scholarly journals Lung transplantation in an intensive care patient with pulmonary alveolar microlithiasis - a case report

F1000Research ◽  
2014 ◽  
Vol 3 ◽  
pp. 118 ◽  
Author(s):  
Bülent Güçyetmez ◽  
Aylin Ogan ◽  
Aylin Çimet Ayyıldız ◽  
Berrin Yalçın Güder ◽  
Walter Klepetko
Keyword(s):  
Heart Failure ◽  
Intensive Care ◽  
Case Report ◽  
Life Expectancy ◽  
Lung Transplantation ◽  
Right Heart Failure ◽  
Right Heart ◽  
Bilateral Lung Transplantation ◽  
Bilateral Lung ◽  
Alveolar Microlithiasis

Introduction: Pulmonary alveolar microlithiasis (PAM) is an autosomal recessive disease characterized by the deposition of phosphate and calcium in the alveoli. The disease progresses asymptomatically until later stages. When it becomes symptomatic, lung transplantations performed before the onset of right heart failure may improve life expectancy and quality. Here we present a case report concerning the very first Turkish PAM patient to have undergone lung transplantation surgery.Patient information: A 52 year-old female, Caucasian patient, already diagnosed with PAM in infancy, was admitted to the intensive care unit, diagnosed with pneumonia and hospitalized for 20 days. We decided to refer the patient to a specialized center for lung transplantation. Bilateral lung transplantation was performed in Vienna 14 months later and no recurrence was observed during the first postoperative year.Conclusion: Bilateral lung transplantation may improve both the life expectancy and the quality of life of PAM diagnosed patients with severe respiratory failure who do not suffer from right heart failure. The risk of recurrence should not be considered as a justifying reason to avoid transplantation as a treatment method.

Near syncope is not always benign

2020 ◽  
pp. 211-226
Author(s):  
Paraskevi Theocharis ◽  
Roberta Bini
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Correct Diagnosis ◽  
Rare Condition ◽  
Right Heart Failure ◽  
Therapeutic Strategies ◽  
Right Heart ◽  
Bilateral Lung Transplantation ◽  
Starting Point ◽  
Bilateral Lung

A ‘not uncommon’ episode of near syncope in a 6-year-old child is the starting point for cardiology screening of pulmonary hypertension, a rare condition in paediatrics, posed two questions—was SVT secondary to right heart failure that is secondary to pulmonary hypertension? Or was SVT precipitated by right heart failure in the presence of pulmonary hypertension? The chapter goes on to describe the meticulous and extensive screening necessary to establish the correct diagnosis, aiming to ascertain the presence, type, and severity of pulmonary hypertension. The differential diagnosis is fundamental to the therapeutic strategies. There is no cure. Palliative procedures and drug therapy improve symptoms and prolong life, but eventually the disease progresses. When the response to therapeutic strategies decreases and symptoms worsen, the only option that remains is bilateral lung transplantation.

scholarly journals Severe thrombocytopenia in patients with idiopathic pulmonary arterial hypertension provided several strategies for lung transplantation

2020 ◽  
Vol 10 (4) ◽  
pp. 204589402096910
Author(s):  
Takayuki Kobayashi ◽  
Ayako Shigeta ◽  
Jiro Terada ◽  
Nobuhiro Tanabe ◽  
Toshihiko Sugiura ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lung Transplantation ◽  
Right Heart Failure ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Right Heart ◽  
Thrombocytopenic Purpura ◽  
Bilateral Lung Transplantation ◽  
Pulmonary Arterial ◽  
Bilateral Lung

