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Cardiac changes in systemic amyloidosis: visualisation by magnetic resonance imaging.

Cardiac involvement is a significant cause of morbidity and mortality in patients with amyloidosis. A 73-year old Caucasian woman was admitted to a hospital with increasing dyspnoea. Examination revealed a large left-sided pleural effusion, gross peripheral oedema and a pan-systolic murmur. The electrocardiogram showed low voltage QRS complexes with lateral T wave inversion. Recent angiography had demonstrated normal coronaries. Echocardiography revealed left ventricular hypertrophy and an ejection fraction of 43%. There was a restrictive filling pattern with elevated left-sided filling pressures, left atrial enlargement and mild mitral regurgitation. The right heart was normal with normal filling pressures. The patient was referred for cardiovascular magnetic resonance imaging for investigation of restrictive cardiomyopathy. This case provides a striking demonstration of the characteristic features of cardiac amyloidosis by cardiovascular magnetic resonance imaging - impaired biventricular systolic function, thickened atrioventricular valves, bi-atrial enlargement, increased atrial septal thickness and left ventricular mass, pleural and pericardial effusions, and the most impressive finding of widespread subendocardial hyperenhancement of both ventricles, as well as the inter-atrial and inter-ventricular septa, representing infiltration with amyloid protein. Cardiac involvement with systemic amyloidosis was suspected and confirmed on biopsy. Cardiovascular magnetic resonance imaging should be considered early in the diagnostic work-up of suspected cardiac amyloidosis.

Aged↗

Echocardiographic assessment of right atrial thrombus related to the implanted port device in patient receiving chemotherapy for non-Hodgkin's lymphoma.

The case of a potentially life-threatening complication related to the use of implanted port device in a 8 year old Non-Hodgkin's Lymphoma patient receiving chemotherapy is described. The device was inserted in early 1997 and used repeatedly for chemotherapy without any complications. In late 1997 during routine screening for cardiac left ventricular function before re-introduction of chemotherapy, an abnormal 1.43 x 1.53 cm mass, consistent with a non-mobile thrombus was found in the right atrium. The initial thrombolytic therapy with recombinant tissue plasmin activator (rt-PA) infused by a central venous catheter was combined with daily echocardiographic examination in order to assess both the timing and mode of thrombus resolution. After 8 days systemic fibrinolytic therapy was discontinued as major hemorrhage from venipuncture sites occurred and the clot dissolution was not obtained. Patient underwent right atriotomy utilizing cardiopulmonary bypass and subsequent surgical thrombus removal was successful. The study evaluated the contribution of two-dimensional echocardiography (2D) in the follow-up of vascuport and other central venous catheter (CVC) location and early diagnosis of related complications such as thrombi. The authors consider that pulmonary flow analyzed with Doppler echocardiography as a reliable, suitable and non-invasive method to evaluate increased pulmonary artery pressure in children with right atrial thrombi and probability of pulmonary microembolism or embolism. As the incidence of right atrial thrombi is highly associated with the catheter tip position in the right atrium, in contrast to their positioning in the superior vena cava or in its junction with the right atrium, the authors recommend that special attention and effort should be given to placing of the catheter tip in the superior vena cava or in its junction with the right atrium avoiding the right atrium during the implantation procedure. The surgical right atrium thrombus removal in patients with no clot dissolution despite systemic thrombolytic treatment underscores the importance of surgical therapy in treating this life-threatening complication of indwelling catheters.

Antineoplastic Agents↗

[The plasma level of an inactive moiety of B-type natriuretic peptide precursor as a predictor of cardiac function during operations under extracorporeal circulation].

