A CASE OF HEART A CASE OF HEART FAILUREFAILURE

Dr.SRIRAM.SDr.SRIRAM.S

PROF.Dr.P.CHITRAMBALAM’S PROF.Dr.P.CHITRAMBALAM’S M5 UNITM5 UNIT

PATIENT PROFILEPATIENT PROFILE

35 years female35 years femaleAdmitted for Admitted for swelling of both legs-6 monthsswelling of both legs-6 monthsBreathlessness-4monthsBreathlessness-4monthsDecreased urine output-1monthDecreased urine output-1month

Swelling of both legs-insidious Swelling of both legs-insidious onset,slowly progressive,painless,initially onset,slowly progressive,painless,initially subsided with rest,but not relieved by rest subsided with rest,but not relieved by rest at presentat present

Breathlessness-grade 2 initially,now Breathlessness-grade 2 initially,now progressed to grade 4,not associated with progressed to grade 4,not associated with chest painchest pain

No h/o palpitations,syncopeNo h/o palpitations,syncopeh/o oliguria+h/o oliguria+h/o swelling of both hands+h/o swelling of both hands+h/o puffiness of face+h/o puffiness of face+

h/o early morning stiffness+ associated h/o early morning stiffness+ associated with pain over small joints of hands and with pain over small joints of hands and feetfeet

h/o intermittent abdominal distension+h/o intermittent abdominal distension+No h/o abd pain,bleeding tendenciesNo h/o abd pain,bleeding tendenciesNo h/o hair fall,oral ulcers,rashes No h/o hair fall,oral ulcers,rashes

anywhere,fever,photosensitivityanywhere,fever,photosensitivityNo h/o dysphagiaNo h/o dysphagiaBowel habits normalBowel habits normal

No past h/o similar illnessNo past h/o similar illnessNo h/o HT,DM,PT,IHD,RHD,BA in the pastNo h/o HT,DM,PT,IHD,RHD,BA in the pastFamily h/o-nil sigFamily h/o-nil sigMenstural h/o-normalMenstural h/o-normal

G/EG/E Pt conscious,oriented,afebrilePt conscious,oriented,afebrile Dyspnoeic,tachypnoeicDyspnoeic,tachypnoeic Tender and swollen small joints of both hands Tender and swollen small joints of both hands

and feet+and feet+ Taut skin over the face+Taut skin over the face+ b/l pitting pedal edema+b/l pitting pedal edema+ Puffiness of face+Puffiness of face+ JVP elevatedJVP elevated BP-130/80mmHgBP-130/80mmHg PR-106bpm,regPR-106bpm,reg RR-26cycles/min,regRR-26cycles/min,reg Temp-normalTemp-normal

SYSTEMSSYSTEMS

CVS-S1 S2+, S3+ no murmurCVS-S1 S2+, S3+ no murmurRS-NVBS+,b/l basal crepts+RS-NVBS+,b/l basal crepts+ABD-soft,tender hepatomegaly+ 2cm ABD-soft,tender hepatomegaly+ 2cm

below RCMbelow RCMCNS-no FNDCNS-no FND

CLINICAL DIAGNOSISCLINICAL DIAGNOSIS

CCFCCF

? Secondary to underlying ? Secondary to underlying

CTDCTD

INVESTIGATIONSINVESTIGATIONS

Hb-9.2Hb-9.2 TC-5200TC-5200 DC-P52 L42 E6DC-P52 L42 E6 ESR-12/25ESR-12/25 PCV-30%PCV-30% PLT-1LakhPLT-1Lakh MCV-65.2MCV-65.2 MCH-24.4MCH-24.4 MCHC-28.6MCHC-28.6

URINE Alb-neg,sugar-nil,URINE Alb-neg,sugar-nil, RBS-85mg%RBS-85mg% Bl urea-22Bl urea-22 Sr creat-0.8Sr creat-0.8 Sr sodium-140meq/lSr sodium-140meq/l Sr potassium-3.8meq/lSr potassium-3.8meq/l ECG-low voltage complexes,IVCD+ECG-low voltage complexes,IVCD+ Chest x-ray-cardiomegaly+Chest x-ray-cardiomegaly+ Echo-global LV hypokinesia+Echo-global LV hypokinesia+ severe LV systolic dysfn+severe LV systolic dysfn+ EF-22%,MR,TR-mild,PHT-moderateEF-22%,MR,TR-mild,PHT-moderate no pericardial effusionno pericardial effusion PFT-Restrictive patternPFT-Restrictive pattern

