Author(s)

Galanis S, Fidanis T, Iatrou K, Palladas P, Kalpakidis V

Patient

male, 56 year(s)

A 56-year-old male patient with a known history of Hereditary Hemorrhagic Telangiectasia (HHT) and multiple symptomatic Pulmonary Arteriovenous Malformations (PAVM) was referred to our department for evaluation and treatment.

We present a case of a 56-year-old male already diagnosed with HHT and multiple PAVM’s. The patient suffered from dyspnoea, cyanosis, clubbing and fatigue. He had SaO2<97% in room air. He was transferred to the angiosuite for DSA of the right pulmonary artery, as he was previously evaluated with CT angiography which revealed the presence of four PAVM in the right lung. Two PAVM in the right middle lobe shown in Fig. 1 from which we decided to embolise the larger one using the Amplatzer Vascular Plug (AVP) leaving the other one to be embolised in a second session. There was also a large PAVM in the right lower lobe which we treated again with the AVP (Fig. 2). Another smaller PAVM was depicted in the right upper lobe left which was left to be embolised in a second session (Fig. 3). The patient was discharged the day after with no complications and was scheduled for the second session in one month.

PAVM are congenital communications between a pulmonary artery and a pulmonary vein allowing a right to left shunt. Up to 80% are associated with HHT and about 15-30% of individuals with HHT have PAVM. PAVM cause hypoxemia, dyspnoea, cyanosis, and fatigue due to the absence of oxygenation of venous blood. The absence of normal filtering of the capillary bed can cause paradoxical embolism and neurological complications such as strokes and brain abscesses. PAVM can also rupture leading to haemoptysis and haemothorax. The current preferred treatment for PAVM consists of embolisation using coils or other intravascular devices such as the Amplatzer Vascular Plug used in our case. PAVM with a feeding artery >3mm are considered to be of clinical importance and should be embolised.
Technique of embolisation: Bilateral pulmonary arteriograms in frontal and oblique projections are obtained using a Grollman or a standard pigtail catheter. Then a 6Fr 90cm long straight sheath combined with a 5Fr Headhunter is used for selective catheterization of the feeding vessel and angiograms in different projections to show the “neck” are obtained. Then the right size AVP is introduced through the sheath and delivered to the feeding artery beyond normal pulmonary artery branches to achieve embolisation. AVP has many advantages over coils: With AVP, occlusion of large vessels is possible using a single device when the same vessel would require the use of multiple coils to achieve the same result. The position of the AVP device is easily assessed before release and can be easily retrieved if too small to occlude the vessel. In addition over-sizing does not displace the catheter tip (like coils do) from the intended site of occlusion and therefore it is much easier to choose the suitably sized device. Also recanalisation is less likely to happen when using AVP as it has several layers of braiding producing a much greater cross-sectional wire coverage of vessel lumen than the most ideally positioned coil “nest”. Coils may be necessary to occlude small feeding branches. The complications reported after embolisation of PAVM’s are: pleurisy mainly in large PAVM’s (13%), pulmonary infarction as a result of occlusion of normal pulmonary branches if the device is placed too proximal (3%). There has also been reported a small percentage (4%) of air embolism from the peripheral lines and wrongly flushed catheters.