Fetal aortic valvuloplasty: investigating institutional bias in surgical decision-making.

OBJECTIVES: Fetal aortic valvuloplasty may prevent the progression of aortic stenosis to hypoplastic left heart syndrome and allow biventricular rather than univentricular postnatal treatment. This study aimed to investigate whether blinded simulation of a multidisciplinary team approach aids interpretation of multicenter data to uncover institutional bias in postnatal decision-making following fetal cardiac intervention for aortic stenosis. METHODS: The study included 109 cases of prenatally diagnosed aortic stenosis from 13 European countries, of which 32 had undergone fetal cardiac intervention. The multidisciplinary team, blinded to fetal cardiac intervention, institutional location and postnatal treatment, retrospectively assigned a surgical pathway (biventricular or univentricular) based on a review of recorded postnatal imaging and clinical characteristics. The team's decisions were the numerical consensus of silent voting, with case review when a decision was split. Funnel plots showing concordance between the multidisciplinary team and the local team's surgical choice (first pathway) and with outcome (final pathway) were created. RESULTS: In 105 cases the multidisciplinary team reached a consensus decision regarding the surgical pathway, with no decision in four cases because the available imaging records were inadequate. Blinded multidisciplinary team consensus for the first pathway matched the decision of the surgical center in 93/105 (89%) cases, with no difference in agreement between those that had undergone successful fetal cardiac intervention (n = 32) and no (n = 74) or unsuccessful (n = 3) valvuloplasty (no fetal cardiac intervention) (κ = 0.73 (95% CI, 0.38-1.00) vs 0.74 (95% CI, 0.51-0.96)). However, funnel plots comparing multidisciplinary team individual decisions with those of the local teams displayed more discordance (meaning biventricular-univentricular conversion) for the final surgical pathway following fetal cardiac intervention than they did for cases without such intervention (36/74 vs 34/130; P = 0.002), and identified one outlying center. CONCLUSIONS: The use of a blinded multidisciplinary team to simulate decision-making and presentation of data in funnel plots may assist in the interpretation of data submitted to multicenter studies and permit the identification of outliers for further investigation. In the case of aortic stenosis, a high level of agreement was observed between the multidisciplinary team and the surgical centers, but one outlying center was identified.

Pulmonary vein stenosis: initial experience with cutting balloon angioplasty.

OBJECTIVE: To determine the safety and effectiveness of cutting balloon angioplasty for pulmonary vein stenosis (PVS). DESIGN AND SETTING: Retrospective review of case notes and cardiac catheterisation data at the Royal Brompton Hospital. MAIN OUTCOME MEASURES: Diameter of pulmonary vein, tricuspid regurgitant jet velocity on echocardiogram, and percutaneous oxygen saturation before and after cutting balloon angioplasty. RESULTS: Three patients had congenital PVS and three had PVS associated with total anomalous pulmonary venous drainage. A total of 27 PVSs were treated during 12 catheterisation procedures. Median patient age at the time of procedure was 12.5 months (range 1.5-36 months) and weight was 7.1 kg (range 2.8-11.1 kg). Minimum pulmonary vein diameter increased significantly on angiography after cutting balloon angioplasty, from mean (SD) 2.3 (0.7) mm to 4.2 (1.9) mm, mean of differences 1.9 mm (95% confidence interval (CI) 0.9 to 2.9 mm, p = 0.0013). Mean (SD) oxygen saturation rose from 79.6 (12.9)% to 83.9 (9.0)%, mean of differences 4.3% (95% CI 0.7% to 8.0%, p = 0.0238). All children's symptoms improved subjectively. Tricuspid regurgitant jet velocity did not change significantly. The longest time interval before repeat intervention was six months. There were no acute deaths; one patient had a small pulmonary haemorrhage and developed a small aneurysm adjacent to the site of angioplasty. CONCLUSION: Cutting balloon angioplasty is safe in the palliation of PVS in children. It gives some acute relief but often needs to be repeated, as improvement is rarely sustained.

Transcatheter coil occlusion of the arterial duct; results of the European Registry.

