Christophe Lions

Prognosis Assessment of Cardiac Involvement in Systemic AL Amyloidosis by Magnetic Resonance Imaging

BACKGROUND Cardiac involvement is one of the most important prognostic factors in systemic AL amyloidosis. The aim of our study was to assess the role of cardiovascular magnetic resonance (CMR) imaging in prognosis evaluation in AL amyloidosis. METHODS We retrospectively analyzed 29 consecutive patients with AL amyloidosis who had undergone CMR. Clinical, laboratory, echocardiographic, and CMR characteristics were compared between CMR-positive (ie, with CMR signs of cardiac localization of AL amyloidosis) and CMR-negative patients. Univariate and multivariate analyses were performed to assess the prognostic value of positive CMR in comparison with other prognostic factors. RESULTS CMR was positive in 11 patients (38%). The overall survival rates for CMR-positive patients were 28%, 14%, and 14% versus 84%, 77%, and 45% at 1, 2, and 5 years, respectively, for CMR-negative patients (P=.002). Late gadolinium enhancement patterns, biventricular hypertrophy, and pericardial effusion on CMR were more frequent in nonsurvivors. Congestive heart failure, abnormal echocardiography, Eastern Cooperative Oncology Group grade >1, brain natriuretic peptide, and left ventricular ejection fraction <55% also were associated with a decreased survival. The presence of congestive heart failure was the only significant variable associated with survival on multivariate analysis. CONCLUSION We found that the presence of a positive CMR in AL amyloidosis was associated with a significantly increased risk of death, in particular of cardiac origin, but was not independent of clinical congestive heart failure.

TEVAR in Patients with Late Complications of Aortic Coarctation Repair

Purpose: To review the use of thoracic endovascular aortic repair (TEVAR) for late pseudoaneurysm formation after surgical repair of aortic coarctation. Methods: From May 2001 to May 2005, 8 patients (5 men; mean age 47.6 years, range 18–73) with a history of aortic coarctation repairs 17 to 40 years prior were referred to our institution for an anastomotic thoracic pseudoaneurysm. TEVAR was performed successfully in 7 patients; 1 died of suspected aneurysm rupture before the scheduled procedure. A carotid-subclavian bypass was performed in 3 patients. Results: All the procedures were immediately successful. No type I endoleaks were seen on the final control angiogram, but 2 of the patients with carotid-subclavian bypasses required additional left subclavian artery embolization due to type II endoleak. One of these patients died before embolotherapy on the 5th postoperative day from presumed aneurysm rupture (14% 30-day mortality rate). Over a follow-up period ranging from 15 to 72 months (mean 37), all the false aneurysms have remained thrombosed and the mean diameter has decreased from 44 to 23 mm. No endograft-related complications have occurred, and no further interventions have so far been necessary. Conclusion: TEVAR is a feasible alternative treatment for patients who have already undergone surgical repair of aortic coarctation. Technical issues regarding the endovascular strategy should be discussed with a multidisciplinary team to define the correct interventional plan.

''Right-Sided'' May-Thurner Syndrome

The May-Thurner syndrome is a well-known anatomical anomaly where the left common iliac vein (LCIV) is compressed between the right common iliac artery and the fifth vertebral body. This report describes the case of a “right-sided” May-Thurner syndrome where the right common iliac vein (RCIV) is compressed by the left common iliac artery in a patient with a left-sided inferior vena cava (IVC). A 26-year-old woman was admitted to our institution with acute edema of the right lower limb. The diagnosis of May-Thurner syndrome was done by CT scan and confirmed by phlebography. An endovascular treatment with stenting was carried out, with good patency and clinical result at 12-month follow-up.

Interest of β-blockers in patients with right ventricular systemic dysfunction.

BACKGROUND β-blockers improve the prognosis of patients with cardiac failure due to left ventricular systolic dysfunction. The aim of this study was to assess the efficacy of β-blockers in patients with dysfunctional systemic right ventricle. METHODS Fourteen patients with systemic right ventricle following a Mustard or a Senning operation for the transposition of the great arteries, or congenitally corrected transposition were included in the study. All had a decreased systemic right ventricular ejection fraction despite having standard cardiac failure therapy. Quality of life, New York Heart Association class, aerobic capacity, and systemic right ventricular function were assessed before treatment with β-blockers and at the end of the follow-up period, mean of 12.8 months with a range from 3 to 36 months. RESULTS Change in New York Heart Association class was significant (p = 0.016). Quality of life improved significantly throughout the study from a median grade 2 with a range from 1 to 3 to a median grade 1 with a range from 1 to 2 (p = 0.008). Systemic right ventricular ejection fraction assessed by radionuclide ventriculography improved significantly from a median of 41% (range: 29-53%) to 49% (range: 29-62%; p = 0.031). However, the change in thee ejection fraction assessed by magnetic resonance imaging was not significant from a median of 29% (range: 12-47%) to 32% (range: 22-63%; p = 0.063). CONCLUSION In patients with cardiac failure due to systemic right ventricular dysfunction, β-blockers improve New York Heart Association class, quality of life, and systemic right ventricular ejection fraction assessed by radionuclide ventriculography.

Métastase myocardique de tumeur bronchique

Monsieur X., âge de 65 ans, fumeur, est adresse pour le complement d’exploration radiologique d’une masse pulmonaire decouverte sur un cliche du thorax systematique. Le scanner thoracique injecte confirme la masse parenchymateuse pulmonaire et met en evidence une masse developpee au niveau du ventricule gauche (fig. 1). Cet examen est complete par une IRM cardiaque synchronisee.

Thrombus compliquant l’évolution d’un infarctus du myocarde

Femme de 36 ans, presentant une aphasie brutale a J1 d’un accouchement par voie basse pour lequel le diagnostic d’accident vasculaire cerebral (AVC) ischemique sylvien gauche est retenu.