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Stubborn Hypoxia in Neonates with D-Transposition of the Great Arteries after Arterial Switch Operation: Central Sleep Apnea as the Cause and Potential Indicator of Brain Immaturity

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Congenital Heart Disease 2024年第2期19卷 185-195页

作者： Camden L.Hebson Kyle Bliton Amr Y.Hammouda Kaitlyn Barr W.Hampton Gray Mohini Gunnett Waldemar F.Carlo Department of pediatrics Division of Pediatric CardiologyUniversity of Alabama BirminghamBirmingham35233USA Department of pediatrics Division of Pediatric PulmonologyUniversity of Alabama BirminghamBirmingham35233USA Department of pediatrics University of Alabama BirminghamBirmingham35233USA Department of Surgery Division of Cardiothoracic SurgeryUniversity of Alabama BirminghamBirmingham35233USA

D-transposition of the great arteries(d-TGA)is surgically repaired with the arterial switch operation(ASO)with excellent results,however short and long-term morbidities still develop including neurocognitive *** significant central sleep apnea is uncommon in non-premature infants,but when present indicates immature autonomic control of respiration likely due to a neurologic *** report the unanticipatedfinding of central sleep apnea in four-term neonates with d-TGA after uncomplicated ASO,with the short-term complication of delayed hospital discharge and long-term concerns regarding this early marker of brain immaturity and its hindrance to normal *** this report,we will review each patient’s clinical course and then examine the literature on pediatric central sleep apnea,neurodevelopmental outcomes after ASO,and the important overlap of these entities in the care of patients going forward.

关键词： Transposition congenital heart disease sleep apnea polysomnography

ccdc141 is required for left-right axis development by regulating cilia formation in the Kupffer's vesicle of zebrafish

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Journal of Genetics and Genomics 2024年第9期51卷 934-946页

作者： Pengcheng Wang Wenxiang Shi Sijie Liu Yunjing Shi Xuechao Jiang Fen Li Sun Chen Kun Sun Rang Xu Department of pediatric cardiology Xinhua HospitalShanghai Jiao Tong University School of MedicineShanghai 200092China Department of Cardiovascular Medicine Heart Failure CenterRuijin HospitalRuijin Hospital Lu Wan BranchShanghai Jiao Tong University School of MedicineShanghai 200025China Scientific Research Center Xinhua HospitalShanghai Jiao Tong University School of MedicineShanghai 200092China Department of pediatric cardiology Shanghai Children's Medical CenterShanghai Jiao Tong University School of MedicineShanghai 200127China

Laterality is a crucial physiological process intricately linked to the cilium-centrosome complex during embryo *** in the process can result in severe organ ***-coil domain containing 141(CCDC141)has been previously known as a centrosome-related gene,but its role in left-right(LR)asymmetry has not been *** this study,we utilize the zebrafish model and human exome analysis to elucidate the function of ccdc141 in laterality *** knockdown of ccdc141 in zebrafish disrupts early LR signaling pathways,cilia function,and Kupffer's vesicle *** ccdc141-knockdown embryos exhibiting aberrant LR patterns,ccdc141-null mutants show no apparent abnormality,suggesting a genetic compensation response *** parallel,we observe a marked reduction inα-tubulin acetylation levels in the ccdc141 *** treatment with histone deacetylase(HDAC)inhibitors,particularly the HDAC6 inhibitor,rescues the ccdc141 crispant ***,exome analysis of 70 patients with laterality defects reveals an increased burden of CCDC141 mutations,with in-vivo studies verifying the pathogenicity of the patient mutation *** findings highlight the critical role of ccdc141 in ciliogenesis and demonstrate that CCDC141 mutations lead to abnormal LR patterns,identifying it as a candidate gene for laterality defects.

