A comparison of venous angiography and radioisotope heart scanning in the diagnosis of pericardial effusion.

Cardiac amyloidosis (CA) is a challenging diagnosis, particularly when the classic signs, such as increased wall thickness in a non-dilated left ventricle (LV), are absent. This makes the diagnosis more difficult in patients with normal LV wall thickness. We present a case of CA without increased wall thickness and without the characteristic granular sparkling echotexture in a non-dilated LV. A 50-year-old female patient presented with worsening breathlessness on exertion, paroxysmal nocturnal dyspnea, oliguria, and lower-extremity edema. Electrocardiography showed low voltage in the limb leads and a pseudoinfarction pattern in the anterior leads. The echocardiographic evaluation revealed a non-dilated LV with normal wall thickness, no granular sparkling echotexture of the myocardium, a mildly dilated left atrium, restrictive filling (grade 3 diastolic dysfunction), and pericardial effusion. A follow-up quantitative echocardiographic study 2 weeks later showed a slight increase in LV wall thickness (still within the normal range), decreased global longitudinal strain, and a relative "apical sparing" pattern of longitudinal strain in the apex of the LV. After 1 month, LV wall thickness increased beyond the normal range, and the granular sparkling echotexture became evident. Cardiac amyloidosis was subsequently confirmed by delayed gadolinium enhancement on cardiac magnetic resonance imaging, abnormal serum-free light chain levels, positive serum immunofixation, and an extracardiac biopsy positive for amyloid. Patients presenting with normal wall thickness in a non-dilated LV might only be in an early stage of CA. Thus, the diagnosis can be easily overlooked. For smaller individuals, relative wall thickness (RWT) may be a more sensitive indicator for further investigation. In patients presenting with increased RWT, restrictive filling, and pericardial effusion in the absence of other plausible causes, CA should be considered, even in the absence of the classic echocardiographic signs of amyloid deposition. Furthermore, two-dimensional speckle-tracking echocardiography can enhance clinical suspicion of CA and should be recommended as part of the diagnostic workup.

30-Year-Old Man With Outside-of-Hospital Cardiac Arrest

Implications of Normal Left Ventricular Wall Thickness in Critical Aortic Stenosis

Anderson-Fabry disease (AFD) is an X-linked disease, with cardiac involvement resulting in increased left ventricular (LV) wall thickness. Speckle tracking echocardiography analysis may be more sensitive in the assessment of myocardial impairment in AFD patients and have prognostic value. Our aim was to evaluate LV and left atrial (LA) dysfunction by traditional and strain parameters in AFD patients and evaluate prognostic utility. Fifty-six AFD patients were age- and sex-matched to 56 healthy controls. LV global longitudinal strain (GLS) and LA reservoir strain (LASR) were significantly lower in male (GLS: 19.38[3.21] vs. 17.8[7.0], P = 0.009; LASR: 38.07 ± 6.67 vs. 31.12 ± 6.76, P = 0.003) and female (GLS: 20.58 ± 1.63 vs. 19.29 ± 1.67, P = 0.003; LASR: 38.77 ± 7.43 vs. 33.13 ± 6.06, P < 0.001) AFD patients compared with controls. Reduced strain parameters were also seen in female AFD patients with normal wall thickness (GLS: 20.88 ± 1.74 vs. 19.72 ± 1.53, P = 0.037; LASR: 40.09 ± 7.15 vs. 34.79 ± 6.20, P = 0.004). 53/56 AFD patients had a median follow-up of 43[81] months; 11/53 experienced an adverse cardiovascular event (i.e. cardiac death, myocardial infarction, arrhythmias, stroke. and heart failure). LV wall thickness, LAVImax, and LV GLS displayed good sensitivity and specificity for adverse cardiac events. A prognostic risk decision tree comprising of these parameters demonstrated good predictive value for adverse events (AUC = 0.910). We demonstrate differences in LV and LA echocardiographic parameters in AFD patients compared with healthy controls, including female AFD patients with normal LV wall thickness. A prognostic risk decision tree stratified AFD patients into three groups with the highest risk group demonstrating more AFD-related adverse events.

