Paediatric cardiac disorders

Question 1

Persistent cyanosis in an otherwise well infant is usually a sign of what?

Answer 1

Structural heart disease

Question 2

Cyanosis in a newborn infant with respiratory distress (respiratory rate > 60) could be due to:

Answer 2

Cardiac disorders (congenital cyanotic heart disease)
Respiratory disorders (e.g RDS, meconium aspiration, pulmonary hypoplasia)
Persistent pulmonary hypertension of the newborn (failure of pulmonary vascular resistance to fall after birth)
Infection (e.g. septicaemia from GBS or other organisms)
Inborn errors of metabolism (metabolic acidosis and shock)

Question 3

How congenital heart disease diagnosed?

Answer 3

If congenital heart disease is suspected, a CXR and ECG should be performed (these are rarely diagnostic but help demonstrate an abnormality)
Echocardiogrpahy with Doppler ultrasound enables almost all causes of congenital heart disease to be diagnosed
Specialist opinion should be sought if the child is haemodynamically unstable, evidence of heart failure, cyanosis, when oxygen saturation are <94% due to heart disease and when there are reduced volume pulses

Question 4

2 main types of ASD

Answer 4

Secundum ASD 80%
Partial atrioventricular septal defect (AVSD or primum ASD)

Both cause similar symptoms and signs

Question 5

Secundum ASD

Answer 5

Defect in the centre of the atrial septum

Involves the foramen ovale

Question 6

Partial ASD

Answer 6

Defect in the atrioventricular septum
There is an interatrial communication between the bottom end of the atrial septum and the atrioventricular valves (primum ASD)
There are abnormal atrioventricular valves, with a left atrioventricular valve which has three leaflets and tends to lead (regurgitant valve)

Question 7

Clinical features of ASD

Answer 7

No symptoms (common)
Recurrent chest infections/wheeze
Arrhythmias (4th decade on)

Ejection systolic murmur best heart at the upper left sternal edge (due to increased flow across the pulmonary valve because of left-to-right shunt)
Fixed and widely split second heart sound (often difficult to hear)- due to right ventricular stroke volume being equal in both inspiration and expiration
IN partial AVSD - an apical pansystolic murmur may be heard from atrioventricular valve regurgitation

Question 8

Investigations for ASD

Answer 8

CXR: Cardiomegaly
Enlarged pulmonary arteries
Increased pulmonary vascular markings

ECG: Partial RBBB and right axis deviation secondary to right ventricular enlargement (in secundum ASD)
Superior QRS, due to a defect in the middle of the heart where the AV node is found. This displaced node then conducts to the ventricles superiorly, resulting in an abnormal axis (partial AVSD)

Echocardiography is essential

Question 9

Management of ASD

Answer 9

Children with significant ASD (large enough to cause right ventricular dilatation) will require treatment

Secundum ASDs - managed by cardiac catheterisation with the insertion of an occlusive device (percutaneous closure/endovascular closure)  

Partial AVSD - managed by surgical correction  

NOTE: treatment is usually undertaken at 3-5 years of age

Question 10

Eisenmenger syndrome

Answer 10

If high pulmonary blood flow due to a large left-to-right shunt is not treated early, the pulmonary arteries become thick walled and resistance to flow increases
Gradually, the symptoms reduce as the shunt decreases
Eventually, at around 10-15 years, the shunt reverses and the teenager becomes blue

The disease is progressive, and the patient will die of right heart failure (usually 30-40 years old)
Treatment is aimed at prevention with early intervention for high pulmonary blood flow

Question 11

How can VSDs be categorised?

Answer 11

Small = smaller than the aortic valve (usually <3mm diameter)
Large = larger than the aortic valve

Question 12

Small VSD clinical features

Answer 12

Asymptomatic

Loud pansystolic murmur at lower left sternal edge (Loud murmur implies smaller defect)
Quiet pulmonary second heart sound

Question 13

Investigations for a suspected small VSD

Answer 13

CXR and ECG are usually normal

Echocardiography demonstrates the precise anatomical defect

Question 14

Management of small VSDs

Answer 14

The lesions will close spontaneously
This is demonstrated by disappearance of the murmur and a normal echocardiogram
Whilst the VSD is present, bacterial endocarditis should be prevented by maintaining good dental hygiene

Question 15

Clinical features of large VSDs

Answer 15

Heart failure with breathlessness and faltering growth after 1 week old
Recurrent chest infections

