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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 -
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 -
BMC Pregnancy and Childbirth Nov 2017To investigate the differences in cardiovascular disease, extracardiac anomalies and outcomes between fetuses with levocardia and dextrocardia. (Comparative Study)
Comparative Study
BACKGROUND
To investigate the differences in cardiovascular disease, extracardiac anomalies and outcomes between fetuses with levocardia and dextrocardia.
METHODS
Clinical demographics, prenatal features, postnatal characteristics and the outcomes of fetuses with levocardia or dextrocardia were recorded and analyzed.
RESULTS
Sixty-five fetuses with dextrocardia and thirty-eight fetuses with levocardia were enrolled. Right ventricle outlet obstruction, atrioventricular septal defect and intestinal malrotation were common in both groups. Univentricular physiology, transposition of the great arteries and esophageal atresia were more frequent in fetuses with levocardia, whereas abnormal pulmonary venous connection, double outlet of right ventricle, left ventricle outlet obstruction and brain abnormalities were more frequent in the dextrocardia group. The accuracy of evaluating cardiac malformations was high, but the sensitivity in assessing extracardiac abnormalities was low.
CONCLUSIONS
Although the disorders have certain overlapping features, there are several differences between fetuses with levocardia and dextrocardia. These findings might improve patient counseling and perinatal management.
Topics: Abnormalities, Multiple; Adult; Dextrocardia; Female; Fetal Heart; Heart Septal Defects; Heart Ventricles; Heterotaxy Syndrome; Humans; Levocardia; Pregnancy; Pulmonary Veins; Transposition of Great Vessels; Ventricular Function
PubMed: 29169330
DOI: 10.1186/s12884-017-1579-y -
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 -
Journal of Cardiology May 2017The right ventricle has a proclivity to wrap around the left ventricle outflow tract (LVOT) in congenitally corrected transposition (CCT) patients with apicocaval...
BACKGROUND
The right ventricle has a proclivity to wrap around the left ventricle outflow tract (LVOT) in congenitally corrected transposition (CCT) patients with apicocaval ipsilaterality, which may influence the outcome of the double switch operation (DSO). The goal of this study was to determine if the LVOT is compressed by the right ventricle in this setting.
METHODS
A total of 103 patients with CCT were divided into four groups according to ventricular looping and apical position, including Group A (D-loop and levocardia), Group B (L-loop and dextrocardia), Group C (D-loop and dextrocardia), and Group D (L-loop and levocardia). Computed tomography was used to define left-right laterality and ventro-dorsal relationship of the LVOT.
RESULTS
Apicocaval ipsilaterality was found in 57 patients (Group A, n=25; Group B, n=32), in whom the right ventricle was found to wrap around the LVOT. Among them, 49 (86%) had LVOT obstruction. In 46 patients without apicocaval ipsilaterality (Group C, n=10; Group D, n=36), 31 had LVOT obstruction (67.4%). LVOT obstruction was more prone to occur in patients with apicocaval ipsilaterality compared with those without (p=0.025), and was more significant in the situs solitus (p=0.058) than in situs inversus (p=0.547).
CONCLUSIONS
LVOT obstruction was prone to occur in CCT patients with situs solitus and apicocaval ipsilaterality (Group B). The ventricular outflow patency was influenced by apical position, which should be considered to avoid a posterior ventricular outflow tract from compression after DSO.
Topics: Adolescent; Child; Female; Heart Ventricles; Humans; Male; Retrospective Studies; Situs Inversus; Transposition of Great Vessels; Ventricular Outflow Obstruction
PubMed: 27842759
DOI: 10.1016/j.jjcc.2016.07.019 -
Experimental and Therapeutic Medicine Feb 2016The present study describes the case of a 2.5-year-old girl with double patent ductus arteriosus (PDA) that was successfully treated following interventional and...
