Abstract

Lung and respiratory health is particularly important in patients living with single ventricle physiology after the Fontan operation. Optimal breathing mechanics achieved through singing have demonstrated benefit in many forms of lung disease and have the potential to improve the health status of children with the Fontan operation.

Posture, muscle and diaphragm strength improvements are known to occur in trained singers and choristers. These benefits have the potential to improve the exercise capacity and wellness of children following the Fontan operation. Although widely employed in adults with asthma, cystic fibrosis, and chronic lung disease, no one to date has trialed singing therapy in children with Fontan physiology.  

The Bienen School of Music and Lurie Children’s Division of Cardiology have collaborated to assess the impact of music instruction in children and teenagers living with Fontan circulation. In conjunction with experienced singing instructors, exercise physiologists, physical therapists, pulmonologists, and single ventricle cardiologists, an introductory program providing both on-site and online singing instruction has been developed. As this represents both a novel and experimental undertaking, close monitoring and observation under an approved Lurie and Northwestern Investigational protocol is required.

Details

The Fontan operation is the recognized definitive palliative therapy for children with single ventricle anatomy1. Despite ongoing attempts to optimize  medical and surgical approaches, the risk of Fontan failure is progressive and unrelenting 2,3. Because of the nature and consequences of the disorder many individuals with single ventricle physiology have a reduction in exercise capacity4 and quality of life 5,6. Singing may provide an appropriate method of exercise and thus improve physical capability. It may also provide a means of emotional expression and community engagement, which may enhance well-being and quality of life (7,8).

Objectives

To evaluate the effects of singing as an adjunctive therapy to standard treatment on the quality of life, measures of morbidity, respiratory muscle strength, and pulmonary function of children and adolescents with single ventricle physiology.  

Feasibility Study

This feasibility study proposes to provide 30 minutes of one-on-one and 1 hour of group instruction weekly for 8 weeks for 2 groups of 12 patients each (ages: 9-11 years and 13-15 years of age). Prior to, and following the intervention, measures of physical exercise capacity, laboratory parameters of organ function and wellness assessments will be performed. Following completion of this feasibility study a multicenter study will be proposed.

References: 

1.          d'Udekem Y, Iyengar AJ, Cochrane AD, et al. The Fontan procedure: contemporary techniques have improved long-term outcomes. Circulation. Sep 11 2007;116(11 Suppl):I157-64. doi:10.1161/CIRCULATIONAHA.106.676445

2.          Goldberg DJ, Shaddy RE, Ravishankar C, Rychik J. The failing Fontan: etiology, diagnosis and management. Expert Rev Cardiovasc Ther. Jun 2011;9(6):785-93. doi:10.1586/erc.11.75

3.          Khairy P, Fernandes SM, Mayer JE, Jr., et al. Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery. Circulation. Jan 1 2008;117(1):85-92. doi:10.1161/CIRCULATIONAHA.107.738559

4.          Sutherland N, Jones B, d'Udekem Y. Should We Recommend Exercise after the Fontan Procedure? Heart Lung Circ. Aug 2015;24(8):753-68. doi:10.1016/j.hlc.2015.03.005

5.          Marshall KH, D'Udekem Y, Sholler GF, et al. Health-Related Quality of Life in Children, Adolescents, and Adults With a Fontan Circulation: A Meta-Analysis. J Am Heart Assoc. Mar 17 2020;9(6):e014172. doi:10.1161/JAHA.119.014172

6.          Grosch IB, Andresen B, Diep LM, Diseth TH, Moller T. Quality of life and emotional vulnerability in a national cohort of adolescents living with Fontan circulation. Cardiol Young. Jun 2022;32(6):874-882. doi:10.1017/S1047951121003188

7.          Whitehead L, Arabiat RND, Foster M. Singing as an adjunct therapy for children and adults with cystic fibrosis: A Cochrane review summary. Int J Nurs Stud. Jun 2018;82:163-164. doi:10.1016/j.ijnurstu.2017.09.007

8.          Lewis A, Philip KEJ, Lound A, Cave P, Russell J, Hopkinson NS. The physiology of singing and implications for 'Singing for Lung Health' as a therapy for individuals with chronic obstructive pulmonary disease. BMJ Open Respir Res. Nov 2021;8(1)doi:10.1136/bmjresp-2021-000996