Outcomes in neonates with pulmonary atresia and intact ventricular septum underwent pulmonary valvulotomy and valvuloplasty using a flexible 2-French radiofrequency catheter.
OBJECTIVE
Outcomes in 6 neonates with pulmonary atresia and intact ventricular septum (PAIVS) undergoing radiofrequency pulmonary valvulotomy and valvuloplasty (RPVV) were reported to identify the factors favorable for RPVV as the treatment of choice.


METHODS
From May 2000 to January 2008, 6 patients with PAIVS were included in this retrospective study. They were aged 1 day to 90 days old. Study modalities included review of recordings of presentations and profiles of chest radiography, electrocardiography, echocardiography, and cardiac catheterization with angiography. Hemodynamic profiles from the echocardiography and the cardiac catheterization were analyzed.


RESULTS
Echocardiography showed severe tricuspid regurgitation, membranous atresia of the pulmonary valve, intact ventricular septum, patent ductus arteriosus, and hypoplastic right ventricle in 6 patients. The pulmonary valve annulus were 4.2 to 6.9 mm in diameters, and those of the tricuspid valve were 7.1 to 10.1 mm. Elevated serum level of cardiac enzymes were found in 1 patient with ventriculocoronary communication (VCC). At cardiac catheterization, the ratio of systolic pressure of the right ventricle to that of the left ventricle ranged from 1.43 to 2.33 before RPVV, and from 0.54 to 1.15 after RPVV (p=0.027). The pressure gradients ranged from 76 to 136 mmHg before RPVV, and from 15 to 39 mmHg after RPVV (p=0.028). The echocardiographic gradients ranged from 16 to 32 mmHg within 24 hours after RPVV, and from 15 to 50 mmHg at the follow-ups.


CONCLUSIONS
RPVV can be a treatment of choice for neonates with PAIVS, if there is patent infundibulum, no right-ventricular dependent coronary circulation, and adequate tricuspid valve and pulmonary valve.
Division of Pediatric Cardiology, Department of Pediatrics, Changhua Christian Hospital, Changhua, Taiwan. ferdielee@yahoo.com
Meng-Luen
ML
Lee
Lon-Yen
LY
Tsao
Han-Yao
HY
Chiu
Ming
M
Chen
Ing-Sh
IS
Chiu
D006328:Cardiac Catheterization / Q000379:methods*; D002404:Catheterization / Q000379:methods*; D004452:Echocardiography; D005260:Female; D006439:Hemodynamics; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D018633:Pulmonary Atresia / Q000601:surgery*; D011664:Pulmonary Valve / Q000002:abnormalities / Q000601:surgery*; D016896:Treatment Outcome; D054088:Ventricular Septum