Cardio Flashcards

Question

What are the Ix for ?PDA?

Answer 1

* **CXR** * Usually normal * If large, same features as large VSD * **ECG** * Usually normal * If large, same features as large VSD * **Echo and Doppler USS** * Diagnostic

Answer 2

_In preterm infants:_ **COX inhibitors** (IV indometacin or ibuprofen) to help closure _In all infants:_ * **Pharmacological treatment of HF** until correction (if necessary) e.g. **Diuretics, captopril, digoxin** * **Closure with coil or occlusion device** introduced via cardiac catheter * At 1yo * Recommended regardless of shunt size to reduce lifelong risk of infective endocarditis and pulmonary HTN * Occasionally, surgical ligation is required (if large ducts or symptomatic infants too small for device closure)

Answer 3

_In preterm infants:_ **COX inhibitors** (**IV indometacin** or **ibuprofen**) to help closure _In all infants:_ * **Pharmacological treatment of HF** until correction (if necessary) e.g. **Diuretics, captopril, digoxin** * **Closure with coil or occlusion device** introduced via cardiac catheter * At 1yo * Recommended regardless of shunt size to reduce lifelong risk of infective endocarditis and pulmonary HTN * Occasionally, **surgical ligation** is required (if large ducts or symptomatic infants too small for device closure)

Answer 4

* Faltering growth (if duct starts to dilate) * Infective endocarditis, endarteritis * Pulmonary HTN * Congestive HF * In preterm infants, associated with RDS, NEC and pulmonary haemorrhage

Answer 5

Prognosis is excellent if closed

Answer 6

An opening in the atrial septum, excluding a parent foramen ovale

Answer 7

There are 2 main types → presentation is similar but anatomy is different: * **Secundum ASD (80%)** * Defect in the centre of the of the atrial septum, in the region of the foramen ovale * **Partial atrioventricular septal defect (AVSD or primum ASD)** * Defect of the atrioventricular septum, characterised by: * An interatrial communication between the bottom end of the atrial septum and the atrioventricular valves (primum ASD) * Abnormal atrioventricular valves, with a L AV valve which has 3 leaflets and tends to leak (regurgitant valve)

Answer 8

In infancy, the R heart is relatively thick and non-compliant and has higher pressure than the L side, so there is minimal L to R shunting * As pulmonary vascular resistance decreases, the R heart becomes more compliant → L to R shunt increases * With age, the L to R shunt is exacerbated by increasing stiffness of the L ventricle (due to ageing and systemic HTN) à R atrium, R ventricle and pulmonary arteries enlarge due to the increased volume load

Answer 9

* **maternal alcohol consumption**, * **FHx** (some causative mutations have been found) * **Most are spontaneous with no FHx of congenital heart defects**

Answer 10

* Usually **asymptomatic** * **Recurrent chest infections/wheeze** * **Failure to thrive (10%)** * On examination: * **Ejection systolic murmur** at upper L sternal edge (due to increased flow across pulmonary valve) * **Fixed widely split 2^nd HS**; doesn’t become single with expiration (due to RV SV being equal in inspiration and expiration) * **Pansystolic murmur** at apex in partial AVSD (due to AV valve regurgitation)

Answer 11

* **CXR** * Cardiomegaly * Enlarged pulmonary arteries; increased pulmonary vascular markings * **ECG** * Secundum ASD: * Partial RBBB is common (but may occur in normal children) * R axis deviation due to RV enlargement * Partial AVSD: * Superior (left) axis deviation (mainly negative in AVF) * Occurs because there is a defect of the middle part of the heart where the AVN is à displaced AVN conducts to the ventricles superiorly, giving the abnormal axis * **Echo and Doppler USS** * Diagnostic

