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Demographics of a large paediatric Postural Orthostatic Tachycardia Syndrome Program
- Jeffrey R. Boris, Thomas Bernadzikowski
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- Published online by Cambridge University Press:
- 23 January 2018, pp. 668-674
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Objective
The aim of this study was to identify and evaluate demographic and clinical features of paediatric patients with postural orthostatic tachycardia syndrome in a tertiary hospital speciality clinic.
MethodThis is a retrospective review of clinical data obtained during initial outpatient evaluation.
ResultsA total of 708 patients met the evaluation criteria. Female patients outnumbered males, 3.45:1. Caucasians were over-represented at 94.1% of patients. Median age at diagnosis was 15.7 years. Joint hypermobility occurred in 57.3% of patients; 22.4% had hypermobile Ehlers–Danlos syndrome; and 34.9% had hypermobility spectrum disorder. Median age of onset of symptoms was 12.6 years in patients with hypermobility versus 13.7 years in those without (p=0.0001). Median duration of symptoms was 3.3 years with hypermobility versus 1.5 years without (p<0.00001). Putative triggers included infection in 23.6% of patients, concussion in 11.4%, and surgery/trauma in 2.8%. Concurrent inflammatory disorders were noted in 5.2% of patients. Six symptoms comprised 80% of initial patient complaints. Overall, 66% of patients subsequently had at least 10 symptoms, 50% had at least 14 symptoms, and 30% reported at least 26 symptoms. Symptoms were largely cardiovascular, gastrointestinal, and neurological. Paediatric patients with postural orthostatic tachycardia syndrome seen in a large speciality clinic are predominantly female, are mostly Caucasian, have onset of symptoms in early adolescence, and have symptoms for over two years before diagnosis. Over half of patients have joint hypermobility. More than one-third of patients have a possible autoimmune or inflammatory trigger, including infection, concussion, or surgery/trauma. Patients experience symptoms that are highly variable and multi-system in origin over the course of illness.
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Percutaneous pulmonary valve implantation with the Venus P-valve: clinical experience and early results
- Worakan Promphan, Pimpak Prachasilchai, Suvipaporn Siripornpitak, Shakeel A. Qureshi, Thanarat Layangool
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- Published online by Cambridge University Press:
- 19 June 2015, pp. 698-710
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Background
At present, the exclusion for percutaneous pulmonary valve implantation for free pulmonary regurgitation after tetralogy of Fallot repair includes an unfavourably large right ventricular outflow tract.
ObjectiveTo report feasibility and early experience with a recently developed transcatheter heart valve, Venus P-valveTM, implanted in six patients with severe pulmonary regurgitation with large right ventricular outflow tracts.
PatientsThere were two female patients and four male patients. The median age of the patients was 18.5 years, and the mean body weight was 53.8 kg. All the patients were in NYHA class II and had severe pulmonary regurgitation after previous transannular patch repair of tetralogy of Fallot. The median time after the last surgical operation was 13.5 years.
ResultsThe Venus P-valveTM was successfully implanted in all the patients with implanted valve diameters ranging from 24 to 32 mm. The mean fluoroscopy time was 29.8 minutes. None of the patients had significant outflow tract gradient or pulmonary regurgitation immediately after valve implantation. Only one patient had unexpected mild proximal valve migration to the right ventricular body during withdrawal of the delivery system. It caused mild paravalvar leak and significant tricuspid regurgitation. At 6 months follow-up, the median of right ventricular end-diastolic volume indices decreased from 146 to 108 ml/m2 (p-value=0.046). The Doppler systolic peak gradient across the valve ranged from 4 to 40 mmHg, and there was no evidence of stent fracture on fluoroscopy or structural valve failure.
ConclusionThe Venus P-valveTM can be implanted successfully and effectively in patients with severe pulmonary regurgitation and a large right ventricular outflow tract. The early results with this valve are encouraging.
