Ebook Pathophysiology flash cards: Part 1

Part 1 book “Pathophysiology flash cards” has contents: Cardiovascular system, respiratory system, renal system, gastrointestinal system and nutritional disorder, nervous system and special senses, psychiatric disorders.

Medicine is an ever-changing science As new research and clinical experiencebroaden our knowledge, changes in treatment and drug therapy are required Theauthors and the publisher of this work have checked with sources believed to bereliable in their efforts to provide information that is complete and generally inaccord with the standards accepted at the time of publication However, in view

of the possibility of human error or changes in medical sciences, neither theauthors nor the publisher nor any other party who has been involved in thepreparation or publication of this work warrants that the information containedherein is in every respect accurate or complete, and they disclaim allresponsibility for any errors or omissions or for the results obtained from use ofthe information contained in this work Readers are encouraged to confirm theinformation contained herein with other sources For example and in particular,readers are advised to check the product information sheet included in thepackage of each drug they plan to administer to be certain that the informationcontained in this work is accurate and that changes have not been made in therecommended dose or in the contraindications for administration Thisrecommendation is of particular importance in connection with new orinfrequently used drugs

Copyright © 2013 by McGraw-Hill Education All rights reserved Except aspermitted under the United States Copyright Act of 1976, no part of thispublication may be reproduced or distributed in any form or by any means, orstored in a database or retrieval system, without the prior written permission ofthe publisher.

ISBN: 978-0-07-182831-4

MHID: 0-07-182831-1

The material in this eBook also appears in the print version of this title: ISBN:978-0-07-176740-8, MHID: 0-07-176740-1

McGraw-Hill Education books are available at special quantity discounts to use

as premiums and sales promotions or for use in corporate training programs Tocontact a representative, please visit the Contact Us page atwww.mhprofessional.com

TERMS OF USE

This is a copyrighted work and McGraw-Hill Education and its licensors reserveall rights in and to the work Use of this work is subject to these terms Except aspermitted under the Copyright Act of 1976 and the right to store and retrieve onecopy of the work, you may not decompile, disassemble, reverse engineer,reproduce, modify, create derivative works based upon, transmit, distribute,disseminate, sell, publish or sublicense the work or any part of it withoutMcGraw-Hill Education’s prior consent You may use the work for your ownnoncommercial and personal use; any other use of the work is strictly prohibited.Your right to use the work may be terminated if you fail to comply with theseterms

THE WORK IS PROVIDED “AS IS.” McGRAW-HILL EDUCATION ANDITS LICENSORS MAKE NO GUARANTEES OR WARRANTIES AS TOTHE ACCURACY, ADEQUACY OR COMPLETENESS OF OR RESULTS

TO BE OBTAINED FROM USING THE WORK, INCLUDING ANY

INFORMATION THAT CAN BE ACCESSED THROUGH THE WORK VIAHYPERLINK OR OTHERWISE, AND EXPRESSLY DISCLAIM ANYWARRANTY, EXPRESS OR IMPLIED, INCLUDING BUT NOT LIMITED

TO IMPLIED WARRANTIES OF MERCHANTABILITY OR FITNESS FOR

A PARTICULAR PURPOSE McGraw-Hill Education and its licensors do notwarrant or guarantee that the functions contained in the work will meet yourrequirements or that its operation will be uninterrupted or error free NeitherMcGraw-Hill Education nor its licensors shall be liable to you or anyone else forany inaccuracy, error or omission, regardless of cause, in the work or for anydamages resulting therefrom McGraw-Hill Education has no responsibility forthe content of any information accessed through the work Under nocircumstances shall McGraw-Hill Education and/or its licensors be liable for anyindirect, incidental, special, punitive, consequential or similar damages thatresult from the use of or inability to use the work, even if any of them has beenadvised of the possibility of such damages This limitation of liability shall apply

to any claim or cause whatsoever whether such claim or cause arises in contract,tort or otherwise

This set of review cards is designed for use by medical students at various levels

of training, inclusive of preclinical students preparing for USMLE Step 1examination to senior students currently in clinical training and preparing forinternship and reviewing for USMLE Step 2 examination

