What is the management plan for a patient with elevated right ventricular systolic pressure (RVSP) and a severely dilated left atrium, as indicated by a transthoracic echocardiographic report?

Management of Elevated RVSP and Severely Dilated Left Atrium

This patient requires right heart catheterization to accurately measure pulmonary pressures, as the incomplete TR jet likely underestimates the true RVSP, and the severely dilated left atrium suggests underlying left-sided pathology that may be driving elevated pulmonary pressures. 1

Immediate Diagnostic Priorities

Confirm True Pulmonary Pressures with Invasive Measurement

• Right heart catheterization is indicated when clinical and noninvasive data regarding pulmonary artery pressures are discordant or insufficient, which applies directly to this case where the TR jet is incomplete 1
• The incomplete Doppler profile means the measured RVSP of 37.3 mmHg is unreliable and likely significantly underestimates true pressures 1
• Invasive measurement will provide accurate pulmonary artery systolic pressure, pulmonary vascular resistance, and right atrial pressure—essential for determining the cause and severity of pulmonary hypertension 1
• Direct measurement of cardiac output via Fick method (not thermodilution, which is inaccurate with significant TR) should be obtained 1

Identify the Underlying Cause of Left Atrial Dilation

The severely dilated left atrium is the critical finding that directs your diagnostic approach:

• Assess for left-sided valvular disease, particularly mitral regurgitation or mitral stenosis, which are common causes of left atrial enlargement and secondary pulmonary hypertension 1
• The moderate mitral annular calcification noted on this echo raises concern for mitral valve disease that may not have been fully characterized 1
• Evaluate for left ventricular diastolic dysfunction as a cause of elevated left atrial pressure, even though LV systolic function is normal 1
• Consider atrial fibrillation as both a cause and consequence of left atrial dilation—obtain ECG and assess rhythm history 1

Determine if Tricuspid Regurgitation is Primary or Secondary

• Measure tricuspid annular diameter in the apical 4-chamber view: >40 mm (or >21 mm/m² indexed to BSA) indicates significant annular dilation and functional TR 1
• Assess for leaflet tethering height and area, which contribute to functional TR with RV remodeling 1
• Distinguish primary TR (from leaflet pathology) versus secondary/functional TR (from annular dilation or RV dysfunction) as this impacts treatment decisions 1

Additional Diagnostic Testing

Advanced Imaging for RV Assessment

• Cardiac MRI is the preferred method to assess RV volumes and systolic function when echocardiographic windows are suboptimal or when precise volumetric data are needed 1
• Real-time 3D echocardiography can provide more accurate RV volume assessment than 2D echo 1
• RV free wall longitudinal strain may help assess RV systolic function more sensitively than conventional parameters 1

Assess Hepatic Function

• Obtain liver function tests, as progressive hepatic dysfunction occurs with elevated right atrial pressure from severe TR 1
• This is particularly important if considering surgical intervention, as hepatic congestion affects perioperative risk 1

Medical Management Strategy

Treat the Underlying Left-Sided Pathology

• If left-sided valvular disease is identified, address this first as it is likely the primary driver of pulmonary hypertension 1
• Optimize management of left ventricular diastolic dysfunction if present, including blood pressure control and diuretics 1
• Rate control or rhythm control for atrial fibrillation if present 1

Diuretic Therapy for Volume Management

• Loop diuretics are indicated if signs of right heart failure develop (peripheral edema, ascites, hepatic congestion) 1
• Aldosterone antagonists may provide additive benefit, especially with hepatic congestion promoting secondary hyperaldosteronism 1
• Caution: Aggressive diuresis can worsen low-flow syndrome in severe TR 1

Consider Pulmonary Vasodilator Therapy

• Medical therapies to reduce pulmonary artery pressures may be considered in severe functional TR, but only after excluding left-sided disease and determining pulmonary vascular resistance via catheterization 1
• This is not first-line therapy and should only be considered after comprehensive hemodynamic assessment 1

Surgical Considerations

Indications for Tricuspid Valve Surgery

Based on the 2022 ESC guidelines, TV surgery should be considered in patients with severe secondary TR who are symptomatic or have RV dilatation, in the absence of severe RV or LV dysfunction and severe pulmonary vascular disease 1

Key surgical decision points:

• If severe isolated primary TR with refractory right-sided heart failure: TV surgery can reduce symptoms and recurrent hospitalizations 1
• If severe secondary TR with previous left-sided valve surgery and refractory symptoms: reoperation with isolated TV surgery may be considered if no severe pulmonary hypertension or severe RV dysfunction 1
• Tricuspid annular dilation >40 mm on TTE (>21 mm/m²) or >70 mm on direct intraoperative measurement is a specific threshold for considering concomitant tricuspid repair during left-sided valve surgery 1

Timing Considerations

• Do not delay evaluation: Progressive RV dilation or systolic dysfunction in the setting of severe TR warrants consideration of surgical intervention before irreversible RV dysfunction develops 1
• Asymptomatic patients with progressive RV dilation may be considered for surgery to prevent irreversible RV damage 1

Common Pitfalls to Avoid

• Do not accept the reported RVSP of 37.3 mmHg as accurate when the report explicitly states the TR jet is incomplete and likely underestimates true pressure 1
• Do not attribute elevated pulmonary pressures to primary pulmonary vascular disease without first excluding left-sided causes, given the severely dilated left atrium 1
• Do not focus solely on the tricuspid valve without addressing the underlying left atrial pathology that may be driving the entire process 1
• Do not delay right heart catheterization in cases where echocardiographic data are inadequate or discordant with clinical findings 1
• Do not use thermodilution cardiac output if severe TR is present; use Fick method instead 1

Surveillance and Follow-up

• Serial echocardiography to monitor RV size and function, left atrial size, and TR severity 1
• Repeat assessment of RVSP with complete Doppler interrogation when technically feasible 1
• Monitor for development of symptoms (fatigue, abdominal fullness, edema, palpitations) that would prompt more aggressive intervention 1
• Assess exercise capacity with formal exercise testing in asymptomatic patients to unmask functional limitations 1