Management of a pregnant woman with Marfan syndrome and aortic root and aberrant right subclavian artery aneurysm: a case report
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PROVIDER: S-EPMC11337004 | biostudies-literature | 2024 Aug
REPOSITORIES: biostudies-literature
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Project description:Right aortic arch with Kommerell's diverticulum is a very rare situation. Surgical treatment is recommended for symptomatic patients or asymptomatic patients with a large diverticulum. However planning the strategy of operation is difficult without a 3D imaging. We report a case of a 57-year-old man with right aortic arch, Kommerell's diverticulum, and aberrant left subclavian artery. After a 3D-CT imaging, the patient underwent descending aortic replacement without reconstruction of aberrant left subclavian artery. After operation, there was no signs or symptoms of ischemia of the left arm. If the reconstruction of the aberrant subclavian artery was too difficult, closing its orifice is an acceptable decision. It has been found advantageous because of a decrease blood loss and a shorter cardiopulmonary bypass duration. If an ischemia of the arm is noticed, additional reconstruction will have to be considered. 3D-CT imaging was very useful to have a proper orientation and plan for the operative strategy.
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Project description:Down syndrome (DS) may be associated with various organ system disorders. Feeding problems are frequent in children with DS and may be caused by associated defects, including congenital heart defects, gastrointestinal defects, or endocrine disorders. In the absence of these associated conditions, feeding problems are often attributed to general hypotonia. However, an aberrant right subclavian artery (ARSA), a rare vascular anomaly and an unusual cause of problems with the passage of solid food through the esophagus, has recently been suggested to occur more frequently in patients with DS. This knowledge is of importance when evaluating feeding difficulties in patients with DS. Additional investigation for identifying an ARSA may be indicated in selected patients. Diagnostic techniques, such as transthoracic echocardiography, barium contrast esophagram, angiography, or computed tomography-angiography (CT) can be used in a diagnostic flow chart. The presence of ARSA is not synonymous to the cause of feeding problems in patients with DS and corrective surgery of this vascular anomaly should be restricted to selected cases.
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Project description:Vascular compression of the esophagus by an aberrant right subclavian artery (aRSA) leading to dysphagia is a rare occurrence. There has been a significant advancement in the diagnostic and surgical treatment modalities available for this disorder. Anesthetic management has evolved too and this case report highlights the anesthetic management of a 41-year-old woman presenting with symptoms of dysphagia because of compression of esophagus by an aRSA, who subsequently underwent re-implantation of aRSA into ascending aorta.
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Project description:An aberrant right subclavian artery (ARSA) is the most common congenital variant of the aortic arch. Usually, this variation is largely asymptomatic, but sometimes it may be involved in aortic dissection (AD). Surgical management of this condition is challenging. The therapeutic options have been enriched in recent decades by establishing individualized endovascular or hybrid procedures. Whether these less invasive approaches bear advantages, and how they have changed the treatment of this rare pathology, is still unclear. Therefore, we conducted a systematic review. We performed a review of literature from the past 20 years (from January 2000 until February 2021) complying with the Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines. All reported patients treated for Type B AD in the presence of an ARSA were identified and classified into three groups according to the received therapy (open, hybrid, and total endovascular). Patient characteristics, as well as in-hospital mortality, and major and minor complications were determined and statistically analyzed. We identified 32 relevant publications comprising 85 patients. Open arch repair has been offered to younger patients, but significantly less often in symptomatic patients needing urgent repair. Therefore, the maximum aortic diameter was also significantly larger in the open repair group compared with that in the hybrid or total endovascular repair group. Regarding the endpoints, we did not find significant differences. The literature review revealed that open surgical therapies are preferred in patients presenting with chronic dissections and larger aortic diameters, most likely because they are unsuitable for endovascular aortic repair. Hybrid and total endovascular approaches are more often applied in emergency situations, where aortic diameters remain smaller. All therapies demonstrated good, early, and midterm outcomes. But, these therapies carry potential risks in the long term. Therefore, long-term follow-up data are urgently needed to validate that these therapies are sustainable.
| S-EPMC9970753 | biostudies-literature
Project description:BackgroundThe coexistence of a right-sided aortic arch (RAA), an aberrant left subclavian artery (ALSA), and a patent ductus arteriosus (PDA) is a rarely seen vascular ring anomaly. There is currently no general guideline consensus on the management and follow-up of this congenital defect, posing a challenge to the clinicians. At this point, the heart team plays a critical role in the management of the disease.Case summaryIn the present case, a 25-year-old male patient was presented to the outpatient clinic with dyspnoea and fatigue. A transthoracic echocardiography revealed PDA with a left-to-right shunt. To evaluate the anatomy thoroughly, a thoracic computed tomographic angiography was performed and showed PDA accompanying ALSA and RAA. The patient was evaluated by the Heart Team, and a percutaneous closure of PDA was recommended due to signs of left ventricular volume overload. The closure was successfully performed with Amplatzer vascular plug II. At follow-up, the patient was free of symptoms.DiscussionClinicians should be aware of the potential concomitant lesions during the diagnostic work-up. In selected patients, percutaneous closure of PDA may be the first-line therapy in experienced centres.
| S-EPMC9194800 | biostudies-literature
Project description:A 13-year-old spayed female cocker spaniel was presented with a 2-month history of swelling in several digits and intermittent hindlimb lameness. Radiographs revealed marked soft-tissue swelling and periosteal new bone formation without cortical bone destruction, characteristic of hypertrophic osteopathy (HO), in the distal parts of all extremities except for the right forelimb. However, no notable findings were detected in thoracic radiographs. An ultrasonography indicated cranial bladder wall thickening, which resolved following antibiotic therapy. Computed tomographic angiography identified a potential underlying cause as an aberrant right subclavian artery (ARSA) originating from the aortic arch, compressing the esophagus and causing mild esophageal cranial dilation to the aberrant vessel. No other intrathoracic or neoplastic lesions were observed. Gastrointestinal symptoms, such as regurgitation, were absent. Although an ARSA was likely the cause of HO, surgical correction was declined by the owner. To the best of our knowledge, this is the first reported case of HO concurrent with ARSA in dogs.
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Project description:Fetal aberrant right subclavian artery (ARSA) is a relatively common sonographic finding. Congenital heart disease (CHD) is the most common structural abnormality in patients with ARSA. We aimed to assess the prevalence of genetic abnormalities, particularly sequence variants, in fetuses with CHD and ARSA. By clinical phenotyping and genomic sequencing, we retrospectively reviewed all fetuses with a prenatal diagnosis of CHD combined with ARSA at a single center. As a result, we identified 30 fetuses with ARSA combined with CHD, with conotruncal anomalies being the most common (n = 12, 40%), followed by left ventricular outflow tract obstruction (n = 6, 20%) and atrioventricular septal defects (n = 6, 20%). Overall, 18 (60%) cases had a genetic diagnosis. Copy number variation sequencing analysis identified six (20%) fetuses with aneuploidy and seven (23%) with pathogenic copy-number variants. Whole-exome sequencing (WES) analysis of the remaining 17 cases revealed diagnostic genetic variants in five (29%) cases, indicating that the diagnostic yield of WES for the entire cohort was 17% (5/30). Our findings reveal the high burden of genetic abnormalities in fetal CHD with ARSA. Single-gene disorders contribute substantially to the genetic etiology of fetal CHD with ARSA.
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