Fetal Ultrasonic Evaluation of Bronchial Morphology in Fetuses with Isomerism

2021 ◽  
pp. 1-10
Author(s):  
Xin Wang ◽  
Song Chen ◽  
Peng Tu ◽  
Xiaowei Liu ◽  
Xiaohang Zhang ◽  
...  
Keyword(s):  
Fetal Ultrasound ◽  
Ultrasonic Evaluation ◽  
Left Isomerism ◽  
Left Bronchus ◽  
Right Isomerism ◽  
Left And Right

<b><i>Objective:</i></b> The aim of the study was to evaluate whether fetal ultrasound could determine bronchial isomerism and distinguish left isomerism from right isomerism. <b><i>Methods:</i></b> We identified 110 healthy fetuses and 28 fetuses with isomerism. The outer angle between the tracheal midline and the inner margin of the bronchus is measured. The bronchial angles and the ratio of left/right bronchial angle were used to differentiate bronchial morphology and confirm the presence of bronchial isomerism in pathological cases. <b><i>Results:</i></b> The normal angles of the left and right bronchi were 146.98° (95% CI, 145.15–147.81°) and 167.37° (95% CI, 166.30–168.44°), separately. The cutoff bronchial angle of 156.5° was used to distinguish left bronchus from right bronchus. The bronchial isomerism could be identified in all pathological cases by autopsy and bronchial-atrial concordance occurred in 27 pathological cases (96.4%). In 21 pathological cases, the bilateral bronchial angle was &#x3c;156.5 versus &#x3e;156.5 differentiated left from right isomerism, respectively. The ratio of the left/right bronchial angle of &#x3e;0.935 identified 92.9% (26/28) of all pathological cases, with a sensitivity of 89.7%. <b><i>Conclusions:</i></b> Fetal ultrasound can detect the bronchial morphology and the presence of bronchial isomerism in fetuses with isomerism according to bronchial angles and the ratio of left/right bronchial angle.

scholarly journals Pre natal evaluation of heterotaxy syndrome by fetal echocardiography and correlating with autopsy

Ultrasound ◽  
2019 ◽  
Vol 27 (2) ◽  
pp. 111-121
Author(s):  
Madhavilatha Routhu ◽  
Imran Ali Mohammad
Keyword(s):  
Inferior Vena Cava ◽  
Inferior Vena ◽  
Vena Cava ◽  
Right Atrial ◽  
Heterotaxy Syndrome ◽  
Fetal Autopsy ◽  
Cardiac Anomalies ◽  
Left Isomerism ◽  
Right Isomerism ◽  
Visceral Heterotaxy

Introduction Heterotaxy syndrome/ isomerism is characterized by an abnormal symmetry of the viscera that are normally dissimilar due to abnormal lateralization of thoracic and abdominal viscera and is frequently associated with complex cardiac anomalies. Isomerism may be of right or left. Materials and methods This article describes the morphological characteristics of heterotaxy and suggests an approach in evaluating the spectrum of abnormalities associated with this syndrome. This study is based on 12 cases diagnosed on antenatal ultrasound as heterotaxy syndrome. Results of the examinations were re-evaluated and compared by fetal autopsy. Result Based on the following echocardiographic criteria, a diagnosis of left isomerism was made if there was viscerocardiac heterotaxy associated with an interruption of inferior vena cava or with bilateral finger-like atrial appendages or if it was associated with heart block. If there was evidence of viscerocardiac heterotaxy with complex cardiac anomalies then it was diagnosed as right atrial isomerism or visceral heterotaxy syndrome. We diagnosed 6/12 as left isomerism and rest of the cases as right isomerism/visceral heterotaxy syndrome. In Autopsy we evaluated visceral situs and morphology of the lungs and the main bronchi, the state of the liver, spleen, bowel, and the precise anatomy of the heart and confirmed 4/12 as left isomerism 4/12 as right isomerism and two cases as visceral heterotaxy syndrome (VHS). Rest of the two cases were included in the study despite missing autopsy data, as the combination of abnormal situs with interrupted inferior vena cava, and cardiac malformation allowed a diagnosis of left isomerism with high probability. Conclusion In this study, we aimed to find common features of heterotaxy syndrome on prenatal ultrasound as well as on fetal autopsy. This syndrome should be accurately diagnosed in the prenatal period in order to allow appropriate counseling of parents.