Left ventricular hypertrophy is a heterogeneous disorder with distinct morphologies. Changes in wall thickness, left ventricular chamber diameter, and mass alter systolic wall stress of the left ventricle and may influence ischemic threshold. Thus, the goal of this study was to investigate the effect of the different patterns of left ventricular hypertrophy on the accuracy of dobutamine-atropine stress echocardiography. Three-hundred eighty-six patients underwent multistage dobutamine-atropine stress echocardiography and diagnostic angiography. Echocardiograms were measured for mean and relative wall thicknesses, chamber size, left ventricular mass, and end-systolic wall stress. The patterns of ventricular hypertrophy were concentric hypertrophy (increased wall thickness and mass), eccentric hypertrophy (normal wall thickness and increased mass), and concentric remodeling (increased wall thickness and normal mass). The overall sensitivity, specificity, and accuracy of dobutamine-atropine stress echocardiography for the detection of coronary artery disease were 85%, 87%, and 86%, respectively. Increased left ventricular mass index alone did not affect accuracy. Sensitivity was markedly reduced (36%) only in those with concentric remodeling. The univariate predictors of false-negative studies were single-vessel left circumflex disease, increased wall thickness, small chamber size, hyperdynamic ejection fraction, and left ventricular concentric remodeling. Multivariate predictors were concentric remodeling (P<0.0001; odds ratio, 13.5), left ventricular ejection fraction >2 SD above normal (P<0.0001), and single-vessel left circumflex disease (P<0.0007; odds ratio, 7.6). Sensitivity was excellent in patients with small ventricles and normal wall thickness and in those with normal or large chambers regardless of wall thickness. Dobutamine-atropine stress echocardiography is an accurate test in most patients with left ventricular hypertrophy, but it is insensitive in the small subset with concentric remodeling.

Development of left ventricular hypertrophy in adults with hypertrophic cardiomyopathy caused by cardiac myosin-binding protein C gene mutations

A previously healthy 50-year-old Chinese woman presented with a low-grade fever, a generalized headache, and chest pain of 2 to 3 weeks’ duration. She described intermittent sharp chest pain over the precordium that intensified when she lay down and shortness of breath after minimal exertion. Despite having lost her appetite, she had gained ≈10 pounds during the past month and had noticed ankle swelling. She denied having had arthralgia or skin rash and had not experienced any nocturnal dyspnea, wheezing, cough, expectoration, or hemoptysis. She had visited her relatives in the Middle East 6 months earlier, but her past medical history was uneventful. She had taken acetaminophen (Tylenol) tablets and a Chinese herbal preparation, but her symptoms continued. On physical examination, she appeared weak and ill. Her temperature was 36.7°C (98.0°F), and her pulse was 110 beats per minute, regular, and had normal volume and character. Her blood pressure was 115/70 mm Hg, which decreased to 90/70 mm Hg on inspiration; her respiratory rate was 22 breaths per minute. Carotid pulsations were normal. Jugular veins were distended to the angle of the mandible when the patient sat upright, but no further venous engorgement was noted on inspiration. There was mild mucosal pallor, but the oropharynx was otherwise normal. The first and second heart sounds were normal, and there were no clicks or gallops. A superficial scratchy systolic sound was heard intermittently over the left lower sternal region. Dullness to percussion, scattered inspiratory crackles, and diminished air entry were evident over both lung bases. Abdominal examination demonstrated a soft, tender liver palpable 2 cm below the right costal margin. Her pelvis and rectum showed no abnormality. A stool guaiac test was negative. Neurological examination was normal. There was moderate pitting edema below the level of the knees. Results of the initial …

A 69-year-old woman presented with symptoms and examination findings consistent with congestive heart failure. She had history of hypertension, mild renal insufficiency, and bilateral hip replacements. Transthoracic echocardiogram showed left ventricular ejection fraction of 25% to 30%, normal left ventricular size and left ventricular wall thickness, and moderate pericardial effusion (Figures 1 and 2; Movie I in the Data Supplement). Pericardiocentesis removed 650 mL of straw-colored fluid, which was negative for infection or malignant cells. Coronary angiogram showed normal coronary arteries. Figure 1. Echocardiographic image showing restriction by mitral inflow. Figure 2. Echocardiographic images showing tissue Doppler imaging. She was discharged on low-dose beta blocker, angiotensin-converting enzyme inhibitor, and diuretics. Over the next 4 months, her biventricular function declined significantly, leading to multiple hospitalizations for congestive heart failure or symptomatic hypotension. Cardiac MRI revealed normal left ventricular size and wall thickness with severely reduced left ventricular ejection fraction of 14%. Right ventricle was normal in size with …

