[Constrictive pericarditis - new methods in the diagnosis of an old disease: a case report]. / Pericardite constritiva - novos métodos no diagnóstico de uma velha doença: a propósito de um caso clínico.

Constrictive pericarditis is a rare clinical entity that can pose diagnostic problems. The gold standard for diagnosis is cardiac catheterization with analysis of intracavitary pressure curves, which are high and, in end-diastole, equal in all chambers. The diastolic profile in both ventricles presents the classic dip-and-plateau pattern and the difference between the diastolic pressures of both ventricles should not exceed 3-5mmHg. Unfortunately, these traditional criteria are not always present and in fact the sensitivity and specificity of equalization of diastolic pressures are relatively low and of limited value in individual patients. This highlights the need to use new cardiac imaging techniques to resolve any doubts. The case described here is a good example.

Thrombosed aneurysm of the left sinus of valsalva presenting as an intramyocardial mass.

Aneurysms of the sinuses of Valsalva are uncommon in clinical practice. Most are congenital, but secondary causes are also recognized. Congenital aneurysms of the left sinus of Valsalva are particularly rare. The authors report a fatal case in which a nonruptured aneurysm of the left sinus of Valsalva dissected into the interventricular septum and presented as heart failure. The concurrent presence of dilated cardiomyopathy and the mechanisms that may have led to it are discussed on the basis of the anatomic and histologic features found at autopsy.

Behind heart failure syndrome: remember AL amyloidosis. Two case-reports.

Amyloidosis is a systemic disease that is a consequence of extracellular deposition of insoluble fibrils composed of subunits of low molecular weight (5-25 kD) derived from a variety of plasma proteins. Identification of the amyloidogenic protein determines the type of amyloidosis. In primary systemic amyloidosis (classically called AL amyloidosis), the amyloid protein is composed of light chains resulting from plasma-cell dyscrasia. Cardiac manifestations are the most common clinical presentation of this type of amyloidosis, occurring in 50% of patients. The authors describe two cases in which hospitalization was due to decompensated heart failure, which were similar in their etiology (multiple myeloma/amyloid cardiomyopathy) and evolution (sudden death). The authors wish to draw attention to an entity that is rarely encountered in clinical practice and that requires a high index of suspicion.

Pharmacogenetics and anticoagulant therapy: two cases of genetically determined response to warfarin.

Inter- and intra-individual variability of response to warfarin means that its anticoagulant effect must be monitored, given the risk of thromboembolic complications and bleeding. This variability is influenced by gender, age, body mass index, smoking, diet, comorbid conditions, drug interactions and genetic factors. Pharmacogenetics refers to the study of genetic background to predict drug response, effectiveness and risk of adverse effects in a given patient. The authors illustrate its relevance in two case reports. A 40-year-old woman admitted for massive pulmonary thromboembolism underwent anticoagulant and fibrinolytic therapy, following which warfarin was needed in unusually high doses to achieve effective anticoagulation. The genetic variants c.430CC and c.1075AA of the CYP2C9 gene were identified, predisposing to rapid warfarin metabolism, as well as the c.-1639GG variant of the VKORC1 gene, associated with low sensitivity to the drug. Together, these variants give high resistance to warfarin. In the second case, a 76-year-old man with permanent atrial fibrillation developed excessive prolongation of prothrombin time after being treated with 5 mg/day warfarin for 5 days. The genetic variants c.430CC and c.1075AC of the CYP2C9 gene and 1639AA of the VKORC1 gene were identified. Together, these polymorphisms confer high sensitivity to warfarin, necessitating smaller doses to maintain therapeutic anticoagulation levels. The authors review the relevance of the study of genetic polymorphisms related to anticoagulant therapy and discuss its potential usefulness in clinical practice.

Acquired left ventricular-to-right atrial shunt (Gerbode defect) due to infective endocarditis.

