eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Tetralogy of Fallot With Absent Pulmonary Valve: Treatment & Medication

Author: Prema Ramaswamy, MD, Co-director of Pediatric Cardiology, Maimonides Medical Center; Assistant Professor, Department of Pediatrics, Mount Sinai School of Medicine
Coauthor(s): Kurt Pflieger, MD, FAAP, Active Staff, Department of Pediatrics, Lake Pointe Medical Center
Contributor Information and Disclosures

Updated: Jul 17, 2008

Treatment

Medical Care

Pulmonary complications are the common cause of infant mortality.

  • 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 infection is particularly hazardous for these patients.
  • Placing the infant in a prone position may be beneficial for respiratory effort. However, this sleeping position is not recommended unsupervised because it increases risk of sudden infant death syndrome. It has been reported to be helpful both preoperatively and postoperatively.18 Takabayashi et al have recommended its use along with bilateral pillows to avoid compression of the sternum.19
  • Maturation of the tracheobronchial tree in infants older than one 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.

Surgical Care

In asymptomatic infants and those with only mild symptoms, surgery is usually deferred until later in childhood.20 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. 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 above, 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 the type of homograft that is best suited. Currently, most centers advocate the use of an aortic homograft.22,23,17 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
  • In a recent article, external stenting of the airway along with intracardiac repair, another ingenious approach, was described.26 This group placed a separate graft and patch around the respiratory tract. They point out that suturing the first graft on the border region between cartilaginous portion and membranous portion is important and not to completely encircle the trachea because this may then hamper growth of the airway. They argue that external stenting of the airway was likely to be more effective that endobronchial stenting.
  • Repair in the critically ill neonate is urgent, high risk, fraught with postoperative complications, and carries a high mortality rate. However, 2 surgical reviews have 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.24,20

Consultations

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

Medication

No specific medications for this disease are indicated. Anticongestive therapy is of limited benefit in the treatment of heart failure.

Inotropic agents

Positive inotropic agents increase the force of contraction of the myocardium and are used to treat acute and chronic CHF. Some may also increase or decrease the heart rate (ie, positive or negative chronotropic agents), provide vasodilatation, or improve myocardial relaxation. These additional properties influence the choice of drug for specific circumstances.


Digoxin (Lanoxin)

Cardiac glycoside with direct inotropic effects in addition to indirect effects on the cardiovascular system. Acts directly on cardiac muscle, increasing myocardial systolic contractions. Its indirect actions result in increased carotid sinus nerve activity and enhanced sympathetic withdrawal for any given increase in mean arterial pressure.

Adult

0.125-0.25 mg/d PO

Pediatric

3.5-5 mcg/kg/dose PO bid

Multiple drug interactions, especially with antiarrhythmic agents and any agent that may reduce renal function; IV calcium may produce arrhythmias in digitalized patients
Medications that may increase digoxin levels include alprazolam, benzodiazepines, bepridil, captopril, cyclosporine, propafenone, propantheline, quinidine, diltiazem, aminoglycosides, PO amiodarone, anticholinergics, diphenoxylate, erythromycin, felodipine, flecainide, hydroxychloroquine, itraconazole, nifedipine, omeprazole, quinine, ibuprofen, indomethacin, esmolol, tetracycline, tolbutamide, and verapamil; medications that may decrease serum digoxin levels include aminoglutethimide, antihistamines, cholestyramine, neomycin, penicillamine, aminoglycosides, PO colestipol, hydantoins, hypoglycemic agents, antineoplastic treatment combinations (eg, carmustine, bleomycin, methotrexate, cytarabine, doxorubicin, cyclophosphamide, vincristine, procarbazine), aluminum or magnesium antacids, rifampin, sucralfate, sulfasalazine, barbiturates, kaolin/pectin, and aminosalicylic acid

Documented hypersensitivity; severe hypokalemia; renal failure; WPW syndrome with antegrade conduction of the accessory pathway

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Check renal function and previously prescribed medications prior to starting digoxin therapy; hypokalemia may reduce positive inotropic effect of digitalis; hypercalcemia predisposes patient to digitalis toxicity, and hypocalcemia can make digoxin ineffective until serum calcium levels are within the reference range; magnesium replacement therapy must be instituted in patients with hypomagnesemia to prevent digitalis toxicity; patients diagnosed with incomplete AV block may progress to complete block when treated with digoxin; exercise caution in hypothyroidism, hypoxia, and acute myocarditis; adjust dose in renal impairment; highly toxic (overdoses can be fatal)

Diuretic agents

These agents promote excretion of water and electrolytes by the kidneys. They are used to decrease pulmonary or systemic edema.


Furosemide (Lasix)

Increases excretion of water by interfering with chloride-binding cotransport system which, in turn, inhibits sodium and chloride reabsorption in ascending loop of Henle and distal renal tubule.

Adult

20-80 mg/d PO divided tid/qid

Pediatric

0.5-1 mg/kg/dose PO tid/qid

Metformin decreases furosemide concentrations; furosemide interferes with hypoglycemic effect of antidiabetic agents and antagonizes muscle-relaxing effect of tubocurarine; auditory toxicity appears to be increased with coadministration of aminoglycosides and furosemide; hearing loss of varying degrees may occur; anticoagulant activity of warfarin may be enhanced when taken concurrently with this medication; increased plasma lithium levels and toxicity are possible when taken concurrently with this medication

Documented hypersensitivity; severe hypovolemia; severe electrolyte imbalance; hepatic coma; anuria

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Monitor electrolytes, serum glucose, and volume status carefully; may increase risk of renal stones

More on Tetralogy of Fallot With Absent Pulmonary Valve

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References
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References

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Further Reading

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Keywords

tetralogy of Fallot, TOF, absent pulmonary valve syndrome, TOF with absent pulmonary valve, Fallot tetralogy, Fallot's tetralogy, Fallot tetrad, Fallot's tetrad, ventricular septal defect, infundibular pulmonary stenosis, pulmonary regurgitation, cyanotic heart disease, hypoxemia, heart failure, respiratory failure, patent ductus arteriosus

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Contributor Information and Disclosures

Author

Prema Ramaswamy, MD, Co-director of Pediatric Cardiology, Maimonides Medical Center; Assistant Professor, Department of Pediatrics, Mount Sinai School of Medicine
Prema Ramaswamy, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Cardiology
Disclosure: Nothing to disclose.

Coauthor(s)

Kurt Pflieger, MD, FAAP, Active Staff, Department of Pediatrics, Lake Pointe Medical Center
Kurt Pflieger, MD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American College of Emergency Physicians, American Heart Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Ira H Gessner, MD, Professor Emeritus, Pediatric Cardiology
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.

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

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.

CME Editor

Gilbert Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College
Gilbert 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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