Surgical Treatment of Pediatric Hypoplastic Left Heart Syndrome Workup

  • Author: Richard G Ohye, MD; Chief Editor: Stuart Berger, MD   more...
 
Updated: Apr 3, 2012
 

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).
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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.
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Other Tests

Electrocardiography reveals right atrial enlargement and right ventricular hypertrophy.

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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.

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Contributor Information and Disclosures
Author

Richard G Ohye, MD  Head, Division of Pediatric Cardiovascular Surgery, Program Director, Pediatric Cardiac Surgery Fellowship, University of Michigan Medical Center

Richard G Ohye, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for Thoracic Surgery, American College of Cardiology, American College of Chest Physicians, American College of Surgeons, Association for Academic Surgery, Congenital Heart Surgeons Society, International Society for Heart and Lung Transplantation, Society of Thoracic Surgeons, and Society of University 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, Division of Pediatric Cardiovascular Surgery, Professor, Department of Surgery, Section of Thoracic Surgery, 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.

Specialty Editor Board

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.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Mary C Mancini, MD, PhD  Professor and Chief of Cardiothoracic Surgery, Department of Surgery, Louisiana State University School of Medicine in Shreveport

Mary C Mancini, MD, PhD is a member of the following medical societies: American Association for Thoracic Surgery, American College of Surgeons, American Surgical Association, Phi Beta Kappa, Society of Thoracic Surgeons, and Southern Surgical Association

Disclosure: Nothing to disclose.

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

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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The main pulmonary artery and ductus arteriosus have been divided. Dashes indicate the line of incision on the hypoplastic ascending aorta. Image courtesy of Edward L. Bove, MD.
The ascending aorta is opened and sutured to the adjacent proximal main pulmonary artery. A patch of pulmonary homograft is fashioned to create the neoaorta (inset). Image courtesy of Edward L. Bove, MD.
Completed Norwood procedure showing reconstructed neoaorta and modified Blalock-Taussig shunt from the innominate artery to the confluence of the branch pulmonary arteries. Image courtesy of Edward L. Bove, MD.
Hemi-Fontan procedure. The modified Blalock-Taussig shunt is ligated, and incisions are made in the right atrial appendage and pulmonary arteries (upper left). The posterior aspect of the right atriotomy is anastomosed to the inferior aspect of the pulmonary arteriotomy (upper right). A patch of polytetrafluoroethylene is placed to prevent the superior vena cava return from entering the right atrium. The cavopulmonary connection is roofed with a patch of pulmonary homograft (lower). Image courtesy of Koji Kagasaki, MD.
Fontan procedure. Through a right atriotomy, the polytetrafluoroethylene (PTFE) patch has been removed. A new PTFE patch is placed to baffle the inferior vena cava return to the cavopulmonary connection constructed during the hemi-Fontan procedure (left). The arrows indicate the systemic venous return bypassing the right heart to directly enter the pulmonary arteries (right). Image courtesy of Edward L. Bove, MD.
 
 
 
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