Collapse of an early adolescent girl. Syncope? Simple or sinister?

An early adolescent girl presented to the emergency department (ED) of her local hospital following a syncopal episode after a warm bath on a background of increasing breathlessness for 1-year duration. On examination, she was fully alert, no pain or injuries from her syncope, she was warm and well perfused, her respiratory rate at rest was normal and she showed no signs of respiratory distress, there were no signs of seizure activity such as incontinence or tongue biting, she had a raised Jugular Venous Pressure (JVP), a loud pansystolic murmur, there was no cyanosis and no oedema. She was referred for a chest radiograph due to her history of breathlessness and for an ECG based on the examination findings of a murmur. Her ECG showed marked right axis deviation with tall T waves in V1 and P waves in lead II. Therefore, she was referred for an outpatient echocardiogram which demonstrated severely dilated right ventricular (RV) with impaired RV function. She was diagnosed with primary pulmonary hypertension.Her history included red flag symptoms including worsening breathlessness and syncope. Syncopal episodes are common and mostly benign, that is, vasovagal, but attention to a detailed history including constitutional symptoms and examination is crucial to ensure appropriate investigations and management. While causes are commonly benign in paediatrics, it is important to ensure that there are no features of an underlying cause. This includes ruling out commonly seen paediatric presentations that could present as an unwitnessed syncopal episode and this should include seizures, anaemia, arrhythmias, hypotension, hypoglycaemia and pain. If other causes are not ruled out, it can lead to an incorrect diagnosis as found in this patient when she presented a further three times to ED while waiting for her tertiary appointment.