Pediatric Hypoplastic Left Heart Syndrome Follow-up

  • Author: P Syamasundar Rao; Chief Editor: Stuart Berger, MD   more...
 
Updated: Nov 10, 2011
 

Further Inpatient Care

  • Initial preoperative management and postoperative care of hypoplastic left heart syndrome (HLHS) take place in the neonatal, pediatric, or cardiac ICUs.
  • When postoperative patients are clinically stable, transfer them to the general cardiac unit for adjusting oral medications, addressing feeding issues, and completing discharge teaching.
  • Involve a pediatric cardiologist during any noncardiac hospital admission of a patient who is status post (S/P) Norwood procedure. This is because of the complex cardiovascular physiology in infants after this surgery.
  • Hospitalization and inpatient care may be required for cardiac catheterizations, catheter interventions, surgical procedures and for treatment of intercurrent infections as well as for management of postsurgical complications, including those after Fontan operation.
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Further Outpatient Care

  • Schedule outpatient follow-up care 2 weeks after discharge in the typical postoperative patient.
  • Schedule those who are S/P cardiac transplantation earlier for necessary laboratory studies.
  • Earlier follow-up care is also necessary if a pericardial effusion is discovered on the discharge echocardiogram.
  • Periodic follow-up visits after stage I, II, and III operations are mandatory. Individualize outpatient follow-up care based on the needs of each patient.
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Inpatient & Outpatient Medications

  • Inpatient medications
    • Prostaglandin E1
    • Dopamine/dobutamine/milrinone
    • Furosemide (Lasix/Aldactone)
    • Captopril/enalapril
    • Digoxin
    • Potassium chloride
  • Outpatient medications
    • Furosemide (Lasix/Aldactone)
    • Captopril/enalapril
    • Digoxin
    • Potassium chloride
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Transfer

  • Transfer the infant to a hospital with appropriate ICUs. Pediatric cardiology and cardiovascular surgery services must be immediately available.
  • Carefully monitor the infant for apnea during transfer while on prostaglandin E1 therapy. If prostaglandin E1 has been started, consider elective endotracheal intubation before transfer.
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Complications

Preoperative complications include acidosis, congestive heart failure (CHF), renal failure, liver failure, necrotizing enterocolitis, sepsis, and death.

Postoperative complications include acidosis, CHF, renal failure, liver failure, necrotizing enterocolitis, sepsis, pericardial or pleural effusion, phrenic or recurrent laryngeal nerve damage, stroke, coarctation of the aorta, and death. Early graft rejection and opportunist infection may occur after cardiac transplantation.

Major complications following the Norwood procedure include aortic arch obstruction at the site of surgical anastomosis and progressive cyanosis caused by limited blood flow through the shunt. An inadequate atrial communication contributes to progressive cyanosis. A study of data from the Society of Thoracic Surgeons Congenital Heart Surgery Database assessed the mortality rates and postoperative complications after the Norwood procedure in 2,557 patients. Factors associated with complications included weight less than 2.5 kg, single right versus single left ventricle, preoperative shock, genetic abnormality, and preoperative mechanical ventilatory or circulatory support.[19]

Major complications following the bidirectional Glenn/hemi-Fontan procedure include transient superior vena cava syndrome and persistent pleural or pericardial effusion. The development of systemic venous to pulmonary venous collateral vessels is possible.

Major complications following the Fontan procedure include persistent pleural or pericardial effusion. Neurodevelopmental abnormalities are reported and may be inherent in some patients with hypoplastic left heart syndrome.

Arrhythmias, obstructed venous pathways, and protein-losing enteropathy are some of the other complications observed following the Fontan operation.

