Claudia Arenz

Early and long.term results for correction of total anomalous pulmonary venous drainage (TAPVD) in neonates and infants

OBJECTIVE To present our 17-year experience of surgical repair of total anomalous pulmonary venous drainage (TAPVD) in 71 consecutive neonates and infants, with particular emphasis on the role of preoperative pulmonary venous obstruction (PVO), the management of postoperative pulmonary hypertensive crises and the long-term results. PATIENTS AND METHODS From July 1977 to May 1994, 36 newborns and 35 infants, aged between 1 and 300 days, had repair TAPVD in our Department. Fifty-four patients had decompensated heart failure, whereas three were moribund. The anomalous drainage was supracardiac in 32 (45%), cardiac in 17 (24%), infracardiac in 17 (24%) and mixed in 5 infants (7%). Major associated cardiac anomalies coexisted in nine cases. Forty-five (63%) exhibited signs of PVO. The repair was performed under moderate hypothermia in 15 and deep hypothermic arrest in 56 patients. In case of postoperative pulmonary hypertensive crises, artificial hyperventilation with high oxygen concentration, sedation and relaxation, inotropic support and afterload reduction with phentolamine were applied selectively. The actual follow-up is based on functional symptomatology and cardiac echo-doppler studies. RESULTS Six children (8.5%) died early: four perioperatively, two on the 2nd and 10th postoperative day, respectively. Poor preoperative condition, operation before 1987 and preoperative PVO were the main incremental risk factors for death. No death occurred among the last 38 cases. There were complications in 45 patients, in particular pulmonary hypertensive crises in 27. Two patients died late, after 80 and 118 days, respectively, of progressive intrapulmonary vein fibrosis: the second despite reoperation. The risk of late death approaches zero after 6 months. Among the 63 survivors, 56 (86%) are asymptomatic and without medication, 3 months-17 years after operation. Five have mild symptoms with ordinary physical activity. Two are lost to follow-up. CONCLUSIONS Early repair of TAPVD with aggressive management of pulmonary hypertensive crises carries low operative mortality nowadays. Preoperative PVO as a risk factor has been neutralized since 1987. Long-term results are gratifying: no late death after 6 months, no reoperation and functional good results. Progressive pulmonary vein fibrosis remains an unpredictable rare cause of death within the 1st year after surgery.

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.

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.

Is there any need for a shunt in the treatment of tetralogy of Fallot with one source of pulmonary blood flow

OBJECTIVES In symptomatic patients, performing a primary repair of tetralogy of Fallot (TOF), irrespective of age or placing a shunt, remains controversial. The aim of the study was to analyse the policy of primary correction. METHODS Between May 2005 and May 2012, a total of 87 consecutive patients with TOF, younger than 6 months of age, underwent primary correction. All patients had one source of pulmonary blood flow, with or without a patent ductus arteriosus. The median age at surgery was 106 ± 52.3 days (8-180 days). Twelve patients (13.8%) were newborns. Two groups were analysed: group I, patients <1 month of age; group II, patients between 2-6 months of age. RESULTS There was no early or late death at 7 years of follow-up. There was no difference in bypass time or hospital stay between the two groups, but the Aristotle comprehensive score (P < 0.0001), ICU stay (P = 0.030) and the length of ventilation (P = 0.014) were significantly different. Freedom from reoperation was 87.3 ± 4.3% and freedom from reintervention was 85.9 ± 4.2% at 7 years, with no difference between the two groups. Neurological development was normal in all patients, but 1 patient in Group II had cerebral seizures and showed developmental delay. Growth was adequate in all patients, except those with additional severe non-cardiac malformations that caused developmental delay. Eighty-five per cent of the patients were without cardiac medication. CONCLUSIONS Even in symptomatic neonates and infants <6 months of age, primary repair of TOF can be performed safely and effectively. One hundred per cent survival at 7 years suggests that early primary repair causes no increase in mortality in the modern era. Shunting is not necessary, even in symptomatic newborns, thus avoiding the risk of shunt-related complications and repeated hospital stays associated with a staged approach.

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.

Open valvotomy for aortic valve stenosis in newborns and infants

The most appropriate management of aortic stenosis in children remains controversial. Both balloon and surgical valvotomy are firmly established as effective initial treatments with encouraging survival rates even in the troublesome neonatal group. Improved early results are based rather on the better understanding of the limits of a biventricular repair than on the method of treatment. Valvotomy of any kind is a palliative procedure and reintervention remains frequent. Direct surgical intervention, where exact splitting of fused commissures and shaving off of obstructing nodules can produce a better valve with maximum valve orifice without causing regurgitation, might offer superior longer-lasting results in comparison with blind ballooning.

