title: Follow-up study of pulmonary artery configuration in hypoplastic left heart syndrome.
abstract: OBJECTIVE
  Pulmonary artery (PA) distortion significantly compromises the outcome of the staged approach to the Fontan operation in patients with hypoplastic left heart syndrome (HLHS). This retrospective study was designed to investigate the influence of the initial operation on postoperative PA anatomy.
  
  
  METHODS
  Forty-nine patients with HLHS and its variant were enrolled in this study. As an initial palliation, the Norwood operation with a modified Blalock-Taussig (BT) shunt was performed in 12, the Norwood operation with a right ventricle to pulmonary artery (RV-PA) shunt in 31, and bilateral PA banding in 6. The incidence and risk factors of postoperative central pulmonary artery stenosis (PS) were investigated, and the PA configuration was followed up until post-Fontan status.
  
  
  RESULTS
  Twenty-two patients (51.2%) had developed central PS after the Norwood operation (33.3% with a BT shunt vs. 58.1% with a RV-PA shunt). The RV-PA shunt with a polytetrafluoroethylene (PTFE) patch at the distal pulmonary stump significantly decreased the central PS (P = 0.035). The PA index after the Norwood operation was not statistically different between the BT and RV-PA shunt groups, although in the RV-PA group it was significantly higher in patients with a PTFE patch on the distal PA stump. PA plasty was performed in 16 patients in the second-stage palliation and in 15 with the Fontan completion. Freedom from PA plasty was significantly lower in the RV-PA shunt group than in the BT shunt group (63.5% vs. 31.1% at 5 years, P = 0.034). Six patients initially palliated with bilateral PA banding had no stenosis at the banding site in the Norwood + Glenn operation, and one patient required stent placement for left PS in the Fontan completion. Post-Fontan catheterization (n = 31) showed central venous pressure of 11.5 +/- 2.6 mmHg, cardiac index of 3.6 +/- 0.8 l/kg/min, and PA index of 194.0 +/- 58.4 mm(2)/m(2); there was no difference between the groups.
  
  
  CONCLUSIONS
  The incidence of central PS after the Norwood operation was significant, and the shunt type and procedure for the distal PA stump influenced the postoperative configuration of the central PA. With an aggressive surgical approach to central PS, PA anatomy was satisfactory with good hemodynamic variables after Fontan completion. Bilateral PA banding did not cause later vascular deformity.
authors.affiliation: Department of Cardiovascular Surgery, Fukuoka Children's Hospital, 2-5-1 Tojin-machi, Chuo-ku, Fukuoka 810-0063, Japan. nakano.t@fukuoka-child.jp
authors.forename: Toshihide
authors.initials: T
authors.lastname: Nakano
authors.forename: Koji
authors.initials: K
authors.lastname: Fukae
authors.forename: Hiromichi
authors.initials: H
authors.lastname: Sonoda
authors.forename: Tsuyoshi
authors.initials: T
authors.lastname: Tachibana
authors.forename: Masaki
authors.initials: M
authors.lastname: Kajimoto
authors.forename: Yusuke
authors.initials: Y
authors.lastname: Ando
authors.forename: Hideaki
authors.initials: H
authors.lastname: Kado
mesh_terms: D000714:Anastomosis, Surgical / Q000009:adverse effects; D017130:Angioplasty / Q000009:adverse effects; D001157:Arterial Occlusive Diseases / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology / Q000601:surgery; D006348:Cardiac Surgical Procedures / Q000009:adverse effects* / Q000295:instrumentation; D003251:Constriction, Pathologic; D004867:Equipment Design; D005500:Follow-Up Studies; D018729:Fontan Procedure; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery*; D015994:Incidence; D007223:Infant; D010166:Palliative Care; D011138:Polytetrafluoroethylene; D011651:Pulmonary Artery / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery*; D011652:Pulmonary Circulation; D011859:Radiography; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome; D014656:Vascular Surgical Procedures / Q000009:adverse effects* / Q000295:instrumentation
chemical_list: D011138:Polytetrafluoroethylene