DEXTROCARDIA WITH

SITUS INVERSUS AND

ASSOCIATED CARDIAC
MALFORMATION
IN A CHILD
A CASE REPORT
Presented by: A. Haris Khoironi

ABBREVIATION

AV atrioventricular

CHD congenital heart disease (or defect)

CHF congestive heart failure

COA coarctation of the aorta

DORV double outlet right ventricle

ECG electrocardiograph or electrocardiographic

echo echocardiography or echocardiographic

IVC inferior vena cava

LA left atrium or left atrial

LPA left pulmonary artery

MPA main pulmonary artery

MR mitral regurgitation

PA pulmonary artery or arterial

PBF pulmonary blood flow

PDA patent ductus arteriosus

INTRODUCTION
Dextrocardia
the heart located at the right chest
baseapex axis directed rightward
primarily resulted from embryologic cardiac

development disorders.

Dextroposition
most of the cardiac mass is located to the

right, but baseapex axis not always directed

rightward

Dextroversion no longer used.

Cooley 1972, Wilkinson 2011, Evans 2010, Hagler 2008
3

introduction
Situs inversus
atria and viscera reversed
morphologic left atria is on the right & vice versa
liver and gallbladder in the left-side of abdomen
whereas the spleen and the stomach on the right
Situs solitus atria and viscera on the normal position
Situs ambiguus/situs indeterminus/heterotaxy

when both of the atria are morphologically left/right

Dextrocardia occurs most commonly with situs inversus

Hagler et al 2008, Wilhelm et al 2009, Park 2008, Coffman et al 2007

introduction
Etiology
Unknown
Genetic & inheritance (?)
X-linked / autosomal recessive (?)
Maternal diabetes, using cocaine during pregnancies,

conjoined twins predisposing factors

Often with normal function delayed dx

Often with other cardiac malformation
dx based on echo
clinical methods of locating cardiac chambers using

chest x-ray films, ECGs, and physical examination are

still beneficial

Coffman et al 2007, Park 2008

5

introduction
Purpose
to report a rare case of dextrocardia with

situs inversus and associated cardiac

malformation, focusing on diagnosis and
hemodynamic consequences

CASE REPORT
N, female, 7 months old
Dec 11th 2010

Dec 14th 2010

hospitalized at Soegiri

Hospital Lamongan
difficulty of breath, cough,

could, and suspicious of

lung tuberculous
treated with injection of
antibiotic, and antituberculous drug
3 days later referred to
Dr. Soetomo Hospital
because the dyspneu
became more severe.

Chief complaint: dyspnea

Referred from Soegiri

Hospital Lamongan
dx: right massive pleural
effusion and pneumonia.
History:
non-productive cough and
could since 2 weeks before
sub-febrile since 1 week
before.
often got choking during
breastfed
7

case report
History taking
Px 1st girl of 3rd pregnancies
1st & 2nd were aborted at 6th and 3rd

month
6th month of last pregnancy the mother
got rash at whole of her body and sub
febrile that became disappear gradually
during 7 days.
The mother had no diabetic, hypertension,
nor taking any drugs and traditional herbs
during pregnancy.
8

case report
The baby delivered spontaneously per

vaginam, aterm, assisted by midwife, and

BW 3600 g
not crying immediately cyanotic during
a few minutes.
The baby have got immunizations of
Hepatitis B, BCG, and DPT I and II.
From the growth and development history,
it was found that the baby lift the head on
6 months old and rolling on 7 m.o.
9

case report
History taking
Px 1st girl of 3rd pregnancies
1st & 2nd were aborted at 6th and 3rd

month
6th month of last pregnancy the mother
got rash at whole of her body and sub
febrile that became disappear gradually
during 7 days.
The mother had no diabetic, hypertension,
nor taking any drugs and traditional herbs
during pregnancy.
10

case report
Physical examination
a weak baby, irritable, and dyspnea
body weight 5.5 kg, height 66 cm, head

circumference 41 cm.
Vital signs:
pulse rate: 140 tpm,
respiratory rate: 39 tpm
body temperature: 37.7oC.

anemic - ; jaundice - ; cyanotic + ; respiratory

distress +

11

case report
Physical examination
The chest was symmetric
retraction of sub-costal was noted
The heart sound was heard maximally at right

side of the chest.

Continuous murmur could be heard on upper
right sternal border with intensity grade 3/6.
There was no gallop.
The fine crackle of breath sound was heard on
both side of the chest.

12

case report
Physical examination
abdominal distended
ascites
liver /spleen: not palpable
bowel sound : normal.
the extremities were warm, cyanotic, and dry.
clubbing finger
edema
external genital: normal
neurological examinations: normal.

