The Australia and New Zealand Fontan Registry: description and initial results from the first population-based Fontan registry.

BACKGROUND: The Fontan procedure is the final in a series of staged palliations for single-ventricle congenital heart disease, which encompasses rare and heterogeneous cardiac lesions. It represents an unusual and novel physiological state characterised by absence of a subpulmonary ventricle. AIMS: The population is growing steadily, prompting creation of this registry to study their epidemiology, demographic trends, treatment and outcomes. METHODS: This multicentre, binational, prospective and retrospective, web-based registry involving all congenital cardiac centres in the region has identified nearly all Fontan patients in Australia and New Zealand. Patients identified retrospectively were approached for recruitment. New recipients are automatically enrolled prospectively unless they choose to opt-out. Follow-up data are collected yearly. RESULTS: Baseline data were obtained in 1072 patients as at 1 January 2011. Ninety-nine patients died; 64 were lost to follow up. Forty-four per cent of patients lost were between 20 and 30 years of age. The size of the Fontan population is increasing steadily. Among 973 living patients, 541 (56%) gave consent for prospective collection of follow up. Between 1 January 2011 and 1 January 2013, an additional 47 subjects were enrolled prospectively. The current proportion of patients operated with hypoplastic left heart syndrome is currently 29% and is growing rapidly. CONCLUSION: The population surviving after the Fontan procedure has been growing in recent decades, especially since survival with hypoplastic left heart syndrome has improved. The Australia and New Zealand Fontan Registry provides population-based data, and only large databases like this will give opportunities for understanding the population and performing prospective trials.

Clinical impact of transcatheter closure of secundum atrial septal defects with the double umbrella device.

The clinical impact of transcatheter closure of the isolated secundum atrial septal defect was reviewed. Closure by echocardiographic evaluation was 23 +/- 14% at 6 months, 49 +/- 16% at 2 years, and 64 +/- 16% at 4 years, and right ventricular end-diastolic dimensions in patients without residual shunts did not differ significantly from those with residual shunts.

Aortic valve regurgitation after surgical versus percutaneous balloon valvotomy for congenital aortic valve stenosis.

To compare characteristics of aortic regurgitation (AR), the results of 213 procedures (110 balloon aortic valvotomies [BAV] and 103 surgical aortic valvotomies [SAV]) for treatment of congenital aortic valve stenosis were reviewed. These procedures were performed in 187 patients from June 1981 to September 1993. Echocardiograms recorded immediately before, within 6 months afterward, and at latest follow-up were compared. Color Doppler was used to assess the degree of AR and was quantified as the ratio of the regurgitant jet width to valve annulus, the jet width ratio. Whereas BAV patients were older (median age 5.7 years vs 3 months; p = 0.0001), there was no significant difference in median follow-up interval (3.1 years [range 0.5 to 7.2] for BAV vs 3.6 years [range 0.6 to 10.4] for SAV; p = 0.44). The mean balloon-to-annulus ratio for BAV was 0.99 +/- 0.09. An open valvotomy was performed in 83% of surgical cases. Acute systolic gradient reduction and subsequent increase at late follow-up was similar for both groups. Acutely, the mean jet width ratio increased similarly (p = 0.84) for BAV (+9 +/- 15%; p = 0.0001) and SAV (+9 +/- 12%; p = 0.0003) and was not related to age at procedure. At late follow-up, mean jet width ratio further increased significantly in both groups, although there was no difference (p = 0.17) in amount of progression (BAV +10 +/- 12%; p = 0.0001, SAV +15 +/- 13%; p = 0.0002). Thus, BAV and SAV produce AR of similar severity with similar rates of progression.

Comparison of two transcatheter closure methods of persistently patent arterial duct.

