Pediatr Cardiol DOI 10.1007/s00246-015-1156-y

ORIGINAL ARTICLE

Clinical Profile and Quality of Life of Adult Patients After the Fontan Procedure Giulia Bordin1 • Massimo Antonio Padalino2 • Sonja Perentaler1 • Biagio Castaldi1 • Nicola Maschietto1 • Pierantonio Michieli3 • Roberto Crepaz4 • Anna Chiara Frigo5 • Vladimiro Lorenzo Vida2 • Ornella Milanesi1

Received: 23 November 2014 / Accepted: 24 March 2015  Springer Science+Business Media New York 2015

Abstract Increasingly, more patients with univentricular heart reach adulthood. Therefore, long-term psychological features are an important concern. The aim of this study was to evaluate the clinical and psychological profile of post-Fontan adult patients and to identify the most significant determinants of quality of life. In this retrospective cross-sectional study, we reviewed the surgical and medical history of post-Fontan adult patients. Patients underwent a 24-h electrocardiogram, echocardiography and exercise testing. Self-report questionnaires were used to assess the Work Ability Index, quality of life (Satisfaction with Life Scale), perceived health status (SF-36 questionnaire), coping strategies (Brief Cope questionnaire) and presence of mood disorders (Hospital Anxiety and Depression Scale). Thirty-nine patients aged between 18 and 48 years (mean 27.5 years) were enrolled. The mean follow-up was 21.5 years. Most patients were unmarried (82.9 %), had a high school diploma (62.9 %) and were employed (62.9 %). Twenty-nine patients

& Giulia Bordin [email protected] 1

Pediatric Cardiology Unit, Department of Women’s and Children’s Health, University of Padua, Via Giustiniani 3, 35128 Padua, Italy

2

Pediatric and Congenital Cardiac Surgery Unit, Department of Thoracic, Cardiac and Vascular Sciences, University of Padua, Padua, Italy

3

Sport Medicine Department, Hospital of Padua, Padua, Italy

4

Department of Cardiology, Bolzano Hospital, Bolzano, Italy

5

Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, Padua, Italy

(82.3 %) had at least one long-term complication. The median single ventricle ejection fraction was 57 %, and the median maximal oxygen consumption was 26.8 ml/ min/kg. This population tended to be anxious and to use adaptive coping strategies. Quality of life was perceived as excellent or good in 57.2 % of cases and was not related to either cardiac function or exercise capacity. Both quality of life and SF-36 domains were related to the Work Ability Index. This cohort of post-Fontan adult patients enjoyed a good quality of life irrespective of disease severity. Keywords of life

Fontan procedure  Follow-up study  Quality

Introduction Since the advent of the surgical repair of tricuspid atresia described by Fontan and Baudet in 1971, various modifications have improved the efficiency of the procedure [2, 4, 17]. Consequently, a large number of patients with single ventricle physiology now reach adulthood [21]. However, long-term complications, mostly related to high venous pressure [8], remain a problem, and new psychosocial, behavioral and emotional issues are emerging [5]. Many studies have evaluated quality of life (QOL) in congenital heart diseases [14, 16, 20] but only a few investigated QOL in patients with Fontan circulation, and none were conducted in Italy. The aim of this study was first to provide an overview of the clinical and psychosocial profile of post-Fontan patients who reach adulthood, and to assess their QOL, and secondly to identify the variables and conditions that most affect the QOL of these patients.

