scholarly journals Tetralogy of Fallot with Scimitar syndrome in VACTERL: a very rare association

2017 ◽  
Vol 5 (1) ◽  
pp. 269
Author(s):  
Puneet Anand
Keyword(s):  
Congenital Anomaly ◽  
Tetralogy Of Fallot ◽  
Management Strategy ◽  
Septal Defect ◽  
Early Embryogenesis ◽  
Scimitar Syndrome ◽  
Cardiac Malformation ◽  
Rare Congenital Anomaly ◽  
Rare Association ◽  
Congenital Cardiac Malformation

The most common congenital cardiac malformation that occurs in VACTERL association is ventricular septal defect. Scimitar syndrome is a very rare congenital anomaly of the heart occurring due to maldevelopment of the lung bud or the pulmonary vascularization during early embryogenesis. The occurrence of Scimitar syndrome in VACTERL and its association with another cardiac congenital anomaly such as Tetralogy of Fallot (TOF) is extremely rare. The association of Tetralogy of Fallot with Scimitar syndrome alters the management strategy. Hereby, a very rare combination of Scimitar syndrome and tetralogy of Fallot in a four-year-old boy with VACTERL is presented, who underwent a successful single-stage surgical correction.

The current surgical perspective to repair of atrioventricular septal defect with common atrioventricular junction

2010 ◽  
Vol 20 (S3) ◽  
pp. 120-127 ◽  
Author(s):  
Tom R. Karl ◽  
Sylvio C. J. Provenzano ◽  
Graham R. Nunn ◽  
Robert H. Anderson
Keyword(s):  
Tetralogy Of Fallot ◽  
Septal Defect ◽  
Surgical Repair ◽  
Atrioventricular Septal Defect ◽  
Indications For Surgery ◽  
Cardiac Malformation ◽  
Technical Aspects ◽  
Atrioventricular Junction ◽  
Congenital Cardiac Malformation ◽  
Surgery Timing

AbstractAtrioventricular septal defect with common atrioventricular junction is a relatively common congenital cardiac malformation. It sometimes presents challenging surgical problems, especially when seen in combination with tetralogy of Fallot. We describe herein our current concepts regarding surgical repair of atrioventricular septal defect with and without tetralogy, discussing indications for surgery, timing, technical aspects, and outcomes.

scholarly journals Double-Outlet Right Ventricle in a Chianina Calf

Animals ◽  
2021 ◽  
Vol 11 (2) ◽  
pp. 318
Author(s):  
Domenico Caivano ◽  
Maria Chiara Marchesi ◽  
Piero Boni ◽  
Noemi Venanzi ◽  
Giovanni Angeli ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Septal Defect ◽  
Heart Defects ◽  
Double Outlet Right Ventricle ◽  
Cardiac Malformation ◽  
Septal Defects ◽  
Congenital Cardiac Malformation ◽  
The Right ◽  
Similar Complex ◽  
Echocardiographic Findings

Congenital heart defects have been occasionally reported in cattle and ventricular septal defect represents the most frequently encountered anomaly. The double-outlet right ventricle is a rare congenital ventriculoarterial malformation reported only in certain cattle breeds. We describe this rare and complex congenital cardiac malformation observed in a 10-day-old male Chianina calf. Clinical examination showed tachycardia, tachypnea, jugular pulses, cyanotic mucous membranes and a right apical systolic murmur. Transthoracic echocardiography revealed severe dilation of the right-sided cardiac chambers with a markedly hypoplastic left ventricle. Both aorta and pulmonary artery leaving the right ventricle in parallel alignment with the tricuspid valve were suggestive of a dual-outlet right ventricle. Interventricular and interatrial septal defects were also visualized. Post-mortem examination confirmed the echocardiographic findings. To the authors’ knowledge, a similar complex congenital cardiac malformation has not been reported in calves of the Chianina breed to date.

