Coarctation of the aorta: pre and postoperative evaluation with MRI and MR angiography; correlation with echocardiography and surgery.

AIMS: To compare MRI and MRA with Doppler-echocardiography (DE) in native and postoperative aortic coarctation, define the best MR protocol for its evaluation, compare MR with surgical findings in native coarctation. MATERIALS AND METHODS: 136 MR studies were performed in 121 patients divided in two groups: Group I, 55 preoperative; group II, 81 postoperative. In group I, all had DE and surgery was performed in 35 cases. In group II, DE was available for comparison in 71 cases. MR study comprised: spin-echo, cine, velocity-encoded cine (VEC) sequences and 3D contrast-enhanced MRA. RESULTS: In group I, diagnosis of coarctation was made by DE in 33 cases and suspicion of coarctation and/or aortic arch hypoplasia in 18 cases. Aortic arch was not well demonstrated in 3 cases and DE missed one case. There was a close correlation between VEC MRI and Doppler gradient estimates across the coarctation, between MRI aortic arch diameters and surgery but a poor correlation in isthmic measurements. In group II, DE detected a normal isthmic region in 31 out of 35 cases. Postoperative anomalies (recoarctation, aortic arch hypoplasia, kinking, pseudoaneurysm) were not demonstrated with DE in 50% of cases. CONCLUSIONS: MRI is superior to DE for pre and post-treatment evaluation of aortic coarctation. An optimal MR protocol is proposed. Internal measurement of the narrowing does not correspond to the external aspect of the surgical narrowing.

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.

[Clinical course following partial cavo-pulmonary bypass: the Geneva experience from 1985 to 2000]. / Evolution après dérivation cavo-pulmonaire partielle: l'expérience genevoise de 1985 à 2000.

Partial cavo-pulmonary bypass (termino-lateral anastomosis between the superior vena cava and one of the pulmonary arteries) is an alternative to total cavo-pulmonary bypass because of a lower morbid-mortality, or a first step to this procedure. The authors report a retrospective study of 35 patients who underwent partial cavo-pulmonary bypass between November 1985 and October 1999 at the Geneva Children's Hospital with an average follow-up of 4 years. The mean age of the patients at the time of surgery was 6.4 years. Fourteen per cent of patients had tricuspid atresia, 37% had a single ventricle, 49% had an irreparable biventricular cardiopathy and 71% had already had a previous palliative procedure. There were 2 early and 6 late deaths. The 5 year survival was 71.2% and the 10 year survival: 62.3%. The commonest early complications were arrhythmias (11/35), chylothorax (9/35) and superior vena cava syndromes (9/35). The late complications observed were arrhythmias (4/35) and suboptimal function of the partial cavo-pulmonary bypass (7/35). Eighty eight per cent of patients were symornatically improved at one year (average NYHA Class 2.8 before and 1.7 after one year), and 40% of patients were asymptomatic at this time. The haematocrit decreased after partial cavo-pulmonary bypass but tended to rise again progressively to preoperative values after 9 years. Only 49% of survivors had not been reoperated 10 years after partial cavo-pulmonary bypass. Partial cavo-pulmonary bypass is therefore associated with a temporary improvement of symptoms and polycythaemia in children with complex cyanotic heart disease: later reoperation is often required.

[False aneurysm complicating ligation of patent ductus arteriosus operated on successfully]. / Faux anévrisme après ligature d'un canal artériel opéré avec succès.

Postoperative aneurysm of ductus arteriosus is a rare complication but may be lethal without treatment. It is less frequent than spontaneous aneurysm of ductus arteriosus. We report the case of 5 years-old girl who underwent a ligation of patent ductus arteriosus complicated, 6 months later, with a false aneurysm of ductus arteriosus and endocarditis of the ductus and the aortic valve. The diagnosis of the aneurysm was suspected on the anteroposterior chest X-ray which showed a left superior mediastinal opacity and confirmed by echocardiography. Through bilateral thoracotomy, an anevrismorraphy and aortic repair was carried out without problem. The postoperative course was unremarkable.

Effects of cisapride on ventricular depolarization-repolarization and arrhythmia markers in infants.

