VSD) by atrial switch and Rastelli procedure, the Norwood procedure, repair of truncus arteriosus, aortic arch repair and VSD closure, and the Ross-Konno procedure, with corresponding standardized ratios of 123.30%, 116.83%, 112.99%, 110.86% and 110.38%, respectively. With a ratio of 82.87%, performance was low for repair of Ebstein's anomaly. CONCLUSION: The standardized ratio of surgical performance integrates three factors into a single value: procedure complexity, postoperative observed survival, and comparison with expected survival. It constitutes an excellent instrument for quality monitoring of congenital heart surgery programs over time. It allows an accurate comparison of surgical performance across institutions with different case mixes.

Tricuspid atresia with absent pulmonary valve and intact ventricular septum: intrauterine course and outcome of an unusual congenital heart defect.

The extremely rare syndrome including absent pulmonary valve associated with membranous tricuspid atresia or severe tricuspid stenosis, intact ventricular septum and patent ductus arteriosus has been reported sporadically in the postnatal literature. This cardiac defect is characterized by right ventricular dysplasia with asymmetrical ventricular septal hypertrophy, ventricular septum bulging into the left ventricle, small right ventricular cavity, membranous tricuspid atresia or severe stenosis with abnormal papillary muscles and leaflets and absence of the pulmonary valve leaflets. The only prenatal case reported so far was diagnosed at 33 weeks of gestation and terminated shortly thereafter; the natural history of prenatally diagnosed cases is therefore unknown. We report on the intrauterine course of a case diagnosed at 17 weeks of gestation that had a favorable postnatal outcome after palliation.

Surgical management of congenital heart disease: correlation between hospital costs and the Aristotle complexity score.

BACKGROUND: Hospital costs are expected to correlate with clinical complexity. Do costs for congenital heart surgery correlate with Aristotle complexity scores? METHODS: 442 inpatient stays in 2008 were evaluated. Aristotle scores and levels were determined. Costs were estimated according to the German Institute for Hospital Reimbursement system. Pearson and Spearman R correlation coefficients and corresponding goodness-of-fit regression coefficients R2 were calculated. RESULTS: Mean basic and comprehensive Aristotle scores were 7.60 +/- 2.74 and 9.23 +/- 2.94 points, respectively. Mean expenses per hospital stay amounted to 29,369 +/- 30,823 Euros. Aristotle basic and comprehensive scores and levels were positively correlated with hospital costs. With a Spearman R of 1 and related R2 of 0.9436, scores of the 6 Aristotle comprehensive levels correlated best. Mean hospital reimbursement was 26,412 +/- 17,962 Euros. Compensation was higher than expenses for patients in comprehensive levels 1 to 3, but much lower for those in levels 4 to 6. CONCLUSIONS: Aristotle comprehensive complexity scores were highly correlated with hospital costs. The Aristotle score could be used as a scale to establish the correct reimbursement after congenital heart surgery.

Congenital heart surgery: applicability of hospital reimbursement according to German diagnosis-related groups system in conformity with the Aristotle complexity score.

