PAINE #PANCE Pearl – Women's Health



Question

A 38yo G2P0202 Caucasian woman, with a BMI of 32, presents to your office for evaluation of a “spot” on her breast that she is concerned about. She explains that her great aunt was diagnosed with breast cancer last year at the age of 62 and she is worried. She has not noticed it before, but upon further inquiry states she does not perform self-breast exams very often. She is not currently using any form of contraception (husband has had a vasectomy), but reports using oral contraception pills from age 17-28. She describes her cycle history as regular for her occurring every 28-30 days, lasting 4-5 days with moderate bleeding. She denies any history of abnormal Pap results or any other cancer.

Past Medical History – Hypertension, Anxiety

Medications – Lisinopril 10mg, Escitalopram 10mg

OBGYN History – Menarche at 13, 1st child at 29, 2nd child at 31

Social History – Never smoker, social alcohol

  1. What parts of her history are significant?
  2. What do you specifically want to assess for on your physical examination?
  3. What findings would be considered benign and what would be considered concerning?

Ep-PAINE-nym



Janeway Lesions

Other Known Aliasesnone

Definitionnon-tender, small erythematous or hemorrhagic lesions on the palms of the hands or soles of the feet.

Clinical Significance these lesions are one of the classic, pathognomonic findings in infectious endocarditis. They are caused by septic emboli which deposit bacteria in the dermis of the skin causing microabscesses. In fact, cultures can be taken from these lesions.

HistoryNamed after Edward G. Janeway (1841-1911), who was an American pathologist and received his medical doctorate from the College of Physicians and Surgeons in New York in 1864. He had a prolific career practicing in and around New York city primarily at Bellevue Hospital and served as Health Commissioner of New York from 1875-1882. He went on to become one of the founders of the Association of American Physicians in 1886, as well as president of the Academy of Medicine in 1897 and 1898. A contemporary of Sir William Osler, Janeway was regarded as one of America’s premier internists of the late nineteeth and early twentieth century. He first noted his eponymous finding in 1899 as a “peculiar skin lesion”, but the eponym was first coined by Emanuel Libman in 1906 and later explained in a footnote in an article in 1923.


References

  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. Prutkin JM, Fye WB. Edward G. Janeway, clinician and pathologist. Clinical cardiology. 2006; 29(8):376-7. [pubmed]
  7. Janeway EG. Certain Clinical Observations upon Heart Disease. The Medical News. New York. 1899;65(9):257-262
  8. Libman E. Johns Hopkins Medicine. 1906
  9. Libman E. Endocarditis. Journal of American Medical Association. 1923;80(12);813-817

#55 – Cardiomyopathies



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Definitions

  • 1980 – WHO characterized cardiomyopathies as “heart muscle diseases of unknown causes”
    • Distinguish between non-cardiovascular pathologies (HTN, coronary disease, valvular disease)
  • 1995 – WHO and International Society and Federation of Cardiology (ISFC) developed a task force specifically looking at the definition and classifications of cardiomyopathies
    • Definition they developed was “disease of the myocardium associated with cardiac dysfunction”
      • Dilated cardiomyopathy (DCM)
      • Hypertrophic cardiomyopathy (HCM)
      • Restrictive cardiomyopathy (FCM)
      • Arrhythmogenic right ventricular cardiomyopathy (ARVC)
      • Unclassified cardiomyopathy
  • 2006 – AHA released a statement to update to a more contemporary definition with two major categories
    • Primary cardiomyopathies (predominantly involving the heart)
      • Genetic
        • HCM, ARVC
      • Mixed
        • DCM, RCM
      • Acquired
        • Myocarditis, stress-induced, peripartum, tachy-induced
    • Secondary cardiomyopathies (other system involvement)
  • 2008 – European Society of Cardiology (ESC) updated the WHO/IFSC classification of cardiomyopathies as
    • “a disorder in which the heart muscle is structurally and functionally abnormal in the absence of coronary artery disease, HTN, valvular disease, and congenital heart disease”
      • Meant to more clinically useful
    • Further subcategorized into familial and non-familial causes, as well as removing CAD, vavlvular, congenital heart disease, and ion channelopathies as causes

Echographic Evaluation

  • Systolic
    • Decrease in myocardial contractility resulting in a decrease in left ventricular ejection fraction
      • To compensate, cardiac output is maintained by LV enlargement (increase stroke volume)
    • As a result, systolic dysfunction is most commonly characterized by a dilated cardiomyopathy
  • Diastolic
    • Dysfunction in LV relaxation resulting in abnormal filling and elevated filling pressures
      • Mostly affected by compliance and distensibility of the myocardium
    • As a result, diastolic dysfunction is most commonly characterized by restrictive cardiomyopathy

