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Outcomes following surgical repair of absent pulmonary valve syndrome : 30 years of experience from a Swedish tertiary referral centre

Avdikos, Vasileios LU ; Johansson Ramgren, Jens LU ; Hanséus, Katarina LU ; Malm, Torsten LU and Liuba, Petru LU (2022) In Interactive Cardiovascular and Thoracic Surgery 35(2).
Abstract

Surgical approach with reduction pulmonary artery plasty and valved conduit in patients with respiratory compromise prior to repair is associated with excellent long-term survival at the cost of a higher reintervention rate. OBJECTIVES: Absent pulmonary valve syndrome is a rare congenital heart defect with pulmonary artery dilatation and secondary airway compression. Although preoperative respiratory support and early surgical repair with pulmonary arterioplasty are often required in patients with airway compromise, the need for extensive plasty in these patients and for plasty in general in those with no or mild respiratory issues remains debatable. METHODS: We performed a retrospective survey of patients with this diagnosis and repair... (More)

Surgical approach with reduction pulmonary artery plasty and valved conduit in patients with respiratory compromise prior to repair is associated with excellent long-term survival at the cost of a higher reintervention rate. OBJECTIVES: Absent pulmonary valve syndrome is a rare congenital heart defect with pulmonary artery dilatation and secondary airway compression. Although preoperative respiratory support and early surgical repair with pulmonary arterioplasty are often required in patients with airway compromise, the need for extensive plasty in these patients and for plasty in general in those with no or mild respiratory issues remains debatable. METHODS: We performed a retrospective survey of patients with this diagnosis and repair from 1988 to 2018. RESULTS: Twenty patients were identified. The median age and weight at repair were 0.8 (0.1-2.4) years and 7.0 (2.5-13.8) kg and included a valved conduit in 17 (85%) patients and a transannular patch in 3 patients. Five (29%) patients were ventilator-dependent prior to repair at the age of 0.3 (0.1-0.4) years. Pulmonary arterioplasty was performed in 7 patients (35%), including all 5 with ventilator dependency and 2 with respiratory symptoms due to recurrent infections. Two patients (10%) with preoperative ventilator dependency underwent extensive intrahilar arterioplasty. Preoperative ventilator dependency was associated with earlier repair and reinterventions (P < 0.05). There were 3 late deaths among cases with repair after 2000 (n = 14), none with preoperative ventilator dependency. CONCLUSIONS: The long-term outcomes of patients with this rare defect are good, comparable to those of other previous studies. Reduction pulmonary arterioplasty, which in this study was used only in patients with respiratory distress and ventilator dependency, is associated with excellent survival. Reinterventions are common in these patients.

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author
; ; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Absent pulmonary valve syndrome, Outcome, Respiratory distress, Surgical repair
in
Interactive Cardiovascular and Thoracic Surgery
volume
35
issue
2
article number
ivac193
publisher
European Association of Cardio-Thoracic Surgery
external identifiers
  • scopus:85135500040
  • pmid:35900146
ISSN
1569-9293
DOI
10.1093/icvts/ivac193
language
English
LU publication?
yes
id
ec946ff9-f44a-4f74-8890-35dae5924c16
date added to LUP
2022-09-20 14:45:50
date last changed
2024-05-30 20:10:58
@article{ec946ff9-f44a-4f74-8890-35dae5924c16,
  abstract     = {{<p>Surgical approach with reduction pulmonary artery plasty and valved conduit in patients with respiratory compromise prior to repair is associated with excellent long-term survival at the cost of a higher reintervention rate. OBJECTIVES: Absent pulmonary valve syndrome is a rare congenital heart defect with pulmonary artery dilatation and secondary airway compression. Although preoperative respiratory support and early surgical repair with pulmonary arterioplasty are often required in patients with airway compromise, the need for extensive plasty in these patients and for plasty in general in those with no or mild respiratory issues remains debatable. METHODS: We performed a retrospective survey of patients with this diagnosis and repair from 1988 to 2018. RESULTS: Twenty patients were identified. The median age and weight at repair were 0.8 (0.1-2.4) years and 7.0 (2.5-13.8) kg and included a valved conduit in 17 (85%) patients and a transannular patch in 3 patients. Five (29%) patients were ventilator-dependent prior to repair at the age of 0.3 (0.1-0.4) years. Pulmonary arterioplasty was performed in 7 patients (35%), including all 5 with ventilator dependency and 2 with respiratory symptoms due to recurrent infections. Two patients (10%) with preoperative ventilator dependency underwent extensive intrahilar arterioplasty. Preoperative ventilator dependency was associated with earlier repair and reinterventions (P &lt; 0.05). There were 3 late deaths among cases with repair after 2000 (n = 14), none with preoperative ventilator dependency. CONCLUSIONS: The long-term outcomes of patients with this rare defect are good, comparable to those of other previous studies. Reduction pulmonary arterioplasty, which in this study was used only in patients with respiratory distress and ventilator dependency, is associated with excellent survival. Reinterventions are common in these patients. </p>}},
  author       = {{Avdikos, Vasileios and Johansson Ramgren, Jens and Hanséus, Katarina and Malm, Torsten and Liuba, Petru}},
  issn         = {{1569-9293}},
  keywords     = {{Absent pulmonary valve syndrome; Outcome; Respiratory distress; Surgical repair}},
  language     = {{eng}},
  number       = {{2}},
  publisher    = {{European Association of Cardio-Thoracic Surgery}},
  series       = {{Interactive Cardiovascular and Thoracic Surgery}},
  title        = {{Outcomes following surgical repair of absent pulmonary valve syndrome : 30 years of experience from a Swedish tertiary referral centre}},
  url          = {{http://dx.doi.org/10.1093/icvts/ivac193}},
  doi          = {{10.1093/icvts/ivac193}},
  volume       = {{35}},
  year         = {{2022}},
}