Thanks to Eric for presenting the case of an “late middle-age” woman with chest pressure, found to have ST depressions, troponin elevation, TTE with apical akinesis, and clean coronaries on cardiac cath concerning for Takotsubo cardiomyopathy.
- Think of heart failure as ischemic vs non-ischemic
- Most common causes of heart disease are ischemia (CAD), HTN, idiopathic, valvular, infectious (viral), and drugs.
- MINOCA or myocardial infarction with nonobstructive coronary arteries is MI in the absence of coronary artery disease with >50% vessel occlusion and includes the following etiologies
- Stress induced cariodmyopathy (Takotsubo)
- Coronary vasospasm
- Microvascular dysfunction
- Takotsubo cardiomyopathy most commonly presents in postmenopausal women and triggered by physical or emotional stress. The pathogenesis is not well understood and the course is self-limited. Treatment is largely supportive. Prognosis to recovery of cardiac function is 1-4 weeks.
- Valvular disease
- Infectious (viral is most common)
- Infiltrative (sarcoid, amyloid, hemochromatosis)
- Stress induced cardiomyopathy (Takotsubo)
- High output (secondary to anemia, Paget’s disease, pregnancy, AV fistula, beriberi, hyperthyroidism)
- Connective tissue disease
MI with non-obstructive coronary arteries (MINOCA)
- Diagnosis: requires the following
- Clinical documentation of MI
- Exclusion of obstructive CAD
- No overt cause for acute MI present
- Etiologies: significant overlap with non-ischemic causes of heart failure
- Reduced troponin clearance (i.e. renal impairment)
- Increased right heart pressures (ex PE)
- Cardiac causes
- Stress induced cardiomyopathy
- Inflammation (myocarditis)
- Coronary artery spasm (vasospastic angina)
- Microvascular dysfunction (microvascular angina, microvascular spasm, coronary slow flow phenomenon)
- Work up
- MINOCA is a working diagnosis
- Exclude non-cardiac cause
- Rule out ischemic etiology
- Cardiac MRI is often indicated
- Provocative spasm testing (with acetylcholine etc in the case of coronary vasospasm)
- First described in Japan in 1990
- It is the underlying etiology in ~1-2% of patients presenting with ACS
- More common in post-menopausal women (mean age 66.4)
- Clinical manifestations
- Often triggered by emotional or physical stress but ~30% of the time, no trigger is identified
- Most commonly present with acute substernal chest pain. Less commonly present with SOB or syncope or heart failure symptoms
- May have late peaking systolic murmur similar to HOCM
- EKG changes:
- ST elevation in anterior leads (43.7%)
- ST depression (7.7%)
- QT prolongation, T wave inversion, abnormal Q waves
- Troponin elevation in most patients (mean initial troponin is ~7-8)
- Diagnostic criteria
Transient LV systolic dysfunction (hypokinesis, akinesis, or dyskinesis), wall motion abnormalities that extend beyond a single epicardial coronary distribution
Absence of CAD based on cath
New EKG abnormalities
Absence of pheochromocytoma or myocarditis
- Work up
- Rule out ACS
- Cardiac MRI to rule out other causes of MINOCA
Picture above and cool video from NEJM here.
Thanks Hong for presenting the case of a middle-aged woman with recent diagnosis of Grave’s disease off methimazole who presented with A fib with RVR and congestive heart failure, raising a debate on thyroid storm!
- Thyroid storm is an extremely rare (1 in 500,000) but life-threatening diagnosis (up to 30% mortality) that should not be missed.
- Degree of thyroid hormone elevation or TSH suppression is not a criteria for diagnosing thyroid storm! In fact, levels are typically similar to those of patients with uncomplicated thyrotoxicosis.
- Common clinical feature is cardiovascular symptoms (heart failure, arrhythmia, tachycardia) but more specific finding is AMS.
