scholarly journals Fatal pulmonary tumour thrombotic microangiopathy in patient with ovarian adenocarcinoma: review and a case report

2022 ◽  
Vol 22 (1) ◽  
Author(s):  
Gintare Neverauskaite-Piliponiene ◽  
Kristijonas Cesas ◽  
Darius Pranys ◽  
Skaidrius Miliauskas ◽  
Lina Padervinskiene ◽  
...  

Abstract Background Pulmonary tumour thrombotic microangiopathy (PTTM) is a fatal disease in which tumour cells embolize to the pulmonary vasculature leading to pulmonary hypertension and right heart failure. Early diagnosis is essential for timely treatment which can reduce intimal pulmonary vascular proliferation and prolong survival, improve the symptoms. Due to rare occurrences and no clear diagnostic guidelines the disorder usually is found post-mortem. We present a review of this rare disease and a case of post-mortem diagnosed pulmonary tumour thrombotic microangiopathy in a young female. Case presentation 51 years old woman presented with progressively worsening dyspnea, right ventricular failure signs and symptoms. Computerized tomography denied pulmonary embolism. 2D transthoracic echocardiography demonstrated right ventricle dilatation and dysfunction, severely increased systolic pulmonary pressure. Right heart catheterization revealed pre-capillary pulmonary hypertension with mean pulmonary artery pressure of 78 mmHg, pulmonary wedge pressure of 15 mmHg, reduced cardiac output to 1.78 L/min with a calculated pulmonary vascular resistance of 35 Wood units, and extremely low oxygen saturation (26%) in pulmonary artery. Because of worsening ascites, pelvic magnetic resonance imaging was performed, tumours in both ovaries were diagnosed. Due to the high operative risk, detailed tumour diagnosis surgically was not established. The patient developed progressive cardiorespiratory failure, unresponsive to optimal heart failure drug treatment. A postmortem morphology analyses revealed tumorous masses in pre-capillary lung vessels, right ventricle hypertrophy, ovary adenocarcinoma. Conclusions An early diagnosis of PTTM is essential. Most cases are lethal due to respiratory failure progressing rapidly. Patients with a history of malignancy, symptoms and signs implying of PH should be considered of having PTTM. If detected early enough, combination of chemotherapy with specific PH therapy is believed to be beneficial in reducing intimal proliferation and prolonging survival, along with improving the symptoms.

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.


2019 ◽  
Vol 29 (5) ◽  
pp. 704-707 ◽  
Author(s):  
Roopesh Singhal

AbstractUnilateral interruption of pulmonary artery is a rare congenital anomaly which is usually associated with other congenital heart disease. Even more rarely it may occur in isolation. Most of the cases are incidentally detected in adulthood. Some cases develop pulmonary hypertension for yet unknown reasons; such cases usually present in infancy with right heart failure. Surgical correction in such cases is associated with adverse outcomes. Heart lung transplantation should be considered in such patients. We report a 3-year-old boy with interruption of right pulmonary artery with severe pulmonary hypertension and right heart failure who was considered for heart lung transplantation.


2020 ◽  
Vol 8 ◽  
pp. 2050313X2096904
Author(s):  
Constanza Burciaga Calderoni ◽  
Dafne T Moretta ◽  
Jeanette Merrill-Henry ◽  
Paresh C Giri

Pulmonary tumor thrombotic microangiopathy is a rare condition in which embolization of tumor cells to the pulmonary arterioles causes fibrocellular intimal thickening and activation of the coagulation cascade resulting in pulmonary hypertension and right heart failure. Herein, we highlight a young 35-year-old male with no known past medical history who presented with recurrent syncope and dyspnea, and was found to have severe right heart failure and pulmonary hypertension. He developed sudden clinical deterioration and died after a cardiac arrest. Autopsy revealed poorly differentiated gastric adenocarcinoma and pulmonary tumor thrombotic microangiopathy. New onset severe pulmonary hypertension and right heart failure without any other obvious etiology should encourage the reader to evaluate for pulmonary tumor thrombotic microangiopathy and undergo a diligent search for underlying malignancy. This case highlights recurrent syncope as a rare presentation of this rapidly fatal disease.


