eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiothoracic Surgery

Hypoplastic Left Heart Syndrome and the Staged Norwood Procedure: Workup

Author: Richard G Ohye, MD, Director, Pediatric Cardiac Transplantation, Fellowship Program Director, Pediatric Cardiac Surgery, Assistant Professor, Department of Surgery, Section of Cardiac Surgery, University of Michigan Medical Center
Coauthor(s): Ralph S Mosca, MD, Director, Pediatric Cardiac Surgery, Associate Professor, Department of Surgery, New York Presbyterian Medical Center; Edward L Bove, MD, Associate Director, PICU, CS Mott Children's Hospital; Director, Department of Surgery, Section of Thoracic Surgery, Division of Pediatric Cardiovascular Surgery, Professor, University of Michigan Medical Center
Contributor Information and Disclosures

Updated: Jul 15, 2008

Workup

Laboratory Studies

  • Routine laboratory studies, such as CBC count, platelets, electrolytes, BUN levels, creatinine levels, liver function tests, and coagulation studies, are indicated. In addition to ensuring that laboratory test values return to reference ranges, maintain the hematocrit level at 45% or more if the patient is cyanotic.
  • Routine serial monitoring of ABGs is important in balancing the relative pulmonary and systemic blood flows in patients with single ventricle physiology (see Medical therapy, Surgical therapy).

Imaging Studies

  • Chest radiography
    • Chest radiographs reveal cardiomegaly and increased pulmonary vascular markings.
    • In 2% of patients, a reticular pattern of obstructed pulmonary venous return is seen because of a restrictive atrial septal defect.
  • Doppler echocardiography
    • Diagnosis is made using 2-dimensional and color Doppler echocardiography for determination of cardiac morphology and evaluation of the arch hypoplasia.
    • Color-flow Doppler images reveal that the blood flow in the ascending aorta is typically retrograde.

Other Tests

  • Electrocardiography reveals right atrial enlargement and right ventricular hypertrophy.

Diagnostic Procedures

  • Cardiac catheterization is rarely necessary. An exception would be to gain additional information in patients with borderline left ventricle size to assist in the decision-making process regarding the optimal treatment method.

More on Hypoplastic Left Heart Syndrome and the Staged Norwood Procedure

Overview: Hypoplastic Left Heart Syndrome and the Staged Norwood Procedure
Workup: Hypoplastic Left Heart Syndrome and the Staged Norwood Procedure
Treatment: Hypoplastic Left Heart Syndrome and the Staged Norwood Procedure
Follow-up: Hypoplastic Left Heart Syndrome and the Staged Norwood Procedure
Multimedia: Hypoplastic Left Heart Syndrome and the Staged Norwood Procedure
References
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References

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Further Reading

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Keywords

hypoplastic left heart syndrome, staged Norwood procedure, HLHS, left ventricular hypoplasia, absence of left ventricle, ascending aorta hypoplasia, staged orthoterminal correction, cardiac anomaly, cardiac disease, cardiac surgery, heart surgery, heart disease, orthotopic cardiac transplantation, cardiac transplantation, staged reconstructive cardiac surgery, right ventricle–to–pulmonary artery conduit, RVPAC, heart transplantation, aortic atresia, mitral atresia, congenital heart disease, patent ductus arteriosus, metabolic acidosis, renal failure, aortic stenosis, mitral stenosis, tricuspid regurgitation, cardiomegaly, obstructed pulmonary venous return, atrial septal defect, Fontan procedure

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Contributor Information and Disclosures

Author

Richard G Ohye, MD, Director, Pediatric Cardiac Transplantation, Fellowship Program Director, Pediatric Cardiac Surgery, Assistant Professor, Department of Surgery, Section of Cardiac Surgery, University of Michigan Medical Center
Richard G Ohye, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Cardiology, American College of Chest Physicians, American College of Surgeons, Association for Academic Surgery, International Society for Heart and Lung Transplantation, and Society of Thoracic Surgeons
Disclosure: Nothing to disclose.

Coauthor(s)

Ralph S Mosca, MD, Director, Pediatric Cardiac Surgery, Associate Professor, Department of Surgery, New York Presbyterian Medical Center
Ralph S Mosca, MD is a member of the following medical societies: American College of Surgeons, Central Surgical Association, Congenital Heart Surgeons Society, International Society for Heart and Lung Transplantation, Michigan State Medical Society, and Society of Thoracic Surgeons
Disclosure: Nothing to disclose.

Edward L Bove, MD, Associate Director, PICU, CS Mott Children's Hospital; Director, Department of Surgery, Section of Thoracic Surgery, Division of Pediatric Cardiovascular Surgery, Professor, University of Michigan Medical Center
Edward L Bove, MD is a member of the following medical societies: American Association for Thoracic Surgery, American College of Cardiology, American College of Chest Physicians, American College of Surgeons, American Heart Association, American Medical Association, American Surgical Association, Central Surgical Association, Congenital Heart Surgeons Society, Medical Society of the State of New York, Society of Thoracic Surgeons, and Society of University Surgeons
Disclosure: Nothing to disclose.

Medical Editor

Jonah Odim, MD, PhD, MBA, Senior Medical Officer, Transplantation Immunology Branch, Division of Allergy, Immunology, and Transplantation, National Institute of Allergy and Infectious Diseases, National Institutes of Health
Jonah Odim, MD, PhD, MBA is a member of the following medical societies: American College of Cardiology, American College of Chest Physicians, American College of Physician Executives, American College of Surgeons, American Heart Association, American Society for Artificial Internal Organs, American Society of Transplant Surgeons, Association for Academic Surgery, Association for Surgical Education, Canadian Cardiovascular Society, International Society for Heart and Lung Transplantation, National Medical Association, New York Academy of Sciences, Royal College of Physicians and Surgeons of Canada, Society of Critical Care Medicine, and Society of Thoracic Surgeons
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Mary C Mancini, MD, PhD, Professor, Department of Surgery, Louisiana State University Health Sciences Center
Mary C Mancini, MD, PhD is a member of the following medical societies: American Heart Association, American Medical Association, American Thoracic Society, Association for Academic Surgery, Association for Surgical Education, International College of Surgeons, International Society for Heart and Lung Transplantation, New York Academy of Sciences, Phi Beta Kappa, and Southern Thoracic Surgical Association
Disclosure: Nothing to disclose.

CME Editor

Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine
Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine
Disclosure: Baxter Honoraria Consulting; Pfizer Honoraria Consulting

Chief Editor

Stuart Berger, MD, Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin
Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

 
 
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