Right-sided cervical aortic arch with stenosis--treatment with an extra-anatomic bypass graft.

Right-sided cervical aortic arch is a very rare vascular anomaly that may lead to stenosis development. Anatomic repair may be impeded by its high course or by abnormal branching of the supraaortic vessels, or both. This report will describe the treatment of a stenotic right-sided cervical aortic arch using an extra-anatomic bypass graft without extracorporeal support in an 11-year-old girl.

Incidence and impact of systemic venous collateral development after Glenn and Fontan procedures.

BACKGROUND: Development of systemic venous collaterals after Glenn or Fontan procedures can lead to systemic desaturation and reduction in ventricular function, resulting in impaired everyday performance in patients with univentricular heart disease. METHODS: We analyzed 79 patients who had undergone a Glenn or Fontan procedure between 1995 and 1999 for the incidence and predilection sites of systemic venous collaterals as well as the therapeutic options. RESULTS: In 16/79 (= 20.2%) patients, 19 veno-venous connections were detected 310 days (1-966 days) postoperatively. Locations were: brachiocephalic angles/pericardial veins (7), azygos/hemiazygos system (5), Thebesian veins (2), epidiaphragmatic veins (5). Drainage was to the pulmonary veins in 5, to the "left" atrium in 9, and to the IVC system in 5 patients. An isolated intervention became necessary because of low saturations in 5/16 pts, with improvement in all of them (catheter embolization 4, surgical closure 1). CONCLUSIONS: After Glenn or Fontan operations, the increased central venous pressure may induce recanalization of embryologically preformed and obliterated vessels. Their predilection sites must be carefully evaluated pre- and postoperatively. During surgical procedures, potential venous channels should be ligated. Interventional or surgical closure of collaterals may become necessary.

Circulatory changes following intrauterine closure of the ductus arteriosus in the human fetus and newborn.

Prenatal detection of intrauterine closure of the ductus arteriosus unrelated to maternal administration of non-steroidal anti-inflammatory drugs or glucocorticoids made it possible to study the circulation in this condition in the human fetus and newborn by pre- and postnatal echocardiography and neonatal cardiac catheterization. At 38 weeks, the fetus presented intrauterine ductal closure associated with right ventricular dilatation and marked hypertrophy of the right ventricle and the interventricular septum, as well as severely diminished right ventricular fractional shortening and diminished pulmonary blood flow. Blood flow redistribution was characterized by reduced blood flow through the right heart and increased right-to-left shunting across the dilated foramen ovale. Pathological Doppler waveforms of the inferior vena cava and the ductus venosus were found, although the cardiotocogram was normal. Following unsuccessful induction of labour a Caesarean section was performed. Postnatal echocardiography confirmed the prenatal findings. Cardiac catheterization, performed because of persistent dependence on additional oxygen administration, revealed increased pulmonary vascular resistance, reduced pulmonary blood flow, and prolonged right-to-left shunt across the foramen ovale. Reduced peripheral pulmonary artery diameters were shown angiographically. Follow-up examinations revealed regression of right ventricular hypertrophy and recovery of right ventricular and pulmonary function. The findings confirm results from haemodynamic studies in animal experiments.

Ductus arteriosus-dependent pulmonary circulation secondary to cardiac malformations in fetal life.

The objective of this study was to describe the characteristic prenatal findings of a ductus arteriosus-dependent pulmonary circulation secondary to cardiac malformations. B-mode, color and pulsed wave Doppler echocardiography were performed in seven fetuses with severe pulmonary stenosis or atresia. All findings were confirmed postnatally by echocardiography and cardiac catheterization or autopsy. Severe fetal pulmonary stenosis or atresia was characterized by decreased pulmonary valve diameters, frequently with reduced pulmonary artery diameters, increased flow velocities or absent flow across the stenotic pulmonary valve, increased ascending aorta diameters, slightly increased aortic velocities and normal umbilical and middle cerebral artery Doppler wave forms. In all cases, prenatal assessment of neonatal ductus dependence was possible by demonstrating reverse flow across the fetal ductus with peak systolic velocities ranging from 0.9-2.0 m/s and absent diastolic flow. Ductal diameters were slightly decreased, ranging from 2-4 mm. Prenatal detection of a ductus-dependent pulmonary circulation is a strong indication of the presence of severe pulmonary stenosis or atresia. Its diagnosis allows avoidance of maternal administration of drugs with constrictive effects upon the ductus, interdisciplinary planning of perinatal management, early postnatal confirmation of the diagnosis, and early postnatal intervention, in particular administration of prostaglandins to prevent life-threatening ductal closure.

Prenatal diagnosis of idiopathic stenosis of the ductus arteriosus associated with fetal atrial flutter.

Antenatal stenosis or closure of the ductus arteriosus unrelated to either congenital heart defects or prostaglandin inhibitors is considered to be uncommon, but may result in congestive heart failure, hydrops fetalis, and perinatal death. We report a case of idiopathic ductal stenosis detected prenatally by two-dimensional and Doppler echocardiography in a fetus presenting atrial flutter and right atrial dilatation at 31 weeks of gestation. Prenatal treatment with digoxin and verapamil resulted in conversion to sinus rhythm. The fetus was closely monitored. Congestive heart failure did not develop, and the fetus was delivered spontaneously in good condition at 39 weeks of gestation. In case of ductal stenosis, the potential risk of congestive heart failure must be considered, and maternal administration of drugs with constrictive effects on the ductus arteriosus, as indomethacin or betamethasone, should be avoided.

