Medication Summary

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 for endocarditis before they undergo procedures that may cause bacteremia. For more information, see Antibiotic Prophylactic Regimens for Endocarditis.

Class Summary

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 IV (Prostin VR)

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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.

Class Summary

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

Atenolol (Tenormin)

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Used to treat hypertension. Selectively blocks beta1-receptors with little or no affect on beta2 types. Beta-blockers affect BP by 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 to reduce congestion and decrease myocardial energy expenditure.

Beta-blockers reduce inotropic state of left ventricle, improve diastolic function, 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.

Esmolol (Brevibloc)

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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.

Propranolol (Inderal)

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

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Media Gallery

Valvar Pulmonary Stenosis. In valvar pulmonic stenosis, the severity of obstruction may be judged by auscultatory findings. In mild stenosis, the ejection click (EC) is clearly separated from the first heart sound (S1). The murmur starts with the click, peaks in early systole, and ends far ahead of the aortic component of the second heart sound (A2). The pulmonary component of the second heart sound (P2) is normal to increased in intensity. In moderate pulmonic stenosis, the click is closer to the first heart sound, the ejection murmur peaks later in systole and the murmur reaches the A2, and the second heart sound is widely split with a soft pulmonary component. In severe valvar obstruction, the click is either absent or occurs so close to S1 that it cannot be heard separately, and the murmur peaks late in systole and extends beyond the A2. The second heart sound is widely split with an extremely soft or inaudible P2. Reproduced from Rao PS: Evaluation of cardiac murmur in children. Indian J Pediatr. 1991 Jul-Aug; 58(4): 471-91.
Valvar Pulmonary Stenosis. Posteroanterior chest roentgenogram in a patient with valvar pulmonic stenosis showing a normal-sized heart with normal pulmonary vascular markings. Note prominent main pulmonary artery. Reproduced with permission from Rao PS: Diagnosis and management of acyanotic heart disease: Part I – Obstructive lesions. Indian J Pediatr. 2005; 72: 495-502.
Valvar Pulmonary Stenosis. Doppler flow velocity recordings from the main pulmonary artery prior to (left) and 1 day (center) and 10 months (right) after successful balloon pulmonary valvuloplasty. Note that no significant fall in the peak flow velocity is present on the day after the balloon procedure, but a characteristic triangular pattern is present, indicative of infundibular obstruction. At 10-month follow-up, the flow velocity decreased, suggesting resolution of the infundibular obstruction. 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.
Valvar Pulmonary Stenosis. Right ventricular (RV) cineangiogram in the lateral view of a child with valvar pulmonary stenosis demonstrating thickened and domed pulmonary valve leaflets and poststenotic dilatation of the pulmonary artery (PA). Reproduced with permission from Rao PS: Diagnosis and management of acyanotic heart disease: Part I – Obstructive lesions. Indian J Pediatr. 2005; 72: 495-502.
Valvar Pulmonary Stenosis. 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.
Valvar Pulmonary Stenosis. Selected frames from the 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 mmHg 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.