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.

[Heart transplantation in Norway]. / Hjertetransplantasjon i Norge.

The first heart transplantation in the Nordic countries was performed at Rikshospitalet, Oslo in 1983. In this paper, we present our experience with this treatment up to 1999. 317 heart transplantations have been performed, an average of 23 transplantations per year. 82% of the recipients were males; 50% had heart failure due to coronary heart disease. Mean age of the recipients was 47 years (range 1-64). Our indications and contraindications are similar to most other transplantation centres. Triple immunosuppression with ciclosporin, prednisolone and azathioprine have been used as standard treatment. The survival rate after one and ten years are 85% and 53% respectively, with a significantly higher survival rate among recipients younger than 50 at transplantation, especially if the graft was from a donor younger than 35 years. The most common early postoperative complications were acute cellular rejections and infections. Transplant accelerated coronary heart disease and cancer were the main causes of late death. We believe that close co-operation between Riskshospitalet and local centres will provide the best treatment for patients needing a heart transplant.

Thyroid function during and after cardiopulmonary bypass in children.

BACKGROUND: The aim of the study was to elucidate the changes in thyroid function during and after cardiopulmonary bypass (CPB) in children. METHODS: Triiodothronine (T3), thyroxine (T4), free thyroxine (FT4) and thyroid-stimulating hormone (TSH) were determined preoperatively, at specific times throughout CPB, and serially up to 48 h postoperatively, in 10 children (median age 35, range 23-68 months) undergoing elective surgery for congenital heart disease. RESULTS: T3 decreased from 2.01 +/- 0.08 preoperatively to 0.94 +/- 0.10 nmol/l 24 h postoperatively (P < 0.05). T4 levels followed a pattern similar to changes in T3. FT4 increased from 17.4 +/- 0.7 preoperatively to 30.0 +/- 0.4 pmol/l after 30 min of CPB (P < 0.05). TSH decreased from 2.44 +/- 0.43 preoperatively to 0.93 +/- 0.21 24 h postoperatively (P < 0.05). CONCLUSION: T3, T4 and TSH are significantly depressed after open heart surgery in children.

Erythromelalgia: a clinical study of 87 cases.

We report on aetiological factors, clinical findings and prognosis of 87 patients with erythromelalgia (EM). This is the largest material reported in the western literature. There is a 100% follow up of patients with observation period up to 11 years. There were 61 females and 26 males. About two-thirds of the patients were primary cases and around three-quarters had a chronic condition. The condition was more common in lower than in upper extremities. Over time patients with erythromelalgic syndrome gradually get worse, those with primary and secondary acute EM get better, whilst primary and secondary chronic EM remain stable.

[Extracorporeal heart pump--a bridge to heart transplantation. First Norwegian experiences]. / Parakorporal hjertepumpe--bro til hjertetransplantasjon. Første norske erfaringer.

By February 1997, the number of heart transplantations at the National Hospital had reached 265. In our department, the first bridge to transplantation in using Thoratec artificial heart was performed in 1995. A 44 year-old patient with life-threatening heart failure was placed for one week on cardiopulmonary assist with an intra-aortic balloon pump and a veno-arterial cardiopulmonary bypass. Due to unceasing heart failure a Thoratec left ventricular assist device was implanted as a bridge to transplant. The patient was successfully transplanted two days after implantation of Thoratec. After eight months follow-up he is in New York Heart Association (NYHA) functional class I. Another patient, 17 years of age, and with dilated cardiomyopathy, went into cardiogenic shock while awaiting heart transplantation. For three days he had cardiopulmonary assist with intra-aortic balloon pump and venoarterial bypass with an oxygenator. After seven weeks on the left ventricular assist device he was transplanted. Although one episode of severe rejection occurred, be is in NYHA functional class I after four months follow-up. Our early experience is encouraging. Ventricular assist devices should be considered as a life support for patients scheduled for heart transplantation and expected to die within a very short time.

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.

Outcome of bypass surgery in patients with chronic ischemic left ventricular dysfunction. Predictive value of MR imaging.

