A 3-year-old female was referred to the emergency room for further evaluation after her local physician noted hepatomegaly to 4 centimeters below the costal margin and tachycardia.
She was previously well but complained of fatigue for ~10 days and generalized, achy abdominal pain that was increasing over the last few days.
The pain was fairly constant but intensity itsdid wax and wane. She lost her appetite about 3 days before and was eating and drinking less.
Her urine was darker and her stools had normal coloration without blood.
She was very tired at the end of the day and was taking extra naps. She was having problems with frequent wakening because of coughing.
The past medical history was negative.
The family history revealed an uncle with some type of inflammatory bowel disease, and distant relatives with cancer.
The review of systems showed that she had an upper respiratory infection about 3 weeks ago and that she had no vomiting, diarrhea, dysuria, fever, or rashes.
She did have a slight cough.
She and her parents denied any weight change or dependent area edema.
The pertinent physical exam showed a very tired, pale appearing preschool female. Her vital signs were heart rate ~200 beats/minute, blood pressure = 70/46 mm Hg, respiratory rate = 50, and she was afebrile. HEENT examination was negative. Heart was tachycardic without an obvious murmur. Heart sounds were slightly decreased.
Pulses in upper and lower extremities were equal but seemed thready. Lungs had rales at the bases. Abdomen had normal bowel sounds with the liver enlarged 4 centimeters below the costal margin. It was uniform in texture and non-tender.
She had no splenomegaly. Her abdomen was tympanitic without a distinct fluid wave. Genitourinary examination was Tanner 1. She had edema of her feet to the ankle.
Neurological examination was normal.
The work-up included an electrocardiogram with a heart rate of ~200-210 beats per minute with morphology consistent with the diagnosis of supraventricular tachycardia.
She was transferred to the intensive care unit and ice applied to her face 3 times was attempted to convert her to sinus rhythm, but she had only transient heart rate slowing.
She was given adenosine which decreased her heart rate again temporarily. Concurrently an echocardiogram showed depressed left ventricular function with an ejection fraction of 31% (normal >55%) and shortening fraction of 18% (normal >27%).
She was given two intravenous boluses of amiodarone for the purpose of cardioversion and then was begun on an infusion that controled the tachycardia.
The patient had an endomyocardial biopsy (for possible myocarditis which eventually was negative) and electrophysiological studies which showed an arrhythmogenic, ectopic focus in her right atrial appendage causing the supraventricular tachycardia.
She had cryothermal ablation and afterwards her heart rate ranged between 80 -120 beats per minute with a sinus rhythm.
Her post-ablation echocardiogram showed improving heart function after the procedure. She was discharged on day 10 on digoxin, captopril, furosemide and propranolol and with much improved clinical signs of heart failure.
The patient’s clinical course found her to be improving one week later. Over the next 3 months she slowly had her medications weaned.
At follow-up, one year later, she has had no recurrences and is doing well in preschool and activities.
Sinus tachycardia is one of the most common arrhythmias. It is a normal sinus rhythm but the heart rate is > 95th percentile for age and usually is less than 220 beats/minute in children and 200 beats/minutes in young adults. It is frequently encountered in children who are febrile or anxious or taking certain drugs such as beta-agonists or theophylline. Sinus tachycardia can be more ominous in cases of anemia, hypovolemia, sepsis, heart failure, pulmonary embolus, or myocardial disease.
Supraventricular tachycardia (SVT) is defined as a run of 3 or more premature supraventricular beats not originating from the sinoatrial node, often seen as abnormal or absent P waves. The rate can vary, but is usually above 220 beats/minute. It is also usually associated with a narrow QRS complex and can be intermittent or sustained in duration. Most often SVT is associated with an accessory pathway of cardiac muscle which provides a “re-entry” circuit for the arrhythmia, but it may also be caused by an ectopic focus of tissue with its own automaticity as in this case. Initial treatment is by using vagal maneuvers such as ice to the face or Valsalva maneuvers. Adenosine can also be used if vagal maneuvers are unsuccessful and will convert most SVT to normal rhythm if due to a re-entry circuit. If the patient is unstable, electrical cardioversion can be tried. It can sometimes be difficult to determine if the rhythm is sinus tachycardia ro SVT, especially if the rates are > 200 beats/minute.
Congestive heart failure occurs when the heart cannot pump enough blood to meet the body’s needs, cannot handle venous return adequately or a combination of both, despite compensatory mechanisms.
