Pulmoner Hipertansiyonlu Hastalarımızın Klinik, Epidemiyolojik ve Hemodinamik Verilerinin Değerlendirilmesi

GİRİŞ ve AMAÇ: Bu çalışma, pulmoner arteriyel hipertansiyon (PAH) tanılı hastalarımızın epidemiyolojik özelliklerinin, yaşam kalitelerinin, tedavi yöntemlerinin ve etkinliğinin belirlenmesi amacıyla yapıldı. YÖNTEM ve GEREÇLER: Ocak 2008-Aralık 2016 tarihleri arasında Çocuk Kardiyoloji bölümünde PAH nedeniyle izlenen 15 hasta (11 kız, 4 erkek) retrospektif olarak değerlendirildi. BULGULAR: Hastaların %73’ü kız, %27’si erkekti. İlk başvuru sırasındaki ortalama yaşları 10,7±5 (3-20) yıldı. Hastaların sadece birinde (%7) primer PAH, iki olguda (%13) kronik akciğer hastalığına sekonder PAH mevcuttu. On iki hastada (%80) doğumsal kalp hastalığına sekonder PAH saptandı. Bu hastalardan 10 tanesi Eisenmenger sendromuyken, iki hastada kapatılmış sol-sağ şantlı kalp hastalığı mevcuttu. Eisenmenger sendromu olan 10 hastanın altısında (%60) VSD, ikisinde (%20) atriyoventriküler septal defekt mevcuttu. En sık görülen semptom çabuk yorulma (%83) idi. Hastaların tamamına telekardiyografi, elektrokardiyogram (EKG) ve ekokardiyografi yapılmış olup, %87’sine altı dakika yürüme testi ve anjiyografi yapılmıştı. Anjiyografi yapılan hastaların hepsine vazoreaktivite testi yapılmıştı ve hepsinde negatif saptanmıştı. Hastaların son kontrollerindeki tedavileri değerlendirildiğinde; %53,3’ünün monoterapi, %46,7’sinin kombine tedavi aldığı görüldü. En sık başlanan monoterapi bosentandı (%46,7). Dört hastamız izlemde kaybedilmişti. TARTIŞMA ve SONUÇ: Pulmoner arteriyel hipertansiyon birçok etiyolojik nedene bağlı oluşabilen kronik ilerleyici bir hastalıktır. Tedavi edilmezse; pulmoner vasküler rezistansta progresif artış sonucunda sağ ventrikül yetmezliği ve erken ölüme neden olur. PAH şüphesi olan hastaların deneyimli merkezlere yönlendirilmeleri, uygun tanı, takip ve tedavi seçeneklerinin kullanılmasını sağlayacaktır.ABSTRACT INTRODUCTION: This study was conducted to determine the epidemiological characteristics, quality of life, treatment modalities and efficacy of our treatment in patients with pulmonary arterial hypertension (PAH). METHODS: Fifteen patients (11 girls, 4 boys) who were followed up for PAH in the Department of Pediatric Cardiology between January 2008 and December 2016 were evaluated retrospectively. RESULTS: The mean age of patients at first admission was 10.7±5 (3-20) years. Only one of the patients (7%) had primary PAH, and two patients (13%) had PAH associated with chronic lung disease. Twelve patients (80%) had PAH secondary to congenital heart disease. Of these patients, 10 had Eisenmenger syndrome and two were operated for congenital heart disease with a left to right shunt. Six patients (60%) had VSD, and two (20%) had atrioventricular septal defect in 10 patients with Eisenmenger syndrome. The most common symptom was easy fatigue (83%). All of the patients had telecardiography, electrocardiogram and echocardiography, and 87% of patients underwent six minute walking test and angiography. All patients undergoing angiography were tested for vasoreactivity and all were negative. When the treatments at the last controls of the patients are evaluated; 53.3% of the patients received monotherapy, 46.7% received combination therapy. The most frequently started monotherapy was bosentan (46.7%). Four patients were died during follow-up. DISCUSSION and CONCLUSION: Pulmonary arterial hypertension is a chronic progressive disease that can be caused by many etiologic factors. If not treated; progressive increase in pulmonary vascular resistance leads to right ventricular failure and premature death. Accumulating PAH patients in experienced centers will ensure appropriate diagnosis, follow-up and treatment options. Keywords: Eisenmenger syndrome, pulmonary hypertension, treatment

Anahtar Kelimeler:

: Eisenmenger sendromu, pulmoner hipertansiyon

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