Preoperative versus postoperative survival in patients with univentricular heart: a nationwide, retrospective study of patients born in 1990-2015.

OBJECTIVES: Few data exist on mortality among patients with univentricular heart (UVH) before surgery. Our aim was to explore the results of intention to perform surgery by estimating preoperative vs postoperative survival in different UVH subgroups. DESIGN: Retrospective. SETTING: Tertiary centre for congenital cardiology and congenital heart surgery. PARTICIPANTS: All 595 Norwegian children with UVH born alive from 1990 to 2015, followed until 31 December 2020. RESULTS: One quarter (151/595; 25.4%) were not operated. Among these, only two survived, and 125/149 (83.9%) died within 1 month. Reasons for not operating were that surgery was not feasible in 31.1%, preoperative complications in 25.2%, general health issues in 23.2% and parental decision in 20.5%. In total, 327/595 (55.0%) died; 283/327 (86.5%) already died during the first 2 years of life. Preoperative survival varied widely among the UVH subgroups, ranging from 40/65 (61.5%) among patients with unbalanced atrioventricular septal defect to 39/42 (92.9%) among patients with double inlet left ventricle. Postoperative survival followed a similar pattern. Postoperative survival among patients with hypoplastic left heart syndrome (HLHS) improved significantly (5-year survival, 42.5% vs 75.3% among patients born in 1990-2002 vs 2003-2015; p<0.0001), but not among non-HLHS patients (65.7% vs 72.6%; p=0.22)-among whom several subgroups had a poor prognosis similar to HLHS. A total of 291/595 patients (48.9%) had Fontan surgery CONCLUSIONS: Surgery was refrained in one quarter of the patients, among whom almost all died shortly after birth. Long-term prognosis was largely determined during the first 2 years. There was a strong concordance between preoperative and postoperative survival. HLHS survival was improved, but non-HLHS survival did not change significantly. This study demonstrates the complications and outcomes encountering newborns with UVH at all major stages of preoperative and operative treatment.

Long-term follow-up and sex differences in adults operated for tetralogy of Fallot.

OBJECTIVE: Adults operated for tetralogy of Fallot (TOF) have high risk of ventricular arrhythmias (VA). QRS duration >180 ms is an established risk factor for VA. We aimed to investigate heart function, prevalence of arrhythmias and sex differences in patients with TOF at long-term follow-up. METHODS: We included TOF-operated patients≥18 years from our centre's registry. We reviewed medical records and the most recent echocardiographic exam. VA was recorded on ECGs, 24-hour Holter registrations and from implantable cardioverter defibrillator. RESULTS: We included 148 patients (age 37±10 years). Left ventricular global longitudinal strain (LV GLS, -15.8±3.1% vs -18.8±3.2%, p=0.001) and right ventricular (RV) GLS (-15.8±3.9% vs -19.1±4.1%, p=0.001) were lower in men at all ages compared with women. Higher RV D1 (4.3±0.5 cm vs 4.6±0.6 cm, p=0.01), lower ejection fraction (55%±8% vs 50%±9%, p=0.02), lower RV GLS (-18.1±4.0 ms vs -16.1±4.8 ms, p=0.04) and N-terminal pro-brain natriuretic peptide (NT-proBNP) over reference range (n=27 (23%) vs n=8 (77%), p<0.001) were associated with higher incidence of VA. QRS duration was longer in men (151±30 ms vs 128±25 ms, p<0.001). No patients had QRS duration >180 ms. QRS duration did not differ in those with and without VA (143±32 ms vs 137±28 ms, p=0.06). CONCLUSIONS: Our results confirmed reduced RV function in adults operated for TOF. Male patients had impaired LV and RV function expressed by lower LV and RV GLS values at all ages. Reduced cardiac function and elevated NT-proBNP were associated with higher incidence of VA and may be important in risk assessment.

Impact of pregnancy and risk factors for ventricular arrhythmias in women with tetralogy of Fallot.

