Cemil Izgi

Association between mid-wall late gadolinium enhancement and sudden cardiac death in patients with dilated cardiomyopathy and mild and moderate left ventricular systolic dysfunction

Background: Current guidelines only recommend the use of an implantable cardioverter defibrillator in patients with dilated cardiomyopathy for the primary prevention of sudden cardiac death (SCD) in those with a left ventricular ejection fraction (LVEF) <35%. However, registries of out-of-hospital cardiac arrests demonstrate that 70% to 80% of such patients have an LVEF >35%. Patients with an LVEF >35% also have low competing risks of death from nonsudden causes. Therefore, those at high risk of SCD may gain longevity from successful implantable cardioverter defibrillator therapy. We investigated whether late gadolinium enhancement (LGE) cardiovascular magnetic resonance identified patients with dilated cardiomyopathy without severe LV systolic dysfunction at high risk of SCD. Methods: We prospectively investigated the association between midwall LGE and the prespecified primary composite outcome of SCD or aborted SCD among consecutive referrals with dilated cardiomyopathy and an LVEF ≥40% to our center between January 2000 and December 2011 who did not have a preexisting indication for implantable cardioverter defibrillator implantation. Results: Of 399 patients (145 women, median age 50 years, median LVEF 50%, 25.3% with LGE) followed for a median of 4.6 years, 18 of 101 (17.8%) patients with LGE reached the prespecified end point, compared with 7 of 298 (2.3%) without (hazard ratio [HR], 9.2; 95% confidence interval [CI], 3.9–21.8; P<0.0001). Nine patients (8.9%) with LGE compared with 6 (2.0%) without (HR, 4.9; 95% CI, 1.8–13.5; P=0.002) died suddenly, whereas 10 patients (9.9%) with LGE compared with 1 patient (0.3%) without (HR, 34.8; 95% CI, 4.6–266.6; P<0.001) had aborted SCD. After adjustment, LGE predicted the composite end point (HR, 9.3; 95% CI, 3.9–22.3; P<0.0001), SCD (HR, 4.8; 95% CI, 1.7–13.8; P=0.003), and aborted SCD (HR, 35.9; 95% CI, 4.8–271.4; P<0.001). Estimated HRs for the primary end point for patients with an LGE extent of 0% to 2.5%, 2.5% to 5%, and >5% compared with those without LGE were 10.6 (95% CI, 3.9–29.4), 4.9 (95% CI, 1.3–18.9), and 11.8 (95% CI, 4.3–32.3), respectively. Conclusions: Midwall LGE identifies a group of patients with dilated cardiomyopathy and an LVEF ≥40% at increased risk of SCD and low risk of nonsudden death who may benefit from implantable cardioverter defibrillator implantation. Clinical Trial Registration: URL: http://clinicaltrials.gov. Unique identifier: NCT00930735.

Isolated cleft of the posterior mitral valve leaflet in a patient with Marfan syndrome

Isolated cleft of the mitral valve is an uncommon congenital cause of mitral regurgitation. Most of the clefts involve the anterior leaflet and isolated clefts of the posterior leaflet have been anecdotally identified. We report a case of Marfan syndrome in whom an echocardiographic examination revealed a cleft in the posterior mitral leaflet. Marfan syndrome frequently involves the mitral valve causing mitral valve prolapse. Occurrence of a cleft may be an extension of its mitral valve involvement.

Severe myocardial ischemia after concentrated epinephrine use for the treatment of anaphylaxis: Kounis syndrome or epinephrine effect?

A 37-year-old woman presented to the emergency department with generalized erythema, flushing, pruritus, abdominal pain, respiratory distress, and disturbed consciousness. Her symptoms had started acutely approximately 30 minutes after ingestion of a tablet of amoxicillin (500 mg). Within seconds of her presentation to the emergency department, her clinical status deteriorated rapidly and she collapsed. Her systolic blood pressure was 40 mm Hg and pulse rate was 82 beats/min. She was immediately placed in the Trendelenburg position, nasal oxygen was started, an intravenous (IV) line was promptly established, and rapid saline infusion was started. Two doses of .5 mg epinephrine (diluted; 1:10,000) bolus administered 5 minutes apart by the IV route failed to restore an adequate blood pressure. A third dose of 1 mg epinephrine, which was accidentally infused undiluted (1:1000), restored the blood pressure to 105/65 mm Hg. However, immediately after the last epinephrine infusion and restoration of blood pressure, the patient had chest tightness, and ST depression was observed on the monitor.

