Jugular Venous Response for Risk Stratification in Heart Failure.

BACKGROUND:  The response of jugular venous pressure (JVP) to increased preload with inspiration has been recognized as a method of stratifying risk in the management of heart failure (HF). Whether the JVP response to inspiration may be more effective than other simple approaches in this setting remains unclear. METHODS:  This study enrolled 79 patients with stable HF. JVP was assessed from the right internal jugular vein in the sitting position and was considered high if visible above the right clavicle at rest. JVP responses to inspiration, the five-repetition sit-to-stand test (5-STS), and squatting were also evaluated. The primary outcome was a composite of all-cause death and hospitalization for worsening HF. RESULTS:  JVP assessment after 5-STS and during squatting was not conducted in two and 14 HF patients, respectively, due to physical limitations. During a mean follow-up of 837 days, the primary outcome was associated with a high JVP at rest (hazard ratio, 2.47; 95% confidence interval [CI], 1.09 to 5.60; P <0.05), with inspiration (hazard ratio, 2.53; 95% CI, 1.17 to 5.46; P <0.05), after 5-STS (hazard ratio, 2.61; 95% CI, 1.23 to 5.97; P <0.05), and during squatting (hazard ratio, 2.40; 95% CI, 1.03 to 6.06; P <0.05). Among patients without a high JVP at rest, the specificity of the primary outcome at one year was greater for the JVP response to inspiration (89%) and squatting (92%) than for the response to 5-STS (80%). CONCLUSIONS:  JVP response to increased preload with inspiration may be a simple and practical method for risk assessment in patients with stable HF.

Takotsubo cardiomyopathy induced by acute coronary syndrome: A case report.

Takotsubo cardiomyopathy (TC) can be provoked by various triggers. It should be differentiated from acute coronary syndrome (ACS). Herein, we report a case of TC triggered by ACS. An 80-year-old woman was referred to the emergency room because of prolonged chest pain and ST-segment elevations. Echocardiography demonstrated left ventricular apical ballooning, findings suggestive of TC rather than ACS. Emergency coronary angiography revealed severe stenosis of the first diagonal branch of the left anterior descending coronary artery with distal flow delay. Recanalization of the diagonal branch was achieved by stent implantation and her chest pain was resolved. Cardiac magnetic resonance imaging showed increased signal intensities in the apex and the inner layer of the anterior wall on fat-suppressed, T2-weighted imaging. The present case highlights the importance of recognizing TC in relation to ACS not only as a differential diagnosis but also as a possibly concomitant condition unless clinical features fit one diagnosis. Learning objective: Takotsubo cardiomyopathy can be provoked by various conditions and differentiated from acute coronary syndrome based on the presence or absence of coronary artery stenosis. Our case highlights the importance of acknowledging that takotsubo cardiomyopathy may be induced by acute coronary syndrome.

Simple Phonocardiography with the Ankle-Brachial Index Measurement System as a Tool for Detecting Fourth Heart Sounds of Hypertrophic Cardiomyopathy.

Conventional phonocardiography is useful for objective assessment of cardiac auscultation, but its availability is limited. More recently, an ankle-brachial index (ABI) measurement system equipped with simple phonocardiography has become widely used for diagnosing peripheral artery disease, however, whether this simple phonocardiography can be an alternative to conventional phonocardiography remains unclear.This retrospective study consisted of 48 patients with hypertrophic cardiomyopathy (HCM) and 107 controls. The presence of the fourth sound (S4) was assessed by conventional phonocardiography, in addition to apexcardiography and auscultation, in all patients with HCM. S4 was also estimated by the ABI measurement system with the phonocardiographic microphone on the sternum (the standard method) or at the apex (the apex method) in HCM patients and controls.S4 on conventional phonocardiography was detected in 42 of 48 patients (88%) with HCM. Auscultation for the detection of S4 had a sensitivity of 0.78, specificity of 0.57, and accuracy of 0.75. These diagnostic values were generally superior to those of the standard method using the ABI measurement system, whereas the apex method using the ABI measurement system had better diagnostic values, with an excellent specificity of 1.0, sensitivity of 0.77, and accuracy of 0.80. No significant differences were observed in low ABI defined as < 0.9.Simple phonocardiography equipped with the ABI measurement system may be an alternative to conventional phonocardiography for the detection of S4 in patients with HCM when the phonocardiographic microphone is moved from the sternum to the apex.

