Ebstein anomaly combined with unique pulmonary venous abnormality in a 9-month-old child

2020 ◽  
Vol 30 (7) ◽  
pp. 1026-1028
Author(s):  
Emily N. Sanders ◽  
Dala Zakaria
Keyword(s):  
Pulmonary Hypertension ◽  
Tricuspid Regurgitation ◽  
Wide Spectrum ◽  
Delayed Diagnosis ◽  
Medical Attention ◽  
Ebstein Anomaly ◽  
Severe Tricuspid Regurgitation ◽  
Age Of Diagnosis

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.

scholarly journals Perioperative Management of Patients with Ventricular Septal Defect, Severe Tricuspid Regurgitation and Gerbode Defects

2021 ◽  
Vol 1 (1) ◽  
pp. 27
Author(s):  
Purwoko Purwoko ◽  
Ardhana Surya Aji
Keyword(s):  
Pulmonary Hypertension ◽  
Ventricular Septal Defect ◽  
Tricuspid Valve ◽  
Tricuspid Regurgitation ◽  
Perioperative Management ◽  
Septal Defect ◽  
The Body ◽  
Severe Pulmonary Hypertension ◽  
Severe Tricuspid Regurgitation ◽  
Gerbode Defect

<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>

Predictors of survival in patients with precapillary pulmonary hypertension

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
M Fontes Oliveira ◽  
MI Oliveira ◽  
R Costa ◽  
A Dias Frias ◽  
I Silveira ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pericardial Effusion ◽  
Tricuspid Regurgitation ◽  
Independent Predictor ◽  
Functional Class ◽  
Walking Distance ◽  
Severe Tricuspid Regurgitation ◽  
Acceleration Time ◽  
Nyha Functional Class ◽  
Eccentricity Index

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.

scholarly journals Transcatheter Tricuspid Valve Intervention in Patients With Right Ventricular Dysfunction or Pulmonary Hypertension

2021 ◽  
Vol 14 (2) ◽  
Author(s):  
Guillem Muntané-Carol ◽  
Maurizio Taramasso ◽  
Mizuki Miura ◽  
Mara Gavazzoni ◽  
Alberto Pozzoli ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Tricuspid Valve ◽  
Tricuspid Regurgitation ◽  
Ventricular Dysfunction ◽  
Right Ventricular Dysfunction ◽  
Hazard Ratio ◽  
Severe Tricuspid Regurgitation ◽  
Increased Risk ◽  
Procedural Success

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.

scholarly journals Haemodynamic determinants of improved aerobic capacity after tricuspid valve surgery in Ebstein anomaly

Heart ◽  
2020 ◽  
pp. heartjnl-2020-317756
Author(s):  
Alexander Egbe ◽  
William Miranda ◽  
Heidi Connolly ◽  
Joseph Dearani
Keyword(s):  
Relative Risk ◽  
Tricuspid Valve ◽  
Aerobic Capacity ◽  
Tricuspid Regurgitation ◽  
Valve Surgery ◽  
Ebstein Anomaly ◽  
Severe Tricuspid Regurgitation ◽  
Tricuspid Valve Surgery ◽  
Postoperative Improvement

BackgroundAlthough tricuspid valve surgery improves functional capacity in patients with Ebstein anomaly, it is not always associated with improvement in aerobic capacity. The purpose of this study was to identify the determinants of improved aerobic capacity after tricuspid valve surgery in adults with Ebstein anomaly with severe tricuspid regurgitation.MethodsRetrospective study of patients with severe tricuspid regurgitation due to Ebstein anomaly that had tricuspid valve surgery at Mayo Clinic Rochester (2000–2019) and had preoperative and postoperative cardiopulmonary exercise tests and echocardiograms. The patients were divided into aerobic capacity(+) and aerobic capacity(-) groups depending on whether they had postoperative improvement in %-predicted peak oxygen consumption (VO2).ResultsOf 76 patients with severe tricuspid regurgitation due to Ebstein anomaly, 28 (37%) and 48 (63%) were in aerobic capacity(+) and aerobic capacity(-) groups, respectively. The average improvement in peak VO2 was 2.1±1.4 mL/kg/min and −0.9±0.4 mL/kg/min in the in aerobic capacity(+) and aerobic capacity(-) groups, respectively. Although both groups had similar severity of residual tricuspid regurgitation, the aerobic capacity(+) group had more postoperative improvement in right atrial (RA) function, left atrial (LA) function and left ventricular preload and stroke volume. Of the preoperative variables analysed, RA reservoir strain (relative risk 1.12; 95% CI 1.06 to 1.18); LA reservoir strain (relative risk 1.09; 95% CI 1.04 to 1.14) and LV stroke volume index (OR 1.04; 95% CI 1.01 to 1.07) were predictors of postoperative improvement in peak VO2.ConclusionsOne-third of patients with severe tricuspid regurgitation due to Ebstein anomaly had postoperative improvement in aerobic capacity, and atrial function indices were the best predictors of postoperative improvement in aerobic capacity. These data provide new insight into the haemodynamic determinants of exercise capacity and lay the foundation for further studies to determine whether postoperative improvement in aerobic capacity translates to improved long-term survival, and whether timing of tricuspid valve surgery based on these echocardiographic indices will improve long-term outcomes.

