Graphical abstract
Keywords: Tricuspid valve stenosis, Pacemaker leads, Advanced echocardiography, Right heart catheterization
Highlights
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Tricuspid valve stenosis following pacemaker implantation is rare.
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Advanced echocardiography can identify the cause.
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Invasive hemodynamic evaluation can quantify the effects.
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A multidisciplinary approach ensures an optimal treatment strategy.
Introduction
Tricuspid valve (TV) stenosis (TS) is a rare condition, occurring in 2.4% of cases of primary TV disease,1 and is most often due to rheumatic disease. Other causes are congenital heart disease, endocarditis, and large right atrial tumor.2 Rarely, TS secondary to pacemaker (PM) leads is reported.3,4 We report a case of PM lead–induced TS and describe a multidisciplinary approach to evaluate such patients.
Case Presentation
A 54-year-old woman with no previous medical history had been diagnosed with dilated cardiomyopathy 12 years earlier. Her primary complaint was functional dyspnea classified as New York Heart Association (NYHA) class II-III. Despite optimal heart failure medical treatment, the patient remained symptomatic with an ejection fraction (EF) of 20%. The electrocardiogram showed sinus rhythm with left bundle branch block and QRS width of 160 msec; therefore, the patient had a cardiac resynchronization therapy device implanted. The patient demonstrated an excellent response to the resynchronization therapy with relief of symptoms (NYHA I), and the EF increased afterward to 50%. Twelve years after the cardiac resynchronization therapy device implantation the patient again experienced functional dyspnea (NYHA III) without signs of clinical decompensation. Computed tomography (CT) excluded pulmonary embolism. Transthoracic echocardiography showed a severe dilated right atrium (RA) and severe TS with transvalvular mean gradient of 10 mm Hg and peak gradient of 20 mm Hg (Figure 1). Two-dimensional transesophageal echocardiography (TEE) redemonstrated the presence of severe TS, yet the exact mechanism could not be determined. In addition, a mobile fibrin coating/thrombus measuring 10 × 4 mm was noted within the RA attached to one of the PM leads, which did not involve the valve (Figure 1, Videos 1 and 2). This was considered a high-risk finding given the presence of patent foramen ovale and associated risk for systemic embolism.
Figure 1.
(A) Two-dimensional transthoracic echocardiography showing transvalvular mean gradient of 10 mm Hg and peak gradient of 20 mm Hg of the TV. (B) Two-dimensional TEE recording demonstrating impression of the atrial septum toward the left atrium and a small chamber size of the right ventricle with fibrin coating/thrombus (10 × 4 mm) on the lead. (C) Three-dimensional TEE recording of the TV area estimated by direct tracing of the opening in mid diastole. (D) Measurement of the TV area using 3D flexi slice. LA, Left atrium; LV, left ventricle; RV, right ventricle.
Carcinoid syndrome was excluded with a blood test for chromogranin-A, 5-hydroxyindolacetate, and serotonin. Furthermore, DOTATOC positron emission tomography-CT excluded the carcinoid syndrome diagnosis. The patient underwent a trial of anticoagulation given the lead-related fibrin coating/thrombus with risk for systemic embolism in the setting of a patent foramen ovale and was ultimately referred to our center for further evaluation and management. A three-dimensional (3D) TEE was performed that demonstrated a TV annular area of 8.5 cm2. The TV area was traced to 0.7-1.0 cm2 using 3D TEE. A fibrin coating/thrombus (10 × 4 mm) associated with the implantable cardioverter defibrillator lead was seen in the RA (Figure 1, Videos 2 and 3). The lead was adherent to the posterior and septal leaflet of the TV. However, the mechanical stress of the PM leads on the valve had resulted in fibrotic changes and commissural adhesion of all three valve leaflets. All three valve leaflets had reduced mobility and contributed to the valve stenosis. No significant tricuspid regurgitation was demonstrated (Video 2). Cardiac CT was performed, but due to attenuation artifacts from the leads it was not possible to gain further information about the TV from this modality. A right heart catheterization was performed with the following measures: RA mean gradient, 8-9 mm Hg; pulmonary artery mean gradient, 9 mm Hg; cardiac index, 1.7 L/minute/m2; pulmonary vascular resistance, 3.1 Wood units; and mean pulmonary capillary wedge pressure gradient, 1 mm Hg. A transvalvular TV mean gradient of 7.6 mm Hg and a TV area of 0.5 cm2 using the Gorlin equation5 were demonstrated, supporting the diagnosis of TS (Figure 2).
Figure 2.
Left panel: Resting simultaneous pressure tracings from the right atrium (light green) and right ventricle (red). Right panel: TV area calculated as 0.49 cm2 using the Gorlin equation. Heart rate, 89 beats per minute; cardiac output, 2.9 L/minute; RA mean gradient, 9 mm Hg; pulmonary artery mean gradient, 9 mm Hg; tricuspid mean gradient, 7.73 mm Hg; and TV flow, 60 mL/sec. In both panels the corresponding electrocardiogram tracings are at the top. Note that the waveforms are shown with different sweep speed, not different heart rate.
