Milder forms of coarctation may not present in signs or symptoms during infancy. As children become older, they may experience chest pain, headache, fatigue, poor growth, dyspnea, dizziness, fainting, and claudication of the lower extremities. Complications such as heart failure are usually rare.

In extreme cases, critical coarctation manifests soon after the closure of the ductus arteriosus. These neonates present with irritability, dyspnea, poor feeding, and diaphoresis. Serious complications include shock [9], renal dysfunction, and metabolic acidosis. The clinical picture resembles that of sepsis. 

Physical exam

The blood pressure disparity in the upper and lower body is characteristic. Upper extremities exhibit elevated blood pressure and strong pulses [10]; whereas the lower extremity has low or immeasurable blood pressure and reduced femoral pulses.

On auscultation, a systolic ejection murmur is heard at the upper left sternal border and left axilla. It may also be present at the left scapular area. Furthermore, a systolic ejection murmur is heard in cases with coexisting bicuspid aortic valve.

Other

Since disorders such as Turner syndrome, DiGeorge Syndrome, and others can be present, their features will also manifest.

• Congestive Heart Failure

  Infants with coarctation frequently come to medical attention because of congestive heart failure. [web.archive.org]

  A 3-month-old boy presented with refractory congestive heart failure since 20 days after birth. [ncbi.nlm.nih.gov]

• Fatigue

  Symptoms vary with the anomaly’s severity and range from headache, chest pain, cold extremities, fatigue, and leg claudication to fulminant heart failure and shock. A soft bruit may be heard over the coarctation site. [merckmanuals.com]

  Symptoms can include: Angina (chest pain) Dizziness Leg fatigue or cramps Nosebleeds Pain with exertion that is relieved by rest Pounding headache Shortness of breath Diagnosis of Coarctation of the Aorta UT Southwestern’s heart doctors might use a number [utswmedicine.org]

  Signs may appear as the child grows, such as: Headaches, chest pain, fatigue, claudication in the legs (particularly during physical activity) Heart failure is unlikely to occur if it has not appeared during the neonatal period Pathology Co-arctation [almostadoctor.co.uk]

• Anemia

  Thyrotoxicosis, anemia and certain cancers, that increase cardiac output may initiate or exacerbate cervical venous hum [ 1, 2, 3, 9 ]. Cruveilhier-Baumgarten syndrome ("medusa head"). [ncbi.nlm.nih.gov]

  Pediatric patients with thalassemia major, or other chronic anemias requiring multiple transfusions are at risk of myocardial iron deposition, progressive fibrosis and systolic impairment. [doi.org]

  On the basis of a Cochrane analysis, S9.3.1-2 most studies favor CCBs to reduce graft loss and maintain higher GFR, with some evidence suggesting potential harm from ACE inhibitors because of anemia, hyperkalemia, and lower GFR. [hyper.ahajournals.org]

• Pediatric Disease

  Probably because coarctation is primarily a pediatric disease; usually it is identified and surgically repaired long before these patients ever see an internist. Fewer than one in four people who have untreated coarctation make it to their 50s. [nytimes.com]

• Respiratory Distress

  A 7-day-old female neonate was referred to the pediatric emergency department due to cyanosis and respiratory distress. Her arterial oxygen saturation was 65%. [ncbi.nlm.nih.gov]

  Neonates often present with profound acidosis and respiratory distress. An infusion of prostaglandin E1 (PGE1) may be administered within a month of birth and often opens a closed ductus. [emedicine.medscape.com]

• Abdominal Bruit

  […] systolic-diastolic bruit; bruits over other arteries (carotid – atherosclerotic or fibromuscular dysplasia), femoral Renal Duplex Doppler ultrasound; MRA; abdominal CT Bilateral selective renal intra-arterial angiography Primary aldosteronism S5.4-5, [doi.org]

• Constipation

  […] supraclavicular fat pads, wide (1-cm) violaceous striae, hirsutism Overnight 1-mg dexamethasone suppression test 24-h urinary free cortisol excretion (preferably multiple); midnight salivary cortisol Hypothyroidism S5.4-10 Dry skin; cold intolerance; constipation [doi.org]

• Hypertension

  Severe persistent hypertension is seen infrequently in newborns and infants, but we came across two infants who developed severe paradoxical hypertension after successful coarctation repair. [ncbi.nlm.nih.gov]

