Tag Archives: aetiology

Diagnosis can be challenging as its symptoms are often non-specific: they may include breathlessness, fatigue, weakness, angina, syncope and abdominal distension. In the mid-1980s, before the availability of ‘targeted’ therapy, median life expectancy from diagnosis in patients with idiopathic PAH (formerly termed primary pulmonary hypertension [PPH]) was only 2.8 years.3 In 1996, continuous intravenous prostacyclin (epoprostenol) was the first drug to demonstrate outcome benefit in PAH.4 Subsequently, over the past ten years, randomised, placebo-controlled trials of other prostacyclin analogues, endothelin receptor antagonists and phospho

Case report Figure 1. Electrocardiogram (ECG) features of left ventricular hypertrophy A 67-year-old man was referred to the cardiology clinic with a history of collapse and a family history of hypertrophic cardiomyopathy (HCM). He denied any history of angina or dyspnoea. On physical examination he had a 3/6 grade ejection murmur in the aortic area and a pansystolic murmur at the mitral area accentuated on squatting. There were no signs of volume overload. X-ray angiography demonstrated normal coronaries but a raised end diastolic pressure. His electrocardiogram (ECG) was compatible with left ventricular (LV) hypertrophy (figure 1). A single

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