@Article{chd.12658,
AUTHOR = {Robin H.S. Chen, Adolphus K.T. Chau, Pak Cheong Chow, Tak Cheung Yung, Yiu Fai Cheung, Kin Shing Lun},
TITLE = {Achieving biventricular circulation in patients with moderate hypoplastic right ventricle in pulmonary atresia intact ventricular septum after transcatheter pulmonary valve perforation},
JOURNAL = {Structural and Congenital Heart Disease},
VOLUME = {13},
YEAR = {2018},
NUMBER = {6},
PAGES = {884--891},
URL = {http://www.techscience.com/schd/v13n6/39071},
ISSN = {3071-1738},
ABSTRACT = {<b>Objective:</b> Transcatheter valve perforation for pulmonary atresia intact ventricular 
septum is the standard of care for patients with mild right ventricular hypoplasia. 
However, its role in moderate right ventricular hypoplasia has been less well defined. 
We sought to report the long‐term outcome of patients with moderate hypoplastic 
right ventricle who had undergone the procedure.<br/>
<b>Design, Settings, and Patients:</b> We performed a retrospective analysis on patients 
who had undergone transcatheter pulmonary valve perforation from January 1996 
to January 2015 at our institution. The procedures would be carried out irrespective 
of the right ventricular size, as long as there were no absolute contraindications.
Intervention and Outcome Measures: Demographic and procedural data were cor‐
related with outcome measures. Outcomes analyzed included procedural success, 
reintervention rates, final circulation type, and functional class. Multivariate analysis 
and receiver operator curve were used to identify for parameters in predicting biven‐
tricular circulation.<br/>
<b>Results:</b> The procedural success rate was 92% (33 out of 36) in this group with mod‐
erate right ventricular hypoplasia (tricuspid valve z score −4.2 ± 3.0, 69.4% of pa‐
tients with z score <−2.5). Early reintervention rate was 39%, mostly being insertion 
of modified Blalock–Taussig shunt. Overall reintervention‐free survival was 53%, 
30%, and 19% at 1, 6, and 12 months postintervention. Despite no significant catch‐
up right ventricular growth, majority of survivors (84%) enjoyed a biventricular circu‐
lation with good functional status. A tricuspid to mitral valve ratio >0.79 was a good 
predictor of biventricular outcome. (specificity of 100%, positive predictive value 
100%).<br/>
<b>Conclusion:</b> Encouraging long‐term results with biventricular circulation and func‐
tional status were demonstrated with transcatheter pulmonary valve perforation in 
patients even with moderate hypoplastic right ventricle, which is comparable to that 
with mild right ventricular hypertrophy. The baseline tricuspid to mitral valve ratio 
was identified as a potentially useful tool in predicting biventricular circulation.},
DOI = {10.1111/chd.12658}
}