Progressive Heart Failure Symptoms Research Articles

Influence of myocardial contractility therapy for advanced heart failure

With more than 5 million patients’ diagnosed with heart failure (HF) and more than 1 million hospitalizations each year, HF continues to have a significant impact on health-care resources. Despite treatment, a significant number of patients continue to have progressive HF symptoms, classified as Class III or IV. The aim of this study was to compare different inotropic therapy for patients of End-Stage Heart Failure. When persistent intravenous access was used and acute hemodynamic improvement was established, the durable medical equipment (DME) benefit category reimbursed 80% of outpatient inotropic medication and supplies for HF. Acute hemodynamic improvement was defined as a 20% reduction in pulmonary capillary wedge pressure and/or a 20% increase in cardiac index, both of which were related to a decrease in dyspnea. The Centers for Medicaid and Medicare Services contracted with regional carriers to run the program. The amounts reimbursed per beneficiary for inotrope and supplies (not adjusted for differential follow-up) were essentially representative of the inotrope cost. Dobutamine, milrinone, and supplies had mean (and median) amounts of 5025 (1168), 87781 (31440), and 7284 (3131), respectively. We found that patients who received this medication had a relatively high death rate, however not as high as people who received chemotherapy. Milrinone-treated patients were compensated more for hospitalizations both before and after starting the inotrope. In addition, the milrinone group had a higher level of digoxin use in the background. Nonetheless, there is an early decrease in overall expenditures following inotrope initiation, which can be attributed to a decrease in hospitalization. Considering the lack of double-blind trials comparing inotropes to placebo or dobutamine to milrinone, decisions regarding the use of inotropes, the type of inotrope used, and the duration of treatment should take into account the impact on resources.

Assessing the impact of atrial fibrillation on symptoms and quality of life in hypertrophic cardiomyopathy.

In hypertrophic cardiomyopathy (HCM), atrial fibrillation (AF) has historically been regarded to have a deleterious impact on clinical course, strongly associated with progressive heart failure (HF) symptoms. However, there is a paucity of information regarding the impact of AF on HCM employing validated quality of life (QoL) surveys. Therefore, we evaluated the impact of AF on QoL utilizing patient reported outcome measures (PROMs). 218 consecutive HCM patients with or without AF at the Lahey HCM center in 2022 completed PROMs at their most recent visit evaluating HF (Kansas City Cardiomyopathy Questionnaire [KCCQ]) and AF symptoms (AF Effect on QoL [AFEQT]). Among the 218 patients, 50 (23%) had a history of AF and comprise the primary study cohort. AF was diagnosed at 55 ± 10 years of age, median of 5.5 years before PROM, with 66% of patients treated with a rhythm control strategy with antiarrhythmic drug and/or AF ablation. AFEQT indicated that 52% of patients experienced no or minimal AF-related disability, mild to moderate in 22%, and severe in 26%. There was no substantial difference in HCM phenotype in patients with no or minimal AF disability compared to those with severe disability. HF symptoms for most HCM patients with prior AF history was consistent with no or minimal (59%) or only mild (27%) disability as measured by KCCQ overall summary scores. In addition, with multivariate analysis, AF history was associated with less HF symptoms and improved QoL (OR 0.4, p = 0.02). In contrast to prior perceptions, HCM patients with prior AF history were less likely to incur HF symptoms impairing QoL compared to HCM patients without AF. After treatment, prior history of AF did not substantially impact current QoL. These data provide a realistic appraisal for the impact that AF has on HCM patients and also offers a measure of reassurance for this patient subgroup.

A clinical case of hereditary transthyretin amyloidosis. Case report

For several years, there has been a significant increase in the number of patients diagnosed with cardiac amyloidosis. However, there are still difficulties associated with the diagnosis, identifying its type and further treatment of the disease. The diversity of the clinical symptoms, the absence of pathognomonic symptoms make it difficult to diagnose, which leads to the progression of the disease. Heart involvement is the main cause of morbidity and mortality in systemic amyloidosis, regardless of the underlying pathogenesis of amyloid production, and in most cases amyloid cardiomyopathy is clinically manifested by rapidly progressive symptoms of heart failure. The article presents a clinical case of a patient with a hereditary type of systemic transthyretin amyloidosis with the involvement of heart, kidneys, liver, nervous system, and the identified mutation in the TTR gene (p.Y89F) has not been previously described in the Russian Federation.

