Tricuspid and pulmonary valve disease

01OverviewDefinition, clinical context and the essential points that orientate the chapter.

Right-sided lesions are often secondary or congenital and may remain clinically subtle until venous congestion, exercise limitation or arrhythmia appears. Decision-making therefore depends more on serial RV structure and function than on murmur intensity alone.

Management combines treatment of the driver, careful decongestion and timely anatomical correction. Advanced liver or kidney dysfunction, severe pulmonary vascular disease and established RV failure can make a late intervention futile or hazardous.

Key points

Most tricuspid regurgitation (TR) is secondary to annular dilatation from left-heart disease, pulmonary hypertension, atrial fibrillation or RV remodelling; exclude device-lead injury and endocarditis.
Severe TR causes giant v waves, a pulsatile liver, ascites, oedema and a pansystolic murmur that increases on inspiration; a quiet murmur does not exclude advanced disease.
Pulmonary stenosis is usually congenital; pulmonary regurgitation commonly follows repaired tetralogy of Fallot or prior valvotomy/conduit surgery.
TTE grades lesion severity and assesses RV size/function and pulmonary pressure; CMR is especially valuable when RV volumes or pulmonary regurgitant fraction will determine intervention.
Diuretics relieve congestion but do not correct the valve or halt progressive RV dysfunction.
Moderate or severe TR should be considered for repair when mitral surgery is already planned; isolated severe disease needs early valve-centre review before major RV or end-organ damage.
Balloon pulmonary valvuloplasty suits significant doming valvar stenosis; dysplastic valves, sub/supravalvar obstruction or associated lesions may require surgery.
A dysfunctional RV-to-pulmonary-artery conduit may be treated by transcatheter pulmonary valve implantation in an adult congenital heart disease centre when anatomy is suitable.

02AetiologyUnderlying causes, associations and risk factors, with why each one matters.

Secondary tricuspid regurgitation

Left-heart disease, pulmonary hypertension, atrial fibrillation or right-ventricular remodelling can dilate the tricuspid annulus and/or tether the leaflets, creating regurgitation despite initially normal valve tissue.

Primary tricuspid injury

Endocarditis, an intracardiac device lead or congenital leaflet abnormality can directly prevent coaptation. Mechanism-focused imaging is needed because a nearby lead is not necessarily causal.

Congenital pulmonary stenosis

A doming pulmonary valve is the usual congenital valvar substrate. Dysplastic leaflets or obstruction below or above the valve alter the physiology and procedural options.

Post-procedural pulmonary regurgitation

Tetralogy repair, pulmonary valvotomy or right-ventricular-to-pulmonary-artery conduit surgery may impair valve competence, leaving chronic regurgitation and progressive right-ventricular volume loading.

03PathophysiologyThe causal sequence from the underlying abnormality to symptoms and harm.

1
Lesion-specific valve dysfunction
Tricuspid annular dilatation or leaflet injury permits systolic backflow into the right atrium. Pulmonary stenosis obstructs systolic right-ventricular outflow, whereas pulmonary regurgitation permits diastolic backflow into the right ventricle.
2
Distinct pressure and volume loads
Pulmonary stenosis creates a right-ventricular pressure load; pulmonary regurgitation creates a right-ventricular volume load. Tricuspid regurgitation volume-loads the right atrium and ventricle while reducing effective forward stroke volume.
3
Chamber-specific remodelling
Pulmonary stenosis tends to produce right-ventricular hypertrophy, while pulmonary regurgitation tends to produce right-ventricular dilatation. In tricuspid regurgitation, right-atrial and annular enlargement can further impair coaptation; advanced pulmonary lesions may also cause secondary tricuspid regurgitation.
4
Reduced forward flow
Severe pulmonary stenosis directly limits pulmonary flow. Severe regurgitation can reduce effective forward flow, particularly once right-ventricular dysfunction develops, leading to exertional intolerance and, in advanced disease, hypotension, syncope or low-output decompensation.
5
Systemic venous consequences
Tricuspid regurgitation directly transmits systolic pressure into the systemic veins; pulmonary stenosis or regurgitation does so mainly after right-ventricular failure raises right-atrial pressure. The result may be prominent venous waves, pulsatile hepatomegaly, ascites, oedema and end-organ congestion.

04Clinical features and red flagsSymptoms, examination findings, patterns of presentation and time-critical warnings.

Systemic venous congestion

Raised JVP with prominent v waves, oedema, ascites, hepatomegaly or pulsatile liver suggests important TR and right-heart failure.

Inspiratory murmur

A left-lower-sternal pansystolic murmur that becomes louder on inspiration supports TR; severe low-flow TR can be soft.

