eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology
Tetralogy of Fallot With Pulmonary Atresia: Treatment & Medication
Updated: Nov 24, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
- Newborn infants with cyanosis due to congenital heart disease almost always benefit from administration of prostaglandin E1 (PGE1) to maintain ductal patency while a definitive diagnosis is made. Once the diagnosis of tetralogy of Fallot with pulmonary atresia (TOF-PA) is made, maintain PGE1 infusion through initial surgery.
- Older infants with increased pulmonary blood flow may require treatment for heart failure.
Surgical Care
Neonates with adequate-sized confluent pulmonary arteries may be amenable to primary definitive surgical repair. A palliative procedure with a systemic–to–pulmonary artery shunt may be performed while awaiting complete repair at a later date. The ultimate surgical goals are to incorporate as many pulmonary artery segments as possible into a pulmonary artery confluence, to place a conduit from the right ventricle to the pulmonary artery confluence, and to close the ventricular septal defect (VSD).
- When the pulmonary arteries are hypoplastic, nonconfluent, and supplied by aortopulmonary collaterals, a multistaged repair is often required.12 Hypoplastic pulmonary arteries generally require palliative shunting to induce enlargement and growth of these vessels so they can be successfully incorporated into the complete repair. The shunts used may be modified Blalock-Taussig or central shunts and may be unilateral or bilateral. If the pulmonary arteries have grown after placement of the palliative shunts, unifocalization of the pulmonary arteries can be performed; this is done by incorporating the aortopulmonary collaterals and connecting them to the conduit from the right ventricle.
- For complete repair to be performed in a child who has undergone palliation, the central pulmonary arterial area must be greater than 50% of normal; predominantly left-to-right intracardiac shunting must be present; the equivalent of an entire lung must be supplied by the central pulmonary artery confluence; and stenotic lesions in the pulmonary artery outflow must be addressed. The choice of the optimal type of conduit for a growing child remains controversial. Current options include cryopreserved aortic or pulmonary homografts, glutaraldehyde fixed bovine jugular vein grafts, and synthetic conduits, with variable intermediate-term results reported in the medical literature.13,14
- Some centers have shifted toward performing a single-stage repair, wherein all the multiple aortopulmonary collaterals (MAPCAs) are ligated at the aorta.15,16 These MAPCAs are then mobilized toward the posterior mediastinum to construct a pulmonary artery confluence, followed by insertion of a pulmonary allograft to establish continuity between these neopulmonary arteries and the right ventricle. The VSD is closed. These centers have reported good results. Infants with postunifocalization pulmonary arteries that, combined, are only mildly hypoplastic (>200 mm2/m2) have a lower mortality rate and acceptable right ventricular pressures. However, most patients require repeat catheterizations for balloon dilation or stent placements in stenotic pulmonary artery segments to alleviate elevated right ventricular pressures.
Consultations
- Pediatric cardiology consultation is advised.
- Consult a geneticist to evaluate the presence of syndromic associations and gene deletions, especially in the presence of associated anomalies or dysmorphic features.
- Once the anatomy of a child with tetralogy of Fallot with pulmonary atresia is determined by echocardiography and angiography findings, consultation with a cardiovascular surgeon is required. The caregivers need to be aware of the possibility of a multistage repair and repeated surgeries and catheterizations.
- If anomalies involving other systems are present, consultations and follow-up with the appropriate specialists are required.
Diet
- Infants who are born with multiple systemic-to-pulmonary collaterals and are in cardiac failure because of pulmonary overcirculation require caloric supplementation to establish a normal growth pattern. Caloric intake as high as 130-150 kcal/kg/d may be required.
- Children that undergo palliative procedures also require optimization of their caloric intake. Adequate nutritional supplementation in the form of total parental nutrition must also be ascertained in the perioperative period. These patients often have a prolonged postoperative recovery course.
Activity
Exercise tolerance and need for restrictions on physical activity depend on the type of repair and hemodynamic state of the patient.
