Repair of complex aortic aneurysms such as those involving the thoracoabdominal and pararenal aorta presents a formidable challenge for surgeons with significant perioperative morbidity and mortality. A hybrid procedure combining renovisceral debranching with endovascular aneurysm exclusion has been developed as an alternative approach for high-risk patients. This paper reports our initial experience with hybrid repair for these complex aortic diseases in three high-risk patients.

BACKGROUND

Endovascular technique has replaced open repair as primary treatment for different aortic disease indications and was associated with low perioperative mortality and acceptable short-, mid-, and long-term survival. Locally, thoracic endovascular aortic repair (TEVAR) was not widely practiced until year 2017. This study aims to determine the predictors of in-hospital and short-term outcomes of patients who underwent TEVAR for aortic aneurysm and aortic syndrome in a single center and how it compares with local and international data.

This study is a retrospective analysis of 52 adult patients who underwent TEVAR for the treatment of aortic aneurysm and aortic syndrome. Demographic and clinical data, diagnostic imaging, and procedural details were obtained via inpatient charts at the medical records section and hospital system database archiving. Outcomes at 30 days and 1 year postprocedure were obtained through telephone follow-up after attaining verbal consent.

Gathered data were analyzed as to association of different variables with or without the presence of complications. Outcomes reported included in-hospital mortality rate, presence of major adverse events (MAEs), 30-day and 1-year survival rates, and rate of freedom from reintervention.

The overall in-hospital mortality was 7.69% (n = 4/52), and complication rate was 32% (n = 20/52), with a survival rate of 92.31% and 87.76% at 30 days and 1 year, respectively. The rates of overall freedom from reintervention were 83.33% and 100% at 30 days and 1 year, respectively. The independent predictors for in-hospital mortality and development of MAEs were increasing weight (odds ratio [OR], 1.0588; 95% confidence interval [CI], 1.003–1.208), preexisting chronic kidney disease (OR, 10.33; 95% CI, 1.1069–96.462), and TEVAR with debranching done as a single procedure (OR, 3.6667; 95% CI, 1.1154–12.054), whereas an estimated glomerular filtration rate of 49.05 ± 19.25 (OR, 0.9402; 95% CI, 0.9019–0.9801) and TEVAR with debranching done as a staged procedure (OR, 0.1624; 95% CI, 0.0321–0.8225) statistically decrease the risk for development of in-hospital mortality and MAEs (P = 0.001 and P = 0.028, respectively).

In this single-center study, indications for TEVAR were fusiform and saccular aneurysm, high-risk intramural hematoma and penetrating aortic ulcer, complicated acute type B dissection, chronic complicated type B dissection with high-risk feature, and aortic rupture. The outcome of this study shows comparable results with other international studies with an acceptable in-hospital mortality rate, complication rate, short-term survival rate, and rate freedom from reintervention at 30 days and 1 year. Increasing weight, preexisting chronic kidney disease, and TEVAR with debranching done as a single setting are independent predictors for developing in-hospital mortality and MAEs, whereas a normal estimated glomerular filtration rate and TEVAR with debranching done as a staged procedure decrease the risk; hence, careful planning and scheduling of procedure among elective and amenable cases could further reduce complication rates of future TEVAR procedures.

BACKGROUND

Takayasu Arteritis (TA) is a rare, primary large-vessel vasculitis frequently leading to stenosis and less commonly, aneurysm formation. Saccular aneurysms of the aortic arch in patients with TA are fatal, have rarely been reported and represent a significant technical challenge due to the difficult anatomical location and need for protection of the cerebral circulation. Concomitant intracerebral aneurysms in patients with TA are extremely uncommon and have mostly been documented in very few case reports in literature.