Ventricular cardiomyocytes secrete brain natriuretuc peptide (BNP) and an inactive moiety of its precursor (nt-proBNP). It is recommended the blood level of the latter should be monitored to ascertain the severity of myocardial lesion. This investigation was undertaken to study the diagnostic and prognostic value of a substantial increase in blood nt-proBNP levels (600 pg/ml or more) in patients operated on under extracorporeal circulation. Thirty-eight patients operated on for coronary heart disease were examined. Before surgery, the plasma concentration of nt-proBNP had been determined by electrochemiluminescence (Elescys, Roche). According to plasma nt-proBNP levels, the patients were divided into 2 groups: 1) 24 patients with nt-proBNP levels of less than 600 pg/ml; 2) 14 patients with its levels of 600 pg/ml or more. Group 1 patients showed signs of diastolic dysfunction of the right ventricle: the lower ratio of its early to atrial filling linear velocity integrals and the increased end-diastolic volume index. In the postperfusion period, nt-proBNP is a significant independent predictor of left ventricular function. The patients with the baseline nt-proBNP levels of above 600 pg/ml were found to have decreases in ejection fraction of the left ventricle and in its pump ratio along with elevated pulmonary wedge pressure.

Adult↗

[Right ventricular function in patients with chronic right ventricular infarction].

To assess right ventricular function in patients with chronic right ventricular infarction, Tc-99m angiocardiography was performed in 64 patients one to three months after the onset of myocardial infarction. These patients were categorized into four groups according to their hemodynamic data in the acute stage using the Forrester classification: 39 patients in group I, 15 in group II, eight in group III and two in group IV. Mean right atrial pressure was nearly equal to or greater than diastolic pulmonary arterial pressure in all patients in group III. We calculated right ventricular ejection fraction (RVEF) and the right ventricular end-diastolic volume index (RVEDVI) as the parameter of right ventricular function, and assessed right ventricular wall motion using the right ventricular regional ejection fraction images (RVREFI). 1. RVEF in group III (25 +/- 3%) was significantly lower than those in groups I, II and IV (44 +/- 6%, 45 +/- 7% and 37 +/- 4%, respectively), and RVEF of all patients in group III was less than 30%. 2. RVEDVI in group III (150 +/- 25 ml/m2) was significantly greater than those in groups I, II and IV (74 +/- 20 ml/m2, 59 +/- 14 ml/m2 and 91 +/- 36 ml/m2, respectively). 3. RVREFI in group III decreased at the inferior and/or septal regions of the right ventricle, indicating wall motion abnormalities at the corresponding sites. 4. Six patients in group III were examined by coronary angiography and all had definite lesions in the proximal portion of the right coronary artery.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Mechanical effects of inspiration on heart functions: a review.

Left ventricular stroke volume decreases during inspiratory efforts whether lung volume is actually increasing as in normal inspiration or whether it remains constant as in a Mueller Maneuver (M.M.). Explanations have included phase lag between lung volume changes and left ventricular volume changes during inspiration as well as increased capacity of pulmonary vessels due to inflation. The capacitance changes could not be used to explain stroke volume (SV) fall in Mueller Maneuvers where lung volume is constant. The increased negative pleural pressure may be responsible for increases in right heart volume due to increased venous return. This mechanism has also been suggested as one of the causes of the fall in left ventricular stroke volume by ventricular interdependence due to changes in left ventricular pressure/volume (P/V) relations, i.e., compliance. In all these explanations a decrease in left ventricular diastolic filling and decreased diastolic pressure is assumed. The fact that decreases in pleural pressure may also act like an increase in left ventricular afterload and impede outflow, thereby decreasing stroke volume, has not been generally considered. We found that left ventricular stroke volume can still decrease without a decrease in left ventricular filling and even when increases in venous return have been prevented (right-heart bypass, i.e., constant pulmonary inflow). Thus the fall in stroke volume during inspiratory effort appears to be caused by a variety of factors, among which increased left ventricular afterload must be considered of primary importance. In addition, decreased left ventricular diastolic compliance due to increased right ventricular (RV) volume also has to be considered (interdependence).

Animals↗

Comparison of left atrial and left ventricular performance in conscious dogs.

