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Situs inversus totalis with congenitally corrected transposition of the great arteries: insights from cardiac MRI.

Sohns JM, Steinmetz M, Schneider H, Fasshauer M, Staab W, Kowallick JT, Schuster A, Ritter C, Lotz J, Unterberg-Buchwald C - Springerplus (2014)

Bottom Line: CMR results showed a mildly impaired function and the switched anatomy.Heart failure symptoms and arrhythmias can appear with increasing age in patients with congenitally corrected transposition.Early CMR allows accurate diagnosis and timely introduction of adequate therapy thereby avoiding disease progression.

View Article: PubMed Central - PubMed

Affiliation: Institute for Diagnostic and Interventional Radiology, Georg-August-University Göttingen, UMG Universitätsmedizin Göttingen, Robert-Koch-Str. 40, 37099 Göttingen, Germany ; DZHK (German Centre for Cardiovascular Research), partner site Göttingen, Göttingen, Germany.

ABSTRACT

Introduction: Situs inversus totalis with congenitally corrected transposition of the great arteries represents a relatively rare congenital condition.

Case description: The current report describes the case of a 56 year old patient with an atrio-ventricular and ventricular-arterial discordance of the heart chambers without surgical correction, incidentally detected during hepatocellular carcinoma evaluation. The systemic venous blood arrived via the right atrium and a mitral valve in the morphologically left but pulmonary arterial ventricle that gave rise to a pulmonary trunk. The pulmonary venous blood passed the left atrium and the tricuspid valve into a morphologically right but systemic ventricle that gave rise to the aorta.

Discussion and evaluation: The switched anatomy was incidentally detected on echocardiography. The patient was referred to cardiac magnetic resonance imaging (CMR) including flow measurements, volumetry and late enhancement. CMR results showed a mildly impaired function and the switched anatomy. During a follow-up period of 2 years the patient was suffering from only mild heart failure and dyspnea.

Conclusions: Heart failure symptoms and arrhythmias can appear with increasing age in patients with congenitally corrected transposition. Early CMR allows accurate diagnosis and timely introduction of adequate therapy thereby avoiding disease progression.

No MeSH data available.


Related in: MedlinePlus

Situs inversus in CMRI. 1.5 T MRI (Magnetom Symphony, Siemens, Healthcare sector, Erlangen, Germany) after application of contrast medium. A: Four-chamber-view with a morphologic right and systemic ventricle (with hypertrophic trabecular structures) as well as a small smoother sub-pulmonary ventricle on the left side (white arrows). B: A dilated pulmonary artery (white arrow) is detected (A). C: Short axis or two-chamber-view (white arrows) demonstrating the cardiac ventricular anatomy of the two ventricles. A D-shaped septum bulging from the systemic right ventricle towards the morphological left ventricle connecting to the pulmonary circulation can be appreciated. D: The outflow of the aortic arch is shown in this image, coming from the functional left ventricle, morphological (original) right ventricle. E: The three-dimensional reconstruction shows the left-sided aorta and right-sided pulmonary trunk (white arrows). F: This image demonstrates the dilated pulmonary trunk and proximal pulmonary arteries in the initial Haste-sequences.
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Fig2: Situs inversus in CMRI. 1.5 T MRI (Magnetom Symphony, Siemens, Healthcare sector, Erlangen, Germany) after application of contrast medium. A: Four-chamber-view with a morphologic right and systemic ventricle (with hypertrophic trabecular structures) as well as a small smoother sub-pulmonary ventricle on the left side (white arrows). B: A dilated pulmonary artery (white arrow) is detected (A). C: Short axis or two-chamber-view (white arrows) demonstrating the cardiac ventricular anatomy of the two ventricles. A D-shaped septum bulging from the systemic right ventricle towards the morphological left ventricle connecting to the pulmonary circulation can be appreciated. D: The outflow of the aortic arch is shown in this image, coming from the functional left ventricle, morphological (original) right ventricle. E: The three-dimensional reconstruction shows the left-sided aorta and right-sided pulmonary trunk (white arrows). F: This image demonstrates the dilated pulmonary trunk and proximal pulmonary arteries in the initial Haste-sequences.

Mentions: The electrocardiogram (ECG) at rest showed a normal sinus rhythm with precordial ST- segment depression (V2 to V4), T-wave inversion (V2 to V3) and signs of biventricular hypertrophy.Echocardiography (Echo) showed a situs inversus totalis with meso- or dextrocardia of the heart. There was a side-by-side position of the great thoracic vessels with suspected atrio-ventricular discordance and ventricular-arterial discordance. We detected an L-transposition or congenitally corrected transposition of the great thoracic vessels. The ventricle of the right side (Figure 2) was hypertrophic with more trabecular structures and a typical right ventricular morphology. This ventricle appeared with impaired function. On Echo it was not possible to assess the ejection of this ventricle in detail. There was mild regurgitation of the atrio-ventricular (AV) valve. This AV-valve was located more apically and right-sided.Figure 2


Situs inversus totalis with congenitally corrected transposition of the great arteries: insights from cardiac MRI.

