Cardiovascular

Constrictive pericarditis vs restrictive cardiomyopathy

Clinical Overview and When to Suspect Constriction vs Restriction

— US etiologies: idiopathic/viral (most common), post-cardiac surgery, mediastinal radiation (Hodgkin, breast CA), TB (global #1), connective tissue disease, uremia

— Etiologies: amyloidosis (AL, ATTR-wt, ATTRv), sarcoidosis, hemochromatosis, eosinophilic (Löffler/endomyocardial fibrosis), radiation, scleroderma, anthracycline toxicity, Fabry, Gaucher

— Constriction clues: prior cardiac surgery, chest XRT, TB exposure, recurrent pericarditis

— Restriction clues: macroglossia, carpal tunnel (bilateral), low-voltage ECG with LV hypertrophy on echo (amyloid), AV block + lymphadenopathy (sarcoid), bronze skin + diabetes (hemochromatosis)

Board pearl: The single highest-yield reason to distinguish CP from RCM is that CP is curable with pericardiectomy, while RCM is generally managed medically (or with disease-modifying therapy like tafamidis for ATTR). Missing constriction means missing a surgical cure.

Step 3 management: In an ambulatory patient with unexplained right-sided HF, normal EF, and clear lungs, your first move is a TTE with tissue Doppler and respirophasic flow assessment, plus targeted history for radiation, prior surgery, and amyloid red flags.

The core problem: both constrictive pericarditis (CP) and restrictive cardiomyopathy (RCM) cause diastolic dysfunction with preserved (or near-preserved) systolic function, producing right-sided heart failure with elevated filling pressures

Constrictive pericarditis (CP): rigid, often calcified pericardium prevents ventricular filling in mid-to-late diastole; the myocardium itself is normal

Restrictive cardiomyopathy (RCM): stiff, infiltrated myocardium impairs filling; pericardium is normal

When to suspect on Step 3: a patient with right heart failure signs (JVD, ascites, edema, hepatomegaly) but clear lungs and a normal EF on echo — particularly if there is disproportionate ascites relative to peripheral edema

Presentation Patterns and Key History

— Orthopnea and PND are less prominent than in left-sided HF because pulmonary congestion is usually mild until late

— Prior cardiac surgery (CABG, valve) — now the leading US cause

— Mediastinal/chest radiation — Hodgkin lymphoma, breast cancer (often 10–20 years out)

— Recurrent or prior acute pericarditis (viral, idiopathic)

— TB exposure in immigrants from endemic regions (#1 cause worldwide)

— Connective tissue disease (RA, SLE), uremia on dialysis, malignancy

— Symptoms often develop over months to a few years

— Amyloidosis: bilateral carpal tunnel syndrome (often years prior), lumbar spinal stenosis, autonomic dysfunction (orthostasis), unexplained proteinuria, easy bruising/periorbital purpura, ATTRv family history (Black patients — V122I variant)

— Sarcoidosis: young-to-middle-aged adult, pulmonary symptoms, AV block, ventricular arrhythmias, erythema nodosum, uveitis

— Hemochromatosis: arthralgias, diabetes, bronze skin, hepatic dysfunction, family history

— Hypereosinophilia (Löffler): asthma, parasites, leukemia, eosinophilia on CBC

— Anthracycline exposure (breast CA, lymphoma survivors)

Key distinction: Ask explicitly about carpal tunnel, mediastinal radiation, and prior cardiac surgery in any unexplained right HF patient — these three questions reorient your differential between amyloid RCM and post-surgical/post-XRT CP within minutes of starting the history.

Shared presentation: insidious, progressive right-sided heart failure — fatigue, exertional dyspnea, abdominal distension, lower extremity edema, early satiety, weight gain from ascites

Constrictive pericarditis history clues:

Restrictive cardiomyopathy history clues:

Key time course distinction: CP often relates to a discrete past insult (surgery, XRT, pericarditis episode); RCM is a systemic infiltrative process with extra-cardiac findings

Physical Exam Findings and Hemodynamic Assessment

— Kussmaul sign: paradoxical rise in JVP with inspiration (normally JVP falls). Highly suggestive of constriction but also seen in RCM, severe TR, RV infarct

— Pericardial knock: early diastolic sound after S2, higher-pitched than S3, from abrupt cessation of ventricular filling against rigid pericardium

— Prominent y-descent on JVP waveform ("M or W" pattern with sharp x and y); occasionally pulsus paradoxus (less than in tamponade)

— Lungs typically clear; ascites often out of proportion to peripheral edema

— Kussmaul sign can be present

— S3 and/or S4 gallop (rather than pericardial knock)

— Signs of underlying disease: macroglossia, periorbital purpura (AL amyloid), bilateral carpal tunnel scars, hepatosplenomegaly out of proportion to HF, neuropathy, bronze skin

— Apical impulse often palpable (vs. CP, where it may be reduced or retracting)

— Both: elevated and equalized diastolic pressures (RA, RV, PA, PCWP within ~5 mm Hg); dip-and-plateau ("square root sign") in ventricular tracings

— Discriminators favoring CP: ventricular interdependence — with inspiration, RV systolic pressure rises while LV systolic pressure falls (discordance); PASP usually <55 mm Hg

— Favoring RCM: concordant RV/LV systolic pressure changes with respiration; PASP often >55 mm Hg; LVEDP typically exceeds RVEDP by >5 mm Hg

Board pearl: A pericardial knock + Kussmaul sign + clear lungs + huge ascites in a post-CABG or post-radiation patient is constrictive pericarditis until proven otherwise — go straight to advanced imaging.

