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

Pulmonary Stenosis, Valvar: 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): Kurt Pflieger, MD, FAAP, Active Staff, Department of Pediatrics, Lake Pointe Medical Center
Contributor Information and Disclosures

Updated: Jul 6, 2009

Treatment

Medical Care

  • Prehospital care in patients with pulmonary valve stenosis
    • Collect essential information about vital signs, including the patient's pulse, respiratory rate, work of breathing, and blood pressure (BP) in the upper and lower extremities. Note the presence or absence of cyanosis.
    • Associated congenital cardiac anomalies should be anticipated until proven otherwise.
    • If the patient has a known large left-to-right shunt, such as patent ductus arteriosus (PDA) or ventricular septal defect (VSD), and if the patient is in respiratory distress, diuresis should be attempted. Diuresis is effective in reducing the cyanosis secondary to pulmonary edema, which is unusual in patients with isolated pulmonary valve stenosis.
    • Use of oxygen may reduce pulmonary artery pressure in patients with a reactive pulmonary vasculature, increasing pulmonary blood flow, which is also unusual in patients with isolated pulmonary valve stenosis.
    • Administer oxygen to any patient with cyanosis and respiratory distress. However, cyanosis-related intracardiac right-to-left shunting does not resolve hypoxemia.
  • Emergency department care
    • Limited diagnostic tests are needed after the structural diagnosis is made.
    • Workup performed for the cyanotic infant with respiratory distress and hypotension or shock is often the same as that performed in a septic patient.
    • If a neonate or young infant presents to the emergency room with severe cyanosis, ductal dependent lesions should be considered. Prostaglandin infusion may open the ductus, augment pulmonary blood flow, and improve oxygen saturation.
  • Therapeutic approach
    • Patients with trivial (gradient <25 mm Hg) or mild (gradient <50 mm Hg) pulmonary stenosis do not need intervention to relieve the obstruction of the pulmonary valve.42 They should be clinically followed up at periodic intervals, perhaps on a yearly basis. During the period of rapid growth (infancy and adolescence), follow-up more frequent than this may be indicated. Routine well-child care, including immunizations by the primary physician, should be provided. Patients with pulmonary stenosis are candidates for infective endocarditis prophylaxis before they undergo any bacteremia -producing procedures and surgery, as indicated in the recommendations of the American Heart Association. Limitations in exercise or activity levels are not needed.
    • Patients with moderate (gradient, 50-79 mm Hg) and severe (gradient, >80 mm Hg) obstruction should undergo intervention to relieve the stenosis of the pulmonary valve. After the obstruction is relieved, recommended routine care, endocarditis prophylaxis, and exercise limitations are the same as those described for trivial and mild stenosis.
    • Patients with signs of right ventricular failure should be promptly treated with anticongestive measures, including digitalis and diuretics. However, the problem does not resolve until the obstruction is relieved. Therefore, prompt balloon or surgical intervention should be undertaken. Right ventricular function may not recover completely if intervention is withheld for too long and if myocardial damage sets in.
    • A fetus with critical pulmonary stenosis or atresia with intact ventricular septum may benefit from pulmonary balloon valvuloplasty in utero, which promotes growth of the right ventricle.43,44
    • Although the consensus is to offer relief of pulmonary valve obstruction in children with moderate or severe stenoses, this approach is somewhat controversial in adults because of reported lack of progression and the lack of complications in 1 group of adults monitored for 5-24 years in the 1970s.16 However, a prudent strategy may be to relieve pulmonary valve obstruction in adults with moderate-to-severe pulmonary stenosis, irrespective of their symptoms, because of the potential (1) for myocardial damage associated with long-term pressure overload of the right ventricle,45 (2) for generally lowered cardiac indices both before and after exercise in adults compared with children,46 and (3) for exercise-induced hemodynamic abnormalities in adults.46

