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Systemic disease involving
cardiovascular system
Presented by
Dr. Md. Ahasanul Kabir
Resident, Phase B
Department of Cardiology, BSMMU
Chairperson:
Assoc. Prof. Dr. Mukhlesur
Rahman
UCC, BSMMU
Systems commonly involve
ī‚§ Rheumatic disease
ī‚§ Endocrine disease including DM
ī‚§ Renal disease – CKD
ī‚§ Respiratory disease
ī‚§ Neurologic disease
Common Cardiovascular Manifestations
of Systemic Autoimmune Diseases
Disease Sex
Distributi
on
Cardiovascular
Manifestations
Rheumatoid
arthritis
F>M o Pericarditis
o Coronary artery disease
o Cardiomyopathy
o Congestive heart failure
Systemic lupus
erythematosus
F>M o Pericarditis
o Libman-Sacks endocarditis
o Coronary artery disease,
o Hypertension
Inflammatory
myopathies
F>M o Pericarditis
o Conduction system abnormalities
o Congestive heart failure
o Myocarditis
Common Cardiovascular Manifestations
of Systemic Autoimmune Diseases
Disease Sex
Distributi
on
Cardiovascular
Manifestations
Systemic sclerosis F>M o Pulmonary hypertension,
o Pericarditis
o Cardiomyopathy
o conduction system disease
Seronegative
spondyloarthropathy
M>F o Aortitis,
o Conduction system disease
Accelerated Atherosclerosis
in Rheumatic Diseases
īļ Association between inflammatory diseases and
accelerated atherogenesis – eg RA & SLE.
īļ Ankylosing spondylitis, psoriatic arthritis, TA, and
APS - associated with premature atherosclerosis.
Accelerated Atherosclerosis
in Rheumatic Diseases
īļ Should consider an underlying inflammatory disease in
young patients with otherwise unexplained angina,
myocardial infarction, or stroke.
īļ Pts with a rheumatic disease who suffer a myocardial
infarction have worse outcomes in terms of both heart
failure and mortality than does the age-matched
general population.
Endothelial Dysfunction and Vascular
Injury
īļ Prolonged systemic inflammation such as that
seen in RA and SLE may promote endothelial
injury, increased endothelial apoptosis, and
endothelial vasodilator dysfunction.
īļ Inflammation may exacerbate the effects of
classic risk factors
Endothelial Dysfunction and Vascular
Injury
īļ Compared with the general population, patients
with systemic inflammatory diseases more
commonly exhibit endothelial dysfunction and
increased aortic stiffness.
īļ Systemic inflammatory environment may
predispose to increased plaque instability and
rupture
Molecular mechanisms for atherosclerotic
disease and cardiovascular events.
īļ Proinflammatory cytokines – TNF-Îą, IL-1 & IL-6 effects on endothelial
activation, leukocyte adhesion, endothelial injury, and permeability.
īļ ↑ endothelial cell apoptosis and ↓ capacity for repair may contribute.
īļ Others:
īƒŧ Autoantibodies (e.g antiphospholipid antibodies)
īƒŧ CD4 + CD28− cytotoxic T cells
īƒŧ Th17/TREG imbalance
īƒŧ Complement deficiency or excessive activation
īƒŧ Genetic polymorphisms
īƒŧ Deleterious effects of drugs, including corticosteroids & cyclosporine
Rheumatoid Arthritis
īļ A variety of studies have shown subclinical arterial disease with
increased carotid intimal-medial thickness (IMT) and early
plaque development.
īļ A recent study of microvascular and macrovascular function in
RA has suggested that the classic cardiovascular risk factors may
influence endothelial function more than disease-related
inflammation does.
īļ The direct effect of chronic inflammation on vascular
endothelium may itself promote atherogenesis, in addition to
exacerbating the actions of traditional cardiovascular risk
factors.
Rheumatoid Arthritisâ€Ļ
īļ Patients with RA have increased classic risk
factors for atherosclerosis.
īļ Tobacco smoking is associated with both
cardiovascular risk and the development of RA.
īļ Insulin resistance and the metabolic syndrome are
more common in RA.
Atherosclerotic Disease in Rheumatoid
Arthritis
īļ High TG levels and low levels HDL & LDL cholesterol
īļ Risk for MI in patients with RA similar to that in those with
DM and women with RA are twice as likely as age-matched
controls in the general population
īļ Patients with RA who suffer a MI are less likely to receive
acute reperfusion therapy and secondary preventive
measures and thus have worse outcomes.
Systemic Lupus Erythematosus
o Pericarditis
o Libman-Sacks endocarditis
o Coronary artery disease
o Hypertension
Pericarditis
īļ The most common cardiovascular manifestation of
SLE is pericarditis,
īļ Up to 42% of pts in echo shows effusion.
īļ May be detected at any point in the disease and are
usually asymptomatic and small.
Pericarditisâ€Ļ
īļ Acute pericarditis may occur - 20% to 30% of
patients
īļ May be associated with cardiac tamponade;
īļ Chronic pericarditis may occasionally lead to
constriction
Systemic Lupus Erythematosus
īļ Studies suggested an ↑ risk for MI and stroke in patients with
SLE(Between 2-fold and 10-fold and up to 50-fold greater than
general population)
īļ Young age of patients with SLE and CVS disease (67% of female
patients with SLE and a first cardiac event are typically
initially seen before 55 years of age) suggests that SLE
accelerates arterial disease.
īļ Worse outcomes following MI than the age-matched general
population with a higher risk of cardiac failure and increased
mortality.
Systemic Lupus Erythematosus
īļ HTN common in SLE - presence of renal disease and
the use of glucocorticoids
īļ Commonly have metabolic syndrome - associated
with renal impairment, higher corticosteroid doses,
and Korean or Hispanic ethnicity.
īļ Lipid abnormalities - including high levels of VLDL
and TG, elevated or normal LDL cholesterol, and
reduced HDL cholesterol.
Coronary Artery Involvement
and the Rheumatic Diseases
Premature Atherosclerosis 1. Systemic lupus erythematosus
2. Rheumatoid arthritis
3. Ankylosing spondylitis
4. Psoriatic arthritis
5. Gout
6. Takayasu arteritis
7. Giant cell arteritis
Coronary Arteritis 1. Systemic lupus erythematosus
2. Takayasu arteritis
3. Kawasaki disease
4. Churg-Strauss syndrome
5. Polyarteritis nodosa
6. Granulomatous polyangiitis
7. Rheumatoid arthritis
Cardiovascular Disease in the
Systemic Vasculitides
VASCULITIDES CARDIOVASCULAR COMPLICATIONS
Large-Vessel Vasculitis
Giant cell arteritis 1. Thoracic/abdominal artery aneurysm,
2. Limb ischemia
3. Pericarditis
4. Coronary arteritis
5. IHD
6. MI
Takayasu arteritis Aortic regurgitation
Limb ischemia
Aortic stenosis
Aortic aneurysm
Stroke
Hypertension
Coronary arteritis and aneurysm
IHD & MI,
Myocarditis
Cardiac failure
Cardiovascular Disease in the
Systemic Vasculitides
Kawasaki disease 1. Coronary artery aneurysm
2. MI
3. Myocarditis
4. Pericarditis
5. Valvular dysfunction,
6. Cardiac failure
Cardiovascular Disease in the
Systemic Vasculitides
Medium-Vessel Vasculitis CARDIOVASCULAR COMPLICATIONS
Churg-Strauss syndrome o Myocarditis
o Pericarditis
o coronary arteritis,
o Cardiomyopathy
o cardiac fibrosis
o Valvular dysfunction
o MI
Polyarteritis nodosa o Myocarditis
o Pericarditis
o coronary arteritis,
o coronary aneurysm
o Hypertension
o cardiac failure
Cardiovascular Disease in the
Systemic Vasculitides
Medium-Vessel Vasculitis CARDIOVASCULAR COMPLICATIONS
Granulomatous polyangiitis o Myocarditis
o Pericarditis
o Coronary arteritis
o Valvular heart disease
o Cardiac failure
Microscopic polyangiitis o Pericarditis
o Coronary microaneurysm
o MI
Giant Cell Arteritis
īļ Severe cardiovascular complications - dissecting
thoracic aortic aneurysms.
īļ Imaging and autopsy studies - aortitis and aortic wall
thickening are frequent in GCA
īļ ↑ FDG uptake in the thoracic aorta associated with an
↑ risk for aortic dilation.
