Abstract
Background
Tricuspid valve (TV) stenosis is a very rare condition and the aetiology is primarily due to rheumatic disease, carcinoid disease, congenital heart disease, endocarditis, and following degeneration of biological valve prosthesis.
Case summary
We present a 45-year-old man with a rare case of symptomatic TV stenosis (TS) in a previously isolated TV repair. A meticulous multimodality diagnostic approach is presented in order to determine the severity of the TS and to evaluate the right ventricular function.
Discussion
This case report presents an integrated multimodality imaging and haemodynamic approach to evaluate and document the suspicion of development of a symptomatic significant stenosis in a previous TV repair. The initial TV repair was done without ring annuloplasty, because only the anterior leaflet was affected and bicuspidalization of the valve made it patent. In addition, minimizing the amount of implanted material was intended to minimize the risk of reinfection. The final treatment was performed as a TV replacement with insertion of a bioprosthesis.
Keywords: Tricuspid valve stenosis, Multimodality approach, Right heart catheterization, 3D transoesophageal echocardiography, Cardiac magnetic resonance imaging, Case report
Learning points
Tricuspid valve (TV) stenosis following TV repair is a very rare diagnosis.
Integrated multimodality imaging and invasive haemodynamic evaluation allows for meticulous evaluation and appropriate treatment of TV stenosis.
Introduction
Tricuspid valve (TV) stenosis (TS) is a very rare condition and the aetiology is primarily due to rheumatic disease, carcinoid disease, congenital heart disease, endocarditis, and following degeneration of biological valve prosthesis.1–3 In the present case, we describe a rare case of symptomatic TS in a patient with a previously isolated TV repair.
Timeline
| March 2012 | Staphylococcus aureus endocarditis |
| Severe tricuspid regurgitation | |
| Isolated repair of the tricuspid valve (TV) | |
| December 2018 | Fatigue and dyspnoea progressing to New York Heart Association Class III |
| December 2018 | Multimodality evaluation
|
| February 2019 | Tricuspid valve replacement, St. Jude Medical Epic valve (Size 27) |
| March 2019 | Routine checkup. Patient asymptomatic |
Case presentation
A 45-year-old man was evaluated on suspicion of dysfunction of a previous TV repair. Six years earlier, he was treated for Staphylococcus aureus endocarditis without any predisposing factors to endocarditis including absence of intravenous drug abuse or implanted pacemaker leads. The patient was unresponsive to standard medical treatment, he developed total destruction of the anterior leaflet of the TV with subsequent severe tricuspid regurgitation. The patient underwent surgery with isolated repair of the TV without insertion of a tricuspid annular ring. The initial post-procedural mean gradient was between 7 and 10 mmHg but declined to a mean gradient of 7–8 mmHg a year after surgery. In this period of time, the patient was asymptomatic.
During the years following surgery, the patient gradually developed fatigue and dyspnoea progressing to New York Heart Association Class III. On physical examination, auscultation revealed a low-pitched diastolic murmur. Blood pressure was 118/70 and the patient developed moderate peripheral oedema requiring loop diuretics (80 mg o.d.). His functional capacity was severely reduced with a maximal oxygen consumption of 16 ml/kg/min (56% of expected VO2 max). The monitoring of the valve repair with serial transthoracic echocardiography (TTE) demonstrated progressive signs of TS (Figure 1). The peak transvalvular gradient was ultimately increased to 18 mmHg with a mean gradient of 11 mmHg. The TV area was calculated by the proximal isovelocity surface area (PISA) method to 1.2 cm2 (Figure 1), which was confirmed by 3D transoesophageal echocardiography (TOE) with an estimated area of 1.1 cm2 by direct tracing of the opening area in mid-diastole (Figure 2). No tricuspid regurgitation was noted. Systolic function was evaluated by 3D TTE with marginally preserved right ventricular (RV) ejection fraction (RVEF) of 44%, and RV global longitudinal strain (GLS) of −18%, LV ejection fraction was 65%. Cardiac magnetic resonance imaging (CMR) confirmed the TTE findings (Figure 3) with an estimated valve area of 1.3 cm2, RVEF of 45%, a normal RV volume of 85 ml/m2, and a dilated right atrium (RA) of 38 mL/m2 (by TTE 34 mL/m2). Laboratory testing and electrocardiography were unremarkable.
Figure 1.
(Upper panel) Colour Doppler recording demonstrating flow profile with aliasing used for estimation of valve area by the proximal isovelocity surface area method. (Lower panel) Two-dimensional transthoracic echocardiography recording of a modified four-chamber view, same as upper panel with colour Doppler removed.
Figure 2.
Three-dimensional transoesophageal echocardiography recording of the tricuspid valve area estimated by direct tracing of the opening in mid-diastole.
Figure 3.
(Left panel) Cardiac magnetic resonance imaging recording showing stenotic tricuspid valve in a modified right ventricular outlow tract view. (Right panel) Cardiac magnetic resonance imaging short-axis view of stenotic tricuspid valve. The yellow arrow points to the leaflets of the TV. LV, left ventricle; RA, right atrium; RV, right ventricle; TV, tricupid valve.
