Weak Femoral Pulses Research Articles

Middle Aortic Syndrome in a 68‐Year‐Old Woman

To the Editor: Middle aortic syndrome (MAS) is a rare condition characterized by a narrowing of the abdominal aorta and its branches; it typically presents with renovascular hypertension in children and young adults. Herein is described a case of infrarenal MAS in a 68-year-old woman who was otherwise asymptomatic. It was encountered incidentally during an examination of a T12 osteoporotic compression fracture. A 68-year-old woman was transferred to the hospital for a T12 osteoporotic compression fracture that was subsequently confirmed using computed tomography (CT) and magnetic resonance imaging (MRI) of the thoracolumbar spine. Because her backache was severe, an immediate vertebroplasty was scheduled. She was otherwise asymptomatic. Her medical history was unremarkable except for a 10-year history of hypertension, which was effectively controlled with amlodipine 5 mg/d. On routine physical examination, weak bilateral femoral and pedal pulses were noted; her blood pressure was 120–110/80–70 mmHg in the upper extremities and 90–80/70–50 mmHg in the lower extremities. Duplex ultrasound scanning of the lower extremity arteries showed a bilateral pulsus parvus tardus pattern with no obstructive lesion, suggesting a stenosis above the aortoiliac arteries. Review of the spinal CT and MRI (Figure 1) revealed a narrowing of the infrarenal abdominal aorta that had initially been overlooked. The aorta was found to have a segmental tubular stenosis (35 mm long, 8 mm in diameter) just below the origin of the renal arteries. The aorta below the stenotic segment exhibited generalized hypoplasia, and the iliac system was also small, with diameters of 8 to 9 mm at each common iliac artery. The ascending aorta, aortic arch, and descending thoracic aorta were normal. There was no obvious stenosis in the renal arteries, celiac trunk, superior mesenteric artery (SMA), or inferior mesenteric artery (IMA). Compensatory dilation of the SMA and IMA was noted, which allows collateral flow to the legs, and she denied claudication or weakness in her legs. Routine blood tests were all normal except for slightly high C-reactive protein (1.1 mg/L; normal range <0.8 mg/L). With these findings, the diagnosis of MAS was reached. A percutaneous vertebroplasty for T12 was performed uneventfully under fluoroscopy guidance. Her backache improved immediately and dramatically after the operation. Because she was asymptomatic, and her hypertension was well controlled with lower doses of a single drug, no additional vascular examination was planned. She was discharged home the day after the procedure. During follow-up over 12 months, at 1-month intervals for the prescription of alendronate, she was still asymptomatic and maintained normal blood pressure while taking amlodipine 5 mg/d. MAS is a clinico-anatomical classification often used to describe segmental narrowing of the abdominal or distal descending thoracic aorta and ostial stenosis of its major branches, irrespective of its etiology and pathogenesis.1, 2 The most frequent manifestations of MAS are uncontrolled renovascular hypertension and arterial insufficiency of the lower extremities.1-4 Renovascular hypertension is particularly severe in individuals with suprarenal and interrenal involvement. If the stenosis is below the renal arteries, leg discomfort is the primary symptom.3, 5 Lower extremity ischemia occurs less frequently, probably secondary to the rich collateral circulation that develops distal to the stenotic region of the aorta.4, 6 In the present case, the patient showed lower body hypotension but no claudication. This is most likely due to collateral pathways between the SMA and IMA. A narrowing of the aorta below the renal arteries does not lead to life-threatening renovascular hypertension and therefore has a much better prognosis.6, 7 The infrarenal type may occur more commonly than reported because these individuals often remain asymptomatic and do not seek treatment.5 This observation seems to be confirmed in this patient. The diagnosis of MAS is usually made during childhood or young adulthood, although it may present at any age.1-4 There have been a few reported cases occurring in individuals aged 60 and older with related symptoms.6, 8 The current patient is remarkable for two reasons; she is the second oldest reported, and she had no symptoms and the MAS was found incidentally. It may be that severe stenosis presents at an early age, whereas less-severe stenosis presents later. In older adults, diminished blood flow distal to the stenosis may manifest as a pulse differential between the arms and legs and represent the first clinical manifestation of MAS, as in this case. In conclusion, MAS, although rare in elderly adults, should be considered when differential blood pressures or weak femoral pulses are present. Conflict of Interest: The editor in chief has reviewed the conflict of interest checklist provided by the authors and has determined that the authors have no financial or any other kind of personal conflicts with this paper. Author Contributions: Paik and Seo were both involved in analysis and interpretation of data and preparation of manuscript. Sponsor's Role: None.

