Kiran J. Philip

Left ventricular noncompaction cardiomyopathy: updated review

The first case of noncompaction was described in 1932 after an autopsy performed on a newborn infant with aortic atresia/coronary–ventricular fistula. Isolated noncompaction cardiomyopathy was first described in 1984. A review on selected/relevant medical literature was conducted using Pubmed from 1984 to 2013 and the pathogenesis, clinical features, and management are discussed. Left ventricular noncompaction (LVNC) is a relatively rare congenital condition that results from arrest of the normal compaction process of the myocardium during fetal development. LVNC shows variability in its genetic pattern, pathophysiologic findings, and clinical presentations. The genetic heterogeneity, phenotypical overlap, and variety in clinical presentation raised the suspicion that LVNC might just be a morphological variant of other cardiomyopathies, but the American Heart Association classifies LVNC as a primary genetic cardiomyopathy. The familiar type is common and follows a X-linked, autosomal-dominant, or mitochondrial-inheritance pattern (in children). LVNC can occur in isolation or coexist with other cardiac and/or systemic anomalies. The clinical presentations are variable ranging from asymptomatic patients to patients who develop ventricular arrhythmias, thromboembolism, heart failure, and sudden cardiac death. Increased awareness over the last 25 years and improvements in technology have increased the identification of this illness and improved the clinical outcome and prognosis. LVNC is commonly diagnosed by echocardiography. Other useful diagnostic techniques for LVNC include cardiac magnetic resonance imaging, computerized tomography, and left ventriculography. Management is symptom based and patients with symptoms have a poorer prognosis. LVNC is a genetically heterogeneous disorder which can be associated with other anomalies. Making the correct diagnosis is important because of the possible associations and the need for long-term management and screening of living relatives.

The Effects of Spirituality and Religion on Outcomes in Patients with Chronic Heart Failure

Heart failure (HF) is a chronic progressive disease with marked morbidity and mortality. Patients enduring this condition suffer from fluctuations in symptom burden such as fatigue, shortness of breath, chest pain, sexual dysfunction, dramatic changes in body image and depression. As physicians, we often ask patients to trust in our ability to ameliorate their symptoms, but oftentimes we do not hold all of the answers, and our best efforts are only modestly effective. The suffering endured by these individuals and their families may even call into question one’s faith in a higher power and portends to significant spiritual struggle. In the face of incurable and chronic physical conditions, it seems logical that patients would seek alternative or ancillary methods, notably spiritual ones, to improve their ability to deal with their condition. Although difficult to study, spirituality has been evaluated and deemed to have a beneficial effect on multiple measures including global quality of life, depression and medical compliance in the treatment of patients with HF. The model of HF treatment incorporates a multidisciplinary approach. This should involve coordination between primary care, cardiology, palliative care, nursing, patients and, importantly, individuals providing psychosocial as well as spiritual support. This review intends to outline the current understanding and necessity of spirituality’s influence on those suffering from HF.

The emergence of oral tadalafil as a once-daily treatment for pulmonary arterial hypertension.

Pulmonary hypertension (PH) is found in a vast array of diseases, with a minority representing pulmonary arterial hypertension (PAH). Idiopathic PAH or PAH in association with other disorders has been associated with poor survival, poor exercise tolerance, progressive symptoms of dyspnea, and decreased quality of life. Left untreated, patients with PAH typically have a progressive decline in function with high morbidity ultimately leading to death. Advances in medical therapy for PAH over the past decade have made significant inroads into improved function, quality of life, and even survival in this patient population. Three classes of pulmonary artery-specific vasodilators are currently available in the United States. They include prostanoids, endothelin receptor antagonists, and phosphodiesterase type 5 (PDE5) inhibitors. In May 2009, the FDA approved tadalafil, the first once-daily PDE5 inhibitor for PAH. This review will outline the currently available data on tadalafil and its effects in patients with PAH.

