Λaθη και παγiδεΣ Στη διaγνωΣη τηΣ ΠΑΥ Ηρακλής Τσαγκάρης Αναπληρωτής Καθηγητής Εντατικής Θεραπείας EKΠΑ Αττικό Νοσοκομείο

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Τι από τα παρακάτω είναι σωστό? H διάγνωση της ΠΑΥ οφείλει να γίνεται σε ειδικά κέντρα Η συνιστώμενη μέθοδος μέτρησης της καρδιακής παροχής είναι η έμμεση μέθοδος Fick Η δοκιμασία αγγειοδραστικότητας γίνεται πάντοτε κατά τη διάγνωση της ΠΑΥ H δοκιμασία χορήγησης υγρών (500ml NS) γίνεται σε 15-20 min, προκειμένου να ελαχιστοποιηθεί η πιθανότητα πνευμονικού οιδήματος Πίεση ενσφήνωσης<15mmHg αποκλείει τη διάγνωση της PH από αριστερή καρδιακή νόσο

Τι από τα παρακάτω είναι σωστό? H διάγνωση της ΠΑΥ οφείλει να γίνεται σε ειδικά κέντρα Η συνιστώμενη μέθοδος μέτρησης της καρδιακής παροχής είναι η έμμεση μέθοδος Fick Η δοκιμασία αγγειοδραστικότητας γίνεται πάντοτε κατά τη διάγνωση της ΠΑΥ H δοκιμασία χορήγησης υγρών (500ml NS) γίνεται σε 15-20 min, προκειμένου να ελαχιστοποιηθεί η πιθανότητα πνευμονικού οιδήματος Πίεση ενσφήνωσης<15mmHg αποκλείει τη διάγνωση της PH από αριστερή καρδιακή νόσο

Septal lines and centrilobular ground-glass opacities Latero-aortic lymphadenopathy

The definition of a ‘positive’ response is controversial The definition of a ‘positive’ response is controversial. According to the ECS, a positive acute vasodilator response requires both a fall of mean PAP of 10 mmHg or greater and final mean PAP of 40 mmHg or less, with no decrease in cardiac output [3]. We believe that this definition is insensitive, and would miss the many patients who respond with a 20% fall in PVR or mean PAP, but whose mean PAP remains above 40 mmHg. As pulmonary vasoconstriction is usually not the major determinant of PVR, a dramatic decrease in mean PAP in response to acute vasodilators is quite rare. Because the ‘responders’ have a much better prognosis when treated with calcium antagonists the ECS definition would disqualify many PAH patients from the simple and cheap calcium antagonist therapy [

Vasoreactivity testing performed during right heart catheterization is recommended in patients with idiopathic PAH, inherited PAH, and PAH induced by drugs or toxin use, to detect patients who can be treated with high doses of a calcium-channel blocker.

Comorbidities Badesh DB, CHEST 2010;137;376-87

mPAP>25, W≤15 Typical (< 3 risk factors for left heart disease) mPAP>25, W≤15 Atypical (≥ 3 risk factors for left heart disease) Arterial hypertension Coronary artery dis Diabetes Atrial fibrillation BMI>30 kg/m2 HEFpEF mPAP>25, W>15 EF>45% Diastolic dysfunction

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<65 yo >65 yo Female 69% 45% Systemic hypertensiom 34% 58% Diabetes mellitus 9% 31% Atrial fibrillation 5% 23% History of stroke 1% 10% Coronary artery disease 26% Radegran, Scand J Cardiol, 2016

Is it PAH with comorbidities or is it PH-HFpEF with ‘pseudonormal’ PAWP? 75yo female Obesity (BMI 32), DM II, hypertension, CAD Atrial fibrillation Echo shows normal LVEF but signs of diastolic dysfunction, LA is dilated RHC: RA 11mmHg, PAWP 12, mPAP 48, CI 2.6, PVR 8WU

PAH in elderly patients – Why are so many male patients affected? Is there a smoking-related pulmonary vasculopathy presenting as a vanishing capillary syndrome?

Loss of pulmonary capillaries due to apoptosis? We report in an emphysema model of mice chronically exposed to tobacco smoke that pulmonary vascular dysfunction, vascular remodeling, and pulmonary hypertension (PH) precede development of alveolar destruction.

PAH with a low DLCO

PAH with a low DLCO

PAH with a low DLCO yo ♂ 19% Smoking 48% 67 yo ♂ 50% Smoking 77%

Right Heart Cath RHC is a technically demanding procedure that requires meticulous attention to detail to obtain clinically useful information. To obtain high-quality results and to be of low risk to patients, the procedure should be limited to expert centres. ESC/ERS guidelines 2015

Pre and periprocedural assesment and care Ensure that full work up before cath and all pertinent data are available and reviewed before the procedure Optimize volume status Review medications Avoid sedation if possible Oxygen therapy

Best practice recommendations for right heart catheterisation: pressure transducer and zeroing [3, 20]. The joint task force of the European Society of Cardiology and the European Respiratory Society recommends setting the pressure transducer to zero at the mid-thoracic line (with a suggested reference point defined by the intersection of the frontal plane at the mid-thoracic level, the transverse plane at the level of fourth anterior intercostal space, and the midsagittal plane [20]) in a supine patient halfway between the anterior sternum and the bed surface [4]. Reproduced from [20] with permission from the publisher. Kovacs G, Avian A, Pienn M, et al. Reading pulmonary vascular pressure tracings. How to handle the problems of zero leveling and respiratory swings. Am J Respir Crit Care Med 2014; 190: 252–257.

The joint task force of the European Society of Cardiology and the European Respiratory Society recommends setting the pressure transducer to zero at the mid-thoracic line (with a suggested reference point defined by the intersection of the frontal plane at the mid-thoracic level, the transverse plane at the level of fourth anterior intercostal space, and the midsagittal plane) in a supine patient halfway between the anterior sternum and the bed surface.

PAH or LHD-PH?

J Cardiac Failure, 2016

PAH or HEFpEF?

Dynamic testing for the identification of HF-PH Fluid loading Fluid challenge with 7 ml/kg NS increases PAWP, more in post-capillary PH(+7) than in pre-capillary PH(+3) or no-PH. A cut-off 18 mmHg allows to re-classify 6-8% of pts with pre-capillary PH or normal hemodynamics at baseline. D’Alto, Chest, 2016

Dynamic testing for the identification of HF-PH Exercise-induced PH (EIPH) mPAP >30 mmHg at a CO >10 L/min, or maximum total PVR >3 WU, measured either invasively or noninvasively The limits of normal of exercise PAWP or VEDP have not yet been defined with certainty Naeije, Progr Card Dis, 2016

Gray zones PH 21-24 mm Hg High risk populations Genetic factors

In conclusion PH is a dynamic multifaceted disease The phenotypic profiles of PH patients become increasingly complex Referral to PH-centers facilitates proper diagnosis and proper management

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