David A. Ashburn

Outcomes of 829 neonates with complete transposition of the great arteries 12–17 years after repair

OBJECTIVEnBetween 1985 and 1989, the surgical management of neonates with complete transposition (TGA) underwent a transition from atrial to arterial repair. We sought to examine the intermediate outcomes and their associated risk factors in neonates repaired during the era of transition.nnnPATIENTS AND METHODSnTwenty-four institutions entered 829 neonates age less than 15 days in a prospective study. Diagnosis was simple TGA (n=631), TGA with ventricular septal defect (VSD) (n=167), TGA with VSD and pulmonary stenosis (TGA/VSD/PS) (n=30), or TGA with PS (n=1). Repair was by arterial switch (n=516), atrial repair (Senning=175, Mustard=110) or Rastelli (n=28). Time-related events were analysed by parametric hazard function modeling and incremental risk factors for mortality, re-intervention, and late functional assessment were sought.nnnRESULTSnSurvival estimates at 6 months, 5, 10, and 15 years are 85, 83, 83, and 81%, respectively. The hazard function for death after repair has two phases: an early rapidly declining phase and an ongoing constant one. Constant phase mortality is less likely after the arterial switch operation and in children with simple TGA. During follow up, at least one re-intervention was required in 167 children (pacemaker, n=35; percutaneous intervention, n=32; baffle re-intervention, n=27; re-operation, n=125). Freedom from re-intervention at 6 months, 5, 10 and 15 years is 93, 82, 77, and 76%, respectively. Of survivors, 87% have been followed up to the last 3 years, including an assessment of functional ability of 562 children (83%). Functional class 15 years after repair is class I in 76%, II in 22%, III in 2%. The proportion in functional class I decreased over time. Psychosocial deficits, especially learning disorders are prevalent.nnnCONCLUSIONSnSurvival 15 years after TGA repair is good with most children functioning well, and results are best after an arterial switch operation. There is an ongoing risk of death that is less after the arterial switch operation. With the exception of Rastelli patients, the likelihood of survivors needing re-intervention after 5 years is low. There is need for improved neurodevelopmental outcomes.

Prevention of Early Sudden Circulatory Collapse After the Norwood Operation

Background—After modifications in our perioperative management protocol, we have observed a decrease in sudden circulatory collapse after the Norwood operation. The current study examines early outcomes after the Norwood operation in our unit in an attempt to identify variables that may have altered the risk of unexpected circulatory collapse. Methods and Results—We studied 105 consecutive neonates who underwent a Norwood operation in our institution. Our treatment protocol has changed in the past 3 years to include the use of alpha-blockade with phenoxybenzamine (POB) for systemic afterload reduction and selective cerebral perfusion. Forty-eight infants had selective cerebral perfusion. Forty-two infants received POB. Sixty patients had hypoplastic left heart syndrome. There was no difference in age, diagnosis, number of neonates with weight <2.5 kg, aortic size diameter <2 mm, highest preoperative lactate level, and shunt size indexed to body weight among patients with or without use of POB. Twenty-five infants had circulatory collapse during the first 72 hours. Twelve of them could be explained by technical issues. Thirteen others who appeared clinically stable had early sudden circulatory collapse without an apparent cause. Sixteen out of 25 neonates died. Of those with technical problems, 8 out of 12 died. Based on the hazard function, 3 incremental risk factors for early circulatory collapse were technical issue at operation (P<0.001), longer cross-clamp time (P<0.007), and no use of POB (P<0.002). For a technically successful operation, freedom from circulatory collapse at 72 hours is 95% with the use of POB versus 69% without (P<0.002). Diagnosis, aortic size, atrioventricular valve function, birth weight, age at operation, and total circulatory arrest time and were not predictive of early sudden circulatory collapse. Conclusion—Recent changes in our treatment protocol have resulted in a decrease incidence of sudden circulatory collapse after the Norwood operation. Optimal surgical technique is the most important predictor of early survival. The use of aggressive afterload reduction with POB reduced the risk of early sudden arrest.