īļ 17-fold increased risk for thoracic aortic aneurysms.
Giant Cell Arteritis
īļ Annual thoracic aortic screening for those with
FDG-PET–positive thoracic aortic uptake or
magnetic resonance angiography (MRA) or
computed tomography angiography (CTA)
evidence of aortic wall thickening
īļ Every 2 to 3 years in the remainder of patients.
Giant Cell Arteritisâ€Ļ.
īĩ Other CVS complications:
īƒ˜ Pericarditis
īƒ˜ Coronary arteritis
īƒ˜ Limb ischemia
īƒ˜ Accelerated atherosclerosis
īƒ˜ Myocardial infarction
īƒ˜ Cerebrovascular accidents
Takayasu Arteritis
īļ Vascular complications:
o Cerebral, internal organ and limb ischemia,
o Aneurysms
o PAH
Takayasu Arteritis
īļ Cardiac complications:
o Aortic valve insufficiency
o Accelerated atherosclerosis,
o Cardiac ischemia,
o Myocardial infarction, and
o Heart failure.
Takayasu Arteritis
īƒ˜ Neither MRA nor 18F-FDG-PET-CT reliably identifies
coronary arteritis.
īƒ˜ Coronary disease is often asymptomatic
īƒ˜ Silent myocardial injury in 27% of a cohort
īƒ˜ ↑ risk from secondary accelerated atherosclerosis.
Takayasu Arteritisâ€Ļ
īƒ˜ Thallium stress scintigraphy revealed myocardial
perfusion defects in 53%,
īƒ˜ intra-arterial angiography has shown that up to
30% have coronary artery lesions typically
affecting the ostia and proximal segments, with the
left main coronary artery being most commonly
affected.
Takayasu Arteritis
īƒ˜ Inflammation of the ascending aorta → coronary
artery involvement
īƒ˜ Dilation of the aortic root → AR
īƒ˜ LV dysfunction up to 20% and is due to
myocarditis, ischemic heart disease, and
hypertension
īƒ˜ Common finding often associated with renal artery
stenosis in TA.
Kawasaki Disease
īą Cardiovascular complications:
īƒ˜ Coronary artery aneurysm( 25% of untreated patient)
īƒ˜ Sudden death – MI following acute coronary thrombosis or rupture of
a coronary artery aneurysm.
īƒ˜ Pericarditis
īƒ˜ Pericardial effusion
īƒ˜ Myocarditis
īƒ˜ Valvular dysfunction
īƒ˜ Heart failure
īƒ˜ Peripheral arterial involvement (limb, renal & visceral arteries) – less
common
Churg-Strauss Syndrome
īƒ˜ Of all the vasculitides, CSS is the most likely to be
associated with severe cardiac disease
īƒ˜ Cardiac involvement complicates up to 60% of
cases.
īƒ˜ Cardiac complications:
o Pericarditis
o Myocarditis
o coronary arteritis
o myocardial infarction,
o cardiac fibrosis,
o arterial thrombosis, and
o valvular dysfunction.
Churg-Strauss Syndrome
īƒ˜ Cardiac disease - prominent cause of death.
īƒ˜ Cardiomyopathy - result of ischemia secondary to arteritis of
intramyocardial arteries or, less frequently, the epicardial
coronary arteries.
īƒ˜ Myocarditis is associated with eosinophilic infiltration, fibrosis,
and occasionally, granulomatous infiltration.
īƒ˜ Myocarditis can be life threatening and may result in the
development of restrictive, congestive, or dilated
cardiomyopathy.
Polyarteritis Nodosa
īƒ˜ Cardiac involvement often subclinical and clinically
apparent in only 10% of patients.
īƒ˜ CCF most commonly seen and consequence of a
specific myocarditis or coronary arteritis.
īƒ˜ 5% of pts – Pericarditis, supraventricular tachycardia
and valvular disease.
īƒ˜ CAG may reveal coronary artery microaneurysms,
coronary arteritis, or coronary spasm.
PERICARDITIS
īļ Pericarditis commonly complicates the
autoimmune connective tissue diseases,
particularly SLE, SSc, and RA.
īļ Clinically significant pericarditis develops in
fewer than 30% of patients.
īļ Diagnosed by echocardiography, which detects
pericardial thickening or small effusions in up to
50% of these patients.
PERICARDITIS
īļ CMR can also provide accurate definition of the extent
of pericardial involvement.
īļ In SLE - pericarditis is usually associated with a flare
of disease and often with polyserositis.
īļ Clinically significant pericarditis affects only 1% to 2%
of patients with RA, more commonly male,
seropositive patients.
MYOCARDITIS
īļ Myocarditis is a rare but recognized cause of
mortality in patients with autoimmune rheumatic
diseases and is most commonly seen in patients
with SLE, SSc, and polymyositis or
dermatomyositis.
īļ Myocarditis is also rarely associated with other
rheumatic diseases, including ankylosing
spondylitis, adult Still disease, GCA, and TA.
VALVULAR HEART DISEASE
īļ Clinically significant valvular disease can complicate
many rheumatic diseases.
īļ Mechanisms - direct damage to cardiac valve leaflets or
aortic valve regurgitation as a consequence of aortitis
affecting the ascending aorta.
VALVULAR HEART DISEASEâ€Ļ.
īļ In SLE pt:
o Verrucous endocarditis (Libman-Sacks endocarditis) and
nonspecific valvular thickening are most commonly
detected.
o Valvulitis with rapid valvular dysfunction
o Libman-Sacks lesions typically affects both valve
surfaces, most commonly the mitral valve.
VALVULAR HEART DISEASE
īą Seronegative Spondyloarthropathies:
īļ Aortic valvulitis leads to aortic cusp thickening and
retraction and subsequently to symptomatic aortic
regurgitation, which may cause heart failure.
īļ Proximal aortitis affecting the ascending aorta leads to
aortic root thickening and subsequently to dilation and
aortic regurgitation, the prevalence of which is related
to disease duration.
VALVULAR HEART DISEASE
īą In RA pts:
īļValvular thickening is commonly associated
with RA in echo
īļEcho- shows mitral valve involvement, with
valve thickening, asymptomatic mitral
regurgitation, and prolapse being the
predominant findings.
VALVULAR HEART DISEASE
īą In TA:
o Cardiac valve dysfunction commonly complicates
TA
o Inflammation of the ascending aorta predisposes
to dilation of the aortic root with subsequent
aortic valve regurgitation.
CARDIAC CONDUCTION DISTURBANCES
īą IN SLE pt:
īļ Adult SLE seldom causes primary conduction abnormalities
or rhythm disturbance, which may instead result from
underlying ischemic heart disease or myocarditis.
īļ Female patients with SLE or SjÃļgren syndrome who test
positive for antibodies against the Ro and/ or La antigens
carry the risk of bearing a child with congenital heart
block, which may be complicated by myocarditis.
īļ These antibodies may cross the placenta and induce
myocardial inflammation and can target the conduction
system and lead to fibrosis
CARDIAC CONDUCTION DISTURBANCES
īą In SS pt:
īļ affects up to 50% of patients.
īļ Cause- patchy myocardial fibrosis → disruption
of the conduction pathways.
īļ Supraventricular arrhythmias are usually benign
and amenable to treatment.
īļ Ventricular conduction abnormalities also
frequently occur in SSc and may impair
myocardial function.
īļ In these patients ventricular ectopy is common
and closely associated with sudden death.
CARDIAC CONDUCTION DISTURBANCES
īą Spondyloarthropathies
īļ Frequently complicate the HLA-B27– related
spondyloarthropathies.
īļ In AS up to 30% of patients experience conduction
system disease,
īļ Cause - subaortic fibrosis extending into the septum
and affecting AV node.
īļ Atrioventricular conduction block occurs commonly
and may become complete.
CARDIAC CONDUCTIONâ€Ļ.
īą Polymyositis and Dermatomyositis:
īļLeft anterior hemiblock and right bundle
branch block occur most frequently and
occasionally progress to complete heart
block.
īļCauses: Inflammation and fibrosis affect the
conduction pathways
CARDIAC CONDUCTIONâ€Ļ.
īą RA patients:
īļECG screening studies - arrhythmias or
conducting system abnormalities in up to 50%
īļCause: myocarditis and amyloid deposition in
the heart can cause atrioventricular node
conduction block.
