Progressive Heart Failure Symptoms Research Articles

Echo-guided percutaneous coil embolization of a symptomatic massive metastasis of a renal cell carcinoma in the right ventricular outflow tract

We present the case of a 41-year-old woman who was admitted to our centre with progressive symptoms of congestive heart failure (NYHA class III) 5 years after a radical nephrectomy for renal cell carcinoma. Magnetic resonance imaging demonstrated a 5 x 3 cm homogeneous intracardial mass causing right ventricular outflow tract obstruction, not accessible to surgical resection. Serial echo-guided, percutaneous coil embolization of the cardial metastasis was performed with Contour SE Microparticles (150-250 or 300-500 microm) after identification of the target region of the metastasis by contrast injection (Levovist) through the balloon catheter into the coronary artery under transoesophageal echocardiographic control prior to induction of the necrosis, corresponding to the technique which has been described for septal ablation in hypertrophic obstructive cardiomyopathy. Follow-up after serial embolization showed a good haemodynamic and a marked clinical response (dyspnoea NYHA I-II) which lasted during the 19 month of survival after the index procedure.

Heart Retransplantation

Retransplants comprise only a small minority (3-4%) of heart transplants, however outcome following retransplantation is compromised. Risk factors for a poor outcome following retransplantation include retransplantation early (<6 months) after primary transplant, retransplantation for acute rejection or early allograft failure, and retransplantation in an earlier era. The incidence of rejection and infection is similar following primary transplant and retransplantation. The compromised outcomes and risk factors for a poor outcome are similar in adult and pediatric heart retransplantation. However, due to the short half-life of the transplanted heart, it is an expectation that patients transplanted in childhood may require retransplantation. Based on the data available and the opinion of the working group, indications for heart retransplantation are (i) chronic severe cardiac allograft vasculopathy with symptoms of ischemia or heart failure (should be considered) or asymptomatic moderate or severe left ventricular dysfunction (may be considered) or (ii) chronic graft dysfunction with symptoms of progressive heart failure in the absence of active rejection. Patients with graft failure due to acute rejection with hemodynamic compromise, especially <6 months post-transplant, are inappropriate candidates for retransplantation. In addition, guidelines established for primary transplant candidacy should be strictly followed.

When is Retransplantation a Viable Option?

As the number of recipients of heart transplantation grows over time and they survive longer, more are at risk for developing severe cardiac allograft vasculopathy and allograft dysfunction, which might lead to consideration for retransplantation. Clearly, outcomes following cardiac retransplantation are compromised, and with donor shortage, the selection of candidates must be judicious. Retransplantation appears most appropriate for those patients more than 6 months following original heart transplantation, who have severe cardiac allograft vasculopathy and associated left ventricular dysfunction, or allograft dysfunction and progressive symptoms of heart failure in the absence of acute rejection. Relative contraindications to transplantation (ie, advanced age, comorbidities, psychosocial issues) require thorough assessment when retransplantation is being considered.

Giant free floating left atrial thrombus

A 74-year-old woman with dilative cardiomyopathy and chronic atrial fibrillation presented with symptoms of progressive heart failure. Transthoracic echocardiography showed a giant free floating mass in the dilated left atrium (Fig. 1A and B). The mass was surgically removed demonstrating an organized thrombus without wall attachment (Fig. 2). The postoperative course was uneventful under optimised anticoagulation. Fig. 1. Transthoracic echocardiography at admission showing a large free floating mass in the left atrium which is occassionally located at the dorsal wall (A) but approaches also the orifice of the mitral valve (B). Appendix. Supplementary data

Clinical and echocardiographic features of aorto-atrial fistulas

Aorto-atrial fistulas (AAF) are rare but important pathophysiologic conditions of the aorta and have varied presentations such as acute pulmonary edema, chronic heart failure and incidental detection of the fistula. A variety of mechanisms such as aortic dissection, endocarditis with pseudoaneurysm formation, post surgical scenarios or trauma may precipitate the fistula formation. With increasing survival of patients, particularly following complex aortic reconstructive surgeries and redo valve surgeries, recognition of this complication, its clinical features and echocardiographic diagnosis is important. Since physical exam in this condition may be misleading, echocardiography serves as the cornerstone for diagnosis. The case below illustrates aorto-left atrial fistula formation following redo aortic valve surgery with slowly progressive symptoms of heart failure. A brief review of the existing literature of this entity is presented including emphasis on echocardiographic diagnosis and treatment.

