Dilation of peripheral vessels in Marfan syndrome: Importance of thoracoabdominal MR angiography

Objectives

Aim of this study was to assess vascular morphology and dimension of the entire aorta and branch vessels in MFS using thoracoabdominal MR angiography (MRA).

Background

Evaluation of vascular involvement in Marfan Syndrome (MFS) is focused to the ascending aorta, which has the major risk of dissection/rupture.

Methods

From March 2006 to January 2011, 64 patients (35 ± 13 years old) with MFS underwent echocardiography and MRA of thoracic and abdominal aorta. Measurements of ascending aorta, aortic arch, descending thoracic and abdominal aorta, and aortic branch vessels were assessed. In patients with aortic dissection only non dissected vessels were considered for measurements.

Results

64 Marfan patients were divided into 2 groups: patients in natural history (group A, 55%) and patients previously submitted to aortic root and/or ascending aorta replacement because of severe aortic dilation or dissection (group B, 45%).Dilation of the descending aorta, mostly of the aortic isthmus, occurred in 18/53 patients (8/35 group A, 10/18 group B). Abdominal aorta resulted in the normal range in group A and in 16/18 patients of group B, while iliac vessels were dilated in 7/35 patients of group A and in 10/23 patients of group B. Four patients of group B presented isolated dilation of subclavian, celiac, mesenteric, or renal arteries.

Conclusions

Vascular dilation beyond the aortic root is not uncommon in MFS. The systematic use of MRA provides a comprehensive evaluation of the entire arterial system.     

Endovascular treatment of penetrating aortic ulcers: mid-term follow-up

Introduction and objectives

The aim of this work is to describe the short- and mid-term results of endovascular treatment of penetrating ulcers in the thoracic aorta.

Methods

Between 1998 and 2010, 22 patients with penetrating ulcers in the thoracic aorta received endografts (mean age 69.8 years, 91% male); 50% were indicated for acute aortic syndrome (8 chest pain, 1 aortic rupture, 1 aortobronchial fistula, 1 distal embolization) and 50% for aortic or ulcer diameter. All preoperative, operative and follow-up data were recorded prospectively and met EUROSTAR criteria.

Results

Technical success was 100% with no intraoperative deaths or open conversions; 6 (27.3%) required preoperative supraaortic trunk debranching and 1.3 endografts were used per patient; 27.3% developed complications in-hospital and 9.1% required reintervention prior to discharge. Mortality at 30 days was 4.5%. After a mean 52.3 month follow-up (range 0.1-122), cumulative survival free from complications and reinterventions at 100 months was 61.7% and 79.5% respectively, with 95.5% cumulative survival free from aorta- or procedure-related death. We identified no factors significantly related to poor intra- or postoperative clinical course.

Conclusions

Endovascular treatment of penetrating aortic ulcers is both possible and effective despite high patient comorbidity. Although a substantial rate of complications and reinterventions can be expected—especially in-hospital—(38.3% and 20.5% respectively at 100 months), long-term mortality is low (4.5%).Full English text available from:www.revespcardiol.org     

Anévrismes infectieux de l’aorte thoracique : présentation de 7 cas et revue de la littérature

Purpose

Infectious aortic aneurysms are rare conditions, being responsible of 2% of aortic aneurysms. Most published results are surgical case series concerning infected abdominal aorta. In this retrospective study, we assessed clinical features and outcome of patients presenting infectious thoracic aortic aneurysms.

Patients and methods

Diagnosis was based upon a combination of imaging evidence for thoracic aorta aneurysm and evidence for an infective aetiology including a culture of a causative pathogen, or a favourable outcome with anti-infective therapy. Retrospective case series.

Results

Six men and one woman were included, with a mean age of 66 years. All the patient presented at least one cardiovascular risk factor or atherosclerosis localisation. Fever (71%) and chest pain (42%) were the most common clinical presenting manifestations. The causative pathogens were: Staphylococcus aureus (N = 1), Salmonella enteritidis (N = 3) and Candida albicans (N = 1). The contrast-enhanced computed-tomography disclosed an aneurysm whose diameter reached more than 50 mm (N = 5), that increased rapidly in size (N = 5), or presented an inflammatory aspect of the aortic wall (N = 4). Management was both medical and interventional: surgery (N = 3) or endoluminal repair (N = 4). Outcome was favourable in six patients; one patient died from aneurysm-related complications.

