bluuebunny/crossref_metadata_2025 huggingface dataset:
Pericardial effusions in infants and children: injection of echo contrast medium enhances the safety of echocardiographically-guided pericardiocentesis
Authors: Mühler Eberhard Georg , Engelhardt Wolfgang , Bernuth Götz von
Published: August 2008

Pericardiocentesis is usually an easy and uncomplicated procedure when guided by cross-sectional echocardiography, but an abnormal intracardiac or extrapericardial position of the puncture system can occur, especially in children. Injection of echo contrast medium through the puncture needle is a very sensitive, quick, easy and harmless procedure which can be performed at the bedside in all cases in which doubt remains concerning the location of the needle. Prompt enhancement of signal intensity of the peri-cardial fluid validates the correct intrapericardial position, and permits visualization of the tip of the needle. Absence of echodense formations excludes an intrapericardial position. We recommend the use of echo contrast medium during pericardiocentesis whenever blood is aspirated through the needle, or if there is any doubt concerning its location.
Coarctation of persistent right fifth aortic arch and pulmonary sequestration
Authors: Tomita Hideshi , Fuse Shigeto , Chiba Shunzo
Published: August 2008

A 41-day-old boy was transferred to our department with severe congestive heart failure. Digital subtraction counter current aortography, and antegrade aortography, revealed coarctation of a persistent right fifth aortic arch, stenosis of the origin of an aberrant left subclavian artery, and sequestration of the lower lobe of the right lung.

As his heart failure seemed to be caused not only by pressure overload to the left ventricle following coarctation, but also by the volume load associated with the shunting effect of the pulmonary sequestration, we performed transcatheter balloon dilation of the coarctation and the origin of the aberrant subclavian artery, together with embolization of the aberrant pulmonary artery. His heart failure responded dramatically to these procedures.
The real fate of pulmonary arteries after bidirectional superior cavopulmonary anastomosis: is there a need for concern?
Authors: Slavik Zdenek , Franklin Rodney C. G. , Radley-Smith Rosemary
Published: August 2008

Controversy remains about the growth of the pulmonary arteries following a bidirectional superior cavopulmonary anastomosis in children with complex cyanotic congenital cardiac malformations. This is partially due to the morphological heterogeneity of the patients, and partially due to methodological differences in series published so far. It is further complicated by the variable use, in different centres, of additional sources of pulmonary blood flow. We believe that the fate of these arteries preoperatively is significantly influenced by the amount of pulmonary blood flow and the initial size of the arteries. Separate assessment of the pulmonary arterial development postoperatively is recommended for those who, initially, had relatively small as opposed to larger than normal pulmonary arteries. Measurement of the diameters of both pulmonary arteries just prior to their first point of branching, together with the use of Z-score evaluation rather than the Nakata index, is discussed. It remains to be established whether, over time, the bidirectional cavopulmonary anastomosis is effective in developing adequately the pulmonary arteries in preparation for an ultimate total cavopulmonary connection, or even as isolated long-term palliation. A prospective, multi-institutional study involving sequential non-invasive assessment of pulmonary arterial development (using, for example, magnetic resonance imaging) is required to solve the outstanding problems.
Balloon dilation of the right ventricular outflow tract in tetralogy of Fallot: a palliative procedure
Authors: Arab Sameh M. , Kholeif Abdel-Fattah E. , Zaher Salah R. , Abdel-Mohsen Aly M. , Kassem A. Samir , Qureshi Shakeel A.
Published: August 2008

Fifteen patients requiring palliation for tetralogy of Fallot were treated by balloon dilation because of hypercyanotic spells. The mean age at dilation was 1.9 ± 0.7 years (range 0.5 – 3), and the mean weight 9.8 ± 2.1kg (range 6.0 –13.5). Dilation of the outflow tract was combined with dilation of the left and/or right pulmonary arteries in 5 patients. Successful dilation was achieved in 12 patients (80%), but failed in 3 patients with hypoplastic pulmonary arteries. In one patient, the stenosis of the right pulmonary artery could not be dilated because of a very sharp angle at the site of the stenosis. Two of the 3 patients in whom the procedure failed died of severe cyanotic spells within 24 hours of the unsuccessful procedure. No major complications occurred during or after the procedure in the cases undergoing successful dilation. The arterial oxygen saturation increased significantly, from 71 ± 5.7% to 89 ± 3.9%, immediately after the procedure (p < 0.005). During a period of follow up of 6 ± 3.7 months (range 1 – 13), the procedure was repeated on 3 occasions, and successfully accomplished in 2 of these. In conclusion, balloon dilation is a satisfactory palliative procedure for tetralogy of Fallot in those units in which total correction is not performed under 2 to 3 years of age.
Balloon valvoplasty in infants with tetralogy of Fallot: effects on oxygen saturation and growth of the pulmonary arteries
Authors: Heusch A. , Tannous A. , Krogmann O. N. , Bourgeois M.
Published: August 2008