- Patients with cyanosis have significantly limited exercise capacity.
- Children and adults who have had complete repair of tetralogy of Fallot with pulmonary atresia may have limited exercise tolerance due to ventricular dysfunction, chronotropic impairment, right ventricular outflow tract obstruction/conduit stenosis, or distal pulmonary artery stenoses.
Medication
Newborns with tetralogy of Fallot with pulmonary atresia (TOF-PA) may require the ductus arteriosus (DA) as the source of pulmonary blood flow. A prostaglandin E1 (PGE1; Alprostadil) infusion maintains patency of the ductus.
Infants with multiple systemic pulmonary collaterals may develop symptomatic heart failure requiring medical therapy.
Prostaglandins
PGE1 (Alprostadil) promotes dilatation of the DA in infants with ductal-dependent cardiac abnormalities. It is also a vasodilator.
Alprostadil (Prostin VR Pediatric Injection)
First-line palliative therapy to temporarily maintain patency of DA before surgery. Beneficial in infants who have congenital defects that restrict pulmonary or systemic blood flow and who depend on a patent DA for adequate oxygenation and lower body perfusion. Produces vasodilation and increases cardiac output. Each 1-mL ampule contains 500 mcg/mL.
Adult
Not indicated
Pediatric
Initial dose: 0.05-0.1 mcg/kg/min IV
Maintenance dose: 0.01-0.4 mcg/kg/min IV
Infuse IV into large vein or umbilical cord
Limited data exist; use caution with concurrent use of antiplatelet drugs or anticoagulants
Documented hypersensitivity; hyaline membrane disease; respiratory distress syndrome
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Adverse effects and toxicity include apnea, seizures, fever, hypotension, leukocytosis, fever, and pulmonary overcirculation; neonates are usually intubated prophylactically because of potential risk of apnea (10-12%); prolonged use is occasionally necessary (in hypoplastic left heart syndrome transplant candidates) and may be associated with third spacing of fluid; monitor blood oxygenation and arterial pressure
Diuretic agents
These agents promote excretion of water and electrolytes by the kidneys. They are used to treat heart failure or hepatic, renal, or pulmonary disease when sodium and water retention results in edema or ascites. Children who have congestive heart failure (CHF) symptoms often require multiple diuretics for effective control.
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. Individualize dose to patient. Depending on response, administer adult doses at increments of 20-40 mg, no sooner than 6-8 h after previous dose, until desired diuresis occurs. When treating infants, titrate with 1-mg/kg/dose increments until satisfactory effect achieved.
Adult
20-80 mg/d PO/IV/IM; titrate up to 600 mg/d for severe edematous states
Pediatric
1-2 mg/kg/dose PO; not to exceed 6 mg/kg/dose; do not administer more frequently than q6h
1 mg/kg/dose IV/IM slowly under close supervision; not to exceed 6 mg/kg/d
Metformin decreases concentrations; interferes with hypoglycemic effect of antidiabetic agents and antagonizes muscle-relaxing effect of tubocurarine; auditory toxicity appears to be increased with coadministration with aminoglycosides; 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; hepatic coma; anuria; severe electrolyte depletion
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
Perform frequent serum electrolyte, CO2, glucose, creatinine, uric acid, calcium, and BUN determinations during first few months of therapy and periodically thereafter
Spironolactone (Aldactone)
For management of edema resulting from excessive aldosterone excretion. Competes with aldosterone for receptor sites in distal renal tubules, increasing water excretion while retaining potassium and hydrogen ions.
Adult
25-200 mg/d PO qd or divided bid
Pediatric
1.5-3.5 mg/kg/d PO qd or divided q6-12h
May decrease effect of anticoagulants; potassium and potassium-sparing diuretics may increase toxicity of spironolactone
Documented hypersensitivity; anuria; renal failure; hyperkalemia
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Caution in renal and hepatic impairment
Hydrochlorothiazide (HydroDIURIL, Esidrix, Microzide)
Inhibits reabsorption of sodium in distal tubules, causing increased excretion of sodium and water as well as potassium and hydrogen ions.
