Staged Fontan procedure for mitral atresia associated with severe tricuspid regurgitation, pulmonary hypertension, and pulmonary artery distortion

Abstract Introduction Tricuspid valve duplication is an extremely rare condition and in most of the cases it is associated with other congenital cardiac malformations. Because of its rarity, the clinical presentation and the management are not defined yet. Clinical Case We report the case of an 18 y/o caucasian male, who was admitted to our Hospital in February 2018 for rapid atrial flutter not responsive to medical therapy (propanolol and digossin). He had a pre-natal diagnose of ventricular septum defect (VSD) and tricuspid straddling. At 1 year of age he underwent pulmonary artery bandage and one year later VSD closure was performed. Blood test showed sub-clinic hypothyroidism, probably related to previous amiodaron therapy. A transthoracic echocardiogram was obtained. The right atrium (RA) was severely dilated and the atrial septum dislocated towards left ventricle (LV); two right atrioventricular valves (tricuspid valves) were detected: the ‘true’ tricuspid opening was inside the right ventricle, and an ‘accessory‘ opening was located inside the LV and severely regurgitant into the RA; the mitral valve was morphologically and functionally normal; both ventricles were dilated with preserved systolic function; systolic pulmonary artery pressure was not detectable. A Cardiac Magnetic Resonance clearly delineated the anomaly. Atrial flutter radio frequency transcatheter ablation was succesfully performed before corrective surgery. The regurgitant accessory tricuspid orifice was closed with an heterologous pericardial patch and a right reduction atrioplasty was also done. The post-operative course was uneventful and only a mild paraseptal tricuspid jet with LV to RA shunt was present at post op echocardiography. After one year follow-up the patient remained asymptomatic, without arrhythmia recurrence. Conclusion DOTV is an extremely rare condition that could be responsible of severe tricuspid regurgitation. At the moment, there are not sufficient data to establish the correct timing for surgical intervention. In our case, the presence of severe tricuspid regurgitation, right atrium dilatation, biventricular overload and atrial flutter guided the clinical management and suggested surgical correction. Abstract P189 Figure.

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

Cardiology in the Young ◽

10.1017/s1047951120001249 ◽

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.

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Diastolic Forward Flow in the Pulmonary Artery in a Patient with Severe Tricuspid Regurgitation - Relationship to Absence of the Carvallo Sign

American Journal of Noninvasive Cardiology ◽

10.1159/000470190 ◽

1994 ◽

Vol 8 (3) ◽

pp. 179-182

Author(s):

Kazuhiro Mori ◽

Suguru Matuoka ◽

Katuyoshi Tatara ◽

Yasunobu Hayabuchi ◽

Yasuhiro Kuroda

Keyword(s):

Pulmonary Artery ◽

Tricuspid Regurgitation ◽

Severe Tricuspid Regurgitation ◽

Forward Flow

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Elevated Estimated Pulmonary Arterial Pressures in Patients with Chuvash Polycythemia.

Blood ◽

10.1182/blood.v104.11.1634.1634 ◽

2004 ◽

Vol 104 (11) ◽

pp. 1634-1634

Author(s):

Victor R. Gordeuk ◽

Adelina I. Sergueeva ◽

Galina Y. Miasnikova ◽

Lydia A. Polyakova ◽

Daniel J. Okhotin ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Pulmonary Artery ◽

Tricuspid Regurgitation ◽

Chronic Hypoxia ◽

Primary Pulmonary Hypertension ◽

Systolic Pressure ◽

Pulmonary Artery Systolic Pressure ◽

Normal Range ◽

Pulmonary Arterial ◽

The Mean

Abstract Chuvash polycythemia is characterized by a homozygous 598C>T mutation in VHL and up regulation of HIF-1α during normoxia. Disorders of chronic hypoxia may be complicated by the development of pulmonary hypertension. Because of the up regulation of the hypoxic response in Chuvash polycythemia, we postulated that there may be a tendency to increased pulmonary artery pressures in this condition as well. To test this hypothesis, we analyzed results for Doppler echocardiography in 15 patients with Chuvash polycythemia and 15 Chuvash individuals without polycythemia. The tricuspid regurgitation velocity (TRV) allows estimation of pulmonary artery systolic pressure. A TRV of 2.5 m/sec or higher corresponds to a pulmonary artery systolic pressure of at least 35 mm Hg (normal up to 32 mm Hg), while a TRV of 3.0 m/sec or higher to a pressure of at least 46 mm Hg. The results are summarized in the Table. Pulmonary artery pressures as estimated by tricuspid regurgitation velocity (TRV) in Chuvash subjects with and without polycythemia Chuvash polycythemia (n = 15) Controls (n = 15) P Age in years; mean (SD) 35 (17) 35 (17) 1.0 Female sex in no. (%) 8 (53%) 8 (53%) 1.0 Hemoglobin in g/dL; mean (SD) 16.7 (2.3) 13.3 (1.2) <0.001 TRV in m/sec; mean (SD) 2.2 (0.6) 1.4 (0.6) 0.001 TRV > 2.4 m/sec in no. (%) 4 (27%) 0 (0%) 0.1 Most of the patients with Chuvash polycythemia were receiving phlebotomy therapy and therefore many had hemoglobin concentrations in the upper normal range. Four of the patients with Chuvash polycythemia and none of the others had TRV ≥ 2.5 m/sec (range of 2.5 to 3.0), and mean TRVs were significantly higher in the patients with Chuvash polycythemia. Interestingly, the mean ± SD TRV in these 15 patients with Chuvash polycythemia was identical to the mean ± SD TRV that was recently reported in 195 American patients with sickle cell disease (Gladwin et al, NEJM2004;350:886), another hematological condition with a tendency to pulmonary hypertension. While the pulmonary arterial pressures detected so far in Chuvash polycythemia patients are lower than those in patients with primary pulmonary hypertension, our results suggest that pulmonary hypertension may be an unrecognized complication of Chuvash polycythemia.

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

Solo Journal of Anesthesi, Pain and Critical Care (SOJA) ◽

10.20961/soja.v1i1.49659 ◽

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

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.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.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.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.

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THE IDIOPATHIC PULMONARY ARTERY HYPERTENSION WITH SEVERE TRICUSPID REGURGITATION

Journal of the American College of Cardiology ◽

10.1016/s0735-1097(19)33441-2 ◽

2019 ◽

Vol 73 (9) ◽

pp. 2835

Author(s):

Sang Hyun Lee ◽

Yong Hyun Park ◽

Soon Myung Jung ◽

Soo Young Lee ◽

Jun Wan Kim ◽

...

Keyword(s):

Pulmonary Artery ◽

Tricuspid Regurgitation ◽

Pulmonary Artery Hypertension ◽

Severe Tricuspid Regurgitation

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Predictors of survival in patients with precapillary pulmonary hypertension

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jeaa356.373 ◽

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.

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

Circulation Cardiovascular Interventions ◽

10.1161/circinterventions.120.009685 ◽

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.

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