CARDIOVASCULAR JOURNAL OF AFRICA • Vol 23, No 8, September 2012
AFRICA
e11
Case Report
A case of unexplained cyanosis
PRASHILLA SOMA, SHIRAZ ELLEMDIN
Abstract
It is now clear that hepatopulmonary syndrome (HPS) may
occur and contribute significantly to gas exchange abnor-
malities in the setting of other cardiopulmonary abnormali-
ties. Since there is no gold-standard diagnostic test for HPS,
diagnosis rests on documenting arterial oxygenation abnor-
malities resulting from intrapulmonary vasodilatation in the
setting of liver disease. Retrospective studies suggest that
many patients with HPS develop progressive intrapulmonary
vasodilatation over time and that mortality is significant.
This case highlights the clinical value in investigating for
HPS and right-to-left shunts when confronted with a patient
presenting with unexplained hypoxia in combination with
platypnoea and/or orthodeoxia.
Keywords:
cyanosis, hepatopulmonary syndrome, hypoxaemia
Submitted 22/8/11, accepted 11/4/12
Cardiovasc J Afr
2012;
23
:
e11–e12
DOI: 10.5830/CVJA-2012-033
Case report
A 26-year-old female presented to our casualty department in
March 2004 with the main symptom of progressive dyspnoea in
the absence of orthopnoea or paroxysmal nocturnal dyspnoea,
with associated marked impaired effort tolerance and generalised
weakness. The onset of symptoms coincided with a vague
episode of blunt chest trauma. There was no chest pain or
palpitations. She further reported being blind in the right
eye. Her past medical history included surgical removal of a
non-functional pituitary macro-adenoma eight years previously,
with insertion of a ventriculo-peritioneal (V-P) shunt and post-
surgical radiotherapy.
On general examination, her height was 146 cm with
minimal secondary sexual characteristics. Her vitals included
a temperature of 37°C, blood pressure of 93/62 mmHg, pulse
of 80 beats/minute, respiratory rate of 18 breaths/minute, with
central and peripheral cyanosis. It was also noted that she was
less dyspnoeic and cyanotic in the supine position. Auscultation
of the chest revealed normal breath sounds with a physiologically
split second heart sound. She was blind in the right eye with a
pale optic disc and visual field defects in the left eye.
Investigations for the cyanosis included a blood gas, the
results of which are shown in Table 1, a ventilation perfusion
scan, chest radiograph, high-resolution CT chest and pulmonary
arteriogram, all of which were normal. The echocardiogram
showed normal cardiac function with normal chamber sizes
and no shunts. The polysomnogram performed revealed no
abnormalities. In addition, a 100% oxygen shunt study was
done, which suggested a 15.4% shunt, the details of which are
illustrated in Table 2. Lung functions could not be done due to
poor co-operation from the patient.
In view of the platypnoea and orthodeoxia, transthoracic
and transoesophageal echocardiograms were performed. Both
revealed an interatrial shunt in the supine position within three
cardiac cycles of injecting. The transthoracic echocardiogram
was diagnostic for the sitting position as well. A final diagnosis of
patent foramen ovale with right-to-left shunting was established
despite normal intracardiac pressures.
The patient underwent open-heart surgery on 22 April 2004
for the closure of an alleged patent foramen ovale. However,
no intracardiac shunt or anomalous drainage could be defined
at cardiac surgery. She died at home on 8 May 2004 while
recovering from surgery.
A post mortem was performed on 11 May 2004. The major
finding consisted of a thrombo-embolus lodged deep within
the right pulmonary artery. In addition, multiple small thrombi
were noted adhered to the right atrial wall. The thrombus had
developed in the area of the previous surgery, from where it gave
origin to the thrombo-embolism that had caused the patient’s
sudden death.
A further finding was a massive right-sided pleural effusion
Department of Physiology, University of Pretoria, Pretoria,
South Africa
PRASHILLA SOMA, MB ChB, MSc (Clin Epi), prashilla.soma@
up.ac.za
Department of Internal Medicine, University of Pretoria,
Pretoria, South Africa
SHIRAZ ELLEMDIN MB ChB, MMed (Int Med)
TABLE 1. RESULTS OF THEARTERIAL BLOOD
GAS IN DIFFERENT POSITIONS
Room air
Sitting 45 degrees Supine
Oxygen saturation (%)
69
81
83
Partial pressure oxygen (mmHg)
26
31
35
Partial pressure carbon dioxide (mmHg)
17
19
20
TABLE 2. RESULTS OF 100% OXYGEN SHUNT STUDY
Arterial blood
gas
Oxygen
saturation
(%)
Partial
pressure
oxygen
(
mmHg)
Partial
pressure
carbon
dioxide
(
mmHg)
Bicarbonate
(
mmol/l)
Room air
56
33
23
13
40%
oxygen
78
47
24
13
100%
oxygen
99
379
22
13
