A 21 Year old male presented with syncope on exertion

Brockenbrough–Braunwald–Morrow sign

A 21 Year old male presented with syncope on exertion. Family history positive for Sudden Cardiac Death. Examination revealed Ejection Systolic Murmur at left sternal border and brisk carotid upstroke (not radiating to neck). Murmur accentuated on standing. ECG showing dagger like Q waves in anterolateral leads.

a) What is the most likely diagnosis?

b) What is brocken brough phenomenon?

c) What are the risk factors for Sudden Cardiac Death in this disease?

d) Name two treatment options available and which will you consider for this patient and why?

Answers:

a.      Hypertrophic cardiomyopathy

b.      Severe increase in the Left ventricular aortic gradient in the beat after a premature ventricular contraction (PVC) due to increase in contractility and decrease in afterload during the post PVC beat.

c.       Risk factors for sudden death

• Previous Cardiac arrest
• Sustained VT
• Prolonged or repetitive episodes of non-sustained VT on holter
• Wall thickness >30mm
• Family history of SCD
• No change or decrease in BP with exercise
• Syncope or near syncope

d.      Medical therapy – beta blockers. Septal Myectomy, Alcohol Septal Ablation                  

Reference:

1.      Chapter 10, page 172– Hypertrophic cardiomyopathy - Manual of Cardiovascular Medicine Fourth Edition - Brian P. Griffin MD FACC

2.      Figure 10.1, Chapter  10, page 166. - Manual of Cardiovascular Medicine Fourth Edition - Brian P. Griffin MD FACC