  Signs and symptoms include hypertension, muscle weakness, shortness of breath, headaches and leg cramps [icd9data.com]

  Beta-blockers are used to treat hypertension in adults before physical correction of the coarctation, and may be used afterwards for persisting hypertension. [patient.info]

• Systolic Murmur

  Examination showed mild tachycardia, tachypnoea, weak femoral pulses, and a soft systolic murmur at the left sternal edge. The lungs were clear and the liver was enlarged by 3 cm. [bmj.com]

  A grade 2 to 3/6 ejection systolic murmur is often present at the upper left sternal border, left axilla, and sometimes most prominently in the left interscapular area (see table Heart Murmur Intensity ). [merckmanuals.com]

  A grade 2 to 3/6 ejection systolic murmur is often present at the upper left sternal border, left axilla, and sometimes most prominently in the left interscapular area (see table Heart Murmur Intensity). [msdmanuals.com]

  The typical heart murmur that is associated with a coarctation is a systolic murmur that is loudest in the back below the left shoulder blade (scapula). [web.archive.org]

• Weak Pulse

  A mild narrowing may go undetected for years but may be discovered due to high blood pressure in the arms, low blood pressure in the legs, weak pulses in the groin and feet, heart murmur, leg cramping with exercise, and frequent headaches. [nationwidechildrens.org]

  However, there may be other symptoms noted on the examination such as weak pulses and a difference between the pulses in the upper and lower half of the body. That alerts doctors to the possibility of coarctation of the aorta. [luriechildrens.org]

  It is associated with notching of the ribs (because of collateral circulation), hypertension in the upper extremities, and weak pulses in the lower extremities. [pedclerk.bsd.uchicago.edu]

  […] pressure difference between the arms and legs A heart murmur A weak pulse in the legs Re-narrowing can occur without symptoms. [wexnermedical.osu.edu]

• Vascular Disease

  This report suggests a new application of ICE for intervention with structural and vascular diseases other than interatrial septum. [ncbi.nlm.nih.gov]

  treatment outcome, 060847, 比較, ﾋｶｸ(ｸﾗﾍﾞﾙ), comparison, 014178, ﾋｶｸ キーワード 狭窄症, ｷｮｳｻｸｼｮｳ, stenosis, 040129, *, 病気, ﾋﾞｮｳｷ, disease, 017213, 大動脈疾患, ﾀﾞｲﾄﾞｳﾐｬｸｼｯｶﾝ, aortic disease, 054530, 血管疾患, ｹｯｶﾝｼｯｶﾝ, vascular disease, 040158, 心血管疾患, ｼﾝｹｯｶﾝｼｯｶﾝ, cardiovascular [togodb.biosciencedbc.jp]

  It is usually discrete but should be thought of as a diffuse vascular disease. [clinicaladvisor.com]

  Learn more » Vascular Surgery Offering comprehensive care of vascular disease through preventive services, diagnostic expertise, minimally invasive therapies and traditional surgical techniques. [surgery.usc.edu]

• Diastolic Hypertension

  Secondary hypertension can underlie severe elevation of BP, pharmacologically resistant hypertension, sudden onset of hypertension, increased BP in patients with hypertension previously controlled on drug therapy, onset of diastolic hypertension in older [doi.org]

• Epistaxis

  Clinical manifestations include dizziness, headaches, fainting, epistaxis, reduced or absent femoral pulses, and muscle cramps in the legs from tissue anoxia during increased exercise. [medical-dictionary.thefreedictionary.com]

  Later in life, patients may present with headache, epistaxis, or visual disturbances. Exertional dyspnea and stroke are other presenting symptoms. [15] Coarctation can be clinically diagnosed depending on the available evidence, as described above. [emedicine.medscape.com]

• Kidney Failure

  Chronic high blood pressure associated with CoA increases the risks of: heart damage an aneurysm a stroke premature coronary artery disease Chronic high blood pressure can also lead to: kidney failure liver failure a loss of eyesight through retinopathy [healthline.com]

  This can cause damage to your heart and also can result in kidney failure or other organ failure. If your coarctation of the aorta was treated when you were young, you have a risk of the aorta re-narrowing (re-coarctation) over time. [mayoclinic.org]