Conclusion

Clinical manifestations revealing an infectious thoracic aneurysm are variable. Diagnosis should be considered in patients presenting a rapidly-growing aneurysm, especially in the presence of elevated acute phase reactants. Endoluminal repair constitutes a treatment option. The role of FDG-PET for diagnosis and follow-up remains to be defined.     

Anévrismes infectieux de l’aorte. À propos d’une série de dix patients

Purpose

Infectious aortic aneurysms are rare, being responsible of less than 3% of aortic aneurysms. In this study, we report the clinical characteristics and the outcome of patients presenting with infectious aortic aneurysms in an internal medicine unit.

Methods

Diagnosis of infection-related aortic aneurysm was obtained using: computed tomographic scan; and blood cultures, cultures and molecular biology testing of aortic wall and intra-aneurismal thrombus.

Results

The 10 consecutive patients included in this study consisted in eight men and two women with a mean age of 61.7 years. Patients presented with fever (n = 9), asthenia (n = 2), abdominal (n = 4) or chest pain (n = 1), lumbar pain (n = 3). Computed tomographic scan showed aneurysm involving both thoracic and abdominal aorta (n = 1), abdominal (n = 8) or thoracic aorta (n = 1). Isolated microorganisms were: positive Gram cocci (70%) and negative Gram bacilli (30%). All patients underwent both medical and surgical therapy. Outcome was favorable in nine patients; the remaining patient died from aneurismal aortic rupture.

Conclusion

Clinical manifestations revealing infectious aortic aneurysms are variable, including aneurysm rupture as well as atypical abdominal pain with inflammatory syndrome. These latter presentations are more common in patients, who are hospitalized in internal medicine. Our study underlines that this clinical pattern should not be ignored, in order to avoid both diagnostic and therapeutic delay that could lead to life-threatening complications and poor prognosis.     

Short term outcome of thoracic endovascular aortic repair in patients with thoracic aortic diseases

Aim and background

Open surgical repair for thoracic aortic diseases is associated with a high perioperative mortality and morbidity. Most of type B aortic dissections are uncomplicated and are medically treated which carries a high mortality rate. Thoracic endovascular aortic repair is the first-line therapy for isolated aneurysms of the descending aorta and complicated type B aortic dissection. The aim of this study is to test the safety of early thoracic endovascular aortic repair in patients with uncomplicated type B aortic dissection and patients with thoracic aortic aneurysms.

Cholangite sclérosante de réanimation : évolution favorable sous acide ursodésoxycholique et fénofibrate

Introduction

Ischemic cholangitis in intensive care unit is a recently reported liver disease in patients who have had a prolonged mechanical ventilation and vasopressive drug support for multiple organ deficiency. Prognosis is usually poor and the only life-saving therapy is liver transplantation despite ursodesoxycholic acid treatment.

Case report

We report a 63-year-old man who presented with a sclerosis cholangitis after a month in intensive care unit, effectively treated with fenofibrate and ursodesoxycholic acid. Recent reports underline fenofibrate efficacy in the treatment of primary biliary cirrhosis, especially in association with ursodesoxycholic acid. This treatment has prevented liver transplantation for our patient with a correct quality of life.

Conclusion

The addition of fibrate to ursodesoxycholic acid improves persistent cholestasis in sclerosing cholangitis.     

Implantación electiva de stent recubierto en la coartación y recoartación en el joven y en el adulto

Introduction and objectives

Stent implantation is an effective therapy for aortic coarctation and recoarctation. However, in adolescents and adults, aortic wall rupture and dissection can occur, as well as aneurysms during follow-up. In order to reduce these complications, we electively implant covered stents.

Methods

Since 2005, we have performed the procedure using femoral access in 17 patients (2 adolescents and 15 adults), 16 electively and 1 as a rescue procedure. We used the Mullins technique in all cases, implanting a NuMED^® covered stent.

