Primary Pulmonary Hypertension

Amber Rashid, Stuart Lehrman, Patricia Romano, William Frishman, Jay B. Dobkin, Joseph Reichel

Research output: Contribution to journalArticle

8 Citations (Scopus)

Abstract

Primary pulmonary hypertension (PPH) is a condition characterized by sustained elevation of pulmonary artery pressure (PAP) without demonstrable cause. The most common symptom at presentation is dyspnea. Other complaints include fatigue, chest pain, syncope, leg edema, and palpitations. Right heart catheterization is diagnostic, showing a mean PAP >25 mmHg at rest and >30 mmHg during exercise, with a normal pulmonary capillary wedge pressure. In the National Institutes of Health-PPH registry, the median survival period was 2.8 years. Treatment is aimed at lowering PAP, increasing cardiac output, and decreasing in situ thrombosis. Vasodilators have been used with some success in the treatment of PPH. They include prostacyclin, calcium-channel blockers, nitric oxide and adenosine. Anticoagulation has also been advised for the prevention of deep vein thrombosis, pulmonary embolism, and in situ thromboses of the lungs. New drug treatments under investigation include L-arginine, plasma endothelin-l, and bosentan. Use of oxygen, digoxin, and diuretics for symptomatic relief have also been recommended. Patients with severe PPH refractory to medical management should be considered for surgery.

Original languageEnglish (US)
Pages (from-to)422-430
Number of pages9
JournalHeart Disease
Volume2
Issue number6
StatePublished - Nov 2000

Fingerprint

Pulmonary Artery
Pressure
Thrombosis
Pulmonary Wedge Pressure
Digoxin
Endothelins
National Institutes of Health (U.S.)
Calcium Channel Blockers
Syncope
Epoprostenol
Cardiac Catheterization
Chest Pain
Vasodilator Agents
Pulmonary Embolism
Diuretics
Venous Thrombosis
Cardiac Output
Dyspnea
Adenosine
Fatigue

Keywords

  • Adenosine
  • Calcium-channel blocker
  • Prostacyclin
  • Pulmonary hypertension

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Rashid, A., Lehrman, S., Romano, P., Frishman, W., Dobkin, J. B., & Reichel, J. (2000). Primary Pulmonary Hypertension. Heart Disease, 2(6), 422-430.

Primary Pulmonary Hypertension. / Rashid, Amber; Lehrman, Stuart; Romano, Patricia; Frishman, William; Dobkin, Jay B.; Reichel, Joseph.

In: Heart Disease, Vol. 2, No. 6, 11.2000, p. 422-430.

Research output: Contribution to journalArticle

Rashid, A, Lehrman, S, Romano, P, Frishman, W, Dobkin, JB & Reichel, J 2000, 'Primary Pulmonary Hypertension', Heart Disease, vol. 2, no. 6, pp. 422-430.
Rashid A, Lehrman S, Romano P, Frishman W, Dobkin JB, Reichel J. Primary Pulmonary Hypertension. Heart Disease. 2000 Nov;2(6):422-430.
Rashid, Amber ; Lehrman, Stuart ; Romano, Patricia ; Frishman, William ; Dobkin, Jay B. ; Reichel, Joseph. / Primary Pulmonary Hypertension. In: Heart Disease. 2000 ; Vol. 2, No. 6. pp. 422-430.
@article{7512c03f05a043bf9cfc59d4a5236ef0,
title = "Primary Pulmonary Hypertension",
abstract = "Primary pulmonary hypertension (PPH) is a condition characterized by sustained elevation of pulmonary artery pressure (PAP) without demonstrable cause. The most common symptom at presentation is dyspnea. Other complaints include fatigue, chest pain, syncope, leg edema, and palpitations. Right heart catheterization is diagnostic, showing a mean PAP >25 mmHg at rest and >30 mmHg during exercise, with a normal pulmonary capillary wedge pressure. In the National Institutes of Health-PPH registry, the median survival period was 2.8 years. Treatment is aimed at lowering PAP, increasing cardiac output, and decreasing in situ thrombosis. Vasodilators have been used with some success in the treatment of PPH. They include prostacyclin, calcium-channel blockers, nitric oxide and adenosine. Anticoagulation has also been advised for the prevention of deep vein thrombosis, pulmonary embolism, and in situ thromboses of the lungs. New drug treatments under investigation include L-arginine, plasma endothelin-l, and bosentan. Use of oxygen, digoxin, and diuretics for symptomatic relief have also been recommended. Patients with severe PPH refractory to medical management should be considered for surgery.",
keywords = "Adenosine, Calcium-channel blocker, Prostacyclin, Pulmonary hypertension",
author = "Amber Rashid and Stuart Lehrman and Patricia Romano and William Frishman and Dobkin, {Jay B.} and Joseph Reichel",
year = "2000",
month = "11",
language = "English (US)",
volume = "2",
pages = "422--430",
journal = "Heart Disease",
issn = "1521-737X",
publisher = "Lippincott Williams and Wilkins",
number = "6",

