Carcinoid heart disease is the collective term for all cardiac manifestations in patients who have carcinoid syndrome. Carcinoid heart disease has a multifactorial pathophysiology, and the right side of the heart is usually involved. Symptoms and signs vary depending upon the affected cardiac components; most typical is right-sided heart failure secondary to diseased tricuspid and pulmonary valves. Despite no single ideal diagnostic test, strong suspicion, coupled with serologic and imaging results, usually enables diagnosis. Advances in imaging, such as speckle-tracking echocardiography and cardiac magnetic resonance, have improved the diagnostic yield. Treatment is challenging, warrants a multidisciplinary approach, and can be medical or surgical depending on the cardiac manifestations. Investigators are exploring the therapeutic use of monoclonal antibodies and new somatostatin analogues. In this review, we cover current knowledge about the pathophysiology, diagnosis, and treatment of carcinoid heart disease.
Introduction
Ethanol infusion was an early mode of ablative treatment for cardiac arrhythmias. Its initial descriptions involved coronary intra-arterial delivery, targeting arrhythmogenic substrates in drug-refractory ventricular tachycardia or the atrioventricular node. Largely superseded by radiofrequency ablation (RFA) and other contact-based technologies as a routine ablation strategy, intracoronary arterial ethanol infusion remains as an alternative option in the treatment of ventricular tachycardia when conventional ablation fails. Arrhythmic foci that are deep-seated in the myocardium may not be amenable to catheter ablation from either the endocardium or the epicardium by RFA, but they can be targeted by an ethanol infusion.
Recent findings
Recently, we have explored ethanol injection through cardiac venous systems, in order to avoid the risks of complications and limitations of coronary arterial instrumentation. Vein of Marshall ethanol infusion is being studied as an adjunctive procedure in ablation of atrial fibrillation, and coronary venous ethanol infusion for ventricular tachycardia.
Conclusion
Ethanol ablation remains useful as a bail-out technique for refractory cases to RFA, or as an adjunctive therapy that may improve the efficacy of catheter ablation procedures.
A 58-year-old man presented with symptomatic high-degree atrioventricular block and congestive heart failure. His medical history included a bicuspid aortic valve (AV), aortic stenosis, and 3 bioprosthetic AV replacement operations-the last, 25 years before, to place a homograft. At the current admission, the patient underwent pacemaker implantation to treat the heart block. Then, a structural AV abnormality was detected. A transesophageal echocardiogram (TEE) showed paravalvular leak and a new finding: a 1.5 × 1.7-cm pseudoaneurysm at the base of the aortic root, adjacent to the fibrous trigone. The pseudoaneurysm, connected by a tract to the left ventricular outflow tract (LVOT), bulged into the left atrium and exhibited pulsatile expansion during systole (Fig. 1). A computed tomographic angiogram showed a 1.8 × 1.9-cm focal outpouching, inferior to the takeoff of the left main coronary artery and posterior to the aortic root. A 3-to 4-mm connection was identified at the 6 o'clock position with respect to the LVOT. The patient's heart failure was probably related to the structural defect. To avoid repeat sternotomy, we chose a percutaneous closure approach. Under diagnostic fluoroscopy (Fig. 2) and intraprocedural TEE (Fig. 3), the pulsatile defect was evident.
Background:
There is growing interest in the observed significant incidence of transthyretin cardiac
amyloidosis in elderly patients with aortic stenosis. Approximately 16% of patients with severe aortic
stenosis undergoing aortic valve replacement have transthyretin cardiac amyloidosis. Outcomes after
aortic valve replacement appear worse in patients with concomitant transthyretin cardiac amyloidosis.
Method:
Publications in PubMed, Cochrane Library, and Embase databases were systematically
searched from January 2012 to September 2018 using the keywords transthyretin, amyloidosis, and
aortic stenosis. All studies published in English that reported the prevalence, association and outcomes
of transthyretin cardiac amyloidosis in patients with aortic stenosis undergoing were included.
Results/Conclusion:
The relationship between aortic stenosis and transthyretin cardiac amyloidosis is
not well understood. A few studies have proven successful surgical management when both
conditions coexist. This systematic review suggests that transthyretin cardiac amyloidosis is common
in elderly patients with aortic stenosis and tend to have high mortality rates after AVR. The
significant incidence of the two diseases occurring simultaneously warrants further investigation to
improve management strategies in the future.
Introduction Electrocardiographic changes imitating myocardial ischemia have been occasionally reported in patients with intra-abdominal pathology including acute pancreatitis. Case Report A 60-year-old man with no past medical history presented to the emergency department (ED) after a syncopal episode. In ED, his vitals were stable. His ECG showed sinus bradycardia at 53 beats per minute, peaked T waves, 1 mm ST-segment elevation in leads II, III, and aVF, and 2 mm ST elevation in V3 as shown in the figures. With the concern for STEMI, he was taken for left heart catheterization (LHC) emergently, showing nonobstructive coronary artery disease (CAD). His laboratory workup was remarkable for lipase of 25,304 IU/l (normal level 8–78 IU/l). His liver function test and triglyceride level were normal. Troponin was <0.01 ng/ml. A computed tomographic exam of the abdomen revealed acute interstitial pancreatitis with a small discrete fluid collection in the uncinate process. He was treated with aggressive intravenous fluid resuscitation and was discharged on day 3. Discussion Intra-abdominal pathologies like acute pancreatitis can lead to transient ECG changes mimicking STEMI. It is important to use ECG clues, echocardiographic findings, and clinical judgement to avoid cardiac catheterization, contrast exposure, and associated health care costs.
Apical hypertrophic cardiomyopathy (Yamaguchi syndrome) is a rare subtype of hypertrophic cardiomyopathy. The syndrome is more common in Japan where it was first described. Outside Asia, it is a very rare cause of hypertrophic cardiomyopathy. Apical hypertrophic cardiomyopathy is usually detected incidentally and has a good long-term outcome. We present a case of apical hypertrophic cardiomyopathy in an African-American patient manifesting as atrioventricular nodal re-entrant tachycardia.
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