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Objective: The purpose of this study was to evaluate the effect of maternal nifedipine on fetal survival when started 24-48 hours before selective fetoscopic laser photocoagulation (SFLP)., Study Design: We conducted a case control study of consecutive cases of twin-twin transfusion syndrome (TTTS) in which TTTS cardiomyopathy was treated with maternal nifedipine 24-48 hours before SFLP, compared with gestational age and stage-matched control cases. The primary outcome was recipient and donor survival., Results: One hundred forty-one cases of TTTS were treated with nifedipine, and 152 gestational age- and stage-matched control cases were analyzed. There was a significant increase in overall fetal survival in nifedipine-treated cases compared with control cases (237/284 [83%] vs 232/308 [75%]; P = .015). There is an increase in survival of recipients who were treated with nifedipine in stage IIIA (100% vs 81%; P = .021) and IIIB (93% vs 71%; P = .014); however, there was no difference in donor survival., Conclusion: Maternal nifedipine is associated with improved recipient survival in TTTS that undergoes SFLP. This is the first study to suggest a benefit of adjunctive maternal medical therapy in patients with TTTS who undergo SFLP., (Copyright © 2010 Mosby, Inc. All rights reserved.)

Objective: We sought to evaluate amniotic fluid brain natriuretic peptide (BNP) levels as a biomarker of recipient twin (RT) cardiomyopathy (RTCM) in twin-twin transfusion syndrome., Study Design: Amniotic fluid samples were obtained from 157 twin-twin transfusion syndrome RTs and from 6 singletons (controls) from 2007 through 2009. N-terminal prohormone BNP (NT-proBNP) levels were quantified by enzyme-linked immunosorbent assay. RTCM was classified as mild (IIIA), moderate (IIIB), or severe (IIIC) by fetal echocardiography. The relationship between NT-proBNP and RTCM was evaluated using analysis of variance. The ability of NT-proBNP to predict moderate or greater RTCM was evaluated by receiver operating characteristic analysis., Results: There is a significant positive correlation between NT-proBNP levels and worsening RTCM (r = 0.33; P < .001). NT-proBNP thresholds of 569 fmol/mg and 369 fmol/mg had a sensitivity of 70% and 87%, and specificity of 67% and 42%, respectively, in predicting moderate or greater RTCM., Conclusion: This is the first large case series that demonstrates a relationship between NT-proBNP and RTCM. This pathophysiologic insight supports ongoing efforts to develop screening biomarkers., (Copyright © 2010 Mosby, Inc. All rights reserved.)

Objective: This study evaluated the acute effects of selective fetoscopic laser photocoagulation (SFLP) on recipient cardiovascular function in a twin-twin transfusion syndrome (TTTS) pregnancy., Study Design: This was a retrospective chart review of echocardiographic data in TTTS including right (RV) and left (LV) ventricular Doppler myocardial performance index (MPI); LV and RV end diastolic wall thickness; and umbilical artery (UA), vein (UV), and ductus venosus (DV) Dopplers. The primary outcome measures were improved MPI defined as greater than 10% interval decrease in left and/or right MPI. Data were analyzed by Student t test and Fisher's exact test., Results: Sixty-five patients met inclusion criteria. SFLP results in a significant improvement in UV and DV Doppler and an increase in both RV and LV wall thickness. A 10% or greater improvement in recipient LV MPI after SFLP is associated with improved recipient survival as compared with unimproved LV MPI (100% vs 86.1%, P = .05)., Conclusion: Improved recipient myocardial performance index after SFLP is associated with improvement in recipient survival.

Background: Mechanisms leading to left ventricular hypoplasia and endocardial fibroelastosis in the fetus remain unknown. Prevailing theory is that obstruction to blood flow through the left ventricle leads to elevated end-diastolic pressures, compromised myocardial perfusion, and endocardial ischemia. Fetal interventions are now being performed, based on the presumption that they would prevent such pathogenic mechanisms., Methods: Forty first-trimester fetal sheep (mean gestational age, 53 days) were studied. Severe fetal left ventricular outflow obstruction was created by banding the ascending aorta in 25 fetuses; 15 control fetuses underwent "sham" surgery with thoracotomy. Serial fetal echocardiography was used to assess left ventricular growth and fetal hemodynamics. Findings were correlated to morphologic and histopathologic changes, and intracardiac pressure measurements obtained from fetal cardiac catheterization., Results: Surviving banded fetuses (n = 13) had one of two phenotypes: compensatory left ventricular hypertrophy (n = 7) or noncompensatory left ventricular dilatation (n = 6) with hydrops and severe left ventricular dysfunction. All fetuses had elevated left ventricular end-diastolic pressures (mean, 21 mm Hg; range, 14 to 28 mm Hg), which correlated to the gradient across the ascending aorta (mean, 41 mm Hg; range, 28 to 73 mm Hg). In vivo echocardiography findings were incongruous with those at autopsy, and demonstrated preservation of left ventricular growth indices in all fetuses. Endocardial fibroelastosis and myocardial fibrosis were not observed in any banded fetus., Conclusions: While early gestational obstruction to flow can compromise left ventricular function in the fetus, it does not retard normal growth. Similarly, an elevated left ventricular end-diastolic pressure is not sufficient to cause myocardial fibrosis or endocardial fibroelastosis in the fetus.

A rare case of truncus arteriosus associated with mitral valve atresia, hypoplastic left ventricle, and intact ventricular septum is reported. Successful medical management and surgical palliation of this defect is described. The possible embryology, pertinent hemodynamics, and clinical concerns with this unusual case are discussed.

Background: Patients with univentricular hearts and ventriculoarterial discordance with potentially obstructed systemic blood flow continue to pose difficult management problems. The goals of neonatal palliative operations are to control pulmonary blood flow while avoiding pulmonary artery distortion, to relieve systemic outflow tract obstruction, and to avoid heart block., Methods: Between January 1987 and December 1996, 38 patients with either tricuspid atresia or a double-inlet left ventricle and ventriculoarterial discordance underwent a modified Norwood procedure. Their mean age was 15 days, and their mean weight was 3.4 kg. Aortic arch anomalies were present in 92% of the patients. Morbidity and mortality statistics, intraoperative data, and postoperative echocardiograms were reviewed., Results: There were 3 early deaths (7.8%) and 5 late deaths (13.1%). The actuarial survival rates at 1 month, 1 year, and 5 years were 89%, 82%, and 71%, respectively. Follow-up was complete in all children at a mean interval of 30 +/- 9 months. None of the patients had significant neoaortic valve insufficiency, and 1 patient required therapy for residual aortic arch obstruction. Nine patients (30% of the survivors) have undergone the hemi-Fontan procedure, and 18 patients (60%) successfully have undergone the Fontan procedure., Conclusions: In this patient population, we recommend the modified Norwood procedure as the neonatal palliative treatment of choice. It can be performed with acceptable early morbidity and mortality, and it improves suitability for the Fontan procedure. It reliably relieves all levels of systemic outflow tract obstruction, controls pulmonary blood flow, and avoids heart block.