- Cited by 63
Cardiovascular findings, and clinical course, in patients with Williams syndrome
- Eva Bruno, Norma Rossi, Oscar Thüer, Roque Córdoba, Luis E. Alday
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- Published online by Cambridge University Press:
- 24 May 2005, pp. 532-536
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Aims: We investigated the incidence and outcome of cardiac malformations in 53 patients with Williams syndrome. Methods and results:The mean age, and period of follow-up, were 3.6 and 5.3 years, with standard deviations of 4.0 and 5.6 years, respectively. Of the patients, 45 (85%) had cardiovascular anomalies, often combined. Males presented earlier than females, at the ages of 2.1 years, with standard deviation of 2.8 years, as opposed to 4.5 years, with standard deviation of 4.2 years (p < 0.01). Supravalvar aortic stenosis occurred in 32 patients (71%), pulmonary arterial stenosis in 17 (38%), and mitral valvar prolapse in 12 (27%), 9 of these having regurgitant valves. Pulmonary valvar stenosis, ventricular septal defect, coarctation of the aorta, persistent patency of the arterial duct, hypertrophic cardiomyopathy, and subaortic stenosis all occurred less frequently. In 21 patients (47%), 24 surgical or catheter interventions had been made, most often for repair of supravalvar aortic stenosis, undertaken on 16 occasions with just one recurrence, and in 4 along with surgery to the mitral valve. Other lesions requiring intervention were pulmonary valvar stenosis, pulmonary arterial stenosis, coarctation of the aorta, and subaortic stenosis. We lost 3 patients (7%), with severe supravalvar aortic stenosis and moderate or severe mitral regurgitation, 2 early and one late after surgery. Conclusion: The most frequent cardiovascular anomalies in Williams syndrome were supravalvar aortic stenosis, pulmonary arterial stenosis, and mitral valvar prolapse, which occurred more frequently in our patients than previously observed. Patients with left ventricular pressure and volume overload were at greater risk.
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A European multicentric experience using the CardioSEAL® and Starflex double umbrella devices to close interatrial communications holes within the oval fossa
- M. Carminati, S. Giusti, G. Hausdorf, S. Qureshi, M. Tynan, M. Witsenburg, J. Hess, JF Piechaud, P. Bonhoeffer, A. Donti, C. Ovaert, H. Sievert, N. Elzenga, M. Talsma, A. van Oort, J. Ernst, M. Gewillig, B. DeGeeter
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- Published online by Cambridge University Press:
- 19 August 2008, pp. 519-526
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In this review, we describe the experience from 13 European centres using the CardioSEAL and Starflex double umbrella devices to close interatrial communications within the oval fossa (so-called ‘stcundum’ defects). Between October 1996 and April 1999, the procedure was attempted in 334 patients with a mean age of 12 years and a mean weight of 44kg. The mean measured stretched diameter of the defect was 15 mm. In the overall group, the defect was solitary in 245 patients (73%), multiple in 21 (6%), associated with an aneurysm of the flap valve in 15 (5%), was represented by patency of the oval foramen in 44 (13%), and was a fenestration in a Fontan repair in 9 (3%). In all patients, the devices were inserted under general anesthesia, using fluoroscopic and transesophageal echocardiographic control. Implantation was achieved in 325 (97,3%). The device embolized within either a few minutes or a few hours in 13 patients (4%). Of these, uncomplicated surgical repair was undertaken in 10, while the device was retrieved in 3 using catheters and a second device was successfully implanted. Residual shunting was detected immediately after the procedure in 41% of the patients, with the incidence decreasing to 31% at discharge, 24% at 1 month, 21% at 6 months, and 20.5% at one year. During the period of follow-up, elective surgical repair became necessary in two patients, due to malposition of the device in one, and late embolization in the other. Fractures of arms were seen in 6.1%, most commonly with the largest devices. All those with fractured arms of the device were asymptomatic, and no clinical complications related to the fractures were observed. There were no arrythmias, endocarditis, valvar distortion, thromboembolic events, or other complications. After one year of follow-up, clinical success, defined as complete closure of the defect or presence of only a trivial leak, had been obtained in 92.5% of the patients. We conclude, therefore, that these devices produce excellent results when used to close defects of small to moderate size. Results are less than optimal, or else complications ensure, when attempts are made to close very large defects.
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Data integrity of the Pediatric Cardiac Critical Care Consortium (PC4) clinical registry
- Michael Gaies, Janet E. Donohue, Gina M. Willis, Andrea T. Kennedy, John Butcher, Mark A. Scheurer, Jeffrey A. Alten, J. William Gaynor, Jennifer J. Schuette, David S. Cooper, Jeffrey P. Jacobs, Sara K. Pasquali, Sarah Tabbutt
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- Published online by Cambridge University Press:
- 11 September 2015, pp. 1090-1096
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Background
Clinical databases in congenital and paediatric cardiac care provide a foundation for quality improvement, research, policy evaluations and public reporting. Structured audits verifying data integrity allow database users to be confident in these endeavours. We report on the initial audit of the Pediatric Cardiac Critical Care Consortium (PC4) clinical registry.
