WBR0042: Difference between revisions
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{{WBRQuestion | {{WBRQuestion | ||
|QuestionAuthor=Gonzalo Romero | |QuestionAuthor=Gonzalo Romero (Reviewed by Rim Halaby) | ||
|ExamType=USMLE Step 1 | |ExamType=USMLE Step 1 | ||
|MainCategory=Pathology | |MainCategory=Pathology | ||
| Line 20: | Line 20: | ||
|MainCategory=Pathology | |MainCategory=Pathology | ||
|SubCategory=Cardiology | |SubCategory=Cardiology | ||
|Prompt=A 21-year-old healthy male college student is playing in | |Prompt=A 21-year-old healthy male college student is playing in the soccer championship for his university when he suddenly falls on the ground while running. The player is unresponsive to athletic staff on the field. Emergency Medical Services arrive promptly, but CPR and resuscitation measures are unsuccessful. According to his family and friends, the patient has always been healthy and he has been playing soccer since high school. Autopsies are obtained in order to determine the cause of death. Which of the following cardiac macroscopic or microscopic changes is most likely present? | ||
|Explanation=This young athlete presents with [[sudden cardiac death]] during intense exercise due to [[ventricular arrhythmias]], a typical clinical presentation of [[hypertrophic cardiomyopathy]] | |Explanation=This young athlete presents with [[sudden cardiac death]] during intense exercise due to [[ventricular arrhythmias]], a typical clinical presentation of [[hypertrophic cardiomyopathy]]. [[Hypertrophic cardiomyopathy]] can be either autosomal dominant or idiopathic. | ||
#'''Macroscopically''' the heart demonstrates [[hypertrophy]] more prominent in the ventricular septum, obstructing the outflow from the [[left ventricle]]. The ventricular cavity acquires a banana shape appearance. [[File:2369.jpg|center|200px]] | #'''Macroscopically''' the heart demonstrates [[hypertrophy]] more prominent in the ventricular septum, obstructing the outflow from the [[left ventricle]]. The ventricular cavity acquires a banana shape appearance. [[File:2369.jpg|center|200px]] | ||
#'''Histologically''' there are hypertrophied [[cardiomyocytes]] in disarray which alters the conduction system and subsequently predisposes to [[arrhythmias]]. [[File:438.jpg|center|200px]] | #'''Histologically''' there are hypertrophied [[cardiomyocytes]] in disarray which alters the conduction system and subsequently predisposes to [[arrhythmias]]. [[File:438.jpg|center|200px]] | ||
|AnswerA=Symmetric left ventricular hypertrophy | |AnswerA=Symmetric left ventricular hypertrophy | ||
|AnswerAExp=Symmetric left ventricular hypertrophy is characteristically present in patients with increased [[afterload]], such as [[aortic stenosis]] or [[hypertension]]. This leads to the increased synthesis of [[actin]] and [[myosin]] that are arranged in a "organized fashion". Ultimately | |AnswerAExp=Symmetric left ventricular hypertrophy is characteristically present in patients with increased [[afterload]], such as [[aortic stenosis]] or [[hypertension]]. This leads to the increased synthesis of [[actin]] and [[myosin]] that are arranged in a "organized fashion". Ultimately, these patients develop diastolic dysfunction due to the inability of the heart to fill in with blood during [[diastole]]. | ||
|AnswerB=White appearance of the endocardium | |AnswerB=White appearance of the endocardium | ||
|AnswerBExp=A white appearance of the [[endocardium]] can be present in [[endocardial fibroelastosis]], a rare restrictive [[cardiomyopathy]] present in young children less than 2 years old. It is caused by the excessive [[fibrosis]] of the [[endocardium]] that causes diastolic dysfunction. Shown below is an image depicting an autopsy of a heart affected with [[endocardial fibroelastosis]]. | |AnswerBExp=A white appearance of the [[endocardium]] can be present in [[endocardial fibroelastosis]], a rare restrictive [[cardiomyopathy]] present in young children less than 2 years old. It is caused by the excessive [[fibrosis]] of the [[endocardium]] that causes diastolic dysfunction. Shown below is an image depicting an autopsy of a heart affected with [[endocardial fibroelastosis]]. | ||
[[Image:Endocardial_fibroelastosis_2.jpg|center|200px]] | [[Image:Endocardial_fibroelastosis_2.jpg|center|200px]] | ||
|AnswerC= | |AnswerC=Cardiomyocyte hypertrophy in an organized fashion | ||
|AnswerCExp= | |AnswerCExp=Cardiomyocyte [[hypertrophy]] in an organized fashion is found among patients with increased [[afterload]] such as [[aortic stenosis]] or [[hypertension]]. | ||
|AnswerD=Prominent ventricular septum hypertrophy | |AnswerD=Prominent ventricular septum hypertrophy | ||
|AnswerDExp=Prominent ventricular septum [[hypertrophy]] is a characteristic finding of [[hypertrophic cardiomyopathy]]. | |AnswerDExp=Prominent ventricular septum [[hypertrophy]] is a characteristic finding of [[hypertrophic cardiomyopathy]]. | ||
|AnswerE=Fibrotic thickening of the endocardium and the valves of the right side of the heart | |AnswerE=Fibrotic thickening of the endocardium and the valves of the right side of the heart | ||
