Cardiac amyloidosis is a manifestation of amyloidosis which is a multisystem disorder. This is difficult to diagnose, rare disease which eventually leads to the mortality. Diagnosis requires a high index of clinical suspicion along with echocardiographic clues like, diastolic dysfunction, bi-atrial enlargement and ventricular thickening. Treatment is mainly supportive with disappointing outcomes. We present a case of systemic amyloidosis with negative congo red staining, presenting with predominantly cardiac features.
Background: Hypertrophic cardiomyopathy (HCM) is a genetic disorder of cardiac myocytes that is characterized by cardiac hypertrophy, cellular disarray and interstitial fibrosis. Mutation of MYH7 and MYBPC3 encoding proteins β-myosin heavy chain and myosin binding protein C, respectively, are the two most common genes involved, together accounting for about 50% of cases. The present study was conducted to evaluate the prognostic value of MR-proANP in patients with HCM. Subjects and Methods: The present study was conducted at Narayana Medical College & Hospital, Chintareddy Palem, Nellore, Andhra Pradesh from July 2016 to July 2017 on 46 patients of hypertrophic cardiomyopathy of both genders. Serum NT-proBNP was measured by a two-site electrochemiluminescence immunoassay on a Roche Diagnostics E170 analyser. Results: The mean systolic blood pressure was 124.8 mm Hg, diastolic blood pressure was 78 mm Hg, heart rate was 68 beats/minutes, NYHA 1(n=4), NYHA 2 (n=27), NYHA 3–4 (n=15) and atrial fibrillation was 12%. The mean MR-proANP was 106 pmol/L and NT-proBNP was 540 pg/mL at the start of the study. At the end of 12 months of follow up, 15 patients had a primary end point defined as heart failure hospitalisation (n=10), heart transplant (n=3) death(n=2). Both mean MR-proANP and NT-proBNP were strongly associated with the primary end points at the end of study period with values of 1010 pmol/L and 2545 pg/ml respectively. This showed a strong association with P <0.05. Conclusion: Authors found that MR-proANP is a valuable biomarker for the prediction of heart failure related events in patients with HCM.
Background: In the past, the left atrial appendage (LAA) has been considered to be a relatively insignificant portion of cardiac anatomy. It is now recognized that it is a structure with important pathological associations. First, thrombus has a predilection to form within the LAA in patients with non-valvar atrial fibrillation and to a lesser extent in those with mitral valve disease (both in atrial fibrillation and in sinus rhythm). Second, the use of transoesophageal echocardiography has made clear imaging of the LAA possible, so that its size, shape, flow pattern, and content can be assessed in health and disease. Subjects and Methods: This study population consisted of 40 patients with symptomatic mitral stenosis who underwent percutaneous mitral balloon valvotomy in the cardiology department of GSL medical college, Rajahmundry over a time period of 1 April 2017 to 30 March2018. Patients in all age groups, with evidence of severe MS (MVA<1.0cm2) admitted in our institution, in whom PBMV was feasible were included. Those who were fulfilling the PBMV intervention criteria and those who had good results only were included. Results: Left atrial appendage late emptying velocity, LAALF: Left atrial appendage late filling velocity Spontaneous echocontrast (SEC) was present in 10 of the 40 patients before a procedure but completely disappeared (6 patients) or decreased (4 patients) after the procedure. LAALE & LAALF velocities measured by Doppler were increased significantly after PBMV and at 6 months follow up compared with baseline (P <0.001). Conclusion: Successful Percutaneous balloon mitral valvotomy decreases the intensity of spontaneous LA contrast, reduces the size of the LA, and improves LA and LAA function. Relief of MS may confer not only hemodynamic benefits for improvement of symptoms but also have a favorable influence on future thromboembolism.
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