OBJECTIVE: Patients with tetralogy of Fallot (TOF) have high survival rates 30 years after surgical repair. Many patients experience pregnancy; however, the effects of pregnancy on the long-term cardiovascular outcome are not well known. We investigated the association of pregnancy and cardiac function with occurrence of ventricular arrhythmia (VA) in women with TOF. METHODS: We recruited 80 women with repaired TOF from the national database. Holter monitoring or implanted devices detected VA, defined as non-sustained or sustained ventricular tachycardia or aborted cardiac arrest. All patients underwent echocardiography. Blood tests included NT-proBNP (N-terminal pro-brain natriuretic peptide). RESULTS: 55 (69%) women had experienced pregnancy. Mean age was lower in nulliparous compared with those with children (30±9 vs 40±9, p<0.01).VA had occurred in 17 (21%) women. Prevalence of VA was higher in women who had experienced pregnancy (n=16, 94%) compared with nulliparous (n=1, 6%) (p=0.02), also when adjusted for age (OR 12.9 (95% CI 1.5 to 113.2), p=0.02).Right ventricular mechanical dispersion was more pronounced in patients with VA (50±8 ms vs 39±14 ms, p=0.01, age-adjusted OR 2.1 (95% CI 1.3 to 7.5), p=0.01). NT-proBNP was also a marker of VA (211 ng/L (127 to 836) vs 139 ng/L (30 to 465), p=0.007). NT-proBNP >321 ng/L (normal values <170 ng/L) detected women with VA (p=0.019), also independent of age (OR 7.2 (95% CI 1.7 to 30.1), p=0.007). CONCLUSION: Pregnancy was associated with higher prevalence of VA among women with TOF. Right ventricular mechanical dispersion and NT-proBNP were age-independent markers of VA. These may have importance for pregnancy counselling and risk stratification.

Survival in patients with univentricular hearts: the impact of right versus left ventricular morphology.

Objective: Patients with univentricular hearts (UVH) have high mortality despite modern treatment, and better methods to identify patients at highest risk are needed. We wanted to improve risk stratification in patients with UVH by focusing on the prognostic significance of single right versus single left ventricular morphology (SRV vs SLV). Methods: All 395 patients with UVH operated at our centre were prospectively included from 1972 to 2016 (195 SRV, 166 SLV, 34 mixed or indeterminate ventricular morphology). Diagnoses, UVH morphology, types of all operations and time and causes of death or heart transplantation (HTX) were recorded. The primary endpoint was death or HTX. Results: Among the 111 non-Fontan patients, 88 died (SRV 62 vs SLV 20; p<0.0001), 32 due to heart failure (SRV 23 vs SLV 5; p=0.0012). Twenty-five years of cumulative SRV versus SLV survival among the 284 Fontan patients (41 deaths/HTX) was 66.9% vs 87.9% (p=0.0027), partly explained by more deaths/HTX due to heart failure among patients with SRV (p=0.0006). Survival in patients with SRV with and without hypoplastic left heart syndrome (HLHS) was similar. SRV versus SLV was a strong predictor of death/HTX in multivariable proportional hazards analyses (RR 3.3, 95% CI 1.6 to 6.6). Conclusion: SRV versus SLV is a strong short-term and long-term predictor of survival among patients with UVH, mainly explained by higher rates of death/HTX due to heart failure in the SRV group. Our findings apply to patients with SRV both with and without HLHS.

Can visual analogue scale be used in radiologic subjective image quality assessment?

