Pulmonary Vascular Disease

  • Eoin P. Judge
  • Dermot O’Callaghan
  • Sean P. Gaine
Chapter

Abstract

A wide variety of disorders affect the pulmonary vasculature including pulmonary hypertension (PH) and pulmonary embolism (PE). Increased understanding of the pathophysiological basis of PH has led to a reclassification, incorporating multiple etiological factors including heritable forms. Accurate classification of PH subtypes relies on a detailed clinical and diagnostic evaluation, with right heart catheterization a requirement for definitive diagnosis. Recently developed therapies have been shown to provide clinical benefit in selected patients with PH.

Significant progress has also been made in PE. Technological improvements in diagnostics, most notably in computed tomography pulmonary angiography (CTPA), have led to increased and more accurate detection of PE. Algorithms based on risk stratification and clinical probabilities have been refined to assist clinicians in the diagnosis and treatment of this frequently complex condition. Treatment with anticoagulation and thrombolytic therapy has evolved. Outpatient management is now increasingly recognized as a viable option.

This chapter will focus on two of the most widely encountered manifestations of pulmonary vascular disease – PH and PE. In addition to discussing the etiology, epidemiology, diagnosis, and treatment of these diseases, the chapter highlights recently published evidence-based guidelines.

Keywords

Catheter Influenza Immobilization Warfarin Thrombin 

Abbreviations

ABG

Arterial blood gas

ACCP

American College of Chest Physicians

ACE

Aangiotensin-converting-enzyme

AHA

American Heart Association

ANCA

Anti-neutrophil cytoplasmic antibody

BNP

Brain natriuretic peptide

CCB

Calcium channel blocker

CHD

Congenital heart disease

CI

Cardiac index

CO

Cardiac output

COPD

Chronic obstructive pulmonary disease

CT

Computed tomography

CTPA

Computed tomography pulmonary angiography

CTD

Connective tissue disease

CTEPH

Chronic thromboembolic pulmonary hypertension

CUS

Compression ultrasonography

CXR

Chest radiograph

DLco

Decreased diffusion capacity for carbon monoxide

dsDNA

Double-stranded DNA

DVT

Deep vein thrombosis

ELISA

Enzyme-linked immunosorbent assay

ERA

Endothelin receptor antagonist

ESC

European Society of Cardiology

ESR

Erythrocyte sedimentation rate

ET-1

Endothelin-1

HIV

Human immunodeficiency virus

INR

International normalized ratio

IPAH

Idiopathic pulmonary arterial hypertension

IPF

Idiopathic pulmonary fibrosis

i.v.

Intravenous

IVC

Inferior vena cava

LMWH

Low molecular weight heparin

LV

Left ventricular

mPAP

Mean pulmonary arterial pressure

MRA

Magnetic resonance angiography

MRI

Magnetic resonance imaging

NIH

National Institute of Health

NT-proBNP

N-terminal proBNP

PaO2

Arterial oxygen pressure

PAP

Pulmonary arterial pressure

PASP

Pulmonary arterial systolic pressure

PCH

Pulmonary capillary hemangiomatosis

PCWP

Pulmonary capillary wedge pressure

PDE5

Phosphodiesterase type-5

PE

Pulmonary embolism

PEA

Pulmonary endarterectomy

PFO

Patent foramen ovale

PH

Pulmonary hypertension

PVOD

Pulmonary veno-occlusive disease

PVR

Pulmonary vascular resistance

RA

Right atrial

RCT

Randomized control trial

RHC

Right heart catheterization

rt-PA

Recombinant tissue plasminogen activator

RV

Right ventricular

SaO2

Saturation level of oxygen in hemoglobin

s.c.

Subcutaneous

SLE

Systemic lupus erythematosus

SSc

Systemic sclerosis

STEMI

ST-elevation myocardial infarction

TR

Tricuspid regurgitation

UFH

Unfractionated heparin

VKA

Vitamin K antagonist

V/Q

Ventilation/perfusion

VTE

Venous thromboembolism

WHO-FC

World Health Organization functional class

6MWT

Six-minute walk test

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Recommended Reading

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Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  • Eoin P. Judge
    • 1
  • Dermot O’Callaghan
    • 1
  • Sean P. Gaine
    • 1
  1. 1.Department of Respiratory MedicineMater Misericordiae University HospitalDublin 7Ireland

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