īļSimilarly, rheumatoid nodules may disrupt
the conduction system and cause all types of
conduction abnormality.
PULMONARY ARTERIAL HYPERTENSION
īƒ˜ PAH can result from the connective tissue diseases
and is of concern to rheumatologists as a
significant cause of premature mortality.
īƒ˜ Often manifested late or goes undiagnosed.
īƒ˜ Frequently proves resistant to optimized
treatment of the underlying connective tissue
diseases.
Pulmonary Arterial Hypertension in
Rheumatic Diseases
RHEUMATIC
DISEASE
FEATURES OF PULMONARY ARTERIAL
HYPERTENSION
Systemic
sclerosis
Prevalence of 5-12%. More common in lSSc
PM/Scl overlap Annual screening recommended. Survival rate at 3 years of 47-
56%
Systemic lupus
erythematosus
Prevalence of 0.5-17.5%. Survival rate at 3 years of 74%.
Thrombotic arteriopathy is the most common underlying cause.
83% of patients have anticardiolipin antibodies. Patients with
severe Raynaud phenomenon, anticardiolipin
antibodies, and anti-U1RNP require screening
Rheumatoid
arthritis
Prevalence data limited; reported to be up to 20%. Clinically
significant disease rare, often secondary to COPD, chronic
thromboembolic disease, or interstitial lung disease. Improved.
RA treatment may result in a reduced incidence
SjÃļgren
syndrome
PAH a very rare complication of SjÃļgren
syndrome. Usually occurs late in the course of
disease. Prevalence unknown
Takayasu arteritis Pulmonary arteritis present in up to 50% of
patients. PAH prevalence of 12%
THROMBOSIS IN THE RHEUMATIC
DISEASES
īļ Thrombosis is an important pathologic process in many
rheumatic diseases and a cause of significant morbidity and
mortality.
īļ Large vessel thrombosis, both venous and arterial, can occur
in Behçet disease and APS.
THROMBOSISâ€Ļ.
īļ Thrombosis in situ also occurs in small vessels, principally
as the end result of chronic vessel wall hyperplasia or
inflammation in diseases such as SSc, the vasculitides, and
PAH.
īļ Chronic thromboembolic PAH can complicate SLE and SSc.
Endocrine diseases and
Cardiovascular system
Acromegaly
Cardiac complications:
īļ Secondary HTN – 20-30% of patients
īļ Cardiomyopathy & LV dysfunction
īļ LVH & asymmetrical septal hypertrophy
īļ Dilatation of aortic root(aortic ectasia) and/or defect of cardiac
conduction system
īļ ECG abnormalities: upto 50% of pts
o Left axis deviation
o Septal Q waves
o ST-T wave depression
o Abnormal QT dispersion
o Conduction system defects
Acromegalyâ€Ļ
īļ Arrythmia:
o Atrial & ventricular ectopics
o SSS
o SVT and VT
o Complex ventricular arrhythmia(4 fold ↑)
Cushing’s syndrome
Cardiovascular manifestation:
o Secondary HTN
o Accelerated atherosclerosis → coronary artery disease
o Heart failure
o LVH & LV dysfunction
o DCM
o Increased coronary artery calcification and plaque volume
o Changes in PR & QT intervals ( abnormalities in voltage gated
Na & k channels)
o Carni complex (Cushing + cardiac myxoma + pigmented
dermal lesions)
Addison disease
Cardiac manifestations:
īļ Hypotension & tachycardia ( with loss of autonomic
tone & electrolyte imbalance) → cardiovascular
collapse and crisis
īļ Low diastolic BP & orthostatic hypotension
īļ ECG abnormalities of hyperkalaemia( low amplitude p
wave and tall peaked T wave)
īļ Cardiac atrophy (teardrop heart)
Hyperaldosteronism
īļ Hypertension (due to sodium retention)
īļ May promote AF in pt with HTN
PARATHYROID DISEASE
Hyperparathyroidism & Hypercalcaemia
īļ ↑ cardiac contractility
īļ Shortening of the ventricular action potential duration,
primarily through changes in phase 2
īļ Blunting of the T wave and changes in the ST segment,
īļ Shortening of QT interval & occasionally decreases in
the PR interval.
īļ Treatment with digitalis glycosides appears to increase
sensitivity of the heart to hypercalcemia.
Hypocalcemia
īļ Low serum levels of total and ionized calcium directly
alter myocyte function.
īļ Hypocalcemia prolongs phase 2 of the action potential
duration and the QT interval.
īļ Severe hypocalcemia can impair cardiac contractility
Hyperthyroidism
īļ Sinus tachycardia & AF
īļ Secondary HTN
īļ Heart failure
īļ Angina
īļ Pulmonary hypertenion
Hypothyroidism
īļ Bradycardia
īļ Diastolic hypertension & narrow pulse pressure.
īļ Pericardial effusion ( around 30% of overt hypothyroid
pts)
īļ ECG abnormalities:
o Sinus bradycardia
o Low voltage ECG
o Prolong QT interval
īļ Accelerated atherosclerosis
īļ ↑ coronary artery disease
Pheochromocytoma
īļ Secondary HTN
īļ Tachycardia
īļ Cardiomyopathy & LV dysfunction
īļ ECG abnormalities:
o LVH Âą strain
o Ventricular & atrial ectopics
o SVT
Diabetes and Cardiovascular Disease
Coronary Heart Disease
īļ Strongly associated with type 2 DM and leading
cause of death regardless of the duration of
disease.
īļ 2-4-fold increase relative risk ratio of
cardiovascular disease in type 2 DM
īļ Degree and duration of hyperglycemia are strong
risk factors for the development of microvascular
and macrovascular complications.
Coronary Heart Disease
īļ IGT increases cardiovascular risk.
īļ Hyperglycemia enhance the progression of
atherosclerosis in type 2 diabetes.
Acute Coronary Syndromes
īļ High-risk group for developing and surviving acute MI.
īļ Pts with type 1 DM have a worse outcome than patients with
type 2 DM
īļ Diabetic women have almost twice the risk of mortality of
diabetic men.
īļ In a meta-analysis of the major trials comparing thrombolytic
therapy to percutaneous coronary intervention (PCI), diabetic
patients had significantly higher 30-day mortality when
compared with nondiabetic patients
Chronic Coronary Artery Disease
īļ Diabetic patients often are unaware of myocardial
ischemic pain, and thus, silent MI and ischemia are
markedly increased
īļ Concern for the development of sudden cardiac
death in those with diabetes.
Diabetic Cardiomyopathy
īļ Diabetic cardiomyopathy is a term used by clinicians to
encompass the multifactorial etiologies of diabetes-related
left ventricular failure characterized by both systolic and
diastolic function
īļ The Framingham heart Study - showed that men with
diabetes are twice as likely to develop congestive heart
failure compared with their nondiabetic counterparts and
females with diabetes had a five-fold increase in the rate of
congestive heart failure.
īļ Diastolic dysfunction is exceedingly common (>50%
prevalence in some studies) and may be linked to diabetes
without the presence of concomitant hypertension.
Diabetic Cardiomyopathy
īļ The etiology of impaired left ventricular function may
involve any of the following mechanisms:
(1) Coronary atherosclerotic disease,
(2) Hypertension
(3) left ventricular hypertrophy
(4) Obesity
(5) Endothelial dysfunction
(6) Coronary microvasculature disease
(7) Autonomic dysfunction, and
(8) Metabolic abnormalities.
THE HEART AND KIDNEY DISEASE
īƒ˜ CKD is associated with↑ risk of heart disease and CVD
the most frequent cause of death in patients with
ESRD.
īƒ˜ Cardiovascular mortality(In ESRD pts) - 10 to 30
times higher than in the general population a
īƒ˜ Similar marked and graded increases in risk of all-
cause death, death due to CVD, and risk of
hospitalization with declining GFR have also been
reported for the general population
Impact of CKD on Cardiovascular Risks and
Outcomes According to the Severity of Kidney
Disease
Description Normal to Mild
Decrease in GFR
Moderate GFR Loss Severe
GFR Loss
CKD stage
eGFR mL/min/1.73 m2
1-2
â‰Ĩ60
3
30-59
4
15-29
Cardiovascular risk factors
Hypertension
Diabetes
C-reactive protein >0.21 mg/dL
Hemoglobin <13 g/dL15
40%
3.1%-6.5%
25%-30%
4%
55%
16.8%
48.7%
7%
77%
22.8%
57.7%
29%
Acute myocardial infarction
Prevalence among patients
Risk of 1-year mortality AMI
28.5%
2.3%
43.5%
9.4%
30%
24.2%
CORONARY HEART DISEASE
īƒ˜ CAD highly prevalent among pts with CKD & a
major cause of morbidity and mortality
īƒ˜ Presence of albuminuria and/or eGFR < 60
mL/min/1.73 m2, considered a major risk for
mortality after an acute coronary event
īƒ˜ Pts with DM and CKD may present with evidence
of a previous “silent” myocardial infarction
CORONARY HEART DISEASEâ€Ļ..
