Heart valve disease module 3: disease presentation

Pulmonary stenosis

Isolated pulmonary stenosis (PS) is generally a benign valve lesion, with overall survival at 25 years of 96%.⁸⁸ It is commonly a feature of inherited or congenital syndromes. Mild PS in adulthood is unlikely to show significant progression, although interval follow up is recommended. However, once the peak gradient across the valve exceeds 50 mmHg, the event-free survival at 25 years is just 31%. Moderate PS (peak gradient 36–60 mmHg) at initial assessment has been associated with a 24% risk of requiring intervention in the subsequent 25 years. Once the PS is severe (peak gradient >60 mmHg), symptoms are more likely and cyanosis may occur.

Compensation for disease progression involves RV hypertrophy and right atrial dilatation. Once these mechanisms fail, symptoms of dyspnoea, fatigue and exertional syncope may develop.

Signs of pulmonary stenosis include an ejection systolic murmur heard in the pulmonary area, louder in inspiration, and which may have a quiet P2. Once RV hypertrophy is significant, an RV heave may be detectable. Prominent A waves may be seen in the JVP. RV failure may also contribute to clinical signs, as may features of associated congenital or genetic disorders.

Pulmonary regurgitation

Mild or trivial pulmonary regurgitation (PR) is a normal finding in most people. Severe PR is rare and may lead to RV dilatation and impaired RV systolic function; these are associated with increased risk of malignant arrhythmias and sudden cardiac death.^89–92

Symptoms, which develop after many years, are:

• dyspnoea on exertion, then at rest
• peripheral oedema
• jaundice and abdominal distension.

Palpitations may reflect atrial arrhythmias, and pre-syncope may be the result of ventricular tachycardia.

Signs of PR itself may be difficult to detect. A diastolic murmur may be heard at the lower left-sternal edge, reflecting the direction of regurgitant flow. The increased stroke volume from the RV may lead to a systolic murmur at the upper left-sternal edge. Signs relating to RV dilatation and failure may again be detected.

Conclusions

HVD is progressive, with a highly variable rate of progression between individuals. The onset of symptoms and LV dysfunction is unpredictable. Once HVD is detected, appropriate surveillance is vital to ensure timely intervention if required.

This module has reviewed the common symptoms and physical signs of heart valve disease. Other modules will review investigations and imaging modalities, echocardiography in particular, which complement cardiac auscultation and the patient’s clinical history, in determining the correct diagnosis.

Acknowledgement

Thanks to Drs Charles Knight and Deven Patel for use of several of the heart sounds in this module

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Suggested reading

Greenwood J, Kurian J. Valvular heart disease, Chapter 11 in; Purcell HJ, Kalra PR (eds). Specialist training in cardiology. Elsevier Mosby. Edinburgh. 2005.

Lancellotti P, Marwick T, Pierard LA. How to manage ischaemic mitral regurgitation. Heart 2008;94:1497–502. http://dx.doi.org/10.1136/hrt.2007.134833

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Bridgewater B, Kinsman R, Walton P, Keogh B. Demonstrating quality: the sixth National Adult Cardiac Surgery database report. Dendrite Clinical Systems Ltd. 2009. ISBN: 1-903968-23-2.

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