CVD

1. Cardiovascular Diseases (CVD)
Dr. Goitom Gebreyesus, MD

2. Cardiovascular Diseases
• Most are congenital
• Older children are more likely to have
acquired heart disease e.g.
• Rheumatic fever, myocarditis
• The heart may also be affected in many
systemic disorders e.g.
• Infections, malnutrition, anemia, electrolyte
disturbance, renal disease etc..

3. Evaluation of CVS: Hx
• Details of the perinatal period:
• cyanosis, respiratory distress or prematurity
• Maternal Diabetes Mellitus, infections,
SLE…
• Timing of 1st presentation of cardiac
symptoms
• Symptoms of CHF – age specific
• Infancy: feeding difficulties, respiratory
distress
• Older children: exercise intolerance

4. Hx….
• Cyanosis during crying or exercise
• Other diseases or disorders or congenital
malformations that affect the heart
Physical examination
• General
• Cyanosis:
needs ~ 5mg/dl of deoxygenated hemoglobin for
detection
• Differential cyanosis
• Abnormality of growth,
• Evidence of respiratory distress

5. Physical…
• Hepatomegaly
Total span: at birth-4.5cm, 1yr-6cm, 5yr-7cm,
and at 12yr-8-9cm
• Occasionally splenomegaly
• Heart rate: persistent tachycardia
>200 in neonates
>150 in infants
>120 in older children
• Character of Pulses:

6. Physical…
Wide pulse pressure with bounding pulse - PDA,
AI, A-V shunts or anemia
Diminished pulse in all extremities: pericardial
tamponade, L-ventricular outflow obstruction or
Cardiomyopathy
Radio–femoral delay in coarctation of aorta
• Blood pressure (arms & legs)
• In older children, JVP in sitting position
• edema

7. Physical…
• Cardiac
• Precordial bulge- cardiomegaly
• Substernal thrust- Right ventricular
hypertrophy
• Apical heave = Left ventricular hypertrophy
• Hyper dynamic precordium- volume load
• Silent precordium- pericardial effusion or
Cardiomyopathy
• Murmurs

8. Investigations
• Chest X-ray
Cardiac size and shape
Pulmonary blood flow and edema
Lung and thoracic anomalies related to CHD
• Hematologic data
Polycythemia (Hct > 65) – in cyanotic patients
Coagulation factors
• Echocardiography
Cardiac structures in CHD
Estimate intra-cardiac pressures
Cardiac muscle contractions

9. Investigation…
Direction of flow across defect
Ejection fractions
Presence of vegetations due to IE
Presence of pericardial effusion
• Exercise test
Severity of cardiac abnormalities

10. Congenital Heart Diseases (CHD)
• Occur in 0.5 – 0.8% of live births
• Etiology unknown in most cases
• Can be classified based on:
Presence or absence of cyanosis
Chest X-ray evidence of increased or decreased
pulmonary vascular flow or markings

11. Classification of CHD
CHD
ACYANOTIC CYANOTIC
Volume Load Pressure Load Volume Load Pressure Load
ASD
VSD
PDA
Regurgitant
Lesions
PS
AS
C.Aorta
TS
TOF
P. Atresia
T. Atresia
Trans. GA
Single Vent.

12. Ventricular Septal Defect (VSD)
• The most common CHD ~ 25% of all
• Two types
Membranous (most common)
Muscular
• Pathophysiology
• Shunting of blood from L to R ventricle
• L to R shunt depend on:
Size of VSD and
Pulmonary vascular resistance (PVR)

13. VSD…
• In small VSD right V pressure is normal ➞ high
pressure gradient from L to R
• In large VSD there is large L to R shunt
Symptoms appear
Increased PVR
• If pulmonary and systemic pressures equalize
➞ clinical symptoms improve
• Later when right V pressure exceeds that of
left ➞ patient becomes cyanotic
• This condition is called Eisenmenger’s
physiology

14. VSD…
• Clinical M.
• Varies according to:
 Size of the defect and
 Pulmonary blood flow & pressure
• In small VSD (most common):
 Patients are asymptomatic
 Characteristically there is a loud, harsh or blowing
holosystolic murmur (over LLSB)
• In large VSD:
 Patients are symptomatic with dyspnea, feeding
difficulties, poor growth, facial diaphoresis, recurrent
respiratory infections and congestive heart failure
 N.B – murmur of large VSD is less harsh than small VSD

