Prenatal Diagnosis and Outcome of Congenital Corrected Transposition of the Great Arteries – A Multicenter Report of 69 Cases

2020 ◽  
Author(s):  
Carina Nina Vorisek ◽  
Christian Enzensberger ◽  
Steven Willomeit ◽  
Andrii Kurkevych ◽  
Rüdiger Stessig ◽  
...  
Keyword(s):  
Septal Defect ◽  
Fetal Echocardiography ◽  
Cardiac Anomaly ◽  
Chromosomal Anomalies ◽  
Term Outcome ◽  
Congenital Cardiac Anomaly ◽  
Intention To Treat ◽  
The Mean ◽  
International Multicenter ◽  
Corrected Transposition

Abstract Objective Congenital corrected transposition of the great arteries (ccTGA) is a rare congenital cardiac anomaly which remains difficult to diagnose prenatally. We aim to investigate the natural history, associated anomalies and the outcome of patients in prenatally diagnosed ccTGA. Method This was an international multicenter retrospective analysis of fetuses with a diagnosis of ccTGA from 2002 to 2017. We reviewed clinical and echocardiographic databases of seven centers. Anatomic survey and fetal echocardiography were performed according to international guidelines of ISUOG. Results We considered 69 fetuses with prenatally suspected ccTGA. There was an overall survival rate of 91 % among 54 patients with a confirmed diagnosis. Survival to live birth was 96 % (52/54) and survival on an intention-to-treat basis was 94 % (49/52). The mean gestational age at the time of diagnosis was 25.6 ± 5.9 weeks of gestation. In 7 out of 54 fetuses (13 %), ccTGA was an isolated finding. Dextro/mesocardia was present in 15 cases (27.8 %). Intracardiac anomalies were present in 46/54 cases (85.2 %) with the most frequent anomaly being a ventricular septal defect present in 41 fetuses (75.9 %). Complete heart block was diagnosed in 10 cases (18.5 %). Extracardiac anomalies were observed in 9 out of 54 cases (16.7 %). Prenatal karyotyping of the fetus was available in 30/54 (55.6 %) cases with chromosomal anomalies in 4/30 (13.3 %). Conclusion ccTGA is a rare cardiac anomaly often accompanied by a variable spectrum of further intracardiac abnormalities. Accurate diagnosis of ccTGA, which can be integrated into parental counselling, is feasible with a favorable short-term outcome for affected neonates.

scholarly journals Surgical Repair of VSD and their Short Term Outcome in a Tertiary Care Hospital

2019 ◽  
pp. 34-37
Author(s):  
Kazi Zahidul Hoque ◽  
Masumul Gani Chowdhury ◽  
Ashraful Islam ◽  
Makbul Hossain ◽  
Mostafizur Rahman ◽  
...  
Keyword(s):  
Cardiac Surgery ◽  
Tertiary Care ◽  
Patch Repair ◽  
Cardiac Anomaly ◽  
Care Hospital ◽  
Short Term ◽  
Term Outcome ◽  
Congenital Cardiac Anomaly ◽  
Short Term Outcome ◽  
The Mean

