Coarctation of the aorta

Coarctation of the aorta
Specialty	Medical genetics

Sketch showing heart with coarctation of the aorta. A: Coarctation (narrowing) of the aorta. 1:Inferior vena cava, 2:Right pulmonary veins, 3: Right pulmonary artery, 4:Superior vena cava, 5:Left pulmonary artery, 6:Left pulmonary veins, 7:Right ventricle, 8:Left ventricle, 9:Pulmonary artery, 10:Aorta

Schematic drawing of alternative locations of a coarctation of the aorta, relative to the ductus arteriosus. A: Ductal coarctation, B: Preductal coarctation, C: Postductal coarctation. 1: Aorta ascendens, 2: Arteria pulmonalis, 3: Ductus arteriosus, 4: Aorta descendens, 5: Truncus brachiocephalicus, 6: Arteria carotis communis sinistra, 7: Arteria subclavia sinistra

Coarctation of the aorta, or aortic coarctation, is a congenital condition whereby the aorta narrows in the area where the ductus arteriosus (ligamentum arteriosum after regression) inserts. Aortic coarctation is considered when a section of the aorta is narrowed to an abnormal width. The word “coarctation” means narrowing. Coarctations are most common where the aorta — the major artery leading away from the heart — arches toward the abdomen and legs. The aortic arch may be small in babies with coarctations. Other cardiac defects may also occur when coarctation is present, typically occurring on the left side of the heart. When a patient has a coarctation, the left ventricle has to work harder. Since the aorta is narrowed, the left ventricle must generate a much higher pressure than normal in order to force enough blood through the aorta to deliver blood to the lower part of the body. If the narrowing is severe enough, the left ventricle may not be strong enough to push blood through the coarctation, thus resulting in lack of blood to the lower half of the body. Physiologically its complete form is manifested as Interrupted aortic arch.

Prevention

Unfortunately, coarctations can not be prevented because they are usually present at birth. The best thing for patients who are affected by coarctations is early detection. Some signs that can lead to a coarctation have been linked to things like Turner syndrome, bicuspid aortic valve, and other family heart conditions.

Types

There are three types:

1. Preductal coarctation: The narrowing is proximal to the ductus arteriosus. Blood flow to the aorta that is distal to the narrowing is dependent on the ductus arteriosus; therefore severe coarctation can be life-threatening. Preductal coarctation results when an intracardiac anomaly during fetal life decreases blood flow through the left side of the heart, leading to hypoplastic development of the aorta. This is the type seen in approximately 5% of infants with Turner Syndrome.
2. Ductal coarctation: The narrowing occurs at the insertion of the ductus arteriosus. This kind usually appears when the ductus arteriosus closes.
3. Postductal coarctation: The narrowing is distal to the insertion of the ductus arteriosus. Even with an open ductus arteriosus, blood flow to the lower body can be impaired. This type is most common in adults. It is associated with notching of the ribs (because of collateral circulation), hypertension in the upper extremities, and weak pulses in the lower extremities. Postductal coarctation is most likely the result of the extension of a muscular artery (ductus arteriosus) into an elastic artery (aorta) during fetal life, where the contraction and fibrosis of the ductus arteriosus upon birth subsequently narrows the aortic lumen.

Signs and symptoms

In mild cases, children may show no signs or symptoms at first and their condition may not be diagnosed until later in life. Some children born with coarctation of the aorta have other heart defects, too, such as aortic stenosis, ventricular septal defect, patent ductus arteriosus or mitral valve abnormalities.

Coarctation is about twice as common in boys as it is in girls. It is common in girls who have Turner syndrome.

Symptoms may be absent with mild narrowings (coarctation). When present, they include: difficulty breathing, poor appetite or trouble feeding, failure to thrive. Later on, children may develop symptoms related to problems with blood flow and an enlarged heart. They may experience dizziness or shortness of breath, faint or near-fainting episodes, chest pain, abnormal tiredness or fatigue, headaches, or nosebleeds. They may have cold legs and feet or have pain in their legs with exercise (intermittent claudication).

In more severe cases, where severe coarctations, babies may develop serious problems soon after birth because not enough blood can get through the aorta to the rest of their body. Arterial hypertension in the arms with normal to low blood pressure in the lower extremities is classic. Poor peripheral pulses in the legs and a weak femoral artery pulse may be found in severe cases.

The coarctation typically occurs after the left subclavian artery. However, if situated before it, blood flow to the left arm is compromised and asynchronous or radial pulses of different "strength" may be detected (normal on the right arm, weak or delayed on the left). In these cases, a difference between the normal radial pulse in the right arm and the delayed femoral pulse in the legs (either side) may be apparent, whilst no such delay would be appreciated with palpation of both delayed left arm and either femoral pulses. On the other hand, a coarctation occurring after the left subclavian artery will produce synchronous radial pulses, but radial-femoral delay will be present under palpation in either arm (both arm pulses are normal compared to the delayed leg pulses).

