History

Fact Explanation
Features of Heart Failure [5] In newborns and in infants symptoms of heart failure are usually poor feeding, scalp sweating during feeds, poor weight gain and lethargy. [1,2] This is due to the left ventricular output obstruction and increased end diastolic volume of the left ventricle. Acute volume overload eventually results in congestive heart failure. [13]
History of hypertension [12] High blood pressure is recorded in the right arm. [11]
Intracranial hemorrhage [3] Due to rupture of intra cranial Berry aneurysm and sub arachnoid hemorrhages. [3,4] Intracranial Berry aneurysm is a known association with coarctation of aorta. Patients might present with recurrent intracranial hemorrhages. [14]
Fatigue Due to heart failure and lower limb hypo perfusion. This also can cause pain or weakness in the lower limbs.
Sudden death May be due to cardiac failure, intracranial hemorrhage or infective endocarditis. [7,8]
References
  1. RAO PS. Balloon angioplasty of native aortic coarctation. J Am Coll Cardiol. [online] Sep 1992;20(3):750-1. [viewed 26 March 2014]
  2. SALAHUDDIN N, WILSON AD, RAO PS. An unusual presentation of coarctation of the aorta in infancy: role of balloon angioplasty in the critically ill infant. Am Heart J. [online] Dec 1991;122(6):1772-5. [viewed 26 March 2014]
  3. SUAREZ JI, TARR RW, SELMAN WR. Aneurysmal subarachnoid hemorrhage N Engl J Med. [online] 2006. pp. 387–396. [viewed 26 March 2014]
  4. BENYOUNES N, BLANC R, BOISSONNET H, PIOTIN M. Subarachnoid hemorrhage revealing aortic coarctation in a young man. Neuroradiology. [online] 2011;53:931–932. [viewed 26 March 2014]
  5. ERIC R. Coarctation of the aorta from fetus to adult: curable condition or life long disease process? Heart [online] 2005: 91(11): 1495-1502. [viewed 26 March 2014] Available from: doi: 10.1136/hrt.2004.057182
  6. CEVIK S, IZGI C, CEVIK C. Asymptomatic severe aortic coarctation in an 80-year-old man. Tex Heart Inst J. [online] 2004;31:429-431 [viewed 26 March 2014] Available from: http://www.medsci.org/v07p0340.htm#B2
  7. CAMPBELL M. Natural history of coarctation of the aorta. Br Heart J. [online] 1970;32:633-640 [viewed 26 March 2014]
  8. JENKINS NP, WARD AR. Coarctation of the aorta: natural history and outcome after surgical treatment. QJM. [online] 1999;92:365-371. [viewed 26 March 2014]
  9. CONVENS C, VERMEERSCH P, PAELINCK B, VAN DEN HEUVEL P, VAN DEN BRANDEN F. AORTIC COARCTATION: a rare and unexpected cause of secondary arterial hypertension in the elderly. Cathet Cardiovasc Diagn. [online] 1996;39:71-74 [viewed 26 March 2014]
  10. MIRO O, JIMENEZ S, GONZALEZ J, DE CARALT TM, ORDI J. Highly effective compensatory mechanisms in a 76-year-old man with a coarctation of the aorta. Cardiology. [online] 1999;92:284-286. [viewed 26 March 2014]
  11. ROBERT E. G. Coarctation of the Aorta. Circulation. [online] 1953;7:757-768. [viewed 26 March 2014] Available from: doi: 10.1161/01.CIR.7.5.757
  12. KOTA S. K1., KOTA S. K., MEHER L. K., SRUTI J., KOTNI G., PANDA S., TRIPATHY P. R., MODI K. Clinical analysis of hypertension in children: an urban Indian study. Saudi J Kidney Dis Transpl. [online] 2013 Jul;24(4):844-52. [viewed 26 March 2014] Available from: www.ncbi.nlm.nih.gov/uniquesig0/pubmed/23816747
  13. MOHAMED A. H. Coarctation of the Aorta: A Comprehensive Review. J. Arab Neonatal Forum [online] 2006; 3:5-13. [viewed 27 March 2014] Available from: http://www.fmhs.uaeu.ac.ae/neonatal/iss005/p2.pdf
  14. O'REILLY J. N., CHAPMAN O. W. Coarctation of the aorta with cerebral aneurysm. Arch Dis Child. [online] Jun 1943; 18(94): 109–111. [viewed 28 March 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1987850/?page=3

