Pulmonary arteriovenous malformations and other pulmonary aspects of hereditary haemorrhagic telangiectasia

@inproceedings{Shovlin2011PulmonaryAM,
  title={Pulmonary arteriovenous malformations and other pulmonary aspects of hereditary haemorrhagic telangiectasia},
  author={Claire L. Shovlin and Peter Wilmshurst and James E. Jackson},
  year={2011}
}
Pulmonary arteriovenous malformations (PAVMs) are vascular structures that provide a direct capillary-free communication between the pulmonary and systemic circulations. The majority of patients have no PAVM-related symptoms, but are at risk of major complications that can be prevented by appropriate interventions. More than 90% of PAVMs occur as part of hereditary haemorrhagic telangiectasia (HHT), the genetic condition most commonly recognised by nosebleeds, anaemia due to chronic haemorrhage… 

Figures and Tables from this paper

British Thoracic Society Clinical Statement on Pulmonary Arteriovenous Malformations
TLDR
The driver for the current Clinical Statement was the plethora of new data since previous hereditary haemorrhagic telangiectasia (HHT) guidelines generated in 2006 and a systematic Cochrane Review for PAVM embolisation in 2011.
Ischaemic Strokes in Patients with Pulmonary Arteriovenous Malformations and Hereditary Hemorrhagic Telangiectasia: Associations with Iron Deficiency and Platelets
TLDR
It is suggested that patients with compromised pulmonary capillary filtration due to pulmonary arteriovenous malformations are at increased risk of ischaemic stroke if they are iron deficient, and that mechanisms are likely to include enhanced aggregation of circulating platelets.
Silent threat? A retrospective study of screening practices for pulmonary arteriovenous malformations in patients with hereditary haemorrhagic telangiectasia.
TLDR
Safe and effective treatment exists for PAVMs although a significant minority of patients has received no screening to date, and Clinicians should refer all patients for screening regardless of symptoms.
Curable hypoxia in an octogenarian with an undiagnosed inherited condition: a case commentary
TLDR
The accompanying case is an excellent illustration of why pulmonary arteriovenous malformations (PAVMs) have been considered very rare and why, irrespective of respiratory symptoms, all PAVMs should be considered for treatment.
The Lung in Hereditary Hemorrhagic Telangiectasia
TLDR
The focus of this review is on delineating how HHT affects the lung.
THORAXJNL201027-127274corr2 80..84
TLDR
Individuals with pulmonary arteriovenous malformations and hereditary haemorrhagic telangiectasia commonly have low oxygen saturations and anaemia, two parameters generally used to indicate medical fitness to fly, and their inflight complications and predictors should influence preflight advice.
Curable hypoxia in an octogenarian with an undiagnosed inherited condition
TLDR
An 84-year-old gentleman was referred to the chest clinic with low oxygen saturations on pulse oximetry and was investigated using lung function testing, capillary blood gas sampling, a chest radiograph and thoracic computed tomography (CT).
A diver’s dilemma – a case report on bronchopulmonary sequestration
TLDR
The diver remains asymptomatic and well at the time of writing and has been accepted to participate in another overseas course involving only dry diving in a hyperbaric chamber, with no prerequisites for him to undergo surgery.
Arterial Oxygen Content Is Precisely Maintained by Graded Erythrocytotic Responses in Settings of High/Normal Serum Iron Levels, and Predicts Exercise Capacity: An Observational Study of Hypoxaemic Patients with Pulmonary Arteriovenous Malformations
TLDR
Haemoglobin and oxygen measurements in isolation do not indicate the more physiologically relevant oxygen content per unit blood volume, and Serum iron concentrations, not ferritin, seem to predict more successful polycythaemic responses.
...
...

References

SHOWING 1-10 OF 269 REFERENCES
Pulmonary arteriovenous malformations: how do I embolize?
  • Robert I. White
  • Medicine
    Techniques in vascular and interventional radiology
  • 2007
TLDR
Pulmonary arteriovenous malformations (PAVM) are high-flow, low-pressure shunts, consisting of a single feeding artery connecting via an aneurysmal sac to a draining vein, which are markers of hereditary hemorrhagic telangiectasia (HHT).
Pulmonary arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia.
TLDR
A screening algorithm based on the combined use of contrast echocardiography and anteroposterior chest radiograph, followed by chest CT if either test is positive, may obviate the need for chest CT in patients without PAVM.
Management of pulmonary arteriovenous malformations in pulmonary hypertensive patients: a pressure to embolise?
TLDR
Treatment of PAVMs by embolisation is an effective means of reducing lifetime risks of paradoxical embolic stroke and cerebral abscess, improving oxygenation, and treating PAVM-induced haemoptysis.
Pulmonary arteriovenous malformations: screening procedures and pulmonary angiography in patients with hereditary hemorrhagic telangiectasia.
TLDR
Initial screening with CE followed by measurement of PaO2 while breathing 100% oxygen seemed to be the best screening procedure for identification of patients with PAVM.
Hereditary haemorrhagic telangiectasia and pulmonary arteriovenous malformations: issues in clinical management and review of pathogenic mechanisms
Hereditary haemorrhagic telangiectasia (HHT, Rendu-Osler-Weber syndrome) exemplifies an important group of diseases which have catalysed advances in the understanding of fundamental
Pulmonary arteriovenous malformations. A state of the art review.
  • J. GossageG. Kanj
  • Medicine
    American journal of respiratory and critical care medicine
  • 1998
Embolotherapy for Pulmonary Arteriovenous Malformations in Patients with Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome)
TLDR
Embolotherapy is a definitive treatment forPAVMs: it is very effective with a high success rate and few complications, and patients with HHT are at risk of PAVM and should be screened and treated for PAVMs when these reach a size that is associated with complications.
Pulmonary arteriovenous malformation
TLDR
Pulmonary arteriovenous malformations (PAVM) are rare pulmonary vascular anomalies that can cause dyspnoea from right-to-left shunt and various central nervous system complications have been described including stroke and brain abscess.
Screening for pulmonary and cerebral arteriovenous malformations in children with hereditary haemorrhagic telangiectasia
TLDR
The present data suggest that children with a definite HHT diagnosis have a high frequency of pulmonary AVMs even when clinically asymptomatic, and genetic testing appears to be useful in defining an at-risk group for pulmonary AVM in childhood.
Pulmonary arteriovenous malformations
TLDR
The prevalence of single PAVM is greater for patients with multiple PAVM, suggesting increased predisposition for paradoxical embolization with a greater number of malformations.
...
...