Materials and methodsParticipants reviewed the entire registry to determine key fields for audit, and defined major and minor discrepancies for the audited variables. In-person audits at the eight initial participating centres were conducted during a 12-month period. The data coordinating centre randomly selected intensive care encounters for review at each site. The audit consisted of source data verification and blinded chart abstraction, comparing findings by the auditors with those entered in the database. We also assessed completeness and timeliness of case submission. Quantitative evaluation of completeness, accuracy, and timeliness of case submission is reported.
ResultsWe audited 434 encounters and 29,476 data fields. The aggregate overall accuracy was 99.1%, and the major discrepancy rate was 0.62%. Across hospitals, the overall accuracy ranged from 96.3 to 99.5%, and the major discrepancy rate ranged from 0.3 to 0.9%; seven of the eight hospitals submitted >90% of cases within 1 month of hospital discharge. There was no evidence for selective case omission.
ConclusionsBased on a rigorous audit process, data submitted to the PC4 clinical registry appear complete, accurate, and timely. The collaborative will maintain ongoing efforts to verify the integrity of the data to promote science that advances quality improvement efforts.
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Echocardiographic and electrocardiographic identification of those children with hypertrophic cardiomyopathy who should be considered at high-risk of dying suddenly
- Ingegerd Östman-Smith, Göran Wettrell, Barry Keeton, Tomas Riesenfeld, Daniel Holmgren, Ulf Ergander
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- Published online by Cambridge University Press:
- 18 November 2005, pp. 632-642
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Background: Hypertrophic cardiomyopathy is a common cause of sudden death in children. In this study, we aimed to identify clinical measures for stratification of this risk in childhood. Patients and methods: By means of a retrospective cohort study from six regional centres of paediatric cardiology, we identified 128 patients with hypertrophic cardiomyopathy presenting below 19 years of age, with a mean follow-up of 10.8 years. Of the patients, 31 had died, 16 suddenly, with a median age at sudden death of 13.3 years. Results: Cox regression shows that electrocardiographic voltages, analysed as the sum of the R and S waves in all six limb leads (p equal to 0.001), and septal thickness expressed as proportion of the 95th centile for age (p equal to 0.036), were independent predictors of sudden death. When the sum of the R and S waves is over 10 millivolts, the odds ratio for sudden death was 8.4, with 95% confidence intervals from 2.2 to 33.7 (p equal to 0.0012), and finding a septal thickness over 190% of 95th centile for age gives an odds ratio of 6.2, with confidence intervals from 1.5 to 25.1 (p equal to 0.011). Noonan's syndrome, with a p value equal to 0.043, and the ratio of the left ventricular wall to its cavity in diastole, with a p value equal to 0.005, were independent predictors of death in cardiac failure, with a ratio of the mural thickness to the dimension of the cavity over 0.30 giving an odds ratio of 36.0, with confidence limits from 4.2 to 311, and a p value equal to 0.00009. At follow-up, patients deemed to be at a high risk of dying suddenly were identified by the combination of the sum of the R and S waves greater than 10 millivolts and septal thickness over 190%, with a sensitivity of 91%, specificity of 78%, positive predictive value of 50%, and a negative predictive value of 97%. Conclusions: Children at high risk of dying suddenly with hypertrophic cardiomyopathy, with a subsequent annual mortality of 6.6%, can be distinguished at the time of diagnosis from those patients having a low risk of sudden death, the latter with an annual mortality of 0.27%.
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Oral feeding outcomes in neonates with congenital cardiac disease undergoing cardiac surgery
- Sharon Sables-Baus, Jon Kaufman, Paul Cook, Eduardo M. da Cruz
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- Published online by Cambridge University Press:
- 04 July 2011, pp. 42-48
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Background
Achievement of adequate oral nutrition is a challenging task after early neonatal cardiac surgery. This study aims to describe predictors of oral feeding outcomes for neonates after early surgical interventions.
Materials and methodsA retrospective review of neonates admitted with congenital cardiac disease over a period of 1 year. We analysed predictors of the need for a feeding tube at discharge and the amount taken at each feeding. Multilevel modelling was used to look at individual change over time predicting oral amount at each feeding.
ResultsWe identified 56 neonates. Diagnoses were heterogeneous; 23% of the infants had associated genetic syndromes and 45% required pre-operative mechanical ventilation. The median time from birth to surgery was 8.4 days, with 29 infants fed orally before surgery. The mean time from surgery to first oral feeding attempt was 12 hours. Time from surgery to oral feeding, the amount taken with first feeding, and cross-clamp times were significant predictors of oral feeding success, whereas the presence of a comorbidity – genetic abnormality – and longer ventilator dependency predicted failure. Almost half of the neonates required a feeding tube upon discharge, and no infant discharged was solely breastfed. Discharge with a feeding tube was associated with greater weight gain at that time.