PURPOSE: To determine the preoperative findings of MR imaging of the left ventricle (LV) that could best predict the functional outcome of the LV after surgical revascularization. MATERIAL AND METHODS: Patients with angina pectoris, previous myocardial infarction, and dysfunction of the LV, and who had a preoperative cine MR, were re-evaluated after bypass surgery with MR in a study on the effects of revascularization after mean 22 months. RESULTS: Angina pectoris was relieved in all patients except one, but the maximum workload during the exercise test was increased in only 3 patients. Coronary angiography showed that 37 of 45 (82%) of the distal anastomoses were open. The LV ejection fraction was the same before and after operation both at angiography and MR imaging. MR showed LV end-diastolic volume to be increased from 190 +/- 50 ml to 250 +/- 70 ml. Compared to angiography, MR provided additional information regarding myocardial wall thickness and function, and the size of myocardial infarction. Improvement in systolic wall thickening was seen in 65% of the segments that had had an end-diastolic wall thickness (EDWT) greater than 15 mm before operation, while only 4% of the segments with EDWT < 6 mm improved. In the wall thickness range of 6-15 mm, MR was unable to predict the functional outcome of the LV. CONCLUSION: Preoperative MR findings of thick myocardial walls with poor function seem predictive of improved function after revascularization. When the LV wall thickness is less than 6 mm, no improvement should be expected.

Release of soluble tumour necrosis factor alpha receptors during and after paediatric cardiopulmonary bypass. Correlation with haemodynamic and clinical variables.

The release of cytokines during cardiopulmonary bypass (CPB) may contribute to haemodynamic alternations encountered after open heart surgery. Regulatory mechanisms exist and include soluble cytokine receptors. We have measured blood levels of tumour necrosis factor (TNF) and its soluble receptor (TNFsr) during and after open heart surgery in children. Correlation analysis to haemodynamic and clinical variables was performed. Using immunoassays the authors registered a significant increase in plasma levels of TNFsr with peak levels 2 h post-operatively at a level of 1702 +/- 170 pg/ml. The concentration of TNFsr remained significantly elevated until 48 h postoperatively but TNF was not significantly elevated. An inverse correlation existed between peak TNFsr and mean arterial pressure (rho = -0.827, P < 0.05), between TNFsr and cardiac index (rho = -0.8, P < 0.05), between TNFsr and left ventricular stroke work index (rho = -0.983, P < 0.01), between TNFsr and weight (rho = -0.85, P < 0.05) and between TNFsr and body surface area (rho = -0.867, P < 0.05). The authors demonstrate that the smallest children experienced the highest TNFsr concentration post-operatively. Furthermore cardiac performance, expressed as cardiac index and left ventricular stroke work index, correlated inversely to peak TNFsr level post-operatively.

Use of rocuronium in a pregnant patient with an open eye injury, receiving magnesium medication, for preterm labour.

We present a case where rocuronium 80 mg (3 x ED95) was used in a rapid sequence induction in a 80-kg pregnant patient with an open eye injury. The patient was also receiving magnesium 2 g h-1 i.v. for preterm labour. The expected duration for neuromuscular block of rocuronium in the absence of magnesium would be approximately 53 min; with infusion of magnesium, the duration of neuromuscular block was prolonged four-fold (215 min). It is important to remember that magnesium potentiates the effects of all non-depolarizing neuromuscular blocking agents, including rocuronium.

[Aortic coarctation and complex heart disease. Surgical treatment during the first year of life]. / Koarktasjon av aorta og kompleks hjertelidelse. Kirurgisk behandling i første leveår.

During the five years 1990 through 1994, 70 neonates and infants had surgery for coarctation of the aorta. 30 patients with complex coarctation of the aorta underwent a first stage correction with supplementary ductus ligation and pulmonary artery banding as needed. The mode of surgical repair of coarctation was end-to-end anastomosis in ten patients and patch graft in 20 patient. Mean follow-up was 766 (range 3-1812) days. Mortality after primary procedure was 16.6% (5/30) and after secondary procedure 11.1% (2/18). Three surviving patient developed recoarctation, but only one patients needed re-operation. In spite of improvement in neonatal cardiac surgery, we conclude that a staged approach is still the current treatment in most cases of complex coarctation of the aorta.