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Prognosis

  • Overall survival to the time of hospital discharge after the Norwood procedure is nearly 75%.[64] Success rates are higher in uncomplicated cases and lower in cases in which important preoperative risk factors are present, such as age greater than 1 month, significant preoperative tricuspid insufficiency, pulmonary venous hypertension, associated major chromosomal or noncardiac abnormalities, and prematurity.
  • High Aristotle scores (>20) are associated with high hospital mortality and low survival at follow-up.[65]
  • Low cerebral near-infrared spectroscopy oxygen saturations during the first 48 hours after Norwood procedure are strongly associated with adverse outcomes.[66]
  • Survival after the bidirectional Glenn/hemi-Fontan and Fontan operations is nearly 90-95%.
  • The actuarial survival rate after staged reconstruction is 70% at 5 years.
  • Institutional success rates vary.
  • Neurodevelopmental prognosis is not known; however, abnormalities are reported.
  • Approximately 20% of infants listed for cardiac transplantation die while waiting for a donor heart. After successful transplantation, the survival rate at 5 years is approximately 80%.
  • When the preoperative mortality is considered, the overall survival rate after cardiac transplantation is approximately 70%, or similar to the results for staged reconstruction.
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Patient Education

  • General: At the outset, appropriately warn the parents and other caregivers that hypoplastic left heart syndrome is a complex heart defect that requires multiple hospitalizations, surgeries, catheter interventions and long-term follow-up.
  • Medication
    • Educate parents regarding the doses and side effects of their child's cardiac medications.
    • Discuss interactions with other medications with the family and the infant's general pediatrician.
  • Feeding
    • Many infants require nasogastric tube feeding after discharge from the hospital. Parents must become comfortable with placement of the nasogastric feeding tube.
    • Frequently, increased-calorie formula is required for adequate growth. Provide the formula recipe or a source for purchasing it to the caregiver.
  • Follow-up care
    • Stress the importance of follow-up care. If necessary, provide cab or bus vouchers to ensure compliance.
    • If noncompliance becomes a critical issue, physicians are required to report to the appropriate family services agency.
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Contributor Information and Disclosures
Author

P Syamasundar Rao  MD, Professor of Pediatrics, Division of Cardiology, University of Texas Medical School at Houston; Director, Division of Pediatric Cardiology, Children's Memorial Hermann Hospital; Professor of Pediatrics, MD Anderson Cancer Center, University of Texas

P Syamasundar Rao is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, American Pediatric Society, Medical Association of Georgia, Society for Cardiac Angiography and Interventions, Society for Pediatric Research, Southern Society for Pediatric Research, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Coauthor(s)

Daniel R Turner, MD  Associate Professor of Pediatrics, Wayne State University School of Medicine; Consulting Staff, Carman and Ann Adams Department of Pediatrics, Division of Cardiology, Children's Hospital of Michigan

Daniel R Turner, MD 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.

Thomas J Forbes, MD, FACC, FSCAI  Associate Professor (Clinical-Educator), Director of Catheterization Laboratory, Division of Pediatric Cardiology, Children's Hospital of Michigan, Wayne State University

Thomas J Forbes, MD, FACC, FSCAI is a member of the following medical societies: American College of Cardiology, American Heart Association, and Society of Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Specialty Editor Board

Ira H Gessner, MD  Professor Emeritus, Pediatric Cardiology, University of Florida College of Medicine

Ira H Gessner, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Pediatric Society, and Society for Pediatric Research

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.

Julian M Stewart, MD, PhD  Associate Chairman of Pediatrics, Director, Center for Hypotension, Westchester Medical Center; Professor of Pediatrics and Physiology, New York Medical College

Julian M Stewart, MD, PhD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

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

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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This echocardiographic still frame shows a long-axis view of the aortic arch in a patient with hypoplastic left heart syndrome (HLHS). The ascending aorta is markedly hypoplastic, serving only to deliver blood in a retrograde fashion to the coronary arteries. An echo-bright coarctation shelf is seen at the insertion of the ductus arteriosus.
This echocardiographic still frame shows a 4-chamber view of the heart in a patient with hypoplastic left heart syndrome (HLHS). A large right ventricle (RV) and hypoplastic left ventricle (star) are seen. Right atrium = RA. Left atrium = LA.
 
 
 
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