Current Classifications

Dilated

  • Definition
    • Dilation and impaired contraction of one or both ventricles resulting in an increase in total cardiac mass
  • Numbers
    • Incidence – 5-8 cases per 100,000 population
    • Prevalence – 36 per 100,000
    • STRONG HEART Study (20021) – Up to 14% of middle-aged and elderly may have asymptomatic LV dysfunction
  • Causes
  • Signs and Symptoms
    • Progressive dyspnea on exertion
    • Impaired exercise capacity
    • Orthopnea
    • Paroxysmal nocturnal dyspnea
    • Peripheral edema
    • Cardiomegaly
      • Radiographic
        • > 50% cardiothoracic ratio
      • Clinical
        • Displaced PMI
        • S3 with gallop
  • Classic Echocardiographic Findings
    • Left ventricular cavitary spherical dilation
    • Normal to decreased wall thickness
    • Reduced inward systolic motion
    • Left > Right atrial enlargement and dysfunction

Hypertrophic

  • Definition
    • Increased Left > Right ventricular wall thickness in the absence of pathologic causing conditions
  • Numbers
    • Prevalence – 1:500 of the adult population
  • Causes
    • Primarily genetic
      • Autosomal dominant with incomplete penetrance
      • 60-70% of patients have mutations in the beta myosin heavy chain and cardiac myosin-binding protein C genes
  • Signs and Symptoms
    • Atypical angina (25-30%)
    • Presyncope and syncope during or immediately after exertion (15-20%)
      • More common in patients < 30yo
    • Palpitations
    • Dyspnea on exertion
    • Fatigue
    • Clinical
      • LVOT obstruction
        • S4
        • Harsh crescendo-decrescendo systolic murmur after S1 best heard at apex and lower left sternal border
          • Accentuated by squatting and standing quickly
          • Diminished by standing and squatting quickly or with handgrip
        • Mitral regurgitation murmur
  • Classic Echocardiographic Findings
    • LV wall thickness > 15mm
    • LV outflow obstruction > 30mmHg
    • Asymmetric septal hypertrophy
    • Systolic anterior motion of the mitral valve (SAM)

Restrictive

  • Definition
    • Non-dilated, nonhypertrophied ventricles with moderate to marked biatrial enlargement
  • Numbers
    • ~5% of all cases of cardiomyopathies
  • Causes
    • Infiltrative
      • Amyloidosis, sarcoidosis
    • Non-infiltrative
      • Diabetic, scleroderma
    • Storage Disease
      • Hemochromatosis, Fabry, Gaucher
    • Endomyocardial
      • Cancer/Cancer therapy, pharmacologic
  • Signs and Symptoms
    • Dyspnea
    • Peripheral edema
    • Palpitations
    • Fatigue
    • Weakness
    • Exercise intolerance
    • Clinical
      • Elevated JVP with a prominent y descent
      • S3
      • Hepatosplenomegaly and ascites
  • Classic Echocardiographic Findings
    • Difficult and often requires doppler interrogation
      • Elevated peak mitral inflow velocity
      • Rapid early mitral inflow deceleration
      • Reduced annular velocity
    • Normal to low diastolic volume
    • Normal to low reduced LVEF
    • Atrial enlargement

The Cottage Physician (1893)



References

  1. Report of the WHO/ISFC task force on the definition and classification of cardiomyopathies. British heart journal. 1980; 44(6):672-3. [pubmed]
  2. Richardson P, McKenna W, Bristow M, et al. Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of cardiomyopathies. Circulation. 1996; 93(5):841-2. [pubmed]
  3. Maron BJ, Towbin JA, Thiene G, et al. Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention. Circulation. 2006; 113(14):1807-16. [pubmed]
  4. Elliott P, Andersson B, Arbustini E, et al. Classification of the cardiomyopathies: a position statement from the European Society Of Cardiology Working Group on Myocardial and Pericardial Diseases. European heart journal. 2008; 29(2):270-6. [pubmed]
  5. Dec GW, Fuster V. Idiopathic dilated cardiomyopathy. The New England journal of medicine. 1994; 331(23):1564-75. [pubmed]
  6. Devereux RB, Roman MJ, Paranicas M, et al. A population-based assessment of left ventricular systolic dysfunction in middle-aged and older adults: the Strong Heart Study. American heart journal. 2001; 141(3):439-46. [pubmed]
  7. Felker GM, Thompson RE, Hare JM, et al. Underlying causes and long-term survival in patients with initially unexplained cardiomyopathy. The New England journal of medicine. 2000; 342(15):1077-84. [pubmed]
  8. Maron BJ, Gardin JM, Flack JM, Gidding SS, Kurosaki TT, Bild DE. Prevalence of hypertrophic cardiomyopathy in a general population of young adults. Echocardiographic analysis of 4111 subjects in the CARDIA Study. Coronary Artery Risk Development in (Young) Adults. Circulation. 1995; 92(4):785-9. [pubmed]
  9. Maron BJ. Clinical Course and Management of Hypertrophic Cardiomyopathy. The New England journal of medicine. 2018; 379(7):655-668. [pubmed]
  10. Richard P, Charron P, Carrier L, et al. Hypertrophic cardiomyopathy: distribution of disease genes, spectrum of mutations, and implications for a molecular diagnosis strategy. Circulation. 2003; 107(17):2227-32. [pubmed]
  11. Nienaber CA, Hiller S, Spielmann RP, Geiger M, Kuck KH. Syncope in hypertrophic cardiomyopathy: multivariate analysis of prognostic determinants. Journal of the American College of Cardiology. 1990; 15(5):948-55. [pubmed]
  12. Muchtar E, Blauwet LA, Gertz MA. Restrictive Cardiomyopathy: Genetics, Pathogenesis, Clinical Manifestations, Diagnosis, and Therapy. Circulation research. 2017; 121(7):819-837. [pubmed]
  13. Ammash NM, Seward JB, Bailey KR, Edwards WD, Tajik AJ. Clinical profile and outcome of idiopathic restrictive cardiomyopathy. Circulation. 2000; 101(21):2490-6. [pubmed]
  14. Kushwaha SS, Fallon JT, Fuster V. Restrictive cardiomyopathy. The New England journal of medicine. 1997; 336(4):267-76. [pubmed]

