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Somatic growth failure after the Fontan operation
- Mitchell I. Cohen, David M. Bush, Robert J. Ferry, Jr, Thomas L. Spray, Thomas Moshang, Jr, Gil Wernovsky, Victoria L. Vetter
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- Published online by Cambridge University Press:
- 19 August 2008, pp. 447-457
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Our study was designed to characterize the patterns of growth, in the medium term, of children with functionally univentricular hearts managed with a hemi-Fontan procedure in infancy, followed by a modified Fontan operation in early childhood. Failure of growth is common in patients with congenital cardiac malformations, and may be related to congestive heart failure and hypoxia. Repair of simple lesions appears to reverse the retardation in growth. Palliation of the functionally single ventricular physiology with a staged Fontan operation reduces the adverse effects of hypoxemia and prolonged ventricular volume overload. The impact of this approach on somatic growth is unknown. Retrospectively, we reviewed the parameters of growth of all children with functionally univentricular hearts followed primarily at our institution who had completed a staged construction of the Fontan circulation between January 1990 and December 1995. Measurements were available on all children prior to surgery, and annually for three years following the Fontan operation. Data was obtained on siblings and parents for comparative purposes. The criterions of eligibility for inclusion were satisfied by 65 patients. The mean Z score for weight was-1·5± 1·2 at the time of the hemi-Fontan operation. Weight improved by the time of completion of the Fontan circulation (−0·91 ±0·99), and for the first two years following the Fontan operation, but never normalized. The mean Z scores for height at the hemi-Fontan and Fontan operations were −0·67 ±1·1 and −0·89±1·2 respectively. At most recent follow-up, with a mean age of 6·1 ± 1·3 years, and a mean time from the Fontan operation of 4·4±1·4 years, the mean Z score for height was −1·15 ±1·2, and was significantly less than comparable Z scores for parents and siblings. In our experience, children with functionally univentricular hearts who have been palliated with a Fontan operation are significantly underweight and shorter than the general population and their siblings
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Nomenclature and databases for the surgical treatment of congenital cardiac disease – an updated primer and an analysis of opportunities for improvement
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- Jeffrey Phillip Jacobs, Marshall Lewis Jacobs, Constantine Mavroudis, Carl Lewis Backer, Francois G. Lacour-Gayet, Christo I. Tchervenkov, Rodney C. G. Franklin, Marie J. Béland, Kathy J. Jenkins, Hal Walters III, Emile A. Bacha, Bohdan Maruszewski, Hiromi Kurosawa, David Robinson Clarke, J. William Gaynor, Thomas L. Spray, Giovanni Stellin, Tjark Ebels, Otto N. Krogmann, Vera D. Aiello, Steven D. Colan, Paul Weinberg, Jorge M. Giroud, Allen Everett, Gil Wernovsky, Martin J. Elliott, Fred H. Edwards
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- 01 December 2008, pp. 38-62
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This review discusses the historical aspects, current state of the art, and potential future advances in the areas of nomenclature and databases for the analysis of outcomes of treatments for patients with congenitally malformed hearts. We will consider the current state of analysis of outcomes, lay out some principles which might make it possible to achieve life-long monitoring and follow-up using our databases, and describe the next steps those involved in the care of these patients need to take in order to achieve these objectives. In order to perform meaningful multi-institutional analyses, we suggest that any database must incorporate the following six essential elements: use of a common language and nomenclature, use of an established uniform core dataset for collection of information, incorporation of a mechanism of evaluating case complexity, availability of a mechanism to assure and verify the completeness and accuracy of the data collected, collaboration between medical and surgical subspecialties, and standardised protocols for life-long follow-up.
