Balloon aortic valvoplasty in paediatric patients: progressive aortic regurgitation is common.

OBJECTIVE: To evaluate immediate and midterm results after balloon valvoplasty in a paediatric population with congenital aortic stenosis, giving special consideration to aortic regurgitation. DESIGN: Retrospective study. SETTING: Two tertiary referral centres for paediatric cardiology. PATIENTS: 70 consecutive patients, with an age range of 0-16.4 years. Group A infants < 3 months old (n = 21). Group B children > 3 months old (n = 49). Median follow up time was 19.8 months, range 0-158 months. INTERVENTION: All patients underwent balloon aortic valvoplasty. The balloon to annulus ratio was selected at a mean of 0.90 (range 0.67-1.0). MAIN OUTCOME MEASURES: Doppler gradients and degree of aortic regurgitation. RESULTS: The pressure gradient dropped significantly with the intervention and increased mildly at follow up. Freedom from relevant aortic regurgitation (that is, moderate and severe) was initially lower in group A (75% v 90% after one month) but after two years the difference between the two groups was not significant (50% v 61%). Freedom from reintervention was significantly lower in group A (with 35% v 80%) after three years. CONCLUSION: Aortic balloon valvoplasty is safe and effective but has a high rate of early reintervention in infants with critical aortic stenosis. The major long term problem is progressive aortic regurgitation, which does not seem to be prevented by the use of small balloons.

Transcatheter occlusion of the patent ductus arteriosus with a single device technique: comparison between the Cook detachable coil and the Rashkind umbrella device.

UNLABELLED: Transcatheter coil occlusion of the patent ductus arteriosus (PDA) has become the interventional treatment option of choice. Immediate occlusion of any residual shunting results in excellent closure rates, but frequently requires multiple coil deployment. AIMS: To assess the efficacy and limitations of single Cook detachable coil PDA closure compared to a preceding series of Rashkind umbrella procedures. METHODS AND RESULTS: Between 1990 and 1999, transcatheter occlusion of a small (<2 mm; n=45) or moderate-sized (2-4 mm; n=47) PDA was successfully attempted in 90/92 consecutive patients (mean age 6+/-4.8 years) with a coil (39/41) or Rashkind device (51/51). Immediate angiographic closure rates for both devices were low, although better for small (54-68%) than moderate ducts (7-22%, P<0.01). A 2-year echocardiographic closure rate of small ducts increased to 92% for the coil group versus 95% for the Rashkind group. By that time, moderate-sized ducts were only occluded in 64% with the coil and 54% with the Rashkind device. A visible residual shunt at post-implant angiography in moderate ducts was associated with a high incidence (59%) of long-term echocardiographic shunt patency and a need for repeat interventions for audible residual shunts (32%). CONCLUSIONS: Single coil transcatheter occlusion is the treatment of choice for the small duct as most residual shunts will resolve spontaneously. However, long-term shunt persistence after single coil deployment in moderate sized ducts is as frequent as with the Rashkind device. A primary multiple coil approach is advocated if the postcoil aortogram shows residual ductal shunting and if there is persistence of a ductal murmur on auscultation.

[Double aortic arch: clinical aspects, diagnosis and therapy in children and adults]. / Doppelter Aortenbogen. Klinik, Diagnose und Therapie bei Kindern und Erwachsenen.

Double aortic arch is a rare vascular anomaly which usually causes tracheal and esophageal compression in the first few months of life. During the last 30 years, 7 children, 2 to 24 months old, and one 29-year-old woman with double aortic arches have been treatedatour institution. Symptoms, diagnosis and treatment of these patients were evaluated. Dyspnoe, stridor, recurrent pulmonary infections, feeding problems and failure to thrive were the leading symptoms. Despite typical symptoms from early childhood, the diagnosis was missed in our adult patient. Typical compression of the esophageus and the trachea was visualized by esophagography by 7 and bronchoscopy/-graphy by 6 patients. Angiography was performed in all children, whereas magnetic resonance angiography and computed tomography were done in the adult patient. Resection of the smaller aortic arch, left in 3 and right in 5, through a right or a left posterolateral thoracotomy was uncomplicated and fully resolved the symptoms in all patients. Typical symptoms in early childhood should lead to prompt diagnosis and surgical treatment of double aortic arch. Surgical resection of the smaller aortic arch should also be performed in oligosymptomatic patients to prevent complications later. Preoperative angiography can be replaced by the less invasive magnetic resonance imaging and computed tomography.

