Long term outcome up to 30 years after the Mustard or Senning operation: a nationwide multicentre study in Belgium.

OBJECTIVE: To assess long term outcome of patients who underwent Mustard or Senning repair for transposition of the great arteries up to 30 years earlier. DESIGN: Retrospective review of medical records. SETTING: The six university hospitals in Belgium with paediatric cardiology departments. PATIENTS: 339 patients were reviewed, of whom 124 underwent the Mustard procedure and 215 the Senning procedure. This represents almost the entire population of patients in Belgium with either simple or complex transposition. MAIN OUTCOME MEASURES: Mortality, morbidity, functional abilities, social integration. RESULTS: Overall mortality was 24.2%. Early mortality (< or = 30 days after surgery) accounted for 16.5%, late mortality for 7.7%. Actuarial survival of early survivors at 10, 20, and 30 years after surgery was 91.7%, 88.6%, and 79.3%, respectively. Patients in the Senning cohort had a slightly better survival rate than those in the Mustard cohort (NS). Baffle obstruction occurred more often after Mustard repair (15.3%) than after the Senning procedure (1.4%). Arrhythmia-free survival did not differ between the two cohorts, but was determined by the complexity of the transposition. Survivors of the Senning cohort had better functional status, and tended to engage in more sports activities. CONCLUSIONS: The long term outcome for patients surviving the Mustard or Senning operation was favourable in terms of late mortality, morbidity, functional, and social status. Overall mortality in the Senning cohort did not differ from the Mustard group, but Senning patients had better functional status, greater participation in sports activities, and fewer baffle related problems.

Tetralogy of Fallot: transannular and right ventricular patching equally affect late functional status.

BACKGROUND: In tetralogy of Fallot, transannular patching is suspected to be responsible for late right ventricular dilatation. METHODS AND RESULTS: In our institution, 191 patients survived a tetralogy of Fallot repair between 1964 and 1984. Transannular patching was used in 99 patients (52%), patch closure of a right ventriculotomy in 35, and direct closure of a right ventriculotomy in 55. Two had a transatrial-transpulmonary approach. To identify predictive factors of adverse long-term outcome related to right ventricular dilatation, the following events were investigated: cardiac death, reoperation for symptomatic right ventricular dilatation, and NYHA class II or III by Cox regression analysis. Mean follow-up reached 22+/-5 years. The 30-year survival was 86+/-5%. Right ventricular patching, whether transannular or not, was the most significant independent predictor of late adverse event (improvement chi(2)=16.6, P:<0.001). In patients who had direct closure, the ratio between end-diastolic right and left ventricular dimensions on echocardiography was smaller (0.61+/-0.017 versus 0. 75+/-0.23, P:=0.007), with a smaller proportion presenting severe pulmonary insufficiency (9% versus 40%, P:=0.005). There was no difference between right ventricular and transannular patching concerning late outcome (log rank P: value=0.6), right ventricular size (0.70+/-0.28 versus 0.76+/-0.26, P:=0.4), or incidence of severe pulmonary insufficiency (30% versus 43%, P:=0.3). CONCLUSIONS: In tetralogy of Fallot, transannular patching does not result in a worse late functional outcome than patching of an incision limited to the right ventricle. Both are responsible for a similar degree of long-term pulmonary insufficiency and right ventricular dilatation.

Late homograft valve insertion after transannular patch repair of tetralogy of Fallot.

BACKGROUND AND AIMS OF THE STUDY: Transannular patch repair of tetralogy of Fallot leads to pulmonary insufficiency and progressive right ventricular dilatation responsible for a decreased exercise capacity. We studied the impact of late homograft insertion on the regression of the right ventricular volumes in symptomatic patients. METHODS: Between July 1992 and August 1996, 15 consecutive patients (age range: 4 to 24 years) were operated on at a median of 13 years (range: 3 to 20 years) after transannular patch repair of tetralogy of Fallot. All patients complained of exertional dyspnea and fatigue. Syncopes were reported in six patients and four patients had sustained episodes of ventricular tachycardia. Fourteen had pulmonary regurgitation grade 3 or 4 and one had an associated stenosis and insufficiency. All patients had a dilated right ventricle. At reoperation, no patients presented with major aneurysm. The patch was resected and the right ventricular outflow tract reconstructed with a cryopreserved pulmonary homograft. Right ventricular volumes were studied before the procedures and at the last follow up consultation. RESULTS: There was no operative death. One patient who had a concomitant patch repair of a hypoplastic left pulmonary artery needed extracorporeal circulatory support for eight days. After a median follow up of 25 months (range: 3 to 54 months) all patients but one are in NYHA class I. There were no late deaths. The mean end-diastolic diameter of the right ventricle decreased from 36 +/- 9 mm before surgery to 31 +/- 6 mm (not significant). The mean ratio between the end-diastolic diameter of the right and left ventricles decreased from 0.94 +/- 0.3 to 0.74 +/- 0.2 (p < 0.01). CONCLUSION: An increasing number of patients who had transannular patch repair for tetralogy of Fallot will require reoperation for symptomatic long-term pulmonary regurgitation. Homograft reconstruction of the right ventricular outflow tract of these patients induces regression of their right ventricular dilatation and leads to their functional recovery.

