Tetralogy of Fallot With Absent Pulmonary Valve Treatment & Management

  • Author: Prema Ramaswamy, MD; Chief Editor: Stuart Berger, MD   more...
 
Updated: Aug 8, 2011
 

Surgical Intervention

Critically ill patients require urgent surgical repair. In asymptomatic infants and those with only mild symptoms, surgery is usually deferred until later in childhood.[20]

Consultation with a pediatric cardiologist and a pediatric cardiothoracic surgeon is essential.

Repair techniques and controversies

Surgical repair techniques vary in accordance with the particular anatomy in a given patient, especially the severity of pulmonary artery dilation.

Definitive repair includes intracardiac repair of as well as the elimination of bronchial compression. Therefore, any intracardiac surgery needs to be accompanied with excision of the main, right, and left pulmonary arteries.[21] However, this is not always uniformly helpful because, as mentioned earlier, abnormalities may be present at the pulmonary arteriolar level.

Another area of controversy is whether to insert a valve in the pulmonic position via a homograft and which type of homograft that is best suited. Currently, most centers advocate the use of an aortic homograft.[17, 22, 23] However, one report argues that the use of a valveless right ventricle–to–pulmonary artery connection, combined with catheter-based intervention, reduces the likelihood of reoperation necessitated by homograft placement.[24]

Other approaches have been suggested to reduce the bronchial compression. One such approach includes translocation of the pulmonary artery anterior to the aorta and away from the airways.[25]

External airway stenting and intracardiac repair

Sakamoto et al described external stenting of the airway along with intracardiac repair, another ingenious approach.[26] This group placed a separate graft and patch around the respiratory tract. The authors pointed out that suturing the first graft on the border region between the cartilaginous portion and membranous portion is important and not to completely encircle the trachea, because this may then hamper growth of the airway.[26] They argued that external stenting of the airway was likely to be more effective than endobronchial stenting.

Next

Approach Considerations

Pulmonary complications are the common cause of infant mortality in patient with tetralogy of Fallot (TOF) with absent pulmonary valve. In a neonate with respiratory distress, transfer to a tertiary care facility with pediatric critical care specialists, pediatric cardiologists, and pediatric cardiothoracic surgeons is expected.

Exacerbation of emphysematous changes and atelectasis from minor respiratory embarrassment, such as an upper respiratory infection, may cause severe problems in the affected neonate. Respiratory syncytial virus (RSV) infection is particularly hazardous for these patients.

Placing the infant in a prone position may be beneficial for respiratory effort; it has been reported to be helpful both preoperatively and postoperatively.[18] However, this sleeping position is not recommended unsupervised, because it increases risk of sudden infant death syndrome (SIDS). Takabayashi et al have recommended use of the prone position along with bilateral pillows to avoid compression of the sternum.[19]

Maturation of the tracheobronchial tree in infants older than 1 year reduces pulmonary obstructive symptoms, presumably by strengthening the underlying cartilaginous structures.

Patients with severe bronchial obstruction present a distinct management problem. If an infant develops respiratory acidosis with retention of carbon dioxide, assisted ventilation may be indicated; however, mechanical ventilation in these patients is of great concern, because once a patient is dependent on positive pressure ventilation, weaning from the respirator can be very difficult. If used, pressure settings should be as low as possible.

Previous
Next

Postoperative Monitoring

Airway morbidity dictates the postoperative recovery and prognosis of neonates from older patients with tetralogy of Fallot (TOF) with absent pulmonary valve. The overall survival is linked to the airway pathology, which is the cause of morbidity and mortality. Preoperative intubation and ventilation are risk factors predictive of poor outcome. (See Prognosis.)

Repair in the critically ill neonate is urgent, high risk, fraught with postoperative complications, and carries a high mortality rate. However, 2 surgical reviews provided a more optimistic picture when surgical strategies are individualized and combined with aggressive postoperative ventilatory management and additional interventions aiming to relieve airway obstruction.[20, 24]

Surgical repair of aneurysmal pulmonary arteries in infants does not necessarily eliminate respiratory symptoms. These patients require close and regular outpatient follow-up.

