Original Article
Trends in prescription of cardiovascular drugs to children in relation to prevalence of CHD from 1999 to 2016
- Christian B. Poulsen, Mads Damkjær
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- Published online by Cambridge University Press:
- 11 July 2018, pp. 1136-1141
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Introduction
Owing to massive improvements in the diagnostics and surgery of children with CHD, fatality has decreased substantially. As more children with CHD survive from infancy into later childhood, more will need medication for chronic heart failure. However, surprisingly little is actually known about which drugs are being used to treat children with CHD, and whether prescription rates and CHD prevalence have changed over time.
ObjectiveThe objective of this study was to assess the total prescription of cardiovascular drugs to children during an 18-year period and to assess concomitant CHD prevalence.
MethodsAll prescription data of cardiovascular drugs to children aged 0–19 years were extracted from publicly available databases in Norway and Denmark from 1999 to 2016. This was coupled with data on CHD prevalence and birth rates.
ResultsThe number of defined daily doses of cardiovascular drugs prescribed to children doubled in the study period. This was because of an increased use of beta blockers, angiotensin-converting-enzyme inhibitors/angiotensin receptor blockers, and anti-arrhythmic agents. The use of some classes of drugs was significantly reduced over time. The prevalence of CHD remained constant in both countries – 80 per 10,000 births.
ConclusionWe show that there is an increase in the overall prescription of cardiovascular drugs to children. Beta blockers, angiotensin-converting enzyme/angiotensin receptor blockers, and anti-arrhythmics account for the largest increase. Birth rates decreased or remained constant together with CHD prevalence, suggesting that the increased use of cardiovascular drugs reflected increased prescription per patient, rather than more patients receiving a constant amount of drugs.
Original Articles
Epinephrine syringe exchange events in a paediatric cardiovascular ICU: analysing the storm
- Barbara-Jo Achuff, Jameson C. Achuff, Hwan H. Park, Brady Moffett, Sebastian Acosta, Craig G. Rusin, Paul A. Checchia
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- Published online by Cambridge University Press:
- 04 December 2017, pp. 409-415
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Introduction
Haemodynamically unstable patients can experience potentially hazardous changes in vital signs related to the exchange of depleted syringes of epinephrine to full syringes. The purpose was to determine the measured effects of epinephrine syringe exchanges on the magnitude, duration, and frequency of haemodynamic disturbances in the hour after an exchange event (study) relative to the hours before (control).
Materials and methodsBeat-to-beat vital signs recorded every 2 seconds from bedside monitors for patients admitted to the paediatric cardiovascular ICU of Texas Children’s Hospital were collected between 1 January, 2013 and 30 June, 2015. Epinephrine syringe exchanges without dose/flow change were obtained from electronic records. Time, magnitude, and duration of changes in systolic blood pressure and heart rate were characterised using Matlab. Significant haemodynamic events were identified and compared with control data.
ResultsIn all, 1042 syringe exchange events were found and 850 (81.6%) had uncorrupted data for analysis. A total of 744 (87.5%) exchanges had at least 1 associated haemodynamic perturbation including 2958 systolic blood pressure and 1747 heart-rate changes. Heart-rate perturbations occurred 37% before exchange and 63% after exchange, and 37% of systolic blood pressure perturbations happened before syringe exchange, whereas 63% occurred after syringe exchange with significant differences found in systolic blood pressure frequency (p<0.001), duration (p<0.001), and amplitude (p<0.001) compared with control data.
ConclusionsThis novel data collection and signal processing analysis showed a significant increase in frequency, duration, and magnitude of systolic blood pressure perturbations surrounding epinephrine syringe exchange events.
Original Article
Examining variation in interstage mortality rates across the National Pediatric Cardiology Quality Improvement Collaborative: do lower-mortality centres have lower-risk patients?
- Katherine E. Bates, Sunkyung Yu, Ray Lowery, Sara K. Pasquali, David W. Brown, Peter B. Manning, Karen Uzark
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- Published online by Cambridge University Press:
- 21 June 2018, pp. 1031-1036
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Background
Although interstage mortality for infants with hypoplastic left heart syndrome has declined within the National Pediatric Cardiology Quality Improvement Collaborative, variation across centres persists. It remains unclear whether centres with lower interstage mortality have lower-risk patients or whether differences in care may explain this variation. We examined previously established risk factors across National Pediatric Cardiology Quality Improvement Collaborative centres with lower and higher interstage mortality rates.
