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

Hypoplastic Left Heart Syndrome: Treatment & Medication

Author: P Syamasundar Rao, MD, Professor of Pediatrics and Medicine, University of Texas-Houston Medical School; Director, Division of Pediatric Cardiology, Children's Memorial Hermann Hospital; Professor of Pediatrics, MD Anderson Cancer Center, University of Texas
Coauthor(s): Daniel R Turner, MD, Associate Professor of Pediatric Cardiology, Wayne State University School of Medicine; Consulting Staff, Carman and Ann Adams Department of Pediatrics, Division of Cardiology, Children's Hospital of Michigan; Thomas J Forbes, MD, FACC, FSCAI, Associate Professor (Clinical-Educator), Director of Catheterization Laboratory, Division of Pediatric Cardiology, Children's Hospital of Michigan, Wayne State University
Contributor Information and Disclosures

Updated: Sep 22, 2009

Treatment

  • No consensus has been reached in the approach to the treatment of neonates with hypoplastic left heart syndrome (HLHS). Supportive care, multistage surgical intervention (ie, Norwood, Glenn, and Fontan procedures) and cardiac transplantation are available options. A thorough explanation of each of these options, including their advantages and disadvantages, should be provided to the parents.
  • Occasionally, some anatomic features favor one choice over the others. In the presence of severe tricuspid or pulmonary valve anomalies, the multistage surgical approach is not likely to be beneficial; cardiac transplantation is the only surgical choice. In most cases, the choice of treatment is based on the parents' preference. While such a decision is being made, the infant should be stabilized.
  • If supportive care is chosen by the parents, they need strong emotional support because the condition is fatal without active treatment.

Medical Care

Successful preoperative management depends on providing adequate systemic blood flow while limiting pulmonary overcirculation.

  • Open the ductus arteriosus
    • Blood flow to the systemic circulation (coronary arteries, brain, liver, kidneys) depends on flow through the ductus arteriosus. If a diagnosis of hypoplastic left heart syndrome is suspected, start prostaglandin E1 infusion immediately to establish ductal patency and ensure adequate systemic perfusion.
    • If the diagnosis is made prenatally or when the infant is relatively asymptomatic, a smaller dose of prostaglandin E1 may be sufficient to keep the ductus arteriosus patent while limiting its side effects.
    • A larger dose of prostaglandin E1 is often required to reopen the ductus arteriosus if an infant has cardiovascular collapse and shock due to ductal closure.
    • Ideally, prostaglandin E1 is administered centrally via an umbilical venous catheter.
  • Correct metabolic acidosis
    • Metabolic acidosis indicates inadequate cardiac output to meet the metabolic demands of the body. Acidosis adversely affects the myocardium.
    • Correction of metabolic acidosis with sodium bicarbonate infusion is essential in early management. This therapy is futile if the ductus arteriosus remains constricted.
  • Manipulate pulmonary vascular resistance
    • The pulmonary vascular resistance of a newborn is slightly less than the systemic vascular resistance and begins to fall soon after birth. In the patient with hypoplastic left heart syndrome, decreased pulmonary vascular resistance causes increased pulmonary blood flow and an undesirable obligatory decrease in systemic blood flow. Increased alveolar oxygen decreases pulmonary vascular resistance, leading to increased pulmonary blood flow. Therefore, oxygen should not be administered unless pulmonary parenchymal disease or pulmonary edema, causing severe hypoxemia, is present. The oxygen should be discontinued once these abnormalities resolve.
    • Consequently, most infants should remain in room air with acceptable oxygen saturation (pulse oximeter) in the low 70s. An exceptional circumstance is the infant with severe hypoxemia caused by pulmonary venous hypertension.
    • Achieving a slightly higher PaCO2, in the range of 45-50 mm Hg, can increase pulmonary vascular resistance. This can be accomplished by intubation, sedation, mechanical hypoventilation, or the addition of nitrogen or carbon dioxide (FIO 2 of 15-19%) to the infant's inspired gas via the endotracheal tube or hood.
    • Intubation is not preferred. However, intubation and ventilation along with measures to balance pulmonary and systemic flows may improve tricuspid regurgitation.41
    • Serial blood gas analysis is necessary. Initially, an umbilical arterial catheter is useful to obtain frequent blood samples.
    • Although administration of subambient inspired oxygen to balance systemic and pulmonary blood flows is an attractive concept and should be applied during stabilization of the neonate, it should not be pursued for long periods because severe pulmonary hypertension may complicate the postoperative course. However, this does not seem to adversely affect the pulmonary vasculature on long-term follow-up.42
  • Inotropes
    • Inotropic support is indicated only in severely ill neonates with concurrent sepsis or profound cardiogenic shock and acidosis.
    • The administration of inotropes can adversely affect the balance between pulmonary and systemic vascular resistance.
    • If needed, wean from inotropic support as soon as the infant is clinically stable.
  • Diuretics
    • Consider diuretics to manage pulmonary overcirculation before surgery.
    • Agents commonly used include furosemide and spironolactone.
  • Antibiotics
    • Antibiotics are indicated if the infant is at risk for antepartum infection.
    • Discontinue antibiotics after obtaining negative blood cultures.