Balloon valvoplasty was undertaken in 27 patients with tetralogy of Fallot for first-stage palli-ation. Indications were arterial saturation of oxygen <80%, hypoxic spells and duct-dependant pulmonary perfusion. The dilation was performed following diagnostic heart catheterization. Saturations improved from 75% ± 8.5 before valvoplasty to 85% ± 8.4 after the procedure, and worsened little to 83% ± 9.6 at follow-up after 3.4 months. The pulmonary valvar orifice was hypoplastic in most patients (Z = — 3.3 ± 1.2), and did not change after the procedure. The cross-sectional area of the pulmonary arteries was diminished initially, reflected by a Nakata index of 186 ± 95 mm2/rn2. After valvoplasty, we found widening of the vessels (Nakata index 225 ± 100 mm2/m2). At follow-up no further growth of the arteries was observed (Nakata index 209 ±109 mm2/m2). The procedure was complicated by hypoxic spells in three patients which were controlled by intravenous propranolol, and deep venous thrombosis in four patients. Our data demonstrate that balloon valvoplasty is feasible for initial palliation in patients with tetralogy of Fallot. It does not, however, produce growth of the pulmonary arteries or of the pulmonary valve.
Palliative balloon valvoplasty of the pulmonary valve in tetralogy of Fallot
Authors: Massoud Ikram , Imam Adel , Mabrouk Ahmed , Boutros Nader , Kassem Ashraf , Daouod Ahmed , Abd Mahmoud , Hakem El
Published: August 2008

Balloon dilation of the pulmonary valve was performed in 54 patients with tetralogy of Fallot with severe cyanosis, high haematocrit and severe valvar pulmonary stenosis. Clinical, echocardiographic, angiographic, and haemodynamic data were analyzed before and after the procedure. After balloon dilation, the systemic oxygen saturation increased from a mean value of 66% to 85<%. The mean value of the haematocrit before dilation was 55 + 13, and decreased to 47 after dilation (p < 0.002) in 2 months follow-up. Balloon dilation increased the size of the pulmonary valvar orifice from a mean value of 9 + 5mm to 11.5 + 2mm (p < 0.005). The mean Z score of the pulmonary valves, which was -3 + 1.3 before dilation, increased to -1.1 + 1.1 immediately after the procedure (p < 0.05).

The size of the right and left pulmonary arteries increased after dilation from 9mm to 10 mm, and from 8.7 + 2.4mm to 9.8 + 2.3mm, respectively (p <0.05). The comparable mean Z scores increased from -2.8 + 1.9 SD to -1.8 + 1.4 SD, and from -2.4 + 1.9 SD to -1.5 + 1.6 SD for the right and left branches, respectively (p < 0.05).

In patients with stenosis at the bifurcation of the pulmonary trunk and hypoplasia of the left artery, successful dilation of the pulmonary valve lead to an increase of flow and improvement in size of the hypoplas-tic segment.

In conclusion, initial balloon dilation of the pulmonary valve in tetralogy of Fallot resulted in increase of the Z score for the pulmonary valve and improved antegrade pulmonary blood flow, inducing growth of the pulmonary arteries and ameliorating the anatomic and physiologic preoperative condition.
Natural course of supravalvar aortic stenosis and peripheral pulmonary arterial stenosis in Williams‘ syndrome
Authors: Kim Yang Min , Yoo Shi-Joon , Choi Jung Yun , Kim Seong Ho , Bae Eun Jung , Lee Young Tak
Published: August 2008

We investigated the catheterization and angiographic findings of 26 patients with Williams‘ syndrome to evaluate the natural course of supravalvar aortic stenosis and peripheral pulmonary arterial stenosis. The severity of the stenosis was correlated with age and body surface area in terms of the pulmonary arterial index, right ventricular systolic pressure, sinutubular ratio(ratio of measured to mean normal diameter of sinutubular junction), and systolic pressure gradient across the sinutubular junction. In patients with pulmonary arterial stenosis (n=20), right ventricular systolic pressure tended to decrease, and pulmonary arterial index increased, with increase in age and body surface area. Between the groups with and without pulmonary arterial stenosis, there was significant difference in age (mean 4.70 vs. 9.87, p = 0.019), body surface area (0.62 vs. 1.16, p = 0.002), pulmonary arterial index (152 vs. 317, p=0.002) and right ventricular systolic pressure (73.9 vs. 33.0, p=0.006). As all patients showed similar diameters at the sinutubular junction regardless of age and body size, sinutubular ratio decreased with increases in age and body surface area. The group with abnormal coronary arteries (n = 7) had smaller sinutubular ratio (0.46 vs. 0.61, p=0.021) and higher pressure gradients between the left ventricle and the aorta (67.6 vs. 42.2, p=0.023) than did the group with normal coronary arteries. Stenosis of a coronary artery, or a branch of the aortic arch, was observed only in three patients with diffuse aortic stenosis.

