Arterial malformations - Arteriology

Arterial malformations

What are arterial malformations

Abnormalities in development or stopping at a certain stage of evolution of the arterial vascular system in the fetal period of life trigger the appearance of congenital arterial malformations.

What are the most common arterial malformations?

1. Lusoria artery - a developmental abnormality of the right subclavian artery is more common in patients diagnosed with Down syndrome or other congenital heart abnormalities being the most common congenital malformation of the aortic arch.

Most people with this condition are asymptomatic, but depending on the severity of the malformation of the artery, symptoms may occur such as dyspnoea (shortness of breath), cough, stridor (noisy breathing and exhalation), shortness of breath, dysphagia, chest pain and aneurysmal dilation with risk of rupture (rupture of the aneurysm may be spontaneous or in case of intense physical exertion). If the right subclavian stenosis occurs during life, the clinical symptoms may be accompanied by claudication of the upper limb on the same side or by manifestations that draw attention to a cerebral embolism or digestive (upper) haemorrhage secondary to fistulization of the esophageal artery.

2. Rendu-Osler telangiectasia - is a rare disease that causes vascular dysplasia (change in the normal structure of the vascular wall) that causes recurrent bleeding. Hereditary damage is manifested in the form of:

  • Arteriovenous malformations - deficit in the formation of small capillary vessels that cause the passage of blood from the arterial level directly into the venous system;
  • Cutaneous mucosal telangiectasias (spider veins).

The symptoms associated with these malformations appear around the second decade of life and are usually hemorrhagic manifested in the form of epistaxis (bleeding of the nasal mucosa spontaneously or minor injuries). The presence of multiple vascular malformations in various organs and systems can manifest itself in the form of pulmonary, intracranial, hepatic hemorrhage or the spinal cord.

3. Persistence of the arterial canal - causes an increase in blood flow between the aorta and the pulmonary artery when the arterial duct, which separates the fetal pulmonary circulation, does not close physiologically at birth. Abnormal blood circulation between the lungs and the newborn's heart can lead to heart failure and other serious, life-threatening complications.

Symptoms of persistent ductus arteriosus are dyspnoea, fatigue, pulmonary hypertension, heart failure, and an increased risk of lung or persistent artery infections;

4. Aortic coarctation -  represents a narrowing of the lumen of the aorta more frequently at the isthmic level, less often at the intrathoracic segment. Similar to many birth defects, the malformation may be unique or may be associated with other cardiac abnormalities: persistent arterial canal, atrial/ventricular septal defect, and other complex cardiac abnormalities.

The malformation is manifested in newborns by the occurrence of congestive heart failure, secondary hypertension, respiratory failure, heart rhythm disorders or necrotic enterocolitis.

Older children may be at increased risk of stroke (specific neurological deficits occur), advanced left ventricular failure (a form of heart failure), high blood pressure in upper extremities, claudication in the lower limbs, infectious bacterial endocarditis and reduced life expectancy up to 35 years due to the listed complications.

  • Pulmonary artery stenosis represents 9% of all congenital heart malformations with predominantly female involvement;
  • Pulmonary artery atresia;
  • Common arterial trunk - a single artery leaves the ventricles which cause an increased volume of blood to the lungs (compared to the required amount). The condition develops with pulmonary blood stasis and pulmonary hypertension, the patient may complain of chronic fatigue or lethargy, a difficult diet with weight loss, tachypnea (increased respiratory rate) or dyspnea (shortness of breath).
  • Arteriovenous malformations (cerebral malformations) - congenital malformations due to the development of abnormal arterial or venous vascular conglomerates with a risk of causing intracerebral hemorrhagic accidents with clinical manifestations corresponding to epileptic seizures.

What treatment should be applied

Therapeutic conduct in the treatment of arterial malformations involves the application of surgical methods of reconstruction of the affected artery (re-establishment with or without a stent, bypass to reduce the phenomenon of "theft" of blood) or removal of aneurysms by classic or minimally invasive surgical techniques and telangiectasias can be reduced following laser ablation, cauterization or sclerotherapy.