Congenital Heart Disease Flashcards by Can I get Uhhhh?

Who gets atrioventricular septal defects?

40% of Down’s children

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What is considered ‘complete AVSD’?

A single leaky 5 leaflet valve which joins the atrium to the ventricle

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What are the clinical features of AVSD?

Pulmonary HTN

Cyanosis at birth/ HF in 2-3 wks

No murmur

Superior axis on ECG

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What investigations do you do for AVSD?

Antenatal US- apparent

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What is the management for AVSD?

Medical treatment for HF (Furosemide/ ACEi)

Surgical repair at 3-6 months

Lifelong follow up –catching AV regurge or LVOT obstruction

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What is the most common form of complex congenital heart disease?

Tricuspid atresia

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What are the clinical features of tricuspid atresia?

Absence of tricuspid valve

Mixing of systemic and pulmonary return in LA

Cyanosis (duct dependent)

May be well at birth

ESM at L sternal edge

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What investigations would you do for tricuspid atresia?

CXR: cardiomegaly, prominent RA border (Ebstein’s abnormality)

ECG: LAD

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What is the management of tricuspid atresia?

Maintain pulmonary blood supply via BT shunt insertion or Pulmonary artery banding
Glenn operation (SVC to pulmonary artery)
Fontan operation (IVC to pulmonary artery)

[Complete correction not possible]

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Which CHD is the most common?

VSD (80%)

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What is the size of a VSD based on?

Larger or smaller than an aortic valve

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What are the clinical features of a small VSD?

Asymptomatic

Loud Pan systolic murmur at LSE

Quiet pulmonary second sound

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What are the features of a large VSD?

HF/ SOB
poor growth
chest infections
tachypnoea
active precordium
soft pansystolic murmur
apical mid diastolic murmur loud pulmonary second sound

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What investigations would you do for VSD?

CXR
ECG
Echo

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What findings would you get if a patient has a VSD?

Small: CXR/ECG: normal
Echo: anatomical defect

Large:
CXR: cardiomegaly, large pulm arteries, increased vassc markings, pulmonary oedema

ECG: Biventricular hypertrophy, demonstrates the anatomy of the defect and Pulm HTN

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What is the management of a (A) small and (B) large VSD?

Small: Closes spontaneously
Good dental hygiene to avoid IE

Large: Diuretics (captopril) for HF, nutritional support, surgery at 4-6 mnths

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What are the types of ASD?

Secundum (common 80%)
Central defect involving FO

Primum (less common)

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What are the clinical features of ASD?

Asymptomatic

Recurrent chest infections

Arrhythmias

ESM, fixed widely split S2

Partial = apical pansystolic murmur

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What are the investigation findings in ASD?

CXR: Cardiomegaly, Enlarged pulm arteries, increased pulm vasc markings

ECG: Superior QRS

Echo: Structural abnormality, definitive

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How common is PDA?

1 in 5000

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Where is the join in PDA?

Pulmonary artery to descending aorta

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What are the clinical findings of PDA?

Continuous murmur below left clavicle

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What are the investigations in PDA?

CXR/ECG normal or LVH and pulmonary HTN

Echo: diagnostic

What is the management of a PDA?

Closure
Preterm: indomethacin/ ibuprofen for closure
Surgical ligation

Older child: Coil or occlusive device via cardiac catheter at 1 year

Diuretics if excess pulm blood flow

(If transposition of great arteries or other cyanotic disease: prostaglandins until corrective surgery because PDA needs to stay open)

What is the most common cyanotic CHD?

Tetralogy of Fallot

What are the 4 diagnostic criteria in Tetralogy of Fallot?

1. Subpulmonary stenosis causing right ventricular outflow tract obstruction 2. A large ventricular septal defect 3. Dextroposition of the aorta so that it overrides the ventricular septum 4. Right ventricular hypertrophy (as a result of RVOT obstruction)

What are some signs of ToF?

Severe cyanosis, tet spells, squatting on exercise Clubbing, ESM on LSE

What investigations are used in ToF?

