title: Late clinical features of patients with pulmonary atresia or critical pulmonary stenosis with intact ventricular septum after biventricular repair.
abstract: BACKGROUND
  We aimed to reveal late clinical features of patients with pulmonary atresia with intact ventricular septum (PA/IVS) or critical pulmonary stenosis (cPS) after biventricular repair (BVR) based on preoperative right ventricular (RV) end-diastolic volume (RVEDV) findings.
  
  
  METHODS
  Since 1985, 23 of 73 patients with PA/IVS (n=22) or cPS (n=1) with a tripartite RV and without major sinusoidal communication underwent BVR with a hybrid approach. The mean age and weight at BVR were 1.4±2.1 years and 6.9±5.9 kg, respectively. Mean follow-up was 10.1±6.4 years (range, 1.1 to 24.6 years).
  
  
  RESULTS
  Overall survival, reintervention-free, and arrhythmia-free rates at 20 years were 90.6%, 75.4%, and 50.4%, respectively. In 19 patients with preoperative RVEDV of 60% to 120% of normal, echocardiography at 10 years after BVR showed well-maintained RV systolic function. However, RV volume was quantitatively dilated in 16 (88.9%) due to moderate or greater tricuspid regurgitation in 8 (44.4%), pulmonary regurgitation in 12 (66.7%), or both, which caused arrhythmia in 3 patients more than 10 years after BVR. Two patients with preoperative RVEDV of greater than 120% of normal required tricuspid valve replacement after BVR, after which refractory atrial tachyarrhythmia developed in both patients. Furthermore, 2 patients with preoperative RVEDV of less than 60% of normal showed a cardiac index value within 2.5 L/min/m2 at 1 year after BVR, which did not improve.
  
  
  CONCLUSIONS
  Patients with PA/IVS or cPS and adequately sized RV showed good late clinical features after BVR. However, long-term follow-up examinations are necessary for RV dilatation and late-onset arrhythmia.
authors: Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan. thoashi@surg1.med.osaka-u.ac.jp
authors: Takaya
authors: T
authors: Hoashi
authors: Koji
authors: K
authors: Kagisaki
authors: Masataka
authors: M
authors: Kitano
authors: Kenichi
authors: K
authors: Kurosaki
authors: Isao
authors: I
authors: Shiraishi
authors: Toshikatsu
authors: T
authors: Yagihara
authors: Hajime
authors: H
authors: Ichikawa
mesh_terms: D000293:Adolescent; D000367:Age Factors; D006328:Cardiac Catheterization / Q000379:methods; D006348:Cardiac Surgical Procedures / Q000379:methods* / Q000401:mortality; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006352:Heart Ventricles / Q000601:surgery*; D017052:Hospital Mortality; D006801:Humans; D007223:Infant; D008297:Male; D008991:Monitoring, Physiologic / Q000379:methods; D018633:Pulmonary Atresia / Q000150:complications / Q000401:mortality / Q000601:surgery*; D011666:Pulmonary Valve Stenosis / Q000150:complications / Q000401:mortality / Q000601:surgery*; D012189:Retrospective Studies; D018570:Risk Assessment; D012737:Sex Factors; D016019:Survival Analysis; D017741:Survivors; D016896:Treatment Outcome; D016278:Ventricular Function, Right / Q000502:physiology; D054088:Ventricular Septum / Q000502:physiology; D055815:Young Adult