Structural heart defects Flashcards
What type of care issues may arise from congenital heart disease?
Intellectual disability Psychosocial issues Transition Explaining lesion and prognosis Building independence/self resilience
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
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
Which congenital heart diseases would be classes as acyanotic with shunts?
Atrial septal defects
Ventricular septal defects
Patent ductus arteriosus
Partial anomalous venous drainage
Which congenital heart diseases would be classed as acyanotic without shunts?
Coarctation of aorta
Congenital aortic stenosis
Which congenital heart diseases would be classed as cyanotic with shunts?
Fallot’s tetralogy
Transposition of great vessels
Severe Ebstein’s anomaly
Which congenital heart diseases would be classed as cyanotic without shunts?
Severe pulmonary stenosis
Tricuspid atresia
Pulmonary atresia
Hypoplastic left heart
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 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
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
What are the haemodynamic consequences of large defects?
Pulmonary hypertension and eventual Eisenmenger’s complex
How would a large non-restrictive VSD present?
Heart failure symptoms in childhood - LA and LV dilatation due to LV volume overload
What type of murmur is associated with small restrictive VSDs?
Loud pan-systolic
What may be seen on CXR and ECG in someone with a large VSD?
Prominent pulmonary arteries
Cardiomegaly
LVH or RVH on ECG
What type of intervention is used for VSDs?
Surgical patch repair
Device closure if isolated muscular VSD
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
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
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
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 would you calculate the left to right shunt in ASDs?
Subcostal view with 2D and colour Doppler on echocardiography
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
What type of intervention can be used for ASDs?
Device closure using transcatheter clamshell device for secundum ASDs
Surgical closure for all other types
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
What drug can be given in patent ductus arteriosus to stimulate the closure of the ductus arteriosus?
Indometacin (prostaglandin inhibitor)
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
