Near-fatal haemorrhage from pulmonary arteriovenous malformation in HHT with increased cardiac output: FIGURE 1.

Abstract

We read with interest the report by MONTANI et al.[1] of fatal rupture of pulmonary arteriovenous malformation (AVM) in a patient with hereditary haemorrhagic telangiectasis (HHT) and severe pulmonary arterial hypertension (PAH) confirmed by right heart catheterisation. The authors hypothesised that bleeding from pulmonary AVM may have been facilitated by increased mean pulmonary artery pressure (Ppa). Here, we report a patient with near-fatal haemorrhage from pulmonary AVM, probably facilitated by increased cardiac output, but without severe PAH.A 35-yr-old female nonsmoker, with a history of obesity and fenfluramide use 10 yrs previously for 2 months, was admitted for exertional dyspnoea during pregnancy. Arterial blood gases showed arterial oxygen tension (Pa,O 2 ) of 8.2 kPa, and arterial carbon dioxide tension of 3.5 kPa, with markedly increased alveolar-arterial oxygen tension difference on 100% oxygen (74 kPa, normally ,18.6 kPa). Chest radiograph revealed numerous pulmonary AVMs. Transthoracic contrast echocardiography showed estimated systolic Ppa of 47 mmHg with moderate dilatation of the right heart cavities, and extracardiac right-to-left shunting. She had recurrent epistaxis since childhood, numerous characteristic telangiectasiae, and possible familial HHT in her paternal grandmother. HHT was diagnosed according to the Curaçao criteria [2]. One deletion was found in exon 9 of the ENG gene, and analysis of the ACVRL1 gene was normal. Long-term oxygen therapy was initiated. Emergency caesarean delivery was performed at 25 weeks of gestation because of a fetal growth defect.

The Lung in Hereditary Hemorrhagic Telangiectasia

“…These are rare, reflecting the low pressures of the pulmonary circulation, but they are more common (1) in pregnancy (see below) [56,57] , (2) if PAVMs acquire a systemic arterial feeder after PAVM embolization [58] , or (3) in the setting of PH [31,59,60] . Emergency PAVM treatment by embolization or surgery is effective.…”

“…Thus, as practiced some years ago [124] , while vaso-occlusion of PAVMs in patients with known PH is technically possible, for patients with severe PH, based on current evidence, we would not usually interpret risk-benefit considerations to be in favor of PAVM embolization unless a patient had life-threatening hemoptysis or hemothorax. The risk of a rapid increase in size of PAVMs and of hemorrhage related to PAVM rupture might be heightened in individuals with severe PH [60] .…”

Persistent hypoxaemia after a pneumonia: a rare cause to consider

“…17 19-23 Serious complications from PAVMs like massive haemoptysis and haemothorax are particularly more common in PAVMs>3 mm. [24][25][26] Risk-benefit analysis in adult patients are almost always in favour of treatment. [27][28][29] However,…”

Pulmonary arteriovenous malformations and other pulmonary aspects of HHT

“…Haemorrhage leading to haemoptysis or haemothorax is a relatively rare feature of PAVMs, with three important exceptions: (1) during pregnancy (discussed below); (2) in association with pulmonary hypertension 127 , and 3, in the presence of spontaneous or postembolization 90,128 systemic arterial blood supply to PAVM sacs.…”