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Absent pulmonary valve syndrome with tetralogy of Fallot and associated dextrocardia detected at an early gestational age of 26 weeks.

Bharati AH, Naware A, Merchant SA - Indian J Radiol Imaging (2008)

Bottom Line: Absence of the pulmonary valve results in a dilated main pulmonary artery, which can be seen as a cystic, pulsatile, paracardiac lesion on antenatal USG.Such a lesion, though rare, can easily be detected.The case was detected at 26 weeks of gestation.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Lokmanya Tilak Municipal General Hospital, Sion, Mumbai, India.

ABSTRACT
Absent pulmonary valve syndrome is a rare congenital anomaly, usually seen in association with a ventricular septal defect. It has been reported to occur in 3-6% of cases of tetralogy of Fallot. Absence of the pulmonary valve results in a dilated main pulmonary artery, which can be seen as a cystic, pulsatile, paracardiac lesion on antenatal USG. Such a lesion, though rare, can easily be detected. We report a case of this rare anomaly which was present in association with a ventricular septal defect, tetralogy of Fallot, and dextrocardia. The case was detected at 26 weeks of gestation.

No MeSH data available.


Related in: MedlinePlus

Color Doppler imaging in the left parasagittal plane of the fetal thorax shows the aorta (ao) and the dilated pulmonary artery, but no ductus arteriosus
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Figure 0003: Color Doppler imaging in the left parasagittal plane of the fetal thorax shows the aorta (ao) and the dilated pulmonary artery, but no ductus arteriosus

Mentions: A 20-year-old primigravida presented for routine prenatal USG scanning. There was a single fetus of about 26 weeks' gestation and severe maternal polyhydramnios. The fetus showed a pulsatile cystic lesion located near the heart. We therefore performed detailed fetal echocardiography, which revealed abdominal situs solitus (liver on the right side and fundic bubble on the left side); there was also dextrocardia with atrial situs solitus [Figure 1]. The superior and inferior vena cava drained normally into the right atrium. Atrioventricular concordance was noted. The tricuspid and mitral valves were normal. The right ventricle (RV) was seen to open into the pulsatile cystic lesion, which was thus confirmed to be the dilated main pulmonary artery along with the right and left pulmonary arteries. The pulmonary annulus showed no pulmonary valve (p-valve) echoes [Figure 2]. The ductus arteriosus was not visualized [Figure 3]. There was back-and-forth flow across the RV and the dilated pulmonary artery during systole and diastole of the RV [Figure 4]. The aorta was seen arising from the left ventricle (LV). A large subaortic ventricular septal defect (VSD) was seen [Figure 5]. The fetus also showed mild ascites and hypoplasia of the right lung [Figure 6]. Other systems were unremarkable. Thus, a diagnosis of TOF with dextrocardia and absent p-valves, along with absent ductus arteriosus and hypoplasia of the right lung, was made. The fetus was stillborn at 29 weeks of gestation and autopsy findings confirmed the USG and fetal echocardiographic findings of TOF with APVS.


Absent pulmonary valve syndrome with tetralogy of Fallot and associated dextrocardia detected at an early gestational age of 26 weeks.

Bharati AH, Naware A, Merchant SA - Indian J Radiol Imaging (2008)

Color Doppler imaging in the left parasagittal plane of the fetal thorax shows the aorta (ao) and the dilated pulmonary artery, but no ductus arteriosus
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2747463&req=5

Figure 0003: Color Doppler imaging in the left parasagittal plane of the fetal thorax shows the aorta (ao) and the dilated pulmonary artery, but no ductus arteriosus
Mentions: A 20-year-old primigravida presented for routine prenatal USG scanning. There was a single fetus of about 26 weeks' gestation and severe maternal polyhydramnios. The fetus showed a pulsatile cystic lesion located near the heart. We therefore performed detailed fetal echocardiography, which revealed abdominal situs solitus (liver on the right side and fundic bubble on the left side); there was also dextrocardia with atrial situs solitus [Figure 1]. The superior and inferior vena cava drained normally into the right atrium. Atrioventricular concordance was noted. The tricuspid and mitral valves were normal. The right ventricle (RV) was seen to open into the pulsatile cystic lesion, which was thus confirmed to be the dilated main pulmonary artery along with the right and left pulmonary arteries. The pulmonary annulus showed no pulmonary valve (p-valve) echoes [Figure 2]. The ductus arteriosus was not visualized [Figure 3]. There was back-and-forth flow across the RV and the dilated pulmonary artery during systole and diastole of the RV [Figure 4]. The aorta was seen arising from the left ventricle (LV). A large subaortic ventricular septal defect (VSD) was seen [Figure 5]. The fetus also showed mild ascites and hypoplasia of the right lung [Figure 6]. Other systems were unremarkable. Thus, a diagnosis of TOF with dextrocardia and absent p-valves, along with absent ductus arteriosus and hypoplasia of the right lung, was made. The fetus was stillborn at 29 weeks of gestation and autopsy findings confirmed the USG and fetal echocardiographic findings of TOF with APVS.

Bottom Line: Absence of the pulmonary valve results in a dilated main pulmonary artery, which can be seen as a cystic, pulsatile, paracardiac lesion on antenatal USG.Such a lesion, though rare, can easily be detected.The case was detected at 26 weeks of gestation.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Lokmanya Tilak Municipal General Hospital, Sion, Mumbai, India.

ABSTRACT
Absent pulmonary valve syndrome is a rare congenital anomaly, usually seen in association with a ventricular septal defect. It has been reported to occur in 3-6% of cases of tetralogy of Fallot. Absence of the pulmonary valve results in a dilated main pulmonary artery, which can be seen as a cystic, pulsatile, paracardiac lesion on antenatal USG. Such a lesion, though rare, can easily be detected. We report a case of this rare anomaly which was present in association with a ventricular septal defect, tetralogy of Fallot, and dextrocardia. The case was detected at 26 weeks of gestation.

No MeSH data available.


Related in: MedlinePlus