Utility and pitfalls of the electrocardiogram in the evaluation of cardiac amyloidosis.

BACKGROUND: Cardiac amyloidosis is a protein misfolding disorder involving deposition of amyloid fibril proteins in the heart. The associated fibrosis of the conduction tissue results in conduction abnormalities and arrhythmias. "Classical" electrocardiogram (ECG) findings in cardiac amyloidosis include that of low voltage complexes with increased left ventricular wall thickness on echocardiography. However, this "classical" finding is neither sensitive nor specific. As cardiac amyloidosis is associated with a generally poor prognosis, the need for early recognition of this disease is important given the availability of new treatment options. In this review, we highlight 3 cases of patients with cardiac amyloidosis. Although presenting with typical clinical signs and symptoms, ECG for all 3 patients was not consistent with the classical findings described. They underwent further diagnostic tests which clinched the diagnosis of cardiac amyloidosis, allowing patients to receive targeted treatment. Through the review of the literature, we will highlight the different ECG patterns in patients with different types of cardiac amyloidosis and clinical scenarios, as well as the pitfalls of using ECG to identify the condition. Lastly, we also emphasize the current paradigms in diagnosing cardiac amyloidosis through the non-invasive methods of echocardiography, cardiac magnetic resonance imaging, and nuclear technetium-pyrophosphate imaging. CONCLUSIONS: Electrocardiogram is often the first investigation used in evaluating many cardiac disorders, including cardiac amyloidosis. However, classical features of cardiac amyloidosis on ECG are often not present. A keen understanding on the ECG features of cardiac amyloidosis and knowledge of the diagnostic workflow is important to diagnose this condition.

Severe Functional Tricuspid Valve Regurgitation: Predictors of Mortality After Initial Diagnosis.

BACKGROUND: Severe functional tricuspid regurgitation (fTR) is associated with adverse clinical outcomes and remains under-treated. There is recent interest in this disease due to emerging tricuspid valve therapies. However, the timing and selection of patients who may benefit from treatment is uncertain. Risk factors associated with mortality after diagnosis of severe fTR may help guide treatment. AIM: We studied patients with severe fTR to assess predictors of mortality. METHODS: We retrospectively identified consecutive patients who had severe fTR diagnosed on transthoracic echocardiography in a single academic tertiary hospital. These were categorised into atrial fibrillation (AF)- and non-AF-related groups. Patient characteristics and echocardiographic parameters were collected. We then analysed the collected parameters on their impact on occurrence of mortality and also on the time to mortality. RESULTS: A total of 635 patients with severe fTR were studied (41.6 % male, mean age of 68.6±15.4 yrs). There were 130 (20.5%) in the AF-related group and 505 (79.5%) in the non-AF related-group. Median follow-up duration was 774 days, during which 154 (24.3%) deaths occurred within the first year. Older age on diagnosis, reduced left ventricular ejection fraction (LVEF) (<50%), high pulmonary systolic pressure (PASP) (>50 mmHg) and a prior history of heart failure admissions were associated with occurrence of mortality. Older age on diagnosis, reduced LVEF, and high PASP were also found to be associated with time to mortality. CONCLUSIONS: For patients diagnosed with severe fTR, advanced age on diagnosis, prior heart failure admission, LVEF <50%, and PASP >50 mmHg are associated with mortality. These factors could form the basis of future studies that determine the timing and decision to intervene in patients with severe fTR.

Clinical outcomes of severe tricuspid valve infective endocarditis related to intravenous drug abuse - a case series.

BACKGROUND: Right-sided infective endocarditis (IE) related to intravenous drug use (IVDU) can follow an acute fulminant course. However, there is limited information on its longer-term clinical outcomes. AIM AND METHODS: We assessed a cohort of consecutive patients who presented with IVDU complicated by severe tricuspid valve regurgitation to determine their presentation, treatment, and long-term outcomes. In this study, severe tricuspid regurgitation (TR) was defined by the European Association of Cardiovascular Imaging criteria at initial presentation to the hospital. RESULTS: Thirty-three patients with a mean age of 35 ± 18 years (72% males) presented with IVDU associated with severe TR. At the initial presentation, 15 patients were in septic shock and required inotropes. 26 patients had septic pulmonary emboli; 10 patients had associated metastatic systemic sites of infection of which 5 patients had central nervous system (CNS) involvement. Three patients were in disseminated intravascular coagulation (DIC) and 1 patient had multi-organ failure (MOF), but not requiring dialysis or mechanical ventilation. Most patients had large tricuspid valve vegetations of >20mm. Eleven patients underwent surgery with 18% perioperative mortality. The Median follow-up was 6.4 years (0.5-11.4). Recurrent IE occurred in one-third of patients, the overall incidence of heart failure and Atrial fibrillation (AF) on follow-up was low in all 3 groups. Five-year survival was 94%. CONCLUSION: Acute severe TR following associated endocarditis IVDU results in a fulminant initial presentation, but a longer-term prognosis is good with surgical and medical treatment.

