2,040 results match your criteria Tricuspid Atresia


Unroofed coronary sinus: An unusual interatrial communication and a rare childhood entity.

Ann Pediatr Cardiol 2019 Jan-Apr;12(1):64-65

Department of Pediatric Cardiology and Cardiothoracic Surgery, Kauvery Hospital, Chennai, Tamil Nadu, India.

Unroofed coronary sinus, an unusual form of interatrial communication, is a rare cardiac anomaly. It is not a true defect of the atrial septum. It is described as a partial (focal or fenestrated) or complete absence of the roof of the coronary sinus, resulting in a communication between the coronary sinus and left atrium. Read More

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http://dx.doi.org/10.4103/apc.APC_119_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6343380PMC
February 2019

Atrioventricular reentrant tachycardia in a child with tricuspid atresia: A case report of catheter ablation.

Medicine (Baltimore) 2019 Feb;98(6):e14320

Department of Cardiology, Hunan Children's Hospital.

Rationale: Atrioventricular reentrant tachycardia (AVRT) is the most common supraventricular tachycardia occurring in children. However, in complex congenital heart disease patients with a different heart anatomy and conduction system morphology, accessory pathway modification may be particularly challenging because of distortion of typical anatomic landmarks.

Patient Concerns: A 10-year-old boy with tricuspid atresia and history of bidirectional Glenn operation had recurrent chest distress and palpitation for 3 months. Read More

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http://dx.doi.org/10.1097/MD.0000000000014320DOI Listing
February 2019
2 Reads

Tricuspid atresia with restrictive foramen ovale: A rare combination with implications on fetal growth.

Echocardiography 2019 Jan 28. Epub 2019 Jan 28.

The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.

Restrictive atrial communication is rarely reported in tricuspid atresia but when present it can lead to important morbidity. We describe two fetuses with tricuspid atresia with restrictive foramen ovale who were found to have fetal growth failure. Fetal echocardiography detected a restrictive atrial communication by flow acceleration on color Doppler and significant right atrial dilation in one patient; the atrial septum was not well interrogated in the other patient. Read More

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http://dx.doi.org/10.1111/echo.14267DOI Listing
January 2019
1 Read

Absent Pulmonary Valve Syndrome in a Fetus: A Case Report and Literature Review.

Fetal Pediatr Pathol 2019 Jan 20:1-6. Epub 2019 Jan 20.

c Department of Medical Ultrasound , Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology , Wuhan , China.

Introduction: The main characteristics of absent pulmonary valve syndrome (APVS) include the absence or hypoplasia of the pulmonary valve, stenosis of the pulmonary valve annulus, and aneurysmal dilatation of the pulmonary trunk and its branches. In the more common type 1, the tetralogy of Fallot-like type, there is a ventricular septal defect, overriding aorta, pulmonary arterial dilatation, and absence of ductus arteriosus, The second type has an intact ventricular septum, less pulmonary artery dilatation, and a patent ductus arteriosus, with or without tricuspid atresia.

Case Report: This APVS had an intact ventricular septum with an absent ductus arteriosus. Read More

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https://www.tandfonline.com/doi/full/10.1080/15513815.2018.1
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http://dx.doi.org/10.1080/15513815.2018.1529066DOI Listing
January 2019
8 Reads

'Absent' pulmonary valve with intact ventricular septum mimicking tricuspid valve atresia: Prenatal diagnosis and postnatal course.

J Obstet Gynaecol Res 2019 Jan 17. Epub 2019 Jan 17.

Pediatric Cardiology, Tuscany Region-National Centre for Research Foundation, Ospedale del Cuore, Massa, Italy.

Absent pulmonary valve syndrome is a rare congenital heart disease characterized by absent or rudimentary pulmonary valve leaflets and hypoplastic pulmonary annulus. The most common variant, associated with tetralogy of Fallot, implies dilatation of pulmonary branches and usually absent ductus arteriosus. Rarely, absent pulmonary valve occurs with intact ventricular septum: pulmonary branches are normally sized or mildly dilated and ductus arteriosus is usually patent. Read More

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http://dx.doi.org/10.1111/jog.13878DOI Listing
January 2019
9 Reads

Revisitation of Double Inlet Left Ventricle or Tricuspid Atresia with Transposed Great Arteries.

Ann Thorac Surg 2018 Dec 23. Epub 2018 Dec 23.

Division of Pediatric Cardiac Surgery, Asan Medical Center, Seoul, South Korea. Electronic address:

Background: Patients with double inlet left ventricle (DILV) or tricuspid atresia (TA) with transposition of the great arteries (TGA) are predisposed to systemic outflow tract obstruction necessitating systemic outflow relief operations (SORO).

Methods: Between January 2000 and February 2018, 30 patients with DILV (n=20) or TA (n=10) with TGA underwent single ventricle palliation. Arch obstruction (AO) was observed in 14 patients. Read More

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http://dx.doi.org/10.1016/j.athoracsur.2018.11.052DOI Listing
December 2018
1 Read

The association between natural gas well activity and specific congenital anomalies in Oklahoma, 1997-2009.

