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[Right ventricular tachyarrhythmias--diagnostics and therapy].

Ventricular tachyarrhythmias originating from the right ventricle frequently occur in young, apparently healthy patients with rare underlying cardiac diseases. Among these are arrhythmogenic right ventricular cardiomyopathy (ARVC), idiopathic right ventricular outflow-tract tachycardia (RVOVT), and Brugada syndrome (BrS). All harbor the risk of sudden cardiac death, whereas they differ substantially as to diagnosis, therapy and prognosis. This is the reason why detailed investigations are an essential prerequisite for further efficient individualized management strategies which are mainly directed to prevent sudden cardiac death and to minimize the risk of arrhythmia recurrences in affected patients, respectively. Both antiarrhythmic drug therapy, catheter ablation, and the implantation of an automatic cardioverter defibrillator may, therefore, be a first-line therapeutic option in tailored treatment regimens. This review is a summary of the available literature on pathogenesis, diagnosis, treatment, and prognosis of such diseases associated with right ventricular tachyarrhythmias.

Anti-Arrhythmia Agents↗

Precordial ST-segment elevation in acute occlusion of the proximal right coronary artery.

Isolated right ventricular myocardial infarction (RVMI) rarely occurs and accounts for only 3% of all myocardial infarction cases. In the literature, there are several reported isolated RVMI cases with precordial ST-segment elevation. We describe a 45-year-old man with marked ST-segment elevations in leads V1 through V4 accompanied by slight ST-segment elevations in the inferior leads (III, aVF) caused by acute occlusion of a nondominant small right coronary artery proximal to the conus branch causing isolated RVMI.

Acute Disease↗

Body surface isopotential T map for assessment of right ventricular volume and pressure overloads in secundum atrial septal defect.

The body surface isopotential T map was analyzed to detect right ventricular volume and pressure overloads in 25 patients with secundum atrial septal defect. Three patterns were distinguished: a T map resembling normal (type A, n = 9); that with an isolated negative area in a positive area (type B, n = 11); and that with rightward movement of maximum (type C, n = 5). Right ventricular end-diastolic volumes in types B (161 +/- 19% of normal; %N) and C (175 +/- 40% N) were significantly (p < 0.01) greater than those in controls (100 +/- 9% N) and type A (113 +/- 18% N). Right ventricular systolic pressure in type C (48 +/- 11 mmHg) was significantly (p < 0.01) higher than those in the controls (30 +/- 5 mmHg), type A (31 +/- 4 mmHg), or type B (34 +/- 5 mmHg). These results suggest that the patients with type B have right ventricular volume overload and those with type C have both volume and pressure overloads.

Body Surface Potential Mapping↗

Nitric oxide inhalation is useful in the management of right ventricular failure caused by myocardial infarction.

OBJECTIVE: To describe hemodynamic improvement in a patient treated with nitric oxide (NO) inhalation in the management of right ventricular failure caused by myocardial infarction. DESIGN: Case report. SETTING: An intensive care unit of a university hospital. PATIENT: A 66-yr-old man with severe right ventricular failure caused by acute myocardial infarction. INTERVENTIONS: Nitric oxide inhalation through a ventilator circuit. MEASUREMENTS AND MAIN RESULTS: The patient complained of chest pain. When myocardial infarction was diagnosed, he underwent percutaneous transluminal coronary angioplasty and percutaneous transluminal coronary recanalization, but they were not effective. We instituted intra-aortic balloon pumping and brought the patient to the intensive care unit (ICU). Even with high-dose inotropic support, his hemodynamics deteriorated gradually. On the patient's seventh day in the ICU, we started NO inhalation at 5-10 ppm in an attempt to relieve his right heart failure. Immediately after NO inhalation was started, his hemodynamics improved significantly, and we could wean the patient from intra-aortic balloon pumping. NO inhalation was continued for 9 days and was successfully discontinued without circulatory deterioration. He was discharged from our hospital uneventfully. CONCLUSION: Nitric oxide inhalation improved hemodynamics in our patient with right ventricular failure after myocardial infarction. Our report suggests that a clinical trial of NO treatment for severe right ventricular failure caused by myocardial infarction is warranted.

