Takeji Saitoh

The usefulness of delayed enhancement magnetic resonance imaging for diagnosis and evaluation of cardiac function in patients with cardiac sarcoidosis

OBJECTIVES Cardiac involvement is an important prognostic factor in patients with sarcoidosis. We evaluated the usefulness of delayed enhancement MRI (DE-MRI) for diagnosing cardiac sarcoidosis by comparing with nuclear imaging and studying the correlation between DE area and left ventricular (LV) function. METHODS Twelve patients (male:female 3:9) diagnosed as having sarcoidosis underwent Gd-MRI, myocardial perfusion SPECT (Tl-201, Tc-99m sestamibi), Ga-67 scintigraphy, and/or F-18 FDG-PET. RESULTS DE was observed in 5 patients, and was positive in 39 (39%) of 100 LV segments. The corresponding perfusion defects in myocardial perfusion SPECT were undetectable in 14 (36%) segments. DE distributed mainly in mid- to epi-myocardium, and the lack of perfusion defects in myocardial perfusion SPECT was more prominent in less transmural DE segments. Two patients with diffuse DE and 1 case with focal DE exhibited positive cardiac uptake in Ga-67 scintigraphy, and 2 other cases with focal DE showed cardiac uptake in F-18 FDG-PET. In 7 patients without DE, there were no significant findings in nuclear imaging. Both LV end-diastolic and end-systolic volume were positively and LV ejection fraction was negatively correlated with the extent of DE area. Four patients treated with corticosteroid showed improvement in nuclear imaging and slight decreases in DE area but no recovery in LV function. CONCLUSIONS DE-MRI is useful to diagnose the cardiac involvement of sarcoidosis and to evaluate cardiac function. It is likely that the distribution of DE in mid- to epi-myocardium is the characteristic of cardiac sarcoidosis, and the larger DE area may be correlated with poor LV function.

Distribution of late gadolinium enhancement in various types of cardiomyopathies: Significance in differential diagnosis, clinical features and prognosis

The recent development of cardiac magnetic resonance (CMR) techniques has allowed detailed analyses of cardiac function and tissue characterization with high spatial resolution. We review characteristic CMR features in ischemic and non-ischemic cardiomyopathies (ICM and NICM), especially in terms of the location and distribution of late gadolinium enhancement (LGE). CMR in ICM shows segmental wall motion abnormalities or wall thinning in a particular coronary arterial territory, and the subendocardial or transmural LGE. LGE in NICM generally does not correspond to any particular coronary artery distribution and is located mostly in the mid-wall to subepicardial layer. The analysis of LGE distribution is valuable to differentiate NICM with diffusely impaired systolic function, including dilated cardiomyopathy, end-stage hypertrophic cardiomyopathy (HCM), cardiac sarcoidosis, and myocarditis, and those with diffuse left ventricular (LV) hypertrophy including HCM, cardiac amyloidosis and Anderson-Fabry disease. A transient low signal intensity LGE in regions of severe LV dysfunction is a particular feature of stress cardiomyopathy. In arrhythmogenic right ventricular cardiomyopathy/dysplasia, an enhancement of right ventricular (RV) wall with functional and morphological changes of RV becomes apparent. Finally, the analyses of LGE distribution have potentials to predict cardiac outcomes and response to treatments.

Delayed Enhancement on Cardiac Magnetic Resonance and Clinical, Morphological, and Electrocardiographical Features in Hypertrophic Cardiomyopathy

BACKGROUND The clinical, morphological, and electrocardiographical relevance of delayed enhancement (DE) in cardiac magnetic resonance (CMR) was studied in patients with hypertrophic cardiomyopathy (HCM). METHODS AND RESULTS A total of 56 patients underwent both gadolinium-enhanced CMR and 12-lead electrocardiogram. The CMR demonstrated DE at the left ventricular (LV) wall in 39 patients. The patients with DE included more cases with dilated phase of HCM, higher New York Heart Association (NYHA) classes and incidence of ventricular tachyarrhythmias (VT), lower LV ejection fraction (LVEF) and mean LV wall thickness (WT), and a larger ratio of maximum to minimum LVWT. The QRS duration was prolonged and the QRS axis deviated toward left with increases in the DE volume (r = 0.58 and r = 0.41, P < .01). Abnormal Q waves were present in 5 patients and the location coincided with the DE segments in 4 patients, but the concordance was not significant. The amplitude of T waves correlated with the ratio of the apex to basal LVWT (r = 0.38, P < .01) and was more negative in cases with DE at the apex. CONCLUSIONS In HCM, the DE was associated with higher NYHA classes and prevalence of VT, impaired global LV function and asymmetrical hypertrophy, and conduction disturbance, abnormal Q waves, and giant negative T waves.