As such, the cards can be used in multiple ways Junior students can use them

to self-test and prepare for examination as well as to provide exposure to some

of the most common clinical presentations with pertinent diagnostic findings.Students in advanced training will find the cards useful to relate therapeuticmanagement decisions to underlying pathophysiology On each card, key clinicalinformation is presented to help the student recognize high yield concepts andpresentation of a particular disease entity Explanations on the back of the cardprovide concise and highly relevant (for clinical care or exam review) details ofphysiologic mechanisms

It is our goal that use of this product will help students to simultaneouslylearn/recognize important (highly testable!) clinical conditions, while reviewingthe important underlying pathophysiologic principles associated with each

Sarina Amin is a fourth year medical student at the University of Central Florida,College of Medicine She will be pursuing a residency in ophthalmology at theUniversity of Florida in Gainesville She has a strong interest in academicmedicine and global health

Keith Connolly is a fourth year medical student at the University of CentralFlorida, College of Medicine, and a military veteran He will be continuing histraining at the University of Pennsylvania with a residency in orthopedic surgery.His additional interests include medical education and training as well asinpatient infection prevention

Maria L Cannarozzi is an associate professor of internal medicine andpediatrics at the University of Central Florida, College of Medicine Dr.Cannarozzi is the Clerkship Director of the Core Clerkship in Internal andFamily Medicine and also provides clinical care in the ambulatory setting

Jonathan Kibble is an associate professor of physiology and is the assistantdean of medical education at the University of Central Florida Dr Kibble hastaught physiology and pathophysiology to medical students for over 15 years

and is coauthor of the textbook Medical Physiology: The Big Picture; his

academic research is on assessment in medical education

AS ankylosing spondylitis

AST aspartate aminotransferase

ATIN acute tubulointerstitial nephritisATM ataxia-telangiectasia gene

ATN acute tubular necrosis

AV atrioventricular

BMI body mass index

CT computed tomography

CVA cerebrovascular accident

DDAVP desmopressin

DEXA dual energy X-ray absorptiometryDHT dihydrotestosterone

EPO erythropoietin

ESR erythrocyte sedimentation rate

FDPs fibrin degradation products

FEV1 forced expiratory volume in the first second of expirationFGF-23 fibroblast growth factor-23

MLF medial longitudinal fasciculus

MN membranous nephropathy

MPGN membranoproliferative glomerulonephritisMRI magnetic resonance imaging

NO nitric oxide

NPH normal pressure hydrocephalus

SIADH syndrome of inappropriate antidiuretic hormoneSLE systemic lupus erythematosus

SNS sympathetic nervous system

STEMI ST segment elevation myocardial infarctionSVC superior vena cava

A 59-year-old male presents to a local emergency department with complaints ofchest pressure and pain The pain is located in his mid-chest and radiates to hisleft jaw area He has had the pain “this time” for about 30 minutes and it isgetting much worse He has previously had similar pain, although not as severe,

on two other occasions in the last 2 weeks His blood pressure is 150/88 mm Hg,pulse is 122/min, respiratory rate is 22/min and labored, and temperature is38.0°C (100.4°F) He appears anxious and dyspneic; his skin is cool and moist.Cardiac auscultation reveals a regular tachycardic heart rate, with a third heartsound present immediately after S2 There is no murmur noted Bilateral lungfields are clear to auscultation, and jugular venous pressure (JVP) is visible 3 to

5 cm above the sternal angle Distal pulses are brisk and equal bilaterally;extremities are pink with brisk capillary refill noted Chest radiograph is withoutsignificant abnormality; ECG reveals sinus tachycardia and ST segmentelevations in lead V1-V4

ST Segment Elevation Myocardial Infarction (STEMI)

This patient is having an acute coronary syndrome (ACS) event, specifically STelevation myocardial infarction This group of disorders includes unstableangina, and both ST and non-ST segment elevation myocardial infarctions(STEMI and NSTEMI, respectively) Classic presentation is substernal chestpressure, heaviness, or pain, which may radiate to the patients’ left arm or leftjaw region The pain is most often precipitated by exertion and relieved by rest,although the syndrome is generally progressive over time and may eventuallyproceed to symptoms at rest Activation of the sympathetic nervous system(SNS) as well as pain has caused elevation of pulse and BP in this patient Anominous sign in this setting would be relatively low blood pressure, whichwould indicate likely significant ischemic left ventricular (LV) damage.Diaphoresis is another indication of SNS response to injury and pain The S3sound is indicative of reduced ventricular compliance, likely due to acuteischemic changes in the myocardium There is no evidence of heart failure inthis patient (normal JVP and clear lung fields) as the event is acute and withoutdecompensation at this time Normal extremity perfusion indicates adequateventricular function, although ECG is significant for myocardial injury in theanteroseptal distribution (V1-V4 leads) In addition to oxygen, nitrate, aspirin,and morphine therapy, this patient needs immediate intervention in the form ofintravascular stent placement or fibrinolysis in order to immediately restorecardiac perfusion