Rare association of Klippel-Feil syndrome with situs inversus totalis and review of the genetic background

2021 ◽  
Vol 14 (5) ◽  
pp. e241906
Author(s):  
Husain Abdulameer Abdali ◽  
Joseph Rivendra Duddu ◽  
Mohamed Jawad Mubarak ◽  
Almughirah Salahaldin Mohamed
Keyword(s):  
Congenital Anomaly ◽  
Causative Agent ◽  
Situs Inversus ◽  
Congenital Anomalies ◽  
Genetic Background ◽  
Cervical Vertebrae ◽  
Situs Inversus Totalis ◽  
Rare Congenital Anomaly ◽  
Rare Association ◽  
High Possibility

Klippel-Feil syndrome (KFS) is a rare congenital anomaly in forming the cervical vertebrae resulting in the fusion of two or more of the vertebrae. KFS is associated with many congenital anomalies, some of which are common and well known. Here, we report a child with an extremely rare association of KFS with situs inversus totalis (SIT). Both KFS and SIT are genetically heterogeneous and their co-occurrence suggests a high possibility of sharing the same underlying causative agent. Here, we review the genetic background that is known for these two conditions in the literature.

scholarly journals Poland Syndrome- A Rare Congenital Condition

2018 ◽  
Vol 36 (4) ◽  
pp. 166-169
Author(s):  
Homayra Tahseen Hossain ◽  
Md Abul Kashem Khandaker ◽  
Quazi Tarikul Islam ◽  
HAM Nazmul Ahasan ◽  
Munshi MB Md Shoaib Adnan ◽  
...  
Keyword(s):  
Congenital Anomaly ◽  
Mitral Valve ◽  
Chest Wall ◽  
Male Patient ◽  
Mitral Valve Prolapse ◽  
Poland Syndrome ◽  
Rare Congenital Anomaly ◽  
Rare Association ◽  
Vascular Disruption ◽  
Congenital Condition

Named after Sir Alfred Poland, Poland syndrome is a rare congenital anomaly classically characterized by the absence of unilateral chest wall muscles and sometimes ipsilateral symbrachydactyly (abnormally short and webbed fingers). The aetiology is probably a vascular disruption sequence of the subclavian arteries. In most cases, Poland Syndrome is sporadic. We report a 26 year old male patient with typical features of Poland Syndrome associated with bronchiectasis and mitral valve prolapse- a very rare association. To the best of our knowledge, this is the first documented case of a Poland Syndrome with rare association reported from Bangladesh.J Bangladesh Coll Phys Surg 2018; 36(4): 166-169

scholarly journals Partial anomalous pulmonary venous connection detected during right pneumonectomy

2019 ◽  
Vol 30 (3) ◽  
pp. 497-498
Author(s):  
Bülent Mustafa Yenigün ◽  
Gökhan Kocaman ◽  
Ayşegül Gürsoy Çoruh ◽  
Rıfat Murat Akal
Keyword(s):  
Lung Cancer ◽  
Congenital Anomaly ◽  
Case Report ◽  
Surgical Treatment ◽  
Septal Defect ◽  
Lung Resection ◽  
Rare Congenital Anomaly ◽  
The Right ◽  
Anomalous Pulmonary Venous Connection ◽  
Lung Resections

Abstract Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital anomaly. Generally, it is seen on the right side and is associated with an atrial septal defect. Herein, we present a case of a 50-year-old male patient with a supracardiac type PAPVC detected during pneumonectomy for a right hilar mass. This is the second case report in the literature presenting surgical treatment of both lung cancer and PAPVC using pneumonectomy. Thoracic surgeons should be aware of this anomaly when they are planning to perform a major lung resection. If PAPVC and lung cancer are in the same lobe, anatomical lung resections including pneumonectomy can be safely performed.

scholarly journals Fortuitious Diagnosis of Scimitar Syndrome in an Asymptomatic Adult

2021 ◽  
Vol 4 (14) ◽  
pp. 01-14
Author(s):  
Shibba Takkar Chhabra ◽  
Tripat Kaur ◽  
Gurleen Kaur ◽  
Rohit Tandon ◽  
Vandana Midha
Keyword(s):  
Congenital Anomaly ◽  
Clinical Symptoms ◽  
Scimitar Syndrome ◽  
Rare Congenital Anomaly ◽  
Diagnosis And Management ◽  
Asymptomatic Adult ◽  
Prompt Diagnosis

