Anomalous right subclavian artery and coarctation-related aneurysm repaired with bilateral subclavian-to-carotid transposition and exclusion stent-grafting.

INTRODUCTION: Aortic arch anomalies are common; however, the presence of concomitant pathology may present a complex management problem. REPORT: A 42 year old lady with anomalous right subclavian artery was found to have recurrent coarctation of the aorta and an aneurysm related to the previous repair. Management of the aneurysm was complicated by the proximity of subclavian artery origins. Bilateral subclavian-to-carotid transposition was undertaken to preserve antegrade vertebral artery flow, with subsequent exclusion stent-grafting of the aneurysm and coarctation. DISCUSSION: This case illustrates combined surgical and interventional radiological repair to deal with a complex thoracic aortic clinical problem.

Kawasaki disease: an update.

Kawasaki disease is one of the commonest vasculitides seen in children. It presents with prolonged fever and a polymorphic exanthem. It is a major cause of acquired heart disease in western society. Its exact cause is not known, but exposure to a superantigen has been suggested as a possible aetiological factor. Diagnosis of Kawasaki disease still relies on clinical criteria (Table 1) and investigations are done mainly to exclude other diseases and to detect early or established cardiac complications. Coronary complications can be reduced significantly by the use of intravenous immunoglobulin therapy combined with oral aspirin. The serious consequences of Kawasaki disease require a heightened awareness of this condition when dealing with childhood exanthems.

Accuracy of prenatal echocardiographic diagnosis and prognosis of fetuses with conotruncal anomalies.

OBJECTIVES: The purpose of the study was to determine the accuracy of the prenatal echocardiographic diagnosis and prognosis of fetuses with conotruncal anomalies. BACKGROUND: The accuracy of prenatal echocardiographic diagnoses of cardiac lesions has been reported, but no previous reports specifically address fetal conotruncal anomalies. METHODS: Medical records of 61 fetuses, in which a fetal diagnosis of a conotruncal anomaly was made, were reviewed. Disease entities included were tetralogy of Fallot (TOF), double outlet right ventricle (DORV), transposition of the great arteries (TGA), and truncus arteriosus (TA). RESULTS: Fetal diagnosis was established at a median of 24.5 weeks' gestation. Termination of pregnancy was chosen in 31% (19/61) of cases. Postnatal assessment of the diagnosis was not obtained in 12 cases. Excluding two sets of conjoined twins, accurate prenatal diagnosis including definition of the great artery orientation was achieved in 36 of 47 cases (77%). Seven of 17 fetuses with DORV anatomy, of which 6 were thought to have a subpulmonary ventricular septal defect (VSD), had incorrect prenatal assessment of the great artery relationships. One fetus thought to show features of TA had aortic atresia with VSD and normal-sized left ventricle. Of the 42 pregnancies that continued, 15 had major extracardiac malformations and/or chromosomal abnormalities of which one died in utero with trisomy-13 and TA. A further nine died within the neonatal period. Among the 27 fetuses without a documented chromosomal or major extracardiac anomaly, 13 (48%) died. Overall, the survival rate beyond 28 days of life was 52% (22/42). In contrast, 75% (6/8) of fetuses with TOF, excluding the absent pulmonary valve syndrome, survived. CONCLUSIONS: Conotruncal anomalies can be diagnosed by prenatal echocardiography with a high degree of accuracy. Defining the exact spatial relationship of the great arteries is problematic in some fetuses. The overall prognosis for fetuses with a conotruncal anomaly is poor, with the exception of uncomplicated TOF.

Balloon expandable stents for systemic venous pathway stenosis late after Mustard's operation.

