Total anomalous pulmonary venous connection: impact of prenatal diagnosis.

OBJECTIVES: To investigate whether prenatal screening is effective in the detection of total anomalous pulmonary venous connection (TAPVC) and to identify common prenatal features. METHODS: This was a retrospective collaborative study involving 19 pediatric cardiac centers in the UK, Ireland and Sweden. Cases with TAPVC born between January 1, 1998 and December 31, 2004, and prenatally diagnosed cases whose estimated dates of delivery were within this time frame, were identified. Cases with functionally univentricular circulation or atrial isomerism were excluded. All available data and stored images were reviewed. RESULTS: Four-hundred and twenty-four cases with TAPVC were identified prenatally or postnatally, of whom eight (1.9%) had a prenatal diagnosis of TAPVC. Median gestational age at fetal diagnosis was 26 + 6 (range, 22 + 4 to 32 + 0) weeks. Six further fetuses with TAPVC had an abnormality diagnosed on prenatal ultrasound, but not the TAPVC. This included other congenital heart defects (four cases) and isolated pleural effusion (two cases). Seventeen (4.0%) of the 422 liveborn infants had a first-degree relative with congenital heart disease; and six of 17 had a sibling with TAPVC. Two died in utero. Of the liveborn infants diagnosed prenatally with TAPVC, none required urgent intervention for pulmonary venous obstruction and all were alive and well at a median of 2.3 (range, 1.0-7.0) years after surgical repair. CONCLUSION: Prenatal diagnosis of TAPVC is infrequent using current screening methods. Where there is a family history of TAPVC, specialized fetal echocardiography at 20 and 28 weeks' gestation may be indicated.

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.

Outcomes of transposition of the great arteries in the ear of atrial inflow correction.

OBJECTIVE: To examine long term morbidity and mortality following atrial inflow corrective procedures for transposition of the great arteries (TGA) and to investigate factors that influence morbidity and mortality. DESIGN: Retrospective cohort study from a single centre. SETTING: Cardiology and cardiothoracic surgical unit in a large tertiary referral centre. PATIENTS: All 130 patients who had TGA diagnosed between August 1972 and May 1988 and were considered suitable for atrial inflow correction; 109 of these underwent surgery (operative cohort: 84 Mustard operations and 25 Senning operations); 95 survived to hospital discharge (hospital surviving cohort). MAIN OUTCOME MEASURES: Death and cardiac events. RESULTS: There were relatively good long term results from atrial inflow correction for TGA with 5, 10, and 15 year survivals of 77.3%, 75.9%, and 71.3%. However, there was an appreciable incidence of late cardiac death and events, with 5, 10, and 15 year cardiac event-free survivals of 74.5%, 67.1%, and 39.6%. Supraventricular tachycardia was the only significant risk factor for late cardiac death (relative risk 8.72, 95% confidence interval, 2.86 to 26.64). Senning patients had better event-free survival (p = 0.04). CONCLUSIONS: Atrial inflow correction for TGA has a reasonably good 15 year survival (71.3%), but there is an appreciable incidence of late cardiac deaths and events (15 year event-free survival 39.6%). The Senning procedure is preferable to the Mustard procedure for cases unsuitable for arterial switching.

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.

Treatment of patent ductus arteriosus by placement of two intravascular embolisation coils in a puppy.

A 14-week-old border collie with a history of exercise intolerance was confirmed to have a patent ductus arteriosus (PDA) from colour flow Doppler echocardiography. Under general anaesthesia, angiography was performed to gauge accurately the width of the ductus, and then two intravascular embolisation coils were introduced into the ductus via percutaneous catheterisation of the femoral vein. The coils were both 8 mm in diameter, each with four loops. This led to an immediate disappearance of the murmur. Some slight residual flow was detected at the time of the procedure by angiography, but by 10 days postoperatively there was no PDA flow detectable. The time taken to complete the procedure was 55 minutes, with a total fluoroscopy time of 15 minutes. Placement of intravascular embolisation coils represents a viable alternative to traditional surgical methods of ductus closure.

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.

Total UK multi-centre experience with a novel arterial occlusion device (Duct Occlud pfm).

OBJECTIVE: To report the total UK multicentre experience of a novel arterial occlusion device (Duct Occlud pfm). DESIGN: Descriptive study of selected non-randomised paediatric patients with a variety of aortopulmonary connections. SETTING: Five UK tertiary referral centres for congenital heart disease. PATIENTS AND METHODS: Between March 1994 and February 1995, 57 children aged 2 weeks to 14 years (median 50 months) underwent attempted closure of their aortopulmonary connection. Fifty one had persistent arterial ducts and 9 of them had had a Rashkind umbrella device implanted. Five patients had superfluous modified Blalock-Taussig shunts (mBTS). In one there was also a native major aortopulmonary collateral artery (MAPCA). Another patient had a native major aortopulmonary connection (APC). Transcatheter occlusion was attempted in all cases through a 4 F delivery catheter. RESULTS: Devices were successfully deployed in 49/57 (86%) patients. Seven of 51 cases with persistent arterial ducts were judged too large for the device and a Rashkind umbrella was used. 40 (91%) of the 44 in whom the detachable coil device was used had complete occlusion at 24 hours on colour flow Doppler echocardiography. Devices were successfully deployed in all 6 remaining patients (4 mBTS, 1 mBTS + MAPCA, and 1 APC). Embolisation of a device occurred on 4 occasions. Two devices were not retrieved but caused no apparent clinical problems. CONCLUSION: This novel detachable coil type occlusion system compares favourably with other methods of transcatheter occlusion of native, residual, or surgically created aortopulmonary shunts. The delivery system allows its use in small children.

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.

Double-orifice right atrioventricular valve associated with partial atrioventricular septal defect.

We describe the case of a double-orifice right atrioventricular valve that was discovered at operation for an associated atrioventricular septal defect. The diagnosis and operative management are discussed. Developmental concepts illustrate the mechanism by which this defect arises, and emphasize that its great rarity remains an enigma.

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.

Role of ECMO in the treatment of respiratory syncytial virus bronchiolitis: a collaborative report.

AIM: To report the collaborative experience of extracorporeal membrane oxygenation (ECMO) in the treatment of respiratory syncytial virus (RSV) bronchiolitis between April 1989 and January 1995. METHODS: The medical records of patients with confirmed RSV bronchiolitis referred to three centres (Leicester, Glasgow, and Great Ormond Street) were reviewed. RESULTS: Twenty four infants were identified. Seventeen had been born prematurely (gestational range 23-40 weeks, median 30 weeks). Thirteen infants had been mechanically ventilated after birth and seven of these had evidence of bronchopulmonary dysplasia (BPD). The age of onset of RSV infection varied from three to 64 weeks (mean 17.4 weeks, median 12 weeks). Ventilation before ECMO ranged from one to 16 days and oxygenation indices at the time of referral ranged from 21-73 (mean 39). Ribavirin was used in eight of the 24 patients. Sixteen patients received venoarterial and eight veno-venous ECMO. ECMO hours ranged from 32-647 (median 196 hours). One infant died (survival rate 96%). Cranial ultrasound abnormalities were detected in three patients. However, at follow up only one of the 23 survivors had evidence of developmental delay. CONCLUSION: A group of paediatric patients in whom ECMO can be of benefit has been identified. The use of ECMO should be considered when other means of support prove unsuccessful.

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.