BACKGROUND: Scores of Aristotle comprehensive complexity (ACC) levels have been demonstrated to correlate with the case-mix index (CMI) (cost-weights) generated by the German Diagnosis-Related Groups (DRG) 2009 version (G-DRG 2009). The equation used was "y = 0.5591 + 0.939 x" whereby y stands for cost-weight and x for ACC score. We hypothesised that each ACC level could be assigned a DRG (ACC DRG) and be used to determine hospital reimbursement. METHODS: 185 patients underwent cardiac surgery between January and June 2009. The ACC scores of these 185 patients were grouped in ACC levels, based on the basic cost-weight value of their DRG. One ACC DRG was assigned to each group and a corresponding cost-weight calculated based on the aforementioned equation. The resulting ACC CMI was compared with the CMI generated by the G-DRG 2009 (G-DRG 2009 CMI). Finally, the ACC surgical performance (complexity x hospital survival) was used to calculate the cost-weight; the obtained CMI was called "effective ACC CMI". RESULTS: Mean ACC score was 9.515 +/- 3.611 points. Derived ACC CMI and related G-DRG 2009 CMI were 9.494 and 8.438, respectively. Hospital survival was 97.8 % (181/184). Therefore ACC surgical performance and "effective ACC CMI" were 9.306 and 9.297, respectively. For each ACC level, the number of patients (n), mean ACC score, ACC CMI and related G-DRG 2009 CMI were as follows: Level 1: n = 25, 4.024 +/- 0.879, 4.338 and 5.911; Level 2: n = 30, 6.563 +/- 0.574, 6.722 and 6.602; Level 3: n = 43, 8.665 +/- 0.540, 8.695 and 8.088; Level 4: n = 73, 11.730 +/- 1.690, 11.574 and 9.612; Level 5: n = 14, 16.710 +/- 1.380, 16.249 and 11.843, respectively. CONCLUSIONS: The Aristotle score can be used to adjust hospital reimbursement by assigning a DRG and cost-weight value to each ACC level. Missing figures for level 6 can be obtained from a previous study which showed a mean score of 22.11 +/- 1.24: the ACC CMI would be 21.320. The 6 ACC DRGs indicate the correct compensation based on the complexity of the procedure. Reimbursement using the German DRG 2009 appears to favour less complex cases, while procedures with a higher complexity are penalised. Reimbursement according to "effective ACC CMIs" would have a strong impact by supporting units providing high-quality care.

The Norwood procedure - does the type of shunt determine outcome?

BACKGROUND: Stage I palliation of hypoplastic left heart syndrome (HLHS) and its variants is usually performed by a Norwood operation. The management of pulmonary blood flow during this procedure remains controversial. The RV-to-PA conduit (RVPAC) has been proposed as the better alternative compared to a systemic-to-pulmonary shunt (SPS). METHODS: A retrospective single center chart review of consecutive patients who underwent a Norwood I procedure between 01/1997 and 09/2006 was performed. All patients were operated in deep hypothermia, with or without circulatory arrest, using different shunt modifications according to surgeon's preference. Patients were divided into two groups depending on surgical management for pulmonary blood flow (modified BT shunt [BT] and non-valved RVPAC [Sano]). RESULTS: Fifty-four patients were included in the study (BT: 31 patients vs. Sano: 23 patients). Diastolic blood pressure during the first 24 hours postoperatively was significantly lower in the BT group (BT: 38.6 +/- 6.9 mmHg vs. Sano: 42.4 +/- 7.2 mmHg; P < 0.01) with a trend towards a higher systolic blood pressure (BT: 74.1 +/- 13.5 mmHg vs. Sano: 69.8 +/- 12.1 mmHg; P = 0.08). Mean circulatory arrest time in the BT group was significantly longer compared to the Sano patients (BT: 41 +/- 21 min vs. Sano: 25 +/- 23 min; P < 0.01). The mean hospital stay was 18.5 days for BT patients and 20 days for Sano patients ( P = 0.45). Early mortality for the total cohort was 14.8 % (n = 8) (BT 19.4 % [n = 6] vs. Sano 8.7 % [n = 2]; P = 0.12). There was no significant difference in inter-stage mortality between the two groups (BT: 18.2 % vs. Sano: 21.1 %; P = 0.47). CONCLUSION: The results for both established surgical methods (BT and Sano) for the palliation of HLHS and its variants have improved over time and are reaching acceptable early mortality rates. There was a trend towards a favorable early outcome for Sano patients, which did not reach statistical significance in this study due to the low patient numbers.

Arrhythmias in congenital heart defects.

AIM OF THE STUDY: Evaluation of the incidence and severity of late arrhythmias in patients with predisposing congenital heart defects--either due to the anatomy of the defect itself or as a result of a particular type of surgical intervention. PATIENTS AND METHODS: In a retrospective long-term study authors analyzed 158 patients (divided into 5 groups) with congenital heart defects after surgical correction. Evaluated were: the incidence of rhythm disturbances, the type of arrhythmia and the need for medication or intervention. RESULTS: The most rhythm disturbances occurred in patients after physiological correction of D-transposition of the great arteries (68.5%) and these patients also mostly needed medication or pacemaker implantation; followed were by patients with hypoplastic left heart syndrome after Fontan procedure (40%), then were patients after long-term correction of tetralogy of Fallot (31.1%), atrial septal defect sinus venosus type with partial anomalous pulmonary venous return after Warden correction (25.7%) and congenitally corrected L-transposition of the great arteries (25 %). Most of these arrhythmias were asymptomatic and there was no need to treat them. There was an increased incidence of arrhythmias with time (p < 0.05). DISCUSSION: During childhood in patients after surgical correction late arrhythmias mostly do not represent a severe problem, but with time, when reaching adulthood, this may be an issue. It is therefore very important to understand the anatomy, physiology and the arrhythmogenic substrate of every high risk congenital heart defect (Tab. 2, Fig. 6, Ref. 10).

