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

Pulmonary Atresia With Intact Ventricular Septum: Treatment & Medication

Author: John R Charpie, MD, PhD, Associate Professor, Department of Pediatrics, University of Michigan Medical Center
Contributor Information and Disclosures

Updated: Apr 17, 2009

Treatment

Medical Care

  • Initial treatment of pulmonary atresia with intact ventricular septum (PAIVS) consists of maintaining ductal patency with continuous intravenous prostaglandin E1 infusion.
  • To correct metabolic acidosis in a neonate, replace fluids and administer sodium bicarbonate.
  • Mechanical ventilation may be necessary if acidosis persists.
  • Patients ultimately require surgical palliation or therapeutic catheterization prior to hospital discharge.

Surgical Care

Surgical algorithms for PAIVS depend on the size and morphology of both the tricuspid valve and the right ventricle, as well as the presence of abnormal coronary artery anatomy.

  • Mild tricuspid valve and right ventricular hypoplasia without ventriculocoronary connections
    • Perform a surgical valvotomy or transannular patch, with or without a systemic-to-pulmonary artery shunt, or a transcatheter valvotomy, with or without stenting of the patent ductus arteriosus.
    • If the right ventricle and tricuspid valve grow, a 2-ventricle correction is probable in the future.
    • One-stage definitive repair has been described in 2 infants.3 The repair comprised resection of hypertrophied muscles in the outflow and trabecular portions of the right ventricle (right ventricular overhaul technique), surgical valvotomy or transannular patch, and adjustable snare-closure of the foramen ovale.
  • Moderate-to-severe tricuspid valve and right ventricular hypoplasia without ventriculocoronary connections
    • Perform a surgical valvotomy or transannular patch with a systemic-to-pulmonary artery shunt or a transcatheter valvotomy with stenting of the patent ductus arteriosus.
    • Future univentricular (Fontan) repair is likely.
  • Moderate-to-severe tricuspid valve and right ventricular hypoplasia with ventriculocoronary connections but no stenoses or interruption
    • Perform a surgical valvotomy or transannular patch with a systemic-to-pulmonary artery shunt or a transcatheter valvotomy with stenting of the patent ductus arteriosus.
    • Future univentricular (Fontan) repair is likely.
  • Moderate-to-severe tricuspid valve and right ventricular hypoplasia with ventriculocoronary connections and proximal stenoses or interruption
    • Perform a systemic-to-pulmonary artery shunt or stenting of the patent ductus arteriosus.
    • Future univentricular (Fontan) repair or heart transplant is likely.

Consultations

  • Pediatric cardiologist
  • Pediatric cardiothoracic surgeon

Diet

  • Patients with PAIVS require increased caloric density during infancy to provide 120-130 kcal/kg/d for approximately 6 months.

Activity

  • No specific activity restrictions are necessary.4

Medication

No specific drug therapies address pulmonary atresia with intact ventricular septum (PAIVS). Following initial palliation and maintenance of ductal patency with alprostadil (PGE1), some patients may benefit from digoxin and diuretic therapy to improve left ventricular contractility and to avoid fluid retention. Patients with stents should receive low-dose aspirin therapy.

Inotropic agents

These agents increase the contractility of cardiac muscle in a dose-dependent manner (ie, positive inotropic effect).


Digoxin (Lanoxin)

Frequently used cardiac glycoside that inhibits sarcolemmal Na-K adenosine triphosphatase, which leads to an increase in intracellular Ca concentration and increased myocardial contractility.

