Arterial Tortuosity Syndrome

Arterial tortuosity syndrome (ATS) is a rare hereditary disorder with variable clinical presentation involving tortuosity and elongation of the ma-

Arterial Tortuosity Syndrome

Fig. 14. MIP reconstruction of a 3D CE MRA dataset (Gd-BOPTA, 0.1 mmol/kg, total contrast agent volume 0.3 ml) in a male newborn with arterial tortuosity syndrome. Imaging was performed with a wrap around shoulder coil covering the whole body of the baby. Note the kinking of the thoracic and abdominal aorta (arrows) as well as of the supraaortic and iliac vessels (arrowheads)

Fig. 14. MIP reconstruction of a 3D CE MRA dataset (Gd-BOPTA, 0.1 mmol/kg, total contrast agent volume 0.3 ml) in a male newborn with arterial tortuosity syndrome. Imaging was performed with a wrap around shoulder coil covering the whole body of the baby. Note the kinking of the thoracic and abdominal aorta (arrows) as well as of the supraaortic and iliac vessels (arrowheads)

jor arteries. Aneurysm, pulmonary artery stenosis, pulmonary hypertension, skin and joint laxity can be associated and are suggestive of a connective tissue disorder. The transmission of ATS follows an autosomal recessive pattern [34].

Genetic analyses of inbred families have revealed a gene localization on chromosome 20q13, which is different to the locations for other connective tissue disorders such as Marfan's, Beal's or Williams' syndromes. As yet, the causative gene has not been completely identified [35].

The tortuous course of the major arterial vessels as well as associated vascular abnormalities can be excellently demonstrated on 3D CE-MRA (Fig. 14). Additionally, stenotic or ectatic vascular changes can be detected. In pediatric patients, for whom conventional angiography is very challenging and combined with a high risk for vascular damage especially in cases with increased vessel tortuosity, CE-MRA should be the preferred technique for diagnostic evaluation.

Fig. 15 a, b. Surface rendering of a 3D CE MRA dataset (Gd-BOPTA, 0.1 mmol/kg) of the thoracic vessels. Due to atresia of the RVOT a Blalock-Taus-sig anastomosis connecting the left subclavian artery with the left pulmonary artery (arrow) was created. On the corresponding posterior-anterior view an additional MAPCA is depicted (arrows). Distally, the MAPCA connects to the pulmonary trunk (arrowhead)

Atresia Pulmonar Com Blalock

Fig. 15 a, b. Surface rendering of a 3D CE MRA dataset (Gd-BOPTA, 0.1 mmol/kg) of the thoracic vessels. Due to atresia of the RVOT a Blalock-Taus-sig anastomosis connecting the left subclavian artery with the left pulmonary artery (arrow) was created. On the corresponding posterior-anterior view an additional MAPCA is depicted (arrows). Distally, the MAPCA connects to the pulmonary trunk (arrowhead)

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