Pulmonary Arterial Hypertension: A Rare Complication of Primary Sjögren Syndrome

This review focuses on a rare complication of pulmonary arterial hypertension (PAH), extravasation compression of the left coronary artery (LCA) dilated by the pulmonary artery. The review described clinical manifestations and methods for diagnostics of LCA compression, and advantages of the endovascular correction of this complication in patients with pulmonary hypertension. Selection of a device to be implanted during the endovascular intervention in these patients was discussed with due account for concomitant treatment with oral anticoagulants. As an illustration of the issue under discussion, a clinical case of acute coronary syndrome in a female patient from the PAH Registry of the V. A. Almazov National Medical Research Center was provided.

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Central airway compression by massively dilated pulmonary artery in a patient with pulmonary arterial hypertension: a rare entity

BMJ Case Reports ◽

10.1136/bcr-2019-232468 ◽

2019 ◽

Vol 12 (11) ◽

pp. e232468

Author(s):

Biplab Kumar Saha ◽

Scott Beegle

Keyword(s):

Pulmonary Arterial Hypertension ◽

Pulmonary Artery ◽

Arterial Hypertension ◽

Left Main Coronary Artery ◽

Rare Complication ◽

Unexpected Death ◽

Pulmonary Arterial ◽

Central Airway ◽

Ventilatory Limitation

Patients with pulmonary arterial hypertension (PAH) usually die from progressive right ventricular failure. Mechanical complications due to pulmonary artery (PA) enlargement are rare and include tracheobronchial and left main coronary artery compression, and PA dissection. A 62-year-old female with PAH was seen in our office for follow-up. During the evaluation, spirometry was performed, which revealed a severe obstructive ventilatory limitation. Subsequent workup identified compression of bilateral mainstem bronchi from the dilated PA as the aetiology for the abnormal spirometry. Very few cases of this rare complication have been reported in the literature. A significant dilation of the PA is necessary (>4 cm) for the occurrence of these complications. Dilation of PA is an independent risk factor for sudden unexpected death in patients with PAH.

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Human Immunodeficiency Virus and Pulmonary Arterial Hypertension

ISRN Cardiology ◽

10.1155/2013/903454 ◽

2013 ◽

Vol 2013 ◽

pp. 1-11 ◽

Cited By ~ 5

Author(s):

Aibek E. Mirrakhimov ◽

Alaa M. Ali ◽

Aram Barbaryan ◽

Suartcha Prueksaritanond

Keyword(s):

Human Immunodeficiency Virus ◽

Pulmonary Hypertension ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Clinical Presentation ◽

Rare Complication ◽

Viral Proteins ◽

Future Studies ◽

Immunodeficiency Virus ◽

Pulmonary Arterial

Human immunodeficiency virus- (HIV-) related pulmonary arterial hypertension (PAH) is a rare complication of HIV infection. The pathophysiology of HIV-related PAH is complex, with viral proteins seeming to play the major role. However, other factors, such as coinfection with other microorganisms and HIV-related systemic inflammation, might also contribute. The clinical presentation of HIV-related PAH and diagnosis is similar to other forms of pulmonary hypertension. Both PAH-specific therapies and HAART are important in HIV-related PAH management. Future studies investigating the pathogenesis are needed to discover new therapeutic targets and treatments.

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Pulmonary Arterial Hypertension In A Female Patient With Systemic Lupus Erythematosus

Internal Medicine ◽

10.2478/inmed-2018-0040 ◽

2018 ◽

Vol 15 (5) ◽

pp. 75-81

Author(s):

Cristiana Drăgănescu ◽

Tudor Constantinescu ◽

Oana Enache ◽

Marina Speriatu ◽

Raida Oneaţă ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Female Patient ◽