Surgical Care

  • Sinha and associates,15 Caylor and colleagues,43 and Dotty44 and associates proposed various palliative operations; however, survival was not feasible until Norwood and associates3,4 demonstrated that a multistage operative approach could be used to treat hypoplastic left heart syndrome.
  • After Fontan and Kreutzer's initial description of the physiologically corrective operation for tricuspid atresia,45,46 corrective surgery was widely adapted to treat this entity. The concept was extended to treat other cardiac defects with a functionally single ventricle, including hypoplastic left heart syndrome.
  • The originally described Fontan operation consisted of the following:45 (1) superior vena cava–to–right pulmonary artery end-to-end anastomosis (Glenn procedure), (2) anastomosis of the proximal end of the divided right pulmonary artery to the right atrium directly or by means of an aortic homograft (3) closure of the atrial septal defect, (4) insertion of a pulmonary valve homograft into the inferior vena caval orifice, and (5) ligation of the main pulmonary artery, thus completely bypassing the right ventricle.
  • Kreutzer performed anastomosis of the right atrial appendage and pulmonary artery directly or via a pulmonary homograft and closed the atrial septal defect.46 A Glenn procedure was not performed, and a prosthetic valve was not inserted into the inferior vena cava. Fontan's concept was to use the right atrium as a pumping chamber;45 therefore, he inserted prosthetic valves into the inferior vena cava and right atrial–pulmonary artery junction. Kreutzer’s view was that the right atrium may not function as a pump and that the left ventricle functions as a suction pump in the system.46
  • Numerous modifications to the aforementioned procedures were undertaken by these and other workers in the field (see Tricuspid Atresia). Currently, staged total cavopulmonary connection is the procedure of choice.47
  • The goal of surgical reconstruction of hypoplastic left heart syndrome is to eventually separate the pulmonary and systemic circulations by achieving a Fontan circulation. The right ventricle remains the systemic ventricle while blood passively flows to the lungs. This ultimate reconstruction is accomplished in the following 3 stages:
    • Norwood procedure (stage I)
      • This procedure is usually performed during the first weeks of life, after the infant has been stabilized in the neonatal intensive care unit (ICU). The goals of the procedure are (1) to establish reliable systemic circulation without the ductus arteriosus and (2) to provide enough pulmonary blood flow for adequate oxygenation, while simultaneously protecting the pulmonary vascular bed in preparation for stages II and III.
      • The Norwood procedure includes (1) performing an atrial septectomy to provide unrestricted blood flow across the atrial septum, (2) ligating the ductus arteriosus, (3) creating an anastomosis between the main pulmonary artery and the aorta to provide systemic blood flow, (4) eliminating coarctation of the aorta, and (5) placing an aorta–to–pulmonary artery shunt (usually a modified Blalock-Taussig shunt) to provide pulmonary circulation. More recently, connecting a Gore-Tex graft from the right ventricular outflow tract to the pulmonary artery (ie, Sano operation) has been advocated instead of conventional modified Blalock-Taussig shunt;48,49 some surgeons have shown better results with the Sano procedure than with the conventional Norwood approach.50
      • Upon hospital discharge, most infants remain on digoxin to augment cardiac function, on diuretics to help manage right ventricular volume overload, and on aspirin to prevent thrombosis of the shunt. If tricuspid regurgitation is present, use afterload reduction with captopril.3 Oxygen saturation is typically 70-80% in room air.5
    • Bidirectional Glenn procedure (stage II)
      • This procedure is performed approximately 6 months after the Norwood procedure. Before surgery, perform a cardiac catheterization to assess right ventricular function, pulmonary artery anatomy, and pulmonary vascular resistance. If results are favorable, schedule elective surgery.
      • The bidirectional Glenn procedure includes creating an anastomosis between the superior vena cava and the right pulmonary artery, end-to-side so that venous return from the upper body can flow directly into both lungs. In the hemi-Fontan, the superior vena cava–right atrial junction is closed with a patch that is removed during the next stage. Blood from the inferior vena cava continues to drain into the right atrium. The aorta–to–pulmonary artery shunt that was placed at stage I is ligated.
      • When both right and left superior vena cavae are present, bilateral bidirectional Glenn shunts should be performed, especially if the bridging innominate vein is absent or small.
      • At the time of bidirectional Glenn, repair of pulmonary artery narrowing, if present, should be undertaken. Issues related to tricuspid valve regurgitation, restrictive atrial septum and any other abnormalities should also be addressed.
      • At discharge, infants usually remain on digoxin, diuretics, aspirin, and captopril for the reasons mentioned above.
    • Fontan procedure (stage III)
      • The Fontan procedure is performed approximately 12 months after the bidirectional Glenn procedure. Again, catheterization is necessary to ensure that the child is a candidate for surgery.