Clinically, congestive heart failure presents with poor feeding, inadequate weight gain, sweating or shortness of breath especially with feeding or other activities, edema of the eyes, feet or hands, or increased fatigue.
Physical examination may reveal poor pulses and capillary refill, cold skin, tachycardia, abnormal rhythms, dyspnea, orthopnea, wheezing or rales, hepatomegaly without splenomegaly, edema of dependent areas, and distended neck veins.
Treatment for congestive heart failure is with inotropic support (e.g. dopamine, digoxin), alleviating venous congestion (e.g. diuretics), vasodilatation (i.e. ACE inhibitors) and beta-blockade (e.g. propanolol). Patients also may need other supportive treatment such as mechanical ventilation, supplemental oxygen, bed rest, positioning, and mechanical circulatory supports.
Treatment for identifiable underlying causes such as the SVT in this patient, need to be instituted.
The most common reason for heart failure in children is volume overload secondary to a left-to-right shunt.
Causes of heart failure include:
- Arrhythmias – complete heart block, SVT
- Arteriovenous malformations, high output
- Bronchopulmonary dysplasia
- Acute cor pulmonale secondary to airway obstruction – large tonsils and adenoids, cystic fibrosis
- Cardiomyopathy, idiopathic dilated
- Drug side effects – doxorubicin (or other anthracyclines), substance abuse
- Endocardial fibroelastosis
- Endocrine – adrenal diseases, hyperthyroidism, hypothyroidism
- Infection – acute rheumatic carditis and/or valve disease, HIV, Lyme disease, sepsis, viral myocarditis
- Metabolic abnormalities – acidosis, anemia, hypoxia, hypocalcemia, hypoglycemia
- Renal disease – failure, hypertension
- Vasculitis – collagen vascular disease, Kawasaki disease
- Arterio-venous canal
- Large atrial septal defect
- Coarctation of the aorta
- Genetic diseases – Friedreich’s ataxia, glycogen storage disease, Hunter-Hurler syndrome, Marfan syndrome, muscular dystrophy, Noonan syndrome
- Hypertrophic left heart syndrome
- Large patent ductus arteriosus
- Persistent pulmonary hypertension
- Totally anomalous pulmonary venous return
- Truncus arteriosus
- Single ventricle
- Large ventricular septal defect
- Valvular problems – critical aortic stenosis, critical pulmonary stenosis, pulmonary insufficiency, tricuspid insufficiency
Questions for Further Discussion
1. What are indications for intubation and ventilation for a patient with an arrhythmia or heart failure?
2. What are the different types of cardioversion and what are their indications?
3. What are common causes of fatigue in a child?
4. What are indications for treatment with mechanical devices such as ventricular assist devices, extracorporal membrane oxygenation?
5. What are the most common causes of SVT and how do their mechanisms differ?
To Learn More
To view pediatric review articles on this topic from the past year check PubMed.
Information prescriptions for patients can be found at MedlinePlus for these topics: Arrhythmias and Heart Failure
and at Pediatric Common Questions, Quick Answers for this topic: Heart Murmurs and Arrhythmias.
To view current news articles on this topic check Google News.
To view images related to this topic check Google Images.
Park MY. Pediatric Cardiology for Practitioners. 3rd Edit. Mosby, St. Louis, MO. 1996:401-403.
Rudolph CD, et.al. Rudolph’s Pediatrics. 21st edit. McGraw-Hill, New York, NY. 2003:1869-1877.
Robertson J, Shilkofski N. The Harriet Lane Handbook. 17th. Edit. Mosby Publications: St. Louis. 2005:177-179.
Odland HH, Thaulow EM. Heart Failure Therapy in Children.
Expert Rev Cardiovasc Ther. 2006;4(1):33-40.
ACGME Competencies Highlighted by Case
1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
2. Essential and accurate information about the patients’ is gathered.
3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
4. Patient management plans are developed and carried out.
5. Patients and their families are counseled and educated.
6. Information technology to support patient care decisions and patient education is used.
7. All medical and invasive procedures considered essential for the area of practice are competently performed.
8. Health care services aimed at preventing health problems or maintaining health are provided.
9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.
10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.
25. Quality patient care and assisting patients in dealing with system complexities is advocated.
26. Partnering with health care managers and health care providers to assess, coordinate, and improve health care and how these activities can affect system performance are known.
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa Children’s Hospital
Nicholas Von Bergan, M.D.
Pediatric Cardiology Fellow, University of Iowa Children’s Hospital
October 15, 2007