Comparison of Global and Regional Abnormalities in 99mTc-sestamibi and Cardiac Magnetic Resonance Imaging in Dilated Cardiomyopathy

OBJECTIVE The clinical relevance of perfusion defects and increased washout rate (WOR) in (99m)Tc-sestamibi (Tc MIBI) imaging has not been well characterized in dilated cardiomyopathy (DCM). We analyzed abnormalities in Tc MIBI imaging in relation to those in cardiac magnetic resonance (CMR) imaging. METHODS AND RESULTS Nineteen DCM patients underwent both Tc MIBI and CMR imaging. The perfusion defects and global and regional MIBI WORs were evaluated with planar and single photon emission computed tomography (SPECT) images. The left ventricular function and the delayed enhancement (DE) were estimated with the cine- and DE-mode CMR. In the DCM patients, the Tc MIBI SPECT showed perfusion defects in 65 segments of 14 patients. The global and regional Tc MIBI WORs were higher than those in 10 normal volunteers (19.4 +/- 9.1% vs. 11.7 +/- 6.8% in global and 13.8 +/- 8.6% vs. 9.6 +/- 8.2% in regional WORs; mean +/- SD, P < .01). The DE-mode CMR demonstrated DE in 103 segments of 14 patients. The severity of perfusion defects was correlated with the extent of DE (r = 0.71, P < .01). The regional Tc MIBI WOR was highest in the segments with perfusion defects or DE, but the Tc MIBI WOR in segments without them was also higher than that in the normal volunteers. There was a weak but significant negative correlation between the regional Tc MIBI WOR and wall thickening (r = -0.23, P < .01). CONCLUSIONS In DCM, the severity of perfusion defects and the increase in Tc MIBI WOR reflect the spread of myocardial fibrosis and/or scar. The increase in Tc MIBI WOR is potentially associated with regional dysfunction of LV wall.

Functional, morphological and electrocardiographical abnormalities in patients with apical hypertrophic cardiomyopathy and apical aneurysm: correlation with cardiac MR.

Objective The prognosis of apical hypertrophic cardiomyopathy (APH) has been benign, but apical myocardial injury has prognostic importance. We studied functional, morphological and electrocardiographical abnormalities in patients with APH and with apical aneurysm and sought to find parameters that relate to apical myocardial injury. Methods Study design: a multicentre trans-sectional study. Patients: 45 patients with APH and 5 with apical aneurysm diagnosed with transthoracic echocardiography (TTE) in the database of Hamamatsu Circulation Forum. Measure: the apical contraction with cine-cardiac MR (CMR), the myocardial fibrotic scar with late gadolinium enhancement (LGE)-CMR, and QRS fragmentation (fQRS) defined when two ECG-leads exhibited RSR’s patterns. Results Cine-CMR revealed 27 patients with normal, 12 with hypokinetic and 11 with dyskinetic apical contraction. TTE misdiagnosed 11 (48%) patients with hypokinetic and dyskinetic contraction as those with normal contraction. Apical LGE was apparent in 10 (83%) and 11 (100%) patients with hypokinetic and dyskinetic contraction, whereas only in 11 patients (41%) with normal contraction (p<0.01). Patients with dyskinetic apical contraction had the lowest left ventricular ejection fraction, the highest prevalence of ventricular tachycardia, and the smallest ST depression and depth of negative T waves. The presence of fQRS was associated with impaired apical contraction and apical LGE (OR=8.32 and 8.61, p<0.05). Conclusions CMR is superior to TTE for analysing abnormalities of the apex in patients with APH and with apical aneurysm. The presence of fQRS can be a promising parameter for the early detection of apical myocardial injury.

Ultrasound analysis of the relationship between right internal jugular vein and common carotid artery in the left head-rotation and head-flexion position

Common carotid artery (CCA) injury is a serious complication of internal jugular vein (IJV) cannulation. To minimize unintentional CCA puncture, the anatomic relationship between the IJV and the CCA and the size of IJV were compared under different head positions. Ultrasound analyses of the IJV and the CCA were performed in 103 consecutive patients. Overlapping angle (OA), real puncture angle (RPA) and diameter of IJV (DIJV) were evaluated with 30° and 60° left rotation and with 30° left flexion. When the head position was changed from 30° left rotation to 60° left rotation, OA increased significantly from 6.5° ± 7.7° to 14.5° ± 7.4° at the cricoid cartilage level (Cricoid-level) and from 14.4° ± 8.4° to 20.6° ± 6.9° at the middle triangle level (Triangle-level), whereas RPA decreased significantly at these levels (from 49.7° ± 11.9° to 43.5° ± 13.1° and from 51.1° ± 14.4° to 44.3° ± 13.9°, respectively; P < 0.01 for both). When the head position was changed from 30° left rotation to 30° left flexion, neither OA nor RPA significantly changed (OA: 6.3° ± 6.1° and 15.0° ± 7.2°, RPA: 48.5° ± 12.4° and 51.8° ± 13.6°, P not significant vs 30° left rotation). There was no difference in DIJV when comparing 30° left rotation and 30° left flexion, although DIJV was largest at 60° left rotation. RPA positively correlated with age, and DIJV positively correlated with body mass index. In conclusion, excessive left rotation should be avoided to minimize the probability of unintentional CCA puncture during IJV cannulation. When 30° left rotation is not feasible, the head-flexion position should be utilized.

