Copyright 2014 Association for Prenatal Cardiology Development 23 Abstract
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Copyright © 2014 Association for Prenatal Cardiology Development 23
The primary aim of this study was to determine the predictive value of prenatal ultrasound and echocardiography for prognosis in congenital heart disease (CHD) with coexisting diaphragmatic hernia (DH) in a tertiary care center. Eleven records from the database of the Department for Diagnoses and Prevention of Congenital Malformations, Polish Mother’s Memorial Hospital Research Institute, were reviewed. The mean maternal age was 29,2 ± 5,1 years, and the mean gestational age at the time of diagnosis was 28,4 ± 6,7 weeks. No information was available for children discharged from hospital. Data of eight cases of prenatal DH and complex heart disease from the literature were also analyzed. Three fetuses survived the neonatal period. In each of these, CHD was other than the urgent or critical type, defined as not requiring cardiac surgical intervention in the first day or month of life. Both sets of data collected from our center and the published literature confirmed the unfavorable prognosis for fetuses with severe or critical CHD with coexisting DH.
Diaphragmatic hernia, complex congenital heart defect, prenatal diagnosis INTRODUCTION Congenital diaphragmatic hernia is a severe pathology leading to pulmonary hypoplasia and pulmonary hypertension. Respiratory distress is conside- red as the major cause of death in neonates suffering from CDH. Coexisting CDH and congenital heart disease increase the risk of serious complications and neonate’s demise.
In the present report, we posed the question: is prenatal diagnosis using ultrasound adequate to predict the prognosis of congenital heart defects (CHD) with coexisting diaphragmatic hernia (DH)? MATERIALS AND METHODS In the database from our unit (Filemaker Pro), all cases occurring between January 2007 and July 2014 and satisfying the following criteria were reviewed: POSTNATAL OUTCOMES OF CHILDREN WITH PRENATALLY DIAGNOSED CONGENITAL HEART DISEASE COMBINED WITH CONGENITAL DIAPHRAGMATIC HERNIA Original report Authors: Katarzyna Więckowska 1 , Lech Dudarewicz 2 , Hanna Moczulska 3 , Maciej Słodki 3 ,Zbigniew Pietrzak 4 , Maria Respondek-Liberska 3,5 1 Student’s Prenatal Cardiology Team, Medical University of Łódź, 2 Department of Genetics, Research Hospital Polish Mother’s Memorial Hospital, 3 Department of Fetal Cardiology Research Hospital Polish Mother’s Memorial Hospital, 4 Department of Obstetrics & Gynecology Reasearch Hospital Polish Mother's Memorial Hospital, 5 Department of Diagnoses and Prevention of Fetal Malformations Medical University of Łódź PRENAT CARDIO. 2014 DEC;4(4):23-27 DOI 10.12847/12143 Corresponding author: Maria Respondek-Liberska, majkares@uni.lodz.pl Submitted: 2014-07-27; accepted: 2014-11-19 diaphragmatic hernia and complex heart disease in singleton pregnancy and prenatal diagnosis, established at the Department of Diagnosis and Prevention of Congenital Malformations, Polish Mother’s Memorial Hospital Research Institute (Table 1). The outcome of pregnancy was verified in both the Polish National Registry of Fetal Cardiac Pathology and Hospital’s database. In the Medline database, publications concerning analogous case and case series reports were also analyzed (Table 2). RESULTS
