Severe pulmonary hypertension with diffuse smooth muscle proliferation of the lungs. Pulmonary tuberous sclerosis?
A 49-year-old man with normal intelligence and no cutaneous lesions had symptoms and signs of cor pulmonale. The chest roentgenogram showed an interstitial pattern. Hemodynamic studies revealed increased pulmonary arterial and wedge pressures. The patient died in shock, and the postmortem findings were a diffuse smooth muscle proliferation in the lungs as the cause of the pulmonary hypertension. We presume that this is a form of pulmonary tuberous sclerosis of which few cases are reported with such severe pulmonary hypertension as shown by our measurements.
Wagener
O E
OE
Instituto de Investigaciones Médicas Alfredo Lanari, Facultad de Medicina, Universidad de Buenos Aires, Argentina.
Roncoroni
A J
AJ
Barcat
J A
JA
D006801:Humans; D006976:Hypertension, Pulmonary / Q000473:pathology*; D008168:Lung / Q000473:pathology*; D008171:Lung Diseases / Q000175:diagnosis / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009130:Muscle, Smooth / Q000473:pathology*; D014402:Tuberous Sclerosis / Q000175:diagnosis / Q000473:pathology*