Long-term Use of Sildenafil in Inoperable Chronic Thromboembolic Pulmonary Hypertension

Suntharalingam, Jay; Treacy, Carinei M.; Doughty, Natalie; Goldsmith, Kimberley; Soon, Elaine; Toshner, Mark; Sheares, Karen; Hughes, Rodney; Morrell, Nicholas W.; Pepke‐Zaba, Joanna

doi:10.1378/chest.07-2681

Cited by 226 publications

(169 citation statements)

References 33 publications

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“…Fosfodiesteraz 5 inhibitörleri: İnoperabl KTEPH'lu küçük bir hasta sayısını içeren çalışmada 12 haftanın sonunda yapılan değerlendirmede sildenafil ile plasebo arasında egzersiz kapasitesi açısından fark bulunmazken, fonksiyonel kapasite ve pulmoner vasküler direnç açısından anlamlı iyileşme saptanmıştır (37). Çalışmanın açık etiketli sildenafil ile devam edilen bölümünde ise 12 aylık takip sonunda 6 dakikalık yürüme testi, aktivite ve semptom sorgulamasında, kardiyak indekste ve pulmoner vasküler dirençte iyileşme görülmüştür.…”

Section: Tıbbi Tedaviunclassified

Chronic thromboembolic pulmonary hypertension: diagnosis, medical therapy and monitoring

Gölbaşı¹

2010

Anadolu Kardiyol Derg

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Chronic thromboembolic pulmonary hypertension (CTEPH) is caused by obstruction of the large pulmonary arteries by acute and recurrent pulmonary emboli with subsequent organization of these blood clots. Because of a lack of clinically evident acute-pulmonary embolism episodes in >50% of patients, the diagnosis of CTEPH can be difficult. Lung ventilation/perfusion scintigraphy showing segmentary mismatched perfusion defects is the best diagnostic tool to detect CTEPH. Although pulmonary endarterectomy is considered the treatment of choice for patients with CTEPH, it can not be performed in a substantial proportion of patients. Medical therapies have been increasingly used in subcategories of CTEPH patients. (Anadolu Kardiyol Derg 2010; 10: Suppl 2; 56-60) Key words: Pulmonary embolism, pulmonary hypertension, chronic thromboembolic pulmonary hypertension ÖZETKronik tromboembolik pulmoner hipertansiyon (KTEPH), akut ve yineleyen pulmoner emboliler ve bunların organize olması sonucu büyük pulmoner arterlerin obstrüksiyonu ile oluşur. Hastaların %50'den fazlasında klinik olarak belirti veren akut pulmoner emboli atağının bulunmayışı sebebiyle KTEPH tanısı zor olabilir. KTEPH'nun tespitinde ventilasyonla uyumsuz segmental perfüzyon defekti bulunan akciğer ventilasyon/perfüzyon sintigrafisi en iyi tanı aracıdır. KTEPH'lu hastaların tedavisinde pulmoner endarterektomi (PEA) en iyi tedavi seçeneği olarak görülmekle birlikte, hastaların önemli bir bölümünde yapılamayabilir. KTEPH'lu hastaların bazı alt gruplarında tıbbi tedavi giderek daha artan oranda kullanılmaktadır.

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Section: Tıbbi Tedaviunclassified

Chronic thromboembolic pulmonary hypertension: diagnosis, medical therapy and monitoring

Gölbaşı¹

2010

Anadolu Kardiyol Derg

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“…The three randomised, controlled trials in patients with CTEPH performed to date have used a prostanoid (iloprost) [33], a phosphodiesterase-5 inhibitor (sildenafil) [34] and an endothelin-receptor antagonist (bosentan) [35]. Iloprost, 2.5 or 5.0 mg, inhaled six to nine times daily (median dose 30 mg?day -1 ) for 12 weeks, was compared with placebo for a population of 203 patients that included 57 patients with inoperable CTEPH at baseline [33].…”

Section: Therapeutic Options For Cteph Current Conventional Treatmentsmentioning

confidence: 99%

“…However, results were analysed according to type of PH (labelled as primary versus nonprimary), and these data showed that patients with nonprimary PH and primary PH experienced similar improvements in Mahler dyspnoea score and health-related quality of life in response to treatment with iloprost. A double-blind, placebo-controlled, 12-week pilot study has investigated the use of sildenafil, 40 mg three times daily, in 19 patients with inoperable CTEPH [34]. Unfortunately, this study was inadequately powered to test the primary end-point (change in 6-min walk distance).…”

Section: Therapeutic Options For Cteph Current Conventional Treatmentsmentioning

confidence: 99%

Managing chronic thromboembolic pulmonary hypertension: pharmacological treatment options

Lang¹

2009

European Respiratory Review

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Chronic thromboembolic pulmonary hypertension (CTEPH) is a life-threatening condition in which organised thrombi obstruct the pulmonary vessels, causing increased pulmonary vascular resistance, progressive pulmonary hypertension (PH) and right heart failure. The treatment of choice is pulmonary endarterectomy, which restores pulmonary haemodynamics with acceptable periprocedural mortality rates in the majority of suitable patients. However, CTEPH may be inoperable owing to surgically inaccessible thrombi or comorbid diseases that confer an unacceptably high risk.Pharmacotherapies, although not yet approved, may be useful in this situation or for treating residual or recurrent PH following surgery. Vasodilator drugs for PH are attracting growing interest as potential treatments for CTEPH because this disease has recently been labelled as a ''dual'' pulmonary vascular disorder: major vessel obstruction and remodelling is combined with a small vessel arteriopathy that is histologically indistinguishable from the classical pulmonary arteriopathy observed in pulmonary arterial hypertension.Of three completed randomised controlled trials in patients with CTEPH, only one was powered to detect a treatment effect. The BENEFIT trial employed the dual endothelin-receptor antagonist bosentan. Although haemodynamics improved significantly, the second component of the primary end-point, exercise capacity, was not met.More evidence is required to resolve whether vasodilator treatments are beneficial for inoperable chronic thromboembolic pulmonary hypertension.

