New A fib, severe MR, and papillary muscle rupture… 7/17/18

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!

Clinical Pearls

  • 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!
  • Treatment:
    • Aggressive afterload reduction AND
    • Surgery (high mortality rate 20-25%)

Atrial Fribrillation


  • 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)
  • Ischemia/infarction/CAD*
  • Rheumatic heart disease/mitral regurgitation
  • Alcohol/anemia (high output failure
  • Thyrotoxicosis/toxins (stimulants)
  • Electrolytes/endocarditis
  • 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
    • Amiodarone
  • Rhythm control
    • Methods:
      • 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
    • Treatment:
      • Aggressive afterload reduction
      • Urgent/emergent surgical intervention (20-25% mortality)

Cholangiocarcinoma – 7/11/18

Eric presented the case of an elderly woman with no known medical history presenting with subacute onset of painless jaundice and liver failure, found to have perihilar cholangiocarcinoma.

Clinical Pearls:

  • Cholangiocarcinomas are the second most common primary malignancy of the liver after HCC.
  • Perihilar disease is most common.  Can also present with intrahepatic or distal duct involvement.
  • Risk factors include Primary Sclerosing Cholangitis, parasites, and biliary cysts.
  • Metastases occur early in the disease course with the liver being the most common site.
  • Treatment
    • Distal cholangiocarcinoma has the highest resectability.
    • Surgery is the only cure but only a minority of patients present early enough



  • Second most common primary malignancy of liver after HCC
  • Can be intrahepatic, perihilar (most common), or distal
  • Risk factors include:
    • PSC
    • Parasitic infections (liver flukes: clonorchis and Opisthorchis)
    • Biliary atresia
    • Biliary cysts
    • Cholelithiasis, cholecystitis, and hepatolithiasis
  • Mets occur early in disease course except for distal disease
    • Perihilar disease: liver is the most common site of met
    • Intrahepatic: peritoneum, lungs, pleura
    • Distal cholangiocarcinoma: liver, lungs, peritoneum
  • Treatment:
    • Distal disease has the highest resectability
    • Surgery is the only cure but only a minority of patients present early enough
    • Liver transplant in an option for those with
      • perihilar disease
      • < 3 cm tumor size
      • No extrahepatic spread
      • No percutaneous biopsy (increases risk of hematogenous spread)


Paradoxical stroke in patient with ASD – 7/9/18

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!

Clinical Pearls

  • 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:

  1. Hypercoagulable state
    • Inherited disorders
      • Protein C/S deficiency
      • Factor V Leiden
      • Prothrombin 20210 mutation
      • High homocysteine levels
      • Sickle Cell Disease
    • Acquired disorders
      • Pregnancy
      • OCPs
      • Estrogen hormone replacement therapy
      • Malignancy
      • APLS
      • DIC
  2. Vasculopathy
    • Noninflammatory
      • Dissection
      • Trauma
      • Connective Tissue Disease
      • Fibromuscular dysplasia
      • Migraine with aura
    • Inflammatory
      • Vasculitis
        • Large vessel: Takayasu, GCA
        • Small to medium: Kawasaki, PAN
      • Secondary vasculitis
        • Bacterial meningitis
        • HIV
        • Varicella
        • Syphilis
        • TB
        • Fungi (esp cocci)
    • Malformations
      • AVMs –> hemorrhagic
      • Aneurysms –> hemorrhagic
    • Venous
      • Cerebral venous sinus thrombosis
    • Other
      • Moyamoya
  3. Metabolic
    • Vessel injury
      • CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy)
      • Fabry
      • Homocystinuria
    • Pure metabolic
      • MELAS (mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes)
      • Organic acid disorders
  4. Drugs
    • Cocaine
    • Meth
  5. Cardiac
    • Congenital
    • Rheumatic valve disease
    • Mitral Valve Prolapse
    • PFO
    • ASD
    • Endocarditis with septic emboli
    • Atrial myxomas
    • Fibroelastoma
    • Arrhythmias
    • 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
      • DOE
      • Platypnea-orthodeoxia syndrome
    • R sided cardiac chamber enlargement
    • L to R shunt > 1.7:1 (based on TTE findings)
    • Before pacemaker/device placement