Heart Failure

• Clinical syndrome with signs/symptoms consistent with impaired cardiac function ± cardiomyopathy 

• Echocardiography: TTE for all new presentations; obtain thereafter only if concern for clinical/functional change 

  • HFrEF (EF ≤40%), HFmrEF (“mid-range” EF 41 49%), HFpEF (EF ≥50%) 

• Ischemic workup: FHx, ECG, TnT, noninvasive or invasive cardiac testing 

• Non-ischemic workup: FHx, genetic testing, med hx, alcohol use history, lipid panel, TSH, A1c, urine hCG, iron studies, HIV, SPEP/SFLC w/ UFLC

  • Consider: cardiac MRI, ANA, T. cruzi serologies, viral panel, antimyosin Ab, tox screen, thiamine/carnitine/selenium, genetic testing, ARVC, sarcoid, PYP scan, cardiac masses; endomyocardial bx (if serologic testing neg, new onset <6mo unexplained HF, unexplained HF <2wks and HDUS, major arrhythmias) to r/o myocarditis 

• Consider high-output etiologies: anemia, thyroid dysfxn, liver failure, Paget’s, systemic infection, AV shunts 

Dilated Cardiomyopathy 

• Etiologies: ischemic (most common cause, 50-75%), HTN/LVH, valvular (e.g. MR), myocarditis, stress-induced (Takotsubo), 

tachyarrhythmia, infiltrative (as below), CTD, ARVC, LVNC (left ventricular non-compaction), HIV, cocaine/methamphetamines, EtOH, 

chemotherapy, nutritional deficiency, cirrhosis, sepsis, peripartum, idiopathic/genetic 

• Condition Etiology and Management 

  • Alcohol-induced 

    • Mechanism: >80g/d EtOH over >5y (toxic to myocytes via O2 free radicals + defects in protein synthesis) 

    • Tx: abstinence + HF therapy 

    • Prognosis: better/equivalent to idiopathic CM if <20g/d (<2 drinks) or abstinence, worse w/ continued EtOH use 

  • Stress-induced  (Takotsubo) 

    • JACC. 2018;72:1955

    • Mechanisms: catecholamine surge from physical/emotional stress, coronary artery spasm, microvascular ACS 

    • Presentation: may present like ACS with CP (most common), SOB, shock, syncope. If in shock, urgent TTE to  assess for LVOT obstruction 

    • Dx (need all 4): 

      • 1) transient dysfxn of LV mid-segments, WMAs extending beyond a single coronary distribution; 

      • 2)  rule out ACS/obstructive CAD (via cath); 

      • 3) new ECG  (STE or TWI) OR TnT; 

      • 4) absence of pheo or myocarditis 

    • Tx/Prognosis: remove stressor, ACEi (may improve survival), BB; most recover LV function in 1-4w

Hypertrophic Cardiomyopathy (HCM) 

JACC 2020:76:e159

• Characteristics: LV and/or RV hypertrophy of various morphologies ± LVOT dynamic obstruction (HOCM), diastolic dysfxn, ischemia, MR; risk of arrhythmia/SCD

• Exam: SEM at LLSB/apex that augments with Valsalva or on standing (due to preload); S2 paradox split, S4 

• Dx: ECG (prominent voltages w/ depolarization abnormalities, large abnormal Q waves in inferior/lateral leads, LAD, giant negative T waves in V2-V4 (apical HCM variant aka “Yamaguchi syndrome”)), TTE (unexplained LVH >15mm, SAM of MV, outflow tract gradient +/- provocative testing), cMRI (late gadolinium enhancement [LGE] = fibrosis) 

• Clinical genetic testing (mutation in ~70%) helpful for family screening; not useful for dx or risk stratification 

• Tx: avoid volume depletion or high dose vasodilators (may worsen obstruction); phenylephrine is pressor of choice if no response to IVF bolus for HoTN (afterload, stents open LVOT). Activity restriction, meds (BB > verapamil), septal ablation or surgical myectomy for medically refractory sx, ICD (for high SCD risk, see below) 

