Pediatric Cardiovascular Disorders
CARDIAC PROCEDURES
Cardiac Catheterization
Cardiac catheterization is an invasive procedure used to identify cardiac anatomy; measure intracardiac pressures, shunts, and oxygen saturations; and calculate systemic and pulmonary vascular resistance.
Procedure
Catheter insertion sites include femoral vein or artery, umbilical vein or artery, brachial vein, or internal jugular vein.
Under fluoroscopy, catheters are guided through the heart, collecting pressure measurements and oxygen saturations.
Contrast dye is injected through the catheters to visualize blood flow patterns and structural abnormalities.
Cardiac catheterization is usually an outpatient procedure for children who undergo an elective procedure. After interventional procedures, some children are observed in the hospital for 12 to 24 hours.
Indications
To confirm or establish the diagnosis.
To measure cardiac output.
To measure pressures and oxygen saturations.
To calculate intracardiac shunting and pulmonary and systemic vascular resistance.
To visualize coronary arteries.
To assess for myocarditis or rejection following heart transplantation.
To intervene in congenital heart disease (see Box 45-1 for definitions of abbreviations):
Balloon atrial septostomy (Rashkind) for restrictive atrial septum.
Balloon valvuloplasty (arterial stenosis [AS], pulmonary stenosis [PS]) and angioplasty (recurrent coarctation of the aorta [CoA]).
Endomyocardial biopsy.
To occlude vessels (coil embolization) or defects (atrial septal defect [ASD], ventricular septal defect [VSD], or patent ductus arteriosus [PDA] closure devices).
To stent vessels open (branch pulmonary artery [PA] stenosis, recurrent CoA).
To dilate stenotic valves (mitral valve, pulmonary valve, tricuspid valve).
Complications
Arrhythmias (usually catheter induced).
Infection.
Bleeding at catheter insertion site; large hematoma.
Allergic reaction to contrast material.
Loss of pulse in the extremity used for cannulation.
Perforation of heart or vessels.
Stroke.
Dislodgment of coils, closure devices, or stents.
Death.
BOX 45-1 Abbreviations Used for Congenital Heart Disease | ||||||||||||||||||||||||||||||||||||||
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Nursing Diagnoses
Preoperative
Fear related to surgical procedure.
Deficient Knowledge regarding surgical procedure and associated nursing care.
Postoperative
Risk for Injury related to complications of cardiac catheterization.
Nursing Interventions
Reducing Fear in Child and Parents
Provide specific instructions in nonthreatening manner:
Day and time of the procedure.
Nothing-by-mouth (NPO) guidelines.
Sedation versus general anesthesia.
Site of the planned arterial and venous puncture.
Routine postprocedure care.
Provide appropriate teaching geared toward the child’s age and level of cognitive development. Use diagrams and models, as appropriate.
Provide parents an opportunity, without the child present, to discuss the procedure, risks, benefits, and alternative choices.
Give child opportunity to express fears and ask questions.
Provide tour of the cardiac catheterization laboratory, if appropriate.
Explaining and Providing Nursing Care
Obtain baseline set of vital signs: heart rate, BP, respiratory rate, and oxygen saturation.
Measure and record child’s height and weight.
Note time of last oral intake: solids and liquids.
Identify known allergies.
List current medications and note time last taken.
Help child change into a hospital gown.
Start peripheral IV, as needed.
Administer sedation, as prescribed.
Assess and mark the location of pulses (dorsalis pedis, posterior tibial).
Observe for and Prevent Complications
Monitor and record routine vital signs (q 15 min × 4, q 30 min × 2, then q 1 hr); extremity temperature, color, and pulse check with vital signs.
Notify health care provider for:
Heart rate, respiratory rate, or BP outside normal parameters for age.
Bleeding or increasing hematoma at puncture site.
Change in oxygen saturations.
Fever.
Cool, pulseless extremity.
Observe puncture site for redness, pain, swelling, or induration.
Maintain the child in a reclining position for 4 to 6 hours after the procedure.
Offer fluids as soon as the child is ready.
Family Education and Health Maintenance
Provide discharge information:
Care of incision or puncture site (keep dry for 48 hours).
Activity restrictions (usually for 48 hours, but may be up to 2 weeks following placement of closure devices).
Observe for and report late complications: redness, swelling, drainage from puncture site.
Follow-up medical care.
Reinforce infective endocarditis precautions.
