Stent Angioplasty for Critical Native Aortic Coarctation in Three Infants: Up to 15-Year Follow-Up Without Surgical Intervention and Review of the Literature.

Management of neonatal native coarctation is debated till now. Surgical therapy remains an option but may be unwarranted in critically sick infants with complex lesions. Balloon dilatation has been employed but with early re-stenosis. Stent angioplasty has also been used but as a bridge towards definitive surgical therapy. Four critically sick infants with complex coarctation and additional co-morbidity factors underwent primary stent therapy as surgical intervention was denied. One patient had died earlier due to reasons unrelated to the procedure. Three survivors underwent multiple dilatations of primary stents as indicated. One of the three survivors did not require any further dilatation after the age of 5 years and remained stable till the time of reporting. High-pressure Cheatham Platinum stents were implanted inside the primary stents in two infants, who developed re-stenosis due to somatic growth. These stents were further balloon dilated at high atmospheric pressure. Femoral arteries in both of them were blocked but were re-canalized after balloon dilatation in one and stent angioplasty in the other. After a follow-up of about 15 years, all of them have been doing fine with acceptable Doppler gradients. They were normotensive and on no cardiac medications. It can be concluded that, though surgical repair remains a standard of care, stent angioplasty in selected infants with complex lesions is feasible and effective. Multiple dilatations can be performed without added risk of stent migration. Bio-absorbable and growth stents hold a promise for future use in such situations.

Stent angioplasty: an effective alternative in selected infants with critical native aortic coarctation.

Management of native aortic coarctation during early months of life poses therapeutic challenges, and there is no consensus among medical professionals regarding a management plan. Much can be argued about the benefits, limitations, and/or complications of transcatheter versus surgical intervention in such cases. Occasionally, the complexity of the lesions limits management options. Therefore, each patient requires individual management decisions because there is no one therapeutic plan that satisfies all patients. In this report, four critically ill infants who had complex native coarctation are presented. Surgical repair was not possible because of relative contraindications. The patients underwent transcatheter stent implantation (six procedures and seven stents) as a nondefinitive procedure with acceptable results. Three patients improved. One patient did not survive, mainly due to other major complications. Multiple reexpansions of the stents were carried out when indicated. After a mean follow-up of 45 months (range, 41-49), the three survivors were doing fine and had gained an average weight of 9.7 kg (range, 6.6-13.3). At the time of reporting, the relative contraindications no longer exist and the final surgical repair can be carried out. Our experience suggests that in certain situations and in critically ill infants with complex form of coarctation, stent angioplasty can be used as a life-saving palliative procedure. Further reexpansions can be done when required. This may serve as a bridge to major surgical repair in the future.

Transcatheter occlusion of a large left coronary artery to right superior vena cava fistula using the Amplatzer duct occluder device.

Transcatheter embolization has become the therapy of choice for most coronary artery fistula. We report a 5.9-kg infant with a coronary artery fistula from the left coronary artery to the right superior vena cava with significant congestive heart failure. The infant underwent successful complete occlusion using the Amplatzer duct occluder.

Congenital heart defects in Sotos syndrome.

Sotos syndrome is a relatively common overgrowth syndrome with characteristic physiognomy. We report on 3 patients with congenital heart defects out of 14 Sotos syndrome patients studied clinically and or by echocardiography. Review showed another 17 patients with variable cardiac defects, mostly closure defects, making an overall incidence of approximately 8%.

[Results of percutaneous mitral dilatation in 11 cases of poorly tolerated mitral stenosis during pregnancy]. / Résultats de la dilatation mitrale percutanée dans 11 cas de rétrécissement mitral mal toléré durant la grossesse.

