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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 -
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 -
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 -
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 -
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 -
The Journal of Maternal-fetal &... Dec 2022We retrospectively analyzed our center's experience with the prenatal diagnosis of isolated perimembranous ventricular septal defects that underwent primary surgical...
OBJECTIVE
We retrospectively analyzed our center's experience with the prenatal diagnosis of isolated perimembranous ventricular septal defects that underwent primary surgical repair in infancy.
METHODS
We identified patients born in Southern Nevada, between October 2012 and October 2020, with prenatal care that underwent surgical closure of an isolated large perimembranous ventricular septal defect between 1 and 12 months of age. The description at surgery defined ventricular septal defect morphology. We included only those with situs solitus, levocardia without dextroposition, and without any other cardiovascular abnormality. We analyzed prenatal detection rates for each of the eight years.
RESULTS
We identified 81 patients. Of the 81, 35 (43%) had trisomy 21. We identified no other aneuploidies in those that underwent surgical repair; however, 1 had a 15q13.3 deletion syndrome, and 1 had a 22 q11.2 deletion syndrome. Of the 81, 27 (33%) overall were prenatally diagnosed. Increasing prenatal detection rates strongly correlated with time ( = 0.92, = .002).
CONCLUSIONS
Trisomy 21 is common in isolated perimembranous ventricular septal defects undergoing primary repair in infancy. Further, prenatal detection rates significantly improved over time, up to 65% detection in the current years.
Topics: Pregnancy; Female; Humans; Retrospective Studies; Down Syndrome; Heart Septal Defects, Ventricular; Prenatal Diagnosis
PubMed: 34139939
DOI: 10.1080/14767058.2021.1940933 -
Journal of Community Hospital Internal... 2019Tetralogy of Fallot is the most common cyanotic congenital heart disease. It consists of right ventricular outflow tract obstruction, a ventricular septal defect,...
Tetralogy of Fallot is the most common cyanotic congenital heart disease. It consists of right ventricular outflow tract obstruction, a ventricular septal defect, abnormally located aorta and right ventricular hypertrophy. It usually occurs as an isolated anomaly with a normally placed heart and abdominal viscera. We present a case of a 19 years old female who presented with a prolonged history of shortness of breath (SOB) and cyanosis. After undergoing echocardiography and cardiac computed tomography angiogram (CTA), she was diagnosed to be a case of Tetrology of Fallot (TOF) in association with situs inversus with levocardia also termed 'isolated levocardia'. The patient underwent surgical correction and she was asymptomatic with no residual cardiac defects on follow up after 6 months. Isolated levocardia is a rare condition that is usually associated with severe cardiac defects and a low life expectancy in untreated patients. It is unusual for it to be diagnosed in adults as in our case.
PubMed: 31258874
DOI: 10.1080/20009666.2019.1601980 -
The Journal of Maternal-fetal &... Feb 2021To analyze the more recent outcomes of fetuses with different laterality defects. Out of 1508 fetuses with congenital heart disease (CHD) diagnosed by echocardiography...
To analyze the more recent outcomes of fetuses with different laterality defects. Out of 1508 fetuses with congenital heart disease (CHD) diagnosed by echocardiography between January 1995 and December 2015, 94 (6.3%) presented abnormal visceroatrial situs: 42 till December 2002 (group 1, analyzed retrospectively) and 52 since then until December 2015 (group 2, analyzed prospectively). Twenty-one had left isomerism (Lisom), 22 right isomerism (Risom), 16 situs inversus- dextrocardia (SVI-dx), 5 situs inversus- levocardia (SVI-levo), and 30 situs solitus-dextrocardia (SSol-dx). Seventy-six cases (81%) had CHD, more frequently complex atrioventricular defect. Eighteen cases showed normal heart: half of subgroups SVI-dx and SVI-levo, a third of SSol-dx. Postoperative mortality in continuing pregnancy in group 2 was 43.7 versus 40% in group 1, lower in SVs-dx (0 versus 50%) and Lisom (33.3 versus 66.7%), worse in Risom (71.4 versus 25%). Total mortality in group 2 was 48.3 versus 55% in group 1, better in Ssol-dx (37.5 versus 50%) and in Lisom (27.4 versus 71.4%). In isolated forms all but one case are alive. Our fetal cases presented a relevant mortality in both periods. The outcomes were slightly better in a more recent era in SSol and Lisom.What is known about this topic?Out of laterality defects, the heterotaxies (i.e. left and right isomerism) are known to be associated to a relevant mortality in fetal and neonatal cases.Heterotaxies have a variable position of the heart, but some authors evaluated only those with dextrocardia in their studies on different laterality defects.Controversial data are presented in the literature for the outcomes of fetuses with laterality defects. Many large studies analyzed the data of a very long time period in which it is opinable to compare the results of the treatment, because of a recent improved management and surgical techniques.What does this study add?Our study presents an experience of a single center with cases of different types of laterality defects observed during a routine fetal echocardiography, not only heterotaxies.The knowledge of different laterality defects and of their characteristics (association with cardiac and extracardiac anomalies or with normal heart) is useful for the prenatal counseling.We compare the postoperative and total mortality in more recent period (since 2003) with respect to the previous era (1995-2002).
Topics: Echocardiography; Female; Fetus; Heart Defects, Congenital; Heterotaxy Syndrome; Humans; Infant, Newborn; Pregnancy; Retrospective Studies; Situs Inversus
PubMed: 31106637
DOI: 10.1080/14767058.2019.1610737