Pediatrics

A 14 year old boy came in for pre-sport screening he had systolic murmur, his dad has hypertrophic…

A 14 year old boy came in for pre-sport screening he had systolic murmur, his dad has hypertrophic cardiomyopathy and died at 40 years old. ECG shows long QT.

  1. A Continue sport and do annual ECG
  2. B Give beta blocker and only do sports that require low effort Correct answer
  3. C Restrict sport and consider pacemaker
  4. D It is ok to do sports

Explanation

Core Concept: Long QT syndrome (LQTS) and Hypertrophic Cardiomyopathy (HCM) are inherited conditions that predispose individuals to sudden cardiac death (SCD) during exertion. Beta-blockers and activity restriction are mainstays of therapy.

Clinical Presentation:

  • Syncope, seizures, or sudden cardiac arrest during exercise or emotional stress.
  • Family history of sudden cardiac death at a young age.

Diagnosis:

  • First-line tool: 12-lead ECG (prolonged QTc interval) and Echocardiography (for HCM).
  • Gold standard: Genetic testing to identify specific channelopathy or sarcomere mutations.

Management:

  • Acute: Avoidance of QT-prolonging drugs and correction of electrolyte abnormalities.
  • Long-term/Definitive: Beta-blockers (e.g., nadolol, propranolol) to blunt sympathetic surges. Restriction from competitive and high-intensity sports; only low-effort recreational activities are permitted. ICD placement if high risk.

40 free questions with an account

The questions published here are a fixed sample. Create a free account to practise on the full bank, with performance analytics by specialty and a page for reviewing your mistakes.

Create a free account

No payment card