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“…[15,16] While PTEA is the only option that provides an immediate and permanent cure for this devastating disease, it is rarely performed today. [9,10,15,17,18] Although currently there is an unwillingness to perform this troublesome surgical procedure, the aim of this article is to present PTEA as a viable alternative for selected patients with low functional capacity in which an obvious improvement in hemodynamic compromise is possible.…”

mentioning

confidence: 99%

Pulmonary embolectomy and thromboendarterectomy in seven cases

Hazan¹,

Şişli²,

Uğurlu³

et al. 2012

Turk Gogus Kalp Dama

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Amaç: Bu çalışmanın amacı, akut pulmoner emboli (APE) ve kronik tromboembolik pulmoner hipertansiyon (KTPHT) olgularının cerrahi ile belirgin olarak düzeldiklerini öngören kanıta dayalı veriler temelinde cerrahi sonrası seyir ile ilgili belirsizliği azaltmak ve bu olgularda cerrahi tedavi seçeneklerini dikkate alan doktorları cesaretlendirmektir. Ça lış ma pla nı: Bu gözlemsel ve geriye dönük gerçekleştirilen çalışmaya Şubat 2009 ile Ekim 2010 tarihleri arasında, APE ve KTPHT tanısı ile ameliyat edilen yedi olgu (3 erkek, 4 kadın; ort yaş 43.8±18.5 yıl; dağılım 25-73 yıl) dahil edildi. Hastaların ameliyat öncesi ve sonrası ortalama pulmoner arter basıncı (mPAP), New York Heart Association (NYHA) fonksiyonel sınıfı, yoğun bakım ünitesi (YBÜ) ve hastanede kalış süresi, demografik, klinik ve ameliyat özellikleri kaydedildi. Olgu sayısının yetersizliğinden dolayı istatistiksel analizler, parametrik olmayan testler ile yapıldı. Grupların karşılaştırılmasında Wilcoxon testi kullanıldı. Bul gu lar: Cerrahi sonrasında bütün hastalarda mPAP'da 20 mmHg (dağılım 5-53) düşüş sağlandı ve altıncı olgu hariç tüm olguların mPAP'ları, 30 mmHg'nın altına çekildi. Ameliyat öncesi ve sonrası mPAP'ları, sırasıyla 43 mmHg (dağılım 33-68) ve 23 mmHg (dağı-lım 15-37) idi. Bu durumda cerrahi sonrasında anlamlı bir düşüş elde edildi (z=-2.36; p=0.018). Buna ilaveten, cerrahi sonrasında hastanın NYHA sınıfındaki iyileşmesi 1 (dağılım 1-3) birimdi. Hastaların ameliyat öncesi ve sonrası NYHA sınıfları sırasıyla III (II-III) ve II (I-II) idi. Ameliyat sonrası NYHA sınıfındaki düşüş ile birlikte cerrahinin iyileştirme üzerine etkisi anlamlıydı (z=-2.26; p= 0.024). Yoğun bakım ünitesi ve hastanede ortalama kalış süreleri, sırasıyla üç (dağılım 2-14) ve 9.5 (dağılım 5-27) gündü. So nuç: Çalışma bulgularımıza göre, ayrıntılı bir ameliyat öncesi değerlendirme ve uygun hasta seçimi ile hekimler, cerrahi seçe-neğin kararında çekimser olmamalı ve hastaların yaşam kalitesini artırmak için çaba harcamalıdır.Anah tar söz cük ler: Akut pulmoner emboli; kronik tromboembolik pulmoner hipertansiyon; fonksiyonel kapasite; ortalama pulmoner arter basıncı; pulmoner tromboendarterektomi.Background:This study aims to reduce the uncertainity related to the prognosis after surgery of the acute pulmonary embolism (APE) and chronic thromboembolic pulmonary hypertension (CTPHT) patients, and to encourage physicians to consider surgical treatment options based on the evidence-based data which suggests that surgery offers the best chance for improvement in these patients. Methods: Between February 2009 and October 2010, seven cases (3 males, 4 females; mean age 43.8±18.5 years; range 25 to 73 years) who were operated on due to APE and CTPHT were included in this observational and retrospective study. The preand postoperative mean pulmonary artery pressure (mPAP), New York Heart Association (NYHA) functional class, and length of stay in the intensive care unit (ICU) and hospital along with the demographic, clinical and operational characteristics of the patients were r...

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Long-term Use of Sildenafil in Inoperable Chronic Thromboembolic Pulmonary Hypertension

Cited by 226 publications

References 33 publications

Chronic thromboembolic pulmonary hypertension: diagnosis, medical therapy and monitoring

Chronic thromboembolic pulmonary hypertension: diagnosis, medical therapy and monitoring

Managing chronic thromboembolic pulmonary hypertension: pharmacological treatment options

Pulmonary embolectomy and thromboendarterectomy in seven cases

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