Administration, Inhalation↗

Suppressive effect of sarpogrelate hydrochloride on respiratory failure and right ventricular failure with pulmonary hypertension in patients with systemic sclerosis.

We evaluated the effect of 12 months of 300-mg oral sarpogrelate hydrochloride (SH) once daily on the symptoms of Raynaud's phenomenon, respiratory failure and cardiac function in seven patients with systemic sclerosis. Arterial blood gases, pulmonary function, mean pulmonary arterial pressure, left ventricular ejection fraction (LVEF), right ventricular ejection fraction (RVEF), white blood cell count, C-reactive protein and the plasma concentrations of fibrinopeptide A, beta-thromboglobulin, platelet factor 4 and thrombomodulin were evaluated before and 2 and 12 months after SH administration. After 2 and 12 months of SH administration, a significant decrease was found in the frequency and duration of Raynaud's phenomenon, as well as the coldness, numbness and pain of Raynaud's phenomenon. Respiratory failure, as estimated by Hugh-Jones classification, was significantly decreased, whereas the percentage carbon monoxide diffusion capacity was significantly increased. The mean pulmonary arterial pressure decreased significantly, as did plasma fibrinopeptide A, beta-thromboglobulin and platelet factor 4. There was no significant change in LVEF after 2 or 12 months, but after 12 months of SH administration, RVEF increased significantly. In conclusion, use of SH may prevent Raynaud's phenomenon, respiratory failure and right ventricular failure in patients with systemic sclerosis.

Adult↗

Brain death leads to abnormal contractile properties of the human donor right ventricle.

OBJECTIVES: Experimental and clinical data suggest that brain death predominantly affects the right ventricle. We aimed to investigate right ventricle function after brain death and during clinical transplantation with load-independent methods. METHODS: Patients with and without brain death were enrolled. A total of 33 consecutive heart donors (5 live, "domino" donors) and 10 patients undergoing coronary surgery (coronary artery bypass graft controls) were studied with pressure-volume loops in the right ventricle. Contractile reserve was measured with dopamine stimulation. RESULTS: Brain-dead donors had a higher mean cardiac index than coronary artery bypass graft controls (3.3 vs 2.8 L/min/m2), but impaired load-independent indices. Despite increased right ventricle stroke volume, the ejection fraction and slope of the end-systolic pressure-volume relationship were significantly reduced in brain-dead donors compared with controls. Diastolic abnormalities were also manifest as increased end-diastolic volume index and prolonged Tau (P < .05). Dopamine improved cardiac output, but without influencing end-systolic pressure-volume relationship, or Tau, and at the expense of further increased right ventricle end-diastolic volume. Before explantation, a significantly higher diastolic volume was also seen in hearts that developed postoperative dysfunction compared with organs without this complication (114.4 vs 77.2 mL/m2, P = .02). CONCLUSIONS: Brain death leads to right ventricle dysfunction, which may go undetected with conventional techniques. Right ventricle dilatation could represent an early marker of failure. Refinement of selection criteria to include load-independent indices of performance may be desirable to help expand the donor pool.

Aged↗

Comparison of modified short axis view and apical four chamber view in evaluating right ventricular function after repair of tetralogy of Fallot.

BACKGROUND: In clinical settings an easy and reliable method for following up right ventricular (RV) function in patients after repair of tetralogy of Fallot (TOF) is needed. It is, however, unclear whether the novel modified short axis view from echocardiography is superior to the apical four chamber view in this aspect. MATERIALS AND METHODS: Thirty postoperative TOF patients with median age 17 years (range 6-45 years) and follow up period of 10 years (range 0.5-40 years) were examined echocardiographically using the apical four chamber view and the novel modified short axis view. RV areas in end-systole (Amin) and end-diastole (Amax) were measured and an area fraction [(Amax - Amin)/Amax * 100%] was calculated from the respective view. RV ejection fraction was assessed through magnetic resonance imaging (MRI). The RV area fractions from echocardiography were compared to the RV ejection fraction. RESULTS: The right ventricular area fraction derived from the modified short axis view was significantly lower than that from the apical four chamber view (34.3+/-9.1% vs. 42.5+/-10.2%, p=0.007). Both the RV area fractions obtained from the modified short axis view (r=0.674, p<0.001) and from the apical four chamber view (r=0.512, p=0.025) correlated significantly with the MRI derived RV ejection fraction. CONCLUSION: The novel modified short axis view from echocardiography may be superior to the apical four chamber view for routine follow up of patients after TOF repair, in whom the right ventricular outflow tract plays an important role in the right ventricular systolic function.

