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

Hypoplastic Left Heart Syndrome and the Staged Norwood Procedure: Treatment

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

Treatment

Medical Therapy

Initial medical support in infants with hypoplastic left heart syndrome (HLHS) requires a specific medical regimen. The goals of preoperative management are to maintain ductal patency and to provide the appropriate balance between the systemic and pulmonary vascular resistances. Intravenous prostaglandin E is infused at 0.05 mcg/kg/min to maintain patency of the ductus arteriosus. This dose may be titrated to keep the ductus arteriosus open while minimizing the risk of apnea.

Oxygen saturation is monitored by pulse oximetry. Acidosis is rapidly reversed using sodium bicarbonate. The fraction of inspired oxygen (FIO2) is adjusted to maintain a relative hypoxemia (oxygen saturation 75-80%), which aids in preventing the pulmonary vasodilatation associated with high oxygen concentrations. Even in the neonate who is being resuscitated because of circulatory collapse, ventilation with a high concentration of oxygen is avoided because it may only further decrease pulmonary vascular resistance and systemic blood flow.

Blood transfusion should be performed to maintain the hematocrit between 45-50%. Mechanical ventilation is avoided when possible, but infants on ventilation may require sedation with intravenous fentanyl to prevent tachypnea. In patients with significant pulmonary overcirculation, hypoventilation to maintain a mild respiratory acidosis (partial pressure of carbon dioxide [PCO2] of 45-55 mm Hg) and elevation of pulmonary vascular resistance may be used. Occasionally, inhaled nitrogen or carbon dioxide can be added to reduce the FIO2 to between 16-18% to increase pulmonary vascular resistance. Inotropic support is advantageous in patients with depressed right ventricular function.

Nourishment is usually provided via intravenous hyperalimentation, which avoids the added risk of necrotizing enterocolitis prior to surgery. Diuretics are added, as necessary, when pulmonary congestion becomes apparent. This regimen simulates the fetal balance of pulmonary and systemic vascular resistance, stabilizing the infant while deciding on therapeutic options.

Surgical Therapy

Parents of children with HLHS are presented with the following 3 options: (1) supportive therapy only (leading usually to rapid demise), (2) staged reconstruction, and (3) orthotopic cardiac transplantation. Each institution must assess its results with the various modes of therapy and counsel the parents accordingly. As outcomes of palliative procedures and heart transplantation in patients with HLHS have improved, even surpassing therapies for other complex forms of congenital heart disease in some patients, the first option of supportive therapy only has been challenged. The techniques and results of staged reconstruction are discussed below.

The goal of staged reconstruction is a Fontan procedure, creating separate pulmonary and systemic circulations supported by a single (right) ventricle. The initial stage must provide unobstructed systemic blood flow from the right ventricle to the aorta and coronary arteries, relieve any obstruction to pulmonary venous return, and limit pulmonary blood flow by virtue of an appropriately sized systemic–to–pulmonary artery shunt or RVPAC.

As a result of the relatively high pulmonary vascular resistance present in the newborn period, a systemic shunt is necessary, and the right ventricle performs the increased volume of work of both the pulmonary and systemic circulations. Preservation of right ventricular function has been aided by using smaller initial aortopulmonary shunts to limit right ventricular volume overload, by using an RVPAC, and by using an interim procedure between the Norwood and Fontan operations. This staging procedure, either a bidirectional Glenn anastomosis or a hemi-Fontan procedure, is usually performed at age 6 months.

Recently, the use of an RVPAC as an alternative to the traditional modified Blalock-Taussig shunt (MBTS) has been proposed. Initially described by Norwood in his original description of the Norwood procedure, the use of the MBTS quickly became the preferred source of pulmonary blood flow. In 2002, Sano and colleagues described their experience with the Norwood procedure, with improvements in hospital survival from 53-89% when compared with historical controls.11 Several other groups also reported similar improvements in outcomes, based on historical controls. A recent comparison using nonrandomized, but contemporary, controls by Tabbutt and colleagues revealed no difference in hospital survival.12

The general trend in the literature has been that centers that have had difficulties in achieving optimal survivals with the Norwood procedure have found a benefit to using the RVPAC, whereas institutions that have had better success with the Norwood procedure have found no improvement. The optimal source of pulmonary blood flow remains to be determined. Each source has inherent advantages and disadvantages, and the long-term, or even intermediate-term, effects are unknown. The MBTS results in significant diastolic run-off and potential coronary steal. The RVPAC avoids this run-off but adds the undesirable need to place an incision on the right ventricle.