Scimitar syndrome is a rare congenital anomaly seen as one of the components of congenital pulmonary venolobar syndrome. Simple and traditional investigation tools like CXR if interpreted with vigil can aid in prompt diagnosis and management of patients even in absence of clinical symptoms.

scholarly journals Tetralogy of Fallot with Double Aortic Arch in an 8-Month-Old Girl: A Rare Association

2020 ◽  
Vol 23 (4) ◽  
pp. E507-E509
Author(s):  
Huang Feng ◽  
Xiaohui Bian ◽  
Yuru Lan ◽  
Yunfei Ling ◽  
Yong jun Qian
Keyword(s):  
Aortic Arch ◽  
Tetralogy Of Fallot ◽  
Rare Case ◽  
Congenital Heart ◽  
Heart Diseases ◽  
Relative Size ◽  
Double Aortic Arch ◽  
Congenital Heart Diseases ◽  
Rare Congenital Anomaly ◽  
Rare Association

Double aortic arch (DAA) is an extremely rare congenital anomaly that can be divided into right dominant, left dominant, and balanced DAA according to the relative size of the two arches. The incidence of balanced DAA is only 5% among double arch anomalies. DAA is symptomatic only when it produces symptoms secondary to compression of the trachea or esophagus. DAA is rarely associated with other congenital heart diseases. In this report, we present a rare case of asymptomatic DAA combined with Tetralogy of Fallot (TOF) in an 8-month-old girl.

scholarly journals Ebstein’s anomaly with constricitve pericarditis

2016 ◽  
Vol 31 (1) ◽  
pp. 46-49
Author(s):  
Apurba Thakur ◽  
Redoy Ranjan ◽  
Mohammad Samir Azam Sunny ◽  
Md Aftabuddin ◽  
Asit Baran Adhikary
Keyword(s):  
Tricuspid Valve ◽  
Life Science ◽  
Septal Defect ◽  
Symptomatic Improvement ◽  
Cardiac Malformation ◽  
Pericardial Disease ◽  
Direct Closure ◽  
Porcine Tissue ◽  
Congenital Cardiac Malformation

Ebstein’s anomaly is a rare congenital cardiac malformation that affects the tricuspid valve commonly, but its association with pericardial disease is even rarer. We report a case of 25 year old man presented with dyspnoea on exertion and fatigability. A diagnosis of Ebstein’s anomaly with atrial septal defect (ASD) with constrictive pericarditis was confirmed using transthoracic echocardiography. Peroperatively pericardiotomy was done after meticulous dissection of pericardium. Under cardiopulmonary bypass tricuspid valve was replaced with 29 mm Edward life science porcine tissue heart valve with direct closure of ASD. Patient showed excellent symptomatic improvement and was discharged on 7th POD with advice and after 3 months of follow up patient was doing well post operartive day.Bangladesh Heart Journal 2016; 31(1) : 46-49

scholarly journals Late diagnosis of total anomalous connection of pulmonary veins during the puerperium

2019 ◽  
Vol 2 (2) ◽  
pp. 4-8
Author(s):  
Susan Pumacayo-Cárdenas
Keyword(s):  
Congenital Anomaly ◽  
Cardiac Magnetic Resonance ◽  
Magnetic Resonance ◽  
Physical Examination ◽  
Septal Defect ◽  
Pulmonary Veins ◽  
Heart Murmur ◽  
Rare Congenital Anomaly ◽  
Hands And Feet ◽  
Anomalous Pulmonary Venous Connection

The total anomalous pulmonary venous connection (TAPVC) is a rare congenital anomaly. We present a case of a 36-year-old woman, in the postpartum period during the physical examination there was a heart murmur and beating in hands and feet. Cardiac magnetic resonance reported supracardiac TAPVC and large atrial septal defect. The survival of these patients is very uncommon in adults and even worse during pregnancy.

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