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Congenital Heart Diseases-1
Congenital Heart Diseases-1
Congenital Heart Diseases-1
Anomaly Associations
Trisomy 21 VSD, AV Canal
Trisomy 18 VSD, PDA
Trisomy 13 VSD, PDA, Dextrocardia
Turner Coarctation, AS
Noonan PS, HCM
Williams Supravalvar AS, Peripheral PS
Holt-Oram ASD
Marfan Aortic root dilation, MVP
DiGeorge VSD, arch anomalies, TOF
CHD - diagnosis
Infective endocarditis
High risk particularly in:
Cyanotic CHD without surgical repair, residual defects, palliative shunts
Prosthetic valves
Residual defects after surgical or cath. closure
Patients with previous IE
CHD – GENERAL PROBLEMS
AND COMPLICATIONS
Pregnancy
Generally well tolerated but;
Extremely High risk (high mortality 30-50%):
Severe pulmonary art. hypertension (Eisenmenger syndrome)
High risk
Cyanosis (esp. SpO2 <85%)
Stenotic left valve disease (severe AS, MS)
Poor EF LV (<40%)
NYHA III-IV
Aortic root dilatation (Marfan syndrome)
Arrhythmias
Congenital heart disease with high risk and extremely high risk
for pregnancy (1)
Significantly increased risk of maternal Extremely high risk of maternal mortality or
mortality or severe morbidity (mWHO class severe morbidity (mWHO class IV)*
III) (cardiac event rate 19–27%) (cardiac event rate 40–100%)
Unrepaired cyanotic heart disease Pulmonary arterial hypertension
Moderate LV impairment (EF 30–45%) Severe LV impairment (EF <30% or NYHA class
III–IV)
Systemic RV with good or mildly decreased Systemic RV with moderate or severely
ventricular function decreased ventricular function
Fontan circulation. If the patient is otherwise Fontan with any complication
well and the cardiac condition uncomplicated
Severe asymptomatic AS Severe symptomatic AS
©ESC
*Pregnancy should definitely be avoided in women with these conditions.
Modified from the ESC Guidelines for the management of cardiovascular disease during pregnancy.
www.escardio.org/guidelines Congenital heart disease with high risk and extremely high risk
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
(European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
for pregnancy (2)
Significantly increased risk of maternal Extremely high risk of maternal mortality or
mortality or severe morbidity (mWHO class severe morbidity (mWHO class IV)*
III) (cardiac event rate 19–27%) (cardiac event rate 40–100%)
Moderate mitral stenosis Severe mitral stenosis
Moderate aortic dilatation (40–45 mm in Severe aortic dilatation (>45 mm in Marfan
Marfan syndrome or other HTAD; 45–50 mm syndrome or other HTAD, >50 mm in BAV,
in BAV, 20–25 mm/m2 in Turner syndrome) >25 mm/m2 in Turner syndrome)
Mechanical valve Severe (re-)coarctation
©ESC
*Pregnancy should definitely be avoided in women with these conditions.
Modified from the ESC Guidelines for the management of cardiovascular disease during pregnancy.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
CHD – GENERAL PROBLEMS
AND COMPLICATIONS
Malpositions of Heart
Dextrocardia may be accompanied by situs inversus
Shunts
Obstructions (Obstructive CHD)
SHUNTS
Left to Right shunts (Acyanotic or Late Cyanotic group): cyanosis months or years
after birth
1. Ventricular septal defect (VSD)
2. Atrial septal defect (ASD)-
3. Patent ductus arteriosus (PDA)
Right to Left shunts (Cyanotic group)
1. Tetralogy of Fallot (TOF)
2. Transposition of great arteries
3. Persistent truncus arteriosus
4. Tricuspid atresia and stenosis
L→R SHUNT
↑pulm blood flow → ↑pulm pressure →RVH →potential cardiac failure
↑pulm blood flow → medial hypertrophy + intimal proliferation to
prevent pulmonary edema
prolonged ↑pulm pressure (> even systemic pressure) → reversing the
flow R →L: deoxygenated blood in systemic circulation → late cyanosis
or Eisenmenger syndrome
Once significant pulmonary HT develops, surgical Rx of cardiac defects
not possible
R→L SHUNT
↓pulm blood flow →poor oxygenation of blood → enters Lt heart
→systemic circulation → dusky blueness of mucus membranes and skin
(Cyanosis)
Functional anemia → increased synthesis of Hb + RBC mass
(polycythemia)
Emboli from peripheral veins do not undergo filtration action of lungs
→enter Lt heart →embolize to systemic circulation(paradoxical emboli) →
cause brain infarction & abscess
Clubbing (hypertrophic osteoarthropathy) of tips of fingers and toes
ATRIAL SEPTAL DEFECT
ANATOMY/PREVALENCE
• Secundum 75%
• Primum 15%
• Sinus Venosus 10%
• Cor Sinus (rare)
ATRIAL SEPTAL DEFECT - PATHOPHYSIOLOGY
Naturally L to R shunt (higher BP in LA)
Volume overload of RV – dilation RA+RV
Arrhythmias (Atrial fibrillation, flutter) (5th decade)
Increase of transpulmonary flow – reactive higher pulmonary vascular
resistance
Pulmonary hypertension can develop in 20s and 30s if large ASD is not
repaired
Severe PAH (only in 5%) and bidirectional shunt (Eisenmenger physiology)
Paradoxical embolism (thrombus from lower limb veins through ASD to
systemic circulation e.g. CNS)
ATRIAL SEPTAL DEFECTS(ASD)- CLINICAL
or LV disease.