Answer 12

**Observation:** * For all patients à defect may close or shrink * If small, closure is not required * Treatment is done if significant ASD (large enough to cause RV dilatation), around 3-5yo **Secundum ASD:** * Cardiac catheterisation with insertion of an occlusive device **Partial AVSD:** * Surgical correction **Prophylactic antibiotics (_amoxicillin_) to prevent endocarditis** * For 6 months after catheterisation/surgical closure * Also given before any procedure that could cause bacteraemia

Answer 13

Complications: * **Device erosion** (after repair) * **Infective endocarditis** * **Eisenmenger’s syndrome** * **Arrhythmias** (from 4^th decade) * **Congestive HF** (in \<10% infants; or 5^th decade)

Answer 14

* **Eisenmenger syndrome (ES)** refers to the combination pulmonary HTN and abnormal blood flow through the heart. * ES most often occurs in those with congenital heart defects that are not repaired in childhood. * The most common type of heart defect associated with Eisenmenger syndrome is a ventricular septal defect, but other types of heart defects can lead to Eisenmenger syndrome. * The symptoms include cyanosis, clubbing and SOB. T * The symptoms of ES typically get worse over time. * Tx for severe ES: heart and lung transplant

Answer 15

* Excellent prognosis for uncomplicated ASDs closed surgically * Poor prognosis for later complications, e.g. Eisenmenger’s syndrome

Answer 16

Defects in the inter-ventricular septum that allow shunting of blood between the L and R ventricles

Answer 17

There is a defect anywhere in the ventricular septum → * **perimembranous** (adjacent to the tricuspid valve – 70%) * or **muscular** (completely surrounded by muscle) Categorised according to size: * Small (80%): smaller than the aortic valve in diameter (up to 3mm) * Leads to minimal L to R shunting, and doesn’t cause increased pulmonary vascular resistance * Large: same size or bigger than aortic valve * Leads to L to R shunting à pulmonary HTN * Gradually, the pulmonary vascular resistance rises à decreased shunting (and ultimately shunt reversal) à R HF (Eisenmenger’s syndrome)

Answer 18

Categorised according to size: * **Small (80%):** smaller than the aortic valve in diameter (up to 3mm) * Leads to minimal L to R shunting, and doesn’t cause increased pulmonary vascular resistance * **Large**: same size or bigger than aortic valve * Leads to L to R shunting → pulmonary HTN * Gradually, the pulmonary vascular resistance rises à decreased shunting (and ultimately shunt reversal) à R HF (Eisenmenger’s syndrome)

Answer 19

* FHx, * some genetic abnormalities (e.g. Down’s), * maternal alcohol consumption during pregnancy

Answer 20

Common → 30% of congenital heart disease

Answer 21

Small VSD: * **Asymptomatic** * On examination: * **Loud pansystolic murmur** at lower L sternal edge * Loud murmur implies smaller defect due to increased resistance to flow

Answer 22

Large VSD: * **HF after 1wk old → breathlessness, growth faltering** * **Recurrent chest infections** * On examination: * Tachypnoea, tachycardia, hepatomegaly (from HF) * Active precordium * **Soft murmur or no murmur** (implies large defect) * **Loud pulmonary component of 2^nd HS (**sign of pulmonary HTN)

Answer 23

* **CXR** * Small VSD: normal * Large VSD: * Cardiomegaly * Enlarged pulmonary arteries, increased pulmonary vascular markings * Pulmonary oedema * **ECG** * Small VSD: normal * Large VSD: * Biventricular hypertrophy by 2mo * Upright T wave in V1 suggests pulmonary HTN * **Echo and Doppler USS** * Diagnostic * Shows the precise anatomy of the defect and its haemodynamic effects (using Doppler) * Small VSD: no pulmonary HTN * Large VSD: pulmonary HTN (due to high flow)

Answer 24

Small VSD: * Don’t need correction (they close spontaneously) * Follow-up: * Disappearance of murmur * Normal ECG and echo * Prevention of bacterial endocarditis by maintaining good dental hygiene

