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2013-10-25-10 Case of the week #359 © Deblieck www.TheFetus.net 

 
Answer to the case of the week #359

December 05, 2013 - January 9, 2014

Philippe Deblieck, MD.

Schermbecker Landstr. 88a, 46485 Wesel, Germany.
 

Case report

This is a case of a 29-year-old patient, G2P1, 176 cm, 160 Kg (BMI = 51), who was referred to our department for second trimester screening. The obese patient was examined in the left lateral decubitus [1] and scanned through the flank.
The main, left and right pulmonary arteries, easily identified, were severely dilated and the cardiac axis was deviated to the left. Absent Pulmonary Valve Syndrome (APVS) was suspected. We looked for the other signs of this rare form of Tetralogy of Fallot and could demonstrate absent ductus arteriosus and ventricular septal defect (VSD).

Images and videos

Video 1: Four-chamber view of the heart shows left cardiac axis deviation and dilated right ventricle due to volume overload. Videos 2 and 3: demonstrate ventricular septal defect.

 



Figures 1 and 2: Transverse view of the upper chest in a plane between the right ventricular short axis and three-vessel view showing the dilated left and right pulmonary arteries giving “wings” to the heart (personal note of the author).

 

Videos 4 and 5: Videos in the same plane demonstrating in color Doppler the turbulent flow across the pulmonary ring.

 

Absent pulmonary valve syndrome (APVS) is a rare cardiac malformation accounting for less than 6 % of all cases of Tetralogy of Fallot (ToF) [2, 3, 4]. It was postulated that the absent patent ductus arteriosus could be the primary cause of the pathogenesis of APVS (Yeager and al.). Berg and al. proposed that the frequent absence of the arterial duct observed in APVS in later pregnancy is more likely the result of early selection than a prerequisite for the development of this lesion (the patency of the arterial duct in TOF with APVS leads to heart failure with subsequent demise early in pregnancy [4]. The distinctive feature of APVS is the airway obstruction caused by tracheobronchial compression that results from massive dilatation of the main pulmonary artery (PA) and its first- and second-order branches. Consequential tracheomalacia and bronchomalacia determine the timing and severity of respiratory compromise, as well as the morbidity and mortality of these patients [5]. 

Early one-stage primary repair using the technique of anterior translocation of the pulmonary artery [4] was performed at the German Pediatric Heart Centre, Sankt Augustin, Germany. The baby is doing well. The karyotype is normal (46 XY) and no deletion was found.

References

1. Benaceraff , A technical tip on scanning obese gravidae, Ultrasound Obstet Gynecol 2010; 35: 615–616.
2. Chaoui R.,Abuhamad A.,A Practical Guide to Fetal Echocardiography, Second Ed.
3. Jeanty P, Chaoui R., Fetal Echocardiography, Part 2: the anomalies.
4. Berg C. et al., Reversed end-diastolic flow in the umbilical artery at 10-14 weeks of gestation is associated with absent pulmonary valve syndrome in the fetus. Ultrasound Obstet Gynecol 2007; 30: 254–258.
5.Hraska V., Surgery for tetralogy of Fallot – absent pulmonary valve syndrome. Technique of anterior translocation of the pulmonary artery in V. Hraska et al. / Multimedia Manual of Cardiothoracic Surgery.

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