scholarly journals Arrhythmogenic right ventricular cardiomyopathy in a dog : case report

Author(s):  
A.J. Möhr ◽  
R.M. Kirberger

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.

2020 ◽  
Author(s):  
Jagjit Khosla ◽  
Reshma Golamari ◽  
Alice Cai ◽  
Jamal Benson ◽  
Wilbert S Aronow ◽  
...  

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic disorder resulting in fibrofatty replacement of the myocardium. Genetic mutations in genes encoding for desmosome proteins result in a ventricular myocardium prone to arrhythmias and heart failure. Although ARVC is known for a few decades, most of the outcomes in pregnancy are reported recently. Pregnancy leads to significant physiological changes with excess mechanical stress on the myocardium. All the retrospective studies suggest that pregnancy is well tolerated in these patients despite the high risk of arrhythmias and heart failure. Our review focuses on the most up-to-date evidence on the management of ARVC patients during the antepartum and postpartum period.


2019 ◽  
Vol 43 (1) ◽  
pp. 25-36 ◽  
Author(s):  
Koray Kalenderoğlu ◽  
Tolga Sinan Güvenç ◽  
Müge Taşdemir Mete ◽  
Hüseyin Kuplay ◽  
Semra Ağustos ◽  
...  

Background: Right ventricular stroke work index is a useful but invasively measured parameter that can be used to predict right heart failure following continuous-flow left ventricular assist device implantation. Right ventricular contraction pressure index is a novel parameter that was developed to measure right ventricular stroke work index with echocardiography. We aimed to investigate the clinical usefulness of right ventricular contraction pressure index to predict short-term mortality and right heart failure in patients who underwent continuous-flow left ventricular assist device implantation. Methods: A total of 49 patients who participated in institutional advanced heart failure registry and underwent continuous-flow left ventricular assist device implantation with a bridge-to-candidacy indication were analyzed retrospectively. Right ventricular contraction pressure index was calculated using offline measurements. Demographic, clinical and outcome data were obtained from the registry data. Patients were grouped according to right ventricular contraction pressure index quartiles. Results: Patients within the lowest right ventricular contraction pressure index quartile had a trend toward higher short-term mortality (46.2%, p = 0.056) and combined short-term mortality and definitive right heart failure (53.8%, p = 0.054) at 15th day postoperatively. Similarly, short-term survival or survival free of definite right heart failure were significantly lower in the lowest right ventricular contraction pressure index quartile (log-rank p = 0.045 and log-rank p = 0.03, respectively). In a proportional hazards model that included echocardiographic parameters, right ventricular contraction pressure index was an independent predictor for short-term mortality (odds ratio: 6.777, 95% confidence interval: 1.118–41.098, p = 0.037), but not for combined short-term mortality and definite right heart failure. No such associations were found for long-term mortality. Right ventricular contraction pressure index had a statistically significant correlation with invasively measured pulmonary capillary wedge pressure, pulmonary vascular resistance, mean pulmonary pressure, and right ventricular stroke work index. Conclusion: Right ventricular contraction pressure index was found as a useful parameter for determining short-term postoperative mortality in patients undergoing continuous-flow left ventricular assist device implantation.


1994 ◽  
Vol 17 (4) ◽  
pp. 224-229 ◽  
Author(s):  
Y. Abe ◽  
K. Kotoh ◽  
P.H. Deleuze ◽  
M. Miyama ◽  
G.J. Cooper ◽  
...  

Changes in the right ventricular function measured with a thermodilution ejection fraction catheter have been recorded in open-chest normal pigs and pigs with acute right heart failure (RVF) undergoing left ventricular assistance with a pneumatic-sactype device (LVAD). To produce acute right heart failure, 5 pigs underwent ligation of the right ventricular free wall coronary arteries. Compared with normal pigs, cardiac output in ligated pigs fell by 21% (7.5 ± 0.5 vs 9.5 ± 1.2 L/min; p < 0.05) and the right ventricular end diastolic pressure rose (11.4 ± 2.6 vs 5.7 ± 3.6 vs mmHg: p <0.05). With the left ventricular assist device connected, the right atrial pressure was increased to 3, 5, 7, 10 and 12 mmHg by volume loading while maintaining the haematocrit at 35 ± 6%. The right ventricular stroke work index (RVSWI) increased with volume loading in normal pigs. In RVF pigs, RVSWI increased significantly with the LVAD (59.2 ± 5.8 vs 23.5 ± 7.8 mmHg ml/min/kg, p<0.01), approaching that of normal pigs (62.3 ± 4.8 mmHg ml/min/kg). Similar changes were observed in the cardiac output and right ventricular stroke volume. These results show that, in this model of open-chest, mild, acute right heart failure, left ventricular assistance allows right ventricular function to return to normal, despite volume overloading, by decreasing right ventricular after load and increasing right ventricular compliance


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M Kanda ◽  
T Nagai ◽  
N Kondou ◽  
K Tateno ◽  
M Hirose ◽  
...  

