Current awareness and use of transthoracic echocardiography in evaluation of valvular heart disease in Kumamoto Prefecture -a report from the Kumamoto cardiovascular echocardiography standardization project.

BACKGROUND: There are few reports on transthoracic echocardiography (TTE) for the evaluation of valvular heart disease in a specific area or region. METHODS AND RESULTS: This cross-sectional questionnaire-based survey was conducted in 2023 in Kumamoto Prefecture, where 106 hospitals provide cardiology services. Ninety-three (88%) of the hospitals completed questionnaires regarding TTE. The severity of low flow/low gradient AS was evaluated by dobutamine stress echocardiography in only 7% of hospitals and exercise stress echocardiography for asymptomatic mitral regurgitation in only 5%. Multivariate logistic regression analysis revealed that participation in remote multi-institutional echocardiographic meetings and use of the Kumamoto Prefecture echocardiographic manual were significantly associated with the use of a multi-window approach (P < 0.05). CONCLUSIONS: In Kumamoto Prefecture, echocardiographic measurements are performed according to the recommendations at a relatively low rate. Dissemination of recommendations through remote meetings and the use of the echocardiographic manual may increase the likelihood of TTE being performed according to the recommendations.

Advent of paradoxical flow after antihypertensive treatment in mid-ventricular obstructive hypertrophic cardiomyopathy - A case report.

A 69-year-old woman was admitted our hospital due to acute cerebral infarction. Transthoracic echocardiography showed massive left ventricular (LV) hypertrophy with small ventricles and normal LV ejection fraction. Apical 4-chamber and longitudinal images showed mild LV obstruction. After treatment for hypertension, her blood pressure decreased from 208/129 mmHg to 150/68 mmHg. Pulsed Doppler echocardiography revealed new paradoxical flow at the mid-ventricle. Decreased LV pressure following treatment with antihypertensive medications may have contributed to the development of early mid-ventricular obstruction and paradoxical flow in the present case. Learning objective: In mid-ventricular obstructive cardiomyopathy, apical aneurysm may be present and cause serious complications such as rupture of the apex and sudden death. In the present case, apical aneurysm newly developed after treatment for hypertension was suggested by advent of paradoxical flow. This case suggests that intraventricular hemodynamic change may become a trigger of paradoxical flow and apical aneurysm, becoming a risk of serious complication.

Severe hypotension during junctional rhythm in a patient with multiple cerebral infarcts.

An 82-year-old woman with parkinsonism and Lewy body dementia was re-admitted to our hospital due to convulsions and the recurrence of cerebral infarction. Parasternal transthoracic echocardiography showed normal left ventricular wall thickness and wall motion. To treat marked bradycardia and hypotension, she underwent temporary pacing. However, she lost her consciousness, when her blood pressure could not be measured. Simultaneous electrocardiogram and blood pressure monitoring showed that systolic blood pressure decreased by almost 30 mmHg from sinus rhythm to junctional rhythm. In the present case of acute cerebral infarction, severe hypotension occurred during junctional rhythm possibly contributed by parkinsonism and Lewy body dementia. Learning objective: As patients with junctional rhythm usually have no or mild symptoms, there are no specific guidelines for its evaluation and treatment. However, severe symptoms such as hypotension, cerebral infarction, loss of consciousness, or breathlessness may occur in some cases. Holter electrocardiogram with 24-hour noninvasive blood pressure monitoring may be helpful in such severe cases.

Mid-ventricular Obstructive Cardiomyopathy after Takotsubo Cardiomyopathy.

A 93-year-old woman was transferred to our hospital for lightheadedness. She had had Takotsubo cardiomyopathy for seven years. Transthoracic apical four-chamber echocardiography showed a large apical aneurysm. Pulsed-wave Doppler echocardiography at the left ventricular (LV) basal obstruction showed flow directed from the apex to the base during systole and isovolumic relaxation time. The patient was therefore diagnosed with mid-ventricular obstructive cardiomyopathy with a large apical aneurysm and paradoxical flow. The present case suggests that Takotsubo cardiomyopathy may become mid-ventricular obstructive hypertrophic cardiomyopathy without change in its structure.

Recurrent Syncope in a Case with Mediastinal Lymphoma.

An 83-year-old man suddenly lost consciousness, and his blood pressure dropped. Results of blood analysis were within normal range. A chest radiograph showed enlargement of the left mediastinum. Computed tomographic scan of the chest showed a large mass surrounding the left common carotid artery in the left upper mediastinum. Histology revealed a large B-cell lymphoma. A Holter ECG showed transient sinus bradycardia and atrioventricular block. The power spectrum revealed increase in power of high frequency, suggesting that general vagal activity might be related to bradycardia. Evaluation of autonomic system from high frequency and low frequency components may be useful in examining the mechanisms of sudden bradycardia and/or hypotension of unknown origin.

