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[Mechanical circulatory assistance using the Novacor implanted device. A postoperative follow-up].

The postoperative follow-up of 8 patients on the waiting list for cardiac transplantation, with implanted left ventricular assist devices of the Novacor type, was marked by right ventricular failure in the first week, controlled by positive inotropic agents and the maintenance of high right ventricular preload. The outcome later on showed that an ambulatory life was possible for 6 of the 8 patients, allowing the wait for cardiac transplantation under excellent conditions. Three major complications were observed: a case of aspergillosis endocarditis, one vertebrobasilar cerebrovascular accident and one low output state by progressive degradation of right ventricular function: after a period of circulatory assistance of 52 to 201 days. 6 out of the 8 patients underwent cardiac transplantation, 5 of which were successful.

Adolescent↗

[Pulmonary hypertension--some problems resolved. More remain to be solved].

Pulmonary hypertension especially arterial and idiopathic is still a poorly understood disease often affecting young adults and leading to right ventricular overload, failure and death due to low cardiac output. Justification for new, aggressive, costly but effective methods of pharmacological and interventional treatment of pulmonary hypertension require precise, repeatedly performed prognostic assessment. This is currently provided not only by repeated right heart catheterization but mostly by exercise testing as well as non-invasive techniques such as echo-Doppler and biochemical myocyte markers including B-type natriuretic peptide and cardiac troponin. Such methods should allow better timing of atrial septostomy and listing for lung transplantation.

Biomarkers↗

[Echocardiographic assessment of cor pulmonale in patients with cystic fibrosis].

Cystic fibrosis (CF) is an inherited multisystem disorder, characterized mainly by obstruction and infection of airways and by maldigestion and its consequences. An increasing interest concerning patients suffering from CF has been observed. Aim of this study was echocardiographic study of morphological structure, functional index and the grade of pulmonary hypertension in patients with CF. In 45 patients aged 3 to 24 years (mean 15.1 years) echocardiography was performed. Right ventricular end-diastolic dimension (RVDd) was increased in 67% of patients, right ventricular anterior wall thickness (RVAWd) was increased in 71% of patients. Pulmonary artery pressure was abnormally high in 84% of CF patients. Significant correlations between RVDd, RVAWd, PAP and Shwachman-Kulczycki clinical score was found. The dimensions of the left ventricular cavity were abnormal in 22% of patients. Insufficiency of pulmonary valve was observed in 47% of CF patients and tricuspid insufficiency in 53%. This study demonstrates that: 1) changes occurs mainly in right ventricle and appear to worsen as the disease progresses, 2) all patients with CF should remain under cardiological control, 3) echocardiography could be a useful method of long-term monitoring patients with CF.

Adolescent↗

Oxygenation improvement with nitric oxide in right-to-left shunt without significant effects on pulmonary arterial pressure.

Following surgical closure of an interventricular communication complicating an anterior myocardial infarction, a 74-year-old woman developed severe right ventricular failure and hypoxemia due to the opening of a patent foramen ovale (PFO). Mean pulmonary artery pressure was 24 mm Hg. Treatment with inhaled nitric oxide (5 ppm) increased PaO2 from 47 to 90 mm Hg (FIo(2)1). The present observation points out that nitric oxide inhalation could be useful in the management of severe hypoxemia from a right-to-left shunt due to a PFO even when there is no significant pulmonary hypertension present.

Administration, Inhalation↗

Right ventricular wall motion abnormalities found in healthy subjects by cardiovascular magnetic resonance imaging and characterized with a new segmental model.

AIM: To evaluate right ventricular wall motion abnormalities in healthy subjects using a new segmental model for the right ventricle. METHODS AND RESULTS: 29 healthy subjects (9 female, 20 male, mean age 48.9+/-15 years) underwent cardiovascular magnetic resonance imaging (CMR; 1.5-Tesla Sonata, Siemens, Erlangen, Germany) for the evaluation of cardiac function and right ventricular wall motion. A steady-state free precession gradient-echo sequence (TrueFISP) was used. Right ventricular wall motion was analyzed, and the site of areas of disordered motion was classified according to the new segmental model. Such areas were seen in 27 (93.1%) of the 29 subjects. Dyskinesia was found in 22 subjects (75.9%), hypokinesia in 11 (37.9%), and bulging in 8 (27.6%). The number of wall motion abnormalities diagnosed was significantly higher in the transverse plane (86.2%) than in the short-axis plane (13.8%) and the horizontal longitudinal plane (41.4%; p = 0.000). CONCLUSION: Right ventricular wall motion abnormalities are one of the criteria for the diagnosis of arrhythmogenic right ventricular cardiomyopathy. However, our findings indicate that they may also be seen around the insertion of the moderator band in healthy subjects, so that the significance of their presence at this site in patients undergoing diagnostic investigations for this disease should be interpreted with caution.