The velocity of contraction has been reported to be more rapid in left atrium (LA) than left ventricle (LV), but the mechanics of contraction in these two chambers have not been compared under physiological conditions. Accordingly, sonomicrometer crystals and solid state pressure transducers were used to measure LV and LA systolic function simultaneously in seven conscious dogs. Two sets of crystals (LA-1, LA-2) were placed perpendicularly across the LA. Phenylephrine was infused at increasing doses from 0.4 to 1.2 microgram . kg-1 . min-1 to increase LV and LA afterloads. Constant heart rates were maintained by atrial pacing. The results indicated that LA motion was virtually identical in both diameters. LA fractional shortening (0.07 +/- 0.01) and ejection time (0.057 +/- 0.002 s) were much smaller than the ventricular values (0.21 +/- 0.01 and 0.18 +/- 0.01 s, respectively). The velocity of circumferential fibre shortening (Vcf) for the LV (1.16 +/- 0.05 cir . s-1) was not significantly different from LA-1 Vcf (1.19 +/- 0.09 cir . s-1) or LA-2 Vcf (1.22 +/- 0.08 cir . s-1). During phenylephrine infusions, LV systolic pressure increased from 120.0 +/- 3.4 to 190.8 +/- 15.5 mmHg and LV end-diastolic pressure increased from 3.6 +/- 0.3 to 27.7 +/- 2.7 mmHg. When Vcf for each chamber was plotted against the peak systolic pressure in that chamber, the curve of the LA was located upward and to the right of the LV curve.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

Surgical treatment of Ebstein's anomaly. Early and late results in seven consecutive cases.

The surgical treatment of Ebstein's anomaly is discussed on the basis of our experience with 7 patients who underwent operation at this clinic during the last ten years. The age of the patients (2 males, 5 females) ranged from 17 to 47 years (mean 27 years) at the time of operation. Two patients were in functional class II and five in classes III or IV (N.Y.H.A.). The Wolff-Parkinson-White syndrome was concomitant in 2/7 cases and patent foramen ovale or atrial septal defect (ASD) with right-to-left shunt was present in 6/7 cases. The valvular anomaly was typical in 5 patients and stenosis of displaced tricuspid leaflets into right ventricular outflow region was detected in two patients. Tricuspid valve replacement (TVR) with disc valve prosthesis was performed in 5 patients (Björk-Shiley model in 4 cases, Kay-Shiley model in one) and tricuspid valve commissurotomy in two patients. All six patients with interatrial communication underwent closure of this communication simultaneously. One patient had concomitant epicardial mapping and attempted division of the anomalous conduction pathways was unsuccessful. This patient died on the 14th postoperative day from ventricular fibrillation. No other early or late mortality was recorded. Life-long anticoagulation therapy was introduced in all 5 patients with TVR. Thrombotic malfunction of the prosthetic valves necessitated re-operation and new prosthetic valve replacement in two patients 15 and 16 months after the first TVR. One of these patients had recurrent prosthetic thrombosis twice which was successfully treated with streptokinase. The other patient's prosthesis is functioning well 18 months after re-operation. All 6 long-term survivors were improved and remained in good condition, in functional class I, 1, 3, 3, 8, 9, and 10 years, respectively, after operation.

Adolescent↗

One and a half ventricle repair with pulsatile bidirectional Glenn: results and guidelines for patient selection.

BACKGROUND: The guidelines for performing a one and a half ventricle repair with pulsatile bidirectional Glenn remains controversial. This retrospective report summarizes the experience of a single institution, with an attempt at providing an answer. METHODS: Fifty consecutive patients, aged 4 months to 42 years, underwent intracardiac repair along with a superior cavopulmonary connection. Twenty-seven of the patients had had previous surgical palliation. Repair consisted of patch closure of the ventricular septal defect (n = 25), tricuspid valve repair (n = 26), reconstruction of the right ventricular outflow tract (n = 34), transpulmonary annular patch (n = 34), right ventricle to pulmonary artery homograft conduit (n = 4), and concomitant repair of atrioventricular canal (n = 9). Ten patients were left with a fenestration in the atrial septum. RESULTS: There were six hospital deaths (12%) and two late deaths (4.5%). Forty-two survivors were followed from 8 months to 116 months. Eighty-eight percent are in functional class I. Actuarial survival at 97 months was 74%. CONCLUSIONS: Moderate right heart hypoplasia constitutes a safe anatomic category for a pulsatile bidirectional Glenn. It is advisable not to proceed with a one and a half ventricle repair if postoperative residual pulmonary artery hypertension is anticipated. Patients requiring an intricate intracardiac repair and those with concomitant right heart hypoplasia may be better suited for a Fontan type of repair to reduce the complexity of the procedure.

Actuarial Analysis↗

Transient atrial dysfunction after conversion of chronic atrial fibrillation to sinus rhythm.

