Joachim Photiadis

Functional outcome of anatomic correction of corrected transposition of the great arteries

OBJECTIVE Anatomic correction of corrected transposition of the great arteries, utilizing the morphologic left ventricle as a systemic pumping chamber, is considered the preferred method. The purpose of the study was to analyze the intermediate functional outcome following anatomical correction. METHODS Between 1997 and 6/2010, 23 patients with corrected transposition of the great arteries and associated lesions underwent anatomical correction. Seventeen (74%) and six patients (26%) had situs solitus {S,L,L} and situs invs {I,D,D}, respectively. Fifteen patients (65%) had undergone 18 palliations before the corrective operation. The median age at palliation was 0.23 years, with a range of 0.016-8.4 years. A corrective, modified Senning-arterial switch procedure was performed in nine patients, 13 patients underwent a modified Senning-Rastelli procedure, and in one patient a combination of modified Senning and aortic translocation (Bex/Nikaidoh) was used. The median age at the corrective operation was 2 years (from 0.3 to 15.7 years). RESULTS There was no mortality or heart transplant within the mean follow-up of 3.4 years. Freedom from reintervention was 77% at 5 years. There were no signs of obstruction of the systemic and pulmonary venous tunnels. The function of both ventricles was normal in all patients, even in the four patients who required retraining of the left ventricle. Mild aortic regurgitation was noticed in three patients. Preoperatively detected significant tricuspid regurgitation either disappeared or became trivial after the operation in all the six patients. All patients except two are in sinus rhythm; one patient is pacemaker-dependent preoperatively and one is pacemaker-dependent postoperatively. There were no clinically apparent neurological problems. All patients, but one, are in the New York Heart Association (NYHA) class I. CONCLUSIONS Anatomic correction of corrected transposition of the great arteries can be performed in selected patients without mortality and with acceptable morbidity. The mid-term functional outcome is excellent, resulting in normal ventricular function, even in retrained left ventricles, and minimal incidence of complete heart block. The long-term function of the aortic valve, intraventricular tunnels, conduits, and ventricles requires close surveillance.

The Long-Term Outcome of Open Valvotomy for Critical Aortic Stenosis in Neonates

BACKGROUND This study analyzed the long-term outcome after operations for open aortic valvotomy, specifically looking at the preservation of the native aortic valve. METHODS Between 1983 and 2011, 34 neonates with critical aortic stenosis underwent open valvotomy. The mean age at operation was 15 days (range, 1 to 28 days). Follow-up was 100% complete. RESULTS Three patients died early. The probability of survival was 91.2%, with a mean follow-up of 11 years (range, 10 months to 21 years). Event-free survival was 77%, 68%, 68%, and 57% at 5, 10, 15, and 20 years, respectively, and freedom from aortic valve replacement was 93%, 83%, 68%, and 57%. Tricuspid valve morphology revealed the best outcome, with event-free survival of 90% (p = 0.006) and 100% freedom from aortic valve replacement (p = 0.0012) at 20 years of follow-up. All but 4 patients were in New York Heart Association class I; 77 % of all patients were without medication. CONCLUSIONS In neonates, predictable and consistent long-term results can be accomplished by open valvotomy in any type of valve morphology, even in an asymmetric arrangement. Operative repair not only offers a 90% survival benefit in the long-term but also preserves the native aortic valve in most patients. Clearly superior results were achieved in tricuspid valve morphology, in which there was no need for aortic valve replacement at 20 years of follow-up.

Surgical management of congenital heart disease: evaluation according to the Aristotle score.

