Background:
Isolated pulmonary valve stenosis (PS) is one of the most common types of congenital heart disease (CHD), accounting for 7-12% of all CHD.^1-5 Outcomes in patients with PS are usually determined by the severity of the lesion and patient age at time of diagnosis. Patients with mild PS are generally asymptomatic and do not require intervention,⁶ but those with severe PS can present with cyanosis, exertional dyspnea, and fatigue. Diagnostic evaluation in these patients can reveal right ventricular hypertrophy and dysfunction.^6-7 Significant PS may be addressed by either catheter-based or surgical intervention.^6-8 There is wide variation in clinical practice in these patients' surveillance and subsequent management,⁹ likely due to lack of evidence-based guidelines, particularly in pediatric patients. To minimize practice variation and appropriately utilize medical resources, a standardized and systematic approach is essential for evaluation and treatment.

Goals and Details of the Algorithm
This clinical practice algorithm was developed with the primary objective of creating a decision support tool to help physicians and other healthcare providers in the management of isolated PS. The algorithm goal is to establish a standardized approach in the management of PS, including cases where catheter-based and/or surgical interventions are indicated. Guidance on initial management, subsequent outpatient follow-up, and testing frequency (i.e., echocardiography and electrocardiography) from birth through adulthood are provided.

Three clinical practice algorithms for isolated PS were developed: (A) PS in patients <18 years of age; (B) PS in patients ≥18 years of age and (C) PS post-intervention. PS as part of a more complex lesion (e.g., with subvalvar or supravalvar PS or significant branch PS) and critical PS (ductal dependency, requiring neonatal intervention) have been excluded. Following intervention, patients with critical PS could be managed based on the post-intervention algorithm recommendations. Pregnant patients are also excluded given the alterations in hemodynamics and cardiovascular physiology during pregnancy and specific clinical considerations around labor and delivery.

Each pathway starts with the initial standard clinical workup and proceeds to a decision tree. For patients <18 years, the first branch point is age, given the known variation in progression and prognosis in isolated PS based on age at diagnosis.¹⁰ After categorization based on age, it stratifies by stenosis severity. The algorithm for patients ≥18 years of age uses PS severity as the primary driver for evaluation and management.¹¹ The decision tree then progresses to frequency of follow-up, testing, and need for intervention. The algorithm is designed to provide general guidance for management of PS, but there may be clinical scenarios where closer follow-up may be warranted. In such cases, independent physician judgment should supersede the algorithm recommendations.

Thresholds for mild, moderate, and severe stenosis are based on transthoracic echocardiography (TTE) Doppler peak gradients as outlined in the 2018 AHA/ACC Guideline for the Management of Adults with Congenital Heart Disease¹¹ with mild stenosis defined as a peak velocity <3 m/s (peak gradient <36 mmHg), moderate stenosis defined as a peak velocity of 3-4 m/s (peak gradient 36-64 mmHg), and severe stenosis as a peak velocity of >4 m/s (peak gradient >64 mmHg). AHA/ACC severity definitions based on TTE peak velocity/peak gradient are consistent with those from the American Society of Echocardiography and the European Association of Echocardiography.¹² While the current pediatric cardiac catheterization guidelines¹³ use a TTE Doppler peak instantaneous gradient of 40 mmHg as a reasonable threshold to consider intervention, in clinical practice, many would choose conservative management with close follow-up in asymptomatic patients at this degree of severity before referral for intervention. A higher threshold is reasonable in this patient population if they are asymptomatic, and this conservative approach is reflected in the algorithm.

The post-intervention algorithm guides management and follow-up after catheter-based or surgical intervention. This starts with standard clinical follow-up and testing requirements after initial discharge post-intervention. The algorithm then delineates a decision tree based on the management of the dominant residual lesion: either stenosis and/or regurgitation. Once the dominant lesion is identified, the guidance progresses to a physiologic aged-based algorithm for residual lesions.^6-8 The presence of symptoms is considered before the next steps for patients with at least moderate regurgitation, which warrants further diagnostic tests and/or repeat intervention.

Methods: Algorithm Development
The isolated PS algorithm was created by the Quality Working Group of the American College of Cardiology (ACC) Adult Congenital and Pediatric Cardiology (ACPC) member section, comprised of experienced pediatric and adult congenital cardiologists with multidisciplinary expertise and diverse training and practice backgrounds. Best practice recommendations were developed using the same quality-driven approach used for the secundum atrial septal defect algorithm.¹⁴ Current clinical evidence on isolated PS and existing guidelines^12-15 were utilized in the development of this evidence-based algorithm, as well as group consensus based on clinical practice if evidence was lacking or there were conflicting reports in the literature.