[Vein of Galen malformation: prenatal diagnosis and noninvasive procedure]. / Vena-Galeni-Malformation: Pränatale Diagnostik und nichtinvasives Vorgehen.

In two fetuses a vein of Galen aneurysmal malformation was prenatally diagnosed causing cardiac failure and hydrocephalus associated with marked encephalomalacia. Blood flow in the vena prosencephalica measured by Doppler ultrasonography was extremely high (1290 and 1500 ml/min maximum). In a interdisciplinary council a strictly noninvasive pre- and postnatal procedure was proposed. After adequate information both parents agreed with this approach. The neonates died immediately after birth.

[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.

[Combination of scimitar syndrome and horseshoe lung. A rare but typical finding--case report and review of the literature]. / Kombination von Scimitar-Syndrom und Hufeisenlunge. Ein seltener aber typischer Befund--Fallbericht und Literaturübersicht.

The scimitar syndrome is a rare congenital cardiopulmonary malformation. In association with a horseshoe lung it is extremely uncommon. We describe a child with typical scimitar syndrome (anomalous right pulmonary venous return, hypoplasia of the right lung with dextro-position of the heart and aberrant systemic arterial supply of the right lung), associated with a horseshoe lung. No clinical symptoms are present, surgical management is not necessary. This is the 20th case in addition to the 19 reported cases with scimitar syndrome associated with horseshoe lung. Eight other additional cases of horseshoe lung without scimitar syndrome are mentioned here. In horseshoe lung the pulmonary parenchyma extends from the right lung base across the midline and fuses with the left lung. In only 3 cases continuous parenchymal tissue could be found histological. In 8 cases the lungs were separated by fissurelike structures, seen in CT or chest film, and in 5 cases fissures were demonstrated histological. From 6 patients no findings are available. The various symptoms, the diagnostic tools and the therapeutic procedure will be discussed.

[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)

Prenatal diagnosis of a left ventricular aneurysm.

In a fetus with ventricular extrasystoles a congenital aneurysm of the left ventricle was diagnosed prenatally. At 32 weeks of gestation, echocardiography showed a large apical left ventricular aneurysm with a thin, hypokinetic wall. Congestive heart failure did not occur. Prenatal and postnatal examinations did not detect the aetiology of the aneurysm, but excluded the majority of possible causes. The 2-year-old child is now asymptomatic and normally developed. Neither medication nor surgical treatment have been necessary, except for antithrombotic prophylaxis with low-dose aspirin.

[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.

Pulmonary hypertensive vasculopathy--no indication for corrective operation of isolated ventricular septum defect in babyhood.

The frequency of surgical closure of a ventricular septum defect (VSD) in the first year of life has risen from 10% to 30% in the last seven years in West Germany. Whereas there was a decrease of mortality in older children, mortality has stagnated at 8% for correction at age 12 months or less (early correction). Development of an irreversible pulmonary hypertensive vasculopathy (PHVP) and recurrent heart failure with growth disorders are given as reasons for operation in the first year of life. In the last six years, we have operated on 31 infants for isolated VSD with pulmonary hypertension (PH) and closed the VSD. In approximately equal preoperative pressure in the two ventricles with a pressure ratio of 0.88 +/- 0.16, there was an immediate fall in pressure in the right ventricle with pressure ratios of 0.43 +/- 0.24. Three patients (9.7%) died perioperatively. In the same period, a primary VSD closure was carried out in 120 children even after over 12 months with three deaths (2.5%). Pulmonary tissue removed in this operation did not show any PHVP in four patients (13%), grades 0 to I and I to II in 10 patients each (total 33%) and a PHVP grade II to III in six patients (20%). This PHVP is capable of full regression. An irreversible PHVP does not develop up to the end of the first year of life in isolated VSD with PH, so that the correction can be safely postponed to the beginning of the second year of life provided that recurrent heart failure with growth disorders does not compel earlier correction.(ABSTRACT TRUNCATED AT 250 WORDS)

[Pacemaker therapy in children]. / Schrittmacher-Therapie im Kindesalter.

Between 1975 and June 1987 55 children underwent first permanent pacemaker (PM) implantation. The patients' age at time of implantation ranged between 1 month and 17 1/2 years (mean 5 5/12 years). In all cases - except a 17 years old girl who received a transvenous endocardial lead - only epicardial screw-in electrodes were used. Only VVI-PM have been implanted. In 84% the indication for implantation was post-surgical brady-arrhythmia, in 16% it was an inborn or acquired disturbance of the conducting system. With our patients we have a survey of 2603 months of PM implantation. During this period 14 children underwent totally 20 revisions, in 50% the electrode was the cause of failure. The interval between two revisions calculated as a quotient from months of PM implantation and number of revisions has increased significantly during the last years and amounts now to 130 months. The relative low incidence of revisions is in our opinion mostly related to the nearly exclusive use of epicardial leads.

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.