[Improvement of perioperative hemodynamics and gas exchange by inhalation of nitric oxide in children with congenital cardiopulmonary defects]. / Verbesserung der perioperativen Hämodynamik und des Gasaustausches durch Inhalation mit Stickstoffmonoxid bei Kindern mit angeborenen kardiopulmonalen Fehlbildungen.

OBJECTIVES: In critically ill infants and children before or after surgery for congenital cardiopulmonary disease it was evaluated whether continuous NO inhalation can reduce pulmonary artery pressure (PAP) and improve arterial oxygen saturation (SaO2). METHODS: All patients (n = 24; age 1 day-6.5 years) were intubated and artificially ventilated. They had either secondary pulmonary hypertension (n = 16), acute respiratory distress syndrome (n = 3), or reduced SaO2 (n = 5) due to pulmonary hypoperfusion. NO was introduced into the afferent limb of the ventilator circuit close to the endotracheal tube, while continuously measuring the inspired NO and O2 concentrations. The initially applied concentration of NO was 20 +/- 2.0 ppm. RESULTS: The hemodynamic condition and/or oxygen saturation was significantly improved by NO in 23 patients (95%). Mean PAP declined significantly from 45 +/- 7 to 28 +/- 3.7 mm Hg, while mean systemic arterial pressure remained constant (56 +/- 2.1 vs. 58 +/- 2.5 mm Hg). This was related to a selective reduction in pulmonary vascular resistance by 48 +/- 8.5%. SaO2 increased significantly (p < 0.05) from 83 +/- 2.5% to 93 +/- 1.5% due to a decreased intrapulmonary right-to-left shunt. NO therapy was applied with a median of 6 days (range 1.5-36 days). During NO inhalation methemoglobin concentration was significantly increased (0.77 +/- 0.05% vs. 1.46 +/- 0.15%), but neither was oxygen transport capacity affected, nor was any evidence for accumulation observed. Using a model ventilatory circuit, a nitric dioxide (NO2) formation of 1.14 +/- 0.11% of the applied NO concentration was measured, i.e. approximately 0.5 ppm NO2 at 40 ppm NO. This amount of NO2 in the inspired gas is well below toxicologically relevant concentrations. CONCLUSIONS: Low-dose NO inhalation selectively reduces PAP and improves SaO2 in children with congenital cardiopulmonary disease during perioperative intensive care. It is expected that the overall hemodynamic improvement is related to a reduced afterload of the subpulmonary ventricle without changes in coronary perfusion pressure, as is often observed with other vasodilators applied intravenously. We recommend an upper dose limit of 40 ppm NO for continuous NO inhalation to avoid possible toxicologically relevant NO2 concentrations.

[Spiro-ergometric studies after corrective surgery of tetralogy of Fallot]. / Spiroergometrische Untersuchungen nach Korrekturoperation einer Fallotschen Tetralogie.

Treadmill exercise electrocardiography and spirometry was recorded in 61 patients (aged from 5 to 23 years) who underwent corrective surgery for tetralogy of Fallot. The Bruce-protocol and a modified interval-protocol were compared to find out their ability to induce arrhythmias. Supraventricular arrhythmias were predominantly provoked by the interval-protocol while maximal exhaustion is often necessary for inducing ventricular arrhythmias which we found in 23% of our patients. Most of them were seen in the periods of recovery between or after the exercise. We propose a combination of both protocols, which will be compared with established forms of exercise-testing in further studies. The results of rhythm analysis of consecutive exercise tests in one patient after surgical repair could possibly be compared by using an "arrhythmia score" which tries to indicate the severity of different forms of arrhythmias. The values of maximal heart rate, oxygen consumption and endurance time recorded at maximal exercise (Bruce-protocol) were about 15% lower than correspondent values of healthy persons found by other authors.

[Progress and change in nuclear magnetic resonance diagnosis of congenital and acquired heart defects]. / Fortschritt und Wandel der kernspintomographischen Diagnostik bei angeborenen und erworbenen Herzfehlern.

