Complete repair of Ebstein anomaly in neonates and young infants: a 16-year follow-up.

OBJECTIVE: The purpose of this study was to review the long-term outcome of patients with Ebstein anomaly who underwent complete repair as neonates and young infants. METHODS: Between March 1994 and May 2010, 32 patients (23 neonates and 9 young infants) underwent surgery for Ebstein anomaly. Mean weight was 3.9 ± 2.0 kg (range, 1.9-8.6 kg). The Great Ormond Street Echocardiography score was greater than 1.5 in 22 of the 23 neonates and greater than 1.0 in all infants. All associated cardiac defects were repaired including pulmonary atresia in 15 and ventricular septal defect in 4. Primary outcome measures included (1) early and late survival, (2) freedom from reoperation, (3) durability of tricuspid valve repair, and (4) functional status. RESULTS: Early survival was 78.1% (25/32). There was 1 late death. Fifteen-year survival estimate was 74% ± 8%. For patients with pulmonary atresia, early and late survival was 60% ± 12% (9/15) versus 94.1% (16/17) (P < .05), respectively; for those without pulmonary atresia, early and late survival was 60% ± 12% versus 85% ± 10 (P = .06), respectively. The mean follow-up was 5.9 ± 4.5 years (0.1-16 years). A biventricular repair was achieved in 90.6% (29/32) patients. Median preoperative tricuspid regurgitation was 4/4 and at late follow-up, 1/4. Freedom from reoperation at 15 years was 74% ± 10% for patients undergoing biventricular repair. All survivors are in New York Heart Association class I or II. CONCLUSIONS: Biventricular repair of Ebstein anomaly in symptomatic neonates is feasible with good early and late survival, especially in those without pulmonary atresia. Tricuspid valve repair is durable, and functional status is excellent.

Repair of neonates and young infants with Ebstein's anomaly and related disorders.

BACKGROUND: Severely symptomatic neonates and young infants with Ebstein's anomaly usually die without surgical intervention. The relative risks and benefits of single-ventricle palliation versus a two-ventricle repair are uncertain. In a recent series, 69% early survival with single-ventricle palliation was reported in 16 neonates with Ebstein's anomaly. Our institutional bias has been to do a two-ventricle repair in all such patients. METHODS: We reviewed our entire surgical experience with a two-ventricle repair in the severely symptomatic neonate (n = 22) and young infant (n = 5). The indications for operation were ventilator dependence, severe cardiac failure, prostaglandin-dependent circulation, and gross cardiomegaly. RESULTS: Between 1994 and 2006, 27 consecutive patients with Ebstein's anomaly underwent operation. Associated comorbidities included anatomic or functional pulmonary atresia (n = 18), ventricular septal defects (n = 3), small left ventricle (n = 3), hypoplastic branch pulmonary arteries (n = 3), previous cardiac surgery (n = 4), significant intracranial hemorrhage (n = 3), hepatic necrosis and renal insufficiency (n = 3), and malignant tachyarrhythmias (n = 4). Operations consisted of tricuspid valve repair (n = 23) or valve replacement (n = 2), Blalock-Taussig shunt only (n = 1), and bilateral pulmonary arterioplasty with bidirectional Glenn (n = 1). Hospital survival was 74%, and there have been no late deaths during a median follow-up period of 5.4 years (range, 0.2 to 12 years). Three patients required tricuspid valve replacement during the follow-up period. Late arrhythmia requiring medication is present in 1 patient. All patients are currently in New York Heart Association functional class I. CONCLUSIONS: Two-ventricle repair currently has similar early survival compared with single-ventricle palliation. The advantages of a better physiologic repair can be anticipated for a longer follow-up period.

Simplified aortic cannulation (SAC) - a useful technique for neonates with small aortas.

A simplified means of arterial cannulation for cardiopulmonary bypass in small neonates and those infants with diminutive aortas, or requiring reconstruction of the ascending aorta, is presented. It involves suturing a long 3.5 mm graft to the innominate artery and inserting the arterial cannula into the end of the graft. This technique improves exposure, thereby greatly simplifying many complex repairs, and may be used for initiation of ECMO or for hybrid procedures in the postoperative period.

Repair of Ebstein's anomaly in the symptomatic neonate: an evolution of technique with 7-year follow-up.

BACKGROUND: Ebstein's anomaly in the severely symptomatic neonate is usually fatal. Until recently, successful repair has not been reported and various palliative operations have been associated with prohibitive mortality. Recently, we published our initial results with biventricular repair in 3 severely symptomatic neonates. We now update our experience with emphasis on the evolution of our surgical technique and the medium-term follow-up of these patients. METHODS: Since 1994, 8 severely symptomatic neonates and young infants underwent biventricular repair by one surgeon. Six had Ebstein's anomaly and 2 had physiologically similar pathology with severe tricuspid valve dysplasia, cyanosis, and gross cardiomegaly. One Ebstein patient (2 months old) had undergone a Starnes operation elsewhere. Weight of the patients at operation ranged from 2.1 to 6.4 kg (mean 2.7 kg). Five patients had either anatomical (n = 3) or functional (n = 2) pulmonary atresia. Severe (4/4) tricuspid regurgitation was present in all except 1 (Starnes operation), and cardiothoracic ratio exceeded 0.85 in all patients. Echocardiography severity scores were >1.5 in 6 (grade 4/4) and 1.3 in 1 (grade 3/4). Repair consisted of tricuspid valve repair, reduction atrioplasty, relief of right ventricular outflow tract obstruction, partial closure of atrial septal defect, and correction of all associated cardiac defects. Technique of tricuspid valve repair evolved over time: 3 had Danielson-type repairs, 3 had DeVega-type repairs, and 2 had complex repairs. RESULTS: One patient died in hospital: a 2.1 kg patient with tricuspid dysplasia, anatomical pulmonary atresia, and hypoplastic pulmonary arteries. The other 7 patients are all in functional class I and in sinus rhythm. Although 3 patients had symptomatic tachyarrhythmias before surgery, no child has experienced SVT after discharge. At recent echocardiography 4 patients had mild tricuspid regurgitation, and 2 had mild-moderate (2/4) tricuspid regurgitation. Three patients are now 7 years old, 2 are almost 2 years old, and the remaining 2 patients are 1 year old. CONCLUSIONS: Surgical repair of the severely symptomatic neonate with Ebstein's anomaly is feasible and safe. The repair appears durable and with good medium-term outcome.