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PubMed1997

Sudden unexpected death in young adult due to right ventricular dysplasia.

This case report illustrates a rare familial cardiomyopathy first reported in the medical literature in 1982 known as right ventricular dysplasia (right ventricular cardiomyopathy). The patient is a young woman with a history of cardiac arrhythmias suspected to be associated with prolapsed mitral valve who presented to the Berks Country Coroner's office as a sudden unexpected death in a young adult. It is important to recognize the illustrated classic cardiac pathology of this rare entity for clinical management, as an anatomic explanation of cause of sudden death and for the accumulation of statistics to establish frequency, conditions of predisposition, response to therapy and predicted outcome.

Adult↗

PubMed2004

Hemodynamic effects of inhaled nitric oxide in right ventricular myocardial infarction and cardiogenic shock.

OBJECTIVES: We sought to determine whether or not inhaled nitric oxide (NO) could improve hemodynamic function in patients with right ventricular myocardial infarction (RVMI) and cardiogenic shock (CS). BACKGROUND: Inhaled NO is a selective pulmonary vasodilator that can decrease right ventricular afterload. METHODS: Thirteen patients (7 males and 6 females, age 65 +/- 3 years) presenting with electrocardiographic, echocardiographic, and hemodynamic evidence of acute inferior myocardial infarction associated with RVMI and CS were studied. After administration of supplemental oxygen (inspired oxygen fraction [F(i)O(2)] = 1.0), hemodynamic measurements were recorded before, during inhalation of NO (80 ppm at F(i)O(2) = 0.90) for 10 min, and 10 min after NO inhalation was discontinued (F(i)O(2) = 1.0). RESULTS: Breathing NO decreased the mean right atrial pressure by 12 +/- 3%, mean pulmonary arterial pressure by 13 +/- 2%, and pulmonary vascular resistance by 36 +/- 8% (all p < 0.05). Nitric oxide inhalation increased the cardiac index by 24 +/- 11% and the stroke volume index by 23 +/- 12% (p < 0.05). The NO administration did not change systemic arterial or pulmonary capillary wedge pressures. Contrast echocardiography identified three patients with a patent foramen ovale and right-to-left shunt flow while breathing at F(i)O(2) = 1.0. Breathing NO decreased shunt flow by 56 +/- 5% (p < 0.05) and was associated with markedly improved systemic oxygen saturation. CONCLUSIONS: Nitric oxide inhalation results in acute hemodynamic improvement when administered to patients with RVMI and CS.

Administration, Inhalation↗

PubMed2004

Tricuspid aseptic endocarditis revealing right endomyocardial fibrosis during an unrecognized Behçet's disease. A case report.

INTRODUCTION: Aseptic endocarditis or/and endomyocardial fibrosis are rarely reported in Behçet's disease. OBSERVATION: We report on a case of a 21-year-old man living in Algeria, revealed by verrucous tricuspid valvulitis extending to the ventricular endomyocardium and complicated with right heart failure, initially misdiagnosed and treated as infective endocarditis occurring on rheumatic cardiac after-effects. DISCUSSION; We discuss the lack of specificity of Jones criteria and emphasize the need to include cardiac involvement in Behçet's disease in the differential diagnosis of rheumatic fever carditis. This message is notably important in some countries where the prevalence of these two entities are among the highest in the world.

Adult↗

PubMed2000

[Circulatory assistance while waiting for heart transplantation. Clinical course].

OUTCOME: In most cases, mechanical circulatory assistance prior to transplantation can restore the main vital functions allowing a progressive physical rehabilitation during the period of assistance. According to several international registries, the transplantation rate ranges from 62% to 69% and the proportion of transplanted patients who had circulatory assistance and who were discharged ranges from 65% to 69%. COMPLICATIONS: Bleeding (42.5%), right heart failure (20-25%), air embolism and multiple organ failure are the main causes of early morbidity and mortality. Infection (28.5%), thromboembolic events and technical failures are the most frequent late complications. DURATION OF CIRCULATORY ASSISTANCE: The optimal time for transplantation is the moment when the incidence of complications and technical problems for transplantation are at their minima while the patient's vital functions are at their maxima.