If cardiac catheterization was a preoperative procedure, use the recovery time to teach the child and family about upcoming hospital stay.
Evaluation: Expected Outcomes
Child describes procedure in own words; parents and child discuss procedure and ask appropriate questions.
Child cooperative with preoperative nursing care.
Insertion site intact without drainage, redness, or hematoma.
Cardiac Surgery
The ultimate goal of treatment of cardiovascular disease in children is to restore normal heart structure and function. Most types of CHDs can be palliated or definitively repaired.
Procedures
Closed-Heart Surgery
Surgical approach: lateral thoracotomy or mediastinal incision.
Indications:
PDA ligation.
PA banding.
CoA repair.
Vascular ring repair.
Blalock-Taussig shunt (BT shunt) placement.
Occasionally, Glenn and Fontan procedures.
Open-Heart Surgery
Surgery is done through a mediastinal incision.
With the use of cardiopulmonary bypass, the surgeon can stop the heart and operate inside to repair the defects.
Deep hypothermia with circulatory arrest allows the surgeon to safely stop cardiopulmonary bypass and remove arterial or venous cannulas to better visualize and repair the defects.
Indications:
ASD, VSD, atrioventricular (AV) canal defect.
Aortic stenosis, pulmonary stenosis.
Tetralogy of Fallot (TOF).
Transposition of great arteries (TGA).
Tricuspid atresia.
Total anomalous pulmonary venous return (TAPVR) or partial APVR.
Truncus arteriosus.
Hypoplastic left heart syndrome (HLHS).
Complex single ventricle.
Potential Complications of Specific Surgeries
PDA ligation: laryngeal nerve damage, phrenic nerve damage, diaphragm paralysis, thoracic duct injury.
CoA: rebound hypertension, mesenteric arteritis (abdominal pain), coarctation restenosis.
Aorta-pulmonary shunt (BT shunt): shunt occlusion, PA distortion, pulmonary overcirculation.
ASD: atrial arrhythmias, sinoatrial node dysfunction.
VSD: transient or permanent heart block, residual VSD, ventricular dysfunction.
TOF: low cardiac output, residual right ventricular outflow tract obstruction (RVOTO) or VSD, ectopic junctional tachycardia or arrhythmias, thoracic duct injury.
TGA: arterial switch operation—coronary artery injury, ventricular dysfunction, suprapulmonary stenosis.
TGA: atrial switch operation—baffle obstruction, RV failure, atrial arrhythmias.
Valvotomy (for valve stenosis): valve insufficiency.
Bidirectional Glenn shunt: superior vena cava (SVC) syndrome, low cardiac output, hypoxia, pleural effusions.
Fontan completion: low cardiac output, pleural effusions, ventricular dysfunction, thrombus formation, arrhythmias, hepatic dysfunction.
Cardiac Transplant Surgery
Cardiac transplantation is a treatment option for children with progressive congestive heart failure (CHF) or certain cardiac diseases not amenable to conventional medical-surgical therapy. Children who cannot grow and meet developmental milestones or who have unacceptable quality-of-life issues may benefit from cardiac transplant surgery. However, approximately one in four children die while waiting for an organ donor. Those children who receive a donor heart must take lifelong immunosuppression medications to prevent organ rejection.
Indications for Cardiac Transplantation
End-stage cardiomyopathy.
Untreatable complex CHD.
Malignant arrhythmia.
Retransplant for cardiac graft failure.
Immunosuppressive Medications
Evidence BaseDenfield, S. W. (2010). Strategies to prevent cellular rejection in pediatric heart transplant recipients. Paediatric Drugs, 12(6), 391-403.
Immunosuppressive therapy is divided into two phases, induction and maintenance. Induction therapies reduce early rejection and may help to reduce the child’s overall exposure to corticosteroids. Combination therapy is usually used in the maintenance phase to ensure ongoing protection against rejection.
Phase I: Induction.
Polyclonal antibodies.
Rabbit or equine antithymocyte globulin.
Interlukin-2 receptor antibodies.
Daclizumab.
Basiliximab.
Phase II: Maintenance therapies.
Calcineurin inhibitors.
Ciclosporin (cyclosporine).
Tacrolimus.
Antiproliferative agent.
Azathioprine.
Mycophenolate mofetil.
Mammalian target of rapamycin inhibitors.
Sirolimus.
Everolimus.
Corticosteroids.
Prednisone.
Complications
Organ rejection: routine surveillance endomyocardial biopsies are performed to assess for rejection.
