I. Miklashevich, E. A. Potrokhova, D. A. Morozov, Yu. S. Isaeva
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Pulmonary arterial hypertension associated with type II Abernethy malformation in an adolescent: a case report
The article provides a case of pulmonary arterial hypertension (PAH) associated with congenital extrahepatic portocaval shunt (CEPS), or Abernethy malformation, in a 17-year-old female patient. CEPS, which remained undiagnosed for a long time, manifested with severe encephalopathy at an early age. By the age of 6 years, it was complicated by PAH, and by the age of 9 years — by hepatic focal nodular hyperplasia. In the absence of timely surgical treatment and adequate therapy of CEPS, PAH progressed. Combined dual therapy for PAH, prescribed only at age 14, was ineffective. Despite the potential for clinical and functional improvement documented with combination triple therapy at 17 years of age, the patient continued to meet criteria for a high risk of adverse events, including mortality. Two months after the initiation of triple therapy for PAH, not achieving the target hemodynamic characteristics that would reduce operative and perioperative risks lead to an attempt of surgical treatment of CEPS, which led to the patient death in the early postoperative period. The description of this case report shows the difficulties of diagnosing a rare form of portopulmonary hypertension, the modern possibilities of drug therapy for a severe, potentially curable PAH in the early stages.
期刊介绍:
The most important objectives of the journal are: the generalization of scientific and practical achievements in the field of cardiology, increasing scientific and practical skills of cardiologists.
The scientific concept of publication does the publication of modern achievements in the field of epidemiology, prevention and treatment of cardiovascular diseases, the results of research, national and international clinical trials.
For publication in the journal are invited both domestic and foreign scientists and clinicians working in the field of cardiology, as well as doctors of other specialties.
The magazine covers various issues in cardiology and related specialties. Each issue is prepared by Executive editor of the issue, a respected specialist in the field of epidemiology, prevention and treatment of cardiovascular diseases.
The main focus of the publication — scientific articles on original research, the pharmacotherapy of cardiovascular disease, new diagnostic methods.
All members of the group of authors should meet all four criteria of authorship set forth in the ICMJE recommendations: 1) concept and design development or data analysis and interpretation, and 2) manuscript justification or verification of critical intellectual content, and 3) final approval for publication of the manuscript, and 4) consent to be responsible for all aspects of the work, and assume that issues relating to the thoroughness and diligent execution of any part of the study submitted are duly investigated and resolved.
Great importance the editors attached to the preparation of scientific papers by groups of authors at a high level, literacy, authors, and their ownership information, availability of research results not only to colleagues in Russia, but also abroad.