Coarctation of the Aorta, Double Mitral a-v Orifice, and Leaking Cerebral Aneurysm

Abstract

The purpose of this paper is to record and discuss the. case of a boy with well-marked coarctation of the aorta, who made a complete recovery from subarachnoid hmmorrhage due to a leak from an intracranial aneurysm, only to die from rupture of a dissecting aortic aneurysm. A double mitral auriculo-ventricular orifice found at autopsy in this case is an extremely rare cardiac anomaly, only ten previous cases having been recorded (Abbott, 1927). In the present case the occasional diastolic or presystolic mitral murmur may have been the clinical counterpart of this anomaly. We can find only two previous cases in which recovery from a subarachnoid Ihemorrhage in association with coarctation has been recorded-one in a girl, aged 12, reported by Lichtenberg and Gallagher (1933), and the other in a woman of 25, reported by Baker and Sheldon (1936)-and in neither case was the cerebrospinal fluid available for examination. CLINICAL FEATURESThe patient, R. W., was found to have abnormal heart sounds at the age of five years during a routine examination at a school clinic. He remained under observation, first of Dr. Carey Coombs and later of Professor Bruce Perry, until the age of 14, when he left school. During this period health and growth were normal and there were no relevant subjective symptoms. The cardiac lesion caused no apparent disability except that sometimes after exercise he became cyanosed. Its nature was not firmly established for some years. A loud apical systolic murmur was present constantly and it was noted that there was also from time to time another apical murmur which was mid-diastolic or presystolic in character; because of the latter murmur the possibility of a rheumatic origin was considered. From 14 to 17 years of age he worked in a factory, where his work included vigorous physical exertion, such as the moving of heavy packing cases: he had infrequent attacks of headache and vomiting. Later he worked as an A.R.P. messenger and he rendered good service during the air raids on Bristol. At the age of 17 years, on July 10, 1941 he had a sudden fainting attack, details of which are not available. On July 15, he'had a severe attack of vomiting, lasting a few hours and followed, on the evening of the next day, by a sudden violent frontal headache' attended by neck rigidity; this persisted until his' admission to hospital on July 17, with a tentative diagnosis of acute meningitis. On examination in hospital the patient, a strongly built youth, had slow slurred speech and slow cerebration. Temperature and pulse and respiratory rates were normal. There was marked neck rigidity and Kernig's sign was positive on both sides, but there was no head retraction, and the abdominal and tendon reflexes were normal, and the plantar reflex on both sides was flexor. There were no signs of cranial nerve lesions, and the motor and sensory systems appeared normal. The cerebrospinal fluid was heavily blood-stained and under slightly increased pressure. 60 c.c. were withdrawn; three tubes were used to collect the fluid a...