The Evaluation Criteria in Diagnosing Ischemia with Stress and Rest Myocardial Perfusion Gated SPECT

Background:The study aimed to compare normal oesophageal wall thickness based on 3-dimensional computed tomography (3DCT), 4-dimensional computed tomography (4DCT) and cone beam computed tomography (CBCT).Methods:Contrast-enhanced 3DCT, 4DCT, and CBCT scans were acquired from 50 patients with lung cancer or metastatic lung cancer. The outer oesophageal wall was manually contoured on each 3DCT, the maximum intensity projection of 4DCT (4DCTMIP) the end expiration phase of 4DCT (4DCT50) (the end expiration phase of 4DCT) and the CBCT data sets. The average wall thicknesses were measured (defined as R3DCT, R50, RMIP, and RCBCT).Results:Whether for thoracic or for intra-abdominal segments, there were no significant differences between R3DCT and R50, but significant differences between R3DCT and RMIP, R3DCT and RCBCT. For upper and middle oesophagus, RCBCT were larger than RMIP. There was no significant difference between upper and middle segments on 3DCT, 4DCT, and CBCT. Intra-abdominal oesophageal wall thickness was greater than that of thoracic oesophagus. There were no differences between upper and lower, and middle and lower oesophagus on CBCT.Conclusion:Our findings indicate normal oesophageal wall thickness differed along the length of oesophagus whatever it was delineated on 3DCT, 4DCT (4DCT50 and 4DCTMIP) or CBCT. It is reasonable to use uniform criterion to identify normal esophageal wall thickness when delineating gross tumor volume on 3DCT and 4DCT50, the same is true of delineating internal gross tumor volume on 4DCTMIP or CBCT images for lower and intra-abdominal oesophagus. But, in spite of using contrast-enhanced scanning, relatively blurred boundary on the CBCT images is noteworthy, especially for upper and middle thoracic esophagus.

Symptomatic Exercise-Induced Left Ventricular Outflow Tract Obstruction without Left Ventricular Hypertrophy

The objective of this study was to investigate whether the use of carbon dioxide (CO2) angiography decreases the intraoperative use of iodine contrast medium (ICM), thereby decreasing the risk of developing postoperative renal damage in patients undergoing iliac branch device (IBD) implantation. Patients undergoing IBD implantation at a single tertiary center between May 2013 and August 2019 were screened for inclusion in the study. A cohort of patients in whom an intraoperative imaging protocol using predominantly CO2 was compared with a control group in whom ICM was used (CO2 and ICM groups). Fusion imaging was used in both groups. Retrospective review of the medical charts and all imaging was performed. Variables were expressed as median with interquartile range (IQR) or absolute number and percentage. Wilcoxon-Mann-Whitney and χ2 tests were used to compare continuous and categorical variables, respectively. P values of <0 .05 were considered statistically significant. Twenty-three patients were included in the CO2 group and 21 in the ICM group without significant differences in patient characteristics between the groups. Intraoperative iodine exposure was lower in the CO2 group than in the ICM group (8.2 g [IQR, 7.1-10.9 g) vs 15.8 g (IQR 7.6-21 g); P = 0.015, respectively). There were no differences in technical success, clinical success, fluoroscopy time, and dose-area product in the 2 groups. There were no adverse events that could be related to the intraoperative use of CO2. There was no difference in postoperative survival nor renal function assessment between the groups despite the higher number of accessory renal arteries embolized in the CO2 group (P = 0.221). Reduction of intraoperative ICM exposure during IBD implantation is feasible through the predominant use of CO2 automated angiography. This can be done safely without affecting the technical success or radiation exposure. The immediate postoperative renal damage was low when a modern intraoperative imaging protocol with ICM was used and was not significantly reduced by the use of CO2. Further and larger studies are needed to investigate the long-term effects. This study aimed to investigate how to minimize intraoperative risk during iliac branched repair exploring the intraoperative use of automated carbon dioxide angiography in order to decrease the use of contrast media protecting renal function and thereby potentially impacting long-term survival.