To study prospectively the effects of cisapride on ventricular repolarization, depolarization, and arrhythmia markers in neonates, we determined before and three days after starting cisapride (1 mg/kg/day): corrected QT interval (QTc) and QT dispersion (QTd) on standard ECGs, and duration of filtered QRS (fQRS) and of low amplitude (<40 microV) terminal signals (LAS40, ms) and root mean square of the last 40 ms (RMS40, microV) using high-gain signal-averaged ECG (SAECG). Twenty-four term and 11 preterm infants (gestational age 23-35 weeks) were studied at a median chronological age of 32 days. QTc and QTd were not different between term and preterm infants. Cisapride lengthened QTc (mean +/- SD; ms: 396.6 +/- 24.8 before vs. 417.0 +/- 35.2 after, p < 0.001). Three term and two preterm infants (5/35 = 14%; 95% CI: 5-30%) had a QTc >450 ms after cisapride. QTd after cisapride increased significantly in all infants with prolonged QTc. Filtered QRS, LAS40, and RMS40 before and after cisapride were within our normal values. We conclude that cisapride prolongs ventricular repolarization in neonates and infants without altering depolarization. Although no clinical arrhythmias were observed the dose of 0.8 mg/kg/day should not be exceeded.

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.

[Non-surgical approach in congenital cardiopathy]. / Approche non chirurgicale des cardiopathies congénitales.

The treatment of congenital or acquired heart defects by transcatheter therapy has crossed a period of rapid expansion in the last 10 years and is now an important adjunct to surgery. Balloon valvuloplasty can be considered as the treatment of choice for pulmonic and aortic congenital valvar stenosis as well as closure of a patent ductus arteriosus. The short hospital stay and absence of a scar make this approach very attractive. With the introduction of new techniques and devices, transcatheter therapy has become common for a number of other anomalies such as the atrial septal defect and soon some ventricular septal defects. It is necessary to maintain careful indications and compare the results with conventional surgery in order to assess the risks and benefits of these new techniques.

[Indications for pediatric cardiology evaluation]. / Indikationen zur kinderkardiologischen Abklärung.

Heart disease in infants and children may often lead to rapid decompensation. Early recognition by the primary physician is essential, since modern medicine and surgery can cure many heart defects. Main clinical signs are cyanosis, heart failure and/or abnormal auscultatory findings. Cyanosis appears shortly after birth or in small infants. In the newborn, cyanosis due to heart disease must be differentiated from pulmonary problems, sepsis or persistent pulmonary hypertension. Heart failure occurs more often in non cyanotic heart defects, mainly with left to right shunts. The main symptoms and signs of heart failure in the infant are briefly discussed. Abnormal auscultatory findings, usually murmurs, are the most common reason for referral to the paediatric cardiologist. However, most murmurs are "innocent" or "functional"; clues to their recognition are given. The present short review should help the primary physician to recognize signs of heart defects in infants and children so as to proceed with timely referral to the specialist.

Inhaled nitric oxide versus aerosolized iloprost in secondary pulmonary hypertension in children with congenital heart disease: vasodilator capacity and cellular mechanisms.

BACKGROUND: Inhaled nitric oxide (iNO) has been used to assess the vasodilator capacity of the pulmonary vascular bed in children with congenital heart disease and elevated pulmonary vascular resistance. Inhaled iloprost is a pulmonary vasodilator for the long-term treatment of pulmonary hypertension (PHT). Because these 2 vasodilators act through different pathways (release of cGMP or cAMP, respectively), we compared the pulmonary vasodilator capacity of each. METHODS AND RESULTS: A total of 15 children with congenital heart disease and PHT who had elevated pulmonary vascular resistance (preoperative, n=10; immediately postoperative, n=5) were first given 20 ppm of iNO for 10 minutes; then, after baseline values were reached again, they were given aerosolized iloprost at 25 ng. kg(-1). min(-1) for another 10 minutes. Finally, iNO and iloprost were given simultaneously for 10 minutes. With iNO, the pulmonary vascular resistance and systemic vascular resistance ratio decreased from 0.48+/-0.38 to 0.27+/-0.16 (P:<0.001). Similarly, iloprost decreased the ratio from 0.49+/-0.38 to 0.26+/-0.11 (P:<0.05). The combination had no additional effect on the resistance ratio. Plasma cGMP increased from 17.6+/-11.9 to 34.7+/-21.4 nmol/L during iNO (P:<0.01), and plasma cAMP increased from 55.7+/-22.9 to 65.1+/-21.2 nmol/L during iloprost inhalation (P:<0.05). CONCLUSIONS: In children with PHT and congenital heart disease, both iNO and aerosolized iloprost are equally effective in selectively lowering pulmonary vascular resistance through an increase in cGMP or cAMP, respectively. However, the combination of both vasodilators failed to prove more potent than either substance alone. Aerosolized iloprost might be an alternative to iNO for early testing of vascular reactivity and for the postoperative treatment of acute PHT.

