Chronic congestive heart failure in infancy and childhood: new aspects of diagnosis and treatment.

Congestive heart failure (CHF) is the inability of the heart to meet the metabolic demands of the body. As a disease of the advanced age with a frequency of 1-2% of the population it is rare in infancy and childhood. The incidence ranges from 2.95 (in all US children's hospitals) to 23.2 (University's Childrens Hospital Essen) on 1 000 discharges. Among the diagnostic procedures echocardiography is the primary modality of imaging. Tei-index and tissue-Doppler are more sensitive parameters for LV-dysfunction. BNP/NT-proBNP are age-dependent and can guide the long-term therapy. Treatment in childhood does not differ basically from that in adulthood, recommended by several guidelines, but data regarding the various substances - outlined in detail - are very limited. Here a big work has to be done in future!

[Adults with congenital heart disease: treatment and medical problems]. / Erwachsene mit angeborenen Herzfehlern : Versorgungssituation und medizinische Probleme.

By improvement of the medical care, children with congenital heart disease survive in much greater numbers: the figure of ACHD-patients in Germany is estimated up to 203,000 to 301,000. The need for a specialized care of these patients is accepted by the scientific societies, who introduced a certification for doctors and centers and created guidelines. The medical problems include imaging, treatment of arrhythmia, congestive heart failure and cyanosis, prophylaxis of infectious endocarditis, exercise and pregnancy. For the most frequent defects, indication for treatment and postoperative care is summarized.

[Ventricular septal defect and aortic regurgitation-pathophysiological aspects and therapeutic consequences]. / Ventrikelseptumdefekt und Aorteninsuffizienz. Pathophysiologische Aspekte und therapeutische Konsequenzen.

The development of aortic regurgitation (AI) is a rare but serious complication of subaortic ventricular septal defects (VSD). Over a period of 5 years we observed VSD-related AI in 24 patients, a frequency of 4.5% of all isolated VSD's encountered during that time frame. The location of the defects was in the infundibular septum in 59%, it was perimembranous in 25% and in the trabecular septum in 16%. Hemodynamically the defects were small except for 2 where the Q(p)/Q(s) ratio was > 2. Of the 24 pts, 16 had surgical closure of their VSD accompanied in 9 by aortic valvuloplasty. AI was caused by elongation or defect of the right coronary leaflet in 42%, of the noncoronary leaflet in 25% and a combination of both, in 8%. In 6 pts with infundibular VSD absence of part of the aortic valve ring above the defect was the underlying mechanism for AI. Postoperatively AI was improved to moderate in one pt and to none to trivial in 15. LV end-diastolic diameter decreased significantly in all pts operated. Pathogenetic mechanisms for the development of AI are a deficiency in the aorto-infundibular junction with prolaps of the right-or non-coronary leaflet, deficiency of the valve supporting structures including the valve ring as well as suction of the already elongated leaflet into the VSD with further damage to the antiregurgitant mechanism of the semilunar valve at risk. In perimembranous VSD's, late AI is probably related to turbulent flow through the adjacent LVOT. Surgical closure of isolated VSD's with a location immediately beneath the aortic valve is indicated regardless of their size to prevent the development of AI. If AI has occurred, VSD closure including aortic valvuloplasty improves the amount of regurgitation and normalizes LV enddiastolic dimension.

Balloon dilation of native aortic coarctation in infancy.