Since 1987, the authors have examined 186 patients (76 girls, 110 boys) with a variety of congenital and acquired heart diseases by means of magnetic resonance imaging (MRI). The patients' ages ranged from 2 days to 20 years (mean age 3.97 years). During the study new techniques were developed, which extend the applicability of MRI. Especially two-dimensional and three-dimensional angiography are demonstrated and discussed. Using all diagnostic tools offered by MRI more than 90% of cardiovascular malformations are correctly diagnosed. MRI is of unquestionable value in the evaluation of the thoracic and abdominal aorta and in demonstrating the vascular status in pulmonary atresia and anomalous pulmonary venous return. It may be useful if question arise in patients with complex lesions, intra- and extracardiac tumours and cardiomyopathies. Although MRI is still under investigation, it may play a major role in diagnosing congenital heart diseases.

[Anomalous origin of the left coronary artery from the pulmonary artery. Variability of clinical aspects, echocardiography and angiography findings]. / Fehlabgang der linken Koronararterie aus der Pulmonalarterie. Variabilität von Klinik, echokardiographischen und angiographischen Befunden.

OBJECTIVES: Analysis of symptoms, diagnostical difficulties and follow-up in infants and children with anomalous origin of the left coronary artery from the pulmonary artery. METHODS: Retrospective study; 12 children between 3 weeks and 2 years old; time period: 1980-1991. RESULTS: Three infants were detected on routine examination because of a new cardiac murmur, the others presented with signs of cardiac failure. Cross sectional echocardiography and color Doppler flow mapping allowed to verify the suspected diagnosis. However, in one infant a false-negative cross sectional echocardiographic result was obtained. In this case nuclear magnetic resonance imaging was able to delineate the exact anatomy. Mean preoperative left ventricular ejection fraction: 33 +/- 4%; percentage of infants below the age of 6 months: 92%; surgery related mortality: 66%; mean follow-up of the remaining 4 patients being in good clinical condition: 2.9 +/- 1 years. CONCLUSIONS: 1. An anomalous origin of the left coronary artery should be included into the differential diagnosis when a new cardiac murmur is detected. 2. Possibility of false-negative echocardiographic results is emphasized. 3. With early symptoms and highly reduced left ventricular function, the mortality is still high.

[Adrenocortical nodular hyperplasia as a cause of Cushing syndrome in the neonatal period]. / Adreno-corticale noduläre Hyperplasie als Ursache eines Cushing-Syndroms in der Neugeborenenperiode.

The case report of a female child born preterm (30th wk of gestation) who developed symptoms of Cushing's syndrome beginning in the neonatal phase is presented. The disease was caused by a unilateral adreno-cortical nodular hyperplasia and was successfully treated by unilateral adrenalectomy. Preoperative treatment with ketoconazole and metyrapone proved to be effective. Symptoms of Cushing's disease including hypertrophic cardiomyopathy were completely reversible within one month after surgery.

Early and late results of the modified Waterston shunt with PTFE grafts for palliation of complex congenital cyanotic heart disease in neonates.

During a 12 year period from 1978 to 1989, 35 infants under 4 weeks of age underwent palliative surgery for complex congenital cyanotic heart disease with a short (1-1.5 cm) PTFE graft between the ascending aorta and the right pulmonary artery (modified Waterston shunt). Twenty-three infants had pulmonary atresia and 14 had severe pulmonary stenosis. Underlying cardiac lesions were tetralogy of Fallot (n = 11), single ventricle (n = 7), transposition complexes (n = 6), and intact ventricular septum and hypoplastic right heart syndrome (n = 13). There were 4 early deaths (10.7%) in the entire series, 2 of which were shunt related. Three of the 4 occurred during our initial experience with this shunt in 1978 and 1979. They led to the modified Waterston shunt being abandoned for 3 years in favor of other shunt procedures. Since 1983 one early death occurred in 28 infants (3.5% mortality) with no death in the latest 26 patients. All patients were followed up between 6 and 108 months. There were 4 late deaths, one of which was shunt related. We observed a significant difference in the shunt patency rate between 4 and 5 mm grafts: palliation was adequate after 2 years in 52% of the patients when a 4 mm graft was used and in 89% of the 5 mm graft group (p less than 0.005). Reshunting was necessary in 7 infants between 5 and 60 months after primary surgery. Recatheterization was performed in 17 infants for suspected shunt failure (n = 6) or diagnostic reasons (n = 11).(ABSTRACT TRUNCATED AT 250 WORDS)

[Aneurysm of the vein of Galen as a possible cause of congestive heart failure: a report of 5 new cases]. / Aneurysma der Vena Galeni als mögliche Ursache für eine kongestive Herzinsuffizienz: Bericht über 5 neue Fälle.

