|INTERESTING CASE REPORT
|Year : 2022 | Volume
| Issue : 1 | Page : 76-79
Dextroversion and D-Transposition of the Great Arteries, Left-Handed Ventricular Topology Related to Multiple Ventricular Septal Defects, and Left Juxtaposition of Atrial Appendage in a 1-Year-Old Child
Elio Caruso1, Silvia Farruggio1, Maria Vittoria Raciti2, Salvatore Agati1
1 Mediterranean Pediatric Cardiology Center, “Bambino Gesù,” San Vincenzo Hospital, Taormina, ME, Italy
2 Radiology Department, Umberto I Hospital, Enna, EN, Italy
|Date of Submission||20-Aug-2021|
|Date of Acceptance||02-Nov-2021|
|Date of Web Publication||06-Jan-2022|
Mediterranean Pediatric Cardiology Center, “Bambino Gesù,” San Vincenzo Hospital, Contrada Sirina, 98039, Taormina, ME
Source of Support: None, Conflict of Interest: None
We present a 1-year-old female infant case in natural history, affected by D-transposition of the great arteries with multiple ventricular septal defects in dextroversion, left juxtaposition of atrial appendages, and persistent left superior caval vein draining into coronary sinus. This is an uncommon case, and its particularity is due to clinical and anatomical findings diagnosed late in a setting of a complex cardiac malformation. After the diagnosis, the patient underwent palliative arterial switch operation without complications.
Keywords: Dextroversion, left juxtaposition of atrial appendages, persistent left superior caval vein, transposition of the great arteries
|How to cite this article:|
Caruso E, Farruggio S, Raciti MV, Agati S. Dextroversion and D-Transposition of the Great Arteries, Left-Handed Ventricular Topology Related to Multiple Ventricular Septal Defects, and Left Juxtaposition of Atrial Appendage in a 1-Year-Old Child. J Indian Acad Echocardiogr Cardiovasc Imaging 2022;6:76-9
|How to cite this URL:|
Caruso E, Farruggio S, Raciti MV, Agati S. Dextroversion and D-Transposition of the Great Arteries, Left-Handed Ventricular Topology Related to Multiple Ventricular Septal Defects, and Left Juxtaposition of Atrial Appendage in a 1-Year-Old Child. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2022 [cited 2023 Jun 10];6:76-9. Available from: https://jiaecho.org/text.asp?2022/6/1/76/335069
| Introduction|| |
Dextroversion refers to a cardiac position anomaly in which the heart's apex is rotated into the right chest, with the morphological right atrium and right ventricle located on the right, dorsally to the left-sided chambers. The prevalence is 1 in about 30,000 in the general population.
Dextroversion is the second most common type of dextrocardia, which is a mirror-image condition.
The right-sided heart anomalies can occur in situs thoracoabdominal viscerum solitus or inversus.
We describe a complex congenital heart disease (CHD) in a setting of dextroversion and situs solitus of the abdominal viscera and atria.
| Case Report|| |
We present a 1-year-old female infant referred to us for growth retardation, coming from a developing country. Her body weight was 6.5 kg, peripheral oxygen saturation was 80%, and on physical examination a grade 3/6 parasternal systolic murmur was noted at auscultation. Echocardiogram from subcostal view [Figure 1]a and [Movie S1] showed situs solitus of abdominal viscera and atria, dextrocardia, concordant atrioventricular connections, and discordant ventricular-arterial connections. Superior and inferior right caval veins drained into the right atrium placed on the right-side and posteroinferiorly, while pulmonary venous connection drained into the left atrium placed on the left-side and anterosuperiorly. Left superior caval vein drained into the dilated coronary sinus [Figure 2]a and [Figure 2]b, two dilated atrial appendages were on the same side, in juxtaposition [Figure 2]c and [Figure 2]d. Heart apex was directed toward the right side, the morphologically left ventricle was anterior and left sided, and the morphologically right ventricle was posteroinferior and right sided. Great arteries were transposed with aorta anterior and rightward to the pulmonary trunk. Four-chamber view [Figure 1]b showed an interatrial septum abnormally oriented in the horizontal plane and displaced with a consequent unusual location, multiple and wide unrestrictive muscular ventricular septal defects (VSDs, one large inlet VSD, two apical and small VSDs), hypertrophic right ventricle and transposition of great arteries (TGA) [Figure 1]a and [Movie S2], [Movie S3]. A right patent arterial duct shunted from aorta to pulmonary trunk [Figure 2]d with a left-sided aortic arch.
