Anomalous left coronary artery from the pulmonary artery in infants. Which operation? When?

The surgical management of anomalous left coronary artery from the pulmonary artery in infants and small children remains controversial, because the ideal surgical procedure and the optimal time for operation are yet to be determined. From 1977 to 1985, 22 patients less than 4 years of age (mean age 18.2 months) underwent direct aortic reimplantation of the anomalous left coronary artery. There were five operative deaths (23%, confidence limits 13%-36%). The determinant risk factor of early mortality was the severity of preoperative left ventricular dysfunction (p = 0.05), not age at operation (p = 0.64) or preoperative clinical status (p = 0.36). There were not late deaths (mean follow-up 38 months). All survivors but one were symptom free. The reimplanted anomalous left coronary artery was patent in each reevaluated case (9/17). Left ventricular function improved significantly in all survivors. Moderate to severe preoperative mitral incompetence lessened in all patients but one, without mitral valve repair. When technically feasible, direct aortic reimplantation of the anomalous left coronary artery is an attractive procedure because it offers a high rate of patency and avoids the potential drawbacks of procedures involving autogenous venous or arterial tissue. Optimal intraoperative myocardial preservation and institution of temporary left ventricular assistance at the end of the operation may decrease the operative risk. Left ventricular function nearly always recovers after successful revascularization, and resection of left ventricular myocardium is rarely indicated, if ever. Mitral incompetence almost always lessens, and the mitral valve should not be repaired at initial operation; however, residual mitral incompetence may necessitate reoperation in a few cases. In infants with moderate left ventricular damage (usually asymptomatic with medical therapy), surgical treatment should be delayed until 18 to 24 months of age so that it can be performed with a low operative risk. Infants with severely impaired left ventricular function and persistent congestive heart failure should probably undergo operation as soon as the diagnosis has been made.

Transaortic approach in double-outlet right ventricle with subaortic ventricular septal defect.

The standard approach (right ventriculotomy and atriotomy) for surgical repair of double-outlet right ventricle with subaortic ventricular septal defect and pulmonary stenosis may not give an adequate view of the intracardiac defect due-to the presence of anomalous coronary artery anatomy or unusual cavity spacial relationship. Thus, a transverse aortotomy was performed and the left ventricular outflow tract was well visualized through the aortic valve and could be reconstructed precisely. Therefore, the transaortic approach also should be considered for systemic route reconstruction in this group.

Intrapericardial teratoma in a newborn infant: use of fetal echocardiography.

The case of an infant with intrapericardial benign teratoma, which was suspected in utero after fetal echocardiography, is reported. This new approach permitted very early diagnosis and surgical treatment before cardipulmonary distress appeared. The tumor was excised completely, and the patient was asymptomatic three months postoperatively.

Mediastinal stabilization by an expansion prosthesis in postoperative congenital diaphragmatic hernia with severe pulmonary hypoplasia.

The authors illustrate a case of right neonatal congenital diaphragmatic hernia (CDH) of Bochdalek with major pulmonary hypoplasia and postoperative massive mediastinal displacement to the right, leading to tracheal compression and recurrent respiratory distress at every attempt to wean the infant from the ventilator. Mediastinal stabilization was obtained by placing an expansion prosthesis (of the cutaneous type) in the right hemithorax, to prevent mediastinal obstruction of the main airways. This procedure may prove to be an original and efficient solution for the critical postoperative respiratory obstruction due to severe pulmonary hypoplasia and mediastinal mass effect in the newborn with CDH. However, this may not prevent progressive scoliosis, a serious problem frequently seen in the absence of one lung.

[Severe hemolysis after endoluminal closure of the ductus arteriosus and recovery following implantation of a second obturator]. / Hémolyse sévère après fermeture d'un canal artériel par voie endoluminale et guérison par implantation d'une deuxième ombrelle.

A few hours after closure of a large patent ductus arteriosus by the endoluminal approach in a 28 month old infant, severe hemolysis occurred due to incomplete closure; cure was obtained by the implantation of a second obturator by the femoral vein. Definitive complete occlusion of the ductus was followed by immediate disappearance of the hemolysis.

[Cardiac rhabdomyoma and apoptosis: are regression controlled by the body?]. / Rhabdomyomes cardiaques et apoptose: les régressions sont-elles contrôlées par l'organisme?

Three rhabdomyomas detected in utero regressed after birth. These personal observations and others in medical literature show that regressions are more frequent than it was formerly believed, and are almost always observed during the first two years of life. The same clinical features of these involutions, histological appearances of apoptosis (programmed cell death), and the absence of inflammation suggest that spontaneous regressions may be controlled by the body. Regression of rhabdomyomas may be compared with other observations of benign and malignant tumours which show spontaneous involution in early life. Medical treatment is advisable even in the absence of life-threatening symptoms.

[Quantitative echography of the fetal heart]. / Echographie quantitative du coeur foetal.

