vlarfan syndrome and coarctation are two aortic diseases that place the pregnant woman at increased risk for aortic dissection (Russo, 2017). Indeed, half of dissection cases in young women are related to pregnancy (O'Gara, 2004). Other risk factors are bicuspid aortic valve and Turner or Noonan syndrome. A high rate of aortic dissection or rupture is also reported in patients with Ehlers-Danlos syndrome (Murray, 2014; Pepin, 2000). Although the mechanism(s) involved are unclear, the initiating event is a tear in the intimal layer of the aorta, followed by hemorrhage into the media, and finally rupture.
In most cases, aortic dissection presents with severe chest pain described as ripping, tearing, or stabbing. Diminution or loss of peripheral pulses coupled with a recently acquired aor tic insuiciency murmur is an important physical inding. he diferential diagnosis of aortic dissection in pregnancy includes myocardial infarction, pulmonary embolism, pneumothorax, aortic valve rupture, and obstetrical catastrophes, especially placental abruption and uterine rupture.
More than 90 percent of patients with aortic dissection have an abnormal chest radiograph. Aortic angiography is the most definitive method for diagnosis confirmation. However, sonog raphy, computed tomography, and MR imaging are used more frequently depending on the urgency of the clinical situation.
Initial medical treatment is given to lower blood pressure.
Proximal dissections most often need to be resected, and the aortic valve replaced if necessary. Distal dissections are more complex, and many may be treated medically. Among non pregnant patients, survival is not improved by immediate elec tive repair compared with surveillance and delayed repair of abdominal aortic aneurysms <5.5 cm. But, Karthikesalingam and colleagues (2016) suggest that the size threshold for aneu rysm repair should be revisited.
This autosomal dominant connective tissue disorder has an incidence of 2 to 3 cases per 10,000 individuals and is without racial or ethnic predilection (Ammash, 2008). As discussed in Chapter 59 (p. 1151), Marfan syndrome is characterized by generalized tissue weakness that can result in dangerous cardiovascular complications. Because all tissues are involved, other defects are frequent and include joint laxity and scoliosis. Progressive aortic dilation causes aortic valve insuiciency, and there may be infective endocarditis and mitral valve prolapse with insuiciency. Aortic dilation and dissecting aneurysm are the most serious abnormalities. Early death is due either to valvular insuiciency and heart failure or to a dissecting aneurysm.
Of outcomes, a study using the Nationwide Inpatient Sample from 2003 to 2010 found 339 deliveries in women with Marfan syndrome. There was one maternal death and six (1.8 percent) aortic dissections (Hassan, 2015). Russo and associates (2017) used Texas obstetrical discharge data and found that eight of 47 women with aortic dissection had Marfan syndrome. A study from the United ingdom reported similar results (Curry, 2014).