Answer 25

Large VSD: * Additional calorie input * Pharmacological treatment of HF prior to surgery: * Diuretics (furosemide) * Captopril * Digoxin * Surgery: * At 3-6mo * Prophylactic antibiotics (amoxicillin) to prevent endocarditis * For 6 months following closure, and consider in any procedures that can cause bacteraemia

Answer 26

Complications: * Small VSD: * Infective endocarditis * Large VSD: * Infective endocarditis * Eisenmenger’s syndrome * Aortic regurgitation (if defect is near aortic valve) * Heart block (if surgery affects conducting tissue) Small VSD has excellent prognosis à 50% close spontaneously; asymptomatic even if they persist Lage VSD has excellent prognosis if closed early, but lead to Eisenmenger’s syndrome if not repaired

Answer 27

A congenital heart defect where there is a large hole between the atria and ventricles in the middle of the heart This is a common mixing condition

Answer 28

AVSD can be **partial** or **complete** * **Partial is a primum ASD** (discussed in ASD) * **Complete AVSD:** * Defect in the middle of the heart with a single five-leaflet (common) valve between the atria and ventricles à this large hole in the centre of the heart means blood can flow between all 4 chambers * It stretches across the entire AV junction * The valves tend to leak (may not be formed correctly) * As there is a large defect, there is pulmonary hypertension

Answer 29

* Down’s, * FHx, * maternal DM/alcohol etc.

Answer 30

Most common in children with Down’s syndrome (40% of children with Down’s) 4-5% of all congenital heart disease

Answer 31

* Usually presents on antenatal USS screening, or on routine echo of patient with Down’s * **Cyanosis at birth or HF at 2-3wks of life** * On examination: * **No murmur** (because large defect)

Answer 32

* **CXR** * Cardiomegaly * Increased pulmonary vascular markings * **ECG** * Superior (left deviation) axis * **Echo and Doppler USS**

Answer 33

**Medical management of HF:** * Furosemide * Captopril * Digoxin **Surgery**: * Repair at 3-6mo * Common valve is separated into 2 valves (pulmonary and mitral) Life-long follow-up for complications

Answer 34

Complications: * Pulmonary HTN * Eisenmenger syndrome * Congestive HF * Progressive AV valve regurgitation Prognosis is generally good with surgical correction

Answer 35

Narrowing of the aorta, most commonly at the site of insertion of the ductus arteriosus (just distal to the L subclavian artery)

Answer 36

May be **adult-type (postductal**) or **infantile (juxtaductal)** **Adult-type (postductal):** * A cause of outflow obstruction in the well child * Not duct-dependent * Gradually becomes more severe over many years (can be clinically silent for a long time) * Collateral blood vessels often enlarge to provide a route for blood to bypass the narrowed aorta * Associated with HTN **Infantile (juxtaductal):** * A cause of outflow obstruction in the sick infant * Due to arterial duct tissue encircling the aorta just at the point of insertion of the duct * When the duct closes, the aorta also constricts, causing severe obstruction of left ventricular outflow * Leads to low-output HF and shock when duct closes May also be **pre-ductal** → **seen in 5% infants with Turner’s syndrome** * Associated with hypoplasia of the aortic arch

Answer 37

* FHx, * male, * Turner’s syndrome, * DiGeorge syndrome

Answer 38

Adult-type: * **Asymptomatic** * On examination: * **HTN in arms** (presenting at young age, resistant to treatment) * Higher BP in arms than legs (because BP increases above the constriction (subclavian arteries supplying the arms branch before constriction but there is less blood flow to aorta after the constriction, which supplies the legs) * **Radio-femoral delay** * Due to blood bypassing the obstruction via collaterals in chest wall → pulse in legs is delayed * **Ejection systolic murmur** at upper sternal edge * **Continuous murmur** between scapulae

Answer 39

Infantile: * Examination on 1^st day of life is usually normal * Acute circulatory collapse at 2 days of age (when duct closes) à severe HF * Absent femoral pulses * Severe metabolic acidosis

