Twenty-year-old woman with pulmonary hypertension, syncope, and severe headache

Abstract

A 20-year-old woman sought medical care due to headache and vomiting following syncope (9/22/95).The patient was born from an uneventful normal delivery at the end of a full-term pregnancy. Up to the age of 1 year and 6 months, the patient had fatigue when being fed. At the age of 7 years, she began to have fatigue on strenuous exertion and palpitations during tense situations. At the age of 8 years, she had 2 episodes of cyanosis and dyspnea on exertion accompanied by the sensation of imminent loss of consciousness. She also complained of arthralgia in her knees and ankles, and pain in her thoracic and lumbar spine. At the age of 9 years, the patient was referred to our hospital (11/26/84).The physical examination at that time (11/26/84) showed an eupneic and acyanotic child, with a regular and symmetric pulse. The heart rate was 72bpm and blood pressure was 120/90mmHg. The lung examination was normal. The heart examination showed an increased intensity of the second cardiac sound, which was palpable in the pulmonary area, where a systolic murmur could also be heard. The liver was palpated in the right costal margin, and no edema of the lower limbs was observed.The electrocardiogram (11/23/84) showed sinus rhythm, a heart rate of 79bpm, QRS axis of +110° forward, and right ventricular hypertrophy ( fig. 1). The chest X-ray showed enlargement of the right ventricle and bulging of the pulmonary trunk. Laboratory tests are shown in table I. The search for lupus erythematosus cells, antinuclear factor, and rheumatoid factor was negative.The echocardiogram (10/3/85) disclosed an aneurysm