Feasibility and role of echocontrast evaluation of patients with LVAD

Abstract Background Advanced heart failure is a clinical syndrome characterized by persistent or progressive symptoms of heart failure despite optimal medical therapy. Left ventricular assist device (LVAD) brings survival benefits and improvement in quality of life, compared with conventional medical treatments. Development of Right ventricle failure in patients with LVAD has a direct effect on mortality and hospitalization. Purpose Evaluation of clinical safety and feasibility of echocontrast in patients implanted with 3 different types of LVAD; improvement in the visualization of heart structures; intra and inter-operator agreement of RV assesement with and without contrast. Methods 43 patients were implanted with LVAD, 7 patients (16%) with Jarvik 2000, 31 (72%) with HeartMAte 3, 5 (12%) with (HeartWAre HVAD). Nine patients (21%) had contraindication or refused contrast. In 3 (7%) patients was technically challenging to obtain apical images at all levels. Two (5%) patients lost their follow-up. Our final population was of 29 (67%) patients (mean age 65±7 y; 100% Male). We assessed the reproducibility of these measurements between two different expert blind operators. Results Total 329 (64%) of 516 RV wall segments were available for qualitative analysis without contrast vs 451 (87%) with contrast (p<0.001) with a significant improvement of the evaluability of regional contractility (especially due to the better evaluation of medial and apical segments of lateral and anterior walls) and FAC (41% vs 90%, p<0.001). Evaluation of TAPSE, TR and sPAP was similar with and without contrast (p=NS). All the RV parameters showed little inter-operator variability when measured with contrast. TAPSE, FAC, and RWMA showed an excellent reproducibility (ICC >0.86) while it was good for 2D-baseline derived parameters (ICC = 0.74) showing improvement of inter operator reproducibility in the evaluation of regional contractility in the contrast echocardiography modality. Conclusion EC is safe with all the types of LVAD examined. Accurate and reproducible visualization of RV is imperative for reliability of information, a routine use of EC could play a pivotal role in interpreting RV features. EC improves RV morphologic and functional judgment allowing greater accuracy and precision in the assessment of both global and regional RV functions. This finding may have important clinical improvement, especially in the future for analysis focused in RV prognostic role in LVAD patients. Funding Acknowledgement Type of funding sources: None.

18F-FDG PET/CT features and management of primary cardiac lymphoma: a case report

An 81-year-old man presented with progressive symptoms of heart failure. Echocardiography showed a mass in the right atrium (approximately 31 × 55 mm) that extended to the tricuspid valves as well as the superior vena cava. Positron emission tomography-computed tomography (PET-CT) showed elevated 18F-fluorodeoxyglucose (18F-FDG) uptake throughout the space-occupying lesions. The patient received surgery based on a preliminary diagnosis of myxoma. Pathological examination of the resected specimen revealed large B-cell lymphoma. The patient received three cycles of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone regimen but no subsequent anti-tumor therapy. At the last follow-up 3.5 years later, he was still alive. In summary, primary cardiac lymphoma should be considered in differential diagnosis in isolated space-occupying lesions in the heart.

Right Heart Failure as an Atypical Presentation of Chronic Type a Aortic Dissection - Multimodality Imaging for Accurate Diagnosis and Treatment. A Case Report and Mini-review of Literature.

BackgroundAn intrapericardial organized haematoma secondary to chronic type A aortic dissection is an extremely rare cause of right heart failure. Imaging studies are essential in recognising and diagnosis of this distinctive medical condition and guiding the anticipated treatment.Case presentationA 70-year-old male patient was admitted for progressive symptoms of right heart failure. His cardiovascular history exposed an aortic valve replacement 22 years before with a Medtronic Hall 23 tilting valve with no regular follow-up. Classical signs of congestion were recognized at physical examination. Transthoracic two-dimensional echocardiography and thoraco-abdominal computed tomography angiography, as essential parts of multimodality imaging algorithm, established the underlying cause of right heart failure. Under total cardiopulmonary bypass and cardiac arrest, surgical removal of the haematoma and proximal repair of the ascending aorta with a patient-matched vascular graft were successfully performed. The patient was discharged in good condition with appropriate pharmacological treatment, guideline-directed; no imagistic signs of acute post-surgery complications were ascertained.ConclusionThis paper highlights the importance of recognizing and providing a timely clinical and imagistic diagnosis of this very rare, potentially avoidable cause of right heart failure in patients with previous cardiac surgery.

