Ujjwal Kumar Chowdhury, Robert H Anderson, Diane E Spicer, Niraj N Pandey, Saurabh K Gupta, Niwin George, Maroof A Khan, Chaitanya Chittimuri
{"title":"The Surgical Significance of Phenotypic Variability in the Setting of Tetralogy of Fallot.","authors":"Ujjwal Kumar Chowdhury, Robert H Anderson, Diane E Spicer, Niraj N Pandey, Saurabh K Gupta, Niwin George, Maroof A Khan, Chaitanya Chittimuri","doi":"10.1177/21501351241274731","DOIUrl":"10.1177/21501351241274731","url":null,"abstract":"<p><p>The phenotypic feature of tetralogy of Fallot is anterocephalad deviation of the muscular outlet septum, or its fibrous remnant, relative to the septoparietal trabeculation, coupled with hypertrophy of septoparietal trabeculations. Although this feature permits recognition of the entity, no two cases are identical. Once diagnosed, treatment is surgical. The results of surgical treatment have improved remarkably over recent decades. The results are now sufficiently excellent, including those in the developing world, that attention is now directed toward avoidance of morbidity, while still seeking, of course to minimize any fatalities due to surgical intervention. It is perhaps surprising that attention thus far has not been directed on the potential significance of phenotypic variation relative to either mortality or morbidity subsequent to surgical correction. The only study we have found specifically addressing this variability focused on the extent of aortic override, and associated malformations, but made no mention of variability in the right ventricular margins of the interventricular communication, nor the substrates for subpulmonary obstruction. In this review, therefore, we assessed the potential significance of known morphological variability to the outcomes of surgical intervention in over 1,000 individuals undergoing correction by the same surgeon in a center of excellence in a developing country. We sought to assess whether the variations were associated with an increased risk of postoperative death, or problems of rhythm. In our hands, double outlet ventriculoarterial connection was associated with increased risk of death, while the presence of a juxta-arterial defect with perimembranous extension was associated with rhythm problems.</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"83-95"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142335652","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Invited Commentary: Morphological Variations in Tetralogy of Fallot and Their Impact in the Surgical Approach and Postoperative Evolution.","authors":"Vera D Aiello","doi":"10.1177/21501351241292620","DOIUrl":"10.1177/21501351241292620","url":null,"abstract":"","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"96-97"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142585363","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Krishna Patel, Tracie K Lin, Joseph B Clark, Gary D Ceneviva, Jason R Imundo, Debra Spear, Allen R Kunselman, Neal J Thomas, John L Myers, Akif Ündar
{"title":"Randomized Trial of Pulsatile and Nonpulsatile Flow in Cyanotic and Acyanotic Congenital Heart Surgery.","authors":"Krishna Patel, Tracie K Lin, Joseph B Clark, Gary D Ceneviva, Jason R Imundo, Debra Spear, Allen R Kunselman, Neal J Thomas, John L Myers, Akif Ündar","doi":"10.1177/21501351241288835","DOIUrl":"https://doi.org/10.1177/21501351241288835","url":null,"abstract":"<p><strong>Background: </strong>The study objective was to determine the impact of cardiopulmonary bypass perfusion modalities on cerebral hemodynamics and clinical outcomes in congenital cardiac surgery patients stratified by acyanotic versus cyanotic heart disease.</p><p><strong>Methods: </strong>A total of 159 pediatric (age <18 years) cardiac surgery patients were prospectively randomized to pulsatile or nonpulsatile cardiopulmonary bypass and stratified by type of congenital heart disease: acyanotic versus cyanotic. Intraoperative cerebral gaseous microemboli counts and middle cerebral artery pulsatility index were assessed. Organ injury was quantified by Pediatric Logistic Organ Dysfunction-2 (PELOD-2) score at 24, 48, and 72 h postoperatively. Additional outcomes included Pediatric Risk of Mortality-3 score, vasoactive-inotropic score, duration of mechanical ventilation, intensive care and hospital length of stay, and 180-day mortality. Heterogenous variance linear models (ie, ANOVA and mixed models) and χ<sup>2</sup> tests were used to compare groups for continuous and categorical variables, respectively.</p><p><strong>Results: </strong>Within congenital heart disease subgroups, patients randomized to nonpulsatile versus pulsatile bypass had similar preoperative and operative characteristics. While the intraoperative pulsatility index was higher in the pulsatile subset of both acyanotic and cyanotic groups (<i>P</i> < .05), regional cerebral oxygen saturation, mean arterial pressure, and gaseous microemboli counts were similar. Postoperative PELOD-2 scores decreased at similar rates in the acyanotic and cyanotic subgroups regardless of the perfusion modality utilized. There were also no significant between-group differences in the additional postoperative outcomes by perfusion modality in either acyanotic or cyanotic groups.</p><p><strong>Conclusions: </strong>Despite patients undergoing pulsatile cardiopulmonary bypass experiencing a more physiologic pulsatility index in both acyanotic and cyanotic groups, no significant differences in cerebral hemodynamics or clinical outcomes were appreciated.</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"21501351241288835"},"PeriodicalIF":0.0,"publicationDate":"2024-12-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142879293","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
