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The Duke Activity Status Index is used to assess an individual patient’s perception of their fitness abilities. It has been validated and shown to predict actual fitness in adults but has been studied less in the paediatric population, specifically those with heart disease. This study aims to assess if the Duke Activity Status Index is associated with measured markers of physical fitness in adolescents and young adults with heart disease.
Methods:
This retrospective single-centre cohort study includes patients who completed a minimum of 12 weeks of cardiac rehabilitation between 2016 and 2022. Cardiac rehabilitation outcomes included physical, performance, and psychosocial measures. A comparison between serial testing was performed using a paired t-test. Univariable and multivariable analyses for Duke Activity Status Index were performed. Data are reported as median [interquartile range].
Results:
Of the 118 participants (20 years-old [13.9–22.5], 53% male), 33 (28%) completed at least 12 weeks of cardiac rehabilitation. Median peak oxygen consumption was 60.1% predicted [49–72.8%], and Duke Activity Status Index was 32.6 [21.5–48.8]. On Pearson’s correlation assessing the Duke Activity Status Index, there were significant associations with % predicted peak oxygen consumption (r = 0.49, p < 0.0001), 6-minute walk distance (r = 0.45, p < 0.0001), Duke Activity Status Index metabolic equivalents (r = 0.45, p < 0.0001), and dominant hand grip (r = 0.48, p < 0.0001). In multivariable analysis, the % predicted peak oxygen consumption (r = 0.40, p = 0.005) and dominant hand grip (r = 0.37, p = 0.005) remained statistically significant.
Conclusions:
Duke Activity Status Index is associated with measures of physical fitness in paediatric and young adults with heart disease who complete a cardiac rehabilitation program.
Patients with single-ventricle CHD undergo a series of palliative surgeries that culminate in the Fontan procedure. While the Fontan procedure allows most patients to survive to adulthood, the Fontan circulation can eventually lead to multiple cardiac complications and multi-organ dysfunction. Care for adolescents and adults with a Fontan circulation has begun to transition from a primarily cardiac-focused model to care models, which are designed to monitor multiple organ systems, and using clues from this screening, identify patients who are at risk for adverse outcomes. The complexity of care required for these patients led our centre to develop a multidisciplinary Fontan Management Programme with the primary goals of earlier detection and treatment of complications through the development of a cohesive network of diverse medical subspecialists with Fontan expertise.
A transannular patch is often used in the contemporary surgical repair of tetralogy of Fallot. This can lead to significant pulmonary insufficiency and increased right ventricular volumes and ultimately pulmonary valve replacement. Cardiopulmonary exercise testing is used to assess exercise capacity in tetralogy of Fallot patients before pulmonary valve replacement. There is only few published literatures on how lung function affects functional capacity in tetralogy of Fallot patients repaired with a transannular patch.
Methods
A retrospective chart review was done from 2015 to 2017 on patients with tetralogy of Fallot who underwent maximal effort cardiopulmonary exercise testing with cycle ergometry and with concurrent pulmonary function testing. Tetralogy of Fallot patients repaired with a transannular patch without pulmonary valve replacement were compared with age, gender, and size-matched normal controls.
Results
In the tetralogy of Fallot group, 24 out of 57 patients underwent primary repair with a transannular patch. When compared to the normal controls, they demonstrated abnormal predicted forced expiratory volume in one second (79 ± 23.1% versus 90.7 ± 14.1%, p<0.05), predicted maximal voluntary ventilation (74 ± 18% versus 90.5 ± 16.2%, p<0.05) while having low-normal predicted forced vital capacity (80.5 ± 17.2% versus 90.2 ± 12.4%, p<0.05) and normal breathing reserve percentage (50.3 ± 11.3% versus 47.5 ± 17.3%, p = 0.52). Cardiopulmonary exercise testing abnormalities included significantly lower percent predicted oxygen consumption (63.2 ± 12.2% versus 87 ± 12.1%, p<0.05), maximal heart rate (171.8 ± 18.9 versus 184.6 ± 13.6, p<0.05), and percent predicted maximum workload (61.7 ± 15.9% versus 88.3 ± 21.5%, p<0.05).
Conclusions
Tetralogy of Fallot patients repaired with a transannular patch can have abnormal pulmonary function testing with poor exercise capacity in addition to chronotropic incompetence and impaired muscular power.
By
Clifford Chin, M.D. Department of Pediatrics, Division of Pediatric Cardiology, Lucile Packard Children's Hospital, Palo Alto, California 94304, USA
Ms. L was pregnant at 37 years of age. Amniocentesis, performed because of her advanced maternal age, revealed a normal 46 XY chromosome analysis. At 34 weeks estimated gestational age she underwent a routine obstetrical ultrasound which revealed an abnormal four-chamber image of the fetus' heart. A complete fetal echocardiogram revealed a small left atrium and ventricle with mitral atresia, and a hypoplastic aorta without forward flow from the left ventricle to the ascending aorta. The coronary arteries and head and neck vessels were supplied by a widely patent ductus arteriosus. A diagnosis of hypoplastic left heart syndrome (HLHS) was made.
As HLHS carries a grave prognosis if untreated, the parents were given the following options: (1) no treatment, (2) conventional surgical approach (Norwood procedure), or (3) heart transplantation. After much discussion with the pediatric cardiologists at the local children's hospital, the family opted for heart transplantation and the fetus was placed on the heart transplant waiting list at 36 weeks gestation. If an appropriate donor heart became available, Ms. L would undergo a cesarean section, the infant would be immediately resuscitated and started on prostaglandin to maintain ductal patency, and would then undergo the heart transplant operation.
Ms. L gave birth at 38 weeks estimated gestational age. A complete neonatal team was present in the delivery room and observed that the neonate was cyanotic with an oxygen saturation of 80% in room air. The umbilical vein was cannulated and prostaglandin therapy instituted.
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