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2016-06-11  Pulmonary atresia with intact venticular septum  © Islam Badr

Pulmonary atresia with intact venticular septum

Rasha Kamel, MD.1; Sameh Abdel Latif Abdel Salam, M.Sc.2; Islam Badr, M.Sc.3

1. Professor of obstetrics and gynecology, Fetal Medicine Unit, Cairo University , Egypt.
2. Radiodiagnosis department, Kasr Alainy teaching hospitals, Cairo University, Egypt. 
3. Fetal Medicine Unit, Cairo University, Egypt.

Case report

A 25-year old woman (G3P2) was referred to us for complete fetal cardiac assessment due to suspected Ebstein malformation.

Ultrasound examination at 35 weeks gestation revealed the following findings:

- Hugely dilated right atrium with severe tricuspid regurgitation.

- Normal insertion of septal leaflet of tricuspid valve. Yet, severe tricuspid valve dysplasia was noted (markedly thickened valve leaflets).

- Dilated hypertrophied right ventricle compared to the smaller but normal left ventricle regarding volume and overall function with early diastolic paradoxical septal motion denoting elevated RV pressures.

- Markedly hypoplastic main pulmonary artery compared to the ascending aorta. The pulmonary valve leaflets appeared thickened and imperforate denoting pulmonary valve membranous atresia. Branches of the pulmonary artery, arterial duct and collaterals if present could not be properly seen. However, ductal dependent pulmonary circulation was considered due to pulmonary valve atresia and severely hypoplastic main pulmonary artery.

- Coronary arteries could not be assessed.

Our final diagnosis was pulmonary atresia with intact ventricular septum (PAIVS) in association with tricuspid dysplasia. It represents a rare morphological variant in which membranous atresia occurred (complete 
fusion of pulmonary valve leaflets) associated with dilated tripartite right ventricle and patent RV infundibulum.

Postnatal echocardiography confirmed the prenatal findings with ductal dependent pulmonary circulation and normal appearance of coronary arteries at their origin. IV infusion of prostaglandins was started due to small tortuous arterial duct (not suitable for stenting) and a decision of modified Blalock-Taussig shunt was taken but oxygen saturation dropped on the next day and the baby died despite intense resuscitation measures.


Images 1, 2, 3 and 4; videos 1, 2, 3 and 4: show dilated RV, severe tricuspid dysplasia with improper coaptation of tricuspid valve leaflets, and hugely dilated right atrium. No abnormal apical displacement of septal leaflet of tricuspid valve was seen.


Image 5: c
olor Doppler image shows severe tricuspid regurgitation.


Image 6; videos 5: 
show patent RV infundibulum with imperforate pulmonary valve leaflets (membranous atresia due to complete fusion of valve leaflets).


Image 7: 
Inversion mode rendering showing the normal differential offset of both mitral and tricuspid valves leaflets. Note that the leaflets are in black with the septal leaflet of the tricuspid valve having normal insertion excluding Ebstien anomaly. A jet of tricuspid regurgitation is also noted.


Image 8 and 9: 
Inversion mode and sonoAVC rendering of both atria showing the size discrepancy between them.


Image 10:
Surface and inversion mode rendering of the pulmonary valve clearly showing the membranous atresia of the valve with absence of systodiastolic excursion. 


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