Israel Medical Association Journal

Background: As the shortcomings of the Bentall operation and its variants in the Marfan syndrome have become apparent, the recent cusp-sparing techniques (remodeling or reimplantation) bear promise of better mid-term and long-term outcomes.

Objective: To examine the results of aortic root surgery in patients with Marfan syndrome.

Methods: During the period March 1994 to September 2007, 220 patients underwent aortic valve-sparing surgery; 20 were Marfan patients (group 1) who were compared with another 20 Marfan patients undergoing composite aortic root replacement (group 2). Fourteen patients had aortic dissection and 26 had thoracic aortic aneurysm. There were 31 males and 9 females with a mean age of 37.9 ± 13.8 years. In group 1, reimplantation was used in 13 patients, remodeling in 4, and aortic valve repair with sinotubular junction replacement in 3. In group 2, a mechanical valve conduit was used. Mean logistic Euroscore was 12.27 ± 14.6% for the whole group, five of whom were emergent cases

Results: Group 2 had more previous cardiac procedures compared to group 1 (9 vs. 2, P = 0.03) and shorter cross-clamp time (122 ± 27.1 vs. 153.9 ± 23.7 minutes, P = 0.0004). Overall mortality was 10%. Early mortality was 10% in group 2 and 5% in group 1(NS). Mean follow-up time was 25 months for group 2 and 53 months for group 1. Three patients were reoperated; all had undergone the remodeling. Five year freedom from reoperation and death was 86% and 90% in group 2 and 70% and 95% in group 1 (P = 0.6, P = 0.6), respectively.

Conclusions: Late survival of patients with Marfan syndrome was similar in both groups. Root reconstruction tends towards a higher incidence of late reoperations if the remodeling technique is used. We now prefer to use the reimplantation technique.
 

The antiphospholipid syndrome is characterized by recurrent fetal loss, venous and/or arterial thrombosis, and thrombocytopenia associated with elevated titers of lupus anticoagulant and anticardiolipin antibodies. Although thrombosis is the characteristic vascular involvement in APS[1], the development of vascular aneurysms in patients with APS has been reported. We describe four patients with established APS, who developed abdominal aortic aneurysm, and review the literature on previous published cases of arterial aneurysms developing in patients with APS. In addition, we discuss the possible pathophysiological association between APS and the development of this vascular abnormality.

Background: In recent years there has been an increase in endovascular repair of thoracic aortic aneurysms. In cases of insufficient neck length, occlusion of left subclavian artery achieves proper sealing and is usually well tolerated. Selected cases require revascularization of the left subclavian artery, including patients after coronary bypass surgery (left internal mammary to left anterior descending) and those with arm claudication or subclavian steal syndrome.

Objectives: To evaluate the tolerability of left subclavian artery occlusion by stent graft without revascularization.

Methods: Thirty patients with thoracic aortic aneurysms underwent endovascular repair between July 2000 and November 2004. Eleven of them had occlusion of the left subclavian artery that required revascularization in two. Follow-up (average 3 years) included a) blood pressure measurements of both arms at rest, after effort and pulse palpation, and b) vertebral blood flow by duplex scan.

Results: Of nine patients with no revascularization, 8 (89%) tolerated left subclavian artery occlusion with no claudication or steal syndrome; one (11%) suffered mild claudication only after effort and required no intervention. No left radial pulses were palpated in the nine patients. Blood pressure measurements in the left arm showed an average decrease of 40%, which remained constant after induced effort in all patients and was clinically insignificant. Duplex scan demonstrated reverse flow in the left vertebral artery in 8 of 9 patients (89%) and occlusion in 1 (originating in the arch and covered by the stent graft) with no clinical symptoms.

Conclusions: Left subclavian artery occlusion by stent graft is a tolerable procedure in the long term. In most cases, the constant decrease in blood pressure remained unchanged during follow-up and had no significant adverse affects. Most patients do not require revascularization prior to the endovascular procedure.
 

Background: Chlamydia pneumoniae has previously been associated with higher prevalence of valvular and cardiac calcifications.

Objectives: To investigate a possible association of seropositivity for C. pneumoniae and the presence of cardiac calcifications (mitral annular or aortic root calcification, and aortic valve sclerosis).

Methods: We retrospectively analyzed serological data (immunoglobulin G TWAR antibodies) from the AZACS trial (Azithromycin in Acute Coronary Syndromes), and correlated the serological findings according to titer levels with the presence of cardiac calcifications as detected by transthoracic echocardiography.

Results: In 271 patients, age 69 ± 13 years, who underwent both serological and echocardiographic evaluation, we found no significant association between the "calcification sum score" (on a scale of 0–3) in seropositive compared to seronegative patients (1.56 ± 1.15 vs.1.35 ± 1.15, respectively, P = 0.26). The median "calcification sum score" was 1 (interquartile range 0–3) for the seronegative group, and 2 (interquartile range 0–3) for the seropositive group (P = 0.2757). In addition, we did not find a significant correlation of any of the individual sites of cardiac calcification and Chlamydia pneumoniae seropositivity.

Conclusion: Our findings suggest that past C. pneumoniae infection may not be associated with the pathogenesis of valvular and cardiac calcifications.
 

Background: Coronary heart disease and ischemic stroke are among the leading causes of morbidity and mortality in adults, and cerebrovascular disease is associated with the presence of symptomatic and asymptomatic CHD[1]. Several studies noted an association between coronary calcification and thoracic aorta calcification by several imaging techniques, but this association has not yet been examined in stable angina pectoris patients with the use of spiral computed tomography.