Future Directions
The PS clinical practice algorithms can be utilized by physicians and healthcare providers as a support tool for decision making. A standardized and systematic approach aims to reduce practice variation and improve resource utilization. The clinical application of the algorithms can form the basis for multicenter research to evaluate the impact on patient outcomes.

References

1. Samánek M, Slavík Z, Zborilová B, Hrobonová V, Vorísková M, Skovránek J. Prevalence, treatment, and outcome of heart disease in live-born children: a prospective analysis of 91,823 live-born children. Pediatr Cardiol 1989;10:205-11.
2. Mitchell SC, Korones SB, Berendes HW. Congenital heart disease in 56,109 births. Incidence and natural history. Circulation 1971;43:323–32.
3. Rowe RD. Pulmonary stenosis with normal aortic root. In: Keith JD, Rowe RD, Vlad P, eds. Heart Disease in Infancy and Childhood. 3rd ed. New York: Macmillan, 1978:761–88.
4. Campbell M. Simple pulmonary stenosis: pulmonary stenosis with closed ventricular septum. Br Heart J 1954;16:273–99.
5. Stephensen SS, Sigfusson G, Eiriksson H, et al. Congenital cardiac malformations in Iceland from 1990 through 1999. Cardiol Young 2004;14:396-401.
6. Hayes CJ, Gersony WM, Driscoll DJ, et al. Second natural history study of congenital heart defects. Results of treatment of patients with pulmonary valvar stenosis. Circulation 1993;87:128-37.
7. Voet A, Rega F, de Bruaene AV, et al. Long-term outcome after treatment of isolated pulmonary valve stenosis. Int J Cardiol 2012;156:11–5.
8. Morray BH, McElhinney DB,. Semilunar valve interventions for congenital heart disease: JACC State-of-the-Art Review. J Am Coll Cardiol 2021;77:71–79.
9. Glatz A, Kennedy K, Rome J, O'Byrne ML. Variations in practice patterns and consistency with published guidelines for balloon aortic and pulmonary valvuloplasty: an analysis of data from the IMPACT Registry. JACC Cardiovasc Interv 2018;11:529–38.
10. Rowland DG, Hammill WW, Allen HD, Gutgesell HP. Natural course of isolated pulmonary valve stenosis in infants and children utilizing Doppler echocardiography. Am J Cardiol 1997;79:344-49.
11. Stout, KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol 2019;73:e81-e192.
12. Baumgartner H, Hung J, Bermejo J, et al; American Society of Echocardiography; European Association of Echocardiography. Echocardiographic assessment of valve stenosis: EAE/ASE recommendations for clinical practice. J Am Soc Echocardiogr  2009;22:1-23.
13. Feltes TF, Bacha E, Beekman RH 3rd, et al; American Heart Association Congenital Cardiac Defects Committee of the Council on Cardiovascular Disease in the Young; Council on Clinical Cardiology; Council on Cardiovascular Radiology and Intervention. Indications for cardiac catheterization and intervention in pediatric cardiac disease: a scientific statement from the American Heart Association. Circulation 2011;123:2607–52.
14. Plummer ST, Parthiban A, Sachdeva R, Zaidi AN, Statile C. Clinical Practice Algorithm for the Follow-up or Unrepaired and Repaired Secundum Atrial Septal Defects. http://www.acc.org. Mar 08, 2022. Accessed [insert access date]. https://www.acc.org/Latest-in-Cardiology/Articles/2022/03/08/19/34/Clinical-Practice-Algorithm-For-the-Follow-up-of-Unrepaired-and-Repaired-SASD
15. Sachdeva R, Valente A, Armstrong A, et al. ACC/AHA/ASE/HRS/ISACHD/SCAI/SCCT/SCMR/SOPE 2020 appropriate use criteria for multimodality imaging during the follow-up care of patients with congenital heart disease. J Am Coll Cardiol 2020;75:657–703.

Clinical Topics: Cardiovascular Care Team, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Congenital Heart Disease, CHD and Pediatrics and Quality Improvement

Keywords: Infant, Newborn, Pregnancy, Cardiologists, Goals, Outpatients, Consensus, Patient Discharge, Conservative Treatment, Follow-Up Studies, Electrocardiography, Heart Septal Defects, Atrial, Prognosis, Cyanosis, Hemodynamics, Referral and Consultation, Dyspnea, Catheters, Fatigue, Algorithms, Catheterization, Hypertrophy

< Back to Listings