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Cardiovascular magnetic resonance assessment of adults late after an atrial redirection operation for transposition is demanding and time consuming. We hypothesised that the relatively fast and standardised 3-dimensional time-resolved contrast-enhanced magnetic resonance angiography, or dynamic angiography, would be valuable in the periodic follow-up of these patients.
We investigated prospectively 36 adults with transposition using dynamic angiography, comparing our results against a comprehensive but non-contrast cardiovascular magnetic resonance protocol. We acquired 6 dynamic angiographic datasets after injection of contrast. The primary aim was to detect significant obstruction of the pathways for venous flow.
In 4 patients (11%), we found evidence of moderate-to-severe, and thus clinically important, obstruction of systemic venous channels on standard cardiovascular magnetic resonance. All these patients were correctly identified by dynamic angiography. In 4 additional patients, we found mild and haemodynamically insignificant obstructions in the systemic venous channels. Of the 8 (22%) patients with any obstruction, 6 were detected by angiography. There were no false positives reported, giving sensitivity of 75% and specificity of 100%, a positive predictive value of 100%, and negative predictive value of 93%. In 1 patient, there was a moderate obstruction of the pulmonary venous compartment which was not readily seen by dynamic angiography.
3-dimensional dynamic angiography is a useful method for detecting anatomically moderate-to-severe, but not mild, obstructions in the systemic venous channels following Mustard repair for transposition. This technique can be used as a single imaging method and/or as complimentary to standard two dimensional cardiovascular magnetic resonance techniques for detection of clinically important obstructions in the systemic venous channels.
To investigate QT dispersion in the surface electrocardiogram of children with rheumatic carditis.
QT dispersion was quantitatively evaluated in 33 children with acute rheumatic carditis. As a control group, we studied 33 healthy children free of any disease. The children were eligible for participation if the following criteria were met: diagnosis of acute rheumatic fever based on the revised Jone’s criteria and suffering from their first attack of carditis. The echo Doppler cardiogram was performed in all children, within 48–72 hours of hospitalisation.
Patients with carditis had a greater QT dispersion than the control group. When we analyse the QT dispersion according to the severity of the carditis, we observed that the dispersion tended to be greater in those with more severe valvar lesion. The sensitivity and specificity of the measurements of the QT dispersion in predicting acute carditis were estimated by using receiver operating characteristic curves. A QT dispersion greater than 40 milliseconds had a sensitivity of 63.6% and a specificity of 93.9% in predicting acute rheumatic carditis.
The lengthening of QT dispersion may reflect on cardiac involvement in rheumatic fever and be a new important parameter in the diagnosis and therapeutic decision for rheumatic carditis.
Data assessing the effect of altitude on Fontan haemodynamics are limited to experimental models and case reports. Both suggest a detrimental impact. This study describes exercise performance in patients with Fontan circulation and matched controls at a low altitude versus at sea level. We sought to assess the impact of increasing altitude on functional capacity in patients with Fontan palliation.
A retrospective review of 22 patients at low altitude (1602 metres) and 119 patients at sea level with Fontan circulation, as well as age-, gender-, and altitude-matched controls, underwent maximal cardiopulmonary exercise testing. Linear regression models were created to determine the influence of altitude on differences in exercise variables between Fontan patients and their matched controls.
Peak oxygen consumption was 28.4 millilitres per kilogram per minute (72% predicted) for the sea-level cohort and 24.2 millilitres per kilogram per minute (63% predicted) for the moderate altitude cohort. The matched case–control differences for patients at moderate altitude were greater for peak oxygen consumption (−29% against −13%, p = 0.04), anaerobic threshold (−36% against −5%, p = 0.001), and oxygen pulse (−35% against −18%, p = 0.007) when compared with patients living at sea level. When compared to institution-matched controls, the same parameters fell by 3%, 8.9%, and 4.2%, respectively, for each increase of 1000 feet in residential altitude (p = 0.03, p = 0.001, and p = 0.05, respectively).
Patients with Fontan circulation at a higher altitude have impairment in aerobic capacity when compared with patients at sea level. Reduction in exercise capacity is associated with a reduction in stroke volume, likely related to increased pulmonary vascular resistance.
It is still uncertain if cardiac natriuretic peptides are useful biomarkers in paediatric cardiology. In this review we identify four clinical scenarios in paediatric cardiology, where clinical decision-making can be difficult, and where we feel the paediatric cardiologists need additional diagnostic tools. Natriuretic peptide measurements could be that extra tool. We discuss and suggest N-terminal pro-B-type natriuretic peptide and B-type natriuretic peptide reference intervals for children without cardiovascular disease and cut-off points for the four specific paediatric heart conditions. We conclude that in premature neonates with persistent arterial ducts; in teenagers with tetralogy of Fallot and pulmonary regurgitation; and in children with heart transplants and potential allograft rejection cardiac peptides can provide the clinician with additional information, but in children with atrial septal defects the peptides are not helpful in guiding treatment or follow-up.
Endocardial fibroelastosis is not a disease but a reaction of the endocardium. I review the history of the term with emphasis on the gradual understanding of the many causes of this reaction. I include a comprehensive list of diseases or other cardiac stresses that authors have reported in association, and I try to explain the mechanism of the reaction. Although endocardial fibroelastosis is rare today, I issue a warning of a possible epidemic recrudescence of some of the associated diseases. My hope is for nosologic purity, therefore that outworn but surviving concepts will be firmly rejected.