AIMS: To report on the data, collected by the Association for European Paediatric Cardiology (AEPC) Registry, on transcatheter coil occlusion of the arterial duct. METHODS AND RESULTS: A retrospective study was conducted of intention-to-treat data from 30 European and Middle Eastern tertiary referral centres which included an analysis of causes of suboptimal outcome. Since 1994, reports have been made on 1291 attempted coil occlusions of the arterial duct in 1258 patients. Median age at procedure was 4 years (range 0.1-52) and median weight was 29 kg (range 1.8-100). Following coil implantation, the immediate occlusion rate was 59%, which rose to 95% at 1 year. A suboptimal outcome occurred on 129 occasions (10% of procedures) and was defined as coil embolization, an abandoned procedure, persistent haemolysis, residual leak requiring a further procedure, flow impairment in adjacent structures and duct re-canalization. A number of clinical factors were chosen but only increasing duct size [odds ratio of 2.6:1 (CI 2-3.2)] and the presence of a tubular shaped duct [odds ratio 2.4:1 (CI 1.4-4)] were positively associated with an unfavourable outcome. CONCLUSION: The results of the European Registry support the view that transcatheter coil occlusion of the persistent arterial duct is a safe and effective procedure. Unfavourable outcomes are more likely when closing larger and/or tubular shaped ducts.

Interventional and surgical management of aortic stenosis and coarctation.

Two patients, aged 42 and 44 years old, presented with the combination of aortic valve stenosis and coarctation. To avoid potential problems associated with one stage repair, both patients underwent successful endovascular stent implantation for coarctation followed within 2 weeks by aortic valve replacement using pulmonary autografts (Ross operation). Spiral thoracic computed tomographic scans were performed within 1 week of stent implantation to ensure the absence of aneurysm formation related to the stent before cardiopulmonary bypass.

Atrioventricular septal defects diagnosed in fetal life: associated cardiac and extra-cardiac abnormalities and outcome.

OBJECTIVES: We sought to establish the outlook for fetuses diagnosed with atrioventricular septal defect (AVSD) prenatally and its relation to additional cardiac, extracardiac and chromosomal abnormalities. BACKGROUND: Prediction of likely outcome of AVSD presenting prenatally is complicated by the wide variation in associated features. METHODS: Computerized records from 14,726 pregnancies referred to a fetal cardiology center were reviewed retrospectively. Pathological reports, postnatal records, follow-up inquiries and review of echocardiographic video recordings supplemented analysis of the records for all those with AVSD. RESULTS: Atrioventricular septal defect was confirmed in 301 fetuses. Eighty-six (39%) of the 218 with known karyotype had trisomy 21, and 21/218 (10%) had other chromosome abnormalities. Right isomerism occurred in 37/301 (12%) fetuses, left isomerism in 62 (20%), mirror image atrial arrangement in 2 (1%), and 200 (67%) had usual arrangement. Atrioventricular septal defect occurred without any other intracardiac abnormality in 155 fetuses (51%). Extracardiac abnormalities and nonkaryotypic syndromes were evident in 40 fetuses (13%, confidence interval [CI] 9.5-17.1%). Uncomplicated cardiac anatomy was significantly associated with the presence of karyotype abnormality (p < 0.0001). Parents opted for termination of pregnancy in 175/298 (58.5%). For the continuing pregnancies, Kaplan-Meier estimates for live birth, survival past the neonatal period and survival to three years were 82% (CI 75.3-88.9%), 55% (CI 46.0%-0/64.3%) and 38% (CI 27.1-48.6%), respectively. Fetal hydrops and earlier year of diagnosis were independent variables with adverse influence on survival. CONCLUSIONS: Despite some improvements in the outlook for AVSD diagnosed prenatally, the overall prognosis remains considerably poorer than that implied from surgical series. The detection of associated cardiac and extracardiac abnormalities is important in order to give the best indication of the likely outcome when counseling parents.

Sildenafil as a selective pulmonary vasodilator in childhood primary pulmonary hypertension.