With mild to moderate rejection, children may initially be asymptomatic.
With severe rejection, children are usually symptomatic with hemodynamic instability.
Infection.
Adverse effects of immunosuppressive agents.
Polyclonal antibodies—fever, chills, rash, headaches, abdominal pain, leukopenia, and thrombocytopenia
Calcineurin inhibitors.
Ciclosporin—nephrotoxicity, hypertension, diabetes mellitus, hirsutism, gingival hyperplasia, tremor.
Tacrolimus—nephrotoxicity, diabetes mellitus, hypertension, hyperkalemia, hypomagnesemia, tremor.
Antiproliferative agent.
Azathioprine—leukopenia, thrombocytopenia, nausea, vomiting.
Mycophenolate mofetil—leukopenia, nausea, vomiting, diarrhea, anorexia.
Mammalian target of rapamycin inhibitors—hyperlipidemia, thrombocytopenia, anemia, leukopenia.
Prednisone—hypertension, Cushing syndrome, growth retardation.
Accelerated diffuse coronary artery disease.
Posttransplant lymphoproliferative disease.
Routine Follow-Up
Routine well-child care visits to primary care provider.
Transplant children should not receive any live virus immunizations (oral polio, measles-mumps-rubella, varicella). Monitor for adverse effects of chronic steroids and immunosuppressive medications.
Routine cardiology clinic visits.
Laboratory studies: chemistry, hematology, therapeutic drug levels.
Vital signs.
Electrocardiogram (ECG).
Echocardiography.
Serial cardiac catheterizations and endomyocardial biopsies.
Yearly coronary angiography or dobutamine stress echocardiography.
Nursing Assessment
Baseline Assessment on Day of Surgery
Measure and record height and weight.
Document vital signs: heart rate, respiratory rate, BP, and oxygen saturation.
Assess for preoperative infection: fever, signs of upper respiratory infection (cough, runny nose, crackles), vomiting, or diarrhea might necessitate delaying surgery.
Document last oral intake.
Void on call to the operating room.
Nursing Diagnoses
Preoperative
Fear related to surgical procedure.
Postoperative
Risk for Injury related to complications of surgery.
Impaired Adjustment related to postsurgery care.
Nursing Interventions
Preparing the Child and Family and Reducing Fear
Be honest and use nonthreatening language the child can understand.
The following are frequently asked questions from parents. Address them with the parents:
What to tell the child.
When to tell the child.
What to bring to the hospital.
Anticipated hospital course: how long in the operating room; how many days in the intensive care unit (ICU); how many days on the general care unit; visiting policy, rooming-in accommodations.
Review preoperative instructions.
NPO guidelines.
Where to report on the day of the surgery.
Time to arrive at the hospital; time of surgery.
Preoperative medications (injection, liquid, inhalation).
What the operating room looks like; what the people wear in the operating room (hats, gowns, and masks).
Explain the preoperative period.
Change into a hospital gown.
Parents stay with the child.
Transportation to the operating room (walking, wheelchair, or stretcher).
Explain the operative period.
Operating room waiting room.
Updates during the surgery will come from the surgical scrub nurses.
Surgeon will meet the family after surgery to review the surgical findings and to describe the operation.
Explain the postoperative period. Use models and diagrams.
Pediatric ICU routines and procedures.
Monitoring lines and equipment.
Ventilators, oxygen therapy.
Protective restraints.
Offer medical equipment to handle and play with (ECG leads, face mask, BP cuff), age-appropriate books about surgery and hospital stays, and tour of ICU and surgical area, as available.
Prehospital tour of the pediatric ICU and general pediatric care unit.
Allow an opportunity for the child and family to ask questions, express their concern, or to ask for more detail.
Observing for and Preventing Complications
Assess respiratory status and maintain respiratory support.
Maintain ventilatory support, as needed.
Maintain patent airway with routine endotracheal suctioning.
Auscultate breath sounds frequently. Decreased breath sounds may indicate pleural effusions, atelectasis, pneumothorax, hemothorax.
Report results of routine chest x-ray.
Perform frequent position changes: side-back-side.
Monitor arterial blood gas levels and oxygen saturations.
Assess chest tube drainage.
Extubate when hemodynamically stable and when patient meets extubation criteria.
Administer oxygen therapy, as needed, after extubation.
Assess cardiac status.
Monitor vital signs and oxygen saturations.
