Case Report: Nonarrhythmogenic Right Ventricular Dysplasia Presenting with Severe Right Ventricular Failure in an Adolescent

2010 ◽  
Vol 13 (1) ◽  
pp. 21
Author(s):  
Mustafa Pac ◽  
Aysenur Pac ◽  
Tugcin Bora Polat ◽  
Sevket Balli ◽  
Nesrin Turhan ◽  
...  

Right ventricular dysplasia is usually discovered by the presence of ventricular arrhythmia. As arrhythmia is an epiphenomenon, the first presentation of some cases can be primarily heart failure. We describe an adolescent girl who presented with progressive right heart failure and whose hallmark was fibrofatty replacement of ventricular muscle, especially of the right side, without ventricular arrhythmia. The patient was successfully treated by orthotopic heart transplantation.

2014 ◽  
Vol 26 (1) ◽  
pp. 155-157
Author(s):  
Eri Mineo ◽  
Takashi Honda ◽  
Masahiro Ishii

AbstractThe introduction of foetoscopic laser photocoagulation has dramatically improved the prognosis of patients with severe twin-to-twin transfusion syndrome. We present the case of a donor who exhibited right-heart failure with a high echoic wall change of the right ventricle after the foetoscopic laser photocoagulation procedure. The prenatal and 1-year postnatal follow-up revealed the gradual recovery of the right ventricular function.


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.


Perfusion ◽  
2017 ◽  
Vol 32 (5) ◽  
pp. 413-415 ◽  
Author(s):  
Saurabh Chiwane ◽  
Tageldin M. Ahmed ◽  
Christian P. Bauerfeld ◽  
Monika Chauhan

Introduction: Neonates are at risk of developing hypernatremic dehydration and its associated complications, such as stroke, dural sinus thrombosis and renal vein thrombosis. Pulmonary hypertension has not been described as a complication of hypernatremia. Case report: We report a case of a seven-day-old neonate with severe hypernatremic dehydration who went on to develop pulmonary hypertension and right heart failure needing extracorporeal membrane oxygenation (ECMO). Discussion: Normal or high anion gap metabolic acidosis commonly accompanies hypernatremic dehydration. The presence of acidosis and/or hypoxia can delay the normal drop in pulmonary vascular resistance (PVR) after birth, causing pulmonary hypertension and right ventricular failure. Conclusion: A high index of suspicion is paramount to diagnose pulmonary hypertension and aggressive correction of the acidosis and hypoxia is needed. In the presence of severe right ventricular failure, ECMO can be used as a bridge to recovery while underlying metabolic derangements are being corrected.


2017 ◽  
Vol 136 (3) ◽  
pp. 262-265 ◽  
Author(s):  
Turgut Karabag ◽  
Caner Arslan ◽  
Turab Yakisan ◽  
Aziz Vatan ◽  
Duygu Sak

ABSTRACT CONTEXT: Obstruction of the right ventricular outflow tract due to metastatic disease is rare. Clinical recognition of cardiac metastatic tumors is rare and continues to present a diagnostic and therapeutic challenge. CASE REPORT: We present the case of a patient who had severe respiratory insufficiency and whose clinical examinations revealed a giant tumor mass extending from the right ventricle to the pulmonary artery. We discuss the diagnostic and therapeutic options. CONCLUSION: In patients presenting with acute right heart failure, right ventricular masses should be kept in mind. Transthoracic echocardiography appears to be the most easily available, noninvasive, cost-effective and useful technique in making the differential diagnosis.


2020 ◽  
Author(s):  
Song Jiyang ◽  
Wan Nan ◽  
Shen Shutong ◽  
Wei Ying ◽  
Cao Yunshan