Lupus Erythematosus ◽

Rare Complication ◽

Heart Catheterization ◽

Systemic Lupus ◽

Cutaneous Manifestations ◽

Pulmonary Arterial

AbstractIntroduction. Systemic lupus erythematosus (SLE) is a chronic autoimmune disease of unknown origin, characterized by multisystemic involvement and a potentially severe evolution. Pulmonary arterial hypertension (PAH) is a rare complication of SLE, with low 5-year survival.Case presentation. We are presenting the case of a female patient, aged 56 years old, diagnosed in 1992 with SLE with cutaneous manifestations (butterfly-shaped erythematous rash), joint manifestations (polyarthritis), serositis manifestations (massive pleuropericarditis), and immunological manifestations (positive anti-dsDNA antibodies, decreased C3), ignored therapeutically for a long time. In 2010 she complained of dyspnea on medium exertion and leg edemas, with marked increase of PAPs by echocardiography. She was diagnosed with severe PAH (confirmed by right heart catheterization) and in the “Marius Nasta” National Institute of Pneumology she started a treatment with an endothelin receptor antagonist (Bosentan) in combination with a prostacyclin receptor agonist (Selexipag). Since 2013 the patient is on oral anticoagulant treatment for permanent atrial fibrillation.In 2015 she was referred back to out clinic as she complained of recurrent episodes of massive ascites with evacuatory paracenteses in amounts of about 6-9L per paracentesis. After excluding other causes, ascites was considered to be secondary to the SLE, and a treatment was initiated with Hydroxychloroquine (HCQ) and pulse therapy with Methylprednisolone, on which the remission of the ascites was achieved during the following months. Currently, the SLE is well controlled without recurrence of ascites on treatment with HCQ and gradual decrease until stopping of cortisone doses, and the PAH is stable.Conclusion. PAH is a rare complication of the SLE, with a complex pathophysiological immune mechanism, for which - together with the specific vasodilator treatment - the increase of immune suppression is recommended.

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Critical Neonatal Congenital Heart Disease – a Rare Complication after Successful Surgical Correction

Journal Of Cardiovascular Emergencies ◽

10.2478/jce-2020-0019 ◽

2020 ◽

Vol 6 (4) ◽

pp. 104-108

Author(s):

Carmen Corina Şuteu ◽

Iolanda Muntean ◽

Cristina Blesneac ◽

Marian Pop ◽

Rodica Togănel

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Neonatal Period ◽

Arterial Switch Operation ◽

Rare Complication ◽

Pediatric Case ◽

Vasodilator Therapy ◽

Switch Operation ◽

Pulmonary Arterial ◽

Favorable Clinical Response

AbstractPulmonary arterial hypertension (PAH) is a rare but severe complication that should be investigated in patients diagnosed with transposition of the great arteries who have undergone neonatal arterial switch operation. Early diagnosis and aggressive combination therapy for PAH could help to improve survival in these patients. We report a favorable clinical response 6 months after the initiation of vasodilator therapy in a pediatric case with transposition of the great arteries, successfully repaired in the neonatal period, who developed pulmonary arterial hypertension at the age of 5 years.

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Dasatinib-Induced Pulmonary Arterial Hypertension Treated with Upfront Combination Therapy

Case Reports in Cardiology ◽

10.1155/2018/3895197 ◽

2018 ◽

Vol 2018 ◽

pp. 1-5 ◽

Cited By ~ 1

Author(s):

Makoto Nishimori ◽

Tomoyuki Honjo ◽

Kenji Kaihotsu ◽

Naohiko Sone ◽

Sachiko Yoshikawa ◽

...

Keyword(s):

Pulmonary Arterial Hypertension ◽

Combination Therapy ◽

Arterial Hypertension ◽

Rare Complication ◽

Functional Class ◽

World Health ◽

First Line Therapy ◽

Mean Pulmonary Arterial Pressure ◽

Pulmonary Arterial ◽

Health Organization

Pulmonary arterial hypertension (PAH) is a rare complication of dasatinib that was approved as a first-line therapy for chronic myelocytic leukemia (CML). A 24-year-old man presenting dyspnea at rest and leg edema was admitted to our hospital. He had been diagnosed with CML and prescribed dasatinib for 4 years. Chest X-ray showed significant bilateral pleural effusion and heart enlargement. Echocardiography revealed interventricular septal compression and elevated peak tricuspid regurgitation pressure gradient of 66.7 mmHg indicating severe pulmonary hypertension. After the other specific diseases to provoke PAH were excluded, he was diagnosed with dasatinib-induced PAH. Despite discontinuation of dasatinib and intravenous administration of diuretic for two weeks, World Health Organization (WHO) functional class was still II and mean pulmonary arterial pressure (PAP) was high at 37 mmHg. Therefore, we administered sildenafil and bosentan together as an upfront combination therapy three weeks after dasatinib discontinuation. Six months later, his symptoms improved to WHO functional class I and mean PAP was decreased to 31 mmHg. Although PAH is a rare complication of dasatinib, symptomatic patients prescribed with dasatinib should have an echocardiogram for PAH screening. Moreover, the upfront combination therapy would be a useful option for symptomatic patients after discontinuation of dasatinib.

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