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{{WBRQuestion
{{WBRQuestion
|QuestionAuthor=Gonzalo Romero (Reviewed by Will Gibson)
|QuestionAuthor=Gonzalo Romero (Reviewed by Will Gibson and Yazan Daaboul)
|ExamType=USMLE Step 1
|ExamType=USMLE Step 1
|MainCategory=Embryology
|MainCategory=Pathology
|SubCategory=Cardiology
|SubCategory=Cardiology
|MainCategory=Embryology
|Prompt=A 6-year-old boy is brought to his pediatrician's office for severe headache and nosebleeds over the past six months. The patient 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. His blood pressure is 170/95mmHg in the both upper extremities and 100/70 mmHg in both lower extremities, heart rate is 80/min, respiratory rate is 15/min, and temperature is 37 °C (98.6 °F). On physical examination, he has clear breath sounds bilaterally, but a continuous murmur is heard over the inter-scapular area. A chest x-ray demonstrates prominence of the descending aorta compared to the spine. Which of the following findings on chest x-ray is most specific for this patient's condition?
|SubCategory=Cardiology
|Explanation=This patient is most likely presenting with symptoms and signs of post-ductal [[coarctation of the aorta]] (COA), which is a localized narrowing or abrupt constriction of the [[aortic arch]] after the [[ligamentum arteriosum]]. COA is commonly associated with [[aortic aneurysm]]s and [[bicuspid aortic valve]]. In severe cases, COA may be present in infancy. Diagnosis may be delayed in less severe narrowing, where patients may be diagnosed during childhood, adolescence, or even early adulthood. It is more common in males than females with a male:female ratio of 2:1.  
|MainCategory=Embryology
|SubCategory=Cardiology
|MainCategory=Embryology
|MainCategory=Embryology
|SubCategory=Cardiology
|MainCategory=Embryology
|SubCategory=Cardiology
|MainCategory=Embryology
|SubCategory=Cardiology
|MainCategory=Embryology
|SubCategory=Cardiology
|MainCategory=Embryology
|MainCategory=Embryology
|SubCategory=Cardiology
|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 heart rate of 80 beats/min, respiratory rate of 15/min, blood pressure of 170/95 mm Hg in the upper extremities and 100/70 mm Hg in the lower extremities. 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.
[[Image:COA_x-ray.jpg|center|500px]]
Which of the following is the most specific radiographic finding for the patient's condition?
|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. This condition is also referred to as [[coarctation of the aorta]]. Aortic coarctation may be associated with a bicuspid aortic valve and it is is more common in males than females with a ratio of 2:1. Clinically, patients often present with 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 the 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.


[[Image:COA_x-ray_labeled.jpg|center|500px]]
Clinically, patients often present with [[headaches]], [[epistaxis]], [[dizziness]], [[syncope]], [[dyspnea]], [[chest pain]], cold feet or legs, leg cramps with exercise, significant blood pressure differences in upper and lower extremities, 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 the scapula.


