2,188 results match your criteria Tricuspid Atresia


The prenatal diagnosis of a rare circular shunt with absent pulmonary valve syndrome.

J Clin Ultrasound 2021 Jun 16. Epub 2021 Jun 16.

Department of Pediatrics, Kindai University, Faculty of Medicine, Osaka-Sayama, Osaka, Japan.

Absent pulmonary valve syndrome (APVS) with tricuspid atresia is very rare. We report a case of APVS in which the fetus died due to severe hydrops fetalis. In addition to weakened cardiac contraction due to left ventricular noncompaction cardiomyopathy, a circular shunt through a ventricular septal defect exacerbated heart failure. Read More

View Article and Full-Text PDF

[Stent ductal implant by axillary via in a newborn with ductal dependent pulmonary blood flow].

Andes Pediatr 2021 Apr;92(2):274-280

Facultad de Medicina, Universidad CES, Medellin, Colombia.

Introduction: Ductus arteriosus stenting is a palliative alternative for neonates with ductal-dependent pulmonary flow.

Objective: To present an alternative of arterial access for percutaneous coronary intervention in neonates.

Clinical Case: A term neonate with low weight diagnosed with pulmonary atresia with intact ventricular septum and severe hypoplasia of the tricuspid valve with dependent coronary circu lation. Read More

View Article and Full-Text PDF

Characterization of hemodynamic patterns in human fetuses with cyanotic congenital heart disease using MRI.

Ultrasound Obstet Gynecol 2021 Jun 7. Epub 2021 Jun 7.

Division of Paediatric Cardiology, Department of Paediatrics, Hospital for Sick Children, University of Toronto, Toronto, Canada.

Objectives: To characterize the distribution of blood flow and oxygen transport in human fetuses with congenital heart disease (CHD) subtypes that present with neonatal cyanosis using magnetic resonance imaging (MRI).

Methods: Blood flow was measured in the major vessels of 152 late gestation human fetuses with CHD and 40 gestational age-matched normal fetuses using cine phase contrast MRI. Oxygen saturation (SaO2) was measured in the major vessels of 57 fetuses with CHD and 40 controls. Read More

View Article and Full-Text PDF

Absent pulmonary valve with tricuspid atresia/stenosis: literature review with new three long-term cases.

Heart Vessels 2021 Jun 5. Epub 2021 Jun 5.

Department of Pediatric Cardiology, Osaka City General Hospital, Osaka, Japan.

Absent pulmonary valve (APV) syndrome with tricuspid atresia or tricuspid stenosis (TA/TS) is an extremely rare malformation recently reported as a variant of APV with intact ventricular septum (VS). The condition, however, has univentricular physiology and unique structural and clinical features. The purpose of this study was to update the current knowledge about this condition by describing long-term outcomes of three new cases and reviewing the available literatures. Read More

View Article and Full-Text PDF

Single Ventricle-A Comprehensive Review.

Children (Basel) 2021 May 24;8(6). Epub 2021 May 24.

McGovern Medical School, University of Texas-Houston, Children's Memorial Hermann Hospital, 6410 Fannin Street, UTPB Suite # 425, Houston, TX 77030, USA.

In this paper, the author enumerates cardiac defects with a functionally single ventricle, summarizes single ventricle physiology, presents a summary of management strategies to address the single ventricle defects, goes over the steps of staged total cavo-pulmonary connection, cites the prevalence of inter-stage mortality, names the causes of inter-stage mortality, discusses strategies to address the inter-stage mortality, reviews post-Fontan issues, and introduces alternative approaches to Fontan circulation. Read More

View Article and Full-Text PDF

Factors associated with mortality or transplantation versus Fontan completion after cavopulmonary shunt for patients with tricuspid atresia.

J Thorac Cardiovasc Surg 2021 Apr 30. Epub 2021 Apr 30.

