Total anomalous pulmonary venous connection: morphology and outcome from an international population-based study.

BACKGROUND: late mortality after repair of total anomalous pulmonary venous connection is frequently associated with pulmonary venous obstruction (PVO). We aimed to describe the morphological spectrum of total anomalous pulmonary venous connection and identify risk factors for death and postoperative PVO. METHODS AND RESULTS: we conducted a retrospective, international, collaborative, population-based study involving all 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. All infants with total anomalous pulmonary venous connection born between 1998 and 2004 were identified. Cases with functionally univentricular circulations or atrial isomerism were excluded. All available data and imaging were reviewed. Of 422 live-born cases, 205 (48.6%) had supracardiac, 110 (26.1%) had infracardiac, 67 (15.9%) had cardiac, and 37 (8.8%) had mixed connections. There were 2 cases (0.5%) of common pulmonary vein atresia. Some patients had extremely hypoplastic veins or, rarely, discrete stenosis of the individual veins. Sixty (14.2%) had associated cardiac anomalies. Sixteen died before intervention. Three-year survival for surgically treated patients was 85.2% (95% confidence interval 81.3% to 88.4%). Risk factors for death in multivariable analysis comprised earlier age at surgery, hypoplastic/stenotic pulmonary veins, associated complex cardiac lesions, postoperative pulmonary hypertension, and postoperative PVO. Sixty (14.8%) of the 406 patients undergoing total anomalous pulmonary venous connection repair had postoperative PVO that required reintervention. Three-year survival after initial surgery for patients with postoperative PVO was 58.7% (95% confidence interval 46.2% to 69.2%). Risk factors for postoperative PVO comprised preoperative hypoplastic/stenotic pulmonary veins and absence of a common confluence. CONCLUSIONS: preoperative clinical and morphological features are important risk factors for postoperative PVO and survival.

Medium term outcome for infant repair in tetralogy of Fallot: Indicators for timing of surgery.

OBJECTIVE: To assess the impact of early corrective surgery on the short and medium term outcome in tetralogy of Fallot (TOF). MATERIALS AND METHODS: All patients under 12 months of age undergoing correction of isolated TOF between February 1997 and July 2003 were reviewed retrospectively. Outcome data for mortality, post-operative care management, major morbidity and clinical follow-up were analysed. RESULTS: Fifty-two operations were performed. The mean age at surgery was 5 months (range 1-12) of whom 16 (30.8%) were less than 3 months old, including 2 neonates, 22 (42.3%) were 3-6 months old and 14 (26.9%) were 7-12 months old. There was 1 (1.9%) early death caused by a cerebro-vascular accident and 1 (1.9%) late death secondary to acute infective endocarditis. There were no differences in post-operative morbidities attributable to age. Patients under 3 months old required greater duration of post-operative ventilation, ITU stay and in-hospital stay. At a mean follow-up of 4.0 years (range 1.5-8.0), 33 (63.5%) patients had well-tolerated pulmonary regurgitation (PR) and 3 (5.8%) patients required re-operation for right ventricular outflow tract obstruction (RVOTO). All patients had right bundle-branch-block but with QRS < 150 ms. CONCLUSION: Early definitive repair of TOF can be performed safely on patients under 6 months old. Age at surgery does not appear to affect the medium term haemodynamic outcome. However, early surgery does escalate the need for ICU care. This data suggests repair in asymptomatic patients be delayed until 3-6 months of age.

Pulmonary atresia with intact ventricular septum: range of morphology in a population-based study.

OBJECTIVES: We describe the morphologic variability in pulmonary atresia with intact ventricular septum (PAIVS) within a population-based study. BACKGROUND: An uncommon disease, PAIVS shows considerable morphologic heterogeneity. Clinical reports, based mostly on small samples of patients, may not reflect the true spectrum of pathology of this condition. We have studied the entire range of morphology in a prospective population-based study of patients over a five-year period (1991 to 1995). METHODS; As part of the United Kingdom and Ireland Collaborative Study of PAIVS, all 18 pediatric cardiac centers were visited by a single investigator. Morphologic features of each case were determined by direct review of the echocardiograms and angiocardiograms, from surgical and autopsy reports, and by review of pathology specimens where available. RESULTS: Among 183 live-born infants, atresia was valvar (membranous) in 74.7% and muscular in 25.3%. Muscular obliteration of the apical trabecular cavity, and in some cases its infundibulum, resulted in "bipartite" right ventricle (RV) in 33.6%, and a "unipartite" chamber in 7.7%. The remaining 58.7% had "tripartite" morphology. Coronary arterial abnormalities were identified in 45.8%, including arterial stenoses, interruptions and ectasia in 7.6%. Ebstein's malformation coexisted in 18 patients. Median tricuspid valvar size and RV inlet Z-scores were -5.2 and -5.1, respectively. CONCLUSIONS: This study provides unique data on the diverse pathology of PAIVS in an unselected population. This will help determine if published reports reflect the true spectrum of pathology of the condition.