Data of the 11 cases from our unit are listed in Table 1. The mean maternal age was 28,0 ± 5,2 years, and the mean gestational age at the time of diagnosis was 29 ± 7 weeks. The graphical representation of distribution of the gestational age of each fetus is shown in Figure 1. How to Cite this Article: Więckowska K, Dudarewicz L, Moczulska H, Słodki M, Pietrzak Z, Respondek-Liberska M.: Postnatal outcomes of children with prenatally diagnosed congenital heart disease combined with congenital diaphragmatic hernia. Prenat Cardio. 2014 Dec;4(4):23-27 PRENAT CARDIO. 2014 DEC;4(4):23-27 24 24 24 In two cases, pregnancy was terminated in accordance to Polish legislation. In five cases, neonates were delivered by caesarean section, and in one case by vaginal delivery, but with death on the first day of life. No information about the other three live-born neonates was found. Eight cases from literature (Table 2) pertaining to prenatal diagnosis of complex heart defect with coexisting DH were analyzed. Termination of pregnancy was performed in three cases, four neonates died in the first days of life (in case N o
oxygenation). The only newborn who did not required a cardiothoracic surgery in the first month of life remains alive and well. This child was not ductal dependent and had a planned later cardiac surgery. DISCUSSION The relation between DH and CHD has been well documented. Their coexistence is attributed to the formation of the diaphragm and heart at similar times during fetal development. CHD, among which hypoplastic left heart syndrome is the most frequent, are noted in 10 to 25 % of DH, especially in the left-sided DH 1 . No Y ear CHD Type of CHD in fetal cardiology ECM Fetal weight (in grams) at the time of diagnosis Wks of gestation at the time of diagnoseses Deliver y Geste age at the time of delivery Neonatal birth weight (in grams) Follow-up 1 2007 DORV, VSD Non-urgent DH, Galen Malformation, Polyhydramnion 2843
37 CS 39 2900 Death on 2 nd
day 2 2007 AV-canal + VSD musc., dextropositio Non-urgent DH, Single umbilical artery, IUGR, Ahydramnion 964 bd
bd No data
3 2008
AVC, Non-urgent DH, Lung hypoplasia, Hydronephrosis, Polyhydramnion, Megaureter 2920 38
CS 40 2980 Death after delivery (1h) 4 2009 Dextropositio, Disproportion, HLHS Severe / planned
DH, polyhydramnion 881
27 CS 39 3150 Death on 6 th
day 5 2009 Dextropositio, AV-canal Non-urgent DH, hydrothorax, Polyhydramnion, SGA
1976 35 CS 35 2450 Death on 1 st
day 6 2009 TGA, HLHS/AV- canal unbalanced, Dextrocardia Severe/ planned DH, Polyhydramnion 1289 30
bd bd No data 7 2012
Single ventricle, Trunus arteriosus, Dektrowersja, pericardial effusion Severe/planned DH, Diaphragma agenesis
316 19
CS 38 3800 Death on 1 st
day 8 2013 Tricuspid valve insuff., Hypoplastic aortic arch, disproportion Severe/planned DH – Right, polyhydramnion 1535
30 - - - Death in utero 9
Dekstrowersja, common AV-canal Non-urgent DH –Left
270 21
- - - TOP (?) 10 2014 SV/Truncus , Pericardial effusion. Myocardial hypetrophy Severe/planned DH, Hydrothorax 300 22
- - TOP (?) 11 2014
AVC/SV + TGA + PA + TAPVC + CS Severe/planned DH 2216
38 SN 39 2410 Death on 2 nd day Table 1: Data from Department of Congenital Malformations Research Institute Polish Mother’s Memorial Hospital regarding 11 fetuses with congenital heart defect and diaphragmatic hernia (years 2007-2014) bd = no data Figure 1. Schematic drawing of the fetal heart defect with diaphragmatic hernia (case nr 11, table 1) PRENAT CARDIO. 2014;4(4) Katarzyna Więckowska et al.