One hundred and twenty two fetal echocardiograms were performed during normal pregnancies between the 20th and 38th weeks of gestation. Freeze frame M mode and 2D imaging was used to perform the following measurements: right and left systolic and diastolic ventricular dimensions, dimensions of the two atria, aortic and pulmonary artery dimensions, fractional shortening of both ventricles, ratio of right ventricular to left ventricular end diastolic dimensions RVEDD/LVEDD, ratio of pulmonary artery to aortic dimensions PA/Ao. The causes of error or failure were analysed. The results classified by gestational age show progressive growth of all structures which double their size during the second half of the pregnancy; the "physiological" septal hypertrophy reported by some workers was not observed in this series; similarly, paradoxical septal motion was only present in 3% of the fetuses studied. The RVEDD/LVEDD ratio approached unity, which questions the classical but hypothetical concept of physiological right ventricular preponderance, a concept also queried by recent fetal Doppler intracardiac flow studies. The repertory of normal values is particularly useful for the detection of cardiac malformations and for the surveillance of some pregnancies with a high risk of cardiomyopathy, as in maternal diabetes.

[Digital subtraction angiography in pediatrics]. / Angiocardiographie numérisée en pédiatrie.

In spite of the prominent position reached by echocardiography in the anatomical and functional diagnosis of congenital heart disease, angiography remains the reference method for preoperative assessment of most cardiovascular malformations. Digitalization of angiographic images, recently applied to cardiology, is of particular interest in paediatrics: the versatility of the method, the smaller amount and dilution of the contrast medium, the diminution of catheter size and the shorter exposure to X-rays are factors that reduce the risk of this invasive investigation. The experience acquired by the exploration of 500 congenital heart disease shows that there is no technical obstacle to using this method in paediatric cardiology, especially since the images obtained clearly are of better quality. Digital angiocardiography already has superseded the conventional analogical exploration in paediatric patients.

[Ductus arteriosus in neonatal cyanotic cardiopathies. Value of the recording of the cutaneous partial pressure of oxygen]. / Le canal artériel dans les cardiopathies cyanogènes néonatales. Intérêt de l'enregistrement de la pression partielle cutanée en oxygène.

Adaptation to extrauterine life is always precarious in the newborn with cyanotic cardiac malformations. Limited survival is possible in cases with obstructive lesions of the right heart providing ductal closure is delayed. This was monitored in 3 cyanotic neonates with ductal dependant cardiac malformations (tricuspid atresia, transposition of the great arteries with atresia or severe stenosis of the pulmonary valve) by continuous measurement of the cutaneous pO2. The value of cutaneous pO2 monitoring has already been established. It is a reliable indicator of arterial pO2 in the neonatal period. Its major advantage, apart from the possibility of continuous monitoring, is that it is non-invasive and may be performed at the bedside. Analysis of curves recorded over several hours or days showed the cyclical nature of cutaneous pO2, probably related to slow variations in ductal diameter equilibrating effective pulmonary flow, arterial pO2 and ductal constriction. Prolonged hyperoxygenation of these desaturated children does not usually improve their condition; progressive deterioration due to the constrictive effect of oxygen on the ductus may be observed. When a clear decrease in the amplitude of oscillation is recorded ductal closure is imminent and palliative surgery should be undertaken whenever possible.

[Measurement of right systolic time intervals in complete transposition of the great vessels. Value in the surveillance of ventricular function]. / Mesure des intervalles de temps systoliques droits dans la transposition complète des gros vaisseaux. Intérêt dans la surveillance de la fonction ventriculaire.

Right ventricular function was regularly assessed pre- and postoperatively by measuring right systolic time intervals at the aortic valve in 21 children with complete transposition of the great arteries. Twenty children underwent "physiological" surgical correction (Senning procedure) and one child with right ventricular hypokinesia underwent detransposition of the arteries. The mean value of the right ventricular pre ejection/ejection period ratio was 0.40 +/- 0.05; this was independent of age and no significant difference was observed between the pre- and postoperative periods (0.41 +/- 0.06 vs 0.39 +/- 0.04) at least with a maximum follow-up of 5 years. This index allows early diagnosis of right ventricular dysfunction and therefore helps to orientate the choice between "physiological" or "anatomical" surgical correction.

[Fetal supraventricular tachycardia associated with anasarca: poor prognosis despite treatment. Apropos of two cases]. / Tachycardies supraventriculaires foetales avec anasarque: mauvais pronostic malgré le traitement. A propos de deux cas.

Two cases of foetal supraventricular tachycardia with hydrops with fatal outcomes illustrate the poor general prognosis of this condition. The absence of therapeutic consensus, of large series in the existing literature, does not prevent logical and reasonable management based on rhythmological, pharmacological and prognostic criteria. A combined approach associating antiarrhythmic therapy by the transplacental and intrafunicular approaches seems acceptable now that funicular puncture can be undertaken easily, and certain antiarrhythmic molecules suggest encouraging results. It is important to try to assess the haemodynamic tolerance by foetal Doppler echocardiography because the foetal prognosis depends on ischaemic cerebral lesions induced by anoxia, difficult to diagnose in utero: when observed, aggressive and occasionally dangerous therapies to foetus and mother may be justified in these extreme situations of foetoplacental hydrops.

