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Objective: Loeys-Dietz syndrome (LDS) is associated with a higher risk of aortic dissections (ADs) during pregnancy and postpartum. However, there is limited evidence about the perinatal management of LDS patients who have undergone prophylactic aortic root replacements (ARRs) before conception., Case Report: We present the case of a 28-year-old nulliparous pregnant woman with LDS with a pathogenic variant within exon 5 of TGFBR2 (c.1379G > T, p.[Arg460Leu]), who underwent an ARR at 20 years of age. Cardiac echocardiography did not show any significant changes in the aorta during pregnancy, and her blood pressure remained normal. She had a cesarean section at 37 weeks of gestation. She developed an acute Stanford type B AD extending from the aortic arch to the infrarenal aorta 8 days postpartum and underwent a total arch replacement., Conclusion: This case report suggests that patients with LDS after prophylactic ARRs still possess a risk for Stanford type B ADs., Competing Interests: Declaration of competing interest The authors declare no conflict of interest., (Copyright © 2021. Published by Elsevier B.V.)

Importance: Turner syndrome (TS) is one of the most common chromosomal abnormalities in women. The condition is characterized by gonadal dysgenesis and is associated with structural cardiac abnormalities. Assisted reproductive technology with oocyte donation may be successful but places women with TS at increased risk of aortic dissection and death., Objective: To summarize all cases of aortic dissection associated with pregnancy in women with TS and provide guidance regarding the safety of pregnancy., Evidence Acquisition: Systematic review of PubMed for reports of women with TS, aortic dissection, and pregnancy., Results: There are 14 total reported cases of aortic dissection associated with pregnancy in women with TS. Ten of these cases occurred during pregnancy or in the first month postpartum. The majority of affected pregnancies resulted from oocyte donation, 2 of which were multiple gestations. Two women had a documented history of hypertension, and 3 pregnancies were complicated by preeclampsia. Bicuspid aortic valve and coarctation of the aorta were the most common associated cardiac anomalies. More than half of women had some degree of aortic dilatation. Two women had no identifiable risk factors., Conclusions and Relevance: Women with TS who desire pregnancy must be thoroughly counseled regarding the increased risk of aortic dissection during pregnancy and postpartum. Preconception consultation with maternal-fetal medicine, reproductive endocrinology, and cardiology is necessary along with a comprehensive physical evaluation. If women with TS choose to pursue pregnancy, they require rigorous cardiac monitoring each trimester during pregnancy and postpartum.

Bicuspid aortic valve, one of the most common congenital cardiac malformations, is associated with other valvular abnormalities and aortopathies. It increases the risk of thoracic aortic aneurysm and dissection, infective endocarditis, and sudden cardiac death. Patients require regular surveillance for progressive valvular disease and aortopathy. Although bicuspid aortic valve may occur sporadically, many cases are inherited, so first-degree relatives should be evaluated and genetic counseling considered., (Copyright © 2018 Cleveland Clinic.)

Right atrial (RA) wall dissection and congenital RA aneurysm is a very rare abnormality. This is may be the first reported case of a spontaneous dissection of the RA wall without rupture, in addition to the presence of a congenital RA aneurysm. The patient, a 4-year-old boy, was successfully treated with RA aneurysm resection under cardiopulmonary bypass. Although the diagnosis of RA dissection poses a challenge even when the advanced multimodality imaging approaches are applied, transthoracic and contrast echocardiography are most valuable tools for making accurate diagnosis., (Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

A single coronary artery, arising from the left sinus of Valsalva, associated with a bicuspid aortic valve and annuloaortic ectasia, is very rare. We report on a surgical case of bicuspid aortic valve regurgitation, annuloaortic ectasia, and dilation of the ascending aorta to the aortic arch in a patient with a single coronary artery. We successfully performed aortic root replacement with reimplantation of the single coronary artery and total arch replacement. The reimplantation of the coronary orifice required particular attention. Postoperative CT demonstrated the expected contours from the aortic annulus to the aortic arch with a patent implanted coronary artery.