Examination

Fact Explanation
Hypertension High systolic blood pressure is recorded. [1]
Blood pressure disparity in the upper and lower limbs [1] Reduced perfusion of the lower limbs causes low blood pressure in the lower limbs. Blood pressure difference is more than 20 mm Hg.
Blood pressure disparity in the right and left arms The blood pressure is 20mm Hg or more higher in the right arm than the left arm. This occurs when the coarctation is proximal to the origin of left subclavian artery. [1]
Tachypnea and shortness of breath [3,4,5] Due to heart failure.
Tachycardia [5] Tachycardia and gallop rhythm are features of heart failure.
Shock [3] Due to congestive heart failure.
Absent or weak pulse in left arm [2] The left subclavian artery originates below the coarctation.
Absent lower extremity pulses or radio-femoral delay [2,3] Due to reduced perfusion of lower limbs.
Differential cyanosis Cyanosis in the lower extremities with pink upper limbs. This occurs when the lower limb perfusion is duct dependent. The lower extremities are perfused with deoxygenated blood coming from the pulmonary arteries.
Systolic murmur [2,5] In the infraclavicular area and under the scapula on left side. Murmur may be continuous in the presence of multiple collateral vessels [6] or in severe coarctation.
Abdominal bruit This is a relatively rare presentation and it is due to the turbulent blood flow at the site of narrowing of the aorta.
References
  1. ROBERT E. G. Coarctation of the Aorta. Circulation. [online] 1953;7:757-768. [viewed 26 March 2014] Available from: doi: 10.1161/01.CIR.7.5.757
  2. ERIC R. Coarctation of the aorta from fetus to adult: curable condition or life long disease process? Heart [online] 2005: 91(11): 1495-1502. [viewed 26 March 2014] Available from: doi: 10.1136/hrt.2004.057182
  3. WARNES CA, DEANFIELD JE. Congenital heart disease in adults. In: (ed.) Alexander RW. et al. Hurst's The Heart Volume 2, 11th edition. New York: McGraw Hill Professional. 2004:1866
  4. DAVRAN CICEKS, CEVAHIR HABERAL, SULEYMAN OZKAN, HALDUN MUDERRISOGLU. A severe coarctation of aorta in a 52-year-old male: a case report. Int J Med Sci [online] 2010; 7(6):340-341. [viewed 26 March 2014] Available from: doi:10.7150/ijms.7.340
  5. MALLIKA P., ANTHONY H., ROBERT T. Coarctation of the aorta in the newborn. BMJ [online] 2011; 343[viewed 26 March 2014] Available from: doi: http://dx.doi.org/10.1136/bmj.d6838
  6. JIARAKONGMUN P, CHEWIT P, PONGPECH S. Ruptured anterior spinal artery aneurysm associated with coarctation of aorta. Case report and literature review. Interv Neuroradiol. [online] 2002 Sep 30;8(3):285-92. [viewed 26 March 2014] Available from: www.ncbi.nlm.nih.gov/uniquesig0/pubmed/20594486

Differential Diagnoses

Fact Explanation
Left ventricular outflow obstruction (aortic stenosis, aortic sclerosis) [4] Both causes low volume pulses. But in left ventricular outflow obstruction all the pulses are low volume.
Adrenal Insufficiency Presents with non-specific symptoms like fatigue, anorexia, and weight loss. [1]
Congenital Adrenal Hyperplasia Hypertension is a common presenting feature. [3] "Salt-losing crisis" may present with features of shock. [2]
Hypertension Other causes of hypertension should also be considered.
Cardiogenic shock Cardiogenic shock is characterized by persistent hyptension and hypoperfusion. [6]
Sepsis Sepsis can result in septic shock, persistent hypotension despite adequate fluid resuscitation. [7]
Endocardial Fibroelastosis Thickening of the endocardium due to excess deposition of collagen and elastin fibers. This results in symptoms of heart failure. [5]
References
  1. ARLT W., ALLOLIO B. Adrenal insufficiency. Lancet. [online] 2003 May 31;361(9372):1881-93. [viewed 27 March 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/12788587
  2. Al-Agha A. E., Ocheltree A. H., Al-Tamimi. Association between genotype, clinical presentation, and severity of congenital adrenal hyperplasia: a review. Turk J Pediatr. [online] 2012 Jul-Aug;54(4):323-32. [viewed 27 March 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/23692712
  3. SICA DA. Endocrine causes of secondary hypertension. J Clin Hypertens (Greenwich). [online] 2008 Jul;10(7):534-40. [viewed 27 March 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/18607139
  4. MOHAMED A. H. Coarctation of the Aorta: A Comprehensive Review. J. Arab Neonatal Forum [online] 2006; 3:5-13. [viewed 27 March 2014] Available from: http://www.fmhs.uaeu.ac.ae/neonatal/iss005/p2.pdf
  5. CHRISTINA M. S., HERWIG A. PATRIZIA L. M. Endocardial fibroelastosis of the heart. The Lancet, [online] 10 March 2012 Vol 379, (9819)- 932, [viewed 28 March 2014] Available from: doi:10.1016/S0140-6736(11)61418-9
  6. HARMONY R. R., JUDITH S. H. Cardiogenic Shock. Current Concepts and Improving Outcomes. Circulation. [online] 2008; 117: 686-697 [viewed 28 March 2014] Available from: doi: 10.1161/​CIRCULATIONAHA.106.613596
  7. CLAUDIO C., ALESSANDRA P., JOHN F., OSBORN, ANTONELLA F., SIMONETTI, LUCIA P. Diagnosis of Neonatal Sepsis: A Clinical and Laboratory Challenge. Clinical Chemistry [online] February 2004 vol. 50(2) 279-287. [viewed 28 March 2014] Available from: doi: 10.1373/clinchem.2003.025171

