Rendu-Osler-Weber Disease with High Pulmonary Hypertension and Interstitial Lung Disease
A 64-year-old female with a family history of hereditary hemorrhagic telangiectasia (HHT) was hospitalized due to complaints of dyspnea during light physical exertion and leg edema. HHT was diagnosed at 20 y.o., recurrent nasal bleeding started at age 52, bleedings severity was aggravated by not com...
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Столичная издательская компания
2023-05-01
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| Series: | Рациональная фармакотерапия в кардиологии |
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| Online Access: | https://www.rpcardio.online/jour/article/view/2652 |
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| author | Yu. A. Lutokhina O. V. Blagova P. O. Savina E. V. Zaklyazminskaya |
| author_facet | Yu. A. Lutokhina O. V. Blagova P. O. Savina E. V. Zaklyazminskaya |
| author_sort | Yu. A. Lutokhina |
| collection | DOAJ |
| description | A 64-year-old female with a family history of hereditary hemorrhagic telangiectasia (HHT) was hospitalized due to complaints of dyspnea during light physical exertion and leg edema. HHT was diagnosed at 20 y.o., recurrent nasal bleeding started at age 52, bleedings severity was aggravated by not completely compensated hypertension. At the age of 60, after a massive hemorrhage, she noted the onset of dyspnea, edema, ascites. Diuretics and iron preparations improved her well-being, but from that period onward her heart failure worsened after each massive blood loss. The last major bleeding was before the present hospitalization (Hgb 67 g/l), after which heart failure symptoms significantly deteriorated. Echocardiography showed preserved left ventricular ejection fraction, but revealed high pulmonary hypertension (systolic pulmonary artery pressure 69 mmHg). Chest computed tomography (CT) with contrast showed no evidence of pulmonary embolism, but interstitial lung lesions were detected. Pulse therapy with glucocorticosteroids did not result in positive dynamics at the control CT scan, which allowed to reject a separate interstitial lung disease. As a result of cardiotropic and diuretic therapy, as well as correction of anemia, the patient's condition improved. Macitentan was administered, but the patient refused from it because one of possible side effects was anemia. A year later the patient diedfrom acute progression of pulmonary hypertension. According to the literature, pulmonary hypertension in HHT can have a significant impact on the prognosis and requires timely diagnosis and treatment. Interstitial lung lesions are a manifestation of the underlying disease and does not require special treatment. |
| format | Article |
| id | doaj-art-0d4b23c48c334811a007ab23a435eedd |
| institution | Kabale University |
| issn | 1819-6446 2225-3653 |
| language | English |
| publishDate | 2023-05-01 |
| publisher | Столичная издательская компания |
| record_format | Article |
| series | Рациональная фармакотерапия в кардиологии |
| spelling | doaj-art-0d4b23c48c334811a007ab23a435eedd2025-08-23T10:00:36ZengСтоличная издательская компанияРациональная фармакотерапия в кардиологии1819-64462225-36532023-05-0119217918510.20996/1819-6446-2023-03-062116Rendu-Osler-Weber Disease with High Pulmonary Hypertension and Interstitial Lung DiseaseYu. A. Lutokhina0O. V. Blagova1P. O. Savina2E. V. Zaklyazminskaya3I.M. Sechenov First Moscow State Medical University (Sechenov University)I.M. Sechenov First Moscow State Medical University (Sechenov University)I.M. Sechenov First Moscow State Medical University (Sechenov University)Petrovsky National Research Centre of SurgeryA 64-year-old female with a family history of hereditary hemorrhagic telangiectasia (HHT) was hospitalized due to complaints of dyspnea during light physical exertion and leg edema. HHT was diagnosed at 20 y.o., recurrent nasal bleeding started at age 52, bleedings severity was aggravated by not completely compensated hypertension. At the age of 60, after a massive hemorrhage, she noted the onset of dyspnea, edema, ascites. Diuretics and iron preparations improved her well-being, but from that period onward her heart failure worsened after each massive blood loss. The last major bleeding was before the present hospitalization (Hgb 67 g/l), after which heart failure symptoms significantly deteriorated. Echocardiography showed preserved left ventricular ejection fraction, but revealed high pulmonary hypertension (systolic pulmonary artery pressure 69 mmHg). Chest computed tomography (CT) with contrast showed no evidence of pulmonary embolism, but interstitial lung lesions were detected. Pulse therapy with glucocorticosteroids did not result in positive dynamics at the control CT scan, which allowed to reject a separate interstitial lung disease. As a result of cardiotropic and diuretic therapy, as well as correction of anemia, the patient's condition improved. Macitentan was administered, but the patient refused from it because one of possible side effects was anemia. A year later the patient diedfrom acute progression of pulmonary hypertension. According to the literature, pulmonary hypertension in HHT can have a significant impact on the prognosis and requires timely diagnosis and treatment. Interstitial lung lesions are a manifestation of the underlying disease and does not require special treatment.https://www.rpcardio.online/jour/article/view/2652rendu-osler syndromehereditary hemorrhagic telangiectasiapulmonary hypertensioncongestive heart failure |
| spellingShingle | Yu. A. Lutokhina O. V. Blagova P. O. Savina E. V. Zaklyazminskaya Rendu-Osler-Weber Disease with High Pulmonary Hypertension and Interstitial Lung Disease Рациональная фармакотерапия в кардиологии rendu-osler syndrome hereditary hemorrhagic telangiectasia pulmonary hypertension congestive heart failure |
| title | Rendu-Osler-Weber Disease with High Pulmonary Hypertension and Interstitial Lung Disease |
| title_full | Rendu-Osler-Weber Disease with High Pulmonary Hypertension and Interstitial Lung Disease |
| title_fullStr | Rendu-Osler-Weber Disease with High Pulmonary Hypertension and Interstitial Lung Disease |
| title_full_unstemmed | Rendu-Osler-Weber Disease with High Pulmonary Hypertension and Interstitial Lung Disease |
| title_short | Rendu-Osler-Weber Disease with High Pulmonary Hypertension and Interstitial Lung Disease |
| title_sort | rendu osler weber disease with high pulmonary hypertension and interstitial lung disease |
| topic | rendu-osler syndrome hereditary hemorrhagic telangiectasia pulmonary hypertension congestive heart failure |
| url | https://www.rpcardio.online/jour/article/view/2652 |
| work_keys_str_mv | AT yualutokhina renduoslerweberdiseasewithhighpulmonaryhypertensionandinterstitiallungdisease AT ovblagova renduoslerweberdiseasewithhighpulmonaryhypertensionandinterstitiallungdisease AT posavina renduoslerweberdiseasewithhighpulmonaryhypertensionandinterstitiallungdisease AT evzaklyazminskaya renduoslerweberdiseasewithhighpulmonaryhypertensionandinterstitiallungdisease |