Adolescent↗

[Precapillary pulmonary hypertension of uncertain etiology].

HISTORY AND CLINICAL FINDINGS: A 39-year-old man was hospitalized for investigation of increasing dyspnoea for 3 month. On admission he was found to have bilateral ankle oedema, an enlarged liver and loud systolic murmur over the lower sternum. INVESTIGATIONS: There were signs of right heart strain/hypertrophy on the chest radiogram and echocardiogram. After treatment of right heart failure cardiac catheterization indicated moderate precapillary pulmonary hypertension (PH) with a mean pulmonary artery pressure (PAPm) of 24 mm Hg and pulmonary vascular resistance (PVR) of 470 dyn.s.cm-5 at rest. All known causes having been excluded, the PH was classified as idiopathic. TREATMENT AND COURSE: Evidence of acute pulmonary vascular reactivity was obtained with nitric oxide (NO) inhalation and oral diltiazem, a calcium-channel blocker. The latter, at a dosage of 3 x 120 mg daily, had after 13 days achieved a persisting reduction of PVR at rest and a reduction in PAP rise during exercise. CONCLUSION: After exclusion of other causes, the acute right heart failure was found to be due to primary pulmonary hypertension. The therapeutic efficacity of diltiazem as a vasodilator can be predicted from the response to inhaled NO.

Adult↗

Early cardiorespiratory patterns in severely burned patients with concomitant inhalation injury.

Hemodynamic and oxygenation parameters were determined during the first 24 h in 13 burned patients with concomitant inhalation injury (burn surface area 40-60 percent). In all patients right ventricular function was severely compromised evidenced as a significant increase in end-diastolic volumes, decrease in ejection fractions, low stroke work indices and increased pulmonary vascular resistances. Inotropic support with dobutamine and careful titration of volume infusion according to end-diastolic volume indices improved the hemodynamics as demonstrated by significant increases in right ventricular ejection fractions in all patients without any changes in mean arterial pressures, urine output and oxygenation. Assessment of ventricular performance by a specially designed pulmonary artery catheter is helpful in the management of severely burned patients with concomitant inhalation injury.

Adult↗

Echocardiographic evaluation of spontaneous recovery of right ventricular systolic and diastolic function in patients with acute right ventricular infarction associated with posterior wall left ventricular infarction.

We evaluated right ventricular (RV) systolic and diastolic function in 30 patients with acute RV myocardial infarction on echocardiography. Systolic and diastolic function were impaired early in the setting of RV myocardial infarction, but improved significantly at 3 months.

Adult↗

Failure of stroke volume augmentation during exercise and dobutamine stress is unrelated to load-independent indexes of right ventricular performance after the Mustard operation.

BACKGROUND: Impaired right ventricular function has been implicated as a cause of reduced maximal exercise capacity after the Mustard operation for transposition of the great arteries. METHODS AND RESULTS: Fourteen asymptomatic survivors of the Mustard operation were studied. Each underwent conventional cardiac catheterization, and after satisfactory hemodynamics were confirmed, load-independent indexes of ventricular function were derived by conductance catheter during dobutamine infusion (0, 5, and 10 microg x kg(-1) x min(-1)). Seven patients also underwent upright exercise testing on a bicycle ergometer with analysis of respiratory gas exchange by continuous mass spectrometry. Accessible pulmonary blood flow was measured at each workload with an automated acetylene rebreathing technique. All patients exercised to a satisfactory end point (respiratory quotient >1.1). Maximum oxygen consumption during exercise was impaired compared with predicted values (mean, 77%; P:<0.02). Both exercise and dobutamine infusion were associated with an increase in cardiac index and heart rate and a reduced stroke volume index response. This was despite significantly improved indexes of myocardial contraction (end-systolic pressure volume relation, P:<0.001), preload recruitable stroke work index (P:<0.01), VA coupling (P:<0.001), and isovolumic relaxation (P:<0.001) during dobutamine infusion. There were no changes observed in end-diastolic pressure-volume relations, but there was failure to augment ventricular filling manifest by absence of change in dV/dt (P:=NS). CONCLUSIONS: The stroke volume response to exercise stress is reduced in patients after the Mustard operation. A similar failure to augment stroke volume occurs during dobutamine stress despite appropriate responses in load-independent indexes of contraction and relaxation. This is due to failure to augment right ventricular filling rates during tachycardia, presumably as a result of impaired AV transport, consequent to the abnormal intra-atrial pathways.