Environ Int 2019 Jan 12;122:381-388. Epub 2018 Dec 12.

Department of Biostatistics and Epidemiology, College of Public Health, University of Oklahoma Health Sciences Center, Oklahoma City, OK, USA.

Background: Natural gas drilling may pose multiple health risks, including congenital anomalies, through air pollutant emissions and contaminated water. Two recent studies have evaluated the relationship between natural gas activity and congenital anomalies, with both observing a positive relationship.

Objectives: We aimed to evaluate whether residence near natural gas wells is associated with critical congenital heart defects (CCHD), neural tube defects (NTD), and oral clefts in Oklahoma, the third highest natural gas producing state in the US. Read More

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http://dx.doi.org/10.1016/j.envint.2018.12.011DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6328052PMC
January 2019
1 Read

Fifty-Five Years Follow-Up of 111 Adult Survivors After Biventricular Repair of PAIVS and PS.

Pediatr Cardiol 2018 Dec 11. Epub 2018 Dec 11.

Division of Paediatric Cardiology, Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, The University of Hong Kong, 102, Pokfulam Road, Hong Kong, China.

There is paucity of long-term data on adult survivors after biventricular repair of pulmonary atresia with intact ventricular septum (PAIVS) and pulmonary stenosis (PS). This study aimed to determine the cardiac and non-cardiac outcomes of adult survivors after biventricular repair of PAIVS and PS. The cardiac, neurodevelopmental and liver problems of 111 adults, 40 with PAIVS and 71 with PS, were reviewed. Read More

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http://link.springer.com/10.1007/s00246-018-2041-2
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http://dx.doi.org/10.1007/s00246-018-2041-2DOI Listing
December 2018
12 Reads
1.550 Impact Factor

A Pathogenic Homozygous Mutation in The Pleckstrin Homology Domain of RASA1 Is Responsible for Familial Tricuspid Atresia in An Iranian Consanguineous Family.

Cell J 2019 Apr 18;21(1):70-77. Epub 2018 Nov 18.

Department of Pediatrics Cardiology, Faculty of Medicine, Tehran University of Medical Sciences, Tehran, Iran. Electronic Address:

Objective: Tricuspid atresia (TA) is a rare life-threatening form of congenital heart defect (CHD). The genetic mechanisms underlying TA are not clearly understood. According to previous studies, the endocardial cushioning event, as the primary sign of cardiac valvulogenesis, is governed by several overlapping signaling pathways including Ras/ ERK pathway. Read More

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http://dx.doi.org/10.22074/cellj.2019.5734DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6275424PMC
April 2019
3 Reads

Twin pregnancy complicated by total placenta previa in a Fontan-palliated patient: A case report.

Case Rep Womens Health 2018 Oct 26;20:e00085. Epub 2018 Oct 26.

Department of Obstetrics and Gynecology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.

We present a case of a twin pregnancy in a Fontan-palliated woman that was complicated by total placenta previa. The patient was diagnosed with tricuspid atresia type II, and underwent the Fontan operation at 11 years of age. At 32 years of age, she was shown to have a dichorionic diamniotic twin pregnancy. Read More

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http://dx.doi.org/10.1016/j.crwh.2018.e00085DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6218841PMC
October 2018
13 Reads

Pheochromocytoma in Congenital Cyanotic Heart Disease.

Case Rep Endocrinol 2018 25;2018:2091257. Epub 2018 Sep 25.

Department of Biomedical Sciences for Health, Università degli Studi di Milano, Milan, Italy.

Studies on genome-wide transcription patterns have shown that many genetic alterations implicated in pheochromocytoma-paraganglioma (P-PGL) syndromes cluster in a common cellular pathway leading to aberrant activation of molecular response to hypoxia in normoxic conditions (the pseudohypoxia hypothesis). Several cases of P-PGL have been reported in patients with cyanotic congenital heart disease (CCHD). Patients affected with CCHD have an increased likelihood of P-PGL compared to those affected with noncyanotic congenital heart disease. Read More

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https://www.hindawi.com/journals/crie/2018/2091257/
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http://dx.doi.org/10.1155/2018/2091257DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6176301PMC
September 2018
5 Reads

Strain and Rotational Mechanics in Children With Single Left Ventricles After Fontan.

J Am Soc Echocardiogr 2018 Dec 18;31(12):1297-1306. Epub 2018 Oct 18.

Heart Institute, Section of Pediatric Cardiology, Department of Pediatrics, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, Colorado. Electronic address:

Background: Left ventricular (LV) mechanics in patients with different single morphologic LV subtypes, including tricuspid atresia, double-inlet left ventricle, and pulmonary atresia with intact ventricular septum, remain poorly studied. Given that histologic studies indicate differences in LV myocardial fiber orientation, we hypothesized that this may result in altered LV mechanics. The aim of this study was to evaluate the influence of LV morphology on LV mechanics. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S08947317183050
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http://dx.doi.org/10.1016/j.echo.2018.09.004DOI Listing
December 2018
11 Reads

Pre-intervention morphologic and functional echocardiographic characteristics of neonates with critical left heart obstruction: a Congenital Heart Surgeons Society (CHSS) inception cohort study.