Jugular Venous Pressure Response to Inspiration for Risk Assessment of Heart Failure.

Simplifying jugular venous pressure (JVP), visibility of the right internal jugular vein above the right clavicle in the sitting position, has been proposed in the management of heart failure (HF) because of its convenience. However, this method may be undervalued for the detection of mildly to moderately increased JVP. Increased JVP on inspiration, known as Kussmaul sign, may be a useful physical finding in this condition. This study consisted of 138 patients who were admitted for the management of HF. Using this simple method, JVP was assessed at rest in the sitting position before discharge; its response to inspiration was also examined if no high JVP was noted at rest. The primary outcome was a composite of cardiac death and hospitalization for worsening HF. Among all the patients, 16 patients (12%) had high JVP at rest and another 16 patients (12%) had high JVP not at rest but on inspiration. During a follow-up period of 249 ± 182 days, a primary outcome event occurred in 63 patients (46%). The incidence of adverse cardiac events was higher in patients with a high JVP at rest (69%; hazard ratio 3.31, 95% confidence interval 1.64 to 6.67, p = 0.0009) and in patients with a high JVP on inspiration (56%; hazard ratio 2.18, 95% confidence interval 1.02 to 4.63, p = 0.043) than in patients without a high JVP in both conditions (41%). In conclusion, a high JVP not only at rest but also on inspiration was associated with a poor prognosis. The response of JVP to inspiration using this simple technique of physical examination may be a new approach in the management of HF.

Absent fourth heart sound as a marker of adverse events in hypertrophic cardiomyopathy with sinus rhythm.

BACKGROUND: Patients with hypertrophic cardiomyopathy (HCM) in sinus rhythm commonly show the fourth heart sound (S4). The lack of S4 may be a marker of impaired atrial function in HCM patients with sinus rhythm. METHODS AND RESULTS: This retrospective study consisted of 47 patients with HCM who had undergone phonocardiography and a cardiopulmonary exercise test. The primary outcome was a composite of cardiac death, stroke, hospitalization for worsening heart failure, and newly developed atrial fibrillation (AF). S4 was detected in 38 of 43 patients with sinus rhythm (88%). Peak oxygen consumption was the highest in 38 sinus rhythm patients with S4 (23.6 ± 5.6 mL/kg/min), middle in five sinus rhythm patients without S4 (19.3 ± 6.7 mL/kg/min), and lowest in four patients with AF (15.7 ± 3.3 mL/kg/min, p = 0.01). After a median of 40.5 months, the incidence of the primary outcome was higher in patients without S4 than in those with S4 (33% vs. 8%; hazard ratio, 6.17; 95% confidence interval, 1.02 - 37.4; p = .04) and higher in sinus rhythm patients without S4 than in those with S4 (60% vs. 8%; hazard ratio, 12.05; 95% confidence interval, 2.31 - 71.41; p = .007). CONCLUSIONS: The absence of S4 on phonocardiography was associated with impaired exercise tolerance and adverse cardiac events in HCM patients with sinus rhythm.

An Adult Case of Heart Failure due to Left Main Coronary Artery Atresia.

