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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.
Pulmonary arterial compliance, the dynamic component of pulmonary vasculature, remains inadequately studied in patients with left to right shunts. We sought to study the pulmonary arterial compliance in patients with left to right shunt lesions and its utility in clinical decision-making.
Materials and methods:
In this single-centre retrospective study, we reviewed cardiac catheterisation data of consecutive patients of left to right shunt lesions catheterised over one year. In addition to the various other parameters, pulmonary arterial compliance was calculated, as indexed pulmonary flow (Qpi) / (Heart rate × pulse pressure in the pulmonary artery). RC time was also calculated, as the product of pulmonary arterial compliance and pulmonary vascular resistance index. Patients were divided into “operable,” “borderline,” and “inoperable” based on the decision of the treating team, and the pulmonary arterial compliance values were evaluated in these groups to study if it can be utilised to refine the operability decision.
298 patients (Median age 16 years, 56% <18 years) with various acyanotic shunt lesions were included. Overall, the pulmonary arterial compliance varied with Qpi, pulmonary artery mean pressure, and pulmonary vascular resistance index, but did not vary with age, type of lesion, or transpulmonary gradients. The median pulmonary arterial compliance in patients with normal pulmonary artery pressure (Mean pulmonary artery pressure less than 20 mmHg) was 4.1 ml/mmHg/m2 (IQR 3.2). The median pulmonary arterial compliance for operable patients was 2.67 ml/mmHg/m2 (IQR 2.2). Median pulmonary arterial compliance was significantly lower in both inoperable (0.52 ml/mmHg/m2, IQR 0.34) and borderline (0.80 ml/mmHg/m2, IQR 0.36) groups when compared to operable patients (p < 0.001). A pulmonary arterial compliance value lower than 1.18 ml/mmHg/m2 identified inoperable patients with high sensitivity and specificity (95%, AUC 0.99). However, in borderline cases, assessment by this value did not agree with empirical clinical assessment.
The median RC time for the entire study population was 0.47 S (IQR 0.30). RC time in operable patients was significantly lower than that in the inoperable patients (Median 0.40 IQR 0.23 in operable, 0.73 0.25 in inoperable patients (p < 0.001).
Addition of pulmonary arterial compliance to the routine haemodynamic assessment of patients with shunt lesions may improve our understanding of the pulmonary circulation and may have clinical utility.
Abernethy malformation is a rare entity. We report a 5-year-old boy presenting with severe pulmonary hypertension in whom Abernethy malformation and inferior caval vein interruption were diagnosed by CT angiography. In addition, the iliac veins were thrombosed with multiple venous collateral drainage. This abnormal venous anatomy caused difficulty in device closure of the Abernethy malformation, which was successfully closed using a vascular plug.
We sought to examine the influence of clinically severe lower respiratory tract infection on pulmonary artery pressure in children having CHD with post-tricuspid left-to-right shunt, as it may have physiological and clinical implications. In a prospective single-centre observational study, 45 children with post-tricuspid left-to-right shunt and clinically severe lower respiratory tract infection were evaluated during the illness and 2 weeks after its resolution. Pulmonary artery systolic pressure was estimated non-invasively using shunt gradient by echocardiography and systolic blood pressure measured non-invasively.
Median pulmonary artery systolic pressure during lower respiratory tract infection was only mildly (although statistically significantly) elevated during lower respiratory tract infection [60 (42–74) versus 53 (40–73) mmHg, (p < 0.0001)]. However, clinically significant change in pulmonary artery systolic pressure defined as the increase of >10 mmHg was present in only 9 (20%) patients. In the absence of hypoxia or acidosis, only a small minority (9%, n = 4) showed significant pulmonary artery systolic pressure rise >10 mmHg. In the absence of hypoxia or acidosis, severe lower respiratory tract infection in patients with acyanotic CHD results in only mild elevation of pulmonary artery systolic pressure in most of the patients.
Guanylate kinase, a nucleoside monophosphate kinase of Brugia malayi which is involved in reversible transfer of phosphate groups from ATP to GMP, was cloned, expressed and characterized. The native molecular mass of BmGK was found to be 45 kDa as determined by size exclusion chromatography and glutaraldehyde cross-linking which revealed that the protein is homodimer in nature. This is a unique characteristic among known eukaryotic GKs. GMP and ATP served as the most effective phosphate acceptor and donor, respectively. Recombinant BmGK utilized both GMP and dGMP, as substrates showing Km values of 30 and 38 μm, respectively. Free Mg+2 (un-complexed to ATP) and GTP play a regulatory role in catalysis of BmGK. The enzyme showed higher catalytic efficiency as compared with human GK and showed ternary complex (BmGK-GMP-ATP) formation with sequential substrate binding. The secondary structure of BmGK consisted of 45% α-helices, 18% β-sheets as revealed by CD analysis. Homology modelling and docking with GMP revealed conserved substrate binding residues with slight differences. Differences in kinetic properties and oligomerization of BmGK compared with human GK can provide the way for design of parasite-specific inhibitors.