Can dopamine prevent the renal side effects of indomethacin? A prospective randomized clinical study.

BACKGROUND: Indomethacin therapy for closure of a patent ductus arteriosus in preterm neonates is responsible for transient renal insufficiency. Dopamine theoretically reduces the renal side effects of indomethacin therapy. PATIENTS: 33 neonates with a mean gestational age of 28.5 weeks who received indomethacin for treatment of a symptomatic PDA were included in a prospective randomized controlled clinical study. METHOD: 15 patients were treated with indomethacin alone (control group), 18 patients with indomethacin and dopamine (study group). Indomethacin was given in a dose of 0.2 mg/kg/dose intravenously, all patients received three doses with intervall of 12 hours. The dose of dopamine was in all patients 4 micrograms/kg per minute commencing 2 hours prior to the first dose of indomethacin and continuing for 12 hours after the third dose. RESULTS: Indomethacin induced a significant increase in serum creatinin (76.3 mumol/l vs 99.7 mumol/l for the control group, and 70.7 mumol/l vs 93.0 mumol/l for the study group), and weight (1259 g vs 1316 g for the control group, and 1187 g vs 1221 g for the study group). The increase systolic blood pressure (61 mmHg vs 65.7 mmHg) in the study group was significant (p < 0.05) but remained unchanged in the control group. The changes between the study group and the control group were not significant either in serum creatinin, fractional excretion of sodium, or weight gain. The failure rate of ductal closure was not different between the two groups. CONCLUSION: The additional use of dopamine does not reduce the renal side effects of indomethacin.

[Indications criteria for total cavopulmonary anastomosis (Fontan operation)]. / Indikationskriterien für eine totale kavopulmonale Anastomose (Fontan-Operation).

OBJECTIVES: In a retrospective study we evaluated the predictive value of the traditional risk factors (Fontan criteria) for mortality and morbidity after total cavopulmonary anastomosis (Fontan operation). DESIGN: We studied 51 consecutive patients who underwent Fontan operations from 1982 through 1994. Morbidity was assessed by a score taking equally into account rhythm disturbances, cardiovascular hospital admissions and heart failure. RESULTS: The mean age at operation was 4.1 +/- 3.4 years. Diagnoses were tricuspid atresia in 20, complex single ventricle in 13 and various complex congenital heart defects in 18 patients. All Fontan criteria were fulfilled in 11 patients while in the remaining 40 up to 6 criteria were not met. The criteria least fulfilled were age in 31 patients and pulmonary artery anatomy in 11. The mean follow-up was 4.6 +/- 3.0 years. The overall 30-day survival was 84% and 1- and 10-year survival 76.5% respectively. All patients who fulfilled all the Fontan criteria survived. The overall mortality and early mortality were associated with a significantly higher number of unfulfilled Fontan criteria (p < 0.01). Age below 2 years at operation and pulmonary resistance > 4 Woods units were risk factors for overall mortality (p < 0.01). The number of unmet Fontan criteria was not significantly different in patients with and without postoperative late morbidity. CONCLUSIONS: The Fontan criteria are reliable predictors for mortality but have little predictive value for morbidity. Surviving patients who do not fulfil all criteria suggest that some criteria may need to be modified. Our limited data support a lower age limit of 2 instead of 4 years.

Application of successive principles of repair to correct supravalvular aortic stenosis.

The diffuse form of supravalvular aortic stenosis may extend in the takeoff of the coronary and arch arteries. Relief of the supravalvular stenosis requires a harmonious enlargement of the sinotubular junction to maintain aortic valve competence. The technique we used in a 9-year-old boy involved patch enlargement of all affected structures. Deep hypothermic circulatory arrest and retrograde cerebral perfusion was used during repair of the aortic arch and arch arteries.

