Vascular Malformation is a general term that includes congenital vascular anomalies of only veins, only lymph vessels, both veins and lymph vessels, or both arteries and veins:

  • Only veins: Venous Malformations (VM)
  • Only lymph vessels: Lymphatic Malformations (LM)
  • Both veins and lymph vessels: Venolymphatic Malformations (VLM)
  • Arteries connected directly to veins without any capillaries in between: Arteriovenous Malformations (AVM)

 

These are all present at birth, and become apparent at different ages. We are just beginning to understand how malformations occur. The pulmonary arteriovenous malformation, when associated with Hereditary Hemorrhagic Telangiectasia, is inherited genetically. There is currently much work being done on the possible genetics of other malformations. Most are only known as something that occurs during development of the arteries, veins, and/or lymph vessels, but without specific cause.

What Are Symptoms of Vascular Malformation?

These vascular malformations can cause a variety of symptoms, depending on the location in the body:

Venous malformations (VMs) may cause pain wherever they are located. VMs and lymphatic malformations (LMs) may cause a lump under the skin. There may be an overlying birthmark on the skin. Bleeding or lymph fluid leaking may occur from skin lesions. LMs tend to become infected, requiring repeated antibiotic treatments. VMs and LMs may be associated with a syndrome called Klippel-Trenaunay Syndrome.

Arteriovenous malformations (AVMs) may cause pain. They are also more stressful on the heart because of the rapid shunting of blood.

Pulmonary arteriovenous malformations (PAVMs) are somewhat different in that they shunt blood from the right heart system to the left heart system without picking up oxygen in the lungs. This results in symptoms of low oxygen, shortness of breath, and fatigue. These malformations may also bleed, resulting in coughing up blood or blood in the chest. Also, these pulmonary artery to pulmonary vein shunts can allow clots to pass through the lungs and travel to the arteries in the body, with risk for stroke or brain abscess. This is a very significant reason to block these shunts.

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What Are Symptoms of Vascular Malformation?

These vascular malformations can cause a variety of symptoms, depending on the location in the body:

Venous malformations (VMs) may cause pain wherever they are located. VMs and lymphatic malformations (LMs) may cause a lump under the skin. There may be an overlying birthmark on the skin. Bleeding or lymph fluid leaking may occur from skin lesions. LMs tend to become infected, requiring repeated antibiotic treatments. VMs and LMs may be associated with a syndrome called Klippel-Trenaunay Syndrome.

Arteriovenous malformations (AVMs) may cause pain. They are also more stressful on the heart because of the rapid shunting of blood.

Pulmonary arteriovenous malformations (PAVMs) are somewhat different in that they shunt blood from the right heart system to the left heart system without picking up oxygen in the lungs. This results in symptoms of low oxygen, shortness of breath, and fatigue. These malformations may also bleed, resulting in coughing up blood or blood in the chest. Also, these pulmonary artery to pulmonary vein shunts can allow clots to pass through the lungs and travel to the arteries in the body, with risk for stroke or brain abscess. This is a very significant reason to block these shunts.

Vascular Malformation Care in Atlanta, GA

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Arteriovenous Malformation Embolization

The embolization technique simply uses a thin, flexible tube, called a catheter, to introduce a substance into a blood vessel to cause a clot and permanently block off that blood vessel. This can be done without staples or stitches, and with only mild sedation. Several types of embolic substance, including sclerosants, small beads, or metal coils, are used to block flow to the malformation.

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Hereditary Hemorrhagic Telanglectasia (HHT) Treatment

The location of the telangiectases or AVM impacts what problems a person with HHT might face. A person with abnormal GI tract blood vessels should watch for black stools and anemia that can cause fatigue, shortness of breath, chest pain, or lightheadedness. Those with AVMs in the lung face the possibility of a life threatening rupture, stroke, or brain abscess—especially during pregnancy. HHT patients with brain AVMs do not generally have warning symptoms prior to a life-threatening brain hemorrhage. Heart failure is another risk HHT patients face due to high-flow AVMs.

Regardless of location or size, abnormal blood vessels have a greater tendency to rupture and bleed compared to normal blood vessels and should be closely monitored.

Those with HHT may have blood vessel abnormalities in the:

  • Nose
  • Skin of the face, hands, and mouth
  • Lungs
  • Brain
  • Lining of the stomach and intestines (GI tract)
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Pulmonary Arteriovenous Malformation Treatment

These malformations should be treated, even prior to symptoms appearing, due to their life threatening/disabling nature. Interventional radiologists, vascular experts who treat patients internally using imaging to guide them, can permanently treat lung AVMs using a minimally invasive procedure known as embolization. During this procedure, an interventional radiologist makes a small nick in the skin, inserts a catheter into the femoral vein in the groin and then directs it to the abnormal blood vessels in the lung. Next, Dacron platinum coils are placed in the artery feeding the AVM to permanently block the blood flow to the AVM. Patients should be seen one year after treatment by the interventional radiologist to be sure the AVM has been resolved. Embolization is performed with local anesthesia and conscious sedation.

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