Arrangement of the systemic and pulmonary venous components of the atrial chambers in hearts with isomeric atrial appendages

2000 ◽  
Vol 10 (4) ◽  
pp. 396-404 ◽  
Author(s):  
Jee-Yeon Min ◽  
Chi-Yon Kim ◽  
Mee Hye Oh ◽  
Yi Kyeong Chun ◽  
Yeon-Lim Suh ◽  
...  
Keyword(s):  
Operative Management ◽  
The Body ◽  
Compound Structure ◽  
Left Isomerism ◽  
Right Isomerism ◽  
Definition Of

AbstractBackgroundThe morphological definition of atrial chambers, and the determination of atrial laterality, are based on analysis of the structure of the atrial appendages. The systemic and pulmonary venous connections to the heart, nonetheless, are important in the management of patients having isomeric appendages. In this study, therefore, we analysed the morphology of the postero-superior walls of the atrial chambers so as to provide evidence concerning the morphogenetic background of those hearts, and to improve operative management.MethodsWe reviewed 15 autopsied specimens with isomeric right appendages, and 10 with isomeric left appendages, paying particular attention to the morphology of the systemic and pulmonary venous connections. The postero-superior walls of the atrial chambers can be made up of the atrial body, the systemic venous components, or the pulmonary venous component. We analysed the contributions made by each of these components.ResultsThe postero-superior walls of the atrial chambers were markedly variable, but could be grouped into five patterns. Bilaterally well-developed systemic venous components and absence of the pulmonary venous component within the hypoplastic atrial body were present in 9 hearts with extracardiac pulmonary venous connections in the setting of right isomerism. Bilaterally well-developed systemic venous components, and a hypoplastic pulmonary venous component within the hypoplastic atrial body, were present in 5 hearts with intracardiac pulmonary venous connections in right isomerism. Bilaterally well-developed systemic venous components, and a hypoplastic pulmonary venous component within the sizable atrial body, were present in 1 heart with an intracardiac pulmonary venous connection in right isomerism. A well-developed pulmonary venous component within the atrial body, and hypoplasia of one systemic venous component, were present in 7 hearts with left isomerism. A well-developed pulmonary venous component within the atrial body, and hypoplasia of bilateral systemic venous components, were present in 3 hearts with left isomerism.ConclusionsThe postero-superior walls of the atrial chambers in hearts with isomeric atrial appendages can be analysed on the basis of a compound structure made of bilateral systemic venous components, a central pulmonary venous component, and the body of the atrium. Hearts with isomeric right appendages have absence or hypoplasia of the pulmonary venous component, while hearts with isomeric left appendages have hypoplastic systemic venous components.

Pulmonary and systemic arteriovenous fistulas in patients with left isomerism

1998 ◽  
Vol 8 (3) ◽  
pp. 290-294 ◽  
Author(s):  
Hiroaki Kawata ◽  
Hidefumi Kishimoto ◽  
Seiichiro Ikawa ◽  
Takayoshi Ueno ◽  
Tohru Nakajima ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Congenital Heart ◽  
Surgical Intervention ◽  
Venous Blood ◽  
Vasoactive Agent ◽  
Arteriovenous Fistulas ◽  
Left Isomerism ◽  
Hepatic Venous Blood ◽  
Right Isomerism ◽  
Pulmonary Arteriovenous Fistulas

AbstractAbstract Hepatic venous blood has been thought to play some role as a vasoactive agent in the development of pulmonary arteriovenous fistulas in patients with congenital heart disease. During the last 15 years, we have observed pulmonary arteriovenous fistulas in 3, and systemic arteriovenous fistulas in 2, patients from our 16 cases of left isomerism. During the same period, neither pulmonary nor systemic arteriovenous fistulas were detected among 50 patients with right isomerism. Pulmonary arteriovenous fistulas had developed in the absence of surgery in 1 of the patients. Both pulmonary and systemic fistulas were detected in an another patient, in whom the hepatic venous blood bypassed the pulmonary circulation. The level of somatostatin, which is known to reduce splanchnic blood flow, was high in the systemic venous blood of this patient. Although the mechanism of development of the fistulas has yet to be clarified, we should be aware that not only pulmonary, but also systemic arteriovenous fistulas can be found in patients with left isomerism, even prior to any surgical intervention.

