Perinatal aortic disease is a rare and extremely serious group of cardiovascular diseases that includes aortic coarctation and aortic aneurysms. Perinatal aortic coarctation and aortic aneurysm rupture can lead to a significantly higher risk of maternal death and, if occurring before delivery, fetal death. Statistically, 50% of all patients with aortic coarctation in women younger than 40 years of age occur in the perinatal period, usually in late pregnancy or early postpartum.
A total of 96 published articles in the national and international literature report experience in the diagnosis and treatment of perinatal aortic coarctation, including 85 case reports and 11 series of cases or clinical treatises, reporting a total of 122 cases of perinatal aortic coarctation, of which the type of aortic coarctation was reported in 118 patients, respectively Stanford type A, 93 cases (78.8%); Stanford type B, 25 cases (21.2%). Perinatal Stanford type B aortic coarctation occurred in about 60% before delivery and in about 40% early after delivery, and some patients also had a combined ascending aortic aneurysm.
Similarly, aortic aneurysms are rare in women younger than 40 years of age, and 50% of the cases of aortic aneurysms in young women are pregnancy-related, usually in late pregnancy. When treating patients with perinatal aortic disease, cardiac surgeons need to consider saving two lives at the same time.
I. Treatment of perinatal Stanford type A aortic coarctation
The refined staging of the three cases of perinatal Stanford type A aortic coarctation that we admitted were all type A2C. For patients with A2C type of aortic coarctation, Bentall or David’s procedure with additional Sun’s procedure should be performed. Considering that the patient’s condition was severe and the combined obstetric surgery required the shortest possible extracorporeal circulation time, the Bentall+Sun procedure was performed in all three patients to avoid the prolongation of extracorporeal circulation and operative time due to unsatisfactory aortic valvuloplasty followed by aortic valve replacement.
The average extracorporeal circulation time was 179 ± 18 min, the aortic block time was 103.3 ± 12.5 min, and the deep hypothermic arrest time was 39 ± 0.5 min. Among the patients: one was 22+ weeks of gestation and was treated with aortic coarctation along with cesarean extraction and total hysterectomy; one was 34+ weeks of gestation and was treated with aortic coarctation along with cesarean delivery and total hysterectomy; and one was treated with Bentall+Sun 2 months after cesarean delivery.
II. Treatment of simple Stanford B-type aortic coarctation in perinatal period
We admitted 4 cases of perinatal simple Stanford B aortic coarctation with refined typing: 2 cases of B1S type, 1 case of B1C type, and 1 case of B3C type. B1S type aortic coarctation is an indication for intracavitary repair of the aorta with overlapping stents, so the patients were recommended for interventional treatment, and 1 patient underwent intracavitary repair of the aorta, and 1 patient was not suitable for interventional treatment because of the thin femoral artery. One patient was treated conservatively and followed up because the femoral artery was too thin for intervention. For patients with B1C aortic coarctation, because the coarctation involved the left subclavian artery opening, a left common carotid artery to left anterior clavian artery diversion + intraoperative descending aortic stent implantation was performed, and the right axillary artery was used for parasternal cerebral perfusion.
The patient with type B3C aortic coarctation was an indication for total thoracic descending aortic and abdominal aortic revascularization, so we performed total thoracoabdominal aortic replacement using femoral arterial cannulation to establish extracorporeal circulation, and the duration of extracorporeal circulation was 112 min. The patient with type B1S aortic coarctation was 28+ weeks of gestation and underwent cesarean section 2 weeks after endovascular treatment of the aorta; the patient with type B1C aortic coarctation was 23+ weeks of gestation and underwent left common carotid artery to left anterior clavicular artery diversion + intraoperative descending aortic stenting 27 days after hydrosalping induction of labor; the patient with type 3C aortic coarctation was 22 weeks of gestation and underwent total thoracoabdominal replacement 3.5 months after cesarean section + double vasectomy. aortic replacement.