Tachypnoea, tachycardia and enlarged liver from heart failure
Active precordium (precordium moves too much)
Soft pansystolic murmur or no murmur
Apical mid-diastolic murmur (from increased blood flow across the mitral valve)
Loud pulmonary second sound (P2)- from raised pulmonary arterial pressure

Question 16

Investigations for large VSDs

Answer 16

CXR: Cardiomegaly
Enlarged pulmonary arteries
Increased pulmonary vascular markings
Pulmonary oedem

ECG: Biventricular hypertrophy by 2 months of age

Echocardiography: demonstrates the anatomy of the defect

Question 17

Management of large VSDs

Answer 17

Heart failure is treated with diuretics (often captopril)
Additional calorie input

Surgery is usually performed at 3-6 months to:
prevent permanent lung damage from pulmonary hypertension and high blood flow (i.e. prevent Eisenmenger syndrome)
Manage heart failure and faltering growth

Question 18

Patent ductus arteriosus

Answer 18

When the ductus arteriosus has failed to close by 1 month after the expected delivery date
Due to a defect in the constrictor mechanism of the duct
In preterm infants, the presence of PDA is due to prematurity and not due to congenital heart disease

Question 19

Clinical features of PDA

Answer 19

Continuous murmur below left clavicle
Murmur continues into diastole (because pulmonary arterial pressure is always lower than aortic pressure)
Increased pulse pressure (causing bounding pulse)

Question 20

Investigations for PDA

Answer 20

ECG and CXR are usually normal

Echocardiography

Question 21

Management of PDA

Answer 21

CLosure is recommended to abolish lifelong risk of bacterial endocarditis and of pulmonary vascular disease
If a cyanotic disease is dependent on a PDA (e.g. transposition of the great arteries), the patient should start a prostaglandin infusion to keep the PDA open until corrective surgery can be performed
Usually closed through a coil or occlusive device introduced through a cardiac catheter at about 1 year of age

Question 22

Left-to-right shunts

Answer 22

ASDs
VSDs
PDA

Question 23

Right-to-left shunts

Answer 23

Tetralogy of Fallot

Transposition of the great arteries

Question 24

How do right-to-left shunts present?

Answer 24

Cyanosis

Usually in the first week of life

Question 25

Hyperoxia (Nitrogen washout) test

Answer 25

Used to determine the presence of heart disease in a cyanosed neonate The infant is placed in 100% oxygen (head box or ventilator) for 10 mins If the right radial arterial pressure of oxygen (PaO2) remains low (<15kPa) after this, a diagnosis of cyanotic congenital heart disease can be made (lung disease and persistent pulmonary hypertension must be excluded) Blood gas analysis is necessary in this scenario because oxygen saturations are not reliable enough

Question 26

Management of the cyanosed neonate

Answer 26

Stabilise the airway, breathing and circulation, with artificial ventilation if necessary Start prostaglandin infusion (5ng/kg per minute) - Most infants with cyanotic heart disease presenting in the first week of life, are duct dependent - Maintaining duct patency is key to early survival - Prostaglandins help maintain duct patency - Side-effects of prostaglandins: apnoea, jitteriness, seizures, flushing, vasodilation, hypotension

Question 27

What is the most common cause of cyanotic heart disease?

Answer 27

Tetralogy of Fallot

Question 28

Clinical features of Tetralogy of Fallot

Answer 28

Large VSD Overriding aorta with respect to the ventricular septum Sub-pulmonary stenosis causing right ventricular outflow tract Right ventricular hypertrophy

Question 29

Symptoms of Tetralogy of Fallot

Answer 29

Most cases are diagnosed antenatally, or following the identification of a murmur in the first 2 months of life (cyanosis may not be obvious at this stage, but some may present with severe cyanosis) The classical description of Tetralogy of Fallot includes: Severe cyanosis Hypercyanotic spells (important to recognise as they can lead to MI, cerebrovascular accidents and death. They are characterised by a rapid increase in cyanosis, usually with irritability and inconsolable crying because of severe hypoxia. Breathlessness and pallor because of tissue acidosis may also be present) Squatting on exercise NB: these features develop in late infancy and are rare in developed countries

Question 30

Signs of Tetralogy of Fallot

Answer 30

Clubbing will develop in older children Loud harsh ejection systolic murmur at the left sternal edge from day 1 of life (NB: as right ventricular outflow tract obstruction increases, the murmur will shorten and cyanosis will increase)

