G von Bernuth

Growth of pulmonary artery after arterial switch operation for simple transposition of the great arteries

Abstract This retrospective study attempts to assess the size and growth pattern of the pulmonary artery about 1 year after neonatal arterial switch operation for simple transposition of the great arteries. Sixty-seven patients underwent cardiac catheterization, including catheterization of the right and left pulmonary arteries, and right ventricular angiography an average of 13.9 months after arterial switch operation. In 34 of these patients pre-operative right ventricular angiocardiograms were available. The diameter of the main pulmonary artery and that of its proximal right and left branches were measured. The values were compared to those of normal children matched for body surface area, taken from the literature and, for the branch values, related to the degree of branch stenosis and to the corresponding values, measured on pre-operative angiocardiograms. The cross-section of the main pulmonary artery after arterial switch operation with Lecompte manoeuvre becomes oval. The branches of the pulmonary artery are sometimes underdeveloped and this finding is related to branch stenosis. The ratio of the branch gradients is inversely proportional to the growth ratio of both branches while the pre- and postoperative Nakata indices are identical. Conclusion The Lecompte manoeuvre induces a flattening of the main pulmonary artery with concomitant reduction of its cross-sectional area. There is also frequently trivial or rarely moderate branch pulmonary stenosis which is accompanied by growth retardation of the concerned branch.

Cardiological and general health status in preschool- and school-age children after neonatal arterial switch operation.

OBJECTIVE Cardiological and general health status 3-9 years after neonatal arterial switch operation for transposition of the great arteries should be evaluated by non-invasive methods. METHODS A total of 77 unselected children with intact ventricular septum (75.3%) or ventricular septal defect (24.7%) without or with aortic isthmic stenosis (5.2%) were prospectively examined 3.2-9.4 years (5.4 +/- 1.6) after neonatal switch. Clinical pediatric and cardiological examination, standard and 24 h Holter electrocardiogram, M-mode, 2D-, Doppler and colour Doppler echocardiography were performed. Outcome data were compared to published normals. RESULTS Reoperation rate was 2.6%, 96.1% were without limitation of physical activity and 98.7% without medication. Compared to normals, growth was adequate, weight and head circumference were slightly reduced. After median sternotomy, 23.4% had abnormal thoracic configuration (16.9% asymmetry, 6.5% funnel chest). ECG and Holter: 93.5% were in sinus, 6.5% in ectopic atrial or junctional rhythm. Incidence of complete right bundle branch block was 15.8% in patients with ventricular septal defect and 5.2% in those without. Ischemic ST-T changes during exercise due to coronary artery occlusion and evidence of old myocardial infarction were found in 1 patient (1.3%) each. Occasional atrial ectopy was found in 27.4%, ventricular ectopy in 15.3%: occasional in 12.5% and frequent (> 30/h) in 2.8% presenting bigemini, couplets and short runs of ventricular tachycardia at rest and during exercise. Echocardiography: Left ventricular function was normal in all. Endsystolic diameter of neoaortic valve annulus was beyond 90% confidence interval for controls in 79.2%, neoaortic root diameter in 100%. Mild aortic insufficiency was seen in 10.4%. No correlation was found between aortic insufficiency and aortic dilatation. Neoaortic stenosis was not seen, mild residual coarctation after end-to-end-anastomosis was found in 2.6%, native coarctation corrected later on in 1.3%. Supravalvular pulmonary stenosis was seen in 29.9% (19.5% trivial, 7.8% mild, 2.6% moderate), mild subvalvular pulmonary stenosis in 1.3%, pulmonary insufficiency in 2.6%. CONCLUSION The study confirms good midterm results after neonatal arterial switch operation for transposition with or without ventricular septal defect. Long-term observation is necessary to assess rhythm, coronary artery and myocardial function as well as development of neo-aorta and pulmonary artery system.