OBJECTIVES: Description and evaluation of current experience with the use of balloon expandable stents for the relief of systemic venous pathway stenosis late after Mustard's operation. DESIGN: Retrospective observational study of technical procedures, angiographic, and haemodynamic findings. PATIENTS: Twenty long term survivors of Mustard's operation for transposition of the great arteries (TGA) with angiographic evidence of systemic venous pathway narrowing. INTERVENTION: Systemic venous pathway stenoses were stented using balloon expandable Palmaz stents. RESULTS: Twenty seven stents were deployed across 24 stenoses. Seventeen stents were placed in the inferior baffle (16 patients), with an increase in mean (range) minimum diameter from 9.6 (4.5-15.9) to 16.5 (11.9-22.2) mm (p = 0.007), and a reduction in mean pressure gradient from 3.1 (0-8) to 0.67 (0-3) mm Hg (p = 0.002). Eight stents were placed in the superior pathways of eight patients, with diameters widened from 9.1 (3.5-14.1) to 15.2 (8.7-19.2) mm (p = 0.018), and gradients reduced from 6.4 (2-11) to 0.9 (0-2) mm Hg (p = 0.02). Two badly deployed stents were safely withdrawn from their intracardiac positions and redeployed in the iliac vein. Transvenous pacemaker insertion was facilitated by prior stent insertion. CONCLUSIONS: The use of balloon expandable stents for late systemic pathway narrowing after Mustard's operation is safe and effective. The beneficial effects of stenting are likely to be more durable than those of balloon angioplasty alone, but longer term follow up is required.

Aortic valve tumor causing acute myocardial infarction in a child.

A 10-year-old child presented with biventricular failure after an acute myocardial infarction. On investigation a large tumor arising from the aortic valve was diagnosed. The patient underwent successful aortic valve replacement for complete excision of the tumor mass, which was reported to be a papillary fibroelastoma. This case report highlights the unusual presentation of an aortic valve tumor in a child with myocardial infarction. The surgical dilemmas of the timing of the operation and the nature of the operation are discussed.

Transcatheter occlusion of the patent ductus arteriosus with Cook detachable coils.

OBJECTIVE: To report initial experience with a new occlusion device for native and residual patent ductus arteriosus. DESIGN: Descriptive study of consecutive non-randomised patients undergoing a new method of patent ductus arteriosus closure with detachable coils. SETTING: Tertiary centres for paediatric cardiology. PATIENTS: 71 consecutive patients, aged 1.2-22 years, with a patent ductus arteriosus (PDA) underwent elective transcatheter closure. 45 had native PDAs (group A) with a minimum diameter of 1.0 mm-5.0 mm (median 2.0 mm). A further 26 had undergone one or more previous occlusion attempts (group B). INTERVENTIONS: A total of 133 detachable (Cook) spring coils were successfully implanted in 70 patients. The procedure was performed transvenously in 51 patients, retrograde arterially in 13, and by both routes in a further 6 patients. One 5 mm coil migrated but was successfully retrieved. MAIN OUTCOME MEASURES: In group A colour flow Doppler echocardiography showed that complete occlusion was achieved in 40/45 (89%) at 24 hours, 41/45 (91%) at 1 month, and 44/45 (98%) by 6 months post procedure. Occlusion rates in residual PDAs were 22/25 (88%) occluded at 24 hours, 23/25 (92%) at 1 month, and 24/25 (96%) at 6 months follow up. CONCLUSIONS: Transcatheter occlusion using detachable (Cook) spring coils is a safe and effective alternative to presently available devices. The delivery system allows full retrieval of the coil until a satisfactory position is obtained.

Role of ECMO in neonatal myocardial infarction.

A neonate with myocardial infarction who failed to respond to conventional treatment was supported by extracorporeal membrane oxygenation (ECMO). Severe mitral valve regurgitation necessitated mitral valve replacement while receiving ECMO following which the infant was successfully weaned. ECMO should be considered for potentially treatable causes of catastrophic heart failure.

Closure of Blalock-Taussig shunts using a new detachable coil device.

Two children underwent interventional closure of a modified Blalock-Taussig shunt using a new detachable coil system (Duct-Occlud). This procedure has advantages over currently available materials to occlude these and other communications between the systemic and pulmonary circulations.