15. VSD…
• Investigations
• Chest X-ray
• In small VSD – normal
• In large VSD –
 Gross cardiomegaly with biventricular prominence
 Increased pulmonary vascular markings
 Pulmonary edema, pleural effusion
• EKG
• In small VSD – normal or Left V hypertrophy
• In large VSD –
 Biventricular hypertrophy
 P wave notched or peaked

16. VSD…
• Echocardiography
Position and size of VSD
• Prognosis and Complications
Natural course depend on the size of defect
30-50% of small defects spontaneously close in the
first 2yrs of life
Majority of VSD that close do so before 4yr of
age
Most with small VSD remain asymptomatic; long
term risk of Infective Endocarditis (IE)
Those with large VSD have repeated respiratory
infections & heart failure despite optimal Rx

17. VSD…
• Treatment
• Small VSD
Reassurance
Encouraged to live normal life; no restriction on
physical activities
Surgical repair not recommended
Prophylaxis against IE for dental procedure,
tonsillectomy, adenoidectomy and instrumentation
of genito-urinary and lower intestinal tracts

18. VSD…
• Large VSD
• Medical includes:
Control of heart failure
Prevent
Maintenance of normal growth
• Surgical closure of defect
Patients at any age with large VSD in whom
medical Rx failed
Infants 6 -12 month with pulmonary HPN even if
symptoms are controlled with medical Rx

19. VSD…
Severe pulmonary vascular disease is a contra-
indication to closure of defect
Long term prognosis after surgery is excellent

20. Tetralogy of Fallot (TOF)
• Cyanotic CHD
• Consists of:
 Pulmonary stenosis
 VSD
 Overriding aorta
 Right ventricular hypertrophy
• Clinical manifestation
• In infants with mild degree of right V outflow
obstruction (RVOO) manifest with:
 Initially with congestive heart failure (CHF)
 Often cyanosis not present at birth

21. TOF…
• In severe degree of RVOO:
Cyanosis noted immediately
Pulmonary blood flow in the first few days depend
on PDA
When ductus closes ➞severe cyanosis and
circulatory collapse
• Dyspnea on exertion (assume SQUATTING
position)
• Characteristic paroxysmal hypercyanotic
attacks (hypoxic, ‘blue’ or ‘tet’ spells)

22. TOF…
• Spells are common in the first 2yrs of life
 Infant becomes hyperpneic and restless, cyanosis
increases; and has gasping respiration and syncope
• Spells occur in the morning on awakening or follow
vigorous crying
• Depending on the frequency and severity of spells one or
more of the following procedures should be done:
 Placement of infant in knee-chest position
 Administration of O2
 Morphine to calm the patient
• Delayed growth and development
• Ejection systolic murmur at upper SB

23. TOF…
• Investigations
• Chest X-ray
Typically narrow base
Concave left heart border
Normal heart size with rounded apex
Decreased pulmonary blood flow
• EKG
Evidence of right ventricular hypertrophy
• Echocardiography
Establishes diagnosis

24. TOF…
• Prognosis
Most are susceptible to complications
Cerebral thrombosis in the presence of extreme
Polycythemia and dehydration
Brain abscess
Infective Endocarditis
• Treatment
• Depends on severity of RVOO

25. TOF…
• In severe form
IV prostaglandin E1 to keep ductus arteriosus open
Surgical intervention: first ➞palliative systemic to
pulmonary artery shunt; second ➞ corrective
surgery to relieve RVOO and repair of VSD

26. Transposition of Great Arteries
(TGA)
• Cyanotic CHD
• Aorta arises from RV and pulmonary artery
from LV
• Systemic and pulmonary circulation consist
of two parallel circuits
• Survival is provided by patent foramen
ovale and PDA
• Half of patients with TGA have VSD

27. TGA…
• Common in infants of diabetic mothers
• Prior to modern surgical treatment
mortality was greater than 90%
• Clinical manifestation
Cyanosis with in the first hours or days of life
A medical emergency
Early diagnosis and appropriate intervention can
avert severe hypoxemia and acidosis which leads to
death

28. TGA…
• Investigations
• Chest X-ray
 Mild cardiomegaly
 Normal to increased pulmonary blood flow
• Echocardiography
 Confirms diagnosis
• Treatment
• prostaglandin E1 should be initiated immediately to
maintain PDA
• Arterial switch (Jaten) procedure which has a survival
rate of 90-95%