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ABSTRACT: The aim of study was to analyse the mid-term results of the Ross-Konno procedure in infants.
Between 2000 and 2011, 16 infants, including five newborns, with complex left ventricular outflow tract (LVOT) obstruction underwent the Ross-Konno procedure. Twelve patients (75%) required multiple concomitant procedures such as: mitral valve (MV) surgery (four patients), resection of endocardial fibroelastosis (EFE) and myectomy (six patients), closure of ventricular septal defect (four patients) and aortic arch reconstruction (three patients). The median age at operation was 4.2 months (from 6 to 333 days).
There was one late death with a median follow-up of 6.2 years. Actuarial survival is 93.3% at 5 years follow-up (95% confidence interval: 61.2-99.0). Postoperatively, two patients required extracorporeal membrane oxygenation support and one MV replacement. The median length of stay in hospital was 30 days (from 11 to 77 days). At 5 years of follow-up, seven patients had no aortic regurgitation (AR) and nine patients (56%) had trivial AR with no gradient in LVOT. Freedom from mitral regurgitation (MR) ≥ moderate or MV replacement was 70%. MR was associated with either structural abnormalities of MV or with development of EFE. Freedom from redo was 81 and 53% at 1 and 5 years of follow-up. Sixty percent of patients are without medication. All patients are in sinus rhythm.
With the technical aspects of this procedure well accomplished, the risk of surgery is minimal and functional outcome is encouraging. However, early postoperative morbidity is significant. At the mid-term follow-up, there was no residual or recurrent outflow tract obstruction and excellent function of the neoaortic valve. A high incidence of MR associated with the development of EFE and structural abnormalities of the MV is worrisome; however, concomitant MV surgery is not associated with increased mortality. In the case of the development of EFE, an early indication for operation might protect MV function. The reoperation rate is high due to early conduit failure.
European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery 10/2012; 42(4):687-94. · 2.40 Impact Factor
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ABSTRACT: Objective The objective of this study was to estimate the morbidity according to observed complications after congenital heart surgery over 1-year period.Methods The previously established list of conditions prone to affect patients' well-being or increase cost of in-hospital stays was used systematically to score the severity of postoperative complications from 1 to 4 points. The morbidity score was calculated by adding the scores of observed complications. When the sum amounted to more than 5 points, a morbidity score of only 5 points was attributed. If no complication was detected, a score of 0.5 points was assigned. The resulting morbidity scores were correlated with the length of stay in the intensive care unit (ICU) and in the hospital, the duration of mechanical ventilation, and Aristotle complexity scores.Results A total of 542 primary procedures performed in the year 2011 were studied. Aristotle basic and comprehensive scores amounted to 7.78 ± 2.65 and 10.15 ± 3.83, respectively. Mortality was 1.85% (10/542). The standardized ratio of surgical performance reached 103.10%. Total cavopulmonary connection with extracardiac fenestrated conduit constituted the most frequent operation (n = 34). No complication was observed following 183 (33.8%) procedures. More than two complications were observed in 114 cases (21%). The three most frequent unfavorable conditions were "mechanical ventilation 25 to 95 hours" (n = 150), low cardiac output syndrome (n = 56), and cardiac arrhythmia requiring medication (n = 50). The estimated mean morbidity score amounted to 2.26 ± 1.80 points. Scores ranged from 0.68 ± 0.50 for primary closure of atrial septal defect to 4.50 ± 0.79 for the Norwood procedure. They were perfectly related to the length of ICU stay and to the duration of mechanical ventilation (Spearman coefficient r = 1). Correlation was high with the length of hospital stay (r = 0.83), Aristotle basic score (r = 0.89) (C-index = 0.96), and comprehensive score (r= 0.94) (C-index= 0.97). The observed mean morbidity score was statistically not different from the expected mean morbidity score according to the basic Aristotle complexity: p = 0.73.Conclusion Quantification of morbidity indicates the length of ICU stay and the duration of mechanical ventilation as the best surrogates for morbidity. Such benchmarking and scoring of observed postoperative complications paves the way for an accurate assessment and improvement of quality care in congenital heart surgery.