Balloon dilation of aortic coarctation in neonates and infants remains controversial. Between 2/1985 and 8/1999, 80 patients <12 months of age, with native aortic coarctation underwent balloon dilation. The systolic pressure gradient across the stenosed area was reduced significantly acutely from a mean of 45.6+/-19.4 mmHg to 17.9+/-13.8 mmHg. In 55 (68.8%) patients, the procedure was initially successful with a residual gradient of <20 mmHg. In 12.5% of patients, intimal tears were detected after dilation. In 21.3% of patients, obstruction of the femoral artery occurred, which responded to heparin or streptokinase in all. Two patients developed aneuryms immediately after the first intervention. In 1 case, surgery was performed with a successful aneurysmectomy. Severe complications or death in relation to the procedure were not detected. Long-term follow-up was obtained in 66 of 80 (82.5%) patients in a period between 6 to 174 months (median 29 months). In 22/66 (33%) of the infants, within a mean period of 10.9+/-15.2 months after first intervention, a redilation was necessary. Sixteen of 22 were successful procedures. In the remaining six patients, surgery was performed electively. A total of 30/66 who had follow-up (45%) patients remained free from reintervention or surgery after the first procedure. Further analysis of the data according to age showed that neonates and infants < or =3 months of age had a 90% and 62% higher residual stenosis rate, respectively, than infants >3 months. In this young age group, balloon dilation can only be recommended as palliation in young infants with severe left venticular dysfunction or in the case surgery is prohibitive for other reasons.

Use of low-dose ketamine and/or midazolam for pediatric cardiac catheterization.

Ketamine and midazolam are commonly used in children undergoing cardiac catheterization. However, there is controversy regarding the safety of administering these agents in the absence of an anesthesiologist. We retrospectively reviewed pediatric cardiac catheterization procedures at our institution between 1996 and 1997. A total of 154 patients (0.3-192 months) underwent a total of 205 procedures. They received ketamine (n = 79, 1.05 +/- 0.88 mg/kg/hr), midazolam (n = 35, 0.14 +/- 0.09 mg/kg/hr), or both (n = 91; ketamine, 1.13 +/- 0.84 mg/kg/hr; midazolam, 1.57 +/- 1.03 mg/kg//hr). In 18.5% of patients there were complex cardiac lesions. Mean procedure time was 79 +/- 36.2 minutes. Pre- and postprocedure systolic and diastolic mean blood procedure 72 +/- 14 and 68 +/- 12 mmHg, respectively. Pre- and postprocedure O2 saturation was 93.19 +/- 8.72 and 93.63 +/- 8.3, respectively. One patient required intubation, and 15% required oxygen therapy. The mortality rate was zero. The anesthesiologist's assistance was requested by the cardiologist in 21 procedures (group A) and not requested in 184 procedures (group B). The two groups were not different in relation to the drug used (p = 0.283) or the complexity of the cardiac lesions (p = 0.051). However, there was significant difference between the two regarding the need for supporting drugs (3/21 vs 3/184, p = 0.02) or oxygen treatment (7/21 vs 26/184, p = 0.014). No patients in group B required intubation, whereas 14% and 1.6% required oxygen therapy and supporting drugs, respectively. We conclude that low-dose ketamine and midazolam can be administered safely to most pediatric patients by the cardiologist, who can safely predict the need for an anesthesiologist.

Clinical features of isolated noncompaction of the myocardium in children.

Isolated noncompaction of the myocardium, also known as "spongy myocardium", is a rare disease in children and adults. It is suggested that, during early development of the heart, the primary spongy structure persists due to an arrest of compaction. No other cardiac malformations are found, but there are familial occurences, relations to genetic disorders or syndromes such as Melnick-needles-syndrome or Xq28-linked cardiomyopathy, and reports of conduction disorders. We have now diagnosed isolated noncompaction in seven children aged between five weeks and 5.5 years. Three are doing well with anticongestive therapy, while transplantation of the heart was performed in one. Three of the children have died, but in only one case due to cardiac failure. Our experience emphasises the need rapidly to establish the diagnosis, to search for associated extracardiac abnormalities, and to consider transplantation at an early stage.

[Dilated cardiomyopathy in childhood]. / Dilatative Kardiomyopathie im Kindesalter.