Persistent Sexual Dysfunction Impairs Quality of Life after Cardiac Transplantation

INTRODUCTION The impact of sexual dysfunction (SD) on mental and physical health after heart transplantation (HTx) has not been established. AIM We investigated the relationship of SD on quality of life (QoL), physical and mental health, and depressive symptoms after HTx. MAIN OUTCOME MEASURES We evaluated SD according to the International Index of Erectile Dysfunction and the Female Sexual Function Index. QoL, physical and mental health were assessed using: 1) Short Form 12 Health Survey Questionnaire, 2) Quality of Life Enjoyment and Satisfaction Questionnaire--Short Form, and two depressive symptoms questionnaires: 1) Beck Depression Inventory-II and 2) Quick Inventory Depressive Symptomatology-Self Report. METHODS We enrolled patients who were greater than 6 months post HTx. Patients unable to read English, had pelvic surgery or trauma, urogenital abnormalities, or sexually inactive were excluded. RESULTS Out of 79 subjects that were screened, 33 men and 6 women participated (mean age 61.4 + 1.4). Response rates were at least 82% for all questionnaires. Overall prevalence of SD was 61%, with 78% of men being affected and 50% of women. There was no significant difference in measures between genders. HTx recipients with SD reported significantly worse QoL on measures of physical health when compared to those without SD. After HTx, patients suffering from SD had significantly worse general health (P = 0.02) and physical health (P = 0.02), including physical functioning (P = 0.01) and physical role limitation (P = 0.01). In contrast, mental health and depressive symptoms after HTx were not significantly different between those with and without SD. CONCLUSIONS After HTx a high prevalence of SD remains among both men and women. Patients with SD had worse general and physical health but not depressive symptoms when compared to those without SD. The contributing factors may be more related to physical rather than psychological causes.

Review: Testosterone Therapy: Treatment of Metabolic Disturbances in Heart Failure

Heart failure (HF) is a complex progressive multisystem disease state with significant morbidity and mortality, which is not solely defined by pathology of the cardiovascular system but also is influenced by neurohormonal regulatory adjustments, peripheral cytokines, as well as hormonal and musculoskeletal dysfunction. Recent attention to the catabolic state found in patients with chronic heart failure has sparked interest in new potential targets for medical therapy. In particular, as many as 26% to 37% of men affected with HF have been found to be testosterone deficient. The severity of androgen deficiency has been shown to correlate with symptoms, functional class, and prognosis in patients with heart failure. Testosterone supplementation has been an accepted therapy in hypogonadal men with fatigue, muscle wasting, and sexual dysfunction for some time. Patients with severe HF show a similar constellation of symptoms and hypothetically would benefit from androgen replacement. Recent clinical studies have confirmed that functional, biochemical, and cardiopulmonary status in patients with HF have significant improvements when treated with testosterone supplementation. Symptomatic improvements may be obtainable in hypogonadal patients with HF who receive supplemental testosterone. This review seeks to outline the cardiovascular and peripheral effects of testosterone supplementation in patients with chronic HF.

Clinical Applications of Bivalirudin in the Cardiac Catheterization Laboratory

Heparin has been used in the catheterization laboratory to prevent ischemic complications of percutaneous coronary intervention (PCI). Bivalirudin, a direct thrombin inhibitor, is an anticoagulant that has several pharmacologic advantages over heparin, and it has been proposed that bivalirudin is superior to heparin in its ability to prevent bleeding complications of PCI. As such, there have been a variety of large prospective clinical trials comparing bivalirudin and heparin over the past 13 years. The results of these trials have prompted the general acceptance of bivalirudin as a safe alternative to heparin use during PCI, and bivalirudin has been given a class 1 recommendation by the American College of Cardiology/American Heart Association (ACC/AHA) guidelines for a variety of clinical indications. This article will review the data supporting the use of bivalirudin in the cardiac catheterization laboratory and describe several advantages of bivalirudin over traditional heparin use. We also include a discussion of the use of bivalirudin in conjunction with other medications that are frequently used in the catheterization laboratory. We end with an analysis of the economic differences between bivalirudin and heparin and the impact that financial factors may have on the choice of anticoagulant.