Late results of palliative atrial switch for transposition, ventricular septal defect, and pulmonary vascular obstructive disease

BACKGROUNDnPalliative atrial switch (PAS) procedures that reroute pulmonary and systemic venous drainage and leave a ventricular septal defect (VSD) open have been used in the treatment of deeply cyanotic patients who have severe pulmonary vascular obstructive disease (PVOD). Palliative atrial switch is beneficial for patients with transposition of the great arteries or other complex lesions with VSD who show higher arterial oxygen saturation in the pulmonary artery than in the aorta (transposition hemodynamics/unfavorable streaming). We reviewed the early and late results of PAS (Mustard, n = 25; Senning, n = 3) in patients at two institutions.nnnMETHODSnBetween April 1965 and March 2000, PAS was performed in 28 cyanotic patients (18 male, 10 female). Median age was 10 years (range, 1 to 27). Mean preoperative pulmonary arterial pressure was 68 mm Hg (range, 30 to 121 mm Hg). Mean systemic arterial oxygen saturation was 65% (range, 47% to 80%). The majority of patients (95%) were in New York Heart Association (NYHA) functional class III or IV preoperatively.nnnRESULTSnOverall early mortality was 21%; for patients after 1972 (n = 23), the early mortality was 8.7%. Mean follow-up was 8.3 years (maximum 20). Mean postoperative systemic arterial oxygen saturation was increased significantly to 88% (p < 0.0001). Late survival for early survivors at 5, 10, and 15 years respectively was 84% (59%, 97%), 64% (39%, 88%), and 54% (15%, 72%). The NYHA functional class was significantly improved; 94% of late survivors (n = 17) were in functional class I or II (p = 0.002).nnnCONCLUSIONSnThe PAS operation significantly improves systemic arterial oxygen saturation and quality of life in selected patients with transposition hemodynamics, VSD, and severe PVOD.

Atrioventricular septal defects: effect of bridging leaflet division on early valve function

BACKGROUNDnBridging leaflet division may facilitate repair of atrioventricular septal defects (AVSD). However, the consequences of bridging leaflet division on early valve function and mortality are not well defined.nnnMETHODSnRecords of children undergoing AVSD repair between January 1995 and January 2002 were reviewed. Multivariable analysis defined risk factors for moderate or greater atrioventricular valve regurgitation (AVVR) and death/reoperation within 1 year of repair.nnnRESULTSnA total of 209 children (median age 5 months, median weight 5 kg) had defects whose repair included the possibility of bridging leaflet division. Bridging leaflets divided were both (n = 119, 58%), one (n = 30, 15%), or none (n = 55, 27%). Freedom from AVVR (moderate or greater) is 84%, 80%, and 78% at 1, 6, and 12 months. Risk factors include technical factors: number of bridging leaflets divided, longer cross-clamp time, and right-sided annuloplasty. Other risk factors include preoperative AVVR (moderate or greater), double-orifice or parachute left AV valve, and younger age. Freedom from death/reoperation for AVVR is 96%, 92%, and 90% at 1, 6, and 12 months. Risk factors are preoperative AVVR (moderate or greater) and parachute left AV valve. Findings at reoperation (n = 15, 7.2%) were cleft dehiscence or tear along cleft closure (n = 10), dehiscence of divided leaflet from septation patch (n = 1), or other (n = 4). Operative mortality (n = 6, 2.9%) included failed reoperations for AVVR (n = 4), dehiscence of divided leaflet from septation patch (n = 1), and sepsis (n = 1).nnnCONCLUSIONSnDivision of bridging leaflets is a risk factor for AVVR (moderate or greater) during the first year after repair. Preservation of bridging leaflet integrity may improve valve competency, decrease the need for future reoperation, and eliminate some causes of operative mortality.

Electrophysiologic surgery in patients with congenital heart disease

As patients with congenital heart disease increase in number and age, arrhythmia is becoming a more prevalent and important clinical problem. Although catheter-based therapy has revolutionized the management of arrhythmia, there remains an increasing patient population with congenital heart disease presenting for repair or reoperation with associated atrial or ventricular arrhythmias. Arrhythmia ablation may be safely and effectively included as an adjunct to repair of underlying structural cardiac lesions. Successful electrophysiologic surgery requires accurate preoperative characterization of the arrhythmia.