Left main coronary artery (LMCA) atresia is a rare congenital heart disease and can be fatal in pediatric patients. We report an adult case of LMCA atresia, in which heart failure developed without episodes suggesting angina. A 40-year-old man presented with difficulty breathing. Echocardiography revealed diffuse hypokinesis of the left ventricle with an ejection fraction of 22% in the absence of significant valvular disease. A diagnosis of heart failure was made, and diuretics, enalapril, bisoprolol and warfarin were administered. Coronary angiography demonstrated no trace of the ostium of the LMCA in the sinuses of Valsalva; the middle to distal part of the LMCA was visualized by rich collateral flow from the right coronary artery to the left anterior descending coronary artery and left circumflex coronary artery. No trace of the ostium of the LMCA from the aorta or main pulmonary artery was detected on computed tomography angiography or echocardiography. The patient underwent coronary artery bypass grafting and a final diagnosis of congenital atresia of LMCA was made. The clinical course was uneventful and computed tomography angiography, performed 5 days after surgery, showed a patent bypass graft. This case demonstrates the importance of considering LMCA atresia even in the absence of chest symptoms suggesting angina in patients with heart failure.

The Bezold-Jarisch Reflex in a Patient With Stanford Type A Acute Aortic Dissection.

BACKGROUND: The Bezold-Jarisch reflex is a depressor reflex of the heart due to the preferential distribution of vagal nerves in the inferior wall of the left ventricle. We report a case of Stanford type A acute aortic dissection, in which coronary spastic angina caused the Bezold-Jarisch reflex in the acute phase. CASE REPORT: A 53-year-old man presented with left chest pain and cold sweating. An electrocardiogram was normal and the high-sensitivity cardiac troponin T level was negative. A diagnosis of Stanford type A acute aortic dissection was made based on computed tomography (CT); there was no evidence of ischemic heart disease on coronary CT angiogram obtained simultaneously. While waiting for emergency surgical repair, chest pain worsened, followed by bradycardia and hypotension, along with ST-segment elevations in the inferior leads, all of which were resolved by conservative treatment. During surgery, no evidence to suggest an extension of the dissection to the ostium of the right coronary artery was observed. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: The present case highlights the importance of recognizing the Bezold-Jarisch reflex because this depressor reflex may require different management than other conditions.

Ventricular late potentials and myocardial fibrosis in hypertrophic cardiomyopathy.

AIMS: Ventricular late potentials (VLPs) represent delayed conduction due in part to myocardial fibrosis. We sought to examine the relationship of signal-averaged electrocardiography findings with myocardial fibrosis as assessed by cardiac magnetic resonance (CMR) in patients with hypertrophic cardiomyopathy (HCM). METHODS: This study consisted of 41 HCM patients with sinus rhythm who had undergone risk assessment including CMR and signal-averaged electrocardiography such as VLPs, filtered QRS duration, low amplitude signal duration of the terminal filtered QRS below 40 µV (LAS), and root mean square voltage of the late 40 ms of the filtered QRS (RMS). The concordance rate between VLPs and myocardial fibrosis as assessed by CMR was examined. RESULTS: Late gadolinium enhancement (LGE) on CMR was detected in 13 patients, and VLPs were detected in 14. Filtered QRS duration, LAS, RMS, and VLPs were not associated with LGE. The results of LGE and VLPs were concordant in 26 patients, whereas 15 exhibited discordance. Patients with discordance had a higher maximum wall thickness (24.1 ±â€¯4.0 mm versus 21.0 ±â€¯5.9 mm, p < 0.05), higher LGE volume (2.3 ±â€¯1.2 g/cm versus 0.0 ±â€¯0.8 g/cm, p < 0.01), lower LGE volume/the total number of sites with LGE (1.5 ±â€¯0.7 versus 3.1 ±â€¯2.8, p < 0.01), and predominant LGE location of the interventricular septum and anterior wall (60% versus 8%, p < 0.01) than patients with concordance. CONCLUSION: VLPs were not a reliable marker for the detection of myocardial fibrosis as assessed by LGE on CMR in our cohort of patients with HCM. CONDENSED ABSTRACT: Ventricular late potentials on signal-averaged electrocardiography represent delayed conduction due in part to myocardial fibrosis but were not an alternative to cardiac magnetic resonance for detecting myocardial fibrosis in patients with hypertrophic cardiomyopathy.