ConclusionsNeonates with congenital cardiac disease face significant barriers to successfully achieving oral feeding on hospital discharge. Enteral feeding guidelines focus on physiological stabilisation and do not always address the developmental milestones necessary to support oral feeding. Future prospective studies are necessary to identify multimodal strategies to optimise early feeding.
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Exercise capacity in the Fontan circulation
- David J. Goldberg, Catherine M. Avitabile, Michael G. McBride, Stephen M. Paridon
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- Published online by Cambridge University Press:
- 09 January 2014, pp. 824-830
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The Fontan operation can create a stable circulation from childhood through early adulthood. However, the absence of a sub-pulmonary pumping chamber leads to a physiology in which exercise capacity is limited and decreases with age starting in adolescence. The limitation in exercise capacity is more pronounced at peak levels of exercise, but is still present during more modest levels of activity. The underlying causes of exercise impairment relate to both central cardiovascular factors (oxygen delivery) and peripheral factors (oxygen extraction). Interventions to improve cardiac preload and to improve lean muscle mass may help to improve exercise capacity and, perhaps, will alter the “natural history” of the progressive decline.
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The registry of anomalous aortic origin of the coronary artery of The Congenital Heart Surgeons’ Society
- Julie A. Brothers, J. William Gaynor, Jeffrey P. Jacobs, Christopher Caldarone, Anusha Jegatheeswaran, Marshall L. Jacobs, The Anomalous Coronary Artery Working Group
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- Published online by Cambridge University Press:
- 01 December 2010, pp. 50-58
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The anomalous aortic origin of a coronary artery from the wrong sinus of Valsalva with interarterial, intramural, and/or intraconal course is a rare congenital anomaly that is associated with a high risk of sudden death in children. The Congenital Heart Surgeons’ Society established the Registry of Anomalous Aortic Origin of the Coronary Artery to help determine the outcome of children and young adults managed with surgical intervention versus observation and to test the hypothesis that subsets of patients with anomalous aortic origin of a coronary artery can be identified in whom the risk of intervention is less than the risk of observation. All institutional members of the Congenital Heart Surgeons’ Society were recruited for participation. The registry consists of a retrospective cohort of patients diagnosed between 1 January, 1998 and 20 January, 2009 and a prospective, population-based cohort of patients newly diagnosed from 21 January, 2009 onwards. Baseline demographics, diagnoses, and results of tests will be obtained through a review of the medical records. Annual follow-up data will be collected. Data will be analysed for different factors of risk at diagnosis, different strategies of treatment, and the impact of both on the outcomes of the patients. As of June 2010, 28 institutions had applied for approval from their institutional review board and 16 institutions had received approval from their institutional review board. Seventy-four patients have enrolled to date. We hope to use the established Pediatric Cardiomyopathy Registry as a guide to successful implementation, with a cooperative effort between institutions. The overall purpose of the Registry of Anomalous Aortic Origin of the Coronary Artery is to determine the outcome of surgical intervention versus observation in children and young adults with anomalous aortic origin of a coronary artery, and to describe the natural and “unnatural” history of these patients over the course of their lifetime. In this report, we describe the creation and design of the Registry of Anomalous Aortic Origin of the Coronary Artery. Data from the registry will be published at a later date.
- Cited by 60
Anatomy and echocardiography of the normal and abnormal tricuspid valve
- Richard M. Martinez, Patrick W. O'Leary, Robert H. Anderson
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- Published online by Cambridge University Press:
- 13 October 2006, pp. 4-11
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Perhaps because it guards the inlet to the lesser circulation, the morphologically tricuspid valve has received less attention in terms of its anatomy than the well-explored mitral valve, which will receive attention in a subsequent review in this supplement.1 As we will show in our initial review, nonetheless, the approach to morphological analysis is the same for both valves, irrespective of whether the specific morphology is displayed in the autopsy room or the echocardiographic laboratory. It is essential that the valve be analysed so as to reveal the precise structure of each if its components – the so-called valvar complex.2 Equally important, in the current era, with the burgeoning use of three-dimensional displays that place the heart firmly within the context of the body, it is essential that the components of the valve be described as seen relative to the bodily axis,3 rather than following the present custom of describing the heart as though it is removed from the body and positioned on its own apex.