During the 1990s, both The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons created databases to assess the outcomes of congenital cardiac surgery. Beginning in 1998, these two organizations collaborated to create the International Congenital Heart Surgery Nomenclature and Database Project. By 2000, a common nomenclature, along with a common core minimal dataset, were adopted by The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons, and published in the Annals of Thoracic Surgery. In 2000, The International Nomenclature Committee for Pediatric and Congenital Heart Disease was established. This committee eventually evolved into the International Society for Nomenclature of Paediatric and Congenital Heart Disease. The working component of this international nomenclature society has been The International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, also known as the Nomenclature Working Group. By 2005, the Nomenclature Working Group crossmapped the nomenclature of the International Congenital Heart Surgery Nomenclature and Database Project of The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons with the European Paediatric Cardiac Code of the Association for European Paediatric Cardiology, and therefore created the International Paediatric and Congenital Cardiac Code, which is available for free download from the internet at [http://www.IPCCC.NET].
This common nomenclature, the International Paediatric and Congenital Cardiac Code, and the common minimum database data set created by the International Congenital Heart Surgery Nomenclature and Database Project, are now utilized by both The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons. Between 1998 and 2007 inclusive, this nomenclature and database was used by both of these two organizations to analyze outcomes of over 150,000 operations involving patients undergoing surgical treatment for congenital cardiac disease.
Two major multi-institutional efforts that have attempted to measure the complexity of congenital heart surgery are the Risk Adjustment in Congenital Heart Surgery-1 system, and the Aristotle Complexity Score. Current efforts to unify the Risk Adjustment in Congenital Heart Surgery-1 system and the Aristotle Complexity Score are in their early stages, but encouraging. Collaborative efforts involving The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons are under way to develop mechanisms to verify the completeness and accuracy of the data in the databases. Under the leadership of The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease, further collaborative efforts are ongoing between congenital and paediatric cardiac surgeons and other subspecialties, including paediatric cardiac anaesthesiologists, via The Congenital Cardiac Anesthesia Society, paediatric cardiac intensivists, via The Pediatric Cardiac Intensive Care Society, and paediatric cardiologists, via the Joint Council on Congenital Heart Disease and The Association for European Paediatric Cardiology.
In finalising our review, we emphasise that analysis of outcomes must move beyond mortality, and encompass longer term follow-up, including cardiac and non cardiac morbidities, and importantly, those morbidities impacting health related quality of life. Methodologies must be implemented in these databases to allow uniform, protocol driven, and meaningful, long term follow-up.
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Paediatric cardiac rehabilitation in congenital heart disease: a systematic review
- Ana Ubeda Tikkanen, Ainhoa Rodriguez Oyaga, Olga Arroyo Riaño, Enrique Maroto Álvaro, Jonathan Rhodes
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- 17 January 2012, pp. 241-250
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Background
Advances in medical and surgical care have contributed to an important increase in the survival rates of children with congenital heart disease. However, survivors often have decreased exercise capacity and health-related issues that affect their quality of life. Cardiac Rehabilitation Programmes have been extensively studied in adults with acquired heart disease. In contrast, studies of children with congenital heart disease have been few and of limited scope. We therefore undertook a systematic review of the literature on cardiac rehabilitation in children with congenital heart disease to systematically assess the current evidence regarding the use, efficacy, benefits, and risks associated with this therapy and to identify the components of a successful programme.
MethodsWe included studies that incorporated a cardiac rehabilitation programme with an exercise training component published between January, 1981 and November, 2010 in patients under 18 years of age.
ResultsA total of 16 clinical studies were found and were the focus of this review. Heterogeneous methodology and variable quality was observed. Aerobic and resistance training was the core component of most studies. Diverse variables were used to quantify outcomes. No adverse events were reported.
ConclusionsCardiac Rehabilitation Programmes in the paediatric population are greatly underutilised, and clinical research on this promising form of therapy has been limited. Questions remain regarding the optimal structure and efficacy of the programmes. The complex needs of this unique population also mandate that additional outcome measures, beyond serial cardiopulmonary exercise testing, be identified and studied.
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Echocardiographic study of paediatric patients with mucopolysaccharidosis
- Gabriela N. Leal, Ana C. de Paula, Cláudio Leone, Chong A. Kim
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- Published online by Cambridge University Press:
- 26 April 2010, pp. 254-261
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Principle
Mucopolysaccharidosis is an inborn error of metabolism causing glucosaminoglycans tissue storage. Cardiovascular involvement is variable but contributes significantly towards the morbidity and mortality of the patients.
