scholarly journals Pulmonary Hypertension and the Right Ventricle—Thinking outside the Box (Third International Right Heart Failure Summit, Part 1)

2014 ◽  
Vol 4 (4) ◽  
pp. 696-704 ◽  
Author(s):  
Jonathan D. Rich
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Right Ventricle ◽  
Right Heart Failure ◽  
Right Heart ◽  
The Right ◽  
Outside The Box

scholarly journals Potts Shunt in Children With Familial Primary Pulmonary Hypertension: A Case Report and Brief Literature Review

2021 ◽  
Vol 9 (3) ◽  
pp. 247-254
Author(s):  
Mahya Mobinikhaledi ◽  
◽  
Ali Arjmand Shabestari ◽  
Yazdan Ghandi ◽  
◽  
...  
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Right Ventricle ◽  
Arterial Hypertension ◽  
Medical Treatment ◽  
Right Heart Failure ◽  
Right Heart ◽  
Pulmonary Arterial ◽  
The Right

Introduction: The primary concern about Familial Pulmonary Arterial Hypertension (FPAH) is the development of right heart failure, which ultimately leads to sudden death. Despite recent advances in pharmacological therapy, the mortality rate in children is still high, emphasizing the importance of novel treatments. Moreover, there is still no definitive treatment for children with severe pulmonary hypertension. The consequences of right heart failure led to the proposal of a surgical approach, the Potts shunt, to decompress the right ventricle, providing shunting of deoxygenated blood to lower extremities and improving overall cardiac output. This surgical technique creates an anastomosis between the left pulmonary artery and the descending aorta, providing a palliative treatment to off-load the right ventricle. Case Presentation: We report an 11-year-old girl with familial pulmonary arterial hypertension and right ventricular failure who benefited from a Potts shunt with good mid-term results. The patient was diagnosed at the age of 5 when she had a history of fainting and persistent syncope. The patient was under medical treatment therapy with bosentan, sildenafil, acetylsalicylic acid, and warfarin for six years. The results of molecular genetic testing, which was confirmed with direct sequencing of the Bone Morphogenetic Protein Receptor Type 2 (BMPR2) gene, revealed a heterozygous pathogenic mutation. Since she was diagnosed with Pulmonary Arterial Hypertension (PAH), she lost her grandmother, an aunt, and father because of PAH. Her 14-year-old sister also had mutated the BMPR2 gene without developing FPAH. Conclusions: The Potts shunt provides an interventional step for palliation of patients with familial pulmonary hypertension and severe right heart failure refractory to medical treatment. It opens the door to the possibility of lung transplantation in the future. We did not see any complications within 6 years after placing the Potts shunt.

scholarly journals Metastatic adenocarcinoma involving the right ventricle and pulmonary artery leading to right heart failure: case report

2017 ◽  
Vol 136 (3) ◽  
pp. 262-265 ◽  
Author(s):  
Turgut Karabag ◽  
Caner Arslan ◽  
Turab Yakisan ◽  
Aziz Vatan ◽  
Duygu Sak
Keyword(s):  
Heart Failure ◽  
Case Report ◽  
Pulmonary Artery ◽  
Right Ventricle ◽  
Right Ventricular ◽  
Right Ventricular Outflow Tract ◽  
Right Heart Failure ◽  
Ventricular Outflow Tract ◽  
Right Heart ◽  
The Right

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.

The Hemodynamic Characteristics of Right Ventricle After Gradient Pulmonary Artery Banding in Rats

2020 ◽  
Author(s):  
Song Jiyang ◽  
Wan Nan ◽  
Shen Shutong ◽  
Wei Ying ◽  
Cao Yunshan
Keyword(s):  
Heart Failure ◽  
Pulmonary Artery ◽  
Right Ventricle ◽  
Right Ventricular ◽  
Right Heart Failure ◽  
Therapeutic Strategies ◽  
Pulmonary Artery Banding ◽  
Right Heart ◽  
Pulmonary Arterial ◽  
The Right

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.

scholarly journals Pathophysiology of the right ventricle and of the pulmonary circulation in pulmonary hypertension: an update

2019 ◽  
Vol 53 (1) ◽  
pp. 1801900 ◽  
Author(s):  
Anton Vonk Noordegraaf ◽  
Kelly Marie Chin ◽  
François Haddad ◽  
Paul M. Hassoun ◽  
Anna R. Hemnes ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Right Ventricle ◽  
Vascular System ◽  
Right Heart Failure ◽  
Imaging Data ◽  
Right Heart ◽  
Future Directions ◽  
Right Heart Catheterisation ◽  
The Right ◽  
Key Pathways