Abstract Background: Right ventricular (RV) failure induced by sustained pressure overload is a major contributor to morbidity and mortality in several cardiopulmonary disorders. Reliable and reproducible animal models of RV failure are important in order to investigate disease mechanisms and effects of potential therapeutic strategies. To establish a rat model of RV failure perfectly, we observed the right ventricle and carotid artery hemodynamics characteristics in different degrees of pulmonary artery banding of rats of different body weights. Methods: Rats were subjected to 6 groups:control(0%, n=5)(pulmonary arterial banding 0%), PAB(1-30%, n=4)(pulmonary arterial banding1-30%), PAB(31-60%, n=6)(pulmonary arterial banding31-60%),PAB(61-70%, n=5)(pulmonary arterial bandin61-70%), PAB(71-80%,n=4)(pulmonary arterial banding71-80%), PAB(100%, n=3)(pulmonary arterial banding 100%). We measured the right ventricular pressure(RVP) by right heart catheterization when the pulmonary arterial was ligated. Results: The RVP gradually increased with increasing degree of banding, but when occlusion level exceeding 70%, high pressure state can be only maintained for a few minutes or seconds, and then the RVP drops rapidly until it falls below the normal pressure, which in Group F particularly evident.Conclusions: RVP have different reactions when the occlusion level is not the same, and the extent of more than 70% ligation is a successful model of acute right heart failure. These results may have important consequences for therapeutic strategies to prevent acute right heart failure.


2017 ◽  
Vol 8 (4) ◽  
pp. 470-474 ◽  
Author(s):  
Jyoti Prasad Kalita ◽  
Nilajan Dutta ◽  
Neeraj Awasthy ◽  
Kuntal Roy Chowdhuri ◽  
Sumir Girotra ◽  
...  

Background: Uhl’s anomaly is an extremely rare congenital heart defect characterized by a near total absence of the myocardium of the parietal wall of the right ventricle. Few reports of surgical management exist in literature. We present three patients with this anomaly who were managed with different surgical strategies. Patients and Methods: Patient 1: This 43-month-old girl had maternal rubella syndrome with speech and hearing deficits and gross right heart failure. Diagnosis was made on echocardiography and magnetic resonance imaging. She underwent partial excision and plication of the right ventricular parietal wall and total cavopulmonary connection. Patient 2: This 19-month-old boy presented with progressive cyanosis and features of right heart failure. Diagnosis was confirmed on echocardiography. He underwent right ventricular exclusion by tricuspid valve closure and free wall plication followed by a bidirectional Glenn procedure. Patient 3: This 21-year-old male presented with right heart failure and cyanosis. Diagnosis was established with transesophageal echocardiography and magnetic resonance imaging. As the hemodynamics were not suitable for a Fontan conversion, a one and a half ventricle repair was done along with plication of the right ventricular free wall and tricuspid valve annuloplasty. Results: All three patients were extubated within 24 hours. Patients 2 and 3 were discharged relatively uneventfully, whereas patient 1 had a more prolonged stay due to transient hepatic failure. All were symptomatically and clinically improved on short-term follow-up. Conclusion: Uhl’s anomaly is an extremely rare condition with varied clinical presentation. Surgical exclusion of the right ventricle yields gratifying results; however, surgical technique has to be adapted to the individual patient.


2009 ◽  
Vol 17 (5) ◽  
pp. 525-530 ◽  
Author(s):  
Sayed Nour ◽  
Guifu Wu ◽  
Zheng Zhensheng ◽  
Juan C Chachques ◽  
Alain Carpentier ◽  
...  

Background: Right heart failure is a frequent hemodynamic disturbance in pediatric cardiac patients. Besides inotropic and chronotropic drugs, fluid administration and inhaled nitric oxide, right ventricular mechanical assistance remains difficult to perform. A circulatory assist device adapted for the right heart biophysics and physiology might be more efficient. Materials and Methods: We are developing a prototype of a non-invasive cardiac assist device (CAD) for neonates and pediatrics. It is based on a pulsatile suit device covering and affecting all territories of the right heart circuit. It will be tested in a neonatal animal model of right ventricular (RV) failure. Experimental models will be matched and compared with control and sham groups. Expected results would be immediate hemodynamic improvement due to synchronized diastolic reduction of stagnant venous capacitance, increasing preload and contractility. On long term, increased shear stress with changing intrathoracic pressure in a phasic way would improve and remodel the pulmonary circulation. Future studies will be focused on: hemodynamic, biochemistry, endothelium function test, and angiogenesis. Comments: A non-invasive CAD guarantees better hemodynamics and endothelial function preservation with low morbidity and mortality. This is a physiological approach, cost-effective method, and particularly interesting in neonates and pediatrics with RV failure.


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


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