Answer 40

* **Adult-type:** * Rib-notching due to development of large collateral intercostal arteries running under the ribs posteriorly to bypass the obstruction * In teenagers and adults * ‘3 sign’ – visible notch in the descending aorta at the site of coarctation * Infantile: * Cardiomegaly from HF and shock * **ECG** * Adult-type: * L ventricular hypertrophy à deep S in V2, tall R in V6, upright T wave * Infantile: * Normal * **Echo and Doppler USS** * For diagnosis

Answer 41

Adult-type: * When severe → percutaneous angioplasty and stent insertion * Sometimes surgical repair is needed Infantile: * ABCDE * Prostaglandin infusion to maintain ductal patency * Referral to cardiac centre for early surgical intervention * Surgical repair performed ASAP after diagnosis * Surgical repair is preferred in neonates (as opposed to stent) because fewer repeat interventions

Answer 42

Complications: * Systemic HTN * Coronary artery disease (if persistent HTN due to delayed repair) * Recoarctation after repair * Aortic aneurysm (due to surgery/stent) Good prognosis * May need long-term HTN treatment (even after repair) * Careful follow-up with cardiologist is needed (as higher incidence of CHD and may have recoarctation)

Answer 43

A congenital heart defect where the aorta is connected to the R ventricle and pulmonary artery is connected to the L ventricle

Answer 44

The aorta and pulmonary artery are connected the wrong way around (discordant ventriculo-arterial connection) * Therefore, the deoxygenated blood is returned to the body and the oxygenated blood is returned to the lungs à there are 2 parallel circulations * Unless there is mixing of blood between them, this is incompatible with life * There are various naturally-occurring associated anomalies, e.g. VSD, ASD, PDA, and therapeutic interventions which can achieve this mixing in the short-term

Answer 45

* FHx * maternal age \>40yo * maternal DM/rubella/alcohol consumption

Answer 46

5% of all congenital heart disease à 20/100,000 live births M\>F

Answer 47

* **Cyanosis** * Usually on day 2 (when ductus arteriosus closes) * May be profound and life-threatening * Less severe and presentation delayed if there is mixing from another abnormality, e.g. ASD * On examination: * 2^nd HS loud and single * R ventricular heave * Usually no murmur

Answer 48

* **CXR** * Narrow upper mediastinum with ‘egg on side’ appearance of the cardiac shadow * Due to anteroposterior relationship of great vessels, narrow vesicular pedicle and hypertrophied R ventricle * Increased pulmonary vascular markings * **ECG** * Usually normal * **Echo and Doppler USS** * For diagnosis à shows the abnormal connections and associated abnormalities

Answer 49

**_Management of the cyanosed neonate:_** * **ABCDE approach**: stabilise the airway, breathing and circulation; artificial ventilation if necessary * Start **prostaglandin infusion** (5ng/kg/min) * **Balloon atrial septostomy** * Emergency; may be life-saving à done in 20% * Catheter with inflatable balloon at its tip is passed through the umbilical or femoral vein through the R atrium and foramen ovale → inflated in the L atrium and pulled back through the atrial septum → tears the atrial septum and makes the flap of the foramen ovale incompetent → mixing of blood **_Surgery_**: * For all patients, **usually in first few days of life** * **Arterial switch procedure →** aorta and pulmonary artery are swapped; coronary arteries are also transferred to the new aorta

Answer 50

Complications: * Neopulmonary stenosis (stenosis of the new pulmonary valve) * Neoaortic regurgitation * Neoaortic root dilatation * Coronary artery disease * Sudden cardiac death (due to arrhythmias) * Neurodevelopmental abnormalities

Answer 51

Survival is \>90% at 20yo

Answer 52

A congenital heart malformation: 1. **VSD with over-riding aorta**, and 2. **RV outflow tract obstruction**, and 3. resulting **RV hypertrophy**

