[Clinico-hemodynamic study and treatment of 44 patients with primary pulmonary hypertension].
BACKGROUND
Primary pulmonary hypertension is a poorly understood disease with a difficult treatment.


METHODS
Retrospective study of a series of 44 patients suffering from pulmonary hypertension who were studied in our center between 1992 and 2000.


RESULTS
At diagnosis, 6 (13%) patients were classified as having NYHA functional class I, 11 (25%) had class II, 25 (57%) had class III, and 2 had class IV. Mean pulmonary artery systolic pressure by echo-doppler was 92 (range: 43-154) mmHg. Basal right catheterization showed a mean (SD) pulmonary artery pressure of 58 (18) mmHg, total basal pulmonary resistances of 1679 (1,071) din/cm2 and cardiac index of 2.2 (1) 1/minute/m2. Five patients improved with anticoagulation and calcium channel blockers therapy. Since 1998, 11 patients had been treated with continuous endovenous epoprostenol, yet only 3 (27%) had significant clinical improvement. Survival at 5 years after diagnosis was 56%. At the end of study, 7 (70%) out of 10 patients who underwent pulmonary transplantation were alive (mean: 34, range: 3-62 months).


CONCLUSIONS
Pulmonary hypertension is a disease with a poor prognosis. However, treatment with prostaglandins and pulmonary transplantation may lead to encouraging results.
Roman
Antonio
A
Servicios de Neumología, Hospital Universitari Vall d'Hebron, Barcelona, Spain. aroman@hg.vhebron.es
Rodés-Cabau
Josep
J
Lara
Beatriz
B
Bravo
Carles
C
Monforte
Víctor
V
Pallissa
Esther
E
Domingo
Enrique
E
Morell
Ferran
F
D000293:Adolescent; D000328:Adult; D000368:Aged; D000925:Anticoagulants / Q000627:therapeutic use; D000959:Antihypertensive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D011464:Epoprostenol / Q000008:administration & dosage / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality / Q000503:physiopathology; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D010975:Platelet Aggregation Inhibitors / Q000008:administration & dosage / Q000627:therapeutic use; D011379:Prognosis; D012189:Retrospective Studies; D013997:Time Factors
D000925:Anticoagulants; D000959:Antihypertensive Agents; D002121:Calcium Channel Blockers; D010975:Platelet Aggregation Inhibitors; D011464:Epoprostenol
Estudio clínico-hemodinámico y tratamiento de 44 pacientes con hipertensión pulmonar primaria.