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Mahdavi, Mohammad, Shahzadi, Hossein, Ghasemnezhad, Mohsen, Hoseinzadeh Moghadam, Soheila. (1403). Pulmonary Capillary Hemangiomatosis as a Rare Underlying Cause of Primary Pulmonary Hypertension: A Case Report in an Adolescent. سامانه مدیریت نشریات علمی, 49(6), 394-398. doi: 10.30476/ijms.2024.101215.3385
Mohammad Mahdavi; Hossein Shahzadi; Mohsen Ghasemnezhad; Soheila Hoseinzadeh Moghadam. "Pulmonary Capillary Hemangiomatosis as a Rare Underlying Cause of Primary Pulmonary Hypertension: A Case Report in an Adolescent". سامانه مدیریت نشریات علمی, 49, 6, 1403, 394-398. doi: 10.30476/ijms.2024.101215.3385
Mahdavi, Mohammad, Shahzadi, Hossein, Ghasemnezhad, Mohsen, Hoseinzadeh Moghadam, Soheila. (1403). 'Pulmonary Capillary Hemangiomatosis as a Rare Underlying Cause of Primary Pulmonary Hypertension: A Case Report in an Adolescent', سامانه مدیریت نشریات علمی, 49(6), pp. 394-398. doi: 10.30476/ijms.2024.101215.3385
Mahdavi, Mohammad, Shahzadi, Hossein, Ghasemnezhad, Mohsen, Hoseinzadeh Moghadam, Soheila. Pulmonary Capillary Hemangiomatosis as a Rare Underlying Cause of Primary Pulmonary Hypertension: A Case Report in an Adolescent. سامانه مدیریت نشریات علمی, 1403; 49(6): 394-398. doi: 10.30476/ijms.2024.101215.3385

Pulmonary Capillary Hemangiomatosis as a Rare Underlying Cause of Primary Pulmonary Hypertension: A Case Report in an Adolescent

Iranian Journal of Medical Sciences
مقاله 7، دوره 49، شماره 6، شهریور 2024، صفحه 394-398    اصل مقاله (885.38 K)
نوع مقاله: Case Report
شناسه دیجیتال (DOI): 10.30476/ijms.2024.101215.3385
نویسندگان
Mohammad Mahdavi؛ Hossein Shahzadi؛ Mohsen Ghasemnezhad؛ Soheila Hoseinzadeh Moghadam*
Shaheed Rajaie Cardiovascular Medical and Research Center, School of Medicine, Iran University of Medical Sciences, Tehran, Iran
چکیده
Despite its rarity, pulmonary capillary hemangiomatosis (PCH) presents a significant diagnostic challenge. Due to its similarity to other pulmonary vascular diseases, such as pulmonary veno-occlusive disease, it is characterized by abnormal pulmonary capillary proliferation, which is a rare cause of primary pulmonary hypertension. This case was the first reported instance of PCH in Shahid Rajaee Heart Hospital in Tehran, Iran, in 2023, which was confirmed by genetic testing. It highlighted the importance of considering PCH among the differential diagnoses for pulmonary hypertension, even in adolescent patients. The 13-year-old patient’s main complaints were progressive exertional dyspnea and chest pain. He had no previous medical history and had not taken any pharmaceutical or herbal medications. Critical clinical findings included a heart murmur, an electrocardiogram revealing right ventricular hypertrophy, and echocardiogram evidence of pulmonary hypertension. The main diagnosis was PCH, as shown by CT findings of pulmonary artery dilatation and diffuse nodular ground glass opacities. Genetic tests indicated pathogenic EIF2AK4 mutations and suspicion of PCH. Therapeutic intervention included vasodilator therapy, which exacerbated the patient’s condition. This case emphasized the importance of maintaining a high index of suspicion for rare causes of pulmonary hypertension, such as PCH. The outcome was to prepare the patient for lung transplantation. To differentiate PCH from other pulmonary vascular diseases, a combination of clinical presentation, radiologic studies, genetic analysis, and response to treatment is required to determine appropriate management, particularly lung transplantation.
کلیدواژه‌ها
Hypertension, Pulmonary؛ Familial primary pulmonary hypertension؛ Lung transplantation
سایر فایل های مرتبط با مقاله
مراجع
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