Use of rapid-deployment extracorporeal membrane oxygenation for the resuscitation of pediatric patients with heart disease after cardiac arrest.

INTRODUCTION: We have recently used extracorporeal membrane oxygenation as a means of rapidly resuscitating pediatric patients with heart disease after cardiopulmonary arrest, in whom conventional resuscitation measures have failed. METHODS: We developed a fully portable extracorporeal membrane oxygenation circuit that is maintained vacuum and carbon dioxide-primed at all times. When needed, the circuit is crystalloid-primed and can be ready for use within 15 minutes. Since February 1996, we have used this rapid-deployment circuit to resuscitate 11 pediatric patients in full cardiopulmonary arrest. RESULTS: The median age of the 11 patients was 120 days (2 days to 4.6 years). Nine patients had a cardiac arrest after cardiac surgery. One patient had a cardiac arrest during cardiac catheterization and one patient had a cardiac arrest before cardiac surgery. Median duration of cardiopulmonary resuscitation was 55 minutes (range 20 to 103 minutes), with no difference in the duration of cardiopulmonary resuscitation between survivors and nonsurvivors. Ten of 11 patients (91%) were weaned from extracorporeal membrane oxygenation and seven (64%) survived to hospital discharge. Six patients are long-term survivors, five of whom are in New York Heart Association class I; one survivor is in class II. Seven patients resuscitated with extracorporeal membrane oxygenation before the use of this rapid-deployment circuit had a median duration of cardiopulmonary resuscitation of 90 minutes, with two (28.6%) survivors. CONCLUSIONS: The use of rapid-deployment extracorporeal membrane oxygenation results in shorter resuscitation times and improved survival in pediatric patients with heart disease after cardiopulmonary arrest.

Mechanical circulatory support in children with cardiac disease.

OBJECTIVE: To review the experience from a single center that uses both extracorporeal membrane oxygenation and ventricular assist devices for children with cardiac disease requiring mechanical circulatory support. METHODS: A retrospective chart review was performed for all pediatric patients with cardiac disease who required support with extracorporeal membrane oxygenation or ventricular assist devices. Statistical analysis of the impact of multiple clinical parameters on survival was performed. RESULTS: From 1987 through 1996 we provided mechanical circulatory support for children with a primary cardiac diagnosis using extracorporeal membrane oxygenation (67 patients) and ventricular assist devices (29 patients). Twenty-seven of 67 (40.3%) patients supported with extracorporeal membrane oxygenation and 12 of 29 (41.4%) patients supported with ventricular assist devices survived to hospital discharge. Failure of return of ventricular function within 72 hours of the institution of support was an ominous sign in patients supported with either modality. Univariate analysis revealed the serum pH at 24 hours of support, the serum bicarbonate at 24 hours of support, the urine output over the first 24 hours of support, and the development of renal failure to have a statistically significant association with survival in children supported with extracorporeal membrane oxygenation. None of the clinical parameters evaluated by univariate analysis were significantly associated with survival in the patients supported with ventricular assist devices. CONCLUSIONS: Extracorporeal membrane oxygenation and ventricular assist devices represent complementary modalities of mechanical circulatory support that can both be used effectively in children with cardiac disease.

A detailed histologic analysis of pulmonary arteriovenous malformations in children with cyanotic congenital heart disease.