īƒ˜ LVH–associated ST-segment changes are common
among CKD patients, thus ST-segment depression on
a resting ECG is considered an unreliable marker of
coronary ischemia in dialysis patient
īƒ˜ ESRD patients may experience changes in serum
potassium, serum calcium, and fluid volume that
affect the ECG, complicating its interpretation.
CONGESTIVE HEART FAILURE
īƒ˜ Impaired renal function ↑ risk for all-cause death,
cardiovascular death, and hospitalization for heart failure in
patients with CHF with both preserved as well as reduced
systolic function.
īƒ˜ Albuminuria an independent baseline risk factor for the
development of CHF in patients with diabetes.
īƒ˜ Echo in ESRD pts- HCM characterized by left ventricular
hypertrophy, ASH and/or impaired contractility, as well as DCM
īƒ˜ High-output cardiac failure is a rare complication of a high-flow
AV fistula.
Pericarditis
īƒ˜ Before dialysis was widely available - preterminal event in
uremic patients.
īƒ˜ Incidence of clinically apparent pericarditis has decreased
from 50% to approximately 5% to 20% of uremic patients
requiring chronic dialysis.
īƒ˜ Both uremic and dialysis pericarditis occur more frequently in
younger than in older persons and occur more commonly in
women than in men.
īƒ˜ Pericardial effusion is even more frequent in dialysis patients
because it is linked to ECV expansion.
īƒ˜ Cardiac tamponade is rare
CARDIAC ARRHYTHMIAS
īƒ˜ the single largest cause of death : 58% of all cardiac
deaths (or 25% of all-cause mortality) among peritoneal
dialysis patients and 64% of all cardiac deaths (or 27% of
all-cause mortality) among HD patients are due to
“cardiac arrest/cause unknown” or arrhythmia
īƒ˜ ↑ risk of ventricular arrhythmia in ESRD patients is likely
due to a combination of factors that include ischemic
heart disease, CHF, calcification of the conduction system
from secondary hyperparathyroidism, pericarditis,
dialysis-associated hypotension, dialysis-induced acid–base
and electrolyte shifts, obstructive sleep apnea, and
hypoxemia
Cardiopulmonary Disease
Cardiopulmonary Disease
1) Pulmonary Hypertension
2) Pulmonary Embolism
3) Chronic Cor Pulmonale
Pulmonary Hypertension
Def: PH is defined as a mean pulmonary arterial
hypertension at least 25 mm Hg at rest measured by
invasive monitoring.
Dana Point 2008 Classification
īļ The Dana Point 2008 4th World Symposium on
Pulmonary Hypertension is based on shared
pathologic, pathobiologic, and clinical features
īļ Type 3. Pulmonary hypertension due to lung diseases
and/or hypoxia
ī‚§ 3.1. Chronic obstructive pulmonary disease
ī‚§ 3.2. Interstitial lung disease
ī‚§ 3.3. Other pulmonary diseases with mixed restrictive and
obstructive pattern
ī‚§ 3.4. Sleep-disordered breathing
ī‚§ 3.5. Alveolar hypoventilation disorders
ī‚§ 3.6. Chronic exposure to high altitude
ī‚§ 3.7. Developmental abnormalities
Chronic Cor Pulmonale
īļ Chronic cor pulmonale is “hypertrophy of the
right ventricle resulting from diseases affecting
the function and/or structure of the lung, except
when these pulmonary alterations are the result
of diseases that primarily affect the left side of
the heart or congenital heart disease.”
Pathophysiologic Classification of Diseases of
the Lungs That Can Cause Cor Pulmonale
Principal Pathophysiologic
Mechanism
Disease Entity
Persistent vasoconstriction High-altitude dwellers
Hypoventilation syndromes
Chest deformities
? Idiopathic pulmonary hypertension
Loss of cross-sectional area
of the
vascular bed
Thromboembolic disease
Emphysema
Lung resection
Fibrotic lung diseases
Cystic fibrosis
Pathophysiologic Classificationâ€Ļ..
Principal Pathophysiologic
Mechanism
Disease Entity
Obstruction of large vessels Extrinsic compression of pulmonary veins
Fibrosing mediastinitis
Adenopathy/tumors
Pulmonary veno-occlusive disease
Chronically increased blood
flow
Chronically increased blood flow
Vascular remodeling Primary pulmonary hypertension
Secondary pulmonary hypertension
Collagen vascular diseases
Cystic fibrosis
SDB-RELATED CARDIOVASCULAR
CONSEQUENCES
īļ Hypertension
īļ Heart Failure
īļ Stroke
īļ Cardiac Arrhythmias
īļ Ischemic Heart Disease
īļ Pulmonary Arterial Hypertension
Neurologic disorders and cardiovascular
disease
Muscular dystrophies
īļ Duchenne and Becker muscular dystrophies
īļ Myotonic dystrophies
īļ Emery-Dreifuss muscular dystrophies and
associated disorders
īļ Limb-girdle muscular dystrophies
īļ Facioscapulohumeral muscular dystrophy
Duchenne and Becker muscular
dystrophies
īą Cardiovascular manifestations:
īļ Cardiomyopathy: most common
â€ĸ 90% of DMD at the age of 18 yrs
â€ĸ BMD – more variable
īļ Arrythmia:
â€ĸ Sinus tachycardia
â€ĸ Atrial arrhythmia – AF & Atrial flutter
â€ĸ AV conduction defects – short or long PR interval
â€ĸ Ventricular arrhythmia (30% of pts) – PVC, complex ventricular
arrhythmia
Myotonic Dystrophies
īļ Cardiovascular manifestations: degeneration,
fibrosis, and fatty infiltration in conduction tissue
including the SA node, AV node, and His-Purkinje
system
īļ Arrythmia:
īƒŧ Symptomatic AV block → PPM
īƒŧ AF & Atrial flutter
īƒŧ VT
īƒŧ Sudden death
Limb-Girdle Muscular Dystrophies
īą Cardiovascular manifestations:
īļ DCM → heart failure
īļ AV block
FRIEDREICH ATAXIA
īą Cardiovascular manifestations:
īļ HCM
īļ Arrhythmia- AF & Atrial flutter, VT
Spinal Muscular Atrophy
īą Cardiovascular manifestations:
īļ Complex congenital heart disease – ASD
īļ Cardiomyopathy, and
īļ Arrhythmias - AF & Atrial flutter & AV conduction defect
Guillain-BarrÊ Syndrome
īļ Cardiac involvement rela- autonomic nervous system
dysfunction (50% of pts)
īļ Cardiac manifestations include hypertension,
orthostatic hypotension, resting sinus tachycardia,
loss of heart rate variability, electrocardiographic ST
abnormalities, and both bradycardia and
tachycardias.
īļ Arrhythmias observed include asystole, symptomatic
bradycardia, rapid atrial fibrillation and ventricular
tachycardia or fibrillation.
