1Medizinische Klinik a Poliklinik I and 2Radiologische Klinik, Universitätsklinikum Bonn, Germany
Correspondence and offprint requests to: Andre Werner Demant, MD, Medizinische Klinik und Poliklinik I, Universitätsklinikum Bonn, Germany. Email: andre.demant{at}t-online.de
Keywords: dialysis; end-stage renal disease; pregnancy; Stanford-B-aneurysm
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Introduction |
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Case |
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The medical history of the patient was characterized by severe idiopathic hypertension for 10 years that had not been treated thus far.
Computer tomographic (CT) and angiographic diagnostics revealed a spontaneous extended Stanford-B-aneurysm of the aorta. The dissection started distal to the origin of the left subclavian artery, according to a Stanford type B dissection (Figure 1A). Compression of the true lumen by the large false lumen led to malperfusion of the right iliac artery resulting in ischaemia of the right leg as well as infarction of the right kidney (Figure 1B). Since the patient refused surgical aneurysm treatment due to life-threatening risk, a catheter-based radiological fenestration of the dissection membrane was performed. After fenestration of the dissection membrane and decompression of the false lumen, the angiogram showed an effective reperfusion of the right iliac artery. However, the patient developed ESRD since the left kidney was atrophic and the right one lost its function. As a consequence, the patient joined our chronic haemodialysis programme. Dialysis was performed for 15 h a week with a constant blood flow of 250 ml/min.
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Amniocentesis showed a normal fetal evolution. After the 21st week of pregnancy, the patient had weekly obstetric follow-ups including ultrasound and cardiotocogram. Despite the occurrence of a polyhydramnio, all Doppler wave velocimetries showed a normal perfusion of both uterine arteries without evidence of malperfusion of the uterus.
Due to increasing blood pressure, oral dihydralazine therapy had to be initiated in the 29th week with primarily good results. Although there were no clinical signs of pre-eclampsia or HELLP syndrome, the patient underwent a prophylactic caesarean section at the 31th week due to labile hypertension.
A female baby weighing 1900 g with an Apgar Score of 5/8/10 was delivered and, due to its prematurity, transferred to the neonatal intensive care unit (ICU). After delivery, the mother was monitored on the ICU for 36 h and discharged 1 week later.
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Discussion |
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In line with current concepts, the dialysis treatment was intensified after diagnosis of pregnancy since a better outcome of pregnancies in haemodialysis patients, a longer gestational period and a higher birth weight are strongly associated with: adequacy of dialysis; control of hypertension with haemodynamic stability; correction of anaemia; and sufficient nutrition, as discussed in detail elsewhere [47].
Spontaneous dissection of the aorta is a rare entity [8,9]. Our patient had had a history of severe and untreated hypertension which is in line with most described cases. However, there were no further established predictors such as thoracic pain, stroke or abnormal anatomy [8]. In contrast, she had a history of heavy and ongoing smoking, which may have adversely affected the clinical course, since ongoing smoking may alter endothelial and vascular properties.
Treatment strategies for aortic aneuryms should comprise reperfusion attempts either by surgical procedures or by catheter-based radiological interventions, which may be less invasive [8]. Furthermore, a strict blood pressure control should be maintained to avoid spontaneous rupture of the aneurysm. Due to the aneurysm's blood flow of our patient, a special device to measure blood pressure on the left ankle was required to gain reliable data. Intradialytic blood pressure controls revealed a mean arterial pressure of 107±4 mmHg, which remained stable over the time course. Conversely, we avoided hypotensive episodes which could be potentially harmful for the fetus.
Obstetric monitoring of the patient included Doppler wave velocimetry and ultrasound examinations, so that a malperfusion of the uterine arteries could be excluded. An amniocentesis in the second trimester showed a normal result. Like many other pregnant haemodialysis patients who usually have an increased incidence of polyhydramnios compared with the general population, our patient also developed a polyhydramnio. However, fetal development was almost normal so that the child weighed 1900 g at delivery. As described elsewhere [47], intensification of the dialysis dose may have led to a more appropriate gestational period and a good birth weight. Although our patient did not exhibit any clinical signs of pre-eclampsia or HELLP syndrome, she underwent a prophylactic caesarean section at the 31th week due to the onset of labile hypertension.
Taken together, we hypothesize that in our patient the conditions for a conception and a successful pregnancy were enabled by the recovery of blood flow to the uterus after catheter-based fenestration of the aneurysm's dissection membrane. Furthermore, this report illustrates that even a pregnancy with severe vascular complications, such as an extended aneurysm of the aorta, permits a successful outcome. However, it has to be taken into account that pregnancies in women on haemodialysis still remain high-risk pregnancies. Thus, the outcome strongly depends on the optimal dialysis strategy and the co-operation between nephrologists, obstetrics and neonatal medicine.
Conflict of interest statement. None declared.
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