Effect of personalized external aortic root support on aortic root motion and distension in Marfan syndrome patients

OBJECTIVE Personalized external aortic root support (PEARS) is a novel surgical approach with the aim of stabilizing the aortic root size and decreasing risk of dissection in Marfan syndrome patients. A bespoke polymer mesh tailored to each patients individual aorta shape is produced by modeling and then surgically implanted. The aim of this study is to assess the mechanical effects of PEARS on the aortic root systolic downward motion (an important determinant of aortic wall stress), aortic root distension and on the left ventricle (LV). METHODS/RESULTS A cohort of 27 Marfan patients had a prophylactic PEARS surgery between 2004 and 2012 with 24 having preoperative and follow-up cardiovascular magnetic resonance imaging studies. Systolic downward aortic root motion, aortic root distension, LV volumes/mass and mitral annular systolic excursion before the operation and in the latest follow-up were measured randomly and blinded. After a median follow-up of 50.5 (IQR 25.5-72) months following implantation of PEARS, systolic downward motion of aortic root was significantly decreased (12.6±3.6mm pre-operation vs 7.9±2.9mm latest follow-up, p<0.00001). There was a tendency for a decrease in systolic aortic root distension but this was not significant (median 4.5% vs 2%, p=0.35). There was no significant change in LV volumes, ejection fraction, mass and mitral annular systolic excursion in follow-up. CONCLUSIONS PEARS surgery decreases systolic downward aortic root motion which is an important determinant of longitudinal aortic wall stress. Aortic wall distension and Windkessel function are not significantly impaired in the follow-up after implantation of the mesh which is also supported by the lack of deterioration of LV volumes or mass.

Caseous calcification and liquefaction of the mitral annulus: a diagnostic confounder.

Caseous calcification of the mitral annulus is a rare form of periannular calcification that has a distinct appearance. It generally appears as a large spheric mass like calcification with a central echolucent area that may lead to diagnostic errors. Cardiac imagers should be familiar with this rare form of periannular calcification. We report the case of a 62-year-old woman in whom a suspicious spheric mass like calcification was detected with multislice computed tomography which was performed for coronary artery calcium scoring. Echocardiography displayed the typical findings of caseous calcification of the mitral annulus with central liquefaction.

Doppler Tissue Evaluation of Atrial Conduction Properties in Patients With Non-alcoholic Fatty-liver Disease

Non-alcoholic fatty liver disease (NAFLD) is the most common cause of chronic liver disease in clinical practice, and there is an increasing trend in its prevalence in the general population. Recent studies have demonstrated increased risk of atrial fibrillation (AF) in NAFLD. However, information on the mechanism of increased risk of AF in NAFLD is lacking. Impaired atrial conduction is an important factor in the pathophysiology of AF. We aimed to investigate atrial conduction properties in patients with NAFLD by tissue Doppler echocardiography. Fifty-nine ultrasound diagnosed NAFLD patients without clinical diagnosis of hypertension, diabetes mellitus, or cardiac disease and 22 normal subjects as controls were included in this study. Atrial conduction properties were assessed by electromechanical delay (EMD) derived from Doppler tissue echocardiography examination and P-wave dispersion (PWD) calculated from the 12-lead electrocardiogram. Inter-atrial and intra-atrial EMD intervals were significantly longer in NAFLD patients than in controls (inter-atrial EMD, 31.9 ± 8.5 ms vs. 23.4 ± 4.6 ms, p = 0.0001, and intra-atrial EMD, 14.3 ± 5.2 vs. 10.2 ± 4.0 ms, p = 0.001). Similarly, PWD was significantly higher in NAFLD patients compared with controls (49.2 ± 6.3 ms vs. 43.3 ± 4.2 ms, p = 0.0001). Maximum left atrial volume was also significantly higher in the NAFLD group than in controls (51 ± 11 mL vs. 34 ± 9 mL, p < 0.0001). This study demonstrated that atrial conduction is impaired in patients with NAFLD. Also, in a patient population of NAFLD without any clinical diagnosis of cardiac disease, diabetes, or hypertension, left atrial volume was increased compared with controls. These findings suggest impaired atrial conduction as a factor in increased risk of AF in NAFLD.

