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Children (Basel, Switzerland) Apr 2023Positional abnormalities per se do not require treatment, but in their place, the accompanying pulmonary pathology in dextroposition patients and pathophysiologic... (Review)
Review
Positional abnormalities per se do not require treatment, but in their place, the accompanying pulmonary pathology in dextroposition patients and pathophysiologic hemodynamic abnormalities resulting from multiple defects in patients with cardiac malposition should be the focus of treatment. At the time of the first presentation, treating the pathophysiologic aberrations caused by the defect complex, whether it is by improving the pulmonary blood flow or restricting it, is the first step. Some patients with simpler or single defects are amenable to surgical or transcatheter therapy and should be treated accordingly. Other associated defects should also be treated appropriately. Biventricular or univentricular repair dependent on the patient's cardiac structure should be planned. Complications in-between Fontan stages and after conclusion of Fontan surgery may occur and should be promptly diagnosed and addressed accordingly. Several other cardiac abnormalities unrelated to the initially identified heart defects may manifest in adulthood, and they should also be treated.
PubMed: 37189988
DOI: 10.3390/children10040739 -
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 -
World Journal For Pediatric &... Jan 2023We investigated the prevalence of isolated vascular rings in the general population of Southern Nevada.
OBJECTIVE
We investigated the prevalence of isolated vascular rings in the general population of Southern Nevada.
METHODS
We identified those prenatally and postnatally diagnosed with an isolated vascular ring between January 2014 and December 2021. We included only those with vascular or ligamentous structures completely encircling the trachea and esophagus. To investigate the prevalence of isolated vascular rings, we included only those with situs solitus, levocardia, and no significant intracardiac malformations.
RESULTS
We identified 112 patients. Of the 112, 66 (59%) were female. There were approximately 211,000 total live births in Southern Nevada for the study period, for an overall prevalence of 5.3 isolated vascular rings per 10 000 live births. However, for the years 2014 to 2017, the average prevalence figure was 3.5 per 10 000 live births, and for the years 2018 to 2021, the average prevalence figure was 7.1 (range 6.5-8.0) per 10 000 live births. Simultaneously, the prenatal detection rate rose from 66% to 86%.
CONCLUSIONS
Isolated vascular rings are common cardiovascular malformations. As prenatal detection rates in the Southern Nevada general population approach 90%, the prevalence figures for isolated vascular rings appear to asymptote at about 7 per 10 000 live births.
Topics: Pregnancy; Humans; Female; Male; Vascular Ring; Cardiovascular Abnormalities; Heart; Trachea; Nevada
PubMed: 36847763
DOI: 10.1177/21501351221122972 -
Children (Basel, Switzerland) Dec 2022A significant number of patients with dextrocardia and other cardiac malpositions have other congenital heart defects (CHDs). The incidence of CHDs in subjects with... (Review)
Review
A significant number of patients with dextrocardia and other cardiac malpositions have other congenital heart defects (CHDs). The incidence of CHDs in subjects with cardiac malpositions is significantly greater than that in normal children, and the prevalence varies with the associated visceroatrial situs. The most useful approach to diagnosis is segmental analysis. Firstly, dextroposition should be excluded. In segmental analysis, the visceroatrial situs, ventricular location, status of atrioventricular connections, the great artery relationship, and conotruncal relationship are determined with the use of electrocardiogram (ECG), chest X-ray, and echocardiographic studies, and, when necessary, other imaging studies, including angiography. Following identification of the afore-mentioned segments, the associated defects in the atrial and ventricular septae, valvar and vascular stenosis or atresia may be determined by a review of the historical information, physical examination, and analysis of chest roentgenogram, ECG, and echocardiographic studies. Along the way, a pictorial rendition of the terminology and diagnosis of cardiac malpositions is undertaken.
PubMed: 36553425
DOI: 10.3390/children9121977 -
Journal of Medical Case Reports Dec 2023Situs inversus with levocardia is a rare anomaly in which the heart is present in the left chest but the abdominal viscera are transposed. It is caused by a single...
BACKGROUND
Situs inversus with levocardia is a rare anomaly in which the heart is present in the left chest but the abdominal viscera are transposed. It is caused by a single incomplete penetration of an autosomal recessive gene. It is unclear what exactly causes situs inversus with levocardia. Even if situs inversus can be identified following a comprehensive physical examination, it is now possible to validate the results and search for further information and pathologies since medical imaging is so widely accessible.
