scholarly journals A Case of Congenitally Corrected Transposition of Great Arteries (CCTGA)

2015 ◽  
Vol 4 (1) ◽  
pp. 37-40
Author(s):  
Tofayel Uddin Ahmed ◽  
M Mafizur Rahman ◽  
Parvez Rahman Khan ◽  
Mohammad Rajibul Hasan
Keyword(s):  
Congenital Heart ◽  
Heart Diseases ◽  
Heart Defects ◽  
Complex Form ◽  
Exertional Dyspnea ◽  
Transposition Of Great Arteries ◽  
Scientific Attention ◽  
Congenitally Corrected Transposition ◽  
Complete Av Block ◽  
Corrected Transposition

Recent years, much scientific attention has been given to congenital heart diseases (CHD) and probable complications. Congenitally corrected transposition of the great arteries (CCTGA) is a rare, complex form of congenital heart defects. CCTGA is characterized by atrioventricular (AV) and ventriculoarterial (VA) discordance and hence by a physiologically normal direction of blood flow sometimes called "double discordance". The development of complete AV block and global ventricular dysfunction has been identified as the cause of cardiac death. This paper presents a case of CCTGA with rhythm disorders and exertional dyspnea. CBMJ 2015 January: Vol. 04 No. 01 P: 37-40

scholarly journals Anaesthetic management of a case of congenitally corrected transposition of great arteries for non cardiac surgery: A case report

2021 ◽  
Vol 8 (2) ◽  
pp. 341-344
Author(s):  
Heena D Pahuja ◽  
Charuta P Gadkari ◽  
Neha G Wakode ◽  
Anjali R Bhure
Keyword(s):  
Cardiac Surgery ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Anaesthetic Management ◽  
Left Ventricular ◽  
Complex Congenital Heart Disease ◽  
Associated Anomalies ◽  
Transposition Of Great Arteries ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

Congenitally corrected transposition of the great arteries (CCTGA) is a rare form of congenital heart diseases, present with or without associated anomalies. It is a complex congenital heart disease with both atrioventricular and ventriculoarterial discordance. Such patients tend to develop systemic ventricular dysfuction with the stress of surgery. Patients with CCTGA are usually diagnosed at early stages of life due to associated anomalies, but they may even remain asymptomatic till later decades of their life. Literature search revealed very few reported cases of anaesthetic management of such high risk cases for non cardiac surgery. These patients have the tendency to develop cardiac dysrhythmias and left ventricular failure during intraoperative and postoperative period. We report anaesthetic management of a case of a 24 years old male with the congenitally corrected transposition of great arteries(CCTGA) who was operated for fracture left distal radius and ulna and right sided galeazzi fracture.

Ventricular assist device in a patient with congenitally corrected transposition of the great arteries and situs inversus totalis

2019 ◽  
Vol 42 (6) ◽  
pp. 321-322
Author(s):  
Radi Wieloch ◽  
Nihat Firat Sipahi ◽  
Udo Boeken ◽  
Alexander Albert ◽  
Payam Akhyari ◽  
...  
Keyword(s):  
Ventricular Assist Device ◽  
Situs Inversus ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Situs Inversus Totalis ◽  
Clinical Scenario ◽  
Assist Device ◽  
Ventricular Assist ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

Congenitally corrected transposition of the great arteries and situs inversus totalis are rare congenital anomalies. While congenital heart diseases affect about 0.75%–0.9% of newborns, less than 1% of them have congenitally corrected transposition of the great arteries. Meanwhile, the incidence of situs inversus totalis is about 0.01%. This is a case report of a patient with congenitally corrected transposition of the great arteries and situs inversus totalis who was supported with a ventricular assist device, resulting in a challenging clinical scenario.

scholarly journals A Case of Congenitally Corrected Transposition of Great Arteries: An Infrequent Happenstance

2017 ◽  
Vol 2017 ◽  
pp. 1-4
Author(s):  
Prakash Ajmera ◽  
Vikas Medep
Keyword(s):  
Ventricular Hypertrophy ◽  
Heart Diseases ◽  
Valvular Regurgitation ◽  
Associated Anomalies ◽  
Transposition Of Great Arteries ◽  
The Past ◽  
Congenitally Corrected Transposition ◽  
Degree Heart Block ◽  
Av Dissociation ◽  
Corrected Transposition