Primary pulmonary hypertension is a rare disease of childhood, which carries a poor prognosis. Patients often present with severe exercise limitation, and untreated life expectancy is less than 1 year. Pharmacological intervention is directed towards reduction of the raised pulmonary artery pressure with vasodilator treatment, initially with calcium antagonists, although more recently long term prostacyclin treatment has shown benefit in some patients. Heart-lung transplantation remains an option for children with severe disease refractory to therapeutic treatment. A 4 year old Bangladeshi girl with dyspnoea, cyanosis, and signs of a low cardiac output, is described. Initial treatment with prostacyclin was gradually reduced, and maintenance treatment with oral sildenafil (Viagra; Pfizer) instituted. At follow up 3 months later, her exercise capacity was greatly improved and she continues to enjoy a good quality of life without obvious side effects. In view of the encouraging initial results, this may become an acceptable adjunct in treating this patient group.

Left ventricular function in adolescents and adults with restrictive ventricular septal defect and moderate left-to-right shunting.

BACKGROUND: The long-term haemodynamic effects of a restrictive ventricular septal defect permitting moderate left-to-right shunting are not known. PATIENTS AND METHODS: Echocardiographic measurements of left heart dimensions and function were compared between a group of 9 older children and adults (median age 21 years, range 12-24.5 years) having restrictive ventricular septal defects (median Qp/Qs 1.7, range 1.4-2.1) and a group of 10 age matched controls. RESULTS: Left ventricular mass indexed to body surface area was significantly greater in subjects than in controls (102+/-29 vs. 75+/-13 g/m2, p=0.02), although there was no significant difference in the ratio of mass to volume. There were no significant differences between indexes of left ventricular systolic function. Ratios of peak early to late diastolic ventricular filling were lower in those with septal defects (1.5+/-0.3 vs. 1.8+/-0.3, p=0.03), but there were no differences in other indexes of diastolic function. CONCLUSIONS: Resting left ventricular function does not appear to have deteriorated by early adult life in patients with restrictive ventricular septal defects and moderate volume loading. This would support a continued conservative approach to management in these patients.

Transseptal approach in children after patch occlusion of atrial septal defect: first experience with the cutting balloon.

Two children required a transseptal approach to the left heart for endovascular stent redilation late after pericardial patch closure of atrial septal defects performed at the time of their initial surgical intervention. Following perforation of thickened interatrial patches in both patients, cutting balloons were used to create adequate interatrial communications. Cathet. Cardiovasc. Intervent. 48:378-381, 1999.

Stent implantation for aortic coarctation and recoarctation.

OBJECTIVE: To determine the early results of balloon expandable stent implantation for aortic coarctation or recoarctation. DESIGN: Prospective observational study. SETTING: Two paediatric cardiology tertiary referral centres. PATIENTS: 17 patients, median age 17 years (range 4.4 to 45) and median weight 61 kg (17 to 92). Six had native aortic coarctation and 11 had aortic recoarctation; 14 had upper limb systolic hypertension. Of those with recoarctation, eight had had at least one previous balloon dilatation attempt and two of these patients also had further surgical interventions. INTERVENTION: Balloon expandable Palmaz iliac stent implantation. MAIN OUTCOME MEASURES: Systolic pressures gradients, minimum aortic diameter, upper limb blood pressures, and incidence of aneurysm formation. RESULTS: 18 stents were implanted during 18 procedures in the 17 patients. Mean peak systolic pressure gradient fell from 26 mm Hg (95% confidence interval (CI), 21 to 31 mm Hg) before to 5 mm Hg (2 to 8 mm Hg) after stent implantation (p < 0.001), and mean minimum aortic diameter increased from 7 mm (95% CI, 6 to 8 mm) before to 11.3 mm (10 to 12.6 mm) after implantation (p < 0.001). Complications occurred in five patients (bleeding in two, stent migration in two, and aneurysm formation in one). Two patients remained borderline hypertensive and eight were receiving antihypertensive treatment at most recent assessment. CONCLUSIONS: Stent implantation for aortic recoarctation and native coarctation gives good immediate results. Careful follow up is necessary to evaluate complications and the long term effect on blood pressure.

Experimental branch pulmonary artery stenosis angioplasty using a novel cutting balloon.

To determined the safety and efficacy of a bladed balloon in the treatment of branch pulmonary artery stenosis, a model of left pulmonary artery stenosis was surgically created in two-week-old pigs. Seven pigs underwent angioplasty, five with the bladed balloon and two with conventional balloons. Overall, acute results showed a fall in the peak systolic pressure gradients from 8.3 +/- 2.3 mmHg to 3.2 +/- 3.1 mmHg and an increase in the minimum stenotic diameters from 4.5 +/- 2mm to 5.6 +/- mm. Acute pathological examination after cutting angioplasty showed regular luminal cuts that healed completely by four to six weeks in chronically surviving animals. Two of three surviving animals had persistent vessel enlargement at follow-up with one showing little overall change. Cutting balloons are effective in branch pulmonary artery angioplasty and may have clinical applications.