Copyright © 2014 Association for Prenatal Cardiology Development 25 Postnatal outcomes of children with prenatally diagnosed congenital heart disease combined with congenital diaphragmatic hernia According to the analysis carried out in Polish Mother’s Memorial Hospital Research Institute in 2006, diaphragmatic hernia coexisting with extracardiac malformations has been lethal. No patients diagnosed with DH and congenital heart disease survived beyond the neonatal period. 2
is poor 2-6
. No new scientific reports regarding DH+CHD have been observed since 2006. Also the late gestational age of the patients referred to our Department with respect to the consultations described in published reports should be emphasized, with regard to probable in utero demise of fetuses with these combined conditions . Chart 1. Presentation of the gestational age of fetuses with diaphragmatic hernia and congenital heart defect (from literature in green, from our uni in blue) PRENAT CARDIO. 2014;4(4) WEEK S OF GEST ATION OF THE ANAL YSED FETUSES PRENAT CARDIO. 2014;4(4)
PRENAT CARDIO. 2014 DEC;4(4):23-27 26 The Fetal Heart Team in Polish Mother’s Memorial Hospital Research Institute is composed of specialists of different sectors of perinatal care to discuss diagnoses and treatment of the youngest patients – fetuses. During Fetal Heart Team meetings, we take into account the recent classification of congenital heart diseases proposed by our team of prenatal cardiologists 7 . In this classification there are: Non-urgent heart defects (demanding intervention during infancy), Urgent but planned ductal dependent (not immediate) (requiring classic cardiac surgery during the neonatal period) Critical (usually also ductal dependent) heart defects for which immediate intervention after delivery or even in utero is required for; The most severe heart defects meaning the treatment is currently not possible. This primary classification of CHD was further changed by Słodki 8 , who added two other categories of CHD: heart defects coexisting with structural extracardiac malformations (which may be potentially corrected by surgical intervention) and heart defects coincidental with extracardiac anomalies (they are not qualified to surgical treatment). According to Słodki, only 14% of 89 neonates admitted to Polish Mother’s Memorial Hospital Research Institute with CHD complicated by extracardiac anomaly survived the first month of life, provided that only one of the malformations was treated surgically. No survival of neonate with severe or critical heart defect and extracardiac malformation was observed if both ECM and CHD required surgical treatment during the first 28 days of postnatal life. In the literature only two survivals have been reported of neonates with prenatal diagnosis of DH and CHD (Table 2, positions 4 and 9). In both cases the heart’s Lp. Y ear Author /Hospital CHD + ECM Type of CHD in prenatal cardiology WKs of gestation at the time of diagnosis Deliver y Bir th weight WK s of gestation at the deliver y Follow-up 1 1994 Respondek ML, Binotto CN, Smith S, Donnenfeld A, Weil SR, Huhta JC; Pennsylvania Hospital, Philadelphia, USA VSD+DH+ dekstrokardia Non-urgent bd - - - Neonatal demise 2 1998
Yung Hang Lam, Mary Hoi Yin Tang, Siu Tsan Yuen, Hongkong, China CHD, membranous VSD, DH (left side), hipertrofia RV, pulmonary valve atresia Severe / planned 12
- - TOP 3 1999
Lucia Migliazza, Christian Otten, Huimin Xia, Jose I. Rodriguez, Juan A. Diez-Padro, Juan A. Tovar, Madrid, Spain DH + TGA Severe / planned bd
- - Neonatal demise 4 2000
Lee Noimark 1 , Mark Sellwood 2 , John Wyatt 3 and Robert Yates 4 1
School, London, UK; 2 Neonatal Intensive Care Unit, University College Hospital, London, UK; 3 Neonatal Intensive Care Unit, University College Hospital, London, UK;
4 Cardiothoracic Unit, Great Ormond Street Hospital, London, UK TGA+VSD+DH, Diproportion Severe / planned 19
- 37 8 month alive & well 5 2004
Hamrick SEG, Brook MM, Farmer DL, San Fransisco, California, USA DH + pulmonary sequestration + TGA Severe / planned 27
(EXIT) 2000 g 34 Demise 6
Isabel López, Juan A. Bafalliu, M. Carmen Bernabé, Francisco García, Miguel Costa and Encarna Guillén-Navarro, Argentina DH, AVSD
Non-urgent 18 - - - TOP 7 2006
