A 49-year-old man with idiopathic pulmonary arterial hypertension has a negative acute vasodilator test.
Which one of the following medications is least likely to be beneficial in his long-term management?
Correct Answer A:
Oral calcium channel blockers are unlikely to be beneficial following a negative acute vasodilator test.
Pulmonary arterial hypertension: features and management:
Pulmonary arterial hypertension (PAH) may be defined as a sustained elevation in mean pulmonary arterial pressure of greater than 25 mmHg at rest or 30 mmHg after exercise.
Features:
Management should first involve treating any underlying conditions, for example with anticoagulants or oxygen.
Following this, it has now been shown that acute vasodilator testing is central to deciding on the appropriate management strategy. Acute vasodilator testing aims to decide which patients show a significant fall in pulmonary arterial pressure following the administration of vasodilators such as intravenous epoprostenol or inhaled nitric oxide.
If there is a positive response to acute vasodilator testing:
If there is a negative response to acute vasodilator testing:
Which of the following is least associated with primary pulmonary hypertension?
Correct Answer C:
Recurrent pulmonary embolism is a cause of secondary pulmonary hypertension Some candidates have questioned whether HIV, cocaine and fenfluramine should be regarded as secondary causes of pulmonary hypertension. This is a fair point and the situation should be improved with the new classification of pulmonary hypertension. However, faced with this question in the exam the correct answer would be recurrent pulmonary embolism - a classical cause of secondary pulmonary hypertension.
Primary pulmonary hypertension:
The classification of pulmonary hypertension is currently changing with the term idiopathic pulmonary arterial hypertension (IPAH) becoming more widely used.
Primary pulmonary hypertension (PPH, now IPAH):
Investigation:
Management:
You review a 47-year-old man in clinic. He has been referred with difficult to control hypertension despite taking a combination of lisinopril, indapamide and amlodipine. Whilst examining the patient you note over 15 large cafeau-lait spots on his trunk. Some axillary freckling is also present. His blood pressure (despite medication) is 170/94 mmHg and pulse 90/min.
Routine bloods are ordered:
Urine dipstick is normal.
Which one of the following investigations is most likely to be diagnostic?
Correct Answer E:
Patients with neurofibromatosis may develop hypertension for three main reasons:
The normal renal function in this patient points away from a diagnosis of renal vascular disease. Phaechromocytoma therefore needs to be excluded first.
Neurofibromatosis:
There are two types of neurofibromatosis, NF1 and NF2. Both are inherited in an autosomal dominant fashion NF1 is also known as von Recklinghausen's syndrome. It is caused by a gene mutation on chromosome 17 which encodes Neurofibromin and affects around 1 in 4,000 NF2 is caused by gene mutation on chromosome 22 and affects around 1 in 100,000.
A 29-year-old man with myotonic dystrophy has an electrocardiogram.
Which one of the following findings is most likely to be present?
A prolonged PR interval is seen in around 20-40% of patients.
Myotonic dystrophy:
Myotonic dystrophy (also called dystrophia myotonica) is an inherited myopathy with features developing at around 20-30 years old. It affects skeletal, cardiac and smooth muscle. There are two main types of myotonic dystrophy, DM1 and DM2.
Genetics:
General features:
Other features:
A 65-year-old man is admitted with palpitations. The ECG shows a ventricular rate of 150/min with an underlying atrial rate of 300/min. A diagnosis of atrial flutter is suspected.
What is the treatment of choice to permanently restore sinus rhythm?
Atrial flutter:
Atrial flutter is a form of supraventricular tachycardia characterized by a succession of rapid atrial depolarization waves.
ECG findings:
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