P104 FEASIBILITY AND ROLE OF ECHOCONTRAST EVALUATION IN PATIENTS WITH LVAD

Abstract Background Advanced heart failure is a clinical syndrome characterized by persistent or progressive symptoms of heart failure despite optimal medical therapy. Left ventricular assist device (LVAD) brings survival benefits and improvement in quality of life, compared with conventional medical treatments. Development of Right ventricle failure in patients with LVAD has a direct effect on mortality and hospitalization. Purpose evaluation of clinical safety and feasibility of echocontrast in patients implanted with 3 different types of LVAD; improvement in the visualization of heart structures; intra and inter–operator agreement of RV assesement with and without contrast. Methods 43 patients were implanted with LVAD, 7 patients (16%) with Jarvik 2000, 31 (72%) with HeartMAte 3, 5 (12%) with (HeartWAre HVAD). Nine patients (21%) had contraindication or refused contrast. In 3 (7%) patients was technically challenging to obtain apical images at all levels. Two (5%) patients lost their follow–up. Our final population was of 29 (67%) patients (mean age 65±7 y; 100% Male). We assessed the reproducibility of these measurements between two different expert blind operators Results Total 329 (64%) of 516 RV wall segments were available for qualitative analysis without contrast vs 451 (87%) with contrast (p < 0.001) with a significant improvement of the evaluability of regional contractility (especially due to the better evaluation of medial and apical segments of lateral and anterior walls) and FAC (41% vs 90%, p < 0.001). Evaluation of TAPSE, TR and sPAP was similar with and without contrast (p=NS). All the RV parameters showed little inter–operator variability when measured with contrast. TAPSE, FAC, and RWMA showed an excellent reproducibility (ICC >0.86) while it was good for 2D–baseline derived parameters (ICC = 0.74) showing improvement of inter operator reproducibility in the evaluation of regional contractility in the contrast echocardiography modality. Conclusion EC is safe with all the types of LVAD examined. Accurate and reproducible visualization of RV is imperative for reliability of information, a routine use of EC could play a pivotal role in interpreting RV features. EC improves RV morphologic and functional judgment allowing greater accuracy and precision in the assessment of both global and regional RV functions. This finding may have important clinical improvement, especially in the future for analysis focused in RV prognostic role in LVAD patients.

Transcutaneous Closure of Aortic Valve Cusp Perforation

We report the case of a 58-year-old patient with history of mechanical mitral valve replacement 1 year ago due to Staphylococcus aureus endocarditis that resulted in severe mitral regurgitation. During follow-up, he had progressive heart failure symptoms, New York Heart Association class III, with severe hemolytic anemia. Transthoracic echocardiography revealed mildly dilated left ventricle and dilated left atrium with evidence of severe mitral paravalvular leak (PVL) and severe aortic regurgitation (AR).

One Heart, Two Problems: Cardiomyopathy and CPVT Secondary to RYR2 Mutation

<h3>Introduction</h3> RYR2 mutations are known to cause catecholaminergic polymorphic ventricular tachycardia (CPVT), an inherited disorder with risk for life threatening atrial (AT) and ventricular (VT) arrhythmias. Individuals with RYR2 mutation less commonly exhibit arrhythmogenic right ventricular cardiomyopathy (ARVC). We report the first case of a pediatric patient with RYR2 mutation, manifesting with CPVT, ARVC, and severe dysfunction requiring heart transplant (HTx). <h3>Case Report</h3> A 4.5 year old male with developmental delay presented after multiple syncopal episodes both at rest and with activity. A Holter showed AT, and he was started on a beta adrenergic antagonist with decreased frequency of syncopal episodes. He then developed sinus bradycardia with marked sinus pauses, prompting dual chamber pacemaker placement at age 6 years. He later exhibited bidirectional VT and whole exome sequencing identified a de novo, likely pathogenic variant in RYR2. He was medically managed until 16 years of age when he had syncope with exercise and was found to have both frequent AT and moderately depressed ventricular function with an ejection fraction of 45%. Given recurrent syncope with exercise, he underwent uncomplicated sympathectomy and optimization of his medical antiarrhythmic management. Over the next few months, he developed progressive heart failure symptoms and was admitted with worsening biventricular dysfunction and tachyarrhythmias. During his admission, he had a cardiac arrest with initial monitoring demonstrating VT, and was placed on extracorporeal membrane oxygenation support. He was transitioned to a durable left ventricular assist device (VAD), and was eventually listed for HTx. His VAD course was complicated by RV dysfunction needing PDE5 inhibitor; his arrhythmia burden was managed on flecainide, sotalol, and nadolol. After 80 days of VAD support, he underwent HTx. Pathology of his explanted heart revealed biventricular fibrofatty myocardial replacement consistent with ARVC with prominent LV involvement. <h3>Summary</h3> This case highlights an expanded spectrum of RYR2 mutation related disease which may include prominent atrial arrhythmias, ARVC, and developmental delay. Patients with CPVT and RYR2 mutation warrant close monitoring for ventricular dysfunction, especially if there is a significant arrhythmia burden.