ObjectiveTo characterise the echocardiographic abnormalities in children and adolescents with different types of mucopolysaccharidosis.
MethodEchocardiograms and medical records of 28 patients aged 2–14 years, seen from 2003 to 2005, were revised. At that time, the enzymatic replacement therapy was still not available in our institution.
ResultsEchocardiographic alterations were detected in 26 patients (93%), whereas 16 (57%) had abnormal auscultation, and only 6 (21%) presented with cardiovascular complaint. Mitral valve thickening with dysfunction (regurgitation, stenosis, or double lesion) was diagnosed in 60.8%, left ventricular hypertrophy in 43% and aortic valve thickening with regurgitation in 35.8% of the patients. There was no systolic dysfunction and mild left diastolic dysfunction was shown in 21.5% of the patients. Pulmonary hypertension was present in 36% of the patients, causing the only two deaths recorded. There was a strong association between the accumulation of dermatan sulphate and the presence of mitral valve dysfunction (p = 0.0003), aortic valve dysfunction (p = 0.006), and pulmonary hypertension (p = 0.006). Among individuals with two or more examinations, 82% had a worsening evolution.
ConclusionsEchocardiographic alterations in children with Mucopolysaccharidosis are frequent and have a progressive character. Left valve lesions, ventricular hypertrophy, and pulmonary hypertension were the most common findings and there was an association between the accumulation of dermatan sulphate and cardiovascular involvement. Unlike in adults, pulmonary hypertension was the main cause of death, not left ventricle systolic dysfunction.
- Cited by 68
Psychosocial risk in families of infants undergoing surgery for a serious congenital heart disease
- Simone J. Hearps, Maria C. McCarthy, Frank Muscara, Stephen J. C. Hearps, Kylie Burke, Bryn Jones, Vicki A. Anderson
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- Published online by Cambridge University Press:
- 27 June 2013, pp. 632-639
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Objective: The aim of this study was to explore the acute psychosocial risk in families with infants undergoing surgery for a congenital heart disease and, secondarily, to explore the psychosocial impact of antenatal versus post-natal diagnoses. Method: The study sample comprised 39 caregivers (28 mothers) of 29 children diagnosed with a congenital heart disease and requiring surgery within the first 4 weeks of life. Psychosocial risk was measured using the Psychosocial Assessment Tool, which was adapted to include four novel items examining infant risk factors, namely, sleeping, feeding, crying, and bonding difficulties. Parents’ psychosocial risk was measured within 4 weeks after their child's surgery and stratified into a three-tiered framework: Universal, Targeted, and Clinical risk. Results: Of the total sample, 61.5% of parents were classified as Universal, that is, at lowest risk; 35.9% as Targeted, and 2.6% as Clinical. The within-family parent total Psychosocial Assessment Tool score correlations were non-significant, and there were no differences between families of infants who received post-natal versus antenatal diagnosis or single ventricle versus biventricular repair. Linear regression found that a higher parent education significantly predicted a lower total Psychosocial Assessment Tool score. Conclusions: Findings indicate that, although the majority of parents adapt to the acute stress of surgery for a serious cardiac illness in their infant, the remaining 38.5% report an increased psychosocial risk associated with higher rates of emotional distress, which may impact on the parental quality of life and capacity for optimal parenting. The distribution of psychosocial risk in parents of children undergoing surgery for a congenital heart disease is consistent with that described for parents of children with other serious paediatric diagnoses.
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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.
