The RACHS-1 risk categories reflect mortality and length of hospital stay in a large German pediatric cardiac surgery population.

OBJECTIVES: The Risk Adjusted classification for Congenital Heart Surgery (RACHS-1) was published in January 2002, based on 4370 operations registered by the Pediatric Cardiac Care Consortium. It is designed for being easily applicable also for retrospective analysis of hospital discharge data sets; the classification was not developed for patients with heart transplantations, ventricular assist devices or patients above 18 years. We apply this classification to our 2368 correspondent procedures that were performed consecutively on 2223 patients between June 1996 and October 2002 in Bad Oeynhausen and analyze its relation to mortality and length of hospital stay. METHODS: The procedures were grouped by the 6 RACHS-1 categories. Groping criteria were mainly the performed procedures; for few procedures age or diagnoses are needed in addition. The classification process itself took less than 10 working hours. Risk group frequencies in our/ the PCCC population were 1: 368/964 (15.5%/22.0%), 2: 831/1433 (35.1%/33.1%), 3: 744/1523 (31.4%/34.7%), 4: 284/276 (12.0%/6.3%), 5: 4/4 (0.2%/0.1%), 6: 137/168 (5.3%/3.8%). 18.8%/19.2% were under 1 month, 37.5%/31.6% 1-12 months of age, respectively. RESULTS: Hospital mortality (%) in our population/ the PCCC Group 1-6 was: 0.3/0.4, 4.0/3.8, 5.6/8.5, 9.9/19.4, 50.0/0, 40.1/47.7%. Geometric means of total (13.1, 19.6, 23.5, 29.1, 31.5, 52.6 days, respectively) and postoperative length of stay of survivors show significant differences between the single risk groups. The prediction capacity of the score as expressed by the area under the receiver-operator curve was nearly equal to the value found for the American hospital discharge data sets. Length of stay rises exponentially with the RACHS-1 category. However, the RACHS-1 category explains only 13.5% of the total and 16.8% of individual postoperative lengths of hospital stay in survivors. CONCLUSION: The RACHS-1 classification is applicable to European pediatric populations, too. Category Distribution, outcome class distinction capacity, distribution and mortality are similar. RACHS-1 is able to classify patients into significantly different groups concerning total and postoperative hospital stay duration, although there remains a large variability within the groups.

Stage 1 palliation of hypoplastic left heart syndrome: implications of blood gases.

OBJECTIVE: To estimate ratios of pulmonary-to-systemic blood flows (Qp/Qs) after stage I palliation (Norwood operation) for hypoplastic left heart syndrome and to determine whether early postoperative death can be associated with abnormalities of Qp/Qs ratios. DESIGN: Retrospective. SETTING: University hospital. PARTICIPANTS: Patients who underwent stage I palliation (Norwood operation) for hypoplastic left heart syndrome (n = 76). INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The results of the last intraoperative blood gas analysis were compared between patients who survived the day of operation (58 of 76) and the patients who died intraoperatively or within 4 hours after operation (18 of 76). Qp/Qs ratios were calculated using the Fick principle from arterial and venous oxygen saturations at estimated pulmonary venous oxygen saturation of 95%. A lower arterial oxygen saturation (SaO(2), 69.0 +/- 20.5% v 77.3 +/- 8.5%; p < 0.05) and more marked metabolic acidosis (pH, 7.244 +/- 0.115 v 7.298 +/- 0.095; p < 0.05; base excess, -6.8 +/- 4.4 v -3.0 +/- 4.2; p < 0.05) were observed in nonsurvivors. Calculated Qp/Qs ratios ranged between 0.2 and 6.5 in survivors and between 0.6 and 1.9 in nonsurvivors. CONCLUSIONS: Postoperative excessive pulmonary blood flow was not implicated as a cause of death based on blood gas data and Qp/Qs ratios. In nonsurvivors, low cardiac output and hypoxemia were assumed to be major problems.

Results after Norwood procedure and subsequent cavopulmonary anastomoses for typical hypoplastic left heart syndrome and similar complex cardiovascular malformations.