Hypertrophic Cardiomyopathy: New Concepts and Therapies.

Hypertrophic cardiomyopathy (HCM), a relatively common, globally distributed, and often inherited myocardial disorder, transformed over the last several years into a treatable condition with the emergence of effective management options that alter natural history at all ages. Now available are a matured risk stratification algorithm selecting patients for prophylactic implantable defibrillators that prevent arrhythmic sudden death; low-risk, high-benefit surgical myectomy to reverse progressive heart failure symptoms due to left ventricular outflow obstruction; anticoagulation prophylaxis to prevent atrial fibrillation-mediated embolic stroke; and heart transplant for refractory end-stage disease in the absence of obstruction. Those strategies have resulted in reduction of HCM-related morbidity and reduction of mortality to 0.5% per year.

Healthcare resource use associated with the diagnosis of transthyretin amyloidosis cardiomyopathy.

ObjectivesOur primary aim was to evaluate the healthcare resource use associated with the diagnosis of transthyretin amyloidosis cardiomyopathy. Second, we aim to assess the effect of the number of diagnostic tests and clinical contact points on the total time and costs between symptom onset and diagnosis defining a quantitative hypothetical optimized diagnostic pathway.SettingClinical and cost data were collected from patients presenting between 2010 and 2018 in a tertiary referral institution in South London involving two participating hospitals.ParticipantsThirty‐eight adult patients with a definite diagnosis of transthyretin amyloidosis cardiomyopathy were included, mostly male (n = 28, 74%) and of African‐Caribbean descent (n = 23, 64%). We excluded patients without a confirmed transthyretin amyloidosis cardiomyopathy or those on inotersen, patisiran, or diflunisal at point of referral.Primary and secondary outcome measuresThe average time between first presentation and final diagnosis, and the cost per patient per month. By comparing to a more optimal clinical pathway towards diagnosis, we considered what could be the theoretical gain in terms of time to diagnosis and financial savings.ResultsThe average time between first presentation and final diagnosis was 2.74 years. The average cost per patient per month was higher with progressive heart failure symptoms. A hypothetical optimal pathway reduces time to diagnosis of 1.65 to 1.74 years per patient. The potential financial savings are estimated within the range of £3000 to £4800 per patient.ConclusionsPatients diagnosed with transthyretin amyloidosis cardiomyopathy have substantial healthcare resource utilization and costs starting from symptom onset. Higher costs were observed with progression in symptoms and appear linked to a delayed diagnosis. The number of additional diagnostic tests and clinical contact points may contribute to this and could represent a path to explore further for important health and cost savings, with more efficient pathways for these patients to be managed.

Usefulness of Global Longitudinal Strain to Predict Heart Failure Progression in Patients With Nonobstructive Hypertrophic Cardiomyopathy

While predicting prognosis to anticipate adverse disease course has long been an aspiration in hypertrophic cardiomyopathy (HC), reliable markers of progressive and unrelenting heart failure symptoms in the absence of obstruction are not well characterized. We sought to evaluate markers of systolic function, including the role of global longitudinal strain (GLS), to identify nonobstructive HC patients at risk for future heart failure. A cohort of 296 consecutive nonobstructive HC patients (42 ± 18years; 75% male) with NYHA class I/II symptoms and preserved systolic function at study entry (EF: 65 ± 6%), were followed for progressive heart failure symptoms (increase in ≥ 1 NYHA functional class) and/or development of systolic dysfunction (EF < 50%). Over median follow-up of 4 ± 3 years, 35 study patients (10%) experienced new heart failure events, including 31 with progressive symptoms and 4 who developed systolic dysfunction. Abnormal GLS < 16% was associated with a 5-fold increase in risk for heart failure compared to GLS > 18% (p < 0.001). GLS remained an independent predictor of heart failure even after adjustment for other relevant disease variables including EF (OR 1.23, p = 0.005). However, notably, when GLS and EF were combined, the prediction of heart failure for individual patients was enhanced (net reclassification improvement 0.55; p = 0.002). Together, GLS < 16% and EF 50% to 59% were associated with a 12.5-fold greater risk for heart failure versus patients with GLS > 18% and EF ≥ 60%, who were at the lowest risk. In conclusion, in nonobstructive HC with no or mild symptoms and preserved EF, abnormal GLS is a strong independent predictor for subsequent development of progressive heart failure symptoms and/or systolic dysfunction. Furthermore, the greatest power in predicting outcome in nonobstructive HC is achieved by combining GLS with EF to identify HC patients at the highest risk for heart failure progression and systolic dysfunction.