Between January, 2002 and December, 2011, 27 patients (19 boys) underwent pericardiectomy. The mean age was 9.3 ± 4.96 years (range 0.4 to 15 years) and the mean duration of symptoms was 16.9 ± 22.15 months. In all, 25 patients had dyspnoea; eight were in New York Heart Association (NYHA) class IV; six had bacterial pericarditis; and 18 were on anti-tuberculosis treatment, although only nine had records suggesting tuberculosis. There were nine patients who underwent pre-operative pigtail catheter drainage of pericardial fluid. Surgical procedures were complete pericardiectomy (n = 20), partial pericardiectomy (n = 6), and pleuropericardial window (n = 1).The mean pre-operative right atrial pressure was 20.4 ± 4.93 mmHg. There were six hospital deaths due to low cardiac output (n = 5) and arrhythmia (n = 1). The mean intensive care unit stay was 2.7 ± 1.2 days and mean post-operative stay was 9.9 days. The mean right atrial pressure dropped to 8.7 ± 1.15 mmHg. Adverse outcomes defined as death/prolonged intensive care unit stay, prolonged post-operative stay were not associated with sex, diagnosis of tuberculosis or pyopericardium, or the duration of symptoms or pre-operative right atrial pressure. Younger patients had prolonged intensive care unit stay (p = 0.03) but not increased mortality. Advanced NYHA class predicted death (p = 0.02). The mean follow-up was 23.1 ± 23.8 months. All except one survivor are in NYHA class I and off all cardiac medications. Despite adequate surgery, pericardiectomy in children is associated with a high mortality, which is related to delayed surgery and poor pre-operative general condition. No specific pre-operative variable other than worse pre-operative NYHA class is a predictor of survival. Therefore, early pericardiectomy should be undertaken in such patients.
Glucose-6-phosphate dehydrogenase (G6PD), a regulatory enzyme of the pentose phosphate pathway from Brugia malayi, was cloned, expressed and biochemically characterized. The Km values for glucose-6-phosphate and nicotinamide adenine dinucleotide phosphate (NADP) were 0·25 and 0·014 mm respectively. The rBmG6PD exhibited an optimum pH of 8·5 and temperature, 40 °C. Adenosine 5′ [γ-thio] triphosphate (ATP-γ-S), adenosine 5′ [β,γ-imido] triphosphate (ATP-β,γ-NH), adenosine 5′ [β-thio] diphosphate (ADP-β-S), Na+, K+, Li+ and Cu++ ions were found to be strong inhibitors of rBmG6PD. The rBmG6PD, a tetramer with subunit molecular weight of 75 kDa contains 0·02 mol of SH group per mol of monomer. Blocking the SH group with SH-inhibitors, led to activation of rBmG6PD activity by N-ethylmaleimide. CD analysis indicated that rBmG6PD is composed of 37% α-helices and 26% β-sheets. The unfolding equilibrium of rBmG6PD with GdmCl/urea showed the triphasic unfolding pattern along with the highly stable intermediate obtained by GdmCl.
There is a reported change in the profile of infectious endocarditis and a reduction in its mortality in the developed world. We present our experience of infectious endocarditis in children seen in the last 5 years in the developing world.
Records of 43 consecutive children with infectious endocarditis admitted to this centre were analysed retrospectively. Diagnosis was based on presence of any two of the following: fever with no extracardiac features; vegetations on echocardiography; Positive blood culture with no extracardiac focus; and embolic episodes.
The age at diagnosis ranged from 40 days to 16 years (mean 8.5 years); Of the patients 3 were under 2 years of age; 28 were males and 15 females. Congenital heart disease was the underlying cause in 32 (74%), and rheumatic heart disease in 11 children. All except the youngest presented with fever. Blood cultures were positive in 16 (37%). Vegetations were detected by cross-sectional echocardiography in all except 1 child. After treatment 31 (72%) responded to a combination of penicillin and aminoglycoside. Emergency surgery was undertaken in 9 (21%). Three patients (7%) died, and all of these had fungal endocarditis.
Blood cultures give a low yield, but cross-sectional echocardiography is a sensitive tool in the diagnosis of infectious endocarditis. Most children respond to penicillin and an aminoglycoside. An aggressive surgical approach in complicated cases lowers the mortality.
Case records of 67 children who presented beyond infancy (57 male, 10 female) with congenital valvar aortic stenosis were reviewed to assess progression utilizing the Doppler derived peak gradient as the index of severity. Age at presentation ranged from 1–13 years, and mean follow-up was 67 ± 29 months (range 12–142 months). The patients were divided into three groups depending on the rate of progression. Those with the most rapid rate of progression were significantly older when aortic stenosis was detected and at the time of presentation compared to the other two groups, who progressed minimally or not at all (p < 0.002 and p < 0.01 respectively). There was no significant correlation between the progression of stenosis and other clinical or Doppler echocardiographic variables.