|Figure 1: (a) Subcostal view showing transposition of great arteries. (b) Four-chamber view showing multiple and wide muscular ventricular septal defects (dotted white lines), horizontal orientation of interatrial septum (white arrow), and small hypertrophic right ventricle. AO: Aorta, LA: Left atrium, LV: Left ventricle, M: Mitral valve, PA: Pulmonary artery, RA: Right atrium, RV: Right ventricle, T: Tricuspid valve|
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|Figure 2: (a) Subcostal view showing left superior caval vein draining into dilated coronary sinus. (b) CT angiography maximum intensity projection reconstruction, coronal plane shows double upper caval veins with left superior caval vein in dilated coronary sinus, dilated pulmonary trunk, and anterior left ventricle. (c) CT angiography, volume rendering, anteroposterior view showing dextroversion, left juxtaposition of atrial appendages and transposition of great arteries. (d) Surgical picture showing left juxtaposition of atrial appendages, dextroversion with anterior left chambers, aorta anterior and rightward to the pulmonary trunk and right patent arterial duct. AO: Aorta, CS: Coronary sinus, CT: Computed tomography, LAA: Left atrial appendage, LA: Left atrium, LSVC: Left superior caval vein, LV: Left ventricle, PAD: Patent arterial duct, PT: Pulmonary trunk, RSVC: Right superior caval vein, RIVC: Right inferior caval vein, RV: Right ventricle|
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Movie S1: Subcostal view shows anterior left chambers and transposition of great vessels.
[Additional file 1]
Movie S2: Four chamber view with simultaneous color Doppler view shows dilated left atrium, horizontal displacement of atrial septum, atrioventricular concordance, multiple and wide ventricular septal defects (one large inlet and two apical ventricular septal defects, shunting left-to-right), and small right chambers.
[Additional file 2]
Movie S3: Short axis view of the pulmonary trunk and branches with color Doppler shows the aorto-pulmonary shunt of arterial duct from aorta to right pulmonary branch.
[Additional file 3]
A subpulmonary stenosis due to the presence of subvalvular tissue caused mild stenosis with a maximum gradient of 30 mmHg at continuous wave Doppler. Computed tomography scan angiography showed bronchial situs solitus and confirmed, together with surgery, the echocardiographic diagnosis [Figure 2]b and [Figure 2]c.
Based on the anatomical relationship, we ultimately defined this condition as dextroversion characterized by situs solitus of the atria and the viscera, with the heart in the right hemithorax and its apex directed toward the right side [Figure 2]b because of the right rotation of the heart, its entire right portion was further displaced to the posterior side, thus demonstrating the dextroversion.
| Discussion|| |
In general, dextroversion consists of a rotation of the ventricular part of the heart to the right, as in turning the page of a book, with the atria remaining in normal position.
As early as in 1956, Robert Grant wrote a paper called “The syndrome of dextroversion of the heart”. His manuscript described three conditions characterized by right-lying heart: dextrocardia, dextroposition, and dextroversion. The most common is the mirror-image dextrocardia, in which the anterior-posterior relationship of the heart is normal, but its right-to-left orientation is reversed. The second is dextroposition, in which an otherwise normal heart is shifted to the right. The last, dextroversion, most important as frequently associated with other intracardiac malformations, has been recognized under several different names in the past, as “isolated” dextrocardia, incomplete rotation of the heart, and dextrotorsion.
