eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology
Heterotaxy, Polysplenia: Treatment & Medication
Updated: Apr 22, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
Medical therapy in polysplenia is typically directed at the findings of the initial evaluation. Anticongestive medication is often required in patients with significant left-to-right shunts. Patients with functional asplenia require antibiotic prophylaxis and the pneumococcal vaccine.
Surgical Care
Surgical care is also directed at the findings of the initial evaluation. Patients with significant cardiac disease may require staged palliation or definitive repair, whereas patients with biliary atresia may require initial palliative surgery followed by liver transplantation.
Consultations
Patients with polysplenia syndrome can present with various noncardiac problems, primarily GI tract abnormalities but also genitourinary abnormalities. Patients presenting with evidence of intestinal obstruction, volvulus, or both should have a consultation with a general surgeon and appropriate imaging studies to rule out malrotations. Consultation with an infectious disease specialist may also be warranted if bacterial infection is suspected because patients with polysplenia associated with abnormal splenic function are more susceptible to certain bacterial pathogens.
Medication
Cardiomyopathy with significant left-to-right shunts may require treatment for congestive heart failure. According to American Heart Association guidelines, pneumococcal vaccine and antibiotics for subacute bacterial endocarditis (SBE) prophylaxis are necessary in patients with functional asplenia. Antibiotic prophylaxis is administered to patients before performing procedures that may cause bacteremia. For more information, see Antibiotic Prophylactic Regimens for Endocarditis.
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 has resulted in edema or ascites. The agents may be used as monotherapy or combination therapy to treat hypertension.
Furosemide (Lasix)
Used to treat edema. 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. Dose must be individualized to patient. Depending on response, administer at 20- to 40-mg increments, no sooner than 6-8 h after the previous dose, until desired diuresis occurs. When treating infants, titrate with 1-mg/kg/dose increments until a satisfactory effect is achieved.
Adult
20-80 mg/d PO/IV/IM; may titrate up to 600 mg/d for severe edematous states
Pediatric
3-6 mg/kg/d PO divided tid
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; hepatic coma; anuria; state of 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, carbon dioxide, 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 divided q12-24h
Pediatric
1-3 mg/kg/d PO divided tid
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
Inotropic agents
Positive inotropes increase the force of contraction of the myocardium and are used to treat acute and chronic congestive heart failure. 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. Those used predominantly for their inotropic effects include cardiac glycosides and phosphodiesterase inhibitors.
Digoxin (Lanoxin)
Used to treat congestive heart failure. 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
0.125-0.375 mg PO qd
Pediatric
10 mcg/kg/d PO divided bid
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 (including 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; patients with incomplete atrioventricular (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)
Angiotensin-converting enzyme (ACE) inhibitors
ACE inhibitors are beneficial in all stages of congestive heart failure. Pharmacologic effects result in a decrease in systemic vascular resistance, reducing blood pressure, preload, and afterload. Dyspnea and exercise tolerance are improved. Unlike diuretics, studies demonstrate improvement of survival and reduced progression of mild or moderate heart failure to more severe stages. ACE inhibitors benefit asymptomatic left ventricular dysfunction.
Enalapril (Vasotec)
Used to treat congestive heart failure. Competitive inhibitor of ACE. Reduces angiotensin II levels, decreasing aldosterone secretion.
Adult
2.5-5 mg/d PO, increase prn; dosing range is 10-40 mg/d PO in 1-2 divided doses
1.25 mg/dose IV over 5 min q6h
Pediatric
0.1 mg/kg/d PO initially; may gradually titrate upward; not to exceed 0.5 mg/kg/d PO divided bid
NSAIDs may reduce hypotensive effects of enalapril; ACE inhibitors may increase digoxin, lithium, and allopurinol levels; rifampin decreases enalapril levels; probenecid may increase enalapril levels; hypotensive effects of ACE inhibitors may be enhanced when coadministered with diuretics
Documented hypersensitivity
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
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Category D in second and third trimester of pregnancy; caution in renal impairment, valvular stenosis, or severe congestive heart failure
Vaccines
Active immunization increases resistance to infection. Vaccines consist of microorganisms or cellular components that act as antigens. Administration of the vaccine stimulates the production of antibodies with specific protective properties.
Pneumococcal vaccine (Pneumovax-23, Pnu-Imune 23)
Polyvalent vaccine used for prophylaxis against infection from Streptococcus pneumoniae. Used in populations at increase risk of pneumococcal pneumonia (ie, age >55 y, chronic infection, asplenia, immunocompromise).