Adolescent↗

Unusual right ventricular flow pattern in a patient with inferior wall myocardial infarction.

We describe a case of an unusual right ventricular flow pattern in a patient with inferior wall myocardial infarction associated with massive right ventricular infarction. Echocardiography was significant for nearly continuous antegrade flow through both the tricuspid and pulmonic valves demonstrated by color and pulsed wave Doppler. The tricuspid and pulmonic valves remained open throughout the cardiac cycle. These findings signify the nearly complete loss of right ventricular pumping function, and indicate that the right heart was serving as a passive conduit with a flow pattern similar to a vein.

Echocardiography, Doppler, Color↗

Misdiagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy.

INTRODUCTION: Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) has major implications for the management of patients and their first-degree relatives. Diagnosis is based on a set of criteria proposed by the International Task Force for Cardiomyopathies. We report our experience in providing a re-evaluation for patients who previously have been diagnosed with ARVD/C. METHODS AND RESULTS: We studied 89 patients who requested a re-evaluation for diagnosis of ARVD/C at our center. Each of these patients had been diagnosed with ARVD/C at their initial evaluation. Each patient was re-evaluated with clinical history, physical examination, and noninvasive testing at our center. Invasive testing, which included electrophysiologic testing, right ventricular angiography, and endomyocardial biopsy, was performed when clinically indicated. Sixty (92%) of the 65 patients who had undergone magnetic resonance imaging (MRI) at an outside institution were reported to have an abnormal MRI consistent with ARVD/C. Among these patients, the only abnormality identified was the qualitative finding of intramyocardial fat/wall thinning in 46 patients. On re-evaluation, these qualitative findings were not confirmed. None of these 46 patients ultimately were diagnosed with ARVD/C. Among the entire patient group, only 24 (27%) of the 89 patients met the Task Force criteria for ARVD/C. CONCLUSION: This study demonstrates that the high frequency of "misdiagnosis" of ARVD/C is due to over-reliance on the presence of intramyocardial fat/wall thinning on MRI, incomplete diagnostic testing, and lack of awareness of the Task Force criteria. Diagnosis of ARVD/C cannot rely solely upon qualitative features on MRI.

Adolescent↗

Acute right ventricular failure during aortic root replacement: intraoperative diagnosis and treatment.

The scope of this article is to report on a patient who underwent a Bentall procedure for type A aortic dissection. Right ventricular (RV) failure developed immediately after completion of the operation. The etiology was determined by using transit time flow measurement (TTFM), an ultrasound-based technique which demonstrated the absence of blood flow to the right coronary artery. This allowed for early and effective treatment, which was followed by a successful outcome.

Adult↗

Routine breath-hold gradient echo MRI-derived right ventricular mass, volumes and function: accuracy, reproducibility and coherence study.

Right ventricular (RV) dysfunction is a predictor of poor outcome in patients with heart disease. Conventional imaging modalities fail to assess RV volumes accurately. We sought to assess the accuracy and reproducibility of routine breath-hold gradient echo magnetic resonance imaging (MRI)-derived RV mass, volumes and function. We assessed: (1) The accuracy of in vivo MRI-derived RV mass in comparison to the RV weight in 9 minipigs. (2) Intra- and inter-observer reproducibility of RV mass, end-diastolic (EDV) and end-systolic (ESV) volumes and ejection fraction (EF) in 15 normal volunteers and 10 patients with heart disease. (3) Inter-study reproducibility of the former parameters in 25 coronary artery disease patients. (4) The correlation between right and left ventricular stroke volumes in the total population. Strong statistically significant correlations were found between: (1) MRI-derived RV mass and RV weight (r = 0.98, bias = 2.5 g), (2) Intra-observer measurements of RV mass (r = 0.96, bias = 0.5 g), EDV (r = 0.99, bias = -1.5 ml), ESV (r = 0.98, bias = 0.1 ml) and EF (r = 0.92, bias = -1.4%), (3) Inter-observer measurements of RV mass (r = 0.95, bias = 1.1 g), EDV (r = 0.98, bias = -1.1 ml), ESV (r = 0.98, bias = 1.2 ml) and EF (r = 0.87, bias = -1.9%), (4) Inter-study measurements of RV mass (r = 0.91, bias = -0.1 g), EDV (r = 0.96, bias = 3.8 ml), ESV (r = 0.98, bias = 0.3 ml) and EF (r = 0.90, bias = 0.9%), (5) MRI-derived right and left ventricular stroke volumes (r = 0.87). The assessment of the RV mass, volumes and function by routine breath-hold gradient echo MRI is accurate and highly reproducible. The correlation between left and RV MRI-derived stroke volumes indicates excellent coherence of simultaneous bi-ventricular volume measurements.