Results

Good stent apposition was achieved in all 17 procedures; 8 patients required a distal flare. Gradient was reduced from 40 (16) mmHg to 2 (2) mmHg (P<.001) and lumen diameter increased from 4 (2) mm to 19 (3) mm (P<.001). Two exceptional cases are discussed: one patient with aortic wall rupture who underwent a rescue procedure using a stent within a covered stent and another patient with total obstruction and intercostal aneurysm in whom the outcome was fatal at 48 h postprocedure (autopsy is shown). Four-year clinical follow-up included Doppler echocardiography; an additional imaging technique was required in 13 patients. All patients recovered well and there were no complications.

Conclusions

Covered stents are effective in treating coarctation and recoarctation in adolescents and adults, are the treatment of choice in patients with complex anatomy, and must be available in the operating room as a rescue device when implanting a conventional stent.Full English text available from:www.revespcardiol.org/en     

Carence en vitamine B12 par toxicité du protoxyde d’azote : une cause méconnue de sclérose combinée de la moelle

Introduction

Nitrous oxide is widely used in anesthesia. It is usually safe but may be associated with severe side effects when it is used repeatedly or on a prolonged time. Here, we report a case of drug-induced subacute combined spinal cord degeneration related to cobalamin deficiency.

Case report

A 20-year-old man with sickle-cell disease (SS) who was followed for severe recurrent vaso-occlusive crisis with repeated hospital admissions presented with ascending motor and sensitive neurological deficits that were later associated with bladder dysfunction. He was first considered to develop Guillain-Barre syndrome. However, persisting neurological signs despite adequate treatment lately led to diagnose subacute combined medullar degeneration due to abnormal cobalamin (vitamin B12) metabolism induced by repeated use of nitrous oxide during painful episodes of sickle-cell disease.

Conclusion

Inhaled nitric oxide is widely used in the treatment of vaso-occlusive crisis. Clinicians should be aware of possible severe neurologic side effects.     

Sarcoïdose vertébrale. Évolution spontanément favorable : une observation et revue de la littérature

Introduction

The prevalence of vertebral sarcoidosis is highly variable (1 to 36% of reported case series). Because of limited clinical expression, its frequency is probably underestimated. Its proper management is not clearly defined.

Case report

A 42-year-old woman who had a past medical history of cutaneous and pulmonary sarcoidosis presented with low back pain that was refractory to usual medical treatment. A diagnosis of vertebral localisation of sarcoidosis was considered on the history of proven sarcoidosis, radiological features, and the absence of evidence of an alternative diagnosis. In the absence of other clinical or biological evidence of active sarcoidosis, a simple follow-up was planned. MRI control at 1 year showed the resolution of vertebral sarcoidosis lesions.

Conclusion

Spontaneous regression is a possible outcome of vertebral sarcoidosis. Initiation of a specific treatment should be discussed in the absence of other visceral involvement.     

Polyradiculonévrite aiguë révélant un lupus érythémateux systémique : une présentation inhabituelle d’évolution fatale

Introduction

Neurological manifestations of systemic lupus erythematosus are common and numerous. They mainly involve the central nervous system, peripheral involvement being rare. Acute polyradiculoneuropathy is very uncommon.

Case report

We report a 44-year-old man, who presented with acute polyradiculoneuropathy revealing systemic lupus erythematosus. Outcome was fatal despite treatment with corticosteroids and immunoglobulin.

Conclusion

Acute polyradiculoneuropathy is a very rare manifestation of systemic lupus erythematosus and can compromise functional and life prognosis. Early diagnosis and management are crucial.     

Validation de la reproductibilité de la mesure de la surface de l’orifice régurgitant par planimétrie dans l’insuffisance aortique : une étude préliminaire d’échocardiographie trans-œsophagienne tridimensionnelle

Background

Aortic regurgitation is mainly evaluated by trans-thoracic echocardiography using multi-parametric qualitative and semi quantitative tools. All those parameters can fail to meet expectations, resulting in an imperfect diagnostic reliability and assessment of aortic regurgitation severity can be challenging.

