The many advances in cardiologic imaging tend to overshadow the contribution of basic physical signs to clinical medicine. In congenital heart disease, detailed clinical examination can be informative and can have a major effect on clinical decision making. In this report, we describe a new physical sign in an adult with complex congenital heart disease and interruption of the aortic arch.

The male patient was first admitted to Freeman Hospital in 1967 at the age of 3 months with cyanosis and cardiac failure. The complex nature of his congenital heart defect is shown in Figure 1. He responded well to diuretic therapy and remained healthy, except for occasional respiratory infections. In 1987, at the age of 20, he was hospitalized with a history of general lassitude and low-grade pyrexia. There were no peripheral stigmata of bacterial endocarditis. He was moderately cyanotic, and the blood pressure was 95/60 mm Hg in his upper limbs and 95/50 mm Hg in his lower limbs. Over the next 3 days, he developed an increasing pulse pressure with collapsing pulses in both lower limbs and with a small increase in systolic pressure in the upper limbs. The blood pressure in the lower limbs was 100/35 mm Hg and was measured using a large cuff width (20 cm). An early diastolic murmur was audible at the left sternal border. Knowing this underlying anatomy, the development of collapsing pulses in his lower limbs in combination with no significant change in his upper limbs clearly pointed to a diagnosis of acute pulmonary valve regurgitation. Echocardiographic evaluation confirmed a vegetation on the pulmonary valve with pulmonary regurgitation. On 25 June 1987, he had a pulmonary valve replacement from which he has made an excellent recovery. Postoperatively, no clinical difference was found between the upper and lower limb pulses.

View larger version (26K): [in this window] [in a new window]   Figure 1. Complex anatomy of the heart in the patient described. AA = ascending aorta; LA = left atrium; LPA = left pulmonary artery; MPA = main pulmonary artery; RA = right atrium; RPA = right pulmonary artery.

The assessment of the pulse character is an important part of clinical examination. Textbooks generally recommend using either the brachial or the carotid pulse to assess the arterial waveform and make no reference to the character of lower-limb pulses. This recommendation is entirely appropriate when dealing with acquired heart disease, but in congenital heart disease, the presence of interruption of the aortic arch with arterial duct supply to the descending aorta creates the potential for differing upper- and lower-limb pulses. When both great vessels originate from the same ventricle and the arterial duct is not restrictive, no clinical difference is found between upper- and lower-limb pulses. The development of a collapsing upper-limb pulse in a patient with normal cardiac connections often suggests aortic regurgitation. In the patient described, however, with interruption of the aortic arch and pulmonary artery supply to the lower limbs, the development of collapsing lower-limb pulses with "normal" upper-limb pulses is virtually diagnostic of pulmonary valve regurgitation. Given the clinical setting, this finding strongly pointed to a diagnosis of pulmonary valve endocarditis.