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Clinical Update on Pulmonary Hypertension J Investig Med: First Published As 10.1136/Jim-2020-001291 on 1 April 2020

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Clinical Update on Pulmonary Hypertension J Investig Med: First Published As 10.1136/Jim-2020-001291 on 1 April 2020 Review Clinical update on pulmonary hypertension J Investig Med: first published as 10.1136/jim-2020-001291 on 1 April 2020. Downloaded from Salim Yaghi, Anastasia Novikov, Theo Trandafirescu Queens Hospital Center, ABStract at least 25 mm Hg was chosen somewhat arbi- Medicine, Mount Sinai Pulmonary hypertension (PH) is a chronic, complex trarily and does not represent the upper limit of Medical Center, Queens, normal mPAP in the general population.2 3 New York, USA and challenging disease. Advances in treatment are for the subset of patients with pulmonary arterial The prior assessment of PAP at exercise is Correspondence to hypertension. Selected review of the literature was not recommended with the current definitions Dr Theo Trandafirescu, conducted incorporating the European Society of due to lack of reliable data that precisely define Queens Hospital Center, Cardiology/European Respiratory Society 2015 which levels of exercise- induced changes in PAP Medicine, Mount Sinai guidelines and recommendations from the Sixth or pulmonary vascular resistance have prog- Medical Center, Queens, nostic implications.2 3 NYC 11432, USA; World Symposium on Pulmonary Hypertension. ttrandaf@ gmail. com PH is classified into five groups based on WHO Hemodynamic definitions of PH which classification. Echocardiography remains the initial include more than just the PAP during right Accepted 6 February 2020 test of choice, and careful assessment of the right heart catheterization (RHC) are probably more system aids in the diagnosis and prognosis of useful clinically as they have prognostic and the disease. Right heart catheterization remains therapeutic implications and can help with the gold standard of diagnosis and key guidance further classification of the disease. The major of treatment. Multidisciplinary approach is players here are pulmonary vascular resis- recommended for the care of patients with PH. tance and pulmonary capillary wedge pressure Treatment selection is based on individual risk (table 1).1–3 stratification of patients, and early referral to specialized PH centers improves outcomes of Classifications patients. Treating PH is complex and is best carried The WHO classification1 2 categorizes multiple out in PH centers and with multidisciplinary clinical conditions into five groups according to approach. Early diagnosis and referral to those similarities in clinical presentation, patholog- centers are key not to delay treatment. ical findings, hemodynamic characteristics and treatment approach. These subtypes of PH are agreed on by the ESC in 2016 and the Sixth World Symposium (box 1 and tables 2–4). INTRODUCTION Pulmonary hypertension (PH) has remained a http://jim.bmj.com/ challenging chronic progressive disease since the Diagnosis First World Symposium meeting in 1973, which ECG findings opened the era to groundbreaking discoveries An ECG may provide supportive evidence of about its pathophysiology and various treatment PH, but a normal ECG does not exclude the options. In this review, we try to summarize the diagnosis.4 An abnormal ECG is more likely in latest evidence about the disease, its definition, severe rather than mild PH. ECG abnormalities classification, diagnosis and treatment, focusing may include P pulmonale, right axis deviation, on October 1, 2021 by guest. Protected copyright. mainly on group 1 pulmonary arterial hyperten- RV hypertrophy, RV strain, right bundle branch sion (PAH). block, and QTc prolongation. Prolongation of the QRS complex and QTc suggests severe 4 Definitions disease. Supraventricular arrhythmias may occur in Pulmonary hypertension is defined as a mean advanced disease, in particular atrial flutter, but pulmonary arterial pressure (mPAP) greater also atrial fibrillation, with a cumulative inci- than 20 mm Hg at rest as per the Sixth World dence of 25% in patients after 5 years.5 Ventric- Symposium on Pulmonary Hypertension in 1 ular arrhythmias are rare. © American Federation for 2018, and greater than 25 mm Hg at rest as per Medical Research 2020. the guidelines issued by the European Society Re- use permitted under of Cardiology (ESC)/European Respiratory Echocardiography CC BY-NC . No commercial 2 re-use . Published by BMJ. Society (ERS) in 2015. A subset of patients Echocardiography remains the first test of with pulmonary arterial pressure (PAP) of choice when PH is suspected,6 not only because To cite: Yaghi S, between 21 and 24 mm Hg are considered high- it estimates systolic PAP but also because it can Novikov A, Trandafirescu T. risk patients with possible poor outcomes if left assess for signs of right ventricular (RV) dysfunc- J Investig Med Epub 7 ahead of print: [please unfollowed, which is why they were included in tion as well as left ventricular (LV) dysfunction. 