Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Left ventricular non-compaction is a myocardial disorder characterized by excessive trabeculations and deep recesses that communicate with the ventricular cavity, which is thought to result from a failure of the trabecular regression that occurs during normal embryonic development. It carries a high mortality from heart failure or sudden cardiac death. A 15-year-old female patient was referred to our institution for moderate symptoms of heart failure. Echocardiography and MRI showed a bicuspid aortic valve with severe regurgitation, subaortic VSD, dilated left ventricle and left ventricular non-compaction with a moderately decreased ejection fraction, as well as isthmic coarctation and transverse arch hypoplasia. We elected to perform transaortic VSD closure and aortic valve replacement using a mechanical prosthetic valve on an arrested heart, and to address aortic coarctation and transverse arch hypoplasia using an extra-anatomic ascending-to-descending aorta bypass. Aortic cross-clamping was limited to 41 minutes. The postoperative recovery was rapid and the girl was discharged in NYHA class I with an estimated LVEF of 39%. Although management must be individualized, extra-anatomic bypass is a good single-stage approach for patients with complex coarctation and concomitant cardiovascular or myocardial disorders, reducing ischemic time and offering a better chance of successful weaning from cardiopulmonary bypass.
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PMID:Complex aortic coarctation, regurgitant bicuspid aortic valve with VSD and ventricular non-compaction: a challenging combination. 2143 59

Valvular disorders are common, and result in a neurohormonal milieu similar to the heart failure state. Although valve surgery is the therapy of choice in symptomatic severe lesions, many patients do not receive surgery for a variety of reasons. Beta-blockers have a role in the management of many patients with valvular disorders, especially in the case of patients with mitral stenosis, where they reduce the transmitral gradient. They may also serve as life-saving therapy in pregnant women with pulmonary edema. Other uses of beta-blockers include the reduction of valve-related hemolysis, the prevention of atrial fibrillation, and the relief of dynamic left and right ventricular outflow tract obstruction. The prevention of aortic root dilation, potentially with beta-receptor blockade, may reduce the risk of aortic insufficiency in Marfan syndrome, and also in those with bicuspid aortic valves or following the Ross procedure. In this review, the potential role of beta-blockers is explored for the treatment of severe mitral and aortic regurgitation and asymptomatic severe aortic stenosis.
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PMID:Beta-blocker therapy for valvular disorders. 2171 12

Noncompaction of the ventricular myocardium (NVM) is a rare cardiac anomaly which can lead to the development of cardiomyopathy. NVM can be isolated or associated with congenital heart anomalies such as ventricular septal defect, atrial septal defect, pulmonic stenosis, bicuspid aortic valve, and Ebstein anomaly. NVM usually presents with heart failure symptoms, ventricular tachyarrhythmias, and thromboembolic events. We report two cases of NVM, one associated with a ventricular septal defect and other associated with bicuspid aortic valve.
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PMID:Coexistence of congenital heart anomalies with noncompaction of the ventricular myocardium. Two case reports. 2230 32

We present a 30-year-old male patient who developed new-onset heart failure and stroke in the setting of myocardial noncompaction. He presented with shortness of breath that progressively worsened in the past 15 days. Chest X-ray showed diffuse bilateral infiltration with bilateral hilar fullness. Electrocardiography showed sinus rhythm, signs of left ventricular hypertrophy, and diffuse T-wave negativity. Transthoracic echocardiography demonstrated moderate systolic dysfunction of the left ventricle and trabeculations and intertrabecular recesses in the anterolateral and apical regions of the inferior wall. Dense spontaneous echo contrast was seen in the left ventricular cavity. He also had a bicuspid aortic valve. The patient developed stroke at the eighth hour of hospitalization. Heparin infusion and heart failure treatment were started. Transesophageal echocardiography showed no thrombus. Clinical and radiological findings improved significantly during the follow-up. Oral anticoagulant therapy was initiated and the patient was discharged with control recommendations. Myocardial noncompaction was also demonstrated by cardiac magnetic resonance imaging.
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PMID:[Development of heart failure and stroke in the setting of myocardial noncompaction]. 2239 77

