Heterotaxy Syndrome and Primary Ciliary Dyskinesia Medication

Updated: Apr 10, 2017
  • Author: Alvin J Chin, MD; Chief Editor: Stuart Berger, MD  more...
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Medication

Medication Summary

Patients with significant left-to-right shunts may benefit from digoxin. Those with severe common atrioventricular valve regurgitation may benefit from vasodilator therapy.

In patients with impaired splenic function, immunization with Haemophilus influenzae vaccine, pneumococcal vaccine, and meningococcal vaccine, as well as antibiotics for subacute bacterial endocarditis (SBE) prophylaxis are necessary. [48, 49]  In a systematic review specifically assessing the risk of infection in heterotaxy syndrome (English language only), investigators noted 42 cases of bacteremia in 32 patients, with more than three quarters (79%) of these involving asplenia. [49] Moreover, patients with heterotaxy syndrome had an increased risk of bacteremia leading to mortality, regardless of the anatomic splenic type. [49] Thus, antibiotic prophylaxis is administered to patients before procedures that may cause bacteremia are performed. For more information, see Antibiotic Prophylactic Regimens for Endocarditis and Infectious Endocarditis.

In addition, because ciliary dysfunction is so common in heterotaxy and primary ciliary dyskinesia, [30] antibiotic therapy should be considered for all patients with respiratory symptoms.

Seasonal flu vaccine and H1N1 vaccine are recommended, especially for those who have undergone Fontan operation.

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Diuretic agents

Class Summary

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. They may be used as monotherapy or in combination 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 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.

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.

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Inotropic agents

Class Summary

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 (eg, 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.

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Angiotensin-converting enzyme (ACE) inhibitors

Class Summary

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. Benefits asymptomatic left ventricular dysfunction.

Enalapril (Vasotec)

Used to treat congestive heart failure. Competitive inhibitor of ACE. Reduces angiotensin II levels, decreasing aldosterone secretion.

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Vaccines

Class Summary

Active immunization increases resistance to infection. Vaccines consist of microorganisms or cellular components, which act as antigens. Administration of the vaccine stimulates the production of antibodies with specific protective properties.

Pneumococcal vaccine polyvalent (Pneumovax-23, Pnu-Imune 23)

Polyvalent vaccine used for prophylaxis against infection from Streptococcus pneumoniae. Used in populations at increased risk of pneumococcal pneumonia (ie, age >55 y, chronic infection, asplenia, immunocompromise).

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Antibiotics, prophylactic

Class Summary

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.

Ampicillin (Marcillin, Omnipen)

For prophylaxis in patients undergoing dental, PO, or respiratory tract procedures.

Coadministered with gentamicin for prophylaxis in GI or genitourinary procedures.

Clindamycin (Cleocin)

Used in penicillin-allergic patients undergoing dental, PO, or respiratory tract procedures. Useful for treatment against streptococcal and most staphylococcal infections.

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.

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.

Cefazolin (Ancef)

First-generation semisynthetic cephalosporin that arrests bacterial cell wall synthesis, inhibiting bacterial growth. Primarily active against skin flora, including Staphylococcus aureus.

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.

Cefadroxil (Duricef)

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.

Azithromycin (Zithromax)

Inhibits bacterial growth, possibly by blocking dissociation of peptidyl tRNA from ribosomes, causing RNA-dependent protein synthesis to arrest.

Clarithromycin (Biaxin)

Inhibits bacterial growth, possibly by blocking dissociation of peptidyl tRNA from ribosomes, causing RNA-dependent protein synthesis to arrest.

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