28353604 Visualization

A 69 Age - year Age - old Age man Sex became aware of the onset of exertional Detailed_description dyspnea Sign_symptom in July Date 2010 Date .
He had a history of smoking History for History more History than History 40 History years, History 20 History cigarettes History per History day History .
His parents Family_history and Family_history 1 Family_history brother Family_history had Family_history lung Family_history cancer Family_history .
His past History medical History history History was History unremarkable History .
He had no History occupational History exposure History .
Due to the exertional Detailed_description dyspnea Sign_symptom , he was admitted Clinical_event to the People's Nonbiological_location Hospital Nonbiological_location of Nonbiological_location Shunde Nonbiological_location District Nonbiological_location for echocardiography Diagnostic_procedure and computed Diagnostic_procedure tomography Diagnostic_procedure pulmonary Diagnostic_procedure angiography Diagnostic_procedure ( CTPA Diagnostic_procedure ).
Echocardiography Diagnostic_procedure indicated that he had severe Severity PAH Disease_disorder , because his pulmonary Diagnostic_procedure arterial Diagnostic_procedure pressure Diagnostic_procedure ( PAP Diagnostic_procedure ) was 104 Lab_value   mmHg Lab_value , and his right Biological_structure heart Biological_structure was enlarged Sign_symptom (the right Diagnostic_procedure ventricle Diagnostic_procedure diameter Diagnostic_procedure : 57 Lab_value   mm Lab_value ).
CTPA Diagnostic_procedure showed that he had not only PAH Disease_disorder , but also a thrombus Disease_disorder in the upper Biological_structure lobe Biological_structure artery Biological_structure of Biological_structure the Biological_structure right Biological_structure lung Biological_structure .
However, the region Biological_structure in Biological_structure the Biological_structure lung Biological_structure affected by this PE Coreference was considered too small to induce such a severe PAH.
In addition, he did History not History have History connective History tissue History disease, History drug History use, History history History of History chemotherapy, History or History HIV History test History (+) History , so he was diagnosed as IPAH Disease_disorder combined with PE Disease_disorder .
He was treated with warfarin Medication ( 2.5 Dosage   mg Dosage per Dosage day Dosage with a target of the international Diagnostic_procedure normalized Diagnostic_procedure ratio Diagnostic_procedure [ INR Diagnostic_procedure ] between 1.5 Lab_value and Lab_value 2.5 Lab_value ) and slidenafil Medication targeted PAH therapy ( 20 Dosage   mg Dosage twice Dosage per Dosage day Dosage ).
The patient's dyspnea Sign_symptom was relieved slightly after the use of these medications Medication for 1 Duration month Duration and the retested PAP Diagnostic_procedure by echocardiography Detailed_description was decreased Lab_value to 72 Lab_value   mmHg Lab_value , but all the other Sign_symptom symptoms Sign_symptom and Sign_symptom signs Sign_symptom failed Lab_value to Lab_value further Lab_value improve Lab_value after taking these medications Medication for 6 Duration months Duration .
In March Date 2011 Date , he went Clinical_event to the Kyorin Nonbiological_location University Nonbiological_location affiliated Nonbiological_location hospital Nonbiological_location in Japan Nonbiological_location for further treatment.
Right Biological_structure heart Biological_structure catheterization Diagnostic_procedure revealed PAP Diagnostic_procedure 72/34/47 Lab_value   mmHg Lab_value , pulmonary Diagnostic_procedure capillary Diagnostic_procedure wedge Diagnostic_procedure pressure Diagnostic_procedure ( PCWP Diagnostic_procedure ) 17/5/10 Lab_value   mmHg Lab_value , cardiac Diagnostic_procedure output Diagnostic_procedure 4.3 Lab_value   L/min Lab_value , cardiac Diagnostic_procedure ejection Diagnostic_procedure index Diagnostic_procedure 2.36 Lab_value   L/min/m2 Lab_value , and pulmonary Diagnostic_procedure vascular Diagnostic_procedure resistance Diagnostic_procedure 7.3 Lab_value Wood Lab_value units Lab_value .
