Flanged Bentall and Cabrol Procedures for Complex Aortic Root Diseases.

For complex aortic root lesions, the flanged Bentall procedure has more advantages than the traditional one. Here, we report two patients with complex root lesions treated with the flanged Bentall and Cabrol procedure: one was a 25-year-old male with interventricular septal dissection with Behçet's disease, and the other was a 4-year-old female with a very large ascending aortic aneurysm with a small annulus and Loeys-Dietz syndrome. Both patients recovered uneventfully and obtained good short-term results.

Successive surgical repair of a progressive aortic dissection in a case of Loeys-Dietz Syndrome.

INTRODUCTION: The Frozen Elephant Trunk technique is a well-established treatment for aortic dissections (Stanford Type A) involving the aortic arch and descending aorta. The Thoraflex™ Hybrid prosthesis (Vascutek Ltd.), consisting of a proximal flexible conduit and a distal self-expanding covered stent, has consistently shown positive results in the treatment of this condition. CASE DESCRIPTION: The following is a description of such a staged reconstruction using the Thoraflex™ Hybrid Ante-Flo™ device, performed in a patient previously diagnosed with Loeys-Dietz Syndrome. After clamping the aorta proximally, an incision was taken at the distal end of the stent. Here, the distal end of the new prosthesis was inserted into the true lumen of the descending aorta and the stent was deployed. Following this, a bypass was established via the left atrium, and blood was returned to the lower body using the perfusion arm of the prosthesis with the proximal part of the descending aorta clamped. The collar of the prosthesis was sutured proximally to the aorta near the inlying previous stent. Air was removed via the perfusion arm of the prosthesis, which was then oversewn. DISCUSSION: To date, hybrid prostheses have only been used on the proximal aorta in patients with aortic aneurysms and aortic dissections (Stanford Type A). CONCLUSION: Using the described technique, it is possible to reconstruct the aortic anatomy using the available prostheses with accuracy and minimal complications.

Repair of an aneurysm of the ascending aorta and arch in an infant with Loeys-Dietz syndrome.

Aortic aneurysms in childhood are rare disease entities and are usually seen in patients with genetic connective tissue disorders such as Marfans, Ehler-Danlos, and Loeys-Dietz syndrome (LDS). Patients affected with LDS present early in life and have a rapid disease progression. We report a case of repair of an ascending and aortic arch aneurysm in an infant with Loeys-Dietz syndrome.

Late Peripheral Thoracic Aneurysms following Aortic Root Surgery in Patients with Loeys-Dietz Syndrome.

The Loeys-Dietz syndrome is a multisystem connective tissue disorder characterized by aortopathy, arterial tortuosity, peripheral aneurysms, and skeletal features. The peripheral arteriopathy is an important cause of morbidity and potential mortality. This report presents 2 cases: the first demonstrating a 5-cm pseudoaneurysm of the right internal mammary artery and the second demonstrating a 2.3-cm aneurysm of the left internal mammary artery, each following aortic root surgery. These were successfully treated with percutaneous techniques. No complications were seen at follow-up as long as 2 years. Patients with Loeys-Dietzs syndrome require comprehensive long-term vascular follow-up and are likely to require percutaneous vascular interventions.

Endovascular Repair of Descending Thoracic Aorta in Loeys-Dietz II Syndrome.

Loeys-Dietz syndrome (LDS) is an autosomal dominant disorder that is predominantly characterized by involvement of the aorta, manifesting as aneurysmal dilatation or aortic dissection. Patients with LDS manifest with spontaneous aneurysms and dissections of central and peripheral arterial beds. We present 2 cases of young male patients with Loeys-Dietz II aortopathy, who manifested with spontaneous intimal tear of descending thoracic aorta and contained aortic rupture. Both patients were managed by endovascular repair, with collaborative efforts of teams comprising interventional cardiologists and radiologists, and a vascular surgeon.

Endovascular repair of an internal mammary artery aneurysm in a patient with Loeys-Dietz syndrome.