Although conversion of atrial fibrillation (AF) to sinus rhythm can usually be accomplished by electrical or drug therapy, effective atrial systole may not be restored. To investigate the return of atrial transport function and its relation to the duration of the arrhythmia, Doppler echocardiography was performed after conversion in 18 patients with acute AF (less than or equal to 1 week duration), 14 patients with chronic AF (greater than 1 week duration) and 15 control patients. Flow velocities during rapid filling (E wave) and atrial systole (A wave) were measured in both left and right ventricles. Patients in the acute AF group had left ventricular A waves (49 +/- 4 cm/s) and A/E ratios (0.97 +/- 0.1) similar to those of the control patients (55 +/- 7 cm/s, 0.87 +/- 0.08, respectively). In contrast, patients in the chronic AF group had much smaller A waves (19 +/- 5 cm/s) and A/E ratios (0.30 +/- 0.08) than those in the other 2 groups (p less than 0.001). Five patients with chronic AF (36%) had complete left atrial paralysis (A/E = 0) despite normal sinus P waves. Measurements in the right ventricle showed similar differences among the groups. Patients with chronic AF who maintained sinus rhythm showed an increase in A/E ratio to control levels, from 0.45 +/- 0.1 to 0.93 +/- 0.1 (p = 0.003) at 48 days (average) after conversion. Thus, atrial transport function is normal after brief periods of AF, but reduced or absent when conversion is achieved after the arrhythmia has been sustained greater than 1 week.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute Disease↗

Epicardial sites for vagal mediation of sinoatrial function.

The posterior atrial fat pad (PAFP) has been described as the probable anatomic location of parasympathetic ganglia mediating sinoatrial (SAN) and atrioventricular nodal function in the mammalian heart. This contrasts with recent localizations of such control elements in the pulmonary vein fat pad (PVFP) and in fatty tissues overlying the junction of inferior vena cava-inferior left atrium (IVC-ILA), respectively. Short bursts (5-8 pulses/burst, 3 bursts/train) of electrical current (1-16 Hz, 400 ms, 1-5 mA) applied directly to the ventral right atrial epicardium via a concentric bipolar electrode (separation 0.3-0.6 mm) during the atrial muscle refractory period, activated subepicardial postganglionic pathways from PVFP and entering the SAN; identical stimulation of dorsal right atrial epicardium between PAFP and SAN excited few or no fiber pathways controlling SAN discharge rate or patterns. In a second series of experiments, injection of a neuronal marker (Fast Blue) into and around SAN, with time (5-10 days) allowed for retrograde transport, resulted in staining of many soma in PVFP but none in IVC-ILA or PAFP. These data strongly affirm the primary, and perhaps exclusive, localization of ganglia that mediate parasympathetic regulation of SAN function in PVFP of the dog's heart, with little or no such participation by ganglia within PAFP or IVC-ILA.

Adipose Tissue↗

[Changes in the level of plasma atrial natriuretic factor in patients with chronic renal failure].

Changes of plasma atrial natriuretic factor (ANF) levels in 89 patients with different kinds of renal diseases (including 39 patients treated with hemodialysis) were studied. The results showed: 1. The mean value of plasma ANF level was significantly increased in patients with renal diseases than that in healthy subjects. The increase was positively correlated with the change of creatinine, serum potassium, mean arterial pressure and ptfV1. The plasma ANF level was markedly decreased after hemodialysis. 2. The right atrial pressure of 8 patients with chronic renal failure measured by right heart catheterization was normal. No significant changes occurred after hemodialysis. 3. Impedance cardiogram and mechanical cardiogram showed that left heart function was significantly improved after hemodialysis. 4. ANF was detected in ultrafiltration liquid. 5. There was no difference of the ANF content between the serum of the first and maintenance hemodialysis. The authors thought that the main causes of increase of plasma ANF level are decrease of catabolic metabolism in renal failure, stimulation of ANF secretion by left heart failure and increased loading of left atrium and increased production of ANF by body compensation as the result of mean arterial pressure increase. The decrease of ANF after hemodialysis was mainly due to improvement of renal and cardiac function, lessening of the left atrial loading, the reduced mean arterial pressure and its direct elimination by hemodialysis.

