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{{WBRQuestion
{{WBRQuestion
|QuestionAuthor=Gerald Chi
|QuestionAuthor=Gerald Chi (Reviewed by {{YD}})
|ExamType=USMLE Step 1
|ExamType=USMLE Step 1
|MainCategory=Anatomy
|MainCategory=Anatomy
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|MainCategory=Anatomy
|MainCategory=Anatomy
|SubCategory=Cardiology
|SubCategory=Cardiology
|MainCategory=Anatomy
|MainCategory=Anatomy
|MainCategory=Anatomy
|MainCategory=Anatomy
|MainCategory=Anatomy
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|MainCategory=Anatomy
|MainCategory=Anatomy
|SubCategory=Cardiology
|SubCategory=Cardiology
|Prompt=A newborn presents with vomiting when fed for the first time. Flat facies, an unusually large tongue, and prominent epicanthal folds are noted on examination. Upper gastrointestinal studies show gas in the stomach and the duodenal bulb. The newborn is most likely to have cardiovascular anomalies due to which of the following defects?
|Prompt=A newborn boy is evaluated for vomiting when fed for the first time. Physical examination is remarkable for flat facies, an unusually large tongue, and prominent epicanthal folds. Upper gastrointestinal studies demonstrate gas in the stomach and the duodenal bulb. Which of the following cardiovascular defects is mostly associated with his newborn's underlying genetic disease?
 
|Explanation=Down syndrome (trisomy 21), as evidenced by the phenotypic profile and the double bubble sign secondary to duodenal atresia, is most commonly associated with the cardiovascular complication of endocardial cushion defects.


|Explanation=The patient is most likely diagnosed with Down syndrome (trisomy 21), as evidenced by the phenotypic profile, Down syndrome is associated with duodenal atresia, which is characterized by the congenital underdevelopment of parts of the duodenum and is suggested by the vomiting and the double bubble sign on imaging. Down syndrome is strongly associated with an increased risk of congenital cardiovascular disease, especially endocardial cushion defects (AV canal). Down syndrome is also associated with other congenital heart diseases (less commonly than with endocardial cushion defects), namely ventricular septal defects (VSD) and atrial septal defects (ASD).
|AnswerA=Ectopic ductal tissue
|AnswerA=Ectopic ductal tissue
 
|AnswerAExp=Coarctation of the aorta, which is commonly associated with Turner syndrome, results from ectopic ductal tissue or from abnormal development of the fourth and sixth aortic arch.
|AnswerAExp=Coarctation of the aorta, which is commonly seen in Turner syndrome, is postulated to result from ectopic ductal tissue or from abnormal development of the fourth and sixth aortic arch.


|AnswerB=Improper alignment of aorticopulmonary septum
|AnswerB=Improper alignment of aorticopulmonary septum
|AnswerBExp=Tetralogy of Fallot is caused by improper alignment of aorticopulmonary septum.
|AnswerBExp=Tetralogy of Fallot is caused by improper alignment of aorticopulmonary septum.
|AnswerC=Failure of aorticopulmonary septum to twist
|AnswerC=Failure of aorticopulmonary septum to twist
 
|AnswerCExp=Transposition of the great vessels is caused by failure of aorticopulmonary septum to twist and is commonly present in children of diabetic mothers.
|AnswerCExp=Transposition of the great vessels is caused by failure of aorticopulmonary septm to twist and is commonly seen in children of diabetic mothers
 
|AnswerD=Inadequate formation of the septum secundum
|AnswerD=Inadequate formation of the septum secundum
 
|AnswerDExp=Ostium secundum atrial septal defect (ASD) is the most common type of ASD. It arises from inadequate growth of the septum secundum and comprises up to 10% of all cases of congenital heart diseases. Although ASD is associated with Down syndrome, endocardial cushion defects are more common.
|AnswerDExp=Ostium secundum atrial septal defect is the most common type of ASD. It arises from inadequate growth of the septum secundum and comprises up to 10% of all cases of congenital heart diseases.
 