Single Coronary Artery in a Young Male with Chest Pain.

A young male presented to the hospital with chest pain. A coronary angiogram and a subsequent computed tomography coronary angiogram revealed a single coronary artery arising from the right coronary sinus which bifurcated into the right coronary artery and a large branch which supplies the left coronary artery territory. (Level of Difficulty: Beginner.).

Asymptomatic phaeochromocytoma in a patient with Holt-Oram syndrome: a case report.

BACKGROUND: Holt-Oram syndrome (HOS) is a rare congenital disease that affects the heart and upper limbs. Phaeochromocytoma, a catecholamine-secreting tumour, is a rare neuroendocrine disorder. We present an interesting case presentation of these two rare disorders in a patient who was asymptomatic for phaeochromocytoma. CASE SUMMARY: A 28-year-old woman who was diagnosed at birth with HOS, presented to the hospital with heart failure. She has a past medical history of corrected cyanotic congenital heart disease. She presented with dyspnoea but she did not have headaches, tremors, or diaphoresis. Cardiac magnetic resonance scan was done to investigate the cause of her heart failure and revealed right ventricular systolic dysfunction and a suspicious adrenal lesion. Magnetic resonance imaging adrenal confirmed the presence of the adrenal lesion and concerns were raised for a possible phaeochromocytoma. Biochemical tests showed raised plasma free metanephrine levels. Gallium-68 DOTA positron emission tomography scan showed intense right adrenal gland uptake in keeping with diagnosis of phaeochromocytoma. DISCUSSION: Phaeochromocytoma appears to be more prevalent in patients who are in a chronic hypoxic state. This hypoxic state has been postulated to cause the proliferation of adrenal tissue and therefore the formation of phaeochromocytomas. The hypoxia-inducing factor, which is increased in patients with phaeochromocytoma, has been identified as one of the key factors driving this process as it modulates genes that regulate angiogenesis and proliferation. Congenital heart defects seen in HOS can progress to cyanotic heart disease if left uncorrected and may have been the driver for the development of phaeochromocytoma in our patient.

Predictors of therapy failure in a series of 741 adult pyogenic liver abscesses.

BACKGROUND: Adult pyogenic liver abscess (PLA) is a major hepato-biliary infection. We aim to identify risk factors associated with therapy failure. METHODS: Retrospective study of 741 PLA patients (2001-2011) and comparison with earlier data (1994-1997). Risk factors associated with therapy failure were identified with multivariate analysis. RESULTS: Incidence of PLA is 86/100 000 admissions, with average size 5.75 cm. 68% of PLA were secondary to Klebsiella pneumoniae and there is increasing extended-spectrum beta-lactamase (ESBL) resistance. Compared with 1990s, there is an increasing annual incidence (from 18 to 67). Elderly age (≥55-years-old), presence of multiple abscesses, malignancy as etiology and patients who underwent endoscopic intervention are independent predictors for failure of antibiotics-only therapy while average intravenous antibiotics duration and average abscess size are not. ECOG performance status ≥2, pre-existing hypertension and hyperbilirubinaemia are independent predictors for failure of percutaneous therapy while the presence of multiple abscesses and average abscess size are not. CONCLUSION: There is an increasing PLA incidence with increasing ESBL resistance. Percutaneous drainage should be considered early for elderly patients (≥55-years-old), with multiple abscesses, malignancy as etiology or who required endoscopic intervention. We should have a low threshold for surgical intervention for patients with ECOG performance status ≥2, co-morbidity of hypertension or hyperbilirubinaemia.