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Neo-aortic valvar function after the arterial switch
- Bradley S. Marino, Gil Wernovsky, Doff B. McElhinney, Abbas Jawad, Dieuwertje L. Kreb, Suzan F. Mantel, Wendy L. van der Woerd, Danielle Robbers-Visser, Rita Novello, J. William Gaynor, Thomas L. Spray, Meryl S. Cohen
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- Published online by Cambridge University Press:
- 20 September 2006, pp. 481-489
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Objectives: The purpose of our study was to assess the prevalence and progression, during childhood and adolescence, of dilation of the neo-aortic root, and neo-aortic valvar regurgitation, and to identify risk factors for such dilation and regurgitation, after the arterial switch operation. Methods: We included all patients who had undergone an arterial switch operation at The Children's Hospital of Philadelphia, and had been followed for a minimum of 4 years, and had at least 2 postoperative echocardiograms. Neo-aortic valvar regurgitation was quantitatively assessed, and measurements were made of the neo-aortic root at the level of the basal attachment of the leaflets, mid-sinusal level, and the sinutubular junction. Results: We found 82 patients who satisfied the criterions for inclusion, of whom 52 patients had transposition with an intact ventricular septum, and 30 had either an associated ventricular septal defect or double outlet right ventricle. The median follow-up time was 8.8 years (4.1 to 16.4 years). The neo-aortic valve had been replaced in 1 patient. Of the patients, 3 had moderate, 66 had trivial to mild, and 12 had no neo-aortic valvar regurgitation at their most recent follow-up. The regurgitation had progressed by at least 1 grade in 38 of the 82 patients (46.4%). Neo-aortic dilation was noted at the basal attachment of the leaflets, and at mid-sinusal level, which was out of proportion to somatic growth. Conclusions: At mid-term follow-up, significant neo-aortic valve regurgitation is present in 3.7%, and trivial to mild regurgitation in 81.4% of patients. The regurgitation progressed in almost half of the patients over time. We also noted progressive dilation of the neo-aortic root out of proportion to somatic growth.
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Feeding, growth, and nutrition in children with congenitally malformed hearts
- Barbara Medoff-Cooper, Maryam Naim, Deborah Torowicz, Antonio Mott
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- 01 December 2010, pp. 149-153
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In the United States of America, approximately 40,000 infants are born annually with congenitally malformed hearts. Children with defects that require complex surgical palliation, or definitive repair, face many challenges in achieving optimal short-term and long-term growth. The presence of associated chromosomal abnormalities, cyanosis, and cardiac failure adds to the complexity and challenge. In this review, we address three themes related to feeding, growth, and nutrition of infants after neonatal cardiac surgery: nutritional challenges after chylothorax; breastfeeding after surgery; and the challenges of feeding after discharge. Chylothorax is a rare complication following cardiothoracic surgery in children. Children with chylothorax have nutritional depletion secondary to protein losses in chylous fluid, hypovolaemia, and electrolyte losses. In spite of the evidence supporting the use of human milk and breastfeeding in preterm infants, barriers to its use appear to persist in infants with critical cardiac disease. Yet, human milk is the preferred form of nutrition for well, preterm, or ill infants. It is well documented that after complex neonatal cardiac surgery medical teams and families struggle with infant feeding problems. Parents have described feeding their children as difficult, time consuming, and anxiety producing. Medical complications such as chylothorax, limited access to human milk, and parental concerns and stress about feeding are but three of the myriad of factors that may contribute to poor outcomes regarding nutrition and growth. Compelling evidence exists that this multi-factorial problem must be addressed with both physiological and behavioural strategies.
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A multicentric study of disease-related stress, and perceived vulnerability, in parents of children with congenital cardiac disease
- C.M. Jantien Vrijmoet-Wiersma, Jaap Ottenkamp, Matty van Roozendaal, Martha A. Grootenhuis, Hendrik M. Koopman
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- Published online by Cambridge University Press:
- 14 October 2009, pp. 608-614
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Parents of children with congenitally malformed hearts can suffer from stress as a result of the medical condition of their child. In this cross-sectional study, we aimed to describe levels of parental stress, and perceived vulnerability, in parents of children who underwent major cardiac surgery, by using both generic and disease-related measures for assessment. We included parents of children who underwent open-heart surgery over the period 2002 through 2007 in the Center for congenital Anomalies Heart Amsterdam/Leiden, abbreviated to provide the acronym CAHAL. In total, we assessed 114 mothers and 82 fathers of 131 children, using the Pediatric Inventory for Parents, short form, General Health Questionnaire, Parental Stress Index-Short Form, State-Trait Anxiety Index and the Child Vulnerability Scale. Compared to the reference groups of the instruments used, parents of children with congenitally malformed hearts did not report higher generic nor disease-related stress scores, and parenting levels of stress were also comparable to reference groups. State anxiety levels, however, were higher in mothers of children with congenitally malformed hearts. Both fathers and mothers reported significantly higher rates of perceived vulnerability than did parents of healthy children. Risk factors for increased anxiety and perceived vulnerability were found in the number of surgical procedures, the time past since the last procedure, and ethnicity. Severity of the lesion did not influence parental levels of stress, but parents of children with hypoplastic left heart syndrome did report higher levels of stress than other parents. Psychosocial screening of parents of children with congenitally malformed hearts is important in order to provide appropriate counselling to those parents most in need.
