Arrhythmogenic Right Ventricular Dysplasia

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... The heart has 4 sections called chambers. The right ventricle is one of the lower chambers. If you have ARVC, ... Kids and Teens, Men, WomenTags: arrhythmia, Arrhythmias, Arrhythmogenic Right ... April 1, 2006 Copyright © American Academy of Family Physicians This ...

Recurrent and founder mutations in the Netherlands Plakophilin-2 p.Arg79X mutation causing arrhythmogenic right ventricular cardiomyopathy/dysplasia

NARCIS (Netherlands)

van der Zwaag, P. A.; Cox, M. G. P. J.; van der Werf, C.; Wiesfeld, A. C. P.; Jongbloed, J. D. H.; Dooijes, D.; Bikker, H.; Jongbloed, R.; Suurmeijer, A. J. H.; van den Berg, M. P.; Hofstra, R. M. W.; Hauer, R. N. W.; Wilde, A. A. M.; van Tintelen, J. P.

Background. Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inherited cardiac disease with reduced penetrance and a highly variable expression. Mutations in the gene encoding the plakophilin-2 gene (PKP2) are detected in about 50% of ARVC/D patients. The p. Arg79X mutation

Recurrent and founder mutations in the Netherlands Plakophilin-2 p.Arg79X mutation causing arrhythmogenic right ventricular cardiomyopathy/dysplasia

NARCIS (Netherlands)

van der Zwaag, P. A.; Cox, M. G. P. J.; van der Werf, C.; Wiesfeld, A. C. P.; Jongbloed, J. D. H.; Dooijes, D.; Bikker, H.; Jongbloed, R.; Suurmeijer, A. J. H.; van den Berg, M. P.; Hofstra, R. M. W.; Hauer, R. N. W.; Wilde, A. A. M.; van Tintelen, J. P.

2010-01-01

Background. Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inherited cardiac disease with reduced penetrance and a highly variable expression. Mutations in the gene encoding the plakophilin-2 gene (PKP2) are detected in about 50% of ARVC/D patients. The p. Arg79X mutation

Genetics Home Reference: arrhythmogenic right ventricular cardiomyopathy

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... cardiomyopathy Merck Manual Consumer Version: Cardiomyopathy Merck Manual Consumer Version: Overview of Abnormal Heart Rhythms Orphanet: Arrhythmogenic right ventricular cardiomyopathy Orphanet: Familial isolated arrhythmogenic right ventricular ...

Characterization of the Left-Sided Substrate in Arrhythmogenic Right Ventricular Cardiomyopathy.

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Berte, Benjamin; Denis, Arnaud; Amraoui, Sana; Yamashita, Seigo; Komatsu, Yuki; Pillois, Xavier; Sacher, Frédéric; Mahida, Saagar; Wielandts, Jean-Yves; Sellal, Jean-Marc; Frontera, Antonio; Al Jefairi, Nora; Derval, Nicolas; Montaudon, Michel; Laurent, François; Hocini, Mélèze; Haïssaguerre, Michel; Jaïs, Pierre; Cochet, Hubert

2015-12-01

The correlates of left ventricular (LV) substrate in arrhythmogenic right ventricular (RV) cardiomyopathy are largely unknown. Thirty-two patients with arrhythmogenic RV cardiomyopathy (47±14 years; 6 women) were included. RV and LV dysplasia were defined from multidetector computed tomography and cardiac magnetic resonance imaging. Arrhythmias were characterized as right-sided or left-sided on 12-lead ECG recordings at baseline and during isoproterenol testing. In 14 patients, the imaging substrate was compared with voltage mapping and local abnormal ventricular activity. Imaging abnormalities were found in 32 (100%) and 21 (66%) patients on the RV and LV, respectively, intramyocardial fat on multidetector computed tomography being the most sensitive feature. LV involvement related to none of the Task Force criteria. Right-sided arrhythmias were more frequent than left-sided arrhythmias (P=0.003) although the latter were more frequent in case of LV involvement (P=0.02). The agreement between low voltage and fat on multidetector computed tomography was high on the RV when using either endocardial unipolar or epicardial bipolar data (κ=0.82 and κ=0.78, respectively) but lower on the LV (κ=0.54 for epicardial bipolar). LV local abnormal ventricular activity was found in all patients with LV involvement, and none of the others. The density of local abnormal ventricular activity within fat areas was similar between the RV and LV (P=0.57). LV substrate is frequent in arrhythmogenic RV cardiomyopathy, but poorly identified by current diagnostic strategies. Left-sided arrhythmias are more frequent in case of LV involvement. LV fat hosts the same density of local abnormal ventricular activity as RV fat, but is less efficiently detected by voltage mapping. These results support the need for alternative diagnostic strategies to identify LV dysplasia. © 2015 American Heart Association, Inc.

Magnetic resonance findings in arrhythmogenic dysplasia of right ventricle

International Nuclear Information System (INIS)

Ramiro, E.; Villacastin, B. P.; Farre, J.

1999-01-01

To compare the magnetic resonance (MR) findings in patients with arrhythmogenic dysplasia of right ventricle (ADRV) with the images of right ventricle (RV) presenting normal morphology. Three groups of patients were studied by MR: a) a group of 20 healthy volunteers; b) 22 patients with ADRV diagnosed by other methods; and c) 11 patients presenting right ventricular tachycardia (VT) with no evidence of ADRV. When compared with the other two groups, the patients with ADRV were found to have an enlarged right atrium and RV, one wall of RV abnormally thin, changes in the myocardial signal and significant left ventricular involvement. MR is a noninvasive method that is useful in the assessment of RV dilation, ventricular wall thinning, deformities and sacculations and in the detection of changes in the myocardial MR signal produced by the replacement. It aids in the localization, characterization and quantification of morphological changes in RV. (Author) 36 refs

Arrhythmogenic Right Ventricular Cardiomyopathy in an Endurance Athlete Presenting with Ventricular Tachycardia and Normal Right Ventricular Function.

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Hedley, Jeffrey S; Al Mheid, Ibhar; Alikhani, Zoubin; Pernetz, Maria A; Kim, Jonathan H

2017-08-01

Arrhythmogenic right ventricular cardiomyopathy, a genetically inherited disease that results in fibrofatty replacement of normal cardiac myocytes, has been associated with sudden cardiac death in athletes. Long-term participation in endurance exercise hastens the development of both the arrhythmic and structural arrhythmogenic right ventricular cardiomyopathy phenotypes. We describe the unusual case of a 34-year-old, symptomatic, female endurance athlete who had arrhythmogenic right ventricular cardiomyopathy in the presence of a structurally normal right ventricle. Clinicians should be aware of this infrequent presentation when evaluating athletic patients who have ventricular arrhythmias and normal findings on cardiac imaging studies.

Arrhythmogenic right ventricular cardiomyopathy in a dog : case report

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A.J. Möhr

2000-07-01

Full Text Available An 8-month-old Labrador retriever bitch was evaluated for sudden-onset, progressive abdominal distension. Physical examination revealed an exaggerated inspiratory effort, severe ascites, bilateral jugular vein distension, and hypokinetic femoral arterial pulses. Thoracic auscultation detected tachycardia with muffled heart sounds, without audible cardiac murmurs. Thoracic radiographs identified severe right ventricular enlargement and pleural effusion. The electrocardiogram was consistent with incomplete right bundle branch block or right ventricular enlargement. Echocardiography demonstrated severe right ventricular and atrial dilation, secondary tricuspid regurgitation, and thinning and hypocontractility of the right ventricular myocardium. Left heart chamber sizes were slightly decreased, with normal left ventricular contractility. Adiagnosis of arrhythmogenic right ventricular cardiomyopathy was reached, based on the characteristic clinical, electrocardiographic, radiographic and echocardiographic findings, and the exclusion of other causes of isolated right ventricular failure. Treatment effected good control of clinical signs, until acutely decompensated congestive right heart failure led to euthanasia after 4 months. Arrhythmogenic right ventricular cardiomyopathy is a well-described clinical entity in humans, and has previously been documented in 3 male dogs. The condition is characterised by progressive fibro-adipose replacement of right ventricular myocardium, while the left ventricle usually remains unaffected. It should be considered a differential diagnosis in any young dog presented with isolated right heart failure, syncope, or unexplained ventricular tachyarrhythmias. This article reports the 1st case of arrhythmogenic right ventricular cardiomyopathy in a female dog, and highlights its echocardiographic features.

Angiotensin-converting enzyme gene polymorphism in arrhythmogenic right ventricular dysplasia: is DD genotype helpful in predicting syncope risk?

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Ozben, Beste; Altun, Ibrahim; Sabri Hancer, Veysel; Bilge, Ahmet Kaya; Tanrikulu, Azra Meryem; Diz-Kucukkaya, Reyhan; Fak, Ali Serdar; Yilmaz, Ercument; Adalet, Kamil

2008-12-01

Arrhythmogenic right ventricular dysplasia (ARVD) is a heritable disorder characterised by fibrofatty replacement of right ventricular myocytes and increased risk of ventricular arrhythmias and sudden cardiac death. Angiotensin-converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism affects myocardial ACE levels. DD genotype favours myocardial fibrosis and is associated with malignant ventricular tachycardia. The aim of this study was to explore ACE gene polymorphism in ARVD patients. Twenty-nine patients with ARVD and 24 controls were included. All ARVD patients had documented sustained ventricular tachycardia. Thirteen patients had syncopal episodes. Six patients were resuscitated from sudden cardiac death. ACE gene polymorphism was identified by polymerase chain reaction technique. There was no significant difference in DD genotype frequency between ARVD patients and controls (44.8% vs. 45.8%, p=0.94). However, DD genotype frequency was significantly higher in ARVD patients with syncopal episodes compared to those without syncope (69.2% vs. 25.0%, p=0.017, odds ratio:6.750, 95% confidence interval: 1.318-34.565). DD genotype was detected in higher frequency also in patients with a family history of sudden cardiac death (66.7% vs. 39.1%,p=0.36). High prevalence of DD genotype in ARVD patients with syncope suggests that ACE I/D polymorphism might be useful in identifying high-risk patients for syncope.

[Diagnosis and therapy of arrhythmogenic right ventricular cardiomyopathy].

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Zorzi, Alessandro; Rigato, Ilaria; Migliore, Federico; Perazzolo Marra, Martina; Basso, Cristina; Thiene, Gaetano; Bauce, Barbara; Corrado, Domenico

2014-11-01

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart muscle disease that predisposes to the occurrence of ventricular arrhythmias and sudden death, particularly in the young and athlete. The classic variant of the disease predominantly affects the right ventricle, but phenotypic variants with early and prevalent left ventricular involvement ("left-dominant" ARVC) have also been described, supporting the concept that arrhythmogenic cardiomyopathy is a disease of both ventricles. The diagnosis is multiparametric and is based on a series of criteria, including ECG abnormalities, arrhythmic manifestations, morpho-functional abnormalities and genetic defects. The main goal of therapy is sudden death prevention. Implant of a cardioverter-defibrillator is the most effective strategy for prevention of sudden death, but it should be reserved to selected patients after accurate risk stratification, in view of the high complication rate over a long-term follow-up, the costs and the significant psychological impact of such therapy, especially in the young individual. The other therapies (either pharmacological or not) are palliative and aimed at relieving symptoms and preventing disease progression. The definitive cure of ARVC will be based on the discovery of the molecular mechanisms that are involved in the etiology and pathogenesis of the disease.

Advanced quantitative echocardiography in arrhythmogenic right ventricular cardiomyopathy

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Kjaergaard, Jesper; Hastrup Svendsen, Jesper; Sogaard, Peter

2007-01-01

BACKGROUND: Arrhythmogenic right ventricular (RV) cardiomyopathy (ARVC) is a regional disease of the RV myocardium with variable degrees of left ventricular involvement. Three-dimensional echocardiography and Doppler tissue imaging (DTI) are new echocardiographic modalities for the evaluation......, patients with ARVC had a decreased RV ejection fraction (0.47 +/- 0.08 vs 0.53 +/- 0.05, P vs 15.1 +/- 3.7 cm/s, P left ventricle (7.0 +/- 2.6 vs 9.5 +/- 1.9 cm/s, P ... of the longitudinal motility appears to be a sensitive marker of preclinical left ventricular involvement....

"Rescue" ablation of electrical storm in arrhythmogenic right ventricular cardiomyopathy in pregnancy.

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Stec, Sebastian; Krynski, Tomasz; Baran, Jakub; Kulakowski, Piotr

2013-08-13

Radiofrequency ablation (RFCA) became a treatment of choice in patients with recurrent ventricular tachycardia, ventricular fibrillation, and appropriate interventions of implanted cardioverter-defibrillator (ICD), however, electrical storm (ES) ablation in a pregnant woman has not yet been reported. We describe a case of a successful rescue ablation of recurrent ES in a 26-year-old Caucasian woman during her first pregnancy (23rd week). The arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) was diagnosed 3 years earlier and several drugs as well as 2 ablations failed to control recurrences of ventricular tachycardia. RFCA was performed on the day of the third electric storm. The use of electroanatomic mapping allowed very low X-ray exposure, and after applications in the right ventricular outflow tract, arrhythmia disappeared. Three months after ablation, a healthy girl was delivered without any complications. During twelve-month follow-up there was no recurrence of ventricular tachycardia or ICD interventions. This case documents the first successful RFCA during ES due to recurrent unstable ventricular arrhythmias in a patient with ARVD/C in pregnancy. Current guidelines recommend metoprolol, sotalol and intravenous amiodarone for prevention of recurrent ventricular tachycardia in pregnancy, however, RFCA should be considered as a therapeutic option in selected cases. The use of 3D navigating system and near zero X-ray approach is associated with minimal radiation exposure for mother and fetus as well as low risk of procedural complication.

Missense variants in plakophilin-2 in arrhythmogenic right ventricular cardiomyopathy patients - disease - causing or innocent bystanders?

DEFF Research Database (Denmark)

Christensen, A.H.; Benn, M.; Tybjaerg-Hansen, A.

2009-01-01

Objectives: Mutations in genes encoding desmosomal proteins have been linked to arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). We hypothesized that a Scandinavian ARVC/D population would have a different spectrum of plakophilin-2 (PKP2) mutations and that some of the reported...... missense mutations may not be pathogenic. Methods: We screened 53 unrelated patients fulfilling Task Force criteria for ARVC/D for mutations in PKP2 by direct sequencing. Results: Seven different mutations were identified: two insertion/deletions (E329fsX352, P401fsX406), 1 splice site (2146-2A>T), 1 non...

Missense variants in plakophilin-2 in arrhythmogenic right ventricular cardiomyopathy patients--disease-causing or innocent bystanders?

DEFF Research Database (Denmark)

Christensen, Alex Hørby; Benn, Marianne; Tybjaerg-Hansen, Anne

2010-01-01

Objectives: Mutations in genes encoding desmosomal proteins have been linked to arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). We hypothesized that a Scandinavian ARVC/D population would have a different spectrum of plakophilin-2 (PKP2) mutations and that some of the reported...... missense mutations may not be pathogenic. Methods: We screened 53 unrelated patients fulfilling Task Force criteria for ARVC/D for mutations in PKP2 by direct sequencing. Results: Seven different mutations were identified: two insertion/deletions (E329fsX352, P401fsX406), 1 splice site (2146-2A>T), 1 non...

Dynamic electrocardiographic changes in patients with arrhythmogenic right ventricular cardiomyopathy.

LENUS (Irish Health Repository)

Quarta, Giovanni

2010-04-01

Electrocardiographic (ECG) abnormalities of depolarisation and repolarisation contribute to the diagnostic criteria for arrhythmogenic right ventricular cardiomyopathy (ARVC). The development of diagnostic ECG features were investigated in a genotyped cohort with ARVC to provide more sensitive markers of early disease.

Arrhythmogenic right ventricular cardiomyopathy in a patient with schizophrenia

OpenAIRE

Kawasaki, Kenta; Miyaji, Kotaro; Kodera, Satoshi; Suzuki, Yoshio; Kanda, Junji; Ikeda, Masayuki

2015-01-01

Key Clinical Message People with schizophrenia are at greater risk of cardiovascular morbidity and mortality than the general population. Arrhythmogenic right ventricular cardiomyopathy is a recognized cause of sudden cardiac death in young people. This report discusses the necessity for close cardiac evaluation to reduce incidence of sudden death in people with schizophrenia.

Value of the Signal-Averaged Electrocardiogram in Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

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Kamath, Ganesh S.; Zareba, Wojciech; Delaney, Jessica; Koneru, Jayanthi N.; McKenna, William; Gear, Kathleen; Polonsky, Slava; Sherrill, Duane; Bluemke, David; Marcus, Frank; Steinberg, Jonathan S.

2011-01-01

Background Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inherited disease causing structural and functional abnormalities of the right ventricle (RV). The presence of late potentials as assessed by the signal averaged electrocardiogram (SAECG) is a minor Task Force criterion. Objective The purpose of this study was to examine the diagnostic and clinical value of the SAECG in a large population of genotyped ARVC/D probands. Methods We compared the SAECGs of 87 ARVC/D probands (age 37 ± 13 years, 47 males) diagnosed as affected or borderline by Task Force criteria without using the SAECG criterion with 103 control subjects. The association of SAECG abnormalities was also correlated with clinical presentation; surface ECG; VT inducibility at electrophysiologic testing; ICD therapy for VT; and RV abnormalities as assessed by cardiac magnetic resonance imaging (cMRI). Results When compared with controls, all 3 components of the SAECG were highly associated with the diagnosis of ARVC/D (p<0.001). These include the filtered QRS duration (fQRSD) (97.8 ± 8.7 msec vs. 119.6 ± 23.8 msec), low amplitude signal (LAS) (24.4 ± 9.2 msec vs. 46.2 ± 23.7 msec) and root mean square amplitude of the last 40 msec of late potentials (RMS-40) (50.4 ± 26.9 µV vs. 27.9 ± 36.3 µV). The sensitivity of using SAECG for diagnosis of ARVC/D was increased from 47% using the established 2 of 3 criteria (i.e. late potentials) to 69% by using a modified criterion of any 1 of the 3 criteria, while maintaining a high specificity of 95%. Abnormal SAECG as defined by this modified criteria was associated with a dilated RV volume and decreased RV ejection fraction detected by cMRI (p<0.05). SAECG abnormalities did not vary with clinical presentation or reliably predict spontaneous or inducible VT, and had limited correlation with ECG findings. Conclusion Using 1 of 3 SAECG criteria contributed to increased sensitivity and specificity for the diagnosis of ARVC/D. This

Role of ventricular tachycardia ablation in arrhythmogenic right ventricular cardiomyopathy

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Alberto Cipriani

2017-11-01

Full Text Available Arrhythmogenic right ventricular cardiomyopathy (ARVC is characterized by progressive fibro-fatty replacement of the myocardium that represents the substrate for recurrent sustained ventricular tachycardia (VT. These arrhythmias characterize the clinical course of a sizeable proportion of patients and have significant implications for their quality of life and long-term prognosis. Antiarrhythmic drugs are often poorly tolerated and usually provide incomplete control of arrhythmia relapses. Catheter ablation is a potentially effective strategy to treat frequent VT episodes and ICD shocks in ARVC patients. The aims of this review are to discuss the electrophysiological and electroanatomic substrates of ventricular tachycardia in patients with ARVC and to analyze the role of catheter ablation in their management with particular reference to selection of patients, technical issues, potential complications and outcomes.

Four cases of right ventricular dysplasia

International Nuclear Information System (INIS)

Takamura, Ichiro; Ando, Joji; Miyamoto, Atsushi; Kobayashi, Takeshi; Sakamoto, Sanya; Yasuda, Hisakazu

1985-01-01

Finding of 81 Kr right ventriculography and 201 Tl myocardial perfusion imaging in 4 patients with right ventricular dysplasia (RVD) were compared with those in 28 patients with dilated cardiomyopathy. Remarkably dilated right ventricle was detected on 201 Tl myocardial perfusion imaging in the RVD group. In a patient with RVD who died suddenly, perfusion defect of the left ventricular myocardium, a decreased right ventricular ejection fraction, and an increased right ventricular end diastolic volume were seen. Perfusion defect of the left ventricular myocardium was seen in 10 of the 28 patients with dilated cardiomyopathy, 4 of whom died suddenly. In these 4 patients, a decreased left ventricular ejection fraction and an increased right ventricular end diastolic volume were seen. These findings obtained by the radionuclide techniques suggested that there are differences in cardiac dysfunction of the both ventricles between the groups with RVD and dilated cardiomyopathy. (Namekawa, K.)

Genetic bases of arrhythmogenic right ventricular cardiomyopathy

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Alessandra Rampazzo

2010-05-01

Full Text Available Arrhythmogenic right ventricular cardiomyopathy (ARVC is a heart muscle disease in which the pathological substrate is a fibro-fatty replacement of the right ventricular myocardium. The major clinical features are different types of arrhythmias with a left branch block pattern. ARVC shows autosomal dominant inheritance with incomplete penetrance. Recessive forms were also described, although in association with skin disorders. Ten genetic loci have been discovered so far and mutations were reported in five different genes. ARVD1 was associated with regulatory mutations of transforming growth factor beta-3 (TGFβ3, whereas ARVD2, characterized by effort-induced polymorphic arrhythmias, was associated with mutations in cardiac ryanodine receptor-2 (RYR2. All other mutations identified to date have been detected in genes encoding desmosomal proteins: plakoglobin (JUP which causes Naxos disease (a recessive form of ARVC associated with palmoplantar keratosis and woolly hair; desmoplakin (DSP which causes the autosomal dominant ARVD8 and plakophilin-2 (PKP2 involved in ARVD9. Desmosomes are important cell-to-cell adhesion junctions predominantly found in epidermis and heart; they are believed to couple cytoskeletal elements to plasma membrane in cell-to-cell or cell-to-substrate adhesions.

Heart transplantation in arrhythmogenic right ventricular cardiomyopathy - Experience from the Nordic ARVC Registry

DEFF Research Database (Denmark)

Gilljam, Thomas; Haugaa, Kristina H; Jensen, Henrik K

2018-01-01

OBJECTIVE: There is a paucity of data on heart transplantation (HTx) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC), and specific recommendations on indications for listing ARVC patients for HTx are lacking. In order to delineate features pertinent to HTx assessment, we......%) and ventricular arrhythmias in 3 patients (10%). During median follow-up of 4.9years (0.04-28), there was one early death and two late deaths. Survival was 91% at 5years after HTx. Age at first symptoms under 35years independently predicted HTx in our cohort (OR=7.59, 95% CI 2.69-21.39, p... consequences of right ventricular failure in conjunction with ventricular arrhythmia....

Arrhythmogenic right ventricular cardiomyopathy in monozygotic twin sisters, and persistent left superior vena cava in one complicating implantation of ICD.

Science.gov (United States)

Astarcıoğlu, Mehmet Ali; Yaymacı, Mehmet; Şen, Taner; Kilit, Celal; Amasyalı, Basri

2015-10-01

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized histologically by fibro-fatty replacement of heart muscle, and clinically by ventricular arrhythmias and right ventricular dysfunction. This report presents monozygotic twins with ARVC, suggesting a genetic abnormality as the most probable cause.

Feature tracking CMR reveals abnormal strain in preclinical arrhythmogenic right ventricular dysplasia/ cardiomyopathy: a multisoftware feasibility and clinical implementation study.

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Bourfiss, Mimount; Vigneault, Davis M; Aliyari Ghasebeh, Mounes; Murray, Brittney; James, Cynthia A; Tichnell, Crystal; Mohamed Hoesein, Firdaus A; Zimmerman, Stefan L; Kamel, Ihab R; Calkins, Hugh; Tandri, Harikrishna; Velthuis, Birgitta K; Bluemke, David A; Te Riele, Anneline S J M

2017-09-01

Regional right ventricular (RV) dysfunction is the hallmark of Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C), but is currently only qualitatively evaluated in the clinical setting. Feature Tracking Cardiovascular Magnetic Resonance (FT-CMR) is a novel quantitative method that uses cine CMR to calculate strain values. However, most prior FT-CMR studies in ARVD/C have focused on global RV strain using different software methods, complicating implementation of FT-CMR in clinical practice. We aimed to assess the clinical value of global and regional strain using FT-CMR in ARVD/C and to determine differences between commercially available FT-CMR software packages. We analyzed cine CMR images of 110 subjects (39 overt ARVD/C [mutation+/phenotype+], 40 preclinical ARVD/C [mutation+/phenotype-] and 31 control) for global and regional (subtricuspid, anterior, apical) RV strain in the horizontal longitudinal axis using four FT-CMR software methods (Multimodality Tissue Tracking, TomTec, Medis and Circle Cardiovascular Imaging). Intersoftware agreement was assessed using Bland Altman plots. For global strain, all methods showed reduced strain in overt ARVD/C patients compared to control subjects (p  0.275). For regional strain, overt ARVD/C patients showed reduced strain compared to control subjects in all segments which reached statistical significance in the subtricuspid region for all software methods (p < 0.037), in the anterior wall for two methods (p < 0.005) and in the apex for one method (p = 0.012). Preclinical subjects showed abnormal subtricuspid strain compared to control subjects using one of the software methods (p = 0.009). Agreement between software methods for absolute strain values was low (Intraclass Correlation Coefficient = 0.373). Despite large intersoftware variability of FT-CMR derived strain values, all four software methods distinguished overt ARVD/C patients from control subjects by both global and subtricuspid

Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy: Progress and Pitfalls.

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Oomen, Ad W G J; Semsarian, Christopher; Puranik, Rajesh; Sy, Raymond W

2018-04-04

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy that predominantly affects the right ventricle. With a prevalence in the range of 1:5000 to 1:2000 persons, ARVC is one of the leading causes of sudden cardiac death in young people and in athletes. Although early detection and treatment is important, the diagnosis of ARVC remains challenging. There is no single pathognomonic diagnostic finding in ARVC; rather, current international task force criteria specify diagnostic major and minor criteria in six categories: right ventricular imaging (including echocardiography and cardiac magnetic resonance imaging (MRI)), histology, repolarisation abnormalities, depolarisation and conduction abnormalities, arrhythmias and family history (including genetic testing). Combining findings from differing diagnostic modalities can establish a "definite", "borderline" or "possible" diagnosis of ARVC. However, there are limitations inherent in the current task force criteria, including the lack of specificity for ARVC; future iterations may be improved, for example, by enhanced imaging protocols able to detect subtle changes in the structure and function of the right ventricle, incorporation of electro-anatomical data, response to adrenergic challenge, and validated criteria for interpreting genetic variants. Copyright © 2018 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.

Arrhythmogenic Right Ventricular Cardiomyopathy: Risk Stratification and Indications for Defibrillator Therapy.

Science.gov (United States)

Zorzi, Alessandro; Rigato, Ilaria; Bauce, Barbara; Pilichou, Kalliopi; Basso, Cristina; Thiene, Gaetano; Iliceto, Sabino; Corrado, Domenico

2016-06-01

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetically determined disease which predisposes to life-threatening ventricular arrhythmias. The main goal of ARVC therapy is prevention of sudden cardiac death (SCD). Implantable cardioverter defibrillator (ICD) is the most effective therapy for interruption of potentially lethal ventricular tachyarrhythmias. Despite its life-saving potential, ICD implantation is associated with a high rate of complications and significant impact on quality of life. Accurate risk stratification is needed to identify individuals who most benefit from the therapy. While there is general agreement that patients with a history of cardiac arrest or hemodynamically unstable ventricular tachycardia are at high risk of SCD and needs an ICD, indications for primary prevention remain a matter of debate. The article reviews the available scientific evidence and guidelines that may help to stratify the arrhythmic risk of ARVC patients and guide ICD implantation. Other therapeutic strategies, either alternative or additional to ICD, will be also addressed.

Mutation analysis of the candidate genes -, , and in patients with arrhythmogenic right ventricular cardiomyopathy

DEFF Research Database (Denmark)

Refsgaard, Lena; Olesen, Morten Salling; Møller, Daniel Vega

2012-01-01

INTRODUCTION: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetically determined heart disease characterized by fibrofatty infiltrations in the myocardium, right and/or left ventricular involvement, and ventricular tachyarrhythmias. Although ten genes have been associated with ARVC......, only about 40% of the patients have an identifiable disease-causing mutation. In the present study we aimed at investigating the involvement of the genes SCN1B-SCN4B, FHL1, and LMNA in the pathogenesis of ARVC. METHODS: Sixty-five unrelated patients (55 fulfilling ARVC criteria and 10 borderline cases...... of the variants was non-synonymous. No disease-causing mutations were identified. CONCLUSIONS: In our limited sized cohort the six studied candidate genes were not associated with ARVC....

Screening of Three Novel Candidate Genes in Arrhythmogenic Right Ventricular Cardiomyopathy

DEFF Research Database (Denmark)

Christensen, Alex Hørby; Benn, Marianne; Tybjærg-Hansen, Anne

2011-01-01

Arrhythmogenic right ventricular cardiomyopathy (ARVC) has been associated with mutations in genes encoding cellular adhesion proteins. However, only about 40% of patients have mutations in known genes. We hypothesized that mutations in the genes encoding ß-catenin (CTNNB1), a-T-catenin (CTNNA3......Scanner melting curve analysis. Our comprehensive mutation scanning did not identify any disease-causing mutations. Thirty-five sequence variants were found, including one rare nonsynonymous variant of unknown significance (CTNNA3 A689V). Fourteen of the variants were novel. In conclusion, in our cohort...

Right precordial-directed electrocardiographical markers identify arrhythmogenic right ventricular cardiomyopathy in the absence of conventional depolarization or repolarization abnormalities.

Science.gov (United States)

Cortez, Daniel; Svensson, Anneli; Carlson, Jonas; Graw, Sharon; Sharma, Nandita; Brun, Francesca; Spezzacatene, Anita; Mestroni, Luisa; Platonov, Pyotr G

2017-10-13

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) carries a risk of sudden death. We aimed to assess whether vectorcardiographic (VCG) parameters directed toward the right heart and a measured angle of the S-wave would help differentiate ARVD/C with otherwise normal electrocardiograms from controls. Task Force 2010 definite ARVD/C criteria were met for all patients. Those who did not fulfill Task Force depolarization or repolarization criteria (-ECG) were compared with age and gender-matched control subjects. Electrocardiogram measures of a 3-dimentional spatial QRS-T angle, a right-precordial-directed orthogonal QRS-T (RPD) angle, a root mean square of the right sided depolarizing forces (RtRMS-QRS), QRS duration (QRSd) and the corrected QT interval (QTc), and a measured angle including the upslope and downslope of the S-wave (S-wave angle) were assessed. Definite ARVD/C was present in 155 patients by 2010 Task Force criteria (41.7 ± 17.6 years, 65.2% male). -ECG ARVD/C patients (66 patients) were compared to 66 control patients (41.7 ± 17.6 years, 65.2% male). All parameters tested except the QRSd and QTc significantly differentiated -ECG ARVD/C from control patients (p right-sided VCG or measured angle markers better than the spatial QRS-T angle, the QRSd or QTc, in the absence of Taskforce ECG criteria.

[Arrhythmogenic right ventricular cardiomyopathy. Case report and a brief literature review].

Science.gov (United States)

Izurieta, Carlos; Curotto-Grasiosi, Jorge; Rocchinotti, Mónica; Torres, María J; Moranchel, Manuel; Cañas, Sebastián; Cardús, Marta E; Alasia, Diego; Cordero, Diego J; Angel, Adriana

2013-01-01

A 51-year-old man was admitted to this hospital because of palpitations and a feeling of dizziness for a period of 2h. The electrocardiogram revealed a regular wide-QRS complex tachycardia at a rate of 250 beats per minute, with superior axis and left bundle branch block morphology without hemodynamically decompensation, the patient was cardioverted to sinus rhythm after the administration of a loading and maintenance dose of amiodarone. The elechtrophysiological study showed the ventricular origin of the arrhythmia. In order to diagnose the etiology of the ventricular tachycardia we performed a coronary arteriography that showed normal epicardial vessels, thus ruling out coronary disease. Doppler echocardiography revealed systolic and diastolic functions of both left and right ventricles within normal parameters, and normal diameters as well. A cardiac magnetic resonance with late enhancement was done, showing structural abnormalities of the right ventricle wall with moderate impairment of the ejection fraction, and a mild dysfunction of the left ventricle. The diagnosis of arrhythmogenic right ventricular cardiomyopathy was performed as 2 major Task Force criteria were met. We implanted an automatic cardioverter defibrillator as a prophylactic measure. The patient was discharged without complications. Copyright © 2012 Instituto Nacional de Cardiología Ignacio Chávez. Published by Masson Doyma México S.A. All rights reserved.

[Assessment of the right ventricular anatomy and function by advanced echocardiography: pathological and physiological insights].

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Lakatos, Bálint; Kovács, Attila; Tokodi, Márton; Doronina, Alexandra; Merkely, Béla

2016-07-01

Accurate assessment of right ventricular geometry and function is of high clinical importance. However, several limitations have to be taken into consideration if using conventional echocardiographic parameters. Advanced echocardiographic techniques, such as speckle-tracking analysis or 3D echocardiography are reliable and simple tools providing a cost-effective and non-invasive alternative of current modalities used to characterize the right ventricle. There is a growing interest in the diagnostic and prognostic value of these methods regarding pathological (right ventricular infarction, pulmonary hypertension, arrhythmogenic right ventricular dysplasia, follow-up of heart transplantation) and even physiological (athlete's heart) alterations of the right ventricle. Orv. Hetil., 2016, 157(29), 1139-1146.

Arrhythmogenic right ventricular cardiomyopathy in Boxer dogs is associated with calstabin2 deficiency

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Oyama, Mark A.; Reiken, Steve; Lehnart, Stephan E.; Chittur, Sridar V.; Meurs, Kathryn M.; Stern, Joshua; Marks, Andrew R.

2010-01-01

Objective To examine the presence and effect of calstabin2-deficiency in Boxer dogs with arrhythmogenic right ventricular cardiomyopathy (ARVC). Animals Thirteen Boxer dogs with ARVC. Materials and methods Tissue samples were collected for histopathology, oligonucleotide microarray, PCR, immunoelectrophoresis, ryanodine channel immunoprecipitation and single-channel recordings, and calstabin2 DNA sequencing. Results In cardiomyopathic Boxer dogs, myocardial calstabin2 mRNA and protein were significantly decreased as compared to healthy control dogs (calstabin2 protein normalized to tetrameric cardiac ryanodine receptor (RyR2) complex: affected, 0.51 ± 0.04; control, 3.81 ± 0.22; P canine calstabin2 gene did not identify any causative mutations. Conclusions Calstabin2 deficiency is a potential mechanism of Ca2+ leak-induced ventricular arrhythmias and heart disease in Boxer dogs with ARVC. PMID:18515204

Mutation analysis and evaluation of the cardiac localization of TMEM43 in arrhythmogenic right ventricular cardiomyopathy

DEFF Research Database (Denmark)

Christensen, A H; Andersen, C B; Tybjærg-Hansen, A

2011-01-01

Christensen AH, Andersen CB, Tybjærg-Hansen A, Haunso S, Svendsen JH. Mutation analysis and evaluation of the cardiac localization of TMEM43 in arrhythmogenic right ventricular cardiomyopathy. A single report has associated mutations in TMEM43 (LUMA) with a distinctive form of arrhythmogenic right...... with anti-TMEM43, anti-plakoglobin, anti-plakophilin-2, anti-connexin-43, and anti-emerin antibodies was performed on myocardium from TMEM43-positive patients (n = 3) and healthy controls (n = 3). The genetic screening identified heterozygous variants in two families: one reported mutation (c.1073C> T......; in two related patients) and one novel variant (c.705+ 7G> A; in one patient) of unknown significance. All three patients fulfilled Task Force criteria and did not carry mutations in any other ARVC-related gene. Immunostaining with TMEM43 antibody showed intense staining of the sarcolemma. The signal...

Arrhythmogenic right ventricular cardiomyopathy mimics: role of cardiovascular magnetic resonance

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2013-01-01

Background Cardiovascular magnetic resonance (CMR) is commonly used in patients with suspected arrhythmogenic right ventricular cardiomyopathy (ARVC) based on ECG, echocardiogram and Holter. However, various diseases may present with clinical characteristics resembling ARVC causing diagnostic dilemmas. The aim of this study was to explore the role of CMR in the differential diagnosis of patients with suspected ARVC. Methods 657 CMR referrals suspicious for ARVC in a single tertiary referral centre were analysed. Standardized CMR imaging protocols for ARVC were performed. Potential ARVC mimics were grouped into: 1) displacement of the heart, 2) right ventricular overload, and 3) non ARVC-like cardiac scarring. For each, a judgment of clinical impact was made. Results Twenty patients (3.0%) fulfilled imaging ARVC criteria. Thirty (4.6%) had a potential ARVC mimic, of which 25 (3.8%) were considered clinically important: cardiac displacement (n=17), RV overload (n=7) and non-ARVC like myocardial scarring (n=4). One patient had two mimics; one patient had dual pathology with important mimic and ARVC. RV overload and scarring conditions were always thought clinically important whilst the importance of cardiac displacement depended on the degree of displacement from severe (partial absence of pericardium) to epiphenomenon (minor kyphoscoliosis). Conclusions Some patients referred for CMR with suspected ARVC fulfil ARVC imaging criteria (3%) but more have otherwise unrecognised diseases (4.6%) mimicking potentially ARVC. Clinical assessment should reflect this, emphasising the assessment and/or exclusion of potential mimics in parallel with the detection of ARVC major and minor criteria. PMID:23398958

Nationwide (Denmark) Study of Symptoms Preceding Sudden Death due to Arrhythmogenic Right Ventricular Cardiomyopathy

DEFF Research Database (Denmark)

Sadjadieh, Golnaz; Jabbari, Reza; Risgaard, Bjarke

2014-01-01

In this study, we investigated medical history and symptoms before death in all subjects aged 1 to 35 years who died a sudden cardiac death (SCD) from arrhythmogenic right ventricular cardiomyopathy (ARVC) in Denmark in the years 2000 to 2006. All deaths (n=6,629) in subjects aged 1 to 35 years...... control groups in the same age group and time interval: one consisting of subjects who died in traffic accidents (n=74) and the other consisting of patients who died a SCD due to coronary artery disease (CAD; n=34). In the case group, 8 of the 16 patients with ARVC experienced antecedent cardiac symptoms...

Management of patients with Arrhythmogenic Right Ventricular Cardiomyopathy in the Nordic countries.

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Haugaa, Kristina H; Bundgaard, Henning; Edvardsen, Thor; Eschen, Ole; Gilljam, Thomas; Hansen, Jim; Jensen, Henrik Kjærulf; Platonov, Pyotr G; Svensson, Anneli; Svendsen, Jesper H

2015-01-01

Diagnostics of patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) are complex, and based on the 2010 Task Force document including different diagnostic modalities. However, recommendations for clinical management and follow-up of patients with ARVC and their relatives are sparse. This paper aims to give a practical overview of management strategies, risk stratification, and selection of appropriate therapies for patients with ARVC and their family members. This paper summarizes follow-up and treatment strategies in ARVC patients in the Nordic countries. The author group represents cardiologists who are actively involved in the Nordic ARVC Registry which was established in 2009, and contains prospectively collected clinical data from more than 590 ARVC patients from Denmark, Norway, Sweden, and Finland. Different approaches of management and follow-up are required in patients with definite ARVC and in genetic-mutation-positive family members. Furthermore, ARVC patients with and without implantable cardioverter defibrillators (ICDs) require different follow-up strategies. Careful follow-up is required in patients with ARVC diagnosis to evaluate the need of anti-arrhythmic therapy and ICD implantation. Mutation-positive family members should be followed regularly for detection of early disease and risk stratification of ventricular arrhythmias.

Mayo AVC Registry and BioBank

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2018-03-05

Arrhythmogenic Right Ventricular Cardiomyopathy; Cardiomyopathies; Heart Diseases; Cardiovascular Diseases; Sudden Cardiac Arrest; Sudden Cardiac Death; Arrhythmogenic Right Ventricular Dysplasia; Arrhythmogenic Ventricular Cardiomyopathy; Familial Dilated Cardiomyopathy; Cardiovascular Abnormalities

Missense variants in plakophilin-2 in arrhythmogenic right ventricular cardiomyopathy patients--disease-causing or innocent bystanders?

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Christensen, Alex Hørby; Benn, Marianne; Tybjaerg-Hansen, Anne; Haunso, Stig; Svendsen, Jesper Hastrup

2010-01-01

Mutations in genes encoding desmosomal proteins have been linked to arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). We hypothesized that a Scandinavian ARVC/D population would have a different spectrum of plakophilin-2 (PKP2) mutations and that some of the reported missense mutations may not be pathogenic. We screened 53 unrelated patients fulfilling Task Force criteria for ARVC/D for mutations in PKP2 by direct sequencing. Seven different mutations were identified: two insertion/deletions (E329fsX352, P401fsX406), 1 splice site (2146-2A>T), 1 non-sense (R79X) and 4 missense mutations (Q62K in 2 patients, G489R, G673V) of undeterminable pathogeneity. None of these mutations was present in 650 controls. Five of the mutations were novel. Seven patients carried reported missense mutations (D26N, S140F, V587I); however, these mutations were identified in our healthy controls, although at a lower frequency. Evaluation of all reported missense mutations in PKP2 showed unclear pathogeneity of several reported mutations. Fifteen percent of Danish ARVC/D patients carried PKP2 mutations. Our finding of reported disease-causing mutations at a low frequency among healthy controls suggests that these variants are disease modifying but not directly disease causing. We recommend conservative interpretation of missense variants in PKP2, functional characterization and large-scale sequencing to clarify normal variation in the gene.

Time domain heart rate variability in Boxer dogs with arrhythmogenic right ventricular cardiomyopathy

Directory of Open Access Journals (Sweden)

Evandro Zacché

Full Text Available ABSTRACT: The aim of the present study was to assess heart rate variability (HRV in Boxer dogs affected by arrhythmogenic right ventricular cardiomyopathy (ARVC. Fourteen Boxer dogs classified as affected and 28 classified as unaffected were included in a prospective case-control study. Dogs underwent 24-hour ambulatory ECG and were classified as affected (>1,000 VPCs/24 hours or unaffected (<20 VPCs/24 hours by ARVC based on the number of ventricular arrhythmias. HRV was assessed using 24-h Holter ECG monitoring; the studied parameters were SDNN, SDANN, SDNNIDX, rMSSD and pNN50. Data were submitted to logarithmic transformation and HRV parameters were compared between groups and correlated according to the disease status, number and severity of ventricular arrhythmias using Student’s t test, linear regression and Spearman’s test. There was no interaction between the HRV parameters and the number and severity of ventricular arrhythmias. SDNNlog (2.35±0.14 vs. 2.46±0.12, P=0.01, SDNNIDXlog (2.18±0.14 vs. 2.24±0.10, P=0.002 and pNN50log (1.47±0.19 vs. 1.64±0.13, P=0.002 were significantly lower in the affected group compared with the unaffected. According to this study, HRV are different in a population of Boxers dogs affected by ARVC compared to a population of unaffected dogs, and these differences are not consequences of low cardiac output caused by ventricular arrhythmias since animals that had higher number and complexity of arrhythmias were not those who had lower values of HRV.

Life-threatening hobbies in the youth? Two autoptic cases suggesting arrhythmogenic right ventricular cardiomyopathy.

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Wingenfeld, Lisa; Freislederer, Andreas; Schulze-Bahr, Eric; Paul, Matthias; Bajanowski, Thomas

2007-08-24

Determining the cause for the sudden death in young adults tends to be complex and difficult. Two cases of death of young people were autoptically investigated who died suddenly while carrying out their hobbies (a 22-year-old male musician and a 20-year-old female dancer). In both cases neither the police investigation, the autopsy, nor the toxicological investigations gave any relevant results. However, when investigating the histology fatty and fibrotic tissue in the right ventricle of the myocardium were found, whereas the myocytes proved to be degenerated--typical for arrhythmogenic right ventricular cardiomyopathy (ARVC). It is important to consider the possibility of heart rhythm failure if a clear reason for sudden death in young adults cannot be detected. Heart rhythm failure often involves the genetic background of the case, which suggests that genetic analysis should be carried out as a supportive means of diagnostics.

Association of competitive and recreational sport participation with cardiac events in patients with arrhythmogenic right ventricular cardiomyopathy

DEFF Research Database (Denmark)

Ruwald, Anne-Christine; Marcus, Frank; Estes, N A Mark

2015-01-01

AIMS: It has been proposed that competitive sport increases the risk of ventricular tachyarrhythmias (VTA) and death in patients with arrhythmogenic right-ventricular cardiomyopathy (ARVC). However, it is unknown whether this only applies to competitive sport or if recreational sports activity also...... three categories of sports participation: competitive (n = 41), recreational (n = 48), and inactive (n = 19). Competitive sport was associated with a significantly higher risk of VTA/death when compared with both recreational sport [HR = 1.99 (1.21-3.28), P = 0.007] and inactive patients [HR = 2.05 (1.......07-3.91), P = 0.030]. No increased risk of VTA/death was associated with recreational sport when compared with patients who were inactive [HR = 1.03 (0.54-1.97), P = 0.930]. Symptoms developed at an earlier age in patients who participated in competitive sport (30 ± 12 years), when compared with patients who...

The Prognostic Value of Right Ventricular Deformation Imaging in Early Arrhythmogenic Right Ventricular Cardiomyopathy.

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Mast, Thomas P; Taha, Karim; Cramer, Maarten J; Lumens, Joost; van der Heijden, Jeroen F; Bouma, Berto J; van den Berg, Maarten P; Asselbergs, Folkert W; Doevendans, Pieter A; Teske, Arco J

2018-03-09

The aim of this study was to investigate the prognostic value of echocardiographic deformation imaging in arrhythmogenic right ventricular cardiomyopathy (ARVC) to optimize family screening protocols. ARVC is characterized by variable disease expressivity among family members, which complicates family screening protocols. Previous reports have shown that echocardiographic deformation imaging detects abnormal right ventricular (RV) deformation in the absence of established disease expression in ARVC. First-degree relatives of patients with ARVC were evaluated according to 2010 task force criteria, including RV deformation imaging (n = 128). Relatives fulfilling structural task force criteria were excluded for further analysis. At baseline, deformation patterns of the subtricuspid region were scored as type I (normal deformation), type II (delayed onset, decreased systolic peak, and post-systolic shortening), or type III (systolic stretching and large post-systolic shortening). The final study population comprised relatives who underwent a second evaluation during follow-up. Disease progression was defined as the development of a new 2010 task force criterion during follow-up that was absent at baseline. Sixty-five relatives underwent a second evaluation after a mean follow-up period of 3.7 ± 2.1 years. At baseline, 28 relatives (43%) had normal deformation (type I), and 37 relatives (57%) had abnormal deformation (type II or III) in the subtricuspid region. Disease progression occurred in 4% of the relatives with normal deformation at baseline and in 43% of the relatives with abnormal deformation at baseline (p values of abnormal deformation were, respectively, 43% (95% confidence interval: 27% to 61%) and 96% (95% confidence interval: 82% to 100%). Normal RV deformation in the subtricuspid region is associated with absence of disease progression during nearly 4-year follow-up in relatives of patients with ARVC. Abnormal RV deformation seems to precede the

Association of competitive and recreational sport participation with cardiac events in patients with arrhythmogenic right ventricular cardiomyopathy: results from the North American multidisciplinary study of arrhythmogenic right ventricular cardiomyopathy

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Ruwald, Anne-Christine; Marcus, Frank; Estes, N.A. Mark; Link, Mark; McNitt, Scott; Polonsky, Bronislava; Calkins, Hugh; Towbin, Jeffrey A.; Moss, Arthur J.; Zareba, Wojciech

2015-01-01

Aims It has been proposed that competitive sport increases the risk of ventricular tachyarrhythmias (VTA) and death in patients with arrhythmogenic right-ventricular cardiomyopathy (ARVC). However, it is unknown whether this only applies to competitive sport or if recreational sports activity also increases the risk of VTA/death. Methods and results Probands diagnosed with ARVC according to the 2010 task force criteria for ARVC (n = 108) were included in the current analysis. At the time of enrolment, study participants were questioned about exercise level prior to and after ARVC diagnosis, within three categories of sports participation: competitive (n = 41), recreational (n = 48), and inactive (n = 19). Competitive sport was associated with a significantly higher risk of VTA/death when compared with both recreational sport [HR = 1.99 (1.21–3.28), P = 0.007] and inactive patients [HR = 2.05 (1.07–3.91), P = 0.030]. No increased risk of VTA/death was associated with recreational sport when compared with patients who were inactive [HR = 1.03 (0.54–1.97), P = 0.930]. Symptoms developed at an earlier age in patients who participated in competitive sport (30 ± 12 years), when compared with patients who participated in recreational sport (38 ± 17 years) (P = 0.015) and inactive patients (41 ± 11 years) (P = 0.002). No difference in age at first symptom was seen between patients who participated in recreational sport and inactive patients (P = 0.651). Conclusion Competitive sport was associated with a two-fold increased risk of VTA/death, and earlier presentation of symptoms, when compared with inactive patients, and to patients who participated in recreational sport. When compared with inactive patients, recreational sport was not associated with earlier onset of symptoms or increased risk of VTA/death. Clinical trials.gov identifier NCT00024505. PMID:25896080

Phenotypic expression is a prerequisite for malignant arrhythmic events and sudden cardiac death in arrhythmogenic right ventricular cardiomyopathy.

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Zorzi, Alessandro; Rigato, Ilaria; Pilichou, Kalliopi; Perazzolo Marra, Martina; Migliore, Federico; Mazzotti, Elisa; Gregori, Dario; Thiene, Gaetano; Daliento, Luciano; Iliceto, Sabino; Rampazzo, Alessandra; Basso, Cristina; Bauce, Barbara; Corrado, Domenico

2016-07-01

Whether a desmosomal (DS)-gene defect may in itself induce life-threatening ventricular arrhythmias regardless of phenotypic expression of arrhythmogenic right ventricular cardiomyopathy (ARVC) is still debated. This prospective study evaluated the long-term outcome of DS-gene mutation carriers in relation to the ARVC phenotypic expression. The study population included 116 DS-gene mutation carriers [49% males; median age 33 years (16-48 years)] without prior sustained ventricular tachycardia (VT) or ventricular fibrillation (VF). The incidence of the arrhythmic endpoint, including sudden cardiac death (SCD), aborted SCD, sustained VT, and appropriate implantable cardioverter-defibrillator (ICD) intervention was evaluated prospectively and stratified by the presence of ARVC phenotype and risk factors (syncope, ventricular dysfunction, and non-sustained VT). At enrolment, 40 of 116 (34%) subjects fulfilled the criteria for definite ARVC while the remaining were either borderline or phenotype negatives. During a median follow-up of 8.5 (5-12) years, 10 patients (9%) had arrhythmic events (0.9%/year). The event rate was 2.3%/year among patients with definite ARVC and 0.2%/year among borderline or phenotype negative patients (P = 0.002). In patients with definite ARVC, the incidence of arrhythmias was higher in those with ≥1 risk factors (4.1%/year) than in those with no risk factors (0.4%/year, P = 0.02). Mortality was 0.2%/year (1 heart failure death and 1 SCD). The ARVC phenotypic expression is a prerequisite for the occurrence of life-threatening arrhythmias in DS-gene mutation carriers. The vast majority of malignant arrhythmic events occurred in patients with an overt disease phenotype and major risk factors suggesting that this subgroup most benefits from ICD therapy. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2015. For permissions please email: journals.permissions@oup.com.

Relation between N-terminal pro-brain natriuretic peptide and cardiac remodeling and function assessed by cardiovascular magnetic resonance imaging in patients with arrhythmogenic right ventricular cardiomyopathy.

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Cheng, Huaibing; Lu, Minjie; Hou, Cuihong; Chen, Xuhua; Wang, Jing; Yin, Gang; Chu, Jianmin; Zhang, Shu; Prasad, Sanjay K; Pu, Jielin; Zhao, Shihua

2015-02-01

Although N-terminal pro-brain natriuretic peptide (NT-proBNP) is a useful screening test of impaired right ventricular (RV) function in conditions affecting the right-sided cardiac muscle, the role of NT-proBNP remains unclear in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). This study was designed to clarify the relation between the plasma NT-proBNP level and the RV function evaluated by cardiovascular magnetic resonance (CMR) imaging. We selected 56 patients with confirmed ARVC only when their blood specimens for NT-proBNP measurements were collected within 48 hours of a CMR scan. The NT-proBNP level was significantly higher in patients with RV dysfunction than in patients without RV dysfunction (median of 655.3 [interquartile range 556.4 to 870.0] vs 347.0 [interquartile range 308.0 to 456.2] pmol/L, p rights reserved.

Role of strain imaging in right heart disease: a comprehensive review.

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Kannan, Arun; Poongkunran, Chithra; Jayaraj, Mahendran; Janardhanan, Rajesh

2014-10-01

Advances in the imaging techniques of the heart have fueled the interest in understanding of right heart pathology. Recently, speckle tracking echocardiography has shown to aid in understanding various right heart diseases and better management. Its role is well established in diagnosing right heart failure, pulmonary artery hypertension, arrhythmogenic right ventricular dysplasia and congenital heart disease. We review the basic mechanics of speckle tracking and analyze its role in various right heart conditions.

Assessment of right ventricular systolic function by tissue Doppler echocardiography

DEFF Research Database (Denmark)

Kjærgaard, Jesper

2012-01-01

This thesis summarizes a series of studies performed in order to assess the clinical usefulness of a novel echocardiographic technology that allows non-invasive assessment of regional right ventricular myocardial velocities and deformation: tissue Doppler echocardiography. While the technology...... is a promising tool for improving our understanding of right ventricular hemodynamics, several aspects of the technology must be evaluated. The accuracy and reproducibility of the technology is evaluated in vitro, and normal values, impact of changes in loading of the right ventricle, response to exercise...... on right ventricular hemodynamics: pulmonary embolism, Arrhythmogenic right ventricular cardiomyopathy and pulmonary regurgitation, the latter in an animal model. The conclusions of the thesis are: Color tissue Doppler echocardiography accurately measures velocities, SR and strain in vitro. No systematic...

Stop-gain mutations in PKP2 are associated with a later age of onset of arrhythmogenic right ventricular cardiomyopathy.

Directory of Open Access Journals (Sweden)

Mireia Alcalde

Full Text Available BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy (ARVC is a cardiac disease characterized by the presence of fibrofatty replacement of the right ventricular myocardium, which may cause ventricular arrhythmias and sudden cardiac death. Pathogenic mutations in several genes encoding mainly desmosomal proteins have been reported. Our aim is to perform genotype-phenotype correlations to establish the diagnostic value of genetics and to assess the role of mutation type in age-related penetrance in ARVC. METHODS AND RESULTS: Thirty unrelated Spanish patients underwent a complete clinical evaluation. They all were screened for PKP2, DSG2, DSC2, DSP, JUP and TMEM43 genes. A total of 70 relatives of four families were also studied. The 30 patients fulfilled definite disease diagnostic criteria. Genetic analysis revealed a pathogenic mutation in 19 patients (13 in PKP2, 3 in DSG2, 2 in DSP, and 1 in DSC2. Nine of these mutations created a truncated protein due to the generation of a stop codon. Familial assessment revealed 28 genetic carriers among family members. Stop-gain mutations were associated to a later age of onset of ARVC, without differences in the severity of the pathology. CONCLUSIONS: Familial genetic analysis helps to identify the cause responsible for the pathology. In discrepancy with previous studies, the presence of a truncating protein does not confer a worse severity. This information could suggest that truncating proteins may be compensated by the normal allele and that missense mutations may act as poison peptides.

Clinical validation of the planar radionuclide ventriculography in patients with right ventricular dysfunction.

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Bontemps, L; Merabet, Y; Chevalier, P; Itti, R

2013-01-01

Gated radionuclide ventriculography (RNV) may be used for the evaluation of the right ventricular function. However, the accuracy of the method should be clinically validated in patients suffering from diseases with specific pathology of the right ventricle (RV) and with possible left ventricular (LV) interaction. Three groups of 15 patients each, diagnosed with arrhythmogenic right ventricular dysplasia (ARVD), pulmonary artery hypertension (PAH) or atrial septal defect (ASD) were compared to a group of normal subjects. The parameters for both ventricles were evaluated separately (ejection fractions: LVEF and RVEF, and intraventricular synchronism quantified as phase standard deviation: LVPSD and RVPSD) as well as the relation or interdependence of the right to left ventricle (RV/LV volume ratio, LV/RV ejection fraction and stroke volume ratios, and interventricular synchronism). All the variables as a whole were analyzed to identify groups of patients according to their functional behaviour. Significant differences were found between the patients and control group for the RV function while the LV function remained mostly within normal limits. When the RV function was considered, the control group and ASD patient group showed differences regarding the ARVD and PAH patients. On evaluating the RV/LV ratios, differences were found between the control group and the ASD group. In the PAH patients, LV function showed differences in relation to the rest of the groups. RNV is a reliable clinical tool to evaluate RV function in patients with RV abnormality. Copyright © 2013 Elsevier España, S.L. and SEMNIM. All rights reserved.

Chamber-specific effects of hypokalaemia on ventricular arrhythmogenicity in isolated, perfused guinea-pig heart

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Osadchii, Oleg E; Bentzen, Bo Hjorth; Olesen, Søren-Peter

2009-01-01

of hypokalaemic perfusion (2.5 mm K(+) for 30 min) were assessed in isolated guinea-pig heart preparations using simultaneous recordings of volume-conducted electrocardiogram and monophasic action potentials from six ventricular epicardial sites. Effective refractory periods, ventricular fibrillation thresholds...... for re-entrant tachyarrhythmias. Taken together, these findings suggest that proarrhythmic effects of hypokalaemia are mostly attributed to increased LV arrhythmogenicity in the guinea-pig heart....

Nationwide (Denmark) study of symptoms preceding sudden death due to arrhythmogenic right ventricular cardiomyopathy.

