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 Table of Contents  
INTERESTING CASE REPORT
Year : 2021  |  Volume : 5  |  Issue : 1  |  Page : 66-69

Complex Congenital Heart Disease with Common Atrium: A Rare Entity


1 Sanjeevani Ultrasound and Diagnostic Centre, Faridabad, Haryana, India
2 Department of Radiology, Medanta-The Medicity, Gurgaon, Haryana, India

Date of Submission28-Jun-2020
Date of Decision15-Aug-2020
Date of Acceptance16-Aug-2020
Date of Web Publication04-Feb-2021

Correspondence Address:
Dr. Navni Garg
B3-101, Mahindra Chloris Apartments, Sector-19, Faridabad, Haryana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiae.jiae_29_20

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  Abstract 

Common atrium (CA) is a rare congenital cardiac anomaly characterized by complete lack of interatrial septal tissue. It is commonly associated with atrioventricular valve malformations and other extracardiac anomalies. Hereby, we report a case of a term neonate with complex congenital heart disease with CA and panorama of dual-source computed tomography imaging findings in the patient.

Keywords: Common atrium, congenital heart disease, single atrium


How to cite this article:
Garg N, Jain B. Complex Congenital Heart Disease with Common Atrium: A Rare Entity. J Indian Acad Echocardiogr Cardiovasc Imaging 2021;5:66-9

How to cite this URL:
Garg N, Jain B. Complex Congenital Heart Disease with Common Atrium: A Rare Entity. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2021 [cited 2021 Jul 23];5:66-9. Available from: https://www.jiaecho.org/text.asp?2021/5/1/66/308729


  Introduction Top


Common atrium (CA) is a rare congenital heart disease comprising <0.5%–1% of all congenital heart malformations.[1] It is a part of the spectrum of atrioventricular (AV) septal defects with attachment of mitral and tricuspid valves to the interventricular septum in the same anatomic plane.[2] It should be differentiated from single atrium which is not associated with endocardial cushion defects.[3] CA might occur as an isolated anomaly or associated with trisomy 21, heterotaxy syndrome with asplenia, or as a cardiac component of Ellis van Creveld syndrome (mesomelic dwarfism, postaxial polydactyly, congenital cardiac malformations, and abnormal hair and nails).[4],[5] Asplenia results in increased risk of repeated infections.


  Case Report Top


A 5 day old term female baby born out of a nonconsanguineous marriage, delivered by cesarean section, presented with respiratory distress and fever. On examination, the limbs, hands, and feet were normal without any evidence of polydactyly or disproportionate distal limb shortening. The blood picture revealed leukocytosis with neutrophilia (31,000/mm3). She was managed for sepsis and received intravenous antibiotics, oxygen, and supportive care. Her condition deteriorated, and she developed abdominal distension, generalized edema, and low oxygen saturation. Blood gas analysis revealed severe metabolic acidosis (base excess = −13, lactate = 13 mmol/L). She was initially managed with soda bicarbonate boluses but soon started on dobutamine and furosemide infusion due to the ongoing heart failure. Bedside chest X ray showed cardiomegaly with dextrocardia and pulmonary plethora [Figure 1]. Transthoracic echocardiography was done, which revealed complex heart disease with dextrocardia, CA with hepatic veins draining into it, ventriculoarterial concordance, severe tricuspid regurgitation, severe mitral regurgitation with cleft in the mitral leaflet, intact interventricular septum, dilated right ventricle, elongated left ventricular outflow tract, large patent ductus arteriosus (PDA) (bidirectional flow), and severe pulmonary arterial hypertension. She further deteriorated and developed bradycardia (80/min) and desaturation (70%–80%). The child was then intubated and ventilated. Post intubation, her vitals and saturation improved. An electrocardiogram gated multiphasic computed tomography (CT) examination of the heart was then performed to check for other anomalies. It revealed cardiomegaly with a prominent right ventricle. There was a common large single atrium with drainage of pulmonary and hepatic veins into it [Figure 2]a and [Figure 3]. Both atrial appendages were triangular, consistent with right atrial isomerism [Figure 2]b. There was left sided superior vena cava (SVC) draining into the CA [Figure 4], ventriculoarterial concordance [Figure 5], right sided aortic arch with common origin of bilateral common carotid arteries [Figure 6], situs ambiguous with midline liver, asplenia, and dextrocardia [Figure 7], large PDA [Figure 8]a and [Figure 8]b, origin of left subclavian artery from the main pulmonary artery and interrupted inferior vena cava (IVC). The origin of coronary arteries from the respective sinuses was normal. Increased pulmonary vascularity with patchy areas of collapse consolidation was seen in the bilateral lungs. Karyotyping was also performed, which came out to be normal. A two stage surgery was planned, with the first being PDA ligation and the second being atrial septation and AV valve repair, but the relatives refused surgery and left against medical advice.
Figure 1: Chest X-ray showing cardiomegaly with dextrocardia and pulmonary plethora

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Figure 2: (a and b) Computed tomography angiography axial section (a) revealing cardiomegaly with large common atrium with drainage of the left pulmonary vein (black arrow) into it and (b) revealing triangular right-sided morphology of bilateral atrial appendages

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Figure 3: (a and b) Computed tomography angiography coronal section (a) and volume-rendered image (b) revealing drainage of the hepatic veins into the common atrium

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Figure 4: Computed tomography angiography coronal section revealing persistent left-sided superior vena cava

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Figure 5: Computed tomography angiography axial section revealing ventriculoarterial concordance with pulmonary artery arising from the right ventricle