Assisted Circulation↗

PubMed2000

Diastolic ventricular interaction in normal and dilated heart during head-up tilting.

BACKGROUND: The normal human heart behaves as a single functional unit during preload reduction; adaptations of the left ventricle to head-up tilting is mediated through ventricular interdependence and biventricular-lung interaction. HYPOTHESIS: We hypothesized that reduction of venous return in dilated cardiomyopathy is likely to have a great effect on ventricular chamber geometry and filling. The aim of this study was to evaluate the effects of gradual head-up tilting in normal subjects and in patients with dilated cardiomyopathy, addressing special attention to right (RV) and left ventricular (LV) dimensions, geometry, and filling, and to biventricular-lung interaction. METHODS: Twenty normal subjects and 23 patients with moderate heart failure due to dilated cardiomyopathy were studied with two-dimensional and Doppler echocardiography in supine position and after 20 degrees, 40 degrees, and 60 degrees tilting. Right ventricular and LV dimensions, LV geometry, and tricuspid, mitral, and pulmonary venous flow patterns were recorded at each step of the study. Geometric changes of the LV were evaluated by measurements of volumes and diameters in the apical four-chamber view (which identifies the interventricular septum and lateral wall) and apical two-chamber view (which identifies the inferior and anterior wall of the LV). RESULTS: In the two groups, tilting was associated with reduction of RV area and LV diameter and volumes; percent variations in LV diameter and volumes recorded in four-chamber view were lower at each step of tilting than with those derived from the two-chamber view in controls and in patients. In normal subjects, mitral and tricuspid peak early flow velocities were decreased at any tilting level; peak late velocities were unchanged; peak velocity of systolic forward flow of the pulmonary vein was reduced, diastolic forward flow was unchanged, and the difference in duration between reverse pulmonary flow and forward mitral A wave was reduced. Doppler findings were qualitatively similar in patients, but tilting induced a more marked redistribution of LV filling to late diastole because of a significant increase in atrial contribution. CONCLUSIONS: Preload reduction by tilting induces profound effects on left and right dimensions, geometry, and filling in normal and dilated heart; reduction or RV dimensions are associated with changes in LV ventricular geometry (minimal reduction in septal-lateral diameter, marked reduction in anterior-posterior diameter), redistribution of right and left diastolic filling to late diastole, and redistribution of pulmonary venous flow to early diastole. These mechanisms are probably due to a favorable interaction between heart and lungs, which increases compliance within the pericardial space and facilitates redistribution of flow from the lungs. Even a minimal amount of preload reduction causes more marked effects in LV filling patterns in dilated cardiomyopathy than in normal hearts, confirming that ventricular interaction and pericardial constraint are increased when heart volume enlarges.

Adult↗

PubMed2006

Electrocardiographic predictors of long-term outcomes after radiofrequency ablation in patients with right-ventricular outflow tract tachycardia.

AIMS: The objectives of this study were to identify electrocardiographic (ECG) predictors of long-term outcomes after radiofrequency (RF) ablation in patients with right-ventricular outflow tract (RVOT) tachycardia. METHODS AND RESULTS: We correlated ECG characteristics with RF ablation outcomes in 144 patients with RVOT tachycardia who underwent RF ablation for >1 year. Unfavourable RF ablation outcomes were predefined as unsuccessful RF ablation or recurrence of tachycardia requiring repeated ablation. RF ablation was not successful in 11 (7.6%) patients and 16 (12%) patients had arrhythmia recurrence requiring repeated ablation. Average follow-up time was 72.2+/-28.4 months. Selected parameters from univariate analysis included number of RF applications, pacemapping, application of bonus burn, procedure time, monophasic R-wave in lead I, QS pattern in leads I and aVL, QRS duration in leads II and V(2), and right axis deviation, in ventricular tachycardia. From logistic regression analysis, only monophasic R-wave in lead I remained in the final equation (P=0.004, odds ratio 12.9). CONCLUSION: Monophasic R-wave in lead I during RVOT tachycardia is associated with unfavourable outcomes after RF ablation. This finding may help clinicians in the selection of patients for RF ablation and for the prediction of RF ablation outcome.