Investigations - for Diagnosis

Fact Explanation
Echocardiography [1,3] Echocardiogram enables the diagnosis and Doppler studies can determine the pressure gradient at the site of coarctation. [3]
Chest x ray [1] Rib notching due to dilatation of collateral vessels. “3” sign of aorta due to the coarctation. [3]
Electrocardiogram (ECG) Shows evidence of left ventricular hypertrophy. [1,2,3] It may show evidence of left ventricular strain pattern (Eg: ST segment depression and T wave inversion.) [3]
Magnetic Resonance Imaging (MRI) [1,3] MRI detects the exact location of the coarctation and the presence of collaterals. MRI and cardiac catheterization gives similar information and enable planning the treatment. [4]
Cardiac catheterization Cardiac catheterization is invasive and now rarely practiced in diagnosis. An advantage of cardiac catheterization is that it can be combined with treatment. [5,6]
References
  1. ERIC R. Coarctation of the aorta from fetus to adult: curable condition or life long disease process? Heart [online] 2005: 91(11): 1495-1502. [viewed 26 March 2014] Available from: doi: 10.1136/hrt.2004.057182
  2. VRIEND J, ZWINDERMAN A, DE GROOT E, et al. Predictive value of mild, residual descending aortic narrowing for blood pressure and vascular damage in patients after repair of aortic coarctation. Eur Heart J [online] 2005;26:84–90. [viewed 26 March 2014] Available from: doi:10.1093/eurheartj/ehi004
  3. MOHAMED A. H. Coarctation of the Aorta: A Comprehensive Review. J. Arab Neonatal Forum [online] 2006; 3:5-13. [viewed 27 March 2014] Available from: http://www.fmhs.uaeu.ac.ae/neonatal/iss005/p2.pdf
  4. NIELSEN JC, POWELL AJ, GAUVREAU K, et al. Magnetic resonance imaging predictors of coarctation severity. Circulation [online] 2005; 111: 622-628. [viewed 27 March 2014]
  5. HAMDAN MA, MAHESHWARI S, FAHEY JT, HELLENBRAND WE. Endovascular stents for coarctation of the aorta: initial results and intermediate-term follow-up. J Am Coll Cardiol [online] 2001; 38:1518-1523. [viewed 27 March 2014]
  6. MAHESHWARI S, BRUCKHEIMER E, FAHEY JT, HELLENBRAND WE. Balloon angioplasty of postsurgical recoarctation in infants: the risk of restenosis and long-term follow-up. J Am Coll Cardiol [online] 2000; 35:209-213. [viewed 27 March 2014]

Investigations - Fitness for Management

Fact Explanation
Chest X-ray Aids in diagnosing heart failure and pre-operative treatment of heart failure. (Heart failure is an added risk. [1])
Full blood count To assess the hemoglobin level. Aids in differentiating sepsis.
Exercise ECG To assess exercise tolerance prior to elective surgery. [1]
Renal function test Serum electrolytes, blood urea and serum creatinin will provide adequate information on renal function. Kidneys are one of the main organs involved in drug metabolism. Patients with renal dysfunction have increased mortality than the patients without renal disease. [2]
References
  1. ACC/AHA 2007 Guidelines on Perioperative Cardiovascular Evaluation and Care for Noncardiac Surgery. Circulation. [online] 2007; 116: e418-e500. [viewed 28 March 2014] Available from: doi: 10.1161/​CIRCULATIONAHA.107.185699
  2. BROSIUS F. C. III, HOSTETTER T. H., KELEPOURIS E, et al. Detection of chronic kidney disease in patients with or at increased risk of cardiovascular disease: a science advisory from the American Heart Association Kidney and Cardiovascular Disease Council; the Councils on High Blood Pressure Research, Cardiovascular Disease in the Young, and Epidemiology and Prevention; and the Quality of Care and Outcomes Research Interdisciplinary Working Group: developed in collaboration with the National Kidney Foundation. Circulation. [online] 2006; 114: 1083–7. [viewed 30 March 2014] Available from: doi: 10.1161/​CIRCULATIONAHA.106.177321