Objectives

We sought to evaluate feasibility and intra- and inter-observer reproducibility of aortic regurgitant orifice area measured by planimetry with tridimensional trans-esophageal echocardiography on patients with at least grade 2/4 aortic regurgitation.

Patients and methods

Consecutive patients with at least grade 2/4 aortic regurgitation measured by trans-thoracic echocardiography and referred for trans-esophageal echocardiography for any reason were included. Planimetric reconstructions of regurgitant orifice area were studied and reproducibility indexes between senior and junior observers were calculated.

Results

Twenty-three patients were included in this study. Intra- and inter-observer reproducibility were excellent with an ICC of 0.95 [0.88–0.98], P < 0.0001 and 0.91 [0.79–0.96], P < 0.0001, respectively. Mean length of the measurement was 6.6 ± 0.9 min [CI95% 6.23–7.01].

Conclusion

Planimetric measurement of the aortic regurgitant orifice using tridimensional trans-esophageal echocardiography seems to be feasible and has great intra- and inter-observer reproducibility. Reconstruction durations were compatible with a daily use. There is a need now to investigate the reliability of this measurement as compared with the reference technique.     

Remodelado cardiaco inverso estructural y funcional en pacientes con aleteo auricular típico sometidos a ablación del istmo cavotricuspídeo

Introduction and objectives

The purpose of the present study is to determine the structural and functional cardiac changes that occur in patients at 1-year follow-up after ablation of typical atrial flutter.

Methods

We enrolled 95 consecutive patients referred for cavotricuspid isthmus ablation. Echocardiography was performed at ≤6 h post-procedure and 1-year follow-up.

Results

Of 95 patients initially included, 89 completed 1-year follow-up. Hypertensive cardiopathy was the most frequently associated condition (39%); 24% of patients presented low baseline left ventricular systolic dysfunction. We observed a significant reduction in right and left atrial areas, end-diastolic and end-systolic left ventricular diameters, and interventricular septum. We observed substantial improvement in right atrium contraction fraction and left ventricular ejection fraction, and a reduction in pulmonary hypertension. Changes in diastolic dysfunction pattern were observed: 60% of patients progressed from baseline grade III to grade I; at 1-year follow-up, this improvement was found in 81%. We found no structural differences between paroxysmal and persistent atrial flutter at baseline and 1-year follow-up, exception for basal diastolic function.

Conclusions

In patients with typical atrial flutter undergoing cavotricuspid isthmus catheter ablation, we found inverse structural and functional cardiac remodeling at 1-year follow-up with much improved left ventricular ejection fraction, right atrium contraction fraction, and diastolic dysfunction pattern.Full English text available from:www.revespcardiol.org     

Acute aortic dissection: Epidemiology and outcomes

Background

Little epidemiological information on acute aortic dissection (AAD) is available in the literature. The objective of the present study was to determine the incidence and mortality rates of AAD in the general population and to analyze its clinical features.

Methods

Data from the Emilia-Romagna regional database of hospital admissions was analyzed. Urgent admissions with the diagnosis of dissection of the aorta, dissection of the thoracic aorta and dissection of the thoracoabdominal aorta were selected.

Results

Between January 2000 and December 2008, 1499 Emilia-Romagna residents were hospitalized with a diagnosis of AAD. The patients were divided into three groups: Group A, 617 patients (41.2%) surgically treated for type A AAD; Group B, 93 complicated patients (6.2%) with type B AAD treated by endovascular stent-grafting and Group C, 789 patients (52.6%) suffering from any type of AAD medically treated. The overall annual incidence rate was 4.7%/100,000 people and was higher for men than for women (6.7% vs 2.9%).Two hundred ninety-six patients (19.8%) were 80 years of age or older.The overall in-hospital mortality rate was 27.7%, with mortality rates of 21.1%, 26.9% and 33% in Groups A, B and C, respectively.

Conclusion

The incidence of AAD is not negligible and a notable rate of patients is ultra-octogenarian. A large number of patients with AAD had no surgery or interventional treatment. The results of surgical treatment for patients with type A dissection are acceptable but the results obtained in patients with complicated type B dissection who were treated with an endoprosthesis are dismal.     