P5567Clinical characteristics, diagnosis and risk stratification of pulmonary hypertension in severe tricuspid regurgitation and implications for transcatheter tricuspid valve repair

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
P Lurz ◽  
K P Rommel ◽  
M Orban ◽  
C Besler ◽  
D Braun ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Tricuspid Valve ◽  
Tricuspid Regurgitation ◽  
Clinical Status ◽  
Tricuspid Valve Repair ◽  
Systolic Pulmonary Artery Pressure ◽  
Clinical Endpoint ◽  
Valve Repair ◽  
Severe Tricuspid Regurgitation ◽  
Regurgitant Orifice Area

Abstract Objective To assess the role of pulmonary hypertension (PHT) in severe tricuspid regurgitation (TR) and its implications for transcatheter tricuspid valve repair (TTVR). Background PHT patients are often excluded from surgical TR therapies. TTVR with the MitraClipTM technique is a novel treatment option for these patients. Methods A total of 164 patients at high surgical risk (median age 78 years) and TR underwent TTVR at two centers. Seventy patients were grouped as iPHT+, defined as invasive systolic pulmonary artery pressure (PAPs) >50 mmHg. Patients were similarly stratified according to echocardiographic PAPs (ePHT). The occurrence of the combined clinical endpoint (death, heart failure hospitalization, reintervention) was investigated. Results iPHT+ patients were at higher pre-operative risk (p<0.01), had more severe symptoms (p=0.01), higher NT-pro-BNP levels (p<0.01) and more impaired biventricular function (left: p=0.03; right: p=0.02). Procedural TTVR success was achieved in 86 vs. 82% in iPHT+ and iPHT- patients respectively (p=0.52). Tricuspid valve effective regurgitant orifice area (EROA) was reduced from 0.49 cm2 to 0.20 cm2 (p<0.01) similarly in both groups. While iPHT+ conveyed risk (HR 1.7 (95% CI 1.1–2.8), p=0.03) for the occurrence of the clinical endpoint, ePHT+ paradoxically conveyed protection (HR 0.61 (95% CI 0.36–0.98), p=0.04). This discrepancy was explained by the highest event rates in patients with iPHT+/ePHT- (n=28). Conversely, iPHT+/ePHT+ patients had comparable outcomes to iPHT- patients. Conclusions PHT in TR is associated with worse clinical status and advanced HF, but not procedural failure. Symptomatic benefit can be achieved irrespective of PHT status by TTVR. Although echocardiographic PHT diagnosis is unreliable, the combination of echocardiographic and invasive assessment may identify ideal candidates for TTVR among PHT patients.

scholarly journals Partial PFO Closure for Persistent Hypoxemia in a Patient with Ebstein Anomaly

2015 ◽  
Vol 2015 ◽  
pp. 1-3 ◽  
Author(s):  
S. A. Zuberi ◽  
S. Liu ◽  
J. W. Tam ◽  
F. Hussain ◽  
D. Maguire ◽  
...  
Keyword(s):  
Tricuspid Valve ◽  
Tricuspid Regurgitation ◽  
Patent Foramen Ovale ◽  
Foramen Ovale ◽  
Atrial Septal Defect Closure ◽  
Ebstein Anomaly ◽  
Severe Tricuspid Regurgitation ◽  
Tricuspid Valve Surgery ◽  
Acute Decompensation ◽  
The Right

Ebstein anomaly is characterized by deformities of the anterior leaflet of the tricuspid valve and atrialization of the right ventricle. Patients with severe tricuspid regurgitation are recommended to have tricuspid valve surgery with concomitant atrial septal defect closure. A 73-year-old female with Ebstein anomaly presented with severe hypoxemia. Transthoracic echocardiography revealed severe tricuspid regurgitation and a patent foramen ovale with right-to-left shunting. Complete percutaneous patent foramen ovale closure led to acute decompensation; however, partial closure led to hemodynamic stability and improved oxygenation. In conclusion, similar patients with “patent foramen ovale dependency” from longstanding shunts may benefit from partial patent foramen ovale closure.

Increased Risk of Possible Paradoxical Embolic Events in Adults with Ebstein Anomaly and Severe Tricuspid Regurgitation

2013 ◽  
Vol 9 (1) ◽  
pp. 30-37 ◽  
Author(s):  
Christine H. Attenhofer Jost ◽  
Heidi M. Connolly ◽  
Christopher G. Scott ◽  
Harold M. Burkhart ◽  
Naser M. Ammash ◽  
...  
Keyword(s):  
Tricuspid Regurgitation ◽  
Ebstein Anomaly ◽  
Severe Tricuspid Regurgitation ◽  
Increased Risk
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