2,115 results match your criteria Tricuspid Atresia


The Adult Patient with a Fontan.

Cardiol Clin 2020 Aug;38(3):379-401

Adult Congenital Heart Disease, Heart Centre, King Faisal Specialist Hospital and Research Centre, Zahrawi Street, Al Maather, Al Maazer, Riyadh 12713, Saudi Arabia. Electronic address:

The authors summarize the most important anatomic and physiologic substrates of Fontan circulation. Common anatomic substrates include hypoplastic left heart syndrome, tricuspid atresia, double inlet left ventricle, and unbalanced atrioventricular septal defects. After the Fontan operation exercise capacity is limited and the key hemodynamic drivers is limited preload due to a relatively fixed pulmonary vascular resistance. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ccl.2020.05.002DOI Listing

Hypnosis and superficial cervical anesthesia for total thyroidectomy in a high-risk patient - A case report.

Int J Surg Case Rep 2020 Jun 6;72:133-136. Epub 2020 Jun 6.

Department of Visceral Surgery, University Hospital of Lausanne and University of Lausanne Switzerland.

Introduction: Total thyroidectomy can be challenging in high-risk patients. Local cervical anesthesia with sedation is an alternative to general anesthesia.

Case Presentation: A 33-year old male patient with cyanotic congenital heart disease due to unrepaired tricuspid atresia type Ic and associated pulmonary arterial hypertension presented with tachycardic atrial fibrillation and amiodarone-induced thyrotoxicosis resulting in recurrent hemodynamic instability. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ijscr.2020.05.078DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7298319PMC

A NONSENSE GATA6 MUTATION EXPLAINS HISTORY OF CONGENITAL HEART DEFECTS AND 10 YEARS OF POORLY-CONTROLLED DIABETES LACKING DKA IN A NON-OBESE 30 YEAR-OLD INCIDENTALLY FOUND TO HAVE PANCREATIC HYPOPLASIA.

AACE Clin Case Rep 2020 May-Jun;6(3):e123-e126. Epub 2020 May 11.

Objective: To report a case of diabetes mellitus (DM) associated with partial pancreatic agenesis and congenital heart disease (CHD) in a patient found to have a nonsense mutation of the gene.

Methods: We present the imaging, laboratory, and genetic findings, and describe the clinical course of a patient with an atypical presentation of DM as well as CHD, who was found to have partial pancreatic agenesis on computed tomography (CT) imaging. Genetic testing was performed to identify monogenic DM. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.4158/ACCR-2019-0156DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7282284PMC

Tricuspid atresia: Where are we now?

J Card Surg 2020 Jun 2. Epub 2020 Jun 2.

School of Clinical Medicine, Addenbrooke's Hospital, University of Cambridge, Cambridge, UK.

Tricuspid atresia (TA) is a complex congenital heart disease that presents with cyanosis in the neonatal period. It is invariably fatal if left untreated and requires multiple stages of palliation. Early recognition and timely surgical intervention are therefore pivotal in the management of these infants. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/jocs.14673DOI Listing

Major aortopulmonary collateral arteries in a case of unrepaired tricuspid and pulmonary atresia with single ventricle physiology.

J Cardiovasc Comput Tomogr 2020 Apr 28. Epub 2020 Apr 28.

Department of Radiology, Weill Cornell Medicine, New York, NY, USA.

Tricuspid and pulmonary atresia with single ventricle physiology and major aortopulmonary collateral arteries (MAPCAs) is a complex cyanotic congenital heart disease with heterogeneous pulmonary artery morphology and arborization. The complex anatomy and physiology, coupled with a dearth of existing literature, pose imitable challenges to treatment. Although the exact surgical algorithm is still unclear, the goal is a well-developed, low-resistance pulmonary vascular bed. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jcct.2020.04.009DOI Listing

Tricuspid Regurgitation in Adults after Repair of Right Ventricular Outflow Obstructive Lesions.

Pediatr Cardiol 2020 May 11. Epub 2020 May 11.

Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, The University of Hong Kong, Hong Kong, China.