We should use with cautious these terms, more so since others have used variations of this terminology, suggesting terms in attempts to compress all the information into a single word. These variations are not universally understood and should be avoided.
According to the Nomenclature Working Group who created the International Paediatric and Congenital Cardiac Code, Jacobs et al. described that the position of the heart in the chest, and the orientation of the cardiac apex, must be described separately from each other because these features can vary independently, and can have no definitive relationship to other cardiac relations and connections. The term “dextrocardia” is most usually considered synonymous with a right-sided ventricular mass, while “dextroversion” is frequently defined as a configuration where the ventricular apex points to the right. In a patient with the usual atrial arrangement, or situs solitus, dextroversion, therefore, implies a turning to the right of the heart.
Usually, dextrocardia in situs inversus is only rarely associated with CHD; in contrast, dextrocardia in situs solitus is often associated with intracardiac anomalies.
The embryogenesis of dextroversion is closely related to the embryogenesis of the cono-truncal region of the heart, and understanding the mechanism of dextroversion sheds light on other congenital malformations. There are three truncoconal morphologies associated with dextroversion: TGA, concordant ventricular-arterial connections, or common trunk. Grant described the association of dextroversion and TGA in 2 of 3 personal cases and 55 of 119 collected from literature stating that one of the most common intracardiac abnormalities associated with dextroversion was TGA.
During embryological development, the incomplete leftward rotation of the ventricles into the right chest results in dextroversion and is commonly associated with CHDs. In 90% of cases, dextroversion is associated with cardiac malformations as atrial septal defects and VSD, anomalous pulmonary venous return, tetralogy of Fallot, pulmonary valve stenosis, coarctation of the aorta, corrected TGA, and single ventricle. It is particularly frequent that a combination of single ventricle, TGA, and stenosis or atresia of the pulmonary artery is found in 64% of these malpositions.
Van Praagh et al. described one of the earliest investigations establishing the importance of segmental analysis and showed that the presence of the heart in the right chest conveyed no information with regard to chamber organization and internal anatomy of the heart but should be used only for description of this cardiac position.
An additional feature of our case is the anatomical course of atrial appendages. As defined, left juxtaposition is characterized by the right appendage taking origin from the right atrium, running posterior to the great arteries, and coursing toward the left side over the left atrium and lying superior to the left appendage, as shown in our surgical pictures [Figure 2]c.
TGA associated with multiple VSDs is a complex anomaly which raises a surgical challenge. Meticulous preoperative anatomical assessment is mandatory. The choice of either one or two-stage repair depends on the anatomical and clinical conditions of each particular patient as well as the experience of the surgical team. The secondary pulmonary arterial banding constitutes a safe solution in case of failure of VSDs closure.
With timely diagnosis, most children with TGA/VSD usually undergo surgical correction within the first 1–3 months after birth. In developing countries, however, for socioeconomic reasons, some of these children unfortunately are brought for surgical correction when they are beyond 6 months of age with severe pulmonary hypertension owing to pulmonary vascular obstructive disease.
Lei et al. reported the first analysis of a series of patients with arterial switch operations (ASOs), concluding that this approach significantly improved the quality of life and possibly life expectancy, postoperative pulmonary vascular resistance might be reversible in some patients.
Our patient underwent palliative ASO and removal of subvalvular tissue in the left outlet; after 4 days of intensive care unit and 7 of hospitalization stay length, she was discharged without complications and with a peripheral oxygen saturation of 95%.
| Conclusion|| |
Our case had rare and particular clinical and anatomical findings that made it rare as a heart in dextroversion, left-handed ventricular topology, D-TGA, multiple VSDs, double upper caval veins with left superior caval vein in coronary sinus,| and unusual arrangement of the atrial appendages in a child who survived up to 1-year-age without diagnosis until our observation.
Informed consent was kept from parents.
Additional supporting information may be found online in the Supporting Information section.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's parents have given her consent for her images and other clinical information to be reported in the journal. The patient's parents understand that her name and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]