关键词： Left-right axis Cilia WES Laterality defects Zebrafish CCDC141

Prognosis and outcome of intrauterine treatment of fetuses with critical congenital heart disease

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Chinese Medical Journal 2024年第12期137卷 1431-1436页

作者： Liqing Zhao Lei Wang Hongping Xia Yurong Wu Xianting Jiao Hong Zhu Sun Chen Kun Sun Department of pediatric cardiology Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai 200092China Department of Diagnosis and Treatment Center for in utero pediatric Disease Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai 200092China Department of Neonatology Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of MedicineShanghai 200092China

Background:Intrauterine valvuloplasty is an innovative therapy,which promotes ventricular growth and function in some congenital heart diseases(CHDs).The technique remains challenging and can only be performed in a few *** study aimed to assess the feasibility and mid-term outcomes of fetal cardiac intervention(FCI)in fetuses with critical CHD in an experienced tertiary ***:Five fetal aortic valvuloplasty(FAV)or fetal pulmonary valvuloplasty(FPV)procedures were performed in our fetal heart center between August 2018 and May *** success was defined as crossing the aortic or pulmonary valve and balloon inflation,followed by evidence of increased blood flow across the valve and/or new ***-up clinical records and echocardiography were obtained during the prenatal and postnatal ***:Five fetuses received FAV or FPV,including critical aortic stenosis(n=2)and pulmonary atresia with intact ventricular septum(n=3).The mean maternal age was 33.0±2.6 *** median gestational age(GA)at diagnosis was 24 weeks(range,22-26 weeks).The median GA at intervention was 29 weeks(range,28-32 weeks).All five cases underwent successful or partially successful *** patient had pulmonary valve perforation without balloon *** procedure-related deaths or significant complications ***,one neonatal death occurred due to heart and renal *** median follow-up period was 29.5 months(range,8.0-48.0 months).The four surviving patients had achieved biventricular circulation,exhibited improved valve,and ventricular development at the last follow-up ***:Intrauterine FCI could be performed safely with good prognosis in critical CHD.

关键词： Fetal cardiac intervention Valvuloplasty Pulmonary atresia Critical aortic stenosis Congenital heart disease

The Impact of a Prior Norwood Procedure on Cardiac Transplantation in Failed Fontan Physiology

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Congenital Heart Disease 2024年第3期19卷 257-266页

作者： Ryan G.McQueen Nikki M.Singh Ronald K.Woods Medical College of Wisconsin Herma Heart Institute at Children’s WisconsinMilwaukeeUSA Division of pediatric cardiology Medical College of WisconsinHerma Heart Institute at Children’s WisconsinMilwaukeeUSA Division of pediatric Cardiothoracic Surgery Medical College of WisconsinHerma Heart Institute at Children’s WisconsinMilwaukeeUSA

Objective:The objective of this study was to compare cardiac transplant operative and postoperative courses of patients with failed Fontan physiology who were initially palliated with a Norwood(FFN)to those without a prior Norwood(FF).Methods:A single-institution retrospective review of all patients with Fontan failure who under-went cardiac transplantation from 2003–2021 was completed-22 underwent prior Norwood(FFN)and 11 did not(FF).Descriptive and inferential statistics were calculated for operative course and patient ***:The operative course of the FFN cohort appeared to be more complex(not statistically significant,but clinically relevant)-this group exclusively experienced sternal re-entry events(3 of 22 patients)and concomitant neo-aor-tic reconstruction(6 patients),had a longer duration of surgery(median of 682 min vs.575.5 min),more time on circulatory arrest(median of 25.5 min vs.12.5 min),and more frequent use of open sternal management[50%of patients(11/22)vs.27.3%of patients(3/11)].Postoperatively,these patients underwent more mediastinal explora-tions[other than sternal closure;40.9%of patients(9/22)vs.18.2%of patients(2/11)],spent more time on mechanical ventilation(median of 5 days vs.2 days),had a longer length of stay(median of 30 days vs.19 days),and required more catheter-based re-interventions[22.7%of patients(5/22)vs.9.1%of patients(1/11)].Conclu-sion:Although underpowered,our results suggest that the operative course of FFN patients is more challenging,based mostly on neo-aortic arch *** turn,this likely leads to a more complex postoperative *** are currently collaborating with other institutions to increase the cohort size and power of the study.

