Fenestration in extracardiac conduits in children after modified Fontan operation by implantation of stent grafts.

Three patients (ages 3, 5, and 8 years) with various forms of functionally univentricular heart lesions received a total cavopulmonary connection with an extracardiac conduit as a final reconstructive procedure. Failure of the Fontan circulation occurred immediately after surgery because of spontaneous closure of surgical fenestrations in two children and absent fenestration in one. As an emergency procedure, in all patients the conduit was perforated by transcatheter intervention in order to create a connection to the anatomical right atrium. Following balloon dilatation of the perforated conduit, in all three patients covered stent grafts were placed in the newly created defect to attain a reliable communication. Patency of the fenestration was demonstrated by angiogram and any leakage was ruled out. Cardiac output improved and severe pleural effusion and ascites subsided. Right-to-left shunt could be demonstrated by echocardiography at follow-up after 7 months (median) in all three patients. Oxygen saturation remained stable between 85 and 90%. These preliminary results suggest that stent graft fenestration can serve as a valuable tool in failing Fontan circulation, particularly in patients with an extracardiac conduit. Covered stents have the potential to reduce the acute risk of bleeding and they help to prevent early spontaneous closure of the newly created fenestration.

Stenting of the ductus arteriosus and banding of the pulmonary arteries: basis for various surgical strategies in newborns with multiple left heart obstructive lesions.

OBJECTIVE: To present an institutional experience with stent placement in the arterial duct combined with bilateral banding of the pulmonary artery branches as a basis for various surgical strategies in newborns with hypoplastic left heart obstructive lesions. DESIGN: Observational study. SETTING: Paediatric heart centre in a university hospital. PATIENTS: 20 newborns with various forms of left heart obstructive lesions and duct dependent systemic blood flow. INTERVENTIONS: Patients underwent percutaneous ductal stenting and surgical bilateral pulmonary artery banding. Atrial septotomy by balloon dilatation was performed as required, in one premature baby by the transhepatic approach. MAIN OUTCOME MEASURES: Survival; numbers of and reasons for palliative and corrective cardiac surgery. RESULTS: One patient died immediately after percutaneous ductal stenting. One patient died in connection with the surgical approach of bilateral pulmonary banding. Stent and ductal patency were achieved for up to 331 days. Two patients underwent heart transplantation and two patients died on the waiting list. Ten patients had a palliative one stage procedure with reconstruction of the aortic arch and bidirectional cavopulmonary connection at the age of 3.5-6 months. There was one death. One patient is still awaiting this approach. Two patients received biventricular repair. In one, biventricular repair will soon be provided. CONCLUSIONS: Stenting the arterial duct combined with bilateral pulmonary artery banding in newborns with hypoplastic left heart or multiple left heart obstructive lesions allows a broad variation of surgical strategies depending on morphological findings, postnatal clinical conditions, and potential ventricular growth.

Heart transplantation in children and infants: short-term outcome and long-term follow-up.

Since 1988, 82 heart transplants have been performed in 80 infants and children. Diagnoses pretransplant were: hypoplastic left heart syndrome (HLHS) (n = 43); cardiomyopathy (n = 19); endocardial fibroelastosis (n = 6); and other complex congenital heart diseases (n = 12). Age at transplantation was < 1 yr in 61 patients. Overall survival rate was 79% at 1 yr and 73% at 5 and 10 yr. To date, 20 patients have died after transplantation. Causes of death were: rejection (eight patients); right ventricular failure (four patients); transplant coronary artery disease (TCAD) (two patients); and other causes (six patients). In the majority of patients somatic growth is not impaired, and renal function is reduced (but stable) in all patients. Two patients developed post-transplant lymphoproliferative disease, which was treated successfully. Major long-term morbidity is neurologic deficit - severe in three patients and minor in six. TCAD was present or suspected in six surviving patients. We conclude that heart transplantation in infants and children can be performed with good early and late results. Quality of life is excellent in most patients. TCAD, however, will become an increasing problem in the long term.

[Fulminant diphtheria myocarditis in an unvaccinated preschool child]. / Maligne diphtherische Myokarditis bei einem ungeimpften Kleinkind.

We describe a case of a three year old unvaccinated child who developed a fulminant diphtheric myocarditis and nephritis four days after the onset of a tonsillar diphtheria. Despite of the administration of antitoxin on day three and four the child died within 48 hours from the beginning of rhythm disturbances. The cardiac involvement rapidly progressed from bradyarrhythmias with the necessity of a temporary pacemaker to ventricular rhythm disturbances with cardiac failure and final ventricular fibrillation.