INTRODUCTION: Pulmonary arteriovenous malformations are a common cause of progressive cyanosis in children after cavopulmonary anastomoses. We analyzed the pulmonary histologic characteristics from children in whom pulmonary arteriovenous malformations developed after procedures that resulted in pulmonary arterial blood flow devoid of hepatic venous effluent. METHODS: We performed routine histologic studies, immunohistochemical staining, and electron microscopic analysis of peripheral lung biopsy specimens from 2 children with angiographically proven pulmonary arteriovenous malformations. Microvessel density was determined with a computer-assisted, morphometric analysis system. RESULTS: Histologic examination demonstrated large, dilated blood vessels ("lakes") and clustered, smaller vessels ("chains") in the pulmonary parenchyma. Microvessel density was significantly greater in these patients than in age-matched controls (P =.01). Immunohistochemistry demonstrated uniform staining for type IV collagen and alpha-smooth muscle actin, weak staining for the endothelial marker CD31 (cluster of differentiation, PECAM-1), and negative staining for proliferating cell nuclear antigen. Electron microscopy revealed endothelial irregularity, a disorganized basement membrane, and increased numbers of collagen and actin filaments beneath the endothelium. CONCLUSIONS: This study represents an attempt to characterize the histologic features of pulmonary arteriovenous malformations in children with congenital heart disease who have pulmonary arterial blood flow devoid of hepatic venous effluent. The histologic correlate of this condition appears to be greatly increased numbers of thin-walled vessels. Immunohistochemistry suggests that the rate of cellular proliferation is not increased in these lesions. The development of these techniques may provide a standardized histologic approach for this condition and aid in understanding its etiology.

A new approach to correction of tetralogy of Fallot with absent pulmonary valve.

A new technique for primary repair of tetralogy of Fallot with absent pulmonary valve syndrome is suggested. In addition to the standard intracardiac portion of tetralogy of Fallot repair, this approach includes translocation of the pulmonary artery anterior to the aorta, shortening of the ascending aorta, and shortening and plication of the pulmonary artery. This new technique should eliminate compression of the tracheobronchial tree caused by impingement by the pulmonary artery.

Evaluation of a pulsatile pediatric ventricular assist device in an acute right heart failure model.

BACKGROUND: The development of pulsatile ventricular assist devices for children has been limited mainly by size constraints. The purpose of this study was to evaluate the MEDOS trileaflet-valved, pulsatile, pediatric right ventricular assist device (stroke volume = 9 mL) in a neonatal lamb model of acute right ventricular failure. METHODS: Right ventricular failure was induced in ten 3-week-old lambs (8.6 kg) by right ventriculotomy and disruption of the tricuspid valve. Control group 1 (n = 5) had no mechanical support whereas experimental group 2 (n = 5) had right ventricular assist device support for 6 hours. The following hemodynamic parameters were measured in all animals: heart rate and right atrial, pulmonary arterial, left atrial, and systemic arterial pressures. Cardiac output was measured by an electromagnetic flow probe placed on the pulmonary artery. RESULTS: All results are expressed as mean +/- standard deviation and analyzed by Student's t test. A p value less than 0.05 was considered statistically significant. Base-line measurements were not significantly different between groups and included systemic arterial pressure, 80.6 +/- 12.7 mm Hg; right atrial pressure, 4.6 +/- 1.6 mm Hg; mean pulmonary arterial pressure, 15.6 +/- 4.2 mm Hg; left atrial pressure, 4.8 +/- 0.8 mm Hg; and cardiac output, 1.4 +/- 0.2 L/min. Right ventricular injury produced hemodynamics compatible with right ventricular failure in both groups: mean systemic arterial pressure, 38.8 +/- 10.4 mm Hg; right atrial pressure, 16.8 +/- 2.3 mm Hg; left atrial pressure, 1.4 +/- 0.5 mm Hg; and cardiac output, 0.6 +/- 0.1 L/min. All group 1 animals died at a mean of 71.4 +/- 9.4 minutes after the operation. All group 2 animals survived the duration of study. Hemodynamic parameters were recorded at 2, 4, and 6 hours on and off pump, and were significantly improved at all time points: mean systemic arterial pressure, 68.0 +/- 13.0 mm Hg; right atrial pressure, 8.2 +/- 2.3 mm Hg; left atrial pressure, 6.4 +/- 2.1 mm Hg; and cardiac output, 1.0 +/- 0.2 L/min. CONCLUSIONS: The results demonstrate the successful creation of a right ventricular failure model and its salvage by a miniaturized, pulsatile right ventricular assist device. The small size of this device makes its use possible even in small neonates.

