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The process of valve formation is a complex process that involves intricate interplay between various pathways at precise times. Although we have not completely elucidated the molecular pathways that lead to normal valve formation, we have identified a few major players in this process. We are now able to implicate TGF-ß, BMP, and NOTCH as suspects in tricuspid atresia (TA), as well as their downstream targets: NKX2-5, TBX5, NFATC1, GATA4, and SOX9. We know that the TGF-ß and the BMP pathways converge on the SMAD4 molecule, and we believe that this molecule plays a very important role to tie both pathways to TA. Similarly, we look at the NOTCH pathway and identify the HEY2 as a potential link between this pathway and TA. Another transcription factor that has been implicated in TA is NFATC1. While several mouse models exist that include part of the TA abnormality as their phenotype, no true mouse model can be said to represent TA. Bridging this gap will surely shed light on this complex molecular pathway and allow for better understanding of the disease process.
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Modelos Animais de Doenças , Transdução de Sinais , Atresia Tricúspide , Animais , Atresia Tricúspide/genética , Atresia Tricúspide/metabolismo , Atresia Tricúspide/patologia , Humanos , Camundongos , Coração Univentricular/genética , Coração Univentricular/metabolismo , Coração Univentricular/fisiopatologia , Coração Univentricular/patologia , Fatores de Transcrição NFATC/metabolismo , Fatores de Transcrição NFATC/genética , Fator de Crescimento Transformador beta/metabolismo , Fator de Crescimento Transformador beta/genética , Receptores Notch/metabolismo , Receptores Notch/genéticaRESUMO
In this review we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. Our hope is to provide a summary of the latest articles published recently in other journals in our field. The articles address: 1- The use of AI in fetal echocardiography, 2- The role of Apixaban in thromboembolism prevention in pediatric congenital heart disease, 3- Cardiovascular events in childhood cancer survivors, and lastly 4- the new consensus statement on cardiac catheterization for pediatrics and adults with congenital heart disease.
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In this review, we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. Our hope is to provide a summary of the latest articles published recently in other journals in our field. The articles address (1) A new index for prenatal diagnosis of total anomalous pulmonary venous return, (2) Outcomes of patients with Tetralogy of Fallot after pulmonary valve replacement (PVR), and (3) Short-term outcomes of the self expanding Harmony valve for transcatheter PVR.
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The Harmony™ Transcatheter Pulmonary Valve (Medtronic) was recently approved by the Food and Drug Administration for transcatheter pulmonary valve replacement in native right ventricular outflow tracts. Despite this milestone, some patients have main pulmonary arteries that are severely dilated and continue to require surgical pulmonary valve replacement. The hybrid approach combines surgical creation of a landing zone, transcatheter valve deployment, and suture stabilization of the implanted valve. In this case series, we report the first use of a hybrid approach for Harmony™ transcatheter pulmonary valve replacement. Two cases are reported with varying approaches for surgical creation of a landing zone followed by successful placement of a Harmony™ valve.
Assuntos
Implante de Prótese de Valva Cardíaca , Próteses Valvulares Cardíacas , Insuficiência da Valva Pulmonar , Valva Pulmonar , Humanos , Valva Pulmonar/diagnóstico por imagem , Valva Pulmonar/cirurgia , Insuficiência da Valva Pulmonar/diagnóstico por imagem , Insuficiência da Valva Pulmonar/cirurgia , Implante de Prótese de Valva Cardíaca/efeitos adversos , Resultado do Tratamento , Cateterismo Cardíaco , Desenho de PróteseRESUMO
This review is meant to highlight recent publications from other journals that are relevant to pediatric cardiologists. The articles chosen for this edition look at the outcomes of catheter-based interventions for aortic stenosis, the effect of atherosclerotic cardiovascular disease risk factors on the adult congenital heart disease population, the difference in mortality from congenital heart disease between rural and urban America, preoperative NT-proBNP as a predictor of Fontan outcomes, and an overview of the utilization and outcomes of the Rastelli, Nikaidoh, and REV procedures.