2015 ◽  
Vol 96 (4) ◽  
pp. 492-497
Author(s):  
I A Kamalov ◽  
M G Tukhbatullin

Aim. Develop new approaches to the diagnosis of right heart failure and pulmonary hypertension in recurrent thromboembolism of small branches of the pulmonary artery in patients with malignant tumors. Methods. 83 patients with malignant tumors of various localizations were examined and followed-up. The main group included 49 patients with malignant tumors of various localizations and related venous thrombosis. The control group included 34 patients who did not have venous thrombosis. Patients in both groups underwent ultrasonography of inferior vena cava system veins and echocardiography at intervals of 3-4 days during the diagnosis and treatment of malignant tumors. Right ventricle ejection fraction and systolic pressure in the pulmonary artery were calculated at echocardiography. Results. No signs of inferior vena cava system veins thromboses, right heart failure, pulmonary hypertension were identified in patients of the control group while setting up the diagnosis and treatment of malignancies. In 38 out of 49 patients of the main group, right ventricular failure and pulmonary hypertension of varying severity were detected. The condition of 46 patients of the main group gradually improved after treating with anticoagulants. Conclusion. Recanalization of venous thrombosis is accompanied by frequent rejection of micro thrombi and embolization of small branches of pulmonary artery, causing right heart failure and pulmonary hypertension, which can be promptly detected by repeated echocardiography.


2018 ◽  
Vol 35 (02) ◽  
pp. 136-142 ◽  
Author(s):  
G. Pretorius ◽  
Stuart Jamieson

AbstractChronic thromboembolic pulmonary hypertension occurs when acute thromboemboli fail to dissolve completely. The resulting fibrotic scar tissue within the pulmonary arteries is obstructive and eventually leads to right heart failure. Medical therapy for this condition is supportive, but surgery with pulmonary artery endarterectomy is curative, and carries a low mortality at experienced centers.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
N Bart ◽  
S.L Hungerford ◽  
S Emmanuel ◽  
K Kearney ◽  
E Kotlyar ◽  
...  

Abstract   The Pulmonary artery pulsatility index (PAPi) is a novel haemodynamic marker that has been shown to be a strong predictor of right heart failure and mortality in patients with pulmonary hypertension and advanced heart failure. Its utility in predicting post transplant outcomes is currently unknown. Aim To determine the prognostic significance of pre-transplant haemodynamic assessment of PAPi, right atrial pressure (RA), and pulmonary vascular resistance (PVR) in predicting mortality post cardiac transplant. Methods All patients who underwent cardiac transplantation between January 2010 and June 2016 were retrospectively analyzed. Pre-operative right heart catheter data was obtained. The PAPi was calculated as the systolic pulmonary artery pressure (sPAP)- diastolic pulmonary artery pressure (dPAP) divided by RA. Statistical analysis was carried out using SPSS v25. Results A total of 158 patients, with a mean age of 48.6±13.5 were studied (115 non- LVAD, 43 LVAD pre-transplant), and 3 patients were excluded due to missing data. There was no significant difference in the non-LVAD group in RA, PVR or PAPi for mortality post transplant. In the LVAD group there was no significant difference in RA, or PAPi with regards to mortality, however there was a significant difference in mean PVR between those that died post transplant (222±100) and those still alive (138±59) Conclusion Despite having a role in prognostication for right heart failure in pulmonary hypertension and post LVAD implantation, PAPi did not discriminate mortality outcomes for patients post cardiac transplantation. PVR remains a marker of mortality in an LVAD cohort bridged to transplant. Funding Acknowledgement Type of funding source: None


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
F Bandera ◽  
G Ghizzardi ◽  
M Agnifili ◽  
S Pizzocri ◽  
S Boveri ◽  
...  