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Cardiac changes in anorexia nervosa
- Michael A. Spaulding-Barclay, Jessica Stern, Philip S. Mehler
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- Published online by Cambridge University Press:
- 14 January 2016, pp. 623-628
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Introduction
Anorexia nervosa is an eating disorder, which is associated with many different medical complications as a result of the weight loss and malnutrition that characterise this illness. It has the highest mortality rate of any psychiatric disorder. A large portion of deaths are attributable to the cardiac abnormalities that ensue as a result of the malnutrition associated with anorexia nervosa. In this review, the cardiac complications of anorexia nervosa will be discussed.
MethodsA comprehensive literature review on cardiac changes in anorexia nervosa was carried out.
ResultsThere are structural, functional, and rhythm-type changes that occur in patients with anorexia nervosa. These become progressively significant as ongoing weight loss occurs.
ConclusionCardiac changes are inherent to anorexia nervosa and they become more life-threatening and serious as the anorexia nervosa becomes increasingly severe. Weight restoration and attention to these cardiac changes are crucial for a successful treatment outcome.
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Optimising echocardiographic screening for rheumatic heart disease in New Zealand: not all valve disease is rheumatic
- Rachel H. Webb, Nigel J. Wilson, Diana R. Lennon, Elizabeth M. Wilson, Ross W. Nicholson, Tom L. Gentles, Clare P. O'Donnell, John W. Stirling, Irene Zeng, Adrian A. Trenholme
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- Published online by Cambridge University Press:
- 31 March 2011, pp. 436-443
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Aims
Echocardiography detects a greater prevalence of rheumatic heart disease than heart auscultation. Echocardiographic screening for rheumatic heart disease combined with secondary prophylaxis may potentially prevent severe rheumatic heart disease in high-risk populations. We aimed to determine the prevalence of rheumatic heart disease in children from an urban New Zealand population at high risk for acute rheumatic fever.
Methods and resultsTo optimise accurate diagnosis of rheumatic heart disease, we utilised a two-step model. Portable echocardiography was conducted on 1142 predominantly Māori and Pacific children aged 10–13 years. Children with an abnormal screening echocardiogram underwent clinical assessment by a paediatric cardiologist together with hospital-based echocardiography. Rheumatic heart disease was then classified as definite, probable, or possible. Portable echocardiography identified changes suggestive of rheumatic heart disease in 95 (8.3%) of 1142 children, which reduced to 59 (5.2%) after cardiology assessment. The prevalence of definite and probable rheumatic heart disease was 26.0 of 1000, with 95% confidence intervals ranging from 12.6 to 39.4. Portable echocardiography overdiagnosed rheumatic heart disease with physiological valve regurgitation diagnosed in 28 children. A total of 30 children (2.6%) had non-rheumatic cardiac abnormalities, 11 of whom had minor congenital mitral valve anomalies.
ConclusionsWe found high rates of undetected rheumatic heart disease in this high-risk population. Rheumatic heart disease screening has resource implications with cardiology evaluation required for accurate diagnosis. Echocardiographic screening for rheumatic heart disease may overdiagnose rheumatic heart disease unless congenital mitral valve anomalies and physiological regurgitation are excluded.
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Preoperative malnutrition is associated with increased mortality and adverse outcomes after paediatric cardiac surgery
- Faith Ross, Gregory Latham, Denise Joffe, Michael Richards, Jeremy Geiduschek, Michael Eisses, Douglas Thompson, Monique Radman
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- Published online by Cambridge University Press:
- 19 June 2017, pp. 1716-1725
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Background
Malnutrition is common in children with CHD and is likely to place them at an increased risk for adverse surgical outcomes. We sought to evaluate the impact of preoperative malnutrition on outcomes after paediatric cardiac surgery.
MethodsWe conducted a retrospective analysis of patients from age 0 to 5 years undergoing cardiac surgery at Seattle Children’s Hospital from 2006 to 2015. We used regression modelling to examine the impact of malnutrition on surgical outcomes.
ResultsWe found a non-linear relationship between low height-for-age and weight-for-age z-scores and mortality after surgery. In the range of z-score ⩽−2, each additional unit decrease in height-for-age or weight-for-age z-score was associated with a 2.9 or 2.1% increased risk for mortality, respectively. Each unit decrease in height-for-age z-score was associated with a 1.2% increased risk for cardiac arrest, 1.1% increased risk for infection, and an average of 1.7 additional hours of mechanical ventilation, 6 hours longer ICU stay, and 13 hours longer hospital stay. Each unit decrease in weight-for-age z-score was associated with a 0.7% increased risk for cardiac arrest, 0.8% increased risk for infection, and an average of 1.9 additional hours of mechanical ventilation and 5.3 additional hours of ICU stay.