Our results suggest that, with time, peripheral pulmonary arterial stenosis tends to improve, and supravalvar aortic stenosis to progress. Failure of growth of the sinutubular junction might be responsible for the progression of the aortic lesion. Progression of the aortic lesion may be associated with involvement of the coronary arteries.
Continuous monitoring of cardiac output in neonates using an intra-aortic Doppler probe
Authors: Ewert P , Nagdyman N , Fischer T , Gortner L , Lange PE
Published: August 2008

Continuous monitoring of cardiac output in neonates would be of considerable benefit but, as yet, there is no practical method to achieve this aim. We have now evaluated the feasibility of using an intra-aortic Doppler probe. We introduced a pulsed Doppler probe of 0.46 mm diameter via the umbilical artery in two term and four preterm neonates. Indications in all patients for umbilical arterial catheter is always an unstable cardiopulmonary state. Body weights were between 770 and 3340 g. Velocities of blood flow in the thoracic aorta were continuously recorded to estimate cardiac output on-line for 12 h. No complications were encountered. It proved possible to derive high-quality Doppler curves. The received Doppler signal was stable but it proved sensitive to pathophysiologic changes in flow. Mean velocity of flow in the descending aorta was 16.4 cm/s (range 13.3_19.0 cm/s). We quantified flow by multiplying the mean velocity of the flow by the cross-sectional area of the descending aorta. Calculated mean flow was 135 ml/kg/min (range 111–179 ml/kg/min). These values are consistent with those measured by transcutaneous Doppler, and it should not be raised by left-to-right ductal shunts. This pilot study proved the feasibility of continuous monitoring of cardiac output. The technique should prove of great value in those infants with unstable circulatory conditions, and can be used even in infants with extremely low birth weights.
Banding of the pulmonary trunk in preparation for a Fontan operation
Authors: Sakamoto Takahiko , Harada Yorikazu , Takeuchi Takamasa , Morishima Katsumasa , Satomi Gengi , Yasukochi Satoshi , Ohta Yoshinori
Published: August 2008

Banding of the pulmonary trunk is an important surgical procedure for patients who have con genital cardiac malformations with unrestricted pulmonary flow. We propose a new concept for determining in such circumstances the most appropriate length of the band used to constrict the pulmonary trunk in preparation for a Fontan operation. We studied 14 patients undergoing banding of the pulmonary trunk and measured the following parameters: diameter of aorta, diameter of pulmonary trunk, length of pulmonary arterial band and maximum flow velocity across the banded segment. We calculated an index from our orig inal parameter based on the formula; length of band/(diameter of aorta diameter of pulmonary trunk). The diameter of aorta was 9.5 ± 1.4 mm, and that of the pulmonary trunk was 9.6 ± 2.3 mm. The length of the band was 16.5 ± 3.4 mm, giving a calculated index of 0.188 ± 0.038. The maximum flow velocity was 4.02 ± 0.46 m/s. No correlation was found between the length of the band and body weight, and also no correlation was found between the length of the band and maximum flow velocity. The calculated index had a negative correlation with the maximum velocity of flow across the band (y = -8.13x + 5.56, R = 0.74, p < 0.01). We believe that the proposed index is a useful guide in determining the length of a pulmonary band when preparing patients for a Fontan operation.
Left bundle branch block in infants with dilated cardiomyopathy conveys a poor prognosis
Authors: Cnota James F. , Samson Ricardo A.
Published: August 2008

We describe three infants < 3 months of age seen consecutively with dilated cardiomyopathy who presented initially with left bundle branch block on the surface 12-lead electrocardiogram. Each infant subsequently had a poor outcome: two died and one required heart transplantation. These results suggest that the presence of left bundle branch block on the 12-lead electrocardiogram conveys a poor prognosis in infants with dilated cardiomyopathy.
Mild or subclinical intravascular haemolysis subsequent to transcatheter occlusion of the patent arterial duct
Authors: Jamjureeruk Vachara , Kirawittaya Tawatchai , Ningsnondh Vandee
Published: August 2008

One of the important complications of transcatheter occlusion of the patent duct by insertion of either the Rashkind double umbrella or coil devices is intravascular haemolysis, particularly the severe form which occurs in 0.5–0.6% of cases. The incidence of subclinical or mild intravascular haemolysis including morphologic changes in the red cells, however, is not known, especially in the group of patients with residual shunt. We studied laboratory and haematologic changes in 37 patients subsequent to transcatheter occlusion. Their ages ranged from 2 years 2 months to 11 years 8 months (6.2 + 2.7 years), and the duration of occlusion had ranged from 1 month to 2 years 11 months (1.24 + 0.89 years). In 30 cases we had used the Rashkind double umbrella device, and nine of these patients (30%) had a residual shunt. The other seven ducts were closed with detachable coils, and five of these cases (71%) had residual shunting. There was evidence of mild or subclinical intravascular haemolysis in almost half the cases, although no patient developed acute intravascular haemolysis. Fragmented red cells, blur cells and spherocytes from blood smears were found in up to one-third of the overall series, with no differences found according to persistence of shunting. Haemosiderin was found in the urine of the group with residual shunting and in 13–23% of those with complete occlusion. Serum hypohaptoglobin was found in 9–13% of the patients with a residual shunt, and in 9–23% of those with complete occlusion. Four of the patients had haptoglo-bin levels <50 mg/dl. There were no patients with reticulocytes counts >2% in the blood smears. We conclude that subclinical or mild intravascular haemolysis, along with morphologic changes in the red blood cells, are significant findings subsequent to occlusion of the patent arterial duct with either the Rashkind double umbrella or detachable coils.
Fatal neonatal hypertrophic cardiomyopathy with severe stenosis of the right ventricular outflow tract: echocardiographic diagnosis and potential therapy
Authors: Orino Tomomi , Ito Tadahiko , Takada Goro
Published: August 2008

