An unusual case of hemoptysis secondary to Abernethy malformation type II and hepatopulmonary syndrome, pulmonary arterial hypertension

A twenty years old male college student came with history of hemoptysis. His chest X-ray showed diffuse infiltrative shadows and he was diagnosed as a case of a case of pulmonary tuberculosis with hemoptysis. On detailed investigations he was found to have Abernethy malformation Type-II with primitive portal vein joining extrahepatic inferior vanacava leading to cirrhosis of liver, porto-pulmonary syndrome, pulmonary arterial hypertension and hemoptysis.

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Role of Bone Morphogenetic Protein Type II Receptor Signaling in Pulmonary Arterial Hypertension

Cardiovascular Pharmacology Open Access ◽

10.4172/2329-6607.1000e120 ◽

2013 ◽

Vol 02 (04) ◽

Author(s):

Qiwei Yang Miranda Sun

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Bone Morphogenetic Protein ◽

Type Ii ◽

Receptor Signaling ◽

Morphogenetic Protein ◽

Pulmonary Arterial

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Repetitive and Brief Occlusion of Total Right Atrium Venous Blood Flow Improves Experimental Pulmonary Arterial Hypertension: a novel potential therapeutic strategy

10.1101/2020.10.04.314708 ◽

2020 ◽

Author(s):

Nan Liu ◽

Ying Xing ◽

Chen Wang

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Therapeutic Strategy ◽

Superior Vena ◽

Venous Blood ◽

Vena Cava ◽

Vegf Receptor ◽

Venous Blood Flow ◽

History Of ◽

Pulmonary Arterial

AbstractPulmonary arterial hypertension (PAH) is a vascular disorder associated with significant morbidity and mortality. The pathophysiology of PAH remains controversial, but the only currently available therapies for PAH are pharmacological pulmonary artery vasodilation, decreasing right ventricular (RV) afterload, and relieving symptoms. By now, there is no therapy being able to minimize vascular remodeling processes and thus to reverse or delay the natural history of the disease. It has been generally thought that reduction of RV preload was detrimental, which deteriorated the systemic hemodynamics. In the present study, however, we repetitively and briefly occluded (RBO) both superior vena cava and inferior vena cava by ligation (occlusion for less than 5 seconds then re-open for 30 seconds and repeated 5 cycles as one sequence, 1 sequence every 6 hours) to intermittently restrict RV preload, for continuous 24 hours, total 5 sequences, in the Sugen 5416 (VEGF receptor blocker) and hypoxia induced PAH rat models and we found this strategy was beneficial for lowering pulmonary vascular resistance (PVR).

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Managing the Patient With Pulmonary Arterial Hypertension and Methamphetamine Use: A Practical Perspective for the Clinician

Advances in Pulmonary Hypertension ◽

10.21693/1933-088x-17.2.55 ◽

2018 ◽

Vol 17 (2) ◽

pp. 55-62 ◽

Cited By ~ 1

Author(s):

Nimaljeet Tarango ◽

Andrea Gergay Baird

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

San Francisco ◽

Medical Center ◽

The United States ◽

Methamphetamine Abuse ◽

Methamphetamine Use ◽

Personal Experiences ◽

History Of ◽

Pulmonary Arterial

Pulmonary arterial hypertension (PAH) is a serious, chronic, progressive cardiopulmonary disease. PAH is associated with several concomitant conditions, as well as drugs and toxins.12 Methamphetamine abuse is likely associated with the development of PAH.3 Methamphetamine abuse is epidemic in the United States and abroad, with rates of new users escalating since 2012. There are over 100,000 new users annually as young as 12 years old. Treating a patient with a history of methamphetamine abuse poses many challenges for a clinician, including nonadherence, therapeutic treatment selection, complex psychosocial issues, and relapse or continued drug abuse. Patients with methamphetamine-associated PAH (Meth-APAH) have higher mortality rates when compared to idiopathic PAH.3 Having a better understanding of the complexities of addiction and working with a multidisciplinary team that includes a social worker to provide care and counseling to these patients can improve their trajectory. In this article, we will offer insight and background into methamphetamine abuse and addiction, as well as discuss a practical approach for clinicians in treating a patient with Meth-APAH, based on the literature, as well as our personal experiences at University of California, San Francisco Medical Center.

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Non-parenteral Therapy for Pulmonary Arterial Hypertension: A Review of Efficacy, Tolerability and Factors Related to Patient Adherence

Clinical Medicine Insights Therapeutics ◽

10.4137/cmt.s2689 ◽

2011 ◽

Vol 3 ◽

pp. CMT.S2689

Author(s):

Geoff Strange ◽

Christianne Manterfield ◽

Trudi Miller ◽

Annette Pidoux ◽

Karen Brown ◽

...

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Patient Adherence ◽

Multidisciplinary Care ◽

Premature Death ◽

Progressive Increase ◽

Effective Manner ◽

History Of ◽

Pulmonary Arterial

Pulmonary arterial hypertension (PAH) is defined as a group of diseases characterized by a progressive increase in pulmonary vascular resistance (PVR) leading to right ventricular failure and premature death. Untreated, it is a potentially devastating disease. However, the past decade has seen remarkable improvements in our understanding of the pathology associated with the condition and the development of multiple PAH-specific therapies with the ability to alter the natural history of the disease. These new advances provide a significant opportunity for practitioners to detect and treat patients with PAH in a timely and effective manner, thereby improving overall mortality, morbidity, and quality of life associated with this disease. The aim of this review is two-fold: firstly to review the evidence for efficacy and safety of non-parenteral PAH therapies and to discuss treatment selection based on clinically meaningful differences among the approved therapies, such as the potential for serious drug-drug interactions, convenience of dosing schedules, and rates of limiting side effects. Secondly, the central role of the PAH clinical nurse in the multidisciplinary care of patients with PAH will be discussed, together with issues relating to adherence and interventions to enhance patient compliance.

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Pulmonary vasculature

10.1093/med/9780199657742.003.0017 ◽

2017 ◽

Author(s):

Sophie Vergnaud ◽

David Dobarro ◽

John Wort

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Treatment Strategies ◽

Symptomatic Improvement ◽

Pulmonary Vasculature ◽

History Of ◽

Diffuse Cutaneous Systemic Sclerosis ◽

Pulmonary Arterial ◽

Phosphodiesterase 5 ◽

Haemodynamic Improvement

A 16-year-old girl with a diagnosis of diffuse cutaneous systemic sclerosis is referred to a specialist pulmonary hypertension centre with a history of progressive breathlessness, reduced exercise tolerance, and raised pulmonary pressures on transthoracic echocardiogram. She is found to have pulmonary arterial hypertension on right cardiac catheterization and is started on sildenafil, a phosphodiesterase-5 inhibitor, which stabilizes her condition. An endothelin receptor antagonist is added, which provides some initial symptomatic improvement. She continues to deteriorate over a period of 5 years, ultimately requiring intravenous prostanoids, the only treatment to provide a real symptomatic and haemodynamic improvement. This chapter explores the physiology and pathophysiology of pulmonary arterial hypertension, its classification, the means of investigation and diagnosis, who to refer to specialist centres, and the concepts behind current and future treatment strategies.

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