- Scoring criteria to screen for thyroid storm include Burch/Wartofsky and the Akamizu criteria, but they have not been validated.
- Consult endocrine early if you suspect thyroid storm!
- Longstanding untreated hyperthyroidism
- Thyroid/non-thyroidal surgery
- Acute iodine load
- Irregular use or discontinuation of antithyroid treatment
Etiology: not clearly understood, but possibly related to the following
- Rapid rate of increase in thyroid hormone levels?
- Increased responsiveness to catecholamines?
- Enhanced cellular responses to thyroid hormone?
- The degree of thyroid hormone elevation or TSH suppression is not typically more profound than uncomplicated thyrotoxicosis
- CV (>60% of cases)
- AMS (considered by many to be essential to diagnosis)
- Agitation, anxiety, delirium, psychosis, stupor, coma
- Features associated with worse outcomes?
- Older age >60
- Mechanical ventilation
- Not using antithyroid drugs or beta blockers
- Clinical! No universally accepted criteria or validated clinical tools. Degree of hyperthyroidism is not a criterion for diagnosis. Some to know of that might be helpful:
- Burch and Wartofsky (sensitive, not specific)
- > 45: highly suggestive of thyroid storm
- 25 – 44: impending storm
- <25: thyroid storm unlikely
- Akamizu (Japanese) system developed in 2012 (less sensitive but more specific)
- ICU admission!
- Beta blockers ⇒ control symptoms from increased adrenergic tone
- Thionamide ⇒ block new hormone synthesis. PTU is preferred because it blocks peripheral conversion of T4 to T3.
- Iodine solution ⇒ block release of thyroid hormone (saturated solution of potassium iodide)
- Iodinated radiocontrast agent (not available anymore in most places) ⇒ inhibit peripheral conversion of T4 to T3
- Glucocorticoids ⇒ reduce T4 to T3 conversion, promote vasomotor stability, and treat any associated relative adrenal insufficiency
- Bile acid sequestrants ⇒ decrease enterohepatic recycling of thyroid hormones (only in very severe cases)
- Start with beta blockers + PTU, and stress dose steroids
- 1 hour later: start SSKI q6h (after hormone synthesis has been halted with PTU, otherwise SSKI can make thyroid storm worse)
Thanks to Michelle for presenting the case of an elderly man with CKD5 presenting with GI symptoms and bradycardia, found to have regularized A fib consistent with dig toxicity!
- A “regularized” atrial fibrillation rhythm should trigger work up for digoxin toxicity. This rhythm is generated because of complete heart block. So the atria continue to fibrillate but no impulse is getting through to the ventricles. As a result, a junctional (narrow complex) escape rhythm takes over.
- Elevated digoxin levels can rule in the diagnosis of dig toxicity but normal levels do not rule it out.
- Risk factors for dig toxicity include: renal dysfunction, hypokalemia, hypomagnesemia, and hypercalcemia.
Clinical manifestations of dig toxicity:
- Acute: predominantly GI symptoms (nausea/vomiting, anorexia, non-specific abdominal pain)
- Chronic: predominantly neurologic symptoms (delirium, confusion, weakness, lethargy, disorientation, vision changes)
- Cardiac manifestations: can be acute or chronic and of greatest concern!
- Most common finding ⇒ PVCs!
- Other arrhythmias: AVB, atrial tachyarrhythmia, ventricular bigeminy, junctional rhythm, bidirectional ventricular tachycardia (RARE and only a few drugs can cause this)
- Scooped ST depressions (the famous Salvador Dali mustache)
- Increased U waves
- QT shortening
Risk factors for developing toxicity:
- Renal dysfunction
- Hypokalemia, hypomagnesemia, hypercalcemia
- Ingestion 1-2 hours ago? ⇒ activated charcoal
- Indications for using Digoxin-specific antibody fragments (AKA DigiFab)
- Severe poisoning
- life threatening/hemodynamically unstable arrhythmia
- K > 5
- Organ hypoperfusion (AMS, renal failure)
- Other considerations before giving DigiFab:
- Hyperkalemia ⇒ do NOT treat. DigiFab will lower levels
- Hypokalemia ⇒ Treat! DigiFab will make it worse
- Hypomagnesemia ⇒ Treat!
