scholarly journals INSIGHTS INTO VENTRICULAR MECHANICS IN THE UNLOOPED HEART: VENTRICULAR TWISTING AND UNTWISTING IN A RIGHT ATRIAL ISOMERISM SINGLE VENTRICLE FETAL MOUSE MODEL

2010 ◽  
Vol 55 (10) ◽  
pp. A44.E418
Author(s):  
David J. Sahn ◽  
Lowell H. Frank ◽  
Linda Leatherbury ◽  
Uyen T. Truong ◽  
Cecelia W. Lo ◽  
...  
Keyword(s):  
Mouse Model ◽  
Single Ventricle ◽  
Right Atrial ◽  
Fetal Mouse ◽  
Ventricular Mechanics ◽  
Atrial Isomerism

Unique foetal diagnosis of aorto-pulmonary collaterals in right atrial isomerism

2020 ◽  
pp. 1-3
Author(s):  
Wadi Mawad ◽  
Nathalie Dutil ◽  
Varsha Thakur
Keyword(s):  
Septal Defect ◽  
Single Ventricle ◽  
Pulmonary Stenosis ◽  
Venous Drainage ◽  
Atrioventricular Septal Defect ◽  
Right Atrial ◽  
Collateral Arteries ◽  
Anomalous Venous Drainage ◽  
Atrial Isomerism ◽  
Foetal Diagnosis

Abstract Right atrial isomerism is associated with complex cardiac malformations, particularly single-ventricle lesions; right atrial isomerism is rarely associated with aorto-pulmonary collateral arteries. We report a foetal diagnosis of right atrial isomerism, with an unbalanced atrioventricular septal defect, pulmonary stenosis, total anomalous venous drainage, and significant aorto-pulmonary collaterals diagnosed at 22 weeks’ gestation.

scholarly journals Antenatal diagnosis of total anomalous pulmonary venous connection in functional single ventricle hearts: Outcomes over 13-year period

Ultrasound ◽  
2018 ◽  
Vol 26 (1) ◽  
pp. 42-48
Author(s):  
Precylia Fernandes ◽  
Lito Mantagou ◽  
Ram Ramaraj ◽  
Umber Agarwal ◽  
Joyce Su Ling Lim
Keyword(s):  
Cardiac Surgery ◽  
Single Ventricle ◽  
Antenatal Diagnosis ◽  
Right Atrial ◽  
Cardiac Anomaly ◽  
Compassionate Care ◽  
Cardiac Ventricle ◽  
Fontan Surgery ◽  
Atrial Isomerism ◽  
Anomalous Pulmonary Venous Connection

Introduction A functionally single cardiac ventricle seen on foetal ultrasound scan carries a guarded prognosis. The antenatal diagnosis of anomalous pulmonary venous connection (APVC) remains challenging, if there is no associated structural cardiac abnormality. Antenatally, a combination of complex cardiac anomaly with suspected isomerism should raise the possibility of associated total anomalous pulmonary venous connection (TAPVC). There needs to be a high index of suspicion for TAPVC, in functional single ventricle and suspected isomerism, as this carries a very grim outcome postnatally. We illustrate foetal echocardiographic findings of suspected TAPVC and review outcomes of antenatal versus postnatal diagnosis of TAPVC with functional single ventricle. Methods We retrospectively reviewed our database over 13 years, focusing on foetal cardiac diagnosis, pregnancy outcomes, management and outcomes of livebirths with diagnosis of TAPVC with functional single ventricle. Results Thirteen patients were included in the review. For the nine antenatal patients, three pregnancies were terminated and six babies were born alive (four babies had compassionate care, two babies had cardiac surgery). One baby is alive at 8.5 years, after Fontan surgery. For the four postnatal patients, three babies had compassionate care (one alive at age 8.1 years) and one baby had cardiac surgery (died age nine weeks). Ten of the 13 patients have right atrial isomerism. Of these 10 patients, only two are alive. For the three non-isomeric babies, only one baby is still alive. There is heterogeneity of the type of TAPVC diagnosed with no particular group that offered better survival. Conclusion Antenatal diagnosis of TAPVC, even in the context of functional single ventricle remains challenging. If isomerism is suspected, targeted evaluation of pulmonary venous connection should be done. This combination of cardiac lesion carries a very grim outcome. The ability to make this diagnosis antenatally will add to the information and counselling given to these parents.

scholarly journals EP12.15: Prenatal diagnosed single ventricle associated with TAPVC, TGA, right atrial isomerism and pulmonary stenosis

2019 ◽  
Vol 54 (S1) ◽  
pp. 306-306
Author(s):  
S.Q. Hamzayeva ◽  
L. Huseynova ◽  
A.T. Farajov ◽  
S. Karimova ◽  
A. Guliyeva ◽  
...  
Keyword(s):  
Single Ventricle ◽  
Pulmonary Stenosis ◽  
Right Atrial ◽  
Atrial Isomerism

Concurrent right atrial isomerism, complete atrioventricular septal defect, and single ventricle in an L-transposition of great arteries patient complicated by brain abscess