Detection and quantification of valvular heart disease with dynamic cardiac MR imaging.

Magnetic resonance (MR) imaging is rapidly gaining acceptance as an accurate, reproducible, noninvasive method for optimal assessment of structural and functional parameters in patients with valvular heart disease. The severity of valvular regurgitation can be evaluated with cine gradient-echo MR imaging, which allows measurement of the area of the signal void corresponding to the abnormal flow jet. Alternatively, this modality can be used to obtain ventricular volumetric measurements and calculate the regurgitant fraction, or velocity-encoded cine (VEC) MR imaging can be used to quantify regurgitant blood flow. The severity of valvular stenosis can be determined by evaluating the flow jet and associated findings with either modality or by using VEC MR imaging to calculate the transvalvular pressure gradient and valve area. Dynamic MR imaging allows accurate assessment of ventricular function and comprehensive evaluation of pathophysiologic changes. In addition, good interstudy reproducibility suggests a role for VEC MR imaging in assessing the effects of therapeutic intervention and monitoring regurgitant fraction, thereby helping in surgical planning and the prevention of ventricular dysfunction. With greater cost-effectiveness and the increasing availability of new hardware and more advanced techniques, MR imaging will become a routine procedure in valvular heart disease.

[Follow-up of patients treated by conduits between the right ventricle and the pulmonary artery]. / Suivi des patients traités par conduits entre le ventricule droit et l'artère pulmonaire.

Between 1981 and 1998, 77 right ventricle-pulmonary artery conduits were implanted in 67 patients (37 boys, 30 girls, average age 6.3 years; range: 3 months to 17 years). The diagnoses were transposition of the great arteries with ventricular septal defect and obstruction of the pulmonary outflow tract (N = 22), tetralogy of Fallot (N = 16), truncus arteriosus (N = 9), double outlet right ventricle with pulmonary stenosis or atresia (N = 8) and agenesis of the pulmonary valve with pulmonary stenosis (N = 2). The implanted conduits were homografts in 50 cases (43 aortic and 7 pulmonary), 11 valved Dacron grafts, 4 valved polystans grafts and 2 non-valved conduits. The average follow-up period was 3.6 years (range: 1 month to 17 years). Early death was observed in 8 patients (12%) and late death in 6 patients (9%). The 5, 10 and 15 year survival rates were 78.4%, 65.3%, and 65.3%, respectively. The conduits had to be replaced in 10 patients (15%). The non-replacement rate of all conduits at 5, 10 and 15 years was 81.4%; 40.7% and 40.7%, respectively. The causes of replacement were pure stenosis (54.5%), pure regurgitation (9%) and mixed stenosis and regurgitation (27.2%). These results are comparable to other published series showing a 15 year survival rate of 65% and a 59% reoperation rate at 15 years. Homografts have a slightly longer life compared with valved Dacron conduits but the difference is not statistically significant.

[Nonsurgical occlusion of an atrial septal defect in childhood: initial Swiss experiences]. / Nichtchirurgischer Verschluss eines Vorhofseptumdefektes im Kindesalter: erste Schweizer Erfahrungen.

The initial experience with catheter closure of an atrial septal defect (ASD) in children, performed at two Swiss centers is presented. The ASD closures were performed according to international multicenter study protocols. 14 children, aged 3.9-17.5 years underwent closure by catheter. The defect size varied between 12 and 22 mm (balloon sized), the ratio between pulmonary and systemic blood flow showed a mean of 2.2 (1.5-3.5). Catheter closure was done using three different occlusion devices. More recently only the Amplatzer occluder was used at both institutions. In 12 children (86%) defect closure was successful and after a follow-up of 3-32 months (mean 17) only one child had a trivial residual interatrial shunt. In all children, echocardiographic follow-up showed an unchanged and correct device position on both sides of the atrial septum. In two children, a floppy aortic segment of the atrial septal rim led to instable device position: both children underwent surgical defect closure later. The children with successful device closure showed no complications during the catheterization or during follow-up. There were no thrombotic complications on the surfaces of the devices. Catheter closure of an ASD during childhood is a safe and efficient alternative to standard surgical treatment.