We determined the prevalence and factors associated with tricuspid regurgitation (TR) in adults with repair of right ventricular (RV) outflow obstruction. A total of 256 patients (128 males) were studied at 25.7 ± 7. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00246-020-02366-3DOI Listing
May 2020
1.550 Impact Factor

Single-Stage Fontan Operation in a Patient With Coronary Sinus Ostium Atresia.

World J Pediatr Congenit Heart Surg 2020 May;11(3):372-373

Pediatric Cardiac Surgery, Dr. Siyami Ersek Thoracic and Cardiovascular Surgery Training and Research Hospital, İstanbul, Turkey.

An eight-year-old boy with tricuspid atresia was found to have atretic coronary sinus ostium during cardiac catheterization. Single-stage extracardiac fenestrated Fontan operation was performed with surgical unroofing of the coronary sinus into the left atrium to avoid the risk of cardiac congestion. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/2150135120905511DOI Listing

Identification and analysis of KLF13 variants in patients with congenital heart disease.

BMC Med Genet 2020 04 15;21(1):78. Epub 2020 Apr 15.

Department of Pediatric Cardiology, Xinhua Hospital, Affiliated to Shanghai Jiao Tong University School of Medicine, 1665 Kongjiang Road, Shanghai, 200092, China.

Background: The protein Kruppel-like factor 13 (KLF13) is a member of the KLF family and has been identified as a cardiac transcription factor that is involved in heart development. However, the relationship between KLF13 variants and CHDs in humans remains largely unknown. The present study aimed to screen the KLF13 variants in CHD patients and genetically analyze the functions of these variants. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1186/s12881-020-01009-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7160950PMC

Heart Rate Responses During Exercise by Dominant Ventricle in Pediatric and Young Adult Patients With a Fontan Circulation.

Can J Cardiol 2019 Nov 15. Epub 2019 Nov 15.

Heart Centre, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.

Background: Patients with univentricular physiology palliated with the Fontan operation have multiple late cardiovascular and extracardiac complications, including autonomic dysfunction. Despite the observation, little is known about autonomic function driving exercise-related heart rate responses in Fontan patients and whether dominant ventricle subtype or underlying cardiac anatomy affects heart rate responses during exercise.

Methods: We performed a retrospective chart review of all single ventricle patients palliated with a Fontan operation who underwent a maximal effort cardiopulmonary exercise test at Cincinnati Children's Hospital Medical Center from 2013 to 2018. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.cjca.2019.10.042DOI Listing
November 2019

Does ascending aorta size affect Norwood outcomes in hypoplastic left heart with aortic atresia?

Ann Thorac Surg 2020 Mar 22. Epub 2020 Mar 22.

Washington University School of Medicine, Department of Surgery, Division of Cardiothoracic Surgery, 660 S Euclid Ave, St. Louis, MO 63110; St. Louis Children's Hospital, Section of Pediatric Cardiothoracic Surgery, One Children's Place, St. Louis, MO 63110. Electronic address:

Background: Conflicting data exists regarding the impact of ascending aorta size on outcomes following the Norwood procedure. Results from multi-institutional studies have largely relied on heterogeneous populations undergoing this surgery for different anatomic defects. Using data from the Single Ventricle Reconstruction Trial, we analyzed the impact of preoperative ascending aortic diameter on Norwood outcomes for patients with aortic atresia variants of hypoplastic left heart syndrome (HLHS). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.athoracsur.2020.02.037DOI Listing

Unusual case of tricuspid atresia and transposition of the great arteries presenting with aortic dissection: A rare condition.

Turk Kardiyol Dern Ars 2020 Mar;48(2):185

Research Institute of Cardiology, Tehran Heart Center, Tehran University of Medical Sciences, Tehran, Iran.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.5543/tkda.2019.89039DOI Listing

[Efficacy of transcatheter pulmonary valve perforation by micro-guidewire and balloon dilation in neonates with pulmonary atresia with intact ventricular septum].

Zhonghua Er Ke Za Zhi 2020 Feb;58(2):96-100

Department of Pediatric Cardiology, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200092, China.

