Case Report Addison s Disease and Dilated Cardiomyopathy ...downloads.hindawi.com/journals/cric/2016/4362514.pdf · presented with a new onset dilated cardiomyopathy due to Addison

Case ReportAddison’s Disease and Dilated Cardiomyopathy: A Case Reportand Review of the Literature

Viktoriya Mozolevska,1 Anna Schwartz,1 David Cheung,1 Bilal Shaikh,1

Kapil M. Bhagirath,2 and Davinder S. Jassal1,3,4

1 Institute of Cardiovascular Sciences, St. Boniface General Hospital, University of Manitoba, Winnipeg, MB, Canada2Section of Cardiology, Surrey Memorial Hospital, University of British Columbia, Vancouver, BC, Canada3Section of Cardiology, Department of Internal Medicine, University of Manitoba, Winnipeg, MB, Canada4Department of Radiology, St. Boniface General Hospital, University of Manitoba, Winnipeg, MB, Canada

Correspondence should be addressed to Davinder S. Jassal; [email protected]

Received 15 September 2016; Accepted 31 October 2016

Academic Editor: Takatoshi Kasai

Copyright © 2016 Viktoriya Mozolevska et al. This is an open access article distributed under the Creative Commons AttributionLicense, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properlycited.

Addison’s disease is often accompanied by a number of cardiovascular manifestations.We report the case of a 30-year-old man whopresented with a new onset dilated cardiomyopathy due to Addison’s disease. The clinical presentation, treatment, and outcomesof this rare hormone mediated cardiac disorder are reviewed.

1. Introduction

Addison’s disease, also known as primary adrenal insuffi-ciency, is associated with a decreased production of gluco-corticoid and mineralocorticoid hormones from the adrenalcortex [1]. Although autoimmune adrenalitis is consideredto be the major cause of Addison’s disease in up to 90% ofdiagnosed individuals, prevalent in female patients between30 and 50 years of age, other etiologies include infectious,drug induced, and/or genetic factors [2, 3]. Common man-ifestations of this condition are hyponatremia, hyperkalemia,and/or hypoglycemia along with mucosal and skin hyperpig-mentations [2, 3]. Although cardiovascular manifestations ofAddison’s disease include hypotension, syncope, and arrhyth-mias, the development of a dilated cardiomyopathy and heartfailure are an uncommon life-threatening complication [4–8]. We describe a rare case of a 30-year-old male with adilated cardiomyopathy in the context of Addison’s diseaseand review all 6 adult cases of this cardiac manifestationpublished to date [4–8].We evaluate the clinical presentation,treatment, and outcomes of these cases and discuss the diag-nosis, pathophysiology, and treatment of this rare hormonemediated cardiac disorder.

2. Case Report

A 30-year-old previously healthy male presented to theemergency department with constitutional symptoms ofweakness, fatigue, dizziness, and anorexia. A baseline EKGand chest X-ray were within normal limits. He was diagnosedwith primary adrenal insufficiency presenting with biochem-ical evidence of high ACTH (>278 pmol/L) and low cortisollevels (32 nmol/L) and started on prednisone 5mg po od andfludrocortisone 0.1mg po od. Two weeks later, he presentedwith a 5-day history of orthopnea and increasing dyspneaon minimal exertion. On presentation, the blood pressurewas 104/74mm Hg with a heart rate of 90 beats per minute.The jugular venous pressure was elevated at the angle of thejaw with displacement of the cardiac apex and normal heartsounds. There were diminished breath sounds bilaterallyin both lower lung fields without evidence of peripheraledema.The serum sodiumwasmildly reduced at 132mmol/L(normal range: 135–145mmol/L) while the potassium wasincreased at 5.6mmol/L (normal range: 3.5–5.0mmol/L).There was a markedly elevated N-terminal brain natriureticpeptide of 4515 pg/mL (normal range<125 pg/mL), consistentwith clinical and biochemical evidence of acute heart failure.The serum TSH, urine toxicology, and metanephrines were

Hindawi Publishing CorporationCase Reports in CardiologyVolume 2016, Article ID 4362514, 5 pageshttp://dx.doi.org/10.1155/2016/4362514

2 Case Reports in Cardiology

Table1:Summaryof

adultcaser

eportsof

Addison’s

diseasea

nddilated

cardiomyopathy.

