What is the treatment plan for a dialysis patient with chronic cough, left ventricular hypertrophy (LVH), normal ejection fraction (EF), severe tricuspid regurgitation (TR), and pulmonary hypertension?

Management of Dialysis Patient with Chronic Cough, LVH, Normal EF, Severe TR, and Pulmonary Hypertension

This dialysis patient requires aggressive volume management as the cornerstone of therapy, combined with ACE inhibitors or ARBs for blood pressure control and LVH regression, while addressing the underlying pulmonary hypertension that is driving the severe tricuspid regurgitation. 1

Immediate Priorities: Volume Status Optimization

The primary therapeutic target is achieving euvolemia through ultrafiltration and sodium restriction, as volume overload is the fundamental driver of hypertension, LVH, and contributes significantly to TR severity in dialysis patients 2, 1.

• Optimize dry weight through aggressive ultrafiltration during dialysis sessions, as fluid retention leads to increased right atrial pressure, hepatic congestion, and worsening TR 2
• Implement strict dietary sodium restriction (typically <2g/day) to minimize interdialytic fluid gains 1
• Add diuretics if residual kidney function exists, as this provides symptomatic benefit and helps maintain volume control 2
• Monitor for signs of adequate volume removal: resolution of peripheral edema, normalization of jugular venous pressure, and improved dyspnea 2

Antihypertensive Management and LVH Regression

ACE inhibitors or ARBs should be initiated as first-line agents for multiple therapeutic benefits in this patient 1:

• These agents reduce LVH in hemodialysis patients and have been associated with decreased mortality in observational studies 1
• ARBs may be more potent than ACE inhibitors for LVH reduction 1
• Target predialysis blood pressure of 140/90 mmHg (sitting position) 1
• Consider the dialyzability of the chosen agent: enalapril and ramipril levels are reduced by hemodialysis, while benazepril and fosinopril are not significantly affected 1

If blood pressure remains uncontrolled with ACE inhibitor/ARB plus optimal volume management, add a calcium channel blocker as the next agent 1. Observational data suggest CCBs are associated with decreased total and cardiovascular mortality in dialysis patients 1.

Pulmonary Hypertension Assessment and Management

Right heart catheterization should be performed to accurately characterize the pulmonary hypertension, as echocardiographic estimates have only 55% diagnostic accuracy in severe TR 3. This is critical because:

• Severe TR frequently overestimates or underestimates cardiac output measured by thermodilution 2
• The discordant diagnosis of invasive PHT+/echocardiographic PHT- carries the highest risk for adverse outcomes 3
• Pulmonary capillary wedge pressure (PCWP) is not an accurate reflection of left ventricular end-diastolic pressure in patients with LVH 2

Once invasive hemodynamics are obtained, classify the pulmonary hypertension type 2:

• If PCWP >15 mmHg: This represents post-capillary pulmonary hypertension from volume overload and diastolic dysfunction (HFpEF with LVH)
• If PCWP ≤15 mmHg with elevated pulmonary vascular resistance: Consider pre-capillary pulmonary hypertension requiring PAH-specific therapy evaluation

For post-capillary pulmonary hypertension (most likely in this dialysis patient):

• Aggressive volume removal remains the primary therapy 2
• Continue ACE inhibitor/ARB for afterload reduction and LVH regression 1
• Do not initiate PAH-specific therapies (phosphodiesterase-5 inhibitors, endothelin receptor antagonists, prostacyclins) as these are indicated only for pre-capillary pulmonary arterial hypertension 2

Chronic Cough Evaluation

Systematically evaluate the cough etiology before attributing it to ACE inhibitor therapy 2:

• First, exclude volume overload as the cause: pulmonary congestion from inadequate ultrafiltration commonly presents as chronic cough in dialysis patients 2
• Rule out respiratory infections, which are common in advanced heart failure patients 2
• Consider uremic lung if volume status is optimized but cough persists
• Only attribute cough to ACE inhibitor if: respiratory disorders are excluded, cough disappears after cessation, and recurs after reinstitution 2
• If ACE inhibitor-related cough is confirmed, switch to an ARB rather than discontinuing renin-angiotensin system blockade, as ARBs do not cause cough 1, 4

Severe Tricuspid Regurgitation Management

Conservative management is the appropriate approach for severe TR in this setting 3, 5:

• Severe TR in pulmonary hypertension is associated with low RV contractility and RV-PA uncoupling 5
• TR volume ≥30 mL is associated with poor event-free survival, but this reflects underlying RV dysfunction rather than the TR itself being the primary problem 5
• Surgical or transcatheter tricuspid valve repair is contraindicated in patients with pulmonary hypertension and low RV contractility, as hemodynamic simulations show detrimental consequences including increased RV myocardial oxygen consumption 5
• The TR will improve with successful treatment of volume overload and pulmonary hypertension 6

Monitoring and Follow-up

Implement monthly monitoring of the following parameters 2:

• Peritoneal dialysate drain volume (if on peritoneal dialysis) or ultrafiltration volumes (if on hemodialysis)
• Blood pressure measurements (predialysis and postdialysis)
• Body weight trends
• Clinical signs of volume overload: peripheral edema, jugular venous distension, orthopnea
• Residual kidney function if present (24-hour urine collection for creatinine clearance) 2

Reassess with echocardiography at 3-4 months after optimizing volume status and initiating ACE inhibitor/ARB therapy to evaluate:

• LVH regression
• TR severity (expected to improve with volume optimization)
• RV size and function
• Estimated pulmonary artery pressures 2

Common Pitfalls to Avoid

• Do not initiate PAH-specific therapies without invasive hemodynamic confirmation of pre-capillary pulmonary hypertension, as these agents can worsen outcomes in post-capillary disease 2
• Do not discontinue ACE inhibitor/ARB for mild increases in creatinine (<3 mg/dL), as these changes are generally clinically insignificant and reversible 2
• Do not pursue tricuspid valve intervention in the setting of pulmonary hypertension and RV dysfunction, as this worsens outcomes 5
• Do not rely solely on echocardiographic estimates of pulmonary artery pressure in severe TR, as accuracy is only 55% 3
• Avoid polyacrylonitrile dialysis membranes if using ACE inhibitors due to risk of anaphylactoid reactions 1