  Problems with blood pressure can lead to complications, such as stroke, kidney failure, and intestinal damage. What causes coarctation of the aorta? COA is a congenital heart defect. [saintlukeshealthsystem.org]

• Anuria

  Symptoms and Signs If coarctation is significant, circulatory shock with renal insufficiency (oliguria or anuria) and metabolic acidosis may develop in the first 7 to 10 days of life and may mimic findings of other systemic disorders such as sepsis. [merckmanuals.com]

• Headache

  Signs and symptoms include hypertension, muscle weakness, shortness of breath, headaches and leg cramps [icd9data.com]

  Here we report a middle-aged female patient who presented with acute severe headache. Head computed tomography (CT) demonstrated an extensive subarachnoid hemorrhage. [ncbi.nlm.nih.gov]

  Signs and symptoms include hypertension, muscle weakness, shortness of breath, headaches and leg cramps. [icd10data.com]

The clinician should obtain a thorough history and physical exam. The following studies will establish the diagnosis:

Chest radiography identifies cardiomegaly as well as the coarctation. An echocardiogram demonstrates the site and degree of severity. Additionally, it provides key findings such as valvular disease, cardiac function, etc.

Further testing such as the electrocardiogram (ECG) can depict ventricular hypertrophy if present. Moreover, magnetic resonance imaging (MRI) indicates the location of the defect and assesses its effects on blood vessels [11] while a computed tomography (CT) angiogram evaluates the arteries to determine narrowing of the aorta [12]. Finally, cardiac catheterization and aortography may also be applied.

• Left Atrial Hypertrophy

  Hypertension has long been recognized as a risk factor for AF because it is associated with LVH, decreased diastolic function with impaired LV filling, rising left atrial pressures with left atrial hypertrophy and enlargement, increased atrial fibrosis [doi.org]

Medical intervention

Neonatal disease

Neonates presenting with symptoms should be treated with prostaglandin E 1 to reopen the ductus arteriosus [13]. If the latter remains open, the pulmonary artery bypasses the coarctation, which subsequently improves blood supply and corrects the metabolic acidosis. Additionally, diuretics can relieve symptoms secondary to heart failure. Oxygen supplementation in these patients should be used carefully since it can alter the pulmonary hemodynamics. In coarctation manifesting with left ventricle failure, dobutamine or other therapies may be utilized.

Hypertension

In stable patients, hypertension treatment consists of beta blockers. Post correction, there are numerous alternatives such as ACE inhibitors, angiotensin II receptor inhibitors, and calcium channel blockers [14]. 

Surgical interventions

The surgery of choice is debatable. Many institutions will initially repair the coarctation while others perform balloon angioplasty with or without stents as the first intervention. In cases with recurrence, the latter may be performed. Of importance, successful outcomes with angioplasty have been demonstrated in both initial and recurrent coarctation (73% and 80%, respectively).

Specific surgical interventions include resection of the coarctation followed by anastomosis of the unattached segments. Also, there are various forms of aortoplasty depending on the anatomy. Marked cases may need widening of the isthmus and transverse segments of the aorta [15].

Surgical planning and decision making revolve around the particular case and expertise. 

The prognosis is good for patients with coarctation of aorta without other cardiac defects. These patients are treated for hypertension with standard drugs such as beta blockers, ACE inhibitors, angiotensin II receptor blockers, and others [7]. However, patients with coexisting cardiac anomalies exhibit a greater risk for chronic complications.

Recurrence is observed in surgically corrected patients. Hence, periodic monitoring is paramount. Additionally, due to the higher occurrence rate of coronary artery disease, all patients require surveillance. 

For symptomatic infants, the post surgery mortality rate is below 5%. This number is even less in children, namely under 1% [8].

The cause of this defect is likely a multifactorial inheritance in origin as are the majority of congenital heart diseases [3]. Although many cases are sporadic, it has been observed in a familial pattern. Furthermore, coarctation has been associated with Turner Syndrome and other genetic diseases.

Several theories have been hypothesized regarding the development of coarctation of the aorta. One explanation suggests that modification of intrauterine blood and the resultant flow through the aortic arch causes coarctation [2]. Another proposes that ductal constriction after birth contributes to the formation of coarctation [4]. Furthermore, one theory postulates that tissue from the ductus arteriosus blends with that of the aorta. Hence, when the ductus closes after birth, the extra tissue narrows the aorta.

