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

Tetralogy of Fallot: Surgical Perspective: Follow-up

Author: Vibhuti N Singh, MD, MPH, FACC, FSCAI, Director, Suncoast Cardiovascular Center; Chair, Cardiology Division and Cath Labs, Department of Medicine, Bayfront Medical Center; Clinical Assistant Professor, Division of Cardiology, University of South Florida College of Medicine
Coauthor(s): Jeffrey P Jacobs, MD, FACS, FACC, FCCP, Clinical Associate Professor, Department of Surgery, University of South Florida College of Medicine; Medical Director, ECMO Program, Division of Thoracic and Cardiovascular Surgery, All Children's Hospital/Bayfront Medical Center
Contributor Information and Disclosures

Updated: Nov 13, 2008

Outcome and Prognosis

The overall outcome after surgical repair of tetralogy of Fallot (TOF) has steadily improved since the technique was initially developed. The continued improvement in outcome can be attributed to improved intraoperative technique, including the avoidance of excessive right ventricular (RV) outflow tract (RVOT) muscle resection, improved cardiopulmonary bypass techniques (especially for infants), and improved postoperative care.

In the early 1980s, the survival rate after tetralogy of Fallot repair was approximately 90%. In the current era, survival to discharge after repair in most reported series is 95-99%.

Late survival was documented in 814 patients undergoing complete repair reported in 1989 at the University of Alabama at Birmingham.6 After complete repair, survival rates at 1 month and at 1 year, 5 years, and 20 years, were 93%, 93%, 92%, and 87%, respectively. These survival rates were only slightly less than those of an age-matched, race-matched, and sex-matched control population. In the current era, late survival should even be better than it was in this historical series.

In the earliest days of surgical repair, postoperative complete heart block was a major problem. The rate of postoperative complete heart block decreased to 5% in earlier series and less than 1% in most recent series.

The Society of Thoracic Surgeons Congenital Heart Surgery Database was used to analyze data from 941 patients undergoing tetralogy of Fallot repair in 1998-2003.7 Tetralogy of Fallot with pulmonary stenosis was present in 888 patients. Tetralogy of Fallot with absent pulmonary valve syndrome was present in 34 patients. Tetralogy of Fallot with common atrioventricular canal (AVSD) was present in 19 patients. The overall survival after discharge was 98.7%. The risk was highest among patients with tetralogy of Fallot with absent pulmonary valve syndrome; they had a survival rate to discharge of only 91.2%. The incidence of insertion of a permanent pacemaker due to heart block was only 0.5%.

Future and Controversies

Tetralogy of Fallot versus double-outlet right ventricle

One area of controversy centers on the differentiation between tetralogy of Fallot (TOF) and double-outlet right ventricle (DORV). The tetralogy of Fallot manuscript of The International Congenital Heart Surgery Nomenclature and Database Project clearly stated that the distinction between DORV and tetralogy of Fallot is controversial.8 Some authors use the term DORV when the pulmonary artery arises from the right ventricle (RV) and when more than 50% of the aorta arises from the RV. Other authors use this term only when the pulmonary artery arises from the RV and when 90% or more of the aorta arises from the RV. Still others use the term only when fibrous continuity is absent between the aortic and mitral valves.

In the DORV manuscript of The International Congenital Heart Surgery Nomenclature and Database Project, DORV is defined as a type of ventriculoarterial connection in which both great vessels arise predominantly from the RV.9 In the tetralogy of Fallot manuscript of The International Congenital Heart Surgery Nomenclature and Database Project, Marshall Jacobs states, "It is inescapable that some hearts will be called tetralogy of Fallot at some centers and DORV at other centers."

Management of late pulmonary insufficiency

After repair of tetralogy of Fallot, many patients present in need of reoperative surgical reconstruction of the RV outflow tract (RVOT). The predominant physiologic lesion is often pulmonary insufficiency but varying degrees of RVOT may also be present. In the past, patients were thought to tolerate pulmonary insufficiency reasonably well. However, in some, the long-term effects of pulmonary insufficiency and subsequent RV dilatation and dysfunction are associated with poor exercise tolerance and increased incidences of arrhythmias and sudden death.

Pulmonary valve insertion or replacement can be performed as treatment for pulmonary insufficiency to improve performance status, optimize hemodynamics, and improve control of arrhythmias. Indications for RVOT reconstruction in this setting and the surgical strategy continue to evolve. Several surgical options for pulmonary valve replacement are available, including the use of aortic and pulmonary homografts, stented and stentless porcine valves, porcine valve conduits, bovine jugular vein conduits, man-made polytetrafluoroethylene pulmonary valves, and even mechanical valves and mechanical valve conduits.