Superior caval venous syndrome after atrial switch procedure: relief of complete venous obstruction by gradual angioplasty and placement of stents.

Superior caval venous syndrome is one of the late problems known to occur after Mustard repair of complete transposition. Reoperation may leave residual stenosis, and carries substantial risk for the patient. It is now feasible to use intravascular stents to overcome systemic venous baffle obstructions, and such an approach is probably more effective. The purpose of our study therefore, was to assess immediate and medium term results of inserting stents subsequent to gradual balloon enlargement of acquired atresia of the intraatrial baffle in patients who had undergone an atrial switch operation. We investigated five patients with complete obstruction of the superior caval venous pathway at perforation of the atretic segment was achieved using a guide wire technique. The procedure was successful in all patients. Gradual angioplasty was performed and intravascular stents were implanted. The pressure in the superior caval vein dropped to normal values, symptoms improved, and the patency of the newly created venoatrial communication was proven at mid-term follow-up. Thus critical obstructions at the superior caval venous pathway after the Mustard procedure can be reopened by interventional catheterization. Implantation of balloon-expandable intravascular stents is safe and effective in the acute relief of the obstructions, but careful long-term follow-up is mandatory.

Clinical experience with heart transplantation in infants.

OBJECTIVE: Orthotopic heart transplantation has become an accepted therapeutic concept for adult patients with endstage heart disease. In newborns and infants this procedure is still a matter of discussion because of unknown long-term results and the lack of donor organs. METHODS: Since March 1988 we have performed 40 orthotopic heart transplantation in 39 infants who were from 1 to 280 days of age. Indications for transplantation included hypoplastic left-heart syndrome (n = 28), dilative cardiomyopathy (n = 4), endocardial fibroelastosis (n = 4) and other complex structural anomalies (n = 3). The mean waiting period for transplantation was 53 days. A donor-recipient weight ratio up to 4.0 was accepted. Profound hypothermic circulatory arrest was used for graft implantation in all those patients who required extensive aortic arch reconstruction (71%). The initial immunomodulation was based on Cyclosporine, Azathioprine and Prednisolone. Patients who underwent transplantation during the first 6 weeks of life received a chronic single-drug therapy with Cyclosporine after 1 year. RESULTS: There were six peri-operative deaths caused by drug-resistant right-heart failure in three cases, humoral rejection (n = 1), CMV infection (n = 1) and multi organ failure (n = 1). One infant died late, due to rejection. The actuarial survival rate for the entire group is now 82%. There is a remarkable influence of increasing experience. Whereas six of 15 infants who had heart transplantation between 1988 and 1993 died early post-operatively (survival rate: 60%), only one late death occurred among 24 recipients in the period from 1994 to April 1997 (survival rate: 96%). Episodes of rejection occurred once or several times in about half of the patients in this series (48%). All surviving children are living at home in excellent condition. CONCLUSIONS: Heart transplantation during early infancy is a rational and durable therapy for heart diseases with irreversible myocardial failure or severe structural anomalies. The intermediate-term results have been encouraging in many centers, but more data must be accumulated to determine the sequelae of chronic immunosuppression. The lack of donor organs remains one of the major problems in pediatric heart transplantation.

[Heart transplantation in infancy: experiences at the Giessen Pediatric Heart Center]. / Herztransplantation im Säuglingsalter: Erfahrungen am Kinderherzzentrum Giessen.

From June 1988 to December 1996 heart transplantations were performed in 36 newborns and infants below one year of age. Diagnosis were hypoplastic left heart syndrome (n = 26), endocardial fibroelastosis (n = 4), cardiomyopathy (n = 3), and other complex congenital heart defects (n = 3). Mean waiting time for transplantation was 52 days, the mean donor-recipient bodyweight ratio was 1.8. Seven patients (19%) died after transplantation mainly within the first month after transplantation. The cumulative probability of survival is 79% in all patients. The influence of increasing experience is indicated when patients transplanted from 1988-1993 (n = 15) are compared with transplants from 1994-1996 (n = 21). The overall survival in the first group was 50%, whereas patients transplanted from 1994 showed a probability of survival of 92%. The 1-year survival rate in the later group was 100%. In 20 patients a total of 31 rejection episodes were observed. 2 infants died due to rejection. 71% of all rejections occurred during the first month after transplantation. Renal function was slightly impaired one year after transplantation in all patients without tendency for deterioration in the sequel. The somatic development is normal in nearly all infants and the quality of life is excellent. All infants live at home without any restrictions. Two patients, however, suffer from a neurologic deficit. Until now there is no evidence of coronary vascular disease or malignancy. Heart transplantation is in our opinion a reconsiderable alternative in the treatment of complex cardiac disease and cardiomyopathy in infants.