Reoperations and survival after primary repair of congenital heart defects in children.

OBJECTIVE: The objective of this article is to evaluate the incidence of reoperation and the associated risk and survival after primary repair of congenital heart defects using cardiopulmonary bypass in children. METHODS: We present a retrospective analysis of 1220 consecutive children under 16 years [649 (53%) under 1 year] operated on between 1976 and 2001 by 1 surgeon (J.L.M.). RESULTS: The early (30-day) mortality was 6.9%; 171 patients had 206 reoperations. The early mortality for the first reoperation was 10.4% and for second reoperation, 3.8%. Of the first-time reoperations 63% were inevitable, 15% were planned, and 22% were unexpected. The overall 20-year freedom from reoperation was 83% and survival (including early mortality) was 86%. The 10-year freedom from reoperation and survival, respectively, was as follows: aortic valvotomy, 77% and 92%; pulmonary valvotomy, 90% and 95%; atrial septal defect, 99% and 100%; partial atrioventricular septal defect (AVSD), 81% and 94%; complete AVSD, 74% and 70%; VSD, 95% and 97%; double-outlet right ventricle, 66% and 68%; truncus arteriosus, 54% and 71%; Mustard, 85% and 86%; arterial switch, 78% and 74%; Fontan, 77% and 66%; Fallot, 91% and 93%; and total anomalous pulmonary venous drainage, 89% and 84%. Of those undergoing aortic valvotomy, 53% were infants, but when aortic valve replacement became necessary an adult valve could be inserted. Introduction of the total cavopulmonary connection to the Fontan procedure in 1990 with prior cavopulmonary anastomoses has greatly improved outcome, with only 1 reoperation and no deaths since then. Patients with AVSD required 12 reoperations for mitral regurgitation, mostly through the "cleft," the closure of which in the past 5 years has provided promising results. CONCLUSIONS: The majority of reoperations after repair of congenital heart defects in children are inevitable, and their incidence varies for different types of procedures. These findings will help in informing parents about the possible outcome of surgery, but the introduction of newer techniques may reduce the need for reoperation further. The survival is encouraging.

Repair of tetralogy of Fallot in infancy with a transventricular or a transatrial approach.

OBJECTIVE: The optimal time and approach of repair of tetralogy of Fallot (TOF) remain controversial. The purpose of this study was to evaluate the outcome following repair of TOF in infants with particular regard to the surgical approach used. PATIENTS: One hundred and sixty infants (mean age 195+/-89 days, range 11-364 days) undergoing repair of a simple TOF were studied. Between 1974 and 2000, a transventricular approach (RV) was used in 91 and between 1988 and 2000, a transatrial (RA) approach in 69 infants. Ten of these infants (6.2%) had a previous palliative shunt (four in the RV versus six in the RA group). A transannular patch (TAP) was inserted in 96 (60%) infants (76 versus 20). Follow-up was complete (mean 14.5+/-5.2 versus 6+/-1 years). RESULTS: There were three operative deaths (1.9%), (two in RV versus one in RA group). A re-operation for right ventricular outflow tract obstruction (RVOTO) was performed in 19 patients (3 versus 16). Ten-year freedom from re-operation for RVOTO (+/-standard error of the mean) was 88+/-4% (98+/-2 versus 72+/-6%, P<0.0001). Within the RA group, 5-year freedom from re-operation for RVOTO for those who had a TAP was 79+/-9% and it was 75+/-4% for those having a simple repair. Six patients in the RV group required pulmonary valve replacement (PVR). Ten-year freedom from PVR was 98+/-1% (97+/-2 versus 100%, P=0.3). There were two late deaths, one in each group. Ten-year survival was 97+/-1%. One patient in the RV group developed late recurrent ventricular tachycardia requiring the implantation of a defibrillator. At most recent echocardiography, all but the patient who had the defibrillator had good right and left ventricular function. CONCLUSIONS: Transventricular and transatrial repair of TOF in infancy, are associated with an acceptable operative risk, low incidence of late arrhythmia, good bi-ventricular function and excellent survival. In our experience, however, transatrial repair has a disturbing incidence of early and mid-term residual or recurrent RVOTO, even when a TAP has been used.

