Congenital Isolated Unilateral Agenesis of Pulmonary Arteries with 3-Year Follow-Up after Initiation of Treatment
<i>Background and objective</i>: Unilateral agenesis of pulmonary arteries (UAPA) is a rare disease, with approximately 400 cases reported to date. UAPA is often associated with congenital heart disease, and the uncomplicated form is isolated UAPA, which accounts for approximately 30% of...
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MDPI AG
2023-06-01
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| Series: | Medicina |
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| Online Access: | https://www.mdpi.com/1648-9144/59/6/1161 |
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| author | Marohito Nakata Naoko Yokota Hiroki Uehara Kazuiko Tabata Tsuneaki Kenzaka |
| author_facet | Marohito Nakata Naoko Yokota Hiroki Uehara Kazuiko Tabata Tsuneaki Kenzaka |
| author_sort | Marohito Nakata |
| collection | DOAJ |
| description | <i>Background and objective</i>: Unilateral agenesis of pulmonary arteries (UAPA) is a rare disease, with approximately 400 cases reported to date. UAPA is often associated with congenital heart disease, and the uncomplicated form is isolated UAPA, which accounts for approximately 30% of all cases of UAPA. The incidence of pulmonary hypertension due to UAPA has been reported to range from 19 to 44%. There is no consensus treatment for pulmonary hypertension associated with UAPA. We present the first reported case in which a three-drug combination, comprising of iloprost inhalation, riociguat, and ambrisentan, was administered to a patient with UAPA, and was followed-up for 3 years post-diagnosis. <i>Case presentation</i>: A 68-year-old Japanese woman presented to our hospital with dyspnea and chest discomfort. She underwent chest radiography, blood tests, and echocardiography; however, the cause of the patient’s symptoms could not be identified. During regular follow-up, an echocardiography 21 months after the initial visit revealed elevated right ventricular pressure (peak tricuspid regurgitation velocity: 5.2 m/s and right ventricular systolic pressure: 120 mmHg) and a diagnosis of pulmonary hypertension was made. Contrast-enhanced computed tomography (CT) of the chest and a pulmonary blood flow scintigram were performed to investigate the cause of pulmonary hypertension, and isolated UAPA was diagnosed. The patient was treated with a three-drug combination of iloprost inhalation, riociguat, and ambrisentan and followed up for 3 years with good therapeutic outcomes. <i>Conclusions</i>: We present a case of pulmonary hypertension caused by isolated UAPA. Although rare, this disease can lead to pulmonary hypertension and should be treated cautiously. While there is no consensus regarding the treatment of this disease, a three-drug combination of iloprost inhalation, riociguat, and oral ambrisentan proved effective. |
| first_indexed | 2024-03-11T02:10:07Z |
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| language | English |
| last_indexed | 2024-03-11T02:10:07Z |
| publishDate | 2023-06-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Medicina |
| spelling | doaj.art-3a3ae77b69c748229664e641c4299ce32023-11-18T11:32:22ZengMDPI AGMedicina1010-660X1648-91442023-06-01596116110.3390/medicina59061161Congenital Isolated Unilateral Agenesis of Pulmonary Arteries with 3-Year Follow-Up after Initiation of TreatmentMarohito Nakata0Naoko Yokota1Hiroki Uehara2Kazuiko Tabata3Tsuneaki Kenzaka4Department of Cardiology, Urasoe General Hospital, Urasoe 901-2132, JapanDepartment of Cardiology, Urasoe General Hospital, Urasoe 901-2132, JapanDepartment of Cardiology, Urasoe General Hospital, Urasoe 901-2132, JapanDepartment of Internal Medicine, Naha City Hospital, Naha 902-8511, JapanDivision of Community Medicine and Career Development, Graduate School of Medicine, Kobe University, Kobe 652-0032, Japan<i>Background and objective</i>: Unilateral agenesis of pulmonary arteries (UAPA) is a rare disease, with approximately 400 cases reported to date. UAPA is often associated with congenital heart disease, and the uncomplicated form is isolated UAPA, which accounts for approximately 30% of all cases of UAPA. The incidence of pulmonary hypertension due to UAPA has been reported to range from 19 to 44%. There is no consensus treatment for pulmonary hypertension associated with UAPA. We present the first reported case in which a three-drug combination, comprising of iloprost inhalation, riociguat, and ambrisentan, was administered to a patient with UAPA, and was followed-up for 3 years post-diagnosis. <i>Case presentation</i>: A 68-year-old Japanese woman presented to our hospital with dyspnea and chest discomfort. She underwent chest radiography, blood tests, and echocardiography; however, the cause of the patient’s symptoms could not be identified. During regular follow-up, an echocardiography 21 months after the initial visit revealed elevated right ventricular pressure (peak tricuspid regurgitation velocity: 5.2 m/s and right ventricular systolic pressure: 120 mmHg) and a diagnosis of pulmonary hypertension was made. Contrast-enhanced computed tomography (CT) of the chest and a pulmonary blood flow scintigram were performed to investigate the cause of pulmonary hypertension, and isolated UAPA was diagnosed. The patient was treated with a three-drug combination of iloprost inhalation, riociguat, and ambrisentan and followed up for 3 years with good therapeutic outcomes. <i>Conclusions</i>: We present a case of pulmonary hypertension caused by isolated UAPA. Although rare, this disease can lead to pulmonary hypertension and should be treated cautiously. While there is no consensus regarding the treatment of this disease, a three-drug combination of iloprost inhalation, riociguat, and oral ambrisentan proved effective.https://www.mdpi.com/1648-9144/59/6/1161unilateral agenesispulmonary arterypulmonary hypertension |
| spellingShingle | Marohito Nakata Naoko Yokota Hiroki Uehara Kazuiko Tabata Tsuneaki Kenzaka Congenital Isolated Unilateral Agenesis of Pulmonary Arteries with 3-Year Follow-Up after Initiation of Treatment Medicina unilateral agenesis pulmonary artery pulmonary hypertension |
| title | Congenital Isolated Unilateral Agenesis of Pulmonary Arteries with 3-Year Follow-Up after Initiation of Treatment |
| title_full | Congenital Isolated Unilateral Agenesis of Pulmonary Arteries with 3-Year Follow-Up after Initiation of Treatment |
| title_fullStr | Congenital Isolated Unilateral Agenesis of Pulmonary Arteries with 3-Year Follow-Up after Initiation of Treatment |
| title_full_unstemmed | Congenital Isolated Unilateral Agenesis of Pulmonary Arteries with 3-Year Follow-Up after Initiation of Treatment |
| title_short | Congenital Isolated Unilateral Agenesis of Pulmonary Arteries with 3-Year Follow-Up after Initiation of Treatment |
| title_sort | congenital isolated unilateral agenesis of pulmonary arteries with 3 year follow up after initiation of treatment |
| topic | unilateral agenesis pulmonary artery pulmonary hypertension |
| url | https://www.mdpi.com/1648-9144/59/6/1161 |
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