CASE
A 15-year-old Oromo male child from a remote area of Bale Zone presented to the Goba Referral Hospital's medical emergency outpatient department complaining of periumbilical pain that had persisted for 4 months. He frequently came to our hospital and was admitted three times with the same problem. Objectively, there was tenderness over the left lower quadrant and periumbilical area. The sonographic evaluation discovered the transposition of the liver and spleen with cardiac apex on the left side. He received conservative treatment with ceftriaxone 1 g intravenous twice a day and metronidazole 500 mg intravenous for 5 days, and he went home improved.
CONCLUSION
Isolated levocardia is a rare form of situs inversus in which the heart is in the traditional levo position while the abdominal organs are in the dextro position. What causes situs inversus with levocardia is unknown. Despite the fact that situs inversus can be diagnosed after a thorough physical examination, medical imaging has allowed us to confirm the findings as well as understand more about diseases. Due to the severity of an underlying heart defect, situs inversus with levocardia has a dismal prognosis.
Topics: Child; Male; Adolescent; Humans; Levocardia; Situs Inversus; Heart Defects, Congenital; Viscera; Abdomen
PubMed: 38042875
DOI: 10.1186/s13256-023-04254-9 -
Turk Kardiyoloji Dernegi Arsivi : Turk... Dec 2021
Topics: Adolescent; Echocardiography; Female; Humans; Levocardia; Pericardium; Tomography, X-Ray Computed
PubMed: 34881712
DOI: 10.5543/tkda.2021.21132 -
Journal of Nuclear Medicine Technology Dec 2020Imaging of dextrocardia in humans requires an understanding of the orientation of the heart chambers and walls. There are many types of cardiac malpositioning, such as...
Imaging of dextrocardia in humans requires an understanding of the orientation of the heart chambers and walls. There are many types of cardiac malpositioning, such as dextrocardia (with or without situs inversus), mesocardia, and levocardia. Myocardial perfusion scintigraphy of dextrocardia has been explained in case reports and imaging atlases; however, myocardial viability assessment using nuclear medicine imaging techniques is less documented in the literature. In 2 cases of dextrocardia with situs inversus and 1 case of mesocardia, myocardial viability was assessed using Tc-sestamibi rest perfusion scintigraphy and F-FDG PET. Cardiac SPECT images of dextrocardia with situs inversus were acquired using the feet-first supine position with a 180° arc from left anterior oblique to right posterior oblique, whereas a right-lateral-to-left-lateral arc was used for mesocardia. The processing and reconstruction were done by entering the dataset for the feet-first supine position and repeating after entering the dataset for the feet-first prone position. The 2 sets of reconstructed images were compared for orientation of walls and cardiac chambers. The first processing, using the feet-first supine position, revealed an interchanged septum and lateral wall in reconstructed images of dextrocardia with situs inversus. This interchange was corrected by changing the position to prone during processing of the rest perfusion and PET raw data. The display of cardiac slices in various axes matched the conventional nomenclature for the septum and lateral wall, leading to easy interpretation. However, this change was not required in the mesocardia, for which the location of the heart chambers was not interchanged. Because the acquisition protocol for SPECT is a semicircular orbit, the various types of dextrocardia require careful selection of the arc, with the patient positioning kept feet-first supine. Processing and reconstruction of data by changing the patient position to prone was found to be most useful method of matching the septum and lateral wall orientation for interpretation of images.
Topics: Dextrocardia; Female; Humans; Male; Middle Aged; Myocardium; Nuclear Medicine; Positron-Emission Tomography; Technetium Tc 99m Sestamibi; Tissue Survival; Tomography, Emission-Computed, Single-Photon
PubMed: 32887761
DOI: 10.2967/jnmt.120.248435 -
Obstetrical & Gynecological Survey Jan 2016The incidence of congenital heart disease is most commonly reported in 8 per 1000 live births. Situs anomalies are among the least common forms of congenital heart... (Review)
Review
The incidence of congenital heart disease is most commonly reported in 8 per 1000 live births. Situs anomalies are among the least common forms of congenital heart disease. This study defines situs and describes the variations of fetal situs anomalies. Situs refers to the arrangement of the viscera, atria, and vessels within the body. Situs solitus describes the normal arrangement; situs inversus describes inverted arrangement of the viscera and atria; and situs ambiguous (also referred to as heterotaxy) describes disturbances in arrangements that can neither be identified as solitus nor inversus. This review will concentrate on heterotaxy, as it is the most complicated situs abnormality to define, classify, and study. Prognosis of heterotaxy is variable but most correlated with cardiac anatomy. Management is concentrated on numerous cardiac operations and requires a multidisciplinary approach to address coexisting congenital anomalies.