Ross procedure in congenital patients: results and left ventricular function.

METHODS: From April 1990 to August 1995, 121 patients (median age 42 years) underwent aortic valve replacement with allografts (69 patients) or autografts (52 patients). In this latter group, 24 Ross procedures have been performed in congenital patients since November 1991 (median age 10 years, range five months to 27 years): aortic incompetence (n = 17), isolated aortic stenosis (n = 5), small stenotic prosthesis (n = 2). Transthoracic echocardiography was obtained preoperatively in all patients and serially after surgery with the aim of measuring aortic and pulmonary annuli and evaluate gradients and incompetence and to study the left ventricular function. Intraoperative transoesophageal echocardiography was routinely used. Complete root replacement was performed in all patients. RESULTS: One patient died in the early postoperative period (4%). There was no late death. All survivors remained in NYHA class I and were free of complications and medications. No gradient nor any significant aortic incompetence could be demonstrated. In 17 patients with predominant aortic incompetence before surgery, the left ventricular function was followed prospectively, end-diastolic left ventricular dimensions diminished drastically from 2 +/- 3.4 S.D. above normal to -0.63 +/- 2.4 S.D. at one week postoperatively (day 10) to reach a normal value one to three months after surgery. Left ventricular mass remained abnormal at day 10 (from 4.7 +/- 3.3 S.D. to 5.3 +/- 3.8 S.D.) and diminished more progressively to reach a normal value (0.14 +/- 1.4 S.D.) at three months. This resulted in a significant decrease of end-systolic wall stress (-3.6 +/- 2.1 S.D.) and in a hyperdynamic function in the immediate postoperative days except in two patients. These two patients were characterized preoperatively by more severely dilated left ventricle (end diastolic dimension 5.3 +/- 0.03 versus 1.6 +/- 3 S.D.) with decreased left ventricular wall thickness (1.19 +/- 0.7 versus 3.44 +/- 1.9 S.D.), decreased ratio between end diastolic wall thickness and end diastolic dimension (0.14 +/- 0.06 versus 0.2 +/- 0.06) and a decreased velocity of shortening. Unlike the other 15 patients, the left ventricular function did not recover completely at mid term follow-up in those two patients. CONCLUSION: The Ross operation is a safe procedure and allows us to suppress completely the abnormal loading conditions of the left ventricle, resulting in a complete recovery of left ventricular function in most patients.

The natural history of cardiac rhabdomyoma with and without tuberous sclerosis.

The aim of the present study is to contribute to the knowledge of the natural history of cardiac rhabdomyoma in children with and without tuberous sclerosis. In a retrospective study, 33 children with cardiac rhabdomyoma were collected from three pediatric cardiology centres. In 30/33 patients tuberous sclerosis was associated. High prevalence of cardiac rhabdomyoma was found in infancy, with 21/23 detected before the age of 1 year, and 11/33 before 1 month of age. Cardiac manifestations were present in 19 patients: cardiac rhythm disturbances were detected in 13; in 6/33 a Wolff-Parkinson-White syndrome was documented, of which 4 presented paroxysmal arrhythmias. Obstructive or regurgitative phenomena were present in 5; and in 2 patients surgical removal proved necessary. With the exception of one tumoural mass in the right atrium, all 77 tumours were located somewhere in the ventricles, including at atrio-ventricular valve level. Because of spontaneous regression of most of the tumoural masses, treatment should at first be symptomatic, while surgical removal is required only in life-threatening conditions, as documented in 2 of our 33 patients.

[Cardiovascular evaluation in athletic children]. / Evaluation cardiovasculaire de l'enfant sportif.

