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Coarctation of the Aorta

Aorta Coarctation

Coarctation of the aorta is a congenital heart defect in which a segment of the aorta is narrowed. Depending on the severity of the coarctation, it may present as a critical neonatal disease or it can remain asymptomatic for years. 


Presentation

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
  • A 3-month-old boy presented with refractory congestive heart failure since 20 days after birth.[ncbi.nlm.nih.gov]
  • Neonates with coarctation frequently present with congestive heart failure and critically reduced perfusion of the descending aorta following ductal closure.[ncbi.nlm.nih.gov]
  • This leads to an increased risk of high blood pressure and congestive heart failure. Adults with coarctation of the aorta will have higher blood pressure and pulse rates in the upper part of the body than the lower.[uwhealth.org]
  • Coarctation of the Aorta generally presents in one of two ways: An infant, often in the second week of life, may not tolerate the obstruction and may develop signs of significant congestive heart failure including labored breathing, poor feeding, and[stmaryhealthcare.org]
  • Infants with coarctation frequently come to medical attention because of congestive heart failure.[web.archive.org]
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]
  • 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]
  • 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]
  • 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.[symptoma.com]
  • They may experience dizziness or shortness of breath, fainting or near-fainting episodes, chest pain, abnormal tiredness or fatigue, headaches, or nosebleeds.[en.wikipedia.org]
Pathologist
  • Dr Maude Abbott (1869-1940), Canadian pathologist, and the world's greatest authority on congenital heart disease of her time, published the first and largest post-mortem series in 1928, collating all 200 previously documented cases from 1791.[patient.info]
  • Although many of the cases had been described in the nineteenth century, CAD was by then well recognized by pathologists, and the clinical features of coronary artery disease had been described by Herrick 30 in 1912.[doi.org]
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
  • bruit; left thoracotomy scar (postoperative) Echocardiogram Thoracic and abdominal CT angiogram or MRA Primary hyperpara-thyroidism S5.4-14 Rare Hypercalcemia Usually none Serum calcium Serum parathyroid hormone Congenital adrenal hyperplasia S5.4-15[doi.org]
Hypertension
  • METHODS: In the national register for CHD, adults with repaired coarctation of the aorta and diagnosed hypertension - defined as a registry diagnosis and/or use of anti-hypertensive prescription medication - were identified.[ncbi.nlm.nih.gov]
  • BACKGROUND: Paradoxical hypertension after repair of coarctation of the aorta is a well-known phenomenon. The pathogenesis involves the activation of the sympathetic nervous system (first phase) and renin-angiotensin system (second phase).[ncbi.nlm.nih.gov]
  • Signs and symptoms include hypertension, muscle weakness, shortness of breath, headaches and leg cramps[icd9data.com]
  • Prevalence of hypertension and aneurysms was similar in both groups. Fifty percent of patients were hypertensive. Two-thirds of patients demonstrating hypertension were not receiving antihypertensive medication.[ncbi.nlm.nih.gov]
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]
  • There is typically a BP gradient between upper and lower extremities and a grade 2 to 3/6 ejection systolic murmur, sometimes most prominent in the left interscapular area.[merckmanuals.com]
  • A systolic murmur in the left infraclavicular area is typical but a range of murmurs may be heard depending on collateral circulation and the presence of other cardiac abnormalities.[patient.info]
  • A systolic murmur may be present over the left precordium or between the scapulae on the patient's back.[emedicine.medscape.com]
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]
  • It is usually discrete but should be thought of as a diffuse vascular disease.[clinicaladvisor.com]
  • disease, 040158, 心血管疾患, シンケッカンシッカン, cardiovascular disease, 023404, 動脈閉塞性疾患, ドウミャクヘイソクセイシッカン, arterial occlusive disease, 054186, 奇形, キケイ, abnormality, 012757, 先天性疾患, センテンセイシッカン, congenital disease, 040292, 心臓病, シンゾウビョウ, heart disease, 023405, 心臓血管外科[togodb.biosciencedbc.jp]
  • 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]
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.[en.wikipedia.org]
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]
Almond-Shaped Eyes
  • […] month-old girl presented with psychomotor retardation, developmental delay, clinodactyly of the thumb, coarctation of the aorta, patent ductus arteriosus, peripheral pulmonary stenosis, atrial septal defect, microcephaly, brachycephaly, a small oval face, almond-shaped[ncbi.nlm.nih.gov]
Fetus Small for Gestational Age
  • [small for gestational age, premature birth]); and 4) severe hypertension, often in the setting of preeclampsia, requiring urgent treatment to prevent HF, stroke, and adverse fetal outcomes.[doi.org]
Epistaxis
  • 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]
Wide Neck
  • A 7-mm wide-necked, saccular spinal artery aneurysm was identified as the source of the hemorrhage and was subsequently successfully coiled through a transbrachial access route.[ncbi.nlm.nih.gov]
Kidney Failure
  • 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]
  • 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]
  • 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]
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]
  • […] arch, congenital Postductal coarctation of aorta Preductal coarctation of aorta Clinical Information Congenital constriction of the aorta just distal to the left subclavian artery; causes upper body hypertension but few other symptoms; may present as headache[icd9data.com]
  • Signs and symptoms include hypertension, muscle weakness, shortness of breath, headaches and leg cramps.[icd10data.com]
  • 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]

Workup

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.

Fetus Small for Gestational Age
  • [small for gestational age, premature birth]); and 4) severe hypertension, often in the setting of preeclampsia, requiring urgent treatment to prevent HF, stroke, and adverse fetal outcomes.[doi.org]
Streptococcus Pneumoniae
  • Aortic aneurysm is uncommon before the second decade of life, and here we report a 10-year-old male patient with new diagnosis of aortic coarctation and bicuspid aortic valve, who developed a rapidly enlarging mycotic aneurysm from Streptococcus pneumoniae[ncbi.nlm.nih.gov]
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]
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]

Treatment

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. 

Prognosis

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].

Etiology

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.

Epidemiology

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.

Sex distribution
Age distribution

Pathophysiology

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].

Prevention

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. 

Summary

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.

Patient Information

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. 

References

Article

  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.

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Last updated: 2019-07-11 20:39