We present a case of a 31 year-old Filipino female with recurrent chest and neck pain radiating to the upper back. Computed tomographic (CT) angiography demonstrated a large saccular aortic arch aneurysm without branch stenosis. CTA of the cerebral circulation likewise demonstrated multiple, saccular, intra-cerebral aneurysms. She underwent hybrid thoracic arch repair with supra-aortic debranching via mini-sternotomy and proximal ligation of the left common carotid artery and staged endovascular aortic arch replacement with coil embolization of the ostial-to-proximal left subclavian artery segment. Post-operative aortogram showed optimal repair with thrombosed aneurysmal sac, optimal graft position, no endoleaks and preservation of cerebral circulation. Patient improved symptomatically post-procedure and remained symptom-free during follow-up after six months. Careful review of local literature suggests that this is the first Philippine TA case with a saccular aortic arch aneurysm successfully managed in this manner.

Saccular aortic arch aneurysms in patients with Takayasu are unusual and presence of concomitant multiple cerebral saccular aneurysms have rarely been reported in literature. This case highlighted that hybrid endovascular arch repair in patients with TA is feasible, minimally invasive and effective.

Ruptured Sinus of Valsalva (RSOV) is a rarely encountered cardiac anomaly that can potentially lead to adverse clinical outcomes. RSOV increases the risk of morbidity during pregnancy due to the physiological changes associated with gestation, that can exacerbate the underlying cardiac pathology. We present the case of a 29-year-old female with an uncorrected RSOV who required an emergency cesarean section for abruptio placenta. The patient underwent the procedure under spinal anesthesia, with careful titration of norepinephrine infusion and close monitoring of hemodynamic parameters using an invasive intra-arterial line. Given the absence of established anesthetic protocols for parturients with RSOV undergoing cesarean delivery, a comprehensive understanding of the complex interaction between the hemodynamic effects of RSOV, pregnancy, and anesthesia is essential. This understanding enables the safe use of spinal anesthesia in urgent situations, leading to favorable patient outcomes.
Sinus of Valsalva ; Aortic Rupture ; Cesarean Section ; Anesthesia, Spinal

Repair of complex aortic aneurysms such as those involving the thoracoabdominal and pararenal aorta presents a formidable challenge for surgeons with significant perioperative morbidity and mortality. A hybrid procedure combining renovisceral debranching with endovascular aneurysm exclusion has been developed as an alternative approach for high-risk patients. This paper reports our initial experience with hybrid repair for these complex aortic diseases in three high-risk patients.
Human ; Male ; Aged: 65-79 yrs old ; Middle Aged: 45-64 yrs old ; aorta ; aortic aneurysm ; endovascular procedures

Humans ; Endovascular Aneurysm Repair ; Endovascular Procedures ; Blood Vessel Prosthesis Implantation ; Aortic Aneurysm, Abdominal/surgery* ; Treatment Outcome ; Postoperative Complications ; Risk Factors ; Aortic Rupture/surgery* ; Retrospective Studies

OBJECTIVE: To summarize the clinical features and spectrum of genetic variants in 12 patients with Loeys-Dietz syndrome (LDS), and to explore the correlation between the type of genetic variants and clinical phenotypes. METHODS: Twelve patients suspected for LDS at Beijing Anzhen Hospital Affiliated to Capital Medical University from January 2015 to January 2022 were selected as the study subjects. Clinical data of the patients were collected. Genomic DNA was extracted from peripheral blood samples and subjected to genetic testing. Pathogenicity of candidate variants was analyzed. RESULTS: The clinical phenotypes of the 12 patients have mainly included cardiovascular, musculoskeletal, craniofacial, skin, ocular and other systemic signs. Four patients (patients 5-1, 5-2, 6, 7) have carried heterozygous missense variants of the TGFBR1 gene, 5 patients (patients 1-1, 1-2, 2, 3, 4) have carried heterozygous variants of the TGFBR2 gene, and 2 patients (patients 8-1, 8-2) had carried heterozygous frameshift variants of the TGFB3 gene. One patient (patient 9) had carried a heterozygous missense variant of the SMAD3 gene. Among these, TGFBR1 c.603T>G (p.1201M) and TGFB3 c.536delA (p.H179FS35) had not been reported previously. CONCLUSION Variants of the TGFBR1, TGFBR2, SMAD3, TGFB2, TGFB3 and SMAD2 genes are mainly associated with LDS. The severity of the disease phenotype caused by the same variant may vary, whilst the clinical phenotype caused by different variant sites may be specific.
Humans ; Loeys-Dietz Syndrome/genetics* ; Receptor, Transforming Growth Factor-beta Type I/genetics* ; Receptor, Transforming Growth Factor-beta Type II/genetics* ; Transforming Growth Factor beta3 ; Face