Total anomalous pulmonary venous connection: outcome of postoperative pulmonary venous obstruction.

OBJECTIVE: Pulmonary venous obstruction (PVO) is an important cause of late mortality in total anomalous pulmonary venous connection (TAPVC). We aimed to describe current practices for the management of postoperative PVO and the efficacy of the different interventional procedures. METHODS: We conducted a retrospective international collaborative population-based study involving 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. Patients with TAPVC born between January 1, 1998, and December 31, 2004, were identified. Patients with functionally univentricular circulation or atrial isomerism were excluded. All available data and images were reviewed. RESULTS: Of 406 patients undergoing repair of TAPVC, 71 (17.5%) had postoperative PVO. The diagnosis was made within 6 months of surgery in 59 (83%) of the 71 patients. In 12, serial imaging documented change in appearance of the pulmonary veins. Good-sized pulmonary veins can progress to diffusely small veins and rarely atresia. Patients presenting after 6 months had less severe disease; all are alive at most recent follow-up. Fifty-six (13.8%) of 406 patients underwent intervention for postoperative PVO: 44 had surgical treatment and 12 had an initial catheter intervention. One half underwent 1 or more reinterventions. Three-year survival for patients with postoperative PVO was 58.7% (95% confidence intervals, 46.2%-69.2%) with a trend that those having a surgical strategy did better (P = .083). Risk factors for death included earlier presentation after TAPVC repair, diffusely small pulmonary veins at presentation of postoperative PVO, and an increased number of lung segments affected by obstruction. CONCLUSIONS: Postoperative PVO tends to appear in the first 6 months after TAPVC repair and can be progressive. Early intervention for PVO may be indicated before irreversible secondary changes occur.

Hepatic changes in the failing Fontan circulation.

BACKGROUND: The failing Fontan circulation is associated with hepatic impairment. The nature of this liver injury is poorly defined. OBJECTIVE: To establish the gross and histological liver changes of patients with Fontan circulation relative to clinical, biochemical and haemodynamic findings. METHODS: Patients were retrospectively assessed for extracardiac Fontan conversion between September 2003 and June 2005, according to an established clinical protocol. Twelve patients, mean age 24.6 (range 15.8-43.4) years were identified. The mean duration since the initial Fontan procedure was 14.1 (range 6.9-26.4) years. RESULTS: Zonal enhancement of the liver (4/12) on CT was more common in patients with lower hepatic vein pressures (p = 0.007), and in those with absent cardiac cirrhosis on histological examination (p = 0.033). Gastro-oesophageal varices (4/12) were more common in patients with higher hepatic vein pressure (21 (6.3) vs 12.2 (2.2) mm Hg, p = 0.013) and associated with more advanced cirrhosis (p = 0.037). The extent of cirrhosis (7/12) was positively correlated with the hepatic vein pressure (r = 0.83, p = 0.003). A significant positive correlation was found between the Fontan duration and the degree of hepatic fibrosis (r = 0.75, p = 0.013), as well as presence of broad scars (r = 0.71, p = 0.021). Protein-losing enteropathy (5/12) occurred more frequently in patients with longer Fontan duration (11.7 (3.2) vs 17.9 (6.1) years, p = 0.038). CONCLUSIONS: Liver injury, which can be extensive in this patient group, is related to Fontan duration and hepatic vein pressures. CT scan assists non-invasive assessment. Cardiac cirrhosis with the risk of developing gastro-oesophageal varices and regenerative liver nodules, a precursor to hepatocellular carcinoma, is common in this patient group.

Correlations with operative anatomy of real time three-dimensional echocardiographic imaging of congenital aortic valvar stenosis.

OBJECTIVE: To define the anatomic characteristics of the congenitally malformed and severely stenotic aortic valve using trans-thoracic real time three-dimensional echocardiography, and to compare and contrast this with the valvar morphology as seen at surgery. DESIGN: Prospective cross-sectional observational study. SETTING: Tertiary centre for paediatric cardiology. METHODS: All patients requiring aortic valvotomy between December 2003 and July 2004 were evaluated prior to surgery with three-dimensional echocardiography. Full volume loop images were acquired using the Phillips Sonos 7500 system. A single observer analysed the images using "Q lab 4.1" software. The details were then compared with operative findings. RESULTS: We identified 8 consecutive patients, with a median age of 16 weeks, ranging from 1 day to 11 years, with median weight of 7.22 kilograms, ranging from 2.78 to 22 kilograms. The measured diameter of the valvar orifice, and the number of leaflets identified, corresponded closely with surgical assessment. The sites of fusion of the leaflets were correctly identified by the echocardiographic imaging in all cases. Fusion between the right and non-coronary leaflets was identified in half the patients. Dysplasia was observed in 3 patients, with 1 patient having nodules and 2 shown to have excrescences. At surgery, nodules were excised, and excrescences were trimmed. The dysplastic changes correlated well with operative findings, though statistically not significant. CONCLUSION: We recommend trans-thoracic real time three-dimensional echocardiography for the assessment of the congenitally malformed aortic valve, particularly to identify sites of fusion between leaflets and to measure the orificial diameter. The definition of nodularity, and the prognosis of nodules based on the mode of intervention, will need a comparative study of patients submitted to balloon dilation as well as those undergoing surgical valvotomy.