      • Completion of the Fontan procedure includes directing blood flow from the inferior vena cava to the pulmonary arteries either via a lateral tunnel procedure or via an extracardiac conduit. Extracardiac conduit diversion of inferior vena caval blood into the right pulmonary artery is currently preferred by most surgeons. To address the growth issue related to extracardiac Fontan, some surgeons use autologous pericardial roll grafts. At the conclusion of the procedure, systemic venous blood returns to the lungs passively without passing through a ventricle.
      • Choussat et al's criteria have been modified by many cardiologists and surgeons.51 Patients violating these criteria are at a higher risk for poor prognosis following Fontan operation than patients within the limits set by Choussat. In this high-risk group, the concept of leaving a small atrial septal defect open to facilitate decompression of the right atrium has been advanced. Laks et al advocated closure of the atrial defect by constricting the preplaced suture in the postoperative period,52 whereas Bridges et al used a transcatheter closure technique.53 Although the fenestrated Fontan was initially conceived for high-risk patients, it has since been used in patients with modest or even low risk. Rare reports of cerebrovascular or other systemic arterial embolic events following the fenestrated Fontan procedure tend to contraindicate its use in non–high-risk patients. Some studies suggest routine fenestration is unnecessary54
      • At discharge, most children remain on digoxin, diuretics, aspirin, and captopril if necessary. In an uncomplicated case, most of these medications can be weaned over 6 months following the Fontan operation. Some cardiologists advocate using aspirin indefinitely. Routine use of more aggressive anticoagulation with Coumadin is debated.
  • Heart transplantation is another surgical option.5,6 The infant must remain on prostaglandin E1 infusion to keep the ductus arteriosus patent while waiting for a donor heart to become available. Approximately 20% of infants listed for heart transplantation die while waiting for a suitable donor organ. After successful cardiac transplantation, infants require multiple medications for modulation of the immune system and prevention of graft rejection. Perform frequent outpatient surveillance to identify rejection early and prevent lasting damage to the transplanted heart. Periodic endomyocardial biopsy usually is performed for more precise monitoring.
  • Emerging therapies include the following:
    • Catheter-assisted Fontan.
      • Following Norwood procedure, 2-stage cavopulmonary connection is currently recommended for achieving Fontan circulation. Konert et al proposed a staged surgical-catheter approach;55 they initially perform a modified hemi-Fontan procedure that is later completed by transcatheter methodology. This reduces the total number of operations required.
      • The modified hemi-Fontan procedure involves the usual bidirectional Glenn procedure. The lower end of the divided superior vena cava is anastomosed to the undersurface of the right pulmonary artery. The superior vena cava is then banded around a 16-gauge catheter with 6-0 Prolene slightly above the cavoatrial junction. A lateral tunnel with a Gore-Tex baffle is created, diverting the inferior vena caval blood toward the superior vena cava. The Gore-Tex baffle is then fenestrated with three to five 5-mm holes. Thus, the first stage achieves a physiologic bidirectional Glenn procedure.
      • At the time of the second stage (the transcatheter stage), the superior vena caval constriction is balloon dilated, and fenestrations are closed with devices or by placement of covered stent. These procedures have been performed in a limited number of patients, and preliminary data suggest that the usual post-Fontan operation complications, such as pleural effusion and ascites, have not occurred with this approach. Additional experience is reported;56,57 however, scrutiny of results of larger experience and longer-term follow-up and ready availability of covered stents are necessary for routine application of this innovative approach.
    • Sano’s modification of the Norwood procedure
      • Because of high mortality that Sano and his associates observed with the conventional Norwood procedure, they performed right ventricular outflow–to–pulmonary artery Gore-Tex graft anastomosis to provide for the pulmonary blood flow instead of conventional modified aortopulmonary shunt.48,49 Significant improvement was demonstrated in both immediate and late mortality with this modification.50
      • Currently, an ongoing multi-institutional study is comparing the results of these 2 types of stage I palliation of hypoplastic left heart syndrome.
    • Other hybrid approaches: Again, because of high mortality following stage I Norwood reconstruction, several groups have performed bilateral banding of the branch pulmonary arteries via median sternotomy and implant stent in the ductus arteriosus.58,59 Maintaining ductal patency with long-term prostaglandin infusion instead of ductal stenting is advocated by some. At the time of the second stage, aortic arch reconstruction, atrial septectomy, and bidirectional Glenn shunt are performed. This is followed by Fontan conversion. Although reduction of early mortality is theoretically feasible, larger experience with this approach than is currently available is necessary prior to general adaptation of this method of management of hypoplastic left heart syndrome.
    • Prevention by fetal intervention: Fetal echocardiography studies have shown development of hypoplastic left heart syndrome in fetuses initially found to have severe/critical aortic stenosis. Some data suggest that fetal intervention to relieve aortic valve stenosis (by balloon aortic valvuloplasty) may promote normal development of the left ventricle.60 Further research into this type of approach is needed.