Shared findings: elevated JVP, peripheral edema, ascites, hepatomegaly, hepatojugular reflux, often a pulsatile liver

Constrictive pericarditis — classic exam:

Restrictive cardiomyopathy — exam:

Hemodynamic hallmarks (right heart cath):

Diagnostic Workup — Initial Labs, ECG, CXR, Echo

— BNP/NT-proBNP: typically markedly elevated in RCM (often >400) but only mildly elevated in CP (pericardium limits wall stretch) — a useful early discriminator

— CBC (eosinophilia → Löffler), iron studies/ferritin/transferrin sat (hemochromatosis), SPEP/UPEP with serum free light chains (AL amyloid), troponin (chronically elevated in amyloid/sarcoid)

— TB workup (IGRA), HIV, ANA, RF, CRP if pericarditis suspected

— LFTs and albumin — congestive hepatopathy common in both

— CP: nonspecific — low voltage, T-wave flattening/inversion, atrial fibrillation in ~30%

— RCM: low voltage with "pseudoinfarction" pattern (poor R-wave progression) plus LVH on echo → highly suggestive of cardiac amyloidosis; conduction blocks suggest sarcoid or amyloid

— CP: pericardial calcification on lateral view (best clue, ~25% sensitivity but very specific), small heart, clear lungs, pleural effusions

— RCM: enlarged cardiac silhouette possible, pulmonary congestion, biatrial enlargement

— Both show biatrial enlargement, normal/near-normal EF, dilated IVC with reduced respiratory variation

— CP-specific echo features: respirophasic septal shift ("septal bounce"), >25% respiratory variation in mitral E inflow, expiratory hepatic vein diastolic flow reversal, medial mitral annular e' ≥ lateral ("annulus reversus") with preserved or increased medial e' (≥8 cm/s) — "annulus paradoxus"

— RCM-specific features: reduced tissue Doppler e' (<7 cm/s medial), markedly elevated E/e' ratio, biatrial enlargement out of proportion, increased wall thickness (amyloid → "speckled" or granular myocardium with apical sparing on strain — "cherry on top")

Step 3 management: Order a TTE with tissue Doppler, strain, and respirophasic Doppler as your first imaging study; pair with NT-proBNP and serum/urine light chains to triage amyloid vs constriction efficiently.

Labs (target the differential):

ECG:

CXR:

Echocardiography — first-line imaging:

Diagnostic Workup — Advanced and Confirmatory Studies

— CP findings: pericardial thickening >3–4 mm, pericardial late gadolinium enhancement (active inflammation, may predict response to anti-inflammatories), real-time cine showing septal bounce and ventricular interdependence

— RCM findings: diffuse subendocardial or transmural late gadolinium enhancement in amyloid (especially with abnormal nulling), patchy mid-myocardial LGE in sarcoid, T1/T2 mapping abnormalities, native T1 elevation in amyloid/Fabry

— Note: up to 20% of CP cases have pericardium <2 mm — normal pericardial thickness does NOT exclude CP

— Simultaneous RV and LV pressure tracings during respiration

— CP: ventricular interdependence — inspiratory increase in RV systolic pressure with decrease in LV systolic pressure (systolic area index >1.1); equalized end-diastolic pressures

— RCM: concordant respiratory variation; LVEDP – RVEDP >5 mm Hg, PASP often >55, RVEDP usually <1/3 of RV systolic pressure

— Serum/urine immunofixation + serum free light chain ratio → screens for AL

— Tc-99m pyrophosphate (PYP) scan: grade 2–3 uptake + negative light chains = ATTR amyloid (no biopsy needed)

— Endomyocardial biopsy reserved for ambiguous cases or AL confirmation (Congo red → apple-green birefringence)

Board pearl: The diagnostic flow is echo → cardiac MRI → invasive hemodynamics if still ambiguous. PYP scan with negative light chains is diagnostic for ATTR amyloid without biopsy — a recent high-yield Step 3 change.