Adult↗

PubMedDate not supplied

[Inhalation nitric oxide during heart transplantation].

Effect and dose dependence of iNO as a selective vasodilator of the lesser circulation on the central hemodynamics was studied in 24 patients subjected to transplantation of the heart. iNO in concentrations of up to 20 ppm ensured objective evaluation of the reversibility of the pretransplantation pulmonary hypertension. Before transplantation iNO in a concentration higher than 20 ppm led to deterioration of left-ventricular function, associated with a 19% increase in pulmonary artery wedge pressure and a 13% decrease of cardiac index in patients with a less than 15 mm Hg transpulmonary gradient and pulmonary vascular resistance higher than 320 din.sec.cm-5. During left-ventricular bypass, iNO dose-dependently decreased the mean pulmonary artery pressure, transpulmonary gradient, pulmonary vascular resistance, pulmonary to peripheral vascular resistance ratio and the right-ventricular stroke index and increased the left-ventricular assist bypass by 17% in patients waiting for orthotopic heart transplantation. Early after transplantation, iNO in a concentration of 20 ppm notably decreased the pulmonary vascular resistance and stabilized the function of transplanted heart, increasing the cardiac index by 19% and blood pressure by 16%. During diagnostic and therapeutic manipulations at 10-20 ppm iNO the level of NO2 was no higher than 2.5 ppm, or 3 ppm at 30-40 ppm.

Administration, Inhalation↗

PubMed2005

Advances in perioperative pacing.

Cardiac resynchronization therapy with biventricular pacing has become a significant management tool in adults with heart failure. In children, right rather than just left ventricular failure, is a key problem in the postoperative period. Also, congenital heart defects vary widely in their nature and prognosis. There are now preliminary reports in the literature of the use of multiple temporary pacing sites after congenital heart surgery and acute comparison of the effects of unsynchronized versus synchronized pacing in the postoperative period. These studies support the use of cardiac resynchronization pacing at least as a temporary measure in cases of acute heart failure after surgery in patients with congenital heart disease.

Adult↗

PubMed1995

Diagnosis of right ventricular overload by body surface QRST isointegral maps in children with postoperative right bundle branch block.

The utility of body surface QRST isointegral maps (QRST-Imaps) for the detection of right ventricular (RV) overload was examined in children with postoperative development of right bundle branch block. In healthy children with no evidence of bundle branch block (n = 31), the QRST-Imap demonstrated a maximum at the left anterior chest and a minimum near the right shoulder with a single dipole distribution. The positive areas extended from the left anterior chest to the left back, and negative areas extended from the right anterior chest to the right back. Children with complete right bundle branch block but without heart disease demonstrated a QRST-Imap that was similar to that seen in normal children. In patients with RV overload (n = 15; 8 with ventricular septal defect and complicated anomaly and 7 with tetralogy of Fallot), the QRST-Imaps were abnormal and demonstrated double maxima, a rightward shift of the maximum, and extension of positive areas to the right chest. In the 10 patients who developed postoperative complete right bundle branch block, 4 had no evidence of RV overload by hemodynamic or echocardiographic assessment and demonstrated a normal QRST-Imap. In the six children who had residual RV overload during hemodynamic assessment, the QRST-Imap was abnormal. These results suggest that the QRST-Imap is a useful method for the detection of RV overload in pediatric patients complicated with conduction disturbances.

Adolescent↗

PubMed2005

Borderline left ventricle.