Index of Suspicion in the Nursery

Article| September 01 2012 Index of Suspicion in the Nursery Author DisclosureDrs Abdelhamid, Castillo, Kumar, Joseph, and Rastogi have disclosed no financial relationships relevant to this article. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device Neoreviews (2012) 13 (9): e551–e555. https://doi.org/10.1542/neo.13-9-e551 Views Icon Views Article contents Figures & tables Video Audio Supplementary Data Peer Review Share Icon Share Twitter LinkedIn Tools Icon Tools Get Permissions Cite Icon Cite Search Site Citation Index of Suspicion in the Nursery. Neoreviews September 2012; 13 (9): e551–e555. https://doi.org/10.1542/neo.13-9-e551 Download citation file: Ris (Zotero) Reference Manager EasyBib Bookends Mendeley Papers EndNote RefWorks BibTex toolbar search nav search search input Search input auto suggest search filter All PublicationsAll JournalsNeoReviewsPediatricsHospital PediatricsPediatrics In ReviewAAP Grand RoundsAAP NewsAll AAP Sites Search Advanced Search Subjects: Hematology/Oncology Topics: chest x-ray, esophageal atresia, heart, nurseries, pericardial effusion, vacterl association with hydrocephalus, down syndrome, hypothyroidism, myeloproliferative syndrome, transient, newborn A boy was born by spontaneous vaginal delivery at 36 weeks’ gestation weighing 2,770 g, with a head circumference of 31.8 cm, to nonconsanguineous white parents. Mother is a 31-year-old primigravida, rubella-immune, with negative serology for hepatitis B and C and HIV, and was treated with sertraline for depression. Antenatal scans, including fetal echocardiography, showed no abnormality. The infant was born in good general condition needing no resuscitation. Apgar scores were 7 at 1 minute and 10 at 5 minutes with normal cord blood gases. However, the placenta was noted to be edematous. A few minutes later, he started to show signs of respiratory distress and significant pallor. Initial examination revealed a temperature of 36.1°C, muffled heart sounds, heart rate of 115 beats per minute, capillary refill time of 3 to 4 seconds, nonrecordable blood pressure, equally weak femoral pulses bilaterally, respiratory rate of 76 breaths per minute, clear chest,... You do not currently have access to this content.

Persistent sciatic artery aneurysm with limb-threatening ischemia

A 50 year old lady with bilateral persistent sciatic artery aneurysm presented with severe limb ischemia due to distal embolization from a thrombus filled persistent sciatic artery aneurysm on one side. Persistent sciatic artery is a rare embryological anomaly which results due to the failure of development of the femoral system. Its complete variant can be clinically recognised by the presence of a weak femoral pulse with a normal popliteal pulse. The diagnostic and treatment considerations of this rare anomaly are described.

Pulmonary hypertension associated with Ehrlichia canis infection in a dog

CANINE monocytic ehrlichiosis (CME) is an important canine disease with a worldwide distribution. The clinical presentation can be acute, subclinical or chronic, and there may be a multitude of clinical...

Letters to Editor Hypertension without renovascular stenosis: a rare case of abdominal aorta coarctation treated with percutaneous transluminal angioplasty

Letters to Editor Hypertension without renovascular stenosis: a rare case of abdominal aorta coarctation treated with percutaneous transluminal angioplasty

Treatment for coarctation of the aorta: where are we and where do we go from here?

