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Congenital Heart Disease

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by

Dr. Pablo Cuellar

on 3 August 2016

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Transcript of Congenital Heart Disease

Cyanotic
: Blue-purple discoloration of the skin due to deoxygenated hemoglobin
(4g/dl) circulating through a defect bypassing the lungs

Acyanotic
: Intracardiac or vascular stenoses and regurgitation or left-to-right shunting of blood.
Categories
Imaging
Chest radiographs: right atrial and ventricular dilation, pulmonary artery may be prominent
Ventricular Septal Defect
Pathophysiology

Depends on the size of defect; if the hole is large the volume of the shunt is determined by the pulmonary and vascular resistances
Over time increased volume overload causes chamber dilatation and Pulmonary vascular disease

Epidemiology
Leading cause of death from birth abnormalities
1st year of life
8 of 1,000 live births
1 mlln in the U.S with CHD
Adults outnumber children
Congenital Heart Disease
Etiology
Common form of birth defects
Genetic abnormalities leading to aberrant
embryonic development

If detection does not occur after birth CHD will become manifest during adulthood
Acyanotic Lesions
Atrial Septal Defect
Persistent opening in the interatrial septum comunicating left and right atria
1 in 1,500 live births (common)
Most common: septum secundum defects
Pathophysiology

Uncomplicated left-to-right shunt
Flow through the defect depends on:
Size of the defect and filling properties of the ventricle
Symptoms
Usually asymptomatic during early life
Tendency for respiratory infections
Adulthood (4th decade) atrial arrhytmias, pulmonary hypertension, bidirectional and then right-to-left shunting of blood and cardiac failure.
Physical Examination
Increased volume of blood flowing across the PV creates a systolic murmur at the upper-left sternal border.
Mid-diastolic murmur at the lower left sternal border-increased flow across the tricuspid valve.
Electrocardiogram (ECG)

Right ventricular hypertrophy and right atrial enlargement

Echocardiography
(GOLD STANDARD)

Right atrial and right ventricular enlargement and ASD is directly visualized
O2 saturation left and right atria
Abnormal opening of the interventricular septum
Most common form of congenital heart defect
1.5-3.5 per 1000 live births
Located in the membranous (70%) and muscular (20%) portions of the septum
Symptoms

Small VSD asymptomatic
Large VSD develop early symptoms of congestive heart failure: tachypnea, poor feeding, failure to thrive, tendency for respiratory infections.
Dyspnea and cyanosis when reversed shunt occurs
Physical Examination

Holosystolic murmur at left sternal border
Smaller defects cause loudest murmurs
A thrill can be palpated over the region of the murmur
Diagnostics
Enlarged pulmonary artery on radiograph and cardiomegaly
Echocardiography shows the VSD accurately and its location
(GOLD STANDARD)
The Ductus Arteriosus connects the left pulmonary artery to the descending aorta during fetal life
PDA results from closing failure of this structure
Patent Ductus Arteriosus (PDA)
1 in every 2,500-5000 live term births
Risk factors: rubella infection, birth at high altitude, prematurity, drugs
Pathophysiology
Normally: Sudden rise in blood oxygen tension and reduction of circulating prostaglandins constricts smooth muscle of DA and intimal proliferation and fibrosis result in permanent closure
The magnitude of flow through the shunt depends on area and lenght of the ductus and the vascular resistances
Postnatally the shunt reverses direction from aorta to the pulmonary circulation and LA and LV become overloaded

"If Eisenmenger develops due to pulmonary disease the resulting flow of low O2 blood causes distal cyanosis (feet) and clubbing"
Symptoms

Early congestive heart failure with tachycardia, poor feeding, slow growth, lower respiratory infections
Fatigue, dyspnea and palpitations in adolescence and adult life
Physical Exam

Continuous,
machine-like
murmur at the left subclavicular region and back.
If Eisenmeger develops murmur becomes shorter (gradient)
Cyanosis
Clubbing
Radiograph shows calcification of the ductus
Left atrial and left ventricular enlargement

Echocardiography
with doppler shows the defect
Congenital aortic stenosis
Caused by abnormal structural development of the valve leaflets.
5 of 10,000 live births and four times as common in males as in females
Usually associated to bicuspid aortic valve
Bicuspid valves become progresively stenotic over time and progressively fibrose and calcify.
Pathophysiology

Valvular orifice is significantly narrowed elevating left ventricular sistolic pressure to pump blood across the valve (ventricular hypertrophy)
Ventricular hypertrophy pumps blood with high velocity and the impact causes dilatation of the aortic wall
Symptoms
Less than 10% of infants experience heart failure symptoms before age 1, if they do: tachycardia, tachypnea, failure to thrive, and poor feeding.

