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2012-10-01-14 Tricuspid valve anomalies ©Moshe Bronshtein www.TheFetus.net 


Tricuspid valve anomalies  

Moshe Bronshtein, MD1Sandro Egenburg, MD2.
1   Department Obstetrics Gynecology, Rambam Medical Center, Haifa, 31096 Israel
2   Bnai Zion Medical Center department of Pathology Haifa  .  

Case report:

We reported all cases of right heart anomalies which were found out in early pregnancy.

1. Tricuspid atresia :

Case 1: This is a case of
tricuspid atresia, low risk pregnancy, at 15 weeks.
 
The abnormal findings were hypoplastic right ventricle without inlet flow (clip 1), ventricular septal defect, echogenic and atretic tricuspid valve (clip 2). Termination was done without autopsy

Video clip 1, 2:




Case 2:  Low risk pregnancy, at 16 weeks

The abnormal findings were : corrected transposition of great vessels (TGA), atretic tricuspid valve (arrow), huge ventricular septal defect, hypoplastic right ventricle (clip 3); 
interruption inferior vena cava and unroofed coronary sinus which were confirmed in autopsy. The spleen was normal. The chromosome were normal.

Video clip 3:





2. T
ricuspid dysplasia:


Case 1: M
aternal age 23, second low risk pregnancy, at 16 weeks

Transvaginal ultrasound revealed the following
 abnormal findings:
  • Severe oligohydramnios
  • The only clue to observe a kidney was a multicystic small structure in the pelvis, no kidney beneath the diaphragm.
  • Spongy, low contractile right ventricle, dysplastic septal tricuspid leaflet, pericardial effusion. We suspected Uhls' anomaly with combination of tricuspid dysplasia.
The couple refused to further investigation (due to religious reason). The neonate died after 4 days without autopsy.

Video clip 4, 5:

 
Case 2: Maternal age 31, third low risk pregnancy, 16 weeks, transvaginal ultrasound showed dysplastic septal tricuspid leaflet, other organs were normal with normal karyotype. 

Video clip 6, 7:


The pregnancy was terminated and the autopsy was attached following:
 
 
 

3. Ebstein anomaly:

Low risk pregnancy, no history of Lithium, 15 weeks: 

At the first glance, it looks like normal four-chamber view.
 
Through a more profound examination, there is a small echogenic "step" apical in the septum (arrow).
In the color mode, there is a severe regurgitation from almost near the apex arrow (in tricuspid regurgitation, the origin of the regurgitation is usually from the level of the AV line).
 
At 23 weeks, the classical appearance of the Ebsteins with atrialization of the right ventricle, the arrow showing the septal low inserted valve.

The couple decided to terminate the pregnancy and the anomaly was proved by the autopsy.

Video clip 8-10:




4. Tricuspid stenosis:

 

Case 1: First low risk pregnancy, week 15, the only abnormal finding was very narrow tricuspid valve opening with diminished inlet flow.   

 

The pregnancy was terminated, the diagnosis was confirmed in autopsy.  The karyotype was normal (clip)

Video clip 11:



Case 2: After infertility treatment - IVF + intra-cytoplasmic sperm injection (ICSI) ( no other risk factors)

At  week 15,  the abnormal findings  hypoplastic right ventricle, severe ventricular septal defects, overriding aorta (clip).

Video clip 12:


 

The couple decided to terminate the pregnancy. 


5. Unguarded tricuspid orifice - A p
ulmonary artery valve atresia


In these 2 cases, the sonographic findings were : severe tachycardia (190bpm) ( some clips were "slow motioned" for a better demonstration) in the right compartment, the ventricle and atrium have the same dimensions .There is a huge parallel bidirectional flow  between right atria and ventricle and between left and right atria. No right outlet (pulmonary/ductal) could be demonstrated.
 
Case 1: Maternal age 40, one normal child, the parents are first degree cousins, week 14, no other anomalies.
Video 
clips show huge bidirectional flow. The pregnancy was terminated without autopsy.

Video clip 13, 14:



Case 2: Maternal age 34, first pregnancy, no risk factors
 
Clip 15 at 12 weeks and and clip 16,17 at 16 weeks with color Doppler  were slowed  for a better demonstration. The pregnancy was terminated.

The autopsy showed normal width of single aorta ( arrows)

Video clip 15-17:



To my experience, in the majority of  cardiac anomalies in which there is an absence or hypoplasia of one of the great arteries, there must be a compensatory dilatation of the second or of the remind vessel.
 
This case and single ventricle are the only cases in which there is a normal width -single artery 


 
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