Journals / Anadolu Kardiyoloji Dergisi / 2008 / Cilt: 8 - Sayı: 4

Seven years of experience in patients with pulmonary arterial hypertension in Ege University Hospital: diagnostic approach of a single center

Ege Üniversitesi Hastanesi’nde pulmoner arteryel hipertansiyonlu hastalarla 7 yıllık deneyimimiz: Tek merkezin tanısal yaklaşımı

Pages
279–285
Publication Date
2022-10-10
DOI
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AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

There is also an urgent need for an epidemiological registry in order to determine the burden of PAH in Turkey and increase the awareness of doctors.

📌 3 Key Takeaways

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Objective & Hypothesis:Objective: Pulmonary arterial hypertension (PAH) is a term used to define a variety of progressive conditions that have in common, increased pulmonary vascular resistance leading to right heart failure and death.
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Key Findings & Data:There has been considerable decrease in mortality and morbidity with the advances in PAH treatment over the past decade.
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Conclusion & Impact:There is also an urgent need for an epidemiological registry in order to determine the burden of PAH in Turkey and increase the awareness of doctors.
🔬Design:Research Article
📍Scope:2000 Verileri
⚙️Variables:Domain Parameters

Abstract

Objective: Pulmonary arterial hypertension (PAH) is a term used to define a variety of progressive conditions that have in common, increased pulmonary vascular resistance leading to right heart failure and death. There has been considerable decrease in mortality and morbidity with the advances in PAH treatment over the past decade. However, since there is no epidemiologic study in Turkey, the prevalence of PAH and its importance is not known yet. This study aimed to evaluate the diagnostic clinical experience of Ege University Medical School Cardiology Department with PAH patients.Methods: We evaluated the diagnostic approach to patients referred to our department with the diagnosis of PAH since 2000 by retrospective analysis method. Results: The diagnosis of pulmonary hypertension was definite in 70 patients (mean age 47±16 years, 61% women). Etiology from mostprevalent to least was as following: congenital heart diseases (27%), chronic thromboembolic pulmonary hypertension (24%), connectivetissue diseases-scleroderma (14%), idiopathic PAH (8%), diastolic dysfunction (3%), pulmonary disease (3%), pulmonary veno-occlusivedisease (2%), hepatopulmonary hypertension (1%), and HIV-infection associated PAH (1%). At diagnosis, 68% of patients were in NYHAfunctional class-III or IV. Six-minute walk test was 263±127m. Mean pulmonary artery pressure was 65±20 mmHg. The prognostic markerpro-BNP (brain natriuretic peptid) level was 3208±4145 pg/ml. Conclusion: Our practice shows that PAH is diagnosed late in the course of the disease in Turkey. This can be overcome with structured management in designated centers with multidisciplinary team-working in a shared care approach. There is also an urgent need for an epidemiological registry in order to determine the burden of PAH in Turkey and increase the awareness of doctors.

Özet

Amaç: Pulmoner arteryel hipertansiyon (PAH), artmış pulmoner vasküler direnç nedeniyle sağ kalp yetersizliği ve ölüme yol açan ilerleyici bir grup hastalık için kullanılan genel bir terimdir. Son yıllarda PAH tedavisindeki gelişmeler ile PAH’da morbidite ve mortalitede önemli azalma sağlanmıştır. Ancak, ülkemizde herhangi bir epidemiyolojik çalışma olmaması nedeniyle Türkiye’deki PAH sıklığı, nedenleri ve olayın boyutu bilinmemektedir. Bu yazıda Ege Üniversitesi Tıp Fakültesi kardiyoloji Anabilim Dalı’nın PAH olgularıyla ilgili klinik tanısal deneyiminin aktarılması amaçlanmıştır. Yöntemler: Bu çalışmada 2000 yılından bu yana PAH ön tanısı ile kliniğimize sevk edilen 79 hastanın tanısal yaklaşımları retrospektif analiz yöntemiyle değerlendirilmiştir. Bulgular: Pulmoner hipertansiyon tanısı kesinleşen 70 hastanın yaş ortalaması 47±16 yıl olup %61’i kadındı. Olgularımızdaki pulmoner hipertansiyon nedenleri en sıktan aza göre sırasıyla doğuştan kalp hastalıkları (%27), kronik trombo-embolik pulmoner hipertansiyon (%24), bağ dokusu hastalıkları (skleroderma) (%14), idiyopatik PAH (%8), diyastolik disfonksiyona bağlı pulmoner hipertansiyon (%3), akciğer patolojileri (%3), pulmoner veno-oklüzif hastalık (%2), hepatopulmoner hipertansiyon (%1) ve HİV enfeksiyonuydu (%1). Tanı sırasında olguların %68’i NYHA fonksiyonel sınıflaması III veya IV'teydi. Ortalama 6dk. yürüme mesafesi 263±127'm idi. Ortalama pulmoner arter basıncı 65±20 mmHg idi. Prognostik bir belirteç olan pro-BNP (beyin natriüretik peptid) düzeyi ortalama 3208±4145 pg/ml idi.Sonuç: Pulmoner arteryel hipertansiyon hastaları ile deneyimimiz, Türkiye’de PAH tanısının hastalığın ileri evrelerinde konduğunu göstermektedir. Bu ancak hekim ve hasta bilincinin artması ile birlikte ileri tanısal yaklaşım ve tedavinin mutlaka özelleşmiş merkezlerde takım çalışmasıyla gerçekleştirilmesi ile yenilebilir. Ayrıca, Türkiye’de PAH yükünü saptamak ve hekimlerin pulmoner hipertansiyon konusundaki farkındalıklarını artırmak amacıyla mutlaka epidemiyolojik araştırmalara gereksinim vardır.

