2,237 results match your criteria Tricuspid Atresia

Pulmonary Atresia with Intact Ventricular Septum: Midterm Outcomes from a Multicenter Cohort.

Pediatr Cardiol 2022 Jun 25. Epub 2022 Jun 25.

Division of Cardiology, Department of Pediatrics, Shawn Jenkins Children's Hospital, Medical University of South Carolina, Charleston, SC, USA.

Contemporary multicenter data regarding midterm outcomes for neonates with pulmonary atresia with intact ventricular septum are lacking. We sought to describe outcomes in a contemporary multicenter cohort, determine factors associated with end-states, and evaluate the effect of right ventricular coronary dependency and coronary atresia on transplant-free survival. Neonates treated during 2009-2019 in 19 United States centers were reviewed. Read More

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Reoperation after modified Björk procedure for tricuspid atresia.

Ann Pediatr Cardiol 2021 Oct-Dec;14(4):530-532. Epub 2022 Mar 25.

Department of Cardiovascular Surgery, Faculty of Medicine of Tunis, La Rabta Hospital, University of Tunis El Manar, Tunis, Tunisia.

Rarely performed nowadays, the Björk procedure is an alternative to the Fontan palliation for biventricular repair in patients with tricuspid atresia. The right atrium to right ventricle conduit failure leads to serious long-term complications. The management of such patients at high surgical risk remains challenging. Read More

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Considerations in Critical-Care and Anesthetic Management of Adult Patients Living With Fontan Circulation.

Can J Cardiol 2022 Apr 28. Epub 2022 Apr 28.

Toronto General Hospital, University Health Network, Toronto, Ontario, Canada. Electronic address:

The Fontan procedure is a staged palliation for various complex congenital cardiac lesions, including tricuspid atresia, pulmonary atresia, hypoplastic left heart syndrome, and double-inlet left ventricle, all of which involve a functional single-ventricle physiology. The complexity of the patients' original anatomy combined with the anatomic and physiologic consequences of the Fontan circulation creates challenges. Teens and adults living with Fontan palliation will need perioperative support for noncardiac surgery, peripartum management for labour and delivery, interventions related to their structural heart disease, electrophysiology procedures, pacemakers, cardioversions, cardiac surgery, transplantation, and advanced mechanical support. Read More

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Novel echocardiographic score to predict duct-dependency after percutaneous relief of critical pulmonary valve stenosis/atresia.

Echocardiography 2022 May 24;39(5):724-731. Epub 2022 Apr 24.

Paediatric Cardiology Unit, University of Campania "Luigi Vanvitelli", A.O.R.N. "Ospedali dei Colli", Naples, Italy.

Objectives: This study aimed to identify clinical, hemodynamic, or echocardiographic predictive features of persistent duct-dependency of pulmonary circulation (PDDPC) after effective percutaneous relief of pulmonary atresia with the intact ventricular septum (PA-IVS) or critical pulmonary stenosis (CPS).

Methods: From 2010 to 2021, 55 neonates with PA-IVS or CPS underwent percutaneous right ventricle (RV) decompression at our Institution. After successfully relief of critical obstruction, 27 patients (group I) showed PDDPC, whereas RV was able to support the pulmonary circulation in the remaining 28 patients (group II). Read More

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Tricuspid atresia with absent pulmonary valve: A rare form of single ventricle.

J Card Surg 2022 Jul 17;37(7):1854-1860. Epub 2022 Apr 17.

Department of Cardiothoracic Surgery, Division of Congenital Cardiothoracic Surgery, Hassenfeld Children's Hospital, New York University, New York, New York, USA.

Tricuspid atresia with an absent pulmonary valve is a rare congenital cardiac defect. Although extensive pathological reviews have been published in the past, there are only a handful of cases that have been successfully palliated to the stage of Fontan. We hereby describe the successful management of one such case and review the surgical strategies described in the literature. Read More

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Pulmonary atresia with intact ventricular septum: Intended strategies.

J Thorac Cardiovasc Surg 2022 Mar 16. Epub 2022 Mar 16.

Division of Cardiothoracic Surgery, Children's Hospital of Philadelphia, Philadelphia, Pa; Department of Biomedical and Health Informatics, Data Science and Biostatistics Unit, Children's Hospital of Philadelphia, Philadelphia, Pa. Electronic address:

Objective: Neonatal interventional strategies for pulmonary atresia with intact ventricular septum are based on tricuspid valve hypoplasia and right ventricle-dependent coronary circulation. We sought to evaluate long-term outcomes comparing biventricular (BiV) versus single-ventricle (SV) strategies.