Carcinoid heart disease of gonadal primary presenting with hypoxia: a case report.

BackgroundCarcinoid heart disease is a potential sequela of metastatic neuroendocrine tumour that has characteristic valve appearances. Patients can present with symptoms of carcinoid syndrome or be relatively asymptomatic until symptoms of progressive heart failure manifest.Case summaryWe present a case of a 54-year-old male who was admitted to the hospital for investigation of hypoxia. Transthoracic echocardiogram was suggestive of carcinoid heart disease which subsequently led to a diagnosis of metastatic neuroendocrine (carcinoid) tumour of the testicular primary. Work-up revealed a patent foramen ovale with evidence of the right to left interatrial shunt from severe tricuspid regurgitation as the cause of his hypoxia. Prior to surgical excision of the primary tumour, percutaneous patent foramen ovale closure was performed resulting in improved arterial oxygen saturation and symptomatic improvement.DiscussionCarcinoid heart disease typically affects the right-sided cardiac valves and the tricuspid valve appearances were critical in leading to a diagnosis of a metastatic neuroendocrine tumour in our patient. This case demonstrates that percutaneous patent foramen ovale closure can be an effective intervention for hypoxia in those not managed surgically. A high index of suspicion should be maintained for gonadal primary carcinoid tumour when there is carcinoid heart disease in the absence of liver metastases.

ANMCO/SIC Consensus document on the management of myocarditis

Myocarditis is an inflammatory heart disease that can occur acutely, as in acute myocarditis, or persistently, as in chronic myocarditis or chronic inflammatory cardiomyopathy. Different agents can induce myocarditis, with viruses being the most common triggers. Generally, acute myocarditis affects relatively young people and men more than women. Myocarditis has a broad spectrum of clinical presentations and evolution trajectories, although most cases resolve spontaneously. Patients with reduced left ventricular ejection fraction, heart failure symptoms, advanced atrioventricular block, sustained ventricular arrhythmias or cardiogenic shock (the latter known as fulminant myocarditis) are at increased risk for death and heart transplantation. The presentation of chronic inflammatory cardiomyopathy may be more subtle, with progressive symptoms of heart failure or appearance of rhythm disturbance, not rarely preceded by an infective episode. Autoimmune disorder or systemic inflammatory conditions can be another significant predisposing substrate of myocarditis, especially in women. Emerging causes of myocarditis are drug-related like the new anticancer therapies, the immune checkpoint inhibitors. In this Italian Association of Hospital Cardiologists (ANMCO) and Italian Society of Cardiology (SIC) expert consensus document on myocarditis, we propose diagnostic strategies for identifying possible causes of the disease and factors associated with increased risk. Finally, we propose potential treatments and when referring patients to tertiary centers, especially for high-risk patients. Even if endomyocardial biopsy is the invasive diagnostic tool for making definitive diagnosis and differentiation of histological subtypes (i.e., lymphocytic vs eosinophilic vs giant cell myocarditis), it is not always readily available in all centers. Thus, we propose when this exam is mandatory or when it can be postponed or substituted by cardiac magnetic resonance imaging. This document reflects the Italian perspective on managing patients with myocarditis and their follow-up, considering also current US and European scientific position statements.

