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CARDIOVASCULAR JOURNAL OF AFRICA • Volume 30, No 1, January/February 2019
AFRICA
7
no statistical difference between the two situs arrangements and
their associated cardiac malformations (
p
=
1.000).
Eighteen patients demonstrated situs ambiguous, with an
incidence of 16.7%. In this subgroup, 17 had documented
ACM (94.4%), five were found to have RAI and eight to have
left LAI. The remaining five patients with situs ambiguous did
not have their atrial isomerism defined. Fourteen patients in the
dextrocardia subgroup had unknown situs arrangements (13%).
All of the 76 patients with dextroposition demonstrated
situs solitus. This is in keeping with the underlying aetiology of
dextroposition and involves external forces that may either be
compressive or pulling in nature.
Of the two patients with dextroversion, one had situs solitus
and one had situs inversus with TGA.
Ventricular arrangements
The majority of patients with dextrocardia had two ventricles
(84.3%), while 17 had a univentricular heart (15.7%). Over
two-thirds of the patients demonstrated atrioventricular (AV)
concordance, 10 had AV discordance, and the AV relationship of
25 patients was not recorded. Sixty-three patients demonstrated
ventriculo-arterial (VA) concordance, 11 had VA discordance,
13 had double-outlet right ventricle (DORV) and the VA
relationship of 22 patients was not recorded.
All the patients with dextroposition had normal AV and VA
relationships.
Both the patients diagnosed with dextroversion had two
ventricles and AV concordance. One had VA discordance (TGA)
while the other patient had a normal heart.
Extra-cardiac abnormalities
A total of 48 patients with dextrocardia had associated extra-
cardiac abnormalities (44.4%), eight of whom (16.7%) were
diagnosed with Kartagener syndrome (situs inversus totalis,
chronic sinusitis and bronchiectasis). Two out of the five patients
diagnosed with dextrocardia and RAI were confirmed to have
asplenia on computerised tomography of the abdomen, and one
patient had gastric outlet obstruction. There were eight patients
diagnosed with LAI, three had an interrupted inferior vena
cava, one patient had heart block, while duodenal atresia was
diagnosed in one other.
Surgery
Cardiac surgery was performed in 25 of the patients with
dextrocardia (23.1%). Eight of these procedures were corrective
(32%), while 17 were palliative (68%). Corrective surgeries
included ligation of a PDA, closure of a ventricular septal
defect (VSD) and/or pulmonary artery (PA) banding. Palliative
procedures included Blalock–Taussig or Glenn shunts. Nine
patients with dextrocardia underwent general gastrointestinal
(GIT) surgeries (Table 2). Four patients in this subgroup
demonstrated isomerism (44.4%).
Twenty-nine patients with dextroposition underwent surgical
procedures. Seven were diagnosed with Scimitar syndrome and
had percutaneous occlusion of the anomalous feeder arteries
from the descending aorta. A wide variety of non-cardiac
surgeries were undertaken in the remaining patients with
dextroposition (Table 3).
Patient outcomes
Twenty-one (19.4%) of the patients with dextrocardia were
reported dead at the time of the study. All had complex ACM,
which included: hypoplastic left ventricle, tricuspid atresia,
double-outflow right ventricle, and single atrium and ventricle.
Five had previously undergone cardiac surgery.
Nine out of the 21 patients had situs inversus (42.9%), six had
situs solitus (28.6%) and five had situs ambiguous (23.8%). In
two patients the situs was undefined.
Infection was the documented cause of death in seven
patients (33.3%). Twenty-two patients (20.4%) were confirmed
alive, and 65 (60.2%) had been lost to follow up.
Eight patients with dextroposition had died at the time of
the study (10.5%), two of whom had Scimitar syndrome. Eleven
were found to be alive (14.5%), while the majority of patients
were lost to follow up (75%).
In the dextroversion subgroup, neither of the patients
underwent surgery and both were alive at the time of the study.
Situs
inversus
Situs
solitus
Situs
ambiguous
Undefined
Number of patients
60
45
30
15
0
52
25
18
14
Fig. 2.
Dextrocardia: situs arrangements.
Table 2. Gastrointestinal surgical procedures performed
in patients with dextrocardia
Surgical procedure
No. of patients
Kasai for biliary atresia (situs inversus, 1)
1
Tracheo-oesophageal fistula repair (heterotaxy: undefined, 1)
1
Jejunal atresia (heterotaxy: undefined, 1, situs solitus, 1)
2
Duodenal atresia (left atrial isomerism, 1)
1
Gastric outlet obstruction (right atrial isomerism, 1)
1
Omphalocele (situs inversus, 2, situs solitus, 1)
3
Total
9
Table 3. General (non-cardiac) surgical procedures
performed in patients with dextroposition
Surgical procedure
No. of patients
Congenital diaphragmatic hernia repair
11
Space-occupying lesion
3
Mass excision (thorax), 2
Cyst drainage (left thorax), 1
Tracheo-oesophageal fistula repair
3
Duodenal atresia
1
Omphalocele
2
Total
20