Patients in whom repair is successful require regular outpatient visits to monitor right ventricular function, hemodynamics of the homograft, if used, and cardiac rhythm stability.

Previous
Proceed to Medication
 
 
Contributor Information and Disclosures
Author

Prema Ramaswamy, MD  Associate Professor of Clinical Pediatrics, State University of New York Downstate; Adjunct Assistant Clinical Professor of Pediatrics, St George's University School of Medicine; Co-Director of Pediatric Cardiology, Maimonides Medical Center, Lutheran Medical Center, and Coney Island Hospital

Prema Ramaswamy, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Cardiology

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.

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.

Additional Contributors

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author Kurt Pflieger, MD, to the development and writing of the source article.

References
  1. Yeager SB, Van Der Velde ME, Waters BL, Sanders SP. Prenatal role of the ductus arteriosus in absent pulmonary valve syndrome. Echocardiography. Aug 2002;19(6):489-93. [Medline].

  2. Rabinovitch M, Grady S, David I, et al. Compression of intrapulmonary bronchi by abnormally branching pulmonary arteries associated with absent pulmonary valves. Am J Cardiol. Oct 1982;50(4):804-13. [Medline].

  3. Tiller GE, Watson MS, Duncan LM, Dowton SB. Congenital heart defect in a patient with deletion of chromosome 7q. Am J Med Genet. Feb 1988;29(2):283-7. [Medline].

  4. Horigome H, Takano T, Hirano T, et al. Interstitial deletion of the long arm of chromosome 6 associated with absent pulmonary valve. Am J Med Genet. Mar 15 1991;38(4):608-11. [Medline].

  5. Galindo A, Gutierrez-Larraya F, Martinez JM, et al. Prenatal diagnosis and outcome for fetuses with congenital absence of the pulmonary valve. Ultrasound Obstet Gynecol. Jun 23 2006;28(1):32-39. [Medline].

  6. Volpe P, Paladini D, Marasini M, et al. Characteristics, associations and outcome of absent pulmonary valve syndrome in the fetus. Ultrasound Obstet Gynecol. Nov 2004;24(6):623-8. [Medline].

  7. Miyabara S, Ando M, Yoshida K, et al. Absent aortic and pulmonary valves: investigation of three fetal cases with cystic hygroma and review of the literature. Heart Vessels. 1994;9(1):49-55. [Medline].

  8. Iserin L, de Lonlay P, Viot G, et al. Prevalence of the microdeletion 22q11 in newborn infants with congenital conotruncal cardiac anomalies. Eur J Pediatr. Nov 1998;157(11):881-4. [Medline].

  9. Emmanoulides GC, Thanopoulos B, Siassi B, Fishbein M. "Agenesis" of ductus arteriosus associated with the syndrome of tetralogy of Fallot and absent pulmonary valve. Am J Cardiol. Mar 4 1976;37(3):403-9. [Medline].

  10. Ettedgui JA, Sharland GK, Chita SK, et al. Absent pulmonary valve syndrome with ventricular septal defect: role of the arterial duct. Am J Cardiol. Jul 15 1990;66(2):233-4. [Medline].

  11. Berg C, Thomsen Y, Geipel A, Germer U, Gembruch U. Reversed end-diastolic flow in the umbilical artery at 10-14 weeks of gestation is associated with absent pulmonary valve syndrome. Ultrasound Obstet Gynecol. Sep 2007;30(3):254-8. [Medline].

  12. Bergwerff M, DeRuiter MC, Gittenberger-de Groot AC. Comparative anatomy and ontogeny of the ductus arteriosus, a vascular outsider. Anat Embryol (Berl). Dec 1999;200(6):559-71. [Medline].