Congenitally corrected transposition of the great arteries (CCTGA) is rare form of congenital heart diseases. It may be present with or without associated anomalies. Patients with CCTGA are usually diagnosed at early stages of life due to associated anomalies, but they may even remain asymptomatic till later decades of their life. We report a case of a 42-year-old man who presented at neurosurgery department with dizziness, seizures, and loss of consciousness, in whom isolated CCTGA was discovered incidentally. Further investigation depicted right ventricular hypertrophy, mild valvular regurgitation, mildly dilated pulmonary artery, low heart rate with AV dissociation, and third-degree heart block. These indicated for implantation of permanent pacemaker into the patient. The implantation of VVI mode pacemaker was uneventful and the patient is being followed up in the past eight months in favorable condition.

scholarly journals The possibilities of computed tomography using heart-axis-oriented multiplanar reformations in diagnostics of the great arteries transposition

2018 ◽  
Vol 9 (4) ◽  
pp. 28-35
Author(s):  
Gulnaz K. Sadykova ◽  
Dmitry O. Ivanov ◽  
Georgiy O. Bagaturia ◽  
Victor V. Ipatov ◽  
Vlarimir V. Ryazanov
Keyword(s):  
Computed Tomography ◽  
Data Analysis ◽  
Heart Diseases ◽  
Transposition Of Great Arteries ◽  
Computed Tomographic ◽  
Heart Chamber ◽  
Multiplanar Reformations ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

This article contents the results of computed tomography with intravenous bolus contrast media administration data analysis in children with transposition and congenitally corrected transposition of the great arteries with the consequental performing of the multiplanar heart-axis-oriented reformations. Among 148 examined children transposition of great arteries was detected in 13 patients (9 boys and 4 girls aged 1-144 day of life); congenitally corrected transposition was found in 4 cases of children aged from 6 months to 15 years and 6 months (2 boys and 2 girls). In this article comprehensive anatomical criteria of each heart chamber morphology are presented and reformations where these criteria can be seen are shown. Also in the article is given comparative characterization of heart and great arteries structures in transposition and congenitally corrected transposition in every certain heart-axis-oriented reformation. By the results of consequently performed heart-axis-oriented reformations data analysis the peculiar anatomical signs of transposition and congenitally corrected transposition are determined. The results of data analysis show that from the list of offered reformations the peculiar anatomical signs of both kinds of transposition are significantly determined in long-axis of right ventricle inflow tract reformation, left heart chambers reformation, supply ventricle division reformation, short-axis reformation at the level of great arteries. Computed tomographic angiocardiography heart-axis-oriented multiplanar reformations permit full and correct assessment of heart and main vessels, which is important for planning of surgical treatment in congenital heart diseases.

scholarly journals Veleszületett szívhibák sebészi kezelése 60 éves kor felett

2016 ◽  
Vol 157 (21) ◽  
pp. 820-824
Author(s):  
István Hartyánszky ◽  
Sándor Varga ◽  
László Csepregi ◽  
Barna Babik ◽  
Judit Simon ◽  
...  
Keyword(s):  
Heart Disease ◽  
Valve Replacement ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Heart Defects ◽  
Multiorgan Failure ◽  
Ductus Arteriosus ◽  
Right Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Transposition Of Great Arteries

Introduction: The population with congenital heart disease is increasing and ageing. Aim: The aim of the authors was to examine the outcome of surgical management of congenital heart diseases beyond the age of 60 years. Method: Between 2013 and 2015, 77 adults were operated (36 younger, and 41 older than forty years, including 12 patients aged over 60 years. The numbers of procedures were as follows (in brackets the number of operations in the three age groups): Ross surgery 5 (3, 2, 0); aortic valve replacement 19 (12, 1, 6); subaortic membrane resection 1 (0, 0, 1); Bentall/ascending aortic plasty 8 (4, 3, 1); myectomy with or without mitral valve replacement in left ventricular outflow obstruction 5 (0, 3, 2); aortic coarctation 1 (1, 0, 0); ligation of ductus arteriosus 2 (1, 1, 0); reconstruction of right ventricular outflow tract with biological valve 4 (0, 3, 1); homograft 5 (5, 0, 0); BioValsalva graft 1 (0, 1, 0); primary reconstruction of complete atrioventricular septum defect 3 (1, 2, 0); valve replacement 2 (1, 1, 0); ventricular septum defect 10 (4, 6, 0); atrial septum defect closure 5 (2, 3, 0); total cavopulmonal anastomosis 1 (1, 0, 0); valve replacement in congenital transposition of great arteries 1 (0, 1, 0), Ebstein operation with valve plasty 2 (0, 1, 1); valve replacement 2 (1, 0, 1). Results: There was no operative mortality, while early mortality occurred in one patient with total cavopulmonal anastomosis due to multiorgan failure. Conclusions: Congenital heart defects can be operated beyond the age of 60 years with good results in a tertiary heart centre having great experience in the management of congenital and acquired heart disease. Orv. Hetil., 2016, 157(21), 820–824.