ConclusionsThis study is unique in demonstrating a significant association between malnutrition and 30-day mortality and other adverse outcomes after paediatric cardiac surgery in a mixed population of CHD patients. By evaluating nutritional status as a continuous variable, we were able to clearly distinguish the point at which malnutrition begins to affect mortality.
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Perioperative feeding management of neonates with CHD: analysis of the Pediatric Cardiac Critical Care Consortium (PC4) registry
- Jeffrey A. Alten, Leslie A. Rhodes, Sarah Tabbutt, David S. Cooper, Eric M. Graham, Nancy Ghanayem, Bradley S. Marino, Mayte I. Figueroa, Nikhil K. Chanani, Jeffrey P. Jacobs, Janet E. Donohue, Sunkyung Yu, Michael Gaies
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- Published online by Cambridge University Press:
- 16 December 2015, pp. 1593-1601
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Introduction
The optimal perioperative feeding strategies for neonates with CHD are unknown. In the present study, we describe the current feeding practices across a multi-institutional cohort.
MethodsInclusion criteria for this study were as follows: all neonates undergoing cardiac surgery admitted to the cardiac ICU for ⩾24 hours preoperatively between October, 2013 and July, 2014 in the Pediatric Cardiac Critical Care Consortium registry.
ResultsThe cohort included 251 patients from eight centres. The most common diagnoses included the following: hypoplastic left heart syndrome (17%), coarctation/aortic arch hypoplasia (18%), and transposition of the great arteries (22%); 14% of the patients were <37weeks of gestational age. The median total hospital length of stay was 21 days (interquartile range (IQR) 14–35) and overall mortality was 8%. Preoperative feeding occurred in 133 (53%) patients. The overall preoperative feeding rates across centres ranged from 29 to 79%. Postoperative feeds started on median day 2 (IQR 1–4); for patients with hypoplastic left heart syndrome postoperative feeds started on median day 4. Postoperative feeds were initiated in 89 (35%) patients before extubation (range across centres: 21–61%). The median cardiac ICU discharge feeding volume was 108 cc/kg/day, varying across centres. The mean discharge weight was 280 g above birth weight, ranging from +100 to 430 g across centres. A total of 110 (44%) patients had discharge feeding tubes, ranging from 6 to 80% across centres, and 40/110 patients had gastrostomy/enterostomy tubes placed. In addition, eight (3.2%) patients developed necrotising enterocolitis – three preoperatively and five postoperatively.
ConclusionIn this cohort, neonatal feeding practices and outcomes appear to vary across diagnostic groups and institutions. Only half of the patients received preoperative enteral nutrition; almost half had discharge feeding tubes. Multi-institutional collaboration is necessary to determine feeding strategies associated with best clinical outcomes.
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How best to assess right ventricular function by echocardiography*
- Michael P. DiLorenzo, Shivani M. Bhatt, Laura Mercer-Rosa
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- Published online by Cambridge University Press:
- 16 December 2015, pp. 1473-1481
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Right ventricular function is a crucial determinant of long-term outcomes of children with heart disease. Quantification of right ventricular systolic and diastolic performance by echocardiography is of paramount importance, given the prevalence of children with heart disease, particularly those with involvement of the right heart, such as single or systemic right ventricles, tetralogy of Fallot, and pulmonary arterial hypertension. Identification of poor right ventricular performance can provide an opportunity to intervene. In this review, we will go through the different systolic and diastolic indices, as well as their application in practice. Quantification of right ventricular function is possible and should be routinely performed using a combination of different measures, taking into account each disease state. Quantification is extremely useful for individual patient follow-up. Laboratories should continue to strive to optimise reproducibility through quality improvement and quality assurance efforts in addition to investing in technology and training for new, promising techniques, such as three-dimensional echocardiography.
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A practical and transferable new protocol for treadmill testing of children and adults**
- Karl-Otto Dubowy, Winfried Baden, Stefan Bernitzki, Brigitte Peters
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- Published online by Cambridge University Press:
- 01 December 2008, pp. 615-623
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Background
It is just as vital to have an exact overview of the physical fitness of young and growing people as it is for adults. The currently used exercise protocols have limitations in healthy small children, and in senior citizens. In particular with chronically ill patients, regardless of their age, there is a need for an exercise protocol that permits observations over the long term. With this need in mind, we have designed a new transferable standardised exercise protocol, constructing reference values based on improved assessments on a treadmill that permitted stepwise increases of speed and gradient every 90 seconds – the so called treadmill protocol from the German Society of Paediatric Cardiology.