Imaging and diagnosis

With imaging, resorption of the lower part of the ribs may be seen, due to increased blood flow over the neurovascular bundle that runs there. Post-stenotic dilation of the aorta results in a classic 'figure 3 sign' on x-ray. The characteristic bulging of the sign is caused by dilatation of the aorta due to an indrawing of the aortic wall at the site of cervical rib obstruction, with consequent post-stenotic dilation. This physiology results in the '3' image for which the sign is named. When the esophagus is filled with barium, a reverse 3 or E sign is often seen and represents a mirror image of the areas of prestenotic and poststenotic dilatation.

Coarctation of the aorta can be accurately diagnosed with magnetic resonance angiography. In teenagers and adults echocardiograms may not be conclusive. In adults with untreated coarctation, blood often reaches the lower body through collaterals, e.g. internal thoracic arteries via the subclavian arteries. Those can be seen on MR, CT or angiography. An untreated coarctation may also result in hypertrophy of the left ventricle.

Your arteries can be checked for narrowing by your cardiologist by using an CT angiogram. This test is very easy on the patient because it is a minimally invasive procedure. The first step in the process is having a dye injected into the patient to help highlight the arteries for the CT scanner. While the CT scanner is imaging the arteries, images will be able to be viewed by the cardiologists almost immediately.

An MRI scan is a test that uses a magnetic field and pulses of radio wave energy to make pictures of your body. An MRI of your chest will reveal the location of the coarctation of the aorta and determine whether it affects other blood vessels in your body.

Cardiac catheterization is an invasive imaging procedure that is a tool for evaluating heart functions in real time. A long, narrow tube called a catheter is inserted through a plastic hollow tube called a introducer sheath. The introducer sheath is typically inserted into the patients arm or leg. The catheter is positioned through the patients blood vessel until it reaches the coronary arteries. Once the catheter is in position, a contrast dye is injected though the tip of the catheter while x-rays are taken to follow the dye through the chambers of the heart and arteries. This part of the procedure is called the coronary angiogram. Coronary artery disease occurs when there is a narrowing or blockage of the arteries leading to or from the heart. The cardiac catheterization is an interventional procedure that opens up the coronary artery to increase the blood flow to the heart. The contrast material injected into the arteries that help produce images of the heart help locate narrowed or blocked areas in arteries around the heart. There are other ways to produce images such as intra-vascular ultrasound (IVUS) and fractional flow reserve (FFR). these two different procedures give cardiologists more diverse options to best suit the patients needs.

coarctation of the aorta by CMR

A case of coarctation of the aorta was published in the New England Journal of Medicine in 2007 employing chest radiography and magnetic resonance images.

A case of long-standing misdiagnosed coarctation of the aorta in an adult was described in the New York Times Magazine.

Treatment

Therapy/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. In some cases angioplasty can be performed to dilate the narrowed artery. If the coarctation is left untreated, arterial hypertension may become permanent due to irreversible changes in some organs (such as the kidney).

For fetuses at high risk for developing coarctation, a novel experimental treatment approach is being investigated, wherein the mother inhales 45% oxygen three times a day (3 x 3–4 hours) beyond 34 weeks of gestation. The oxygen is transferred via the placenta to the fetus and results in dilatation of the fetal lung vessels. As a consequence, the flow of blood through the fetal circulatory system increases, including that through the underdeveloped arch. In suitable fetuses, marked increases in aortic arch dimensions have been observed over treatment periods of about two to three weeks. Complications of this treatment include left ventricular failure, aortic dissection, cerebral hemorrhage and progressive aortic regurgitation or stenosis.

The long term outcome is very good. Some patient may, however, develop narrowing (stenosis) or dilatation at the previous coarctation site. All patients with unrepaired or repaired aortic coarctation require follow up in specialized Congenital Heart Disease centers.

Post Procedure Life

Mortality Data

Looking at a study conducted by the Divisions of Pediatric Cardiology at the University of Minnesota School of Medicine, University of Connecticut Medical School, and University of Michigan where research was done on 252 survivors of the coarctation procedure, the age of the patient at the time of surgery is crucial in their longevity. Patients with the greatest chance of survival were those administered the procedure at very young ages, with children between the ages one and five having the lowest mortality rate. A further breakdown of the relationship between mortality rate and certain age groups proves to be a major indicator of the importance of early detection and diagnosis. Ninety-five percent of the 252 patients were still alive at ten years post-surgery, eighty-nine percent at twenty years, eighty-two percent at thirty years, and seventy-nine percent at forty years. The causes of late death vary but the most common cause was coronary artery disease. At the mean age of fifty-three years, ten patients were diagnosed and died from this disease. All of these patients were older than ten years of age when the initial coarctation procedure occurred. Seven patients died during a second cardiac operation: aortic valve replacement caused three patients to die, recoarctation surgery led to two patients' deaths, and mitral valve operation caused two others to die. In seven different patients sudden death occurred and of these seven patients, six were older than ten years of age at the time of the initial coarctation repair. Ruptured aortic aneurysm caused the death of six subjects; five occurred from ascending aortic dissection and rupture, while the last patient died from an aneurism occurring at the site of the original repair (Rene M. et al.)