He was retested for CTPA Diagnostic_procedure that showed that the thrombus Disease_disorder in the upper Biological_structure lobe Biological_structure artery Biological_structure of Biological_structure the Biological_structure right Biological_structure lung Biological_structure disappeared.
He was still diagnosed as having IPAH Disease_disorder , so treated with sildenafil Medication ( 20 Dosage   mg Dosage 3 Dosage times Dosage a Dosage day Dosage ) and sorafenib Medication ( 400 Dosage   mg Dosage once Dosage a Dosage day Dosage ) targeted PAH Therapeutic_procedure therapy Therapeutic_procedure and oral Administration warfarin Medication anticoagulation Therapeutic_procedure therapy Therapeutic_procedure (2.5–3.75  mg Dosage per Dosage day Dosage , but the daily dose was adjusted by INR Diagnostic_procedure that was between 2 Lab_value and Lab_value 3 Lab_value ).
Disappointingly, his dyspnea Sign_symptom was not relieved and the PAP Diagnostic_procedure tested by the echocardiography Detailed_description was around Lab_value 95 Lab_value   mmHg Lab_value .
In September Date 2011 Date , he visited Clinical_event the Shanghai Nonbiological_location Pulmonary Nonbiological_location hospital Nonbiological_location in China Nonbiological_location because of no relief of dyspnea Sign_symptom .
Arterial Diagnostic_procedure blood Diagnostic_procedure gas Diagnostic_procedure analysis Diagnostic_procedure showed that PaO2 Diagnostic_procedure was 63 Lab_value   mmHg Lab_value with oxygen Diagnostic_procedure saturation Diagnostic_procedure 93% Lab_value ; lung Diagnostic_procedure function Diagnostic_procedure test Diagnostic_procedure revealed a mild Severity obstructive Disease_disorder dysfunction Disease_disorder of the pulmonary Detailed_description ventilation Detailed_description ( FEV1/FVC Diagnostic_procedure 62.06% Lab_value , FEV1% Diagnostic_procedure Prediction Detailed_description 80.9% Lab_value ) and a severe reduction Lab_value in the diffusing Diagnostic_procedure capacity Diagnostic_procedure of Diagnostic_procedure the Diagnostic_procedure lungs Diagnostic_procedure for Diagnostic_procedure carbon Diagnostic_procedure monoxide Diagnostic_procedure ( DLCO Diagnostic_procedure 42% Lab_value ); 6 Detailed_description - minute Detailed_description walk Diagnostic_procedure test Diagnostic_procedure was 385 Lab_value  m.
Chest Biological_structure high Diagnostic_procedure - resolution Diagnostic_procedure computed Diagnostic_procedure tomography Diagnostic_procedure ( HRCT Diagnostic_procedure ) (Fig.1A and B) showed smooth Texture thickening Sign_symptom of interlobular Biological_structure septa Biological_structure , diffuse Detailed_description centrilobular Biological_structure nodules Sign_symptom , and the enlargement Sign_symptom of mediastinal Biological_structure lymph Biological_structure nodes Biological_structure .
The right Biological_structure heart Biological_structure catheterization Diagnostic_procedure revealed that PAP Diagnostic_procedure was 83/34/53 Lab_value   mmHg Lab_value and PCWP Diagnostic_procedure was 14/6/11 Lab_value   mmHg Lab_value .
Acute Diagnostic_procedure vasodilator Diagnostic_procedure testing Diagnostic_procedure was negative Lab_value .
Coronary Diagnostic_procedure angiography Diagnostic_procedure and CTPA Diagnostic_procedure were both normal Lab_value ; pulmonary Biological_structure ventilation/perfusion Diagnostic_procedure ( V’/Q’ Diagnostic_procedure ) lung Biological_structure scan showed a matched Sign_symptom ventilation Sign_symptom and Sign_symptom perfusion Sign_symptom defects Sign_symptom .