MethodsLower-mortality centres were defined as those with >25 consecutive interstage survivors. Higher-mortality centres were defined as those with cumulative interstage mortality rates >10%, which is a collaborative historic baseline rate. Baseline risk factors and perioperative characteristics were compared.
ResultsSeven lower-mortality centres were identified (n=331 patients) and had an interstage mortality rate of 2.7%, as compared with 13.3% in the four higher-mortality centres (n=173 patients, p<0.0001). Of all baseline risk factors examined, the only factor that differed between the lower- and higher-mortality centres was postnatal diagnosis (18.4 versus 31.8%, p=0.001). In multivariable analysis, there remained a significant mortality difference between the two groups of centres after adjusting for this variable: adjusted mortality rate was 2.8% in lower-mortality centres compared with 12.6% in higher-mortality centres, p=0.003. Secondary analyses identified multiple differences between groups in perioperative practices and other variables.
ConclusionsVariation in interstage mortality rates between these two groups of centres does not appear to be explained by differences in baseline risk factors. Further study is necessary to evaluate variation in care practices to identify targets for improvement efforts.
Original Articles
Perinatal and early postnatal outcomes for fetuses with prenatally diagnosed d-transposition of the great arteries: a prospective cohort study assessing the effect of standardised prenatal consultation
- Yanji Qu, Shusheng Wen, Xiaoqing Liu, Wei Pan, Fengzhen Han, Jinzhuang Mai, Yanqiu Ou, Zhiqiang Nie, Xiangmin Gao, Yong Wu, Richard G. Ohye, Jimei Chen, Jian Zhuang
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- Published online by Cambridge University Press:
- 08 August 2017, pp. 66-75
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Background
The aim of this study was to explore perinatal and early postnatal outcomes in fetuses with prenatally diagnosed d-transposition of the great arteries and impacts of standardised prenatal consultation.
MethodsAll fetuses with prenatally diagnosed d-transposition of the great arteries prospectively enrolled at South China cardiac centre from 2011 to 2015. Standardised prenatal consultation was introduced in 2013 and comprehensive measures were implemented, such as establishing fetal CHD Outpatient Consultation Service, performing standard prenatal consultation according to specifications, and establishing a multidisciplinary team with senior specialists performing in-person consultations. Continuous follow-up investigation was conducted. Perinatal and postnatal outcomes were compared before and after consultation including live birth, elective termination of pregnancy, spontaneous fetal death, stillbirths, referral for surgery, and survival.
ResultsIn all, 146 fetuses were enrolled with 41 (28%) lost to follow-up. Among 105 remaining fetuses, 29 (28%) were live births and 76 (72%) were terminated. After consultation, live birth rate was higher (50 versus 33%) and termination rate was lower (50 versus 76%), although there was no statistical significance. Excluding three live births without postnatal d-transposition of the great arteries, 65% (17/26) underwent arterial switch operation within 30 days. A total of three in-hospital deaths occurred and during the 10-month follow-up period, one death was observed. In one case, the switch procedure was performed at 13 months and the infant survived. Out of eight infants without arterial switch operation, two died.
ConclusionsLive birth rate increased after consultation; however, termination remained high. Combining termination, patients without arterial switch operation, and operative mortality, outcomes of d-transposition of the great arteries infants can be improved. Standard consultation, multidisciplinary collaboration, and improved perinatal care are important to improve outcomes.
Retrieval of large Occlutech Figula Flex septal defect occluders using a commercially available bioptome: proof of concept
- Stanimir Georgiev, Daniel Tanase, Thomas Genz, Peter Ewert, Susanne Naumann, Robert Dalla Pozza, Andreas Eicken
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- Published online by Cambridge University Press:
- 21 May 2018, pp. 955-960
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Objectives
This study aimed to develop a method for retrieval of the new meshed nitinol atrial septal defect occluders – Ceraflex and Occlutech.
BackgroundThe newly developed atrial septal defect occluders have potential benefits considering implantation, but concerns regarding their removal in case of embolisation have been raised.
MethodsOver 21 years, 1449 patients underwent interventional atrial septal defect occlusion in our institution. We reviewed the cases of embolisation of the device, developed a strategy for device removal, and tested it on the benchside and in animal tests.