Systemic venous collateral development after the bidirectional cavopulmonary anastomosis. Prevalence and predictors.

OBJECTIVES: To determine the prevalence of systemic venous collaterals after the bidirectional cavopulmonary anastomosis and the factors associated with their development. BACKGROUND: Systemic venous collaterals have been found after cavopulmonary anastomosis. Methods. Cardiac catheterization was performed in 103 patients before and after a bidirectional cavopulmonary anastomosis. RESULTS: After surgery, 51 venous collaterals were identified in 32 patients (31%). Collateral development was associated with an abnormal superior vena caval connection (56% incidence vs. 26% with a single right superior vena cava, p = 0.01) and postoperative factors including pulmonary artery distortion (53% incidence vs. 22% without distortion, p = 0.002); increased superior vena caval mean pressure (14 +/- 5 mm Hg versus 11 +/- 4 mm Hg with no collaterals, p = 0.0002); increased pulmonary artery mean pressure (13 +/- 4 mm Hg vs. 11 +/- 4 mm Hg with no collaterals, p = 0.02); lower right atrial mean pressure (5 +/- 2 mm Hg vs. 6 +/- 3 mm Hg with no collaterals, p = 0.04); and increased mean gradient between superior vena cava and right atrium (8 +/- 3 mm Hg vs. 5 +/- 4 mm Hg with no collaterals, p = 0.0002). Using multiple logistic regression, only this last factor was independently associated with collateral development with an odds ratio per 1 mm Hg of 1.33 (95% CI 1.12-1.58, p = 0.001) for their presence. CONCLUSIONS: Systemic venous collaterals occur frequently after a bidirectional cavopulmonary anastomosis and are found postoperatively when a significant pressure gradient occurs between cava and right atrium.

Echocardiography and cardiac catheterization in the preoperative assessment of ventricular septal defect in infancy.

BACKGROUND: Cardiac catheterization is commonly performed before repair of ventricular septal defect (VSD) in infancy. No study has addressed the accuracy of echocardiography alone in defining all of the important anatomic features in this population. METHODS: Consecutive infants undergoing VSD repair between 1991 and 1995 (n = 156) were reviewed. The number of additional VSDs and the presence of commonly associated lesions were recorded for each technique and compared with a reference standard consisting of a combination of surgical findings plus postoperative echocardiography and clinical findings. The associated lesions were right ventricular muscle bundles, subaortic ridge, and persistent ductus arteriosus. RESULTS: The sensitivity and specificity of echocardiography in the detection of additional VSDs was 60% and 99% compared with 53% and 97% for cardiac catheterization and 73% and 96% for both techniques. These differences were not statistically significant. No differences were seen in the detection of commonly associated lesions. CONCLUSION: Routine preoperative cardiac catheterization for infants with a primary diagnosis of VSD is probably no longer justified.

Unguarded tricuspid orifice with pulmonary atresia: successful radiofrequency ablation of an accessory pathway in an infant.

A male infant with the rare lesion of unguarded tricuspid orifice in the setting of pulmonary valve atresia, intact ventricular septum, and a hypoplastic right ventricle is described. The patient presented with cyanosis at 1 day old; transcutaneous oxygen saturations were between 20% and 30% in room air, and 60% in 100% inspired oxygen. Pre-excitation was found incidentally on the ECG and the potential for rapid antegrade conduction of atrial tachyarrhythmias, after eventual extended palliation with the Fontan procedure, was demonstrated at electrophysiological study. By 11 months old the patient was becoming increasingly cyanosed and interim palliation with a bidirectional cavopulmonary shunt was proposed. Successful radiofrequency ablation of the accessory pathway was performed before bidirectional cavopulmonary shunt, which would have prevented access to the heart via the superior vena cava. Difficulty with femoral venous access because of previous occlusion of a femoral vein was overcome by the use of 2 F pacing electrodes and a 5 F ablation catheter.

