An overlooked cause for reversible severe tricuspid regurgitation and pulmonary hypertension: Hibernating right ventricle

2021 ◽  
Author(s):  
Kadriye Memic Sancar ◽  
Cagdas Topel ◽  
Selahattin Turen ◽  
Mehmet Erturk ◽  
Gamze Babur Guler
2019 ◽  
Vol 23 ◽  
pp. 100239
Author(s):  
Hanna Jung ◽  
Joon Yong Cho ◽  
Gun-Jik Kim ◽  
Young ok Lee ◽  
Kyoung Hoon Lim ◽  
...  

2020 ◽  
Vol 30 (7) ◽  
pp. 1026-1028
Author(s):  
Emily N. Sanders ◽  
Dala Zakaria

AbstractEbstein anomaly is a rare CHD known for its wide spectrum of presentation with the age of diagnosis dependent on the malformation’s severity. Here, the authors describe a case of delayed diagnosis of Ebstein anomaly, secondary to lack of medical attention, which resulted in severe tricuspid regurgitation and pulmonary hypertension. Furthermore, the case was complicated by a unique pulmonary venous abnormality.


2019 ◽  
Vol 9 (4) ◽  
pp. 204589401989542 ◽  
Author(s):  
Umberto Annone ◽  
Pier P. Bocchino ◽  
Walter G. Marra ◽  
Fabrizio D’Ascenzo ◽  
Corrado Magnino ◽  
...  

Prognosis in pulmonary hypertension is strictly linked to right ventricle failure, which results from uncoupling between right ventricle function and its afterload. This study sought to describe how to estimate with echocardiography right ventricular wall tension, its correlation with right ventricle haemodynamics and its prognostic role. A total of 190 patients without overt right ventricle failure but with suspected pulmonary hypertension on a previous echocardiogram underwent right heart catheterization and nearly-simultaneous echocardiography. Right ventricular wall tension was estimated according to Laplace’s law as right ventricle length × tricuspid regurgitation peak gradient and it was correlated with right ventricle haemodynamic profile; its potential prognostic impact was tested along with canonical right ventricle function parameters. Right ventricular wall tension correlated significantly with invasive estimation of right ventricle end-diastolic pressure (R: 0.343, p < 0.001) and with several other haemodynamic variables, such as mean pulmonary artery pressure, pulmonary artery compliance, transpulmonary gradient, pulmonary vascular resistance, right atrial pressure and right ventricle stroke work index (all p < 0.001). At a mean follow-up of five years and three months, only right ventricular wall tension was associated to all-cause mortality ( p = 0.036), while tricuspid annular plane systolic excursion ( p = 0.536), right ventricle fractional area change ( p = 0.383), right ventricle fractional area change ( p = 0.076), tricuspid regurgitation peak gradient ( p = 0.107) and tricuspid annular plane systolic excursion/tricuspid regurgitation peak gradient ( p = 0.181) could not. We identified a novel bedside echocardiographic predictor of altered right ventricle haemodynamics, which is precociously altered in patients without overt right ventricle failure and is associated to all-cause mortality at a long-term follow-up. Further studies are needed to confirm its role in pulmonary hypertension patients.


Author(s):  
Purwoko Purwoko ◽  
Ardhana Surya Aji

<p>Ventricular Septal Defect (VSD) is a congenital heart disease that causes the connection between left and right ventricles called a Gerbode defect. Manifestation of a Gerbode defect is damage to the opening tricuspid valve caused regurgitation of the tricuspid valve. Delay in diagnosis and intervention will affect pre-operative nutritional status and malnutrition.</p><p>We reported a boy aged 2 months, weighing 3100 grams with biliary atresia followed by VSD, severe TR, and Gerbode defect who will undergo the Kasai procedure. Preoperative physical examination showed GCS E4V5M6, SpO2 100%. The skin gets icteric all over the body and conjunctiva. The cardiovascular system has a regular I-II heart sound, 2/3 mid clavicular S noise as high as 2 ICS and a pansystolic murmur. The examination of the abdomen is slight distended. Child pug score 8. Hemoglobin value 6.7gr%, hematocrite 37%, APTT 44.8 seconds, SGOT 443 U / L, SGPT 560 U / L, total bilirubin 23.89 mg / dl, direct bilirubin 13.92 mg / dl, and indirect bilirubin 9.97 mg / dl.</p><p>The goal of anesthesia in VSD, Severe Tricuspid Regurgitation (TR) with Gerbode Defect is preventing excessive ventilation to avoid severe pulmonary hypertension. The choice of anesthetic agent is based on the patient's physiology and balancing pulmonary and systemic blood flow. Perioperative management of cases of VSD, TR Severe with Gerbode defect in the following report describes the importance of understanding the pathophysiology of VSD and Gerbode defects to obtain a good outcome.</p><p>Perioperative management of VSD patients, severe tricuspid regurgitation with Gerbode defect requires more supervision, especially to minimize the increase in PVR, maintain systemic vascular resistance (SVR) and avoid excessive ventilation to prevent severe pulmonary hypertension.</p>


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M Gavazzoni ◽  
E V Vizzardi ◽  
A C Castiello ◽  
R R Raddino ◽  
L P B Badano ◽  
...  