Abstract Background the TAPSE/SPAP ratio has gained a role as an easy-to-use surrogate of right ventricle-to-pulmonary circulation (RV-PC) coupling, showing a strong prognostic significance in heart failure (HF) patients. The hemodynamic determinants of TAPSE/SPAP ratio, as assessed by invasive approach, have not been fully clarified. Aim To identify the right heart hemodynamic variables correlated with TAPSE/SPAP in a HF with reduced EF (HFrEF) cohort, both at rest and during exercise. Methods 30 HFrEF patients (age 68 ± 10 years LV EF 28 ± 7) underwent to rest and exercise echocardiography and performed right heart catheterization within 24 hours. Bivariate correlations between TAPSE/SPAP ratio (at rest and during exercise), right heart hemodynamic variables, RV systolic function and NTproBNP have been explored. Results TAPSE/SPAP ratio at rest showed a moderate correlation with pulmonary artery wedge pressure (PAWP: r= 0.441; p= 0.039), pulmonary artery pressures (PAP systolic: r = 0.481; p= 0.026; PAP diastolic: r= 0.434; p= 0.043; mPAP: r= 0.476; p= 0.025), pulmonary vascular resistance and compliance (r= 0.475; p= 0.041). A stronger correlation was identified with right atrial (RAP systolic: r= 0.586; p= 0.017; RAP diastolic: r= 0.681; p= 0.006) and right ventricular pressures- in particular diastolic ones (RVP systolic: r= 0.584; p= 0.004; RAP diastolic: r= 0.652; p= 0.002). No significant correlation with NTproBNP and RV 3D EF emerged. Exercise TAPSE/SPAP ratio significantly correlated with right atrium (RAP systolic: r= 0.564; p= 0.036) and right ventricle systolic pressures only (RVP systolic: r= 0.789; p< 0.001). Conclusions TAPSE/SPAP ratio at rest showed a stronger correlation with invasively derived diastolic right heart pressure rather than pulmonary vascular bed pressures. A similar correlation was also observed for exercise TAPSE/SPAP ratio. This tight correlation with RV, rather than with vascular pressures, supports the significance of this ratio as a marker of RV adaptation to vascular overload.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
F Bandera ◽  
G Ghizzardi ◽  
M Agnifili ◽  
S Pizzocri ◽  
S Boveri ◽  
...  

Abstract Background The TAPSE/SPAP ratio has gained a role as an easy-to-use surrogate of right ventricle-to-pulmonary circulation (RV-PC) coupling, showing a strong prognostic significance in heart failure (HF) patients. The hemodynamic determinants of TAPSE/SPAP ratio, as assessed by invasive approach, have not been fully elucidated. Aim To identify the right heart hemodynamic variables correlated with TAPSE/SPAP in a HF with reduced EF (HFrEF) cohort, at rest and during exercise. Methods 24 HFrEF patients (age 67±11 years LV EF 27±7) underwent to rest and exercise echocardiography and performed right heart catheterization within 24 hours. Bivariate correlations between TAPSE/SPAP ratio (at rest and exercise), right heart hemodynamic variables, RV systolic function and NTproBNP have been explored. Results TAPSE/SPAP ratio at rest showed a moderate correlation with pulmonary artery wedge pressure (PAWP: r=0.432; p=0.039), pulmonary artery pressures (PAP systolic: r=0.474; p=0.026; PAP diastolic: r=0.434; p=0.043; mPAP: r=0.476; p=0.025), pulmonary vascular resistance and compliance (r=0.475; p=0.041), while a stronger correlation was identified with right atrial (RAP systolic: r=0.571; p=0.017; RAP diastolic: r=0.675; p=0.006) and right ventricular pressures- in particular diastolic ones (RVP systolic: r=0.584; p=0.004; RAP diastolic: r=0.646; p=0.002). No significant correlation was found with NTproBNP and RV 3D EF. Exercise TAPSE/SPAP ratio significantly correlated with right atrium (RAP systolic: r=0.564; p=0.036) and right ventricle systolic pressures only (RVP systolic: r=0.765; p<0.001). TAPSE/PAPS correlation graphics Conclusions TAPSE/SPAP ratio at rest showed a stronger correlation with invasively derived diastolic right heart pressure rather than pulmonary vascular bed pressures. A similar correlation was also observed for exercise TAPSE/SPAP ratio. This tight correlation with RV, rather than vascular pressures, supports the significance of the ratio as a marker of RV adaptation to vascular overload.


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