Pulmoner Hipertansiyonlu Hastalarımızın Klinik, Epidemiyolojik ve Hemodinamik Verilerinin Değerlendirilmesi

Yıl 2018, Cilt: 16 Sayı: 1, 19 - 28, 01.04.2018

Dolunay Gürses Özlem Gül

Öz

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

Kaynakça

• 1. Ivy DD, Abman SH, Barst RJ, Bonnet D, Fleming TR, Haworth SG, et al. Pediatric pulmonary hypertension. J Am Coll Cardiol 2013;62(25):117-26.
• 2. Simonneau G,Gatzoulis MA, Adatia I, Celermajer D, Denton C, Ghofrani A, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2013;62(25):34-41.
• 3. Olguntürk R. Pediatrik pulmoner hipertansiyon ve doğumsal kalp hastalıklarına bağlı pulmoner arteriyel hipertansiyon. Anadolu Kardiyol Derg 2010; Özel Sayı 1:50-6.
• 4. Abman SH, Hansmann G, Archer SL, Ivy DD, Adatia I, Chung WK, et al. Pediatric Pulmonary Hypertension: Guidelines from the American Heart Association and American Thoracic Society. Circulation 2015;132(21):2037-99.
• 5. Özben B, Başaran Y. Pulmoner arteriyel hipertansiyonda ekokardiyografi ve diğer görüntüleme yöntemleri. Anadolu Kardiyol Derg 2010;10 (1):27-35.
• 6. Hansmann G, Apitz C, Abdul-Khaliq H, Alastalo TP, Beerbaum P, Bonnet D, et al. Executive summary. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK. Heart 2016;102(Suppl. 2):ii86-ii100.
• 7. Chen IC, Dai ZK. Insight into pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD): Classification and pharmacological management from a pediatric cardiological point of view. Acta Cardiol Sin 2015;31(6):507-15.
• 8. Humpl T, Ingram SN. Pulmonary vascular disease. In Anderson R, Baker EJ, Penny D, Redington AN, Rigby ML, Wernovsky G (eds). Pediatric Cardiology. 3rd edition. Churchill Livingstone Publication; 2010.p.1147.
• 9. Elliott CG, Barst RJ, Seeger W, Porres-Aguilar M, Brown LM, Zamanian RT, et al. World wide physician education and training in pulmonary hypertension: pulmonary vascular disease: the global perspective. Chest 2010;137(6):85-94.
• 10. Gatzoulis MA, Alonso-Gonzalez R, Beghetti M. Pulmonary arterial hypertension in paediatric and adult patients with congenital heart disease. Eur Respir Rev 2009;18(113):154-61.
• 11. Galiè N, Beghetti M, Gatzoulis MA, Granton J, Berger RM, Lauer A, et al. Bosentan Randomized Trial of Endothelin Antagonist Therapy-5 (BREATHE-5) Investigators. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation 2006;114(1):48-54.
• 12. Yıldız M, Sunar H, Esen AM. Treatment strategies of pulmonary arterial hypertension associated with congenital heart defects with shunt: A group of important intensive care patient in cardiology and cardiovascular surgery. Kosuyolu Kalp Derg 2013;16(3):173-6.
• 13. Rosenzweig EB, Kerstein D, Barst RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 1999;99(14):1858-65.
• 14. Galie N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J 2016;37(1):67-119.