To investigate the safety and efficacy of transcatheter perforation of pulmonary valve by micro-guidewire and balloon dilation in the treatment of neonatal pulmonary atresia with intact ventricular septum (PA-IVS). The retrospective study included 21 neonates (14 males and 7 females) with PA-IVS who underwent transcatheter micro-guidewire pulmonary valve perforation and balloon dilation in Xinhua Hospital from January 2012 to December 2018. All patients underwent the pulmonary valve perforation by micro-guidewire through the Simmons catheter. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3760/cma.j.issn.0578-1310.2020.02.006DOI Listing
February 2020

A Tribute to the Pioneers of Right Heart Bypass: An Historical Review.

Authors:
Giovanni Stellin

World J Pediatr Congenit Heart Surg 2020 Mar;11(2):198-203

Pediatric and Congenital Cardiac Surgery Unit, Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, Padua, Italy.

Cavopulmonary anastomosis was first described by Carlon, Mondini, De Marchi in a canine model in 1951 and later, in the clinical practice, by Glenn in 1958. Total right heart bypass was first introduced by Fontan and Kreutzer in 1971, in each instance as treatment for tricuspid atresia. Several modifications of such a procedure followed the initial concept of the right atrium as a pumping chamber, including modifications aimed to minimize energy loss at the anastomotic level and arrhythmias. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/2150135119894478DOI Listing

Quantification of Initial Right Ventricular Dimensions by Computed Tomography in Infants with Congenital Heart Disease and a Hypoplastic Right Ventricle.

Authors:
Hyun Woo Goo

Korean J Radiol 2020 Feb;21(2):203-209

Department of Radiology and Research Institute of Radiology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.

Objective: To demonstrate the feasibility of using cardiothoracic CT for quantification of the initial right ventricle (RV) dimensions in infants with congenital heart disease (CHD) and a hypoplastic RV and to compare these measurements with those obtained in a control group with CHD without a hypoplastic RV.

Materials And Methods: Initial RV dimensions, including RV volumes, RV/left ventricle (LV) volume ratios, atrioventricular valve annulus diameter ratios, and RV/LV length ratios based on CT data, were collected from 57 infants with CHD and a hypoplastic RV (hypoplastic RV group; age range, 1 day to 6 months) and 33 infants with tetralogy of Fallot (control group; age range, 1 day to 6 months) and compared between the 2 groups. The type of final surgery was also evaluated in the hypoplastic RV group over a follow-up period of 3-8 years. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3348/kjr.2019.0662DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6992442PMC
February 2020

Heart transplantation and in-hospital outcomes in adult congenital heart disease patients with Fontan: A decade nationwide analysis from 2004 to 2014.

J Card Surg 2020 Mar 23;35(3):603-608. Epub 2020 Jan 23.

Cardiovascular Division, Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee.

Introduction: Treatment of adult congenital heart disease patients who require advanced therapies remains challenging due to high perioperative and wait-list mortality and limited donors. Patients palliated with Fontan are at the highest risk of early mortality due to multiorgan involvement and few centers able to safely transplant them. We sought to evaluate the early outcomes of heart transplants in these adult Fontan patients. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/jocs.14430DOI Listing

Prenatally diagnosed case of tricuspid valve dysplasia: A case report with review of the literature.

Indian J Radiol Imaging 2019 Oct-Dec;29(4):452-456. Epub 2019 Dec 31.

Department of Ultrasound, Meera Hospital, Shiv Marg, Bani Park, Jaipur, Rajasthan, India.

We present a case of fetal tricuspid valve dysplasia (TVD) and pulmonary atresia, diagnosed during a routine obstetric ultrasound scan. Serial fetal echocardiographic evaluations revealed progressively augmented prodigious thickening of the tricuspid valvular and subvalvular structures, which eventually extensively obliterated the right ventricle cavity. Thickened dysplastic valve displayed a "cotton-wool" appearance. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.4103/ijri.IJRI_463_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6958872PMC
December 2019

Long-term therapeutic effect of Fontan conversion with an extracardiac conduit.

Eur J Cardiothorac Surg 2020 May;57(5):951-957

Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan.

Objectives: The aim of this study was to identify the long-term therapeutic effect of total cavopulmonary connection (TCPC) conversion with an extracardiac conduit.

Methods: Between 1991 and 2014, 36 patients underwent TCPC conversion with an extracardiac conduit. Half of these patients were diagnosed with tricuspid atresia or its variant. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1093/ejcts/ezz355DOI Listing

Diagnosis of major heart defects by routine first-trimester ultrasound examination: association with increased nuchal translucency, tricuspid regurgitation and abnormal flow in ductus venosus.