scholarly journals Spontaneous Left Cardiac Isomerism in Chick Embryos: Case Report, Review of the Literature, and Possible Significance for the Understanding of Ventricular Non-Compaction Cardiomyopathy in the Setting of Human Heterotaxy Syndromes

2019 ◽  
Author(s):  
Jörg Männer
Keyword(s):  
Present Report ◽  
Bilateral Symmetry ◽  
Normal Function ◽  
Special Importance ◽  
Left Isomerism ◽  
Inner Organs ◽  
Cardiovascular Defects ◽  
Right Isomerism ◽  
Heterotaxy Syndromes ◽  
Made In

Except for a few species, the outer shape of vertebrates normally is characterized by bilateral symmetry. The inner organs, on the other hand, normally are arranged in bilaterally asymmetric patterns, which are of special importance for the normal function of the cardiovascular system of lung-breathing vertebrates. Deviations from the normal organ asymmetry can occur in the form of mirror imagery of the normal arrangement (situs inversus), or in the form of arrangements that have the tendency for development of bilateral symmetry, either in a pattern of bilateral left-sideness (left isomerism) or bilateral right-sidedness (right isomerism). The latter two forms of visceral situs anomalies are called &ldquo;heterotaxy syndromes&rdquo;. During the past 30 years, remarkable progress has been made in uncovering of the genetic etiology of heterotaxy syndromes. However, the pathogenetic mechanisms causing the spectrum of cardiovascular defects found in these syndromes remain poorly understood. In the present report, a spontaneous case of left cardiac isomerism found in a HH-stage 23 chick embryo is described. The observations made in this case suggest that hearts with left cardiac isomerism may have the tendency for development of a non-compaction cardiomyopathy caused by defective development of the proepicardium.

Disparity between the arrangement of the abdominal vessels and the bronchial tree and atrial appendages in a patient with mirror-imaged venous return

1998 ◽  
Vol 8 (2) ◽  
pp. 247-249 ◽  
Author(s):  
Nikos D. Soukias ◽  
Elliot A. Shinebourne ◽  
Julene S. Carvalho
Keyword(s):  
Venous Return ◽  
Bronchial Tree ◽  
Abdominal Vessels ◽  
Left Isomerism ◽  
Right Isomerism

SummaryEither arrangement of the abdominal vessels or the morphology of the bronchial tree are usually used to predict atrial arrangement. We report a patient in whom, in the setting of normal physiology as seen with mirror-imaged venous return, and due to disparity between the arrangement of the abdominal vessels (suggestive of right isomerism) and thoracic arrangement (indicative for left isomerism), atrial arrangement could not be inferred. Direct echocardiographic imaging of both appendages revealed left isomerism.

Improving results of atrioventricular valve repair in challenging patients with heterotaxy syndrome

2010 ◽  
Vol 20 (1) ◽  
pp. 60-65 ◽  
Author(s):  
Noritaka Ota ◽  
Yoshifumi Fujimoto ◽  
Keiichi Hirose ◽  
Yuko Tosaka ◽  
Tomohiro Nakata ◽  
...  
Keyword(s):  
Patient Population ◽  
Successful Outcome ◽  
Atrioventricular Valve ◽  
Valve Repair ◽  
Heterotaxy Syndrome ◽  
Excellent Outcome ◽  
Poor Outcome ◽  
Left Isomerism ◽  
Right Isomerism

AbstractObjectivesHeterotaxy syndrome, which is often associated with atrioventricular valvar regurgitation, has been considered a risk factor for the surgical repair for Fontan candidates. The results of atrioventricular valve repair in this challenging patient population remain largely unknown.MethodsFrom July, 1994 to January, 2007, 25 patients with the heterotaxy syndrome consisting of 22 right isomerism and three left isomerism presented to the Shizuoka Children’s Hospital Japan with significant (3–4+) atrioventricular valvar regurgitation necessitating atrioventricular valve repair. The clinical and Doppler/echocardiographic data were retrospectively reviewed to determine the efficacy of the repair and patient outcome.ResultsPatients were divided into two groups on the basis of atrioventricular valvar regurgitation at the most recent follow-up: those with a successful late outcome, (0–2) and those with a poor outcome (3–4). There were 17 (67%) patients with a successful outcome and 8 (33%) with a poor outcome. The repair technique including leaflet apposition was predictive of a successful outcome (p = 0.003). Overall survival was 64% (16/25). Survival was 88.2% (15/17) for patients with a successful result versus 12.5% (1/8) for those with a poor outcome (p = 0.0007). Of the 15 survivors, 13 have reached final completion of the Fontan circulation, and two currently remain at the bi-directional Glenn shunt stage.ConclusionAtrioventricular valve repair can be accomplished in this challenging patient population with excellent results. The combination of the leaflet apposition technique and the Kay suture can be performed with an excellent outcome in the majority of patients with heterotaxy syndrome, even with significant atrioventricular valvar regurgitation.

