Congenital Heart Disease Flashcards
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
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
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
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
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
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
Features of Eisenmenger’s ??
A/W
- VSD, ASD, PDA
Features
- Original murmur may disappear
- Cyanosis, - Clubbing
- RV Hypertrophy
- Haemoptysis
- Embolism
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
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
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
CI of Pregnancy in VSD ??
Pulm. HTN as it carries a 30- 50% risk of Mortality
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
Features of TGA ??
Cyanosis
Tachypnoea
Loud Single S2
Prominent RV impulse
CXR: ‘Egg-on-side’ appearance
Rx-
- Maintain Ductus Arteriosus with PGs
- Surgical Correction
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
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
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