In childhood, dilative cardiomyopathy (DCM) has a prevalence of 2.6 patients in 100,000 inhabitants. Manifestation age is, in 75%, the first two years of life. There are no specific symptoms. Diagnosis is often made, when congestive heart failure occurs. Despite intensive therapy with digitalis, diuretics and ACE inhibitors, DCM is the main indication for heart transplantation. The prognosis is critical: 11 studies with approximately 450 children showed a mean 1-year survival rate of 75% and a 5-year survival rate of 60%. In most cases etiology of DCM remains unclear ("idiopathic"). In the second position (approximately 40%) is inflammatory pathogenesis. While familiar DCM is manifested mainly during the 3rd and 4th decade, specific cardiomyopathy is often found during infancy: inborn errors of metabolism, neuromuscular diseases or malformation syndromes are the causes of a cardiomyopathy, which can appear as dilative or hypertrophic cardiomyopathy. A causal therapy exists only in a few cases.

[Myocarditis in childhood: results of a decade's research]. / Myokarditis im Kindesalter--Forschungsergebnisse einer Dekade.

The research of the last decade gave new insights in the pathogenesis of viral myocarditis. The virus infection of the myocardium may be controlled by the infiltrating inflammatory cells and heal with more or less scarring. An autoimmune mediated chronic inflammation is observed as well as the persistence of virus genoms, which causes with low replication rate again chronic inflammation. The immunohistochemical characterization of the infiltrating immune cells and the molecular biological proof of virus genoms in endomyocardial specimens allow a sensitive diagnostic of the various forms of myocarditis and should guide therapy. Until now treatment trials did not differentiate between these various forms, had no control groups or very small numbers of patients. Two multicenter treatment trials in Germany referring to immunosuppressive or interferon therapy in children and adults may give--provided a better participation--recommendations for therapy.

Effective doses to patients from paediatric cardiac catheterization.

The Council Directive of the European Communities 97/43/Euratom requires dose assessment, especially for X-ray examinations of children and if high doses to the patient are involved. Both these aspects apply in cardiac catheterization and angiocardiography of children. Effective doses are a good indicator of radiation risk, particularly for leukaemia. Effective doses have been determined for 2114 infants and children undergoing cardiac catheterization from 1984 to 1996 at the University Hospital in Essen. Conversion factors (effective dose/dose-area product) were calculated based on direct dose-area product measurements for posteroanterior (PA) and lateral (Lat) projections as well as on patient records and examination details. The factors are calculated for eight age groups of children, taking into account the X-ray tube voltage for fluoroscopy and cine-film sequences, with and without zoom mode. Frequency distributions are presented for 2114 patients, for dose-area product, number of angiographic examinations (each combined with one cine-film sequence both PA and Lat) and for calculated effective doses. Highest effective doses are found in newborns (18.0 mSv and 6.5 mSv 90th and 50th percentiles, respectively) compared with adolescents of 15-21 years (8.0 mSv and 3.0 mSv 90th and 50th percentiles, respectively). Effective dose for cardiac catheterization is highest for newborns, in spite of lowest measured dose-area products, because the decreased value of the conversion factors overcompensates for the increase of dose-area product with age. This is especially important because of the higher tumour risk for equal effective dose for young children compared with adults.

Safety and efficacy of interventional occlusion of patent ductus arteriosus with detachable coils: a multicentre experience.

UNLABELLED: In order to define the safety and efficacy of Cook detachable coils for interventional closure of patent ductus arteriosus, we performed a retrospective analysis of all patients who underwent cardiac catheterization in seven centres for intended interventional occlusion of patent arterial duct. From January 1995 until March 1998, cardiac catheterization for intended interventional occlusion of patent arterial duct was performed in 317 consecutive children. Successful placement of at least one coil was achieved in 282 children (89%). The mean diameter of the ductus in children treated with Cook detachable coils was 1.65 mm, mean fluoroscopy time was 10.7 min. Occlusion rates were 62% 10 min after the procedure, 82% at the time of discharge, 91% at 4 months and 95% at late follow-up (2 years). In children with a ductus diameter of < or = 2.5 mm the rate of successful coil deployment was 94% with a 98% occlusion rate at late follow-up. Complications occurred in 11 procedures (3.5%) including haemolysis (3 patients), embolization of a coil to the pulmonary artery (7 patients, 2.3%) and inability to release a coil (1 patient). CONCLUSION: In our opinion, Cook detachable coils are safe and effective especially in the treatment of persistent ductus arteriosus with a diameter < or = 2.5 mm. Due to the low costs these coils appear to be superior to other devices in this subgroup of patients.