Over the last several years, concerns regarding postoperative pulmonary insufficiency or combined insufficiency and stenosis have increasingly emerged. The brief about patients' tolerance of pulmonary insufficiency after valvectomy and/or transannular patching during repair of tetralogy of Fallot is no longer simply accepted. The sequence of pulmonary insufficiency that causes volume overload leading to RV dilatation and dysfunction has been demonstrated with echocardiography and MRI. Exertional symptoms often follow these objective changes in ventricular function and size and can be documented with and exercise testing. Finally, life-threatening ventricular arrhythmias seem to be associated with relatively severe cases of pulmonary insufficiency and ventricular changes.

RV dilatation and dysfunction are reversible after pulmonary valve replacement. Therefore, as the population of children with repaired congenital heart disease ages, an increasing number of patients will benefit from pulmonary valve insertion. However, recent data suggest a lack of notable recovery of RV indices after pulmonary valve replacement in adults with long-standing pulmonary insufficiency. Therefore, the timing of pulmonary valve replacement is of major importance in the overall maintenance of ventricular function and optimal long-term outcomes. In addition, a program of aggressive pulmonary valve replacement in conjunction with intraoperative cryoablation is effective in decreasing QRS duration and in controlling ventricular arrhythmias in patients with tetralogy of Fallot and severe pulmonary insufficiency.

In general, indications for pulmonary valve replacement are evolving but currently include patients with moderate-to-severe pulmonary insufficiency and/or stenosis and any of the following: exertional symptoms of New York Heart Association (NYHA) class II or worse, RV dysfunction, RV dilatation, decreased performance capacity on exercise testing, ventricular arrhythmias, and/or QRS duration of more than 160 ms.

 


More on Tetralogy of Fallot: Surgical Perspective

Overview: Tetralogy of Fallot: Surgical Perspective
Workup: Tetralogy of Fallot: Surgical Perspective
Treatment: Tetralogy of Fallot: Surgical Perspective
Follow-up: Tetralogy of Fallot: Surgical Perspective
References
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References

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Further Reading

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Keywords

tetralogy of Fallot, TOF, Fallot's tetralogy, Fallot's tetrad, ventricular septal defect, VSD, right ventricular outflow tract obstruction, RVOTO, aorta overriding the ventricular septum, right ventricular hypertrophy, RVH, common atrioventricular canal, atrioventricular septal defect, AVSD, complete AVSD, cyanotic heart defect, absent pulmonary valve syndrome, pulmonary atresia, pulmonary stenosis, infundibular stenosis, polycythemia, growth retardation, brain abscess, stroke, aortic regurgitation, atrial septal defect, secundum atrial septal defect, patent foramen ovale, patent ductus arteriosus 

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Contributor Information and Disclosures

Author

Vibhuti N Singh, MD, MPH, FACC, FSCAI, Director, Suncoast Cardiovascular Center; Chair, Cardiology Division and Cath Labs, Department of Medicine, Bayfront Medical Center; Clinical Assistant Professor, Division of Cardiology, University of South Florida College of Medicine
Vibhuti N Singh, MD, MPH, FACC, FSCAI is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Heart Association, American Medical Association, and Florida Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Jeffrey P Jacobs, MD, FACS, FACC, FCCP, Clinical Associate Professor, Department of Surgery, University of South Florida College of Medicine; Medical Director, ECMO Program, Division of Thoracic and Cardiovascular Surgery, All Children's Hospital/Bayfront Medical Center
Jeffrey P Jacobs, MD, FACS, FACC, FCCP 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, Congenital Heart Surgeons Society, Society of Thoracic Surgeons, and Southern Thoracic Surgical Association
Disclosure: Nothing to disclose.

Medical Editor

Jeff L Myers, MD, PhD, Chief, Pediatric and Congenital Cardiac Surgery, Department of Surgery, Massachusetts General Hospital; Associate Professor of Surgery, Harvard Medical School
Jeff L Myers, MD, PhD is a member of the following medical societies: American College of Surgeons, American Heart Association, and International Society for Heart and Lung Transplantation
Disclosure: Nothing to disclose.

Pharmacy Editor

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

Managing Editor

Robert DB Jaquiss, MD, Professor of Surgery, University of Arkansas for Medical Sciences; Chief, Pediatric Cardiothoracic Surgery, Arkansas Children's Hospital and Chief, Cardiothoracic Surgery, University of Arkansas for Medical Sciences
Robert DB Jaquiss, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for Thoracic Surgery, American College of Cardiology, American College of Surgeons, American Heart Association, Congenital Heart Surgeons Society, International Society for Heart and Lung Transplantation, and Society of Thoracic Surgeons
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

John Kupferschmid, MD, Director of Congenital Heart Surgery, Department of Surgery, Methodist Children's Hospital at San Antonio
John Kupferschmid, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, Society of Thoracic Surgeons, and Society of Thoracic Surgeons
Disclosure: Nothing to disclose.

 
 
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