Coarctation of the aorta accounts for 6% to 8% of all congenital heart defects [5]. Regarding gender, it is observed more in males than females. This congenital heart defect often manifests in conjunction with a ventral septal defect, aortic valve abnormalities, and other cardiac malformations. It is present in 15% to 20% of Turner syndrome open link patients. Additionally, it is also found in hypoplastic left heart syndrome, Shone's complex, and DiGeorge syndrome [5]. This cardiac defect appears to have a seasonal preference as it occurs more in September and November.

Cardiovascular changes with coarctation

This congenital defect is due to the narrowing of the aorta. The coarctation in most cases occurs after the arteries supplying the upper body branch off. While this spares the blood flow to the head and arms, there is reduced flow to the lower extremities. This causes the observed blood pressure discrepancy between the upper and lower halves of the body.

Since the narrowing of the aorta limits blood flow out of the left ventricle, the blood backs up in this chamber. As a result, structural adaptations such as left ventricular hypertrophy occurs. Severe cases of coarctation develop left ventricular failure and congestive heart failure.

Medical implications of coarctation

The impact of the aortic coarctation is related to the degree of narrowing of the aortic arch and its effect on the afterload.

Mild and moderate narrowing can remain asymptomatic for years. However, the resultant chronic hypertension is accompanied by medical complications even in cases where the coarctation is surgically corrected.

Severe narrowing in the neonate with low cardiac output and shock is referred to as critical coarctation. Following prostaglandin E1 administration, surgical repair is necessary.

Coarctation repair is performed in cases with congenital heart failure (CHF), systolic hypertension, or peak pressure gradient across the coarctation measured to be greater than 20mmg Hg [6].

Endocarditis prophylactic treatment is indicated for 6 months post repair. Well-child doctor appointments include palpation of pulses, particularly that of the femoral artery. Blood pressures should be obtained and if there is a discrepancy in pulses or hypertension, this warrants measurements in all extremities. For patients with cardiac defects, thorough evaluation with an echocardiogram is essential. Additionally, surveillance includes screening for brain aneurysms. Patients with presentations suggestive of coexisting genetic disorders or other cardiac anomalies can undergo genetic evaluation. Aortic valvular abnormalities should prompt evaluation of family members. Patients with a berry aneurysm and hypertension should have blood pressure measurements on upper and lower extremities. 

Coarctation of the aorta is a common congenital cardiac defect characterized by narrowing of the descending aorta, typically near the ductus of arteriosus. Due to the anatomical location of the narrowing, the arterial branches that supply the upper extremities are spared [1] [2]. However, circulation to the lower body is impaired, hence the marked discrepancy in the blood pressure and pulses between the two halves of the body. 

Coarctation can occur as an isolated anomaly or in conjunction with manifestations such as ventral septal defect, bicuspid aortic valve, or other left ventricular outflow tract obstructive lesions. Furthermore, it has been observed with familial patterns as well as sporadically.

In neonates, the clinical picture is critical requiring stabilization and surgery. In milder cases, the patients may be asymptomatic for years.

Hypertension is a consequence of this defect and requires treatment whether through surgery or standard antihypertensives. Additionally, significant coarctation leads to pathophysiologic and cardiac structural modifications that may culminate in heart failure.

Diagnosis is established through a comprehensive evaluation consisting of history, physical examination, and imaging such as an echocardiogram, chest radiograph, and others as well, if indicated.

Correction of the coarctation is conducted through surgery or balloon angioplasty. All patients require surveillance since recurrence and other coexisting cardiac anomalies may present complications.

The aorta is the major blood vessel coming out of the heart which leads the blood to the body. Coarctation of the aorta is the narrowing of a segment of this blood vessel that occurs as a birth defect before birth. It is one of the most common heart defects. It is typically diagnosed in babies, children, or adults younger than 40 years old. 

While the cause is not exactly known, sometimes it runs in families. Other times, it occurs randomly. Coarctation may present as a single heart abnormality, or can occur with other defects such as bicuspid aortic valve, ventral septal defect, and others. 

It may be seen in patients with genetic disorders like Turner syndrome, DiGeorge syndrome, and other as well. People with brain aneurysms may also have coarctation of the aorta.  

The narrow part of the aorta causes reduced blood pressure in the lower half of the body. However, the upper body will have higher blood pressures. Likewise, there are diminished pulses in the lower body.

