| Snapshot |
| A 4-year-old girl presents to her pediatrician’s office for a well-child visit. She and her parents report no concerns except for occasional headaches. During evaluation of her vital signs, her lower extremity systolic blood pressure is found to be more than 20 mmHg less than her upper extremity systolic blood pressure. |
| Introduction |
| Clinical definition narrowing of the aorta causing hypertension in the upper extremities relative to the lower extremitiespost-ductal type (adult type)narrowing occurs distal to the ductus arteriosumpre-ductal (infantile type)narrowing occurs proximal to the ductus arteriosumEpidemiologydemographicsmale > female< 10% of all congenital heart defectslocationaortarisk factorsfamily historyPathogenesismechanism of coarctation is unknown but thought to be due todecreased blood flow across aorta in utero, which can lead to defects in the aortaectopic ductal tissue in the aorta, which can cause the aorta to be pulled inwards into a coarctationbecause of the aortic narrowing, there is hypertension in the upper extremitiespulse is delayed in the lower extremitiescollateral circulation causes intercostal arteries to enlarge these arteries then erode the ribs, causing a notched appearance on radiographyAssociated conditionsTurner syndrome bicuspid aortic valveWilliams syndromePrognosissome may present early in life, but others may remain asymptomatic until adulthoodinfants may be asymptomatic until the ductus arteriosus closes |
| Presentation |
| Symptoms headacheshortness of breath with exertionexercise intolerancefatiguepoor feeding in infantsPhysical examcardiacharsh systolic murmur along the left sternal bordermay also have systolic murmur along left and right side of the chest with thrillsfrom collateral circulation over timepulsesfemoral < brachial pulsesbounding pulses in upper extremities and carotidsdelay in femoral pulse compared to the radial pulsedifferential cyanosiscyanotic lower extremitieshypertension in upper extremitiessystolic blood pressure in the arms are > 20 mmHg higher than the legsunderdeveloped legs compared with arms |
| Imaging |
| Radiography indicationto rule out any pulmonary pathologiesviewschestfindings rib notching on the inferior surface due to collateral circulation through the intercostalscardiomegalyincreased pulmonary markingsEchocardiographyindicationperformed as a diagnostic testmost specific testfindingscoarctation of aorta visualized |
| Studies |
| Electrocardiogramfindingsleft ventricular hypertrophyMaking the diagnosisbased on clinical presentation and echocardiography |
| Differential |
| Tetralogy of Fallotdistinguishing factortet spells (cyanosis) that are resolved when placed in knee-chest position |
| Treatment |
| Medicalprostaglandin E1 indicationneonates with coarctation of the aortamechanism of actionmaintains a patent ductus arteriosus for adequate lower extremity perfusiondiureticsindicationsheart failureOperativesurgical or transcatheter repairindicationadults and neonates after stabilization of heart failure or shockchildren with hypertensionmodalitiesballoon angioplastystent placement |
| Complications |
| Heart failure Systemic hypertension Berry aneurysms leading to cerebral hemorrhage Aortic rupture Endocarditis |
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