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Sumario Vol. 42 - Nº 2 Abril - Junio 2013

Coarctation/Pseudocoarctation of Aorta
and Left Superior Vena Cava in the Adult
.
An Uncommon Association.

Bayona Emanuel, Alfonso Fernando, Corzo Omar, Sonzini Luis

Servicio de Cardiología, Clínica Sucre.
San Lorenzo 446. 10 piso A.
Córdoba Capital (5000), Argentina.
Correo electrónico

The authors declare not having a conflict of interest.
 


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SUMMARY

Aortic coarctationis the narrowing ofa segment ofthe aortathat causes apartial or totalblockageofbloodflow.Its prevalence in newborns is2.09/10,000. Pseudocoarctation of the aorta is an infrequent anomaly. First reports ofpersistentleft superior vena cava(LSVC) appeared in the nineteenth century.The persistence ofthe LSVC is an anomaly usually not detected in healthy hearts because it does not causehemodynamic changes.We report the case of a woman, 64 years old, who lives in the city of Cordoba, Argentina.The chest X-ray showsthicker mediastinum; and Doppler ultrasound revealed a bicuspid aortic valve with aortic root of 3.19 cm2.A CT scan showsaortic coarctation and thepresence ofpersistent LSVC.The association between pseudocoarctationor aortic coarctation with persistent LSVC although rare, should be kept in mind.

Key words: Aortic coarctation. Pseudocoarctation. Left superior vena cava. Computed tomography.
 
Rev Fed Arg Cardiol. 2013; 42(2): 146-149

 

 

INTRODUCTION
The term aortic coarctation refers to a narrowing of the aortic artery that causes an obstruction, in a variable degree, for the arterial flow. Typically, it is located in the thoracic descending aorta, distal to the origin of the left subclavian artery. Most of the coarctations involve the posterior wall of the aorta, opposite to the insertion of the ductus. It occurs by hypertrophy of the middle layer of the posterior portion of the vessel that protrudes toward the interior and reduces the vessel lumen. It can be accompanied by hypoplasia of the transverse aortic arch and/or the area of the aortic isthmus. In 4-5% of the cases, it is associated to anomalous right subclavian artery, which is born below the coarctation [1,2,3].

The aortic coarctation entails approximately 5.1% (3-10%) of the congenital cardiac malformations and constitutes the eighth cardiac malformation by frequency ranking. Its prevalence is estimated in 2.09 per 10,000 living newborn babies [2]. Its incidence is greater in males, in a 2:1 ratio in regard to females. It is a malformation typically associated to Turner syndrome [1,2,3].

It can be related to other intracardial malformations, ventricular septal defect in a third of the patients and bicuspid aortic valve in 30 to 85% of the cases. At times, it is accompanied by other left obstructive lesions, such as subaortic stenosis (15%), left ventricular hypoplasia and mitral valve obstructive lesions (Shone syndrome) [3].

The persistence of the left superior vena cava (LSVC) may disturb the normal development of the left ventricle and is strongly associated to LV inflow and outflow obstruction [4]. It is an anomaly that may be undetected in healthy hearts, since it does not cause hemodynamic alterations. Associated to congenital heart diseases, its presence may hinder diagnostic cardiac catheterization and complicate some surgical procedures.


CLINICAL CASE
Female, 64-year-old patient; a physician living in the capital city of Córdoba, Argentina. She consulted because of dry, unproductive, persistent, irritant cough, lasting 10 days, without fever or any other concomitant symptoms. She presents as pathological history, lower gastrointestinal bleeding, 10 years ago, secondary to angiodysplasia. She mentions not having toxic habits, allergies, surgical history or being under any treatment. In the physical examination, synchronic peripheral pulses are felt. BP: 120/70 mmHg, ejective systolic murmur 2/6 in aortic focus radiating to the neck. The rest of the physical examination presents no particular aspects. The ECG is within physiological limits, the lab only highlights the total cholesterol value (235 mg%); the rest of the analytical chemistry is within the physiological range. Her chest X-ray shows thickened mediastinum and Doppler echo shows mild increase in the diameters of the left atrium, parietal thickness, wall motility and preserved systolic left ventricular function. The aortic valve is bicuspid, with a good opening and the rest of the valve structures are normal. The aortic root is 3.19 cm2. The computerized tomography (CT) requested (Figure 1) reports mild dilatation of the ascending aorta with an approximate caliber of 40 mm at the mediastinum level, with tortuous descending aorta and discrete coarctation in the posterior sector of the aortic arch, immediately after the left subclavian artery outlet, with maximal caliber of approximately 13 mm and dilatation distal to the coarctation with maximal caliber of 34 mm. The CT also shows the persistence of the left superior vena cava trunk. Then, an angioresonance is requested (Figure 2) to confirm the diagnosis, the image of which clearly shows the aortic coarctation (arrows).

Figure 1. A) Chest computerized tomography; frontal view. The arrows indicate left SVC. B) CT; transversal view. C) Magnetic resonance: 3-dimensional reconstruction. The arrows indicate the aortic coarctation.