OBJECTIVE: To explore the genetic basis of a patient with clinically suspected Loeys-Dietz syndrome (LDS). METHODS: A child who had presented at Beijing Anzhen Hospital in September 2018 was selected as the study subject. Clinical data and family history of the patient were collected, along with peripheral blood samples of the proband and his parents. Whole exome sequencing (WES) was carried out through next-generation sequencing. RESULTS: Candidate variants were searched through bioinformatic analysis focusing on genes associated with hereditary aortic aneurysms. Candidate variant was verified by Sanger sequencing. The patient was found to have cardiovascular abnormalities including early-onset aortic dilatation and coarctation, and LDS syndrome was suspected. WES revealed that he has harbored a heterozygous c.1526G>T missense variant of the TGFBR2 gene. The same variant was not found in either parent and was predicted as likely pathogenic (PM1+PM2_Supporting+ PM6+PP3+PP4) based on the guidelines from the American College for Medical Genetics and Genomics (ACMG). CONCLUSION The TGFBR2 c.1526G>T variant probably underlay the LDS in this patient and was unreported previously in China. Above finding has enriched the mutational spectrum of the TGFBR2 gene associated with the LDS and provided a basis for the genetic counseling for the patient.
Child ; Humans ; Male ; China ; Computational Biology ; Family ; Loeys-Dietz Syndrome/genetics* ; Mutation ; Receptor, Transforming Growth Factor-beta Type II/genetics*

Objective: To evaluate the efficacy of surgical treatment of aortic coarctation combined with descending aortic aneurysm in adult patients. Methods: This is a retrospective cohort study. Adult patients with aortic coarctation who were hospitalized in Beijing Anzhen Hospital from January 2015 to April 2019 were enrolled. The aortic coarctation was diagnosed by aortic CT angiography, and the included patients were divided into the combined descending aortic aneurysm group and the uncomplicated descending aortic aneurysm group based on descending aortic diameter. General clinical data and surgery-related data were collected from the included patients, and death and complications were recorded at 30 days after surgery, and upper limb systolic blood pressure was measured in all patients at discharge. Patients were followed up after discharge by outpatient visit or telephone call for their survival and the occurrence of repeat interventions and adverse events, which included death, cerebrovascular events, transient ischemic attack, myocardial infarction, hypertension, postoperative restenosis, and other cardiovascular-related interventions. Results: A total of 107 patients with aortic coarctation aged (34.1±15.2) years were included, and 68 (63.6%) were males. There were 16 cases in the combined descending aortic aneurysm group and 91 cases in the uncomplicated descending aortic aneurysm group. In the combined descending aortic aneurysm group, 6 cases (6/16) underwent artificial vessel bypass, 4 cases (4/16) underwent thoracic aortic artificial vessel replacement, 4 cases (4/16) underwent aortic arch replacement+elephant trunk procedure, and 2 cases (2/16) underwent thoracic endovascular aneurysm repair. There was no statistically significant difference between the two groups in the choice of surgical approach (all P>0.05). In the combined descending aortic aneurysm group at 30 days after surgery, one case underwent re-thoracotomy surgery, one case developed incomplete paraplegia of the lower extremity, and one case died; and the differences in the incidence of endpoint events at 30 days after surgery were similar between the two groups (P>0.05). Systolic blood pressure in the upper extremity at discharge was significantly lower in both groups compared with the preoperative period (in the combined descending aortic aneurysm group: (127.3±16.3) mmHg vs. (140.9±16.3) mmHg, P=0.030, 1 mmHg=0.133 kPa; in the uncomplicated descending aortic aneurysm group: (120.7±13.2) mmHg vs. (151.8±26.3) mmHg, P=0.001). The follow-up time was 3.5 (3.1, 4.4) years. There were no new deaths in the combined descending aortic aneurysm group, no transient ischemic attack, myocardial infarction or re-thoracotomy surgery, and one patient (1/15) suffered cerebral infarction and 10 patients (10/15) were diagnosed with hypertension. The differences in the occurrence of endpoint events during postoperative follow-up were similar between the two groups (P>0.05). Conclusion: In experienced centers, long-term prognosis of patients with aortic coarctation combined with descending aortic aneurysm is satisfactory post surgical intervention.
Male ; Humans ; Adult ; Female ; Aortic Coarctation/surgery* ; Retrospective Studies ; Aortic Aneurysm, Abdominal/surgery* ; Treatment Outcome ; Blood Vessel Prosthesis Implantation/adverse effects* ; Endovascular Procedures/adverse effects* ; Hypertension/complications* ; Myocardial Infarction/complications* ; Aortic Aneurysm, Thoracic/surgery*