Question 31

Investigations for Tetralogy of Fallot

Answer 31

CXR: Relatively small heart May have an up-tilted apex due to right ventricular hypertrophy Pulmonary artery bay - a concavity on the left heart border where a normal pulmonary artery and right ventricular outflow tract should be Decreased pulmonary vascular markings (due to reduced flow) ECG: Normal at birth Right ventricular hypertrophy in older children

Question 32

Management of Tetralogy of Fallot

Answer 32

Initially medical, with surgery at around 6 months Surgery - involves closing the VSD and relieving right ventricular outflow tract obstruction Very cyanosed neonates require a shunt to increase pulmonary blood flow This can be done by inserting an artificial tube between the subclavian artery and pulmonary artery or by balloon dilatation of the right ventricular outflow tract Hypercyanotic spells are usually self-limiting followed by a period of sleep If this is prolonged, they need prompt treatment: Sedation and pain relief (morphine) IV propranolol (works as a peripheral vasoconstrictor and by relieving sub-pulmonary muscle obstruction) IV volume administration Bicarbonate to correct acidosis Muscle paralysis and artificial ventilation to reduce metabolic oxygen demand

Question 33

Transposition of the great arteries

Answer 33

A condition in which the main arteries are connected the wrong way (aorta --> right ventricle, pulmonary artery --> left ventricle) This means that deoxygenated blood is returned to the body and oxygenated blood is returned to the lungs This means that there are two parallel circulations (which is not usually compatible with life) However, this condition tends to be associated with some naturally occurring associated anomalies (e.g. VSD, ASD< PDA)

Question 34

Clinical features of Transposition of the Great Arteries

Answer 34

Cyanosis (may be profound and life-threatening) Present on day 2 of life when ductal closure leads to a reduction in mixing of saturated and desaturated blood Second heart sound is often loud and single Usually no murmur, but may be a systolic murmur from increased flow or stenosis within the left ventricular outflow tract

Question 35

Investigations for Transposition of the Great Arteries

Answer 35

CXR: Narrow mediastinum with an egg on side appearance of the cardiac shadow (this is caused by the anteroposterior relationship of the great vessels, narrow vesicular pedicle and hypertrophied right ventricle) Increased pulmonary vascular markings (due to increased pulmonary blood flow) ECG usually normal Echocardiogrpahy is essential

Question 36

Management of Transposition of the Great Arteries

Answer 36

In the sick cyanosed neonate, you need to improve mixing. Maintain patency of ductus arteriosus with a prostaglandin infusion. Balloon atrial septostomy may be a life-saving procedure (breaks the flap valve of the foramen ovale and encourages mixing of blood) ALL patients will require surgery (arterial switch procedure in the neonatal period): NB- the coronary arteries also need to be transferred to the new aorta

Question 37

In what population is atrioventricular septal defect most commonly seen?

Answer 37

Children with Down syndrome

Question 38

AVSD

Answer 38

Defect in the middle of the heart with a single five-leaflet valve between the atria and ventricles It stretches across the entire atrioventricular junction and tends to leak The large defect leads to pulmonary hypertension

Question 39

Features of atrioventricular septal defects

Answer 39

Present at antenatal ultrasound screening Cyanosis at birth or heart failure at 2-3 weeks NO murmur heard Always a superior axis on the ECG

Question 40

Management of AVSD

Answer 40

Treat heart failure medically and surgical repair at 3-6 months

Question 41

What conditions are categorised under complex congenital heart disease?

Answer 41

Tricuspid atresia (MOST COMMON) Mitral atresia Double inlet left ventricle COmmon arterial trunk (truncus arteriosus)

Question 42

Tricuspid atresia

Answer 42

Only the left ventricle is effective. The right ventricle is small and non-functional. This is due to complete absence of the tricuspid valve.

Question 43

Clinical features of Tricuspid atresia

Answer 43

Common mixing of systemic and pulmonary venous return in the left atrium Cyanosis in the newborn period if duct-dependent The child may be well at birth and hen become cyanosed or breathless

Question 44

Management of tricuspid atresia

Answer 44

Early palliation to maintain a secure supply of blood to the lungs at low pressure by: - Ballock-Taussig shunt insertion (between subclavian and pulmonary arteries) - Pulmonary artery banding operation to reduce pulmonary blood flow if breathless IMPORTANT: complete corrective surgery is not possible in most cases because there is only one functioning ventricle Operations can be performed to connect the SVC to the pulmonary artery (Glenn) and the IVC to the pulmonary artery (Fontan)