Case

(reference)

Repo

rtyear

Age/sex

CVmanifestations

LVEF

(%)

Treatm

ent

Outcome

Cushnere

tal.

[4]

1963

53/M

Dyspn

ea,ortho

pnea,LV

enlargem

ent,pu

lmon

ary

edem

a,andrig

htpleural

effusion

N/A

(1)M

erallurid

e,antic

oagu

lationtherapy,

quinidine,digitalis

(2)A

CTH,20un

its(3)9

-alpha-Fluoroh

ydrocortiso

ne,0.2mg3tim

esdaily

for7

days

priortodeath

Day

20—decrease

inpu

lmon

arycongestio

nDay

31—patie

ntdied

Bhattacharyya

andTy

mms[5]

1998

47/F

Edem

a,dyspnea,

tachypnea,lung

crepitatio

ns,cardiom

egaly,

andpu

lmon

arycongestio

n

N/A

(1)IVfurosemidefor

6days

(2)F

ludrocortison

edosaged

ecreased

by50%

(from

30mgdaily)a

ndthen

stopp

edaft

er2days

(i)CH

Fsymptom

sresolved

(ii)A

ddiso

nian

crisisa

t8mon

ths,tre

ated

with

hydrocortison

eand

fludrocortison

e(iii)At

12mon

ths,patie

ntwas

doingwell

AfzalandKhaja

[6]

2000

36/M

Dyspn

ea,fatigue,dizziness,

malaise,and

tachycardia

25%

(1)C

ortic

osteroid

therapy

(2)N

otre

atmentfor

CHF

(i)At

7weeks,LVEF

improved

to55%

Wolffetal.[7]

2007

42/F

Hypotensio

n,tachycardia,

respira

tory

failu

re,bilateral

pulm

onaryedem

a,and

cardiogenics

hock

30%

(1)IVhydrocortison

eloading

dose

100m

gand

thereafte

r10m

g/h

(2)M

echanicalventilationandcontinuo

usno

repineph

rinea

t4.4𝜇g/kg

min

(3)A

tdisc

harge,oralhydrocortison

eand

fludrocortison

e,sta

ndard

(i)At

discharge,theL

VEF

improved

to52%

with

noevidence

ofwall-m

otion

abno

rmalities

Krish

namoo

rthy

etal.[8]

2013

21/M

Nausea,weakn

ess,

progressived

yspn

ea,

asystolic

cardiaca

rrest,

peric

ardialeffusion,

severe

biventric

ular

failu

re,and

cardiogenics

hock

N/A

(1)T

andemHeartim

plantatio

n(2)IVhydrocortison

eloading

dose

100m

gand

thereafte

r50m

g/8h

(3)W

eanedto

physiologicd

oseo

foral

hydrocortison

ebydischarge

(4)2

mon

thsa

fterd

ischarge,mineralocortic

oid

replacem

entfor

hypo

tension

(i)At

2weeks

after

RVADandLV

AD

removal,n

ormalbiventric

ular

functio

n(ii)A

t2mon

ths,remainedwell

Case Reports in Cardiology 3

Table1:Con

tinued.

Case

(reference)

Repo

rtyear

Age/sex

CVmanifestations

LVEF

(%)

Treatm

ent

Outcome

Mozolevskae

tal.