Constrictive pericarditis following mRNA COVID-19 vaccination in a patient with systemic sclerosis.

A 59-year-old man with systemic sclerosis and interstitial pneumonia was referred to our department because he developed dyspnea and leg edema after receiving a first shot of coronavirus disease 2019 (COVID-19) vaccine. Transthoracic echocardiography showed moderate pericardial effusion with conspicuous fibrin deposition. Prednisolone was increased from 6 mg/day for systemic sclerosis to 20 mg/day. Thereafter, pericardial effusion gradually decreased. However, his symptoms continued. Transthoracic echocardiography showed disappearance of pericardial effusion and thickened pericardium. Pulsed-wave and tissue Doppler echocardiography revealed that the patient suffered from newly developed constrictive pericarditis. COVID-19 vaccination might have contributed to acute pericarditis and subsequent constrictive pericarditis in the present case of systemic sclerosis and pulmonary fibrosis. Learning objective: Incidence of adverse effects after coronavirus disease 2019 vaccination is rare. The present case suggests the risk of pericarditis that may lead to constrictive pericarditis.

Incidence, clinical characteristics, and diagnostic approach in transthyretin amyloid cardiomyopathy: The Kumamoto Cardiac Amyloidosis Survey.

BACKGROUND: In recent years, transthyretin amyloid cardiomyopathy (ATTR-CM) has received increasing attention; however, the epidemiology of ATTR-CM in Japan is not yet understood. In the Kumamoto Cardiac Amyloid Survey, we evaluated the current incidence, clinical characteristics, diagnostic approaches, and treatment strategies for ATTR-CM and compared tafamidis-prescription hospitals with regional hospitals. METHODS: We conducted a retrospective multicenter observational cohort study. The registry included patients with ATTR-CM diagnosed in two tafamidis-prescription hospital institutes [Japanese Circulation Society (JCS)-certified facilities] and 15 regional cardiovascular facilities in Kumamoto between January 2018 and December 2020. RESULTS: In total, 174 patients were diagnosed with ATTR-CM. The incidence of ATTR-CM was estimated to be approximately 1 per 10,000 person-years in the elderly population (>65 years old) in Kumamoto. Compared with that in the JCS-certified facilities cohort (n=115), age at diagnosis was significantly older (84.5 ± 5.6 vs. 77.5 ± 6.3 years old; p<0.01) in the regional hospitals cohort (n=59). Histological (25% vs. 81%; p<0.01) and genetic diagnosis (7% vs. 82%) were also less frequently performed. Probable (as indicated by positive bone scintigraphy findings with confirmation of monoclonal protein absence) and possible (as indicated by positive bone scintigraphy findings without confirmation of monoclonal protein absence) ATTR-CM accounted for the majority of cases (75% vs. 19%; p<0.01) in the regional hospitals cohort compared to the JCS-certified facilities cohort. There were no cases of hereditary ATTR-CM among the patients who underwent TTR genetic testing (n=98). CONCLUSIONS: We confirmed the incidence of ATTR-CM in Kumamoto and the diagnostic approach used in patients with ATTR-CM. Further prospective studies with a larger sample are needed to validate our results and to further shed light on the epidemiology of ATTR-CM in Japan.

A case of malignant hypertension with multi-organ injury.

A 33-year-old woman visited our hospital due to visual loss. Her BP was 280/150 mm Hg and pulse rate was 111 beats per minute. A urinalysis showed protein in urine, suggesting kidney injury. A transthoracic echocardiography showed left ventricular hypertrophy. A Cardiac magnetic resonance imaging suggested left ventricular endocardial edema or inflammation. Ophthalmoscopy showed optic disc edema and hard exudates in both eyes. A brain MRI showed multiple high-intensity areas at the pons and white matter of the cerebrum and cerebellum. Although the patient had malignant hypertension, she was successfully treated by medication.

Current Awareness and Status of Venous Ultrasonography in Kumamoto Prefecture　- A Report of the Kumamoto Cardiovascular Echocardiography Standardization Project.