For further haemodynamic characterization, a right heart catheterization with thermodilution was performed at rest and during submaximal exercise. The direct invasive mean gradient was assessed by catheter pull-back from the right ventricle to RA at rest and during submaximal exercise (75 W) demonstrating mean gradients of 10 mmHg and 24 mmHg, respectively. In addition, right atrial mean pressure increased from 14 mmHg to 26 mmHg during exercise (Figure 4). The pulmonary vascular resistance was within normal limits at rest (2.3 wood units) and during exercise (0.8 wood units). The mean pulmonary arterial wedge pressure was 7 mmHg at rest and 15 mmHg at peak exercise. The systemic blood pressure increased as expected during exercise from 133/74 to 169/79 mmHg (Supplementary material).
Figure 4.
(Upper left panel, A) Pressure tracings of resting right atrial pressure. (Upper right panel, B) Resting mean pulmonary arterial pressure. (Lower left panel, C) Right atrial during exercise. (Lower left panel, D) Mean pulmonary artery pressure during exercise. Yellow arrows denote a-wave (from right atrial contraction), c-wave (bulging of the tricuspid valve into the atrium during iso-volumetric contraction) and v-wave (filling of the right atrium during right ventricular contraction). Green arrows and lines denote x-descent (from atrial relaxation) and y-descent (from opening of the tricuspid wave—note the blunted y-descent compared to x-descent). In all four panels corresponding electrocardiogram tracings II and V5 are in the top of the tracing.
At the multidisciplinary heart team conference, it was decided to perform a TV replacement. Before surgery, a normal computed tomography coronary angiogram was performed. A St. Jude Medical Epic valve (Size 27) (St Jude Medical, Inc., St Paul, MN, USA) was implanted, the procedure was uneventful and the patient is now asymptomatic.
Discussion
This case report presents an integrated multimodality imaging and haemodynamic approach to evaluate and document the suspicion of development of a symptomatic significant stenosis in a previous TV repair. Development of stenosis in TR repair requiring reintervention is rare as documented in a recent valve in valve international registry in which 22 patients out of total 306 cases from 80 centres over a 10 year period underwent a valve in valve procedure.4 The failure of TR repair or replacement is often due to infective endocarditis but might also be due valve thrombosis.4 In the present case, the patient initially had TV endocarditis with S. aureus without any known predisposing risk factors. However, during the time he developed symptoms and signs of a TR repair stenosis no episodes of either endocarditis or valve thrombosis were noted. The TV repair was done without ring annuloplasty, because only the anterior leaflet was affected and bicuspidalization of the valve made it patent. In addition, minimizing the amount of implanted material was intended to minimize the risk of reinfection. The patient was therefore not considered for a valve in valve procedure. However, insertion of a Melody valve in a TR repair without a ring has previously been performed with success in a single patient with TR repair stenosis.5
The evaluation of a suspected TR repair stenosis can be challenging due to the complex anatomy and function of the TV and right ventricle. Transthoracic echocardiography, TOE (including 3D), CMR and right heart catheterization can provide anatomical and functional data in order to fully characterize the degree of stenosis. This also provides overview of the impact on the right-side haemodynamics and RV myocardial function. The multimodality approach offers important information with regard to surgery indication, per-and post-operative outcome which is especially important if a re-do procedure is considered.
The severity of the patient symptoms was documented by a considerable reduced maximal oxygen consumption of 16 mL/kg/min. In order to evaluate the degree of TV stenosis echocardiography (both 2D and 3D) and CMR was performed. Transthoracic echocardiography indicated a severe TS as peak and mean Doppler gradients were 18 mmHg and 11 mmHg, respectively which was supported by a valve area of 1.2 cm2 estimated by the PISA method. The formula used for the PISA calculation is originally intended for use on the mitral valve and no angle correction was applied. However, reduced valve opening area was also documented by 3D TOE planimetry and CMR with similar results. The evaluation of right atrial and ventricle volumes and RVEF was done by CMR and 3D TTE. Right ventricular myocardial function was considered to be marginally reduced evaluated by RVEF obtained by 3D TTE and CMR analysis. Right ventricular GLS was also in the lower normal range and invasive cardiac index was slightly reduced. The resting mean right atrial pressure was at least moderately elevated (14 mmHg) but increased dramatically during submaximal exercise (26 mmHg). A direct transvalvular mean gradient from RV–RA was at rest 10 mmHg corresponding reasonably to the Doppler gradient obtained by TTE. Remarkably, the transvalvular mean gradient was as high as 24 mmHg and increase of the cardiac output was diminished during modest exercise (75 W) both observations translate directly to the poor physical capacity and functional dyspnoea at mild exertion.
Conclusion
Development of TS following valve repair is a rare condition requiring a meticulous multimodality diagnostic approach to determine the severity of the TS and to evaluate the RV function. In the present case, the combination of advanced imaging and invasive exercise haemodynamics extended our knowledge of the patient and the underlying pathophysiological mechanism allowing for appropriate treatment.
Lead author biography
Dr Jesper Khedri Jensen is a cardiology specialist registrar at the Department of Cardiology, Aarhus University Hospital, Skejby, Denmark. His areas of interest include structural heart disease and heart failure. He has authored research in clinical cardiology including biomarkers and ischaemic heart disease. He is currently conducting research in patients with secondary tricuspid regurgitation to extend his interest in this field.
Supplementary material
Supplementary material is available at European Heart Journal - Case Reports online.
Slide sets: A fully edited slide set detailing this case and suitable for local presentation is available online as Supplementary data.
Consent: The author/s confirm that written consent for submission and publication of this case report including image(s) and associated text has been obtained from the patient in line with COPE guidance.
Conflict of interest: none declared.
Supplementary Material
References
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