A 72-year-old male presents to an urgent care center with chest pain The pain islocated in his mid-chest and radiates to his left jaw and neck area He has had thepain “this time” for about 30 minutes and it is getting much worse He haspreviously had similar pain, although not as severe, on at least four otheroccasions in the last 1 to 2 months His blood pressure is 150/88 mm Hg, pulse is122/min, respiratory rate is 22/min and labored, and temperature is 38.0°C(100.4°F) He appears mildly anxious His skin is cool and moist Cardiacauscultation reveals a regular tachycardic heart rate and normal S1 and S2 There

is no murmur noted Bilateral lung fields are clear to auscultation, and JVP isvisible 3 to 5 cm above the sternal angle Distal pulses are brisk and equalbilaterally; extremities are pink with brisk capillary refill noted Chestradiograph is without significant abnormality; ECG reveals sinus tachycardiaand a right bundle branch block, which is unchanged from previous ECGtracing No other abnormalities are noted

Non-ST Segment Elevation Myocardial Infarction (NSTEMI)

This patient is having an ACS event, most likely unstable angina or NSTEMI.This group of disorders includes unstable angina, and both STEMI andNSTEMI The disorders are differentiated by the presence or absence of ECGchanges and biochemical markers Classic presentation of ACS is substernalchest pressure, heaviness, or pain, which may radiate to the patient’s left arm orleft jaw region The pain is most often precipitated by exertion and relieved byrest, although the syndrome is generally progressive over time and mayeventually proceed to symptoms at rest Activation of the SNS as well as painhas caused elevation of pulse and BP in this patient An ominous sign in thissetting would be relatively low blood pressure, which would indicate likelysignificant ischemic LV damage Diaphoresis is another indication of SNSresponse to injury and pain This patient has no signs of ventricular ischemiacurrently in that there is normal perfusion noted to extremities and no ECGchanges nor S3 or S4 heart sounds There is also no evidence of heart failure inthis patient (normal JVP and clear lung fields) as the event is acute and withoutdecompensation at this time In addition to oxygen, nitrate, aspirin, andmorphine therapy, this patient needs further evaluation and close monitoring forpossible progression to NSTEMI or STEMI Evaluation of biochemical markers(cardiac troponin and creatine kinase MB evaluation) is indicated as well

A 75-year-old woman presents to a physician’s office concerned aboutworsening shortness of breath She denies any chest pain but states that she hasbecome more short of breath over the last week First her shortness of breath wasintermittent; now it is more constant Her blood pressure in the office is 133/58

mm Hg, pulse is 111/min, respiratory rate is 18/min, and temperature is 38.0°C(100.4°F) She appears anxious Cardiac auscultation reveals a rhythm that isirregularly irregular and mildly tachycardic Distal pulses are intact with thesame irregularity, which is noted at the precordium Chest radiograph showsborderline heart enlargement but no evidence of vascular congestion or fluid inthe lungs ECG is pending

This patient has atrial fibrillation This fairly common arrhythmia often has asubacute onset as described in the scenario above This rhythm disturbance may

be intermittent (paroxysmal) or continuous Palpitations and shortness of breathare frequently seen Ineffective atrial contraction disrupts laminar flowthroughout the heart, with several consequences Ineffective flow can lead to

“stagnant” cardiac circulation, predisposing the patient to atrial blood clots andincreased risk of pulmonary embolus and stroke Dyspnea occurs as a result ofirregular and often rapid ventricular response to chaotic atrial activity.Ineffective ventricular pumping causes inadequate forward flow of blood to thesystemic circulation The most common predisposing factors are hypertensive orvalvular heart disease, although many cardiac or pulmonary conditions canprecipitate this condition Diagnosis of atrial fibrillation is easily made withECG, which shows absence of P waves and an irregularly irregular pattern ofQRS complexes due to rapid and random stimulation of the atrioventricular (AV)node