Annuloplasty of the regurgitant mitral valve after myocarditis in children.

Acute myocarditis is characterized by the development of rapid life-threatening congestive heart failure and arrhythmias. In many cases with hemodynamic compromise, medical therapy and mechanical support alone are not sufficient. Various surgical procedures have been tried to bridge patients with myocarditis to both transplant and recovery. Mitral regurgitation is a frequent association with end stage cardiomyopathy and predicts poor outcome. Mitral annuloplasty is well-established in adults with ischemic and dilated cardiomyopathy and the results are superior to medical therapy alone and are comparable to cardiac transplantation. However, its effectiveness and use is not well-established in children with cardiomyopathy. We report our experience in two children.

A novel use of atrial septostomy and stent implantation in a patient with complex cyanotic congenital heart disease and severe pulmonary hypertension.

Patients with unpalliated complex cyanotic congenital heart may have significant morbidity resulting from severe pulmonary hypertension. In late stages, medical management is often difficult, and worsening right heart failure is resistant to medication. The risk of complications and early death can be averted by detailed evaluation and prompt intervention to identify the reversible elements that compound their physiology. It is vital to address any treatable issue to improve a patient's quality of life while awaiting heart-lung transplantation. Our case is a good example of such an approach, where the identification and treatment of reversible pulmonary venous hypertension in the background of irreversible pulmonary arterial hypertension, by offloading the left atrium by atrial septostomy and stent implantation, resulted in significant improvement in the quality of life for the patient.

Different techniques for closure of multiple interatrial communications with the Amplatzer septal occluder.

We present three cases of multiple interatrial communications to illustrate the extended use of the Amplatzer septal occluder and to highlight procedural techniques. The first case demonstrates how a single device can be used to close two adjacent defects. It is important to balloon size both defects and to note if balloon occlusion of one defect closes the other and vice versa. The second case demonstrates closure of multiple atrial septal defects using two devices. The technique needed to obtain an "interleaved" position of the two devices is detailed. This technique allows for a lower profile that reduces stress on the devices and surrounding structures and may decrease the risk of thromboembolism and device embolization. The third case describes device closure of a residual defect adjacent to a previous device implanted at an earlier sitting. In this situation, an overlapping position without the option for "interleaving" is inevitable.

Recommendations for the use of palivizumab as prophylaxis against respiratory syncytial virus in infants with congenital cardiac disease.

New data are emerging on the use of palivizumab as prophylaxis against infection with the respiratory syncytial virus in infants with congenital cardiac disease. Following a 4-year multicentre randomised trial, it was shown that prophylactic injections with palivizumab were effective and safe for such children. Prophylaxis consists of 5, monthly, intramuscular injections of palivizumab, at a dose of 15 mg/kg, given during the season for infection with the respiratory syncytial virus. Timing is at the discretion of the physician, depending on the onset of the season locally. It is suggested that, in the United Kingdom, this should be commenced in mid-September. To help clinicians to identify appropriate candidates for palivizumab, a working group of the British Paediatric Cardiac Association has developed recommendations. Infants, namely those under 1 year old, with congenital cardiac disease likely to benefit from prophylaxis include those with haemodynamically significant lesions, particularly increased pulmonary blood flow with or without cyanosis; pulmonary venous congestion, pulmonary hypertension or long-term pulmonary complications, residual haemodynamic abnormalities following medical or surgical intervention (patients who have undergone cardiopulmonary bypass should receive an injection as soon as they are medically stable), cardiomyopathy requiring treatment, and congenital cardiac disease likely to need hospital admission for medical or surgical intervention during the season of infection with the virus. Prophylaxis with palivizumab may also be indicated, at the discretion of the physician, in some children with complex cardiac disease over the age of 1 year. Children less likely to benefit from prophylaxis are those with haemodynamically insignificant disease, or those with lesions adequately corrected by medical or surgical intervention.