Sohns JM, Steinmetz M, Schneider H, Fasshauer M, Staab W, Kowallick JT, Schuster A, Ritter C, Lotz J, Unterberg-Buchwald C - Springerplus (2014)

Situs inversus in CMRI. 1.5 T MRI (Magnetom Symphony, Siemens, Healthcare sector, Erlangen, Germany) after application of contrast medium. A: Four-chamber-view with a morphologic right and systemic ventricle (with hypertrophic trabecular structures) as well as a small smoother sub-pulmonary ventricle on the left side (white arrows). B: A dilated pulmonary artery (white arrow) is detected (A). C: Short axis or two-chamber-view (white arrows) demonstrating the cardiac ventricular anatomy of the two ventricles. A D-shaped septum bulging from the systemic right ventricle towards the morphological left ventricle connecting to the pulmonary circulation can be appreciated. D: The outflow of the aortic arch is shown in this image, coming from the functional left ventricle, morphological (original) right ventricle. E: The three-dimensional reconstruction shows the left-sided aorta and right-sided pulmonary trunk (white arrows). F: This image demonstrates the dilated pulmonary trunk and proximal pulmonary arteries in the initial Haste-sequences.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4216825&req=5

Fig2: Situs inversus in CMRI. 1.5 T MRI (Magnetom Symphony, Siemens, Healthcare sector, Erlangen, Germany) after application of contrast medium. A: Four-chamber-view with a morphologic right and systemic ventricle (with hypertrophic trabecular structures) as well as a small smoother sub-pulmonary ventricle on the left side (white arrows). B: A dilated pulmonary artery (white arrow) is detected (A). C: Short axis or two-chamber-view (white arrows) demonstrating the cardiac ventricular anatomy of the two ventricles. A D-shaped septum bulging from the systemic right ventricle towards the morphological left ventricle connecting to the pulmonary circulation can be appreciated. D: The outflow of the aortic arch is shown in this image, coming from the functional left ventricle, morphological (original) right ventricle. E: The three-dimensional reconstruction shows the left-sided aorta and right-sided pulmonary trunk (white arrows). F: This image demonstrates the dilated pulmonary trunk and proximal pulmonary arteries in the initial Haste-sequences.
Mentions: The electrocardiogram (ECG) at rest showed a normal sinus rhythm with precordial ST- segment depression (V2 to V4), T-wave inversion (V2 to V3) and signs of biventricular hypertrophy.Echocardiography (Echo) showed a situs inversus totalis with meso- or dextrocardia of the heart. There was a side-by-side position of the great thoracic vessels with suspected atrio-ventricular discordance and ventricular-arterial discordance. We detected an L-transposition or congenitally corrected transposition of the great thoracic vessels. The ventricle of the right side (Figure 2) was hypertrophic with more trabecular structures and a typical right ventricular morphology. This ventricle appeared with impaired function. On Echo it was not possible to assess the ejection of this ventricle in detail. There was mild regurgitation of the atrio-ventricular (AV) valve. This AV-valve was located more apically and right-sided.Figure 2

Bottom Line: CMR results showed a mildly impaired function and the switched anatomy.Heart failure symptoms and arrhythmias can appear with increasing age in patients with congenitally corrected transposition.Early CMR allows accurate diagnosis and timely introduction of adequate therapy thereby avoiding disease progression.

View Article: PubMed Central - PubMed

Affiliation: Institute for Diagnostic and Interventional Radiology, Georg-August-University Göttingen, UMG Universitätsmedizin Göttingen, Robert-Koch-Str. 40, 37099 Göttingen, Germany ; DZHK (German Centre for Cardiovascular Research), partner site Göttingen, Göttingen, Germany.

ABSTRACT

Introduction: Situs inversus totalis with congenitally corrected transposition of the great arteries represents a relatively rare congenital condition.

Case description: The current report describes the case of a 56 year old patient with an atrio-ventricular and ventricular-arterial discordance of the heart chambers without surgical correction, incidentally detected during hepatocellular carcinoma evaluation. The systemic venous blood arrived via the right atrium and a mitral valve in the morphologically left but pulmonary arterial ventricle that gave rise to a pulmonary trunk. The pulmonary venous blood passed the left atrium and the tricuspid valve into a morphologically right but systemic ventricle that gave rise to the aorta.

Discussion and evaluation: The switched anatomy was incidentally detected on echocardiography. The patient was referred to cardiac magnetic resonance imaging (CMR) including flow measurements, volumetry and late enhancement. CMR results showed a mildly impaired function and the switched anatomy. During a follow-up period of 2 years the patient was suffering from only mild heart failure and dyspnea.

Conclusions: Heart failure symptoms and arrhythmias can appear with increasing age in patients with congenitally corrected transposition. Early CMR allows accurate diagnosis and timely introduction of adequate therapy thereby avoiding disease progression.

No MeSH data available.


Related in: MedlinePlus