Loeys-Dietz syndrome is a condition that predisposes the development of central and peripheral arterial aneurysms and dissections. The standard approach for treatment of these lesions has been open repair. We present the case of a 34-year-old woman with a previous history of multiple open aortic repairs who presented with a right large internal mammary artery aneurysm. The patient was successfully treated with endovascular coil embolization. This case report demonstrates the feasibility of using endovascular interventions to treat unusual aneurysms in patients with Loeys-Dietz syndrome.

Complete Aortic Replacement in a Patient With Loeys-Dietz Syndrome.

Background: LDS is an autosomal dominant connective tissue disease. It is a rare multi-systemic disorder with serious vascular impact. Case report: We report a case of a 38-year-old male with Loeys-Dietz syndrome (LDS) suffering from major aortic complications. The disease initially manifested itself as a type A aortic dissection, successfully treated by open ascending aorta replacement. Ten days later, the patient developed an uncomplicated type B dissection. During follow up, the patient became symptomatic in both legs (rest pain) due to major true lumen compression. A thoracic endovascular aortic repair was performed with immediate improvement of the symptoms. During follow up, a computer tomography angiogram, showed a persistence false lumen perfusion and an aortic diameter increase. Multiple additional endovascular procedures and a final open thoracoabdominal aortic replacement were needed to exclude completely the false lumen. Conclusion: Open surgical repair is still the gold standard therapy for patients with connective tissue disease. However, with the nowadays progress, hybrid procedures could be a better option.

Treatment of chronic chest wall pain in a patient with Loeys-Dietz syndrome using spinal cord stimulation.

OBJECTIVE: Spinal cord stimulation (SCS) has been used with increased frequency for the treatment of intractable pain including chronic chest pain. METHODS: The patient with a history of Loeys-Dietz syndrome who underwent repair of pectus excavatum with subsequent chronic chest wall pain underwent an uneventful SCS trial with percutaneous epidural placement of two temporary eight-electrode leads placed at the level of T3-T4-T5. RESULTS: After experiencing excellent pain relief over the next two days, the patient was implanted with permanent leads and rechargeable generator four weeks later and reported sustained pain relief at 12-month follow-up visit. CONCLUSION: SCS provides an effective, alternative treatment option for select patients with Loeys-Dietz syndrome who underwent repair of pectus excavatum with subsequent chronic chest wall pain who have failed conservative treatment. SCS may provide pain relief with advantages over conservative treatments and more invasive techniques.

Postpartum Stanford type B aortic dissection in a woman with Loeys-Dietz syndrome who underwent a prophylactic aortic root replacement before conception: A case report.

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.

Two-stage total aortic replacement for Loeys-Dietz syndrome.

Loeys-Dietz syndrome (LDS) is a recently described connective tissue disorder characterized by generalized arterial tortuosity and aggressive aortopathy that untreated leads to early death even at aortic dimensions as small as 4 cm. We report the case of a young man with LDS successfully treated for aortic root, arch, and thoracoabdominal pathology.

A contemporary approach to a young female patient with Loeys-Dietz syndrome and an uncomplicated type B aortic dissection: a case report.

BACKGROUND: Aortic dissection is a relatively uncommon, but often catastrophic disease that requires early and accurate diagnosis. It often presents in patients with congenital connective tissue disorders. The current aortic surgical techniques are related with serious early and late complications. This case report emphasizes the importance of early diagnosis of aortic root dilatation and the risk of dissection, especially in patients with congenital connective tissue disorders. We present an alternative, contemporary and multidisciplinary approach based on the present state of knowledge. CASE PRESENTATION: We present a rare case of a young female patient with Loeys-Dietz syndrome who was admitted with an uncomplicated aortic dissection (Stanford type B / DeBakey type III) and a dilated aortic root. After a period of close surveillance and extensive vascular imaging, thoracic endovascular aortic repair was deemed to be technically not possible. Medical treatment was optimized and our patient successfully underwent a personalised external aortic root support procedure (PEARS) as a contemporary alternative to existing aortic root surgical techniques. CONCLUSIONS: This case highlights the importance of interdisciplinary approach, close follow-up and multimodality imaging. The decision to intervene in a chronic type B aortic dissection is still challenging and should be made in experienced centers by an interdisciplinary team. However, if an acute complication occurs, thoracic endovascular aortic repair TEVAR is the method of choice. In all cases optimal medical treatment is important. There is increasing evidence that personalized external aortic root support procedure PEARS is effective in stabilizing the aortic root and preventing its dilatation and dissection not only in patients with Marfan syndrome, but also in other cases of aortic root dilation of other etiologies. Moreover, many publications have reported the additional benefit of reduction or even eradication of aortic regurgitation by improving coaptation of the aortic valve leaflets in dilated aortas.

