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Diagnosis and Treatment of Pulmonary Arterial Hypertension

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PanVascular Medicine

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Abstract

Pulmonary hypertension has become an increasingly identified comorbidity to numerous common disease processes and portends a very poor prognosis. Recent advancements in clinical recognition, innovative imaging modalities, and new classification schemes have shed new light on our understanding of this condition and advanced our treatment options. The principal subtype, pulmonary arterial hypertension, is a rare, progressive, and fatal condition manifest by elevated blood pressure in the pulmonary circulation as a result of increased pulmonary vascular resistance (PVR). Elevated pulmonary blood pressure increases the afterload of the right ventricle creating strain, which can progress to right ventricular failure and death. This condition is manifest by clinical symptoms including dyspnea, fatigue, and chest pain along with signs of elevated pulmonary pressures and right heart dysfunction. The clinical evaluation of pulmonary hypertension includes numerous invasive and noninvasive testing including blood work, imaging modalities, and right heart catheterization. Treatment of pulmonary hypertension includes both supportive care therapeutics and targeted pulmonary vasodilators. While supportive care with diuretics, oxygen, anticoagulation, and inotropic agents remain commonplace, newer agents including prostacyclin analogs, endothelin receptor antagonists, phosphodiesterase type 5 inhibitors, and guanylate cyclase stimulators have clearly shown safety and efficacy in numerous clinical trials. Recent years have produced substantial progress in our management of pulmonary hypertension; still much needs to be done in order to identify novel targets that may have far-reaching impact on overall morbidity and mortality.

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Abbreviations

6MWT:

Six-minute walk testing

ANCA:

Antineutrophil cytoplasmic antibody

BMPR2:

Bone morphogenetic protein receptor type 2

cAMP:

Cyclic adenosine monophosphate

cGMP:

Cyclic guanosine monophosphate

CCB:

Calcium channel blocker

CO:

Cardiac output

COPD:

Chronic obstructive pulmonary disease

CT:

Computed tomography

CTEPH:

Chronic thromboembolic pulmonary hypertension

dAA:

Diameter of ascending aorta

dPA:

Diameter of pulmonary artery

ECG:

Electrocardiogram

ERA:

Endothelin receptor antagonist

ET:

Endothelin

INR:

International normalized ratio

IPAH:

Idiopathic pulmonary arterial hypertension

IPR:

Prostacyclin receptor

IVC:

Inferior vena cava

HIV:

Human immunodeficiency virus

HPAP:

Heritable pulmonary arterial hypertension

LV:

Left ventricle

MRI:

Magnetic resonance imaging

NO:

Nitric oxide

NYHA:

New York Heart Association

PA:

Pulmonary artery

PAP:

Pulmonary arterial pressure

PaO2 :

Partial pressure of arterial oxygen

PASP:

Pulmonary artery systolic pressure

PAWP:

Pulmonary artery wedge pressure

PDE5:

Phosphodiesterase type 5

PH:

Pulmonary hypertension

PAH:

Pulmonary arterial hypertension

PGI2 :

Prostacyclin

PVR:

Pulmonary vascular resistance

RAP:

Right atrial pressure

RHC:

Right heart catheterization

RV:

Right ventricle

RVF:

Right ventricular failure

RVH:

Right ventricular hypertrophy

RVSP:

Right ventricular systolic pressure

SpO2 :

Oxygen saturation

SVR:

Systemic vascular resistance

TAPSE:

Tricuspid annular plane systolic excursion

WHO:

World Health Organization

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Further Readings

  • Galie N, Hoeper MM, Humbert M, Torbicki A, Vachiery JL, Barbera JA, Beghetti M, Corris P, Gaine S, Gibbs JS, Gomez-Sanchez MA, Jondeau G, Klepetko W, Opitz C, Peacock A, Rubin L, Zellweger M, Simonneau G, ESC Committee for Practice Guidelines (CPG) (2009) Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 30(20):2493–2537

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  • Lewis JE, Channick RN (2011) Idiopathic pulmonary arterial hypertension. In: Yuan JX-J, Garcia JGN, Hales CA, Rich S, Archer SL, West JB (eds) Textbook of pulmonary vascular disease. Springer, Boston, pp 979–995

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Authors and Affiliations

  1. Department of Medicine, Division of Pulmonary, Critical Care, Sleep and Allergy Medicine, Center for Cardiovascular Research, University of Illinois at Chicago, 909 South Wolcott Avenue COMRB Rm. 3131 (MC 719), Chicago, IL, 60612, USA

    Dr. Dustin R. Fraidenburg & Jason X. -J. Yuan M.D., Ph.D. (Professor of Medicine and Pharmacology)

  2. Department of Pharmacology, University of Illinois at Chicago, Chicago, IL, 60612, USA

    Jason X. -J. Yuan M.D., Ph.D. (Professor of Medicine and Pharmacology)

  3. Department of Respiratory Medicine, Mater Misericordiae University Hospital, University College Dublin, Eccles Street 7, Dublin, Ireland

    Prof. Sean P. Gaine

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  1. Dr. Dustin R. Fraidenburg
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  2. Jason X. -J. Yuan M.D., Ph.D.
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  3. Prof. Sean P. Gaine
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Correspondence to Jason X. -J. Yuan M.D., Ph.D. .

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  1. Akademisches Lehrkrankenhaus MLU, Bitterfeld-Wolfen, Germany

    Peter Lanzer

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Fraidenburg, D.R., Yuan, J.X.J., Gaine, S.P. (2014). Diagnosis and Treatment of Pulmonary Arterial Hypertension. In: Lanzer, P. (eds) PanVascular Medicine. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-37393-0_155-1

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