Consultations

  • Consult a pediatric cardiologist.
  • Consult a pediatric cardiovascular surgeon.
  • Consult a genetic specialist if a chromosomal abnormality is suspected.

    Diet

    • Adequate nutrition is important before and after surgery. Many infants require nasogastric feeding with increased-calorie breast milk or formula after the Norwood procedure.61 However, normal oral feeding is reestablished with time. Adequate oral iron intake prevents development of iron deficiency anemia.
    • After completion of the Fontan operation, specific dietary restrictions are not necessary unless protein-losing enteropathy develops.38 In such cases, a medium-chain triglyceride diet may be helpful.

    Activity

    • Specific activity restrictions are not imposed on children after completion of the Fontan operation. In general, encourage children to participate in activities that they are able to tolerate. Children who underwent a Fontan procedure may not be able to tolerate highly competitive sports.
    • Studies have shown that these children may have impaired exercise performance when compared to age-matched peers. Perform an exercise stress test when the child is old enough.
    • Neurodevelopmental abnormalities occur often in patients with hypoplastic left heart syndrome.

    Medication

    Before the Norwood procedure or cardiac transplantation in patients with hypoplastic left heart syndrome (HLHS), treat infants with prostaglandin E1 infusion, diuretics, inotropes, and afterload reduction. Drug management after cardiac transplantation is not discussed in this article.

    Prostaglandins

    Prostaglandin E1 promotes dilatation of the ductus arteriosus in infants with ductal-dependent cardiac abnormalities.


    Alprostadil (Prostaglandin E1, Prostin)

    Causes relaxation of smooth muscle, primarily within the ductus arteriosus. Used in infants with ductal-dependent congenital heart disease due to restricted systemic blood flow. The drug is also useful in neonates with ductal dependent pulmonary circulation.

    Adult

    Pediatric

    0.01-0.1 mcg/kg/min IV infusion

    Coadministration with heparin may increase PTT or PT

    Documented hypersensitivity; respiratory distress syndrome or persistent fetal circulation

    Pregnancy

    X - Contraindicated; benefit does not outweigh risk

    Precautions

    Closely monitor respiratory status, cardiovascular status, and coagulation; apnea, fever, irritability, and cutaneous flushing are common; inhibits platelet aggregation

    Diuretic agents

    These agents decrease preload by increasing free-water excretion. Decreasing preload may improve systolic ventricular function.


    Furosemide (Lasix)

    Loop diuretic that blocks sodium reabsorption in the ascending limb of loop of Henle.

    Adult

    20-80 mg IV/IM/PO up to tid

    Pediatric

    0.5-2 mg/kg IV/IM/PO up to tid

    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

    Documented hypersensitivity; hepatic coma, anuria, and 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

    Profound diuresis and electrolyte loss may result; metabolic alkalosis; use caution with other medications known to decrease renal function; may cause hypercalciuria and renal stones, especially in premature infants


    Spironolactone (Aldactone)

    This drug is a potassium-sparing loop diuretic.