Abstract Background/Introduction Speckle tracking echocardiography has been recently proposed as an accurate and sensitive measure of right ventricle (RV) function that could integrate other more conventional parameters. This tool can be important in the clinical context of severe tricuspid regurgitation (TR), since TAPSE is not fully representative of global RV function and can overestimate this in presence of severe TR. Purpose Evaluate the prognostic relevance of different parameters of RV structure and function derived from 2D and speckle tracking echocardiographic analysis of clinically stable patients with severe TR referred for routine follow up in the context of many etiologies of left side heart disease (secondary TR). Methods The present is a retrospective analysis of prospectively acquired echocardiographic studies including patients with severe secondary TR in the context of left side heart disease. Fractional area change (FAC), tricuspid annular plane systolic excursion (TAPSE), RV global longitudinal strain (RVLS) and RV free-wall longitudinal strain (RVFWLS) as well as LV function were measured. As suggested in previous studies, we also aimed to explored the use in this population of: i)RVLS/pulmonary systolic arterial pressure (PASP); ii) RVFWLS (average lateral 3 segments strain)/IVSLS (average medial 3 segment strain) as index of RV-LV dependency. The composite end-point of this study included death for any cause and heart failure hospitalization. Results 61 patients (mean age 58±20 years, 65% men), were included. After a mean follow up period of 3,6±2 years 57% of patients reached the combined end-point. At Cox regression univariate analysis a significant correlation with outcomes was found for RVend-diastolic diameter (HR 0,42, p: 0.018), right atrial area (HR: 3, p: 0.02), RVFWLS/IVSLS (HR: 0.5, p: 0.020), RVLS/PASP (HR 0.186, p: 0.039). In multivariable Cox-regression model we found that LVEF, RV dimension and RVFWLS/IVSLS were independently related to outcome; this last one parameter showed the best correlation with outcomes. Conclusions In asymptomatic and clinically stable patients with severe secondary TR longitudinal function of RV free wall is not related to outcomes but RV-arterial coupling and the ratio between deformation of free wall and septal wall of RV are good predictors of clinical deterioration at follow up. The last one conceptually represents the interaction between RV and LV in secondary TR and allows a real “correction” of those effects of severity of TR on the base to apex gradient of lateral wall longitudinal deformation (TR increases movement of basal segments).


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Muhammad Soofi ◽  
Swapnil Garg ◽  
Srikanth Sadhu

Background: Adult congenital heart disease is most commonly complicated by arrhythmias and very rarely by acute coronary syndromes. We present a rare case of ST elevation myocardial infarction (STEMI) in a young 35-year-old patient with congenital transposition of great vessels. Case Presentation: A 35-year-old female with a past medical history of transposition of the great vessels (d-TGA) status post arterial baffle operation in infancy, with prior atrial arrhythmias and severe RV failure, presented to our facility with crushing substernal chest pain radiating down her left arm. ECG was significant for ST elevation in the inferior leads and high sensitivity troponin was elevated. She was taking Rivaroxaban. She was taken emergently to the catheterization lab where she was found to have 100% occlusion of the apical left anterior descending artery secondary to an embolus with no angiographic disease in any other epicardial vessel. Transthoracic echocardiography showed severe dilation of the right ventricle with severe tricuspid regurgitation. The embolic lesion was deemed not amenable to percutaneous intervention and the patient was treated conservatively with a heparin drip. The embolus was presumed to originate from the hyper-trabeculated and distended right ventricle in spite of chronic rivaroxaban therapy and she was started on warfarin. Presently she is stable, awaiting a heart transplant. Discussion: Extensive literature review revealed four reported cases of acute coronary syndrome secondary to emboli in patients with surgically corrected TGA. Patients with d-TGA are most often surgically corrected with an arterial switch procedure. But in Baffle procedures (Mustard or Senning) such as the one in this patient, the right ventricle becomes the systemic ventricle. The most common complications as an adult are right ventricular dilation, severe tricuspid regurgitation and atrial arrhythmias, all three of which were noted in our patient. Conclusion: Our patient developed all three of the most common adult complications of surgically corrected d-TGA, ultimately resulting in the rare and extreme manifestation of an embolic STEMI.


