Original Article
Heart rate variability in the course of chemotherapy and haematopoietic stem cell transplantation for peadiatric patients with haematological malignancies
- Honami Kobayashi, Noriko Motoki, Saori Yokota, Ayako Kanai, Shoko Yamazaki, Masafumi Utsumi, Yozo Nakazawa
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- Published online by Cambridge University Press:
- 29 May 2020, pp. 967-974
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Background:
High-dose chemotherapy and haematopoietic stem cell transplantation are essential for patients with paediatric haematologic diseases, although cardiotoxicity remains a concern. Heart rate variability analysis can evaluate autonomic nervous function interactions with cardiac function.
Objective:This study aimed to characterise heart rate variability differences between patients undergoing chemotherapy and controls, and the effects of haematopoietic stem cell transplantation on the autonomic nervous system in patients with haematological malignancies.
Methods:Nineteen patients (11 male, median age: 11.6 years) who received conventional chemotherapy followed by transplantation and 19 non-transplant patients (10 male, median age: 11.5 years) receiving chemotherapy only between 2006 and 2018 for haematological malignancies were retrospectively enrolled. Data from 24-hour Holter monitoring were recorded after chemotherapy and before and after transplantation. Heart rate variability was analysed in patients and 32 matched normal controls.
Results:There were significant differences between patients and normal controls in all heart rate variability analysis parameters apart from coefficient of variation of RR interval and standard deviation of the average normal RR interval for all 5-minute segments during sleeping. There was a significant difference in the cumulative anthracycline dose and heart rate variability during sleep between the non-transplant and pre-transplant groups. We observed no remarkable differences in time-domain analysis parameters between before and after transplantation, although the low-frequency component of power-spectrum analysis during awake hours was significantly decreased after transplantation.
Conclusion:Conventional chemotherapy for paediatric haematologic diseases may be a risk factor for autonomic dysfunction. Further declines in heart rate variability after transplantation appear minor.
Registry-based trials: a potential model for cost savings?
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- Brett R. Anderson, Evelyn G. Gotlieb, Kevin Hill, Kimberly E. McHugh, Mark A. Scheurer, Carlos M. Mery, Glenn J. Pelletier, Jonathan R. Kaltman, Owen J. White, Felicia L. Trachtenberg, Danielle Hollenbeck-Pringle, Brian W. McCrindle, Donna M. Sylvester, Aaron W. Eckhauser, Sara K. Pasquali, Jeffery B. Anderson, Marcus S. Schamberger, Subhadra Shashidharan, Jeffrey P. Jacobs, Marshall L. Jacobs, Marko Boskovski, Jane W. Newburger, Meena Nathan
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- Published online by Cambridge University Press:
- 08 May 2020, pp. 807-817
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Background/Aims:
Registry-based trials have emerged as a potentially cost-saving study methodology. Early estimates of cost savings, however, conflated the benefits associated with registry utilisation and those associated with other aspects of pragmatic trial designs, which might not all be as broadly applicable. In this study, we sought to build a practical tool that investigators could use across disciplines to estimate the ranges of potential cost differences associated with implementing registry-based trials versus standard clinical trials.
Methods:We built simulation Markov models to compare unique costs associated with data acquisition, cleaning, and linkage under a registry-based trial design versus a standard clinical trial. We conducted one-way, two-way, and probabilistic sensitivity analyses, varying study characteristics over broad ranges, to determine thresholds at which investigators might optimally select each trial design.
Results:Registry-based trials were more cost effective than standard clinical trials 98.6% of the time. Data-related cost savings ranged from $4300 to $600,000 with variation in study characteristics. Cost differences were most reactive to the number of patients in a study, the number of data elements per patient available in a registry, and the speed with which research coordinators could manually abstract data. Registry incorporation resulted in cost savings when as few as 3768 independent data elements were available and when manual data abstraction took as little as 3.4 seconds per data field.
Conclusions:Registries offer important resources for investigators. When available, their broad incorporation may help the scientific community reduce the costs of clinical investigation. We offer here a practical tool for investigators to assess potential costs savings.
Heart failure biomarker levels correlate with invasive haemodynamics in pulmonary valve replacement
- Phillip M. Zegelbone, Richard E. Ringel, John D. Coulson, Melanie K. Nies, Meagan E. Stabler, Jeremiah R. Brown, Allen D. Everett
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- Published online by Cambridge University Press:
- 27 November 2019, pp. 50-54
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Background:
Although widely used in cardiology, relation of heart failure biomarkers to cardiac haemodynamics in patients with CHD (and in particular with pulmonary insufficiency undergoing pulmonary valve replacement) remains unclear. We hypothesised that the cardiac function biomarkers N-terminal pro-brain natriuretic peptide (NT-proBNP), soluble suppressor of tumorigenicity 2, and galectin-3 would have significant associations to right ventricular haemodynamic derangements.
