Tetralogy of Fallot

Other Known AliasesFallot’s tetrad, Fallot’s syndrome, Steno-Fallot tetralogy

DefinitionCongenital cyanotic heart disease due to ventriculo-septal defect, pulmonary stenosis, right ventricular hypertrophy, and overiding aorta.

Clinical SignificanceThis is one of the six congenital cyanotic heart defects and is also the most common.  Read/listen to an amazing review of “Congenital Cyanotic Heart Diseases” here.

HistoryNamed after Etienne-Louis Arthur Fallot (1850-1911), who was a French physician and received his medical doctorate from the University of Marseille in 1867. He described this tetrad in 1888 in an article entitled “Contribution à l’anatomie pathologique de la maladie bleue (cyanose cardiaque)” using previous observations from the work of Dutch anatomist Neils Stenson (1638-1686). Unfortunately, Fallot’s work garnered little contemporary acclaim and it wasn’t until Dr. Paul Dudley White (of WPW fame) translated and republished Fallot’s work in his landmark textbook “Heart Disease” in 1931.


  1. Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  2. Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  3. Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  4. Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  5. Up To Date. www.uptodate.com
  6. Starr JP. Tetralogy of fallot: yesterday and today. World journal of surgery. 2010; 34(4):658-68. [pubmed]
  7. E. L. A. Fallot. Contribution à l’anatomie pathologique de la maladie bleue (cyanose cardiaque). Marseille médical, 1888;25: 77-93.


Hamman’s Sign

Other known aliasesHamman’s crunch

Definitionsystolic precordial crunching sound that occurs with each contraction of the heart that is best heard over precordium in the left lateral decubitus position

Clinical Significancethis is one of the classic physical examination findings in pneumomediastinum or pneumopericardium as a result of trauma to the bronchial tree, bleb rupture, or esophageal rupture.

HistoryNamed after Louis Virgil Hamman (1877-1946), an American internists who received his medical doctorate from Johns Hopkins University in 1902. He was considered one of the great physicians of his era and made significant progress in the management of tuberculosis as the head of the Phipps Tuberculosis Clinic at Johns Hopkins. He described this finding in patients with spontaneous mediastinal emphysema in two separate articles, first in 1939 in The Bulletin of Hopkins Hospital, and then in JAMA in 1945.


  • Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  • Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  • Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  • Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  • Up To Date. www.uptodate.com
  • Cohen AG. Hamman’s Crunch: An historical note. Bulletin of the New York Academy of Medicine. 1971; 47(9):1111-2. [pubmed]
  • Hamman, L. Spontaneous mediastinal emphysema. Bull. Hopkins Hosp. 1939;64:1-21.
  • Hamman L. Mediastinal emphysema: The Frank Billings Lecture. JAMA. 1945;128(1):1–6.

PAINE #PANCE Pearl – Cardiovascular


Angiotensin converting enzyme (ACE) inhibitors are one of the more common medications used in the management of primary hypertension. What are some potential adverse reactions from and contraindications to using ACE inhibitors?


  • Cough
    • 10% of patient experience a dry, hacking cough
  • Hypotension
    • 2% of patients can experience hypotension, weakness, or dizziness from excessive reduction in blood pressure
  • Reduction in GFR
    • 2% of patients can see a doubling in creatinine due to decreased intrarenal perfusion as a result of increased efferent arteriole resistance
  • Hyperkalemia
    • 3% of patients can have a potassium > 5.5 mEq/L by reducing urinary potassium excretion
  • Angioedema
    • 0.3% of patients can experience potentially life-threatening angioedema
  • Pregnancy
    • Associated with cardiac malformations in the first trimester and renal malformations in the second/third trimesters


  • Israili ZH, Hall WD. Cough and angioneurotic edema associated with angiotensin-converting enzyme inhibitor therapy. A review of the literature and pathophysiology. Annals of internal medicine. 1992; 117(3):234-42. [pubmed]
  • Bangalore S, Kumar S, Messerli FH. Angiotensin-converting enzyme inhibitor associated cough: deceptive information from the Physicians’ Desk Reference. The American journal of medicine. 2010; 123(11):1016-30. [pubmed]
  • ONTARGET Investigators – Yusuf S, Teo KK, et al. Telmisartan, ramipril, or both in patients at high risk for vascular events. The New England journal of medicine. 2008; 358(15):1547-59. [pubmed]


Roth’s Spots

Other know aliasesLitten’s spots

Definitionexudative, edematous hemorrhagic lesions of the retina with pale, white centers that can be composed of coagulated fibrin, platelets, infectious organisms, or neoplastic cells

Clinical Significanceone of the classic physical examination findings in bacterial endocarditis seen on fundoscopy. Further research and analysis has shown these can be present in leukemia, diabetes, and hypertensive retinopathy

Historynamed after Mortiz Roth (1839-1914), who was a Swiss pathologist and recieved his medical doctorate from University of Basel in 1864. He practiced all around Switzerland before returning to Basel as professor extraordinary of pathology in 1872, when he published his now eponymous findings in an article entitled “Uber Netzhauteffecstionen bei wundfiebren [Retinal Manifestations of wound fever]”. Dr. Roth, though, never described the classic appearance of the retinal red spot with a white center. Dr. Moritz Litten described this finding 6 years later and would coin the term we still use today.


  • Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  • Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  • Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  • Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  • Up To Date. www.uptodate.com
  • Roth Spots – StatPearls. [article]
  • Roth M. Uber Netzhauteffecstionen bei wundfiebren [Retinal manifestations of wound fever]. Deutsch A Chir. 1872;1:471–84.
  • Litten M. Ueber akute maligne endocarditis
  • und die dabei vorkommenden retinal veranderungen.
  • Charite-Ann 1878;3:135.


Korotkoff Sounds

Other known aliasesnone

Definitionsounds heard during auscultation of the brachial artery during blood pressure measurements

Clinical Significancethese sounds are generated as the sphygmomanometer cuff is slowly being deflated to the point that the maximal impulse of the pressure wave is more than cuff, but the cuff still occluded the artery at the nadir of the impulse. This pressure difference produces turbulence in the blood flow and the characteristic sound on auscultation. There are five phases to the Korotkov sounds with the initiation of Phase 1 as the systolic pressure and the end of Phase 4 as the diastolic pressure.

Historynamed after Nicholai Korotkov (1874-1920), who was a Russian surgeon and earned his medical degree from Moscow University in 1895. He had a prestigious career as a military physician and surgeon earning him an appointment as professor of surgery at the Military Medical Academy at St. Petersberg in 1903. He was preparing his doctoral thesis on vascular surgery when he described his now famous technique for measuring blood pressure in only a 281 word excerpt from a presentation to the Imperial Military Medical Academy entitled “Izvestie Imp. Voiennomedicinskoi Akademii” in 1905.

Nicolai Korotkov
Korotkov’s personal sphygmomanomter


  • Firkin BG and Whitwirth JA.  Dictionary of Medical Eponyms. 2nd ed.  New York, NY; Parthenon Publishing Group. 1996.
  • Bartolucci S, Forbis P.  Stedman’s Medical Eponyms.  2nd ed.  Baltimore, MD; LWW.  2005.
  • Yee AJ, Pfiffner P. (2012).  Medical Eponyms (Version 1.4.2) [Mobile Application Software].  Retrieved http://itunes.apple.com.
  • Whonamedit – dictionary of medical eponyms. http://www.whonamedit.com
  • Up To Date. www.uptodate.com
  • Shevchenko YL, Tsitlik JE. 90th Anniversary of the development by Nikolai S. Korotkoff of the auscultatory method of measuring blood pressure. Circulation. 1996; 94(2):116-8. [pubmed]

#46 – Heart Failure


Definition of Heart Failure

  • Complex clinical syndrome resulting from conditions that affect the structure and/or function of the heart culminating in reduced systemic perfusion that is inadequate to meet the metabolic demands of the body
    • Main effect is decreased cardiac output
  • No more “congestive”


  • 5.7 million adults in US
    • 550,000 new cases each year
    • 1.4 million are under 60 years of age
  • Annual incidence is 10 per 1,000 population AFTER 65 years of age
  • 287,000 deaths per year
    • 1 in 9 deaths included heart failure as contributing causes
    • Most common diagnosis in hospital patients over 65 years of age
  • Responsible for 11 million office visits each year in the US and more hospitalizations than all cancers COMBINED
  • 50% of adults who develop heart failure die within 5 years
  • Cost to US is ~$30 billion/year

Causes and Pathophysiology

Any condition that leads to alteration in left ventricular structure or function can cause heart failure and the specific causes depends on the preservation of ejection fraction.  There is considerable overlap between these with coronary artery disease and hypertension causes the majority of cases.

The problem is that the rest of the body feel the effects of the decreased cardiac output and activate the neurohormonal systems to compensate.  The issue is that this makes the heart failure worse and it is a vicious cycle until it can be broken.

Signs and Symptoms

  • History
    • Reduced cardiac output
      • Fatigue, weakness
    • Excessive fluid accumulation
      • Dyspnea, orthopnea, paroxysmal nocturnal dyspnea, leg swelling, abdominal discomfort, palpitations
  • Physical Examination
    • Appearance and Vital Signs
      • Resting sinus tachycardia
      • Narrow pulse pressure
      • Cool, pale skin (peripheral vasoconstriction)
    • Volume Assessment
      • Pulmonary congestion
        • Rales on auscultation
        • AUDIO
      • Peripheral edema
        • Leg swelling, hepatic congestion, ascites, scrotal edema
      • Elevated jugular venous pressure
        • Hepatojugular reflux
  • Cardiac
    • S3 with gallop (if systolic failure)
    • S4 (if diastolic failure)
    • Displaced PMI past midclavicular line and below the 5th intercostal space
    • Pulsus alternans
      • Evenly spaced alternating strong and weak peripheral pulses
Pulsus Alternans