Perioperative management of pulmonary hypertension after heart transplantation in childhood.

BACKGROUND: Pulmonary hypertension is responsible for a substantial part of perioperative and postoperative mortality and morbidity after cardiac transplantation. Treatment of right ventricular failure after increased pulmonary vascular resistance is difficult especially in infants and children. Therefore we started a preventive therapy of pulmonary hypertension after cardiac transplantation to avoid right ventricular failure and compared the results with a group of patients with conventional therapy. METHODS: Group 1 (n = 13), with transplantation from 1988 to 1991, was treated with vasodilators when symptoms of right ventricular failure developed. Group 2 (n = 19) had preventive treatment with prostaglandin E1 (PGE1), the phosphodiesterase-III inhibitor enoximone, and alkalinazation starting during weaning from cardiopulmonary bypass. RESULTS: Six patients in group 1 died; four of them as the result of right ventricular failure in the immediate postoperative course despite aggressive treatment. In group 2 there were three deaths as the results of rejection (2) and infection (1). None of these patients developed right ventricular failure (p = 0.02). Cold ischemic time, extracorporeal circulation time, and waiting time before transplantation were significantly longer in group 2. Side effects of this preventive therapy were not observed. CONCLUSIONS: We conclude that prophylactic therapy of pulmonary hypertension with vasodilators in infants and children after heart transplantation is safe and effective in preventing right ventricular failure in the postoperative course.

Cardiac dysrhythmias in children with idiopathic dilated or hypertrophic cardiomyopathy.

To assess the incidence and prognostic significance of cardiac dysrhythmias in children with idiopathic dilated or hypertrophic cardiomyopathy, the clinical course of 59 patients was retrospectively reviewed over a period of 27 years. Dilated cardiomyopathy (DCM) was diagnosed in 28 patients and hypertrophic cardiomyopathy (HCM) in 31 patients. The mean age at the time of diagnosis was 2.8 +/- 0.7 years in DCM patients and 6.7 +/- 0.8 years in HCM patients. Mean follow-up time after diagnosis of cardiomyopathy was 4.1 +/- 1.0 years in DCM patients and 6.6 +/- 0.8 years in HCM patients. Clinically significant cardiac dysrhythmias were found in 17 of 59 patients (29%): 7 of 28 patients (25%) with DCM and 10 of 31 patients (32%) with HCM. The initial diagnosis of a cardiac dysrhythmia was made by standard electrocardiography in 12 of 17 patients (71%) and by 24-hour Holter monitoring in 5 of 17 patients (29%). Ventricular dysrhythmias were present in 5 of 7 patients with dilated cardiomyopathy and in 5 of 10 patients with hypertrophic cardiomyopathy. During the followup time, death occurred in 18 of 59 patients (31%): 8 of 59 patients (14%) died from congestive heart failure and 10 of 59 patients (17%) died suddenly. Among the sudden deaths were 4 of 28 patients (14%) with dilated cardiomyopathy and 6 of 31 patients (19%) with hypertrophic cardiomyopathy. Cardiac dysrhythmias had been documented in 6 of the 10 patients dying suddenly (3 of 4 patients with DCM and 3 of 6 patients with HCM).(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiac transplantation in a neonate with endocardial fibroelastosis.

An investigational orthotopic cardiac transplantation was performed to manage subendocardial fibroelastosis in a neonate. No unmanageable technical complications arose from the transplantation. Postoperative observation showed the infant developed normally except for moderate cerebral palsy.

Diverticulum of the right ventricle associated with ventricular septal defect.

A true diverticulum of the ventricle is a very rare congenital cardiac lesion which is characterized by a normal myocardial wall and active participation in the ventricular contractions. Diverticula of the right ventricle may originate from the apical or anterosuperior wall. We report three patients with an anterosuperior diverticulum of the right ventricle associated with a perimembranous ventricular septal defect, one of whom additionally had obstruction of the right ventricular outflow tract. These patients presented clinically with symptoms related only to their associated defects. The right ventricular diverticulum was diagnosed by angiography in all, but was also seen on cross-sectional echocardiography in one patient. The ventricular septal defect was repaired through an incision of the diverticulum, which was reduced in size at the same time. Complete resection appears necessary only if severe endocardial fibrosis is present within the diverticulum, because such a diverticulum does not contribute to right ventricular contraction, but rather is at risk of rupture.