Interventional catheterization in surgically treated patients with congenital heart disease.

Interventional catheterization is an alternative to surgery for some congenital heart defects. For other malformations, the surgeon and the interventionist will join in an effort to obtain an optimal result: the typical example is pulmonary atresia with VSD and aortopulmonary collaterals. In other cases, the cardiologist may be called upon to intervene with catheter techniques to correct sequelae or residual lesions after surgical correction, avoiding redo surgery. Most often, the task consists of opening stenoses by balloon dilatation and/or stenting the main targets being pulmonary artery branch stenoses, venous obstructions after Mustard procedure, and recoarctations. Whereas simple balloon dilatation of recoarctation often brings good results, stents are often needed to obtain optimal results in pulmonary branch stenoses. Stenting of pulmonary veins has been disappointing. Closing unwanted vessels and defects is another task for the interventional cardiologist after cardiac surgery. Here, the most frequent procedure is closing aortopulmonary collaterals in pulmonary atresia and VSD after corrective surgery. Advantages and limitations of these procedures are discussed.

Normal values of signal-averaged electrocardiographic parameters and QT dispersion in infants and children.

Ventricular late potentials, and dispersion of the QT interval, are markers for risk of ventricular arrhythmias. Normal values for these parameters are well established in adults, but may not apply for children. This study has investigated the age dependency of signal averaged electrocardiographic parameters and QT dispersion in 111 normal children aged from 5 days to 16 years. The results indicate that parameters change with age: duration of filtered QRS and low amplitude (< 40 microV) terminal signal increase with age, especially in the younger patients. Filtered QRS is 88.9 +/- 7.87 ms in infants, and increases to 108.7 +/- 8.51 in teenagers (p<0.001). Low amplitude terminal signals are 17.0 +/- 3.44 ms in infants, and 24.5 +/- 5.64 ms in teenagers (p<0.001). Root mean square of the last 40 ms decreases with age, but remains stable after the age of 10 years (122.4 +/- 33.30 microV in infants, 60.9 +/- 31.27 in teenagers, p<0.001). QT dispersion, on the other hand, does not change significantly with age. The mean value for the whole group is 36 +/- 13.7 ms. A weak but significant correlation exists between QT dispersion and filtered QRS. Thus, age must be taken into consideration when interpreting signal-averaged electrocardiograms, but not when measuring QT dispersion.

Morphologic and functional evaluation of congenital heart disease by magnetic resonance imaging.

Magnetic resonance imaging (MRI) has evolved sufficiently to be recognized as a useful complementary noninvasive method to echocardiography in the evaluation of congenital heart disease (CHD). In some cases, MRI is superior to other imaging modalities, particularly in the evaluation of thoracic aortic anomalies and in defining the anatomy of central pulmonary arteries; it is also the procedure of choice in the postoperative follow-up of patients with CHD. Recent technological advances permit not only morphological evaluation (provided by spin-echo and MR angiographic techniques) but functional and flow information (provided by fast cine-GE and velocity-encoded sequences), causing it to be recognized by pediatric cardiologists and cardiac surgeons as an unavoidable technique for pre- and postoperative evaluation of some CHD. This review describes the various techniques used in the evaluation of CHD with emphasis on recent developments as well as recognized clinical applications. J. Magn. Reson. Imaging 1999;10:639-655.

[The pediatrician managing a child with heart disease]. / Le pédiatre face à l'enfant cardiopathe.

The pediatrician can and must play a pivotal role in the care of children with heart disease. He must provide the preventive care and health supervision as for all children, provide advice to the patient and the family in regards to everyday life, monitor for potential complications or deterioration of the cardiac lesion, and finally play a central role for patients with multiple problems and a chronic illness. The specialist must actively support the generalist with his experience and provide advice for each particular patient. This collaboration should allow the best care to be provided for the patient and maintain the patient and family in an ideal context.

Electrophysiological follow-up of tetralogy of fallot.

