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Interventional and Surgical Treatments for Pulmonary Arterial Hypertension

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Interventional and Surgical Treatments for Pulmonary Arterial Hypertension Journal of Clinical Medicine Review Interventional and Surgical Treatments for Pulmonary Arterial Hypertension Tomasz St ˛acel 1,* , Magdalena Latos 1, Maciej Urlik 1, Mirosław N˛ecki 1, Remigiusz Anto ´nczyk 1, Tomasz Hrapkowicz 1, Marcin Kurzyna 2 and Marek Ochman 1,* 1 Silesian Centre for Heart Diseases in Zabrze, Department of Cardiac, Vascular and Endovascular Surgery and Transplantology, Medical University of Silesia, 40-055 Katowice, Poland; [email protected] (M.L.); [email protected] (M.U.); [email protected] (M.N.); [email protected] (R.A.); [email protected] (T.H.) 2 European Health Centre Otwock, Centre of Postgraduate Medical Education, Department of Pulmonary Circulation, Thromboembolic Diseases and Cardiology, 05-400 Otwock, Poland; [email protected] * Correspondence: [email protected] (T.S.); [email protected] (M.O.); Tel.: +48-691-045-785 (T.S.); +48-60-923-4437 (M.O.) Abstract: Despite significant advancements in pharmacological treatment, interventional and sur- gical options are still viable treatments for patients with pulmonary arterial hypertension (PAH), particularly idiopathic PAH. Herein, we review the interventional and surgical treatments for PAH. Atrial septostomy and the Potts shunt can be useful bridging tools for lung transplantation (Ltx), which remains the final surgical treatment among patients who are refractory to any other kind of therapy. Veno-arterial extracorporeal membrane oxygenation (V-A ECMO) remains the ultimate Citation: St ˛acel,T.; Latos, M.; bridging therapy for patients with severe PAH. More importantly, VA-ECMO plays a crucial role Urlik, M.; N˛ecki,M.; Anto´nczyk,R.; during Ltx and provides necessary left ventricular conditioning during the initial postoperative Hrapkowicz, T.; Kurzyna, M.; period. Pulmonary denervation may potentially be a new way to ensure better transplant-free Ochman, M. Interventional and survival among patients with the aforementioned disease. However, high-quality randomized con- Surgical Treatments for Pulmonary trolled trials are needed. As established, obtaining the Eisenmenger physiology among patients with Arterial Hypertension. J. Clin. Med. severe pulmonary hypertension by creating artificial defects is associated with improved survival. 2021, 10, 3326. https://doi.org/ However, right-to-left shunting may be harmful after Ltx. Closure of the artificially created defects 10.3390/jcm10153326 may carry some risk associated with cardiac surgery, especially among patients with Potts shunts. In Academic Editor: conclusion, PAH requires an interdisciplinary approach using pharmacological, interventional, and Sukhwinder Singh Sohal surgical modalities. Received: 5 July 2021 Keywords: pulmonary arterial hypertension; interdisciplinary approach; review; idiopathic pul- Accepted: 27 July 2021 monary arterial hypertension Published: 28 July 2021 Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in 1. Introduction published maps and institutional affil- Pulmonary arterial hypertension (PAH) is associated with poor prognosis despite iations. recent advancements in pharmacological treatments, as there is no curative therapy [1,2]. Oxygen therapy should be considered in patients who develop hypoxemia [3,4]. Oxygen saturation should be maintained above 90% at rest [4]. Advanced treatment for PAH is directed at the pulmonary arterioles and includes prostanoids, endothelin receptor Copyright: © 2021 by the authors. antagonists, phosphodiesterase 5 inhibitors, soluble guanylate cyclase stimulators, and in Licensee MDPI, Basel, Switzerland. some cases calcium channel blockers. It is believed that no more than 5% of PAH patients This article is an open access article may benefit from calcium channel blockers during long term treatment [5]. Patients with distributed under the terms and idiopathic, heritable, and drug-related PAH should undergo vasoreactivity testing during conditions of the Creative Commons right heart catheterization because such tests facilitate drug selection [3,6]. Despite such Attribution (CC BY) license (https:// treatment, severe pulmonary hypertension can still lead to hemodynamic impairment creativecommons.org/licenses/by/ due to right ventricular (RV) dysfunction. Interventions such as atrial septostomy (AS), 4.0/). J. Clin. Med. 2021, 10, 3326. https://doi.org/10.3390/jcm10153326 https://www.mdpi.com/journal/jcm J. Clin. Med. 2021, 10, x FOR PEER REVIEW 2 of 17 J. Clin. Med. 2021, 10, 3326 2 of 16 bridginguse of the therapy Potts shunt, for thoracic and pulmonary transplantations, artery denervationbeing the ultimate (PADN) surgical could betreatment applied for as suchbridging diseases. therapy Moreover, for