Intra-cardiac distribution of late gadolinium enhancement in cardiac sarcoidosis and dilated cardiomyopathy

Cardiac involvement of sarcoid lesions is diagnosed by myocardial biopsy which is frequently false-negative, and patients with cardiac sarcoidosis (CS) who have impaired left ventricular (LV) systolic function are sometimes diagnosed with dilated cardiomyopathy (DCM). Late gadolinium enhancement (LE) in magnetic resonance imaging is now a critical finding in diagnosing CS, and the novel Japanese guideline considers myocardial LE to be a major criterion of CS. This article describes the value of LE in patients with CS who have impaired LV systolic function, particularly the diagnostic and clinical significance of LE distribution in comparison with DCM. LE existed at all LV segments and myocardial layers in patients with CS, whereas it was localized predominantly in the midwall of basal to mid septum in those with DCM. Transmural (nodular), circumferential, and subepicardial and subendocardial LE distribution were highly specific in patients with CS, whereas the prevalence of striated midwall LE were high both in patients with CS and with DCM. Since sarcoidosis patients with LE have higher incidences of heart failure symptoms, ventricular tachyarrhythmia and sudden cardiac death, the analyses of extent and distribution of LE are crucial in early diagnosis and therapeutic approach for patients with CS.

Intra-left ventricular flow dynamics in patients with preserved and impaired left ventricular function: Analysis with 3D cine phase contrast MRI (4D-Flow).

To examine how left ventricular (LV) volume and function affect flow dynamics by analyzing 3D intra‐LV vortex features using 4D‐Flow.

Accidental Entrapment of Electrical Mapping Catheter by Chiari’s Network in Right Atrium during Catheter Ablation Procedure

A 78-year-old male was admitted to our hospital due to frequent palpitation. His electrocardiogram (ECG) presented regular narrow QRS tachycardia with 170 bpm, and catheter ablation was planned. During electroanatomical mapping of the right atrium (RA) with a multiloop mapping catheter, the catheter head was entrapped nearby the ostium of inferior vena cava. Rotation and traction of the catheter failed to detach the catheter head from the RA wall. Exfoliation of connective tissue twined around catheter tip by forceps, which were designed for endomyocardial biopsy, succeeded to retract and remove the catheter. Postprocedural echocardiography and pathologic examination proved the existence of Chiaris network. The handling of complex catheters in the RA has a potential risk of entrapment with Chiaris network.

Crowned dens syndrome

Dear Editor, A 90-year-old man visited our emergency department owing to neck pain and occipital headache. The pain occurred suddenly a few days previous, and had become gradually worse. He had a history of cerebral infarction, dementia, and cholelithiasis, but was not on any medication. The patient’s vital signs were stable excluding a body temperature of 37.5°C. Physical examination showed the reduction of passive cervical spine movements with posterior neck pain and occipital headache. Rotation, extension, and flexion in the neck were all limited. However, there was no tenderness in the nape of the neck. In terms of neurological findings, overall cranial nerve, motor, and sensory nervous systems were intact. In laboratory data, the value of the C-responsive protein was 4.34 mg/dL (normal range, 0.0–0.1 mg/dL); all other findings were unremarkable. Computed tomography (CT) of the neck showed crown-shaped calcium deposits surrounding the odontoid process (Fig. 1A, arrows), and dotted calcifications of the transverse ligament of the atlas (Fig. 1B, arrows).There were no other lesions causative of pain in brain or spine. By integrating the results, we diagnosed crowned dens syndrome (CDS). The chief complaint of patients of CDS is neck pain, due to calcification deposition around the odontoid process. The cause of CDS is thought to be the microcrystalline deposition, most often calcium pyrophosphate dihydrate crystals and/or hydroxyapatite crystals, in the transverse ligament of the atlas around the odontoid process. However, the pathophysiologic process has not been completely proven. Clinicians could confuse the differential diagnosis and misdiagnose CDS as meningitis, polymyalgia rheumatica, or dissecting aneurysm of the vertebral and basilar artery. In particular, meningitis must be considered first in the differential diagnosis because the triad of fever, headache, and neck stiffness due to meningitis are very similar to the symptoms of CDS. Using CT scans, CDS is identified in up to 5% of the patients over the age of 70 years who present to hospital with the chief symptom of neck pain. Therefore, it is not a rare entity but becoming unclear using plain radiography for the diagnosis because of improper images. Computed tomography is the gold standard for detecting calcification in transverse, apical, and alar ligaments. However, when the CT scan is carried out a long time after the onset of symptoms, calcification around the odontoid process may not be detected as it may have been absorbed. Non-steroidal anti-inflammatory drugs (NSAIDs) have been commonly used as first-line therapy for CDS. In cases without improvement using NSAIDs alone, treatment with moderate dosage of corticosteroids is recommended. In our case, NSAID therapy alleviated the patient’s symptoms completely. Taiki Yamada, Takeji Saitoh, Hironao Hozumi, Yoshiaki Takahashi, Masashi Nozawa, Toshiaki Mochizuki, and Atsuto Yoshino Department of Emergency and Disaster Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan