Egil Seem

Impaired weight gain predicts risk of late death after surgery for congenital heart defects

Objectives: To describe long-term somatic growth in terms of weight for age in children operated on for congenital heart defects who die late (after the first 30 postoperative days) and to study the relationship between postoperative weight gain and survival after surgery for congenital heart defects. Methods: This was a nested case–control study of 80 children born in 1990–2002 who died late after surgery for congenital heart defects at Rikshospitalet, Norway. Weight data were obtained for 74 children, of whom 31 with no extra-cardiac anomalies were defined as cases and 31 surviving children with similar surgical complexity were defined as controls. Results: In the 74 children who died late, mean weight for age converted to z scores at birth, at last operation and at last recorded weight were 0.12, −1.31 and −2.09. In the 31 children defined as cases, the same weight z scores were 0.07, −1.21 and −2.01 compared with 0.05, −1.10 and −0.99 in the 31 matched controls. The odds ratio (OR) for death was 13.5 (95% CI 3.6 to 51.0) if there was a decrease in weight z score of >0.67 after the last operation. Median follow-up time after operation was 5.7 months. Conclusions: A decrease in weight for age during the first months after surgery for congenital heart defects of more than 0.67 z scores, corresponding to a downward percentile crossing through at least one of the displayed percentile lines on standard growth charts, is strongly related to late mortality in children operated on for congenital heart defects.

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.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. # CLINICAL PERSPECTIVE {#article-title-36}

Single-center 50 years’ experience with surgical management of tetralogy of Fallot

OBJECTIVE The aim of this study was to evaluate the long-term outcome of total repair for tetralogy of Fallot. We aimed to characterize late survival and the time-related risk of late reoperation. METHODS Operative protocols, patient records, and the database of the department were evaluated from 1951 until 2008. The official death registry of Norway was used for follow-up. Of the patients identified, the follow-up was 99.6% complete. RESULTS A total of 627 patients were studied. Of these, 570 could be identified for follow-up. There were a total of 41 early and 30 late deaths. The total early (including palliative procedures) mortality was 7.2% and total late mortality was 7.9%. However, during the last 10 years, no early mortality has been observed following repair. A total of 264 patients underwent some form of palliative procedure as their first treatment, and 541 patients had a reparative procedure performed, with an early mortality of 31 (5.7%). In patients subjected to a reparative procedure, there was no difference in freedom from death or reoperation following primary repair versus primary palliation. The use of transannular patch was associated with a highly significant risk of reoperation. CONCLUSIONS Surgical treatment of the tetralogy of Fallot and related congenital cardiac malformations has good long-term prognosis. In this cohort of patients, more than one-third required additional procedures later on, and, in some cases, as many as four additional surgeries. Palliative procedures followed by repair do not influence survival or reoperation-free survival. There are no differences between transatrial versus transventricular repair on survival or re-repair. Any transannular incision increases the risk of re-repair, but does not influence long-time survival. There is an almost linear decrease in reoperation-free survival following any type of repair of tetralogy of Fallot, even for as long as 50 years since the first procedure.

Ultrafiltration after cardiopulmonary bypass in children: effects on hemodynamics, cytokines and complement

OBJECTIVES The purpose of the study was to evaluate the clinical and hemodynamic effect of intraoperative extracorporeal ultrafiltration (UF) and its potential in reducing the plasma concentration of circulating cytokines and complement activation products following open heart surgery in children. METHODS Eighteen children with congenital heart disease were prospectively randomized into a control group (n = 9) and a group who underwent UF (n = 9). Serial plasma samples for measurements of circulating cytokines (interleukin 6 (IL-6), tumor necrosis factor alpha (TNF), and its soluble receptor (sTNF receptor)), and complement factors (C3 activation products (C3a and C3bc) and terminal complement complex (TCC)) were obtained before, during and up to 48 h after cardiopulmonary bypass (CPB). A pulmonary artery thermodilution catheter was introduced preoperatively for hemodynamic monitoring. RESULTS Postoperative hemodynamics were similar in both groups. Plasma levels of IL-6, sTNF receptors, C3a, C3bc and TCC increased significantly perioperatively (P < 0.01) in both groups. TNF was detected transiently in 16 patients perioperatively and in 4 of the 9 ultrafiltrate samples in concentrations similar to the plasma levels. Complement activating products were not detected in the ultrafiltration samples except for small amounts of C3a in two cases. Compared to the control group the plasma levels of C3a, C3bc and TCC were unaffected by the ultrafiltration procedure. The level of IL-6 and sTNF receptors increased significantly after 15 min of UF but there was no significant difference between the two groups postoperatively. CONCLUSIONS In this study no clinical or hemodynamic effect was registered after UF. TNF and C3a were occasionally detected in the ultrafiltrate but we were unable to demonstrate reduction of these or any of the other markers tested in the group subjected to ultrafiltration.