Department of Paediatric Cardiology, Evelina Children’s Hospital, Guy’s & St Thomas NHS Foundation Trust, Westminster Bridge Road, London, SE1 7EH, UK Tel.: +44 207 188 4562 thomas.krasemann@gstt.nhs.uk Coarctation of the aorta Coarctation of the aorta is typically a stenosis of the descending aorta in the area of the ductus arteriosus Botalli and caused by ductal tissue in the aortic wall. Once the duct constricts postnatally, this tissue constricts as well and leads to the typical posterior shelf, which is visible on echocardiography. Aortic coarctation is found in approximately 1:3000 newborns and, if left untreated, reduces the life expectancy to 34 years on average [1]. Associated congenital heart disease such as bicuspid aortic valves, hypoplastic aortic arch, ventricular septal defects and others are frequently observed [2]. Clinical signs are weak or absent femoral pulses and blood pressure difference from upper to lower limbs. A heart murmur may be audible on the left sternal border, but it is more important to listen between the scapula. While in neonates echocardiography alone is sufficient to establish the diagnosis, as the treatment is typically surgical, later in life further imaging such as MRI or computed tomography scans will allow adequate therapeutical planning.

Alterations in peripheral circulation in dogs with cardiac tamponade

DISTURBANCES related to the pericardium have been increasingly observed in routine clinical examinations carried out on domestic animals. In dogs, pericardial effusion is commonly associated with neoplasia or idiopathic pericarditis...

1157 Outcome of Babies From the Postnatal Ward Who Underwent Echocardiography

Objective: To review our echocardiography service for postnatal ward babies, focusing on the indications, findings and the subsequent outcomes. Methods: Information on all echocardiograms performed on the neonatal unit was collected prospectively over 24 months (January 2008 to December 2009). We then retrospectively reviewed all echocardiograms carried on babies from the postnatal ward over the same period. Results: 285 echocardiograms were performed on 244 babies in the postnatal ward. The most common indication for performing echocardiography was murmur in 52% babies. The other indications for echocardiography included: 29% failed pulse oximetry, 8% family history of congenital heart disease, 3% antenatal cardiac concerns, 3% dysmorphology, 3% dysrhythmia and 2% weak femoral pulses. The median age for performing the scan was two days. In 40% babies, the echocardiogram was normal. The lesions identified include; 25% patent ductus arteriosus, 15% ventricular septal defects, 9% patent foramen ovale or atrial septal defect, 4.4% right ventricular outflow problems, 2.3 % left ventricular outflow problems, 1.5% tricuspid regurgitation, 1% atrioventricular septal defect, 0.4% transposition of great arteries, 0.4% tetralogy of fallot and 1% persistent pulmonary hypertension. 41% babies were discharged with no follow-up. 36% babies were followed up in the local cardiac murmur clinic and 23 % babies were referred to the paediatric cardiologist. Conclusion: Postnatal echocardiogram is valuable in early identification and appropriate referral of structural heart defects to the paediatric cardiologist. It has an equally important role in providing reassurance to families of babies with normal hearts prior to discharge from hospital.

Aortic Dissection during Diagnostic Aortography

A 30-year-old man presented to our clinic for evaluation of newly diagnosed hypertension. On examination, he had an apical systolic ejection click, a grade 2/6 systolic murmur, and weak femoral pulses. The difference in blood pressure between his arms and legs was 60 mm Hg. Echocardiography revealed coarctation of the aorta and a bicuspid aortic valve with mild stenosis. (See the Supplementary Appendix, available with the full text of this article at NEJM.org, for a chest radiograph showing the consequences of coarctation.) Catheterization through the right femoral artery was performed in preparation for stenting of the coarctation. Contrast material was injected . . .