Methods:Consecutive patients ( n = 16) undergoing cardiac catheterisation for transcatheter pulmonary valve replacement were studied. NT-proBNP, soluble suppressor of tumorigenicity 2, and galectin-3 levels were measured using a multiplex enzyme-linked immunosorbent assay from a pre-intervention blood sample obtained after sheath placement. Spearman correlation was used to identify significant correlations (p ≤ 0.05) of biomarkers with baseline cardiac haemodynamics. Cardiac MRI data (indexed right ventricular and left ventricular end-diastolic volumes and ejection fraction) prior to device placement were also compared to biomarker levels.
Results:NT-proBNP and soluble suppressor of tumorigenicity 2 were significantly correlated (p < 0.01) with baseline mean right atrial pressure and right ventricular end-diastolic pressure. Only NT-proBNP was significantly correlated with age. Galectin-3 did not have significant associations in this cohort. Cardiac MRI measures of right ventricular function and volume were not correlated to biomarker levels or right heart haemodynamics.
Conclusions:NT-proBNP and soluble suppressor of tumorigenicity 2, biomarkers of myocardial strain, significantly correlated to invasive pressure haemodynamics in transcatheter pulmonary valve replacement patients. Serial determination of soluble suppressor of tumorigenicity 2, as it was not associated with age, may be superior to serial measurement of NT-proBNP as an indicator for timing of pulmonary valve replacement.
Comparison of growth and feeding method in infants with and without genetic abnormalities after neonatal cardiac surgery
- Alyson R. Pierick, Trudy A. Pierick, Benjamin E. Reinking
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- Published online by Cambridge University Press:
- 25 September 2020, pp. 1826-1832
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Introduction:
Congenital heart disease (CHD) is multifactorial in origin, resulting from an interaction between environmental and genetic factors. Multifactorial growth delay is common in infants with CHD. The impact of a genetic abnormality and CHD on the growth of an infant is lacking in the literature. The aim of this study is to compare the growth and method of feeding following neonatal cardiac surgery in infants with normal versus abnormal genetic testing.
Methods:A retrospective chart review of neonates who underwent a Risk Adjustment in Congenital Heart Surgery IV–VI procedure between 1 January, 2006 and 22 September, 2016 was performed at our institution. Weight, length, head circumference measurements, and feeding method were collected at birth, time of neonatal surgery, and monthly up to 6 months of age.
Results:A total of 53 infants met inclusion criteria, of which 22 had abnormal genetic testing. Approximately 90% of infants were discharged following neonatal cardiac surgery with supplemental tube feeds. At each monthly follow-up visit, more infants were exclusively fed orally: 80% of infants with normal genetics at 5 months post-operative follow-up versus 60% of infants with abnormal genetic testing, although statistically insignificant. Growth was not different among the two groups.
Conclusions:Infants with critical CHD with or without genetic abnormalities are at risk for growth delays and many need supplemental tube feeds post-operatively and throughout follow-up. Infants with genetic abnormalities are slower to achieve oral feeds and more likely to require tube feedings. It is important to have a systematic protocol for managing these high-risk infants.
RACHS-1 score as predictive factor for postoperative ventilation time in children with congenital heart disease
- Luisa Geier, Christoph Menzel, Ingo Germund, Uwe Trieschmann
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- Published online by Cambridge University Press:
- 17 January 2020, pp. 213-218
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Background:
Congenital heart disease is the most frequent malformation in newborns. The postoperative mortality of these patients can be assessed with the Risk Adjustment in Congenital Heart Surgery-1 (RACHS-1) score. This study evaluates whether the RACHS-1 score can also be used as a predictor for the length of postoperative ventilation and what is the influence of age.
Material and Methods:In a retrospective study over the period from 2007 to 2013, all patient records were evaluated: 598 children with congenital heart disease and cardiac surgery were identified and 39 patients have been excluded because of additional comorbidities. For evaluation of mortality, 559 patients could be analysed, after exclusion of 39 deceased patients, 520 cases remained for analysis of postoperative ventilation.
Results:Overall mortality was 7% with a dependency on RACHS-1 categories. The median length of postoperative ventilation rose according to the RACHS-1 categories: RACHS-1 category 1: 9 hours (interquartile range (IQR) 7–13 hours), category 2: 30 hours (IQR 12–85 hours), category 4: 58 hours (IQR 13–135 hours), category 4: 71 hours (IQR 29–165 hours), and category 6: 189 hours (IQR 127–277 hours). Some of the RACHS-1 subgroups differed significantly from the categories, especially the repair of tetralogy of Fallot with a longer ventilation time and strong variability. Younger age was an independent factor for longer postoperative ventilation.
