History and clinical findings: A 58-year-old man, previously resident in Russia, was known since the age of 18 years to have arterial hypertension of unknown cause in only the right arm. A single syncope was the only previous symptom. On examination the pressure was 230/110 mmHg in the right arm, 150/100 mmHg in the left one. The pulse in the right arm and neck was strong and heaving, that in the left arm and the legs much more weakly palpable.
Investigations: Electrocardiogram and echocardiogram showed left ventricular hypertrophy. The chest radiogram demonstrated rib notching. Digital subtraction angiography revealed aortic arch atresia just distal to the common carotid artery. No other cardiovascular abnormalities were found.
Treatment: The patient declined operative treatment. Cautious antihypertensive drug treatment with Atenolol (25 mg daily) reduced the pressure in the right arm to 180/90 mmHg.
Conclusion: This rare malformation of aortic arch atresia should be considered in the differential diagnosis of asymmetrical arterial hypertension in an adult.