F Doig, H Kheslat, Y d'Udekem, S P Namachivayam, A J Iyengar
{"title":"Peritoneal Dialysis Catheter Placement on the Right Side of the Falciform Ligament to Prevent Obstruction by the Omentum.","authors":"F Doig, H Kheslat, Y d'Udekem, S P Namachivayam, A J Iyengar","doi":"10.1177/21501351241299762","DOIUrl":"https://doi.org/10.1177/21501351241299762","url":null,"abstract":"<p><p>We describe a simple and reproducible technique for neonatal peritoneal dialysis catheter insertion following cardiac surgery which prevents the catheter from becoming blocked by the omentum.</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"21501351241299762"},"PeriodicalIF":0.0,"publicationDate":"2024-12-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142866957","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kwadwo A Danso, Grace Appah, Rosemary S Akuaku, Yaa S Karikari, Annette K Ansong, Frank Edwin, Nana-Akyaa Yao
{"title":"Prevalence of Congenital Heart Disease in Children and Adolescents Under 18 in Africa: A Systematic Review and Meta-Analysis.","authors":"Kwadwo A Danso, Grace Appah, Rosemary S Akuaku, Yaa S Karikari, Annette K Ansong, Frank Edwin, Nana-Akyaa Yao","doi":"10.1177/21501351241299405","DOIUrl":"https://doi.org/10.1177/21501351241299405","url":null,"abstract":"<p><strong>Background: </strong>There are limited population-based studies on congenital heart disease (CHD) in the pediatric population in Africa. Technological advancements in diagnostic tools have resulted in multiple echocardiographic studies in hospital settings. We aimed to determine the prevalence of CHD in both settings (population-based and hospital based) followed by comparing the two estimates for a difference.</p><p><strong>Methods: </strong>We systematically searched PubMed, Google Scholar, African Journals Online, and African Index Medicus for eligible studies from 1992 through 2022. We performed a meta-analysis using the random-effects model.</p><p><strong>Results: </strong>We selected 42 studies; 10 population studies with 1,011,163 participants, and 32 hospital-based studies with 605,268 patients for the analyses. The population and hospital-based prevalence were 5.12 versus 12.63 per 1,000 population of children (<i>P</i> = .007). Ventricular septal defect was the most common type of CHD in both settings (0.61 vs 1.88 per 1,000), followed by atrial septal defect (0.26 vs 0.68 per 1,000). Tetralogy of Fallot was the most common cyanotic heart lesion in both settings (0.08 vs 0.52 per 1,000).</p><p><strong>Conclusions: </strong>The population-based prevalence of CHD was significantly lower than the hospital-based prevalence (5.12 vs 12.63 per 1,000 population of children). Juxtaposing these two prevalence estimates against each other can be a reasonable alternative to quantifying the contemporary burden of CHD in the pediatric population of Africa. Moving forward, efforts should bolster awareness of CHD in Africa, and further advocacy for children with CHD should be a priority on the continent.</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"21501351241299405"},"PeriodicalIF":0.0,"publicationDate":"2024-12-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142866255","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Aleksei Ye Chernogrivov, Aleksei I Kim, Frank Cetta, Tatyana V Rogova, Inessa Ye Nefedova, Igor E Chernogrivov, Aleksandr A Esayan, Vera I Dontsova, Alina S Sarkisyan
{"title":"Classical Ozaki Procedure in a Seven-Month-Old Infant.","authors":"Aleksei Ye Chernogrivov, Aleksei I Kim, Frank Cetta, Tatyana V Rogova, Inessa Ye Nefedova, Igor E Chernogrivov, Aleksandr A Esayan, Vera I Dontsova, Alina S Sarkisyan","doi":"10.1177/21501351241297712","DOIUrl":"https://doi.org/10.1177/21501351241297712","url":null,"abstract":"<p><p>We present a case study of neotricuspidization of the aortic valve with neocusp suturing based on templates similar to those used in the Ozaki procedure but adapted for infants. This is an alternative for patients when valve reconstruction is impossible or risky. A good postoperative hemodynamic outcome was obtained, but it is necessary to evaluate this approach on the basis of long-term results.</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"21501351241297712"},"PeriodicalIF":0.0,"publicationDate":"2024-12-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142796754","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Bharat Siddharth, Pavan Kumar Dammalapati, Vasudev V, Sameh Said
{"title":"Novel Use of the Right Atrial Appendage as a Neopulmonary Valve in a Child With Infective Endocarditis.","authors":"Bharat Siddharth, Pavan Kumar Dammalapati, Vasudev V, Sameh Said","doi":"10.1177/21501351241297713","DOIUrl":"https://doi.org/10.1177/21501351241297713","url":null,"abstract":"<p><p>Infective endocarditis (IE) is a serious complication following any cardiac surgery. <i>Pseudomonas</i> as the causative organism is very rare. The authors report a case of IE post cardiac surgery for ventricular septal defect closure which was found to be caused by <i>Pseudomonas</i> involving the pulmonary valve. It was successfully managed by resection of a considerable amount of vegetation that had damaged the pulmonary valve. A previously placed patch was removed and pulmonary valve reconstruction using the right atrial appendage was accomplished.</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"21501351241297713"},"PeriodicalIF":0.0,"publicationDate":"2024-12-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142796755","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