Adult
25-100 mg PO qd; not to exceed 200 mg/d
Pediatric
<6 months: 2-3 mg/kg/d PO divided bid
>6 months: 2 mg/kg/d PO divided bid
May decrease effects of anticoagulants, antigout agents, and sulfonylureas; thiazides may increase toxicity of allopurinol, anesthetics, antineoplastics, calcium salts, loop diuretics, lithium, diazoxide, digitalis, amphotericin B, and nondepolarizing muscle relaxants
Documented hypersensitivity; anuria; renal decompensation
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
Caution in renal disease, hepatic disease, gout, diabetes mellitus, and erythematosus
Inotropic agents
Positive inotropic agents increase the force of contraction of the myocardium and are used to treat acute and chronic CHF. Poor ventricular function may necessitate the use of inotropic medications.
Digoxin (Lanoxin)
Cardiac glycoside with direct inotropic effects and indirect effects on the cardiovascular system. Acts directly on cardiac muscle, increasing myocardial systolic contractions. Indirect actions result in increased carotid sinus nerve activity and enhanced sympathetic withdrawal for any given increase in mean arterial pressure.
Adult
0.125-0.375 mg PO qd
Pediatric
Total digitalizing dose (TDD):
Administer one-half of TDD, then one-fourth TDD at 6-hour to 12-hour intervals
5-10 years: 20-35 mcg/kg PO
>10 years: 10-15 mcg/kg PO
Maintenance dose: Use 25-35% of PO TDD
IV calcium may produce arrhythmias in digitalized patients
Medications that may increase 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 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; beriberi heart disease; idiopathic hypertrophic subaortic stenosis; constrictive pericarditis; carotid sinus syndrome
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
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; 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)
More on Tetralogy of Fallot With Pulmonary Atresia |
| Overview: Tetralogy of Fallot With Pulmonary Atresia |
| Differential Diagnoses & Workup: Tetralogy of Fallot With Pulmonary Atresia |
 Treatment & Medication: Tetralogy of Fallot With Pulmonary Atresia |
| Follow-up: Tetralogy of Fallot With Pulmonary Atresia |
| Multimedia: Tetralogy of Fallot With Pulmonary Atresia |
| References |
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References
Tchervenkov CI, Roy N. Congenital Heart Surgery Nomenclature and Database Project: pulmonary atresia--ventricular septal defect. Ann Thorac Surg. Apr 2000;69(4 Suppl):S97-105. [Medline].
Van Praagh R, Van Praagh S, Nebesar RA, et al. Tetralogy of Fallot: underdevelopment of the pulmonary infundibulum and its sequelae. Am J Cardiol. Jul 1970;26(1):25-33. [Medline].
Perry LW, Neill CA, Ferencz C. Infants with congenital heart disease: the cases. In: Ferencz C, Rubin JD, Loffredo CA, et al. Perspectives in Pediatric Cardiology: Epidemiology of Congenital Heart Disease. Armonk, NY: The Baltimore Washington Infant study; 1997:59-102.
Bertranou EG, Blackstone EH, Hazelrig JB, et al. Life expectancy without surgery in tetralogy of Fallot. Am J Cardiol. Sep 1978;42(3):458-66. [Medline].
Leonard H, Derrick G, O'Sullivan J, Wren C. Natural and unnatural history of pulmonary atresia. Heart. Nov 2000;84(5):499-503. [Medline].
Marino B, Digilio MC, Toscano A, et al. Anatomic patterns of conotruncal defects associated with deletion 22q11. Genet Med. Jan-Feb 2001;3(1):45-8. [Medline].
Digilio MC, Marino B, Grazioli S, et al. Comparison of occurrence of genetic syndromes in ventricular septal defect with pulmonic stenosis (classic tetralogy of Fallot) versus ventricular septal defect with pulmonic atresia. Am J Cardiol. Jun 15 1996;77(15):1375-6. [Medline].
