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

Pulmonary Atresia With Intact Ventricular Septum

John R Charpie, MD, PhD, Associate Professor, Department of Pediatrics, University of Michigan Medical Center

Updated: Apr 17, 2009

Introduction

Background

Pulmonary atresia with intact ventricular septum (PAIVS) is a rare congenital cardiac lesion characterized by heterogeneous right ventricular development, an imperforate pulmonary valve, and possible extensive ventriculocoronary connections. Prognosis and management depend on the degree of right ventricular hypoplasia (including tricuspid valve hypoplasia) and the dependency of the myocardial blood supply on abnormal communications between the right ventricle and coronary arteries. These 2 factors are the most important prognostic determinants.

Pathophysiology

The PAIVS spectrum ranges from a normal-sized or slightly hypoplastic tripartite right ventricle with a well-formed infundibulum and imperforate pulmonary valve with commissural fusion to a diminutive unipartite right ventricle, narrowed or atretic infundibulum, primitive pulmonary valve, and ventriculocoronary artery connections (with or without stenoses). In PAIVS, the tricuspid valve is rarely normal and demonstrates a continuum of abnormalities, ranging from severe stenosis (often related to annular hypoplasia) to severe regurgitation. In addition, PAIVS has an obligatory right-to-left atrial-level shunt (through a patent foramen ovale or secundum atrial septal defect). Pulmonary blood flow usually depends on a patent ductus arteriosus. Aortopulmonary collaterals that originate from the descending thoracic aorta are rare.

Frequency

United States

Despite overall low prevalence, PAIVS is one form of cyanotic congenital heart disease (CCHD) that usually presents during the neonatal period (along with transposition of the great arteries, tricuspid atresia, and pulmonary atresia with ventricular septal defect). PAIVS has no known genetic etiology, although rare familial cases have been described. PAIVS occurs in 7.1-8.1 per 100,000 live births and in 0.7-3.1% of patients with congenital heart disease (CHD).

International

PAIVS occurs in 4.5 per 100,000 live births in the United Kingdom and Ireland.

Mortality/Morbidity

Early survival depends on maintaining ductal patency until a palliative procedure can be performed to establish a reliable source of pulmonary blood flow. Placement of a systemic-to-pulmonary artery shunt is the most common procedure. In both the short- and long-term, patients are at risk for sudden death, angina, arrhythmias, and congestive heart failure (CHF), in addition to complications of prolonged cyanosis and hypoxemia. The overall probability of survival for patients with PAIVS is approximately 65-82% at age 1 year and 64-76% at age 5 years.

• Sudden death, angina, and arrhythmias: PAIVS is associated with ventriculocoronary connections in approximately 45% of patients. Because coronary artery stenoses are present in nearly 9% of patients, the coronary circulation is considered dependent on right ventricular systolic events. These patients are at particularly high risk for myocardial ischemia, angina, ventricular arrhythmias, and sudden death compared with patients who have other forms of CHD.
• CHF: Depending on the particular anatomic substrate, these patients may have an early predilection for heart failure due to both tricuspid regurgitation and left-to-right, ductal-dependent, pulmonary blood flow. Postoperatively, the risk of heart failure may continue, depending on the ratio of pulmonary-to-systemic blood flow and on the degree of tricuspid and pulmonary regurgitation (following possible right ventricular outflow-tract reconstruction or pulmonary valvotomy). Most late reoperations following biventricular repair are pulmonary valve replacements.
• Cyanosis: Long-term complications of cyanosis and hypoxemia include polycythemia and a hyperviscosity syndrome. These patients may develop headache, decreased exercise tolerance, and stroke. In addition, thrombocytopenia is a common finding that leads to bleeding complications in patients with CCHD.

Age

PAIVS is a cyanotic congenital heart lesion that presents in the newborn period coincident with closure of the patent ductus arteriosus.

Clinical

History

• Infants with pulmonary atresia with intact ventricular septum (PAIVS) are usually born at term, and cyanosis is apparent within hours.
• These babies develop progressively worsening cyanosis and tachypnea associated with closure of the patent ductus arteriosus.

Physical

• The most common finding upon physical examination is central (perioral and periorbital) cyanosis. Following ductal closure, profound generalized cyanosis is present.
• Apical left ventricular impulse may be pronounced.
• The first and second heart sounds are single.
• A pansystolic murmur is often heard at the left lower sternal border, consistent with tricuspid regurgitation. If severe, the murmur of tricuspid regurgitation may be associated with a thrill and a diastolic rumble.
• A systolic ejection murmur of the patent ductus arteriosus may be heard at the left second or third intercostal space, particularly after initiating prostaglandin infusion.
• Normal arterial pulses are usually present.
• Hepatomegaly is uncommon unless the atrial septal defect is restrictive (rare).

Causes

• As with many forms of congestive heart disease (CHD), the genetic cause of PAIVS is unknown.
• Kutsche and Van Mierop suggest that PAIVS probably occurs relatively late in cardiac morphogenesis after cardiac septation compared with pulmonary atresia with ventricular septal defect.^{[1 ]}This may reflect a prenatal inflammatory or infectious condition; however, no histopathological evidence currently supports this view.
• In rare familial cases, some researchers advocate a single gene theory.

Differential Diagnoses

Ebstein Anomaly
Pulmonary Stenosis, Valvar
Tetralogy of Fallot With Absent Pulmonary Valve
Tetralogy of Fallot With Pulmonary Atresia
Transposition of the Great Arteries
Tricuspid Atresia

Workup

Laboratory Studies

• No laboratory blood tests help to confirm a specific diagnosis of pulmonary atresia with intact ventricular septum (PAIVS).
• An ABG study is likely to show hypoxemia and hypocarbia refractory to inspired oxygen concentration, consistent with cyanotic congenital heart disease (CCHD) and a fixed right-to-left shunt.