OBJECTIVE: From October 1989 to June 1998, 60 patients have undergone the Norwood procedure (stage I) at our institution. The results of the staged surgical reconstruction and risk factors were analysed. Typical hypoplastic left heart syndrome (HLHS) and complex lesions with aortic hypoplasia were compared with each other. PATIENTS: Typical HLHS: N = 48, median age 15 days (5-190 days), median weight 3.6 kg (2.6-5.3 kg). Complex lesions (dominant left ventricle): N = 12, median age 59 days (10-884 days), median weight 3.4 kg (2.4-12 kg). RESULTS: Typical HLHS: The stage-I hospital survival was 73% (35/48). It improved from 60% (95% confidence interval: 49-71%) during 1989-1994 to 91% (95% CI: 81-100%) during 1997-1998. Seven patients were lost late. The right ventricular end diastolic diameter (P = 0.015), shortening fraction (P = 0.027), and the presence of an obstructed pulmonary venous return (P = 0.0032) were significant risk factors. 23 children underwent stage-II operation with four (17%) deaths. All survivors experienced an improvement of their statomotoric development. Stage-III operation was performed in 13 patients with no hospital death. Follow up after stage-III procedure was 7 months to 7 years. 4 year actuarial survival, including hospital mortality and deaths at subsequent stages, improved from 28% (95% CI: 18-38%) during 1989-1994 to 58% (95% CI: 48-68%) during 1994-1997. No patient had signs of myocardial insufficiency. Complex lesions: Stage-I hospital survival was 83% (10/12) with no late death. Stage-II was performed in 8 and stage-III in 6 patients with no death respectively. CONCLUSION: In typical HLHS the results have improved over time. Both size and function of the right ventricle determined significantly stage-I survival. An early operation prevents the natural progression of pulmonary blood flow and may weaken all three risk factors. Patients with complex lesions seemed to have better chances of surviving the early postoperative period. The multistage reconstructions have become a realistic option for patients with HLHS and similar lesions, regardless of the morphologic subtype or diminutiveness of the aorta, and lead to an acceptable quality of life.

[Anatomic and functional hypoplastic left heart syndrome and its surgical Norwood and Fontan treatment]. / Das anatomische und funktionelle hypoplastische Linksherz-Syndrom und seine chirurgische Behandlung nach Norwood und Fontan.

UNLABELLED: The surgical therapy of newborns with hypoplastic left heart syndrome (HLHS) is still regarded with some distrust. The complete heart conserving palliation includes not only the Norwood operation during the newborn period but also the complete separation of both circuits by the Fontan operation some time later. Our experiences with each surgical step are presented. From 1989 to 1996, 43 infants with anatomical (n = 33) or functional (n = 10) HLHS underwent the Norwood operation. Functional HLHS were: Mitralatresia with double outlet right ventricle and subaortic stenosis (n = 2), atrioventricular septal defect with hypoplastic left ventricle, subaortic stenosis, and aortic coarctation (n = 1), hypoplastic, subaortic right ventricle with restrictive ventricular septal defect and aortic hypoplasia (n = 7). The median age at operation was 15 days (5 to 182 days), mean weight was 3.3 kg (3.0 to 4.9 kg). Total operative mortality was 32% (n = 14) with 16% since 1994 (3/19 patients). Five infants (12%) died 2 weeks to 6 months later, and 2 patients underwent cardiac transplantation. Up to now, 19 out of the 22 long term survivors underwent the bidirectional cavopulmonary anastomosis (Hemi-Fontan) at a median age of 7 months (2 to 14 months). Two infants died (10%). Up to now, 12 out of the remaining 17 survivors received the total cavopulmonary anastomosis after a mean period of 12 months. All children survived, and they are now completely palliated. The longest follow up after the complete Fontan operation is 6 years. CONCLUSION: With increasing experience the results of the Norwood operation improved. The following two-stage Fontan procedure bears only a low risk and leads to good quality of life.

Stentless xenograft valve in a truncus arteriosus communis.