Division of Pediatric Cardiology, The Hospital for Sick Children, Toronto, Ontario Canada. Electronic address:

Objective: Tricuspid atresia with normally related great vessels (TA) is considered the optimal substrate for the Fontan pathway. The factors associated with death or transplantation after cavopulmonary shunt (CPS) are underappreciated. We aimed to determine factors associated with CPS-Fontan interstage death/transplantation versus transition to Fontan in TA. Read More

View Article and Full-Text PDF

A unique arrangement of "kissing" atrial appendages.

J Card Surg 2021 May 25. Epub 2021 May 25.

Department of Cardiology, All India Institute of Medical Sciences, New Delhi, India.

We report a case of a 2-month-old boy with tricuspid and pulmonary atresia with a unique configuration of "kissing" atrial appendages. The case highlights the importance of computed tomography angiography in identifying such anomalies associated with complex congenital heart defects. Read More

View Article and Full-Text PDF

Effects of universal critical CHD screening of neonates at a mid-sized California congenital cardiac surgery centre.

Cardiol Young 2021 May 24:1-8. Epub 2021 May 24.

Division of Cardiology, Loma Linda University Children's Hospital, 11234 Anderson St, Loma Linda, CA92354, USA.

Introduction: CHD affects over 1 million children in the United States. Studies show decreased mortality from CHD with newborn cardiac screening. California began a screening programme on 1 July, 2013. Read More

View Article and Full-Text PDF

Tricuspid Atresia Type 1B With Persistent Left Superior Vena Cava in a Four-Month-Old Child: An Unusual Combination.

Cureus 2021 Apr 13;13(4):e14450. Epub 2021 Apr 13.

Cardiology, Apex Heart Institute, Ahmedabad, IND.

Tricuspid atresia (TA) is a rare cyanotic congenital heart disease. A persistent left superior vena cava (LSVC) may be associated with TA. The presence of LSVC raises important considerations for eventual repair, in that it may lead to persistent arterial desaturation even after corrective surgery, if associated with an unroofed coronary sinus. Read More

View Article and Full-Text PDF

The Glenn procedure: Clinical outcomes in patients with congenital heart disease in pakistan.

Ann Card Anaesth 2021 Jan-Mar;24(1):30-35

Cardiothoracic Section, Department of Surgery, Aga Khan University Hospital, Karachi, Pakistan.

Objectives: Congenital heart defects (CHDs) affect more than 40,000 children annually in Pakistan. Approximately 80' of patients require at least one surgical intervention to achieve a complete or palliative cardiac repair. The Glenn shunt, a palliative procedure is established between superior vena cava (SVC) and the right pulmonary artery to provide an anastomosis offering minimal risk to patients with univentricular heart disease. Read More

View Article and Full-Text PDF

Myocardial deformation in patients with a single left ventricle using 2D cardiovascular magnetic resonance feature tracking: a case-control study.

Int J Cardiovasc Imaging 2021 Mar 31. Epub 2021 Mar 31.

Department of Congenital Heart Disease and Paediatric Cardiology, University Hospital Schleswig-Holstein, Campus Kiel, Arnold-Heller-Str. 3, 24105, Kiel, Germany.

Ventricular dysfunction is a well-known complication in single ventricle patients in Fontan circulation. As studies exclusively examining patients with a single left ventricle (SLV) are sparse, we assessed left ventricular (LV) function in SLV patients by using 2D-cardiovascular magnetic resonance (CMR) feature tracking (2D-CMR-FT) and 2D-speckle tracking echocardiography (2D-STE). 54 SLV patients (11. Read More

View Article and Full-Text PDF

Biatrial Drainage of the Right Superior Vena Cava: Imaging Findings.

Radiol Cardiothorac Imaging 2020 Dec 10;2(6):e200414. Epub 2020 Dec 10.

Division of Pediatric Cardiology, Department of Pediatrics, University of Texas Health Science Center at Houston, 7000 Fannin Suite 1200, Houston, TX 77030 (M.D.P.); Division of Pediatric Cardiology, Department of Pediatrics (S.B., A.L.D., J.C.L.), Section of Pediatric Radiology, Department of Radiology (S.B., A.L.D., A.J., J.C.L., M.G.M.), and Division of Cardiothoracic Radiology, Department of Radiology (M.G.M., P.P.A.), Michigan Congenital Heart Center, C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, Mich.