BACKGROUND: Assessment of qualitative or subjective image quality in radiology is traditionally performed using a fixed-point scale even though reproducibility has proved challenging. OBJECTIVE: Image quality of 3-T coronary magnetic resonance (MR) angiography was evaluated using three scoring methods, hypothesizing that a continuous scoring scale like visual analogue scale would improve the assessment. MATERIALS AND METHODS: Adolescents corrected for transposition of the great arteries with arterial switch operation, ages 9-15 years (n=12), and healthy, age-matched controls (n=12), were examined with 3-D steady-state free precession magnetic resonance imaging. Image quality of the coronary artery origin was evaluated by using a fixed-point scale (1-4), visual analogue scale of 10 cm and a visual analogue scale with reference points (figurative visual analogue scale). Satisfactory image quality was set to a fixed-point scale 3=visual analogue scale/figurative visual analogue scale 6.6 cm. Statistical analysis was performed using Cohen kappa coefficient and agreement index. RESULTS: The mean interobserver scores for the fixed-point scale, visual analogue scale and figurative visual analogue scale were, respectively, in the left main stem 2.8, 5.7, 7.0; left anterior descending artery 2.8, 4.7, 6.6; circumflex artery 2.5, 4.5, 6.2, and right coronary artery 3.2, 6.3, 7.7. Scoring with a fixed-point scale gave an intraobserver κ of 0.52-0.77 while interobserver κ was lacking. For visual analogue scale and figurative visual analogue scale, intraobserver agreement indices were, respectively, 0.08-0.58 and 0.43-0.71 and interobserver agreement indices were up to 0.5 and 0.65, respectively. CONCLUSION: Qualitative image quality evaluation with coronary 3-D steady-state free precession MR angiography, using a visual analogue scale with reference points, had better reproducibility compared to a fixed-point scale and visual analogue scale. Image quality, being a continuum, may be better determined by this method.

Psychosocial and clinical outcomes of percutaneous versus surgical pulmonary valve implantation.

Objective: This prospective non-randomised study was performed to compare the psychosocial function and clinical outcomes following surgical and percutaneous implantation of a pulmonary valve at 3 months and 1 year after treatment. Methods: All patients were consecutively admitted for treatment by either method from June 2011 to October 2014. The data of 20 patients treated with the percutaneous technique and 14 patients treated with open heart surgery were compared. Psychosocial function was measured by the Achenbach System of Empirically Based Assessment (ASEBA). We used linear mixed-effect models to investigate group changes between the psychosocial function and clinical data of 34 patients with congenital pulmonary valve disease. Results: A significant difference in favour of the percutaneous pulmonary valve implantation group was observed regarding the ASEBA scores, specifically in the Thought problems subscale at 1 year (p=0.015), Attention problems subscale at 3 months (p=0.016) and 1 year (p=0.007) after treatment. After adjustment for the right ventricle to pulmonary artery pressure gradient at 3 months, a significant change in the Attention problems subscale (p=0.038) was noted in the percutaneous group. The New York Heart Association functional score significantly improved in both groups. The measured right ventricle to pulmonary artery pressure gradient was reduced significantly in both groups at 1 year. Conclusions: Both methods led to significant clinical improvement. Thought and attention problems such as intrusive behaviour significantly decreased only in patients who underwent the percutaneous procedure. Complications as reintervention, bleeding and arrhythmia were only observed in the open surgery group.

Right ventricular pressure response to exercise in adults with isolated ventricular septal defect closed in early childhood.

We previously demonstrated an abnormally high right ventricular systolic pressure response to exercise in 50% of adolescents operated on for isolated ventricular septal defect. The present study investigated the prevalence of abnormal right ventricular systolic pressure response in 20 adult (age 30-45 years) patients who underwent surgery for early ventricular septal defect closure and its association with impaired ventricular function, pulmonary function, or exercise capacity. The patients underwent cardiopulmonary tests, including exercise stress echocardiography. Five of 19 patients (26%) presented an abnormal right ventricular systolic pressure response to exercise ⩾ 52 mmHg. Right ventricular systolic function was mixed, with normal tricuspid annular plane systolic excursion and fractional area change, but abnormal tricuspid annular systolic motion velocity (median 6.7 cm/second) and isovolumetric acceleration (median 0.8 m/second2). Left ventricular systolic and diastolic function was normal at rest as measured by the peak systolic velocity of the lateral wall and isovolumic acceleration, early diastolic velocity, and ratio of early diastolic flow to tissue velocity, except for ejection fraction (median 53%). The myocardial performance index was abnormal for both the left and right ventricle. Peak oxygen uptake was normal (mean z score -0.4, 95% CI -2.8-0.3). There was no association between an abnormal right ventricular systolic pressure response during exercise and right or left ventricular function, pulmonary function, or exercise capacity. Abnormal right ventricular pressure response is not more frequent in adult patients compared with adolescents. This does not support the theory of progressive pulmonary vascular disease following closure of left-to-right shunts.