Extended follow-up after atrial repair for transposition of the great arteries: a younger age at surgery improves late survival.

BACKGROUND: Surgical treatment for transposition of the great arteries (TGA) usually involves anatomic repair, although atrial repair is used in cases with special coronary artery patterns. METHODS: Records of all 239 patients surviving at least 30 days after atrial correction of TGA between 1962 and 1987 at the University Hospital in Zurich were reviewed. The mean length of follow-up time was 13.7 years (median 14.9 years; range from 0.05 to 30.1 years). The average age at surgery was 45.7 months (range from 7 days to 24.4 years). One hundred twenty-one patients had a simple TGA, whereas the remaining 118 had a complex TGA. RESULTS: The surviving patients were in NYHA class I at time of follow-up. The most common reasons for death were systemic right ventricular dysfunction and sudden rhythm disturbances. There was a major risk for late cardiac event in patients over 3 years old at operation (p = 0.02) and also in patients with complex TGA (p = 0.03). However, date of surgery, previous surgery, or the postoperative requirement for a pacemaker did not greatly affect late cardiac mortality. CONCLUSIONS: Although the procedure of choice for TGA is the arterial switch operation, the promising findings of the current long-term study, which reports the longest follow-up to date, indicate that the atrial switch operation is a good alternative procedure for the rare cases where the use of arterial switch procedures is limited.

Myocarditis and coronary dilatation in the 1st week of life: neonatal incomplete Kawasaki disease?

UNLABELLED: Acute heart failure in the early neonatal period is rare. Normally it is due to asphyxia, severe septicaemia, a congenital heart malformation or a viral myocarditis. Kawasaki disease (KD) as a cause of an neonatal myocarditis is not an established diagnosis. KD is a vasculitis of still unknown origin occurring predominantly in infants and preschool children. KD before the age of 3 months is rare. There are only few reports about KD in the 1st month. We present a newborn who showed the cardiac symptoms of KD in the 1st week of life with coronary dilatation and myocarditis. CONCLUSION: The diagnosis of incomplete KD should be considered not only in infants but also in newborns with signs of myocarditis and coronary abnormalities. Therapy with gammaglobulins may prevent the sequelae of coronary involvement.

22q11.2 deletions in a series of patients with non-selective congenital heart defects: incidence, type of defects and parental origin.

Previous studies have indicated a wide spectrum of incidences of 22q11.2 deletions in isolated and syndromic (sporadic or familial) cases of conotruncal heart defects, whereby the detection rate of the deletion varied from 65% in one study to 0 in another. We analysed 110 patients with non-selective syndromic or isolated non-familial congenital heart malformations by fluorescence in situ hybridization (FISH) using the D22S75 DiGeorge chromosome (DGS) region probe. A 22q11.2 microdeletion has been detected in 9/51 (17.6%) syndromic patients. Five were of maternal origin and four of paternal origin. None of the 59 patients with isolated congenital cardiac defect had a 22q11.2 deletion. We compared the cardiac anomalies of our patients with a 22q11.2 deletion with those of previously published series and we describe types of congenital heart defects which appear to be often associated with a 22q11.2 deletion. The ability to detect such types of heart defects and to provide an early diagnosis of 22q11.2 deletion is particularly relevant in very young infants, who often show only very mild expression of the otherwise well-characterized phenotypes of the DiGeorge/velo-cardio-facial syndrome (DG/VCFS).

The effect of the N-methyl-D-aspartate receptor antagonist dextromethorphan on perioperative brain injury in children undergoing cardiac surgery with cardiopulmonary bypass: results of a pilot study.