David M Kwiatkowski, Scott Auerbach, Robert A Niebler
{"title":"Adjudicating Registry Data: Benefits and Pitfalls-The ACTION Experience.","authors":"David M Kwiatkowski, Scott Auerbach, Robert A Niebler","doi":"10.1177/21501351241293714","DOIUrl":"https://doi.org/10.1177/21501351241293714","url":null,"abstract":"<p><p>The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) registry collects data and adjudicates adverse events on pediatric patients receiving mechanical circulatory support at participating centers. To date, the registry includes over 1500 patients from 48 centers. Data collected has been used for research publications, quality improvement projects, and the regulatory support of several ventricular assist devices. While this dataset is large and adjudicated, the registry has real limitations that may limit the interpretation of findings. A full understanding of potential biases and limitations allows researchers and clinicians to make meaningful conclusions from the patient population. This report reviews the strengths and limitations of the ACTION registry to ensure optimal utility among pediatric patients receiving mechanical circulatory support. It also suggests potential areas for improvement to facilitate best care of this vulnerable population.</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"21501351241293714"},"PeriodicalIF":0.0,"publicationDate":"2024-12-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142796753","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Catherine E Stauber, Andrew Well, Catherine Dawson-Gore, Michelle Mizrahi, Charles D Fraser, Carlos M Mery, Daniel Stromberg
{"title":"Aspirin Nonresponsiveness in Congenital Heart Disease-Prevalence, Risk Factors, and Management.","authors":"Catherine E Stauber, Andrew Well, Catherine Dawson-Gore, Michelle Mizrahi, Charles D Fraser, Carlos M Mery, Daniel Stromberg","doi":"10.1177/21501351241293721","DOIUrl":"https://doi.org/10.1177/21501351241293721","url":null,"abstract":"<p><strong>Background: </strong>Aspirin is frequently utilized for antiplatelet therapy in children with congenital heart disease (CHD). Patients who are unresponsive to aspirin, as measured by aspirin reaction units (ARU), are at higher risk for thrombotic events. It is undetermined if dose modification of aspirin results in adequate responsiveness in these patients. This study evaluates the prevalence and risk factors for aspirin nonresponsiveness and the results of dose escalation in this population.</p><p><strong>Methods: </strong>This is a retrospective review of patients cared for in the cardiac care unit at a quaternary care academic congenital heart center who received aspirin and had responsiveness evaluated between January 2018 and January 2023. Patient demographics and clinical characteristics were extracted from the medical record. Descriptive, parametric, and nonparametric univariate analysis were employed.</p><p><strong>Results: </strong>A total of 142 patients (69 [49%] female, 45 [32%]Non-Hispanic White, and 63 [44%] Hispanic]) were identified. Median age at first aspirin responsiveness assessment was 54 [interquartile range, IQR: 23.3-411.5] days with a median weight of 5.2 [IQR: 3.64-9.29] kg. Of these, 32/142 (22.5%) were nonresponsive on their initial testing. Of these patients, 23/32 (72%) had follow-up testing with 19/23 (83%) subsequently becoming therapeutic. This was achieved with an increased dose in 12/19 (63%) patients and increased duration of therapy in 7/19 (37%) patients. Seventeen of 142 (12%) patients experienced a thrombotic event, 13/17 (77%) of which were therapeutic on initial responsiveness assessment.</p><p><strong>Conclusions: </strong>It is common for CHD patients to be aspirin nonresponsive with initial weight-based dosing. If aspirin is used in this population, it is necessary to evaluate ARUs on all patients as underdosing is not uncommon with current weight-based dosing methods.</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"21501351241293721"},"PeriodicalIF":0.0,"publicationDate":"2024-12-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142788356","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Subxiphoid Insertion of a Pediatric Implantable Pulse Generator in a Neonate With Congenital Complete Heart Block.","authors":"Benjamin P Kirk, Benjamin Hale, Mohsen Karimi","doi":"10.1177/21501351241297711","DOIUrl":"https://doi.org/10.1177/21501351241297711","url":null,"abstract":"<p><p>Permanent pacemaker placement is often indicated in newborns with congenital complete heart block (CCHB). The disproportionate size between commercially available devices and the neonate can lead to delayed intervention and high postoperative complication rates. Recently, Medtronic developed a Pediatric Implantable Pulse Generator (IPG); a very small single chamber pacemaker requiring a bipolar lead. This case report describes a minimally invasive approach for the implantation of this Pediatric IPG shortly after birth in a 2.32 kg neonate with CCHB.</p>","PeriodicalId":94270,"journal":{"name":"World journal for pediatric & congenital heart surgery","volume":" ","pages":"21501351241297711"},"PeriodicalIF":0.0,"publicationDate":"2024-12-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142788357","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}