Cardiac arrest in a patient with trichorhinophalangeal syndrome and dilated cardiomyopathy.
Gautam SenEleanor BarendtManas SinhaPublished in: BMJ case reports (2021)
A 44-year-old woman with known trichorhinophalangeal syndrome presented with an unheralded out of hospital cardiac arrest. Transthoracic echocardiography showed severe left ventricular systolic dysfunction with an ejection fraction <25% and cardiac MRI confirmed a diagnosis of congenital non-ischaemic dilated cardiomyopathy. The case highlights a very rare syndrome, it is previously unknown association with dilated cardiomyopathy and the possible benefit of cardiac screening for patients with known trichorhinophalangeal syndrome.
Keyphrases
- left ventricular
- case report
- ejection fraction
- cardiac arrest
- aortic stenosis
- heart failure
- magnetic resonance imaging
- blood pressure
- acute myocardial infarction
- hypertrophic cardiomyopathy
- computed tomography
- mitral valve
- left atrial
- cardiac resynchronization therapy
- acute coronary syndrome
- oxidative stress
- pulmonary hypertension
- cardiopulmonary resuscitation
- early onset
- drug induced
- percutaneous coronary intervention
- atrial fibrillation