Experimental evidence indicates a role of the N-methyl-D-aspartate receptor in the pathogenesis of brain injury occurring during cardiac surgery with cardiopulmonary bypass (CPB). Dextromethorphan is a noncompetitive antagonist of this receptor with a favorable safety profile. Thirteen children age 3-36 months undergoing cardiac surgery with expected CPB of 60 minutes or more were randomly assigned to treatment with dextromethorphan (36-38 mg/kg/day) or placebo administered by naso-gastric tube. Dextromethorphan was absorbed well and reached putative therapeutic levels in blood and cerebrospinal fluid. Adverse effects were not observed. Mild hemiparesis developed after operation in one child of each group, and severe encephalopathy in one of the placebo group. Sharp waves were recorded in postoperative continuous electroencephalography in all placebo (n = 7) but only in 2/6 dextromethorphan treated children (p = 0.02). Pre- and postoperative cranial magnetic resonance imaging (MRI) revealed less pronounced ventricular enlargement in the dextromethorphan group (not significant). An increase of periventricular white matter lesions was visible in two placebo-treated children only. No elevations of cerebrospinal fluid enzymes were observed in either group. Although children with dextromethorphan showed less abnormalities in electroencephalography and MRI, dissimilarities of the treatment groups by chance diminished conclusions to possible protective effects of dextromethorphan at this time.

Clinical implications of phrenic nerve injury after pediatric cardiac surgery.

Phrenic nerve injury with resulting diaphragm paralysis occurred in 25 (1.5%) of 1,656 cardiac surgical procedures in children during a 10-year period. Phrenic nerve injury was most commonly noted in patients who had undergone previous cardiac surgery (16 of 165, 10%; P < .0001), typically after a previous Blalock-Taussig shunt (10 of 53, 19%; P = .007). Plication of the diaphragm (7 thoracic, 4 abdominal) was performed in 11 patients (44%). Indications for plication were inability to wean from mechanical ventilation (5 patients) and persistent or recurrent respiratory distress (6 patients). The patients who needed diaphragm plication were significantly younger than those who were managed conservatively (median, 11 months [4 days to 23 months] versus 20 months [4 months to 16 years]; P = .01). All patients older than 2 years were extubated within 3 days (mean, 1.5 days) and did not need any surgical intervention. The median follow-up period was 3.2 years, and no patient has had recurrent respiratory problems. There were no deaths as a direct result of phrenic nerve injury. Phrenic nerve injury after cardiac surgery is a serious complication that often leads to respiratory insufficiency in patients under than 2 years of age. For such patients, early diaphragm plication is a simple and effective procedure that prevents the complications of prolonged mechanical ventilation.

Congenital coronary fistulas in children and adults: diagnosis, surgical technique and results.

Coronary artery fistula is a very rare congenital malformation with an abnormal coronary-cameral communication that may involve any chamber and any or all coronary artery branches. We present our experience with 11 consecutive patients (mean age 16.6 years, ranging from 4 to 64 years); 9 of them were treated surgically, spontaneous closure of the fistula was observed in 1 patient and 1 patient is still under observation. Nine patients were under 17 years of age at the time of operation whereas only 2 patients were older (56 and 64 years). Fistulas originated from the right coronary artery in 4 patients and drained either into the right (n = 3) or into the left system (n = 1). In 8 patients, the origin of the coronary artery from the aorta was normal and the fistulous communications developed with the right cardiac structures only. In 2 patients, both coronary arteries were involved in the pathological drainage and 2 patients were demonstrated to have multiple drainage from the left coronary artery. Additional congenital cardiac malformations were found in 2 patients: severe tricuspid valve regurgitation in 1 and ventricular septal defect in another patient. Surgical closure of the fistula was successful in all operated patients (in 1 case treatment was possible without cardiopulmonary bypass). Simple ligation of the fistula was performed in 1 patient, intracardiac closure of the fistula was combined with different reconstructive procedures in the other patients. No hospital mortality nor severe complications occurred in this small group of patients. The mean follow-up interval was 39.4 months and all patients were in NYHA functional class I, except 1 with moderate tricuspid and mitral valve regurgitation. In the presence of symptoms of congestive heart failure, significant left-to-right shunt and arrhythmias, elective closure of coronary fistula is generally accepted, whereas the indication is more controversial in asymptomatic patients. Considering the low perioperative morbidity, we recommend surgical closure of coronary fistulas with significant shunt and/or increased coronary artery diameter.