Treatment of simple aortic root aneurysm in perinatal period
Based on the anatomical characteristics of aortic root aneurysms, the conventional surgical treatment is to replace the aortic valve and ascending aortic root with a valved conduit and to perform left and right coronary artery grafts [6]. The average extracorporeal circulation time was 100 ± 26.9 min and the aortic block time was 73.5 ± 30.4 min. In the other case, a cesarean section was performed before the aortic surgery at 27+ weeks of gestation.
Treatment of aortic root aneurysm combined with Stanford type B aortic coarctation in perinatal period
One patient with aortic root aneurysm combined with Stanford type B aortic coarctation was treated with B1C aortic coarctation subclassification. The duration of extracorporeal circulation was 224 min, the duration of aortic block was 172 min, and the duration of deep hypothermic arrest was 28 min. The patient was 8+ weeks of gestation and negative pressure aspiration was performed 27 days before the aortic surgery.
Outcome of perinatal Stanford type A aortic coarctation
Two of the three patients had postoperative complications, including renal failure, and these two patients died postoperatively, both due to multi-organ failure related to the procedure: one was 22+ weeks of gestation and was treated for aortic coarctation with a cesarean section and total hysterectomy, and the fetus died; one was treated with a Bentall+Sun procedure 2 months after the cesarean section, and the fetus was in good condition with an Arthritis score of 10. The other patient had a good postoperative recovery with no associated surgical complications. Due to the cesarean delivery at 34+ weeks of gestation, the fetus had an Arrhenius score of only 4, but recovered and was discharged after treatment.
Outcome of perinatal simple Stanford type B aortic coarctation
Patients who received conservative treatment for B1S aortic coarctation were discharged in good condition, having undergone a cesarean section at an outside hospital prior to admission, and the fetus was in good condition with an Arrhenius score of 10. The patient with B1S aortic coarctation who underwent intracavitary aortic repair recovered well and underwent a cesarean section 2 weeks after surgery with a good fetus and an Arthritis score of 10. The patient with B1C aortic coarctation with no significant postoperative complications recovered and was discharged at 23+ weeks of gestation, having undergone a cesarean section prior to surgery. The patient with B3C aortic coarctation was 22 weeks of gestation and underwent a cesarean section + bilateral salpingo-oophorectomy followed by The patient was discharged with no significant postoperative complications.
IV. Treatment outcome of perinatal simple aortic root aneurysm
In one case, the patient recovered well after Bentall’s operation at 18+ weeks of gestation without any related surgical complications, and the fetus was examined normally and the pregnancy continued; in the other case, the patient was treated for aortic root aneurysm 6.5 months after cesarean section at 27+ weeks of gestation, and the patient recovered well without any related surgical complications and was discharged from the hospital. aortic mucinous lesions.
Treatment outcome of perinatal aortic root aneurysm combined with Stanford type B aortic coarctation
The patient with aortic root aneurysm combined with Stanford type B aortic coarctation was 8+ weeks of gestation and was discharged after 27 days of negative pressure aspiration followed by Bentall+Sun surgery.
Aortic disease is a critical cardiovascular disease that includes aortic coarctation and aortic aneurysm. Aortic coarctation and aortic aneurysm rupture in the perinatal period can lead to a significantly increased risk of fetal and maternal mortality. In the published literature reporting perinatal aortic coarctation both nationally and internationally, three cases of Stanford type A, five cases of Stanford type B, and two cases of aortic root walk were included in this group of cases.
Aortic coarctation and aortic aneurysm are associated with pregnancy in 50% of women younger than 40 years of age, usually in late pregnancy. The increase in blood volume during pregnancy leads to an increase in left ventricular output and an increase in the impact of blood flow on the aortic wall, while estrogen inhibits the deposition of collagen and elastic fibers in the aortic wall and progesterone promotes the deposition of non-collagenous proteins in the vessel wall, resulting in a decrease in the elasticity of the vessel wall and an increase in brittleness, thus promoting the formation and rupture of aortic coarctation. The age of all patients in this group was less than 35 years, which also confirms that pregnancy is a high risk factor for the development of aortic disease in young women.