PAINE #PANCE Pearl – Cardiology



Question

A 5yo boy is brought to you clinic by his parents for reporting that his legs hurt “when he plays too much”. His parents corroborate this saying that when he is climbing on the playground for too long he complains that his legs hurt and he needs to stop and rest for awhile. Vaccinations are UTD and he has had a relatively healthy childhood without significant illnesses. He has no significant past medical history and mother reports that she was 38 weeks when he was born via NSVD without any complications. Cardiac auscultation reveals a normal S1 and S2 without murmurs, gallops, or rubs.

  1. What would you expect to find on physical examination?
  2. What other physical assessment can you perform at the bedside to help with the diagnosis?
  3. What findings on diagnostics would also help with the diagnosis?

Answer

The above scenario suggests coarctation of the aorta. The classic physical exam findings are hypertension in the upper extremities, delayed or dminished femoral pulses, and low or unobtainable blood pressures in the lower extremities. Thus, in patients you suspect coarctation of the aorta your should perform a supine bilateral brachial artery blood pressures and prone, supine popliteal blood pressure. In older children and adults, you may see rib notching on chest radiographs from development of large collateral arteries, as well as an indentation of the aortic wall at the site of the coarctation producing the class “3” sign.

Up-to-Date. 2020

Ep-PAINE-nym



Prinzmetal angina

Other Known Aliasesvariant angina

Definitionchest pain that occurs in the absence of exertion, often at rest and sometimes waking the patient up from sleep.

Clinical Significance this type of angina classically is caused by vasospasm of the coronary vessels with or without superimposed antherosclerosis.

HistoryNamed after Myron Prinzmetal (1908-1987), who was an American cardiologist and received his medical doctorate from UCSF School of Medicine in 1933. His career focused mainly on hypertension and heart dysrhythmias with over 160 publications to his name. He published the article describing his eponymous disease in 1959 entitled “Angina pectoris I: A variant form of angina pectoris”.


References

  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. PRINZMETAL M, KENNAMER R, MERLISS R, WADA T, BOR N. Angina pectoris. I. A variant form of angina pectoris; preliminary report. The American journal of medicine. 1959; 27:375-88. [pubmed]

PAINE #PANCE Pearl – Cardiology



Question

A 5yo boy is brought to you clinic by his parents for reporting that his legs hurt “when he plays too much”. His parents corroborate this saying that when he is climbing on the playground for too long he complains that his legs hurt and he needs to stop and rest for awhile. Vaccinations are UTD and he has had a relatively healthy childhood without significant illnesses. He has no significant past medical history and mother reports that she was 38 weeks when he was born via NSVD without any complications. Cardiac auscultation reveals a normal S1 and S2 without murmurs, gallops, or rubs.

  1. What would you expect to find on physical examination?
  2. What other physical assessment can you perform at the bedside to help with the diagnosis?
  3. What findings on diagnostics would also help with the diagnosis?

Ep-PAINE-nym



Takotsubo Cardiomyopathy

Other Known AliasesBroken-Heart Syndrome

Definitionstress-induced cardiomyopathy

Clinical Significance this syndrome is characterized by transient regional systolic dysfunction of the left ventricle, that mimics a myocardial infarction, but with an absence of angiographic evidence of coronary artery involvement.

HistoryNamed after Japanese word for “octopus trap” as the left ventricle takes the shape of this unique hunting vessel. This condition was first studied in Japan by Hikaru Sato in 1991, but it was not “introduced” to the western medical world until 1997.


References

  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. Tofield A. Hikaru Sato and Takotsubo cardiomyopathy. European Heart Journal, Volume 37, Issue 37, 1 October 2016, Page 2812
  7. Pavin D, Breton HL, Daubert C. Human stress cardiomyopathy mimicking acute myocardial syndrome. Heart. 1997;78:509-511.