Left ventricular-to-right atrial communications are a rare type of ventricular septal defect, known as the Gerbode defect. They are usually congenital, but rare cases have been described secondary to bacterial endocarditis. The authors present a rare case of Gerbode defect and severe pericardial effusion secondary to Staphylococcus aureus endocarditis, in a patient with alcoholic liver cirrhosis.

Routine transthoracic echocardiography in a general Intensive Care Unit: an 18 month survey in 704 patients.

The authors analyzed 704 transthoracic echocardiographic (TTE) examinations, performed routinely to all admitted patients to a general 16-bed Intensive Care Unit (ICU) during an 18-month period. Data acquisition and prevalence of abnormalities of cardiac structures and function were assessed, as well as the new, previously unknown severe diagnoses. A TTE was performed within the first 24 h of admission on 704 consecutive patients, with a mean age of 61.5+/-17.5 years, ICU stay of 10.6+/-17.1 days, APACHE II 22.6+/-8.9, and SAPS II 52.7+/-20.4. In four patients, TTE could not be performed. Left ventricular (LV) dimensions were quantified in 689 (97.8%) patients, and LV function in 670 (95.2%) patients. Cardiac output (CO) was determined in 610 (86.7%), and mitral E/A in 399 (85.9% of patients in sinus rhythm). Echocardiographic abnormalities were detected in 234 (33%) patients, the most common being left atrial (LA) enlargement (n=163), and LV dysfunction (n=132). Patients with these alterations were older (66+/-16.5 vs 58.1+/-17.4, p<0.001), presented a higher APACHE II score (24.4+/-8.7 vs 21.1+/-8.9, p<0.001), and had a higher mortality rate (40.1% vs 25.4%, p<0.001). Severe, previously unknown echocardiographic diagnoses were detected in 53 (7.5%) patients; the most frequent condition was severe LV dysfunction. Through a multivariate logistic regression analysis, it was determined that mortality was affected by tricuspid regurgitation (p=0.016, CI 1.007-1.016) and ICU stay (p<0.001, CI 1-1.019). We conclude that TTE can detect most cardiac structures in a general ICU. One-third of the patients studied presented cardiac structural or functional alterations and 7.5% severe previously unknown diagnoses.

Abnormal myocardial flow reserve in sickle cell disease: a myocardial contrast echocardiography study.

BACKGROUND: Sickle cell disease (SCD) is characterized by obstruction of microvessels leading to ischemia and necrosis. We have aimed to demonstrate whether myocardial contrast echocardiography (MCE) is able to detect myocardial perfusion abnormalities in SCD patients and to assess their relationship with left ventricle (LV) perfusion and systolic function. METHODS: A group of 25 patients with SCD and a control group of 19 normal individuals were studied. Using MCE, myocardial perfusion reserve indices (A, beta, and A x beta) were obtained, before and after hyperemia with dypiridamole. LV function was also analyzed: ejection fraction (EF), index of myocardial performance (IMP), the ratio of transmitral early-diastolic flow velocity E and the pulsed tissue Doppler mitral annular early diastolic velocity Ea (E/Ea) (E/Ea), tissue Doppler mitral annular peak systolic velocity (Sa), and peak systolic strain (S) were obtained. RESULTS: Myocardial velocity (beta) and myocardial blood flow (A x beta) reserves were lower in the patients than in controls (1.7 +/- 0.4 vs. 3.3 +/- 0.2, P = 0.000 and 2.1 +/- 0.6 vs. 4.1 +/- 0.2, P = 0.000, respectively). In SCD patients, a correlation was found between beta reserve and EF, IMP, Sa, E/Ea, and S% and between A x beta reserve and Sa. CONCLUSIONS: MCE detected abnormal perfusion reserve in patients with SCD, which correlated with systolic function indices. This suggests that perfusion plays a role in SCD ventricular dysfunction.

Impact of aortic dimensions and pulse pressure on late aneurysm formation in operated type A aortic dissection. A magnetic resonance imaging study.