The authors report the results of percutaneous mitral dilatation (PMD) during pregnancy in 11 cases of tight and poorly tolerated mitral stenosis (MS). This dilatation was performed on average at 22 +/- 5 weeks of pregnancy, protecting the fetus against radiation and using the double balloon technique. Fluoroscopy time, with a mean of 10.5 +/- 2.2 minutes, was greatly reduced in the last five patients by the elimination of cineangiography. Mitral insufficiency was then sought and estimated by colour Doppler. This also avoided iodine overload and its possible effects on the fetal thyroid. Mitral surface area and hemodynamic parameters were very significantly improved by PMD: mean capillary pressure fell from 25.1 +/- 6.2 to 10.7 +/- 4.4 mmHg, mean transmitral gradient from 18.8 +/- 6.2 to 5.9 +/- 2.9 mmHg and mitral surface area increased from 0.9 +/- 0.2 to 2.1 +/- 0.4 cm2 (p < 0.0001). Pregnancy continued under good hemodynamic conditions until delivery which occurred after 8 and a half months, vaginally, in 10 cases. Cesarean section was performed at 8 months in only one patient because of fetal distress not related to the dilatation and with a satisfactory outcome. The 11 children, now with a mean age of 18 months, are all well and free of any functional or organic abnormality. Measurement of thyroid hormone levels in the first 6 also proved normal. In the light of these results, PMD during pregnancy can be considered as an excellent alternative to surgery when it is possible in terms of the anatomical status of the mitral valve.

[Study of cor triatriatum. Apropos of 6 cases]. / Contribution à l'étude du coeur triatrial. A propos de six observations.

The authors report 6 cases of triatrial heart with various and non-specific manifestations and attribute the difficulty of its clinical identification above all to the frequency of associated lesions, which were present in 5 of the 6 cases reported here. An atrial septal defect and left superior vena cava were each seen twice while a ventricular septal defect, sub-valvular aortic stenosis, mitral insufficiency and transposition of the great vessels were each seen once. It was routine echocardiography which led to the discovery of triatrial heart in each of these 6 cases. The value of this investigation in the positive diagnosis of triatrial heart proved, in these patients, to be greater than that of cardiac catheterisation.

[Superior vena cava syndrome disclosing dissection of the aorta]. / Syndrome cave supérieur révélateur d'une dissection de l'aorte.

The authors report a case of dissection of the aorta presenting as a superior vena cava syndrome. Because of the rarity of such a manifestation, this dissection was not suspected before CT scan. A review of the literature confirms the rarity of obstruction of the superior vena cava during dissection and indicates that it results from a compressive hemomediastinum. In this case, the superior vena cava was compressed by the ascending aorta itself, which had become very large but had not ruptured into the mediastinum. This forms the basis of the originality of the case.

[Endomyocardial fibrosis in children]. / Fibrose endomyocardique chez l'enfant.

BACKGROUND: Recent advances in imaging techniques can facilitate the diagnosis of endomyocardial fibrosis. CASE REPORTS: Two cases of endomyocardial fibrosis were diagnosed in two Tunisian children, one aged 3 years and the other 12 years. Both were admitted with severe cardiac failure, predominantly of the right ventricule. Two dimensional echocardiography showed an apical amputation with echogenic material plus a thick, dense endocardial image, particularly at the level of the papillary muscles. The older child who also had cardiac catheterization, died 2 years later and the younger child died 4 years after diagnosis. Post mortem examination showed fibroelastic thickening of the endocardium and areas of fibrosis in the muscles of both children. CONCLUSIONS: The advantage of two-dimensional echocardiography over other imaging techniques is that it can confirm the diagnosis of endomyocardial fibrosis and indicate the extent and degree of fibrosis.

[Cardiac involvement in mucopolysaccharidosis. Apropos of an echocardiographic study in 8 cases]. / L'atteinte cardiaque dans la mucopolysaccharidose. A propos d'une étude échocardiographique de huit cas.

Of all the storage diseases, mucopolysaccharidosis is the one whose cardiac manifestations are probably the least well known. Clinical and above all echocardiographic findings of heart involvement were studied in 8 patients with mucopolysaccharidosis, including four with Hunter disease. The paucity of clinical manifestations was in sharp contrast with the highly informative echocardiographic results. Valvular dystrophy, usually of the left side of the heart, was the most common anomaly, with five patients affected. Whereas some valvular lesions had no consequences, others led to stenosis or incompetence. Asymmetrical hypertrophy of the septum was found in one patient. No patient had evidence suggestive of vascular involvement, in particular of the coronary arteries.