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Sadjadieh, Golnaz; Jabbari, Reza; Risgaard, Bjarke; Olesen, Morten S; Haunsø, Stig; Tfelt-Hansen, Jacob; Winkel, Bo G

2014-04-01

In this study, we investigated medical history and symptoms before death in all subjects aged 1 to 35 years who died a sudden cardiac death (SCD) from arrhythmogenic right ventricular cardiomyopathy (ARVC) in Denmark in the years 2000 to 2006. All deaths (n=6,629) in subjects aged 1 to 35 years in Denmark in the period 2000 to 2006 were included. A total of 16 cases of SCD due to ARVC were identified based on histopathologic examination. Information on medical history was retrieved from The National Patient Registry, general practitioners, and hospitals. Symptoms before death were compared with 2 control groups in the same age group and time interval: one consisting of subjects who died in traffic accidents (n=74) and the other consisting of patients who died a SCD due to coronary artery disease (CAD; n=34). In the case group, 8 of the 16 patients with ARVC experienced antecedent cardiac symptoms and 7 of them sought medical attention. None were diagnosed with ARVC before death. Only 1 patient in the healthy control group and 31 of the 39 patients with CAD experienced cardiac symptoms before death. A total of 6 patients of the 16 with ARVC died during strenuous physical activity and 4 of the deaths were sports-related SCDs. In conclusion, antecedent cardiac symptoms before SCD in the young were seen in 1/2 of the patients who died because of ARVC, and this is significantly higher than in the healthy control group. When considering the ARVC and CAD groups collectively, antecedent cardiac symptoms are seen in the majority. Copyright © 2014 Elsevier Inc. All rights reserved.

Fibroblasts and the extracellular matrix in right ventricular disease.

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Frangogiannis, Nikolaos G

2017-10-01

Right ventricular failure predicts adverse outcome in patients with pulmonary hypertension (PH), and in subjects with left ventricular heart failure and is associated with interstitial fibrosis. This review manuscript discusses the cellular effectors and molecular mechanisms implicated in right ventricular fibrosis. The right ventricular interstitium contains vascular cells, fibroblasts, and immune cells, enmeshed in a collagen-based matrix. Right ventricular pressure overload in PH is associated with the expansion of the fibroblast population, myofibroblast activation, and secretion of extracellular matrix proteins. Mechanosensitive transduction of adrenergic signalling and stimulation of the renin-angiotensin-aldosterone cascade trigger the activation of right ventricular fibroblasts. Inflammatory cytokines and chemokines may contribute to expansion and activation of macrophages that may serve as a source of fibrogenic growth factors, such as transforming growth factor (TGF)-β. Endothelin-1, TGF-βs, and matricellular proteins co-operate to activate cardiac myofibroblasts, and promote synthesis of matrix proteins. In comparison with the left ventricle, the RV tolerates well volume overload and ischemia; whether the right ventricular interstitial cells and matrix are implicated in these favourable responses remains unknown. Expansion of fibroblasts and extracellular matrix protein deposition are prominent features of arrhythmogenic right ventricular cardiomyopathies and may be implicated in the pathogenesis of arrhythmic events. Prevailing conceptual paradigms on right ventricular remodelling are based on extrapolation of findings in models of left ventricular injury. Considering the unique embryologic, morphological, and physiologic properties of the RV and the clinical significance of right ventricular failure, there is a need further to dissect RV-specific mechanisms of fibrosis and interstitial remodelling. Published on behalf of the European Society of

Patient's Guide to Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

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... M., H.T., H.C.); and Departments of Psychology and Cardiovascular Sciences, East Carolina University, Greenville, ... and now incorporate genetic testing. They look for abnormal changes in the structure and function of the ...

Animal models of arrhythmogenic right ventricular cardiomyopathy: what have we learned and where do we go? Insight for therapeutics.

Science.gov (United States)

Padrón-Barthe, Laura; Domínguez, Fernando; Garcia-Pavia, Pablo; Lara-Pezzi, Enrique

2017-09-01

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare genetically-determined cardiac heart muscle disorder characterized by fibro-fatty replacement of the myocardium that results in heart failure and sudden cardiac death (SCD), predominantly in young males. The disease is often caused by mutations in genes encoding proteins of the desmosomal complex, with a significant minority caused by mutations in non-desmosomal proteins. Existing treatment options are based on SCD prevention with the implantable cardioverter defibrillator, antiarrhythmic drugs, and anti-heart failure medication. Heart transplantation may also be required and there is currently no cure. Several genetically modified animal models have been developed to characterize the disease, assess its progression, and determine the influence of potential environmental factors. These models have also been very valuable for translational therapeutic approaches, to screen new treatment options that prevent and/or reverse the disease. Here, we review the available ARVC animal models reported to date, highlighting the most important pathophysiological findings and discussing the effect of treatments tested so far in this setting. We also describe gaps in our knowledge of the disease, with the goal of stimulating research and improving patient outcomes.

Safety of American Heart Association-recommended minimum exercise for desmosomal mutation carriers

NARCIS (Netherlands)

Sawant, Abhishek C; Te Riele, Anneline S J M; Tichnell, Crystal; Murray, Brittney; Bhonsale, Aditya; Tandri, Harikrishna; Judge, Daniel P; Calkins, Hugh; James, Cynthia A

BACKGROUND: Endurance exercise is associated with adverse outcomes in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). Exercise recommendations for family members remain undetermined. OBJECTIVE: The purposes of this study were to determine if (1) endurance exercise

Combining computer modelling and cardiac imaging to understand right ventricular pump function.

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Walmsley, John; van Everdingen, Wouter; Cramer, Maarten J; Prinzen, Frits W; Delhaas, Tammo; Lumens, Joost

2017-10-01

Right ventricular (RV) dysfunction is a strong predictor of outcome in heart failure and is a key determinant of exercise capacity. Despite these crucial findings, the RV remains understudied in the clinical, experimental, and computer modelling literature. This review outlines how recent advances in using computer modelling and cardiac imaging synergistically help to understand RV function in health and disease. We begin by highlighting the complexity of interactions that make modelling the RV both challenging and necessary, and then summarize the multiscale modelling approaches used to date to simulate RV pump function in the context of these interactions. We go on to demonstrate how these modelling approaches in combination with cardiac imaging have improved understanding of RV pump function in pulmonary arterial hypertension, arrhythmogenic right ventricular cardiomyopathy, dyssynchronous heart failure and cardiac resynchronization therapy, hypoplastic left heart syndrome, and repaired tetralogy of Fallot. We conclude with a perspective on key issues to be addressed by computational models of the RV in the near future. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2017. For permissions, please email: journals.permissions@oup.com.

Right and Left Ventricular Function and Mass in Male Elite Master Athletes: A Controlled Contrast-Enhanced Cardiovascular Magnetic Resonance Study.

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Bohm, Philipp; Schneider, Günther; Linneweber, Lutz; Rentzsch, Axel; Krämer, Nadine; Abdul-Khaliq, Hashim; Kindermann, Wilfried; Meyer, Tim; Scharhag, Jürgen

2016-05-17

It is under debate whether the cumulative effects of intensive endurance exercise induce chronic cardiac damage, mainly involving the right heart. The aim of this study was to examine the cardiac structure and function in long-term elite master endurance athletes with special focus on the right ventricle by contrast-enhanced cardiovascular magnetic resonance. Thirty-three healthy white competitive elite male master endurance athletes (age range, 30-60 years) with a training history of 29±8 years, and 33 white control subjects pair-matched for age, height, and weight underwent cardiopulmonary exercise testing, echocardiography including tissue-Doppler imaging and speckle tracking, and cardiovascular magnetic resonance. Indexed left ventricular mass and right ventricular mass (left ventricular mass/body surface area, 96±13 and 62±10 g/m(2); Pright ventricular mass/body surface area, 36±7 and 24±5 g/m(2); Pleft ventricular end-diastolic volume and right ventricular end-diastolic volume (left ventricular end-diastolic volume/body surface area, 104±13 and 69±18 mL/m(2); Pright ventricular end-diastolic volume/body surface area, 110±22 and 66±16 mL/m(2); PRight ventricular ejection fraction did not differ between athletes and control subjects (52±8 and 54±6%; P=0.26). Pathological late enhancement was detected in 1 athlete. No correlations were found for left ventricular and right ventricular volumes and ejection fraction with N-terminal pro-brain natriuretic peptide, and high-sensitive troponin was negative in all subjects. Based on our results, chronic right ventricular damage in elite endurance master athletes with lifelong high training volumes seems to be unlikely. Thus, the hypothesis of an exercise-induced arrhythmogenic right ventricular cardiomyopathy has to be questioned. © 2016 American Heart Association, Inc.

Stress induced right ventricular dysfunction: An indication of reversible right ventricular ischaemia

International Nuclear Information System (INIS)

Underwood, S.R.; Walton, S.; Emanuel, R.W.; Swanton, R.H.; Campos Costa, D.; Laming, P.J.; Ell, P.J.

1987-01-01

Stress induced changes in left ventricular ejection fraction are widely used in the detection and assessment of coronary artery disease. This study demonstrates that right ventricular dysfunction may also occur, and assesses its significance in terms of coronary artery anatomy. This study involved 14 normal subjects and 26 with coronary artery disease investigated by equilibrium radionuclide ventriculography, at rest and during maximal dynamic exercise. Mean normal resting right ventricular ejection fraction (RVEF) was 0.40 (SD 0.118), and all normal subjects increased RVEF with stress (mean ΔRVEF+0.13 SD 0.099). Mean ΔRVEF in the subjects with coronary artery disease was significantly lower at 0.00 (SD 0.080), but there was overlap between the two groups. The largest falls in RVEF were seen if the right coronary artery was occluded without retrograde filling. In this subgroup with the most severely compromised right ventricular perfusion (nine subjects), RVEF always fell with stress, and mean ΔRVEF was -0.08 (SD 0.050). There was no significant correlation between ΔLVEF and ΔRVEF, implying that the right ventricular dysfunction was due to right ventricular ischaemia, rather than secondary to left ventricular dysfunction. Stress induced right ventricular ischaemia can therefore be detected readily by radionuclide ventriculography. (orig.)

High-resolution electrocardiography in the diagnosis of arrhythmogenic right ventricular cardiomyopathy in Boxer dogs Eletrocardiografia de alta resolução no diagnóstico da cardiomiopatia arritmogênica do ventrículo direito em cães da raça Boxer

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Evandro Zacché Pereira

2013-06-01

Full Text Available To evaluate the reliability of high-resolution electrocardiography in the diagnosis of arrhythmogenic right ventricular cardiomyopathy in Boxers, 20 dogs with no structural cardiac alterations at echocardiographic examination were grouped on the basis of frequency of ventricular arrhythmias, evaluated by 24-hour ambulatory ECG, and undergoing a high-resolution electrocardiography. High frequency QRS duration, duration of terminal QRS complex less than 40µV (LAS40 and root mean square voltage of the terminal 40 milliseconds of the QRS complex (RMS40 were measured. Differences in high-resolution ECG variables were not observed between groups. Therefore, the results of this investigation suggest that high-resolution electrocardiography is not a useful method for the diagnosis of arrhythmogenic right ventricular cardiomyopathy in Boxers without detectable myocardial alterations or systolic dysfunction.Com o objetivo de avaliar a importância da eletrocardiografia de alta resolução no diagnóstico da cardiomiopatia arritmogênica do ventrículo direito do Boxer, 20 cães sem evidências de doença cardíaca estrutural à avaliação ecodopplercardiográfica foram agrupados de acordo com a frequência de arritmias ventriculares, avaliadas pela eletrocardiografia ambulatorial de 24 horas, e submetidos ao exame eletrocardiográfico de alta resolução. Duração do complexo QRS filtrado, duração dos sinais de baixa amplitude (menor que 40µV dos últimos 40 milissegundos do complexo QRS e raiz quadrada média da voltagem ao quadrado dos últimos 40 milissegundos do complexo QRS (RMS40 foram as variáveis avaliadas. Não foram observadas diferenças significativas entre os grupos em relação às variáveis estudadas. Sendo assim, os resultados do presente estudo sugerem que a eletrocardiografia de alta resolução não é uma ferramenta útil no auxílio diagnóstico da cardiomiopatia arritmogênica do ventrículo direito dos cães da raça Boxer

Can sudden cardiac death in the young be predicted and prevented? Lessons from autopsy for the emergency physician.

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White, Jennifer L; Chang, Anna Marie; Cesar, Sergi; Sarquella-Brugada, Georgia

2018-06-01

Sudden unexpected death in the young, though rare, is devastating for both the family and the community. Although only 1.3 to 8.5 cases of sudden cardiac death (SCD) occur per 100 000 young people, autopsy is often inconclusive. Many causes of SCD are related to autosomal dominant inherited risk, however; therefore, answers are important for survivors. Causes of autopsy-positive SCD in young patients include hypertrophic cardiomyopathy and arrhythmogenic right ventricular dysplasia. Autopsy-negative SCD has been related to inherited arrhythmogenic causes such as long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, Wolff- Parkinson-White syndrome, and idiopathic ventricular fibrillation. The important question for the emergency physician is how SCD can be predicted and prevented in the young so that there is no need for an autopsy.

Recurrent missense mutations in TMEM43 (ARVD5) due to founder effects cause arrhythmogenic cardiomyopathies in the UK and Canada

KAUST Repository

Haywood, Annika; Merner, Nancy D.; Hodgkinson, Kathy A.; Houston, Jim; Syrris, Petros; Booth, Valerie; Connors, Sean; Pantazis, Antonios; Quarta, Giovanni; Elliott, Perry; McKenna, William; Young, Terry Lynn

2012-01-01

AimsAutosomal dominant arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) (in the group of arrhythmogenic cardiomyopathies) is a common cause of sudden cardiac death in young adults. It is both clinically and genetically heterogeneous, with 12 loci (ARVC/D1-12) and eight genes identified, the majority of which encode structural proteins of cardiac desmosomes. The most recent gene identified, TMEM43, causes disease due to a missense mutation in a non-desmosomal gene (p.S358L) in 15 extended families from Newfoundland, Canada. To determine whether mutations in TMEM43 cause ARVC/D and arrhythmogenic cardiomyopathy in other populations, we fully re-sequenced TMEM43 on 143 ARVC/D probands (families) from the UK and 55 probands (from 55 families) from Newfoundland.Methods and resultsBidirectional sequencing of TMEM43 including intron-exon boundaries revealed 33 variants, the majority located in non-coding regions of TMEM43. For the purpose of validation, families of probands with rare, potentially deleterious coding variants were subjected to clinical and molecular follow-up. Three missense variants of uncertain significance (p.R28W, p.E142K, p.R312W) were located in highly conserved regions of the TMEM43 protein. One variant (p.R312W) also co-segregated with relatives showing clinical signs of disease. Genotyping and expansion of the disease-associated haplotype in subjects with the p.R312W variant from Newfoundland, Canada, and the UK suggest common ancestry.ConclusionAlthough the p.R312W variant was found in controls (3/378), identification of an ancestral disease p R312W haplotype suggests that the p.R312W variant is a pathogenic founder mutation. © 2012 The Author.

Recurrent missense mutations in TMEM43 (ARVD5) due to founder effects cause arrhythmogenic cardiomyopathies in the UK and Canada

KAUST Repository

Haywood, Annika

2012-11-15

AimsAutosomal dominant arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) (in the group of arrhythmogenic cardiomyopathies) is a common cause of sudden cardiac death in young adults. It is both clinically and genetically heterogeneous, with 12 loci (ARVC/D1-12) and eight genes identified, the majority of which encode structural proteins of cardiac desmosomes. The most recent gene identified, TMEM43, causes disease due to a missense mutation in a non-desmosomal gene (p.S358L) in 15 extended families from Newfoundland, Canada. To determine whether mutations in TMEM43 cause ARVC/D and arrhythmogenic cardiomyopathy in other populations, we fully re-sequenced TMEM43 on 143 ARVC/D probands (families) from the UK and 55 probands (from 55 families) from Newfoundland.Methods and resultsBidirectional sequencing of TMEM43 including intron-exon boundaries revealed 33 variants, the majority located in non-coding regions of TMEM43. For the purpose of validation, families of probands with rare, potentially deleterious coding variants were subjected to clinical and molecular follow-up. Three missense variants of uncertain significance (p.R28W, p.E142K, p.R312W) were located in highly conserved regions of the TMEM43 protein. One variant (p.R312W) also co-segregated with relatives showing clinical signs of disease. Genotyping and expansion of the disease-associated haplotype in subjects with the p.R312W variant from Newfoundland, Canada, and the UK suggest common ancestry.ConclusionAlthough the p.R312W variant was found in controls (3/378), identification of an ancestral disease p R312W haplotype suggests that the p.R312W variant is a pathogenic founder mutation. © 2012 The Author.

Valvular dysplasia and congestive heart failure in a juvenile African penguin (Spheniscus demersus).

Science.gov (United States)

McNaughton, Allyson; Frasca, Salvatore; Mishra, Neha; Tuttle, Allison D

2014-12-01

Abstract: An aquarium-housed, 6-mo-old African penguin (Spheniscus demersus) presented with acute respiratory distress. Auscultation revealed a grade II-III systolic murmur in the absence of adventitial sounds, and an enlarged heart without pulmonary edema was seen radiographically. Echocardiographic evaluation revealed atrioventricular (AV) valvular dysplasia and ventricular enlargement. The penguin was treated with enalapril, furosemide, and pimobendan but died within 3 wk of detection of the murmur. Congenital dysplasia of the right AV valve with right atrial and ventricular dilation and ventricular hypertrophy were diagnosed on postmortem examination.

[The reasonable use of right ventricular protection strategy in right ventricular outflow tract reconstruction].

Science.gov (United States)

Zhang, Y; Yuan, H Y; Liu, X B; Wen, S S; Xu, G; Cui, H J; Zhuang, J; Chen, J M

2018-06-01

As a result of right ventricular outflow tract reconstruction, which is the important and basic step of complex cardiac surgery, the blood flow of right ventricular outflow tract is unobstructed, while pulmonary valve regurgitation and right heart dysfunction could be happened. These problems are often ignored in early days, more and more cases of right heart dysfunction need clinical intervention, which is quite difficult and less effective. How to protect effectively the right ventricular function is the focus. At present main methods to protect the right ventricular function include trying to avoid or reduce length of right ventricular incision, reserving or rebuilding the function of the pulmonary valve, using growth potential material for surgery. The protection of the right ventricular function is a systemic project, it involves many aspects, single measures is difficult to provide complete protection, only the comprehensive use of various protection strategy, can help to improve the long-term prognosis.

Persistently elevated right ventricular index of myocardial performance in preterm infants with incipient bronchopulmonary dysplasia.

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Christoph Czernik

Full Text Available OBJECTIVES: Elevated pulmonary vascular resistance occurs during the first days after birth in all newborn infants and persists in infants at risk for bronchopulmonary dysplasia (BPD. It is difficult to measure in a non-invasive fashion. We assessed the usefulness of the right ventricular index of myocardial performance (RIMP to estimate pulmonary vascular resistance in very low birth weight infants. STUDY DESIGN: Prospective echocardiography on day of life (DOL 2, 7, 14, and 28 in 121 preterm infants (median [quartiles] gestational age 28 [26]-[29] weeks, birth weight 998 [743-1225] g of whom 36 developed BPD (oxygen supplementation at 36 postmenstrual weeks. RESULTS: RIMP derived by conventional pulsed Doppler technique was unrelated to heart rate or mean blood pressure. RIMP on DOL 2 was similar in infants who subsequently did (0.39 [0.33-0.55] and did not develop BPD (0.39 [0.28-0.51], p = 0.467. RIMP declined steadily in non-BPD infants but not in BPD infants (DOL 7: 0.31[0.22-0.39] vs. 0.35[0.29-0.48], p = 0.014; DOL 14: 0.23[0.17-0.30] vs. 0.35[0.25-0.43], p<0.001; DOL 28: 0.21[0.15-0.28] vs. 0.31 [0.21-0.35], p = 0.015. CONCLUSIONS: In preterm infants, a decline in RIMP after birth was not observed in those with incipient BPD. The pattern of RIMP measured in preterm infants is commensurate with that of pulmonary vascular resistance.

Altered Right Ventricular Mechanical Properties Are Afterload Dependent in a Rodent Model of Bronchopulmonary Dysplasia

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Jitandrakumar R. Patel

2017-10-01

Full Text Available Infants born premature are at increased risk for development of bronchopulmonary dysplasia (BPD, pulmonary hypertension (PH, and ultimately right ventricular (RV dysfunction, which together carry a high risk of neonatal mortality. However, the role alveolar simplification and abnormal pulmonary microvascular development in BPD affects RV contractile properties is unknown. We used a rat model of BPD to examine the effect of hyperoxia-induced PH on RV contractile properties. We measured in vivo RV pressure as well as passive force, maximum Ca2+ activated force, calcium sensitivity of force (pCa50 and rate of force redevelopment (ktr in RV skinned trabeculae isolated from hearts of 21-and 35-day old rats pre-exposed to 21% oxygen (normoxia or 85% oxygen (hyperoxia for 14 days after birth. Systolic and diastolic RV pressure were significantly higher at day 21 in hyperoxia exposed rats compared to normoxia control rats, but normalized by 35 days of age. Passive force, maximum Ca2+ activated force, and calcium sensitivity of force were elevated and cross-bridge cycling kinetics depressed in 21-day old hyperoxic trabeculae, whereas no differences between normoxic and hyperoxic trabeculae were seen at 35 days. Myofibrillar protein analysis revealed that 21-day old hyperoxic trabeculae had increased levels of beta-myosin heavy chain (β-MHC, atrial myosin light chain 1 (aMLC1; often referred to as essential light chain, and slow skeletal troponin I (ssTnI compared to age matched normoxic trabeculae. On the other hand, 35-day old normoxic and hyperoxic trabeculae expressed similar level of α- and β-MHC, ventricular MLC1 and predominantly cTnI. These results suggest that neonatal exposure to hyperoxia increases RV afterload and affect both the steady state and dynamic contractile properties of the RV, likely as a result of hyperoxia-induced expression of β-MHC, delayed transition of slow skeletal TnI to cardiac TnI, and expression of atrial MLC1. These

The overloaded right heart and ventricular interdependence.

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Naeije, Robert; Badagliacca, Roberto

2017-10-01

The right and the left ventricle are interdependent as both structures are nested within the pericardium, have the septum in common and are encircled with common myocardial fibres. Therefore, right ventricular volume or pressure overloading affects left ventricular function, and this in turn may affect the right ventricle. In normal subjects at rest, right ventricular function has negligible interaction with left ventricular function. However, the right ventricle contributes significantly to the normal cardiac output response to exercise. In patients with right ventricular volume overload without pulmonary hypertension, left ventricular diastolic compliance is decreased and ejection fraction depressed but without intrinsic alteration in contractility. In patients with right ventricular pressure overload, left ventricular compliance is decreased with initial preservation of left ventricular ejection fraction, but with eventual left ventricular atrophic remodelling and altered systolic function. Breathing affects ventricular interdependence, in healthy subjects during exercise and in patients with lung diseases and altered respiratory system mechanics. Inspiration increases right ventricular volumes and decreases left ventricular volumes. Expiration decreases both right and left ventricular volumes. The presence of an intact pericardium enhances ventricular diastolic interdependence but has negligible effect on ventricular systolic interdependence. On the other hand, systolic interdependence is enhanced by a stiff right ventricular free wall, and decreased by a stiff septum. Recent imaging studies have shown that both diastolic and systolic ventricular interactions are negatively affected by right ventricular regional inhomogeneity and prolongation of contraction, which occur along with an increase in pulmonary artery pressure. The clinical relevance of these observations is being explored. Published on behalf of the European Society of Cardiology. All rights

Large right ventricular sinusoids in an infant with aorta-left ventricular tunnel and proximal right coronary artery atresia.

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Chen, Peter C; Spinner, Joseph A; Heinle, Jeffrey S

2018-07-01

We report a 1-month-old infant diagnosed with an aorta-left ventricular tunnel, ventricular septal defect, and right coronary atresia with right ventricular sinusoids. The patient's anatomy and physiology did not indicate right-ventricular-dependent coronary circulation, and therefore right ventricular decompression could be performed without compromising coronary perfusion during surgical correction. A detailed understanding of the coronary anatomy is critical in managing this defect when coronary anomalies are present.

Right Ventricular Outflow Tract Tachycardia with Structural Abnormalities of the Right Ventricle and Left Ventricular Diverticulum

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Bortolo Martini

2015-01-01

Full Text Available A 43-year-old woman presented to the emergency room with a sustained ventricular tachycardia (VT. ECG showed a QRS in left bundle branch block morphology with inferior axis. Echocardiography, ventricular angiography, and cardiac magnetic resonance imaging (CMRI revealed a normal right ventricle and a left ventricular diverticulum. Electrophysiology studies with epicardial voltage mapping identified a large fibrotic area in the inferolateral layer of the right ventricular wall and a small area of fibrotic tissue at the anterior right ventricular outflow tract. VT ablation was successfully performed with combined epicardial and endocardial approaches.

Vigorous physical activity impairs myocardial function in patients with arrhythmogenic right ventricular cardiomyopathy and in mutation positive family members

DEFF Research Database (Denmark)

Saberniak, Jørg; Hasselberg, Nina E; Borgquist, Rasmus

2014-01-01

patients and 45 mutation-positive family members. Athletes were defined as subjects with ≥4 h vigorous exercise/week [≥1440 metabolic equivalents (METs × minutes/week)] during a minimum of 6 years. Athlete definition was fulfilled in 37/110 (34%) subjects. We assessed right ventricular (RV) and left...

[The complex origin of ventricular tachycardia after the total correction of tetralogy of Fallot].

Science.gov (United States)

Ressia, L; Graffigna, A; Salerno-Uriarte, J A; Viganò, M

1993-09-01

Two patients underwent surgical treatment of ventricular tachycardia after repair of tetralogy of Fallot. Both patients had right bundle branch block, moderate pulmonary valve incompetence and right ventricular dilatation, and were refractory to electrophysiologically guided drug therapy. Both patients underwent intraoperative epicardial mapping, which located the arrhythmogenic focus on the right ventricular outflow tract, on the border of the previous ventriculotomy. In one patient removal of the previous scar and endocardial cryoablation was successful in ablating the arrhythmia. In the other, the same procedure was only temporarily effective. VT recurred and was subsequently identified at the superior border of the closed ventricular septal defect. It was ablated by means of transcatheter radiofrequency. While VT from foci located on the right ventricular free wall can be easily detected and ablated, septal origin of VT requires extensive preoperative and intraoperative electrophysiological evaluation and may necessitate combined surgical and transcatheter procedures.

Evaluation of Right Ventricular Function with Radionuclide Cardiac Angiography - Right Ventricular Ejection Fraction in Chronic Obstructive Lung Disease

International Nuclear Information System (INIS)

Sohn, In; Shin, Sung Hae; Chung, June Key; Lee, Myung Chul; Cho, Bo Youn; Lee, Young Woo; Han, Yong Cheol; Koh, Chang Soon

1982-01-01

To evaluate the usefulness of radionuclide cardiac angiography in the assessment of the right ventricular function, we measured right ventricular ejection fraction (RVEF) using single pass method. In 12 normal persons, RVEF averaged 52.7±5.9% (mean±S.D.). In 25 patients with chronic obstructive lung disease, RVEF was 37.2±10.6% and significantly lower than that of normal person (p<0.01). All 10 patients with right ventricular failure had abnormal RVEF, which was significantly lower than that of 14 persons without right ventricular failure (27.6±5.7%, 43.9±8.5%, respectively, p<0.01). It concluded that RVEF measured by single pass radionuclide cardiac angiography was a useful, noninvasive method to assess right ventricular function.

Right ventricular function in patients with ischemic heart disease

International Nuclear Information System (INIS)

Araki, Haruo; Hisano, Ryuichi; Nagata, Yoshiyuki; Caglar, N.; Nakamura, Motoomi

1985-01-01

Thirty-five patients with ischemic heart disease (IHD) and 10 normal subjects were studied. Right and left ventricular ejecction fractions (EF) were determined using equilibrium radionuclide ventriculography with technetium-99m. Furthermore, abnormal motion of the right ventricular septal wall was obtained by cardiac cathetelization, and its relation to the right ventricular EF was examined. In IHD patients with anterior myocardial infarction, left ventricular EF decreased, but right ventricular EF was normal. This suggested that left ventricular dysfunction does not always have an effect on right ventricular function. Right ventricular EF was normal even when akinesis or dyskinesis was present in the ventricular septul, suggesting that abnormal motion of the ventricular septal wall has no significantly stimulant effect on right ventricular function. A decreased right ventricular EF was likely to occur only when the right ventricular free wall became ischemic or necrotic simultaneously with occurrence of posterior myocardial infarction. (Namekawa, K.)

Two-dimensional echocardiographic features of right ventricular infarction

International Nuclear Information System (INIS)

D'Arcy, B.; Nanda, N.C.

1982-01-01

Real-time, two-dimensional echocardiographic studies were performed in 10 patients with acute myocardial infarction who had clinical features suggestive of right ventricular involvement. All patients showed right ventricular wall motion abnormalities. In the four-chamber view, seven patients showed akinesis of the entire right ventricular diaphragmatic wall and three showed akinesis of segments of the diaphragmatic wall. Segmental dyskinetic areas involving the right ventricular free wall were identified in four patients. One patient showed a large right ventricular apical aneurysm. Other echocardiographic features included enlargement of the right ventricle in eight cases, paradoxical ventricular septal motion in seven cases, tricuspid incompetence in eight cases, dilation of the stomach in four cases and localized pericardial effusion in two cases. Right ventricular infarction was confirmed by radionuclide methods in seven patients, at surgery in one patient and at autopsy in two patients

Delayed recovery of right ventricular systolic function after repair of long-standing tricuspid regurgitation associated with severe right ventricular failure.

Science.gov (United States)

Kim, Jong Hun; Kim, Kyung Hwa; Choi, Jong Bum; Kuh, Ja Hong

2016-03-01

After tricuspid valve surgery for long-standing tricuspid regurgitation associated with right ventricular failure, reverse remodelling of the enlarged right ventricle, including recovery of right ventricular systolic function, is unpredictable. We present the case of a 31-year old man with early reduction of dilated right ventricular dimensions and delayed recovery of impaired right ventricular systolic function after valve repair for traumatic tricuspid regurgitation lasting 16 years. © The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

Right ventricular strain in heart failure: Clinical perspective.

Science.gov (United States)

Tadic, Marijana; Pieske-Kraigher, Elisabeth; Cuspidi, Cesare; Morris, Daniel A; Burkhardt, Franziska; Baudisch, Ana; Haßfeld, Sabine; Tschöpe, Carsten; Pieske, Burket

2017-10-01

The number of studies demonstrating the importance of right ventricular remodelling in a wide range of cardiovascular diseases has increased in the past two decades. Speckle-tracking imaging provides new variables that give comprehensive information about right ventricular function and mechanics. In this review, we summarize current knowledge of right ventricular mechanics in heart failure with reduced ejection fraction and preserved ejection fraction. We searched PubMed, MEDLINE, Ovid and Embase databases for studies published from January 2000 to December 2016 in the English language using the following keywords: "right ventricle"; "strain"; "speckle tracking"; "heart failure with reduced ejection fraction"; and "heart failure with preserved ejection fraction". Investigations showed that right ventricular dysfunction is associated with higher cardiovascular and overall mortality in patients with heart failure, irrespective of ejection fraction. The number of studies investigating right ventricular strain in patients with heart failure with reduced ejection fraction is constantly increasing, whereas data on right ventricular mechanics in patients with heart failure with preserved ejection fraction are limited. Given the high feasibility, accuracy and clinical implications of right ventricular strain in the population with heart failure, it is of great importance to try to include the evaluation of right ventricular strain as a regular part of each echocardiographic examination in patients with heart failure. However, further investigations are necessary to establish right ventricular strain as a standard variable for decision-making. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

Sudden cardiac death in children and adolescents (excluding Sudden Infant Death Syndrome

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Gajewski Kelly

2010-01-01

Full Text Available Sudden death in the young is rare. About 25% of cases occur during sports. Most young people with sudden cardiac death (SCD have underlying heart disease, with hypertrophic cardiomyopathy and coronary artery anomalies being commonest in most series. Arrhythmogenic right ventricular dysplasia and long QT syndrome are the most common primary arrhythmic causes of SCD. It is estimated that early cardiopulmonary resuscitation and widespread availability of automatic external defibrillators could prevent about a quarter of pediatric sudden deaths.

Value of the Electrocardiogram as a Predictor of Right Ventricular Dysfunction in Patients With Chronic Right Ventricular Volume Overload.

Science.gov (United States)

Alonso, Pau; Andrés, Ana; Rueda, Joaquín; Buendía, Francisco; Igual, Begoña; Rodríguez, María; Osa, Ana; Arnau, Miguel A; Salvador, Antonio

2015-05-01

Pulmonary regurgitation is a common complication in patients with repaired tetralogy of Fallot or congenital pulmonary stenosis. Electrocardiographic variables have been correlated with parameters used to evaluate right ventricular function. We aimed to analyze the diagnostic value of the width and fragmentation of the electrocardiogram in the identification of patients with right ventricular dysfunction and/or dilation. We selected 107 consecutive patients diagnosed with severe pulmonary insufficiency after repair of pulmonary stenosis or tetralogy of Fallot. The tests included electrocardiography, echocardiography, and magnetic resonance. Each electrocardiogram was analyzed manually to measure QRS duration. We defined QRS fragmentation as the presence of low-voltage waves in the terminal portion of the QRS complex in at least 2 contiguous leads. We found a significant negative correlation between QRS width and right ventricular function, as well as a positive correlation with right ventricular volume. The receiver operating characteristic curve indicated a cut-off point for QRS width of 140ms, which showed good sensitivity for a diagnosis of right ventricular dilation (> 80%) and dysfunction (> 95%). In logistic regression models, a QRS duration > 140ms was found to be the only independent predictor of right ventricular dilation and dysfunction. Electrocardiography is a rapid, widely available, and reproducible tool. QRS width constitutes an independent predictor of the presence of right ventricular dilation and dysfunction. This study is the first to provide a cutoff value for QRS width to screen for right ventricle involvement. Copyright © 2014 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.

Left ventricular function in right ventricular overload

International Nuclear Information System (INIS)

Iwanaga, Shiro; Handa, Shunnosuke; Abe, Sumihisa; Onishi, Shohei; Nakamura, Yoshiro; Kunieda, Etsuo; Ogawa, Koichi; Kubo, Atsushi

1989-01-01

This study clarified regional and global functions of the distorted left ventricle due to right ventricular overload by gated radionuclide ventriculography (RNV). Cardiac catheterization and RNV were performed in 13 cases of atrial septal defect (ASD), 13 of pure mitral stenosis (MS), 10 of primary pulmonary hypertension (PPH), and 10 of normal subjects (NL). Right ventricular systolic pressure (RVSP) was 32.9±13.9, 45.0±12.2, 88.3±17.1, and 21.2±4.5 mmHg, respectively. The end-systolic LAO view of the left ventricle was halved into septal and free-wall sides. The end-diastolic halves were determined in the same plane. Ejection fractions of the global left ventricle (LVEF), global right ventricle (RVEF), the septal half of the left ventricle (SEPEF), and the free-wall half of the left ventricle (FWEF) were obtained. LVEF was 56.8±9.8% in NL, 52.8±10.5% in ASD, and 49.5±12.9% in PPH. In MS, LVEF (47.0±13.0%) was smaller than those in the other groups. RVEF was 37.0±5.2% in NL, 43.7±15.5% in ASD, and 32.8±11.5% in MS. In PPH, RVEF (25.0±10.6%) was smaller than those in the other groups. SEPEF was smaller in ASD (42.5±13.2%), MS (40.4±13.1%), PPH (40.5±12.5%) than in NL (53.5±8.5%). Systolic function of the septal half of the left ventricle was disturbed by right ventricular overload. RVEF (r=-0.35, p<0.05) and SEPEF (r=-0.51, p<0.01) had negative correlations with RVSP. As RVSP rose, systolic function of the septal half of the left ventricle was more severely disturbed. FWEF was the same among the four groups; NL (57.0±12.6%), ASD (48.6±15.2%), MS (50.5±12.0%), and PPH (51.1±12.3%). There was a good correlation between SEPEF and LVEF in NL (r=0.81), although in PPH this correlation was poor (r=0.64). These data showed that the distorted left ventricular due to right ventricular overload maintains its global function with preserved function of the free-wall side. (J.P.N.)

Right ventricular function in patients with dilated cardiomyopathy

International Nuclear Information System (INIS)

Kubota, Shuhei; Kubota, Sachio; Iwase, Takashi; Iizuka, Toshio; Imai, Susumu; Murata, Kazuhiko; Inoue, Tomio; Suzuki, Tadashi; Sasaki, Yasuhito.

1993-01-01

The characteristics and pathogenesis of right ventricular dysfunction in 14 patients with dilated cardiomyopathy (DCM) were investigated by equilibrium right ventricular blood pool scintigraphy using ultrashort-lifetime 81m Kr. Thirteen patients with severe left ventricular dysfunction due to old anterior myocardial infarction (OMI) and nine normal subjects were used as controls. The right ventricular end-diastolic pressure and volume index, mean pulmonary arterial pressure, and total pulmonary vascular resistance index were almost the same in the DCM and OMI patients. The right ventricular ejection fraction was 44.2±6.0% (mean±SD) in DCM patients and 47.1±7.9% in OMI patients, both significantly lower than those in the normal subjects (54.5±5.3%), but with no difference between the two case groups. The right ventricular peak filling rate was significantly reduced in both case groups as compared with the normal subjects (2.46±0.81 EDV/sec). The reduction was significantly greater (p 81m Kr blood pool scintigraphy is useful in the study of the right ventricular systolic and diastolic function. The diastolic parameters are more sensitive indicators for evaluation of right ventricular function in DCM than the systolic parameters. (author)

Situs inversus totalis associated with subaortic stenosis, restrictive ventricular septal defect, and tricuspid dysplasia in an adult dog.

Science.gov (United States)

Piantedosi, Diego; Cortese, Laura; Meomartino, Leonardo; Di Loria, Antonio; Ciaramella, Paolo

2011-11-01

A rare association between situs inversus totalis (SIT), restrictive ventricular septal defect, severe subaortic stenosis, and tricuspid dysplasia was observed in an adult mixed-breed dog. Primary ciliary dyskinesia and Kartagener's syndrome were excluded. After 15 mo the dog died suddenly. The association between SIT and congenital heart diseases is discussed.

Situs inversus totalis associated with subaortic stenosis, restrictive ventricular septal defect, and tricuspid dysplasia in an adult dog

OpenAIRE

Piantedosi, Diego; Cortese, Laura; Meomartino, Leonardo; Di Loria, Antonio; Ciaramella, Paolo

2011-01-01

A rare association between situs inversus totalis (SIT), restrictive ventricular septal defect, severe subaortic stenosis, and tricuspid dysplasia was observed in an adult mixed-breed dog. Primary ciliary dyskinesia and Kartagener’s syndrome were excluded. After 15 mo the dog died suddenly. The association between SIT and congenital heart diseases is discussed.

[Ventricular tachyarrhythmias in patients with cardiomyopathy

DEFF Research Database (Denmark)

Henningsen, K.; Christensen, A.H.; Svendsen, Jesper Hastrup

2008-01-01

by disease, gender, age, previous cardiac arrest and treatment with implantable cardioverter-defibrillator (ICD). RESULTS: 993 patients were screened and 128 patients with cardiomyopathy were identified, corresponding to 13% of the screened patients. 58 (45%) of the patients had dilated cardiomyopathy (DCM......), 57 (45%) patients had arrhythmogenic right ventricular cardiomyopathy (ARVC) and 13 (10%) had hypertrophic cardiomyopathy (HCM). The average age was 44 years for HCM, 41 years for ARVC and 58 years for DCM. The majority of the patients were male. ICD treatment was used in 95% of the patients...... with ARVC, 70% of the patients with HCM and 59% of the patients with DCM. Only 5 patients had previous cardiac arrest without reversible cause. CONCLUSION: The study shows that cardiomyopathies are relatively frequent causes of ventricular tachyarrhythmias in patients discharged from a specialised...

Right ventricular involvement in cardiac sarcoidosis demonstrated with cardiac magnetic resonance.

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Smedema, Jan-Peter; van Geuns, Robert-Jan; Ainslie, Gillian; Ector, Joris; Heidbuchel, Hein; Crijns, Harry J G M

2017-11-01

Cardiac involvement in sarcoidosis is reported in up to 30% of patients. Left ventricular involvement demonstrated by contrast-enhanced cardiac magnetic resonance has been well validated. We sought to determine the prevalence and distribution of right ventricular late gadolinium enhancement in patients diagnosed with pulmonary sarcoidosis. We prospectively evaluated 87 patients diagnosed with pulmonary sarcoidosis with contrast-enhanced cardiac magnetic resonance for right ventricular involvement. Pulmonary artery pressures were non-invasively evaluated with Doppler echocardiography. Patient characteristics were compared between the groups with and without right ventricular involvement, and right ventricular enhancement was correlated with pulmonary hypertension, ventricular mass, volume, and systolic function. Left ventricular late gadolinium enhancement was demonstrated in 30 patients (34%). Fourteen patients (16%) had right ventricular late gadolinium enhancement, with sole right ventricular enhancement in only two patients. The pattern of right ventricular enhancement consisted of right ventricular outflow tract enhancement in 1 patient, free wall enhancement in 8 patients, ventricular insertion point enhancement in 10 patients, and enhancement of the right side of the interventricular septum in 11 patients. Pulmonary arterial hypertension correlated with the presence of right ventricular enhancement (P Right ventricular enhancement correlated with systolic ventricular dysfunction (P Right ventricular enhancement was present in 16% of patients diagnosed with pulmonary sarcoidosis and in 48% of patients with left ventricular enhancement. The presence of right ventricular enhancement correlated with pulmonary arterial hypertension, right ventricular systolic dysfunction, hypertrophy, and dilation. © 2017 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of the European Society of Cardiology.

Alternative right ventricular pacing sites.

Science.gov (United States)

Łuciuk, Dariusz; Łuciuk, Marek; Gajek, Jacek

2015-01-01

The main adverse effect of chronic stimulation is stimulation-induced heart failure in case of ventricular contraction dyssynchrony. Because of this fact, new techniques of stimulation should be considered to optimize electrotherapy. One of these methods is pacing from alternative right ventricular sites. The purpose of this article is to review currently accumulated data about alternative sites of cardiac pacing. Medline and PubMed bases were used to search English and Polish reports published recently. Recent studies report a deleterious effect of long term apical pacing. It is suggested that permanent apical stimulation, by omitting physiological conduction pattern with His-Purkinie network, may lead to electrical and mechanical dyssynchrony of heart muscle contraction. In the long term this pathological situation can lead to severe heart failure and death. Because of this, scientists began to search for some alternative sites of cardiac pacing to reduce the deleterious effect of stimulation. Based on current accumulated data, it is suggested that the right ventricular outflow tract, right ventricular septum, direct His-bundle or biventricular pacing are better alternatives due to more physiological electrical impulse propagation within the heart and the reduction of the dyssynchrony effect. These methods should preserve a better left ventricular function and prevent the development of heart failure in permanent paced patients. As there is still not enough, long-term, randomized, prospective, cross-over and multicenter studies, further research is required to validate the benefits of using this kind of therapy. The article should pay attention to new sites of cardiac stimulation as a better and safer method of treatment.

Evaluation of right ventricular volumes measured by magnetic resonance imaging

DEFF Research Database (Denmark)

Møgelvang, J; Stubgaard, M; Thomsen, C

1988-01-01

stroke volume was calculated as the difference between end-diastolic and end-systolic volume and compared to left ventricular stroke volume and to stroke volume determined simultaneously by a classical indicator dilution technique. There was good agreement between right ventricular stroke volume......Right ventricular volumes were determined in 12 patients with different levels of right and left ventricular function by magnetic resonance imaging (MRI) using an ECG gated multisection technique in planes perpendicular to the diastolic position of the interventricular septum. Right ventricular...... determined by MRI and by the indicator dilution method and between right and left ventricular stroke volume determined by MRI. Thus, MRI gives reliable values not only for left ventricular volumes, but also for right ventricular volumes. By MRI it is possible to obtain volumes from both ventricles...

Right Ventricular Endomyocardial Fibrosis Presenting With Ventricular Tachycardia And Apical Thrombus - An Interesting Presentation

Directory of Open Access Journals (Sweden)

Amitesh Aggarwal

2009-11-01

Full Text Available Endomyocardial fibrosis is a progressive disease of unknown origin affecting children and young adults. It involves inflow portion of right and/or left ventricle and apex. It may be associated with thrombus. Literature regarding right ventricular endomyocardial fibrosis with thrombus is scarce. Here we report a rare case of right ventricular endomyocardial fibrosis presenting as ventricular tachycardia and echocardiographic evidence of apical thrombus. Interestingly there was no pulmonary involvement or evidence of deep venous thrombosis. This case also underscores the importance of urgent echocardiography in diagnosis of obscure cases of ventricular tachycardia.

Role of Electrophysiological Study and Catheter Ablation for Recurrent Ventricular Tachycardia Complicating Myocarditis

Directory of Open Access Journals (Sweden)

Emanuele Cecchi

2012-01-01

Full Text Available Here we report the case of a 31-year-old man admitted to our hospital with echocardiografic and Cardiac Magnetic Resonance signs of myocarditis complicated by ventricular tachycardia, initially resolved with direct current shock. After the recurrence of ventricular tachycardia the patient was submitted to electrophysiological study revealing a re-entrant circuit at the level of the medium segment of interventricular septum, successfully treated with transcatheter ablation. This case highlights how the presence of recurrent ventricular arrhythmias at the onset of acute myocarditis, suspected or proven, could be associated with a pre-existing arrhythmogenic substrate, therefore these patients should be submitted to electrophysiological study in order to rule out the presence of arrhythmogenic focuses that can be treated with transcatheter ablation.

Right Ventricular Pseudoaneurysm Following Endomyocardial Biopsy.

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Pita; Santos; Manteiga; Rodriguez; Beiras

1996-03-01

Ventricular perforation is an unusual complication after endomyocardial biopsy in heart transplanted patients. We report a case of asymptomatic right ventricular perforation and pseudoaneurysm formation, secondary to endomyocardial biopsy, diagnosed by angiography. The spontaneous obliteration of the pseudoaneurysm was observed.

Arrhythmogenic effects of illicit drugs in athletes.

Science.gov (United States)

Furlanello, Francesco; Bentivegna, Stefano; Cappato, Riccardo; De Ambroggi, Luigi

2003-12-01

Cardiac arrhythmias are among the most important causes of non-eligibility to sports activities, and may be due to different causes (cardiomyopathies, myocarditis, coronary abnormalities, valvular diseases, primary electrical disorders, abuse of illicit drugs). The list of illicit drugs banned by the International Olympic Committee and yearly updated by the World Anti-Doping Agency includes the following classes: stimulants, narcotics, anabolic agents (androgenic steroids and others such as beta-2 stimulants), peptide hormones, mimetics and analogues, diuretics, agents with an antiestrogenic activity, masking agents. Almost all illicit drugs may cause, through a direct or indirect arrhythmogenic effect, in the short, medium or long term, a wide range of cardiac arrhythmias (focal or reentry type, supraventricular and/or ventricular), lethal or not, even in healthy subjects with no previous history of cardiac diseases. Therefore, given the widespread abuse of illicit drugs among athletes, in the management of arrhythmic athletes the cardiologist should always take into consideration the possibility that the arrhythmias be due to the assumption of illicit drugs (sometimes more than one type), especially if no signs of cardiac diseases are present. On the other hand, in the presence of latent underlying arrhythmogenic heart disease including some inherited cardiomyopathies at risk of sudden cardiac death, illicit drugs could induce severe cardiac arrhythmic effects.

Congenital heart defects in oculodentodigital dysplasia: Report of two cases.

Science.gov (United States)

Izumi, Kosuke; Lippa, Andrew M; Wilkens, Alisha; Feret, Holly A; McDonald-McGinn, Donna M; Zackai, Elaine H

2013-12-01

Oculodentodigital dysplasia is caused by mutations in the GJA1 gene. Oculodentodigital dysplasia presents with a spectrum of clinical features including craniofacial, ocular, dental, and limb anomalies. Although recent findings implicate the major role of GJA1 during cardiac organogenesis, congenital heart defects are infrequently reported in oculodentodigital dysplasia. Here we report on two patients with GJA1 mutations presenting with cardiac malformations and type III syndactyly. Patient 1 presented with pulmonary atresia, an intact septum, right ventricular hypoplasia and tricuspid stenosis. The infant had a small nose, thin columella and bilateral 4-5 syndactyly of the fingers. A de novo c.226C>T (p.Arg76Cys) mutation was identified. Patient 2 presented at 6 months with a ventricular septal defect. The child had hypoplastic alae nasi with a thin columella and bilateral 4-5 syndactyly of the digits. A de novo missense mutation, c.145C>G (p.Gln49Glu) was found. Our two patients underscore the importance of cardiac evaluations as part of the initial workup for patients with findings of oculodentodigital dysplasia. Conversely, those patients with type III syndactyly and congenital heart defect should be screened for GJA1 mutations. © 2013 Wiley Periodicals, Inc.

Usefulness of Genetic Study by Next-generation Sequencing in High-risk Arrhythmogenic Cardiomyopathy.

Science.gov (United States)

Ruiz Salas, Amalio; Peña Hernández, José; Medina Palomo, Carmen; Barrera Cordero, Alberto; Cabrera Bueno, Fernando; García Pinilla, José Manuel; Guijarro, Ana; Morcillo-Hidalgo, Luis; Jiménez Navarro, Manuel; Gómez Doblas, Juan José; de Teresa, Eduardo; Alzueta, Javier

2018-03-29

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized by progressive fibrofatty replacement of predominantly right ventricular myocardium. This cardiomyopathy is a frequent cause of sudden cardiac death in young people and athletes. The aim of our study was to determine the incidence of pathological or likely pathological desmosomal mutations in patients with high-risk definite ARVC. This was an observational, retrospective cohort study, which included 36 patients diagnosed with high-risk ARVC in our hospital between January 1998 and January 2015. Genetic analysis was performed using next-generation sequencing. Most patients were male (28 patients, 78%) with a mean age at diagnosis of 45 ± 18 years. A pathogenic or probably pathogenic desmosomal mutation was detected in 26 of the 35 index cases (74%): 5 nonsense, 14 frameshift, 1 splice, and 6 missense. Novel mutations were found in 15 patients (71%). The presence or absence of desmosomal mutations causing the disease and the type of mutation were not associated with specific electrocardiographic, clinical, arrhythmic, anatomic, or prognostic characteristics. The incidence of pathological or likely pathological desmosomal mutations in ARVC is very high, with most mutations causing truncation. The presence of desmosomal mutations was not associated with prognosis. Copyright © 2017 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.

Right ventricular pressure response to exercise in adults with isolated ventricular septal defect closed in early childhood.

Science.gov (United States)

Moller, Thomas; Lindberg, Harald; Lund, May Brit; Holmstrom, Henrik; Dohlen, Gaute; Thaulow, Erik

2018-06-01

We previously demonstrated an abnormally high right ventricular systolic pressure response to exercise in 50% of adolescents operated on for isolated ventricular septal defect. The present study investigated the prevalence of abnormal right ventricular systolic pressure response in 20 adult (age 30-45 years) patients who underwent surgery for early ventricular septal defect closure and its association with impaired ventricular function, pulmonary function, or exercise capacity. The patients underwent cardiopulmonary tests, including exercise stress echocardiography. Five of 19 patients (26%) presented an abnormal right ventricular systolic pressure response to exercise ⩾ 52 mmHg. Right ventricular systolic function was mixed, with normal tricuspid annular plane systolic excursion and fractional area change, but abnormal tricuspid annular systolic motion velocity (median 6.7 cm/second) and isovolumetric acceleration (median 0.8 m/second2). Left ventricular systolic and diastolic function was normal at rest as measured by the peak systolic velocity of the lateral wall and isovolumic acceleration, early diastolic velocity, and ratio of early diastolic flow to tissue velocity, except for ejection fraction (median 53%). The myocardial performance index was abnormal for both the left and right ventricle. Peak oxygen uptake was normal (mean z score -0.4, 95% CI -2.8-0.3). There was no association between an abnormal right ventricular systolic pressure response during exercise and right or left ventricular function, pulmonary function, or exercise capacity. Abnormal right ventricular pressure response is not more frequent in adult patients compared with adolescents. This does not support the theory of progressive pulmonary vascular disease following closure of left-to-right shunts.

Right atrial and right ventricular ultrasound-guided biopsy technique in standing horses.

Science.gov (United States)

Decloedt, A; de Clercq, D; Ven, S; van der Vekens, N; Chiers, K; van Loon, G

2016-05-01

Endomyocardial biopsies could be a valuable tool in equine cardiology for diagnosing myocardial disease, which is probably underdiagnosed in horses because of lack of specific diagnostic measures and limitations of currently available diagnostic methods. To describe a technique for serial right atrial and right ventricular endomyocardial biopsy in standing horses using a percutaneous approach through the jugular vein. Prospective study. Biopsy was performed in 10 healthy standing horses sedated with detomidine, under continuous electrocardiography monitoring. A 10 cm (n = 6), 45 cm (n = 1) or 98 cm (n = 3) introducer sheath was inserted into the right jugular vein. Under echocardiographic guidance, a biopsy forceps was introduced through the sheath into the right ventricle and right atrium and endomyocardial biopsies were acquired. In all horses, 3 right ventricular biopsies were obtained from the right ventricular apex and 3 right atrial biopsies were obtained from the dorsal right atrial wall near the tuberculum intervenosum. The presence of myocardial tissue was confirmed by histology. All horses showed atrial and ventricular premature depolarisations associated with acquisition of the biopsies. In 9 horses, the arrhythmia disappeared after retraction of the forceps and introducer sheath. In one horse, ventricular premature depolarisations disappeared only after 8 h. No other complications were observed. Endomyocardial biopsy of the right atrium and right ventricle could be performed in standing horses using a percutaneous approach through the jugular vein and was not associated with complications other than temporary arrhythmias. This technique may be useful for research purposes or as a diagnostic tool, although further research is needed to establish the safety of the technique in clinical patients with myocardial disease. © 2015 EVJ Ltd.

Assessment of right ventricular function using gated blood pool single photon emission computed tomography in inferior myocardial infarction with or without hemodynamically significant right ventricular infarction

International Nuclear Information System (INIS)

Takahashi, Masaharu

1992-01-01

Right ventricular function was assessed using gated blood pool single photon emission computed tomography (GSPECT) in 10 normal subjects and 14 patients with inferior myocardial infarction. Three-dimensional backbround subtraction was achieved by applying an optimal cut off level. The patient group consisted of 6 patients with definite hemodynamic abnormalities indicative of right ventricular infarction (RVI) and 8 other patients with significant obstructive lesion at the proximal portion of right coronary artery without obvious hemodynamic signs of RVI. Right ventricular regional wall motion abnormalities were demonstrated on GSPECT functional images and the indices of right ventricular function (i.e the right ventricular ejection fraction (RVEF), the right ventricular peak ejection rate (RVPER) and the right ventricular peak filling rate (RVPFR)) were significantly reduced in the patient group, not only in the patients with definite RVI but also in those without hemodynamic signs of RVI, even in the absence of definite hemodynamic signs, when the proximal portion of right coronary artery is obstructed. It is concluded that GSPECT is reliable for the assessment of right ventricular function and regional wall motion, and is also useful for the diagnosis of RVI. (author)

Clinical use of ultrashort-lived radionuclide krypton-81m for noninvasive analysis of right ventricular performance in normal subjects and patients with right ventricular dysfunction

International Nuclear Information System (INIS)

Nienaber, C.A.; Spielmann, R.P.; Wasmus, G.; Mathey, D.G.; Montz, R.; Bleifeld, W.H.

1985-01-01

The ultrashort-lived radionuclide krypton-81m, eluted in 5% dextrose from a bedside rubidium-81m generator, was intravenously infused for rapid imaging of the right-sided heart chambers in the right anterior oblique projection adjusted for optimal right atrioventricular separation. Left-sided heart and lung background was minimized by rapid decay and efficient exhalation of krypton-81m, requiring no algorithm for background correction. A double region of interest method decreased the variability in the assessment of ejection fraction to 5%. In 10 normal subjects, 11 patients with pulmonary hypertension, 4 patients with right ventricular outflow tract obstruction and 4 patients with right ventricular infarction, right ventricular ejection fraction determined by krypton-81m equilibrium blood pool imaging ranged from 14 to 76%. The correlation between these values and those determined by cineangiography according to Simpson's rule was close: r . 0.93 for all data points, r . 0.92 for studies at rest and r . 0.93 for exercise studies. Exercise-related changes in right ventricular function revealed a disturbed functional reserve with pulmonary hypertension and right ventricular infarction, whereas in compensated right ventricular outflow tract obstruction there was a physiologic increase in ejection fraction with exercise. Thus, equilibrium-gated right ventricular imaging using ultrashort-lived krypton-81m is a simple, accurate and reproducible method with potential for serial assessment of right ventricular ejection fraction in a variety of right ventricular anatomic and functional abnormalities, both at rest and during exercise. Advantages of this method include an extremely low radiation dose to patients and clear right atrioventricular separation without the need to correct for background activity

Right ventricular function: methodologic and clinical considerations in noninvasive scintigraphic assessment

International Nuclear Information System (INIS)

Manno, B.V.; Iskandrian, A.S.; Hakki, A.H.

