Repair of neonates and young infants with Ebstein's anomaly and related disorders
| Autor: | James K. Kirklin, Edward D. Overholt, Steven P. Goldberg, Edward V. Colvin, Christopher J. Knott-Craig |
|---|---|
| Rok vydání: | 2007 |
| Předmět: |
Pulmonary and Respiratory Medicine
Thorax Heart Defects Congenital medicine.medical_specialty medicine.medical_treatment Necrosis Valve replacement Ebstein's anomaly medicine Humans Retrospective Studies business.industry Heart Septal Defects Patient Selection Respiratory disease Infant Newborn Infant Retrospective cohort study medicine.disease Respiration Artificial Survival Analysis Surgery Cardiac surgery Ebstein Anomaly medicine.anatomical_structure Treatment Outcome Liver Ventricle Tricuspid Valve Cardiology and Cardiovascular Medicine business Pulmonary atresia Intracranial Hemorrhages |
| Zdroj: | The Annals of thoracic surgery. 84(2) |
| ISSN: | 1552-6259 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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