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Materials and Methods Study Population This retrospective cross-sectional study was conducted between May 2011 and November 2012. Two centers participated in the study: the Pediatric Cardiology Unit of the University of Padua and the Department of Cardiology of the Bolzano Hospital. The study protocol was approved by the institutional ethics committees, and patients’ written informed consent was collected. Inclusion criteria were: diagnosis of functional single ventricle physiology palliated with the Fontan procedure, age [18 years, living in Italy, and monitored in one of the centers participating in the study. Patients with chromosome syndrome were excluded. Each patient was invited to a follow up evaluation that included physical examination, electrocardiogram and 24-h electrocardiogram, echocardiography, exercise testing and psychological interview. The following demographic variables were recorded: age, gender, education, social status and employment status. Medical and surgical records were examined for: primary diagnosis, type of systemic ventricle, number of surgical procedures, number of cardiopulmonary bypasses, type of Fontan connection, age at repair, number of medications per day and oxygen saturation percentage (SpO2). Complications The following long-term complications were recorded: •

• • • •

arrhythmias, defined as one of the following: the assumption of antiarrhythmic therapy, a permanent pacemaker or a 24-h electrocardiogram positive for supraventricular tachycardia, atrial flutter or atrial fibrillation; protein-losing enteropathy; plastic bronchitis; motor disabilities; hepatic dysfunction, defined as at least two altered values among the following: serum aspartate aminotransferase, serum alanine aminotransferase, serum cglutamyl transferase, serum bilirubin and the spontaneous international normalized ratio (for patients not on anticoagulant therapy).

Echocardiography Each patient underwent two-dimensional echocardiography with color Doppler, tissue Doppler velocity profiles and M-mode recordings. The systolic function was evaluated

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with the ejection fraction calculated using a biplane-modified Simpson rule and with tissue Doppler peak systolic velocity (S0 ). Ventricular diastolic function [19] was assessed by measuring atrioventricular valve peak early diastolic inflow velocity (E), atrioventricular valve peak late diastolic inflow velocity (A), the E/A ratio, tissue Doppler peak early diastolic velocity (E0 ), tissue Doppler peak late diastolic velocity (A0 ) and the E/E0 ratio. Tissue Doppler measurements were taken from the atrioventricular annulus of the free wall of the dominant ventricle. Exercise Performance A bicycle or treadmill ergometry was used to assess exercise performance. Maximal oxygen consumption (VO2max—expressed as ml/min/kg) served as index of exercise capacity. Ability at Work We used the Work Ability Index questionnaire [12] to determine the self-assessed work ability in employed patients. This instrument is constituted by seven domains for a total score that ranges between 7 (poor work ability) and 49 (excellent work ability). Anxiety and Depression The Hospital Anxiety and Depression Scale was used to identify mood disorders [26]. It consists of two subscales that generate two separate total scores: one for anxiety and one for depression; both range from 0 to 21. Depending on the score, patients are defined ‘‘case,’’ ‘‘not case’’ and ‘‘possible case’’ of anxiety and depression. Coping Strategies The Brief Cope questionnaire was used to assess the patients’ coping strategies and their capacity to react to stress. It consists of 28 questions that measure 14 coping strategies [3]: positive reframing, self-distraction, venting, use of instrumental support, active coping, denial, religion, humor, behavioral disengagement, use of emotional support, substance use, acceptance, planning and self-blame. Quality of Life Quality of life is a multidimensional construct that consists of somatic, behavioral, emotional, cognitive and psychosocial dimensions of life. According to Moons et al. [20], QOL is ‘‘the degree of overall life satisfaction, which is positively or negatively influenced by an individual’s perception of certain aspects of life important to them,

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including matters both related and unrelated to health.’’ Therefore, we evaluated life satisfaction as an indicator of overall QOL using the Satisfaction with Life Scale Questionnaire [6]. This questionnaire contains five statements; each one has seven response categories going from ‘‘strongly disagree’’ to ‘‘strongly agree.’’ The scale generates a total score ranging from 5 to 35. Its psychometric reliability and validity are well recognized. Perceived Health Status Perceived health status was defined as an individual’s perceived impact of disease on daily function and wellbeing in physical, mental and social domains of health [23]. Perceived health status was assessed with the Short Form 36 (SF-36), which has reliable psychometric properties [18]. It comprises 36 items, divided into eight domains: physical function, role-physical function, bodily pain, general health, vitality, social functioning, role-emotional functioning and mental health. A score ranging from 0 to 100 is generated for each domain.

number of medications per day, oxygen saturation, presence of long-term complications) cardiac function score VO2max Work Ability Index score Hospital Anxiety and Depression Scale scores.