Cardiac MRI — best noninvasive test to distinguish CP from RCM:

Cardiac CT: best for pericardial calcification and surgical planning before pericardiectomy

Right and left heart catheterization (gold-standard hemodynamics):

Amyloid-specific workup (if RCM suspected):

Sarcoid workup: FDG-PET, chest CT, ACE level (poor sensitivity), endomyocardial biopsy (low yield, patchy)

Endomyocardial biopsy: definitive for RCM when noninvasive workup ambiguous

Risk Stratification and First-Line Management Logic

— CP: pericardiectomy is curative in the right candidate

— RCM: treat the underlying infiltrative disease + supportive HF management; transplant for end-stage

— Transient constriction (10–20% of cases): recent pericarditis, active inflammation on MRI (pericardial LGE), elevated CRP → trial of NSAIDs ± colchicine ± steroids for 2–3 months before committing to surgery; many resolve

— Chronic constriction (calcified, fibrotic, no inflammation): refer for pericardiectomy — definitive treatment

— Symptomatic relief with diuretics as a bridge; avoid over-diuresis (preload-dependent)

— ATTR amyloid (wild-type or hereditary): tafamidis (transthyretin stabilizer) reduces mortality and hospitalization; emerging: patisiran, inotersen, vutrisiran (gene-silencing)

— AL amyloid: urgent hematology referral — chemotherapy (daratumumab + CyBorD), possible autologous stem cell transplant; prognosis is worst among RCMs

— Cardiac sarcoid: immunosuppression (prednisone ± methotrexate); ICD if EF ≤35%, scar burden, or syncope/VT

— Hemochromatosis: therapeutic phlebotomy (or chelation if anemic); reverses early disease

— Hypereosinophilic (Löffler): treat underlying cause; corticosteroids, hydroxyurea, imatinib (FIP1L1-PDGFRA)

— Fabry: enzyme replacement (agalsidase) or migalastat

— CP: radiation-induced, mixed constriction-restriction, severe LV dysfunction → worse pericardiectomy outcomes

— RCM: AL subtype, troponin elevation, NT-proBNP >8500, low BP, advanced NYHA class → high mortality

Step 3 management: Always ask "is there reversibility?" — transient constriction, hemochromatosis, sarcoid, and ATTR amyloid all have disease-modifying therapy that changes prognosis dramatically.

Step 1: Confirm the diagnosis and identify the etiology — management diverges sharply

Constrictive pericarditis — management triage:

Restrictive cardiomyopathy — disease-specific management:

Risk stratifiers for poor outcome:

Pharmacotherapy — First-Line Regimens

— These are preload-dependent diastolic-filling-limited states — be cautious with aggressive diuresis, vasodilators, and negative inotropes

— Avoid: standard HFrEF doses of beta-blockers, ACEi/ARB, and especially digoxin in amyloid (binds amyloid fibrils → toxicity at therapeutic levels) and calcium channel blockers in amyloid (similar binding, hypotension)

— Mainstay for volume control in both

— Start low, titrate to euvolemia; monitor BUN/Cr, electrolytes

— Add spironolactone for diuretic synergy and to limit hypokalemia

— Amiodarone is generally preferred in amyloid (avoid CCBs and beta-blockers if possible; cautious low-dose beta-blocker may be used if tolerated)

— Anticoagulation with DOAC or warfarin — amyloid patients have high LAA thrombus risk even in sinus rhythm; some experts recommend anticoagulation regardless of CHA₂DS₂-VASc in cardiac amyloid

— ATTR amyloid: tafamidis 61 mg PO daily — only oral therapy proven to reduce CV mortality and hospitalization (ATTR-ACT trial); patisiran/vutrisiran (RNAi) for hereditary

— AL amyloid: daratumumab + CyBorD (cyclophosphamide, bortezomib, dexamethasone) — hematology-driven

— Cardiac sarcoid: prednisone 30–40 mg/day taper over 6–12 months, ± methotrexate or infliximab

— Hemochromatosis: phlebotomy to ferritin <50 ng/mL

— Acute/transient constrictive pericarditis: NSAIDs (ibuprofen 600 mg TID or ASA 750 mg TID) + colchicine 0.6 mg BID × 3 months; steroids only for refractory or contraindicated NSAIDs

Board pearl: Digoxin is contraindicated in cardiac amyloidosis — a Step 3 favorite. It binds amyloid fibrils, producing toxicity at "normal" levels.

General principles in both CP and RCM:

Loop diuretics (furosemide, torsemide, bumetanide):

Rate control for atrial fibrillation (common in both, especially amyloid):

Disease-specific pharmacotherapy:

Avoid in CP: beta-blockers and CCBs that slow HR — patients depend on tachycardia for cardiac output given fixed stroke volume

Procedures and Invasive Management

— Indication: symptomatic chronic CP (NYHA III–IV), no active inflammation, failed medical trial

— Approach: radical/total pericardiectomy via median sternotomy preferred over partial — better symptom relief and lower recurrence

— Outcomes: operative mortality 6–12%; symptom relief in 70–80% of survivors

— Predictors of worse outcomes: radiation-induced CP (operative mortality up to 20%, often progresses to restriction), advanced NYHA class, renal dysfunction, prior cardiac surgery, mixed constriction-restriction physiology

— Timing: don't delay in well-selected patients — late referral after severe cachexia, hepatic dysfunction, or low cardiac output worsens outcomes

