Michiyo Yamano

Early cardiac involvement in senile systemic amyloidosis: a case report

Senile systemic amyloidosis (SSA) is caused by the deposition of wild-type transthyretin (TTR)-derived amyloid fibrils. This type of amyloidosis is not rare in elderly individuals over the age of 80 and is usually detected on postmortem microscopic examination of myocardium. We report a 67-year-old male patient who was clinically diagnosed as having SSA with cardiac involvement. The initial event was cerebral infarction conceivably due to cardiac embolism. Endomyocardial biopsy was performed twice, which led to a definitive diagnosis of amyloidosis at the second biopsy. This amyloid was immunolabeled by an anti-TTR antibody and direct DNA sequencing of the TTR gene did not detect any mutation. Clinical confirmation of SSA in individuals before the age of 70 is infrequent and cardiac amyloidosis associated with this disease might have been the cause of cerebral embolism in our patient. Additionally, it is important to distinguish cardiac amyloidosis of SSA from that of primary immunoglobulin light chain (AL)-derived amyloidosis, because the treatment and prognosis differ considerably from those of primary AL amyloidosis.

Mitral Valve Prolapse Revisited.

A 75-year-old man was admitted with abrupt onset of dyspnea during sleep. On examination, his blood pressure was 222/165 mm Hg, pulse was 160 bpm, and oxygen saturation was 75% while breathing ambient air. Heart sounds were difficult to assess because of coarse crackles and his moaning. There was no edema in the extremities. An ECG showed sinus tachycardia with a QRS duration of 111 milliseconds, and a chest radiograph showed pulmonary edema without cardiomegaly. A presumed diagnosis of acute heart failure was made, and isosorbide dinitrate was administered intravenously with supplemental oxygen via a face mask. Cardiac auscultation after stabilization of vital signs showed a holosystolic murmur at the apex and an early systolic click at the left sternal border. Mitral regurgitation resulting from mitral valve prolapse (MVP) was most likely but was not confirmed by transthoracic echocardiography because of a lack of definitive evidence (Figure 1). Of note, another extra sound during early diastole was heard best at the third left sternal border. Figure 1. Apical 2-chamber views of the left ventricle (LV) on transthoracic echocardiography show insufficient coaptation of the mitral leaflets ( A , arrow) and …

Murmur Associated with Diastolic Paradoxical Jet Flow in a 43-Year-Old Man with Hypertrophic Cardiomyopathy

A diastolic paradoxical jet flow, often seen in patients with hypertrophic cardiomyopathy, is a unique flow from the apex toward the base of the left ventricle during isovolumic relaxation. To date, this phenomenon appears to have been noninvasively detected only on echocardiograms. We report the case of a 43-year-old man with hypertrophic cardiomyopathy and a diastolic paradoxical jet flow, in whom cardiac auscultation revealed a soft S4, a systolic ejection murmur, and a low-pitched early diastolic murmur immediately after S2 at the apex. On comparing his echocardiographic findings with those on phonocardiograms and apexcardiograms, we confirmed that the unusual murmur coincided with the diastolic jet flow. To our knowledge, this is the first case in which heart murmurs associated with a diastolic paradoxical jet flow have been clearly described. Because these flows can increase the risk of adverse outcomes, detecting any associated murmurs by methods other than echocardiography is worthwhile, even in the era of advanced imaging techniques.

Imaging of Right-to-Left Shunt in an Adult Patient with Unroofed Coronary Sinus with Persistent Left Superior Vena Cava: Diagnostic Clue and Pitfall

A 54-year-old woman with a history of multiple cardiac surgeries suffered from hypoxemia caused by a right-to-left intra-cardiac shunt due to coronary sinus (CS) anomaly with persistent left superior vena cava (PLSVC). Both the contrast echocardiography and enhanced computed tomography (CT) provided conclusive diagnosis of this rare congenital anomaly, which was overlooked for a long time. However, an important diagnostic clue was left-arm injection of the contrast media. In the present case, previously performed enhanced CT with its routine manner, i.e., contrast through the right arm, missed this anomaly. It is crucial to note that the unusual type of unroofed CS with PLSVC, presenting with an entirely right-to-left intra-cardiac shunt, cannot be delineated on an enhanced routine chest CT if the contrast media is injected through the right arm.

Brain Natriuretic Peptide Levels and Long-Term Outcomes in Acute Decompensated Heart Failure

Background: Brain natriuretic peptide (BNP) is a useful biomarker for managing patients with heart failure. Left ventricular morphology is an important determinant of left ventricular wall stress or BNP secretion, but little data is available regarding the long-term prognostic value of BNP in relation to left ventricular morphology in patients with heart failure. Methods: Plasma BNP levels and left ventricular morphology including end-diastolic/end-systolic volume and mass index on echocardiography were assessed before discharge in 89 consecutive patients hospitalized with acute decompensated heart failure. All patients were prospectively followed for cardiac events defined as cardiac death or rehospitalization due to heart failure. Results: During a follow-up period of up to 10 years, 28 patients died and 24 survived with cardiac events. Levels of BNP in nonsurvivors, survivors with cardiac events, and survivors without cardiac events were as follows: 240 ± 107 pg/ml, 357 ± 71 pg/ml, and 177 ± 23 pg/ml at 1 year after discharge (P = 0.01); 300 ± 63 pg/ml, 307 ± 66 pg/ml, and 157 ± 24 pg/ml at 5 years (P = 0.03); and 357 ± 65 pg/ml, 222 ± 43 pg/ml, and 145 ± 25 pg/ml at 10 years (P

Sludge in a giant left atrium

A man aged 78 years who had been diagnosed with end-stage hypertrophic cardiomyopathy presented with dyspnoea. He was admitted to our hospital with a diagnosis of acutely exacerbated chronic heart failure. The patient had previously undergone bioprosthetic mitral valve replacement for non-rheumatic mitral regurgitation and pacemaker implantation for atrial fibrillation, with a slow ventricular response. He had received anticoagulation therapy (warfarin). Chest radiography revealed cardiomegaly with a markedly enlarged left atrial appendage (figure 1A). CT revealed …