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Article*
"Development of the European Network in Orphan Cardiovascular Diseases"
„Rozszerzenie Europejskiej Sieci Współpracy ds Sierocych Chorób Kardiologicznych”
Title: Pulmonary hypertension associated with
congenital heart disease - definition and
classification.
RCD code: II-1A.4d
Author: Grzegorz Kopeć
Affiliation: Department of cardiac and vascular diseases at John Paul II Hospital in Krakow.
Date: 30.03.2015
[* The article should be written in English]
John Paul II Hospital in Kraków
Jagiellonian University, Institute of Cardiology
80 Prądnicka Str., 31-202 Kraków;
tel. +48 (12) 614 33 99; 614 34 88; fax. +48 (12) 614 34 88
e-mail: [email protected]
www.crcd.eu
Pulmonary hypertension is a patophysiologic state characterised by elevated
pulmonary artery pressure and defined as a mean pulmonary artery pressure ≥25 mm Hg.
Pulmonary arterial hypertension is not synonymous with pulmonary hypertension, as it
requires elevated pulmonary vascular resistance and normal pulmonary wedge pressure to
be diagnosed.
The prevalence of pulmonary hypertension in patients with congenital heart disease
is estimated at 5% to 10%.
The most recent classification of pulmonary hypertension (Nice 2013) describes 5
groups: (1) pulmonary arterial hypertension; (2) pulmonary hypertension due to left heart
disease; (3) pulmonary hypertension due to lung diseases and hypoxia; (4) chronic
thromboembolic pulmonary hypertension; and (5) pulmonary hypertension with
unclear/multifactorial mechanisms.
The 2013 update to clinical classification of pulmonary hypertension recognizes that
pulmonary hypertension associated with congenital heart disease can represent not only
class I of the WHO classification. For example, congenital left-sided obstructive lesions such
as Shone complex (the combined presence of parachute mitral valve, supravalvular mitral
ring, subaortic stenosis, and coarctation of the aorta) are classified as group 2.
Due to Paul Wood classification pulmonary hypertension can be passive,
hyperkinetic, resistive, and polygenic. Passive is defined by high pulmonary venous pressure
due to for example left ventricular outflow or inflow obstruction, mitral or aortic
regurgitation. Hyperkinetic pulmonary hypertension results from increased pulmonary blood
John Paul II Hospital in Kraków
Jagiellonian University, Institute of Cardiology
80 Prądnicka Str., 31-202 Kraków;
tel. +48 (12) 614 33 99; 614 34 88; fax. +48 (12) 614 34 88
e-mail: [email protected]
www.crcd.eu
flow due to large left to right shunt with normal pulmonary vascular resistance, severe
pulmonary regurgitation, cardiac cirrhosis. Resistive Obstructive: pulmonary
artery embolism,
thrombosis or stenosis
Obstructive or vasoconstrictive pulmonary hypertension can be a consequence of pulmonary
embolism or in situ thrombosis, branch pulmonary artery stenosis, reduction of pulmonary
vascular capacity, pulmonary atresia with reduced distal pulmonary vascular capacity,
functional vasoconstrictive reaction, hypoxic/hypercarbic vasoconstriction, kyphoscoliosis.
Polygenic pulmonary hypertension is a consequence of at least two of the abovementioned
types of pulmonary hypertension. The example is left ventricular inflow obstruction with
reactive pulmonary vasoconstriction and remodeling, large left to right shunt and early
pulmonary vascular remodeling with mildly elevated PVR. Two categories are particularly
relevant to pulmonary hypertension associated with congenital heart disease: hyperkinetic
pulmonary hypertension and polygenic pulmonary hypertension.
References:
1. Wood P. Pulmonary hypertension with special reference to the vasoconstrictive factor. Br
Heart J. 1958;20:557–570
2. Lowe BS, Therrien J, Ionescu-Ittu R, Pilote L, Martucci G, Marelli AJ. Diagnosis of
pulmonary hypertension in the congenital heart disease adult population impact on
outcomes. J Am Coll Cardiol. 2011;58:538–546.
John Paul II Hospital in Kraków
Jagiellonian University, Institute of Cardiology
80 Prądnicka Str., 31-202 Kraków;
tel. +48 (12) 614 33 99; 614 34 88; fax. +48 (12) 614 34 88
e-mail: [email protected]
www.crcd.eu
3. Engelfriet PM, Duffels MG, Möller T, Boersma E, Tijssen JG, Thaulow E, Gatzoulis MA,
Mulder BJ. Pulmonary arterial hypertension in adults born with a heart septal defect: the
Euro Heart Survey on adult congenital heart disease. Heart. 2007;93:682–687.
4. Simonneau G, Gatzoulis MA, Adatia I, Celermajer D, Denton C, Ghofrani A, Gomez Sanchez
MA, Krishna Kumar R, Landzberg M, Machado RF, Olschewski H, Robbins IM, Souza R.
Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25
suppl):D34–D41.
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John Paul II Hospital in Kraków
Jagiellonian University, Institute of Cardiology
80 Prądnicka Str., 31-202 Kraków;
tel. +48 (12) 614 33 99; 614 34 88; fax. +48 (12) 614 34 88
e-mail: [email protected]
www.crcd.eu