• Risk factors for SCD/VT: prior VT/SCD/unexplained syncope; FHx of SCD in 1° relative; massive LVH (>30mm); NSVT on Holter; abnormal BP response to exercise; burden of LGE on cMRI; h/o suspected cardiac syncope; EF<50% 

JACC 2010;55:1769 

NEJM 2018; 379:1007

• Admission orders: tele, 2g Na restricted diet, daily standing weights, strict I/Os, DVT ppx

• Avoid: CCB (esp. non-dihydropyridines), NSAIDs, flecainide

• Check NT-proBNP (& weight) on admission and at discharge

  • ADHF unlikely if NT-proBNP <300 (NPV 98%), likely if >450 (>900 if age >50) (Am J Cardiol 2005;95;948)

  • Difficult to interpret in CKD/dialysis. May be falsely low in obesity, HFpEF

• Screen for & treat iron deficiency in all HF pts independent of Hgb (JACC HF 2019;7:36)

  • Dx: ferritin <100 or ferritin <300 + TSat <20% (JACC 2017;70:776); though some evidence that TSat ≤19.8% or serum iron ≤13mol/L most predictive & ferritin may be less useful (Circ Heart Fail 2018;11:e004519)

  • Tx: replete with IV iron (JACC 2018;71:782) to sx, functional capacity, QOL (FAIR-HF, NEJM 2009;361:2436); PO ineffective in HF (JAMA 2017;317:1958)

• Identify hemodynamic profile & triage accordingly (JACC 2019;74:1966)

  • Warm vs Cold: adequate vs inadequate tissue perfusion (AMS, lactate, cool extremities, narrow PP)

  • Dry vs Wet: presence vs absence of congestion (JVD, crackles, pleural effusions, ascites, LE edema, interstitial/alveolar edema on CXR)

  • Eval for signs of pulmonary congestion on exam. Pulm edema may be absent on CXR in chronic HF due to lymphatic compensation (Chest 2004;125:669)

  • ~80% of decomp HFrEF and nearly all decomp HFpEF pts will be warm and wet

• History

  • Identify precipitants: dietary/med non-adherence (~40%), new ischemia/infarction, uncontrolled HTN, arrhythmia, inadequate diuretic dose, meds (NSAIDs, steroids, CCB, TZDs, anthracyclines), acute infection (URI, PNA, UTI), AKI, PE, toxins (EtOH, cocaine), new/worsening valve disease, myocarditis

  • Cardiac/HF history (last EF, dry weight, prior ischemic w/u, prior TTE, cardiologist); accurate PAML; social history

• Early/Acute Management:

  • Diuresis: CVP, PCWP to optimize Starling curve mechanics & relieve sx (NEJM 2017;377:1964; JACC 2020;75:1178)

    • Initial tx: IV loop diuretics (furosemide, torsemide, bumetanide), start with 2-2.5x home dose (IV/PO). No difference between continuous gtt vs bolus dosing (NEJM 2011;364:797); furosemide & torsemide have equivalent outcomes (JAMA 2023;329:214-223). See Advanced Diuresis for conversions

    • Refractory diuresis: metolazone 2.5-5mg (or chlorothiazide 500mg IV) administered 30min before loop diuretic. May need RHC to clarify hemodynamics or inotropes to augment diuresis. Acetazolamide (500mg daily) may also augment successful decongestion (NEJM 2022;387:1185). Step-up pharmacologic therapy superior to RRT in the setting of cardiorenal syndrome (NEJM 2012;367:2296)

    • Worsening renal function: occurs in ~23% of pts treated for ADHF. Mild-mod “Cr bumps” are likely benign hemodynamic changes, should not necessarily preclude further diuresis if pt still congested (Circ 2018;137:2016)

    • Endpoints: target resolution of signs/symptoms of congestion. Daily weights & hemoconcentration are useful adjuncts

  • If acute pulmonary edema, NIPPV may improve mortality and reduce need for intubation (Annals 2010;152:590)

  • Vasodilators: arterial/venous dilation can relieve symptoms by afterload, PCWP and SV. Can acclerate early sx relief. 