Conclusion:RACHS-1 is a good predictor for the length of postoperative ventilation after cardiac surgery with the exception of some subgroups. Younger age is another independent factor for longer postoperative ventilation. These data provide better insight into ventilation times and allow better planning of operations in terms of available intensive care beds.
3D advanced imaging overlay with rapid registration in CHD to reduce radiation and assist cardiac catheterisation interventions
- Yousef Arar, Surendranath R. Veeram Reddy, Heidi Kim, V. Vivian Dimas, Thomas M. Zellers, Riad Abou Zahr, Ravi Vamsee, Joshua S. Greer, Animesh Tandon, Antonia Pontiki, Jeanne Dillenbeck, Luis Zabala, Gerald Greil, Alan W. Nugent, Tarique Hussain
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- Published online by Cambridge University Press:
- 15 April 2020, pp. 656-662
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Novel commercially available software has enabled registration of both CT and MRI images to rapidly fuse with X-ray fluoroscopic imaging. We describe our initial experience performing cardiac catheterisations with the guidance of 3D imaging overlay using the VesselNavigator system (Philips Healthcare, Best, NL). A total of 33 patients with CHD were included in our study. Demographic, advanced imaging, and catheterisation data were collected between 1 December, 2016 and 31 January, 2019. We report successful use of this technology in both diagnostic and interventional cases such as placing stents and percutaneous valves, performing angioplasties, occlusion of collaterals, and guidance for lymphatic interventions. In addition, radiation exposure was markedly decreased when comparing our 10–15-year-old coarctation of the aorta stent angioplasty cohort to cases without the use of overlay technology and the most recently published national radiation dose benchmarks. No complications were encountered due to the application of overlay technology. 3D CT or MRI overlay for CHD intervention with rapid registration is feasible and aids decisions regarding access and planned angiographic angles. Operators found intraprocedural overlay fusion registration using placed vessel guidewires to be more accurate than attempts using bony structures.
Impact of protein-losing enteropathy in children who underwent the Fontan operation
- Baneen Alkofair, Abdulmajeed Alruwaili, Jiaxiang Gai, Ashraf S. Harahsheh
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- Published online by Cambridge University Press:
- 20 July 2020, pp. 1273-1280
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Previous reports have identified risk factors associated with development of post-Fontan protein-losing enteropathy. Less is known about the economic impact and resource utilisation required for post-Fontan protein-losing enteropathy in the current era. We conducted a single-centre retrospective study to assess the impact of post-Fontan protein-losing enteropathy on transplant-free survival. We also described resource utilisation and treatment variations among post-Fontan protein-losing enteropathy patients. Children who received care at our centre between 2009 and 2017 after the Fontan surgery were eligible. Initial admissions for the Fontan operative procedure were excluded. Demographics, hospital admissions, resource utilisation, medications and charges were reviewed. Patients were divided into two groups based on the presence of post-Fontan protein-losing enteropathy. Of the 343 patients screened, 147 met the eligibility criteria. Of these, 28 (19%) developed protein-losing enteropathy. After adjusting for follow-up duration, the protein-losing enteropathy group had higher number of encounters (2.15 ± 2.16 versus 1.47 ± 2.56, p 0.002), hospital length of stay (days) (25 ± 51.3 versus 11.4 ± 41.7, p < 0.0001) and total charges (2018US$) (388,489 ± 759,859 versus 202,725 ± 1,076,625, p < 0.0001). Encounters for patients with protein-losing enteropathy utilised more therapies. Among those with protein-losing enteropathy, use of digoxin was associated with slightly decreased odds for mortality and/or transplant (0.95, confidence interval 0.90–0.99, p 0.021). The 10-year transplant-free survival for patients with/without protein-losing enteropathy was 65.7/97.3% (p 0.002), respectively. Post-Fontan protein-losing enteropathy is associated with reduced 10-year transplant-free survival, higher resource utilisation, charges and medication use compared with the non-protein-losing enteropathy group. Practice variation among post-Fontan protein-losing-enteropathy patients is common. Further larger studies are needed to assess the impact of standardisation on the well-being of children with post-Fontan protein-losing enteropathy.
Comparison of outcomes following thoracoscopic versus thoracotomy closure for persistent patent ductus arteriosus
- Chen Wei, Steven Staffa, David Zurakowski, Susan Saleeb, Francis Fynn-Thompson, Sitaram M. Emani
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- 05 August 2020, pp. 1433-1438
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Background:
Patent ductus arteriosus closure is traditionally performed by thoracotomy approach. Video-assisted thoracoscopic surgery is a less frequently utilised alternative. We sought to compare elective surgical outcomes between the two methods via a single-centre retrospective cohort analysis.