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Results of 546 Blalock-Taussig shunts performed in 478 patients
- Khalid A. Al Jubair, Mohamed R. Al Fagih, Abdullah S. Al Jarallah, Saad Al Yousef, M.A. All Khan, Abdullah Ashmeg, Yahya Al Faraidi, William Sawyer
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- Published online by Cambridge University Press:
- 19 August 2008, pp. 486-490
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Between 1983 and 1995, 546 Blalock-Taussig shunt procedures were performed in 472 patients: 128 (23.0%) were classical shunts, 90 of them on the same side as to the aortic arch, and 418 (77.0%) were modified shunts, 182 on the same side of the arch. At the time of surgery, 78 patients were aged below one week, 270 from one week to 12 months, and 198 patients were over one year of age. The mean pre-operative arterial saturation (71.7%±16.5%) was significantly increased to 83%±17.9% imme-diately after the procedure (p=0.017). The overall hospital mortality rate was 2.9% (16/546), with rates of 2.3% (3/128) for the classical, and 3.1% (13/418) for the modified shunts (p= not significant). The rate was significantly higher, however, for classical shunts when the pulmonary arterial diameter was less than 4 mm (15.4% versus zero; p=0.047), though this relationship was reversed for modified shunts (zero versus 3.6%; p=0.338). Early mortality was significantly influenced by the age at surgery, 5/78 (6.4%) in patients aged below 1 week, 3.7% between 1 week and 1 year, and 0.5% over 1 year (p=0.019). Early mortality was also significantly increased in patients weighing 3kg or less, 8/156 (5.1%), versus 3/303 (1.0%), p=0.037. Overall, 51 shunts failed (9–3%), 10 early and 41 late. Early failure was significantly increased in patients weighing 3kg or less, 8/156 (5.1%) versus 3/303 (1.0%), p=0.016. The overall early failure rate was 1.4% (3/215) when heparin was administered intra-operatively and for 48 hours postoperatively, in contrast to an early failure rate of 3.4% (7/203) when heparin was not used (p = 0.294). Overall rates of failure during follow-up were 9.1% (17/188) in heparinized patients versus!3.6% (24/177), (p=0.173) in non-heparinized patients. Failure of classical shunts was 10.2% (13/128), compared with 6.7% (28/418) for modified shunts (p=0.195). Failure was more common overall if the pulmonary arterial diameter was less than 4 mm, 14.7% (9/61), as opposed to 8.7% (26/300) when the diameter was 4 mm or greater, (p=0.144). Administration of aspirin during follow-up after the modified shunt procedure reduced failure from 11 % (18/163) to 6.7% (10/150), p=0.176. Classical or modified Blalock-Taussig shunts, either on the same side or opposite to the aortic arch, can be performed on patients of any age with minimum postoperative complications and low operative mortality. The use of intra- and post-operative heparin appears to reduce the overall rate of failure, and the administration of aspirin during follow-up appears to reduce failure of modified Blalock-Taussig shunts.
