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Heart Views : the Official Journal of... Oct 2011Congenitally corrected transposition of great arteries is a rare congenital anomaly. This case report describes a 30-year-old patient of congenitally corrected...
Congenitally corrected transposition of great arteries is a rare congenital anomaly. This case report describes a 30-year-old patient of congenitally corrected transposition of the great arteries with rheumatic involvement of systemic (tricuspid) atrio-ventricular valve.
PubMed: 22574246
DOI: 10.4103/1995-705X.90908 -
Journal of Radiology Case Reports Feb 2015When approaching a case with a situs abnormality, using the proper terminology, making a specific diagnosis, and understanding the other often associated abnormalities...
When approaching a case with a situs abnormality, using the proper terminology, making a specific diagnosis, and understanding the other often associated abnormalities that need to be excluded are of great importance. We present a case of situs ambiguous in the presence of intestinal nonrotation and an obstructing duodenal web. Our patient initially presented at two days old with bilious emesis and failure to pass meconium after birth. An abdominal radiograph demonstrated an unusual bowel gas pattern, a reversed "double bubble" sign. A subsequent thorough imaging survey was crucial to further characterize our patient's unique anatomy. Overall, our case demonstrates many of the unusual plain radiographic and sonographic findings associated with our patient's situs abnormality and allows for review of situs abnormalities and their significance.
Topics: Abnormalities, Multiple; Diagnosis, Differential; Duodenum; Female; Humans; Infant, Newborn; Intestines; Levocardia; Radiography; Stomach; Ultrasonography
PubMed: 25926924
DOI: 10.3941/jrcr.v9i2.2358 -
Singapore Medical Journal Apr 2015Levocardia (left-sided cardiac apex) with abdominal situs inversus is extremely rare. This is also known as isolated levocardia and is almost always associated with...
Levocardia (left-sided cardiac apex) with abdominal situs inversus is extremely rare. This is also known as isolated levocardia and is almost always associated with severe forms of congenital heart defects with poor prognosis. We report isolated levocardia in a 13-year-old symptomatic male patient. The purpose of this paper is to outline the imaging features of isolated levocardia and to highlight the role of cardiovascular magnetic resonance imaging (CMR) in the diagnosis and management of such cases. Other forms of cardiac malposition, including dextrocardia, mesocardia and criss-cross heart, with chest radiograph and CMR correlation, are also discussed.
Topics: Abnormalities, Multiple; Adolescent; Diagnostic Imaging; Humans; Levocardia; Magnetic Resonance Imaging, Cine; Male; Radiography, Thoracic; Situs Inversus; Tomography, X-Ray Computed
PubMed: 25917470
DOI: 10.11622/smedj.2015059 -
BMC Pregnancy and Childbirth Jul 2022Monochorionic dizygotic twins are a rare condition, mostly related to assisted reproductive technology. This type of twinning is burdened by the same risk of pregnancy...
BACKGROUND
Monochorionic dizygotic twins are a rare condition, mostly related to assisted reproductive technology. This type of twinning is burdened by the same risk of pregnancy complications found in monochorionic monozygotic pregnancies.
CASE PRESENTATION
We report a case of spontaneous monochorionic dizygotic twins sharing situs inversus abdominalis and isolated levocardia, with only one twin affected by biliary atresia with splenic malformation syndrome. We also conducted a literature review of the 14 available documented monochorionic dizygotic twin gestations spontaneously conceived.
CONCLUSIONS
It is still unclear how this unusual type of twinning can occur in spontaneous conception. The evidence so far suggest the importance to timely diagnose the chorionicity, in order to adequately manage the typical complications associated with monochorionicity.
Topics: Chorion; Female; Humans; Pregnancy; Pregnancy Complications; Pregnancy, Twin; Reproductive Techniques, Assisted; Twins, Dizygotic; Twins, Monozygotic
PubMed: 35836143
DOI: 10.1186/s12884-022-04866-x -
Electronic Physician Jan 2017Situs inversus with levocardia and congenitally corrected transposition of the great arteries represents a relatively very rare congenital condition and most patients...