Methods:All patients >3.2 kg undergoing surgical patent ductus arteriosus ligation at a single institution from 2000 to 2018 were retrospectively reviewed. Propensity matching for age, weight, diuretic usage, and preterm status was conducted to adjust for differences in baseline patient characteristics. Outcome measures included operative time, hospitalisation duration, post-operative complications, and re-operation.
Results:A total of 173 patients were included, 127 thoracoscopy and 46 thoracotomy. In the unmatched cohorts, no significant difference in closure success was found (94% thoracoscopy versus 100% thoracotomy, p = 0.192). Although median operative time was longer for thoracoscopy (87 versus 56 minutes, p < 0.001), hospitalisation duration was shorter (1.05 versus 2.41 days, p < 0.001), as was ICU stay (0.00 versus 0.75 days, p < 0.001). There were no significant differences in re-operation or complication rates, except chest tube placement (11% thoracoscopy versus 50% thoracotomy, p < 0.001). After matching (69 thoracoscopy versus 20 thoracotomy), these differences persisted, including median operative time (81 versus 56 minutes, p = 0.007; thoracoscopy versus thoracotomy), hospitalisation duration (1.25 versus 2.27 days, p < 0.001), and chest tube placement (17% versus 60%, p < 0.001). There remained no significant difference in complications or re-operations.
Conclusions:Thoracoscopic ligation was associated with shorter ICU and hospital stays and less frequent chest tube placement, but longer operative times. Other risks, including bleeding, chylothorax, and recurrent laryngeal nerve injury, were similar.
Health-related quality of life in adult CHD surgical patients in a low middle-income country: a mixed-methods study
- Laila A. Ladak, Robyn Gallagher, Babar S. Hasan, Khadija Awais, Ahmed Abdullah, Janice Gullick
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- Published online by Cambridge University Press:
- 23 June 2020, pp. 1126-1137
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Background and objectives:
This mixed-methods study aimed to assess health-related quality of life in young adults with CHD following surgery in a low middle-income country, Pakistan. Despite the knowledge that geographic, cultural and socio-economic factors may shape the way health and illness is experienced and managed and consequently determine a person’s health-related quality of life, few health-related quality of life studies are conducted in low middle-income countries. This deficit is pronounced in CHD, so there is little guidance for patient care.
Methods:The study utilised concurrent, mixed methods. Adults with CHD (n = 59) completed health-related quality of life surveys (PedsQLTM 4.0 Generic Core Scale, PedsQLTM Cognitive Functioning Scale and PedsQLTM 3.0 Cardiac Module). Semi-structured interview data were collected from a nested sub-sample of 17 participants and analysed using qualitative content analysis, guided by the revised Wilson–Cleary model of health-related quality of life.
Results:The lowest health-related quality of life domain was emotional with the mean score (71.61 ± 20.6), followed by physical (78.81 ± 21.18) and heart problem (79.41 ± 18.05). There was no statistical difference in general or cardiac-specific health-related quality of life between mild, moderate or complex CHD. Qualitative findings suggested low health-related quality of life arose from a reduced capacity to contribute to family life including family income and gender. A sense of reduced marriageability and fear of dependency were important socio-cultural considerations.
Conclusions:CHD surgical patients in this low-income country experience poor health-related quality of life, and contributing factors differ to those reported for high-income countries. Socio-cultural understandings should underpin assessment, management and care-partnering with young adults with CHD following surgical correction.
Effective myocardial perfusion and concomitant haemodynamic status determine the clinical diversity of anomalous left coronary artery from the pulmonary artery
- Amr A. Matoq, Takeshi Tsuda
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- 22 January 2020, pp. 362-368
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Background:
Anomalous left coronary artery from the pulmonary artery is a rare congenital heart disease (CHD) with diverse clinical presentations despite the same anatomy. Factors determining this heterogeneous presentation are not well understood.
Method and Results:We retrospectively investigated 14 patients (12 females) who underwent surgical repair of anomalous left coronary artery from the pulmonary artery. These patients were divided into three groups based upon the severity of initial presentation: (1) severe, life-threatening condition (n = 5), (2) mild-to-moderate distress (n = 6), and (3) asymptomatic (n = 3). All patients presented with left ventricular dilation and retrograde flow in left coronary artery by echocardiogram. Eight patients in (1) and (2) presented with severe left ventricular dysfunction. All but one showed abnormal ECG consistent with myocardial ischemia or infarction. Asymptomatic patients had preserved left ventricular systolic function despite ischemic findings on ECG. In 13 patients after surgical repair, all but one normalised left ventricular geometry and systolic function, suggesting nearly full myocardial recovery upon improvement of myocardial perfusion; 8 patients had residual echogenic papillary muscle with variable degree of mitral regurgitation.
