Somatic growth in single ventricle patients: A systematic review and meta‐analysis

Abstract

Aim:To map somatic growth patterns throughout Fontan palliation and summarise evidence on its key modifiers.Methods: Databases were searched for relevant articles published from January 2000 to December 2021. Height and weight z scores at each time point (birth, Glenn procedure, Fontan procedure and >5 years after Fontan completion) were pooled using a random effects meta-analysis. A random effects meta-regression model was fitted to model the trend in z scores over time.Results: Nineteen studies fulfilled eligibility criteria, yielding a total of 2006 participants. The z scores for height and weight were markedly reduced from birth to the interstage period, but recovered by about 50% following the Glenn procedure. At >10 years after the Fontan procedure, the z scores for weight seemed to normalise despite persistent lower height, resulting in increased body mass index. The review revealed a number of modifiers of somatic growth, including aggressive nutritional management, timing of Glenn/Fontan, prompt resolution of complications and obesity prevention programmes in adolescence and adulthood. | 187Van DEN EYNDE et al. Conclusion:This review mapped the somatic growth of single ventricle patients and summarised key modifiers that may be amendable to improvement. These data provide guidance on strategies to further optimise somatic growth in this population and may serve as a benchmark for clinical follow-up. K E Y W O R D Scongenital heart disease, development, Fontan procedure, functionally univentricular hearts, growth Key notes• In single ventricle patients, the z scores for height and weight were markedly reduced from birth to interstage period, but recovered by about 50% following the Glenn procedure.• At >10 years after the Fontan procedure, the z scores for weight seemed to normalise despite persistent lower height.

Issue highlights

“…A systematic review and meta-analysis by Van den Eynde et al maps the somatic growth of patients with a single ventricle from birth, through the various stages of Fontan palliation and long-term after surgery. 3 The review also reveals modifiers of somatic growth, including aggressive nutritional management, the timing of Glenn/Fontan procedures, the prompt resolution of complications and preventing obesity in adolescence and adulthood. Möller comments on the findings.…”

Development of Weight and Height Age z-Score after Total Cavopulmonary Connection

“…During childhood, growth is a fundamental measure of response to therapy [3,4]. Thus, the weight and height of children with univentricular hearts who have undergone Fontan procedure were studied to determine the effect of each intervention on growth [5][6][7][8][9][10][11][12][13][14][15][16]. It is generally known that early surgical and catheter intervention and early removal of volume loading and cyanosis leads to improvement of somatic growth [6][7][8].…”

Somatic growth in congenital heart disease—A balance between genetic potential and treatment effects

“…Between 2 and 4 years of age, the Fontan procedure provides a serial circulation with the single ventricle responsible for systemic blood flow, while the lung blood flow is passive and depends on an elevated venous pressure as the driving force. However, the Fontan circulation has its serious limitations and it is prone to failure sooner or later in many patients, with somatic growth failure being part of the late 'Fontan failure' which may herald the need for heart transplantation.In this issue, Van den Eynde et al9 present a thorough metaanalysis of previous data on somatic growth in different phases throughout the life of patients with single ventricle physiology.Hereby, the authors have tried to direct unsolved questions or conflicting data about growth and associations with other main characteristics of this special population. Due to the stepwise physiological transformation from a parallel to a serial circulation during the Fontan-type surgical approach, there are different growth patterns during different phases of the surgical palliation.…”

“…In this issue, Van den Eynde et al 9 present a thorough meta‐analysis of previous data on somatic growth in different phases throughout the life of patients with single ventricle physiology. Hereby, the authors have tried to direct unsolved questions or conflicting data about growth and associations with other main characteristics of this special population.…”

“…The authors' graphical presentation of somatic growth illustrates the association of growth to both surgical milestones and to elapsed time after Fontan completion (ref. 9: figure 2). Because the surgical steps are performed at slightly different ages depending on to individual factors and institutional tradition, the first part of the growth pattern is displayed at time of birth and initial surgical rescue, Glenn operation and Fontan operation.…”