关键词： Fontan Fontan failure Norwood cardiac transplantation

A 63-Year-Old Male with D-Transposition of the Great Arteries Who Had an Early Form of the Arterial Switch Operation

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Congenital Heart Disease 2024年第1期19卷 65-68页

作者： Michael A.Rebolledo Jane S.Yao Jason N.Johnson Umar S.Boston Benjamin R.Waller III Division of pediatric cardiology Department of PediatricsThe University of Tennessee Health Science CenterThe Heart InstituteLe Bonheur Children’s HospitalMemphisUSA College of Medicine The University of Tennessee Health ScienceMemphisUSA Division of pediatric Cardiothoracic Surgery Department of SurgeryThe University of Tennessee Health Science CenterHeart InstituteLe Bonheur Children’s HospitalMemphisUSA

We describe a 63-year-old male who appears to have undergone an early form of the arterial switch operation for D-transposition of the great arteries performed in the *** review the clinical and imaging data that support our *** had a diagnostic cardiac catheterization which demonstrated severe pulmonary hypertension responsive to epoprostenol and *** case may represent one example of the experimental surgical work done prior to *** Jatene’s description of theﬁrst successful arterial switch performed in 1975.

关键词： Adult congenital heart disease arterial switch operation great vessel anomaly imaging(all modalities)

Fate of Right Coronary Artery Occlusion after a Surgically Repaired Aorto- Ventricular Tunnel in a Neonate

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Congenital Heart Disease 2024年第3期19卷 267-273页

作者： Sherif Negm Loic Mace Fedoua El Louali Beatrice Desnous Philipe Aldebert Virginie Fouilloux Marien Lenoir Department of pediatric Cardiac Surgery Hôpital Timone enfantAssistance Public-Hopitaux MarseilleAix-Marseille UniversitéMarseilleFrance Department of Cardiothoracic Surgery Tanta UniversityTantaEgypt Department of pediatric cardiology Hôpital Timone enfantAssistance Public-Hopitaux MarseilleAix-Marseille UniversitéMarseilleFrance Department of Neurology Hôpital Timone enfantAssistance Public-Hopitaux MarseilleAix-Marseille UniversitéMarseilleFrance

The aorto-ventricular tunnel is a rare congenital cardiac *** present a case of aorto-ventricular tunnel diagnosed via fetal *** surgery was performed on the 2nd day of life to close the tun-nel,located just in front of the right coronary ostium,due to the patient’s unstable health *** post-operative period revealed complete occlusion of the right coronary *** to the patient’s stability,we opted not to reintervene on the right coronary *** patient fully recovered without the need for further coronary *** cases of patients with an aorto-ventricular tunnel(AVT)and associated coronary lesions,it is crucial to exercise caution when intervening in the coronary arteries.

关键词： Aorto-ventricular tunnel aorta right coronary artery

SARS-CoV-2 variants are associated with different clinical courses in children with MIS-C

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World Journal of pediatrics 2024年第2期20卷 143-152页

作者： Andres F.Moreno Rojas Emelia Bainto Helen Harvey Adriana H.Tremoulet Jane C.Burns Kirsten B.Dummer Department of pediatrics Division of Pediatric CardiologyUniversity of California San Diegoand Rady Children’s Hospital3020 Children’s Way MC 5004San DiegoCA 92123USA Kawasaki Disease Research Center La JollaCAUSA Department of pediatrics Division of Pediatric Critical CareUniversity of California San Diegoand Rady Children’s HospitalSan DiegoCAUSA Department of pediatrics University of California San Diegoand Rady Children’s HospitalSan DiegoCAUSA

Background Recent infection with SARS‑CoV-2 in children has been associated with multisystem inflammatory syndrome in children(MIS-C).SARS‑CoV-2 has undergone different *** publications exist about specific variants and their correlation with the severity of *** This was a single-center,retrospective study including all patients admitted with MIS-C at Rady Children’s Hospital-San Diego between May 2020 and March *** epidemiologic data,including viral genomic information,were obtained from public ***,clinical presentation,laboratory values,and outcomes were obtained from electronic medical *** The analysis included 104 pediatric *** MIS-C waves were *** variants in San Diego during the first wave included clades 20A to *** the second wave,there were variants from clades 20A to C,20G,21C(Epsilon),20I(Alpha),and 20J(Gamma).The third wave had Delta strains(clades 21A,21I,and 21J),and the fourth had Omicron variants(clades 21K,21L,and 22C).MIS-C presented with similar symptoms and laboratory findings across all *** patients were admitted to the pediatric intensive care unit(PICU)(74%)and required inotropic support(63%)during the second *** of the patients required mechanical circulatory support,and only two required invasive ventilatory *** was no *** The various strains of SARS-CoV-2 triggered MIS-C with differing severities,with the second wave having a more severe clinical *** the differences in disease severity across variants were due to changes in the virus or other factors remains unknown.