The function of the right ventricle determines the fate of patients with pulmonary hypertension. Since right heart failure is the consequence of increased afterload, a full physiological description of the cardiopulmonary unit consisting of both the right ventricle and pulmonary vascular system is required to interpret clinical data correctly. Here, we provide such a description of the unit and its components, including the functional interactions between the right ventricle and its load. This physiological description is used to provide a framework for the interpretation of right heart catheterisation data as well as imaging data of the right ventricle obtained by echocardiography or magnetic resonance imaging. Finally, an update is provided on the latest insights in the pathobiology of right ventricular failure, including key pathways of molecular adaptation of the pressure overloaded right ventricle. Based on these outcomes, future directions for research are proposed.

Tricuspid valve relocation with endomyocardial fibrosis removal for Löeffler’s endocarditis

2019 ◽  
Vol 56 (3) ◽  
pp. 622-624
Author(s):  
Tohru Asai ◽  
Fumihiro Miyashita ◽  
Hiromitsu Nota ◽  
Piers N Vigers
Keyword(s):  
Heart Failure ◽  
Right Ventricle ◽  
Right Atrium ◽  
Hypereosinophilic Syndrome ◽  
Right Heart Failure ◽  
Right Atrial ◽  
Endomyocardial Fibrosis ◽  
Right Heart ◽  
Atrial Enlargement ◽  
The Right

Abstract Löffler endocarditis with hypereosinophilic syndrome is rare but can cause critical ventricular obliteration by endomyocardial fibrosis. A 52-year-old woman experienced severe right heart failure with extreme shrinkage of her right ventricle, severe tricuspid regurgitation and marked right atrial enlargement. Preoperative tests showed identical pressures in the right atrium and pulmonary artery. Endocardial stripping was done, and to enlarge the right ventricle, we relocated the anterior and posterior tricuspid leaflets cephalad, up the right atrium wall, to ‘ventricularize’ a portion of the right atrium, with autologous pericardial augmentation of the tricuspid leaflets. An annuloplasty ring was added to reinforce the relocated tricuspid attachment. Right heart pressures normalized postoperatively. The patient recovered uneventfully. She has received corticosteroid therapy continuously and has shown no recurrence of heart failure in the 5 years since surgery.

P5370Pulmonary pressure overload stimulates cardiac stem or progenitor cell-derived cardiac regeneration in the right ventricular area

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M Kanda ◽  
T Nagai ◽  
N Kondou ◽  
K Tateno ◽  
M Hirose ◽  
...  
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Right Ventricular ◽  
Progenitor Cell ◽  
Pressure Overload ◽  
Right Heart Failure ◽  
Left Ventricular ◽  
Free Wall ◽  
Right Heart ◽  
The Right

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

scholarly journals Jobb kamrai adaptáció pulmonalis artériás hypertoniában

2021 ◽  
Vol 162 (37) ◽  
pp. 1485-1493
Author(s):  
Györgyi Csósza ◽  
Zsófia Lázár ◽  
Zsolt Rozgonyi ◽  
Hajnalka Vágó ◽  
György Losonczy ◽  
...  
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Right Ventricular Dysfunction ◽  
Right Heart Failure ◽  
Progressive Increase ◽  
Right Heart ◽  
Cardiac Adaptation ◽  
The Face ◽  
Pulmonary Arterial ◽  
The Right

