Cardiology

Question 1

ACYANOTIC VSDs:
- Definition
- Pathophysiology
- Risk factors (6)
- Symptoms - mild, moderate, large
- Signs - Inspection, palpation, auscultation
- DD (2)
- Investigations (4)
- Management - small, symptom control, surgical options
- Complications (4)

Answer 1

VSDs:

• Definition: hole in septum separating L+R ventricle. If small can close by 10 years old. Perimembranous in membranous portion or muscular defect in muscular portion.
• Pathophysiology: when born decrease in pulmonary resistance means decreased pressure in pulmonary circulation and increased pressure in systemic circulation so LV-> RV shunt therefore remain acyanotic as blood still goes to lungs. However inc pul blood flow = right sided overload / HF/pulm HTN inc pressure in RV = RV->LV shunt hence cyanotic (eisenmenger syndrome)

VSD can be small. Can be moderate (inc pul circ blood flow leads to left dilatation + pul htn). Can be large (HF + pul HTN)

• Risk factors: maternal diabetes mellitus, maternal rubella, foetal alc syndrome, uncontrolled mat phenylketonuria, fam hist, downs (strongly assoc with cardiac abnorm!!!!)
• Symptoms -
  Small: mild, none, systolic murmur detected on baby check. Can present in late adulthood as intol to exercise, dizzy, chest pain, ankle swelling, peripheral cyanosis, hemoptysis, freq chest infections
  Mod: easily fatigued, excessive sweating by 2-3 months as pul vasc resistance decreases (inc SNS compensates for decreased CO)
  Sev: HF symptoms (SOB, feeding issues, height/weight issues)
• Signs
  Inspection: undernourished, inc work breathing (pul congestion), central cyanosis, clubbing, tachypnoea
  Palp: tachyc, systolic thrill in lower left sternal border
  Ausc: pansystolic blowing murmur lower left sternal border in 3/4 ICS
• DD: mitral regurg, tricuspid regurg, ASD
• Investigations: ecg (LV hypertrophy), bloods (septic screen, U+Es before acei/diuretics, genetic screen), cxr (large vsds may show cardiomeg/pul oed/pleu effusion), transthoracic echo gold standard diagnosis seeing size/location/severity
• Management
  Small/assymp watched
  Symptom control: calorie dense feeds, diuretics, acei, digoxin if CHF
  Surgical options: transvenous catheter closure via femoral vein or open heart surgery via cardiopul bypass, give abx before to reduce infective endocard risk, advise good dental hygeine
• Complications: CHF, growth failure, aortic valve regurg, eisenmengers syndrome, arrythmias

Question 2

ACYANOTIC ASD:
- Definition
- Pathophysiology
- Risk factors (4)
- Symptoms
- Signs
- Investigations (4)
- Management
- Complications (7)

Answer 2

ASD:

• Definition: septum between L + R atrium is not completely formed
• Pathophysiology: during 4th week gestation 2 walls septum premum + secondum grown down from top of heart and fuse with atrioventricular cushions in middle of heart. Small hole in secondum called foramen ovale normally closes at birth when pul pressure decreases at birth. There’s different types of ASD - septum secondum fails to close, septum primum fails to close (however this leads to AVSD), or patent ovale. When born LA pressure > RA so L->R shunt hence acyanotic however then eisenmenger syndrome.
• Risk factors: maternal smoking in trimester 1, maternal diabetes, maternal rubella, maternal drug use, some autos dom inheritance
• Symptoms: most assymp, if large tachypnoea, poor weight gain, recurrent chest infections, feeding issues. If adults: stroke, HF, exercise intol, palpits, fatigue, syncope
• Signs: mid systolic crescendo decrescendo murmur loudest upper left sternal border 2nd ics
• Investigations: ECG (if large then tall P wave/rbbb/rad, if adult maybe AF), transthoracic echo gold standard diagnosis, cardiac MRI/CT, cxr
• Management: if small/assymp <5mm should close within 12 months so watch (if adult echo every 2-3 months). Repair if >1cm via transvenous catheter closure via femoral vein or open heart surgery. Anticoags to reduce risk clots/stroke. If arrhythmia give drugs before repair. Diuretics if HF
• Complications: if untreated arrythmias, pul htn, eisenmenger syndrome, cyanosis, pul oedema, tia/stroke

Question 3

CYANOTIC Tetralogy of Fallot:
- Definition + tetrad
- Pathophysiology (3)
- Risk factors (4)
- Symptoms: mild, moderate, extreme
- Signs (6)
- Differentials (2)
- Investigations (4)
- Management - acute, definitive, follow up, tet spells
- Complications (4)
- Extra facts - tet spells

Answer 3

Tetralogy of Fallot:

• Definition + tetrad: cyanotic CHD consisting of the tetrad VSD, pul stenosis, RV hypertrophy and overriding aorta
• Pathophysiology: VSD and overriding aorta (aorta is dilated and displaced over the intraventricular septum) means when heart contracts more deoxy blood enters aorta from right side heart. Stenosis of pul valve means greater resistance against flow into it encouraging more blood so R->L shunt which bypasses lungs so cyanosis. More blood from right side puts strain on RV hence hypertrophy
• Risk factors: male, fam hist, teratogens (alc, warfarin), rubella, mum >40, alc, diabetes mum, digeorge syndrome
• Symptoms: mostly picked up on antenatal scans
  Mild/pink (mild PS/RVH): assymp but by 1-3 years can become cyanotic
  Mod/severe/cyanotic: first few weeks of life resp distress + cyanosis, recurrent chest infections, fail to thrive, poor feeding, poor weight gain, tet spells
  Extreme: absent pul outflow so dependant on PDA so in first few hours life with resp distress + cyanosis
• Signs:
  General: central cyanosis, clubbing, signs CHF (sweating, pallor, tachyc, bilat bibasal crackles)
  Palp: thrill or heave (RVH)
  Ausc: pansystolic murmur best in left sternal edge
• Differentials: transposition of great arteries, tricuspid atresia
• Investigations: ecg (rvh, rad), cxr (boot shaped heart rvh, pul vasc markings show dec pul blood flow), echo, cardiac mri, cardiac catheter to measure haemodynamics + delinate anatomy
• Management:
  Acute: squatting (inc venous return hence inc systemic resistance), prostaglandin infusion (maintains PDA so blood flows from aorta to pul arts however can cause apnoea/bradycardia/hypotension), b blockers during tet spells/prophylaxis to reduce hr/venous return, morphine if hyperpnoea to reduce resp distress, saline 0.9% bolus if tet spell to inc vol hence inc pul blood flow
  Surgical: transcatheter stent insertion or modified blalock taussig shunt inc pul blood flow to buy time
  Definitive: surgical repair via open heart cardiopul bypass if 3 months - 4 years to correct rv outflow obstruction + close vsd
  Lifelong follow up: ecg, echo, cardiopul exercise testing
• Complications: polycythaemia, cerebral abscess, stroke, infective endocarditis, CHF, pul regurg
• Extra facts: tet spells:
  Intermittent symptomatic periods where r->l shunt becomes worsened causing cyanosis/sob/seizures. This is when pul vasc resis inc or systemic resis decreases (e.g. when lots carbon dioxide vasodilator made when walking, exercise, crying)

Question 4

CYANOTIC Transposition of great arteries:
- Definition + pathophysiology
- Risk factors (5)
- Symptoms (3)
- Signs (4)
- Investigations (4)
- Management - emergency + definitive
- Complications (4)
- Predictors of poor development outcome
- Extra facts

Answer 4

Transposition of great arteries:

• Definition + pathophysiology: ventriculoarterial discordance in which aorta arises from the morphologic RV and pul art arises from LV due to failure of spiralling of aortopulmon septum. This establishes parrellel circuit so oxy blood only circs in pulmon circ and deoxy in systemic circ resulting in deep hypoxaemia resulting in lactic acidosis. Is ok during preg as exchange happens at placenta
  Can be dextrotransposition (aorta is ant/right of pul art), or levo (ant / left)
• Risk factors: >40, maternal diab, rubella, poor nutrition mum, alc
• Symptoms: antenatal scan, cyanosis in first 24 hours, CHF signs within 6 weeks (tachyp/cardia, diaphoresis, no weight gain)
• Signs: RV heave, loud s2, central cyanosis
• Investigations: fetal uss during antenatal period, cbg (met acidosis bc inc lactate from anaerobic with dec pao2), echo diagnosis, cxr egg on string (narrowed upper mediastinum, cardiomeg, inc pul vasc markings)
• Management:
  Emergency: prostaglandin e1 infusion (alprostadil) to keep ductus art patent, correct met acid, emergency atrial balloon septostomy to allow mixing
  Definitive: surgical arterial switch at 4 weeks
• Complications: neopul stenosis, neoaortic regurg, neoaortic root dilatation, coron art disease
• Predictors of poor development outcome: low gestational age, high pre op lactate
• Extra facts: assoc with VSD, coarctation, PS

Question 5

ACYANOTIC Congenital aortic valve stenosis:
- Definition
- Symptoms - mild, mod, severe
- Signs (4)
- Investigations (2)
- Management (3)
- Complications (5)

Answer 5

Congenital aortic valve stenosis:

• Definition: bivalvular leaflets instead of semilunar valves which can narrow valve restricts blood flow from LV-> aorta
• Symptoms:
  Mild: assymp
  Mod: fatigue, sob, dizzy, worse on exertion
  Severe: HF months after birth
• Signs: crescendo decrescendo ejection systolic murmur 2nd ics right sternal border radiating to carotids, slow rising pulse, narrow pulse pressure, palpable thrill during systole
• Investigations: ecg, echo gold standard
• Management: percut balloon aortic valvuloplasty, surgical aortic valvotomy, valve replacement
• Complications: LV outflow obstruction hence LV hypertrophy, HF, vent arrythmias, endocarditis, sudden death

Question 6

ACYANOTIC Congenital pulmonary valve stenosis:
- Definition
- Symptoms (5)
- Signs (5)
- Management

Answer 6

ACYANOTIC Congenital pulmonary valve stenosis:

• Definition: abnormal dev of valve makes it narrow leading to RVH. Assoc with TOF, william syndrome, noonan syndrome, congen rubella
• Symptoms: assymp, fatigue, sob, exertion, dizzy
• Signs: ejection systolic murmur 2nd ics left sternal border, palp thrill, rv heave (if rvh), raised jvp, a waves
• Management: mild then watch, if signif balloon valvuloplasty via venous catheter via femoral vein into ivc into pul valve or open heart surgery

Question 7

Eisenmenger sydrome:
- Pathophysiology
- Signs
- Management

Question 8

Coarctation of aorta:

• Definition
• Associated conditions
• Symptoms
• Signs
• Investigations
• Management

Question 9

PDA:

• Definition
• Risk factors
• Symptoms
• Signs
• Investigations
• Management

Question 10

Blue baby

• Pulmonary causes (4)
• Cardiac causes (1)
• Airway obstruction causes (4)
• Neuromuscular causes (2)
• Investigations
• Management