Cardiac Surgical Procedures↗

[Cardiovascular system in subjects who have previously suffered from hemorrhagic fever with renal syndrome].

AIM: To study cardiovascular system in convalescence of hemorrhagic fever with renal syndrome (HFRS). MATERIALS AND METHODS: ECG was made in 112 patients who had HFRS 1-3, 4-12 and 12 months ago (groups 1, 2 and 3, respectively). Ultrasound assessed hemodynamics in 27 patients. RESULTS: In HFRS patients the electric axis right deviation was observed statistically more frequently (45%, 24%, 28.6%, 5% of cases of groups 1, 2, 3 and control). 20% of group 1 patients had overloading of right heart. Ultrasound detected dilation of the right ventricle and decrease of blood flow velocity in the pulmonary artery. CONCLUSION: Patients after HFRS may have changes in the lesser circulation which emerge as a rule in early convalescence.

Adolescent↗

[Arrhythmogenic right ventricular disease].

In patients with arrhythmogenic right ventricular disease (ARVD), life threatening ventricular tachyarrhythmias and sudden cardiac death mostly occur in adolescence, or in young adults before the age of 40. In the right ventricle, progressive fibrolipomatous replacement of the ventricular myocardium is pathognomonic. In severe cases progressive congestive right heart failure can develop although mild forms are extremely difficult to recognized. In most cases the disease can be diagnosed only by elaborate investigation. Right ventricular cineangiography, myocardial biopsy, MRI, and electrophysiological investigation are the most important diagnostic procedures. If the disease is diagnosed in an early stage the risk of life-threatening arrhythmias can be reduced by carefully selected antiarrhythmic therapy.

Adolescent↗

Neurohormonal activation in patients with right ventricular failure from pulmonary hypertension: relation to hemodynamic variables and endothelin levels.

OBJECTIVES: This study sought to determine whether neurohormonal activation occurs in isolated right heart failure. BACKGROUND: Neurohormonal activation appears to parallel the severity of left heart failure, but little is known about its role in right heart failure. METHODS: We evaluated neurohormonal activation and endothelin levels in 21 patients with primary pulmonary hypertension at the time of right heart catheterization. RESULTS: Plasma norepinephrine levels correlated significantly with pulmonary artery pressure (r = 0.66, p < 0.01), cardiac index (r = -0.56, p < 0.01) and pulmonary vascular resistance (r = 0.69, p < 0.001). Atrial natriuretic peptide levels were higher in the pulmonary artery than the right atrium and femoral artery and correlated closely with pulmonary artery oxygen saturation (r = -0.73, p < 0.0001). Plasma renin levels were not elevated. Endothelin levels were increased and correlated with right atrial pressure (r = 0.74, p < 0.0001) and pulmonary artery oxygen saturation (r = -0.070, p < 0.0004). CONCLUSIONS: Neurohormonal activation occurs in patients with isolated right ventricular failure and inherently normal left ventricles and appears to be related to the overall severity of cardiopulmonary derangements. The elevation in endothelin levels is consistent with its release in response to pulmonary hypertension.

Adolescent↗

Transoesophageal echocardiography for definitive diagnosis of haemodynamically significant pulmonary embolism.