Congenital heart disease is the most common birth defect in humans. Identifying factors that are critical to embryonic heart development could further our understanding of the disease and lead to new strategies of its prevention and treatment. Nitric oxide synthase-3 (NOS3) or endothelial nitric oxide synthase (eNOS) is known for many important biological functions including vasodilation, vascular homeostasis and angiogenesis. Over the past decade, studies from our lab and others have shown that NOS3 is required during heart development. More specifically, deficiency in NOS3 results in congenital septal defects, cardiac hypertrophy and postnatal heart failure. In addition, NOS3 is pivotal to the morphogenesis of major coronary arteries and myocardial capillary development. Interestingly, these effects of NOS3 are mediated through induction of transcription and growth factors that are crucial in the formation of coronary arteries. Finally, deficiency in NOS3 results in high incidences of bicuspid aortic valves, a disease in humans that often leads to complications with age including aortic valve stenosis or regurgitation, endocarditis, aortic aneurysm formation, and aortic dissection. In summary, these data suggest NOS3 plays a critical role in embryonic heart development and morphogenesis of coronary arteries and aortic valves.
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PMID:NOing the heart: role of nitric oxide synthase-3 in heart development. 2257

We report the case of 42-year-old male, who developed infective endocarditis on bicuspid aortic valve due to infection of the central line with Staphylococcus aureus MSSA. The patient was hospitalised several times in district hospitals because of worsening of heart failure. He had a few transthoracic (TTE) and one transesophageal (TEE) echocardiography which did not reveal early stages of perivalvular abscess and was disqualified from surgical procedures due to bad left ventricular systolic function. The second TEE done a month after the first one showed an abscess with perforation to the right ventricle. The patient died disqualified again from surgical procedure due to multiorgan failure.
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PMID:[Is establishing diagnosis of perivalvular abscess still difficult?]. 2318 May 27

We report a case of fistula between the descending aorta and the left atrium with associated aortopulmonary window and secundum atrial septal defect. No previous reports of such association have been found in the literature. A 5-month-old infant presented with heart failure from the age of 2 weeks. Echocardiography confirmed the presence of an aorto-left atrial fistula, aortopulmonary window, and atrial septal defect. The diagnosis was additionally supported by computed tomography. The entity aorto-left atrial fistula is rare. It is mostly acquired and usually between the ascending aorta and the right atrium or right ventricle or left ventricle. We do not know about all the associated anomalies, which have implications in management. There is a case report of ascending aorto-left atrial fistula associated with bicuspid aortic valve and anomalous origin of coronaries. Here we are reporting a case of descending aorto-left atrial fistula associated with aortopulmonary window.
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PMID:Association of congenital descending aorto-left atrial fistula with the aortopulmonary window and atrial septal defect. 2338 15

A 51-year-old lady presented with increasing heart failure symptoms and palpitations. She had recently been diagnosed with a congenital ventricular septal defect, bicuspid aortic valve, and Eisenmenger's syndrome. There was clinical evidence of right heart failure and differential clubbing and cyanosis affecting the feet but not hands. A cardiac magnetic resonance imaging demonstrated interruption of the aortic arch beyond the left subclavian artery, with the descending aorta perfused entirely through a large patent ductus arteriosus.
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PMID:Interrupted aorta diagnosed in a 51-year-old woman. 2412 98

The management of concomitant aortic and aortic valve disease with left ventricular assist device (LVAD) implantation for patients with severe cardiomyopathy is challenging, and has not been established given the complexity of LVAD surgery with concomitant aortic interventions. A 45-year-old patient presented to our institution with end-stage heart failure symptoms and non-ischemic cardiomyopathy. The patient was found to have a bicuspid aortic valve, severe native aortic regurgitation, a significant ascending aortic aneurysm, and severely depressed left ventricular (LV) function requiring two inotropes. He underwent a successful hemiarch repair of the ascending aortic aneurysm using a back table outflow graft anastomosis technique, and subsequent placement of a HeartWare Ventricular Assist Device (HVAD) with concomitant aortic valve closure with a modified Park's stitch. The patient did well postoperatively and is currently listed for heart transplantation.
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PMID:Back table outflow graft anastomosis technique for HeartWare HVAD implantation. 2464 22

We present a case of fulminant diffuse systemic sclerosis (dSSc) developed after the aortic valve replacement followed by fatal congestive heart failure within the 6 months from the initial symptoms. A 61-year-old male developed rapidly progressive diffuse systemic sclerosis following aortic valve replacement due to stenosis of bicuspid aortic valve. He presented with diarrhoea, weight loss, mialgia and arthralgia after cardiac surgery. Heart failure, due to myocardial fibrosis, was noted as a cause of death. We hypothesize that artificial materials like the ones used in mechanical valves or silicon materials in breast implants may induce fulminant course of pre-existing systemic sclerosis or create a new onset in predisposed individual.
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PMID:Fulminant diffuse systemic sclerosis following aortic valve replacement. 2473 43


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