Fetal aortic valvuloplasty for evolving HLHS

Circulation 2014;130:638-45

Fetal Aortic Valvuloplasty for Evolving Hypoplastic Left Heart Syndrome

Postnatal Outcomes of the First 100 Patients

Linday R. Freud et al. from Boston

Background—Fetal aortic valvuloplasty can be performed for severe midgestation aortic stenosis in an attempt to prevent progression to hypoplastic left heart syndrome (HLHS). A subset of patients has achieved a biventricular (BV) circulation after fetal aortic valvuloplasty. The postnatal outcomes and survival of the BV patients, in comparison with those managed as HLHS, have not been reported.

Methods and Results—We included 100 patients who underwent fetal aortic valvuloplasty for severe midgestation aortic stenosis with evolving HLHS from March 2000 to January 2013. Patients were categorized based on postnatal management as BV or HLHS. Clinical records were reviewed. Eighty-eight fetuses were live-born, and 38 had a BV circulation (31 from birth, 7 converted after initial univentricular palliation). Left-sided structures, namely aortic and mitral valve sizes and left ventricular volume, were significantly larger in the BV group at the time of birth (P<0 .01="" 10="" 5.4="" 5="" 84="" 96="" a="" after="" all="" among="" and="" at="" better="" bv="" cardiac="" death="" em="" follow-up="" freedom="" from="" hlhs="" log-rank="" median="" of="" patients="" than="" was="" which="" years="">P

=0.04). There was no cardiac mortality in patients with a BV circulation from birth. All but 1 of the BV patients required postnatal intervention; 42% underwent aortic or mitral valve replacement. On the most recent echocardiogram, the median left ventricular end-diastolic volume z score was +1.7 (range, −1.3 to +8.2), and 80% had normal ejection fraction.

Conclusions—Short- and intermediate-term survival among patients who underwent fetal aortic valvuloplasty and achieved a BV circulation postnatally is encouraging. However, morbidity still exists, and ongoing assessment is warranted. 

Spectrum and outcome of primary cardiomyopathies diagnosed during fetal life

R Weber, ..., E. Jaeggi.
From Toronto group
JACC Heart Failure 2014;2(4):403-411

Echo, Fetal echo: Predicting need for balloon atrial septostomy in d-TGA

J Am Soc Echocardiogr. 2011 Apr;24(4):425-30. Epub 2011 Feb 15.

Fetal predictors of urgent balloon atrial septostomy in neonates with complete transposition.

Punn R, Silverman NH.

Source

Stanford University, Lucile Packard Children's Hospital, Palo Alto, California, USA.

Abstract

BACKGROUND:

In complete transposition of the great vessels, a restrictive patent foramen ovale leads to inadequate circulatory mixing and severe cyanosis. Urgent balloon atrialseptostomy (BAS) improves mixing and bridges neonates to surgery. Several studies have determined risk factors in utero for poor postnatal outcomes in complete transposition of the great vessels, particularly a restrictive patent foramen ovale and ductus arteriosus. In addition to these risk factors, we studied two new features, a hypermobile septum and reverse diastolic patent ductus arteriosus shunt, to determine which patients will require an urgent BAS.

METHODS:

We reviewed all 26 fetuses from 2001 to 2010 with complete transposition of the great vessels and closely examined the patent foramen ovale and septum primum for hypermobility, restriction, flat appearance, or redundancy. We defined hypermobility as a septum primum flap that oscillates between both atria. We also examined the ductus size and shunting pattern to evaluate whether these features contributed to urgent BAS.

RESULTS:

In total, 14 of 26 fetuses required urgent BAS with improved cyanosis. Nine fetuses had an urgent BAS and a hypermobile septum, and 12 fetuses had no urgent BAS or hypermobile septum. Eight fetuses had an urgent BAS and a reverse diastolic patent ductus arteriosus, and 11 fetuses had no urgent BAS or reverse diastolic patent ductus arteriosus. A hypermobile septum and reverse diastolic patent ductus arteriosus had a significant association with urgent BAS (P < .01, sensitivity = 0.64 and 0.57, specificity = 1.0 and 0.92, positive predictive value = 1.0 and 0.89, negative predictive value = 0.71 and 0.65). No fetus had a restrictive patent foramen ovale/ductus arteriosus.

CONCLUSION:

A hypermobile septum and reverse diastolic patent ductus arteriosus are new prenatal findings to help predict the need for an urgent BAS postnatally in patients with complete transposition of the great vessels.

Fetal AS with MR

Pathophysiology, Fetal intervention and Outcome

JACC 2011;57:348-355

Congenital Heart Block - Maternal Lupus

Dexamethasone therapy & Outcome
Friedman, D. et al. PRIDE study group.
Circulation 2008;117:485-93.


http://docs.google.com/fileview?id=0B5SNr50OQN-XZDczMWY5NmYtNTYwMi00NGZhLWExZTItYTkyNjM3Y2UxNThj&hl=en

Maternal antibody titre is an important predictor of congenital heart block.
Jaeggi, E. et al. JACC 2010;55:2778-84

http://docs.google.com/fileview?id=0B5SNr50OQN-XNTZmZmYxMDktMDY5OS00MzM1LWJkZGQtNzEzODg3MjgyNmVi&hl=en