Background: Ventricular Septal Defect (VSD) is the commonest congenital cardiac anomaly in Bangladesh. For this reason, patch repair of VSD is commonly performed cardiac surgery here. Aim: To observe the short term outcome of patch repair of VSD. Methods: 50 consecutive patients were enrolled in the study by purposive sampling who received treatment for isolated VSD in Department of Paediatric Cardiac Surgery of Dhaka Shishu Hospital, Dhaka, Bangladesh. They underwent ventricular patch repair from January, 2017 to December, 2017. A prospective observational cross-sectional study was conducted for this 12 months period. A pre-structured, interview and observation based, peer reviewed data collection sheet was prepared. Data regarding Sociodemographic, clinical, surgical and outcome profile were recorded. Data were compiled, edited and analyzed with SPSS version 23. Data were presented as mean and standard deviation, frequency percentage and median with range. Results: The mean birth weight of 50 patients was 2.8 kg (range: 2.1-3.5 kg) whereas the median of gestational age was 38 weeks. Among these 50 patients, 22 (44%) and 28 (56%) were boys and girls respectively. The indication of surgery was volume load, failure to thrive and repeated respiratory tract infection. Out of 50 patients, 46 (92%) had perimembranous variety. On the contrary, 4 (8%) experienced Double committed type of VSD. The mean Bypass time and Aortic cross-clamp time were recorded as 70 ± 13.7 minutes and 35 ± 6.85 minutes respectively. Out of 50 patients who underwent ventricular patch repair, 2 (4%) experienced pneumothorax, 1 (2%) got chylothorax and 1 (2%) had transient heart block. Total 45 (90%) patients showed uneventful outcome. Conclusion: Contemporary results of ventricular patch repair in case of VSD showed unparallel outcome with extremely low morbidity in our perspective.

Fatal disseminated paradoxical embolism in inferior sinus venosus atrial septal defect

2021 ◽  
Vol 3 (4) ◽  
pp. 1-6
Author(s):  
Zul Khairul Azwadi Ismail ◽  
Khairil Amir Sayuti ◽  
Mohd Shafie Abdullah
Keyword(s):  
Septal Defect ◽  
Incidental Finding ◽  
Transoesophageal Echocardiography ◽  
Systemic Circulation ◽  
Paradoxical Embolism ◽  
Sinus Venosus ◽  
Cardiac Anomaly ◽  
Limb Ischaemia ◽  
Congenital Cardiac Anomaly ◽  
Acute Limb Ischaemia

Inferior sinus venosus arterial septal defect (ASD) is a rare congenital cardiac anomaly. As with other types of ASDs and patent foramen ovale (PFO), this defect results in arteri-venous shunting with the risk of developing paradoxical embolism (PDE) to the systemic circulation from venous emboli. We report  a case of a 67-year-old lady presented to emergency department (ED) with massive pulmonary embolism (PE) and recurrent acute limb ischaemia. On CT pulmonary angiography (CTPA), an incidental finding of inferior sinus venosus ASD was discovered that has caused the PDE. Thrombolysis was administered followed by open mechanical thrombectomy. The patient developed massive lower gastrointestinal bleed post thrombolysis then passed away despite embolization. This case report describes the catastrophic effect of  PDE to cause disseminated multisystem thromboembolism. It also emphasizes the importance of early detection of a possible right-to-left shunt in patients presented with recurrent acute limb ischaemia. Transthoracic echocardiography has reduced sensitivity for inferior sinus venosus ASD requiring further assessment with transoesophageal echocardiography in selected cases.

Accuracy of Fetal Echocardiography in the Differential Diagnosis between Truncus Arteriosus and Pulmonary Atresia with Ventricular Septal Defect

2015 ◽  
Vol 39 (2) ◽  
pp. 90-99 ◽  
Author(s):  
Olga Gómez ◽  
Iris Soveral ◽  
Mar Bennasar ◽  
Fatima Crispi ◽  
Narcis Masoller ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Fetal Echocardiography ◽  
Ductus Arteriosus ◽  
Pulmonary Atresia ◽  
Image Correlation ◽  
Truncus Arteriosus ◽  
Chromosomal Anomalies ◽  
Collateral Arteries ◽  
Arterial Trunk

