- Family history of a cardiac or noncardiac defect
Classification – I:-
- With left-to-right shunting (↑ pulmonary circulation)
- With right-to-left shunting (↓ pulmonary circulation)
- Without shunting
With left-to-right shunting:-
- Ventricular septal defect
- Atrial septal defect
- Patent ductusarteriosus
With right-to-left shunting:-
- Transposition of magistral vessels
- Fallot’s tetralogy
- Left heart hypoplasia
Without shunting:-
- Coarctation of aorta
- Stenosis of aorta
- Stenosis of pulmonary artery
Classification – II:-
- With increase of pulmonary circulation
- With decrease of pulmonary circulation With decrease of greater systemic circulation
- Without hemodynamic disturbances
- ___________
- With or without cyanosis
Echocardiography:-
- 3-dimensional (3D) echocardiographic images with a Doppler system with superimposing a color-coded direction and velocity of blood flow on the real-time images
Ventricular septal defect:-
- Most common: 17-30 %
- Size:0.1 cm – 3 cm
- Localization: in membranous or muscular part
- Delayed diagnosis: first months pulmonary pressure is higher → no cardiac murmur
Ventricular septal defect:-
- Small VSD: no significant hemodynamic derangement; loud murmur; can close spontaneously (in muscle segment)
- Large VSD: progressively leads to higher pulmonary resistance → irreversible pulmonary vascular changes, so-called Eisenmenger syndrome (reversal of shunt to right-to-left shunt)
- Rough pansystolic murmur in 3-4th intercostal space on the left border of sternum
- Accent of S2 over pulmonary artery (pulmonary hypertension)
- Left and right ventricular enlargement (palpable parasternal lift, laterally displaced apical impulse, widened heart borders)
ECG:-
- Small VSD → Normal
- Moderate VSD → signs of LV volume overload (deep Q and tall R waves with tall T waves in leads V5 and V6), LA overload (broad P wave), atrial fibrillation
- Severe VSD → right ventricular hypertrophy, with further progression - biventricular hypertrophy
- Early onset of CoA: cardiomegaly, pulmonary edema, and other signs of CHF.
- Late onset of CoA: cardiomegaly, arch indentation in the area of the coarctation, and rib notching
ECG:-
- Early onset of symptoms: RV rather than LV hypertrophy and evidence of ischemia.
- Late onset of symptoms: LV hypertrophy and signs of LV ischemia or strain.
Other tests:-
- Preductal and postductal pulse oximetry readings
- Cardiac catheterization (evaluation and possible balloon aortoplasty)
Management:-
- Prostaglandin E1 (0.05-0.15 mcg/kg/min) IV to open the ductusarteriosus
- Treatment of hypertension (beta-blockers and vasodilators)
- Treatment of CHF and shock in infants
Surgery:-
- End-to-endanastomosis
- Patchaortoplasty
- Left subclavian flap aortoplasty (left subclavian artery is brought back toward the left carotid artery to enlarge an area of transverse arch hypoplasia)
Complications:-
- Hypertension
- Intracranialhemorrhageand aneurysms
- Aorticruptureordissection
- CHF
- Recurrentcoarctationafter surgery
- Aortic aneurythm
- Paralysis(spinal cord ischemia)
- Cardiomyopathy
Prognosis:-
- Meanlife expectancy is 35 years
- Without surgery 1-st year mortality is 25%
- Requires bacterialendocarditisprophylaxis
Transposition of magistral vessels:-
- Aorta comes from RV and pulmonary artery comes from LV → 2 separated circulations
- No disturbance of fetal circulation
- Postnatal life is only possible with blood shunts (VSD, ASD, PDA)
Clinics:-
- Early total cyanosis from birth, not responsive to oxygen
- Tachycardia, tachypnoe, decreased BP
- Developmental deficit
- CHF by 1st-2nd month of life
Physical exam:-
- Murmurs – due to other defects (VSD, ASD, PDA)
- Cardiac borders widening
Chest X-ray and ECG:-
- Signs of RV (or both) hypertrophy
Management:-
- Urgent surgery!
Prognosis:-
- 95% die during 1st year of life without surgery
- Mean life expectancy is 3 months
Fallot’s tetralogy:-
- Most common among cyanotic heart defects
- Right-to-left shunt
Defects of TOF:-
- Ventricularseptaldefect
- Dextrapositionoftheaorta
- Obstruction of the right ventricular outflow tract (PA stenosis)
- Rightventricularhypertrophy
- Pentade: + Atrial septal defect
- Triade: no VSD, shunt through Open oval fenestra