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The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery (WCPCCS) will be held in Washington DC, USA, from Saturday, 26 August, 2023 to Friday, 1 September, 2023, inclusive. The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery will be the largest and most comprehensive scientific meeting dedicated to paediatric and congenital cardiac care ever held. At the time of the writing of this manuscript, The Eighth World Congress of Pediatric Cardiology and Cardiac Surgery has 5,037 registered attendees (and rising) from 117 countries, a truly diverse and international faculty of over 925 individuals from 89 countries, over 2,000 individual abstracts and poster presenters from 101 countries, and a Best Abstract Competition featuring 153 oral abstracts from 34 countries. For information about the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery, please visit the following website: [www.WCPCCS2023.org]. The purpose of this manuscript is to review the activities related to global health and advocacy that will occur at the Eighth World Congress of Pediatric Cardiology and Cardiac Surgery.
Acknowledging the need for urgent change, we wanted to take the opportunity to bring a common voice to the global community and issue the Washington DC WCPCCS Call to Action on Addressing the Global Burden of Pediatric and Congenital Heart Diseases. A copy of this Washington DC WCPCCS Call to Action is provided in the Appendix of this manuscript. This Washington DC WCPCCS Call to Action is an initiative aimed at increasing awareness of the global burden, promoting the development of sustainable care systems, and improving access to high quality and equitable healthcare for children with heart disease as well as adults with congenital heart disease worldwide.
In many parts of the world, mostly low- and middle-income countries, timely diagnosis and repair of congenital heart diseases (CHDs) is not feasible for a variety of reasons. In these regions, economic growth has enabled the development of cardiac units that manage patients with CHD presenting later than would be ideal, often after the window for early stabilisation – transposition of the great arteries, coarctation of the aorta – or for lower-risk surgery in infancy – left-to-right shunts or cyanotic conditions. As a result, patients may have suffered organ dysfunction, manifest signs of pulmonary vascular disease, or the sequelae of profound cyanosis and polycythaemia. Late presentation poses unique clinical and ethical challenges in decision making regarding operability or surgical candidacy, surgical strategy, and perioperative intensive care management.
The Fontan connection, originally described in 1971, is used to provide palliation for patients with many forms of CHDs that cannot support a biventricular circulation. An increasing number of females who have undergone these connections in childhood are now surviving into adulthood and some are becoming pregnant. We report a case of a 29-year-old woman who presented with a twin pregnancy at 33 weeks of gestation. She had significant deterioration of her cardiovascular status before the twin babies were delivered by emergency caesarean section owing to associated obstetric complications. This report also highlights the various maternal and fetal complications occurring in pregnancy of Fontan-palliated patients and suggests the need for meticulous pre-conception counselling and strict perinatal care.
In this study, we present the case of a neonate with obstructed infracardiac total anomalous pulmonary venous connection with severe pulmonary hypertension and a patent ductus arteriosus with right-to-left shunting. The patient had an unusual finding of pandiastolic flow reversal in the upper descending thoracic aorta. He underwent emergency surgical re-routing of the pulmonary veins to the left atrium, and postoperative echocardiography showed disappearance of the descending aortic flow reversal. We hypothesise that in severely obstructed total anomalous pulmonary venous connection the left ventricular output may be extremely low, resulting in flow reversal in the descending aorta.
This study was carried out to define the anatomical criteria for the diagnosis of inferior-type caval vein defect and compare the echocardiographic findings with surgical findings.
The records of 19 patients – 13 male and six female patients in the age group of 18 months to 27 years, who were diagnosed as inferior-type caval vein defect with or without anomalous drainage of right pulmonary vein(s) on echocardiography – were retrospectively reviewed and compared with surgical findings.
Surgical diagnosis of inferior-type caval vein defect was confirmed in 17 of the 19 patients. In two patients, the surgical diagnosis was that of a large fossa ovalis atrial septal defect – confluent defect and fossa ovalis atrial septal defect with deficient inferior rim in one patient each. Surgical diagnosis of anomalous drainage of pulmonary vein(s) was based on the course of the superior rim of the defect in relation to the pulmonary veins. Our echocardiographic impression of the pulmonary veins appearing in its normal position but showing abnormal drainage to right atrium was in agreement with the surgical notes. Discrepancy was found in the number of pulmonary veins draining anomalously. The discordance was related to overdiagnosis of anomalous drainage in all except one, that is, three out of four. In one, only the right lower pulmonary vein was diagnosed to be anomalous, whereas both right upper and lower pulmonary veins were found to be anomalous.