Objective: To investigate the pathological changes of placenta in pregnant women with aortic dissection/aneurysm and their relationship with clinical features. Methods: The placental samples of 14 pregnant women with aortic dissection/aneurysm diagnosed from January 2012 to October 2021 and 10 normal placental samples of pregnant women from January 2021 to December 2021 at Beijing Anzhen Hospital Affiliated to Capital Medical University, Beijing, China were selected. Routine H&E staining and immunohistochemistry were used to analyze the histological features under light microscope. The clinical data were also analyzed. Results: The age of 14 pregnant patients with aortic dissection/aneurysm for placental examination ranged from 22 to 38 years (median, 28 years). The gestational ages ranged from 22 to 39 weeks (median, 34 weeks). The pregnancy of second trimester was noted in 2 cases, and the third trimester in 12 cases. All cases were singleton pregnancy. Seven cases were Stanford type A aortic dissection, 6 cases were Stanford type B aortic dissection, and one case was aortic root aneurysm. Four of the pregnant women underwent aortic dissection surgery after caesarean section, three underwent caesarean section after aortic dissection surgery, and seven underwent both caesarean section and aortic dissection procedures. Among the newborns, 2 cases were full-term birth, and 12 cases were premature birth. Twelve cases had alive newborns, and 2 cases stillbirths. Fetal/placental weight ratio (FPR)<10th percentile was in 5 cases and FPR>90th percentile in one case. Compared with the normal group, accelerated villus maturation and distal villus dysplasia were more frequently found in pregnancy with aortic dissection group (P<0.05). There was no significant difference in villi infarction and decidua vascular lesions between the two groups (P>0.05), nor was there correlation between the type of aortic dissection and distal villus dysplasia and accelerated villus maturation of placentas (P>0.05). The number of villous interstitial blood vessels in the placentas of pregnancy with aortic dissection group was significantly fewer than that in the normal control group (P<0.01). Conclusions: There are considerable pathological changes in the placentas of pregnant women with aortic dissection/aneurysm. The main histological features are accelerated villus maturation and distal villus dysplasia, which are manifestations of villous ischemia and hypoxia, and also a part of the placental pathological manifestations of maternal vascular dysperfusion.
Pregnancy ; Female ; Infant, Newborn ; Humans ; Infant ; Young Adult ; Adult ; Placenta/pathology* ; Cesarean Section ; Aortic Dissection/surgery* ; Gestational Age ; Aortic Aneurysm/pathology*