International Journal of Clinical Case Reports 2015, Vol.5, No. 41, 1-5
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exception central cyanosis obstruction of the pulmonary
outflow tract and pulmonary atresia. Serious
extracardiac anomalies have also been associated with
RI. Extracardiac abnormalities of HTS are abnormalities
of the renal tract, especially biliary atresia, duodenal
atresia or compression. The clinical scenario of a late
intestinal obstruction in a child with HTS may be a
result of gastrointestinal malrotation or gastric
volvulus (Ticho et al., 2000; Kim et al., 2008). The LI
in the baby presented was hemodynamically stable
and the duodenal atresia required surgery.
The ever changing advancement in the field of
ultrasonography has helped clinicians and radiologists
interested in fetal ultrasonography to detect the
congenital anomalies and there lies the significance of
HTS as it is associated with many prenatal and
perinatal morbidity and mortality in the fetus and the
newborn. So it helps in the diagnosis of these
problems very early and appropriate measures can be
taken depending upon the viability of the fetus ,to
continue the pregnancy and also in the undue care of
the newborn. The case in the study was detected to
have a HTS in the antenatal scan at fifth month of
gestation and was under regular follow up. The
radiological evaluation especially ultrasonogram in
the hands of an experienced radiologist can clinch the
diagnosis. Imaging features can be extremely complex
(Yoo and Jaeggi, 2008). There could be duplication of
the left or right-sided intra-thoracic contents with
associated changes below the diaphragm. Malposition
of the liver, stomach and spleen (which may be absent)
along with an altered the vascular supply above and
below the diaphragm may be identified (Kim et al.,
2008; Yoo and Jaeggi, 2008).
A case of fetal HTS have been highlighted in literature
(Chon et al., 2011). Lee et al. reported six cases of
HTS with left isomerism having intra-abdominal
anomalies such as including malrotation, biliary
atresia and acute appendicitis with polysplenia in only
one of them (Lee et al., 2006). HTS in adults have
been reported but is rare in paediatric population
(Carneiro et al., 2013).
The ECG and abnormalities of cardiac rhythm are
important as these children can have varying degrees
of heart blocks and can be identified in the ECG. Atrio
ventricular block is rare in the RI and is common in LI
(Escobar-Diaz et al., 2014). The use of 2D Echo is
very worthwhile to diagnose these complex CHD
and is quite challenging for the cardiologist. The
2DEcho and ECG was done again at higher centre and
confirmed the HTS with LI and ECG did not show
any evidence of a trio ventricular block. An usual
protocol in the work up of the case of HTS should
include chest radiograph and 2DEcho to assess for
congenital heart disease, abdominal ultrasound and
barium studies for assessing intra abdominal contents
(especially spleen) and find associated intestinal
malrotation and anomalies (Kim et al., 2008; Sutherland
and Ware, 2009).
The prognosis of patients with HTS is poor. The
1-year mortality postulated is >85% for patients with
asplenia and >50% for patients with polysplenia.
Isomerism and associated extra cardiac anomalies also
have their consequences. There is risk of septicaemia
in the absent spleen situations (Dyke et al., 1991). The
preterm newborn had septicemia and excellent
antibiotic coverage was administered but succumbed
to the illness.
The treatment of these children with HTS is directly
proportional to the the nature and severity of the
associated cardiac and extra cardiac lesions (Kawashima
et al., 1984; Bartz et al., 2006
et al., 2010). The
cardiac surgeries for patients with isomerism in HTS
are palliative in nature, as the normal anatomy is
rarely achieved. The abnormalities of the systemic
venous connection, a partial or total anomalous
pulmonary venous connection, a common atrioventricular
valve and a single ventricle carry high risk and about
60% of patients with LI have anatomy suitable for
biventricular repair. The anomalies in RI are very
difficult to correct by this procedure even though
cases have been presented (Kawashima et al., 1984;
Lin et al., 2000; Bartz et al., 2006). Patients with HTS
usually require palliative surgery in the neonatal
period. Many procedures have been advocated which
include Fontan-type procedure, modified Fontan
procedures or the bidirectional cavopulmonary
anastomosis (Kawashima operation) (Kawashima et al
1984; Lin et al., 2000; Bartz et al., 2006;
t al.,
2010). Patients with HT may have varying degrees of
intestinal rotation (Ditchfield and Hutson, 1998). The
extra cardiac complications requires early recognition