There are many other factors that play an important role in the development of perinatal aortic coarctation and aortic aneurysm, most notably a history of hypertension and Marfan’s syndrome, which is associated with most perinatal aortic disease. The combination of hypertension in 3 cases and Marfan’s syndrome in 2 cases in our group also confirms that a history of hypertension and Marfan’s syndrome are high risk factors for perinatal aortic disease. In addition, we found that a significant feature of this group of cases was that the patients were all taller, much higher than the average height of adult women aged 25-35 years in China, suggesting that excessive height may also be a risk factor for the development of perinatal aortic disease.
Of course, the higher average height in this group was related to the higher height of the 2 patients with Marfan’s syndrome, but the average height after removing these 2 patients was still much higher than the average height of Chinese women of the same age group, and all but 1 of the 10 patients in this group were slightly lower than the average height of the Chinese population. Therefore, we speculate that high height may also be a risk factor for the development of perinatal aortic disease, but given the small sample size in this group, our speculation needs to be confirmed by a large sample size study.
When treating patients with perinatal aortic disease, cardiac surgeons need to consider saving the lives of two people at the same time to the greatest extent possible. The overall mortality rate of pregnancy and delivery in women with aortic disease is as high as 30%, and the rate of cesarean delivery is high, 30% to 60%, in order to avoid prolonged delivery and the risk of cardiovascular system pathology exacerbated by obstructed labor [10]. In this group of cases, the fetus survived in four patients (one other continued pregnancy), two patients had delivered by cesarean section before admission for treatment of aortic disease, one delivered by cesarean section 2 weeks after intervention, and one delivered by cesarean section and hysterectomy at the same time during surgical procedure, and the mode of delivery was cesarean section in all cases.
The remaining 5 patients were less than 28 weeks of gestation, 1 of whom received negative pressure suction before aortic surgery at 8+ weeks of gestation, 1 of whom was induced with a water bag before surgery, 2 of whom underwent cesarean section before surgery, and 1 of whom underwent cesarean section plus hysterectomy at the same time as aortic surgery. It can be seen that the management of the fetus with aortic disease needs to make different choices according to the size of the gestational week: for patients with less than 28 weeks of gestation, if the condition allows, the aortic disease should be treated after abortion, if the condition is critical, such as patients with type A aortic coarctation, the aortic surgery should be performed first, and then the decision to induce labor or continue the pregnancy should be made according to the condition of the fetus; for patients with more than 28 weeks of gestation, they should If the condition of the fetus allows, the aortic disease can be treated after the cesarean section, and if the condition is critical, the cesarean section can be performed at the same time as the aortic surgery. Regardless of cesarean delivery or cesarean retrieval, as long as it is performed in conjunction with surgical procedures for the aorta, we recommend adding a hysterectomy to avoid uncontrollable uterine bleeding due to prolonged extracorporeal circulation and heparinization.
In addition, factors such as environmental pollution seriously affect pregnancy and lead to infertility and miscarriage, and many young couples have had successful pregnancies only through assisted reproductive techniques, as was the case in two of our cases (nos. 5 and 8). Such a fetus is extremely precious to the patient, so although interventions and surgical procedures can have some impact on the health of the fetus and we do not recommend continuing the pregnancy, patients in such cases often insist on continuing the pregnancy. The overall mortality rate in this group was 20%, of which all the patients who died were type A aortic coarctation, while the rest had no deaths, also indicating that type A aortic coarctation is the most aggressive type of perinatal aortic disease.
In summary, perinatal aortic disease includes Stanford type A and B aortic coarctation and aortic aneurysm. Treatment strategies include pharmacological treatment, endoluminal repair treatment and surgical treatment, and various indications for treatment need to be judged comprehensively according to the perinatal clinical status, with Stanford type A aortic coarctation having the worst prognosis, and the management of the fetus needs to be selected according to the gestational week and the severity of the aortic disease.