Although rare, sports related sudden cardiac deaths in children and adolescents justify the search for risk factors in any child or adolescent who wishes to practice sports. Each time that history and careful clinical cardiovascular examination point to a possible cardiovascular abnormality, an electrocardiogram and an echocardiography must be performed. Exercise testing is useful to appreciate the cardiovascular tolerance, either in normal subjects or in subjects with a cardiovascular abnormality; its interpretation requires good knowledge and understanding of hemodynamic responses to exercise. Indications, risks and procedures of exercise testing are discussed with reference to exercise physiology.

Early balloon dilatation of the pulmonary valve in infants with tetralogy of Fallot. Risks and benefits.

BACKGROUND: Balloon dilatation, an established treatment for pulmonary valve stenosis, remains a controversial procedure in tetralogy of Fallot. METHODS AND RESULTS: Balloon dilatation of the pulmonary valve was performed in 19 infants with tetralogy of Fallot. Its effects on the severity of cyanosis, the growth of the pulmonary valve and pulmonary arteries, and the need for transannular patching were evaluated. Clinical, echographic, angiographic, hemodynamic, and operative data were analyzed. The procedure was safe in all, without significant complications. After balloon dilatation, systemic oxygen saturation increased from a mean value of 79% to 90%. This increase proved to be short-lasting in 4 patients, who required surgery before the age of 6 months. Balloon dilatation increased pulmonary annulus size in each case, from a mean value of 4.9 to 6.9 mm (P < .001). This gain in size remained stable over time, with a mean Z score of -4.8 SD before dilatation, -3.1 SD immediately after the procedure, and -2.7 SD at preoperative catheterization (P < .001). Pulmonary artery dimensions remained unchanged immediately after balloon dilatation but increased at follow-up from a Z score mean value of -2.5 to -0.06 SD and from -2.2 to 0.04 SD for right and left pulmonary arteries, respectively (P < .001). At the time of corrective surgery, the pulmonary annulus was considered large enough to avoid a transannular patch in 69% of the infants. This represented a 30% to 40% reduction in the need for a transannular patch compared with the incidence of transannular patch expected before balloon dilatation. CONCLUSIONS: Pulmonary valve dilatation in infants with tetralogy of Fallot is a relatively safe procedure and appears to produce adequate palliation in most patients. It allowed the growth of the pulmonary annulus and of the pulmonary arteries, resulting in a mean gain of 2 SD for those structures.

Bacterial pericarditis in infancy and childhood.

The authors report on a cooperative study of 43 cases of bacterial pericarditis observed in children. This disorder was suspected in patients with septicemia who developed symptoms and signs of pericarditis (precordial pain, muffled heart sounds, pericardial friction rub, cardiomegaly). Early diagnosis of this condition is now facilitated by echocardiography. A combination of medical and surgical treatments (appropriate antibiotic therapy after culture and sensitivity tests and early pericardial drainage) led to complete recovery in almost all of the cases (42 of 43). After long-term follow-up, no cases of constrictive pericarditis were observed.

[Scimitar syndrome complicated by stenosis of the right pulmonary vein. Apropos of 4 cases]. / Syndrome du cimeterre compliqué de sténose de la veine pulmonaire droite. A propos de 4 observations.

The authors report 4 cases of the scimitar syndrome with pulmonary hypertension by stenosis of an abnormally draining right pulmonary vein and they also review the literature. All cases were symptomatic from infancy. The diagnosis was confirmed by catheterisation which showed a significant pressure gradient between the right pulmonary vein and the inferior vena cava, and by angiography which demonstrated the stenosis. None of the treatments proposed (interventional catheterisation with dilatation and eventual implantation of a stent, surgery with treatment of the stenosis and reimplantation of the right pulmonary vein in the left atrium, or pneumonectomy) were satisfactory. However, it is possible that earlier treatment could be effective as changes in the pulmonary vascular bed seem to occur very early in these patients.

[Percutaneous embolization of a pulmonary arteriovenous fistula in a child]. / Embolisation percutanée d'une fistule artérioveineuse pulmonaire chez un enfant.

BACKGROUND: Pulmonary arteriovenous fistula is a rare vascular abnormality in children that is usually treated by lobectomy. This report describes a case that was improved by coil embolization. CASE REPORT: A thoracic X-ray was routinely taken before amygdalectomy in a 6 year-old girl. It showed a consolidation area in the left superior lobe that persisted despite treatment with erythromycin for 3 weeks. Bronchoscopy was normal, but study of blood gases showed a refractory hypoxemia with moderate cyanosis and hemoglobin at 16.2 g/dl. Right-heart catheterization and angiography showed a pulmonary arteriovenous fistula that was treated by coil embolization. A control investigation 3 months later showed normal arterial oxygen saturation, hemoglobin at 14.5 g/dl and reduced pulmonary condensation. CONCLUSION: Pulmonary arteriovenous fistula may show few symptoms. Coil embolization appears to be a useful initial non-aggressive treatment.