Tropical endomyocardial fibrosis (Davies? disease): case report demonstrating the role of magnetic resonance imaging

Tropical endomyocardial fibrosis (TEMF), a restrictive cardiomyopathy of unclear etiology, is an endemic disease in equatorial Africa, South America and India. The patients are usually young, the onset of the disease and its clinical manifestations insidious, and the prognosis poor. We currently present a 50-year-old Congolese female who was referred with symptoms of progressive right-sided heart failure due to isolated TEMF of the right ventricle. Surgical resection of regional endomyocardial fibrosis was not possible and our patient was referred for cardiac transplantation. Cardiac magnetic resonance imaging (CMR) demonstrated the primary and secondary structural and functional abnormalities. CMR seems ideally suited to diagnose this condition and monitor response to medical and/or surgical therapy.

1032-121 Utility of B-type natriuretic peptide assay in the assessment of symptomatic state in hypertrophic cardiomyopathy

Background. Hypertrophic cardiomyopathy (HCM) is a common genetic cardiovascular disease with a diverse clinical spectrum which often includes functional disability due to progressive heart failure symptoms at any age. Assessment of symptom severity may be highly subjective and encumbered by the heterogenous clinical presentation of HCM. Plasma B-type natriuretic peptide (BNP) has been used widely as an objective marker for the severity of heart failure and clinical outcome predominantly in coronary heart disease with ventricular dilatation and systolic dysfunction. We considered the possibility that BNP would be an accurate and independent predictor of heart failure severity in HCM, a disease characterized by intact ventricular function in the absence of chamber dilatation. Methods. We prospectively assessed plasma BNP as a quantitative clinical marker of heart failure severity in 107 consecutive HCM patients. Results. BNP showed a statistically significant relationship to the magnitude of functional limitation assessed by New York Heart Association (NYHA) functional class: I: 136 ± 159 pg/ml; II: 338 ± 439 pg/ml; III/IV: 481 ± 334 pg/ml (p 200 pg/ml was the most reliable predictor of heart failure symptoms with positive and negative predictive values of 65% and 79%, respectively. Conclusions. Plasma BNP is independently related to the presence and magnitude of heart failure-related symptoms in patients with HCM. The clinical power of BNP as a marker for heart failure in HCM is, however, restricted by the overlap in BNP values between symptom-related subgroups, due largely to the important confounding variables of advancing age and substantial left ventricular wall thickness characteristic of this heterogeneous disease.

Effect of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy.

The influence of left ventricular outflow tract obstruction on the clinical outcome of hypertrophic cardiomyopathy remains unresolved. We assessed the effect of outflow tract obstruction on morbidity and mortality in a large cohort of patients with hypertrophic cardiomyopathy who were followed for a mean (+/-SD) of 6.3+/-6.2 years. Of the 1101 consecutive patients, 273 (25 percent) had obstruction of left ventricular outflow under basal (resting) conditions with a peak instantaneous gradient of at least 30 mm Hg. A total of 127 patients (12 percent) died of hypertrophic cardiomyopathy, and 216 surviving patients (20 percent) had severe, disabling symptoms of progressive heart failure (New York Heart Association [NYHA] functional class III or IV). The overall probability of death related to hypertrophic cardiomyopathy was significantly greater among patients with outflow tract obstruction than among those without obstruction (relative risk, 2.0; P=0.001). The risk of progression to NYHA class III or IV or death specifically from heart failure or stroke was also greater among patients with obstruction (relative risk, 4.4; P<0.001), particularly among patients 40 years of age or older (P<0.001). Age-adjusted multivariate analysis confirmed that outflow tract obstruction was independently associated with an increased risk of both death related to hypertrophic cardiomyopathy (relative risk, 1.6; P=0.02) and progression to NYHA class III or IV or death from heart failure or stroke (relative risk, 2.7; P<0.001). The likelihood of severe symptoms and death related to outflow tract obstruction did not increase as the gradient increased above the threshold of 30 mm Hg. In patients with hypertrophic cardiomyopathy, left ventricular outflow tract obstruction at rest is a strong, independent predictor of progression to severe symptoms of heart failure and of death.