BACKGROUND: Patients operated on for type A aortic dissection remain at risk of long-term aneurysm development, the main cause for late death. The aim of this study was to identify early predictors for aneurysm formation at three-years after surgery. METHODS: A study group of 70 consecutive patients (52 +/- 10 years-old, 41 male), operated on for aortic dissection with replacement of the ascending segment, was evaluated prospectively for three-years. In order to detect aneurysm formation, the dimension of residual distal aortic segments was obtained soon after surgery and then annually for three years using cardiovascular magnetic resonance. RESULTS: During follow-up (38 +/- 2.6 months), aneurysm was found in 25 patients (35%) involving residual segments. Larger initial dimension of segments, higher pulse pressure, lower distensibility of residual segments and the presence of a residual flap were univariately associated with aneurysm. Multivariate analysis identified the initial dimension (mm) of the descending thoracic aorta (OR 1.47, 95%CI: [1.19-1.82]) and pulse pressure (OR 1.43,95%CI: [1.10-1.86]) as independent variables for aneurysm formation. A risk score using pulse pressure and descending thoracic dimension was constructed. Patients with or=45 yielded 100% of aneurysm frequency. CONCLUSIONS: Type A aortic dissection treated by graft interposition is associated with a high risk of aneurysm formation. Early post-operative pulse pressure and the descending thoracic aortic dimension were independent variables and seem to be the main predictors for the outcome.

Heart rate and blood pressure in mitral valve prolapse patients: divergent effects of long-term propranolol therapy and correlations with catecholamines.

OBJECTIVE: There is a well known association between mitral valve prolapse (MVP) and low blood pressure (BP), although patients often have high levels of catecholamines and high heart rate (HR). The main objective of our study was to evaluate the effects of long-term adrenergic beta-blockade on these parameters. METHODS: The study population consisted of 46 patients with MVP and the control group consisted of 20 normal individuals. The study had two phases: in the first phase, patients were free of medications. In the second phase, patients were under treatment with propranolol for 10 to 12 months. The tests were performed in normal individuals and patients in the first phase. Only patients underwent the same tests in the second phase. Measurement of urinary epinephrine and norepinephrine levels, by high performance liquid chromatography, was done. Rest HR was determined by electrocardiogram (ECG), and ambulatory blood pressure and HR were evaluated by 24 hours ambulatory blood pressure monitoring (ABPM) using the auscultatory method. RESULTS: The levels of epinephrine and norepinephrine were significantly higher in patients than in normal controls and decreased under propranolol. Rest and ambulatory HR were higher in patients and decreased under propranolol. The 24 hours systolic and diastolic BPs were lower in patients, and their values increased under propranolol. Heart rate decreasing and epinephrine levels reduction were positively correlated. No correlation was found between BP increase and catecholamine levels. CONCLUSION: The study results show divergent effects of propranolol on blood pressure, which increased, and on heart rate, that decreased, in patients with MVP. Heart rate decrease was an expected result and depends, namely, on b1 receptors blockade. Increase in BP is an unusual response to adrenergic beta-blockade in normal conditions, and this finding supports the preponderance of b2 receptors on the BP control in patients with MVP.

Diastolic function in several stages of chronic kidney disease in patients with autosomal dominant polycystic kidney disease: a tissue Doppler imaging study.

BACKGROUND: This study evaluates the prevalence of diastolic dysfunction (DD) in several stages of chronic kidney disease (CKD) in patients with autosomal dominant polycystic kidney disease (ADPKD). METHODS: 107 ADPKD patients performed echocardiographic and Doppler studies and a tissue Doppler imaging (TDI) study. Patients were divided in three groups: group 1, 57 patients with CKD stage I, group 2, 37 patients in stages II and III, and group 3, 13 patients with CKD stages IV and V (not on dialysis). RESULTS: In transmitral Doppler, 1 patient in group 1 compared to 5 in group 2, and 4 in group 3 exhibited DD (p < 0.005); moreover, E/A ratio decreases progressively from group 1 to 3 (p < 0.0001). In TDI, DD was observed in 8 patients in group 1, 17 in group 2, and 8 in group 3 had DD (p < 0.001). Em velocity, the best TDI parameter for DD, correlated with age, renal function and blood pressure. When adjusted for age, increased left ventricular mass index and decreased renal function were independent risk factors of DD. CONCLUSIONS: DD occurred progressively as renal function deteriorates in patients with ADPKD and this effect is independently related to age and blood pressure.