Surgical Care

Balloon Pulmonary Valvuloplasty

  • Introduction: Rubio-Alverez et al first attempted to relieve pulmonary valve obstruction with transcatheter methods in the early 1950s.47 They used a ureteral catheter with a wire to cut open the stenotic pulmonary valve.
    • In 1979, Semb et al used a balloon-tipped angiographic (Berman) catheter to rupture pulmonary valve commissures by rapidly withdrawing the inflated balloon across the valve.48
    • More recently, Kan et al49 applied the techniques of Dotter and Judkins50 and Gruntzig et al51 to relieve pulmonary valve obstruction by using the radial forces of balloon inflation of a balloon catheter positioned across the pulmonic valve. This static balloon-dilation technique is currently performed worldwide to relieve pulmonary valve obstruction.
    • The general consensus based on current data is that balloon valvuloplasty is the treatment of choice for managing isolated pulmonary valve stenosis.52,53
  • Indications: In general, indications for balloon pulmonary valvuloplasty are similar to those used in surgical pulmonary valvotomy (ie, moderate pulmonary valve stenosis with a peak-to-peak gradient >50 mm Hg with a normal cardiac index). Some cardiologists change this criterion to a gradient of 40 mm Hg or right ventricular pressure of 50 mm Hg. Careful evaluation of the available data suggests that (1) right ventricular pressure is only marginally reduced if mildly stenotic valves are dilated,40 (2) trivial and mild stenoses (gradient <50 mm Hg) are likely to remain mild at follow-up (as shown in natural-history studies),41,54 and (3) an increase in gradient is easily quantitated on follow-up Doppler echocardiography.31,31,32,33 If an increased gradient is documented, the patient can then undergo balloon dilatation. Given these observations, balloon dilation should be performed only in patients with peak-to-peak gradient of more than 50 mm Hg.
  • Technique
    • The technique of balloon pulmonary valvuloplasty involves positioning a balloon catheter (see Media file 5) across the stenotic valve, usually over an extra-stiff exchange-length guide wire and inflating the balloon with diluted contrast material to accomplish valvotomy.55,56

      Selected cineradiographic frames of a balloon dil...

      Selected cineradiographic frames of a balloon dilatation catheter placed across a stenotic pulmonary valve. Note "waisting" of the balloon during the initial phases of the balloon inflation (A), which was almost completely abolished during the later phases of balloon inflation (B). Reproduced from Rao PS: Balloon pulmonary valvuloplasty for isolated pulmonic stenosis. In: Rao PS, ed: Transcatheter Therapy in Pediatric Cardiology New York, NY: Wiley-Liss; 1993: 59-104.

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      Selected cineradiographic frames of a balloon dil...

      Selected cineradiographic frames of a balloon dilatation catheter placed across a stenotic pulmonary valve. Note "waisting" of the balloon during the initial phases of the balloon inflation (A), which was almost completely abolished during the later phases of balloon inflation (B). Reproduced from Rao PS: Balloon pulmonary valvuloplasty for isolated pulmonic stenosis. In: Rao PS, ed: Transcatheter Therapy in Pediatric Cardiology New York, NY: Wiley-Liss; 1993: 59-104.

    • The initially recommended balloon-to-annulus ratio was 1.2-1.4;54,57,58 subsequent data suggested a ratio of 1.2-1.25.59,60 When the pulmonary valve annulus is too large to dilate with a single balloon (about 20 mm), valvuloplasty with simultaneous inflation of 2 balloons across the pulmonary valve may be performed,61,62 although the current availability of large-diameter balloons make this technique unnecessary. However, the double balloon technique may be more effective and stable in some cases. The Inoue balloon has been used in adults with success.63 The major advantage of the Inoue balloon over conventional balloons is its adjustable diameter that makes stepwise dilation possible.
  • Mechanism of valvuloplasty: Inflation of a balloon placed across an obstructive lesion exerts radial forces on the stenotic lesion without any axial component.64,65
    • The mechanism of valvuloplasty is assessed by directly visualizing the valvar mechanism during surgery66 and postmortem examination67 and by indirect means, such as angiography and echocardiography.68,69 Splitting of the valve commissures and tearing and avulsion of the valve leaflets have been observed and are conceivably the mechanism by which balloon dilation relieves pulmonary valve obstruction. The circumferential dilating force that balloon inflation exerts is likely to rupture (tear) the weakest part of the valve mechanism. The fused commissures are the likely weakest links that can be broken with balloon dilation. However, when fused commissures are strong and cannot be torn, the valve cusps can be torn or the valve leaflet avulse. These events are likely to worsen pulmonary insufficiency.
    • Pulmonary valve dysplasia, if severe, may preclude successful balloon valvuloplasty unless an associated commissural fusion is present.70,71
  • Immediate results: Results observed immediately after balloon valvuloplasty include reduced peak-to-peak gradients and right ventricular–to–left ventricular pressure ratios and increased pulmonary artery pressures, jet widths, and free motion of the pulmonary valve leaflets with decreased doming.72,52,53,73,74 Improvement of right ventricular function, tricuspid insufficiency,52 and right-to-left shunt,12 if present before dilation, are also documented.
  • Infundibular stenosis: Infundibular gradients occur in nearly 30% patients.12,75 The older the patient's age and the greater the severity of obstruction, the greater the prevalence of infundibular reaction. When residual infundibular gradient is >50 mm Hg beta-blockade is generally recommended. Infundibular obstruction greatly regresses at follow-up (see Media file 6),5 as demonstrated for infundibular reactions after surgical valvotomy.76,77,78 Rare patients require surgical intervention.

