RADICAL CORRECTION OF COMBINED CONGENITAL HEART DISEASE IN PATIENT WITH EXTRAHEPATIC PORTAL HYPERTENSION
Aim. To present rare clinical observation of successful surgical treatment of the patient with double-chamber right ventricle in combination with ventricular septal defect, subaortic fi brosis and muscular stenosis, insufficiency of the aortic valve and extrahepatic portal hypertension.Description....
| Main Authors: | , , , , , , |
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| Format: | Article |
| Language: | Russian |
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Federal Research Center of Transplantology and Artificial Organs named after V.I.Shumakov
2014-05-01
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| Series: | Вестник трансплантологии и искусственных органов |
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| Online Access: | https://journal.transpl.ru/vtio/article/view/224 |
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| author | A.S. Ivanov O.M. Tsirulnikova S.V. Glamazda A.V. Lebedeva N.N. Abramova V.V. Kuzmin E.K. Galnykina |
| author_facet | A.S. Ivanov O.M. Tsirulnikova S.V. Glamazda A.V. Lebedeva N.N. Abramova V.V. Kuzmin E.K. Galnykina |
| author_sort | A.S. Ivanov |
| collection | DOAJ |
| description | Aim. To present rare clinical observation of successful surgical treatment of the patient with double-chamber right ventricle in combination with ventricular septal defect, subaortic fi brosis and muscular stenosis, insufficiency of the aortic valve and extrahepatic portal hypertension.Description. Patient F., 15 years old, was diagnosed with Congenital Heart Disease – double-chambered right ventricle, ventricular septal defect. Fibromuscular subaortic stenosis. Aortic valve insuffi ciency II. Circulatory failure II A., functional class III. Extrahepatic portal hypertension. Splenomegaly. Thrombocytopenia. The state after the imposition of splenorenal anastomosis in September 1998 and the imposition of mesocaval H-shaped anastomosis in 1998. Non-functioning anastomoses. Patient indicated for surgical correction of CHD, however, given the low level of platelets, expressed splenomegaly, leucopenia, the patient was referred for a preliminary treatment to Rogachev’s Pediatric Hematology, Oncology and Immunology Center. The treatment (stimulating thrombopoiesis using romiplostim) gave no signifi cant effect. Platelet count reached 70–90 Å~ 109. Seeing of frequent bleeding from esophageal varices, the patient underwent varix sclerosis. At the time of hospitalization – no esophageal varices. The patient appealed to V.I. Shumakov Federal Research Center of Transplantology and Artifi cial Organs where he was recommended for surgical treatment.Result. The patient performed surgery: radical correction of CHD: resection of the stenosis of the outfl ow tract of the right ventricle, subaortic stenosis resection, closure of ventricular septal defect, AV plasty under cardiopulmonary bypass. The early postoperative period was uneventful. Leukopenia was observed to 1,2 Å~ 109, thrombocytopenia 70–90 Å~ 109. Despite the low level of platelets bleeding in pre-and postoperative period was not registered. Antibiotic therapy with tienam. Good postoperative results were obtained. Liver function was not affected.Conclusion. Presented clinical case demonstrates the possibility of surgical intervention of combined heart disease with cardiopulmonary bypass in patient with extrahepatic portal hypertension. |
| first_indexed | 2024-04-10T00:54:20Z |
| format | Article |
| id | doaj.art-89ac3b3c351e49eeb3a1094c6cc8ebb8 |
| institution | Directory Open Access Journal |
| issn | 1995-1191 |
| language | Russian |
| last_indexed | 2025-03-14T05:34:39Z |
| publishDate | 2014-05-01 |
| publisher | Federal Research Center of Transplantology and Artificial Organs named after V.I.Shumakov |
| record_format | Article |
| series | Вестник трансплантологии и искусственных органов |
| spelling | doaj.art-89ac3b3c351e49eeb3a1094c6cc8ebb82025-03-05T14:11:45ZrusFederal Research Center of Transplantology and Artificial Organs named after V.I.ShumakovВестник трансплантологии и искусственных органов1995-11912014-05-011629510210.15825/1995-1191-2014-2-95-102166RADICAL CORRECTION OF COMBINED CONGENITAL HEART DISEASE IN PATIENT WITH EXTRAHEPATIC PORTAL HYPERTENSIONA.S. Ivanov0O.M. Tsirulnikova1S.V. Glamazda2A.V. Lebedeva3N.N. Abramova4V.V. Kuzmin5E.K. Galnykina6Cardiac Division number 4 (Div. of CHD) V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs the Ministry of Health, Moscow, Russian FederationDivision of Clinical Transplantation at V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs the Ministry of Health, Moscow, Russian Federation. Division of Transplantation and Artificial Organs Medical University «The First Sechenov’s MGMU», Moscow, Russian FederationCardiac Division number 4 (Div. of CHD) V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs the Ministry of Health, Moscow, Russian FederationCardiac Division number 4 (Div. of CHD) V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs the Ministry of Health, Moscow, Russian FederationX-ray computed and magnetic resonance imaging Division at V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs the Ministry of Health, Moscow, Russian FederationCardiac Division number 4 (Div. of CHD) V.I. Shumakov Federal Research Center of Transplantology and Artifi cial Organs the Ministry of Health, Moscow, Russian FederationCardiac Division number 4 (Div. of CHD) V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs the Ministry of Health, Moscow, Russian FederationAim. To present rare clinical observation of successful surgical treatment of the patient with double-chamber right ventricle in combination with ventricular septal defect, subaortic fi brosis and muscular stenosis, insufficiency of the aortic valve and extrahepatic portal hypertension.Description. Patient F., 15 years old, was diagnosed with Congenital Heart Disease – double-chambered right ventricle, ventricular septal defect. Fibromuscular subaortic stenosis. Aortic valve insuffi ciency II. Circulatory failure II A., functional class III. Extrahepatic portal hypertension. Splenomegaly. Thrombocytopenia. The state after the imposition of splenorenal anastomosis in September 1998 and the imposition of mesocaval H-shaped anastomosis in 1998. Non-functioning anastomoses. Patient indicated for surgical correction of CHD, however, given the low level of platelets, expressed splenomegaly, leucopenia, the patient was referred for a preliminary treatment to Rogachev’s Pediatric Hematology, Oncology and Immunology Center. The treatment (stimulating thrombopoiesis using romiplostim) gave no signifi cant effect. Platelet count reached 70–90 Å~ 109. Seeing of frequent bleeding from esophageal varices, the patient underwent varix sclerosis. At the time of hospitalization – no esophageal varices. The patient appealed to V.I. Shumakov Federal Research Center of Transplantology and Artifi cial Organs where he was recommended for surgical treatment.Result. The patient performed surgery: radical correction of CHD: resection of the stenosis of the outfl ow tract of the right ventricle, subaortic stenosis resection, closure of ventricular septal defect, AV plasty under cardiopulmonary bypass. The early postoperative period was uneventful. Leukopenia was observed to 1,2 Å~ 109, thrombocytopenia 70–90 Å~ 109. Despite the low level of platelets bleeding in pre-and postoperative period was not registered. Antibiotic therapy with tienam. Good postoperative results were obtained. Liver function was not affected.Conclusion. Presented clinical case demonstrates the possibility of surgical intervention of combined heart disease with cardiopulmonary bypass in patient with extrahepatic portal hypertension.https://journal.transpl.ru/vtio/article/view/224двухкамерный правый желудочек, внепеченочная форма портальной гипертензии, искусственное кровообращение |
| spellingShingle | A.S. Ivanov O.M. Tsirulnikova S.V. Glamazda A.V. Lebedeva N.N. Abramova V.V. Kuzmin E.K. Galnykina RADICAL CORRECTION OF COMBINED CONGENITAL HEART DISEASE IN PATIENT WITH EXTRAHEPATIC PORTAL HYPERTENSION Вестник трансплантологии и искусственных органов двухкамерный правый желудочек, внепеченочная форма портальной гипертензии, искусственное кровообращение |
| title | RADICAL CORRECTION OF COMBINED CONGENITAL HEART DISEASE IN PATIENT WITH EXTRAHEPATIC PORTAL HYPERTENSION |
| title_full | RADICAL CORRECTION OF COMBINED CONGENITAL HEART DISEASE IN PATIENT WITH EXTRAHEPATIC PORTAL HYPERTENSION |
| title_fullStr | RADICAL CORRECTION OF COMBINED CONGENITAL HEART DISEASE IN PATIENT WITH EXTRAHEPATIC PORTAL HYPERTENSION |
| title_full_unstemmed | RADICAL CORRECTION OF COMBINED CONGENITAL HEART DISEASE IN PATIENT WITH EXTRAHEPATIC PORTAL HYPERTENSION |
| title_short | RADICAL CORRECTION OF COMBINED CONGENITAL HEART DISEASE IN PATIENT WITH EXTRAHEPATIC PORTAL HYPERTENSION |
| title_sort | radical correction of combined congenital heart disease in patient with extrahepatic portal hypertension |
| topic | двухкамерный правый желудочек, внепеченочная форма портальной гипертензии, искусственное кровообращение |
| url | https://journal.transpl.ru/vtio/article/view/224 |
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