    Adult

    25-100 mg PO divided bid/qid

    Pediatric

    2-3 mg/kg PO qd or divided bid

    May decrease effect of anticoagulants; potassium and potassium-sparing diuretics may increase toxicity of spironolactone

    Documented hypersensitivity; anuria, renal failure or hyperkalemia

    Pregnancy

    D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

    Precautions

    Electrolyte imbalance, especially hyperkalemia, may result; concomitant use with indomethacin or ACE inhibitors may cause hyperkalemia

    Cardiac glycosides

    These medications improve ventricular systolic function by increasing the calcium supply available for myocyte contraction.


    Digoxin (Lanoxin)

    This form inhibits the sodium-potassium ATPase pump in cardiac myocytes.

    Adult

    Total digitalizing dose (TDD): 1-1.5 mg PO given in divided doses over 1 d
    Maintenance dose: 0.125-0.375 mg PO in 1-2 doses

    Pediatric

    TDD:
    For more rapid action, most pediatric cardiologists recommend 50% of TDD dose, then followed with 25% TDD q8h x2 more doses
    If administered IV, give only 75% of PO dose
    Premature infants: 0.02 mg/kg PO
    Full-term infants: 0.03 mg/kg PO
    1-24 months: 0.04-0.05 mg/kg PO
    >2 years: 0.03-0.04 mg/kg PO

    Maintenance dose:
    Infants: 6-8 mcg/kg/d PO divided q12h
    2-5 years: 10-15 mcg/kg/d PO divided q12h
    5-10 years: 7 to 10 mcg/kg/d PO divided q12h
    >10 years: 3-5 mcg/kg PO daily
    <10 years: Administer calculated daily dose in 2 equally divided doses (ie, bid)

    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, and 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; IV calcium may produce arrhythmias in digitalized patients; hypercalcemia predisposes patient to digitalis toxicity, and hypocalcemia can make digoxin ineffective until serum calcium levels are normal; magnesium replacement therapy must be instituted in patients with hypomagnesemia to prevent digitalis toxicity; patients with incomplete AV block may progress to complete block when treated with digoxin; exercise caution in hypothyroidism, hypoxia, and acute myocarditis

    Inotropic agents

    These agents stimulate alpha-adrenergic and beta-adrenergic and beta-dopaminergic receptors in the heart and vascular bed.


    Dopamine (Intropin)

    At lower doses, stimulation of beta1-adrenergic and beta1-dopaminergic receptors results in positive inotropism and renal vasodilatation; at higher doses, stimulation of alpha-adrenergic receptors results in peripheral and renal vasoconstriction.

    Adult

    2-20 mcg/kg/min IV infusion

    Pediatric

    Administer as in adults

    Phenytoin, alpha- and beta-adrenergic blockers, general anesthesia, and MAOIs increase and prolong effects of dopamine

    Documented hypersensitivity; pheochromocytoma or ventricular fibrillation

    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

    Use caution with intravascular volume depletion; administration via a central venous catheter is recommended; the umbilical artery should not be used; doses higher than 20 mcg/kg/min generally are not helpful and other agents should be considered; subcutaneous infiltration may cause tissue sloughing; prompt treatment with subcutaneous phentolamine (Regitine) is recommended


    Dobutamine (Dobutrex)

    This drug primarily stimulates the beta1-adrenergic receptor and has less alpha-adrenergic stimulation, leading primarily to increased myocardial contractility.

    Adult

    2-20 mcg/kg/min IV infusion

    Pediatric

    Administer as in adults

    Beta-adrenergic blockers antagonize effects of dobutamine; general anesthetics may increase toxicity

    Documented hypersensitivity; idiopathic hypertrophic subaortic stenosis and atrial fibrillation or flutter

    Pregnancy

    B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

    Precautions

    Use caution with intravascular volume depletion; administration via a central venous catheter is recommended; the umbilical artery should not be used; doses higher than 20 mcg/kg/min generally are not helpful, and other agents should be considered; subcutaneous infiltration may cause tissue ischemia

    Afterload-reducing agents

    Afterload reduction improves myocardial performance and theoretically reduces atrioventricular and semilunar valve insufficiency.