Results of modified Norwood's operation for hypoplastic left heart syndrome.

OBJECTIVE: The aim of the study was to analyze intermediate results of treatment of the hypoplastic left heart syndrome based on selective indication criteria. METHODS: Between February 1997 and May 1999 38 patients with hypoplastic left heart syndrome (n=35), or with functional variant of hypoplastic left heart syndrome (n=3) were admitted to our department. Contraindications for surgery were birth weight <2500 g, diameter of ascending aorta <2 mm, severe tricuspid regurgitation persisting after initial stabilization, pulmonary regurgitation more than mild, dysfunction of the systemic right ventricle and failure to effectively resuscitate circulation before surgery. RESULTS: Based on these criteria surgery was not indicated in 17 patients. Twenty-one infants were operated on by modified Norwood's procedure using only autologous great vessel tissue for reconstruction of systemic outflow. Overall hospital mortality was 14% (three patients). Eighteen survivors (86%) were discharged with well-balanced circulation. There was one late death (5%). Thirteen patients had already undergone the second stage (bi-directional Glenn) with no death. The mean follow-up was 13. 2+/-9.1 months (range 4-32 months). Considering both early and late events the probability of survival for the whole group (n=21) from the time of surgery was 86% at 1 month, 80% at 12 months, and it remained unchanged at 18 and 24 months of follow-up. CONCLUSIONS: Only a limited number of European countries offer surgical treatment of hypoplastic left heart syndrome. Promising intermediate results (80% survival rate after stage I and II) achieved at our department do not only reflect overcoming the learning curve but also a selective approach to indication for surgery as well. In a country with limited resources selective approach to the patients with hypoplastic left heart syndrome is justified.

Thyroid hormone metabolism in pediatric cardiac patients treated by continuous povidone-iodine irrigation for deep sternal wound infection.

OBJECTIVE: The purpose of this study was to assess the influence of povidone-iodine mediastinal irrigation used for the treatment of deep sternal wound infection (DSWI) on thyroid function. METHODS: Thyroid function was studied in 18 pediatric cardiac patients treated with continuous povidone-iodine irrigation for DSWI. The median age of patients was 8 months (18 days-5.3 years). Serum concentrations of total triiodothyronine (TT3), total thyroxine (TT4), free triiodothyronine (FT3), free thyroxine (FT4), thyroid-stimulating hormone (TSH), reverse triiodothyronine (rT3) and thyroxine-binding globulin (TBG) were measured at three time points: (a) prior to mediastinal reexploration (before povidone-iodine exposure); (b) immediately after discontinuation of povidone-iodine irrigation; (c) 2 weeks after discontinuation of mediastinal irrigation. Urinary iodine excretion was examined on the last day of povidone-iodine exposure. RESULTS: Prior to the mediastinal reexploration, the median TT3 and TT4 levels were below the normal range, then increased significantly to concentrations within the normal range. The median serum FT3 levels were within the normal range throughout the observation period, though a significant increase of FT3 levels was observed after discontinuation of irrigation. The median serum FT4 concentrations were within the normal range prior to irrigation and did not change significantly. The median rT3 levels were within the normal range, close to upper normal limit. The median TBG levels were within the normal range throughout the observation period, though a significant increase of TBG levels was observed during the period of mediastinal irrigation. The median TSH level was within the normal range prior to mediastinal irrigation and did not change significantly. Urinary iodine concentrations in infants with povidone-iodine irrigation were significantly higher 6700 microg/l (range, 1600-15000 microg/l) than in the group of 53 healthy infants 200 microg/l (range, 20-780 microg/l, P<0,001). CONCLUSIONS: Our data showed that the use of povidone-iodine irrigation in the patients with DSWI has not lead to any significant alteration in thyroid function within the study period.

Ross and Ross-Konno procedure in children and adolescents: mid-term results.