In-hospital cost comparison between percutaneous pulmonary valve implantation and surgery.

Objectives: Today, both surgical and percutaneous techniques are available for pulmonary valve implantation in patients with right ventricle outflow tract obstruction or insufficiency. In this controlled, non-randomized study the hospital costs per patient of the two treatment options were identified and compared. Methods: During the period of June 2011 until October 2014 cost data in 20 patients treated with the percutaneous technique and 14 patients treated with open surgery were consecutively included. Two methods for cost analysis were used, a retrospective average cost estimate (overhead costs) and a direct prospective detailed cost acquisition related to each individual patient (patient-specific costs). Results: The equipment cost, particularly the stents and valve itself was by far the main cost-driving factor in the percutaneous pulmonary valve group, representing 96% of the direct costs, whereas in the open surgery group the main costs derived from the postoperative care and particularly the stay in the intensive care department. The device-related cost in this group represented 13.5% of the direct costs. Length-of-stay-related costs in the percutaneous group were mean $3885 (1618) and mean $17 848 (5060) in the open surgery group. The difference in postoperative stay between the groups was statistically significant ( P ≤ 0.001). Conclusions: Given the high postoperative cost in open surgery, the percutaneous procedure could be cost saving even with a device cost of more than five times the cost of the surgical device.

Surgical options after Fontan failure.

OBJECTIVE: The objective of this European multicenter study was to report surgical outcomes of Fontan takedown, Fontan conversion and heart transplantation (HTX) for failing Fontan patients in terms of all-cause mortality and (re-)HTX. METHODS: A retrospective international study was conducted by the European Congenital Heart Surgeons Association among 22 member centres. Outcome of surgery to address failing Fontan was collected in 225 patients among which were patients with Fontan takedown (n=38; 17%), Fontan conversion (n=137; 61%) or HTX (n=50; 22%). RESULTS: The most prevalent indication for failing Fontan surgery was arrhythmia (43.6%), but indications differed across the surgical groups (p<0.001). Fontan takedown was mostly performed in the early postoperative phase after Fontan completion, while Fontan conversion and HTX were mainly treatment options for late failure. Early (30 days) mortality was high for Fontan takedown (ie, 26%). Median follow-up was 5.9 years (range 0-23.7 years). The combined end point mortality/HTX was reached in 44.7% of the Fontan takedown patients, in 26.3% of the Fontan conversion patients and in 34.0% of the HTX patients, respectively (log rank p=0.08). Survival analysis showed no difference between Fontan conversion and HTX (p=0.13), but their ventricular function differed significantly. In patients who underwent Fontan conversion or HTX ventricular systolic dysfunction appeared to be the strongest predictor of mortality or (re-)HTX. Patients with valveless atriopulmonary connection (APC) take more advantage of Fontan conversion than patients with a valve-containing APC (p=0.04). CONCLUSIONS: Takedown surgery for failing Fontan is mostly performed in the early postoperative phase, with a high risk of mortality. There is no difference in survival after Fontan conversion or HTX.

Off-pump hepatic to azygos connection via thoracotomy for relief of fistulas after a Kawashima procedure: Ten-year results.

OBJECTIVES: An almost universal incidence of developing pulmonary arteriovenous fistulas after the Kawashima operation has been reported. Exclusion of the hepatic venous flow from the pulmonary circulation causes the development of these malformations. Redirection of hepatic venous flow to the pulmonary circulation mostly leads to the regression of the arteriovenous fistulas. METHODS: We analyzed 11 patients with arteriovenous fistulas that developed after the Kawashima operation. The hepatic-to-azygos shunts were performed with an off-pump technique through a lateral thoracotomy in all but one. Operative and postoperative data were retrospectively collected. RESULTS: No intraoperative complications occurred, and no patient died in the hospital. Up to 10-year follow-up showed a significant postoperative improvement of patients' oxygen saturation and New York Heart Association class. Apart from 2 re-thoracotomies for bleeding in 1 patient, no complications occurred and no patient died during follow-up. Two other patients underwent reoperation for an undiagnosed additional hepatic vein. The improvement of patients' oxygen saturation and New York Heart Association class persisted during the follow-up period. CONCLUSIONS: The surgical connection can be performed safely with an off-pump technique that avoids the risks related to extracorporeal circulation and circulatory arrest. The results at 10 years follow-up confirmed the efficacy and safety of the surgical technique described.