Right ventricular dysplasia in an asymptomatic young man: An uncommon case with biventricular involvement and no known family history

A 33-year-old man had cardiomegaly on a routine x-ray examination. He was asymptomatic with no history of infarction, syncope, or palpitations. There was no family history of congenital heart disease or sudden death. Two-dimensional transthoracic echocardiography demonstrated marked enlargement of the right atrium and ventricle with severely depressed right and left ventricular function that was consistent with right ventricular dysplasia. The patient was treated with an angiotensin-converting enzyme inhibitor and did well for 6 months, but then developed symptomatic left-sided congestive heart failure. Short-term improvement was obtained with intravenous inotropic therapy, but he continued to have progressive symptoms of heart failure. Approximately 7 months after his initial presentation, the patient underwent orthotopic heart transplantation for intractable congestive heart failure. Pathologic examination of the explanted heart established the diagnosis of right ventricular dysplasia with left ventricular involvement. This is an uncommon presentation of right ventricular dysplasia with biventricular involvement and no known family history. (J Am Soc Echocardiogr 2001;14:317-20.)

Thrombosis of mechanical cardiac valves: A qualitative comparison of the Silastic ball valve and the tilting disc valve

A review of 817 mitral and aortic Silastic ball valve implantations with a follow-up of 3,554 total patient-years yielded only seven cases of valve thrombosis. Time-related risk was 0.4% per patient-year in the mitral position and 0.1% per patient-year in the aortic position. Four of five mitral and one of two aortic ball valve thromboses were successfully managed by valve rereplacement . At least five of the seven patients presented with a prodrome (lasting at least 3 months) of symptoms of progressive heart failure and, occasionally, embolic episodes due to gradually increasing prosthetic stenosis by thrombus. This lengthy time course is in contrast to the more frequent rapid catastrophic thrombosis that occurs with the Björk-Shiley tilting disc valve. Recognition of the prodrome of Silastic ball valve thrombosis provides an opportunity for life-saving surgical intervention.

Lethal tricuspid and mitral regurgitation in Marfan's syndrome ∗

A 4 3 4 month old white male infant with Marfan's syndrome had presenting clinical signs and symptoms of progressive congestive heart failure due to severe mitral and tricuspid insufficiency which resulted in death. The classic features of Marfan's syndrome that the patient exhibited included arachnodactyly, high-arched palate, floppy ears, iridodonesis and subluxated lenses. Cardiac catheterization and selective angiography documented the diagnosis of severe bilateral regurgitation from the atrioventricular valve. At autopsy, the cystic medial changes seen in Marfan's syndrome were observed in the pulmonary artery and aorta. Myxomatous transformation involving the mitral and tricuspid valves, with similar changes within the annulus, and fibrosis of each valve led to the hemodynamic alterations and demise. In all infants and children with otherwise unexplained valvular regurgitation, occult or overt Marfan's syndrome should be considered in the differential diagnosis. Furthermore, in infants and children with any of the clinical features of Marfan's syndrome careful cardiovascular evaluations should be made to detect valvular insufficiency due to fibromyxomatous abiotrophy.

