Ventricular Septal Defect - Clinicals, Diagnosis, and Management

Cardiovascular

Clinicals - History

Fact Explanation
Asymptomatic , Small ventricular septal defects (VSD) less than 0.5 cm in diameter are usually asymptomatic. 40% of small VSD's close spontaneously in early childhood and muscular VSD's are more likely to close spontaneously. ,,, Asymptomatic ,
Small ventricular septal defects (VSD) less than 0.5 cm in diameter are usually asymptomatic. 40% of small VSD's close spontaneously in early childhood and muscular VSD's are more likely to close spontaneously. ,,,
Breathlessnes Medium sized VSD's are uncommon
unless if it is associated with protective valvular or sub valvular pulmonary stenosis (25% to 30%). Those patients present with dyspnea.
Breathlessnes
Medium sized VSD's are uncommon
unless if it is associated with protective valvular or sub valvular pulmonary stenosis (25% to 30%). Those patients present with dyspnea.
Cyanosis - bluish discoloration This occurs only if Eisenmenger syndrome is present. Patients with large VSD have a left to right shunt that causes systemic circulation to be underfilled and the pulmonary circulation to be overfilled. This congestion alters the gas exchange at alveoli. Therefore present in adolescence with symptoms as cyanosis, dyspnoea and syncope. , Cyanosis - bluish discoloration
This occurs only if Eisenmenger syndrome is present. Patients with large VSD have a left to right shunt that causes systemic circulation to be underfilled and the pulmonary circulation to be overfilled. This congestion alters the gas exchange at alveoli. Therefore present in adolescence with symptoms as cyanosis, dyspnoea and syncope. ,
Pulmonary hypertension Patients with a large VSD usually present in infancy. It can be present with pulmonary stenosis and pulmonary hypertension. Pulmonary hypertension commonly presents in adolescence with symptoms of cyanosis, dyspnoea and syncope. Pulmonary hypertension
Patients with a large VSD usually present in infancy. It can be present with pulmonary stenosis and pulmonary hypertension. Pulmonary hypertension commonly presents in adolescence with symptoms of cyanosis, dyspnoea and syncope.
Syncope , Patients with large VSD's usually present in infancy. It can coexist with pulmonary stenosis and pulmonary hypertension. Pulmonary hypertension commonly presents in adolescence with symptoms of cyanosis, dyspnoea and syncope ,. Patients with VSD and Aortic Regurgitation also present with syncope secondary to the right ventricular outflow
tract obstruction due to a prolapsing coronary
cusp, or heart failure due to progressive left ventricular volume overload.
Syncope ,
Patients with large VSD's usually present in infancy. It can coexist with pulmonary stenosis and pulmonary hypertension. Pulmonary hypertension commonly presents in adolescence with symptoms of cyanosis, dyspnoea and syncope ,. Patients with VSD and Aortic Regurgitation also present with syncope secondary to the right ventricular outflow
tract obstruction due to a prolapsing coronary
cusp, or heart failure due to progressive left ventricular volume overload.
Cardiac failure , This occurs due to progressive left ventricular
volume overload,
Cardiac failure ,
This occurs due to progressive left ventricular
volume overload,
Infective endocarditis (IE) IE occurs when there is a interaction between the bloodstream pathogen with matrix molecules and platelets at sites of endocardial cell damage. Initially there is formation of nonbacterial thrombotic endocarditis (NBTE) on the surface of a cardiac valve or elsewhere that endothelial damage occurs, bacteremia, later adherence of the bacteria in the bloodstream to NBTE and proliferation of bacteria within a vegetation occurs. Infective endocarditis (IE)
IE occurs when there is a interaction between the bloodstream pathogen with matrix molecules and platelets at sites of endocardial cell damage. Initially there is formation of nonbacterial thrombotic endocarditis (NBTE) on the surface of a cardiac valve or elsewhere that endothelial damage occurs, bacteremia, later adherence of the bacteria in the bloodstream to NBTE and proliferation of bacteria within a vegetation occurs.