A randomized trial of arterial duct occlusion with a double umbrella (DU) or wire coil (WC) was undertaken for patients <18 years of age, weighing >10 kg with isolated ducts < or = 3 mm in diameter. Baseline, procedural, and outcome characteristics were compared in an intention-to-treat analysis according to randomization group. From 40 consecutively screened patients, 2 were not enrolled due to a ductal diameter of >3 mm on initial aortography, 38 patients were randomized to either the DU (n = 20) or WC (n = 18) groups. The groups did not differ significantly with respect to age, weight, gender, duct size, type, or branch pulmonary artery diameters. Crossover occurred only in the DU group, where 4 patients (20%) had a ductal diameter of < or = 1 mm and could not be entered for umbrella placement. All remaining DU group patients had ductal diameters of > or = 1.3 mm (p <0.0001). There were no embolizations or secondary implants in the DU group, but in the WC group there was 1 early and 1 late embolization, with 6 patients (33%) with > or = 2 coils. Mean times for the procedure (DU 68+/-19 minutes; WC 65+/-27 minutes; p = 0.70) and fluoroscopy (DU 14+/-4 minutes; WC 11+/-6 minutes; p = 0.22) did not differ significantly. Angiographic duct closure was documented in 4 of 13 patients (31%) of the DU group and 4 of 18 patients (22%) of the WC group (p = 0.69). Combined with an echocardiogram, closure in 11 of 17 patients with DU (65%) and 13 of 18 patients with WC (72%) (p = 0.64) was documented before hospital discharge. One WC group patient received thrombolytic therapy for a femoral artery thrombus. Follow-up at a median of 6.5 months (range 3.2 to 37) showed closure by Doppler echocardiography in 15 of 19 patients with DU (79%) versus 14 of 18 patients with WC (78%) (p = 1.0). Thus, with a tendency toward similar procedural characteristics and outcomes, the higher cost of the DU system compared with coil implants favors the use of coils for closure of the small arterial duct.

Fundoplication in preterm infants with gastro-oesophageal reflux.

Gastro-oesophageal reflux in preterm infants may result in recurrent pulmonary insult due to aspiration of gastric contents, and intractable obstructive apnoea. Fundoplication is effective in controlling reflux when medical management has failed. Our experience between 1981 and 1990 was reviewed to assess the efficacy of early surgery in the management of 11 such infants. The infants had a median gestational age of 29 weeks and a median birthweight of 1032 g. Nine infants had fundoplication for recurrent aspiration pneumonitis and two for intractable apnoea. The median age at the time of surgery was 100 days and the median weight of the infants was 2640 g. Nine infants were oxygen dependent and two were still ventilated at the time of surgery. The operative procedure was well tolerated by 10 of the 11 infants. Surgery failed to control reflux in two infants, although good control was obtained in the one who had subsequent surgery. Three infants required prolonged ventilation postoperatively; two of them died later from pulmonary failure. The median time to discharge was 24 days (8-113 days). All infants with intractable apnoea were cured by surgery. Fundoplication is an effective method of management when used early in the treatment of chronic gastro-oesophageal reflux in preterm infants. There is minimal morbidity from the surgical procedure.

Unraveling of a Gianturco coil during reocclusion of a patent ductus arteriosus.

The Gianturco spring coil has been employed in the settling of the small arterial duct and for reocclusion of residual shunts; the ability to manipulate the coil is advantageous for optimal results. This case study reports an associated complication of unraveling of the coil. Careful attention to coil appearance during manipulation and the use of 0.038-inch coils may reduce this risk.

Percutaneous transluminal coronary angioplasty in a 30-month-old child with embolic long segment occlusion of the left anterior descending artery.

A 30-mo-old girl developed occlusion of her left anterior descending coronary artery following mitral valve replacement. She presented with refractory angina pectoris. Successful percutaneous transluminal coronary angioplasty of the left anterior descending artery was performed, resulting in restoration of flow, resolution of anginal symptoms, and early improvement in left ventricular function.

Quality assurance of paediatric cardiac surgery: a prospective 6-year analysis.

OBJECTIVE: To audit effective quality assurance methods to monitor outcomes following paediatric cardiac surgery at a single institution. METHODS: All patients undergoing cardiac surgery from January 1996 to December 2001 were enrolled prospectively. Patients were stratified by complexity of surgical procedure into four groups, with Category 4 being the most complex procedure. Outcome measures included death, length of admission and morbidity from complications. RESULTS: A total of 1815 patients underwent 1973 surgical procedures. Of these, 1447 (73.3%) were cardiopulmonary bypass procedures, and 543 (27.5%) were more complex (Category 3 and 4) procedures. Median patient age was 3.5 years (range, 1 day-20 years) and patient weight 15.0 kg (range, 900 g to 90 kg). Sixty-six patients (3.6%) died during the study period. Of the procedures in 1996, 22.7% were classified as complex compared with 29.2% of procedures in 2001. The annual surgical mortality ranged from 1.9-4.7% (P = 0.20), and when mortality was adjusted for complexity of surgery, there was no significant yearly variation in the mortality rate (P = 0.57). Analysis of individual surgeon's results showed no significant difference in the mortality rate by complexity of surgery performed (P = 0.90). Mean ventilation times did not change significantly over time (P = 0.79). The yearly incidence of significant neurological complications ranged from 0.6% to 4.5% and the incidence of arrhythmias from 4.2% to 8.0%. No difference was detected between the years. CONCLUSIONS: Stratifying complexity of surgery proved valuable in monitoring surgical outcomes and detecting differences in performance over time as large subgroups were created for analysis.