Ambulatory blood pressure measurement in young normotensive patients with autosomal dominant polycystic kidney disease.

INTRODUCTION: Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic disorder causing chronic kidney disease in adults. Hypertension occurs early and frequently precedes the development of renal failure. It has been shown that clinically normotensive young adults with ADPKD exhibit increased left ventricular mass and left ventricular mass index (LVMI), which contributes to the increased cardiovascular risk in these patients. We set out to investigate whether normotensive patients have a prehypertensive state that could account for their increased LVMI. METHODS: Patients with ADPKD followed as outpatients were selected if they were aged between 21-30 years, were normotensive (office and sporadic blood pressure < 140/90 without medication), and had normal renal function (GFR > 90 ml/min). Normotensive controls aged between 21-30 years were selected, all with normal renal ultrasound, serum creatinine, dipstick analysis and microalbuminuria /creatinine ratio. Patients and controls underwent 24-hour ambulatory blood pressure measurement (ABPM) according to the local protocol. RESULTS: Systolic (124.7 +/- 7.6 vs. 115.2 +/- 6.9; p < 0.0001), diastolic (77.3 +/- 6.3 vs. 70.5 +/- 3.9; p < 0.0001) and mean (92.7 +/- 8.5 vs. 85.7 +/- < 0.001) 24-hour blood pressure was significantly higher in patients with ADPKD compared to controls. Statistically significant differences were also found when daytime and night-time periods were analyzed separately. Hypertension on ABPM was diagnosed in 6 patients but differences in the ABPM profile persisted even when these patients were excluded from the analysis. CONCLUSION: In young adults with ADPKD there is a prehypertensive state that can be detected using ABPM.

Detection of preclinical left ventricular dysfunction in Fabry disease: the contribution of tissue Doppler.

INTRODUCTION: Fabry disease is a rare X-linked lysosomal storage disorder caused by a deficiency of alpha-galactosidase, which results in progressive intracellular accumulation of glycosphingolipids in various tissues. Cardiac involvement is frequent, with left ventricular (LV) hypertrophy (concentric, apical or asymmetric septal) as the most common finding. Evaluating LV systolic dysfunction with conventional echocardiography is not sufficiently sensitive to detect impaired myocardial function early in the course of the disease. Doppler myocardial imaging can quantify changes in global and regional longitudinal myocardial function with great precision. AIM: To identify parameters of cardiac dysfunction in patients with Fabry disease with no cardiac symptoms using conventional or Doppler echocardiography and tissue Doppler (including tissue tracking, strain and strain rate). METHODS: Four patients with Fabry disease (3 female; mean age 47 +/- 17 years) and 29 healthy controls (19 female and 10 male; mean age 38 +/- 14 years) were studied with conventional echocardiography and tissue Doppler imaging using a Vivid 7 scanner. The following parameters were measured: LV dimensions, ejection fraction (using Simpson's rule), systolic and diastolic velocities and tissue tracking of the mitral annulus at six sites (apical views). LV peak systolic strain and strain rate were obtained in 12 segments from apical views. RESULTS: Two patients had LV hypertrophy (one concentric and one apical). Diastolic impairment was detected in three patients by reduced flow propagation velocity of early transmitral flow (Vp) and E/Vp ratio. Mitral annulus systolic velocities were lower in Fabry disease than in the controls. Peak systolic strain and strain rate were diminished in all segments in three patients, showing impaired myocardial systolic function. The results are presented for each of the four patients. CONCLUSIONS: In patients with Fabry disease, with no cardiac symptoms and normal LV systolic function on conventional echocardiography, diastolic dysfunction was detected by Vp and E/Vp ratio regardless of LV hypertrophy. However, tissue Doppler imaging was able to detect impaired myocardial systolic function, particularly in patients with LV hypertrophy.

