COMBINED SEVERE AORTIC AND PULMONARY VALVULAR STENOSIS IN A CHILD AND ITS MANAGEMENT: A CASE REPORT

Combined congenital aortic and pulmonary valvular stenosis is a rare congenital heart defect. Prevalence of severe combined valvular stenosis of aortic and pulmonary valve accounts about 0.01% and also has association with many syndromes. This combination presents unusual diagnostic as well as management problems. Apart from a few case reports, there is little in the literature on the combined stenosis of both semilunar valves and its management. We present this rare combination in a 9 year old boy which was promptly managed with the balloon valvoplasty without any complications in the same setting under local anesthesia with sedation.

Circulating metabolome does not predict development of aortic stenosis

Background Calcific aortic valve disease (CAVD) is the most common valvular heart disease in Western world. CAVD is ranging from mild aortic valve sclerosis to severe obstructive aortic stenosis (AS). The development of AS has been associated with several risk factors including age, sex and hypertension. However, there is limited knowledge about factors that predict the development of aortic stenosis. Purpose We investigated if the circulating metabolite profile can predict the development of aortic stenosis in Finnish males. Methods We did a non-targeted LC-MS metabolomics analysis to baseline (1984–1989) serum samples from a prospective population-based Kuopio Ischemic Heart Disease risk factor study (KIHD) cohort of 2682 random Finnish males aged from 42 to 60 years. During the follow-up (until year 2020), 53 subjects developed either moderate (peak flow gradient 36–64mmHg or mean flow gradient 20–40mmHg) or severe aortic valvular stenosis (peak flow gradient over 64mmHg or mean gradient over 40mmHg). The AS patients were collected from the KIHD database using appropriate ICD-10 -codes for aortic valvular disease (from baseline to the end of the year 2017) and the diagnosis was checked manually using hospital medical records of the individuals. Results The AS patients seemed to have altered lipid metabolism and possibly altered composition of gut microbiota, since several acylcarnitines (e.g. octanoylcarnitine [Cohen's d=−0.40], decanoylcarnitine [d=−0.43], layroylcarnitine [d=−0.41], and oleoylcarnitine [d=−0.40]), and branched chain amino acids (BCAA, e.g. leucine [d=0.39], and isoleucine [d=0.49]) had p-values below 0.05. However, after correction for multiple testing, there were no significant differences between the cases and controls. Conclusions The present preliminary results, in need of verification with a larger set of samples, suggest that subjects, who will later develop AS might have reduced levels of acylcarnitines and increased levels of BCAA when compared to matched controls. However, these changes do not have large effects sizes and are likely not good candidates for biomarkers to predict future diagnosis of AS. FUNDunding Acknowledgement Type of funding sources: Foundation. Main funding source(s): The Finnish Cultural Foundation, The Finnish Foundation for Cardiovascular Research.

An infant with suspected missed diagnosis of Williams syndrome failed weaning off CPB after surgical correction of pulmonary stenosis: a case report and literature review

Williams syndrome (WS) is a rare congenital developmental disorder caused by the deletion of between 26 and 28 genes on chromosome 7q11.23. For patients with WS, in view of the particularity of the supravalvular aortic stenosis, choosing appropriate arterial cannula, maintaining higher perfusion pressure as well as strengthening myocardial protection during cardiopulmonary bypass (CPB) is essential to the clinical outcome. Here, we report a child with pulmonary artery valvular stenosis who failed to wean off CPB because of malignant arrhythmias and cardiac insufficiency after surgical correction of pulmonary valvular stenosis. With the assistance of extracorporeal membrane oxygenation (ECMO), emergency cardiac catheterization revealed supravalvular aortic stenosis (SVAS), which suggests a suspected missed diagnosis of WS. Finally, under the support of ECMO, the cardiac function gradually returned to normal, and the child was discharged 23 days after surgery.

Transthoracic Vector Flow Imaging in Pediatric Patients with Valvular Stenosis – A Proof of Concept Study

Purpose Continuous wave Doppler ultrasound is routinely used to detect cardiac valve stenoses. Vector flow imaging (VFI) is an angle-independent real-time ultrasound method that can quantify flow complexity. We aimed to evaluate if quantification of flow complexity could reliably assess valvular stenosis in pediatric patients. Materials and Methods Nine pediatric patients with echocardiographically confirmed valvular stenosis were included in the study. VFI and Doppler measurements were compared with transvalvular peak-to-peak pressure differences derived from invasive endovascular catheterization. Results Vector concentration correlated with the catheter measurements before intervention after exclusion of one outlier (r=−0.83, p=0.01), whereas the Doppler method did not (r=0.49, p=0.22). The change in vector concentration after intervention correlated strongly with the change in the measured catheter pressure difference (r=−0.86, p=0.003), while Doppler showed a tendency for a moderate correlation (r=0.63, p=0.07). Conclusion Transthoracic flow complexity quantification calculated from VFI data is feasible and may be useful for assessing valvular stenosis severity in pediatric patients.

Acute heart failure in pregnancy: Importance of a timely multidisciplinary approach to recognition and management

Increased cardiovascular demands of pregnancy may unmask occult diseases, such as dilated cardiomyopathy or valvular stenosis, or precipitate peripartum cardiomyopathy. We report a case of the emergency management and delivery of a young pregnant woman who presented with acute decompensated heart failure that was not immediately recognized. An emergency transfer to a tertiary care institution was arranged. Once diagnosed, the patient received multidisciplinary care shared between cardiologists, obstetricians, cardiac anesthetists, a neonatologist, and a midwife, resulting in good maternal and fetal outcomes.