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Percutaneous carotid access (PCA) in infants has been reported in small multicenter cohorts, case reports and wider studies over the last 20 years. Compare outcomes after implementation of a systematic approach to PCA in a single center including an imaging follow-up protocol. Retrospective case-control study of PCA at Children's Hospital Colorado was performed from January 2013 to December 2022. Seventy-four patients underwent 82 PCAs for cardiac catheterization. The median age (range) was 14 days (1-359), and weight was 3.25-kg (1.9-7.9). Median sheath size was 4-Fr (3.3-6). Seventy-seven interventions performed included PDA stenting, aortic valvoplasty, BTT shunt stenting, and coarctation stenting. Vascular access was performed using a modified 21 g butterfly needle. A protocolized approach was implemented in 2020 reversing the patient head-to-toe orientation on the catheterization table, maintaining intubation and sedation for 4-h during recovery and routine use of a specific vascular ultrasound protocol. Following these changes, time to access significantly improved with no major complications. Before 2020, two access related complications occurred. One requiring surgical vascular repair and one occlusive thrombus. A significant increase in sheath time in post-era was associated with increased case complexity. Longer sheath times were not associated with increased risk of vessel injury or thrombus. No neurological insults were reported. Our experience confirms that PCA is safe and achievable with preserved vessel patency regardless of patient weight or sheath size. A protocolized planning, recovery, and follow-up regimen is recommended to establish safe practice and identify and treat complications as necessary.
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Cateterismo Periférico , Trombose , Criança , Humanos , Lactente , Recém-Nascido , Cateterismo Periférico/efeitos adversos , Seguimentos , Estudos Retrospectivos , Estudos de Casos e Controles , Resultado do Tratamento , Stents , Trombose/etiologia , Estudos Multicêntricos como AssuntoRESUMO
In this review, we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. This review includes (1) Fetal diagnosis, associated anomalies, and factors affecting outcomes in fetal congenitally corrected transposition of the great arteries (ccTGA), (2) The impact of Fontan-associated liver disease on heart and heart/liver transplant, (3) 1-year outcomes of the Harmony transcatheter pulmonary valve, (4) Risk factors associated with major adverse cardiac events in patients with pulmonary atresia and intact ventricular septum undergoing intervention, and (5) Benefits of pulmonary valve replacement in tetralogy of Fallot patients.
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Safety and acute outcomes for patients who need catheterization shortly after congenital cardiac surgery has been established; literature on mid-term outcomes is lacking. We sought to evaluate the mid-term outcomes of patients who undergo early postoperative cardiac catheterization. This is a retrospective cohort study of patients who underwent cardiac catheterization within 6 weeks of congenital cardiac surgery with longitudinal follow-up and assessment of mid-term outcomes. Multivariable analysis was utilized to relate variables of interest to outcomes. 99 patients underwent cardiac catheterizations within 6 weeks of cardiac surgery between January 2008 and September 2016. Forty-six (45.5%) interventional procedures were performed at a median age of 41 days (IQR 21-192) and a median weight of 3.9 kg (3.3-6.6). During a median follow-up duration of 4.24 years (1.6-5.6) in study survivors, 61% of patients remained free from the primary endpoint (death and/or transplant). Sixty-nine patients (69.7%) underwent an unplanned surgical or catheter procedure. Renal failure at catheterization (OR 280.5, p 0.0199), inotropic medication at catheterization (OR 14.8, p 0.002), and younger age were all significantly associated with meeting the primary endpoint. Patients requiring surgical intervention as an initial additional intervention underwent more unplanned re-interventions, while patients who survived to hospital discharge demonstrated favorable mortality, though with frequent need for re-intervention. In patients requiring early postoperative cardiac catheterization, renal failure, younger age, and need for inotropic support at catheterization are significantly associated with meeting the primary endpoint.