13

case report
Laboratory finding

BGA

Blood Exam
Hb

11.1 g/dl

pH

7.31

WBC

9200/L

pCO2

34 mmHg

Platelet

451.000/L

pO2

121.5 mmHg

HCt

35,1%

Base excess

-9

Na

143 meq/L

HCO3

20 mmol/L

3.65 meq/L

O2 Sat

98%

Ca

8.3 mg/dL

Cl

105.4 meq/L

BUN

4.8 mg/dL

Glucose

60 mg/L

S Cret

0.5 mg/dL

CRP

4,67 mg/L

AST

36 IU/L

ALT

16 IU/L

14

case report
Chest X-ray AP/Lat
the apex of the heart and pulmonal cones were

on the right-side of the chest

bronchopneumonia

15

case report
Chest X-ray AP/Lat October 13th 2010
right massive pleural effusion and left lung

edema with DD lung tuberculous and massive

right lung pneumonia

16

case report
Assesment
dextrocardia and acyanotic congenital heart

disease s. VSD and pneumonia

Planning
Dx:
work-up for tuberculous i.e. Mantoux test,
erythrocyte sedimentation rate, and Fast Acid
Bacilli
ECG
Echocardiography

17

case report
Planning
Tx:
fluid management
Oxygenation
Ampicillin-sulbactam 150 mg t.i.d. intravenously
anti-tuberculous drug stopped
Thermoregulation
Nebulization
fasted for a while because of dyspneu.

18

case report
On 2nd day of admission,
the dyspneu was decreased.
pulse rate was 132 times per minute,
the respiratory rate was 36 times per

minute
the body temperature was 36.8oC
the oxygen saturation was 89-91% on the
upper limb, and 79 80% on lower limb

19

case report
On 2nd day of admission,
ECG:
sinus rhythm 136 per minute;
P wave inverted in lead I, aVL
P axis was between +90 and
+180 (right deviation axis);
no transitional zones
also loss of voltages of V3 until
V6
after the leads reversed, the
transitional zones appeared

20

case report
On 2nd day of admission,
Echocardiography:
dextrocardia
situs inversus
hypoplasia of right ventricle
double outlet right ventricle (DORV)
moderate patent ductus arteriosus (PDA)
coarctation of aorta (CoA)
large subpulmonic ventricular septal defect (VSD)
severe mitral regurgitation (MR)
moderate tricuspid regurgitation (TR)
suggested for ACE inhibitor administration and

planned for catheterization.

21

case report
On 3rd day of admission
cough and cold were decreased
acid fast bacilli test I (from gastric aspirate): -

On 5th day of admission

acid fast bacilli test I (from gastric aspirate): Mtx test: -

Blood Exam
Hb

12.7 g/dl

WBC

7100/L

Platelet

suff

ESR

10-20 mm/hr

Diff Count

1/0/0/60/38/3

22

case report
On 7th day of admission,
vital sign was stable,
patient was moved from PICU to the pediatric

ward
culture of blood/urine/feces no bacterial
growth.

On 8th day of admission

the breath sound was clear

no crackles heard.
chest X-ray photo examination
a little patchy infiltrate.
23

case report
On 9th day of admission,
the pulse rate was 120 tpm
the respiratory rate was 37 tpm
the body temperature was 36.7oC
the oxygen saturation was 88 91%
Discharged
advised to have a regular follow up

observation
scheduling of catheterization.

24

DISCUSSION
This patient came with the chief

complaint of dyspnea, cough and

sub-febrile.
the heart sound
best heard on the
right chest
the picture of
dextrocardia was
appeared in chest
X-ray

25

discussion
Position of the heart on the chest
physical examination chest auscultation

heart sound is louder or best heard on

the right chest
electrocardiography

no transitional zone in precordial leads /loss

of voltage usually on V3-V6
P inverted in lead I and
echocardiography is the best

way to determine the orientation

of base-apex axis of the heart.
26

discussion
Position of the heart on the chest

Hagler 2008
27

discussion
In this case
the heart sound was best heard on the

right chest
ECG :
loss of voltage or disappearance of transition
zone on precordial leads
P inverted appeared on lead I and aVL.
Echocardiography:
Four chamber view of
the apex of the heart
pointing to the right
28

discussion
In this case
The chance to find out the dextrocardia actually

could be done while the patient hospitalized on

the Soegiri Hospital.
Unfortunately the chest
X-ray on that hospital
revealed massive radioopaque on the right
hemithorax, so that was
masking the
dextrocardia. Also the
ECG was not obtained.
29

discussion
Once cardiac malposition found
evaluate the chamber localization.
the heart and great arteries can

be viewed as three separate

segments: the atria, the
ventricles, and the great arteries.
The segmental approach of van
Praagh is useful in determining
the relationship at each segment.
Philosophy of segmental analysis
is founded on morphology.
From: Hagler DJ, O'leary PW. Cardiac
Malpositions and Abnormalities of Atria and
Visceral Situs. In: Allen HD, Driscoll DJ,Wilhelm
Shaddy RE, Feltes TF, editors. Moss and
Adams Heart Disease In Infants, Children,
and Adolescents Including The Fetus and
Young Adult. 7th ed. Philadelphia: Lippincot

2008, Park 2008, Oechslin 2008

30

discussion
The steps in the segmental approach are:
(1) determine cardiac sidedness / position
chest-X-ray, ECG, echo
(2) identify the three segments
(atria,ventricles, great arteries)
echo
(3) define the connections (atrio-ventricular /
ventriculo-arterial)
Echo
Park 2008, Oechslin 2008

31

discussion
(1) determine cardiac sidedness /
position
Chest X-ray gastric air bubble +
liver shadow
ECG
Echocardiography

Park 2008, Oechslin 2008

32

discussion
determine cardiac sidedness / position
Chest X-ray
ECG p-axis
echocardiography

Park MK. Chamber Localization and Cardiac Malposition. In: Park MK, editor. Pediatric
Cardiology for Practitioners 5th ed. 5th ed. Philadelphia: Mosby, 2008:309-12.

