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Year : 2018  |  Volume : 2  |  Issue : 2  |  Page : 132-136

Left atrium mass in an infant with complex cyanotic congenital heart disease

Heartline Cardiac Care Centre, Allahabad, Uttar Pradesh, India

Date of Web Publication6-Sep-2018

Correspondence Address:
Dr. Ishita Banerji
Heartline Cardiac Care Centre, 14/18 Elgin Road, Civil Lines, Allahabad - 211 001, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiae.jiae_84_17

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The clinical and echocardiographic findings in a 3-month-old cyanotic infant with congestive cardiac failure are described with stress on segmental approach to diagnosis. Echocardiography reveals dextrocardia, situs inversus, d-loop ventricle, tricuspid atresia, transposition of great vessels, subpulmonic ventricular septal defect (VSD), d-malposed aorta, restrictive atrial septal defect (ASD), and large mass in high-pressure left atrium (LA). Systemic veins drain to left-sided morphological right atrium (RA) and pulmonary veins returned to the right-sided morphological LA. In the presence of tricuspid atresia, blood from the morphological LA has only one outlet into the morphological RA through a restrictive ASD resulting in a high-pressure left atrial chamber. Blood from the morphological RA drains into the left-sided morphological left ventricle. From the left ventricle through the subpulmonic VSD, a portion of blood is directed into the pulmonary artery (PA) by passing the hypoplastic morphological right ventricle (RV) as the PA overrides the VSD. Blood flows from the hypoplastic RV into the aorta. Besides, LA mass seen in this 3 month-old-baby in the given setting is an extremely rare finding and to my knowledge has not been reported in literature before.

Keywords: Corrected transposition of great vessels, d-loop ventricle, dextrocardia, left atrium mass, situs inversus, tricuspid atresia

How to cite this article:
Banerji I. Left atrium mass in an infant with complex cyanotic congenital heart disease. J Indian Acad Echocardiogr Cardiovasc Imaging 2018;2:132-6

How to cite this URL:
Banerji I. Left atrium mass in an infant with complex cyanotic congenital heart disease. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2018 [cited 2019 May 23];2:132-6. Available from: http://www.jiaecho.org/text.asp?2018/2/2/132/240646

  Introduction Top

Cardiac malformations associated with cardiac malpositions are complex. The problems of terminology in complex congenital cardiac anomalies, particularly those with abnormalities of position, are well known. In January 2005, the International Society for Nomenclature of Pediatric and Congenital Heart Disease had constituted the overall mission of unifying pediatric cardiac diseases under a single terminology. The international congenital heart surgery nomenclature and database project have incorporated the extant Van Praaghian and Andersonian segmental and sequential approaches in a comprehensive fashion.

A sequential approach while performing an echocardiogram is mandatory to unravel the anatomic cardiac complexity. Three segments must be considered: (1) the total body configuration or situs, which could be situs solitus, situs inversus, or situs ambiguous followed by the identification of the atria. Van Praagh has stated that the atria usually follow the body situs; (2) the ventricular positions and connections to the atria that can be delineated by the bulboventricular loop. A d-loop is normal for situs solitus and an l-loop for situs inversus. Van Praagh termed these concordant loops. Discordant loops are an l-loop in situ s solitus and a d-loop in situ s inversus. The combination of situs inversus and a d-loop may be regarded as a mirror image of situs solitus with an 1-loop; and (3) the positions of the great arteries and connections to the ventricles. The anatomy of the great arteries may best be defined in terms of their lateral interrelationships and ventricular attachments as normally related or transposition of great arteries.[1]

Tricuspid atresia denotes a group of uncommon congenital heart defects in which there is absence or complete closure of the atrioventricular (AV) orifice of the morphological right ventricle (RV). This condition was reported with certainty in 1817 by Kreysig.[2] According to the segmental combination and ventriculoarterial relation, tricuspid atresia can be grouped in a three-member subset showing the basic layout of the heart as indicated by the visceroatrial situs, ventricular loop, and the infundibulum and great arteries, for example, S, D, S 8211; situs solitus with normally related great vessels; S, D, D 8211; situs solitus with transposition of the great arteries (TGA); S, D, D 8211; situs solitus with TGA with pulmonary atresia; S, D, L 8211; situ solitus with TGA with l malposed aorta; and S, L, L 8211; situs solitus with corrected TGA are some of the groups but not the only ones. Tricuspid atresia may occur in situ s inversus, in cases of double-outlet RV and double-outlet left ventricle.[3]

TGA with the segmental combination of visceroatrial situs solitus (S), L-loop ventricles, and L-TGA (S, L, L) is the classical form of physiologically corrected TGA. On the other hand, corrected TGA with visceroatrial situs inversus (I), d-loop ventricles, and D-TGA (I, D, D) is rare, occurring in only 3 of the I36 cases of dextrocardia. Although the segmental cardiac connections would theoretically be physiologically correct, the associated anomalies in the cases reported were severe, resulting in profound hemodynamic impairment.[4],[5]

Kartagener's syndrome (KS) is a triad of situs inversus, chronic sinusitis, and bronchiectasis. It includes mirror-image dextrocardia. In the literature of KS, Bitar et al. of the Departments of Pediatrics and Surgery, American University of Beirut, Lebanon first reported a patient with dextrocardia and corrected TGA (I, D.D), subpulmonary ventricular septal defect (VSD), and valvular pulmonary stenosis.[6] KS is usually associated with atriovisceral situs inversus and mirror-image dextrocardia, with the segmental combination of atrial situs inversus (I), L-loop ventricles, and inverted normally related great arteries (I, L, L).