The Thoracic and Cardiovascular Surgeon 09/2012; · 0.88 Impact Factor
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ABSTRACT: Improved results have evolved from the modified Norwood procedure (NP). This study compares the incidence of interventions after NP with the Sano (n = 37) and modified Blalock-Taussig (BT n = 70) shunt. Incidence, location, interval of interventions, and weight were retrospectively analysed for 107 neonates undergoing NP during the period from October 2002 to December 2009. Forty-six (43.0 %) patients underwent interventions, mostly for dilatation of the aortic arch ([DAA] n = 26 [24.3 %]; Sano n = 10, BT n = 16, p = 0.6), dilatation of the shunt ([DS] n = 15 [14.0 %]; Sano n = 11, BT n = 4; p = 0.002), or closure of aortopulmonary collaterals ([APC] n = 15 [14.0 %]; Sano n = 3, BT n = 12; p = 0.08). Mean interval after NP and body weight at DAA, DS, and APC were 72.4 ± 18.9, 108.5 ± 15.8, and 110.7 ± 17.8 days and 4.5 ± 1.3, 4.9 ± 1.9, 5.3 ± 1.2 kg, respectively. The interventions were not associated with mortality but with a greater rate of complications (9 of 46 [21.4 %]) compared with the rate after diagnostic catheterization (0 of 45, p = 0.03). Complications included closure of the femoral or subclavian artery (n = 5), cerebral embolic or bleeding events (n = 4), cardiopulmonary resuscitation (n = 3), and temporary heart block (n = 2). Actuarial survival was similar from the postoperative month 8 onward at 78.6 ± 4.9 % (95 % confidence interval [CI] 67.0-86.5 %) for Sano and 78.4 ± 6.8 % (95 % CI 61.4-88.6 %) for BT (p = 0.95). Interventions after NP were common irrespective of shunt type. However, a significantly greater rate of shunt interventions was noted in the Sano group. In particular, interventions addressing the aortic arch and the shunt were related with a significant rate of complications.
Pediatric Cardiology 06/2012; · 1.30 Impact Factor
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ABSTRACT: This study analyzed the long-term outcome after operations for open aortic valvotomy, specifically looking at the preservation of the native aortic valve.
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.
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.
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.
The Annals of thoracic surgery 05/2012; 94(5):1519-26. · 3.74 Impact Factor
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ABSTRACT: 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.
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 surgeon's 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.
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).
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.
European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery 02/2012; 42(2):209-15; discussion 215-6. · 2.40 Impact Factor
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ABSTRACT: In paediatric patients with a pacemaker (PM) or an implantable cardioverter defibrillator (ICD) device, interrogation during in-clinic visits is usually required to obtain information on arrhythmias or system failures. An automated telemonitoring system tracking patient- and system-related parameters provides this information on a daily basis and might assist patient management.
This retrospective analysis evaluates telemetric data obtained from 48 devices implanted in 45 patients (median age, 12.4 years; range, 5 weeks to 37.6 years) using an automated system [Home Monitoring (HM)] over the last 5 years. Regular transmissions were received on 72% of all days. The event messages and monitoring data of 34 (71%) devices induced system revisions, electrophysiological studies including ablation, programming changes, medication changes, and alterations in sporting activity. Approximately 47% of event messages and 50% of emergency messages arrived within 4 weeks of implantation or latest outpatient visit; 113 (17%) emergency messages reported acute changes in lead parameters or tachycardia, which required medical intervention. In four ICD patients, delivered shocks were unreported and only detected by HM.
An automated telemonitoring system reporting patient- and system-related parameters, generated within the patient's home surrounding, can improve the safety and quality of PM and ICD therapy, especially in children, by allowing early detection of system failure and changes in arrhythmic events.
Europace 02/2012; 14(7):1030-7. · 1.98 Impact Factor
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ABSTRACT: Patients with combined aortopulmonary window (APW) and interrupted aortic arch (IAA) malformations are rarely seen. We reviewed cases with such association with emphasis on surgical management and long-term outcome.
109 patients with IAA and 25 patients with APW were operated upon between 1981 and 2011. The clinical records, operation and follow-up data were analyzed. Long-term outcome was completed with the help of either outpatient data or inquiring. Related literature was investigated.