A 38-year-old male presents to a physician’s office for ongoing management ofhis chronic illnesses His medical history is significant for asthma andhypertension He has no complaints today His blood pressure is 136/86 mm Hg,pulse is 82/min, respiratory rate is 12/min, and temperature is 36.4°C (97.5°F).Physical examination is notable for frequent ectopy noted upon cardiacauscultation, best appreciated at the cardiac base The patient is asked and deniespalpitations, dizziness, or lightheadedness ECG and rhythm strip are obtainedand are notable for sinus rhythm with intermittent biphasic P waves, whichappear to be conducted to the ventricles early QRS complexes appear normal;there is no T wave or ST segment abnormality

This patient is having premature atrial contractions (PACs) PACs are a commoncardiac arrhythmia and are generally considered to be benign, almost nevercausing hemodynamic instability or other serious clinical symptoms Frequently,however, PACs or PVCs (premature ventricular contractions) can bedisconcerting to patients, causing palpitations, anxiety, or psychosomatic chestpain The etiology of this disorder is not well understood, but the prematurebeats are commonly associated with high stress, excessive use of caffeine, orlack of adequate sleep Interestingly, these abnormal beats frequently disappearwith exercise as do the symptoms caused by them Most commonly PACs arenot conducted through the AV node to the ventricles, but the “fate” of a PAC isultimately determined by intrinsic heart rate and the refractory period of the AVnode or bundle branch fascicles If a PAC is able to pass through the AV nodeand to the fascicular bundles, the outcome can be a right bundle branch blockdue to slowed transmission through normal conduction pathways The ECGwaveform for PACs may have unusual appearances since they have an ectopicorigin and follow a different conduction pathway through the atria than normalsinoatrial (SA) nodal stimulation

A 68-year-old male presents to a physician’s office for ongoing management ofhis chronic illnesses His medical history is significant for long-standing tobaccoabuse, chronic obstructive pulmonary disease (COPD) – emphysematous type,and hypertension Other than chronic dyspnea and cough, he complains ofintermittent dizziness and lightheadedness over the past few weeks His bloodpressure is 138/90 mm Hg, pulse is 128/min, respiratory rate is 20/min, andtemperature is 36.8°C (98.2°F) The patient is noted to have an increasedanterior-posterior (AP) thorax diameter or “barrel chest” appearance Hebreathes deliberately with the expiratory phase exceeding that of inspiration.Sinus tachycardia and an S4 are noted upon cardiac auscultation ECG andrhythm strip are obtained and are notable for sinus tachycardia with P waves ofvariable morphology QRS complexes appear normal; there are no T wave or STsegment changes noted

This patient has multifocal atrial tachycardia (MAT) This condition occurs whenmultiple sites within the right atrium (other than the SA node) initiate cardiaccontractions Unlike PACs, many of these atrial beats are conducted through AVnode, causing an increase in ventricular rate, sometimes up to 200 beats perminute Hemodynamic instability and symptoms can occur if the tachycardialeads to inadequate ventricular filling The increased workload of the heart mayalso lead to cardiomyopathy, especially in patients with other chronic medicalillnesses MAT is most commonly seen in patients older than 50 years and thosewith medical conditions that lower the oxygen content of the blood, such asCOPD, lung cancer, bacterial pneumonia, or pulmonary embolism Treatment ofMAT is best accomplished by evaluation and treatment of the underlyingmedical conditions The S4 heart sound heard in this patient is due to forcefulatrial contraction against a noncompliant left ventricle, a common finding inpatients with COPD In this patient there is also increased afterload on the leftventricle due to chronic systemic hypertension, which becomes hypertrophiedand noncompliant over time

