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Authors: Robert Daum,Jason Canel

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And finally to you who hopefully purchased this book in preparation for your board examination or as an aid for your teaching efforts. To the former, welcome to our discipline of pediatric medicine. May you delight and profit from the knowledge we have tried to impart in these pages. May you also take a minute, should you disagree with any little thing, to inform us of inaccurate questions, statements, or answers. If there are to be future editions, we will make sure your advice is incorporated.
Good luck with your board examination. May this volume make your preparations more successful.
Robert S. Daum, MD, CM
Jason J. Canel, MD, FAAP

ACKNOWLEDGMENTS

 

 

 

Many images found in this book were made available by multiple sources including the personal archives of contributors and colleagues thereof.
The amazing diligence of current and former members of the faculty in the Department of Pediatrics at the University of Chicago have been a wonder to behold. While some have moved on to greener pastures, our sense of community persists and I hope will be reinforced by their belonging to the distinguished group of authors contributing to this book.
We are grateful for the outstanding support of Dr. Neha Kumar, who put forth a friendly face to those authors needing an extra week, month, or year to complete their contributions. She also helped facilitate the addition of numerous new images as well as general formatting for much of the book.
The McGraw-Hill staff has been supportive. In particular, we would like to thank Alyssa Fried, Sponsoring Editor, and Cindy Yoo, Development Editor, as well as Harleen Chopra at Glyph International. Without their support and encouragement, this project would not have been possible.
Finally, our thanks to you, our reader. For this project to be a success, we depend on both your need and enthusiasm for vignette-style learning and, ultimately, for our product. We hope to learn from you if there is any way to improve this book’s utility to your practice. We thereby encourage you to send us your ideas and comments for future editions.
Robert S. Daum, MD, CM
Jason J. Canel, MD, FAAP

Chapter 1

CARDIOLOGY

 

 

 

CASE 1: A NEONATE WITH A HEART MURMUR

 

A full-term newborn delivered without complications is noted to have a heart murmur at 1 day of age. The murmur is described as a grade 2/6 systolic ejection-type murmur heard best at the upper left sternal border. The heart sounds are normal. The blood pressure is normal. The peripheral pulses are normal. The oxygen saturation is 100% in room air. On day of life 3, the murmur has changed from a systolic murmur to a continuous murmur again heard best at the upper left sternal border. The baby appears well and is tolerating feeds without difficulties.

SELECT THE ONE BEST ANSWER

 

1.
What is the likely cause of this heart murmur?

(A) patent ductus arteriosus
(B) atrial septal defect
(C) ventricular septal defect
(D) peripheral pulmonary stenosis
(E) aortic stenosis

2.
This continuous murmur persists for more than 72 hours. What is the most appropriate test to evaluate the etiology of this murmur?

(A) chest radiograph
(B) electrocardiogram (ECG)
(C) complete blood count
(D) echocardiogram
(E) cardiac catheterization

3.
The echocardiogram demonstrates a small patent ductus arteriosus with no associated cardiac lesions and no evidence of left heart volume overload. What advice would you give to the family regarding further follow-up?

(A) no further cardiology follow-up is needed
(B) there is a lifelong ongoing risk for endarteritis and antibiotic prophylaxis should be given as needed
(C) immediate surgical repair is required
(D) there is minimal risk for endarteritis and no antibiotic prophylaxis is needed
(E) B and C

4.
At day of life 7, the neonate described develops increased shortness of breath, decreased feeding, and a chest radiograph shows pulmonary edema. What is the best first-line course of action?

(A) prostaglandin E
1
infusion
(B) a course of indomethacin
(C) supplemental oxygen
(D) nitric oxide
(E) phenylephrine

5.
A full-term newborn delivered via normal spontaneous vaginal delivery with Apgars of 9 at 1 minute and 9 at 5 minutes is noted to have a grade 2/6 systolic murmur at the left sternal border on day 1 of life. Upon reexamination on day 4 of life, the neonate is found to have poor perfusion, poor pulses, hypotension, and shock. What is the least likely cause of shock in this neonate?