Homografts for Right Ventricular Outflow Tract Reconstruction in Congenital Heart Disease

In 49 patients aged 2.2-34.8 (mean 11) years, homografts (20 aortic, 29 pulmonary) were implanted in the right ventricular outflow tract as an isolated procedure or part of corrective surgery for congenital heart disease: tetralogy of Fallot with pulmonary stenosis (23 cases), pulmonary atresia with ventricular septal defect (10 cases) truncus arteriosus (8 cases) or transposition of the great arteries with pulmonary stenosis (8 cases). Previous palliative procedures had been performed on 34 patients, and 37 had undergone repair of right ventricular outflow tract, with one to four sternotomies prior to homograft implantation. Homograft valve sizes ranged from 14 to 25 mm internal diameter. Concomitant intra- or extracardiac procedures were performed in 29 cases. Follow-up was complete at a mean of 3 +/- 0.3 (0-8) years. Early and total mortality was 2.0% (1/49), due to sepsis and multi-organ failure unrelated to the homograft. At follow-up all but one of the patients had an improved New York Heart Association function class. Eight patients (16.3%) with a mean age of 9.2 +/- 1.8 (2.8-15.5) years at implantation had homograft malfunction (stenosis in three, regurgitation in two and combined in three) at follow-up, averaging 4.1 +/- 1.0 (0.4-6.9) years, with no significant difference between aorta and pulmonary homograft subsets. Freedom from structural valve deterioration was 46.6 +/- 22% for pulmonary and 32.3 +/- 21.3% for aortic homografts at the 7-year follow-up (difference not significant). In two patients an aortic homograft was uneventfully replaced. In conclusion, homograft implantation in patients with right ventricular outflow tract obstruction improves function class and can entail low mortality and morbidity, even after multiple previous median sternotomies.

A comparison of hospital costs with reimbursement received for patients undergoing the Norwood procedure for hypoplasia of the left heart

OBJECTIVES To determine whether the present system of reimbursement, based on diagnosis-related groups and regular financial budgeting, covers the costs incurred during hospitalisation of 7 children undergoing the three stages of the Norwood sequence for surgical treatment of hypoplastic left heart syndrome. METHODS Between January and September 2003, 7 patients underwent initial surgical palliation with the Norwood procedure at the Rikshospitalet University Hospital. A prospective methodology was developed by our group to measure the costs associated with each individual patient. The patients were closely observed, and the relevant data was collected during their stay in hospital. The stay was divided into four different periods of requirements of resources, defined as heavy intensive care, light intensive care, intermediate care, and ordinary care. At each stage, we recorded the number of staff involved and the duration of surgery and other major procedures, as well as the cost of pharmaceuticals and other consumables. Based on these data, we calculated the cost for each patient. These costs were compared with the corresponding revenue received by the hospital for each of the patients. RESULTS We found the total mean cost for the three stages of the Norwood sequence was 138,934 American dollars, while the corresponding revenue received by the hospital was 43,735 American dollars. During this period, one patient died during the first stage of the Norwood sequence. CONCLUSIONS Our study shows that steps involved in the Norwood sequence are low-volume but high-cost procedures. The reimbursement received by our hospital for the procedures was less than one-third of the recorded costs.

Carbomedics® Valve in Congenital Heart Disease: Midterm follow-up study of 14 patients

In 14 patients aged 5-329 (mean 131) months a CarboMedics valve was implanted because of congenital heart disease. The preoperative NYHA function class was III-IV in ten cases. Seven aortic and seven atrioventricular valves were replaced without early mortality. All patients were followed up, with mean observation time 27 months (total 384 months). One of the 14 patients died of heart failure 10 months postoperatively. Thrombosis occurred in four valves, three in tricuspid and one in mitral position. In all patients who received only warfarin, anticoagulation was demonstrably inadequate. Consequently we now recommend antiplatelet medication in addition to warfarin for children with atrioventricular mechanical valve replacement. In our experience the complication rate with CarboMedics prosthesis is acceptable, provided that anticoagulant therapy is adequate.