Eur Heart J Cardiovasc Imaging 2018 Oct 17. Epub 2018 Oct 17.

Department of Pediatrics, Division of Cardiology, The Hospital for Sick Children, 555 University Ave, Toronto, ON M5G 1X8, Canada.

Aims: The aims of this study were to provide a detailed descriptive analysis of pre-intervention morphologic and functional echocardiographic parameters in a large, unselected, multicentre cohort of neonates diagnosed with critical left heart obstruction and to compare echocardiographic features between the different subtypes of left-sided lesions.

Methods And Results: Pre-intervention echocardiograms for 651 patients from 19 Congenital Heart Surgeons' Society (CHSS) institutions were reviewed in a core lab according to a standardized protocol including >150 morphologic and functional variables. The four most common subtypes of lesions were: aortic atresia (AA)/mitral atresia (MA) (29% of patients), AA/mitral stenosis (MS) (20%), aortic stenosis (AS)/MS (26%), and isolated AS (iAS) (18%). Read More

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https://academic.oup.com/ehjcimaging/advance-article/doi/10.
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http://dx.doi.org/10.1093/ehjci/jey141DOI Listing
October 2018
14 Reads

Converting Fontan-Björk to 1.5- or 2-ventricle circulation.

Ann Thorac Surg 2018 Oct 10. Epub 2018 Oct 10.

Indiana University School of Medicine, Department of Medicine, Division of Cardiology, Riley Hospital for Children, 705 Riley Hospital Dr. Indianapolis, IN, 46202.

Patients with tricuspid atresia and ventricular septal defect have in the past occasionally undergone a Fontan with "Björk" modification to create a connection between the right atrium and the right ventricular outflow tract. While rarely performed now, patients with this physiology often face severe complications requiring re-intervention. We hypothesize that surgical conversion to a 2-ventricle or 1. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00034975183145
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http://dx.doi.org/10.1016/j.athoracsur.2018.07.097DOI Listing
October 2018
7 Reads

Patients diagnosed with long QT syndrome after repair of congenital heart disease.

Pacing Clin Electrophysiol 2018 11 9;41(11):1435-1440. Epub 2018 Oct 9.

Department of Laboratory Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, South Korea.

Background: Patients with repaired congenital heart disease (CHD) can have both depolarization and repolarization abnormalities. A coexisting long QT syndrome (LQTS) may cause electrical instability in these patients; however, diagnosing LQTS is difficult owing to intraventricular conduction delay or paced rhythm after the operation. We report on six patients diagnosed with LQTS after CHD repair. Read More

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http://dx.doi.org/10.1111/pace.13512DOI Listing
November 2018
6 Reads

Echocardiographic parameters associated with biventricular circulation and right ventricular growth following right ventricular decompression in patients with pulmonary atresia and intact ventricular septum: Results from a multicenter study.

Congenit Heart Dis 2018 Nov 21;13(6):892-902. Epub 2018 Sep 21.

Department of Pediatrics, Division of Pediatric Cardiology, Emory University School of Medicine, Atlanta, Georgia.

Background: In patients with pulmonary atresia, intact ventricular septum (PA/IVS) following right ventricular (RV) decompression, RV size and morphology drive clinical outcome. Our objectives were to (1) identify baseline and postdecompression echocardiographic parameters associated with 2V circulation, (2) identify echocardiographic parameters associated with RV growth and (3) describe changes in measures of RV size and changes in RV loading conditions.

Methods: We performed a retrospective analysis of patients who underwent RV decompression for PA/IVS at four centers. Read More

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http://doi.wiley.com/10.1111/chd.12671
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http://dx.doi.org/10.1111/chd.12671DOI Listing
November 2018
5 Reads

Achieving biventricular circulation in patients with moderate hypoplastic right ventricle in pulmonary atresia intact ventricular septum after transcatheter pulmonary valve perforation.

Congenit Heart Dis 2018 Nov 21;13(6):884-891. Epub 2018 Sep 21.

Department of Pediatric Cardiology, Queen Mary Hospital, Hong Kong SAR.

Objective: Transcatheter valve perforation for pulmonary atresia intact ventricular septum is the standard of care for patients with mild right ventricular hypoplasia. However, its role in moderate right ventricular hypoplasia has been less well defined. We sought to report the long-term outcome of patients with moderate hypoplastic right ventricle who had undergone the procedure. Read More

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http://dx.doi.org/10.1111/chd.12658DOI Listing
November 2018
3 Reads

Mechanical and structural analysis of the pulmonary valve in congenital heart defects: A presentation of two case studies.

J Mech Behav Biomed Mater 2019 Jan 31;89:9-12. Epub 2018 Aug 31.