Ultrasound Obstet Gynecol 2020 May;55(5):637-644

Fetal Medicine Research Institute, King's College Hospital, London, UK.

Objective: To examine the association between fetal major heart defects and increased nuchal translucency thickness (NT), tricuspid regurgitation and abnormal flow in the ductus venosus in a large population of singleton pregnancies undergoing routine ultrasound examination at 11-13 weeks' gestation.

Methods: This was a retrospective study of prospectively collected data from singleton pregnancies attending for a routine ultrasound scan at 11-13 weeks' gestation, which included examination of fetal anatomy, measurement of NT and assessment of blood flow across the tricuspid valve and in the ductus venosus, according to a standardized protocol. The incidence of fetal NT ≥ 95 and ≥ 99 percentiles, tricuspid regurgitation and reversed a-wave in the ductus venosus in fetuses with and those without a major heart defect was determined and the performance of each marker and their combination in the detection of major heart defects was calculated. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/uog.21956DOI Listing

Isolated left superior vena cava in tricuspid atresia with a rare reverse venous garland.

J Cardiovasc Comput Tomogr 2019 Nov 26. Epub 2019 Nov 26.

Department of Cardiovascular Radiology & Endovascular Interventions, All India Institute of Medical Sciences, New Delhi, 110029, India. Electronic address:

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jcct.2019.11.013DOI Listing
November 2019

A 39-Year-Old Male Congenital Tricuspid Atresia Patient Who Presented with a New Axillary Lesion after an Orthotopic Heart Transplant.

Dermatopathology (Basel) 2019 Oct-Dec;6(4):220-224. Epub 2019 Oct 30.

Department of Dermatology, Massachusetts General Hospital, Boston, Massachusetts, USA.

Mucormycosis is a rare and aggressive fungal infection, most often caused by species of the , , , , and genera. The condition most commonly affects patients with uncontrolled diabetes, HIV/AIDS, malignancy, and those receiving long-term immunosuppressive therapy. We report the case of a 39-year-old male with biopsy-proven cutaneous mucormycosis of the left axilla 4 months after an orthotopic heart transplant for congenital tricuspid atresia. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1159/000503661DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6902244PMC
October 2019

Tricuspid atresia with absent pulmonary valve and intact ventricular septum: successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle.

Cardiol Young 2020 Jan 26;30(1):126-128. Epub 2019 Nov 26.

Department of Pediatrics, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea.

Tricuspid atresia with absent pulmonary valve and intact ventricular septum is an extremely rare cardiac malformation, historically associated with a poor prognosis. Only a few cases with successful surgical palliation have been reported in the literature. We present the case of an 8-month-old infant with this malformation who underwent successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1017/S1047951119002749DOI Listing
January 2020
4 Reads

Failure of Cellularization of Ventriculotomy Patch Leading to Right Ventricular Pseudoaneurysm.

World J Pediatr Congenit Heart Surg 2020 Jan 22;11(1):123-126. Epub 2019 Nov 22.

Department of Pediatric Cardiology, Cleveland Clinic Children's Hospital, Cleveland, OH, USA.

Enlargement of the bulboventricular foramen (BVF) in double-inlet left ventricle or the ventricular septal defect (VSD) in tricuspid atresia with transposition of the great arteries is one approach for prevention or treatment of systemic ventricular outflow obstruction. Most often, BVF/VSD restriction is bypassed preemptively or addressed directly at the time of Glenn/Fontan procedures as part of staged univentricular palliation. We describe a patient who underwent enlargement of a restrictive VSD during Fontan completion and subsequently presented with an asymptomatic pseudoaneurysm of the right ventricle at the ventriculotomy site. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/2150135119880547DOI Listing
January 2020

A rare case of adult congenital heart disease: single ventricular chamber with anomalous right coronary artery in an octogenarian.

J Community Hosp Intern Med Perspect 2019 1;9(5):446-448. Epub 2019 Nov 1.

Department of Cardiology, Rapides Regional Medical Center, Alexandria, LA, USA.