Methods: Retrospective review was performed of 119 patients diagnosed with pulmonary atresia with intact ventricular septum from 1995 to 2018. Read More

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Type I and II pulmonary atresia with intact ventricular septum in infants: a 10-year experience in initial surgery at one center.

BMC Cardiovasc Disord 2022 03 17;22(1):112. Epub 2022 Mar 17.

Department of Cardiac Surgery, the Second Hospital of Hebei Medical University, No. 215, Heping West Road, Shijiazhuang, Hebei, China.

Background: To explore the effect of initial surgery for type I and II pulmonary atresia with intact ventricular septum (PA/IVS).

Methods: 50 children with type I PA/IVS and 50 with type II PA/IVS who had undergone initial surgery were enrolled. Children with Type I were divided into groups A (n = 25) and B (n = 25). Read More

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Tracheobronchial Release for Left Bronchus Compression After Aortic Arch Repair.

World J Pediatr Congenit Heart Surg 2022 Jul 9;13(4):525-527. Epub 2022 Mar 9.

Division of Cardiovascular and Thoracic Surgery, 6186University of Oklahoma Health Sciences Center, Oklahoma City, OK, USA.

Narrowing of the retroaortic space after repair of common arterial trunk (CAT) with interrupted aortic arch (IAA) is a well-known issue. We present a newborn with CAT, IAA, and functionally univentricular heart (tricuspid atresia) who underwent repair of CAT, IAA, and left ventricle-to-pulmonary artery (LV-to-PA) conduit placement with the Lecompte maneuver. The patient suffered from left bronchus compression postoperatively, which was relieved by tracheobronchial release. Read More

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Infective endocarditis in a cohort of adult CHD patients.

Cardiol Young 2022 Mar 4:1-6. Epub 2022 Mar 4.

Cardiology Department, Hospital de Santa Marta, Centro Hospitalar Universitário de Lisboa Central, Lisbon, Portugal.

Background: CHD increases the risk of infective endocarditis due to the substrate of prosthetic materials and residual lesions. However, lesion-specific and mortality risks data are lacking. We sought to analyse clinical course and mortality of infective endocarditis in a cohort of adult CHD. Read More

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Evaluating the proportion of isolated cases among a spectrum of birth defects in a population-based registry.

Birth Defects Res 2022 Feb 26. Epub 2022 Feb 26.

Birth Defects Epidemiology and Surveillance Branch, Texas Department of State Health Services, Austin, Texas, USA.

Introduction: Because the etiology and outcomes of birth defects may differ by the presence vs. absence of co-occurring anomalies, epidemiologic studies often attempt to classify cases into isolated versus non-isolated groupings. This report describes a computer algorithm for such classification and presents results using data from the Texas Birth Defects Registry (TBDR). Read More

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February 2022

Postmortem 9.4-T MRI for Fetuses With Congenital Heart Defects Diagnosed in the First Trimester.

Front Cardiovasc Med 2021 27;8:764587. Epub 2022 Jan 27.

Department of Obstetrics and Gynecology, The Affiliated Drum and Tower Hospital of Medical School of Nanjing University, Nanjing, China.

Objective: To evaluate the feasibility of 9. 4-T postmortem MRI (pm-MRI) for assessment of major congenital heart defects (CHD) cases terminated in the early stage of gestation.

Methods: Fetuses with CHD detected by the detailed first-trimester ultrasound scan and terminated before 18 gestational weeks were recruited between January 2018 and June 2020. Read More

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January 2022

Juxtaposition of atrial appendages: Evaluation of cardiovascular morphology and associated anomalies on multidetector computed tomography angiography.

J Card Surg 2022 May 9;37(5):1145-1152. Epub 2022 Feb 9.

Department of Cardiovascular Radiology and Endovascular Interventions, All India Institute of Medical Sciences, New Delhi, India.

Aim: To evaluate the morphology and associated cardiovascular abnormalities in patients with juxtaposition of atrial appendages (JAA) on multidetector computed tomography (MDCT) angiography.