2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
M Fontes Oliveira ◽  
MI Oliveira ◽  
R Costa ◽  
A Dias Frias ◽  
I Silveira ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Although the perceived prognosis of patients with precapillary pulmonary hypertension (PH) is poor, the natural history of this condition is very heterogeneous. In this study we sought to identify predictors of poor outcomes which could help refine prognosis. Methods We studied consecutive patients referred to our centre from 12/2016 to 11/2018 with confirmed precapillary PH. A range of clinical, laboratory, echocardiographic and right heart catheterization (RHC) data variables were collected to assess predictors of survival. Outcome was defined as mortality from any cause. Results Of the 80 included patients, 51 (64%) were female and mean age was 60.5 ± 16.0 years. The majority of patients (45%) had pulmonary arterial hypertension (group 1) and 41% were chronic thromboembolic pulmonary hypertensive disease patients (group 4). During a median follow-up of 18.7 [IQR 12.3 – 26.7] months, 10 patients (12.5%) died. New York Heart Association (NYHA) functional class (HR 19.4 [95% CI 2.56 - 147.5], p = 0.004) was the strongest predictor of mortality, whereas higher haemoglobin (HR 0.70 [0.49 - 0.99], p= 0.047) and 6-minute walking distance (6MWD) expressed as percentage of predicted (HR 0.96 [0.93 - 0.99], p = 0.004) were associated with better survival overall. Echocardiographic parameters such as eccentricity index (HR 3.35 (95% CI 1.11 - 10.0), p = 0.031), short pulmonary acceleration time (HR 0.98 [95% CI 0.96 - 0.99], p = 0.008), the presence of moderate to severe tricuspid regurgitation (HR 6.46 [95% CI 1.67 - 25.0], p = 0.007) and pericardial effusion (HR 3.86 [95% CI 1.12 - 13.4], p = 0.033) were also associated with death. Traditional right ventricular function parameters such as fractional area change, tricuspid annular plane systolic excursion (TAPSE) and S velocity of the lateral annular tricuspid annulus did not predict mortality in these patients. Invasive pressures and pulmonary vascular resistance measured by RHC were also not associated with mortality. In multivariable analysis, NYHA functional class was the only independent predictor of mortality in patients with precapillary PH (HR 14.5 [95% CI 2.3 - 146.8], p = 0.006). Conclusion Eccentricity index, short pulmonary acceleration time, moderate to severe tricuspid regurgitation and pericardial effusion were associated with poor survival. Functional class was the strongest independent predictor of mortality in precapillary PH patients. These parameters may help stratify the risk of death in this heterogenous population.


2020 ◽  
Vol 30 (8) ◽  
pp. 1183-1185 ◽  
Author(s):  
Alina Z. Naqvi ◽  
Susan M. Lanni ◽  
Joanna B. Rosenthal

AbstractConstriction of the fetal ductus arteriosus is rare and usually attributed to medications or CHD. We describe a 24-year-old multigravida at 33 weeks 5 days gestation with echocardiographic findings of severe ductal constriction, a dilated, hypertrophied and hypocontractile right ventricle, and severe tricuspid regurgitation following BC powder® use. Treatment with Digoxin and oxygen resulted in a progressive 71% reduction in peak systolic ductal gradient, improved right ventricular function, and decreased tricuspid regurgitation.


Author(s):  
Guillem Muntané-Carol ◽  
Maurizio Taramasso ◽  
Mizuki Miura ◽  
Mara Gavazzoni ◽  
Alberto Pozzoli ◽  
...  

Background: Scarce data exist on patients with right ventricular dysfunction (RVD) or pulmonary hypertension (PH) undergoing transcatheter tricuspid valve intervention. This study aimed to determine the early and midterm outcomes and the factors associated with mortality in this group of patients. Methods: This subanalysis of the multicenter TriValve (Transcatheter Tricuspid Valve Therapies) registry included 300 patients with severe tricuspid regurgitation with RVD (n=244), PH (n=127), or both (n=71) undergoing transcatheter tricuspid valve intervention. RVD was defined as a tricuspid annular plane systolic excursion <17 mm, and PH as an estimated pulmonary artery systolic pressure ≥50 mm Hg. Results: Mean age of the patients was 77±9 years (54% women). Procedural success was 80.7%, and 9 patients (3%) died during the hospitalization. At a median follow-up of 6 (interquartile range, 2–12) months, 54 patients (18%) died, and the independent associated factors were higher gamma-glutamyl transferase values at baseline (hazard ratio, 1.02 for each increase of 10 u/L [95% CI, 1.002–1.04]), poorer renal function defined as an estimated glomerular filtration rate <45 mL/min (hazard ratio, 2.3 [95% CI, 1.22–4.33]), and the lack of procedural success (hazard ratio, 2.11 [95% CI, 1.17–3.81]). The grade of RVD and the amount of PH at baseline were not found to be predictors of mortality. Most patients alive at follow-up improved their functional class (New York Heart Association I–II in 66% versus 7% at baseline, P <0.001). Conclusions: In patients with severe tricuspid regurgitation and RVD/PH, transcatheter tricuspid valve intervention was associated with high procedural success and a relatively low in-hospital mortality, along with significant improvements in functional status. However, about 1 out of 5 patients died after a median follow-up of 6 months, with hepatic congestion, renal dysfunction, and the lack of procedural success determining an increased risk. These results may improve the clinical evaluation of transcatheter tricuspid valve intervention candidates and would suggest a closer follow-up in those at increased risk. Registration: URL: https://www.clinicaltrials.gov . Unique identifier: NCT03416166.


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