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Procedimentos Cirúrgicos Cardíacos , Cardiopatias Congênitas , Criança , Humanos , Lactente , Estudos Retrospectivos , Resultado do Tratamento , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Cateterismo Cardíaco/efeitos adversosRESUMO
Transcatheter pulmonary valve replacement (TPVR) is a staple in the pediatric cardiac catheterization lab. Despite the ubiquitous use of this procedure, guidelines for antithrombosis post pulmonary valve replacement are non-existent. A survey was sent out via email to the members of the Congenital Cardiovascular Interventional Study Consortium (CCISC) and the Pediatric and Adult Interventional Cardiac Symposium (PICS-AICS). Responses were received from 109 cardiologists who perform this procedure. Following TPVR 76.5% of respondents use only Aspirin, while the remainder prescribe a second antithrombotic agent at discharge. The majority (78%) of respondents do not educate patients about avoiding enteric formulations of aspirin, and another 86% do not educate patients about the need to avoid proton pump inhibitors (PPI). In addition, 67% do not advise against concomitant use of Non-Steroidal Anti-Inflammatory Drugs (NSAIDs). Lastly, the survey showed a wide discrepancy in prescribed dose with 56% choosing to always prescribe 70-100 mg regardless of weight, 28% choosing to do weight-based dosing, and 7.5% choosing 325 mg regardless of weight. In a survey sent out to pediatric cardiac interventionalists worldwide, a significant discrepancy was noted in antithrombotic regimens used following TPVR. We hypothesize that these discrepancies may contribute to early valve failure and suggest the need for further study and the development of unified antithrombosis guidelines following TPVR.
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Implante de Prótese de Valva Cardíaca , Próteses Valvulares Cardíacas , Valva Pulmonar , Adulto , Aspirina , Cateterismo Cardíaco , Criança , Humanos , Valva Pulmonar/cirurgia , Estudos Retrospectivos , Resultado do TratamentoRESUMO
In cases where femoral access is untenable for secundum atrial septal defect closure, the internal jugular vein can be used instead. We report a case of atrial septal defect closure in a patient with significant vascular thrombosis, requiring the use of the left internal jugular vein for access. To the best of our knowledge, this is the first report that documents the use of the left internal jugular vein for atrial septal defect closure in a patient with situs solitus anatomy.
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Comunicação Interatrial , Dispositivo para Oclusão Septal , Veias Braquiocefálicas , Cateterismo Cardíaco , Comunicação Interatrial/diagnóstico , Comunicação Interatrial/cirurgia , Humanos , Veias Jugulares/diagnóstico por imagem , Veias Jugulares/cirurgia , Resultado do TratamentoRESUMO
BACKGROUND: Polymer-based bioresorbable scaffolds (PBBS) have been assessed for coronary revascularization with mixed outcomes. Few studies have targeted pediatric-specific scaffolds. We sought to assess safety, efficacy, and short-term performance of a dedicated drug-free PBBS pediatric scaffold compared to a standard low-profile bare metal stent (BMS) in central and peripheral arteries of weaned piglets. METHODS: Forty-two devices (22 Elixir poly-L-lactic-acid-based pediatric bioresorbable scaffolds [BRS] [6 × 18 mm] and 20 control BMS Cook Formula 418 [6 × 20 mm]) were implanted in the descending aorta and pulmonary arteries (PAs) of 14 female Yucatan piglets. Quantitative measurements were collected on the day of device deployment and 30 and 90 days postimplantation to compare device patency and integrity. RESULTS: The BRS has a comparable safety profile to the BMS in the acute setting. Late lumen loss (LLL) and percent diameter stenosis (%DS) were not significantly different between BRS and BMS in the PA at 30 days. LLL and %DS were greater for BRS versus BMS in the aorta at 30 days postimplantation (LLL difference: 0.96 ± 0.26; %DS difference: 16.15 ± 4.51; p < .05). At 90 days, %DS in the aortic BRS was less, and PA BRS LLL was also less than BMS. Histomorphometric data showed greater intimal proliferation and area stenosis in the BRS at all time points and in all vessels. CONCLUSIONS: A dedicated PBBS pediatric BRS has a favorable safety profile in the acute/subacute setting and demonstrates characteristics that are consistent with adult BRSs.