Congenital diseases of the aorta tend to be obstructive when they present early in life, and aneurysmal when they present later in life. The latter group also tends to be associated with connective tissue disorders and with repaired conotruncal lesions. The indications for intervention in the aneurysm group are still in evolution but are clearly age- and lesion-dependant. Disorders such as Loeys-Dietz syndrome and Turner syndrome may deserve aggressive prophylactic surgery, as well as Marfan syndrome to a lesser extent. The natural history of the dilated aorta after repair of congenital heart lesions is probably more benign than de novo aneurysms and therefore should be treated conservatively., (Copyright © 2015 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Cervical artery dissection is rare in the neonatal period and is most often caused by birth injury during dystocic labor. The severity of this pathology is due to the possibility of serious neurological complications. We report a case of a male newborn who was born vaginally after shoulder dystocia. The extraction was difficult, resulting in a fracture of the right humerus. On the second day of life, the child presented generalized clonic convulsions. Computed tomography of the brain showed an ischemic stroke in the territory of the right middle cerebral artery, the territory of the right posterior cerebral artery, and the right lenticulostriate and capsular regions. Doppler ultrasonography and magnetic resonance angiography showed bilateral carotid artery thrombosis and dissection at the left common carotid artery and its two branches and the right vertebral artery. We discuss the mechanisms of this pathology and we emphasize preventive measures., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Background: There are few families with the diagnosis of ascending aortic aneurysm and acute type-A aortic dissection inherited as an autosomal-dominant disorder in the absence of a known genetic syndrome., Methods: We investigated a family with 26 members in whom ascending aortic aneurysms and acute type-A aortic dissections occurred over three generations. Examinations were performed to identify family members at specific risk., Results: Six members presented with acute type-A aortic dissections and three relatives had ascending aortic aneurysms. Clinical examinations showed no characteristics of a known genetic syndrome. Molecular genetic analysis revealed no mutations known to cause a form of autosomal-dominant inherited aortic disease., Conclusion: Adequate diagnostic measures are mandatory in families with ascending aortic aneurysms or type-A aortic dissections to identify or exclude family members at risk for aortic diseases. Even in the absence of identifiable mutations causing isolated aortic aneurysms or aortic dissections, we recommend standardised examinations of all first-degree relatives of affected families. An indication for prophylactic aortic root replacement should be considered for patients at risk.

Congenital bicuspid aortic valve is a relatively rare malformation. It is reported that the presence of this anomaly predisposes the patient to development of true aortic aneurysms or dissecting aortic aneurysms. Between 1981 and August 1997, 25 patients with an aneurysm of the thoracic aorta associated with congenital bicuspid aortic valve underwent surgical treatment at the authors' institution. There were 20 males and five females. The age of the patients ranged from 27 to 74 years (mean 53 years). There were 18 patients with true ascending aortic aneurysms (of which 10 presented with annulo-aortic ectasia) and seven with dissecting aortic aneurysms (four with DeBakey type I dissection, two with type II and one with type IIIb). These 25 patients constituted 2.6% (25/973) of all cases of surgical operations for aneurysms in the thoracic aorta. Aortic valve dysfunction was noted in 20 patients. The authors performed a valved conduit operation in nine patients, aortic valve replacement and wrapping of the ascending aorta in six, graft replacement of the ascending aorta in five, graft replacement of the ascending aorta and aortic arch in four, and graft replacement of the descending aorta in one. No hospital deaths occurred in the authors' patients. Pathological examination of surgical specimens of the aortic wall showed cystic medial necrosis in 11 patients and mucoid degeneration in nine. In patients with congenital bicuspid aortic valve, attention should be paid to aneurysmal dilatation and aortic dissection as complications in addition to valve dysfunction.

We report the case of a congenital aneurysm of the right sinus of Valsalva dissecting into the interventricular septum in a 21-year-old man. This condition was diagnosed by echocardiography and magnetic resonance imaging. In particular, transthoracic and transesophageal color-coded Doppler echocardiography showed that there was no communication between the aneurysm and the surrounding heart chambers. The findings were confirmed by angiography. Heart enlargement and other symptoms and signs of heart failure indicated surgical repair of the lesion. Early postoperative dehiscence of the sutured orifice was accurately diagnosed with Doppler echocardiography and angiography. Reintervention was successful in filling and sealing the septal cavity. This case confirms the reported difficulties in the surgical correction of aneurysms of sinus of Valsalva lying within the interventricular septum.

Dilatation of the ascending aorta is relatively infrequent during childhood. Besides the Marfan syndrome, the congenital origin should be considered. We report a patient with dilatation of the ascending aorta diagnosed at the age of 10 who presented acute aortic dissection and rupture after a 13-year period of follow-up. Several aspects of the proper diagnosis are discussed.

A male newborn exhibited congenital cutis laxa, bone disease and multiple fractures, and lethal ruptured arterial dissections. At post-mortem examination, the histologic elastic tissue of skin and arteries was dysplastic. The bone fragility is unexplained.