Background
Pathological tricuspid regurgitation is more often secondary than due to a primary valve lesion. Isolated primary tricuspid regurgitation progresses slowly and can be well-tolerated for years. However, the data on the natural history of this heart disease suggest that the prognosis is poor. The timing of surgical treatment of severe isolated tricuspid regurgitation can be difficult. The valve repair is preferable method of surgery.
Case presentation
Presentation A 47-year-old man with severe tricuspid regurgitation and suspicion of Ebstein anomaly was admitted to our Centre in January 2013 for cardiologic evaluation. The heart defect was incidentally diagnosed during routine examination before planned left parotid gland cyst surgery. The patient is active and asymptomatic. Physical examination revealed systolic murmur 3/6 in the Levine scale and a 4-centimeter tumor located anterior to the left ear. Laboratory workup did not show any significant pathology, NT-proBNP was 76 pg/ml. Echocardiography showed enlarged right atrium and right ventricle, severe tricuspid regurgitation, RVSP 32mmHg, paradoxical movement of intraventricular septum and small pericardial effusion, left ventricular ejection fraction was normal. Furthermore transesophageal echocardiography revealed patent foramen ovale. The distance in 6-minutes walking test was 500m without desaturation (pulse oximetry after the test was 97%) and dyspnea estimated at 0 in Borg Scale. But maximum oxygen consumption in spiroergometry was only 16,2ml/kg/min (Bruce modified protocol, 10,2METs, time 12:47min). The study was repeated after three weeks in the same protocol and maximum oxygen consumption was 23,1ml/kg/min, what was better but still low. Cardiac magnetic resonance imaging was performed and confirmed very enlarged right ventricle (61cm2) and right atrium (54cm2) and severe tricuspid regurgitation, reverse flow through the tricuspid valve was 170-55=115ml, but the position of tricuspid valve leaflets, width of pulmonary trunk and thickness of right ventricle wall were normal. The coronary angiography was without significant atherosclerosis in the coronary arteries. The right heart catheterization excluded pulmonary hypertension and revealed low cardiac index – it was only 2l/min/m2 (hemodynamic data: RV pressure 23/2/5mmHg, RA pressure 7/5/3mmHg – unexpectedly low, mPAP 12mmHg, PCWP 4/5/3mmHg, PVR 176ARU, Qp/Qs 1:1). Is the patient a candidate for surgery?
Current guidelines
guidelines (ESC 2012) When it comes to severe isolated tricuspid regurgitation, the surgery is indicated in symptomatic patients without severe right ventricular dysfunction (class I) and should be considered in asymptomatic or mildly symptomatic patients with progressive right ventricular dilatation or deterioration of right ventricular function (class IIa).
References
1. Guidelines on the management of valvular heart disease (version 2012). European Heart Journal (2012) 33, 2451-2496
Expert’s comments:(Written authorization required from each expert)
1. Prof. Alfieri O., M.D.
I am in favour of recommending surgery for the tricuspid valve. Although the patient is asymptomatic, the right atrium and the right ventricle are remarkably enlarged. There is no doubt that the regurgitation is severe. This situation is expected to progress and the right ventricular function will deteriorate. One could wait for initial deterioration of the right ventricle, but in my opinion this is not advisable, particularly if tricuspid repair is possible. In the new VHD guidelines (version 2012), the recommendation for surgery in this case is class II a.
2. Prof. Biederman A. MD, PhD
I think that in this case the surgery is recommended. If valve replacement is necessary, rather biological valve should be used.
3. Sieniawski H. MD, PhD
In my opinion classical surgical tricuspid reconstruction should be performed.
4. Assoc. Prof. Kapelak B. MD, PhD
I agree that annuloplasty with valve repair is a method of choice.
5. Assoc. Prof. Gackowski A. MD, PhD
Despite the fact that the right heart is significantly changed, the patient is asymptomatic. It seems that the heart disease processed slowly and right ventricle is well trained.
6. Prof. Gąsior Z. MD, PhD
The heart in this patient may look like right heart cardiomyopathy.
7. Kopeć G. MD, PhD
There is no doubt that the leaflets look abnormally but the etiology of valve damaged is unknown. It is probably isolated primary tricuspid regurgitation.
Expert’s conclusions:
Tricuspid valve surgery is recommended. Tricuspid reconstruction is preferred.
Authors:
Sarnecka A. MD 1, Kopeć G. MD, PhD1, Leśniak-Sobelga A. MD, PhD1, Prof. Podolec P. MD, PhD1
Experts:
Prof. Alfieri O., M.D., Prof. Biederman A. MD, PhD, Sieniawski H. MD, PhD, Assoc. Prof. Kapelak B. MD, PhD, Assoc. Prof. Gackowski A. MD, PhD, Prof. Gąsior Z. MD, PhD, Kopeć G. MD, PhD
1Department of Cardiac and Vascular Disease in John Paul II Hospital, Institute of Cardiology, Faculty of Medicine, Jagiellonian University, Krakow, Poland.