Összefoglaló. Pulmonalis artériás hypertoniában (PAH) a tüdőartériák falának átépülése az elsődleges patofiziológiai eltérés, amely a pulmonalis vascularis rezisztencia (PVR) és a pulmonalis nyomás progresszív emelkedéséhez vezet. Ez a nyomásemelkedés a jobb szívfélben az afterload fokozódásához vezet, ami hosszú távon jobbkamra-diszfunkciót és jobbszívfél-elégtelenséget okoz. Az egyre növekvő PVR mellett kialakuló cardialis adaptáció pontos patomechanizmusa nem ismert, de egyes betegek esetén nagyon eltérő lehet az adaptáció mértéke és kialakulásának üteme. A kialakuló myocardium-hypertrophia és -dilatáció mértéke nagyban függ a PAH etiológiájától, de emellett egyéb tényezők – mint az életkor, a neurohumoralis aktiváció mértéke, genetikai és epigenetikai faktorok – is jelentősen befolyásolják. Minél kevésbé képes a jobb kamra megtartani funkcióját az egyre növekvő ellenállással szemben, annál gyorsabban alakul ki a jobbszívfél-elégtelenség, és annál rosszabbak a beteg életkilátásai. Ezen folyamatok jobb megismerése klinikai jelentőséggel bír, mivel a jobb kamrai adaptáció elősegítése javíthatja a betegség kimenetelét. Orv Hetil. 2021; 162(37): 1485–1493. Summary. Remodeling of the pulmonary artery wall is the primary pathophysiological abnormality in pulmonary arterial hypertension leading to a progressive increase in pulmonary vascular resistance (PVR) and pulmonary arterial pressure. The elevation of pressure increases the afterload in the right heart, causing right ventricular dysfunction and right heart failure in the long term. The exact pathomechanism of cardiac adaptation with increasing PVR is unknown, but the degree and rate of adaptation may be very different in patients suffering from pulmonary hypertension. The development of myocardial hypertrophy and dilatation is highly dependent on the etiology of pulmonary hypertension, but is also significantly influenced by other factors such as age, degree of neurohumoral activation, and genetic and epigenetic factors. Right heart failure develops and life expectancy shortens if the right ventricle is unable to maintain its function in the face of increasing resistance. Orv Hetil. 2021; 162(37): 1485–1493.

scholarly journals P1242 Giant right atrium aneurysm presenting as right heart failure

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
F V Moniz Mendonca ◽  
J A S Sousa ◽  
J M Monteiro ◽  
M R Mraquel ◽  
M N Neto ◽  
...  
Keyword(s):  
Heart Failure ◽  
Atrial Fibrillation ◽  
Right Ventricle ◽  
Right Atrium ◽  
Adult Life ◽  
Right Heart Failure ◽  
Outflow Tract ◽  
Right Heart ◽  
History Of ◽  
The Right

Abstract Introduction Idiopathic aneurysmal dilatations of the right atrium are rare anomalies. It can be diagnosed at any time between foetal and adult life. This exceptional condition can be confused with other conditions that involve enlargement of right atrium. We report a clinical case of a symptomatic adult who was diagnosed with giant right atrium aneurysm. Case report An 83-year-old female presented with complaints of fatigue, paroxysmal nocturnal dyspnoea, exertional dyspnoea, orthopnoea and cough since last week. There were no history of syncope, convulsions or evidence of thromboembolism. There were a medical history of diverticulosis and atrial fibrillation (warfarin therapy). The principal findings on physical examination included holosystolic murmur at the left middle sternal border, pulmonary rales, jugular venous distension, enlarged liver and peripheral oedema. An electrocardiogram showed an atrial fibrillation with a controlled heart rate response, right axis deviation, right bundle-branch block. A chest radiography posteroanterior view showed a markedly enlarged cardiac silhouette, increased pulmonary vascular congestion, and bilateral pleural effusions. Computed tomography (CT) scan showed aneurysmal dilated right atrium communicating with right ventricle. Right ventricle (RV) and RV outflow tract were dilated with normal pulmonary arteries. Two-dimensional transthoracic echocardiography revealed aneurysmal dilated right atrium measuring 398mL/m2. The tricuspid valve was no displaced. There was severe tricuspid regurgitation and no stenosis. The right atrium was kinetic without any intracavitary thrombus. The intertrial and interventricular septa were intact. The right ventricle and outflow tract were mildly dilated with preserved systolic function. The left atrium and left ventricle were normal. The patient was admitted to the cardiology department with the diagnosis of right heart failure. Conclusion Aneurysm of right atrium is an uncommon condition. It is diagnosed as a disproportionately enlarged right atrium compared to the other cardiac chambers in the absence of other cardiac or hemodynamic abnormalities and must be distinguished from other anomalies causing structural pathology of the right atrium. Approximately, one-half of the patients have no symptoms. Others presented with arrhythmia, palpitations, chest pain, shortness of breath, and fatigue. The major rhythm abnormality is atrial fibrillation or atrial flutter. Our patient presented with symptoms of right heart failure and atrial fibrillation. The right enlargement is usually associated with tricuspid annular dilatation responsible for functional regurgitation, which can be severe in some cases. The diagnosis of right atrium malformation can be established by echocardiography, CT or magnetic resonance imaging. Literature reports various ways to manage these patients. Treatment ranges from conservative to surgical resection specially in the presence of arrhythmias. Abstract P1242 Figure. Aneurysm of right atrium

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