European Journal of Echocardiography 2008 9(1):47-49; doi:10.1016/j.euje.2006.08.003
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2006. For permissions please email: journals.permissions@oxfordjournals.org.
Profound hypoxaemia corrected by PFO closure device in carcinoid heart disease
Philip M. Mottram1,2,*,
David J. McGaw1,2,
Ian T. Meredith1,2,
Roger E. Peverill1,2 and
Richard W. Harper1,2
1 Monash Medical Centre, Department of Cardiology, Melbourne, Australia
2 Monash University, Department of Medicine, Melbourne, Australia
Received 15 July 2006; accepted after revision 11 August 2006; online publish-ahead-of-print 4 October 2006.
* Corresponding author. Department of Cardiology, Monash Medical Centre, 246 Clayton Road, Clayton 3168, Melbourne, Australia. Tel: +61 3 9594 2242; fax: +61 3 9594 6239. E-mail address: philip.mottram{at}med.monash.edu.au
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Abstract
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A 66-year-old man with known metastatic carcinoid tumor presented
with increasing dyspnoea, right heart failure and marked hypoxaemia
which did not correct with oxygen. Echocardiography demonstrated
severe tricuspid regurgitation, moderate pulmonary regurgitation
and marked right heart dilatation. The inter-atrial septum was
aneurysmal, with a large patent foramen ovale (PFO) with continuous
right to left shunting. Cardiac catheterization demonstrated
oxygen saturations of 96% in the pulmonary veins and 74% in
the left atrium with a significant right to left shunt. During
percutaneous closure of the PFO, anaesthetic induction resulted
in marked systemic hypotension and worsening hypoxia related
to systemic vasodilatation and increased shunting. PFO flow
was temporarily obstructed with a sizing balloon resulting in
a rapid increase in arterial oxygen saturation from 60% to >90%,
but marked systemic hypotension due to acute left ventricular
preload reduction, requiring volume replacement and adrenaline.
Following deployment of a PFO occluder device, prominent pulsatile
splaying of the right and left discs was noted due to the severe
tricuspid regurgitation, resulting in some residual inter-atrial
shunting. Arterial oxygen saturation was 83%, increasing to
92% at day 4 post-procedure as tissue organization occurred
within the device, and the patient reported improvement in dyspnoea.
Keywords: Valves; Shunts; Hypoxia
A 66-year-old man presented with increasing dyspnoea, exercise intolerance, and peripheral oedema, on a background of known metastatic carcinoid tumor. Examination revealed central cyanosis, a dominant v-wave in the neck veins, a pan-systolic murmur at the left sternal border and pitting lower limb oedema. Blood gas analysis revealed marked hypoxaemia, with arterial partial oxygen pressure 42 mmHg which did not correct with 12 L/min oxygen by mask. Metastatic carcinoid tumour involving the liver had been confirmed by computed tomography and biopsy with typical histology and positive immunoperoxidase stains for chromogranin and synaptophysin. No primary tumor was found and no pulmonary involvement was evident. Symptoms of flushing and diarrhoea had been present for 2 years and were treated with octreotide, a somatostatin analogue. Transthoracic and transesophageal echocardiography revealed marked thickening of the right heart valves resulting in severe tricuspid regurgitation, moderate pulmonary regurgitation, marked right heart dilatation and right ventricular systolic dysfunction (Figure 1). The inter-atrial septum was aneurysmal and associated with a large patent foramen ovale (PFO) with continuous right to left shunting (Figure 2). Left ventricular systolic function was normal. There was mild thickening, restriction and regurgitation of the aortic valve, and mild mitral valve thickening. Such a pattern of left-sided valve involvement is typically seen in the presence of either pulmonary metastases or an intracardiac shunt,1 presumably because of bypass of trans-pulmonary inactivation of humoral mediators secreted by the tumour.

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Figure 1 Transoesophageal echocardiographic images: (A) severe tricuspid valve disease with thickened, retracted leaflets fixed in a partially open position (arrows); (B) 4-chamber view demonstrating right heart dilatation and aneurysmal inter-atrial septum (arrow); (C) severe tricuspid regurgitation. RA, right atrium; LA, left atrium; RV, right ventricle.
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Cardiac catheterization revealed oxygen saturations at the following
locations: right atrium and right ventricle 37%, pulmonary vein
96%, left atrium and aorta 74%. Pulmonary artery pressure was
35/15 mmHg. The pulmonary to systemic flow ratio (QP:QS) was
0.4:1. These findings represented a significant right to left
shunt at the atrial level. Signs of right heart failure responded
to diuresis; persistent dyspnoea was thought to be primarily
related to profound hypoxaemia from the intracardiac shunt.
Percutaneous closure of the PFO was considered the most appropriate
intervention and was performed under general anaesthetic with
transoesophageal echocardiographic guidance. Anaesthetic induction
resulted in marked systemic hypotension and a fall in arterial
oxygen saturation from 73% to 45%, presumably related to
systemic vasodilatation and increased shunting, and both responded
to intravenous volume replacement and adrenalin. During the
procedure, PFO flow was temporarily obstructed with a 24-mm
Amplatzer sizing balloon (
Figure 3), resulting in a rapid
increase in arterial oxygen saturation from 60% to >90%.
Left ventricular diastolic volume decreased and was accompanied
by increased right ventricular dilatation. Systemic systolic
blood pressure fell from 130 mmHg to 75 mmHg consistent with
acute left ventricular preload reduction and responded to further
volume replacement and adrenaline. Following routine deployment
of a 35-mm Amplatzer PFO occluder device, prominent pulsatile
splaying of the right and left discs was noted due to the effect
of the severe tricuspid regurgitation, resulting in some residual
inter-atrial shunting (
Figure 4). The post-deployment arterial
oxygen saturation of 83% was moderately improved, and considered
acceptable given the expectation that the shunt would decrease
further as tissue organization occurred within the device. Indeed,
at day 4 post-procedure, oxygen saturation was 92% on room
air and the patient reported marked improvement in dyspnoea.

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Figure 4 PFO occluder device in situ during diastole (A); Systolic images demonstrating marked systolic separation of the left and right atrial discs (arrow) (B), and intervening color flow (C). No inter-atrial shunting was visible with color Doppler (c), however a saline microbubble contrast study was positive for residual inter-atrial shunting (D).
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This case demonstrates that severe hypoxaemia due to right to
left shunting across an atrial septal aneurysm and patent foramen
ovale can be effectively treated percutaneously. It also demonstrates
real-time changes in cardiovascular physiology
in the catheterization laboratory, highlighting the procedural
risks of hemodynamic instability due to rapid reduction in left
ventricular preload caused by closure of a large right to left
shunt, and potential difficulties related to device deployment
in the setting of severe tricuspid regurgitation.
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Reference
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- Millward MJ, Blake MP, Byrne MJ, Hung J, Gibson P. Left heart involvement with cardiac shunt complicating carcinoid heart disease. Aust N Z J Med (1989) 19:716–7.[Web of Science][Medline]

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