Structural heart defects Flashcards by Eve Setford

What type of care issues may arise from congenital heart disease?

Intellectual disability
Psychosocial issues
Transition
Explaining lesion and prognosis
Building independence/self resilience

How well did you know this?

Not at all

Perfectly

What complication may occur in later life in people who suffered from tetralogy of Fallot as a child?

Pulmonary valve regurgitation - requires surgery to fix

How well did you know this?

Not at all

Perfectly

What are the possible causes of congenital heart disease?

Maternal prenatal rubella infection
Maternal alcohol misuse
Maternal drug treatment and radiation
Genetic abnormalities
Chromosomal abnormalities

How well did you know this?

Not at all

Perfectly

Which congenital heart diseases would be classes as acyanotic with shunts?

Atrial septal defects
Ventricular septal defects
Patent ductus arteriosus
Partial anomalous venous drainage

How well did you know this?

Not at all

Perfectly

Which congenital heart diseases would be classed as acyanotic without shunts?

Coarctation of aorta

Congenital aortic stenosis

How well did you know this?

Not at all

Perfectly

Which congenital heart diseases would be classed as cyanotic with shunts?

Fallot’s tetralogy
Transposition of great vessels
Severe Ebstein’s anomaly

How well did you know this?

Not at all

Perfectly

Which congenital heart diseases would be classed as cyanotic without shunts?

Severe pulmonary stenosis
Tricuspid atresia
Pulmonary atresia
Hypoplastic left heart

How well did you know this?

Not at all

Perfectly

What are the symptoms and signs of congenital heart disease?

Central cyanosis
Pulmonary hypertension
Clubbing of fingers
Paradoxical embolism
Polycythaemia
Growth retardation
Syncope
Squatting

How well did you know this?

Not at all

Perfectly

How may a congenital heart disease present?

Endocarditis
Progression of valvular lesions
Atrial and ventricular arrhythmias
Sudden cardiac death
Right heart failure
End-stage heart failure

How well did you know this?

Not at all

Perfectly

What happens to blood in the heart in ventricular septal defects?

Blood moves from high pressure in left side to low pressure in right through defect in septum

How well did you know this?

Not at all

Perfectly

What are the haemodynamic consequences of large defects?

Pulmonary hypertension and eventual Eisenmenger’s complex

How well did you know this?

Not at all

Perfectly

How would a large non-restrictive VSD present?

Heart failure symptoms in childhood - LA and LV dilatation due to LV volume overload

How well did you know this?

Not at all

Perfectly

What type of murmur is associated with small restrictive VSDs?

Loud pan-systolic

How well did you know this?

Not at all

Perfectly

What may be seen on CXR and ECG in someone with a large VSD?

Prominent pulmonary arteries
Cardiomegaly
LVH or RVH on ECG

How well did you know this?

Not at all

Perfectly

What type of intervention is used for VSDs?

Surgical patch repair

Device closure if isolated muscular VSD

How well did you know this?

Not at all

Perfectly

What are the indications for intervention in VSD?

LA and LV enlargement with/without early LV dysfunction
Reversible pulmonary hypertension with residual L-R shunt and no significant desaturation with exercise
Infective endocarditis

How well did you know this?

Not at all

Perfectly

What are the 3 main types of atrial septal defects and where would they be found?

Sinus venosus defects - superior/inferior part of septum near SVC/IVC
Ostium secundum defects - mid-septum
Ostium primum (AV septal) defects - lower part of atrial septum

How well did you know this?

Not at all

Perfectly

What symptoms would occur in an adult with an unprepared ASD?

Dyspnoea and exercise intolerance
May develop atrial arrhythmias from RA dilatation
Increased pulmonary vascular flow

How well did you know this?

Not at all

Perfectly

What investigations would be carried out for ASD and what would they show?

CXR - prominent pulmonary arteries
ECG - RBBB and right axis deviation (left deviation in ostium primum)
Echocardiography - hypertrophy and dilatation of right heart and pulmonary arteries
CMR and CT

How well did you know this?

Not at all

Perfectly

How would you calculate the left to right shunt in ASDs?

Subcostal view with 2D and colour Doppler on echocardiography

How well did you know this?

Not at all

Perfectly

What are the indications for intervention in ASDs?

ASD with significant L-R shunting > RA/RV enlargement - should be closed irrespective of symptoms
Thromboembolic events, including patients with patent foramen ovale

How well did you know this?

Not at all

Perfectly

What type of intervention can be used for ASDs?

Device closure using transcatheter clamshell device for secundum ASDs
Surgical closure for all other types

How well did you know this?

Not at all

Perfectly

What problem occurs in patent ductus arteriosus?

Failure of ductus arteriosus to close after birth > persistent communication between proximal left pulmonary artery and descending aorta > continuous L-R shunt

How well did you know this?

Not at all

Perfectly

What drug can be given in patent ductus arteriosus to stimulate the closure of the ductus arteriosus?

Indometacin (prostaglandin inhibitor)

How well did you know this?

Not at all

Perfectly

What are the characteristic clinical signs of PDA?

Bounding pulse and continuous 'machinery murmur' | As pulmonary hypertension develops, murmur becomes softer

What investigations should be done in PDA and what would they show?

CXR - prominent aorta and pulmonary arterial system ECG - LA abnormality and LVH Echocardiography - dilated LA and LV with right heart changes occurring late

What are the indications for intervention in PDA?

