scholarly journals Prenatal diagnosis of total anomalous pulmonary venous connection to the portal vein associated with right atrial isomerism

2003 ◽  
Vol 21 (4) ◽  
pp. 393-396 ◽  
Author(s):  
S. Boopathy Vijayaraghavan ◽  
A. R. Rao ◽  
G. Padmashree ◽  
M. L. Raman
Keyword(s):  
Prenatal Diagnosis ◽  
Portal Vein ◽  
Right Atrial ◽  
Atrial Isomerism ◽  
Anomalous Pulmonary Venous Connection

scholarly journals BIDIRECTIONAL GLENN SHUNT AND REPAIR OF TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION IN A 3 YEARS OLD CHILD WITH HETEROTAXY SYNDROME: A CASE REPORT

2021 ◽  
Vol 54 (3) ◽  
pp. 273-274
Author(s):  
Shahyad Salehi-ardebili ◽  
Mohammad Radvar ◽  
Behnam Askari
Keyword(s):  
Surgical Repair ◽  
Rare Condition ◽  
Right Atrial ◽  
Older Children ◽  
Glenn Shunt ◽  
Bidirectional Glenn Shunt ◽  
Atrial Isomerism ◽  
Suitable Group ◽  
Anomalous Pulmonary Venous Connection ◽  
Very High

Right Atrial Isomerism (RAI) and total anomalous pulmonary venous connection (TAPVC) is a rare condition with very high mortality in neonatal period, but outcomes may be better in older children. A cyanotic 3 years old boy with dyspnea and growth retardation diagnosed with RAI and supracardiac type of TAPVC undergone a successful repair of TAPVC and simultaneous Glenn shunt. Older patients with RAI may be a distinct, more suitable group for surgical repair.

Pulmonary atresia with intact ventricular septum: a multicenter analysis of 21 prenatal diagnosis cases

2017 ◽  
Author(s):  
M.V. Medvedev , M.V. Kubrina , O.L. Galkina et all
Keyword(s):  
Prenatal Diagnosis ◽  
Aortic Arch ◽  
Tricuspid Valve ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Right Atrial ◽  
Intact Ventricular Septum ◽  
Multicenter Analysis ◽  
The Right ◽  
Atrial Isomerism

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.

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.

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