1984-01-01

Right ventricular function plays an important role in many cardiac disorders. Changes in left ventricular function, right ventricular afterload and preload, cardiac medications and ischemia may affect right ventricular function. Radionuclide ventriculography permits quantitative assessment of regional and global function of the right ventricle. This assessment can be made at rest, during exercise or after pharmacologic interventions. The overlap between right ventricle and right atrium is a major limitation for gated scintigraphic techniques. The use of imaging with newer short-lived radionuclides may permit more accurate and reproducible assessment of right ventricular function by means of the first pass method. Further work in areas related to improvement of techniques and the impact of right ventricular function on prognosis is needed

Clinical studies on myocardial perfusion imaging in patients with right ventricular overload

International Nuclear Information System (INIS)

Abo, Kenji; Yamagata, Takashi; Nakajima, Masao; Fujita, Kimiaki; Morita, Nobuo

1979-01-01

Patients with heart disease which had been clinically diagnosed underwent 201 Tl myocardial perfusion imaging. The thickness of right ventricular wall measured from original images was directly proportional to systolic pressure of the right ventricle measured by cardiac catheterization, and 201 Tl activity in the right ventricle was more directly proportional to systolic pressure of the right ventricle. Imaging patterns of various diseases were also described. Images of patients with hypertrophic cardiomyopathy revealed that right ventricular wall was thin and right ventricular cavity was small, but the thickness of septal wall and left ventricular wall were maximal. Images of patients with mitral insufficiency revealed that the thickness of right ventricular wall, septal wall, and left ventricular wall was medium, and the right ventricular cavity was smaller than the left ventricular cavity. Images of patients with congestive cardiomyopathy and congestive cardiac failure showed that enlargement of both ventricular cavities was disproportionate to the thickness of each wall. Images of patients with arterial septal defect revealed that the thickness of each wall was comparatively normal, the right ventricular cavity was maximal, and the left ventricular cavity was minimal. Images of patients with primary pulmonary hypertention, pulmonary stenosis and tetralogy of Fallot in whom pressure overload was recognized revealed severe thickenings of right ventricular wall, moderate enlargement of the right ventricle, small left ventricle, and thin left ventricular wall. (Tsunoda, M.)

Radionuclide analysis of right and left ventricular response to exercise in patients with atrial and ventricular septal defects

International Nuclear Information System (INIS)

Peter, C.A.; Bowyer, K.; Jones, R.H.

1983-01-01

In patients with ventricular or atrial septal defect, the ventricle which is chronically volume overloaded might not appropriately respond to increased demand for an augmentation in output and thereby might limit total cardiac function. In this study we simultaneously measured right and left ventricular response to exercise in 10 normal individuals, 10 patients with ventricular septal defect (VSD), and 10 patients with atrial septal defect (ASD). The normal subjects increased both right and left ventricular ejection fraction, end-diastolic volume, and stroke volume to achieve a higher cardiac output during exercise. Patients with VSD failed to increase right ventricular ejection fraction, but increased right ventricular end-diastolic volume and stroke volume. Left ventricular end-diastolic volume did not increase in these patients but ejection fraction, stroke volume, and forward left ventricular output achieved during exercise were comparable to the response observed in healthy subjects. In the patients with ASD, no rest-to-exercise change occurred in either right ventricular ejection fraction, end-diastolic volume, or stroke volume. In addition, left ventricular end-diastolic volume failed to increase, and despite an increase in ejection fraction, left ventricular stroke volume remained unchanged from rest to exercise. Therefore, cardiac output was augmented only by the heart rate increase in these patients. Right ventricular function appeared to be the major determinant of total cardiac output during exercise in patients with cardiac septal defects and left-to-right shunt

Noninvasive assessment of right ventricular wall motion by radionuclide cardioangiography

International Nuclear Information System (INIS)

Nishimura, Tsunehiko; Uehara, Toshiisa; Naito, Hiroaki; Hayashida, Kohei; Kozuka, Takahiro

1981-01-01

Radionuclide cardioangiography is a useful method to evaluate the left ventricular wall motion in various heart diseases. It has been also attempted to assess the right ventricular wall motion simultaneously by radionuclide method. In this study, using the combination of first-pass (RAO 30 0 ) and multi-gate (LAO 40 0 ) method, the site of right vetricle was classified in five. (1 inflow, 2 sinus, 3 outflow, 4 septal, 5 lateral) and the degree of wall motion was classified in four stages (dyskinesis, akinesis, hypokinesis, normal) according to the AHA committee report. These methods were applied clinically to forty-eight patients with various heart diseases. In the cases with right ventricular pressure or volume overload such as COLD, pulmonary infarction, the right ventricle was dilated and the wall motion was reduced in all portions. Especially, in the cases with right ventricular infarction, the right ventricular wall motion was reduced in the infarcted area. The findings of radionuclide method were in good agreement with those of contrast right ventriculography or echocardiography. In conclusion, radionuclide cardioangiography is a useful and noninvasive method to assess not only the left but also the right ventricular wall motion. (author)

Morning surge of ventricular arrhythmias in a new arrhythmogenic canine model of chronic heart failure is associated with attenuation of time-of-day dependence of heart rate and autonomic adaptation, and reduced cardiac chaos.

Science.gov (United States)

Zhu, Yujie; Hanafy, Mohamed A; Killingsworth, Cheryl R; Walcott, Gregory P; Young, Martin E; Pogwizd, Steven M

2014-01-01

Patients with chronic heart failure (CHF) exhibit a morning surge in ventricular arrhythmias, but the underlying cause remains unknown. The aim of this study was to determine if heart rate dynamics, autonomic input (assessed by heart rate variability (HRV)) and nonlinear dynamics as well as their abnormal time-of-day-dependent oscillations in a newly developed arrhythmogenic canine heart failure model are associated with a morning surge in ventricular arrhythmias. CHF was induced in dogs by aortic insufficiency & aortic constriction, and assessed by echocardiography. Holter monitoring was performed to study time-of-day-dependent variation in ventricular arrhythmias (PVCs, VT), traditional HRV measures, and nonlinear dynamics (including detrended fluctuations analysis α1 and α2 (DFAα1 & DFAα2), correlation dimension (CD), and Shannon entropy (SE)) at baseline, as well as 240 days (240 d) and 720 days (720 d) following CHF induction. LV fractional shortening was decreased at both 240 d and 720 d. Both PVCs and VT increased with CHF duration and showed a morning rise (2.5-fold & 1.8-fold increase at 6 AM-noon vs midnight-6 AM) during CHF. The morning rise in HR at baseline was significantly attenuated by 52% with development of CHF (at both 240 d & 720 d). Morning rise in the ratio of low frequency to high frequency (LF/HF) HRV at baseline was markedly attenuated with CHF. DFAα1, DFAα2, CD and SE all decreased with CHF by 31, 17, 34 and 7%, respectively. Time-of-day-dependent variations in LF/HF, CD, DFA α1 and SE, observed at baseline, were lost during CHF. Thus in this new arrhythmogenic canine CHF model, attenuated morning HR rise, blunted autonomic oscillation, decreased cardiac chaos and complexity of heart rate, as well as aberrant time-of-day-dependent variations in many of these parameters were associated with a morning surge of ventricular arrhythmias.

Morning surge of ventricular arrhythmias in a new arrhythmogenic canine model of chronic heart failure is associated with attenuation of time-of-day dependence of heart rate and autonomic adaptation, and reduced cardiac chaos.

Directory of Open Access Journals (Sweden)

Yujie Zhu

Full Text Available Patients with chronic heart failure (CHF exhibit a morning surge in ventricular arrhythmias, but the underlying cause remains unknown. The aim of this study was to determine if heart rate dynamics, autonomic input (assessed by heart rate variability (HRV and nonlinear dynamics as well as their abnormal time-of-day-dependent oscillations in a newly developed arrhythmogenic canine heart failure model are associated with a morning surge in ventricular arrhythmias. CHF was induced in dogs by aortic insufficiency & aortic constriction, and assessed by echocardiography. Holter monitoring was performed to study time-of-day-dependent variation in ventricular arrhythmias (PVCs, VT, traditional HRV measures, and nonlinear dynamics (including detrended fluctuations analysis α1 and α2 (DFAα1 & DFAα2, correlation dimension (CD, and Shannon entropy (SE at baseline, as well as 240 days (240 d and 720 days (720 d following CHF induction. LV fractional shortening was decreased at both 240 d and 720 d. Both PVCs and VT increased with CHF duration and showed a morning rise (2.5-fold & 1.8-fold increase at 6 AM-noon vs midnight-6 AM during CHF. The morning rise in HR at baseline was significantly attenuated by 52% with development of CHF (at both 240 d & 720 d. Morning rise in the ratio of low frequency to high frequency (LF/HF HRV at baseline was markedly attenuated with CHF. DFAα1, DFAα2, CD and SE all decreased with CHF by 31, 17, 34 and 7%, respectively. Time-of-day-dependent variations in LF/HF, CD, DFA α1 and SE, observed at baseline, were lost during CHF. Thus in this new arrhythmogenic canine CHF model, attenuated morning HR rise, blunted autonomic oscillation, decreased cardiac chaos and complexity of heart rate, as well as aberrant time-of-day-dependent variations in many of these parameters were associated with a morning surge of ventricular arrhythmias.

Videodensitometric assessment of right and left ventricular functions by digital subtraction angiography

International Nuclear Information System (INIS)

Ikeda, Hisao; Yoshiga, Osamu; Shibao, Keigo

1987-01-01

Intravenous digital subtraction (DS) ventriculography was performed in a series of 50 patients with heart diseases to determine right and left ventricular volumes and systolic indices. Right ventricular volume and right ventricular ejection fraction obtained by DS ventriculography were well correlated with those by geometric methods. In 43 patients with left ventricular ejection fraction of 55 % or greater, end-diastolic volume, stroke volume, and ejection fraction in the right ventricle did not differ from those in the left ventricle ; however, both the 1/3 ejection fraction and the peak ejection rate of the right ventricle were significantly lower than those of the left ventricle, suggesting the different modes of left and right ventricular contraction. In the other seven patients with chronic left ventricular failure, right ventricular systolic function may be preserved, even when left ventricular function is severely impaired. Digital subtraction ventriculography has proved to be a simple, useful method in the quanlitative and quantitative assessments of the right and left ventricles. (Namekawa, K.)

Acute Right Ventricular Dysfunction Complicating Prolonged ...

African Journals Online (AJOL)

We report a case of transient right ventricular dysfunction associated with prolonged cardiac tamponade, an unusual complication of uncertain etiology. We believe that in this case dynamic coronary flow restriction resulted in ischemic injury and stunning of the right ventricle. Other possible causes are briefly reviewed. Right ...

Correlation between right ventricular T1 mapping and right ventricular dysfunction in non-ischemic cardiomyopathy.

Science.gov (United States)

Jellis, Christine L; Yingchoncharoen, Teerapat; Gai, Neville; Kusunose, Kenya; Popović, Zoran B; Flamm, Scott; Kwon, Deborah

2018-01-01

Right ventricular (RV) fibrosis is increasingly recognized as the underlying pathological substrate in a variety of clinical conditions. We sought to employ cardiac magnetic resonance (CMR) techniques of strain imaging and longitudinal relaxation time (T 1 ) mapping to better examine the relationship between RV function and structure. Our aim was to initially evaluate the feasibility of these techniques to evaluate the right ventricle. We then sought to explore the relationship between RV function and underlying fibrosis, along with examining the evolution of RV remodeling according to the amount of baseline fibrosis. Echocardiography was performed in 102 subjects with non-ischemic cardiomyopathy. Right ventricular parameters were assessed including: fractional area change (FAC) and longitudinal strain. The same cohort underwent CMR. Post-contrast T 1 mapping was performed as a marker of fibrosis with a Look-Locker technique using inversion recovery imaging. Mid-ventricular post-contrast T 1 values of the RV free wall, RV septum and lateral LV were calculated using prototype analysis software. Biventricular volumetric data including ejection fraction was measured by CMR using a cine short axis stack. CMR strain analysis was also performed to assess 2D RV longitudinal and radial strain. Simultaneous biochemical and anthropometric data were recorded. Subjects were followed over a median time of 29 months (IQR 20-37 months) with echocardiography to evaluate temporal change in RV FAC according to baseline post-contrast T 1 values. Longitudinal data analysis was performed to adjust for patient loss during follow-up. Subjects (62% men, 51 ± 15 years) had mild to moderately impaired global RV systolic function (RVEF = 39 ± 15%; RVEDV = 187 ± 69 ml; RVESV = 119 ± 68 ml) and moderate left ventricular dysfunction at baseline (LVEF 30 ± 17%). Good correlation was observed between mean LV and RV post-contrast T 1 values (r = 0.652, p right

Flow-related Right Ventricular - Pulmonary Arterial Pressure Gradients during Exercise.

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Wright, Stephen P; Opotowsky, Alexander R; Buchan, Tayler A; Esfandiari, Sam; Granton, John T; Goodman, Jack M; Mak, Susanna

2018-06-06

The assumption of equivalence between right ventricular and pulmonary arterial systolic pressure is fundamental to several assessments of right ventricular or pulmonary vascular hemodynamic function. Our aims were to 1) determine whether systolic pressure gradients develop across the right ventricular outflow tract in healthy adults during exercise, 2) examine the potential correlates of such gradients, and 3) consider the effect of such gradients on calculated indices of right ventricular function. Healthy untrained and endurance-trained adult volunteers were studied using right-heart catheterization at rest and during submaximal cycle ergometry. Right ventricular and pulmonary artery pressures were simultaneously transduced, and cardiac output was determined by thermodilution. Systolic pressures, peak and mean gradients, and indices of chamber, vascular, and valve function were analyzed offline. Summary data are reported as mean ± standard deviation or median [interquartile range]. No significant right ventricular outflow tract gradients were observed at rest (mean gradient = 4 [3-5] mmHg), and calculated effective orifice area was 3.6±1.0 cm2. Right ventricular systolic pressure increases during exercise were greater than that of pulmonary artery systolic pressure. Accordingly, mean gradients developed during light exercise (8 [7-9] mmHg) and increased during moderate exercise (12 [9-14] mmHg, p < 0.001). The magnitude of the mean gradient was linearly related to cardiac output (r2 = 0.70, p < 0.001). In healthy adults without pulmonic stenosis, systolic pressure gradients develop during exercise, and the magnitude is related to blood flow rate.

Right ventricular failure after implantation of a continuous-flow left ventricular assist device

DEFF Research Database (Denmark)

Cordtz, Johan Joakim; Nilsson, Jens C; Hansen, Peter B

2014-01-01

Right ventricular failure (RVF) is a significant complication after implantation of a left ventricular assist device. We aimed to identify haemodynamic changes in the early postoperative phase that predicted subsequent development of RVF in a cohort of HeartMate II (HMII) implanted patients....

Right ventricular function during acute exacerbation of severe equine asthma.

Science.gov (United States)

Decloedt, A; Borowicz, H; Slowikowska, M; Chiers, K; van Loon, G; Niedzwiedz, A

2017-09-01

Pulmonary hypertension has been described in horses with severe equine asthma, but its effect on the right ventricle has not been fully elucidated. To evaluate right ventricular structure and function after a 1-week period of pulmonary hypertension secondary to acute exacerbation of severe equine asthma. Prospective study. A clinical episode of severe equine asthma was induced experimentally in six susceptible horses. Examinations in remission and on day 7 of the clinical episode included a physical examination with clinical scoring, echocardiography, arterial blood gas measurements, venous blood sampling for cardiac biomarkers, intracardiac pressure measurements, right ventricular and right atrial myocardial biopsies, airway endoscopy and bronchoalveolar lavage. After 1 month of recovery, physical examination, echocardiography and cardiac biomarker analysis were repeated. Echocardiographic and pressure measurements were compared with those in 10 healthy control horses. All horses developed clinical signs of acute pulmonary obstruction. Right heart pressures increased significantly. Altered right ventricular function could be detected by tissue Doppler and speckle tracking echocardiography. Cardiac troponin concentrations did not increase significantly, but were highly elevated in one horse which exercised in the paddock prior to sampling. Focal neutrophil infiltration was present in two myocardial samples. Even in remission, asthmatic horses showed a thicker right ventricular wall, an increased left ventricular end-systolic eccentricity index at chordal level and decreased right ventricular longitudinal strain compared with controls. The induced clinical episode was rather mild and the number of horses was limited because of the invasive nature of the study. Pulmonary obstruction in asthmatic horses induces pulmonary hypertension with right ventricular structural and functional changes. © 2017 EVJ Ltd.

Severity of structural and functional right ventricular remodeling depends on training load in an experimental model of endurance exercise.

Science.gov (United States)

Sanz-de la Garza, Maria; Rubies, Cira; Batlle, Montserrat; Bijnens, Bart H; Mont, Lluis; Sitges, Marta; Guasch, Eduard

2017-09-01

Arrhythmogenic right ventricular (RV) remodeling has been reported in response to regular training, but it remains unclear how exercise intensity affects the presence and extent of such remodeling. We aimed to assess the relationship between RV remodeling and exercise load in a long-term endurance training model. Wistar rats were conditioned to run at moderate (MOD; 45 min, 30 cm/s) or intense (INT; 60 min, 60 cm/s) workloads for 16 wk; sedentary rats served as controls. Cardiac remodeling was assessed with standard echocardiographic and tissue Doppler techniques, sensor-tip pressure catheters, and pressure-volume loop analyses. After MOD training, both ventricles similarly dilated (~16%); the RV apical segment deformation, but not the basal segment deformation, was increased [apical strain rate (SR): -2.9 ± 0.5 vs. -3.3 ± 0.6 s -1 , SED vs. MOD]. INT training prompted marked RV dilatation (~26%) but did not further dilate the left ventricle (LV). A reduction in both RV segments' deformation in INT rats (apical SR: -3.3 ± 0.6 vs. -3.0 ± 0.4 s -1 and basal SR: -3.3 ± 0.7 vs. -2.7 ± 0.6 s -1 , MOD vs. INT) led to decreased global contractile function (maximal rate of rise of LV pressure: 2.53 ± 0.15 vs. 2.17 ± 0.116 mmHg/ms, MOD vs. INT). Echocardiography and hemodynamics consistently pointed to impaired RV diastolic function in INT rats. LV systolic and diastolic functions remained unchanged in all groups. In conclusion, we showed a biphasic, unbalanced RV remodeling response with increasing doses of exercise: physiological adaptation after MOD training turns adverse with INT training, involving disproportionate RV dilatation, decreased contractility, and impaired diastolic function. Our findings support the existence of an exercise load threshold beyond which cardiac remodeling becomes maladaptive. NEW & NOTEWORTHY Exercise promotes left ventricular eccentric hypertrophy with no changes in systolic or diastolic function in healthy rats. Conversely, right

Dynamic Changes of QRS Morphology of Premature Ventricular Contractions During Ablation in the Right Ventricular Outflow Tract: A Case Report.

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Yue-Chun, Li; Jia-Feng, Lin; Jia-Xuan, Lin

2015-10-01

Electrocardiographic characteristics can be useful in differentiating between right ventricular outflow tract (RVOT) and aortic sinus cusp (ASC) ventricular arrhythmias. Ventricular arrhythmias originating from ASC, however, show preferential conduction to RVOT that may render the algorithms of electrocardiographic characteristics less reliable. Even though there are few reports describing ventricular arrhythmias with ASC origins and endocardial breakout sites of RVOT, progressive dynamic changes in QRS morphology of the ventricular arrhythmias during ablation obtained were rare.This case report describes a patient with symptomatic premature ventricular contractions of left ASC origin presenting an electrocardiogram (ECG) characteristic of right ventricular outflow tract before ablation. Pacing at right ventricular outflow tract reproduced an excellent pace map. When radiofrequency catheter ablation was applied to the right ventricular outflow tract, the QRS morphology of premature ventricular contractions progressively changed from ECG characteristics of right ventricular outflow tract origin to ECG characteristics of left ASC origin.Successful radiofrequency catheter ablation was achieved at the site of the earliest ventricular activation in the left ASC. The distance between the successful ablation site of the left ASC and the site with an excellent pace map of the RVOT was 20 mm.The ndings could be strong evidence for a preferential conduction via the myocardial bers from the ASC origin to the breakout site in the right ventricular outflow tract. This case demonstrates that ventricular arrhythmias with a single origin and exit shift may exhibit QRS morphology changes.

Right ventricular myxoma with heart failure

International Nuclear Information System (INIS)

Nuri, M.M.H.; Naseem, A.; Raza, A.; Ahmad, W.; Raja, M.K.; Ahmad, A.; Rasheed, A.; Gohar, A.

2005-01-01

Cardiac myxomas are histologically benign tumors but they may be lethal because of their strategic position. These are mostly located in the left atrium and uncommonly in the right atrium. We report a case of a large right ventricular myxoma presenting with heart failure. (author)

Right Ventricular Ejection Fraction using ECG-Gated First Pass Cardioangiography

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Moon, Young Hee; Lee, Hae Giu; Lee, Sung Yong; Park, Suk Min; Chung, Soo Kyo; Yim, Jeong Ik; Bahk, Yong Whee; Shinn, Kyung Sub; Kim, Young Gyun; Kwon, Soon Seog [Catholic University College of Medicine, Seoul (Korea, Republic of)

1993-03-15

Radionuclide cardioangiography has been widely applied and has played major roles in moninvasive assessment of cardiac function. Three techniques, first-pass gated first and gated equilibrium methods have commonly been used to evaluate right ventricular ejection fraction which usually abnormal in the patients with cardiopulmonary disease. It has been known that the gated first pass method is most accurate method among the three techniques in assessment of fight ventricular ejection fraction. The radionuclide right ventricular ejection fraction values were determined in 13 normal subjects and in 15 patients with chronic obstructive pulmonary disease by the gated first pass method and compared with those of the first pass method because there has been no published data of fight ejection fraction by the gated first pass method were compared with the defects from the pulmonary function test performed in the patients with chronic obstructive pulmomary disease. The results were as follows; 1) The values of fight ventricular ejection fraction by the gated first pass method were 50.1 +- 6.1% in normal subjects and 38.5 +- 8.5 in the patients with chronic obstructive pulmonary disease. There was statistically significant difference between the right ventricular ejection fraction of each of the two groups (p<0.05) 2) The right ventricular ejection fraction by the gated first pass method was not linearly correlated ith FEV{sub 1}, VC. DLCO. and FVC as well as P{sub a}O2 and P{sub a}CO2 of the patients with chronic obstructive pulmonary disease. We concluded that right ventricular ejection fraction by the gated first pass method using radionuclide cardioangiography may be useful in clinical assessment of the right ventricular function.

Impact of Major Pulmonary Resections on Right Ventricular Function: Early Postoperative Changes.

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Elrakhawy, Hany M; Alassal, Mohamed A; Shaalan, Ayman M; Awad, Ahmed A; Sayed, Sameh; Saffan, Mohammad M

2018-01-15

Right ventricular (RV) dysfunction after pulmonary resection in the early postoperative period is documented by reduced RV ejection fraction and increased RV end-diastolic volume index. Supraventricular arrhythmia, particularly atrial fibrillation, is common after pulmonary resection. RV assessment can be done by non-invasive methods and/or invasive approaches such as right cardiac catheterization. Incorporation of a rapid response thermistor to pulmonary artery catheter permits continuous measurements of cardiac output, right ventricular ejection fraction, and right ventricular end-diastolic volume. It can also be used for right atrial and right ventricular pacing, and for measuring right-sided pressures, including pulmonary capillary wedge pressure. This study included 178 patients who underwent major pulmonary resections, 36 who underwent pneumonectomy assigned as group (I) and 142 who underwent lobectomy assigned as group (II). The study was conducted at the cardiothoracic surgery department of Benha University hospital in Egypt; patients enrolled were operated on from February 2012 to February 2016. A rapid response thermistor pulmonary artery catheter was inserted via the right internal jugular vein. Preoperatively the following was recorded: central venous pressure, mean pulmonary artery pressure, pulmonary capillary wedge pressure, cardiac output, right ventricular ejection fraction and volumes. The same parameters were collected in fixed time intervals after 3 hours, 6 hours, 12 hours, 24 hours, and 48 hours postoperatively. For group (I): There were no statistically significant changes between the preoperative and postoperative records in the central venous pressure and mean arterial pressure; there were no statistically significant changes in the preoperative and 12, 24, and 48 hour postoperative records for cardiac index; 3 and 6 hours postoperative showed significant changes. There were statistically significant changes between the preoperative and

Dilemma in clinical diagnosis of right ventricular masses.

Science.gov (United States)

Sušić, Livija; Baraban, Vedrana; Vincelj, Josip; Maričić, Lana; Ćatić, Jasmina; Blažeković, Robert; Manojlović, Spomenka

2017-07-08

Detection of an intracardiac mass always represents a clinical challenge. We present a 61-year-old female patient with symptoms of New York Heart Association class III. Two-dimensional transthoracic echocardiography revealed a hypoechogenic mass in the cavity of the dilated right ventricle (RV). Cardiac MRI described a pathologic structure of the RV free wall with pedunculated tumor in its cavity. Three months later, on a repeated echocardiography, there were three individual masses. The patient underwent surgery and the pathohistologic report demonstrated thrombotic masses. During the postoperative period, after reviewing all medical records, the conclusion was arrhythmogenic RV cardiomyopathy. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 45:362-369, 2017. © 2016 Wiley Periodicals, Inc.

Stunning and Right Ventricular Dysfunction Is Induced by Coronary Balloon Occlusion and Rapid Pacing in Humans: Insights From Right Ventricular Conductance Catheter Studies.

Science.gov (United States)

Axell, Richard G; Giblett, Joel P; White, Paul A; Klein, Andrew; Hampton-Til, James; O'Sullivan, Michael; Braganza, Denise; Davies, William R; West, Nick E J; Densem, Cameron G; Hoole, Stephen P

2017-06-06

We sought to determine whether right ventricular stunning could be detected after supply (during coronary balloon occlusion [BO]) and supply/demand ischemia (induced by rapid pacing [RP] during transcatheter aortic valve replacement) in humans. Ten subjects with single-vessel right coronary artery disease undergoing percutaneous coronary intervention with normal ventricular function were studied in the BO group. Ten subjects undergoing transfemoral transcatheter aortic valve replacement were studied in the RP group. In both, a conductance catheter was placed into the right ventricle, and pressure volume loops were recorded at baseline and for intervals over 15 minutes after a low-pressure BO for 1 minute or a cumulative duration of RP for up to 1 minute. Ischemia-induced diastolic dysfunction was seen 1 minute after RP (end-diastolic pressure [mm Hg]: 8.1±4.2 versus 12.1±4.1, P right coronary artery balloon occlusion both cause ischemic right ventricular dysfunction with stunning observed later during the procedure. This may have intraoperative implications in patients without right ventricular functional reserve. © 2017 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.

Independence of intrapericardial right and left ventricular performance in septic pulmonary hypertension

International Nuclear Information System (INIS)

Boeck, J.C.; Eichstaedt, H.; Barker, B.C.; Lewis, F.R.; Lim, A.D.; Pollycove, M.

1990-01-01

To study the effect of septic pulmonary hypertension on right/left ventricular intrapericardial interactions thirteen trauma patients, seven septic and six nonseptic controls, were compared. Ventricular volumes were derived from firstpass or gated equilibrium radionuclide angiocardiography, and related to body surface area. Systemic and pulmonary pressures were measured invasively. Pulmonary arterial pressure was significantly increased in the sepsis group. Although right ventricular end-diastolic volumes were higher in sepsis, left ventricular end-diastolic volumes were not decreased. In terms of intrapericardial right/left ventricular interactions these results indicate that the right and left ventricles operate independently in septic pulmonary hypertension. (orig.) [de

Clinical application of a right ventricular pressure-volume loop determined by gated blood-pool imaging and simultaneously measured right ventricular pressure

International Nuclear Information System (INIS)

Yasue, Takao; Watanabe, Sachiro; Sugishita, Nobuyoshi; Tanaka, Tsutomu; Yokoyama, Hideo

1983-01-01

The data obtained by ECG-gated radionuclide angiography were collected simultaneously with right ventricular pressure and thermal cardiac output (CO) obtained by a Swan-Ganz catheter in Scintipac 1200 (Shimazu Co) in order to create a right ventricular pressure-volume (RV P-V) loop. Subjects consisted of 15 patients with old myocardial infarction (MI group), seven with angina pectoris (AP group), six with congestive cardiomyopathy (CCM group) and five with neurocirculatory asthenia (NCA group). Right ventricular end-diastolic volume (RVEDV) was calculated as RVEDV = CO/(EF x HR) (CO = cardiac output; HR = heart rate). Systolic work (W sub(S)), diastolic work (W sub(D)) and net work (W sub(N)) were calculated from a RV P-V loop by Simpson's method. The measurements were performed before and 5 min after sublingual administration of nitroglycerin (NG) (0.3 mg). The results were as follows: 1. RV P-V loops shifted towards the left lower part of the P-V plane after sublingual administration of nitroglycerin, indicating the reduction of pressure and volume of the right ventricle. 2. Right ventricular ejection fraction (RVEF) in the MI, AP and CCM groups showed smaller values than that of the NCA group. 3. Right ventricular end-diastolic volume index (RVEDVI) showed a converse relation with RVEF. 4. Cardiac index in all groups decreased after NG and a statistical significance was seen in the MI, AP and NCA groups (p<0.05). 5. RV W sub(S), RV W sub(D) and RV W sub(N) showed no difference among each groups in the control state, and significantly decreased after NG. We conclude that the present method using RV P-V loop might be useful as a noninvasive bedside monitoring and permits the evaluation of RV function in a clinical setting

An Incidentally Detected Right Ventricular Pseudoaneurysm

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Vamsi C. Gaddipati

2017-01-01

Full Text Available Ventricular pseudoaneurysm is an uncommon, potentially fatal complication that has been associated with myocardial infarction, cardiac surgery, chest trauma, and infectious processes. Diagnosis can be challenging, as cases are rare and slowly progressing and typically lack identifiable features on clinical presentation. As a result, advanced imaging techniques have become the hallmark of identification. Ahead, we describe a patient who presents with acute decompensated heart failure and was incidentally discovered to have a large right ventricular pseudoaneurysm that developed following previous traumatic anterior rib fracture.

Limitations and pitfalls in measurements of right ventricular stroke volume in an animal model of right heart failure

International Nuclear Information System (INIS)

Vildbrad, Mads Dam; Andersen, Asger; Andersen, Thomas Krarup; Axelgaard, Sofie; Holmboe, Sarah; Andersen, Stine; Nielsen-Kudsk, Jens Erik; Ringgaard, Steffen

2015-01-01

Right heart failure occurs in various heart and pulmonary vascular diseases and may be fatal. We aimed to identify limitations in non-invasive measurements of right ventricular stroke volume in an animal model of right ventricular failure. Data from previous studies randomising rats to pulmonary trunk banding (PTB, n = 33) causing pressure-overload right ventricular failure or sham operation (n = 16) was evaluated retrospectively. We measured right ventricular stroke volume by high frequency echocardiography and magnetic resonance imaging (MRI). We found correlation between right ventricular stroke volume measured by echocardiography and MRI in the sham animals (r = 0.677, p = 0.004) but not in the PTB group. Echocardiography overestimated the stroke volume compared to MRI in both groups. Intra- and inter-observer variation did not explain the difference. Technical, physiological and anatomical issues in the pulmonary artery might explain why echocardiography over-estimates stroke volume. Flow acceleration close to the pulmonary artery banding can cause uncertainties in the PTB model and might explain the lack of correlation. In conclusion, we found a correlation in right ventricular stroke volume measured by echocardiography versus MRI in the sham group but not the PTB group. Echocardiography overestimated right ventricular stroke volume compared to MRI. (paper)

The evaluation of right ventricular performance using krypton-81m

International Nuclear Information System (INIS)

Ham, H.R.; Piepsz, A.; Vandevivere, J.; Guillaume, M.; Goethals, P.; Lenaers, A.

1983-01-01

The availability of krypton-81m suitable for intravenous injection provides an easy means for assessing right ventricular function. The superimposition of the other heart chambers does not constitute a problem. The activity in the left heart is negligible and the study can be performed in the right anterior oblique position to obtain optimal separation between the right atrium and the right ventricle. Lung activity is quite high and should be corrected before computing the right ventricular ejection fraction. This correction can be accomplished by performing Tc-99m MAA perfusion scintigraphy. The measurement of the right ventricular ejection fraction appears to be reproducible with very small inter-and intraobserver variability. Good quality phase and amplitude images can be obtained with such high count rates

The right ventricular response to ventricular hypofunction in anteroseptal infarction

International Nuclear Information System (INIS)

Kanayama, Sugako

1992-01-01

Thirty-seven patients with acute anteroseptal infarction but not significant right coronary artery stenosis were examined by using thallium-201 (Tl-201) myocardial perfusion SPECT to determine how the right ventricular (RV) free wall responded to a severely impaired ventricular septum. The patients were divided into the group in which RV free wall was visualized on Tl-201 myocardial SPECT (n=19, RV(+) Group) and the group in which it was not visualized (n=18, RV(-) Group). The relationship between visualization of RV free wall and both RV and left ventricular (LV) function was evaluated. RV(+) Group had larger extent of anteroseptal necrosis and severer impairment of RV free wall, as compared with RV(-) Group. LV ejection fraction (LVEF) was significantly lower in RV(+) Group than RV(-) Group in both acute and chronic phases. Although RV ejection fraction (RVEF) in acute phase was significantly lower in RV(+) Group than RV(-) Group, it did not differ in chronic phase between the two groups. In RV(+) Group, RV stroke work index (RVSWI), pulmonary artery end diastolic pressure (PAEDP), and mean pulmonary artery pressure (MPA) in chronic phase showed a statistically significant increase compared with those in acute phase; these hemodynamic variables in chronic phase were also significantly higher than those in RV(-) Group. RV/LV ratio inversely correlated with LVEF, and both necrotic extent and impairment severity positively correlated with both PAEDP and MPA. RV free wall could be visualized more clearly, corresponding to extremely decreased LV function. These findings suggest that RV free wall may play an important role in maintaining LV and RV function when ventricular septum is severely impaired by anteroseptal infarction. (N.K.)

Effect of right ventricular electrode location (outflow tract vs. apex) on mechanical Ventricular synchrony in patients that underwent pacemaker implant therapy

International Nuclear Information System (INIS)

Rincon, Oscar S; Saenz, Luis C; Salazar, Gabriel; Hernandez, Edgar

2008-01-01

Objective: to assess in depth the effect of ventricular stimulation from the right ventricular outflow tract and the apex on mechanical ventricular synchrony. Materials And Methods: cohort analytical study. 20 patients with indication of definitive pacemaker indication underwent trans thoracic echocardiogram before and after pacemaker implant with electrode implantation in the right ventricular outflow tract and in the apex (10 patients in each group). There was no structural cardiopathy, ejection fraction was ? 50%, QRS and AV conduction were normal. Mechanical ventricular asynchrony (M mode and tissue doppler) and implant and device parameters were evaluated. Statistical Analysis: results are given as mean values, standard deviation or percentages.Continuous variables were compared using Chi-square test and ANOVA. A p <0.05 value was considered statistically significant. Results: in five patients (25%) a pre-implant ventricular asynchrony was found; in seven (70%) ventricular asynchrony post-implant in the right ventricle outflow tract and in 5 (50%) in the apex. Mean interventricular pot-implant delay was 21,6 ms in the right ventricular outflow tract and 11,5 ms in the apex (p = 0,8); mean septal to lateral wall delay was 73 ms in the right ventricular outflow tract and 26 ms in the apex (p = 0,8). QRS post-implant delay was 134 ms in the right ventricular outflow tract and 140 ms in the apex (p = 0,1). No differences between implant parameters and device programming were found. Conclusions: presence of ventricular asynchrony was evidenced in patients with normal QRS and structurally healthy heart. Ventricular stimulation with pacemaker from the apex or the right ventricular outflow tract suggests acute ventricular asynchrony at least in 60% of the cases, without statistically significant difference between both groups.

Right ventricular systolic and diastolic function at rest in patients with coronary artery disease

International Nuclear Information System (INIS)

Caglar, N.M.; Araki, Haruo; Taira, Yuji; Fukuyama, Takaya; Nakamura, Motoomi

1985-01-01

Right ventricular systolic and diastolic function was studied in patients with ischemic heart disease using equilibrium radionuclide ventriculography. In patients with inferior myocardial infarction and proximal right coronary lesions, the right ventricular ejection fraction (0.43+-0.06, n=10, mean+-SD) and peak filling rate (1.7+-0.4 EDV/sec) were lower than normals (0.57+-0.07 and 2.7+-0.4 EDV/sec, n=10, p<0.001, respectively). In these patients, the right ventricular time to peak filling rate was longer than in normals (225+-36 msec vs 136+-45 msec, p<0.001), while the left ventricular ejection fraction remained normal. In patients with inferior myocardial infarction and distal right coronary lesions, the right ventricular ejection fraction, peak filling rate and time to peak filling rate were not different from those in normals. Even in patients with proximal right coronary lesions, the right ventricular ejection fraction was normal unless they had an inferior myocardial infarction. A decreased left ventricular ejection fraction and abnormal motion of the ventricular septum did not affect the right ventricular ejection fraction. The present results suggest that patients with an inferior myocardial infarction and proximal right coronary lesion often develop right ventricular systolic and diastolic dysfunction. (author)

Right Ventricular Hemodynamics in Patients with Pulmonary Hypertension

Science.gov (United States)

Browning, James; Fenster, Brett; Hertzberg, Jean; Schroeder, Joyce

2012-11-01

Recent advances in cardiac magnetic resonance imaging (CMR) have allowed for characterization of blood flow in the right ventricle (RV), including calculation of vorticity and circulation, and qualitative visual assessment of coherent flow patterns. In this study, we investigate qualitative and quantitative differences in right ventricular hemodynamics between subjects with pulmonary hypertension (PH) and normal controls. Fifteen (15) PH subjects and 10 age-matched controls underwent same day 3D time resolved CMR and echocardiography. Echocardiography was used to determine right ventricular diastolic function as well as pulmonary artery systolic pressure (PASP). Velocity vectors, vorticity vectors, and streamlines in the RV were visualized in Paraview and total RV Early (E) and Atrial (A) wave diastolic vorticity was quantified. Visualizations of blood flow in the RV are presented for PH and normal subjects. The hypothesis that PH subjects exhibit different RV vorticity levels than normals during diastole is tested and the relationship between RV vorticity and PASP is explored. The mechanics of RV vortex formation are discussed within the context of pulmonary arterial pressure and right ventricular diastolic function coincident with PH.

Right ventricular function in asthmatic children determined with PW-TDI

International Nuclear Information System (INIS)

Wang Shaona; Song Lili; Cong Lin; Li Liping; Huang Yanxia

2007-01-01

Objective: To assess the right ventricular function of asthmatic children in onset period or remission period by using PW-TDI. Method: 36 children with confirmed asthma were enrolled. In tricuspid annular, the ECHO were recorded with PW-PDI pattern to obtain fight ventricular function (Sm, e-peak, Vmax for a-peak, and e/a), ICT, ET, IRT and Tei-Index. These were carried out in acute attack period and stable period. Result: Right ventficular function parameter (Sm, Vmax for e-peak, Vmax for a-peak, e/a and Tei-Index) in acute attack period were significant different compared to those in stable period (P<0.05). Conclusions: The fight ventricular function was impaired in patients with asthma in acute attack period. PW-TDI is helpful for assessing right ventricular function to asthmatic children. (authors)

Repaired tetralogy of Fallot with coexisting unrepaired partial anomalous pulmonary venous connection is associated with diminished right ventricular ejection fraction and more severe right ventricular dilation

International Nuclear Information System (INIS)

Chan, Sherwin S.; Whitehead, Kevin K.; Kim, Timothy S.; Fu, Gregory L.; Fogel, Mark A.; Harris, Matthew A.; Keller, Marc S.

2015-01-01

There is an established association between tetralogy of Fallot and partial anomalous pulmonary venous connections. This association is important because surgically repaired tetralogy patients have increased risk of right heart failure. We hypothesize that partial anomalous venous connections increase right ventricular volumes and worsen right ventricular failure. We reviewed cardiac MRI exams performed at a tertiary pediatric hospital from January 2005 to January 2014. We identified patients with repaired tetralogy and unrepaired partial anomalous pulmonary venous connection. We used age- and gender-matched repaired tetralogy patients without partial anomalous pulmonary venous connection as controls. We analyzed the MRI results and surgical course and performed comparative statistics to identify group differences. There were eight patients with repaired tetralogy and unrepaired partial anomalous pulmonary venous connection and 16 controls. In all cases, the partial anomalous pulmonary venous connection was not detected on preoperative echocardiography. There were no significant differences in surgical course and body surface area between the two groups. Repaired tetralogy patients with unrepaired partial anomalous pulmonary venous connection showed significantly higher indexed right ventricular end diastolic volume (149 ± 33 mL/m 2 vs. 118 ± 30 mL/m 2 ), right ventricle to left ventricle size ratios (3.1 ± 1.3 vs. 1.9 ± 0.5) and a higher incidence of reduced right ventricular ejection fraction compared to controls (3/8 vs. 0/16). Repaired tetralogy of Fallot with unrepaired partial anomalous pulmonary venous connection is associated with reduced right ventricular ejection fraction and more significant right ventricular dilation. (orig.)

Repaired tetralogy of Fallot with coexisting unrepaired partial anomalous pulmonary venous connection is associated with diminished right ventricular ejection fraction and more severe right ventricular dilation

Energy Technology Data Exchange (ETDEWEB)

Chan, Sherwin S. [Children' s Mercy Hospital and Clinics, Department of Radiology, Kansas City, MO (United States); Whitehead, Kevin K.; Kim, Timothy S.; Fu, Gregory L.; Fogel, Mark A.; Harris, Matthew A. [Children' s Hospital of Philadelphia, Department of Cardiology, Philadelphia, PA (United States); Keller, Marc S. [Children' s Hospital of Philadelphia, Department of Radiology, Philadelphia, PA (United States)

2015-09-15

There is an established association between tetralogy of Fallot and partial anomalous pulmonary venous connections. This association is important because surgically repaired tetralogy patients have increased risk of right heart failure. We hypothesize that partial anomalous venous connections increase right ventricular volumes and worsen right ventricular failure. We reviewed cardiac MRI exams performed at a tertiary pediatric hospital from January 2005 to January 2014. We identified patients with repaired tetralogy and unrepaired partial anomalous pulmonary venous connection. We used age- and gender-matched repaired tetralogy patients without partial anomalous pulmonary venous connection as controls. We analyzed the MRI results and surgical course and performed comparative statistics to identify group differences. There were eight patients with repaired tetralogy and unrepaired partial anomalous pulmonary venous connection and 16 controls. In all cases, the partial anomalous pulmonary venous connection was not detected on preoperative echocardiography. There were no significant differences in surgical course and body surface area between the two groups. Repaired tetralogy patients with unrepaired partial anomalous pulmonary venous connection showed significantly higher indexed right ventricular end diastolic volume (149 ± 33 mL/m{sup 2} vs. 118 ± 30 mL/m{sup 2}), right ventricle to left ventricle size ratios (3.1 ± 1.3 vs. 1.9 ± 0.5) and a higher incidence of reduced right ventricular ejection fraction compared to controls (3/8 vs. 0/16). Repaired tetralogy of Fallot with unrepaired partial anomalous pulmonary venous connection is associated with reduced right ventricular ejection fraction and more significant right ventricular dilation. (orig.)

SR Ca2+-leak and disordered excitation-contraction coupling as the basis for arrhythmogenic and negative inotropic effects of acute ethanol exposure.

Science.gov (United States)

Mustroph, Julian; Wagemann, Olivia; Lebek, Simon; Tarnowski, Daniel; Ackermann, Jasmin; Drzymalski, Marzena; Pabel, Steffen; Schmid, Christof; Wagner, Stefan; Sossalla, Samuel; Maier, Lars S; Neef, Stefan

2018-03-01

Ethanol has acute negative inotropic and arrhythmogenic effects. The underlying mechanisms, however, are largely unknown. Sarcoplasmic reticulum Ca 2+ -leak is an important mechanism for reduced contractility and arrhythmias. Ca 2+ -leak can be induced by oxidative stress and Ca 2+ /Calmodulin-dependent protein kinase II (CaMKII). Therefore, we investigated the influence of acute ethanol exposure on excitation-contraction coupling in atrial and ventricular cardiomyocytes. Isolated human atrial and murine atrial or ventricular cardiomyocytes were preincubated for 30 min and then superfused with control solution or solution containing ethanol. Ethanol had acute negative inotropic and positive lusitropic effects in human atrial muscle strips and murine ventricular cardiomyocytes. Accordingly, Ca 2+ -imaging indicated lower Ca 2+ -transient amplitudes and increased SERCA2a activity, while myofilament Ca 2+ -sensitivity was reduced. SR Ca 2+ -leak was assessed by measuring Ca 2+ -sparks. Ethanol induced severe SR Ca 2+ -leak in human atrial cardiomyocytes (calculated leak: 4.60 ± 0.45 mF/F 0 vs 1.86 ± 0.26 in control, n ≥ 80). This effect was dose-dependent, while spontaneous arrhythmogenic Ca 2+ -waves increased ~5-fold, as investigated in murine cardiomyocytes. Delayed afterdepolarizations, which can result from increased SR Ca 2+ -leak, were significantly increased by ethanol. Measurements using the reactive oxygen species (ROS) sensor CM-H 2 DCFDA showed increased ROS-stress in ethanol treated cells. ROS-scavenging with N-acetylcysteine prevented negative inotropic and positive lusitropic effects in human muscle strips. Ethanol-induced Ca 2+ -leak was abolished in mice with knockout of NOX2 (the main source for ROS in cardiomyocytes). Importantly, mice with oxidation-resistant CaMKII (Met281/282Val mutation) were protected from ethanol-induced Ca 2+ -leak. We show for the first time that ethanol acutely induces strong SR Ca 2+ -leak, also altering

Right ventricular function after acute myocardial infarction: dependence upon infarct related coronary artery

International Nuclear Information System (INIS)

Cho, Ihn Ho; Chun, Kyung A; Won, Kyu Chang; Lee, Hyung Woo; Hong, Geu Ru; Park, Jong Seon; Shin, Dong Gu; Kim, Young Jo; Shim, Bong Sub

2004-01-01

We studied to know the relation between right ventricular function and infarct-related artery after acute myocardial infarction. The right and left ventricular function after a first myocardial infarction was assessed ECG-gated blood pool single photon emission computed tomography (GBPS) algorithms (Cedars-Sinai Medical Center, Los Angels, Calif) (12 after LAD related infarction (group 1) and 15 after RCA related infarction (group 2)). The left ventricular ejection fraction, end-diastolic volume and end-systolic volume did not differ significantly between two groups( group 1 vs 2 :LVEF 50.8% vs 55.1%. LVEDV=73.2 vs 79.7 ml, LVESV=38 vs 44 ml : P>0.05), but right ventricular ejection fraction, end-diastolic volume and end-systolic volume were significantly different after anterior myocardial infarction between two groups( group 1 vs 2 : RVEF=57.3% vs 46.3%. RVEDV=56.4 vs 95.1 ml, RVESV=25.6 vs 54.6ml : P<0.05). There was evidence of right ventricular dilatation in the group with RCA related infarction. Six with inferior infarction had abnormal right ventricular ejection fractions (< 40%). The relation between right and left ventricular ejection fractions was markedly different in the two groups. In the group with RCA related infarction there was a significant linear relation between right and left ventricular ejection fraction(R=0.5). Whereas in the group with LAD related infarction there was not (R=-0.3). Thus right ventricular dysfunction commonly occurs after RCA related infarction. Right ventricular impairment is related after RCA related infarction, but are independent after LAD related infarction. Finally, the different effects of LAD and RCA related infarction on right ventricular function may be explained by site of the myocardial wall involvement after infarction

Measurement of right ventricular volumes using 131I-MAA

International Nuclear Information System (INIS)

Sekimoto, T.; Grover, R.F.

1975-01-01

A method is presented for determining the right ventricular residual ratio, that is, the ratio of the end-systolic volume to the end-diastolic volume during each cardiac cycle. 131 I-MAA was injected as a bolus into the right ventricle, and the ratio of isotope remaining in the chamber during the succeeding cardiac cycles was determined with a collimated scintillation counter placed over the right ventricle. Since the counter detected the radioactivity from the entire right ventricular cavity, potential errors from incomplete mixing were minimized. The washout curve from the ventricle was distorted somewhat by the accumulation of isotope in intervening lung tissue. This distortion was eliminated by subtracting the build-up curve of radioactivity in the lung recorded simultaneously with a second scintillation counter positioned over the lateral chest wall. In 14 dogs anesthetized with chloralose, the right ventricular residual ratio was relatively constant at 40.4 +- 3.1 per cent. Duplicate measurements differed by less than 3 percent indicating the good reproducibility of the method. Right ventricular stroke volume was determined from cardiac output (dye dilution) and heart rate. With this and the simultaneously determined residual ratio ( 131 I-MAA), end-diastolic volume could be calculated. Stroke volume and stroke work were highly correlated with end-diastolic volume, in keeping with the Frank-Starling mechanism. (U.S.)

[Tricuspid insufficiency and right traumatic ventricular aneurysm. Apropos of a case].

Science.gov (United States)

Boisselier, P; Lombaert, M; Rey, J L; Quiret, J C; Bernasconi, P

1981-12-01

Tricuspid incompetence associated with a right ventricular aneurysm wa discovered after a non-penetrating thoracic injury. The severity of the tricuspid lesion was confirmed by phonomechanography, catheterisation and angiography. The mechanism was demonstrated by two-dimensional echocardiography: the right ventricular aneurysm was located in the right ventricular outflow tract. As the hemodynamic tolerance was good, surgery was not performed. A review of the literature found 41 other reports of traumatic tricuspid incompetence, and 4 cases of right ventricular aneurysm, only one of which was associated with tricuspid regurgitation. The points of interest of ths case: the rarity of the association, the good hemodynamic tolerance and the value of two-dimensional echocardiography for the diagnosis of ruptured chordae in the absence of surgical observations.

Left Ventricular Function Improves after Pulmonary Valve Replacement in Patients with Previous Right Ventricular Outflow Tract Reconstruction and Biventricular Dysfunction

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Kane, Colin; Kogon, Brian; Pernetz, Maria; McConnell, Michael; Kirshbom, Paul; Rodby, Katherine; Book, Wendy M.

2011-01-01

Congenital heart defects that have a component of right ventricular outflow tract obstruction, such as tetralogy of Fallot, are frequently palliated in childhood by disruption of the pulmonary valve. Although this can provide an initial improvement in quality of life, these patients are often left with severe pulmonary valve insufficiency. Over time, this insufficiency can lead to enlargement of the right ventricle and to the deterioration of right ventricular systolic and diastolic function. Pulmonary valve replacement in these patients decreases right ventricular volume overload and improves right ventricular performance. To date, few studies have examined the effects of pulmonary valve replacement on left ventricular function in patients with biventricular dysfunction. We sought to perform such an evaluation. Records of adult patients who had undergone pulmonary valve replacement from January 2003 through November 2006 were analyzed retrospectively. We reviewed preoperative and postoperative echocardiograms and calculated left ventricular function in 38 patients. In the entire cohort, the mean left ventricular ejection fraction increased by a mean of 0.07 after pulmonary valve replacement, which was a statistically significant change (P < 0.01). In patients with preoperative ejection fractions of less than 0.50, mean ejection fractions increased by 0.10. We conclude that pulmonary valve replacement in patients with biventricular dysfunction arising from severe pulmonary insufficiency and right ventricular enlargement can improve left ventricular function. Prospective studies are needed to verify this finding. PMID:21720459

The use of cell-sheet technique eliminates arrhythmogenicity of skeletal myoblast-based therapy to the heart with enhanced therapeutic effects.

Science.gov (United States)

Narita, Takuya; Shintani, Yasunori; Ikebe, Chiho; Kaneko, Masahiro; Harada, Narumi; Tshuma, Nomathamsanqa; Takahashi, Kunihiko; Campbell, Niall G; Coppen, Steven R; Yashiro, Kenta; Sawa, Yoshiki; Suzuki, Ken

2013-09-20

Clinical application of skeletal myoblast transplantation has been curtailed due to arrhythmogenicity and inconsistent therapeutic benefits observed in previous studies. However, these issues may be solved by the use of a new cell-delivery mode. It is now possible to generate "cell-sheets" using temperature-responsive dishes without artificial scaffolds. This study aimed to validate the safety and efficacy of epicardial placement of myoblast-sheets (myoblast-sheet therapy) in treating heart failure. After coronary artery ligation in rats, the same numbers of syngeneic myoblasts were transplanted by intramyocardial injection or cell-sheet placement. Continuous radio-telemetry monitoring detected increased ventricular arrhythmias, including ventricular tachycardia, after intramyocardial injection compared to the sham-control, while these were abolished in myoblast-sheet therapy. This effect was conjunct with avoidance of islet-like cell-cluster formation that disrupts electrical conduction, and with prevention of increased arrhythmogenic substrates due to exaggerated inflammation. Persistent ectopic donor cells were found in the lung only after intramyocardial injection, strengthening the improved safety of myoblast-sheet therapy. In addition, myoblast-sheet therapy enhanced cardiac function, corresponding to a 9.2-fold increase in donor cell survival, compared to intramyocardial injection. Both methods achieved reduced infarct size, decreased fibrosis, attenuated cardiomyocyte hypertrophy, and increased neovascular formation, in association with myocardial upregulation of a group of relevant molecules. The pattern of these beneficial changes was similar between two methods, but the degree was more substantial after myoblast-sheet therapy. The cell-sheet technique enhanced safety and therapeutic efficacy of myoblast-based therapy, compared to the current method, thereby paving the way for clinical application. Copyright © 2012 Elsevier Ireland Ltd. All rights

Assessment of 123I-β-methyl iodophenyl pentadecanoic acid (BMIPP) myocardial scintigraphy in patients of chronic right ventricular overload. Fatty acid metabolism in right ventricular myocardium

International Nuclear Information System (INIS)

Mutoh, Hiroshi

1997-01-01

An investigation on the right ventricular pressure level and the abnormalities in the fatty acid metabolism of myocardium was made using 123 I-βmethyl-iodophenyl pentadecanoic acid (BMIPP) myocardial SPECT in patients with chronic right ventricular overloading. Twenty patients who presented with right ventricular systolic pressure (RVSP) of 35 mmHg or more were used as the subjects. Dual myocardial SPECT with 201 TlCl (Tl) and BMIPP was carried out for the subjects and RVc/LVc, a ratio of radioactivity count incorporated in the right ventricular free wall to the left one was determined for Tl and BMIPP. And the correlations between RVc/LVc and RVSP, and RVc/LVc and RVSP/LVSP were examined. The subjects were classified into 3 groups based on the RVSP levels and the count ratio, BMIPP/Tl was compared among the three groups. With respect of Tl uptake, there were significant, positive correlations between RVc/LVc and RVSP (correlation coefficient r=0.51, p<0.05) and between RVc/LVc and RVSP/LVSP (correlation coefficient r=0.59, p<0.01). On the other hand, no significant correlation was found between them with respect of the uptake of BMIPP. The BMIPP/Tl ratio in the group with higher than 80 mmHg of RVSP was 0.82±0.06, which was significantly lower than the ratio's for two groups of less than 80 mmHg; 0.91±0.07 and 0.98±0.04 in the group with 35-49 and 50-79 mmHg of RVSP, respectively. These results show that when compared with BMIPP, Tl is superior for the estimation of right ventricular pressure. For the patients with right ventricular overloading, it was suggested that when RVSP reaches 80 mmHg or more, there appear some disorders in the fatty acid metabolism in the right ventricular myocardium. (author)

A case of Lamin C gene-mutation with preserved systolic function and ventricular dysrrhythmia

Directory of Open Access Journals (Sweden)

Kevin Kit Ng

2013-02-01

Full Text Available Lamin A/C gene-related cardiomyopathy is associated with progressive heart failure and malignant arrhythmias. Current guidelines advise the use of implantable defibrillators to prevent arrhythmogenic sudden cardiac death only in situations where there is evidence of severe left ventricular dysfunction. We describe a case of a woman with genetically confirmed Lamin C deficiency with preserved left ventricular function in whom an implantable defibrillator was inserted and within a month of implantation was used to terminate symptomatic ventricular tachycardia.

Acute right ventricular dysfunction: real-time management with echocardiography.

Science.gov (United States)

Krishnan, Sundar; Schmidt, Gregory A

2015-03-01

In critically ill patients, the right ventricle is susceptible to dysfunction due to increased afterload, decreased contractility, or alterations in preload. With the increased use of point-of-care ultrasonography and a decline in the use of pulmonary artery catheters, echocardiography can be the ideal tool for evaluation and to guide hemodynamic and respiratory therapy. We review the epidemiology of right ventricular failure in critically ill patients; echocardiographic parameters for evaluating the right ventricle; and the impact of mechanical ventilation, fluid therapy, and vasoactive infusions on the right ventricle. Finally, we summarize the principles of management in the context of right ventricular dysfunction and provide recommendations for echocardiography-guided management.

Role of high-resolution image integration to visualize left phrenic nerve and coronary arteries during epicardial ventricular tachycardia ablation.

Science.gov (United States)

Yamashita, Seigo; Sacher, Frédéric; Mahida, Saagar; Berte, Benjamin; Lim, Han S; Komatsu, Yuki; Amraoui, Sana; Denis, Arnaud; Derval, Nicolas; Laurent, François; Montaudon, Michel; Hocini, Mélèze; Haïssaguerre, Michel; Jaïs, Pierre; Cochet, Hubert

2015-04-01

Epicardial ventricular tachycardia (VT) ablation is associated with risks of coronary artery (CA) and phrenic nerve (PN) injury. We investigated the role of multidetector computed tomography in visualizing CA and PN during VT ablation. Ninety-five consecutive patients (86 men; age, 57 ± 15) with VT underwent cardiac multidetector computed tomography. The PN detection rate and anatomic variability were analyzed. In 49 patients undergoing epicardial mapping, real-time multidetector computed tomographic integration was used to display CAs/PN locations in 3-dimensional mapping systems. Elimination of local abnormal ventricular activities (LAVAs) was used as ablation end point. The distribution of CAs/PN with respect to LAVA was analyzed and compared between VT etiologies. Multidetector computed tomography detected PN in 81 patients (85%). Epicardial LAVAs were observed in 44 of 49 patients (15 ischemic cardiomyopathy, 15 nonischemic cardiomyopathy, and 14 arrhythmogenic right ventricular cardiomyopathy) with a mean of 35 ± 37 LAVA points/patient. LAVAs were located within 1 cm from CAs and PN in 35 (80%) and 18 (37%) patients, respectively. The prevalence of LAVA adjacent to CAs was higher in nonischemic cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy than in ischemic cardiomyopathy (100% versus 86% versus 53%; P < 0.01). The prevalence of LAVAs adjacent to PN was higher in nonischemic cardiomyopathy than in ischemic cardiomyopathy (93% versus 27%; P < 0.001). Epicardial ablation was performed in 37 patients (76%). Epicardial LAVAs could not be eliminated because of the proximity to CAs or PN in 8 patients (18%). The epicardial electrophysiological VT substrate is often close to CAs and PN in patients with nonischemic cardiomyopathy. High-resolution image integration is potentially useful to minimize risks of PN and CA injury during epicardial VT ablation. © 2015 American Heart Association, Inc.