Myasthenia Gravis
īą Cardiovascular manifestations:
īƒ˜ Myocarditis
īƒ˜ Arrhythmia – AF, Atrial flutter, AV block, asystole,
ACUTE CEREBROVASCULAR DISEASE
īą Cardiovascular manifestations:
īƒ˜ Most commonly found in SAH
īƒ˜ ECG abnormalities: 70% pts with SAH
ī‚§ ST elevation and depression,
ī‚§ T wave inversion, and
ī‚§ pathologic Q waves
ī‚§ Peaked inverted T waves and
ī‚§ a prolonged QT interval
ACUTE CEREBROVASCULAR DISEASE
īƒ˜ ↑ Troponins
īƒ˜ LV dysfunction (in Echo)
īƒ˜ Pulmonary oedema
īƒ˜ Life threatening arrhythmia: VT, VF, torsades De Pointes
īƒ˜ Atrial arrhythmia – AF (most commonly acute
thromboembolic stroke)
īƒ˜ Bradycardias including SA block, sinus arrest, and AV block
- 10% of patients with SAH
THANK YOU
References
BRAUNWALD’S HEART DISEASE, 10th
edition
Hurst’s The Heart, 13th Edition

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Systemic disease involving cardiovascular system

  • 1. Systemic disease involving cardiovascular system Presented by Dr. Md. Ahasanul Kabir Resident, Phase B Department of Cardiology, BSMMU Chairperson: Assoc. Prof. Dr. Mukhlesur Rahman UCC, BSMMU
  • 2. Systems commonly involve ī‚§ Rheumatic disease ī‚§ Endocrine disease including DM ī‚§ Renal disease – CKD ī‚§ Respiratory disease ī‚§ Neurologic disease
  • 3. Common Cardiovascular Manifestations of Systemic Autoimmune Diseases Disease Sex Distributi on Cardiovascular Manifestations Rheumatoid arthritis F>M o Pericarditis o Coronary artery disease o Cardiomyopathy o Congestive heart failure Systemic lupus erythematosus F>M o Pericarditis o Libman-Sacks endocarditis o Coronary artery disease, o Hypertension Inflammatory myopathies F>M o Pericarditis o Conduction system abnormalities o Congestive heart failure o Myocarditis
  • 4. Common Cardiovascular Manifestations of Systemic Autoimmune Diseases Disease Sex Distributi on Cardiovascular Manifestations Systemic sclerosis F>M o Pulmonary hypertension, o Pericarditis o Cardiomyopathy o conduction system disease Seronegative spondyloarthropathy M>F o Aortitis, o Conduction system disease
  • 5. Accelerated Atherosclerosis in Rheumatic Diseases īļ Association between inflammatory diseases and accelerated atherogenesis – eg RA & SLE. īļ Ankylosing spondylitis, psoriatic arthritis, TA, and APS - associated with premature atherosclerosis.
  • 6. Accelerated Atherosclerosis in Rheumatic Diseases īļ Should consider an underlying inflammatory disease in young patients with otherwise unexplained angina, myocardial infarction, or stroke. īļ Pts with a rheumatic disease who suffer a myocardial infarction have worse outcomes in terms of both heart failure and mortality than does the age-matched general population.
  • 7. Endothelial Dysfunction and Vascular Injury īļ Prolonged systemic inflammation such as that seen in RA and SLE may promote endothelial injury, increased endothelial apoptosis, and endothelial vasodilator dysfunction. īļ Inflammation may exacerbate the effects of classic risk factors
  • 8. Endothelial Dysfunction and Vascular Injury īļ Compared with the general population, patients with systemic inflammatory diseases more commonly exhibit endothelial dysfunction and increased aortic stiffness. īļ Systemic inflammatory environment may predispose to increased plaque instability and rupture
  • 9. Molecular mechanisms for atherosclerotic disease and cardiovascular events. īļ Proinflammatory cytokines – TNF-Îą, IL-1 & IL-6 effects on endothelial activation, leukocyte adhesion, endothelial injury, and permeability. īļ ↑ endothelial cell apoptosis and ↓ capacity for repair may contribute. īļ Others: īƒŧ Autoantibodies (e.g antiphospholipid antibodies) īƒŧ CD4 + CD28− cytotoxic T cells īƒŧ Th17/TREG imbalance īƒŧ Complement deficiency or excessive activation īƒŧ Genetic polymorphisms īƒŧ Deleterious effects of drugs, including corticosteroids & cyclosporine
  • 10. Rheumatoid Arthritis īļ A variety of studies have shown subclinical arterial disease with increased carotid intimal-medial thickness (IMT) and early plaque development. īļ A recent study of microvascular and macrovascular function in RA has suggested that the classic cardiovascular risk factors may influence endothelial function more than disease-related inflammation does. īļ The direct effect of chronic inflammation on vascular endothelium may itself promote atherogenesis, in addition to exacerbating the actions of traditional cardiovascular risk factors.
  • 11. Rheumatoid Arthritisâ€Ļ īļ Patients with RA have increased classic risk factors for atherosclerosis. īļ Tobacco smoking is associated with both cardiovascular risk and the development of RA. īļ Insulin resistance and the metabolic syndrome are more common in RA.
  • 12. Atherosclerotic Disease in Rheumatoid Arthritis īļ High TG levels and low levels HDL & LDL cholesterol īļ Risk for MI in patients with RA similar to that in those with DM and women with RA are twice as likely as age-matched controls in the general population īļ Patients with RA who suffer a MI are less likely to receive acute reperfusion therapy and secondary preventive measures and thus have worse outcomes.
  • 13. Systemic Lupus Erythematosus o Pericarditis o Libman-Sacks endocarditis o Coronary artery disease o Hypertension
  • 14. Pericarditis īļ The most common cardiovascular manifestation of SLE is pericarditis, īļ Up to 42% of pts in echo shows effusion. īļ May be detected at any point in the disease and are usually asymptomatic and small.
  • 15. Pericarditisâ€Ļ īļ Acute pericarditis may occur - 20% to 30% of patients īļ May be associated with cardiac tamponade; īļ Chronic pericarditis may occasionally lead to constriction
  • 16. Systemic Lupus Erythematosus īļ Studies suggested an ↑ risk for MI and stroke in patients with SLE(Between 2-fold and 10-fold and up to 50-fold greater than general population) īļ Young age of patients with SLE and CVS disease (67% of female patients with SLE and a first cardiac event are typically initially seen before 55 years of age) suggests that SLE accelerates arterial disease. īļ Worse outcomes following MI than the age-matched general population with a higher risk of cardiac failure and increased mortality.
  • 17. Systemic Lupus Erythematosus īļ HTN common in SLE - presence of renal disease and the use of glucocorticoids īļ Commonly have metabolic syndrome - associated with renal impairment, higher corticosteroid doses, and Korean or Hispanic ethnicity. īļ Lipid abnormalities - including high levels of VLDL and TG, elevated or normal LDL cholesterol, and reduced HDL cholesterol.
  • 18. Coronary Artery Involvement and the Rheumatic Diseases Premature Atherosclerosis 1. Systemic lupus erythematosus 2. Rheumatoid arthritis 3. Ankylosing spondylitis 4. Psoriatic arthritis 5. Gout 6. Takayasu arteritis 7. Giant cell arteritis Coronary Arteritis 1. Systemic lupus erythematosus 2. Takayasu arteritis 3. Kawasaki disease 4. Churg-Strauss syndrome 5. Polyarteritis nodosa 6. Granulomatous polyangiitis 7. Rheumatoid arthritis
  • 19. Cardiovascular Disease in the Systemic Vasculitides VASCULITIDES CARDIOVASCULAR COMPLICATIONS Large-Vessel Vasculitis Giant cell arteritis 1. Thoracic/abdominal artery aneurysm, 2. Limb ischemia 3. Pericarditis 4. Coronary arteritis 5. IHD 6. MI Takayasu arteritis Aortic regurgitation Limb ischemia Aortic stenosis Aortic aneurysm Stroke Hypertension Coronary arteritis and aneurysm IHD & MI, Myocarditis Cardiac failure
  • 20. Cardiovascular Disease in the Systemic Vasculitides Kawasaki disease 1. Coronary artery aneurysm 2. MI 3. Myocarditis 4. Pericarditis 5. Valvular dysfunction, 6. Cardiac failure
  • 21. Cardiovascular Disease in the Systemic Vasculitides Medium-Vessel Vasculitis CARDIOVASCULAR COMPLICATIONS Churg-Strauss syndrome o Myocarditis o Pericarditis o coronary arteritis, o Cardiomyopathy o cardiac fibrosis o Valvular dysfunction o MI Polyarteritis nodosa o Myocarditis o Pericarditis o coronary arteritis, o coronary aneurysm o Hypertension o cardiac failure
  • 22. Cardiovascular Disease in the Systemic Vasculitides Medium-Vessel Vasculitis CARDIOVASCULAR COMPLICATIONS Granulomatous polyangiitis o Myocarditis o Pericarditis o Coronary arteritis o Valvular heart disease o Cardiac failure Microscopic polyangiitis o Pericarditis o Coronary microaneurysm o MI
  • 23. Giant Cell Arteritis īļ Severe cardiovascular complications - dissecting thoracic aortic aneurysms. īļ Imaging and autopsy studies - aortitis and aortic wall thickening are frequent in GCA īļ ↑ FDG uptake in the thoracic aorta associated with an ↑ risk for aortic dilation. īļ 17-fold increased risk for thoracic aortic aneurysms.