2016

30/M

Ortho

pnea,dyspn

ea,

elevated

jugu

larv

enou

spressure,elevatedBN

P,dilated

LV,severes

ystolic

dysfu

nctio

n,andbilateral

pleuraleffu

sions

15%

(1)IVfurosemide,40

mgoralatdischarge

(2)R

amipril

5mg

(3)B

isoprolol10mg

(4)F

ludrocortison

edosaged

ecreased

by50%

(from

0.1m

gdaily),prednisone

unchangedat

5mgdaily

(i)LV

EFim

proved

to44

%

LV,left

ventric

le;IV,intravenou

s;CH

F,congestiv

eheartfailu

re;LVEF,left

ventric

ular

ejectio

nfractio

n;ICU,

intensivec

areu

nit;RV

AD,right

ventric

ular

assis

tdevice;LV

AD,left

ventric

ular

assis

tdevice;BN

P,brain

natriuretic

peptide.

4 Case Reports in Cardiology

within normal limits. A 12-lead EKG demonstrated poor R-wave progression across the precordial leads with evidenceof a left anterior fascicular block. Chest X-ray findings wereconsistent with bilateral pleural effusions, vascular redistri-bution, and interstitial edema. Transthoracic echocardiogra-phy (TTE) confirmed a dilated left ventricle (LV), severe LVsystolic dysfunction with an ejection fraction of 15%, andmoderate functional mitral regurgitation. Cardiac magneticresonance imaging with late gadolinium enhancement wasnormal with no evidence of a viralmyocarditis nor infiltrativecardiomyopathy. Cardiac catheterization demonstrated nor-mal coronaries consistentwith the diagnosis of a nonischemicdilated cardiomyopathy.A computed tomographic scan of theabdomen revealed hyperdense adrenal glands with centralnecrotic areas of hypoattenuation and peripheral enhance-ment, consistent with the recent diagnosis of primary adrenalinsufficiency. With a new diagnosis of acute heart failure dueto primary Addison’s disease, the patient was admitted formedical treatment with parenteral diuretics, ACE inhibition,beta blockade, and a decrease in the fludrocortisone dosage(thatwas started 2weeks before). A 6-month follow-upmulti-gated acquisition scan (MUGA) revealed an improvement inLV ejection fraction to 40–45%.

3. Discussion

With an incidence of approximately 120 cases per millionin the population, Addison’s disease is a rare long termendocrine disorder in which the adrenal glands produceinsufficient steroid hormones, including glucocorticoids andmineralocorticoids [1–3, 9].The clinical presentation ofAddi-son’s disease encompasses a multitude of systemic symptomsincluding fatigue, weight loss, salt craving, and joint andback discomfort [1–3, 9]. Individuals with primary adrenalinsufficiency may also experience a number of cardiovas-cular symptoms including hypotension, arrhythmias, andcongestive heart failure [4–8]. In fact, the development of adilated cardiomyopathy due to Addison’s disease is extremelyrare [4–8]. Although the exact etiology of heart failuredue to Addison’s disease remains unknown, a number ofpotential mechanisms include (i) reduced effects of corticalhormones on the LV myocardium; (ii) poor alimentationwith low glycogen reserves; (iii) hemoconcentration, lowblood volume, and reduced coronary blood flow; and (iv)disturbances in electrolyte levels [9].

After a systematic review of the literature of all adultcases of Addison’s disease and dilated cardiomyopathy, atotal of 6 published cases (including the current report)were identified as shown in Table 1 [4–8]. The mean agewas 38 years (range 21–53) with a male to female ratio of2 : 1. Common cardiovascular features at presentation werehypotension, dyspnea, tachycardia, and pulmonary edema.Our case was unique in that the patient developed bilateralpleural effusions and the lowest LVEF as measured by TTE.Moreover, our case was only the second in which a patientdeveloped symptoms of acute CHF following administrationof hormone replacement therapy (HRT) and the one with theshortest time lapse between the two events of only 6 days(Table 1) [4–8].

