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European Journal of Echocardiography 2008 9(1):47-49; doi:10.1016/j.euje.2006.08.003
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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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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.


Figure 1
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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.

 


Figure 2
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Figure 2 (A) Transoesophageal echo image demonstrating large patent foramen ovale (PFO) (arrow); (B) colour flow imaging demonstrating large right to left shunt across PFO. RA, right atrium; Ao, aorta.

 
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.


Figure 3
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Figure 3 Cine image of Amplatzer sizing balloon positioned across the PFO, with the ‘waist’ of the balloon (arrow) indicating the site of occlusion of the defect.

 


Figure 4
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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).

 
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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  1. 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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