Mackie AS, Gauvreau K, Perry SB, et al. Echocardiographic predictors of aortopulmonary collaterals in infants with tetralogy of fallot and pulmonary atresia. J Am Coll Cardiol. Mar 5 2003;41(5):852-7. [Medline].
Mair DD, Julsrud PR. Diagnostic evaluation of pulmonary atresia and ventricular septal defect cardiac catheterization and angiography. Prog Pediatr Cardiol. 1992;1(1):23-26.
Geva T, Greil GF, Marshall AC, et al. Gadolinium-enhanced 3-dimensional magnetic resonance angiography of pulmonary blood supply in patients with complex pulmonary stenosis or atresia: comparison with x-ray angiography. Circulation. Jul 23 2002;106(4):473-8. [Medline]. [Full Text].
Bernardes RJ, Marchiori E, Bernardes PM, Monzo Gonzaga MB, Simoes LC. A comparison of magnetic resonance angiography with conventional angiography in the diagnosis of tetralogy of Fallot. Cardiol Young. Jun 2006;16(3):281-8. [Medline].
Duncan BW, Mee RB, Prieto LR, et al. Staged repair of tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg. Sep 2003;126(3):694-702. [Medline].
Sierra J, Christenson JT, Lahlaidi NH, Beghetti M, Kalangos A. Right ventricular outflow tract reconstruction: what conduit to use? Homograft or Contegra?. Ann Thorac Surg. Aug 2007;84(2):606-10; discussion 610-1. [Medline].
Niemantsverdriet MB, Ottenkamp J, Gauvreau K, Del Nido PJ, Hazenkamp MG, Jenkins KJ. Determinants of right ventricular outflow tract conduit longevity: a multinational analysis. Congenit Heart Dis. May 2008;3(3):176-84. [Medline].
Lofland GK. The management of pulmonary atresia, ventricular septal defect, and multiple aorta pulmonary collateral arteries by definitive single stage repair in early infancy. Eur J Cardiothorac Surg. Oct 2000;18(4):480-6. [Medline].
Reddy VM, Petrossian E, McElhinney DB, et al. One-stage complete unifocalization in infants: when should the ventricular septal defect be closed?. J Thorac Cardiovasc Surg. May 1997;113(5):858-66; discussion 866-8. [Medline].
Marrelli AJ, Perloff JK, Child JS, Laks H. Pulmonary atresia with ventricular septal defect in adults. Circulation. 1994;89(1):243-51. [Medline].
Dearani JA, Danielson GK, Puga FJ, et al. Late follow-up of 1095 patients undergoing operation for complex congenital heart disease utilizing pulmonary ventricle to pulmonary artery conduits. Ann Thorac Surg. Feb 2003;75(2):399-410; discussion 410-1. [Medline].
Mohammadi S, Belli E, Martinovic I, et al. Surgery for right ventricle to pulmonary artery conduit obstruction: risk factors for further reoperation. Eur J Cardiothorac Surg. Aug 2005;28(2):217-22. [Medline].
Further Reading
Keywords
tetralogy of Fallot, TOF, tetralogy of Fallot with pulmonary atresia, TOF-PA, pulmonary atresia with ventricular septal defect, VSD, end-stage tetralogy of Fallot, Fallot tetralogy, Fallot's tetralogy, Fallot tetrad, Fallot's tetrad, CATCH22 syndrome, cardiac defect, abnormal face, thymic hypoplasia, cleft palate, hypocalcemia, microdeletion of band 22q11, vertebral defects, anal atresia, tracheoesophageal fistula with esophageal atresia, renal and radial anomalies, VATER syndrome, coloboma, heart disease, atresia choanae, retarded growth, retarded development, CNS anomalies, genital hypoplasia, ear anomalies, deafness, CHARGE syndrome, Alagille syndrome, cat's eye syndrome, de Lange syndrome, Klippel-Feil syndrome, trisomy 21, maternal diabetes mellitus, maternal phenylketonuria
