WBR0043

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Author PageAuthor::Gonzalo Romero (Reviewed by Will Gibson)
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Embryology
Sub Category SubCategory::Cardiology
Prompt [[Prompt::A 17-year-old male is brought to his pediatrician by his father for severe headache and nosebleeds over the last six months. He complains that his feet become very cold at night for which he has been wearing two pairs of thick socks. At school he gets occasional cramps in his legs and is easily winded when running in gym class. He denies using any drugs or drinking alcohol. His grades are stable and he has a girlfriend, but is not sexually active. On physical exam his vitals are

Respiratory Rate:15 Blood Pressure: 170/95 on the upper extremities and 100/70 in the lower extremities. His neck is supple without lymphadenopathy. He has clear breath sounds bilaterally, but a continuous murmur is heard over the inter-scapular area. A chest x-ray is ordered and is shown below.

Which of the following is the most specific radiographic finding for the patient's condition?]]

Answer A AnswerA::Kerley B lines
Answer A Explanation [[AnswerAExp::Kerley lines are short parallel lines at the lung periphery. These lines represent distended interlobular septa, which are usually less than 1 cm in length and parallel to one another at right angles to the pleura. They are located peripherally in contact with the pleura, but are generally absent along fissural surfaces. They may be seen in any zone but are most frequently observed at the lung bases at the costophrenic angles on the PA radiograph, and in the substernal region on lateral radiographs. These lines are present when interstitial edema is present, therefore not being specific for aortic coarctation.]]
Answer B AnswerB::Ribs notching
Answer B Explanation [[AnswerBExp::The following are radiologic findings in aortic coarctation:
  1. Irregularities or notching of the inferior margins of the posterior ribs results from collateral flow through dilated and pulsatile intercostal arteries. These collateral appear after 6 years of age if the coarctation is significant.
  2. An inverted "3" sign of the barium-filled esophagus or a "3" sign on a highly penetrated chest radiograph may be visualized. Post-stenotic dilation of the aorta results in a classic reverse 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 reversed 3 image for which the sign is named.
  3. Signs of congestive heart failure Cardiomegaly, pulmonary edema,and prominent pulmonary vasculature are evident. The signs of CHF are not specific to this condition. Aortic coarctation]]
Answer C AnswerC::Cardiomegaly
Answer C Explanation [[AnswerCExp::Cardiomegaly causes There are many causes of cardiomegaly, including medications, genetic conditions, endocrine conditions, infectious processes, toxins and iatrogenic causes. Therefore is not a specific radiologic finding.]]
Answer D AnswerD::Pleural effusion
Answer D Explanation [[AnswerDExp::Pleural effusion The most common causes of transudative pleural effusions in the United States are left ventricular failure, pulmonary embolism, and cirrhosis (causing hepatic hydrothorax), while the most common causes of exudative pleural effusions are bacterial pneumonia, cancer (with lung cancer, breast cancer, and lymphoma causing approximately 75% of all malignant pleural effusions), viral infection, and pulmonary embolism. Therefore pleural effusion is not specific for aortic coarctation.]]
Answer E AnswerE::Patchy alveolar infiltrates
Answer E Explanation [[AnswerEExp::Cardiogenic pulmonary edema can be distinguished from noncardiogenic pulmonary edema by the presence of redistribution of blood flow to the upper lobes (increased blood flow to the higher parts of the lung) and interstitial edema. In contrast, patchy alveolar infiltrates with air bronchograms are more indicative of noncardiogenic edema, therefore not being specific for aortic coarctation. [[1]]]]
Right Answer RightAnswer::B
Explanation [[Explanation::This teenager is presenting with symptoms of post-ductal aortic stenosis, which is a localized narrowing or abrupt constriction of the Aortic arch after the ligamentum arteriosum. It may be associated with a bicuspid aortic valve. It is more common in males than females with a ratio of 2:1. Clinically the patient has headaches, epistaxis, dizziness, syncope, dyspnea, chest pain, cold feet or legs, leg cramps with exercise, differential hypertension, poor growth and decreased exercise performance. There are 3 potential sources of a murmur: multiple arterial collaterals (continuous murmur), an associated bicuspid aortic valve (systolic ejection click) and the coarctation itself, which can be heard over the left infraclavicular area and under scapula. The chest X ray shows irregular notching of the inferior margins of the posterior ribs resulting from collateral flow through dilated and pulsatile intercostal arteries. Aortic coarctation

Educational Objective: Aortic coarctation can produce specific signs on chest x-ray such as: irregularities or notching of the inferior margins of the posterior ribs, an inverted "3" sign of the barium-filled esophagus

References: First Aid 2014 page 283
Educational Objective:
References: ]]

Approved Approved::Yes
Keyword WBRKeyword::WJG
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