Abstract Introduction and purpose The number of patients with right heart failure due to pulmonary hypertension has been increasing. Although several drugs have reportedly improved pulmonary hypertension, no treatments have been established for decompensated right heart failure. The heart has an innate ability to regenerate, and cardiac stem or progenitor cells (e.g., side population [SP] cells) have been reported to contribute to the regeneration process. However, their contribution to right ventricular pressure overload has not been clarified. Here, this regeneration process was evaluated using a genetic fate-mapping model. Methods and results We used Cre-LacZ mice, in which more than 99.9% of the cardiomyocytes in the left ventricular field were positive for 5-bromo-4-chloro-3-indolyl-β-D-galactoside (X-gal) staining immediately after tamoxifen injection. Then, we performed either a pulmonary binding (PAB) or sham operation on the main pulmonary tract. In the PAB-treated mice, the right ventricular cavity was significantly enlarged (right-to-left ventricular [RV/LV] ratio, 0.24±0.04 in the sham group and 0.68±0.04 in the PAB group). Increased peak flow velocity in the PAB group (1021±80 vs 1351±62 mm/sec) was confirmed by echocardiography. One month after the PAB, the PAB-treated mice had more X-gal-negative (newly generated) cells than the sham mice (94.8±34.2 cells/mm2 vs 23.1±10.5 cells/mm2; p<0.01). The regeneration was biased in the RV free wall (RV free wall, 225.5±198.7 cells/mm2; septal area, 88.9±56.5/mm2; LV lateral area, 46.8±22.0/mm2; p<0.05). To examine the direct effects of PAB on the cardiac progenitor cells, bromodeoxyuridine was administered to the mice daily until 1 week after the PAB operation. Then, the hearts were isolated and SP cells were harvested. The SP cell population increased from 0.65±0.23% in the sham mice to 1.87% ± 1.18% in the PAB-treated mice. Immunostaining analysis revealed a significant increase in the number of BrdU-positive SP cells, from 11.6±2.0% to 44.0±18%, therefore showing SP cell proliferation. Conclusions Pulmonary pressure overload stimulated cardiac stem or progenitor cell-derived regeneration with a RV bias, and SP cell proliferation may partially contribute to this process. Acknowledgement/Funding JSPS KAKENHI Grant Number JP 17K17636, GSK Japan Research Grant 2016


2001 ◽  
Vol 280 (1) ◽  
pp. H11-H16 ◽  
Author(s):  
Yuji Ishibashi ◽  
Judith C. Rembert ◽  
Blase A. Carabello ◽  
Shintaro Nemoto ◽  
Masayoshi Hamawaki ◽  
...  

Severe left ventricular volume overloading causes myocardial and cellular contractile dysfunction. Whether this is also true for severe right ventricular volume overloading was unknown. We therefore created severe tricuspid regurgitation percutaneously in seven dogs and then observed them for 3.5–4.0 yr. All five surviving operated dogs had severe tricuspid regurgitation and right heart failure, including massive ascites, but they did not have left heart failure. Right ventricular cardiocytes were isolated from these and from normal dogs, and sarcomere mechanics were assessed via laser diffraction. Right ventricular cardiocytes from the tricuspid regurgitation dogs were 20% longer than control cells, but neither the extent (0.171 ± 0.005 μm) nor the velocity (2.92 ± 0.12 μm/s) of sarcomere shortening differed from controls (0.179 ± 0.005 μm and 3.09 ± 0.11 μm/s, respectively). Thus, despite massive tricuspid regurgitation causing overt right heart failure, intrinsic right ventricular contractile function was normal. This finding for the severely volume-overloaded right ventricle stands in distinct contrast to our finding for the left ventricle severely volume overloaded by mitral regurgitation, wherein intrinsic contractile function is depressed.


2019 ◽  
Author(s):  
Amalio Ruiz-Salas ◽  
Isabel Navarro-Arce ◽  
Carmen Medina-Palomo ◽  
Alberto Barrera-Cordero ◽  
Manuel Jiménez-Navarro ◽  
...  

Abstract Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC/D) is an inherited cardiomyopathy characterized by ventricular arrhythmias and heart failure. The aim of our study was to analyze the impact of the ICD indication in the prognosis of patients with high-risk ARVC/D according to the consensus document. Methods The high-risk category includes patients who experienced cardiac arrest due to sustained ventricular tachycardia or ventricular fibrillation and patients with severe right or left ventricular dysfunction. We included 41 patients with high-risk ARVC/D: 33 in secondary prevention and 8 in primary prevention. Results We followed 41 patients during 6.37 ± 4.88 years. Twenty-six patients (63.4%) had at least one appropriate arrhythmic event: 24 p (72.7%) in secondary prevention and 2 p (25%) in primary prevention; p=0.02. Twenty-four patients (72.7%) in secondary prevention and five (62.5%) in primary prevention had a cardiovascular event such as arrhythmias, admission due to heart failure, heart transplantation or cardiovascular death. Conclusions High-risk ARVC/D patients had a high number of cardiovascular events, but their nature and treatment were different. Arrhythmic prognosis was worse in secondary prevention and most of the events found in primary prevention were related to heart failure and, therefore, without benefit of the ICD.