Biatrial drainage of the right superior vena cava (SVC) is a rare form of interatrial shunting that can have substantial clinical consequences. Cross-sectional imaging techniques (CT and MRI) are well suited for evaluation and surgical planning. This review article focuses on the embryologic development, hemodynamics, and imaging features to enable a timely diagnosis. Read More

View Article and Full-Text PDF
December 2020

Bilateral coronary ostial atresia in a patient with pulmonary atresia and ventricular septal defect.

Cardiol Young 2021 Apr 8;31(4):644-645. Epub 2021 Mar 8.

Department of Cardiovascular Surgery, Japan Community Health Organization Chukyo Hospital, Minami-ku, Nagoya, Japan.

This is a case of a female infant with bilateral coronary ostial atresia associated with pulmonary atresia and ventricular septal defect. She developed coronary ischemia at 1-month of age, when she underwent an aortopulmonary shunt and an aorta-right ventricle shunt. The double-orifice tricuspid valve was separating the right ventricle from the left ventricle. Read More

View Article and Full-Text PDF

Balloon Valvuloplasty via the Pulmonary Artery Trunk for Treating Neonates With Severe Pulmonary Valve Disease.

Heart Surg Forum 2021 02 19;24(1):E185-E187. Epub 2021 Feb 19.

Department of Cardiac Surgery, Fujian Medical University Union Hospital, Fujian, China.

Background: Percutaneous balloon pulmonary valvuloplasty has proven to be a standard of care for neonates with severe pulmonary valve disease. However, the peripheral vessel injury, tricuspid chordae tendineae rupture, and cardiac tamponade could occur. Recently, we performed balloon valvuloplasty through pulmonary artery trunk. Read More

View Article and Full-Text PDF
February 2021

The failing Fontan.

Indian J Thorac Cardiovasc Surg 2021 Jan 7;37(Suppl 1):82-90. Epub 2020 Mar 7.

Department of Cardiothoracic Surgery, New York University, 530 First Avenue, New York, NY 10016 USA.

Nearly 50 years back, Francis Fontan pioneered an operation for tricuspid atresia that bears his name today. The operation has since undergone numerous modifications and continues to be widely applied to an array of single ventricles. Despite restoring normal oxygen levels in the body, the operation creates a neoportal system where adequate cardiac output can be generated only at the expense of increased systemic venous congestion. Read More

View Article and Full-Text PDF
January 2021

Natural history of pulmonary atresia with intact ventricular septum (PAIVS) and critical pulmonary stenosis (CPS) and prediction of outcome.

Arch Gynecol Obstet 2021 Jul 14;304(1):81-90. Epub 2021 Feb 14.

Division of Prenatal Medicine, Department of Obstetrics and Gynecology, Justus-Liebig-University Giessen and University Hospital Giessen & Marburg, Giessen, Germany.

Objectives: To analyse prenatal parameters predicting biventricular (BV) outcome in pulmonary atresia with intact ventricular septum/critical pulmonary stenosis (PAIVS/CPS).

Methods: We evaluated 82 foetuses from 01/08 to 10/18 in 3 centres in intervals 1 (< 24 weeks), 2 (24-30 weeks) and 3 (> 30 weeks).

Results: 61/82 (74. Read More

View Article and Full-Text PDF

A 40-Year-Old Man With Tricuspid Atresia, Status Post-Fontan, With Severe COVID-19 Pneumonia and Pneumothorax.

JACC Case Rep 2021 Feb 3;3(2):187-191. Epub 2021 Feb 3.

Department of Medicine, Division of Cardiovascular Medicine, Stanford University School of Medicine, Palo Alto, California, USA.