Cause of Late Death	No.
Coronary artery disease	10
Second cardiac operation	7
Aortic dissection	6
Sudden unexplained	7
Automobile accident	3
Other	7
Unknown	5

Other=Suicide, endocarditis, cardiomyopathy, alcohol, cancer, autoimmune deficiency syndrome; and prostate surgery.

Side Effects

Hypertension is defined when a patient's blood pressure in the arm exceeds 140/90 mmHg under normal conditions. This is a severe problem for the heart and can cause many other complications. 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. Undetected chronic hypertension can lead to sudden death among coarctation repair patients, at higher rates as time progresses.

Angioplasty is a procedure done to dilate an abnormally narrow section of a blood vessel to allow better blood flow. This is done in a cardiac catheterization laboratory. Typically taking two to three hours, the procedure may take longer but usually patients are able to leave the hospital the same day. After a coarctation repair 20-60% of infant patients may experience reoccurring stenosis at the site of the original operation. This can be fixed by either another coarctation or by the procedure of choice among cardiologists; angioplasty.

Coronary artery disease, otherwise simply known as heart disease, 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 a study conducted by Mare Cohen, MD, et al, one fourth of the patients who experienced a coarctation died of heart disease, some at a relatively young age (Cohen, Marc, MD et al.) (G Di Salvo et al.)

Clinical criteria are used in most studies when defining recurrence of coarctation (recoarctation) when blood pressure is at a difference of >20 mmHg between the lower and upper limbs. This procedure is most common in infant patients and is uncommon in adult patients. In a study conducted by Koller et al., 10.8% of infant patients underwent recoarctations at less than two years of age while another 3.1% of older children received a recoarctation (Michael Giuffre et al.)

Patients 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 (Michael Giuffre et al.)

Followup

Leaving the hospital after a coarctation is only one step in a lifelong process. Just because the coarctation was fixed does not mean that the patient is cured. It is extremely important to visit the cardiologist on a regular basis. Depending on the severity of the patient's condition, which is evaluated on a case-by-case level, visiting a cardiologist can be a once a year surveillance check up. Keeping a regular schedule of appointments with a cardiologist after the coarctation is complete helps increase the chances of survivability for the patients.

History

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

References

• The American Journal of Cardiology, Volume 89, Issue 5, 1 March 2002, Pages 541–547 SURGERY FOR CONGENITAL HEART DISEASE: Influence of age on survival, late hypertension, and recoarctation in elective aortic coarctation repairIncluding long-term results after elective aortic coarctation repair with a follow-up from 25 to 44 years. René M. H. J. Brouwer, MD, Michiel E. Erasmus, MD, Tjark Ebels, MD, PhD, Anton Eijgelaar, MD, PhD
• Journal of Human Hypertension 25, 739-745 Masked hypertension in young patients after successful aortic coarctation repair: impact on left ventricular geometry and function. G Di Salvo, B Castaldi, L Baldini, S Gala, F del Gaizo, A D'Andrea, G Limongelli, A F D'Aiello, G Scognamiglio, B Sarubbi, G Pacileo, M G Russo and R Calabrò
• Cohen, Marc, MD, Valentin Fuster, MD, Peter M. Steele, MBBS, David Driscoll, MD, and Dwight C. McGoon, MD. "Coarctation of the Aorta Long-term Follow-up and *Prediction of Outcome After SurgicalCorrection." Circulation 80 (1989): 840-45. Print.

External links

• Aortic Coarctation information from Seattle Children's Hospital Heart Center
• Diagram at kumc.edu
• Overview and diagram at umich.edu
• Down's Heart Group Specific information relating to heart conditions and Down's Syndrome.
• http://www.uniklinik-bonn.de/dzft Specific information about hyperoxygenation approach (Note: in German)
• "English version" of some (other?) information from the [same web site as the] above source
• 3D CT Angiogram Radiology of Coarctation
• Staff, Mayo Clinic. "Definition." Mayo Clinic. Mayo Foundation for Medical Education and Research, 20 Apr. 2012. Web. 22 Feb. 2013.
• Nonductal Dependent Coarctation: A 20-year Study of Morbidity and Mortality Comparing Early-to-Late Surgical Repair

Michael Giuffre, MD, MBA, FRCPC, FACC; Lindsay Ryerson, MD; Denise Chapple, MD, FRCPC; Susan Crawford, MSc; Joyce Harder, MD, FRCPC; and Alexander K.C. Leung, MBBS, FRCPC, FRCP(UK & Irel), FRCPCH

• Coarctation of the aorta: natural history and outcome after surgical treatment

N.P. Jenkins and C. Ward From the University Department of Cardiology, Regional Cardiac Centre, Withenshawe Hospital, Manchester, UK

• Cohen, Marc, MD, Valentin Fuster, MD, Peter M. Steele, MBBS, David Driscoll, MD, and Dwight C. McGoon, MD. "Coarctation of the Aorta Long-term Follow-up and Prediction of Outcome After SurgicalCorrection." Circulation 80 (1989): 840-45. Print.
• Clinic, Cleveland. "Cardiac Catheterization." Cardiac Catheterization. Cleveland Clinic, n.d. Web. 22 Feb. 2013.