Balloon dilation of severe aortic stenosis in the neonate: comparison of anterograde and retrograde catheter approaches.

OBJECTIVES: We sought to compare anterograde and retrograde balloon dilation of severe aortic valve stenosis in neonates. BACKGROUND: There is a high incidence of iliofemoral artery complications after retrograde balloon dilation of the aortic valve in the neonate. Therefore, a nonarterial technique of catheter access to the aortic valve would be worth exploring. METHODS: Group 1 included 11 consecutive patients (median age 6 days, range 1 to 42; median weight 3.5 kg, range 2.16 to 4.25) undergoing attempted anterograde dilation through a femoral venous approach. Group 2 included 15 patients (median age 3 days, range 1 to 35; median weight 3.4 kg, range 2.5 to 4.4 kg) who underwent attempted retrograde dilation, including 2 in whom attempted anterograde approach had failed. RESULTS: The valve was successfully crossed in 9 of 11 anterograde and 13 of 15 retrograde dilations. In both groups, the peak gradient across the valve decreased significantly (both p = 0.001). On echocardiography, the jet width of the aortic incompetence/ annulus diameter ratio was 0.16 +/- 0.08 (mean +/- SD) after anterograde and 0.51 +/- 0.24 after retrograde dilation (p = 0.03), possibly because of unrecognized valve leaflet perforation. Two patients in group 1 developed persistent, mild mitral insufficiency. Femoral artery thrombosis developed in one patient after anterograde dilation and in eight after retrograde dilation (p = 0.03). CONCLUSIONS: This series demonstrates that an anterograde approach for balloon angioplasty of severe neonatal aortic valve stenosis is feasible, achieves good hemodynamic relief and lessens morbidity compared with retrograde arterial techniques.

Coil embolization of a coronary artery to pulmonary artery fistula from an antegrade approach.

Transcatheter embolization of coronary artery fistulae has become the therapy of choice for this uncommon lesion. We report a case in which transcatheter embolization was performed in a nine-year-old boy with a large fistula originating from the proximal portion of the left anterior descending artery and communicating with the pulmonary artery and the right coronary artery. The fistula was occluded 'antegrade' by cannulating the connection with the pulmonary artery to deliver occluding coils.

Medium-term follow up of residual shunting and potential complications after transcatheter occlusion of the ductus arteriosus.

OBJECTIVES: To determine the causes and outcome of residual shunting after transcatheter occlusion of persistent ductus arteriosus with the Rashkind double umbrella occluder, and to determine the potential of the device to produce obstruction to flow in the aorta and left pulmonary artery. DESIGN: Angiographic examination of morphology of ductus followed by prospective clinical and ultrasound evaluation (including cross sectional imaging, colour flow mapping, and pulse wave Doppler) of all patients undergoing occlusion of persistent ductus arteriosus between October 1987 and July 1992. PATIENTS: 140 patients with ages between 0.5 and 78 (median 3.8) years and weights between 6.8 and 74 (median 13.8) kg. INTERVENTIONS: Attempted implantation of the Rashkind double umbrella ductus occluder under angiographic control through a transvenous (n = 136) or transarterial (n = 4) approach. MAIN OUTCOME MEASURES: Successful occlusion of ductus; frequency, pattern, and prognosis of residual shunts; Doppler velocities in left pulmonary artery and aorta; volume loading of the left heart. RESULTS: Including reocclusions the overall rate of successful occlusion was 96%. A total of six devices embolised at the time of operation (4.3%) with no sequelae. There were no anatomical factors that predicted a poor outcome, but suboptimal positioning of the device led to a significantly higher incidence of residual shunts (p < 0.001). Colour flow mapping correctly identified shunts that were unlikely to close spontaneously (n = 9) and to date seven have undergone successful closure with a second device. Encroachment of device legs produced statistically (p < 0.001) but not clinically significant increases in left pulmonary artery Doppler velocities that diminished with time. CONCLUSIONS: Transcatheter occlusion provides a safe and effective means of closing a persistent ductus arteriosus. Doppler colour flow mapping is necessary for follow up and shows those ducts requiring reocclusion. The device did not produce significant disturbance to flow in the pulmonary arteries or aorta.