ConclusionsEvidence of myocardial ischemic injury was present in all patients with anomalous left coronary artery from the pulmonary artery regardless of their initial presentation. Retrograde flow in left coronary artery, implying collateral vessel development from right coronary artery to left coronary artery, was noted in all patients, yet only few patients had preserved systolic function at the time of diagnosis. The balance between effective myocardial perfusion and a deleterious fistulous flow provided by these collaterals and the simultaneous haemodynamic status are what determine the clinical diversity of anomalous left coronary artery from the pulmonary artery.
Palivizumab compliance in congenital heart disease patients: factors related to compliance and altered lower respiratory tract infection viruses after palivizumab prophylaxis
- Kutay Sel, Ebru Aypar, Yasemin Nuran Dönmez, Emil Aliyev, Hakan Hayrettin Aykan, Tevfik Karagöz, Dursun Alehan
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- 19 May 2020, pp. 818-821
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Background:
Lower respiratory tract infections caused by respiratory syncytial virus can be severe during infancy, which requires admission to the hospital. These infections may be more severe especially in patients with congenital heart disease. Passive immunisation with palivizumab, a monoclonal antibody, is recommended in high-risk infants. We tried to determine the compliance rates, factors affecting compliance, and also other microorganisms responsible for lower respiratory tract infections after palivizumab prophylaxis in these patients.
Methods:We evaluated patients’ compliance to prophylaxis with palivizumab in two consecutive respiratory syncytial virus seasons from pharmacy records. We also investigated factors affecting compliance and the frequency of hospitalisations for lower respiratory tract infections. We investigated the causative microorganisms detected in hospitalised patients.
Results:In this study, 86.7% of the desired number of injections was achieved in 176 patients in two seasons. Out of these, 117 patients (66.4%) received all the doses they were prescribed. Although not statistically significant, compliance to prophylaxis was higher in male patients, cyanotic patients, those who started under 1 year old, and who lived in the city centre. Human metapneumovirus, parainfluenza type 3, and bocavirus were detected in the hospitalised patients.
Conclusion:Patients with congenital heart disease can survive the period of infancy with less problem by making palivizumab prophylaxis more effective, and awareness about non- respiratory syncytial virus factors may be a guide for the development of new treatments.
Adolescents with congenital heart defects: a patient and parental perspective of genetic information and genetic risk
- Christopher A. Crawford, Courtney E. Vujakovich, Lindsey Elmore, Emily Fleming, Benjamin J. Landis, Katie G. Spoonamore, Stephanie M. Ware
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- Published online by Cambridge University Press:
- 27 January 2020, pp. 219-226
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Congenital heart defects (CHDs) occur in 8 of 1000 live-born children, making them common birth defects in the adolescent population. CHDs may have single gene, chromosomal, or multifactorial causes. Despite evidence that patients with CHD want information on heritability and genetics, no studies have investigated the interest or knowledge base in the adolescent population. This information is necessary as patients in adolescence take greater ownership of their health care and discuss reproductive risks with their physicians. The objectives of this survey-based study were to determine adolescents’ recall of their own heart condition, to assess patient and parent perception of the genetic contribution to the adolescent’s CHD, and to obtain information about the preferred method(s) for education. The results show that adolescent patients had good recall of their type of CHD. Less than half of adolescents and parents believed their CHD had a genetic basis or was heritable; however, adolescents with a positive family history of CHD were more likely to believe that their condition was genetic (p = 0.0005). The majority of patients were interested in receiving additional genetics education and preferred education in-person and in consultation with both parents and a physician. The adolescents who felt most competent to have discussions with their doctors regarding potential causes of their heart defect previously had a school science course which covered topics in genetics. These results provide insight into adolescents’ perceptions and understanding about their CHD and genetic risk and may inform the creation and provision of additional genetic education.
Ivabradine in children with postural orthostatic tachycardia syndrome: a retrospective study
- Arooge Towheed, Zeid Nesheiwat, Muhammad A Mangi, Beverly Karabin, Blair P Grubb
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- Published online by Cambridge University Press:
- 05 June 2020, pp. 975-979
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Background:
Ivabradine is a unique medication that reduces the intrinsic heart rate by specifically blocking the inward funny current that controls the pacemaker activity of the sinus node. We conducted a retrospective cohort study to assess the efficacy of ivabradine in children suffering from postural orthostatic tachycardia syndrome.
Methods:A chart review was conducted of patients less than 18 years of age who were diagnosed with postural orthostatic tachycardia syndrome who had received ivabradine as treatment from January 2015 to February 2019 at our institution. Twenty-seven patients (25 females, 92.5%) were identified for the study. The outcomes which were assessed included a change in the severity and frequency of symptoms, heart rate, and blood pressure before and after starting ivabradine.