We discuss the clinical course of aneonate with idiopathic hypertrophic cardiomyopathy who showed rapid progression of stenosis of the right ventricular outflow tract and reduction in size of the right ventricular cavity due to thicken ing of the ventricular septum. Medical treatment proved unsuccessful. We suggest that balloon atrial septostomy combined with construction of a Blalock-Taussig shunt may be effective in view of the hemodynamics, which mimic pulmonary atresia with intact interventricular septum.
Infective endocarditis on an occluder closing an atrial septal defect
Authors: Bullock A. M. , Menahem S. , Wilkinson J. L.
Published: August 2008

Closure of atrial septal defects be means of intravenous catheterisation has been undertaken using a variety of devices as an alternative to surgical closure.1–5We describe the first case, to the best of our knowledge, of infective endocarditis complicating a successful transcatheter closure. This highlights the potential risk of this procedure, and emphasises the need for appropriate antibiotic prophylaxis until complete endothelialization of the device has occurred.
Divided left atrium in association with tricuspid atresia and discordant ventriculo-arterial connections
Authors: Dindar Aygun , Brawn William J. , De Giovanni Joseph V.
Published: August 2008

Division of the morphologically left atrium is a rare abnormality, constituting around 0.1% of congenital heart disease. It may coexist with other cardiac abnormalities, and this association may obscure its recognition. We report an uncommon association with tricuspid atresia and discordant ventriculo-arterial connections, the latter dominating the clinical picture. Accurate diagnosis was made by transthoracic cross-sectional echocardiography, and the patient was referred to surgery without need for cardiac catheterisation.
Aneurysm of the pulmonary trunk with patent arterial duct
Authors: Alva Carlos , Argüero Ruben , Valero Guillermo
Published: August 2008

We present a 14-year-old female with a massive aneurysm of the pulmonary trunk occurring secondary to bacterial endocarditis of the patent arterial duct. Echo Doppler imaging, magnetic resonance and angiography confirmed the diagnosis. On intermediate follow-up after surgical correction, the echo studies and cardiac catheterization disclosed no residual lesions. The patient is now asymptomatic.
Atrioventricular septal defect with separate right and left atrioventricular valvar orifices in a patient with foetal hydantoin syndrome
Authors: Grech Victor , Vella Cecil
Published: August 2008

The teratogenic properties of phenytoin, including cardiac malformations, have been previously documented. We report one patient with foetal hydantoin syndrome and atrioventricular septal defect with common atrioventricular junction but separate right and left atrioventricular valves, an association that has not been described, to the best of our knowledge.
Tetralogy of Fallot with non-confluent pulmonary arteries and aortopulmonary septal defect
Authors: Dipchand Anne I. , Giuffre Michael , Freedom Robert M.
Published: August 2008

Aortopulmonary septal defect and tetralogy of Fallot is a rare combination. We report a case of tetralogy of Fallot, non-confluent pulmonary arteries with the left arising from the arterial duct, and a large aortopulmonary septal defect diagnosed by echocardiogram and confirmed by cardiac catheterization.
Declining mortality from congenital heart disease related to innovations in diagnosis and treatment: a population-based study
Authors: Grech Victor , Savorja-Ventura Charles
Published: August 2008

The majority of live births with severe congenital cardiac malformations that do not undergo intervention of some form die prematurely. The population of Malta provides an ideal closed cohort from which to monitor trends. We have observed a significant declining specific mortality from congenital heart disease, and circumstantial evidence relates this decline to the introduction of new techniques used in the diagnosis and treatment of these malformations.
Cross-sectional echocardiographic demonstration of duct-related intimal tissue plaques within the pulmonary artery
Authors: Gennery A. R. , Hunter A. S.
Published: August 2008

The cross-sectional and colour flow mapping echocardiographic appearances of extension of ductal tissue within the pulmonary artery in three infants referred for echocardiographic assessment of a cardiac murmur are reported. The ductal plaques described may be common but are not necessarily of clinical significance.
An unusual tethering of the bridging leaflets in atrioventricular septal defect producing a communication from left atrium to right ventricle
Authors: Delgado C , onnin O , Barturen F
Published: August 2008

We describe a 39-year-old woman who was diagnosed as having an unusual atrioventricular septal defect with a communication from left atrium to right ventricle. A common atrioventricular junction, with partially separated right and left atrioventricular orifices, was found at transoesophageal ultrasonic examination. Both bridging leaflets were attached to the underside of the atrial septum, which was grossly malaligned relative to the ventricular septum. The shunt was exclusively from left atrium to right ventricle because of the overriding of the left atrioventricular valve, with the left component of the inferior bridging leaflet firmly fused to the ventricular septal crest.
Outcome of pregnancy in women with congenital heart disease
Authors: Schmaltz A. A. , Neudorf U. , Winkler U. H.
Published: August 2008

Improvements in diagnosis and surgical technique for correction have led to an increasing number of women with congenital heart disease reaching the child-bearing age. Pregnancy places considerable strain on the heart and circulation and necessitates marked cardiorespiratory adaptation. Today, with the exception of the Eisenmenger syndrome, there is no increased mortality associated with pregnancy in congenital heart disease. In contrast, there is still considerable morbidity, due to congestive heart failure, thromboembolic complications and disturbances of rhythm.