The following are radiologic findings in aortic coarctation:
1. Irregularities or notching of the inferior margins of the posterior ribs that result from collateral flow through dilated and pulsatile intercostal arteries. These collaterals appear after 6 years of age if the coarctation is significant.<br>
2. An inverted "3" sign 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.<br>
3. Signs of [[congestive heart failure]], including: [[cardiomegaly]], [[pulmonary edema]], and prominent pulmonary vasculature. These signs of [[congestive heart failure]] are not specific to this coarctation of the aorta.<br>
|AnswerA=Kerley B lines
|AnswerA=Kerley B lines
|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 posterior-anterior 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.
|AnswerAExp=[[Kerley B lines]] are abnormal, short, horizontal shadows that are present in the costophrenic angles that are present in thickened interlobular septa in cases of pulmonary edema and neoplasms.
|AnswerB=Rib notching
|AnswerB=Rib notching
|AnswerBExp=The following are radiologic findings in aortic coarctation:
|AnswerBExp=The patient's radiologic signs, heart murmur, and symptoms are consistent with post-ductal coarctation of the aorta. Due to increased resistance proximal to the coarctation, collateral arterial circulation often develops. The collateral circulation often manifests as dilated intercostal vessels, which impinge on the inferior surface of ribs. These vessels appear as "rib notching" on X-ray.
# 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.
# 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.
# 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]]
|AnswerC=Cardiomegaly
|AnswerC=Cardiomegaly
|AnswerCExp=There are many causes of [[Cardiomegaly causes| cardiomegaly]], including medications, genetic conditions, endocrine conditions, infectious processes, toxins and iatrogenic causes. Therefore cardiomegaly is not a specific radiologic finding.  In particular, cardiomegaly is not associated with this patient's underlying condition, coarctation of the aorta.
|AnswerCExp=There are many causes of [[Cardiomegaly causes| cardiomegaly]], including genetic disorders, endocine diseases, infectious processes, [[toxins]], systemic diseases, and iatrogenic causes. In particular, [[cardiomegaly]] is not associated with this patient's COA.
|AnswerD=Pleural effusion
|AnswerD=Pleural effusion
|AnswerDExp=The most common causes of transudative [[Pleural effusion | pleural effusions]] in the United States are left ventricular failure, pulmonary embolism, and cirrhosis (causing hepatic hydrothorax). Conversely, 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. Pleural effusion therefore, is not a specific finding in general, and furthermore, pleural effusion is not associated with aortic coarctation.
|AnswerDExp=The most common causes of transudative [[Pleural effusion | pleural effusions]] in the United States are [[heart failure|left ventricular failure]], [[pulmonary embolism]], and [[cirrhosis]]. Conversely, the most common causes of exudative pleural effusions are bacterial [[pneumonia]], [[cancer]] (especially lung, breast, and hematological cancers), viral infection, and [[pulmonary embolism]]. Therefore, pleural effusion is not a specific finding and is not classically associated with coarctation of the aorta.
|AnswerE=Patchy alveolar infiltrates
|AnswerE=Patchy alveolar infiltrates
|AnswerEExp=Patchy alveolar infiltrates are not associated with coarctation of the aorta. 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 patchy alveolar infiltrates are not specific for aortic coarctation. [[http://www.wikidoc.org/index.php/Pulmonary_edema_chest_x_ray]]
|AnswerEExp=Patchy alveolar infiltrates are not associated with coarctation of the [[aorta]]. Cardiogenic pulmonary edema can be distinguished from non-cardiogenic pulmonary edema by the presence of redistribution of blood flow to the upper lobes and interstitial edema. In contrast, patchy alveolar infiltrates with air bronchograms are more indicative of non-cardiogenic edema.
|EducationalObjectives=[[Aortic coarctation]] can produce specific signs on chest x-ray such as: irregularities or notching of the inferior margins of the posterior ribs and an inverted "3" sign of the barium-filled esophagus.
|EducationalObjectives=Coarctation of the aorta is characterized by the presence of aortic narrowing proximal or distal to the ligamentum arteriosum. While it may be diagnosed shortly following birth in severe cases, some patients remain undiagnosed until early adulthood. On physical examination, a significant difference between upper and lower extremity blood pressure and a continuous murmur in the inter-scapular area are suggestive of the diagnosis. Chest x-ray often shows a "3" or inverted "3" sign, prominence of descending aorta in relation to the spine, and rib notching due to formation of collaterals.
|References=First Aid 2014 page 283
|References=Brown ML, Burkhart HM, Connolly HM, et al. Coarctation of the aorta: lifelong surveillance is mandatory following surgical repair. J Am Coll Cardiol. 2013;62(11):1020-1025.<br>First Aid 2014 page 283
|RightAnswer=B
|RightAnswer=B
|WBRKeyword=Heart, Aorta, Vessels, Vasculature, Coarctation, Cardiology, Pulmonology, Radiology, Chest, Chest X ray,
|WBRKeyword=Heart, Aorta, Vessels, Vasculature, Coarctation, Cardiology, Pulmonology, Radiology, Chest, Chest X ray,
|Approved=Yes
|Approved=Yes
}}
}}
{{WBRImage}}