We report the use of a recently developed stentless porcine valve in a 2-year-old child with truncus arteriosus communis (type I). The child had received no previous surgical palliation. Despite a markedly increased pulmonary arteriolar resistance the right ventricle was connected to the pulmonary artery with a homograft. The postoperative course was complicated at first by right ventricular failure due to pulmonary hypertension and secondarily by left ventricular failure caused by rapidly increasing truncal valve incompetence. Because we did not have a suitable homograft at hand we replaced the truncal valve with a stentless porcine xenograft valve.

Coexpression of alpha and beta myosin heavy-chain isoforms in atria of neonates and infants with congenital heart disease.

The relative amounts of cardiac myosin heavy-chain isoforms (MyHC) in right atrial tissue (RA) of 16 neonates and children suffering from congenital heart disease have been investigated. Quantification of MyHC was based on one-dimensional gel electrophoresis and on histometrical evaluation of cyro-sections stained with monoclonal antibodies against alpha- and beta-MyHC. The mean right atrial pressures ranged from 2 to 14 Hg. The RA load was normal in eight patients (5.1 +/- 1.3 mm Hg) and overloaded in eight cases (10 +/- 2.5 mm Hg). The arterial oxygen saturation was normal in 12 and ranged between 85% and 89% in four cases. In all patients a large proportion of atrial myocytes coexpressed alpha- and beta-MyHC. However, in the cases with pressure overloaded RA the amount of beta-MyHC was found to be 1.6 times higher than in the cases with normal pressure. This indicates an adaptational response to overload, as was previously described for the adult human heart. In light of this finding, it seems important to conserve as much as possible of the trained right atrial wall during a Fontan type of operation.

Early rhythm disorders after arterial switch and intraatrial repair in infants with simple transposition of the great arteries.

Between 1986 and 1990 68 infants with transposition of the great arteries (TGA) have been repaired within their first six months of life. From the 51 simple TGA, forming the study group, 20 underwent intraatrial repair according to Mustard and Brom (group I) and 31 received the arterial switch procedure (group II). We compared the observed rhythm disorders after both surgical methods. There were no deaths in group I and 3 deaths in group II. Group I: 7 cases (35%) had early postoperative arrhythmias, 6 patients received transient therapy. There were 3 cases of supraventricular tachycardia (SVT), 2 cases of frequent supraventricular premature beats (SVPB), 1 infant with temporary AV junctional rhythm and 1 with temporary total AV block. Both needed external pacemaker support for maximal two days. Group II: 14 cases (50%) presented rhythm disorders, 13 patients received transient therapy. There were 4 SVT and 3 SVPB. The time of occurrence was between the first and the sixth postoperative day. 2 cases of temporary AV junctional rhythm and 2 infants with transient total AV block needed external pacemaker support for maximal two days. 3 cases with relative sinus bradycardia (less than 130 beats/min) improved clinically with temporary external pacemaker support for several hours. Finally all patients of both groups were in sinus rhythm and after two months all antiarrhythmic drugs could be stopped. There have not been any ventricular arrhythmias in the early postoperative period. During the whole follow up (mean 28 months, range 4-60 m) no arrhythmias reappeared with two (4%) exceptions.

Primary repair of complete atrioventricular septal defect in infancy.

Forty infants with complete atrioventricular septal defect have undergone primary repair within their 1st year of life. The mean age at time of surgery was 4 months (range 1-12 months) and the mean weight 4.2 kg (range 2.9-7.0 kg). Either the one- or the two-patch method was used. Four patients died (10%). There were no late deaths. Three patients needed reoperation due to mitral valve insufficiency. Postoperative complications were: 1 total atrioventricular block in an infant with an absent ventricular septum 3 weeks after surgery, 9 complete right bundle branch blocks, 3 small residual ventricular septal defects. Mean follow-up of the 36 survivors is 22 months (3-46 months). Thirty (83%) are in NYHA functional class I, 4 (11%) in NYHA class II and 2 (6%) in NYHA class III. No atrioventricular valve regurgitation is detectable by the color Doppler technique in 19 (53%) patients. Mitral incompetence is mild in 13 cases (36%) and moderate in 4 (11%). In ten recatheterized patients the average systolic pulmonary to systemic artery pressure ratio dropped from 1.0 to 0.42 and the average pulmonary arteriolar resistance was reduced from 5.5 to 3.9 U/m2. All but one patient gained weight and underwent adequate statomotoric development. Primary repair should be performed at the age of 5-6 months or even earlier, if pulmonary arteriolar resistance exceeds 5 U/m2.