Maintain continuous ECG monitoring.
Daily 12-lead ECG to assess rhythm.
Temporary epicardial pacemaker wires available for pacing, as needed.
Continuous BP monitoring (arterial line).
Monitor intracardiac pressures (central venous pressure [CVP], left atrial [LA], PA).
Monitor peripheral perfusion, capillary refill, toe temperature.
Titrate vasopressors (dopamine, dobutamine, milrinone), as prescribed.
Assess fluid status.
Record hourly intake (IV fluids, blood products, fluid boluses).
Record hourly output (urine, chest tube drainage, nasogastric drainage).
Perform daily weights.
Administer diuretics, as prescribed.
Assess neurologic status.
Monitor level of responsiveness, response to verbal commands, and response to pain.
Check pupil size and reactivity to light.
Document movement of all extremities.
Monitor all invasive lines for air bubbles and potential air embolism.
Observe for signs of neurologic injury related to hypoperfusion or embolism.
Monitor for potential specific complications related to particular surgery for CHD.
Assess for postoperative pain.
Monitor level of responsiveness, agitation.
Implement pediatric pain scale rating to assist the child in identifying the severity of pain.
Administer pain medication, as prescribed: continuous IV infusion and IV boluses, as necessary.
Utilize patient-controlled analgesia continuous pump, as appropriate.
Assess serum electrolyte balance. Obtain blood tests and report results. Treat deficits with supplements.
Assess packed cell volume, platelet count, and coagulation studies.
Low hematocrit (less than 30): consider directed donor transfusion or blood bank transfusion.
Low platelet count: continue to monitor; if bleeding persists, transfuse platelets.
Prolonged coagulation studies: continue to monitor; if bleeding or oozing persists, give protamine, fresh frozen plasma.
Enhancing Adjustment Postoperatively
Provide continuity of care (primary nursing and consistent medical team).
Explain all procedures and routines to the child and parents to minimize fear.
Explain to the parents a child’s typical reactions to stressful events.
Regression—temporary loss of developmental milestones.
Fear of any medical personnel (white coat anxiety).
Sibling jealousy that one child is receiving a lot of attention.
Withdrawal—related to stimulation overload and lack of undisturbed sleep.
Nightmares.
Increased dependency; clinging behavior.
Suggest parent and patient support groups, Child Life Therapy, community resources, and counseling, as needed.
Family Education and Health Maintenance
Provide the child and family with oral and written discharge instructions and recommendations.
Medications.
Activity restrictions.
No strenuous activity or contact sports for 6 to 8 weeks after surgery.
Most children are ready to return to school at least part time about 2 weeks after surgery.
If child needs prolonged recovery time at home, may need to consider home tutoring.
No gym class until full recovery.
Care of the incision.
Dietary recommendations.
Bathing or showering guidelines.
Provide the child and family with a list of potential signs of complications and instructions to notify the health care provider.
Fever greater than or equal to 101.5° F (38.6° C).
Any redness, swelling, or drainage from chest incision.
Partial opening of the chest incision.
Poor appetite, nausea, vomiting.
Breathing difficulties, shortness of breath.
Provide the family with the names and phone numbers of people to call for questions and emergencies.
Make follow-up appointments for the child to be seen by his or her primary care provider 2 to 3 days after discharge from the hospital and 10 to 14 days to be seen by their pediatric cardiologist.
Review American Heart Association’s recommendations for infective endocarditis prophylaxis: standard general prophylaxis for children at risk: amoxicillin 50 mg/kg (maximum dose = 2 g) given orally 1 hour before the procedure.
Community and Home Care Considerations
Arrange skilled home nursing visits, as needed, to:
Review medications.
Assess wound healing.
Monitor vital signs and oxygen saturations.
Assess oral intake; nutritional supplements.
Resource for family.
Make referral to community agencies, as needed (infant and toddler program).
Arrange for home medical equipment (oxygen, feeding pump, and supplies), as needed.
Review safety precautions in the home:
Childproof medication bottles.
Poison control phone number for accidental medication overdose.
Infant and child CPR techniques.
Bleeding precautions for children on anticoagulation therapy. No aspirin or ibuprofen products. Be sure to read the labels on over-the-counter cold and cough syrups. Avoid activities with high risk of injury. All head injuries need to be evaluated by a physician. Signs of bleeding:
Blood in the urine.
Black tarry stools.
Prolonged nosebleeds.
Bleeding gums.
