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Conservative Management and Recommendations for Pulmonary Arterial Hypertension Related to Congenital Heart Disease

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Pulmonary Hypertension in Adult Congenital Heart Disease

Part of the book series: Congenital Heart Disease in Adolescents and Adults ((CHDAA))

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Abstract

Pulmonary arterial hypertension (PAH) in congenital heart disease (PAH–CHD) is the result of numerous pathomechanistic pathways affecting the pulmonary vascular bed and leading to a rise in pulmonary vascular resistance (PVR), right heart failure and premature death. Depending on the type of underlying defect or previous repair (timing and method used), patients can present with diverse anatomy and pathophysiology. At the extreme end of the spectrum is Eisenmenger syndrome (ES), which is characterized by pulmonary arterial pressure elevated to near-systemic levels with shunt reversal and cyanosis. It is a multisystem disorder driven by slow progressive chronic hypoxemia and the persistence of a shunt, resulting in coagulation disorders, iron deficiency anaemia, renal dysfunction, hypertrophic osteoarthropathy, paradoxical emboli and heart failure. Differences between various types of PAH–CHD are not purely academic, but significantly impact on presentation, treatment and outcome. This chapter covers the conservative management of PAH–CHD, with a specific focus on Eisenmenger syndrome.

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Abbreviations

ACE:

Angiotensin converting enzyme

ARB:

Angiotensin receptor blocker

ASD:

Atrial septal defect

BNP:

B-type natriuretic protein

CHD:

Congenital heart disease

CI:

Cardiac Index

CO2 :

Carbon dioxide

CPET:

Cardiopulmonary exercise test

CT:

Computed tomography

CTCA:

Computed tomography coronary angiogram

CTPA:

Computed tomography pulmonary angiogram

CVE:

Cerebrovascular events

DCCV:

Direct current cardioversion

ECG:

Electrocardiogram

ERCP:

Endoscopic retrograde cholangiopancreatography

ES:

Eisenmenger syndrome

ET:

Exercise training

ICD:

Implantable cardioverter defibrillator

ID:

Iron deficiency

INR:

International normalized ratio

iPAH:

Idiopathic pulmonary arterial hypertension

LA:

Left atrium

LV:

Left ventricle

MCH:

Mean corpuscular haemoglobin

MCV:

Mean corpuscular volume

PA:

Pulmonary artery

PAH:

Pulmonary arterial hypertension

PH:

Pulmonary hypertension

PRV:

Polycythemia rubra vera

PVR:

Pulmonary vascular resistance

QoL:

Quality of life

RA:

Right atrium

RAAS:

Renin-angiotensin-aldosterone system

RAP:

Right atrial pressure

RV:

Right ventricle

RVAD:

Right ventricular assist device

SVR:

Systemic vascular resistance

TAPSE:

Tricuspid annular plane systolic excursion

TOE:

Transesophageal echocardiogram

VE:

Minute volume

VF:

Ventricular fibrillation

VO2:

Volume of oxygen

VSD:

Ventricular septal defect

VT:

Ventricular tachycardia

WHO FC:

World Health Organization functional class

6MWT:

6 minute walk distance

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Authors and Affiliations

  1. Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK

    Heba Nashat, Carl Harries, Konstantinos Dimopoulos & S. John Wort

  2. Department of Adult Congenital Heart Disease, University Hospital Southampton, Southampton, UK

    Samantha J. Fitzsimmons

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  1. Heba Nashat
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  2. Samantha J. Fitzsimmons
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  4. Konstantinos Dimopoulos
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Correspondence to S. John Wort .

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Editors and Affiliations

  1. Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital and Imperial College, London, United Kingdom

    Konstantinos Dimopoulos

  2. University Hospital, Münster, Germany

    Gerhard-Paul Diller

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Nashat, H., Fitzsimmons, S.J., Harries, C., Dimopoulos, K., Wort, S.J. (2017). Conservative Management and Recommendations for Pulmonary Arterial Hypertension Related to Congenital Heart Disease. In: Dimopoulos, K., Diller, GP. (eds) Pulmonary Hypertension in Adult Congenital Heart Disease. Congenital Heart Disease in Adolescents and Adults. Springer, Cham. https://doi.org/10.1007/978-3-319-46028-4_15

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