- Serum digoxin concentration is irrelevant!
- You’ve ordered DigiFab but pharmacy is taking too long? ⇒ atropine!
Thanks to Janhavi for presenting the case of a middle-aged man with no significant PMH presenting with acute onset of malaise, myalgias, and a “stubbed toe,” septic with petechiae on palms and soles, found to have mitral valve endocarditis.
- Endocarditis is more common in men (2:1)
- ~50% of cases of endocarditis occur in people with no known underlying valve disease
- 80% of cases are caused by staph and strep species
- TEE is the gold standard for diagnosis and recommended when clinical suspicion for endocarditis is high. TTE is more helpful to rule out disease when clinical suspicion is low.
- Indications for early surgery based on this NEJM article include:
- Heart failure
- Uncontrolled infection
- Prevention of embolic events
- Blood culture positive:
- Typical organism in two separate blood cultures
- Persistently positive blood cultures
- Single positive culture for Coxiella
- E/o endocardial involvement
- Echo positive for vegetation
- New valve regurgitation
- Predisposition to IE (i.e. IVDU, prosthetic valve, congenital cyanotic heart disease)
- Fever >38
- Vascular phenomena ⇒ arterial emboli, pulmonary infarcts, mycotic aneurysms, intracranial hemorrhage, conjunctival hemorrhage, Janeway lesions
- Immunologic phenomena ⇒ GN, Osler’s nodes, Roth’s spots, RF
- Microbiologic evidence: positive blood culture not meeting major criteria
Probability of endocarditis:
- 2 major, 1 major + 3 minor, 5 minor
- 1 major + 1 minor, or 3 minor
- Firmly established alternative diagnosis
- Resolution of symptoms < 4 days with antibiotics
- Does not meet definite/possible criteria
Indications for surgery:
Valve dysfunction causing heart failure
Perivalvular extension with development of abscess, fistula, and/or heart block
Fungi or other highly resistant organisms that are difficult to treat with abx alone
Persistent bacteremia despite maximal treatment
Recurrent embolization with persistent vegetations
Large vegetations (>1 cm) with severe valvular regurg
S aureus prosthetic valve endocarditis
Indications for early surgery:
- Heart failure
- Uncontrolled infection
- Prevention of embolic events
- Most common cause of death: heart failure
- Heart block
- More likely with s. aureus or S. bovis, veg > 1 cm, or increased veg mobility on echo
- Antiplatelet therapy initiation is not recommended because of increased risk of hemorrhagic conversion of septic emboli
- Check out this blog post and this great review article in the NEJM.
Thank you Naina for presenting the case of an elderly man with 20 packyear smoking history presenting with acute onset of dyspnea and scant hemoptysis, found to have new onset A fib and L heart failure secondary to severe mitral regurgitation resulting from papillary muscle rupture!
- In patients with severe mitral regurgitation (MR) and a normal L atrium size, think about acute causes of MR. TEE is often indicated to better visualize the valve structure and determine need for operative intervention.
- MR can be caused by papillary muscle rupture, especially 3-7 days post MI. Other etiologies of rupture include endocarditis and myxomatous valve degeneration.
- Patients with rupture present with acute onset hypotension, pulmonary edema, and a hyperactive precordium. A systolic murmur is not always present!
- Aggressive afterload reduction AND
- Surgery (high mortality rate 20-25%)
- Paroxysmal (terminates within 7 days)
- Persistent (>7 days)
- Long-standing persistent (>1 year)
Differential for new onset A fib: (PIRATES!)