Adult
0.5 mL IM/SC once
Pediatric
<2 years: Contraindicated (antibody response is poor in this age group)
>2 years: 0.5 mL IM/SC; repeat dose after 3-5 y for high-risk children (eg, functional or anatomic asplenia, conditions associated with rapid antibody decline after initial vaccination)
Immunosuppressive agents (eg, large amounts of corticosteroids, antimetabolites, alkylating agents, cytotoxic agents) may reduce effectiveness; therapy with immunoglobulin preparations is likely to block the active immunity induced with pneumococcal vaccination, withhold for 3 mo after discontinuation of immunoglobulin therapy
Documented hypersensitivity; hypersensitivity to any component or thimerosal; severe or even moderate febrile illness; age <2 y; thrombocytopenia or any coagulation disorder that would contraindicate IM injection unless potential benefit clearly outweighs risk of administration
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
May cause relapse in patients with stable idiopathic thrombocytopenia purpura; adverse effects include arthralgia, fever, urticaria, and Guillain-Barré syndrome (rarely)
Antibiotics, prophylactic
Antibiotic prophylaxis is administered to patients before performing procedures that may cause bacteremia.
Amoxicillin (Amoxil, Trimox)
Interferes with synthesis of cell wall mucopeptides during active multiplication, resulting in bactericidal activity against susceptible bacteria. Used as prophylaxis in minor procedures.
Adult
2 g PO 1 h before procedure; alternatively, 3 g PO 1 h before procedure, followed by 1.5 g PO 6 h after initial dose
Pediatric
50 mg/kg PO 1 h before procedure; not to exceed 2 g/dose
Reduces efficacy of PO contraceptives
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Adjust dose in renal impairment
Ampicillin (Marcillin, Omnipen)
For prophylaxis in patients undergoing dental, PO, or respiratory tract procedures. Coadministered with gentamicin for prophylaxis in GI or genitourinary procedures.
Adult
2 g IV/IM 30 min before procedure
High-risk patients: 2 g ampicillin IV/IM plus gentamicin 1.5 mg/kg IV 30 min before procedure, followed 6 h later by 1 g ampicillin IV/IM or 1 g amoxicillin PO
Pediatric
50 mg/kg IV/IM 30 min before procedure; not to exceed 2 g/dose
High-risk patients: 50 mg/kg IV/IM ampicillin plus gentamicin 1.5 mg/kg IV 30 min before procedure, followed 6 h later by ampicillin 25 mg/kg IV/IM or amoxicillin 25 mg/kg PO
Probenecid and disulfiram elevate levels; allopurinol decreases effects and has additive effects on ampicillin rash; may decrease effects of PO contraceptives
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Adjust dose in renal failure; evaluate rash and differentiate from hypersensitivity reaction
Clindamycin (Cleocin)
Used in penicillin-allergic patients undergoing dental, PO, or respiratory tract procedures. Useful for treatment against streptococcal and most staphylococcal infections.
Adult
600 mg PO/IV 1 h before procedure and
150 mg PO/IV 6 h after first dose
Pediatric
20 mg/kg PO 1 h or 20 mg/kg IV 30 min before procedure; not to exceed 600 mg/dose
Increases duration of neuromuscular blockade induced by tubocurarine and pancuronium; erythromycin may antagonize effects; antidiarrheals may delay absorption
Documented hypersensitivity; regional enteritis; ulcerative colitis; hepatic impairment; antibiotic-associated colitis
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Adjust dose in severe hepatic dysfunction; no adjustment necessary in renal insufficiency; associated with severe and possibly fatal colitis
Gentamicin (Garamycin)
Aminoglycoside antibiotic for gram-negative coverage. Used in combination with an agent against gram-positive organisms and one that covers anaerobes.
Used in conjunction with ampicillin or vancomycin for prophylaxis in GI or genitourinary procedures.
Adult
1.5 mg/kg IV; not to exceed 120 mg/dose; administer with ampicillin 2 g IV 30 min before procedure
Pediatric
1.5 mg/kg IV; not to exceed 120 mg/dose; administer 30 min before procedure with ampicillin 50 mg/kg IV; not to exceed 2 g/dose
Coadministration with other aminoglycosides, cephalosporins, penicillins, and amphotericin B may increase nephrotoxicity; because aminoglycosides enhance effects of neuromuscular blocking agents, prolonged respiratory depression may occur; coadministration with loop diuretics may increase auditory toxicity of aminoglycosides; possible irreversible hearing loss of varying degrees may occur (monitor regularly)
Documented hypersensitivity; non–dialysis-dependent renal insufficiency
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
Narrow therapeutic index (not intended for long-term therapy); caution in renal failure (patient not on dialysis), myasthenia gravis, hypocalcemia, and conditions that depress neuromuscular transmission; adjust dose in renal impairment
Vancomycin (Vancocin)
Potent antibiotic directed against gram-positive organisms and active against Enterococcus species. Useful in the treatment of septicemia and skin structure infections. Indicated for patients who cannot receive or have not responded to penicillins and cephalosporins or have infections with resistant staphylococci.
Use CrCl to adjust dose in renal impairment. Used in conjunction with gentamicin for prophylaxis in penicillin-allergic patients undergoing GI or genitourinary procedures.
Adult
Dental, PO, or upper respiratory tract surgery: 1 g IV, infused over 1 h, 1 h before procedure
GI/GU procedures: 1 g IV plus gentamicin 1.5 mg/kg IV, infused over 1 h, 1 h before surgery
Pediatric
Dental, PO, or upper respiratory tract surgery: 20 mg/kg IV, infused over 1 h, 1 h before procedure
Erythema, histaminelike flushing, and anaphylactic reactions may occur when administered with anesthetic agents; when taken concurrently with aminoglycosides, risk of nephrotoxicity may increase above that with aminoglycoside monotherapy; effects in neuromuscular blockade may be enhanced when coadministered with nondepolarizing muscle relaxants
Documented hypersensitivity
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 failure or neutropenia; red man syndrome caused by too rapid IV infusion (dose administered over a few min) but rarely happens when dose is administered IV over 2 h or as PO/IP administration; red man syndrome is not an allergic reaction
Cefazolin (Ancef)
First-generation semisynthetic cephalosporin that arrests bacterial cell wall synthesis, inhibiting bacterial growth. Primarily active against skin flora, including Staphylococcus aureus.
Adult
1 g IV/IM within 30 min before procedure
Pediatric
25 mg/kg IV/IM within 30 min before procedure; not to exceed 1 g/dose
Probenecid prolongs effect; coadministration with aminoglycosides may increase renal toxicity; may yield false-positive urine dipstick test results for glucose
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Adjust dose in renal impairment; superinfections and promotion of nonsusceptible organisms may occur with prolonged use or repeated therapy
Cephalexin (Keflex)
First-generation cephalosporin that arrests bacterial growth by inhibiting bacterial cell wall synthesis. Bactericidal activity against rapidly growing organisms. Primary activity against skin flora and used for skin infections or prophylaxis in minor procedures.
Adult
2 g PO 1 h before procedure
Pediatric
50 mg/kg PO 1 h before procedure; not to exceed 2 g/dose
Coadministration with aminoglycosides increases nephrotoxic potential
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Adjust dose in renal impairment
Cefadroxil (Duricef)
First-generation cephalosporin arrests bacterial growth by inhibiting bacterial cell wall synthesis. Bactericidal activity against rapidly growing organisms. Primary activity against skin flora and used for skin infections or prophylaxis in minor procedures.
Adult
2 g PO 1 h before procedure
Pediatric
50 mg/kg PO 1 h before procedure; not to exceed 2 g/dose
Coadministration with furosemide or aminoglycosides may increase nephrotoxicity; probenecid prolongs effects
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Adjust dose in renal impairment; superinfections and promotion of nonsusceptible organisms may occur with prolonged use or repeated therapy
Azithromycin (Zithromax)
Inhibits bacterial growth, possibly by blocking dissociation of peptidyl tRNA from ribosomes, causing RNA-dependent protein synthesis to arrest.
Adult
500 mg PO 1 h before procedure
Pediatric
15 mg/kg PO 1 h before procedure; not to exceed 500 mg/dose
May increase toxicity of theophylline, warfarin, and digoxin; effects are reduced with coadministration of aluminum and/or magnesium antacids; nephrotoxicity and neurotoxicity may occur when coadministered with cyclosporine
Documented hypersensitivity; hepatic impairment; administration with pimozide
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Bacterial or fungal overgrowth may result from prolonged antibiotic use; may increase hepatic enzymes and cholestatic jaundice; caution in patients with impaired hepatic function, prolonged QT intervals, or pneumonia; caution in patients who are hospitalized, geriatric, or debilitated
Clarithromycin (Biaxin)
Inhibits bacterial growth, possibly by blocking dissociation of peptidyl tRNA from ribosomes, causing RNA-dependent protein synthesis to arrest.
Adult
500 mg PO 1 h before procedure
Pediatric
15 mg/kg PO 1 h before procedure; not to exceed 500 mg/dose
Toxicity increases with coadministration of fluconazole, astemizole, and pimozide; effects decrease and GI adverse effects may increase with coadministration of rifabutin or rifampin; may increase toxicity of anticoagulants, cyclosporine, tacrolimus, digoxin, omeprazole, carbamazepine, ergot alkaloids, triazolam, HMG CoA-reductase inhibitors; cardiac arrhythmias may occur with coadministration of cisapride; plasma levels of certain benzodiazepines may increase, prolonging CNS depression; arrhythmias and increases in QTc intervals occur with disopyramide; coadministration with omeprazole may increase plasma levels of both agents
Documented hypersensitivity; coadministration with pimozide
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
Coadministration with ranitidine or bismuth citrate not recommended with CrCl <25 mL/min; administer one half dose or increase dosing interval if CrCl <30 mL/min; diarrhea may be sign of pseudomembranous colitis; superinfections may occur with prolonged or repeated antibiotic therapies
More on Heterotaxy, Polysplenia |
| Overview: Heterotaxy, Polysplenia |
| Differential Diagnoses & Workup: Heterotaxy, Polysplenia |
 Treatment & Medication: Heterotaxy, Polysplenia |
| Follow-up: Heterotaxy, Polysplenia |
| Multimedia: Heterotaxy, Polysplenia |
| References |
| Further Reading |
| « Previous Page | Next Page » |
References
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Kapa S, Gleeson FC, Vege SS. Dorsal pancreas agenesis and polysplenia/heterotaxy syndrome: a novel association with aortic coarctation and a review of the literature. JOP. Jul 9 2007;8(4):433-7. [Medline].
Ferdman B, States L, Gaynor JW, Hedrick HL, Rychik J. Abnormalities of intestinal rotation in patients with congenital heart disease and the heterotaxy syndrome. Congenit Heart Dis. Jan 2007;2(1):12-8. [Medline].
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Cohen MS, Anderson RH, Cohen MI, Atz AM, Fogel M, Gruber PJ, et al. Controversies, genetics, diagnostic assessment, and outcomes relating to the heterotaxy syndrome. Cardiol Young. Sep 2007;17 Suppl 2:29-43. [Medline].
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Bartram U, Wirbelauer J, Speer CP. Heterotaxy syndrome -- asplenia and polysplenia as indicators of visceral malposition and complex congenital heart disease. Biol Neonate. 2005;88(4):278-90. [Medline].
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Chen SJ, Li YW, Wang JK, et al. Usefulness of electron beam computed tomography in children with heterotaxy syndrome. Am J Cardiol. Jan 15 1998;81(2):188-94. [Medline].
Ditchfield MR, Hutson JM. Intestinal rotational abnormalities in polysplenia and asplenia syndromes. Pediatr Radiol. May 1998;28(5):303-6. [Medline].
Gayer G, Apter S, Jonas T, et al. Polysplenia syndrome detected in adulthood: report of eight cases and review of the literature. Abdom Imaging. Mar-Apr 1999;24(2):178-84. [Medline].
Gilljam T, McCrindle BW, Smallhorn JF, et al. Outcomes of left atrial isomerism over a 28-year period at a single institution. J Am Coll Cardiol. Sep 2000;36(3):908-16. [Medline].
Hofstaetter C, Plath H, Hansmann M. Prenatal diagnosis of abnormalities of the fetal venous system. Ultrasound Obstet Gynecol. Mar 2000;15(3):231-41. [Medline].
Ticho BS, Van Praagh R. Inherited structural heart diseases associated with arrhythmias: Defects in laterality. In: Berul CI, Towbin JA, eds. Molecular Genetics of Cardiac Electrophysiology. Boston, MA: Kluwer Academic Publishers; 2000:317-28.
Uemura H, Ho SY, Anderson RH, Yagihara T. Ventricular morphology and coronary arterial anatomy in hearts with isometric atrial appendages. Ann Thorac Surg. May 1999;67(5):1403-11. [Medline].
Uemura H, Ho SY, Devine WA, Anderson RH. Analysis of visceral heterotaxy according to splenic status, appendage morphology, or both. Am J Cardiol. Oct 15 1995;76(11):846-9. [Medline].
Yoo SJ, Kim YM, Choe YH. Magnetic resonance imaging of complex congenital heart disease. Int J Card Imaging. Apr 1999;15(2):151-60. [Medline].
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Further Reading
Clinical guidelines include the following:
- Infectious Diseases Society of America and American Thoracic Society consensus guidelines on the management of community-acquired pneumonia in adults
- American College of Radiology appropriateness criteria for suspected congenital heart disease in the adult
More recent clinical trials include the following:
- Efficacy and safety of clopidogrel In neonates and infants with systemic to pulmonary artery shunt palliation
- Iron prophylaxis for anemia in infants with cyanotic congenital heart disease
Related eMedicine topics include the following:
Keywords
polysplenia, heterotaxy syndrome, left atrial isomerism, polysplenia syndrome, cyanotic congenital heart disease, biliary atresia, intestinal malrotation, functional asplenia, jaundice, congenital heart disease, sepsis, dextrocardia, treatment, diagnosis