• • • •

The cardiac function score (see Table 1) and the maximal oxygen consumption (VO2max) were compared, using the Mann–Whitney test or Spearman’s correlation as appropriate, with age, age at repair, type of repair (Bjo¨rk repair, intracardiac and extracardiac), employment status and presence of long-term complications. Cardiac function score was categorized into low and high cardiac function based on the median value. No multivariate analysis has been made because of the small numerosity of the study population. Statistical significance was set at p value 0.05. The SAS software version 9.2 for Windows (SAS Institute inc., Cary, NC, USA) was used for data processing and statistical analysis.

Results

Statistical Analysis Categorical variables are expressed as absolute frequency and percentage. Continuous variables are expressed as means and standard deviations or median and quartiles (Q1–Q3) depending on their distribution. The SF-36 results were expressed as mean and standard deviation, irrespective of their distribution. For the statistical analysis, a cardiac function score was devised as the sum of four function indexes (ejection fraction, S0 , E0 and E/E0 ) on a scale from 0 to 2, and summing the four values (Table 1). The Mann–Whitney test or Spearman’s correlation was used, as appropriate, to evaluate correlations between the replies to the Satisfaction with Life Scale Questionnaire and the SF-36 and the following variables:

Patients Two centers participated in the survey for a total of 35 enrolled patients [mean age 27.5 ± 7.6; 12 (34.3 %) female]. Their social and demographic data and clinical and surgical data are listed in Tables 2 and 3. Most patients were unmarried (82.9 %), had a high school diploma (62.9 %) and were employed (62.9 %). Most had a morphologic left ventricle (29; 82.9 %), 19 (54.3 %) had a primary diagnosis of tricuspid atresia, and 29 (82.9 %) had a total cavo-pulmonary connection (16 patients with an intracardiac conduit and 13 with an extracardiac conduit). Table 2 Sociodemographic data of the cohort



sociodemographic data: age, gender, education, job status clinical and surgical records (type of ventricle, type of Fontan procedure, age at repair, number of surgical procedures, number of cardiopulmonary bypasses,



Variable Gender

n (%) or average ± SD/ median (Q1*–Q3 ) Male Female

Age Social status Table 1 Cardiac function score Cardiac function score

0

1

2

EF

\0.35

0.35–0.55

C0.55

S0

B4

4–9

C9

B4

4–9

C9

\15

8–15

\8

E0 0

E/E

EF ejection fraction

Education

Employment status

*

23 (65.7 %) 12 (34.3 %) 27.5 ± 7.6

Unmarried Married or coupled

29 (82.9 %) 6 (17.1 %)

Secondary school

11 (31.4 %)

High school diploma

22 (62.9 %)

University degree

2 (5.7 %)

Student

6 (17.1 %)

Employed

22 (62.9 %)

Unemployed

7 (20.0 %)

 

Q1 first quartile, Q3 third quartile, SD standard deviation

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Pediatr Cardiol Table 3 Clinical and surgical data of the cohort n (%) or average ± SD/median (Q1*–Q3 )

Variable Primary diagnosis

Ventricle

Pre-Fontan interventions

Fontan type

TA

19 (54.3 %)

DILV

6 (17.1 %)

DORV

2 (5.7 %)

PA-IVS

3 (8.6 %)

HLHS

1 (2.9 %)

CAVC

2 (5.7 %)

Others

2 (5.7 %)

Right

4 (11.4 %)

Left

29 (82.9 %)

Undetermined

2 (5.7 %)

Glenn Blaloch-Taussig shunt/systemic shunt

7 (20.0 %) 20 (57.1 %)

Norwood, first stage

1 (2.9 %)

Pulmonary artery banding

4 (11.4 %)

Others

3 (8.6 %)

Atriopulmonary anastomosis Bjo¨rk

0 (0.0 %)

Intracardiac fenestrated TCPC

7 (20.0 %)