Tissue Mechanics Laboratory, The Wallace H. Coulter Department of Biomedical Engineering, Georgia Institute of Technology and Emory University, Atlanta, GA, United States. Electronic address:

Objective: Congenital Heart Disease (CHD) is the leading cause of pediatric mortality, with many cases affecting the right ventricular outflow tract (RVOT) or pulmonary valve (PV). Understanding the mechanics of the disease condition can provide insight into development of durable repair techniques and bioengineered replacement devices. This work presents a mechanical and structural analysis of the pulmonary valve of two pediatric cases. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S17516161183045
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http://dx.doi.org/10.1016/j.jmbbm.2018.08.053DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6223622PMC
January 2019
10 Reads

Long Term Consequences of the Fontan Procedure and How to Manage Them.

Prog Cardiovasc Dis 2018 Sep - Oct;61(3-4):365-376. Epub 2018 Sep 17.

Children's Hospital at Montefiore, Montefiore Medical Center, Albert Einstein College of Medicine, NY. Electronic address:

In 1971, Fontan and Baudet described a surgical technique for successful palliation of patients with tricuspid atresia. Subsequently, this technique has been applied to treat most forms of functional single ventricles and has become the current standard of care for long-term palliation of all patients with single ventricle congenital heart disease. Since 1971, the Fontan procedure has undergone several variations. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00330620183018
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http://dx.doi.org/10.1016/j.pcad.2018.09.005DOI Listing
December 2018
11 Reads

Prevalence of congenital cardiac anomalies in patients with cleft lip and palate - Its implications in surgical management.

J Oral Biol Craniofac Res 2018 Sep-Dec;8(3):241-244. Epub 2017 Oct 3.

Sharad Pawar Dental College & Hospital, DMIMS, Wardha, India.

Background: Cleft lip and palate is one of the most common congenital craniofacial deformities seen in children. Various congenital anomalies are reported in the literature to be associated with cleft lip and palate. Cardiac anomalies are one of the most common congenital disorders associated in cleft lip and palate patientsIt includes Cyanotic and acyanotic cardiac diseases likel fallot's tetralogy, transposition of greater vessels, atresia of tricuspid, total anomalous pulmonary venous return (TAPVR), truncus arteriosus, ebstein's anomaly, hypoplastic left heart syndrome and pulmonary atresia, patent ductus arteriosus, ventricular septal defect, atrial septal defect, pulmonary stenosis, aortic stenosis and coarctation of aorta. Read More

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http://dx.doi.org/10.1016/j.jobcr.2017.09.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6107920PMC
October 2017
3 Reads

Thromboprophylaxis in Adults With Atrio-Pulmonary Fontan.

World J Pediatr Congenit Heart Surg 2018 09;9(5):504-508

1 Division of Cardiology, Department of Medicine, Ahmanson/UCLA Adult Congenital Heart Disease Center, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.

Background: Although aspirin has been compared to warfarin for thromboembolic prophylaxis in the general Fontan population, little is known about the optimal preventative strategy for the atriopulmonary right atrium-pulmonary artery [RA-PA]) Fontan particularly.

Methods: A retrospective cohort study was performed including adult patients identified in the Ahmanson/UCLA Adult Congenital Heart Disease Center database with a history of RA-PA Fontan and use of either aspirin or warfarin as most recent primary prophylaxis against thromboembolism. Primary outcome was incident thromboembolism, defined as space-occupying lesion on imaging consistent with thrombus within the Fontan or pulmonary arterial circuit. Read More

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http://dx.doi.org/10.1177/2150135118772837DOI Listing
September 2018
13 Reads

Atresia of the inferior vena cava in a patient undergoing mitral and tricuspid valve surgery.

Interact Cardiovasc Thorac Surg 2019 Feb;28(2):324-326

Department of Cardiac Surgery, Kerckhoff Heart Center, Bad Nauheim, Germany.

We report on a 61-year-old man with a previously unknown interruption of the inferior vena cava who was scheduled to undergo minimally invasive mitral and tricuspid valve surgery. We aimed to establish a cardiopulmonary bypass using femorofemoral cannulation under transoesophageal echocardiography. In spite of multiple attempts, the positioning of the venous wire in the right atrium was not accomplished. Read More

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https://academic.oup.com/icvts/advance-article/doi/10.1093/i
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http://dx.doi.org/10.1093/icvts/ivy240DOI Listing
February 2019
12 Reads

Cantrell Syndrome-A Rare Complex Congenital Anomaly: A Case Report and Literature Review.

Front Pediatr 2018 17;6:201. Epub 2018 Jul 17.

Department of Neonatology, University of Medicine and Pharmacy Tîrgu Mureş, Tîrgu Mureş, Romania.

Cantrell syndrome (CS) or pentalogy of Cantrell is defined as a rare condition involving a midline anterior abdominal wall defect, a distal sternal cleft, a defect of the anterior diaphragm, and a defect of the apical pericardium with pericardio-peritoneal communication, as well as intracardiac anomalies. We report the case of a male newborn with type 2 CS diagnosed during intrauterine life based on ultrasonographic evaluation. Clinical examination at birth revealed an abdominal wall defect with extrathoracic displacement of the heart and a diastasis of the sagittal suture. Read More

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https://www.frontiersin.org/article/10.3389/fped.2018.00201/
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http://dx.doi.org/10.3389/fped.2018.00201DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6056637PMC
July 2018
5 Reads

Tei Index Is the Best Echocardiographic Parameter for Assessing Right Ventricle Function in Patients With Unrepaired Congenital Heart Diseases With Outflow Tract Obstruction.

