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

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.

2021 ◽  
Vol 5 (2) ◽  
Author(s):  
Anas Abudan ◽  
Brent Kidd ◽  
Peter Hild ◽  
Bhanu Gupta

Abstract Background Inferior vena cava (IVC) obstruction is a rare complication of orthotopic heart transplantation (OHT) and is unique to bicaval surgical technique. The clinical significance, diagnosis, complications, and management of post-operative IVC anastomotic obstruction have not been adequately described. Case summary Two patients with end-stage heart failure presented for bicaval OHT. Post-operative course was complicated with shock refractory to fluid resuscitation and inotropic/vasopressor support. Obstruction at the IVC-right atrial (RA) anastomosis was diagnosed on transoesophageal echocardiography (TOE), prompting emergent reoperation. In both cases, a large donor Eustachian valve was found to be restricting flow across the IVC-RA anastomosis. Resection of the valve resulted in relief of obstruction across the anastomosis and subsequent improvement in haemodynamics and clinical outcome. Discussion Presumably rare, we present two cases of IVC obstruction post-bicaval OHT. Inferior vena cava obstruction is an under-recognized cause of refractory hypotension and shock in the post-operative setting. Prompt recognition using TOE is crucial for immediate surgical correction and prevention of multi-organ failure. Obstruction can be caused by a thickened Eustachian valve caught in the suture line at the IVC anastomosis, which would require surgical resection.


2018 ◽  
Vol 2018 ◽  
pp. 1-3 ◽  
Author(s):  
Sneha R. Gadi ◽  
Benjamin K. Ruth ◽  
Alan Johnson ◽  
Sula Mazimba ◽  
Younghoon Kwon

Inferior vena cava (IVC) diameter and respirophasic variation are commonly used echocardiographic indices to estimate right atrial pressure. While dilatation of the IVC and reduced collapsibility have traditionally been associated with elevated right heart filling pressures, the significance of isolated IVC dilatation in the absence of raised filling pressures remains poorly understood. We present a case of an asymptomatic 28-year-old male incidentally found to have IVC dilatation, reduced inspiratory collapse, and normal right heart pressures.


2013 ◽  
Vol 2013 ◽  
pp. 1-6 ◽  
Author(s):  
Christian Steinberg ◽  
Suzanne Boudreau ◽  
Felix Leveille ◽  
Marc Lamothe ◽  
Patrick Chagnon ◽  
...  

Hepatocellular carcinoma usually metastasizes to regional lymph nodes, lung, and bones but can rarely invade the inferior vena cava with intravascular extension to the right atrium. We present the case of a 75-year-old man who was admitted for generalized oedema and was found to have advanced HCC with invasion of the inferior vena cava and endovascular extension to the right atrium. In contrast to the great majority of hepatocellular carcinoma, which usually develops on the basis of liver cirrhosis due to identifiable risk factors, none of those factors were present in our patient.


2017 ◽  
Vol 23 (10) ◽  
pp. S39
Author(s):  
Akiko Idemoto ◽  
Haruhiko Abe ◽  
Kaori Yasumura ◽  
Hiroki Nishida ◽  
Taishi Kato ◽  
...  

Perfusion ◽  
2019 ◽  
Vol 35 (4) ◽  
pp. 306-315
Author(s):  
Muhammad Jamil ◽  
Mohammad Rezaeimoghaddam ◽  
Bilgesu Cakmak ◽  
Yahya Yildiz ◽  
Reza Rasooli ◽  
...  

Objective: Malposition of dual lumen cannula is a frequent and challenging complication in neonates and plays a significant role in shaping the in vitro device hemodynamics. This study aims to analyze the effect of the dual lumen cannula malposition on right-atrial hemodynamics in neonatal patients using an experimentally validated computational fluid dynamics model. Methods: A computer model was developed for clinically approved dual lumen cannula (13Fr Origen Biomedical, Austin, Texas, USA) oriented inside the atrium of a 3-kg neonate with normal venous return. Atrial hemodynamics and dual lumen cannula malposition were systematically simulated for two rotations (antero-atrial and atrio-septal) and four translations (two intravascular movements along inferior vena cava and two dislodged configurations in the atrium). A multi-domain compartmentalized mesh was prepared to allow the site-specific evaluation of important hemodynamic parameters. Transport of each blood stream, blood damage levels, and recirculation times are quantified and compared to dual lumen cannula in proper position. Results: High recirculation levels (39 ± 4%) in malpositioned cases resulted in poor oxygen saturation where maximum recirculation of up to 42% was observed. Apparently, Origen dual lumen cannula showed poor inferior vena cava blood–capturing efficiency (48 ± 8%) but high superior vena cava blood–capturing efficiency (86 ± 10%). Dual lumen cannula malposition resulted in corresponding changes in residence time (1.7 ± 0.5 seconds through the tricuspid). No significant differences in blood damage were observed among the simulated cases compared to normal orientation. Compared to the correct dual lumen cannula position, both rotational and translational displacements of the dual lumen cannula resulted in significant hemodynamic differences. Conclusion: Rotational or translational movement of dual lumen cannula is the determining factor for atrial hemodynamics, venous capturing efficiency, blood residence time, and oxygenated blood delivery. Results obtained through computational fluid dynamics methodology can provide valuable foresight in assessing the performance of the dual lumen cannula in patient-specific configurations.


1996 ◽  
Vol 83 (3) ◽  
pp. 354-355
Author(s):  
K. J. Oldhafer ◽  
G. Ziemer ◽  
S. Raygrotzki ◽  
R. Pichlmayr

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