scholarly journals Ellis-Van-Creveld Syndrome and Congenital Cardiac Anomaly: Common Atrium with Atrioventricular Canal Septal Defect

2021 ◽  
Vol 11 (12) ◽  
pp. 133-140
Author(s):  
Srikrishna Sirivella
2015 ◽  
Vol 6 (1) ◽  
pp. 40-42
Author(s):  
Ravi Nirmalkumar Rajdeo ◽  
Kishor Taori ◽  
Jawahar Rathod ◽  
Nekee Navin Sejpal

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.


2021 ◽  
Vol 3 (4) ◽  
pp. 1-6
Author(s):  
Zul Khairul Azwadi Ismail ◽  
Khairil Amir Sayuti ◽  
Mohd Shafie Abdullah

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.


Author(s):  
Changqing Gao ◽  
Ming Yang ◽  
Cangsong Xiao ◽  
Huajun Zhang ◽  
Gang Wang

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.


2021 ◽  
Vol 5 (5) ◽  
Author(s):  
Junko Okamoto ◽  
Shinji Fukuhara ◽  
Hideki Ozawa ◽  
Takahiro Katsumata

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.


2019 ◽  
Vol 6 (1) ◽  
pp. 37-40
Author(s):  
Pradeep Raj Regmi ◽  
Isha Amatya ◽  
Prajawal Dhakal ◽  
Ranjit Kumar Chaudhary ◽  
Prakash Kayastha ◽  
...  

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.  


Author(s):  
Amit Kumar Verma ◽  
Anil Rawat ◽  
Siddharth Mishra ◽  
Nitin Arun Dikshit

Truncus arteriosus (TA) is a rare congenital cardiac anomaly caused by failure of normal conotruncal septation during the fetal development. This aberration leads to a common ventricular outflow artery over the malaligned large ventricular septal defect (VSD), supplying systemic, coronary and pulmonary circulation. People with such anomalous anatomy show variable presentation from early childhood to adult life depending on the severity of defects. We here present three cases of truncus arteriosus with aortic interruption / hypoplasia-coarctation (type A4 truncus arteriosus) with focus on relevant dual source MDCTA findings.


Author(s):  
Carina Nina Vorisek ◽  
Christian Enzensberger ◽  
Steven Willomeit ◽  
Andrii Kurkevych ◽  
Rüdiger Stessig ◽  
...  

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.


2020 ◽  
Vol 41 (12) ◽  
pp. 2087-2093
Author(s):  
Francesca Piceci‐Sparascio ◽  
Adrian Palencia‐Campos ◽  
Patricia Soto‐Bielicka ◽  
Angela D'Anzi ◽  
Valentina Guida ◽  
...  

2021 ◽  
Vol 14 (9) ◽  
pp. e245186
Author(s):  
Sho Takagi ◽  
Akio Nakasu ◽  
Junji Yanagisawa ◽  
Yoshihiro Goto

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital cardiac anomaly. There are a few reports of untreated TAPVC diagnosed in patients older than 60 years. Herein, we report the successful surgical treatment of TAPVC in a 70-year-old woman. A 70-year-old woman with TAPVC presented with symptoms of acute heart failure. We closed an atrial septal defect and performed tricuspid annuloplasty and commissurotomy of the pulmonary valve. Postoperative CT showed no residual shunt, and the pulmonary veins drained into the left atrium. She had an uneventful postoperative course. This report describes the case of the oldest known patient who underwent surgical treatment for TAPVC. Surviving into adulthood with little or no symptoms is uncommon in patients with TAPVC, and cases of late-onset TAPVC, such as our case, are rare. Nevertheless, close vigilance is necessary to prevent misdiagnosis in patients with this clinical presentation.


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