(A) perinatal asphyxia
(B) sepsis
(C) hypoglycemia
(D) cardiogenic shock
(E) myocarditis

6.
Which of the following congenital heart lesions may result in cardiogenic shock following spontaneous closure of the ductus arteriosus?

(A) atrial septal defect
(B) hypoplastic left heart syndrome
(C) mitral valve prolapse
(D) ventricular septal defect
(E) tetralogy of Fallot

7.
What test would be most helpful to diagnose the cause of shock in this neonate?

(A) chest radiograph
(B) arterial blood gas
(C) blood glucose level
(D) echocardiogram
(E) a blood culture

8.
What is the most appropriate acute treatment for cardiogenic shock as a result of left heart obstructive lesions?

(A) prostaglandin E
1
infusion
(B) indomethacin administration
(C) supplemental oxygen administration
(D) nitric oxide
(E) intravenous (IV) furosemide

9.
What are other courses of action to be taken for patients with cardiogenic shock?

(A) inotropic medications such as epinephrine and dopamine
(B) ventilatory support
(C) oxygen supplementation
(D) correct any metabolic abnormalities
(E) all of the above

10.
A neonate with a heart murmur on examination is noted to have a bluish tinge to the lips. The baby is smiling and eating well in no apparent distress. The baby is warm with good pulses. An oxygen saturation monitor was placed on the baby’s finger and the oxygen saturation in room air is 80%. Which of the following is (are) likely to account for this patient’s finding of cyanosis?

(A) cyanotic congenital heart disease
(B) hemoglobinopathy
(C) respiratory disease
(D) anemia
(E) A, B, and C

11.
Which test would most likely help differentiate a cardiac versus pulmonary cause for cyanosis?

(A) hyperoxia test
(B) blood gas on room air
(C) chest radiograph
(D) complete blood count
(E) chest computed tomography (CT)

12.
What is the most common cyanotic heart lesion in the neonate?

(A) transposition of the great arteries
(B) total anomalous pulmonary venous return
(C) tricuspid atresia
(D) tetralogy of Fallot
(E) Ebstein anomaly

13.
What would be an appropriate first-line treatment for the management of cyanosis as a result of heart disease?

(A) prostaglandin E
1
infusion
(B) indomethacin administration
(C) nitric oxide
(D) surfactant
(E) dopamine

14.
A 4-year-old with a known diagnosis of unrepaired tetralogy of Fallot becomes severely cyanotic while crying. Which of the following is not indicated for acute management of this situation?

(A) administering of oxygen
(B) placing the child in the knee-chest position
(C) administering morphine sulfate
(D) rectal stimulation
(E) administration of phenylephrine

15.
Which of the following is not a complication of chronic cyanosis?

(A) polycythemia
(B) stroke
(C) brain abscess
(D) cognitive abnormalities
(E) cerebral aneurysms

ANSWERS

 

1.
(A)
The most likely cause of this murmur is a patent ductus arteriosus (
Figure 1-1
). Atrial septal defects are unlikely to cause a heart murmur at this early age, and ventricular septal defects cause a holosystolic murmur that does not evolve into a continuous murmur. Peripheral pulmonary stenosis is associated with a systolic ejection murmur that radiates to the back and to both axillae. Aortic stenosis causes a systolic murmur that does not evolve into a continuous murmur. The ductus arteriosus is required for fetal circulation; however, the structure usually closes within hours after delivery secondary to changes in oxygen tension as well as other circulating factors such as prostaglandin. Persistence of the ductus arteriosus occurs in approximately 0.8 per 1000 live births but is much more common in premature newborns with an incidence as high as 20% in neonates weighing less than 1750 g. In patients with low oxygen tension, pulmonary hypertension, or congenital heart disease, there is delayed closure of the ductus arteriosus.

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