- Pulmonary (OSA, PE, COPD, PNA)
- Rheumatic heart disease/mitral regurgitation
- Alcohol/anemia (high output failure
- Thyrotoxicosis/toxins (stimulants)
- Sepsis/sick sinus syndrome
- Other: HTN*, congenital heart disease, previous cardiac surgery, viral infections
* Most common causes in the US.
- Rate control (preferred method based on AFFIRM and RACE trials)
- Beta blockers
- Calcium channel blockers ⇒ contraindicated in decompensated heart failure
- Digoxin ⇒ avoid use in renal failure, hypokalemia, hypomagnesemia, or hypercalcemia
- Rhythm control
- Chemical (~30% success rate)
- Class III (amiodarone, sotalol, ibutilide)
- Electrical (synchronized to QRS, ~80% success rate)
- Preferred modality in
- Hemodynamically unstable
- Young patient (age <65) or good functional status
- Early in natural history of disease
- Failure of rate control agents
- Heart failure
Complications post MI:
Figure from article by Reed et al. Lancet. 2017.
Papillary muscle rupture:
- Posteromedial muscle is 6-12x more likely because blood supply is through PDA only. Anterolateral muscle receives dual supply from LAD and LCx.
- Clinical presentation
- Acute onset hypotension, pulmonary edema
- Hyperactive precordium
- Mid, late, or holosystolic murmur with widespread radiation (though many have no murmur!)
- Diagnosis requires TTE/TEE
- Aggressive afterload reduction
- Urgent/emergent surgical intervention (20-25% mortality)
Today, Joe presented the case of a young woman presenting with acute onset of L sided weakness, found to have a paradoxical stroke due to ASD!
- Paradoxical stroke is a diagnosis of exclusion
- Atrial septal defects (ASDs) have been associated with cryptogenic stroke (stroke of unknown etiology). An embolic source is often not identified.
- Ostium secundum is the most common type of ASD (>70% of cases)
- Indications for ASD closure include the following
- Symptomatic patient (DOE, platypnea-orthodeoxia)
- R sided cardiac chamber enlargement
- Left to right shunt >1.7:1
- Before pacemaker or device placement
- After a stroke
- What about PFOs and cryptogenic stroke?
- In the past, the recommendation was not to close them. However, the 2017 CLOSE and REDUCE trials (as well as the 2013 RESPECT trial) showed that closure of PFO reduces the risk of a second stroke compared with medical therapy alone. Thus, the latest ACC recommendation is to close PFOs after stroke!
Etiologies of stroke in a young adult:
- Hypercoagulable state
- Inherited disorders
- Protein C/S deficiency
- Factor V Leiden
- Prothrombin 20210 mutation
- High homocysteine levels
- Sickle Cell Disease
- Acquired disorders
- Estrogen hormone replacement therapy
- Connective Tissue Disease
- Fibromuscular dysplasia
- Migraine with aura
- Large vessel: Takayasu, GCA
- Small to medium: Kawasaki, PAN
- Secondary vasculitis
- Bacterial meningitis
- Fungi (esp cocci)
- AVMs –> hemorrhagic
- Aneurysms –> hemorrhagic
- Cerebral venous sinus thrombosis
- Vessel injury
- CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy)
- Pure metabolic
- MELAS (mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes)
- Organic acid disorders
- Rheumatic valve disease
- Mitral Valve Prolapse
- Endocarditis with septic emboli
- Atrial myxomas
- Cardiac surgery
Atrial Septal Defects
- Secundum is seen in 75% of cases
- Exam findings:
- Fixed split S2
- Parasternal impulse
- Mid-systolic mumur at LSB (can be mid-diastolic also)
- EKG with RV strain and partial RBBB
- Indications for closure:
- After stroke
- Symptomatic patient
- Platypnea-orthodeoxia syndrome
- R sided cardiac chamber enlargement
- L to R shunt > 1.7:1 (based on TTE findings)
- Before pacemaker/device placement