[Percutaneous valvotomy of aortic valve stenoses in children]. / Valvulotomie percutanée des sténoses valvulaires aortiques de l'enfant.

A retrospective cooperative study of percutaneous aortic valvuloplasty was undertaken in 12 French centres from 1985 and included 90 children over 3 months of age (average 8.5 +/- 5.2 years) treated for isolated aortic valve stenosis with peak transvalvular pressure gradients of 80.7 +/- 23 mmHg. over 50 mmHg in 92% of cases. The majority of cases were performed by a retrograde femoral arterial approach with inflation of a balloon with a diameter approximating that of the aortic annulus. There was one serious complication (lethal collapse occurring before dilatation) and 12% of local complications due to arterial trauma: the latter were temporary or accessible to simple therapeutic measures. Overall, the pressure gradient decreased by an average of 39.2 +/- 25.4 mmHg. There were 15 immediate failures (17%) and 21 partial results (23%) requiring a further procedure at varying intervals after the valvuloplasty. There were 54 primary successes (60%) with annulation of the pressure gradient and this result was sustained in 45 of the 51 cases followed up for an average of 34 +/- 21 months. Aortic regurgitation was observed or aggravated in 29 children; at the end of the study, this remained a serious problem in 15 cases (17%). The authors conclude that interventional catheterisation is an elegant. simple and relatively economical alternative to conventional surgery. It is as safe, but less immediately effective overall; aortic continence may be compromised in the long-term. In the absence of technical innovations, a parallel development of the two therapeutic procedures is to be expected.

[Interruption of the aortic arch and malformative cardiac lesions requiring repair under extracorporeal circulation. Apropos of 3 cases]. / Interruption de l'arche aortique et lésions malformatives cardiaques imposant une réparation sous circulation extracorporelle. A propos de trois cas.

Interruption of the aortic arch is practically always associated with intracardiac malformations of variable complexity, at the least, a ventricular septal defect. Surgery is usually performed in two stages: aortic repair and pulmonary artery banding after intravenous prostaglandin administration. The second stage comprises debanding and repair of the intracardiac lesions under cardiopulmonary bypass. However, in some cases, interruption of the aortic arch is associated with intracardiac lesions which necessitate correction under cardiopulmonary bypass from the onset, this was the situation in two of the three cases described by the authors: aorto-pulmonary window, a lesion which can only be corrected under circulatory arrest and deep hypothermia. One of these two children, operated in the neonatal period, did not survive: the other, operated at 6 weeks, had an excellent result. In the third case, the association of tricuspid atresia and a restrictive ventricular septal defect necessitated enlargement of the septal defect and therefore, open heart surgery under circulatory arrest; the results were favourable.

[Aortic valve stenosis. Prenatal diagnosis. A case]. / Rétrécissement aortique valvulaire. Diagnostic prénatal. Une observation.

Closed aortic valvotomy through the left ventricular apex could be performed in a 3-day old child thanks to an echocardiographic diagnosis in utero, at 32 weeks, of aortic stenosis, confirmed immediately after birth. The functional and echocardiographic results 5 months after the operation are excellent. This case shows that the prenatal diagnosis of cardiac malformation is feasible and facilitates an early treatment in the post-natal period.

Transcatheter closure of patent foramen ovale in patients with platypnea-orthodeoxia: results of a multicentric French registry.

BACKGROUND: Dyspnea and the decrease in arterial saturation in the upright position in elderly subjects is described as platypnea-orthodeoxia syndrome (POS). POS is secondary to the occurrence of an atrial right-to-left shunt through a patent foramen ovale (PFO). METHODS: This French multicentric study reports on 78 patients (mean age 67 +/- 11.3 years) with POS who had transcatheter closure of the PFO; frequently associated diseases were pneumonectomy (n = 36) and an ascending aortic aneurysm (n = 11). In all patients, the diagnosis was confirmed by transthoracic or/and transesophageal echocardiography. Five different closure devices were used: Amplatz (n = 45), Cardioseal (n = 13), Sideris (n = 11), Das Angel Wings (n = 8) and Starflex (n = 1). Closure was successful in 76 patients (97%). RESULTS: Oxygen saturation increased immediately after occlusion from 84.6 +/- 10.7% to 95.1 +/- 6.4% (p < 0.001) and dyspnea improved from grade 2.7 +/- 0.7 to grade 1 +/- 1 (p < 0.001). A small residual shunt was immediately observed in 5 patients (3 with the Cardioseal device, 1 with the Sideris and 1 with the Amplatz) leading to the implantation of a second device in one case (Cardioseal). Two early deaths occurred unrelated to the procedure (one due to sepsis probably related to pneumonectomy, another due to respiratory insufficiency). Other complications were: a small shunt between the aorta and the left atrium, two atrial fibrillations and a left-sided thrombus which disappeared with anticoagulant therapy. At a mean follow-up of 15 +/- 12 months, there were 7 late deaths related to the underlying disease. CONCLUSION: Percutaneous occlusion of the foramen ovale is safe and gives excellent results thanks to continuing improvement in available devices. This technique enables some patients in an unstable condition to avoid a surgical closure.