scholarly journals Perspectives in the management of congenital heart defects in adult patients

2015 ◽  
Vol 156 (3) ◽  
pp. 92-97
Author(s):  
István Hartyánszky ◽  
Sándor Varga ◽  
Kálmán Havasi ◽  
Barna Babik ◽  
Márta Katona ◽  
...  
Keyword(s):  
Heart Transplantation ◽  
Congenital Heart Defects ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Heart Defects ◽  
Adult Patients ◽  
Univentricular Heart ◽  
Transposition Of Great Arteries ◽  
Number Of Patients

Due to improving results in congenital heart surgery, the number of adult patients with congenital heart defect is increasing. The question is: what kind of problems can be managed in this patient-group? The authors review the different problems of management of congenital heart defects in adults based on national and international literature data. Simple defects recognised in adults, postoperative residual problems, changing of small grafts and valves, correction of primary or operated coarctation aortae can be usually managed without problems. A very close follow-up is necessary to establish the correct period for heart transplantation in patients with transposition of great arteries with Senning/Mustard operation, and univentricular heart corrected with “Fontan-circulation” type surgical procedure. The authors conclude that although the number of patients increases, only a few congenital heart diseases may cause problems. It seems important (1) to monitor asymptomatic patient who underwent operation (Fallot-IV, Ross procedure, etc.), (2) follow up regularly patients who underwent Senning/Mustard procedure (magnetic resonance imaging, echocardiography, brain natriuretic peptide measurement), (3) define the proper period of preparation for heart transplantation of patients with a univentricular heart, with special attention to the possibility of multiorgan (lung, liver, etc.) failure. Due to the improvement of foetal diagnosis of congenital heart defects, the number of patients with complex congenital heart defects is decreasing. The standard management of these patients could be primary heart transplantation in infancy. Orv. Hetil., 2015, 156(3), 92–97.

scholarly journals Discordance is the Key

2017 ◽  
Vol 02 (01) ◽  
pp. 052-055
Author(s):  
K. Prasad ◽  
A. Kumar
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Mechanical Complication ◽  
Rare Form ◽  
Detailed Evaluation ◽  
Artery Disease ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

AbstractCongenitally corrected transposition of the great arteries (CCTGA) is a rare form of congenital heart disease characterised by atrioventricular as well as ventriculoarterial discordance. It is usually associated with a variety of severe intracardiac defects. Few patients with this abnormality survive past 50 years. A 54 Yr old male patient referred to our hospital for the management of post MI VSD. At the presentation Patient had mild central cyanosis, clubbing grade 2 and the vitals are stable. Cardiac examination revealed a 2/6 long ESM in a left parasternal area with pulsations in left 2nd and 3rd ICS. There is a lot of discrepancy in both clinical examination and workup. After detailed evaluation patient is diagnosed as having cyanotic congenital heart disease – L TGA + VSD with PS. Survival up to this age is very rare with this combination of associated defects in LTG. The patient is misdiagnosed as coronary artery disease with mechanical complication in another hospital. Unless there is suspicion and having knowledge about the asymptomatic presentation of congenital heart diseases up to this age group, patients will be misdiagnosed and treatment will be delayed.