The first problem to solve when dealing with the topic 'borderline left ventricle' is to find the appropriate definition. Several parameters have been taken into consideration, either morphometric (diameter of the mitral valve, indexed mitral valve area, left ventricular inflow dimension, left ventricular cross-sectional area, ratio between the apex-to-base left ventricular dimension and right ventricular dimension, left ventricular long axis to heart long axis ratio, left ventricular end diastolic volume, left ventricular mass index, ratio of the right/left ventricular wall thickness, presence of endocardial fibroelastosis, cardiac apex not formed by the left ventricle, diameter of the ventriculo-aortic junction, diameter of the aortic valve annulus and indexed aortic root diameter) as well as functional (left ventricular ejection fraction, left ventricular end diastolic pressure, mean pulmonary artery pressure, direction of the blood flow in the ascending aorta and at the level of the patent ductus arteriosus). Pre-operative determination whether the left ventricle is adequate to sustain the systemic circulation, or it may became adequate with the available surgical approaches, and therefore a bi-ventricular type of repair is feasible, can be extremely difficult, particularly in the presence of a 'borderline left ventricle'. In the clinical practice pediatric cardiologists and cardiac surgeons are faced with the problem of the 'borderline left ventricle' in four different groups of congenital heart defects: (a) aortic valve stenosis, (b) aortic coarctation, with or without hypoplastic aortic arch, (c) hypoplastic left heart complex, (d) right ventricular pressure and/or volume overload. In all the above situations in the presence of a left ventricle smaller than normal a very exhaustive approach has been reviewed in the decision making process, taking in account the literature reports as well as the personal experience. In each patient with 'borderline left ventricle' the elements to be considered for the decision making process between uni- and bi-ventricular type of repair, or for less ideal options of management, are the following: morphometric and functional parameters, hemodynamic data, available surgical options, results of the personal and institutional experience.

Aorta, Thoracic↗

PubMedDate not supplied

[Dynamics of the interrelationships of functional and morphologic parameters of the left and right ventricle in coarctation and decoarctation of the ascending aorta].

The contractile and ultrastructural parameters of the left and right ventricles were studied in rabbits 3 weeks after ascending aortic coarctation by a third of the baseline diameter. In other rabbits, the same parameters were studied 1, 2, 3, and 4 weeks after recovery of the normal aortic lumen following three weeks of its narrowing. It was found that a rapid recovery of the parameters under study did not occur after decoarctation despite the fact that left ventricular overload was abolished. A fortnight after decoarctation there was a profound increase in the contractility of the two ventricles and three weeks there was a significant enhancement of cardiomyocytic mitochondrial function.

Animals↗

PubMed2005

Poly(ADP-ribose) polymerase inhibition attenuates biventricular reperfusion injury after orthotopic heart transplantation.

OBJECTIVE: Poly (ADP-ribose) polymerase (PARP) activation plays a key role in free radical induced injury in ischemia/reperfusion. We investigated the effects of INO-1001 a novel PARP inhibitor on postischemic myocardial and endothelial function. METHODS: In dogs, 12 orthotopic heart transplantations were performed after 4 h ischemic preservation. At the beginning of reperfusion either saline vehicle (control, n=6), or INO-1001 (1 mg/kg, n=6) was applied. Before explantation and after 120 min of reperfusion we measured biventricular pressure-volume relationships by a combined conductance catheter and the adaptation potential of the right ventricle to acute afterload increase by pulmonary banding. Coronary blood flow (CBF), vasoreactivity, PARP-activation and ATP-content were also determined. RESULTS: INO-1001 led to significantly better recovery of contractility (91+/-3 vs. 44+/-7%, P<0.05) and CBF (44+/-4 vs. 29+/-3 ml/min, P<0.05) and higher increase in CBF after acetylcholine (61+/-10 vs. 27+/-8%, P<0.05). In addition, the inotropic adaptation potential of the right ventricle to an increased afterload was better preserved after INO-1001. ATP content was significantly higher in the INO-1001 group (11.0+/-2.1 vs. 4.5+/-1.1 micromol/g drw). Immunohistology revealed PARP activation in the control group which was abolished by INO-1001 treatment. CONCLUSIONS: PARP inhibition reduces myocardial and endothelial reperfusion injury after orthotopic heart transplantation.

