How serious is a pulmonary AVM?
Pulmonary symptoms include dyspnea, fatigue, cyanosis, and orthodeoxia (decreased arterial oxygen content while upright), all due to right-to-left shunting of blood through the pulmonary AVM. The most serious complications of pulmonary AVMs are potentially fatal hemoptysis or hemothorax (in up to 10% of patients).
How is pulmonary AVM treated?
The majority of PAVM are treated by a technique called embolization which blocks the feeding arteries to the PAVM. Rarely when there is a very extensive abnormality located in a single part of the lung this may be treated by a surgical operation to remove this area.
What is pulmonary AVM?
Pulmonary arteriovenous malformations (PAVMs) are abnormal vascular structures that most often connect a pulmonary artery to a pulmonary vein, bypassing the normal pulmonary capillary bed and resulting in an intrapulmonary right-to-left shunt.
What causes AVMs in the lungs?
Certain types of AVMs can occur after penetrating trauma such as stab wounds or projectile injuries. Most AVMs are not inherited, but AVMs may occur as part of heritable genetic diseases such as Hereditary Hemorrhagic Telangiectasia (HHT).
Is an AVM an embolism?
AVM (arteriovenous malformation) embolization reduces the number of abnormal connections between blood vessels that occur in the brain. An AVM causes blood to flow directly from the arteries to the veins without supplying blood to vital tissues.
Can a pulmonary AVM cause shortness of breath?
Symptoms vary and many patients with pulmonary AVM are asymptomatic. With larger or multiple pulmonary AVMs, shortness of breath may be present, especially with exertion.
What does the Fontan procedure do?
The Fontan procedure refers to any surgical procedure that leads to systemic flow of venous blood to the lungs without passing through a ventricle.
When is the Fontan procedure performed?
The Fontan procedure is the third stage of the repair. It is done when the child is between 18 months and 2 years of age. A heart catheterization is done before the Fontan.
Why does pavm develop after a Fontan operation?
PAVM might develop after the establishment of the Fontan circulation due to the unbalanced pulmonary perfusion of HV blood flow [1]. A few studies have found that revision of the Fontan pathway after a Fontan operation by redirection of HV blood flow causes PAVM resolution in patients with LI and absent IVC [12–14].
How old is the average patient after a Fontan operation?
Median age at Fontan operation was 6.2 years (1.0–24.1). Diagnosis of pulmonary arteriovenous malformations was made by pulmonary angiography and bubble contrast echocardiography. Results: Median follow-up was 5.3 years (0.2–17.3) with one hospital death.
How many patients suffer from pulmonary arteriovenous malformations?
Seven patients suffered from pulmonary arteriovenous malformations. In six out of the seven patients, the occurrence of pulmonary arteriovenous malformations was related to biased pulmonary perfusion of the hepatic venous flow.
How is Fontan related to pulmonary vasoconstriction?
Furthermore, coincident with clinical and angiographic resolution of the PAVMs, the central venous pressure increased to a more normal Fontan level (12 mm Hg), suggesting progressive pulmonary vasoconstriction.