}

TY - JOUR

T1 - Primary Pulmonary Hypertension

AU - Rashid, Amber

AU - Lehrman, Stuart

AU - Romano, Patricia

AU - Frishman, William

AU - Dobkin, Jay B.

AU - Reichel, Joseph

PY - 2000/11

Y1 - 2000/11

N2 - Primary pulmonary hypertension (PPH) is a condition characterized by sustained elevation of pulmonary artery pressure (PAP) without demonstrable cause. The most common symptom at presentation is dyspnea. Other complaints include fatigue, chest pain, syncope, leg edema, and palpitations. Right heart catheterization is diagnostic, showing a mean PAP >25 mmHg at rest and >30 mmHg during exercise, with a normal pulmonary capillary wedge pressure. In the National Institutes of Health-PPH registry, the median survival period was 2.8 years. Treatment is aimed at lowering PAP, increasing cardiac output, and decreasing in situ thrombosis. Vasodilators have been used with some success in the treatment of PPH. They include prostacyclin, calcium-channel blockers, nitric oxide and adenosine. Anticoagulation has also been advised for the prevention of deep vein thrombosis, pulmonary embolism, and in situ thromboses of the lungs. New drug treatments under investigation include L-arginine, plasma endothelin-l, and bosentan. Use of oxygen, digoxin, and diuretics for symptomatic relief have also been recommended. Patients with severe PPH refractory to medical management should be considered for surgery.

AB - Primary pulmonary hypertension (PPH) is a condition characterized by sustained elevation of pulmonary artery pressure (PAP) without demonstrable cause. The most common symptom at presentation is dyspnea. Other complaints include fatigue, chest pain, syncope, leg edema, and palpitations. Right heart catheterization is diagnostic, showing a mean PAP >25 mmHg at rest and >30 mmHg during exercise, with a normal pulmonary capillary wedge pressure. In the National Institutes of Health-PPH registry, the median survival period was 2.8 years. Treatment is aimed at lowering PAP, increasing cardiac output, and decreasing in situ thrombosis. Vasodilators have been used with some success in the treatment of PPH. They include prostacyclin, calcium-channel blockers, nitric oxide and adenosine. Anticoagulation has also been advised for the prevention of deep vein thrombosis, pulmonary embolism, and in situ thromboses of the lungs. New drug treatments under investigation include L-arginine, plasma endothelin-l, and bosentan. Use of oxygen, digoxin, and diuretics for symptomatic relief have also been recommended. Patients with severe PPH refractory to medical management should be considered for surgery.

KW - Adenosine

KW - Calcium-channel blocker

KW - Prostacyclin

KW - Pulmonary hypertension

UR - http://www.scopus.com/inward/record.url?scp=0034329565&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0034329565&partnerID=8YFLogxK

M3 - Article

C2 - 11728293

AN - SCOPUS:0034329565

VL - 2

SP - 422

EP - 430

JO - Heart Disease

JF - Heart Disease

SN - 1521-737X

IS - 6

ER -