eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Aortic Stenosis, Valvar: Differential Diagnoses & Workup

Author: Howard S Weber, MD, FAAP, FACC, FSCAI, Professor, Assistant Chief, Section of Pediatric Cardiology, Penn State University School of Medicine; Director, Pediatric Catheterization Laboratory, Milton S Hershey Medical Center
Coauthor(s): Paul M Seib, MD, Associate Professor of Pediatrics, University of Arkansas for Medical Sciences; Medical Director, Cardiac Catheterization Laboratory, Co-Medical Director, Cardiovascular Intensive Care Unit, Arkansas Children's Hospital
Contributor Information and Disclosures

Updated: Sep 29, 2009

Differential Diagnoses

Aortic Stenosis, Subaortic
Aortic Stenosis, Supravalvar
Hypertrophic cardiomyopathy, IHSS

Workup

Laboratory Studies

The following may be indicated in patients with aortic valve stenosis:

  • The evaluation for sepsis in infants presenting with shock includes blood, urine, and cerebrospinal fluid (CSF) cultures.
  • Pulse oximeter assessment is performed both preductally (typically in the right arm) and postductally (typically in a lower extremity). Oxygen saturation may be lower in the legs (postductal circulation) because of right-to-left shunting at the level of the ductus arteriosus.

Imaging Studies

  • In the neonate, transthoracic echocardiography usually provides complete diagnostic and hemodynamic information. Essential considerations are the details of valve anatomy, anulus size, distribution of valve tissue, degree of left ventricular hypertrophy, and left ventricular systolic function. The presence or absence of associated lesions, such as coarctation of the aorta or subaortic stenosis, can also be well delineated. Patients presenting with critical aortic valve obstruction and poor left ventricular systolic function may have echodense endocardium typical of endocardial fibroelastosis. Variable degrees of left ventricular hypoplasia or dilation may also be noted. These findings usually indicate that the severe obstruction was present for a significant amount of time prenatally.
  • In older patients, transthoracic echocardiography is usually diagnostic; however, rarely, a large adolescent or adult patient may require transesophageal echocardiography to clearly delineate the left ventricular outflow tract and to detail the valve anatomy.
  • Doppler echocardiography is used to estimate the severity of aortic valve stenosis. The peak instantaneous systolic gradient often overestimates the transvalvular peak-to-peak gradient obtained during cardiac catheterization. Mean Doppler gradients correlate well with mean gradients measured during cardiac catheterization. Significant individual variation exists in how closely the peak or mean Doppler-derived gradient predicts the peak-to-peak gradient measured at the time of cardiac catheterization. In neonatal critical aortic valve stenosis with poor left ventricular systolic function and low cardiac output, the Doppler-derived peak instantaneous gradient may be negligible and may not be indicative of the severity of obstruction.

Other Tests

  • Compared with echocardiography, MRI is rarely used to assess the details of aortic valve anatomy and is much more difficult to use in neonates, who have faster heart rates and more motion artifacts. New developments in gated MRI for assessing ventricular function may make MRI increasingly useful in adult patients.
  • Chest radiography may reveal cardiomegaly with pulmonary venous congestion primarily in neonates who present with critical stenosis and symptoms of congestive heart failure.
  • ECG in older patients may reveal left ventricular hypertrophy with or without a strain pattern but is less useful in neonates or young children with significant aortic valve disease.

Procedures

  • Exercise stress testing
    • Exercise stress testing can usually be performed in children aged 6 years or older and is helpful in eliciting symptoms that may not be evident from routine history. Doppler studies can be helpful in determining whether exercise restrictions are necessary by measuring the change in aortic valve gradient from rest to immediately after maximal exercise. Stress echocardiography is also useful in delineating the response of the left ventricle to increasing afterload during exercise in the setting of significant aortic valve disease. The exercise stress test findings establish a baseline against which to compare subsequent study results, especially if the patient's symptoms change, or the Doppler-derived gradient worsens and aids in the evaluation of the effectiveness of an intervention.
    • Exercise stress testing may also provide some risk stratification if intervention is delayed or contemplated. Factors such as heart rate, blood pressure response to exercise (blunted), exercise duration (reduced), provocable arrhythmias (ventricular ectopy of left ventricular origin) or ECG ischemic changes, and measured oxygen consumption provide useful data on which to base decisions regarding timing of intervention.
  • Cardiac catheterization
    • Cardiac catheterization is usually performed in infants, children, and older adolescents in anticipation of balloon aortic valvuloplasty. In adult patients with calcific changes, the valve is usually less compliant and amenable to balloon dilation, with a higher risk for aortic valve insufficiency. Occasionally, the peak systolic gradient measured in the catheterization laboratory with the patient under conscious sedation is significantly less than that estimated by Doppler echocardiography, causing intervention to be deferred.
    • Other indications for catheterization may include the need for accurate hemodynamic assessment in patients with multiple levels of obstruction, such as mitral stenosis or subaortic stenosis in combination with aortic valve stenosis. In the latter instance, high-fidelity catheters capable of discriminating between multiple levels of obstruction in close proximity are probably preferable but are significantly more difficult to use, especially in young patients.

