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

Author(s):  
M Medvedev, M.V. Kubrina, O.S. Zarubina et all

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

Author(s):  
I.N. Daminov , S.F. Nasirova

The case of prenatal ultrasound diagnosis of fetal superior occipital encephalocele at 19 weeks of gestation is presented. The pregnancy was terminated at 20 weeks of gestation. An external examination of the abortus confirmed the prenatal ultrasound diagnosis.


Author(s):  
I.N. Daminov

A case of prenatal ultrasound diagnosis of fibular hemimelia, type I associated with oligodactyly of the foot at 22 weeks of gestation is presented. Ultrasound examination at 22 weeks of gestation revealed hypoplasia of the left fibula and the foot oligodactyly. After prenatal counseling, the patient opted for termination of pregnancy. During external examination of the abortus asymmetry of the lower extremities due to shortening of the left lower leg and oligodactyly of the left foot were noted, those confirmed prenatal ultrasound data.


2015 ◽  
Vol 19 (2) ◽  
Author(s):  
Lili Huang ◽  
Belinda J. Mitchell ◽  
Savvas Andronikou ◽  
Zarina I. Lockhat ◽  
Farhana Suleman

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.


2021 ◽  
pp. 1-8
Author(s):  
Anastasia Fotaki ◽  
Victoria L. Doughty ◽  
Winston Banya ◽  
Stefano Giuliani ◽  
Sarah Bradley ◽  
...  

Abstract Background and aim: Heterotaxy syndrome, being right atrial isomerism (RAI) or left atrial isomerism (LAI), often presents with Congenital Heart Disease (CHD). Intestinal abnormalities, including malrotation are common. We assessed the spectrum of gut abnormalities and their impact on medium-term outcome in a cohort of patients with fetal and postnatal diagnoses of heterotaxy syndrome. Methods: We reviewed the cardiology records of heterotaxy syndrome patients from two centres, regarding the presence of CHD, time for cardiac intervention, presence of gastrointestinal abnormalities, and type/time of surgery. A questionnaire about gastrointestinal status was sent to patients <18 years old. Kaplan–Meier curves were derived for survival data and freedom from intervention. Results: Data were included for 182 patients (49 RAI and 133 LAI) of 247 identified. Questionnaires were sent to 77 families and 47 replied. CHD was present in all RAI and 61.7% of LAI cases. Thirty-eight patients had abdominal surgery (20.9%), similar for RAI and LAI (20.4% versus 21%, p> 0.99): Ladd procedure in 17 (44.7%), non-Ladd in 12 (31.5%), and both procedures in 9 (23.7%). Ten-year freedom from Ladd procedure for all was 86% for the whole cohort (RAI = 87%; LAI = 85%, p = 0.98). Freedom from any gastrointestinal surgery at 10 years was 79% for the whole cohort (RAI = 77%; LAI = 80%, p = 0.54). Ten-year freedom from cardiac surgery was 31% for the whole cohort (RAI = 6%; LAI = 43%, p < 0.0001). Conclusions: In our cohort, one in five patients required abdominal surgery, mostly in their first year of life, similar for RAI and LAI. Between 1 and 10 years of follow-up, the impact of gastrointestinal abnormalities on outcome was minimal. Medium term survival was related to CHD.


2007 ◽  
Vol 35 (5) ◽  
pp. 250-255 ◽  
Author(s):  
Ágnes Harmath ◽  
Ákos Csaba ◽  
Erik Hauzman ◽  
Júlia Hajdú ◽  
Barbara Pete ◽  
...  

Author(s):  
M.V. Medvedev , M.V. Kubrina , O.L. Galkina et all

A retrospective analysis of 21 cases of prenatal diagnosed of pulmonary atresia with intact ventricular septum (PA-IVS) is presented. In the study of the four chambers view of the fetal heart the right ventricle was hypoplastic in 16 (76.1 %), normal in 5 (23.8 %) cases. Tricuspid atresia identified in 8 (38 %) cases. The dysplasia of the tricuspid valve identified in 5 (23.8 %) cases. In 2 (8.7 %) cases PA-IVS were in combination with atrioventricular defects. The changes of the tricuspid valve were not identified in 6 (28.5 %) cases. In 100 % cases reduced pulmonary artery with retrograde flow in the pulmonary valve was registrated. Hydrops fetal is detected in 2 (9.5 %) cases. The left aortic arch was in 17 (80.9 %) cases and right aortic arch – 4 (19.1 %). The average gestational age at prenatal diagnosis was 22,6 weeks (range 11–32 weeks). Early diagnosis is represented by one case in 11 weeks. Cardiac defects were registered in 6 (28.5 %) cases and extracardiac defects in 5 (23.8 %) cases. Ventriculocoronary arterial communications were found in 2 (9.5 %) cases of PA-IVS. In 3 (13 %) cases were in combination with heterotaxy syndrome. In 2 (8.7 %) cases PA-IVS were in combination with left atrial isomerism and 1 (4.8 %) with right atrial isomerism. Outcomes in fetuses with PA-IVC: termination of pregnancy — 13 (61.9 %), fetal death — 2 (9.5 %), neonatal death — 5 (23.8 %) cases, only 1 (4.8 %) survivor.


2003 ◽  
Vol 285 (5) ◽  
pp. H1837-H1848 ◽  
Author(s):  
Alan E. Lomax ◽  
Colleen S. Kondo ◽  
Wayne R. Giles

Consistent differences in K+ currents in left and right atria of adult mouse hearts have been identified by the application of current- and voltage-clamp protocols to isolated single myocytes. Left atrial myocytes had a significantly ( P < 0.05) larger peak outward K+ current density than myocytes from the right atrium. Detailed analysis revealed that this difference was due to the rapidly activating sustained K+ current, which is inhibited by 100 μM 4-aminopyridine (4-AP); this current was almost three times larger in the left atrium than in the right atrium. Accordingly, 100 μM 4-AP caused a significantly ( P < 0.05) larger increase in action potential duration in left than in right atrial myocytes. Inward rectifier K+ current density was also significantly ( P < 0.05) larger in left atrial myocytes. There was no difference in the voltage-dependent L-type Ca2+ current between left and right atria. As expected from this voltage-clamp data, the duration of action potentials recorded from single myocytes was significantly ( P < 0.05) shorter in myocytes from left atria, and left atrial tissue was found to have a significantly ( P < 0.05) shorter effective refractory period than right atrial tissue. These results reveal similarities between mice and other mammalian species where the left atrium repolarizes more quickly than the right, and provide new insight into cellular electrophysiological mechanisms responsible for this difference. These findings, and previous results, suggest that the atria of adult mice may be a suitable model for detailed studies of atrial electrophysiology and pharmacology under control conditions and in the context of induced atrial rhythm disturbances.


Author(s):  
O.V. Chursina, V.G. Volkov

The case of prenatal ultrasound diagnosis of partial aplasia of the parietal bones of the skull in the second trimester of pregnancy is presented. Questions of prenatal differential diagnosis and prognosis are discussed from the point of view of possible embryogenesis of this pathology.


Author(s):  
K.K. Otaryan , A.N. Chulkov , D.B. Shekhovtsov et all

A case of prenatal ultrasound diagnosis of pericardial lymphangioma at 19+3 weeks of gestation is presented. Prenatal diagnosis verified on histological analysis.


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