ObjectivesWe investigated the exercise performance in a healthy Caucasian population ranging in age from 4 to 75 years.
MethodsWe measured, using a prospective study design, the distance run, the endurance, and the consumption of oxygen in 548 females and 647 males undergoing an enhanced spiroergometric treadmill protocol in two centres.
Results and conclusionsUntil puberty, boys and girls have the same indicators of exercise performance. Subsequent to puberty, uptake of oxygen and distance run differ, with males showing higher uptake of oxygen. There is still an age-dependent dynamic of peak uptake of oxygen related to body surface area. Using these new reference values, covering the whole range of age, it proves possible to compare performance during growth and aging of the individual. In this fashion, we have calculated centiles for all recorded variables. External calibration, validation and quality control ensures transferability of our data to other spiroergometry units.
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The effect of milrinone on right and left ventricular function when used as a rescue therapy for term infants with pulmonary hypertension
- Adam T. James, John D. Corcoran, Patrick J. McNamara, Orla Franklin, Afif F. El-Khuffash
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- Published online by Cambridge University Press:
- 20 January 2015, pp. 90-99
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Introduction
Milrinone may be an appropriate adjuvant therapy for infants with persistent pulmonary hypertension of the newborn. We aimed to describe the effect of milrinone administration on right and left ventricular function in infants with persistent pulmonary hypertension not responding to inhaled nitric oxide after 4 hours of administration.
Materials and methodsThis is a retrospective review of infants born after or at 34 weeks of gestation with persistent pulmonary hypertension who received milrinone treatment. The primary endpoint was the effect of milrinone on myocardial performance and haemodynamics, including right and left ventricular outputs, tissue Doppler velocities, right ventricle and septal strain, and strain rate. Secondary endpoints examined included duration of inhaled nitric oxide and oxygen support.
ResultsA total of 17 infants with a mean (standard deviation) gestation and birth weight of 39.8 (2.0) weeks and 3.45 (0.39) kilograms, respectively, were included in the study. The first echocardiogram was performed 15 hours after the commencement of nitric oxide inhalation. Milrinone treatment was started at a median time of 1 hour after the echocardiogram and was associated with an increase in left ventricular output (p=0.04), right ventricular output (p=0.004), right ventricle strain (p=0.01) and strain rate (p=0.002), and left ventricle s` (p<0.001) and a` (p=0.02) waves. There was a reduction in nitric oxide dose and oxygen requirement over the subsequent 72 hours (all p<0.05).
ConclusionThe use of milrinone as an adjunct to nitric oxide is worth further exploration, with preliminary evidence suggesting an improvement in both oxygenation and myocardial performance in this group of infants.
- Cited by 57
Nomenclature of the functionally univentricular heart
- Marshall L. Jacobs, Robert H. Anderson
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- Published online by Cambridge University Press:
- 10 January 2006, pp. 3-8
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Hearts which, at first sight, seem to have a solitary chamber within their ventricular mass have long been the subject of controversy. As difficult as it is to manage these cardiac malformations medically and surgically, it has been at least as challenging, to date, merely to describe and classify them. Even the most commonly used terms, “single ventricle” and “univentricular heart”, spark heated debate. In distant times, when congenitally malformed hearts were pathological curiosities, these entities were described as “cor triloculare biatriale”. Therein lies the beginning of the problem, since when hearts of this type were examined by more enlightened pathologists, such as the great Maude Abbott,1 it became plain that the apparently solitary ventricular mass in reality possessed a second, albeit much smaller, chamber. Abbott described this second structure as the “outlet chamber”. This convention of describing a “single ventricle”, albeit with a co-existing “outlet chamber”, that presumably lacked ventricular status, continued throughout the first half of the twentieth century, although it had been recognised by then that hearts could rarely be found with truly solitary ventricles, and these were typically deemed to be common structures. Van Praagh et al.2 neatly summarised the problem with this approach when they pointed out that the so-called “single ventricle” possessed two ventricular chambers, whilst the “common ventricle” described the truly solitary arrangement. In their seminal investigation of 1964, Van Praagh et al.2 analysed only those hearts unified because of double inlet atrioventricular connection, or alignment. They excluded arbitrarily from their investigation all hearts with atrioventricular valvar atresia, despite the similarity in morphology between many of these latter lesions and the hearts with double inlet.3
