L5: Cyanotic CHD Flashcards
Def of Cyanosis
It is bluish discoloration of skin and mucus membranes.
When Does Clinical Cyanosis Present?
Cyanosis is recognized at …..
- Higher levels of SaO2 ⇢ in patients with polycythemia.
- Lower levels of SaO2 ⇢ in patients with anemia
Etiology of Cyanosis
- Central
- Peripheral
- Methemoglobinemia
Etiology of Cyanosis
- Central
Cental Causes of Cyanosis
- Cardiac
Cental Causes of Cyanosis
- Pulmonary
◈ Obstruction of the airway
◈ Parenchymatous lung disease (e.g. pneumonia)
◈ Weakness of the respiratory muscles
Cental Causes of Cyanosis
- CNS
Central nervous system depression
Causes of Cyanosis
- Peripheral
“⇡⇡ extraction of oxygen by tissues.”
Causes of Cyanosis
- Methemoglobinemia
“Hemoglobinopathies
INVx for Cyanosis
INVx for Cyanosis
- Hyperoxia Test
Consequences of Cyanosis
Polycythemia in Cyanosis
- Pathogenesis
Polycythemia in Cyanosis
- Effects
Clubbing in Cyanosis
- Pathogenesis
◈ It results from soft tissue proliferation at the base of nail beds 2ry to chronic hypoxemia.
◈ It appears when the duration of central cyanosis > 6 months.
Clubbing in Cyanosis
- Site & Grades
Hypoxic “Hypercyanotic” in Cyanosis
- Incidence
◈ Common in 1st 6 months of life & decrease after 1st 2 years.
◈ Common in early morning.
Hypoxic “Hypercyanotic” in Cyanosis
- Provoked By
Feeding, crying & straining.
Hypoxic “Hypercyanotic” in Cyanosis
- Mechanism
Hypoxic “Hypercyanotic” in Cyanosis
- CP
Hypoxic “Hypercyanotic” in Cyanosis
- TTT
Squatting in Cyanosis
- Cause
- Seen in children with right-to-left shunt (e.g., TOF) to increase arterial oxygen saturation
Squatting in Cyanosis
- Effect
CNS Complications in Cyanosis
Brain Abcess in Cyanosis
- Cause
CVT in Cyanosis
- Site
- Incidence
- CP
Arterial Ischemic Stroke in Cyanosis
- Causes
- Caused by embolization of a thrombus in the cardiac chamber or in the systemic veins.
Bleeding Disorders in Cyanosis
- Causes
◈ Thrombocytopenia and defective platelet aggregation
◈ Consumption coagulopathy with prolonged PT & APTT
◈ Decreased levels of fibrinogen and factors V and VIII
Bleeding Disorders in Cyanosis
- CP
- Easy bruising,
- petechiae of skin & MM
- epistaxis
- gingival bleeding
Bleeding Disorders in Cyanosis
- TTT
Red cell withdrawal and replacement with equal volume of plasma
Low IQ in Cyanosis
- Children with cyanosis and chronic hypoxia have lower than expected intelligent quotient, poorer perceptual and gross motor function than children with acyanotic CHDs
Scoliosis in Cyanosis
Children with chronic cyanosis, particularly girls with TOF may have scoliosis
Incidence of TOF
The most common congenital cyanotic heart disease.
Pathology of TOF
Pathology of TOF
- The more the infundibular septum is displaced anteriorly, ……
Associated Anomalies to TOF
Associated Anomalies to TOF
- Coronary Arteries
Associated Anomalies to TOF
- PS
(sub valvular / valvular).
Associated Anomalies to TOF
- Aortic Arch
aortic arch is right sided in 20% of cases with TOF.
Associated Anomalies to TOF
- Venous Abnormalities
(TAPVR, PAPVR)
◈ Persistent left SVC drains into coronary sinus.
◈ Intrahepatic interruption of IVC with azygous continuation,
Associated Anomalies to TOF
- PDA
…
Phsyiology of TOF
Phsyiology of TOF
- The magnitude and direction of the shunt are determined by
Phsyiology of TOF
- Mild PS
Phsyiology of TOF
- Moderate PS
Phsyiology of TOF
- Severe PS
Phsyiology of TOF
- Cyanosis will increase in the following conditions:
Phsyiology of TOF
◈ If the shunt is left to right ⇢
◈ If the shunt is balanced ⇢
◈ If the right to left ⇢
◈ However, mild to moderate neonatal cyanosis tends to increase.
◈ By 5-8 years of age the majority of children are cyanotic and symptomatic.
….
CP of TOF
CP of TOF
- Hx
◈ Cyanosis
◈ Dyspnea on exertion
◈ Squatting
◈ Hypoxic spell
CP of TOF
- General Ex
- Central cyanosis “Mostly delayed to 2nd month”
- Blue clubbing “After 6 months”
- Squatting position
CP of TOF
- Local Ex
CP of TOF
- Inspection & Palpation
CP of TOF
- Apex
CP of TOF
- Auscultation
CP of TOF
- Heart Sounds
CP of TOF
- Murmur
INVx in TOF
INVx in TOF
- Chest X-Ray
INVx in TOF
- ECG
◈ Right axis deviation
◈ Right ventricular hypertrophy
INVx in TOF
- ECHO
Complications of TOF
Complications of TOF
- Hypoxic Spells
in infant less than 2 years
Complications of TOF
- Hematologic Abnormalities
Complications of TOF
- Infective endocarditis
Occurs on
- stenotic pulmonary valve
- thickened tricuspid valve
Complications of TOF
- CNS Complications
- Brain abscess in older children.
- Arterial ischemic strokes
- Cerebral venous thrombosis in infants < 2 years.
- Cerebrovascular accidents as hemorrhage
Management of TOF
Management of TOF
- Medical
Medical Management of TOF
- 1st Thing to do
Give PGE1
Medical Management of TOF
- Oral Iron
To prevent anemia
Medical Management of TOF
- Oral Propranolol
Medical Management of TOF
- Phlebotomy & Plasma Replacemet
⇢ For patients with high hematocrit
⇢ Prior to surgery in case of coagulation abnormalities
Medical Management of TOF
- IE Prophylaxis
…
Medical Management of TOF
- TTT of Hypercyanotic Spells
If present
“As mentioned before”
Surgical Management of TOF
Incidence of TGA
The most common congenital cyanotic heart disease in newborns.
Pathology in TGA
CP of TGA
CP of TGA
- General Ex
CP of TGA
- Auscultation
CP of TGA
- Heart Sounds
S2 ⇢ single, loud
CP of TGA
- murmur
No heart murmur is heard in infants with an intact ventricular septum.
- If associated with VSD ⇢ Pan systolic murmur
- If associated with PS ⇢ Ejection systolic murmur
INVx of TGA
INVx of TGA
- Chest X-Ray
INVx of TGA
- Others
Same as in TOF
+
ECG, ECHO, Pulse oximetry & ABG, CBC, Cardiac catheterization “Angiography”
Management of TGA
Management of TGA
- Medical
◈ Prostaglandin El infusion to improve arterial oxygen saturation by reopening the ductus.
◈ If associated with CHF may be treated with
- ACE
- Diuretics
- Digoxin.
Management of TGA
- Cardiac Cathetrization
Balloon atrial septostomy to ensure adequate intracardiac mixing.
Management of TGA
- Surgical