Primary pulmonary hypertension of unusually long duration.

Abstract

Primary pulmonary hypertension in a male patient began at the age of 6, and he died at 25, after a course of I9 years. No previous report of such a long course has been found.A congenital rather than a thrombotic cause is supported by the onset in a boy, the absence of breathlessness on exertion until his last few years, and the medial hypertrophy of the arterioles, without evidence of organic obstruction.Long intervals, up to 12 years, occurred without symptoms, and there was lack of constancy of the degree of effort required to produce syncope. It is suggested that these features resulted from a large reflex factor, in view of the absence of any great rise of pulmonary artery pressure on catheterization.Primary pulmonary hypertension is not so uncommon as was previously thought, or it is increasing in frequency. Wood (I956) recorded an incidence of O-I7 per cent among his patients with cardiovascular disease, and Fleming (I960) reported 8 patients in one year in Sydney. This case may be of interest because of its long duration, the onset of symptoms in a boy aged 6, the long intervals without symptoms, and inconstancy between the degrees of exertion and attacks of syncope. Case ReportThe patient, a European in Kenya, was 24 when first seen in June 1955. There was no family history of cardiac or other disease. He had one brother who was well. His symptoms began at 6 years when he used to faint for some seconds on exerting himself at games. Attacks persisted for 6 months, after which he was free from them for 12 years. They then recurred about once in two months but not consistently, for sometimes he could carry out physical exertion without attacks. His episodes again diminished in frequency, though occasional attacks continued until 2I, when they became more frequent and then gradually got worse and he had to discontinue playing games. He was more liable to attacks when exerting himself on a full stomach or walking fast uphill without 'getting previously warmed up'. He never passed urine or bit his tongue during attacks, though he sometimes bruised or cut his head and once fell and broke a tooth. His unconsciousness lasted up to a minute.He sometimes had minor attacks of tightness, but not pain, in the lower part of his chest, warning signs that he would faint if he continued his exertion. He also experienced slight breathlessness and sweating before attacks and was stated to go a grey colour. If he sat down when these symptoms developed they gradually passed away in three or four minutes. He had no breathlessness on exertion until his last few years, and no swelling of his feet or cough. He was observed to have bradycardia during attacks, and a trained nurse found his pulse rate I30 immediately after one. Apart from his syncope his general health was good. He did not smoke.On examination in I955, aged 24, he looked healthy. No cyanosis, clubbing, or dyspnoea at rest. Slight venous congestion of cervical veins with prominent a waves, but no oedema or enlargement of his liver. Pulse was 8o, regular, and of somew...

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