[Heart transplantation as a life saving procedure after conventional heart surgery]. / Hjertetransplantasjon som livreddende prosedyre etter konvensjonell hjertekirurgi.

The first cardiac transplantation in Norway was performed in November 1983. At the end of 1995 the number of heart transplantations at our institution had reached 238. Five of these patients (2.1%) were transplanted in the early postoperative period of failed conventional cardiac surgery (respectively 20, 26, 54, 84, and 234 hours post surgery). The primary procedure consisted of mitral valve replacement plus left ventricular aneurysm resection, coronary artery bypass grafting, Konz-Konno procedure, aortic valve replacement, and redo coronary artery bypass grafting in patients aged 47, 54, 49, 52, and 43 years respectively. All except one were high-risk patients. All patients were on circulatory support (IABP, LVAD or ECMO) before transplantation. One patient died 12 hours after the transplant due to multiorgan failure that was not reversed by improved pump function. There was one late death four months postoperatively due to severe acute vascular rejection. Three patients are doing well respectively six years, 20 and four months after transplant. We conclude that cardiac transplantation in the early postoperative period of failed conventional heart surgery is an encouraging option in highly selected patients otherwise considered to die in a very short time.

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.

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 balance and pulmonary functions during and after coronary artery bypass surgery: Ringer's acetate compared with dextran, polygeline, or albumin.

The effects on fluid balance, pulmonary functions and economics were evaluated in a randomized comparison of one colloid free and three colloid containing fluid regimens, for 48 hours during and after coronary artery bypass (CAB) surgery. A standard regimen for anaesthesia, extracorporeal circulation and monitoring was used. Only Ringer's acetate (RAc) was used as priming solution for extracorporeal circulation. Forty patients were randomized to receive either RAc, polygeline 35 mg.ml-1 (Haemaccel), dextran 70 (Macrodex) 60 mg.ml-1, or albumin 40 mg.ml-1 in saline whenever fluid volume was needed to stabilize haemodynamics. At the end of the operation, fluid retention was significantly lower in patients receiving polygeline and dextran 70, compared with patients receiving RAc. At 48 hours, however, there were no differences in cumulative fluid balance. Patients in the colloid groups postoperatively had a higher serum colloid osmotic pressure (s-COP), but a higher net lung capillary filtration pressure (delta P) only on the second postoperative day than the RAc group. However, this did not adversely affect intrapulmonary venous admixture, arterial oxygen tension, or time on respirator in the RAc group compared with the colloid groups. The most expensive colloid fluid regimen (albumin) cost about 230 US$ more per patient than the RAc fluid regimen. We conclude that Ringer's acetate for volume replacement to stabilize haemodynamics during and after CAB surgery is associated with increased fluid retention only during the intraoperative period, compared with dextran 70 or polygeline, and with a lower serum colloid osmotic pressure and net lung capillary filtration pressure postoperatively, compared with all three colloid groups. This does not affect pulmonary functions adversely.(ABSTRACT TRUNCATED AT 250 WORDS)

[Tetralogy of Fallot. A 20-year material]. / Fallots tetrade. Et 20 års materiale.

Surgical repair of tetralogy of Fallot may be a first stage palliation with secondary physiologic correction or repair as a primary procedure. The aim of palliation is to increase the pulmonary blood flow. Since 1981 we have used an interposition shunt between the subclavian and the pulmonary artery for this purpose. Repair comprises closure of the ventricular septal defect and relief of the pulmonary stenosis. A primary repair may be performed if the pulmonary arteries have reached a certain size. The size and function of the left ventricle must also be able to cope with the increased work load. During the last 20 years, 333 operative procedures have been performed in patients with tetralogy of Fallot. This material comprises 107 shunts and 226 repairs. The total mortality while in hospital was 6%. During the last period from 1990 until January 1993 the mortality in hospital was 3% after operative repair. There have been no mortality following palliative procedures during the same period.

