Korean J Thorac Cardiovasc Surg.  2005 Dec;38(12):815-820.

Mid-term Results of Neonatal Surgical Management of Pulmonary Atresia with Intact Ventricular Septum

• Kwak JG
• Kim WH
• Kim DJ
• Lee CH
• Lee JR
• Kim YJ
• Rho JR

Abstract

BACKGROUND: Pulmonary atresia with intact ventricular septum is a rare congenital cardiac disease. There have been various surgical options, but there are still controversies. MATERIAL AND METHOD: Fourteen neonates who were operated on between 1999 and 2000 were enrolled in this study. We measured Z-value of tricuspid valve by echocardiography. We performed right ventricular outflow tract transannular patch in 9 patients. Modified Blalock-Taussig shunt was placed according to the condition of patients. Mean follow up period was 46.0 months. RESULT: There were 2 operative mortality and 1 late mortality. Biventricular repair was possible in 7 patients. One and a half ventricular repair were done in 3 (tricuspid valve Z-value was -2, -2.5 and -3) and single ventricular repair in 2 patients (tricuspid z-value was -4.6, -4.5) The tricuspid valve Z-value for the patients who had biventricular repair and one and a half ventricular repair were -0.8+/-1.50 (-3.2~1.2) and -2.5+/-0.5 (-3~-2) respectively. All patients who survived had fair to good right ventricular function, good left ventricular function and good clinical states.
CONCLUSION
Neonatal surgical management of pulmonary atresia with intact ventricular septum has a good surgical outcome, z-value of tricuspid may be helpful for the determination of surgical options.

Keyword

Congenital heart disease; Pulmonary atresia; Tricuspid valve

MeSH Terms

Blalock-Taussig Procedure
Echocardiography
Follow-Up Studies
Heart Defects, Congenital
Heart Diseases
Humans
Infant, Newborn
Mortality
Pulmonary Atresia*
Tricuspid Valve
Ventricular Function, Left
Ventricular Function, Right
Ventricular Septum*