Fetal outcome is complicated by a high rate of spontaneous abortions (20–25%), retardation of fetal growth, and premature delivery (almost 100% in cyanotic mothers). Based on an extensive review of the literature, we discuss the specific risks in pregnancy depending on the hemodynamic situations produced by different heart defects. We also discuss the risks and advantages of different regimens for anticoagulation.

Counselling concerning contraception is frequently inadequate. The most important problems are thromboembolic complications with the use of hormonal contraception, and hyper- and dysmenorrhea in those using intrauterine devices. Finally, the genetic risks must be considered, differentiating between single gene defects and the sex of the parents suffering from congenital heart diseases.
Sudden death in an adult with a small ventricular septal defect and an aneurysmal membranous septum
Authors: Sarubbi B , Gerlis L M , Ho S Y , Somerville J
Published: August 2008

An apparently healthy man of 26 years of age suddenly died. He was known to have had a small ventricular septal defect and complete right bundle branch block from early childhood. At post-mortem examination the small ventricular septal defect was found associated with an aneurysm of the membranous septum. Histological examination showed a normal atrioventricular node and bundle, adjacent to the aneurysm. There was fibrous interruption at the commencement of the right bundle branch, which was considered the basis of the bundle branch block. It was also considered that the right bundle branch block was of the hereditary type and that this, rather than the aneurysm of the membranous septum, was responsible for the unexpected death.
Helen Brooke Taussig MD May 24, 1898 to May 21, 1986
Authors: Neill Catherine A. , Clark Edward B.
Published: August 2008

The life and accomplishments of Helen Taussig are reviewed at the centennial of her birth in 1898. Now, a little over 50 years since the first Blalock Taussig shunt in 1944, her legacy remains a model of brilliant scientific innovation and loving patient care.

“Faithful indeed is the spirit that remembers” (Emily Bronte, Remembrance)
Report of the Coding Committee of the Association for European Paediatric Cardiology
Authors: Franklin Rodney C. G. , Anderson Robert H. , Daniëls Otto , Elliott Martin , Gewillig Marc H. M. L. , Ghisla Renzo , Krogmann Otto N. , Ulmer Herbert E. , Stocker Franco P.
Published: August 2008

For many years, paediatric cardiologists and paediatric cardiac surgeons have felt the need for a common diagnostic and therapeutic coding system with which to classify patients of all ages with congenital and acquired heart disease. This desire has been highlighted by the recent, and ongoing, enquiry into the outcome of paediatric cardiac surgery at the unit in Bristol, in the United Kingdom, and the resulting obligation to provide national and international comparisons of surgical results between centres caring for these patients. In order to incorporate effective clinical governance and best practice into our speciality, a method is required to gather accurate and validated data on the diagnosis, therapy and outcome of patients with heart disease from prenatal life through to adulthood. This would facilitate comparisons between individual units which fully take into account the mix of cases involved, and thus attempt to focus on the relevant and genuine factors underlying the differing outcomes in terms of both mortality and morbidity. For this to be achieved, it is essential to have a comprehensive system of coding and classification, using mutually exclusive and unambivalent terms. The system must be both easy to use, and fulfil the needs and expectations of widely different cultures of practice. Although many centres have developed their own system of internal audit, with their own coding system, and some co-operation has taken place between centres nationally and across international boundaries within Europe by the European Congenital Heart Surgeons Federation (see below), a cohesive, and comprehensive system suitable for setting standards has yet to emerge. Historically, this has partly been due to the lack of recognition by governments of the importance of such a process, with underfunding of initiatives aimed at addressing these issues, both technologically and in terms of human resources.
The European Paediatric Cardiac Code Long List: structure and function
Authors: Franklin Rodney C. G.
Published: August 2008

The Long List is a comprehensive hierarchical system of coding and classification for the diagnosis and treatment of heart disease. Although originally aimed at the clinician or surgeon treating heart disease first appearing in infancy or childhood, it has now been enlarged to encompass abnormalities first diagnosed in fetal life, as well as heart disease first acquired during adult life. There are a total of 3,876 individual terms. For the most part, these are mutually exclusive and unambivalent, given the constraints of clinical ambiguities and differing cultures of practice. In addition, there are 564 qualifier terms. These provide additional detail to individual or multiple items, such as fine anatomical detail, the severity of a lesion, the size of an interposition shunt or conduit, or the material used to close a septal defect. Finally, there are a further 341 duplicate diagnostic or procedural terms included in the List to aid its use, as some terms may have relevance in multiple areas. For example, Hypoplastic left heart syndrome is found under congenital anomalies of the left ventricle as well as in the section dealing with the aortic valve. Thus, altogether the listing consists of 4,781 items. The System is hierarchically arranged, with a major division between diagnostic and therapeutic subhierarchies. The developmental history of the Long List has been published previously, and is detailed earlier in this supplement.
Induction of cardiac anomalies with all-trans retinoic acid in the chick embryo
Authors: Broekhuizen Monique L. A. , Wladimiroff Juriy W. , Tibboel Dick , Poelmann Robert E. , Wenink Arnold C. G. , Groot Adriana C. Gittenberger-de
Published: August 2008