Question 45

Outflow obstruction in the well child

Answer 45

Aortic stenosis Pulmonary stenosis Adult-type coarctation of the aorta

Question 46

Aortic stenosis

Answer 46

The aortic valve leaflets are partly fused together, thus restricting the exit from the left ventricle There could be 1-3 aortic leaflets Aortic stenosis is often associated with mitral valve stenosis and coarctation of the aorta

Question 47

Clinical features of aortic stenosis

Answer 47

Reduced exercise tolerance Chest pain Syncope NB: these only occur when stenosis is severe ``` Small volume, slow rising pulse Carotid thrill Ejection systolic murmur maximal at the upper right sternal edge and radiating to the carotids Delayed and soft aortic second sound Apical ejection click ```

Question 48

Investigations for aortic stenosis

Answer 48

CXR: Normal or prominent left ventricle (due to post-stenotic dilatation of the ascending aorta) ECG: may be left ventricular hypertrophy

Question 49

Management of aortic stenosis

Answer 49

Regular clinical and echocardiographic monitoring Balloon valvulotomy (if high resting pressure gradient or symptomatic on exercise) Most children with significant stenosis will eventually need aortic valve replacement

Question 50

Pulmonary stenosis

Answer 50

Pulmonary valve leaflets are partly fused together and restricting right ventricular outflow

Question 51

Clinical features of pulmonary stenosis

Answer 51

Usually asymptomatic Some may present with cyanosis Ejection systolic murmur best heard at the upper left sternal edge Ejection click best heard at the upper left sternal edge Prominent right ventricular impulse (heave)- only if severe

Question 52

Investigations for pulmonary stenosis

Answer 52

CXR: normal or post-stenotic dilatation of the pulmonary artery ECG: evidence of right ventricular hypertrophy

Question 53

Management of pulmonary stenosis

Answer 53

If the pressure gradient across the pulmonary valve is markedly increased (>64mmHg), intervention is needed Transcatheter balloon dilatation is the treatment of choice

Question 54

Clinical features of adult-type coarctation of the aorta

Answer 54

Gradually gets more severe over many years Asymptomatic Systemic hypertension in the right arm Ejection systolic murmur at the upper sternal edge Collaterals heard with continuous murmur at the back Radio-femoral delay

Question 55

Investigations for adult-type coarctation of the aorta

Answer 55

``` CXR: rib notching (due to the development of large collateral intercostal arteries running under the ribs posteriorly to bypass the obstruction) 3 sign (visible notch in the descending aorta at the site of the coarctation) ``` ECG: left ventricular hypertrophy

Question 56

Management of adult-type coarctation of the aorta

Answer 56

A stent may be inserted when the condition becomes severe

Question 57

Examples of lesions causing an outflow obstruction in a sick infant

Answer 57

Coarctation of the aorta Interruption of the aortic arch Hypoplastic left heart syndrome

Question 58

Clinical features of outflow obstruction in a sick infant

Answer 58

Usually present sick with heart failure and shock in the neonatal period

Question 59

Management of an outflow obstruction in a sick infant

Answer 59

``` Resuscitate (ABC) Prostaglandin infusion (at the earliest opportunity) Referral for early surgical intervention ```

Question 60

Coarctation of the aorta

Answer 60

Due to arterial duct tissue encircling the aorta just at the point of insertion of the duct This causes severe obstruction to left ventricular outflow

Question 61

Clinical features of coarctation of the aorta

Answer 61

Examination may be normal on the first day | Neonates usually present with acute circulatory collapse at 2 days when duct closes

Question 62

Signs of coarctation of the aorta

Answer 62

Sick baby Severe heart failure Absent femoral pulses Severe metabolic acidosis

Question 63

Investigations for coarctation of the aorta

Answer 63

CXR: cardiomegaly from heart failure and shock ECG: normal

Question 64

Management of coarctation of the aorta

Answer 64

Surgical repair

Question 65

Interruption of the aortic arch

Answer 65

RARE No connection between the proximal aorta and distal to the ductus arteriosus Cardiac output is dependent on RIGHT-to-LEFT shunt via the duct VSD is usually present

Question 66

Presentation of interruption of the aortic arch

Answer 66

Shock in the neonatal period Complete correction with closure of the VSD and repair of the aortic arch within first few days of life Associated with other conditions (e.g. Di George syndrome)

