In utero diagnosis of obstructed supracardiac total anomalous pulmonary venous connection in a patient with right atrial isomerism and asplenia.
We describe a rare case of right atrial isomerism, levocardia, right-side stomach, obstructed supracardiac total anomalous pulmonary venous connection, double outlet right ventricle with complete atrioventricular septal defect and absent spleen. From the pulmonary venous confluence behind the atrium an ascending as well as a descending vertical vein communicated with the systemic venous system in the supracardiac as well as the infracardiac position. The pulsed and color Doppler examination of the individual pulmonary veins as well as of the vertical vein helped in making the diagnosis of obstructed total anomalous pulmonary venous connection. The diagnosis was made by fetal echocardiographic examination at 22 weeks of gestation and confirmed on postnatal echocardiography, cardiac catheterization, and at surgery.
Patel
C R
CR
Department of Pediatrics, Division of Pediatric Cardiology, Rainbow Babies and Children's Hospital, Case Western Reserve University School of Medicine, Cleveland, Ohio 44106, USA.
Lane
J R
JR
Muise
K L
KL
D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging*; D000328:Adult; D004374:Ductus Arteriosus, Patent / Q000000981:diagnostic imaging; D005260:Female; D005315:Fetal Diseases / Q000000981:diagnostic imaging; D006325:Heart Atria / Q000002:abnormalities*; D006801:Humans; D007536:Isomerism; D011667:Pulmonary Veins / Q000002:abnormalities*; D013154:Spleen / Q000002:abnormalities*; D018615:Ultrasonography, Doppler, Color; D018617:Ultrasonography, Doppler, Pulsed; D016216:Ultrasonography, Prenatal