33

discussion
determine cardiac sidedness / position
Chest X-ray
ECG p-axis
Echocardiography

Park 2008, Oechslin

Oechslin 2008
34

discussion
(2) identify the three segments
(atria,ventricles, great arteries) echo
(morphologically)
(3) define the connections (atrio-ventricular /
ventriculo-arterial) echo

RV

LA

LV

RA

Oechslin 2008

35

discussion
the location of ventricle also can be identified
by the ECG
By identifying q-wave in lead I, V1, V6, V4R, and
V6R.

In this case: q-wave appeared in lead I and V6,

and disappear in V1, V4R, and V6R RV is on
the right side.

Park MK. Chamber Localization and Cardiac Malposition. In: Park MK, editor. Pediatric
Cardiology for Practitioners 5th ed. 5th ed. Philadelphia: Mosby, 2008:309-12.

36

discussion
For brevity, some authors proposed the symbols in

describing the segmental relationship.

Richard and Stella van Praagh on 1960s, firstly
notated symbols for cardiac segmental.
The following symbols are used in describing the
segmental relationship
(1). Visceroatrial relationship: S (solitus), I (inversus), or A

(ambiguus);
(2) Ventricular loop: D (D-loop), L (L-loop), or X (uncertain
or indeterminate);
(3) Great arteries: S (solitus), I (inversus), D (Dtransposition), or L (L-transposition).

Evans et al 2010, Cooley 1972, Coffman 2007, Marx 2011

37

discussion
In this case:
the visceroatrial relationship was inversus (I),
the ventricular loop was D-loop (D),
and the relationship of the great arteries was
DORV with the aorta anterior and to the right
of pulmonary artery, or D-loop (D)
the segmental expression for this patient
was [I,D,D].

38

atomy. 2, 4, 9, 12 The following symbols are used in describing the segmental relationship: (1). Visceroatrial relationship: S (solitus), I (inversu

discussion
Many variants of associated cardiac malformations in

dextrocardia with situs inversus.

Garga et al (2003) reported among total 125 patients,
dextrocardia was most common with situs inversus
(39.2%) followed by situs solitus (34.4%) and situs
ambiguous [26.4% (right isomerism in 18.4% and left
isomerism in 8.0%)]. Forty percents dextrocardia also
associated with cardiac malformation.
Roodpyema (2003) reported 17.7% patients with
dextrocardia had situs inversus. Related cardiac
anomalies included: VSD, TGA, and ToF, which occurs 4,
4, and 1 among 15 patients observed, respectively. The
remaining are patients with extracardiac malformations.
39

atomy. 2, 4, 9, 12 The following symbols are used in describing the segmental relationship: (1). Visceroatrial relationship: S (solitus), I (inversu

discussion
Bohun, et al (2006) reported 81 cases of

dextrocardia, which 27 cases were situs solitus,

30 situs inversus, and 24 situs ambiguous or
isomerism. Cardiac malformations were found in
26 of 27 cases of situs solitus, 7 of 30 cases of
situs inversus, and 24 of 24 cases of isomerism.
Evans, et al (2010) reported 28 patients among
61 with dextrocardia which the situs are situs
inversus. Only one patient who segmental
expression was [I,D,D]. Associated cardiac
malformations included: VSD, CoA, ToF, and
PDA.
40

atomy. 2, 4, 9, 12 The following symbols are used in describing the segmental relationship: (1). Visceroatrial relationship: S (solitus), I (inversu

discussion
In this case, the associated

cardiac malformations :
double outlet right ventricle (DORV)
patent ductus arteriosus (PDA)
ventricular septal defect (VSD)
coarctation of the aorta (CoA)

41

The hemodynamic could be explained

diagramatically as below:

atomy. 2, 4, 9, 12 The following symbols are used in describing the segmental relationship: (1). Visceroatrial relationship: S (solitus), I (inversu

discussion
In this case, the patient also suffered

from pneumonia
occasionally occurred in patient with left

to-right shunt
the oxygenated blood flows back into the
lung
increase the pulmonary circulation above
the systemic
increasing the risk of pulmonary infection
pushed by great vessel obstructed
retention of the secret in respiratory tract

Stanger et al 1969
43

SUMMARY
A rare case of dextrocardia with situs

inversus and associated cardiac malformation

in a child has been presented.
The best way to describe the malformations
is by using echocardiography, but clinical
finding, chest X-ray, and ECG is still beneficial
The hemodynamic is depend on the type of
the malformations.
Pneumonia is the most common infection
comes along with left-to-right shunt cardiac
anomaly.
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THANK

YOU

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