To study the interaction between hemodynamics and morphology in abnormal development of the heart, it is essential to have an animal model with specific and reproducible cardiac malformations. A standardized method was developed for inducing such an anomaly in the chick embryo. For this purpose, all-trans retinoic acid, dissolved in 2% dimethylsulphoxide, was applied on the vitelline membrane of the embryo in concentrations of 1.0 μgm and 0.3 μgm. A total of 207 embryos, staged according to Hamburger and Hamilton, were treated at stages 10 (33 hours of incubation) and 15 (50–55 hours of incubation). Three control groups consisted of embryos solely treated with dimethylsulphoxide, sham operated chicks, and untreated embryos, respectively. The embryos were sacrificed on the 9th, 10th and 11th days of incubation and hearts were examined by microdissection. Additional histologic studies were undertaken in 110 cases. Embryos treated with retinoic acid at both stages of development showed a high percentage of lesions falling within the general spectrum of double outlet right ventricle. The abnormality varied from merely a rightward shift of the aorta to cases with an additional subaortic ventricular septal defect, which, in combination with the rightward positioned aorta, was classified as a double outlet right ventricle. Histologic examination showed the presence of muscular tissue between the leaflets of the aortic and mitral valves. No other types of cardiac malformations were seen. Our results, with respect to timing, resemble data from studies made of hearts after surgical ablation of neural crest at comparable stages. Both method and timing of treatment with retinoic acid are important factors for the induction of specific and reproducible cardiac anomalies. This chick model allows for further detailed study of development of double outlet right ventricle, as well as the assessment of early detectable hemodynamic changes.
Expression of myosin heavy chain in neonatal human hearts
Authors: Wessels Andy , Mijnders Tom A. , de Gier-de Vries C. , Vermeulen Jacqueline L. M. , Vir´gh Szabolcs , Lamers Wouter H. , Moorman Antoon F. M.
Published: August 2008

We have investigated the spatial distribution of the α and β isoforms of myosin heavy chain in the force-producing ventricular myocardium, the ventricular conduction system and the atrioventricular junctional region of five neonatal human hearts–three born prior to and two at term. The results can be summarized as follows: 1)the force-producing myocardium:in each heart, the β isoform was found to be the predominating isoform, β-negative cells never being observed. Considerable amounts of the α isoform were observed only in those hearts that were obtained from patients dying within the first two weeks of postnatal life. This transient expression is probably correlated with the postnatal increase in levels of circulating thyroid hormone. 2)the ventricular conduction system:in addition to the expression of the β isoform, a strong expression of the α isoform was detected in all the components of the ventricular conduction system in the two full-term hearts. In the preterm hearts, however, this strong expression was mainly restricted to the atrioventricular node and the penetrating atrioventricular bundle. The level of expression of the α isoform in the conduction system of the neonatal hearts, therefore, might reflect the state of maturity of this system at the time of birth. 3)the atrioventricular junctional region:in each heart examined, structures were detected that resembled immunohistochemically and morphologically the atrioventricular node. Although the expression of the isoforms of myosin heavy chain in the conduction system, and in these comparable structures, cannot, as yet, be interpreted with regard to function, these resemblances, coupled with the same developmental background for these structures (they all develop from the so-called “primary ring”) suggest functional affinities during this stage of cardiac development.
An anatomical study of 110 cases with deficiency of the aorticopulmonary septum with emphasis on the role of the arterial duct
Authors: Gerlis Leon M. , MacGregor Candida C. d'A. , Ho Siew Yen
Published: August 2008

To assess the prevalence and role of the arterial duct in hearts with incomplete development of the aorticopulmonary septum, 110 autopsy specimens, comprising 100 examples of common arterial trunk and 10 cases with aorticopulmonary window, were studied. In addition to intracardiac malformations, these specimens were analyzed to determine the side of the aortic arch, the presence and location of any interruption of the arch, the presence of any anomaly of origin and course of the subclavian arteries, and the status of the arterial duct. The arterial duct was present in 33 cases, absent in 63 cases and undetermined in four cases with common arterial trunk. The duct provided an essential pathway for flow in the 20 cases with interrupted aortic arch, and in two cases with interruption of the proximal portion of the left pulmonary artery. The presence of the duct in 11 cases, in functional terms, was not essential. In the 10 hearts with aorticopulmonary window, the duct was present in eight. It was an obligatory part of the circulatory pathways in three cases where the aortic arch was interrupted. The prevalence of non-obligatory ducts was 71% in hearts with aorticopulmonary window compared to 15% in hearts with common arterial trunk. The prevalence of the duct in cases with aorticopulmonary window suggests this lesion is a later developmental defect. The functional role of an arterial duct in these hearts should be properly assessed in the clinical situation.
Use in children of an additional umbrella for transcatheter occlusion of residual patency of the arterial duct following initial insertion of an umbrella device
Authors: Yousef Saad Al , Khan Ali , Mullins Charles E. , Sawyer William
Published: August 2008