Pulmonary stenosis phenotypeRed flag

Ejection click, harsh upper-left-sternal systolic murmur, RV heave and exertional dyspnoea/syncope suggest significant RV outflow obstruction.

Post-repair pulmonary regurgitation

Previous tetralogy-of-Fallot repair, pulmonary valvotomy or RV-PA conduit plus falling exercise capacity, palpitations or RV dilatation is a key pattern.

Acute severe TRRed flag

Fever, injection-drug exposure or an intracardiac lead with new right-heart failure raises endocarditis or lead-related injury; obtain cultures and urgent imaging.

DecompensationRed flag

Hypotension, cool peripheries, oliguria, lactate rise, syncope or cyanosis indicates low output or critical obstruction and needs emergency escalation.

05InvestigationsWhat to request, why it matters and how to interpret it.

Investigation order

Read from the initial assessment onwards. Tests may run in parallel in urgent care; first-line, preferred, confirmatory, definitive and gold-standard labels appear only when the chapter explicitly states them.

01
12-lead ECG and rhythm monitoringFirst step
Why
Detect AF, atrial enlargement, RV hypertrophy and ventricular arrhythmia.
Interpretation and limitations
AF often drives atrial functional TR; ventricular arrhythmia after congenital repair increases urgency and may precede overt RV failure.
02
Transthoracic echocardiography
Why
Define mechanism and severity; measure chambers, RV function, vena contracta/flow and estimated pulmonary pressure.
Interpretation and limitations
Integrate several parameters. Severe TR can make Doppler pulmonary-pressure estimates unreliable; preserved TAPSE alone does not prove a healthy RV.
03
TOE or 3D echocardiography
Why
Clarify leaflet anatomy, lead interaction, vegetations and transcatheter suitability.
Interpretation and limitations
Use when TTE is discordant or an intervention/endocarditis is being considered.
04
Cardiac MRI
Why
Quantify RV end-diastolic/end-systolic volumes, ejection fraction and pulmonary regurgitant fraction.
Interpretation and limitations
Preferred reference for serial RV assessment in repaired congenital disease; adverse trends can trigger referral before symptoms become marked.
05
CT and/or right-heart catheterisation
Why
Map RVOT/conduit anatomy and coronary proximity; resolve pulmonary haemodynamics when non-invasive estimates are uncertain.
Interpretation and limitations
Invasive measurement distinguishes pulmonary vascular disease from valve-driven congestion and informs procedural risk.
06
FBC, renal profile, LFT, BNP/NT-proBNP and blood cultures when febrile
Why
Assess end-organ impact and identify infection.
Interpretation and limitations
Congestive cholestasis, falling eGFR or rising natriuretic peptide supports advanced haemodynamic burden; multiple pre-antibiotic culture sets are required if stable.

06Differential diagnosisRealistic alternatives and the features that help distinguish them.

Left-sided heart failure

Pulmonary crackles, left-sided valve disease or primary left-ventricular dysfunction may drive secondary right-heart signs. Echocardiography clarifies whether tricuspid disease is cause or consequence.

Constrictive pericarditis

Raised JVP, oedema and ascites can resemble severe tricuspid disease. Respiratory ventricular interaction, pericardial thickening or calcification and a non-regurgitant valve support constriction.

Pulmonary vascular disease

Pulmonary hypertension or acute embolism can cause right-ventricular dilatation and functional regurgitation without primary leaflet disease. Pulmonary pressure, vascular imaging and clinical onset discriminate.

Hepatic or renal oedema

Cirrhosis and kidney disease cause oedema or ascites, but usually lack prominent tricuspid v waves, a pulsatile liver and valve-specific abnormalities on echocardiography.

Atrial septal defect

A pulmonary flow murmur with right-heart enlargement may reflect an atrial shunt rather than pulmonary stenosis. Fixed splitting of the second sound and imaging identify the communication.

07ManagementImmediate care, first-line treatment, alternatives and escalation.

01TRSevere tricuspid regurgitationFirst stepSevere TR with symptoms, RV enlargement/dysfunction, end-organ congestion or planned left-sided surgery.

1Confirm primary versus secondary mechanism and optimise AF, left-sided valve disease, pulmonary pressure and congestion.
2If mitral surgery is planned and TR is moderate or severe, discuss concomitant tricuspid repair with the Heart Team.
3If isolated severe primary TR is symptomatic or causing progressive RV dilatation, refer early for repair/replacement assessment before severe RV or hepatic dysfunction.
4If surgery is prohibitive, obtain TOE/CT anatomical assessment for a specialist transcatheter option; if anatomy is unsuitable, continue structured decongestion and advanced-HF review rather than declaring the lesion benign.