ResultsIn 11 patients (0.8%), the intended atrial septal defect occlusion was complicated by an embolisation of the device. In contrast to the Amplatzer septal occluders, retrieval of Occlutech devices larger than 16 mm with snare techniques was impossible. In benchside tests, this was confirmed and a new method for removal of large meshed devices was developed. This involved the commercially available Maslanka® biopsy forceps. The feasibility of this technique in vivo was tested in a pig model. During animal tests, using the Maslanka biopsy forceps it was possible to interventionally retrieve embolised Ceraflex and Occlutech devices of different sizes – 10, 16, 30, and 40 mm – into a 12-F sheath.
ConclusionIt was impossible to retrieve Occlutech and Ceraflex devices larger than 16 mm into a large sheath in vivo and during benchside tests. However, this was feasible on the bench and in vivo using the Maslanka biopsy forceps even with the largest available devices.
Relationship between mean platelet volume-to-lymphocyte ratio and coronary artery abnormalities in Kawasaki disease
- Gulcin Bozlu, Derya Karpuz, Olgu Hallioglu, Selma Unal, Necdet Kuyucu
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- Published online by Cambridge University Press:
- 16 April 2018, pp. 832-836
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Objectives
Recently, mean platelet volume-to-lymphocyte ratio has emerged as a novel parameter of inflammation. No study has investigated the role of mean platelet volume-to-lymphocyte ratio in children with Kawasaki disease. We aimed to evaluate the relationship between mean platelet volume-to-lymphocyte ratio and coronary artery abnormalities in Kawasaki disease.
MethodsBetween January 2008 and January 2017, a total of 58 children with Kawasaki disease and 42 healthy subjects matched for sex and age were enrolled. Before the treatment, transthoracic echocardiography for all children was performed. Clinical and laboratory results including mean platelet volume, platelet distribution width, red blood cell distribution width, and counts of platelets, neutrophils, lymphocytes, and white blood cells, erythrocyte sedimentation rate, and C-reactive protein levels were measured. Mean platelet volume-to-lymphocyte ratio was calculated as mean platelet volume divided by lymphocyte count.
ResultsCompared with healthy controls, mean platelet volume-to-lymphocyte ratio was significantly lower in the children with Kawasaki disease (p<0.01). A total of 14 patients (24.1%) had incomplete Kawasaki disease and 15 (25.8%) children with Kawasaki disease had coronary involvement. Mean platelet volume-to-lymphocyte ratio was significantly lower in patients with coronary artery abnormalities (p<0.01). According to receiver operating characteristic curve analysis performed for the prediction of coronary artery abnormalities, the best cut-off point for mean platelet volume-to-lymphocyte ratio was 2.5 (area under curve=0.593, sensitivity 53.3%, specificity 51.1%).
ConclusionIt was first shown that the children with Kawasaki disease have lower mean platelet volume-to-lymphocyte ratio compared with control subjects. Mean platelet volume-to-lymphocyte ratio may be helpful in predicting coronary artery lesions in patients with Kawasaki disease.
A randomised trial of early palliative care for maternal stress in infants prenatally diagnosed with single-ventricle heart disease
- Hayley S. Hancock, Ken Pituch, Karen Uzark, Priya Bhat, Carly Fifer, Maria Silveira, Sunkyung Yu, Suzanne Welch, Janet Donohue, Ray Lowery, Ranjit Aiyagari
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- Published online by Cambridge University Press:
- 10 January 2018, pp. 561-570
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Children with single-ventricle disease experience high mortality and complex care. In other life-limiting childhood illnesses, paediatric palliative care may mitigate maternal stress. We hypothesised that early palliative care in the single-ventricle population may have the same benefit for mothers. In this pilot randomised trial of early palliative care, mothers of infants with prenatal single-ventricle diagnoses completed surveys measuring depression, anxiety, coping, and quality of life at a prenatal visit and neonatal discharge. Infants were randomised to receive early palliative care – structured evaluation, psychosocial/spiritual, and communication support before surgery – or standard care. Among 56 eligible mothers, 40 enrolled and completed baseline surveys; 38 neonates were randomised, 18 early palliative care and 20 standard care; and 34 postnatal surveys were completed. Baseline Beck Depression Inventory-II and State-Trait Anxiety Index scores exceeded normal pregnant sample scores (mean 13.76±8.46 versus 7.0±5.0 and 46.34±12.59 versus 29.8±6.35, respectively; p=0.0001); there were no significant differences between study groups. The early palliative care group had a decrease in prenatal to postnatal State-Trait Anxiety Index scores (−7.6 versus 0.3 in standard care, p=0.02), higher postnatal Brief Cope Inventory positive reframing scores (p=0.03), and a positive change in PedsQL Family Impact Module communication and family relationships scores (effect size 0.46 and 0.41, respectively). In conclusion, these data show that mothers of infants with single-ventricle disease experience significant depression and anxiety prenatally. Early palliative care resulted in decreased maternal anxiety, improved maternal positive reframing, and improved communication and family relationships.