|AnswerEExp=[[Fibrosis|Fibrotic thickening]] of the [[endocardium]] and the valves of the right side of the heart is the macroscopic description of an endocardium affected by a [[carcinoid syndrome|carcinoid syndrome]] due to chronic [[serotonin]] exposure, which causes fibrosis of the tricuspid valve and pulmonary valve. [[Carcinoid syndrome]] occurs when the [[carcinoid tumor]] | |AnswerEExp=[[Fibrosis|Fibrotic thickening]] of the [[endocardium]] and the valves of the right side of the heart is the macroscopic description of an endocardium affected by a [[carcinoid syndrome|carcinoid syndrome]] due to chronic [[serotonin]] exposure, which causes fibrosis of the tricuspid valve and pulmonary valve. [[Carcinoid syndrome]] occurs when the [[carcinoid tumor]] metastasizes to the liver, thereby bypassing the first-pass metabolism of the liver. These patients present with [[diarrhea]], [[wheezing]], [[telangiectasias]], and [[flushing]] of the skin. | ||
|EducationalObjectives=Hypertrophic obstructive cardiomyopathy (HOCM) is a common cause of sudden death in young athletes during intense exercise. It is characterized by the presence of cardiac hypertrophy more prominent in the ventricular septum and hypertrophied [[cardiomyocytes]] in disarray. | |EducationalObjectives=Hypertrophic obstructive cardiomyopathy (HOCM) is a common cause of sudden death in young athletes during intense exercise. It is characterized by the presence of cardiac hypertrophy more prominent in the ventricular septum and hypertrophied [[cardiomyocytes]] in disarray. | ||
|References=First Aid 2014 page 290 | |||
|RightAnswer=D | |RightAnswer=D | ||
|WBRKeyword=Cardiology, Pathology, Hypertrophic cardiomyopathy, HOCM | |WBRKeyword=Cardiology, Pathology, Hypertrophic cardiomyopathy, HOCM, HCM, Ischemia, Sudden death, Cardiomyopathy, | ||
|Approved=Yes | |Approved=Yes | ||
}} | }} | ||
{{WBRImage}} | {{WBRImage}} | ||
Revision as of 20:01, 15 March 2014
| Author | PageAuthor::Gonzalo Romero (Reviewed by Rim Halaby) |
|---|---|
| Exam Type | ExamType::USMLE Step 1 |
| Main Category | MainCategory::Pathology |
| Sub Category | SubCategory::Cardiology |
| Prompt | [[Prompt::A 21-year-old healthy male college student is playing in the soccer championship for his university when he suddenly falls on the ground while running. The player is unresponsive to athletic staff on the field. Emergency Medical Services arrive promptly, but CPR and resuscitation measures are unsuccessful. According to his family and friends, the patient has always been healthy and he has been playing soccer since high school. Autopsies are obtained in order to determine the cause of death. Which of the following cardiac macroscopic or microscopic changes is most likely present?]] |
| Answer A | AnswerA::Symmetric left ventricular hypertrophy |
| Answer A Explanation | [[AnswerAExp::Symmetric left ventricular hypertrophy is characteristically present in patients with increased afterload, such as aortic stenosis or hypertension. This leads to the increased synthesis of actin and myosin that are arranged in a "organized fashion". Ultimately, these patients develop diastolic dysfunction due to the inability of the heart to fill in with blood during diastole.]] |
| Answer B | AnswerB::White appearance of the endocardium |
| Answer B Explanation | [[AnswerBExp::A white appearance of the endocardium can be present in endocardial fibroelastosis, a rare restrictive cardiomyopathy present in young children less than 2 years old. It is caused by the excessive fibrosis of the endocardium that causes diastolic dysfunction. Shown below is an image depicting an autopsy of a heart affected with endocardial fibroelastosis.
 |
| Answer C | AnswerC::Cardiomyocyte hypertrophy in an organized fashion |
| Answer C Explanation | [[AnswerCExp::Cardiomyocyte hypertrophy in an organized fashion is found among patients with increased afterload such as aortic stenosis or hypertension.]] |
| Answer D | AnswerD::Prominent ventricular septum hypertrophy |
| Answer D Explanation | [[AnswerDExp::Prominent ventricular septum hypertrophy is a characteristic finding of hypertrophic cardiomyopathy.]] |
| Answer E | AnswerE::Fibrotic thickening of the endocardium and the valves of the right side of the heart |
| Answer E Explanation | [[AnswerEExp::Fibrotic thickening of the endocardium and the valves of the right side of the heart is the macroscopic description of an endocardium affected by a carcinoid syndrome due to chronic serotonin exposure, which causes fibrosis of the tricuspid valve and pulmonary valve. Carcinoid syndrome occurs when the carcinoid tumor metastasizes to the liver, thereby bypassing the first-pass metabolism of the liver. These patients present with diarrhea, wheezing, telangiectasias, and flushing of the skin.]] |
| Right Answer | RightAnswer::D |
| Explanation | [[Explanation::This young athlete presents with sudden cardiac death during intense exercise due to ventricular arrhythmias, a typical clinical presentation of hypertrophic cardiomyopathy. Hypertrophic cardiomyopathy can be either autosomal dominant or idiopathic.
Educational Objective: Hypertrophic obstructive cardiomyopathy (HOCM) is a common cause of sudden death in young athletes during intense exercise. It is characterized by the presence of cardiac hypertrophy more prominent in the ventricular septum and hypertrophied cardiomyocytes in disarray. |
| Approved | Approved::Yes |
| Keyword | WBRKeyword::Cardiology, WBRKeyword::Pathology, WBRKeyword::Hypertrophic cardiomyopathy, WBRKeyword::HOCM, WBRKeyword::HCM, WBRKeyword::Ischemia, WBRKeyword::Sudden death, WBRKeyword::Cardiomyopathy |
| Linked Question | Linked:: |
| Order in Linked Questions | LinkedOrder:: |
Image [[WBRImage::|]] Caption WBRImageCaption::no-display Position [[WBRImagePlace::|]]