Closure of the zone of apposition at correction of complete atrioventricular septal defect improves outcome.

OBJECTIVE: Outcome after correction of atrioventricular septal defect depends to a great deal on the postoperative function of the left atrioventricular valve. The related role of the zone of apposition ('cleft') has been debated: should it be closed (bileaflet repair) or should it be left untouched (trileaflet repair)? This study aims to answer the question by comparing the outcome of patients treated according to these two approaches. METHODS: We reviewed all our patients who underwent repair of complete atrioventricular septal defect from 1984 to 1997 and selected those in whom the closure of the zone of apposition in principle would have been possible. Two groups with similar characteristics were constituted: group I (n=63), where the zone of apposition was deliberately not closed as part of a trileaflet repair (postoperative open zone of apposition) and group II (n=96), where it was electively closed as part of a bileaflet AV valve repair (closed zone of apposition). Since we changed from a trileaflet to a bileaflet repair in 1987, the two groups differ in terms of size and length of follow-up. Outcome was compared with regard to survival and freedom from reoperation for left atrioventricular valve incompetence. Late atrioventricular valve function was evaluated by Echo-Doppler. For statistical analysis, we used Chi-square or Fisher's exact test, the Mann-Whitney test and the log-rank test for comparison of Kaplan-Meier curves. The difference was considered statistically significant with a P-value of 0.05 or less. RESULTS: Early mortality was 9.5% (6/63) in group I and 3.1% (3/96) in group II (P=0.16). Actuarial survival after 1, 4 and 8 years was 80.4, 68.4 and 64.8%, respectively, for group I. Actuarial survival for group II was 94.7, 92.1 and 92.1% (P=0.0002). Freedom from reoperation for left atrioventricular valve regurgitation was 90.2, 85.6 and 77.8% for group I at the same time interval. It was a constant 97.9% for group II (P=0.0016). At reoperation, left atrioventricular valve regurgitation was present through the open zone of apposition in 63% of group I cases. The follow-up is 96% (126/131) complete. An increase in degree of left atrioventricular valve incompetence was noted in 28% (11/39) of group I cases and in 9% (8/87) of group II cases (P=0.0131). CONCLUSION: This study demonstrates the advantage of closing the zone of apposition ('cleft') as part of repair of complete atrioventricular septal defect. Survival, freedom from reoperation for left atrioventricular valve incompetence and over-all outcome were more favourable in patients of group II. The zone of apposition should be surgically addressed whenever the morphology of the left atrioventricular valve allows for closure without producing stenosis.

Double-chambered right ventricle in 73 patients: spectrum of the disease and surgical results of transatrial repair.

OBJECTIVE: To review the spectrum of double-chambered right ventricle (DCRV) and the outcome of surgical repair in patients diagnosed between February 1988 and March 1999. DESIGN: The charts of patients with DCRV were studied. SETTING: Tertiary care hospital. PATIENTS AND METHODS: A total of 73 patients were identified. Sixty-nine underwent surgical repair, while four are awaiting surgery. The repair was through a transatrial approach in 61 patients, while in eight an additional ventriculotomy was performed. MAIN RESULTS: An associated ventricular septal defect (VSD) was present in 56 of 73 patients (77%). These patients were significantly younger (P<0.05) than the 17 patients without a VSD. Among patients with a VSD, the 31 requiring patch closure were significantly younger than the 25 patients having direct closure. Five older patients among those with intact septum had impaired right ventricular (RV) function as well as higher intraventricular gradients. At surgery the intraventricular obstruction was relieved by myomectomy. There was no hospital or late mortality. Following surgery, at a mean follow-up of 13.6 months, no increase in the intraventricular gradient was detected by Doppler echocardiography. CONCLUSIONS: The development of DCRV is associated with VSD in early life. The probability of the presence of a VSD decreases with age. The disease is progressive, resulting in increased intracavitary gradient within the RV and in RV impairment if it is not treated in a timely fashion. Transatrial repair is safe with excellent midterm results. In the presence of high gradients within the RV, a ventriculotomy may be necessary to obtain acceptable results.