Original Article
Association of left atrial pressure with left atrial volume and N-terminal prohormone brain natriuretic peptide in children with cardiomyopathy
- Arpit Agarwal, Suying Lam, Hua Li, Sudheer R. Gorla, Nao Sasaki, Paolo G. Rusconi, Sethuraman Swaminathan
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- Published online by Cambridge University Press:
- 31 July 2018, pp. 1333-1337
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Background
Enlargement of the left atrium is a non-invasive marker of diastolic dysfunction of the left ventricle, a determinant of prognosis in children with cardiomyopathy. Similarly, N-terminal prohormone brain natriuretic peptide is a useful marker in the management of children with cardiomyopathy and heart failure. The aim of this study is to evaluate the association of left atrial pressures with left atrial volume and N-terminal prohormone brain natriuretic peptide in children with cardiomyopathy.
MethodsThis was a retrospective study reviewing the medical records of patients <18 years of age, who were diagnosed with cardiomyopathy or acute myocarditis with eventual development of cardiomyopathy. Left atrial volume by transthoracic echocardiogram and pulmonary capillary wedge pressure, a surrogate of left atrial pressure, obtained by means of cardiac catheterisation were analysed. In addition, N-terminal prohormone brain natriuretic peptide levels obtained at the time of the cardiac catheterisation were also reviewed. Statistical analysis was performed to evaluate the association of left atrial pressures with left atrial volume and N-terminal prohormone brain natriuretic peptide levels.
ResultsThere was a linear correlation of left atrial pressure estimated in the cardiac catheterisation with indexed left atrial volume (r=0.63; p<0.001) and left atrial volume z-scores (r=0.59; p<0.001). We found no statistically significant association between the left atrial pressure and N-terminal prohormone brain natriuretic peptide levels.
ConclusionsLeft atrial volume measured non-invasively by echocardiography can be used as a surrogate for left atrial pressure in assessing diastolic dysfunction of the left ventricle in children with cardiomyopathy. The larger the size of the left atrium, worse is the diastolic function of the left ventricle.
Original Articles
Histopathological evaluation of aortic coarctation after conventional balloon angioplasty in neonates
- Atakan Atalay, Aysenur Pac, Tugba Avci, Neslihan İnci Zengin, Nadide Demir Amac, Deniz Eris, Irfan Tasoglu, Mustafa Pac
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- Published online by Cambridge University Press:
- 18 January 2018, pp. 683-687
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Background
Optimal management strategy for native aortic coarctation in neonates and young infants is still a matter of debate. The surgical procedure, histopathologic research, and clinical outcome in 15 neonates who underwent surgery after successful balloon angioplasty is the basis of this study.
MethodBetween 01 October, 2014 and 01 August, 2017, we enrolled 15 patients with native aortic coarctation for this study. These patients had complications regarding recoarctation, following balloon angioplasty intervention at our institute and other centres. Surgically extracted parts were examined histopathologically and patient’s data were collected retrospectively.
ResultThe reasons for recurrence of recoarctation after balloon angioplasty are as follows: patients with higher preoperative echocardiographic gradients had recoarctation earlier, neointimal proliferation, aortic intimal fibrosis at the region of ductal insertion, and ductal residual tissue debris after balloon angioplasty. No repeat intervention was required in the 15 patients who underwent surgery followed by balloon angioplasty. Early mortality was seen in one patient after surgery. Postoperative complication in the surgical group occurred in the form of chylothorax in one patient.
ConclusionIn centres in which the neonatal ICU is inexperienced, balloon angioplasty is particularly recommended. In developing neonatal clinics, balloon angioplasty, when performed on patients at their earliest possible age, delays actual corrective operation to a later date, which in turn provides less risky surgical outcomes in infants who are gaining weight, growing, and do not have any haemodynamic complaints.
Original Article
Single-centre experience in stenting arterial shunts for adult CHD patients with single-ventricle physiology and pulmonary blood flow dependent on arterial shunts
- Mohamed Kasem, James Bentham, John Thomson
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- 12 October 2018, pp. 1431-1435
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Objectives
The objectives of this study were to describe the approach to stenting arterial shunts in adult congenital patients with single-ventricle physiology and to assess the medium-term clinical and haemodynamic outcomes following stent insertion.