    Selected frames from lateral view of the right ve...

    Selected frames from lateral view of the right ventricular (RV) cineangiogram showing severe infundibular stenosis (A) immediately following balloon valvuloplasty (corresponding Media file 3, center). At 10 months after balloon valvuloplasty, the right ventricular outflow tract (B) is wide open and corresponds to Media file 3, right. Peak-to-peak pulmonary valve gradient was 20 mm Hg and no infundibular gradient was present. PA = Pulmonary artery. Reproduced with permission from Thapar MK: Significance of infundibular obstruction following balloon valvuloplasty for valvar pulmonic stenosis. Am Heart J 1989; Jul; 118(1): 99-103.

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    Selected frames from lateral view of the right ve...

    Selected frames from lateral view of the right ventricular (RV) cineangiogram showing severe infundibular stenosis (A) immediately following balloon valvuloplasty (corresponding Media file 3, center). At 10 months after balloon valvuloplasty, the right ventricular outflow tract (B) is wide open and corresponds to Media file 3, right. Peak-to-peak pulmonary valve gradient was 20 mm Hg and no infundibular gradient was present. PA = Pulmonary artery. Reproduced with permission from Thapar MK: Significance of infundibular obstruction following balloon valvuloplasty for valvar pulmonic stenosis. Am Heart J 1989; Jul; 118(1): 99-103.

  • Follow-up evaluation: Clinical, ECG, and Doppler echocardiographic evaluations are generally recommended at 1 month, 6 months, and 12 months after the procedure and yearly thereafter.72,52,53,79 Regression of right ventricular hypertrophy, as shown on ECG after balloon dilatation, is well documented. ECG is a useful adjunct in the evaluation of follow-up results.80 However, ECG evidence of hemodynamic improvement does not become apparent until 6 months after valvuloplasty.80 The Doppler gradient generally reflects residual obstruction and is a useful and reliable noninvasive monitoring tool.72,52,73,81
  • Intermediate-term results: At intermediate-term follow-up (usually <2 y), both catheterization-measured peak-to-peak and Doppler-measured peak instantaneous gradients remain improved as a whole.72,52,53 However, restenosis (gradient >50 mm Hg) is observed in nearly 10% patients.82
    • Predictors of restenosis include a balloon-to-annulus ratio of less than 1.2 and a gradient of more than 30 mm Hg immediately after valvuloplasty. In addition, early in the study period, a small valve annulus, and a postsurgical or complex pulmonary stenosis were also predictive of restenosis.83
    • Patients with restenosis have been successfully treated with redilatation with balloons larger than those used for initial balloon valvuloplasty.84 Redilatation is the procedure of choice for managing restenosis after previous balloon dilatation. However, if the pulmonary valve annulus is hypoplastic, if the pulmonary valve leaflets are dysplastic, or if clinically significant supravalvar pulmonary artery stenosis is the major reason for the restenosis, surgery is recommended.
  • Long-term follow-up results: Although immediate and short-term results have been documented,74,79 data on long-term results are scarce. Published studies reveal generally low residual peak instantaneous Doppler gradients with minimal (1-2%) late recurrence of pulmonary stenosis (beyond that seen at intermediate follow-up).58,74
    • In one study, approximately 5% of patients needed surgical intervention to relieve fixed subvalvar or supravalvar stenosis.74 Actuarial freedom for reintervention was 88% and 84%, respectively, at 5 and 10 years. Pulmonary valve insufficiency was noted in 80-90% patients, but right ventricular volume overloading did not develop, and none of the patients required surgical intervention because of pulmonary insufficiency. Based on these data, the authors concluded that balloon pulmonary valvuloplasty may continue to be the treatment of choice for moderate-to-severe valvar pulmonary stenosis, and 10-year to 20-year follow-up studies to evaluate the clinical significance of residual pulmonary insufficiency should be undertaken.
    • One report documented the development of clinically significant pulmonary insufficiency in 6 (6%) of 107 patients at late follow-up. Some of these patients required pulmonary valve replacement.59
  • Comparison with surgical valvotomy: Comparison of balloon therapy with surgical valvotomy has limitations,52,74 but the mortality and morbidity rates are generally higher after surgery. Greatest reduction of the gradient is observed after surgery, but the degree and frequency of pulmonary insufficiency may be higher after surgery than after balloon therapy.85
    • Critical pulmonary stenosis in the neonate: The term critical pulmonary stenosis with intact ventricular septum is applied to severe pulmonary valvar obstruction resulting in suprasystemic right ventricular systolic pressure with resultant tricuspid insufficiency, a right-to-left shunt across the atrial septum, and often a ductal-dependent pulmonary circulation.
      • Although the surgical approach to relieve the obstruction with or without aorta-pulmonary shunt was standard treatment in the past, transcatheter treatment is now first-line therapy.
      • Prostaglandin E1 is infused to augment pulmonary blood flow and improve systemic arterial desaturation, followed by cardiac catheterization and biplane (sitting-up and lateral views) right ventricular cineangiography. A right coronary artery, angled glide, balloon wedge, or cobra catheter (according to the operator's preference) is placed in the right ventricular outflow tract, and a floppy-tipped coronary guidewire is advanced across the pulmonary valve and into the right or left pulmonary artery or into the descending aorta through the ductus. The catheter is advanced across the pulmonary valve into a distal pulmonary artery or the descending aorta. The guidewire is then exchanged for a guidewire that is suited to position the balloon-dilation catheter. Then, balloon pulmonary valvuloplasty is performed in manner described earlier.86,87
      • In some cases, the balloon catheter cannot be advanced across a severely stenotic pulmonary valve, and balloon catheters 3-6 mm in diameter may be used for initial predilation and then replaced with larger, more appropriately sized balloon catheters.
      • Although the results of this approach are reasonably good, the need for reintervention to address the complications associated with the procedure, residual obstruction, or associated defects is 25% in neonates compared with older children 8-10%.86,87,88

Other Catheter Interventions

  • Numerous other catheter interventions may become necessary in patients with pulmonary stenosis.
    • Transcatheter occlusion of a patent ductus arteriosus (PDA): Some patients with pulmonary stenosis may have a PDA of significant size. In such patients, transcatheter occlusion of the PDA performed with a coil (for small PDAs) or with an Amplatzer duct occluder (for medium or large PDAs) is recommended immediately after balloon pulmonary valvuloplasty. (See also the eMedicine article Patent Ductus Arteriosus.)
    • Occlusion of a patent foramen ovale or atrial septal defect: A patent foramen ovale or atrial septal defect may occur in association with pulmonary stenosis. If these atrial defects do not spontaneously close during follow-up after balloon valvuloplasty, they may be closed with an Amplatzer septal occluder or Helex device, if the criteria for their closure are met. Sometimes, the defects may need to be closed to prevent recurrent paradoxical embolisms. (See also the eMedicine article Atrial Septal Defect, General Concepts.)
    • Balloon atrial septostomy:51 In neonates with a severely hypoplastic right ventricle, balloon atrial septostomy may be necessary to provide adequate egress to the systemic venous return. Indications are clinical signs of systemic venous congestion, restrictive patent foramen ovale on Doppler echocardiography, markedly elevated right atrial pressure with tall a waves, and/or a mean atrial pressure difference of more than 5 mm Hg.
      • In neonates, Rashkind balloon septostomy is effective.
      • Beyond the neonatal period, Park blade septostomy or surgical septostomy may be necessary. In patients with only mild or moderate right ventricular hypoplasia, balloon septostomy should be avoided so that forward flow through the right ventricle is encouraged with a consequent opportunity for its growth.
    • Cardiac catheterization with balloon valvuloplasty: This is the preferred therapy for severe or critical valvar pulmonary stenosis. In neonates with critical valvar pulmonary stenosis, mortality rates related to balloon dilation are lower than those related to surgery mortality, and balloon dilation is the treatment of choice.

Other Surgical Techniques

  • Since the first description of surgical relief of pulmonary stenosis by closed pulmonary valvotomy in the late 1940s,89,90  various techniques were developed and include valvotomy with inflow occlusion, hypothermia and cardiopulmonary bypass. The currently preferred approach is transpulmonary arterial valvotomy under cardiopulmonary bypass.91
    • Results: Results of surgery are generally good with low mortality rate (3-7%) and decreased right ventricular pressures and pulmonary valve gradients. In a natural-history study in the United States, only 3% of 294 operated patients had gradients of more than 50 mm Hg at 4-8 years after surgery.41 The incidence of pulmonary insufficiency at follow-up was 60-90%.92 Despite these good results, transluminal balloon pulmonary valvuloplasty (discussed above) has replaced surgical pulmonary valvotomy.
    • Indications: Surgery is reserved for cases in which balloon valvuloplasty is not feasible or not successful. One example is dysplastic pulmonary valve with valve ring hypoplasia. The treatment of this disorder is to excise the obstructive valve leaflets and enlarge the annulus by a transannular patch. Other examples are fixed infundibular and supravalvar stenosis after successful balloon valvuloplasty.74
    • Blalock-Taussig shunt: Patients with critical pulmonary stenosis and marked hypoplasia of the right ventricle may need a Blalock-Taussig shunt in addition to or instead of balloon or surgical valvotomy.
    • Right ventricular repair/bypass
      • Right ventricular hypoplasia, as alluded to above, occurs in some patients with pulmonary stenosis, although this hypoplasia is most common in patients with pulmonary atresia and an intact ventricular septum. The right ventricle may enlarge after right ventricular outflow obstruction is relieved, particularly in tripartite right ventricles.93,94,95
      • If the right ventricle does not grow adequately to support the pulmonary circulation, 1.5 or single ventricular repair should be considered. In 1.5-ventricular repair, a bidirectional Glenn procedure (superior vena cava–to–right pulmonary artery anastomosis, end to side) is performed to divert the blood from the upper part of the body directly into the pulmonary artery, and the atrial septal defect is closed to allow the blood from the lower part of the body to go into the lungs through the right ventricle. In the single ventricle repair, the right ventricle is bypassed by the Fontan procedure. In the Fontan procedure, staged total cavopulmonary connection is performed by bidirectional Glenn initially and then extra conduit diversion of the inferior cava into the pulmonary artery.
    • See the eMedicine article Tricuspid Atresia.

Consultations

  • Consultation with a pediatric cardiologist precedes consultation with a cardiothoracic surgeon.
  • Patients with pulmonary valve atresia or a critical pulmonary stenosis with an inadequate right ventricle require a shunt (usually a modified Blalock-Taussig or central shunt) after the ductus arteriosus is pharmacologically kept patent with prostaglandin E1.
  • Definitive repair may not be possible if the right ventricle is hypoplastic. Single ventricular palliation, such as the Fontan procedure, or variation, such as staged total cavopulmonary connection, may be required.

Activity

  • A prudent philosophy is to allow patients to limit their own activity according to personal tolerance.
  • Restriction from highly exertional and competitive sports is recommended only for patients with severe pulmonary stenosis.
  • After successful relief of the obstruction, normal activity may be resumed.

Medication

No medications are useful in isolated valvar pulmonary stenosis. Patients with congestive heart failure (CHF) may benefit from anticongestive therapy. Patients with cyanosis may benefit from oxygen and prostaglandin E1. Patients with cyanosis due to a large right-to-left shunt require a definitive surgical procedure.

Patients with certain cardiac conditions, such as pulmonary stenosis, typically require antibiotic prophylaxis of endocarditis before they undergo procedures that may cause bacteremia. For more information, see Antibiotic Prophylactic Regimens for Endocarditis.

Prostaglandins

Alprostadil (Prostaglandin E1, PGE1) is used to treat ductal-dependent cyanotic congenital heart disease, which is caused by decreased pulmonary blood flow. Alprostadil acts as a smooth muscle relaxer and maintains patency of the ductus arteriosus when a cyanotic lesion (eg, critical pulmonary stenosis or atresia) or when an interrupted aortic arch occurs in a newborn. This drug is effective only in the neonatal period.


Alprostadil (Prostin VR)

First-line drug used as palliative therapy to temporarily maintain patency of ductus arteriosus before surgery. Produces vasodilation and increases cardiac output. Also inhibits platelet aggregation and stimulates intestinal and uterine smooth muscle. Used in suspected critical pulmonary stenosis when presentation includes cyanosis. Also used in ductal-dependent lesion (eg, pulmonary atresia variants, coarctation of aorta, interrupted aortic arch). Each 1-mL ampule contains 500 mcg/mL.

Adult

Pediatric

0.05-0.1 mcg/kg/min IV initially; after ductal dilatation achieved and O2 saturation improved, may gradually decrease to 0.02-0.025 mcg/kg/min if O2 saturations do not fall

Data limited; caution with concurrent use of antiplatelet drugs or anticoagulants

Documented hypersensitivity; hyaline membrane disease, 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

Adverse effects and toxicity include apnea, seizures, fever, hypotension, leukocytosis, and pulmonary overcirculation; neonates usually intubated prophylactically because of potential risk of apnea (10-12%); prolonged use is occasionally necessary (in candidates with hypoplastic left heart syndrome awaiting transplantation) and may be associated with third spacing of fluid; monitor blood oxygenation and arterial pressure

Beta-blockers

These drugs inhibit chronotropic, inotropic, and vasodilatory responses to beta-adrenergic stimulation.


Atenolol (Tenormin)

Used to treat hypertension. Selectively blocks beta1-receptors with little or no affect on beta2 types. Beta-blockers affect BP by means of several mechanisms, including negative chronotropic effect that decreases heart rate at rest and after exercise, negative inotropic effect that decreases cardiac output, reduction of sympathetic outflow from the CNS, and suppression of renin release from the kidneys. Used to improve and preserve hemodynamic status by acting on myocardial contractility, reducing congestion, and decreasing myocardial energy expenditure.

Beta-blockers reduce inotropic state of left ventricle, improve diastolic dysfunction, and increase LV compliance, reducing pressure gradient across LV outflow tract. Decreases myocardial oxygen consumption, reducing myocardial ischemia potential. Decreases heart rate, reducing myocardial oxygen consumption and reducing potential for myocardial ischemia. During IV administration, carefully monitor BP, heart rate, and ECG.

The drug may be used to reduce hypercontractility of the right ventricle in patients with significant infundibular stenosis (gradients >50 mm Hg) following balloon pulmonary valvuloplasty.

Adult

25-50 mg PO qd initially; may increase to 100 mg/d prn

Pediatric

0.8-1 mg/kg/d PO initially; may uptitrate according to symptoms; not to exceed 2 mg/kg/d

Coadministration with aluminum salts, barbiturates, calcium salts, cholestyramine, NSAIDs, penicillins, and rifampin may decrease effects; haloperidol, hydralazine, loop diuretics, and MAOIs may increase toxicity

Documented hypersensitivity; CHF, pulmonary edema, cardiogenic shock, AV conduction abnormalities, and heart block (without pacemaker)

Pregnancy

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

Precautions

Beta-blockade may reduce symptoms of acute hypoglycemia and mask signs of hyperthyroidism; abrupt withdrawal may exacerbate symptoms of hyperthyroidism and cause thyroid storm; monitor patients closely and withdraw slowly; during an IV, carefully monitor BP, heart rate, and ECG


Esmolol (Brevibloc)

Ultra–short-acting that selectively blocks beta1-receptors with little or no effect on beta2-receptors. Particularly useful in elevated arterial pressure, especially if surgery planned. Reduced episodes of chest pain and clinical cardiac events compared with placebo. Can discontinue abruptly if necessary. Useful in patients at risk for complications from beta-blockade, particularly those with reactive airway disease, mild-to-moderate LV dysfunction, and/or peripheral vascular disease. Short half-life of 8 min allows for titration to desired effect and quick discontinuation if needed.

Adult

Loading dose: 250-500 mcg/kg/min IV for 1 min, followed by maintenance infusion of 50 mcg/kg/min for 4 min; if adequate therapeutic effect (decreased HR and BP) not observed within 5 min, repeat loading dose and follow with maintenance infusion of 100 mcg/kg/min for 4 min; may repeat sequence q5-10min, increasing maintenance infusion by 50 mcg/kg/min with each sequence; not to exceed 200 mcg/kg/min

Pediatric

100-500 mcg/kg IV administered over 1 min initially; followed by continuous IV infusion titrated to 25-100 mcg/kg/min; continue titration according to BP or HR response; doses exceeding 200 mcg/kg/min rarely necessary

Aluminum salts, barbiturates, NSAIDs, penicillins, calcium salts, cholestyramine, and rifampin may decrease bioavailability and plasma levels of esmolol, possibly decreasing pharmacologic effect; cardiotoxicity may increase with concurrent sparfloxacin, astemizole, calcium channel blockers, quinidine, flecainide, or contraceptives; toxicity increases with concurrent digoxin, flecainide, acetaminophen, clonidine, epinephrine, nifedipine, prazosin, haloperidol, phenothiazines, or catecholamine-depleting agents

Documented hypersensitivity; uncompensated CHF, bradycardia, cardiogenic shock, and AV conduction abnormalities

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

Beta-blockers may mask signs and symptoms of acute hypoglycemia and clinical signs of hyperthyroidism; symptoms of hyperthyroidism, including thyroid storm may worsen, when abruptly withdrawn; withdraw slowly and monitor patient closely


Propranolol (Inderal)

Nonselective beta-blocker. Lipophilic (penetrates CNS). Has membrane-stabilizing activity and decreases automaticity of contractions. Also has class II antiarrhythmic properties.

Adult

40-80 mg PO bid initially; increase to 160-320 mg/d (<640 mg/d in some patients)

Pediatric

1-4 mg/kg/d PO divided q6-8h; not to exceed 16 mg/kg/d; avoid IV use; however, if necessary (eg, hypercyanotic spell in tetralogy of Fallot or severe infundibular reaction after balloon pulmonary valvuloplasty), 0.1-0.25 mg/kg/dose IV over >10 min; not to exceed 1 mg/dose (infants) or 3 mg/dose (children)

Coadministration with aluminum salts, barbiturates, NSAIDs, penicillins, calcium salts, cholestyramine, and rifampin may decrease effects; calcium channel blockers, cimetidine, loop diuretics, and MAOIs may increase toxicity; may increase toxicity of hydralazine, haloperidol, benzodiazepines, and phenothiazines

Documented hypersensitivity; uncompensated CHF; bradycardia, cardiogenic shock; AV conduction abnormalities

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

Beta-blockade may decrease signs of acute hypoglycemia and hyperthyroidism; abrupt withdrawal may exacerbate symptoms of hyperthyroidism, including thyroid storm; withdraw slowly and monitor closely

More on Pulmonary Stenosis, Valvar

Overview: Pulmonary Stenosis, Valvar
Differential Diagnoses & Workup: Pulmonary Stenosis, Valvar
Treatment & Medication: Pulmonary Stenosis, Valvar
Follow-up: Pulmonary Stenosis, Valvar
Multimedia: Pulmonary Stenosis, Valvar
References
Further Reading
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References

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Keywords

valvar pulmonary stenosis, pulmonary artery, right ventricle, valvular pulmonary stenosis, valvate pulmonary stenosis, pulmonary stenosis with a normal aortic root, pulmonary stenosis with an intact ventricular septum, isolated pulmonary valve stenosis, ventricular septal defect, rheumatic fever, pulmonary valve dysplasia, atrial septal defect, Noonan syndrome, Turner syndrome, rubella syndrome, Williams syndrome, ventricular septal defect, VSD, tetralogy of Fallot, heart failure, patent foramen ovale, underdeveloped right ventricle, hypoplastic right ventricle, syncope, angina pectoris, tricuspid regurgitation, 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)

Kurt Pflieger, MD, FAAP, Active Staff, Department of Pediatrics, Lake Pointe Medical Center
Kurt Pflieger, MD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American College of Emergency Physicians, American Heart Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Jeffrey Allen Towbin, MD, MSc, FAAP, FACC, FAHA, Professor, Departments of Pediatrics (Cardiology), Cardiovascular Sciences, and Molecular and Human Genetics, Baylor College of Medicine; Chief of Pediatric Cardiology, Foundation Chair in Pediatric Cardiac Research, Texas Children's Hospital
Jeffrey Allen Towbin, MD, MSc, FAAP, FACC, FAHA is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American College of Cardiology, American College of Sports Medicine, American Heart Association, American Medical Association, American Society of Human Genetics, Cardiac Electrophysiology Society, New York Academy of Sciences, Society for Pediatric Research, Texas Medical Association, and Texas Pediatric Society
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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