2019 ◽  
Vol 29 (7) ◽  
pp. 999-1001 ◽  
Author(s):  
Emir Yonas ◽  
Raymond Pranata ◽  
Nuvi Nusarintowati
Keyword(s):  
Brain Abscess ◽  
Septal Defect ◽  
Single Ventricle ◽  
Surgical Excision ◽  
Atrioventricular Septal Defect ◽  
Right Atrial ◽  
Transposition Of Great Arteries ◽  
Complete Atrioventricular Septal Defect ◽  
Atrial Isomerism ◽  
Complete Atrioventricular

AbstractWe present a case of an 11-year-old Indonesian female who was referred to our facility after surgical excision of brain abscess. The patient has been previously diagnosed with right atrial isomerism, complete atrioventricular septal defect, and L-Transposition of great arteries. Multiple staged surgeries are required for the management of this condition.

scholarly journals Apex rotation as a risk factor for total anomalous pulmonary connection repair in single ventricle

2021 ◽  
Author(s):  
Hidetsugu Asai ◽  
Yasushige Shingu ◽  
Jin Ikarashi ◽  
Yuchen Cao ◽  
Daisuke Takeyoshi ◽  
...  
Keyword(s):  
Risk Factor ◽  
Vertebral Body ◽  
Single Ventricle ◽  
Right Atrial ◽  
Pulmonary Venous Obstruction ◽  
Sutureless Technique ◽  
High Incidence ◽  
Atrial Isomerism ◽  
Anomalous Pulmonary Venous Connection

Background: The high incidence of postoperative pulmonary venous obstruction (PVO) is a major mortality-associated concern in patients with right atrial isomerism and extracardiac total anomalous pulmonary venous connection (TAPVC). We evaluated new anatomical risk factors for reducing the space behind the heart after TAPVC repair. Methods: 18 patients who underwent TAPVC repair between 2014 and 2020 were enrolled. Sutureless technique was used in 12 patients and conventional repair in six patients. The angle between the line perpendicular to the vertebral body and that from the vertebral body to the apex was defined as the “vertebral-apex angle (V-A angle).” The ratio of post- and preoperative angles, indicating the apex’s lateral rotation, was compared between patients with and without PVO. Results: The median (interquartile range) age and body weight at repair were 102 (79-176) days and 3.8 (2.6-4.8) kg, respectively. The 1-year survival rate was 83% (median follow-up, 29 [11-36] months). PVO occurred in seven patients (39%), who showed an obstruction of one or two branches in the apex side. The postoperative V-A angle (46° [45°-50°] vs. 36° [29°-38°], P = 0.001) and the ratio of post- and preoperative V-A angles (1.27 [1.24-1.42] vs. 1.03 [0.98-1.07], P = 0.001) were significantly higher in the PVO group than in the non-PVO group. The cut-off values of the postoperative V-A angle and ratio were 41° and 1.17, respectively. Conclusions: A postoperative rotation of the heart apex into the ipsilateral thorax was a risk factor for branch PVO after TAPVC repair.

Prenatal ultrasound diagnosis of pulmonary atresia in combination with left atrial isomerism

2017 ◽  
Author(s):  
M Medvedev, M.V. Kubrina, O.S. Zarubina et all
Keyword(s):  
Left Atrial ◽  
Reverse Flow ◽  
Ductus Arteriosus ◽  
Pulmonary Atresia ◽  
Prenatal Ultrasound ◽  
Ultrasound Diagnosis ◽  
Right Atrial ◽  
Second Trimester ◽  
Left And Right ◽  
Atrial Isomerism

Two cases of prenatal ultrasound diagnosis of left atrial isomerism in the second trimester of gestation is presented. These two cases were in combination with pulmonary atresia and right aortic arch. Left atrial isomerism was identify by the digit-like shape of the left and right atrial appendages. The pulmonary atresia was identified on the basis of reverse flow in small pulmonary artery. A right aortic was identified by “U”-shaped confluence of aorta and ductus arteriosus in view of three vessels and trachea. The trachea was located between the vessels. The pregnancies were terminated and prenatal diagnosis was conformed at autopsy

scholarly journals Heterotaxy syndrome: This is the left, right?

2015 ◽  
Vol 19 (2) ◽  
Author(s):  
Lili Huang ◽  
Belinda J. Mitchell ◽  
Savvas Andronikou ◽  
Zarina I. Lockhat ◽  
Farhana Suleman
Keyword(s):  
Morphological Characteristics ◽  
Imaging Modalities ◽  
Right Atrial ◽  
Abdominal Organ ◽  
Heterotaxy Syndrome ◽  
Diagnostic Challenge ◽  
Complex Disorder ◽  
Left And Right ◽  
Atrial Isomerism ◽  
Heterotaxy Syndromes

Heterotaxy syndrome is a rare and complex disorder of the chest and abdominal organ arrangements, and presents a diagnostic challenge to the radiologist. This article describes the morphological characteristics of heterotaxy and situs abnormalities, in particular left and right atrial isomerism, and suggests an approach in evaluating the spectrum of abnormalities associated with heterotaxy syndromes, using appropriate imaging modalities.

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