Physical Examination as a Clue to Transthyretin Cardiac Amyloidosis: A Case Report.

Transthyretin (ATTR) cardiac amyloidosis has recently received increased attention; however, the diagnosis is often delayed. We present a case of ATTR cardiac amyloidosis in which a comprehensive history-taking and focused physical examination played an important role in establishing the diagnosis. A 75-year-old man was referred to the cardiology department for left ventricular hypertrophy on electrocardiography. No fourth sound was audible despite concentric biventricular hypertrophy and diastolic dysfunction on echocardiography. Additional history-taking revealed that he had undergone bilateral carpal tunnel syndrome surgery almost 35 years earlier and had a biceps tendon rupture about 15 years earlier; bunching of the arm on flexion, or Popeye's sign, was noted. Technetium-99m-pyrophosphate showed diffuse uptake not only in both ventricles but also in both atria. The findings were consistent with the absence of the fourth sound. The present case highlights the importance of a focused physical examination as well as history-taking as a clue to ATTR cardiac amyloidosis in patients with unexplained left ventricular hypertrophy.

Impaired Left Ventricular Contractile Reserve in Patients With Hypertrophic Cardiomyopathy and Abnormal Blood Pressure Response: A Stress Echocardiographic Study.

BACKGROUND: Abnormal blood pressure response (ABPR) has been reported to be a risk factor for sudden cardiac death in patients with hypertrophic cardiomyopathy (HCM). We aimed to elucidate the relationship between ABPR during exercise stress echocardiography (ESE) and impaired left ventricular (LV) contractile reserve based on two-dimensional strain in patients with HCM. METHODS: Patients with HCM underwent ESE with treadmill exercise. Patients whose blood pressure elevation at maximum workload was lower than 20 mmHg from baseline were classified as having ABPR. Echocardiographic parameters were compared between patients with and without ABPR.  Results: Of 26 patients with HCM, nine patients were diagnosed with ABPR. Significant LV outflow tract obstruction (>50 mmHg) was provoked only in one patient with ABPR (baseline to the conclusion of the exercise, 15.2 mmHg to 63.0 mmHg). Change in cardiac output (CO) and the ratio of early diastolic velocity to early annular velocity (E/e') from baseline to just after the conclusion of exercise did not differ between patients with and without ABPR (CO, 102±40% vs. 122±45%, P = 0.19; E/e', 4±22% vs. 2±20%, P = 0.86). Change in systemic vascular resistance change was not significant (patients with vs. without ABPR, -52±10% vs. -46±13%, P = 0.24). Percent change in LV global longitudinal strain was lower in patients with ABPR than patients without ABPR (12±17% vs. 27±15%, P = 0.02). CONCLUSION: In conclusion, impaired LV contractile reserve during exercise might contribute to ABPR in patients with HCM.

Exercise-induced diastolic paradoxical jet flow in a patient with hypertrophic cardiomyopathy.

Early diastolic flow from the apex toward the base of the left ventricle - diastolic paradoxical jet flow - may be observed at rest in patients with hypertrophic cardiomyopathy (HCM). We herein report a case of HCM with exercise-induced diastolic paradoxical jet flow, accompanied by an apical myocardial perfusion abnormality of the left ventricle. A 56-year-old man was referred for the further evaluation of abnormal electrocardiography at a medical check-up. Echocardiography showed myocardial hypertrophy predominantly in the apex of the left ventricle with a maximum wall thickness of 27 mm without an apical aneurysm. Paradoxical jet flow was not detected at rest, but developed after treadmill exercise and lasted for approximately six minutes. Exercise scintigraphy with thallium-201 showed reduced tracer uptake in the left ventricular apex with complete redistribution, findings consistent with myocardial ischemia of the left ventricular apex.

Silent pulmonary artery dissection in a centenarian.

Pulmonary artery dissection is an extremely rare and fatal disease that is difficult to diagnose. We report a case of a 97-year-old woman with heart failure, who was diagnosed as having silent pulmonary artery dissection by chance during transthoracic echocardiography. Surgical treatment for pulmonary artery dissection was not performed, but the patient has been doing well in a sanatorium for more than 2 years, being a centenarian.