Myocardial imaging by direct injection of thallium-201 into coronary artery

International Nuclear Information System (INIS)

Sugihara, Hiroki; Inagaki, Suetsugu; Kubota, Yasushi

1988-01-01

Myocardial perfusion images were evaluated by direct injection of Thallium (Tl)-201 into coronary artery. Approximately 0.5 - 1 mCi of Tl-201 were instilled into the right coronary artery and/or the left coronary artery after coronary arteriography. Three images were obtained in the anterior, left anterior oblique and left lateral projections. Myocardial perfusion images of single photon emission computed tomography were also acquired in some patients. An image of supreme quality could be obtained in spite of small dose of Tl-201 since there was a lack of interference from background activity. Myocardial perfusion images corresponded to areas which were supplied by left or right coronary artery respectively. And the regional myocardial blood flow distribution of a coronary artery bypass graft could be revealed by instilling Tl-201 into the graft. Further, contribution of collateral channels to myocardial perfusion was showed. Not only left ventricle but also right ventricle was clearly visualized by injection of Tl-201 into right coronary artery. But in a case with arrhythmogenic right ventricular dysplasia, there was an area of decreased tracer uptake in the apex of the right ventricle which was identified as the site of dysplasia by electrophysiologic study. We conclude that direct injection of Tl-201 into coronary artery is an useful method to clarify the correlation between coronary anatomical findings and coronary perfusion and contribution of collaterals to myocardial perfusion, and also to detect the right ventricular myopathic site. (author)

Earlier Right Ventricular Pacing in Cardiac Resynchronization Therapy for a Patient with Right Axis Deviation.

Science.gov (United States)

Hattori, Yusuke; Ishibashi, Kohei; Noda, Takashi; Okamura, Hideo; Kanzaki, Hideaki; Anzai, Toshihisa; Yasuda, Satoshi; Kusano, Kengo

2017-09-01

We describe the case of a 37-year-old woman who presented with complete right bundle branch block and right axis deviation. She was admitted to our hospital due to severe heart failure and was dependent on inotropic agents. Cardiac resynchronization therapy was initiated but did not improve her condition. After the optimization of the pacing timing, we performed earlier right ventricular pacing, which led to an improvement of her heart failure. Earlier right ventricular pacing should be considered in patients with complete right bundle branch block and right axis deviation when cardiac resynchronization therapy is not effective.

Effect of Hemodialysis on Left and Right Ventricular Volume and Function

International Nuclear Information System (INIS)

Han, Jin Suk; Koh, Chang Soon

1985-01-01

With the improvement of hemodialysis, the course of thc discase in patient with endstage renal disease has been clearly improved. Nevertheless, among several shortcomings to our present mode of renal replacement therapy, cardiovascular complications have been the leading cause of morbidity and mortality. Several factors such as anemia, arteriovenous shunting of blood, intermittent extracorporeal circulation and hypertension may be contributing. But little is known about the quantitative cardiac hemodynamic characteristics occurred during hemodialysis. The purpose of this study is to observe the sequential hemodynamic changes before, during and after the hemodialysis and to investigate: reliable parameters in the detection of ventricular dysfunction. In the present study, equilibrium radionuclide cardiac angiography was performed and left and right ventricular volume indices, ejection phase indices of both ventricular, performance were measured in the 16 stable patients with chronic renal failure treated with maintenance hemodialysis sequentially i.e. before, during (carly and late phase) and after the hemodialysis. The results obtained were as follows; 1) The indices of the left ventricular function were not changed during the hemodialysis but increased after the hemodialysis. 2) The indices of the right ventricular function(EF, SVI) were significantly decreased in the early phase (15, 30 minutes after starting extracorporeal circulation) but recovered after the hemodialysis, 3) The ratio of right ventricular to left ventricular ejection fraction was significantly decreased in the early phase and the lung volume indices were significantly increased at the same phase. As a conclusion, hemodialysis improves left ventricular function maybe du to increased contractility, and effects on the right ventricular function maybe due to the increased lung volume in the early phase of hemodialysis.

Effect of Hemodialysis on Left and Right Ventricular Volume and Function

Energy Technology Data Exchange (ETDEWEB)

Han, Jin Suk; Koh, Chang Soon [Seoul National University College of Medicine, Seoul (Korea, Republic of)

1985-09-15

With the improvement of hemodialysis, the course of thc discase in patient with endstage renal disease has been clearly improved. Nevertheless, among several shortcomings to our present mode of renal replacement therapy, cardiovascular complications have been the leading cause of morbidity and mortality. Several factors such as anemia, arteriovenous shunting of blood, intermittent extracorporeal circulation and hypertension may be contributing. But little is known about the quantitative cardiac hemodynamic characteristics occurred during hemodialysis. The purpose of this study is to observe the sequential hemodynamic changes before, during and after the hemodialysis and to investigate: reliable parameters in the detection of ventricular dysfunction. In the present study, equilibrium radionuclide cardiac angiography was performed and left and right ventricular volume indices, ejection phase indices of both ventricular, performance were measured in the 16 stable patients with chronic renal failure treated with maintenance hemodialysis sequentially i.e. before, during (carly and late phase) and after the hemodialysis. The results obtained were as follows; 1) The indices of the left ventricular function were not changed during the hemodialysis but increased after the hemodialysis. 2) The indices of the right ventricular function(EF, SVI) were significantly decreased in the early phase (15, 30 minutes after starting extracorporeal circulation) but recovered after the hemodialysis, 3) The ratio of right ventricular to left ventricular ejection fraction was significantly decreased in the early phase and the lung volume indices were significantly increased at the same phase. As a conclusion, hemodialysis improves left ventricular function maybe du to increased contractility, and effects on the right ventricular function maybe due to the increased lung volume in the early phase of hemodialysis.

Androgenic anabolic steroids also impair right ventricular function.

Science.gov (United States)

Kasikcioglu, Erdem; Oflaz, Huseyin; Umman, Berrin; Bugra, Zehra

2009-05-01

Chronic anabolic steroid use suppresses left ventricular functions. However, there is no information regarding the chronic effects of anabolic steroids on right ventricular function which also plays a key role in global cardiac function. The main objective of the present study was to investigate the effects of androgenic anabolic steroids usage among athletes on remodeling the right part of the heart. Androgenic-anabolic steroids-using bodybuilders had smaller diastolic velocities of both ventricles than drug-free bodybuilders and sedentary counterparts. This study shows that androgenic anabolic steroids-using bodybuilders exhibited depressed diastolic functions of both ventricles.

August rats are more resistant to arrhythmogenic effect of myocardial ischemia and reperfusion than Wistar rats.

Science.gov (United States)

Belkina, L M; Kirillina, T N; Pshennikova, M G; Arkhipenko, Yu V

2002-06-01

As differentiated from Wistar rats, myocardial ischemia and reperfusion produce no ventricular fibrillation in August rats. Pretreatment with nitric oxide synthase inhibitor Nw-nitro-L-arginine increased mortality rate in August rats with acute myocardial infarction from 20 to 40%. Under these conditions mortality rate in Wistar rats increased from 50 to 71%. Interstrain differences in the resistance of these animals to the arrhythmogenic effect of ischemia are probably associated with higher activity of the nitric oxide system in August rats compared to Wistar rats.

Effect of chronic right ventricular apical pacing on left ventricular function.

Science.gov (United States)

O'Keefe, James H; Abuissa, Hussam; Jones, Philip G; Thompson, Randall C; Bateman, Timothy M; McGhie, A Iain; Ramza, Brian M; Steinhaus, David M

2005-03-15

The determinants of change in left ventricular (LV) ejection fraction (EF) over time in patients with impaired LV function at baseline have not been clearly established. Using a nuclear database to assess changes in LV function over time, we included patients with a baseline LVEF of 25% to 40% on a gated single-photon emission computed tomographic study at rest and only if second-gated photon emission computed tomography performed approximately 18 months after the initial study showed an improvement in LVEF at rest of > or =10 points or a decrease in LVEF at rest of > or =7 points. In all, 148 patients qualified for the EF increase group and 59 patients for the EF decrease group. LVEF on average increased from 33 +/- 4% to 51 +/- 8% in the EF increase group and decreased from 35 +/- 4% to 25 +/- 5% in the EF decrease group. The strongest multivariable predictor of improvement of LVEF was beta-blocker therapy (odds ratio 3.9, p = 0.002). The strongest independent predictor of LVEF decrease was the presence of a permanent right ventricular apical pacemaker (odds ratio 6.6, p = 0.002). Thus, this study identified beta-blocker therapy as the major independent predictor for improvement in LVEF of > or =10 points, whereas a permanent pacemaker (right ventricular apical pacing) was the strongest predictor of a LVEF decrease of > or =7 points.

Assessment of right ventricular afterload in mitral valve diseases with radionuclide angiography

International Nuclear Information System (INIS)

Shimizu, Mitsuharu; Nakagawa, Tomio; Kohno, Yoshihiro; Kuroda, Masahiro; Takeda, Yoshihiro; Hiraki, Yoshio; Nagaya, Isao; Senoh, Yoshimasa; Teramoto, Shigeru

1991-01-01

Right ventricular function at rest and during exercise was studied in 33 patients with mitral valve disease by equilibrium gated radionuclide angiography using 99m Tc in vivo labeled red blood cells. Radionuclide measurements of right ventricular ejection fraction (RVEF) were correlated with mean pulmonary arterial pressure (mPAP). RVEF decreased significantly with exercise. There was no significant correlation between RVEF at rest and mPAP. However, mPAP showed significant negative correlation with RVEF during exercise and with the changes of RVEF from rest to exercise. It is concluded that RVEF during exercise in mitral valve disease is affected by right ventricular afterload, and the measurements of RVEF at rest and during exercise by equilibrium gated radionuclide angiography is useful to assess right ventricular afterload. (author)

Pulmonary Right Ventricular Resynchronization in Congenital Heart Disease: Acute Improvement in Right Ventricular Mechanics and Contraction Efficiency.

Science.gov (United States)

Janoušek, Jan; Kovanda, Jan; Ložek, Miroslav; Tomek, Viktor; Vojtovič, Pavel; Gebauer, Roman; Kubuš, Peter; Krejčíř, Miroslav; Lumens, Joost; Delhaas, Tammo; Prinzen, Frits

2017-09-01

Electromechanical discoordination may contribute to long-term pulmonary right ventricular (RV) dysfunction in patients after surgery for congenital heart disease. We sought to evaluate changes in RV function after temporary RV cardiac resynchronization therapy. Twenty-five patients aged median 12.0 years after repair of tetralogy of Fallot and similar lesions were studied echocardiographically (n=23) and by cardiac catheterization (n=5) after primary repair (n=4) or after surgical RV revalvulation for significant pulmonary regurgitation (n=21). Temporary RV cardiac resynchronization therapy was applied in the presence of complete right bundle branch block by atrial-synchronized RV free wall pacing in complete fusion with spontaneous ventricular depolarization using temporary electrodes. The q-RV interval at the RV free wall pacing site (mean 77.2% of baseline QRS duration) confirmed pacing from a late activated RV area. RV cardiac resynchronization therapy carried significant decrease in QRS duration ( P right bundle branch block QRS morphology, increase in RV filling time ( P =0.002), pulmonary artery velocity time integral ( P =0.006), and RV maximum +dP/dt ( P right bundle branch block, RV cardiac resynchronization therapy carried multiple positive effects on RV mechanics, synchrony, and contraction efficiency. © 2017 American Heart Association, Inc.

Impact of the right ventricular lead position on clinical outcome and on the incidence of ventricular tachyarrhythmias in patients with CRT-D

DEFF Research Database (Denmark)

Kutyifa, Valentina; Bloch Thomsen, Poul Erik; Huang, David T.

2013-01-01

Data on the impact of right ventricular (RV) lead location on clinical outcome and ventricular tachyarrhythmias in cardiac resynchronization therapy with defibrillator (CRT-D) patients are limited.......Data on the impact of right ventricular (RV) lead location on clinical outcome and ventricular tachyarrhythmias in cardiac resynchronization therapy with defibrillator (CRT-D) patients are limited....

Right ventricular volume and mass determined by cine magnetic resonance imaging in HIV patients with possible right ventricular dysfunction

DEFF Research Database (Denmark)

Kjaer, Andreas; Lebech, Anne-Mette; Gerstoft, Jan

2006-01-01

Impaired right ventricular (RV) function has been reported to occur in patients with HIV when studied by echocardiography. However, for accurate evaluation of RV function and morphology, first-pass radionuclide ventriculography (RNV) and cine magnetic resonance imaging (cine-MRI) are methods...... ventricular ejection fraction (RVEF). To do so, we screened patients with RNV and performed an additional cine-MRI in those with reduced RVEF determined by RNV. Ninety patients with HIV were included. To evaluate the MRI measures exactly we included 18 age- and gender-matched healthy volunteers to establish...

Diabetes Mellitus Associates with Increased Right Ventricular Afterload and Remodeling in Pulmonary Arterial Hypertension.

Science.gov (United States)

Whitaker, Morgan E; Nair, Vineet; Sinari, Shripad; Dherange, Parinita A; Natarajan, Balaji; Trutter, Lindsey; Brittain, Evan L; Hemnes, Anna R; Austin, Eric D; Patel, Kumar; Black, Stephen M; Garcia, Joe G N; Yuan Md PhD, Jason X; Vanderpool, Rebecca R; Rischard, Franz; Makino, Ayako; Bedrick, Edward J; Desai, Ankit A

2018-06-01

Diabetes mellitus is associated with left ventricular hypertrophy and dysfunction. Parallel studies have also reported associations between diabetes mellitus and right ventricular dysfunction and reduced survival in patients with pulmonary arterial hypertension. However, the impact of diabetes mellitus on the pulmonary vasculature has not been well characterized. We hypothesized that diabetes mellitus and hyperglycemia could specifically influence right ventricular afterload and remodeling in patients with Group I pulmonary arterial hypertension, providing a link to their known susceptibility to right ventricular dysfunction. Using an adjusted model for age, sex, pulmonary vascular resistance, and medication use, associations of fasting blood glucose, glycated hemoglobin, and the presence of diabetes mellitus were evaluated with markers of disease severity in 162 patients with pulmonary arterial hypertension. A surrogate measure of increased pulmonary artery stiffness, elevated pulmonary arterial elastance (P = .012), along with reduced log(pulmonary artery capacitance) (P = .006) were significantly associated with the presence of diabetes mellitus in patients with pulmonary arterial hypertension in a fully adjusted model. Similar associations between pulmonary arterial elastance and capacitance were noted with both fasting blood glucose and glycated hemoglobin. Furthermore, right ventricular wall thickness on echocardiography was greater in pulmonary arterial hypertension patients with diabetes, supporting the link between right ventricular remodeling and diabetes. Cumulatively, these data demonstrate that an increase in right ventricular afterload, beyond pulmonary vascular resistance alone, may influence right ventricular remodeling and provide a mechanistic link between the susceptibility to right ventricular dysfunction in patients with both diabetes mellitus and pulmonary arterial hypertension. Copyright © 2018 Elsevier Inc. All rights reserved.

Left Ventricular Myocardial Function in Children With Pulmonary Hypertension: Relation to Right Ventricular Performance and Hemodynamics.

Science.gov (United States)

Burkett, Dale A; Slorach, Cameron; Patel, Sonali S; Redington, Andrew N; Ivy, D Dunbar; Mertens, Luc; Younoszai, Adel K; Friedberg, Mark K

2015-08-01

Through ventricular interdependence, pulmonary hypertension (PH) induces left ventricular (LV) dysfunction. We hypothesized that LV strain/strain rate, surrogate measures of myocardial contractility, are reduced in pediatric PH and relate to invasive hemodynamics, right ventricular strain, and functional measures of PH. At 2 institutions, echocardiography was prospectively performed in 54 pediatric PH patients during cardiac catheterization, and in 54 matched controls. Patients with PH had reduced LV global longitudinal strain (LS; -18.8 [-17.3 to -20.4]% versus -20.2 [-19.0 to -20.9]%; P=0.0046) predominantly because of reduced basal (-12.9 [-10.8 to -16.3]% versus -17.9 [-14.5 to -20.7]%; Pright ventricular free-wall LS (r=0.64; PBrain natriuretic peptide levels correlated moderately with septal LS (r=0.48; P=0.0038). PH functional class correlated moderately with LV free-wall LS (r=-0.48; P=0.0051). The septum, shared between ventricles and affected by septal shift, was the most affected LV region in PH. Pediatric PH patients demonstrate reduced LV strain/strain rate, predominantly within the septum, with relationships to invasive hemodynamics, right ventricular strain, and functional PH measures. © 2015 American Heart Association, Inc.

Generalised left ventricular dysfunction after traumatic right coronary ...

African Journals Online (AJOL)

A patient with traumatic right coronary artery to right atrial fistula, which was repaired by direct closure and aortocoronary saphenous vein bypass grafting, is described. Cardiac catheterisation and selective cine angiocardiography were performed pre- and postoperatively, and left ventricular (LV) function was studied in ...

Subclinical changes in MRI-determined right ventricular volumes and function in subjects with prediabetes and diabetes.

Science.gov (United States)

Patscheider, Hannah; Lorbeer, Roberto; Auweter, Sigrid; Schafnitzel, Anina; Bayerl, Christian; Curta, Adrian; Rathmann, Wolfgang; Heier, Margit; Meisinger, Christa; Peters, Annette; Bamberg, Fabian; Hetterich, Holger

2018-07-01

The aim of this study was to assess subclinical changes in right ventricular volumes and function in subjects with prediabetes and diabetes and controls without a history of cardiovascular disease. Data from 400 participants in the KORA FF4 study without self-reported cardiovascular disease who underwent 3-T whole-body MRI were obtained. The right ventricle was evaluated using the short axis and a four-chamber view. Diabetes was defined according to WHO criteria. Associations between glucose tolerance and right ventricular parameters were assessed using multivariable adjusted linear regression models. Data from 337 participants were available for analysis. Of these, 43 (13%) had diabetes, 87 (26%) had prediabetes, and 207 (61%) were normoglycaemic controls. There was a stepwise decrease in right ventricular volumes in men with prediabetes and diabetes in comparison with controls, including right ventricular end-diastolic volume (β = -20.4 and β = -25.6, respectively; p ≤ 0.005), right ventricular end-systolic volume (β = -12.3 and β = -12.7, respectively; p ≤ 0.037) and right ventricular stroke volume (β = -8.1 and β = -13.1, respectively, p ≤ 0.016). We did not observe any association between prediabetes or diabetes and right ventricular volumes in women or between prediabetes or diabetes and right ventricular ejection fraction in men and women. This study points towards early subclinical changes in right ventricular volumes in men with diabetes and prediabetes. • MRI was used to detect subclinical changes in right ventricular parameters. • Diabetes mellitus is associated with right ventricular dysfunction. • Impairment of right ventricular volumes seems to occur predominantly in men.

Evaluation of right ventricular ejection fraction by first-pass radionuclide angiocardiography

International Nuclear Information System (INIS)

Uehara, Toshiisa; Nishimura, Tsunehiko; Naito, Hiroaki; Hayashida, Kohei; Kozuka, Takahiro

1981-01-01

Left ventricular ejection fraction (LVEF) obtained by radionuclide angiocardiography is a convenient and good parameter of the left ventricular function. Right ventricular ejection fraction (RVEF) also seems to be a good parameter of the right ventricular function. RVEF calculated from volumetry with contrast right ventriculography is not necessarily correct because of the complex figure of the right ventricle. On the other hand, the method of calculation of RVEF with radionuclide angiocardiography has the advantage of being able to ignore the complexity of figure of RV, because RI counts extracted from the time-activity curve represent changes in ventricular volume. In this study, we developped an original method to calculate RVEF with first pass method. After setting of region of interest (ROI) of RV, background and ROI for correction of motion of tricuspid valve, we calculated RVEF with these time-activity curves, Since we found that too rapid infusion of RI made the time-activity curve of RV too steep, and too slow infusion of RI made the background of lung field too high, the appropriate infusion rate was required to get correct value of RVEF. In addition, the time-activity curve often became steep or flat in dependence of the speed of venous return and cardiac output of patients. In order to avoid the effect of infusion speed, the time-activity curve was fitted to linear curves and the value of RVEF was corrected. The validity of these methods was confirmed in our study. As the result, RVEF obtained with our methods appeared to have good correlation with that obtained from volumetry of contrast right ventriculography (r = 0.77) and to be very useful in clinical estimation of right ventricular function. (author)

Thallium-201: quantitation of right ventricular hypertrophy in chronically hypoxic rats

International Nuclear Information System (INIS)

Rabinovitch, M.; Fisher, K.; Gamble, W.; Reid, L.; Treves, S.

1979-01-01

Sprague Dawley rats were divided into two groups. Ten were kept in room air and 10 in hypobaric hypoxia (air at 380 m Hg). After two weeks all were injected intravenously with 50 μCi of 201 Tl and sacrificed. The right and left ventricles were separated, weighed, and measured for radioactivity in a gamma well counter. Left and right ventricular mass ratios (MR) correlated with 201 Tl radioactivity ratios (TAR) in both control and hypoxic rats: r = 0.962 where MR = 0.863 TAR + 0.27. Myocardial 201 Tl uptake reflects and quantitates normal and abnormal ventricular mass, the abnormal mass in this model consisting of right ventricular hypertrophy associated with hypoxic pulmonary hypertension

Added clinical value of applying myocardial deformation imaging to assess right ventricular function.

Science.gov (United States)

Sokalskis, Vladislavs; Peluso, Diletta; Jagodzinski, Annika; Sinning, Christoph

2017-06-01

Right heart dysfunction has been found to be a strong prognostic factor predicting adverse outcome in various cardiopulmonary diseases. Conventional echocardiographic measurements can be limited by geometrical assumptions and impaired reproducibility. Speckle tracking-derived strain provides a robust quantification of right ventricular function. It explicitly evaluates myocardial deformation, as opposed to tissue Doppler-derived strain, which is computed from tissue velocity gradients. Right ventricular longitudinal strain provides a sensitive tool for detecting right ventricular dysfunction, even at subclinical levels. Moreover, the longitudinal strain can be applied for prognostic stratification of patients with pulmonary hypertension, pulmonary embolism, and congestive heart failure. Speckle tracking-derived right atrial strain, right ventricular longitudinal strain-derived mechanical dyssynchrony, and three-dimensional echocardiography-derived strain are emerging imaging parameters and methods. Their application in research is paving the way for their clinical use. © 2017, Wiley Periodicals, Inc.

Distribution of late gadolinium enhancement in various types of cardiomyopathies: Significance in differential diagnosis, clinical features and prognosis.

Science.gov (United States)

Satoh, Hiroshi; Sano, Makoto; Suwa, Kenichiro; Saitoh, Takeji; Nobuhara, Mamoru; Saotome, Masao; Urushida, Tsuyoshi; Katoh, Hideki; Hayashi, Hideharu

2014-07-26

The recent development of cardiac magnetic resonance (CMR) techniques has allowed detailed analyses of cardiac function and tissue characterization with high spatial resolution. We review characteristic CMR features in ischemic and non-ischemic cardiomyopathies (ICM and NICM), especially in terms of the location and distribution of late gadolinium enhancement (LGE). CMR in ICM shows segmental wall motion abnormalities or wall thinning in a particular coronary arterial territory, and the subendocardial or transmural LGE. LGE in NICM generally does not correspond to any particular coronary artery distribution and is located mostly in the mid-wall to subepicardial layer. The analysis of LGE distribution is valuable to differentiate NICM with diffusely impaired systolic function, including dilated cardiomyopathy, end-stage hypertrophic cardiomyopathy (HCM), cardiac sarcoidosis, and myocarditis, and those with diffuse left ventricular (LV) hypertrophy including HCM, cardiac amyloidosis and Anderson-Fabry disease. A transient low signal intensity LGE in regions of severe LV dysfunction is a particular feature of stress cardiomyopathy. In arrhythmogenic right ventricular cardiomyopathy/dysplasia, an enhancement of right ventricular (RV) wall with functional and morphological changes of RV becomes apparent. Finally, the analyses of LGE distribution have potentials to predict cardiac outcomes and response to treatments.

Dynamic right ventricular outflow tract (infundibular) stenosis and pectus excavatum in a dog

OpenAIRE

Fournier, Tanya E.

2008-01-01

This is the first published report of a dog with dynamic right ventricular outflow tract (infundibular) stenosis, right ventricular hypertrophy, and pectus excavatum. A juvenile dog presented with a grade V/VI left base systolic heart murmur, tachycardia, and pectus excavatum. Diagnosis of the aforementioned conditions was based on radiography, electrocardiography, and echocardiography. At 9 1/2 wk of age the heart murmur was no longer audible and the right ventricular stenosis and hypertroph...

Molecular diagnostics of catecholaminergic polymorphic ventricular tachycardia using denaturing high-performance liquid chromatography and sequencing

NARCIS (Netherlands)

Postma, Alex V.; Bhuiyan, Zahurul A.; Bikker, Hennie

2006-01-01

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an arrhythmogenic disease characterized by adrenergic-induced arrhythmias in the form of bidirectional and PVT. CPVT is a distinct clinical entity associated with a high mortality rate of up to 50% by the age of 30 yr. Recently, the

Prognostic significance of radionuclide-assessed right ventricular function in dilated cardiomyopathy

International Nuclear Information System (INIS)

Ohno, Akira; Nishimura, Tsunehiko; Uehara, Toshiisa; Shimonagata, Tsuyoshi; Kumita, Shinichiro; Ogawa, Youji; Nagata, Seiki; Miyatake, Kunio

1991-01-01

To assess the prognostic significance of right ventricular function in dilated cardiomyopathy (DCM), we studied consecutive 57 DCM patients. There were 41 men and 16 women, whose mean age was 48 years (range 3-68 years). The mean left ventricular ejection fraction (LVEF) in all patients was 29±11%, and the mean interval from the onset of symptom of cardiac failure (CHF history) was 4 years (range 0-33 years). With follow-up of 3.8 years, five patients had died until the first year, and 14 had died until the third year. By using multivariate regression analysis, there were no prognostic significance in clinical parameters such as age, CHF history, sex, atrial fibrillation, except for NYHA class, and medication at the third year. In survival curves according to Kaplan-Meier method, right ventricular ejection fraction (RVEF) and mean pulmonary artery (PA) had predictive value (p<0.05), while LVEF did not. The patients with RVEF<45% had poor survival rate compared to those with RVEF≥45%. The patients with RVEF<45% showed lower LVEF and left ventricular end-systolic volume index. RVEF may offer prognostic predictive value through the effect of not only mean PA but also left ventricular parameter. In conclusion, radionuclide assessment of right ventricular function should be valuable for the prognostic evaluation of DCM patients. (author)

Prognostic significance of radionuclide-assessed right ventricular function in dilated cardiomyopathy

Energy Technology Data Exchange (ETDEWEB)

Ohno, Akira; Nishimura, Tsunehiko; Uehara, Toshiisa; Shimonagata, Tsuyoshi; Kumita, Shinichiro; Ogawa, Youji; Nagata, Seiki; Miyatake, Kunio [National Cardiovascular Center, Suita, Osaka (Japan)

1991-09-01

To assess the prognostic significance of right ventricular function in dilated cardiomyopathy (DCM), we studied consecutive 57 DCM patients. There were 41 men and 16 women, whose mean age was 48 years (range 3-68 years). The mean left ventricular ejection fraction (LVEF) in all patients was 29{+-}11%, and the mean interval from the onset of symptom of cardiac failure (CHF history) was 4 years (range 0-33 years). With follow-up of 3.8 years, five patients had died until the first year, and 14 had died until the third year. By using multivariate regression analysis, there were no prognostic significance in clinical parameters such as age, CHF history, sex, atrial fibrillation, except for NYHA class, and medication at the third year. In survival curves according to Kaplan-Meier method, right ventricular ejection fraction (RVEF) and mean pulmonary artery (PA) had predictive value (p<0.05), while LVEF did not. The patients with RVEF<45% had poor survival rate compared to those with RVEF{>=}45%. The patients with RVEF<45% showed lower LVEF and left ventricular end-systolic volume index. RVEF may offer prognostic predictive value through the effect of not only mean PA but also left ventricular parameter. In conclusion, radionuclide assessment of right ventricular function should be valuable for the prognostic evaluation of DCM patients. (author).

Wolff-Parkinson-White syndrome concomitant with idiopathic ventricular fibrillation associated with inferior early repolarization.

Science.gov (United States)

Takahashi, Naohiko; Shinohara, Tetsuji; Hara, Masahide; Saikawa, Tetsunori

2012-01-01

We encountered a 39-year-old man with documented ventricular fibrillation (VF). His ECGs showed intermittent Wolff-Parkinson-White (WPW) syndrome pattern. During electrophysiological study, no ventricular preexcitation was observed. An accessory pathway located at the posterior mitral annulus was identified, and successfully eliminated by radiofrequency catheter ablation. VF was not induced. His ECGs in the absence of delta waves demonstrated early repolarization in the inferior leads. This case raises the possibility that patients with manifest WPW syndrome may have an arrhythmogenic substrate associated with early repolarization, and the characteristic J waves can be masked by the presence of ventricular preexcitation.

Gated cardiac blood pool studies in arrhythmias

International Nuclear Information System (INIS)

Itti, R.; Casset, D.; Philippe, L.; Cosnay, P.; Fauchier, J.P.

1988-01-01

Biventricular phase analysis a gated blood pool studies may help to solve two fundamental questions raised by patients suffering from arrhythmias: localization of an electrical cardiac activation abnormality by means of contraction mapping and assesment of an underlying organic disease using the phase histograms and their standard deviations. Three groups of patients have been evaluated to demonstrate the usefulness of radioisotopic techniques in arrhythmias: 36 patients with a Wolff-Parkinson-White syndrom, 27 patients studied during a ventricular tachycardia attack and 32 patients suspected of arrhythmogenic ventricular dysplasia. Correlations with invasive electrophysiologic studies are presented and the diagnostic and therapeutic implications of these results are discussed [fr

Gated cardiac blood pool studies in arrhythmias

Energy Technology Data Exchange (ETDEWEB)

Itti, R.; Casset, D.; Philippe, L.; Cosnay, P.; Fauchier, J.P.

1988-01-01

Biventricular phase analysis a gated blood pool studies may help to solve two fundamental questions raised by patients suffering from arrhythmias: localization of an electrical cardiac activation abnormality by means of contraction mapping and assesment of an underlying organic disease using the phase histograms and their standard deviations. Three groups of patients have been evaluated to demonstrate the usefulness of radioisotopic techniques in arrhythmias: 36 patients with a Wolff-Parkinson-White syndrom, 27 patients studied during a ventricular tachycardia attack and 32 patients suspected of arrhythmogenic ventricular dysplasia. Correlations with invasive electrophysiologic studies are presented and the diagnostic and therapeutic implications of these results are discussed.

2D-speckle tracking right ventricular strain to assess right ventricular systolic function in systolic heart failure. Analysis of the right ventricular free and posterolateral walls.

Science.gov (United States)

Mouton, Stéphanie; Ridon, Héléne; Fertin, Marie; Pentiah, Anju Duva; Goémine, Céline; Petyt, Grégory; Lamblin, Nicolas; Coisne, Augustin; Foucher-Hossein, Claude; Montaigne, David; de Groote, Pascal

2017-10-15

Right ventricular (RV) systolic function is a powerful prognostic factor in patients with systolic heart failure. The accurate estimation of RV function remains difficult. The aim of the study was to determine the diagnostic accuracy of 2D-speckle tracking RV strain in patients with systolic heart failure, analyzing both free and posterolateral walls. Seventy-six patients with dilated cardiopathy (left ventricular end-diastolic volume≥75ml/m 2 ) and left ventricular ejection fraction≤45% had an analysis of the RV strain. Feasibility, reproducibility and diagnostic accuracy of RV strain were analyzed and compared to other echocardiographic parameters of RV function. RV dysfunction was defined as a RV ejection fraction≤40% measured by radionuclide angiography. RV strain feasibility was 93.9% for the free-wall and 79.8% for the posterolateral wall. RV strain reproducibility was good (intra-observer and inter-observer bias and limits of agreement of 0.16±1.2% [-2.2-2.5] and 0.84±2.4 [-5.5-3.8], respectively). Patients with left heart failure have a RV systolic dysfunction that can be unmasked by advanced echocardiographic imaging: mean RV strain was -21±5.7% in patients without RV dysfunction and -15.8±5.1% in patients with RV dysfunction (p=0.0001). Mean RV strain showed the highest diagnostic accuracy to predict depressed RVEF (area under the curve (AUC) 0.75) with moderate sensitivity (60.5%) but high specificity (87.5%) using a cutoff value of -16%. RV strain seems to be a promising and more efficient measure than previous RV echocardiographic parameters for the diagnosis of RV systolic dysfunction. Copyright © 2017 Elsevier B.V. All rights reserved.

Epsilon wave on an electronic loop in a case of arrhythmogenic right ventricular dysplasia with myocarditis: an updated definition of the Epsilon wave.

Science.gov (United States)

Fontaine, Guy Hugues; Duthoit, Guillaume; Li, Guoliang; Andreoletti, L; Gandjbakhch, Estelle; Frank, Robert

2017-07-01

A young man presented with a history of myocarditis with palpitations and dizziness. He had implantation of a loop recorder that showed repetitive short episodes of VT. In addition, there were fragmented potentials immediately following the large and sharp electrograms (EGMs) before as well as after episodes of VT suggesting an Epsilon wave. This signal can be observed in multiple cardiac conditions including coronary artery disease. It was originally recorded on the epicardium as well as on the endocardium. However, in ARVD it can be defined as an electric signal observed after the end of the QRS complex in the right as opposed to the left precordial leads (difference ≥ 25 ms). It can also be an aid to the diagnosis of patients with ARVD who have other signs or symptoms suggesting ARVD including episodes of myocarditis. This potential consists of a slurring at the end of the QRS complex or an independent potential after the return to the isoelectric line. It can be better observed by increasing amplification of the ECG tracing as well as double speed using the Fontaine lead system. Epsilon wave too small to be recorded on the standard ECG can be extracted by Signal Averaging ECG SAECG). Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2017. For Permissions, please email: journals.permissions@oup.com.

Evaluation of right ventricular regional wall motion in inferior myocardial infarction by cine MRI

International Nuclear Information System (INIS)

Nishino, Masami; Ohnishi, Shusaku; Hasegawa, Shinji

1991-01-01

The purpose of this study is to evaluate right ventricular regional wall motion in inferior myocardial infarction by cine MRI. Thirteen patients with inferior myocardial infarction were investigated by cine MRI and were divided into proximal group which consisted of seven patients: >90% stenosis in segment 1 or 2 of right coronary artery and distal group which consisted of six patients: >90% stenosis in segment 3 or 4 of right coronary artery. Cine MRI was performed by 1.5 tesla magnet system (Signa, GE). To depict the regional asynergy, right ventricular wall was divided into 6 segments as follows: Segments 1 and 2 were upper and lower segments in transverse planes, respectively. Segments 3 and 4 were free wall and diaphragmatic segments of outflow tract, and segments 5 and 6 were of inflow tract in sagittal planes. Our results were as follows: (1) In proximal group, right ventricular asynergy was detected in six patients but in distal group it was detected in only one patient; (2) Right ventricular asynergy was detected most frequently at diaphragmatic segments in sagittal planes; (3) All the patients who had shown the hemodynamic deterioration of right ventricle on acute phase of inferior myocardial infarction presented the broad asynergy in right ventricle; (4) Cine MRI is clinically useful in evaluating right ventricular regional wall movement and diagnosing right ventricular infarction. (author)

Right ventricular longitudinal strain and right ventricular stroke work index in patients with severe heart failure: left ventricular assist device suitability for transplant candidates.

Science.gov (United States)

Cameli, M; Bernazzali, S; Lisi, M; Tsioulpas, C; Croccia, M G; Lisi, G; Maccherini, M; Mondillo, S

2012-09-01

Right ventricular (RV) systolic function has a critical role in determining the clinical outcome and the success of using left ventricular assist devices in patients with refractory heart failure. RV deformation analysis by speckle tracking echocardiography (STE) has recently allowed the analysis of RV longitudinal function. Using cardiac catheterization as the reference standard, this study aimed to explore the correlation between RV longitudinal function by STE and RV stroke work index (RVSWI) among patients referred for cardiac transplantation. Right heart catheterization and transthoracic echo-Doppler were simultaneously performed in 47 patients referred for cardiac transplant assessment due to refractory heart failure (ejection fraction 25.1 ± 4.5%). Thermodilution RV stroke volume and invasive pulmonary pressures were used to obtain RVSWI. RV longitudinal strain (RVLS) by STE was assessed averaging RV free-wall segments (free-wall RVLS). We also calculated. Tricuspid S' and tricuspid annular plane systolic excursion (TAPSE). No significant correlation was observed for TAPSE on tricuspid S' with RV stroke volume (r = 0.14 and r = 0.06, respectively). A close negative correlation between free-wall RVLS and RVSWI was found (r = -0.82; P rights reserved.

Quantitative evaluation of right ventricular overload with thallium-201 myocardial perfusion scintigraphy

International Nuclear Information System (INIS)

Watanabe, Toshiya; Miyakoda, Hiroyuki; Koike, Yoshihiro; Itatsu, Hidetaka; Kawai, Naoki; Sotobata, Iwao.

1983-01-01

Thallium-201 myocardial perfusion scintigraphy and right-heart catheterization were performed on patients with right ventricular pressure overload (RVPO) or right ventricular volume overload (RVVO). In 18 patients with RVPO, right ventricular systolic pressure correlated significantly both with the RV/LV wall thallium-201 uptake ratios (r=0.54, p<0.02) and the RV wall/background thallium-201 uptake ratios (r=0.70, p<0.01). RV/LV work ratios also significantly correlated with RV/LV wall thallium-201 uptake ratios (r=0.57, p<0.02). In 19 patients with RVVO, Qp/Qs and RV/LV work ratios both significantly correlated with RV/LV wall thallium-201 uptake ratios (r=0.78 and 0.87, respectively; p<0.001 for both) and RV wall/background thallium-201 uptake ratios (r=0.69, p<0.01 for both parameters). Right ventricular systolic pressure also correlated with RV/LV wall thallium-201 uptake ratios (r=0.57, p<0.02). Feasibility of the differentiation between RVPO and RVVO was suggested with use of ''transitional view angle'' and RV/LV diameter ratios obtained from the scintigram. In patients who underwent cardiac surgery, post-operative alleviations of the right ventricular overload were evaluated. There was a significant decrease in RV/LV wall thallium-201 uptake ratios, but no significant decrease in RV wall/background thallium-201 uptake ratios in patients with RVPO. On the other hand, there was a significant decrease both in RV/LV wall thallium-201 uptake ratios and RV wall/background thallium-201 uptake ratios in patients with RVVO. No significant changes were observed between the scintigraphic measurements obtained 1 month and 1 year after the surgery, irrespective of the type of right ventricular overloading. (J.P.N.)

Effect of coronary artery recanalization on right ventricular function in patients with acute myocardial infarction

International Nuclear Information System (INIS)

Verani, M.S.; Tortoledo, F.E.; Batty, J.W.; Raizner, A.E.

1985-01-01

The effects of coronary artery recanalization by intracoronary administration of streptokinase on left ventricular function during acute myocardial infarction have received increasing attention in recent years. Although myocardial dysfunction is often more pronounced in the right ventricle than in the left ventricle in patients with acute inferior wall myocardial infarction, the effect of coronary artery recanalization on right ventricular dysfunction has not been previously addressed. Accordingly, in this investigation, 54 patients who participated in a prospective, controlled, randomized trial of recanalization during acute myocardial infarction were studied. Among 30 patients with inferior wall infarction, 19 had right ventricular dysfunction on admission; 11 of these 19 had positive uptake of technetium-99m pyrophosphate in the right ventricle, indicative of right ventricular infarction. Patients with successful recanalization exhibited improved right ventricular ejection fraction from admission to day 10. However, control patients and patients who did not undergo recanalization also exhibited improvement. These data indicate that the right ventricular dysfunction commonly associated with inferior wall infarction is often transient, and improvement is the rule, irrespective of early recanalization of the infarct vessel

Right and left ventricular performance by nuclear stethoscope

International Nuclear Information System (INIS)

Nishimura, T.; Uehara, T.; Hayashida, K.; Kozuka, T.

1983-01-01

Right and left ventricular performance by Nuclear Stethoscope using Kr-81m and Tc-99m-RBC were performed. And physiological and pharmacological interventions were also investigated for the evaluation of coronary artery diseases (CAD)

Right ventricular hydatid cyst ruptured to pericardium

Directory of Open Access Journals (Sweden)

Feridoun Sabzi

2015-01-01

Full Text Available Cardiac hydatidosis is rare presentation of body hydatidosis. Incidence of cardiac involvements range from 5% to 5% of patients with hydatid disease. Most common site of hydatid cyst in heart is interventricular septum and left ventricular free wall. Right ventricular free wall involvement by cyst that ruptured to pericardial cavity is very rare presentation of hydatid cyst. Cardiac involvement may have serious consequences such as rupture to blood steam or pericardial cavity. Both the disease and its surgical treatment carry a high complication rate, including rupture leading to cardiac tamponade, anaphylaxis and also death. In the present report, a 43-year-old man with constrictive pericarditis secondary to a pericardial hydatid cyst is described.

Successful Treatment of Free-Floating Right Ventricular Thrombus with Acute Pulmonary Embolism

Directory of Open Access Journals (Sweden)

Sanjay Singhal

2015-01-01

Full Text Available Acute pulmonary embolism with floating right intra-ventricular thrombus is a rare phenomena associated with poor prognosis. Here, we are presenting a case of 35-year-young male with no co-morbid illness diagnosed to have right ventricular thrombus with bilateral pulmonary embolism, which was managed successfully with thrombolytic therapy.

Independent effects of both right and left ventricular function on plasma brain natriuretic peptide

DEFF Research Database (Denmark)

Vogelsang, Thomas Wiis; Jensen, Ruben J; Monrad, Astrid L

2007-01-01

BACKGROUND: Brain natriuretic peptide (BNP) is increased in heart failure; however, the relative contribution of the right and left ventricles is largely unknown. AIM: To investigate if right ventricular function has an independent influence on plasma BNP concentration. METHODS: Right (RVEF), left......, which is a strong prognostic marker in heart failure, independently depends on both left and right ventricular systolic function. This might, at least in part, explain why BNP holds stronger prognostic value than LVEF alone....... ventricular ejection fraction (LVEF), and left ventricular end-diastolic volume index (LVEDVI) were determined in 105 consecutive patients by first-pass radionuclide ventriculography (FP-RNV) and multiple ECG-gated equilibrium radionuclide ventriculography (ERNV), respectively. BNP was analyzed by immunoassay...

Independent effects of both right and left ventricular function on plasma brain natriuretic peptide.

Science.gov (United States)

Vogelsang, Thomas Wiis; Jensen, Ruben J; Monrad, Astrid L; Russ, Kaspar; Olesen, Uffe H; Hesse, Birger; Kjaer, Andreas

2007-09-01

Brain natriuretic peptide (BNP) is increased in heart failure; however, the relative contribution of the right and left ventricles is largely unknown. To investigate if right ventricular function has an independent influence on plasma BNP concentration. Right (RVEF), left ventricular ejection fraction (LVEF), and left ventricular end-diastolic volume index (LVEDVI) were determined in 105 consecutive patients by first-pass radionuclide ventriculography (FP-RNV) and multiple ECG-gated equilibrium radionuclide ventriculography (ERNV), respectively. BNP was analyzed by immunoassay. Mean LVEF was 0.51 (range 0.10-0.83) with 36% having a reduced LVEF (left and right ventricular systolic function. This might, at least in part, explain why BNP holds stronger prognostic value than LVEF alone.

Intraoperative Transesophageal Echocardiography and Right Ventricular Failure After Left Ventricular Assist Device Implantation.

Science.gov (United States)

Silverton, Natalie A; Patel, Ravi; Zimmerman, Josh; Ma, Jianing; Stoddard, Greg; Selzman, Craig; Morrissey, Candice K

2018-02-15

To determine whether intraoperative measures of right ventricular (RV) function using transesophageal echocardiography are associated with subsequent RV failure after left ventricular assist device (LVAD) implantation. Retrospective, nonrandomized, observational study. Single tertiary-level, university-affiliated hospital. The study comprised 100 patients with systolic heart failure undergoing elective LVAD implantation. Transesophageal echocardiographic images before and after cardiopulmonary bypass were analyzed to quantify RV function using tricuspid annular plane systolic excursion (TAPSE), tricuspid annular systolic velocity (S'), fractional area change (FAC), RV global longitudinal strain, and RV free wall strain. A chart review was performed to determine which patients subsequently developed RV failure (right ventricular assist device placement or prolonged inotrope requirement ≥14 days). Nineteen patients (19%) subsequently developed RV failure. Postbypass FAC was the only measure of RV function that distinguished between the RV failure and non-RV failure groups (21.2% v 26.5%; p = 0.04). The sensitivity, specificity, and area under the curve of an abnormal RV FAC (failure after LVAD implantation were 84%, 20%, and 0.52, respectively. No other intraoperative measure of RV function was associated with subsequent RV failure. RV failure increased ventilator time, intensive care unit and hospital length of stay, and mortality. Intraoperative measures of RV function such as tricuspid annular plane systolic excursion, tricuspid annular systolic velocity, and RV strain were not associated with RV failure after LVAD implantation. Decreased postbypass FAC was significantly associated with RV failure but showed poor discrimination. Copyright © 2018 Elsevier Inc. All rights reserved.

The effect of heart failure and left ventricular assist device treatment on right ventricular mechanics: a computational study.

Science.gov (United States)

Park, Jun I K; Heikhmakhtiar, Aulia Khamas; Kim, Chang Hyun; Kim, Yoo Seok; Choi, Seong Wook; Song, Kwang Soup; Lim, Ki Moo

2018-05-22

Although it is important to analyze the hemodynamic factors related to the right ventricle (RV) after left ventricular assist device (LVAD) implantation, previous studies have focused only on the alteration of the ventricular shape and lack quantitative analysis of the various hemodynamic parameters. Therefore, we quantitatively analyzed various hemodynamic parameters related to the RV under normal, heart failure (HF), and HF incorporated with continuous flow LVAD therapy by using a computational model. In this study, we combined a three-dimensional finite element electromechanical model of ventricles, which is based on human ventricular morphology captured by magnetic resonance imaging (MRI) with a lumped model of the circulatory system and continuous flow LVAD function in order to construct an integrated model of an LVAD implanted-cardiovascular system. To induce systolic dysfunction, the magnitude of the calcium transient function under HF condition was reduced to 70% of the normal value, and the time constant was reduced by 30% of the normal value. Under the HF condition, the left ventricular end systolic pressure decreased, the left ventricular end diastolic pressure increased, and the pressure in the right atrium (RA), RV, and pulmonary artery (PA) increased compared with the normal condition. The LVAD therapy decreased the end-systolic pressure of the LV by 41%, RA by 29%, RV by 53%, and PA by 71%, but increased the right ventricular ejection fraction by 52% and cardiac output by 40%, while the stroke work was reduced by 67% compared with the HF condition without LVAD. The end-systolic ventricular tension and strain decreased with the LVAD treatment. LVAD enhances CO and mechanical unloading of the LV as well as those of the RV and prevents pulmonary hypertension which can be induced by HF.

Reverse right ventricular remodeling after pulmonary endarterectomy in patients with chronic thromboembolic pulmonary hypertension: utility of magnetic resonance imaging to demonstrate restoration of the right ventricle

NARCIS (Netherlands)

Reesink, Herre J.; Marcus, J. Tim; Tulevski, Igor I.; Jamieson, Stuart; Kloek, Jaap J.; Vonk Noordegraaf, Anton; Bresser, Paul

2007-01-01

OBJECTIVES: Pulmonary arterial hypertension causes right ventricular remodeling; that is, right ventricular dilatation, hypertrophy, and leftward ventricular septal bowing. We studied the effect of pulmonary endarterectomy on the restoration of right ventricular remodeling in patients with chronic

Calculation of the ALMA Risk of Right Ventricular Failure After Left Ventricular Assist Device Implantation.

Science.gov (United States)

Loforte, Antonio; Montalto, Andrea; Musumeci, Francesco; Amarelli, Cristiano; Mariani, Carlo; Polizzi, Vincenzo; Lilla Della Monica, Paola; Grigioni, Francesco; Di Bartolomeo, Roberto; Marinelli, Giuseppe

2018-05-08

Right ventricular failure after continuous-flow left ventricular assist device (LVAD) implantation is still an unsolved issue and remains a life-threatening event for patients. We undertook this study to determine predictors of the patients who are candidates for isolated LVAD therapy as opposed to biventricular support (BVAD). We reviewed demographic, echocardiographic, hemodynamic, and laboratory variables for 258 patients who underwent both isolated LVAD implantation and unplanned BVAD because of early right ventricular failure after LVAD insertion, between 2006 and 2017 (LVAD = 170 and BVAD = 88). The final study patients were randomly divided into derivation (79.8%, n = 206) and validation (20.1%, n = 52) cohorts. Fifty-seven preoperative risk factors were compared between patients who were successfully managed with an LVAD and those who required a BVAD. Nineteen variables demonstrated statistical significance on univariable analysis. Multivariable logistic regression analysis identified destination therapy (odds ratio [OR] 2.0 [1.7-3.9], p = 0.003), a pulmonary artery pulsatility index right ventricle/left ventricle end-diastolic diameter ratio >0.75 (OR 2.7 [1.5-5.5], p = 0.001), an right ventricle stroke work index 17 (OR 3.5 [1.9-6.9], p the major predictors of the need for BVAD. Using these data, we propose a simple risk calculator to determine the suitability of patients for isolated LVAD support in the era of continuous-flow mechanical circulatory support devices.

Intraoperative Hemodynamic and Echocardiographic Measurements Associated With Severe Right Ventricular Failure After Left Ventricular Assist Device Implantation.

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Gudejko, Michael D; Gebhardt, Brian R; Zahedi, Farhad; Jain, Ankit; Breeze, Janis L; Lawrence, Matthew R; Shernan, Stanton K; Kapur, Navin K; Kiernan, Michael S; Couper, Greg; Cobey, Frederick C

2018-06-05

Severe right ventricular failure (RVF) after left ventricular assist device (LVAD) implantation increases morbidity and mortality. We investigated the association between intraoperative right heart hemodynamic data, echocardiographic parameters, and severe versus nonsevere RVF. A review of LVAD patients between March 2013 and March 2016 was performed. Severe RVF was defined by the need for a right ventricular mechanical support device, inotropic, and/or inhaled pulmonary vasodilator requirements for >14 days. From a chart review, the right ventricular failure risk score was calculated and right heart hemodynamic data were collected. Pulmonary artery pulsatility index (PAPi) [(pulmonary artery systolic pressure - pulmonary artery diastolic pressure)/central venous pressure (CVP)] was calculated for 2 periods: (1) 30 minutes before cardiopulmonary bypass (CPB) and (2) after chest closure. Echocardiographic data were recorded pre-CPB and post-CPB by a blinded reviewer. Univariate logistic regression models were used to examine the performance of hemodynamic and echocardiographic metrics. A total of 110 LVAD patients were identified. Twenty-five did not meet criteria for RVF. Of the remaining 85 patients, 28 (33%) met criteria for severe RVF. Hemodynamic factors associated with severe RVF included: higher CVP values after chest closure (18 ± 9 vs 13 ± 5 mm Hg; P = .0008) in addition to lower PAPi pre-CPB (1.2 ± 0.6 vs 1.7 ± 1.0; P = .04) and after chest closure (0.9 ± 0.5 vs 1.5 ± 0.8; P = .0008). Post-CPB echocardiographic findings associated with severe RVF included: larger right atrial diameter major axis (5.4 ± 0.9 vs 4.9 ± 1.0 cm; P = .03), larger right ventricle end-systolic area (22.6 ± 8.4 vs 18.5 ± 7.9 cm; P = .03), lower fractional area of change (20.2 ± 10.8 vs 25.9 ± 12.6; P = .04), and lower tricuspid annular plane systolic excursion (0.9 ± 0.2 vs 1.1 ± 0.3 cm; P = .008). Right ventricular failure risk score was not a significant predictor of

Prognostic impact of isolated right ventricular dysfunction in sepsis and septic shock: an 8-year historical cohort study.

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Vallabhajosyula, Saraschandra; Kumar, Mukesh; Pandompatam, Govind; Sakhuja, Ankit; Kashyap, Rahul; Kashani, Kianoush; Gajic, Ognjen; Geske, Jeffrey B; Jentzer, Jacob C

2017-09-07

Echocardiographic myocardial dysfunction is reported commonly in sepsis and septic shock, but there are limited data on sepsis-related right ventricular dysfunction. This study sought to evaluate the association of right ventricular dysfunction with clinical outcomes in patients with severe sepsis and septic shock. Historical cohort study of adult patients admitted to all intensive care units at the Mayo Clinic from January 1, 2007 through December 31, 2014 for severe sepsis and septic shock, who had an echocardiogram performed within 72 h of admission. Patients with prior heart failure, cor-pulmonale, pulmonary hypertension and valvular disease were excluded. Right ventricular dysfunction was defined by the American Society of Echocardiography criteria. Outcomes included 1-year survival, in-hospital mortality and length of stay. Right ventricular dysfunction was present in 214 (55%) of 388 patients who met the inclusion criteria-isolated right ventricular dysfunction was seen in 100 (47%) and combined right and left ventricular dysfunction in 114 (53%). The baseline characteristics were similar between cohorts except for the higher mechanical ventilation use in patients with isolated right ventricular dysfunction. Echocardiographic findings demonstrated lower right ventricular and tricuspid valve velocities in patients with right ventricular dysfunction and lower left ventricular ejection fraction and increased mitral E/e' ratios in patients with combined right and left ventricular dysfunction. After adjustment for age, comorbidity, illness severity, septic shock and use of mechanical ventilation, isolated right ventricular dysfunction was independently associated with worse 1-year survival-hazard ratio 1.6 [95% confidence interval 1.2-2.1; p = 0.002) in patients with sepsis and septic shock. Isolated right ventricular dysfunction is seen commonly in sepsis and septic shock and is associated with worse long-term survival.

