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Number 14-14: Late Presentation of Hemitruncus - the importance of XMR for clinical decision making
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Number 14-14: Late Presentation of Hemitruncus - the importance of XMR for clinical decision making

Case from: Srinivas Narayan, Martin Grips, Tarique Hussain

Institute: Evelina London Children's Hospital.

 

Clinical history:

A 3-year-old girl was referred for evaluation of breathlessness on exertion (NYHA class 3) in conjunction with cyanosis. She was born at term without any perinatal problems and was growing and developing normally. Her parents noticed her becoming very breathless and blue around her mouth during exertion about 5-6 months before presentation. She did not have any other symptoms such as chest pain, palpitations, or loss of consciousness and there was no family history of any congenital heart disease.

On examination she was centrally cyanosed with a saturation of 88% in air. She had a hyperactive precordium with a right ventricular heave and palpable P2. There was a soft 1-2/6 systolic murmur over the left lower sternal border.

  

 

Figure 1: 12-lead EKG showing right-axis deviation and RVH

 

         

 

In addition, echocardiography showed that the aortic arch was right-sided. Pulmonary venous drainage appeared normal.

The clinical team wished to evaluate the cardiac anatomy, in particular the origin of LPA and whether there was a fixed and increased pulmonary vascular resistance. A comprehensive XMR (cardiac catheter & MRI in a dedicated combined hybrid lab) protocol was therefore performed.

CMR Findings:

The procedure began with a high temporal resolution angiogram (also known as 4D-TRAK (Time-resolved angiography using Keyhole)).

 

 

Figure 2: Angiography immediately demonstrated that the child has a Hemitruncus with the LPA arising from the ascending aorta. This is shown using volume rendering above. A right arch with normal branching is also seen.

Once the anatomy was known, the patient was immediately trasferred to the X-ray angiogrphy end of the room to undergo diagnostic cardiac catheter. Non-ferromagnetic catheters were placed anterograde in the RPA and retrograde in the LPA. The patient was transferred back to MRI for haemodynamic measurements including balanced SSFP cine images and selected phase contrast flow images. This was done under two conditions –

Condition 1. Baseline:  30% inhaled oxygen and

Condition 2: 100% inhaled oxygen and inhaled Nitric Oxide at 20 parts per million

 

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Conclusion:

The clinical importance of the XMR facility was demonstrated in this case. Time-resolved angiography was rapidly able to give the anatomical diagnosis. The attending physicians could then progress to accurately determining pulmonary vascular resistance, in order to assess for operability. This type of split lung anatomy and vascular resistance calculation is only possible using the XMR facility. It would not be feasible or meaningful using traditional catheterisation with application of the Fick principle. (ref 4)

The resistance calculation demonstrated the the condition was not operable and symptomatic care was pursued.

Perspective:

XMR facility allows a rapid diagnostic service with invaluable clinical information. This type of accurate diagnostic information is not possible using traditional means and higlights the necessity for all units to have access to XMR facilities for complex congenital heart disease cases.

References:

1. Razavi R, Hill DL, Keevil SF, Miquel ME, Muthurangu V, Hegde S, Rhode K, Barnett M, van Vaals J, Hawkes DJ, Baker E. Cardiac catheterisation guided by mri in children and adults with congenital heart disease. Lancet. 2003;362:1877-1882

2. Kuehne T, Yilmaz S, Schulze-Neick I, Wellnhofer E, Ewert P, Nagel E, Lange P. Magnetic resonance imaging guided catheterisation for assessment of pulmonary vascular resistance: In vivo validation and clinical application in patients with pulmonary hypertension. Heart. 2005;91:1064-1069

3. Bell A, Beerbaum P, Greil G, Hegde S, Toschke AM, Schaeffter T, Razavi R. Noninvasive assessment of pulmonary artery flow and resistance by cardiac magnetic resonance in congenital heart diseases with unrestricted left-to-right shunt. JACC. Cardiovascular imaging. 2009;2:1285-1291

4. Talwar S, Meena A, Ramakrishnan S, Choudhary S, and Airan B. Hemitruncus with ventricular septal defect in a 6-year-old child. Ann Pediatr Cardiol. 2013 Jul-Dec; 6(2): 194–196.



COTW handling editor: Tarique Hussain, MD 

Have your say:  What do you think?  Latest posts on this topic from the forum

 

Re: Number 14-14: Late Presentation of Hemitruncus
Hi - Thank you Nadine & Carlos for the great questions.I agree with your experience about aortic regurgitation measurements which can be difficult. An excellent article has just recently been published about this by Professor Shi-Joon Yoo and colleagues...
On: 10/09/2014 By: mohammad.hussain@kcl.ac.uk Read more?

Re: Number 14-14: Late Presentation of Hemitruncus
This is an interesting case since most pulmonary arteries arising from the ascending aorta are usually right and not left pulmonary artery. One question I have is why was a time resolved MRA done instead of a conventional MRA. ...
On: 10/09/2014 By: nchoueiter Read more?

Re: Number 14-14: Late Presentation of Hemitruncus
Hi, Mohammad. Thanks for your answer. I do QFlow at the same plane aortic SSFP, but if there is aortic insufficiency I might do another QFlow proximal to the valve leaflets (LVOT); is that correct or it?s unnecessary ? ...
On: 10/09/2014 By: cfrosental Read more?

Re: Number 14-14: Late Presentation of Hemitruncus
Hi Carlos, thank you for your comment.I can see what you mean about the long-axis echo, that's a good spot. I think it may just be the cut that makes it appear that way. The valve was trifoliate in...
On: 10/08/2014 By: mohammad.hussain@kcl.ac.uk Read more?

Re: Number 14-14: Late Presentation of Hemitruncus
Hi, I am Carlos Fernando Rosental, ped cardiologist from Buenos Aires, Argentina. It?s a very intresting late presentation of hemitruncus. I wonder it you?d performed an aortic valve SSFP due to truncus association with dysplastic valve. However it seems to...
On: 10/08/2014 By: cfrosental Read more?

Number 14-14: Late Presentation of Hemitruncus
Welcome to the forum. I am opening myself to critical review by encouraging all Congenital Heart Disease sepcialists to become involved with case of the week. The case is indeed one of my own and I would be interested in...
On: 10/07/2014 By: mohammad.hussain@kcl.ac.uk Read more?

 

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