Surgical repair and postoperative course of an infant with infracardiac total anomalous pulmonary venous connection, cor triatriatum sinistrum and transposition of the great arteries.

The combination of a d-transposition of the great arteries, cor triatriatum sinistrum and a total anomalous pulmonary venous connection of the infracardiac type is a very rare condition. Up to now, one surgical repair in an adolescent with transposed great arteries and total anomalous pulmonary venous drainage of the supracardiac type has been reported. In this paper, an infant with the above mentioned cardiovascular malformation is presented. The common pulmonary vein drained into the inferior vena cava and was obstructed. There were arborisation abnormalities in both lungs with mild pulmonary hypertension. The infant has been successfully operated upon at the age of 6 months and a weight of 4.5 kg. The membrane within the left atrium was resected, the common pulmonary vein was anastomosed to the left atrium and a Mustard procedure was performed. During the first 6 postoperative weeks, the infant had problems with adaptation. There was both a transient ballooning of the Mustard patch with significant obstruction of the pulmonary venous drainage and a delayed pulmonary recovery. Two months later, the patch was straightened and the child could be discharged from hospital. After 12 months, the child died from an infection of the airways.

[Changes in fatty acid metabolism in dilated cardiomyopathy and hypertrophic cardiomyopathy in childhood]. / Veränderungen im Fettsäuremetabolismus bei dilatativer und hypertropher Kardiomyopathie im Kindesalter.

The aetiology and the pathomechanisms in both types of cardiomyopathy (CM) are still unknown. In vivo measurements of myocardial metabolism in CM may be useful, but there is hardly any information on this subject. Free fatty acids (FFA) are the main source of energy for the normal myocardium. A method for external measurement of the FFA extraction rate (FFA-ER) in different myocardial regions by the simultaneous use of two isotopes has been developed. 201 TI indicates the myocardial perfusion and 15-(p-123 I-phenyl)-pentadecanoid acid (IPPA) represents the FFA uptake. The relation of IPPA/TI reflects the FFA-ER. 8 patients with hypertrophic CM (HCM), age 0.2-20 years, and 8 patients with dilated CM (DCM), age 0.2-18 years were investigated. 12 healthy adults and 4 infants after an arterial switch operation were used as a control group. All patients with HCM showed normal myocardial perfusion but the FFA uptake was strongly diminished, resulting in a reduction in FFA-ER to 42 +/- 12% of the normal value. The maximal influx rate (IR) of FFA was diminished too. In patients with DCM both the myocardial perfusion and the FFA uptake were globally reduced, resulting in a virtually normal FFA-ER. The IR was slightly increased. In HCM and DCM FFA utilisation was disturbed. The alterations were significantly different in both types of CM and different pathomechanisms can be assumed.

Ewing's sarcoma metastazing from the right pelvis via the inferior vena cava into the heart: diagnosis and successful surgical treatment.

A case of Ewing's sarcoma metastazing from the pelvis into the heart is presented. Fainting as a result of circulatory obstruction within the right heart was the leading symptom. Open-heart surgery was performed to excise the tumor from the right atrium and the inferior vena cava. Postoperative angiography showed the tumor coming up out of the right iliac vein and growing along the wall of the inferior vena cava.

[Postnatal development of the sympathoadrenergic system in premature and newborn infants]. / Postnatale Entwicklungen sympatho-adrenerger Systeme bei Früh- und Neugeborenen.