Answer 53

There are **4 cardinal anatomical feature**s: * **Large VSD** * **Overriding of the aorta** with respect to the ventricular system (i.e. aorta is in the middle) * **Subpulmonary stenosis** causing R ventricular outflow tract obstruction * **R ventricular hypertrophy** (as a result of outflow obstruction)

Answer 54

Cyanosis is due to R to L shunting of deoxygenated blood at the VSD (as the blood from the RV can’t be pumped through the pulmonary artery) * If there is a large pulmonary obstruction, blood is shunted from the R to L ventricle through the VSD à into the systemic circulation without being oxygenated * If a small obstruction, there is less cyanosis because more blood is oxygenated and less is shunted * Until the ductus arteriosus closes, there is shunting from the aorta through the pulmonary circulation à no cyanosis Hypercyanotic spells are episodes of severe cyanosis associated with hyperpnoea * They are due to an increase in R ventricular outflow tract obstruction, causing decreased pulmonary blood flow and increased R to L shunting across the VSD Aetiology is unknown à genetics and environmental

Answer 55

* chromosomal abnormalities (trisomy 21, 18, 13, DiGeorge), * FHx, * maternal DM, * teratogens (retinoic acid)

Answer 56

Most common cause of cyanotic congenital heart disease Accounts for 4-9% of congenital heart defects

Answer 57

* Degree of cyanosis varies (may not be obvious) * Classical presentation is in late infancy with **severe cyanosis**, **hypercyanotic spells** and **squatting on exercise** * Hyper-cyanotic spells are characterised by a rapid increase in cyanosis, with irritability or inconsolable crying, and breathlessness or pallor; there is a very short murmur during a spell * Rare in developed countries (due to earlier diagnosis; often detected antenatally) * On examination: * **Loud ejection systolic murmur** at L sternal edge from day 1 of life * With increasing RV outflow tract obstruction, the murmur shortens and cyanosis increases * **Clubbing** (in older children) * **Tachypnoea**

Answer 58

* **Hyperoxia (nitrogen washout) test** * To help determine presence of heart disease in a cyanosed neonate * Infant placed in 100% O2 (headbox or ventilator) for 10mins * If the R radial arterial PaO2 from a blood gas remains low (\<15kPa) after this time à diagnosis of cyanotic congenital heart disease can be made if lung disease and persistent pulmonary hypertension of the newborn have been excluded * If PaO2 \>20kPa, it is not cyanotic heart disease * **CXR** * Relatively small heart * Uptilted apex (boot shaped) due to R ventricular hypertrophy * More prominent in older children * Pulmonary artery ‘bay’ * Concavity on L heart border where the convex-shaped main pulmonary artery and R ventricular outflow tract would usually be * Decreased pulmonary vascular markings (as reduced pulmonary blood flow) * **ECG** * Normal at birth * R ventricular hypertrophy when older * **Echo and Doppler USS** * For definitive diagnosis → shows carinal features

Answer 59

Management of the cyanosed neonate: * **ABCDE approach**: stabilise the airway, breathing and circulation; artificial ventilation if necessary * Start **prostaglandin infusion** (5ng/kg/min) * Most infants with cyanotic heart disease presenting in the first few days of life are duct-dependent (i.e. there is reduced mixing between the oxygenated blood returning from lungs and deoxygenated blood from the body) à maintenance of ductal patency is key to survival * **Observe for SEs:** apnoea, jitteriness, seizures, flushing, vasodilation, hypotension * Consider **balloon atrial septostomy**

Answer 60

Management of hypercyanotic spells: * They are **self-limiting** and **followed by sleep** * **If prolonged (\>15mins) treat promptly with:** * **Sedation and pain relief** (morphine) * **IV fluids** * **IV propranolol** (for peripheral vasoconstriction and to relieve the subpulmonary obstruction) * **Bicarbonate** to correct acidosis * **Muscle paralysis** and **artificial ventilation** to reduce metabolic oxygen demand