Results:There was an improvement in the symptoms of 18 (67%) out of 27 patients. The most notable symptom affected was syncope/presyncope with a reduction in 90%, followed by lightheadedness (85%) and fatigue (81%). The vital signs of the patients showed an overall significant lowering of the heart rate during sitting (89.7 ± 17.9 versus 73.2 ± 12.1; p-value <0.05) and standing (100.5 ± 18.1 versus 80.9 ± 10.1; p-value <0.05) without a significant change in the blood pressure. Two patients had visual disturbances (luminous phenomena). Severe bradycardia and excessive flushing were seen in two patients, respectively. Another one patient reported joint pain and fatigue.
Conclusion:This study indicates that 67% of children treated with ivabradine report an improvement in symptoms.
The effect of milrinone on hemodynamic and gas exchange parameters in children
- Rohit S. Loomba, Vincent Dorsey, Enrique G. Villarreal, Saul Flores
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- 17 December 2019, pp. 55-61
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Milrinone is a drug frequently used for hemodynamic support in children during critical illness. Although the hemodynamic changes induced by milrinone in children may appear similar to those of adults, the physiologic contributors of these changes remain vastly unknown. A systematic review was conducted to identify studies characterising the hemodynamic effects of milrinone in children during critical illness for hemodynamic support for various medical conditions. Studies were assessed for quality and those of satisfactory quality with pre- and post-operative hemodynamics for each patient were included in the final analyses. Those not limited to children and those not limited to patients with critical illness were excluded from the final analyses. A total of six studies with 791 patients were included in the final analyses. Milrinone infusion doses ranged from 0.3 to 0.75 mcg/kg/minute with the mean infusion dose being 0.5 mcg/kg/minute. Patients whom received milrinone infusion had greater cardiac output, greater left ventricle shortening fraction, lower right ventricular systolic pressure, and lower serum lactate levels. Systolic blood pressure mean arterial blood pressure and arterial oxygen concentration did not significantly change with administration of milrinone. These results were irrespective of milrinone infusion dose, infusion duration, and study size. Milrinone was found to have several beneficial hemodynamic effects in children during critical illness when used at usual clinical doses.
Clinical significance of family history and bicuspid aortic valve in children and young adult patients with Marfan syndrome
- Emanuele Monda, Adelaide Fusco, Daniela Melis, Martina Caiazza, Felice Gragnano, Alfredo Mauriello, Annapaola Cirillo, Marta Rubino, Augusto Esposito, Angelina Grammegna, Stefano Nistri, Guglielmina Pepe, Paolo Calabrò, Pietro Strisciuglio, Alessandro Della Corte, Guido Oppido, Mariagiovanna Russo, Giuseppe Limongelli
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- Published online by Cambridge University Press:
- 15 April 2020, pp. 663-667
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Background:
Marfan syndrome is an autosomal dominant disorder of the connective tissue, whose cardinal features affect eyes, musculoskeletal, and cardiovascular system. Despite prevalence and natural history of cardiovascular manifestation are well known in adults, little is known about children and young adult patients. The aim of this study was to describe a well-characterised cohort of consecutive children and young patients with marfan syndrome, looking at the impact of family history and presence of bicuspid aortic valve on disease severity.
Methods:A total of 30 consecutive children and young patients with Marfan syndrome were evaluated. All patients underwent a comprehensive clinical–instrumental–genetic evaluation. Particular attention was posed to identify differences in prevalence of cardiovascular abnormalities between patients with and without family history of Marfan syndrome or bicuspid aortic valve.
Results:Of these 30 patients, family history of Marfan syndrome and bicuspid aortic valve were present in 76 and 13%, respectively. Compared to patients with family history of Marfan syndrome, those without showed higher prevalence of aortic sinus dilation (87 versus 32%, p-value = 0.009), greater aortic sinus diameters (4.2 ± 2.1 versus 1.9 ± 1.1 z score, p-value = 0.002), and higher rate of aortic surgery during follow-up (37 versus 0%, p-value = 0.002). Compared to patients with tricuspid aortic valve, those with bicuspid aortic valve were younger (3.2 ± 4.3 versus 10.7 ± 6.8 years old, p-value = 0.043), showed greater aortic sinus diameters (4.2 ± 0.9 versus 2.2 ± 1.6 z score, p-value = 0.033), and underwent more frequently aortic root replacement (50 versus 4%, p-value = 0.004).
Conclusions:In our cohort of patients with Marfan syndrome, the absence of family history and the presence of bicuspid aortic valve were associated to severe aortic phenotype and worse prognosis.