A 59-year-old male presents to a local emergency department with complaints ofchest pressure and pain The pain is located in his mid-chest and radiates to hisleft jaw area His blood pressure is 150/88 mm Hg, pulse is 122/min, respiratoryrate is 22/min, and temperature is 38.0°C (100.4°F) He appears anxious anddyspneic; his skin is cool and moist Cardiac auscultation reveals a regulartachycardic heart rate, with a third heart sound present immediately after S2.Chest radiograph is without significant abnormality; ECG reveals sinustachycardia and ST segment elevations in lead V1 - V4 He undergoes immediatepercutaneous coronary intervention with stent placement in the left anteriordescending artery Six hours after the procedure, the patient is recovering in theintensive care unit (ICU); his cardiac monitor shows wide and bizarre QRScomplexes with a heart rate of 90 beats per minute BP is 145/82 The patient iscomfortable and in no acute distress When asked, he denies palpitations or othersymptoms

This patient is experiencing an accelerated idioventricular rhythm Thisarrhythmia is generated by the ventricles and occurs when a normally suppressedventricular “pacemaker” begins to control cardiac rate instead of the SA or AVnodes Idioventricular rhythm is considered to be “accelerated” when the rate isgreater than 40 beats per minute (intrinsic ventricular rate) The most commonscenario for this to occur is after coronary reperfusion (restoration of flow toischemic cardiac tissue) as in the case vignette The condition is benign, usuallynot apparent to the patient, and does not require any treatment Normal SA nodefunction generally returns within 24 to 48 hours of reperfusion therapy ECGmonitoring is imperative, as the condition needs to be differentiated fromventricular tachycardia, which can convert to ventricular fibrillation, a life-threatening arrhythmia The rate of idioventricular rhythm is less than 120 beatsper minute and more commonly less than 100 beats per minute The usual rate ofventricular tachycardia is significantly higher than 120 beats per minute

A 66-year-old male is evaluated in a physician’s office for ongoing medical care

He has overall felt well, but checks his pulse after exercising and thinks he hasnoticed that his heart occasionally skips a beat He denies dizziness,lightheadedness, or fainting He is able to exercise without symptoms His bloodpressure is 144/72 mm Hg, pulse is 82/min, respiratory rate is 14/min, andtemperature is 36.0°C (96.8°F) Physical examination is without abnormality.ECG/cardiac rhythm strip is obtained and is significant for a repeating pattern ofprogressive PR interval prolongation over 2 to 4 beats, with a subsequent

“dropped” ventricular beat

This patient has second-degree AV heart block, Type I, also known as “Mobitz I”

or “Wenckebach periodicity.” This condition occurs due to abnormal condition atthe AV node of the heart, resulting in incomplete transmission of all atrialimpulses to the ventricles The pattern seen on ECG is described in the vignette– prolongation of PR intervals that progress until a single atrial impulse is notconducted through the AV node and a ventricular beat is thus “dropped.” The PRinterval then “resets” and the cycle repeats Atrial rhythm must be regular inorder for the condition to be properly diagnosed, or other atrial conditions must

be considered This condition does not usually cause significant symptoms andmost commonly requires no treatment This is in contrast to second-degree AVblock, Type II (also known as “Mobitz II”) in which nonconducted atrial activityoccurs in a much less predictable fashion (i.e., no PR interval prolongation andresetting) Second-degree AV block, Type II can rapidly progress to complete(third-degree) heart block and as such should be treated with cardiac pacemakertherapy Complete heart block occurs when atria and ventricles are beatingindependently of one another and must also be treated with an implanted cardiacpacemaker

A 17-year-old girl faints while involved in cheerleading at a football game.When she regains consciousness, she tells her mother that she felt her heartracing “again.” She has felt this way on several occasions and this has occurredwith increasing frequency over the past year She has no known chronic illness

This patient has ECG evidence of Wolff-Parkinson-White syndrome Thiscondition is a pre-excitation supraventricular tachycardia, which occurs when anabnormal accessory conduction pathway exists between atria and ventricles.Electrical signals can travel down this pathway (accessory bundle of Kent) andcause PVC Most common symptoms of this condition include paroxysmalepisodes of palpitations, dizziness, lightheadedness, and syncope or near-syncope Diagnosis of the condition is made with ECG evidence of a “delta”wave, which appears as an upward “slurring” of the QRS complex Also noted

on ECG is a short PR interval These two findings actually represent the aberrantatrial impulse exciting the ventricle prematurely, without normal AV nodalcontrol Sudden death in this condition is rare The condition is often seen inyoung people, and ablation of the accessory pathway will definitively treat thecondition