LV dilatation | Mild to moderate pulmonary arterial hypertension

What intervention would be used for PDA?

Percutaneous devices

Small PDA defects may predispose to...

Endarteritis

What is coarctation of the aorta?

Narrowing of the aorta at or just distal to insertion of ductus arteriosus

Which syndrome is associated with coarctation of the aorta?

Turner's syndrome

In 80% of cases of coarctation of the aorta, what is wrong with the aortic valve?

Bicuspid and potentially stenotic or endocarditic

What conditions are associated with coarctation of the aorta?

Patent ductus arteriosus Ventricular septal defect Mitral stenosis or regurgitation Circle of Willis aneurysm

What symptoms may be present in coarctation of the aorta?

Headaches and nosebleeds due to hypertension | Claudication and cold legs due to poor blood flow

What would be found on physical examination in someone with coarctation of the aorta?

Hypertension in upper limbs and weak, delayed pulses in legs (radiofemoral delay) Murmur Right arm hypertension Buzzes over scapula and back from collateral vessels

When may asynchronous radial pulses in left and right arms be seen?

Coarctation of the aorta proximal to left subclavian

What investigations would be done for coarctation of the aorta and what would be found?

CXR - dilated aorta indented at site of coarctation, rib notching ECG - LVH Echocardiography - coarctation and associated abnormalities CT and CMR - demonstrate coarctation and quantify flow

When would intervention be required in someone with coarctation of the aorta?

Peak-peak gradient across coarctation >20mmHg and/or proximal hypertension

What intervention is preferred by most centres for coarctation of the aorta in neonates as well as older children and adults?

Surgical repair

What alternative intervention can be used for older children and young adults for coarctation of the aorta?

Balloon dilatation and stenting

What does Fallot's tetralogy consist of?

Large mal-aligned VSD Overriding aorta RV outflow tract obstruction RVH

When would cyanosis develop in Fallot's tetralogy?

Increased right sided pressure causing right to left shunt

What are Fallot's spells and how are they relieved?

Episodes of severe cyanosis in children due to spasm of sub pulmonary muscle; relieved by increasing systemic resistance by squatting

What happens to the heart in complete transposition of great arteries?

RA connects to morphological RV > aorta LA connects to morphological LV > pulmonary artery Blood circulates in two parallel circuits: deoxygenated blood > systemic; oxygenated blood > lungs

What procedure is carried out in babies as soon as they are born with TGA?

Atrial septostomy: Rashkind's balloon used to dilate foramen ovale and main saturations at 50-80% until definitive procedure can be performed

What procedure is performed within the first 2 weeks of a baby's life with TGA?

Arterial switch procedure - aorta reconnected to LV and pulmonary artery connected to RV; coronary arteries reimplanted

What limits the life expectancy in people with TGA?

Eventual failure of systemic RV

What happens in congenitally corrected TGA?

Systemic venous return to RA enters morphological LV > pulmonary artery Pulmonary venous blood returns to LA then enters morphological RV > aorta

What is ccTGA often associated with?

``` Cardiac lesions Systemic (tricuspid) AV valve abnormalities with valve insufficiencies VSD Sub-pulmonary stenosis Complete heart block Wolff-Parkinson-White syndrome Dextrocardia ```

What interventions may some people require if they have ccTGA?

Pacemaker | Surgery for regurgitant tricuspid

What would be seen in a child with a large VSD?

High pulmonary blood flow Breathless Poor feeding Failure to thrive

What are the clinical signs of a large VSD?

``` Small breathless skinny baby Increased respiratory rate Tachycardia Cardiomegaly Murmur varies in intensity ```

What are the clinical signs of a small VSD?

Loud systolic murmur Thrill (buzzing sensation) Well grown Normal heart size/rate

What are the clinical signs of ASD?

Pulmonary flow murmur Fixed split second heart sound Big pulmonary arteries on CXR Cardiomegaly on CXR

What symptoms and signs would be seen in someone with a complete AV septal defect?

``` Breathless as neonate Poor weight gain Poor feeding Torrential pulmonary blood flow Needs repair or PA band in infancy ```

What symptoms and signs would be seen in someone with a partial AV septal defect?

Presents like small VSD/ASD | Can present in late adulthood

What symptoms and signs would be seen in someone with a large PDA?

Torrential flow from aorta to pulmonary arteries in infancy | Breathless, poor feeding, failure to thrive

What symptoms and signs would be seen in someone with a small PDA?

Usually asymptomatic | Murmur found incidentally

What are some of the long-term problems that can arise from coarctation of the aorta?

Hypertension - early coronary artery disease, early strokes, sub arachnoid haemorrhage Re-coarctation requiring repeat intervention Aneurysm formation at site of repair

What are the clinical signs of severe pulmonary stenosis?

``` Right ventricular failure as neonate Collapse Poor pulmonary blood flow RVH Tricuspid regurgitation ```

What treatment would be used for pulmonary stenosis?

Balloon valvuloplasty Open valvotomy Open trans-annular patch Shunt (to bypass blockage)

Describe what happens in Eisenmenger's syndrome

High pressure pulmonary blood flow > damages to delicate pulmonary vasculature > resistance to blood flow through the lungs increases > RV pressure increases > shunt direction reverses > cyanosis