A Case of Isolated Septal Myocardial Infarction: Myocardial Perfusion-metabolism Mismatch as a Tool for Diagnosis.

Isolated septal myocardial infarction is an uncommon condition with diagnostic difficulty due to small infarction size and anatomical variations. We report a case of isolated septal myocardial infarction, in which the diagnosis was confirmed not by electrocardiographic, echocardiographic, or angiographic findings, but by nuclear imaging. A 46-year-old man with chest discomfort exhibited ST-segment elevations in leads V1 and V2, and borderline abnormalities of the septal wall motion on echocardiography. Emergency coronary angiography demonstrated delayed flow in the second septal branch of the left anterior descending coronary artery. Intravascular ultrasound showed plaque in the proximal portion of the septal branch without evidence of plaque rupture. No balloon angioplasty or stent implantation was required because the flow delay in the septal branch disappeared after the intravascular ultrasound procedure. Myocardial perfusion-metabolism mismatch, as assessed by resting thallium-201 and iodine-123-beta-methyl-p-iodophenyl-pentadecanoic acid, was seen in the mid-septal region.

Third and Fourth Heart Sounds and Myocardial Fibrosis in Hypertrophic Cardiomyopathy.

BACKGROUND: The 4th heart sound (S4) is commonly heard in patients with hypertrophic cardiomyopathy (HCM). The 3rd heart sound (S3) is also audible in HCM patients regardless of the presence or absence of heart failure. These extra heart sounds may be associated with myocardial fibrosis because myocardial fibrosis has been suggested to affect left ventricular compliance.Methods and Results:The present retrospective study evaluated 53 consecutive HCM patients with sinus rhythm who had no symptoms of heart failure and underwent an initial assessment including phonocardiography, echocardiography, and late gadolinium enhancement (LGE) magnetic resonance imaging (MRI). S3 was detected on phonocardiography in 13% of all patients, and S4 was recorded in 75% of patients. Patients with S3 had a higher incidence of LGE and larger LGE volumes (86% and 11.5±2.4 g/cm, respectively) than patients without S3 (33% and 2.5±0.8 g/cm, respectively; P=0.02 and P=0.002). The presence of S4 was not associated with MRI findings, including the incidence of LGE and LGE volume. The diagnostic value of S3 for the detection of LGE was highly specific (97%), with a low sensitivity (29%). CONCLUSIONS: Myocardial fibrosis, as assessed by LGE, was associated with S3 but not with S4 in patients with HCM. These results may contribute to the risk stratification of patients with HCM.

Unusual clinical course after surgical repair of unruptured aneurysm of sinus of Valsalva.

Aneurysms of the sinus of Valsalva are characterized by dilatation of at least one of the three aortic sinuses. We experienced a case with unruptured aneurysm of the right sinus of Valsalva, in which serial imaging studies were useful in assessing a rare complication after surgical repair. An asymptomatic 75-year-old man underwent patch closure of the aneurysm orifice because of progressive enlargement of the aneurysm. The postoperative course was uneventful, and computed tomography (CT), performed a week after the patch repair, showed no leakage of contrast medium into the isolated aneurysm. Three months later, echocardiography showed decreased size of the aneurysm with heterogeneous echogenicity and possible blood flow in the aneurysm, findings suggestive of thrombus formation and a recurrent fistula. CT with contrast medium showed partial recanalization between the patched aneurysm and the right sinus of Valsalva. Follow-up echocardiography, performed 1 year after surgery, revealed neither definite aneurysm nor shunt flow of Valsalva. The present case highlights that non-invasive follow-up can be an alternative option when carried out with caution in selected patients with incomplete closure of Valsalva aneurysm.

Notched QRS for the assessment of myocardial fibrosis in hypertrophic cardiomyopathy.