We report a case of COVID-19 in an adult single-ventricle patient post-Fontan-to our knowledge, the first report in this population documenting the use of the latest management recommendations for this novel disease. Additionally, this patient had significant pre-existing ventricular dysfunction, valvular disease, and comorbidities including HIV. (). Read More

View Article and Full-Text PDF
February 2021

Glenn surgery: a safe procedure in the path of univentricular correction.

Bol Med Hosp Infant Mex 2021 01 21;78(2):123-129. Epub 2021 Jan 21.

Unidad de Investigación en Epidemiología Clínica, Unidad Médica de Alta Especialidad, Hospital de Pediatría, Centro Médico Nacional Siglo XXI, Instituto Mexicano del Seguro Social. Mexico City, Mexico.

Background: This study describes 35 years of experience in a tertiary care level hospital that treats cardiac patients with univentricular heart physiology who underwent Glenn surgery.

Methods: The study consisted of a retrospective analysis of patients who underwent Glenn surgery, including variables related to pre-operative, intra-operative, and post-operative morbidity and mortality.

Results: From 1980 to 2015, 204 Glenn surgeries were performed. Read More

View Article and Full-Text PDF
January 2021

Complex congenital heart diseases among children presenting for cardiac surgery in a tertiary health facility in Enugu; South-East Nigeria. A rising trend.

Niger J Clin Pract 2021 Jan;24(1):100-103

National Cardiothoracic Center of Excellence UNTH Ituku/Ozalla Enugu and University of Nigeria, Enugu Campus, Nigeria.

Background: Complex cardiac anomalies are a set of associated heart structural anomalies requiring some adaptive mechanism to maintain life.

Objectives: This is to determine pattern and prevalence of common complex cardiac anomalies among children presenting for cardiac surgery in Enugu.

Methodology: The children that present for cardiac surgery in our center over a six-month period were admitted and rescreened using a standardized echocardiographic method, information on biodata, oxygen saturation and 2D echo diagnosis were recorded in a proforma. Read More

View Article and Full-Text PDF
January 2021

Neonatal Cone Valvuloplasty for Pulmonary Atresia/Intact Ventricular Septum and Ebsteinoid Valve.

Ann Thorac Surg 2021 Jan 6. Epub 2021 Jan 6.

Section of Congenital and Pediatric Cardiothoracic Surgery, Division of Cardiac, Thoracic and Vascular Surgery, Department of Surgery, Morgan Stanley Children's Hospital, New York-Presbyterian Hospital/Columbia University Medical Center, New York, NY, USA.

Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare lesion with wide anatomic variability. A rare variant exists, marked by right-sided dilatation, an Ebsteinoid tricuspid valve (TV), and severe tricuspid regurgitation. Neonatal cone valvuloplasty allows for a biventricular circulation and avoids complications of prosthetic valves; however, this technique inevitably requires reintervention to exchange the RV-PA conduit as the patient grows. Read More

View Article and Full-Text PDF
January 2021

High Birth Prevalence of Congenital Heart Diseases in Conjoined Twins and Higher Order Multiple Births.

Am J Cardiol 2021 03 6;142:159-160. Epub 2021 Jan 6.

Division of Pediatric Cardiology, Department of Pediatrics, University of California, San Francisco, California.

View Article and Full-Text PDF

A role of Hey2 transcription factor for right ventricle development through regulation of Tbx2-Mycn pathway during cardiac morphogenesis.

Dev Growth Differ 2021 Jan 27;63(1):82-92. Epub 2021 Jan 27.

Department of Molecular Physiology, National Cerebral and Cardiovascular Center Research Institute, Suita, Osaka, Japan.

A basic helix-loop-helix transcription factor Hey2 is expressed in the ventricular myocardium and endocardium of mouse embryos, and Hey2 null mice die perinatally showing ventricular septal defect, dysplastic tricuspid valve and hypoplastic right ventricle. In order to understand region-specific roles of Hey2 during cardiac morphogenesis, we generated Hey2 conditional knockout (cKO) mice using Mef2c-AHF-Cre, which was active in the anterior part of the second heart field and the right ventricle and outflow tract of the heart. Hey2 cKO neonates reproduced three anomalies commonly observed in Hey2 null mice. Read More

View Article and Full-Text PDF
January 2021

[Fetal cardiac intervention and perioperative management of fetus with hypoplastic right heart syndrome].