Investigations - Followup

Fact Explanation
Echocardiography [1] Post operative echocardiogram ensure the restoration of normal anatomy. [1] There is a considerable risk of re-stenosis, aneurysm and pseudo-aneurysm formation even after corrective interventions. [5] Echo cardiogram enables detection of those complications. [2] Coarctation of aorta is a life long disease [1,4] and long term follow up is indicated after the definitive treatment. (There is no established evidence on the duration of follow up.)
MRI MRI is considered a better option to detect re-coarctation and aneurysm formation than the echocardiogram. [3] Patients should be followed up with an MRI in 12 to 24 months intervals. Reduction in the compliance of the aorta is an established cause for the development of hypertension in later life. MRI can determine the vascular compliance and predict the risk of hypertension. [6]
Cardiac catheterization This is the gold standard investigation in detection of the re-coarctation and other vascular complications. How ever due to the increased risk of radiation exposure, it is reserved for patients with strong suspicion of the complications. [6]
References
  1. MOHAMED A. H. Coarctation of the Aorta: A Comprehensive Review. J. Arab Neonatal Forum [online] 2006; 3:5-13. [viewed 27 March 2014] Available from: http://www.fmhs.uaeu.ac.ae/neonatal/iss005/p2.pdf
  2. MODENA M. G,, BENASSI A,, MATTIOLI G., Computerised tomography and ultrasound in the noninvasive evaluation of coarctation of the aorta. Am J Cardiol [online]1985; 56:822–7. [viewed 27 March 2014]
  3. KILNER P. Imaging of adults with congenital heart disease. In: Lima JA, ed. Diagnostic Imaging In Clinical Cardiology, 1st edn. London, Martin Dunitz, 1998:211–33.
  4. ERIC R. Coarctation of the aorta from fetus to adult: curable condition or life long disease process? Heart [online] 2005: 91(11): 1495-1502. [viewed 30 March 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1769162/
  5. YUAN S. M., RAANANI E. Late complications of coarctation of the aorta. Cardiol J. [online] 2008;15(6):517-24. [viewed 30 March 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/19039755
  6. SWAN L., WILSON N., HOUSTON A. B., DOIG W., POLLOCK J. C. S., STEWART H. W. The long-term management of the patient with an aortic coarctation repair. European Heart Journal [online] (1998) 19, 382–386. [viewed 30 March 2014] Available from: http://eurheartj.oxfordjournals.org/content/19/3/382.full.pdf

Investigations - Screening/Staging

Fact Explanation
Fetal echocardiography [1,6] This allows early detection of the coarctation and anticipation of the possible outcomes like congestive heart failure. [2,7] However this is very difficult to diagnose antenatally, especially towards the late gestation. [3,4,5] Screening is better done during early gestation, preferably in the second trimester. [8]
References
  1. ERIC R. Coarctation of the aorta from fetus to adult: curable condition or life long disease process? Heart [online] 2005: 91(11): 1495-1502. [viewed 26 March 2014] Available from: doi: 10.1136/hrt.2004.057182
  2. FRANKLIN O, BURCH M, MANNING N, SLEEMAN K, GOULD S, ARCHER N. Prenatal diagnosis of coarctation of the aorta improves survival and reduces morbidity. Heart. [online] 2002; 87: 67–69. [viewed 26 March 2014]
  3. HIKORO MATSUI, MATS MELLANDER, MICHAEL ROUGHTON, HANA JICINSKA, HELENA M. GARDINER. Morphological and Physiological Predictors of Fetal Aortic Coarctation. Circulation. [online] 2008; 118: 1793-1801. [viewed 26 March 2014] Available from: doi: 10.1161/CIRCULATIONAHA.108.787598
  4. SHARLAND GK, CHAN KY, ALLAN LD. Coarctation of the aorta: difficulties in prenatal diagnosis. Br Heart J. [online] 1994; 71: 70–75. [viewed 26 March 2014]
  5. HEAD CE, JOWETT VC, SHARLAND GK, SIMPSON JM. Timing of presentation and postnatal outcome of infants suspected of having coarctation of the aorta during fetal life. Heart [online] 2005; 91:1070-1004 . [viewed 26 March 2014]
  6. PALADINI D, VOLPE P, RUSSO MG, et al. Aortic coarctation: prognostic indicators of survival in the fetus. Heart [online] 2004;90: 1348-1349. . [viewed 26 March 2014]
  7. FRANKLIN O, BURCH M, MANNING N, et al. Prenatal diagnosis of coarctation of the aorta improves survival and reduces morbidity. Heart [online] 2002; 87:67-69. [viewed 26 March 2014]
  8. SHARLAND G. K., CHAN K. Y., ALLAN L. D. Coarctation of the aorta: difficulties in prenatal diagnosis. Br Heart J. [online] Jan 1994; 71(1): 70–75. [viewed 26 March 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC483614/

