CMA

• Congestive Heart Failure (CHF)

  The inability of the heart to supply adequate oxygenated blood to meet the metabolic demands of the body
• Heart failure is a cluster of symptoms and physical examination findings that are secondary to an underlying process
• Most common causes of CHF in children

  • Congenital heart defects that produce an excessive workload on the myocardium
  • Cardiomyopathies due to metabolic disorders, infectious diseases, drugs, Kawasaki disease, and myocardial dysfunction after heart surgery
• Etiology of CHF


  • Inability to meet metabolic demands
  • Decreased filling or obstruction of flow
  • Either one side or both sides of the heart are unable to pump effectively and will eventually fail if the root problem is not corrected
• Assessment of CHF


  • Tachycardia
  • Tachypnea
  • Poor feeding and failure to thrive in infants and young children
  • Older children may complain of fatigue, exercise intolerance, and breathlessness
  • May gain weight from fluid overload
• Therapeutic Management of CHF

  • Symptomatically manage the patient
  • Treat the underlying cause of the heart failure
  • Goal is to decrease any fluid overload, enhance myocardial contractility, and decrease afterload in order to ensure adequate perfusion and decrease the work of the heart
  • Interventions focus on helping support heart function and helping parents deal with this crisis until the child is stabilized and the underlying condition can be treated, if possible
• Acyanotic Heart Disease


  The blood shunts left to right, then oxygenated blood from the left side mixes with blood in the right side of the heart and goes back to the lungs again
• Cyanotic Heart Disease


  When venous blood from the right side of the heart mixes with blood on the left side, this is a "right-to-left" shunt that delivers deoxygenated blood to the body
• Patent Ductus Arteriosus (PDA)


  Occurs when the ductus arteriousus fails to close after several days of life, occurs more frequently in children born prematurely
• Assessment of PDA


  • Produces a systolic murmur early in life and a continuous murmur as the child ages at the second intercostal space, left upper sternal border, or out to the left clavicular area
  • If the defect is largely significant that can cause rales, congestion, increased work of breathing, difficulty feeding, or failure to thrive
• Management of PDA


  • Diuretics (Furosemide) for the symptoms of the increased blood flow
  • If the child is experiencing significant manifestations of the increased pulmonary blood flow or if left heart dilation is noted from the extra blood flow returning to the left side of the heart, the child will be referred for PDA closure
  • Indomethacin, a nonsteroidal anti-inflammatory and prostaglandin inhibitor, can be utilized to facilitate closure
• Transposition of Great Arteries (TGA)

  The second most common congenital defect and is described as reversal of the great arteries - the aorta coming off of the right ventricle and the pulmonary artery arising from the left ventricle
• Assessment of TGA


  • Transposition of the great arteries is a cyanotic defect noted immediately after birth
  • Tachypneic, and a murmur may not be noted
  • Prostaglandin E1 (PGE1) infusion is started immediately after birth to maintain patency of the ductus arteriosus and encourage mixing of blood
  • Side effects: apnea and hypotension
  • Best for the child to remain on room air, spontaneously breathing
  • Echocardiogram will confirm this diagnosis and will also delineate the coronary artery anatomy
• Management of TGA


  • Requires surgical correction within the first 14 days of life to ensure the most positive outcomes
  • Arterial switch or Jatene procedure - dissecting both the pulmonary artery and aorta above their respective valves and switching the vessels to the appropriate location
  • Before surgery includes ensuring their oxygen saturation remains normal for this defect, which is 75% to 85%; monitoring for signs of increased pulmonary blood flow; and supporting the family as they wait for their newborn to undergo heart surgery
• Atrial Septal Defect (ASD)


  Is created when a portion of the atrial septal tissue does not completely form, an acyanotic defect that allows blood to flow from the high-pressure left atrium to the low-pressure right atrium and continue out to the lungs
• Types of ASD


  • Secundum - located in the center of the atrial septum
  • Primum - found low in the atrial septum near the IVC
  • Sinus Venosus -allow communication of one or more of the pulmonary veins with the right atrium
• Assessment of ASD


  • If the defect is small, it may go undetected or may cause no clinical concern and require no Intervention
  • If the defect is large enough- pulmonary overcirculation, such as rales, congestion, tiring with activity, or poor weight gain
  • Murmurs are not associated with the defect but is related to the relative increased blood volume traveling across the pulmonary valve
• Management of ASD


  • If ASD is less than 5 mm, and is producing no clinical symptoms, it will not be closed
  • With clinically significant defect diuretics will be attempted first in an effort to allow the defect to close spontaneously
  • 5 mm or greater with persistent shunting after an observation period should be referred for closure to decrease the incidence of supraventricular dysrhythmias and prevent pulmonary vascular disease
  • If the defect is 8 mm or larger with evidence of increased pulmonary blood flow, the child will be referred for closure immediately
  • Secundum ASD is typically closed in the catheterization lab with a device that sits in the defect space
  • Sinus venosus and primum defects referred for surgical closure
  • Surgical closure involves a median sternotomy incision and cardiopulmonary bypass
• Truncus Arteriosus (TA)


  Is characterized by a single arterial vessel that originates from the heart, overrides the ventricular septum, and supplies all of the systemic, coronary, and pulmonary blood flow
• Truncus Arteriosus occurs as a result of the great artery failing to divide in utero into two vessels, the pulmonary artery and the aorta
• Truncus Arteriosus requires urgent surgery
• Ventricular Septal Defect (VSD)


  The most common defect found in children, either in isolation or combined with other defects, occurs when a portion of the ventricular septum does not completely close
• Assessment of VSD


  • Holosystolic murmur noted at the left lower sternal border
  • Symptoms associated with a VSD are similar to those found with a PDA or ASD and are determined by the size of the defect
• Management of VSD