  • 24. Giant Cell Arteritis īļ Annual thoracic aortic screening for those with FDG-PET–positive thoracic aortic uptake or magnetic resonance angiography (MRA) or computed tomography angiography (CTA) evidence of aortic wall thickening īļ Every 2 to 3 years in the remainder of patients.
  • 25. Giant Cell Arteritisâ€Ļ. īĩ Other CVS complications: īƒ˜ Pericarditis īƒ˜ Coronary arteritis īƒ˜ Limb ischemia īƒ˜ Accelerated atherosclerosis īƒ˜ Myocardial infarction īƒ˜ Cerebrovascular accidents
  • 26. Takayasu Arteritis īļ Vascular complications: o Cerebral, internal organ and limb ischemia, o Aneurysms o PAH
  • 27. Takayasu Arteritis īļ Cardiac complications: o Aortic valve insufficiency o Accelerated atherosclerosis, o Cardiac ischemia, o Myocardial infarction, and o Heart failure.
  • 28. Takayasu Arteritis īƒ˜ Neither MRA nor 18F-FDG-PET-CT reliably identifies coronary arteritis. īƒ˜ Coronary disease is often asymptomatic īƒ˜ Silent myocardial injury in 27% of a cohort īƒ˜ ↑ risk from secondary accelerated atherosclerosis.
  • 29. Takayasu Arteritisâ€Ļ īƒ˜ Thallium stress scintigraphy revealed myocardial perfusion defects in 53%, īƒ˜ intra-arterial angiography has shown that up to 30% have coronary artery lesions typically affecting the ostia and proximal segments, with the left main coronary artery being most commonly affected.
  • 30. Takayasu Arteritis īƒ˜ Inflammation of the ascending aorta → coronary artery involvement īƒ˜ Dilation of the aortic root → AR īƒ˜ LV dysfunction up to 20% and is due to myocarditis, ischemic heart disease, and hypertension īƒ˜ Common finding often associated with renal artery stenosis in TA.
  • 31. Kawasaki Disease īą Cardiovascular complications: īƒ˜ Coronary artery aneurysm( 25% of untreated patient) īƒ˜ Sudden death – MI following acute coronary thrombosis or rupture of a coronary artery aneurysm. īƒ˜ Pericarditis īƒ˜ Pericardial effusion īƒ˜ Myocarditis īƒ˜ Valvular dysfunction īƒ˜ Heart failure īƒ˜ Peripheral arterial involvement (limb, renal & visceral arteries) – less common
  • 32. Churg-Strauss Syndrome īƒ˜ Of all the vasculitides, CSS is the most likely to be associated with severe cardiac disease īƒ˜ Cardiac involvement complicates up to 60% of cases. īƒ˜ Cardiac complications: o Pericarditis o Myocarditis o coronary arteritis o myocardial infarction, o cardiac fibrosis, o arterial thrombosis, and o valvular dysfunction.
  • 33. Churg-Strauss Syndrome īƒ˜ Cardiac disease - prominent cause of death. īƒ˜ Cardiomyopathy - result of ischemia secondary to arteritis of intramyocardial arteries or, less frequently, the epicardial coronary arteries. īƒ˜ Myocarditis is associated with eosinophilic infiltration, fibrosis, and occasionally, granulomatous infiltration. īƒ˜ Myocarditis can be life threatening and may result in the development of restrictive, congestive, or dilated cardiomyopathy.
  • 34. Polyarteritis Nodosa īƒ˜ Cardiac involvement often subclinical and clinically apparent in only 10% of patients. īƒ˜ CCF most commonly seen and consequence of a specific myocarditis or coronary arteritis. īƒ˜ 5% of pts – Pericarditis, supraventricular tachycardia and valvular disease. īƒ˜ CAG may reveal coronary artery microaneurysms, coronary arteritis, or coronary spasm.
  • 35. PERICARDITIS īļ Pericarditis commonly complicates the autoimmune connective tissue diseases, particularly SLE, SSc, and RA. īļ Clinically significant pericarditis develops in fewer than 30% of patients. īļ Diagnosed by echocardiography, which detects pericardial thickening or small effusions in up to 50% of these patients.
  • 36. PERICARDITIS īļ CMR can also provide accurate definition of the extent of pericardial involvement. īļ In SLE - pericarditis is usually associated with a flare of disease and often with polyserositis. īļ Clinically significant pericarditis affects only 1% to 2% of patients with RA, more commonly male, seropositive patients.
  • 37. MYOCARDITIS īļ Myocarditis is a rare but recognized cause of mortality in patients with autoimmune rheumatic diseases and is most commonly seen in patients with SLE, SSc, and polymyositis or dermatomyositis. īļ Myocarditis is also rarely associated with other rheumatic diseases, including ankylosing spondylitis, adult Still disease, GCA, and TA.
  • 38. VALVULAR HEART DISEASE īļ Clinically significant valvular disease can complicate many rheumatic diseases. īļ Mechanisms - direct damage to cardiac valve leaflets or aortic valve regurgitation as a consequence of aortitis affecting the ascending aorta.
  • 39. VALVULAR HEART DISEASEâ€Ļ. īļ In SLE pt: o Verrucous endocarditis (Libman-Sacks endocarditis) and nonspecific valvular thickening are most commonly detected. o Valvulitis with rapid valvular dysfunction o Libman-Sacks lesions typically affects both valve surfaces, most commonly the mitral valve.
  • 40. VALVULAR HEART DISEASE īą Seronegative Spondyloarthropathies: īļ Aortic valvulitis leads to aortic cusp thickening and retraction and subsequently to symptomatic aortic regurgitation, which may cause heart failure. īļ Proximal aortitis affecting the ascending aorta leads to aortic root thickening and subsequently to dilation and aortic regurgitation, the prevalence of which is related to disease duration.
  • 41. VALVULAR HEART DISEASE īą In RA pts: īļValvular thickening is commonly associated with RA in echo īļEcho- shows mitral valve involvement, with valve thickening, asymptomatic mitral regurgitation, and prolapse being the predominant findings.
  • 42. VALVULAR HEART DISEASE īą In TA: o Cardiac valve dysfunction commonly complicates TA o Inflammation of the ascending aorta predisposes to dilation of the aortic root with subsequent aortic valve regurgitation.
  • 43. CARDIAC CONDUCTION DISTURBANCES īą IN SLE pt: īļ Adult SLE seldom causes primary conduction abnormalities or rhythm disturbance, which may instead result from underlying ischemic heart disease or myocarditis.
  • 44. īļ Female patients with SLE or SjÃļgren syndrome who test positive for antibodies against the Ro and/ or La antigens carry the risk of bearing a child with congenital heart block, which may be complicated by myocarditis. īļ These antibodies may cross the placenta and induce myocardial inflammation and can target the conduction system and lead to fibrosis
  • 45. CARDIAC CONDUCTION DISTURBANCES īą In SS pt: īļ affects up to 50% of patients. īļ Cause- patchy myocardial fibrosis → disruption of the conduction pathways. īļ Supraventricular arrhythmias are usually benign and amenable to treatment.
  • 46. īļ Ventricular conduction abnormalities also frequently occur in SSc and may impair myocardial function. īļ In these patients ventricular ectopy is common and closely associated with sudden death.
  • 47. CARDIAC CONDUCTION DISTURBANCES īą Spondyloarthropathies īļ Frequently complicate the HLA-B27– related spondyloarthropathies. īļ In AS up to 30% of patients experience conduction system disease, īļ Cause - subaortic fibrosis extending into the septum and affecting AV node. īļ Atrioventricular conduction block occurs commonly and may become complete.
  • 48. CARDIAC CONDUCTIONâ€Ļ. īą Polymyositis and Dermatomyositis: īļLeft anterior hemiblock and right bundle branch block occur most frequently and occasionally progress to complete heart block. īļCauses: Inflammation and fibrosis affect the conduction pathways
  • 49. CARDIAC CONDUCTIONâ€Ļ. īą RA patients: īļECG screening studies - arrhythmias or conducting system abnormalities in up to 50% īļCause: myocarditis and amyloid deposition in the heart can cause atrioventricular node conduction block. īļSimilarly, rheumatoid nodules may disrupt the conduction system and cause all types of conduction abnormality.