Autonomic regulatory mechanisms and some metabolic functions are predominantly influenced by the sympathetic nervous system. Premature and mature newborns show a high variability and a low adaptability of the sympathetic nervous system. In order to investigate whether sympathetic systems are completely developed at birth or underly a postnatal maturation process we have determined plasma levels of adrenaline and noradrenaline as well as the density and affinity of alpha- and beta-adrenoceptors on thrombocytes and lymphocytes in pre- and mature newborns and adults. Catecholamines were determined by means of a radioenzymatic method, number and affinity of adrenoceptors by use of the radioactive labelled antagonists 3-H-Yohimbine and 125-I-Cyano-Pindolol. Beta-adrenoceptor responsiveness was assessed by measurement of cyclic AMP in lymphocytes before and after stimulation of beta-adrenoceptors by isoprenaline. A linear relationship occurred between the gestational age and the number of adrenoceptors on lymphocytes, whereas the alpha-adrenoceptors on thrombocytes showed no age dependency. The basal content of cyclic AMP and the accumulation in response to beta-adrenoceptor stimulation by isoprenaline was significantly lower in newborns than in adults. Since noradrenaline and adrenaline plasma levels were not significantly different in newborns and adults it is assumed that the low density of beta-adrenoceptors in premature and mature newborns is due to a postnatal maturation and not to a "down regulation" by circulating catecholamines. Our results suggest that an immaturity of beta-adrenoceptors is involved in the poorly developed adaptive control in newborns.

Spectrum of hearts with one underdeveloped and one dominant ventricle.

Hearts with one underdeveloped and one dominant ventricle form a spectrum of anomalies extending from the heart with two clearly adequate chambers to those with a true single ventricle. An angiographic concept of grouping such hearts is presented. 129 patients with unequal ventricles underwent catheterization and cineangiography between 1974 and 1983. The age at first catheterization ranged from one day to 24 years (mean 3.9 years). Male-female ratio was 2:1. Five groups of hearts (with their relative frequencies in the spectrum) were established: dominant left ventricle (53%); dominant right ventricle (20%), each with normally related chambers; dominant left ventricle (20%); dominant right ventricle (3%), each with ventricular inversion; true single ventricle (5%). The incidence of atrial anatomy, venous return, intracardiac connections and associated lesions within each group was assessed. From the standpoint of deranged physiology as well as surgical implications there are more similarities than differences among these hearts. The fact that one ventricle will not generate an adequate stroke volume after repair is overwhelmingly more important than most other considerations. For the diagnostic and surgical approach, we believe that the system offers many advantages.

Angiographic anatomy of hearts with one ventricular chamber: the true single ventricle.

Fifteen patients with an isolated ventricular chamber were studied using angiography. These patients constitute 7% of the 218 patients with single ventricle who were examined at our institution over a 10-year period. The atrial situs was solitus in 6 and isomeric in 9. The ventricular chamber showed 3 varieties based on trabecular pattern: right type, mixed and indeterminate. The atrioventricular (AV) connection was through 2 valves in 4 hearts, through a common AV valve in 8 and through a single valve in 3. The morphologic features of the ventricular chamber and the AV valves are considered the most important factors for planning surgical management.

[Pulmonary function during a 10-day successful extracorporeal CO2 elimination in acute respiratory failure. Case report]. / Lungenfunktion während 10-tägiger erfolgreicher extrakorporaler CO2-Elimination bei schwerem akuten Lungenversagen. Fallbricht.

Extracorporeal CO2-removal (ECCO2-R) with low-frequency positive-pressure ventilation (LFPPV) may relieve the acutely injured lung from the burden and the risks of excessively high ventilatory minute volumes and airway pressures. It was the purpose of this study to document the evolution of lung function during clinical ECCO2-R with special emphasis on extravascular lung water. ECCO2-R was applied in a 21-year-old female patient suffering from severe post-traumatic infectious adult respiratory distress syndrome. The indication for ECCO2-R was based on the following findings: total static lung compliance 25 cm X cm H2O-1; arterial pO2 50 mm Hg with an inspiratory oxygen concentration of 100%; intrapulmonary right-to-left shunt over 50% of the cardiac output; and extravascular lung water 24 ml X kg-1 (normal 4.5-7 ml X kg-1). ECCO2-R was shown to provide satisfactory conditions for improving the above-mentioned abnormal parameters of pulmonary function. Pressure-limited low-frequency mechanical ventilation allowed successful management of several pneumothoraces with bronchopleural fistulas which occurred during the procedure. It is concluded that these complications of positive airway pressure would have led to the patient's death under the conditions of conventional mechanical ventilation.