ANA-POSITIVE,1:100 DIL,SPECKLED PATTERNANA-POSITIVE,1:100 DIL,SPECKLED PATTERN RA factor,ASO-neg; CRP-+VE;6mg/lRA factor,ASO-neg; CRP-+VE;6mg/l Anti CCP-NEGAnti CCP-NEG Anti SCL 70-NEGAnti SCL 70-NEG 24 hours urine protein-280mg/day24 hours urine protein-280mg/day LFT-sr bilirubin-1mg%;sr total proteins-6.8LFT-sr bilirubin-1mg%;sr total proteins-6.8 TFT-T3-130ng/dl(60-200)TFT-T3-130ng/dl(60-200) T4-7.7micg/dl(4.5-12)T4-7.7micg/dl(4.5-12) TSH-4.46(0.3-5.5)TSH-4.46(0.3-5.5) aPTT 31.2aPTT 31.2 PT-18.2,INR-1.9PT-18.2,INR-1.9 HRCT chest-features s/o ILDHRCT chest-features s/o ILD PFT-restrictive patternPFT-restrictive pattern Anti U1RNP- 1:100 POSITIVEAnti U1RNP- 1:100 POSITIVE Sr.CPK-24Sr.CPK-24

Cardiologist opinion-severe lv dysfunctionCardiologist opinion-severe lv dysfunctionDermatology opinion-skin biopsy s/o Dermatology opinion-skin biopsy s/o

sclerodermasclerodermaRheumatologist opinion-to r/o MCTD,To Rheumatologist opinion-to r/o MCTD,To

do ENA PROFILEdo ENA PROFILE

TREATMENT GIVENTREATMENT GIVEN

Antifailure measuresAntifailure measuresAntiplatelet agentsAntiplatelet agentsChloroquineChloroquinePrednisolonePrednisolonePt developed dry gangrene of rt index Pt developed dry gangrene of rt index

finger in hospital.she was started on finger in hospital.she was started on heparin and warfarin.gangrene didn’t heparin and warfarin.gangrene didn’t spread and few days later there was spread and few days later there was autoamputation of gangrenous partautoamputation of gangrenous part

FINAL DIAGNOSISFINAL DIAGNOSIS

DCMP/CCF/RLD-SECONDARY TO DCMP/CCF/RLD-SECONDARY TO

OVERLAP SYNDROMEOVERLAP SYNDROME

PLASMA AUTOANTIBODIESPLASMA AUTOANTIBODIES

RA FACTORRA FACTOR 1.sjogrens-100%1.sjogrens-100% 2.felty’s-nearing 100%2.felty’s-nearing 100% 3.RA-70%3.RA-70% 4.Infections(endocarditis,hepatitis)<50%4.Infections(endocarditis,hepatitis)<50% 5.MCTD-50%5.MCTD-50% 6.SLE-<40%6.SLE-<40% 7.Systemic sclerosis-30%7.Systemic sclerosis-30% 8.normal-2 to10%8.normal-2 to10%

Rheumatoid Factor

Positive in 75% RA (range 26 – 90%) Negative RF dose not exclude diagnosis of RA Should order only if inflammatory joint

symptoms or signs of RA Low specificity in general population

(positive predictive value of less than 20%) False positives in 2-25% over 75 (Low titre) Not used to monitor response to therapy

Anti-citrullinated peptides (antiCCP)

Auto-antibodies that react with citrullinated proteins (post-translational modification of arginine residues) Sensitivity 60-80% Specificity >90% (RF specificity 70%) Present 70% cases RA, 5% controls Associated with erosive disease May predict RA in cases of early inflammatory arthritis