Combined APW/IAA was found in 8 cases: 7.3% (8/109) of those with IAA diagnosis and 32% (8/25) with APW. All APWs were proximal. 7 patients had interruption type A. A ventricular septal defect (VSD) was associated in one case only. Median age at surgery was 10 days. The two first patients were operated upon without cardio-pulmonary bypass (CPB) and one died during operation. The other six underwent single stage approach under CPB with no death: overall early mortality of 12.5% (⅛). APW was closed with one or two patches; aortic arch was reconstructed either directly (extended end-to-side anastomosis) or by patch augmentation. There was no late death and no reoperation during mean follow-up of 118 months (range 1-360 months). Six patients were in functional NYHA class I, the remainder in class II. The cumulative APW/IAA incidence from literature varies between 4.9% (56/1105) and 22.2% (42/189). Early mortality reaches 15.1% (8/53) (95% CI: 6.8% - 27.6%).
Neonatal repair with thorough mobilization of the aortic arch and extended end-to-side anastomosis or use of patch augmentation carries potential for the best early and late outcome for combined APW/IAA malformation.
The Thoracic and Cardiovascular Surgeon 01/2012; 60(3):215-20. · 0.88 Impact Factor
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ABSTRACT: 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.
The Thoracic and Cardiovascular Surgeon 01/2012; 60(3):181-8. · 0.88 Impact Factor
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ABSTRACT: The aim of this study was to develop a morbidity score based on observed postoperative complications after congenital heart surgery.
Conditions or diseases that impair patients after congenital heart surgery or increase costs of hospital stay were called complications and attributed to scores ranging from 1 (mild) to 4 (severe) points, according to estimated severity or costliness. 'No complication' was assigned 0.5 points. From January to March 2011, scores for each observed 'complication' for every main (primary) surgical procedure were recorded and a morbidity score was calculated. In conformity with the Aristotle score methodology, if the sum of observed complication scores amounted to more than 5 points, a morbidity score of only 5 points was attributed. The estimated morbidity score was compared with the morbidity score attributed by the Aristotle basic complexity (ABC) score.
One hundred and thirty-nine primary procedures were carried out. The mean ABC and Aristotle comprehensive complexity scores reached 8.31 ± 2.52 and 9.62 ± 3.47 points, respectively. Two patients died. No complication was detected after 46 procedures. Overall, there were 185 listed 'morbidity' conditions in connection with the other 93 surgical procedures, rendering a total score of 385 points. The most frequent event was 'mechanical ventilation 25-95 h': n = 39. The mean morbidity score was 2.14 ± 1.63. The morbidity scores ranged from 0.5 points (n = 46) to 5 points (n = 23) with a median of 2.0 points. The scores for 11 different procedures that were performed at least five times positively correlated with the corresponding Aristotle morbidity scores: Pearson's coefficient r = 0.75. But the morbidity score for bidirectional cavopulmonary anastomosis (3.14 ± 1.77) was higher than the corresponding Aristotle morbidity score (2.0). It was lower for 'conduit placement, right ventricle to pulmonary artery': 1.08 ± 0.97, versus 2.0, and for arterial switch operation: 2.08 ± 1.11, versus 3.0.
The reported morbidity scores need to be tested on larger series and in different institutions. The introduced morbidity score has the potential to quantify postoperative complications accurately. Its estimation over time can facilitate the assessment of quality of congenital heart surgery. It will allow comparison of morbidity outcomes across institutions with different case-mixes.
European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery 12/2011; 41(4):898-904. · 2.40 Impact Factor
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ABSTRACT: Planning and budgeting for congenital heart surgery depend primarily on how closely reimbursement matches costs and on the number and complexity of the surgical procedures. Aristotle complexity scores for the year 2010 were correlated with hospital costs and with reimbursement according to the German diagnosis-related groups (DRG) system. Unit surgical performance was estimated as surgical performance (complexity score × hospital survival) times the number of primary procedures. This study investigated how this performance evolved during years 2006 to 2010. Hospital costs and reimbursements correlated highly with Aristotle comprehensive complexity levels (Spearman r = 1). Mean costs and reimbursement reached 35,050 ± 32,665 and 31,283 ± 34,732 , respectively, for an underfunding of 10.7%. Basic and comprehensive unit surgical performances were respectively 3036 ± 1009 and 3891 ± 1591 points in 2006. Both performances increased in sigmoid fashion to reach 3883 ± 1344 and 5335 ± 1314 points, respectively, in 2010. Top performances would be achieved in year 2011, and extrapolated costs would comprise about 19,434,094.92 (95% confidence interval, 11,961,491.22-22,495,764.42 ). The current underfunding of congenital heart surgery needs correction. The Aristotle score can help to adjust reimbursement according to complexity of procedures. Unit surgical performance allows accurate budgeting in the current German DRG system.