[Pregnancy in mothers with congenital heart defects. An overview]. / Schwangerschaft bei Müttern mit angeborenen Herzfehlern. Eine Ubersicht.

The improved pediatric-cardiological diagnostics and cardio-surgical therapy result in a numerical increase of women reaching reproductive age. Pregnancy considerably strains heart and circulation, which is countered by various cardiorespiratory mechanisms. Today mothers are not endangered vitally (exception: Eisenmenger-syndrome), but congestive heart failure, thromboembolic complications and rhythm disturbances may occur. Spontaneous abortion rate is 20-25%, prematurity and underweight are obligatory in cyanotic mothers. On the basis of an extensive review of literature the specific risks during pregnancy are discussed taking into account the hemodynamic situations in different heart defects. Anticoagulation of valve prosthesis depends upon the model and location of the prosthesis. Contraceptive counselling is deficient; it has to consider the risk of thromboembolic complications. Regarding the genetic risk we have to differentiate between each single defect and between the sex of the parents.

[Interventions in congenital heart disease and their sequelae in adults]. / Neue Therapiemöglichkeiten in der interventionellen Kardiologie. Bedeutung für angeborene Herzfehler im Erwachsenenalter.

The advancements of cardiac surgery over the last decades led to larger numbers of patients with operated congenital heart diseases surviving into adulthood. In Germany it is estimated that over 120,000 adults have operated congenital heart diseases. Five to 7% of them will need yearly hospital admissions. Interventional procedures are additional tools used to treat these patients with various sequelae or residua (Table 1). In the following review we concentrate on 2 different interventional procedures: dilatation and stent implantations for treatment of stenosis and the different devices used for the closure of shunt lesions. For congenital valvular pulmonary stenosis, balloon dilatation is the therapy of choice regardless the age of the patient. Stent implantation for the treatment of peripheral pulmonary stenosis (e.g., after previous systemic pulmonary shunts) can decrease the need for redo surgery, which is accompanied with increased risk. Stent implantations proved also to be useful to treat stenoses after Mustard patch in patients with transposition of the great arteries, after Fontan procedures or dealing with the rare pulmonary venous stenosis. In contrast, dilatation of bioprosthesis and conduit stenosis are not promising. Balloon dilatation of valvular aortic stenosis is an accepted therapy in childhood up to adolescents. Table 2 compares a surgical series including many infants with critical aortic stenosis with a series of balloon dilatation in children and another one in adults regarding lethality, complications, and results. Table 3 illustrates the immediate and late results of balloon dilatation of aortic coarctation in 3 different studies. The high recurrence rate in infants made clinicians refrain from taking this age group for balloon dilatation. In children and adult patients, good results are reported (75% reduction of gradients). The complication rate is low (2.3 to 3.3%) and aneurysm formation rate seldom (1 to 7%). Stenosed aorto-pulmonary collaterals will rarely need balloon dilatation. Surgical closure of atrial septal defect is a low risk procedure with a very low rate of residual shunts (2%). Of the 5 available devices for transcatheter closure of atrial septal defect Type II, only 2 occluders are in use in Germany, the Clamshell and the Amplatzer device. The largest clinical studies of the different systems, their efficacy, complications and residual shunt rate are presented in Table 4. For the deployment of these occluders a TEE is always needed. There are many more systems in clinical use to close the patent arterial duct (PDA) (Table 5). The Ivalon plug as well as the Rashkind device have probably only historical value. Different types of coils (Gianturco, Cook detachable, PFM) are now in use worldwide. The reason for their widespread use, besides their easy application, is the fact that most coils are relatively cheap and need only small sheaths for deployment. Their further evaluation identified a residual shunt rate of 5% as well as a number of complications (embolization, hemolysis, stenosis of the left pulmonary artery) in 0 to 6%. For the large PDA the Amplatzer device has recently been introduced. An additional indication for the use of the different occluding devices are aorto-pulmonary collaterals, venovenous fistulae, pulmonary or coronary artery fistulae. Aorto-pulmonary collaterals are often associated with complex cardiac lesions and occasionally appear after palliative procedures. An excellent cooperation between adult and pediatric cardiologists is needed in order to offer the group of adults with congenital heart diseases an adequate and comprehensive management.

