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CardiacTransplantation and Prolonged Assisted Circulation

Chapter 271 | Part 6: Disorders of the Cardiovascular System · Part 6 – Cardiovascular Disorders · Chapter 271


Key Clinical Points

  1. Cardiac transplantation offers a 1-year survival >80% and median survival of 12.5 years (conditional survival of 14.8 years if the recipient survives the first year).
  2. Cardiac allograft vasculopathy (CAV) is a diffuse, aggressive form of coronary artery disease; IVUS and optical coherence tomography are more sensitive than angiography for early detection.
  3. Standard triple-drug immunosuppression includes a calcineurin inhibitor (e.g., Tacrolimus), a corticosteroid (e.g., Prednisone), and an antiproliferative agent (e.g., Mycophenolate Mofetil).
  4. The HeartMate 3 LVAS is a centrifugal, magnetically levitated pump with median survival >5 years and improved functional status (NYHA class I in 68%).
  5. CAV is often silent; it is present in ~30% of patients at 5 years and ~50% at 10 years post-transplant.
  6. Acute cellular rejection (ACR) is T cell–mediated, while antibody-mediated rejection (AMR) is noncellular/antibody-driven.
  7. The REMATCH trial demonstrated a 48% reduction in risk of death with LVAS compared to medical therapy alone.
  8. Infection rates for LVAS are 20-50% at 5 years, most often involving the driveline; infection predisposes to thrombosis and neurologic complications.
  9. Donor selection considers advanced disease severity, comorbidities, donor-recipient matching, and psychosocial factors.
  10. Retransplantation is the only definitive therapy for advanced CAV.

1. DEFINITION & OVERVIEW

Cardiac Transplantation: A life-altering surgical intervention for patients with advanced heart failure who are otherwise healthy enough to receive it. • Clinical Context: ◦ Growing importance of diagnosing TTR amyloidosis (common in elderly) and AL amyloidosis (seen in younger patients). ◦ TTR amyloidosis can be diagnosed via noninvasive nuclear scanning rather than cardiac biopsy. • Survival Statistics: ◦ 1-year survival: >80% ◦ Median survival: 12.5 years ◦ Conditional survival: 14.8 years (if surviving the first year). • Candidate Selection Criteria (Table 271-1):Advanced disease severity: Refractory heart failure; VO <14 mL/kg per min (<12 if on beta blockers) or percent predicted VO <50%; cardiorenal syndrome; use of inotropic therapy to maintain stability. ◦ Comorbidity: Age is not an absolute contraindication, but frailty is a relative contraindication; BMI >35 kg/m² requires weight loss; eGFR <30 mL/min/1.73 m² is a relative contraindication.

1.2 Candidate Selection (Continued)

Donor-recipient matching: ◦ Sensitized individuals require prospective or virtual cross match. ◦ Pulmonary vascular matching: PVR >3 Wood units, transpulmonary gradient >15, and absolute PA systolic pressure >50 mmHg (provided SBP >85 mmHg) are relative contraindications unless responsive to therapy. ◦ Psychosocial: ◦ Tobacco use must cease for ≥6 months. ◦ Substance abuse: Contraindicated if the individual cannot demonstrate control and cessation. ◦ Cognitive-behavioral disabilities/dementia: Contraindicated due to risk of self-harm.


2. EPIDEMIOLOGY

Global Scale: >150,000 patients have undergone cardiac transplantation. • Amyloidosis Trends: ◦ T1R amyloidosis: Increasing prevalence in aging population. ◦ AL amyloidosis: Significant in younger populations. • Infection Prevalence: Chronic infections (Chagas' disease, tuberculosis, viral hepatitis) are increasingly relevant in the candidate pool.