  • Children with an audible murmur but no symptoms of pulmonary overcirculation and may be very small defects that will close spontaneously needs no medical management
  • If the child exhibits signs of pulmonary overload diuretic such as Furosemide is given
  • If closure does not occur, surgical closure can be done
• Atrioventricular Septal Defect (AVSD)


  Comprises several congenital heart defects: a primum ASD, a high VSD, and failure of the tricuspid and mitral valves to develop and attach correctly
• Complete AVSD also allows for right-to-left shunting, which may lead to desaturation, and is associated with Trisomy 21
• Assessment of AVSD


  • Can present with symptoms of CHF similar to those seen in other defects that increase pulmonary blood flow
  • Diagnosis confirmed with echocardiography
  • A cardiac catheterization may be performed before surgical correction to measure pulmonary pressures and confirm reactivity with the administration of oxygen or nitric oxide, both of which should cause pulmonary pressures to decrease
• Management of AVSD


  • Medications such as furosemide, digoxin, and an ACE inhibitor like captopril or enalapril
  • Children may also require concentrated feeds to help maintain weight
  • Children with Down Syndrome they are typically referred for surgical correction by 3 months of age or earlier
  • Children without Down Syndrome are referred for repair by 5 or 6 months of age or as clinically necessary
  • Pulmonary artery band, which is a band surgically placed around the pulmonary artery that constricts it to increase the resistance within the pulmonary artery
  • Surgical repair of an AVSD consists of closing atrial and VSDs and repairing the mitral and tricuspid valves to make them functional
• Coarctation of Aorta


  Typically causes a discrete narrowing of the aorta, occurs more commonly in males than in females
• Assessment of Coarctation of Aorta

  • The narrowed area is most frequently distal to the right subclavian artery, so the pressures before that area increase and refer out these vessels causes elevated blood pressures to be noted in the right arm
  • Can cause a systolic murmur heard along the left sternal border and the left midscapular area
  • The narrowing increases the resistance to the left ventricle and can lead to left ventricular hypertension and hypertrophy over time
  • Coarctation noted in infancy is usually more critical and can produce heart failure within weeks of birth that require immediate surgical intervention
  • Check radial and femoral pulses concurrently - normal should be no absence or weakness in the femoral pulses compared with the radial or brachial pulse
  • Systolic pressure reading in the right upper extremity is 10 mmHg higher than that in the lower extremity
• Management of Coarctation of Aorta

  • Echocardiographic evidence of a significant gradient across the narrowing, or any signs of left ventricular hypertrophy due to the increased resistance, are indicators for correction either by balloon angioplasty in the cardiac catheterization lab or by surgical intervention
  • CoAo is surgically repaired through a left thoracotomy incision and with end-to-end anastomosis
  • Future interventions with balloon dilation or stent placement are typically performed in the catheterization lab
• Hypoplastic Left Heart Syndrome (HLHS)


  A rare disorder, accounting for only 1% to 3% of congenital heart disease, and is detectable on prenatal ultrasound, there is poor or no flow to the left ventricle, typically secondary to mitral or aortic stenosis or atresia, the ascending aorta is also hypoplastic, and the coronary arteries are perfused through retrograde flow down the ascending aorta
• Assessment of HLHS


  • In utero, the circulation is adequate to meet the needs of the developing fetus. But at birth, survival is dependent on a PDA
  • Infants are typically male, rarely premature, and have no other associated cardiac anomalies
  • Has normal right-sided flow out to the lungs
  • These children are at high risk for sudden death due to dysrhythmias, inability to fight infection, or metabolic imbalance
• Surgical Management of HLHS

  • Norwood Procedure - Dissection of the main pulmonary artery from the branch pulmonary arteries, which is then attached to the hypoplastic ascending aorta so there is one great vessel taking blood out of the heart (right ventricle) to the body and providing blood flow to the coronary arteries
  • Shunt placement, typically either a BT(Blalock-Taussig) shunt or a Sano shunt
  • Ligation of the PDA
  • Ensuring a widely patent atrial septum
• Subsequent Surgical Procedures for HLHS

  • Bidirectional Glenn Shunt - Ligation of the previously placed BT or Sano shunt, Dissection of the SVC at its insertion to the right atrium and attachment of the SVC directly to the branch pulmonary artery
  • Fontan Procedure - The IVC is dissected from the right atrium, and flow from there is directed through an artificial conduit directly to the branch pulmonary artery
• Cardiac transplantation is an alternative form of therapy, but the availability of donor hearts, rejection problems, and the need for retransplantation are a secondary choice for most infants
• Aortic Stenosis


  Obstruction out the left ventricle can occur below the aortic valve (subvalvar), at the valve (valvar), or above the valve (supravalvar), most common location for obstruction is valvar aortic stenosis, which is typically associated with an abnormal, bicuspid aortic valve
• Assessment of Aortic Stenosis

  • Systolic heart murmur at the right second intercostal space, but asymptomatic
  • Severe obstruction may experience chest pain with exercise
  • Aortic regurgitation is frequently associated with stenosis, a diastolic murmur may also be noted
  • ECG may note left ventricular hypertrophy if the stenosis has been ongoing
  • Echocardiography will determine the exact location and severity of the narrowing as well as any impact on ventricular size and function
• Management of Aortic Stenosis


  • Stabilization with a beta-blocker or a calcium channel blocker may be necessary to reduce or prevent further ventricular hypertrophy
  • Routine echocardiograms and possibly cardiac catheterizations to best quantify the pressure gradient across the valve
  • Surgical intervention may be necessary for recurrent stenosis or ineffective balloon dilation
• Enuresis


  Involuntary passage of urine past the age when a child should be expected to have attained bladder control