Vignettes: Peds Cardiology # 1

1. You are evaluating a 15-year-old boy in your office. He tells you that he experiences chest pain at times while at home and at school. He describes it as sharp, located in the left chest, exacerbated by deep breathing, and resolving spontaneously. He does not feel palpitations or lightheadedness and has not had syncope. His 54-year-old father and grandfather both have had hypertension and myocardial infarctions. At this time, he has no pain, and results of physical examination are normal.

= Reassurance with clinical follow-up

i) In fact, the likelihood that chest pain in an otherwise well child represents cardiovascular disease is extremely low. Overall, the incidence of chest pain attributable to a cardiac cause is less than 1% in children.

ii) Pain that is constant or frequent, dull, pressurelike, or associated with exercise is more likely to be associated with cardiac causes than is pain that is brief, infrequent, and sharp, as described for the boy in the vignette.

Pain that worsens with inspiration is generally reassuring, suggesting a musculoskeletal or pulmonary rather than cardiac cause.

2. A 16-year-old boy has experienced chest pain twice with intense exercise during practice for his high school varsity soccer team. Each time the pain felt like pressure, radiated to his left shoulder, and was associated with lightheadedness. He did not seek medical attention after either episode. His father, who is age 49 years, has hypertension and uses lipid-lowering medication. Results of the boy's physical examination are normal, including his blood pressure and cardiovascular examination.

Of the following, the BEST management plan is:

cardiology referral, with restriction of all exercise pending evaluation.

Now read:

As for the boy described in the vignette, the pain typically occurs during or following exercise or activity, and it may improve with rest. The clinician should consider myocardial ischemia strongly in the patient who has had Kawasaki disease or cardiac surgery.

It also is important to consider use of illicit drugs, such as cocaine and other adrenergic stimulators, as potential causes of coronary vasospasm.

Some of the tachyarrhythmias, such as supraventricular tachycardia, may present with chest pain, although this usually is described as a discomfort associated with palpitation and other symptoms. Although it may be associated with exercise, this is not the typical presentation.

Pain from aortic dissection is usually acute and sharp and may present in the anterior chest or the back, depending on the area of the aorta that is affected.

A history of Marfan syndrome or Ehlers-Danlos syndrome should be pursued in affected patients and their families.

3. A 2-week-old infant presents to the emergency department with a 1-day history of decreased feeding, pallor, diaphoresis, and increasing somnolence. He was born at term, and the delivery was uncomplicated. On physical examination, his heart rate is 190 beats/min, his respiratory rate is 80 breaths/min, his blood pressure is 50/30 mm Hg, and his extremities are cool and pale with poor pulses. You place the infant on a cardio-respiratory monitor and begin your assessment and management.

Ans: Do ECG + ECHO

Well, this is CARDIOGENIC SHOCK. Beware!!

i) In the first weeks after birth, cardiogenic shock often is due to cardiac anomalies.

ii) Therefore, left heart obstructive lesions should be considered in any neonate who presents with congestive failure and shock, and administration of prostaglandin E to establish or maintain ductal patency should be considered. Among these are critical aortic stenosis, aortic coarctation, interruption of the aortic arch, and hypoplastic left heart syndrome.

4. Two true statements about TOF (Tetralogy of Fallot):

i) complete repair is associated with an excellent result, with less than 5 % mortality.

ii)TOF is somtimes associated with chromosomal abnormalities.

It is now known that TOF can be associated with microdeletions in the q11 region of chromosome 22. Some reports suggest that such deletions occur in up to 20% of patients who have TOF.

5. A 5-day-old child is brought to ER because he has been difficult to arouse over the last 6 hours. He has not been interested in feeding today and that he has been breathing rapidly and with a grunting noise. P/E: HR 185 , RR 80, and BP 55/40. A pulse is palpable in the right brachial region, but not in the feet. All of his extremities are cool and mottled, with a capillary refill of more than 2 seconds.

= Initiate a prostaglandin infusion

This picture is consistent with critical Coarc of aorta.

i) Left heart obstruction in the newborn often has a dramatic presentation that may include shock, cardiovascular collapse, and death if not recognized in a timely manner.

ii) HLHS and coarctation of the aorta are the two most common forms of left heart obstructive congenital heart disease that present in the first few postnatal days.