 

Figure 2. Angioresonance: sample of the aortic
coarctation (arrows).

 


DISCUSSION
The aortic coarctation is a congenital narrowing of any region or segment of the aorta, in the territory within the distal aortic arch and the abdominal aortic bifurcation. It is an alteration that requires care and repair during childhood, since in adults it may present diagnostic difficulties, causing significant clinical problems as it fosters late complications [5,6,7].

The aortic coarctation is 2-5 times more frequent in males (adults or children) than in females, and is usually associated to gonadal dysgenesis (Turner syndrome), bicuspid aortic valve, ventricular septal defects, patent ductus arteriosus, stenosis or mitral valve insufficiency, and aneurysms in the Willis polygon [6,7].

In the last two decades, the prevalence of congenital heart diseases in adults has remarkably increased, a fact probably linked to the technological advancements that allowed a greater accuracy and diagnostic precocity, along with better surgical results with a greater survival of this children, who in their adulthood will require special care in hospitals of the third level [8]. Most of the publications about aortic coarctation include pediatric series [9,10,11].

The first reports about the persistence of the left superior vena cava arise in the 19th century; and during the first half of the 20th century there are numerous reports that stand out, about the persistence of the LSVC. The works by Halpert and Coman [12], Atwell and Zoltowski [13], and those by Winter [14] contributed in a large extent, to this pathological entity acquiring an identity of its own. Hurwitt et al [15], reported the first surgical correction of a case that displayed cyanosis.The first descriptions of the development of the nervous system established the foundations to explain the morphogenesis of this malformation [16].

The presence of persistent LSVC may be suspected by its image in a chest X-ray [17], and the diagnosis of certainty is defined by echocardiography, computerized tomography [18], nuclear magnetic resonance [19], and/or cardiac catheterization. Surgical inspection [20], or necropsy [21] confirm the finding.

The knowledge of the morphogenesis of the systemic venous system is useful to understand the origin of the alterations and facilitate their classification.

LSVC persistence, when associated to complex congenital and severe heart diseases, may complicate some hemodynamic and surgical procedures for the treatment of these congenital cardiac malformations [22,23,24].

Pseudocoarctation of the aorta is an embryological and anatomical anomaly, similar to a true coarctation. It is an infrequent anomaly, caused by the elongation and congenital kinking or tortuosity of the aorta. It differentiates from true coarctation by the absence of aortic light stenosis and significant gradient of pressure through the lesion, so there are no classical signs of coarctation as hypertension in upper limbs, hypotension of lower limbs, costal incisures, delayed femoral pulses and collateral blood flow. The treatment of the patients with non-complicated pseudocoarctation should be conservative, although a thorough follow-up is necessary, given the potential for aneurysm formation [25].

The management adopted in the presented case, assumed as pseudocoarctation of the aorta, was waiting with developmental control every 6 months.


CONCLUSIONS
Aortic coarctation represents 5% of congenital cardiac malformations. Pseudocoarctation is an infrequent anomaly. In literature its association has been described, although infrequently, with persistent LSVC, the most common anomaly of the systemic venous system. The possibility of this combination should be considered in clinical practice, as in the reported case.

 

REFERENCES

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    2. Beekman RH. Coarctation of the aorta. En: Moss and Adams (Eds). Heart disease in infants, children and adolescents. Including the fetus and young adult.. Williams and Wilkins. Baltimore, USA. 1995.
    3. Rudolph, AM: Aortic arch obstruction. Congenital diseases of the heart. Clinical-Physiological considerations. 2001. Futura, Armonk. New York, USA.
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    16. Allen EV, Barker NW, Hines EA(Jr). Peripheral vascular diseases (3rd. Ed.). 1962. WB Saunders. Philadelphia, USA.
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    19. Fridland GW, De Vries PA, Nino-Murcia M, et al. Congenital anomalies of the inferior vena cava: embryogenesis and MR features. Urol Radiol 1992; 13: 237-48.
    20. Wilcox BR, Anderson RH. Surgical anatomy of the heart. 1992. Gower. London, United Kingdom.
    21. Edwards JE: Malformations of the thoracic veins in Gould. SE (Ed): Pathology of the heart. 1960. Charles C. Thomas. Springfield, USA.
    22. Bosher LH (Jr). Problems in extracorporal circulation relating to venous cannulation and drainage. Ann Surg 1959; 149: 652-63.
    23. Shumacker HB (Jr), King H, Waldhausen JA. The persistent left superior cava venous: surgical implications, with special reference to cava drainage into the left atrium. Am Surg 1967; 165: 797-805.
    24. De Leval MR, Ritter DG, McGoon DC, et al. Anomalous systemic venous connection. Surgical consideration. Mayo Clin Proc 1975; 50: 599-610.
    25. Galeote G, Oliver JM, Domínguez FJ, et al.Seudocoartación de aorta complicada con seudoaneurisma gigante. Rev Esp Cardiol 2000; 53: 287-89.

 

Publication: June 2013

 
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