Coarctation of the aorta

This article discusses the clinical and laboratory findings of isolated coarctation of the aorta, with emphasis on the newer methods of diagnosis and treatment, as well as the long term natural history following surgical repair.     

Coarctation of the aorta

Opinion statement  

Coarctation of the aorta

Coarctation of the aorta is an important, treatable cause of secondary hypertension. Its prevalence varies from 5% to 8% of all congenital heart defects. This condition is most often detected because of a murmur or hypertension found on routine examination. Delayed or absent femoral pulses and an arm/leg systolic blood pressure difference of 20 mm Hg or more in favor of the arms may be considered as evidence for aortic coarctation. The coarctation may be demonstrated on a suprasternal notch two-dimensional echocardiographic view along with increased Doppler flow velocities across the coarctation site. Cardiac catheterization reveals significant systolic pressure gradient (> 20 mm Hg) across the coarctation and angiography demonstrates the degree and type of aortic narrowing. Aortic obstruction may be relieved by surgery or by transcatheter techniques; the latter include balloon angioplasty and stent implantation. In the past, surgery has been used exclusively, but because of morbidity and complications associated with surgery, catheter techniques are increasingly used in the management of aortic coarctation. Balloon angioplasty in children and stents in adolescents and adults are becoming initial therapeutic options for management of coarctation. Studies evaluating long-term follow-up results of the interventional techniques are needed.     

Pseudocoarctation of the aorta

In this report 5 patients between the ages of 5-14 years admitted to the Department of Pediatric Cardiology, Hacettepe University, and diagnosed as having pseudocoarctation of the aorta are presented. The clinical and angiographic findings of this rare abnormality of childhood are discussed, the importance of differential diagnosis of this entity from coarctation of the aorta is emphasized, and the literature reviewed.     

Tandem coarctation of the thoracic aorta with hypoplasia of the abdominal aorta

After resection of the coarctation in a 3-year-old child with end-to-end anastomosis, a second membrane was found 1.5 cm lower. After a successful reoperation with an aortoplasty using Gore-Tex weak femoral pulses persisted. At catheterization, an area of irregular narrowing of the abdominal aorta just distal to the superior mesenteric artery was found with hypoplasia of the iliac and femoral arteries. In addition, this child had a two-sided cheilo-gnatho-palatoschisis, vesicoureteral reflux and absence of gall bladder and hepato-duodenal ligament. It is worthwhile considering that a second or third aortic abnormality can be hidden by a coarctation of the aorta.     

Angiosarcoma of the aorta

Primary aortic tumors are extremely rare. A 73-year-old woman presented with a 8 kg weight loss associated with abdominal pain. Physical examination was normal. Laboratory tests disclosed increased acute phase reactants. Thoracic and abdominal CT scan showed diffuse splenic and renal hypodense lesions with thrombotic feature of the thoracic aorta extending on 9 cm length. Transesophageal echocardiography showed a large and heterogeneous floating mass advocating a thrombus developed on atheroma. Because of the high risk of embolism the patient underwent surgical replacement of the thoracic aorta. Histopathology revealed an epithelioid angiosarcoma of the aorta. A primary tumor of the aorta should be suspected in the presence of an intra-aortic process presenting features of thrombosis.     

Imaging of the aorta

The aorta is an integral part of the cardiovascular system and should not be considered as just a conduit for blood supply from the heart to the limbs and major organs. A range of important pathologies affect the aorta and are responsible for a high level of morbidity and mortality in affected patients. Many of these conditions are seen in the adult congenital population, especially as advances in diagnosis and treatment mean these patients are surviving well into adulthood. As we gain a greater understanding of these disorders, especially the underlying genetics and pathophysiology, it becomes clear that the aorta is a highly complex part of the vascular tree. As such, the aorta requires increasingly sophisticated imaging techniques for the diagnosis, treatment and follow-up of these patients. The advantages and disadvantages of the various imaging techniques available to clinicians will be discussed in the context of both acute and chronic aortic pathology.