Clinical symptoms : The presentation of TR secondary to PPM/ICD placement may involve symptoms of decompensated right-sided congestive heart failure, such as abdominal distension and fullness, lower extremity edema, dyspnea on exertion, and palpitations related to atrial fibrillation [16, 21, 26]. An enlarged, pulsatile liver is a late finding [27]. In one study, patients with significant lead-induced TR following PPM or ICD implantation (increase of TR severity by ≥2 grades at follow up) had more heart failure related events. This significant TR was even independently associated with increased all-cause mortality [5, 28]. Many patients may remain asymptomatic despite the presence of new or worsening TR. Larger studies have demonstrated that the majority of patients have new-onset or worsening of pre-existing TR several years following implantation, with some suggestion of acute worsening of TR in a small number of patients.

Physical examination: The physical examination may reveal the characteristic respirophasic, high-pitched, holosystolic murmur at the left lower sternal border that increases with inspiration (Carvallo’s sign or maneuver). However, in many this murmur is unimpressive. In fact, the literature reports that only 28% of those with TR evidenced by echocardiography may have a regurgitant murmur on physical exam [29]. Nevertheless, when detected the Carvallo’s maneuver has a sensitivity and specificity of 80% and 100%, respectively [30]. The TR murmurs that increase with inspiration are different from those which are associated with congestive heart failure, which often diminish with inspiration.

Other findings on exam may be consistent with isolated right-sided congestive heart failure, such as jugular venous distension, pulsatile liver, abdominal distension, and lower extremity pitting edema [19, 31]. Hepatojugular reflex may also be seen, with a sensitivity of 66% and specificity of 100% in detecting TR [30]. Likewise, the right atrial V wave is highly sensitive, yet it is not entirely specific for detecting the presence or severity of TR [32]. In addition to signs and symptoms of right-sided heart failure, some may have concurrent signs and symptoms of left-sided heart failure, especially those with some functional TR prior to the procedure and those who need ICD implantation for reduced left ventricular ejection fraction (LVEF).

Risk factors (see Table 4.1 ): The risk factors for developing TR following PPM/ICD implantation are not entirely understood. Some predictors have been shown to be significant in recent studies. Advanced age is found to be a risk factor, with an average age of 73 years [7, 8]. Other predictors include body mass index, pre-device atrial fibrillation, heart rate, moderate or severe mitral regurgitation, history of mitral valve surgery, pulmonary artery systolic pressure ≥37 mmHg, elevated right ventricular systolic pressure and RV dilation [5, 7]. There is conflicting data in the literature regarding whether the placement of more than one lead predisposes to worsening of TR. In the pediatric population, a risk factor for lead-related TR was congenital heart disease which is not right-sided [9].

Diagnosing TR requires both 2D echocardiography (see Fig. 4.3) and color Doppler flow mapping . The severity of TR is graded based on the direction and size of the regurgitant jet, the presence of proximal flow convergence, and vena contracta width [33]. Using the vena contracta width of ≥6.5 mm, the sensitivity and specificity of detecting severe TR is 88.5% and 93.3%, respectively [34]. Other findings in new or worsening TR following PPM or ICD placement include increased RV and RA dimensions, greater pulmonary artery systolic pressure, elevated right ventricular systolic pressure, and decreased right ventricular ejection fraction (RVEF) compared to the pre-procedural values [4].

The utility of 2D echocardiography may be limited as it may underestimate the presence and severity of TR. It proves to be difficult to appreciate the full anatomical relationships between the TV and the ICD or PPM lead(s), as only two out of the three leaflets are visible simultaneously when using any 2D imaging plane [18, 35]. The posterior leaflet, which is implicated in most cases, is only visualized in some views, and is less commonly imaged during the routine echocardiographic examination [35]. In fact, the PPM lead may become entrapped in the thickened, fibrotic, and fused posterior and septal leaflets. These leads are visualized in only 12–17% of patients using 2D echocardiography [18, 19].

Three-dimensional transthoracic echocardiography (3D TTE) affords the ability to visualize all three TV leaflets and the short axis of the TV, not obtainable with 2D echocardiography. This allows the assessment of the route and position of the PPM/ICD lead within the TV apparatus [18, 31, 35, 36]. Mediratta et al. demonstrated that 3D TTE clearly depicted lead position in 90% of patients, in which 46% of patients had impinging leads visualized in the posterior (20%), septal (23%), and anterior (4%) leaflets [36]. Those who did not have lead impingement, i.e. when the lead was visualized intercommisurally or in the middle of the tricuspid orifice, did not have evidence of significant TR compared to those with lead impingement. Due to this strong association between lead impingement and post-procedural TR, it has been suggested that 3D TTE targeted guidance of device- lead placement may be beneficial to avoid lead impingement, as lead placement is solely done under fluoroscopic guidance as of now [36]. It is undetermined, however, whether the lead would maintain its position from the time of placement to the time of development of TR, and therefore, the ultimate utility of intraprocedural 3D TTE is unclear. Additionally, due to the need of dedicated probes and image analysis software, as well as higher cost, 3D echocardiography is not widely used at the moment.

Other evolving imaging methods include contrast-enhanced multidetector computed tomography, which can indirectly be used to detect and grade TR. This is based on early opacification of hepatic veins or the inferior vena cava during first-pass intravenous contrast enhancement. In detecting echocardiographic TR, this particular method has a sensitivity of 90.4% and a specificity of 100% [37, 38].

An additional imaging modality is cardiac magnetic resonance (CMR) , which can be used to detect and quantify TR based on the regurgitant jet area and volume. The sensitivity and specificity of this is 88% and 94%, respectively, compared to right ventricular angiography. Despite the favorable detection of this imaging modality, most pacemaker devices and leads are not compatible with CMR [39].

