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5. Case Study: Coarctation of the Aorta

By Marie Hanley, Vascular Scientist - South West

Introduction

Coarctation of the Aorta (CoA) refers to the narrowing of the aorta usually in the region of ligamentum arteriosum that leads to blood flow obstruction. It accounts 4–6% of all congenital heart defects.[1]  It is related to other cardiac as well as extracardiac conditions. It rarely presents in adulthood because of early detection in childhood, especially in developed countries.

It is associated with high morbidity and mortality. Average survival without treatment is 31 years. [2]

An anecdotal history statement describes the first diagnosed case of the coarctation of the aorta in Julia the daughter of the French poet Alphonse de Lamartine after the autopsy in 1832 in Beirut, the reference manuscript still exists in one of the Maronite monasteries in Mount Lebanon.

A 23 year old male with ongoing poorly controlled hypertension, was referred to the vascular laboratory by the GP for Renal artery Duplex scan. As a rule we do not normally accept direct referrals from the GP for this, however due to change in admin staff,the appointment was booked. He attended the vascular laboratory with his wife. He was slightly overweight and smoked 5-10 a day.

Duplex Examination

Duplex scanning of the aorta demonstrated a patent normal calibre aorta but with very damped flow signals with velocities measuring 55 cm/s.

The renal arteries were demonstrated well despite the patient’s size.  Both renal arteries had damped flow signals. The renal hilum, parenchymal and cortical flow signals were also obtained and were the flow signals were also damped bilaterally.

                           

 

 

 

 

 

 

 

 

 

 

The kidneys were normal in size and ultrasound appearance.

The CIA and EIA were patent and normal in calibre bilaterally but with damped monophasic flow signals.

 

 

 

 

 

 

 

 

 

 

 

 

The cardiology team, as well as the GP, were informed of the result and the patient was referred for more imaging.

Further investigations

A chest x-ray was booked to look for the typical signs of coarctation of the aorta which is the ‘FIGURE 3 SIGN’ which is demonstrated by an indentation of coarct and post stenotic aorta shown in the form of a ‘3’.

                                                 

MRA aorta thoracic reported

‘The lungs are clear. Biscuspid aortic valve was demonstrated with dilated aortic root and severe coarctation of the descending aorta. Normal atria and AV valves. Normal LV function with mild global hypertrophy.

Treatment

In adults and children found to have coarctation, treatment is conservative if asymptomatic, but may require surgical resection of the narrow segment if there is arterial hypertension. The first operations to treat coarctation were carried out by Clarence Crafoord in Sweden in 1944.[3] In some cases angioplasty /stenting can be performed to dilate the narrowed area, with or without the placement of a stent graft.

Side Effects

In a study of 120 coarctation repair recipients done in Groningen, The Netherlands, twenty-nine patients (25%) experienced hypertension in the later years of life due to the repair. While hypertension has many different factors that lead to this stage of blood pressure, people who have had a coarctation repair — regardless of the age at which the operation was performed — are at much higher risk than the general public of hypertension later in life.

Coronary artery disease (CAD) is a major issue for patients who have undergone a coarctation repair. Many years after the procedure is done, heart disease not only has an increased chance of affecting coarctation patients, but also progresses through the levels of severity at an alarmingly increased rate. In one study, one fourth of the patients who experienced a coarctation later died of heart disease, some at a relatively young age.[4][5]

People who have had a coarctation of the aorta are likely to have bicuspid aortic valve disease. Between 20% and 85% of patients are affected with this disease. Bicuspid aortic valve disease is a big contributor to cardiac failure, which in turn makes up roughly 20% of late deaths to coarctation patients. [6]       

References             

1. Reller MD, Strickland MJ, Riehle-Colarusso T, et al. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr 2008;153:807.

2. Campbell M. Natural history of coarctation of aorta; Br Heart J 1970;32(5):633–40.

3. Valdes-Cruz, Lilliam M.; Cayre, Raul O., eds. (1999). Echocardiographic Diagnosis of Congenital Heart Disease: An Embryologic and Anatomic Approach. Philadelphia: Lippincott Williams & Wilkins. ISBN 978-0-7817-1433-4.[page needed]

4. Cohen, M.; Fuster, V.; Steele, P. M.; Driscoll, D.; McGoon, D. C. (1989). "Coarctation of the aorta. Long-term follow-up and prediction of outcome after surgical correction". Circulation. 80 (4): 840–5. doi:10.1161/01.CIR.80.4.840. PMID 2791247.

5. Di Salvo, G; Castaldi, B; Baldini, L; Gala, S; del Gaizo, F; D'Andrea, A; Limongelli, G; D'Aiello, A F; Scognamiglio, G; Sarubbi, B; Pacileo, G; Russo, M G; Calabrò, R (2011). "Masked hypertension in young patients after successful aortic coarctation repair: impact on left ventricular geometry and function". Journal of Human Hypertension. 25 (12): 739–45. doi:10.1038/jhh.2010.118. PMID 21228825.

6. Giuffre, Michael; Ryerson, Lindsay; Chapple, Denise; Crawford, Susan; Harder, Joyce; Leung, Alexander K. C. (2005). "Nonductal dependent coarctation: a 20-year study of morbidity and mortality comparing early-to-late surgical repair". Journal of the National Medical Association. 97 (3): 352–6. PMC 2568624. PMID 15779499.