VALVULAR, CONGENITAL

& INFLAMMATORY HEART

DISEASES
DR. S.I. SINGHA
MBBS, MD, DM (Cardiology)
Fellowship in cardiac electrophysiology
TOPICS FOR DISCUSSION
• VALVULAR HEART DISEASES

• CONGENITAL HEART DISEASES

• RHEUMATIC HEART DISEASES

• ENDOCARDITIS

• MYOCARDITIS

• PERICARDITIS
 VALVULAR HEART DISEASES

• SIGNS & SYMPTOMS

 AORTIC STENOSIS

• Aortic stenosis (AS) is the commonest acquired valvular lesions. The normal
aortic valve has three leaflets (cusps); however, some people are born with
bicuspid aortic valves.
• Causes
- Calcification
- Congenital bicuspid valve
- Stenosis occurring above or
below the aortic ring
- Rheumatic fever
• Diagnosis
- ECG—LVH pattern. Left-axis deviation.
- Echocardiogram—appearance of valve cusps (i.e. thickening or
calcification), presence of LVH and left ventricular outflow tract obstruction
(LVOTO) can be assessed. The gradient across the valve can be
measured: normal gradient is 20mmHg.
• Treatment
- Aortic valve replacement surgery is usually the first line of treatment once
symptoms have developed because of poor prognosis.
- TAVI or TAVR can now be used, particularly for those patients not suitable
for open heart surgery.
- Asymptomatic patients are usually followed up every 6/2mths.
 AORTIC REGURGITATION

• Aortic regurgitation (AR) leads to a leakage of blood from the aorta into the LV during
diastole. AR can be acute or chronic depending on the cause.
• Causes
- Rheumatic fever
- Infective carditis (IE)
- Bicuspid aortic valve
- Degenerative changes
- Trauma
- Dilatation of the aortic root
(e.g. Marfan’s syndrome)
• Diagnosis
- ECG—LVH and LAH.
- Echo—Valve & LV function assessed.
• Management
- Patients with mild or moderate AR are commonly asymptomatic and will
not usually require surgery.
- Assessment of symptoms, ECG, and imaging (echocardiogram and CXR) will
be performed every 12mths.
- Diuretics may be used to improve shortness of breath.
- ACE inhibitors can be used for LV dilatation.
- Inotropes may be required for acute AR.
- Aortic valve replacement surgery is usually considered once patients
develop symptoms, or in asymptomatic patients with EF less than 50% or
LV enlargement with LVEDD >70mm.
- Urgent surgery may be required if there is IE or aortic dissection.
 MITRAL STENOSIS

• MS reduces the amount of blood flowing into the LV during diastole.

Narrowing of the MV orifice to <2 cm sq. leads to high pressure gradient
across the mitral valve.
• Rheumatic fever is the most
common cause of MS.
• Diagnosis: ECG, Echo, CXR
• Rx:
- MS is clinically significant when
the valve area is <1.5cm2 .
- A percutaneous mitral commissurotomy
or valve replacement surgery may be performed.
 MITRAL REGURGITATION

• MR leads to a leakage of blood between the LV and LA during systole.

• Causes: RHD, IHD, Degenerative, IE, trauma, tumour, MVP, HCM
• Diagnosis: ECG, Echo, CMR, CXR
• Rx:
- Asymptomatic patients are normally followed up yearly.
- The MV may be repaired or replaced surgically.
- Symptomatic patients with an EF >30% or asymptomatic patients with an
EF <60% usually considered for surgery.
- Percutaneous techniques such as MitraClip can be used for patients who
are not suitable for surgery
 PULMONARY VALVE DISEASES

• Pulmonary valve disorders are usually related to a congenital problem,

such as tetralogy of Fallot, and will usually be discovered in infants more
commonly than in adults.
• Causes: Congenital, Carcinoma, IE
• Common signs and symptoms: Peripheral oedema, Abdominal distension,
Shortness of breath.
• Diagnosis: ECG, Echo
• Management: Repair or replacement either percutaneously or open heart
surgery
 Tricuspid Valve Diseases

• Tricuspid valve disease is rare. Tricuspid valve disease may exist in the
presence of disease of the MV or aortic valve.
• Causes: Rheumatic fever (rare in developed countries), Congenital
disorders, Trauma, e.g. pacing wire insertion.
• Common signs and symptoms of tricuspid valve disorders include the
following: Peripheral oedema, Abdominal distension.
• Diagnosis: ECG, Echo, CXR
• Management: Diuretics may be required. Surgery may be considered at the
time for surgery for left heart problem.
• Criteria to consider in the management of valvular heart disease (VHD)
(data from ESC guidelines)
- How severe is VHD?
- What is the aetiology?
- Is the patient experiencing any symptoms and if so are they related to
VHD?
- Do benefits of the intervention outweight the risks?
- What is the life expectancy and quality of life?
- Are there any signs in the symptomatic patient that indicate worse
outcomes if the treatment is delayed?
- What is the optimal treatment modality?
- What are the patient’s wishes?
 RHEUMATIC HEART DISEASE