Currently, a multi-institutional, randomized, controlled trial is being performed by the Pediatric Heart Network with funding from the National Heart, Lung, Blood Institute of the National Institutes of Health. The trial is predicted to complete enrollment in June 2008. Allowing 12 months for the primary outcome of death or cardiac transplantation at 12 months, the results are anticipated to be published in late 2009.

Whichever source of pulmonary blood flow is selected, these procedures provide adequate pulmonary blood flow while decreasing volume overload to the right ventricle and improving effective pulmonary blood flow until the patient can undergo a completion Fontan procedure. As described in detail in Second-stage palliation: Hemi-Fontan or bidirectional Glenn anastomosis, the hemi-Fontan procedure is a modification of the bidirectional Glenn procedure. The hemi-Fontan procedure involves (1) a side-to-side connection between the superior vena cava (SVC)/right atrial junction and the pulmonary arteries, (2) routine augmentation of the branch pulmonary arteries, and (3) temporary patch closure between the pulmonary arteries and the right atrium.

Preoperative Details

See Medical therapy.

Intraoperative Details

First-stage palliation: Norwood procedure

Through a midline sternotomy, cardiopulmonary bypass (CPB) is established. A minimum of 20 minutes of cooling to a core temperature of 18°C is begun for deep hypothermic circulatory arrest. Alternatively, some groups have reported the use of regional low-flow cerebral perfusion in lieu of deep hypothermic circulatory arrest (see Future and Controversies).

Regardless of the technique used, the septum primum is completely excised (atrial septectomy). The ductus is ligated and divided. The main pulmonary trunk is proximally divided to the bifurcation of the pulmonary arteries. The resultant opening in the pulmonary artery is closed with a patch of pericardium, polytetrafluoroethylene, or homograft. The remaining ductal tissue (on the undersurface of the aortic arch) is completely excised, and the incision is extended at least 10 mm further down the descending aorta into a normal-appearing and normal-caliber aorta (see Media file 1). This incision is proximally extended under the transverse arch and down the diminutive ascending aorta until the level of the previously divided main pulmonary trunk is reached (see Media file 2).

A cryopreserved pulmonary allograft is trimmed to fashion a patch that serves to enlarge the aorta and allow anastomosis to the proximal main pulmonary trunk (see inset in Media file 2). The remainder of the aorta is attached to the pulmonary allograft, proximally incorporating the main pulmonary trunk. The cannulae are replaced to begin bypass and commence systemic rewarming to 37°C.

A polytetrafluoroethylene shunt is placed from the innominate artery to the central pulmonary artery during rewarming (see Media file 3). A 4-mm shunt is used in patients who weigh more than 3.5-4 kg; smaller patients receive a 3.5-mm shunt. The distal end of the shunt is centrally placed on the pulmonary arteries, rather than onto the right pulmonary artery, to promote even distribution of blood flow to both lungs. Alternatively, the right ventricle–to–pulmonary artery conduit can be placed. These polytetrafluoroethylene grafts are generally either 5 mm or 6 mm in diameter. 

Second-stage palliation: Hemi-Fontan or bidirectional Glenn anastomosis procedure

The hemi-Fontan operation or a bidirectional Glenn anastomosis is typically performed in infants aged 3-10 months to minimize the period of time during which the right ventricle is subject to volume overload. Cardiac catheterization is performed prior to this procedure to evaluate pulmonary vascular resistance, pulmonary artery anatomy, tricuspid valve regurgitation, and right ventricular function.