Objectives: To report on the accuracy of fetal echocardiography in the distinction between truncus arteriosus communis (CAT) and pulmonary atresia with ventricular septal defect (PA-VSD) and to describe the association with extracardiac and chromosomal anomalies. Methods: This was a retrospective study on 31 fetuses with a single arterial trunk overriding a VSD with a nonidentifiable right ventricle outflow tract with anterograde flow. Data on the type of cardiac defect, gestational age, characteristics of the arterial trunk valve, presence of additional vascular, chromosomal and extracardiac abnormalities and postnatal outcome were obtained. Misdiagnosed cases were reevaluated by four-dimensional spatiotemporal image correlation (4D-STIC) echocardiography. Results: The overall diagnostic accuracy was 81% and increased to 93.5% with 4D-STIC. Chromosomal and extracardiac anomalies were detected in 40 and 27%, respectively. In the PA-VSD group, patent ductus arteriosus and major aortopulmonary collateral arteries (MAPCAs) were present in 70 and 50% of the cases, respectively, coexisting in 1 of 5 cases. MAPCAs were significantly associated with a right aortic arch and with a 22q11 microdeletion in 50% of cases. Conclusions: A prenatal distinction between CAT and PA-VSD can currently be achieved in most cases. MAPCAs should be actively searched for when PA-VSD is suspected, as they are associated with a higher risk of 22q11 microdeletion and potentially complicate postnatal treatment.

Totally Endoscopic Robotic Correction of Cor Triatriatum Sinister Coexisting with Atrial Septal Defect

2016 ◽  
Vol 11 (6) ◽  
pp. 451-452 ◽  
Author(s):  
Changqing Gao ◽  
Ming Yang ◽  
Cangsong Xiao ◽  
Huajun Zhang ◽  
Gang Wang
Keyword(s):  
Surgical Technique ◽  
Atrial Septal Defect ◽  
Septal Defect ◽  
Cor Triatriatum ◽  
Cardiac Anomaly ◽  
Safe Alternative ◽  
Congenital Cardiac Anomaly ◽  
Cor Triatriatum Sinister ◽  
Da Vinci Si ◽  
Surgical System

Cor triatriatum sinister (CTS) is a rare congenital cardiac anomaly and is mainly corrected through conventional surgery through sternotomy. We described our successful novel surgical technique of totally robotic correction of CTS in one case of CTS with concomitant repair of atrial septal defect using da Vinci SI Surgical System (Intuitive Surgical, Inc, Sunnyvale, CA USA) with excellent surgical outcome. We conclude that robotic correction of CTS is a feasible and safe alternative to conventional surgical technique.

scholarly journals A case report of an elderly male with isolated coronary sinus atrial septal defect

2021 ◽  
Vol 5 (5) ◽  
Author(s):  
Junko Okamoto ◽  
Shinji Fukuhara ◽  
Hideki Ozawa ◽  
Takahiro Katsumata
Keyword(s):  
Elderly Patients ◽  
Atrial Septal Defect ◽  
Coronary Sinus ◽  
Septal Defect ◽  
Superior Vena ◽  
Vena Cava ◽  
Cardiac Anomaly ◽  
Elderly Male ◽  
Direct Closure ◽  
Congenital Cardiac Anomaly

Abstract Background Isolated coronary sinus atrial septal defect (CSASD) is a rare congenital cardiac anomaly, comprising <1% of atrial septal defects. Elderly patients with this anomaly are even more uncommon and sometimes overlooked. Case summary A 73-year-old man with a history of electrical defibrillation therapy for atrial flutter presented with worsening exertional dyspnoea. Cardiac examination revealed CSASD without persistent left superior vena cava, showing only moderate tricuspid regurgitation. Surgical repair of the defect and regurgitant valve improved symptoms dramatically. Discussion Elderly patients with atrial arrhythmias might show uncommon presentations of congenital heart disease. Cardiologists should pay attention to enlarged right ventricle, pulmonary artery, and, in particular, enlarged coronary sinus. Direct closure with interrupted sutures secured with pledgets is effective in some case of terminal type of CSASD.

scholarly journals Transposition of Great Arteries

2021 ◽  
Author(s):  
Rita Prasad Verma
Keyword(s):  
Fetal Echocardiography ◽  
Arterial Switch Operation ◽  
Cardiac Anomaly ◽  
First Year ◽  
Term Outcome ◽  
Hemodynamic Pattern ◽  
Switch Operation ◽  
The Right ◽  
First Year Of Life

