Congenital Heart Disease Flashcards by Arhanth Prema

MCC of Acyanotic Heart disease

VSD (MC, accounts for 30% cases)
ASD
PDA
Coarctation of Aorta
AV stenosis
Note: VSD are MC than ASD. But in adult pts., ASD are the MC new Dx. as they generally present later

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MCC of Cyanotic HD ??

ToF, TGA, Truncus Arteriosus, TAPVR
Hypoplastic Left Heart Syndrome

ToF is more common than TGA
- At births, TGA is more common at birth as ToF pts. present at 1-2 months

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Hallmark of Ebstein’s Anomaly ??

Low insertion of TV => Large RA & Small RV
- Atrialisation of RV
Cause: LITHIUM Exposure in-utero
Associations
- Patent FO or ASD in 80% cases => Shunt b/w RA & LA
- WPW Syndrome

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C/F of Ebstein’s Anomaly

Cyanosis
Prominent ‘A’ wave on JVP
Hepatomegaly
TR: Pan Systolic murmur, worse on inspiration
RBBB => widely split S1 & S2

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Hallmarks of Eisenmenger’s Synd. ??

Reversal of Left to Rt. shunt in a Congenital Heart defect
- Due to Pulm. HTN
Uncorrected Lt. => Rt. shunt leads to Remodelling of Pulm. Vasculature => Pulm. Blood flow obstruction & Pulm. HTN
Rx.- Heart-Lung Transplantation

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Hallmarks of VSD ??

MCC of Congenital Heart defect
- Close spontaneously on 50%
Features
Can be detected in-utero at 20wks scan. Postnatal C/F
- FTT
- Features of HF: Hepatomegaly, Tachyponea, Tachycardia, Pallor
- Pan Systolic Murmur: Louder in SMALLER defect

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Features of Eisenmenger’s ??

A/W
- VSD, ASD, PDA
Features
- Original murmur may disappear
- Cyanosis, - Clubbing
- RV Hypertrophy
- Haemoptysis
- Embolism

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Causes of VSD ??

Congenital VSDs are a/w Chr. defects
- Down’s
- Edward’s
- Patau
- Cri-du-chat syndrome
Congenital Infection
Acquired Causes
- Post-MI

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Rx. of VSD ??

Small VSDs
- Asymptomatic, close Spontaneously requires monitoring
Maderate to Large VSD (usually result in a degree of HF in the 1st few months
- Nutritional Support
- HF medicines: eg.- Diuretics
- Surgical Closure of Defect

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Complications of VSD ??

AR : Due to poorly supported Rt. Coronary Cusp => Cusp collapse
Infective Endocarditis
Eisenmenger’s Complex
- Pulm. HTN => RVH & Increased RV pressure => Eventually exceeds LV Pressure => Reversal of Blood flow
- This results in Cyanosis & Clubbing
RV Failure
Pulm. HTN

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CI of Pregnancy in VSD ??

Pulm. HTN as it carries a 30- 50% risk of Mortality

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Hallmarks of TGA ??

Form of Cyanotic Heart Disease
- Failure of Aortico-Pulmonary Septum to Spiral during Septation
- Kids of DM mothers are at increased risk
AORTA leaves the RV
Pulm. TRUNK leaves the LV

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Features of TGA ??

Cyanosis
Tachypnoea
Loud Single S2
Prominent RV impulse
CXR: ‘Egg-on-side’ appearance
Rx-
- Maintain Ductus Arteriosus with PGs
- Surgical Correction

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Ostium Secundum & Primum ??

O SECUNDUM
- 70% of ASD
- a/w Holt-Oram Synd. (Tri-Phalangeal Thumbs)
- RBBB with RAD
O PRIMUM
- Presents earlier than O Secundum
- a/w abnormal AV valves
- RBBB with LAD, Prolonged PR interval

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Hallmark of ASD ??

MC congenital heart defect found in Adulthood
- 50% are dead by 50 yrs
2 types of ASD
- Ostium Secundum (MC defect)
- Ostium Primum
ESM + Fixed splitting S2

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Hallmark of PDA ??

Generally classed as ‘Acyanotic’ but if left uncorrected will result in late Cyanosis in the Lower limbs
- DIFFERENTIAL Cyanosis
Connection b/w Pulm. Trunk & Descending Aorta
- PDA closes with 1st breath due to increased Pulm. Flow => enhances PG clearance

What is Paradoxical Embolus ??

MC in PFO cases
Less common in ASD
DVT emboli from venous side through ASD can pass to the Left Heart & cause STROKE

Features of PDA ??

