Lung transplantation in patients with pulmonary arterial  hypertension: The opinion of the Polish Cardiac Society  Working Group on Pulmonary Circulation

Mularek‐Kubzdela, Tatiana; Wojnarski, Jacek; Kamiński, Karol; Ochman, Marek; Kasprzak, Jarosław D.; Stącel, Tomasz; Kurzyna, Marcin; Karolak, Wojtek; Mroczek, Ewa; Kopeć, Grzegorz; Przybylski, Roman; Skorzylas, Ilona; Żegleń, Sławomir; Lewicka, Ewa; Urlik, Maciej; Grabka, Marek; Furdal, Michał; Florczyk, Michał; Torbicki, Adam

doi:10.33963/kp.a2022.0259

Cited by 3 publications

(2 citation statements)

References 64 publications

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“…A necessary indication for surgical repair of PAA is complicated with signs of dissection and rupture, which requires verification of etiology and specific PAP beforehand. Other indications include clinical symptoms, associated with pulmonary valvular disease, and compression of adjacent structures or tissues by the dilated PAA [4,24]. Sometimes conservative treatment also can be an option for patients with suitable situations but still need a meticulous and comprehensive evaluation [16,18].…”

Section: Discussionmentioning

confidence: 99%

Surgical Repair of Giant Dissecting Pulmonary Artery Aneurysm Associated with Atrial Septal Defect and Pulmonary Arterial Hypertension: A Case Report

Xie,

Ma,

Yuan

et al. 2024

HSF

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Pulmonary artery aneurysm (PAA) and pulmonary artery dissection (PAD) are rare and potentially fatal conditions that may lead to pulmonary artery (PA) rupture and cardiac tamponade. PAA is often associated with other cardiac and congenital heart diseases, such as atrial septal defect (ASD). We report a case of a patient with ASD and a giant dissecting PAA who underwent surgical repair to prevent potentially fatal outcome and discuss the probable etiologies of this case. We present a rare case of a 50-year-old woman with a secundum ASD and severe pulmonary arterial hypertension (PAH) who developed a giant PAA of 114 mm with dissection. The PAA caused extrinsic compression of the left main coronary artery (LMCA), which was misdiagnosed as coronary artery disease (CAD) at local hospital. Right heart catheterization revealed PAH of 73 mmHg and she was referred to our center for further treatment. After 4 months of lowering PAH treatment, she underwent successful surgical repair of the PAA to release the compression of LMCA, as well as mechanical valve replacement, fenestrated ASD closure and tricuspid valvuloplasty. She had an uneventful recovery and showed significant improvement in pulmonary hemodynamics and clinical symptoms at one-year follow-up. PAA with dissection is a rare complication of ASD and PAH that can potentially be fatal. Patients with large or symptomatic PAA and PAD may require early surgical intervention, particularly if they experience compression of nearby structures or are at risk of rupture. It is crucial to promptly refer and consult with specialists and ensure optimal preoperative hemodynamic management to enhance patient outcomes.

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Section: Discussionmentioning

confidence: 99%

Surgical Repair of Giant Dissecting Pulmonary Artery Aneurysm Associated with Atrial Septal Defect and Pulmonary Arterial Hypertension: A Case Report

Xie,

Ma,

Yuan

et al. 2024

HSF

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“…Lung transplantation is not contraindicated in patients with CTD-PAH. However, evaluating other potentially involved organs, especially the digestive system, kidneys, heart and skin, must be considered [ 15 , 26 ].…”

Section: Therapymentioning

confidence: 99%

Recent Advances in the Treatment of Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases

Smukowska-Gorynia,

Gościniak,

Woźniak

et al. 2023

Pharmaceuticals

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Pulmonary hypertension (PH) is a severe vascular complication of connective tissue diseases (CTD). Patients with CTD may develop PH belonging to diverse groups: (1) pulmonary arterial hypertension (PAH), (2) PH due to left heart disease, (3) secondary PH due to lung disease and/or hypoxia and (4) chronic thromboembolic pulmonary hypertension (CTEPH). PAH most often develops in systemic scleroderma (SSc), mostly in its limited variant. PAH-CTD is a progressive disease characterized by poor prognosis. Therefore, early diagnosis should be established. A specific treatment for PAH-CTD is currently available and recommended: prostacyclin derivative (treprostinil, epoprostenol, iloprost, selexipag), nitric oxide and natriuretic pathway: stimulators of soluble guanylate cyclase (sGC: riociguat) and phosphodiesterase-five inhibitors (PDE5i: sildenafil, tadalafil), endothelin receptor antagonists (ERA: bosentan, macitentan, ambrisentan). Moreover, novel drugs, e.g., sotatercept, have been intensively investigated in clinical trials. We aim to review the literature on recent advances in the treatment strategy and prognosis of patients with PAH-CTD. In this manuscript, we discuss the mechanism of action of PAH-specific drugs and new agents and the latest research conducted on PAH-CTD patients.

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Recent Advances in the Treatment of Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases

Smukowska-Gorynia,

Gościniak,

Woźniak

et al. 2023

Preprint

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Pulmonary hypertension (PH) is a severe vascular complication of connective tissue diseases (CTD). Patients with CTD may develop PH belonging to different groups: 1) pulmonary arterial hypertension (PAH), 2) PH secondary to lung disease and/or hypoxia, 3) PH due to left heart disease, and 4) chronic thromboembolic pulmonary hypertension (CTEPH). PAH most often develops in systemic scleroderma (SSc), mostly in its limited variant. PAH-CTD is a progressive disease characterized by poor prognosis, therefore the early diagnosis should be established A specific treatment for PAH-CTD is currently available and recommended: prostacyclin derivative (treprostinil, epoprostenol, iloprost, selexipag), nitric oxide and natriuretic pathway: stimulators of soluble guanylate cyclase (sGC: riociguat) and phosphodiesterase 5 inhibitors (PDE5i: sildenafil, tadalafil), endothelin receptor antagonists (ERA: bosentan, macitentan, ambrisentan). Moreover, novel drugs e.g. sotatercept are intensively investigated in the clinical trials. The aim of this paper is to review the literature on the recent advances in treatment strategy and prognosis of patients with PAH-CTD.

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Lung transplantation in patients with pulmonary arterial hypertension: The opinion of the Polish Cardiac Society Working Group on Pulmonary Circulation

Cited by 3 publications

References 64 publications

Surgical Repair of Giant Dissecting Pulmonary Artery Aneurysm Associated with Atrial Septal Defect and Pulmonary Arterial Hypertension: A Case Report

Surgical Repair of Giant Dissecting Pulmonary Artery Aneurysm Associated with Atrial Septal Defect and Pulmonary Arterial Hypertension: A Case Report

Recent Advances in the Treatment of Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases

Recent Advances in the Treatment of Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases

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