Guidelines Summary

2018 American Heart Association/American College of Cardiology (AHA/ACC) guidelines

Class I and IIa recommendations for valvar pulmonary stenosis^[47]

Balloon valvuloplasty is recommended in adults with moderate or severe valvar pulmonary stenosis and otherwise unexplained symptoms of heart failure, cyanosis from interatrial right-to-left communication, and/or exercise intolerance (class I, level of evidence [LOE]: B).

Surgical repair is recommended in adults with moderate or severe valvular pulmonary stenosis and otherwise unexplained symptoms of heart failure, cyanosis, and/or exercise intolerance who are not candidates for balloon valvuloplasty or for whom balloon valvuloplasty was unsuccessful (class I, LOE: B).

In asymptomatic adults with severe valvar pulmonary stenosis, intervention is reasonable (class IIa, LOE: C).

Class I and IIb recommendations for isolated pulmonary regurgitation (PR) after repair of pulmonary stenosis^[47]

Pulmonary valve replacement is recommended in symptomatic patients with moderate or greater PR resulting from treated isolated pulmonary stenosis, with right ventricular (RV) dilation or RV dysfunction (class I, LOE: C).

For asymptomatic patients with residual PR as a result of treatment of isolated pulmonary stenosis with a dilated RV, serial follow-up is recommended (class I, LOE: C).

In asymptomatic patients with moderate or greater PR from treatment of isolated pulmonary stenosis with progressive RV dilation and/or RV dysfunction, pulmonary valve replacement may be reasonable (class IIb, LOE: C).

2020 ESC guidelines

The European Society of Cardiology (ESC) updated their 2010 guidelines on the management of adult congenital heart disease (ACHD) in 2020.^{[48, 49]} Their class I and III recommendations for RV outflow tract obstruction (OTO) are outlined below.

In valvular pulmonary stenosis, balloon valvuloplasty is the intervention of choice, if anatomically suitable.

As long as no valve replacement is required, RVOTO intervention at any level is recommended regardless of symptoms when the stenosis is severe (Doppler peak gradient >64 mmHg).

If surgical valve replacement is the only option, it is indicated in (1) symptomatic patients with severe stenosis; or (2) asymptomatic patients with severe stenosis in the presence of ≥1 of the following:

Objective decrease in exercise capacity
Falling RV function and/or progression of tricuspid regurgitation (TR) to at least moderate
RV systolic pressure (SP) >80 mmHg
Right-to-left (RL) shunting via an atrial septal defect (ASD) or ventricular septal defect (VSD)

Medication

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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.