Chapter·Internal MedicineArrhythmias

Inherited arrhythmia syndromesDownloads

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1

A 43-year-old woman presents to her primary care provider with shortness of breath. She reports a 4-month history of progressively worsening difficulty breathing with associated occasional chest pain. She is a long-distance runner but has had trouble running recently due to her breathing difficulties. Her past medical history is notable for well-controlled hypertension for which she takes hydrochlorothiazide. She had a tibial osteosarcoma lesion with pulmonary metastases as a child and successfully underwent chemotherapy and surgical resection. She has a 10 pack-year smoking history but quit 15 years ago. She drinks a glass of wine 3 times per week. Her temperature is 98.6°F (37°C), blood pressure is 140/85 mmHg, pulse is 82/min, and respirations are 18/min. On exam, she has increased work of breathing with a normal S1 and loud P2. An echocardiogram in this patient would most likely reveal which of the following?

ABiventricular dilatation with a decreased ejection fraction

BLeft ventricular dilatation with an incompetent aortic valve

CLeft atrial dilatation with mitral valve stenosis

DRight ventricular hypertrophy with a dilated pulmonary artery

ELeft ventricular hypertrophy with a bicuspid aortic valve

2

An ECG from an 8-year-old male with neurosensory deafness and a family history of sudden cardiac arrest demonstrates QT-interval prolongation. Which of the following is this patient most at risk of developing?

AHypertrophic cardiac myopathy

BCardiac tamponade

CEssential hypertension

DTorsades de pointes

EFirst degree atrioventricular block

3

An 8-year-old girl is brought to the physician by her parents for the evaluation of an episode of unconsciousness while at the playground that morning. She was unconscious for about 15 seconds and did not shake, bite her tongue, or lose bowel or bladder control. Her grandfather died suddenly at the age of 29 of an unknown heart condition; her parents are both healthy. An ECG shows sinus rhythm and a QT interval corrected for heart rate (QTc) of 470 milliseconds. Laboratory studies are within normal limits. Which of the following is the most likely additional finding in this patient?

AOblique palpebral fissures

BSubvalvular ventricular outflow obstruction murmur

CSensorineural hearing loss

DBrachial-femoral pulse delay

ESkin folds between the mastoid process and acromion

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