  • 50. PULMONARY ARTERIAL HYPERTENSION īƒ˜ PAH can result from the connective tissue diseases and is of concern to rheumatologists as a significant cause of premature mortality. īƒ˜ Often manifested late or goes undiagnosed. īƒ˜ Frequently proves resistant to optimized treatment of the underlying connective tissue diseases.
  • 51. Pulmonary Arterial Hypertension in Rheumatic Diseases RHEUMATIC DISEASE FEATURES OF PULMONARY ARTERIAL HYPERTENSION Systemic sclerosis Prevalence of 5-12%. More common in lSSc PM/Scl overlap Annual screening recommended. Survival rate at 3 years of 47- 56% Systemic lupus erythematosus Prevalence of 0.5-17.5%. Survival rate at 3 years of 74%. Thrombotic arteriopathy is the most common underlying cause. 83% of patients have anticardiolipin antibodies. Patients with severe Raynaud phenomenon, anticardiolipin antibodies, and anti-U1RNP require screening Rheumatoid arthritis Prevalence data limited; reported to be up to 20%. Clinically significant disease rare, often secondary to COPD, chronic thromboembolic disease, or interstitial lung disease. Improved. RA treatment may result in a reduced incidence
  • 52. SjÃļgren syndrome PAH a very rare complication of SjÃļgren syndrome. Usually occurs late in the course of disease. Prevalence unknown Takayasu arteritis Pulmonary arteritis present in up to 50% of patients. PAH prevalence of 12%
  • 53. THROMBOSIS IN THE RHEUMATIC DISEASES īļ Thrombosis is an important pathologic process in many rheumatic diseases and a cause of significant morbidity and mortality. īļ Large vessel thrombosis, both venous and arterial, can occur in Behçet disease and APS.
  • 54. THROMBOSISâ€Ļ. īļ Thrombosis in situ also occurs in small vessels, principally as the end result of chronic vessel wall hyperplasia or inflammation in diseases such as SSc, the vasculitides, and PAH. īļ Chronic thromboembolic PAH can complicate SLE and SSc.
  • 56. Acromegaly Cardiac complications: īļ Secondary HTN – 20-30% of patients īļ Cardiomyopathy & LV dysfunction īļ LVH & asymmetrical septal hypertrophy īļ Dilatation of aortic root(aortic ectasia) and/or defect of cardiac conduction system īļ ECG abnormalities: upto 50% of pts o Left axis deviation o Septal Q waves o ST-T wave depression o Abnormal QT dispersion o Conduction system defects
  • 57. Acromegalyâ€Ļ īļ Arrythmia: o Atrial & ventricular ectopics o SSS o SVT and VT o Complex ventricular arrhythmia(4 fold ↑)
  • 58. Cushing’s syndrome Cardiovascular manifestation: o Secondary HTN o Accelerated atherosclerosis → coronary artery disease o Heart failure o LVH & LV dysfunction o DCM o Increased coronary artery calcification and plaque volume o Changes in PR & QT intervals ( abnormalities in voltage gated Na & k channels) o Carni complex (Cushing + cardiac myxoma + pigmented dermal lesions)
  • 59. Addison disease Cardiac manifestations: īļ Hypotension & tachycardia ( with loss of autonomic tone & electrolyte imbalance) → cardiovascular collapse and crisis īļ Low diastolic BP & orthostatic hypotension īļ ECG abnormalities of hyperkalaemia( low amplitude p wave and tall peaked T wave) īļ Cardiac atrophy (teardrop heart)
  • 60. Hyperaldosteronism īļ Hypertension (due to sodium retention) īļ May promote AF in pt with HTN
  • 62. Hyperparathyroidism & Hypercalcaemia īļ ↑ cardiac contractility īļ Shortening of the ventricular action potential duration, primarily through changes in phase 2 īļ Blunting of the T wave and changes in the ST segment, īļ Shortening of QT interval & occasionally decreases in the PR interval. īļ Treatment with digitalis glycosides appears to increase sensitivity of the heart to hypercalcemia.
  • 63. Hypocalcemia īļ Low serum levels of total and ionized calcium directly alter myocyte function. īļ Hypocalcemia prolongs phase 2 of the action potential duration and the QT interval. īļ Severe hypocalcemia can impair cardiac contractility
  • 64. Hyperthyroidism īļ Sinus tachycardia & AF īļ Secondary HTN īļ Heart failure īļ Angina īļ Pulmonary hypertenion
  • 65. Hypothyroidism īļ Bradycardia īļ Diastolic hypertension & narrow pulse pressure. īļ Pericardial effusion ( around 30% of overt hypothyroid pts) īļ ECG abnormalities: o Sinus bradycardia o Low voltage ECG o Prolong QT interval īļ Accelerated atherosclerosis īļ ↑ coronary artery disease
  • 66. Pheochromocytoma īļ Secondary HTN īļ Tachycardia īļ Cardiomyopathy & LV dysfunction īļ ECG abnormalities: o LVH Âą strain o Ventricular & atrial ectopics o SVT
  • 68. Coronary Heart Disease īļ Strongly associated with type 2 DM and leading cause of death regardless of the duration of disease. īļ 2-4-fold increase relative risk ratio of cardiovascular disease in type 2 DM īļ Degree and duration of hyperglycemia are strong risk factors for the development of microvascular and macrovascular complications.
  • 69. Coronary Heart Disease īļ IGT increases cardiovascular risk. īļ Hyperglycemia enhance the progression of atherosclerosis in type 2 diabetes.
  • 70. Acute Coronary Syndromes īļ High-risk group for developing and surviving acute MI. īļ Pts with type 1 DM have a worse outcome than patients with type 2 DM īļ Diabetic women have almost twice the risk of mortality of diabetic men. īļ In a meta-analysis of the major trials comparing thrombolytic therapy to percutaneous coronary intervention (PCI), diabetic patients had significantly higher 30-day mortality when compared with nondiabetic patients
  • 71. Chronic Coronary Artery Disease īļ Diabetic patients often are unaware of myocardial ischemic pain, and thus, silent MI and ischemia are markedly increased īļ Concern for the development of sudden cardiac death in those with diabetes.
  • 72. Diabetic Cardiomyopathy īļ Diabetic cardiomyopathy is a term used by clinicians to encompass the multifactorial etiologies of diabetes-related left ventricular failure characterized by both systolic and diastolic function īļ The Framingham heart Study - showed that men with diabetes are twice as likely to develop congestive heart failure compared with their nondiabetic counterparts and females with diabetes had a five-fold increase in the rate of congestive heart failure. īļ Diastolic dysfunction is exceedingly common (>50% prevalence in some studies) and may be linked to diabetes without the presence of concomitant hypertension.
  • 73. Diabetic Cardiomyopathy īļ The etiology of impaired left ventricular function may involve any of the following mechanisms: (1) Coronary atherosclerotic disease, (2) Hypertension (3) left ventricular hypertrophy (4) Obesity (5) Endothelial dysfunction (6) Coronary microvasculature disease (7) Autonomic dysfunction, and (8) Metabolic abnormalities.
  • 74. THE HEART AND KIDNEY DISEASE īƒ˜ CKD is associated with↑ risk of heart disease and CVD the most frequent cause of death in patients with ESRD. īƒ˜ Cardiovascular mortality(In ESRD pts) - 10 to 30 times higher than in the general population a īƒ˜ Similar marked and graded increases in risk of all- cause death, death due to CVD, and risk of hospitalization with declining GFR have also been reported for the general population
  • 75. Impact of CKD on Cardiovascular Risks and Outcomes According to the Severity of Kidney Disease Description Normal to Mild Decrease in GFR Moderate GFR Loss Severe GFR Loss CKD stage eGFR mL/min/1.73 m2 1-2 â‰Ĩ60 3 30-59 4 15-29 Cardiovascular risk factors Hypertension Diabetes C-reactive protein >0.21 mg/dL Hemoglobin <13 g/dL15 40% 3.1%-6.5% 25%-30% 4% 55% 16.8% 48.7% 7% 77% 22.8% 57.7% 29% Acute myocardial infarction Prevalence among patients Risk of 1-year mortality AMI 28.5% 2.3% 43.5% 9.4% 30% 24.2%
  • 76. CORONARY HEART DISEASE īƒ˜ CAD highly prevalent among pts with CKD & a major cause of morbidity and mortality īƒ˜ Presence of albuminuria and/or eGFR < 60 mL/min/1.73 m2, considered a major risk for mortality after an acute coronary event īƒ˜ Pts with DM and CKD may present with evidence of a previous “silent” myocardial infarction
  • 77. CORONARY HEART DISEASEâ€Ļ.. īƒ˜ LVH–associated ST-segment changes are common among CKD patients, thus ST-segment depression on a resting ECG is considered an unreliable marker of coronary ischemia in dialysis patient īƒ˜ ESRD patients may experience changes in serum potassium, serum calcium, and fluid volume that affect the ECG, complicating its interpretation.