[Value of flexible fiberoptic bronchoscopy under local anesthesia in infants]. / Intérét de la fibroscopie bronchique souple sous anesthésie locale chez le nourrisson.

From October 1991 through April 1992, 16 infants aged 5 to 25 months (mean age 14.3 months) underwent bronchoscopy with a flexible fiberoptic bronchoscope, under local anesthesia. The technique is described in detail. Reasons for bronchoscopy included recurrent or persistent pneumonia (n = 4), persistent atelectasia (n = 4), lymphadenopathy and/or airway compression (n = 2), suspected foreign body (n = 2), bronchoalveolar lavage to investigate diffuse interstitial lung disease (n = 2), and severe recurrent wheezing (n = 2). The procedure established the accurate diagnosis in 14 cases. Adverse events (32%) were minor (transient hypoxia, n = 3; moderate fever, n = 1; and laryngospasm, n = 1) and resolved completely. Flexible fiberoptic bronchoscopy under local anesthesia is a simple procedure which is safe in patients under 30 months of age when performed by a experienced operator in an adequate facility. This method is useful for the diagnosis and/or treatment of a broad spectrum of conditions.

Surgical repair of coarctation of the aorta in infants under one year of age. Long-term results in 146 patients comparing subclavian flap angioplasty and modified end-to-end anastomosis.

Between July 1976 and February 1991, 146 consecutive infants underwent surgical repair of coarctation of the aorta. Age at operation varied from 2 days to 11 months (median 1 month). Ninety-two (63%) were less than 2 months. Isolated coarctation was present in 65 patients (group 1), associated ventricular septal defect in 49 patients (group 2) and complex anomalies in 32 patients (group 3). The majority (65%) were in a critical condition and 45 patients (31%) were artificially ventilated. Subclavian flap angioplasty was performed in 39 patients and resection and end to end anastomosis in 107 patients. Neither hospital mortality was significantly different between subclavian flap angioplasty (15%) and end-to-end anastomosis (18%) nor was the postoperative hypertension. Actuarial survival at 10 years were 100% for group 1, 94% for group 2, and 62% for group 3. Seventeen patients had recurrent coarctation. No significant difference was found in terms of types of repair or age at operation. As no major advantage in terms of mortality and morbidity to either technique was found, we recommend resection and end-to-end anastomosis. This technique not only relieves the obstruction whatever the level is but also eliminates the ductal tissue, preserves the subclavian artery and avoids the use of prosthetic material.

Pulse oximetry versus measured arterial oxygen saturation: a comparison of the Nellcor N100 and the Biox III.

Pulse oximetry is noninvasive, fast, and simple, making it a very popular way of assessing oxygenation in pediatric patients. However, there are few studies that establish the accuracy of this technology over a wide range of oxygen saturations in children. This study, done in 47 children aged from 1 day to 16 years with congenital heart disease and undergoing cardiac catheterization, compared the direct measurement of arterial oxygen saturation to values from pulse oximetry. Oxygen saturation was measured by an IL-282 Co-oximeter, which also measured carboxyhemoglobin and methemoglobin, and was compared to values obtained from both a Biox III and Nellcor N100. Both pusle oximeters gave values that closely correlated with the actual saturation (r = 0.91 and 0.93, respectively) with standard errors of the estimate of 4.1 and 3.2%, respectively. For both devices, the error increased with decreasing saturations, being progressively larger below a saturation of 80%. The difference between the actual saturation and that measured by pulse oximetry bore no relationship to the presence of carboxyhemoglobin, methemoglobin, fetal hemoglobin, bilirubin, cardiac index, or age of the patient. In conclusion, pulse oximetry, while a very useful technology in pediatrics, must be interpreted with some caution in children with severe cyanosis.

Surgical treatment of subvalvular aortic stenosis. Long-term results.