3. ETIOLOGY & PATHOPHYSIOLOGY

Cardiac Allograft Vasculopathy (CAV): ◦ Characterized by proliferative thickening of the vascular intima. ◦ Initiated as diffuse endothelialitis from combined brain death, ischemia-reperfusion injury, and early immunologic insults. ◦ Chronic factors: Hypertension, hyperlipidemia, and disordered glucose regulation. • Rejection Mechanisms:Acute Cellular Rejection (ACR): T cell–mediated; lymphocytic infiltrates in myocardium; typically declines after 6 months. ◦ Antibody-Mediated Rejection (AMR): Noncellular antibody-driven; characterized by immunoglobulin deposition, complement fixation, and allograft arteriolar vasculitis. ◦ Clinical Impact: AMR leads to allograft dysfunction and higher risk of CAV compared to ACR.


4. CLINICAL FEATURES

Advanced Heart Failure Symptoms: ◦ Refractory to conventional therapy; high symptom burden; frequent hospitalization. ◦ Low cardiac output, elevated pulmonary pressures, and poor quality of life. • CAV Presentation: ◦ Often silent in development. ◦ Diffuse involvement throughout the coronary tree. ◦ Prevalence: ~30% at 5 years; ~50% at 10 years post-transplant. • Grading (Table 271-1): ◦ Based on angiographic disease extent and allograft dysfunction (echocardiography/restrictive physiology). ◦ Grades: CAV0 (absent), CAV1 (mild), CAV2 (moderate), CAV3 (severe).


5. DIFFERENTIAL DIAGNOSIS

ACR vs. AMR: ACR is T cell-mediated; AMR is antibody-driven with complement/immunoglobulin markers. • CAV vs. Native CAD: CAV is diffuse and aggressive (immunologic/nonimmunologic); native CAD is typically focal. • Rejection vs. Infection: Must rule out CMV, Aspergillus, Nocardia, or toxoplasmosis. • Malignancy vs. Infection: Posttransplant lymphoproliferative disorders (EBV-driven) require reduced immunosuppression and antiviral therapy.


6. INVESTIGATIONS & DIAGNOSIS

Imaging Modalities: ◦ Routine coronary angiography to assess for diffuse disease. ◦ IVUS or optical coherence tomography (OCT) are more sensitive than angiography for detecting early intimal thickening. • Prognostic Assessment: ◦ Noninvasive testing for coronary flow reserve used to arbitrate prognosis in CAV. • Pharmacotherapy for Prevention: ◦ Statin use to prevent CAV; PCSK9 inhibitors show promise as non-statin options. • Grading System: ◦ Standardized grading (CAV0–CAV3) based on angiographic extent and allograft dysfunction.

6.2 Diagnostic Algorithm for CAV

  1. Routine coronary angiography to assess for diffuse disease.
  2. If angiography is negative but suspicion remains → proceed to IVUS or optical coherence tomography (OCT).
  3. Assess coronary flow reserve using noninvasive testing → use to arbitrate prognosis in CAV.
  4. Evaluate allograft dysfunction via echocardiography or restrictive cardiac physiology.

7. MANAGEMENT & TREATMENT

Immunosuppression Strategy: ◦ Standard: Triple-drug (Tacrolimus, Mycophenolate Mofetil, Prednisone). ◦ Induction (High risk/Renal dysfunction): Basiliximab or Antithymocyte globulin. ◦ ACR Treatment: Corticosteroid pulses; if hemodynamically compromised → rescue polyclonal antibodies + corticosteroids. ◦ AMR Treatment: Plasmapheresis, rituximab, bortezomib, eculizumab, and IVIG. • CAV Management: ◦ Medical options are limited; revascularization (PCI/CABG) only for focal lesions; retransplantation is the only definitive therapy for advanced CAV. • LVAS Management: 1. Blood Pressure: Target mean arterial pressure ≤90 mmHg using RAAS drugs or vasodilators. 2. Antithrombotic Regimen: Vitamin K antagonist (INR 2–3); omit aspirin to reduce bleeding risk. 3. Hemolysis/Anemia: Monitor for iron deficiency and acquired von Willebrand disease. 4. Right Ventricle: Diuretic therapy for congestion. 5. Arrhythmia: Antiarrhythmic therapy or ablation. 6. Infection: Local internal exploration and long-term suppressive antibiotics (20–50% incidence at 5 years). • Malignancy Management: ◦ Monitor for skin malignancies; reduce immunosuppression for EBV-driven lymphoproliferative disorders.