Achievements in congenital heart defect surgery: a prospective, 40-year study of 7038 patients.

BACKGROUND: This article presents an update of the results achieved by modern surgery in congenital heart defects (CHDs) over the past 40 years regarding survival and the need for reoperations, especially focusing on the results from the past 2 decades. METHODS AND RESULTS: From 1971 to 2011, all 7038 patients <16 years of age undergoing surgical treatment for CHD at Rikshospitalet (Oslo, Norway) were enrolled prospectively. CHD diagnosis, date, and type of all operations were recorded, as was all-cause mortality until December 31, 2012. CHDs were classified as simple (3751/7038=53.2%), complex (2918/7038=41.5%), or miscellaneous (369/7037=5.2%). Parallel to a marked, sequential increase in operations for complex defects, median age at first operation decreased from 1.6 years in 1971 to 1979 to 0.19 years in 2000 to 2011. In total, 1033 died before January 1, 2013. Cumulative survival until 16 years of age in complex CHD operated on in 1971 to 1989 versus 1990 to 2011 was 62.4% versus 86.9% (P<0.0001). In the comparison of patients operated on in 2000 to 2004 versus 2005 to 2011, 1-year survival was 90.7% versus 96.5% (P=0.003), and 5-year cumulative survival was 88.8% versus 95.0% (P=0.0003). In simple versus complex defects, 434 (11.6%) versus 985 (33.8%) patients needed at least 1 reoperation before 16 years of age. In complex defects, 5-year cumulative freedom of reoperation among patients operated on in 1990 to 1999 versus 2000 to 2011 was 66% versus 73% (P=0.0001). CONCLUSIONS: Highly significant, sequential improvements in survival and reductions in reoperations after CHD surgery were seen. A future challenge is to find methods to reduce the need for reoperations and further reduce long-term mortality.

Favourable mid-term outcome after heart transplantation for late Fontan failure.

OBJECTIVES: Fontan failure (FF) represents a growing and challenging indication for paediatric orthotopic heart transplantation (OHT). The aim of this study was to identify predictors of the best mid-term outcome in OHT after FF. METHODS: Twenty-year multi-institutional retrospective analysis on OHT for FF. RESULTS: Between 1991 and 2011, 61 patients, mean age 15.0 ± 9.7 years, underwent OHT for failing atriopulmonary connection (17 patients = 27.8%) or total cavopulmonary connection (44 patients = 72.2%). Modality of FF included arrhythmia (14.8%), complex obstructions in the Fontan circuit (16.4%), protein-losing enteropathy (PLE) (22.9%), impaired ventricular function (31.1%) or a combination of the above (14.8%). The mean time interval between Fontan completion and OHT was 10.7 ± 6.6 years. Early FF occurred in 18%, requiring OHT 0.8 ± 0.5 years after Fontan. The hospital mortality rate was 18.3%, mainly secondary to infection (36.4%) and graft failure (27.3%). The mean follow-up was 66.8 ± 54.2 months. The overall Kaplan-Meier survival estimate was 81.9 ± 1.8% at 1 year, 73 ± 2.7% at 5 years and 56.8 ± 4.3% at 10 years. The Kaplan-Meier 5-year survival estimate was 82.3 ± 5.9% in late FF and 32.7 ± 15.0% in early FF (P = 0.0007). Late FF with poor ventricular function exhibited a 91.5 ± 5.8% 5-year OHT survival. PLE was cured in 77.7% of hospital survivors, but the 5-year Kaplan-Meier survival estimate in PLE was 46.3 ± 14.4 vs 84.3 ± 5.5% in non-PLE (P = 0.0147). Cox proportional hazards identified early FF (P = 0.0005), complex Fontan pathway obstruction (P = 0.0043) and PLE (P = 0.0033) as independent predictors of 5-year mortality. CONCLUSIONS: OHT is an excellent surgical option for late FF with impaired ventricular function. Protein dispersion improves with OHT, but PLE negatively affects the mid-term OHT outcome, mainly for early infective complications.