Materials And Methods: We performed the retrospective study at a tertiary referral center to identify patients diagnosed with JAA on MDCT angiography using dual-source CT scanner between January 2014 and April 2021. The various imaging abnormalities evaluated included the type of JAA, morphological and positional classification of JAA, atrial situs, cardiac position, atrioventricular and ventriculoarterial connections, great vessel relationship, and other associated cardiovascular anomalies. Read More

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Juxtaposition of the Atrial Appendages: A Large Echocardiographic Series.

Pediatr Cardiol 2022 Jun 2;43(5):1094-1103. Epub 2022 Feb 2.

Mount Sinai Hospital, 1 Gustave L Levy Place, New York, NY, 10029, USA.

Juxtaposition of the atrial appendages is known to occur in specific congenital heart lesions. Recognition of these variants is critical in balloon atrial septostomy and atrial switch operations. There remains little clinical data on the prevalence and associations of these lesions. Read More

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Tricuspid atresia in adulthood.

Eur Heart J Case Rep 2022 Jan 27;6(1):ytab474. Epub 2021 Nov 27.

Department of Neuroscience, Imaging and Clinical Sciences, Institute for Advanced Biomedical Technologies, "G. d'Annunzio" University of Chieti-Pescara, Via Luigi Polacchi, 11, Chieti 66100, Italy.

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January 2022

Exercise Intolerance, Benefits, and Prescription for People Living With a Fontan Circulation: The Fontan Fitness Intervention Trial (F-FIT)-Rationale and Design.

Front Pediatr 2021 6;9:799125. Epub 2022 Jan 6.

Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia.

Despite developments in surgical techniques and medical care, people with a Fontan circulation still experience long-term complications; non-invasive therapies to optimize the circulation have not been established. Exercise intolerance affects the majority of the population and is associated with worse prognosis. Historically, people living with a Fontan circulation were advised to avoid physical activity, but a small number of heterogenous, predominantly uncontrolled studies have shown that exercise training is safe-and for unique reasons, may even be of heightened importance in the setting of Fontan physiology. Read More

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January 2022

Fetal Tricuspid Valve Agenesis/Atresia: Testing Predictions of the Embryonic Etiology.

Pediatr Cardiol 2022 Apr 6;43(4):796-806. Epub 2022 Jan 6.

Department of Medical Biology, Amsterdam Cardiovascular Sciences, Amsterdam University Medical Centres, 1105 AZ, Amsterdam, The Netherlands.

Tricuspid valve agenesis/atresia (TVA) is a congenital cardiac malformation where the tricuspid valve is not formed. It is hypothesized that TVA results from a failure of the normal rightward expansion of the atrioventricular canal (AVC). We tested predictions of this hypothesis by morphometric analyses of the AVC in fetal hearts. Read More

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Common Arterial Trunk Associated with Functionally Univentricular Heart: Anatomical Study and Review of the Literature.

J Cardiovasc Dev Dis 2021 Dec 6;8(12). Epub 2021 Dec 6.

Congenital and Pediatric Cardiology Unit, M3C-Necker, Hôpital Necker-Enfants Malades, AP-HP, 75015 Paris, France.

Common arterial trunk (CAT) is a rare congenital heart disease that is commonly included into the spectrum of conotruncal heart defects. CAT is rarely associated with functionally univentricular hearts, and only few cases have been described so far. Here, we describe the anatomical characteristics of CAT associated with a univentricular heart diagnosed in children and fetuses referred to our institution, and we completed the anatomical description of this rare condition through an extensive review of the literature. Read More

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December 2021

Concomitant Single-Stage Unifocalization and Cavopulmonary Anastomosis (Glenn Shunt) in an Adolescent Patient With Univentricular Physiology and Major Aortopulmonary Collateral Arteries: A Technically Challenging Case.

Cureus 2021 Dec 8;13(12):e20260. Epub 2021 Dec 8.

Internal Medicine, Shree Krishna Hospital, Anand, IND.

Long-segment pulmonary atresia (PA), non-confluent branch pulmonary arteries, ventricular septal defect, tricuspid valve atresia (type 1A), and single ventricle physiology is a relatively rare and extremely heterogeneous form of congenital heart disease. This subset of patients having pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries (MAPCAs) have to undergo multiple unifocalization staging operations before a complete repair is attempted. Most of the patients were deemed inoperable. Read More

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December 2021

Mechanical thrombectomy of COVID-19 DVT with congenital heart disease leading to phlegmasia cerulea dolens: a case report.

BMC Cardiovasc Disord 2021 12 9;21(1):592. Epub 2021 Dec 9.

