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A 20 year-old patient with dyspnea and thoracic pain

A 20-jear old patient was admitted to our ambulance with dyspnea, NYHA II – III and unspecific thoracic pain on exertion. The anamnesis was free of trauma, venous thrombosis, peripheral oedemas or hemostasiologic abnormalities. Body temperature was normal and the blood pressure was also in a normal range (110 / 70 mmHg). At initial presentation his ECG showed a sinus rhythm (85 bpm) with sparse ventricular extra-systoles but without any ST segment changes. Blood analysis was inconspicuous. To the best of his knowledge he could not remember an infective disease accompanied by fever in the last 6 month.
Myocardial Disease


Because his symptoms were triggered by excitation, we conducted a non-invasive stress testing with ECG. Surprisingly, this ECG displayed negative T waves in V4-V6 already under moderate excitation.

To confirm the positive ECG stress test and due to his young age we decided to perform cardiac magnetic resonance imaging (cMRI) including an additional stress protocol with adenosine prior to any invasive diagnostic.

To our surprise, the myocardial perfusion analysis revealed in subendocardial regions a reduced perfusion already under resting conditions. The analysis of myocardial texture was conducted with T2-mapping and late gadolinium enhancement (LGE) and is highlighted in Figure 1. While T2-mapping (left) revealed pathologic T2 times in the apex and in the midmyocardium (arrowheads) suspicious to be rather fresh than old, these lesions could also be identified by LGE. These lesions were characterized to most likely be ischemic on the basis of their subendocardial appearance.

For further diagnostic workup with cMRI we conducted more structural heart analyses including a MR coronary angiography (Figure 2). Interestingly, already a 3D-whole-heart-sequence displayed coronary aneurysms of greater than 1 cm diameter with signal depletion in the outer circumferences as a sign of calcification (arrowheads) for the right (left image) and the left coronary artery (middle image). The reconstructed MR coronary angiography (sb TFE) confirmed the diagnosis of a RIVA aneurysm after a normal LCA main stem (arrow) without the possibility to track the RIVA vessel towards the apex of the heart. The right coronary artery was only recognizable as a thin structure without any major vessel.

T2 mapping and late gadolinium enhancement in two chamber view and short axis


Figure 1: T2 mapping and late gadolinium enhancement in two chamber view and short axis.


Whole heart sequence with marked coronary aneurysm


Figure 2: Whole heart sequence with marked coronary aneurysm (marked with arrows).



QUESTIONS:

1.     What additional work-up would you perform in order to confirm the diagnosis?

2.     What is your differential diagnosis?



Conclusion:

Answers:


We itensified anamnesis in detail and got the important information that the patient suffered from a Kawasaki syndrome in his early childhood. However, he never consulted medical care afterwards due to his mild clinical symptoms.
Due to the finding of coronary aneurysms in cMRI for each coronary artery and the advanced structural heart disease we decided to perform a coronary angiography to evaluate coronary circulation and feasibility of a coronary intervention.


Figure 3: Aortography as well as coronary angiography acquired by fluoroscopy in 0° (figure 3A) and 90° LAO-projection (figure 3B). Marked with arrows: partly calcified aneurysms of LAD and RCX.

Already aortography identified three big, polymorph and partly calcified aneurysms (arrows). In line with MRI findings, the left main stem was still intact, while the left anterior descending artery (LAD) was totally occluded behind a partially filled aneurysm. The circumflex artery (RCX) was the major vessel of the left coronary artery (LCA) with an interposed subtotal stenosis. The proximal right coronary artery (RCA) was completely occluded with antegrade collaterals to the peripheral RCA and to the LAD.

Due to these findings, it was not possible to perform interventional revascularization. Instead, a coronary artery bypass grafting was successfully accomplished.

Kawasaki syndrome, also known as mucocutaneous lymph node syndrome is usually seen in early childhood (under 5 years of age). It affects mainly small and medium sized vessels with consecutive systemic inflammation. It´s most serious complication affects the heart with sometimes fatal coronary aneurysms.

According to the fact, that no specific laboratory test for Kawasaki disease exists, it can only be diagnosed clinically, by evaluation of clinical symptoms and medical signs. These symptoms are:
-    Five days of fever
-    erythema of the lips
-    plantar or palmar erythema
-    bilateral conjunctival injection
-    swollen lymph node in the neck

As differential diagnosis other serious diseases e.g. toxic shock syndrome, scarlet fever or juvenile idiopathic arthritis should be considered.

Intravenous immunoglobulin (IVIG) is the standard treatment of Kawasaki disease. Aspirin therapy should be added in high doses additionally to IVIG-therapy until the fever decreased to normal temperature and then should be continued for lifetime, when coronary aneurysms are found.

Notes to editor


Presented by :
Florian Bönner, Ralf Erkens, Britta Butzbach and Mirja Neizel
Department of Cardiology, Pulmonology and Vascular Medicine, Heinrich Heine University, Düsseldorf, Germany
The content of this article reflects the personal opinion of the author/s and is not necessarily the official position of the European Society of Cardiology.