Intracardiac non-fenestrated TCPC

8 (22.8 %)

6 (17.1 %)

Extracardiac fenestrated TCPC

2 (5.7 %)

Extracardiac non-fenestrated TCPC

1 (2.9 %)

Redo Fontan with an intracardiac conduit

1 (2.9 %)

Redo Fontan with an extracardiac conduit

10 (28.6 %)

Number of interventions

2 (2–3)

Number of CPBs

2 (1–2)

Age at repair

Non-redo patients

2.5 (1–8.8)

Redo patients

23 (16.5–29.3) 90 ± 3 %

Oxygen saturation Number of medications

2 (1–4)

Arrhythmias

20 (57.1 %)

Permanent PM

9 (25.7 %)

Protein-losing enteropathy

1 (2.9 %)

Plastic bronchitis

0

Neurological sequelae

3 (8.6 %)

Hepatic dysfunction

15 (42.9 %)

Q1* first quartile, Q3  third quartile, SD standard deviation, TA tricuspid atresia, DILV double inlet left ventricle, DORV double outlet right ventricle, PA-IVS pulmonary atresia–intact ventricular septum, HLHS hypoplastic left heart syndrome, CAVC common atrioventricular canal, TCPC total cavo-pulmonary connection, CPBs cardiopulmonary bypasses, PM pacemaker

Patients underwent a median of two interventions and two cardiopulmonary bypasses, took a median of two medications per day and had a SpO2 of 90 %. The median age at repair was 23 years for patients who underwent redo Fontan surgery after the first repair and 2.5 years for the other patients. Twenty-nine patients had at least one longterm complication: 20 (57.1 %) had arrhythmias of whom nine (25.7 %) had a permanent pacemaker leading the cardiac rhythm in five at the follow-up evaluation. One patient (2.9 %) had protein-losing enteropathy, and three

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(8.6 %) had motor disabilities after cardiac surgery or cardiac catheterization. Fifteen patients (42.9 %) had hepatic dysfunction. Plastic bronchitis was not reported in our cohort. Echocardiography and Exercise Performance Echocardiographic and exercise testing data are listed in Table 4. Mean ejection fraction was 57 %. Tissue Doppler velocity profiles were collected in 27 patients: Three

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patients were not on sinus rhythm at the follow-up, and data were not available in five patients. The median E’ velocity was 8 cm/s, and the median E/E’ ratio was 7.9. The cardiac function score (Table 1) was not associated with age (p value = 0.821), age at repair (p value = 1), type of repair (Bjo¨rk repair, intracardiac and extracardiac; p value = 0.234), employment status (p value = 0.29) or long-term complications (p value = 0.383). Twenty patients underwent the exercise test; clinical conditions prevented six patients from undergoing the test; and nine declined the test. The median VO2max was 26.8 ml/min/kg. The VO2max was not related with age at repair (p value = 0.093), type of repair (Bjo¨rk repair, intracardiac and extracardiac, p value = 0.18), employment status (p value = 0.256) or long-term complications (p value = 0.874). Contrary to the cardiac score, there was an inverse correlation between age and VO2max (p value = 0.033, correlation coefficient = -0.478). Work Ability Twenty-two patients (62.9 %) were employed at the time of follow-up, and their median Work Ability Index score was 36. Only three patients (13.6 %) considered their work ability poor; most considered their work ability mediocre (eight patients, 36.4 %) or good (nine patients, 40.9 %). Two subjects (9.1 %) considered their work ability excellent. The Work Ability Index score was not correlated with the cardiac function score described in Table 1 (p value = 0.862). Psychological Profile Our cohort was anxious (median Hospital Anxiety and Depression Scale for anxiety = 13) and to a lesser extent

Table 4 Echocardiographic and exercise testing data Variable

n (%) or average ± SD/ median (Q1*–Q3 )

Systolic function

EF (%) 0

Diastolic function

S (cm/s)

5.7 (4.8–6.5)

E (cm/s)

59.5 (50.3–9.07)