After an initial attempt to close arterial ducts by the transcatheter insertion of an umbrella device had left significant residual patency, further attempts at closure were made in 14 patients by the insertion of an additional umbrella device. The ages of the patients ranged from four to 11 years. Overall, 17 attempts were made to insert additional devices at intervals of between six to 26 months after the insertion of the first umbrella. Successful insertion of a device was achieved in two patients only at a third attempt. One patient required the insertion of a third umbrella to achieve complete occlusion of the patent duct. Complete closure of the residual patency was achieved in 13 of the 14 patients. The remaining patient has been scheduled for insertion of a third umbrella.
Anomalous mitral arcade leading to congenital mitral insufficiency with an associated anomaly of the tricuspid valve
Authors: Mandke Jyoti V. , Dharnidharka Vikas R. , Sanzgiri Vasundhara P.
Published: August 2008

A 21-day-old girl presented with congestive heart failure of acute onset and Doppler evidence of mitral regurgitation, which subsequently proved fatal. Necropsy revealed an anomalous arcade lesion of the mitral valve. The tendinous cords of the tricuspid valve were also abnormal, a finding, to the best of our knowledge, not reported before.
Extracorporeal membrane oxygenation and cyanotic congenital heart disease
Authors: Pearson Gale A. , Firmin Richard K. , Leanage Ranjit
Published: August 2008

Worldwide figures suggest that two percent of appropriate referrals for neonatal extracorporeal membrane oxygenation turn out to have previously covert congenital heart disease. This is despite the fact that expert cardiological evaluation is routine prior to cannulation. The experience in the United Kingdom includes such a case which is reported here. The implications for the role of pediatric cardiologists in such a service are considered.
Morphologic and functional analysis of myocardial status in pulmonary atresia with intact ventricular septum—an angiographic, histologic and morphometric study
Authors: Daliento Luciano , Scognamiglio Roldano , Thiene Gaetano , Hegerty Anita , Ho Siew Yen , Caneve Francesca , Anderson Robert H.
Published: August 2008

We studied 11 patients with pulmonary atresia and intact ventricular septum who died at ages ranging from one day to three years. All but two neonates died after surgery. Pre-surgical catheterization with left and right cineventriculography was performed in nine patients, and quantitative parameters were calculated. By histologic examination of transverse transmural sections, the incidence and extent of myocardial ischemia or infarction were determined, as well as the percentage area per field occupied by vessels, myocytes orientated longitudinally or transversely, interstitial space, and disarrayed fibers. Comparable sections were obtained from six normal neonates to constitute a control group. Myocardial ischemia or infarction were frequent complications. Morphometric analysis demonstrated a significant difference between the control group and the patients with regard to the percentage of myocytes (longitudinal and transverse), the percentage of area occupied by myocardial disarray, and the presence of fibrosis. The highest values for the proportion of disarray were seen in those patients with small right ventricles. The percentage of myocytes varied, but the highest degree of disarray always correlated with the lowest percentage of myocytes. The highest percentage value of left ventricular disarray was associated with the lowest ratio of left ventricular mass to end diastolic volume (inadequate hypertrophy). The lowest indices of contractility and left ventricular pump function were associated with myocytes less than 55% of normal. A linear correlation was found between the percentage area of fibrosis and the ratio of systolic pressure to end systolic volume in the left ventricle, arid between fibrosis and ejection fraction. Values for fibrosis exceeding 15% were associated with decreased functional indices. Disarray, therefore, seems to express a primary inability of myocardium to respond to anomalous overload with adequate hypertrophy and/or hyperplasia.
Pulmonary atresia with intact ventricular septum in the fetus
Authors: Allan Lindsey D. , Cook Andrew
Published: August 2008

The objective of this study was to review the echocardiographic findings and autopsy correlates of pulmonary atresia with an intact ventricular septum when diagnosed during fetal life. All cases with this lesion (nearly 60) seen in the Perinatal Cardiology unit of Guy's Hospital since 1983 are part of this study. More than half of the cases were detected within the last two years, and all bar three were referred because of suspicion of a cardiac malformation detected on routine obstetric scanning. The cases were divided into two distinct groups. On the one hand were those with dilatation of the chambers of the right heart. These had a uniformly poor prognosis. Severe regurgitation of the tricuspid valve was a characteristic feature. On the other hand were the cases with cavitary hypoplasia due to mural overgrowth. Direct measurements of pressure in one such case revealed suprasystemic values in the right ventricle. Although contemplated, fetal intervention proved impossible. Autopsy revealed cases with either valvar or infundibular atresia. In most cases diagnosed prior to 24 weeks of gestation, mothers opted for termination of pregnancy. All cases with dilatation of the right ventricle died. Of pregnancies which continued, only 30% of children are still alive, all but one being less than two years of age at present. The use of cross-sectional and Doppler echocardiography permits the accurate diagnosis of pulmonary atresia with an intact ventricular septum during fetal life. Autopsied cases showed strong correlation with the echocardiographic findings. The poor prognosis in most cases warrants consideration in the future of intervention during fetal life.
Determinants for outcome of hypoplastic right ventricle with duct-dependent pulmonary blood flow presenting in the neonatal period
Authors: Rigby Michael L. , Salgado Micelia , Silva Celia
Published: August 2008

