Matthias W. Freund

Sotalol as first-line treatment for fetal tachycardia and neonatal follow-up

In fetal tachycardia, pharmacological therapy with digoxin, flecainide and sotalol has been reported to be effective. In a recent retrospective multicenter study, sotalol was considered to be less effective than the other drugs in treatment of fetal supraventricular tachycardia (SVT). The aim of this study was to re‐evaluate the efficacy and safety of maternally administered sotalol in the treatment of fetal tachycardia.

Comparability of Z-score equations of cardiac structures in hypoplastic left heart complex.

BACKGROUND Hypoplastic left heart complex (HLHC) is characterized by a mitral valve or an aortic valve annular Z score < -2, antegrade flow in the ascending aorta, ductal dependency, coarctation or aortic arch hypoplasia, and absence of significant (sub)valvar stenosis. The Z scores of the mitral and aortic valve annuli are major determinants of HLHC. Therefore, the algorithm for Z-score calculation is essential for diagnosis. However, no single universal method of calculation is in use. In the scientific literature addressing HLHC, various Z-score calculation methods have been applied. The aim of this study was to evaluate Z scores derived from two-dimensional echocardiographic dimensions in patients with HLHC. METHODS To compare the different published methods using two-dimensional echocardiographic measures for Z-score calculation, a cohort of 18 newborns diagnosed with HLHC was retrospectively evaluated. In addition, the methods to determine body surface area in newborns were evaluated. RESULTS Three Z-score calculation methods were included and compared. Using the method of Daubeney et al. to calculate Z scores in our cohort illustrated a lack of correlation beyond a Z score < 0, compared with the methods of Zilberman et al. and Pettersen et al. Z scores calculated using Zilberman et al.s and Pettersen et al.s methods were fairly consistent. The equations used by Pettersen et al. are based on the largest population of neonates. CONCLUSION Although the different methods for calculating Z scores for mitral and aortic valve dimensions correspond fairly well in the normal range, Z scores < -2 diverge substantially. A useful scientific comparison of published data and outcomes of patients with HLHC remains elusive. The Z-score calculation algorithms used by Pettersen et al. appear to be the most appropriate for use in an evaluation of HLHC. Because these different methods can yield different values, reporting the method as well as the Z score is essential for an accurate diagnosis. Similarly, the method used to determine body surface area should be reported.

Chiari’s Network as a Cause of Fetal and Neonatal Pathology

Chiari’s network is a remnant of the eustachian valve located in the right atrium. Incomplete involution of the fetal sinus venosus valves results in “redundant” Chiari’s network, which may compromise cardiovascular function. This report describes a case with the novel finding of prenatal compromise due to redundant Chiari’s network and an uncommon case with significant postnatal symptoms. In both cases, the symptoms (fetal hydrops and postnatal cyanosis) resolved spontaneously. The variety of cardiovascular pathologies described in the literature is believed to be associated with persistence of a Chiari network. Knowledge about this not always harmless structure is important for perinatologists, pediatricians, and pediatric cardiologists alike. The clinical importance of this rare pathology is that prenatal counseling may anticipate a generally positive outcome and that surgical intervention generally should be avoided.

Aortico-Right Ventricular Tunnel: Prenatal Diagnosis Leading to Neonatal Survival

In the 36th week of gestation a large aortico-right ventricular tunnel with an otherwise structurally normal heart was diagnosed by fetal echocardiography. This report describes for the first time the impact of the timely prenatal diagnosis of an aortico-right ventricular tunnel followed by successful management in early infancy.

Fetal Heart Disease: Severity, Associated Anomalies and Parental Decision

Objective: This study describes the association between the complexity of congenital cardiac and extracardiac malformations, and the parental decision of pregnancy continuation or termination. Methods: Congenital heart defects (CHD) was diagnosed by ultrasound in 251 fetuses before the 24th week (23 + 6 weeks) of gestation during the four year period from 2007 to 2010. All fetuses from the Utrecht region were referred to our center due to a strict referral pattern. The complexity and severity of the cardiac and possible extracardiac malformations were retrospectively categorized by a pediatric cardiologist and a perinatologist who were blinded to the pregnancy outcome. The groups with and without termination of pregnancy were compared using a univariate analysis and multivariate logistic regression. Results: In 119 (47%) of the 251 fetuses, parents opted for termination of pregnancy. In 103 of these cases (87%) there was a high complex or lethal cardiac, or a major or lethal extracardiac malformation. Of the 132 continued pregnancies, 42 fetuses (32%) had a high complex or lethal cardiac, or a major or lethal extracardiac malformation. There were significantly more terminations of pregnancy in case of a high or lethal complex cardiac or extracardiac anomaly (71 vs. 15%, p <0.001). Conclusion: Parents opted for termination of pregnancy significantly more often in cases with high complex cardiac and extracardiac malformations. It was rare for parents to opt for pregnancy termination in the absence of a severe cardiac or extracardiac malformation.

