Advanced Pulmonary Hypertension Due to Congenital Double-shunt Successfully Treated with Surgical Repair and Up-front Combination Therapy

This case report concerns a 22-year-old woman with large patent ductus arteriosus and atrial septal defect. She was referred to our hospital because of exertional dyspnea and was revealed to have advanced pulmonary arterial hypertension (PAH) with a mean pulmonary arterial pressure (PAP) of 79 mmHg....

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Detalles Bibliográficos
Autores principales: Suto, Makiko, Matsumoto, Kensuke, Kido, Sachiko, Mori, Shumpei, Nakayama, Kazuhiko, Tanaka, Hidekazu, Inoue, Takeshi, Emoto, Noriaki, Okita, Yutaka, Hirata, Ken-ichi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2019
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6543216/
https://www.ncbi.nlm.nih.gov/pubmed/31061260
http://dx.doi.org/10.2169/internalmedicine.2183-18
Descripción
Sumario:This case report concerns a 22-year-old woman with large patent ductus arteriosus and atrial septal defect. She was referred to our hospital because of exertional dyspnea and was revealed to have advanced pulmonary arterial hypertension (PAH) with a mean pulmonary arterial pressure (PAP) of 79 mmHg. Although both shunts had bidirectional flow, based on the results of acute pulmonary vasoreactive testing, one-stage surgical closure was performed followed by up-front combination therapy for post-operative pulmonary hypertensive crisis and residual PAH. At 14 months after the surgery, her symptoms were markedly improved, and her mean PAP had dramatically decreased to 13 mmHg.

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