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Regional right and left ventricular function after the Senning operation: an ultrasonic study of strain rate and strain
- Benedicte Eyskens, Frank Weidemann, Miroslaw Kowalski, Jan Bogaert, Steven Dymarkowski, Bart Bijnens, Marc Gewillig, George Sutherland, Luc Mertens
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- Published online by Cambridge University Press:
- 21 January 2005, pp. 255-264
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Background: Systemic right ventricular dysfunction is a major concern in the follow-up of patients who underwent an atrial redirection procedure for transposition (concordant atrio-ventricular and discordant ventriculo-arterial connections). No good non-invasive method is currently available for quantifying right ventricular function. Aims: We have used ultrasonically based imaging of strain rate and strain to quantify regional deformation in the right ventricle after the Senning operation, comparing properties of regional deformation of the right ventricle with right ventricular ejection fraction as measured using magnetic resonance imaging. Methods: In 20 asymptomatic patients who had undergone the Senning procedure, we measured peak systolic strain rate and systolic strain values in the right ventricular free wall, the septum and the left ventricular lateral wall using colour Doppler myocardial imaging, comparing the data with findings obtained in 30 healthy subjects. Global right ventricular ejection fraction was assessed using magnetic resonance imaging. Results: Properties of deformation of the right ventricular free wall were reduced and homogeneous after the Senning procedure compared to normals, with significantly lower values for peak systolic strain rate and systolic strain (−1.1 ± 0.4 vs. −2.5 ± 0.9 s−1; p < 0.05 and −16 ± 7% vs. −38 ± 13%; p < 0.05, respectively). There was a significant correlation between regional longitudinal right ventricular systolic strain values and right ventricular ejection fraction (r = −0.87, p < 0.001). In the septum, peak systolic strain rate was again reduced and homogeneous (−1.2 ± 0.4 vs. −1.8 ± 0.5 s−1; p < 0.05 vs. normals). Also in the left ventricle, the lateral wall peak systolic strain rate and systolic strain values were reduced (−1.5 ± 0.5 vs. −2.1 ± 0.9 s−1; p < 0.05 and −20 ± 6% vs.−25 ± 9%; p < 0.05, vs. normals, respectively). Conclusions: Properties of regional longitudinal deformation of the systemic right ventricle are reduced after the Senning procedure compared to normal controls, and correlate well with global right ventricular performance. These findings suggest that ultrasonic strain rate and strain imaging could be used in the non-invasive follow-up of ventricular function in these patients.
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Abnormalities of the major intrathoracic arteries in Turner syndrome as revealed by magnetic resonance imaging
- Kristian Havmand Mortensen, Britta Eilersen Hjerrild, Niels Holmark Andersen, Keld Ejvind Sørensen, Arne Hørlyck, Erik Morre Pedersen, Erik Lundorf, Jens Sandahl Christiansen, Claus Højbjerg Gravholt
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- Published online by Cambridge University Press:
- 22 March 2010, pp. 191-200
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Background
Ectatic aortopathy and arterial abnormalities cause excess morbidity and mortality in Turner syndrome, where a state of vasculopathy seemingly extends into the major head and neck branch arteries.
ObjectiveWe investigated the prevalence of abnormalities of the major intrathoracic arteries, their interaction with arterial dimensions, and their association with karyotype.
DesignMagnetic resonance imaging scans determined the arterial abnormalities as well as head and neck branch artery and aortic dimensions in 99 adult women with Turner syndrome compared with 33 healthy female controls. Echocardiography determined aortic valve morphology.
ResultsIn Turner syndrome, the relative risk of any congenital abnormality was 7.7 (p = 0.003) and 6.7 of ascending aortic dilation (p = 0.02). A bovine aortic arch was seen in both Turner syndrome and controls. Other abnormalities were only encountered in Turner syndrome: elongated transverse aortic arch (47%), bicuspid aortic valve (27%), aortic coarctation (13%), aberrant right subclavian artery (8%), and aortic arch hypoplasia (2%). The innominate and left common carotid arteries were enlarged in Turner syndrome (p < 0.001). Significant associations were first, bicuspid aortic valve with aortic coarctation, elongated transverse aortic arch, and ascending aortic dilation; second, aortic coarctation with elongated aortic arch and descending aortic dilation; third, 45,X with aortic coarctation, elongated transverse aortic arch and ascending aortic dilation; and fourth, branch artery dilation with bicuspid aortic valve, aortic coarctation, elongated transverse aortic arch and 45,X.
ConclusionAn increased risk of arterial abnormalities, aortic dilation, and enlargement of the branch arteries was found in Turner syndrome without distinct patterns of co-segregation.