— Optimize volume status with diuretics

— Treat anemia, nutrition, hepatic congestion

— If active inflammation (elevated CRP, pericardial LGE on MRI): trial anti-inflammatories first

— No surgical cure for the myocardial process

— Pacemaker for AV block (especially sarcoid, amyloid)

— ICD: secondary prevention universal; primary prevention in cardiac sarcoid per HRS criteria (EF ≤35%, syncope, LGE with significant scar, inducible VT)

— Heart transplantation: option for end-stage RCM, especially AL amyloid in remission after chemo, ATTR amyloid (often combined with liver transplant if hereditary); careful candidate selection

— Left ventricular assist devices (LVADs) generally poor option in RCM due to small LV cavity and inadequate filling

CCS pearl: For a post-CABG patient with progressive ascites, JVD, and a pericardial knock, your CCS order set should include: TTE, cardiac MRI, RHC with simultaneous LV pressures, CT surgery consult for pericardiectomy, and furosemide IV while inpatient. Don't anticoagulate empirically; don't load with metoprolol.

Pericardiectomy — definitive treatment for chronic constrictive pericarditis:

Bridging therapy before surgery:

Restrictive cardiomyopathy — invasive options:

Endomyocardial biopsy: therapeutic role in eosinophilic endomyocardial fibrosis (endocardectomy)

Special Populations — Elderly and Renal/Hepatic Impairment

— Wild-type ATTR amyloidosis (ATTRwt) is now recognized as a common, underdiagnosed cause of HFpEF in elderly men — prevalence up to 13% of HFpEF hospitalizations in patients >60

— Suspect when: HFpEF + bilateral carpal tunnel history, lumbar stenosis, discordant LV wall thickness with low ECG voltage, intolerance to standard HF therapy (hypotension on small doses of ACEi/beta-blocker)

— Screen with PYP scan + light chains — non-invasive diagnosis is now standard

— Tafamidis is approved regardless of age; cost-effectiveness debated but clearly indicated in symptomatic ATTRwt

— Pericardiectomy in elderly CP patients: higher operative mortality; carefully assess frailty, comorbidities, life expectancy

— Diuretic resistance common — may need IV loop + thiazide or metolazone combination ("sequential nephron blockade")

— Avoid NSAIDs for transient constriction if CKD stage 3+ — use colchicine + steroids instead

— Colchicine dose reduce for CrCl <30 (0.3 mg daily or alternate days); avoid with strong CYP3A4 inhibitors

— Cardiorenal syndrome common — gentle diuresis, watch for rising Cr (mild rise of 0.3 acceptable if symptoms improving)

— Amyloidosis with renal involvement (AL): nephrotic-range proteinuria is a classic clue

— Congestive hepatopathy ("cardiac cirrhosis") is common in chronic CP and advanced RCM

— Elevated INR, low albumin, ascites with high SAAG (>1.1) mimicking cirrhosis

— Distinguish from primary liver disease: JVD is the giveaway — cirrhosis does not elevate JVP

— Hepatic decompensation predicts poor pericardiectomy outcome — operate before severe hepatic dysfunction develops

Key distinction: In elderly HFpEF patients failing standard therapy, think amyloid first, not "diastolic dysfunction." Tafamidis is now a Step 3–era standard of care.

Elderly patients (>70):

Renal impairment:

Hepatic impairment:

Special Populations — Pregnancy, Pediatrics, and Other Subgroups

— Both CP and RCM are high-risk pregnancies (mWHO class III–IV) due to inability to augment cardiac output for the 30–50% physiologic plasma volume expansion

— Pre-pregnancy counseling with maternal-fetal medicine + cardio-obstetrics team is essential

— Patients may decompensate in 2nd trimester or peripartum; ICU-level care often needed

— Medication adjustments: avoid ACEi/ARB, spironolactone, amiodarone (controversial), warfarin in 1st trimester; furosemide is acceptable when clinically necessary

— Tafamidis — limited data, generally avoided in pregnancy

— Mode of delivery: vaginal preferred with assisted second stage; epidural with careful preload management

— RCM in children is rare but devastating — median survival 1–2 years without transplant

— Causes: idiopathic, familial (sarcomeric mutations — troponin I, MYH7), Friedreich ataxia, endomyocardial fibrosis (tropical), storage diseases (Gaucher, Hurler)

— Pediatric CP: post-viral, post-surgical (congenital heart repair), TB in endemic regions

— Early referral for transplant evaluation is standard for pediatric RCM

— V122I variant in 3–4% of Black/African American population — predominantly cardiac presentation in 6th–7th decade

— V30M variant — neuropathy-predominant (Portuguese, Swedish kindreds)

— Genetic counseling and cascade family screening indicated when ATTRv diagnosed

— Can develop mixed constrictive-restrictive physiology (pericardial + myocardial fibrosis)

— Worse surgical outcomes; valvular and coronary disease often coexist

Board pearl: A Black patient >60 with unexplained HFpEF and a family history of "heart trouble" — screen for ATTRv (V122I) with PYP scan and genetic testing.

