Journal Article

Absent pulmonary valve syndrome

Martin A. Nørgaard, Nelson Alphonso, Andrew E. Newcomb, Christian P. Brizard and Andrew D. Cochrane

in European Journal of Cardio-Thoracic Surgery

Published on behalf of European Association for Cardio-Thoracic Surgery

Volume 29, issue 5, pages 682-687
Published in print May 2006 | ISSN: 1010-7940
Published online May 2006 | e-ISSN: 1873-734X | DOI:
Absent pulmonary valve syndrome

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  • Clinical Genetics
  • Professional Development in Medicine
  • Cardiothoracic Surgery
  • Cardiovascular Medicine


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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.

Keywords: Absent; Pulmonary; Valve; Syndrome; Congenital

Journal Article.  3782 words.  Illustrated.

Subjects: Clinical Genetics ; Professional Development in Medicine ; Cardiothoracic Surgery ; Cardiovascular Medicine

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