Harald Lindberg

Outcome of congenital heart defects--a population-based study.

In a population‐based study including 35 218 infants born alive during the 15‐y period 1982–96, 360 (1%) were diagnosed as having a congenital heart defect (CHD). At a follow‐up 3–18 y after birth (median 9.5 y) 154 patients (42.8%) were spontaneously cured; of these, 142 (92.2%) had ventricular septal defects (VSDs). Forty‐two patients (11.7%) died, 22 of these (52.4%) during the neonatal period (0–28 d after birth). A total of 119 patients (33.1%) underwent therapeutic procedures (surgery, catheter interventions), 24 (20.2%) of whom died. Of the 95 children surviving therapeutic procedures, 54 (56.8%) had their defects completely repaired, while 41 (43.2%) had residual defects or cardiac sequelae, often of minor importance. In 69 children (19.2%) with persistent non‐operated defects, 43 (62.3%) had VSDs. A chromosomal disorder, syndrome or associated extracardiac malformation occurred in 72 children (20%).

Mental health and psychosocial functioning in adolescents with congenital heart disease. A comparison between adolescents born with severe heart defect and atrial septal defect

Twenty‐six adolescents, aged 13–18 years, with severe congenital heart disease were matched for sex, age and living area with 26 adolescents with repaired atrial septal defect and regarded as physically fit. These two groups were compared according to somatic condition, psychopathology, psychosocial functioning and chronic family difficulties. A higher rate of psychiatric problems in the complex group, an association between psychosocial functioning and physical capacity, as well as an association between psychosocial functioning and chronic family difficulties were observed. These findings suggest that physical capacity is of crucial importance for mental health and functioning of adolescents with congenital heart disease. The association with chronic family difficulties also suggests that a comprehensive biopsychosocial approach is necessary in the treatment and rehabilitation of these patients.

Optimal Structure of a Congenital Heart Surgery Department in Europe: by EACTS Congenital Heart Disease Committee1

2003;24:343-351 Eur J Cardiothorac Surg Hamilton, H. Lindberg, B. Maruszewski and J. Monro W. Daenen, F. Lacour-Gayet, T. Aberg, J.V. Comas, S.H. Daebritz, R. Di Donato, J.R.L. 1 Congenital Heart Disease Committee Optimal Structure of a Congenital Heart Surgery Department in Europe: by EACTS This information is current as of April 16, 2008 http://ejcts.ctsnetjournals.org/cgi/content/full/24/3/343 the World Wide Web at: The online version of this article, along with updated information and services, is located on

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.

Some Observations on Cerebral Perfusion during Cardiopulmonary Bypass

Blood flow was recorded with an electromagnetic flow probe on one internal carotid artery (ICA) during cardiopulmonary bypass (CPB) in 5 patients. The ICA flow was monitored continuously along with arterial blood pressure, epidural intracranial pressure, and cerebral electrical activity using a cerebral function monitor (3 patients). The ICA flow increased by 50 to 100% at the inception of extracorporeal circulation. This rapid enhancement of flow occurred within a thirty-second period and was due to rapid arterial hemodilution caused by introduction of the priming solution. A transitory fall in ICA flow was observed during subsequent minutes when the well-recognized drop in blood pressure took place and the cerebral perfusion pressure (CPP = blood pressure - epidural intracranial pressure) was reduced to less than 30 mm Hg. In only one instance, however, when CPP fell to 15 mm Hg, was the fall in flow lower than the prebypass level. Throughout the rest of CPB, with steady-state hemodilution and CPP levels in the range of 30 to 50 mm Hg, ICA flow was markedly enhanced (50 to 100% above the prebypass level). The flow pattern, however, disclosed a pressure-passive system, indicating that cerebral autoregulation was impaired or that the CPP levels were lower than the individual lower limit of cerebral autoregulation during the period of steady-state hemodilution on CPB. A transient depression of cerebral electrical activity was seen in 2 patients shortly after the introduction of CPB. This phenomenon is suggestive of qualitatively insufficient perfusion and was observed even when ICA bulk flow was increased (hematocrit values, 13 to 17%).

Effect of Perfusion Temperature on the Inflammatory Response During Pediatric Cardiac Surgery

BACKGROUND Cardiopulmonary bypass (CPB) triggers the whole body inflammatory response, and it has been suggested that the degree of hypothermia may influence these responses. The aim of this prospective study was to compare the inflammatory response in children undergoing CPB for repair of congenital heart defects, randomized to mild or moderate hypothermia. METHODS We measured inflammatory markers in blood samples of thirty children with body weight less than 10 kg undergoing open heart surgery randomized to surgery at either mild (32 degrees C) or moderate (25 degrees C) hypothermia. Blood was sampled after induction of anesthesia, at skin closure, 2 hours, 24 hours, and 48 hours postoperatively. RESULTS Except for an enhanced interleukin-8 response in the moderate hypothermia group, there were no differences in levels of inflammatory mediators between those with mild and those with moderate hypothermia. In contrast to the modest influence of the degree of hypothermia, long CPB time and long aortic cross-clamp time were accompanied by enhanced inflammation involving raised levels of interleukin-8 and myeloperoxidase, as well as increased leukocyte counts. CONCLUSIONS Only minor differences in cytokine levels were detected between those with moderate and those with mild hypothermia during CPB. Ischemic aortic cross-clamp time and time on CBP should be as short as possible to avoid an excessive inflammatory response and possibly adverse clinical effects.