8. PROGNOSIS & COMPLICATIONS

CAV Progression: \sim30% at 5 years → \sim50% at 10 years. • LVAS Complications: ◦ Hemolysis (monitored via LDH). ◦ Thrombosis: Risk of pump malfunction; reoperation (pump exchange) is preferred but has mortality risk. ◦ Infection: Often driveline-related → leads to thrombosis and neurologic complications. ◦ Neurologic events: Ischemic/hemorrhagic strokes occur in both LVAS and advanced heart failure populations.


9. SPECIAL CONSIDERATIONS

Donor Selection: ◦ Inclusion of donor organs from circulatory death (no difference in outcome vs. brain death). ◦ Consideration of chronic infections (Chagas, TB, Hepatitis) in candidates.


10. KEY PEARLS & CLINICAL TRAPS

CAV Detection: IVUS is superior to angiography for identifying early intimal thickening. • LVAS Safety: HeartMate 3 significantly reduces pump thrombosis compared to older devices. • Antithrombotic Choice: Eliminating aspirin in LVAS patients significantly reduces bleeding complications without increasing stroke risk. • Immune Distinction: ACR (T-cell) vs. AMR (Antibody/Complement) dictates the use of steroids vs. plasmapheresis/rituximab.


Reference Tables

TABLE 271-1 Principles for Listing Candidates for Cardiac Transplantation PRINCIPLE Advanced disease severity

Harrison's 22e, p.2028

PRINCIPLE COMMENT
Advanced
disease
severity
Refractory heart failure with a VO of <14 mL/kg per min (<12, if
2
on beta blockers) or percent predicted VO <50%; combination
2
of intolerance to disease-modifying therapy, cardiorenal
syndrome, use of inotropic therapy to maintain stability, or need
for a left ventricular assist system.
271 Cardiac Transplantation
and Prolonged Assisted
Circulation
Mandeep R. Mehra
Donor-
recipient
matching
Sensitized individuals with circulating antibodies should have
a prospective or virtual cross match; pulmonary vascular
resistance with a transpulmonary gradient >15, PVR >3
Wood units, and absolute PA systolic pressure >50 mmHg
(provided the systolic blood pressure is >85 mmHg) is a relative
contraindication unless reactive to therapy.

TABLE 271-2 Immunoprophylaxis Drugs in Cardiac Transplantation

Harrison's 22e, p.2029

DRUG CLASS GENERIC DRUG CELLULAR TARGET MAJOR SIDE EFFECTS
Calcineurin inhibitors Cyclosporine Binds to cyclophilin, which then inhibits calcineurin Hypertension, dyslipidemia, gum hypertrophy,
hypertrichosis
Tacrolimus Binds to immunophilin FK506 binding protein, which inhibits
calcineurin
Hypertension, dyslipidemia, alopecia, diabetes mellitus
Rabbit ATG T-cell depletion in blood and peripheral lymphoid tissues through
complement-dependent lysis and T-cell activation and apoptosis
Horse ATG Same as above
Interleukin-2 receptor
antagonists
Basiliximab Inhibition of CD-25 of interleukin 2 receptor Well tolerated; rare hypersensitivity; increased infection
risk if used with calcineurin inhibitors
Azathioprine Imidazolyl derivative and prodrug of 6-mercaptopurine (cell cycle
inhibitor)
Mycophenolate
Mofetil
Inhibits inosine monophosphate dehydrogenase, which controls
guanine monophosphate in the de novo pathway of purine
synthesis (inhibits T- and B-cell proliferation)
Proliferation signal
inhibitors
Sirolimus Binds with FKBP12 and complex inhibits the mechanistic target
of rapamycin (mTOR)
Delayed wound healing, nonspecific
pneumonia, pericardial effusion, hyperlipidemia
(hypertriglyceridemia)
Everolimus Binds to FKBP12, which inhibits mTORC1 (and not mTORC2) Dyslipidemia, stomatitis, pericardial effusions, and
pancytopenia