Centro de Estudios Ultrasonográficos Perinatales. Maternidad Las Acacias. Valencia. Venezuela.

Introduction: Anomalies of the aortic arch may be associated with congenital heart defects (50%), or present as incidental findings in asymptomatic infants. Coarctation, pseudocoarctation, interruption of the arch aortic, aberrant ramification with or without vascular rings, are some of the anomalies of the aortic arch whose diagnosis is possible by means of the radiological studies, magnetic resonance imaging (MRI) and spiral computed tomography (CT).  Several of these congenital anomalies of the aortic arch have been described in pediatrics and adult patient, while there are few prenatal diagnoses by ultrasound.

Achiron et al., indicate that the anomalies of the aortic arch require a clear definition of the position of the fetal trachea in relation to the pulmonary artery, the ductus arteriosus and the aortic arch.  In this sense Yoo et al., suggested that the view of cross sectional of the upper fetal mediastinum, that demonstrates the three major vessels: the superior vena cava, aorta and pulmonary artery owed complementary the exhaustive study of the heart fetal. While Yagel et al., in their study defined in an understandable way to undertake this "three vessel and trachea view", established nomograms of the major vessels and they showed the benefit of their methodology in the precise diagnosis of some great-vessels anomalies.

Aortic diverticulum or Kommerell's diverticulum is a rare aortic congenital anomaly that is produced as a result of the aberrant origin of the left subclavian artery, that arises of right arch aortic as a fourth branches and passes behind of the esophagus to the left shoulder instead of arising from the right brachiocephalic artery.

This anomaly is a result of regression in the 4th left aortic arch between the left carotid and left subclavian arteries. The left subclavian artery arises from an aortic pouch or diverticulum at the junction of the right aortic arch and the right descending aorta and passes obliquely upward, behind the esophagus, toward the left arm. This anomaly can occur in a number of anomalies of the aortic arch system that can, but do not always, cause symptoms of tracheal or esophageal compression, which they can be declared at the moment of the birth, during the lactation or in the adulthood (dysphagia lusoria).

The vast majority of patients with a vascular ring present with symptoms in infancy or very early in childhood. However, a small number of patients do not manifest symptoms until later in life, and others remain entirely asymptomatic
Common symptoms include laryngeal stridor, cyanosis, respiratory distress, apnea, and/or a characteristic high-pitched, brassy cough. Additional findings include a history of asthma, recurrent pneumonia, or evidence of dysphagia or difficulty with feedings. In some cases, airway symptoms are worsened or aggravated by feedings. Intercostal retractions during respiration are observed in some infants with severe obstruction.
When is associated with a right aortic arch, there is a 5 — 10% incidence of accompanying congenital heart diseases. These include tetralogy of Fallot. Atrial septal defect, ventricular septal defect and coarctation of the aorta.

Case report: The patient (G1) was referred at 34 weeks of gestation for a routine prenatal ultrasonographic study. The study includes the seven images standard utilized for the fetal echocardiographic views, calling the attention the presence of a diverticulum located toward the top end of the thoracic aorta and in the three vessel and trachea view we could observe an anomaly in the position of the major vessels with regard to the trachea.

In the figures 1 and 2 we can observe the normal anatomy of upper mediastinum and the normal ultrasound of the three vessel and trachea view, while in the figures 3 and 4 the ones that show the Kommerell's aortic diverticulum. The left subclavian artery arises from a diverticulum at the junction of the right aortic arch and the right descending aorta. The figure 5 show the three vessel and trachea view in this case, with loss of relations of the trachea with de major vessels (U shape image).