Question 67

Hypoplastic left heart syndrome

Answer 67

Characterised by underdevelopment of the entire left side of the heart The mitral valve is small and atretic Left ventricle is small There is usually aortic valve atresia The ascending aorta is also small Usually accompanied by coarctation of the aorta

Question 68

Clinical features of hypoplastic left heart syndrome

Answer 68

May be detected antenatally Children presenting after birth are the sickest of all neonataes presenting with duct-dependetn systemic circulation There is no flow through the left side of the heart, so ductal constriction leads to profound acidosis and rapid cardiovascular collapse Weakness or absence of all peripheral pulses

Question 69

Management of hypoplastic left heart syndrome

Answer 69

Surgery (Norwood procedure)

Question 70

Supraventricular tachycardia

Answer 70

Most common form of childhood arrhythmia Rapid heart rate (250-300) Can cause poor cardiac output and pulmonary oedema

Question 71

Presentation of supraventricular tachycardi

Answer 71

Symptoms of heart failure It can cause hydrops foetalis and intrauterine death

Question 72

Investigations for supraventricular tachycardia

Answer 72

ECG: Narrow complex tachycardia of 250-300bpm It may be possible to discern P waves May be features of myocardial ischaemia (e.g. TWI) In Wolff-Parkinson-White syndrome, a delta wave may be visible

Question 73

Management of SVT

Answer 73

Prompt restoration of sinus rhythm is key to improvement Circulatory and respiratory support - Tissue acidosis should be corrected - Positive pressure ventilation is needed Vagal stimulating procedures (e.g. carotid sinus massage, cold ice pack to face) IV adenosine: Treatment of choice Induces AV block after rapid bolus infusion Terminates the tachycardia Electrical cardioversion with synchronised DC shock if adenosine fails Once sinus rhythm is restored, maintenance therapy will be required (e.g. flecainide or sotalol) 90% of children will have no further attacks after infancy Children who relapse or are at risk usually treated with percutaneous radiofrequency ablation or cryoablation of the accessory pathway

Question 74

Congenital complete heart block

Answer 74

RARE Usually related to the present of anti-Ro or anti-La antibodies in maternal serum The mothers will have latent or active connective tissue disease The antibodies seem to prevent the normal development of the electrical conduction system in the developing heart It can cause foetal hydrops, death in utero and heart failure in the neonatal period Most will remain asymptomatic for many years Some may present with syncope All children with symptoms require insertion of an endocardial pacemaker

Question 75

Long QT syndrome

Answer 75

May be associated with sudden loss of consciousness during exercise, stress or emotion Usually presents in late childhood It can be misdiagnosed as epilepsy Sudden death from ventricular tachycardia can occur Follows autosomal dominant inheritance Associated with erythromycin therapy, electrolyte disturbances and head injury

Question 76

Causes of syncope

Answer 76

Neurally mediated syncope: In response to a range of stressors. Prodrome of dizziness and light-headed feeling and abnormal vision, along with pallor, nausea and sweating. In most people, there is a maladaptive drop in BP Cardiac syncope: may be arrhytmic (e.g. heart block, SVT, ventricular tachycardia). May be structural (e.g. aortic stenosis, HOCM)

Question 77

Features indicative of a cardiac cause in syncope

Answer 77

Symptoms on exercise Family history of sudden unexpected death Palpitation NB: check blood pressure and for signs of cardiac disease (e.g. murmur, femoral pulses, features of Marfan's syndrome) ALL patients presenting with a transient loss of consciousness should be investigated with a 12-lead ECG to check the corrected QT interval)

Question 78

Acute rheumatic fever

Answer 78

Rare in the developed world but is an important cause of heart disease worldwide Acute rheumatic fever is a short-lived, multisystem autoimmune response to a preceding infection with group A b-haemolytic streptococcus It progresses to chronic rheumatic heart disease in up to 80% of cases

Question 79

Clinical features of rheumatic fever

Answer 79

Symptoms manifest about 2-6 weeks after a pharyngeal or skin infection Initial symptoms include polyarthritis, mile fever and malaise Diagnosis is based on the Jones criteria

Question 80

Chronic rheumatic heart disease

Answer 80

MItral stenosis is the most common consequence of scarring and fibrosis of the valve tissue in the heart This could occur at a relatively young age if the patient has repeated attacks of rheumatic fever with carditis The other valves can also get affected