关键词： Complications COVID-19 MIS-C SARS-CoV-2 variants

Successful treatment of acute liver failure due to Wilson’s disease: Serendipity or fortuity?

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World Journal of Hepatology 2024年第8期16卷 1111-1119页

作者： Valeria Delle Cave Fabiola Di Dato Pier Luigi Calvo Maria Immacolata Spagnuolo Raffaele Iorio Department of Translational Medical Science Section of PediatricsUniversity of Naples Federico IINaples 80131Italy pediatric Gastroenterology Unit Regina Margherita Children's HospitalAzienda Ospedaliera-Universitaria Cittàdella Salute e della ScienzaTurin 10126Italy

BACKGROUND Acute liver failure(ALF)may be the first and most dramatic presentation of Wilson’s disease(WD).ALF due to WD(WD-ALF)is difficult to distinguish from other causes of liver disease and is a clear indication for liver *** is no firm recommendation on specific and supportive medical treatment for this *** To critically evaluate the diagnostic and therapeutic management of WD-ALF patients in order to improve their survival with native *** A retrospective analysis of patients with WD-ALF was conducted in two pediatric liver units from 2018 to *** During the study period,16 children(9 males)received a diagnosis of WD and 2 of them presented with *** first was successfully treated with an unconventional combination of low doses of D-penicillamine and zinc plus steroids,and survived without liver *** second,exclusively treated with supportive therapy,needed a hepatotransplant to overcome *** Successful treatment of 1 WD-ALF patient with low-dose D-penicillamine and zinc plus steroids may provide new perspectives for management of this condition,which is currently only treated with liver transplantation.

关键词： Fulminant liver failure Copper Chelator Zinc Steroids Liver transplantation Autoimmune hepatitis Prognostic score 24 hour-urinary copper excretion Native liver

Adherence to Pharmacotherapy in Post-Menopausal Women with Hypertension or Metabolic Syndrome: Real World Experience

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International Journal of Clinical Medicine 2024年第3期15卷 145-154页

作者： Maria Maiello Francesca Amati Vincenzo Ezio Santobuono Andrea Igoren Guaricci Cinzia Forleo Marco Matteo Ciccone Pasquale Palmiero Department of cardiology ASL BR SSN3 Brindisi Italy Cardiovascular Diseases section Interdisciplinary Department of Medicine (DIM) University of Bari “Aldo Moro” Bari Italy

Background: Adherence to medications is dependent upon a variety of factors, including individual characteristics of the patient, interactions with health care providers, and medication complexity. Even though several studies were conducted to test intervention strategies, results are uncertain. Aim: The aim of the study is to assess if a tailored combined intervention strategy improves medication adherence in a large population of post-menopausal women affected by hypertension or metabolic syndrome. Methods: We enrolled 6833 patients aged 50 to 69 years, 85.7% with hypertension, and 14.3% with metabolic syndrome. A network between patients, general practitioners, and cardiologists was established. Interventions included education, adequate information to patients, a simplified scheme of treatment, and periodic adherence assessment. These were either delivered as healthcare provider supports or using modern technology. Medication adherence was estimated by the proportion of days covered for all classes of drugs after the index date. Results: Non-adherent hypertensive women were 297 (5%), and those with metabolic syndrome were 73 (7.4%) (p Conclusions: The rate of non-adherence in both settings of postmenopausal women was 7.7%, much lower than that described in the literature. This rate was increased in patients with metabolic syndrome;probably it is related to the complexity of the therapeutic scheme or to a poor consciousness of the disease. Therefore, implementing a tailored combined intervention can improve significantly patients’ adherence to medical therapy.

关键词： Adherence Cardiovascular Prevention Postmenopausal Women Hypertensive Metabolic Syndrome