Topics: Adult; Dextrocardia; Female; Genetic Counseling; Heterotaxy Syndrome; Humans; Incidence; Levocardia; Pregnancy; Prognosis; Terminology as Topic; Ultrasonography, Prenatal
PubMed: 26819133
DOI: 10.1097/OGX.0000000000000262 -
Taiwanese Journal of Obstetrics &... Feb 2015Rapid genome-wide aneuploidy diagnosis using uncultured amniocytes and array comparative genomic hybridization (aCGH) is useful in pregnancy with abnormal ultrasound... (Review)
Review
Prenatal diagnosis and array comparative genomic hybridization characterization of trisomy 21 in a fetus associated with right congenital diaphragmatic hernia and a review of the literature of chromosomal abnormalities associated with congenital diaphragmatic hernia.
OBJECTIVE
Rapid genome-wide aneuploidy diagnosis using uncultured amniocytes and array comparative genomic hybridization (aCGH) is useful in pregnancy with abnormal ultrasound findings. The purpose of this report is to report a case of right congenital diaphragmatic hernia (CDH) associated with trisomy 21 diagnosed prenatally by aCGH and to review the literature of chromosomal abnormalities associated with CDH.
CASE REPORT
A 29-year-old woman was referred for genetic counseling at 25 weeks of gestation because of fetal CDH. The pregnancy was uneventful until 25 weeks of gestation when level II ultrasound detected isolated right CDH. Ultrasound showed that the liver and gallbladder were located in the right hemithorax, and there was levocardia. Fetal magnetic resonance imaging confirmed the diagnosis of right CDH with the gallbladder and part of the liver appearing in the right hemithorax and the heart shifting to the left hemithorax. Amniocentesis was immediately performed. About 10 mL of amniotic fluid was sent for aCGH analysis by use of the DNA extracted from uncultured amniocytes, and 20 mL of amniotic fluid was sent for conventional cytogenetic analysis. aCGH analysis revealed the result of arr 21p11.2q22.3 (9,962,872-48,129,895) × 3, consistent with the diagnosis of trisomy 21. Conventional cytogenetics revealed a karyotype of 47,XY,+21. Postnatally, polymorphic DNA marker analysis using DNAs extracted from the placenta and parental bloods showed a heterozygous extra chromosome 21 of maternal origin consistent with the result of maternal meiosis I nondisjunction.
CONCLUSION
Prenatal diagnosis of right CDH should raise a suspicion of chromosomal abnormalities especially trisomy 21 and the association of Morgagni hernia.
Topics: Abnormalities, Multiple; Adult; Amniocentesis; Chromosomes, Human, Pair 21; Comparative Genomic Hybridization; Down Syndrome; Female; Genetic Counseling; Hernias, Diaphragmatic, Congenital; Humans; Infant, Newborn; Karyotype; Magnetic Resonance Imaging; Male; Pregnancy; Prenatal Diagnosis
PubMed: 25675923
DOI: 10.1016/j.tjog.2014.12.001 -
Congenital Heart Disease Jul 2018We reviewed all patients evaluated at our center with situs solitus, levocardia, and a right aortic arch that were born between January 2000 and January 2018. (Observational Study)
Observational Study
PATIENTS
We reviewed all patients evaluated at our center with situs solitus, levocardia, and a right aortic arch that were born between January 2000 and January 2018.
RESULTS
From our databases, we identified 204 patients. We excluded patients with a double aortic arch from analysis. Of the 204 patients, 103 (50%) were male. Of the 204 patients, 95 (47%) had an isolated right aortic arch. Of the 95 with an isolated right aortic arch, 4 (4%) had chromosome 22q11.2 deletion syndrome, and 89 (94%) had a vascular ring. Of the 204 patients, 109 (53%) had a surgical intracardiac malformation. Of the 109, with an associated surgical intracardiac malformation, 38 (35%) had chromosome 22q11.2 deletion syndrome, and 28 (26%) had a vascular ring. Overall, of the 204 patients, 200 (98%) had a vascular ring, an associated intracardiac malformation, or both. Prenatal detection was 85% (40/47) for the subset of patients born in Southern Nevada between January 2015 and January 2018.
CONCLUSION
this review, to best of our knowledge, reports one of largest series of right aortic arches in the literature. Data from our cohort demonstrates that a right aortic arch in situs solitus is almost always associated with pathology. Further, our center's right aortic arch prenatal detection rate exceeds previous reports.
Topics: Abnormalities, Multiple; Aorta, Thoracic; Diagnosis, Differential; Echocardiography; Female; Humans; Infant, Newborn; Magnetic Resonance Imaging, Cine; Male; Situs Inversus; Tomography, X-Ray Computed; Vascular Malformations
PubMed: 30033669
DOI: 10.1111/chd.12623