The natural history and course of hypertension with papilledema (malignant hypertension).

Abstract Material for this study consisted of the 104 cases of malignant hypertension seen at the University of California Hospital from 1936 through 1949. Follow-up information was obtained on all patients. The general features which distinguished the group were the following: 1. 1. The average age was 42 years. 2. 2. The ratio of men to women was 3:2. 3. 3. A family history of hypertension was found in half the patients. 4. 4. Knowledge of pre-existing hypertension was present in 71 per cent of the group. 5. 5. Significant past illnesses (scarlet fever or frequent sore throats, glomerulonephritis, pyelonephritis, and toxemia of pregnancy) had occurred in 56 per cent of the patients. An effort was made to determine the actual duration of the syndrome of malignant hypertension by estimating the date of onset from the patient's history. Impairment of vision was considered to have marked the onset in seventy-eight cases, gross hematuria in five and severe headaches alone in six. The average estimated duration of the malignant phase was 8.7 months, with a range of two weeks to six and one-half years. To check the reliability of these estimations, we also determined the average survival after the discovery of papilledema, which was 8.4 months, with a range of one day to seven years. In three instances, it was believed that the remission in the course of the disease was spontaneous. Because of the importance of early treatment in patients with malignant hypertension, we tried to determine some sort of predictability of onset. In about one-fourth of the patients the onset was acute, in one-third the symptoms were of less than six months' duration, and in the remainder the complaints were long standing. The type of symptoms was similar in all three groups—headaches, congestive failure, gastrointestinal complaints, and the “fatigue complex.” The suddenness and severity of onset appeared to be the distinguishing feature—the more sudden and severe the onset, the more likely a rapid progression into the malignant phase. The significance of retinal changes in hypertension was reviewed. Subjective and objective changes in our patients were analyzed. Ninety per cent of our subjects complained of impairment of vision during the course of their illness. Papilledema, vascular changes and exudates were present in all at some time during the course; hemorrhages occurred in all but eight patients. Loss of vision and appearance of Keith-Wagener IV retinitis were closely correlated. Only three of fifteen patients who showed regression of Keith-Wagener IV retinitis did not receive specific treatment. The effect of malignant hypertension on the heart was also reviewed. Three-fourths of our patients had symptoms of cardiac failure, while half had signs confirming its presence. Angina occurred in one-fifth of the group. Seventy-five perce cent of the determinations of cardiac size by roentgenogram showed enlargement, while over 90 per cent of the electrocardiograms were abnormal. Seventy per cent of these electrocardiograms showed a pattern due to left ventricular hypertrophy. All patients who were examined at autopsy had enlarged hearts, the average weight being 527 grams. The heart is almost universally affected in the course of malignant hypertension. The blood pressure levels in our group were as follows: the highest readings ranged from 300−160 180−100 mm. Hg ; the lowest readings from 265−90 150−60 mm. Hg . Changes in the central nervous system in malignant hypertension were also thoroughly studied, both as reported in the literature and as observed in our own group. Headaches were a complaint of 85 per cent of our patients, but neither in characteristics nor timing could they be considered definitive of malignant hypertension. Cerebrovascular disturbances were manifested in forty-four patients, of whom twenty-five had “strokes.” Convulsions occurred in eighteen patients, two-thirds of whom were dying in uremia. Lumbar punctures showed elevation of cerebrospinal fluid pressure, ranging as high as 500 mm. of water in 62 per cent of our patients. The therapeutic effectiveness of repeated spinal taps was estimated as beneficial in 22 per cent of our subjects, ineffective in 66 per cent, and harmful in 12 per cent. The spinal fluid protein content was increased in 69 per cent of the patients, in ten of whom the spinal fluid pressure was normal. Seventy-five per cent of our group showed some evidence of central nervous system damage. The kidney is the organ most seriously involved in malignant hypertension. Eighty-four per cent of our patients had symptoms relating to the kidney, and all patients had some abnormality demonstrable by one or more laboratory tests. The significance of the symptoms has been analyzed. Results of laboratory studies have been summarized and correlated with rate of survival and pathologic changes observed at autopsy. The impression that nephrosclerosis is the lesion most apt to occur when good renal function is retained in the presence of hypertension and Keith-Wagener IV retinitis was amply verified. In our series of patients the basic renal lesions found at post-mortem examination were nephrosclerosis in fourteen cases, glomerulonephritis in seven, and pyelonephritis in twelve. A bleeding tendency developed in 57 per cent of our patients. Uremia was twice as common in this group as in the remaining patients. A moderate or severe anemia was present in half the group at the time when papilledema was discovered. When the hemoglobin was normal at the time malignant hypertension was diagnosed, nephrosclerosis was the most likely lesion. Reports on the various forms of treatment applicable in malignant hypertension were mentioned, and the experience with their use in our group was presented briefly. An increased survival time may be expected, provided vigorous therapy is supplied to hypertensive patients with papilledema (a) before renal function is impaired, and (b) before irreversible damage is done to cerebral vessels or to the heart.