We report on five newborns with an arterio-venous malformation of the vein of Galen. All newborns were cyanotic and in congestive heart failure without any evidence of congenital heart disease. Congestive heart failure in these cases was mainly due to an almost two-fold increase in cardiac output of approximately 8 l/min/m2 (normal: 4.5 l/min/m2). According to previous reports, mortality is very high in patients with this malformation when becoming symptomatic during infancy, and therapy by surgery or embolization is only successful in 10-30%. While three of our patients died shortly after diagnosis because of untreatable heart failure, the other two were operated on either by subtotal ligation of the draining vein or by ligation of 4 arterial feeders. In the first case secondary thrombosis of the aneurysm occurred and cardiac failure subsided. In the second case a large shunt remained and a balloon-embolization was performed successfully. However, in both patients severe neurologic defects occurred, the severity of which remains to be assessed later since the post-operative observation period is only 2 and 5 months, respectively.

Repair of symptomatic aortic coarctation in the first three months of life. Early and late results after resection and end-to-end anastomosis and subclavian flap angioplasty.

During a 9 year period between January 1977 and December 1985, 98 consecutive infants under 3 months of age underwent surgical repair of symptomatic aortic coarctation. Resection and end-to-end anastomosis was performed in 73, subclavian flap angioplasty in 14, and other procedures in 11 patients. There were 20 (20.5%) early and 12 (12.5%) late deaths. No early deaths occurred in the isolated coarctation group. Associated complex cardiac malformations and age under 2 weeks at operation influenced significantly early and late outcome but not any particular surgical procedure. The survivors were followed from 6 months to 8 years and 8 months postoperatively. There were 16 (28%) re-coarctations among 56 survivors after end-to-end anastomosis requiring re-operation in 7 (12%) infants and 3 (30%) re-coarctations among 10 survivors after subclavian flap angioplasty requiring re-operation in 1 infant. After end-to-end anastomosis re-coarctation as well as re-operation rate was markedly lower when an interrupted suture line for the entire anastomosis was used as compared to the group with a continuous suture line of the posterior aortic wall (21% vs. 33% re-coarctation rate and 4% vs. 18% re-operation rate respectively). From our results it is concluded that subclavian flap angioplasty for relief of aortic coarctation in early infancy is not superior to resection and end-to-end anastomosis. In the end-to-end anastomosis group an interrupted suture line has a lower re-coarctation as well as re-operation rate as compared to a continuous suture line of the posterior aortic wall.

[Hypertrophic cardiomyopathy in childhood: studies of the clinical picture and course]. / Hypertrophe Kardiomyopathie im Kindesalter: Beobachtungen zum klinischen Bild und Verlauf.

30 children with hypertrophic cardiomyopathy and a mean age of 4.9 +/- 4.4 years, 11 infants and 19 children, were followed up for an average of 6.6 +/- 5.6 years. Apart from a systolic murmur in nearly all patients, only a few had symptoms. 7 patients from 5 families had a positive family history. The ECG often showed left ventricular hypertrophy, twice a deep Q wave, and no cases of giant T wave. A subaortic pressure gradient greater than or equal to 40 mm Hg was measured in 6 children, a secondary cardiomyopathy was excluded by biopsy in 8. Associated lesions were coarctation (n = 2) and patent ductus arteriosus (n = 2). 7 children died: 1 infant in congestive heart failure, 3 children suddenly, and 3 postoperatively. The annual mortality rate was 3.5%. Also in childhood, beta-blocker therapy may not prevent sudden cardiac death, nor may operation prevent late complications. In spite of the lack of pressure gradients, the children did not fulfil the criteria of hypertrophic non-obstructive cardiomyopathy.

[Combination of ventricular septal defect with unilateral pulmonary artery stenoses and contralateral pulmonary hypertension]. / Uber die Kombination von Ventrikelseptumdefekt mit einseitigen Pulmonalarterienstenosen und kontralateraler pulmonaler Hypertonie.