Galectin-3 plasma levels in adult congenital heart disease and the pressure overloaded right ventricle: reason matters

2020 ◽  
Vol 14 (13) ◽  
pp. 1197-1205
Author(s):  
Ewa Kowalik ◽  
Beata Kuśmierczyk-Droszcz ◽  
Anna Klisiewicz ◽  
Aleksandra Wróbel ◽  
Anna Lutyńska ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Adult Congenital Heart Disease ◽  
Clinical Status ◽  
Pressure Overload ◽  
Cross Sectional ◽  
Galectin 3 ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

Aim: To assess galectin-3 (Gal-3) levels and their relationship with clinical status and right ventricular (RV) performance in adults with RV pressure overload of various mechanisms due to congenital heart disease. Materials & methods: A cross-sectional study was conducted. Patients underwent clinical examination, blood testing and transthoracic echocardiography. Results: The study included 63 patients with congenitally corrected transposition of the great arteries, 41 patients with Eisenmenger syndrome and 20 healthy controls. Gal-3 concentrations were higher in patients compared with controls (7.83 vs 6.11 ng/ml; p = 0.002). Biomarker levels correlated with age, New York Health Association class, N-terminal probrain natriuretic peptide and RV function only in congenitally corrected transposition of the great arteries patients. Conclusion: Gal-3 profile in congenital heart disease patients and pressure-overloaded RV differs according to the cause of pressure overload.

scholarly journals Personalized Genetic Diagnosis of Congenital Heart Defects in Newborns

2021 ◽  
Vol 11 (6) ◽  
pp. 562
Author(s):  
Olga María Diz ◽  
Rocio Toro ◽  
Sergi Cesar ◽  
Olga Gomez ◽  
Georgia Sarquella-Brugada ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart Defects ◽  
Congenital Malformations ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Heart Defects ◽  
Genetic Diagnosis ◽  
Single Nucleotide Variants ◽  
Personalized Approach

Congenital heart disease is a group of pathologies characterized by structural malformations of the heart or great vessels. These alterations occur during the embryonic period and are the most frequently observed severe congenital malformations, the main cause of neonatal mortality due to malformation, and the second most frequent congenital malformations overall after malformations of the central nervous system. The severity of different types of congenital heart disease varies depending on the combination of associated anatomical defects. The causes of these malformations are usually considered multifactorial, but genetic variants play a key role. Currently, use of high-throughput genetic technologies allows identification of pathogenic aneuploidies, deletions/duplications of large segments, as well as rare single nucleotide variants. The high incidence of congenital heart disease as well as the associated complications makes it necessary to establish a diagnosis as early as possible to adopt the most appropriate measures in a personalized approach. In this review, we provide an exhaustive update of the genetic bases of the most frequent congenital heart diseases as well as other syndromes associated with congenital heart defects, and how genetic data can be translated to clinical practice in a personalized approach.

scholarly journals How do caregivers of children with congenital heart diseases access and navigate the healthcare system in Ethiopia?

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Sugy Choi ◽  
Heesu Shin ◽  
Jongho Heo ◽  
Etsegenet Gedlu ◽  
Berhanu Nega ◽  
...  
Keyword(s):  
Health Care ◽  
Cardiac Surgery ◽  
Health Care System ◽  
Low Income ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Heart Defects ◽  
Care Facility ◽  
Long Distance ◽  
Care System

Abstract Background Surgery can correct congenital heart defects, but disease management in low- and middle-income countries can be challenging and complex due to a lack of referral system, financial resources, human resources, and infrastructure for surgical and post-operative care. This study investigates the experiences of caregivers of children with CHD accessing the health care system and pediatric cardiac surgery. Methods A qualitative study was conducted at a teaching hospital in Ethiopia. We conducted semi-structured interviews with 13 caregivers of 10 patients with CHD who underwent cardiac surgery. We additionally conducted chart reviews for triangulation and verification. Interviews were conducted in Amharic and then translated into English. Data were analyzed according to the principles of interpretive thematic analysis, informed by the candidacy framework. Results The following four observations emerged from the interviews: (a) most patients were diagnosed with CHD at birth if they were born at a health care facility, but for those born at home, CHD was discovered much later (b) many patients experienced misdiagnoses before seeking care at a large hospital, (c) after diagnosis, patients were waiting for the surgery for more than a year, (d) caregivers felt anxious and optimistic once they were able to schedule the surgical date. During the care-seeking journey, caregivers encountered financial constraints, struggled in a fragmented delivery system, and experienced poor service quality. Conclusions Delayed access to care was largely due to the lack of early CHD recognition and financial hardships, related to the inefficient and disorganized health care system. Fee waivers were available to assist low-income children in gaining access to health services or medications, but application information was not readily available. Indirect costs like long-distance travel contributed to this challenge. Overall, improvements must be made for district-level screening and the health care workforce.

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