Imaging Studies

• Echocardiography and Doppler
  • Two-dimensional echocardiography is usually diagnostic for PAIVS.^{[2 ]}A combination of subcostal and precordial views reveals anatomic pulmonary atresia in addition to tricuspid valve and right ventricular morphology and size. Echocardiography and angiography are the 2 most important studies in diagnosis of pulmonary atresia.
  • Absolute volume measurements of the right ventricle usually have limited value. Data from the Congenital Heart Surgeons Study showed that the diameter of the tricuspid valve normalized to body surface area (tricuspid valve z-value) was highly correlated with size of the right ventricular cavity. In addition, retrospective data suggest that the degree of right ventricular hypoplasia was the most important determinant in a single-ventricle versus 2-ventricle repair.
  • In addition, color flow and continuous wave Doppler studies reveal the degree of tricuspid regurgitation, allow the estimation of right ventricular pressure, and reveal restriction of the interatrial communication (uncommon).
  • A combination of imaging and Doppler echocardiography reveals branch pulmonary artery size and configuration (usually within reference ranges), as well as ductal patency. Echocardiographic imaging may reveal ventriculocoronary connections (coronary sinusoids) but has limited use for identifying coronary artery stenoses and right ventricular–dependent coronary circulation.
• Angiocardiography
  • The prognosis of a neonate with PAIVS directly relates to the presence or absence of ventriculocoronary connections and right ventricular–dependent coronary circulation. Although echocardiography is diagnostic for PAIVS, angiocardiography is an important imaging modality for planning future intervention.
  • Right ventricular angiocardiography defines the presence or absence of ventriculocoronary connections and provides information about the size, morphology, and function of the tricuspid valve and right ventricle.
  • Balloon occlusion aortography reveals the proximal coronary arteries and coronary arterial stenosis or interruption.
• Radiography
  • Chest radiography usually reveals mild cardiomegaly and decreased or normal pulmonary vascular markings.
  • With severe tricuspid regurgitation (and a dysplastic tricuspid valve), profound cardiomegaly due to right atrial enlargement may be present.

Other Tests

• Electrocardiography
  • ECG often reveals normal sinus rhythm, QRS axis +30° to +90°, decreased right ventricular forces, left ventricular dominance, and right atrial enlargement (proportional to the degree of tricuspid regurgitation).
  • In addition, ST-T wave abnormalities are common in patients with ventriculocoronary connections or coronary artery stenosis and are consistent with subendocardial ischemia.

Procedures

• Cardiac catheterization
  • Cardiac catheterization allows right ventricular pressure measurement, confirms anatomic pulmonary atresia, and evaluates right and left ventricular function.
  • Ventriculocoronary connections can also be delineated with cardiac catheterization, as can the morphology and size of the tricuspid valve and right ventricle.
  • In the rare instance of a restrictive atrial communication, a transcatheter balloon or blade atrial septostomy may help maintain adequate cardiac output. Recently, transcatheter wire puncture, laser, and radiofrequency-assisted balloon pulmonary valvotomy have been used as alternatives to surgical valvotomy in patients with PAIVS.  However, despite the fact that a technically adequate valvotomy can be achieved in a fairly high percentage of patients, catheter-related complications are common, and valvotomy alone rarely obviates the need for an additional source of pulmonary blood flow (shunt or ductal stenting).
    
    

    

    The heart catheterization.

    
    

Histologic Findings

• Patients with PAIVS can demonstrate a wide range of myocardial abnormalities including ischemia, fibrosis, infarction, rupture, fiber disarray, spongy myocardium, and endocardial fibroelastosis.
• The degree of endocardial fibroelastosis inversely relates to the degree of ventriculocoronary connections.

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.

Dosing

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

Interactions

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

Contraindications

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

Precautions

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.

Dosing

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

Interactions

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

Contraindications

Documented hypersensitivity; hypokalemia; renal failure

Precautions

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.

Dosing

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

Interactions

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

Contraindications

Documented hypersensitivity; hyaline membrane disease or respiratory distress syndrome

Precautions

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

Follow-up

Further Inpatient Care

• Admit patients with pulmonary atresia with intact ventricular septum (PAIVS) for future preoperative testing and surgical interventions.
• Pulmonary valve replacement constitutes most late reoperations.

Further Outpatient Care

• Carefully monitor medication doses and adverse effects.
• Monitor adequacy of repair/palliation with periodic echocardiography.

Inpatient & Outpatient Medications

• Possible discharge medications include digoxin, furosemide, and aspirin.

Transfer

• Transfer may be required for specialized diagnostic evaluation and surgical intervention.

Complications

• Congestive heart failure (CHF)
• Sudden death
• Arrhythmia

Prognosis

• Prognosis depends on the specific anatomy and type of intervention (univentricular or biventricular correction).
• Overall survival is approximately 64-76% at age 5 years; however, single institutions report improved intermediate-term outcomes in relatively small series.

Patient Education

• Provide cardiopulmonary resuscitation (CPR) instruction to family members.
• Educate family members about congenital heart disease (CHD).
• Consider genetics counseling for future pregnancies.^{[5 ]}

Miscellaneous

Medicolegal Pitfalls

• Failure to consider pulmonary atresia with intact ventricular septum (PAIVS), especially in a cyanotic newborn
• Preoperative failure to evaluate for ventriculocoronary connections and coronary artery stenosis

Multimedia

Media file 1: The heart catheterization.

References

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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

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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