Tricuspid Valve Disease: Symptoms, Types, Causes and Treatment

Tricuspid valve disease is often called the “forgotten valve disease” due to its subtle onset and historically limited attention compared to other heart valve disorders. Yet, as our understanding and technology improve, it’s increasingly clear that tricuspid valve pathology can have a profound impact on health and quality of life. In this comprehensive guide, we’ll explore the symptoms, types, causes, and modern treatment options for tricuspid valve disease, synthesizing the latest evidence from clinical research.

Symptoms of Tricuspid Valve Disease

Tricuspid valve disease often progresses slowly, making its symptoms easy to overlook until the disease is advanced. Awareness of early and late symptoms can facilitate timely diagnosis and improve outcomes.

Symptom	Description	Typical Presentation	Source(s)
Fatigue	Reduced energy due to poor cardiac output	Early, non-specific	1 6
Swelling	Edema in legs, ankles, abdomen (ascites)	Advanced disease	1 4 6
Hepatomegaly	Enlarged liver from blood backing up	Advanced disease	1
Jugular Vein Distension	Visible neck vein swelling	Advanced, visible sign	1 6

Table 1: Key Symptoms

Early vs. Late Symptoms

Early symptoms are subtle and often attributed to general tiredness or aging. Fatigue is commonly the first sign, reflecting the heart's decreased efficiency in pumping blood. As the disease progresses, fluid begins to accumulate in tissues due to impaired right heart function, leading to swelling in the legs, ankles, and abdomen (ascites) 1 4 6.

Physical Signs in Advanced Disease

Advanced tricuspid valve disease can cause hepatomegaly (enlarged liver) and visible swelling of the neck veins (jugular vein distension). These signs occur because the malfunctioning tricuspid valve allows blood to back up into the venous system, increasing pressure in the veins and organs 1.

Why Symptoms Are Overlooked

The slow progression and initially mild symptoms often delay diagnosis. Physical signs like edema and ascites become prominent only when the disease is advanced. Furthermore, the symptoms can mimic other diseases, making clinical suspicion and appropriate imaging (especially echocardiography) essential for diagnosis 1 4 6.

Types of Tricuspid Valve Disease

Tricuspid valve disease is not a single entity but encompasses several distinct conditions affecting the valve, each with unique features and clinical implications.

Type	Key Features	Prevalence/Context	Source(s)
Tricuspid Regurgitation (TR)	Leaky valve, backward blood flow	Most common, often secondary	2 4 6 11
Tricuspid Stenosis (TS)	Narrowed valve, restricted blood flow	Rare, often rheumatic	2 6
Mixed Disease	Both regurgitation and stenosis	Uncommon	1 2
Congenital Abnormalities	Ebstein anomaly, tricuspid atresia	Pediatric, rare	3 7

Table 2: Types of Tricuspid Valve Disease

Tricuspid Regurgitation (TR)

TR is by far the most common tricuspid valve abnormality. It occurs when the valve fails to close properly, allowing blood to leak backward from the right ventricle into the right atrium. Most cases are secondary, meaning they result from changes in the heart’s structure, such as annular dilatation due to right ventricular enlargement or left-sided heart disease 2 4 6 11.

• Primary TR: Caused by direct disease of the valve leaflets (e.g., infection, congenital defects, rheumatic disease).
• Secondary (Functional) TR: Stems from annular dilatation or right ventricular dysfunction without intrinsic leaflet disease 2 4 6 11.

Tricuspid Stenosis (TS)

TS is rare, especially compared to regurgitation. It is characterized by narrowing of the tricuspid valve, which restricts blood flow from the right atrium to the right ventricle. TS is most often rheumatic in origin and is usually found alongside other valve diseases 2 6.

Mixed Disease

Some patients exhibit both regurgitation and stenosis. This mixed presentation is uncommon and typically occurs in the context of chronic rheumatic heart disease or extensive valve damage 1 2.

Congenital Tricuspid Valve Disease

Congenital abnormalities include Ebstein anomaly (malformation causing atrialization of the right ventricle) and tricuspid atresia (absence of the tricuspid valve), which are rare and primarily affect pediatric populations 3 7.

Causes of Tricuspid Valve Disease

Understanding the underlying causes of tricuspid valve disease is crucial for both prevention and targeted treatment.