Tachyarrhythmic syncopes in children with structurally normal hearts with and without QT-prolongation in the electrocardiogram

Forty children with presumed ventricular tachyarrhythmic syncopes in the absence of structural heart disease were studied.Twenty-nine patients, one of whom was deaf, had a prolonged QT-interval in the resting electrocardiogram (Group 1); eleven patients had a normal QT-interval (Group 2). The median QTc-interval was 0.51s in Group 1 and 0.40s in Group 2. Familial occurence suggesting autosomal dominant inheritance was found in 21 of 28 normally hearing patients in Group 1 and in 2 of 11 patients in Group 2. Syncopes were definitely stress-induced in 22 patients in Group 1 and in all 11 patients in Group 2. Of 23 patients in Group 1 in whom an electrocardiogram was obtained during physical exercise, only one showed severe ventricular dysrhythmia. In contrast, all eleven patients in Group 2 developed severe ventricular dysrhythmia with exercice. Treatment with beta-blocking medication prevented further syncopes in 15 of 19 patients with several previous attacks in Group 1 and in 3 of 5 patients of Group 2. Four of the 29 patients in Group 1 died suddenly and one more remained apallic after an attack. Of the 11 patients in Group 2, four died suddenly and one retains severe cerebral damage after resuscitation from ventricular fibrillation.We conclude that, besides the group of patients with the long QT-syndrome, there may be a distinct group of patients with a consistently normal QT-interval and severe ventricular dysrhythmia with exercise. Patients of both groups are threatened by sudden death and are improved by treatment with beta-blocking medication.

Neurodevelopmental outcome related to cerebral risk factors in children after neonatal arterial switch operation

BACKGROUND Neurodevelopmental outcome after neonatal arterial switch operation for complete transposition of the great arteries is an important topic needing prospective assessment. METHODS A group of 33 unselected children (3.0 to 4.6 years) operated on as neonates with combined deep hypothermic circulatory arrest and low flow cardiopulmonary bypass and a control group of 32 age-matched healthy children (3.0 to 4.8 years) underwent evaluation of socioeconomic and clinical neurological status and a standardized test comprising all areas of child development. Results of patients were related to those of the control group, to population norms, and to preoperative, perioperative, and postoperative cerebral risk factors. RESULTS Clinical neurological status was normal in 26 patients (78.8%) and reduced in 7 (21.2%). Complete developmental score and the subscores for motor function, visual perception, learning and memory, cognitive function, language, and socioemotional functions were not different compared to population norms. Compared to the patients, the children of the control group scored higher on tests of complete development, cognition, and language, but also on socioeconomic status. Complete developmental score and the scores for motor, cognitive, and language functions were weakly inversely related to the duration of circulatory arrest, but not to the duration of bypass. Cerebral risk factors such as serum levels of the neuron-specific enolase, perinatal acidosis, perinatal asphyxia, peri- and postoperative cardiocirculatory insufficiency, or clinical seizures were not correlated to the test results. CONCLUSIONS Neonatal arterial switch operation with combined circulatory arrest and low flow bypass is associated with neurological impairment, but not with reduced development as assessed by formal testing of motor, cognitive, language, and behavioral functions. Perioperative serum level of the neuron-specific enolase is not a valid marker for later developmental impairment.

Mittelfristige Ergebnisse nach arterieller Switch-Operation im Neugeborenenalter bei Transposition der großen Arterien