Adolescent↗

Effect of rate and rhythm control on left ventricular function and cardiac dimensions in patients with persistent atrial fibrillation: results from the RAte Control versus Electrical Cardioversion for Persistent Atrial Fibrillation (RACE) study.

OBJECTIVES: The purpose of this study was to evaluate left ventricular function and atrial and ventricular diameters in patients with persistent atrial fibrillation (AF) treated with rate or rhythm control. BACKGROUND: Restoration of sinus rhythm in patients with persistent AF may improve left ventricular function and reduce atrial dimensions. Adequate rate control in AF may preserve ventricular function. METHODS: In 335 patients included in the RAte Control versus Electrical Cardioversion for Persistent Atrial Fibrillation (RACE) study, echocardiography was performed at baseline and 1- and 2-year follow-up. Echocardiography was compared between patients randomized to rate control (n = 160) and rhythm control (n = 175). In the rhythm control group, echocardiography was compared between patients with AF versus sinus rhythm at study end. Multivariate analysis was performed to determine parameters related to improvement of left ventricular function and increase of atrial diameters. RESULTS: Fractional shortening improved significantly under rate and rhythm control (31 +/- 10% at baseline to 33 +/- 9% at 2 years, and from 30 +/- 10% to 34 +/- 9%; both P < .05, respectively). Under rate control, left and right atrial size increased significantly compared to baseline. Under rhythm control, only left atrial size increased. Multivariate analysis revealed that only sinus rhythm at study end was associated with an increase of fractional shortening. AF at study end, hypertension, and no use of angiotensin-converting enzyme inhibitors were independently associated with increase in atrial size. CONCLUSIONS: Routine rate control prevents deterioration of left ventricular function. Maintenance of sinus rhythm is associated with improvement of left ventricular function and reduction of atrial sizes.

Aged↗

[Congestive heart failure in the elderly: severe tricuspid regurgitation by two-dimensional Doppler echocardiography].

We studied five cases of atrial fibrillation in the elderly showing severely decreased exercise tolerance (NYHA III) and normal left ventricular function. There were no signs of classical tricuspid regurgitation. Both atria were enlarged and right ventricular sizes were shown by two-dimensional echocardiography to have mildly increased. Two-dimensional Doppler echocardiography revealed severe tricupid regurgitation but at a maximal velocity less than 2.3 m/sec, meaning that right ventricular pressure was not increased. Cardiac output (CO) and circulating blood volume (BV), measured by radiocardiography showed CO to have decreased and BV to have increased. No intracardiac shunt was detected. The cause of this decreased exercise tolerance in the group in our study is thought to be right ventricular dysfunction due to the aging process. This also occurs in a similar pattern in younger people who have arrhythmogenic right ventricular dysplasia (ARVD), Uhl's disease, or right ventricular dilated cardiomyopathy.

Aged↗

Preparation for repeated study of left ventricular function in the conscious dog.

A method is presented for a relatively simple nontraumatic chronic left heart catheter preparation for the study of left ventricular hemodynamics in the conscious dog. In 30 dogs an 8 Fr Cordis catheter was modified and implanted into the left ventricle via the right atrial septum. Transseptal catheterization was performed without significant morbidity and mortality. Left ventricular cineangiograms and pressures and cardiac outputs have been repeatedly performed on fully conscious dogs with no apparent discomfort displayed by the dog.

Animals↗

[The echocardiographic examination in adult cardiomyopathy].

Echocardiography allows distinction between the diastolic dysfunction of hypertrophic or restrictive cardiomyopathies and the systolic dysfunction of dilated cardiomyopathy. The diagnosis and prognosis may be deduced from echocardiographic parameters. In hypertrophic cardiomyopathy systolic function is normal and there is asymmetric left ventricular hypertrophy (> 13 mm) associated with a reduced diastolic dimension and atrial dilatation resulting from diastolic dysfunction. The prognosis could be related to the severity of left ventricular hypertrophy; right ventricular hypertrophy is uncommon and its severity seems to be related to that of left ventricular hypertrophy. Restrictive cardiomyopathies are less common and amyloidosis is the commonest cause. Symmetric hypertrophy with reduced diastolic dimensions is observed; right ventricular involvement occurs in about 30% of cases. The prognosis seems to be related to the degree of parietal infiltration and, at advanced stages, systolic function is abnormal (fractional shortening < 20% with a left ventricular diastolic dimension > or = 55 mm) and rapidly fatal. Dilated cardiomyopathy is diagnosed when wall thickness is normal but left ventricular diastolic dimensions > 27 mm/m2 and ejection fraction < 45%. Right and left ventricular dimensions of the same size, left ventricular diastolic dimensions > 70 mm and left ventricular ejection fractions < 20% are poor prognostic indicators.

