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Absent pulmonary valve syndrome : operation in infants with airway obstruction

Watterson, K G ; Malm, T K LU ; Karl, T R and Mee, R B (1992) In Annals of Thoracic Surgery 54(6). p.9-1116
Abstract

From 1979 through 1991, 19 infants with absent pulmonary valve syndrome and airway obstruction were seen for surgical treatment. All patients underwent extensive pulmonary artery aneurysmorrhaphy using cardiopulmonary bypass. Fourteen patients had simultaneous transatrial ventricular septal defect (VSD) closure, infundibular resection, and placement of a short transannular patch; 2 had transventricular VSD closure and infundibular resection without a transannular patch; 1 underwent transventricular VSD closure and transannular patching; and 2 underwent pulmonary artery aneurysmorrhaphy alone with the VSD left open. All 19 infants had good hemodynamics when taken from the operating theater, but 3 died postoperatively of severe airway... (More)

From 1979 through 1991, 19 infants with absent pulmonary valve syndrome and airway obstruction were seen for surgical treatment. All patients underwent extensive pulmonary artery aneurysmorrhaphy using cardiopulmonary bypass. Fourteen patients had simultaneous transatrial ventricular septal defect (VSD) closure, infundibular resection, and placement of a short transannular patch; 2 had transventricular VSD closure and infundibular resection without a transannular patch; 1 underwent transventricular VSD closure and transannular patching; and 2 underwent pulmonary artery aneurysmorrhaphy alone with the VSD left open. All 19 infants had good hemodynamics when taken from the operating theater, but 3 died postoperatively of severe airway obstruction, despite further tracheobronchopexy procedures in 2 (hospital mortality rate, 16%; confidence limits, 7% to 29%). Among the 16 patients discharged from the hospital, there was one late death. Five other patients have required reoperation for branch pulmonary artery stenosis (n = 2), residual airway obstruction resulting from persistent pulmonary artery dilatation (n = 1), closure of VSD (n = 1), and homograft valve insertion for pulmonary incompetence and right ventricular dysfunction (n = 1). There are 15 long-term survivors. Eight of them have episodic bronchospasm of mild to moderate severity, and all are responsive to sympathomimetic bronchodilator aerosols. The remaining 7 are asymptomatic.

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author
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Airway Obstruction/complications, Cardiac Surgical Procedures/methods, Cardiopulmonary Bypass/methods, Congenital Abnormalities/mortality, Female, Follow-Up Studies, Hospital Mortality, Hospitals, Pediatric, Humans, Infant, Infant, Newborn, Male, Pulmonary Valve/abnormalities, Reoperation/statistics & numerical data, Survival Rate, Treatment Outcome, Victoria/epidemiology
in
Annals of Thoracic Surgery
volume
54
issue
6
pages
4 pages
publisher
Elsevier
external identifiers
  • pmid:1449295
  • scopus:0026621013
ISSN
0003-4975
DOI
10.1016/0003-4975(92)90078-I
language
English
LU publication?
no
id
aae90219-f2fb-4a01-adf0-d5e1dd50a01e
date added to LUP
2018-12-05 15:30:56
date last changed
2019-10-06 04:35:39
@article{aae90219-f2fb-4a01-adf0-d5e1dd50a01e,
  abstract     = {<p>From 1979 through 1991, 19 infants with absent pulmonary valve syndrome and airway obstruction were seen for surgical treatment. All patients underwent extensive pulmonary artery aneurysmorrhaphy using cardiopulmonary bypass. Fourteen patients had simultaneous transatrial ventricular septal defect (VSD) closure, infundibular resection, and placement of a short transannular patch; 2 had transventricular VSD closure and infundibular resection without a transannular patch; 1 underwent transventricular VSD closure and transannular patching; and 2 underwent pulmonary artery aneurysmorrhaphy alone with the VSD left open. All 19 infants had good hemodynamics when taken from the operating theater, but 3 died postoperatively of severe airway obstruction, despite further tracheobronchopexy procedures in 2 (hospital mortality rate, 16%; confidence limits, 7% to 29%). Among the 16 patients discharged from the hospital, there was one late death. Five other patients have required reoperation for branch pulmonary artery stenosis (n = 2), residual airway obstruction resulting from persistent pulmonary artery dilatation (n = 1), closure of VSD (n = 1), and homograft valve insertion for pulmonary incompetence and right ventricular dysfunction (n = 1). There are 15 long-term survivors. Eight of them have episodic bronchospasm of mild to moderate severity, and all are responsive to sympathomimetic bronchodilator aerosols. The remaining 7 are asymptomatic.</p>},
  author       = {Watterson, K G and Malm, T K and Karl, T R and Mee, R B},
  issn         = {0003-4975},
  language     = {eng},
  number       = {6},
  pages        = {9--1116},
  publisher    = {Elsevier},
  series       = {Annals of Thoracic Surgery},
  title        = {Absent pulmonary valve syndrome : operation in infants with airway obstruction},
  url          = {http://dx.doi.org/10.1016/0003-4975(92)90078-I},
  doi          = {10.1016/0003-4975(92)90078-I},
  volume       = {54},
  year         = {1992},
}