Antenatal Murmur at 2months of life CXR: normal but small heart, uptilted apex (boot shaped heart), pulm art bay, decreased pulm vasc markings ECG: Normal at birth, RVH/ RAD Echo

What is the management of ToF?

Medical- prostaglandins to open DA Surgical at 6 months- closure of VSD and relieving RVOT Maybe: shunting to increase pulmonary flow (balloon or tube) Prolonged Tet spells: Sedation/ pain relief, IV propranolol, IV volume administration, bicarbonate, muscle paralysis and artificial ventilation

What is transposition of the great arteries? (TGA)

Aorta from RV and PA from LV Only compatible with life if opening between two circuits (e.g. PDA)

What are the signs of TGA?

Cyanosis Loud single second heart sound No murmur but potentially systolic

What are the investigation findings of TGA?

CXR: Egg on string mediastinum, increase pulm vasc ECG: normal Echo: confirmatory

What is the management of TGA?

Maintain PDA with prostaglandins Balloon atrial septostomy Arterial switch

What is pulmonary stenosis?

Partial fusion of Pulmonary valve leaflets

What are the clinical features of Pulmonary Stenosis?

Asymptomatic Cyanotis ESM at ULSE Ejection click at ULSE RV Heave

What are the investigation findings of Pulmonary Stenosis?

CXR: normal or post stenotic dilatation of the pulmonary artery ECG: RVH Echocardiogram

What is the management of Pulmonary Stenosis?

Transcatheter balloon dilation if pressure grad across pulmonary valve >64mm Hg

What is Aortic Stenosis?

Stenosis, valve 1-3 leaflets

What are the clinical features of Aortic Stenosis?

Reduced ET, chest pain, syncope ESM, slow rising pulse, carotid thrill, delayed and soft aortic second sound, apical ejection click

What are the investigation findings of Aortic Stenosis?

CXR: normal or prominent LV ECG: LVH Echo

What is the management of Aortic Stenosis?

Monitoring Balloon valvulotomy AV replacement

What is Coarctation of the Aorta?

Adult type: Narrowing of aorta, not duct dependent Other: ductus arteriosus, duct tissue encircling aorta

What are the clinical features of Coarctation of the Aorta (adult)?

Gradual Asymptomatic Radio femoral delay ESM at ULSE Collaterals heard at back HTN

What are the investigation findings of Coarctation of the Aorta (adult)?

CXR: Rib nothcing, 3 sign ECG: LVH Echo

What is the management of Coarctation of the Aorta?

Stent if severe Surgical repair

What are the clinical features of Coarctation of the Aorta (adult)?

Other: Acute circulatory collapse at day 2 when DA closes, sick baby, HF, absent femorals, metabolic acidosis

What are the investigation findings of Coarctation of the Aorta (adult)?

Other: CXR: Cardiomegaly (HF) ECG: normal

What is Eisenmenger syndrome and how does it present?

L to R shunt not treated and progresses onto thick resistive pulmonary arteries which eventually undergoes shunt reversal in teen years (cyanotic)

What is the management objective of Eisenmenger syndrome?

Early intervention

What is Interruption of the aortic arch?

Rare, R to L shunt, VSD present Presents with shock as a neonate

What is the management of an interruption of the aortic arch?

Complete correction with VSD closure and repair of aortic arch within first few days Associated with DiGeorge’s

What is hypoplastic left heart syndrome?

An underdeveloped left heart

How does hypoplastic left heart syndrome present?

Sick baby at birth Weakness/ absence of peripheral pulses Acidosis Cardiovascular collapse

What is the management of hypoplastic left heart syndrome?

Surgery (Norwood procedure)

How can we classify congenital heart disease?

Acyanotic: L to R (ASD, VSD, PDA), outflow obstruction (AS/PS, COA, hypoplastic L heart, Eisenmonger's syndrome) Cyanotic (TA, TGA (R to L), AVSD, ToF (R to L), Eisenmenger)