Most older children are asymptomatic and develop normally. When symptoms occur, they are similar to those of adult: fatigue, dyspnea, angina pectoris and syncope.
Physical Exam
Systolic murmur (crescendo-decresendo) loudest at the base of the heart with radiation to the neck.
Present from birth, not depending from the pulmonary vascular resistance.

Advanced disease

Peak of the murmur
occurs later in systole
Coarctation of the Aorta
Pulmonic Stenosis
Obstruction to right ventricular outflow may occur at the level of the valve, within the body of the RV, or in the pulmonary artery.
Valvular pulmonic stenosis is the most frequent form.
Pathophysiology
Impairment of right ventricular outflow
Increased RV pressures and chamber hypertrophy
Right heart failure
Symptoms
Severe stenosis: dyspnea with exertion, exercise intolerance and abdominal fullness with pedal edemas (right sided heart failure).
Physical Exam
Jugular Venous "
a
" wave
Systolic murmur at the upper-left sternal border and thrill
In moderate stenosis a pulmonic ejection sound or "click" before the murmur ( inspiration)
Diagnostics
Chest radiography shows pulmonary artery dilation and RV and RA enlargement
Echocardiography with doppler assesses
Narrowing of the aortic lumen
Incidence 1 in 6,000 live births with associated bicuspid aortic valve.
Often occurs in patients with Turner Syndrome (45,XO)
Often juxtaductal ("next" to the ductus)
Pathogenesis contends that reduce antegrade blood flow and ectopic muscular ductus arteriosus tissue may affect
Impedance of aortic narrowing causes LV increased afterload. Blood flow to the upper part of the body and lower part of the body differ. If coarctation persists, compensatory alterations include: 1) ventricular hypertrophy and 2) dilatation of collateral blood vessels (intercostal arteries).
Symptoms
Heart failure symptoms for severe cases
Cyanosis in case of patent ductus (
distal
)
Weakness or pain in lower extremities following excercise (claudication)
Physical Exam
Pulses are weak and delayed (femoral)
Blood pressure may differ
(15-20 mm Hg)
Midsystolic ejection murmur (chest or back)
Cyanotic Lesions
Tetralogy of Fallot


Most common form of cyanotic CHD
5 every 10,000 live births
Pathophysiology

Increased resistance by the pulmonic stenosis causes the deoxygenated blood to be diverted into the systemic circulation resulting in hypoxemia and cyanosis
Symptoms
Dyspnea on exertion
(feeding, crying)
Syncope or convulsions
Squating down (increase systemic vascular resistance)
Physical examination
Cyanosis (lips, mucous membranes and digits)
Clubbing of the fingers and toes
Systolic ejection murmur
Transposition of the Great Arteries (TGA)
Aorta originates from the RV and pulmonary artery from the LV
7% of CHDs, affecting 40 of 100,000 live births
Pathophysiology
TGA Forces desaturated blood from the systemic venous system to return to the systemic circulation without undergoing oxygenation
Symptoms and physical examination
Infants with TGA appear blue (generalized cyanosis)
Palpation of the chest reveals a right ventricular impulse at the lower sternal border
Eisenmenger syndrome
Severe pulmonary vascular obstruction that results from chronic left-to-right shunting
Elevated pulmonary vascular resistance causes reversal of the shunt and systemic cyanosis
Pulmonary media hypertrophies and the intima proliferates reducing the cross-sectional area of the pulmonary vascular bed
Other ASDs:
Persistent foramen ovale (PFO): non-closure of the foramen ovale*
Septum primum: no fusion with endocardial cushions
Sinus Venous defect
Diagnostic studies
Chest radiography:
Prominent Right Ventricle and decreased size of the main pulmonary artery segment (boot-shaped heart).
Pulmonary markings are diminished because of decreased flow*
Defects can be seen on echocardiography
Echocardiography gives definitive
diagnosis
A 24 year-old med-student is brought to the consultation after having a murmur detected during class practice. She's never had any complains or symptoms until now. Physical examination reveals a holosystolic murmur best heard at the left sternal border. Echochardiography with doppler identifies a shunt from the left ventricle to the right ventricle, pressure is the same in both ventricles and there is augmented circulation in the pulmonary vasculature.
Which of the following complications is most likely to develop if this patient is not treated correctly?
A. Left sided heart failure
B. Eisenmenger syndrome
C. Ischemic heart disease
D. Endocarditis
E. Clubbing after treatment
Functional closure of the DA
24 hrs - 30%
48 hrs - 85%
96 hrs - 100%
NKX2-5 widely involved in ASDs
Left-to-right shunt
Right-to-left shunt
Obstructive malformations
Excessive load and pressure will cause complicated ASD
Imaging
EDICAL DOCTOR
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