ZusammenfassungFragestellung: Die arterielle Switch-Operation ist Methode der Wahl zur operativen Korrektur einer einfachen Transposition der großen Arterien im Neugeborenenalter. Bei erfreulichen kurzzeitigen Ergebnissen ist die Langzeitprognose der betroffenen Kinder unklar. Methode: Wir analysierten retrospektiv den Verlauf von 165 konsekutiven Patienten mit Transposition der großen Arterien nach arterieller Switch-Operation zwischen 1986 und 1993 (Operationsalter 9±7 Lebenstage; 15 Patienten mit gleichzeitigem Verschluß eines Ventrikelseptumdefekts; Nachbeobachtungsdauer 43±24 Monate). Ergebnisse: Neun Patienten (5,5%) verstarben früh, 3 Patienten spät nach der arteriellen Switch-Operation. Bei 4 Patienten bestehen Zeichen eines alten Myokardinfarkts im EKG. 18 Patienten weisen eine deutliche Stenosierung der peripheren Pulmonalarterien auf (Dopplergradient 30–50 mmHg, n=14; >50 mmHg, n=4). Aufgrund einer Aortenisthmusstenose (9 Patienten), peripheren Pulmonalstenose (1 Patient), subvalvulären Pulmonalstenose (1 Patient) und eines Verschlusses der linken Koronararterie (1 Patient) wurden bei 12 Patienten Zweitoperationen durchgeführt. Bei 22 Patienten besteht der Verdacht auf eine psychomotorische Retardierung, 2 Kinder entwickelten einen ventilpflichtigen Hydrozephalus. Nach dem 2. Lebensjahr erhielt nur 1 Kind (mit ventrikulärer Arrhythmie) eine medikamentöse Therapie. 131 Kinder entwickeln sich normal. Schlußfolgerung: Die arterielle Switch-Operation führt in der Regel zu sehr guten mittelfristigen Ergebnissen. Langfristige Nachbeobachtungen sind jedoch erforderlich, um definitive Aussagen über die Dauerhaftigkeit der guten Ergebnisse machen zu können.SummaryQuestion: The arterial switch operation is the procedure of choice for the neonatal anatomical repair of a simple transposition of the great arteries. Whereas short term prognosis is good, long-term prognosis is uncertain. Method: Between 1986 und 1993 165 children with a mean age of 9±7 days underwent arterial switch operation for transposition of the great arteries (closure of a ventricular septal defect simultaneously performed in 15 patients). To obtain more detailed insight into the prognosis of children after the arterial switch operation, we analyzed the clinical outcome of our patients (mean follow-up period 43±24 months), especially considering the prognosis of postoperative complications. Results: Early mortality was 5.5% (9 patients), and there were 3 late deaths. During follow-up electrocardiographic signs of myocardial infarction persisted in 4 patients. Significant supravalvular pulmonary stenosis was noted in 18 children (pressure gradient 30–50 mmHg: n=14; >50 mmHg: n=4). Twelve patients had a second operation: 9 for aortic coarctation, 2 for supra- and subvalvular pulmonary stenosis respectively, and 1 for occlusion of the left coronary artery. Neuromotor function is normal in 131 children while 22 children are suspected to have a developmental delay. Posthemorrhagic hydrocephalus requiring a ventriculoperitoneal shunt occured in 2 patients. After two years of life only 1 patient is medicated (for exercise-induced ventricular arrhythmia). Conclusion: Interim results after the arterial switch operation for transposition of the great arteries are encouraging. Further observations should clarify the long term prognosis of affected children.

Myocardial cell damage related to arterial switch operation in neonates with transposition of the great arteries

It was of objective of this study to investigate clinical and laboratory risk factors for myocardial dysfunction (MD) in neonates after arterial switch operation for transposition of the great arteries.

Evaluation of myocardial cell damage in cardiac lymph during cardiopulmonary bypass.

Cardiac lymph is a medium which reflects the myocardial interstitial space. The purpose of our study was to evaluate the inflammatory response marker TNFα (tumor necrosis factor-α) and myocardial injury marker Troponin I during cardiopulmonary bypass (CPB) in cardiac lymph and to compare them with blood values taken from the coronary sinus (SC).

Invasive Diagnostik angeborener Herzfehler

Mit Einfuhrung der invasiven Diagnostik, das heist von Herzkatheterisierung und Angiokardiographie, wurde es moglich, Funktion und Anatomie des intakten menschlichen Herzens zu untersuchen. Damit gelang auch die genaue Analyse angeborener Herzfehler. Gleichzeitig trug die invasive Diagnostik durch ihre kontrollierende Funktion wesentlich zur Verfeinerung alter nicht-invasiver Untersuchungstechniken wie der klinischen Untersuchung sowie zur Entwicklung neuer Methoden wie z.B. der Echokardiographie bei.