Comparison of right ventricular septal pacing and right ventricular apical pacing in patients receiving cardiac resynchronization therapy defibrillators: the SEPTAL CRT Study

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Leclercq, Christophe; Sadoul, Nicolas; Mont, Lluis; Defaye, Pascal; Osca, Joaquim; Mouton, Elisabeth; Isnard, Richard; Habib, Gilbert; Zamorano, Jose; Derumeaux, Genevieve; Fernandez-Lozano, Ignacio; Dupuis, Jean-Marc; Rouleau, Frédéric; Tassin, Aude; Bordachar, Pierre; Clémenty, Jacques; Lafitte, Stephane; Ploux, Sylvan; Reant, Patricia; Ritter, Philippe; Defaye, Pascal; Jacon, Peggy; Mondesert, Blandine; Saunier, Carole; Vautrin, Estelle; Kacet, Salem; Guedon-Moreau, Laurence; Klug, Didier; Kouakam, Claude; Marechaux, Sylvestre; Marquie, Christelle; Polge, Anne Sophie; Richardson, Marjorie; Chevallier, Philippe; De Breyne, Brigitte; Lotek, Marcin M.; Nonin, Emilie; Pineau, Julien; Deharo, Jean-Claude; Bastard, Emilie; Franceschi, Frédéric; Habib, Gilbert; Jego, Christophe; Peyrouse, Eric; Prevot, Sebastien; Saint-Joseph, Hôpital; Bremondy, Michel; Faure, Jacques; Ferracci, Ange; Lefevre, Jean; Pisapia, Andre; Davy, Jean-Marc; Cransac, Frederic; Cung, Tien Tri; Georger, Frederic; Pasquie, Jean-Luc; Raczka, Franck; Sportouch-Dukhan, Catherine; Sadoul, Nicolas; Blangy, Hugues; Bruntz, Jean-François; Freysz, Luc; Groben, Laurent; Huttin, Olivier; Bammert, Antoine; Burban, Marc; Cebron, Jean-Pierre; Gras, Daniel; Frank, Robert; Duthoit, Guillaume; Hidden-Lucet, Françoise; Himbert, Caroline; Isnard, Richard; Lacotte, Jérôme; Pousset, Françoise; Zerah, Thierry; Leclercq, Christophe; Bellouin, Annaïk; Crocq, Christophe; Deplace, Christian; Donal, Erwan; Hamon, Cécile; Mabo, Philippe; Romain, Olivier; Solnon, Aude; Frederic, Anselme; Bauer, Fabrice; Bernard, Mathieu; Godin, Benedicte; Kurtz, Baptiste; Savoure, Arnaud; Copie, Xavier; Lascault, Gilles; Paziaud, Olivier; Piot, Olivier; Touche, Thierry; Delay, Toulouse Marc; Chilon, Talia; Detis, Nicolas; Duparc, Alexandre; Hebrard, Aurélien; Massabuau, Pierre; Maury, Philippe; Mondoly, Pierre; Rumeau, Philippe; Pasteur, Clinique; Boveda, Serge; Adrover, Laurence; Combes, Nicolas; Deplagne, Antoine; Marco-Baertich, Isabelle; Fondard, Olivier; Martínez, Juan Gabriel; Ibañez Criado, José Luis; Ortuño, Diego; Mont, Lluis; Berruezo, Antonio; Eduard, Belu; Martín, Ana; Merschon, Franco M.; Sitges, Marta; Tolosana, José María; Vidal, Bárbara; Hebron, H. Valle; i Mitjans, Angel Moya; Rodriguez, Oscar Alcalde; Rodriguez Palomares, José Fernando; Rivas, Nuria; Teixidó, Gisela; de Hierro, H. Puerta; Lozano, Ignacio Fernández; Ruiz Bautista, Maria Lorena; Castro, Victor; Cavero, Miguel Angel; Gutierrez, Carlos; Ros, Natalia; de la Victoria, H. Virgen; Alzueta Rodriguez, Francisco Javier; Cabrera, Fernando; Cordero, Alberto Barrera; Peña, José Luis; de Valme Sevilla, H.; Gonzáles, Juan Lealdel Ojo; Garcia Medina, Mª Dolores; Jiménez, Ricardo Pavón; Villagomez, David; de la Salud Toledo, H. Virgen; Castellanos Martinez, Eduardo; Alcalá, Juan; Maicas, Carolina; Arias Palomares, Miguel Angel; Puchol, Alberto; Valencia, H. La Fé; OscaAsensi, Joaquim; Carmona, Anastasio Quesada; De Carranza, Mª José Sancho-Tello; De Ros, José Olagüe; Pareja, Enrique Castro; Pérez, Oscar Cano; Saez, Ana Osa; Hortega, H. Rio; Guilarte, Benito Herreros; Muñoz San Jose, Juan Francisco; Pérez Sanz, Teresa Myriam; Logeart, Damien; Gil, Maria Lopez; Leclercq, Christophe; Lozano, Ignacio Fernandez; de Hierro, H. Puerta; Derumeaux, Genevieve

2016-01-01

Abstract Aims Cardiac resynchronization therapy (CRT) is a recommended treatment of heart failure (HF) patients with depressed left ventricular ejection fraction and wide QRS. The optimal right ventricular (RV) lead position being a matter of debate, we sought to examine whether RV septal (RVS) pacing was not inferior to RV apical (RVA) pacing on left ventricular reverse remodelling in patients receiving a CRT-defibrillator. Methods and results Patients (n = 263, age = 63.4 ± 9.5 years) were randomly assigned in a 1:1 ratio to RVS (n = 131) vs. RVA (n = 132) pacing. Left ventricular end-systolic volume (LVESV) reduction between baseline and 6 months was not different between the two groups (−25.3 ± 39.4 mL in RVS group vs. −29.3 ± 44.5 mL in RVA group, P = 0.79). Right ventricular septal pacing was not non-inferior (primary endpoint) to RVA pacing with regard to LVESV reduction (average difference = −4.06 mL; P = 0.006 with a −20 mL non-inferiority margin). The percentage of ‘echo-responders’ defined by LVESV reduction >15% between baseline and 6 months was similar in both groups (50%) with no difference in the time to first HF hospitalization or death (P = 0.532). Procedural or device-related serious adverse events occurred in 68 patients (RVS = 37) with no difference between the two groups (P = 0.401). Conclusion This study demonstrates that septal RV pacing in CRT is non-inferior to apical RV pacing for LV reverse remodelling at 6 months with no difference in the clinical outcome. No recommendation for optimal RV lead position can hence be drawn from this study. ClinicalTrials. gov number NCT 00833352. PMID:26374852

Successful ablation of a right atrium-axillary ventricular accessory pathway associated with Wolff-Parkinson-White syndrome.

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Yuan, Yuan; Long, Deyong; Dong, Jianzeng; Tao, Ling; Ma, Changsheng

2017-12-01

We report a case of a patient with right axillary ventricular. Similar congenital anomaly of the right atrium was reported as "right appendage diverticulum or right atrial diverticulum." However, this independent chamber has its own annulus, synchronizes with the right ventricular, and generates large ventricular potential. Under the guidance of the CARTO mapping system (Biosense Webster, Diamond Bar, CA, USA), a right atrioventricular accessory pathway associated with type B Wolff-Parkinson-White syndrome was ablated successfully. This pathway was close to the annulus of the axillary ventricular. The patient remained free of arrhythmia at 1-year follow-up. © 2017 Wiley Periodicals, Inc.

Ventricular arrhythmias in Chagas disease

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Marco Paulo Tomaz Barbosa

2015-02-01

Full Text Available Sudden death is one of the most characteristic phenomena of Chagas disease, and approximately one-third of infected patients develop life-threatening heart disease, including malignant ventricular arrhythmias. Fibrotic lesions secondary to chronic cardiomyopathy produce arrhythmogenic substrates that lead to the appearance and maintenance of ventricular arrhythmias. The objective of this study is to discuss the main clinical and epidemiological aspects of ventricular arrhythmias in Chagas disease, the specific workups and treatments for these abnormalities, and the breakthroughs needed to determine a more effective approach to these arrhythmias. A literature review was performed via a search of the PubMed database from 1965 to May 31, 2014 for studies of patients with Chagas disease. Clinical management of patients with chronic Chagas disease begins with proper clinical stratification and the identification of individuals at a higher risk of sudden cardiac death. Once a patient develops malignant ventricular arrhythmia, the therapeutic approach aims to prevent the recurrence of arrhythmias and sudden cardiac death by the use of implantable cardioverter defibrillators, antiarrhythmic drugs, or both. In select cases, invasive ablation of the reentrant circuit causing tachycardia may be useful. Ventricular arrhythmias are important manifestations of Chagas cardiomyopathy. This review highlights the absence of high-quality evidence regarding the treatment of ventricular arrhythmias in Chagas disease. Recognizing high-risk patients who require specific therapies, especially invasive procedures such as the implantation of cardioverter defibrillators and ablative approaches, is a major challenge in clinical practice.

An angiographic study of left- and right-ventricular function in patients with alcoholic heart and dilatation cardiomyopathy

International Nuclear Information System (INIS)

Savchenko, A.P.; Samko, A.N.; Smetnev, A.S.; Grudtsyn, G.V.

1986-01-01

An angiographic study of left- and right-ventricular function in 57 patients with alcoholic heart and dilatation cardiomyopathy demonstrated preclinical disorders of left-ventricular myocardial contractility and more marked right-ventricular changes in patients with second-stage chronic alcoholism. In cases of dilatation cardiomyopathy, left-ventricular dysfunction was predominant, while right-venricular changes were less pronouced

Evaluation of right and left ventricular function in the patients with myocardial infarction using quantitative radionuclide cardioangiography

International Nuclear Information System (INIS)

Nishimura, Tsunehiko; Uehara, Toshiisa; Hayashida, Kohei; Ohmine, Hiromi; Kozuka, Takahiro

1984-01-01

We evaluated right and left ventricular function in the case of myocardial infarction (MI) by radionuclide cardioangiography. The right and left ventricular ejection fraction (RVEF, LVEF) and ventricular volume (RVEDV, LVEDV) were calculated. And, phase and amplitude images were also obtained from gated blood pool scan. There were 60 cases of MI (35 anterior MI, 17 inferior MI and 8 right ventricular MI) and 10 normal cases. The LVEF was depressed in anterior MI (36 +- 14%) and RVEF was depressed in right ventricular MI (37 +- 5%). In addition, LVEDV was increased in anterior MI (163 +- 47 ml) and RVEDV increased in right ventricular MI (208 +- 33 ml), respectively. The amplitude of infarcted area was decreased. And the phase angle of LV was delayed in anterior MI and that of RV was delayed in right ventricular MI. Therefore, in the case of right ventricular MI, the depression of LVEF and RVEF was caused by the biventricular myocardial damage. And the cross talk phenomenon of biventricle was rarely observed, since the lung acts as the buffer between RV and LV. In conclusion, these noninvasive, methods provide useful information on the biventricular function in the case of myocardial infarction. (author)

Right ventricular functional analysis utilizing first pass radionuclide angiography for pre-operative ventricular assist device planning: a multi-modality comparison.

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Avery, Ryan; Day, Kevin; Jokerst, Clinton; Kazui, Toshinobu; Krupinski, Elizabeth; Khalpey, Zain

2017-10-10

Advanced heart failure treated with a left ventricular assist device is associated with a higher risk of right heart failure. Many advanced heart failures patients are treated with an ICD, a relative contraindication to MRI, prior to assist device placement. Given this limitation, left and right ventricular function for patients with an ICD is calculated using radionuclide angiography utilizing planar multigated acquisition (MUGA) and first pass radionuclide angiography (FPRNA), respectively. Given the availability of MRI protocols that can accommodate patients with ICDs, we have correlated the findings of ventricular functional analysis using radionuclide angiography to cardiac MRI, the reference standard for ventricle function calculation, to directly correlate calculated ejection fractions between these modalities, and to also assess agreement between available echocardiographic and hemodynamic parameters of right ventricular function. A retrospective review from January 2012 through May 2014 was performed to identify advanced heart failure patients who underwent both cardiac MRI and radionuclide angiography for ventricular functional analysis. Nine heart failure patients (8 men, 1 woman; mean age of 57.0 years) were identified. The average time between the cardiac MRI and radionuclide angiography exams was 38.9 days (range: 1 - 119 days). All patients undergoing cardiac MRI were scanned using an institutionally approved protocol for ICD with no device-related complications identified. A retrospective chart review of each patient for cardiomyopathy diagnosis, clinical follow-up, and echocardiogram and right heart catheterization performed during evaluation was also performed. The 9 patients demonstrated a mean left ventricular ejection fraction (LVEF) using cardiac MRI of 20.7% (12 - 40%). Mean LVEF using MUGA was 22.6% (12 - 49%). The mean right ventricular ejection fraction (RVEF) utilizing cardiac MRI was 28.3% (16 - 43%), and the mean RVEF calculated by

Recent Inferior Myocardial Infarction Complicated with a Right Ventricular Thrombus Detected by Three Cardiac Imaging Modalities.

Science.gov (United States)

Kuno, Toshiki; Imaeda, Syohei; Hashimoto, Kenji; Ryuzaki, Toshinobu; Saito, Tetsuya; Yamazaki, Hiroyuki; Tabei, Ryota; Kodaira, Masaki; Hase, Manabu; Numasawa, Yohei

2018-03-01

We report the case of a 71-year-old woman diagnosed with recent inferior myocardial infarction complicated with right ventricular infarction and a right ventricular thrombus. Three-dimensional transthoracic echocardiography, contrast-enhanced computed tomography, and cardiac magnetic resonance imaging clearly detected a thrombus. We consider cases with a recent right ventricular infarction to require assessment for thrombus formations in the right ventricle. Fortunately, vigorous anticoagulation therapy resolved the thrombi in both the right ventricle and right coronary artery.

Quantitative estimation of the right ventricular overloading by thallium-201 myocardial scintigraphy

International Nuclear Information System (INIS)

Owada, Kenji; Machii, Kazuo; Tsukahara, Yasunori

1982-01-01

Thallium-201 myocardial scintigraphy was performed on 55 patients with various types of right ventricular overloading. The right ventricular (RV) free wall was visualized in 39 out of the 55 patients (71%). The mean values of right ventricular systolic pressure (RVSP) and pulmonary artery mean pressure (PAMP) in the visualized cases (uptakers) were 54.6 +- 24.1 and 30.5 +- 15.3 mmHg, respectively. These values were significantly higher than those of the non-visualized cases (non-uptakers). There were 12 RVSP-''normotensive'' uptakers and 15 PAMP-''normotensive'' uptakers. The RV free wall images were classified into three types according to their morphological features. Type I was predominantly seen in cases of RV pressure overloading, type II in RV volume overloading and type III in combined ventricular overloading. RVSP in the type III group was significantly higher than that in other two groups. The radioactivity ratio in RV free wall and interventricular septum (IVS), the RV/IVS uptake ratio was calculated using left anterior oblique (LAO) view images. The RV/IVS uptake ratio closely correlated with RVSP and PAMP (r = 0.88 and 0.82, respectively). In each group of RV free wall image, there were also close correlations between the RV/IVS uptake ratio and both RVSP and PAMP. Our results indicate that the RV/IVS uptake ratio can be used as a parameter for the semi-quantitative estimation of right ventricular overloading. (author)

Increased brain and atrial natriuretic peptides in patients with chronic right ventricular pressure overload : correlation between plasma neurohormones and right ventricular dysfunction

NARCIS (Netherlands)

Tulevski, I.I.; Groenink, M; van der Wall, EE; van Veldhuisen, DJ; Boomsma, F; Hirsch, A; Lemkes, JS; Mulder, BJM; Stoker, J

Objective-To evaluate the role of plasma neurohormones in the diagnosis of asymptomatic or minimally symptomatic right ventricular dysfunction. Setting-Tertiary cardiovascular referral centre. Methods-Plasma brain natriuretic peptide (BNP) and atrial natriuretic peptide (ANP) concentrations were

Increased brain and atrial natriuretic peptides in patients with chronic right ventricular pressure overload: correlation between plasma neurohormones and right ventricular dysfunction

NARCIS (Netherlands)

Tulevski, I. I.; Groenink, M.; van der Wall, E. E.; van Veldhuisen, D. J.; Boomsma, F.; Stoker, J.; Hirsch, A.; Lemkes, J. S.; Mulder, B. J.

2001-01-01

OBJECTIVE: To evaluate the role of plasma neurohormones in the diagnosis of asymptomatic or minimally symptomatic right ventricular dysfunction. SETTING: Tertiary cardiovascular referral centre. METHODS: Plasma brain natriuretic peptide (BNP) and atrial natriuretic peptide (ANP) concentrations were

Mechanistic insights of the left ventricle structure and fibrosis in the arrhythmogenic mitral valve prolapse.

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Fernández-Friera, Leticia; Salguero, Rafael; Vannini, Luca; Argüelles, Ana Fidalgo; Arribas, Fernando; Solís, Jorge

2018-03-14

Mitral valve prolapse (MVP) is a common and benign condition. However, some anatomic forms have been recently associated with life-threatening ventricular arrhythmias and sudden cardiac death. Imaging MVP holds the promise of individualized MVP risk assessment. Noninvasive imaging techniques available today are playing an increasingly important role in the diagnosis, prognosis and monitoring of MVP. In this article, we will review the current evidence on arrhythmogenic MVP, with special focus on the utility of echocardiography and CMR for identifying benign and "malignant" forms of MVP. The clinical relevance of this manuscript lies in the value of imaging technology to improve MVP risk prediction, including those arrhythmic-MVP cases with a higher risk of sudden cardiac death.

Evaluation of right ventricular function for lung transplantation. Role of electron flow scan

International Nuclear Information System (INIS)

Stern, M.; Joint-Lambert, O.; Tainturier, C.; Seigneur, F.; Caubarrere, I.; Hernigou, A.

1994-01-01

Several invasive or not invasive technics were used to evaluate right ventricular insufficiency associated to severe chronic pulmonary insufficiency. But none of them were very accurate and now the use of EBT appears as a real improvement. We performed a prospective study with 50 patients waiting for a lung transplantation and we compared the values of right ventricular function obtained by EBT to those obtained by nuclear medicine and catheterism. Accuracy of EBT for left ventricle evaluation has already been proved. Stroke volumes calculated by EBT in right and left ventricles are similar and this constitutes a good validation of the method for right ventricle evaluation. Correlations with hemodynamic measurements are poor and nuclear medicine technics underestimate the ejection fraction. So, EBT is recommended for right ventricular study before and after lung transplantation. (authors). 11 refs., 4 figs

Peculiarities of cardiac hemodynamics and functional state of left ventricular myocardium in teenagers with connective heart tissue dysplasia

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Makhmudova F.M.

2011-03-01

Full Text Available The aim of investigation is to study heart hemodynamics in teenagers with connective tissue dysplasia of heart (CTDH. 35 patients ages 12 to 15 years with CTDH have been observed: Group I (n=14 are the patients with mitral valve prolapse (MVP without mitral regurgitation (MR and myxomatous degeneration(MD or isolated minor heart abnormalities (MHA, Group II (n=11are patients with MVP and MR in combination with 1 or2 MHA, and Group III (n=10 are patients with MVP and mixoid degeneration (MD in combination with 2 or more MHA. The control group consisted of 15 patients of the same age without MHA. All the children passed Doppler and echocardiography. According to the results significant changes of cardiohemodynamic indices in patients of Group I were not observed. The changes of size and volume indices of the left ventricle (LV, increase in wall thickness and diastolic dysfunction of the LV were observed in Group II. The significant changes of systolic function of left ventricular myocardium were observed in Group III. The study comes to the conclusion that teenagers with CTDH have definite changes of heart hemodynamics and functional state of left ventricular myocardium. These changes depend on mitral regurgitation, myxomatous degeneration and MHA combination

Right ventricular function assessed by 2D strain analysis predicts ventricular arrhythmias and sudden cardiac death in patients after acute myocardial infarction

DEFF Research Database (Denmark)

Risum, Niels; Valeur, Nana; Søgaard, Peter

2017-01-01

Aims: Left ventricular function is a well-established predictor of malignant ventricular arrhythmias, but little is known about the importance of right ventricular (RV) function. The aim of this study was to investigate the importance of RV function for prediction of sudden cardiac death (SCD) or...

Alpha B-crystallin prevents the arrhythmogenic effects of particulate matter isolated from ambient air by attenuating oxidative stress

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Park, Hyelim [The Division of Cardiology, Yonsei University College of Medicine, Seoul (Korea, Republic of); Brain Korea 21 Project for Medical Science, Yonsei University, Seoul (Korea, Republic of); Park, Sanghoon; Jeon, Hyunju [The Division of Cardiology, Yonsei University College of Medicine, Seoul (Korea, Republic of); Song, Byeong-Wook [The Division of Cardiology, Yonsei University College of Medicine, Seoul (Korea, Republic of); Brain Korea 21 Project for Medical Science, Yonsei University, Seoul (Korea, Republic of); Kim, Jin-Bae [Division of Cardiology, Kyung Hee University College of Medicine, Seoul (Korea, Republic of); Kim, Chang-Soo [The Department of Preventive Medicine, Yonsei University College of Medicine, Seoul (Korea, Republic of); Pak, Hui-Nam [The Division of Cardiology, Yonsei University College of Medicine, Seoul (Korea, Republic of); Hwang, Ki-Chul [The Division of Cardiology, Yonsei University College of Medicine, Seoul (Korea, Republic of); Brain Korea 21 Project for Medical Science, Yonsei University, Seoul (Korea, Republic of); Lee, Moon-Hyoung [The Division of Cardiology, Yonsei University College of Medicine, Seoul (Korea, Republic of); Chung, Ji Hyung, E-mail: jhchung@yuhs.ac [The Division of Cardiology, Yonsei University College of Medicine, Seoul (Korea, Republic of); Joung, Boyoung, E-mail: cby6908@yuhs.ac [The Division of Cardiology, Yonsei University College of Medicine, Seoul (Korea, Republic of); Brain Korea 21 Project for Medical Science, Yonsei University, Seoul (Korea, Republic of)

2013-01-15

Ca{sup 2+}/calmodulin-dependent protein kinase II (CaMKII) is activated by particulate matter (PM) isolated from ambient air and linked to prolonged repolarization and cardiac arrhythmia. We evaluated whether alpha B-crystallin (CryAB), a heat shock protein, could prevent the arrhythmogenic effects of PM by preventing CaMKII activation. CryAB was delivered into cardiac cells using a TAT-protein transduction domain (TAT-CryAB). ECGs were measured before and after tracheal exposure of diesel exhaust particles (DEP) and each intervention in adult Sprague–Dawley rats. After endotracheal exposure of DEP (200 μg/mL for 30 minutes, n = 11), QT intervals were prolonged from 115 ± 14 ms to 144 ± 20 ms (p = 0.03), and premature ventricular contractions were observed more frequently (0% vs. 44%) than control (n = 5) and TAT-Cry (n = 5). However, DEP-induced arrhythmia was not observed in TAT-CryAB (1 mg/kg) pretreated rats (n = 5). In optical mapping of Langendorff-perfused rat heats, compared with baseline, DEP infusion of 12.5 μg/mL (n = 12) increased apicobasal action potential duration (APD) differences from 2 ± 6 ms to 36 ± 15 ms (p < 0.001), APD restitution slope from 0.26 ± 0.07 to 1.19 ± 0.11 (p < 0.001) and ventricular tachycardia (VT) from 0% to 75% (p < 0.001). DEP infusion easily induced spatially discordant alternans. However, the effects of DEP were prevented by TAT-CryAB (1 mg/kg, n = 9). In rat myocytes, while DEP increased reactive oxygen species (ROS) generation and phosphated CaMKII, TAT-CryAB prevented these effects. In conclusion, CryAB, a small heat shock protein, might prevent the arrhythmogenic effects of PM by attenuating ROS generation and CaMKII activation. -- Highlights: ► Particulate matter (PM) increases arrhythmia. ► PM induced arrhythmias are related with oxidative stress and CaMKII activation. ► Alpha B-crystallin (CryAB) could attenuate the arrhythmogenic effect of PM. ► CryAB decreases oxidative stress and CaMKII activation

The evaluation of right ventricular performance using krypton-81m multiple-gated equilibrium cardiac blood pool scintigraphy

International Nuclear Information System (INIS)

Hosoi, Tsutomu

1993-01-01

Technetium-99m first pass angiography and multiple-gated equilibrium blood pool scintigraphy have been used for evaluating right ventricular performance, but both techniques have several methodologic limitations. The activity of kr-81m is exhaled during its transit through the lungs and residual activity in the left heart is negligible. Therefore, right heart study can be performed in the right anterior oblique position with optimal separation between the right ventricle and the right atrium. Thus, the author assessed right ventricular function using Kr-81m multiple-gated equilibrium cardiac blood pool scintigraphy in 40 patients with old myocardial infarction and 9 normal subjects. The patient group included 11 cases of inferior infarction with a significant stenosing lesion proximal to the origin of the right ventricular branch of the right coronary artery (group I), 11 cases of inferior infarction without a significant lesion in the proximal portion of the right coronary artery (group II) and 18 cases of anterior wall infarction (group III). The right ventricular ejection fraction (EF) was 45.6±9.8% in group I, 56.6±5.7% in group II, 55.6±4.4% in group III and 53.7±3.2% in the 9 normal subjects. There were no statistically significant differences among these 4 groups. However, right ventricular EF was significantly reduced in 5 group I patients who had hemodynamic evidence of right ventricular infarction (39.4±9.5%, P<0.01 vs group II, group III and normal subjects). No significant correlation was found between right and left ventricular EF or between right ventricular EF and wall motion abnormality of the interventricular septum. (author)

Infarto del ventrículo derecho Right ventricular infarction

Directory of Open Access Journals (Sweden)

Alberto Barón C

importante recanalizar rápidamente la arteria obstruida mediante trombólisis o angioplastia. Si persisten signos de bajo gasto se debe usar inotrópico parenteral. Se puede usar un balón de contrapulsación aórtica o dispositivo de asistencia mecánica. Recientemente, se ha descrito el uso de óxido nítrico para reducir la resistencia vascular pulmonar y mejorar el gasto cardiaco.In general, right ventricular infarction is associated with left ventricular inferior wall infarction. Obstructive chronic pulmonary disease and right ventricular hypertrophy are predisposing factors. It usually occurs as a consequence of proximal obstruction of the right coronary artery, which leads to right systolic and diastolic ventricle dysfunction. Stroke volume is diminished and diastolic volume and right ventricular filling pressure increase, causing hypotension and peripheral congestion. Pulmonary blood flow and left ventricular venous return are diminished, which may lead to shock. Besides, complications such as atrioventricular block, sinus dysfunction and ventricular aneurysm may occur. The electrocardiogram shows ST elevation in leads III, V1 to V3 and in V4R. The echocardiogram shows right ventricular free wall hypokinesis or akinesis and there is right cavities dilation and tricuspid regurgitation. The Doppler shows an increment in the duration of isovolumetric contraction and relaxation intervals; the ejection period is shortened and the myocardial performance index increase to abnormal values. The tissue Doppler is abnormal because of the decrease of systolic velocity in the tricuspid annulus. The optimization of rhythm and heart rate is an important part of treatment, and by this reason, beta-blockers may be avoided; depending on the severity of bradycardia, atropine, aminophylline or transient pace-maker can be used in order to ensure an adequate heart rate. In case of atrial fibrillation, anti-arrhythmic drugs or electric cardioversion may be used. An adequate filling volume

QRS Width as a Predictor of Right Ventricular Remodeling After Percutaneous Pulmonary Valve Implantation.

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Paech, C; Dähnert, I; Riede, F T; Wagner, R; Kister, T; Nieschke, K; Wagner, F; Gebauer, R A

2017-08-01

Recent data showed a right ventricular dyssynchrony in patients with tetralogy of Fallot (TOF). Percutaneous pulmonary valve implantation (PPVI) has become an important procedure to treat a pulmonary stenosis and/or regurgitation of the right ventricular outflow tract in these patients. Despite providing good results, there is still a considerable number of nonresponders to PPVI. The authors speculated that electrical dysfunction of the right ventricle plays an underestimated role in the outcome of patients after PPVI. This study aimed to investigate the influence of right ventricular electrical dysfunction, i.e., right bundle branch block (RBBB) on the RV remodeling after PPVI. The study included consecutive patients after correction of TOF with or without RBBB, who had received a PPVI previously at the Heart Center of the University of Leipzig, Germany during the period from 2012 to 2015. 24 patients were included. Patients without RBBB, i.e., with narrow QRS complexes pre-intervention, had significantly better RV function and had smaller right ventricular volumes. Patients with pre-interventionally QRS width below 150 ms showed a post-interventional remodeling of the right ventricle with the decreasing RV volumes (p = 0.001). The parameters of LV function and volume as well as RV ejection fraction remained unaffected by RBBB. The presented data indicate that the QRS width seems to be a valuable parameter in the prediction of right ventricular remodeling after PPVI, as it represents both electrical and mechanical functions of the right ventricle and may serve as an additional parameter for optimal timing of a PPVI.

Aorto-Right Ventricular Tunnel in Transposition of the Great Arteries

Directory of Open Access Journals (Sweden)

Antonio F. Corno

2018-02-01

Full Text Available Aorto-ventricular tunnel is an extremely rare congenital heart defect, consisting of failure of attachment of an aortic leaflet along the semilunar hinge. In all published reports the leaflet involved was either the right coronary leaflet, most frequently, or the left coronary leaflet, in most of the cases opening toward the left ventricle, with only one-eighth of the reported cases communicating with the right ventricle. Treatment of the aorto-ventricular tunnel has been anecdotally reported by interventional closure with a device and more frequently with surgical approach, either as an isolated malformation or as associated lesions. To the best of our knowledge, the presence of an aorto-ventricular tunnel of the non-adjacent aortic leaflet in transposition of the great arteries has never been reported. We have observed an aorto-ventricular tunnel involving the non-adjacent leaflet of the aortic root, which after arterial switch became the pulmonary root. The patient presented 18 years after the arterial switch with progressive dilatation of the right ventricle due to severe degree of pulmonary valve regurgitation, confirmed by echocardiography and cardiac MRI. Indication for surgery was given with the plan for a pulmonary valve implantation. Because of the intra-operative finding of disconnection of the anterior leaflet of the pulmonary valve (former aortic valve along the semilunar hinge, the surgical plan was modified and the anterior leaflet was attached to the valve annulus, with subsequent plasty in correspondence with the right and left commissurae to reduce the size of the dilated annulus to normal diameter. The post-operative course was uneventful, with extubation after few hours and discharge 4 days after surgery, with echocardiography showing trivial degree of pulmonary valve regurgitation. The patient remains in good conditions 6 months after surgery.

Ventricular repolarization time, location of pacing stimulus and current pulse amplitude conspire to determine arrhythmogenicity in mice

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Speerschneider, T; Grubb, Søren Jahn; Olesen, S P

2017-01-01

) were measured in isolated hearts using floating microelectrodes. RESULTS: Proarrhythmia in WT and KChIP2(-/-) was not sensitive to changes in refractory periods. Action potentials were longer in KChIP2(-/-) hearts compared to WT hearts. Isolated WT hearts had large apico-basal dispersion...... of repolarization time, whereas hearts from KChIP2(-/-) mice had large left-to-right ventricular dispersion of repolarization time. Pacing from the right ventricle in KChIP2(-/-) mice in vivo revealed significant lower current pulse amplitudes needed to induce arrhythmias in these mice. CONCLUSION: Large...

Right ventricular hypoplasia syndrome. Atresia of the right atrioventricular ostium (X-ray examination)

International Nuclear Information System (INIS)

Golonzko, R.R.; Chernova, M.P.; Berishvili, I.I.; Bulchinskij, S.A.

1982-01-01

Data on routine radiologic examination of 51 patients aged 3 days to 3 years with the right atrio-ventricular ostium atresia were presented. The evidence obtained was compared with angiographic and, in part of cases, with morphometric findings. All patients were divided in two groups, depending on pulmonary circulation functions. Characteristic radiologic features of the valvular defect were revealed: ''quadrangle'' heart configuration; increase of right auricle; ''sheared off'' profile of the right ventricle, absence of increase of leftauricle in children up to 1 year

Outcomes of patients with right ventricular failure on milrinone after left ventricular assist device implantation.

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Tsiouris, Athanasios; Paone, Gaetano; Brewer, Robert J; Nemeh, Hassan W; Borgi, Jamil; Morgan, Jeffrey A

2015-01-01

Previous studies have grouped together both patients requiring right ventricular assist devices (RVADs) with patients requiring prolonged milrinone therapy after left ventricular assist device (LVAD) implantation. We retrospectively identified 149 patients receiving LVADs and 18 (12.1%) of which developed right ventricular (RV) failure. We then separated these patients into those requiring RVADs versus prolonged milrinone therapy. This included 10 patients who were treated with prolonged milrinone and eight patients who underwent RVAD placement. Overall, the RV failure group had worse survival compared with the non-RV failure cohort (p = 0.038). However, this was only for the subgroup of patients who required RVADs, who had a 1, 6, 12, and 24 month survival of 62.5%, 37.5%, 37.5%, and 37.5%, respectively, versus 96.8%, 92.1%, 86.7%, and 84.4% for patients without RV failure (p milrinone therapy for RV failure had similar survivals compared with patients without RV failure. In the RV failure group, age, preoperative renal failure, and previous cardiac surgery were predictors of the need for prolonged postoperative milrinone. As LVADs become a more widely used therapy for patients with refractory, end-stage heart failure, it will be important to reduce the incidence of RV failure, as it yields significant morbidity and increases cost.

Case report: paradoxical ventricular septal motion in the setting of primary right ventricular myocardial failure.

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Maslow, Andrew; Schwartz, Carl; Mahmood, Feroze; Singh, Arun; Heerdt, Paul M

2009-07-01

In this report, a case of right ventricular (RV) failure, hemodynamic instability, and systemic organ failure is described to highlight how paradoxical ventricular systolic septal motion (PVSM), or a rightward systolic displacement of the interventricular septum, may contribute to RV ejection. Multiple inotropic medications and vasopressors were administered to treat right heart failure and systemic hypotension in a patient following combined aortic and mitral valve replacement. In the early postoperative period, echocardiographic evaluation revealed adequate left ventricular systolic function, akinesis of the RV myocardial tissues, and PVSM. In the presence of PVSM, RV fractional area of contraction was > or =35% despite akinesis of the primary RV myocardial walls. The PVSM appeared to contribute toward RV ejection. As a result, the need for multiple inotropes was re-evaluated, in considering that end-organ dysfunction was the result of systemic hypotension and prolonged vasopressor administration. After discontinuation of phosphodiesterase inhibitors, native vascular tone returned and the need for vasopressors declined. This was followed by recovery of systemic organ function. Echocardiographic re-evaluation two years later, revealed persistent akinesis of the RV myocardial tissues and PVSM, the latter appearing to contribute toward RV ejection. This case highlights the importance of left to RV interactions, and how PVSM may mediate these hemodynamic interactions.

Right ventricular exclusion and univentricular palliation for failed one and a half ventricle repair for Ebstein's anomaly.

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Sasikumar, Navaneetha; Krishna Manohar, Soman R; Philip, Saji; Cherian, Kottoorathu Mammen; Suresh Kumar, Raghavannair

2013-08-01

A 20 year-old male was diagnosed to have Ebstein's anomaly with severe right ventricular dysfunction. He was taken up for 1.5 ventricle repair. Post procedure, there was difficulty in weaning from cardiopulmonary bypass due to progressive right ventricular dilatation compromising the systemic output. An atrial septectomy did not help. Progressive right ventricular dilatation compressing the left ventricle, demonstrated on transoesophageal echocardiogram, prompted us to perform a right ventricular exclusion and univentricular palliation. The patient was successfully weaned off cardiopulmonary bypass and had a smooth postoperative recovery. Judicious use of right ventricular exclusion and univentricular palliation could be an effective bailout strategy in difficult surgical scenarios in Ebstein's anomaly. Copyright © 2012 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.

Study on thallium-201 myocardial perfusion scanning for detection of right ventricular hypertrophy in chronic pulmonary disease

International Nuclear Information System (INIS)

Kawai, Seiki

1980-01-01

Thallium-201 myocardial perfusion scanning was performed in 34 patients with chronic pulmonary disease for the purpose of detecting right ventricular hypertrophy. Thallium-201 activity ratio of left ventricle plus ventricular septum/right ventricle (TAR) was significantly correlated with hemodynamic findings such as pulmonary arterial mean pressure (r = -0.75, p 2 (p < 0.001). Assuming that TAR < 2 or TAR = 2 would indicate pulmonary hypertension, sensitivity was 95%, specificity 79%, validity score 75%, false positive 14% and false negative was 8%. TAR was compared with left to right ventricular mass ratio using Fulton's method in 6 autopsied patients in whom thallium-201 myocardial perfusion scanning were performed within three months before death. TAR closely correlated with left to right ventricular mass ratio (r = 0.978, p < 0.001). The comparison of validity of TAR with those of electrocardiographic interpretation according to WHO, Sasamoto, Roman or Milnor for the detection of right ventricular hypertrophy revealed the former was much superior to all of latters. From the results obtained, it may be inferred that TAR reflects the degree of pulmonary hypertension and anatomical right ventricular hypertrophy and is a useful non-invasive method to detect right ventricular hypertrophy in chronic pulmonary disease. (J.P.N.)

Validity of the accuracy of right ventricular volume measurement with single plane Simpson's rule based on a new half-circle model

International Nuclear Information System (INIS)

Cui Wei

2003-01-01

Objective: Right ventricular volume calculation is the basis of right ventricular function evaluation. The complexity of right ventricular shape makes it more difficult for measuring right ventricular volume, and therefore limits the widespread use of right ventricular function assessment. However, the short-axis view of the right ventricle is usually crescent, and might be assumed as half of a circle. This hypothesis can be applied to calculate right ventricular volume by using the plane Simpson's rule, but the final right ventricular volume should be about half of the original calculated value. The aim of this study was to test the accuracy of right ventricular volume measurement based on this new assumption in human right ventricular casts. Methods: Fifteen human right ventricular casts were obtained within 24 hours after death from 15 subjects who did not have overt cardiac disease. These casts were scanned with Toshiba multislice helical CT (Aquilion) using a scanning protocol similar to clinical practice. Right ventricular sagittal MPR images that correspond to right anterior oblique view were reconstructed later and single-plane Simpson's method was used to calculate right ventricular volumes. The calculated right ventricular volume was defined as the original calculated value divided by 2. The actual right ventricular casts volumes were determined by water displacement method. Results: The actual right ventricular volume was (64.23±24.51) ml, while the calculated volume was (53.18±26.22) ml. The calculated right ventricular volume significantly underestimated the actual volume by (11.05±13.09) ml (t=3.27, P=0.006). However, there was a close correlation between the actual cast volume and the calculated volume (r=0.869, P<0.001), with a regression equation of RV actual volume=21.04+0.812 x RV calculated volume. Conclusion: Right ventricular volume could be calculated accurately with single-plane Simpson's rule based on our new proposed half

Monitoring of right ventricular function by single probe system (nuclear stethoscope) and sup(81m)Kr

International Nuclear Information System (INIS)

Nishimura, Tsunehiko; Uehara, Toshiisa; Hayashida, Kohei; Ohmine, Hiromi; Kimura, Motomasa

1982-01-01

A single probe system (nuclear stethoscope) was developed to evaluate the monitoring of right ventricular function by sup(81m)Kr continuous infusion method. Optimal right ventricular and background positions were determined by position/monitor mode on beat-to-beat basis, and right ventricular ejection fraction (RVEF) was calculated by multi-gated method (MUGA). The correlation coefficient of nuclear stethoscope RVEF and camera-computer RVEF (MUGA) was 0.82, while the correlation coefficient of nuclear stethoscope RVEF and camera-computer RVEF (first-pass method) was 0.64. Therefore, the RVEF by nuclear stethoscope is useful for monitoring of right ventricular function. Clinical applications by sup(81m)Kr and nuclear stethoscope were as follows. 1. Exercise/rest RVEF is an extremely sensitive indicator to discriminate coronary artery disease (RCA occlusion) from normal subjects. 2. Beat-to-beat right ventricular volume response is useful for the monitoring of arrythmia such as atrial fibrillation. (author)

Monitoring of right ventricular function by single probe system (nuclear stethoscope) and sup(81m)Kr

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Nishimura, Tsunehiko; Uehara, Toshiisa; Hayashida, Kohei; Ohmine, Hiromi; Kimura, Motomasa [National Cardiovascular Center, Suita, Osaka (Japan)

1982-12-01

A single probe system (nuclear stethoscope) was developed to evaluate the monitoring of right ventricular function by sup(81m)Kr continuous infusion method. Optimal right ventricular and background positions were determined by position/monitor mode on beat-to-beat basis, and right ventricular ejection fraction (RVEF) was calculated by multi-gated method (MUGA). The correlation coefficient of nuclear stethoscope RVEF and camera-computer RVEF (MUGA) was 0.82, while the correlation coefficient of nuclear stethoscope RVEF and camera-computer RVEF (first-pass method) was 0.64. Therefore, the RVEF by nuclear stethoscope is useful for monitoring of right ventricular function. Clinical applications by sup(81m)Kr and nuclear stethoscope were as follows. 1. Exercise/rest RVEF is an extremely sensitive indicator to discriminate coronary artery disease (RCA occlusion) from normal subjects. 2. Beat-to-beat right ventricular volume response is useful for the monitoring of arrythmia such as atrial fibrillation.

Factors influencing the development of low output state in patients with right ventricular infarction

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Goto, Yoichi; Kanno, Kazuji; Saito, Muneyasu; Haze, Kazuo; Sumiyoshi, Tetsuya; Fukami, Kenichi; Fujiwara, Yasushi; Hiramori, Katsuhiko; Ikeda, Masao [National Cardiovascular Center, Suita, Osaka (Japan)

1984-06-01

Right ventricular infarction is frequently accompanied by a low output state, but the factors influencing the development of this state remain unknown. To elucidate these factors, clinical findings, hemodynamic findings and left ventricular infarct size (Tl-score) calculated from thallium-201 myocardial scintigrams by a circumferential profile method were evaluated in 147 consecutive patients with acute transmural inferior myocardial infarction. They were divided into two groups: 44 patients with right ventricular involvement (RVI group) and 103 patients without right ventricular involvement (IMI group). A low cardiac output state was defined when the cardiac index was less than 2.2 L/min/M/sup 2/. There was a good correlation between Tl-score and any of peak value of serum creatine phosphokinase (CPKmax), total released CPK (CPKr) and left ventricular ejection fraction (LVEF) (r=o.66, 0.74 and -0.54, respectively), indicating the usefulness of Tl-score as an index of left ventricular damage. Compared to the IMI group, the RVI group showed a higher average of age (p < 0.01), lower systemic blood pressure (p < 0.01), higher right atrial pressure (p < 0.001) and lower cardiac index (p < 0.01). Furthermore, the incidence of a low output state (RVI group:47.7% vs IMI group:14.6%, p < 0.001) and mortality (25.0% vs 7.8%, p < 0.01) were higher in the RVI group. However, CPKmax, CPKr, LVEF and Tl-score, which were considered to reflect the severity of left ventricular damage, were not different between the two groups. Tl-score was inversely correlated with cardiac index in the RVI group (r=-0.49, p < 0.05), and with left ventricular stroke work index in the both groups (RVI group; r=-0.46, p < 0.01, IMI group; r=-0.64, p < 0.01).

[Radioisotopic mapping of the arrhythmogenic focus in patients with chronic chagasic cardiomyopathy and sustained ventricular tachycardia].

Science.gov (United States)

de Paola, A A; Balbão, C E; Castiglioni, M L; Barbieri, A; Mendonça, A; Netto, O S; Guiguer Júnior, N; Vattimo, A C; Souza, I A; Portugal, O P

1993-06-01

To localize the site of the origin of sustained ventricular tachycardia in chronic chagasic cardiomyopathy patients refractory to antiarrhythmic therapy by radionuclide angiography techniques. Five patients underwent radionuclide angiography by intravenous administration of 25mCi 99mTc. The images were obtained in sinus rhythm and during sustained ventricular tachycardia induced in the electrophysiologic laboratory for endocardial mapping. Amplitude and phase images were obtained resulting in a contraction wave synchronic to ventricular dispolarization. All patients had haemodynamic stability during the arrhythmia. One patient had incessant ventricular tachycardia. Mean ejection fraction was 0.38. In 4 patients the site of the origin of ventricular tachycardia was posterior and in one it was localized in the interventricular septum. There was identity in the site of the origin of ventricular tachycardia obtained by endocardial mapping or radionuclide angiography in all patients. The therapy was chemical ablation in 3 patients, surgical aneurysmectomy in one and pharmacologic therapy in the last patient. The site of the origin of ventricular tachycardia can be estimated by analyzing the contraction wave obtained by radionuclide angiography techniques in patients with hemodynamic stable sustained ventricular tachycardia.

Mechanical stress is associated with right ventricular response to pulmonary valve replacement in patients with repaired tetralogy of Fallot.

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Tang, Dalin; Yang, Chun; Del Nido, Pedro J; Zuo, Heng; Rathod, Rahul H; Huang, Xueying; Gooty, Vasu; Tang, Alexander; Billiar, Kristen L; Wu, Zheyang; Geva, Tal

2016-03-01

Patients with repaired tetralogy of Fallot account for a substantial proportion of cases with late-onset right ventricular failure. The current surgical approach, which includes pulmonary valve replacement/insertion, has yielded mixed results. Therefore, it may be clinically useful to identify parameters that can be used to predict right ventricular function response to pulmonary valve replacement. Cardiac magnetic resonance data before and 6 months after pulmonary valve replacement were obtained from 16 patients with repaired tetralogy of Fallot (8 male, 8 female; median age, 42.75 years). Right ventricular ejection fraction change from pre- to postpulmonary valve replacement was used as the outcome. The patients were divided into group 1 (n = 8, better outcome) and group 2 (n = 8, worst outcome). Cardiac magnetic resonance-based patient-specific computational right ventricular/left ventricular models were constructed, and right ventricular mechanical stress and strain, wall thickness, curvature, and volumes were obtained for analysis. Our results indicated that right ventricular wall stress was the best single predictor for postpulmonary valve replacement outcome with an area under the receiver operating characteristic curve of 0.819. Mean values of stress, strain, wall thickness, and longitudinal curvature differed significantly between the 2 groups with right ventricular wall stress showing the largest difference. Mean right ventricular stress in group 2 was 103% higher than in group 1. Computational modeling and right ventricular stress may be used as tools to identify right ventricular function response to pulmonary valve replacement. Large-scale clinical studies are needed to validate these preliminary findings. Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

Right and Left Ventricular Volumes in Atrial Septal Defect Studied by Radiocardiography

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Ivancevic, D. [Radioisotope Department, Internal Clinic, Rebro, Zagreb, Yugoslavia (Croatia); Vernejoul, P. de; Kellershohn, C. [CEA, Service Hospitalier Frederic Joliot, Departement de Biologie, Orsay (France)

1971-02-15

Radiocardiography with radioiodinated ({sup 131}I) human serum albumin and barium ({sup 137m}Ba) solution injected into the right subclavian vein has been performed in a group of 43 patients with atrial septal defect and left-to-right shunt. Data on the output and ejection index of each ventricle are essential for the estimation of the diastolic and residual volumes of the right and left ventricle. The systemic flow was therefore calculated according to Veall's formula and the pulmonary flow and the shunt How were determined using the method of de Vernejoul and co-workers. The formulas for the calculation of ventricular volumes were modified. The results show that many cases of atrial septal defect have an enlarged right ventricle whereas the left ventricle remains normal or is diminished. These changes correlate well with the amount of the shunt flow. In both ventricles the ventricular volumes show a good correlation with the stroke volumes. For the regulation of the pulmonary blood volume the right ventricle seems to be more important than the left ventricle. The operative closure of atrial septal defect (in 14 patients) has normalized the size of ventricular volumes. (author)

Right ventricular visualization by Tl-201 myocardial scintigraphy in chronic obstructive pulmonary disease

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Fujii, Tadashige; Tanaka, Masao; Takeda, Masashi; Matsuzawa, Yukinori; Handa, Kenjiro; Yoshimura, Kazuhiko [Shinshu Univ., Matsumoto, Nagano (Japan). Faculty of Medicine

1993-04-01

Tl-201 myocardial scintigraphy was performed in 130 patients with chronic obstructive pulmonary disease (COPD) to evaluate right ventricular hypertrophy, and the clinical significance of this method was studied. Tl-201 uptake ratios of the right ventricle, which represents the ratio of total counts of the right ventricle to counts of the administered dose of Tl-201, was higher in COPD, especially in pulmonary emphysema and B type COPD by Burrows classification than in controls. The grade of visualization of the right ventricle by visual assessment (RVV) was marked (+++) in only a few cases and moderate (++) in many cases (more than 80%) in all diseases except bronchial asthma. The incidence of right ventricular hypertrophy by electrocardiogram, right-sided heart failure and marked dyspnea (Hugh-Jones 4[center dot]5) were very low in cases with RVV grade ++ and very high in cases with +++. The grade of RVV was related to the severity of pulmonary perfusion impairment, although in diffuse panbronchiolitis the RVV was relatively slight compared with the impairment of perfusion. May parameters of pulmonary function such as %VC, FEV1.0%, RV/TLC, V25, %DLCO, Raw, [Delta]N[sub 2] and PaO[sub 2] showed abnormal values in patients with RVV grade of (++) or (+++) in all diseases except bronchial asthma. In COPD, Tl-201 myocardial scintigraphy seems to be useful for assessment of right ventricular overloading, and for follow-up observation and differentiation between cor pulmonale and right ventricular hypertrophy secondary to cardiac diseases by observing Tl-201 uptake of the lung and left ventricle. (author).

Right ventricular visualization by Tl-201 myocardial scintigraphy in chronic obstructive pulmonary disease

International Nuclear Information System (INIS)

Fujii, Tadashige; Tanaka, Masao; Takeda, Masashi; Matsuzawa, Yukinori; Handa, Kenjiro; Yoshimura, Kazuhiko

1993-01-01

Tl-201 myocardial scintigraphy was performed in 130 patients with chronic obstructive pulmonary disease (COPD) to evaluate right ventricular hypertrophy, and the clinical significance of this method was studied. Tl-201 uptake ratios of the right ventricle, which represents the ratio of total counts of the right ventricle to counts of the administered dose of Tl-201, was higher in COPD, especially in pulmonary emphysema and B type COPD by Burrows classification than in controls. The grade of visualization of the right ventricle by visual assessment (RVV) was marked (+++) in only a few cases and moderate (++) in many cases (more than 80%) in all diseases except bronchial asthma. The incidence of right ventricular hypertrophy by electrocardiogram, right-sided heart failure and marked dyspnea (Hugh-Jones 4·5) were very low in cases with RVV grade ++ and very high in cases with +++. The grade of RVV was related to the severity of pulmonary perfusion impairment, although in diffuse panbronchiolitis the RVV was relatively slight compared with the impairment of perfusion. May parameters of pulmonary function such as %VC, FEV1.0%, RV/TLC, V25, %DLCO, Raw, ΔN 2 and PaO 2 showed abnormal values in patients with RVV grade of (++) or (+++) in all diseases except bronchial asthma. In COPD, Tl-201 myocardial scintigraphy seems to be useful for assessment of right ventricular overloading, and for follow-up observation and differentiation between cor pulmonale and right ventricular hypertrophy secondary to cardiac diseases by observing Tl-201 uptake of the lung and left ventricle. (author)

Right ventricular morphology and function in chronic obstructive pulmonary disease patients living at high altitude.

Science.gov (United States)

Güvenç, Tolga Sinan; Erer, Hatice Betül; Kul, Seref; Perinçek, Gökhan; Ilhan, Sami; Sayar, Nurten; Yıldırım, Binnaz Zeynep; Doğan, Coşkun; Karabağ, Yavuz; Balcı, Bahattin; Eren, Mehmet

2013-01-01

Pulmonary vasculature is affected in patients with chronic pulmonary obstructive disease (COPD). As a result of increased pulmonary resistance, right ventricular morphology and function are altered in COPD patients. High altitude and related hypoxia causes pulmonary vasoconstriction, thereby affecting the right ventricle. We aimed to investigate the combined effects of COPD and altitude-related chronic hypoxia on right ventricular morphology and function. Forty COPD patients living at high altitude (1768 m) and 41 COPD patients living at sea level were enrolled in the study. All participants were diagnosed as COPD by a pulmonary diseases specialist depending on symptoms, radiologic findings and pulmonary function test results. Detailed two-dimensional echocardiography was performed by a cardiologist at both study locations. Oxygen saturation and mean pulmonary artery pressure were higher in the high altitude group. Right ventricular end diastolic diameter, end systolic diameter, height and end systolic area were significantly higher in the high altitude group compared to the sea level group. Parameters of systolic function, including tricuspid annular systolic excursion, systolic velocity of tricuspid annulus and right ventricular isovolumic acceleration were similar between groups, while fractional area change was significantly higher in the sea level groups compared to the high altitude group. Indices of diastolic function and myocardial performance index were similar between groups. An increase in mean pulmonary artery pressure and right ventricular dimensions are observed in COPD patients living at high altitude. Despite this increase, systolic and diastolic functions of the right ventricle, as well as global right ventricular performance are similar in COPD patients living at high altitude and sea level. Altitude-related adaptation to chronic hypoxia could explain these findings. Copyright © 2012 Australian and New Zealand Society of Cardiac and Thoracic

Assessment of subclinical right ventricular systolic dysfunction in coal miners using myocardial isovolumic acceleration.

Science.gov (United States)

Ozcan Abacıoglu, Ozge; Kaplan, Mehmet; Abacıoglu, Serkan; Quisi, Ala

2017-09-01

Several studies have been conducted regarding the effects of coal mining on the respiratory system. However, there is a lack of data concerning potential effects of coal mining on the cardiovascular system. In this study, we aimed to evaluate the potential subclinical right and left ventricular dysfunction in coal miners. This single-center, prospective study included a total of 102 patients. Patient and control groups consisted of 54 coal miners and 48 healthy men, respectively. All patients underwent 12-lead electrocardiography, transthoracic echocardiography, and pulmonary function test. As compared to control group, coal miners had significantly higher right ventricular myocardial performance index (RVMPI) (0.41 ± 0.03 vs 0.37 ± 0.02, P right ventricular fractional area change (RVFAC) (33.55% ± 6.70% vs 37.04 ± 9.26 P right and left ventricular dysfunction, including RVMPI, RVFAC, TAPSE, IVA, and AD, are impaired in coal miners. © 2017 The Authors Echocardiography Published by Wiley Periodicals, Inc.

Management of Arrhythmias in Athletes: Atrial Fibrillation, Premature Ventricular Contractions, and Ventricular Tachycardia.

Science.gov (United States)

Lai, Ernest; Chung, Eugene H

2017-10-09

Management of atrial fibrillation, premature ventricular contractions, and ventricular tachycardia without underlying cardiac disease or arrhythmogenic conditions differs in athletes from the general population. Athletes tend to be younger, healthier individuals with few comorbidities. Therapies that work well in the general population may not be appropriate or preferable for athletes. Management strategies include deconditioning, pharmacologic therapy, such as rate control with β-blockers or non-dihydropyridine calcium channel blockers and rhythm control with class I or class III antiarrhythmic drugs, and catheter ablation. Deconditioning is not preferred by athletes because of lost playing time. Pharmacologic therapy is well tolerated among most individuals, but is not as favorable in athletes. Rate control medications can reduce performance and β-blockers, in particular, are prohibited in many sports. Antiarrhythmic drugs are preferred over rate control with athletes, but many, especially younger athletes, may not like the idea of long-term medical therapy. Catheter ablation has been proven to be safe and efficacious, may eliminate the need for long-term medical therapy, and is supported by the major societies (AHA, ACC, ESC).

Radionuclide stroke count ratios for assessment of right and left ventricular volume overload in children

International Nuclear Information System (INIS)

Parrish, M.D.; Graham, T.P. Jr.; Born, M.L.; Jones, J.P.; Boucek, R.J. Jr.; Artman, M.; Partain, C.L.

1983-01-01

The ratio of left ventricular to right ventricular stroke counts measured by radionuclide angiography has been used in adults to estimate the severity of left-sided valvular regurgitation. The validation of this technique in children for assessment of right and left ventricular volume overload is reported herein. Radionuclide stroke count ratios in 60 children aged 0.5 to 19 years (mean 11) were determined. Based on their diagnoses, the patients were divided into 3 groups: (1) normal--40 patients with no shunts or valvular regurgitation, (2) left ventricular volume overload--13 patients with mitral or aortic regurgitation, or both, and (3) right ventricular volume overload--7 patients, 2 with severe tricuspid regurgitation, 3 with atrial septal defects, and 2 with total anomalous pulmonary venous drainage. The radionuclide stroke count ratio clearly differentiated these groups (p less than 0.05): normal patients had a stroke count ratio of 1.04 +/- 0.17 (mean +/- 1 standard deviation), the left ventricular volume overload group had a stroke count ratio of 2.43 +/- 0.86, and the right ventricular volume overload group had a stroke count ratio of 0.44 +/- 0.17. In 22 of our 60 patients, radionuclide stroke count ratios were compared with cineangiographic stroke volume ratios, resulting in a correlation coefficient of 0.88. It is concluded that radionuclide ventriculography is an excellent tool for qualitative and quantitative assessment of valvular regurgitation in children

Quantitative thallium-201 myocardial imaging in assessing right ventricular pressure in patients with congenital heart defects

International Nuclear Information System (INIS)

Rabinovitch, M.; Fischer, K.C.; Treves, S.

1981-01-01

Thallium-201 myocardial scintigraphy was performed in patients with congenital heart defects to determine whether, by quantification of right ventricular isotope uptake, one could assess the degree of right ventricular hypertrophy and so predict the level of right ventricular pressure. It is shown that quantitative analysis of myocardial imaging with thallium-201 is of use clinically in patients with congenital heart defects, in assessing the severity of pulmonary stenosis or the presence of pulmonary artery hypertension. (author)

Sildenafil attenuates pulmonary inflammation and fibrin deposition, mortality and right ventricular hypertrophy in neonatal hyperoxic lung injury

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Boersma Hester

2009-04-01

Full Text Available Abstract Background Phosphodiesterase-5 inhibition with sildenafil has been used to treat severe pulmonary hypertension and bronchopulmonary dysplasia (BPD, a chronic lung disease in very preterm infants who were mechanically ventilated for respiratory distress syndrome. Methods Sildenafil treatment was investigated in 2 models of experimental BPD: a lethal neonatal model, in which rat pups were continuously exposed to hyperoxia and treated daily with sildenafil (50–150 mg/kg body weight/day; injected subcutaneously and a neonatal lung injury-recovery model in which rat pups were exposed to hyperoxia for 9 days, followed by 9 days of recovery in room air and started sildenafil treatment on day 6 of hyperoxia exposure. Parameters investigated include survival, histopathology, fibrin deposition, alveolar vascular leakage, right ventricular hypertrophy, and differential mRNA expression in lung and heart tissue. Results Prophylactic treatment with an optimal dose of sildenafil (2 × 50 mg/kg/day significantly increased lung cGMP levels, prolonged median survival, reduced fibrin deposition, total protein content in bronchoalveolar lavage fluid, inflammation and septum thickness. Treatment with sildenafil partially corrected the differential mRNA expression of amphiregulin, plasminogen activator inhibitor-1, fibroblast growth factor receptor-4 and vascular endothelial growth factor receptor-2 in the lung and of brain and c-type natriuretic peptides and the natriuretic peptide receptors NPR-A, -B, and -C in the right ventricle. In the lethal and injury-recovery model we demonstrated improved alveolarization and angiogenesis by attenuating mean linear intercept and arteriolar wall thickness and increasing pulmonary blood vessel density, and right ventricular hypertrophy (RVH. Conclusion Sildenafil treatment, started simultaneously with exposure to hyperoxia after birth, prolongs survival, increases pulmonary cGMP levels, reduces the pulmonary

Is right ventricular mid-septal pacing superior to apical pacing in patients with high degree atrio-ventricular block and moderately depressed left ventricular function?

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Chen, Kang; Mao, Ye; Liu, Shao-hua; Wu, Qiong; Luo, Qing-zhi; Pan, Wen-qi; Jin, Qi; Zhang, Ning; Ling, Tian-you; Chen, Ying; Gu, Gang; Shen, Wei-feng; Wu, Li-qun

2014-06-01

We are aimed to investigate whether right ventricular mid-septal pacing (RVMSP) is superior to conventional right ventricular apical pacing (RVAP) in improving clinical functional capacity and left ventricular ejection fraction (LVEF) for patients with high-degree atrio-ventricular block and moderately depressed left ventricle (LV) function. Ninety-two patients with high-degree atrio-ventricular block and moderately reduced LVEF (ranging from 35% to 50%) were randomly allocated to RVMSP (n=45) and RVAP (n=47). New York Heart Association (NYHA) functional class, echocardiographic LVEF, and distance during a 6-min walk test (6MWT) were determined at 18 months after pacemaker implantation. Serum levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) were measured using an enzyme-linked immunosorbent assay (ELISA) kit. Compared with baseline, NYHA functional class remained unchanged at 18 months, distance during 6MWT (485 m vs. 517 m) and LVEF (36.7% vs. 41.8%) were increased, but BNP levels were reduced (2352 pg/ml vs. 710 pg/ml) in the RVMSP group compared with those in the RVAP group, especially in patients with LVEF 35%-40% (for all comparisons, Pfunction capacity and LV function measurements were not significantly changed in patients with RVAP, despite the pacing measurements being similar in both groups, such as R-wave amplitude and capture threshold. RVMSP provides a better clinical utility, compared with RVAP, in patients with high-degree atrioventricular block and moderately depressed LV function whose LVEF levels ranged from 35% to 40%.