Nouveautés dans la prise en charge de l’infarctus cérébral en 2014 : ce que le cardiologue doit savoir

Introduction

Interventional cardiology procedures are regularly exposed to ischemic neurological complications. IV fibrinolysis is the only approved treatment in ischemic stroke but is very often contraindicated in these situations. Many techniques of interventional neuroradiology (mechanical thrombectomy) have been developed over the past years and are used to treat these patients.

Observation

We report the case of two patients who were admitted in emergency for ischemic stroke with contraindication to IV fibrinolysis (cardioversion for atrial fibrillation under anticoagulation; 24 hours after carotid surgery). These patients were treated by endovascular thrombectomy procedure.

Discussion

After validation of IV fibrinolysis within 4.5 hours after stroke onset, techniques of mechanical thrombectomy have gradually been developed, either as a complementary treatment or as an alternative in the case of CI to fibrinolysis. These endovascular thrombectomy devices currently allow recanalization of proximal cerebral occlusions, which correlates with a favorable clinical prognosis. A review of the literature is provided, along with a discussion about the techniques currently being improved, their advantages and disadvantages and the selection of patients that can benefit from endovascular procedures.

Conclusion

In the case of a sudden occurrence of a neurological deficit during a cardiovascular procedure, a “thrombolysis alert” should be triggered. This will permit the rapid establishment of a clinico-radiological report for selecting stroke patients eligible for a procedure of recanalization by thrombolysis and/or mechanical thrombectomy.     

Anorexie psychogène et carences vitaminiques sévères dans les suites d’une gastrectomie longitudinale

Introduction

Morbid obesity is an emerging condition in the general population. Bariatric surgery, which has demonstrated its effectiveness for weight loss, mortality and morbidity related to obesity, is required in some patients. However, it may be associated with various adverse effects, including vitamin deficiencies.

Case report

We report a 33-year old man who presented central and peripheral neurological deficits and cardiac manifestations related to multiple vitamin deficiencies, following “sleeve” gastrectomy. The vitamin deficiencies were related to insufficient ingesta secondary to psychogenic anorexia. The patient improved with vitamins, antidepressant drugs and atypical neuroleptics.

Conclusion

Post-operative complications of “sleeve” gastrectomy include vitamin deficiencies that can develop in the context of psychogenic anorexia and ingesta reduction, in the absence of any digestive malabsorption.     

Intoxication par ingestion de plombs de pêche

Introduction

Lead colic is a rare cause of abdominal pain. The diagnosis of lead poisoning is most often mentioned in at risk populations (children, psychotic patients).

Case report

We report a 21-year-old man who presented with colicky abdominal pain. Abdominal plain radiograph showed multiple intracolonic metallic bodies. Markedly elevated lead and zinc protoporphyrin serum levels confirmed the diagnosis of lead poisoning. The patient reported that he commonly chewed fishing lead sinker and may sometimes swallow them during the preparation of fishing rod. Clinical outcome was favourable with chelation therapy.

Conclusion

Lead poisoning following fishing sinker ingestion is very uncommon. Diagnosis may be discussed in the presence of foreign metallic bodies on plain abdominal radiograph and confirmed by high serum level of lead. A prompt treatment with chelation therapy and digestive emptying is usually effective.     

Mort subite au cours d’une dégénérescence cérébelleuse subaiguë paranéoplasique avec dysautonomie associée aux anticorps anti-Hu révélant un cancer bronchique à petites cellules

Introduction

Paraneoplastic syndromes are a rare cancer complication with a frequent subacute evolution.

Observation

A 62-year-old man was admitted presenting with a cerebellar syndrome and orthostatic hypotension with dysautonomia. Anti-Hu antibody research was positive. A subcarinal adenopathy biopsy found out a small cell lung carcinoma. Despite a treatment with immunoglobulin and chemotherapy, the patient died suddenly, after a raise of dysautonomia symptoms.

Conclusion

Sudden death observations represent exceptional complications of paraneoplastic syndrome. They might be secondary to arrhythmias, ictal asystol or laryngospasm. Systematic research of paroxystic heart arrhythmias with holter-ECG in paraneoplastic syndrome may prevent sudden deaths.