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Morbidity and mortality after surgery for congenital cardiac disease in the infant born with low weight
- Anne M. Ades MD, Troy E. Dominguez MD, Susan C. Nicolson MD, James W. Gaynor MD, Thomas L. Spray MD, Gil Wernovsky MD, Sarah Tabbutt, MD, PhD
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- Published online by Cambridge University Press:
- 18 December 2009, pp. 8-17
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Objective
Low weight at birth is a risk factor for increased mortality in infants undergoing surgery for congenitally malformed hearts. There has been a trend towards performing surgery in patients early, and for amenable lesions, in a single stage rather than following initial palliative procedures. Our goal was to report on the current incidences of morbidities and mortality in infants born with low weight and undergoing surgery for congenital cardiac disease.
MethodsWe made a retrospective review of the data from patients meeting our criterions for entry from July, 2000, through July, 2004. The criterions for inclusion were weight at birth less than or equal to 2500 grams, and congenital cardiac malformations requiring surgery during the initial hospitalization. A criterion for exclusion was isolated persistent patency of the arterial duct. We assessed preoperative, intraoperative, and postoperative variables.
ResultsWe found a total of 105 patients meeting the criterions for inclusion. The median weight at birth was 2130 grams, and median gestational age was 36 weeks. The most common morbidity identified was infections of the blood stream. Infections, and chronic lung disease, were associated with increased length of stay. Survival overall was 76%. Patients with hypoplastic left heart syndrome, or a variant thereof, had the lowest survival, of 62%. The needs for cardiopulmonary resuscitation, or extracorporeal membrane oxygenation, post-operatively were the only factors identified as independent risk factors for mortality.
ConclusionPatients undergoing surgery during infancy for congenital cardiac disease who are born with low weight have a higher mortality and morbidity than those born with normal weight.
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The clinical anatomy of tetralogy of Fallot
- Robert H. Anderson, Paul M. Weinberg
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- 08 September 2005, pp. 38-47
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It is now well over one hundred years since Arthur Louis Etienne Fallot showed that four discrete morphologic abnormalities co-existed in the majority of patients he had autopsied with “la maladie bleu”,1 or cyanosis as we now describe it. The lesions he identified were an interventricular communication, subpulmonary stenosis, biventricular origin of the aortic valve, and right ventricular hypertrophy. We now know that the combination of these anomalies had been recognised long before Fallot's epochal description. Indeed, it is Neils Stensen, the Danish monk who also described the parotid duct, who is usually acknowledged as being the first to describe the entity that we now call tetralogy of Fallot.2 Cases were certainly described by John Hunter,2 whilst with the benefit of hindsight, we can see an unequivocal example illustrated by the Baron von Rokitansky3 in his ground-breaking atlas (Fig. 1).
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The significance of ventriculo-coronary arterial connections in the setting of pulmonary atresia with an intact ventricular septum
- Robert M. Freedom, Robert H. Anderson, Don Perrin
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- 16 September 2005, pp. 447-468
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Thoracic sequels after thoracotomies in children with congenital cardiac disease
- Serpil Bal, Huda Elshershari, Reyhan Çeliker, Alpay Çeliker
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- 24 May 2005, pp. 264-267
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The standard surgical approach for closed heart procedures in small infants and children is to use a posterolateral thoracotomy incision, which results in the division of the latissimus dorsi and serratus anterior muscles. The aim of our study was to determine the frequency and type of musculoskeletal deformities in children undergoing surgery with this approach for congenital cardiac disease.
We included 49 children, 28 boys and 21 girls, in the study. Their mean age was 10.2 ± 4.8 years, the mean age at the time of surgery was 3.8 ± 4.0 years, and they were evaluated at an average of 6 years after the thoracotomy. Of the patients, 94% had various musculoskeletal deformities. Scoliosis was observed in 15 patients (31%) but only in two patients did the curves exceed 25 degrees. Of these patients, three-fifths had aortic coarctation. Elevation of the shoulder was seen in 61%, winged scapula in 77%; while 14% had asymmetry of the thoracic wall due to the atrophy of the serratus anterior muscle. Deformity of the thoracic cage was observed in 18%; and 63% had asymmetry of the nipples.