BackgroundAdult patients with single-ventricle physiology and pulmonary blood flow dependent on a surgically placed arterial shunt who did not progress to venous palliation are extremely challenging to manage. Progressive cyanosis secondary to narrowing of the shunt has a marked impact on exercise tolerance and results in intolerable well-being for these patients. Stenting arterial shunts in adult patients is one method that can help improve pulmonary blood flow. There is very limited information in the literature about this patient.
MethodsThis is a retrospective study for arterial shunts stenting conducted between 2008 and 2016. The peripheral oxygen saturations, the NYHA status, the haemoglobin, and the degree of atrio-ventricular valve regurgitation as assessed on transthoracic echo were compared before and 6–12 months after procedures.
ResultsThere was a short-term improvement in oxygen saturations; the pre-procedure mean was 75.8 (SD 2.55)% (range 70–85%) and post-procedure mean was 83 (SD 2.52)% (range 78–87%), with a p value of 0.04. Haemoglobin level decreased from a pre-procedure mean of 22.06–20.28 g/L 6 months post procedure (range 18.1–24.4 to 13–23.3 g/L), with a p value of 0.44. NYHA class decreased from a mean of 3.2–2.2 post procedure. Left atrial volume for four of the cases did not change (22.6–76.6 ml [mean 48.4 ml] to 29.6–72.9 ml [mean 52 ml], p value: 0.83).
ConclusionsStenting stenotic arterial shunts is a useful method to gain a medium-term improvement on the oxygen saturation and clinical symptoms, and may act as a useful intermediate step for further management plans.
Clinical impact of a novel ambulatory rhythm monitor in children
- Joseph W. May, Elizabeth L. Carter, J. Ryan Hitt, Thomas R. Burklow
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- Published online by Cambridge University Press:
- 10 July 2018, pp. 1134-1140
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Traditional ambulatory rhythm monitoring in children can have limitations, including cumbersome leads and limited monitoring duration. The ZioTM patch ambulatory monitor is a small, adhesive, single-channel rhythm monitor that can be worn up to 2 weeks. In this study, we present a retrospective cross-sectional analysis of the ZioTM monitor’s impact in clinical practice. Patients aged 0–18 years were included in the study. A total of 373 studies were reviewed in 332 patients. In all, 28.4% had structural heart disease, and 16.9% had a prior surgical, catheterisation, or electrophysiology procedure. The most common indication for monitoring was tachypalpitations (41%); 93.5% of these patients had their symptoms captured during the study window. The median duration of monitoring was 5 days. Overall, 5.1% of ZioTM monitoring identified arrhythmias requiring new intervention or increased medical management; 4.0% identified arrhythmias requiring increased clinical surveillance. The remainder had either normal-variant rhythm or minor rhythm findings requiring no change in management. For patients with tachypalpitations and no structural heart disease, 13.2% had pathological arrhythmias, but 72.9% had normal-variant rhythm during symptoms, allowing discharge from cardiology care. Notably, for patients with findings requiring intervention or increased surveillance, 56% had findings first identified beyond 24 hours, and only 62% were patient-triggered findings. Seven studies (1.9%) were associated with complications or patient intolerance. The ZioTM is a well-tolerated device that may improve what traditional Holter and event monitoring would detect in paediatric cardiology patients. This study shows a positive clinical impact on the management of patients within a paediatric cardiology practice.
Impact of age at bidirectional cavopulmonary anastomosis on haemodynamics after Fontan operation
- Shuichi Shiraishi, Toshihide Nakano, Shinichiro Oda, Hideaki Kado
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- Published online by Cambridge University Press:
- 10 September 2018, pp. 1436-1443
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Backgrounds
The aim of this study was to assess the impact of age at bidirectional cavopulmonary anastomosis on haemodynamics after total cavopulmonary connection.
MethodsWe conducted a retrospective analysis of 100 consecutive patients who underwent total cavopulmonary connection from 2010 to 2014. All patients had previously undergone bidirectional cavopulmonary anastomosis. These patients were classified into two groups according to age at bidirectional cavopulmonary anastomosis: younger group, <6 months (n=33), and older group, >6 months (n=67).
