TY - JOUR
T1 - Treatment of pulmonary atresia with intact ventricular septum in early infancy
AU - Hsieh, Wang Huu
AU - Hwang, Betau
AU - Lu, Jen Her
AU - Lee, Pi Chang
AU - Fu, Yun Ching
AU - Shing, Hsueh Ping
AU - Meng, C. C.Laura
PY - 1999/9
Y1 - 1999/9
N2 - Background. Early treatment for the neonate with pulmonary atresia and intact ventricular septum (PA-IVS) is important if a high mortality rate is to be avoided. The treatment includes prostaglandin administration, balloon atrial septostomy, pulmonary valvotomy, a shunting procedure and patch repair for the right ventricular outflow tract (RVOT). This study discusses the early treatment and risk factors of this disease that are essential for assessing the risk of surgery and predicting outcome. Methods. The medical records, echocardiograms, catheterization data and cineangiograms of 29 patients with PA-IVS diagnosed at our institution from 1987 to 1997 were reviewed retrospectively. Clinical manifestations including age, body weight, sex ratio, type of surgery and outcome were analyzed. Results. Three of four patients with a right ventricular volume of less than 1 ml died, and all patients with a right ventricular volume of greater than 2 ml survived. Four of six patients with a tricuspid valvular area of between 0.25 and 0.5 cm2 died, but patients with an area of greater than 0.5 cm2 survived. One case with a monopartite right ventricle died. Patients with a bipartite or tripartite right ventricle had higher survival rates than those with a monopartite right ventricle. Patients with a the right ventricle dependent coronary sinusoid had a higher risk for mortality, even after surgery, than those with normal coronary circulation. Conclusions. A small right ventricular volume, the presence of a coronary sinusoid involving the right ventricle, a short linear length of the tricuspid valve and a small area of the tricuspid valve were the risk factors associated with a negative outcome in neonates with PA-IVS.
AB - Background. Early treatment for the neonate with pulmonary atresia and intact ventricular septum (PA-IVS) is important if a high mortality rate is to be avoided. The treatment includes prostaglandin administration, balloon atrial septostomy, pulmonary valvotomy, a shunting procedure and patch repair for the right ventricular outflow tract (RVOT). This study discusses the early treatment and risk factors of this disease that are essential for assessing the risk of surgery and predicting outcome. Methods. The medical records, echocardiograms, catheterization data and cineangiograms of 29 patients with PA-IVS diagnosed at our institution from 1987 to 1997 were reviewed retrospectively. Clinical manifestations including age, body weight, sex ratio, type of surgery and outcome were analyzed. Results. Three of four patients with a right ventricular volume of less than 1 ml died, and all patients with a right ventricular volume of greater than 2 ml survived. Four of six patients with a tricuspid valvular area of between 0.25 and 0.5 cm2 died, but patients with an area of greater than 0.5 cm2 survived. One case with a monopartite right ventricle died. Patients with a bipartite or tripartite right ventricle had higher survival rates than those with a monopartite right ventricle. Patients with a the right ventricle dependent coronary sinusoid had a higher risk for mortality, even after surgery, than those with normal coronary circulation. Conclusions. A small right ventricular volume, the presence of a coronary sinusoid involving the right ventricle, a short linear length of the tricuspid valve and a small area of the tricuspid valve were the risk factors associated with a negative outcome in neonates with PA-IVS.
KW - Hypoplastic right ventricle
KW - Prostaglandin E
KW - Pulmonary atresia
UR - http://www.scopus.com/inward/record.url?scp=0032862669&partnerID=8YFLogxK
M3 - Article
C2 - 10502854
AN - SCOPUS:0032862669
SN - 0578-1337
VL - 62
SP - 626
EP - 632
JO - Chinese Medical Journal (Taipei)
JF - Chinese Medical Journal (Taipei)
IS - 9
ER -