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Cite This Article

KAYIKÇIOĞLU, M., KÜLTÜRSAY, H. (2022). Seven years of experience in patients with pulmonary arterial hypertension in Ege University Hospital: diagnostic approach of a single center. Anadolu Kardiyoloji Dergisi, Cilt: 8 - Sayı: 4, 279–285. https://ajindex.com/articles/436997

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References (15)

  1. Rubin LJ. Pathology and pathophysiology of primary pulmonary hypertension. Am J Cardiol 1995; 75 (Suppl): 51A-4
  2. Rubin LJ, Badesch DB. Evaluation and management of the patient with pulmonary arterial hypertension. Ann Intern Med 2005; 143: 282-92
  3. Rubin LJ. Primary pulmonary hypertension. Practical therapeutic recommendations. Drugs 1992; 43: 37-43
  4. D'Alonzo GE, Barst RJ, Ayres SM, Bergofsky EH, Brundage BH, Detre KM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 1991; 115: 343-9
  5. Humbert M, Nunes H, Sitbon O, Parent F, Hervé P, Simonneau G. Risk factors for pulmonary arterial hypertension Clin Chest Med 2001; 22: 459-75
  6. Lilienfeld DE, Rubin LJ. Mortality from primary pulmonary hypertension in the United States, 1979-1996. Chest. 2000; 117: 796-800
  7. Humbert M, Sitbon O, Chaouat A, Bertocchi M, Habib G, Gressin V, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med 2006; 173: 1023-30
  8. Kayıkçıoğlu M, Can LH, Payzin S, Kültürsay H, Soydan I. The combined use of sildenafil with epoprostenol in a patient with primary pulmonary hypertension Anadolu Kardiyol Derg 2002; 2: 262-4
  9. Sayın T, Özenci M. Sustained long-term benefit of sildenafil in primary pulmonary hypertension. Acta Cardiol 2007; 62: 47-9
  10. Önen ZP, Akkoca Yıldız O, Eriş Gülbay B, Karabıyıkoğlu G. Inhaled iloprost as a long-term additional therapy to oral sildenafil in severe idiopathic pulmonary arterial hypertension. Tuberk Toraks 2006; 54: 177-81
  11. Okyay K, Cemri M, Boyacı B, Yalçın R, Çengel A. Use of long-term combined therapy with inhaled iloprost and oral sildenafil in an adult patient with Eisenmenger syndrome. Cardiol Rev 2005; 13: 312-4
  12. Saygılı A, Canter B, İriz E, Kula S, Tunaoğlu FS, Olguntürk R, et al. Use of sildenafil with inhaled nitric oxide in the management of severe pulmonary hypertension. J Cardiothorac Vasc Anesth 2004; 18: 775-6
  13. Sayın T, Zenci M. Sildenafil in primary pulmonary hypertension-is there a subset of patients who respond favourably? Can J Cardiol 2002; 18: 676-8
  14. Galiè N, Torbicki A, Barst R, Dartevelle P, Haworth S, Higenbottam T, et al. Task Force. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology. Eur Heart J 2004; 25: 2243-78
  15. Simonneau G, G