Patients with single-ventricle physiology encompass a wide array of anatomic subtypes, including but not limited to: tricuspid atresia, hypoplastic left heart syndrome, double-outlet or double-inlet ventricles. The outcomes for patients with single ventricle born before 1990 are relatively poor. An 81-year-old female presented to the hospital as non-ST elevation myocardial infarction. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1080/20009666.2019.1655625DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6830298PMC
November 2019

Acute ischaemic stroke in secondary polycythaemia due to complex congenital cyanotic heart disease.

BMJ Case Rep 2019 Oct 30;12(10). Epub 2019 Oct 30.

Department of Neurology, Christian Medical College & Hospital Ludhiana, Ludhiana, India

A 65-year-old woman presented to the emergency department with an acute onset of left-sided hemiparesis and slurred speech for 6 hours. Physical examination was notable for clubbing and peripheral cyanosis. Brain MRI showed an acute infarct involving the right gangliocapsular region. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1136/bcr-2019-231261DOI Listing
October 2019
1 Read

Prenatal echocardiographic classification and prognostic evaluation strategy in fetal pulmonary atresia with intact ventricular septum.

Medicine (Baltimore) 2019 Oct;98(42):e17492

Children's Heart Center, Henan Provincial People's Hospital Cardiac Center, Fuwai Central China Cardiovascular Hospital, People's Hospital of Zhengzhou University Cardiac Center, Zhengzhou, China.

Fetal pulmonary atresia with intact ventricular septum (PA/IVS) is a rare congenital heart disease. The present study aimed to classify PA/IVS and determine the relationship between prenatal echocardiographic characteristics and postnatal biventricular or univentricular repair strategies.A total of 51 fetuses with PA/IVS were examined from 2012 to 2019. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1097/MD.0000000000017492DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6824646PMC
October 2019
1 Read

Catheter ablation for patients with end-stage complex congenital heart disease or cardiomyopathy considered for transplantation: Trials and tribulations.

Int J Cardiol 2020 02 10;301:127-134. Epub 2019 Sep 10.

Department of Cardiology, Royal Brompton and Harefield Hospital, Imperial College London, United Kingdom. Electronic address:

Introduction: Arrhythmia contributes significantly to morbidity and mortality of patients with congenital heart disease (CHD) or cardiomyopathy (CMP). It also has the potential to worsen symptoms and is particularly detrimental to patients with advanced heart failure awaiting cardiac transplantation. We report our experience using catheter ablation to treat recurrent arrhythmia in patients with CHD or CMP considered for transplantation. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ijcard.2019.09.021DOI Listing
February 2020
1 Read

Pregnancy in Women with Complex Congenital Heart Disease. A Constant Challenge.

Arq Bras Cardiol 2019 12;113(6):1062-1069

Instituto de Coração do Departamento de Cardiopneumologia da Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP - Brazil.

Background: The improvement in surgical techniques has contributed to an increasing number of childbearing women with complex congenital heart disease (CCC). However, adequate counseling about pregnancy in this situation is uncertain, due to a wide variety of residual cardiac lesions.

Objectives: To evaluate fetal and maternal outcomes in pregnant women with CCC and to analyze the predictive variables of prognosis. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.5935/abc.20190197DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7021269PMC
December 2019
1 Read

Superior vena cava obstruction after the Glenn procedure.

Autops Case Rep 2019 Jul-Sep;9(3):e2019118. Epub 2019 Sep 11.

Universidade de São Paulo, Ribeirão Preto Medical School, Department of Pediatrics. Ribeirão Preto, SP, Brazil.

Acute obstruction of superior vena cava anastomosis right after the Glenn procedure may lead to tragic consequences. We describe the case of a one-year-old child with tricuspid atresia and a previous Blalock-Taussig shunt procedure, who presented severe low cardiac output syndrome right after the Glenn procedure and died forty-four hours after the procedure. The autopsy showed obstruction of the superior vena cava anastomosis. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.4322/acr.2019.118DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6746204PMC
September 2019
2 Reads

Tricuspid atresia with Type-III levo-transposition of great arteries: A tryst with twin tribulations.

J Cardiovasc Comput Tomogr 2019 Sep 23. Epub 2019 Sep 23.