Midaortic Syndrome in Neurofibromatosis Type 1 Resulting in Bilateral Renal Artery Stenosis

We describe the case of a 23-year-old white woman with a long history of hypertension. She was referred to us 7 years after her initial diagnosis of hypertension when her blood pressure control worsened during pregnancy. Clinical examination showed an abdominal bruit and weak femoral pulses. Imaging showed midaortic syndrome with bilateral renal artery stenosis as the cause of her hypertension, and further investigations showed neurofibromatosis type 1 as the underlying disorder. Midaortic syndrome, a rare disorder of the abdominal aorta that is different from classic coarctation, typically is associated with neurofibromatosis. Renal artery stenosis is common, as are weak femoral pulses and impaired development of the lower limbs. Because of the rarity of this syndrome, only anectodal evidence exists with regard to treatment. Surgery and interventional treatment with stent placement in the abdominal aorta have been reported, as well as good outcomes with long-term medical management. Our patient continues to be healthy without intervention, with reasonable blood pressure control and normal kidney function on a 4-drug antihypertensive regimen. We discuss midaortic syndrome with a focus on diagnosis, differential diagnosis, associated conditions, and management. Nephrologists, radiologists, and ultrasonographers should be aware of this rare cause of renovascular hypertension.

Electrical cardioversion of sustained ventricular tachycardia in three Boxers

2 spayed female (8 and 9 years old) and 1 sexually intact male (6.5 years old) Boxers were treated because of sustained ventricular tachycardia by electrical cardioversion. Physical examination of the 8-year-old female Boxer revealed tachycardia (heart rate, 250 beats/min), weak femoral pulses, pale mucous membranes, panting, and lethargy. The 6.5-year-old male Boxer had similar physical examination findings, with the addition of a syncopal event. Analysis of the ECG rhythm strips for the 8- and 6.5-year-old dogs indicated a right ventricular origin of the ventricular tachycardia. The 9-year-old female Boxer was being treated with an IV constant rate infusion of lidocaine hydrochloride because of ventricular arrhythmias during the initial examination; physical examination re-vealed weakness, pale mucous membranes, prolonged capillary refill time, weak femoral pulses, and tachycardia (heart rate, 265 beats/min). Analysis of the ECG rhythm strip for the 9-year-old Boxer indicated a left ventricular origin of the ventricular tachycardia. Pharmacological cardioversion treatment was unsuccessful in all 3 Boxers; however, electrical cardioversion by use of a biphasic defibrillator synchronized to conduct 30 J of energy during the peak of the QRS complex was successful in each dog. The electrical cardioversion procedure was performed 2 times (5-day interval between procedures) in the 9-year-old female as a result of relapse of the ventricular tachycardia condition. Results and follow-up monitoring suggested electrical cardioversion of sustained ventricular tachycardia may be a safe and effective treatment in Boxers that are unresponsive to medical treatment.

Coarctation of the Abdominal Aorta

A 14-year old male with no previous medical history presented with headache, left third cranial nerve palsy, and generalized tonic-clonic seizures. Magnetic resonance imaging of the brain revealed subarachnoid hemorrhage secondary to an aneurysm of the left posterior communicating artery. The patient underwent successful transcatheter coil occlusion of the aneurysm, and his neurological symptoms improved (Figure 1). Figure 1. A, Cerebral angiogram demonstrating a large ruptured aneurysm (arrow) of the posterior communicating artery. B, Angiogram after endovascular coil embolization showing exclusion of the aneurysm. During admission, he remained hypertensive, with an arterial blood pressure of 170/96 mm Hg in the upper limbs despite medical treatment with atenolol, amlodipine, and enalapril. On physical examination, an abdominal systolic bruit was heard, and weak femoral pulses were noted. Blood pressure in the right and left arms was similar, but there was a significant differential …