Adult

0.125-0.5 mg PO qd

Pediatric

Preterm infant: 5-7.5 mcg/kg PO divided bid
Term infant: 6-10 mcg/kg PO divided bid
1 month to 2 years: 10-15 mcg/kg PO divided bid
2-5 years: 7.5-10 mcg/kg PO divided bid
5-10 years: 5-10 mcg/kg PO divided bid
>10 years: 2.5-5 mcg/kg PO qd

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; atrioventricular block, idiopathic hypertrophic subaortic stenosis, constrictive pericarditis, hypokalemia, or renal failure

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 serum K levels; use cautiously with hypokalemia; monitor serum digoxin level due to narrow therapeutic index; reduce dose in renal dysfunction; CNS effects (eg, drowsiness) and GI effects (eg, nausea, vomiting) are among more common adverse reactions; administer at same time of day in relation to meals

Loop diuretics

These agents inhibit electrolyte reabsorption in the thick ascending limb of the Henle loop in the kidney, thus promoting diuresis.


Furosemide (Lasix)

Commonly used loop diuretic; has moderate diuretic potency.

Adult

20-80 mg/d PO/IV/IM in divided doses q6-12h

Pediatric

1 mg/kg PO/IV qd; may increase dose up to tid

Increases nephrotoxicity of cephalosporins; 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; varying degrees of hearing loss 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; hypokalemia; renal failure

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

Closely monitor serum K levels; may produce intravascular dehydration, severe hypokalemia, and significant hypochloremic metabolic alkalosis; may cause hyperuricemia; may produce deafness due to ototoxicity; titrate dose to effect; administer PO dose with food or milk to decrease stomach upset

Prostaglandins

PGE1 is used for treatment of ductal dependent cyanotic congenital heart disease, which is due to decreased pulmonary blood flow.


Alprostadil (Prostin VR)

Relaxes smooth muscle of the ductus arteriosus. Beneficial in infants with congenital defects that restrict pulmonary or systemic blood flow and who depend on a patent ductus arteriosus to achieve adequate oxygenation and lower body perfusion.

Adult

Not indicated

Pediatric

Initial dose: 0.05 mcg-0.1 mcg/kg/min IV into large vein or umbilical cord
Maintenance dose: 0.01-0.4 mcg/kg/min IV into large vein or umbilical cord

Limited data available; caution with concurrent use of antiplatelet drugs or anticoagulants

Documented hypersensitivity; hyaline membrane disease or respiratory distress 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

Long-term infusions may cause cortical proliferation of the long bones in neonates; due to the inhibitory effects of prostaglandins in platelet aggregation, exercise caution when administering to neonates with bleeding tendencies; apnea occurs in 10-12% of neonates with congenital heart defects; use cautiously in neonates with bleeding tendencies (inhibits platelet aggregation); may cause systemic hypotension, flushing, bradycardia, rhythm disturbances, fever, or seizure-like activity; long-term infusions associated with cortical proliferation of long bones and gastric outlet obstruction

More on Pulmonary Atresia With Intact Ventricular Septum

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References
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References

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Further Reading

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Keywords

pulmonary atresia with intact ventricular septum, PA/IVS, PAIVS, membranous pulmonary atresia, cardiac lesion, imperforate pulmonary valve, ventriculocoronary connections, right ventricular hypoplasia, tricuspid valve hypoplasia, stenosis, right-to-left shunt, patent foramen ovale, secundum atrial septal defect, cyanotic congenital heart disease, CCHD, transposition of the great arteries, tricuspid atresia, ventricular septal defect, angina, arrhythmia, congestive heart failure, CHF, prolonged cyanosis, hypoxemia, myocardial ischemia, angina, polycythemia, hyperviscosity syndrome, thrombocytopenia, apical left ventricular impulse, treatment, diagnosis

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Contributor Information and Disclosures

Author

John R Charpie, MD, PhD, Associate Professor, Department of Pediatrics, University of Michigan Medical Center
John R Charpie, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Medical Editor

Charles I Berul, MD, Associate Professor of Pediatrics, Harvard Medical School; Senior Associate, Department of Cardiology, Children's Hospital of Boston
Charles I Berul, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Heart Rhythm 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

John W Moore, MD, MPH, Professor of Clinical Pediatrics, Section of Pediatric Cardiology, Department of Pediatrics, University of California San Diego School of Medicine; Director of Cardiology, Rady Children's Hospital
John W Moore, MD, MPH 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.

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

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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