Front Pediatr 2018 26;6:181. Epub 2018 Jun 26.

Dirección de Investigación, Hospital Infantil de México Federico Gómez, Mexico City, Mexico.

Magnetic resonance imaging (MRI) and cardiac catheterization are diagnostic tools for right ventricle dysfunction (RVD), but those are expensive and often unavailable techniques. Thus, our objective was to identify clinical and/or echocardiographic variables capable of predicting a catheterization-based diagnosis of RVD. This was cross-sectional, diagnostic test accuracy study, considering the catheterization-based diagnosis of RVD as the gold standard. Read More

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http://dx.doi.org/10.3389/fped.2018.00181DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6028761PMC
June 2018
16 Reads

Transcatheter perforation of atretic pulmonary valve by the stiff end of a coronary wire in neonates with pulmonary atresia with intact ventricular septum: A solution in developing countries.

J Saudi Heart Assoc 2018 Jul 31;30(3):222-232. Epub 2018 Jan 31.

Cardiology Department, Tanta University Hospitals, Tanta, Egypt.

Objectives: To evaluate the safety of using the stiff end of a coronary wire to perforate an atretic pulmonary valve (PV) in patients with pulmonary atresia with intact ventricular septum (PAIVS).

Background: Radiofrequency perforation is an accepted modality to perforate the PV in patients PAIVS. However, the high cost precludes its widespread use. Read More

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http://dx.doi.org/10.1016/j.jsha.2018.01.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6035381PMC
July 2018
18 Reads

The Impact of the Left Ventricle on Right Ventricular Function and Clinical Outcomes in Infants with Single-Right Ventricle Anomalies up to 14 Months of Age.

J Am Soc Echocardiogr 2018 Oct 3;31(10):1151-1157. Epub 2018 Jul 3.

Division of Cardiology, Medical College of Wisconsin, Milwaukee, Wisconsin.

Background: Children with single-right ventricle anomalies such as hypoplastic left heart syndrome (HLHS) have left ventricles of variable size and function. The impact of the left ventricle on the performance of the right ventricle and on survival remains unclear. The aim of this study was to identify whether left ventricular (LV) size and function influence right ventricular (RV) function and clinical outcome after staged palliation for single-right ventricle anomalies. Read More

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http://dx.doi.org/10.1016/j.echo.2018.05.003DOI Listing
October 2018
29 Reads

Regenerative Therapy for Patients with Congenital Heart Disease.

Authors:
Naritaka Kimura

Keio J Med 2018 Jun 19. Epub 2018 Jun 19.

Department of Cardiovascular Surgery, Keio University School of Medicine, Tokyo, Japan.

Congenital heart disease (CHD) is the most common birth defect, affecting 1 in 100 babies. Among CHDs, single ventricle (SV) physiologies, such as hypoplastic left heart syndrome and tricuspid atresia, are particularly severe conditions that require multiple palliative surgeries, including the Fontan procedure. Although the management strategies for SV patients have markedly improved, the prevalence of ventricular dysfunction continues to increase over time, especially after the Fontan procedure. Read More

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http://dx.doi.org/10.2302/kjm.2018-0002-IRDOI Listing
June 2018
2 Reads

[Prenatal interventional therapy in two cases with critical pulmonary stenosis or pulmonary atresia with intact ventricular septum].

Zhonghua Er Ke Za Zhi 2018 Jun;56(6):445-450

Department of Maternal-Fetal Cardiology, Guangdong Cardiovascular Institute, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangdong Province Key Laboratory of Structural Heart Disease, Guangzhou 510080, China.

Two cases who underwent fetal pulmonary valvuloplasty (FPV) for pulmonary atresia with intact ventricular septum (PA-IVS) or critical pulmonary stenosis with intact ventricular septum (CPS-IVS) successfully were reported. The aim of the report was to explore the criteria for case selection, the technical essentials of FPV, and the postpartum outcome of the fetus. One case with PA-IVS and the other with CPS-IVS were enrolled in September 2016 and February 2017 in Guangdong General Hospital, and both cases were diagnosed with severe right ventricular dysplasia and tricuspid regurgitation by fetal echocardiogram. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0578-1310.2018.06.008DOI Listing
June 2018
4 Reads

Anesthetic Implications for Cesarean Section in a Parturient with Complex Congenital Cyanotic Heart Disease.

Case Rep Anesthesiol 2018 29;2018:2616390. Epub 2018 Mar 29.

Department of Anesthesia and Intensive Care, Singapore General Hospital, Singapore.

The discordance between increased physiological demand during pregnancy and congenital cardiac pathology of a parturient is a perilous threat to the maternal-fetal well-being. Early involvement of a multidisciplinary team is essential in improving peripartum morbidity and mortality. Designing the most appropriate anesthetic care will require a concerted effort, with inputs from the obstetricians, obstetric and cardiac anesthesiologists, cardiologists, neonatologists, and cardiothoracic surgeons. Read More

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http://dx.doi.org/10.1155/2018/2616390DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5896257PMC
March 2018
5 Reads

Pregnancy in Women With a Fontan Circulation: A Systematic Review of the Literature.