• Rheumatic fever (RF) is an inflammatory disease that can involve the 

heart, joints, skin, and brain.
- The disease typically develops two to four weeks after a 
streptococcal throat infection. 
- Signs and symptoms include fever, multiple painful joints, 
involuntary muscle movements, and occasionally a characteristic non-itchy
 rash known as erythema marginatum.
- The heart is involved in about half of the cases. 
• Damage to the heart valves, known as rheumatic heart disease (RHD),
usually occurs after repeated attacks but can sometimes occur after
one. The damaged valves may result in heart failure, atrial fibrillation and 
infection of the valves.
• Diagnosis of acute rheumatic fever
- Modified Jones criteria ( 2 major or 1 major + 2 minor)
- Major: Carditis, arthritis, subcutaneous nodules, erythema nodosum &
Sydenham’s chorea.
- Minor: Arthralgia, Fever, Raised ESR, ECG- prolonged PR
• Prevention:
- The management of rheumatic fever is directed toward the reduction of
inflammation with anti-inflammatory medications such as aspirin or 
corticosteroids. Individuals with positive cultures for strep throat should
also be treated with antibiotics.
- Aspirin is the drug of choice and should be given at high doses.
- Monthly injections of long-acting penicillin must be given for a period of
five years in patients having one attack of rheumatic fever. If there is
evidence of carditis, the length of therapy may be up to 40 years
 CONGENITAL HEART DISEASES

• Congenital heart disease is a term used to cover a wide range of cardiac

conditions that result from an abnormality of cardiac structure or function
present at birth.
• Classification of congenital heart defects:
 ATRIAL SEPTAL DEFECT

• An ASD is an opening or communication between the left and right atria,

with midseptal defects occurring most frequently.
• There are four types: ostium secundum (most common, centre of atrium),
sinus venosus (high in the RA near the SVC), ostium primum (low in
atrium), and coronary sinus defects.
• Adults with untreated ASDs might present with gradually worsening
symptoms in their 40s onwards.
• If a significant shunt has developed, the ASD should be closed regardless of
symptoms to prevent further problems. The majority of secundum defects
can be closed percutaneously if the defect is <4cm.
• Other types of ASDs and larger secundum defects require surgical closure.
 PATENT FORAMEN OVALE

• A PFO is a remnant of normal fetal circulation rather than a true ASD and is
present in up to 30% of the population.
• An unexplained stroke (CVA)/ Migraine with aura.
• There may not be any signs or symptoms and it may also not show any
abnormalities on ECG, CXR, or Echo.
• Indications for closure include embolic stroke. It can also be done for
severe recurrent migraine
• Closure is usually by deploying a percutaneous closure device.
 Ventricular Septal defect

• This is the most common defect found in children, but 70% of VSDs close
spontaneously.
• If significant, left-to-right shunting is present; the defect is usually
surgically closed in childhood. Left untreated it can lead to CCF, high PVR
and infective endocarditis (IE).
• Adults with small, persistent VSDs require regular follow-up. Some might
present with IE later in life. Patients may present with heart blocks, valve
problems, or ventricular arrhythmias.
• VSDs may be closed surgically or percutaneously depending on the size
and location of the VSD.
 OTHER ACYANOTIC LESIONS

• Pulmonary Stenosis
- Valvular, subvalvular, or supravalvular. It is often associated with other
defects, such as an ASD or VSD.
- Severe PS requires treatment, which is usually in the form of balloon
valvoplasty; although in some cases surgical valvotomy is required.
• Coarctation of Aorta
- Narrowing of the aorta, commonly just below the origin of the left
subclavian artery.
- The patient usually has a weak or absent femoral pulse. Hypertension is
common and should be treated pharmacologically.
- Requires surgical or percutaneous intervention.
• Patent Ductus Arteriosus
- The ductus arteriosus should close shortly after birth. Most patients with a
PDA will have had corrective surgery in childhood.
- Adults with a persistent small lumen ductus arteriosus might be discovered
in later life but are usually asymptomatic.
• Bicuspid aortic valve
- The patient may be born with two aortic
valve leaflets or may have three, but two
can be fused together.
- They may present with symptoms of aortic
stenosis.
- There is a risk of developing IE, aortic
dissection, or rupture.
- The treatment includes valvuloplasty,
valvotomy, or valve replacement
 CYANOTIC LESIONS

• Tetralogy of fallot
- VSD+ PS+ RVH + overriding aorta
- Cyanosis develops as systemic
deoxygenated venous blood mixes
with oxygenated pulmonary venous blood.
- Surgical intervention is almost always
required in early childhood and depends
on the severity of the abnormalities
• Transposition of Great Arteries

- The aorta arises from the RV and

the PA arises from the LV.

- Also one of PFO, ASD, VSD or PDA

- Surgical intervention is mandatory.