Notwithstanding the controversies evoked by the term “single ventricle”, most patients with this condition would undergo the Fontan procedure. In addition, there is a large group of patients in whom a biventricular repair would be abandoned in favour of a univentricular one because of the presence of unfavourable morphologic features. There is a need for a uniformly acceptable system of nomenclature that would permit precise description and classification of hearts with complex malformations to facilitate reporting and help in understanding the reasons for choosing a univentricular repair.
Echocardiographic, angiographic and operative records of 240 patients undergoing the Fontan procedure were analysed.
Out of the 104 patients with univentricular atrioventricular connections, 2 ventricles were discernible in all but 3 patients. A Fontan repair was performed in 136 patients with biventricular atrioventricular connections because of the presence of a hypoplastic ventricle in 52 patients and a non-committed ventricular septal defect in the remaining 84.
The Fontan operation is probably the only definitive treatment option for patients with univentricular atrioventricular connections. The decision to perform a univentricular repair in preference to a biventricular one in hearts with biventricular atrioventricular connections is based on the presence of a hypoplastic ventricle or a non-reroutable ventricular septal defect. This decision is subjective. In hearts with discordant atrioventricular connections and pulmonary stenosis, we prefer the Fontan operation to the classical repair.
A large number of patients in developing countries require surgical correction of congenitally malformed hearts. Unfortunately, only a limited number of centres offer these patients surgery at an affordable cost. In this review, we discuss the problems in managing these patients, with an emphasis on reduction of costs, so that the maximum number of patients can benefit. It is apparent that containing costs requires a multipronged approach, which begins with timely referral, and continues with early surgical correction and adequate postoperative care carried out in a scientific manner. Indigenization, innovation, training of manpower, and building a team, are essential to cut the costs, and to improve the quality of care.
A patent vertical vein might be desirable in patients with obstructive totally anomalous pulmonary venous connection with pulmonary hypertension, in order to decrease perioperative pulmonary arterial pressure and avoid pulmonary hypertensive crises. A subset of patients with an unligated vertical vein requires interruption of the vein due to the development of significant left-to-right shunt and right heart failure. We describe here a new device, permitting adjustable ligation of the vertical vein, which permits us to avoid multiple reoperations.
Patients and methods
In five patients, aged 2, 4, 3, 4, and 3 months respectively, and undergoing rechannelling of totally anomalous pulmonary venous connection with an unligated vertical vein, were treated with a device permitting adjusted ligation of the vertical vein over the course of postoperative congestive cardiac failure.
There was no early or late death. Postoperatively, all ligatures were tightened gradually over a period of 24 to 96 hours, maintaining stable haemodynamics. At a mean follow-up of 55.40 months, there was no evidence of congestive heart failure in any patient, the clinical risk score varying from zero to 2, and no requirement of anti-failure medications. Computed tomographic angiograms during follow-up revealed absence of flow through the vertical vein, and ruled out distortion of the left upper pulmonary and left brachiocephalic veins.
Use of a percutaneously adjustable device to ligate the vertical vein allows gradual tightening or loosening of the ligature under optimal physiologic conditions, without re-opening the sternum, or having to resort to another thoracotomy once the reactive components of pulmonary hypertension disappear.
Background: We describe alternative surgical techniques for construction of systemic-to-pulmonary arterial shunts, and propose criterions for their application in selected patients. Patients and methods: We constructed a variety of modified systemic-to-pulmonary arterial shunts, using polytetrafluroethylene grafts, in 92 selected patients with cyanotic congenital heart disease and anomalies of the aortic arch and systemic veins. Their age ranged from 7 days to 3.6 years, with a mean of 7.08 months. We performed 88 operations through a thoracotomy. Of this cohort, 60 patients underwent a second-stage operation, with 15 receiving a superior cavopulmonary connection, 16 a total cavopulmonary connection, and 29 proceeding to biventricular repair after a mean interval of 15.6 months. We have 21 patients awaiting their second or final stage of palliation. Results: There were five early (5.4%) and six late deaths (6.8%), two of which were related to construction of the shunts. At a mean follow-up of 45.29 months, the increase in diameter of pulmonary trunk and its right and left branches was uniform and significant (p value less than 0.001). Pulmonary arterial distortion requiring correction at the time of second-stage operation was observed in 5 patients (6.1%). Adequate overall palliation was achieved in 98% of the cohort at 8 months, 91% at 12 months, and 58% at 18 months. Conclusions: Patients with a right- or left-sided aortic arch and right-sided descending thoracic aorta, those with anomalies of systemic venous drainage masking the origin of great arterial branches, and those withdisproportionately small subclavian arteries, constitute the ideal candidates for our suggested modification of the construction of a modified Blalock-Taussig shunt. The palliation provided by these shunts was satisfactory, with predictable growth of pulmonary arteries, insignificant distortion in the great majority, and easy take-down.
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