Echocardiography provides definite diagnosis of inferior-type caval vein defect. Inferior caval vein straddling and an intact fossa ovalis are prerequisites for diagnosis. Anomalous pulmonary venous drainage of the right pulmonary veins is very common in our series, although accurate diagnosis of the number of pulmonary veins was not possible in all cases. Multiple views on transthoracic echocardiography starting from the subxiphoid views delineate the morphology accurately. Transoesophageal echocardiography is required only in patients in whom the windows, especially the subxiphoid, are not adequate.
The traditional approach to repair of tetralogy of Fallot involves a right ventriculotomy for closure of ventricular septal defect. During the past two decades, reports of progressive right ventricular dilation and dysfunction, and late occurrence of ventricular arrhythmias, have led investigators to re-evaluate this approach and advocate instead the transatrial-transpulmonary approach, hoping to preserve global right ventricular function. We studied the short term effects on right ventricular function of either of the two approaches through a prospective randomised study, involving two comparable groups of patients operated in the same time frame.
Between June 1993 and February 1994, 40 patients having tetralogy of Fallot with comparable preoperative characteristics, were assigned randomly to each of two groups for surgical correction.
In 20 patients, correction was achieved via the transatrial-transpulmonary route. In the other 20 patients, transventricular correction was the chosen option. Six months after surgery, patients were evaluated clinically, by Doppler echocardiography, cardiac catheterisation, first pass radionuclide angiography and by 24 hours ambulatory electrocardiographic monitoring, taking note of hemodynamics, abnormalities in rhythm, and global right ventricular function.
There were no early deaths or morbidity in either group. Mean immediate postoperative ratio between peak right ventricular and systemic pressures was 0.62 ± 0.22 after transatrial and 0.70 ± 0.007 after transventricular correction. All patients were in functional class I. Six months after surgery the mean ratio between peak ventricular pressures was similar in the two groups (transatrial group: 0.37 ± 0.02, transventricular group: 0.38 ( 0.01), but significantly lower than that measured in the operating room. There were no significant arrythmias in either group. Mean right ventricular ejection fraction was nearly the same in both groups (transatrial group versus transventricular group; 44.83 ± 5.65% versus 42.37 ± 8.70%). Significant global hypokinesia of the right ventricle was documented in three patients, and mild hypokinesia in another three, undergoing repair through the transventricular route while in the group undergoing transatrial repair only one patient had mild hypokinesia.
We conclude that comparable hemodynamic results are obtained on short term follow-up after repair of tetralogy of Fallot by either the transatrial or transventricular route. Although more patients in the transventricular group were found to have global hypokinesia of the right ventricle, longer follow-up is necessary to establish the clinical relevence of these findings.
We present an unusual case of criss-cross heart with supero-inferior ventricles, double outlet of the right ventricle, restrictive ventricular septal defect, severe infundibular stenosis and “absent” pulmonary valve who underwent successful surgical repair. Complete preoperative diagnosis was established with cross-sectional echocardiography combined with colour Doppler and angiocardiography, which facilitated successful surgical correction.
We report a 14-year-old boy in whom an anomalous muscle bundle in the left ventricle caused subaortic obstruction. It was diagnosed by echocardiography and resected at operation. The anomalous muscle most likely represents the hypertrophied anterolateral muscle bundle.
The expectation, variance and covariance for different states of a k-state Markov chain have been given by Patankar (6), Whittle (7), Good (3) and Bhat (l). Patankar's results involve the k latent roots of the determinantal equation. As it is not easy to determine the latent roots when k > 2, the actual asymptotic values of variances and covariances cannot be readily evaluated. Whittle gives exact probability distributions for the transitions, but the moments have been obtained after some gross approximations. Good (3) and Bhat(l) have given the first two moments and product moment for the frequency of different states. By using certain methods developed by Iyer and Kapur(4), the first four cumulants and product cumulants for the transition numbers of a two-state Markov chain were calculated and presented in an earlier publication(5).
The asymptotic values of the variances and covariances for the distribution of the transition numbers AA, AB, BA and BB for a two-state Markoff chain have been given by Bartlett (l). Whittle (3) obtained the probability distribution and their factorial moments for the k-state chain under the restriction that the first and the last observations belong to the rth and the sth state. But the expressions for the factorial moments according to Whittle himself are of little immediate use. He obtains useful results for the low order factorial moments by approximating in a grosser fashion, and the evaluation of the cumulants from these factorial moments is rather cumbersome. The object of this note is to give the first four cumulants and product cumulants which are exact to the order nr (p11 – p21)n−r where r is the order of the cumulant, for the distribution of the transition numbers of a two-state Markoff chain. They have been calculated by using the method developed by Iyer and Kapur(2). This method can equally be used for evaluating the asymptotic values of the cumulants for the k-state also. This is done by differentiating directly the determinantal characteristic equation.
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