Situs inversus with levocardia and congenitally corrected transposition of the great arteries represents a relatively very rare congenital condition and most patients are diagnosed in infancy or early age. This case report describes a 35-year old man with congenitally corrected transposition of the great arteries which presented with a five month history of exertional dyspnea. A diagnosis was confirmed by transesophageal echocardiogram, showing situs inversus, levocardia, atrioventricular and ventriculoarterial discordance. He underwent physiologic repair, and was discharged thirty five days after the operation, in a good general condition. Although management of the corrected transposition of the great arteries patients remains controversial, the recommendation is that physiologic repair may be the procedure of choice for some patients, particularly complicated cases.
PubMed: 28243408
DOI: 10.19082/3570 -
Texas Heart Institute Journal Dec 2017Aneurysm of the sinus of Valsalva, a rare cardiac condition, results from dilation of an aortic sinus. Sudden aneurysm rupture can trigger rapidly progressive heart...
Aneurysm of the sinus of Valsalva, a rare cardiac condition, results from dilation of an aortic sinus. Sudden aneurysm rupture can trigger rapidly progressive heart failure. We discuss the case of a 57-year-old woman with situs ambiguus, isolated levocardia, and polysplenia who presented with acute-onset heart failure. Transesophageal echocardiograms revealed an aneurysm of the right coronary sinus of Valsalva that had ruptured into the right atrial cavity. The patient underwent successful surgical repair. To our knowledge, this is the first report of a sinus of Valsalva aneurysm in a patient with this combination of congenital abnormalities. We briefly review the association between congenital heart disease, situs ambiguus, and ciliary dysfunction.
Topics: Abnormalities, Multiple; Aortic Rupture; Echocardiography, Transesophageal; Female; Heterotaxy Syndrome; Humans; Levocardia; Middle Aged; Sinus of Valsalva; Spleen; Splenic Diseases; Tomography, X-Ray Computed
PubMed: 29276443
DOI: 10.14503/THIJ-16-6106 -
Cureus Jan 2021An unroofed coronary sinus is a rare congenital anomaly in the roof of the coronary sinus causing a communication between the coronary sinus and the left atrium leading...
An unroofed coronary sinus is a rare congenital anomaly in the roof of the coronary sinus causing a communication between the coronary sinus and the left atrium leading to a left to right shunt. It is often associated with a persistent left superior vena cava and other complex congenital lesions like anomalous pulmonary venous return and heterotaxy. Since it is a deep-seated defect, it is seldom diagnosed by transthoracic two-dimensional (2D) echocardiography and requires multimodal imaging for a diagnosis. Here, we present the case of a 27-year-old male in whom the defect was very apparent on standard 2D transthoracic echocardiography. Transthoracic 2D echocardiography revealed situs solitus, levocardia, and a dilated coronary sinus with unroofing which was most prominent in the standard parasternal long-axis view and the foreshortened apical four-chamber view. A color Doppler demonstrated a flow from the left atrium into the dilated coronary sinus. The right ventricle and atrium were dilated with mild pulmonary arterial hypertension. There was no right ventricular dysfunction. Examination with modified suprasternal views showed a left superior vena cava. All four pulmonary veins drained into the left atrium. Other chambers of the heart and great vessels were structurally normal without coarctation or patent ductus arteriosus. The interventricular septum was intact and atrioventricular and ventriculoatrial concordance was preserved. Detection of a dilated coronary sinus by transthoracic 2D echocardiography must be followed by multimodal imaging techniques like cardiac computed tomography and transesophageal echocardiography to detect and manage associated defects.
PubMed: 33680587
DOI: 10.7759/cureus.13041 -
BMC Pregnancy and Childbirth Mar 2013Fetal unilateral lung agenesis, complicated with cardiac shifting, is a rare anomaly, the diagnosis of which remains a challenge for many sonographers in routine...
BACKGROUND
Fetal unilateral lung agenesis, complicated with cardiac shifting, is a rare anomaly, the diagnosis of which remains a challenge for many sonographers in routine screening programs. The present study describes a systematic approach for the diagnosis of fetal unilateral lung agenesis and cardiac malpositions in routine prenatal screening.
METHODS
A total of 18 cases of fetal unilateral lung agenesis complicated with cardiac malposition were reviewed. A systematic method was proposed to identify the fetal left side and right side according to the fetal head position and posture by acquiring a long axis and transverse view of the fetus. Fetal unilateral lung agenesis was diagnosed by evaluation of the ipsilateral pulmonary artery. The diagnosis was confirmed by postnatal echocardiography, digital radiology, and computed tomography after birth or by autopsy findings.