OBJECTIVE The Aristotle basic complexity (ABC) score (1.5-15 points) is the sum of potentials for early mortality, morbidity and anticipated surgical technique difficulty. The Aristotle comprehensive complexity (ACC) score (1.5-25 points) is the sum of ABC score and patient-adjusted complexity score; it comprises six complexity levels. We used the ACC score to evaluate quality in surgical management of congenital heart disease. METHODS Procedures performed in year 2002 and 2007 were analysed. Proportion of procedures requiring at least 1 week of stay in the intensive care unit was chosen as the marker of morbidity. We adopted threshold duration of 120 min for cardio-pulmonary bypass (CPB) cases and the same duration for operations without CPB as surrogate of surgical technical difficulty. The ACC scores were correlated to mortality, morbidity and technical difficulty. RESULTS This study included 758 patients who underwent 787 primary procedures. The mean ABC and ACC scores amounted to 7.61+/-2.46 and 9.51+/-3.84. Early mortality was 3.05% (24/787), 95% confidence interval (CI): 1.97-4.51%. Zero at ACC levels 1 and 2, it increased from 1.2% (2/161) for level 3 up to 22.2% (2/9) for level 6. Morbidity index was evaluated at 25.9% (204/787), 95% CI: 22.9-29.1%. 1.9% at level 1, it escalated up to 77.8% at level 6. Index of technique difficulty was estimated at 35.2% (277/787), 95% CI: 31.8-38.6%, ranging from 4.8% for level 1 to 66.7% for level 6. A high correlation was found between the ACC scores and mortality, indices of morbidity and technique difficulty, Spearmans correlation coefficient r being 0.9856, 1 and 0.9429, respectively. Mortality (p=0.037) and morbidity (p=0.041) were lower in year 2007 than in 2002 with ABC (p=0.18) and ACC (p=0.37) surgical performance being not significantly different. CONCLUSIONS The Aristotle score is still under development. Morbidity evaluation should be ideally based on observed postoperative complications; estimation of surgical technical difficulty chosen in this study may not be generalised. Nevertheless, the actual Aristotle comprehensive complexity score, as evaluated in its three components, accurately determined the outcome of surgical management of congenital heart disease. It appears to be an adequate tool to evaluate quality in paediatric cardiac surgery, over time.

Implantation of stents as an alternative to reoperation in neonates and infants with acute complications after surgical creation of a systemic-to-pulmonary arterial shunt.

Creation of a systemic-to-pulmonary shunt is still the firstline treatment in neonates with duct-dependent pulmonary circulation, or in patients with hypoplastic left heart syndrome as a part of the first stage of the Norwood sequence. Acute complications after such surgery, such as stenosis, thrombosis, or kinking, are potentially lifethreatening. These complications require immediate revision or exchange of the shunt. In this report, we discuss interventional treatment as an alternative to surgery in 5 patients with acute stenosis or complete occlusion of a shunt. The age of the patients ranged from 12 to 62 days, with a median of 30 days, and their weights ranged between 2.2 and 4.6 kilogrammes, with a median of 3.2 kilogrammes. In 3 patients, the shunts were central or of modified Blalock-Taussig type, while the 2 patients with hypoplastic left heart syndrome had shunts from the right ventricle to the pulmonary arteries. We implanted 6 coronary arterial and 2 peripheral stent systems. The diameter of the balloon used for implantation had a ratio to the shunt of 0.9. to 1. All shunts were successfully reopened by stenting. During follow-up, 3 patients underwent further procedures on an elective basis. We have one patient on the waiting list for further surgical intervention, but one patient died of septicaemia unrelated to the interventional procedure one month after implantation. In our limited experience, implantation of stents is an effective and long-lasting treatment for complications of shunts in an emergency situation.

Tranexamic acid: an alternative to aprotinin as antifibrinolytic therapy in pediatric congenital heart surgery

OBJECTIVE There has been concern about the usage of aprotinin, an antifibrinolytic drug that was often used in pediatric cardiac surgery until 2006. At our center, these concerns led to the replacement of aprotinin with tranexamic acid for antifibrinolytic treatment. METHODS In this retrospective observational study, two groups of pediatric patients were studied during two different periods, receiving either aprotinin (n=70) or tranexamic acid (n=70) upon cardiac surgery. Data were collected from children with cyanotic heart defects, children who weighed less than 10 kg, and children who underwent re-operation. RESULTS There was no difference in terms of blood loss or amount of erythrocyte concentrates and fresh frozen plasma transfused. Only the intraoperative amount of platelet concentrate received by children in the tranexamic acid group was 29 ml (p=0.013) higher. There was no significant difference in the length of stay at the intensive care unit, in renal function values, or in the rate of rethoracotomy. CONCLUSIONS The results of this study suggest that tranexamic acid represents an adequate alternative to aprotinin in congenital cardiac surgery.