Bruising for no known trauma.
Spitting or coughing up blood.
Any unusual swelling or pain.
MedicAlert bracelet or tags.
Discuss developmental issues with the family.
A child on diuretics may have difficulty with toilet training.
Disciplining, establishing behavioral expectations, and setting limits in a child with CHD should be similar to those for a child without CHD.
Discuss the need for home schooling or tutoring during recovery time. Return to the classroom as soon as the child is ready.
Discuss infants with CHD in daycare situations—address each case individually. Daycare programs usually have increased risk of URIs and other communicable diseases.
Encourage routine dental visits to prevent dental caries (dental caries predispose to bacteremia and endocarditis).
Encourage heart-healthy eating and exercise routines.
Encourage age-appropriate activities. A few children will need exercise restrictions. Children with CHD should be allowed to participate in activities with rest periods, as needed. Children with pacemakers and children on anticoagulation therapy should refrain from contact sports.
Maintain standard childhood immunization schedule. Delay vaccines around the perioperative time until fully recovered from surgery (no immunizations for 6 weeks postoperatively).
Encourage yearly influenza vaccine for children with unrepaired or complex CHD.
Encourage respiratory syncytial virus (RSV) immunization for children younger than age 2 with complex CHD and those at risk of CHF or pulmonary hypertension.
Evaluation: Expected Outcomes
Child describes procedure without fear; parents and child discuss procedure and ask appropriate questions.
Vital signs stable, no signs of infection.
Parents offer support to child.
CONGENITAL HEART DISEASE
Evidence BaseDolbec, K., & Mick, N. W. (2011). Congenital heart disease. Emergency Medical Clinics of North America, 29(4), 811-827.
Congenital heart disease (or defects) (CHD) is one of the most common forms of congenital anomalies. It involves the chambers, valves, and vessels arising from the heart (see Figure 45-1, page 1526).
In most cases, the cause of CHD is not known. Some infants and children with CHD may appear perfectly healthy, whereas others may be critically ill. Most infants and children with CHD can be successfully managed with medications and surgeries.
Etiology and Incidence
CHD affects 8 to 12 of every 1,000 neonates.
Exact cause of CHD is unknown in 90% of cases.
The heart begins as a single cell and develops into a fourchambered pumping system during the 3rd to 8th weeks of gestation.
Associated factors for CHD include:
Fetal or maternal infection during the first trimester (rubella).
Chromosomal abnormalities (trisomy 21, 18, 13).
Maternal insulin-dependent diabetes.
Systemic lupus erythematosus.
Teratogenic effects of drugs and alcohol.
Lithium—Ebstein anomaly.
Phenytoin—aortic and pulmonic stenosis.
Alcohol—ASD and VSD.
DRUG ALERT Teratogenic drugs also include amphetamines, estrogen, progesterone, retinoic acid, selective serotonin reuptake inhibitors, trimethadione, valproic acid.
Syndromes that include CHD:
Marfan’s syndrome—mitral valve prolapse (MVP), dilated aortic root.
Turner’s syndrome—aortic valve stenosis (AVS), CoA.
Noonan’s syndrome—dysplastic pulmonary valve.
William’s syndrome—supravalvular PS.
DiGeorge syndrome—interrupted aortic arch (IAA), truncus arteriosus, TGA, TOF.
Down syndrome (trisomy 21)—AV canal defect, VSD. About 50% of children with Down syndrome have a CHD.
 Figure 45-1 Congenital heart abnormalities. |
Common Congenital Heart Malformations
Congenital heart defects can be classified into three categories: obstruction to blood flow, increased pulmonary blood flow (acyanotic lesions), and decreased pulmonary blood flow (cyanotic lesions).
Obstructive lesions:
AS—valvular, subvalvular, or supravalvular.
CoA.
PS—valvular, subvalvular, or supravalvular.
IAA.
Increased pulmonary blood flow (acyanotic):
PDA.
ASD.
VSD.
AV canal.
PAPVR.
Decreased pulmonary blood flow (cyanotic):
TOF.
TA.
TAPVR.
TGA.
Truncus arteriosus.
HLHS.
DORV.
Aortic Stenosis
Evidence BaseLoscalzo, M. L. (2010). Left outflow tract anomalies in children. Current Opinion in Pediatrics, 22(5), 593-597. Schnieder, D. J., & Moore, J. W. (2013). Aortic stenosis. In H. D. Allen, D. J. Driscoll, R. E. Shaddy, & T. F. Feltes, (Eds.), Moss and Adams’ heart disease in infants, children, and adolescents (8th ed.), (pp. 1023-1043). Philadelphia: Lippincott Williams & Wilkins.