BACKGROUND: Patients with hypertrophic cardiomyopathy (HCM) sometimes develop myocardial fibrosis in association with adverse cardiovascular events. Electrocardiography (ECG) could provide helpful information on myocardial fibrosis in HCM, as in coronary artery disease. METHODS AND RESULTS: A total of 60 patients with HCM without bundle branch block underwent cardiac magnetic resonance imaging (CMR). The extent or location of late gadolinium enhancement (LGE) was examined in relation to 12-lead ECG. A notch on QRS was defined as at least 2 consecutive spikes in the same polarity with a reversal of direction ≥90° and the initial negative deflection ≥0.05 mV. LGE was associated with notched QRS, leftward QRS axis, and prolonged QRS duration, but not with any other findings such as abnormal Q waves, R-wave amplitude, or ST-T changes. Notched QRS was most useful in determining the presence or absence of myocardial fibrosis, with a sensitivity of 70% and a specificity of 81% using a cut-off of the number of leads with notched QRS ≥2. The number of notched QRS leads was positively correlated with LGE volume (P<0.01) and the lead distribution of notched QRS was informative about the location of LGE. CONCLUSIONS: A notch on QRS was useful in estimating myocardial fibrosis as assessed on CMR LGE in HCM patients without bundle branch block.

Right bundle branch block and ventricular septal fibrosis in patients with hypertrophic cardiomyopathy.

BACKGROUND: Right bundle branch block (RBBB) is associated with ventricular septal fibrosis in patients with hypertrophic cardiomyopathy (HCM) after alcohol septal ablation, but little data are available in HCM patients without a history of septal ablation. METHODS: Magnetic resonance late gadolinium enhancement (LGE) was performed in 59 HCM patients with no history of alcohol septal ablation. The location and extent of LGE were examined in relation to electrocardiographic features including RBBB. RESULTS: LGE volume was higher in 7 HCM patients with RBBB (7.3±7.4g/cm) than in patients without RBBB (2.9±7.4g/cm, p=0.016). LGE volume was positively correlated to QRS duration of RBBB (correlation coefficient=0.93, p=0.023). The diagnostic value of RBBB was highly specific for the detection of LGE in the ventricular septum, with sensitivity 21% and specificity 94%. CONCLUSIONS: The presence of RBBB may be a simple marker for detecting ventricular septal fibrosis in HCM patients who had no history of alcohol septal ablation. Further studies are necessary to confirm our findings.

Usefulness of high-sensitivity cardiac troponin T and brain natriuretic peptide as biomarkers of myocardial fibrosis in patients with hypertrophic cardiomyopathy.

Myocardial fibrosis assessed by late gadolinium enhancement cardiac magnetic resonance imaging is associated with cardiovascular events in patients with hypertrophic cardiomyopathy (HC), but few data are available regarding the utility of biomarkers for detecting late gadolinium enhancement. The aim of this study was to examine serum levels of myoglobin, cardiac myosin light chain I, high-sensitivity cardiac troponin T (hs-cTnT), and creatine kinase-MB isoenzyme and plasma levels of brain natriuretic peptide (BNP) in relation to late gadolinium enhancement in 53 patients with HC. Levels of hs-cTnT and BNP were higher in 23 patients with late gadolinium enhancement than in 30 patients without it (p <0.01 for both). An hs-cTnT level ≥0.007 ng/ml or a BNP level ≥70 pg/ml had good diagnostic value for detecting late gadolinium enhancement, with sensitivity of 96% or specificity of 90% with the combination of these 2 biomarkers. The extent of late gadolinium enhancement was correlated with BNP level (p <0.01) but not with hs-cTnT level in 23 patients with HC with late gadolinium enhancement. The increase in the extent of late gadolinium enhancement was related to hs-cTnT level in 8 patients during 22 months of follow-up (p = 0.02). In conclusion, the combination of hs-cTnT and BNP is useful in detecting myocardial fibrosis in patients with HC. The findings of this study indicate that hs-cTnT is a direct marker of ongoing myocardial fibrosis and that BNP is a marker of left ventricular overload partially associated with myocardial fibrosis.