At a multidisciplinary heart team conference, it was concluded that the TS was likely to be a consequence of PM lead–induced damage of the valvular endothelium leading to leaflet fibrosis and retraction.
The patient was referred to and subsequently operated, and the intraoperative findings were as demonstrated on echocardiography (Figures 3 and 4). A biological valve was inserted without complications, and a new cardiac resynchronization therapy PM was also implanted without defibrillator due to the normal EF, with an epicardial right ventricular lead inserted by the subcutaneous tunneling technique. The peroperative and postoperative course was uneventful, and the patient was discharged in good condition.
Figure 3.
(A, B) Intraoperative picture of the TV seen from the RA. The valve leaflets are marked “anterior,” “posterior,” and “septal.” Retractor placed on the left side of the patient. Venous cannulas are shown. The PM lead passing through the valve is shown with widespread adhesions to the valve tissue as seen in panel C. The valve leaflets are fibrotic and in combination with the adhesions make the valve stenotic. (B) Forceps holding a fibrotic valve leaflet released from the annulus. (C) Removed PM lead.
Figure 4.
Three-dimensional rendering and multiplanar reconstruction of TV showing “the surgeon’s view” and demonstrating the narrowed TV orifice and PM lead.
Discussion
The most common dysfunction of the TV related to PM implantation is tricuspid regurgitation, while TS has been reported very rarely.1 Based on the few available cases of TS caused by PM lead implantation, the pathology of TS appears to be due to either mechanical damage to the endothelium or an obstruction based on a loop formation of the lead. The mechanical damage causes an inflammatory response resulting in fibrosis, calcification, and eventually stenosis. The mechanical damage can be caused by lead perforation, lead adherence and tethering, lead loop restricting the opening, and adhesion to subvalvular apparatus.6, 7, 8
In the present case no findings indicated a mechanical obstruction due to loop formation of the PM lead, and the cause of TS was considered likely to be due to mechanical damage of the endothelium of the valve with induction of inflammation and fibrosis leading to the development of TS. In addition, no thrombus material was demonstrated at the TV. The fact that the therapeutic anticoagulation therapy treatment did not alter the morphological or functional performance of the TV supports these considerations.
The most commonly used treatment for TS caused by PM implantation has been surgical removal of the lead and subsequent repair or replacement of the TV. Other treatment options are medical treatment or percutaneous balloon valvotomy.6 Although long-term data are limited, percutaneous treatment of PM lead–induced TS seems to be safe and effective.7
Due to the complex structure and function of the TV, TS can be challenging to evaluate. Therefore, it is of great importance to use a multidisciplinary and multimodality approach, which can provide anatomical and functional data, to thoroughly evaluate the degree of stenosis and plan the optimal treatment strategy.8,9
With the use of a transthoracic echocardiography we were able to establish TS as a working diagnosis for the patient. To further characterize the TS and confirm the diagnosis, 3D TEE offered a more accurate TV area and a more detailed anatomical characterization of the valve pathology. Using right-sided catheterization, the TV area was estimated to be 0.5 cm2, which was almost equivalent to the TEE finding (0.7-1.0 cm2). Cardiac CT was also used, but due to artifacts from the PM lead it could not be used to describe the TV and the underlying pathology. Thus, by use of advanced echocardiography and right-sided catheterization, we were able to ensure the rare diagnosis and provide an optimal strategy for treatment.
Autopsy studies and heart surgical data have shown that PM leads develop fibrotic attachment to the TV during long-term follow-up and will result in a greater degree of fibrotic adhesion.10,11 With an increasing number of PM implantations, the risk of TS may be expected to increase, but further studies must be conducted to determine a possible increased incidence of TS.
Conclusion
Tricuspid valve stenosis caused by PM lead implantation is very rare, and to ensure the diagnosis and optimal treatment strategy, a multidisciplinary approach is recommended. Given the increasing number of PM implantations, a lead-induced TS must always be included in the differential diagnosis for patients with a PM who present with unexplained TS.
Footnotes
Conflicts of Interest: None.
Supplementary data to this article can be found online at https://doi.org/10.1016/j.case.2021.07.003.
Supplementary Data
TEE, midesophageal four-chamber view, demonstrating turbulent flow across the TV without regurgitation. Figure 1B corresponds to this video.
TEE, midesophageal four-chamber view, demonstrating the fibrin coating/thrombus (10 × 4 mm) on the lead. Figure 1B corresponds to this video.
Three-dimensional TEE recording of the TV with the aortic valve at 11 o’clock demonstrating a narrow area of the TV with a fixed PM lead in the posterior area of the valve. Figures 1C and 4 correspond to this video.
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Associated Data
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Supplementary Materials
TEE, midesophageal four-chamber view, demonstrating turbulent flow across the TV without regurgitation. Figure 1B corresponds to this video.
TEE, midesophageal four-chamber view, demonstrating the fibrin coating/thrombus (10 × 4 mm) on the lead. Figure 1B corresponds to this video.
Three-dimensional TEE recording of the TV with the aortic valve at 11 o’clock demonstrating a narrow area of the TV with a fixed PM lead in the posterior area of the valve. Figures 1C and 4 correspond to this video.