To perform a bidirectional Glenn procedure, CPB is achieved with neoaortic arch cannulation and separate right-angle IVC and right-angle SVC cannulae. The aortopulmonary shunt is ligated and divided when CPB is initiated. If any stenosis of the pulmonary artery secondary to the prior shunt or patch is present, the stenosis is repaired with patch augmentation. The azygous vein is ligated and divided. The SVC is transected and anastomosed in an end-to-side fashion to the superior aspect of the right pulmonary artery. The cardiac end of the transected SVC is oversewn. Some groups routinely perform the bidirectional Glenn procedure without CPB, with or without an SVC-to–right aorta temporary shunt, during the anastomosis to minimize high cerebrovenous pressures.

The hemi-Fontan procedure has the same physiologic factors as a bidirectional Glenn anastomosis but includes an anastomosis of the pulmonary arteries to an incision in the atriocaval junction. The cavopulmonary connection may be performed under a brief period of deep hypothermic circulatory arrest. Alternatively, cannulation of the IVC and high on the SVC can be used to perform the procedure entirely during CPB.

Whether the procedure is performed under circulatory arrest or during CPB, the remainder of the procedure is the same. The aortopulmonary shunt is divided, and the pulmonary arteries are mobilized from the right to the left upper lobe. The azygous vein is ligated. The right atrium is opened along the superior aspect of the appendage, and a corresponding incision is made transversely along the confluence of the branch pulmonary arteries (see upper left of Media file 4). The posterior aspect of the right arteriotomy is anastomosed to the inferior aspect of the pulmonary arteriotomy (see upper right of Media file 4).

A patch of pulmonary allograft tissue is fashioned to augment the pulmonary arteries. The allograft patch is begun at the left upper lobe, incorporating a separate end-to-side anastomosis for a left SVC, if necessary (see lower portion of Media file 4). A patch is placed within the right atrium, which isolates SVC return into the pulmonary arteries and provides an unobstructed pathway for connection of IVC return during the Fontan procedure (see lower portion of Media file 4). The atrial septal defect is inspected and enlarged, if necessary, which is completed best by cutting back the coronary sinus into the left atrium. Tricuspid valve repair is also performed as needed.

The advantage of the hemi-Fontan is that it shortens the length of time of CPB and dissection required for the completion Fontan procedure, which requires only the removal of the intra-atrial patch and placement of a lateral tunnel in the right atrium from the IVC to the SVC. In addition, routine augmentation of the branch pulmonary arteries helps optimize the anatomy for the completion Fontan procedure.

Third-stage palliation: Fontan procedure

The completion Fontan procedure is usually performed in children aged 18-24 months. The infant is evaluated using cardiac catheterization prior to surgery. The Fontan technique used by the authors for HLHS anatomy is the technique termed total cavopulmonary connection with a lateral tunnel. After achieving CPB, the right atrium is opened.

If a hemi-Fontan procedure has been performed, the intra-atrial baffle is resected. In bidirectional Glenn anastomosis, a right atrium–to–pulmonary artery anastomosis is created. A baffle of polytetrafluoroethylene is fashioned and placed inside of the right atrium to convey the IVC return to the cavopulmonary connection (see Media file 5). This technique minimizes the possibility of obstruction of the pulmonary venous return, which can be caused by an atriopulmonary anastomosis. Fenestration of the baffle may help prevent complications in high-risk patients and shorten the period of pleural drainage. Some centers opt for an extracardiac, instead of an intracardiac, lateral tunnel Fontan.

Postoperative Details

First-stage palliation: Norwood procedure

After weaning from CPB, an atrial-monitoring catheter is placed to measure central venous pressure, and infusion of inotropes is initiated. University of Michigan medical staff routinely use continuous infusions of milrinone and low-dose dopamine, adding epinephrine in doses of 0.02-0.06 mcg/kg/min if hypotension is significant. Ventilation, with an initial FIO2 of 100% to achieve a PCO2 of approximately 35 mm Hg, is initiated and adjusted depending on the systemic arterial oxygen saturation and the systemic perfusion. If poor peripheral perfusion with systemic saturation in excess of 80-85% is noted, the FIO2 and minute ventilation are decreased to avoid excess pulmonary vasodilatation. The opposite maneuvers are used if systemic oxygen saturation is less than 70-75%.

Postoperative management is aimed at maintaining the delicate balance between the systemic and pulmonary vascular resistances and, therefore, relative systemic and pulmonary blood flow. Many regimens of ventilation, inotropic support, and vasodilatory support have been used, and multiple indicators of perfusion adequacy (mixed venous oxygen, lactate) have been measured with varying degrees of success.

