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Article*
"Development of the European Network in Orphan Cardiovascular Diseases"
„Rozszerzenie Europejskiej Sieci Współpracy ds Sierocych Chorób Kardiologicznych”
Title: Diagnosis and treatment of inborn
valvular aortic stenosis
RCD code: IV-6.0
Author: Grzegorz Kopeć
Affiliation: Department of Cardiac and Vascular Diseases and the Centre for Rare
Cardiovascular
Date: 2013.12.16
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
[* The article should be written in English]
Introduction:
Left ventricular outflow tract can be stenosed at the level of valves, subvalvular or
supravalvular. Valvular obstruction is the most common and usually is a consequence of
bicuspid aortic valve. Less than 5% of congenitally malformed aortic valves are unicuspid,
tricuspid, or quadricuspid. The prevalence of bicuspid aortic valve in population is estimated
at 1-2% and bicuspid valve i s the most common congenital cardiac anomaly.
Bicuspid aortic valve is often associated with abnormalities in the structure of aortic wall
which can lead to dilatation, aortic aneurysm and rupture or dissection. In 6% of patients
bicuspid aortic valve is accompanied by aortic coarctation. The other abnormalities associated
with bicuspid aortic valve are subvalvular stenosis, parachute mitral valve, arial and
ventricular septal defect, patent dusctus arteriosus, bicuspid pulmonary valve, Ebstein
anomaly, and hypoplastic left heart syndrome.
Classification
Based on the severity, aortic stenosis is classified as:
- mild is characterized by maximal velocity through aortic valve 2.0-2.9 m/s, mean gradient
through the aortic valve <30 mmHg, aortic valve area >1.5 cm2, aortic valve area index >1.0
cm2/m2,
- moderate is characterized by maximal velocity through aortic valve 3.0-3.9 m/s, mean
gradient through the aortic valve 30-49 mmHg, aortic valve area >1.0 -1.5 cm2, aortic valve
area index 0.6-0.9 cm2/m2,
- severe is characterized by maximal velocity through aortic valve ≥4.0 m/s, mean gradient
through the aortic valve ≥50 mmHg, aortic valve area <1.0 cm2, aortic valve area index <0.6
cm2/m2,
Symptoms
The occurence of symptoms depends on patient age, severity of stenosis and cardiovascular
risk factors. Appearance of symptoms such as stenocardia, dyspnoea or syncope) is associated
with poor prognosis.
Typical sing is a systolic murmur over aortic valve radiating to the neck.
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
Diagnostic work-up
ECG usually shows left ventricular hypertrophy. Echocardiography is a gold standard to
diagnose aortic stenosis and to assess its severity. Usually the gradient through aortic valve,
aortic valve area and aortic valve area indexed for body surface area are measured. In patients
with severe stenosis and without symptoms exercise stress test is recommended to confirm the
lack of symptoms. Echocardiography with low dose dobutamine is recommended in patients
with aortic stenosis and low ejection fraction of the left ventricle.
Treatment
Treatment of aortic stenosis must be interventional as no drugs have been shown to slow the
progression of the disease. Usually surgical replacement of the aortic valve is recommended
but baloon valvuloplasty can be considered in youn patients without aortic valve
calcifications. Patients with severe aortic stenosis and symptoms such as dyspnoea,
stenocardia, syncope should have their valve replaced. This indication also applies to patients
who do not declare symptoms but they appear during exercise stress test. The valve should
also be replaced in patients decreased left ventricular ejection fraction irrespective of
symptoms or the patient is undergoing operation on ascending aorta, another valve or
coronary artery bypass grafting. Patients with severe aortic stenosis who are asymptomatic
but have a significant drop of blood pressure during exercise stress test or have fast
progression of the disease (≥0.3 m/s/year) also deserve consideration of aortic valve
replacement. Aortic valve replacement should also be considered in patients with moderate
aortic stenosis who undergo coronary artery bypass grafting or operation on aorta and in
patients with severe aortic stenosis and low gradient (<40 mmHg) and left ventricular
dysfunction with preserved contraction reserve.
References
1. Roberts WC, Ko JM. Frequency by decades of unicuspid, bicuspid, and tricuspid aortic
valves in adults having isolated aortic valve replacement for aortic stenosis, with or without
associated aortic regurgitation. Circulation. 2005 Feb 22;111(7):920-5
2. Baumgartner H, Bonhoeffer P, De Groot NM, de Haan F, Deanfield JE, Galie N, Gatzoulis
MA, Gohlke-Baerwolf C, Kaemmerer H, Kilner P, Meijboom F, Mulder BJ, Oechslin E,
Oliver JM, Serraf A, Szatmari A, Thaulow E, Vouhe PR, Walma E; Task Force on the
Management of Grown-up Congenital Heart Disease of the European Society of Cardiology
(ESC); Association for European Paediatric Cardiology (AEPC); ESC Committee for Practice
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
Guidelines (CPG). ESC Guidelines for the management of grown-up congenital heart disease
(new version 2010). Eur Heart J. 2010 Dec;31(23):2915-57.
3. Freeman RV, Otto CM. Spectrum of calcific aortic valve disease: pathogenesis, disease
progression, and treatment strategies. Circulation. 2005 Jun 21;111(24):3316-26.
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Author’s signature**
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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