(IV-1D.2c) 20-year-old male with asymptomatic aortic stenosis Joanna Łuszczak MD, Lidia Tomkiewicz- Pajak MD, PhD, Maria Olszowska MD, PhD, Prof. Piotr Podolec MD, PhD, Paweł Rubiś MD, PhD, Jakub Podolec MD.

Aortic stenosis (AS) is the most common valvular heart disorder. The prevalence increases with age and is estimated on 2-7% in population over 65 years old. Congenital AS is often associated with bicuspid aortic valve. Deterioration of the left ventricle (LV) systolic function is a predictor of the worse outcome after aortic valve replacement (AVR). Among the asymptomatic patients with preserved LV systolic function the risk of death is relatively low- about 1%, but increases significantly when symptoms occur. Standard treatment of AS is aortic valve replacement which is associated with 1.2% perioperative risk of death in patients younger than 56 years old. Newer method, transcatheter aortic valve implantation (TAVI) seem to be very promising but nowadays is reserved for patients disqualified from AVR due to high operative risk and is contraindicated in bicuspid aortic valve.

Case presentation
We present a case of a 20-year-old man with congenital aortic stenosis. The patient was admitted to our center in June 2012 for further evaluation. He denied symptoms (chest pain, dyspnea, syncope) or decreased exercise capacity. Since 2010 every six month transtoracic echocardiography was performed and the progression of the stenosis as well as the development of left ventricle hypertrophy was observed (table 1.)
The patient was admitted to our hospital for further evaluation and qualification to aortic valve replacement. Physical examination revealed systolic murmur best heard on upper right sternal border, heart rate was regular 100 bpm, blood pressure 110/70 mmHg, vesicular breath sounds. The liver edge was not palpable. The patient didn’t have peripheral edema. The laboratory tests were unremarkable (table 2). The ECG showed sinus rhythm 95 bpm, left ventricle hypertrophy and overload. In the 24-hours ECG monitoring minimal heart rate was 43 bpm at 1.59 AM, maximal heart rate 141 bpm at 1.13 PM and mean hart rate 68 bpm. The echocardiographic examination showed concentric left ventricle (LV) hypertrophy (interventricular septum thickness of 17.5 mm and posterior wall of 15.7 mm), bicuspid AV (three- commissural, fusion of the left- and right coronary leaflets) with the gradient of 94/58 mmHg, aortic valve area (AVA) of 0.92 cm² and AVA indexed to body surface area of 0.48 cm²/m². Left ventricle (LV) systolic function was normal with ejection fraction (EF) of 83% but LV diastolic function was impaired (E/A =0.55, E’lateral= 0.07 m/s). There was a mild aortic, mitral and tricuspide regurgitation. The exercise test was performed and despite lack of symptoms showed ST segment depression in II, III, aVF, V5-V6 up to 1.35mm on reference, that progressed up to 2 mm on peak exercise.
The patient was treated by bisoprolol 5mg. The patient’s medical history was presented on the Meeting of the Center for Rare Cardiovascular Diseases where he was qualified to the surgical treatment.

03.2010 09.2011 04.2012
AS with AVG 44 mmHg, Vmax 3.3 m/s, AR I°, no LV hypertrophy AVG 70/38 mmHg, AVA 1,18 cm2, (HR 105 bpm) IVSd 12mm, PWd 12 mm AVG 92/59 mmHg, AVA 1.1 cm2 (HR 100 bpm), mild/moderate AR, IVSd 15 mm, PWd 14 mm

Table 1. AVG- aortic valve gradient, LV-left ventricle, EF-ejection fraction, AVA-aortic valve area, AR- aortic valve reguritance, Ao- aorta, PWd- posterior wall in diastole, IVSd- intrerventricular septum in diastole

• WBC 7.92 x10³/µl
• RBC 5.02 x10³/µl
• Hb 15.1 g/dl
• HCT 43.2 %
• PLT 228 x10³/µl
• INR 1.04
• Creatynine 93 µmol/l
• e-GFR 89.8
• HsCRP <0.15 mg/l
• ALT 20 U/l
• AST 19 U/l
• Glucose 5.3 mmol/l
• TSH 1.63 IU/ml

Table 2.

Current guidelines
According to the current guidelines patients with symptomatic severe AS, patients with severe AS and impaired LV systolic function (LV ejection fraction<50%), patients with symptoms occurring during exercise test and patients with severe AS undergoing CABG, surgery on ascending aorta or other valve should be scheduled for the AVR. The surgical treatment should be considered in patients with severe AS and high risk when they are suitable for TAVI, in asymptomatic patients when drop of the blood pressure during exercise is observed, with moderate AS when CABG, surgery on ascending aorta or other valve is planned, with low-flow low-gradient AS with preserved LV systolic function- after careful examination, with low-flow low-gradient AS with compromised LV systolic function- after confirmation of flow reserve, finally, in asymptomatic severe AS if surgical risk is low and peak transvalvular velocity is over 5.5 m/s and/or when valve is severly calcified and yearly progression of the velocity ≥ 0.3. AVR may be considered in symptomatic patients with low-flow, low-gradient severe AS and no flow reserve as well as in patients with asymptomatic severe AS with normal systolic LV function when al least one of factors are present: markedly elevated level of the natiuretic peptide, increase of mean pressure gradient more than 20 mmHg during exercise or large LV hypertrophy in patient without hypertension.

1. Vahanian A, Alfieri O, Andreotti F et al. Guidelines on the management of valvular heart disease (version 2012): the Joint Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology (ESC) and the European Association for Cardio-Thoracic Surgery (EACTS). Eur J Cardiothorac Surg. 2012;42:S1-44.
2. The European Association for Cardio-Thoracic Surgery. Fourth EACTS adult cardiac surgical
database report 2010. Henley-on-Thames, UK Dendrite Clinical Systems Ltd; ISBN 9781-9039-682-60.
3. Baumgartner H, Bonhoeffer P, De Groot NM et al. ESC Guidelines for the management of grown-up congenital heart disease (new version2010). Eur Heart J. 2010;31:2915-57.

Expert’s comments:(Written authorization required from each expert)

1. Bogusław Kapelak, MD, PhD
The patient should be qualified to the AVR. If the patient wants to avoid lifelong oral anticoagulotherapy, implantation of the biological aortic valve is possible, but the patient must be informed about the need of the reoperation in the future. The coronary catheterization is not necessary prior to the surgery.

2. Prof. Andrzej Rudziński MD, PhD
The patient should be qualified to AVR.

Expert’s conclusions:
Because of the rapid progression of the aortic stenosis and significant left ventricular hypetrophy development the patient should be qualified to the aortic valve replacement. Implantation of the biological valve is possible but the patient must be informed about the necessity of the reoperation in the future. There is no need of the coronary artery catheterization before AVR.

Joanna Łuszczak MD1, Lidia Tomkiewicz- Pajak MD, PhD1, Maria Olszowska MD, PhD1, Prof. Piotr Podolec MD, PhD1, Paweł Rubiś MD, PhD1, Jakub Podolec MD.1

Bogusław Kapelak MD, PhD2, Prof. Andrzej Rudziński MD, PhD3

1Department of Heart and Vascular Disease, John Paul II Hospital, Kraków, Poland
2Department of Cardiovascular Surgery and Transplantology, John Paul II Hospital, Krakow, Poland.
3Department of Pediatric cardiology, Polish-American Children’s Hospital, Krakow, Poland


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