Circ Cardiovasc Qual Outcomes 2018 May;11(5):e004575

Heart Institute, Cincinnati Children's Hospital Medical Center, OH (S.B. G.R.V., N.B.)

Background: The Fontan operation has provided life-saving palliation and adult survival for individuals born with single ventricle physiology. Many now seek advice about safe pregnancy. Little data are, however, available, consisting mainly of anecdotal experience and small series. Read More

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https://www.ahajournals.org/doi/10.1161/CIRCOUTCOMES.117.004
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http://dx.doi.org/10.1161/CIRCOUTCOMES.117.004575DOI Listing
May 2018
12 Reads

An update work of pulse oximetry screening for detecting critical congenital heart disease in the newborn.

Images Paediatr Cardiol 2017 Jul-Sep;19(3):12-18

Department of Pediatrics, Jawaharlal Nehru Medical College, Sawangi Meghe, Wardha, Maharashtra -442102.

Background: Congenital Heart Disease (CHD) is the commonest group of congenital malformations and affects 7-8 per 1000 live born newborns. Nevertheless, it is estimated that more than 50% of babies with undiagnosed CHD are not detected by routine neonatal cardiac examination.

Aim: To find the incidence of CHD in newborns and to determine the accuracy of pulse oximetry for detecting clinically unrecognized critical congenital heart disease (CCHD) in the newborns. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5917867PMC
May 2018
5 Reads

Multidetector Computed Tomography (CT) in Evaluation of Congenital Cyanotic Heart Diseases.

Pol J Radiol 2017 17;82:645-659. Epub 2017 Nov 17.

Department of Pediatrics, Zagazig University, Zagazig, Egypt.

Background: The aim of the study is to emphasize the role of 128 MSCT angiography in the diagnosis of congenital cyanotic heart diseases.

Material/methods: This study included sixty patients and was conducted from December 2014 to July 2016 in the Multidetector CT unit of Zagazig University hospitals. All images included axial, MPR, MIP, and VRT and were interpreted in one session. Read More

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http://dx.doi.org/10.12659/PJR.903222DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5894010PMC
November 2017
9 Reads

End-Organ Saturation Differences in Early Neonatal Transition for Left- versus Right-Sided Congenital Heart Disease.

Neonatology 2018 12;114(1):53-61. Epub 2018 Apr 12.

Division of Neonatal and Developmental Medicine, Stanford University - Lucile Packard Children's Hospital, Palo Alto, California, USA.

Background: For neonates with congenital heart disease (CHD), left-sided (LL) and right-sided (RL) single ventricular physiologies (LL, hypoplastic left heart syndrome; RL, tricuspid atresia or pulmonary atresia with intact ventricular septum) may demonstrate distinct changes in tissue saturation in the first 72 h of life. Near-infrared spectroscopy (NIRS) can measure regional cerebral saturation (Csat) and renal saturation (Rsat) to clarify differences between LL and RL over time.

Objectives: Our primary objective was to measure changes in Csat and Rsat in the first 72 h of life using NIRS between CHD infants with LL compared to RL. Read More

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http://dx.doi.org/10.1159/000487472DOI Listing
April 2018
3 Reads

Respiratory exacerbation in a young adult with cystic fibrosis and tricuspid atresia.

Respirol Case Rep 2018 07 1;6(5):e00318. Epub 2018 Apr 1.

Department of CardiologyACHD Service, Sir Charles Gairdner Hospital Perth Australia.

Tricuspid atresia (TAt) is a complex congenital heart defect (CHD) characterized by the absence of the tricuspid valve and right ventricular hypoplasia requiring surgery in childhood, the Fontan procedure. We present a case of a 21-year-old male with TAt and cystic fibrosis (CF), who underwent a Fontan procedure in childhood, presenting to an adult CF clinic with severe deterioration in his respiratory status and multi-organ dysfunction associated with CF. This report describes problems associated with the management of a CF respiratory exacerbation and extrapulmonary manifestations of CF in the unique situation of a Fontan circulation, a circulation with absence of a subpulmonary ventricle and pulsatile pulmonary arterial blood flow where maintenance of systemic cardiac output is totally dependent on good respiratory function and low pulmonary artery pressures. Read More

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http://dx.doi.org/10.1002/rcr2.318DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5879030PMC
July 2018
9 Reads

Immediate effects and outcome of in-utero pulmonary valvuloplasty in fetuses with pulmonary atresia with intact ventricular septum or critical pulmonary stenosis.

Ultrasound Obstet Gynecol 2018 08;52(2):230-237

Children's Heart Center Linz, Department of Pediatric Cardiology, Kepler University Hospital, Linz, Austria.

Objective: To assess the immediate effects of fetal pulmonary valvuloplasty on right ventricular (RV) size and function as well as in-utero RV growth and postnatal outcome.