Objectives: To examine by spiral CT the association between the presence and severity of CC[2] and thoracic aorta calcification in patients with stable angina pectoris.

Methods: The patients were enrolled in ACTION (A Coronary Disease Trial Investigating Outcome with Nifedipine GITS) in Israel. The 432 patients (371 men and 61 women aged 40–89 years) underwent chest CT and were evaluated for CC and aortic calcification.

Results: CC was documented in 90% of the patients (n=392) and aortic calcification in 70% (n=303). A significant association (P < 0.05) was found between severity of CC and severity of aortic calcification (as measured by area, volume and slices of calcification). We also found an association between the number of coronary vessels calcified and the presence of aortic calcification: 90% of patients with triple-vessel disease (n=157) were also positive for aortic calcification (P < 0.05). Age also had an effect: 87% of patients ≥ 65 years (n=219) were positive for both coronary and aortic calcification (P = 0.005) while only 57% ≤ 65 (n=209) were positive for both (P = 0.081).

Conclusions: Our study demonstrates a strong association between the presence and severity of CC and the presence and severity of calcification of thoracic aorta in patients with stable angina pectoris as detected by spiral CT.

Objectives: To determine whether there is an association between the presence and severity of adult calcific aortic stenosis and Chlamydia pneumoniae seropositivity

Methods: Forty adult patients (23 women, 17 men) were divided into three groups according to echocardiographic aortic valve area: Group A – 7 symptomatic subjects (age 67 ± 7 years) with normal aortic valve and normal coronary angiogram, Group B – 16 patients (age 73 ± 6) with moderate ACAS[1] (AVA[2]> 0.8 £ 1.5 cm²), and Group C – 17 patients (age 76 ± 7) with severe ACAS (AVA £ 0.8 cm²). We tested for immunoglobulins M, G and A as retrospective evidence of C. pneumoniae infection using the micro-immunofluorescence method. Past C. pneumoniae infection was defined by IgG titer > 16 £ 512.

Results: No patients in Group A showed positive Ig[3] for C. pneumoniae. IgM was not detected in any of the patients with ACAS (groups B and C) while 2 of 17 patients (12%) in group C showed IgA for the pathogen. High titers of IgG were found in 14 of 33 (42%) of the patients with moderate or severe ACAS: 5 of 16 (31%) in group B and 9 of 17 (53%) in group C (P = 0.2). Both groups had the same prevalence of coronary artery disease (66%). AVA was lower in IgG-seropositive patients than in the seronegative group (0.88 ± 0.3 cm² vs. 1.22 ± 0.4 cm², respectively, P = 0.02).

Conclusions: Past C. pneumoniae infection may be associated with a higher prevalence and greater severity of ACAS.

A 61 year old woman with chronic untreated hypertension presented for routine examination without any complaints.

Background: The indication for aortic valve replacement in patients with significant aortic stenosis is symptomatology. Aortic stenosis may be associated with bleeding from colonic angiodysplasia, resulting in anemia. Persistent anemia in such patients, despite lack of an identifiable source of bleeding, is not considered an indication for valve replacement.

Objectives: To report our experience with two elderly female patients who suffered from severe asymptomatic aortic stenosis, low levels of large von Willebrand factor multimer (10% and 5% respectively) and persistent anemia requiring multiple blood transfusions.

Methods: Both patients underwent an intensive work-up, but a source of bleeding could not be identified. Aortic valve replacement was performed in both patients.

Results: Aortic valve replacement abolished the need for further blood transfusions during a follow-up period of 20 months with normalization of the vWF[1] multimer level (20% and 30% respectively).

Conclusion: We suggest that aortic valve replacement be considered in selected patients with severe, otherwise asymptomatic aortic stenosis, who suffer from persistent anemia requiring multiple blood transfusions, lack an identifiable source of bleeding and have low levels of large vWF multimers.

Background: Arterial involvement in Behçet's syndrome is rare. Aneurysms are common among the arterial lesions, affecting various arteries but mostly the abdominal aorta. Surgical interposition graft insertion is the treatment of choice for large aneurysms. However, vasculitis in these patients is the reason for the notorious surgical complications that result in up to 50% false aneurysms in anastomotic sites. Recently, endovascular repair for abdominal aortic aneurysms has been established.

Objectives: To learn more about vascular Behçet and, specifically, to compare the results of surgical treatment and endovascular repair of AAA[1] in patients with Behçet's syndrome.

Methods: We retrieved the medical records of all 53 patients with Behçet disease admitted to Rambam Medical Center during the years 1985 and 2001 and analysed the results and follow-up of open surgery versus endovascular repair of AAA in patients with known Behçet's syndrome.

Results: Of the 53 patients with Behçet's disease 18 had vascular manifestations (34%). AAAs were encountered in 8 patients (15%) and 5 were treated. Open surgery (group 1), under general anesthesia, lasted less than 3 hours with an average aortic clamping time of 34 minutes (range 26–41 min) after which the patients were transferred to the intensive care unit for 24–48 hours. Endovascular treatment (group 2), although lasting about the same time without the need for intensive care, necessitated contrast media and fluoroscopy. The length of hospital stay was considerably shorter for patients after endovascular repair compared to open surgery (3 days vs. 6 days). Combined mortality and morbidity was higher in patients who underwent open surgery compared to endovascular repair (one death, one major amputation and three anastomotic pseudoaneurysms compared to one temporary contrast-induced nephropathy).

Conclusions: Vasculo-Behçet patients with AAA are better candidates for endovascular treatment than atherosclerotic patients. Combined morbidity (especially anastomotic pseudoaneurysms) and mortality of Behçet patients after endovascular repair is considerably lower than after open surgery.