Adult↗

Acute effects of intravenous nicardipine on hemodynamics and cardiac function in patients with a healed myocardial infarction and no evidence of congestive heart failure.

Acute effects of intravenous nicardipine (10 micrograms/kg) on systemic hemodynamics and cardiac function were evaluated in 17 patients with a healed myocardial infarction and no evidence of congestive heart failure. Mean New York Heart Association functional class was 1.6 +/- 0.5 (mean +/- standard deviation). Aortic systolic pressure (p less than 0.001) and left ventricular end-diastolic pressure decreased (10 +/- 3 to 8 +/- 3 mm Hg, p less than 0.01), and systemic vascular resistance decreased significantly (p less than 0.001), whereas pulmonary and right atrial pressure and pulmonary arteriolar resistance did not change. Cardiac and stroke indexes showed biphasic changes. Although positive and negative maximal rate of left ventricular pressures decreased significantly (p less than 0.05 and p less than 0.01, respectively), they did not change significantly when aortic systolic pressure was corrected. There was a significant inverse correlation between the negative rate of left ventricular pressure/aortic systolic pressure before nicardipine infusion and its maximal percent increase after infusion (r = -0.56, p less than 0.05), indicating a beneficial effect on diastolic relaxation in patients with impaired diastolic function. Our data show that a low dose (10 micrograms/kg) of intravenous nicardipine exerts a favorable effect on impaired diastolic function, but depresses left ventricular pump function with much less effect on right heart circulation.

Adult↗

The failing Fontan circulation: successful conversion of atriopulmonary connections.

OBJECTIVES: Symptoms from low cardiac output or refractory atrial arrhythmias are complicating atriopulmonary (classical) Fontan connections. We present our experience of converting such patients to total cavopulmonary connections with and without arrhythmia surgery. METHODS: Between 1997 and 2002, 15 patients (mean age, 19.7 +/- 7.0 years) underwent conversion operations 12.7 +/- 3.5 years after atriopulmonary Fontan operations. Preoperative New York Heart Association functional class was I in 2 patients, II in 2 patients, III in 6 patients, and IV in 5 patients. Four patients underwent intracardiac lateral tunnel conversion alone, and 11 received extracardiac total cavopulmonary connection, right atrial reduction, and cryoablation. RESULTS: No mortality occurred. One patient had conduit obstruction in the immediate postoperative period requiring replacement, and another required a redo operation for endocarditis. Average hospitalization was 17.9 +/- 9.38 days; chest drains were removed on median day 4 (range, 1-29; mean, 7.4 +/- 7.58 days). At follow-up (mean, 42.6 +/- 22.1 months), late atrial arrhythmias had recurred in 3 of 4 patients with intracardiac total cavopulmonary connections (without ablation) and 1 of 11 patients with extracardiac total cavopulmonary connections with ablation. All patients are in New York Heart Association class I or II. Exercise ability (Bruce protocol) improved 69% from a mean of 6.18 +/- 4.01 minutes to 10.45 +/- 2.11 minutes (P <.05). Need for antiarrhythmic agents decreased postoperatively (patients receiving < or =1 antiarrhythmic: 9 preoperatively vs 15 at long-term follow-up, P <.05). No patient has required transplantation. Protein-losing enteropathy, which was present in 1 patient, improved transiently with conversion. There was 1 late death from gastrointestinal hemorrhage. CONCLUSIONS: Fontan conversion can be achieved with low mortality and improvement in New York Heart Association class and exercise ability. Concomitant arrhythmia surgery reduces the incidence of late arrhythmias.

Adolescent↗