Department of Cardiovascular Radiology & Endovascular Interventions, All India Institute of Medical Sciences, New Delhi, 110029, India. Electronic address:

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jcct.2019.09.010DOI Listing
September 2019
2 Reads

Quantitative Assessment of Left Ventricular Dysfunction in Fetal Ebstein's Anomaly and Tricuspid Valve Dysplasia.

J Am Soc Echocardiogr 2019 12 21;32(12):1598-1607. Epub 2019 Sep 21.

Division of Cardiology, Department of Pediatrics, Fetal and Neonatal Cardiology Program, Women's and Children Health Research Institute, University of Alberta, Edmonton, Alberta, Canada. Electronic address:

Background: Fetal Ebstein's anomaly and tricuspid valve dysplasia (EA/TVD) are associated with high perinatal mortality relative to pulmonary atresia with intact ventricular septum (PAIVS), despite both requiring redistribution of the cardiac output (CO) to the left ventricle (LV). LV dysfunction is suspected to contribute to adverse outcomes in EA/TVD.

Objective: We sought to examine global and segmental LV function in fetal EA/TVD with comparison to normal controls and PAIVS. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.echo.2019.07.008DOI Listing
December 2019
3 Reads

Subaxillary bidirectional cavopulmonary anastomosis in an infant.

J Thorac Cardiovasc Surg 2020 03 25;159(3):e235-e237. Epub 2019 Aug 25.

Pediatric Cardiac Surgery Unit, CEDIMAT Cardiovascular Center, Santo Domingo, Dominican Republic.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jtcvs.2019.07.090DOI Listing

Double Orifice Mitral Valve in Tricuspid Atresia: A Rare Association.

Pediatr Cardiol 2019 Dec 10;40(8):1761-1762. Epub 2019 Sep 10.

Fondazione G. Monasterio CNR-Regione Toscana, Massa and Pisa, Italy.

We here report a neonate with prenatal echocardiographic diagnosis of tricuspid atresia, with normally related great vessels, and large ventricular septal defect. This diagnosis could be confirmed with echocardiography at birth. An additional double mitral orifice was also seen. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00246-019-02204-1DOI Listing
December 2019
3 Reads

Tricuspid Atresia With Truncus Arteriosus: Off-Pump Stage I Palliation.

World J Pediatr Congenit Heart Surg 2019 09;10(5):635-637

Department of Pediatric Cardiac Sciences, Sir Ganga Ram Hospital, New Delhi, Delhi, India.

Coexistence of tricuspid atresia and truncus arteriosus (common arterial trunk) is an extremely rare combination of anomalies and requires staged palliation. Initial palliation involves disconnection of the pulmonary arteries from the common trunk and creation of a systemic-to-pulmonary artery shunt. This has generally been accomplished with the use of cardiopulmonary bypass. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/2150135119847607DOI Listing
September 2019
2 Reads

Short-term results in infants with multiple left heart obstructive lesions.

Congenit Heart Dis 2019 Nov 6;14(6):1193-1198. Epub 2019 Sep 6.

Department of Pediatric Cardiology, Cleveland Clinic Children's, Cleveland, Ohio.

Objective: Deciding on a surgical pathway for neonates with ≥2 left heart obstructive lesions is complex. Predictors of the successful biventricular (2V) repair in these patients are poorly defined. The goal of our study was to identify patients who underwent the 2V repair and assess anatomic and echocardiographic predictors of success. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/chd.12829DOI Listing
November 2019
4 Reads

Relation of Magnetic Resonance Elastography to Fontan Failure and Portal Hypertension.

Am J Cardiol 2019 11 7;124(9):1454-1459. Epub 2019 Aug 7.

Cincinnati Children's Hospital Medical Center, Department of Radiology, University of Cincinnati College of Medicine Cincinnati, Ohio.

Fontan associated liver disease is associated with morbidity and mortality in palliated single-ventricle congenital heart disease patients. Magnetic resonance elastography (MRE) provides a quantitative assessment of liver stiffness in Fontan patients. We hypothesized that MRE liver stiffness correlates with liver enzymes, hemodynamics, portal hypertension, and Fontan failure (FF). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.amjcard.2019.07.052DOI Listing
November 2019
1 Read

Intravascular Papillary Endothelial Hyperplasia of the Maxillary Sinus in Patient with Tricuspid Atresia.