OBJECTIVES: The aim of the study was to analyze mid-term results of aortic root replacement with pulmonary autograft in children and adolescents in two centers. METHODS: From December 1997 through August 2003, a total of 66 patients underwent the Ross procedure in two centers. Indication for Ross procedure was predominantly aortic stenosis in 24 patients and predominantly aortic regurgitation (AR) in 22 patients. Twenty patients with severe left ventricular outflow tract obstruction (LVOTO) underwent Ross-Konno procedure. No patient had a geometric mismatch of more than 5 mm in favor of the aortic annulus. RESULTS: There was no early death. One patient died 3 months after surgery due to bacterial endocarditis. Survival on median follow-up period of 2.4 years was 98.5%. Neo-aortic regurgitation was none in 29 (44%) patients, trivial in 35 (53%) patients and mild in 2 (3%) patients. One patient (1.5%) needed aortic valve replacement because of autograft failure. Actuarial freedom from more than trivial neo-aortic regurgitation, or aortic valve replacement was 95% at 5 years follow-up. There was no patient either with recurrent LVOTO or significant aortic root dilatation. Freedom from redo was 93% at 5 years of follow-up. There had been a significant reduction (P = 0.001) and normalization in the left ventricle diastolic diameter index and left ventricle mass index, respectively, within 3-12 months after operation. Sixty-three percent of all operated patients are without medication; no one is on anticoagulation therapy. CONCLUSIONS: Our 7 years experience with the Ross and Ross-Konno operation has shown excellent mid-term results, with mortality rate approaching zero in both simple and complex left heart lesions, even in the neonates and infants. It is a procedure of choice in children with severe anomaly of the aortic valve and/or left ventricular outflow tract obstruction. The main concern is dilatation of the neo-aortic root leading to progression of AR, especially in the settings of geometric mismatch of aortic and pulmonary roots and bicuspid, regurgitant aortic valve. The risk of autograft failure in these specific subsets of patients remains to be determined.

Is a learning curve for arterial switch operation in small countries still acceptable? Model for cooperation in Europe.

OBJECTIVES: To assess the results of a cooperative arrangement between Slovakia and Slovenia for neonatal cardiac surgery. The aim of the study was to analyze the performance of this approach for complete transposition of the great arteries (D-TGA). METHODS: Due to the overall small number of new patients with D-TGA in Slovenia a decision was made to avoid a prolonged learning curve by centralizing the experience of two countries at one center. Since 1995 the center in Slovakia has become the only referral center for Slovenia. Between February 1993 and June 2002 in this center, 147 patients with D-TGA underwent arterial switch operation (ASO). The median age at operation was 11 days, with 110 patients from Slovakia and 37 patients from Slovenia. RESULTS: Overall hospital mortality was 4.8% (seven patients). The 1, 2, 3, 4 and 5 year survival rate was 95% with the mean follow-up of 4 years. Operation before 1997 (P=0.0001) was identified as a risk predictor for death by multivariate analysis. There are no deaths among the 90 patients operated on after 1996. All patients are without medication with normal left ventricular function. Stenosis (gradient >30 mmHg) was noted in the pulmonary artery reconstruction in seven patients (5%). More than mild aortic regurgitation was noted in five patients (4%). The incidence of redo or reintervention was 5% at 5 years of follow-up. CONCLUSIONS: In the current era a prolonged learning curve for ASO is not acceptable to most European countries and their patients. The risk of surgery can be minimized by concentrating surgical experience as part of the quality control of congenital heart programs. If the number of new patients is small due to the birth rate and size of the population, institutions should merge activity. Such centralization amplifies the experience to the benefit of the patient.

Flow cytometric analysis of some activation/proliferation markers on human thymocytes and their correlation with cell proliferation.