Zhonghua Fu Chan Ke Za Zhi 2020 Dec;55(12):837-842

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

To summarize the experience of perioperative management strategy of fetal pulmonary valvuloplasty (FPV) for hypoplastic right heart syndrome (HRHS). In the retrospective study of perioperative data, 13 fetuses of HRHS were treated with FPV in Qingdao Women and Children's Hospital from July 2018 to June 2019. (1) The evaluation indexes of the right ventricle in 13 fetuses before FPV: the mean ratio of tricuspid/mitral annulus, right/left ventricular length, pulmonary/aortic annulus, and tricuspid inflow time/cardiac cycle were 0. Read More

View Article and Full-Text PDF
December 2020

H-type tracheo-oesophageal fistula in an infant.

BMJ Case Rep 2020 Dec 17;13(12). Epub 2020 Dec 17.

Department of Pediatrics, Fukushima Medical University, Fukushima, Japan.

View Article and Full-Text PDF
December 2020

Transvenous shock-only implantable cardioverter defibrillator after an atrio-pulmonary Fontan surgery.

Pacing Clin Electrophysiol 2021 01 27;44(1):181-184. Epub 2020 Nov 27.

Department of Cardiology, Tokyo Women's Medical University, Tokyo, Japan.

A 42-year-old woman with tricuspid atresia who underwent a Fontan surgery (atrio-pulmonary connection) was admitted to our hospital due to symptomatic ventricular tachycardia (VT). A defibrillation lead was implanted in a distal site of a coronary vein since there was no usual entry to the ventricle. Ventricular pacing was impossible due to the high threshold, however, good sensing was obtained. Read More

View Article and Full-Text PDF
January 2021

Orthotopic Heart Transplantation in a Child with Single Ventricle after Pneumonectomy.

ASAIO J 2020 Nov 4. Epub 2020 Nov 4.

Department of Pediatrics, University of Minnesota Masonic Children's Hospital, Minneapolis, Minnesota.

We report a 6-year-old with single ventricle physiology secondary to tricuspid atresia who had cardiorespiratory failure who was not a candidate for further single ventricle palliation. The patient underwent planned staged left pneumonectomy for recurrent pneumonias secondary to bronchomalacia followed by orthotopic heart transplantation. This aggressive approach improved the patient candidacy for heart transplantation by removing the source of recurrent infection and respiratory failure (left lung). Read More

View Article and Full-Text PDF
November 2020

Outcomes after the Fontan operation in the Middle East: A large Saudi Arabian single centre experience.

Int J Cardiol 2021 02 23;325:56-61. Epub 2020 Oct 23.

Adult Congenital Heart Disease, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudia Arabia.

Background: Fontan outcomes data from large volume Middle Eastern Centres are lacking. We report our experience after the Fontan operation from a tertiary cardiac centre in Saudi Arabia.

Method: All 458 consecutive patients who had Fontan surgery 1986 through 2015 at the Prince Sultan Cardiac Centre, Riyadh [PSCC], Saudi Arabia, were evaluated for baseline, early and late post-operative outcomes and their uni and multivariate determinants. Read More

View Article and Full-Text PDF
February 2021

Best management in isolated right ventricular hypoplasia with septal defects in adults.

J Cardiovasc Thorac Res 2020 27;12(3):237-243. Epub 2020 Aug 27.

Cardiovascular Research Center, Institute of Basic and Clinical Physiology Sciences, Kerman University of Medical Sciences, Kerman, Iran.

Hypoplastic right ventricle is a rare congenital disease usually associated with pulmonary atresia or tricuspid atresia. Isolated right ventricular hypoplasia is a rare anomaly without important valvular abnormalities. It is associated with inter atrial septal defects leading to the right-to-left shunting of blood. Read More

View Article and Full-Text PDF