2019 ◽  
Vol 12 ◽  
pp. 117954761982871
Author(s):  
Kentaro Yamamoto ◽  
Xin Guo ◽  
Ken-ichi Mizutani ◽  
Nozomu Kurose ◽  
Motona Kumagai ◽  
...  

We presented an unusual arrhythmogenic right ventricular cardiomyopathy (ARVC) case of a late-60s elderly man’s death, due to severe pericardial/pleural effusion and ascites, and arrhythmic events, with unique pathological features. The hypertrophic heart grossly displayed yellowish to yellow-whitish predominantly in the variably thinned wall of the dilated right ventricle. Microscopic findings showed diffuse fatty/fibrofatty replacement in not only the right but left ventricular myocardium, together with an outer lymphoplasmacytic infiltrate. According to the lipid contents analysis, the triglyceride content, but not the cholesterol content, in our patient’s right and left ventricular cardiac muscle was much higher than that in the control subject. We propose that this unique triglyceride deposition in our possibly late-onset ARVC case might be one of new clues to understand its enigmatic cause. Further prospective studies are needed to validate the presence and significance of a greater volume of triglyceride deposit, after collecting and investigating a larger number of early and late-onset ARVC cases examined.


Author(s):  
Carolina Shalini Singarayar ◽  
Foo Siew Hui ◽  
Nicholas Cheong ◽  
Goay Swee En

Summary Thyrotoxicosis is associated with cardiac dysfunction; more commonly, left ventricular dysfunction. However, in recent years, there have been more cases reported on right ventricular dysfunction, often associated with pulmonary hypertension in patients with thyrotoxicosis. Three cases of thyrotoxicosis associated with right ventricular dysfunction were presented. A total of 25 other cases of thyrotoxicosis associated with right ventricular dysfunction published from 1994 to 2017 were reviewed along with the present 3 cases. The mean age was 45 years. Most (82%) of the cases were newly diagnosed thyrotoxicosis. There was a preponderance of female gender (71%) and Graves’ disease (86%) as the underlying aetiology. Common presenting features included dyspnoea, fatigue and ankle oedema. Atrial fibrillation was reported in 50% of the cases. The echocardiography for almost all cases revealed dilated right atrial and or ventricular chambers with elevated pulmonary artery pressure. The abnormal echocardiographic parameters were resolved in most cases after rendering the patients euthyroid. Right ventricular dysfunction and pulmonary hypertension are not well-recognized complications of thyrotoxicosis. They are life-threatening conditions that can be reversed with early recognition and treatment of thyrotoxicosis. Signs and symptoms of right ventricular dysfunction should be sought in all patients with newly diagnosed thyrotoxicosis, and prompt restoration of euthyroidism is warranted in affected patients before the development of overt right heart failure. Learning points: Thyrotoxicosis is associated with right ventricular dysfunction and pulmonary hypertension apart from left ventricular dysfunction described in typical thyrotoxic cardiomyopathy. Symptoms and signs of right ventricular dysfunction and pulmonary hypertension should be sought in all patients with newly diagnosed thyrotoxicosis. Thyrotoxicosis should be considered in all cases of right ventricular dysfunction or pulmonary hypertension not readily explained by other causes. Prompt restoration of euthyroidism is warranted in patients with thyrotoxicosis complicated by right ventricular dysfunction with or without pulmonary hypertension to allow timely resolution of the abnormal cardiac parameters before development of overt right heart failure.


2018 ◽  
Vol 54 (1) ◽  
pp. 75
Author(s):  
Fajar Perdhana ◽  
Herdono Purnomo

Right ventricular dysfunction and failure receive much less attention than the left ventricular failure. Right ventricular dysfunction or failure is associated with increased mortality rates in cardiac surgery, surgical cases other than cardiac surgery and also in patients treated in the ICU. The purpose of this article review was to describe the anatomy, physiology and pathophysiology of right ventricular failure, its detection and diagnosis, and management considerations from anesthetic point of view, including preoperative, intraoperative and postoperative stages. Cardiac surgery may result in right ventricular failure. For example, 0.1% post cardiotomy patients experience severe right heart failure and require long-term inotropic support, and so do 2-3% of post-transplant patients, and 20-30% of patients installed with instrument in his left heart. Therefore, anesthesiologists play a major role in perioperative and postoperative intensive care and are obliged to comprehend the nature of right ventricular dysfunction and failure so as to carry out early detection, prevent and manage patients with right ventricular dysfunction.


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