Diagnostic performance of ultrasonic tissue characterization for subendocardial ischaemia in patients with hypertrophic cardiomyopathy.

AIMS: Hypertrophic cardiomyopathy (HCM) patients often develop left--ventricular subendocardial ischaemia, a cause of chest symptoms, despite normal epicardial coronary arteries. The aim of this study was to examine whether ultrasonic tissue characterization or late gadolinium enhancement on cardiac magnetic resonance imaging can detect subendocardial ischaemia in patients with HCM. METHODS AND RESULTS: Subendocardial ischaemia was quantified on exercise Tc-99m tetrofosmin myocardial scintigraphy in 29 non-obstructive HCM patients with asymmetric septal hypertrophy. Ultrasonic tissue characterization using cyclic variation of integrated backscatter (CV-IB) and late gadolinium enhancement on cardiac magnetic resonance imaging were analysed separately in the right halves and the left halves of the ventricular septum in relation to subendocardial ischaemia. Subendocardial ischaemia was identified in 17 (59%) patients. The ratio of CV-IB in the right-to-left halves of the ventricular septum was significantly higher in patients with subendocardial ischaemia (1.19 ± 0.10) than those without (0.84 ± 0.10, P = 0.04). The optimal cutoff for the detection of subendocardial ischaemia was the ratio of CV-IB >1.0, with a sensitivity of 80%, specificity of 71%, and accuracy of 76%. On the other hand, late gadolinium enhancement was not associated with subendocardial ischaemia in our cohort. CONCLUSION: Ultrasonic tissue characterization using CV-IB separately in the right and left halves of the ventricular septum, but not late gadolinium enhancement on magnetic resonance imaging, provided useful information in detecting subendocardial ischaemia in patients with HCM. Ultrasonic tissue characterization may be useful in selecting patients who will benefit from medications to relieve chest symptoms.

Metastatic chordoma to the left ventricle: A case report.

Chordoma is a rare neoplasm of bone that develops from the remnants of the primitive notochord. We present a 78-year-old woman with metastatic chordoma to the left ventricle. She had been diagnosed with sacral chordoma 5 years earlier, and chordoma gradually spread to numerous muscles, the ankle joint of the left leg, bilateral lungs, and the brain, despite repeated surgical excisions and carbon heavy-ion radiotherapy. Positron emission tomography/computed tomography showed an abnormal accumulation in the heart, with features similar to the other metastatic lesions. Transthoracic echocardiography could not provide a clear view of the heart, but multidetector computed tomography revealed that a giant abnormal mass was attached to the apical inferior segment of the left ventricle. The patient stated that she did not want to undergo surgical resection for the cardiac mass. The chordoma has been slowly increasing in size, but she has remained asymptomatic without cardiovascular events for more than a year since the diagnosis of cardiac metastasis. .

Sick sinus syndrome as a late complication after ventricular septal defect surgery.

Ventricular septal defect is a common congenital heart disease for which patch closure technique using cardiopulmonary bypass has been applied for a few decades, resulting in a low incidence of perioperative complications. We report a 26-year-old woman with bradycardia of 25-30 beats per minute, which was considered to be a late-onset complication of surgical closure of an isolated ventricular septal defect performed 21 years earlier. She has been stable for more than 9 months without an implanted pacemaker, although her heart rate was always around 40 beats per minute in scheduled follow-up visits. .

Beer-induced angina pectoris.

Moderate alcohol consumption is associated with reduced morbidity and mortality from coronary heart disease. However, alcohol has been reported to provoke myocardial ischemia in some cases. We report a 60-year-old man with beer-induced myocardial ischemia, which was clearly proved by beer-stress electrocardiography and beer-stress thallium-201 scintigraphy. .