A 77-year-old female is evaluated in a physician’s office for symptoms offatigue, nausea/vomiting, and visual disturbances She states that she has beenhaving these symptoms for the past 2 weeks and has felt progressively worseover this time She has had fatigue, nausea, and emesis most days and hasrecently noticed that when she is looking at an object, often she will see a ring orhalo around that object She denies vision loss, headache, or hearing changes.She denies syncope Medical history is significant for congestive heart failuretreated with furosemide, lisinopril, and digitalis Her blood pressure is 122/66

mm Hg, pulse is 52/min, respiratory rate is 16/min, and temperature is 37.0°C(98.6°F) ECG is obtained and shows bradycardia with a prolonged PR interval

This patient has digitalis toxicity This condition occurs when serum levels ofdigitalis (used as a positive inotropic agent in some patients with congestiveheart failure) exceed a very narrow therapeutic range Symptoms of fatigue,nausea/emesis, abdominal pain, and agitation or confusion are common ECGabnormalities are frequently seen and are variable in appearance Paroxysmalatrial tachycardia with heart block, bradycardia, accelerated idioventricularrhythm, or ventricular tachycardia can occur The condition and resultingarrhythmias are exacerbated by low serum potassium levels Treatment withdigoxin immune Fab is preferred However, if immune Fab is not available,magnesium, phenytoin, and lidocaine may be used

A 52-year-old male with dialysis-dependent chronic renal disease reportssymptoms of malaise and general weakness He admits to not attending hisdialysis session 2 days ago His blood pressure is 196/110 mm Hg, pulse is88/min, respiratory rate is 20/min, and temperature is 37.7°C (99.9°F) Cardiacexamination is notable for frequent ectopy There is 1 + pitting edema of thepatient’s feet, ankles, and mid-calf area ECG is obtained and is shown here.Laboratory studies are ordered urgently and are pending

Source: Knoop KJ, Stack LB, Storrow AB, Thurman RJ: The Atlas of Emergency Medicine, 3rd Edition:

http://www.accessmedicine.com Copyright © The McGraw Hill Companies, Inc All rights reserved.

This patient has fluid overload and hyperkalemia Both of these conditionsresulted from missing scheduled dialysis in this patient with severelycompromised renal function He has accumulated fluid and toxins in his bodyand is in need of urgent dialysis Mild hyperventilation is noted – this is an effort

to compensate for metabolic acidosis, which occurs as a result of renalinsufficiency ECG findings are particularly worrisome for hyperkalemia in thispatient, which can cause fatal dysrhythmia ECG findings of moderatehyperkalemia include diminished amplitude of P waves and high (“peaked”) Twaves With more severe electrolyte imbalance, QRS widening and development

of a sine wave ECG appearance can occur Conduction abnormalities arise aselevated serum potassium levels exert direct effects on ventricular cellmembrane repolarization Management of this patient includes use ofintravenous calcium to stabilize membrane potentials and decrease arrhythmiarisk Other temporary strategies include IV administration of insulin (withglucose), bicarbonate, or beta-2 agonist medications to induce an intracellularpotassium shift and decrease serum potassium levels Emergent dialysis isrequired, however, to effectively manage this patient’s dangerous condition

A 68-year-old female is evaluated in the local emergency department forshortness of breath unresponsive to her usual medical therapy She has a history

of coronary artery disease with coronary artery bypass grafting (CABG) of fourcoronary vessels 8 years ago Several years ago, she was diagnosed with heartfailure due to decreased systolic function She has done fairly well on medicaltherapy until the past 2 months, when she has had progressive fatigue,orthopnea, and dyspnea requiring frequent use of oxygen She has alsoexperienced weight gain of more than 4.4 kg during this same time period Herblood pressure is 102/70 mm Hg, pulse is 123/min, respiratory rate is 24/min,and temperature is 36.7°C (98.1°F) Physical examination reveals a pale,diaphoretic female who appears dyspneic Cardiac examination is notable for alaterally displaced point of maximal impulse (PMI), a holosystolic murmur, and