Abstract 17341: Obesity is Associated With Progressive Heart Failure in Hypertrophic Cardiomyopathy

Background: The impact of comorbid disease states and lifestyle on the natural history of patients with hypertrophic cardiomyopathy (HCM) remains unknown. Objective: Evaluate the association of non-HCM comorbidities including obesity, hypertension, diabetes, obstructive sleep apnea, kidney disease, tobacco use, alcohol use, and lung disease with disease progression in a large cohort of HCM patients. Methods: 2269 patients evaluated at the Tufts HCM Institute between 2004 to 2019, ≥ 18 years of age (54 ± 15 years; 1392 male), and followed for an average of 4 ± 3.4 years for disease progression including progressive heart failure (HF) symptoms (from NYHA class I/II to NYHA class III/IV), new-onset atrial fibrillation (AF), or sudden death (SD) event (including appropriate defibrillation for ventricular arrhythmias, resuscitated cardiac arrest, or SD). Results: Of 1376 patients with NYHA class I/II symptoms at initial clinical evaluation, 252 (18%) developed progressive HF symptoms to NYHA class III/IV over follow-up (5%/year). Obesity (BMI ≥ 30) was significantly more prevalent in patients who had progressive HF during follow-up (43%) compared to those who remained without HF (34%, p = 0.014). In contrast, other comorbidities were not significantly associated with progressive HF symptoms (p &gt; 0.10 for all other comorbidities). Of the 1823 patients without AF history at initial clinical visit, 198 (11%) developed new-onset AF over follow-up (3%/year). No comorbidities were significantly associated with new-onset AF in HCM (p &gt; 0.10), although obesity was more common in patients who developed new-onset AF (48%) compared to those who had no AF (41%, p = 0.08). Notably, SD events were not associated with non-HCM comorbidities (p &gt; 0.10 for all comorbidities), and patients with SD events were less likely to have comorbidities than patients without SD events. Conclusions: In adult HCM patients, obesity is associated with progressive symptoms and outcomes supporting weight loss as an important modifier in obese HCM patients to potentially help prevent HCM complications. In contrast, other non-HCM comorbidities do not appear to impact disease course, and SD events are not associated with comorbidities in HCM.

Catecholaminergic polymorphic ventricular tachycardia complicated by dilated cardiomyopathy: a case report.

Background Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a severe genetic arrhythmogenic disorder characterized by adrenergically induced ventricular tachycardia manifesting as stress-induced syncope and sudden cardiac death. While CPVT is not associated with dilated cardiomyopathy (DCM) in most cases, the combination of both disease entities poses a major diagnostic and therapeutic challenge.Case summary We present the case of a young woman with CPVT. The clinical course since childhood was characterized by repetitive episodes of exercise-induced ventricular arrhythmias and a brady-tachy syndrome due to rapid paroxysmal atrial fibrillation and sinus bradycardia. Medical treatment included propranolol and flecainide until echocardiography showed a dilated left ventricle with severely depressed ejection fraction when the patient was 32 years old. Cardiac magnetic resonance imaging revealed non-specific late gadolinium enhancement. Myocardial inflammation, however, was excluded by subsequent endomyocardial biopsy. Genetic analysis confirmed a mutation in the cardiac ryanodine receptor but no pathogenetic variant associated with DCM. Guideline-directed medical therapy for HFrEF was limited due to symptomatic hypotension. Over the next months, the patient developed progressive heart failure symptoms that were finally managed by heart transplantation.Discussion Management in patients with CPVT and DCM is challenging, as Class I antiarrhythmic drugs are not recommended in structural heart disease and prophylactic internal cardioverter-defibrillator implantation without adjuvant antiarrhythmic therapy can be detrimental. Regular echocardiographic screening for DCM is recommendable in patients with CPVT. A multidisciplinary team of heart failure specialists, electrophysiologists, geneticists, and imaging specialists is needed to collaborate in the delivery of clinical care.

Heart Transplantation - Part I: General Considerations

Heart failure (HF) is a major public health problem with significant associated morbidity and mortality. In 2001, the American College of Cardiology/American Heart Association (ACC/AHA) guideline committee proposed a new approach to the classification of HF that emphasized both the development and progression of disease. Stage A and B patients are at high risk for developing HF, and include those without structural heart disease (Stage A) and those with structural heart disease, but without signs or symptoms of HF (Stage B). Stage C and D patients have structural heart disease with prior or current symptoms of HF (Stage C) or refractory HF requiring specialized interventions (Stage D). Rregistries suggest that between 5% and 10% of patients with HF have advanced disease, which is associated with 1-year mortality in excess of 50% and a poor quality of life. The Heart Failure Society of America (HFSA) defines Stage D heart failure as “the presence of progressive and/or persistent severe signs and symptoms of heart failure despite optimized medical, surgical, and device therapy…generally accompanied by frequent hospitalization, severely limited exertional tolerance, and poor quality of life.” In this two-part chapter, we focus on heart transplantation, which remains the standard-of-care for highly selected patients with end-stage HF and absence of contraindications to transplant.1-5 This review contains 7 figures, 8 tables, and 46 references. Key words: heart failure, cardiomyopathy, heart transplant, mechanical circulatory support, prognosis, pulmonary hypertension, diabetes, HLA sensitization, donor