[The determination of heart minute volumes and systolic time intervals using echo- and Doppler echocardiography in severely ill premature infants]. / Zur Bestimmung von Herzminutenvolumina und systolischen Zeitintervallen mittels Echo- und Dopplerechokardiographie bei schwerkranken Frühgeborenen.

The here represented echo- and doppler-echocardiographic measurements of critically ill preterm infants demonstrate the possibility to watch closely to hemodynamic changes due to new treatments of these patients, thus to gather more information than with the usual monitoring of a neonatal intensive care unit. Using systolic time intervals and cardiac output, measured in the ascending aorta and the pulmonary truncus by echo- and doppler echocardiography, gives more ease and accuracy in actual therapeutic decisions. This is demonstrated with the use of dopamine, prostaglandin E1 and indomethacin.

Myxoma of the aortic valve in a child.

Myxomas are predominantly located in the right on left atrium. We report an unusual case of a myxoma adherent to the aortic valve. The child presented with symptoms of subaortic stenosis. By invasive methods a tumor was diagnosed located at the aortic valve. After surgical removal the child recovered soon and is doing well. According to our knowledge this is the first report of a myxoma of the aortic valve.

Surgical therapy of pulmonary thrombosis due to candidiasis in a premature infant.

We report a case of surgical removal of a pulmonary thrombosis in a premature infant with candidemia. The pulmonary thrombotic material contained candida albicans. Following surgical therapy the child recovered soon and candidemia did not recur. We conclude that surgical treatment of pulmonary thrombosis can be successfully done even in premature infants.

[Cervical aortic arch--symptoms and diagnosis in 2 children]. / Zervikaler Aortenbogen--Symptome und Diagnostik bei zwei Kindern.

Two children with cervical aortic arch are presented. This anomaly is very rare. In one patient cervical aortic arch Type A (Haughton) was associated with a ventricular septal defect and patent ductus arteriosus. In the other child we report the only case of cervical aortic arch (Type B) in association with partial anomalous pulmonary venous return.

Severe digoxin intoxication in a child treated by infusion of digoxin-specific Fab-antibody-fragments.

Accidental digitalis ingestion in children is a rare, but potentially life-threatening emergency. We report the case of a 2 10/12-year-old boy with accidental ingestion of 6 mg beta-Acetyl-digoxin. Soon after admission, the boy developed sinus bradycardia, SA and AV-block, of increasing severity without circulatory impairment. As the serum digoxin level reached 21.7 ng/ml digoxin-specific Fab-antibody-fragments were used to bind free serum digoxin. Immediately after infusion, serum free digoxin was below the detection limit, whereas total digoxin peaked at 219 ng/ml 5 h thereafter. The arrhythmias did not subside totally, so that in addition, a transvenous pacemaker was placed, but never used. The antibody-infusion was well tolerated and the boy was discharged in good health.

[Sonographic image of intrarenal and intra-arterial fungus thrombi in a newborn infant with Candida infection]. / Sonographische Darstellung von intrarenalen und intraarteriellen Pilzthromben bei einem Frühgeborenen mit Candida-Sepsis.

Shortly after birth a preterm infant suffering from aspiration syndrome and subsequent Pseudomonas aeruginosa sepsis showed signs of renal insufficiency and mycotic infection: Yeast cells were identified in several urinalyses; there was also an increasing anti-Candida IgM antibody titer. At the same time sonographic examinations revealed an increasing echogenicity of the renal cortex and echogenic masses of variable size which did not cause acoustic shadows in both enlarged kidneys. A few days later, we found a right-sided hydronephrosis caused by an intraureteric prevesical mass of equal echogenicity. As we could observe sonographically, the aggressive antimycotic therapy was successful. Eleven weeks later there were signs of cardiac insufficiency. An angiographically demonstrated filling defect within the pulmonary artery showed the same sonographic findings as the previously found intrarenal masses. The baby underwent embolectomy and recovered. The thrombotic material contained yeast cells giving evidence of systemic Candidiasis. Provided appropriate equipment is available, ultrasound today is an excellent non-invasive screening and follow-up method not only for echoencephalography, but also for more complicated neonatal problems as seen here. The detailed observation of a changing echogenicity of the renal cortex and pelvis is important and often allows a decisive diagnostic clue before other radiological methods become conclusive.