A (cm/s)

38.2 (33.9–51.5)

E/A ratio

1.5 (1.2–1.8)

E0 (cm/s)

8.0 (6.2–10.6)

A0 (cm/s)

4.3 (3.7–5.4)

E0 /A0 ratio E/E0 ratio

2.1 (1.3–2.7) 7.9 (5.8–10.8)

VO2max (ml/min/kg) *

57 ± 12

26.8 (22.5–30.1)

depressed (median Hospital Anxiety and Depression Scale for depression = 8) (see Table 5). We identified 19 (54.3 %) possible cases and five definite (14.3 %) cases of depression and eight (22.9 %) possible cases and 23 definite (65.7 %) cases of anxiety. The Hospital Anxiety and Depression Scale was not correlated with the cardiac function score (see Table 1; p value = 0.574 for depression and 0.485 for anxiety). As shown in Table 5, our cohort tended to use adaptive coping strategies (in particular active coping, planning, acceptance, positive reframing and self-distraction) more than maladaptive coping (i.e., denial, behavioral disengagement or substance use). Quality of Life and Health Status Our cohort enjoyed a good quality of life, with a median score on the Satisfaction with Life Scale of 25. In particular, 12 patients (34.3 %) were satisfied with their lives and eight (22.9 %) were extremely satisfied (Table 5). Both the Satisfaction with Life Scale and SF-36 scores, which measure perceived health status, were not related to surgical history (number of interventions and cardiopulmonary bypasses), type of ventricle (right or left one), number of medications per day or SpO2 (Tables 6, 7). Similarly, they were not related to long-term complications, age at repair, or to demographic or social conditions (age, gender, education, social status and employment status) (Tables 6, 7). Neither the cardiac function score (see Table 1) nor the maximal oxygen consumption (VO2max) value was related to the Satisfaction with Life Scale score (Table 6) or to the SF-36 domains (Table 7). Role-emotional, which is one of the domains explored in the SF-36 questionnaire, was related to the depression subscale of the Hospital Anxiety and Depression Score (p value = 0.015). Finally, the Satisfaction with Life Scale and SF-36 domains were strongly related to the Work Ability Index score (Tables 6, 7).

Discussion Because most patients with univentricular heart now reach adulthood, their psychological outcome has become as important as their clinical outcome. Here, we report the results of the first study of an Italian Fontan population. The mean age of our cohort was 27.5 years, and the mean follow-up was 21.5 years. Functional Status

 

Q1 first quartile, Q3 third quartile SD standard deviation, EF ejection fraction, VO2max maximal oxygen consumption

Systolic function of the single ventricle was normal in our population (mean EF: 57 %), and the VO2max, although

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Pediatr Cardiol Table 5 Outcomes of the five questionnaires administered Questionnaire

Value

SWLS

Frequency (%)

Table 6 Mann–Whitney test and correlation test results for the Satisfaction with Life Scale Questionnaire Variable

p value

Extremely unsatisfied

2 (5.7 %)

Sex

1.0

Unsatisfied

4 (11.4 %)

Age

0.465

Mildly unsatisfied

3 (8.6 %)

Employment status

0.207

Mildly satisfied

6 (17.1 %)

Ventricle

0.472

12 (34.3 %)

Interventions

0.427

8 (22.9 %)

CPBs

0.063

Average ± SD 81.1 ± 21.9

Long-term complications SpO2 %

0.861 0.299

Role-physical function

75.0 ± 32.7

Medications

0.747

Bodily pain General health

79.2 ± 26.4 67.1 ± 24.3

Cardiac function score

0.335

VO2max

0.191

Satisfied Extremely satisfied SF-36 Physical function

*C (if applicable)

?0.128

?0.183

-0.305

Vitality

64.7 ± 18.7

WAI

Clinical Profile and Quality of Life of Adult Patients After the Fontan Procedure.

Increasingly, more patients with univentricular heart reach adulthood. Therefore, long-term psychological features are an important concern. The aim o...
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