title: Evidence for palliative enlargement of the right ventricular outflow tract in severe tetralogy of Fallot.
abstract: OBJECTIVE
  If the pulmonary artery (PA) tree in patients with Fallot's tetralogy (TOF) is extremely hypoplastic, a shunt procedure may be difficult and not desirable because of side-effects. Moreover, the failing catch-up growth of the pulmonary annulus is well known. In patients with a severe form of TOF, we performed palliative transannular patching of the right ventricular outflow tract. The early and long-term follow-up was evaluated.
  
  
  METHODS
  Eleven patients (93 days (10-245 days); 3.5+/-0.7 kg (2.5-4.3 kg)) had highly symptomatic TOF (Hb: 18+/-2g/dl, SO2: 68+/-11%); angiographic diameters: RPA: 4.1 mm (2.5-6.4 mm), LPA: 3.4 mm (1.6-7.0 mm), PA trunc: 4.4 mm (2.5-7.0 mm). All 11 underwent transannular enlargement of the right ventricular outflow tract without closure of the ventricular septum defect. A PA index (cross-sectional area of the pulmonary arteries to BSA) was used to compare pre- and postoperative data. For follow-up, the patients were repetitively examined clinically and echocardiographically.
  
  
  RESULTS
  Preoperative PA index was 87+/-40 mm2/m2 (normal: 330+/-35 mm2/m2). Postpalliation angiograms (age: 10-14 months) demonstrated a significant catch-up growth in nine patients (PA index from 99+/-40 to 310+/-54 mm2/m2) and inadequate growth in two patients (PA index 63 and 115 mm2/m2). Perioperative mortality was zero. Ten patients (43 months; 6-105 months) underwent elective repair. Six patients received pulmonary homograft valves (6-15 years after repair) because of severe pulmonary valve insufficiency and severe RV dilation.
  
  
  RESULTS
  One patient died 10 months postpalliation due to pneumonia, one patient received a pacemaker after repair and died (2 months post-repair) due to pacemaker failure, a 5-year-old patient died 1 month after repair due to sepsis. All eight long-term survivors (12-17 years) are in excellent clinical condition. Echocardiography revealed good RV function and near normal diameters at peak systolic pressures between 25 and 50 mmHg. Only one patient developed brady-arrhythmia; a pacemaker was implanted 8 years after repair and 2 years after homograft implantation.
  
  
  CONCLUSIONS
  In a very severe form of TOF, palliative right ventricular outflow tract construction may provide the potential for complete repair. In the presented high-risk patient group, mortality was not related to the hypoplastic pulmonary arteries. Obviously, all patients need pulmonary valve implantation in the long run.
authors.affiliation: Department of Thoracic- and Cardiovascular Surgery, University Hospital Duesseldorf, Duesseldorf, Germany.
authors.forename: Bernhard
authors.initials: B
authors.lastname: Korbmacher
authors.forename: Andreas
authors.initials: A
authors.lastname: Heusch
authors.forename: Ulrich
authors.initials: U
authors.lastname: Sunderdiek
authors.forename: Emmeran
authors.initials: E
authors.lastname: Gams
authors.forename: Spiros
authors.initials: S
authors.lastname: Rammos
authors.forename: Mike R
authors.initials: MR
authors.lastname: Langenbach
authors.forename: Jochen D
authors.initials: JD
authors.lastname: Schipke
mesh_terms: D003251:Constriction, Pathologic; D004452:Echocardiography; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D010166:Palliative Care / Q000379:methods*; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery*; D011859:Radiography; D012086:Reoperation; D013771:Tetralogy of Fallot / Q000000981:diagnostic imaging / Q000601:surgery*; D016896:Treatment Outcome; D014694:Ventricular Outflow Obstruction / Q000601:surgery*