Arrhythmias and sudden death are well-recognized complications that occur late after correction of tetralogy of Fallot. This study, based on the literature and personal work, reviews the numerous investigations prompted by these complications and provides recommendations regarding follow-up. Conduction disturbances and ventricular arrhythmias are both common; although mostly asymptomatic, they are the likely cause of syncopy and sudden death. Supraventricular arrhythmias are less common but cause symptoms more frequently. Detecting patients at risk for life-threatening arrhythmias is an important task. The simple surface electrocardiogram (ECG) gives a host of information regarding patients at risk. Holter recordings, signal-averaged ECG, and invasive electrophysiological studies may all be helpful; the indications to perform these investigations are discussed and propositions are made for the follow-up of the postoperative patient. There is hope that a systematic approach along these lines, together with surgery done at an earlier age, will decrease the incidence of severe arrhythmias and sudden death in the future.

Aortic valve repair by cusp extension with the use of fresh autologous pericardium in children with rheumatic aortic insufficiency.

OBJECTIVES: Our goal was to evaluate the midterm results of aortic valve repair by a more sophisticated tailoring of cusp extension-taking into account the dimensions of the native aortic cusps-with the use of fresh autologous pericardium. PATIENTS AND METHODS: Forty-one children who had severe rheumatic aortic insufficiency (mean age 11.5 +/- 2.7 years) underwent aortic valve repair by means of this cusp extension technique over a 5-year period. Twenty-four of them underwent concomitant mitral valve repair for associated rheumatic mitral valve disease. All children were then followed up by transthoracic echocardiography before discharge, at 3 and 6 months after the operation, and at yearly intervals thereafter. RESULTS: Follow-up was complete in all patients and ranged from 3 months to 5 years (median 3 years). No operative and no early postoperative deaths occurred. Only 1 patient died, 9 months after the operation, of septicemia and multiple organ failure. Actuarial survival was 97% at 1 year and has remained unchanged at 3 years. On discharge, the degree of aortic insufficiency was grade 0 for 27 children and grade I for 14. Exacerbation of aortic insufficiency from grade I to grade II was observed in only 1 patient, and none of the children required reoperation for aortic insufficiency during the follow-up period. Mean peak systolic aortic valve gradients at discharge were lower than preoperative values (P =.04), and no significant increase in the peak systolic transvalvular gradient was detected thereafter during the follow-up period. Mean left ventricular dimensions were significantly reduced at discharge when compared with preoperative values (P <.0001). CONCLUSIONS: Functional results of aortic valve repair with cusp extension using fresh pericardium have been satisfactory at medium term, particularly in children with a small aortic anulus at the time of initial repair, because the expansion potential of fresh autologous pericardium is equivalent to that of the growing sinotubular junction and aortic anulus diameters.

Fungal endocarditis in critically ill children.

UNLABELLED: All cases of infective endocarditis occurring from January 1990 to December 1996 at our institution were reviewed, with a special focus on fungal endocarditis. Five critically ill children with fungal endocarditis and eleven children with bacterial endocarditis were recorded. The proportion of fungal endocarditis in our series was 5/16 (31%) and Candida albicans (4/5) was the most common fungal pathogen. Only one patient required heart surgery because of a loose patch but all the others were treated only by medical management for cure. The hospital survival rate was 80% (4/5) and the overall long-term survival rate was 60% (3/5) with only one death directly related to fungal infection. CONCLUSION: Despite the small number of cases, a sole medical approach including amphotericin B and long-term fluconazole prophylaxis for the treatment of fungal endocarditis in critically ill children seems to offer an alternative to surgical treatment which may be kept for failure of medical treatment.

Massive thromboembolism due to transcatheter ASD closure with ASDOS device.

Transcatheter occlusion of atrial septal defects (ASD) is currently being investigated as an attractive alternative to surgical correction. Thromboembolic events are rare in both techniques. However, we report a case of massive systemic embolization and residual left atrial thrombus after secundum ASD transcatheter closure by the ASDOS device (Atrial Septal Defect Occlusion System, Dr. Ing Osypka Corporation, Germany). The patient was successfully treated by femoral embolectomies, surgical removal of the device and closure of the ASD without a patch. No thrombophilia was found on subsequent exploration. Transcatheter ASD closure with the ASDOS device may therefore expose the patient to severe embolic complications. Further evaluation is needed before this technique can be safely recommended.