In patients with Addison’s disease, dual HRT with glu-cocorticoids and mineralocorticoids is a necessity [1–3, 10].Although oral hydrocortisone and fludrocortisone are themainstay of treatment in this patient population [1–3, 10],the use of these medications may need to be altered in thesetting of a concomitant heart failure crisis. In particular,fludrocortisone corrects hypotension by increasing sodiumretention, thereby increasing systemic afterload. However, inthe presence of a dilated cardiomyopathy and reduced LVEF,increased afterload by fludrocortisone may precipitate acutecongestive heart failure [4–8]. In individuals with Addison’sdisease who experience acute cardiovascular symptoms dueto the development of a dilated cardiomyopathy, the dosageof fludrocortisone may need to be decreased or stopped alto-gether in order to correct the acute heart failure syndrome.In 4 of the 6 cases reviewed in Table 1, the patients wereable to tolerate a carefully balanced dose of fludrocortisoneafter appropriate treatment of the underlying heart failuresymptoms with medical therapy including diuretics, ACEinhibition, and beta blockade [4–8]. These agents, whileappropriate for acute heart failure in the setting of a reducedLVEF, may interfere with the effectiveness of fludrocorti-sone in correcting hormonal deficiency, necessitating carefulhemodynamicmonitoring of the patient’s condition through-out the treatment period.

4. Conclusion

Amultidisciplinary approach must be sought in the manage-ment of patients with dilated cardiomyopathy secondary toAddison’s disease. HRT dosage should be optimized, heartfailure therapy should be initiated, and patients should beclosely monitored for future cardiovascular complications.

Competing Interests

No commercial relationship existed in the form of financialsupport or personal financial interest in relation to any partof the manuscript.

References

[1] L. K. Nieman and M. L. Chanco Turner, “Addison’s disease,”Clinics in Dermatology, vol. 24, no. 4, pp. 276–280, 2006.

[2] S. Bensing, A. Hulting, E. S. Husebye, O. Kampe, and K. Løvas,“Management of endocrine disease: epidemiology, quality of lifeand complications of primary adrenal insufficiency: a review,”European Journal of Endocrinology, vol. 175, no. 3, pp. R107–R116, 2016.

[3] E. Charmandari, N. C. Nicolaides, and G. P. Chrousos, “Adrenalinsufficiency,”TheLancet, vol. 383, no. 9935, pp. 2152–2167, 2014.

[4] G. B. Cushner, S. F. Zahler, and A. G. Hills, “UntreatedAddison’s disease complicated by pulmonary congestion due toleft ventricular failure,” Annals of Internal Medicine, vol. 58, pp.341–346, 1963.

[5] A. Bhattacharyya and D. J. Tymms, “Heart failure with fludro-cortisone in Addison’s disease,” Journal of the Royal Society ofMedicine, vol. 91, no. 8, pp. 433–434, 1998.

Case Reports in Cardiology 5

[6] A. Afzal and F. Khaja, “Reversible cardiomyopathy associatedwith Addison’s disease,” Canadian Journal of Cardiology, vol. 16,no. 3, pp. 377–379, 2000.

[7] B.Wolff, K.Machill, I. Schulzki, D. Schumacher, andD.Werner,“Acute reversible cardiomyopathy with cardiogenic shock ina patient with Addisonian crisis: a case report,” InternationalJournal of Cardiology, vol. 116, no. 2, pp. e71–e73, 2007.

[8] A. Krishnamoorthy, R. J. Mentz, K. A. Hyland et al., “A crisisof the heart: an acute reversible cardiomyopathy bridged torecovery in a patientwith addison’s disease,”ASAIO Journal, vol.59, no. 6, pp. 668–670, 2013.

[9] I. I. Goodof and C. M. Macbryde, “Heart failure in Addison’sdisease with myocardial changes of potassium deficiency,” TheJournal of Clinical Endocrinology &Metabolism, vol. 4, no. 1, pp.30–34, 2008.

[10] W. Arlt and B. Allolio, “Adrenal insufficiency,” The Lancet, vol.361, no. 9372, pp. 1881–1893, 2003.

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Case Report Addison s Disease and Dilated Cardiomyopathy ...downloads.hindawi.com/journals/cric/2016/4362514.pdf · presented with a new onset dilated cardiomyopathy due to Addison