Severe Takaysu arteritis in a 43 year old woman with pulmonary hypertension. Therapy with reconstruction of aortic root, left coronary artery origin, aortic arch and the pulmonary artery bifurcation

History: A 43-year-old women presented in the 14 week of pregnancy with increasing shortness of breath. The only significant medical event was a phlegmon of the right upper leg with an ANA titer of 1:400 two years ago. Therefore, a short prednisolone therapy was initiated with the diagnosis of cutaneous lupus erythematosus.

 

Examination: The electrocardiogram showed a sinus rhythm and signs of leftventricular hypertrophy. On echocardiography a pericardial effusion and an advanced biventricular hypertrophy could be seen (Fig. 1A). The doppler gradient across the tricuspid valve was 80 mmHg and a stenosis of the pulmonary artery was suspected.

After therapeutic drainage of 1,5 liters of pericardial fluid a diagnostic cardiac catheter was performed following a detailed informed consent. Both side branches of the pulmonary arteries where stenosed, the right to 5,5 mm diameter, the left with an gradient of 80 mmHg (Fig. 1D). The origin of the left main coronary artery was stenosed (Fig.1B) with an area of 7,4 qmm on intravascular ultrasound (Fig. 1C).

The brachiocephalic trunk and the left carotid artery showed stenoses at the origin with poststenotic dilatation (Fig. 1E,1F) The left subclavian artery was occluded. The aortic arch itself was stenosed with a gradient of 80 mmHg (Fig 1F). The gradients in the pulmonary artery and aortic arch explained the biventricular hypertrophy.

 

Therapy: In an interdisciplinary conference with heart surgeons and gynecologists the decision of operative treatment was made. In the operating room the aortic root showed severe adhesions and a thickening of the wall from 1 to 2 cm. The resection of the distal aortic arch was only possible after resection of the vasculitic infiltrated and stenosed left pulmonary artery. The pulmonary valve was soft, only the pulmonary artery was replaced with a xenogeneic pericardial tube and xenogeneic patch of the truncus pulmonalis (Fig 1G).

 

The thickened aortic wall stenosed the ostium of the left main coronary artery. A reconstruction of the coronary ostium with a 8 mm dacron graft was performed. The aortic arch was completely replaced with a prothesis. A saving of the left recurrent nerve was not possible. The occluded left subclavian artery was reinserted. The brachiocephalic trunk was stenosed with low flow after reinsertion. Therefore, an 8 mm bypass from the aortic prothesis to the left subclavian artery was applied (Fig. 1H,1I). Histology of the aorta showed degerenative media hyperplasie, lymphocytic infiltration, bleeding and calcification.

The total operation time was 11 hours with 412 min on extracorporeal circulation. Postoperatively spontaneous abortion occurred. The next day low flow in the right carotid artery was diagnosed. A second operation showed thrombotic material in the brachiocephalic trunk and a new 6 mm graft was applied from the aortic prosthesis to the right common carotid artery (Fig 1H,1I).

The patient recovered well with a hoarse voice and was discharged 15 days after operation with cortisone therapy for her vasculitis.

 

Summary: Due to the increase of cardiac output in pregnancy the young patient presented with cardiac decompensation. An advanced vasculitis with involvement of all branches of the aorta and the pulmonary branches was diagnosed (Takayasu arteritis). It could be treated with vascular grafts with a good result. Long-term therapy with prednisolone was started.




Figure 1: Medical record of a 43-year-old women with Takayasu arteritis