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A 10 week old female who presents to the emergency room with the chief complaints of lethargy, poor feeding, and respiratory distress. She was well until 2 weeks prior to presentation when she developed a febrile illness with cough, rhinorrhea, and emesis. She subsequently developed progressive respiratory distress. Her parents report that she sweats a lot on her forehead when feeding.
Her parents have also noted her to be increasingly lethargic, with tachypnea, and retractions.
She is the product of a G3P2, full term, uncomplicated pregnancy. Delivery was unremarkable except for meconium stained fluid. She did well at delivery and in the nursery. Her pediatric follow-up has been poor.
Exam: VS T 36.8, RR 72, HR 160, BP 92/68. Oxygen saturation in room air is 84%. She is a mildly cachetic, acyanotic infant who was pale, lethargic, and tachypneic, with mild to moderate subcostal and intercostal retractions. HEENT exam is unremarkable.
Neck is supple without lymphadenopathy. Her skin is clear with no rashes or other significant skin lesions. Her lungs have scattered crackles with slightly decreased aeration in the left lower lobe. The precordium is mildly active. Her heart is of regular rate and rhythm, with a Grade II/VI holosystolic murmur at the mid lower left sternal border with radiation to the cardiac apex. The S1 is normal and the S2 is prominent. An S4 gallop is noted at the cardiac apex. There are no rubs or valve clicks. Her abdomen is soft, non-distended, and nontender.
The liver edge is palpable 3 to 4 cm below the right costal margin. There are no palpable masses or splenomegaly. Bowel sounds are hypoactive. Her extremities are symmetric and cool, with peripheral pulses 1+/4+ in all extremities with no radial-femoral delay. The capillary refill is 4 to 5 seconds (delayed).
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A chest x-ray shows moderate cardiomegaly with a moderate degree of pulmonary edema. There are no pleural effusions. A 12 lead electrocardiogram shows a sinus tachycardia, normal PR and QTc intervals, and a left axis deviation. Voltage evidence of biventricular hypertrophy is present. No significant Q-waves or ST segment changes are noted. An echocardiogram reveals a large perimembranous ventricular septal defect with non-restrictive left to right shunting. All cardiac chambers are dilated. Left ventricular contractility is at the lower range of normal. There is no pericardial effusion.
She is admitted to the hospital and loaded with digoxin, and also started on diuretics and afterload reduction. Her symptoms improve and she is discharged on 24 calorie/ounce formula due to poor weight gain on standard 20 calorie/ounce formula. She continues to have poor weight gain on higher caloric density formula and continues to have symptoms of heart failure on medical management. She is referred for surgical correction of the ventricular septal defect at 6 months of age.
Heart failure (or congestive heart failure) is defined as the inability of the myocardium to meet the metabolic requirements of the body. This may arise as a consequence of excessive work or volume load imposed on the myocardium, primary alterations in myocardial performance, metabolic derangements, or a combination of these elements. Heart failure leads to a neurohormonal response, which contributes to the symptoms associated with heart failure and increased morbidity and mortality.
In the pediatric age group, the underlying abnormality is often a large left to right intracardiac shunt, most commonly a ventricular septal defect, or an obstructive lesion, such as an aortic coarctation.
In contrast to heart failure in adults, pediatric patients often have normal left ventricular function. Exceptions to this may include patients with myocarditis, dilated cardiomyopathy, ischemia-reperfusion injury following cardiopulmonary bypass, or a congenital coronary artery anomaly.
Heart failure can be classified into 4 functional classes:
1) Volume overload: Large left to right shunts, valvular insufficiency, or systemic arteriovenous fistulae.
2) Pressure overload: Outflow or inflow obstruction.
3) Disorders affecting the inotropic state: Myocarditis, electrolyte disturbances, hypoxia, acidosis, various cardiomyopathies,
coronary artery lesions, endocrine or metabolic derangements, septic shock, toxic shock.
4) Alterations in the chronotropic state: Supraventricular or ventricular tachycardia, complete heart block.
To better understand congestive heart failure in pediatric patients, especially infants, one must have an understanding of the developing heart.
Congenital Ventricular Septal Defect
Congenital Ventricular Septal Defect is more commonly referred to as a common hole in heart. It is a hole between the two lower chambers (ventricles) of the heart. Causes abnormal heart sound or murmur.
Congenital Ventricular Septal Defect occurs as a result of incomplete septation of the ventricles. Embryologically, the interventricular septum (the portion which separates the 2 ventricles) is composed of a membranous and a muscular portion.
Most episodes of the Congenital Ventricular septal defect occurs at the Junction of the membranous and muscular portions.
The Ventricular Septal Defect is the most common congenital cardiac defect, with an occurrence rate of 1 in 500 live births. It may occur in isolation or as part of a complex heart defect, e.g, in Tetralogy of Fallot.
Acquired VSD may be as a result of rupture following Myocardial infarction, though this occurs less commonly.
Most episodes of congenital ventricular septal defects close spontaneously in early life. In children with persistent and large defects, as a result of the higher pressure in the Left ventricle compared to that of the right, blood flows into the right ventricle from the left, during ventricular contraction (systole). This left-right shunt gives rise to pulmonary hypertension.
Clinical Features of VSD
Symptoms of VSD are usually minimal at first and consisting of dyspnoea, recurrent chest infections, poor feeding, prolonged feeding time, and failure to thrive. This symptoms develop in children with large VSD causing cardiac failure.
Such children tend to present with Respiratory distress, decreased SPO2, increased Pulse/Heart rate.
On examination, evidence of an enlarged and displaced apex heart beat with a right heart heave and thrill might be seen.
As a result of the Left-right shunt of blood, there is presence of a pansystolic murmur, heard best at the left sternal edge.
Congenital Ventricular septal defect usually present as heart failure in infants, which usually becomes apparent in the first 10-15 weeks of life.
There is also fast breathing, Intercoastal and sub-costal recession.
Management of VSD
Heart failure, associating VSD in infancy is usually treated with digoxin and diuretics. Small VSD require no specific treatment. Persistent heart failure after management with digoxin and diuretics is an indication for surgical repair.
Doppler’s and 2D Echocardiography can be used for accessing the size and nature of the VSD, in order to predict the defects that are likely to close spontaneously, and those that require surgical repair.
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