[Transthoracic echocardiography for evaluation of hypotensive critically ill patient]. / A ecocardiografia transtorácica na avaliação da hipotensão em cuidados intensivos polivalentes.

OBJECTIVES AND DESIGN: a prospective study to determine the value of transthoracic echocardiography (TTE) in assessing hypotensive patients in a medical/surgical Intensive care Unit (ICU). SETTING: a 16-bed medical/surgical ICU. MATERIAL AND METHODS: patients with hypotension were studied (systolic arterial pressure < 90 mmHg or mean arterial pressure < 60 mmHg, non responsive to fluid therapy in a 30-minute period). TTE was aimed to: exclude severe cardiac dysfunction(abnormalities requiring immediate therapeutic decision); to evaluate other non severe cardiac alterations; to determine cardiac index (CI) and analyse inferior vena cava (IVC). RESULTS: Two hundred eight patients were studied, and 198 enrolled (4.5% of impossible examinations), with a mean age 63.4 +/- 16.2 years, 129 male, APACHE II score 30.1 +/- 9.9, SAPS II 68.8 +/- 20.5, SOFA 11.6 +/- 3.8, MODS 10.9 +/- 3.9. Mortality was 51% (n=101) and 168 (82.2%) patients were under mechanical ventilation. Forty four patients (44.4%) presented cardiac abnormalities, 28 of whom (14%) severe cardiac diseases. Of these, 18 (9%) presented unsuspected situations (aortic stenosis, 3; endocarditis, 4, dilated miocardiopathy, 9, cardiac tamponade, 2). Patients with cardiac abnormalities were older and presented higher severity scores and mortality. Most patients (158, 79.7%) presented a normal/high IC, all with low peripheral vascular resistance. Through logistic regression analysis, a statistically significant between IVC index and ICU stay (p=0.05); IC and IVC index correlated with overall mortality (p=0,008 and 0,041 respectively). CONCLUSIONS: Patients with hypotension in a medical/surgical ICU presented a high rate of cardiac abnormalities (44.4%, n=88), including 26 patients with severe diseases, requiring immediate therapeutic decisions. IC and IVC analysis may be useful to determine hemodynamic profile and several TTE parameters may have prognostic value.

[Non invasive assessment of pulmonary capillary wedge pressure using echocardiography]. / Determinação não invasiva da pressão de encravamento da artéria pulmonar por ecocardiografia.

Pulmonary capillary wedge pressure (Pw) is an important hemodynamic parameter frequently used in Intensive Care. Much effort was taken by investigators to noninvasively obtain this parameter particularly using echocardiography. The authors reviewed the published works in this field. There are a great number of correlations between echocardiographic variables and Pw, including equations for its precise assessment. Changes in echocardiographic parameters due to modifications in volume status and more recently tissue Doppler evaluation are also related fields. Despite the quality and quantity of investigation, no single method was established or accepted for non-invasive assessment of Pw using echocardiography. The major problems are related to the study of different populations regarding its basic clinical condition and to the complexity of equations described. Non-invasive assessment of Pw in clinical setting using echocardiography is still a hard task for this technique. Probably, the use of echocardiography as a non-invasive tool for hemodynamic assessment should use other methodologies, and not rely on Pw determination.

Haemodynamic parameters obtained by transthoracic echocardiography and Swan-Ganz catheter: a comparative study in liver transplant patients.

