Terry Robertson

Long-Term Outcomes of Dilated Cardiomyopathy Diagnosed During Childhood: Results from a National Population-Based Study of Childhood Cardiomyopathy

Background— Existing studies of childhood dilated cardiomyopathy deal mainly with early survival. This population-based study examines long-term outcomes for children with dilated cardiomyopathy. Methods and Results— The diagnosis of dilated cardiomyopathy was based on clinical, echocardiographic, and pathological findings. The primary study end point included time to the combined outcome of death or cardiac transplantation. There were 175 patients 0 to <10 years of age at the time of diagnosis. Survival free from death or transplantation was 74% (95% confidence interval, 67–80) 1 year after diagnosis, 62% (95% confidence interval, 55–69) at 10 years, and 56% (95% confidence interval, 46–65) at 20 years. In multivariable analysis, age at diagnosis <4 weeks or >5 years, familial cardiomyopathy, and lower baseline left ventricular fractional shortening Z score were associated with increased risk of death or transplantation, as was lower left ventricular fractional shortening Z score during follow-up. At 15 years after diagnosis, echocardiographic normalization had occurred in 69% of surviving study subjects. Normalization was related to higher baseline left ventricular fractional shortening Z score, higher left ventricular fractional shortening Z score during follow-up, and greater improvement in left ventricular fractional shortening Z score. Children with lymphocytic myocarditis had better survival and a higher rate of echocardiographic normalization. At the latest follow-up, 100 of 104 of survivors (96%) were free of cardiac symptoms, and 83 (80%) were no longer receiving pharmacotherapy. Conclusions— Death or transplantation occurred in 26% of patients with childhood dilated cardiomyopathy within 1 year of diagnosis and ~1% per year thereafter. Risk factors for death or transplantation include age at diagnosis, familial cardiomyopathy, and severity of left ventricular dysfunction. The majority of surviving subjects are well and free of cardiac medication.Background— Existing studies of childhood dilated cardiomyopathy deal mainly with early survival. This population-based study examines long-term outcomes for children with dilated cardiomyopathy. Methods and Results— The diagnosis of dilated cardiomyopathy was based on clinical, echocardiographic, and pathological findings. The primary study end point included time to the combined outcome of death or cardiac transplantation. There were 175 patients 0 to 5 years, familial cardiomyopathy, and lower baseline left ventricular fractional shortening Z score were associated with increased risk of death or transplantation, as was lower left ventricular fractional shortening Z score during follow-up. At 15 years after diagnosis, echocardiographic normalization had occurred in 69% of surviving study subjects. Normalization was related to higher baseline left ventricular fractional shortening Z score, higher left ventricular fractional shortening Z score during follow-up, and greater improvement in left ventricular fractional shortening Z score. Children with lymphocytic myocarditis had better survival and a higher rate of echocardiographic normalization. At the latest follow-up, 100 of 104 of survivors (96%) were free of cardiac symptoms, and 83 (80%) were no longer receiving pharmacotherapy. Conclusions— Death or transplantation occurred in 26% of patients with childhood dilated cardiomyopathy within 1 year of diagnosis and ~1% per year thereafter. Risk factors for death or transplantation include age at diagnosis, familial cardiomyopathy, and severity of left ventricular dysfunction. The majority of surviving subjects are well and free of cardiac medication. # Clinical Perspective {#article-title-45}

Effectiveness of prenatal diagnosis of congenital heart defects in South Australia: A population analysis 1999–2003

Aims: To report on the efficiency of fetal echocardiography, outcome after prenatal diagnosis of congenital heart disease (CHD) and the effectiveness of South Australias obstetric screening program in detecting CHD.

Outcomes of Surgery for Simple Total Anomalous Pulmonary Venous Drainage in Neonates

BACKGROUND Repair of total anomalous pulmonary venous drainage (TAPVD) in neonates remains a challenge as it is often associated with severe obstruction. We describe a large cohort of neonates who underwent TAPVD repair at a single institution. METHODS From 1973 to 2008, 112 patients underwent simple TAPVD surgery during the first month of life. Data collection occurred retrospectively. RESULTS Preoperative pulmonary venous obstruction (PVO) occurred in 89 (79.5%) patients. There were 12 (10.7%) early deaths. Significant risk factors were bypass time greater than 65 minutes (p=0.014) and emergent surgery (p=0.005). Hospital mortality was unchanged throughout the 3 eras (1973 to 1988, 1989 to 1998, 1999 to 2008), despite an increase in patients with preoperative acidosis (p=0.004) and severe TAPVD obstruction (p=0.038) during the recent 10 years. There were 6 (6.25%) late deaths within 2 years of repair. Survival at 20 years was 83.4% (95% confidence interval 75 to 89). Risk factors for late death were operative weight 2.5 kg or less (p=0.004) and postoperative pulmonary hypertensive crisis (p=0.02). Reoperation for recurrent PVO was required in 13 patients (11.9%). Risk factors were operative weight 2.5 kg or less (p=0.035) and postoperative pulmonary hypertensive crisis (p=0.002). Follow-up was 96% complete and survivors (n=90) were asymptomatic at a median age of 11.7 years. CONCLUSIONS Hospital mortality remained unchanged over the 36-year period. Survival beyond 2 years offers excellent outcome. Risk factors for mortality were the preoperative clinical status, prolonged bypass time, persisting micro-obstruction, and low operative weight. A reduction in mortality will likely require development of effective medical management for patients who have peripheral PVO not amenable to surgical repair.

Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collaterals: Neonatal Pulmonary Artery Rehabilitation Without Unifocalization

BACKGROUND This study analyzed a protocol of neonatal rehabilitation of hypoplastic pulmonary arteries in the management of pulmonary atresia, ventricular septal defect (VSD), and major aortopulmonary collateral arteries (MAPCAs). Ideal management of patients with pulmonary atresia, VSD, and MAPCAs is the subject of controversy. METHODS From June 2003 to December 2008, 25 consecutive patients were diagnosed with pulmonary atresia, VSD, and MAPCAs, and 20 were entered into a neonatal shunting regimen. The median age at the first operation was 3.6 weeks (range, 0.7 to 17 weeks). All patients underwent an initial central or modified Blalock-Taussig shunt, or both. Further preparatory procedures included 26 pulmonary artery patch reconstructions, 19 right ventricle-to-pulmonary artery conduits, 4 MAPCA ligations, and 4 further shunts. No patient underwent translocation of the collateral arteries. RESULTS At the latest follow-up, 12 of 20 patients have had a complete repair at a median age of 18 months (range, 11 to 48 months), 6 are awaiting repair, and 2 are considered unlikely to be repaired. No patient was missing follow-up. Median pulmonary artery indices had grown from 14.51 to 118.7 in 17 patients. Postoperative angiograms were performed at a median of 8 months (range, 1.9 to 32.7 months) in 9 of 12 completely repaired patients. The median right/left ventricular pressure ratio was 0.64 (range, 0.54 to 0.91). CONCLUSIONS Rehabilitation of hypoplastic native pulmonary arteries by a neonatal shunting regimen, without MAPCA translocation, for pulmonary atresia, VSD, and MAPCAs, provides encouraging results with excellent early survival.

Sudden Death in Childhood Cardiomyopathy: Results From a Long-Term National Population-Based Study

BACKGROUND Children with cardiomyopathy (CM) are at risk of sudden cardiac death (SCD), but the incidence and risk factors for this outcome are not clear. OBJECTIVES This study sought to determine the incidence and risk factors for SCD in children with varying CM phenotypes from a long-term population-based study of childhood CM. METHODS The NACCS (National Australian Childhood Cardiomyopathy Study) is an ongoing longitudinal cohort study including all children in Australia with primary CM who were diagnosed between January 1, 1987, and December 31, 1996, and were <10 years of age. The cumulative incidence and risk factors for SCD within individual CM phenotypes were explored using survival analysis. RESULTS Of 289 eligible patients, 16 (5.5%) experienced SCD over a median follow-up of 11.9 years (interquartile range: 1.7 to 15.4). The risk of SCD varied according to CM phenotype (p=0.007). The cumulative incidence of SCD at 15 years was 5% for dilated cardiomyopathy (DCM), 6% for hypertrophic cardiomyopathy (HCM), 12% for restrictive cardiomyopathy, and 23% for left ventricular (LV) noncompaction. Older age at diagnosis, positive family history of CM, and severity of LV dysfunction were related to increased risk of SCD in patients with DCM, and a higher posterior wall thickness Z-score was the sole risk factor identified for patients with HCM. CONCLUSIONS Predictors of SCD include CM phenotype, family history of CM (DCM), severity of systolic dysfunction (DCM), and extent of LV hypertrophy (HCM). Continuing follow-up of this cohort into adulthood is likely to reveal an ongoing risk of SCD.

Aortic dissection and rupture in adolescents after tetralogy of Fallot repair

Lobectomy pT2N1M0, IIB Wild type None Bone metastasis (6 mo), DWD (1 y, 5 mo) Lobectomy pT1N0M0, IA Exon 19 (del) None Brain metastasis (2 mo), AWD (3 y, 6 mo) Left pneumonectomy Pathologic CR Exon 19 (del)* Gefitinib (2 y) Brain metastasis (2 y, 4 mo), AWD (2 y, 7 mo) Bilobectomy pT1N1M0, IIA Exon 19 (del) Gefitinib (11 mo) AWOD (11 mo) Lobectomy pT1N2M0, IIIA Unknown None Brain metastasis (5 mo), AWD (2 y) Left extrapleural pneumonectomy pT4N2M0, IIIB Exon 19 (del) Gefitinib (3 mo) Brain metastasis (3 mo), DWD (1 y, 7 mo)

Long-term outcomes of patients with absent pulmonary valve syndrome: 38 years of experience.