The clinical features and hemodynamics of the triad ventricular septal defect, unilateral stenoses of the pulmonary artery and contralateral pulmonary hypertension are illustrated by means of 2 case histories. At the time of cardiac catheterization the discovery of severe pulmonary vascular disease of the left lung with inoperable multiple peripheral stenoses of the right pulmonary artery in the first patient, a 4-year old girl, meant that corrective surgery could no longer be undertaken. The second patient, a 10-month old infant with embryofetal alcohol syndrome, suffered from peripheral and two central stenoses of the right pulmonary artery with a total systolic pressure gradient of 62 mm Hg. In our opinion, if the contralateral pulmonary vascular bed has to cope largely single-handed with the increased cardiac output of the right ventricle as a result of additional unilateral pulmonary artery stenosis, the hemodynamic impact of a ventricular septal defect is considerably increased and the development of pulmonary vascular disease accelerated. Since there are strong indications that unilateral pulmonary artery stenosis has more than a mere mechanical effect causing regulative (humorally?, neurogenically?) pulmonary hypertension due also to increased resistance, we are in favour of early closure of the ventricular septal defect and additionally, if technically possible, of an angioplasty or a bypass operation of the stenosis. Since, to the best of our knowledge, an association between proved pulmonary artery stenoses and embryofetal alcohol syndrome has not yet been reported, we feel that our findings are worth sharing.

[Echocardiographic diagnosis and successful resection of a left ventricular rhabdomyoma in the neonatal period]. / Echokardiographische Diagnose und erfolgreiche Resektion eines linksventrikulären Rhabdomyoms im Neugeborenenalter.

Among the rare heart tumours in childhood rhabdomyomas are mostly found in the neonatal period. They cause in 50% left- or right-sided obstruction. By means of 2D-echocardiography extent and relation to neighbouring structures can clearly be disclosed. Because of the severe natural history - mortality in the first year of life between 60 and 78% - successful operation was performed in a 2,5 kg weighing neonate.

[Dilated cardiomyopathy in childhood: intravital differentiation of endocardial fibroelastosis using transvascular endomyocardial biopsy]. / Dilatative Kardiomyopathie im Kindesalter: Intravitale Differenzierung von der Endokardfibroelastose mittels transvaskulärer Endomyokardbiopsie.

Possibly due to the lack of clinical reports concerned with dilated cardiomyopathy in childhood, pediatric cardiologists may inadvertently designate the angiographic finding of a dilated left ventricle as endocardial fibroelastosis. We report historical and noninvasively-obtained data from twelve children, aged seven months to 17 years, with poorly-functioning dilated left ventricles, in whom no differentiation between the two diseases was enabled. Only by means of transvascular endomyocardial biopsies, performed during cardiac catheterization with a 6-F guide-catheter and a Machida bioptome for light and electron microscopal examination, was differentiation achieved among six patients with endocardial fibroelastosis and six with the nonspecific histological findings of muscular hypertrophy, interstitial fibrosis and marked degenerative changes. After exclusion of noxious and inflammatory etiologies, even in childhood, the diagnosis of primary dilated cardiomyopathy must be regarded as established. Since experience previously reported with endocardial fibroelastosis has not been based on histologic documentation of the diagnosis, questions with respect to natural history and prognosis remain unanswered.

Mechanics of the isolated ventricular myocardium of rats conditioned by physical training.

Force-velocity relations from after-loaded contractions, from isometric and isotonic QR experiments, resting-tension curves and biochemical analyses were conducted on sixteen trabecular muscles (SH) from hearts of rats conditioned by eight weeks of swimming training (increase in heart weight 8%), and compared to a control (CH) of eighteen trabecular muscles. (SH) showed increased tension development (p less than 0.01), whereas the diastolic properties remained almost unchanged. Analysis of the amount of hydroxyproline did not prove any variation. Vmax of (SH) was only slightly increase when there was a singificnat rise in actomyosin and myosin ATPase activity, while PO of the force-velocity relations of (SH) on the x axis (tension) shifted clearly to the right (p less than 0.01). Consequently, the maximum instantaneous power of (SH), expressed by the maximum rectangular plane under the force-velocity curve, increased considerably (p less than 0.01) in comparison with (CH). The experiments show that haemodynamic load induced by training does not alter the passive properties of the myocardium, but does bring about an increase in the contractile capabiltiy.