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Figure 6: Computed tomography angiography coronal section revealing right-sided aortic arch with common origin of bilateral common carotid arteries

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Figure 7: Computed tomography angiography axial section revealing situs ambiguous with midline liver

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Figure 8: (a and b) Computed tomography angiography sagittal section (a) and axial section (b) revealing large patent ductus arteriosus between the descending thoracic aorta and pulmonary artery

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  Discussion Top


CA is a rare cardiac anomaly first reported by Young and Robinson in 1907.[6] Levy et al. suggested the term “common atrium” to denote the condition of complete absence of the atrial septum, accompanied by malformation of the AV valves, with or without interventricular communication. They proposed that the term “single atrium” should be reserved for condition characterized by (a) complete absence of interatrial septum; (b) absence of malformation of the AV valves; and (c) absence of interventricular communication.[3]

CA is usually diagnosed in childhood, though adult presentation of the disease has also been reported.[7],[8] Patients present with decrease in exercise tolerance, shortness of breath, cyanosis, upper respiratory tract infections, fatigue, developmental delay, and heart failure.[7],[8] As there is mixing of systemic and pulmonary venous blood in the CA and AV regurgitation, there is usually decreased systemic arterial oxygen saturation.[8]

CA may be associated with different ventricular morphologies: two ventricles, single right ventricle, single left ventricle, or an undifferentiated single ventricle. Associated malformations include intracardiac anomalies (dextrocardia, mesocardiac, levoverted heart, atrial isomerism, complete AV septal defect, and single AV valve), anomalies of great vessels (anomalous pulmonary venous connection, left-sided SVC, right-sided aortic arch, persistent ductus arteriosus, and pulmonary artery anomalies), and aortopulmonary collaterals.[9] It is almost always associated with a cleft in the anterior leaflet of the mitral valve.[2] Our case had CA with situs ambiguous, asplenia, dextrocardia with left-sided SVC, cleft in the mitral leaflet, right-sided aortic arch, and PDA.

Detailed anatomic evaluation is mainstay for effective patient management. Transesophageal/transthoracic echocardiography plays a pivotal role in defining the anatomy of the single atrium and ruling out classical partial AV septal defect, however it lacks delineation of the extracardiac structures. Multiphasic dual-source CT (DSCT) is preferred nowadays. It uses two CT tubes (dual X-ray sources) at right angles to each other, which doubles the speed of the scan, leading to less radiation exposure and sharper images. Multiphasic examination of the heart includes sequential series of images in the mid-arterial, mid-venous, and delayed phases of enhancement to ensure opacification of both sides of the heart and all extracardiac vessels. It is noninvasive, is readily available, provides detailed anatomic evaluation about cardiac and extracardiac anomalies, and allows advanced postprocessing techniques.

Patients with CA without any associated malformations usually have a good prognosis even without surgical management.[8] Patients with associated malformations usually require surgical intervention. The single atrium is divided into two compartments by a Dacron or an autologous pericardial patch. As there are no marks by which the location of the AV node and bundle of His area can be identified, it is critical to avoid injury to them during surgery. The sutures are anchored on the base of the mitral anterior valve or placed on the annulus of the tricuspid valve. Some surgeons prefer superficial interrupted sutures to the tissues between the two AV valves.[7]


  Conclusion Top


CA is a rare cardiac anomaly. Evaluation with echocardiography to rule out malformation of the AV valves and DSCT for other intracardiac anomalies, anomalies of great vessels, and heterotaxy syndromes is necessary for effective patient management.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Campbell M. Incidence of cardiac malformations at birth and later, and neonatal mortality. Br Heart J 1973;35:189-200.  Back to cited text no. 1
    
2.
Munoz-Armas S, Gorrın JR, Anselmi G, Hernandez PB, Anselmi A. Single atrium. Embryologic, anatomic, electrocardiographic and other diagnostic features. Am J Cardiol 1968;21:639-52.  Back to cited text no. 2
    
3.
Levy MJ, Salomon J, Vidne BA. Correction of single and common atrium, with reference to simplified terminology. Chest 1974;66:444-6.  Back to cited text no. 3
    
4.
Digilio MC, Marino B, Giannotti A, Dallapiccola B. Single atrium, atrioventricular canal/postaxial hexodactyly indicating Ellis-van Creveld syndrome. Hum Genet 1995;96:251-3.  Back to cited text no. 4
    
5.
Ferdman DJ, Brady D, Rosenzweig EB. Common atrium and pulmonary vascular disease. Pediatr Cardiol 2011;32:595-8.  Back to cited text no. 5
    
6.
Young AH, Robinson A. Some malformations of the human heart. Med Chron 1907/1908;47:96-106.  Back to cited text no. 6
    
7.
Hirai S, Hamanaka Y, Mitsui N, Isaka M, Mizukami T. Surgical repair of a common atrium in an adult. Ann Thorac Cardiovasc Surg 2003;9:130-3.  Back to cited text no. 7
    
8.
Kim K, Choi J, Doo Y, Yun YS, Kim J, Lee JB. Well-tolerated and undiscovered common atrium until late adulthood. J Cardiovasc Ultrasound 2016;24:243-6.  Back to cited text no. 8
    
9.
Zhang Y, Yang ZG, Yang MX, Shi K, Li R, Diao KY, et al. Common atrium and the associated malformations: Evaluation by low-dose dual-source computed tomography. Medicine (Baltimore) 2018;97:e12983.  Back to cited text no. 9
    


    Figures

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



 

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