Does bilateral pulmonary banding in comparison to Norwood procedure improve outcome in neonates with hypoplastic left heart syndrome beyond second-stage palliation? A review of the current literature.

Best evidence protocol was applied and the question addressed, whether there is an outcome advantage of a hybrid approach including bilateral pulmonary banding (BPB) and ductal stenting over Norwood procedure (NP) to maintain systemic circulation and to restrict pulmonary blood flow in neonates with hypoplastic left heart syndrome (HLHS). Out of 80 articles published since the original description of BPB in 2002, eight were selected to answer the clinical question. All studies were retrospective case-series after BLB, only three compared results with NP controls, but not in a randomized fashion. Only three chose high-risk patients. Mean follow-up interval was mentioned only in one study. Reported mean hospital, interstage, and stage 2 mortalities after BPB was 17.3, 12.4, and 25.3%, respectively. Estimated transplant-free survival beyond stage 2 palliation ranged from 7 to 80%, with no significant differences to NP controls in three studies. Reintervention rate was high, ranging from 20 to 42% at different sites. Due to study design or inhomogeneity of patient groups, current literature does not show evidence that bilateral pulmonary banding improves outcome with respect to survival beyond second-stage HLHS palliation.

Survival and reintervention after neonatal repair of truncus arteriosus with valved conduit

OBJECTIVE Neonatal primary repair has progressively become the treatment of choice for truncus arteriosus with encouraging survival. However, use of valved conduits to reconstruct the right ventricular outflow tract (RVOT) inevitably induces reintervention. This study estimates survival and rate of catheter-interventional and surgical reinterventions. METHODS Thirty-five consecutive neonates who underwent truncus repair with 27 homografts and 8 Contegras from 1987 to 2007 were studied. Interrupted aortic arch (IAA) was associated in nine patients. Actuarial survival and freedom from reintervention were evaluated according to Kaplan-Meier method. RESULTS Five patients died early after repair. Two died late and one death was related to reintervention. Survival was 91.9%+/-5.4% from postoperative month 2 onwards when IAA was not associated and 41.7%+/-17.3% from month 4 in IAA presence. During a median follow-up of 68 months (range 1-180 months), 42 reinterventions (of which 17 reoperations) were performed in 21 patients. Rate of reintervention was 2.6 per early survivor per 10 years. RVOT obstruction constituted the main indication: branch pulmonary arteries often being involved (n=25). Uncommon indication was subaortic stenosis (n=3), aortic arch obstruction (n=2) and truncal valve regurgitation (n=2). At year 10, freedom from first, second and third reintervention was 17.9 %+/-8.1%, 46.1%+/-10.6% and 81.9%+/-9.5%, respectively. Sixteen first conduits were explanted. Freedom from first conduit replacement was 87.5%+/-6.8%, 64.1%+/-10.2% and 39.5%+/-10.7% at year 1, 3 and 5, respectively. Homografts enjoyed higher durability than Contegras. CONCLUSION Neonatal repair of truncus arteriosus results in high survival, the only risk being IAA association. The rate of reintervention is heavily influenced by stenosis of branch pulmonary arteries.

Does the shunt type determine mid-term outcome after Norwood operation?