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Aorta Abdominal/patologia , Procedimentos Endovasculares/instrumentação , Metais , Poliésteres , Artéria Pulmonar/patologia , Stents , Angiografia , Animais , Animais Recém-Nascidos , Aorta Abdominal/diagnóstico por imagem , Proliferação de Células , Constrição Patológica , Procedimentos Endovasculares/efeitos adversos , Estudos de Viabilidade , Feminino , Teste de Materiais , Neointima , Desenho de Prótese , Artéria Pulmonar/diagnóstico por imagem , Suínos , Porco Miniatura , Fatores de Tempo , Ultrassonografia de IntervençãoRESUMO
We carried out a device-needs survey to evaluate the gaps in device and equipment availability for congenital interventional cardiologists. As the complexity and demand for more complete solutions to congenital heart lesions increase, there is a growing need for modification and development of devices and equipment to support this endeavor. The survey was sent out via e-mail to members of the Congenital Cardiovascular Interventional Study Consortium and the Society for Cardiac Angiography and Interventions with a reach of over 350 congenital interventionalists. Responses were received from 68 cardiologists in 8 countries. In terms of the most desired device, 41 % ranked bioresorbable stents as their first choice from a list of 12 possible devices. Similarly, 23 % ranked large covered stents as their first choice. Twenty-seven percent of participants believed bioresorbable stents would have the greatest potential to improve morbidity of their patients, with another 27 % reporting that covered stents would have the greatest impact. Fifty percent of participants reported that they would like to see large covered stents available in their country. These data point toward a perceived need for the development/approval of bioresorbable stents for the pediatric age group, as well as the need to approve the use of large covered stents in the pediatric age group in the USA.
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Cardiopatias/congênito , Cardiologistas , Humanos , Stents , Inquéritos e QuestionáriosRESUMO
Although proteomics has been exploited in a wide range of diseases for identification of biomarkers and pathophysiological mechanisms, there are still biomedical disciplines such as otology where proteomics platforms are underused due to technical challenges and/or complex features of the disease. Thus, in the past few years, healthcare and scientific agencies have advocated the development and adoption of proteomic technologies in otological research. However, few studies have been conducted and limited literature is available in this area. Here, we present the state of the art of proteomics in otology, discussing the substantial evidence from recent experimental models and clinical studies in inner-ear conditions. We also delineate a series of critical issues including minute size of the inner ear, delicacy and poor accessibility of tissue that researchers face while undertaking otology proteomics research. Furthermore, we provide perspective to enhance the impact and lead to the clinical implementation of these proteomics-based strategies.
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Orelha Interna/fisiopatologia , Proteínas/metabolismo , Proteômica , Animais , Biomarcadores , Orelha Interna/crescimento & desenvolvimento , Humanos , Ligação ProteicaRESUMO
We present a case of venous thoracic outlet syndrome involving upper extremity venous thrombosis confirmed by hyperabduction during balloon inflation in the subclavian vein. This provocative test provides clear evidence of extrinsic venous compression, confirming venous thoracic outlet syndrome.
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Sports participation in patients with congenital heart disease is an evolving subject. The American Heart Association/American College of Cardiology released a set of guidelines that advise the type and level of sports participation based primarily on anatomical defects with secondary consideration given to hemodynamic effects. Recently, the European Association of Preventive Cardiology/European Society of Cardiology/Association for European Paediatric and Congenital Cardiology offered a contrasting approach to sports participation that is based on hemodynamic and electrophysiological profiles of each patient, regardless of anatomical consideration. These guidelines are drastically different in their approaches but do have some similarities. In this review, we compare both documents, focusing on the aim, population, classification of sports, and the methodology of making recommendations. This review aims to assist practicing cardiologists in integrating the available published data and recommendations when counseling patients for sports participation.