Management - General Measures

Fact Explanation
Prostaglandin infusion [1] When the lower limb perfusion is duct dependent the patency of the ductus arteriosus is maintained with an infusion of prostaglandin. [3]
Treatment of cardiogenic shock Intravenous fluids and inotrops should be used. [1]
Correction of metabolic acidosis [1] Anerobic metabolism might cause a metabolic acidosis.
Treatment of heart failure Pharmacological management of heart failure should be done prior to surgery.
Treatment of hypertension [3] Hypertension should be controlled before the surgery.
Antibiotic prophylaxis against infective endocarditis [2] There is a risk of bacterial endocarditis with coarctation of aorta.
Screen the females for Turner syndrome Turner syndrome is associated with 10% of cases of coarctation of the aorta. [1]
References
  1. ERIC R. Coarctation of the aorta from fetus to adult: curable condition or life long disease process? Heart [online] 2005: 91(11): 1495-1502. [viewed 26 March 2014] Available from: doi: 10.1136/hrt.2004.057182
  2. BAUER M, ALEXI-MESKISHVILI V, BAUER U. Benefits of surgical repair of coarctation of the aorta in patients older than 50 years. Ann Thorac Surg. [online] 2001;72:2060- 2064. . [viewed 26 March 2014]
  3. DAVRAN C., CEVAHIR H., SULEYMAN O., HALDUN M. A severe coarctation of aorta in a 52-year-old male: a case report. Int J Med Sci [online] 2010; 7(6):340-341. [viewed 26 March 2014] Available from: doi:10.7150/ijms.7.340

Management - Specific Treatments

Fact Explanation
Surgical repair The access is gained through a left lateral thoracotomy. [1] Resection and end-to-end anastomosis is the preferred method of treatment. Subclavian flap repair and patching with a Dacron patch are less practiced treatment options.
Balloon dilation [1,3] This is a first line treatment option in the management. [4,5]
Stent implantation [2,3] In adolescents and adults stent placement is considered as a primary mode of treatment. [6]
References
  1. ERIC R. Coarctation of the aorta from fetus to adult: curable condition or life long disease process? Heart [online] 2005: 91(11): 1495-1502. [viewed 26 March 2014] Available from: doi: 10.1136/hrt.2004.057182
  2. THANOPOULOSA B. D., HADJINIKOLAOUB L., KONSTADOPOULOUA G. N., TSAOUSISA G. S., TRIPOSKIADISB F., SPIROUB P. Stent treatment for coarctation of the aorta: intermediate term follow up and technical considerations. Heart [online] 2000;84:65-70 . [viewed 26 March 2014] Available from: doi:10.1136/heart.84.1.65
  3. MOHAMED A. H. Coarctation of the Aorta: A Comprehensive Review. J. Arab Neonatal Forum [online] 2006; 3:5-13. [viewed 27 March 2014] Available from: http://www.fmhs.uaeu.ac.ae/neonatal/iss005/p2.pdf
  4. HIJAZI ZM, FAHEY JT, KLEINMAN CS, HELLENBRAND WE. Balloon angioplasty for recurrent coarctation of aorta. Immediate and long-term results. Circulation [online] 1991;84: 1150-1156. [viewed 27 March 2014]
  5. OVAERT C, MCCRINDLE BW, NYKANEN D, et al. Balloon angioplasty of native coarctation: clinical outcomes and predictors of success. J Am Coll Cardiol [online] 2000;35: 988-996. [viewed 27 March 2014]
  6. HAMDAN MA, MAHESHWARI S, FAHEY JT, HELLENBRAND WE. Endovascular stents for coarctation of the aorta: initial results and intermediate-term follow-up. J Am Coll Cardiol [online] 2001; 38:1518-1523. [viewed 27 March 2014]