The present study describes the case of a 2.5-year-old girl with double patent ductus arteriosus (PDA) that was successfully treated following interventional and surgical treatment. Bilateral ductus arteriosus is a very rare condition, which is assumed to occur when the branchial-type arterial system transforms into the mammalian-type arterial system during the development of the aorta and its branches. This case was misdiagnosed as ordinary PDA by echocardiography prior to the first surgery and the surgery was not successful because of poor accessibility. Enhanced computed tomography subsequently showed situs solitus, atrial situs, levocardia, right-sided aortic arch with right-sided descending aorta, an isolated left subclavian artery and double PDA. Interventional treatment was performed and intraoperative aortic arch angiography showed that the descending aorta was the origin of the first funnel-type PDA (PDA-1). The left subclavian artery was not connected to the aorta but was connected to the pulmonary artery with a very narrow winding duct, which was PDA-2. Interventional treatment via PDA-2 also failed because passing a guidewire through the twisted PDA-2 was difficult. The child was immediately transferred to the surgical operation room for double PDA ligation and left subclavian artery reconstruction under median thoracotomy. The surgical procedure succeeded and the patient recovered quickly. The failure of the interventional treatment may be attributed to the difficulty in establishing a path. The soft tip of the hardened guidewire was relatively long. If the hardened part of the wire was sent to the appropriate place to support the pathway, the soft tip would be forced to enter the vertebrobasilar artery system. A similar problem was encountered when the left subclavian artery was selected for intervention. Shortening the length of the soft tip of the hardened guidewire may have enabled smooth completion of the establishment of the pathway. However, this type of hardened guidewire requires specific production.
PubMed: 26893638
DOI: 10.3892/etm.2015.2916 -
Journal of Cardiovascular Echography 2016
PubMed: 28465957
DOI: 10.4103/2211-4122.178470 -
BMJ Case Reports Dec 2015A 69-year-old man with situs inversus, levocardia and inverted great arteries developed severe dynamic left ventricular outflow tract (LVOT) obstruction with systolic...
A 69-year-old man with situs inversus, levocardia and inverted great arteries developed severe dynamic left ventricular outflow tract (LVOT) obstruction with systolic anterior motion of the anterior mitral leaflet. There was no asymmetric septal hypertrophy. A possible mechanism of the LOVT obstruction in the present case may have been related to an abnormally long and bent outflow tract resulting from overriding of the right ventricle over the LVOT due to a congenital malposition of the heart. Mitral valve replacement with septal myectomy was performed in order to eliminate systolic anterior motion. The postoperative course has been excellent.
Topics: Aged; Heart Ventricles; Humans; Levocardia; Male; Tomography, X-Ray Computed; Ventricular Outflow Obstruction
PubMed: 26628311
DOI: 10.1136/bcr-2015-213030 -
Cardiology Research Dec 2015We present a case of a 19-year-old female presenting with cyanosis since birth. The major anomaly demonstrated was a "triply twisted heart" with a balanced physiology,...
We present a case of a 19-year-old female presenting with cyanosis since birth. The major anomaly demonstrated was a "triply twisted heart" with a balanced physiology, allowing her to survive into adulthood. Non-invasive imaging was done using 2D and real-time 3D (or 4D) echocardiography with multi-slice imaging from 4D volume datasets. Findings were confirmed using cardiac magnetic resonance imaging (MRI). A segmental approach revealed atrial and visceral situs inversus, levocardia, atrioventricular discordance, and ventriculoarterial discordance. Both the aorta and pulmonary artery were malposed and arise from the right ventricle (double outlet right ventricle or DORV). There was also a complete atrioventricular septal defect (CAVSD) associated with a functional single atrium and a functional univentricle (single ventricle). Other findings include a severe pulmonic stenosis (PS), preserved right and left ventricular systolic function, and a normal pulmonary arterial pressure. She also had a persistent left superior vena cava (SVC) that drains into the morphologic right atrium, while the right-sided SVC drains into the morphologic left atrium. A multidisciplinary team deemed that management be palliative. She is on regular follow-up at our clinics for non-invasive monitoring. To our knowledge, this is the first reported case in an adult with this combination of anomalies.
PubMed: 28197259
DOI: 10.14740/cr440w -
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