|AnswerE=Inadequate migration of neural crest cells
|AnswerE=Inadequate migration of neural crest cells
 
|AnswerEExp=Failure of migration of neural crest cells to the endocardial cushion is the most common congenital heart disease associated with Down syndrome.
|AnswerEExp=Failure of migration of neural crest cells to the endocardial cushion is most commonly associated with Down syndrome
|EducationalObjectives=Down syndrome is strongly associated with endocardial cushion defects (formation of AV canal), which is characterized by failure of migration of neural crest cells to the endocardial cushion.
|References=FIrst Aid 2015 page 290.
|RightAnswer=E
|RightAnswer=E
|WBRKeyword=Down syndrome
|WBRKeyword=Down syndrome, AV canal, Endocardial cushion defect
|Approved=No
|Approved=Yes
}}
}}

Revision as of 01:28, 17 August 2015

 
Author [[PageAuthor::Gerald Chi (Reviewed by Yazan Daaboul, M.D.)]]
Exam Type ExamType::USMLE Step 1
Main Category MainCategory::Anatomy
Sub Category SubCategory::Cardiology
Prompt [[Prompt::A newborn boy is evaluated for vomiting when fed for the first time. Physical examination is remarkable for flat facies, an unusually large tongue, and prominent epicanthal folds. Upper gastrointestinal studies demonstrate gas in the stomach and the duodenal bulb. Which of the following cardiovascular defects is mostly associated with his newborn's underlying genetic disease?]]
Answer A AnswerA::Ectopic ductal tissue
Answer A Explanation AnswerAExp::Coarctation of the aorta, which is commonly associated with Turner syndrome, results from ectopic ductal tissue or from abnormal development of the fourth and sixth aortic arch.
Answer B AnswerB::Improper alignment of aorticopulmonary septum
Answer B Explanation AnswerBExp::Tetralogy of Fallot is caused by improper alignment of aorticopulmonary septum.
Answer C AnswerC::Failure of aorticopulmonary septum to twist
Answer C Explanation AnswerCExp::Transposition of the great vessels is caused by failure of aorticopulmonary septum to twist and is commonly present in children of diabetic mothers.
Answer D AnswerD::Inadequate formation of the septum secundum
Answer D Explanation [[AnswerDExp::Ostium secundum atrial septal defect (ASD) is the most common type of ASD. It arises from inadequate growth of the septum secundum and comprises up to 10% of all cases of congenital heart diseases. Although ASD is associated with Down syndrome, endocardial cushion defects are more common.]]
Answer E AnswerE::Inadequate migration of neural crest cells
Answer E Explanation AnswerEExp::Failure of migration of neural crest cells to the endocardial cushion is the most common congenital heart disease associated with Down syndrome.
Right Answer RightAnswer::E
Explanation [[Explanation::The patient is most likely diagnosed with Down syndrome (trisomy 21), as evidenced by the phenotypic profile, Down syndrome is associated with duodenal atresia, which is characterized by the congenital underdevelopment of parts of the duodenum and is suggested by the vomiting and the double bubble sign on imaging. Down syndrome is strongly associated with an increased risk of congenital cardiovascular disease, especially endocardial cushion defects (AV canal). Down syndrome is also associated with other congenital heart diseases (less commonly than with endocardial cushion defects), namely ventricular septal defects (VSD) and atrial septal defects (ASD).

Educational Objective: Down syndrome is strongly associated with endocardial cushion defects (formation of AV canal), which is characterized by failure of migration of neural crest cells to the endocardial cushion.
References: FIrst Aid 2015 page 290.]]

Approved Approved::Yes
Keyword WBRKeyword::Down syndrome, WBRKeyword::AV canal, WBRKeyword::Endocardial cushion defect
Linked Question Linked::
Order in Linked Questions LinkedOrder::