Department of Internal Medicine/Division of Cardiology, UCLA, 757 Westwood Ave, Ste 2125, Los Angeles, CA, 90095, USA.

Background: COVID-19 and Fontan physiology have each been associated with an elevated risk of venous thromboembolism (VTE), however little is known about the risks and potential consequences of having both.

Case Presentation: A 51 year old male with tricuspid atresia status post Fontan and extracardiac Glenn shunt, atrial flutter, and sinus sick syndrome presented with phlegmasia cerulea dolens (PCD) of the left lower extremity in spite of supratherapeutic INR in the context of symptomatic COVID-10 pneumonia. He was treated with single session, catheter directed mechanical thrombectomy that was well-tolerated. Read More

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December 2021

Influence of Shunt Type on Survival and Right Heart Function after the Norwood Procedure for Aortic Atresia.

Semin Thorac Cardiovasc Surg 2021 Nov 25. Epub 2021 Nov 25.

Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universität München, Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Munich, Germany.

The study objective was to compare the results after Norwood procedure between modified Blalock-Taussig shunt (MBTS) and right ventricle-to-pulmonary artery conduit (RVPAC) according to Sano in patients with hypoplastic left heart syndrome (HLHS) and aortic atresia (AA). A total of 146 neonates with HLHS and AA who underwent the Norwood procedure at our institution between 2001 and 2020 were divided into 2 groups according to shunt type (MBTS or RVPAC). Survival after the Norwood procedure was compared between the groups. Read More

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November 2021

Valve-Sparing Reimplantation for Right Coronary Artery Compression after Fontan Procedure.

Ann Thorac Surg 2021 Nov 25. Epub 2021 Nov 25.

Department of Cardiovascular Surgery, Faculty of Medical Sciences, Kyushu University, Fukuoka, Japan. Electronic address:

An incidental finding of right coronary artery compression, between the dilated aortic root and the sternum, was detected on follow-up cardiac catheterization after Fontan completion for tricuspid atresia with transposition of the great arteries. Although this 31-year-old man had no subjective symptoms, an exercise stress test and perfusion scan revealed ischemic changes in the inferior region. Valve-sparing aortic root replacement using the reimplantation technique proved to be an effective approach for this condition. Read More

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November 2021

Anesthetic Management of Resection of Metastatic Occipital Malignancy in a Patient With Fontan Physiology.

Cureus 2021 Oct 11;13(10):e18662. Epub 2021 Oct 11.

Department of Anaesthesiology, University of Florida College of Medicine, Gainesville, USA.

Significant advances in surgical management have allowed patients with congenital heart disease to survive to adulthood. Often, these patients present for non-cardiac surgeries, including patients who have undergone the three-staged Fontan repair for congenital single ventricle. The primary aim in the anesthetic management of adult patients with Fontan physiology is to maintain adequate venous pressure, low pulmonary vascular resistance (PVR) and normal contractility to maintain the cardiac output. Read More

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October 2021

Severe COVID-19 acute respiratory distress syndrome in an adult with single-ventricle physiology: a case report.

BMC Anesthesiol 2021 11 13;21(1):280. Epub 2021 Nov 13.

Department of Anaesthesiology, Intensive Care Medicine and Pain Therapy, Justus Liebig University of Giessen, Rudolf-Buchheim-Strasse 7, 35392, Giessen, Germany.

Background: COVID-19 can induce acute respiratory distress syndrome (ARDS). In patients with congenital heart disease, established treatment strategies are often limited due to their unique cardiovascular anatomy and passive pulmonary perfusion.

Case Presentation: We report the first case of an adult with single-ventricle physiology and bidirectional cavopulmonary shunt who suffered from severe COVID-19 ARDS. Read More

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November 2021

Multiple Organ Failure Associated with SARS-CoV-2 Infection in a Child with Down Syndrome: Is Trisomy 21 Associated with an Unfavourable Clinical Course?

Case Rep Pediatr 2021 1;2021:5893242. Epub 2021 Nov 1.

Clinical Epidemiology Medical Research Unit, National Medical Center "Siglo XXI", Mexican Institute of Social Security, Mexico City 06720, Mexico.

Introduction: Down syndrome (DS) is one of the most frequent genomic disorders around the globe (∼1:700 births). During the COVID-19 pandemic, it has been recognized that children with DS are patients with a greater risk of presenting SARS-CoV-2 infection-related poor outcomes. Nonetheless, a few cases with DS and SARS-CoV-2 infection have been reported. Read More

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November 2021

First trimester prenatal diagnosis of a severe tricuspid valve regurgitation and pulmonary valve atresia.