Answer 61

* Definitive surgery for all patients at around 6mo * VSD is closed and R ventricular outflow tract obstruction is relieved * Neonates who are very cyanosed need a shunt to increase pulmonary blood flow → surgical placement of a tube between the subclavian artery and pulmonary artery (modified Blalock-Taussig shunt) * Antibiotics to reduce risk of infective endocarditis (e.g. cefalexin, amoxicillin) * Before surgery and before surgical procedures (incl dental) if unrepaired TOF

Answer 62

Complications: * Hypercyanotic spells can lead to MI, CVAs and death * Progressive pulmonary regurgitation and RV failure (in adulthood) * Arrhythmias later in life * Sudden cardiac death (from VT/VF) 93% survival at 5yo; 80% survival at 35yo

Answer 63

**Medical management of HF:** * Furosemide * Captopril * Digoxin **Surgery**: * Repair at 3-6mo * Common valve is separated into 2 valves (pulmonary and mitral) Life-long follow-up for complications

Answer 64

**Medical management of HF:** * Furosemide * Captopril * Digoxin **Surgery**: * Repair at 3-6mo * Common valve is separated into 2 valves (pulmonary and mitral) Life-long follow-up for complications

Answer 65

**Medical management of HF:** * Furosemide * Captopril * Digoxin **Surgery**: * Repair at 3-6mo * Common valve is separated into 2 valves (pulmonary and mitral) Life-long follow-up for complications

Answer 66

Complications: * Systemic HTN * Coronary artery disease (if persistent HTN due to delayed repair) * Recoarctation after repair * Aortic aneurysm (due to surgery/stent) Good prognosis * May need long-term HTN treatment (even after repair) * Careful follow-up with cardiologist is needed (as higher incidence of CHD and may have recoarctation)

Answer 67

Obstruction of the blood flow from the R ventricle into the pulmonary artery A cause of outflow obstruction in the well child

Answer 68

The pulmonary valve leaflets are partly fused together, causing a restrictive exit from the R ventricle * The increased pressure in the R ventricle may lead to hypertrophy * Mild-moderate obstruction is usually well-tolerated à asymptomatic * If severe-critical → symptoms (because not enough blood is getting oxygenated) NB a small amount is acquired, due to carcinoid syndrome, myocardial tumours and external compression

Answer 69

* Noonan syndrome, * other syndromes (e.g. LEOPARD, William’s), * congenital rubella syndrome

Answer 70

8% of all congenital heart disease; up to 30% when in association with other lesions Commonly associated with other forms of congenital heart disease

Answer 71

* Usually **asymptomatic** * Occasionally **neonates present with critical pulmonary stenosis** (very severe narrowing) → duct-dependent pulmonary circulation → cyanosis **in the first few days of life** * On examination: * **Ejection systolic murmur** at upper L sternal edge * **Ejection click** at upper left sternal edge * The most severe the stenosis, the earlier in systole the click occurs (if very severe it my merge with the 1^st HS and become inaudible) * **Thrill** * **R ventricular heave** (if severe)

Answer 72

* **CXR** * May be normal * May show post-stenotic dilation of the pulmonary artery * **ECG** * R ventricular hypertrophy (upright T wave in V1) * R axis deviation * **Echo and Doppler USS**

Answer 73

**_Management of the cyanosed neonate (in critical disease):_** * **ABCDE** * **Prostaglandins** (to maintain patency of ductus arteriosus) **_Mx of all patients_** * **Transcatheter balloon dilatation** (percutaneous balloon pulmonary vavuloplasty) * Intervention is needed when the pressure gradient across the pulmonary valve becomes markedly increased (\>64mmHg), or in critical disease in neonates * In mild disease no treatment is necessary * **Antibiotics for endocarditis prophylaxis** (for 6 months after repair)

Answer 74

* Complications of valvuloplasty: vascular lacerations, tearing of pulmonary valve, arrhythmias, pulmonary valve insufficiency, cardiac perforation * R HF * Sudden death (due to limited pulmonary blood flow)