While the prognosis of idiopathic pulmonary arterial hypertension has improved significantly due to newer medications, lung transplantation remains a critical therapeutic option for severe pulmonary arterial hypertension. Hence, it is essential for patients awaiting lung transplantation to avoid complications, including thrombocytopenia, which may affect their surgical outcomes. Herein we present the case of a 21-year-old woman diagnosed with idiopathic pulmonary arterial hypertension at the age of 15. She developed thrombocytopenia while awaiting lung transplantation. Her medication was switched from epoprostenol to treprostinil, suspecting possible drug-induced thrombocytopenia. Furthermore, she was administered thrombopoietin receptor agonists in view of the possibility of idiopathic thrombocytopenic purpura, along with maximum support for right heart failure. Subsequently, her platelet count increased to >70,000/µL, enabling her to successfully undergo bilateral lung transplantation. Post-bilateral lung transplantation, pulmonary arterial hypertension as well as thrombocytopenia appeared to have resolved. In this case, we suspected that thrombocytopenia could have resulted owing to a combination of pulmonary arterial hypertension, right heart failure, drug interactions, and idiopathic thrombocytopenic purpura. Thrombocytopenia is a very critical condition in patients with pulmonary arterial hypertension, especially those awaiting lung transplantation. Several approaches are known to improve intractable thrombocytopenia in patients with pulmonary arterial hypertension.

scholarly journals The waffle procedure as treatment of a first episode of right heart failure: a case report

2018 ◽  
Vol 2 (4) ◽  
Author(s):  
Carolina Espejo-Paeres ◽  
Pedro Marcos-Alberca ◽  
Carlos Nicolás-Pérez ◽  
Carlos Macaya
Keyword(s):  
Heart Failure ◽  
Case Report ◽  
Right Heart Failure ◽  
First Episode ◽  
Right Heart ◽  
Waffle Procedure

scholarly journals Metastatic adenocarcinoma involving the right ventricle and pulmonary artery leading to right heart failure: case report

2017 ◽  
Vol 136 (3) ◽  
pp. 262-265 ◽  
Author(s):  
Turgut Karabag ◽  
Caner Arslan ◽  
Turab Yakisan ◽  
Aziz Vatan ◽  
Duygu Sak
Keyword(s):  
Heart Failure ◽  
Case Report ◽  
Pulmonary Artery ◽  
Right Ventricle ◽  
Right Ventricular ◽  
Right Ventricular Outflow Tract ◽  
Right Heart Failure ◽  
Ventricular Outflow Tract ◽  
Right Heart ◽  
The Right

ABSTRACT CONTEXT: Obstruction of the right ventricular outflow tract due to metastatic disease is rare. Clinical recognition of cardiac metastatic tumors is rare and continues to present a diagnostic and therapeutic challenge. CASE REPORT: We present the case of a patient who had severe respiratory insufficiency and whose clinical examinations revealed a giant tumor mass extending from the right ventricle to the pulmonary artery. We discuss the diagnostic and therapeutic options. CONCLUSION: In patients presenting with acute right heart failure, right ventricular masses should be kept in mind. Transthoracic echocardiography appears to be the most easily available, noninvasive, cost-effective and useful technique in making the differential diagnosis.

scholarly journals Critical Care Management of Decompensated Right Heart Failure in Pulmonary Arterial Hypertension Patients – An Ongoing Approach

2021 ◽  
Vol 7 (3) ◽  
pp. 170-183
Author(s):  
Ioan Tilea ◽  
Andreea Varga ◽  
Anca-Meda Georgescu ◽  
Bianca-Liana Grigorescu
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Intensive Care ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Care Management ◽  
Right Heart Failure ◽  
Right Heart ◽  
Continuous Increase ◽  
Pulmonary Arterial

Abstract Despite substantial advancements in diagnosis and specific medical therapy in pulmonary arterial hypertension patients’ management, this condition continues to represent a major cause of mortality worldwide. In pulmonary arterial hypertension, the continuous increase of pulmonary vascular resistance and rapid development of right heart failure determine a poor prognosis. Against targeted therapy, patients inexorable deteriorate over time. Pulmonary arterial hypertension patients with acute right heart failure who need intensive care unit admission present a complexity of the disease pathophysiology. Intensive care management challenges are multifaceted. Awareness of algorithms of right-sided heart failure monitoring in intensive care units, targeted pulmonary hypertension therapies, and recognition of precipitating factors, hemodynamic instability and progressive multisystem organ failure requires a multidisciplinary pulmonary hypertension team. This paper summarizes the management strategies of acute right-sided heart failure in pulmonary arterial hypertension adult cases based on recently available data.

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