Pregnancy:

Pediatrics:

Hereditary ATTR amyloid (ATTRv):

Post-radiation patients (breast CA, Hodgkin):

Complications and Adverse Outcomes

— Atrial fibrillation (30–50%): poorly tolerated due to loss of atrial kick into a stiff/restricted ventricle; stroke risk elevated — anticoagulate per CHA₂DS₂-VASc (lower threshold in amyloid given LAA stasis even in sinus rhythm)

— Cardiac cachexia and protein-losing enteropathy from chronic venous congestion

— Cardiac cirrhosis from chronic hepatic congestion → portal hypertension, varices, hepatocellular dysfunction

— Renal dysfunction (cardiorenal syndrome type 2) — chronic venous congestion + low forward output

— Recurrent hospitalizations for volume overload — a key quality metric in Step 3 health-systems framing

— Progression despite anti-inflammatory therapy in chronic disease

— Post-pericardiectomy low cardiac output syndrome — especially in radiation-induced and severe cases

— Persistent symptoms after pericardiectomy in 20–30% — often due to occult restrictive component or incomplete pericardial resection

— Recurrent pericarditis if underlying inflammatory etiology not controlled

— Heart block / conduction system disease — particularly cardiac sarcoid (AV block, VT, sudden death) and amyloid

— Sudden cardiac death from VT (sarcoid > amyloid)

— Intracardiac thrombus (especially AL amyloid, eosinophilic endomyocardial fibrosis) → embolic stroke

— Orthostatic hypotension from autonomic neuropathy in amyloid — limits diuretic and antihypertensive tolerance

— Progressive multi-organ amyloid deposition in AL: nephrotic syndrome, neuropathy, GI dysmotility, easy bruising

Step 3 management: A cardiac sarcoid patient with syncope or LGE on MRI warrants ICD evaluation even with preserved EF — Step 3 increasingly tests these guideline updates.

Common to both CP and RCM:

Constriction-specific complications:

Restriction-specific complications:

Late post-radiation cardiac syndrome: combination of CP + valvular disease + CAD + RCM — challenging to manage

When to Escalate — ICU, Consult, and Triage

— New or worsening HF symptoms with hypotension, hyponatremia, rising Cr

— Suspicion of new constrictive pericarditis with cardiogenic compromise

— Symptomatic atrial fibrillation with rapid ventricular response in known CP/RCM

— New syncope in a known sarcoid or amyloid patient — admit for telemetry and EP workup

— Cardiogenic shock from low-output state — these patients tolerate hypotension and vasodilators poorly

— Hemodynamically unstable arrhythmias

— Post-pericardiectomy patients routinely require ICU for 24–72 hours (low cardiac output syndrome risk)

— Acute fulminant amyloid or end-stage AL with multi-organ failure

— Cardiology (always): for diagnostic confirmation, hemodynamics

— Cardiothoracic surgery: when chronic CP confirmed and pericardiectomy considered

— Hematology/Oncology: urgent for AL amyloid (time-sensitive — chemo within days to weeks)

— Pulmonology / Rheumatology: for sarcoidosis

— Genetics: for ATTRv, Fabry, hemochromatosis (cascade testing)

— Hepatology: for cardiac cirrhosis vs primary liver disease

— Heart failure / transplant team: advanced RCM, refractory CP

1. Vitals, telemetry, daily weights, strict I/O

2. IV furosemide titrated to euvolemia

3. NT-proBNP, troponin, CMP, LFTs, CBC, INR, BNP

4. ECG, CXR, TTE with Doppler

5. Cardiac MRI with contrast

6. Cardiology consult, CT surgery consult once CP confirmed

7. Right and left heart catheterization for hemodynamics

CCS pearl: Don't reflexively load with metoprolol or lisinopril in a CP/RCM admission for HF — these patients depend on heart rate and preload. Diurese gently, image quickly, consult early.

Triggers for inpatient admission:

ICU-level care:

Consultations to obtain:

CCS-style order priorities for new suspected constriction with right HF:

Key Differentials — Same-Category (Right HF/Diastolic) Causes

— Shares elevated JVP, dyspnea, hypotension; but pulsus paradoxus is prominent, y-descent is blunted (vs. prominent in CP), Kussmaul sign typically absent

— Echo: pericardial effusion with RA/RV collapse, respiratory variation across valves

— Acute vs. CP's chronic course

— RV failure, JVD with giant CV wave, pulsatile liver, ascites, edema

— Echo confirms; coexists with CP/RCM commonly

— Acute presentation, ECG changes (ST elevation in V4R), Kussmaul sign present

— Preload-dependent — give fluids, avoid nitrates

— Elevated JVP, peripheral edema, but echo shows markedly elevated PASP (often >55–60), dilated RV, often septal flattening

— PASP >55 favors RCM or PH over CP (CP usually keeps PASP modest)

— Hypertension, age, obesity, diabetes; pulmonary congestion more prominent

— Echo: LVH, elevated E/e', but no septal bounce or annulus reversus

— Many "HFpEF" patients are actually undiagnosed cardiac amyloid (~10–15%)

— VQ scan abnormal, history of PE, RV strain

— Common in post-radiation patients; pericardiectomy alone gives partial relief

— Bounding pulses, warm extremities, wide pulse pressure — opposite of low-output CP/RCM

Key distinction: Within the right-HF differential, the trio that mimic CP most closely are tamponade, severe TR, and RV infarct — all share Kussmaul-like signs but each has unique echo/ECG/timing features.