RESULTS
The left-sided fetal heart with the cardiac apex rotating to the left and posterior were confirmed in all 7 left lung agenesis cases, while the rightward shifting of the fetal heart together with the cardiac axis deviating to the right were confirmed in all 11 cases of right lung agenesis. The disappearance of the ipsilateral pulmonary artery was confirmed in all 18 cases of unilateral lung agenesis. Cardiac anomalies were present in a total of 7 of the 18 cases of lung agenesis with 4 of 7 in cases of left lung agenesis and 3 of 11 in cases of right agenesis.
CONCLUSIONS
The systematic approach introduced in the current report is helpful in the diagnosis of fetal unilateral lung agenesis complicated with cardiac malposition. The information provided by this study may be helpful to better understand unilateral lung agenesis anatomically and to facilitate its potential examination.
Topics: Abnormalities, Multiple; Echocardiography, Doppler, Color; Female; Humans; Levocardia; Lung; Lung Diseases; Pregnancy; Pulmonary Artery; Retrospective Studies; Ultrasonography, Prenatal
PubMed: 23530545
DOI: 10.1186/1471-2393-13-79 -
Annals of Surgical Treatment and... Dec 2014We report a case of common bile duct (CBD) cancer, successfully managed with pancreaticoduodenectomy, in a patient with isolated levocardia, malrotation, and situs...
We report a case of common bile duct (CBD) cancer, successfully managed with pancreaticoduodenectomy, in a patient with isolated levocardia, malrotation, and situs ambiguous (without splenic anomalies). A 59-year-old male patient was referred to Chung-Ang University Hospital with epigastric pain and jaundice. CT and MRI revealed distal CBD cancer without significant lymphadenopathy. Multiple abdominal anatomic anomalies were identified preoperatively, whereas no anatomic anomalies were detected within the chest. The patient had a right-sided stomach and spleen, liver at the midline, several vascular variations around the celiac axis, and intestinal malrotation, but the inferior vena cava and portal vein were normal. A pancreaticoduodenectomy was performed to treat the cancer. The postoperative course was favorable, and the patient was started on combined chemotherapy and radiotherapy 15 days after the surgery. In this case study, we report that pylorus preserving pancreaticoduodenectomy was successful for distal CBD cancer in a patient with rare situs anomalies.
PubMed: 25485244
DOI: 10.4174/astr.2014.87.6.340 -
Surgical Case Reports Feb 2018Situs inversus is a rare congenital condition that is currently classified into two types: complete situs inversus (situs inversus totalis, SIT) and partial situs...
BACKGROUND
Situs inversus is a rare congenital condition that is currently classified into two types: complete situs inversus (situs inversus totalis, SIT) and partial situs inversus (situs inversus partialis, SIP). In SIP patients, some organs are inverted and others are in their expected position, and individual patient variation in organ position increases surgical difficulty. Several surgeons have performed laparoscopic or robotic surgeries in situs inversus patients, but almost all were SIT patients. We report the first case, to our knowledge, of an SIP patient with gastric cancer who was successfully treated by robot-assisted distal gastrectomy (RADG) with lymph node dissection.
CASE PRESENTATION
A 64-year-old woman diagnosed with early gastric cancer on the posterior midbody of the stomach was referred to our hospital for treatment. Computed tomography showed levocardia and inverted abdominal organs without enlarged lymph nodes or distant metastases. Polysplenia syndrome, intestinal malrotation, and left-sided gallbladder were also detected. RADG with D1+ lymph node dissection and Billroth I reconstruction (delta-shaped anastomosis) were performed using robotics. Hepatopathy caused by a liver retractor and pancreatic fistula were identified during the postoperative course, and the latter was classified as grade II based on Clavien-Dindo classification. The patient was discharged 18 days after the operation.
CONCLUSIONS
Preoperative three-dimensional imaging is beneficial, and anatomical organ identification should be routinely performed, especially in SIP patients. We consider RADG a therapeutic option in SIP patients.
PubMed: 29441475
DOI: 10.1186/s40792-018-0422-7