Growth hormone and testosterone in heart failure therapy

Importance of the field: Heart failure is a progressive disease affecting millions of people worldwide. The disease carries a significantly high morbidity and mortality risk. There are multiple pharmaceutical options to decrease this risk and prolong survival; however, despite optimization of medical management, several patients still await heart transplant, the only definitive cure for heart failure. To slow the progression of disease preventing need for transplantation, improve clinical symptoms, and improve heart failure outcomes, there is a persistent need to discover new therapeutic strategies. Of interest, low growth hormone and testosterone levels have been associated with a worsening degree of heart failure. Many studies have begun to show a clinical improvement in heart failure symptoms when these levels are corrected with hormonal therapy. These findings, although mixed, are promising and indicate that both testosterone and growth hormone therapy should be considered as adjunctive therapy in advanced heart failure patients. Areas covered in this review: This review discusses the physiology of both of these natural hormones, their therapeutic effects in heart failure and data from the published literature on studies using growth hormone or testosterone in patients with chronic heart failure. An extensive search of PubMed was conducted for topics on heart failure, growth hormone, insulin-like growth factor, testosterone, their physiology and pathophysiology, and trials in which they have been used as therapeutic interventions between 1989 and 2009. What the reader will gain: The reader will gain an understanding of the intricate balance of both of these hormones in the disease state of heart failure. In addition, the trials conducted using these hormones in pharmacotherapy for heart failure are discussed along with proposed theories for interstudy variability. Take home message: Testosterone deficiency and growth hormone resistance are positively associated with a poor state of heart failure. Treatment of deficiency improves outcomes in heart failure; however, there is a significant paucity of data with regard to testosterone and heart failure as well as a significant amount of study variability with growth hormone and heart failure.

Review Article: The New Concept of Interventional Heart Failure Therapy—Part 1: Electrical Therapy, Treatment of CAD, Fluid Removal, and Ventricular Support

Congestive heart failure is a chronic and debilitating disease responsible for high cardiac morbidity and mortality in the world and is associated with more than 290 000 deaths in the United States each year. Recent advances in heart failure therapy target many of the mechanical and structural aspects of heart failure that are not addressed by drug-based therapies; these include abnormalities in electrical conduction, coronary artery or valvular architecture, and in ventricular size and shape. To target these abnormalities, newer therapies have largely been mechanical and device-based in nature and can be collectively described as interventional therapy. Interventional therapy includes the use of interventional medical therapy, electrical-based devices to augment ventricular function, catheterization-based devices for the treatment of underlying coronary artery disease and valvular disease, machines for the removal of excess fluid, mechanical pumps to assist the ventricles, surgical techniques aimed at reshaping the ventricles, the use of tissue therapies such as stem cell transplantation or complete heart transplant, palliative care, and self-care. This article is the first in a 2-part series that will review interventional heart failure therapy and present the most recent data supporting its use.

Maximal care considerations when treating patients with end-stage heart failure: ethical and procedural quandaries in management of the very sick

Deciding who should receive maximal technological treatment options and who should not represents an ethical, moral, psychological and medico-legal challenge for health care providers. Especially in patients with chronic heart failure, the ethical and medico-legal issues associated with providing maximal possible care or withholding the same are coming to the forefront. Procedures, such as cardiac transplantation, have strict criteria for adequate candidacy. These criteria for subsequent listing are based on clinical outcome data but also reflect the reality of organ shortage. Lack of compliance and non-adherence to lifestyle changes represent relative contraindications to heart transplant candidacy. Mechanical circulatory support therapy using ventricular assist devices is becoming a more prominent therapeutic option for patients with end-stage heart failure who are not candidates for transplantation, which also requires strict criteria to enable beneficial outcome for the patient. Physicians need to critically reflect that in many cases, the patient’s best interest might not always mean pursuing maximal technological options available. This article reflects on the multitude of critical issues that health care providers have to face while caring for patients with end-stage heart failure.

Heart failure therapy: beyond the guidelines.

Heart failure is a chronic and debilitating disease responsible for high cardiac morbidity and mortality in the world and is associated with over 290 000 deaths in the United States each year. This article reviews palliative care and self-care, which are critical components of heart failure management that are inadequately defined in the current American College of Cardiology/American Heart Association Guidelines for the Diagnosis and Management of Heart Failure. Palliative care describes a multidisciplinary approach to the treatment of heart failure therapy that addresses both the symptomatic and psychosocial aspects of the disease. Self-care aims to maintain disease stability and prevent clinical decline through a variety of patient-based behavioral and lifestyle modifications.