Clip ligation for ruptured intracranial aneurysm in a child with Loeys-Dietz syndrome: case report.

The authors present the case of a pediatric patient with Loeys-Dietz syndrome (LDS) who underwent craniotomy for clip ligation of a ruptured intracranial aneurysm. To the authors' knowledge, this is the youngest reported patient with LDS who has been treated for a ruptured intracranial aneurysm. The patient presented with aneurysmal subarachnoid hemorrhage even though the results of surveillance screening were negative, and the aneurysm arose from the wall of the parent artery away from an arterial branch point. She was treated with open clip ligation and recovered well. The authors review the other reported cases of treated intracranial aneurysms in patients with LDS.

Endovascular treatment of intracranial aneurysms in Loeys-Dietz syndrome.

BACKGROUND: Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder characterized by extensive arterial aneurysms. The successful clipping of intracranial aneurysms in patients with LDS has been reported in a limited number of patients but endovascular treatment of intracranial aneurysms in LDS has never before been described. The first successful cases of endovascular management of five intracranial aneurysms in two patients with LDS are reported. CLINICAL PRESENTATION: The first patient was an asymptomatic 45-year-old woman with LDS and two incidentally discovered, unruptured wide necked aneurysms (6×5 mm and 4×2 mm) arising from the ophthalmic segment of the left internal carotid artery. Both aneurysms were successfully embolized via Neuroform stent assisted coiling. The second patient was an asymptomatic middle 40s woman with LDS found to have an unruptured anterior communicating artery aneurysm (7×4 mm) as well as two ophthalmic segment aneurysms (2.5×3.7 mm and 2.9×3.5 mm). All three aneurysms were successfully embolized via Neuroform stent assisted coiling in two staged procedures. There were no periprocedural complications in either patient. Long term follow-up imaging demonstrated durable embolization in all aneurysms in both patients. CONCLUSION: This is the first reported case series of endovascular management of intracranial aneurysms in the setting of LDS. The endovascular approach was safe, effective and durable in all aneurysms in both patients, and should be considered when treating patients with LDS and intracranial aneurysms.

Clipping of a cerebral aneurysm in a patient with Loeys-Dietz syndrome: case report.

BACKGROUND AND IMPORTANCE: his is the first case report of clipping a cerebral aneurysm in a patient with Loeys-Dietz syndrome (LDS). LDS is a newly described autosomal dominant connective tissue disease with systemic vascular involvement. Unique to this syndrome is the development of aneurysms at a young age with the propensity of dissection or rupture at a stage that is earlier than when surgical intervention is typically indicated. We describe the nuances in intraoperative and postoperative management. CLINICAL PRESENTATION: A 31-year-old woman who recently received a diagnosis of with LDS type II presented to neurosurgical attention for management of an unruptured right ophthalmic artery aneurysm. The patient underwent a right pterional craniotomy for clipping of the aneurysm, with lumbar drain placement before the procedure. Papaverine had to be used several times to counteract vasospasm of the vessels during arachnoid dissection. Because of vascular reactivity, temporary clipping was not used, and the aneurysm was clipped successfully. CONCLUSION: LDS is a newly described disorder that warrants awareness in the neurosurgical community because of its association with intracerebral aneurysms as well as craniosynostosis (19%), scoliosis (20%), cervical spine instability (7%), hydrocephalus, and Arnold-Chiari malformation. When clipping aneurysms in these patients, the surgeon should be aware of the potential for severe vascular reactivity during dissection and avoid temporary clipping when possible. Avoidance of lumbar drainage intraoperatively reduces the risk of intracranial hypotension after removal.