Methods: Patients with pulmonary atresia with intact ventricular septum (PAIVS) or critical pulmonary stenosis (CPS) who underwent fetal pulmonary valvuloplasty at our center between October 2000 and July 2017 were included. Echocardiographic data obtained before and after the procedure were analyzed retrospectively (median interval after intervention, 1 (range, 1-3) days) for ventricular and valvular dimensions and ratios, RV filling time (duration of tricuspid valve (TV) inflow/cardiac cycle length), TV velocity time integral (TV-VTI) × heart rate (HR) and tricuspid regurgitation (TR) velocity. Read More

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http://dx.doi.org/10.1002/uog.19047DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6100104PMC
August 2018
7 Reads

A Novel Mechanism for Improved Exercise Performance in Pediatric Fontan Patients After Cardiac Rehabilitation.

Pediatr Cardiol 2018 Jun 9;39(5):1023-1030. Epub 2018 Mar 9.

Cincinnati Children's Hospital Medical Center, Heart Institute, 3333 Burnet Avenue, MLC 2003, Cincinnati, OH, 45229-3026, USA.

Patients with a Fontan circulation have impaired exercise capacity. Cardiac rehabilitation (CR) has shown promise in enhancing peak exercise parameters in this population, but an improvement in submaximal exercise has not been consistently demonstrated. We assessed the hypothesis that participation in CR will be associated with more efficient oxygen extraction and ventilation during submaximal exercise. Read More

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http://dx.doi.org/10.1007/s00246-018-1854-3DOI Listing
June 2018
8 Reads

Evaluation of the anatomic and hemodynamic abnormalities in tricuspid atresia before and after surgery using computational fluid dynamics.

Medicine (Baltimore) 2018 Jan;97(2):e9510

Department of Medical Imaging, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, China.

Analysis of hemodynamics inside tricuspid atresia (TA) chamber is essential to the understanding of TA for optimal treatment. In this study, we introduced a combined computational fluid dynamics (CFD) to simulate blood flow in the left ventricle (LV) to study the diastolic flow changes in TA.Real-time 3-dimentional echocardiography loops (ECHO) were acquired in normal control group, in TA patients before surgery (pre-op group) and after surgery (post-op group). Read More

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http://dx.doi.org/10.1097/MD.0000000000009510DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5943871PMC
January 2018
10 Reads

Successful management of living donor liver transplantation for biliary atresia with single ventricle physiology-from peri-transplant through total cavopulmonary connection: A case report.

Pediatr Transplant 2018 05 19;22(3):e13118. Epub 2018 Feb 19.

Department of Pediatric Surgery, Keio University School of Medicine, Tokyo, Japan.

Children with single ventricle physiology have complete mixing of the pulmonary and systemic circulations, requiring staged procedures to achieve a separation of these circulations, or Fontan circulation. The single ventricle physiology significantly increases the risk of mortality in children undergoing non-cardiac surgery. As liver transplantation for patients with single ventricle physiology is particularly challenging, only a few reports have been published. Read More

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http://dx.doi.org/10.1111/petr.13118DOI Listing
May 2018
14 Reads

Prenatal diagnosis of congenital heart disease: A review of current knowledge.

Indian Heart J 2018 Jan - Feb;70(1):150-164. Epub 2017 Dec 16.

Department of Obstetrics, Paulista School of Medicine - Federal University of São Paulo (EPM-UNIFESP), São Paulo, SP, Brazil. Electronic address:

This article reviews important features to improve the diagnosis of congenital heart disease (CHD) by applying ultrasound in prenatal cardiac screening. As low and high-risk pregnancies for CHD are subject to routine obstetric ultrasound, the diagnosis of structural heart defects represents a challenge that involves a team of specialists and subspecialists on fetal ultrasonography. In this review, the images highlight normal anatomy of the heart as well as pathologic cases consistent with cardiac malposition and isomerism, septal defects, pulmonary stenosis/atresia, aortic malformations, hypoplastic left ventricle, conotruncal anomalies, tricuspid dysplasia, and Ebstein's anomaly, and univentricular heart, among other congenital cardiovascular defects. Read More

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http://dx.doi.org/10.1016/j.ihj.2017.12.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5903017PMC
July 2018
5 Reads

Mechanism for temporal changes in exercise capacity after Fontan palliation: Role of Doppler echocardiography.

Am Heart J 2018 02 22;196:144-152. Epub 2017 Oct 22.

Depertment of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA.

Background: The objective was to better understand Doppler hemodynamics and exercise capacity in patients with Fontan palliation by delineating the hemodynamic mechanism for temporal changes in their peak oxygen consumption (V̇o).

Methods: We performed a retrospective review of adult Fontan patients with systemic left ventricle (LV) who underwent serial transthoracic echocardiograms (TTE) and cardiopulmonary exercise tests (CPET) at Mayo Clinic in 2000-2015. TTE and CPET data were used (1) to determine agreement between V̇o and Doppler-derived LV function indices (eg, stroke volume index [SVI] and cardiac index [CI]) and (2) to determine agreement between temporal changes in peak V̇o and LV function indices. Read More

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http://dx.doi.org/10.1016/j.ahj.2017.10.010DOI Listing
February 2018
11 Reads
4.460 Impact Factor

Pulmonary Atresia With an Intact Ventricular Septum: Preoperative Physiology, Imaging, and Management.