Eight years of pulmonary valve replacement with a suggestion of a promising alternative

Abstract In a retrospective study we assessed surgical results following right ventricular to pulmonary artery connection repair or replacement at a medium of 2.4 years (0–8) follow-up. Data were retrieved from hospital charts. Results. Three hundred and sixty five operations were performed in 286 patients in eight years starting in 2000 using different surgical methods. Homografts and Monocusps had a more than 50% significantly lower risk for reoperation than Contegra or bicuspid valves (p < 0.01). Data for infants and older children and grown ups were analysed separately. In the infant group no significant difference between the different methods (homograft, Contegra and Monocusp) was detected. In older patients, the Perimount valves performed extremely well with no need for reoperation after 2.5 years of follow-up. Perimount valves and homografts performed better than other solutions (p = 0.01). Conclusion. Although the follow-up for the Perimount valves was short, they are promising and need to be followed long-term. The homograft and the Monocusp remain valuable choices.

RELEASE OF INTERLEUKIN-8 AND CALPROTECTIN DURING AND AFTER PAEDIATRIC CARDIOPULMONARY BYPASS WITH AND WITHOUT ULTRAFILTRATION

Release of calprotectin and interleukin-8 (IL-8), changes in leukocyte counts and subsets and influence of extracorporeal ultrafiltration were evaluated during and after cardiopulmonary bypass (CPB) in 18 children undergoing open-heart surgery for congenital heart anomalies. Ultrafiltration was used in nine cases and nine were controls. Calprotectin concentration rose after start of CPB, peaking 48 hours postoperatively, with no significant intergroup difference. Positive correlation was found between duration of CPB and calprotectin (peak level and accumulated total). Circulating IL-8 was detected in all patients perioperatively, peaking at wound closure in the ultrafiltration group and at termination of bypass in the controls. CPB duration correlated significantly to peak level and accumulated total of IL-8. Seven of nine ultrafiltrate samples contained IL-8 at levels similar to the plasma concentration. Changes in white cell counts were mainly attributable to neutrophils. The two subgroups did not differ significantly in neutrophil counts. Neutropenia found after 10 minutes of CPB was replaced by neutrophilia, with maximal values postoperatively. Calprotectin and IL-8 thus were released into the circulation during CPB in children. Ultrafiltration did not affect the plasma concentrations of these substances, and only IL-8 was detected in the ultrafiltrate.

Fluid accumulation after closure of atrial septal defects: the role of colloid osmotic pressure

OBJECTIVES Following paediatric cardiac surgery with cardiopulmonary bypass (CPB), there is a tendency for fluid accumulation. The colloid osmotic pressure of plasma (COPp) and interstitial fluid (COPi) are determinants of transcapillary fluid exchange but only COPp has been measured in sick children. The aim of this study was to assess the net colloid osmotic pressure gradient in children undergoing atrial septal defect closure. METHODS Twenty-three patients had interventional and 18 had surgical atrial septal defect closures. Interstitial fluid was harvested using a wick method before and after surgery with CPB with concomitant blood samples. COP was measured using a colloid osmometer for small fluid samples. Baseline COP was compared with data from healthy children. RESULTS COPp at baseline was 21.9 ± 2.8 and 21.4 ± 2.2 mmHg in the interventional and surgical groups, respectively, and was significantly lower than in healthy children (25.5 ± 3.1 mmHg) (P < 0.001). In the surgical group, the use of CPB significantly reduced COPp to 16.9 ± 2.9 mmHg (P < 0.001) and the colloid osmotic gradient [ΔCOP (COPp - COPi)] to 2.9 ± 3.8 mmHg (P < 0.001) compared with interventional procedure. One hour after the procedure, COPi was 15.6 ± 3.8 mmHg and 9.9 ± 2.1 mmHg (P < 0.001) and the ΔCOP was 5.4 ± 3.0 mmHg and 9.1 ± 3.1 mmHg (P < 0.003) in the interventional and surgical groups, respectively. CONCLUSIONS Baseline COPp and COPi were lower in atrial septal defect patients compared with healthy children. The significantly lower COP gradient during CPB may explain the tendency for more fluid accumulation with pericardial effusion in the surgical group. The increased COP gradient after CPB may represent an oedema-preventive mechanism.