OBJECTIVE: To compare the haemodynamic measurements of cardiac output (CO), central venous pressure (CVP), pulmonary capillary wedge pressure (Pw) and pulmonary artery systolic pressure (PASP), obtained by Swan-Ganz catheter and transthoracic echocardiography. MATERIAL AND METHODS: Prospective study in a Medical/Surgical Intensive Care Unit (ICU). A total of 41 post liver transplant patients were enrolled. CO, CVP, Pw and PASP, were simultaneously determined by two independent observers, utilizing a Swan-Ganz catheter and transthoracic echocardiography, using equations described in the literature. A linear correlation and a Bland-Altman analysis were performed. RESULTS: A good correlation between invasive and non- invasive measurements for CO (r=0.97) and CVP (r=0.88) was found, but determinations of Pw (r=0.41) and PASP (r=0.18) did not correlate well. Bias and 95% confidence interval for CO were negligible namely when a CO<6 l/min was considered. Pulsed-wave Doppler-echocardiography underestimates the CO when compared with thermodilution, but the 2 techniques agree on average and can be used interchangeably. CONCLUSIONS: The non-invasive determination of CO in critical care post liver transplant patients correlates well with the invasive determinations. Transthoracic echocardiography was not appropriate for calculating filling parameters studied. Although the data was obtained in post liver transplant patients, they could be useful in defining the role of echocardiography in the ICU.

Cardiac Doppler variation with volume status changes in general intensive care.

The authors studied the effect of volume status modification on cardiac Doppler features, with negative fluid balance and corresponding central venous pressure change. This was carried out in 64 patients admitted to the Intensive Care Unit, 24 of whom were under mechanical ventilation. With volume status change, the mitral E/A ratio showed a tendency to decrease, mitral E wave deceleration time decreased, isovolumic relaxation time increased, and the expiratory diameter of the inferior vena cava reduced and its inspiratory collapse increased. No significant correlation was observed between the parameters studied and volume changes, or between central venous pressure and fluid balance. Volume changes in critical care patients modify certain features of Doppler echocardiography, but the magnitude of such variations is unpredictable.

Pulmonary artery catheter--what role in the 21st century?

The pulmonary artery catheter is a diagnostic tool used routinely in cardiological and general intensive care. Since its implementation in the 1970s, serious doubts have been raised concerning its use and efficacy. These problems are a legacy of the 20th century, and may not be solved in this century, but they cannot be ignored. The authors present an overview of this subject, recording in chronological order the principal events and publications concerning the pulmonary artery catheter, highlighting some controversies, most of them unresolved.

Reconstruçäo tridimensional dinâmica do coraçäo através da ecocardiografia transesofágica / Dynamic three-dimensional reconstruction of the hearth by transesophageal echocardiography

Objective - To evaluate echocardiography accuracy in performing and obtaining for dynamical three-dimensional (3D) reconstruction. Methods - Three-dimensional (3D) image reconstruction was obtained in 20 consecutive patients who underwent transesophageal echocardiography. A multiplanar 5 Mhz-transducer was used 3D reconstruction. Results - Twenty patients were studied consecutively. The following cardiac diseases were present: valvar prostheses - 6(2 mitral, 2 aortic and 2 mitral and aortic); mitral valve prolapse - 3; mitral and aortic disease - 2; aortic valve disease - 5; congenital heart disease - 3 (2 atrial septal defect - ASD-and 1 transposition of the great arteries - TGA); arteriovenous fistula - 1. In 7 patients, color Doppler was also obtained and used for 3D flow reconstruction. Twenty five cardiac structures were acquired and 60 reconstructions generated (28 of mitral valves, 14 of aortic valves, 4 of mitral prostheses, 7 of aortic prostheses and 7 of the ASD). Fifty five of 60 (91.6 per cent) reconstructions were considered of good quality by 2 independent observers. The 11 reconstructed mitral valves/prostheses and the 2 reconstructed ASDs provided more anatomical information than two dimensional echocardiography (2DE) alone. Conclusion - 3D echocardiography using a transesophageal transducer is a feasible technique, which improves detection of anatomical details of cardiac structures, particulary of the mitral valve and atrial septum.