CASE REPORT Arrythmogenic right ventricular dysplasia ...

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revised in 2010.6,7 Cardiovascular magnetic resonance (CMR) findings ... Gadolinium was ... images. Global decreased contractility of the RV was recorded with the .... current MRI techniques to detect a small amount of fibrosis in early.

Arrythmogenic right ventricular dysplasia/cardiomyopathy | Scholtz ...

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SA Journal of Radiology. Journal Home · ABOUT THIS JOURNAL · Advanced Search · Current Issue · Archives · Journal Home > Vol 15, No 3 (2011) >. Log in or Register to get access to full text downloads.

Exercise and the right ventricle: a potential Achilles' heel.

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La Gerche, Andre; Rakhit, Dhrubo J; Claessen, Guido

2017-10-01

Exercise is associated with unequivocal health benefits and results in many structural and functional changes of the myocardium that enhance performance and prevent heart failure. However, intense exercise also presents a significant hemodynamic challenge in which the right-sided heart chambers are exposed to a disproportionate increase in afterload and wall stress that can manifest as myocardial fatigue or even damage if intense exercise is sustained for prolonged periods. This review focuses on the physiological factors that result in a disproportionate load on the right ventricle during exercise and the long-term consequences. The changes in cardiac structure and function that define 'athlete's heart' disproportionately affect the right-sided heart chambers and this can raise important diagnostic overlap with some cardiac pathologies, particularly some inherited cardiomyopathies. The interaction between exercise and arrhythmogenic right ventricular cardiomyopathy (ARVC) will be highlighted as an important example of how hemodynamic stressors can combine with deficiencies in cardiac structural elements to cause cardiac dysfunction predisposing to arrhythmias. The extent to which extreme exercise can cause adverse remodelling in the absence of a genetic predisposition remains controversial. In the athlete with profound changes in heart structure, it can be extremely challenging to determine whether common symptoms such as palpitations may be a marker of more sinister arrhythmias. This review discusses some of the techniques that have recently been proposed to identify pathology in these circumstances. Finally, we will discuss recent evidence defining the role of exercise restriction as a therapeutic intervention in individuals predisposed to arrhythmogenic cardiomyopathy. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2017. For permissions, please email: journals.permissions@oup.com.

MicroRNAs in right ventricular remodelling.

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Batkai, Sandor; Bär, Christian; Thum, Thomas

2017-10-01

Right ventricular (RV) remodelling is a lesser understood process of the chronic, progressive transformation of the RV structure leading to reduced functional capacity and subsequent failure. Besides conditions concerning whole hearts, some pathology selectively affects the RV, leading to a distinct RV-specific clinical phenotype. MicroRNAs have been identified as key regulators of biological processes that drive the progression of chronic diseases. The role of microRNAs in diseases affecting the left ventricle has been studied for many years, however there is still limited information on microRNAs specific to diseases in the right ventricle. Here, we review recently described details on the expression, regulation, and function of microRNAs in the pathological remodelling of the right heart. Recently identified strategies using microRNAs as pharmacological targets or biomarkers will be highlighted. Increasing knowledge of pathogenic microRNAs will finally help improve our understanding of underlying distinct mechanisms and help utilize novel targets or biomarkers to develop treatments for patients suffering from right heart diseases. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2017. For permissions, please email: journals.permissions@oup.com.

Right ventricular ejection fraction: an indicator of increased mortality in patients with congestive heart failure associated with coronary artery disease

International Nuclear Information System (INIS)

Polak, J.F.; Holman, B.L.; Wynne, J.; Colucci, W.S.

1983-01-01

The predictive value of radionuclide ventriculography was studied in 34 patients with depressed left ventricular ejection fraction (less than 40%) and clinically evident congestive heart failure secondary to atherosclerotic coronary artery disease. In addition to left ventricular ejection fraction, right ventricular ejection fraction and extent of left ventricular paradox were obtained in an attempt to identify a subgroup at increased risk of mortality during the ensuing months. The 16 patients who were alive after a 2 year follow-up period had a higher right ventricular ejection fraction and less extensive left ventricular dyskinesia. When a right ventricular ejection fraction of less than 35% was used as a discriminant, mortality was significantly greater among the 21 patients with a depressed right ventricular ejection fraction (71 versus 23%), a finding confirmed by a life table analysis. It appears that the multiple factors contributing to the reduction in right ventricular ejection fraction make it a useful index not only for assessing biventricular function, but also for predicting patient outcome

Doubly committed subarterial ventricular septal defect with prolapsed right coronary cusp with moderate aortic regurgitation

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Redoy Ranjan

2017-11-01

Full Text Available A 4 year old girl was presented with the respiratory tract infection, breathlessness after taking meal, failure to thrive, abnormal movement of the chest on left side overlying the area of heart and systolic murmur. She developed these symptoms gradually for the last 3.5 years. Echocardiography revealed doubly committed subarterial ventricular septal defect with moderate aortic regurgitation. The size of the ventricular septal defect was 7 x 9 mm at the left ventricular outflow tract. The right coronary cusp of the aortic valve was prolapsed. Left atrium and left ventricle were dilated. The pulmonary artery systolic pressure was 35 mm Hg. The ventricular septal defect was closed with the standard surgical procedure using cardiopulmonary bypass followed by aortotomy and right atriotomy. Immediate post-operative period of this case was uneventful and the patient was discharged on 9th post-operative day. Follow-up echocardiography showed no residual ventricular septal defect or aortic regurgitation and the ventricular function was good.

Supracristal ventricular septal defect with severe right coronary cusp prolapse

International Nuclear Information System (INIS)

Hussain, A.H.; Hanif, B.; Khan, G.; Hasan, K.

2011-01-01

The case of a 20 years old male, diagnosed as supracristal ventricular septal defect (VSD) for last 6 years is being presented. He came in emergency department with decompensated congestive cardiac failure. After initial stabilization, he underwent trans thoracic echocardiogram which showed large supracristal VSD, severely prolapsing right coronary cusp, severe aortic regurgitation and severe pulmonary hypertension. Right heart catheterization was performed which documented reversible pulmonary vascular resistance after high flow oxygen inhalation. He underwent VSD repair, right coronary cusp was excised and aortic valve was replaced by mechanical prosthesis. Post operative recovery was uneventful. He was discharged home in one week.The case of a 20 years old male, diagnosed as supracristal ventricular septal defect (VSD) for last 6 years is being presented. He came in emergency department with decompensated congestive cardiac failure. After initial stabilization, he underwent trans thoracic echocardiogram which showed large supracristal VSD, severely prolapsing right coronary cusp, severe aortic regurgitation and severe pulmonary hypertension. Right heart catheterization was performed which documented reversible pulmonary vascular resistance after high flow oxygen inhalation. He underwent VSD repair, right coronary cusp was excised and aortic valve was replaced by mechanical prosthesis. Post operative recovery was uneventful. He was discharged home in one week. (author)

Evolutionary changes in left and right ventricular function in acute myocardial infarction

International Nuclear Information System (INIS)

Ohsuzu, Fumitaka; Yasuda, Tsunehiro; Gold, H.K.; Leinbach, R.C.; Rosenthal, S.V.; Alpert, N.M.; Boucher, C.A.; McKusick, K.A.; Strauss, H.W.

1987-01-01

To determine the evolutionary changes in right and left ventricular function in acute myocardial infarction, 3 serial gated blood pool scans were performed in 76 patients within 24 hours (24 H), at 10 days (10 D) and 3 months (3 M) following the onset of myocardial infarction. The patients were divided into 3 groups: ANT (anterior MI), INF (inferior MI without right ventricular dysfunction) and RVF (inferior MI with right ventricular dysfunction). LVEF in ANT was significantly lower than that of INF and RVF at 24 H, 10 D and 3 M. The ratio of right ventricular volume to LV volume (RVV/LVV) was compared among 3 groups. The mean values of RVV/LVV in RVF were 1.3 through 24 H and 3 M and they were significantly higher than the other two groups. The RVV/LVV in ANT and INF were around 1.0. LVEDVI in RVF was rather smaller than that of ANT and INF. LVESVI in ANT at 24 H was significantly larger than that of INF and RVF and the mean value of LVESVI in ANT were around 60 ml/M 2 from 24 H to 3 M. LVEF in ANT, RVF and INF did not increase significantly during peak exercise at 3 M. However, quantitative regional wall motion analysis revealed that regional wall motion of R2 (posterolateral wall motion) in ANT and R5 (septal wall motion) in INF decreased significantly during peak exercise. These impairments in regional wall motion might be due to the exacerbation of ischemia of non-infarcted area. (author)

Evolutionary changes in left and right ventricular function in acute myocardial infarction

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Ohsuzu, Fumitaka; Yasuda, Tsunehiro; Gold, H K; Leinbach, R C; Rosenthal, S V; Alpert, N M; Boucher, C A; McKusick, K A; Strauss, H W

1987-09-01

To determine the evolutionary changes in right and left ventricular function in acute myocardial infarction, 3 serial gated blood pool scans were performed in 76 patients within 24 hours (24 H), at 10 days (10 D) and 3 months (3 M) following the onset of myocardial infarction. The patients were divided into 3 groups: ANT (anterior MI), INF (inferior MI without right ventricular dysfunction) and RVF (inferior MI with right ventricular dysfunction). LVEF in ANT was significantly lower than that of INF and RVF at 24 H, 10 D and 3 M. The ratio of right ventricular volume to LV volume (RVVLVV) was compared among 3 groups. The mean values of RVVLVV in RVF were 1.3 through 24 H and 3 M and they were significantly higher than the other two groups. The RVVLVV in ANT and INF were around 1.0. LVEDVI in RVF was rather smaller than that of ANT and INF. LVESVI in ANT at 24 H was significantly larger than that of INF and RVF and the mean value of LVESVI in ANT were around 60 mlM/sup 2/ from 24 H to 3 M. LVEF in ANT, RVF and INF did not increase significantly during peak exercise at 3 M. However, quantitative regional wall motion analysis revealed that regional wall motion of R2 (posterolateral wall motion) in ANT and R5 (septal wall motion) in INF decreased significantly during peak exercise. These impairments in regional wall motion might be due to the exacerbation of ischemia of non-infarcted area

The Achilles' heel of left ventricular assist device therapy: right ventricle.

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Ranganath, Neel K; Smith, Deane E; Moazami, Nader

2018-06-01

Many patients suffer from either persistent right ventricular failure (RVF) at the time of left ventricular assist device (LVAD) or have ongoing symptoms consistent with RVF during chronic mechanical circulatory support. The lack of long-term right ventricular assist devices (RVADs) has limited the impact that mechanical circulatory support can provide to patients with biventricular failure. We aim to review the entire spectrum of RVF in patients receiving LVADs and reflect on why this entity remains the Achilles' heel of LVAD therapy. In the early postoperative period, LVAD implantation reduces right ventricle (RV) afterload, but RV dysfunction may be exacerbated secondary to increased venous return. With prolonged therapy, the decreased RV afterload leads to improved RV contractile function. Bayesian statistical models outperform previously published preoperative risk scores by considering inter-relationships and conditional probabilities amongst independent variables. Various echocardiographic parameters and the pulmonary artery pulsatility index have shown promise in predicting post-LVAD RVF. Recent publications have delineated the emergence of 'delayed' RVF. Several devices are currently being investigated for use as RVADs. Post-LVAD RVF depends on the RV's ability to adapt to acute hemodynamic changes imposed by the LVAD. Management options are limited due to the lack of an easily implantable, chronic-use RVAD.

The Effect of Elective Percutaneous Coronary Intervention of the Right Coronary Artery on Right Ventricular Function

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Farahnaz Nikdoust

2014-12-01

Full Text Available Background: Right Ventricular (RV dysfunction has been introduced as a predictor of mortality in acute myocardial infarction. Objectives: This study aimed to investigate the effect of right coronary revascularization on systolic and diastolic RV dysfunction. Patients and Methods: This study was conducted on unstable angina patients who were candidate for elective Percutaneous Revascularization Intervention (PCI on the right coronary artery. The participants were initially evaluated by transthoracic echocardiography and tissue Doppler imaging prior to PCI and the RV function parameters were assessed. Echocardiography was repeated two months after PCI and the results were compared with baseline. Paired t-test was used to compare the pre- and post-procedural measurements. Besides, Pearson’s correlation was used to find out the linear association between the RV function parameters and Left Ventricular Ejection Fraction (LVEF. P value < 0.05 was considered as statistically significant. Results: This study was conducted on 30 patients (mean age = 60.00 ± 8.44 years; 24 [80%] males. In the pre-procedural echocardiography, 15 patients (50% had normal RV function, 14 patients (46.7% had grade-1 RV dysfunction, and only 1 patient (3.3% had grade-2 RV dysfunction. Following PCI, however, all the patients had normal systolic and diastolic RV functions. Comparison of echocardiographic RV function parameters showed an improvement in both systolic and diastolic functional parameters of the RV. Nonetheless, no significant correlation was observed between these parameters and Left Ventricular (LV function. Conclusions:: A significant improvement was found in RV function, but not LV function, after right coronary PCI. Revascularization of the right coronary artery may be beneficial for the patients who suffer from RV failure due to ischemia

Stenting of the right ventricular outflow tract in 2 dogs for palliation of dysplastic pulmonary valve stenosis and right-to-left intracardiac shunting defects.

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Scansen, Brian A; Kent, Agnieszka M; Cheatham, Sharon L; Cheatham, John P; Cheatham, John D

2014-09-01

Two dogs with severe dysplastic pulmonary valve stenosis and right-to-left shunting defects (patent foramen ovale, perimembranous ventricular septal defect) underwent palliative stenting of the right ventricular outflow tract and pulmonary valve annulus using balloon expandable stents. One dog received 2 over-lapping bare metal stents placed 7 months apart; the other received a single covered stent. Both procedures were considered technically successful with a reduction in the transpulmonary valve pressure gradient from 202 to 90 mmHg in 1 dog and from 168 to 95 mmHg in the other. Clinical signs of exercise intolerance and syncope were temporarily resolved in both dogs. However, progressive right ventricular concentric hypertrophy, recurrent stenosis, and erythrocytosis were observed over the subsequent 6 months leading to poor long-term outcomes. Stenting of the right ventricular outflow tract is feasible in dogs with severe dysplastic pulmonary valve stenosis, though further study and optimization of the procedure is required. Copyright © 2014 Elsevier B.V. All rights reserved.

Three-Dimensional Echocardiography-Derived Non-Invasive Right Ventricular Pressure-Volume Analysis.

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Huang, Kuan-Chih; Lin, Lian-Yu; Hwang, Juey-Jen; Lin, Lung-Chun

2017-09-01

In patients with pulmonary hypertension, repeated evaluations of right ventricular (RV) function are still required for clinical decision making, but the invasive nature of current pressure-volume analysis makes conducting regular follow-ups in a clinical setting infeasible. We enrolled 12 patients with pulmonary arterial hypertension (PAH) and 10 with pulmonary venous hypertension (PVH) May 2016-October 2016. All patients underwent a clinically indicated right heart catheterization (RHC), from which the yielded right ventricular pressure recordings were conjugated with RV volume by 3-D echocardiography to generate a pressure-volume loop. A continuous-wave Doppler envelope of tricuspid regurgitation was transformed into a pressure gradient recording by the simplified Bernoulli equation, and then a systolic pressure gradient-volume (PG-V) diagram was generated from similar methods. The area enclosed by the pressure-volume loop was calculated to represent semi-invasive right ventricular stroke work (RVSW RHC ). The area between the PG-V diagram and x-axis was calculated to estimate non-invasive RVSW (RVSW echo ). Patients with PAH have higher RV pressure, lower pulmonary arterial wedge pressure and larger RV volume that was contributed by the dilation of RV mid-cavity minor dimension. We found no significant difference of traditional parameters between these two groups, but RVSW values were significantly higher in PAH patients. The RVSW values of these two methods were significantly correlated by the equation RVSW echo  = 0.8447 RVSW RHC  + 129.38 (R 2  = 0.9151, p rights reserved.

Diastolic And Systolic Right Ventricular Dysfunction Precedes Left Ventricular Dysfunction In Patients Paced From Right Ventricular Apex

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Dwivedi SK

2006-07-01

Full Text Available Background: Cardiac dysfunction after right ventricular (RV apical pacing is well known but its extent, time frame of appearance and individual effect on left ventricular (LV, RV systolic and diastolic parameters has not evaluated in a systematic fashion. Methods: Patients with symptomatic bradycardia and ACC-AHA Class I indication for permanent pacemaker implantation (PPI were implanted a single chamber (VVI pacemaker. They were followed prospectively by echocardiographic examination which was done at baseline, 1 week, 1 month and 6 months after implantation. Parameters observed were chamber dimensions (M-line, chamber volumes, cardiac output (modified Simpson's method, systolic functions (ejection fraction, pre-ejection period, ejection time and ratio and diastolic functions( isovolumic relaxation time & deceleration time of left and right heart. Results: Forty eight consecutive patients (mean age 65.6±11.8 yrs, 66.7% males, mean EF 61.82±10.36% implanted a VVI pacemaker were enrolled in this study. The first significant change to appear in cardiac function after VVI pacing was in diastolic properties of RV as shown by increase in RV isovolumic relaxation time (IVRT from 65.89±15.93 to 76.58±17.00 ms,(p<0.001 at 1week and RV deceleration time (DT from 133.84±38.13 to 153.09±31.41 ms, (p=0.02 at 1 month. Increase in RV internal dimension (RVID from 1.26±0.41 to 1.44±0.44, (p<0.05 was also noticed at 1 week. The LV diastolic parameters were significantly altered after 1 month with increase in LV-IVRT from 92.36±21.47 to 117.24±27.21ms, (p<0.001 and increase in LV DT from 147.56±31.84 to 189.27±28.49ms,(p<0.01. This was followed by LV systolic abnormality which appeared at 6 months with an increase in LVPEP from 100.33±14.43 to 118.41±21.34ms, (p<0.001 and increase in LVPEP/LVET ratio from 0.34±0.46 to 0.44±0.10, (p<0.001]. The reduction in LV EF was manifested at 6 months falling from 61.82±10.36% to52.52±12.11%, (p<0

Late deterioration of left ventricular function after right ventricular pacemaker implantation.

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Bellmann, Barbara; Muntean, Bogdan G; Lin, Tina; Gemein, Christopher; Schmitz, Kathrin; Schauerte, Patrick

2016-09-01

Right ventricular (RV) pacing induces a left bundle branch block pattern on ECG and may promote heart failure. Patients with dual chamber pacemakers (DCPs) who present with progressive reduction in left ventricular ejection fraction (LVEF) secondary to RV pacing are candidates for cardiac resynchronization therapy (CRT). This study analyzes whether upgrading DCP to CRT with the additional implantation of a left ventricular (LV) lead improves LV function in patients with reduced LVEF following DCP implantation. Twenty-two patients (13 males) implanted with DCPs and a high RV pacing percentage (>90%) were evaluated in term of new-onset heart failure symptoms. The patients were enrolled in this retrospective single-center study after obvious causes for a reduced LVEF were excluded with echocardiography and coronary angiography. In all patients, DCPs were then upgraded to biventricular devices. LVEF was analyzed with a two-sided t-test. QRS duration and brain natriuretic peptide (BNP) levels were analyzed with the unpaired t-test. LVEF declined after DCP implantation from 54±10% to 31±7%, and the mean QRS duration was 161±20 ms during RV pacing. NT-pro BNP levels were elevated (3365±11436 pmol/L). After upgrading to a biventricular device, a biventricular pacing percentage of 98.1±2% was achieved. QRS duration decreased to 108±16 ms and 106±20 ms after 1 and 6 months, respectively. There was a significant increase in LVEF to 38±8% and 41±11% and a decrease in NT-pro BNP levels to 3088±2326 pmol/L and 1860±1838 pmol/L at 1 and 6 months, respectively. Upgrading to CRT may be beneficial in patients with DCPs and heart failure induced by a high RV pacing percentage.

Effects of right ventricular morphology and function on outcomes of patients with degenerative mitral valve disease.

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Ye, Ying; Desai, Ravi; Vargas Abello, Lina M; Rajeswaran, Jeevanantham; Klein, Allan L; Blackstone, Eugene H; Pettersson, Gösta B

2014-11-01

The study objective was to investigate whether, in patients undergoing surgery for degenerative mitral valve disease, associated right ventricular remodeling and dysfunction are stronger determinants of preoperative organ dysfunction and prognosis than functional tricuspid regurgitation. From January 2001 to January 2011, 4197 patients underwent primary mitral valve surgery for degenerative valve disease at Cleveland Clinic. Using a quasi-experimental enriched study design, 781 patients were randomly selected within each grade of functional tricuspid regurgitation. Renal function was assessed by glomerular filtration rate and blood urea nitrogen, and hepatic function was assessed by Model for End-stage Liver Disease (MELD) score. Preoperative and postoperative right ventricular morphology and function were measured de novo on stored echocardiographic images. To assess survival, 3471 patient-years of follow-up data were available for analysis. Several preoperative right ventricular variables, but not functional tricuspid regurgitation grade (P>.05), were associated with preoperative renal and hepatic dysfunction, but neither was associated with early mortality (.2), was among the risk factors for later mortality. Postoperative unadjusted right ventricular function, but not functional tricuspid regurgitation grade (P≥.2), was associated with both early (P=.04) and later (P=.01) mortality, but in multivariable analysis appeared to be a surrogate for worse preoperative condition. Together with patient comorbidities and operative factors, right ventricular morphology and function are associated more strongly with preoperative organ dysfunction and prognosis than is functional tricuspid regurgitation severity in patients undergoing surgery for degenerative mitral valve disease. Our previous study showed that tricuspid valve repair remains the most effective treatment to improve right ventricular function. Copyright © 2014 The American Association for Thoracic Surgery

Fibromuscular Dysplasia Leading to Spontaneous Coronary Artery Dissection with Sudden Cardiac Arrest

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Ata Bajwa

2015-01-01

Full Text Available A 30-year-old previously healthy female, who was six-week postpartum, experienced sudden collapse and tonic-clonic seizure. Emergency medicine services arrived at the scene and the patient was found to be in ventricular fibrillation. Advanced cardiovascular life support (ACLS was initiated with return of spontaneous circulation. Afterwards, her initial EKG showed atrial fibrillation with rapid ventricular rate, ST elevation in leads II, III, and aVF, and ST depression in V2–V4. She was transferred to a tertiary care hospital where emergent angiogram was performed revealing obstruction of blood flow in the proximal and mid right coronary artery (RCA. A hazy and irregularly contoured appearance of the RCA was consistent with diagnosis of fibromuscular dysplasia. Subsequently, intravascular ultrasonogram (IVUS was performed which confirmed the diagnosis of RCA dissection. Successful revascularization of the RCA was performed using two bare mental stents. After a complicated course in hospital, she was discharged in stable condition and did very well overall.

Right ventricular function assessment in single LAD lesion patients ...

African Journals Online (AJOL)

Rania Gaber

2015-10-09

Oct 9, 2015 ... Doppler method in patients with single LAD lesion. Methods: The patient group was ... Results: The right ventricular tissue Doppler parameters (Sm, E, A, E/A ratio, IVA, E/E00) of the patients group were significantly .... cardial interaction effect of tethered LV anterior myocardium. Mittal et al. reported that Left ...

Validation of attenuation-corrected equilibrium radionuclide angiographic determinations of right ventricular volume: comparison with cast-validated biplane cineventriculography

International Nuclear Information System (INIS)

Dell'Italia, L.J.; Starling, M.R.; Walsh, R.A.; Badke, F.R.; Lasher, J.C.; Blumhardt, R.

1985-01-01

To determine the accuracy of attenuation-corrected equilibrium radionuclide angiographic determinations of right ventricular volumes, the authors initially studied 14 postmortem human right ventricular casts by water displacement and biplane cineventriculography. Biplane cineventriculographic right ventricular cast volumes, calculated by a modification of Simpson's rule algorithm, correlated well with right ventricular cast volumes measured by water displacement (r = .97, y = 8 + 0.88x, SEE = 6 ml). Moreover, the mean volumes obtained by both methods were no different (73 +/- 28 vs 73 +/- 25 ml). Subsequently, they studied 16 patients by both biplane cineventriculography and equilibrium radionuclide angiography. The uncorrected radionuclide right ventricular volumes were calculated by normalizing background corrected end-diastolic and end-systolic counts from hand-drawn regions of interest obtained by phase analysis for cardiac cycles processed, frame rate, and blood sample counts. Attenuation correction was performed by a simple geometric method. The attenuation-corrected radionuclide right ventricular end-diastolic volumes correlated with the cineventriculographic end-diastolic volumes (r = .91, y = 3 + 0.92x, SEE = 27 ml). Similarly, the attenuation-corrected radionuclide right ventricular end-systolic volumes correlated with the cineventriculographic end-systolic volumes (r = .93, y = - 1 + 0.91x, SEE = 16 ml). Also, the mean attenuation-corrected radionuclide end-diastolic and end-systolic volumes were no different than the average cineventriculographic end-diastolic and end-systolic volumes (160 +/- 61 and 83 +/- 44 vs 170 +/- 61 and 86 +/- 43 ml, respectively)

Exploring digenic inheritance in arrhythmogenic cardiomyopathy.

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König, Eva; Volpato, Claudia Béu; Motta, Benedetta Maria; Blankenburg, Hagen; Picard, Anne; Pramstaller, Peter; Casella, Michela; Rauhe, Werner; Pompilio, Giulio; Meraviglia, Viviana; Domingues, Francisco S; Sommariva, Elena; Rossini, Alessandra

2017-12-08

Arrhythmogenic cardiomyopathy (ACM) is an inherited genetic disorder, characterized by the substitution of heart muscle with fibro-fatty tissue and severe ventricular arrhythmias, often leading to heart failure and sudden cardiac death. ACM is considered a monogenic disorder, but the low penetrance of mutations identified in patients suggests the involvement of additional genetic or environmental factors. We used whole exome sequencing to investigate digenic inheritance in two ACM families where previous diagnostic tests have revealed a PKP2 mutation in all affected and some healthy individuals. In family members with PKP2 mutations we determined all genes that harbor variants in affected but not in healthy carriers or vice versa. We computationally prioritized the most likely candidates, focusing on known ACM genes and genes related to PKP2 through protein interactions, functional relationships, or shared biological processes. We identified four candidate genes in family 1, namely DAG1, DAB2IP, CTBP2 and TCF25, and eleven candidate genes in family 2. The most promising gene in the second family is TTN, a gene previously associated with ACM, in which the affected individual harbors two rare deleterious-predicted missense variants, one of which is located in the protein's only serine kinase domain. In this study we report genes that might act as digenic players in ACM pathogenesis, on the basis of co-segregation with PKP2 mutations. Validation in larger cohorts is still required to prove the utility of this model.

Acute and chronic effects of dysfunction of right ventricular outflow tract components on right ventricular performance in a porcine model: implications for primary repair of tetralogy of fallot

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Bove, Thierry; Bouchez, Stefaan; de Hert, Stefan; Wouters, Patrick; de Somer, Filip; Devos, Daniel; Somers, Pamela; van Nooten, Guido

2012-01-01

This study investigates the contribution of infundibular versus pulmonary valve (PV) dysfunction on right ventricular (RV) function in a porcine model. Clinical outcome after repair of tetralogy of Fallot is determined by the adaptation of the right ventricle to the physiological sequelae of the

Early Right Ventricular Apical Pacing-Induced Gene Expression Alterations Are Associated with Deterioration of Left Ventricular Systolic Function

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Haiyan Xu

2017-01-01

Full Text Available The chronic high-dose right ventricular apical (RVA pacing may have deleterious effects on left ventricular (LV systolic function. We hypothesized that the expression changes of genes regulating cardiomyocyte energy metabolism and contractility were associated with deterioration of LV function in patients who underwent chronic RVA pacing. Sixty patients with complete atrioventricular block and preserved ejection fraction (EF who underwent pacemaker implantation were randomly assigned to either RVA pacing (n=30 group or right ventricular outflow tract (RVOT pacing (n=30 group. The mRNA levels of OPA1 and SERCA2a were significantly lower in the RVA pacing group at 1 month’s follow-up (both p<0.001. Early changes in the expression of selected genes OPA1 and SERCA2a were associated with deterioration in global longitudinal strain (GLS that became apparent months later (p=0.002 and p=0.026, resp. The altered expressions of genes that regulate cardiomyocyte energy metabolism and contractility measured in the peripheral blood at one month following pacemaker implantation were associated with subsequent deterioration in LV dyssynchrony and function in patients with preserved LVEF, who underwent RVA pacing.

A case report of the fibrous dysplasia

International Nuclear Information System (INIS)

You, Dong Soo

1975-01-01

The author observed a rare case of fibrous dysplasia in 12 year old female who came to the Infirmary of Dental College, Seoul National University, complaining of facial asymmetry of 3 years' duration in right maxillofacial region. The serial radiograms has been taken, and the nature of the lesion established as a typical fibrous dysplasia according to the interpreted findings in their images. The author has obtained the results as follows: 1. Fibrous dysplasia occurred at 3 years of age in this case. 2. On familial tendency, traumatic history and endocrine disturbances were not noted in this patient. 3. The serial radiograms revealed a typical fibrous dysplasia encroaching right zygomatic bone.

A case report of the fibrous dysplasia

Energy Technology Data Exchange (ETDEWEB)

You, Dong Soo [Department of Radiology, College of Dentistry, Seoul National University, Seoul (Korea, Republic of)

1975-11-15

The author observed a rare case of fibrous dysplasia in 12 year old female who came to the Infirmary of Dental College, Seoul National University, complaining of facial asymmetry of 3 years' duration in right maxillofacial region. The serial radiograms has been taken, and the nature of the lesion established as a typical fibrous dysplasia according to the interpreted findings in their images. The author has obtained the results as follows: 1. Fibrous dysplasia occurred at 3 years of age in this case. 2. On familial tendency, traumatic history and endocrine disturbances were not noted in this patient. 3. The serial radiograms revealed a typical fibrous dysplasia encroaching right zygomatic bone.

Right heart failure due to loss of right ventricular capture in a patient with atrioventricular junction ablation and biventricular pacing.

Science.gov (United States)

Raffa, Santi; Fantoni, Cecilia; Restauri, Luigia; Auricchio, Angelo

2005-10-01

We describe the case of a patient with atrioventricular (AV) junction ablation and chronic biventricular pacing in which intermittent dysfunction of the right ventricular (RV) lead resulted in left ventricular (LV) stimulation alone and onset of severe right heart failure. Restoration of biventricular pacing by increasing device output and then performing lead revision resolved the issue. This case provides evidence that LV pacing alone in patients with AV junction ablation may lead to severe right heart failure, most likely as a result of iatrogenic mechanical dyssynchrony within the RV. Thus, probably this pacing mode should be avoided in pacemaker-dependent patients with heart failure.

Usefulness of ventricular endocardial electric reconstruction from body surface potential maps to noninvasively localize ventricular ectopic activity in patients

Science.gov (United States)

Lai, Dakun; Sun, Jian; Li, Yigang; He, Bin

2013-06-01

As radio frequency (RF) catheter ablation becomes increasingly prevalent in the management of ventricular arrhythmia in patients, an accurate and rapid determination of the arrhythmogenic site is of important clinical interest. The aim of this study was to test the hypothesis that the inversely reconstructed ventricular endocardial current density distribution from body surface potential maps (BSPMs) can localize the regions critical for maintenance of a ventricular ectopic activity. Patients with isolated and monomorphic premature ventricular contractions (PVCs) were investigated by noninvasive BSPMs and subsequent invasive catheter mapping and ablation. Equivalent current density (CD) reconstruction (CDR) during symptomatic PVCs was obtained on the endocardial ventricular surface in six patients (four men, two women, years 23-77), and the origin of the spontaneous ectopic activity was localized at the location of the maximum CD value. Compared with the last (successful) ablation site (LAS), the mean and standard deviation of localization error of the CDR approach were 13.8 and 1.3 mm, respectively. In comparison, the distance between the LASs and the estimated locations of an equivalent single moving dipole in the heart was 25.5 ± 5.5 mm. The obtained CD distribution of activated sources extending from the catheter ablation site also showed a high consistency with the invasively recorded electroanatomical maps. The noninvasively reconstructed endocardial CD distribution is suitable to predict a region of interest containing or close to arrhythmia source, which may have the potential to guide RF catheter ablation.

Epicardial deployment of right ventricular disk during perventricular device closure in a child with apical muscular ventricular septal defect

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Nageswara Rao Koneti

2013-01-01

Full Text Available We report a successful perventricular closure of an apical muscular ventricular septal defect (mVSD by a modified technique. An eight-month-old infant, weighing 6.5 kilograms, presented with refractory heart failure. The transthoracic echocardiogram showed multiple apical mVSDs with the largest one measuring 10 mm. perventricular device closure using a 12 mm Amplatzer mVSD occluder was planned. The left ventricular disk was positioned approximating the interventricular septum; however, the right ventricular (RV disk was deployed on the free wall of the RV due to an absent apical muscular septum and a small cavity at the apex. The RV disk of the device was covered using an autologous pericardium. His heart failure improved during follow-up.

[Interest of tricuspid annular displacement (TAD) in evaluation of right ventricular ejection fraction].

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Hugues, T; Ducreux, D; Bertora, D; Berthier, F; Lemoigne, F; Padovani, B; Gibelin, P

2010-04-01

The ultrasound assessment of RV structure and function is often sub-optimal. The range of excursions of the mitral or tricuspid annulus measured in millimetre by 2D or TM-mode in centimetre per second by DTI-mode echocardiography has been shown to reflect the systolic function of both ventricles. We studied a new technique based on a tissue tracking algorithm that is ultrasound beam angle independent for automated detection of tricuspid annular displacement (TAD) (QLAB, Philips Medical Imaging). Twenty-six patients (pts) referred for magnetic resonance imaging (MRI) and 44 control subjects underwent a complete transthoracic echocardiography. MRI of the right ventricular ejection fraction (RVEF) was correlated by linear regression with TAD. Sixteen pts (61.5%) exhibited right ventricular systolic dysfunction (MRI RVEFTAD (R(2)=0,65; pTAD TAD values exceeding this cut-off point (mean: 16.9+/-1.64mm; range: 13.3 to 24.8mm). Negative correlation was found between TAD and age (R(2)=0,36; pTAD with MRI RVEF. TAD is a simple, rapid, and non-invasive tool for right ventricular systolic function assessment.

A new "twist" on right heart failure with left ventricular assist systems.

Science.gov (United States)

Houston, Brian A; Shah, Keyur B; Mehra, Mandeep R; Tedford, Ryan J

2017-07-01

Despite significant efforts to predict and prevent right heart failure, it remains a leading cause of morbidity and mortality after implantation of left ventricular assist systems (LVAS). In this Perspective, we review the underappreciated anatomic and physiologic principles that govern the relationship between left and right heart function and contribute to this phenomenon. This includes the importance of considering the right ventricle (RV) and pulmonary arterial circuit as a coupled system; the contribution of the left ventricle (LV) to RV contractile function and the potential negative impact of acutely unloading the LV; the influence of the pericardium and ventricular twist on septal function; the role of RV deformation in reduced mechanical efficiency after device placement; and the potential of ongoing stressors of an elevated right-sided preload. We believe an appreciation of these complex issues is required to fully understand the expression of the unique phenotypes of right heart failure after LVAS implantation and for developing better prognostic and therapeutic strategies. Copyright © 2017 International Society for the Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.

Transient Outward K+ Current (Ito) Underlies the Right Ventricular Initiation of Polymorphic Ventricular Tachycardia in a Transgenic Rabbit Model of Long-QT Syndrome Type 1.

Science.gov (United States)

Choi, Bum-Rak; Li, Weiyan; Terentyev, Dmitry; Kabakov, Anatoli Y; Zhong, Mingwang; Rees, Colin M; Terentyeva, Radmila; Kim, Tae Yun; Qu, Zhilin; Peng, Xuwen; Karma, Alain; Koren, Gideon

2018-06-01

Sudden death in long-QT syndrome type 1 (LQT1), an inherited disease caused by loss-of-function mutations in KCNQ1, is triggered by early afterdepolarizations (EADs) that initiate polymorphic ventricular tachycardia (pVT). We investigated ionic mechanisms that underlie pVT in LQT1 using a transgenic rabbit model of LQT1. Optical mapping, cellular patch clamping, and computer modeling were used to elucidate the mechanisms of EADs in transgenic LQT1 rabbits. The results showed that shorter action potential duration in the right ventricle (RV) was associated with focal activity during pVT initiation. RV cardiomyocytes demonstrated higher incidence of EADs under 50 nmol/L isoproterenol. Voltage-clamp studies revealed that the transient outward potassium current (I to ) magnitude was 28% greater in RV associated with KChiP2 but with no differences in terms of calcium-cycling kinetics and other sarcolemmal currents. Perfusing with the I to blocker 4-aminopyridine changed the initial focal sites of pVT from the RV to the left ventricle, corroborating the role of I to in pVT initiation. Computer modeling showed that EADs occur preferentially in the RV because of the larger conductance of the slow-inactivating component of I to , which repolarizes the membrane potential sufficiently rapidly to allow reactivation of I Ca,L before I Kr has had sufficient time to activate. I to heterogeneity creates both triggers and an arrhythmogenic substrate in LQT1. In the absence of I Ks , I to interactions with I Ca,L and I Kr promote EADs in the RV while prolonging action potential duration in the left ventricle. This heterogeneity of action potential enhances dispersion of refractoriness and facilitates conduction blocks that initiate pVTs. © 2018 American Heart Association, Inc.

Right ventricular function after repair of tetralogy of Fallot: a comparison between bovine pericardium and porcine small intestinal extracellular matrix.

Science.gov (United States)

Naik, Ronak; Johnson, Jason; Kumar, T K S; Philip, Ranjit; Boston, Umar; Knott-Craig, Christopher J

2017-05-29

The porcine small intestinal extracellular matrix reportedly has the potential to differentiate into viable myocardial cells. When used in tetralogy of Fallot repair, it may improve right ventricular function. We evaluated right ventricular function after repair of tetralogy of Fallot with extracellular matrix versus bovine pericardium. Subjects with non-transannular repair of tetralogy of Fallot with at least 1 year of follow-up were selected. The extracellular matrix and bovine pericardium groups were compared. We used three-dimensional right ventricular ejection fraction, right ventricle global longitudinal strain, and tricuspid annular plane systolic excursion to assess right ventricular function. The extracellular matrix group had 11 patients, whereas the bovine pericardium group had 10 patients. No differences between the groups were found regarding sex ratio, age at surgery, and cardiopulmonary bypass time. The follow-up period was 28±12.6 months in the extracellular matrix group and 50.05±17.6 months in the bovine pericardium group (p=0.001). The mean three-dimensional right ventricular ejection fraction (55.7±5.0% versus 55.3±5.2%, p=0.73), right ventricular global longitudinal strain (-18.5±3.0% versus -18.0±2.2%, p=0.44), and tricuspid annular plane systolic excursions (1.59±0.16 versus 1.59±0.2, p=0.93) were similar in the extracellular matrix group and in the bovine pericardium group, respectively. Right ventricular global longitudinal strain in healthy children is reported at -29±3% in literature. In a small cohort of the patients undergoing non-transannular repair of tetralogy of Fallot, there was no significant difference in right ventricular function between groups having extracellular matrix versus bovine pericardium patches followed-up for more than 1 year. Lower right ventricular longitudinal strain noted in both the groups compared to healthy children.

Assessment of right ventricular function by myocardial performance index in diabetic patients

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Ayman Ahmed Abdelaziz

2011-09-01

Conclusion: Myocardial performance index is a useful noninvasive tool for the detection of early right ventricular systolic and diastolic dysfunction in diabetic patients, regardless of coexisting hypertension.

Non-invasive assessment of right ventricular function at rest and on exercise in obstructive airways disease

International Nuclear Information System (INIS)

Tweddel, A.; Martin, W.; McGhie, I.; Neilly, B.; Stevenson, R.; Hutton, I.

1985-01-01

Non-invasive assessment of right ventricular function is of clinical interest in the patient with obstructive airways disease. Gated Xenon 133 scanning allows right ventricular function to be evaluated in isolation from the left ventricle, and with rapid clearance from the lungs, scans may be repeated within 5 minutes. 400mBq of Xenon 133 were injected intravenously over 20 seconds and images were obtained using a mobile gamma camera. Maximal symptom limited exercise was performed on a supine bicycle ergometer. The normal range for right ventricular ejection fraction (RVEF) was obtained from 10 volunteers - 40-55% at rest rising by 5-15% during exercise. In 10 patients with acute obstructive airways disease, all had reduced RVEF 21 +- 3%. In chronic obstructive airways disease, if resting RVEF was greater than 30%, ejection fraction increased on exercise. If resting ejection fraction was abnormal than RVEF was reduced or unchanged on exercise (mean 15 +- 9%), and this was associated with dilatation of both the right ventricle and atrium. In conclusion, gated Xenon 133 offers a simple method of assessing right ventricular function at rest and on exercise in the patient with obstructive airways disease

Echocardiographic assessment of right ventricular contractile reserve in patients with pulmonary hypertension.

Science.gov (United States)

Almeida, Ana Rita; Loureiro, Maria José; Lopes, Liliana; Cotrim, Carlos; Lopes, Luís; Repolho, Débora; Pereira, Hélder

2014-03-01

Right ventricular function is a major determinant of prognosis in pulmonary hypertension. The aim of this study was to assess and compare right ventricular contractile reserve in healthy subjects (controls) and in subjects with pulmonary hypertension (cases). In this prospective study of seven cases and seven controls undergoing treadmill stress echocardiography, right ventricular S-wave velocity, tricuspid annular plane systolic excursion (TAPSE), right ventricular fractional area change (RVFAC) and stroke volume index were assessed at rest and with exercise. The increase in each parameter between rest and exercise for cases and controls was analyzed and the magnitude of change in each parameter with exercise between cases and controls was compared. A significant increase in S-wave velocity was observed in cases (rest: 9.4 ± 3.1; exercise: 13.7 ± 4.8 cm/s [p < 0.05]). In controls there was a statistically significant increase in S-wave velocity (12.9 ± 2.3 to 23.0 ± 7.2 cm/s [p < 0.005]), TAPSE (25.7 ± 2.4 to 31.0 ± 3.5 mm [p < 0.05]) and RVFAC (53.8 ± 14.7% to 64.4 ± 9.9% [p < 0.005]). The magnitude of change in S-wave velocity (cases: 4.3 ± 3.3; controls: 10.1 ± 5.5 cm/s [p < 0.05]), TAPSE (cases: 0.6 ± 2.5; controls: 5.3 ± 3.8 mm [p < 0.05]) and RVFAC (cases: -0.4 ± 11.8; controls: 10.6 ± 5.9% [p < 0.05]) was significantly different between cases and controls. S-wave velocity, TAPSE and RVFAC increased significantly with exercise in controls. S-wave velocity was the only parameter that showed a significant increase in cases, although the magnitude of this increase was significantly less than in controls. Copyright © 2013 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

Nonischemic changes in right ventricular function on exercise. Do normal volunteers differ from patients with normal coronary arteries

International Nuclear Information System (INIS)

Caplin, J.L.; Maltz, M.B.; Flatman, W.D.; Dymond, D.S.

1988-01-01

Factors other than ischemia may alter right ventricular function both at rest and on exercise. Normal volunteers differ from cardiac patients with normal coronary arteries with regard to their left ventricular response to exercise. This study examined changes in right ventricular function on exercise in 21 normal volunteers and 13 patients with normal coronary arteries, using first-pass radionuclide angiography. There were large ranges of right ventricular ejection fraction in the two groups, both at rest and on exercise. Resting right ventricular ejection fraction was 40.2 +/- 10.6% (mean +/- SD) in the volunteers and 38.6 +/- 9.7% in the patients, p = not significant, and on exercise rose significantly in both groups to 46.1 +/- 9.9% and 45.8 +/- 9.7%, respectively. The difference between the groups was not significant. In both groups some subjects with high resting values showed large decreases in ejection fraction on exercise, and there were significant negative correlations between resting ejection fraction and the change on exercise, r = -0.59 (p less than 0.01) in volunteers, and r = -0.66 (p less than 0.05) in patients. Older volunteers tended to have lower rest and exercise ejection fractions, but there was no difference between normotensive and hypertensive patients in their rest or exercise values. In conclusion, changes in right ventricular function on exercise are similar in normal volunteers and in patients with normal coronary arteries. Some subjects show decreases in right ventricular ejection fraction on exercise which do not appear to be related to ischemia

Effects of projection and background correction method upon calculation of right ventricular ejection fraction using first-pass radionuclide angiography

International Nuclear Information System (INIS)

Caplin, J.L.; Flatman, W.D.; Dymond, D.S.

1985-01-01

There is no consensus as to the best projection or correction method for first-pass radionuclide studies of the right ventricle. We assessed the effects of two commonly used projections, 30 degrees right anterior oblique and anterior-posterior, on the calculation of right ventricular ejection fraction. In addition two background correction methods, planar background correction to account for scatter, and right atrial correction to account for right atrio-ventricular overlap were assessed. Two first-pass radionuclide angiograms were performed in 19 subjects, one in each projection, using gold-195m (half-life 30.5 seconds), and each study was analysed using the two methods of correction. Right ventricular ejection fraction was highest using the right anterior oblique projection with right atrial correction 35.6 +/- 12.5% (mean +/- SD), and lowest when using the anterior posterior projection with planar background correction 26.2 +/- 11% (p less than 0.001). The study design allowed assessment of the effects of correction method and projection independently. Correction method appeared to have relatively little effect on right ventricular ejection fraction. Using right atrial correction correlation coefficient (r) between projections was 0.92, and for planar background correction r = 0.76, both p less than 0.001. However, right ventricular ejection fraction was far more dependent upon projection. When the anterior-posterior projection was used calculated right ventricular ejection fraction was much more dependent on correction method (r = 0.65, p = not significant), than using the right anterior oblique projection (r = 0.85, p less than 0.001)

Beneficial aspects of real time flow measurements for the management of acute right ventricular heart failure following continuous flow ventricular assist device implantation

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Spiliopoulos Sotirios

2012-11-01

Full Text Available Abstract Background Optimal management of acute right heart failure following the implantation of a left ventricular assist device requires a reliable estimation of left ventricular preload and contractility. This is possible by real-time pump blood flow measurements. Clinical case We performed implantation of a continuous flow left ventricular assist device in a 66 years old female patient with an end-stage heart failure on the grounds of a dilated cardiomyopathy. Real-time pump blood flow was directly measured by an ultrasonic flow probe placed around the outflow graft. Diagnosis The progressive decline of real time flow and the loss of pulsatility were associated with an increase of central venous pressure, inotropic therapy and progressive renal failure suggesting the presence of an acute right heart failure. Diagnosis was validated by echocardiography and thermodilution measurements. Treatment Temporary mechanical circulatory support of the right ventricle was successfully performed. Real time flow measurement proved to be a useful tool for the diagnosis and ultimately for the management of right heart failure including the weaning from extracorporeal membrane oxygenation.

Right ventricular longitudinal strain correlates well with right ventricular stroke work index in patients with advanced heart failure referred for heart transplantation.

Science.gov (United States)

Cameli, Matteo; Lisi, Matteo; Righini, Francesca Maria; Tsioulpas, Charilaos; Bernazzali, Sonia; Maccherini, Massimo; Sani, Guido; Ballo, Piercarlo; Galderisi, Maurizio; Mondillo, Sergio

2012-03-01

Right ventricular (RV) systolic function has a critical role in determining the clinical outcome and success of using left ventricular assist devices (LVADs) in patients with refractory heart failure. Tissue Doppler and M-mode measurements of tricuspid systolic motion (tricuspid S' and tricuspid annular plane systolic excursion [TAPSE]) are the most currently used methods for the quantification of RV longitudinal function; RV deformation analysis by speckle-tracking echocardiography (STE) has recently allowed the analysis of global RV longitudinal function. Using cardiac catheterization as the reference standard, this study aimed at exploring the correlation between RV longitudinal function by STE and RV stroke work index (RVSWI) in patients referred for cardiac transplantation. Right-side heart catheterization and transthoracic echo Doppler were simultaneously performed in 41 patients referred for cardiac transplantation evaluation for advanced systolic heart failure. Thermodilution RV stroke volume and invasive pulmonary pressures were used to obtain RVSWI. RV longitudinal strain (RVLS) by STE was assessed averaging all segments in apical 4-chamber view (global RVLS) and by averaging RV free-wall segments (free-wall RVLS). Tricuspid S' and TAPSE were also calculated. No significant correlations were found for TAPSE or tricuspid S' with RVSWI (r = 0.14; r = 0.06; respectively). Close negative correlations between global RVLS and free-wall RVLS with the RVSWI were found (r = -0.75; r = -0.82; respectively; both P rights reserved.

Anti-ischemic effects of inotropic agents in experimental right ventricular infarction.

NARCIS (Netherlands)

Hein, M.; Roehl, A.B.; Baumert, J.H.; Scherer, K.; Steendijk, P.; Rossaint, R.

2009-01-01

BACKGROUND: Right ventricular (RV) function is an important determinant of survival after myocardial infarction. The efficacy of reperfusion therapy might be increased by the cardioprotective action of inotropic agents, which are used for symptomatic therapy in situations with compromised

Tissue-resident Sca1+ PDGFRα+ mesenchymal progenitors are the cellular source of fibrofatty infiltration in arrhythmogenic cardiomyopathy [v1; ref status: indexed, http://f1000r.es/17s

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Ben Paylor

2013-06-01

Full Text Available Arrhythmogenic cardiomyopathy (AC is a disease of the heart involving myocardial dystrophy leading to fibrofatty scarring of the myocardium and is associated with an increased risk of both ventricular arrhythmias and sudden cardiac death. It often affects the right ventricle but may also involve the left. Although there has been significant progress in understanding the role of underlying desmosomal genetic defects in AC, there is still a lack of data regarding the cellular processes involved in its progression. The development of cardiac fibrofatty scarring is known to be a principal pathological process associated with ventricular arrhythmias, and it is vital that we elucidate the role of various cell populations involved in the disease if targeted therapeutics are to be developed. The known role of mesenchymal progenitor cells in the reparative process of both the heart and skeletal muscle has provided inspiration for the identification of the cellular basis of fibrofatty infiltration in AC. Here we hypothesize that reparative processes triggered by myocardial degeneration lead to the differentiation of tissue-resident Sca1+ PDGFRα+ mesenchymal progenitors into adipocytes and fibroblasts, which compose the fibrofatty lesions characteristic of AC.

Recurrent venous thromboembolism in patients with pulmonary embolism and right ventricular dysfunction: a post-hoc analysis of the Hokusai-VTE study

NARCIS (Netherlands)

Brekelmans, Marjolein P. A.; Ageno, Walter; Beenen, Ludo F.; Brenner, Benjamin; Buller, Harry R.; Chen, Cathy Z.; Cohen, Alexander T.; Grosso, Michael A.; Meyer, Guy; Raskob, Gary; Segers, Annelise; Vanassche, Thomas; Verhamme, Peter; Wells, Philip S.; Zhang, George; Weitz, Jeffrey I.

2016-01-01

In patients with pulmonary embolism, right ventricular dysfunction is associated with early mortality. The Hokusai-VTE study used N-terminal pro-brain natriuretic peptide (NT-proBNP) and right to left ventricular diameter ratio on CT as indicators of right ventricular dysfunction and reported that

Serial Doppler echocardiographic assessment of left and right ventricular performance after a first myocardial infarction

DEFF Research Database (Denmark)

Møller, J E; Søndergaard, E; Poulsen, S H

2001-01-01

We sought to investigate the relation between left ventricular (LV) and right ventricular (RV) function assessed with the Doppler-derived myocardial performance index (MPI), to assess serial changes, and to investigate the prognostic value of biventricular assessment of cardiac function after a f...

Mechanisms and Clinical Management of Ventricular Arrhythmias following Blunt Chest Trauma

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Daniel H. Wolbrom

2016-01-01

Full Text Available Nonpenetrating, blunt chest trauma is a serious medical condition with varied clinical presentations and implications. This can be the result of a dense projectile during competitive and recreational sports but may also include other etiologies such as motor vehicle accidents or traumatic falls. In this setting, the manifestation of ventricular arrhythmias has been observed both acutely and chronically. This is based on two entirely separate mechanisms and etiologies requiring different treatments. Ventricular fibrillation can occur immediately after chest wall injury (commotio cordis and requires rapid defibrillation. Monomorphic ventricular tachycardia can develop in the chronic stage due to underlying structural heart disease long after blunt chest injury. The associated arrhythmogenic tissue may be complex and provides the necessary substrate to form a reentrant VT circuit. Ventricular tachycardia in the absence of overt structural heart disease appears to be focal in nature with rapid termination during ablation. Regardless of the VT mechanism, patients with recurrent episodes, despite antiarrhythmic medication in the chronic stage following blunt chest injury, are likely to require ablation to achieve VT control. This review article will describe the mechanisms, pathophysiology, and treatment of ventricular arrhythmias that occur in both the acute and chronic stages following blunt chest trauma.

Truncating Plakophilin-2 Mutations in Arrhythmogenic Cardiomyopathy Are Associated with Protein Haploinsufficiency in Both Myocardium and Epidermis

DEFF Research Database (Denmark)

Rasmussen, Torsten Bloch; Nissen, Peter H; Palmfeldt, Johan

2014-01-01

BACKGROUND: Arrhythmogenic cardiomyopathy (AC) is a hereditary cardiac condition associated with ventricular arrhythmias, heart failure, and sudden death. The disease is most often caused by mutations in the desmosomal gene for plakophilin-2 (PKP2), which is expressed in both myocardial...... and epidermal tissue. This study aimed to investigate protein expression in myocardial tissue of patients with AC carrying PKP2 mutations and elucidate whether keratinocytes of the same individuals exhibited a similar pattern of protein expression. METHODS AND RESULTS: Direct sequencing of 5 AC genes in 71...... unrelated patients with AC identified 10 different PKP2 mutations in 12 index patients. One patient, heterozygous for a PKP2 nonsense mutation, developed severe heart failure and underwent cardiac transplantation. Western blotting and immunohistochemistry of the explanted heart showed a significant decrease...

Three-dimensional Speckle Tracking Echocardiography in Light Chain Cardiac Amyloidosis: Examination of Left and Right Ventricular Myocardial Mechanics Parameters.

Science.gov (United States)

Urbano-Moral, Jose Angel; Gangadharamurthy, Dakshin; Comenzo, Raymond L; Pandian, Natesa G; Patel, Ayan R

2015-08-01

The study of myocardial mechanics has a potential role in the detection of cardiac involvement in patients with amyloidosis. This study aimed to characterize 3-dimensional-speckle tracking echocardiography-derived left and right ventricular myocardial mechanics in light chain amyloidosis and examine their relationship with brain natriuretic peptide. In patients with light chain amyloidosis, left ventricular longitudinal and circumferential strain (n=40), and right ventricular longitudinal strain and radial displacement (n=26) were obtained by 3-dimensional-speckle tracking echocardiography. Brain natriuretic peptide levels were determined. All myocardial mechanics measurements showed differences when compared by brain natriuretic peptide level tertiles. Left and right ventricular longitudinal strain were highly correlated (r=0.95, P<.001). Left ventricular longitudinal and circumferential strain were reduced in patients with cardiac involvement (-9±4 vs -16±2; P<.001, and -24±6 vs -29±4; P=.01, respectively), with the most prominent impairment at the basal segments. Right ventricular longitudinal strain and radial displacement were diminished in patients with cardiac involvement (-9±3 vs -17±3; P<.001, and 2.7±0.8 vs 3.8±0.3; P=.002). On multivariate analysis, left ventricular longitudinal strain was associated with the presence of cardiac involvement (odds ratio = 1.6; 95% confidence interval, 1.04 to 2.37; P=.03) independent of the presence of brain natriuretic peptide and troponin I criteria for cardiac amyloidosis. Three-dimensional-speckle tracking echocardiography-derived left and right ventricular myocardial mechanics are increasingly altered as brain natriuretic peptide increases in light chain amyloidosis. There appears to be a strong association between left ventricular longitudinal strain and cardiac involvement, beyond biomarkers such as brain natriuretic peptide and troponin I. Copyright © 2015 Sociedad Española de Cardiología. Published by

Impact of right ventricular distensibility on congestive heart failure with preserved left ventricular ejection fraction in the elderly.

Science.gov (United States)

Harada, Daisuke; Aasanoi, Hidetsugu; Ushijima, Ryuichi; Noto, Takahisa; Takagawa, Junya; Ishise, Hisanari; Inoue, Hiroshi

2018-06-01

To elucidate involvement of age-related impairments of right ventricular (RV) distensibility in the elderly congestive heart failure (CHF), we examined the prevalence of less-distensible right ventricle in patients with preserved left ventricular ejection fraction (LVEF) over a wide range of ages. In 893 patients aged from 40 to 102 years, we simultaneously recorded electrocardiogram, phonocardiogram, and jugular venous pulse wave. Using signal-processing techniques, the prominent 'Y' descent of jugular pulse waveform was detected as a hemodynamic sign of a less-distensible right ventricle. Prevalence of less-distensible right ventricle and elevated RV systolic pressure increased along with aging from the 50s to the 90s in an exponential fashion from 3.3 and 12% up to 33 and 61%, respectively (p right ventricle (Odds ratio, 1.05 per 1 year, p = 0.003; and 1.03 per 1 mmHg, p = 0.026, respectively). The elderly CHF was associated with high prevalence of the less-distensible right ventricle and higher RV systolic pressure, both of which were independent risk factors for CHF (Odds ratio, 5.27, p = 0.001, and 1.08 per 1 mmHg, p right ventricle and a high RV systolic pressure seems to be related to developing CHF. The less-distensible right ventricle and elevated RV systolic pressure are closely associated with CHF with preserved LVEF in the elderly patients.