1 include Day Month Year]. the Sixth World Symposium definition. Reading and interpreting echocardiographic doi:10.1136/jim-2020- The authors based this recommendation on imaging in respect to PH and its related 001291 the fact that the original definition of mPAP of effects on the right cardiac side is challenging; Yaghi S, et al. J Investig Med 2020;0:1–7. doi:10.1136/jim-2020-001291 1 Review Table 1 Hemodynamicdefinitions of pulmonary hypertension Box 1 Updated clinical classification of pulmonary J Investig Med: first published as 10.1136/jim-2020-001291 on 1 April 2020. Downloaded from types hypertension (PH) Mean pulmonary artery Pulmonary capillary Pulmonary vascular Definition pressure (mm Hg) wedge pressure (mm Hg) resistance (Wood units) 1. Pulmonary arterial hypertension (PAH). Isolated precapillary PH 20 <15 3 (formerly pulmonary ► Idiopathic. arterial hypertension) Heritable. Combined postcapillary 15 3 ► and precapillary pulmonary ► Drug- induced and toxin- induced (see table 2). hypertension (PH) Isolated postcapillary PH 15 <3 ► Associated with the following: – Connective tissue disease. –P ortal hypertension. – Congenital heart disease. therefore, the ESC guidelines issued a probability score for – Schistosomiasis. PH based on echocardiographic features. ► PAH long- term responders to calcium channel blockers The ESC/ERS and the Sixth World Symposium do not (see table 3). recommend using estimated systolic PAP anymore given ► PAH with overt features of pulmonary veno- occlusive inaccuracies of right atrial pressure (RAP) estimation and disease/or pulmonary capillary hemangiomatosis (see the amplification of measurement errors using derived vari- table 4). ables; thus, continuous-wave Doppler measurement of peak ► Persistent PH of the newborn syndrome. tricuspid regurgitation velocity (TRV) is the main variable 2. Pulmonary hypertension due to left heart disease. 1 2 8 9 for assigning echocardiographic probability of PH ► PH due to heart failure with preserved left ventricular (tables 5 and 6). ejection fraction. The algorithm in figure 1 summarizes how to use ► PH due to heart failure with reduced left ventricular echocardiography and the probability score for PH to ejection fraction. determine further testing and whether RHC for defin- ► Valvular heart disease. itive diagnosis is indicated. It can be said that patients ► Congenital/acquired cardiovascular conditions leading with symptoms plus intermediate or high echocardio- to postcapillary PH. graphic probability score should go for RHC, and only 3. Pulmonary hypertension due to lung disease and/ asymptomatic patients with high probability should go or hypoxia. for RHC; otherwise, follow- up with repeat echocardiog- ► Obstructive pulmonary diseases. raphy is reasonable (figure 1). ► Restrictive pulmonary diseases. ► Other lung disease with mixed restrictive/obstructive Cardiac MRI pattern. Cardiac MRI (CMR) is accurate and reproducible in the ► Hypoxia without lung disease. assessment of RV size, morphology and function, and ► Developmental lung disorders. allows non- invasive assessment of blood flow, including 4. Pulmonary hypertension due to pulmonary artery http://jim.bmj.com/ stroke volume, cardiac output (CO), pulmonary arterial obstruction. distensibility and RV mass.10 ► Chronic thromboembolic PH. In patients with suspected PH, the presence of late gado- ► Other pulmonary artery obstructions. linium enhancement, reduced pulmonary arterial distensi- – Sarcoma (high or intermediate grade) or bility and retrograde flow has high predictive value in the angiosarcoma. identification of PH; however, no single CMR measure- – Other malignant tumors (renal, uterine, germ cell on October 1, 2021 by guest. Protected copyright. ment can exclude PH.2 In patients with PH, CMR may also tumor of the testis, other tumors). be useful in cases with suspected congenital heart disease if – Non-malignant tumors (uterine leiomyoma). echocardiography is not conclusive. – Arteritis without connective tissue disease. Contrast- enhanced and unenhanced magnetic resonance – Congenital pulmonary arteries stenoses. angiography has potential in the study of the pulmonary –P arasites (hydatidosis). vasculature in patients with suspected chronic thromboem- 5.Pulmonary hypertension with unclear and/or bolic PH, particularly in clinical scenarios such as suspected multifactorial mechanisms. chronic embolism in pregnant women, young patients or ► Hematological disorders: chronic hemolytic anemia and when iodine- based contrast media injection is contraindi- myeloproliferative disorders. cated.2 10 ► Systemic and metabolic disorders: pulmonary CMR provides useful prognostic information for patients Langerhans cell histiocytosis, Gaucher disease, glycogen with PAH
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