From 1966 till May 1988, 53 patients underwent surgery for fixed subaortic stenosis. Subvalvular obstruction was isolated in 27 patients (Group I) and associated with aortic valve lesions in 26 (Group II). A membranous stricture was documented in 5 patients and a fibromuscular ring in 48. Excision of the ring and myectomy were performed in all patients, and an associated aortic valve replacement or reconstruction in 7 and 4 patients respectively. There were no hospital deaths. Follow-up evaluation in 50 patients ranged from 6 months to 22 years. Eight patients had to be reoperated upon (1 from group I, 7 from group II): aorto-ventriculoplasty was performed in 3, aortic valve replacement with redo myectomy in 3 and mitro-aortic valve replacement in 2. One of them had 2 reoperations. Functional status at the time of the last outpatient visit was most satisfactory. Continued evaluation remains necessary as obstruction may reappear despite the absence of symptoms.

[Interventional catheterization in infantile congenital aortic valve diseases]. / Cathétérisme interventionnel dans les valvulopathies aortiques congénitales en pédiatrie.

The authors present their experience of percutaneous aortic valvuloplasty in 10 neonates and 12 children with critical aortic stenosis. In the neonate group the left ventricular aortic gradient dropped from 49 +/- 20 mmHg to 15 +/- 16 mmHg. This improvement persisted in 57 p. cent of the cases after 2 years. In the older group (mean age 6 years) the gradient was reduced from 73 +/- 22 mmHg to 30 +/- 17 mmHg with maintained results in 78 p. cent after 2 years. Aortic incompetence occurred in the majority of cases but did not seem to be more important than after surgical commissurotomy. In critical stenosis, balloon aortic valvuloplasty seems to be a valuable alternative to surgery as a palliative measure before prosthetic valve replacement.

[Dilation of the aortic root in ventricular septal defects. Risk factor of aortic insufficiency]. / La dilatation du bulbe aortique dans la communication interventriculaire. Facteur de risque d'insuffisance aortique?

The diameter of the aortic root was measured in 151 patients with small ventricular septal defect (Qp/Qs less than 2 and pulmonary vascular resistance less than 1.5 U.m-2) and correlated with the presence of absence of aortic regurgitation. In 26 cases the aortic juxtavalvular region (bulbus) was dilated above 2 SD compared to a control group. All patients with aortic regurgitation of with cusp prolapse belonged to this group of 26 cases. Aortic root dilatation seems to have a specificity of 89 p. 100, a sensitivity of 75 p. 100 and a negative predictive value of 98 p. 100 in relation to the occurrence of aortic insufficiency. The finding of an aortic root dilatation associated with a ventricular septal defect should incite to a closer supervision of non-operated patients should be used as an argument when discussing the surgical closure of the ventricular septal defect.

[Arrhythmogenic dysplasia of the right ventricle in a 16-month-old child]. / Dysplasie arythmogène du ventricule droit chez un enfant de 16 mois.

The clinical history of a 16-month old child with an arrhythmogenic right ventricular dysplasia is reported. This is one of the youngest patients described in the literature. The pitfalls of the diagnosis and the particularities of the treatment of recurrent ventricular tachycardias are underlined.

Ventricular paired pacing to control intractable junctional tachycardia following open heart surgery in a child.

A 5-month-old girl presented postoperatively with an atrioventricular (A-V) junctional tachycardia at a rate of 245/min following surgical repair of tetralogy of Fallot. The systolic blood pressure dropped to 60 mmHg with this rapid heart rate, and the infant became shocked. Drugs and overdrive pacing were ineffective in suppressing the A-V junctional tachycardia and in improving cardiac output. Ventricular paired pacing was used successfully to halve the mechanically effective ventricular rate and to restore cardiac output. When ventricular paired pacing was stopped after 12 h, the spontaneous rhythm was an atrial rhythm with 1-1 A-V conduction. The patient was discharged in sinus rhythm on the 5th postoperative day.

[Various aspects of the medical treatment of newborn infants with heart disease]. / Quelques aspects du traitement médical des nouveau-nés cardiaques.

Some aspects of the medical treatment of newborns with congenital heart disease. The outcome of new-born babies with critical congenital heart disease has dramatically improved over the last 25 years. Better understanding of the abnormal hemodynamic situations and thus more appropriate intensive care decisions, increased accuracy of the diagnosis obtained by color-doppler and echocardiography and improved surgical and anesthetic skills and technics, are the main factors of these better results. The authors describe the benefits of prostaglandin infusion in situations where anatomic anomalies prevent the parallel foetal circulation to change into a serial circulation system: severe stenosis or atresia of right or left cardiac valves, transposition of great vessels. Another example of improved medical treatment concerns balloon valvuloplasty. This new technic offers a valuable alternative to surgery in cases of severe isolated stenosis of aortic or pulmonary valves in the neonatal period.