Neonatal lupus erythematosus with congenital heart block and severe heart failure due to myocarditis and endocardititis of the mitral valve.

We report a case of neonatal lupus erythematosus (NLE) with congenital heart block and severe myocardial failure, which was followed from the 25th week of gestation because of fetal bradycardia. The child was delivered at the 37th week of gestation by elective cesarean section because of echocardiographically documented heart enlargement, pericardial effusion and moderate insufficiency of the mitral and tricuspid valves. In spite of immediate pacing, intubation and supportive treatment, the newborn developed progressive heart failure. Echocardiography showed endocarditis of the mitral valve and diffuse myocarditis. The heart failure resolved under steroid treatment. Our experience supports the early use of steroids in treating myocarditis due to NLE. Intrauterine steroid treatment in the presence of fetal hydrops and congenital heart block is discussed.

Congenital coronary fistulas in children and adults: diagnosis, surgical technique and results.

Coronary artery fistula is a very rare congenital malformation with abnormal coronary-cameral communication that may involve any chamber and any or all coronary artery branches. We present our experience with 11 consecutive patients [mean age 16.6 years, ranging from 4 to 64 years]; 9 of them were treated surgically, spontaneous closure of the fistula was observed in one patient and one patient is still under observation. Fistulas originated from the right coronary artery in 4 patients and drained either into the right [n=3] or into the left system [n=1]. In 9 patients, the fistulas originated from the left coronary system and the fistulous communication developed with the right cardiac structures only. In 2 patients both coronary arteries were involved in the pathological drainage and 2 patients were demonstrated to have multiple drainage from the left coronary artery. Additional congenital cardiac malformations were found in 2 patients. Surgical closure of the fistula was successful in all operated patients [in one case treatment was possible without cardiopulmonary bypass]. Simple ligation of the fistula was performed in 1 patient, intracardiac closure of the fistula was combined with different reconstructive procedure in the other patients. Neither hospital mortality nor severe complications occurred. Mean follow-up interval was 39.4 months and all patients except one were in NYHA I. In presence of symptoms of congestive heart failure, significant left-to-right shunt and arrhythmias, elective closure of coronary fistula is generally accepted, whereas indication is more controversial in asymptomatic patients.

[32 years of Senning's correction for transposition of the great vessels]. / 32 Jahre Vorhofsumkehr nach Senning.

Between 1962 and 1994 342 patients with transposition of the great arteries (TGA) were treated by atrial correction. Since 1992 the atrial switch operation is the treatment of choice for TGA. We reviewed our 32 year experience. Average age of the patients at operation was 69 months (7 days--8.5 years). 177/342 (52%) patients had a complex TGA: 74 patients with ventricular septal defect (VSD), 49 patients with pulmonary stenosis (PS) and 54 with both (VSD and PS). The 30 day mortality was for the whole series 15.7%. In the last 4 years 7.5%. The actuarial survival rate for all patients was 88% after 10 years and 82% after 20 years. For simple TGA 91% after 10 years and 83% after 20 years, for complex TGA 84% and 81%. The most important cause of death during our longterm observation were heart failure (19 patients) and sudden death (7 patients). Average follow-up for the whole group was 13.4 years. Most of the survivors are functionally symptom free (66% NYHA I) or they have slight symptoms (29% NYHA II). Only 5% were NYHA III or IV. Arterial switch operation has replaced the atrial correction for TGA. Nevertheless the longterm results after atrial correction remains encouraging. The main threat to the patients is the failure of the systemic ventricle.

Prevention of residual ventricular septal defects with fibrin sealant.