Latest revision as of 23:08, 27 October 2020

 
Author PageAuthor::Gonzalo Romero (Reviewed by Will Gibson and Yazan Daaboul)
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Pathology
Sub Category SubCategory::Cardiology
Prompt [[Prompt::A 6-year-old boy is brought to his pediatrician's office for severe headache and nosebleeds over the past six months. The patient 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. His blood pressure is 170/95mmHg in the both upper extremities and 100/70 mmHg in both lower extremities, heart rate is 80/min, respiratory rate is 15/min, and temperature is 37 °C (98.6 °F). On physical examination, he has clear breath sounds bilaterally, but a continuous murmur is heard over the inter-scapular area. A chest x-ray demonstrates prominence of the descending aorta compared to the spine. Which of the following findings on chest x-ray is most specific for this patient's condition?]]
Answer A AnswerA::Kerley B lines
Answer A Explanation [[AnswerAExp::Kerley B lines are abnormal, short, horizontal shadows that are present in the costophrenic angles that are present in thickened interlobular septa in cases of pulmonary edema and neoplasms.]]
Answer B AnswerB::Rib notching
Answer B Explanation [[AnswerBExp::The patient's radiologic signs, heart murmur, and symptoms are consistent with post-ductal coarctation of the aorta. Due to increased resistance proximal to the coarctation, collateral arterial circulation often develops. The collateral circulation often manifests as dilated intercostal vessels, which impinge on the inferior surface of ribs. These vessels appear as "rib notching" on X-ray.]]
Answer C AnswerC::Cardiomegaly
Answer C Explanation [[AnswerCExp::There are many causes of cardiomegaly, including genetic disorders, endocine diseases, infectious processes, toxins, systemic diseases, and iatrogenic causes. In particular, cardiomegaly is not associated with this patient's COA.]]
Answer D AnswerD::Pleural effusion
Answer D Explanation [[AnswerDExp::The most common causes of transudative pleural effusions in the United States are left ventricular failure, pulmonary embolism, and cirrhosis. Conversely, the most common causes of exudative pleural effusions are bacterial pneumonia, cancer (especially lung, breast, and hematological cancers), viral infection, and pulmonary embolism. Therefore, pleural effusion is not a specific finding and is not classically associated with coarctation of the aorta.]]
Answer E AnswerE::Patchy alveolar infiltrates
Answer E Explanation [[AnswerEExp::Patchy alveolar infiltrates are not associated with coarctation of the aorta. Cardiogenic pulmonary edema can be distinguished from non-cardiogenic pulmonary edema by the presence of redistribution of blood flow to the upper lobes and interstitial edema. In contrast, patchy alveolar infiltrates with air bronchograms are more indicative of non-cardiogenic edema.]]
Right Answer RightAnswer::B
Explanation [[Explanation::This patient is most likely presenting with symptoms and signs of post-ductal coarctation of the aorta (COA), which is a localized narrowing or abrupt constriction of the aortic arch after the ligamentum arteriosum. COA is commonly associated with aortic aneurysms and bicuspid aortic valve. In severe cases, COA may be present in infancy. Diagnosis may be delayed in less severe narrowing, where patients may be diagnosed during childhood, adolescence, or even early adulthood. It is more common in males than females with a male:female ratio of 2:1.

Clinically, patients often present with headaches, epistaxis, dizziness, syncope, dyspnea, chest pain, cold feet or legs, leg cramps with exercise, significant blood pressure differences in upper and lower extremities, 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 the scapula.

The following are radiologic findings in aortic coarctation: 1. Irregularities or notching of the inferior margins of the posterior ribs that result from collateral flow through dilated and pulsatile intercostal arteries. These collaterals appear after 6 years of age if the coarctation is significant.
2. An inverted "3" sign 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.
3. Signs of congestive heart failure, including: cardiomegaly, pulmonary edema, and prominent pulmonary vasculature. These signs of congestive heart failure are not specific to this coarctation of the aorta.

Educational Objective: Coarctation of the aorta is characterized by the presence of aortic narrowing proximal or distal to the ligamentum arteriosum. While it may be diagnosed shortly following birth in severe cases, some patients remain undiagnosed until early adulthood. On physical examination, a significant difference between upper and lower extremity blood pressure and a continuous murmur in the inter-scapular area are suggestive of the diagnosis. Chest x-ray often shows a "3" or inverted "3" sign, prominence of descending aorta in relation to the spine, and rib notching due to formation of collaterals.
References: Brown ML, Burkhart HM, Connolly HM, et al. Coarctation of the aorta: lifelong surveillance is mandatory following surgical repair. J Am Coll Cardiol. 2013;62(11):1020-1025.
First Aid 2014 page 283]]

Approved Approved::Yes
Keyword WBRKeyword::Heart, WBRKeyword::Aorta, WBRKeyword::Vessels, WBRKeyword::Vasculature, WBRKeyword::Coarctation, WBRKeyword::Cardiology, WBRKeyword::Pulmonology, WBRKeyword::Radiology, WBRKeyword::Chest, WBRKeyword::Chest X ray
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