A Pilot Study using the Compensatory Reserve Index to evaluate individuals with Postural Orthostatic Tachycardia syndrome
- Niti Shahi, Gabrielle Shirek, Kaci Pickett, Alexandra Schwartz, Jamie Shoop, Ryan Phillips, Rachel Workman, David Kaplan, Clio Pitula, Steven Moulton
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- Published online by Cambridge University Press:
- 30 September 2020, pp. 1833-1839
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Purpose:
The diagnosis of Postural Orthostatic Tachycardia syndrome traditionally involves orthostatic vitals evaluation. The Compensatory Reserve Index is a non-invasive, FDA-cleared algorithm that analyses photoplethysmogram waveforms in real time to trend subtle waveform features associated with varying degrees of central volume loss, from normovolemia to decompensation. We hypothesised that patients who met physiologic criteria for Postural Orthostatic Tachycardia syndrome would have greater changes in Compensatory Reserve Index with orthostatic vitals.
Methods:Orthostatic vitals and Compensatory Reserve Index values were assessed in individuals previously diagnosed with Postural Orthostatic Tachycardia syndrome and healthy controls aged 12–21 years. Adolescents were grouped for comparison based on whether they met heart rate criteria for Postural Orthostatic Tachycardia syndrome (physiologic Postural Orthostatic Tachycardia syndrome).
Results:Sixty-one patients were included. Eighteen percent of patients with an existing Postural Orthostatic Tachycardia syndrome diagnosis met heart rate criteria, and these patients had significantly greater supine to standing change in Compensatory Reserve Index (0.67 vs. 0.51; p<0.001). The optimal change in Compensatory Reserve Index for physiologic Postural Orthostatic Tachycardia syndrome was 0.60. Patients with physiologic Postural Orthostatic Tachycardia syndrome were more likely to report previous diagnoses of anxiety or depression (p = 0.054, 0.042).
Conclusion:An accurate diagnosis of Postural Orthostatic Tachycardia syndrome may be confounded by related comorbidities. Only 18% (8/44) of previously diagnosed Postural Orthostatic Tachycardia syndrome patients met heart rate criteria. Findings support the utility of objective physiologic measures, such as the Compensatory Reserve Index, to more accurately identify patients with true autonomic dysfunction.
Immediate outcome following valve surgery for rheumatic heart disease: the first local experience from Ethiopia
- Fekede A. Debel, Belete Zekarias, Tomasa Centella, Atnafu M. Tekleab
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- Published online by Cambridge University Press:
- 17 July 2020, pp. 1281-1287
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Background:
Rheumatic heart disease is the most common cardiac diseases in developing countries including Ethiopia. The current study aimed to describe the immediate surgical outcome following valve surgery for rheumatic heart disease in Ethiopia.
Methods:Data were collected through chart abstraction from two centres in Addis Ababa, Ethiopia: the Cardiac Center of Ethiopia and El Ouzier cardiac centre. Included were all patients who were operated for rheumatic valvular heart disease in the mentioned centres by local cardiac surgical team during the period from June 2017 to April 2020. Demographic and clinical characteristics of the study population at admission and within 30 days of the index cardiac surgery were collected. Statistical Package for Social Sciences version 20.0 for windows was used to analyse the data.
Result:Of the 114 patients included in the study (median age 31 years with interquartile range of 23–40), 62 (54.4%) of them were female. Surgical procedures done were triple valve surgery 9 (7.9%) patients, mitral and tricuspid valves 26 (22.8%) patients, double-valve 16 (14.0%) patients, single-valve surgery 50.9% (11 aortic and 47 mitral valves) of patients, redo mitral valve surgery 3 (2.6%) patients, and left maze with mitral valve surgery 2 (1.8%) patients. Of the total, 103 (90.4%) of them had mitral valve surgery. Post-operatively, 5 (4.4%) patients died within 30 days following the index surgery.
Conclusion:Immediate surgical outcome following valve surgery for rheumatic heart disease had excellent outcome in our setting. This evidence can be taken as a show of success in building local capacity to manage rheumatic heart disease surgically.
Health literacy and caregiver understanding in the CHD population
- Megan E. Rodts, Ndidi I. Unaka, Christopher J. Statile, Nicolas L. Madsen
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- Published online by Cambridge University Press:
- 04 August 2020, pp. 1439-1444
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Background:
CHD is the most common birth defect type, with one-fourth of patients requiring intervention in the first year of life. Caregiver understanding of CHD may vary. Health literacy may be one factor contributing to this variability.
Methods:The study occurred at a large, free-standing children’s hospital. Recruitment occurred at a free-of-charge CHD camp and during outpatient cardiology follow-up visits. The study team revised the CHD Guided Questions Tool from an eighth- to a sixth-grade reading level. Caregivers of children with CHD completed the “Newest Vital Sign” health literacy screen and demographic surveys. Health literacy was categorised as “high” (Newest Vital Sign score 4–6) or “low” (score 0–3). Caregivers were randomised to read either the original or revised Guided Questions Tool and completed a validated survey measuring understandability and actionability of the Guided Questions Tool. Understandability and actionability data analysis used two-sample t-testing, and within demographic group differences in these parameters were assessed via one-way analysis of variance.