Echocardiography 2021 12 7;38(12):2119-2121. Epub 2021 Nov 7.

Division of Pediatric Cardiology, National Research Center - Tuscany Region Foundation "G. Monasterio", Ospedale del Cuore, Massa, Italy.

Early diagnosis of congenital heart defect (CHD) increased in the last two decades, following technological evolution. A recent meta-analysis (Yu, 2020), on diagnostic accuracy in ultrasound detection of major CHD in the first trimester of pregnancy, reported an overall sensitivity of 75%. Ultrasound imaging of this case refers to a tricuspid valve dysplasia with right atriomegaly and pulmonary valve atresia diagnosed in a 13-week gestational-age fetus with low risk for chromosomal abnormalities. Read More

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December 2021

Umbilical artery to common femoral artery (CFA) transposition, a novel technique for limb salvage in a newborn: a case report.

Turk J Pediatr 2021;63(5):932-936

Department of Pediatric Surgery, Shiraz University of Medical Sciences, Shiraz, Iran.

Background: Cardiac catheterization is a course of action with a low rate of complication; however, the potential risk factors should be considered before the procedure. The risk of arterial complications increases in sick premature infants, especially in the first days of life.

Case: A four-day-old neonate with cyanotic heart disease (tricuspid atresia) was referred to our tertiary center for patent ductus arteriosus (PDA) stenting by cardiac catheterization. Read More

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January 2022

Cohort study of intervened functionally univentricular heart in England and Wales (2000-2018).

Heart 2022 Jun 10;108(13):1046-1054. Epub 2022 Jun 10.

Institute of Cardiovascular Science, University College London, London, UK

Objective: Given the paucity of long-term outcome data for complex congenital heart disease (CHD), we aimed to describe the treatment pathways and survival for patients who started interventions for functionally univentricular heart (FUH) conditions, excluding hypoplastic left heart syndrome.

Methods: We performed a retrospective cohort study using all procedure records from the National Congenital Heart Diseases Audit for children born in 2000-2018. The primary outcome was mortality, ascertained from the Office for National Statistics in 2020. Read More

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Clinical features and echocardiographic findings of isolated foramen ovale restriction in foetuses.

J Obstet Gynaecol 2021 Oct 27:1-5. Epub 2021 Oct 27.

Faculty of Medicine, Deparment of Pediatric Cardiology, Çukurova University, Adana, Turkey.

Isolated restrictive foramen ovale (rFO) without complex heart defects is a rare pathology. There may be difficulties in managing this situation, which can lead to right heart enlargement, tricuspid regurgitation and hydrops findings in the foetus. We conducted a retrospective analysis of 8451 foetuses. Read More

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October 2021

Emergency Department Presentation and Outcome of Children With Cyanotic Congenital Heart Diseases.

Cureus 2021 Sep 14;13(9):e17960. Epub 2021 Sep 14.

Paediatric Cardiology, Lady Reading Hospital, Medical Teaching Institute, Peshawar, PAK.

Objective This study aimed to assess the outcome and see the spectrum of presenting symptoms of children with cyanotic congenital heart diseases (CHDs) admitted through the emergency department in Lady Reading Hospital (LRH) in Peshawar, Pakistan. Materials and methods This cross-sectional study included 104 children with cyanotic CHD admitted through the emergency department in the pediatric cardiology unit of LRH from February 2019 to January 2020. Diagnosis of cyanotic CHD was confirmed through two-dimensional echocardiography. Read More

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September 2021

[Interim follow-up of fetal cardiac intervention in five fetuses with pulmonary atresia with intact ventricular septum].

Zhonghua Er Ke Za Zhi 2021 Sep;59(9):782-786

Department of Obstetrics, Women and Children's Hospital, Qingdao University, Qingdao 266034, China.

To summarize the interim outcome and right heart development of pulmonary atresia with intact ventricular septum (PA-IVS) in children after fetal cardiac intervention (FCI). The clinical data of 5 live births underwent FCI from October 2018 to April 2019 in Women and Children's Hospital, Qingdao University were analyzed retrospectively. The development of right ventricle (RV) and tricuspid valve (TV) in uterus after FCI, at birth, the age of 6 months, 1 year and 2 years, and the final outcome were assessed. Read More

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September 2021