Background: There are few reports on the current awareness and status of venous ultrasonography, including the number of specialists who perform this procedure, in a specific regional area in Japan. Methods and Results: This cross-sectional survey study was conducted in Kumamoto Prefecture from October 2018 to March 2019. Of the 366 medical institutions providing cardiology services in Kumamoto Prefecture, 259 (101 general hospitals, 158 small clinics) responded to our questionnaire. In 2017, 21,773 venous ultrasound tests were performed, 21,101 (97%) of which were performed in hospitals and only 672 (3%) were performed in clinics. Both the number of institutions performing venous ultrasounds and the number of tests performed increased over time. Although 317 medical staff in Kumamoto Prefecture were performing transthoracic echocardiography (TTE) when the questionnaires were collected, only 210 performed venous ultrasounds. Although 91% (61/67) of medical institutions could perform TTE within 30 min, only 77% (53/69) performed venous ultrasounds within 30 min. The number of venous ultrasounds per population×100 was largest in the Kumamoto and Kamimashiki areas (1.67) and smallest in the Kamoto area (0.05). Conclusions: This is the first report to reveal the current awareness and status of venous ultrasonography in a specific region in Japan. There are several problems to be overcome, such as a lack of venous ultrasound specialists and the regional disparity in venous ultrasounds in Kumamoto Prefecture.

A Coronary Artery-left Ventricular Fistula through the Sinusoid.

A 78-year-old woman was referred to our hospital because of repetitive suppurative arthritis at the artificial left knee joint. Her plasma brain natriuretic peptide level was 122 pg/mL. A 12-lead electrocardiogram showed a QS pattern in the inferior leads. A two-dimensional echocardiogram revealed hypokinesis at the inferior wall and hypertrophy at the apical lateral wall. Color flow imaging revealed this hypertrophic region to be a myocardial sinusoid, demonstrating diastolic coronary to left ventricular flow and early systolic flow vice versa. This was a very rare case of coronary to left ventricular fistula through a sinusoid without cyanotic congenital heart disease or severe coronary artery disease.

Left Ventricular Apical Thrombus Mimicking Hypertrabeculation.

A 73-year-old man visited our hospital due to dyspnea and epigastralgia. His plasma brain natriuretic peptide level was 1,205 pg/mL. A 12-lead electrocardiogram showed ST segment depression in leads I, V5, and V6. Transthoracic echocardiography showed dilatation and severe hypokinesis of the left ventricle. Hypertrabeculation was observed at the septum, apex, and lateral wall. Delayed enhancement of cardiac magnetic resonance imaging revealed a relatively low uptake of contrast agent at a large apical trabecula. After treatment with diuretics, follow-up echocardiography showed the disappearance of the controversial apical trabecula, which was later confirmed to have been a thrombus.

Late-gadolinium enhancement in a subject with normal left ventricular function.

A 27-year-old man visited our hospital after experiencing palpitations. His 12-lead electrocardiogram and chest radiograph were unremarkable. Blood test results showed normal plasma brain natriuretic peptide level (<5.8 pg/mL). Transthoracic echocardiography revealed normal left ventricular structure and function by demonstrating left ventricular wall thickness of 10 mm, end-diastolic dimension of 46 mm, end-systolic dimension of 31 mm, and ejection fraction of 64%. Pulsed-wave Doppler echocardiography demonstrated normal E/e' ratio of 7.5. Cardiac magnetic resonance imaging showed normal coronary artery. However, there was massive late-gadolinium enhancement at the mid-layer wall, suggesting massive left ventricular fibrosis. This case reveals that left ventricular function may be normal even in massive late-gadolinium enhancement. Pathophysiology other than fibrosis might have contributed to this specific finding in late-gadolinium enhancement.

Subdivision of the left ventricle by a discrete ridge and anomalous papillary muscle causing mid-ventricular obstruction: A case report.

A 16-year-old healthy boy visited our department because of a heart murmur. A 12-lead electrocardiogram showed left QRS axis deviation and repolarization abnormalities. Transthoracic echocardiography and a computed tomographic scan revealed a hypertrophied papillary muscle and a discrete ridge arising from the septal wall, causing mid-ventricular obstruction. Doppler echocardiography revealed that the pressure gradient at the obstruction was mild. The patient will be followed up annually, without medication or physical restriction.

Triphasic mitral inflow pattern associated with hemodynamic deterioration in anemia or mitral regurgitation: a report of two cases.

We present two cases with a mid-diastolic mitral inflow peak called an "L wave" due to underlying diseases. Case 1 was an 81-year-old woman with severe anemia (hemoglobin level of 3.9 g/dL). Pulsed Doppler echocardiography showed an L wave that disappeared after transfusion of red cell concentrates. Case 2 was a 72-year-old woman with severe mitral regurgitation due to ruptured chordae tendineae. Pulsed Doppler echocardiography showed an L wave that disappeared after mitral valve repair. The present report suggests that both severe anemia and mitral regurgitation might have contributed to L wave formation, which suggests hemodynamic deterioration.