Computed tomographic pulmonary angiography in the assessment of severity of chronic thromboembolic pulmonary hypertension and right ventricular dysfunction

International Nuclear Information System (INIS)

Liu Min; Ma Zhanhong; Guo Xiaojuan; Zhang Hongxia; Yang Yuanhua; WangChen

2011-01-01

Purpose: The aim was to investigate the role of computed tomographic pulmonary angiography (CTPA) in the assessment of severity and right ventricular function in chronic thromboembolic pulmonary hypertension (CTEPH). Materials and methods: Clinical and radiological data of 56 patients with CTEPH January 2006–October 2009 were retrospectively reviewed in the present study. All patients received CTPA with a 64-row CT using the retrospective ECG-Gated mode before digital subtraction pulmonary angiography and right-heart catheterization. CTPA findings including Right Ventricular diameter (RVd) and left ventricular diameter (LVd) were measured at the end diastole. CT Pulmonary Artery Obstruction Indexes including Qanadli Index and Mastora Index were used in the assessment of severity of pulmonary arterial obstruction. Hemodynamic parameters and pulmonary hypertension classification were evaluated by right-heart catheterization in all patients. Right ventricular function was measured with echocardiography in 49 patients. Results: Qanadli Index and Mastora Index respectively were (37.93 ± 14.74)% and (30.92 ± 16.91)%, which showed a significant difference (Z = −5.983, P = 0.000) and a good correlation (r = 0.881, P = 0.000). Neither Qanadli nor Mastora Index correlated with pulmonary hypertension classification (r = −0.009, P = 0.920) or New York Heart Association heart function classification (r = −0.031, P = 0.756). Neither Qanadli nor Mastora Index correlated with any echocardiographic right ventricular parameters (P > 0.05), while RVd/LVd by CTPA correlated with echocardiographic right ventricular functional parameters (P 2 . CTPA findings correlated with hemodynamic variables. Backward linear regression analysis revealed that the RVd/LVd, Right Ventricular Anterior Wall Thickness (RVAWT), Main Pulmonary Artery trunk diameter (MPAd) were shown to be independently associated with mean Pulmonary Artery Pressure (mPAP) levels (model: r 2 = 0.351, P = 0.025; RVd

Right ventricular performance and central circulatory hemodynamics during upright exercise in patients with chronic obstructive pulmonary disease

International Nuclear Information System (INIS)

Mahler, D.A.; Brent, B.N.; Loke, J.; Zaret, B.L.; Matthay, R.A.

1984-01-01

A combined hemodynamic and radionuclide approach was used to evaluate right ventricular performance during upright exercise in 12 male patients with chronic obstructive pulmonary disease. To assess the influence of intrathoracic pressure on hemodynamic parameters, pleural pressure was measured using an esophageal balloon. Mean age was 58.5 +/- 6.7 yr (+/- SD), and all had dyspnea on physical exertion. For the group, forced expiratory volume in one second (FEV1) was 1.04 +/- 0.40 L and arterial oxygen-tension (PaO 2 ) was 77 +/- 11 mmHg. During steady-state, upright exercise on the bicycle ergometer at 58% of maximal oxygen consumption (VO 2 max): (1) mean pulmonary artery pressure (Ppa) and pulmonary vascular resistance index (PVRI) increased significantly; (2) right ventricular ejection fraction (RVEF) failed to augment appropriately (less than 5% increase); and (3) right ventricular end-diastolic volume index (RVEDVI) increased significantly, whereas right ventricular end-systolic volume index (RVESVI) did not change. A diminished pulmonary vascular bed, the change in PaO 2 , and possibly increased alveolar pressure appeared to contribute to the increased load placed on the right ventricle. Both RVEDVI and RVESVI were significantly correlated with Ppa at rest and during exercise. In 2 of the 12 patients, stroke volume index and left ventricular end-diastolic volume index showed minimal change with exercise. VO 2 max was correlated with the FEV1 (r . 0.75; p . 0.01) as well as resting (r . -0.60; p . 0.02) and exercise (r . -0.61; p . 0.02) PVRI. These results suggest that exercise performance may be limited by right ventricular dysfunction in addition to respiratory impairment in some patients with chronic airway disease

[Experimental studies on the diffusion of excitation on the right ventricular surface in the dog, during normal and stimulated beats].

Science.gov (United States)

Arisi, G; Macchi, E; Baruffi, S; Musso, E; Spaggiari, S; Stilli, D; Taccardi, B

1982-01-01

Previous work on the spread of excitation on the dog's ventricular surface enabled us to locate up to 30 breakthrough points (BKTPs) where excitation reaches the ventricular surface. In particular the equipotential contour maps enabled us to detect 3 to 5 BKTPs on the anterior right ventricular surface, near the a-v groove when a large part of ventricular surface was still at rest. With a view to investigating the mechanism underlying the early excitation of these basal regions, we stimulated the heart at several right ventricular BKTPs and in other points located at a distance from the BKTPs. The instantaneous equipotential maps showed that after stimulation most right ventricular BKTPs remained in the same position as observed the normal beats. The early appearance of epicardial wavefronts in the basal region and generally in other areas of the right ventricle was attributed to the rapid propagation of excitation waves through the Purkinje network, probably associated to a short transmural crossing time, due to a local thinness of the ventricular wall.

Assessment of right ventricular function with nonimaging first pass ventriculography and comparison of results with gamma camera studies.

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Zhang, Z; Liu, X J; Liu, Y Z; Lu, P; Crawley, J C; Lahiri, A

1990-08-01

A new technique has been developed for measuring right ventricular function by nonimaging first pass ventriculography. The right ventricular ejection fraction (RVEF) obtained by non-imaging first pass ventriculography was compared with that obtained by gamma camera first pass and equilibrium ventriculography. The data has demonstrated that the correlation of RVEFs obtained by the nonimaging nuclear cardiac probe and by gamma camera first pass ventriculography in 15 subjects was comparable (r = 0.93). There was also a good correlation between RVEFs obtained by the nonimaging nuclear probe and by equilibrium gated blood pool studies in 33 subjects (r = 0.89). RVEF was significantly reduced in 15 patients with right ventricular and/or inferior myocardial infarction compared to normal subjects (28 +/- 9% v. 45 +/- 9%). The data suggests that nonimaging probes may be used for assessing right ventricular function accurately.

Possible involvement of mitochondrial energy-producing ability in the development of right ventricular failure in monocrotaline-induced pulmonary hypertensive rats.

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Daicho, Takuya; Yagi, Tatsuya; Abe, Yohei; Ohara, Meiko; Marunouchi, Tetsuro; Takeo, Satoshi; Tanonaka, Kouichi

2009-09-01

The present study was undertaken to explore the possible involvement of alterations in the mitochondrial energy-producing ability in the development of the right ventricular failure in monocrotaline-administered rats. The rats at the 6th week after subcutaneous injection of 60 mg/kg monocrotaline revealed marked myocardial hypertrophy and fibrosis, that is, severe cardiac remodeling. The time-course study on the cardiac hemodynamics of the monocrotaline-administered rat by the cannula and echocardiographic methods showed a reduction in cardiac double product, a decrease in cardiac output index, and an increase in the right ventricular Tei index, suggesting that the right ventricular failure was induced at the 6th week after monocrotaline administration in rats. The mitochondrial oxygen consumption rate of the right ventricular muscle isolated from the monocrotaline-administered animal was decreased, which was associated with a reduction in myocardial high-energy phosphates. Furthermore, the decrease in mitochondrial oxygen consumption rate was inversely related to the increase in the right ventricular Tei index of the monocrotaline-administered rats. These results suggest that impairment of the mitochondrial energy-producing ability is involved in the development of the right ventricular failure in monocrotaline-induced pulmonary hypertensive rats.

Radionuclide determination of right and left ventricular stroke volumes

Energy Technology Data Exchange (ETDEWEB)

Shen, Wei Feng; Roubin, G S; Choong, C Y.P.; Harris, P J; Flether, P J; Kelly, D T; Uren, R F; Hutton, B F

1985-03-01

The relationship between radionuclide and thermodilution measurement of stroke volumes (SV) was investigated in 30 patients without valvular regurgitation or intracardiac shunt (group A) at rest and during exercise. Both attenuated radionuclide right ventricular (RV) and left ventricular (LV) SV measurements correlated well with the SV determined by the thermodilution method (r = 0.87 and r = 0.93, all P < 0.001). The reliability of the radionuclide method to estimate SV was evaluated prospectively in two additional groups of patients. In 11 patients without valvular regurgitation or intracardiac shunt (group B) the radionuclide RVSV and LVSV closely approximated to thermodilution SV at rest and during exercise. In 15 patients with aortic regurgitation (group C) the radionuclide stroke volume ratio correlated well with the angiographic regurgitant fraction. Thus, both RVSV and LVSV and the severity of aortic regurgitation can be reliably measured with gated radionuclide ventriculography.

Thallium-201 myocardial imaging for evaluation of right-ventricular overloading

International Nuclear Information System (INIS)

Kondo, M.; Kubo, A.; Yamazaki, H.; Ohsuzu, F.; Handa, S.; Tsugu, T.; Masaki, H.; Kinoshita, F.; Hashimoto, S.

1978-01-01

This study evaluated the specificity and sensitivity of Tl-201 myocardial imaging in the detection of right-ventricular (RV) overloading. Right-ventricular visualization (RVV) after administration of Tl-201 chloride was studied on 99 patients with various heart diseases. Tracer uptake in the free wall of the RV was graded in four degrees. The degree of RVV was compared with the findings of cardiac catheterization. The comparisons indicated that the uptake increased in step with the inreases in RV systolic pressure, RV end-diastolic pressure, mean pulmonary arterial pressure, total pulmonary vascular resistance, and stroke-work index of the right ventricle (P < 0.05--P < 0.001). Of the patients with visible RV, all but three had RV overloading, and all but three of those without RVV had normal RV systolic pressure. Myocardial images also reflect the type of RV overloading. In patients with RV pressure overloading, the septum showed a tendency to appear straight. In patients with atrial septal defect leading to RV volume overloading, the RV cavity was dilated, the LV image small, and the septum convex toward the RV cavity. These results indicate that Tl-201 myocardial imaging is a sensitive and specific method for the study of RV overloading

Effects of Percutaneous LVAD Support on Right Ventricular Load and Adaptation.

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Yourshaw, Jeffrey P; Mishra, Prabodh; Armstrong, M Christopher; Ramu, Bhavadharini; Craig, Michael L; Van Bakel, Adrian B; Steinberg, Daniel H; DiSalvo, Thomas G; Tedford, Ryan J; Houston, Brian A

2018-04-30

Both operative and hemodynamic mechanisms have been implicated in right heart failure (RHF) following surgical left ventricular assist device (LVAD) implantation. We investigated the effects of percutaneous LVAD (pLVAD; Impella®, Abiomed) support on right ventricular (RV) load and adaptation. We reviewed all patients receiving a pLVAD for cardiogenic shock at our institution between July 2014 and April 2017, including only those with pre- and post-pLVAD invasive hemodynamic measurements. Hemodynamic data was recorded immediately prior to pLVAD implantation and up to 96 h post-implantation. Twenty-five patients were included. Cardiac output increased progressively during pLVAD support. PAWP improved early post-pLVAD but did not further improve during continued support. Markers of RV adaptation (right ventricular stroke work index, right atrial pressure (RAP), and RAP to pulmonary artery wedge pressure ratio (RAP:PAWP)) were unchanged acutely implant but progressively improved during continued pLVAD support. Total RV load (pulmonary effective arterial elastance; E A ) and resistive RV load (pulmonary vascular resistance; PVR) both declined progressively. The relationship between RV load and RV adaptation (E A /RAP and E A /RAP:PAWP) was constant throughout. Median vasoactive-inotrope score declined after pLVAD placement and continued to decline throughout support. Percutaneous LVAD support in patients with cardiogenic shock did not acutely worsen RV adaptation, in contrast to previously described hemodynamic effects of surgically implanted durable LVADs. Further, RV load progressively declined during support, and the noted RV adaptation improvement was load-dependent as depicted by constant E A /RA and E A /RAP:PAWP relationships. These findings further implicate the operative changes associated with surgical LVAD implantation in early RHF following durable LVAD.

Right ventricular oxygen supply/demand balance in exercising dogs.

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Hart, B J; Bian, X; Gwirtz, P A; Setty, S; Downey, H F

2001-08-01

This is the first investigation of right ventricular (RV) myocardial oxygen supply/demand balance in a conscious animal. A novel technique developed in our laboratory was used to collect right coronary (RC) venous blood samples from seven instrumented, conscious dogs at rest and during graded treadmill exercise. Contributions of the RV oxygen extraction reserve and the RC flow reserve to exercise-induced increases in RV oxygen demand were measured. Strenuous exercise caused a 269% increase in RV oxygen consumption. Expanded arteriovenous oxygen content difference (A-V(Delta)O2) provided 58% of this increase in oxygen demand, and increased RC blood flow (RCBF) provided 42%. At less strenuous exercise, expanded A-V(Delta)O2 provided 60-80% of the required oxygen, and increases in RCBF were small and driven by increased aortic pressure. RC resistance fell only at strenuous exercise after the extraction reserve had been mobilized. Thus RC resistance was unaffected by large decreases in RC venous PO2 until an apparent threshold at 20 mmHg was reached. Comparisons of RV findings with published left ventricular data from exercising dogs demonstrated that increased O2 demand of the left ventricle is met primarily by increasing coronary flow, whereas increased O2 extraction makes a greater contribution to RV O2 supply.

Total Artificial Heart Implantation after Excision of Right Ventricular Angiosarcoma.

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Bruckner, Brian A; Abu Saleh, Walid K; Al Jabbari, Odeaa; Copeland, Jack G; Estep, Jerry D; Loebe, Matthias; Reardon, Michael J

2016-06-01

Primary cardiac sarcomas, although rare, are aggressive and lethal, requiring thorough surgical resection and adjuvant chemotherapy for the best possible outcome. We report the case of a 32-year-old woman who underwent total artificial heart implantation for right-sided heart failure caused by right ventricular angiosarcoma. For the first several weeks in intensive care, the patient recovered uneventfully. However, a postoperative liver biopsy indicated hepatocellular injury consistent with preoperative chemotherapy. She developed continuing liver failure, from which she died despite good cardiac function.

Cardiac rhythm disturbance in athletes with cardiac connective tissue dysplasia syndrome

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Shahrjerdi Sh

2007-06-01

Full Text Available Background: Cardiac connective tissue dysplasia syndrome consists of mitral valve prolapse (MVP, anomalously located chordae tendinae of the left ventricle, or a combination of the two. MVP is marked by the displacement of an abnormally thickened mitral valve leaflet into the left atrium during systole. The nonclassic form of MVP carries a low risk of complications. Patients with severe classic MVP can suffer from mitral regurgitation (MR, infective endocarditis, and, infrequently, sudden death from cardiac arrest. Anomalously located left ventricular chordae tendinae are fibrous or fibromuscular bands that stretch across the left ventricle from the septum to the free wall. They have been associated with murmurs and arrhythmias. The purpose of this study is to assess the performance, as measured by the physical working capacity (PWC170 and maximal oxygen consumption (VO2 max, in athletes with cardiac connective tissue dysplasia syndrome. Methods: Of the 183 male athletes studied, 158 had cardiac connective tissue dysplasia syndrome and 25 were normal, healthy controls. Their mean age was 16.23 (± 5.48 years and mean training time was 5.2 (±- 4.6 years. Athletes with cardiac connective tissue dysplasia syndrome were divided to four groups. Group 1 consisted of those with MVP; Group 2 had patients with an additional cord in left ventricle; Group 3 was made up of athletes with a combination of MVP and additional cord; Group 4 contained athletes with a combination of MVP and MR. All sportsmen were studied by echocardiograph, veloergometer, and those with arrhythmias were studied and recorded using a Holter monitor. Results: The most common form of this syndrome in our study groups was MVP. The PWC170and VO2 max among the athletes with the combination of MVP+MR (Group 4 was lower than that of athletes in other groups (P<0.05. The most common arrhythmia among the athletes with anomalously located left ventricular chordae, Group 2, was Wolf

Lower Lid Ectropion in Hypohidrotic Ectodermal Dysplasia

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Xiaoyun Zhang

2015-01-01

Full Text Available We report a case of a lower lid ectropion with ectodermal dysplasia and ectropion blepharoplasty surgery experience. A 14-year-old Han nationality male patient with typical characteristics of hypohidrotic ectodermal dysplasia presented to our clinic for his right lower lid eversion. The patient was diagnosed as having hypohidrotic ectodermal dysplasia and underwent an uneventful blepharoplasty surgery. The lower lid maintained normal position during the 10-month follow-up period. Patients with ectodermal dysplasia could firstly visit ophthalmologist for their ectropion and blepharoplasty surgery could be useful for the disease.

Determination of left and right ventricular ejection and filling by fast cine MR imaging in the breath-hold technique in healthy subjects

International Nuclear Information System (INIS)

Rominger, M.B.; Bachmann, G.F.; Geuer, M.; Puzik, M.; Rau, W.S.; Pabst, W.

1999-01-01

Purpose: Evaluation and comparison of localized and global left and right ventricular ejection and filling with fast cine MR imaging in the breath-hold technique. Materials and Methods: 10 healthy volunteers were examined with a 1.5 Tesla unit and phased-array-coil using a segmented FLASH-2D sequence in breath-hold technique. Peak ejection and peak filling rates [PER, PFR enddiastolic volume (EDV)/s], time to PER and PFR [TPER, TPFR ms] and time of endsystole [TSYS in % RR-intervall] of all slices (complete-slice-evaluation) were evaluated and compared to three left ventricular and one right ventricular slices (reduced three-slice-evaluation). Results: There were significant regional left ventricular differences of PER (p=0.002) and PFR (p=0.007), but not of TPER and TPFR. Ejection and filling indices of the left ventricular middle slice were closest to the overall evaluation of all sections. In the left-/right-side comparison the right ventricular PFR was higher than the left ventricular (5.1 and 4.2 EDV/s) and the right ventricular TPFR was earlier than the left (92.2 and 123.5 ms). Conclusions: With fast cine techniques, regional and global left and right ventricular ejection and filling indices can be evaluated in addition to the global heart volume indices. The three-slice-evaluation represents a comprehensive, clear and time-saving method for daily routine. (orig.) [de

Interdependence of right ventricular systolic function and left ventricular filling and its association with outcome for patients with pulmonary hypertension.

Science.gov (United States)

Motoji, Yoshiki; Tanaka, Hidekazu; Fukuda, Yuko; Sano, Hiroyuki; Ryo, Keiko; Imanishi, Junichi; Miyoshi, Tatsuya; Sawa, Takuma; Mochizuki, Yasuhide; Matsumoto, Kensuke; Emoto, Noriaki; Hirata, Ken-ichi

2015-04-01

Although impaired right ventricular (RV) performance has been associated with adverse outcomes for pulmonary hypertension (PH) patients, the relationship between bi-ventricular interdependence and outcomes is not yet fully understood. We studied 96 PH patients. RV systolic function was assessed by means of RV free-wall longitudinal speckle-tracking strain (RV-free), and left ventricular (LV) filling as early diastolic transmitral flow velocity (TMF-E). RV-free ≤19 % and TMF-E functional class IV and brain natriuretic peptide >150 pg/dl (χ(2) = 1.2) was improved by the addition of RV-free (χ(2) = 5.5, p = 0.04) as well as of TMF-E (χ(2) = 11.5, p = 0.01). In conclusions, RV systolic function was shown to correlate significantly with LV filling in PH patients. In addition, not only assessment of RV systolic function, but also of a combined bi-ventricular parameter comprising RV systolic function and LV filling may well have clinical implications for more successful management of PH patients.

Right ventricular systolic function in hypertensive heart failure

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Oketona OA

2017-09-01

Full Text Available OA Oketona,1 MO Balogun,2 AO Akintomide,2 OE Ajayi,2 RA Adebayo,2 TO Mene-Afejuku,3 OT Oketona,1 OJ Bamikole2 1Fort Nelson General Hospital, Fort Nelson, BC, Canada; 2Cardiology Unit, Department of Medicine, Obafemi Awolowo University Teaching Hospitals Complex, Ife, Osun State, Nigeria; 3Department of Medicine, Metropolitan Hospital Center, New York, NY, USA Background: Heart failure (HF is a major cause of cardiovascular admissions and hypertensive heart failure (HHF is the most common cause of HF admissions in sub-Saharan Africa, Nigeria inclusive. Right ventricular (RV dysfunction is being increasingly recognized in HF and found to be an independent predictor of adverse outcomes in HF. This study aimed to determine the prevalence of RV systolic dysfunction in HHF by several echocardiographic parameters.Methodology: One hundred subjects with HHF were recruited consecutively into the study along with 50 age and sex-matched controls. All study participants gave written informed consent, and had a full physical examination, blood investigations, 12-lead electrocardiogram, and transthoracic echocardiography. RV systolic function was assessed in all subjects using different methods based on the American Society of Echocardiography guidelines for echocardiographic assessment of the right heart in adults. This included tricuspid annular plane systolic excursion (TAPSE, RV myocardial performance index (MPI, and RV systolic excursion velocity by tissue Doppler (S′.Results: RV systolic dysfunction was found in 53% of subjects with HHF by TAPSE, 56% by RV MPI, and 48% by tissue Doppler systolic excursion S′. RV systolic dysfunction increased with reducing left ventricular ejection fraction (LVEF in subjects with HHF.Conclusion: A high proportion of subjects with HHF were found to have RV systolic functional abnormalities using TAPSE, RV MPI, and RV S′. Prevalence of RV systolic dysfunction increased with reducing LVEF. Keywords: right ventricle

ANP, BNP and D-dimer predict right ventricular dysfunction in patients with acute pulmonary embolism

DEFF Research Database (Denmark)

Borgwardt, Henrik Gutte; Mortensen, Jann; Jensen, Claus V

2010-01-01

The aim of this study was to predict right ventricular dysfunction (RVD) using plasma concentration of D-dimer, pro-atrial natriuretic peptide (pro-ANP), brain natriuretic peptide (BNP), endothelin-1 (ET-1) and cardiac troponin I (TNI) in patients with pulmonary embolism (PE).......The aim of this study was to predict right ventricular dysfunction (RVD) using plasma concentration of D-dimer, pro-atrial natriuretic peptide (pro-ANP), brain natriuretic peptide (BNP), endothelin-1 (ET-1) and cardiac troponin I (TNI) in patients with pulmonary embolism (PE)....

RV Fractional Area Change and TAPSE as Predictors of Severe Right Ventricular Dysfunction in Pulmonary Hypertension: A CMR Study.

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Hoette, Susana; Creuzé, Nicolas; Günther, Sven; Montani, David; Savale, Laurent; Jaïs, Xavier; Parent, Florence; Sitbon, Olivier; Rochitte, Carlos Eduardo; Simonneau, Gerald; Humbert, Marc; Souza, Rogerio; Chemla, Denis

2018-04-01

The right ventricular ejection fraction (RVEF) is a surrogate marker of right ventricular function in pulmonary hypertension (PH), but its measurement is complicated and time consuming. The tricuspid annular plane systolic excursion (TAPSE) measures only the longitudinal component of RV contraction while the right ventricular fractional area change (RVFAC) takes into account both the longitudinal and the transversal components. The aim of our study was to evaluate the relationship between RVEF, RVFAC, and TAPSE according to hemodynamic severity in two groups of patients with PH: pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). Fifty-four patients with PAH (n = 15) and CTEPH (n = 39) underwent right heart catheterization and cardiac magnetic resonance (CMR). The ventricular volumes and areas, TAPSE, and eccentricity index were measured. The RVFAC was more strongly correlated with the RVEF (r = 0.81, p good index to estimate RVEF in PH patients; even better than the TAPSE in patients with more severe hemodynamic profile, possibly for including the transversal component of right ventricular function in its measurement. Furthermore, RVFAC performance was similar in the two PH groups (PAH and CTEPH).

Right heart function in impaired left ventricular diastolic function: 2D speckle tracking echocardiography-based and Doppler tissue imaging-based analysis of right atrial and ventricular function.

Science.gov (United States)

Brand, Anna; Bathe, Marny; Oertelt-Prigione, Sabine; Seeland, Ute; Rücke, Mirjam; Regitz-Zagrosek, Vera; Stangl, Karl; Knebel, Fabian; Stangl, Verena; Dreger, Henryk

2018-01-01

The aim of our study was to describe right atrial (RA) and right ventricular (RV) function, assessed by Doppler tissue imaging and 2D speckle tracking echocardiography (2DSTE), in women with signs of early impaired left ventricular diastolic function (DD). In a cross-sectional trial, standard parameters of diastolic and right heart function were investigated in 438 women of the Berlin Female Risk Evaluation (BEFRI) study. In a subset of women, average peak systolic RA strain (RAS), as well as the average peak systolic RV strain of the free wall (RVS free wall) and of all RV segments (average RV strain; RVS Avg), was analyzed using 2DSTE. Compared to women with normal diastolic function (DD0), RAS, RVS free wall and RVS Avg were significantly reduced in DD (43.1% ± 11.9%, -26.7% ± 5.6%, and -23.3% ± 3.5% in DD0; vs 35.1% ± 10.4%, -23.9% ± 5.5%, and -20.6% ± 3.8% in DD; P right heart function is significantly altered in DD. DTI as well as 2DSTE constitute sensitive echocardiographic tools that enable the diagnosis of impaired right heart mechanics in early-staged DD. © 2017 Wiley Periodicals, Inc.

Development of left ventricular longitudinal speckle tracking echocardiography in very low birth weight infants with and without bronchopulmonary dysplasia during the neonatal period.

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Christoph Czernik

Full Text Available OBJECTIVES: In preterm infants, postnatal myocardial adaptation may be complicated by bronchopulmonary dysplasia (BPD. We aimed to describe the development of left ventricular function by serial 2D, Doppler, and speckle tracking echocardiography (2D-STE in infants with and without BPD during the neonatal period and compare these to anthropometric and conventional hemodynamic parameters. STUDY DESIGN: Prospective echocardiography on day of life (DOL 1, 7, 14, and 28 in 119 preterm infants 10% were seen for the apical segment. While anthropometric parameters show rapid development during the first 4 weeks of life, the speckle tracking parameters did not differ statistically significantly during the neonatal period. Infants with and without BPD differed significantly (p<0.001 in the development of anthropometric parameters, conventional hemodynamic parameters except for heart rate, and 2D-STE parameters: global longitudinal systolic strain rate (GLSSR and longitudinal systolic strain for the mid left wall (LSSR. The largest differences were seen at DOL 1 and 7 in GLSSR (p<0.001 and in LSSR (p<0.01. CONCLUSIONS: Reproducible 2D-STE measurements are possible in preterm infants <1500 g. Cardiac deformation reveals early (DOL 1 and 7 ventricular changes (GLSSR and LSSR in very low birth weight infants who develop BPD.

Spondylo-megaepiphyseal-metaphyseal dysplasia: an unusual bone dysplasia

International Nuclear Information System (INIS)

Agarwal, Prachi Pragya; Srinivasan, Ashok; Sharma, Raju; Gupta, Arun Kumar; Kabra, Madhulika

2003-01-01

A rare case of spondylo-megaepiphyseal-metaphyseal dysplasia is reported in a 10-year-old boy. The features were metaphyseal dysplasia, markedly defective ossification of vertebral body centres and enlarged epiphyses. Although it shares some features with spondylo-metaphyseal dysplasia, oto-spondylo-megaepiphyseal dysplasia and cleidocranial dysplasia, the presence of several unusual radiological findings sets it apart. (orig.)

Echocardiographic assessment with right ventricular function improvement following ultrasound-accelerated catheter-directed thrombolytic therapy in submassive pulmonary embolism.

Science.gov (United States)

Doheny, Charles; Gonzalez, Lorena; Duchman, Stanley M; Varon, Joseph; Bechara, Carlos F; Cheung, Mathew; Lin, Peter H

2018-06-01

Introduction The objective of this study was to evaluate the efficacy of ultrasound-accelerated catheter-directed thrombolytic therapy in patients with submassive pulmonary embolism. Methods Clinical records of 46 patients with submassive pulmonary embolism who underwent ultrasound-accelerated catheter-directed pulmonary thrombolysis using tissue plasminogen activator, from 2007 to 2017, were analyzed. All patients experienced clinical symptoms with computed tomography evidence of pulmonary thrombus burden. Right ventricular dysfunction was present in all patients by echocardiographic finding of right ventricle-to-left ventricle ratio > 0.9. Treatment outcome, procedural complications, right ventricular pressures, and thrombus clearance were evaluated. Follow-up evaluation included echocardiographic assessment of right ventricle-to-left ventricle ratio at one month, six months, and one year. Results Technical success was achieved in all patients ( n = 46, 100%). Our patients received an average of 18.4 ± 4.7 mg of tissue plasminogen activator using ultrasound-accelerated thrombolytic catheter with an average infusion time of 16.5± 5.4 h. Clinical success was achieved in all patients (100%). Significant reduction of mean pulmonary artery pressure occurred following the treatment, which decreased from 36 ± 8 to 21 ± 5 mmHg ( p right ventricular dysfunction based on echocardiographic assessment. The right ventricle-to-left ventricle ratio decreased from 1.32 ± 0.18 to 0.91 ± 0.13 at the time of hospital discharge ( p right ventricular function remained improved at 6 months and 12 months of follow-up, as right ventricle-to-left ventricle ratio were 0.92 ± 0.14 ( p right ventricular function in patients with submassive pulmonary embolism.

Disease Model Discovery from 3,328 Gene Knockouts by The International Mouse Phenotyping Consortium

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Meehan, Terrence F.; Conte, Nathalie; West, David B.; Jacobsen, Julius O.; Mason, Jeremy; Warren, Jonathan; Chen, Chao-Kung; Tudose, Ilinca; Relac, Mike; Matthews, Peter; Karp, Natasha; Santos, Luis; Fiegel, Tanja; Ring, Natalie; Westerberg, Henrik; Greenaway, Simon; Sneddon, Duncan; Morgan, Hugh; Codner, Gemma F; Stewart, Michelle E; Brown, James; Horner, Neil; Haendel, Melissa; Washington, Nicole; Mungall, Christopher J.; Reynolds, Corey L; Gallegos, Juan; Gailus-Durner, Valerie; Sorg, Tania; Pavlovic, Guillaume; Bower, Lynette R; Moore, Mark; Morse, Iva; Gao, Xiang; Tocchini-Valentini, Glauco P; Obata, Yuichi; Cho, Soo Young; Seong, Je Kyung; Seavitt, John; Beaudet, Arthur L.; Dickinson, Mary E.; Herault, Yann; Wurst, Wolfgang; de Angelis, Martin Hrabe; Lloyd, K.C. Kent; Flenniken, Ann M; Nutter, Lauryl MJ; Newbigging, Susan; McKerlie, Colin; Justice, Monica J.; Murray, Stephen A.; Svenson, Karen L.; Braun, Robert E.; White, Jacqueline K.; Bradley, Allan; Flicek, Paul; Wells, Sara; Skarnes, William C.; Adams, David J.; Parkinson, Helen; Mallon, Ann-Marie; Brown, Steve D.M.; Smedley, Damian

2017-01-01

Although next generation sequencing has revolutionised the ability to associate variants with human diseases, diagnostic rates and development of new therapies are still limited by our lack of knowledge of function and pathobiological mechanism for most genes. To address this challenge, the International Mouse Phenotyping Consortium (IMPC) is creating a genome- and phenome-wide catalogue of gene function by characterizing new knockout mouse strains across diverse biological systems through a broad set of standardised phenotyping tests, with all mice made readily available to the biomedical community. Analysing the first 3328 genes reveals models for 360 diseases including the first for type C Bernard-Soulier, Bardet-Biedl-5 and Gordon Holmes syndromes. 90% of our phenotype annotations are novel, providing the first functional evidence for 1092 genes and candidates in unsolved diseases such as Arrhythmogenic Right Ventricular Dysplasia 3. Finally, we describe our role in variant functional validation with the 100,000 Genomes and other projects. PMID:28650483

Cardiac damage in athlete's heart: When the "supernormal" heart fails!

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Carbone, Andreina; D'Andrea, Antonello; Riegler, Lucia; Scarafile, Raffaella; Pezzullo, Enrica; Martone, Francesca; America, Raffaella; Liccardo, Biagio; Galderisi, Maurizio; Bossone, Eduardo; Calabrò, Raffaele

2017-06-26

Intense exercise may cause heart remodeling to compensate increases in blood pressure or volume by increasing muscle mass. Cardiac changes do not involve only the left ventricle, but all heart chambers. Physiological cardiac modeling in athletes is associated with normal or enhanced cardiac function, but recent studies have documented decrements in left ventricular function during intense exercise and the release of cardiac markers of necrosis in athlete's blood of uncertain significance. Furthermore, cardiac remodeling may predispose athletes to heart disease and result in electrical remodeling, responsible for arrhythmias. Athlete's heart is a physiological condition and does not require a specific treatment. In some conditions, it is important to differentiate the physiological adaptations from pathological conditions, such as hypertrophic cardiomyopathy, arrhythmogenic dysplasia of the right ventricle, and non-compaction myocardium, for the greater risk of sudden cardiac death of these conditions. Moreover, some drugs and performance-enhancing drugs can cause structural alterations and arrhythmias, therefore, their use should be excluded.

Automated determination of the right ventricular ejection fraction by digital processing of sup(81m)Kr scintigrams

International Nuclear Information System (INIS)

Elfner, R.; Vaknine, R.; Knapp, W.H.; Tillmanns, H.; Lorenz, W.J.

1986-01-01

A method is presented for the automated determination of the right ventricular ejection fraction (RVEF) by digital image processing of scintigrams obtained by intravenous infusion of Krypton 81m (sup(81m)Kr) dissolved in a glucose solution. End-diastolic and end-systolic sum pictures were computed by the addition of approximately 30-40 frames selected from the time-activity curve of a preliminary, manually drawn, right ventricular region of interest. After processing these two images with an adaptive Wiener filter, the right ventricular contour was determined by a recently developed algorithm using morphological and functional criteria. The RVEF was calculated for a series of 51 patients from the counts in the detected right ventricular regions in the end-diastolic and end-systolic sum images. In 16 patients without evidence of cardiopulmonary disease, the mean RVEF was 50+-6.1%. RVEF was significantly reduced in 18 patients with obstructive pulmonary disease (42+-6.5%) and in 17 patients with congestive cardiomyopathy (36+-7.1%). The correlation coefficient between two determinations of the RVEF was r=0.94. Through digital image processing, the determination of the RVEF by radioventriculography with sup(81m)Kr showed high reliability and reproducibility. (orig.)

Right Ventricular Involvement in either Anterior or Inferior Myocardial Infarction

Directory of Open Access Journals (Sweden)

Firoozeh Abtahi

2016-06-01

Full Text Available Background: Unlike left ventricular function, less attention has been paid to Right Ventricular (RV function after Myocardial Infarction (MI. Objectives: The current study aimed to compare RV function in patients with inferior and anterior MI. Patients and Methods: During the study period, 60 patients consecutively presented to the Emergency Department with chest pain were divided into two groups based on their electrocardiographic findings. Accordingly, 25 patients had inferior MI (IMI group and 35 ones had anterior MI (AMI group. Echocardiography was performed 48 hours after starting the standard therapy. Conventional echocardiographic parameters and Tissue Doppler Imaging (TDI measurements were acquired from the standard views. Student t-test and the chi-square test were respectively used for comparisons of the normally distributed continuous and categorical variables in the two groups. Besides, P < 0.05 was considered to be statistically significant.

Rest and exercise ventricular function in adults with congenital ventricular septal defects

International Nuclear Information System (INIS)

Jablonsky, G.; Hilton, J.D.; Liu, P.P.; Morch, J.E.; Druck, M.N.; Bar-Shlomo, B.Z.; McLaughlin, P.R.

1983-01-01

Rest and exercise right and left ventricular function were compared using equilibrium gated radionuclide angiography in 19 normal sedentary control subjects and 34 patients with hemodynamically documented congenital ventricular septal defect (VSD). Gated radionuclide angiography was performed at rest and during each level of graded supine bicycle exercise to fatigue. Heart rate, blood pressure, maximal work load achieved, and right and left ventricular ejection fractions were assessed. The control subjects demonstrated an increase in both the left and right ventricular ejection fractions with exercise. All study groups failed to demonstrate an increase in ejection fraction in either ventricle with exercise. Furthermore, resting left ventricular ejection fraction in Groups 2 and 3 was lower than that in the control subjects and resting right ventricular ejection fraction was lower in Group 3 versus control subjects. Thus left and right ventricular function on exercise were abnormal in patients with residual VSD as compared with control subjects; rest and exercise left ventricular ejection fractions remained abnormal despite surgical closure of VSD in the remote past; resting left and right ventricular function was abnormal in patients with Eisenmenger's complex; lifelong volume overload may be detrimental to myocardial function

Direct right ventricular puncture for hemodynamic evaluation of a mechanical tricuspid valve prosthesis: a new indication for an old procedure.

Science.gov (United States)

Gibson, M A; Carell, E S

1997-11-01

The advent of transvenous right heart catheterization has relegated direct transthoracic right ventricular puncture largely to the role of "interesting historical footnote." However, in the case of a right ventricle that is "protected" by a mechanical tricuspid valve prosthesis, direct right ventricular puncture represents a reasonable alternative for obtaining accurate hemodynamic information.

Importance of Non-invasive Right and Left Ventricular Variables on Exercise Capacity in Patients with Tetralogy of Fallot Hemodynamics.

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Meierhofer, Christian; Tavakkoli, Timon; Kühn, Andreas; Ulm, Kurt; Hager, Alfred; Müller, Jan; Martinoff, Stefan; Ewert, Peter; Stern, Heiko

2017-12-01

Good quality of life correlates with a good exercise capacity in daily life in patients with tetralogy of Fallot (ToF). Patients after correction of ToF usually develop residual defects such as pulmonary regurgitation or stenosis of variable severity. However, the importance of different hemodynamic parameters and their impact on exercise capacity is unclear. We investigated several hemodynamic parameters measured by cardiovascular magnetic resonance (CMR) and echocardiography and evaluated which parameter has the most pronounced effect on maximal exercise capacity determined by cardiopulmonary exercise testing (CPET). 132 patients with ToF-like hemodynamics were tested during routine follow-up with CMR, echocardiography and CPET. Right and left ventricular volume data, ventricular ejection fraction and pulmonary regurgitation were evaluated by CMR. Echocardiographic pressure gradients in the right ventricular outflow tract and through the tricuspid valve were measured. All data were classified and correlated with the results of CPET evaluations of these patients. The analysis was performed using the Random Forest model. In this way, we calculated the importance of the different hemodynamic variables related to the maximal oxygen uptake in CPET (VO 2 %predicted). Right ventricular pressure showed the most important influence on maximal oxygen uptake, whereas pulmonary regurgitation and right ventricular enddiastolic volume were not important hemodynamic variables to predict maximal oxygen uptake in CPET. Maximal exercise capacity was only very weakly influenced by right ventricular enddiastolic volume and not at all by pulmonary regurgitation in patients with ToF. The variable with the most pronounced influence was the right ventricular pressure.

Quantitative Evaluation of the Fetal Right and Left Ventricular Fractional Area Change Using Speckle Tracking Technology.

Science.gov (United States)

DeVore, Greggory R; Klas, Berthold; Satou, Gary; Sklansky, Mark

2018-03-14

The purpose of this study was to measure the fractional area change (FAC) of the right and left ventricles in normal fetal hearts between 20 and 40 weeks of gestation using speckle-tracking software. The 4-chamber view of the fetal heart was obtained in 200 control fetuses between 20 and 40 weeks of gestation. The FAC was computed from the ventricular areas [((end-diastolic area) - (end-systolic area)/(end-diastolic area)) x 100] for the right and left ventricles and regressed against 7 independent biometric and age variables. The FAC was correlated with longitudinal fractional shortening (LFS) [((end-diastolic longitudinal length) - (end-systolic longitudinal length) /(end-diastolic longitudinal length)) x 100] obtained from the mid ventricular basal-apical lengths of the right and left ventricular chambers and the transverse fractional shortening (TFS) [((end-diastolic transverse length) - (end-systolic transverse length)/(end-diastolic transverse length)) x 100] from three transverse positions (base, mid, apical) located within each ventricular chamber. To evaluate potential clinical utility, the FAC, LFS, and TFS results were examined in 9 fetuses with congenital heart defects (CHD). Regression analysis demonstrated significant associations between the FAC and the biometric and age independent variables (R 2 = 0.13 - 0.15). The FAC was significantly correlated with the LFS (R 2 =0.18 to 0.28) and TFS (R 2 = 0.13 to 0.33). The 9 fetuses with CHD illustrated the interrelationship between the FAC, LFS, and TFS when identifying abnormal ventricular function. This study reports results from measuring the FAC of the right and left ventricles, and demonstrates a correlation with longitudinal fractional shortening (LFS) and transverse fractional shortening (TFS). This article is protected by copyright. All rights reserved.

Mechanistic basis of desmosome-targeted diseases

NARCIS (Netherlands)

Al-Jassar, Caezar; Bikker, Hennie; Overduin, Michael; Chidgey, Martyn

2013-01-01

Desmosomes are dynamic junctions between cells that maintain the structural integrity of skin and heart tissues by withstanding shear forces. Mutations in component genes cause life-threatening conditions including arrhythmogenic right ventricular cardiomyopathy, and desmosomal proteins are targeted

The ratio of (18)F-FDG activity uptake between the right and left ventricle in patients with pulmonary hypertension correlates with the right ventricular function.

Science.gov (United States)

Yang, Tao; Wang, Lei; Xiong, Chang-Ming; He, Jian-Guo; Zhang, Yan; Gu, Qing; Zhao, Zhi-Hui; Ni, Xin-Hai; Fang, Wei; Liu, Zhi-Hong

2014-05-01

It is known that patients with pulmonary hypertension (PH) can have elevated F-FDG uptake in the right ventricle (RV) on PET imaging. This study was designed to assess possible relationship between FDG uptake of ventricles and the function/hemodynamics of the RV in patients with PH. Thirty-eight patients with PH underwent FDG PET imaging in both fasting and glucose-loading conditions. The standard uptake value (SUVs) corrected for partial volume effect in both RV and left ventricle (LV) were measured. The ratio of FDG uptake between RV to LV (SUVR/L) was calculated. Right heart catheterization and cardiac magnetic resonance (CMR) were performed in all patients within 1 week. The FDG uptake levels by the ventricles were compared with the result form the right heart catheterization and CMR. The SUV of RV (SUVR) and SUV of LV were significantly higher in glucose-loading condition than in fasting condition. In both fasting and glucose-loading conditions, SUVR and SUVR/L showed reverse correlation with right ventricular ejection fraction derived from CMR. In addition, in both fasting and glucose-loading conditions, SUVR and SUVR/L showed positive correlations with pulmonary vascular resistance. However, only SUVR/L in glucose-loading condition could independently predict right ventricular ejection fraction after adjusted for age, body mass index, sex, mean right atrial pressure, mean pulmonary arterial pressure, and pulmonary vascular resistance (P = 0.048). The FDG uptake of RV increases with decreased right ventricular function in patients with PH. Increased FDG uptake ratio between RV and LV might be useful to assess the right ventricular function.

The value of right ventricular longitudinal strain in the evaluation of adult patients with repaired tetralogy of Fallot: a new tool for a contemporary challenge.

Science.gov (United States)

Almeida-Morais, Luís; Pereira-da-Silva, Tiago; Branco, Luísa; Timóteo, Ana T; Agapito, Ana; de Sousa, Lídia; Oliveira, José A; Thomas, Boban; Jalles-Tavares, Nuno; Soares, Rui; Galrinho, Ana; Cruz-Ferreira, Rui

2017-04-01

The role of right ventricular longitudinal strain for assessing patients with repaired tetralogy of Fallot is not fully understood. In this study, we aimed to evaluate its relation with other structural and functional parameters in these patients. Patients followed-up in a grown-up CHD unit, assessed by transthoracic echocardiography, cardiac MRI, and treadmill exercise testing, were retrospectively evaluated. Right ventricular size and function and pulmonary regurgitation severity were assessed by echocardiography and MRI. Right ventricular longitudinal strain was evaluated in the four-chamber view using the standard semiautomatic method. In total, 42 patients were included (61% male, 32±8 years). The mean right ventricular longitudinal strain was -16.2±3.7%, and the right ventricular ejection fraction, measured by MRI, was 42.9±7.2%. Longitudinal strain showed linear correlation with tricuspid annular systolic excursion (r=-0.40) and right ventricular ejection fraction (r=-0.45) (all ptetralogy of Fallot. It correlated with echocardiographic right ventricular function parameters and was independently associated with right ventricular ejection fraction derived by MRI.

Implications of abnormal right ventricular thallium uptake in acute myocardial infarction

International Nuclear Information System (INIS)

Nestico, P.F.; Hakki, A.H.; Felsher, J.; Heo, J.; Iskandrian, A.S.

1986-01-01

The correlates of abnormal right ventricular (RV) thallium uptake were examined in 116 patients with documented acute myocardial infarction (AMI) who underwent predischarge thallium-201 scintigraphy at rest, radionuclide angiography and 24-hour ambulatory electrocardiography. The patients were separated into 2 groups: patients group 1 (n = 31) had increased RV thallium uptake and those in group 2 (n = 85) had no such uptake. The 2 groups were comparable in age, type and site of AMI, peak creatine kinase level, systolic blood pressure and heart rate. However, compared with group 2, group 1 had a lower mean left ventricular (LV) ejection fraction (33 +/- 15% vs 39 +/- 14%, p less than 0.05), higher prevalence of increased lung thallium uptake (45% vs 22%, p less than 0.02), more extensive LV perfusion defects (4.4 +/- 2.9 vs 3.0 +/- 3.0 segments, p less than 0.03) and more complex ventricular arrhythmias (55% vs 35%, p less than 0.05). At a mean follow-up of 6 months, 17 patients (8 in group 1 and 9 in group 2) died from cardiac causes. Actuarial life-table analysis showed that the survival rate was better in group 2 than in group 1 (Mantel-Cox statistics = 4.62, p = 0.03). Thus, patients with AMI and abnormal RV thallium uptake have worse LV function, more complex ventricular arrhythmias and worse prognosis

A modified Glenn shunt reduces right ventricular stroke work during left ventricular assist device therapy.

Science.gov (United States)

Schiller, Petter; Vikholm, Per; Hellgren, Laila

2016-03-01

Right ventricular (RV) failure is a major cause of morbidity and mortality after left ventricular assist device (LVAD) placement and remains hard to predict. We hypothesized that partial surgical exclusion of the RV with a modified Glenn shunt during LVAD treatment would reduce RV stroke work. An LVAD was implanted in eight pigs and a modified Glenn shunt was constructed. A conductance pressure-volume catheter was placed in the right ventricle through the apex. Haemodynamic data and pressure-volume loops were obtained at the following time periods: (i) baseline, (ii) open shunt, (iii) LVAD with closed shunt and (iii) LVAD and open shunt. During LVAD therapy, the right atrial (RA) pressure increased from 9 mmHg (9-9) to 15 mmHg (12-15), P = 0.01. RV stroke volume increased from 30 ml (29-40) to 51 ml (42-53), P work increased to 708 mmHg ml (654-1193) from 535 mmHg ml (424-717), P = 0.04, compared with baseline. During LVAD therapy in combination with a Glenn shunt, the RA pressure decreased from 15 mmHg (12-15) to 10 mmHg (7-11) when compared with LVAD therapy only, P = 0.01. A decrease in RV stroke work from 708 mmHg ml (654-1193) to 465 mmHg ml (366-711), P = 0.04, was seen when the LVAD was combined with a shunt, not significantly different from the baseline value (535 mmHg ml). The developed pressure in the right ventricle decreased from 29 mmHg (26-32) to 21 mmHg (20-24), P work during the use of the shunt with LVAD treatment. A modified Glenn shunt reduced RV volumes, RV stroke work and RA pressure during LVAD therapy in an experimental model of heart failure in pigs. © The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

Quantification of the relative contribution of the different right ventricular wall motion components to right ventricular ejection fraction: the ReVISION method.

Science.gov (United States)

Lakatos, Bálint; Tősér, Zoltán; Tokodi, Márton; Doronina, Alexandra; Kosztin, Annamária; Muraru, Denisa; Badano, Luigi P; Kovács, Attila; Merkely, Béla

2017-03-27

Three major mechanisms contribute to right ventricular (RV) pump function: (i) shortening of the longitudinal axis with traction of the tricuspid annulus towards the apex; (ii) inward movement of the RV free wall; (iii) bulging of the interventricular septum into the RV and stretching the free wall over the septum. The relative contribution of the aforementioned mechanisms to RV pump function may change in different pathological conditions.Our aim was to develop a custom method to separately assess the extent of longitudinal, radial and anteroposterior displacement of the RV walls and to quantify their relative contribution to global RV ejection fraction using 3D data sets obtained by echocardiography.Accordingly, we decomposed the movement of the exported RV beutel wall in a vertex based manner. The volumes of the beutels accounting for the RV wall motion in only one direction (either longitudinal, radial, or anteroposterior) were calculated at each time frame using the signed tetrahedron method. Then, the relative contribution of the RV wall motion along the three different directions to global RV ejection fraction was calculated either as the ratio of the given direction's ejection fraction to global ejection fraction and as the frame-by-frame RV volume change (∆V/∆t) along the three motion directions.The ReVISION (Right VentrIcular Separate wall motIon quantificatiON) method may contribute to a better understanding of the pathophysiology of RV mechanical adaptations to different loading conditions and diseases.

Knowledge-based reconstruction for measurement of right ventricular volumes on cardiovascular magnetic resonance images in a mixed population.

Science.gov (United States)

Pieterman, Elise D; Budde, Ricardo P J; Robbers-Visser, Daniëlle; van Domburg, Ron T; Helbing, Willem A

2017-09-01

Follow-up of right ventricular performance is important for patients with congenital heart disease. Cardiac magnetic resonance imaging is optimal for this purpose. However, observer-dependency of manual analysis of right ventricular volumes limit its use. Knowledge-based reconstruction is a new semiautomatic analysis tool that uses a database including knowledge of right ventricular shape in various congenital heart diseases. We evaluated whether knowledge-based reconstruction is a good alternative for conventional analysis. To assess the inter- and intra-observer variability and agreement of knowledge-based versus conventional analysis of magnetic resonance right ventricular volumes, analysis was done by two observers in a mixed group of 22 patients with congenital heart disease affecting right ventricular loading conditions (dextro-transposition of the great arteries and right ventricle to pulmonary artery conduit) and a group of 17 healthy children. We used Bland-Altman analysis and coefficient of variation. Comparison between the conventional method and the knowledge-based method showed a systematically higher volume for the latter group. We found an overestimation for end-diastolic volume (bias -40 ± 24 mL, r = .956), end-systolic volume (bias -34 ± 24 mL, r = .943), stroke volume (bias -6 ± 17 mL, r = .735) and an underestimation of ejection fraction (bias 7 ± 7%, r = .671) by knowledge-based reconstruction. The intra-observer variability of knowledge-based reconstruction varied with a coefficient of variation of 9% for end-diastolic volume and 22% for stroke volume. The same trend was noted for inter-observer variability. A systematic difference (overestimation) was noted for right ventricular size as assessed with knowledge-based reconstruction compared with conventional methods for analysis. Observer variability for the new method was comparable to what has been reported for the right ventricle in children and congenital

Assessment of congenital heart disease by a thallium-201 SPECT study in children; Accuracy of estimated right to left ventricular pressure ratio

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Ishii, Iwao; Nakajima, Kenichi; Taki, Junichi; Taniguchi, Mitsuru; Bunko, Hisashi; Tonami, Norihisa; Hisada, Kinichi; Ohno, Takashi (Kanazawa Univ. (Japan). School of Medicine)

1993-01-01

The characteristics of correlation between the right-to-left ventricular systolic pressure ratios (RVp/LVp) and the thallium-201 right-to-left ventricular ([sup 201]Tl R/L) count ratios was investigated in children with various congenital heart diseases. High-resolution three-headed SPECT system equipped with either parallel-hole or fan-beam collimators was used. In a total of 102 patients, the correlation between RVp/LVp and [sup 201]Tl R/L average count ratios was good in both planar (r=0.89, p=0.0001) and SPECT studies (r=0.80, p=0.0001). Quantitative analysis of myocardial uptake by SPECT demonstrated the characteristic pattern of each disease as well as the differences in the right ventricular overload types. When the linear regression analysis was performed in each heart disease, ventricular septal defect showed most excellent correlation. Complex heart anomalies also showed positive correlation (r=0.51, p=0.05) with RVp/LVp, and it can be used to estimate right ventricular pressure. After surgical treatment of tetralogy of Fallot and pulmonary stenosis, the decrease of [sup 201]Tl R/L count ratio was in accordance with improvement of right ventricular overload. We conclude that [sup 201]Tl SPECT study can be a good indicator for estimation of right ventricular pressure. (author).

Wave Intensity Analysis of Right Ventricular Function during Pulsed Operation of Rotary Left Ventricular Assist Devices.

Science.gov (United States)

Bouwmeester, J Christopher; Park, Jiheum; Valdovinos, John; Bonde, Pramod

2018-05-29

Changing the speed of left ventricular assist devices (LVADs) cyclically may be useful to restore aortic pulsatility; however, the effects of this pulsation on right ventricular (RV) function are unknown. This study investigates the effects of direct ventricular interaction by quantifying the amount of wave energy created by RV contraction when axial and centrifugal LVADs are used to assist the left ventricle. In 4 anesthetized pigs, pressure and flow were measured in the main pulmonary artery and wave intensity analysis was used to identify and quantify the energy of waves created by the RV. The axial pump depressed the intensity of waves created by RV contraction compared with the centrifugal pump. In both pump designs, there were only minor and variable differences between the continuous and pulsed operation on RV function. The axial pump causes the RV to contract with less energy compared with a centrifugal design. Diminishing the ability of the RV to produce less energy translates to less pressure and flow produced, which may lead to LVAD-induced RV failure. The effects of pulsed LVAD operation on the RV appear to be minimal during acute observation of healthy hearts. Further study is necessary to uncover the effects of other modes of speed modulation with healthy and unhealthy hearts to determine if pulsed operation will benefit patients by reducing LVAD complications.

[Cochleovestibular dysplasia: a case report].

Science.gov (United States)

Darbi, A; Cenoui, F; Atmane, A; Amil, T; Hanine, A

2010-04-01

Cochleovestibular dysplasia or Mondini malformation is exceptional. Cochlear dysplasia is due to early cessation of the development of the inner ear during embryonic life. We report on the case of an infant who presented with perception deafness and repeated meningitis. CT allowed the diagnosis of Mondini malformation. We underline the importance of high-resolution CT in the diagnosis of Mondini malformation. Copyright 2010 Elsevier Masson SAS. All rights reserved.

Intravenous sildenafil in right ventricular dysfunction with pulmonary hypertension following a heart transplant

Directory of Open Access Journals (Sweden)

Luis Almenar Bonet

2014-08-01

Full Text Available The objective of the present work is to describe the experience with intravenous (IV sildenafil in heart transplant (HT patients with reactive pulmonary hypertension (PH who developed right ventricular dysfunction (RVD in the immediate postoperative period. The first 5 patients who received IV sildenafil followinga HT are presented. The HTs took place between March 2011 and September 2012 in patients aged 37 to 64 years; all patients were male. Prior to the HT, mean pulmonary artery pressure (mPAP was 32-56 mmHg. In all cases, the hemodynamic study demonstrated PH reactivity (positive vasodilator test with nitric oxide. All 5 patients developed RVD with hemodynamic instability immediately after the HT, despite the administration of nitric oxide from the time of intubation prior to the implant, optimal medical treatment in all cases, and a ventricular assist in 2 cases. In all patients, IV sildenafil was initiated at 10 mg/8 h for 48 h and was subsequently increased to 20 mg/8 h. in its oral formulation until discharge from the hospital. The change in pulmonary pressure was assessed using a Swan-Ganz catheter. Ventricular function was assessed using echocardiography. Length of stay in the Resuscitation Unit and mid-term survival were also assessed. Average time of extracorporeal circulation was 200 ± 110 min and organ ischemic time was 210 ± 95 min. All of the patients demonstrated pulmonary and systemic hemodynamic improvement, as well as recovery of right ventricular function after completing the treatment with IV sildenafil. The stay in the Resuscitation Unit lasted 3-25 days. All the patients were discharged from hospital with no mortality to date. Intravenous sildenafil improves right ventricle hemodynamics associated with pulmonary hypertension post-HT. Prophylactic prevention with this drug could be indicated for patients with reactive PH who are about to receive a transplant.

Intravenous sildenafil in right ventricular dysfunction with pulmonary hypertension following a heart transplant.