  • Consider esp. in severe HTN, acute MR, acute AR

    • Floor: isosorbide dinitrate, hydralazine, nitropaste, captopril; SDU/CCU: TNG, nitroprusside

  • Guideline-Directed Medical Therapy (GDMT): if not in cardiogenic shock or new AKI, continue ACEi/ARB/ARNi and βB during ADHF (but do not newly initiate βB) (EHJ 2009;30:2186)

• Pre-Discharge Optimization: document d/c weight & NT-proBNP, appt in HF Transitions Clinic if pt has MGH cardiologist

  • HFrEF (EF ≤40%) GDMT, including HFrecEF (recovered EF):

    • Beta blockers: initiate, uptitrate evidence-based βB (carvedilol, metoprolol succ., bisoprolol) (COPERNICUS; MERIT-HF). Caution if recently weaned from inotropes

    • RAAS inhibitors: if renal fxn stable, initiate/titrate ARNI (sacubitril/valsartan) (PARADIGM-HF; PIONEER-HF), second line ACEi/ARB (CONSENSUS; CHARM). Switch to ARNI from ACEi/ARB if no contraindications/cost concerns, 36h washout period for ACEi prior to starting ARNI.

      • Guidance for GDMT in advanced CKD: JACC HF 2019;7:371

    • Mineralocorticoid receptor antagonist: initiate spironolactone or eplerenone if CrCl>30 (EMPHASIS-HF; RALES). Watch for rebound hyperK after de-escalation of diuretics (check K, Cr within 72h of discharge)

    • SGLT2i (dapaglifozin, empagliflozin): further reduce CV mortality & HF admissions regardless of DM (DAPA-HF; EMPEROR-Reduced; Lancet 2020;396:819) (see Outpatient Type 2 Diabetes) 

  • Diuretic plan: determine maintenance diuretic dose and provide specific instructions for taking additional rescue doses. Observe on maintenance dose and decide if needs K replacement

  • HFmrEF (EF 40-49%): treat with diuretics & consider adding GDMT agents for HFrEF (Curr Heart Fail Rep 2020;17:1)

  • HFpEF (EF ≥50%): prevent volume overload, treat with diuretics, treat comorbidities (DM, HTN, AF)

    • SGLT2i only agent found to CV death/hospitalizations in pt with EF ≥40% (EMPEROR-Preserved)

    • Consider spironolactone if normal renal fxn/K, CV death/admits in N/S Am. sites in TOPCAT (Circ 2015;131:34)

    • No proven benefit to BB (EHJ 2018;39:26), ACEi (PEP-CHF), ARNi (PARAGON-HF), ARB (CHARM-Preserved)

  • ICD indicated if: ischemic CM w/ EF ≤30 or ≤35% w/ NYHA II-III; CRT if: EF ≤35% & prolonged QRS ± LBBB & some w/ EF ≤50% (see Cardiac Devices: PPM/ICD & guidelines for specifics: JACC 2013;61:e6; EHJ 2016;37:2129)

(JACC 2021;77:772, Circ 2022;145:895) 

Not comprehensive; assumes EF ≤ 40% (HFrEF), co-management by cardiology and/or heart failure specialist. 

• At each visit: assess med/dietary/lifestyle compliance, document weight, detailed volume exam, assess symptoms and classify ACC Stage/NYHA Class, review current GDMT, review ambulatory BP / HR measurements for GDMT titration targets

• Lab monitoring: BMP/Mg and 1-2 weeks following uptitration of ACE-i/ARB/ARNi; spironolactone requires close monitoring

• Goal: toleration of target doses of ACE/ARB/ARNI, -blocker, aldosterone antagonist, and SGLT-2 inhibitor without symptomatic hypotension or metabolic disarray. For  blockade, target resting HR < 70 without postural hypotension or unacceptable SE.

• Principles: start GDMT immediately on dx, often “quad therapy” at lower than target dosing is preferred over maxing out a single agent

• Diuretic: see Advanced Diuresis. Diuretic needs may change when SGLT-2i is added due to mild diuretic effect.