Congenital aortic stenosis (AS) may be caused by a bicuspid aortic valve with fused commissures that does not open completely, by a hypoplastic aortic valve annulus, or by stenosis above or below the aortic valve (subvalvular or supravalvular stenosis). The result is turbulent blood flow across the aortic valve and into the ascending aorta. Patients with AS must be evaluated for additional left heart lesions that include CoA, mitral valve stenosis, hypertrophic cardiomyopathy, and hypoplastic left heart. AS is the most common form of left ventricular outflow tract obstruction (LVOTO). It accounts for 3% to 6% of CHDs. AS may occur at any age, and it occurs more commonly in boys than in girls (4:1 ratio). In most children, it is a progressive lesion that creates LVOTO.
Pathophysiology and Etiology
Blood flows at an increased velocity across the obstructive valve or stenotic area and into the aorta.
During systole, left ventricular pressure rises dramatically to overcome the increased resistance at the aortic valve.
Myocardial ischemia may occur because of an imbalance between the increased oxygen requirements related to the hypertrophied left ventricle (LV) and the amount of oxygen that can be supplied.
Left-sided heart failure results in an increased LV end diastolic pressure that is reflected back to the left atrium and pulmonary veins.
Clinical Manifestations
Neonate
Severe CHF.
Metabolic acidosis.
Tachypnea.
Faint peripheral pulses, poor perfusion, poor capillary refill, cool skin.
Poor feeding and feeding intolerance.
Child and Adolescent
Chest pain on exertion, decreased exercise tolerance.
Dyspnea, fatigue, shortness of breath.
Syncope, light-headedness.
Palpitations.
Sudden death.
Diagnostic Evaluation
Auscultation.
Systolic ejection murmur heard best at right upper sternal border, radiates to neck.
Ejection click. May be absent with severe stenosis.
S2 splits normally or narrowly.
Four-limb blood pressure.
Systolic blood pressure normally 5 to 10 mm higher in lower extremities.
Systolic blood pressure in lower limbs the same as or lower than that in the arms seen in coarctation of the aorta.
Palpation.
Thrill may be palpable in right upper sternal border.
ECG: left ventricular hypertrophy (LVH) with a strain pattern may be seen in severe cases.
Chest x-ray: increased cardiac silhouette, increased pulmonary vascular markings. A prominent aortic knob may be seen occasionally from poststenotic dilatation with valvular AS.
Echocardiogram: two-dimensional echocardiogram with Doppler study and color flow mapping to visualize the anatomy and to estimate the gradient across the valve and through the aorta.
Management
Neonate
Stabilize with prostaglandin E1 (PGE1) infusion to maintain cardiac output through the PDA.
Inotropic support, as needed.
Intubation and ventilation, as needed.
Infective endocarditis prophylaxis (lifelong).
Cardiac catheterization: aortic balloon valvuloplasty or aortic balloon angioplasty.
Surgical valvotomy, commissurotomy, or myectomy/myotomy.
Child and Adolescent
Medical management with close follow-up to monitor increasing gradient across the aortic valve or through the aorta.
Restrict strenuous exercise and anaerobic exercise (eg, weight lifting).
Restrict participation in competitive sports.
Aortic balloon valvuloplasty or aortic balloon angioplasty.
Infective endocarditis prophylaxis (lifelong).
Surgical intervention.
Surgical valvotomy, commissurotomy, or myectomy/myotomy.
Aortic valve replacement.
Mechanical prosthesis (St. Jude valve).
Ross procedure (pulmonary autograft).
Complications
CHF and pulmonary edema.
Dizziness, light-headedness, and syncope.
Palpitations, arrhythmias.
Infective endocarditis.
Sudden death.
Coarctation of the Aorta
Evidence BaseBeekerman, R. H. (2013). Coarctation of the Aorta. In H. D. Allen, D. J. Driscoll, R. E. Shaddy, & T. F. Feltes, (Eds.), Moss and Adams’ heart disease in infants, children, and adolescents (8th ed.). Philadelphia: Lippincott Williams & Wilkins.
Kenny, D., & Hijazi, Z. M. (2011). Coarctation of the aorta: From fetal life to adulthood. Cardiology Journal, 18(5), 487-495.
