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Pulmonary Hypertension

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MGH Cardiology Board Review

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Abstract

The right ventricular-pulmonary vascular unit is a low resistance, high compliance system that is normally capable of accommodating large increases in blood flow with a minimal increment in pressure. The development of elevated pulmonary arterial pressures, either in unselected populations or in individuals with a variety of cardiopulmonary diseases, is increasingly recognized to be associated with a markedly increased risk of mortality. Regardless of the etiology, pulmonary hypertension leads to right ventricular dysfunction that is closely associated with impaired exercise capacity, renal and hepatic dysfunction. This chapter will briefly review the epidemiology and pathophysiology of pulmonary hypertension (PH) in addition to current diagnostic and treatment approaches with particular emphasis on PH arising in the setting of other cardiovascular diseases.

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Abbreviations

6 MW:

6 Minute Walk

APAH:

Associated with Pulmonary Arterial Hypertension

BMP:

Bone Morphogenic Protein

CCB:

Calcium Channel Blockers

COPD:

Chronic Obstruction Lung Disease

CTEPH:

Chronic Thromboembolic Pulmonary Hypertension

DVT:

Deep Venous Throbosis

FPAH:

Familial Pulmonary Arterial Hypertension

HFpEF:

Heart Failure with preserved Ejection Fraction

IPAH:

Idiopathic Pulmonary Arterial Hypertension

JVP:

Jugular Venous Pressure

LFT:

Liver Function Test

LVEF:

Left Ventricular Ejection Fraction

mPAP:

Mean Pulmonary Artery Pressure

NO:

Nitric Oxide

NYHA:

New York Heart Association

PA:

Pulmonary Artery

PAH:

Pulmonary Arterial Hypertension

PAP:

Pulmonary Artery Pressure

PCH:

Pulmonary Capillary Hemangiomatosis

PCWP:

Pulmonary Capillary Wedge Pressure

PDE-5:

Phosphodiesterase-5

PE:

Pulmonary Embolism

PH:

Pulmonary Hypertension

PVOD:

Pulmonary Veno-Occlusive Disease

PVR:

Pulmonary Vascular Resitance

RV:

Right Ventricle

RVEF:

Right Ventricular Ejection Fraction

TAPSE:

Tricuspid Annular Plane Systolic Excursion

TTE:

Transthoracic echocardiogram

WHO:

World Health Organization

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Author information

Authors and Affiliations

  1. Harvard Medical School Cardiology Division, Department of Medicine, Massachusetts General Hospital, Boston, MA, USA

    Jonathan Clarke MD (Clinical and Research Fellow)

  2. Mass General Cardiopulmonary Exercise Laboratory, Cardiology Division, Department of Medicine, Massachusetts General Hospital, Boston, MA, USA

    Gregory D. Lewis MD (Assistant Professor of Medicine)

  3. Harvard Medical School, Boston, USA

    Gregory D. Lewis MD (Director)

Authors
  1. Jonathan Clarke MD
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  2. Gregory D. Lewis MD
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Editor information

Editors and Affiliations

  1. Cardiology Division Yawkey Building 5B, Room 5850, Massachusetts General Hospital, Boston, Massachusetts, USA

    Hanna K. Gaggin

  2. Cardiology Division Yawkey Building, Suite 5984, Massachusetts General Hospital, Boston, Massachusetts, USA

    James L. Januzzi, Jr.

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© 2014 Springer-Verlag London

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Clarke, J., Lewis, G.D., Lewis, G.D. (2014). Pulmonary Hypertension. In: Gaggin, H., Januzzi, Jr., J. (eds) MGH Cardiology Board Review. Springer, London. https://doi.org/10.1007/978-1-4471-4483-0_22

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  • DOI: https://doi.org/10.1007/978-1-4471-4483-0_22

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  • Print ISBN: 978-1-4471-4482-3

  • Online ISBN: 978-1-4471-4483-0

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