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Tricuspid Valve Disease

Chapter 277 | Part 6: Disorders of the Cardiovascular System · Part 6 – Cardiovascular Disorders · Chapter 277


Key Clinical Points

  1. Tricuspid stenosis (TS) is rare in North America and Western Europe, usually rheumatic in origin, and almost always associated with mitral stenosis (MS).
  2. Tricuspid regurgitation (TR) is common; >85% of cases are secondary (functional) due to annular dilation and leaflet tethering from RV remodeling.
  3. Carvallo's sign: A holosystolic murmur of TR that intensifies during inspiration and reduces during expiration or Valsalva maneuver.
  4. Surgical indications for TR include annular dilation >40 mm on TTE (or >70 mm intraoperatively), history of right heart failure, or PA hypertension.
  5. Transcatheter edge-to-edge repair (TEER) and transcatheter replacement are options for high/prohibitive surgical risk patients.
  6. Diuretics and aldosterone antagonists are used to manage systemic venous congestion in severe TR.
  7. ECG in TS shows RA enlargement (tall P waves in lead II, upright P waves in V) without evidence of RVH.
  8. Severe TS is characterized by a valve area ≤1 cm² or pressure half-time of ≥190 ms.
  9. Severe TR leads to RA enlargement, RV volume overload, and eventual systolic dysfunction.
  10. Perioperative mortality for isolated tricuspid valve surgery is high (~8–9%).

DEFINITION & OVERVIEW

Tricuspid Stenosis (TS): ◦ Rare in North America and Western Europe; generally rheumatic in origin. ◦ More common in women than men. ◦ Does not occur as an isolated lesion; almost always associated with mitral stenosis (MS). ◦ Hemodynamically significant TS occurs in 5–10% of patients with severe MS. • Tricuspid Regurgitation (TR): ◦ Common clinical finding. ◦ Primary (Organic): Resulting from congenital defects, endocarditis, radiation, or trauma. ◦ Secondary (Functional): >85% of cases; caused by annular dilation and leaflet tethering due to RV remodeling (pressure/volume overload), MI, or trauma. ◦ CIED-related: Associated with pacemaker or defibrillator leads causing leaflet entrapment or perforation; also includes functional TR from RV apex pacing.


EPIDEMIOLOGY

Tricuspid Stenosis: ◦ Rare; primarily rheumatic; more common in women. • Tricuspid Regurgitation: ◦ Common; >85% are secondary (functional) in nature.


ETIOLOGY & PATHOPHYSIOLOGY

Tricuspid Stenosis Pathophysiology: ◦ Defined by a diastolic pressure gradient between the RA and RV. ◦ Gradient increases during inspiration and decreases during expiration. ◦ Mean diastolic pressure gradient of 4 mmHg is sufficient to cause systemic venous congestion (hepatomegaly, ascites, edema). ◦ In sinus rhythm: RA a-wave may be very tall; y-descent is prolonged. ◦ Impact on MS: TS can mask hemodynamic/clinical features of associated MS by lowering cardiac output (CO) and preventing rise in CO during exercise. • Tricuspid Regurgitation Pathophysiology: ◦ Backward flow from RV to RA depends on driving pressure (RV systolic pressure) and orifice size. ◦ Severity influenced by: PA systolic pressure, tricuspid valve annulus dimensions, respiratory cycle changes in RV preload, and RA compliance. ◦ Clinical impact: Reduced forward CO; RA enlargement; prominent c-v waves; "ventricularization" of the RA wave form. ◦ Progression: Severe TR → RV dilation (volume overload) → systolic dysfunction (accelerated by PA hypertension or myocardial fibrosis). • Etiologies of Tricuspid Valve Disease: ※ Table 277-1 summarizes causes:

VALVE LESION ETIOLOGIES
Tricuspid stenosis Rheumatic, Congenital
Tricuspid regurgitation Rheumatic, Endocarditis, Myxomatous (TVP), Carcinoid, Radiation, Congenital (Ebstein's), Trauma (including that due to RV endomyocardial biopsy), CIED related, Leaflet impingement, adherence, laceration, perforation, avulsion; chordal entrapment

Secondary TR: Caused by RV/tricuspid annular dilation from pulmonary HTN, post-RV MI, left-sided heart disease, cardiomyopathy, or AF.


CLINICAL FEATURES

General Features: ◦ TS: Marked systemic venous congestion; patients may have little dyspnea despite severe hepatomegaly/ascites. ◦ TR: Often coexists with left-sided disease; symptoms (dyspnea, fatigue) may be dominated by those lesions. ◦ Atrial fibrillation (AF): May cause palpitations and drive functional TR. • Tricuspid Stenosis Specifics: ◦ Severe TS → marked hepatic congestion (cirrhosis, jaundice, malnutrition), anasarca, ascites. ◦ Physical signs: Distended jugular veins; in sinus rhythm, giant a-waves and slow y-descent; prominent presystolic pulsations of enlarged liver. • Tricuspid Regurgitation Specifics: ◦ Neck veins: Distended with prominent c-v waves and rapid y-descents (if no TS). ◦ Physical signs: Hepatomegaly with systolic pulsations, ascites, pleural effusions, positive hepatojugular reflux. ◦ Carvallo's Sign: Holosystolic murmur of TR → intensifies during inspiration → reduces during expiration or Valsalva maneuver. ◦ Differentiation from MR: Distinguished by respiratory variation and extent of RV enlargement.


DIFFERENTIAL DIAGNOSIS

Tricuspid Stenosis: ◦ Often confused with MS due to similar diastolic murmur qualities. ◦ Differentiation: Tricuspid murmur heard best along left lower sternal border/xiphoid; augmented during inspiration; reduced during expiration/Valsalva.