Restrictive Atrial Septum Defect Becomes a Risk Factor for Norwood Palliation of Hypoplastic Left Heart Syndrome Only When It Is Combined with Mitral or Aortic Atresia.

BACKGROUND Restrictive atrial septal defect (ASD) is described as risk factor for Norwood procedure because of elevated pulmonary resistance. We hypothesized that it invariably could not cause pulmonary hypertension, unless it was combined with mitral valve or aortic valve atresia. We investigated how restrictive ASD influenced survival of patients with hypoplastic left heart syndrome (HLHS) who underwent Norwood operation. PATIENTS AND METHODS A total of 118 HLHS patients who underwent surgery between January 2005 and December 2012 were grouped into three groups. Group 1 included 31 patients with restrictive ASD combined with mitral or aortic atresia; Group 2 composed of 12 patients with restrictive ASD and mitral and aortic stenosis; Group 3 (n = 75) had no ASD restriction. Survival was determined for each group. Multivariate analysis was conducted to test risk factors for mortality. RESULTS Mean follow-up was 26.3 ± 24.1 months. Survival was 78.7% ± 4.2% at 30-month interval and onward after Norwood procedure for the whole cohort; it was 43.8% ± 10.0%, 91.7% ± 8.0%, and 77.3% ± 5.0% for Group 1, 2, and 3, respectively. The difference was significant between Group 1 and Group 2 and 3: p < 0.001. Survival was similar for Group 2 and Group 3: p = 0.45. Combination of restrictive ASD and mitral or aortic atresia was found to be the sole risk factor for early and late mortality (odds ratio: 3.5, 95% confidence interval: 1.8-7.1, p < 0.001). CONCLUSION Restrictive ASD only affects survival of HLHS patients following Norwood procedure if it is associated with mitral or aortic atresia.

The modified Senning procedure as an integral part of an anatomical correction of congenitally corrected transposition of the great arteries.

In the current era of anatomical correction of complete transposition of the great arteries, the Senning operation is reserved only for the atrial switch part of anatomical corrections of congenitally corrected transposition of the great arteries. These rare operations are performed in only a few centers all over the world; the majority of practicing cardiac surgeons therefore have limited experience with the Senning operation. The proposed modified Senning procedure might simplify the original concept. Once the technical aspect of the procedure is accomplished, the risk of systemic and pulmonary baffle obstructions is minimal, even in situs solitus with dextrocardia or situs inversus with levocardia. Furthermore, this technique has the potential to provide adequate capacity of the pulmonary venous atrium, to preserve optimal geometry of the mitral valve, to minimize damage of sinus node and to make the coronary sinus accessible for electrophysiological studies or intervention by keeping the coronary sinus in the systemic venous baffle. The modified technique is simple, highly reproducible and applicable, regardless of the situs and position of the apex of the heart.

Contemporary results of aortic valve repair for congenital disease: lessons for management and staged strategy†

OBJECTIVES Any aortic valve (AoV) operation in children (repair, Ross or mechanical replacement) is a palliation and reinterventions are frequent. AoV repair is a temporary solution primarily aimed at allowing the patient to grow to an age when more definitive solutions are available. We retrospectively analysed AoV repair effectiveness across the whole age spectrum of children, excluding neonates and AoV disease secondary to congenital heart disease. METHODS From 2003 to 2015, 193 consecutive patients were included. The mean age was 9.2 ± 6.9 years (22% <1 year); 86 (45%) had a preceding balloon valvuloplasty. The indications for the procedure were stenotic (n = 123; 64%), regurgitant (n = 63; 33%) or combined (n = 7; 4%) disease. The procedures performed were commissurotomy shaving (n = 74; 38%), leaflet replacement (n = 78; 40%), leaflet extension (n = 21; 11%) and neocommissure creation (n = 21; 11%). Post‐repair geometry was tricuspid in 137 (71%) patients. RESULTS The 10‐year survival rate was 97.1%. Freedom from reoperation and replacement at 7 years was, respectively, 57% (95% confidence interval, 47‐66) and 68% (95% confidence interval, 59‐76). In multivariate analysis, balloon dilatation before 6 months, the absence of a developed commissure, a non‐tricuspid post‐repair geometry and cross‐clamp duration were predictors for reoperation and replacement. After a mean follow‐up period of 5.1 ± 3.0 years, 145 (75%) patients had a preserved native valve, with undisturbed valve function (peak gradient <40 mmHg, regurgitation ≤mild) in 113 (58%). CONCLUSIONS Aortic valve repair in children is safe and effective in delaying the timing for more definitive solution. Surgical strategy should be individualized according to the age of the patient. Avoidance of early balloon dilatation and aiming for a tricuspid post‐repair arrangement may improve outcomes.