Rescue cardiac transplantation for failing staged palliation in patients with hypoplastic left heart syndrome

OBJECTIVE Orthotopic heart transplantation is considered a rescue option for children with failing staged palliation or repair of hypoplastic left heart syndrome. We present our strategy for management, and outcomes, for these complex patients. METHODS We transplanted 68 consecutive children, with diagnoses of hypoplastic left heart syndrome in 31, cardiomyopathy in 20, and post-operative complex congenital heart disease in 17. Of these patients, 9 (13.2%) were neonates, and 46 (67.6%) were infants. Median age was 118.5 days. Operative technique involves bicaval cannulation and anastamoses with continuous low flow bypass, and either short periods of circulatory arrest or continuous low flow antegrade cerebral perfusion for reconstruction of the aortic arch. Initial reperfusion of the donor heart utilizes glutamate and aspartate substrate enriched white blood cell filtered cardioplegia. Immunosuppressive therapy includes induction (pulse steroids, gamma globulin, and polyclonal rabbit antithymocyte globulin) and initial maintenance (calcineurin inhibitor, an anti-proliferative agent, and a weaning steroid protocol). Of the 31 patients with hypoplastic left heart syndrome, 23 underwent primary transplantation, and 8 underwent rescue transplantation from failing staged palliation in seven, or attempted biventricular repair in one. Of the seven patients who had failing staged palliation, three had undergone only the Norwood Stage 1 operation, 2 had undergone a Norwood Stage 1 operation and a Glenn superior cavopulmonary anastomosis and two had undergone a Norwood Stage 1 operation, a Glenn superior cavopulmonary anastomosis, and a completion Fontan operation. RESULTS The group undergoing primary transplantation was younger (p equals 0.007), weighed less (p equals 0.003), and waited longer for an appropriate donor heart (p equals 0.021) compared to those requiring rescue transplantation. No significant difference exists between the groups with regards to donor heart ischaemic time or post-transplant length of hospital stay. Thirty day survival (p equals 0.156) and overall survival (p equals 0.053) was better in those having primary transplantation, although these differences were not statistically significant when a p value of less than 0.05 is considered to be significant. In those having primary transplantation, no patients had elevated panel reactive antibody greater than 10%. Half of the 8 requiring rescue transplantation had panel reactive antibody greater than 10%, and this subgroup did especially poorly. CONCLUSION Cardiac transplantation can offer children with failing staged palliation their only chance of survival. Transplantation, however, carries a high risk in this subgroup, especially in the setting of elevated panel reactive antibody.

Congenital heart defects: the patients who die.

AIMS To register mortality and causes of death in patients with congenital heart defects (CHDs). METHODS Prospective population-based observational study. RESULTS 553 infants with CHD (1.1% of live born) were observed for 1-22 y (median 10 7/12 y). Sixty-four died (11.6%), of whom 32 (50%) died during the first 4 wk, and 51 (79.7%) during the first year of life. Of the total neonatal deaths in the population (3 per 1000), CHDs occurred in 21.5%. Mortality for children with CHDs was not significantly different between the cohorts born in 1982-1991 and 1992-2002, for either neonatal deaths or deaths later on (p>0.05). Out of 170 patients in whom therapeutic procedures (surgery, catheter interventions) were undertaken, 34 (20%) died. Nine cases (1.6%) died with unrecognized CHDs; seven of these on the first day of life with severe extracardiac malformations. In 50 (78.1%) cases, death was judged to be caused directly or indirectly from the CHD, and in 14 (21.9%) from extracardiac malformations or other conditions. CONCLUSION CHDs occur in a substantial number of neonatal deaths. Most deaths are caused by cardiac insufficiency. The mortality rate remained unchanged.

Polytetrafluoroethylene Bicuspid Pulmonary Valve Implantation: Experience With 126 Patients

This article reports our initial experience in 126 consecutive patients treated with placement of a surgically created polytetrafluoroethylene (PTFE) bicuspid pulmonary valve at The Congenital Heart Institute of Florida (CHIF). A bicuspid pulmonary valve is created with PTFE and sutured into the right ventricular outflow tract. PTFE bicuspid pulmonary valves were placed in 126 patients (age: range, 3.1-64.7 years, mean, 17.9 years; weight: range, 14.2-113.6 kg, mean, 55.4 kg). All patients had pulmonary insufficiency, pulmonary stenosis, or both, most commonly after previous repair of tetralogy of Fallot (71 patients). Follow-up was up to 8.3 years (range, 0-8.3 years, mean, 3.34 years). Operative mortality was 1 patient (0.8%). Late mortality was non–valve-related in 3 patients (2.4%). The initial 84 patents in this series received valves constructed from PTFE with 0.6-mm thickness. The next 42 patients received valves constructed from PTFE with 0.1-mm thickness. Six patients of 126 (4.8%) required replacement of the PTFE bicuspid pulmonary valve because of immobile and calcified leaflets. All 6 who required replacement of the PTFE bicuspid pulmonary valve initially received a valve constructed from porous 0.6-mm PTFE material. We currently use nonporous 0.1-mm PTFE, which does not allow cellular in-growth and thickening. Early echocardiographic follow-up of these valve leaflets made with 0.1-mm PTFE has demonstrated improved leaflet mobility and pliability and lower transvalvar gradients. PTFE bicuspid pulmonary valve implantation is safe and effective and demonstrates acceptable performance for the intermediate term. It is anticipated that using thinner 0.1-mm PTFE will improve valve function and durability. Long-term follow-up is necessary to determine the true value of this technique.