Cardiac tamponade:

Severe tricuspid regurgitation:

Right ventricular infarction:

Pulmonary hypertension / cor pulmonale:

HFpEF (garden-variety diastolic dysfunction):

Chronic thromboembolic pulmonary hypertension (CTEPH):

Mixed constrictive-restrictive physiology:

High-output failure (thyrotoxicosis, AVF, beriberi, anemia):

Key Differentials — Other-Category Causes

— Ascites, edema, hepatomegaly, low albumin, elevated INR — looks like cardiac cirrhosis

— Key discriminator: JVP is normal or low in cirrhosis; markedly elevated in CP/RCM

— Hepatic vein Doppler differs; cardiac biomarkers (NT-proBNP) typically not elevated in pure cirrhosis

— Anasarca, ascites, edema; but no JVD elevation, prominent proteinuria, hypoalbuminemia

— Note: AL amyloid can present with both nephrotic syndrome and restrictive cardiomyopathy simultaneously — check light chains

— Edema, low albumin without proteinuria; can occur secondary to chronic venous congestion in CP/RCM (Fontan-like physiology)

— Edema, pericardial effusion, fatigue; check TSH

— Bilateral leg edema without JVD, ascites, or systemic signs

— Localized or drug-related, no congestion physiology

— Anthracyclines, trastuzumab → cardiotoxicity (often dilated, but can be restrictive)

— Methysergide, ergotamine, serotonin agents → carcinoid-like fibrosis

— Carcinoid heart disease (right-sided valvular fibrosis from serotonin) → similar right HF picture; flushing, diarrhea, elevated 5-HIAA

— Sub-Saharan Africa, India; eosinophilic; right or biventricular involvement

— Young patients with restrictive physiology and family history; echo wall thickness may be normal

Board pearl: When a patient looks like cirrhosis but has elevated JVD or a history of cardiac surgery/radiation — think constrictive pericarditis masquerading as liver disease. Hepatologists routinely miss this; you shouldn't on Step 3.

Cirrhosis (primary hepatic):

Nephrotic syndrome:

Protein-losing enteropathy:

Hypothyroidism / myxedema:

Chronic venous insufficiency:

Lymphedema, idiopathic edema, calcium channel blocker–induced edema:

Drug-induced:

Endomyocardial fibrosis (tropical RCM):

Familial / sarcomeric RCM:

Secondary Prevention, Discharge Meds, and Long-Term Plan

— Continue diuretics (often furosemide + spironolactone) for weeks to months as hemodynamics remodel; many patients can wean off entirely as venous congestion resolves

— Colchicine 0.6 mg BID for 3–6 months post-op if inflammatory etiology, to prevent recurrence

— Cardiac rehab referral

— Surveillance for recurrence: clinic at 2 weeks, 1 month, 3 months, then every 6–12 months; serial echo at 3 and 12 months

— Address remaining etiology — TB therapy, dialysis optimization, treatment of CTD

— ATTR amyloid: lifelong tafamidis + diuretics + anticoagulation if AF or LA thrombus risk

— AL amyloid: ongoing hematology follow-up — monitor light chains, durable hematologic response key to cardiac stabilization

— Sarcoidosis: taper steroids over 6–12 months, monitor with FDG-PET, continue immunosuppression as needed; ICD per HRS criteria

— Hemochromatosis: maintenance phlebotomy every 2–4 months, ferritin <50, transferrin sat <50%

— Cardiac rehab referral when ambulatory

— Sodium restriction (<2 g/day), fluid restriction (<2 L/day) if hyponatremic

— Daily weights; alert provider if >2 lb/day or 5 lb/week

— Pneumococcal, influenza, COVID, RSV vaccinations

— Avoid NSAIDs (worsen fluid retention, except in transient constriction context)

— Medication reconciliation, especially after specialist visits

Step 3 management: For a discharged CP patient post-pericardiectomy, schedule cardiology follow-up within 1–2 weeks with a repeat TTE at 3 months — early identification of incomplete resection or restrictive component is critical.

