Right ventricular diastolic dysfunction and patent foramen ovale causing profound cyanosis

Abstract

A 73 year old woman presented with profound central cyanosis and a history of a minor stroke. She had normal heart morphology, normal pulmonary artery pressure, and a normal coronary angiography. A patent foramen ovale (PFO) with a massive right to left shunt was demonstrated at the atrial level, with normal pulmonary venous saturations and PO 2 values. This rare, age related case of right ventricular diastolic dysfunction in a normotensive patient revealed a generous PFO allowing a pronounced right to left shunt.A 73 year old woman presented with recent onset cyanosis and a past history of a minor stroke. Room air arterial PO 2 was 40 mm Hg. Transoesophageal echocardiography revealed a patent foramen ovale (PFO) with a large right to left shunt. No other anomaly was seen.Cardiac catheterisation disclosed normal coronary arteries and normal pulmonary pressure. Right ventricular (RV) diastolic pressure was slightly higher than left ventricular (LV) diastolic pressure. On 100% Fi O 2 , pulmonary venous P O 2 was 540 mm Hg and the left atrial P O 2 was 97 mm Hg. Balloon occlusion of the PFO served to test the tolerance of PFO occlusion and to measure the effective stretched defect size. Immediate post-transcatheter PFO closure using a 24 mm atrial septal defect (ASD) Amplatzer occluder resulted in a rise of arterial PO 2 to 320 mm Hg. Other causes of the atrial level right to left shunt such as pulmonary disease, pulmonary vascular disease, RV hypertrophy, RV systolic dysfunction, right atrial myxoma, tricuspid valve disease, and pericardial effusion were excluded. This rare, age related case of RV diastolic dysfunction in a normotensive patient revealed a generous PFO allowing a pronounced right to left shunt. DISCUSSIONA persistent right to left shunt at the atrial level across a PFO has previously been described in relation to RV dysfunction or elevation of the pulmonary pressure in conditions such as chronic obstructive pulmonary disease, or pulmonale, recurrent pulmonary embolism, tricuspid valve dysfunction, RV infarction, and right sided myxoma.1 A significant right to left shunt via PFO has rarely been described without underlying cardiac or pulmonary disease.2-4 Several theoretical pathophysiological explanations were offered to explain such a phenomenon. Preferential streaming of the blood from the inferior vena cava across the PFO and transatrial septal pressure gradients were suggested as the likely cause for the haemodynamic state. This is a case report of a 73 year old woman who presented with profound central cyanosis and a history of a minor stroke. She had normal heart morphology, normal pulmonary artery pressure and a normal coronary angiography. A massive right to left shunt was demonstrated at atrial level with normal pulmonary venous saturations and PO 2 values. The reason for this huge right to left shunt at the PFO level is illustrated by the diastolic pressure curves representing compliance differences between right and left ventricles (fig 1). This is probably an age related phenomenon in t...

Hypoxic adaptation during development: relation to pattern of neurological presentation and cognitive disability

“…Possible mechanisms for covert infarction include residua of acute hypoxic posterior leukencephalopathy, cerebral oedema and basal ganglia infarction (Henderson et al , 2003; Usui, Inoue, Kimura, Kirino, Nagaoka, Abe, Nagata & Arai, 2004; Jeong, Kwon, Chin, Yoon & Na, 2002) and venous sinus thrombosis (Sébire et al , 2005) as well as transient ischaemic attack secondary to arterial disease. Patent foramen ovale (PFO) is a well‐recognized cause of sustained and intermittent hypoxia common in patients with right ventricular dysfunction, obstructive airways disease and OSA (Shnaider, Shiran & Lorber, 2004; Soliman, Shanoudy, Liu, Russell & Jarmukli, 1999; Shanoudy, Soliman, Raggi, Liu, Russell & Jarmukli, 1998), and is a potentially treatable cause of stroke and migraine in young adults (Finsterer, Sommer, Stiskal, Stollberger & Baumgartner, 2005). However, the possibility that PFO is a risk factor for overt or covert infarction in SCD has received little attention (Dowling, 2005).…”

Recurrent episodic hypoxaemic respiratory failure following a stroke

“…The potential importance of RV dysfunction in RLIAS is also supported by a case report of profound cyanosis in which the only predisposing factor to RLIAS was right-ventricular diastolic dysfunction 29. Milder degrees of RV dysfunction may be insufficient to cause RLIAS, but sufficient to increase susceptibility to RLIAS with provocation.…”

Patent Foramen Ovale: A Primer for Headache Physicians

“…Paradoxical embolization to the retina, coronary arteries, spleen, kidneys, peripheral arteries, and the splanchnic bed, in patients as young as 6 years of age, have been seen with no attributable source other than a PFO. Clinical cyanosis, owing to large right to left shunts at the atrial level, have been associated with PFO in some patients 28–31 . This large shunt can be the result of right ventricular noncompliance, tricuspid valve disease, or the platypnea–orthodeoxia syndrome (cyanosis which is variable in association with changes in position).…”

“…Clinical cyanosis, owing to large right to left shunts at the atrial level, have been associated with PFO in some patients. [28][29][30][31] This large shunt can be the result of right ventricular noncompliance, tricuspid valve disease, or the platypnea-orthodeoxia syndrome (cyanosis which is variable in association with changes in position). Decompression illness in divers has been associated with the presence of PFO, presumably related to a right to left shunt of air bubbles from the venous circulation.…”