Annuloaortic Ectasia and Ascending Aortic Aneurysm as a Cardiovascular Manifestation of Acromegaly

A 65-year-old man was referred for evaluation of a cardiac murmur. The patient reported decreased effort capacity and dyspnea. Cardiac examination revealed a grade 3–4 diastolic murmur at the aortic area and a blood pressure of 145/45 mmHg. A comprehensive metabolic panel and lipid profile were within normal limits. The patient did not have a history of smoking. He was tall of stature and had enlarged hands and facial bones, which suggested acromegaly. These features had been noted for at least 10– 15 years by the patient’s family members. An echocardiographic examination revealed an enlarged ascending aorta (4.8 cm) with moderate to severe aortic regurgitation and a hypertrophied and enlarged left ventricle (end diastolic diameter 72 mm) with preserved systolic function. The aortic valve was apparently normal and the cause of aortic regurgitation was the ascending aortic aneurysm with dilated aortic annulus, namely, annuloaortic ectasia. A computed tomographic scan confirmed the ascending aortic aneurysm and anuloaortic ectasia, with a maximum aortic diameter of 6 cm in the root and 5.6 cm at the sinotubular junction (Figs. 1, 2). Fasting growth hormone (GH) and insulin-like growth factor-1 (IGF-1) levels were increased to [40 ng/ml (normal range 0–1 ng/ml) and 930 ng/ml (normal range for age 75–212 ng/ml), respectively. GH did not decrease with glucose loading. Fasting blood glucose was within normal limits, but glucose loading revealed impaired glucose tolerance. A magnetic resonance image of the hypophysis showed a macroadenoma (18 9 17 mm) of the anterior hypophysis (Fig. 3) consistent with a GH-secreting tumor. Coronary angiogram revealed normal coronary arteries. The patient was started on octreotide treatment and a composite graft of the ascending aorta and aortic valve replacement were performed.

Sex- and age-based differences in the natural history and outcome of dilated cardiomyopathy.

To evaluate the relationship between sex, age and outcome in dilated cardiomyopathy (DCM).

Aortic flow patterns before and after personalised external aortic root support implantation in Marfan patients

Implantation of a personalised external aortic root support (PEARS) in the Marfan aorta is a new procedure that has emerged recently, but its haemodynamic implication has not been investigated. The objective of this study was to compare the flow characteristics and hemodynamic indices in the aorta before and after insertion of PEARS, using combined cardiovascular magnetic resonance imaging (CMR) and computational fluid dynamics (CFD). Pre- and post-PEARS MR images were acquired from 3 patients and used to build patient-specific models and upstream flow conditions, which were incorporated into the CFD simulations. The results revealed that while the qualitative patterns of the haemodynamics were similar before and after PEARS implantation, the post-PEARS aortas had slightly less disturbed flow at the sinuses, as a result of reduced diameters in the post-PEARS aortic roots. Quantitative differences were observed between the pre- and post-PEARS aortas, in that the mean values of helicity flow index (HFI) varied by -10%, 35% and 20% in post-PEARS aortas of Patients 1, 2 and 3, respectively, but all values were within the range reported for normal aortas. Comparisons with MR measured velocities in the descending aorta of Patient 2 demonstrated that the computational models were able to reproduce the important flow features observed in vivo.

Letter Regarding Article by Augé et al, “Role for Matrix Metalloproteinase-2 in Oxidized Low- Density Lipoprotein-Induced Activation of the Sphingomyelin/Ceramide Pathway and Smooth Muscle Cell Proliferation”

To the Editor: We have read the recent article by Auge et al1 with great interest. The authors demonstrated the role of matrix metalloproteinase (MMP)-2 in oxidized (ox)-LDL–induced sphingolipid signaling and smooth muscle cell (SMC) proliferation. We have several comments about their findings and statements. The authors depicted the activated neutral sphingomyelinase (SMase) as the membrane-bound form of the enzyme. However, as far as we know, the precise cellular localization of the signaling sphingomyelinase in the ox-LDL–treated SMC is still not exactly clear. Wherever the enzyme is located, the critical point, as stated also by the authors, is how the extracellular ox-LDL is linked to the signaling SMase. The authors suggest this link to be MMP-2, subsequent to its activation by membrane type 1 (MT1)-MMP. However, although the results of their experiments underline the novel, crucial role of both MT1-MMP and MMP-2 in ox-LDL–induced SMase activation, …