[Absent pulmonary valve syndrome in tetralogy of Fallot: report of two cases].
A combination of absence of the pulmonary valve with ventricular septal defect, annular pulmonary stenosis and aneurysm of the pulmonary artery is called "absent pulmonary valve syndrome". Most patients with this condition suffered from severe respiratory symptoms in early infancy. Respiratory failure which is the leading cause of mortality in infants with this syndrome, results from pulmonary emphysema, pulmonary atelectasis or pulmonary infection as a consequence of compression of the bronchus by the pulmonary aneurysm. If these patients can be treated medically to survive beyond one year old, gradual decrease in severity of symptoms is expected. A definite correction in childhood is recommended. Otherwise, palliative operation in infancy has a high mortality rate. This paper reported two cases with severe symptoms in infancy; one patient expired at nine months of age, and the other lived beyond one year to receive surgical correction at the age of three. The etiology, clinical features and treatment of absent pulmonary valve syndrome are also discussed.
Yang
H Y
HY
Department of Pediatrics, National Taiwan University Hospital, Taipei, Republic of China.
Lue
H C
HC
Wang
J K
JK
D000015:Abnormalities, Multiple / Q000601:surgery*; D000783:Aneurysm; D005260:Female; D006345:Heart Septal Defects, Ventricular; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011651:Pulmonary Artery; D011664:Pulmonary Valve / Q000002:abnormalities*; D011666:Pulmonary Valve Stenosis; D013577:Syndrome; D013771:Tetralogy of Fallot / Q000601:surgery