Pulmonary valve replacement with a bovine pericardial valve: a five year follow-up study.

OBJECTIVES: From a population of 90 patients after pulmonary valve replacement with a biological valve (Carpentier-Edwards Perimount valve), 56 of 80 available patients were examined five years after surgery. BACKGROUND: Pulmonary valve replacement is needed in many patients with congenital heart disease. Homografts have limited availability and predictable degeneration, and mechanical valves require anticoagulation. No superiority of one kind of pulmonary valve replacement has been shown. Biological valves that are readily available are being used and evaluated in increasing numbers. METHODS: In this cross-sectional study, five years following surgery, data were gathered from hospital charts, echocardiography, stress echocardiography, magnetic resonance imaging, and exercise testing. RESULTS: In 90 patients, there were three new valve replacements, one early cardiac death, and four late noncardiac deaths. Echocardiographic assessment of the study group showed pulmonary Doppler velocities (m/s) before, after operation, and at five-year follow-up of 2.8±1.1, 1.6±0.4, and 2.3±0.7, respectively. The assessed insufficiencies (0-3) at the same times were 2.3±1.0, 0.3±0.4, and 1.1±0.8. Maximal oxygen uptake increased from 65.6%±10.1% to 77.1%±18.2% of predicted and QRS width increased by 7±23 ms. Valve degeneration could be associated with young age but not with diagnosis or valve size. CONCLUSION: In our study, the biological valve in the pulmonary position showed excellent mid-term results with few reoperations, low gradients, and mild to moderate insufficiency. Oversizing, in contrast to young age, was not a risk factor for valve degeneration. In younger patients, this allows later percutaneous replacement, reducing the need for further surgery. However, longer follow-up is needed.

Perceived health after percutaneous pulmonary valve implantation: in-depth interviews of patients and next-of-kin.

OBJECTIVE: Percutaneous pulmonary valve implantation is an alternative to open heart surgery in selected patients with pulmonary outflow tract disorder. The technique may reduce the number of open-chest surgeries in these patients. This study was conducted to understand how the patients and their next-of-kin experienced this new treatment option. DESIGN: Qualitative explorative design with individual in-depth interviews. SETTING: Oslo University Hospital, the only cardiac centre in Norway offering advanced surgical and interventional treatment to patient with congenital heart defects, serving the whole Norwegian population. PARTICIPANTS: During a 2-year period a total of 10 patients, median age 17 (7-30) and 18 next-of-kin were consecutively selected for individual in-depth interviews 3-6 months after the pulmonary valve implantation. The verbatim transcripts were analysed using a phenomenological methodology. RESULTS: Patients emphasised the importance of regaining independence and taking control of daily life shortly after the new interventional treatment. Renewed hope towards treatment options was described as 'a light in the tunnel'. Next-of-kin emphasised the importance both for the patient and their family of resuming normal life quickly after the procedure. The physical burden was experienced as minor after the minimally invasive intervention, compared to their previous experience with surgical procedures. MAIN OUTCOME MEASURE: The importance of maintaining normality in everyday life for a good family function. CONCLUSIONS: The repeated surgeries during infancy and adolescence of patients with congenital heart disease represent a heavy burden both for the patient and their family. All families especially emphasised the importance of resuming normal life quickly after each procedure. The novel technique of pulmonary valve implantation is thus a favourable approach because of minor interference in daily life.

Pediatric coronary artery revascularization: a European multicenter study.

