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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

Imaging of situs inversus before CMRI. A: Chest X-ray with dextro-cardia (black arrow), posterior anterior projection, initial image of the first appearance in our hospital in January 2010. B: Lateral view of the chest. C: CT in axial and coronal (E) view after intra-venous contrast medium application showing a situs inversus totalis of the abdomen. The liver is on the left side of this axial view showing a hepatocellular carcinoma infiltrating a liver lobe (black arrows; 64-VCT Light Speed, GE, Healthcare, USA). The same view was seen in transversal (D) and coronal (F) views after application of contrast medium in 1.5 T MRI (Magnetom Symphony, Siemens AG, Healthcare sector, Erlangen, Germany). C: Coronal view in CT and MRI with contrast medium. The spleen was right sided (not demonstrated on these slices).
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Fig1: Imaging of situs inversus before CMRI. A: Chest X-ray with dextro-cardia (black arrow), posterior anterior projection, initial image of the first appearance in our hospital in January 2010. B: Lateral view of the chest. C: CT in axial and coronal (E) view after intra-venous contrast medium application showing a situs inversus totalis of the abdomen. The liver is on the left side of this axial view showing a hepatocellular carcinoma infiltrating a liver lobe (black arrows; 64-VCT Light Speed, GE, Healthcare, USA). The same view was seen in transversal (D) and coronal (F) views after application of contrast medium in 1.5 T MRI (Magnetom Symphony, Siemens AG, Healthcare sector, Erlangen, Germany). C: Coronal view in CT and MRI with contrast medium. The spleen was right sided (not demonstrated on these slices).

Mentions: A 56-year old patient was referred to our center with hepatocellular carcinoma for further diagnostic work-up. Incidentally, an inversion of the liver and abdominal structures to the opposite side was seen on computed-tomography (CT) and abdominal magnetic-resonance imaging (MRI). During preoperative clinical check-up and postoperative follow-up after partial liver resection (Figure 1), standardized cardiac imaging including Chest X-ray and echocardiography were performed. Pre-operative diagnostics demonstrated suspected pulmonary artery hypertension, elevated flow of the atrio-ventricular valve and higher pressure in the supposed right ventricle. Consequently, complete cardiac work-up was performed in the adult congenital heart disease unit.Figure 1


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)

Imaging of situs inversus before CMRI. A: Chest X-ray with dextro-cardia (black arrow), posterior anterior projection, initial image of the first appearance in our hospital in January 2010. B: Lateral view of the chest. C: CT in axial and coronal (E) view after intra-venous contrast medium application showing a situs inversus totalis of the abdomen. The liver is on the left side of this axial view showing a hepatocellular carcinoma infiltrating a liver lobe (black arrows; 64-VCT Light Speed, GE, Healthcare, USA). The same view was seen in transversal (D) and coronal (F) views after application of contrast medium in 1.5 T MRI (Magnetom Symphony, Siemens AG, Healthcare sector, Erlangen, Germany). C: Coronal view in CT and MRI with contrast medium. The spleen was right sided (not demonstrated on these slices).
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Fig1: Imaging of situs inversus before CMRI. A: Chest X-ray with dextro-cardia (black arrow), posterior anterior projection, initial image of the first appearance in our hospital in January 2010. B: Lateral view of the chest. C: CT in axial and coronal (E) view after intra-venous contrast medium application showing a situs inversus totalis of the abdomen. The liver is on the left side of this axial view showing a hepatocellular carcinoma infiltrating a liver lobe (black arrows; 64-VCT Light Speed, GE, Healthcare, USA). The same view was seen in transversal (D) and coronal (F) views after application of contrast medium in 1.5 T MRI (Magnetom Symphony, Siemens AG, Healthcare sector, Erlangen, Germany). C: Coronal view in CT and MRI with contrast medium. The spleen was right sided (not demonstrated on these slices).
Mentions: A 56-year old patient was referred to our center with hepatocellular carcinoma for further diagnostic work-up. Incidentally, an inversion of the liver and abdominal structures to the opposite side was seen on computed-tomography (CT) and abdominal magnetic-resonance imaging (MRI). During preoperative clinical check-up and postoperative follow-up after partial liver resection (Figure 1), standardized cardiac imaging including Chest X-ray and echocardiography were performed. Pre-operative diagnostics demonstrated suspected pulmonary artery hypertension, elevated flow of the atrio-ventricular valve and higher pressure in the supposed right ventricle. Consequently, complete cardiac work-up was performed in the adult congenital heart disease unit.Figure 1

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