Framingham Clinical Criteria for Heart Failure

Clinical Decision Rule for Heart Failure

  • Age
    • < 60 = 0 points
    • 60-70 = 4 point
    • 71-80 = 7 points
    • > 80 = 10 points
  • History of coronary disease = 15 points
    • AMI, CABG, PCI
  • Loop diuretic = 10 points
  • Displaced PMI = 20 points
  • Rales = 14 points
  • Irregularly irregular pulse = 11 points
  • Heart murmur = 10 points
  • Pulse Rate = (HR-60)/3 points
  • Elevated jugular venous pressure = 12 points
  • NT-proBNP (pg/mL)
    • < 100 = 0 points
    • 100-200 = 8 points
    • 200-400 = 16 points
    • 400-800 = 24 points
    • 800-1600 = 32 points
    • 1600-3200 = 40 points
    • > 3200 = 48 points
  • Interpretation
    • < 13 points = < 10% probability of heart failure
    • > 54 points = > 70% probability of heart failure

Diagnostic Studies

  • Electrocardiogram
    • Not really diagnostic, but can evaluate for current ischemia, past infarction, low voltage, dysrhythmias
    • A normal EKG makes systolic dysfunction unlikely (98% NPV)
  • Laboratory studies
    • Brain natriuretic peptide (BNP) and N-terminal proBNP (NT-proBNP)
      • Released from ventricles  when stretched
      • < 100 pg/mL = very high NPV and rule out heart failure
    • Cardiac enzymes
    • CBC
    • CMP
  • Chest Radiography
    • Increased cardiothoracic ratio
    • Cephalization of pulmonary vessels
    • Kerley B-lines
    • Pleural effusions
  • Echocardiography
    • Recommended for all patient with dyspnea and suspicion of heart failure
    • Provides vital information on:
      • Ejection fraction
        • < 40% = reduced = systolic
        • > 50% = preserved = diastolic
      • Valvular disease
        • Aortic and mitral regurgitation/insufficiency
      • Atrial and Ventricular size and function
        • Enlarged left ventricle = systolic
        • Left atrial enlargement with normal/small left ventricle = diastolic
      • Left ventricular wall size
        • Thin = systolic
        • Thick = diastolic
  • Exercise/Stress Testing
    • Evaluate for underlying coronary disease, as well as potential candidates for transplantation
      • Patients with a peak oxygen uptake (VO2) < 14 mL/kg/min have better outcomes with transplanted
  • Coronary angiography
    • Not strongly recommended as part of the work-up, but can be useful to evaluate for underlying coronary disease and get an accurate ejection fraction

Classification and Grading

Current Nomenclature

Heart failure with reduced ejection fraction (HFrEF)

  • Systolic
  • < 40% EF

Heart failure with preserved ejection fraction (HFpEF)

  • Diastolic
  • > 50% EF


  • Heart failure with reduced ejection fraction (HFrEF)
    • Lifestyle Modifications
      • Smoking cessation
      • Restrict sodium to < 3g/day
      • Restrict fluid to < 2L/day
    • Pharmacotherapy
      • Loop diuretic (if overload is present)
        • Furosemide, bumetanide, torsemide
      • ACE inhibitors
        • Lisinopril, enalapril
      • Angiotension receptor-neprilysin inhibitor (ARNI)
        • NYHA II or III and:
          • BNP > 150 ng/mL or hospitalized with last 12 months
          • SBP > 100 mmHg
          • GFR > 30 mL/min
          • No history of angioedema
      • ARB
        • Candesartan, valsartan
      • Beta blockers
        • Carvedilol, metoprolol, bisoprolol
      • Mineralcorticoid receptor antagonist (MRA)
        • Spironolactone, eplerenone
      • Selective sinus node inhibitor
        • Ivabradine
        • Need a resting HR > 70 bpm on maximum BB therapy
    • Ischemic heart disease
      • Increase coronary perfusion
      • Decrease myocardial demand
    • Hyperlipidemia
      • High-intensity statin
    • Cardiac rehabilitation
  • Heart failure with preserved ejection fraction (HFpEF)
    • Differences with HFrEF
      • ACEI/ARB not as useful
      • MRA used more often
      • Diuretics OK but be careful for volume depletion
      • Don’t use BB unless compelling indication


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  2. CDC Heart Failure Data Sheet. https://www.cdc.gov/dhdsp/data_statistics/fact_sheets/fs_heart_failure.htm
  3. Emory Healthcare Heart Failure Statistics. https://www.emoryhealthcare.org/heart-vascular/wellness/heart-failure-statistics.html
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  8. Maisel A. B-type natriuretic peptide levels: diagnostic and prognostic in congestive heart failure: what’s next? Circulation. 2002; 105(20):2328-31. [pubmed]
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  15. Ponikowski P, Voors AA, Anker SD, et al. 2016 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure: The Task Force for the diagnosis and treatment of acute and chronic heart failure of the European Society of Cardiology (ESC)Developed with the special contribution of the Heart Failure Association (HFA) of the ESC. European heart journal. 2016; 37(27):2129-2200. [pubmed]
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