Post-pericardiectomy discharge plan:

Restrictive cardiomyopathy long-term plan:

Universal HF discharge bundle:

Family screening: ATTRv, Fabry, hemochromatosis, familial RCM — cascade genetic testing offered to first-degree relatives

Follow-Up, Monitoring Parameters, and Counseling

— Stable chronic CP not surgical: cardiology every 3–6 months

— Post-pericardiectomy: 2 weeks → 1 month → 3 months → 6 months → annually

— RCM: every 3–6 months depending on severity; more frequent during therapy initiation

— AL amyloid: monthly during chemotherapy with hematology, cardiology every 3 months

— Symptoms: NYHA class, exercise tolerance, weight trends, edema

— Labs: BMP (electrolytes, renal function) at each diuretic adjustment; NT-proBNP trend; LFTs for congestion; for amyloid → serum free light chains (AL), troponin trend

— Imaging: TTE every 6–12 months or with clinical change; cardiac MRI if disease activity uncertain (sarcoid, amyloid response)

— Disease-specific: ferritin/transferrin sat (hemochromatosis), FDG-PET (sarcoid activity), 24-hr urine protein (AL amyloid)

— Holter / loop recorder: in sarcoid for arrhythmia surveillance

— Improves functional status and quality of life

— Particularly beneficial after pericardiectomy

— Tailored low-intensity programs for amyloid (autonomic dysfunction risk)

— Salt and fluid restriction, daily weights, sick-day rules

— Genetic counseling and cascade screening for ATTRv, Fabry, hemochromatosis, familial RCM

— Prognosis discussions — AL amyloid has high early mortality; honest goals-of-care conversations critical

— Advance care planning for advanced RCM

— Driving restrictions after syncope or ICD shocks (per local DMV rules)

— Pregnancy counseling for women of reproductive age

Board pearl: Falling NT-proBNP and troponin in AL amyloid after chemotherapy is a strong predictor of cardiac improvement and survival — track them serially.

Outpatient cadence:

Monitoring parameters:

Cardiac rehabilitation:

Counseling points:

Value-based care framing: these patients are high readmission risk — coordinated multidisciplinary clinics (HF, amyloid centers) reduce hospitalizations

Ethical, Legal, and Patient Safety Considerations

— Discuss 6–12% operative mortality, especially in radiation-induced or advanced disease

— Discuss 20–30% chance of persistent symptoms post-op

— Document patient-specific risk factors (frailty, hepatic dysfunction, prior cardiac surgery, radiation exposure)

— For high-risk candidates, explicit goals-of-care discussion: is the goal symptom control, life prolongation, or both?

— AL amyloid carries the worst prognosis among RCMs — median survival without treatment ~6 months in advanced cardiac involvement

— Frank discussion of prognosis, chemotherapy intensity vs. palliation; early palliative care consultation is appropriate even at diagnosis

— Document advance directives, code status, healthcare proxy

— ATTRv, Fabry, hemochromatosis, familial RCM raise duty-to-warn considerations for at-risk relatives

— In the US, the patient is the disclosing party — clinician's role is to encourage and facilitate cascade testing; direct contact of relatives without consent generally not permitted (varies by state)

— Discharge after diuresis is a high-risk window for rehospitalization

— Specific safety items: medication reconciliation (avoid restarting BBs/ACEi inappropriately in amyloid), weight monitoring instructions, clear plan for diuretic titration

— Avoid digoxin and verapamil/diltiazem in amyloid — flag in chart and EMR allergy/intolerance list

— Communicate clearly with PCP about diagnosis and monitoring plan

— Cardiac sarcoid patients receiving ICDs face driving restrictions after shocks per state law and AHA/HRS recommendations

— Fall risk assessment (orthostasis common in amyloid)

— Anticoagulation safety (bleeding risk in amyloid coagulopathy)

— Vaccination status before immunosuppression for sarcoid

Step 3 management: Mandatory chart flag and EMR allergy entry for "no digoxin, no verapamil/diltiazem" in any patient with confirmed cardiac amyloidosis — a transition-of-care safety priority tested on Step 3.

Informed consent for pericardiectomy:

Goals of care in AL amyloid:

Genetic disclosure ethics:

Transition-of-care risks:

Driving and ICDs:

Patient safety bundle:

High-Yield Associations and Rapid-Fire Facts

— Post-cardiac surgery (#1 in US)

— Mediastinal radiation (Hodgkin, breast cancer)

— TB (#1 worldwide)

— Idiopathic/viral recurrent pericarditis

— Uremia, RA, SLE, malignancy

— Bilateral carpal tunnel syndrome (often years prior)

— Lumbar spinal stenosis

— Macroglossia + periorbital purpura = pathognomonic for AL

— Low-voltage ECG + LVH on echo = "voltage-mass discordance"

— Apical sparing ("cherry on top") on strain imaging

— V122I mutation in 3–4% of Black Americans

— Intolerance to standard HF therapy (hypotension)

— Young/middle-aged patient with AV block or VT

— Patchy mid-myocardial LGE on cardiac MRI

— FDG-PET uptake

— CP: PASP <55, ventricular interdependence (discordance), equalized diastolic pressures

— RCM: PASP often >55, concordant respiration, LVEDP > RVEDP by >5

— CP: septal bounce, annulus reversus (medial e' ≥ lateral), preserved/increased medial e' (>8)

— RCM: reduced e' (<7), elevated E/e', apical sparing strain (amyloid)

Board pearl: Memorize this triad — clear lungs + huge ascites + elevated JVD = constrictive pericarditis or restrictive cardiomyopathy. From there, history + echo + MRI sort it out.