Newborns with severe narrowing experience symptoms such as fussiness, sweating, difficulty in breathing, rapid breathing, problems with eating, pale skin, etc. They are very ill and require medicine and surgery. 

Mild narrowing develops in childhood or beyond. The symptoms are usually chest pain, shortness of breath, headache, dizziness, poor growth, and leg cramps with physical activity. 

Diagnosis is achieved through assessment of the patient including a history, physical exam, and imaging of the heart. The history includes any signs and symptoms and further information. The physical exam reveals higher blood pressures in the top half of the body and lower blood pressures in the bottom half. Also, the pulses are reduced or absent in the lower body. The doctor will also listen to the patient's heart with a stethoscope to determine if there are abnormal heart sounds or murmurs. 

The imaging consists of an echocardiogram, which is the ultrasound of the heart. It will show the narrowing of the aorta and any other problems. A chest x ray and other tests may be performed as well. 

Treatment is usually surgery, but the timing and type of procedure depends on the severity and the expertise of the clinician. One surgery uses a balloon to widen the aorta. Another surgery involves removing the narrow part and then connecting the free ends. In addition, since coarctation causes high blood pressures, medicine will be used for control. 

1. Rudolph AM, Heymann MA, Spitznas U. Hemodynamic considerations in the development of narrowing of the aorta. American Journal of Cardiology. 1972; 30(5):514-25.
2. Talner NS, Berman MA. Postnatal development of obstruction in coarctation of the aorta: role of the ductus arteriosus. Pediatrics. 1975; 56(4):562-9.
3. Nora JJ. Multifactorial inheritance hypothesis for etiology of congenital heart disease: the genetic-environmental interaction. Circulation. 1968; 38(3):604-17.
4. Cassels DE. The Ductus Arteriosus. Springfield, IL,: Charles C. Thomas; 1973. 161. 
5. McBride KL, Pignatelli R, Lewin M, et al. Inheritance analysis of congenital left ventricular outflow tract obstruction malformations: segregation, multiplex relative risk, and heritability. American Journal of Medical Genetics. Part A. 2005;134A(2):180-186.
6. Abbruzzese PA, Aidala E. Aortic coarctation: an overview. Journal of Cardiovascular Medicine (Hagerstown). 2007;8(2):123-128.
7. Moltzer E, Mattace Raso FU, Karamermer Y, et al. Comparison of Candesartan versus Metoprolol for treatment of systemic hypertension after repaired aortic coarctation. American Journal of Cardiology. 2010;105(2):217-222.
8. Wright GE, Nowak CA, Goldberg CS, et al. Extended resection and end-to-end anastomosis for aortic coarctation in infants: results of a tailored surgical approach. Annals of Thorac Surgery. 2005;80(4):1453-1459.
9. Rao PS. Balloon angioplasty of native aortic coarctation. Journal of the American College Cardiology.1992; 20(3):750-1.
10. Strafford MA, Griffiths SP, Gersony WM. Coarctation of the aorta: a study in delayed detection. Pediatrics.1982; 69(2):159-63.
11. Goubergrits L, Riesenkampff E, Yevtushenko P, Schaller J, Kertzscher U, Hennemuth A, et al. MRI-based computational fluid dynamics for diagnosis and treatment prediction: Clinical validation study in patients with coarctation of aorta. Journal of Magnetic Resonance Imaging. 2015;41(4):909-16.
12. Mohiaddin RH, Kilner PJ, Rees S, Longmore DB. Magnetic resonance volume flow and jet velocity mapping in aortic coarctation. Journal of the American College of Cardiology. 1993; 22(5):1515-21.
13. Fiore AC, Fischer LK, Schwartz T, et al. Comparison of angioplasty and surgery for neonatal aortic coarctation. Annals of Thoracic Surgery. 2005;80(5):1659-1664.
14. Frobel AK, Hulpke-Wette M, Schmidt KG, Laer S. Beta-blockers for congestive heart failure in children. Cochrane Database of Systematic Reviews. 2009 Jan 21;(1):CD007037.
15. Erbel R, Aboyans V, Boileau C, et al; Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC). 2014 ESC guidelines on the diagnosis and treatment of aortic diseases: document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. European Heart Journal. 2014;35(41):2873-2926.