Approach
The main goal of treatment is to relieve the symptoms that result from restricted diastolic filling of the right ventricle and diminished pulmonary blood flow.
Symptomatic management
Initial medical care consists of:
Assessment and treatment of the underlying etiology for the valvular pathology (e.g., antibiotics for bacterial endocarditis or somatostatin analogs for carcinoid syndrome)
Fluid and sodium restriction
Medications to treat cardiac arrhythmias secondary to right atrial distension (usually atrial fibrillation and/or flutter)
Pharmacotherapy with diuretics to reduce morbidity associated with systemic venous congestion.
Activity is usually self-limited by the patient due to easy fatigability and diminished pulmonary blood flow.
Surgery
If right heart failure persists or if low cardiac output develops, surgical commissurotomy or valve replacement is indicated. Historically, the morbidity and mortality of tricuspid valve replacement have been very high, with most series reporting operative mortality in excess of 20%.[43] However, surgery on the tricuspid valve is most commonly carried out alongside surgery for other valve dysfunction, and many patients are treated later in the course of the disease.[36] Risk factors for mortality include urgent/emergent status, age >50 years, functional classification of heart failure, and elevated pulmonary artery pressures.[43]
Successful percutaneous balloon valvotomy has been reported when the annulus size and subvalvar structures are normal.
Once the pathology is corrected, dietary and activity restrictions are no longer necessary and medications may be weaned.
Congenital tricuspid stenosis (TS)
Infants with congenital TS often have incompletely developed cardiac valve leaflets, shortened or malformed chordae, small annuli, abnormal size, and number of the papillary muscles, or any combination of these defects. These patients usually have associated abnormalities of the pulmonary valve as well. In the most severe cases, the entire right heart is underdeveloped and incapable of handling an entire cardiac output.
The infant with congenital TS may require single ventricle palliation (with an initial systemic-to-pulmonary artery shunt followed by a Fontan operation) or cardiac transplantation, plus preoperative prostaglandins and postoperative antiplatelet therapy. Alprostadil is given preoperatively. Infants should have frequent blood pressure checks and respiratory status should be monitored throughout treatment (e.g., continuous pulse oximetry). The duration and choice of antiplatelet therapy varies by institution. However, the most common protocol is to continue low-dose aspirin until the shunt is either taken down surgically or allowed to thrombose over time as the patient grows.
TS with carcinoid heart disease
Right heart failure should be treated with fluid and sodium restriction and use of diuretics. Somatostatin analogs (e.g., octreotide) have been shown to provide symptomatic improvement and improved perioperative survival.[44]
Absolute and relative indications for intervention are not well established. Reviews have suggested that early valve intervention has better results compared with waiting for advanced disease.[45] However, early disease has not been clearly defined in the literature. Most authors support considering intervention at the onset of any cardiac-related symptoms or with the development of right ventricular dysfunction. Furthermore, expert guidelines recommend intervention in the following settings: severe TS (plus tricuspid regurgitation) with symptoms despite medical therapy; and severe TS (plus tricuspid regurgitation) in a patient undergoing left-sided valve intervention.[42]
Some case reports have demonstrated that percutaneous balloon tricuspid dilatation is safe and effective for treating isolated severe TS in carcinoid heart disease.[46] However, the surgical approach is preferred by most experts, especially when there is concomitant regurgitation. Bioprosthetic valves are preferred and long-term results are promising.[47][48] Concomitant replacement of affected pulmonary valves may also result in less right ventricular dilatation.[49] Valve surgery for carcinoid heart disease is of higher risk compared with most other forms of valve surgery; however, significant improvement in functional class has been reported in those who survive the surgery. Long-term complications generally relate to the tumor itself.[50]
TS with rheumatic fever sequelae
Mild to moderate disease:
Medical therapy for symptomatic patients is targeted towards relief of systemic venous congestion secondary to right heart failure and includes fluid and sodium restriction and diuretics to help decrease symptoms and improve hepatic function.
Severe disease:
Surgical valve repair or replacement are the primary options. Warfarin therapy may be advised following either mechanical or bioprosthetic valve placement. Therefore, surgical repair is preferable to valve replacement whenever feasible. Successful percutaneous balloon valvotomy has been reported in some cases in which the annulus size and subvalvar structures were normal.[43][51][52][53][54]
In rheumatic TS, the concomitant involvement of the mitral and aortic valve makes determining absolute and relative indications for tricuspid valve surgery very difficult. Usually the mitral valve is considered the most important valve to address as symptoms present much sooner and more frequently when it is affected. However, studies have shown that patients who have combined mitral and tricuspid valve repair have a better prognosis than those undergoing replacement of the mitral valve alone.[32][55] However, a limited retrospective study has shown patients did well whether or not the tricuspid valve was surgically treated, or when treated they still had residual gradient.[3] Because rheumatic heart disease has declined in developed countries, literature on the long-term outcome of modern valve surgery for rheumatic disease is now scarce.[55] Evidence-based recommendations are therefore lacking. As rheumatic tricuspid stenosis is frequently associated with other valve lesions, indications for interventions should be based on symptoms and clinical consequences (global hemodynamic burden) of the valve lesion(s) and their interactions.[32]
TS with infective endocarditis
Absolute indications for surgery for right-sided endocarditis:
Persistent bacteremia after 1 week of appropriate antibiotic therapy
Right ventricular dysfunction secondary to severe tricuspid regurgitation not manageable with diuretics
Respiratory failure requiring mechanical ventilation with recurrent pulmonary emboli
Involvement of left-sided heart structures
Large residual vegetations (>20 mm) with recurrent pulmonary emboli.[2]
Relative indications for surgery:
Persistent bacteremia despite appropriate antibiotic therapy, candidal endocarditis, and large vegetations (>10 mm).
No indications for surgery:
Appropriate antimicrobial therapy is initiated at the time of diagnosis, and further determined by the sensitivity of the organisms cultured. Duration is usually 4-6 weeks.[2]
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