ANAANA

Homogenous,peripheral-SLEHomogenous,peripheral-SLESpeckled-MCTDSpeckled-MCTDNucleolar-systemic sclerosisNucleolar-systemic sclerosisCentromere-limited systemic sclerosisCentromere-limited systemic sclerosis

CAUSESCAUSES

SLE->95%SLE->95%Autoimmune hepatitis-75%Autoimmune hepatitis-75%Sjogrens syndrome-68%Sjogrens syndrome-68%Systemic sclerosis-64%Systemic sclerosis-64%RA -30%RA -30%JIA-1%JIA-1%Normal-0 to 2%Normal-0 to 2%

Anti histone antibody-drug induced SLEAnti histone antibody-drug induced SLEAnti ds DNA-SLE(60%,more specific than Anti ds DNA-SLE(60%,more specific than

ANA)ANA)Anti phospolipid Ab-APLA,SLEAnti phospolipid Ab-APLA,SLEANTI CENTROMERE Ab-limited syst ANTI CENTROMERE Ab-limited syst

sclerosissclerosis

ANTI ENA ANTIBODIESANTI ENA ANTIBODIES

Anti Ro(SSA)- SLE,sjogrens,systemic Anti Ro(SSA)- SLE,sjogrens,systemic sclerosissclerosis

Anti La(SSB)-sjogrens,SLE(15%)Anti La(SSB)-sjogrens,SLE(15%)Anti SM-SLE(20-30%)Anti SM-SLE(20-30%)Anti RNP-SLE,MCTDAnti RNP-SLE,MCTDAnti Jo,anti Mi2-Anti Jo,anti Mi2-

polymyositis,dermatomyositispolymyositis,dermatomyositisAnti scl 70- diffuse systemic sclerosisAnti scl 70- diffuse systemic sclerosis

AFFECTION OF HEART IN LUNG AFFECTION OF HEART IN LUNG DISEASEDISEASE

Lung disease causes hypoxia with cyanosis and Lung disease causes hypoxia with cyanosis and polycythemiapolycythemia

Hypoxia is sensed by kidneys and via carotid Hypoxia is sensed by kidneys and via carotid body,generating increases in sympathetic activity and body,generating increases in sympathetic activity and renal vasoconstrictionrenal vasoconstriction

Increased sympathetic activity leads to salt and water Increased sympathetic activity leads to salt and water retentionretention

This extra salt and water is mainly held in capacitance This extra salt and water is mainly held in capacitance vessels with raised JVPvessels with raised JVP

If vascular permeability raises(particularly when PaCO2 If vascular permeability raises(particularly when PaCO2 rises producing peripheral vasodilation and increase in rises producing peripheral vasodilation and increase in capillary pressure)etra fluid accumulates independent capillary pressure)etra fluid accumulates independent tissues mainly anklestissues mainly ankles

A raised JVP and ankle edema in this A raised JVP and ankle edema in this setting are not due to impaired right setting are not due to impaired right ventricular function,but fluid overload and ventricular function,but fluid overload and increased vascular permeabilityincreased vascular permeability

LEFT VENTRICULAR CHANGES LEFT VENTRICULAR CHANGES IN ILDIN ILD

pulmonary hypertension may be the reason for changes in left ventricular (LV) geometry due to an enlarged and overloaded right ventricle

However,ventricular overload may be caused by an increase in pressure or volume, or a combination of both.

Resting LV systolic function is more severely depressed in patients with right ventricular (RV) volume overload compared with patients with RV pressure overload.

Most studies have demonstrated that the underfilled left ventricle found in RV pressure overload conditions, such as idiopathic pulmonary fibrosis (IPF), has relatively preserved systolic function

Increasing RV pressure by pulmonary artery constriction caused regional changes in systolic shortening in the anterior, posterior, and lateral walls of the left ventricle and the septum.

Similar to RV volume overload, RV pressure overload distorts left ventricular end-diastolic geometry.

In contrast to RV volume overload, with RV pressure overload,the left ventricle does not return to its normal shape in systole

Angiographic and echocardiographic data from patients with chronic pulmonary hypertension have also shown abnormalities in septal geometry and motion

LV systolic dysfunction, rarely present, is often due to coexisting severe right-sided heart failure with pulmonary vascular disease

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