Coarctation of the aorta (CoA) is a discrete narrowing or a long segment hypoplasia of the aortic arch, usually in the juxtaductal position. It accounts for 8% to 10% of CHDs and is two to five times more common in males. Thirty percent of infants with Turner syndrome have CoA. Commonly associated defects include VSD, mitral valve stenosis, and bicuspid aortic valve.
Pathophysiology and Etiology
The discrete narrowing or hypoplastic segment of the aorta increases the workload of the left ventricle (increased LV systolic pressure).
In a neonate with critical CoA, lower-body blood flow occurs through the PDA (right-to-left shunting).
In the older child, collateral vessels grow and bypass the coarctation to perfuse the lower body.
Clinical Manifestations
The neonate with critical CoA (ductal dependent lesion):
Asymptomatic until the PDA begins to close.
After PDA closure: severe CHF, poor lower body perfusion, tachypnea, acidosis, progressive circulatory shock, absent femoral and pedal pulses.
The child or adolescent with CoA:
Usually asymptomatic—normal growth and development.
Hypertension in the upper extremities, with absent or weak femoral pulses.
Nosebleeds, headaches, leg cramps.
Diagnostic Evaluation
Auscultation—varies; nonspecific systolic ejection murmur. Murmur may be heard over the back or spinous processes. Single S2 loud S3 gallop is usually present.
Four-limb blood pressure.
Systolic blood pressure normally 5 to 10 mm higher in lower extremities
Systolic blood pressure in lower limbs the same as or lower than that in the arms seen in CoA.
Chest x-ray—cardiomegaly and pulmonary edema or pulmonary venous congestion.
ECG—varies; normal or right ventricular hypertrophy (RVH) in infants and LVH in older children.
Two-dimensional echocardiography with Doppler and color flow mapping identifies area of aortic arch narrowing and associated lesions (bicuspid aortic valve, VSD, PDA).
Invasive studies (cardiac catheterization) usually not needed to make the initial diagnosis; may need aortic angiography to identify collateral vessels before surgery.
Cardiac magnetic resonance imaging may be done to noninvasively assess the location and degree of narrowing and identify collateral vessels.
Management
Critical Coarctation in the Neonate
Medical management:
Resuscitation and stabilization with PGE1 infusion: monitor for complications related to PGE1 therapy (fever, apnea).
Intubation and ventilation, as needed.
Infective endocarditis prophylaxis (lifelong).
Anticongestive therapy (digoxin and Lasix) and inotropic support, as needed.
Assessment of renal, hepatic, and neurologic function.
Balloon angioplasty may be indicated for infants who are at high surgical risk.
Surgical intervention: usually performed as soon as the diagnosis is made.
Subclavian flap repair (Waldhausen procedure).
End-to-end anastomosis.
Dacron patch repair.
Stent implantation.
Coarctation in the Child or Adolescent
Recurrent Coarctation in the Neonate or Child
Balloon angioplasty in the cardiac catheterization laboratory.
Redo surgical intervention.
Complications
Systemic hypertension.
CHF—occurs in 20% to 30% of all infants with CoA by age 3 months.
Cerebral hemorrhage.
Infective endocarditis.
Left ventricular failure.
Aortic aneurysm.
Pulmonary Stenosis
Evidence BasePrieto, L. R., & Latson, L. A. (2013). Pulmonary stenosis. In H. D. Allen, D. J. Driscoll, R. E. Shaddy, & T. F. Feltes, (Eds.), Moss and Adams’ heart disease in infants, children, and adolescents (8th ed.). (pp. 913-938). Philadelphia: Lippincott Williams & Wilkins.
The pulmonary valve opens during systole to let blood flow from the right ventricle into the main PA. Obstruction to flow can occur at three levels: subvalvular, valvular, or supravalvular. The most common cause of RV outflow tract obstruction is pulmonary valve stenosis (PS). PS accounts for 8% to 12% of CHDs.
Pathophysiology and Etiology
Critical PS in the neonate: blood flows into the right atrium, across a patent foramen ovale (PFO) into the left side of heart; pulmonary blood flow comes from a left-to-right shunt through a PDA.
Right ventricular pressure increases to pump blood across the obstructive pulmonary valve.
RVH develops in response to the increased pressure gradient across the pulmonary valve.
Signs of right-sided heart failure include hepatic congestion, neck vein distention, elevated CVP.
Clinical Manifestations
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