Segregating bodily isomerism or heterotaxy: potential echocardiographic correlations of morphological findings

2017 ◽  
Vol 27 (8) ◽  
pp. 1470-1480 ◽  
Author(s):  
Cornelia Tremblay ◽  
Rohit S. Loomba ◽  
Peter C. Frommelt ◽  
Donald Perrin ◽  
Diane E. Spicer ◽  
...  
Keyword(s):  
Coronary Sinus ◽  
Venous Return ◽  
Caval Vein ◽  
Segmental Analysis ◽  
Morphological Findings ◽  
Inferior Caval Vein ◽  
Left Isomerism ◽  
Right Isomerism ◽  
Systemic Venous Return ◽  
Anomalous Pulmonary Venous Connection

AbstractBackgroundBodily isomerism, also referred to as heterotaxy, involves predominantly the thoracic organs, although other organs are usually abnormally positioned. Previously assessed on the basis of splenic anatomy, it is now understood that isomerism is better segregated on the basis of atrial appendage morphology. This allows for anticipation of associated findings. We aimed to assess the accuracy of segregation based on the morphology of the atrial appendages and other structures more easily identified by echocardiography.MethodsWe reviewed postmortem specimens of hearts from the archives at four institutions categorised as obtained from patients with “heterotaxy”. The cardiac structures were analysed using sequential segmental analysis. Non-cardiac structures were also examined if available. Statistical analyses were performed to compare differences in the settings of right as opposed to left isomerism.ResultsSpecimens were available from 188 patients. Of these, 57 had left isomerism, and 131 had right isomerism. Atrial appendages were isomeric in all patients. A coronary sinus was found only in left isomerism, whereas a terminal crest, or a Eustachian valve, was found only in right isomerism. Interruption of the inferior caval vein was associated with left isomerism, whereas totally anomalous pulmonary venous connection was associated with right isomerism.ConclusionIsomerism is uniformly segregated on the basis of the morphology of the atrial appendages, itself defined by the extent of the pectinate muscles. Other features such as the presence of a coronary sinus and systemic venous return can further help with such segregation of isomerism.

Clinical investigations over 13 years to establish the nature of the cardiac defects in patients having abnormalities of lateralization

2007 ◽  
Vol 17 (3) ◽  
pp. 275-282 ◽  
Author(s):  
Selman Vefa Yildirim ◽  
Kürsad Tokel ◽  
Birgül Varan ◽  
Sait Aslamaci ◽  
Enver Ekici
Keyword(s):  
Pulmonary Atresia ◽  
Mirror Image ◽  
The Body ◽  
Diagnostic Tools ◽  
Fetal Life ◽  
Cardiac Defects ◽  
Cardiac Malformations ◽  
Left Isomerism ◽  
Right Isomerism ◽  
Anomalous Pulmonary Venous Connection