Pediatric Cardiology 07/2011; 33(1):36-41. · 1.30 Impact Factor
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ABSTRACT: 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.
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.
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.
The results of this study suggest that tranexamic acid represents an adequate alternative to aprotinin in congenital cardiac surgery.
European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery 04/2011; 39(4):495-9. · 2.40 Impact Factor
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ABSTRACT: 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.
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).
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.
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.
European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery 03/2011; 40(5):1227-34. · 2.40 Impact Factor
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ABSTRACT: Aristotle score methodology defines surgical performance as 'complexity score times hospital survival'. We analysed how this performance evolved over time and in correlation with case volume.
Aristotle basic and comprehensive complexity scores and corresponding basic and comprehensive surgical performances were determined for primary (main) procedures carried out from 2006 to 2009. Surgical case volume performance described as unit performance was estimated as 'surgical performance times the number of primary procedures'.
Basic and comprehensive complexity scores for the whole cohort of procedures (n=1828) were 7.74±2.66 and 9.89±3.91, respectively. With an early survival of 97.5% (1783/1828), mean basic and comprehensive surgical performances reached 7.54±2.54 and 9.64±3.81, respectively. Basic surgical performance varied little over the years: 7.46±2.48 in 2006, 7.43±2.58 in 2007, 7.50±2.76 in 2008 and 7.79±2.54 in 2009. Comprehensive surgical performance decreased from 9.56±3.91 (2006) to 9.22±3.94 (2007), and then to 9.13±3.77 (2008), thereafter increasing up to 10.62±3.67 (2009). No significant change of performance was observed for low comprehensive complexity levels 1-3. Variation concerned level 4 (p=0.048) which involved the majority of procedures (746, or 41% of cases) and level 6 (p<0.0001) which included a few cases (20, or 1%), whereas for level 5, statistical significance was almost attained: p=0.079. With a mean annual number of procedures of 457, mean basic and comprehensive unit performance was estimated at 3447±362 and 4405±577, respectively. Basic unit performance increased year to year from 3036 (2006, 100%) to 3254 (2007, 107.2%), then 3720 (2008, 122.5%), up to 3793 (2009, 124.9%). Comprehensive unit performance also increased: from 3891 (2006, 100%) to 4038 (2007, 103.8%), 4528 (2008, 116.4%) and 5172 (2009, 132.9%).
Aristotle scoring of surgical performance allows quality assessment of surgical management of congenital heart disease over time. The newly defined unit performance appears to well reflect the trend of activity and efficiency of a congenital heart surgery department.
European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery 01/2011; 39(4):e33-7. · 2.40 Impact Factor
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ABSTRACT: The Disease-Related Groups (DRGs) system postulates that inpatient stays with similar levels of clinical complexity are expected to consume similar amounts of resources. This, applied to surgery of congenital heart disease, suggests that the higher the complexity of procedures as estimated by the Aristotle complexity score, the higher hospital reimbursement should be. This study analyses how much case-mix index (CMI) generated by German DRG 2009 version correlates with Aristotle score.
A total of 456 DRG cases of year 2008 were regrouped according to German DRG 2009 and related cost-weight values and overall CMI evaluated. Corresponding Aristotle basic and comprehensive complexity scores (ABC and ACC) and levels were determined. Associated surgical performance (Aristotle score times hospital survival) was estimated. Spearman 'r' correlation coefficients were calculated between Aristotle scores and cost-weights. Goodness of fit 'r(2)' from derived regression was determined. Correlation was estimated to be optimal if Spearman 'r' and derived goodness of fit 'r(2)' approached 1 value.
CMI was 8.787 while mean ABC and ACC scores were 7.64 and 9.27, respectively. Hospital survival was 98.5%: therefore, surgical performance attained 7.53 (ABC score) and 9.13 (ACC score). ABC and ACC scores and levels positively correlated with cost-weights. With Spearman 'r' of 1 and goodness of fit 'r(2)' of 0.9790, scores of the six ACC levels correlated at best. The equation was y = 0.5591 + 0.939x, in which y stands for cost-weight (CMI) and x for score of ACC level.
ACC score correlates almost perfectly with corresponding cost-weights (CMI) generated by the German DRG 2009. It could therefore be used as the basis for hospital reimbursement to compensate in conformity with procedures' complexity. Extrapolated CMI in this series would be 9.264. Modulation of reimbursement according to surgical performance could be established and thus 'reward' quality in congenital heart surgery.