Ideally, systemic arterial saturation should be maintained at 75-80%, which usually indicates that an optimal pulmonary-to-systemic blood flow ratio of less than 1 has been achieved. However, measurements of mixed venous oxygen saturation and pulmonary venous oxygen saturation are necessary to accurately assess the ratio of pulmonary blood flow (Qp) to systemic blood flow (Qs). The authors have found that serial lactate measurements provide an excellent indication of low cardiac output, and they rely on these determinations rather than mixed venous oxygen saturations.

Second- and third-stage palliation: Hemi-Fontan and Fontan procedures

For second- and third-stage operations, maintaining a low pulmonary vascular resistance is paramount. The pulmonary blood flow no longer is driven by an arterial shunt (or systemic RV, in cases of Sano modification), but by central venous pressure. Hypoxia and acidosis, which increase pulmonary vascular resistance, are avoided. Although mild respiratory alkalosis may be beneficial for pulmonary vascular resistance, a pH higher than 7.45-7.5 decreases cerebral blood flow and, hence, SVC return. After the hemi-Fontan procedure is performed, this decrease in SVC return decreases pulmonary blood flow. Inhaled nitrous oxide (NO) may also be used to decrease pulmonary vascular resistance but is infrequently needed.

Follow-up

After discharge from the hospital, regular cardiovascular evaluations are important. The child should be carefully observed for aortic arch obstruction, tricuspid insufficiency, and increasing cyanosis secondary to a limited atrial septal defect, shunt stenosis, or pulmonary artery distortion. For other long-term concerns, see Complications and Outcomes and Prognosis.

Complications

Complications that result from a procedure of the magnitude of the Norwood palliation are fairly common. Complications may include bleeding, low cardiac output syndrome, and arrhythmia in the immediate postoperative period. Aggressive correction of thrombocytopenia and coagulation factors is warranted. Poor peripheral and end-organ perfusion may represent poor cardiac output or pulmonary overcirculation, which may be treated by inotropic support or manipulation of relative pulmonary and systemic resistances. Common arrhythmias include junctional ectopic tachycardiac, which can be treated either with surface cooling to 35-36°C and pacing or with amiodarone infusion.

Incidence of unexplained sudden death, both in the immediate postoperative period and after discharge, remains problematic. In the postoperative period, the authors found that serial serum lactate determinations demonstrating failure to clear lactic acidosis have been helpful in predicting patients who will do poorly despite an apparently stable clinical condition.

Shunt complications, such as thrombosis, can occur. All patients are started on low-dose aspirin when they begin enteral nutrition. Other causes of increasing cyanosis during the postoperative period include pulmonary artery stenosis or distortion and restriction at the level of the atrial septal defect.

Evaluation prior to the hemi-Fontan procedure may reveal pulmonary artery stenosis, particularly of the left branch or at the insertion of the shunt. These stenoses are managed with patch augmentation during the hemi-Fontan procedure. Residual coarctation should be rare if the initial homograft patch is brought sufficiently onto the descending aorta during the Norwood procedure. Postoperative coarctation can usually be managed with balloon dilatation or, if necessary, surgical augmentation.

Long-term complications following the Fontan operation include atrial arrhythmia, thromboembolic events, and protein-losing enteropathy. Atrial arrhythmias are less common with the current techniques of cavopulmonary connections and may be treated with standard antiarrhythmic therapy. All of the authors' patients who undergo the Fontan procedure are maintained on aspirin therapy, whereas others have advocated low-dose warfarin as prophylaxis against thromboembolism. Patients in whom the classic atriopulmonary connection Fontan procedure is performed with a dilated right atrium may benefit from conversion to a lateral tunnel or extracardiac Fontan operation to treat both arrhythmia and thrombosis.

Protein-losing enteropathy remains a difficult problem, affecting as many as 5% of patients who undergo the Fontan procedure. Treatment with intravenous infusions of albumin and immunoglobulin are supportive but not curative. Several other methods, including intravenous heparin, Fontan takedown, and cardiac transplantation, have been used with varying success.

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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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