Histologic Findings

  • Pathologically, the severity of stenosis is secondary to thickening and increased rigidity of the valve tissue, with varying degrees of diminished commissural separation. Most commonly, the valve is bicuspid with a single fused commissure and an eccentrically placed orifice. A third or rudimentary commissure may sometimes be apparent. Less commonly, the valve is unicuspid and dome shaped. Rarely, the valve has 3 unseparated cusps, with the stenosis being centrally located. Secondary calcification of the valve is extremely rare, and, at times, the aortic valve anulus may also be underdeveloped or hypoplastic, adding to the severity of left ventricular outflow tract obstruction.
  • The left ventricular myocardium hypertrophies concentrically in the presence of significant obstruction. Critically ill neonates may have extensive endocardial fibroelastosis, especially in the presence of a dilated nonhypertrophied left ventricle. Patients with chronic aortic stenosis and significantly elevated left ventricular systolic pressure may exhibit fibrotic changes in the myocardium. Left ventricular volume may be reduced, normal, or increased. One study identified mitral valve abnormalities in 7.5% of 200 infants undergoing surgical valvotomy. Ischemia or infarction can result in papillary muscle dysfunction and mitral valve regurgitation.

More on Aortic Stenosis, Valvar

Overview: Aortic Stenosis, Valvar
Differential Diagnoses & Workup: Aortic Stenosis, Valvar
Treatment & Medication: Aortic Stenosis, Valvar
Follow-up: Aortic Stenosis, Valvar
Multimedia: Aortic Stenosis, Valvar
References
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References

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Further Reading

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Keywords

valvar aortic stenosis, left ventricular outflow tract obstruction, aortic valve stenosis, supravalvar aortic stenosis, subvalvular aortic stenosis, valvular aortic stenosis, exercise-induced syncope, myocardial ischemia, congestive heart failure, bicuspid aortic valve, patent ductus arteriosus, coarctation of aorta, ventricular septal defect, mitral valve abnormalities, left ventricular hypoplasia, cardiac murmur, chest pain, hypoplastic left heart syndrome, Turner syndrome

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Contributor Information and Disclosures

Author

Howard S Weber, MD, FAAP, FACC, FSCAI, Professor, Assistant Chief, Section of Pediatric Cardiology, Penn State University School of Medicine; Director, Pediatric Catheterization Laboratory, Milton S Hershey Medical Center
Howard S Weber, MD, FAAP, FACC, FSCAI is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Coauthor(s)

Paul M Seib, MD, Associate Professor of Pediatrics, University of Arkansas for Medical Sciences; Medical Director, Cardiac Catheterization Laboratory, Co-Medical Director, Cardiovascular Intensive Care Unit, Arkansas Children's Hospital
Paul M Seib, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Arkansas Medical Society, International Society for Heart and Lung Transplantation, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Medical Editor

Juan Carlos Alejos, MD, Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California at Los Angeles
Juan Carlos Alejos, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, and International Society for Heart and Lung Transplantation
Disclosure: Actelion Honoraria Speaking and teaching

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

John W Moore, MD, MPH, Professor of Clinical Pediatrics, Section of Pediatric Cardiology, Department of Pediatrics, University of California San Diego School of Medicine; Director of Cardiology, Rady Children's Hospital
John W Moore, MD, MPH is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

CME Editor

Gilbert Z Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center
Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Chief Editor

Steven R Neish, MD, SM, Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine
Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association
Disclosure: Nothing to disclose.

 
 
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