Heart Transplantation - Part I: General Considerations

Heart failure (HF) is a major public health problem with significant associated morbidity and mortality. In 2001, the American College of Cardiology/American Heart Association (ACC/AHA) guideline committee proposed a new approach to the classification of HF that emphasized both the development and progression of disease. Stage A and B patients are at high risk for developing HF, and include those without structural heart disease (Stage A) and those with structural heart disease, but without signs or symptoms of HF (Stage B). Stage C and D patients have structural heart disease with prior or current symptoms of HF (Stage C) or refractory HF requiring specialized interventions (Stage D). Rregistries suggest that between 5% and 10% of patients with HF have advanced disease, which is associated with 1-year mortality in excess of 50% and a poor quality of life. The Heart Failure Society of America (HFSA) defines Stage D heart failure as “the presence of progressive and/or persistent severe signs and symptoms of heart failure despite optimized medical, surgical, and device therapy…generally accompanied by frequent hospitalization, severely limited exertional tolerance, and poor quality of life.” In this two-part chapter, we focus on heart transplantation, which remains the standard-of-care for highly selected patients with end-stage HF and absence of contraindications to transplant.1-5 This review contains 7 figures, 8 tables, and 46 references. Key words: heart failure, cardiomyopathy, heart transplant, mechanical circulatory support, prognosis, pulmonary hypertension, diabetes, HLA sensitization, donor

Hypertrophic Cardiomyopathy in “Real-World” Community Cardiology Practice

Differences in presentation and natural history of hypertrophic cardiomyopathy (HC) between community cardiology practice and referral centers has been a source of considerable uncertainty. We report here a cross-sectional analysis of 253 consecutive HC patients from a "real-world" clinical cardiology setting. When compared with a highly selected referral center cohort, patients in clinical practice proved to be similar with regard to disease expression such as left ventricular (LV) wall thickness, outflow obstruction, and natural history, including stable and largely benign clinical course with no or mild symptoms (61% in community practice vs. 55% in referred patients, p = 0.23), occurrence of atrial fibrillation (22% vs. 24%, p = 0.75) and nonfatal sudden death (SD) events (3% vs. 4%, p = 0.8). In contrast, progressive heart failure symptoms were most common in the referral cohort (36% vs. 26%, p = 0.04). In clinical practice, SD was prevented by prophylactic implatable cardioverter defibrillators (ICD) in 5 of 44 patients (11%), although risk was overestimated in 6 patients who were implanted with ICDs in the absence of risk markers (14%). In 16 of 61 (26%) severely symptomatic drug-refractory patients with LV outflow obstruction, recommendation for surgical myectomy (or alcohol septal ablation) was delayed. In conclusion, clinical characteristics and course of HC patients in community practice were generally similar to those in HC referral centers. Community cardiologists managed HC patients predominantly in concert with guideline-based strategies, although risk for SD could be overestimated, and the significance of outflow obstruction with timely reversal of refractory heart failure by intervention was underappreciated.

Care of the Post-CABG Patient.

An estimated 400,000 coronary artery bypass graft operations are performed annually in the United States. Saphenous vein grafts are the most commonly used conduits; however, graft failure is common. In contrast, left internal mammary artery grafts have more favorable long-term patency rates. Guidelines recommend aggressive secondary prevention. In the 2 decades following surgery, 16% of patients require repeat revascularization, and percutaneous coronary intervention accounts for 98% of procedures performed. Post-coronary artery bypass graft patients presenting with symptoms of acute coronary syndrome or progressive heart failure should undergo early coronary angiography given the high likelihood that such a presentation represents graft failure. Percutaneous coronary intervention in degenerated saphenous vein grafts is associated with embolization that may cause the "no-reflow phenomenon," which can be avoided with the use of embolic protection devices. Hybrid revascularization procedures are a promising emerging strategy to avoid the placement of vein grafts.