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Pulmonary arteriovenous malformations

Key sources
The following summarized guidelines for the evaluation and management of pulmonary arteriovenous malformations are prepared by our editorial team based on guidelines from the Hereditary Haemorrhagic Telangiectasia Working Group (HHT-WG 2020), the British Thoracic Society (BTS 2017), and the American College of Radiology (ACR 2016).
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Guidelines

1.Screening and diagnosis

Indications for screening, patients, HHT-WG: screen all patients with possible or confirmed HHT for PAVMs.
B
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  • Indications for screening (family relatives)

2.Diagnostic investigations

Diagnostic imaging
As per HHT-WG 2020 guidelines:
Consider obtaining contrast TTE as initial screening test for PAVMs.
C
Obtain low-dose non-contrast chest CT for diagnostic testing in pregnant patients with HHT and symptoms suggestive of PAVM (at any gestational age, as clinically indicated).
B

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  • Screening for HHT

3.Respiratory support

Supplemental oxygen
Recognize that despite oxygenation parameters that would fulfil requirements for supplementary oxygen in other clinical settings, patients with hypoxemia due to PAVMs usually display good exercise capacity, flight tolerance and successful pregnancies, assuming normal physiological compensations can operate.
E
Do not offer supplementary oxygen in asymptomatic, hypoxemic patients with PAVMs.
D

4.Medical management

Antiplatelet therapy
Consider initiating antiplatelet agents in patients with PAVMs, as they are at increased risk of ischemic stroke.
E
Recognize that the safety of thrombolysis is not established in patients with PAVMs.
E

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  • Preprocedural antibiotic prophylaxis

5.Nonpharmacologic interventions

Dental hygiene: advise judicious dental hygiene in patients with PAVMs.
E

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  • Iron supplementation

  • Rest

6.Therapeutic procedures

Transcatheter embolotherapy: as per HHT-WG 2020 guidelines, perform transcatheter embolotherapy for the treatment of PAVMs in patients with HHT.
B

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  • Therapeutic venesection

7.Surgical interventions

Lung resection: consider performing limited surgical resection if patients with PAVMs are unsuitable for embolization.
E

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  • Lung transplantation

8.Specific circumstances

Pediatric patients: treat large pulmonary ateriovenous malformations and pulmonary ateriovenous malformations associated with reduced oxygen saturation in pediatric patients to avoid serious complications.
B

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  • Pregnant patients

9.Patient education

General counseling: provide the following long-term advices in patients with documented pulmonary ateriovenous malformations (treated or untreated):
take antibiotic prophylaxis for procedures with risks of bacteremia
take extra care to avoid IV air when IV access is in place
avoid scuba diving
B

10.Follow-up and surveillance

Follow-up, general principles, HHT-WG: provide long-term follow-up in patients with pulmonary ateriovenous malformations in order to detect growth of untreated pulmonary ateriovenous malformations and also reperfusion of treated ateriovenous malformations.
B

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  • Follow-up (treated PAVMs)

  • Follow-up (residual or untreated PAVMs)