Transesophageal echocardiographic evaluation of right and left pulmonary arteries, up to the origin of their lobar branches, was prospectively performed with a single plane probe in 32 consecutive patients (18 M, 14 F, aged 55.5 +/- 14.6, from 32 to 80 years) with clinical or echocardiographic suspicion of pulmonary embolism, who met transthoracic echocardiographic criteria of right ventricular overload. Transoesophageal echocardiography showed unequivocal (20 patients) or suspected (three patients) intraluminar thrombi in 88.5% of 26 patients with haemodynamically significant acute or chronic pulmonary embolism, confirmed with reference methods. The sensitivity of the unequivocal transoesophageal echocardiographic diagnosis was 80% for acute and 73% for chronic haemodynamically significant pulmonary embolism. No false-positive results were found (specificity 100%). Additionally, in three cases, transoesophageal echocardiography disclosed the cause of the right ventricular overload revealing a previously undiagnosed atrial septal defect or Ebstein anomaly. Direct visualization of proximal pulmonary arterial thrombi by transoesophageal echocardiography emerges as a useful new method of prompt and definite diagnosis of haemodynamically important pulmonary embolism.

Adult↗

Determining optimal cardiac preload during resuscitation using measurements of ventricular compliance.

BACKGROUND: While the right ventricular end-diastolic volume index (RVEDVI) has been shown to be a better indicator of preload than cardiac filling pressures, optimal values during resuscitation from trauma are unknown. This study examines right ventricular stiffness as a guide to optimal values of RVEDVI. METHODS: Prospective study of 19 critically injured patients monitored with a volumetric pulmonary artery catheter during resuscitation. Per resuscitation protocol, the target RVEDVI was > or = 120 mL/m2. Sequential fluid boluses of 500 to 1000 mL were administered to obtain at least four values of RVEDVI and right ventricular end-diastolic pressure (estimated by central venous pressure [CVP]). For each patient, nonlinear regression was used to construct the ventricular compliance curve based on the equation, CVP = aek(RVEDVI), where k is the coefficient of chamber stiffness. RESULTS: Overall, the derived compliance curves had excellent fit with the theoretical equation (mean R2, 0.95 +/- 0.04). Mean k was 0.043 +/- 0.012 (range, 0.029-0.067). For each patient, mean RVEDVI during resuscitation was significantly correlated with k (R2 = 0.75, p < 10-5) indicating that chamber stiffness, measured during initial fluid administration, may be used to determine RVEDVI during the ensuing resuscitation. CONCLUSION: In critically injured patients, bedside assessment of right ventricular compliance is possible and may help determine optimal values of RVEDVI during resuscitation.

Adult↗

Sudden death in childhood due to right ventricular dysplasia: report of two cases.

Two cases of sudden death due to arrhythmogenic right ventricular dysplasia (ARVD) occurring in the pediatric age group are described. One of the subjects, at the age of 7 years, is believed to be the youngest child in whom ARVD has been diagnosed at autopsy. The clinical and pathological characteristics of ARVD are discussed.

Adolescent↗

Age related dilatation of the right ventricle in arrhythmogenic right ventricular dysplasia-cardiomyopathy.

'Progression' in typical arrhythmogenic right ventricular dysplasia cardiomyopathy could be mainly demonstrated in case reports and generally implies decreasing left ventricular function; systematic follow-up data are only available on clinical features such as recurrent arrhythmias, late potentials or left ventricular ejection fraction. Due to methodological problems, continuous data on structural and functional right ventricular performance are not available. In order to get some more information about basic mechanisms of possible progression in arrhythmogenic right ventricular dysplasia-cardiomyopathy, the age-dependency of right ventricular enddiastolic volume, right ventricular ejection fraction, number of bulges, left ventricular involvement and the form of tachycardias was analysed at a single examination after having made the diagnosis of arrhythmogenic right ventricular dysplasia-cardiomyopathy. In the whole cohort of patients (n = 66), age-dependency of right ventricular volume could be demonstrated using linear regression analysis, chi-square-test and paired t-test (P < 0.01); global right ventricular ejection fraction and the number of right ventricular bulges did not differ significantly in younger and older patients. The same was true for mild left ventricular impairment and sustained or non-sustained ventricular tachycardias, respectively. In a follow-up, two older patients developed dramatic left ventricular dilatation and reduction of left ventricular function requiring heart transplantation. A third older patient died from isolated right heart failure. Increasing age-related right ventricular dilatation is possibly a marker of disease progression as a basis for right heart failure. Dramatic left heart involvement seems to be an event with abrupt onset in most cases due to acute or chronic myocarditis.

Adolescent↗