INVESTIGATIONS & DIAGNOSIS

  1. Electrocardiography (ECG): ◦ Look for RA enlargement: Tall, peaked P waves in lead II; prominent, upright P waves in lead V. ◦ Clinical Clue: Absence of RVH in a patient with right-sided heart failure and suspected MS → suggests associated tricuspid valve disease.
  2. Chest Radiography: ◦ Combined TS/MS: Prominent RA and superior vena cava; azygos vein engorgement; less pulmonary vascular congestion than isolated MS.
  3. Echocardiography (TTE):Tricuspid Stenosis: → Identify thickened valve, diastolic dome → measure transvalvular gradient. → Severe TS criteria: Valve area ≤ 1 cm² OR pressure half-time ≥ 190 ms. ◦ Tricuspid Regurgitation: → Assess RA dilation, RV volume overload, and tricuspid leaflet status (prolapsing, flail, scarred, or displaced/tethered). → Identify hepatic vein systolic flow reversal (sign of severe TR). → Use color flow Doppler to assess severity.
  4. Cardiac Catheterization: ◦ Not routinely necessary for assessment of TS.

MANAGEMENT & TREATMENT

  1. Preoperative Preparation (TS): → Salt restriction, bed rest, and diuretic therapy → reduces hepatic congestion/bleeding risk.
  2. Medical Management: → Diuretics: Useful for severe TR with systemic venous congestion. → Aldosterone antagonists: Used to manage systemic venous congestion in severe TR.
  3. Surgical Intervention (Tricuspid Stenosis): → Recommended for moderate or severe TS. → Timing: Preferably at time of surgical mitral commissurotomy or MVR.
  4. Surgical Intervention (Tricuspid Regurgitation): → Recommended for severe TR undergoing left-sided surgery. → Also performed for moderate/mild TR if: → Annular dilation >40 mm (TTE) or >70 mm (intraoperative) → OR history of right heart failure → OR PA hypertension.
  5. Transcatheter Options: → Transcatheter edge-to-edge repair (TEER): Option for high/prohibitive surgical risk patients. → Transcatheter replacement: Option for high/prohibitive surgical risk patients.

PROGNOSIS & COMPLICATIONS

Survival and Mortality: ◦ Isolated tricuspid valve surgery: High perioperative mortality (~8–9%). ◦ Risk factors for mortality: Reoperation hazards, reduced RV function. • Complications of Severe TR: ◦ RV volume overload → systolic dysfunction. ◦ Progression accelerated by PA hypertension or myocardial fibrosis.


SPECIAL CONSIDERATIONS

Infective Endocarditis: → Prophylaxis indicated for patients with prior history of endocarditis. • Atrial Fibrillation (AF): → Can be the driver of TR or emerge later due to remodeling.


KEY PEARLS & CLINICAL TRAPS

Diagnostic Clues: ◦ Carvallo's sign is a hallmark of TR. ◦ Tricuspid murmur is best heard at the left lower sternal border; its respiratory variation distinguishes it from MR. ◦ Absence of RVH in patients with right-sided failure and MS suggests TS. • Exclusion Criteria: ◦ TS rarely occurs as an isolated lesion; if suspected, always look for associated MS.


FLOWCHARTS

Management of Tricuspid Regurgitation (Figure 277-2)

The following pathways determine the management of Tricuspid Regurgitation (TR) based on severity, etiology, and clinical context:

Pathway A (Severe, Primary): 1. Tricuspid regurgitation → Severe TR (Stages C and D) → At time of left-sided valve surgery → Primary TR → TV surgery (1).

Pathway B (Severe, Secondary - Medical Failure): 1. Tricuspid regurgitation → Severe TR (Stage C or D) → Right heart failure Stage D → Secondary TR → Poorly responsive to GDMT → Annular dilation without \uparrowPAP or left-sided disease → Absence of severe \uparrowPR or RV systolic dysfunction → TV surgery (2b).

Pathway C (Severe, Secondary - Anatomical/Hemodynamic): 1. Tricuspid regurgitation → Severe TR (Stage C or D) → Right heart failure Stage D → Secondary TR → Poorly responsive to GDMT → Annular dilation without \uparrowPAP or left-sided disease → [Presence of severe \uparrowPR or RV systolic dysfunction] → TV surgery (2a).

Pathway D (Severe, Primary - Specific Conditions): 1. Tricuspid regurgitation → Severe TR (Stage C and D) → At time of left-sided valve surgery → Primary TR → [Annular dilation or systolic dysfunction] → TV surgery (2a).

Pathway E (Progressive, Surgical Timing): 1. Tricuspid regurgitation → Progressive TR (Stage B) → At time of left-sided valve surgery → Annular dilation >0.4 cm or prior right HF → TV surgery (2a).

Pathway F (Progressive, Non-Surgical Timing): 1. Tricuspid regurgitation → Progressive TR (Stage B) → [No left-sided surgery mentioned/implied] → Annular dilation >0.4 cm or prior right HF → TV surgery (2b).

Key Thresholds & Definitions:Severity Stages: Stage C and D (Severe), Stage B (Progressive). • Annular Dilatation: >0.4 cm (used to define significant dilation in the Progressive TR branch). • Clinical Status: "Poorly responsive to GDMT" (Guideline-Directed Medical Therapy). • Hemodynamics: \uparrowPAP (Pulmonary Artery Pressure), \uparrowPR (Pulmonary Resistance), and RV systolic dysfunction.


Reference Tables

TABLE 277-1 Causes of Tricuspid Valve Diseases

Harrison's 22e, p.2059

VALVE LESION ETIOLOGIES
Tricuspid
stenosis
Rheumatic
Congenital