Animals↗

PubMed1996

Right heart failure due to ventricular adiposity: 'adipositas cordis'--an old diagnosis revisited.

Adiposity of the heart is characterized by an increase in the amount of epicardial and other adipose tissue. The most pronounced changes involve the right ventricle. The adipocytes may be interposed between myocytes, and in severe cases the normal mechanics and function of the ventricle are impaired. Adiposity of the heart is usually an incidental finding at autopsy, and only rarely is it of clinical significance. This report describes a 46-year-old female with multiorgan failure secondary to bronchopneumonia, purulent pericarditis, tamponade and sepsis, whose clinical course was altered due to severe adiposity of the heart, so-called 'adipositas cordis'.

Adipose Tissue↗

PubMed2002

Isolated metastatic myocardial carcinoid tumor in a 48-year-old man.

This brief report describes an asymptomatic patient with a myocardial mass. Two-dimensional echocardiography, technetium Tc 99m cardiac nuclear scan, and transesophageal echocardiography were performed to define the mass. The mass, which involved the subvalvar right ventricular free wall, was resected and determined to be a metastatic carcinoid tumor by histologic and immunohistochemical analysis. Further investigation revealed the presence of a midgut carcinoid tumor located within the terminal ileum, which was also resected surgically. The patient recovered well after surgery and adjunctive chemotherapy. To our knowledge, this is the first report of comprehensive nuclear and echocardiographic imaging, supplemented by surgical and pathologic findings, in an asymptomatic patient with isolated myocardial metastasis of an ileal carcinoid tumor.

Carcinoid Tumor↗

PubMed1995

Clinical use of metaiodobenzylguanidine imaging in cardiology.

Cardiac function is predominantly regulated by autonomic innervation. Many heart diseases involve alterations of cardiac adrenergic neurotransmission. In patients with cardiomyopathies, numerous therapeutic agents act directly or indirectly on cardiac adrenergic disorders. Metaiodobenzylguanidine (MIBG) imaging can provide in vivo information on one of the main components of adrenergic nerve function, i.e. the norepinephrine reuptake and storage system. Diminished MIBG uptake has been reported in patients with congestive heart failure, indicating an impaired norepinephrine reuptake and storage system. In patients with dilated cardiomyopathy (either idiopathic or ischemic), this alteration has been linked to the severity of the disease, evaluated on the basis of clinical or hemodynamic parameters. Moreover, MIBG imaging has been reported in such patients to be a potent prognostic marker in comparison with other recognized indices. After myocardial infarction, the decrease in MIBG uptake was transient in some patients and was suggested to be a viability indicator. Diminished MIBG uptake in ischemic patients was linked to the occurrence of ventricular arrhythmias. In patients with primary hypertrophic cardiomyopathy, decreased cardiac MIBG uptake has also been related to the clinical indices of severity. In patients suffering from various arrhythmias such as idiopathic ventricular arrhythmias, arrhythmogenic right ventricular cardiomyopathy or a long-QT syndrome, MIBG imaging has evidenced regional abnormalities of adrenergic nerve function and has provided new insights into the pathophysiological mechanisms of such disorders. Finally, MIBG scintigraphy may permit the evaluation of anthracyclin cardiotoxicity. Thus, MIBG imaging appears to be a promising tool for the cardiologist.

3-Iodobenzylguanidine↗

PubMed2000

Effects of cardiac glycosides on atrial contractile dysfunction after short-term atrial fibrillation.