BACKGROUND: Modern echocardiography now allows for the detection of a substantial number of residual ventricular septal defects (VSDs) after surgical patch repair that remained hidden in the past. Mostly without hemodynamic significance, residual VSDs may have clinical consequences (progressive dehiscence, hemolysis, prophylactic antibiotic treatment, endocarditis). To reduce the number and size of residual VSDs we performed an experimental and a clinical study. METHODS: (1) In an experimental setup, burst pressure of 60 fibrin glue-sealed defects (calibrated between 1.0 and 5.0 mm in diameter) was determined using a computerized recording system and pressure loads up to 500 mm Hg. (2) In a prospective clinical trial with blinded postoperative echocardiographic controls VSD closure was performed in 36 consecutive patients (age, 37 +/- 40 months; range, 4 to 134 months) using a polytetrafluoroethylene patch and running sutures reinforced with pledgets (22 of 36 patients) or sealed with fibrin glue (14 of 36 patients) in accordance to the surgeon's preference. RESULTS: (1) Experimentally, mean pressure load achieved was more than 500 +/- 0 mm Hg for 1.0-mm defects, 413 +/- 52 mm Hg for 2.5-mm defects, 363 +/- 58 mm Hg for 4.0-mm defects, and 313 +/- 48 mm Hg for 5.0-mm defects (r 0.873, p < 0.001). (2) Clinically, all patients survived. Residual VSDs at echocardiography were observed in 16 of 22 patients (72%) for reinforced versus 5 of 14 patients (36%) for sealed with fibrin glue (p < 0.05). Diameter of residual VSDs accounted for 1.3 +/- 1.2 mm for reinforced versus 0.3 +/- 0.4 mm for sealed with fibrin glue (p < 0.01). Hemodynamically significant residual VSDs were fond in 2 of 22 patients (9%) for reinforced versus 0 of 14 patients (0%) for sealed with fibrin glue (p = not significant). CONCLUSIONS: Small defects sealed with fibrin glue resist physiologic pressure load. Fibrin glue sealing of prosthetic patches during intracardiac VSD repair allows for significant reduction of number and size of residual VSDs. Improved long-term outcome can be expected.

Anomalous origin of the right coronary artery from the pulmonary artery.

Isolated anomalous origin of the right coronary artery (RCA) is a very rare congenital malformation. The case of a 15-month-old boy with anomalous origin of the RCA from the pulmonary artery is presented. The RCA was reimplanted into the aorta. The anatomical correction was uneventful and its success demonstrates that diagnosis and surgical correction are possible at a very young age.

Early and late results after correction for double-outlet right ventricle: uni- and multivariate analysis of risk factors.

Between April 68 and May 91, 59 patients underwent total correction for double-outlet right ventricle. The mean age was 59.2 +/- 7.8 months. The hospital mortality rate was 27%; it was 41% before 1980 and 13% thereafter. Low cardiac output failure was the leading cause of early death. Myocardial protection without cardioplegia and long cardiopulmonary bypass time were significant predictors of hospital mortality in univariate as well as in multivariate analysis. The late death rate was 7%. The actuarial survival rate was 67% (CL 55-80%) after 10 and 20 years. The reoperation rate was 42%, a loose VSD patch constituted the main indication (23%). The actuarial freedom from reoperation rate was 51% (CL 33%-69%) after 10 years and 31% (12%-50%) after 20 years. The mean follow-up time was 8.4 +/- 0.9 years (2 to 20 years). Seventy-five percent of all postoperative survivors are in NYHA functional class I and left ventricular ejection fraction is normal in 82%.

[Development of surgical treatment of transposition of great vessels in the last 15 years. Methods and early results]. / Entwicklung der chirurgischen Behandlung bei Transposition der grossen Gefsse in den letzten 15 Jahren.Methoden und Frühzeitresultate.

Between 1978 and June 1992, 213 patients were operated for transposition of the great arteries at our institution. 148 patients underwent atrial repair, 41 patients an anatomic repair and 24 patients only palliation. Since 1986 the anatomic repair has progressively replaced the arterial repair: 1978-1986 95-100% atrial repair, 1992 100% anatomic repair. At operation the average age of the patients treated by atrial repair was 22 months, resp. 10 months from 1986 to 1991, for anatomic repair 9 months, resp. 46 days in 1991/1992. The 30-day mortality for atrial repair was 11% (17/148), for anatomic repair 22% (9/41), resp. 12% (3/24) in 1991/1992. Potentially superior long-term results for anatomic repair are possible, but cannot be demonstrated at present.