ResultsThe proportion of hypoplastic left heart syndrome was higher in the younger group (48 versus 4%). After total cavopulmonary connection, the chest tube period was longer in the younger group (10.1±6.6 versus 6.7±4.5 days; p=0.009). Catheterisation 6 months after total cavopulmonary connection revealed that pulmonary artery pressure was higher (11.5±1.9 versus 10.4±2.1 mmHg; p=0.017) and Nakata index was lower (219±79 versus 256±70 mm2/m2; p=0.024) in the younger group. In patients with a non-hypoplastic left heart syndrome, there was no difference in post-operative haemodynamics between two groups, but the total amount of chest drainage after total cavopulmonary connection was larger in the younger group (109±95 versus 55±40 ml/kg; p=0.044).
ConclusionsEarly bidirectional cavopulmonary anastomosis did not affect the outcome of total cavopulmonary connection. Longer chest tube period, smaller pulmonary artery, and higher pulmonary artery pressure after total cavopulmonary connection were recognised in early bidirectional cavopulmonary anastomosis patients, especially in hypoplastic left heart syndrome.
Parental stress and resilience in CHD: a new frontier for health disparities research
- Amy J. Lisanti
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- Published online by Cambridge University Press:
- 11 July 2018, pp. 1142-1150
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Parental stress is a universal experience for parents who have children diagnosed with CHD and has been studied within the context of the child’s illness, but not through a broader health disparity lens. This paper provides a thorough synthesis of the current literature on parental stress addressing disparities in parents of children with CHD. Several theories and models from within this literature are described and a new comprehensive framework, the Parental Stress and Resilience in CHD Model, is presented. Future research and clinical implications are discussed.
Original Articles
Dynamic flow imaging using 320-detector row CT and motion coherence analysis in coronary aneurysms associated with Kawasaki disease
- Yamato Shimomiya, Michinobu Nagao, Yuzo Yamasaki, Takashi Shirasaka, Masatoshi Kondo, Tsukasa Kojima, Akihiro Nishie, Kenichiro Yamamura, Hiroshi Honda
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- Published online by Cambridge University Press:
- 14 December 2017, pp. 416-420
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Introduction
We propose a new dynamic flow imaging using 320-detector row CT, and investigate the assessment of coronary flow in aneurysms of Kawasaki disease in adulthood.
MethodsSix patients with Kawasaki disease and coronary aneurysms associated (26.7 years old) and six controls were enrolled. Dynamic coronary CT angiography with 320-row CT was continuously performed at mid-diastole throughout 15–25 cardiac cycles with prospective Electrocardiogram gating after injection of contrast media. Dynamic data sets of 15–25 cycles were computed into 90–100 data sets by motion coherence image processing. Next, time–density curves for coronary arteries were calculated for all the phases. On the basis of the maximum slope method, coronary flow index was defined as the ratio of the maximum upslope of the attenuation of coronary arteries to the upslope of the attenuation of ascending aorta on the time–density curves. Coronary flow indexes for the proximal and distal sites of coronary arteries and intra-aneurysm were measured.
Chylothorax following paediatric cardiac surgery: a case–control study
- Thomas G. Day, Diana Zannino, Daniel Golshevsky, Yves d’Udekem, Christian Brizard, Michael M. H. Cheung
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- Published online by Cambridge University Press:
- 24 August 2017, pp. 222-228
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Objectives
The aims of this study were to investigate risk factors for the development of postoperative chylothorax following paediatric congenital heart surgery and to investigate the impact of a management guideline on management strategies and patient outcome.
MethodsAll patients with chylothorax following cardiac surgery at the Royal Children’s Hospital, Melbourne, over a 48-month period beginning in January 2008 were identified. A control group, matched for age, date of surgery, and sex, was identified. To investigate potential risk factors, univariable and multivariable logistic regression models were constructed with paired analysis. To examine the effect of a standardised management protocol, data before and after the implementation of the guideline were compared.
ResultsIn total, 121 cases of chylothorax were identified, with 121 controls, matched for age at surgery, date of surgery, and sex. The incidence of chylothorax was 5.23%. Increasing surgical complexity (univariable OR 0.17 for the least complex versus the most complex group, p=0.02), closed-heart surgeries (OR 0.07 for open versus closed, p<0.001), and redo chest incisions (OR 10.0 for redo versus virgin, p<0.001) were significantly associated with chylothorax. The standardised management protocol had no significant impact on either drainage duration or management strategy.
ConclusionsWe have replicated the previously reported association between surgical complexity and chylothorax risk, and have shown, for the first time, that redo chest openings are also associated with a significantly increased risk. The implementation of a standardised management protocol in our institution did not result in a significant change in either chylothorax drainage duration or management strategy.