BACKGROUND: Despite a long history of use in the treatment of paroxysmal atrial fibrillation (AF), the efficacy of cardiac glycosides has not been established. If such drugs are beneficial in this condition, the general view is that the benefit must be related to their inotropic actions. METHODS AND RESULTS: To assess the effects of the rapid-acting cardiac glycoside, acetylstrophanthidin (AS), on AF and AF-induced right atrial (RA) "stunning," RA wall motion (with ultrasonic crystals), RA pressure, and peak first derivative of pressure (dp/dt) (with microtip transducers) were measured before and after 5 min of high-intensity rapid atrial stimulation (10 Hz; 10 mA; 1 ms) and after the cessation of poststimulation AF. Measurements were made in neurally intact and autonomically blockaded dogs both before and after the administration of AS (0.01 mg/kg IV bolus and 0.015 mg/kg/h IV infusion). AS prevented the post-AF reduction in RA peak dp/dt under neurally intact and autonomically blockaded conditions, and it prevented the post-AF increase in the RA end-systolic dimension and the decrease in the percentage of RA systolic shortening with autonomic blockade. AS was beneficial whether or not baseline inotropy was enhanced by AS. The duration of AF following atrial stimulation was the same before and after AS, but when compared to controls, AS treatment appeared to prolong AF. CONCLUSIONS: Cardiac glycosides exert a favorable effect on AF-induced RA stunning, but this action is unrelated to its effects on the duration of AF.

Animals↗

PubMed2006

Sequence of echocardiographic changes during development of right ventricular failure in rat.

BACKGROUND: The temporal relations between the onset of echocardiographic changes and clinical diagnosis of right ventricular (RV) failure are unresolved. We have characterized such relations in a rat monocrotaline (MCT) model of RV failure. METHODS: Eight-week-old male Wistar rats were injected with MCT (60 mg/kg) or vehicle and underwent serial echocardiography. RV free-wall thickness (RVWT), pulmonary artery acceleration time normalized to cycle length (PAAT/CL), RV end-diastolic diameter (RVEDD), and tricuspid annular plane systolic excursion (TAPSE) were measured. RESULTS: Significant differences in echocardiographic parameters between MCT-treated and control rats were found as early as 14 days before RV failure for RVWT, 10 days for PAAT/CL, and 7 days for RVEDD and TAPSE. The time intervals between the onset of changes in RVWT, PAAT/CL, RVEDD, and TAPSE and diagnosis of RV failure were 11.3 +/- 0.8, 10.9 +/- 0.7, 6.5 +/- 0.5, and 5.4 +/- 0.7 days, respectively. The sequence of echocardiographic changes was consistent in all animals during development of RV failure. CONCLUSIONS: Pulmonary hypertension (assessed by PAAT/CL) and RV free-wall thickening (characterized by RVWT) precede RV dilation and RV systolic dysfunction (measured by RVEDD and TAPSE, respectively). Echocardiographic analysis permits accurate determination of the stage of disease development in MCT-induced RV failure.

Animals↗

PubMed2003

Defibrillation causes immediate cardiac dilation in humans.

UNLABELLED: Defibrillation Causes Dilation. INTRODUCTION: Prior studies in isolated heart tissue have shown both excitation and deexcitation to be the primary mechanism of defibrillation. This article presents the first evidence in man of deexcitation immediately following defibrillation by tracking the heart's mechanical response. METHODS AND RESULTS: The geometric changes of the ventricular chambers were measured before and after defibrillation in seven human subjects receiving an implantable cardioverter defibrillator (ICD). The ICD was used to produce approximately three episodes of ventricular fibrillation and defibrillation in each subject. Twenty-two two-dimensional echocardiographic images of the right ventricle (RV) and 11 images of the left ventricle (LV) were recorded and analyzed at 30 frames per second. Just over 2 seconds of each episode were digitized, beginning half a second before the defibrillation shock. Individual frames were analyzed to yield cross-sectional, ventricular chamber area as a function of time. Immediately following defibrillation, ventricular chambers dilated with significant fractional area increase (RV: 1.58 +/- 0.25, LV: 1.10 +/- 0.06), with peak dilation at 194 +/- 114 msec. CONCLUSION: Defibrillation causes a rapid increase in ventricular chamber area due to relaxation of the myocardium, suggesting that defibrillation synchronizes the cardiac cells to the deexcited state in man.

Aged↗