Advances in Neonatal Cardiac Surgery

After completing this article, readers should be able to: 1. Describe the developments that have allowed advances in neonatal cardiac surgery. 2. Describe the diagnostic approach to hypoplastic left heart syndrome (HLHS). 3. Delineate the preoperative management of the neonate who has HLHS. 4. Describe the original Norwood procedure and subsequent modifications. 5. Delineate the optimal therapy in the postoperative period for neonates who have a single ventricle. Advances in the surgical and medical management of children who have congenital heart disease (CHD) have led to a dramatic improvement in overall survival and reduced the long-term sequelae of open-heart surgery. With improved survival and decreased morbidity over the past 2 decades, the early complete repair of complex congenital heart problems in preterm and low-birthweight neonates has gained wider acceptance. Cardiologists and cardiothoracic surgeons now consider and perform complete neonatal repair more frequently than palliative surgery in this patient population. The treatment of hypoplastic left heart syndrome (HLHS) best illustrates the evolution of management strategies to improve survival and long-term outcome. A recent modification of the Norwood operation that involves a right ventricle-to-pulmonary artery conduit has led to much improved early postoperative stability and, in many centers, improved survival to a stage II superior cavopulmonary connection. A multidisciplinary team approach is required to provide the necessary care for this complex patient population. In 1938, Robert Gross at Children’s Hospital in Boston successfully ligated a patent ductus arteriosus, and this procedure—performed against the wishes of surgeon-in-chief William Ladd—opened the era of surgery for CHD. (1) Gross and Hufnagel performed detailed animal experiments to develop a technique for treatment of coarctation of the aorta that involves excision and end-to-end anastomosis. (2) Clarence Craaford of Stockholm, Sweden, had visited Gross to observe his experimental work, and in October 1944, Craaford and Nylin were the first to repair aortic …

Blunt trauma to the intrapericardial inferior vena cava: A case report and review of the literature

A 45-year-old woman was involved in a high-speedrear-endmotorvehiclecollisionandwastransportedby ambulance to our level I trauma centre. Shearrived to the emergency department with a heartrate of 116 beats per minute, and a systolic bloodpressure of 70 mm Hg without jugular venousdistention. Her initial Glasgow Coma Scale was 11.She was intubated in the emergency department.She had bilateral breath sounds, and weak but palp-able femoral pulses. Her hypotension respondedappropriately to initial resuscitation with 4 l of crys-talloid and two units of packed red blood cells(PRBC). Initial chest X-ray demonstrated a widenedmediastinum. Subsequent radiographs revealed aquestion of a left pneumothorax for which a tubethoracostomy was performed. Initial focused assess-ment by ultrasound for trauma (FAST) examinationdemonstratednofreeintraperitonealfluid;however,afollowupultrasoundshowedasmallamountoffluidin Morison’s pouch. There was no evidence of peri-cardialeffusion.Thepatient’shaemodynamicstatusnormalized and she was transported for computedtomography (CT). Her chest CTwas remarkable for aperiaortichaematomaalongthedescendingthoracicaorta with pericardial blood (Fig. 1B and C) andmultiple rib fractures. Abdominal CT also demon-stratedasmallamountoffreefluidwithoutevidenceof solid organ injury (Fig. 1B). During CTimaging thepatient developed recurrent hypotension despiteongoing aggressive resuscitation. This findingtogether with the CT observation prompted us totake the patient to the operating room intendingtoperformapericardialwindowasthesmallamountoffreefluidobservedbyCT-abdomendidnotexplainthedegreeofhypotension.Apericardialwindowwasperformed and demonstrated the presence of grossblood. Thus, a median sternotomy was immediatelyundertaken. This revealed a 3 cm laceration ofthe intrapericardial IVC at its junction with the rightatrium. This injury was primarily repaired using4-0 non-absorbable monofilament sutures withpledgets. Because of the intrapericardial injuryidentified and the small amount of fluid in detectedbyCT-abdomen.Anexploratorylaparotomywasper-formed to exclude an intrahepactic IVC injury. Thisdemonstrated a linear tear in the liver along thefalciform ligament. This injury was managed by acombination of electrocautery and applicationof topical haemostatic agents. No other intra-abdominal injuries were noted. Postoperative aorticangiography revealed no evidence of thoracic aorticinjury. Her post-operative course was notable for

