PMID- 16520058
OWN - NLM
STAT- MEDLINE
DCOM- 20060720
LR  - 20140729
IS  - 1010-7940 (Print)
IS  - 1010-7940 (Linking)
VI  - 29
IP  - 5
DP  - 2006 May
TI  - Absent pulmonary valve syndrome. Surgical and clinical outcome with long-term 
      follow-up.
PG  - 682-7
AB  - OBJECTIVE: To identify a large group of patients operated for absent pulmonary 
      valve syndrome (APVS), and describe the outcomes of various subgroups and 
      surgical strategies. METHODS: Thirty-six consecutive patients operated during 
      1979-2004 were included. Thirty-one percent furthermore had other vascular 
      abnormalities. Eighty-three percent had airway obstruction symptoms before 
      surgery. Twenty-eight percent were ventilator dependent, while 72% underwent 
      repair electively. The median age and weight at repair were 0.8 years (4 days-24 
      years) and 6.7 kg (1.8-56 kg). The surgical approach was modified several times, 
      including the following: homograft conduit (14%), monocusp valve (39%), and no 
      pulmonary valve (47%). All patients underwent ventricular septal defect (VSD) 
      patching. Seventy-four percent underwent right ventricular outflow tract 
      resection and 91% underwent transannular patching. Pulmonary artery reduction 
      plasty was performed in 86%. RESULTS: The follow-up was 94% complete. Nineteen 
      percent had died. The median follow-up time was 9.2 years (1 day-20.5 years). 
      Survival after repair was 82% at 1 year and 79% at 5, 10, and 15 years. 
      Postoperative survival was strongly associated to preoperative ventilator 
      dependency (p=0.002). The current New York Heart Association (NYHA) 
      classification was established in 93% of survivors. Eighty-nine percent were in 
      NYHA 1 or 2, 11% were in NYHA 3. No patient was in NYHA 4. Freedom from 
      reoperation 1, 5, and 10 years after repair was 78, 65, and 55%, respectively. 
      CONCLUSIONS: The surgical outcome of APVS was closely related to preoperative 
      ventilator dependency. Efforts to improve the surgical outcome should be focused 
      on identifying and correcting this.
FAU - Norgaard, Martin A
AU  - Norgaard MA
AD  - Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia.
FAU - Alphonso, Nelson
AU  - Alphonso N
FAU - Newcomb, Andrew E
AU  - Newcomb AE
FAU - Brizard, Christian P
AU  - Brizard CP
FAU - Cochrane, Andrew D
AU  - Cochrane AD
LA  - eng
PT  - Journal Article
DEP - 20060307
PL  - Germany
TA  - Eur J Cardiothorac Surg
JT  - European journal of cardio-thoracic surgery : official journal of the European 
      Association for Cardio-thoracic Surgery
JID - 8804069
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Child
MH  - Child, Preschool
MH  - Epidemiologic Methods
MH  - Female
MH  - Heart Arrest, Induced
MH  - Heart Septal Defects, Ventricular/surgery
MH  - Heart Valve Prosthesis Implantation
MH  - Humans
MH  - Infant
MH  - Infant, Newborn
MH  - Male
MH  - Prognosis
MH  - Pulmonary Artery/surgery
MH  - Pulmonary Valve/*abnormalities/*surgery
MH  - Reoperation
MH  - Respiration, Artificial
MH  - Treatment Outcome
MH  - Ventricular Outflow Obstruction/surgery
EDAT- 2006/03/08 09:00
MHDA- 2006/07/21 09:00
CRDT- 2006/03/08 09:00
PHST- 2005/11/02 00:00 [received]
PHST- 2006/01/22 00:00 [revised]
PHST- 2006/01/30 00:00 [accepted]
PHST- 2006/03/08 09:00 [pubmed]
PHST- 2006/07/21 09:00 [medline]
PHST- 2006/03/08 09:00 [entrez]
AID - S1010-7940(06)00127-8 [pii]
AID - 10.1016/j.ejcts.2006.01.050 [doi]
PST - ppublish
SO  - Eur J Cardiothorac Surg. 2006 May;29(5):682-7. doi: 10.1016/j.ejcts.2006.01.050. 
      Epub 2006 Mar 7.