Pulmonary veno-occlusive disease: diagnosis during life in four patients.

Pulmonary veno-occlusive disease is a rare form of primary pulmonary hypertension of unknown aetiology. Four cases were diagnosed in young patients. The diagnosis was suspected on the basis of clinical, radiological, echocardiographic, and catheter evidence and confirmed by taking a lung biopsy sample. In all patients the histology showed obstruction of the pulmonary veins by intimal fibrosis. The clinical course of all patients has been one of progressive deterioration. Although there is no specific treatment for this disease, to establish the diagnosis during life is of great importance in overall clinical management, including counselling the patient and family.

Transcatheter embolisation of coronary artery fistulae.

BACKGROUND: Most children with coronary artery fistulae are asymptomatic, but because of associated late morbidity, early intervention is usually indicated. AIM: To assess the outcome following transcatheter embolisation of coronary artery fistulae. PATIENTS AND METHODS: Six children, with a median age of 9.5 years (range: 1.3-13.7 years), underwent transcatheter embolisation of coronary artery fistulae. Four patients had simple fistulae, which drained from the right coronary artery to the right ventricle (n = 2), the left coronary artery to the right ventricle (n = 1), or the left coronary artery to the coronary sinus (n = 1). Two patients had complex multiple fistulae arising from both coronary arteries, which communicated with either the right ventricle or the pulmonary arterial system. RESULTS: A stable position with a 5-Fr coronary catheter was obtained proximally and a 3-Fr coaxial catheter was advanced through the coronary catheter to a distal position in the coronary artery fistulae. The number of embolisation microcoils used per procedure ranged from one to 12, and the coil diameter ranged from 3 mm. Polyvinyl alcohol foam embolisation particles (1000 microm) were used to embolise small fistulae to the pulmonary arterial tree. Complete occlusion was obtained in four patients, while two children were left with insignificant residual shunts. There were no early or late cardiac complications. CONCLUSION: Transcatheter embolisation of coronary artery fistulae is a safe and effective therapy in patients with suitable anatomy.

Transcatheter perforation of the right ventricular outflow tract as initial therapy for pulmonary valve atresia and intact ventricular septum in the newborn.

The clinical impact of transcatheter perforation and dilatation of the right ventricular outflow tract in neonates with pulmonary atresia and intact ventricular septum was reviewed. Between April 1992 and December 1994, 8 neonates underwent transcatheter perforation of the right ventricular outflow tract. Radiofrequency energy was employed in 6 patients and wire perforation in 2 patients. Mean patient age at intervention was 1.9 +/- 0.6 days and weight 3.4 +/- 0.5 kg. Median tricuspid valve annulus was 10.9 mm (range: 4.0-13.0 mm) and Z-value -0.85 (range: -4.5-1.0). The mean right ventricular systolic pressure fell from 117 +/- 16 to 55 +/- 15 mm Hg (P < 0.0001), and the right ventricular to aortic pressure ratio decreased from 1.81 +/- 0.33 to 0.82 +/- 0.28 (P < 0.0001). The arterial duct was patent in all. No acute complications occurred. Aortopulmonary shunts were performed in 7 patients at a median 6 days (range: 3-23 days) following catheterization. One patient developed sepsis and died after surgical resection of infected tissue, while a second patient died of a blocked aortopulmonary shunt 17 months following discharge. Median follow-up for the 6 surviving patients was 8 months (range: 4-32 months). One patient has achieved and a second is awaiting biventricular repair. Transcatheter perforation appears to be a promising form of therapy in selected patients with pulmonary atresia, and potentially facilitates algorithms leading to a biventricular repair.