European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery 02/2010; 37(6):1271-6. · 2.40 Impact Factor
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ABSTRACT: 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.
Cardiology in the Young 10/2009; 19(6):594-600. · 0.76 Impact Factor
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ABSTRACT: Paediatric cardiac surgery is often performed under hypothermic conditions, that is, with a reduced core body temperature. Certain interventions even require the circulation to be stopped. This can only be done at a body temperature of 18 degrees C, with no risk of neurological damage and harm to the brain and other organs. Vasoconstriction is a natural reaction of the body to cold, causing the blood vessels to contract. Such a reaction would lead to a clear rise in blood pressure on cardiopulmonary bypass (CPB). Since the blood pressure is regulated in the arteriolar loop of the capillary system, there is a marked increase in blood pressure and a suppression of free water into the surrounding tissue, which, in turn, may lead to the intra-operative development of oedemas. This study aimed to investigate whether the high-flow, low-resistance (HFLR) technique offers any benefits over conventional methods.
This open, prospective, randomised study was to recruit 48 children scheduled to undergo surgery for congenital heart disease. To investigate the two different perfusion strategies, we have measured intestinal perfusion as well as skin perfusion with laser Doppler spectroscopy. To identify the effects on the immune system, selected immunologic parameters of systemic inflammation were additionally measured. Laser Doppler spectroscopy is a method that uses a glass fibre probe to determine the parameters of oxygen saturation of haemoglobin and relative haemoglobin quantity in an illuminated tissue volume, as well as the perfusion parameters of relative blood flow and blood flow velocity in the sample volume of the probe.
During the study period, the change in oxygen saturation over time was comparable in both groups. At the end of surgery, the patients of the high-flow group had significantly higher saturation levels in the intestinal mucosa (p<0.05). Over the course of intensive care, the groups did not differ in terms of fluid supply, administration of packed red blood cells, platelet concentrates or fresh frozen plasma. Analysis of urinary output revealed significant group differences. It was higher in the patients of the high-flow group than the normal-flow group (p<0.03), without differences in diuretic administration.
Laser Doppler spectroscopy is highly suited to the detection even of the slightest changes in flow characteristics and oxygenation of the skin, musculature and intestinal mucosa during surgery with extracorporeal circulation using CPB. At the same time, the technique of HFLR perfusion was found to have benefits over conventional bypass methods.
European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery 09/2009; 37(3):651-7. · 2.40 Impact Factor
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ABSTRACT: 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.
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.
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, Spearman's 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.
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.
European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery 08/2009; 37(1):210-7. · 2.40 Impact Factor
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ABSTRACT: 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.
Pediatric Cardiac Surgery Annual of the Seminars in Thoracic and Cardiovascular Surgery 02/2009;
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ABSTRACT: Integrated telemonitoring systems controlling circulatory and electrical parameters in adults with an implanted pacemaker have shown to be advantageous during follow-up of this patient group. In children and young adults with a congenital heart disease (CHD), these systems have to cope with a diversity of varying arrhythmias and a broad range of intrinsic cardiac parameters. Additional problems arise from the patients' growth and anatomic anomalies.
Since 2005, eight young patients (age 4.1- 37 years, mean 15.5 years) with a CHD received a pacemaker or implantable cardioverter defibrillator with an autonomous telemonitoring system at our clinic. The mean follow-up time was 395 days (range 106-834 days, 8.7 patient years).
In seven of eight patients the system transmitted information, which led to beneficial modifications of the current antiarrhythmic therapy. In three patients the reported events were of a critical nature. One patient remained event-free for 192 days after implantation. During follow-up, 96% of the days were covered. The system also transferred additional information on the effectiveness of antiarrhythmic medication and the impact of physical activity.
Young patients with an insufficient intrinsic heart rate or progressing arrhythmia, in addition to the conventional indications for pacemaker or defibrillator implantation, seem to profit to a high percentage from a telemetric surveillance system. The fully automated procedure of device interrogation and information transmission gives a daily overview on system function and specific arrhythmic events, especially in children who are unaware of any symptoms.
Pacing and Clinical Electrophysiology 11/2008; 31(10):1291-9. · 1.35 Impact Factor
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ABSTRACT: 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.
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.
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.
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.
European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery 09/2008; 34(4):732-7. · 2.40 Impact Factor