  • 78. CONGESTIVE HEART FAILURE īƒ˜ Impaired renal function ↑ risk for all-cause death, cardiovascular death, and hospitalization for heart failure in patients with CHF with both preserved as well as reduced systolic function. īƒ˜ Albuminuria an independent baseline risk factor for the development of CHF in patients with diabetes. īƒ˜ Echo in ESRD pts- HCM characterized by left ventricular hypertrophy, ASH and/or impaired contractility, as well as DCM īƒ˜ High-output cardiac failure is a rare complication of a high-flow AV fistula.
  • 79. Pericarditis īƒ˜ Before dialysis was widely available - preterminal event in uremic patients. īƒ˜ Incidence of clinically apparent pericarditis has decreased from 50% to approximately 5% to 20% of uremic patients requiring chronic dialysis. īƒ˜ Both uremic and dialysis pericarditis occur more frequently in younger than in older persons and occur more commonly in women than in men. īƒ˜ Pericardial effusion is even more frequent in dialysis patients because it is linked to ECV expansion. īƒ˜ Cardiac tamponade is rare
  • 80. CARDIAC ARRHYTHMIAS īƒ˜ the single largest cause of death : 58% of all cardiac deaths (or 25% of all-cause mortality) among peritoneal dialysis patients and 64% of all cardiac deaths (or 27% of all-cause mortality) among HD patients are due to “cardiac arrest/cause unknown” or arrhythmia īƒ˜ ↑ risk of ventricular arrhythmia in ESRD patients is likely due to a combination of factors that include ischemic heart disease, CHF, calcification of the conduction system from secondary hyperparathyroidism, pericarditis, dialysis-associated hypotension, dialysis-induced acid–base and electrolyte shifts, obstructive sleep apnea, and hypoxemia
  • 82. Cardiopulmonary Disease 1) Pulmonary Hypertension 2) Pulmonary Embolism 3) Chronic Cor Pulmonale
  • 83. Pulmonary Hypertension Def: PH is defined as a mean pulmonary arterial hypertension at least 25 mm Hg at rest measured by invasive monitoring.
  • 84. Dana Point 2008 Classification īļ The Dana Point 2008 4th World Symposium on Pulmonary Hypertension is based on shared pathologic, pathobiologic, and clinical features īļ Type 3. Pulmonary hypertension due to lung diseases and/or hypoxia ī‚§ 3.1. Chronic obstructive pulmonary disease ī‚§ 3.2. Interstitial lung disease ī‚§ 3.3. Other pulmonary diseases with mixed restrictive and obstructive pattern ī‚§ 3.4. Sleep-disordered breathing ī‚§ 3.5. Alveolar hypoventilation disorders ī‚§ 3.6. Chronic exposure to high altitude ī‚§ 3.7. Developmental abnormalities
  • 85. Chronic Cor Pulmonale īļ Chronic cor pulmonale is “hypertrophy of the right ventricle resulting from diseases affecting the function and/or structure of the lung, except when these pulmonary alterations are the result of diseases that primarily affect the left side of the heart or congenital heart disease.”
  • 86. Pathophysiologic Classification of Diseases of the Lungs That Can Cause Cor Pulmonale Principal Pathophysiologic Mechanism Disease Entity Persistent vasoconstriction High-altitude dwellers Hypoventilation syndromes Chest deformities ? Idiopathic pulmonary hypertension Loss of cross-sectional area of the vascular bed Thromboembolic disease Emphysema Lung resection Fibrotic lung diseases Cystic fibrosis
  • 87. Pathophysiologic Classificationâ€Ļ.. Principal Pathophysiologic Mechanism Disease Entity Obstruction of large vessels Extrinsic compression of pulmonary veins Fibrosing mediastinitis Adenopathy/tumors Pulmonary veno-occlusive disease Chronically increased blood flow Chronically increased blood flow Vascular remodeling Primary pulmonary hypertension Secondary pulmonary hypertension Collagen vascular diseases Cystic fibrosis
  • 88. SDB-RELATED CARDIOVASCULAR CONSEQUENCES īļ Hypertension īļ Heart Failure īļ Stroke īļ Cardiac Arrhythmias īļ Ischemic Heart Disease īļ Pulmonary Arterial Hypertension
  • 89. Neurologic disorders and cardiovascular disease
  • 90. Muscular dystrophies īļ Duchenne and Becker muscular dystrophies īļ Myotonic dystrophies īļ Emery-Dreifuss muscular dystrophies and associated disorders īļ Limb-girdle muscular dystrophies īļ Facioscapulohumeral muscular dystrophy
  • 91. Duchenne and Becker muscular dystrophies īą Cardiovascular manifestations: īļ Cardiomyopathy: most common â€ĸ 90% of DMD at the age of 18 yrs â€ĸ BMD – more variable īļ Arrythmia: â€ĸ Sinus tachycardia â€ĸ Atrial arrhythmia – AF & Atrial flutter â€ĸ AV conduction defects – short or long PR interval â€ĸ Ventricular arrhythmia (30% of pts) – PVC, complex ventricular arrhythmia
  • 92. Myotonic Dystrophies īļ Cardiovascular manifestations: degeneration, fibrosis, and fatty infiltration in conduction tissue including the SA node, AV node, and His-Purkinje system īļ Arrythmia: īƒŧ Symptomatic AV block → PPM īƒŧ AF & Atrial flutter īƒŧ VT īƒŧ Sudden death
  • 93. Limb-Girdle Muscular Dystrophies īą Cardiovascular manifestations: īļ DCM → heart failure īļ AV block
  • 94. FRIEDREICH ATAXIA īą Cardiovascular manifestations: īļ HCM īļ Arrhythmia- AF & Atrial flutter, VT
  • 95. Spinal Muscular Atrophy īą Cardiovascular manifestations: īļ Complex congenital heart disease – ASD īļ Cardiomyopathy, and īļ Arrhythmias - AF & Atrial flutter & AV conduction defect
  • 96. Guillain-BarrÊ Syndrome īļ Cardiac involvement rela- autonomic nervous system dysfunction (50% of pts) īļ Cardiac manifestations include hypertension, orthostatic hypotension, resting sinus tachycardia, loss of heart rate variability, electrocardiographic ST abnormalities, and both bradycardia and tachycardias. īļ Arrhythmias observed include asystole, symptomatic bradycardia, rapid atrial fibrillation and ventricular tachycardia or fibrillation.
  • 97. Myasthenia Gravis īą Cardiovascular manifestations: īƒ˜ Myocarditis īƒ˜ Arrhythmia – AF, Atrial flutter, AV block, asystole,
  • 98. ACUTE CEREBROVASCULAR DISEASE īą Cardiovascular manifestations: īƒ˜ Most commonly found in SAH īƒ˜ ECG abnormalities: 70% pts with SAH ī‚§ ST elevation and depression, ī‚§ T wave inversion, and ī‚§ pathologic Q waves ī‚§ Peaked inverted T waves and ī‚§ a prolonged QT interval
  • 99. ACUTE CEREBROVASCULAR DISEASE īƒ˜ ↑ Troponins īƒ˜ LV dysfunction (in Echo) īƒ˜ Pulmonary oedema īƒ˜ Life threatening arrhythmia: VT, VF, torsades De Pointes īƒ˜ Atrial arrhythmia – AF (most commonly acute thromboembolic stroke) īƒ˜ Bradycardias including SA block, sinus arrest, and AV block - 10% of patients with SAH
  • 101. References BRAUNWALD’S HEART DISEASE, 10th edition Hurst’s The Heart, 13th Edition