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.

Deep vein thrombosis: a 7-year follow-up study.

OBJECTIVES: To study the sequelae of deep venous thrombosis (DVT) in terms of symptoms and objective signs of deep venous insufficiency (DVI) and their relationship to the initial extension of DVT, and to assess the control legs in the same way. DESIGN: Follow-up study after an average of 89 (range 79-102) months. SETTING: Out-patient clinic, University Hospital, Oslo. SUBJECTS: Seventy-six patients with DVT 7 years previously. At follow-up 41 patients were dead and 10 were not available for restudy, thus twenty-five patients were studied in all. MAIN OUTCOME MEASURES: Symptom rating. Objective verification of DVI by invasive pressure recordings (DVI-I) and by the Doppler ultrasound technique (DVI-D). RESULTS: At follow-up, 42% of the patients had symptoms, half of these severe, while 68% had DVI. Eighty-two per cent of symptomatic patients and 60% of the asymptomatic patients had DVI. There were no more symptoms in proximal than in distal DVT, but slightly more DVI. Control legs had neither symptoms nor DVI. CONCLUSIONS: Seven years after DVT few patients had severe symptoms, although objective signs of DVI were common. Symptoms were no more frequent after proximal than after distal DVT. We found no symptoms or DVI in control legs.

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.

Haemodynamic effects of heparin during coronary bypass surgery.

Heparin has been suggested as an activator of the plasma kallikrein-kinin system, with possible formation of bradykinin, a potent vasodilator. Haemodynamic effects and changes in the kallikrein-kinin system were studied after heparin- and saline-injections in ten patients undergoing coronary bypass surgery. A moderate decrease in mean arterial pressure was found in all patients in the observation period, but significantly more at 2 and 3 min after heparin-injection compared with saline-injection. None of the other haemodynamic variables measured were significantly different when comparing heparin- to saline-injection. Heparin-injection resulted in significant changes in the kallikrein-kinin system, with a marked increase in spontaneous kallikrein-like activity as the most prominent feature, while no changes were found after saline-injection. Liberation of bradykinin would be expected to give a decrease in systemic vascular resistance with an increase in cardiac output. The results indicate that the plasma kallikrein-kinin system, though apparently activated after heparin-injection, does not contribute significantly to the decrease in arterial pressure in the patients studied.

Transcapillary forces in muscle compartments of lower limbs with deep venous thrombosis.

Interstitial fluid pressure (Pif), plasma- and interstitial fluid colloid osmotic pressures (COPpl, COPif) were measured in muscle compartments of the calf in lower limbs of patients with deep venous thrombosis (DVT) and in contralateral limbs. Pif was measured with the 'wick-in-needle' technique in the anterior and deep posterior compartments in three periods of time following start of treatment for DVT. The first period was from 0-24 h, the second from 24-48 h and the third later than 48 h. We found a significant increase in both muscular compartments in legs with DVT compared to the contralateral in the three periods except for Pif in the anterior compartment in the third period. A significant reduction of Pif in both muscular compartments of limbs with DVT was found when comparing the first and second periods but not from the second to the third period. No difference was found in Pif in the muscular compartments of the contralateral limbs between the three periods. Interstitial fluid for COPif measurements was obtained by inserting intravenous cannulas in the deep posterior muscle compartment of the calf of limbs with DVT and contralateral limbs. In oedematous tissue it was possible to collect some fluid, but this was almost impossible in the non-oedematous tissues of the contralateral limbs. COPif was 4.2(2.4) mmHg (mean(SD)) in the deep posterior compartment of limbs with DVT, which is probably significantly reduced. Increased Pif and reduced COPif indicate increased transcapillary fluid filtration and reduced reabsorption as the main cause of oedema in muscular compartments in DVT of the lower limbs.(ABSTRACT TRUNCATED AT 250 WORDS)