Cause	Mechanism/Trigger	Commonality	Source(s)
Left-sided Heart Disease	Leads to secondary TR via RV/annular dilation	Most common	2 5 6 11
Rheumatic Heart Disease	Directly damages valve leaflets	Decreasing	2 6 11
Infective Endocarditis	Infection damages valve structure	IV drug use risk	2 12
Congenital Defects	Structural malformation at birth	Rare	2 3 7
Carcinoid Heart Disease	Serotonin-induced leaflet thickening	Rare	2
Blunt Trauma/Tumors	Direct injury or infiltration	Uncommon	2
Chronic Pulmonary Disease	Pulmonary hypertension → RV changes	Contributory	2 6 11
Post-Transplant Complications	Graft failure, rejection	In specific groups	8

Table 3: Causes of Tricuspid Valve Disease

Secondary (Functional) Causes

Secondary tricuspid valve disease is by far the most prevalent. It develops due to annular dilation and leaflet tethering, most commonly as a consequence of:

• Left-sided heart diseases (e.g., mitral or aortic valve disease, left ventricular dysfunction)
• Pulmonary hypertension leading to right ventricular enlargement
• Chronic atrial fibrillation 2 5 6 11

Primary (Organic) Causes

Primary disease involves direct damage or malformation of the tricuspid valve leaflets. Causes include:

• Rheumatic fever: Once common, now less so in developed countries 2 6 11
• Infective endocarditis: Especially among intravenous drug users 2 12
• Congenital defects: Ebstein anomaly, tricuspid atresia 2 3 7
• Carcinoid syndrome: Tumors secrete substances that thicken valve leaflets 2
• Blunt chest trauma, tumors, or myxomatous degeneration 2

Special Populations and Rare Causes

• Heart transplant recipients may develop TR due to early primary graft failure or rejection, which negatively impacts survival 8.
• Genetic mutations (e.g., NFATC1 in tricuspid atresia) have been identified, although these remain rare 7.

Treatment of Tricuspid Valve Disease

Recent years have seen significant advances in the treatment of tricuspid valve disease, with an increased emphasis on early intervention and minimally invasive therapies.

Treatment	Indication/Use Case	Key Considerations	Source(s)
Medical Therapy	Symptom control, high surgical risk	Diuretics, manage causes	4 6 10
Surgical Repair	Severe primary/secondary disease	Preferred if anatomy allows	2 4 6 11 12
Surgical Replacement	When repair is not feasible	Higher risk, complex cases	4 12
Transcatheter Therapies	Severe TR, inoperable/high risk	Emerging, less invasive	4 6 9 10 11
Annuloplasty	At time of left valve surgery, annular dilation	Prevents progression	5 6 10 11

Table 4: Treatment Approaches

Medical Management

• Initial therapy often involves medications such as diuretics to control fluid overload and manage symptoms.
• Treat underlying causes, such as controlling pulmonary hypertension, atrial fibrillation, or infection 4 6 10.
• Medical management is typically a bridge to intervention or for those not suitable for surgery.

Surgical Approaches

Repair vs. Replacement

• Surgical repair (e.g., annuloplasty) is preferred over replacement when feasible, especially for functional TR or infective endocarditis, as it preserves native valve function and reduces the risk of prosthetic complications 2 4 6 11 12.
• Replacement is reserved for cases where repair is not possible due to severe valve destruction or fibrosis. It is associated with higher morbidity and mortality, particularly in isolated tricuspid valve surgery or reoperations 4 12.

Annuloplasty in Left-sided Valve Surgery

• Prophylactic annuloplasty is recommended during left-sided valve surgery if there is tricuspid annular dilatation, even when TR is mild. This has been shown to prevent progression to severe TR and the need for reoperation 5 6 10 11.

Transcatheter and Minimally Invasive Therapies

• Transcatheter tricuspid valve interventions (repair and replacement) have emerged as promising alternatives for patients at high surgical risk or with inoperable disease 4 6 9 10 11.
• These therapies are still in early stages, but they offer hope for those previously deemed untreatable due to high operative risk.

Special Scenarios

• Endocarditis: Valve repair is favored over replacement or valvectomy, as the latter is linked to higher mortality and recurrent infection 12.
• Congenital Disease: Surgical intervention remains the mainstay, but in some cases, transcatheter “valve-in-valve” procedures may be considered after prior surgery 3.

Conclusion

Tricuspid valve disease, long overlooked, is now recognized as a significant contributor to heart failure and poor outcomes, particularly when severe. Early recognition, appropriate classification, and tailored treatment are essential to improving patient quality of life and survival.

Key takeaways:

• Tricuspid valve disease often starts with subtle symptoms but can progress to severe edema, liver enlargement, and jugular vein distension 1 4 6.
• The most common form is tricuspid regurgitation, usually secondary to left-sided heart disease or right ventricular enlargement 2 4 6 11.
• Causes range from acquired (rheumatic, infective, functional) to rare congenital or genetic defects 2 3 7 12.
• Treatment is individualized, with medical management for symptom control and surgery (preferably repair) or transcatheter interventions for severe disease 4 6 9 10 11 12.
• Advances in imaging and intervention are transforming outcomes, but early diagnosis and intervention remain critical to success.

By staying alert to the symptoms and advances in treatment, both patients and clinicians can better manage this once-neglected but impactful heart valve disease.