A 45-year-old woman working as a supermarket cashier comes to the outpatient clinic with the diagnosis of mitral stenosis. She tells a history of rheumatic fever during childhood but remained asymptomatic until the age of 38, when the diagnosis of rheumatic valve disease was established after a first episode of atrial fibrillation (AF). Electrical cardioversion was attempted but AF ultimately relapsed and became permanent the year after. With effective rate control, the patient resumed to an asymptomatic status and has been treated with warfarin and atenolol. Currently, she is in NYHA class I. On physical examination, an accentuated first heart sound with a low-pitched rumbling diastolic murmur is heard at the apex, with no other relevant abnormalities. ECG demonstrates atrial fibrillation with 65 bpm, otherwise unremarkable. On chest X-ray, the cardiac silhouette shows signs of left atrial enlargement and redistribution of pulmonary vascular flow towards the upper lung fields (Fig.1).

Question 1

Which of the treatment options would you consider adequate for an appropriate management of this patient?


A. Recommend rheumatic fever prophylaxis B. Recommend infective endocarditis prophylaxis C. Add a diuretic to her current medication D. Perform a cardioversion to restore sinus rhythm E. None of the previous

The patient underwent an echocardiographic evaluation that revealed pure moderate mitral stenosis (orifice valve area of 1.3 cm2 by 2-D planimetry and 1.2 cm2 by the PHT method, with a mean gradient of 9 mmHg) - (video1 and figure2). The aortic and tricuspid valves were not affected. The left atrium was significantly enlarged (end-systolic biplane indexed volume of 110 ml/m2) and pulmonary artery systolic pressure was 33 mmHg. Laboratory results were normal except for a NT-pro BNP value of 342 pg/ml (reference value <125 pg/ml).

Question 2

Which of the strategies/procedures do you judge reasonable at this time?


A. Transoesophageal echocardiography B. Assess exercise capacity and haemodynamic response to exercise C. Valvular intervention and additional atrial fibrillation ablation D. Clinical follow-up until symptoms develop E. Clinical follow-up until the mitral valve area is <1.0 cm2 or NT-proBNP significantly increases

Under medical therapy, the patient underwent a symptom-limited graded bicycle exercise echo, in a semi-supine position on a tilting table. Two-dimensional and Doppler measurements were obtained at baseline and along the exercise, and digitally stored. After an initial 2 min workload at 25W, the intensity was increased to 50W, under ECG and BP monitoring. The test was interrupted 3 minutes after the beginning (50W), because of severe dyspnoea. Heart rate rose from 65 to 115 bpm. At peak exercise, the mean mitral gradient increased to 28 mmHg and systolic pulmonary artery pressure to 55 mm Hg (Fig.3).

Question 3

Regarding this test, which of the sentences is appropriate?


A. A dobutamine stress echo test would have been preferable to assess exercise tolerance B. The test was inconclusive because heart rate didn't reached 85% of maximal predicted for the patient’s age C. Cut-off values for interpreting the results of the test remain arbitrary and consensus driven D. Post-exercise acquisition of echo Doppler parameters would have been enough E. Left ventricle ejection fraction measured at peak exercise is a valuable parameter for decision-making

Given the results of the standard transthoracic and exercise Doppler echo exams, the patient was conditionally considered a candidate to percutaneous balloon valvulotomy (PBV).

Question 4

Before this procedure, which of the following investigations would you recommend?


A. RT-3D Echo to confirm the severity of mitral stenosis B. Cardiac catheterization to confirm noninvasive haemodynamic measurements and exclude coronary artery disease C. Cardiac magnetic resonance imaging to confirm LA volume and to measure the tricuspid annular size, as it is the gold standard to assess cardiac chamber dimensions D. Transoesophageal echocardiography to confirm the Wilkins score and exclude contraindications to PBV E. Cardiac computed tomography to exclude left atrial thrombus and associated coronary artery disease that might deserve concomitant surgical revascularization

Despite initial reluctance, the patient agreed to undergo PBV after transoesophageal echocardiography. The procedure was successful and without complications. The mitral valve orifice area increased to 2.1 cm2 by 2D-planimetry (Video 2). Reassessed one month after the procedure in the outpatient clinic, the patient confessed that only at that time she realized what it was to be really asymptomatic. The NT-proBNP decreased to 241 pg/ml.

Question 5

Which follow-up strategy would you adopt for this patient?


A. Yearly follow-up with both clinical and transthoracic echo examinations. B. Yearly follow-up with clinical examination, chest X-ray, and ECG; echo reserved for changes in clinical status or every 3-5 years. C. Clinical and transthoracic echo examinations every 6 months during the first two years for detection of early restenosis D. Yearly follow-up with clinical and transesophageal echocardiography examinations, since the transthoracic approach is no longer reliable after PBV E. In the case of restenosis, referral for surgical prosthetic valve implantation

References

1. Michael A. Gerber, Robert S. Baltimore, Charles B. Eaton et al. Prevention of Rheumatic Fever and Diagnosis and Treatment of Acute Streptococcal Pharyngitis. Circulation (2009); 119: 1541-1551.

2. Gilbert Habib, Bruno Hoen, Pilar Tornos et al, for the Task Force on the Prevention, Diagnosis, and Treatment of Infective Endocarditis of the European Society of Cardiology (ESC). Guidelines on the prevention, diagnosis, and treatment of infective endocarditis (new version 2009). Eur Heart Journal (2009); 30, 2369–2413.

3. A. Vahanian, B. Iung, L. Pierard, R. Dion, and J. Pepper. Chapter 21 Valvular Heart Disease. ESC Textbook of Cardiovascular Medicine, January 1, 2009.

4. Alec Vahanian, Helmut Baumgartner, Jeroen Bax et al for the Task Force on the Management of Valvular Heart Disease of the European Society of Cardiology. Guidelines on the management of valvular heart disease. Eur Heart Journal (2007); 28: 230–268.

5. Kadriye Orta Kilickesmez, Alev Arat Ozkan, Okay Abaci et al. Serum N-Terminal Brain Natriuretic Peptide Indicates Exercise Induced Augmentation of Pulmonary Artery Pressure in Patients with Mitral Stenosis. Echocardiography (2011);28: 8-14.

6. Brochet E, Détaint D, Fondard O et al. Early hemodynamic changes versus peak values: what is more useful to predict occurrence of dyspnea during stress echocardiography in patients with asymptomatic mitral stenosis? J Am Soc Echocardiogr 2011; 24:392-8.

7. A. John Camm, Paulus Kirchhof, Gregory Y.H. Lip et al. Guidelines for the management of atrial fibrillation. Eur Heart Journal (2010); 31: 2369–2429.

8. Robert O. Bonow, Blase A. Carabello, Kanu Chatterjee et al. 2008 focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines Writing Committee to Develop Guidelines for the Management of Patients With Valvular Heart Disease. Journal of the American College of Cardiology (2008); 52: e1–142.

9. Luc A Piérard, Patrizio Lancellotti. Stress testing in valve disease. Heart (2007); 93: 766–772.

10. Eugenio Picano, Philippe Pibarot, Patrizio Lancellotti et al. The Emerging Role of Exercise Testing and Stress Echocardiography in Valvular Heart Disease. Journal of the American College of Cardiology (2009); 54: 2251–60.

11. Helmut Baumgartner, Judy Hung, Javier Bermejo et al. Echocardiographic assessment of valve stenosis: EAE/ASE recommendations for clinical practice. Eur Journal of Echocardiography (2009) 10; 1–25.

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