Figure 1

Figure 2

Figure 3

Figure 4

Figure 5

References
1. Eggerstedt, JM. Vascular rings. Last Updated:  June 10, 2000 http://www.emedicine.com/med/topic2981.htm
2. Achiron R, Rotstein Z, Heggesh J, Bronshtein M, Zimand S, Lipitz S, Yagel S.
Anomalies of the fetal aortic arch: a novel sonographic approach to in-utero diagnosis.Ultrasound Obstet Gynecol. 2002 Dec;20(6):553-7.PMID: 12493043
3.  Chaoui R, Kalache KD, Heling KS, Tennstedt C, Bommer C, Korner H. Absent or hypoplastic thymus on ultrasound: a marker for deletion 22q11.2 in fetal cardiac defects.Ultrasound Obstet Gynecol. 2002 Dec;20(6):546-52.PMID: 12493042
4:  Yagel S, Arbel R, Anteby EY, Raveh D, Achiron R.  The three vessels and trachea view (3VT) in fetal cardiac scanning. Ultrasound Obstet Gynecol. 2002 Oct; 20(4):340-5.PMID: 12383314
5:  Volpe P, Marasini M, Caruso G, Gentile M.  Prenatal diagnosis of interruption of the aortic arch and its association with deletion of chromosome 22q11.Ultrasound Obstet Gynecol. 2002 Oct;20(4):327-31.PMID: 12383312
6:  Oztunc F, Eroglu AG, Aksoy F, Saltik IL, Turan A. Antenatal diagnosis of
postductal coarctation of the aorta. A case report. Turk J Pediatr. 2001 Jan-Mar; 43(1):67-9.PMID: 11297163
7:  Muster AJ, Idriss RF, Backer CL. The left-sided aortic arch in humans, viewed as the end-result of natural selection during vertebrate evolution. Cardiol Young. 2001 Jan; 11(1):111-22. PMID: 11233389
8:  Achiron R, Zimand S, Hegesh J, Lipitz S, Zalel Y, Rotstein Z. Links 
 Fetal aortic arch measurements between 14 and 38 weeks" gestation: in-utero ultrasonographic study. Ultrasound Obstet Gynecol. 2000 Mar; 15(3):226-30.
PMID: 10846779
9: Guillem P, Triboulet JP, Fontaine C, Bailleul JP.  [Right aortic arches: anatomic and embryologic classification] Morphologie. 1999 Sep; 83(262):33-8. PMID: 10546245
10:  Takahashi K, Kuwahara T, Nagatsu M. Interruption of the aortic arch at the isthmus with DiGeorge syndrome and 22q11.2 deletion. Cardiol Young. 1999 Sep; 9(5):516-8. PMID: 10535835
11:  McElhinney DB, Tworetzky W, Hanley FL, Rudolph AM. Congenital obstructive lesions of the right aortic arch. Ann Thorac Surg. 1999 Apr; 67(4):1194-202. PMID: 10320289
12:  Zielinsky P, Oliveira LT.  [Aneurysm of arterial duct associated to aortic arch interruption] Arq Bras Cardiol. 1998 Jul;71(1):65-7. PMID: 9755537
13: Bronshtein M, Lorber A, Berant M, Auslander R, Zimmer EZ. Sonographic diagnosis of fetal vascular rings in early pregnancy. Am J Cardiol. 1998 Jan 1; 81(1):101-3. PMID: 9462619
14:  Devloo-Blancquaert A, Titus JL, Edwards JE, Vallaeys JH, De Gezelle HR, Coppens M. Interruption of aortic arch and hypoplastic left heart syndrome.
Pediatr Cardiol. 1995 Nov-Dec; 16(6):304-8. PMID: 8650020
15:  Kirby ML, Waldo KL.  Neural crest and cardiovascular patterning. Circ Res. 1995 Aug; 77(2):211-5. PMID: 7614707
16: Matsushita T, Nakajima T, Kishimoto H.  Interruption of aortic arch associated with pulmonary valve stenosis. Int J Cardiol. 1995 Mar 24;49(1):86-8.PMID: 7607770
17:  Hornberger LK, Sahn DJ, Kleinman CS, Copel J, Silverman NH. Antenatal diagnosis of coarctation of the aorta: a multicenter experience. J Am Coll Cardiol. 1994 Feb;23(2):417-23.PMID: 8294696
18:  Sharland GK, Chan KY, Allan LD. Coarctation of the aorta: difficulties in prenatal diagnosis.Br Heart J. 1994 Jan; 71(1):70-5.PMID: 8297700
19:  Momma K, Ando M. Fetal cardiovascular morphology of interrupted aortic arch type B in rats. Fetal Diagn Ther. 1994 Jan-Feb; 9(1):44-52.PMID: 8142052
20:  McNally PR, Rak KM.  Dysphagia lusoria caused by persistent right aortic arch with aberrant left subclavian artery and diverticulum of Kommerell. Dig Dis Sci. 1992 Jan; 37(1):144-9.PMID: 1728521
21:  Allan LD, Chita SK, Anderson RH, Fagg N, Crawford DC, Tynan MJ. Coarctation of the aorta in prenatal life: an echocardiographic, anatomical, and functional study Br Heart J. 1988 Mar; 59(3):356-60.PMID: 3355726
22:  Marasini M, Pongiglione G, Lituania M, Cordone M, Porro E, Garello-Cantoni L. Aortic arch interruption: two-dimensional echocardiographic recognition in utero. Pediatr Cardiol. 1985;6(3):147-9.PMID: 4080574
23:  Morel V, Corbineau H, Lecoz A, Verhoye JP, Heautot JF, Bassen R, Delaval P, Desrues B. Two cases of "asthma" revealing a diverticulum of Kommerell. Respiration. 2002;69(5):456-60.PMID: 12232456
24: van Son JA, Konstantinov IE. Burckhard F. Kommerell and Kommerell"s diverticulum.Tex Heart Inst J. 2002; 29(2):109-12. PMID: 12075866
25:  Mossad E, Farid I, Youssef G, Ando M. Diverticulum of Kommerell: a review of a series and a report of a case with tracheal deviation compromising single lung ventilation. Anesth Analg. 2002 Jun; 94(6):1462-4. PMID: 12032007
26:  Malas MB, Barr ML, Starnes VA, Shapiro S, Palmer S, Schwartz DS. Dyspnea lusoria: compression of the pulmonary artery by a Kommerell"s diverticulum. Ann Thorac Surg. 2002 Jan; 73(1):312-3. PMID: 11834041
26:  Parker JM, Cary-Freitas B, Berg BW.  Symptomatic vascular rings in adulthood: an uncommon mimic of asthma. J Asthma. 2000 May;37(3):275-80. PMID: 10831152
27:  Brown DL, Chapman WC, Edwards WH, Coltharp WH, Stoney WS. Dysphagia lusoria: aberrant right subclavian artery with a Kommerell"s diverticulum. Am Surg. 1993 Sep; 59(9):582-6. PMID: 8368665
28:  Florio F, Armillotta M, Palladino D, Petronelli S, Polverosi R.  [Kommerell diverticulum. Current aspects of the imaging diagnosis] Radiol Med (Torino). 1989 Jan-Feb; 77(1-2):132-4. PMID: 2928561
29:  McIntyre MD, Lynn RB. Kommerell"s diverticulum. Can J Surg. 1980 Jul;23(4):356. PMID: 7417897