Introduction: The first step in diagnosing congenital cardiac malformations is to assess the arrangement of the atrial appendages. In patients with abnormal lateralization of the organs of the body, the arrangement of atrial appendages is neither normal, nor a mirror image of normal. There are 2 categories of abnormal arrangement based on the morphology of the atrial appendices, namely right isomerism and left isomerism, and in almost all instances these are found in the setting of so-called heterotaxy syndromes. Objective: To evaluate the various congenital cardiac malformations those are associated with abnormalities of lateralization, and to discuss the diagnostic tools, therapeutic options, and outcome for these patients. Patients and methods: We studied 134 patients, who had been admitted to our department of paediatric cardiology with known abnormalities of lateralization and congenital cardiac defects between 1990 and 2003. The data relating to each patient was evaluated retrospectively. The arrangement of the atrial appendages was established echocardiographically, and/or angiographically, and/or on the basis of morphologic investigations during the operation. These studies showed that 43 (32.1%) of the patients had right isomerism, and 88 (65.7%) had left isomerism. In 2 (1.5%), there was mirror-imaged arrangement, while in the final patient (0.07%), we were unable to determine sidedness with certainty. The median age at diagnosis was 0.66 years, and the females outnumbered the males in a ratio of 3 to 1. Patients in the 2 isomeric groups were compared with regard to age, cardiac defects, diagnostic tools and outcomes. Results: The difference in mean ages of the two groups of patients was statistically significant, those with right isomerism being 1.0 minus or plus 1.5 years, as opposed to those with left isomerism being 3.3 minus or plus 4.7 years (P is less than 0.005). Of the patients with right isomerism, 32 (74.4%) had left-sided, and 11 (25.6%) right-sided hearts, whereas in those with left isomerism, the hearts were left-sided in 65 (73.9%), and right-sided in 21 (23.9%), with 2 (2.2%) positioned in the midline. Extracardiac totally anomalous pulmonary venous connection was more common in those with right isomerism, being found in 13 patients (30.2%) as opposed to 5 patients (5.7%) with left isomerism. In only 8 of those with right isomerism did we find two perforate atrioventricular valves (18.6%), this arrangement being found in 34 (38.6%) of those with left isomerism. Pulmonary atresia and stenosis were present in 40 (93.0%) of those with right isomerism, but also in 41 (46.6%) of the patients with left isomerism. Angiographic and echocardiographic investigations were concordant in about three-quarters of patients with both right and left isomerism. All patients with extracardiac totally anomalous pulmonary venous connection died. Overall, 22 of the patients with right isomerism died (51.2%), as opposed to 20 (22.7%) of those with left isomerism. Conclusion: Our experience confirms that patients with right isomerism have more complex cardiac defects than those with left isomerism. Overall, the presence of isomerism carries a poor prognosis, the more so for right isomerism, with this related to the complex cardiac abnormalities. In our cohort, extracardiac totally anomalous pulmonary venous connection with pulmonary arterial obstruction was always a fatal combination. The mapping of cardiac and abdominal morphologies is still essential for proper diagnosis of these syndromes, especially in fetal life.

scholarly journals Surgical Management of Hearts with Isomeric Atrial Appendages

2022 ◽  
Author(s):  
Ujjwal Chowdhury ◽  
Robert Anderson ◽  
Diane E. Spicer ◽  
Lakshmi Sankhyan ◽  
Niraj Pandey ◽  
...  
Keyword(s):  
Septal Defect ◽  
Atrioventricular Septal Defect ◽  
Double Outlet Right Ventricle ◽  
Surgical Approaches ◽  
Caval Vein ◽  
Inferior Caval Vein ◽  
Left Isomerism ◽  
Right Isomerism ◽  
Pulmonary Arterial ◽  
Anomalous Pulmonary Venous Connection

Background and aim: On the basis of previously published accounts, coupled with our own experience, we have assessed the surgical approaches to patients with isomeric atrial appendages. Methods: We reviewed pertinent published studies on surgical treatment of individuals with isomeric atrial appendages, with the pertinent surgical details provided by most of the manuscripts. Results: Half of patients with right isomerism, and two-thirds of those with left isomerism have bilateral superior caval veins. Azygos extension of the inferior caval vein is reported in three-quarters of those with left isomerism. The coronary sinus is universally absent in right isomerism, along with totally anomalous pulmonary venous connection, and is absent in two-fifths of those with left isomerism.. Univentricular atrioventricular connections are expected in up to three-quarters of those with right isomerism. Atrioventricular septal defect is reported in up to four-fifths, more frequently in right isomerism, with such patients typically having discordant ventriculoatrial connections or double outlet right ventricle. Reported mortalities extend to 85% for those with right, and 50% for those with left isomerism. In right isomerism, mortality is up to 54% for systemic-to-pulmonary arterial shunting, up to 75% for univentricular repair, and up to 95% for repair of totally anomalous pulmonary venous connection itself. No more than one-quarter had undergone Fontan completion, with reported mortalities of 21%. Conclusion: Early surgical results are satisfactory in patients with left isomerism, but disappointing for those with right. Recent advances in cardiac and liver transplantation may offer improved survival.

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