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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 -
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 -
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 -
Revista Espanola de Cardiologia Feb 2003
Topics: Female; Humans; Levocardia; Magnetic Resonance Angiography; Middle Aged
PubMed: 12605767
DOI: No ID Found -
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 -
Open Heart 2020Congenital pericardial defect (CPD) is a rare entity with an estimated frequency of 0.01%-0.04%. The recognition of this anomaly is important since it can be associated...
OBJECTIVE
Congenital pericardial defect (CPD) is a rare entity with an estimated frequency of 0.01%-0.04%. The recognition of this anomaly is important since it can be associated with serious complications. The aim of this study and review was to describe clinical and imaging features that help in establishing the diagnosis of this condition.
METHODS
We retrospectively reviewed all adult patients at the Cleveland Clinic Health System with the diagnosis of CPD between the years 2000 and 2015. Baseline clinical characteristics, clinical manifestations, ECG, transthoracic echocardiography (TTE), cardiac CT and cardiac magnetic resonance (CMR) images were reviewed.
RESULTS
Eight patients were included in the study. Sixty-three percent of patients were males with mean age at diagnosis of 48 years, 63% had a partial pericardial defect on the left side and right ventricular (RV) dilation on TTE. Three patients had CMR. Levocardia was present in all CMRs. One patient had greater than 60° clockwise rotation and none of the CMRs showed ballooning of the left ventricular apex. One patient required surgical pericardioplasty. The remaining seven patients had a median follow-up of 17.3 months (5-144.9 months) and all remained asymptomatic.
CONCLUSION
CPDs are more likely to be partial on the left side and patients often have RV dilation on the TTE and levocardia on CMR. Most patients remain stable and do not require surgical intervention. TTE and CMR play an important role in making the diagnosis of this anomaly.
Topics: Adult; Cardiac Imaging Techniques; Clinical Decision-Making; Critical Pathways; Decision Support Techniques; Echocardiography; Electrocardiography; Female; Heart Defects, Congenital; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Pericardium; Predictive Value of Tests; Prognosis; Retrospective Studies; Time Factors; Tomography, X-Ray Computed
PubMed: 32076559
DOI: 10.1136/openhrt-2019-001103 -
Cleveland Clinic Journal of Medicine 1991Isolated levocardia with situs inversus, or ILSI, is a rare anomaly with a reported incidence of 1:22,000 in the general population and from 0.4% to 1.2% of all patients... (Review)
Review
Isolated levocardia with situs inversus, or ILSI, is a rare anomaly with a reported incidence of 1:22,000 in the general population and from 0.4% to 1.2% of all patients with congenital heart diseases. It is commonly associated with both congenital heart disease and splenic abnormalities, either asplenia or polysplenia. The prognosis is poor, and only about 5% to 13% of patients survive more than 5 years. The case described here, which had computed tomographic findings, is the first case reported with multiple malignancies associated with ILSI. This patient, at 73 years of age, is the second longest survivor reported in the literature.
Topics: Abnormalities, Multiple; Aged; Female; Humans; Levocardia; Neoplasms, Multiple Primary; Prognosis; Situs Inversus; Survival Rate; Tomography, X-Ray Computed
PubMed: 1893555
DOI: 10.3949/ccjm.58.3.243 -
Heart Asia 2018A 38-year-old woman, 37+3 weeker was incidentally detected to have fetal cardiomegaly during 36 weeks ultrasound and referred for fetal echocardiogram. Antenatal history...
UNLABELLED
A 38-year-old woman, 37+3 weeker was incidentally detected to have fetal cardiomegaly during 36 weeks ultrasound and referred for fetal echocardiogram. Antenatal history and anomaly scan were normal. Fetal echocardiogram showed heart rate of 153/min, sinus rhythm, situs solitus, levocardia, dilated right atrium and ventricle with venoatrial, atrioventricular and ventriculoarterial concordances. All cardiac valves were normal; cardiac crux was intact with separate mitral and tricuspid annuli (figure 1A-C and online supplementary video 1). Mild tricuspid regurgitation with peak velocity of 1.77 m/s was noted. There was no evidence of Ebstein's anomaly. Interventricular septum was intact. Pulmonary veins were seen entering left atrium (LA) without a retrocardiac chamber. Both aortic and ductal arches were adequate but with visualised in descending aorta. Three-vessel view showed (SVC) with normal spatial arrangement and sizes of aorta and pulmonary artery. Patent foramen ovale and patent ductus arteriosus were shunting normally. Inferior vena cava (IVC), hepatic and portal veins were normal. Significant hepatomegaly was seen without any evidence of hydrops. 10.1136/heartasia-2018-010998.supp1Supplementary data Figure 1(A) Fetal echocardiogram showing atrioventricular concordance with right atrium (RA) connected to morphological right ventricle and left atrium (LA) connected to morphological left ventricle (LV). Blue arrow depicts a large foramen ovale shunting from RA to LA. There is no retrocardiac chamber behind LA. (B) Three-vessel view in fetal echocardiogram showing normal right to left arrangement of superior vena cava (SVC), aorta (Ao) and pulmonary artery (PA). Interestingly, SVC is bigger than its neighbours which is against the norm. (C) Fetal short-axis echocardiographic view showing situs solitus with normal arrangement of RA and LA. RA is draining into right ventricle which is draining through PA into the ductal arch. However, significant aortic run-off is noted in aorta.
QUESTION
As per the available echocardiographic data, what is the most likely diagnosis for fetal heart failure?Idiopathic dilatation of SVC?Supracardiac total anomalous pulmonary venous connection?Fetal vein of Galen malformation?Fetal anaemia?
PubMed: 29636830
DOI: 10.1136/heartasia-2018-010998