Medical management: Medical management has been largely studied in patients with functional TR, which involves treating the underlying cause as well as management of congestive heart failure [37]. Aggressive volume diuresis in acute decompensation and general balance of hemodynamics largely by the use of diuretics may be beneficial. There is, however, a paucity of data elucidating the outcomes of these patients with lead-related TR who are medically managed.

Lead(s) extraction: Lead placement is acutely associated with inflammatory changes, as well as chronically with fibrosis and scar tissue formation, which allows for lead adherence to the TV. The main indication for lead extraction is device and lead related infection [40]. The methods and techniques for lead extraction have become more sophisticated and specialized over time. Some leads can be removed by simple traction alone, while others require advanced techniques using locking stylets and laser-equipped sheaths. Percutaneous removal of PPM or ICD leads is often performed in large specialty centers with advanced technical skill and experience. However this carries significant and potentially fatal risk [31]. Major complications of lead extraction include cardiac or vascular avulsion requiring open chest interventions, pulmonary embolism, respiratory distress, stroke, and even death in up to 0.5% of patients. However, in the last decade, success rate of lead extraction has been between 95% and 97%, and the complication rate has remained low at 0.4–1% [40].

Lead extraction itself may paradoxically lead to worsening TR [31, 35, 41]. Major risk factors and predictors of developing TR after extraction are the use of laser sheath or any additional tools for extraction beyond simple traction, extraction of more than two leads, female sex, and patients with longer duration of implantation [42]. Fortunately, There is no significantly increased mortality in those who develop TR post-extraction compared to those who do not develop TR. Tricuspid regurgitation is more likely to occur after PPM extraction than ICD lead extraction. This may be due to a longer duration of implantation or more fibrous tissue deposition and adherence to the TV [41].

Valvular surgery: The decision to operate on a regurgitant TV depends on the severity and clinical situation [27]. Tricuspid valve surgery is clearly indicated in primary severe TR at the time of left-sided valve surgery . It can also be considered in those with symptomatic severe TR who are unresponsive to medical management. Additionally, it may be considered in those who are asymptomatic or have minimal symptoms but have increasing RV dilation and dysfunction prior to any clinical right-sided heart failure.

Tricuspid regurgitation is managed surgically either via surgical repair or replacement depending on the etiology and mechanisms of valve dysfunction. Tricuspid valve repair often involves suture or ring annuloplasty as well as additional adjunctive techniques. It is often used in the case of secondary TR, with the goal of restoring tricuspid annulus geometry as well as concurrently reducing RV afterload by correcting left-sided valvular dysfunction [27]. On the other hand, tricuspid valve replacement is generally done when valve repair is technically not feasible, as in the setting of complex lesions causing severe primary TR and severe tricuspid stenosis. Those with secondary TR with marked RV remodeling and leaflet tethering may also benefit from replacement rather than repair.

It is estimated that approximately 8000 surgical tricuspid repairs occur annually in the United States, the majority of these cases involving patients without ICD or PPM-related TV pathology. Tricuspid valve repair has a success rate of above 85%. Recurrence of TR is common and occurs in about 20–30% of patients [37, 43]. Tricuspid valve replacement is associated with a 6% 30-day post-operative mortality rate, as well as 8% in-hospital mortality [21, 23, 35]. The 10-year survival for patients after a tricuspid valve replacement combined with left-sided heart valve surgery is 78%, but is only 41% in those with triple valve surgery. This is lower than the 10-year survival of patients undergoing aortic valve replacement (65%), mitral valve replacement (55%), and combined aortic and mitral valve replacement (55%) [37, 38].

There is limited data in the literature about surgical treatment in patients with TR secondary to PPM/ICD leads. Some of the literature recommends the removal of the original pacemaker leads and placement of an epicardial or transcoronary sinus lead in those patients who require TV replacement [44]. The disadvantage of epicardial leads has been the relatively high capture thresholds as resulting in frequent battery changes. An alternative is to surgically position the original lead between the sewing ring and the native annulus, or to place it inside the posteroseptal annulus with Lembert-type sutures [26]. Although promising, this technique makes it difficult to remove the lead transvenously in the future.

In regards to TV repair, ring annuloplasty is preferred over suture repair as it lends a lower incidence of recurrent and residual TR, fewer reoperations, and an improved survival for functional TR [43]. Some studies supporting this have included patients who have needed TV repair due to lead-related TR, with favorable outcomes. There is ongoing discussion and controversy over whether flexible versus rigid annuloplasty rings are superior. Some of the literature has shown that a rigid annuloplasty ring does not result in worsening of any residual TR following TV repair, while worsening was appreciated with flexible ring annuloplasty [23]. However, residual TR is still a significant problem which exists following TV repair regardless of the type of annuloplasty ring used. Additionally, there is an associated risk of increased postoperative conduction disturbances with the prosthetic ring compared to the suture annuloplasty technique [27].

Average time to surgery has been described in the literature as 72 months following device implantation [19]. This allows for the argument that lead-related TR is likely to occur over a longer period of time, although a small number of patients have demonstrated acute decompensation within a shorter time frame. These patients fare well and show significant improvement postoperatively [19].

There is some recent data in the literature which outlines and demonstrates significant mortality associated with ≥3+ TR related to PPM’s in particular [5]. Another study described that patients with device-related infection had an 18% all-cause mortality after 6 months of infection [45]. Overall, however, there is a paucity in data in the literature in order to come to a firm conclusion on these patients’ potential benefits from surgery. Specifically, the long-term durability of surgical results remains unknown.