thoracic they transplantations,can be used as palliative being the care ultimate tools [7]. surgical treatment for suchDespite diseases. significant Moreover, advancements they can be used in pharma as palliativecological care treatment, tools [7]. interventional and surgicalDespite options significant are still a advancements viable treatment in pharmacologicalfor patients with IPAH treatment, and other interventional diseases from and groupsurgical 1 optionsof pulmonary are still hypertension. a viable treatment Herein, for patientswe review with the IPAH interventional and other diseasesand surgical from treatmentsgroup 1 of for pulmonary PAH. hypertension. Herein, we review the interventional and surgical treatments for PAH. 2. Right-to-Left Shunting 2. Right-to-LeftPulmonary Shuntinghypertension increases vascular resistance to the point of RV systolic dysfunction.Pulmonary The hypertensionleft ventricle increasesin patients vascular with severe resistance forms to of the PAH point can of RVpresent systolic with dys- a properfunction. ejection The left fraction. ventricle However, in patients patholog with severeically formsreduced of PAHpulmonary can present flow withsignificantly a proper decreasesejection fraction. preload, However, which pathologically ultimately reduceddeconditions pulmonary this flowventricle. significantly The decreasesoverall pathophysiologypreload, which ultimately of cardiac deconditions dysfunction this amon ventricle.g patients The overallwith PAH pathophysiology is associated ofwith car- impaireddiac dysfunction systemic among bloodpatients flow, oxygen with PAH transport, is associated and passive with impaired venous blood systemic stasis blood [8]. flow,Due tooxygen these transport,changes, advanced and passive pulmonary venous blood vessel stasis diseases [8]. Duelead to to these multiorgan changes, failure advanced and death.pulmonary At this vessel point, diseases obtaining lead the to multiorganEisenmenger failure physiology and death. among At patients this point, with obtaining severe pulmonarythe Eisenmenger hypertension physiology is the among goal, patientsas it is associated with severe with pulmonary longer life hypertension expectancy is and the moregoal, asstable it is associatedhemodynamics with longer[9]. Procedures life expectancy involving and moreright-to-left stable hemodynamicsshunting provide [9]. decompressionProcedures involving of the right-to-leftright ventricle, shunting and th provideey may decompression increase the systemic of the right blood ventricle, flow throughand they increasing may increase the left the ve systemicntricle’s bloodpreload. flow However, through such increasing treatment the leftwill ventricle’s lead to a decreasepreload. However,in oxygen such saturation; treatment hence, will leadthis tome athod decrease is not in oxygensuitable saturation;for all patients hence, [10]. this Providingmethod is such not suitable treatment for is all well patients studied [10]. amon Providingg children such with treatment idiopathic is well PAH studied (IPAH), among as achildren significant with scarcity idiopathic of donors PAH (IPAH),with matching as a significant anthropometrics scarcity of is donors observed with among matching this groupanthropometrics of potential is thoracic observed organ among recipients this group [11]. ofAs potential the Potts thoracicshunt and organ AS aim recipients to transfer [11]. suprasystemicAs the Potts shunt pulmonary and AS hypertension aim to transfer to Eisenmenger suprasystemic physiology, pulmonary it is debatable hypertension which to procedureEisenmenger provides physiology, such an it is effect debatable in a better which way. procedure A modeling provides study such involving an effect inthe a CircAdaptbetter way. cardiovascular A modeling study model involving of a thepatient CircAdapt with PAH cardiovascular of various model severities of a patient was conductedwith PAH ofby various Delhaas severities et al. [12]. was They conducted reported by Delhaasthe theoretical et al. [12 superiority]. They reported of Potts the shuntingtheoretical over superiority AS. However, of Potts these shunting theoretical over AS.studies However, were not these supported theoretical by studiesrandomized were not supported by randomized controlled trials, as we did not find any in our literature controlled trials, as we did not find any in our literature review. Detailed depictions of AS review. Detailed depictions of AS and Potts shunting are presented in Figure1. and Potts shunting are presented in Figure 1. FigureFigure 1. 1. TreatmentsTreatments utilizing utilizing right-to-left right-to-left shunting: shunting: (1) (Atrial1) Atrial septostomy, septostomy, (2a) ( 2aatrial) atrial flow flow regulator regulator (AFR) (AFR) at the at site, the ( site,2b) scheme(2b) scheme of the of AFR, the AFR, (3a) Potts
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