Answer 75

Excellent prognosis with treatment * 90% success rate for treatment in infants with critical pulmonary stenosis; 5% complications

Answer 76

Impaired ability of the ventricles to fill with and/or eject blood

Answer 77

**_Causes of HF vary with age:_** * **Neonates**: obstructed (duct-dependent) systemic circulation à L heart obstruction means flow of blood is predominantly by R-to-L flow by ductus arteriosus à closure of the duct leads rapidly to severe acidosis, collapse and death unless ductal patency is restored * Caused by hypoplastic left heart syndrome, critical aortic valve stenosis, severe coarctation of aorta, interruption of the aortic arch * **Infants**: high pulmonary blood flow due to L to R shunt à as pulmonary vascular resistance falls over a few weeks of life there is increased shunting à pulmonary oedema and breathlessness à these symptoms increase until 3mo and then may improve as pulmonary resistance rises in response * Caused by VSD, ASD, AVSD, large PDA * **Older children/adolescents** (R or L HF) * Eisenmenger syndrome (R HF only) * In children with a L to R shunt or common mixing à increased pulmonary resistance à irreversibly raised pulmonary vascular resistance due to chronically raised pulmonary arterial pressure and flow à shunt occurs from R to L (reversal) à de-oxygenated blood flows to systemic arterial circulation à cyanosis * Rheumatic heart disease * Cardiomyopathy **_Causes that are not related to congenital heart disease include:_** * Cardiomyopathy, myocarditis (usually due to viral infection), arrhythmias * Non-cardiac causes: sepsis, CKD, respiratory disorders (obstructive sleep apnoea, CF), SLE

Answer 78

* **Breathlessness** (esp on feeding or exertion) * **Poor feeding/anorexia** * **Poor weight gain/growth faltering** * **Recurrent chest infections** * On examination: * Tachypnoea, tachycardia * **Heart murmur**, **gallop rhythm**, **enlarged heart** * **Signs of R HF**: hepatomegaly, ankle/sacral oedema, ascites * JVP is not reliable in young children * **Uncompensated CHF:** cool peripheries, prolonged capillary refill tie, decreased urine output

Answer 79

* **Basic obs** * **Bloods**: * BNP, * FBC (anaemia may contribute), * U&Es (renal impairment may contribute, monitor baseline), * LFTs (may be raised in R HF), * ABG * **CXR** * Cardiomegaly * Increased pulmonary vascular markings * Pulmonary oedema * **ECG** * Evidence of myocarditis/cardiac ischaemia or ventricular strain * **Echo** * Diagnostic for congenital heart disease; assess cardiac function * **Cardiac catheterisation** * Measures intracardiac pressures and shunts

Answer 80

**ABCDE approach** * Sit patient up * **Oxygen**, consider **CPAP** * **Fluid restrict** * If volume depleted, give fluids slowly (boluses of 5-ml/kg) * **Prostaglandin** infusion for infants with unexplained shock **Medical management:** * **Furosemide** (reduce preload) * **ACEi** (reduce afterload) * **Beta blockers** * **Inotropes** (e.g. dobutamine) in decompensated HF (improve CO) * **Digoxin** **Management of the underlying condition**

Answer 81

**For chronic HF:** * Correct anything contributing (anaemia, obesity) * Nutritional support (may need intermittent or continuous NG feeds) * Promote exercise

Answer 82

Eisenmenger syndrome: * Treatment is aimed at **prevention** → early intervention for high pulmonary blood flow * Only treatment is **heart-lung transplant** * **Medication** can palliate the symptoms until transplantation * **CCBs** (nifedipine, amlodipine) * **Phosphodiesterase type 5 (PDE5) inhibitors** (sildenafil) * **Endothelin receptor antagonists** (bosentan) * **Prostacyclin analogues** (iloprost)

Answer 83

Depends on the cause Eisenmenger syndrome: * If untreated, death due to R HF (usually in 4^th/5^th decade)