  13. Lakier JB, Stanger P, Heymann MA, et al. Tetralogy of Fallot with absent pulmonary valve. Natural history and hemodynamic considerations. Circulation. Jul 1974;50(1):167-75. [Medline].

  14. Smith RD, DuShane JW, Edwards JE. Congenital insufficiency of the pulmonary valve,including a case of fetal heart failure. Circulation. 1959;20:554.

  15. Freedom RM, Patel RG, Bloom KR, et al. Congenital absence of the pulmonary valve associated with imperforate membrane type of tricuspid atresia, right ventricular tensor apparatus and intact ventricular septum: a curious developmental complex. Eur J Cardiol. Sep 1979;10(3):171-96. [Medline].

  16. Moon-Grady AJ, Tacy TA, Brook MM, et al. Value of clinical and echocardiographic features in predicting outcome in the fetus, infant, and child with tetralogy of Fallot with absent pulmonary valve complex. Am J Cardiol. Jun 1 2002;89(11):1280-5. [Medline].

  17. Castaneda AR, Jonas RA, Mayer JE. Tetralogy of Fallot. In: Cardiac Surgery of the Neonate and Infant. WB Saunders; 1994:232-3.

  18. Heinemann MK, Hanley FL. Preoperative management of neonatal tetralogy of Fallot with absent pulmonary valve syndrome. Ann Thorac Surg. Jan 1993;55(1):172-4. [Medline].

  19. Takabayashi S, Shimpo H, Miyake Y, et al. Postoperative prone position management of tetralogy of fallot with absent pulmonary valve syndrome. Jpn J Thorac Cardiovasc Surg. Mar 2005;53(3):150-3. [Medline].

  20. Alsoufi B, Williams WG, Hua Z, Cai S, Karamlou T, Chan CC, et al. Surgical outcomes in the treatment of patients with tetralogy of Fallot and absent pulmonary valve. Eur J Cardiothorac Surg. Mar 2007;31(3):354-9; discussion 359. [Medline].

  21. Stellin G, Jonas RA, Goh TH, et al. Surgical treatment of absent pulmonary valve syndrome in infants: relief of bronchial obstruction. Ann Thorac Surg. Oct 1983;36(4):468-75. [Medline].

  22. Karl TR, Musumeci F, de Leval M, et al. Surgical treatment of absent pulmonary valve syndrome. J Thorac Cardiovasc Surg. Apr 1986;91(4):590-7. [Medline].

  23. Snir E, de Leval MR, Elliott MJ, Stark J. Current surgical technique to repair Fallot's tetralogy with absent pulmonary valve syndrome. Ann Thorac Surg. Jun 1991;51(6):979-82. [Medline].

  24. Chen JM, Glickstein JS, Margossian R, Mercando ML, Hellenbrand WE, Mosca RS, et al. Superior outcomes for repair in infants and neonates with tetralogy of Fallot with absent pulmonary valve syndrome. J Thorac Cardiovasc Surg. Nov 2006;132(5):1099-104. [Medline].

  25. Hraska V. Repair of tetralogy of Fallot with absent pulmonary valve using a new approach. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2005;132-4. [Medline].

  26. Sakamoto T, Nagase Y, Hasegawa H, et al. One-stage intracardiac repair in combination with external stenting of the trachea and right bronchus for tetralogy of Fallot with an absent pulmonary valve and tracheobronchomalacia. J Thorac Cardiovasc Surg. Dec 2005;130(6):1717-8. [Medline].

Previous
Next
 
Drawing showing absence of the pulmonary valve with features of tetralogy of Fallot. Note the small nubbins of tissue at the pulmonary valve annulus in the center of the drawing. Characteristic muscular right ventricular hypertrophy and infundibular pulmonary stenosis are present. A right aortic arch, a ventricular septal defect with overriding aortic valve, and massively dilated main and branch pulmonary arteries are present.
Pulmonary artery branching in a healthy person and in a patient with absent pulmonary valve syndrome.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.