'Tobacco pipe' sign in the fetus: patent left superior vena cava with absent right superior vena cava.

1. Kutsche LM, Van Mierop LH. Anomalous origin of a pulmonary artery from the ascending aorta: associated anomalies and pathogenesis. Am J Cardiol 1988; 1: 850–856. 2. Carretero J, Rissech M, Mortera C, Mayol J, Caffarena J, Prada F. Aortic origin of the left pulmonary artery in an infant with Fallot’s tetralogy. Rev Esp Cardiol 2005; 58: 1124–1126. [Spanish] 3. Peng EW, Shanmugam G, Macarthur KJ, Pollock JC. Ascending aortic origin of a branch pulmonary artery – surgical management and long-term outcome. Eur J Cardiothorac Surg 2004; 26: 762–764. 4. Prifti E, Crucean A, Bonacchi M, Bernabei M, Leacche M, Murzi B, Bartolozzi F, Vanini V. Postoperative outcome in patients with anomalous origin of one pulmonary artery branch from the aorta. Eur J Cardiothorac Surg 2003; 24: 21–27. 5. Prifti E, Bonacchi M, Murzi B, Crucean A, Leacche M, Bernabei M, Bartolozzi F, Nathan NS, Vanini V. Anomalous origin of the right pulmonary artery from the ascending aorta. J Card Surg 2004; 19: 103–112. 6. Jung MJ, Yoo SJ. Prenatal diagnosis of anomalous origin of the right pulmonary artery from the ascending aorta. Cardiol Young 2002; 12: 186–188.

Contents Vol. 33, 2013

R. Achiron, Tel Hashomer N.S. Adzick, Philadelphia, Pa. L. Allan, London A.A. Baschat, Baltimore, Md. K.J. Blakemore, Baltimore, Md. T.-H. Bui, Stockholm F.A. Chervenak, New York, N.Y. T. Chiba, Tokyo R. Chmait, Los Angeles, Calif. F. Crispi, Barcelona J.E. De Lia, Milwaukee, Wisc. J.A. Deprest, Leuven G.C. Di Renzo, Perugia J.W. Dudenhausen, Berlin N.M. Fisk, Brisbane, Qld. A.W. Flake, Philadelphia, Pa. U. Gembruch, Bonn M.R. Harrison, San Francisco, Calif. J.C. Hobbins, Denver, Colo. L.K. Hornberger, San Francisco, Calif. E.R.M. Jauniaux, London M.P. Johnson, Philadelphia, Pa. C. Jorgensen, Copenhagen J.-M. Jouannic, Paris P.M. Kyle, London O. Lapaire, Basel S. Lipitz, Tel-Hashomer G. Malinger, Holon G. Mari, Detroit, Mich. M. Martinez-Ferro, Buenos Aires K.J. Moise, Houston, Tex. F. Molina, Granada K.H. Nicolaides, London D. Oepkes, Leiden L. Otaño, Buenos Aires Z. Papp, Budapest R.A. Quintero, Miami, Fla. G. Ryan, Toronto J. Rychik, Philadelphia, Pa. H. Sago, Tokyo W. Sepulveda, Santiago P. Stone, Auckland D.V. Surbek, Bern B.J. Trudinger, Westmead, N.S.W. J.M.G. van Vugt, Amsterdam Y. Ville, Paris Clinical Advances and Basic Research

618 Benefit of Prenatal Diagnosis of Complex Congenital Heart Defects on Neonatal Morbidity

Background and Aims As survival rates in neonatal cardiac surgery improve over time, morbidity becomes more important in complex congenital heart disease (cCHD). We investigated whether prenatal diagnosis of cCHD affects preoperative and early postoperative morbidity in neonates. Methods Retrospective observational study, performed between July 1st 2004 and July 1st 2011, including 247 infants with cCHD requiring surgery within the neonatal period. 113/247 patients were diagnosed with cCHD by prenatal screening (PreDx). Morbidity was based on preoperative use of high dose prostaglandin E and pre- and postoperative cardiac function, ventilation time, length of admission (LOF), inotropic support requirement and presence of severe complications (resuscitation, acute kidney and liver injury, and neurological events). Results Complexity of cCHD was higher in children diagnosed PreDx. After correction of complexity, median pre-operative ventilation time (3 vs 0 days), LOF (7 vs 9 days) and preoperative cardiac function were significantly better in PreDx neonates; and correction of acidosis, preoperative inotropic support and high dose prostaglandin E was significantly less common. Severe complications occurred significantly less when PreDx. There were no significant differences in postoperative morbidity and overall mortality. Conclusions Prenatal diagnosis of cCHD improves pre-operative morbidity significantly. The patient condition is better compared to postDx and the need for preoperative intensive care treatment is less. Seemingly, intensive care treatment can achieve a good pre-operative condition, even in neonates deteriorated due to an at birth unknown cCHD. This could explain why post-operative morbidity was equal in both groups.