BACKGROUND Absent pulmonary valve syndrome is associated with aneurysmal dilatation of the pulmonary arteries and compression of the tracheobronchial tree and may lead to significant respiratory compromise. We describe the outcomes of surgical correction of absent pulmonary valve syndrome and risk factors for mortality and reoperation. METHODS A review of 52 patients with absent pulmonary valve syndrome who underwent surgical correction between 1975 and 2013 was conducted. The median age and weight at repair were 9 months (range, 4 days to 24.2 years) and 6.9 kg (range, 1.8 to 56 kg). Preoperative intubation was required in 15 patients (29%), and 21 patients (40%) underwent urgent repair. The pulmonary valve was replaced with a valved conduit in 16 patients (31%) or monocusp valve in 16 patients (31%). Valveless repair was performed in 20 patients (38%). Pulmonary artery reduction was performed in 39 patients (75%), and 2 patients (4%) underwent a Lecompte maneuver. RESULTS The median follow-up time was 13 years (range, 1 month to 35 years). Early mortality was 18.8% (3 of 16) during 1975 through 1989, 19% (4 of 21) during 1990 through 2000, and 0% (0 of 15) during 2001 through 2013. Late mortality was 6.7% (3 of 45). Overall survival at 5, 10, and 20 years was 81.4%±5.6%. On multivariate analysis, preoperative ventilation (p=0.009) was the only risk factor for overall mortality. Freedom from late reoperation at 5, 10, and 20 years was 79.7%±6.9%, 69.4%±8.2%, and 52.1%±9.8%, respectively. No difference in reoperation rates was found between valved conduit, monocusp, or valveless techniques. Risk factors for late reoperation on multivariate analysis were prematurity (p=0.001) and neonatal primary repair (p=0.007). Longer postoperative ventilation periods were predicted by preoperative ventilation (p<0.001) and surgery during infancy (p=0.01). CONCLUSIONS Long-term survival for absent pulmonary valve syndrome has improved during the last decade. Preoperative ventilation predicted longer postoperative ventilation and mortality.

Repair of Atrioventricular Septal Defect Associated With Tetralogy of Fallot or Double-Outlet Right Ventricle: 30 Years of Experience

BACKGROUND The surgical outcomes of atrioventricular septal defect (AVSD) associated with tetralogy of Fallot (TOF) or double-outlet right ventricle (DORV) have improved in recent times. However, high mortality and reoperation rates are still reported. This study reviews our surgical experience in patients with complete AVSD and TOF or DORV. METHODS Between 1980 and 2010, 48 consecutive patients with AVSD associated with TOF (n = 26) or DORV (n = 22) underwent complete repair; of which, 19 had staged repair. A transatrial-transpulmonary approach with the 2-patch technique was the preferred surgical technique. Data were obtained from inpatient and outpatient medical files. RESULTS The mortality rates were 8.3% (4 of 48) for in-hospital death and 13.6% (6 of 44) for late death. Mean follow-up was 8.0 ± 8.7 years (median 10.8 years; range from 2 months to 30 years). Actuarial survival was 76% at 5 years and 71% at 20 years. No deaths occurred after 2001. Reoperations were required in 16 of 48 patients (33%). The overall freedom from reoperation was 55% at 5 and 20 years. Down syndrome was protective for reoperation (p = 0.022). CONCLUSIONS Complete AVSD associated with TOF or DORV can be repaired with good survival. Detachment of the superior bridging leaflet provided excellent exposure. However, reoperation rate remains high.

Surgical management of pulmonary artery sling in children

OBJECTIVES Pulmonary artery (PA) sling is a rare vascular anomaly associated with congenital tracheal stenosis. The natural history is poor and these patients often require early surgical intervention. We describe our experience with repair of this condition. METHODS From 1984 to 2011, 21 patients with PA sling underwent repair at the Royal Childrens Hospital (median age, 5.9 months). PA sling was associated with compression of the trachea in all patients. Tracheal surgery was required in 12 (57.1%) patients. All patients had an echocardiogram, and concomitant repair of coexisting cardiac anomalies was performed in 6 (28.6%, 6/21) patients. RESULTS Operative mortality was 14.3% (3/21), occurring at 19 days, 4.4 months, and 5 months after surgery. Operative mortality for the first 10 years was 22.2% (1984-1993; 2/9), the next 10 years was 14.3% (1994-2003; 1/7), and 0% for the most recent 7 years (2004-2011; 0/5). All deaths occurred in patients requiring tracheal repair (25%, 3/12). No deaths have occurred since 2004 with introduction of the slide tracheoplasty technique. One (5.6%, 1/18) late death occurred at 8 months after repair. After tracheal repair, intervention for excessive granulations and tracheomalacia was necessary in 6 (50%, 6/12) patients. Median follow-up was 8 years (mean, 8.6 ± 6.4 years; range, 5 months to 20.6 years), and all survivors (100%, 17/17) remain asymptomatic. CONCLUSIONS Children with PA sling who do not require tracheal surgery have excellent outcomes. Mortality is determined by the need for tracheal surgery. However, with the advent of the slide tracheoplasty technique, mortality can be reduced. Survival beyond 1 year after surgery offers excellent prognosis.

Medium‐term outcomes of bovine jugular vein graft and homograft conduits in children

The bovine jugular vein (Contegra) conduit has been described as an alternative to the homograft for right ventricle (RV) to pulmonary artery (PA) connection. We assessed the outcomes of Contegra conduits and homografts at a single institution.