Reduced maternal vitamin A status increases the incidence of normal aortic arch variants.

While common in the general population, the developmental origins of "normal" anatomic variants of the aortic arch remain unknown. Aortic arch development begins with the establishment of the second heart field (SHF) that contributes to the pharyngeal arch arteries (PAAs). The PAAs remodel during subsequent development to form the mature aortic arch and arch vessels. Retinoic acid signaling involving the biologically active metabolite of vitamin A, plays a key role in multiple steps of this process. Recent work from our laboratory indicates that the E3 ubiquitin ligase Hectd1 is required for full activation of retinoic acid signaling during cardiac development. Furthermore, our study suggested that mild alterations in retinoic acid signaling combined with reduced gene dosage of Hectd1, results in a benign aortic arch variant where the transverse aortic arch is shortened between the brachiocephalic and left common carotid arteries. These abnormalities are preceded by hypoplasia of the fourth PAA. To further explore this interaction, we investigate whether reduced maternal dietary vitamin A intake can similarly influence aortic arch development. Our findings indicate that the incidence of hypoplastic fourth PAAs, as well as the incidence of shortened transverse arch are increased with reduced maternal vitamin A intake during pregnancy. These studies provide new insights as to the developmental origins of these benign aortic arch variants.

Surgical Repair of Cervical Aortic Arch With Brain Circulation Anomaly Through Clamshell Incision.

We report the successful surgical repair of a cervical aortic arch and diverticulum with a brain circulation anomaly through a clamshell incision. Because of the reliability of selective antegrade cerebral perfusion and superior exposure, we chose an approach through a clamshell incision. We describe the utility of this approach for treating a cervical aortic arch with a diverticulum.

Rosuvastatin prevents aortic arch plaque progression and improves prognosis in ischemic stroke patients.

OBJECTIVES: Complicated aortic arch plaques (CAP) and their progression are important for recurrent ischemic stroke (IS) and its prognosis. We investigated the effects and clinical benefits of rosuvastatin therapy on this pathophysiology. The purpose of this study was to investigate whether rosuvastatin prevention of aortic arch plaque progression improved the prognosis of IS patients. METHODS: Ninety-seven consecutive acute cerebral embolism patients were retrospectively surveyed. All had transesophageal echocardiography (TEE) to assess the presence or absence of CAP, defined as aortic wall thickness ≥4 mm or plaque ulceration. Patients received conventional antithrombotic therapy as clinically indicated. All patients with CAP were recommended to receive 5 mg rosuvastatin/day, administered by their attending physicians; not all physicians followed this recommendation. Six-month follow-up TEEs were performed in patients with CAP who received rosuvastatin. Major adverse cerebrovascular events (MACEs) comprised recurrent IS and death. RESULTS: CAP was detected in 39 patients (40%), and MACEs in 15. Multivariate regression analysis showed that patients with CAP not taking rosuvastatin was an independent risk factor for MACEs (odds ratio = 18.044; 95% confidential interval = 2.089-155.846, p < 0.01). When patients were divided into three groups: those with CAP taking rosuvastatin, those with CAP not taking rosuvastatin, and those without CAP, Kaplan-Meier analysis demonstrated that patients with CAP not taking rosuvastatin had significantly more MACEs than those in the other two groups (long-rank test; χ2 = 6.553, p < 0.05). Six-month TEE follow-ups in the 26 patients with CAP taking rosuvastatin showed significant improvement in CAP diameter with improved lipid profiles; 88% (23/26 patients) showed no morphological CAP progression; 15 of these showed CAP regression. DISCUSSION: Rosuvastatin therapy prevented aortic arch plaque progression in IS patients with CAP, and may also have long-term clinical benefits.

Extracranial giant cell arteritis: A narrative review.

A systematic literature search was performed to summarise current knowledge on extracranial giant cell arteritis (GCA), i.e. large-artery involvement in patients with or without clinically apparent temporal arteritis (cranial GCA). Extracranial GCA is increasingly recognised, both in patients with cranial GCA and with solitary extracranial GCA, due to increased awareness among physicians and development of modern imaging modalities. The literature on the pathogenesis and histopathology of extracranial GCA is scarce. It is considered to be similar to cranial GCA. Patients with solitary extracranial GCA often present with non-specific signs and symptoms, although vascular manifestations, mostly secondary to stenosis, may occur. Due to the non-specific clinical presentation and low sensitivity of temporal artery biopsies, extracranial GCA is usually diagnosed by imaging. 18F-FDG-PET, MRI, CT angiography and ultrasound are used for this purpose. At present, the optimal diagnostic strategy is undetermined. The choice for a particular modality can be guided by the clinical scenario that raises suspicion of extracranial GCA, in addition to local availability and expertise. Extracranial complications in GCA consist of aortic aneurysm or dissection (mainly the ascending aorta), aortic arch syndrome, arm claudication and posterior stroke (although this is technically a cranial complication, it often results from stenosis of the vertebrobasilar arteries). Mortality is generally not increased in patients with GCA. Treatment of patients with solitary extracranial and those with extracranial and cranial GCA has been debated in the recent literature. In general, the same strategy is applied as in patients with temporal arteritis, although criteria regarding who to treat are unclear. Surgical procedures may be indicated, in which case optimal medical treatment prior to surgery is important.

Fetuses with right aortic arch: a multicenter cohort study and meta-analysis.