OBJECTIVES With improved short-term outcomes the right ventricular to pulmonary artery shunt (Sano) has become the preferred pulmonary blood source in the Norwood procedure in many centres. However, most studies analysed consecutive cohorts, with a first modified Blalock-Taussig shunt (BT) followed by the Sano cohort. Besides, neither comprehensive preoperative risk analysis nor outcome beyond 1 year of age was investigated. METHODS This study reviews 109 neonates undergoing the Norwood procedure in the same interval between October 2002 and December 2009. The Sano (38) or BT shunt (71) was assigned according to the surgeons preference. Two neonates subsequently underwent successful biventricular repair and were excluded. The Aristotle comprehensive score (ACS) was used to evaluate preoperative risk, with high-risk patients (n = 39) classified as having an ACS ≥ 20, and low-risk patients (n = 68) given an ACS <20. RESULTS Mean Aristotle score at the Norwood operation was 18.8 ± 0.4 and 18.9 ± 0.3 (P = 0.9) in Sano and BT, respectively. Mean follow-up interval was 4.1 ± 2.1 years (range: 1.7-8.9 years). Actuarial survival was similar, stabilizing from the 8th postoperative month onwards at 78.6 ± 4.9% (95% CI: 67.0-86.5%) for Sano and 78.4 ± 6.8% (95% CI: 61.4-88.6%) for BT; P = 0.95. Midterm actuarial survival was higher in low-risk patients, 88.2 ± 3.9% (95% CI: 77.8-93.9%) than in high-risk patients: 61.5 ± 7.8% (95% CI: 44.5-74.7%, P = 0.0003). No survival benefit was detected in low- or high-risk cases for either shunt type. Risk factors for midterm mortality were cardiorespiratory failure requiring ventilation (13/34, P = 0.004), and ACS ≥ 20 (15/39, P = 0.001), but not shunt type (8/37, P = 0.95). Increased number of shunt-related interventions before the Glenn procedure were noted with Sano (32.4 versus 6.5%, P = 0.002). CONCLUSIONS Preoperative risk factors, regardless of shunt type, influence midterm survival after the Norwood procedure with an excellent outcome in low-risk patients, while high-risk cases still incur a significant mortality. Sano shunt interventions occurred with increased numbers. Although, Sano shunt may be the only feasible option in some instances, given the possible negative effects of ventriculotomy on right ventricle function, the widespread use of Sano shunt should be reconsidered.

A Novel Approach to the Repair of Tetralogy of Fallot With Absent Pulmonary Valve and the Reduction of Airway Compression by the Pulmonary Artery

Controversy persists regarding the management of patients suffering from tetralogy of Fallot with absent pulmonary valve syndrome. Airway obstruction caused by the dilated pulmonary arteries is the determining factor in the mortality of symptomatic newborns and infants. A number of surgical techniques for the reduction of bronchial obstruction have been proposed, producing variable results. All strategies have focused on plication and reduction of the anterior or posterior wall of the normally positioned pulmonary arteries, with or without pulmonary valve replacement. An alternative approach is to bring the pulmonary arteries anterior to the aorta and away from the trachea and bronchial tree. This technique has the potential to reduce or eliminate bronchial compression by the central pulmonary arteries. Failure of the treatment could be expected in symptomatic patients, where the pathology of the airways extends beyond the proximal pulmonary arteries.

Poor outcome for patients with totally anomalous pulmonary venous connection and functionally single ventricle

Totally anomalous pulmonary venous connection, when also associated with a functionally univentricular connection, is known to have a poor outcome. We retrospectively analysed results for 19 patients undergoing surgery for this combination of lesions between 1995 and February 2009.Of the patients, 12 were neonates, with 11 presenting with signs of pulmonary venous obstruction. In 3 patients, a modified Blalock-Taussig shunt had been constructed. The dominant ventricle was of right ventricular morphology in 17 of the 19 patients, and double inlet was present in all bar 1. Pulmonary atresia or stenosis was found in 14 patients, a common atrioventricular junction in 14 patients, and isomerism of the right atrial appendages in 12 patients, respectively. Comprehensive Aristotle scores ranged from 14 to 23.50. The mean was 16.55, with a standard deviation of 2.19. Pulmonary venous rerouting was combined in 6 patients with construction of a modified Blalock-Taussig, in 4 with banding of the pulmonary trunk, in another 4 with a bidirectional Glenn anastomosis, in 3 with creation of a total cavo-pulmonary connection, and in 1 each with enlargement of the right ventricular outflow tract and the Norwood procedure. Of the cohort, 8 patients died early due to pulmonary hypertension, with all patients having Aristotle scores of at least 18 points dying. Among the 11 early survivors, 5 needed mechanical ventilation for over a week, but 5 patients died later, 3 due to pulmonary hypertension and 2 due to infection. Actuarial survival stabilized at 31.6%, with standard error of 10.7%, from one year onwards. Conversion to the Fontan circulation was successful in 5 patients.Our experience confirms that totally anomalous pulmonary venous connection, when associated with the functionally univentricular arrangement, carries one of the worst outcomes in current surgical practice. Use of the Aristotle comprehensive complexity scores effectively discriminates those patients with this condition at particularly high risk.