Original Article
Hypotension within 1 week of life associated with poor short- and long-term outcomes in very low birth weight infants
- Tae-Hoon Kim, Cheong-Jun Moon, In K. Sung, Young A. Youn
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- Published online by Cambridge University Press:
- 02 July 2018, pp. 1037-1041
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We examined whether hypotension in very low birth weight infants aged⩽1 week was associated with hospital morbidities and overall mortality. Further, we studied whether hypotension was associated with poor neurodevelopmental outcomes in these patients at the corrected age of 18 months. A total of 166 very low birth weight infants were studied during this period. Hospital outcomes and neurodevelopmental outcomes at the corrected age of 18 months were evaluated. Among the 166 very low birth weight infants, 95 patients (57.2%) experienced hypotension at⩽1 week and were associated with an increased incidence of morbidities and mortality. At the corrected age of 18 months, hypotension of the⩽1 week group had significantly lower scores in all three – cognitive, language, and motor – composites of the Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III) screening tests. In addition, a multivariable logistic regression analysis showed that longer mechanical ventilation and periventricular leukomalacia were additionally associated with worse cognitive and language neurodevelopmental outcomes. Hypotension in very low birth weight infants within 1 week of life was associated with increased morbidities and overall mortality. It was also associated with an increased risk of cognitive and language outcomes.
Aortic elasticity and the influence of valve morphology in children with bicuspid aortic valve
- Elif Erolu, Figen Akalın, Nilüfer Çetiner, Berna Ç. Şaylan
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- Published online by Cambridge University Press:
- 06 August 2018, pp. 1338-1344
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Aim
We investigated dimensions and elasticity of whole aorta in patients with bicuspid aortic valve and influence of valve phenotype.
MethodThe study group included 44 patients and 42 controls. Patients were divided into groups according to the type of valve opening as horizontal – fusion between right and left coronary cusps – and vertical – fusion between right–non-coronary cusps; according to age they were divided into younger (5–10 years) and older patients (11–16 years). Our study did not include valve phenotype with fusion between left and non-coronary cusps. Systolic–diastolic diameters of aortic annulus, sinus valsalva, sinutubular junction, arcus, and ascending–descending and abdominal aorta were measured and z-scores were obtained. Aortic strain, distensibility, and stiffness index were calculated. Flow-mediated dilatation of brachial artery was studied.
Resultsz-Scores at annulus, sinus valsalva, sinutubular junction, and ascending aorta were higher in study patients (p=0.001, p=0.0001, p=0.0001, p=0.0001, respectively). z-Scores of sinus valsalva and sinotubular junction were higher in the horizontal group than in the vertical group (p=0.006, p=0.023, respectively). z-Score was over +2 in 51% of patients with horizontal morphology and 33% of patients with vertical morphology (p=0.0001). Ascending aorta was more distensible and less stiff in the study group (11.3±5.63 versus 7.91±4.5, p=0.002; 4.76±3.60 versus 6.19±3.44 cm2.dyn−1.10−6, p=0.033, respectively). Stiffness index of ascending, arcus, and abdominal aorta were higher in the horizontal group (p=0.004, p=0.038, p=0.006, respectively). Ascending aorta was more distensible and less stiff in the younger group (p=0.007, p=0.027, respectively) but did not differ in the older group compared with the control group.
ConclusionAortic dimensions are enlarged in patients with bicuspid aortic valve starting from childhood, suggesting the presence of generalised aortopathy. Aortic elasticity is increased at young age and decreased with age.
Original Articles
Whole-exome sequencing identifies a Novel SCN5A mutation (C335R) in a Chinese family with arrhythmia
- Hao Huang, Dong-Bo Ding, Liang-Liang Fan, Jie-Yuan Jin, Jing-Jing Li, Shuai Guo, Ya-qin Chen, Rong Xiang
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- Published online by Cambridge University Press:
- 06 February 2018, pp. 688-691
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Background
SCN5A encodes sodium-channel α-subunit Nav1.5. The mutations of SCN5A can lead to hereditary cardiac arrhythmias such as the long-QT syndrome type 3 and Brugada syndrome. Here we sought to identify novel mutations in a family with arrhythmia.
MethodsGenomic DNA was isolated from blood of the proband, who was diagnosed with atrial flutter. Illumina Hiseq 2000 whole-exome sequencing was performed and an arrhythmia-related gene-filtering strategy was used to analyse the pathogenic genes. Sanger sequencing was applied to verify the mutation co-segregated in the family.