Corrected TGA is an unusual cardiac malformation, in which the normal hemodynamic pathways are not altered by the malformation. I report here a unique case of possible KS with dextrocardia, visceroatrial situs inversus, d-ventricular loop (hence AV discordance), ventriculoarterial discordance (hence corrected TGA [I, D, D]), subpulmonic VSD with tricuspid atresia, high-pressure left atrium (LA) with small fossa ovalis atrial septal defect (ASD), left to right shunt, and multiple masses of varying sizes in LA, in a 3-month-old baby boy.

  Clinical Presentation Top

A 3-month-old baby boy, born to nonconsanguineous parents, presented with a history of recurrent respiratory tract infections since birth requiring frequent hospitalization and antibiotic therapy. He had a history of noisy breathing, persistent cough, and excessive cry with bluish discoloration of skin and lips. On examination, the patient had respiratory distress with respiration rate of 80 breaths/min. He had central cyanosis and his O2 saturation was 71% by pulse oximetry. On auscultation, no audible murmur was heard. Auscultation of the chest revealed crepitations bilaterally. The liver border was felt over the right upper quadrant.

Chest X-ray revealed dextrocardia with base-apex axis located in the right side, cardiomegaly (cardiothoracic ratio: 0.75), a right aortic arch, and infiltrates in the bilateral lung fields. A gastric air bubble was in the right side, and liver shadow was seen in the left side.

Two-dimensional echocardiography was done following the sequential segmental analysis of cardiac anatomy using terms as defined by the international pediatric and congenital cardiac code endorsed by the Society of Thoracic Surgeons (STS).[7] The echocardiography demonstrated:

  • Visceroatrial situs inversus (STS Harvest code 2120) with dextrocardia (STS Harvest code 2090) [Figure 1]
  • The left-sided morphologic right atrium receives systemic veins (superior vena cava and inferior vena cava) [Figure 2] and is seen connected to the anterior retro sternal morphologic left ventricle (absent moderator band with elongated anechoic lumen reaching cardiac apex, smooth septal endocardial surface, two papillary muscles attached to free wall, two leaflets of mitral valve with no septal attachment in short axis view). This pattern of ventricular looping in the presence of dextrocardia and situs inversus is named d-looping with associated discordant atrioventricular connection
  • The right-sided and posteriorly located morphologic LA receives the pulmonary veins and is seen above the right-sided posteriorly located small morphologic RV with rough trabeculations and apical offset of the tricuspid valve tissue
  • Discordant ventriculoarterial connections. The ascending aorta arises from the small morphologic RV [Figure 3] and appears anterior and to the right side of the main pulmonary artery (PA) that arises from the morphologic left ventricle
  • Large VSD Type I (infundibular/conal septal defect/supracristal/subarterial) (STS Harvest code 71) is noted with physiological right to left shunt, flow directed to the PA as the PA overrides the defect. TGA with VSD (STS Harvest code 900) [Figure 4]
  • The PA annulus size is found to be half of the aortic annulus size with mild flow acceleration at the valve. The pulmonary valve peak gradient was maximally recorded as 30 mmHg [Figure 5]
  • The tricuspid valve is atretic with no flow seen across it from LA to the hypoplastic morphological RV. Single ventricle, tricuspid atresia (STS Harvest code 820) with hypoplastic RV (STS Harvest code 1290) [Figure 6]
  • ASD secundum (STS Harvest code 20) restrictive in nature with left to right shunt is the only exit from the LA [Figure 7]
  • Mass seen in LA 8211; one large mass suggestive of a thrombus and a pedunculated mass attached to the tricuspid valve tissue [Figure 8] and [Figure 9]
  • Right-sided aortic arch is seen. No evidence of aortic arch coarctation or interruption seen and usual branching pattern is noted
  • A schematic diagram of the complex cardiac anatomy is presented [Figure 10].
Figure 1: Situs inversus

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Figure 2: Bicaval view

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Figure 3: Aorta from right ventricle. Ao: Aorta, RA: Right atrium, RV: Right ventricle, MV: Mitral valve, LV: Left ventricle

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Figure 4: Pulmonary artery overriding the ventricular septal defect

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Figure 5: Mild flow acceleration at pulmonary artery

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Figure 6: Tricuspid atresia, left atrium appendage

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Figure 7: Restrictive atrial septal defect

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Figure 8: Left atrium mass

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Figure 9: Left atrium pedunculated mass

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Figure 10: Schematic diagram of the cardiac anatomy

