Rheumatic mitral stenosis is considered to be a disease of past, especially in younger population in developed countries. [1] We came across a young Caucasian who presented with severe rheumatic mitral stenosis associated with pulmonary hypertension; indicating that rheumatic heart disease although declining in incidence in the United Kingdom, may still present at a relatively young age. In underdeveloped countries the incidence of rheumatic heart disease is still numerous. [2]

A 30-year-old Caucasian male from a low socioeconomic background was seen in the clinic with one year history of worsening exertional dyspnoea and orthopnoea. He was not an immigrant from any underdeveloped country nor had lived in underdeveloped country during childhood or teenage. He had no recollection of any previous symptoms of rheumatic fever. No other past medical illness of note apart from mild asthma. He admitted to intravenous drug abuse in the past.

On clinical examination, he was slim built with a normal blood pressure and a regular pulse. No evidence of peripheral oedema was present. Jugular venous pressure was not raised. Cardiovascular examination revealed a loud first heart sound, opening snap and mid diastolic murmur with presystolic accentuation. Electrocardiograph demonstrated sinus rhythm with right bundle branch block along with evidence of left atrial enlargement. Transthoracic and transoesophageal echocardiography (Figure 1) confirmed severe rheumatic mitral stenosis with thickening and fusion of the commissures, mitral valve area of 0.7-0.9 cm² measured by planimetry method, mild mitral regurgitation and left atrial dilatation. There was also marked pulmonary hypertension, with a systolic pulmonary artery pressure estimated to be 70 mmHg. These findings were confirmed at cardiac catheterisation together with normal appearance of coronary arteries. The clinical and biochemical markers were not suggestive of infective endocarditis, given his intravenous drug abuse. He underwent percutaneous balloon mitral valvuloplasty successfully. Post-operative echocardiogram showed an increase in the mitral valve area to 1.4 cm² (pressure half-time method) and regression of left atrial pressure. Subsequent patient follow up in the clinic showed symptomatic improvement.

Rheumatic mitral stenosis in young population is now considered to be rare in developed countries with an incidence of less than 1 per 100,000 population [1] and relates to the decline of rheumatic fever. A similar condition occasionally occurs in association with other diseases such as systemic lupus or infective endocarditis. [3] However, our case report highlights that rheumatic mitral stenosis in young from low socioeconomic background, still exists and is a cause of concern in the United Kingdom. Consequently, we should remain vigilant and be aware of the presenting symptoms and signs of rheumatic fever and rheumatic heart disease. [4] Another interesting feature to note in this case is the marked degree of pulmonary hypertension secondary to mitral stenosis, which is particularly uncommon in this age group, as it has been estimated that progression from mild to severely disabling symptoms usually takes up to 10 years. [5]

From United Kingdom experience, in the year 2006, a total of 153 of percutaneous mitral valvuloplasty procedures were performed. [6] In the north west region of UK, where this patient came from, there were none in this age group in the last seven years.

Our case emphasises that despite the decline in the incidence of rheumatic heart disease in the UK, severe mitral stenosis may still occur even in young population in their third decade. Consequently, we should remain vigilant and be aware of the presenting symptoms and signs of rheumatic fever and rheumatic heart disease.

1. Rheumatic fever and rheumatic heart disease: report of a WHO expert consultation, Geneva. WHO Tech Rep Ser 2001;923. http://www.who.int/cardiovascular-diseases/resources/trs923/en/rheumatic%20heart%20disease.    
2. Carapetis JR, Steer AC, Mulholland EK, Weber M. The global burden of group A streptococcal diseases. Lancet Infect Dis 2005;5:685-694.   [Pubmed]    
3. Kinkare SG. and Kulkarni HL. Quantitative study of mitral valve in chronic rheumatic heart disease. Int J Cardiology 1987;16:271-72.   [Pubmed]    
4. Wood P. An appreciation of mitral stenosis: Clinical features. Br Med J 1954;1(4870):1051-63.   [CrossRef]   [Pubmed]    
5. Rowe JC, Bland EF, Sprague HB, White PD. The course of mitral stenosis without surgery: ten - and twenty- year perspectives. Ann Intern Med 1960;52:741-9.   [Pubmed]    
6. Ludman PF, BCIS Audit Returns 2006. Adult Interventional Procedures. Bcis.org.uk [homepage on the Internet].[British Cardiovascular Intervention Society] http://www.bcis.org.uk/. Accessed December 13, 2010.