This would normally have been a
Mustard or Senning Procedure
• Truncus Arteriosus (Common arterial trunk)
- The aorta and pulmonary artery form one common arterial trunk. The patient usually
has a VSD. There may also be valve abnormalities.
- Corrective surgery is usually performed in childhood.
• Hypoplastic left heart
- The left ventricle is underdeveloped (hypoplastic). The MV and AV are either narrow or
completely blocked. The aorta may also be hypoplastic.
- Prostaglandin will be given to keep the ductus arteriosus open
• Tricuspid Atresia
- The RV is usually very small. The tricuspid valve fails to open and therefore there is no
connection between the RA and RV.
- The patient may have an ASD and VSD. Associated abnormalities include PS, TGA,
pulmonary atresia, and COA.
- Treatment will depend on the blood flow to the lungs and may include pulmonary
artery banding.
 INFECTIVE ENDOCARDITIS

• IE occurs when the endocardium is exposed to an infecting organism, with

resultant colonization and tissue damage.
• There are several factors that can predispose individuals to developing IE:
- Prosthetic valves—the subvalvular ring is a common site in mechanical
valves.
- Congenital heart disorders—mostly those with high-flow or turbulent jet
streams (e.g. tetralogy of Fallot, VSD, bicuspid aortic valve, PDA, and
coarctation of the aorta).
- Valvular heart disease—most commonly AR, AS, and MR.
- History of rheumatic fever, because this can damage cardiac valves.
- Hypertrophic cardiomyopathy.
• Common infective organisms
- Streptococcus viridans. Common after dental work and the most frequent
causative organism in NVE.
- Streptococcus bovis. This is common in patients with underlying colon
cancer.
- Enterococcus faecalis. Common in elderly ♂ after genitourinary surgery and
in young ♀ after gynaecological procedures and childbirth.
- Staphylococcus aureus. Common in NVE and IV drug users. It often causes
metastatic abscesses.
- Staphylococcus epidermidis. The most common cause of PVE.
- Fungal
 Clinical Management
• Because symptoms are often nonspecific, it can be difficult to make a
definitive diagnosis.
• IE is usually diagnosed on the basis of clinical presentation, predisposing
factors, positive blood cultures, and echocardiography.
• Treatment includes:
- Antibiotics
- Supportive therapy
- Surgical intervention, such as valve replacement surgery, may be necessary
in approximately half of patients with IE because of complications.
 MYOCARDITIS

• Myocarditis is inflammation of the myocardium. It may be associated with

pericarditis—myopericarditis. It causes a wide range of symptoms, from
minor influenza-like symptoms to severe heart failure and sudden death.
• Patients seek medical help when the following occur: *Chest pain
*Dyspnoea *Palpitations *Syncope *Fatigue *Fever.
• Causes of myocarditis • Viral • Fungal • Bacterial • Protozoal • Drug-
induced• Sarcoidosis • Others (e.g. radiation, peripartum, and
autoimmunity).
• Investigations: ECG/Echo/ CXR/Blood tests/CMR/Biopsy
 Nursing considerations

• Initially continuous cardiac monitoring is advised to assess for signs of

arrhythmias and ST changes.
• Monitor BP and pulse to assess cardiovascular status at least every 4h— i
frequency if symptoms deteriorate.
• Record temperature at least every 4h.
• Monitor fluid status—either using a fluid-balance chart or by measuring the
patient’s weight daily to detect developing heart failure or evaluate
treatment of heart failure.
• Risk assessment for pressure ulcers is also indicated because the patient is
immobile—implement preventive measures, depending on risk assessment
score.
• Antiembolism stockings are contraindicated if peripheral oedema is present.
 PERICARDIAL DISEASES

• Pericardium: Fibrous & Visceral; 10-30 ml serous fluid.

• Acute pericarditis, Constrictive pericarditis, pericardial effusion, cardiac tamponade
• Causes of pericardial disease include the following:
Infection—viral, fungal, parasitic, or bacterial.
Collagen and connective tissue diseases—e.g.SLE, PAN, and scleroderma.
Autoimmune disorders—rheumatoid arthritis, rheumatic fever, postmyocardial infarction (MI) (Dressler’s syndrome)
Acute MI.
Bleeding into the pericardium—trauma, dissecting aortic aneurysm, or warfarin therapy.
Neoplastic disease—usually invasive from other organs, e.g. lung, breast, or skin.
Radiation.
Hypothyroidism.
Acute renal failure.
Metabolic—uraemia or gout.
Idiopathic—no known cause.
 CARDIAC TAMPONADE

• Pericardial effusion is an abnormal collection of fluid, clots, blood, or gas in

the pericardial sac. If this causes haemodynamic comprise it becomes
cardiac tamponade. Tamponade usually occurs quite rapidly. If fluid builds
up slowly, it can be quite well tolerated.
• The symptoms include tachycardia, raised CVP or JVP, with a further
increase during inspiration, low BP, SOB, muffled heart sounds, signs of low
CO, and, occasionally, abdominal pain. In acute cases, e.g. following cardiac
surgery, cardiac arrest could occur.
• Management depends on the patient’s condition.
- Many patients have pericardial effusions after cardiac surgery that resolve without problems.
- If an effusion is recurring, a pericardial window might be created.
- If the patient is severely compromised, pericardiocentesis (needle aspiration) is performed.
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