Isabel López, Juan A. Bafalliu, M. Carmen Bernabé, Francisco García, Miguel Costa and Encarna Guillén-Navarro, Argentina IUGR, CHD+DH (left side), overlapped toes - 19 - - - TOP 8 2008 Wojciech Fendler, Andrzej Piotrowski, Medical University Hospital, Lodz, Poland DH + d-TGA (in autopsy) Severe / planned bd
bd 36/37
Death 1 h after delivery
Table 2: Data from literature: fetal heart defect + diaphragmatic hernia (1994-2008) Katarzyna Więckowska et al. bd = no data Copyright © 2014 Association for Prenatal Cardiology Development 27 Postnatal outcomes of children with prenatally diagnosed congenital heart disease combined with congenital diaphragmatic hernia septal or atrial defects did not constitute indication for cardiosurgical intervention. These defects would not be considered as severe or critical from the viewpoint of prenatal cardiology. Some cases of CHD complicated by DH might be still diagnosed postnatally but these are likely to exist in the wide spectrum of these malformations that are significantly less severe. CONCLUSION The presented analyses of cases from the literature, as well as those observed by our Department, confirm the unfavorable prognosis for neonates in cases of severe or critical prenatally detected congenital heart defect with coexisting diaphragmatic hernia. Additional cases with milder forms may survive neonatal surgery for diaphragmatic hernia and later on (in infancy) surgery for heart defect. Figure 3. Abnormal pulmonary venous drainage behind the left atrium (case nr 11, table 1) PRENAT CARDIO. 2014;4(4) References: 1. Noimark L, Sellwood M, Wyatt J, Yates R.: Transposition of the great arteries, ventricular septal defect and diaphragmatic hernia in a fetus: the role of prenatal diagnosis in helping to predict postnatal survival. Prenat Diagn 2000, 20: 924-926 2. Respondek-Liberska M, Foryś S, Janiszewska-Skorupa J, Szaflik K, Wilczyński J, Oszukowski P, Krasomski G, Maroszyńska I, Biegański T, Kulig A, Jakubowski L, Chilarski A.: Problemy diagnostyczne i losy płodów z przepukliną przeponową w ośrodku referencyjnym ICZMP w latach 1994-2006. Ginekol Pol 2008, 79: 23-30 3. Respondek ML, Binotto CN, Smith S, Donnenfeld A, Weil SR, Huhta JC.: Extracardiac anomalies, aneuploidyand growth retardation in 100 consecutive fetal congenital heart defects. Ultrasound Obstet. Gynecol 1994, 4: 272-278 4. Cohen MS, Rychik J, Bush DM, Tian ZY, Howell LJ, Adzick NS, Flake AW, Johnson MP, Spray TL, Crombleholme TM.: Infleunce of congenital heart didease on survival in children with congenital diaphragmatic hernia. J Pediatr 2002, 141 (1): 25-30 5. Migliazza L, Otten C, Xia H, Rodriguez JI, Diez-Padro JA, Tovar JA. Cardiovascular malformations in congenital diaphragmatic hernia: Human and experimental studies. Journal of Pediatric Surgery 1999, 34 (9): 1352-1358 6. Hamrick SEG, Brook MM, Farmer DL.: Fetal surgery for congenital diaphragmatic hernia and pulmonary sequestration complicated by postnatal diagnosis of Transposition of the Great Arteries. Fetal Diagn Ther 2004, 19: 40-42 7. Respondek-Liberska M.: Atlas Wad Serca Płodów, Adi Art, Łódź, 2011 8. Słodki M.: Opracowanie modelu opieki nad ciężarną z wrodzoną wadą serca u płodu na podstawie nowego prenatalnego podziału wad serca. Rozprawa habilitacyjna. Łódź, 2012 9. Lam YH, Tang MH, Yuen ST.: Ultrasound diagnosis of fetal diaphragmatic hernia and complex congenital heart disease at 12 weeks‘ gestation – a case report. Prenat Diagn. 1998, 18 (11):1159-62 10. Fendler W, Piotrowski A.: Przełożenie wielkich naczyń u noworodka z wrodzoną przepukliną przeponową – Opis przypadku. Medycyna Wieku Rozwojowego 2008, 3; 778-781 Financing: The research was not financed from the external sources Conflict of interest: The authors declare no conflict of interest and did not receive any remuneration related to the creation of this work. Authors and division in work: K. Więckowska: data search and first draft M. Słodki: discussion and correction of the manuscript L. Dudarewicz: discussion and correction of the manuscript H. Moczulska: data search, photo search, discussion, submission Z. Pietrzak: discussion and correction of the manuscript M. Respondek-Liberska: concept of the research, correction of the paper, final version Yüklə 80,54 Kb. Dostları ilə paylaş:
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