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Cardiologistas , Cardiologia , Cardiopatias Congênitas , Esportes , Estados Unidos/epidemiologia , Humanos , Criança , Eletrofisiologia Cardíaca , American Heart Association , Cardiopatias Congênitas/terapiaRESUMO
There has been a paradigm shift in the management of patients with congenital heart disease with a move away from conventional surgical treatment in favor of a percutaneous catheter-based approach across the spectrum of valvular heart diseases. The Sapien S3 valve implantation in the pulmonary position has been previously reported using a conventional transcatheter approach in patients with pulmonary insufficiency due to an enlarged right ventricular outflow tract. In this report, we present 2 unique cases of intraoperative hybrid implantation of Sapien S3 valves in patients with complex pulmonic and tricuspid valvular disease.
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Doenças das Valvas Cardíacas , Implante de Prótese de Valva Cardíaca , Próteses Valvulares Cardíacas , Insuficiência da Valva Pulmonar , Valva Pulmonar , Humanos , Cateterismo Cardíaco/métodos , Catéteres , Doenças das Valvas Cardíacas/diagnóstico por imagem , Doenças das Valvas Cardíacas/cirurgia , Implante de Prótese de Valva Cardíaca/métodos , Desenho de Prótese , Valva Pulmonar/cirurgia , Insuficiência da Valva Pulmonar/cirurgia , Resultado do Tratamento , Masculino , Feminino , Adolescente , AdultoRESUMO
Background Prolonged exposure to a hemodynamically significant patent ductus arteriosus (PDA) is associated with major morbidity, particularly in infants born at <27 weeks' gestation. High-frequency jet ventilation (HFJV) is a standard of care at our center. There are no data about transcatheter PDA closure while on HFJV. The aim of this study was to assess the feasibility, safety, and outcomes of HFJV during transcatheter PDA closure. Methods and Results This is a retrospective cohort study of premature infants undergoing transcatheter device closure on HFJV. The primary outcome was successful device placement. Secondary outcomes included procedure time, fluoroscopy time and dose, time off unit, device complications, need for escalation in respiratory support, and 7-day survival. Subgroup comparative evaluation of patients managed with HFJV versus a small cohort of patients managed with conventional mechanical ventilation was performed. Thirty-eight patients were included in the study. Median age and median weight at PDA device closure for the HFJV cohort were 32 days (interquartile range, 25.25-42.0 days) and 1115 g (interquartile range, 885-1310 g), respectively. There was successful device placement in 100% of patients. There were no device complications noted. The time off unit and the procedure time were not significantly different between the HFJV group and the conventional ventilation group. Infants managed by HFJV had shorter median fluoroscopy times (4.5 versus 6.1 minutes; P<0.05) and no increased risk of adverse respiratory outcomes. Conclusions Transcatheter PDA closure in premature infants on HFJV is a safe and effective approach that does not compromise device placement success rate and does not lead to secondary complications.
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Permeabilidade do Canal Arterial , Ventilação em Jatos de Alta Frequência , Cateterismo Cardíaco/métodos , Permeabilidade do Canal Arterial/cirurgia , Estudos de Viabilidade , Ventilação em Jatos de Alta Frequência/efeitos adversos , Humanos , Lactente , Recém-Nascido , Recém-Nascido Prematuro , Estudos Retrospectivos , Resultado do TratamentoRESUMO
A novel coronary anatomy in the form of anomalous left anterior descending coronary artery from pulmonary artery with a retroaortic left circumflex arising from the right coronary artery is presented. This unreported anatomy was discovered in a 7-month-old girl with failure to thrive. (Level of Difficulty: Intermediate.).
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We present a rare case of double-outlet right ventricle with pulmonary atresia and discontinuous branch pulmonary arteries supplied by bilateral ducti from a right aortic arch. To our knowledge, this is only the second documented case of double-outlet right ventricle with bilateral ducti. (Level of Difficulty: Advanced.).