OBJECTIVES: Use of recent antenatal screening guidelines for cardiac abnormalities has increased fetal diagnoses of right aortic arch (RAA). We aimed to establish the outcome of fetal RAA without intracardiac abnormalities (ICA) to guide postnatal management. METHODS: In the retrospective cohort part of our study, outcome measures were rates of chromosomal abnormalities, 22q11.2 deletion, fetal extracardiac abnormalities (ECA), postnatal ICA and ECA, and symptoms of and surgery for vascular ring. A systematic review and meta-analysis was also performed; results are reported as proportions. Kaplan-Meier analysis of vascular ring cases with surgery as endpoint was performed. RESULTS: Our cohort included 86 cases; 41 had a vascular ring. Rates of chromosomal abnormalities, 22q11.2 deletion and fetal ECA were 14.1%, 6.4% and 17.4%, respectively. Sixteen studies including our cohort (312 fetuses) were included in the systematic review. Overall rates of chromosomal abnormalities and 22q11.2 deletion were 9.0% (95% CI, 6.0-12.5%) and 6.1% (95% CI, 3.6-9.3%), whilst the respective rates for cases with no ECA were 4.6% (95% CI, 2.3-7.8%) and 5.1% (95% CI, 2.4-8.6%). ECA were seen in 14.6% (95% CI, 10.6-19.0%) prenatally and in 4.0% (95% CI, 1.5-7.6%) after birth. Postnatal ICA were identified in 5.0% (95% CI, 2.7-7.9%). Rate of symptoms of vascular rings (follow-up ≥ 24 months postpartum) was 25.2% (95% CI, 16.6-35.0%), and 17.1% (95% CI, 9.9-25.7%) had surgery. Two-year freedom from surgery was 83.0% (95% CI, 74.3-90.1%). CONCLUSIONS: Fetal RAA without ICA is more frequently associated with ECA than with chromosomal abnormalities. Most cases, however, are isolated. Vascular-ring symptoms occur in about 25% of cases. Postnatal surveillance is required mainly in the first 2 years after delivery.

Double aortic arch: postnatal obliteration of the left aortic arch. Is arterial duct closure responsible?

We present a case of double aortic arch with a predominant right and a double arterial duct detected by echocardiogram in a 28-week gestation foetus. The first evaluation revealed that both arches were perfused; the 1-month postnatal echocardiogram showed the closure of both arterial ducts and the partial obliteration of the left aortic arch between the left subclavian artery and the dorsal aorta. In our case, the postnatal obliteration of the left arch in a double aortic arch was probably due to the closure of the left-sided arterial duct.

[Mid-aortic syndrome in 18 year-old woman with arterial hypertension and bilateral renal artery stenosis]. / Zespól aorty brzusznej u 18-letniej chorej z nadcisnieniem tetniczym i obustronnym zwezeniem tetnic nerkowych.

Mid-aortic syndrome (MAS) is characterised by narrowing of the abdominal aorta, often with involvement of the renal and splanchnic arterial branches. Although uncommon, MAS is an important cause of renovascular hypertension in children and adolescents and should be considered in the differential diagnosis of hypertension . Hypertension is typically severe and often difficult to manage. The management of MAS should always be individualised and may include percutaneous transluminal renal angioplasty, stent implantation or surgical revascularisation. We present a 18 year-old woman with hypertension and MAS coexisting with bilateral renal artery stenosis who underwent left renal artery angioplasty and than was followed-up for one year.

[Management of acute aortic syndromes]. / Tratamiento de los síndromes aórticos agudos.

Acute aortic syndrome (AAS) describes the acute presentation of patients with characteristic "aortic pain" caused by one of several life threatening thoracic aortic conditions including aortic dissection, intramural hematoma or penetrating atherosclerotic ulcer. Recent advances in imaging and therapeutic techniques have emphasized the importance of early diagnosis of acute aortic syndrome because it is crucial for survival. Not just because the cardiovascular community knows little, the management of AAS remains a therapeutic challenge, while diverse surgical and percutaneous strategies for the treatment of aortic syndromes are continuously evolving. As a result of increasing knowledge and better management strategies in this area, the outcomes of patients treated for acute aortic syndromes have improved. This review discusses the etiology and clinical presentation, but mainly focuses on modern management and both established and emerging therapeutic approaches to acute aortic syndromes.

Stenosis of the upper body arteries in association with coarctation and interrupted aortic arch.

Coarctation of the aorta and interrupted aortic arch are not usually associated with stenosis of the innominate, common carotid, or subclavian arteries. We report two patients with stenoses of combinations of these arteries, one in association with coarctation (stented later in life) and the other in association with interrupted arch (arterial stenoses surgically repaired at the time of surgery for the arch proper). Both have done well.

Transesophageal echocardiographic diagnosis of Aspergillus fumigatus aortitis after percutaneous coronary intervention.

Aspergillus aortitis is an uncommon infection with high mortality and has been reported in patients after cardiopulmonary bypass. We report the first case of Aspergillus aortitis in an immunocompetent man immediately after percutaneous coronary intervention to an aortocoronary bypass graft. In this case, transesophageal echocardiography played a pivotal role in diagnosis.

[Imaging diagnosis in acute aortic syndromes]. / Diagnóstico por imagen en el síndrome aórtico agudo.

Acute aortic dissection is a clinical emergency. Its prognosis is related mainly with prompt and accurate diagnosis, as well as rapid treatment. In this paper we review the importance of different imaging techniques in the diagnosis of patients with acute aortic syndrome. Aortic dissection, intramural haematoma, and penetrating aortic ulcer are discussed.