Congenital heart disease: A clinical and pathological study of two cases of truncus solitarius aorticus (pulmonary atresia)

Atresia of the pulmonary artery is a relatively uncommon condition. In the cases with a defect in the interventricular septum, the average duration of life is three and four-tenths years according to the statistics collected by Abbott 2 in a review of 24 cases. The maximum age reached was thirteen years. In our Case 2 the child lived up to ten months. Its death was hastened by a mild bronchitis and bronchopneumonia. This anomaly is far more serious when associated with a closed interventricular septum. Of the 7 cases collected by Abbott, the average duration of life was twelve weeks, with a maximum age period of six months. Our Case 1 lived six months with symptoms of progressive heart failure and increasing cyanosis and dyspnea, finally succumbing to an erysipelas. Clinically, both children were cyanotic at birth. When admitted to the hospital, cyanosis, distinct clubbing of the fingers, and enlargement of the liver were noted. These symptoms were, however, more intense in Case 1, with the intact interventricular septum. Here, also, the diagnosis of a congenital cardiac defect was aided by the extreme enlargement of the organ and by the presence of a rough systolic murmur with a peculiar resonant quality suggestve of an aneurysm, heard best over the second interspace on the left side and transmitted to the apex. The x-ray film of the heart presented an unusual appearance, that of a large egg occupying almost the entire chest wall (Fig. 1). The correlation of the x-ray findings with the post-mortem findings showed the enlargement to be due to the aneurysm of the right ventricle and the dilated and hypertrophied right auricle. It was also possible to distinguish both ventricles on the x-ray plate. In the case with the defect in the interventricular septum no enlargement of the heart was noted, neither were there any murmurs heard. Zimmerman 11 recently reported a case of truncus arteriosus communis with an interventricular septal defect in a colored male of twenty-five years, the cause of whose death was an automobile accident. Considered from the point of view of mechanics of circulation, Zimmerman's case is similar to our second case in which the baby lived to the age of ten months. A case reported by Wheeler and Abbott 7 of pulmonary atresia and other cardiac anomalies survived until the age of twenty-nine. The absence of one coronary in both cases in an unusual anomaly which is extremely rare in otherwise normal hearts. It is well known that one coronary artery can be an adequate source of blood supply to maintain intracardiac circulation, provided there are sufficient anastomoses. 12 This fact is well demonstrated by Case 2 in which both ventricles were well developed. Microscopical examination of the myocardium showed no evidences of degeneration or scarring. The case with the closed interventricular septum and marked dilatation of the aplastic right ventricle with aplasia of the tricuspid valves is puzzling. The most obvious explanation as to the cause of the aplasia and dilatation of the right ventricle is a nutritional disturbance and the increased intraventricular pressure necessary to force blood into the left side of the heart. It is also possible that in this case the pulmonary artery was originally patient though incompletely developed. The right coronary artery may have had an abnormal origin from this vessel. With increasing stenosis of the pulmonary artery two phenomena arose: namely, the right coronary supply became insufficient and the right ventricle had to overcome the pressure of the left heart in order to force blood through the foramen ovale. Possibly both of these factors led to the final aplasia and dilatation of the right ventricle. While it is impossible to find traces of this hypothetical right coronary artery in this case, it is to be noted that a case of aplasia of the right ventricle occurring with atresia of the pulmonary artery and closed interventricular septum described by Abbott apparently possessed two coronary arteries arising from the aorta and, secondly, cases with abnormal origin of one coronary artery from the pulmonary artery described by Abrikossoff, 13 Heitzmann, 14 Krumbhaar, 15 Schley 16 and Scholte 17 showed evidences of myocardial degeneration and extensive fibrosis in the areas supplied by the misplaced coronary artery. In the case described by Abrikossoff, aneurysmal dilatation of the left ventricle was associated with a misplaced coronary artery arising from the pulmonary artery. The association of cardiac anomalies with abnormalities elsewhere in the body has been frequently emphasized. Other anomalies were present in both of our cases. Of special interest, however, was the presence of a neuroblastoma of the adrenal gland with metastases to the liver in Case 1, a child of six months.