P208 Differentiating intestinal tuberculosis from Crohn’s Disease: External validation of a new nomogram

Background Intestinal tuberculosis (IT) poses a real problem of differential diagnosis with Crohn’Disease (CD). Indeed, the distinction between these two pathologies represents a real challenge for clinicians because of their multiple similarities. Based on simple biological, endoscopic and radiological criteria, A new nomogram was developed by Yao He et al. who would differentiate between the two diseases. Objective To validate externally this new nomogram in a series of patients with IT and CD. Methods We have collected retrospectively patients diagnosed with CD and IT in our center for a period 11 years old. Patients whose medical file included the data used in the nomogram have been included. The discrimination performance of the nomogram was evaluated by calculating the area under the ROC curve. Results Of the 76 included patients, 16 had one IT and 60 had one CD. The average age of patients with IT was 44.76 ± 19 years old. The most frequent revealing symptoms were subocclusive syndromes (n = 10) followed by abdominal pain (n = 8). Endoscopic lesions were located in the majority of cases in the ileocecal region (n = 12) and were dominated by the retracted aspect of the cecum (n = 5), transverse colonic ulcerations (n = 4) and valvular stenosis (n = 2). A tumor appearance was noted in two patients.The radiological images of the lungs suggestive of pulmonary tuberculosis were found only in two patients. Intradermal reaction (IDR) to tuberculin was positive in all cases where it was practiced. (n = 14). In ten cases, the diagnosis of ITwas made following bowel resection whose indications were diagnostic uncertainty (n = 4), acute bowel obstruction (n = 4) and suspected CD ileocecal refractory to medical treatment (n = 2). The discrimination of the nomogram analyzed by the ROC curve was 0.956 (95% CI [0.875; 1]). A threshold of 0.5 was associated with a sensitivity of 98.3%; a specificity of 92.8% and a positive and negative predictive value for the diagnosis CD of 98.3 and 92.8 respectively. Conclusion Although Tunisia is a country of strong endemicity for tuberculosis, intestinal localization seems infrequent and misdiagnosed. The nomogram applied seems to have excellent performance diagnose what could prevent resections intestinal for diagnostic purposes. However, studies multicenter prospective studies remain necessary for a large-scale validation.

Three steps approach for preoperative evaluation of tetralogy of Fallot patients: role of 128 MDCT

Background Tetralogy of Fallot is the most common congenital cyanotic heart disease. Advances in surgical repair recently facilitate survival of the affected patients into adulthood with good quality of life. Despite imaging plays crucial role in diagnosis of TOF patients, no single diagnostic modality suffices for complete evaluation of TOF. Thus, different diagnostic tools should be used alone or in combination according to patient’s clinical question. This study aims to find out the agreement between the echocardiography and MDCT in evaluation of TOF patients prior to surgical repair. Results Three-step approach used in scanning TOF patients. Firstly, classic TOF findings, MDCT was comparable to echocardiography in overriding aorta (k = 0.6), VSD (k = 0.8), RVH (k = 0.4), and sub valvular pulmonary stenosis (k = 0.6). Echocardiography is superior to MDCT in evaluation of valvular stenosis (k = 0.4). MDCT was superior to echocardiography in supravalvular pulmonary stenosis. Secondary, surgical relevant findings, McGoon ratio calculated in 94.2%, small and major APCs in 45.5% and 28.5% respectively, PDA in 35% and anomalous coronary artery in 5.1%. Thirdly, other TOF-associated anomalies, common: ASD (34.3%) and RAA (28.3%), and persistent LSVC (10.3%). Uncommon: situs ambiguous (2.5 %), PAPVR (1.2%), and IVC anomalies (3.8%). MDCT was superior to echocardiography in surgical relevant findings and other TOF-associated anomalies. Conclusion MDCT is a crucial imaging tool for extra cardiac evaluation of TOF patients prior to surgical repair.

Crosslinking porcine aortic valve by radical polymerization for the preparation of BHVs with improved cytocompatibility, mild immune response, and reduced calcification

Over one million artificial heart valve transplantations are performed each year due to valvular stenosis or regurgitation. Among them, bioprosthetic heart valves (BHVs) are increasingly being used because of the absence of the need for lifelong anticoagulation. Almost all of the commercial BHVs are treated with Glutaraldehyde (GLUT). As GLUT-treated BHVs are prone to calcification and structural degradation, their durability is greatly reduced with a service life of only 12–15 years. The physiological structure and mechanical properties of the porcine aortic valve (PAV) are closer to that of a human heart valve, so in this study, PAV is used as the model to explore the comprehensive properties of the prepared BHVs by radical polymerization crosslinking method. We found that PAV treated by radical polymerization crosslinking method showed similar ECM stability and biaxial mechanical properties with GLUT-treated PAV. However, radical polymerization crosslinked PAV exhibited better cytocompatibility and endothelialization potential in vitro cell experiment as better anticalcification potential and reduced immune response than GLUT-treated PAV through subcutaneous animal experiments in rats. To conclude, a novel crosslinking method of non-glutaraldehyde fixation of xenogeneic tissues for the preparation of BHVs is expected.