Left Sun-Clavicular Thrill
Continuous Machinery Murmur
Large vol., Bounding, Collapsing pulse
Wide Pulse Pressure
Heaving Apex Beat

Rx. of PDA ??

INDOMETHACIN or IBUPROFEN
- Given to the Neonate
- Inhibits PGs synthesis
- Closes the connection in majority of cases
If a/w another Congenital H D amenable to Sx., then
- PG-E1 is useful to keep the duct open until surgical repair

What drug keeps the PDA
- Open ???
- Closes PDA ??

PGs keep it open
Indomethacin or Ibuprofen closes PDA

Hallmark of Arrhythmogenic RV CM

ARVC aka ARVD is a form of Inherited CV disease which may present with Syncope or SCD
- 2nd MCC of death in Young adults (after HOCM)
A D condition, RV myocardium is replaced by Fatty & Fibro-Fatty tissue
- Due to mutation in the genes encoding for components of Desmosome

In whom is PDA more common ??

Pre-Mature babies, born at High Altitude
Maternal Rubella infection in 1st Trimester

Hallmark of Patent F O ??

PFO is present in 20% population
- It may allow Embolus to pass from Rt. Heart to Lt. Heart => STROKE termed as ‘Paradoxical Embolus’
- Improvement in Migraine after PFO closure
Rx. of PFO with Stroke
- Anti-Platelet therapy, AC (or)
- PFO Closure

Features of ARVD or ARVC ??

Palpitations, Syncope, SCD
ECG
- TwI at V1 to V3
- Epsilon wave : Terminal notching in QRS complex
Echo: Enlarged Hypokinetic RV with Thin free wall
MRI: To show Fibro-fatty tissue

What is Naxos Disease ??

A R Variant of ARVC Triad of - ARVC - Palmoplantar Keratosis - Wooly Hair

What is Catecholaminergic Polymorphic VT ??

A D condition It is a form of inherited cardiac disease a/w SCD Due to defect in Ryanodine receptors (RYR2) which is found in Myocardial Sarcoplasmic Reticulum

Rx. of ARVD or ARVC ??

SOTALOL (Most widely used) Catheter Ablation to prevent VT ICD

Hallmarks of Brugada Syndrome ??

Inherited CV disease; A D condition - MC among Asians Mutation in SCN5A gene which encodes the myocardial Na+ ion channel protein Rx.- ICD

Features of Catecholaminergic Polymorphic VT ??

Exercise or Emotion induced Polymorphic VT resulting in Syncope SCDeath C/F generally develop before 20yrs Rx.- - Beta Blockers - ICD

ECG changes in Brugada syndrome ??

Convex ST elevation > 2mm in > 1 of V1 to V3 followed by (-)ve T wave Partial RBBB IoC: Administer Flecainide or Ajmaline => ECG changes become more apparent

Hallmark of Long QT Synd. ??

LQTS is an Inherited condition a/w delayed repolarization of Ventricles - This can lead to VT or T de pointes => Collapse/ Sudden death MC variant: Defect in Alpha subunit of Slow delayed Rectifier K+ Channel

MC defect seen in LQTS ??

MC variants (LQT1 & LQT2) are caused by defect in - Alpha subunit of Slow delayed Rectifier K+ Channel. Normal Corrected QT interval is - Men: < 430ms - Women: < 450ms

Causes of Prolonged QT Interval ??

Congenital - J-L Nielsen (SNHL + due to Abnormal K+ channel) - R-Ward Syndrome (No SNHL) DRUGS (By K+ channel blockade) - Amiodarone, Sotalol, Class 1a - TCAs, SSRIs (specially Citalopram) - Atypical Antipyschotics (Risperidone) - Methadone, - Chloroquine - Terfenadine, - Ondansetron - Erythromycin, - Haloperidol ELECTROLYTES - Hypo- Ca2+, K+, Mg2+ - Acute MI - Myocarditis - Hypothermia - SAH

Features of LQTS ??

Picked up in Routine ECGs or Family Screening - LQT-1 : a/w Exertional Syncope, often after Swimming - LQT-2 : a/w Syncope following Emotional stress, Exercise or Auditory Stimuli - LQT-3 : Often occur at Night or at Rest - SCDeath

Rx. of LQTS ??

Avoid drugs that prolong QT interval Beta-blocker ICD in High risk cases Sotalol can exacerbate LQTS

Strongest marker of Poor Prognosis in HOCM ??

Septal thickness > 3 cm