TGA is the commonest complex congenital cyanotic cardiac anomaly occurring during the first week of life. It is characterized by the unusual anomaly of ventriculoarterial discordance, with the aorta (A) originating from the right ventricle (RV) and the pulmonary artery (PA) from the left ventricle (LV). In the common Dextro form (DTGA), A is abnormally located to the right, anterior, and inferior of PA. The anatomic configuration results in the lethal hemodynamic pattern of 2 independent and parallel running circulatory circuits, which mandates creating a conduit to ensure the mixing of oxygenated and deoxygenated blood for survival. In the rare Levo form (LTGA), the aorta is placed anterior and to the left of PA with ventricular inversion. TGA is well tolerated in the fetus and is challenging to diagnose by fetal echocardiography unless the outflow tracts are specifically visualized. Postnatally the typical findings of murmur and cyanosis vary according to the associated cardiac defects and the degree of intercirculatory mixing. The arterial switch operation (ASO), which involves establishing ventriculoarterial concordance, is the standard surgical repair of D-TGA and has replaced the atrial switch procedures due to its superior long-term outcomes. The Rastelli procedure is used for complex DTGA cases. DTGA has a 90% mortality rate in the first year of life if untreated, while over 95% survive for 5 to 25 years after surgery. Post-surgical course may be complicated and require surgical revisions. The long-term outcome is associated with normal or mild to moderate neurodevelopmental disabilities, depending upon the type, complexity, and course of the disease. Expert follow-up of the patients into adulthood is an integral part of the management of TGA for best outcomes.

scholarly journals Computed tomography diagnosis of truncus arteriosus type IV: a case report

2019 ◽  
Vol 6 (1) ◽  
pp. 37-40
Author(s):  
Pradeep Raj Regmi ◽  
Isha Amatya ◽  
Prajawal Dhakal ◽  
Ranjit Kumar Chaudhary ◽  
Prakash Kayastha ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Mirror Image ◽  
Truncus Arteriosus ◽  
Cardiac Anomaly ◽  
Descending Thoracic Aorta ◽  
Congenital Cardiac Anomaly ◽  
Type Iv ◽  
Ct Finding

Truncus arteriosus (TA) is an uncommon congenital cardiac anomaly of which type IV is considered a rare variant. Recognition is crucial for proper treatment planning. The prognosis without treatment is poor. Echocardiography alone may not be useful in evaluation. Computed tomography (CT) finding is complicated. We report an 18 months child with ventricular septal defect (VSD), diagnosed on echocardiography, and further review by CT showed VSD with descending thoracic aorta giving rise to the pulmonary arteries suggestive of pseudo truncus (Collet and Edwards Truncus arteriosus Type IV) and right sided aortic arch with mirror image branching.  

scholarly journals Cor Triloculare Biventriculare with Ventricular Septal Defect: Prenatal Diagnosis

2015 ◽  
Vol 6 (1) ◽  
pp. 40-42
Author(s):  
Ravi Nirmalkumar Rajdeo ◽  
Kishor Taori ◽  
Jawahar Rathod ◽  
Nekee Navin Sejpal
Keyword(s):  
Prenatal Diagnosis ◽  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Congenital Abnormalities ◽  
Superior Vena ◽  
Vena Cava ◽  
Interatrial Septum ◽  
Cardiac Anomaly ◽  
Congenital Cardiac Anomaly ◽  
Ellis Van Creveld Syndrome

ABSTRACT Cor triloculare biventriculare is a very rare congenital cardiac anomaly in which there is complete absence of interatrial septum resulting in three-chambered heart. It can be associated with various other congenital abnormalities like ventricular septal defect, polysplenia, Ellis-van creveld syndrome or persistent left-sided superior vena cava. Here we report a case of antenatal diagnosis common atrium and ventricular septal defect in a fetus of average gestational age of 29 weeks in a primigravida. How to cite this article Rajdeo RN, Taori K, Rathod J, Sejpal NN. Cor Triloculare Biventriculare with Ventricular Septal Defect: Prenatal Diagnosis. Int J Infertil Fetal Med 2015;6(1):40-42.

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