Bronchoalveolar Diagnostic_procedure lavage Diagnostic_procedure showed an elevated Lab_value percentage ( 65% Lab_value ) of hemosiderin Biological_structure - laden Biological_structure macrophages Biological_structure with the Golde Diagnostic_procedure score Diagnostic_procedure 80 Lab_value (the normal range: 0–20).
He was still diagnosed as having IPAH Disease_disorder , but he was suspected as actually having PVOD Disease_disorder .
He was continuously treated with the targeted PAH Therapeutic_procedure therapy Therapeutic_procedure , and he was asked to continue his anticoagulation Therapeutic_procedure treatment Therapeutic_procedure , but he stopped warfarin Medication anticoagulation treatment by Detailed_description himself Detailed_description sometime Date in Date 2014 Date (he failed to recall the exact date.) because he felt that it was too cumbersome to frequently test the INR for blood coagulation monitoring.
Furthermore, after he discontinued his warfarin therapy, he also failed to monitor Diagnostic_procedure D Diagnostic_procedure - Dimer Diagnostic_procedure to prevent PE recurrence.
His dyspnea Sign_symptom was relatively stable Lab_value , but he developed many Detailed_description , but not Severity fatal Severity , episodes of pulmonary Disease_disorder edema Disease_disorder during the therapy.
In January Date 2015 Date , he was rushed Clinical_event to hospital Nonbiological_location because his dyspnea Sign_symptom was suddenly exacerbated Detailed_description .
CTPA Diagnostic_procedure showed a thrombus Disease_disorder in the right Biological_structure main Biological_structure pulmonary Biological_structure artery Biological_structure (Fig.1C).
Rivaroxaban Medication was administered for anticoagulation Therapeutic_procedure treatment Therapeutic_procedure , but this medication did not relieve his dyspnea Sign_symptom , therefore, he was too Sign_symptom ill Sign_symptom to Sign_symptom walk Sign_symptom .
Instead, he was bound Activity to Activity his Activity wheelchair Activity for half Duration a Duration year Duration .
The severe PAH Disease_disorder (suspected as PVOD Disease_disorder ) combined with a life Severity - threatening Severity PE Disease_disorder mandates a lung Therapeutic_procedure transplantation Therapeutic_procedure for his survival.
Six Date months Date later Date , he was undergone a bilateral Detailed_description sequential Detailed_description allogenic Detailed_description lung Therapeutic_procedure transplantation Therapeutic_procedure assisted by veno Therapeutic_procedure - arterial Therapeutic_procedure extra Therapeutic_procedure - corporeal Therapeutic_procedure membrane Therapeutic_procedure oxygenation Therapeutic_procedure ( VA Therapeutic_procedure - ECMO Therapeutic_procedure ) after he signed the written consent on July Date 29, Date 2015 Date .
Histology Diagnostic_procedure of the explanted Biological_structure lung Biological_structure specimen Biological_structure showed pulmonary Biological_structure vein Biological_structure occlusion Lab_value (Fig.1D–F) and the pulmonary Biological_structure embolism Lab_value in the right Biological_structure pulmonary Biological_structure artery Biological_structure .
Until then, he was confirmed as having PVOD Disease_disorder combined with PE Disease_disorder , instead of IPAH Disease_disorder .
He has been living well Sign_symptom without dyspnea Sign_symptom for more Duration than Duration 1 Duration year Duration after the lung transplantation and the echocardiography Diagnostic_procedure showed the normalizations Lab_value of PAP Diagnostic_procedure and the once enlarged Sign_symptom right Biological_structure ventricle Biological_structure of Biological_structure his Biological_structure heart Biological_structure .
This study was approved by the Ethics Committee of The People's Hospital of Zhongshan City.
The written informed consent was obtained from the patient.