Erstdiagnose eines kompletten Aortenbogenverschlusses bei einer 65-jährigen Hypertonikerin

A 65-year-old woman presented in a hypertensive crisis and with angina pectoris. She had a history of hypertension for several years and medication included five different antihypertensive drugs. On physical examination a faint systolic murmur was heard. Weak femoral pulses were felt, but not the arterial pulses distal to the groin. An attempt to perform coronary arteriography failed because it was impossible to pass the catheter across the aortic arch. MR-angiography and cardiac catheterization via the brachial artery confirmed the diagnosis of a complete interruption of the aortic arch distal to the left subclavian artery and showed distinct collateral circulation predominantly via the internal mammary arteries. Because of the age of the patient we assumed that the pathogenesis of this interruption of the aortic arch probably was progression and finally occlusion of an aortic coarctation. It was not possible to distinguish the findings from a true congenital atresia, because there was no histological examination. The patient rejected surgery and conservative therapy with frequent monitoring seemed justified, considering the good blood pressure adjustment and the extensive collateral vascularization. Patients with complete interruption of the aortic arch very rarely do reach late adult age without previous surgical intervention. Indeed, such a situation is only conceivable when there is a good collateral blood supply and no concomitant shunt defects. There is an indication for operation, but as there are insufficient follow-up data this should be weighed up carefully in elderly patients whose blood pressure is well controlled.

Determinants of weak femoral artery pulse in dogs with mitral valve prolapse

In three substudies encompassing 247 dogs from two breeds predisposed to myxomatous mitral valve disease (MMVD), femoral artery pulse strength was palpated and related to potential explanatory factors, including quantitative echocardiographic measures of MMVD, aortic and femoral artery diameter and wall thickness and blood pressure. In addition, in 109 Cavalier King Charles Spaniels (of which 61 were included in the three substudies mentioned above), the relation between femoral artery pulse strength and presence of thrombocytopenia was investigated. In 26% of the dogs, a pulse ⩽50% of normal strength was detected; six dogs (2.0%) had an absent pulse unilaterally or bilaterally. Mitral valve prolapse severity, degree of obesity, heart rate and age all influenced femoral artery pulse strength negatively. Weak femoral artery pulses were not related to clinical signs or to decreased pulse pressure or stroke volume. A weak femoral artery pulse reflected reduced artery diameter and/or distension rather than occlusion. In conclusion, weak femoral artery pulses in dogs with MMVD seem to be associated with regional/local reductions in blood flow and not arteriosclerosis or platelet count.

Chronic lower limb ischemia.

Peripheral vascular disease commonly affects the arteries supplying the leg and is mostly caused by atherosclerosis. Restriction of blood flow, due to arterial stenosis or occlusion, often leads patients to complain of muscle pain on walking (intermittent claudication). Any further reduction in blood flow causes ischaemic pain at rest, which affects the foot. Ulceration and gangrene may then supervene and can result in loss of the limb if not treated. The Fontaine score is useful when classifying the severity of ischaemia. #### Fontaine classification of chronic leg ischaemia Stage I Asymptomatic Stage II Intermittent claudication Stage III Ischaemic rest pain Stage IV Ulceration or gangrene, or both Angiogram showing bilateral occlusions of superficial femoral arteries in thighs. Collaterals arising from the profunda femoris artery can functionally bypass this occlusion Although many patients with claudication remain stable, about 150–200 per million of the population progress to critical limb ischaemia (Fontaine III or IV) each year. Many patients with critical limb ischaemia can undergo revascularisation, which has a reasonable chance of saving the limb. A recent audit by the Vascular Surgical Society found a success rate of over 70% for these patients. However, many patients still require major amputation. Rehabilitation of elderly patients after amputation can prove difficult, with high community costs. Critical limb ischaemia has been estimated to cost over £200m a year in the United Kingdom. ### History and examination A history of muscular, cramp-like pain on walking that is rapidly relieved by resting, together with absent pulses, strongly supports the diagnosis of intermittent claudication. Disease of the superficial femoral artery in the thigh results in absent popliteal and foot pulses and often causes calf claudication. Disease of the aorta or iliac artery results in a weak or absent femoral pulse, often associated with a femoral bruit. Disease at this level may cause calf, thigh, or buttock claudication. Method of palpating …