Answer 84

Acute childhood febrile illness with systemic small and medium vessel vasculitis

Answer 85

Aetiology is **unknown** but it is thought that an **infectious agent may induce disease in genetically susceptible people** * 3 hypotheses: * Infectious hypothesis: winter-spring seasonality * Genetic susceptibility hypothesis: more common in Japanese people * Superantigen hypothesis * Marked cytokine cascade stimulation and endothelial cell activation leads to systemic vasculitis

Answer 86

* Asian ancestry (esp Japanese), * age 6 months – 4yrs, * male

Answer 87

* **Fever \>5d, usually \>39⁰C** * Suspect Kawasaki in **prolonged** **fever**; unresponsive to antibiotics * **_4 of the other 5 features of:_** * **Conjunctivitis** * **Mucous membrane changes** (pharyngeal injection, red/dry/cracked lips, strawberry tongue) * **Cervical lymphadenopathy** * The above symptoms are present at the start of the illness for around 2wks * **Rash (polymorphous)** * Usually a diffuse, maculopapular, erythematous rash * Begins after a few days; present until middle of 3^rd week * **Extremities**: * Red and oedematous palms and soles (first few days – 2wks) * Peeling of fingers and toes (desquamation) (after around 2wks)

Answer 88

* **Fever \>5d, usually \>39⁰C** * Suspect Kawasaki in **prolonged** **fever**; unresponsive to antibiotics * **_4 of the other 5 features of:_** * **Conjunctivitis** * **Mucous membrane changes** (pharyngeal injection, red/dry/cracked lips, strawberry tongue) * **Cervical lymphadenopathy** * The above symptoms are present at the start of the illness for around 2wks * **Rash (polymorphous)** * Usually a diffuse, maculopapular, erythematous rash * Begins after a few days; present until middle of 3^rd week * **Extremities**: * Red and oedematous palms and soles (first few days – 2wks) * Peeling of fingers and toes (desquamation) (after around 2wks)

Answer 89

* **_Clinical diagnosis_** * **Bloods**: * **FBC**: anaemia, high WCC with L shift, platelets rise in 2^nd week of illness * **ESR/CRP** (high) * **Echo** * For coronary artery dilatation or aneurysm

Answer 90

Aims to prevent coronary artery disease and relieve symptoms, by controlling the inflammatory process 1. **IVIG** * Best results when treatment is started within 10d * Also indicated in patients who present \>10d with RFs for complications (e.g. fever, raised ESR/CRP) * Single infusion; give 2^nd dose if fever persists after 36-48hrs * If no response to 2^nd dose à consider corticosteroids, infliximab or cyclosporin **2. Aspirin** * To lower risk of thrombosis * Given at a high anti-inflammatory dose (80-100mg/kg/d) until fever subsides and inflammatory markers return to normal; continued at a low antiplatelet dose (3-5mg/kg/d) until echo at 6wks * If aneurysms on echo à continue aspirin until they regress; start warfarin and thromboprophylaxis (with cardio input) **If child presents after 10d without persistent fever and RFs for coronary aneurysms (normal CRP/ESR, normal echo) → just give aspirin (no IVIG)**

Answer 91

Complications: * **Myocarditis** * Common * Rarely causes clinical problems; responds promptly to IVIG * **Pericarditis with small pericardial effusions** * Occurs in 25% before IVIG; resolves completely with IVIG * **Coronary artery aneurysm** * Due to development of an acute coronary vasculitis * Develop in 20-25% untreated patients; 3% if treated with IVIG (prompt treatment reduces incidence) * Subsequent narrowing of the vessels from scar formation can lead to myocardial ischaemia and sudden death

Answer 92

* **It is self-limiting** * Unless there is coronary artery aneurysm → **increased risk ischaemia or thrombosis** * **Overall mortality with treatment 0.5%**

Cardio Flashcards

(116 cards)