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Acyanotic congenital heart defects: Pathology review




Cardiovascular system

Vascular disorders
Congenital heart defects
Cardiac arrhythmias
Valvular disorders
Heart failure
Cardiac infections
Pericardial disorders
Cardiac tumors
Cardiovascular system pathology review

Acyanotic congenital heart defects: Pathology review


1 / 9 complete

USMLE® Step 1 style questions USMLE

9 questions

A 15-year-old girl is brought to the pediatrician for evaluation of leg pain that is exacerbated by exercise. The patient reports having difficulty keeping up with her classmates while playing soccer at school. She has not started having menses. Her mother had menarche at the age of 12. The patient’s temperature is 37.2°C (99.0°F), pulse is 80/min, respirations are 14/min. Upper extremity blood pressure is 140/81 mmHg, and lower extremity blood pressure is 118/70 mmHg. Physical examination reveals a low posterior hairline, Tanner stage I breasts and webbed neck.  Which of the following additional examination findings will most likely be seen in this patient? 


Content Reviewers:

Antonia Syrnioti, MD

In a pediatric cardiology clinic, 4-year-old Tara is brought in by her parents because she has not been acting herself over the past month. The mother also mentioned that she can’t keep up with the other children when playing and often gets fatigued or short of breath. Vital signs include a temperature of 37.0 degrees Celsius or 98.6 degrees Fahrenheit, a heart rate of 100 beats per minute, a blood pressure of 110 over 70 mmHg, and a respiratory rate of 18 breaths per minute. On examination, her skin is pink, and auscultation of the heart reveals a holosystolic murmur over the left sternal border.

Ok, so Tara has some sort of congenital heart defect. Congenital heart diseases are defects in the embryological development of the heart or its major blood vessels. When the defect causes blood to move from the right to the left side, it’s called a right-to-left shunt. This is because deoxygenated blood from the right side goes to the left side, and then enters the systemic circulation. A large amount of deoxygenated blood in the systemic circulation gives the physical appearance of cyanosis, which is a bluish discoloration of the skin. Therefore, right-to-left defects are called cyanotic heart diseases. Conversely, left-to-right shunts are called acyanotic heart defects, because there is no cyanosis. In general, individuals with acyanotic congenital heart diseases could be asymptomatic or present with signs of heart failure, such as exercise intolerance, shortness of breath, and in the case of infants and young children; poor feeding and failure to thrive.

Okay, of the acyanotic congenital heart diseases, ventricular septal defect, or VSD, is the most common. The ventricular septum normally separates the left and right ventricles, and is made of a membranous component, which is the upper one-third, and a muscular component, which is the lower two-thirds. The defect most commonly occurs in the membranous portion of the septum. Ventricular septal defects are usually small and they often end up closing on their own. Individuals are asymptomatic at birth, and if symptoms develop, they usually occur a couple of weeks later or even later in life. This is a helpful clue on exams. On auscultation, a systolic murmur can be heard along the left sternal border. Bear in mind though that the smaller the defect, the more audible the murmur. That’s because when blood rushes through a more narrow opening, it produces more turbulence and therefore, more noise. In addition, because the defect allows