A retrospective study was undertaken of patients with hypoplastic right ventricles, either with pulmonary atresia and intact ventricular septum or critical pulmonary valvar stenosis, and duct-dependent pulmonary blood flow who were investigated at the Royal Brompton Hospital between January 1976 and December 1990. The diagnosis was made on the basis of at least one diagnostic method (cross-sectional echocardiography, cardiac catheterization and angiography, or autopsy). Of the patients, 56 (82%) were found to have an imperforate pulmonary valve or infundibulum (pulmonary atresia), while 12(18%) had critical pulmonary valvar stenosis. The ratio of the diameters of the tricuspid and mitral valvar orifices was measured angiographically during diastole, and the right ventricle was analyzed according to the presence or overgrowth of the inlet, apical trabecular and outlet components. A correlation was made between the severity of the disease and the outcome. The overall mortality was 53% when those not undergoing any surgery were excluded, and significant differences were found between the group dying and those who survived. The incremental risk factors for death were a ratio between the diameter of the tricuspid and mitral valves of less than 0.6; the presence of fistulous communications with the coronary arteries; and obliteration of the apical trabecular component of the right ventricle. There was no significant difference between the group with pulmonary valvar atresia and the group with critical stenosis of the pulmonary valve.
Antegrade diastolic pulmonary arterial flow as a marker of right ventricular restriction after complete repair of pulmonary atresia with intact septum and critical pulmonary valvar stenosis
Authors: Redington Andrew N. , Penny Daniel , Rigby Michael L. , Hayes Alison
Published: August 2008

In order to examine right ventricular diastolic function after complete correction of pulmonary atresia with intact ventricular septum and critical pulmonary valvar stenosis, we undertook a prospective analysis of seven patients who had previously undergone successful repair. Comparison was with age and sex-matched controls. The relationship between antegrade pulmonary arterial diastolic flow and other indices of right ventricular dysfunction were compared. The ratio of the velocity of early rapid filling to that of atrial systolic flow was no different from normal, but the deceleration of early rapid filling showed significant shortening with inspiration in patients, but not in control subjects. All patients, but none of the controls, demonstrated significant antegrade diastolic pulmonary arterial flow during late diastole. Despite “normal” ratios ofvelocity of early rapid filling to systolic atrial flow, patients after complete correction of pulmonary atresia with intact ventricular septum and critical pulmonary stenosis have abnormal right ventricular diastolic function typical of a restrictive physiology. This presumably reflects incomplete adaptation due to the presence of endomyocardial fibrosis.
Laser or radiofrequency pulmonary valvotomy in neonates with pulmonary atresia and intact ventricular septum—description of a new method avoiding arterial catheterization
Authors: Redington Andrew N. , Cullen Seamus , Rigby Michael L.
Published: August 2008

We describe a new method of transvenous laser or radiofrequency perforation of the pulmonary valve with subsequent balloon pulmonary valvoplasty. The technique obviates the need for arterial catheterisation and considerably shortens the time needed for the procedure. Uncomplicated and successful relief of pulmonary valvar atresia was achieved in both patients attempted, although one ultimately required a Blalock-Taussig shunt because of increasing muscular obstruction of the right ventricular outflow tract.
Pulmonary atresia with intact ventricular septum: Is it possible to improve survival?
Authors: Vosa Carlo , Arciprete Paolo , Caianiello Giuseppe , Palma Gaetano
Published: August 2008

Between February 1986 and December 1991, 41 patients with pulmonary atresia and intact ventricular septum were treated in our institution following a multistage protocol of management. In all cases, the first step was to construct a right modified Blalock-Taussig shunt during the neonatal period regardless of the right ventricular anatomy. Then, in patients with well-developed right ventricles possessing all three components, we proceeded to early surgical repair. In contrast, in patients with right ventricles having obliteration of some components, yet deemed to be recoverable, the next step was to provide palliative relief of obstruction in the right ventricular outflow tract followed, if possible, by subsequent repair. Fontan's operation was performed in patients with right ventricles considered unsuitable from the outset to support the pulmonary circulation. Only one patient died following the initial shunt procedure (mortality of 2.43%). The subsequent program of treatment has now been concluded in 22 patients. In all those deemed to have favorable native anatomy (10 cases), the subsequent complete repair was successful. Among the 24 patients who required palliation of the outflow tract, five died while total repair was subsequently performed in eight. Fontan's operation was performed without mortality in five patients with small right ventricles, although one patient died while waiting for surgery. In all, 89 procedures were performed with an overall mortality of 14%.
Staged surgical management of pulmonary atresia with intact ventricular septum
Authors: Battistessa Sergio A. , Jackson Mark , Hamilton David I. , Patel Raman G. , McKay Roxane
Published: August 2008

Review of an institutional experience of 81 patients admitted to the Royal Liverpool Children's Hospital between January 1958 and December 1990 with a diagnosis of pulmonary atresia and intact ventricular septum revealed that successful definitive repair was achieved in about one-third of those who underwent surgical treatment. A three-stage protocol, consisting of a systemic-pulmonary anastomosis followed by right ventricular decompression on cardiopulmonary bypass and subsequent separation of the systemic and pulmonary circulations, facilitated a biventricular correction but rarely led to a successful Fontan procedure. Both functional class and probability of survival improved after each stage of management.