Question 81

Management of rheumatic fever

Answer 81

Acute rheumatic fever is treated with bed rest and anti-inflammatory agents (aspirin is very effective at suppressing the inflammatory response of the joints and heart, and should be given at a high dose) Anti-streptococcal anitbiotics may be used if there is any evidence of a persistent infection (e.g. penicillin V, benzathine benzylpenicillin, amoxicillin) If the fever and inflammation does not resolve rapidly, corticosteroids may be required Symptomatic heart failure is treated with diuretics and ACE inhibitors After the acute episode, recurrence should be prevented The most effective prophylaxis is monthly injections of benzathine penicillin: - Alternative: oral penicillin OD - Most recommend prophylaxis either 10 years after the last episode of acute rheumatic fever or until the age of 21 years - Lifelong prophylaxis is recommended for those with severe valvular disease Surgical treatment with valve repair or replacement may be required

Question 82

Risk factors for infective endocarditis

Answer 82

ALL children with congenital heart disease are at risk of infective endocarditis Highest risk groups= VSD, coarctation of the aorta, PDA or prosthetic materials ``` Should be suspected in any child or adult with: Sustained fever Malaise Raised ESR Unexplained anaemia Haematuria ```

Question 83

Clinical features of infective endocarditis

Answer 83

``` Fever Anaemia and pallor Splinter haemorrhages Clubbing Necrotic skin lesions Changing cardiac signs Splenomegaly Neurological signs from cerebral infarction Retinal infarcts Arthritis/arthralgia Haematuria (microscopic) ```

Question 84

Investigations for infective endocarditis

Answer 84

Multiple blood cultures should be taken before antibiotics are started Detailed echocardiography may be able to confirm the diagnosis by the identification of vegetations NB: vegetations consist of fibrin and platelets and infecting organisms

Question 85

What is the most common causative organism of infective endocarditis?

Answer 85

Streptococcus viridans (a-haemolytic streptococci)

Question 86

Management of infective endocarditis

Answer 86

Involve the MDT: cardiologists, cardiac surgeons, infectious disease specialists, neurologists, neurosurgeons and microbiologists 50% of patients will require surgery Empirical antibiotics should be given (varies depending on local epidemiology, presence of prosthetic valves and possibility of nosocomial infection) - an infectious disease specialist should be consulted Native valves: Penicillin-sensitive Streptococcus viridans= beta-lactam (benzylpenicillin sodium, ampicillin, ceftriaxone, amoxicillin) +/- gentamicin or vancomycin MRSA= (beta-lactam OR vancomycin OR daptomycin OR co-trimoxazole) AND clindamycin Prosthetic valves: Penicillin-sensitive Streptococcus viridans = same as for native valves Methicillin-sensitive Staphylococcus aureus = (nafcillin OR oxacillin OR cefazolin OR vancomycin) AND rifampicin AND gentamicin In other words…high-dose penicillin with an aminoglycoside 6 weeks of therapy If there is infected prosthetic material (e.g. valves), they may require surgical removal

Question 87

Prophylaxis for infective endocarditis

Answer 87

Good dental hygiene Avoidance of body piercings or tattoos Antibiotics prophylaxis is no longer recommended in the UK. However, it may be required in other countries for Dental treatment Surgery which is likely to be associated with bacteraemia

Question 88

Dilated cardiomyopathy causes

Answer 88

Inheritance Secondary to metabolic disease May result from direct viral infection of the myocardium

Question 89

How is dilated cardiomyopathy diagnosed?

Answer 89

Echocardiography

Question 90

Management of myocarditis/cardiomyopathy

Answer 90

Symptomatic with diuretics, ACE inhibitors and carvedilol | Myocarditis usually improves spontaneously

Question 91

Causes of pulmonary hypertension

Answer 91

Pulmonary arterial hypertension: Idiopathic Post-tricuspid shunts (e.g. VSD, VSD, PDA) HIV infection Persistent pulmonary hypertension of the newborn Pulmonary venous hypertension: Left-sided heart disease Pulmonary vein stenosis or compression Pulmonary hypertension with respiratory disease: Chronic obstructive lung disease or BPD in preterm infants Interstitial lung disease Obstructive sleep apnoea or upper airway obstruction Pulmonary thromboembolic disease Pulmonary inflammatory or capillary disease

Question 92

Risks of untreated pulmonary hypertension

Answer 92

Can lead to changes to the pulmonary vascular bed which cannot be corrected other than with heart/lung transplantation

Question 93

Medications to reduce pulmonary hypertension

Answer 93

Inhaled nitric oxide IV magnesium sulphate Oral phosphodiesterase inhibitors