We immunophenotyped cells from ten human thymuses with emphasis on expression of the CD38 and CD71 antigens. These antigens play role in activation cells and increased expression of them was observed in some leukemia. Simultaneously, certain attention has also been devoted to some further activation markers, e.g. CD25, CD26 and HLA-DR. The classification of leukemia is based on comparison of normal and pathological cells. The study of expression of CD38, CD71 and other markers on thymocytes simultaneously with DNA analysis can be useful for answer if expression of CD38 and CD71 on pathologic cells is a sign of their proliferative ability, a part of immature phenotype in some leukemia, or it is a case of aberrant immunophenotype. In our study, 94% thymocytes were CD38+ and only 16% were CD71+. From our immunophenotypic results including MESF (molecules of equivalent soluble fluorochrome) values and analysis of the cell cycle, the conclusion could be drawn that antigen CD71 can participate in regulation of thymocyte development and presence of both -CD38 and CD71 on pathologic cells will be in all probability the case of aberrant phenotype. We observed a clear correlation of the percentage and MESF values of CD71-positive cells with the cell proliferation only after in vitro thymocytes stimulation with PHA and IL-2. In summary, a strong parallelism was observed regarding the positive relationship between the proliferative rate (assessed by the number of S-phase cells) of stimulated thymocytes and the quantitative (% and MESF) values of some markers - CD71, CD25, CD26 and HLA-DR and negative one with CD38 marker values.

[Late postoperative complications after primary treatment of aortic coarctation]. / Neskoré pooperacné komplikácie po primárnom riesení koarktácie aorty.

During the period between 1963 and 1988, 343 patients were operated due to aortic coarctation. 14 patients (4%) were necessarily reoperated for late postoperative complications. In 3 cases (0.8%) reoperation was indicated due to the development of aneurysm in the region of primary operation of aortic coarctation. The development of aneurysm is prevalently predisposed by Vosschulte's indirect aortoplastic operation by the use of a synthetic patch. In the remaining 11 patients (3.2%) the reoperation was indicated owing to the development of recoarctation. Today recoarctation concerns children which were operated under the age of two. The most important moment favourable for the recoarctation development is the secondary constriction of the repaired region in consequence of the coarctation listel fibroid tissue and owing to the incapability of the anastomosis to grow. The perspective rests on using new suture material and combined techniques. Late postoperative complications which follow operations performed due to aorta coarctation remarkably augment the peri and postoperative mortality. Three cases out of the given group of 14 patients terminated by exitus (21%). Hence, it is necessary to remove all moments of predisposition creating a condition favourable for the development of recoarctation and aneurysm. (Tab 2, Fig. 1, Ref. 14.).

Improved patient survival following surgery for coarctation of the aorta.

BACKGROUND: We conducted a retrospective review of children undergoing surgery for coarctation of the aorta in our institution over the last ten years with the aim of evaluating overall patient survival as well as detecting factors affecting it. We tried to identify the risk factors for mortality. METHODS AND DATA: Between January 1992 and December 2001, 201 patients with aortic coarctation were operated on at the Department of Cardiac Surgery of the Children's University Hospital, Bratislava. The three classes of aortic coarctation were represented: isolated coarctation, coarctation with ventricular septal defect (VSD) and coarctation with complex cardiac anomalies. Patients' preoperative, operative and immediate postoperative medical records were carefully studied with special attention paid to the type of lesion, patients' preoperative state, type of surgical technique employed, as well as the period of operation. For comparison, two equal time periods of follow-up were reviewed--1992 to 1996 and 1997 to 2001. The overall postoperative conditions of patients were also regularly monitored. Patient data were statistically analyzed using the JMP program version 4.04. RESULTS: An overall survival of 90% was recorded over the period of follow-up, ranging between one and ten years. A further break down showed a statistically significant difference between the various types of aortic coarctation, p=0.0001. Patients with simple or isolated coarctation had a survival rate of 100%, those with ventricular septal defect (VSD) in addition to coarctation had a survival rate of 80% while patients with associated complex cardiac anomalies had a survival rate of 65%. An improvement on overall patient survival was recorded in the period between 1997 and 2001--96% as against 86% for the period between 1992 and 1996. On univariate statistical analysis, the following variables were identified as significant risk factors for death: 1) Complex cardiac anomalies (p<0.0001), 2) Age at operation less than one month (p<0.0001) and 3) Treatment prior to the year 1997 (p=0.02). CONCLUSION: A considerable improvement on patient survival following surgery for coarctation of the aorta was recorded over the last five years. This could be attributed to new measures in preoperative, operative and postoperative care for patients with aortic coarctation. (Tab. 4, Fig. 5, Ref. 8.).