In utero valvuloplasty for pulmonary atresia with hypoplastic right ventricle: techniques and outcomes.
BACKGROUND
Prenatal intervention for fetuses with pulmonary atresia with an intact ventricular septum (PA/IVS) has the potential to alter right heart physiologic features in utero, facilitating right heart growth and improving the prospect of a biventricular outcome after birth.


METHODS
Since 2002, we have considered prenatal intervention for fetal PA/IVS in patients with (1) membranous pulmonary atresia, with identifiable pulmonary valve (PV) leaflets or membrane; (2) an intact or highly restrictive ventricular septum; and (3) right heart hypoplasia, with a tricuspid valve annulus z score of -2 or below and an identifiable but small right ventricle. Intervention was performed through direct cardiac puncture under ultrasound guidance, with percutaneous access or access through a limited laparotomy.


RESULTS
Ten fetuses underwent attempted balloon dilation of the PV in utero. The first 4 procedures were technically unsuccessful, and the most-recent 6 were technically successful. Compared with control fetuses with PA/IVS who did not undergo prenatal intervention and had univentricular outcomes after birth, the tricuspid valve annulus, right ventricle length, and PV annulus grew significantly more from midgestation to late gestation in the 6 fetuses who underwent successful interventions.


CONCLUSIONS
In utero perforation and dilation of the PV in midgestation fetuses with PA/IVS is technically feasible and may be associated with improved right heart growth and postnatal outcomes for fetuses with moderate right heart hypoplasia in midgestation. There is an important learning curve for this procedure, and much remains to be learned about the selection of appropriate fetuses for prenatal intervention.
Tworetzky
Wayne
W
Department of Cardiology, Children's Hospital, Boston, MA 02115, USA. wayne.tworetzky@cardio.chboston.org
McElhinney
Doff B
DB
Marx
Gerald R
GR
Benson
Carol B
CB
Brusseau
Roland
R
Morash
Donna
D
Wilkins-Haug
Louise E
LE
Lock
James E
JE
Marshall
Audrey C
AC
D002404:Catheterization / Q000379:methods*; D005315:Fetal Diseases / Q000628:therapy*; D006330:Heart Defects, Congenital / Q000150:complications*; D006352:Heart Ventricles / Q000002:abnormalities*; D006801:Humans; D018633:Pulmonary Atresia / Q000150:complications* / Q000628:therapy*; D016896:Treatment Outcome
Ultrasound Obstet Gynecol. 2003 Feb;21(2):186-8
12601844
Pediatr Res. 2007 Sep;62(3):325-30
17622948
Ultrasound Obstet Gynecol. 2007 Oct;30(5):715-20
17764106
Fetal Diagn Ther. 2006;21(1):100-4
16354986
J Thorac Cardiovasc Surg. 1993 Mar;105(3):406-23, 424-7; discussion 423-4
8445920
J Am Soc Echocardiogr. 2006 Nov;19(11):1393-400
17098143
J Pediatr. 2005 Oct;147(4):535-9
16227042
Circulation. 2004 Oct 12;110(15):2125-31
15466631
Heart. 1999 Jun;81(6):661-8
10336930
Circulation. 1998 Aug 11;98(6):562-6
9714114
Circulation. 2004 Jul 20;110(3):253-8
15226215
J Thorac Cardiovasc Surg. 2004 Apr;127(4):1000-7; discussion 1007-8
15052196
Ann Thorac Surg. 2006 Jun;81(6):2250-7; discussion 2258
16731162
Pediatrics. 2006 Aug;118(2):e415-20
16882782
Lancet. 2002 Nov 16;360(9345):1567-8
12443597
J Thorac Cardiovasc Surg. 1998 May;115(5):1055-62
9605075
J Thorac Cardiovasc Surg. 1999 Dec;118(6):1046-55
10595977