In patients with cyanotic congenital heart disease, chronic hypoxaemia leads to important changes in blood vessel function and structure. Some of these alterations are maladaptive and probably contribute to impaired cardiopulmonary performance and an increased incidence of thrombotic and embolic events. Recent evidence suggests that deranged endothelial function, a sequel of chronic cyanosis, could be an important factor in the pathogenesis of cyanosis-associated cardiovascular risk. In this article, we discuss the physiological and mechanical consequences of compensatory erythrocytosis and possible pathophysiological mechanisms of vascular dysfunction in chronic cyanosis.
Abnormalities of the coronary arterial circulation represent a broad spectrum of anomalies. Some of these coronary arterial anomalies are of little clinical significance and are generally discovered either as incidental findings at autopsy or at the time of diagnostic coronary angiography in patients with atherosclerotic ischaemic cardiac disease. Some of these coronary arterial anomalies, however, are associated with a markedly abnormal natural history that may include significant cardiac morbidity very early in infancy, or increased risk of cardiac sudden death in childhood or adolescence. It is therefore important that we have a system for the naming and classification of these anomalies, to stratify them according to the associated risk and to develop rational approaches to evaluation and management. To apply any system of nomenclature reliably and responsibly, it is essential that each term be associated with a definition that is known and accepted by users of terminology. Such definitions are included in the specifications of both the Society of Thoracic Surgeons Congenital Heart Surgery Database and the European Association for Cardio-Thoracic Surgery Congenital Heart Surgery Database. These databases use the Diagnostic Short List of the version of the International Paediatric and Congenital Cardiac Code derived from 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. This article will provide the nomenclature and definitions used by these databases for anomalies of the coronary arteries.
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.
We 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.
We 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.
Patients 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.
Although most Kawasaki disease with giant coronary aneurysms is asymptomatic, conventional investigations might not identify previous lesions, or all Kawasaki disease with giant aneurysms at risk of future myocardial lesions. We evaluated the long-term histopathology of the myocardium, especially of intramural small vessels in asymptomatic Kawasaki disease with giant aneurysms.
The initial study comprised 16 consecutive Kawasaki patients – male-to-female ratio was 12:4 – aged from 2 to 12 years, and in the subsequent study, the same patients were aged from 4.9 to 16 years. Endomyocardial biopsies were histopathologically evaluated. Microangiopathies, mitochondrial abnormalities, and loss or disarray of myofibrils were compared by electron microscopy.
The incidence of histopathological abnormalities such as degeneration, hypertrophy, and inflammatory cell infiltration was quite high in the initial study, and inflammatory cell infiltration, interstitial fibrosis, and disarray were very noticeable at follow-up biopsies. The area of fibrous tissue was significantly higher in patients administered with intravenous immunoglobulin at follow-up biopsies. Electron microscopy showed microangiopathies including microthrombi within intramural small vessels in some patients at follow-up biopsies. The sites of the coronary aneurysms did not seem to have an impact on the biopsy findings, suggesting that the underlying pathophysiology is related to the original disease process.
Whether the abnormalities were due to direct myocardial injury, chronic ischaemia, repeated small-vessel thrombosis, or other problems associated only with biopsies, is difficult to determine. However, this subgroup had residual abnormal lesions in the myocardium. Follow-up should be more aggressive in this group of patients to identify myocardial damage that could be asymptomatic.
The purpose of this study was to evaluate the utilisation of a web-based multimedia patient-accessible electronic health record, for patients with congenital cardiac disease.
Patients and methods
This was a prospective analysis of patients undergoing congenital cardiac surgery at a single institution from 1 September, 2006 to 1 February, 2009. After meetings with hospital administration, physicians, nurses, and patients, we configured a subset of the cardiac program’s web-based clinical electronic health record for patient and family access. The Electronic Health Record continuously measured frequency and time of logins, logins during and between hospitalisations, and page views by type (imaging versus textual data).
Of the first 270 patients offered access to the system, 252 became users (93% adoption rate). System uptime was 99.9%, and no security breaches were reported. Users accessed the system more often while the patients were in hospital (67% of total logins) than after discharge (33% of total logins). The maximum number of logins by a family was 440, and the minimum was 1. The average number of logins per family was 25. Imaging data were viewed significantly more frequently than textual data (p ⩽0.001). A total of 12 patients died during the study period and 11 members of their families continued to access their Electronic Health Records after the date of death.
A web-based Patient Accessible Electronic Health Record was designed for patients with congenital cardiac disease. The adoption rate was high, and utilisation patterns suggest that the Electronic Health Record could become a useful tool for health information exchange.
Few paediatric cardiologists know of Maude Abbott. Yet before Helen Taussig, no one contributed more to founding the speciality than Maude Abbott. She achieved international fame as the early 20th century expert on cardiac malformations. We summarise here her life and contributions, indicating how she is more than justified in being inducted to the Hall of Fame.
With the ever-increasing sophistication of the Internet, and the wealth of information that can now be mined through this source, it may be thought that the age of the book has finished, and hence there is no further need for reviews of newly published books. There is much to be said, nonetheless, for the greater facility of acquiring information from the pages of a book compared to the computer screen. In this review, therefore, I discuss the advantages of obtaining information from the printed page when presented in such splendid form as shown by new books devoted, on the one hand, to the clinical anatomy of the coronary arteries, and on the other hand, to paediatric congenital cardiac surgery. At the same time, I also attempt to provide some insights into the reasons why the authors and editors produced these excellent volumes, which deserve to be on the bookshelves of all those concerned with the diagnosis and treatment of children with congenitally malformed hearts.