Thus, we found that musculoskeletal deformities are frequent after thoracotomies in children with congenital cardiac disease. Patients who have undergone such procedures for cardiac or noncardiac surgery should be followed until their skeletal maturation is complete. Techniques sparing the serratus anterior and latissimus dorsi muscles should be preferred. These adverse effects of thoracotomy may be another reason for using interventional procedures in these cases.
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Are children with congenital cardiac malformations at increased risk of deficits in social cognition?
- David C. Bellinger
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- 20 December 2007, pp. 3-9
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In this review, I summarize observations suggesting that social cognition deficits are an important element of the neurodevelopmental morbidities of children with congenital cardiac malformations. Referred to as “theory of mind” deficits, they pertain to the ability to “read” other people, that is, to infer their internal states and thus to interpret their actions appropriately. I also hypothesize that children with such congenital cardiac malformations have difficulty identifying and describing their own internal states, which is referred to as alexithymia. These hypotheses are based on data collected as part of a prospective study of the neurologic outcomes of children with concordant atrioventricular and discordant ventriculo-arterial connections, or transposition. On a variety of tasks that assess pragmatic language and discourse skills, such as elicited personal narratives, free play with a parent, and written narratives, the children consistently performed below age-expected levels and in ways that suggested the presence of “theory of mind” deficits. Their narratives lacked coherence, failed to include critical information required by a listener, and made infrequent reference to internal states. In ongoing evaluations of the children as adolescents, we are testing these hypotheses more systematically by administering several instruments specifically developed to assess “theory of mind” and alexithymia.
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Motor development in children with congenital cardiac diseases compared to their healthy peers
- Birna Bjarnason-Wehrens, Sigrid Dordel, Sabine Schickendantz, Constanze Krumm, Daniel Bott, Narayanswami Sreeram, Konrad Brockmeier
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- Published online by Cambridge University Press:
- 01 October 2007, pp. 487-498
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Their perceptual and motor experiences determine the physical and motor development of children, and impact also on their emotional, psychosocial, and cognitive development. Our aim, therefore, was to evaluate motor development in children with congenitally malformed hearts compared to their healthy peers.
We compared 194 children, with a mean age of 10.0 years, and standard deviation of 2.7 years, representing the entire spectrum of congenital cardiac disease, to a control group of 455 healthy children, having a mean age 9.6 years, with standard deviation of 2.17 years. The bodily coordination test for children was used to examine motor development.
Of the children with congenitally malformed hearts, 26.8% showed moderate, and 31.9% had severe disturbances of motor development, compared to 16.5% and 5.5% of the control group, the p-value for these differences being less than 0.001. The mean motor quotient adjusted for age and gender was lower in the children with congenitally malformed hearts than in their healthy peers, at 79.6, with standard deviation of 18.9 as opposed to 96.6, with standard deviation of 15, this difference having a p-value of less than 0.001. Depending on the presence, and/or the degree, of residual sequels, the children with congenitally malformed hearts were divided into two subgroups, with either no or mild residual sequels, or with significant sequels. The mean motor quotient was lower in those with significant residual sequels, at 75, with standard deviation of 19.3, as opposed to 83, with standard deviation of 17.9, the p-value for this difference being less than 0.01. In both subgroups, the mean motor quotient was lower, with a p-value of less than 0.01, than in the control group.
Our findings show that children with congenitally malformed hearts have deficits in their motor development, these being found in the presence of no or mild sequels, as well as with significant residual sequels. Parental overprotection may contribute to these findings.
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Associations between knowledge of disease, depression and anxiety, social support, sense of coherence and optimism with health-related quality of life in an ambulatory sample of adolescents with heart disease*
- QiFeng Wang, Margaret Hay, David Clarke, Samuel Menahem
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- Published online by Cambridge University Press:
- 12 February 2013, pp. 126-133
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Introduction
Advances in overall management have led to an increasing number of adolescents with congenital heart disease reaching adulthood. This study aimed to evaluate the health-related quality of life in adolescents with heart disease, and examine its relationship with the adolescents’ knowledge and understanding of their congenital heart disease, its severity, and its relationship to the degree of anxiety and depression, feeling of optimism and sense of coherence experienced by the adolescents together with their social support.
