Mr Symonds was 31 years old, and when he came to see me his medical history contained so few entries I thought the consultation would be easy. It was something of a disappointment that he began to recount a history of feeling light-headed and occasional headaches.
I asked him about causes of stress, perhaps a little too soon in the consultation. He admitted he was under considerable pressure, but just when I thought we had reached the nub of the problem he asked: ‘But why would the stress make me feel short of breath?’
My thoughts immediately went to panic attacks and over-breathing, but he stopped my explanation to make it quite clear that the shortness of breath was related to exertion.
Further enquires revealed that he had never been active or pursued any sport.
On one occasion while walking he said he nearly passed out and said that sometimes his legs ached so much during a brisk walk that he had to stop and rest, but he never had any chest pain.
I examined him at that point. His lungs were clear but his systolic BP was raised at 178/85mmHg with a pulse rate of 76 per minute. I listened to his heart and thought I could hear a systolic murmur, but he said no one had mentioned this before.
Establishing the cause
My mind was running over a number of differential diagnoses from cardiomyopathy to fibrosing alveolitis, but the patient professed that he felt fine and was not unduly worried.I suggested a chest X-ray.
The report surprised me. There was an enlargement of the left ventricle, an irregularity of the descending aortic contour, a ‘figure 3’ sign and notching of the inferior aspect of the ribs. I sent him to the cardiologist immediately.
Mr Symonds had a postductal coarctation of the thoracic aorta. The figure-3 sign on his X-ray refers to an indentation of the lateral margin of the aorta, with poststenotic dilation and the notching of the inferior ribs is due to collateral blood flow through the intercostal arteries.
He had marked dilation of the internal mammary arteries. The hospital letter also noted poor femoral pulses and a gradient in systolic BP between his arms and legs.
My patient underwent resection of the short coarctation with end-to-end anastomosis with an excellent improvement in his exercise tolerance, but he is likely to face persistence of his hypertension.
Coarctation of the aorta is a narrowing of the aorta at, or distal to, the origin of the left subclavian artery at the insertion of the ductus arteriosus.
It may be preductal or postductal. The preductal form is nearly always diagnosed early in life, but postductal lesions are often incidental lesions found in later life, with a male predominance.
It is often associated with other abnormalities including a bicuspid aortic valve in 85 per cent of cases and Turner syndrome in about 15 per cent.
Symptoms include headache, epistaxis, visual disturbances, exertional dyspnoea, claudication in the lower limbs and stroke. A systolic murmur may be heard over the left praecordium or between the scapulae. The BP may differ in the arms if there is an anomalous origin of the right subclavian artery distal to the coarcted segment, and lower limb pulses are reduced. The perioperative mortality rate is between 3 per cent and 11 per cent.
Dr Barnard is a former GP in Fareham, Hampshire
- Postductal aortic coarctation is often not diagnosed until adulthood and has a male predominance.
- Symptoms include headache, nose bleed and breathing difficulties during exertion.
- Clinical signs can include a systolic murmur over the left praecordium or between the scapulae, hypertension and a BP that differs between the two arms.
- Surgical treatment can improve exercise tolerance but hypertension is likely to remain.