World Cardiology Experts Meeting July 22-23, 2019 Kyoto Japan

Dr. Shaun Khanna has completed his MBBS in 2016 from Bond University and is currently completing his Master of Medicine (Advanced) with the University of Sydney. He has a current appointment with Blacktown Hospital as a medical officer and as a conjoint lecturer with the University of Western Sydney. He is heavily involved in research with interests in arrhythmias and cardiovascular imaging.

Systemic amyloidosis is a disease that causes extracellular deposition of misassembled proteins in various organs causing dysfunction. Wild-type Transthyretin amyloidosis (ATTRwt) accounts for 18% of all cases of cardiac amyloidosis. \r\nWe describe a case of a 73-year-old African gentleman who frequently presented to our facility with recurrent episodes of CCF. There was progressive deterioration noted in his left ventricular systolic function and worsening diastolic dysfunction (E’E of 18) leading to a RV biopsy. The biopsy results revealed the diagnosis of cardiac amyloid disease with typical apple green birefringence under polarized light. See Figure 1. He was followed up in haematology clinic and diagnosed with ATTRwt amyloidosis.\r\nHe has multiple other comorbidites including of VT arrest requiring AICD insertion, atrial arrhythmias, cirrhosis, portal hypertension complicated by recurrent ascites and chronic kidney disease (secondary to Type V Cardio Renal Syndrome). During his last admission, he was inotrope dependant which unfortunately failed to improve his renal function, subsequently becoming anuric (Cr 643 umol/L) with uremic complications. He passed away within weeks of this episode. \r\nCardiac Amyloidosis carries a significant heart failure and arrhythmia burden, with a median survival of 13 months. Echocardiograph typically showed concentric wall thickening and abnormal myocardial sparkling texture. Definitive diagnosis remains via endomyocardial biopsy. Type 5 Cardio-renal syndrome is a poorly understood entity that further worsens the prognosis in these patients. Further studies are required in both ATTRwt and CRS to improve morbidity and mortality. \r\n

Dr. Shaun Khanna has completed his MBBS in 2016 from Bond University and is currently completing his Master of Medicine (Advanced) with the University of Sydney. He has a current appointment with Blacktown Hospital as a medical officer and as a conjoint lecturer with the University of Western Sydney. He is heavily involved in research with interests in arrhythmias and cardiovascular imaging.

Left Ventricular non-compaction (LVNC) is rarely seen in the clinical setting with prevalence described as affecting approximately 8 – 12 patients per million per year.\r\nHere, we explore a case of a 63-year-old gentleman who presented to our facility after a cardiac syncope. His examination and ECG were completely normal. Other investigations; CT Brain, MRI DWI, EEG and CT Coronary Angiogram (CTCA) were all normal as well. He subsequently had a transthoracic echocardiogram (TTE) which showed normal left cavity size with mild impaired systolic function and marked LVH with anteroseptal hypokinesis. \r\nThe patient subsequently had an inpatient cardiac MRI done to delineate a cause of the syncope. The results of the Cardiac MRI indicated regional left ventricular non-compaction in the mid-distal lateral wall and apex, with the greatest ratio of non-compacted to compacted myocardium (NC:C): 4.3. \r\nThe patient’s syncope was considered arrhythmogenic and hence went on to have an automated implantable cardioverter-defibrillator (AICD) insertion for primary prevention. The patient was subsequently discharged with outpatient follow-up with a Cardiac Geneticist. \r\nLVNC is a congenital cardiomyopathy results in a spongy myocardium and deep trabeculations in the myocardial wall. Echocardiographic criterias developed by Chin, Jenni and Stöllberger, assess trabeculations, LV wall thickness and NC:C ratio of >2: 1. Peterson has described diagnosis of LVNC using CMR. The ratio of noncompacted myocardium to compacted myocardium during diastole should be greater than 2.3 (sensitivity of 86% and specificity of 99%). \r\n