cIn elderly patients not
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
VENTRICULAR SEPTAL DEFECT
• Several types
• Asymptomatic if small
VENTRICULAR SEPTAL DEFECT
PATHOPHYSIOLOGY
©ESC
bIncludes all patients with desaturation at rest (Eisenmenger physiology) or on exercise
cCareful individual decision in expert centres is required.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
PATENT DUCTUS ARTERIOSUS (PDA)
Communication between the pulmonary
artery and the aorta
Location- distal to the subclavian artery
F:M = 2:1
Maternal rubella, prematurity
Asymptomatic if small
Signs of pulmonary over-circulation if
large
PATENT DUCTUS ARTERIOSUS
Ductus arteriosus is normal connection b/w aorta and bifurcation of
pulmonary A
Normally closes at 1st or 2nd day of life, > 3months persistence is abnormal
Cause: possibly due to ↑ levels of PGE2 after birth - seen in children with
respiratory distress syndrome - pharmacologic closure with indomethacin
(PGE2 inhibitor)
Most often does not produce functional difficulties at birth
A narrow ductus: no effect on growth and development during childhood
PDA-PHYSICAL EXAMINATION
36
aLV enlargement with increased stroke volume.
bIncludes all patients with lower limb desaturation at rest (Eisenmenger physiology) or on exercise.
cCareful individual decision in expert centres is required.
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
EISENMENGER’S SYNDROME
Final common pathway for all significant LR shunting in which
unrestricted pulmonary blood flow leads to pulmonary vaso-
occlusive disease (PVOD); RL shunting/cyanosis develops
Generally need Qp:Qs >2:1
PULMONARY HYPERTENSION AND EISENMENGER SYNDROME
Coagulopathy/platelet consumption
Polycythemia
Brain abscesses
Cerebral micro emboli
Airway hemorrhage
especially moving from lowerhigher altitudes (air travel, mountains)
EISENMENGER: TREATMENT
Polycythemia phlebotomy
Careful if microcytosis, strongest predictor of cerebrovascular events
RULE OUT CORRECTABLE DISEASE
Once diagnosis established, avoid aggressive testing as many patients die
during cardiovascular procedures
Diuretics prn, oxygen
Definitive: Heart Lung transplant
Prostacyclin therapy may delay, expensive
OBSTRUCTIONS
Coarctation of Aorta
Aortic stenosis and atresia
Pulmonary stenosis and atresia
COARCTATION OF AORTA
Narrowing in proximal descending aorta
May be long/tubular but most commonly discrete ridge
30 % of Turner syndrome patients have CoA
50 – 85% of patients with CoA have bicuspid aortic
valve
More common in males ( 2: 1)
Natural hx: poor prognosis if unrepaired
Aortic Aneurysm/dissection
CHF
Premature CADz
COARCTATION OF THE AORTA
• Surgical correction
1) Patch aortoplasty with removal of segment
and end to end anastomosis or subclavian flap repair
2) bypass tube grafting around segment
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
RIGHT TO LEFT SHUNTS (CYANOTIC CHD)
Electrophysiologic
Conduction defects – CRBBB
Sinoatrial dysfunction – sick sinus
Tachyarrhythmias
Complete heart block
Ventricular ectopy
58
Management of repaired tetralogy of Fallot: long-term
complications to address during follow-up
©ESC
Ao = aorta; LV = left ventricle;
RV = right ventricle; PA =
Pulmonary artery; TV =
tricuspid valve; RA = right
atrium; RV = right ventricular;
LA = left atrium; PA =
pulmonary artery; PS =
pulmonary stenosis; SCD =
sudden cardiac death; VSD =
ventricular septal defect.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Thank you for your attention!!!