Results:Eighty-two caregivers participated who were largely well educated with a high income. The majority (79.3%) of participants scored “high” for health literacy. No differences in understanding (p = 0.43) or actionability (p = 0.11) of the original and revised Guided Questions Tool were noted. There were no socio-economic-based differences in understandability or actionability (p > 0.05). There was a trend towards improved understanding of the revised tool (p = 0.06).
Conclusions:This study demonstrated that readability of the Guided Questions Tool could be improved. Future work is needed to expand the study population and further understand health literacy’s impact on the CHD community.
Post-operative acute kidney injury is associated with a biomarker of acute brain injury after paediatric cardiac surgery
- Michael Parsons, Jason Greenberg, Chirag Parikh, Jeremiah Brown, Devin Parker, Jie Zhu, Luca Vricella, Allen D. Everett
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- Published online by Cambridge University Press:
- 30 March 2020, pp. 505-510
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Introduction:
Children with CHD who undergo cardiopulmonary bypass are at an increased risk of acute kidney injury. This study evaluated the association of end-organ specific injury plasma biomarkers for brain: glial fibrillary acidic protein and heart: Galectin 3, soluble suppression of tumorgenicity 2, and N-terminal pro b-type natriuretic peptide with acute kidney injury in children undergoing cardiopulmonary bypass.
Materials and Methods:We enrolled consecutive children undergoing cardiac surgery with cardiopulmonary bypass. Blood samples were collected pre-bypass in the operating room and in the immediate post-operative period. Acute kidney injury was defined as a rise of serum creatinine ≥50% from pre-operative baseline within 7 days after surgery.
Results:Overall, 162 children (mean age 4.05 years, sd 5.28 years) were enrolled. Post-operative acute kidney injury developed in 55 (34%) children. Post-operative plasma glial fibrillary acidic protein levels were significantly higher in patients with acute kidney injury (median 0.154 (inter-quartile range 0.059–0.31) ng/ml) compared to those without acute kidney injury (median 0.056 (inter-quartile range 0.001–0.125) ng/ml) (p = 0.043). After adjustment for age, weight, and The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery category, each natural log increase in post-operative glial fibrillary acidic protein was significantly associated with a higher risk for subsequent acute kidney injury (adjusted odds ratio glial fibrillary acidic protein 1.25; 95% confidence interval 1.01–1.59). Pre/post-operative levels of galectin 3, soluble suppression of tumorgenicity 2, and N-terminal pro b-type natriuretic peptide did not significantly differ between patients with and without acute kidney injury.
Conclusions:Higher plasma glial fibrillary acidic protein levels measured in the immediate post-operative period were independently associated with subsequent acute kidney injury in children after cardiopulmonary bypass. Elevated glial fibrillary acidic protein likely reflects intraoperative brain injury which may occur in the context of acute kidney injury-associated end-organ dysfunction.
Neurodevelopmental evaluation for school-age children with congenital heart disease: recommendations from the cardiac neurodevelopmental outcome collaborative
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- Dawn Ilardi, Jacqueline H. Sanz, Adam R. Cassidy, Renee Sananes, Caitlin K. Rollins, Catherine Ullman Shade, Gretchen Carroll, David C. Bellinger
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- Published online by Cambridge University Press:
- 04 November 2020, pp. 1623-1636
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In 2012, the American Heart Association and the American Academy of Paediatrics released a scientific statement with guidelines for the evaluation and management of the neurodevelopmental needs of children with CHD. Decades of outcome research now highlight a range of cognitive, learning, motor, and psychosocial vulnerabilities affecting individuals with CHD across the lifespan. The number of institutions with Cardiac Neurodevelopmental Follow-Up Programmes and services for CHD is growing worldwide. This manuscript provides an expanded set of neurodevelopmental evaluation strategies and considerations for professionals working with school-age children with CHD. Recommendations begin with the referral process and access to the evaluation, the importance of considering medical risk factors (e.g., genetic disorders, neuroimaging), and the initial clinical interview with the family. The neurodevelopmental evaluation should take into account both family and patient factors, including the child/family’s primary language, country of origin, and other cultural factors, as well as critical stages in development that place the child at higher risk. Domains of assessment are reviewed with emphasis on target areas in need of evaluation based on current outcome research with CHD. Finally, current recommendations are made for assessment batteries using a brief core battery and an extended comprehensive clinical battery. Consistent use of a recommended assessment battery will increase opportunities for research collaborations, and ultimately help improve the quality of care for families and children with CHD.