Methods and resultsAdolescents with heart disease were recruited from an ambulatory setting at a tertiary centre. Patients completed self-report questionnaires including the Paediatric Quality of Life Inventory 3.0-Cardiac Module, a questionnaire assessing the adolescents’ knowledge of their cardiac condition, the Hospital Anxiety and Depression Scale, Multidimensional Scale of Perceived Social Support, Life Orientation Test-Revised, and Sense of Coherence-13, supplemented by clinical information provided by the attending cardiologists. A total of 114 patients aged 12–20 years were recruited over 15 months. In all, 98% of patients were in New York Heart Association class I. Their health-related quality of life was found to positively correlate with a low level of anxiety and depression (Pearson correlation, r = −0.57, p < 0.001), a good knowledge of their cardiac condition (r = 0.31, p < 0.01), feelings of optimism (r = 0.39, p < 0.001), adequate social support (r = 0.27, p < 0.01), and a strong sense of coherence (r = 0.24, p < 0.01).
ConclusionsAdolescents’ knowledge and understanding of their cardiac abnormality together with an improved sense of well-being had a positive influence on their health-related quality of life.
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Neurodevelopmental evaluation strategies for children with congenital heart disease aged birth through 5 years: recommendations from the cardiac neurodevelopmental outcome collaborative
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- Janice Ware, Jennifer L. Butcher, Beatrice Latal, Anjali Sadhwani, Caitlin K. Rollins, Cheryl L. Brosig Soto, Samantha C. Butler, Patricia B. Eiler-Sims, Catherine V. Ullman Shade, Gil Wernovsky
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- Published online by Cambridge University Press:
- 04 November 2020, pp. 1609-1622
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This paper provides specific guidelines for the neurodevelopmental evaluation of children aged birth through 5 years with complex congenital heart disease. There is wide recognition that children with congenital heart disease are at high risk for neurodevelopmental impairments that are first apparent in infancy and often persist as children mature. Impairments among children with complex congenital heart disease cross developmental domains and affect multiple functional abilities. The guidelines provided are derived from the substantial body of research generated over the past 30 years describing the characteristic developmental profiles and the long-term trajectories of children surviving with complex congenital heart conditions. The content and the timing of the guidelines are consistent with the 2012 American Heart Association and the American Academy of Pediatrics scientific statement documenting the need for ongoing developmental monitoring and assessment from infancy through adolescence. The specific guidelines offered in this article were developed by a multidisciplinary clinical research team affiliated with the Cardiac Neurodevelopmental Outcome Collaborative, a not-for-profit organisation established to determine and implement best neurodevelopmental practices for children with congenital heart disease. The guidelines are designed for use in clinical and research applications and offer an abbreviated core protocol and an extended version that expands the scope of the evaluation. The guidelines emphasise the value of early risk identification, use of evidence-based assessment instruments, consideration of family and cultural preferences, and the importance of providing multidimensional community-based services to remediate risk.
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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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- 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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Sequential segmental analysis - description and categorization for the millennium
- Robert H. Anderson, Siew Yen Ho
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- 19 August 2008, pp. 98-116
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The concept of sequential analysis of congenitally malformed hearts is now widely accepted, although some aspects remain controversial. In this review, we analyse the evolution of the scgmental approach, showing how our own concepts of analysis have chaanged little since our initial description, heart dicrates the presence of the atrial, ventriclar, and arterial segments, Analysis depends upon recognizing the morphologic differences of the components within each segment, and then how these parts are joined together across the atrioventicular and ventriculo-arterial Junctions. The distinction of chambers and arterial trunks follows the principle of the morphologic method, namely that scructures be identified on the basis of their own intrinsic morphology, and not according to other features which themselves might be variable. Differentiation of junctional arrangements as opposed to segmental stucture resolves ongoing controversics such as the nature of “univentricular hearts”.Analysis in the fashion proposed shows that there is nothing intrinsically complicated with malformed hearts.