BACKGROUND: We sought to evaluate the hospital and midterm results of different surgical revascularization techniques in pediatric patients within the European Congenital Heart Surgeons Association. METHODS: From 1973 to 2011, 80 patients from 13 European Congenital Heart Surgeons Association centers underwent 65 pediatric coronary artery bypass grafting (PCABG) and 27 other coronary artery procedures (OCAP; 12 patients had combined PCABG and other coronary artery procedures). Excluded were patients with Kawasaki disease. Median age at the time of coronary procedure was 2.3 years (range, 2 days to 16.9 years); 33 patients (41.2%) were younger then 12 months. An emergency procedure was necessary in 34 patients (42.5%). RESULTS: Twelve patients (15%) died in the hospital; age at surgery (p=0.02) and the need for an emergent procedure (p=0.0004) were related to hospital mortality. Median follow-up time was 7.6 years (range, 0.9 to 23 years). There were 3 late cardiac deaths, all after a median time of 4 years (range, 9 months to 8.8 years) after PCABG. Fourteen patients (20.5%) presented with symptoms, including congestive heart failure (n=10) and angina (n=4), that were significantly associated with a low ejection fraction (p<0.001) and the presence of moderate or severe mitral valve regurgitation (p=0.0003). Six patients underwent a reintervention for impaired myocardial perfusion; all of them had a stenotic or atretic PCABG (p=0.001), and the majority were symptomatic (5 of 6 patients; 83.3%; p=0.001). CONCLUSIONS: Both PCABG and other coronary artery procedures are suitable surgical options in pediatric patients with impaired myocardial perfusion, which increases operative and midterm survival. Such population of patients needs to be followed for life to prevent and treat any possible cause of further myocardial ischemia.

Combined repair of upper sternal cleft and transposition of the great arteries in a newborn.

We report the case of a newborn with the unusual association of an upper sternal defect and transposition of the great arteries. Surgical correction of the cardiac disease consisted of the arterial switch procedure. The already less compliant bony thorax of the infant made direct approximation of the upper sternal defect only possible with adjuvant bilateral chondrotomy. Sternal cleft repair is advised during the very first weeks of life.

Balloon dilatation of pulmonary artery banding: Norwegian experience over more than 20 years.

AIMS: The purpose of this paper was to present the results of a simple modification of the suture technique for pulmonary artery banding (PAB), which allows for stepwise debanding by use of balloon catheter. METHODS AND RESULTS: During the period 1985-2007, PAB operations were performed in 227 children at Rikshospitalet. Of these children, 14.5% (n = 33) were treated by balloon dilatation of the PAB. Nine were treated twice. The intention of the procedure was total debanding in 17 and palliative treatment by stepwise dilatation of the PAB in 16 patients. Median follow up time was 59 months. The mean reduction of the gradient was more pronounced in the first group (37.0 ± 19.0 vs. 14.5 ± 10.3 mmHg, P < 0.001). The average mean oxygen saturation improved, however, significantly within the palliated group. The median time for reintervention after stepwise dilatation was 9 months. Serious, procedure-related complications occurred in 2 of 42 catheterizations (4.8%). Debanding by catheter replaced surgery in 8 of the 17 patients (47%). CONCLUSION: We consider catheter debanding a valuable alternative in selected cases. Combination with additional interventional techniques may extend the future indications.

Pediatric cardiac surgery in Northern Russia. Results from an international cooperative program.

OBJECTIVES: In order to evaluate a Norwegian-Russian clinical cooperation, this study sought to compare the incidence, early mortality and morbidity of surgically treated patients with atrial septum defect (ASD), ventricular septum defect (VSD) and Tetralogy of Fallot (TOF) in Norway and Archangels region in Russia. DESIGN: A retrospective analysis of patient records of all those surgically treated for ASD, VSD and TOF in Norway and from Archangels region from 1 January 2000 to 31 December 2005. RESULTS: The Norwegian cohort consisted of 191 ASDs, 227 VSDs and 126 TOFs. The Russian cohort counted 128 ASDs, 77 VSDs and nine TOFs. Thirty-days mortality was 0.4% in the Norwegian VSD, 1.6% in the Norwegian TOF and 1.3% in the Russian VSD cohort. Postoperative complications in the Norwegian cohorts were 18.3% (ASDs), 15.9% (VSDs) and 34.1% (TOFs). The corresponding findings in Archangels were 4.7%, 11.7% and 0%. CONCLUSION: Surgery for ASDs, VSDs and TOFs can be carried out with minimal early mortality in both countries. The discrepancy in early postoperative morbidity illustrates the challenges in comparative studies between different countries, cultures and health care systems. The establishment of a decentralized surgical facility has increased the access to cardiac surgery to the population in Archangels region.