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Copyright © 2014 Association for Prenatal Cardiology Development

23

Abstract

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.

Key words: 

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



6 parents decided to withdraw support of extracardiac 



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



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

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

2014



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

 

As other authors reported, the prognosis in DH and CHD 



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)

Figure 2. Congenital heart defect with dilated coronay sinus and stomach in the fetal chest (case nr 11, Table 1)


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



and Robert Yates

4

1

University College London Medical 



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

CS 



(EXIT)

2000 g 34

Demise 

6

2006



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

CS



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





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