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  Discussion Top

The anatomic findings of this case are atypical and to my knowledge have not been reported in literature. The LA mass is in particular an interesting observation, and echocardiographically, it appears to be a thrombus. However, the pedunculated mass apparently attached to the tricuspid valve tissue could well be the rudimentary chordae or a part of the tricuspid valve tissue itself, thereby qualifying for the valvular type of tricuspid atresia. The major difference between this type of tricuspid atresia and the membranous type is that this membrane is located between the atrium and RV, not the LV.[3] Since the restrictive fossa ovalis ASD is the only exit from LA, pooling of blood occurs in the LA resulting in stagnation of blood and sluggish circulation which predisposes to the formation of a thrombus in the LA. In the absence of a trial with heparin infusion, this is assumed to be the most likely cause of the LA mass. The response of this mass to heparin infusion would have confirmed the etiology of the thrombus. Dissolution or reduction in the size of the mass on postheparin echocardiogram could have clinched the diagnosis in favor of a thrombus as against a myxoma or tricuspid valve tissue. Unfortunately, as the family was unwilling to do further investigations, a clinical therapeutic response with heparin infusion was not possible.

In a large study of dextrocardia done at the Department of Cardiology, Sanjay Gandhi PGIMS, Lucknow, cardiac anatomy and hemodynamics of patients with dextrocardia were segmentally analyzed.[8] It was found that among the 125 cases of dextrocardia, situs inversus was the most common with 39.2%, which was in contrast to the series from the west which had reported situs solitus dextrocardia as the most common type. It was considered in the SGPGI study that because most of the patients with situs inversus dextrocardia have structurally normal heart, it evades detection. It was found that discordant AV connections were present in only 12.3% of patients. Studies have shown that heart in situ s inversus dextrocardia is most likely to be normal when it is associated with concordant AV connections.[4] However, when malformations occur in this setting, they are likely to be complete transposition or double-outlet right ventricle.[4] Furthermore, in the SGPGI study of 125 patients, it was found that among the discordant AV connections, majority (66.7%) had congenitally corrected transposition, which was in accordance to the previous studies. The case reported here is in tandem to these findings. However, despite the complexity of the cardiac malposition, the segmental cardiac connections is physiologically correct complicated by the associated anomaly of tricuspid atresia with restricted ASD, resulting in a profound hemodynamic impairment, the brunt of which is borne by the LA, pulmonary venous hypertension being an obvious consequence.

In view of the visceroatrial inversus and dextrocardia with respiratory symptoms, the possibility of KS cannot be ruled out. However, in view of the associated complex cardiac anomalies, the respiratory symptoms cannot wholly be attributed to KS. Furthermore, due to lack of a workup in the form of high-resolution computed tomography or ciliary dyskinesia or even a paranasal sinus X-ray, to gather supportive evidence of KS in view of the unwillingness of the family, it would not be prudent to label this child with Kartagener's syndrome.

  Conclusion Top

In this case, history is suggestive of predominant congestive cardiac failure. Cyanosis is relatively a later finding that became apparent with the onset of the chest infection. The low oxygen saturation cannot be attributed wholly to the mild flow acceleration at the pulmonary valve but can primarily be explained by the associated chest infection. Despite the limitations in getting a thorough workup done for this cyanotic infant, the exciting cardiac anatomy and the occurrence of LA thrombus, a rare and interesting finding, was considered worth reporting.

Declaration of patient consent

The author certifies that she has obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Stanger P, Rudolph AM, Edwards JE. Cardiac malpositions. An overview based on study of sixty-five necropsy specimens. Circulation 1977;56:159-72.  Back to cited text no. 1
Kreysig FL. The Diseases of heart. Vol. 3. Berlin: Maurer; 1817.  Back to cited text no. 2
Weinberg PM. Anatomy of tricuspid atresia and its relevance to current forms of surgical therapy. Ann Thorac Surg 1980;29:306-11.  Back to cited text no. 3
Van Praagh R, Weinberg PM, Smith SD, Foran RB, Van Praagh S. Malpositions of the heart. In: Adams FH, Emmanouilides GC, Riemenschneider TA, editors. Heart Disease in Infants, Children and Adolescents. 4th ed. Baltimore: Williarns and Wilkins; 1989. p. 530-80.  Back to cited text no. 4
Vanpraagh R, Vanpraagh S, Vlad P, Keith JD. Diagnosis of the anatomic types of congenital dextrocardia. Am J Cardiol 1965;15:234-47.  Back to cited text no. 5
Bitar FF, Shbaro R, Mroueh S, Yunis K, Obeid M. Dextrocardia and corrected transposition of the great arteries (I, D, D) in a case of Kartagener's syndrome: A unique association. Clin Cardiol 1998;21:298-9.  Back to cited text no. 6
Franklin RC, Jacobs JP, Krogmann ON, Béland MJ, Aiello VD, Colan SD, et al. Nomenclature for congenital and paediatric cardiac disease: Historical perspectives and the International Pediatric and Congenital Cardiac Code. Cardiol Young 2008;18 Suppl 2:70-80.  Back to cited text no. 7
Garg N, Agarwal BL, Modi N, Radhakrishnan S, Sinha N. Dextrocardia: An analysis of cardiac structures in 125 patients. Int J Cardiol 2003;88:143-55.  Back to cited text no. 8


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10]


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