Head Neck Pathol 2019 Aug 31. Epub 2019 Aug 31.

Department of Neurosurgery, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Intravascular papillary endothelial hyperplasia (IPEH) is a benign, highly vascularized, endothelial growth that can be mischaracterized as a malignancy. While hundreds of IPEH cases are reported, only four occurred in the maxillary sinus. We present the case of a 28-year-old male who underwent surgical resection of IPEH of the right maxillary sinus. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s12105-019-01070-wDOI Listing
August 2019
2 Reads

Wolff-Parkinson-White Syndrome after Fontan-Bjork operation and its Successful Ablation from Coronary Sinus.

Authors:
Mustafa Yolcu

J Coll Physicians Surg Pak 2019 Sep;29(9):886-887

Department of Cardiology, Medicine Faculty, Yeni Yuzyil Universty, Istanbul, Turkey.

Wolff-Parkinson-White (WPW) syndrome causes paroxysmal supraventricular tachycardia in which short PR intervals and delta waves are seen in electrocardiography, which may cause sudden cardiac death. A 19-year female presented with increasing episodes of wide and narrow QRS complex tachycardia for the past 5 years. She had tricuspid atresia and Fontan Bjork operation in her past history. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.29271/jcpsp.2019.09.886DOI Listing
September 2019
4 Reads

Severe Pulmonary Stenosis or Atresia with Intact Ventricular Septum in the Fetus: The Natural History.

Fetal Diagn Ther 2020 27;47(5):420-428. Epub 2019 Aug 27.

Division of Prenatal Medicine, Department of Obstetrics and Gynecology, University of Cologne, Cologne, Germany.

Purpose: To assess the intrauterine course, the outcome, and to establish a new prenatal echocardiographic scoring system to predict biventricular (BV) versus univentricular (UV) outcome of fetuses with severe pulmonary stenosis or atresia with intact ventricular septum (PSAIVS).

Methods: All cases of PSAIVS diagnosed prenatally over a period of 14years were retrospectively collected in 2 tertiary referral centers.

Results: Forty-nine fetuses with PSIVS (n = 11) or PAIVS (n = 38) were identified prenatally. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1159/000502178DOI Listing
August 2019
2 Reads

Diagnosis of fetal non-chromosomal abnormalities on routine ultrasound examination at 11-13 weeks' gestation.

Ultrasound Obstet Gynecol 2019 Oct;54(4):468-476

Fetal Medicine Research Institute, King's College Hospital, London, UK.

Objective: To examine the performance of the routine 11-13-week scan in detecting fetal non-chromosomal abnormalities.

Methods: This was a retrospective study of prospectively collected data from 100 997 singleton pregnancies attending for a routine ultrasound examination of fetal anatomy, performed according to a standardized protocol, at 11-13 weeks' gestation. All continuing pregnancies had an additional scan at 18-24 weeks and 71 754 had a scan at either 30-34 or 35-37 weeks. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/uog.20844DOI Listing
October 2019
6 Reads

Aortic root replacement in a patient with unoperated tricuspid atresia.

Cardiol Young 2019 Sep 6;29(9):1211-1213. Epub 2019 Aug 6.

Department of Cardiovascular Surgery, Chiba Cerebral and Cardiovascular Center, Chiba, Japan.

A 42-year-old man was referred to our hospital with heart failure and unoperated tricuspid atresia with pulmonary valve stenosis. His condition was initially managed with medical therapy; however, he required repeat hospitalisations for congestive heart failure. We diagnosed the chief cause of his heart failure as aortic valve regurgitation secondary to aortic root dilatation. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1017/S1047951119001719DOI Listing
September 2019
3 Reads

Cardiomyopathy presenting prenatally with functional tricuspid and pulmonary atresia.

Echocardiography 2019 09 5;36(9):1779-1782. Epub 2019 Aug 5.

Saint Louis University School of Medicine, St. Louis, MO, USA.