Science.gov (United States)

Bonet, Luis Almenar; Guillén, Rosario Vicente; Lázaro, Ignacio Sánchez; de la Fuente, Carmen; Osseyran, Faisa; Dolz, Luis Martínez; Hernández, Mónica Montero; Sanz, Manuel Portolés; Otero, Miguel Rivera; Sanz, Antonio Salvador

2014-01-01

The objective of the present work is to describe the experience with intravenous (IV) sildenafil in heart transplant (HT) patients with reactive pulmonary hypertension (PH) who developed right ventricular dysfunction (RVD) in the immediate postoperative period. The first 5 patients who received IV sildenafil followinga HT are presented. The HTs took place between March 2011 and September 2012 in patients aged 37 to 64 years; all patients were male. Prior to the HT, mean pulmonary artery pressure (mPAP) was 32-56 mmHg. In all cases, the hemodynamic study demonstrated PH reactivity (positive vasodilator test with nitric oxide). All 5 patients developed RVD with hemodynamic instability immediately after the HT, despite the administration of nitric oxide from the time of intubation prior to the implant, optimal medical treatment in all cases, and a ventricular assist in 2 cases. In all patients, IV sildenafil was initiated at 10 mg/8 h for 48 h and was subsequently increased to 20 mg/8 h. in its oral formulation until discharge from the hospital. The change in pulmonary pressure was assessed using a Swan-Ganz catheter. Ventricular function was assessed using echocardiography. Length of stay in the Resuscitation Unit and mid-term survival were also assessed. Average time of extracorporeal circulation was 200 ± 110 min and organ ischemic time was 210 ± 95 min. All of the patients demonstrated pulmonary and systemic hemodynamic improvement, as well as recovery of right ventricular function after completing the treatment with IV sildenafil. The stay in the Resuscitation Unit lasted 3-25 days. All the patients were discharged from hospital with no mortality to date. Intravenous sildenafil improves right ventricle hemodynamics associated with pulmonary hypertension post-HT. Prophylactic prevention with this drug could be indicated for patients with reactive PH who are about to receive a transplant.

Arterial homograft and medical therapy in pseudoaneurysm of infrarenal aorta concomitant with recurrent right ventricular thrombus in Behcet's disease

International Nuclear Information System (INIS)

Vahedian, Jalal; Sadeghapour, Ali

2006-01-01

Pseudoaneurysm of the infrarenal aorta due to Behcet s disease is very rare. We report a case of pseudoaneurysm associated with Behcet s disease in a 17-year-old boy with a recurrent right ventricular thrombus successfully treated with revascularization using arterial homograft patch. Echocardiography examination revealed a right ventricular mass, thought to be a thrombus in an unusual location. Postoperatively, the findings of the pathologic examination confirmed the mass as a thrombus. When the patient was subsequently re-admitted to the emergency unit with complaints of severe abdominal pain, fever, fatigue, sinus tachycardia, and a pulsating and tender abdominal mass, a right ventricular thrombus and a large pseudoaneurysm of the abdominal aorta were found on echocardiography and angiography. The patient underwent resection of the aortic aneurysm and aortoplasty, using arterial homograft patch, and received immunosuppressive and anticoagulation therapy. The thrombus of the right ventricle disappeared 4 months later. This case indicates that a right ventricular thrombosis in Behcet s disease may be managed by medical therapy. (author)

Serial Noninvasive Assessment of Apoptosis During Right Ventricular Disease Progression in Rats

NARCIS (Netherlands)

Campian, Maria E.; Verberne, Hein J.; Hardziyenka, Maxim; de Bruin, Kora; Selwaness, Mariana; van den Hoff, Maurice J. B.; Ruijter, Jan M.; van Eck-Smit, Berthe L. F.; de Bakker, Jacques M. T.; Tan, Hanno L.

2009-01-01

Right ventricular (RV) function is the major determinant of survival in patients with pulmonary hypertension. Yet, the pathophysiologic basis of RV disease is unresolved. We aimed to study the role of apoptosis in RV disease by monitoring it serially during disease progression using in vivo

Polyostotic Fibrous Dysplasia of Cranio-Maxillofacial Area

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Han, Jin Woo; Kwon, Hyuk Rok; Lee, Jin Ho; Park, In Woo [Dept. of Oral and Maxillofacial Radiology, College of Dentistry, Kangnung National University, Kangnung (Korea, Republic of)

2000-06-15

Fibrous dysplasia is believed to be a hamartomatous developmental lesion of unknown origin. This disease is divided into monostotic and polyostotic fibrous dysplasia. Polyostotic type can be divided into craniofacial type, Lichtenstein-Jaffe type, and McCune-Albright syndrome. In this case, a 31-year-old female presented spontaneous loss of right mandibular teeth before 5 years and has shown continuous expansion of right mandibular alveolus. Through the radiographic view, the coarse pattern of the mixed radiopaque-lucent lesion was seen on the right mandibular body, and there was diffuse pattern of the mixed radiopaque-lucent lesion with ill-defined margin in the left mandibular body. In the right calvarium, the lesion had cotton-wool appearance. Partial excision for contouring, multiple extraction, and alveoloplasty were accomplished under general anesthesia for supportive treatment. Finally we could conclude this case was polyostotic fibrous dysplasia of cranio-maxillofacial area based on the clinical, radiologic finding, and histopathologic examination.

Polyostotic Fibrous Dysplasia of Cranio-Maxillofacial Area

International Nuclear Information System (INIS)

Han, Jin Woo; Kwon, Hyuk Rok; Lee, Jin Ho; Park, In Woo

2000-01-01

Fibrous dysplasia is believed to be a hamartomatous developmental lesion of unknown origin. This disease is divided into monostotic and polyostotic fibrous dysplasia. Polyostotic type can be divided into craniofacial type, Lichtenstein-Jaffe type, and McCune-Albright syndrome. In this case, a 31-year-old female presented spontaneous loss of right mandibular teeth before 5 years and has shown continuous expansion of right mandibular alveolus. Through the radiographic view, the coarse pattern of the mixed radiopaque-lucent lesion was seen on the right mandibular body, and there was diffuse pattern of the mixed radiopaque-lucent lesion with ill-defined margin in the left mandibular body. In the right calvarium, the lesion had cotton-wool appearance. Partial excision for contouring, multiple extraction, and alveoloplasty were accomplished under general anesthesia for supportive treatment. Finally we could conclude this case was polyostotic fibrous dysplasia of cranio-maxillofacial area based on the clinical, radiologic finding, and histopathologic examination.

Epicardial Breakthrough Waves During Sinus Rhythm: Depiction of the Arrhythmogenic Substrate?

Science.gov (United States)

Mouws, Elisabeth M J P; Lanters, Eva A H; Teuwen, Christophe P; van der Does, Lisette J M E; Kik, Charles; Knops, Paul; Bekkers, Jos A; Bogers, Ad J J C; de Groot, Natasja M S

2017-09-01

Epicardial breakthrough waves (EBW) during atrial fibrillation are important elements of the arrhythmogenic substrate and result from endo-epicardial asynchrony, which also occurs to some degree during sinus rhythm (SR). We examined the incidence and characteristics of EBW during SR and its possible value in the detection of the arrhythmogenic substrate associated with atrial fibrillation. Intraoperative epicardial mapping (interelectrode distances 2 mm) of the right atrium, Bachmann's bundle, the left atrioventricular groove, and the pulmonary vein area was performed during SR in 381 patients (289 male, 67±10 years) with ischemic or valvular heart disease. EBW were referred to as sinus node breakthrough waves if they were the earliest right atrial activated site. A total of 218 EBW and 57 sinus node breakthrough waves were observed in 168 patients (44%). EBW mostly occurred at right atrium (N=105, 48%) and left atrioventricular groove (N=67, 31%), followed by Bachmann's bundle (N=27, 12%) and pulmonary vein area (N=19, 9%; P <0.001). EBW occurred most often in ischemic heart disease patients (N=114, 49%) compared with (ischemic and) valvular heart disease patients (N=26, 17%; P <0.001). EBW electrograms most often consisted of double and fractionated potentials (N=137, 63%). In case of single potentials, an R wave was observed in 88% (N=71) of EBW, as opposed to 21% of sinus node breakthrough waves (N=5; P <0.001). Fractionated EBW potentials were more often observed at the right atrium and Bachmann's bundle ( P <0.001). During SR, EBW are present in over a third of patients, particularly in thicker parts of the atrial wall. Features of SR EBW indicate that muscular connections between endo- and epicardium underlie EBW and that a slight degree of endo-epicardial asynchrony required for EBW to occur is already present in some areas during SR. Hence, an anatomic substrate is present, which may enhance the occurrence of EBW during atrial fibrillation, thereby

Comparison of Venous Return Characteristics with Right Ventricular Mechanics During Cephalic Fluid Shift

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Elliott, Morgan; Martin, David

2015-01-01

For my summer internship project, I organized a pilot study to analyze the effects of a cephalic fluid shift on venous return and right ventricular mechanics to increase right ventricular and venous knowledge. To accomplish this pilot study, I wrote a testing protocol, obtained Institutional Review Board (IRB) approval, completed subject payment forms, lead testing sessions, and analyzed the data. This experiment used -20deg head down tilt (20 HDT) as the ground based simulation for the fluid shift that occurs during spaceflight and compared it to data obtained from the seated and supine positions. Using echocardiography, data was collected for the right ventricle, hepatic vein, internal jugular vein, external jugular vein, and inferior vena cava. Additionally, non-invasive venous pressure measurements, similar to those soon to be done in-orbit, were collected. It was determined that the venous return from below the heard is increased during 20 HDT, which was supported by increased hepatic vein velocities, increased right ventricular inflow, and increased right ventricular strain at 20 HDT relative to seated values. Jugular veins in the neck undergo an increase in pressure and area, but no significant increase in flow, relative to seated values when a subject is tilted 20 HDT. Contrary to the initial expectations based on this jugular flow, there was no significant increase in central venous pressure, as evidenced by no change in Doppler indices for right arterial pressure or inferior vena cava diameter. It is suspected that these differences in pressure are due to the hydrostatic pressure indifference point shifting during tilt; there is a potential for a similar phenomenon with microgravity. This data will hopefully lead to a more in-depth understanding of the response of the body to microgravity and how those relate to the previously mentioned cardiovascular risk of fluid shift that is associated with spaceflight. These results were presented in greater detail

Genetic basis of arrhythmogenic cardiomyopathy.

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Karmouch, Jennifer; Protonotarios, Alexandros; Syrris, Petros

2018-05-01

To date 16 genes have been associated with arrhythmogenic cardiomyopathy (ACM). Mutations in these genes can lead to a broad spectrum of phenotypic expression ranging from disease affecting predominantly the right or left ventricle, to biventricular subtypes. Understanding the genetic causes of ACM is important in diagnosis and management of the disorder. This review summarizes recent advances in molecular genetics and discusses the application of next-generation sequencing technology in genetic testing in ACM. Use of next-generation sequencing methods has resulted in the identification of novel causative variants and genes for ACM. The involvement of filamin C in ACM demonstrates the genetic overlap between ACM and other types of cardiomyopathy. Putative pathogenic variants have been detected in cadherin 2 gene, a protein involved in cell adhesion. Large genomic rearrangements in desmosome genes have been systematically investigated in a cohort of ACM patients. Recent studies have identified novel causes of ACM providing new insights into the genetic spectrum of the disease and highlighting an overlapping phenotype between ACM and dilated cardiomyopathy. Next-generation sequencing is a useful tool for research and genetic diagnostic screening but interpretation of identified sequence variants requires caution and should be performed in specialized centres.

Do right-ventricular trabeculae gain energetic advantage from having a greater velocity of shortening?

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Pham, Toan; Han, June-Chiew; Taberner, Andrew; Loiselle, Denis

2017-10-15

We designed a study to test whether velocity of shortening in right-ventricular tissue preparations is greater than that of the left side under conditions mimicking those encountered by the heart in vivo. Our experiments allowed us to explore whether greater velocity of shortening results in any energetic advantage. We found that velocity of shortening was higher in the rat right-ventricular trabeculae. These results at the tissue level seem paradoxical to the velocity of ventricular ejection at the organ level, and are not always in accord with shortening of unloaded cells. Despite greater velocity of shortening in right-ventricular trabeculae, they neither gained nor lost advantage with respect to both mechanical efficiency and the heat generated during shortening. Our study aimed to ascertain whether the interventricular difference of shortening velocity, reported for isolated cardiac tissues in vitro, affects interventricular mechano-energetic performance when tested under physiological conditions using a shortening protocol designed to mimic those in vivo. We isolated trabeculae from both ventricles of the rat, mounted them in a calorimeter, and performed experiments at 37°C and 5 Hz stimulus frequency to emulate conditions of the rat heart in vivo. Each trabecula was subjected to two experimental protocols: (i) isotonic work-loop contractions at a variety of afterloads, and (ii) isometric contractions at a variety of preloads. Velocity of shortening was calculated from the former protocol during the isotonic shortening phase of the contraction. Simultaneous measurements of force-length work and heat output allowed calculation of mechanical efficiency. The shortening-dependent thermal component was quantified from the difference in heat output between the two protocols. Our results show that both extent of shortening and velocity of shortening were higher in trabeculae from the right ventricle. Despite these differences, trabeculae from both ventricles

TIPSS Procedure in the Treatment of a Single Patient After Recent Heart Transplantation Because of Refractory Ascites Due to Cardiac Cirrhosis

International Nuclear Information System (INIS)

Fava, Mario; Meneses, Luis; Loyola, Soledad; Castro, Pablo; Barahona, Fernando

2008-01-01

We present the case of a female patient with arrhythmogenic dysplasia of the right ventricle who evolved to refractory heart failure, ascites, and peripheral edema. As a result, heart transplantation was performed. Subsequently, refractory ascites impaired the patient's respiratory function, resulting in prolonged mechanical ventilation. She was successfully treated with transjugular intrahepatic portosystemic shunt (TIPSS) placement, which allowed satisfactory weaning of ventilatory support.

Bilateral multicystic renal dysplasia with potter sequence: A case with penile agenesis

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Dursun, Ahmet; Ermis, Bahri; Bahadir, Burak; Seckiner, Ilker

2006-01-01

Hereditary renal adysplasia (HRA) is rare autosomal dominant condition. Patients have several other anomalies including Potter facies, thoracic, cardiac, and extremity deformities. The case present dysmorphic facial features such as hypertelorism, prominent epicanthic folds, a flat and broad nose, choanal stenosis, low set ears and a receding chin. He had femoral bowing, hypoplastic right tibia and agenesis of the right foot. He had rich and thin skin. He had also a dysplastic empty scrotum, penile agenesis, and anal atresia. The autopsy revealed pulmonary hypoplasia, ventricular septal defect, bilateral multicystic renal dysplasia, agenesis of ureter and bladder, intraabdominal testicles, and a single umbilical artery. The penile agenesis was first reported, and including the consanguinity in the parents might further delineate the bilateral multicystic HRA. Vater/caudal regression anomalies, Mullerian duct/aplasia, unilateral renal agenesis, and cervicothoracic somite anomalies association and Coloboma, heart anomaly, choanal atresia, retardation, retardation, genital and ear anomalies syndrome has been considered in differential diagnosis. (author)

Efecto de la localización del electrodo ventricular derecho (tracto de salida vs. ápex sobre la sincronía ventricular mecánica, en pacientes sometidos a terapia de implante de marcapaso cardiaco Effect of right ventricular electrode location (outflow tract vs. apex on mechanical ventricular synchrony in patients that underwent pacemaker implant therapy

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Oscar S Rincón

2008-12-01

Full Text Available Objetivo: evaluar a profundidad el efecto de la estimulación ventricular desde el tracto de salida del ventrículo derecho y el ápex, sobre la sincronía ventricular mecánica. Materiales y métodos: estudio analítico de cohorte, en el que se realizó ecocardiograma transtorácico pre y post implante de marcapaso a 20 pacientes (diez por cada grupo con indicación de marcapaso definitivo, con implante del electrodo en el tracto de salida del ventrículo derecho y el ápex, sin cardiopatía estructural, fracción de eyección > 50%; QRS y conducción aurículo-ventricular normal, con el fin de evaluar la asincronía ventricular mecánica (modo M y Doppler tisular y los parámetros de implante y programación del dispositivo. Análisis estadístico: los resultados se presentan como promedios, desviación estándar o porcentajes. Las variables continuas se compararon utilizando prueba Chi cuadrado y ANOVA. Se consideró como estadísticamente significativa una p Objective: to assess in depth the effect of ventricular stimulation from the right ventricular outflow tract and the apex on mechanical ventricular synchrony. Materials and Methods: cohort analytical study. 20 patients with indication of definitive pacemaker indication underwent transthoracic echocardiogram before and after pacemaker implant with electrode implantation in the right ventricular outflow tract and in the apex (10 patients in each group. There was no structural cardiopathy, ejection fraction was > 50%, QRS and AV conduction were normal. Mechanical ventricular asynchrony (M mode and tissue doppler and implant and device parameters were evaluated. Statistical Analysis: results are given as mean values, standard deviation or percentages. Continuous variables were compared using Chi-square test and ANOVA. A p <0.05 value was considered statistically significant. Results: in five patients (25% a pre-implant ventricular asynchrony was found; in seven (70% ventricular asynchrony

Global Optimization of Ventricular Myocyte Model to Multi-Variable Objective Improves Predictions of Drug-Induced Torsades de Pointes

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Trine Krogh-Madsen

2017-12-01

Full Text Available In silico cardiac myocyte models present powerful tools for drug safety testing and for predicting phenotypical consequences of ion channel mutations, but their accuracy is sometimes limited. For example, several models describing human ventricular electrophysiology perform poorly when simulating effects of long QT mutations. Model optimization represents one way of obtaining models with stronger predictive power. Using a recent human ventricular myocyte model, we demonstrate that model optimization to clinical long QT data, in conjunction with physiologically-based bounds on intracellular calcium and sodium concentrations, better constrains model parameters. To determine if the model optimized to congenital long QT data better predicts risk of drug-induced long QT arrhythmogenesis, in particular Torsades de Pointes risk, we tested the optimized model against a database of known arrhythmogenic and non-arrhythmogenic ion channel blockers. When doing so, the optimized model provided an improved risk assessment. In particular, we demonstrate an elimination of false-positive outcomes generated by the baseline model, in which simulations of non-torsadogenic drugs, in particular verapamil, predict action potential prolongation. Our results underscore the importance of currents beyond those directly impacted by a drug block in determining torsadogenic risk. Our study also highlights the need for rich data in cardiac myocyte model optimization and substantiates such optimization as a method to generate models with higher accuracy of predictions of drug-induced cardiotoxicity.

Usefulness of right ventricular and right atrial two-dimensional speckle tracking strain to predict late arrhythmic events in adult patients with repaired Tetralogy of Fallot.

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Timóteo, Ana T; Branco, Luísa M; Rosa, Sílvia A; Ramos, Ruben; Agapito, Ana F; Sousa, Lídia; Galrinho, Ana; Oliveira, José A; Oliveira, Mário M; Ferreira, Rui C

2017-01-01

To determine whether right ventricular and/or atrial speckle tracking strain is associated with previous arrhythmic events in patients with repaired tetralogy of Fallot. We studied right ventricular and atrial strain in 100 consecutive patients with repaired tetralogy of Fallot referred for routine echocardiographic evaluation. Patients were divided into two groups, one with previous documentation of arrhythmias (n=26) and one without arrhythmias, in a median follow-up of 22 years. Patients with arrhythmias were older (pFallot, although a prospective study is required. Copyright © 2016 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.

Atrialised right ventricular myxoma in a patient with Ebstein’s anomaly

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T-J John

2018-03-01

Full Text Available Ebstein’s anomaly is a rare entity affecting around 1 in 200,000 live births and accounts for less than 1% of congenital heart diseases. Ebstein’s anomaly with an associated right-sided myxoma is extremely rare, with only one other case report found in the literature. Previous reports have also noted cases of Ebstein’s anomaly associated with left-sided myxomas. We describe a female patient with, to our knowledge, the first case of a histopathologically confirmed right ventricular myxoma in the setting of Ebstein’s anomaly.

Characterizing the Molecular Pathology of Arrhythmogenic Cardiomyopathy in Patient Buccal Mucosa Cells.

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Asimaki, Angeliki; Protonotarios, Alexandros; James, Cynthia A; Chelko, Stephen P; Tichnell, Crystal; Murray, Brittney; Tsatsopoulou, Adalena; Anastasakis, Aris; te Riele, Anneline; Kléber, André G; Judge, Daniel P; Calkins, Hugh; Saffitz, Jeffrey E

2016-02-01

Analysis of myocardium has revealed mechanistic insights into arrhythmogenic cardiomyopathy but cardiac samples are difficult to obtain from probands and especially from family members. To identify a potential surrogate tissue, we characterized buccal mucosa cells. Buccal cells from patients, mutation carriers, and controls were immunostained and analyzed in a blinded fashion. In additional studies, buccal cells were grown in vitro and incubated with SB216763. Immunoreactive signals for the desmosomal protein plakoglobin and the major cardiac gap junction protein Cx43 were markedly diminished in buccal mucosa cells from arrhythmogenic cardiomyopathy patients with known desmosomal mutations when compared with controls. Plakoglobin and Cx43 signals were also reduced in most family members who carried disease alleles but showed no evidence of heart disease. Signal for the desmosomal protein plakophilin-1 was reduced in buccal mucosa cells in patients with PKP2 mutations but not in those with mutations in other desmosomal genes. Signal for the desmosomal protein desmoplakin was reduced in buccal mucosa cells from patients with mutations in DSP, DSG2, or DSC2 but not in PKP2 or JUP. Abnormal protein distributions were reversed in cultured cells incubated with SB216763, a small molecule that rescues the disease phenotype in cardiac myocytes. Buccal mucosa cells from arrhythmogenic cardiomyopathy patients exhibit changes in the distribution of cell junction proteins similar to those seen in the heart. These cells may prove useful in future studies of disease mechanisms and drug screens for effective therapies in arrhythmogenic cardiomyopathy. © 2016 American Heart Association, Inc.

Echocardiographic evaluation of right ventricular stroke work index in advanced heart failure: a new index?

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Frea, Simone; Bovolo, Virginia; Bergerone, Serena; D'Ascenzo, Fabrizio; Antolini, Marina; Capriolo, Michele; Canavosio, Federico Giovanni; Morello, Mara; Gaita, Fiorenzo

2012-12-01

Right ventricular (RV) function plays a pivotal role in advanced heart failure patients, especially for screening those who may benefit from left ventricular assist device (LVAD) implantation. We introduce RV contraction pressure index (RVCPI) as a new echo-Doppler parameter of RV function. The accuracy of RVCPI in detecting RV failure was compared with the criterion standard, the RV stroke work index (RVSWI) obtained through right heart catheterization in advanced heart failure patients referred for heart transplantation or LVAD implantation. Right heart catheterization and echo-Doppler were simultaneously performed in 94 consecutive patients referred to our center for advanced heart failure (ejection fraction (EF) 24 ± 8.8%, 40% NYHA functional class IV). RV stroke volume and invasive pulmonary pressures were used to obtain RVSWI. Simplified RVCPI (sRVCPI) was derived as TAPSE × (RV - right atrial pressure gradient). Close positive correlation between sRVCPI and RVSWI was found (r = 0.68; P rights reserved.

Thallium-201 myocardial imaging in children with heart disease. Quantitative assessment of right ventricular pressure

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Mitomori, Toshihiro; Ono, Yasuo; Kamiya, Tetsuro; Nishimura, Tsunehiko; Kozuka, Takahiro (National Cardiovascular Center, Suita, Osaka (Japan)); Kohata, Tohru; Tanimoto, Takeshi

1985-02-01

201-TlCl myocardial imaging studies were performed to evaluate systolic pressure of right ventricle on 107 patients including 89 patients with congenital cardiac disease, patients with primary pulmonary hypertension and 13 patients with history of MCLS with the age range of 2 months to 17 years. The congenital cardiac disease group included 30 patients with tetralogy of Fallot, 8 patients with pulmonary stenosis (included 1 patient with double chambered right ventricle), 20 patients with ventricular septal defect, 10 patients with patent ductus arteriosus, 9 patients with atrial septal defect and 12 patients with complete transposition of great arteries. None of the patients with history of MCLS had coronary involvement. 50 ..mu..Ci/kg of 201-TlCl was infused intravenously and after 15 minutes the images were obtained from 5 directions (anterior, LAO 30, LAO 45, LAO 60 and lateral). The angle was determined to demonstrate the intraventricular septum and ventricular free walls cleary separated. The image of end-diastolic phase was obtained with ECG synchronized gated method from that direction. The ROI (region of interest) was defined as a slice line by drawing two lines perpendicular to the septum and the counts of the left and right ventricular free wall (Cl and Cr) were analyzed to evaluate the pressure of right ventricle. The pressure of ventricles were obtained by cardiac catheterization performed at the same time with myocardial imaging. The ratio of Cl and Cr (Cr/Cl) had good correlation with the ratio of peak systolic pressure of left ventricle and right ventricle (regression equation Y=0.84X + 0.08, r=0.87). In each congenital heart disease group, very good correlation was also demonstrated (Y=1.46X - 0.30, r=0.96 in PS; Y=0.70X0.20, r=0.91 in VSD; Y=0.86X + 0.15, r=0.78 in ASD and Y=0.72X + 0.24, r=0.80 in PDA). Thus 201-TlCl myocardial imaging was useful method to evaluate right ventricular pressure noninvasively in congenital cardiac disease.

Right ventricular to pulmonary artery conduit instead of modified Blalock-Taussig shunt improves postoperative hemodynamics in newborns after the Norwood operation.

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Mair, Rudolf; Tulzer, Gerald; Sames, Eva; Gitter, Roland; Lechner, Evelyn; Steiner, Jürgen; Hofer, Anna; Geiselseder, Gertraud; Gross, Christoph

2003-11-01

Perioperative mortality, prolonged postoperative recovery after the Norwood procedure, and mortality between stage I and stage II might be related to shunt physiology. A right ventricular to pulmonary artery conduit offers a banded physiology in contrast to a Blalock-Taussig shunt. The purpose of this study was to assess the hemodynamic differences and their consequences in the postoperative course between Norwood patients with a Blalock-Taussig shunt and those with a right ventricular to pulmonary artery conduit. From October 1999 until May 2002, 32 unselected consecutive patients underwent a Norwood procedure at the General Hospital Linz. The first 18 patients received a Blalock-Taussig shunt. In the remaining 14 patients we performed a right ventricular to pulmonary artery conduit. Both groups were compared. The diastolic blood pressure was significantly higher in the right ventricular to pulmonary artery conduit group (P <.001). Despite a higher FIO(2), PO(2) levels tended to be lower in the first 5 postoperative days. At the age of 3 months, catheterization laboratory data showed a lower Qp/Qs ratio in the same group (0.86 [0.78; 1] versus 1.55 [1.15; 1.6]; P =.005) and a higher dp/dt (955 [773; 1110] vs 776 [615; 907]; P =.018). (Descriptive data reflect medians and quartiles [in brackets].) Hospital survival was 72% in the Blalock-Taussig shunt group versus 93% in the right ventricular to pulmonary artery conduit group. Mortality between stage I and stage II was 23% in the Blalock-Taussig shunt group versus 0% in the right ventricular to pulmonary artery conduit group. A higher diastolic blood pressure and a lower Qp/Qs ratio were associated with a more stable and efficient circulation in patients with a right ventricular to pulmonary artery conduit. More intensive ventilatory support was necessary during the first postoperative days. We did not note any adverse effects of the ventriculotomy on ventricular performance.

CT Imaging of Craniofacial Fibrous Dysplasia

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Zerrin Unal Erzurumlu

2015-01-01

Full Text Available Fibrous dysplasia is a benign fibroosseous bone dysplasia that can involve single (monostotic or multiple (polyostotic bones. Monostotic form is more frequent in the jaws. It is termed as craniofacial fibrous dysplasia, when it involves, though rarely, adjacent craniofacial bones. A 16-year-old girl consulted for a painless swelling in the right posterior mandible for two years. Panoramic radiography revealed ground-glass ill-defined lesions in the three different regions of the maxilla and mandible. Axial CT scan (bone window showed multiple lesions involving skull base and facial bones. Despite lesions in the skull base, the patient had no abnormal neurological findings. The lesion was diagnosed as fibrous dysplasia based on radiological and histopathological examination. In this paper, CT findings and differential diagnosis of CFD are discussed. CT is a useful imaging technique for CFD cases.

Lamin A/C mutation affecting primarily the right side of the heart

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Laura Ollila

2013-04-01

Full Text Available LMNA mutations are amongst the most important causes of familial dilated cardiomyopathy. The most important cause of arrhythmogenic right ventricular cardiomyopathy (ARVC is desmosomal pathology. The aim of the study was to elucidate the role of LMNA mutations among Finnish cardiomyopathy patients. We screened 135 unrelated cardiomyopathy patients for LMNA mutations. Because of unusual phenotype, two patients were screened for the known Finnish ARVC-related mutations of desmosomal genes, and their Plakophilin-2b gene was sequenced. Myocardial samples from two patients were examined by immunohistochemical plakoglobin staining and in one case by electron microscopy. We found a new LMNA mutation Phe237Ser in a family of five affected members with a cardiomyopathy affecting primarily the right side of the heart. The phenotype resembles ARVC but does not fulfill the Task Force Criteria. The main clinical manifestations of the mutation were severe tricuspid insufficiency, right ventricular enlargement and failure. Three of the affected patients died of the heart disease, and the two living patients received heart transplants at ages 44 and 47. Electron microscopy showed nuclear blebbing compatible with laminopathy. Immunohisto - chemical analysis did not suggest desmosomal pathology. No desmosomal mutations were found. The Phe237Ser LMNA mutation causes a phenotype different from traditional cardiolaminopathy. Our findings suggest that cardiomyopathy affecting primarily the right side of the heart is not always caused by desmosomal pathology. Our observations highlight the challenges in classifying cardiomyopathies, as there often is significant overlap between the traditional categories.

The association of sphenoidal encephalocele and right anophthalmia with septo-optic dysplasia: a case report.

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Erol, Fatih Serhat; Ucler, Necati; Kaplan, Metin; Yilmaz, Ilhan

2012-01-01

Septo-optic dysplasia (SOD) is an extremely rare congenital anomaly, characterized with optic nerve hypoplasia and absence of septum pellucidum and/or pituitary dysfunction. In addition to classical findings of SOD, we report for the first time an 11-year-old boy, with encephalocele extending to the right sphenoidal sinus, right anophthalmia and normal pituitary functions. Despite all the major anomalies, the patient's presenting symptoms were very few and during the 11-year period the SDO had caused no complaints in our case. These findings show that the SOD course may be fairly benign. No neurological problem was encountered in the patient's follow-up, except headache. We believe that SOD should be kept in mind because of its rarity and the severity of its combined pathologies.

Ectodermal dysplasias

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Anhidrotic ectodermal dysplasia; Christ-Siemens-Touraine syndrome; Anondontia; Incontinentia pigmenti ... These resources can provide more information on ectodermal dysplasias: Ectodermal Dysplasia Society -- www.ectodermaldysplasia.org National Foundation for ...

Right Ventricular Thrombosis Combined With Fetal Death and Acrocyanosis in Pregnancy.

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Sabzi, Feridoun; Heidari, Aghigh

2017-07-01

Prepartum or postpartum right ventricular thrombosis (RVT) is an exceedingly rare and potentially lethal phenomenon in pregnancy. We here report a case of a pregnant patient with near term pregnancy admitted for dyspnea, amniotic fluids discharge and labor pain in a gynecology center that an eight-month dead fetus was diagnosed and delivered vaginally by induction. A post delivery period was complicated by aggravation of her dyspnea and pleuritic chest pain that she referred for further evaluation in our cardiac center. Physical exam revealed normal head and neck exam, and history taking revealed that her fetus had intra-uterine growth failure as reported by her gynecologist. Chest exam except to left lung crackle was normal. Lower and upper left extremities were normal. However, acrocyanosis was found in tips of 4 and 5th right-hand digits. Chest x-ray revealed some linear consolidation in left lower lung lobes, and the precordial exam was normal. ECG was normal. Post delivery transthoracic echocardiography (TEE) showed a 1.5×1.5 cm mobile right ventricular clot. C-T angiography revealed obstruction of left upper lung pulmonary artery branches. Complete thrombophilia assay showed the presence of high titer of antiphospholipid, anticardiolipin antibody, and β1 glycoprotein antibody. However, others test were normal. The patient was scheduled for cardiac surgery, and her hemodynamic was monitored by left radial artery line and central pressure venous line, and thrombus was removed from the right ventricle (RV), and subsequent anticoagulation therapy constituted. Six-month follow-up revealed no recurrence of thrombus and recovery of patient's symptoms.

Right Ventricular Thrombosis Combined With Fetal Death and Acrocyanosis in Pregnancy

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Feridoun Sabzi

2017-09-01

Full Text Available Prepartum or postpartum right ventricular thrombosis (RVT is an exceedingly rare and potentially lethal phenomenon in pregnancy. We here report a case of a pregnant patient with near term pregnancy admitted for dyspnea, amniotic fluids discharge and labor pain in a gynecology center that an eight-month dead fetus was diagnosed and delivered vaginally by induction. A post delivery period was complicated by aggravation of her dyspnea and pleuritic chest pain that she referred for further evaluation in our cardiac center. Physical exam revealed normal head and neck exam, and history taking revealed that her fetus had intra-uterine growth failure as reported by her gynecologist. Chest exam except to left lung crackle was normal. Lower and upper left extremities were normal. However, acrocyanosis was found in tips of 4 and 5th right-hand digits. Chest x-ray revealed some linear consolidation in left lower lung lobes, and the precordial exam was normal. ECG was normal. Post delivery transthoracic echocardiography (TEE showed a 1.5×1.5 cm mobile right ventricular clot. C-T angiography revealed obstruction of left upper lung pulmonary artery branches. Complete thrombophilia assay showed the presence of high titer of antiphospholipid, anticardiolipin antibody, and β1 glycoprotein antibody. However, others test were normal. The patient was scheduled for cardiac surgery, and her hemodynamic was monitored by left radial artery line and central pressure venous line, and thrombus was removed from the right ventricle (RV, and subsequent anticoagulation therapy constituted. Six-month follow-up revealed no recurrence of thrombus and recovery of patient’s symptoms.

Müllerian agenesis with hypohidrotic ectodermal dysplasia syndrome.

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Whaley, Katie; Winter, Jordan; Eyster, Kathleen M; Hansen, Keith A

2012-04-01

To describe the association of müllerian agenesis with hypohidrotic ectodermal dysplasia. Case report. University medical center. A 17-year-old woman with hypohidrotic ectodermal dysplasia referred for evaluation of primary amenorrhea. History, physical examination, and ultrasound. Physical findings of these two syndromes. Physical examination and ultrasound demonstrated müllerian agenesis with findings of hypohidrotic ectodermal dysplasia. This is the first description of the association of müllerian agenesis with ectodermal dysplasia. This rare case might provide further insight into the development of the uterus and the ectoderm as well as its derivatives. Copyright © 2012 American Society for Reproductive Medicine. Published by Elsevier Inc. All rights reserved.

Double Outlet Right Ventricle With Intact Ventricular Septum: Avulsion or Exclusion.

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Menon, Sabarinath; Kumar, C J Ashok; Mathew, Thomas; Venkateshwarn, S; Jayakumar, K; Dharan, Baiju S

2016-03-01

Double outlet right ventricle (DORV) is almost always associated with a ventricular septal defect. The variant of DORV with intact ventricular septum is very uncommon and may be associated with fetal demise or death immediately after birth. Reports of successful palliation of these patients to the stage of superior cavopulmonary anastomosis (bidirectional Glenn shunt) are rare. We describe the case of a child with DORV with intact ventricular septum who underwent successful palliation. This condition often provides a diagnostic, interventional, and surgical challenge. Patients generally require either balloon atrial septostomy or surgical atrial septectomy for survival, with the addition of a Blalock-Tausig shunt or pulmonary artery band depending on the pulmonary blood flow. Very few patients survive to a Glenn procedure. For those who do survive, a decision must be made regarding the management of the hypertensive and hypoplastic left ventricle (LV) and associated mitral regurgitation. The LV can be excluded by either mitral valve avulsion or closure (exclusion) of the mitral valve with a patch. The choice of the technique should be carefully made and depends in part on the size of the LV. In our patient, who was successfully palliated by bidirectional Glenn shunt, the LV was managed by means of mitral valve avulsion. © The Author(s) 2015.

Inflammatory Mediators Drive Adverse Right Ventricular Remodeling and Dysfunction and Serve as Potential Biomarkers

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Sydykov, Akylbek; Mamazhakypov, Argen; Petrovic, Aleksandar; Kosanovic, Djuro; Sarybaev, Akpay S.; Weissmann, Norbert; Ghofrani, Hossein A.; Schermuly, Ralph T.

2018-01-01

Adverse right ventricular (RV) remodeling leads to ventricular dysfunction and failure that represents an important determinant of outcome in patients with pulmonary hypertension (PH). Recent evidence indicates that inflammatory activation contributes to the pathogenesis of adverse RV remodeling and dysfunction. It has been shown that accumulation of inflammatory cells such as macrophages and mast cells in the right ventricle is associated with maladaptive RV remodeling. In addition, inhibition of inflammation in animal models of RV failure ameliorated RV structural and functional impairment. Furthermore, a number of circulating inflammatory mediators have been demonstrated to be associated with RV performance. This work reviews the role of inflammation in RV remodeling and dysfunction and discusses anti-inflammatory strategies that may attenuate adverse structural alterations while promoting improvement of RV function. PMID:29875701

Stenting of right ventricular outflow tract in Tetralogy of Fallot with subarterial ventricular septal defect: A word of caution

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Jonathan Lee

2017-01-01

Full Text Available We report a case of Tetralogy of Fallot with severe cyanosis who underwent a successful right ventricular outflow tract stenting. Follow-up echocardiography revealed moderate aortic regurgitation due to the impingement of the stent on the aortic valve. The patient underwent successful surgical correction at which time the stent was removed completely with a resolution of the aortic regurgitation.

Quantitation of right and left ventricular volume with MR imaging in patients with primary pulmonary hypertension

International Nuclear Information System (INIS)

Boxt, L.M.; Katz, J.; Kolb, T.; Czegledy, F.P.; Barst, R.J.

1990-01-01

This paper tests the utility of MR imaging in quantitating changes in ventricular volume and function in patients with primary pulmonary hypertension (PPH). Right ventricular (RV) and left ventricular (LV) end-diastolic (ED) and end-systolic (ES) volumes were determined in six patients with PPH and in eight controls. Short-axis images were obtained from the cardiac apex to the base at ED and ES, and the ventricular cavities were planimetered. Volumes were computed by summing the areas of the cavities times the thickness of the sections (12-14 mm). The intersection gap (1-3 mm) was averaged between adjacent sections. Results were indexed to the subject's body surface area. This technique was verified by comparison of results obtained by this method with the water displacement volumes of ventricular casts of eight excised bovine hearts and six water-filled balloons. Linear regression and the unpaired Students t test were used to test significance

Double-chambered right ventricle, ventricular septal defect, patent ductus arteriosus in a dog

Energy Technology Data Exchange (ETDEWEB)

Morozumi, M. [Togasaki Animal Hospital, Misato, Saitama (Japan); Kurosu, Y.; Kogure, K.; Chimura, S.; Shibata, S.; Kanemoto, I.

1989-12-15

A 4-month-old female mongrel puppy was presented with an anophthalmos. On physical examination, systolic murmur was heard at the 4th left intercostal space near the sternum. However the dog appeared healthy without cyanosis and had no history of exercise intolerance. The phonocardiogram revealed a pansystolic murmur and a continuous murmur on the mitral area. A systolic ejection murmur was also recorded on the pulmonic area. The electrocardiogram indicated bi-ventricular hypertrophy. Left ventricular enlargement was seen on chest radiographs. Ventricular septal defect (VSD) and patent ductus arteriosus (PDA) were diagnosed from these findings. PDA closure was performed at 2 years of age. After 2 months from the operation, the dog died during an attempted repair of the VSD. At necropsy, it was found that the double-chambered right ventricle (DCRV) was formed by an anomalous septal band. The VSD was localized on the proximal conus and was 8 mm in diameter. (author)

Double-chambered right ventricle, ventricular septal defect, patent ductus arteriosus in a dog

International Nuclear Information System (INIS)

Morozumi, M.; Kurosu, Y.; Kogure, K.; Chimura, S.; Shibata, S.; Kanemoto, I.

1989-01-01

A 4-month-old female mongrel puppy was presented with an anophthalmos. On physical examination, systolic murmur was heard at the 4th left intercostal space near the sternum. However the dog appeared healthy without cyanosis and had no history of exercise intolerance. The phonocardiogram revealed a pansystolic murmur and a continuous murmur on the mitral area. A systolic ejection murmur was also recorded on the pulmonic area. The electrocardiogram indicated bi-ventricular hypertrophy. Left ventricular enlargement was seen on chest radiographs. Ventricular septal defect (VSD) and patent ductus arteriosus (PDA) were diagnosed from these findings. PDA closure was performed at 2 years of age. After 2 months from the operation, the dog died during an attempted repair of the VSD. At necropsy, it was found that the double-chambered right ventricle (DCRV) was formed by an anomalous septal band. The VSD was localized on the proximal conus and was 8 mm in diameter. (author)

Right ventricular volume determination by two-dimensional echocardiography and radiography in model hearts using a subtraction method

International Nuclear Information System (INIS)

Krebs, W.; Erbel, R.; Schweizer, P.; Richter, H.A.; Massberg, I.; Meyer, J.; Effert, S.; Henn, G.

1982-01-01

The irregularity and complexity of the right ventricle is the reason why no accurate method for right ventricular volume determination exists. A new method for right ventricular volume determination particularly for two-dimensional echocardiography was developed - it is called subtraction method - and was compared with the pyramid and Simpson's methods. The partial volume of the left ventricle and septum was subtracted from total volume of right and left ventricle including interventricular septum. Thus right ventricular volume resulted. Total and partial volume were computer-assisted calculated by use of biplane methods, preferably Simpson's rule. The method was proved with thinwall silicon-rubber model hearts of the left and right ventricle. Two orthogonal planes in the long-axis were filmed by radiography or scanned in a water bath by two-dimensional echocardiography equivalent to RAO and LAO-projections of cineangiocardiograms or to four- and two-chamber views of apical two-dimensional echocardiograms. For calculation of the major axes of the elliptical sections, summed up by Simpson's rule, they were derived from the LAO-projection and the four-chamber view, respectively, the minor axis approximated from the RAO-projection and the two-chamber view. For comparison of direct-measured volume and two-dimensional echocardiographically determined volume, regression equation was given by y = 1.01 x - 3.2, correlation-coefficient, r = 0.977, and standard error of estimate (SEE) +-10.5 ml. For radiography, regression equation was y = 0.909 x + 13.3, r = 0.983, SEE = +-8.0 ml. For pyramid method and Simpson's rule, higher standard errors and lower correlation coefficients were found. Between radiography and two-dimensional echocardiography a mean difference of 4.3 +- 13.2 ml, using subtraction method, and -10.2 +- 22.9 ml, using pyramid method, as well as -0.6 +- 18.5 ml, using Simpson's rule, were calculated for right ventricular volume measurements. (orig./APR) [de

The Prevalence, Correlates, and Impact on Cardiac Mortality of Right Ventricular Dysfunction in Nonischemic Cardiomyopathy.

Science.gov (United States)

Pueschner, Andreas; Chattranukulchai, Pairoj; Heitner, John F; Shah, Dipan J; Hayes, Brenda; Rehwald, Wolfgang; Parker, Michele A; Kim, Han W; Judd, Robert M; Kim, Raymond J; Klem, Igor

2017-10-01

This study sought to determine the prevalence, correlates, and impact on cardiac mortality of right ventricular (RV) dysfunction in nonischemic cardiomyopathy. Current heart failure guidelines place little emphasis on RV assessment due to limited available data on determinants of RV function, mechanisms leading to its failure, and relation to outcomes. We prospectively studied 423 patients with cardiac magnetic resonance (CMR). The pre-specified study endpoint was cardiac mortality. In 100 patients, right heart catheterization was performed as clinically indicated. During a median follow-up time of 6.2 years (interquartile range: 2.9 to 7.6 years), 101 patients (24%) died of cardiac causes. CMR right ventricular ejection fraction (RVEF) was a strong independent predictor of cardiac mortality after adjustment for age, heart failure-functional class, blood pressure, heart rate, serum sodium, serum creatinine, myocardial scar, and left ventricular ejection fraction (LVEF). Patients with the lowest quintile of RVEF had a nearly 5-fold higher cardiac mortality risk than did patients with the highest quintile (hazard ratio: 4.68; 95% confidence interval [CI]: 2.43 to 9.02; p right atrial pressure (r = -0.32; p = 0.001), pulmonary artery pressure (r = -0.34; p = 0.0005), transpulmonary gradient (r = -0.28; p = 0.006) but not with pulmonary wedge pressure (r = -0.15; p = 0.13). In multivariable logistic regression analysis of CMR, clinical, and hemodynamic data the strongest predictors of right ventricular dysfunction were LVEF (odds ratio [OR]: 0.85; 95% CI: 0.78 to 0.92; p Right ventricular dysfunction is strongly associated with both indices of intrinsic myocardial contractility and increased afterload from pulmonary vascular dysfunction. Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

One-and-a-half ventricular repair for isolated right ventricle metastatic tumor resection after lobectomy for lung cancer.

Science.gov (United States)

Shiose, Akira; Desai, Parag; Criner, Gerard J; Pai, Sheela; Steiner, Robert M; Kaiser, Larry R; Guy, T Sloane; Toyoda, Yoshiya

2014-01-01

A 77-year-old woman presented with shortness of breath 1 year after a right upper lobectomy for lung cancer. She showed a possible intracardiac metastasis on positron emission tomography scan. There was no other evidence of recurrence. The large right ventricular mass was associated with the right ventricle free wall, the apex, the papillary muscle, and the chordae to the tricuspid valve. After mass resection of the right ventricle, a one-and-a-half ventricular repair was performed with tricuspid valve replacement and defect closure. The patient was discharged on postoperative day 14 without complications and has been well for the first 3 months after the surgery.

Effect of right ventricular pacing lead site on left ventricular function in patients with high-grade atrioventricular block: results of the Protect-Pace study.

Science.gov (United States)

Kaye, Gerald C; Linker, Nicholas J; Marwick, Thomas H; Pollock, Lucy; Graham, Laura; Pouliot, Erika; Poloniecki, Jan; Gammage, Michael

2015-04-07

Chronic right ventricle (RV) apical (RVA) pacing is standard treatment for an atrioventricular (AV) block but may be deleterious to left ventricle (LV) systolic function. Previous clinical studies of non-apical pacing have produced conflicting results. The aim of this randomized, prospective, international, multicentre trial was to compare change in LV ejection fraction (LVEF) between right ventricular apical and high septal (RVHS) pacing over a 2-year study period. We randomized 240 patients (age 74 ± 11 years, 67% male) with a high-grade AV block requiring >90% ventricular pacing and preserved baseline LVEF >50%, to receive pacing at the RVA (n = 120) or RVHS (n = 120). At 2 years, LVEF decreased in both the RVA (57 ± 9 to 55 ± 9%, P = 0.047) and the RVHS groups (56 ± 10 to 54 ± 10%, P = 0.0003). However, there was no significant difference in intra-patient change in LVEF between confirmed RVA (n = 85) and RVHS (n = 83) lead position (P = 0.43). There were no significant differences in heart failure hospitalization, mortality, the burden of atrial fibrillation, or plasma brain natriutetic peptide levels between the two groups. A significantly greater time was required to place the lead in the RVHS position (70 ± 25 vs. 56 ± 24 min, P function requiring a high percentage of ventricular pacing, RVHS pacing does not provide a protective effect on left ventricular function over RVA pacing in the first 2 years. ClinicalTrials.gov number NCT00461734. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2014. For permissions please email: journals.permissions@oup.com.

Prostacyclin therapy increases right ventricular capillarisation in a model for flow-associated pulmonary hypertension

NARCIS (Netherlands)

van Albada, Mirjam E.; Berger, Rolf M. F.; Niggebrugge, Marnix; van Veghel, Richard; Cromme-Dijkhuis, Adri H.; Schoemaker, Regien G.

2006-01-01

Pulmonary hypertension, and consequently right ventricular failure, complicates several congenital heart defects. Although intervention in the prostacyclin-thromboxane ratio is known to improve outcome, the underlying mechanism is not clear. Therefore, effects of acetyl salicylic acid and iloprost

Right and left ventricular ejection fraction at rest and during exercise assessed with radionuclide angiocardiography

International Nuclear Information System (INIS)

Dahlstroem, J.A.

1982-01-01

Right (RVEF) and left ventricular ejection fraction (LVEF) assessed with radionuclide angiocardiography were compared to simultaneously obtained catheterization results at rest and during exercise in patients with pulmonary hypertension and ischemic heart disease. Blood pool imaging was performed with red blood cells (RBC) labelled with 99 Tcsup(m) in vivo as this method gave more stable 99 Tcsup(m) levels in blood compared to 99 Tcsup(m)-labelled human serum albumin and because it was more convenient than labelling RBC in vitro. RVEF measured with first pass (FP) technique and equilibrium (EQ) technique correlated well at rest, r = 0.86, and during exercise, r = 0.91. The FP technique had the best reproducibility and reference values at rest were 49+-5 per cent increasing with exercise. When 99 Tcsup(m) and 133 Xe were compared to assess RVEF with FP technique, the correlation was good, r = 0.88. LVEF assessed with EQ technique and a fixed end-diastolic region of interest was very reproducible at rest and during exercise; reference values at rest were 56+-8 per cent increasing with exercise. In 10 patients with pulmonary hypertension significant negative correlations were found between RVEF assessed with FP technique and pressures in pulmonary artery and right atrium. Abnormal RVEF was found in all patients with right ventricular disfunction. In 22 patients with recent myocardial infarction measurements of LVEF detected left ventricular disfunction better than did measurement of pulmonary artery diastolic pressure. During effort angina in another 10 patients all had abnormal LVEF and abnormal hemodynamics. By combining ejection fraction and stroke volume, ventricular volumes were calculated at rest and during exercise. (author)

Phenotyping of left and right ventricular function in mouse models of compensated hypertrophy and heart failure with cardiac MRI

NARCIS (Netherlands)

Nierop, van B.J.; Assen, van H.C.; Deel, van E.D.; Niesen, L.B.P.; Duncker, D.J.; Strijkers, G.J.; Nicolay, K.

2013-01-01

Background: Left ventricular (LV) and right ventricular (RV) function have an important impact on symptom occurrence, disease progression and exercise tolerance in pressure overload-induced heart failure, but particularly RV functional changes are not well described in the relevant aortic banding

Pre-chemotherapy values for left and right ventricular volumes and ejection fraction by gated tomographic radionuclide angiography using a cadmium-zinc-telluride detector gamma camera

DEFF Research Database (Denmark)

Haarmark, Christian; Haase, Christine; Jensen, Maria Maj

2016-01-01

age and both left and right ventricular volumes in women (r = -0.4, P right end systolic ventricular volume in men (r = -0.3, P = .001). CONCLUSION: A set of reference values for cardiac evaluation prior to chemotherapy in cancer patients without other known cardiopulmonary......BACKGROUND: Estimation of left ventricular ejection fraction (LVEF) using equilibrium radionuclide angiography is an established method for assessment of left ventricular function. The purpose of this study was to establish normative data on left and right ventricular volumes and ejection fraction......, using cadmium-zinc-telluride SPECT camera. METHODS AND RESULTS: From routine assessments of left ventricular function in 1172 patients, we included 463 subjects (194 men and 269 women) without diabetes, previous potentially cardiotoxic chemotherapy, known cardiovascular or pulmonary disease. The lower...

Histiocytoid cardiomyopathy and ventricular noncompaction presenting as sudden death in an adult male.

Science.gov (United States)

Val-Bernal, J Fernando; Mayorga, Marta; Ortega, Clara; Linares, Emma

2017-11-01

Histiocytoid/oncocytic cardiomyopathy (HCM) is a rare, distinctive arrhythmogenic disorder that presents as arrhythmia or sudden death in infants and children. Ventricular noncompaction (VNC) is a rare cardiomyopathy characterized by a thickened endocardial layer of noncompacted myocardium and a thin epicardial layer of compacted myocardium. Only six cases of the association of both cardiomyopathies have been reported previously in the literature. All these cases were in children. To the best of our knowledge, a case of HCM has not been described in the adult. We report the case of a 45-year-old man with an increased heart weight and involvement of both ventricles by HCM and VNC cardiomyopathy. Besides, multiple foci of myocardial disorganization were detected. He died suddenly while hiking. The association of both processes HCM and VNC was an unexpected finding at autopsy. The death was linked to functional abnormalities of the cardiac histiocytoid cells, and it was favored by a state of abnormal development of the heart. Copyright © 2017 Elsevier GmbH. All rights reserved.

New population-based exome data are questioning the pathogenicity of previously cardiomyopathy-associated genetic variants

DEFF Research Database (Denmark)

Andreasen, Charlotte Hartig; Nielsen, Jonas B; Refsgaard, Lena

2013-01-01

Cardiomyopathies are a heterogeneous group of diseases with various etiologies. We focused on three genetically determined cardiomyopathies: hypertrophic (HCM), dilated (DCM), and arrhythmogenic right ventricular cardiomyopathy (ARVC). Eighty-four genes have so far been associated with these card......Cardiomyopathies are a heterogeneous group of diseases with various etiologies. We focused on three genetically determined cardiomyopathies: hypertrophic (HCM), dilated (DCM), and arrhythmogenic right ventricular cardiomyopathy (ARVC). Eighty-four genes have so far been associated...... with these cardiomyopathies, but the disease-causing effect of reported variants is often dubious. In order to identify possible false-positive variants, we investigated the prevalence of previously reported cardiomyopathy-associated variants in recently published exome data. We searched for reported missense and nonsense...... variants in the NHLBI-Go Exome Sequencing Project (ESP) containing exome data from 6500 individuals. In ESP, we identified 94 variants out of 687 (14%) variants previously associated with HCM, 58 out of 337 (17%) variants associated with DCM, and 38 variants out of 209 (18%) associated with ARVC...

Cardiothoracic ratio may be misleading in the assessment of right- and left-ventricular size in patients with repaired tetralogy of Fallot

International Nuclear Information System (INIS)

Śpiewak, M.; Małek, Ł.A.; Biernacka, E.K.; Kowalski, M.; Michałowska, I.; Hoffman, P.; Miśko, J.; Demkow, M.; Rużyłło, W.; Marczak, M.

2014-01-01

Aim: To assess the relationship between cardiothoracic ratio (CTR) and ventricular and atrial volumes in patients with repaired tetralogy of Fallot (TOF). Materials and methods: Patients with repaired TOF undergoing cardiac magnetic resonance (CMR) and chest radiography within 1 day were included (n = 82; median age: 24.7 years, interquartile range: 21.5–35.9). The CTR was obtained from upright posteroanterior chest roentgenograms. Analyses of CMR images and radiographs were performed in a blinded fashion. Results: There were 35.1% (13/37) of patients with normal CTR (<0.5) who had severe right ventricular (RV) dilatation. There were six patients (13.3%, 6/45) with high CTR with both normal RV and left-ventricular (LV) volumes. CTR did not correlate with either RV or LV volumes but showed a weak correlation with right- and left-atrial volumes (r = 0.43, p = 0.0001; r = 0.27, p = 0.01, respectively). CTR ≥0.5 showed poor ability in the identification of severe RV dilatation (sensitivity: 61.8%, specificity: 50%). The combination of CTR and signs of RV enlargement on lateral radiographs did not improve the diagnostic accuracy of any of those parameters alone. Conclusion: CTR in patients with repaired TOF reflected atrial rather than ventricular dilatation. The use of CTR or lateral radiographs in patients with repaired TOF may lead to false conclusions concerning ventricular size. - Highlights: • Cardiothoracic ratio did not correlate with either right- or left-ventricular volumes. • Cardiothoracic ratio reflected atrial rather than ventricular dilatation. • Normal cardiothoracic ratio did not exclude severe right ventricular dilatation. • Neither RVEF nor LVEF showed correlation with cardiothoracic ratio

Phenotyping of left and right ventricular function in mouse models of compensated hypertrophy and heart failure with cardiac MRI

NARCIS (Netherlands)

van Nierop, Bastiaan J.; van Assen, Hans C.; van Deel, Elza D.; Niesen, Leonie B. P.; Duncker, Dirk J.; Strijkers, Gustav J.; Nicolay, Klaas

2013-01-01

Left ventricular (LV) and right ventricular (RV) function have an important impact on symptom occurrence, disease progression and exercise tolerance in pressure overload-induced heart failure, but particularly RV functional changes are not well described in the relevant aortic banding mouse model.

Effect of Losartan on Right Ventricular Dysfunction: Results From the Double-Blind, Randomized REDEFINE Trial (Right Ventricular Dysfunction in Tetralogy of Fallot: Inhibition of the Renin-Angiotensin-Aldosterone System) in Adults With Repaired Tetralogy of Fallot.

Science.gov (United States)

Bokma, Jouke P; Winter, Michiel M; van Dijk, Arie P; Vliegen, Hubert W; van Melle, Joost P; Meijboom, Folkert J; Post, Martijn C; Berbee, Jacqueline K; Boekholdt, S Matthijs; Groenink, Maarten; Zwinderman, Aeilko H; Mulder, Barbara J M; Bouma, Berto J

2018-04-03

The effect of angiotensin II receptor blockers on right ventricular (RV) function is still unknown. Angiotensin II receptor blockers are beneficial in patients with acquired left ventricular dysfunction, and recent findings have suggested a favorable effect in symptomatic patients with systemic RV dysfunction. The current study aimed to determine the effect of losartan, an angiotensin II receptor blocker, on subpulmonary RV dysfunction in adults after repaired tetralogy of Fallot. The REDEFINE trial (Right Ventricular Dysfunction in Tetralogy of Fallot: Inhibition of the Renin-Angiotensin-Aldosterone System) is an investigator-initiated, multicenter, prospective, 1:1 randomized, double-blind, placebo-controlled study. Adults with repaired tetralogy of Fallot and RV dysfunction (RV ejection fraction [EF] 0.30 for all). In predefined subgroup analyses, losartan did not have a statistically significant impact on RV EF in subgroups with symptoms, restrictive RV, RV EFtetralogy of Fallot. Future larger studies may determine whether there might be a role for losartan in specific vulnerable subgroups. URL: https://www.clinicaltrials.gov. Unique identifier: NCT02010905. © 2017 American Heart Association, Inc.