Results and conclusionsA novel missense mutation in SCN5A (C335R) was identified, and this mutation co-segregated within the affected family members. This missense mutation was predicted to result in amplitude reduction in peak Na+ current, further leading to channel protein dysfunction. Our study expands the spectrum of SCN5A mutations and contributes to genetic counselling of families with arrhythmia.
Left ventricular diastolic dysfunction without left ventricular hypertrophy in obese children and adolescents: a Tissue Doppler Imaging and Cardiac Troponin I Study
- Sonia A. El Saiedi, Marwa F. Mira, Sahar A. Sharaf, Maysoun M. Al Musaddar, Rania M. H. El Kaffas, Antoine F. AbdelMassih, Ihab H. Y. Barsoum
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- Published online by Cambridge University Press:
- 07 August 2017, pp. 76-84
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Background
Obesity increases the risk for various cardiovascular problems. Increase in body mass index is often an independent risk factor for the development of elevated blood pressure and clustering of various cardiovascular risk factors.
ObjectiveTo determine early markers of left ventricular affection in obese patients before the appearance of left ventricular hypertrophy.
MethodsIn this cross-sectional study, we evaluated 42 obese patients and 30 healthy controls. Their ages ranged from 6 to 19 years. Studied children were subjected to anthropometric, lipid profile, and serum Troponin I level measurements. Echocardiographic evaluation performed to assess the left ventricle included left ventricular dimension measurement using motion-mode echocardiography, based on which patients with left ventricular hypertrophy (10 patients) were eliminated, as well as conventional and tissue Doppler imaging.
ResultsTissue Doppler findings in the study groups showed that the ratio of transmitral early diastolic filling velocity to septal peak early diastolic myocardial velocity (E/e′) was significantly higher in cases compared with controls [6.9±1.4 versus 9.0±1.6, p (Pearson’s coefficient)=0.001, respectively]. The level of cardiac troponin I was significantly higher in cases compared with controls [0.14±0.39 ng/ml versus 0.01±0.01 ng/ml, p (Pearson’s coefficient)=0.047, respectively] and there was a significant correlation between troponin I and transmitral early diastolic filling velocity to septal peak early diastolic myocardial velocity ratio (E/e′) [R (correlation coefficient)=0.6].
ConclusionTissue Doppler Imaging and Troponin I evaluation proved useful tools to detect early affection of the left ventricle in obese patients even in the absence of left ventricular hypertrophy.
Left ventricular mass of persistent masked hypertension in Hong Kong Chinese adolescents: a 4-year follow-up study
- Man-Ching Yam, Hung-Kwan So, Sit-Yee Kwok, Fung-Cheung Lo, Chi-Fung Mok, Chuk-Kwan Leung, Wai-Kwok Yip, Yn-Tz Sung
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- Published online by Cambridge University Press:
- 22 April 2018, pp. 837-843
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Objective
In our previous study, the prevalence of childhood masked hypertension was 11%. This study aims to assess the left ventricular mass index of persistent masked hypertension and determine the factors of elevated left ventricular mass index in Hong Kong Chinese adolescents from a community cohort.
DesignCommunity prospective cohort study, follow-up of a case-control study in community.
SubjectsPatients with masked hypertension at baseline were invited to recheck ambulatory blood pressure for the persistence of masked hypertension.
ResultsA total of 144 out of 165 patients with masked hypertension in the 2011/2012 ambulatory blood pressure survey consented to participate in the study. In all, 48 patients were found to have persistent masked hypertension by ambulatory blood pressure rechecking and were matched with normotensive controls by sex, age, and body height. The left ventricular mass (117.3±39.9 g versus 87.0±28.2 g versus 102.0±28.2 g) and left ventricular mass index (30.1±8.4 g/m2.7 versus 23.9±6.3 g/m2.7 versus 25.1±5.7 g/m2.7) were significantly higher in the persistent masked hypertension group (p<0.0001) compared with the patients without persistent masked hypertension and controls. In multivariate linear regression analysis, left ventricular mass index was found to be higher in male gender (β=4.874, p<0.0001) and the patients with persistent masked hypertension (β=2.796, p=0.003). In addition, left ventricular mass index was positively associated with body mass index z-score (β=3.045, p<0.0001) and low-density lipoprotein cholesterol concentration (β=1.634, p=0.015).
ConclusionsPersistent masked hypertension in adolescents is associated with elevated left ventricular mass index.