Clinicals - Examination

Fact Explanation
Murmur Typical murmur heard in VSD is a harsh holosystolic plateau-shaped murmur of relatively high frequency, best heard in the
left sternal border. If the defect is subarterial blood is shunted directly into the pulmonary artery and due to that the murmur is heard maximally in the second intercostal space and may become “diamond-shaped” (crescendo–decrescendo) or simply consist of a systolic ejection component.
If the defect is muscular, the murmur may stop well before s2 because the defect decreases in size or obliterates in the later part of systole.
In a VSD with mild pulmonary stenosis, the murmur is holosystolic but the
pulmonary closure sound is delayed. A VSD with moderately severe pulmonary stenosis, the murmur gets shorter as the left to right shunt diminishes, and the pulmonary sound is soft and delayed. The VSD with severe pulmonary stenosis, the murmur is replaced by a long systolic ejection murmur which is typical of pulmonary stenosis.,
Murmur
Typical murmur heard in VSD is a harsh holosystolic plateau-shaped murmur of relatively high frequency, best heard in the
left sternal border. If the defect is subarterial blood is shunted directly into the pulmonary artery and due to that the murmur is heard maximally in the second intercostal space and may become “diamond-shaped” (crescendo–decrescendo) or simply consist of a systolic ejection component.
If the defect is muscular, the murmur may stop well before s2 because the defect decreases in size or obliterates in the later part of systole.
In a VSD with mild pulmonary stenosis, the murmur is holosystolic but the
pulmonary closure sound is delayed. A VSD with moderately severe pulmonary stenosis, the murmur gets shorter as the left to right shunt diminishes, and the pulmonary sound is soft and delayed. The VSD with severe pulmonary stenosis, the murmur is replaced by a long systolic ejection murmur which is typical of pulmonary stenosis.,
Palpable thrill In a small VSD a palpable thrill is present in 3rd or 4th intercostal space. Palpable thrill
In a small VSD a palpable thrill is present in 3rd or 4th intercostal space.
Palpable P2 (pulmonary valve closure) With VSD there is left to right shunt and blood flow in pulmonary circulation increase causing pulmonary hypertension and right ventricular hypertrophy. ,, Palpable P2 (pulmonary valve closure)
With VSD there is left to right shunt and blood flow in pulmonary circulation increase causing pulmonary hypertension and right ventricular hypertrophy. ,,
Heave at the sternal edge With VSD there is left to right shunt and blood flow in pulmonary circulation increase causing pulmonary hypertension and right ventricular hypertrophy. ,, Heave at the sternal edge
With VSD there is left to right shunt and blood flow in pulmonary circulation increase causing pulmonary hypertension and right ventricular hypertrophy. ,,
Jugular Venous Pressure In VSD most patients get increased venous pressure, with a predominant “A” wave
because of the right ventricular hypertrophy and decreased compliance . A "V" wave is seen if there is a failing right ventricle and tricuspid regurgitation.
Jugular Venous Pressure
In VSD most patients get increased venous pressure, with a predominant “A” wave
because of the right ventricular hypertrophy and decreased compliance . A "V" wave is seen if there is a failing right ventricle and tricuspid regurgitation.
Cyanosis If the VSD progress into Eisenmenger syndrome these signs can be seen. As deoxygenated blood is shunted into the systemic circulation. Cyanosis
If the VSD progress into Eisenmenger syndrome these signs can be seen. As deoxygenated blood is shunted into the systemic circulation.
Clubbing If the VSD progress into Eisenmenger syndrome these signs can be seen. Clubbing
If the VSD progress into Eisenmenger syndrome these signs can be seen.
Wide pulse pressure If there is aortic regurgitation with the VSD there will be wide pulse pressure and other features of aortic regurgitation such as collapsing pulse, dancing carotids, uvula pulsation, capillary pulsation etc. Wide pulse pressure
If there is aortic regurgitation with the VSD there will be wide pulse pressure and other features of aortic regurgitation such as collapsing pulse, dancing carotids, uvula pulsation, capillary pulsation etc.

Investigations - Diagnosis

Fact Explanation
Echocardiogram Sensitivity - 88% Specificity - 95%. It is most sensitive for VSDs larger than 5 mm, in the membranous inlet or outlet portion of the septum. It is least sensitive for apical muscular defects. It can identify the morphological features as size, borders and associated defects. It also provides an accurate assessment of the shunt, severity, volume overload, subpulmonic or pulmonic stenosis, and pulmonary hypertension. Echocardiogram
Sensitivity - 88% Specificity - 95%. It is most sensitive for VSDs larger than 5 mm, in the membranous inlet or outlet portion of the septum. It is least sensitive for apical muscular defects. It can identify the morphological features as size, borders and associated defects. It also provides an accurate assessment of the shunt, severity, volume overload, subpulmonic or pulmonic stenosis, and pulmonary hypertension.

Investigations - Management

Fact Explanation
Electrocardiogram (ECG/EKG) Preexisting ECG abnormalities depend on the size and location of the VSD. Commonest abnormality is Right Bundle Branch Block (RBBB). When VSD progress into Eisenmenger syndrome the ECG shows right axis deviation, right atrial and ventricular enlargment and ventricular hypertrophy. Electrocardiogram (ECG/EKG)
Preexisting ECG abnormalities depend on the size and location of the VSD. Commonest abnormality is Right Bundle Branch Block (RBBB). When VSD progress into Eisenmenger syndrome the ECG shows right axis deviation, right atrial and ventricular enlargment and ventricular hypertrophy.
Chest X-ray (CXR) Small VSD - Normal CXR
When a VSD progress into Eisenmenger syndrome cardiomegaly is seen in CXR
Chest X-ray (CXR)
Small VSD - Normal CXR
When a VSD progress into Eisenmenger syndrome cardiomegaly is seen in CXR
Cardiac catherterization Cardiac catheterization is an important method used to assess pulmonary vascular resistance in VSD especially when complicated. Cardiac catherterization
Cardiac catheterization is an important method used to assess pulmonary vascular resistance in VSD especially when complicated.
Cardiac catheterization This is an important method used to
assess pulmonary vascular resistance in VSD especially when complicated. Should be routinely performed prior to surgical closure.
Cardiac catheterization
This is an important method used to
assess pulmonary vascular resistance in VSD especially when complicated. Should be routinely performed prior to surgical closure.

