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UPFRONT TRIPLE THERAPY IN A PATIENT WITH SEVERE IDIOPATHIC PULMONARY ARTERIAL HYPERTENSION – A CASE REPORT

Idiopathic pulmonary arterial hypertension (IPAH) is a chronic lung disorder characterized by impaired function of pulmonary circulation due to an increase in pulmonary vascular resistance and chronically increased right ventricular afterload. Although a rare condition, it is more common in a young adult woman. A 42-year-old woman, ex-smoker (10 pack-years) with a history of arterial hypertension and gestational diabetes during the third pregnancy, reported to our clinic for further evaluation of worsening dyspnea, peripheral edemas, and syncope. At presentation, her functional class (FC) according to the New York Heart Association (NYHA) was III/IV, she was tachypneic and tachycardic with a split-second heart sound with an accentuated pulmonary component, systolic murmur at Erb's point, and bilateral peripheral edemas. Her medications included amlodipine, acetylsalicylic acid, spironolactone, furosemide, and risperidone. An initial diagnostic test revealed type I respiratory insufficiency, enlarged right heart and pulmonary arteries on chest X-ray, and increased levels of proBNP. Initial suspicion of pulmonary arterial hypertension was confirmed by transthoracic echocardiography and right heart catheterization, while extensive workup yielded the final diagnosis of severe (mPAP 74mmHg) IPAH irreversible to selective pulmonary vasodilator. Given the signs of right heart failure (reduced systolic function, high central venous pressure (CVP) with ascites and pericardial effusion, increased levels of proBNP), and a poor functional status (only 250 meters in 6-minute walk test (6MWT) with a saturation drop of 13% despite supplemental oxygen therapy), we opted for upfront triple therapy with sildenafil, macitentan and treprostinil, together with anticoagulation and oxygen therapy (3L/min). Over the course of the next three months, the patient's clinical status greatly improved. She is now NYHA II, respiratory sufficient and normoxemic, proBNP levels decreased from 7719 ng/L to 2134 ng/L, and 6MWT increased to 460 m (without significant saturation drop). The systolic function of the right heart and CVP normalized, thus postponing the need for lung transplantation. Patients with severe PAH and NYHA III/IV or IV FC are at great risk of dying if not treated immediately. Our therapy approach is in line with the growing amount of published evidence about the long-term benefits of upfront triple therapy in severe PAH.

Idiopathic pulmonary arterial hypertension

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