Cardiomyopathy is a rare diagnosis in the fetus that usually presents as a dilated, poorly functioning ventricle. We present the case of a fetus that developed functional tricuspid and pulmonary atresia due to progressive right ventricular cardiomyopathy. The baby was supported with prostaglandin and inotropic infusions after delivery, eventually weaning off without need for surgical intervention. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/echo.14446DOI Listing
September 2019
4 Reads

149 Fontan Conversions.

Methodist Debakey Cardiovasc J 2019 Apr-Jun;15(2):105-110

FLORIDA HOSPITAL FOR CHILDREN, ORLANDO, FLORIDA.

Patients with a functionally univentricular heart who have had an atriopulmonary Fontan are at risk for atrial dilatation, atrial arrhythmias, and progressive circulatory failure. Between 1994 and 2018, we performed 149 Fontan conversions with arrhythmia surgery and epicardial pacemaker placement at Ann & Robert H. Lurie Children's Hospital of Chicago. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.14797/mdcj-15-2-105DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6668738PMC
August 2019
19 Reads

Prediction of biventricular repair by echocardiography in borderline ventricle.

Chin Med J (Engl) 2019 Sep;132(17):2105-2108

Heart Center, Children's Hospital of Fudan University, Shanghai 201102, China.

Objective: In recent years, attempting the biventricular pathway or biventricular conversions in patients with borderline ventricle has become a hot topic. However, inappropriate pursuit of biventricular repair in borderline candidates will lead to adverse clinical outcomes. Therefore, it is important to accurately assess the degree of ventricular development before operation and whether it can tolerate biventricular repair. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1097/CM9.0000000000000375DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6793783PMC
September 2019
3 Reads

Heart Transplantation in a Fontan Patient Using a Hepatitis C-Positive Donor.

World J Pediatr Congenit Heart Surg 2019 07;10(4):504

1 Cardiovascular Medicine, Vanderbilt University Medical Center, Nashville, TN, USA.

A 38-year-old female with tricuspid atresia and normally related great arteries, initially palliated with Björk modified Fontan, and ultimately converted to extracardiac conduit Fontan, with a history of ventricular tachycardia and hepatitis C virus (HCV) treated with sofosbuvir/ledipasvir, was referred to our center for consideration of combined heart and liver transplantation. The patient's blood group was O with panel reactive antibodies of 52%. She consented to consideration of HCV-positive donors. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/2150135119852037DOI Listing
July 2019
7 Reads

Ostial atresia of the coronary sinus in patients with supraventricular arrhythmias.

J Arrhythm 2019 Jun 16;35(3):554-557. Epub 2019 May 16.

Department of Cardiology Kokura Memorial Hospital Kitakyushu Japan.

Of 3577 patients with supraventricular arrhythmias, 3 demonstrated an atresia of the coronary sinus (CS) ostium. Two patients had the accessory pathways. One had atrial fibrillation. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/joa3.12189DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6595369PMC
June 2019
3 Reads

[Joint Cardiovascular Surgeries of Two Hospitals in Shizuoka for Adult Congenital Heart Disease (ACHD) Patients].

Kyobu Geka 2019 Apr;72(4):290-295

Department of Cardiovascular Surgery, Shizuoka Children's Hospital, Shizuoka, Japan.

Background: As for the medical management including surgery for the patients with adult congenital heart disease(ACHD), it may be difficult for most hospitals other than that have both adult/congenital cardiologists/cardiovascular surgeons. Between Shizuoka Prefectural General Hospital and Mt. Fuji Shizuoka Children's Hospital, medical stuff and information have been shared for these 4 years. Read More

View Article

Download full-text PDF

Source
April 2019
4 Reads

Evaluation and Management of the Child and Adult With Fontan Circulation: A Scientific Statement From the American Heart Association.

Circulation 2019 Jul 1:CIR0000000000000696. Epub 2019 Jul 1.

It has been 50 years since Francis Fontan pioneered the operation that today bears his name. Initially designed for patients with tricuspid atresia, this procedure is now offered for a vast array of congenital cardiac lesions when a circulation with 2 ventricles cannot be achieved. As a result of technical advances and improvements in patient selection and perioperative management, survival has steadily increased, and it is estimated that patients operated on today may hope for a 30-year survival of >80%. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1161/CIR.0000000000000696DOI Listing
July 2019
24 Reads