S3 gallop Bibasilar rales are noted There is pitting edema of her bilateral lowerextremities with abdominal ascites Echocardiography reveals four-chambercardiac dilatation with global hypokinesis and an estimated LV ejection fraction

of 17%

This patient has end-stage dilated cardiomyopathy This condition of diseasedmyocardium with associated cardiac dysfunction is most commonly the result ofischemic heart disease (approximately 65% of cases) as in this patient Othercommon causes of dilated cardiomyopathy include toxins (alcohol) or viralinfections, inherited disorders of metabolism, neuromuscular disorders, or druginduced (cancer chemotherapeutic agents) Patients present in a decompensatedstate of inadequate perfusion, which occurs over time, commonly including fluidretention in peripheral extremities, abdomen, and pulmonary circulation Anarrowed pulse pressure is often noted, as in this patient, due to ineffectivesystolic contraction Her displaced PMI is due to the significantly dilated leftventricle, and the murmur appreciated is due to disruption of optimal flow withinthe failing heart The S3 gallop noted is the result of low ventricular compliance,

a result of long-standing cardiac ischemia All body systems are affected andmedical therapy is directed at reducing workload on the heart and removal ofexcess fluid Cardiac transplantation is the best option when the myocardium is

as badly damaged as described in this case

A 62-year-old female is evaluated in her physician’s office The patient has notedthat she has had some shortness of breath with exertion, which has increasedover the past several months She has also noticed some difficulty breathingwhen lying down and some swelling of her ankles bilaterally She has a history

of sarcoidosis diagnosed 11 year ago, which has primarily affected herpulmonary function and her eyes, requiring frequent courses of oral steroidtherapy Her blood pressure is 122/70 mm Hg, pulse is 88/min, respiratory rate is18/min, and temperature is 36.4°C (97.5°F) Physical examination reveals awell-appearing female in no acute distress Cardiac examination isunremarkable Bibasilar rales are noted as is mild pitting edema of her bilaterallower extremities ECG shows no abnormalities Echocardiography is orderedand shows biatrial enlargement, concentric ventricular hypertrophy with normalventricular chamber size, reduced ventricular filling, and moderate pulmonaryhypertension Estimated LV ejection fraction is 50%

This patient has restrictive cardiomyopathy This condition of diseasedmyocardium with associated cardiac dysfunction is most commonly the result ofinfiltrative systemic diseases such as amyloidosis or sarcoidosis Infiltration ofmyocardial tissue results in a “stiff” heart, which may hypertrophy in order tocompensate for decreased muscular compliance Most symptoms and decreasedperfusion are related to diastolic dysfunction in which stiffened ventricles areunable to relax completely, causing inadequate diastolic filling Patients usuallybecome symptomatic in a chronic fashion, as in the presented vignette Commonfindings including fluid retention in peripheral extremities and lungs as bothsides of the heart are affected (global infiltrative process) Theechocardiographic findings of four-chamber hypertrophy represent an attempt ofthe myocardium to compensate for decreased filling Pulmonary hypertension isthe result of chronically increased left heart filling pressures due to stiff cardiacmusculature Systolic function is affected in this patient as evidenced bydecreased LV ejection fraction Medical therapy is directed at reducing workload

on the heart and removal of excess fluid Ongoing treatment of underlyingmedical conditions (sarcoidosis) is essential Cardiac transplantation is the bestoption when medical therapy begins to fail

A 17-year-old male is evaluated in an emergency department after passing outwhile playing basketball He states that he was running down the court andbecame very short of breath and then does not remember anything else, otherthan waking up on the court a short time later He states that he has had similarepisodes of shortness of breath with exercise but has never passed out; when hehas become short of breath in the past, he has stopped playing and rested, but didnot do that this time He has no other significant medical history and no othersymptoms His blood pressure is 130/80 mm Hg, pulse is 106/min, respiratoryrate is 20/min, and temperature is 36.2°C (97.2°F) Cardiac examination shows anormally placed PMI, normal S1, S2, and a holosystolic murmur best appreciatedover the cardiac base The murmur increases in intensity when the patient isasked to perform a Valsalva maneuver Extremities are well-perfused, distalpulses are intact and equal bilaterally, lungs are clear, and there is no peripheraledema Echocardiography is ordered and shows a large hypertrophic cardiacseptum and asymmetric LV hypertrophy There is no LV outflow obstructionnoted at rest