02PSPulmonary stenosisSymptoms, severe Doppler obstruction or RV pressure/functional consequence.

1Confirm that obstruction is valvar rather than subvalvar, supravalvar or conduit-related and assess associated congenital lesions.
2For suitable doming valvar stenosis, discuss balloon valvuloplasty in an adult congenital heart disease centre.
3If the valve is dysplastic, regurgitation is important, anatomy is multilevel or another repair is required, obtain surgical review.
4If shock or cyanosis accompanies critical obstruction, stabilise in a congenital/structural centre and proceed to urgent catheter or surgical relief.

03PR/RVOTPulmonary regurgitation or conduit failureRepaired congenital disease with symptoms, arrhythmia, RV dilatation/dysfunction or important RVOT obstruction.

1Quantify RV volumes and pulmonary regurgitation by CMR and document exercise capacity and rhythm burden.
2Review serial change rather than waiting for severe symptoms; refer when RV enlargement/function, symptoms or arrhythmia reach intervention thresholds.
3Use CT/catheter assessment to test conduit size, landing zone and coronary compression risk for transcatheter pulmonary valve implantation.
4Choose surgery when anatomy is unsuitable, endocarditis is suspected, the outflow tract needs reconstruction or other cardiac lesions need correction.

Key medicines and prescribing safety1 treatment · regimens, roles and cautions

Symptomatic relief of oedema and ascites while the cause and intervention plan are addressed.

Furosemide

Usually 20-40 mg by mouth once daily initially; titrate to congestion and renal response, with IV treatment for acute decompensation.

Check blood pressure, weight, sodium, potassium and creatinine; excessive diuresis can reduce preload and cardiac output in RV failure or severe obstruction.

08ComplicationsImportant consequences, why they occur and why they matter clinically.

Progressive right-heart failure

Persistent pressure or volume overload can eventually exceed right-ventricular compensation, causing low forward output together with worsening venous congestion and reduced exercise capacity.

Congestive liver disease

Chronically raised hepatic venous pressure can produce painful hepatomegaly, abnormal liver function and fibrosis. Advanced hepatic injury can increase procedural risk and limit benefit from late intervention.

Cardiorenal dysfunction

Renal venous congestion and reduced perfusion lower filtration, promote sodium retention and make decongestion more difficult, reinforcing oedema and worsening prognosis.

Atrial arrhythmia

Right-atrial enlargement and surgical scar promote atrial flutter or fibrillation. Loss of coordinated filling and rapid conduction can precipitate right-heart decompensation.

Endocarditis

Abnormal right-sided valves, prosthetic conduits and intracardiac leads can become infected. Septic pulmonary emboli, valve destruction or acute regurgitation may follow.

09Monitoring and follow-upTreatment response, safety checks and longer-term review.

Record NYHA class, exercise tolerance, weight, oedema, ascites and JVP at each review.
Repeat TTE at an interval set by severity and RV response; shorten the interval for new symptoms, pregnancy planning or changing measurements.
Use serial CMR for repaired congenital lesions when RV volumes or pulmonary regurgitation are not reliably quantified by echo.
Monitor renal function, electrolytes and liver profile during diuretic adjustment and advanced congestion.
Use ambulatory ECG and/or exercise testing for palpitations, syncope, congenital repair or unexplained functional decline.
After repair/replacement, establish a lifelong valve or adult congenital follow-up plan, including endocarditis prevention advice and prosthesis surveillance.

10Special situationsVariants, exceptions and circumstances that change the usual approach.

Carvallo sign is supportive, not exclusionary

Inspiratory augmentation supports TR, but marked regurgitation with equalised pressures can produce little audible change.

TR is not simply a bystander

Persistent severe secondary TR can progress despite successful treatment of the left-sided lesion; timing should be decided before RV failure is fixed.

Pacemaker leads need mechanism review

A lead may impinge a leaflet, but association alone is not causation; 3D imaging distinguishes direct injury from annular functional TR.

CMR earns its place

The crescent-shaped RV is difficult to quantify by 2D echo, so serial CMR trends often determine pulmonary-valve timing.

Intervention does not erase congenital follow-up

A transcatheter pulmonary valve treats the conduit/valve lesion but does not remove arrhythmia, RV or endocarditis risk.

11Common pitfallsFrequent interpretation and management errors.

01
Calling severe TR harmless because it is secondary or the murmur is quiet.
02
Using diuretics as definitive treatment while RV size, function and hepatic congestion worsen.
03
Accepting an echo-derived pulmonary pressure at face value when severe TR makes the estimate unreliable.
04
Sending repaired congenital pulmonary disease to a general valve pathway without adult congenital expertise.
05
Waiting for severe symptoms before referral despite progressive RV dilatation or arrhythmia.