[Isolated abnormality ("noncompaction") of the myocardium in 3 children]. / Isolierte Fehlentwicklung ("Noncompaction") des Myokards bei drei Kindern.

UNLABELLED: In three asymptomatic children an isolated myocardial noncompaction was detected by echocardiography at age 11 months, 5 weeks and 5.5 years. In the first male infant both ventricles and septum were severely affected and myocardial function was depressed. Nevertheless, during a follow up of 16 months he remained asymptomatic on anticongestive therapy. In the other two children apex and lateral wall of the left ventricle were affected and myocardial function was still normal. The second boy had also an infantile epilepsy-encephalopathy syndrome and the third child (a girl) had a Wolff-Parkinson-White syndrome; an association of either syndromes with myocardial noncompaction has not been reported earlier. DISCUSSION: Myocardial noncompaction (spongy myocardium) is a rare maldevelopment, which occurs either associated with certain congenital heart defects or, even more rarely, isolated, as the two cases reported here. Myocardial failure, severe arrhythmias or thromboembolism may occur at any age and determine the outcome. Clinical course, therapy and prognosis are similar to dilatative cardiomyopathy, which represents an important differential diagnosis.

Outcome of pregnancy in women with congenital heart disease.

Improvements in diagnosis and surgical technique for correction have led to an increasing number of women with congenital heart disease reaching the child-bearing age. Pregnancy places considerable strain on the heart and circulation and necessitates marked cardiorespiratory adaptation. Today, with the exception of the Eisenmenger syndrome, there is no increased mortality associated with pregnancy in congenital heart disease. In contrast, there is still considerable morbidity, due to congestive heart failure, thromboembolic complications and disturbances of rhythm. Fetal outcome is complicated by a high rate of spontaneous abortions (20-25%), retardation of fetal growth, and premature delivery (almost 100% in cyanotic mothers). Based on an extensive review of the literature, we discuss the specific risks in pregnancy depending on the hemodynamic situations produced by different heart defects. We also discuss the risks and advantages of different regimens for anticoagulation. Counselling concerning contraception is frequently inadequate. The most important problems are thromboembolic complications with the use of hormonal contraception, and hyper- and dysmenorrhea in those using intrauterine devices. Finally, the genetic risks must be considered, differentiating between single gene defects and the sex of the parents suffering from congenital heart diseases.

[Congestive heart failure in infancy and childhood]. / Herzinsuffizienz im Kindesalter.

Congestive heart failure is the inability of the heart to provide a sufficient output at a sufficient arterial pressure for the metabolic needs of the organism. Having presented the physiologic mechanisms of the contraction of the heart and its regulatory mechanisms the different aspects of classification of heart failure and its neurohormonal modulation are described. The epidemiology of heart failure in childhood is largely unknown, the causes vary considerably, symptoms are age-dependent. Main diagnostic tools for etiologic clarification are echocardiography and chest-x-ray. Focus of therapy is to eliminate the causes, but general recommendations may not be forgotten. Medical treatment of acute severe heart failure is use of catecholamines, ino- and vasodilators in the intensive care unit. Therapy of chronic heart failure is performed with diuretics, digitalis and ACE-inhibitors. Their pharmacologic effect, therapeutic trials in adults and children and peculiarities of pharmacology and side effects in childhood are reported. Low dose beta-blockers are a new therapeutic strategy still not established in children.