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Surgical Treatment of Pediatric Hypoplastic Left Heart Syndrome Workup

  • Author: Ming-Sing Si, MD; Chief Editor: Stuart Berger, MD  more...
 
Updated: Apr 11, 2014
 

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 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.

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

Ming-Sing Si, MD Assistant Professor of Cardiac Surgery, University of Michigan Medical School

Ming-Sing Si, MD is a member of the following medical societies: American Heart Association, Biomedical Engineering Society, Society of Thoracic Surgeons, Tissue Engineering and Regenerative Medicine International Society, International Society for Stem Cell Research

Disclosure: Nothing to disclose.

Coauthor(s)

Richard G Ohye, MD Head, Section of Pediatric Cardiovascular Surgery, Associate Professor of Cardiac 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, Congenital Heart Surgeons Society, Society of University Surgeons, American College of Cardiology, American College of Chest Physicians, American College of Surgeons, Association for Academic Surgery, International Society for Heart and Lung Transplantation, 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, Society of University Surgeons

Disclosure: Nothing to disclose.

Jennifer C Hirsch-Romano, MD, MS, FACS, FACC Assistant Professor of Cardiac Surgery, Pediatric Cardiac Surgery, Department of Cardiac Surgery, Assistant Professor, Pediatric Cardiac Intensive Cre, Department of Pediatrics, Surgical Director, Pediatric Cardiothoracic Intensive Care Unit, Co-Director, Pediatric Cardiac Extracorporeal Life Support, Pediatric Cardiac Surgeon, Fetal Diagnosis and Treatment Center, University of Michigan Medical Center

Jennifer C Hirsch-Romano, MD, MS, FACS, FACC is a member of the following medical societies: American College of Cardiology, American College of Surgeons, Association for Academic Surgery, Congenital Heart Surgeons Society, Society of Thoracic Surgeons, Association of Women Surgeons, European Association for Cardio-Thoracic Surgery, John Alexander Society, Frederick A Coller Surgical Society, Midwest Pediatric Cardiology Society

Disclosure: Nothing to disclose.

Specialty Editor Board

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, MMM Professor and Chief of Cardiothoracic Surgery, Department of Surgery, Louisiana State University School of Medicine in Shreveport

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

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD Medical Director of The Heart Center, Children's Hospital of Wisconsin; Associate Professor, Department of Pediatrics, Section of Pediatric Cardiology, Medical College 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, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Additional Contributors

Jonah Odim, MD, PhD, MBA Section Chief of Clinical Transplantation, Transplantation Branch, Division of Allergy, Immunology, and Transplantation, National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health (NIH)

Jonah Odim, MD, PhD, MBA is a member of the following medical societies: American College of Cardiology, American College of Chest Physicians, American Association for Physician Leadership, 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, 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, Society of Thoracic Surgeons, Canadian Cardiovascular Society

Disclosure: Nothing to disclose.

Acknowledgements

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.

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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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