Adult↗

Ventricular tachycardias with left bundle branch block morphology.

The differential diagnosis of VTs with LBBB morphology includes several well-defined syndromes. Although the majority of cases are attributable to acquired structural heart disease, including ischemia, prior infarction, or dilated cardiomyopathy, consideration of specific right ventricular processes is essential to proper evaluation and treatment. The approach to older patients or those with evidence for heart disease should begin with an evaluation for coronary artery disease and an assessment of biventricular function. Careful evaluation for bundle branch reentry should be performed during electrophysiological study, especially when there is underlying conduction system disease. Younger patients, those without overt heart disease, or those with isolated right ventricular disease, should receive a complete noninvasive evaluation of right and left ventricular size and function. An abnormal SAECG or identification of intracardiac late potentials suggest right ventricular dysplasia or cardiomyopathy, whereas responsiveness to adenosine and absence of detectable heart disease support the diagnosis of idiopathic right VT. Newer techniques, including MRI, show promise in identifying subtle right ventricular disease not otherwise detectable even in the setting of presumed idiopathic right VT. Following surgical repair of selected congenital heart defects, particularly tetralogy of Fallot, symptoms of recurrent palpitations, near syncope, syncope, or aborted sudden death may be attributable to recurrent VT, and diagnostic electrophysiological study should be considered for these patients. Finally, SVTs with LBBB morphology, particularly cases associated with right-sided or septal accessory pathways, should always be considered in this differential diagnosis.

Bundle of His↗

[Pulmonary hypertension and right ventricular failure. Part IV. Chronic lung disease].

In a series of articles the authors discuss literature data concerning epidemiology of pulmonary hypertension, its modern classification; peculiarities of its pathogenesis and treatment in various diseases and conditions. In the forth lecture they present literature data on prevalence of chronic cor pulmonale among patients with congestive heart failure and its value for prognosis. The authors consider peculiarities of pathogenesis of pulmonary hypertension in patients with chronic obstructive pulmonary disease (COPD) and interstitial lung diseases. Attention is paid to the fact that chronic cor pulmonale develops mainly in bronchitic type of COPD and rarely is met in patients with emphysematous type of COPD. Pathogenetic mechanisms of right ventricular failure in COPD and the role of interaction of hemodynamic, renal, and neuro-humoral factors in pathogenesis of edematous syndrome in patients with chronic cor pulmonal are presented. The article also contains discussion of possibilities and limitations of clinical and instrumental diagnostics of pulmonary hypertension and right ventricular failure in patients with COPD.

Disease Progression↗

Severe right ventricular contraction asynchronism revealing a large pericardial effusion.

A gated blood-pool equilibrium radionuclide angiography was performed in a patient to determine the ejection fraction for doxorubicin cardiotoxicity evaluation. The phase image of the first harmonic of the Fourier analysis revealed a severe delay of the right ventricular contraction compared with that of the left ventricle. This right ventricular contraction asynchronism was due to a large pericardial effusion, confirmed by the presence of the halo sign on the summed gated images and by echocardiography. The phase delay moves towards normalization after pericardiocentesis. Although radionuclide angiocardiography is not the best method for identification of pericardial effusion, this diagnosis should be evoked when a severe homogenous delay of the right ventricular contraction is observed.

Cardiac Tamponade↗