Management - Supportive

Fact Explanation
Endocarditis prophylaxis Before a dental procedure - Amoxicillin 2g oral, or Ampicillin or Cefazolin or ceftriaxone 2g IM/IV
Before invasive procedure in respiratory tract (without ongoing infection) - same as dental procedure
Before invasive procedure in respiratory tract(with ongoing infection) - antibiotic regime should act against viridans group of sptreptococci.
Prior to Gastrointestinal or Genitourinary procedures - antibiotic prophylaxis is not recommended.
Endocarditis prophylaxis
Before a dental procedure - Amoxicillin 2g oral, or Ampicillin or Cefazolin or ceftriaxone 2g IM/IV
Before invasive procedure in respiratory tract (without ongoing infection) - same as dental procedure
Before invasive procedure in respiratory tract(with ongoing infection) - antibiotic regime should act against viridans group of sptreptococci.
Prior to Gastrointestinal or Genitourinary procedures - antibiotic prophylaxis is not recommended.

Management - Specific

Fact Explanation
Surgical repair In a small VSD, surgical repair is consider if a second attack of endocarditis occurs but some evidence suggests it is better to do surgical repair even without endocarditis as there is a risk of developing volume overload in left ventricle due to the long standing shunt.
A medium to large VSD is not common among adults as most are surgically repaired during childhood. If the patient has pulmonary hypertension decision to do the surgery is taken after cardiac catheterization evaluation.
Surgical repair
In a small VSD, surgical repair is consider if a second attack of endocarditis occurs but some evidence suggests it is better to do surgical repair even without endocarditis as there is a risk of developing volume overload in left ventricle due to the long standing shunt.
A medium to large VSD is not common among adults as most are surgically repaired during childhood. If the patient has pulmonary hypertension decision to do the surgery is taken after cardiac catheterization evaluation.
Device closure Transcatheter device closure is an alternative strategy for management of both complex muscular and postoperative residual VSDs.This is especially valuable in patients with associated complex congenital heart disease. ,,, Device closure
Transcatheter device closure is an alternative strategy for management of both complex muscular and postoperative residual VSDs.This is especially valuable in patients with associated complex congenital heart disease. ,,,
Management of Pulmonary hypertension: General Measures Encourage to be active within symptom limits and avoid severe exertion. Management of Pulmonary hypertension: General Measures
Encourage to be active within symptom limits and avoid severe exertion.
Management of Pulmonary hypertension: Pregnancy and Birth Control There is a 30-50% risk of mortality associated pregnancy in pulmonary hypertension. Recommended methods are barrier methods (but have questionable efficacy), Mirena and progesterone only preparations such as medroxyprogesterone acetate and etonogestrel. Management of Pulmonary hypertension: Pregnancy and Birth Control
There is a 30-50% risk of mortality associated pregnancy in pulmonary hypertension. Recommended methods are barrier methods (but have questionable efficacy), Mirena and progesterone only preparations such as medroxyprogesterone acetate and etonogestrel.
Management of Pulmonary hypertension: Supportive therapy Oral anticoagulants (Warfarin) is given for the prevention of vascular thrombotic lesions. Diuretics can be prescribed in event of Right Heart Failure for fluid retention. While supplemental oxygen therapy has been shown to reduce pulmonary vascular resistance. Digoxin improves cardiac output and reduces the ventricular rate. Management of Pulmonary hypertension: Supportive therapy
Oral anticoagulants (Warfarin) is given for the prevention of vascular thrombotic lesions. Diuretics can be prescribed in event of Right Heart Failure for fluid retention. While supplemental oxygen therapy has been shown to reduce pulmonary vascular resistance. Digoxin improves cardiac output and reduces the ventricular rate.
Management of Pulmonary hypertension: Specific pharmacological management Specific management includes, Calcium Channel Blockers to prevent smooth muscle cell hypertrophy, hyperplasia, and vasoconstriction. Prostacyclins are used for their property of being a potent vasodilator. Sildenafil is a commonly prescribed drug for pulmonary hypertension it is a phosphodiesterase type 5 inhibitor. Management of Pulmonary hypertension: Specific pharmacological management
Specific management includes, Calcium Channel Blockers to prevent smooth muscle cell hypertrophy, hyperplasia, and vasoconstriction. Prostacyclins are used for their property of being a potent vasodilator. Sildenafil is a commonly prescribed drug for pulmonary hypertension it is a phosphodiesterase type 5 inhibitor.

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