Carbon dioxide angiography during peripheral vascular interventions is associated with decreased cardiac and renal complications in patients with chronic kidney disease

A 74-year-old man presented with decreasing exercise tolerance and mild ankle edema. He was previously fit but was now breathless on climbing 2 flights of stairs. He had no history of angina, orthopnea, or paroxysmal nocturnal dyspnea. His medical history included non–insulin-dependent diabetes mellitus treated for 10 years and mild hypertension. Six years earlier he had been diagnosed with a monoclonal gammopathy of unknown significance. At that time, a bone marrow biopsy showed 30% overall cellularity with 5% to 10% plasmacytosis (normal <4%) and immunoglobulin light-chain restriction. Approximately 3 years ago, he developed deep vein thrombosis and was treated with low-molecular-weight heparin. A year later, leg swelling occurred and was attributed to venous insufficiency. The following year, he developed progressive fatigue on exertion, and an abnormal ECG (Figure 1) led to a treadmill test that was considered normal. An echocardiogram showed concentric wall thickening (Movie 1 in the Data Supplement), and the possibility of cardiac amyloidosis was raised. A fat pad biopsy was negative for amyloid deposits. The bone marrow biopsy performed in 2005 (when his monoclonal gammopathy of unknown significance was diagnosed) was restained and was negative for amyloid. At that time, serum-free λ light chains were 108.9 mg/L (normal range, 5.7–26.3) with κ light chains of 13 mg/L (normal, 3.3–19) and an abnormal ratio of 0.12 (normal, 0.26–1.65). His brain natriuretic peptide measured 275 pcg/mL. He was treated with oral diuretics, which improved leg swelling, but because of persistent symptoms, he sought medical care at our institution. On review of symptoms, he denied jaw claudication, symptoms of postural hypotension, easy bruising, or tongue swelling. He did give a history suggestive of neuropathy with a leathery feeling in his feet but no numbness in his hands. Medications included metformin 500 mg twice a day, aspirin 80 mg daily, lisinopril …

A 48-year-old man, with only a history of mild systemic hypertension, was initially evaluated after presenting with symptoms of exertional dyspnea occurring predominantly with inclines. At that time, an abnormal 12-lead ECG was obtained demonstrating left ventricular hypertrophy by conventional voltage criteria, prompting additional testing with a 2-dimensional echocardiogram that showed normal systolic function (ejection fraction=65%), with 14-mm ventricular septal thickness and 12 mm in the posterolateral wall, and mild systolic anterior motion (SAM) of the mitral valve (bend of anterior leaflet into outflow tract without septal contact). A stress nuclear stress test showed no myocardial ischemia at rest or at peak exercise with a normal blood pressure response and no arrhythmias or ST-T changes during exercise or in recovery. The patient was prescribed a β-blocker for treatment of systemic hypertension. During the next 2 years, the patient developed more limiting exertional symptoms with routine activities. β-Blocker dosage was increased, and a repeat echocardiogram demonstrated similar findings to the initial study, borderline left ventricular (LV) wall thickness despite well-controlled blood pressure. The abnormal ECG, and mild SAM at rest, raised consideration for a diagnosis of hypertrophic cardiomyopathy (HCM) and management for limiting heart failure symptoms. HCM is often suspected in a patient based on the presence of cardiovascular symptoms, detection of abnormal ECG, systolic ejection murmur on routine examination, or as part of pedigree screening.1,2 Abnormalities on ECG are present in >90% of patients with HCM, although no specific ECG pattern is pathognomonic.1 Clinical diagnosis of HCM can reliably be made in the majority of patients with 2-dimensional transthoracic echocardiography by imaging increased LV wall thickness (≥15 mm) with a nondilated cavity in the absence of any disease known to cause LV hypertrophy of that magnitude (ie, systemic hypertension or aortic stenosis).1–5 In …