    Captopril (Capoten)

    ACE inhibitor, which decreases the production of angiotensin II, a potent vasoconstrictor, resulting in peripheral vasodilatation and afterload reduction, improving myocardial performance and theoretically reducing AV and semilunar valve insufficiency.
    Administer a test dose of 0.1 mg PO to assess initial response

    Adult

    6.25-12.5 mg PO tid; not to exceed 150 mg tid

    Pediatric

    0.1-1 mg/kg PO tid

    NSAIDs may reduce hypotensive effects of captopril; ACE inhibitors may increase digoxin, lithium, and allopurinol levels; rifampin decreases captopril levels; probenecid may increase captopril levels; the hypotensive effects of ACE inhibitors may be enhanced when given concurrently with diuretics

    Documented hypersensitivity; renal impairment

    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

    Pregnancy category D in second and third trimesters; caution in renal impairment, valvular stenosis, or severe congestive heart failure; profound hypotensive response is observed rarely after the initial dose in smaller children; an initial test dose should be given and blood pressure should be monitored carefully; dose should be titrated based on clinical response and tolerance; use caution with decreased renal function; ACE inhibitors have a potassium-sparing effect when administered with furosemide; simultaneous administration of spironolactone should be done with caution

    Antiplatelet agents

    These agents are used in the treatment or prevention of thrombo-occlusive disease mediated by the action of platelets. They inhibit platelet function by blocking cyclooxygenase and subsequent aggregation.


    Aspirin (Anacin, Ascriptin, Bayer Aspirin)

    Inhibits the enzyme cyclooxygenase that reduces production of thromboxane A2, which is a potent vasoconstrictor and platelet-aggregating agent.
    Antiplatelet effects of aspirin last the entire life of the platelet (6-10 d) and are not reversible.

    Adult

    325 mg PO qd

    Pediatric

    5-10 mg/kg PO qd

    Effects may decrease with antacids and urinary alkalinizers; corticosteroids decrease salicylate serum levels; additive hypoprothrombinemic effects and increased bleeding time may occur with coadministration of anticoagulants; may antagonize uricosuric effects of probenecid and increase toxicity of phenytoin and valproic acid; doses >2 g/d may potentiate glucose-lowering effect of sulfonylurea drugs

    Documented hypersensitivity; liver damage, hypoprothrombinemia, vitamin K deficiency, bleeding disorders, asthma; because of association of aspirin with Reye syndrome, do not use in children (<16 y) with flu

    Pregnancy

    D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

    Precautions

    May cause transient decrease in renal function and aggravate chronic kidney disease; avoid use in patients with severe anemia, with a history of blood coagulation defects, or who are taking anticoagulants

More on Hypoplastic Left Heart Syndrome

Overview: Hypoplastic Left Heart Syndrome
Differential Diagnoses & Workup: Hypoplastic Left Heart Syndrome
Treatment & Medication: Hypoplastic Left Heart Syndrome
Follow-up: Hypoplastic Left Heart Syndrome
Multimedia: Hypoplastic Left Heart Syndrome
References
Further Reading
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Keywords

hypoplastic left heart syndrome, HLHS, prostaglandin, PGE, prostaglandin E1, PGE1, Fontan, hemi-Fontan, pre-Fontan, Norwood, Sano, hybrid, hypoplasia of the aortic tract complex, patent foramen ovale, atrial septal defect, patent ductus arteriosus, pulmonary stenosis, endocardial fibroelastosis, metabolic acidosis, oliguria, tricuspid regurgitation, hypothermia, tachycardia, respiratory distress, hepatosplenomegaly, treatment, diagnosis

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Contributor Information and Disclosures

Author

P Syamasundar Rao, MD, Professor of Pediatrics and Medicine, University of Texas-Houston Medical School; Director, Division of Pediatric Cardiology, Children's Memorial Hermann Hospital; Professor of Pediatrics, MD Anderson Cancer Center, University of Texas
P Syamasundar Rao, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, American Pediatric Society, Medical Association of Georgia, Society for Cardiac Angiography and Interventions, Society for Pediatric Research, Southern Society for Pediatric Research, and Western Society for Pediatric Research
Disclosure: Nothing to disclose.

Coauthor(s)

Daniel R Turner, MD, Associate Professor of Pediatric Cardiology, Wayne State University School of Medicine; Consulting Staff, Carman and Ann Adams Department of Pediatrics, Division of Cardiology, Children's Hospital of Michigan
Daniel R Turner, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Thomas J Forbes, MD, FACC, FSCAI, Associate Professor (Clinical-Educator), Director of Catheterization Laboratory, Division of Pediatric Cardiology, Children's Hospital of Michigan, Wayne State University
Thomas J Forbes, MD, FACC, FSCAI is a member of the following medical societies: American College of Cardiology, American Heart Association, and Society of Cardiac Angiography and Interventions
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 financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

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 Z Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center
Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Chief Editor

Steven R Neish, MD, SM, Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine
Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association
Disclosure: Nothing to disclose.

 
 
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