Semin Cardiothorac Vasc Anesth 2018 Sep 7;22(3):245-255. Epub 2018 Feb 7.

2 Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.

Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare complex cyanotic congenital heart disease with heterogeneous morphological variation. Prenatal diagnosis allows for developing a safe plan for delivery and postnatal management. While transthoracic echocardiography allows for detailed delineation of the cardiac anatomy, additional imaging modalities such as computed tomography, magnetic resonance imaging, and catheterization may be necessary to further outline features of the cardiac anatomy, specifically coronary artery anatomy. Read More

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http://dx.doi.org/10.1177/1089253218756757DOI Listing
September 2018
9 Reads

A persistent giant Eustachian valve: functional tricuspid atresia in a newborn.

Interact Cardiovasc Thorac Surg 2018 Jun;26(6):1043-1045

Department of Cardiovascular Surgery, German Heart Center Munich, Technical University Munich, Munich, Germany.

Persistent right valve of the systemic venous sinus is a rare anomaly with anatomical variations. This anomaly may present as an obstructive structure that can inhibit the antegrade flow through the tricuspid valve. We report on a 4-day-old neonate who presented with pronounced peripheral and central cyanosis. Read More

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http://dx.doi.org/10.1093/icvts/ivy004DOI Listing
June 2018
11 Reads

Filling pressures in Fontan revisited: Comparison between pulmonary artery wedge, ventricular end-diastolic, and left atrial pressures in adults.

Int J Cardiol 2018 Mar 28;255:32-36. Epub 2017 Dec 28.

Department of Cardiovascular Diseases, Mayo Clinic, MN, United States.

Background: Pulmonary artery wedge pressure (PAWP) has been shown to correlate better with left atrial pressure (LAP) than ventricular end-diastolic pressure (VEDP) in acquired heart disease. The correlation between VEDP and PAWP and their performance as surrogates for LAP in Fontan patients is unknown.

Methods: Offline single-beat simultaneous measurement of PAWP and VEDP was performed in 50 adult Fontan patients and non-simultaneous hemodynamic data abstracted for calculation of pulmonary vascular resistance (PVR). Read More

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http://dx.doi.org/10.1016/j.ijcard.2017.12.098DOI Listing
March 2018
11 Reads
4.040 Impact Factor

Anesthetic Management During Atrial Septostomy in a Conjoined Thoraco-Omphalopagus Twin With Tricuspid Atresia and d-Transposition of the Great Arteries Before Separation: A Case Report.

A A Pract 2018 Jun;10(11):298-301

From the Departments of Anesthesiology.

Conjoined twins are uncommon with reported incidences of 1 in 30,000-200,000 births. They represent a heterogeneous population in regard to location of joint body parts and presence/extent of internal organ fusion. Positioning, airway management, possible presence of cross-circulation, and the fact that 2 patients require anesthesia for each procedure present significant challenges to the anesthesiologist. Read More

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http://dx.doi.org/10.1213/XAA.0000000000000691DOI Listing
June 2018
9 Reads

Percutaneous pulmonary valve implantation in grown-up congenital heart disease patients: Insights from the Zurich experience.

J Interv Cardiol 2018 Apr 25;31(2):251-260. Epub 2017 Dec 25.

University Heart Center, Zurich, Switzerland.

Objectives: The aim of the study was to assess indications, procedural success, complications, echocardiographic, and clinical outcomes of percutaneous pulmonary valve implantation (PPVI) in adult patients with congenital heart disease (CHD).

Background: PPVI offers a non-surgical treatment option for failing prosthetic conduits in pulmonary position. However, efficacy and clinical outcomes after PPVI are still underreported. Read More

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http://dx.doi.org/10.1111/joic.12477DOI Listing
April 2018
19 Reads

Energetic performance analysis of staged palliative surgery in tricuspid atresia using vector flow mapping.

Cardiovasc Ultrasound 2017 Dec 14;15(1):27. Epub 2017 Dec 14.

Department of Anesthesiology, Kyoto Prefectural University of Medicine, 465 Kajii Cho, Hirokoji Agaru, Kawaramachi Street, Kamigyo Ward, Kyoto City, Kyoto Prefecture, 602-8566, Japan.

Background: Staged palliative surgery markedly shifts the balance of volume load on a single ventricle and pulmonary vascular bed. Blalock-Taussig shunt necessitates a single ventricle eject blood to both the systemic and pulmonary circulation. On the contrary, bidirectional cavopulmonary shunt release the single ventricle from pulmonary circulation. Read More

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http://dx.doi.org/10.1186/s12947-017-0118-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5731082PMC
December 2017
19 Reads

ECGs in the ED.

Authors:

Pediatr Emerg Care 2017 Dec;33(12):815-816

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http://dx.doi.org/10.1097/PEC.0000000000001396DOI Listing
December 2017
4 Reads