• Avoid: NSAIDs, CCBs, flecainide, steroids, OTC decongestants (e.g. Sudafed), Bactrim

• Special considerations/scenarios:

  • Ivabradine: add if sinus rhythm & resting HR > 70 on max blockade. Start at 2.5 mg – 5 mg qd

  • Hydralazine/isosorbide dinitrate: add if SBP persistently > 130 on target GDMT; especially beneficial in AA patients

  • Symptomatic hypotension on GDMT: consider over-diuresis or other offending prior to reducing GDMT

• Adjuvant care/ambulatory referrals to consider: cardiac rehab (often under-prescribed); nutrition consultation for assistance with dietary adherence; patient-centered education is critical to enhance adherence

• Definition: HoTN (SBP<90 for 30min or pressor req) + hypoperfusion (cold extremities, oliguria, lactate) + hemodynamics (CI <2.2, PCWP >15) (EHJ 2019;40:2671)

• Etiology: acute MI ± mechanical complications, end-stage heart failure, acute myocarditis, acute MR/AR, myocardial contusion

• Evaluation: ECG, troponin to r/o acute MI. TTE to exclude tamponade/mechanical lesions/contraindications to MCS

• Monitoring: A-line, consider PA catheter for inotropes/pressors and MVO2 monitoring

• Immediate Management:

  • If c/f acute MI, activate cath lab for PCI (only intervention proven to improve outcomes in cardiogenic shock) (NEJM 1999;341:625)

  • Consider early SHOCK evaluation. Escalating inotropes/pressors exacerbate myocardial supply/demand imbalance and are associated with poor outcomes. Emerging evidence supports early initiation of MCS (Cath Cardio Interv 2019;93:1173)

  • Stabilize MAP with norepinephrine PRN prior to obtaining PA catheter to guide tailored therapy

• Tailored Therapy: uses invasive hemodynamic monitoring (i.e., PAC) to guide medical therapy

• Goals: tissue perfusion (CO, MAP), decongestion (CVP, PCWP), ventricular unloading (minimize myocardial O2 demand)

  • Preload: LVEDV ∝ LVEDP  PCWP; goal PCWP 14-18, PAD 16-20, CVP 8-12 → diuresis, UF with RRT, TNG

  • Afterload: wall stress ∝ MAP (Laplace’s law); SVR = (MAP - CVP)/CO; goal MAP >60, SVR <800-1200

    • Vasodilators: captopril, hydralazine, nitroprusside, TNG

    • Vasopressors: afterload, sometimes needed to MAP in mixed shock or to counteract vasodilatory effect of inodilators

    • IABP: see Mechanical Circulatory Support

  • Contractility: ∝ CO for given preload/afterload; goal CO>4, CI >2.0-2.2, MVO2 >65

    • Dobutamine (inodilator): β1>β2 agonist (production of cAMP); initial dose 0.5-1 mcg/kg/min

      • Watch for tachycardia, ventricular response to AF, arrhythmias, ischemia, HoTN, tachyphylaxis in infusions >24-48h

    • Milrinone (inodilator): PDE-3 inhibitor (breakdown of cAMP); initial dose 0.125 mcg/kg/min

      • Watch for tachycardia, arrhythmias, ischemia, HoTN. Compared to dobutamine, milrinone has longer half-life, greater pulmonary vasodilatation, slightly less chronotropy, fewer arrhythmic events

      • Preferred in patients on βB and w/ RV failure. Renally cleared. Often choice for home inotrope for palliative therapy

    • Epinephrine, norepinephrine, dopamine (inopressors): use if severe HoTN often in combination with inodilators

      • Watch for tachycardia, arrhythmias, end-organ hypoperfusion

  • Advanced: consideration of need for mechanical circulatory support or transplant

    • Goal of mechanical circulatory support: improve systemic perfusion while reducing myocardial oxygen demand (in contrast to inotropes which CO at the expense of increased oxygen demand); see MCS

• Limitations:

  • CO approximated via thermodilution or Fick equation: CO = VO2/(13.4 x Hgb x [SpO2-MVO2]); CI = CO/BSA; VO2  = 125 x BSA

    • Thermodilution: uses temp gradient between two points on PAC. Less reliable if shunt/valvular insufficiency (e.g., TR)

    • Fick equation: assumes a VO2 (oxygen consumption) that in reality varies depending on physiologic state (e.g., infxn)

Intermacs Explanation