Cultivating Conscience: Learning to Make End-of-Life Decisions in the Emergency Department

Cultivating Conscience: Learning to Make End-of-Life Decisions in the Emergency Department

Femoral artery occlusion in Cavalier King Charles Spaniels

Objective To investigate development of femoral artery occlusion in Cavalier King Charles Spaniels. Design Prospective study. Animals 954 Cavalier King Charles Spaniels. Procedure 1,750 cardiovascular examinations consisting of visual inspection of mucous membranes, thoracic auscultation in areas associated with the heart valves, thoracic palpation, and palpation of the femoral arteries were made at 10 dog shows on 954 dogs. Findings of clinically normal, weak, or undetectable femoral pulses were recorded. Pathologic changes in occluded femoral arteries of 2 dogs were examined histologically. Results Of the 954 dogs, 22 (2.3%) had an undetectable right or left femoral pulse on 1 or more examinations. Forty (4.2%) additional dogs had weak unilateral or bilateral femoral pulses. Only 1 dog had exercise intolerance, and it had coexistent congestive heart failure. Histologic examination of serial sections of an occluded femoral artery from 1 dog revealed intimai thickening with breaks in the internal elastic lamina proximal to the occluded segment. The occluded segment of the femoral artery was contracted and filled with an organizing, recanalizing thrombus. Similar histopathologic changes were found in sections of a femoral artery from another dog. Clinical Implications Femoral artery occlusion is rare in other breeds and is not clinically important in dogs because of adequate collateral circulation; however, its rather common development in Cavalier King Charles Spaniels indicates a genetic predisposition and probable weakness in the femoral artery wall. (J Am Vet Med Assoc 1997;211:872–874)

The mid-aortic dysplastic syndrome. Surgical considerations with a 2 to 18 year follow-up and selective histopathological study

Mild-aortic dysplastic syndrome is usually presented with advanced hypertension in young individuals in association with either weak or absent femoral pulses, due to diffuse narrowing of the aorta in its mid thoraco-abdominal course. There is frequent involvement of visceral arterial branches such as renal and superior mesenteric arteries. Although the disease has been popularised by the appealing name of "abdominal coarctation", the term mid-aortic dysplastic syndrome is more appropriate. In spite of the fact that the syndrome was described almost three decades ago, its exact aetiology remains obscure and pathogenesis speculative. Surgical revascularisation remains the only therapeutic remedy, in dealing with this particular group of young hypertensive patients. The clinical presentation, angiographic assessment and long-term outcome, following reconstruction in 11 patients (mean age 24.4 years) with mid-aortic dysplastic syndrome were evaluated, in an effort to determine the effectiveness of surgery. Late follow-up, slightly exceeding 16 years (mean 5.6 years) has shown normal and relief from hypertension in practically all individuals. Furthermore, in order to elucidate at least some aspects of histopathology, studies were undertaken upon specimens from the aortic wall and the renal, carotid, lumbar and brachial arteries. The results suggested predominantly dysplastic features in the media, intima and particularly along the course of internal lamina. Aortography, using different projections, revealed variable patterns of high mid-aortic stenosis with or without associated renal artery disease. All patients were hypertensive (mean blood pressure 170 mmHg) and co-exist renal artery disease, unilateral in three and bilateral in three cases, was detected.(ABSTRACT TRUNCATED AT 250 WORDS)