Constrictive pericarditis classic associations:

Cardiac amyloidosis red flags:

Cardiac sarcoidosis triggers:

Hemochromatosis triad: cirrhosis + diabetes + bronze skin + cardiomyopathy (often arrhythmia)

Löffler endocarditis: eosinophilia + biventricular RCM + mural thrombi

Hemodynamic discriminators:

Echo discriminators:

NT-proBNP: markedly elevated in RCM, mildly elevated in CP

Drugs to avoid in amyloid: digoxin, verapamil, diltiazem, high-dose beta-blockers

Tafamidis = transthyretin stabilizer for ATTR (only oral agent with mortality benefit)

PYP scan + negative light chains = ATTR amyloid (no biopsy needed)

Pericardial calcification on lateral CXR → highly specific for CP

Pericardiectomy = curative for chronic CP

Board Question Stem Patterns

— Answer: Constrictive pericarditis → next step: cardiac MRI or right/left heart cath; definitive: pericardiectomy

— Answer: Cardiac amyloidosis (likely ATTRwt) → next step: serum free light chains + PYP scan; treatment: tafamidis

— Answer: AL amyloidosis → urgent hematology referral, SPEP/UPEP, free light chains, bone marrow biopsy

— Answer: Cardiac sarcoidosis → cardiac MRI/FDG-PET, corticosteroids, ICD evaluation

— Answer: Tuberculous constrictive pericarditis → pericardiectomy + anti-TB therapy

— Higher operative risk; counsel accordingly

— Ventricular interdependence = CP (discordance)

Step 3 management: When the stem mentions carpal tunnel + HFpEF + low ECG voltage + elderly man, the answer is cardiac amyloidosis, and the next step is PYP scan with free light chains.

Classic CP stem: "A 62-year-old man with a history of CABG 8 years ago presents with progressive abdominal distension, leg swelling, and fatigue. JVP is 14 cm, lungs are clear, abdomen is distended with shifting dullness. Auscultation reveals an early diastolic sound after S2. Echo shows normal EF with respirophasic septal motion."

Cardiac amyloidosis stem: "A 74-year-old man presents with worsening dyspnea. He had bilateral carpal tunnel release 8 years ago. BP 92/60. ECG shows low voltage. Echo shows LV wall thickness 16 mm with EF 50%. He becomes hypotensive on small-dose lisinopril."

AL amyloidosis stem: "A 58-year-old woman with periorbital bruising, macroglossia, and nephrotic-range proteinuria has echo showing thickened LV with reduced strain except at the apex."

Sarcoid stem: "A 38-year-old Black woman with hilar lymphadenopathy and uveitis presents with syncope. ECG shows complete heart block."

TB constriction stem: "An immigrant from India with prior TB has elevated JVP, ascites, and pericardial calcification on CXR."

Radiation stem: "Hodgkin survivor 20 years out develops right HF; mixed CP-RCM physiology."

CP vs RCM discriminator stem: "On simultaneous RV/LV cath, with inspiration RV systolic pressure rises while LV systolic pressure falls."

Digoxin toxicity in amyloid: elderly patient with HFpEF, normal digoxin level, but symptoms of toxicity — recognize amyloid–digoxin binding

One-Line Recap

Constrictive pericarditis and restrictive cardiomyopathy both cause right-sided heart failure with elevated, equalized diastolic pressures and clear lungs — distinguishing them matters because constriction is surgically curable with pericardiectomy, while restriction requires disease-specific therapy (tafamidis for ATTR, chemotherapy for AL, steroids for sarcoid, phlebotomy for hemochromatosis), and the diagnostic pathway runs echo → cardiac MRI → invasive hemodynamics with attention to ventricular interdependence, septal bounce, annulus reversus, NT-proBNP magnitude, and amyloid red flags.

CP clues: prior cardiac surgery, mediastinal radiation, TB exposure, pericardial knock, pericardial calcification on CXR, septal bounce + annulus reversus on echo, ventricular interdependence on cath, mildly elevated BNP

RCM clues: bilateral carpal tunnel + macroglossia + low-voltage ECG with LVH (amyloid); AV block + sarcoidosis features (cardiac sarcoid); bronze skin + diabetes (hemochromatosis); markedly elevated NT-proBNP, reduced tissue Doppler e', apical-sparing strain pattern

Treatment divergence: pericardiectomy cures chronic CP (try anti-inflammatories first if transient/active inflammation); tafamidis for ATTR amyloid; urgent hematology + CyBorD-based chemotherapy for AL amyloid; steroids ± ICD for cardiac sarcoid; phlebotomy for hemochromatosis

Step 3 safety priorities: avoid digoxin, verapamil, and diltiazem in cardiac amyloidosis; diurese gently in both conditions; cascade genetic testing for ATTRv, Fabry, hemochromatosis, and familial RCM; coordinate transition of care with cardiology, hematology, and primary care to prevent readmission